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Supplementary Materials Contents Overview of the Project ...... 1 Detailed Examples for Recommendation 1 ...... 2 Detailed Examples for Recommendation 4 ...... 4 Supplementary References ...... 6 Figure 1: Part of the data extraction form created in REDCap...... 7 MEDLINE Search Strategy ...... 8 Symptom Management Search ...... 18 Alertness Symptom Search ...... 37 Behaviour Symptom Search...... 38 Bowel Incontinence Symptom Search ...... 39 Breathing Difficulty Symptom Search ...... 39 Symptom Search ...... 40 Feeding Difficulties Symptom Search ...... 41 Sleep Symptom Search ...... 42 Temperature Regulation Symptom Search ...... 43 Tone and Motor Difficulties Symptom Search ...... 44 Symptom Search ...... 45

Overview of the Project Quadrant 3 (Q3) conditions, our area of investigation, are progressive, metabolic, neurological,

or chromosomal childhood conditions for which there currently exists no curative treatment. The

term Q3 derives from the four-category model for palliative care services developed by the

Association for Children’s Palliative Care (ACT; now renamed Together for Short Lives), which

categorizes the disease trajectories of children in palliative care into four quadrants (Q1-Q4).[1]

Children with Q3 conditions have unknown lifespans and face a high burden of symptoms.

Families and healthcare professionals caring for children with Q3 conditions are challenged by

the lack of research on symptom management in these conditions. In the context of this lack of

evidence, we undertook a scoping review to identify and map the literature with regard to 10

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important symptoms that affect children with Q3 conditions, excluding and

/ as those symptoms have been well-documented in previous studies.[2] Our

goals were to identify evidence for clinical decision-making and to highlight gaps for future

research.

Detailed Examples for Recommendation 1 Engage the expertise of a librarian throughout the process In our study, creating the search strategy was particularly complex due to the large number of Q3

conditions. As no definitive name for this grouping of conditions exists, it was not possible to

search for these conditions as a whole. There also is no single, accepted definitive list of Q3

conditions and often there are multiple names for the same condition. Other researchers may find

themselves with similar issues in defining the population or interventions of interest and so a

librarian can be helpful in developing a way to delineate the conditions or other key concepts so

they can be used as a basis for the search strategy and for developing inclusion/exclusion criteria.

In our study, the librarian helped us to construct a single list of Q3 conditions by combining the

conditions identified in a longitudinal study that aimed to describe the symptoms of children with

180 Q3 conditions and information from two other lists of life-threatening conditions compiled

by experts in the field.[3-5]

A list of conditions or a specified area of interest is only a place to start however. Inclusion and

exclusion criteria need to be developed and terms appropriate to the databases to be searched

must be identified. Many healthcare researchers are somewhat familiar with the indexing

systems used in databases such as MEDLINE or CINAHL, but it is unlikely that most

researchers understand the systems in the same way as a well-educated health librarian. A

systematic search strategy uses both keywords and controlled vocabulary, when the database

allows, and aims to balance sensitivity with specificity. Controlled vocabulary refers to pre-

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selected terms in databases that are assigned to references so that they can be more easily

retrieved without the use of synonyms or alternative spellings.

In our study, the librarian began developing the search strategy using Ovid MEDLINE and found

that many of these conditions either did not have a MeSH term (i.e., the controlled vocabulary in

MEDLINE) or the MeSH term had only been added recently. Without the librarian, we may not

have been able to clearly identify these issues and, most importantly, then develop a strategy to

ensure we accessed as much of the relevant literature as possible. Thus, we suggest that scoping

review teams without a librarian may inadvertently put constraints on their searches that result in

limited coverage of the existing literature. Our librarian proposed that to cover all conditions,

including those without MeSH terms, we should include all possible names for the conditions in

our keywords. In addition, for more recently added MeSH terms she recommended that we use

broader terms for that condition and limit them to the years that were not covered by the current

MeSH term. These types of suggestions from a librarian can make the process much easier for

researchers who are not as familiar with the databases.

Our librarian’s proposals resulted in a final MEDLINE search strategy that used 219 MeSH

terms and 608 keywords to describe Q3 conditions. The full search was 957 lines long, which is

likely to be daunting to many researchers and indeed may not have been possible without the

librarian (see below for our full search strategy). The next issue that researchers may face is that

each database uses different controlled vocabulary and database commands. Therefore, having a

librarian to translate the search into the other databases, after the search is finalized and approved

by the full team, is invaluable. This process is time-consuming and greatly benefits from the

expertise of a librarian.

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Detailed Examples for Recommendation 4 Explore new research management tools, or re-purpose old ones A tool that is useful for the title and abstract screening phase of a scoping review is the web-

based software abstrackr.[6] In conducting past reviews, we have used Excel, which required

multiple sheets to be created and multiple versions to be tracked and compiled. That process was

time-consuming and somewhat complicated, so we sought a different approach to facilitate our

scoping review and we found abstrackr to be very helpful. Unlike Excel, abstrackr automatically

sets up a double-blind screening and allows each team member to complete as much screening as

needed without assigning separate Excel sheets for each set of screeners. Abstrackr also

automatically records decisions that can be downloaded to an Excel sheet and records when two

screeners do not agree, thus allowing the team to identify and discuss any areas of disagreement.

The next phase of a scoping review is a full-text review, which requires a different approach than

that provided in abstrackr. Though abstrackr is user-friendly and may be well-liked by a team, it

does not allow for full-text upload to facilitate screening of the full texts. Researchers may find

that the web and mobile application Rayyan provides access to what they need.[7] For example,

we had 338 full-text papers to review across seven reviewers and so we believed it was essential

to have the full text readily available within the system rather than requiring the reviewers to

search through a separate folder for the paper they were to examine. We had considered Rayyan

for the screening phase, but it was more complicated than abstrackr to use for that purpose. At

the time that we used Rayyan, it did not support double-blind screening for multiple reviewers. It

required more work and time to set up the reviews and oversee them, as we had to split the

studies to be reviewed into multiple parts and assign two reviewers to each one. However, due to

user feedback, this feature has since been added to Rayyan. A user is still required to use a filter

in the side-bar to narrow to papers that have only been judged once, so it remains less

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straightforward than abstrackr and other paid software; it also may require more training for team

members. Once the review set up is complete Rayyan allows team members to view the attached

PDFs and record their decisions within the application. It also keeps track of conflicts between

reviewers and allows the results to be downloaded in multiple formats when completed.

For the final phase, data extraction, we used Research Electronic Data Capture (REDCap), a

secure application designed for creating surveys and databases.[8] We were able to obtain remote

access to REDCap for all reviewers who were off-site and so all members of the team could

contribute to this final phase. Though not designed for use in knowledge synthesis, we

considered how to repurpose this application and found that we were able to create a data

extraction form that all team members could access in a single space without creating multiple

versions. Using multiple choice options for most of the data extraction fields allowed team

members to click rather than type out answers (see Figure 1). This approach not only improved

efficiency, but also ensured data integrity by limiting options to agreed-upon terms such as

disease or symptom name. By using the record status dashboard, we were also able to track our

progress as we reviewed each study. When the first reviewer completed data extraction from a

paper, the REDCap record was marked “unverified” and colour-coded yellow. When a second

team member checked the extracted data, the record was then marked “completed” and colour-

coded green. For studies not found to meet our inclusion criteria, we built in the option to

exclude the study and provide a reason for exclusion. We recommend that other researchers

consider adapting REDCap for their own purposes, if it is available within their setting.

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Supplementary References 1. ACT. A Guide to the Development of Children’s Palliative Care Services. 3rd ed. Bristol, UK; 2009.

2. Pawliuk C, Widger K, Dewan T, et al. Scoping review of symptoms in children with rare, progressive, life-threatening disorders. BMJ Supportive & Palliative Care. December 2019.

3. Siden H, Steele R. Charting the Territory: Children and families living with progressive life-threatening conditions. Paediatr Child Health. 2015;20(3):139-144.

4. Feudtner C, Feinstein J, Zhong W, Hall M, Dai D. Pediatric complex chronic conditions classification system version 2: updated for ICD-10 and complex medical technology dependence and transplantation. BMC Pediatr. 2014;14(1):199.

5. Hain R, Devins M, Hastings R, Noyes J. Paediatric palliative care: development and pilot study of a “Directory” of life-limiting conditions. BMC Palliat Care. 2013;12(1):43.

6. Wallace BC, Small K, Brodley CE, Lau J, Trikalinos TA. Deploying an Interactive Machine Learning System in an Evidence-based Practice Center: Abstrackr. In: Proceedings of the 2Nd ACM SIGHIT International Health Informatics Symposium. New York, NY, USA: ACM; 2012:819–824.

7. Ouzzani M, Hammady H, Fedorowicz Z, Elmagarmid A. Rayyan-a web and mobile app for systematic reviews. Syst Rev. 2016;5(1):210.

8. Harris PA, Taylor R, Thielke R, Payne J, Gonzalez N, Conde JG. Research Electronic Data Capture (REDCap) - A metadata-driven methodology and workflow process for providing translational research informatics support. J Biomed Inform. 2009;42(2):377-381.

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Figure 1: Part of the data extraction form created in REDCap.

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MEDLINE Search Strategy

1 Achondroplasia/ or Thanatophoric Dysplasia/ [ Diseases, Developmental] (2080) 2 (saddan dysplasia* or Achondroplasia* or achondroplasy or achondrosis).mp. (2247) 3 Acrocallosal Syndrome/ (77) 4 Acrocallosal.mp. (152) 5 / (56) 6 (Aicardi or (callosal adj3 agenesis) or (ocular adj3 abnormalit*)).mp. (2860) 7 (((3-Methylcrotonyl-CoA Carboxylase or 3MCC or BMCC) adj3 Deficiency) or 3- Methylcrotonylglycinuria).mp. (93) 8 , Acute Disseminated/ (1708) 9 (((post or acute) adj3 vaccina* *) or ((post or acute) adj3 infection* encephalitis*)).mp. (72) 10 "Agenesis of Corpus Callosum"/ (1967) 11 ((Agenesis adj2 Corpus Callosum) or ((hypogenes* or dysgenes* or absence*) adj2 corpus Callosum)).mp. (2300) 12 Aicardi Goutieres.mp. (289) 13 Alagille Syndrome/ (525) 14 (watson miller or alagille* or cadiovertebral or hepatofacioneurocardiovertebral or arteriohepatic displasia or (hepatic ductular adj2 hypoplasia)).mp. (835) 15 Holoprosencephaly/ (1046) 16 (Holoprosencephal* or arhinencephal*).mp. (2002) 17 Aspartylglucosaminuria/ (111) 18 ((Aspartylglucosaminuria* or glycoasparaginase* or aspartylglucosamidase) adj3 deficienc*).mp. (4) 19 ((Asphyxiating Thoracic adj3 dystroph*) or (short rib thoratic adj1 dysplasia*) or (jeune adj3 syndrome)).mp. (235) 20 Telangiectasia/ (3029) 21 ((Ataxia adj3 Telangiectasia) or (Louis adj2 Bar)).mp. (8344) 22 / (2049) 23 ((Rett adj3 (Syndrome or morbus)) or (cerebroatrophic adj3 hyperammonemia*)).mp. (2858) 24 Bartter Syndrome/ (1300) 25 Bartter.mp. (1626) 26 ((Bowen adj3 Conradi) or (Bowen adj3 Hutterite)).mp. (26) 27 Carbamoyl-Phosphate Synthase I Deficiency Disease/ (100) 28 ("Carbamoyl-Phosphate Synthase I Deficiency Disease" or "Carbamoyl Phosphate Synthase I Deficiency Disease").mp. (100) 29 Carbohydrate-Deficient Glycoprotein Syndrome/ (498) 30 ("Carbohydrate-Deficient Glycoprotein Syndrome" or "Carbohydrate Deficient Glycoprotein Syndrome" or (congenital adj3 glycosylation) or (congenital adj3 dyserythropoietic anemia) or (fukuyama congenital adj3 dystrophy) or (hereditary multiple adj3 exostosis) or (walker adj3 Warburg)).mp. (1821) 31 Carnitine O-Palmitoyltransferase/df (397) 32 (Carnitine O-Palmitoyltransferase adj3 deficienc*).mp. (0) 33 myopathies, structural, congenital/ or myopathies, nemaline/ or myopathy, central core/ (987) 34 ((Centronuclear or myotubular) adj3 myopath*).mp. (581) 35 ((autosomal recessive or Charlevoix Saguenay) adj2 Spastic Ataxia).mp. (130) 36 Cholesterol Ester Storage Disease/ (111) 37 ("Cholester#l Ester Storage Disease" or "Cholester#lester Storage Disease").mp. (207) 38 Wolf-Hirschhorn Syndrome/ (103)

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39 ((Wolf adj3 Hirschhorn) or (Pitt Rogers adj3 Danks) or ( 4p adj2 (Deletion or syndrome* or monosomy))).mp. (436) 40 Cri-du-Chat Syndrome/ (623) 41 Crying Cat Syndrome/ (623) 42 limit 41 to yr="1964 - 1976" (298) 43 ((Chromosome 5p adj3 Deletion) or Cri-du-Chat or (Crying adj3 Cat) or (cat adj3 cry)).mp. (706) 44 , Human, Pair 5/ and (Tetrasomy/ or Mosaicism/) (37) 45 (Tetrasomy 5p adj3 Mosaicism).mp. (4) 46 Chromosomes, Human, Pair 9/ and (Trisomy/ or Mosaicism/) (405) 47 ( adj3 Trisomy 9).mp. (48) 48 (Chromosomes, Human, Pair 18/ and Trisomy/) or (Chromosomes, Human, Pair 11/ and Trisomy/) (1659) 49 (Trisomy 11 or Trisomy 18).mp. (2224) 50 Chromosomes, Human, Pair 12/ and (Tetrasomy/ or Mosaicism/) (141) 51 ((Pallister adj3 Killian) or (isochromosome adj2 12p) or (Chromosome 12p adj2 (Mosaicism or Tetrasomy))).mp. (279) 52 Chromosomes, Human, Pair 13/ and Trisomy/ (928) 53 (Patau or Trisomy 13 or Trisomy d).mp. (1447) 54 Chromosomes, Human, Pair 18/ and Trisomy/ (1400) 55 ((Edward* adj3 Syndrome) or trisomy 18 or trisomy e).mp. (2249) 56 / (21870) 57 Down's Syndrome/ (21870) 58 limit 57 to yr="1975 - 1992" (5759) 59 Mongolism/ (21870) 60 limit 59 to yr="1966 - 1974" (2473) 61 (Trisomy 21 or (Down* adj2 Syndrome) or mongolism or mongoloid*).mp. (28715) 62 Chromosomes, Human, Pair 22/ and (Trisomy/ or Mosaic/) (265) 63 ((Whole chromosome adj3 trisomy) or (Meiotic adj3 Nondisjuntion) or Trisomy 22).mp. (274) 64 DiGeorge Syndrome/ (1677) 65 (DiGeorge or "Chromosome 22q11.2 Deletion").mp. (2345) 66 Common Variable Immunodeficiency/ (1718) 67 ("Common Variable Immunodeficiency" or (hypogammaglobulin?emia* adj3 (acquired or common variable)) or (agammaglobulin?emia* adj3 (acquired or common variable))).mp. (2753) 68 "Congenital Disorders of Glycosylation"/ (498) 69 ((Congenital Disorder* adj3 Glycosylation) or (carbohydrate deficient adj2 glycoprotein)).mp. (953) 70 /cn (215) 71 (Congenital adj3 Hydrocephal*).mp. (962) 72 (Congenital Myopathies and fib?r* type disproportion).mp. (51) 73 ((Congenital or Fetal) adj3 Varicella).mp. (235) 74 Costello Syndrome/ (89) 75 (Costello or faciocutaneoskeletal).mp. (472) 76 Creutzfeldt-Jakob Syndrome/ (5925) 77 Jakob-Creutzfeldt Syndrome/ (5925) 78 limit 77 to yr="1981 - 1990" (686) 79 Creutzfeldt-Jakob Disease/ (5925) 80 limit 79 to yr="1969 - 1980" (467) 81 ((Creutzfeldt adj3 Jakob) or (spongiform adj3 subacute) or (corticostriatospinal adj3 degeneration) or (spastic adj3 pseudosclerosis)).mp. (7453) 82 De Lange Syndrome/ (680) 83 (De Lange* or "Bushy Syndrome" or "Amsterdam dwarfism").mp. (1290) 84 "Diffuse Cerebral Sclerosis of Schilder"/ (2317)

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85 (Alper#s or (myelinoclastic adj3 diffuse sclerosis) or (diffuse cerebral adj3 sclerosis) or (balo* adj3 concentric sclerosis) or (progressive adj3 sclerosing poliodystroph*) or schilder*).mp. (2479) 86 Epidermolysis Bullosa, Junctional/ or Epidermolysis Bullosa/ (2829) 87 ((Epidermolysis adj3 Bullosa) or Herlitzs).mp. (5195) 88 Farber Lipogranulomatosis/ (25) 89 Lipidoses/ (3927) 90 limit 89 to yr="1976 - 2008" (974) 91 / (343) 92 limit 91 to yr="1992 - 2008" (108) 93 (Farber* or ((ceramidase or n-laurylsphingosine deacylase) adj3 deficienc*)).mp. (942) 94 Friedreich Ataxia/ (2270) 95 ((Friedreich* adj3 (Ataxia or disease or syndrome)) or (hereditary adj3 (spinal scleroses or spinal staxia)) or (spinal adj3 heredoataxia)).mp. (3007) 96 Fucosidosis/ (138 97 (Fucosidosis or (alpha fucosidase adj3 deficienc*)).mp. (309) 98 Gangliosidoses/ [Lysosomal Storage Diseases, ] (567) 99 (Gangliosidos* or ( adj3 (storage or lipidosis))).mp (1761) 100 alpha-N-Acetylgalactosaminidase/df [Deficiency] (5) 101 (((alpha-N-Acetylgalactosaminidase or alpha-NAGA) adj3 Deficienc*) or Schindler or Kanzaki).mp. (171) 102 Argininosuccinic Aciduria/ (139) 103 ((Argininosuccinic adj3 Aciduria*) or ((Argininosuccinate Lyase or argininosuccinase or ALS) adj3 deficienc*) or (arginino adj3 succinic) or mckusick 20790 or argininosuccinicaciduria).mp. (311) 104 Chromosomes, Human, Pair 1/ and monosomy/ (103) 105 (Chromosome 1p36 adj3 (Monosomy or deletion)).mp. (49) 106 Chromosomes, Human, Pair 3/ and Chromosome Inversion/ (111) 107 (Chromosome 3 adj3 Inversion).mp. (42) 108 Chromosomes, Human, Pair 3/ and Monosomy/ and (Chromosomes, Human, Pair 5/ and Trisomy/) (2) 109 (Chromosome adj3 (3p25 Partial Monosomy or 5p15* Trisomy)).mp. (0) 110 Chromosomes, Human, Pair 5/ and Trisomy/ (206) 111 ( adj3 Trisomy).mp. (42) 112 Chromosomes, Human, Pair 5/ and Chromosome Deletion/ (861) 113 (Chromosome 5q14* adj5 Deletion).mp. (0) 114 (Chromosomes, Human, Pair 6/ and Translocation, Genetic/) or (Chromosomes, Human, Pair 4/ and Translocation, Genetic/) (1445) 115 ((Chromosome 6 or Chromosome 4) and Unbalanced Translocation).mp. (32) 116 (Chromosomes, Human, Pair 7/ and Trisomy/) or (Chromosomes, Human, Pair 6/ and Monosomy/) (426) 117 ((Chromosome adj3 Unbalanced Rearrangement) or Partial Trisomy 7 or Monosomy 6).mp. (46) 118 Chromosomes, Human, Pair 14/ and Chromosome Deletion/ (303) 119 (Chromosome 14 adj3 Deletion).mp. (42) 120 Chromosomes, Human, Pair 18/ and Chromosome Deletion/ (447) 121 (Chromosome 18q adj3 Deletion).mp. (38) 122 Chromosomes, Human, Pair 18/ and Chromosomes, Human, Pair 5/ and Chromosome Deletion/ (35) 123 (Chromosome 18 and Chromosome 5 and Deletion).mp. (14) 124 Triploidy/ (226) 125 (Chromosome 69 adj3 Triploidy).mp. (0) 126 (Desminopath* or (Myopath* adj3 Myofibrillar) or (Desmin adj3 Related)).mp. (441)

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127 ((Dilated Cardiomyopathy adj3 Ataxia) or (3 Methylglutaconic adj3 Aciduria)).mp. (159) 128 , Myoclonic/ [Epilepsy] (2619) 129 ((Infantile Myoclonic adj3 Epilepsy) or Dravet*).mp. (544) 130 (Early adj3 Myoclonic Encephalopathy).mp. (77) 131 Gangliosidoses, GM2/ or Tay-Sachs Disease/ or Tay-Sachs Disease, AB Variant/ (1303) 132 ((GM2 adj3 Gangliosidos*) or (Tay adj2 Sach*) or ( adj3 deficiency)).mp. (2215) 133 (Gangliosidos* adj4 GM3).mp. (5) 134 Glucosephosphate Dehydrogenase Deficiency/ (4294) 135 ((Glucosephosphate Dehydrogenase or g6pd or Glucose-6-Phosphate Dehydrogenase) adj3 Deficiency).mp. (5089) 136 Glutaryl-CoA Dehydrogenase/df [Deficiency] (120) 137 ((Glutaric adj3 Acidemia Type I) or (Glutaric adj3 Aciduria 1) or (Glutaryl-CoA dehydrogenase adj3 deficienc*)).mp. (210) 138 Multiple Acyl Coenzyme A Dehydrogenase Deficiency/ (90) 139 ((Glutaric Aciduria adj3 (Type 2 or Type II)) or ((Multiple Acyl Coenzyme A Dehydrogenase or Electron transfer flavoprotein or oxidoreductase) adj3 deficienc*)).mp (340) 140 Glycogen Storage Disease Type II/ [Lysosomal Storage Diseases, Nervous System] (1323) 141 ((Glycogen Storage Disease adj3 Type II) or Pompe* or ((acid maltase or alpha-glucosidase) adj3 deficienc*)).mp. [Lysosomal Storage Diseases, Nervous System] (2342) 142 Glycogen Storage Disease Type IIb/ (98) 143 (Danon or antopol or (glycogen storage adj3 cardiomyopath*) or pseudoglycogenosis 2 or ((LAMP-2 or LAMP2 or associated membrane 2) adj3 deficenc*)).mp. (190) 144 Glycogen Storage Disease Type IV/ (176) 145 ((Glycogen Storage adj3 (Type IV or type 4)) or brancher or (andersen* adj3 disease) or glycogenos*).mp. (1783) 146 Hereditary Demyelinating Diseases/ (282) 147 (Hereditary Central Nervous System adj3 Demyelinat*).mp. (282) 148 Histiocytosis, Langerhans-/ (5024) 149 (((Histiocytos* or granulomatos*) adj3 Langerhans Cell) or (hand schu#ller adj3 Christian) or (aleukemic adj3 reticuloendotheliosis) or (histiocytos* adj3 x)).mp. (7973) 150 Hyperargininemia/ (166) 151 (Hyperargininemia* or (arginase adj3 deficienc*) or argininemia*).mp. (272) 152 / (628) 153 (Incontinentia Pigmenti or (bloc* adj3 sulzberger) or (hypomelanosis adj3 ito) or (neonatal pigment adj3 incontinence)).mp. (1272) 154 Pantothenate Kinase-Associated / (430) 155 ((Infantile Neuroaxonal adj3 Dystrophy) or (Pantothenate Kinase-Associated adj3 Neurodegeneration) or (Pigmentary Pallidal adj3 Degeneration) or hallervorden spatz).mp. (787) 156 Refsum Disease, Infantile/ (8) 157 ((Infantile adj3 Refsum) or (infantile adj3 phytanic acid storage disease)).mp. (135) 158 ((Joubert or Boltshauser) adj3 syndrome).mp. (557) 159 Huntington Disease/ (10175) 160 (huntington* adj5 juvenile).mp. (126) 161 Kearns-Sayre Syndrome/ (606) 162 ((Kearns adj3 Sayre) or (cpeo adj3 myopathy) or oculocraniosomatic or ophthalmoplegia).mp. (10663) 163 Klippel-Trenaunay-Weber Syndrome/ (1083) 164 (Klippel Trenaunay or angioosteohypertrophy or angio osteohypertrophy or (dysplastic adj3 angiopathy congenital) or congenital angiodysplasisa or (osteohypertrophic varicose adj3 naevus) or (parkes adj3 weber)).mp. (1525) 165 , Globoid Cell/ (860)

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166 (Krabbe or (Leukodystrophy adj3 Globoid Cell) or ( beta galactosidase adj3 deficienc*)).mp. (1191) 167 / (222) 168 (Lafora or (progressive adj3 (type 2 or type II))).mp. (525) 169 Leigh Disease/ (864) 170 ((Leigh adj3 (syndrome or disease)) or (necrotizing encephalomyelopath* adj3 subacute)).mp. (1237) 171 Lennox Gastaut Syndrome/ (192) 172 Spasms, Infantile/ (3025) 173 limit 172 to yr="1988 - 2014" (2109) 174 ((Lennox adj4 Gastaut) or (childhood epileptic adj3 encephalopathy)).mp. (1139) 175 Lesch-Nyhan Syndrome/ (1160) 176 (Lesch Nyhan or (juvenile adj3 hyperuricemia) or (guanine phosphoribosyltransferase adj3 deficienc*) or (hypoxanthine phosphoribosyltransferase adj3 deficienc*) or ( adj3 self mutilation)).mp. (1485) 177 / or Leukodystrophy, Metachromatic/ or / or Pelizaeus- Merzbacher Disease/ or Hereditary Central Nervous System Demyelinating Diseases/ (3349) 178 ((Alexander adj3 Disease) or Leukodystrophy or Adrenoleukodystrophy or (Pelizaeus adj3 Merzbacher)).mp. (5722) 179 Lipidoses/ or Cholesterol Ester Storage Disease/ or Sjogren-Larsson Syndrome/ or Gaucher Disease/ (8094) 180 (Lipidos* or ( storage adj3 disorder*) or ( adj3 lipidosis) or Sphingolipidoses or (glucocerebrosidase adj3 deficienc*) or (Sjogren adj3 Larsson) or Gaucher or Ichthyosis).mp. (14996) 181 "Classical Lissencephalies and Subcortical Band Heterotopias"/ (107) 182 (Lissencephal* or (Miller adj3 Dieker) or (agyria pachygyria adj3 band spectrum)).mp. (1397) 183 Infant/ and Osteopetrosis/ (443) 184 (Malignant Infantile adj3 Osteopetrosis).mp. (58) 185 alpha-Mannosidosis/ (236) 186 Mannosidosis/ (5) 187 limit 186 to yr="1985 - 2003" (1) 188 beta-Mannosidosis/ (9) 189 Mannosidase Deficiency Diseases/ (5) 190 limit 189 to yr="1984 - 2003" (1) 191 ((alpha or beta) adj3 Mannosidos*).mp. (361) 192 ((Marshall adj3 Smith) or (Greig* adj3 syndrome) or (Polysyndactyly adj3 cephalopolysyndactyly)).mp. (194) 193 (Meckel or Gruver or (Dysencephalia adj3 Splanchnocystica)).mp. (4100) 194 (Menkes or (copper transport adj3 disease) or ((steely or kinky) adj3 hair) or (dystrophy adj3 trichopolio) or trichopoliodystrophy).mp. (1425) 195 MERRF Syndrome/ (321) 196 (MERRF or ((myoclon* adj3 epilepsy) and ragged red fibers) or fukuhara).mp. (629) 197 Leukodystrophy, Metachromatic/ (1139) 198 ((Metachromic Leukodystroph* adj3 Juvenile) or ((arylsulfatase or sulfatid*) adj3 deficienc*) or (sulfatid* adj3 lipidosis)).mp. (235) 199 ((Methylcrotonyl-CoA Carboxylase adj3 Deficienc*) or 3 Methylcrotonygycinuria or (Methylcrotonygycinuria adj3 type 1)).mp. (87) 200 Methylmalonyl-CoA Mutase/df [Deficiency] (105) 201 ((Methylmalonyl CoA Mutase adj3 deficienc*) or (methylmalonic adj3 acidemia)).mp. (565) 202 Mevalonate Kinase Deficiency/ (135)

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203 ((Mevalonate Kinase adj3 Deficienc*) or mevalonicacidurias or (hyper igd adj3 syndrome*) or hyperimmunoglobulinemia d).mp. (366) 204 Mitochondrial Diseases/ (3856) 205 (((Mitochondrial adj3 (Disorder* or Disease*)) or (phosphorylation oxidative mitochondrial or electron transport chain or mitochondrial respiratory chain)) adj3 deficienc*).mp. (232) 206 MELAS Syndrome/ (1079) 207 ((MELAS adj3 Syndrome*) or ((mitochondrial myopathy adj3 encephalopathy) and lactic acidosis)).mp. (1377) 208 mitochondrial encephalomyopathies/ (939) 209 (Mitochondria* adj3 Encephalomyopath*).mp. (1683) 210 Mitochondrial Myopathies/ (1499) 211 ((Mitochondrial adj3 Myopath*) or (luft* adj3 disease*) or (megaconial adj3 myopath*)).mp. (2907) 212 ((Mowat adj3 Wilson) or ( adj3 mental) or (hirschsprung adj3 disease)).mp. (4771) 213 Mucolipidoses/ (925) 214 ((Mucolipidos* adj3 Type III) or (Pseudo Hurler adj3 Polydystrophy)).mp. (82) 215 (Mucolipidos* adj3 Type IV).mp. (140) 216 ((Mucolipidos* adj3 Type II) or (i cell adj3 Disease) or sialolipidos* or mucolipidos* or ( adj3 cherry red spot) or (glycoprotein neuraminidase adj3 deficienc*) or (inclusion cell adj3 disease)).mp. (1330) 217 I/ (1578) 218 (Mucopolysaccharidosis I or Hurler or Scheie or (alpha l iduronidase adj3 deficienc*) or gargoylism or lipochondrodystroph*).mp. (2034) 219 Mucopolysaccharidosis II/ (820) 220 ((Mucopolysaccharidosis adj3 (II or type 2)) or (Hunter adj3 syndrome) or (iduronate sulfatase adj3 deficienc*)).mp. (1004) 221 Mucopolysaccharidosis III/ (572) 222 ((Mucopolysaccharidosis adj3 (III or type 2)) or Sanfilippo* or San filippo* or ((n- acetylglucosamine-6-sulfatase or acetyl-coa:alpha-glucosaminide n-acetyltransferase or sulfamidase) adj3 deficienc*) or oligophrenia polydystrophic).mp. (781) 223 (((Mucopolysaccharidosis adj3 (IIIa or type 3a)) or (Heperan sulfate or sulfamidase)) adj3 deficienc*).mp. (23) 224 (((Mucopolysaccharidosis adj3 (IIIb or type 3b)) or (n acetyl alpha d glucosaminidase or naglu)) adj3 deficienc*).mp. (11) 225 Multiple Sulfatase Deficiency Disease/ (24) 226 SPHINGOLIPIDOSES/ (343) 227 limit 226 to yr="2000 - 2006" (63) 228 Sulfatases/ (3289) 229 limit 228 to yr="1973 - 2006" (2109) 230 ((Multiple Sulfatase adj3 Deficienc*) or (Austin adj3 Disease) or mucosulfatidosis or (juvenile adj3 sulfatidos*)).mp. (124) 231 Myoclonic Epilepsies, Progressive/ (298) 232 ((Myoclon* Epileps* adj3 Progressive) or (haw adj3 river) or encephalopathyadj3 biotin- responsive or (naito adj2 oyanagi) or (dentatorubral-pallidoluysian adj3 atrophy) or (myoclonus adj3 nephropathy) or (atypical adj3 inclusion-body) or (may adj2 white) or (dentatorubral pallidoluysian adj3 atrophy)).mp. (1755) 233 ((myotubular or x-linked centronuclear) adj3 myopath*).mp. (348) 234 Myopathies, Nemaline/ (408) 235 ((Rod adj3 Myopath*) or (nemaline adj3 myopath*)).mp. (749) 236 Neuroaxonal Dystrophies/cn [Congenital] (1)

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237 (Seitelberger*adj3 Disease or (Neuroaxonal Dystroph* adj3 Congenital) or (congenital adj3 neuroaxonal dystrophy)).mp. (1) 238 Neuronal Ceroid-Lipofuscinoses/ (1741) 239 ((Neuronal Ceroid Lipofuscinosis adj3 (Type 3 or juvenile)) or cerebroretinaladj3 degeneration* or (batten adj3 disease) or (vogt adj3 spielmeyer) or (spielmeyer adj3 sjogren) or (Janksy adj3 Bielschowsky) or (Kuf* adj3 Disease) or (Spielmeyer Vogt adj3 Sjogren Batten) or (amaurotic adj3 idocy)).mp. (852) 240 (Neuronal Ceroid Lipofuscinosis adj3 (Type 6 or late infantile)).mp. (255) 241 Niemann-Pick Disease, Type A/ (38) 242 ((Niemann Pick adj3 (Type A or classical)) or (neuronal cholesterol adj3 lipidos*) or (sphingomyelinas* adj3 deficienc*) or ( adj3 (lipidos* or storage disease))).mp. (319) 243 Niemann-Pick Disease, Type C/ (430) 244 (Niemann-Pick Disease adj3 (Type C or nova scotia*)).mp. (786) 245 Hyperglycinemia, Nonketotic/ (177) 246 ((Hyperglycin?emia* adj3 (Nonketotic or non ketotic)) or (glycine adj3 encephalopath*)).mp. (496) 247 Oculocerebrorenal Syndrome/ (359) 248 Olivopontocerebellar Atrophies/ (633) 249 ((Olivopontocerebellar adj3 (Atroph* or degeneration*)) or (olivo ponto cerebellar adj3 (Atroph* or degeneration*)) or (pontoolivocerebellar adj3 (Atroph* or degeneration*)) or (dejerine adj3 thomas) or (presenile adj3 ) or (Lowe* adj3 syndrome)).mp. (3366) 250 Sleep Apnea, Central/ (1015) 251 ((Ondine* adj3 curse) or (congenital adj3 central hypoventilation) or (primary alveolar adj3 hypoventilation) or (central sleep breathings adj3 disorder*)).mp. (629) 252 Ophthalmoplegia, Chronic Progressive External/ (488) 253 ((Ophthalmoplegia Chronic adj3 Progressive) or (ocular myopathy adj3 von graefe fuchs) or (dystrophy ocular adj3 muscular) or (ocular myopathy adj3 mitochondrial) or (graefe* adj3 disease)).mp. (505) 254 Pelizaeus-Merzbacher Disease/ (269) 255 "Diffuse Cerebral Sclerosis of Schilder"/ (2317) 256 limit 255 to yr="1966 - 1999" (1716) 257 / (43327) 258 limit 257 to yr="1966 - 1999" (17568) 259 ((Pelizaeus adj3 Merzbacher) or (leukodystrophy adj3 hypomyelinating 1) or (balo adj3 disease) or ((brain or cerebral) adj3 sclerosis) or (concentric adj3 periaxial encephalitis)).mp. (3896) 260 Peroxisomal Disorders/ (507) 261 ((Peroxisomal adj3 (Disorder* or disease)) or disfunction* or (adrenoleukodystrophy autosomal adj3 neonatal) or hyperpipecolatemia* or (acidemia* adj3 hyperpipecolic) or (neonatal adj3 adrenoleukodystroph*)).mp. (2589) 262 ((Pontocerebellar Hypoplasia adj3 (Type II or type 2)) or (volendam adj3 neurodegenerative)).mp. (21) 263 (3 Hydroxyisobutyric adj3 Aciduria).mp. (15) 264 Progeria/ (1274) 265 ((Hutchinson adj3 Gilford) or Gilford or progeria or premature senility).mp. (1739) 266 Propionic Acidemia/ (109) 267 ((Propionic* adj3 Acidemia*) or (propionyl coa carboxylase adj3 deficienc*) or (hyperglycinemia ketoacidosis adj3 leukopenia) or propionicaciduria* or (glycin?emia* adj3 ketotic)).mp. (492) 268 Pyruvate Carboxylase Deficiency Disease/ (139) 269 ((Pyruvate Carboxylase adj3 Deficienc*) or (ataxia adj3 lactic acidosis)).mp. (186) 270 Pyruvate Dehydrogenase Complex Deficiency Disease/ (366)

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271 (Pyruvate Dehydrogenase Complex adj3 Deficienc*).mp. (404) 272 Pyruvate Metabolism, Inborn Errors/ (54) 273 (Pyruvate Metabolism adj3 Inborn Errors).mp. (55) 274 Refsum Disease/ (625) 275 ((Refsum adj2 (Disease or syndrome)) or ((hereditary motor adj3 sensory neuropathy) and (type iv or type 4)) or (polyneuritiformis hemeralopia adj3 heredoataxia) or (phytanic acid oxidase adj3 (deficienc* or storage disease)) or (heredopathia adj3 (atactica polyneuritiformis or atacticapolyneuritiformis))).mp. (785) 276 Rubinstein-Taybi Syndrome/ (433) 277 ((Rubinstein adj3 Taybi) or (broad thumb adj3 hallux) or (rubinstein adj3 syndrome)).mp. (640) 278 / (395) 279 ((Sandhoff adj3 Disease) or ((total hexosaminidase or beta hexosaminidase beta subunit) adj3 deficienc*) or (( adj5 deficiency) and (a and b))).mp. (512) 280 "Malformations of Cortical Development"/ (947) 281 /ab [Abnormalities] (1727) 282 limit 281 to yr="1995 - 2007" (1073) 283 ((Schizencephaly adj2 (Type 2 or type II)) or (cerebral cortical adj3 dysplasia*) or (cortical development adj3 malformation*) or (schizocephal* adj3 (Type 2 or type II))).mp. (1514) 284 Short Rib-Polydactyly Syndrome/ (126) 285 ((Short Rib adj3 Polydactyly) or (polydactyly adj3 neonatal chondrodystrophy) or (saldino adj3 noonan) or (majewski adj3 syndrome)).mp. (234) 286 Sialic Acid Storage Disease/ (64) 287 ((Sialic Acid adj3 Storage Disease) or (sialuria* adj3 (finnish type or french type))).mp. (121) 288 "Spinal Muscular Atrophies of Childhood"/ (1031) 289 (( adj3 (Type 1 or type i)) or (Infantile adj3 (spinal Muscular Atrophy or muscular atrophy)) or (Werdnig adj3 Hoffmann) or (hereditary progressive spinal adj3 muscular atrophy)).mp. (585) 290 Smith-Lemli-Opitz Syndrome/ (519) 291 Abnormalities, Multiple/ (38849) 292 limit 291 to yr="1967 - 1995" (19000) 293 (((Smith Lemli Opitz or reductase deficiency 7-dehydrocholesterol or rutledge friedman harrod or lethal acrodysgenital or rutledge lethal multiple congenital anomaly) adj2 Syndrome) or (hyperotosis corticalis adj3 generalisata familiaris)).mp. (785) 294 "NAV1.1 Voltage-Gated Sodium Channel"/ (597) 295 ((Voltage-Gated Sodium Channel adj3 "NAV1.1") or (Sodium channel voltage-gated adj3 (alpha subunit and type 1))).mp. (602) 296 Sphingolipidoses/ (343) 297 (Sphingolipidoses or ( storage adj3 diseases)).mp. (458) 298 Subacute Sclerosing Panencephalitis/ (2223) 299 ((Subacute Sclerosing adj3 Panencephalitis) or ((Dawson or Bogaert*) adj3 Disease) or (sclerosing leukoencephalitid* adj3 subacute) or (inclusion body encephalitis adj3 measles)).mp. (2649) 300 ((Systemic adj3 Juvenile Xanthogranulomato*) or JXK).mp. (4) 301 / (5188) 302 ((Tuberous adj3 Sclerosis) or (bourneville adj3 pringle*) or (bourneville adj3 (syndrome or phacomatosis or adenoma sebace*))).mp. (7889) 303 Unverricht-Lundborg Syndrome/ (118) 304 ((Unverricht adj3 Lundborg) or (mediterranean adj3 myoclonic epileps*) or (unverricht adj3 disease*) or (myoclon* adj3 baltic) or (epilepsy progressive adj3 myoclonic 1a)).mp. (297) 305 von Hippel-Lindau Disease/ (2293) 306 ((von Hippel adj3 Lindau) or (lindau adj3 disease*) or (retina* adj5 angiomatos*) or (cerebelloretinal adj3 hemangioblastomatosis)).mp. (5235)

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307 Spasms, Infantile/ (3025) 308 ((West adj3 Syndrome) or ((juvenile or infantile) adj3 spasm*)).mp. (4116) 309 Wolman Disease/ (166) 310 ((Wolman* adj3 (Disease or xanthomatos*)) or (familial adj3 xanthomatos*) or (acid lipase adj3 deficienc*)).mp. (413) 311 Adrenoleukodystrophy/ (1546) 312 ((X linked adj3 Adrenoleukodystroph*) or (Addison adj3 Schilder) or (Addison adj3 disease)).mp. (5274) 313 Xeroderma Pigmentosum/ (3302) 314 (((Xeroderma or atrophoderma) adj3 Pigmentos*) or (kaposi* adj3 disease) or (melanosis lenticularis adj3 progressiva)).mp. (6076) 315 Zellweger Syndrome/ (531) 316 (((Zellweger adj3 (Syndrome or disease)) or (cerebro hepato renal or cerebrohepatorenal)) adj3 syndrome*).mp. (942) 317 Anencephaly/ (2970) 318 (Anencephal* or (congenital absence adj3 brain)).mp. (4021) 319 Lipodystrophy, Congenital Generalized/ (126) 320 DIABETES MELLITUS, LIPOATROPHIC/ (194) 321 limit 320 to yr="2005 - 2006" (30) 322 Lipodystrophy/ (3042) 323 limit 322 to yr="1968 - 2006" (1880) 324 ((LIPODYSTROPH* adj3 CONGENITAL GENERALIZED) or (BERARDINELLI adj3 LIPODYSTROPH*) or (SEIP adj3 SYNDROME) or (LIPODYSTROPH* adj3 ACROMEGALOID GIGANTISM) or (LIPOATROPH* DIABETES adj3 CONGENITAL)).mp. (344) 325 (Bisalbuminemia or (Hartnup adj3 disorder)).mp. (167) 326 / (264) 327 ((canavan adj3 (disease or syndrome)) or ( adj3 deficiency) or (bogaert adj3 bertrand) or (spongy degeneration and white matter and infancy) or (spongy disease adj3 central nervous system) or (spongiform adj3 leukodystrophy)).mp. (400) 328 ((MECP2 Duplication adj3 Syndrome) or (trisomy adj3 Xq28) or (Xq28 duplication adj3 syndrome)).mp. (60) 329 Methyl-CpG-Binding Protein 2/ge [Genetics] (940) 330 Cytomegalovirus Infections/cn [Congenital] (1480) 331 ((Congenital Cytomegalovirus adj3 (Infection or inclusion)) or (CMV adj3 (disease or syndrome))).mp. (3730) 332 Toxoplasmosis, Congenital/ (2530) 333 ((Congenital adj3 Toxoplasm*) or (toxoplasm* adj3 (fetal or newborn))).mp. (3029) 334 ((multiple Pterygium adj3 Syndrome) and (leathal or deadly or terminal)).mp. (1) 335 (Galactosialidosis or (Goldberg adj3 syndrome) or (Neuraminidase deficiency adj3 beta galactosidase deficiency) or (sialidosis adj3 galacto)).mp. (286) 336 Hyperglycinemia, Nonketotic/ (177) 337 ((Glycine adj3 Encephalopath*) or ((Non ketotic or nonketotic) adj3 hyperglycinemia)).mp. (427)

338 Holoprosencephaly/ (1046) 339 (Holoprosencephal* or arhinencephal*).mp. (2002) 340 ((Hyperleucine adj6 isoleucineaemia) or (Hyper Leucine adj6 isoleucine anemia)).mp. (0) 341 Striatonigral Degeneration/ (65) 342 ((Infantile adj3 Bilateral Striatal Necrosis) or (Striatonigral adj3 (Degeneration* or atroph*)) or (nigrostriatal adj3 degeneration)).mp. (1128)

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343 Jacobsen Distal 11q Deletion Syndrome/ (50) 344 ((paris adj3 trousseau) or (11q23 adj3 (distal deletion or terminal deletion)) or (chromosome 11q adj3 deletion) or (jacobsen adj3 (thrombocytop?enia or syndrome))).mp. (185) 345 (((Molybdenum cofactor or Encephalopathy or Aldehyde Oxidase Combined) adj3 deficiency) or (Sulfite Oxidase adj3 Xanthine Dehydrogenase)).mp. (290) 346 Mucopolysaccharidosis VI/ (413) 347 ((Mucopolysaccharidosis adj3 (VI or type 6)) or (Mucopolysaccharidosis adj3 Aicardi) or (callosal adj3 agenesis) or (ocular adj3 abnormalit*) or (Maroteaux adj3 Lamy) or ((arylsulfatase b or arsb) adj3 deficienc*) or (dwarfism adj3 polydystrophic)).mp. (2887) 348 Mucopolysaccharidosis VII/ (251) 349 (Mucopolysaccharidosis VII or Mucopolysaccharidosis 7 or ((Beta glucuronidase or gusb) adj3 deficienc*) or (sly adj3 (disease or syndrome))).mp. (352) 350 Ornithine Carbamoyltransferase Deficiency Disease/ (844) 351 ((Ornithine Carbamoyltransferase or Ornithine Carbamoyl transferase) adj3 Deficienc*).mp. (865) 352 Lissencephaly/ (165) 353 (Pachygyria* or Lissencephal* or macrogyria* or agyria* or ((large or broad) adj3 gyri)).mp. (1615) 354 Sulfite Oxidase/df (33) 355 Sulfatidosis/ (0) 356 Sulfocysteinuria.mp. (1) 357 (Chromosome Deletion/ and Chromosomes, Human, Pair 79/) or (Chromosome Deletion/ and Chromosomes, Human, Pair 23/) or (Chromosome Deletion/ and Chromosomes, Human, Pair 24/) or (Chromosome Deletion/ and Chromosomes, Human, Pair 46/) (0) 358 (chromosome adj3 (deletion 79 or deletion 23q or deletion q24 or Deletion 46xx)).mp. (0) 359 Osteochondrodysplasias/mo (19) 360 ((Wolcott adj3 Rallison) or (Schwartz adj3 jampel) or (Myotonia adj3 chondrodystrophic) or (Stuve adj3 Wiedemann) or (osteochondrodysplasia* adj3 lethal) or Schimke immuno-osseous dysplasia or (lethal adj3 dyssegmental dysplasia) or Achondrogenesis I or Achondrogesis II or (Thanatophoric adj3 dwarfism)).mp. (547) 361 ((Pena adj3 Shokeir) or (Cerebrooculofacioskeletal adj3 syndrome) or (Arthrogryposis multiplex adj3 congenita) or COFS Syndrome).mp. (721) 362 Trisomy/ and Chromosomes, Human, Pair 10/ (183) 363 (Mosaicism adj3 Trisomy 10).mp. (9) 364 1 or 2 or 3 or 4 or 5 or 6 or 7 or 8 or 9 or 10 or 11 or 12 or 13 or 14 or 15 or 16 or 17 or 18 or 19 or 20 or 21 or 22 or 23 or 24 or 25 or 26 or 27 or 28 or 29 or 30 or 31 or 32 or 33 or 34 or 35 or 36 or 37 or 38 or 39 or 40 or 41 or 42 or 43 or 44 or 45 or 46 or 47 or 48 or 49 or 50 or 51 or 52 or 53 or 54 or 55 or 56 or 58 or 60 or 61 or 62 or 63 or 64 or 65 or 66 or 67 or 68 or 69 or 70 or 71 or 72 or 73 or 74 or 75 or 76 or 78 or 80 or 81 or 82 or 83 or 84 or 85 or 86 or 87 or 88 or 90 or 92 or 93 or 94 or 95 or 96 or 97 or 98 or 99 or 100 or 101 or 102 or 103 or 104 or 105 or 106 or 107 or 108 or 109 or 110 or 111 or 112 or 113 or 114 or 115 or 116 or 117 or 118 or 119 or 120 or 121 or 122 or 123 or 124 or 125 or 126 or 127 or 128 or 129 or 130 or 131 or 132 or 133 or 134 or 135 or 136 or 137 or 138 or 139 or 140 or 141 or 142 or 143 or 144 or 145 or 146 or 147 or 148 or 149 or 150 or 151 or 152 or 153 or 154 or 155 or 156 or 157 or 158 or 159 or 160 or 161 or 162 or 163 or 164 or 165 or 166 or 167 or 168 or 169 or 170 or 171 or 173 or 174 or 175 or 176 or 177 or 178 or 179 or 180 or 181 or 182 or 183 or 184 or 185 or 187 or 188 or 190 or 191 or 192 or 193 or 194 or 195 or 196 or 197 or 198 or 199 or 200 or 201 or 202 or 203 or 204 or 205 or 206 or 207 or 208 or 209 or 210 or 211 or 212 or 213 or 214 or 215 or 216 or 217 or 218 or 219 or 220 or 221 or 222 or 223 or 224 or 225 or 227 or 229 or 230 or 231 or 232 or 233 or 234 or 235 or 236 or 237 or 238 or 239 or 240 or 241 or 242 or 243 or 244 or 245 or 246 or 247 or 248 or 249 or 250 or 251 or 252 or 253 or 254 or 256 or 258 or 259 or 260 or 261 or 262 or 263 or 264 or 265 or 266 or 267 or 268 or 269 or 270 or 271 or 272 or 273 or 274 or 275 or 276 or 277 or 278 or 279 or 280 or 282 or 283 or 284 or

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285 or 286 or 287 or 288 or 289 or 290 or 292 or 293 or 294 or 295 or 296 or 297 or 298 or 299 or 300 or 301 or 302 or 303 or 304 or 305 or 306 or 307 or 308 or 309 or 310 or 311 or 312 or 313 or 314 or 315 or 316 or 317 or 318 or 319 or 321 or 323 or 324 or 325 or 326 or 327 or 328 or 329 or 330 or 331 or 332 or 333 or 334 or 335 or 336 or 337 or 338 or 339 or 340 or 341 or 342 or 343 or 344 or 345 or 346 or 347 or 348 or 349 or 350 or 351 or 352 or 353 or 354 or 355 or 356 or 357 or 358 or 359 or 360 or 361 or 362 or 363 (286895)

Symptom Management Search

365 ((practice adj3 guideline) or (health care adj3 quality) or (clinical adj3 handover) or (clinical adj3 (pathway or protocol)) or (consensus adj3 development) or (good adj3 clinical practice) or (nursing adj3 care plan) or (nursing adj3 protocol) or (refusal adj3 participate)).mp. (167722) 366 ((quality adj3 life) or (international classification adj3 functioning) or (quality adjusted adj3 life year)).mp. (236616) 367 (Management adj3 (manifestation* or comorbidity or co-morbidity)).mp. (1231) 368 Symptom Assessment/ (1307) 369 (Symptom* adj3 (assessment or evaluation or screening tool* or cluster* or control or management or intervention*)).mp. (32428) 370 Treatment Outcome/ or Treatment Failure/ (746573) 371 (treatment adj3 (outcome* or failure* or efficacy or effectiveness)).mp. (837759) 372 ((medical adj3 futility) or (outcome adj3 (management or assessment or measurement)) or (therapy adj3 outcome)).mp. (104073) 373 ((disease free adj3 (interval or period)) or (outcome adj3 assessment)).mp. (88680) 374 ((therapy adj3 failure) or (ineffective adj3 drug)).mp. (9653) 375 (clinical adj3 (effective* or efficacy or outcome*)).mp. (175344) 376 (tool* or trial* or guideline* or protocol*).mp. (2334802) 377 (Clinical rating adj3 (instrument* or tool or scale)).mp. (261) 378 Patient Acuity/ (453) 379 (patient adj3 acuit*).mp. (1489) 380 Severity of Illness Index/ (188269) 381 (illness index adj3 severit*).mp. (188317) 382 Karnofsky Performance Status/ (1959) 383 (karnofsky adj3 (index or scale or score)).mp. (2897) 384 Sickness Impact Profile/ (6447) 385 (sickness adj3 impact profile*).mp. (7163) 386 Therapeutics/ (8215) 387 (Therapeutic* or treatment* or intervention* or therapy).mp. (5585786) 388 Acoustic Stimulation/ (37593) 389 (stimulation adj3 (acoustic or auditory)).mp. (39449) 390 (((acoustic or audio) adj3 stimulus) or (sound adj3 stimulation)).mp. (1630) 391 airway management/ (1574) 392 (airway adj3 (control or management or extubation*)).mp. (8389) 393 ((breathing adj3 (control or regulation)) or (control adj3 (respiration or respiratory)) or (respiration adj3 regulation)).mp. (8556) 394 airway extubation/ or intubation, intratracheal/ (31519) 395 ((extubation* adj3 endotracheal) or (intubation adj3 (intratraceal or tracheal or endotracheal or orotracheal or trachea))).mp. (14484) 396 laryngeal masks/ (4508) 397 (laryngeal adj3 mask*).mp. (5641) 398 respiration, artificial/ (41225)

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399 (artificial adj3 respiration*).mp. (41874) 400 (mechanical adj3 (ventilation* or respiration)).mp. (30508) 401 (controlled adj3 (respiration or ventilation)).mp. (3566) 402 high-frequency ventilation/ or high-frequency jet ventilation/ or liquid ventilation/ or noninvasive ventilation/ or one-lung ventilation/ (3684) 403 (ventilation* adj3 (high frequency or proportional assist or liquid or fluorocarbon or non invasive or noninvasive or one lung or one-lung or single lung)).mp. (11023) 404 interactive ventilatory support/ (127) 405 (interactive adj3 ventilatory support).mp. (128) 406 (neurally adjusted adj3 ventilatory assist).mp. (163) 407 (lung separation adj3 technique*).mp. (8) 408 positive-pressure respiration/ or continuous positive airway pressure/ or intermittent positive- pressure breathing/ (20740) 409 (positive pressure adj3 (respiration* or ventilation* or breathing)).mp. (19901) 410 (hyperbaric adj3 (respiration or ventilation)).mp. (42) 411 (end expiratory adj3 positive pressure*).mp. (27) 412 (airway pressure adj3 release ventilation).mp. (191) 413 (aprv adj3 ventilation mode).mp. (1) 414 intermittent positive-pressure ventilation/ (2108) 415 ((intermittent or inspiratory) adj3 positive pressure).mp. (3824) 416 ventilator weaning/ (3168) 417 ((ventilator or respirator) adj3 weaning).mp. (3561) 418 Tracheostomy/ (5997) 419 tracheostom*.mp. (13073) 420 Apitherapy/ (90) 421 (apitherap* or ((bee sting or bee venom or honey or propolis) adj3 therap*)).mp. (331) 422 balneology/ (5670) 423 (balneology or balneotherapy or balneo therapy or bath therapy).mp. (6027) 424 ammotherapy/ (32) 425 (((artificial or induced or treatment) adj3 hyperthermi*) or ((dry heat or fever or heat or infra red or infrared) adj3 therap*) or pyretotherapy or thermotherapy ammotherapy).mp. (19534) 426 baths/ (4847) 427 (sand bath* or sandbath* or bath).mp. (25802) 428 mud therapy/ (1311) 429 ((mud adj3 (bath* or pack* or application)) or fangotherapy or peat therapy or peloid therapy or pelotherapy).mp. (448) 430 steam bath/ (660) 431 ((bath adj3 (finnish or steam)) or sauna or sweat lodge).mp. (970) 432 Bed Rest/ (3642) 433 (bed rest* or bedrest*).mp. (6934) 434 Behavior control/ (1539) 435 (behavior?r adj3 (control or manipulation*)).mp. (0) 436 (behavior?r adj3 (control or manipulation*)).mp. (0) 437 Restraint, physical/ (10651) 438 ((physical adj3 restraint*) or (physical adj3 immobilization)).mp. (11486) 439 fecal microbiota transplantation/ (62) 440 (Fecal adj3 transplantation*).mp. (430) 441 (donor adj3 feces infusion*).mp. (2) 442 (intestinal adj3 microbiota transfer*).mp. (0) 443 Immunotherapy/ (32769) 444 (immunotherap* or (biologic* response adj3 modifier therapy) or ((immune or immunoglobulin or

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immunological) adj3 therapy)).mp. (80805) 445 Chronotherapy/ or Drug Chronotherapy.mp. [mp=title, abstract, original title, name of substance word, subject heading word, keyword heading word, protocol supplementary concept word, rare disease supplementary concept word, unique identifier] (822) 446 (chronotherap* or (time dependent adj3 drug therapy)).mp. (1418) 447 Climatotherapy/ (532) 448 (((climate or climatic) adj3 therapy) or climatotherapy or thalassotherapy or climatic treatment).mp. (955) 449 Clinical Protocols/ or Antineoplastic Protocols/ or Antineoplastic Combined Chemotherapy Protocols/ (138009) 450 ((clinical or treatment or antineoplastic or cancer) adj3 protocol*).mp. (160721) 451 (((anticancer or antineoplastic) adj3 drug combinations) or ((combined antineoplastic adj3 agent*) or protocol* or regimens) or (cancer adj3 "chemotherapy protocol")).mp. (501755) 452 combined modality therapy/ (151424) 453 ((combined modality adj3 therap*) or (multimodal adj3 (treatment* or therapy)) or (multiple modality adj3 (therapy or treatment))).mp. (155297) 454 chemoradiotherapy/ or chemoradiotherapy, adjuvant/ or chemotherapy, adjuvant/ (38733) 455 (chemoradiotherap* or radiohemotherap* or chemoradiation).mp. (20364) 456 ((adjuvant adj3 (chemotherapy* or drug therapy or chemoradiotherap* or radiochemotherap* or radiotherapy*)) or (neoadjuvant adj3 chemotherapy)).mp. (67345) 457 electroacupuncture/ (2880) 458 (elctroacupuncture or electric acupunture).mp. (1) 459 photochemotherapy/ (14211) 460 (photochemotherap* or (photodynamic adj3 therap*) or (hematoporphyrin adj3 photoradiation)).mp. (19494) 461 neoadjuvant therapy/ (13369) 462 (neoadjuvant adj3 (therap* or treatment*)).mp. (17839) 463 radioimmunotherapy/ or radiotherapy, adjuvant/ (21420) 464 (radioimmunotherap* or immunoradiotherap* or (radioimmuno adj3 therapy) or (adjuvant adj3 radiotherapy)).mp. (27061) 465 complementary therapies/ (14930) 466 ((alternative adj3 (medicine or therap*)) or (complementary adj3 (medicine or therap*))).mp. (36416) 467 acupuncture therapy/ or acupuncture analgesia/ or acupuncture, ear/ or moxibustion/ (15268) 468 (acupuncture* or mox?bustion).mp. (22356) 469 auriculotherapy/ (30) 470 auriculotherap*.mp. (90) 471 diffuse noxious inhibitory control/ (13) 472 (diffuse noxious adj3 inhibitory control).mp. (105) 473 holistic health/ (7258) 474 ((holistic adj3 (health or medicine or therap*)) or (wholistic adj3 (health or medicine or therap*))).mp. (8135) 475 bioresonance therapy/ (0) 476 ((bioresonance or bio resonance or mora) adj3 therap*).mp. (29) 477 homeopathy/ (4330) 478 (hom#eopathy or homeopathia).mp. (381) 479 horticultural therapy/ (26) 480 (horticultural adj3 therap).mp. (0) 481 mesotherapy/ (51) 482 mesotherapy.mp. (191)

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483 mind-body therapies/ (701) 484 (mind body adj3 (medicine or therap*)).mp. (1075) 485 aromatherapy/ (603) 486 ((aroma adj3 therap*) or aromatherap*).mp. (996) 487 ((aroma adj3 therap*) or aromatherap*).mp. (996) 488 biofeedback, psychology/ or neurofeedback/ (6943) 489 ((psychology or physiological or alpha or brainwave or elctroencephalography) adj3 biofeedback*).mp. (6558) 490 breathing exercises/ (2929) 491 (((breathing or respiratory) adj3 exercise*) or (respiratory adj3 muscle training) or ((breathing or chest physical or respiration) adj3 therapy) or chest physiotherapy).mp. (6254) 492 hypnosis/ (8494) 493 (hypnos#s* or hypnotherapy* or mesmerism).mp. (11281) 494 autogenic training/ (1024) 495 ((autogenic adj3 training) or (progressive adj3 muscle relaxation)).mp. (1535) 496 "imagery (psychotherapy)"/ (1348) 497 (imagery or (directed reverie adj3 therap*)).mp. (11123) 498 laughter therapy/ (142) 499 (laughter adj3 therapy).mp. (159) 500 meditation/ (1862) 501 meditation.mp. (3800) 502 relaxation therapy/ (5928) 503 (relaxation adj3 (technique* or therapy or training or method or technics)).mp. (8462) 504 yoga/ (1827) 505 yoga.mp. (3134) 506 musculoskeletal manipulations/ (1120) 507 ((musculoskeletal adj3 manipulation*) or ((manipulation or manipulative or manual) adj3 therap*)).mp. (5021) 508 kinesiology, applied/ (241) 509 (applied adj3 kinesiology).mp. (294) 510 manipulation, chiropractic/ or manipulation, osteopathic/ (1560) 511 (chiropractic or cheirotherapy or chiropraxi* or chirotherapy or osteopathic or osteopathy).mp. (11158)

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512 therapy, soft tissue/ or acupressure/ or massage/ (5742) 513 ((soft tissue adj3 therapy) or massage or bodywork* or reflexology or rolfing or acupressure or (zone adj3 therap*)).mp. (13802) 514 naturopathy/ (903) 515 naturopath*.mp. (1263) 516 organotherapy/ (167) 517 organotherap*.mp. (202) 518 phytotherapy/ (32590) 519 (phytotherapy or ((herb or herbal) adj3 therapy)).mp. (33365) 520 reflexotherapy/ (452) 521 (reflex adj3 therapy).mp. (210) 522 sensory art therapies/ or art therapy/ (1268) 523 sensory art therapies/ or art therapy/ (1268) 524 (art adj3 (therap* or treatment)).mp. (11453) 525 color therapy/ (74) 526 (chrom#totherapy or colo#r therap*).mp. (9) 527 dance therapy/ or music therapy/ or play therapy/ (3859) 528 (((dance or music or play or sandplay) adj3 therap*) or jocotherapy).mp. (5906) 529 "continuity of patient care"/ (15790) 530 (care adj3 (continuity or continuum)).mp. (21027) 531 patient discharge/ (21738) 532 ((patient or planning* or hospital) adj3 discharge).mp. (46697) 533 patient handoff/ (482) 534 (((clinical or patient or nursing) adj3 handoff*) or ((clinical or patient or nursing) adj3 hand off*) or ((clinical or patient or nursing) adj3 handover*) or ((clinical or patient or nursing) adj3 hand over*)).mp. (925) 535 patient transfer/ (6360) 536 ((patient adj3 (transfer* or transition* or turfing* or transport or transportation)) or (care adj3 transition*)).mp. (14755) 537 transition to adult care/ (515) 538 ((transition or pe#diatric or transfer* or hand over or handoff) adj3 adult care).mp. (697) 539 transitional care/ (43) 540 (transitional adj3 care*).mp. (839)

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541 Cryotherapy/ or Hypothermia, Induced/ (22312) 542 (((cold or cryogenic) adj3 therap*) or cryotherap* or ((cold or hypothermal) adj3 bath) or cryothermy or cryotreatment).mp. (8943) 543 ((induced or therapeutic) adj3 hypothermia).mp. (22010) 544 Directly Observed Therapy/ (1374) 545 (directly adj3 observed therapy).mp. (1941) 546 Drainage/ or Drainage, Postural/ or Negative-Pressure Wound Therapy/ or Paracentesis/ (40432) 547 (drainage or (vacuum assisted adj3 closure*) or (negative pressure adj3 (dressing* or therap*))).mp. (93557) 548 ((aspiration adj3 puncture) or centes#s).mp. (665) 549 Drug Therapy/ (28831) 550 ((drug adj3 (therap* or treatment)) or chemotherap* or pharmacotherap* or ((medicament or medicinal or orthomolecular or pharmaceutical or phadrarmaco or pharmacological) adj3 therapy) or ((medicament or medicinal or orthomolecular or pharmaceutical or pharmaco or pharmacological) adj3 treatment) or ).mp. (815419) 551 / (1239) 552 (chelation adj3 therap*).mp. (2967) 553 Chemoprevention/ or Antibiotic Prophylaxis/ (15932) 554 (chemoprevention or chemoprophylaxis or ((tuberculosis or tuberculous) adj3 chemoprophylasis) or (antibiotic adj3 (premedication* or prophylaxis))).mp. (32816) 555 Consolidation Chemotherapy/ (243) 556 (consolidation adj3 chemotherap*).mp. (1384) 557 drug administration routes/ or administration, inhalation/ (30898) 558 ((aerosol adj3 therapy) or (inhalat* adj3 (drugs or dose or administration or therapy or treatment)) or inhalational or vapo#r therapy).mp. (44654) 559 administration, intravenous/ or infusions, intravenous/ or injections, intravenous/ (130749) 560 ((intravenous adj3 (injection* or administration* of infusion* or dose or drip or therapy or medication or transfuction)) or (IV adj3 (administration or delivery or injection or therapy or infusion))).mp. (150453) 561 ((drip or intravenous or intracerebroventricular or intraventricular or spinal or subcutaneous) adj3 infusion*).mp. (79770) 562 administration, oral/ or administration, buccal/ or administration, sublingual/ or administration, topical/ or administration, cutaneous/ or administration, intravaginal/ or administration, intravesical/ or administration, mucosal/ or administration, intranasal/ or administration, rectal/ or administration, ophthalmic/ (191712) 563 ((oral or buccal or sublingual or "sub lingual" or ocular or ophthalmic or mucosal or transmucosal or bladder drug or intravesical or intra vesical or intravaginal or vaginal or intra vaginal or

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cutaneous or dermal or intradermal or percutaneous or transcutaneous or transdermal or skin drug or topical or topic or intranasal or nasal or anal or rectal or subcutaneous fluid) adj3 administration*).mp. (235491) 564 ((transmucosal adj3 routing*) or (mucosal adj3 (delivery or dosage or dose or injection or therapy or treatment)) or (transmucosal adj3 (delievery or dosage))).mp. (2154) 565 ((bladder or injection* or intravesical or drug) adj3 instillation*).mp. (3575) 566 (vaginal adj3 instillation*).mp. (38) 567 chemotherapy, cancer, regional perfusion/ (3381) 568 (cancer chemotherapy adj3 (regional perfusion or isolation perfusion)).mp. (10) 569 infusions, parenteral/ or infusions, intra-arterial/ or infusions, intralesional/ or infusions, intraosseous/ or infusions, intraventricular/ or infusions, spinal/ or infusions, subcutaneous/ or hypodermoclysis/ (36662) 570 ((intra abdominal or intraperitoneal or parenteral or peritoneal or intra arterial or intraarterial or regional aterial or intralesional or intra osseous or intraosseous) adj3 infusion*).mp. (38419) 571 ((subcutaneous adj3 hydration) or hypodermoclysis).mp. (201) 572 (cheek injection or (cheek adj3 drug delivery) or (buccal adj3 (dosage or dose or injection))).mp. (112) 573 ((topic adj3 (application or medication or therapy or treatment)) or topical).mp. (93946) 574 ((intravaginal adj3 (dosage or dose or drug deliver or injection or suppository or tablet or therapy or treatment)) or (vaginal adj3 (administration or dosage or dose or application or delivery or infusion or injection or suppository))).mp. (12349) 575 ((intra vesical adj3 instillation) or (intravesical adj3 (administration or application or delivery or dose or injection or instillation or medication or therapy or treatment))).mp. (4910) 576 injections/ or injections, intra-arterial/ or injections, intra-articular/ or viscosupplementation/ or injections, intralesional/ or injections, intralymphatic/ or injections, intramuscular/ or injections, intraocular/ or intravitreal injections/ or injections, intraperitoneal/ or injections, intraventricular/ or injections, spinal/ or injections, epidural/ or blood patch, epidural/ or injections, subcutaneous/ or injections, intradermal/ or injections, jet/ or biolistics/ or injection, intratympanic/ or microinjections/ (192911) 577 (injection* or injectable* or viscocupplementation* or microinjection* or epidural or biolistic*).mp. (694443) 578 ( gun adj3 technique).mp. (13) 579 (partical mediated or ((epidermal or extradural) adj3 injection*)).mp. (179) 580 ((transtympanic adj3 administration*) or (intratypanic adj3 injection*)).mp. (39) 581 (phonophores#s or iontophores#s).mp. (8860) 582 Drug Administration Schedule/ (89362) 583 (drug schedule* adj3 administration).mp. (1)

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584 Administration, Metronomic/ (166) 585 (metronomic adj3 (administration* or therap*)).mp. (316) 586 Pulse Therapy, Drug/ (1369) 587 (drug pulse adj3 therap*).mp. (3) 588 Drug Delivery System/ (42162) 589 (drug delivery adj3 system*).mp. (50445) 590 (drug adj3 targeting*).mp. (4688) 591 Delayed-Action Preparations/ (30225) 592 ((drug release or delayed action or depot or sustained release or timed release or prolonged action) adj3 preparation*).mp. (30884) 593 Drug carriers/ (28423) 594 (drug adj3 carrier*).mp. (32083) 595 / (3591) 596 ((dendritic adj3 compound) or * or dendrons or (dendritic adj3 polymer*)).mp. (6943) 597 Nanocapsules/ or Nanoconjugates/ (2704) 598 (nanocapsule or nanoconjugate).mp. (537) 599 Insulin Infusion Systems/ (4184) 600 ((artificial endocrine adj3 pancreas) or (artificial beta adj3 cell*) or (insulin infusion adj3 system*) or (programmable implantable adj3 insulin infusion)).mp. (4477) 601 Pharmaceutical Vehicles/ (4540) 602 (pharmaceutical adj3 vehicle*).mp. (4591) 603 (drug adj3 prescribing*).mp. (1776) 604 Drug substitution/ (1779) 605 ((drug adj3 (substitution* or switching*)) or ((generic or therapeutic) adj3 subsititution*)).mp. (2594) 606 "Off-label use"/ (1494) 607 ((unlabeled adj3 indication*) or (off label adj3 (prescribing* or use* or prescribing))).mp. (3883) 608 Drug therapy, Combination/ (146283) 609 ((combination adj3 (drug therapy or chemotherapy* or medication or pharmacotherapy)) or (drug adj3 (polytherap* or combinations)) or polychemotherap* or (combination drug adj3 (regimen or treatment)) or (combined drug adj3 (regimen or therapy or treatment)) or (multi drug adj3 (regimen or therapy)) or (multidrug adj3 (regimen or therapy)) or (multiple drug adj3 (regimen or therapy or treatment))).mp. (234598) 610 Antiretroviral Therapy, Highly Active/ (18158)

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611 (antiretroviral therapy adj3 highly active).mp. (21863) 612 enema/ (6710) 613 (enema* or clysma or clyster or enteroclysis).mp. (12376) 614 Drug Therapy, Computer-Assisted/ (1535) 615 (computer assisted adj3 drug therap*).mp. (1540) 616 Electrochemotherapy/ (378) 617 (electrochemotherap* or (electropermeablisation adj3 therap*)).mp. (664) 618 instillation, drug/ or iontophoresis/ (8407) 619 ((instillation adj3 drug) or iontophoresis).mp. (9973) 620 phonophoresis/ (280) 621 (phonophoresis or ultrasound or ultrasonic or sonication or sonification or ultra sound or ultrashell).mp. (218760) 622 Therapy/ or Enzyme Replacement Therapy/ (1257) 623 ((enzyme adj3 (therap* or suppletion)) or enzymotherapy).mp. (6051) 624 Fluid Therapy/ (16705) 625 ((fluid or oral rehydration) adj3 therap*).mp. (19349) 626 Hypodermoclysis/ (98) 627 ((subcutaneous adj3 (fluid administration or hydration)) or hypodermoclys#s).mp. (212) 628 Home Infusion Therapy/ (637) 629 ((home infusion or outpatient infusion) adj3 therap*).mp. (720) 630 Hormone Replacement Therapy/ (8274) 631 (hormone adj3 (replacement or substitution)).mp. (20284) 632 Estrogen Replacement Therapy/ (14316) 633 (#estrogen adj3 (replacement* or administration or substitution or therapy)).mp. (1079) 634 Induction Chemotherapy/ (1217) 635 Maintenance Chemotherapy/ (753) 636 ((induction or maintenance) adj3 chemotherap*).mp. (9564) 637 Medication Reconciliation/ (512) 638 ((medication adj3 reconciliation) or ((drug or medication) adj3 management)).mp. (7731) 639 Near Miss, Healthcare/ (9) 640 (healthcare adj3 (near miss* or close call*)).mp. (12) 641 Molecular Targeted Therapy/ (14405)

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642 (target* adj3 therap*).mp. (132449) 643 Orthomolecular Therapy/ (194) 644 ((megavitamin or orthomolecular) adj3 therap*).mp. (240) 645 Photochemotherapy/ (14211) 646 (photochemotherap* or chemophototherapy or (photodynamic adj3 therap*) or (hematoporphyrin adj3 photoradiation)).mp. (19495) 647 Pleurodesis/ (1196) 648 Pleurodesis.mp. (2318) 649 Polypharmacy/ or Deprescriptions/ (2929) 650 (polypharmacy or poly pharmacy or polypragmas* or deprescribing or deprescription*).mp. (5911) 651 Premedication/ or Antibiotic Prophylaxis/ (23099) 652 (premedication* or (antibiotic adj3 (prophylaxis or prevention)) or pretreatment or (preanaesthetic adj3 (treatment or medication))).mp. (178205) 653 Sclerotherapy/ (4629) 654 (sclerotherap* or sclero#ation or sclerosing therapy).mp. (7591) 655 self administration/ (9872) 656 (self adj3 administration*).mp. (13235) 657 Self Medication/ (4217) 658 (self adj3 medication*).mp. (7461) 659 Thrombolytic Therapy/ or Hirudin Therapy/ (20684) 660 (((fibrinolytic or hirudin) adj3 therap*) or (therapeutic adj3 thrombolys#s) or (anticoagula* adj3 (therapy or treatment))).mp. (21730) 661 electric stimulation therapy/ (18018) 662 (((electric stimulation or electrostimulation) adj3 therap*) or electrotherap*).mp. (18636) 663 cardiac pacing, artificial/ (19295) 664 ((artificial cardiac or biventricular) adj3 pacing*).mp. (1460) 665 cardiac resynchronization therapy/ (2390) 666 ((cadiac adj3 resynchronic#ation*) or (biventricular adj3 pacing)).mp. (1415) 667 / (5714) 668 (((brain adj3 stimulation*) or electrical stimulation) adj3 brain).mp. (13843) 669 electric countershock/ (13049) 670 ((cardiac adj3 electroversion*) or cardioversion* or (electric* adj3 (countershock or cadioconversion)) or (electric adj3 defibrillation)).mp. (15280)

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671 pulsed radiofrequency treatment/ (170) 672 ((pulsed radio adj3 frequency treatment) or (pulsed radiofrequency adj3 treatment*)).mp. (277) 673 stimulation/ (353) 674 (spinal adj3 stimulation).mp. (3660) 675 transcranial direct current stimulation/ (454) 676 ((transcranial direct adj3 current stimulation) or (transcranial adj3 electric stimulation)).mp. (2107) 677 transcutaneous electric stimulation/ (3707) 678 ((transcutaneous electric* adj3 nerve stimulation) or (percutaneous electric* adj3 nerve stimulation) or ((analgesic cutaneous or transcutaneous or transdermal) adj3 electrostimulation) or electroanalgesia).mp. (4500) 679 / (807) 680 ((vagal or vagus) adj3 nerve stimulation*).mp. (2494) 681 emergency treatment/ (9177) 682 (emergency adj3 (therap* or treatment*)).mp. (16567) 683 advanced trauma life support care/ (87) 684 (advanced trauma adj3 life support).mp. (637) 685 first aid/ (7238) 686 (first adj3 aid*).mp. (10681) 687 heimlich maneuver/ (13) 688 ((abdominal adj3 thrust*) or (Heimlich adj3 man#euver)).mp. (34) 689 resuscitation/ (23335) 690 resuscitation*.mp. (58915) 691 cardiopulmonary resuscitation/ or advanced cardiac life support/ (13277) 692 (((basic cardiac or advanced cardiac) adj3 life support) or ((cardio pulmonary or cardiopulmonary) adj3 resuscitation) or (mouth adj3 mouth resuscitation*)).mp. (18735) 693 ((cardiac or heart) adj3 massage*).mp. (3415) 694 respiration, artificial/ (41225) 695 ((artificial adj3 respiration) or (mechanical adj3 ventilation*)).mp. (59676) 696 resuscitation orders/ (3330) 697 ((resuscitation adj3 (order* or decision* or polic*)) or (do adj3 resuscitate order*) or (withholding adj3 resuscitation)).mp. (4059) 698 "transportation of patients"/ (8361) 699 (patient* adj3 (transportion* or transport or transfer)).mp. (12299)

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700 Feeding Methods/ (800) 701 (feeding adj3 method*).mp. (2651) 702 Bottle Feeding/ (3422) 703 ((bottle adj3 feeding) or bottlefeeding*).mp. (4373) 704 Enteral Nutrition/ (16782) 705 ((enter* adj3 (feeding or nutrition)) or ((force or tube*) adj3 feeding) or (gastric adj3 feeding tube*) or (feeding adj3 (intestinal or intragastric))).mp. (24213) 706 Parenteral Nutrition/ or Parenteral Nutrition, Home/ or Parenteral Nutrition, Home Total/ or Parenteral Nutrition, Total/ (22059) 707 (((intravenous or parenteral) adj3 feeding*) or (parenteral adj3 nutrition)).mp. (28816) 708 (hyperalimentation adj3 (parenteral or intravenous)).mp. (866) 709 hyperthermia, induced/ (13795) 710 ((fever adj3 therap*) or (hyperthermia adj3 (induced or local or therapeutic)) or thermotherapy).mp. (19093) 711 diathermy/ (3322) 712 (diatherm* or elctrodiathermy or (high frequency adj3 (current therapy or electrotherapy or therapy))).mp. (5684) 713 short-wave therapy/ or ultrasonic therapy/ (8784) 714 ((short wave or shortwave or ultrasonic) adj3 therap*).mp. (8958) 715 ultrasound, high-intensity focused, transrectal/ (414) 716 high-intensity focused ultrasound ablation/ (885) 717 (high intensity adj3 focused ultrasound).mp. (2268) 718 Leeching/ (298) 719 (hirudotherapy or leeching or (leech adj3 therap*)).mp. (461) 720 Magnetic Field Therapy/ (779) 721 ((magnetic field or magnetic stimulation) adj3 therap*).mp. (1060) 722 Transcranial Magnetic Stimulation/ (7985) 723 (transcranial adj3 magnetic stimulation*).mp. (11803) 724 Mechanical Thrombolysis/ (407) 725 ((mechanical adj3 (clot disruption* or thrombolysis or thrombectomy or embolectomy)) or (pharmacomechanical adj3 thrombectomy)).mp. (1740) 726 nutrition therapy/ or diet therapy/ (11126) 727 (((nutrition or diet or dietary) adj3 therap*) or ((nutrition or diet or dietary) adj3 treatment)).mp. (26884)

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728 caloric restriction/ or diet, diabetic/ or diet, carbohydrate-restricted/ or diet, -restricted/ or diet, gluten-free/ or diet, mediterranean/ or diet, paleolithic/ or diet, protein-restricted/ or diet, reducing/ or diet, sodium-restricted/ or diet, vegetarian/ or diet, macrobiotic/ or diet, vegan/ or ketogenic diet/ or nutritional support/ (41172) 729 ((caloric adj3 restriction) or (diet* adj3 (low calorie or diabetic or carbohydrate restricted or atkins or south beach or low carbohydrate or fat free or fat restricted or low fat or gluten free or Mediterranean or caveman or hunter gatherer or paleo or Paleolithic or stone age or low protein or protein free or reducing or weight loss or weight reduction or low salt or low sodium or salt free or sodium restricted or vegetarian or macrobiotic or vegan or ketogenic or supplementation))).mp. (66009) 730 ((artificial adj3 feeding) or (nutritional adj3 support)).mp. (11828) 731 orthopedic procedures/ (20226) 732 (orthopedic adj3 (procedure* or surger* or operation)).mp. (26217) 733 cementoplasty/ (68) 734 (cementoplast* or (osteoplast* adj3 percutaneous)).mp. (187) 735 vertebroplasty/ (1421) 736 vertebroplasty.mp. (2859) 737 kyphoplasty/ (496) 738 ((balloon adj3 vetebroplasty) or kyphoplasty).mp. (1369) 739 intervertebral disc chemolysis/ (459) 740 ((intervertebral adj3 dis# chemolysis) or chemonucleolys#s or discolys#s).mp. (797) 741 manipulation, orthopedic/ (3633) 742 (orthop#edic adj3 manipulation).mp. (7) 743 viscosupplementation/ (102) 744 viscocupplementation*.mp. (0) 745 Pain Management/ (23239) 746 ((pain adj3 (management* or relief)) or ((audio or sequential analgetic or surgical) adj3 analgesia) or audioanalgesia).mp. (66195) 747 patient care/ or ambulatory care/ (45981) 748 ((patient or informal or after or ambulatory or outpatient or urgent or advance) adj3 care*).mp. (267906) 749 aftercare/ (6862) 750 ((after adj3 treatment*) or aftercare or after care).mp. (317618) 751 ((clinic adj3 visit*) or (outpatient adj3 health service*)).mp. (10094) 752 peritoneal dialysis, continuous ambulatory/ (9633)

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753 (continuous ambulatory adj3 peritoneal dialysis).mp. (10704) 754 "bloodless medical and surgical procedures"/ (112) 755 (((blood conservation or transfusion alternative) adj3 strateg*) or (bloodless medical adj3 (surgical procedures or surgery programs)) or ((bloodless or transfusion free) adj3 surger*)).mp. (525) 756 critical care/ (42672) 757 ((critical or intensive) adj3 care).mp. (159935) 758 intensive care, neonatal/ (4660) 759 ((neonatal or infant or newborn) adj3 intensive care).mp. (22358) 760 custodial care/ or day care, medical/ (5035) 761 ((custodial or day) adj3 care).mp. (14382) 762 (partial adj3 hospitalization).mp. (335) 763 "episode of care"/ (1563) 764 (care adj3 episode*).mp. (2966) 765 hospitalization/ (80771) 766 hospitalization*.mp. (156067) 767 "length of stay"/ (66073) 768 ((hospital adj3 stay*) or (stay* adj3 length*)).mp. (124410) 769 patient admission/ (20041) 770 ((patient or voluntary) adj3 admission).mp. (23279) 771 patient discharge/ (21738) 772 ((patient or hospital) adj3 discharge).mp. (45891) 773 patient readmission/ (10100) 774 readmission*.mp. (18514) 775 institutionalization/ or deinstitutionalization/ (8012) 776 (institutionalization or institutionalized or deinstitutionalization or deinstitutionalized).mp. (18083) 777 life support care/ (7219) 778 ((life support adj3 care) or (extraordinary adj3 treatment*) or (life adj3 prolongation)).mp. (8748) 779 advanced cardiac life support/ or advanced trauma life support care/ (870) 780 (advanced trauma adj3 life support).mp. (637) 781 long-term care/ (22903) 782 (long term adj3 care).mp. (34157) 783 night care/ (1341)

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784 (night adj3 care*).mp. (1499) 785 home nursing/ (8219) 786 (home care or home nursing).mp. (50054) 787 respite care/ (922) 788 (respite adj3 care*).mp. (1315) 789 primary care nursing/ (266) 790 (primary care adj3 nursing).mp. (570) 791 palliative care/ (44133) 792 (palliative adj3 (care or surger* or therap* or treatment*)).mp. (57265) 793 "Hospice and Palliative Care Nursing"/ or Palliative Care Nursing/ (212) 794 (palliative adj3 nursing).mp. (662) 795 Palliative medicine/ or Palliative Care Medicine/ (88) 796 (palliative adj3 medicine).mp. (1594) 797 patient positioning/ (3364) 798 (patient adj3 positioning*).mp. (5809) 799 kangaroo-mother care method/ (145) 800 ((Kangaroo mother adj3 care) or (skin adj3 skin care)).mp. (6542) 801 perinatal care/ or postnatal care/ (7509) 802 (perinatal adj3 (care or medicine)).mp. (6107) 803 ((postnatal or postpartum) adj3 care).mp. (5937) 804 perioperative care/ or intraoperative care/ or perioperative nursing/ (30562) 805 ((perioperative or intraoperative or postoperative or preoperative or intraoperative) adj3 care).mp. (125003) 806 ((perianesthesia or perioperative) adj3 nursing).mp. (6644) 807 preoperative care/ or postoperative care/ (97200) 808 (postoperative adj3 (procedure* or therapy or treatment)).mp. (18963) 809 (preoperative adj3 (procedure* or therapy or treatment)).mp. (9252) 810 subacute care/ (759) 811 (("sub acute" or subacute) adj3 care*).mp. (1093) 812 Terminal Care/ (22955) 813 ((life care adj3 end*) or (terminal adj3 care)).mp. (26321) 814 hospice care/ (5074)

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815 (hospice adj3 (care or program*)).mp. (7706) 816 time-to-treatment/ (1795) 817 (time adj3 (treatment* or time)).mp. (3176095) 818 transitional care/ (43) 819 (transitional adj3 care).mp. (827) 820 Euthanasia/ or Euthanasia, Active/ or Euthanasia, Active, Voluntary/ or Euthanasia, Passive/ (12848) 821 (euthanasia or mercy killing*).mp. (25217) 822 withholding treatment/ (9844) 823 ((withholding adj3 treatment) or ((cessation or withdraw*) adj3 treatment)).mp. (21925) 824 Suicide, Assisted/ (4970) 825 (assisted adj3 (death* or suicide)).mp. (5577) 826 Patient Care Bundles/ (138) 827 care bundles.mp. (287) 828 phototherapy/ (6297) 829 (phototherap* or ((light or photoradiation or ultraviolet or sunlight) adj3 therap*)).mp. (19044) 830 heliotherapy/ (443) 831 Heliotherapy.mp. (501) 832 intense pulsed light therapy/ (70) 833 (intense pulsed adj3 (therap* or treatment)).mp. (201) 834 low-level light therapy/ (3723) 835 ((low level adj3 (light therap* or laser irradiation or laser therap*)) or ((biostimuation or phototherapy) adj3 laser) or (low power adj3 (light therap* or laser irradiation or laser therap*))).mp. (4584) 836 hematoporphyrin photoradiation/ (636) 837 ((hematoporphyrin adj3 photoradiation) or photochemotherapy or chemophototherapy).mp. (15589) 838 ultraviolet therapy/ (4065) 839 ((ultraviolet adj3 therap*) or actinotherap*).mp. (4468) 840 puva therapy/ (3294) 841 ((puva adj3 (therap* or radiation)) or (psoralen ultraviolet adj3 a therapy)).mp. (3645) 842 photopheresis/ (752) 843 ((extracorporeal adj3 photochemotherap*) or photopheresis).mp. (1115)

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844 modalities/ (31080) 845 ((physical therapy adj3 (modalit* or technique*)) or neurophysiotherapy or (neurological adj3 physiotherapy)).mp. (31337) 846 animal assisted therapy/ or equine-assisted therapy/ (266) 847 (((animal or pet) adj3 assisted therap*) or (per adj3 therap*) or ((animal or pet) adj3 facilitated therap*)).mp. (2125) 848 ((equine assisted adj3 (psychotherap* or therap*)) or hippotherap* or (horseback riding adj3 therap*)).mp. (199) 849 exercise movement techniques/ (480) 850 kinesiotherapy.mp. (121) 851 qigong/ (54) 852 (Qigong or ch'I king or qi gong or chi kung or chigung).mp. (535) 853 dance therapy/ (244) 854 dance therap*.mp. (274) 855 tai ji/ (737) 856 (tai ji or tai chi or (tai adj3 ji quan) or t'ai chi or (tai chi adj3 chuan) or taijiqua).mp. (1166) 857 exercise therapy/ (29984) 858 (exercise adj3 (therap* or techniques or treatment)).mp. (35677) 859 motion therapy, continuous passive/ (596) 860 motion therap*.mp. (674) 861 muscle stretching exercises/ (1116) 862 ((stretching adj3 exercises) or ((active or ballistic or dynamic or isometric or passive or relaxed) adj3 stretching)).mp. (2291) 863 plyometric exercise/ (163) 864 ((stretch shortening adj3 (drill* or exercise*)) or plyometric*).mp. (618) 865 resistance training/ (4500) 866 ((resistance adj3 training) or ((weight lifting or weight bearing) adj3 exercise program) or (strength adj3 training)).mp. (10845) 867 hydrotherapy/ (2365) 868 (hydrotherap* or (whirlpool adj3 bath*) or (water immersion adj3 therapy)).mp. (2714) 869 therapeutic irrigation/ (15946) 870 ((therapeutic adj3 irrigation) or douching* or lavage*).mp. (64050) 871 manipulation, spinal/ (1263)

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872 ((spinal or cervical or lumbar) adj3 manipulation*).mp. (2421) 873 myofunctional therapy/ (264) 874 ((myofunction adj3 therap*) or ((oral or orofacial) adj3 myotherap*)).mp. (3) 875 Placebos/ (33210) 876 (placebo* or (sham adj3 treatment)).mp. (190231) 877 Rehabilitation/ (17110) 878 (rehabilitation or habilitation).mp. (135266) 879 Activities of Daily Living/ (53891) 880 ((daily living adj3 activit*) or (chronic limitation adj3 activit*)).mp. (63638) 881 Bibliotherapy/ (365) 882 Bibliotherap*.mp. (473) 883 Correction of hearing impairment/ (1761) 884 ((audiologic adj3 (habilitation* or rehabilition*)) or (aural adj3 (habilitation* or rehabilition*)) or (hearing adj3 (habilitation* or rehabilition* or revalidation))).mp. (45) 885 Communication Methods, Total/ (276) 886 (Total adj3 communication method*).mp. (278) 887 Lipreading/ (920) 888 (lipreading or lip reading* or speechreading*).mp. (1284) 889 Sign language/ (1807) 890 sign language*.mp. (2258) 891 Manual Communication/ (513) 892 (manual adj3 communication*).mp. (593) 893 Early Ambulation/ (2314) 894 (((early or accelerated) adj3 ambulation) or (early adj3 mobilization)).mp. (5166) 895 Neurological Rehabilitation/ (19) 896 ((neurologic adj3 rehabilitation) or neurorehabilitation).mp. (1939) 897 / (11244) 898 ((occupational adj3 therap*) or ergotherapy).mp. (16172) 899 Recreation Therapy/ (78) 900 ((recreational or recreation) adj3 therap*).mp. (435) 901 "Rehabilitation of Speech and Language Disorders"/ (0)

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902 ((speech adj3 (rehabilitation or reeduction)) or (voice adj3 (reeducation or rehabilitation))).mp. (1041) 903 Language Therapy/ (1445) 904 (language adj3 (therap* or training*)).mp. (3366) 905 Speech, Alaryngeal/ (1375) 906 (alaryngeal adj3 (speech or voice)).mp. (1421) 907 Speech, Esophageal/ (622) 908 (#esophag* adj3 (speech* or voice)).mp. (860) 909 Speech, Therapy/ (5640) 910 ((speech adj3 (therap* or education or training)) or (language adj3 (learning or therapy or training)) or (logopedic adj3 (education or training)) or logotherapy or phoniatrics or phoniatry).mp. (12339) 911 Voice Training/ (1214) 912 (voice adj3 training*).mp. (1306) 913 Telereganilitation/ (0) 914 ((remote or virtual or tele) adj3 rehabilitation*).mp. (262) 915 Respiratory Therapy/ (6036) 916 ((respiratory or inhalation) adj3 therapy).mp. (21884) 917 Chest Wall Oscillation/ (237) 918 ((high frequency adj3 chest compression) or (chest wall adj3 oscillation*)).mp. (295) 919 Extracorporeal Membrane Oxygenation/ (6574) 920 ((extracorporeal or extra corporeal or extrapulmonary) adj3 oxygenation).mp. (8748) 921 Oxygen Inhalation Therapy/ (12564) 922 (oxygen adj3 (therap* or administration or insufflation or treatment)).mp. (23103) 923 Hyperbaric Oxygenation/ (10589) 924 ((hyperbaric adj3 (oxygenation* or o2 or oxygen therapy)) or ((high adj3 oxygen pressure) or tension o2 or tension oxygen)).mp. (11618) 925 Self Care/ (26463) 926 (self adj3 (care or management or treatment)).mp. (44912) 927 Blood Glucose Self-Monitoring/ (4838) 928 ((blood glucose or blood sugar) adj3 self monitoring).mp. (5568) 929 Self Administration/ (9872) 930 (self adj3 administration*).mp. (13235)

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931 Self Medication/ (4217) 932 (self adj3 medication*).mp. (7461) 933 Therapies, Investigational/ (1177) 934 (((experimental or innovative or investigational) adj3 therap*) or ((experimental or innovative or investigational) adj3 treatment*)).mp. (24082) 935 "Compassionate Use Trials"/ (335) 936 (expanded access* or compassionate use* or (humanitarian adj3 device exemption*)).mp. (1632) 937 Therapy, Computer-Assisted/ or Radiotherapy Planning, Computer-Assisted/ (20604) 938 (computer assisted adj3 (protocol directed or therapy or dosimetry calculation* or radiotherapy or drug therapy)).mp. (23406) 939 Surgery, Computer-Assisted/ (11922) 940 ((computer aided or computer assisted or image guided) adj3 surger*).mp. (13086) 941 Robotic Surgical Procedures/ (1136) 942 (robotic surgical adj3 procedure*).mp. (1177) 943 Therapy with Helminths/ (50) 944 ((helminthic or helminth or worm) adj3 therap*).mp. (191) 945 or/365-944 (10516979) 946 364 and 945 (95446)

Alertness Symptom Search

947 arousal/ or attention/ or wakefulness/ (101709) 948 / or mental fatigue/ (22958) 949 Torpor/ (63) 950 (mental adj3 fatigue*).mp. (1979) 951 (cortical adj3 vigilance).mp. (23) 952 (concentration adj3 (less or lack or reduce* or mental)).mp. (17042) 953 (alertness* or attention* or fatigue* or arousal* or wakeful* or torpor or lassitude).mp. (463225) 954 letharg*.mp. (6326) 955 fatigability.mp. (1766) 956 (awareness or consciousness or distractab* or dysthymia).mp. (140947) 957 or/946-955 (614354)

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958 946 and 956 (3989)

959 limit 957 to "all child (0 to 18 years)" (1697) 960 (infan* or neonat* or newborn* or baby or babies or toddler* or p?ediatric* or child* or teen* or adoles* or youth*).mp. (3616267) 961 957 and 959 (2007) 962 958 or 960 (2007)

Behaviour Symptom Search

947 Behavioral Symptoms/ (2034) 948 / (4286) 949 Irritable Mood/ (1272) 950 self-injurious behavior/ or self mutilation/ (8628) 951 (behavio?r* adj3 (symptom* or disturbance* or change)).mp. (33127) 952 (psychomotor adj3 (excitement or hyperactivity)).mp. (76) 953 (body adj2 rocking).mp. (96) 954 (head adj2 banging).mp. (126) 955 agitation.mp. (14279) 956 .mp. (2489) 957 restless*.mp. (7219) 958 irritabil*.mp. (7235) 959 aggress*.mp. (168145) 960 antagonistic.mp. (24929) 961 destructive*.mp. (25892) 962 parasuicide.mp. (464) 963 (self adj3 (injur* or destructive or harm or mutilat*)).mp. (13954) 964 ((flat or negative) adj3 affect).mp. (6898) 965 or/947-964 (293813) 966 946 and 965 (2190)

967 limit 966 to "all child (0 to 18 years)" (878)

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Pawliuk C, et al. BMJ EBM 2020;0:1–5. doi: 10.1136/bmjebm-2020-111452 BMJ Publishing Group Limited (BMJ) disclaims all liability and responsibility arising from any reliance Supplemental material placed on this supplemental material which has been supplied by the author(s) BMJ EBM

968 (infan* or neonat* or newborn* or baby or babies or toddler* or p?ediatric* or child* or teen* or adoles* or youth*).mp. (3616267) 969 966 and 968 (1035) 970 967 or 969 (1035)

Bowel Incontinence Symptom Search

947 Fecal Incontinence/ (8443) 948 Feces/ (76546) 949 Defecation/ (6117) 950 Diarrhea/ (41675) 951 Gastrointestinal Motility/ (19976) 952 (defecate* or diarrh* or f#eces).mp. (112801) 953 ((symptom or function* or habit* or movement* or strain*) adj3 bowel).mp. (8874) 954 (f#ecal* adj3 (incontin* or evacuat*)).mp. (1718) 955 loose stool*.mp. (852) 956 ((intestin* or gastrointestin* or stomach) adj3 motility).mp. (23055) 957 (colon adj3 transit*).mp. (432) 958 or/947-957 (212123) 959 946 and 958 (1126)

960 (infan* or neonat* or newborn* or baby or babies or toddler* or p?ediatric* or child* or teen* or adoles* or youth*).mp. (3616267) 961 959 and 960 (722) 962 limit 959 to "all child (0 to 18 years)" (652) 963 961 or 962 (722) Breathing Difficulty Symptom Search

947 mucus/se or saliva/se (5660) 948 respiratory insufficiency/ or hypoventilation/ or hyperventilation/ (35045) 949 (hypoventilation* or hyperventilation).mp. (14497) 950 Dyspnea/ (16973) 951 Cough/ (13323)

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Pawliuk C, et al. BMJ EBM 2020;0:1–5. doi: 10.1136/bmjebm-2020-111452 BMJ Publishing Group Limited (BMJ) disclaims all liability and responsibility arising from any reliance Supplemental material placed on this supplemental material which has been supplied by the author(s) BMJ EBM

952 cough*.mp. (47784) 953 Apnea/ (6627) 954 airway obstruction/ or nasal obstruction/ (21025) 955 ((mucus or saliv*) adj3 secretions).mp. (960) 956 ((airway adj3 obstruction*) or choking).mp. (26948) 957 dyspn#ea*.mp. (7859) 958 Apn#ea*.mp. (7006) 959 (Breath* adj3 (difficult* or abnormalit* or dysfunction*)).mp. (2228) 960 (Respirat* adj3 distress).mp. (44587) 961 ((respiratory or ventilatory) adj3 depression).mp. (5913) 962 (respiratory adj3 (failure or insufficien* or arrest)).mp. (49120) 963 (breathless* or (breath adj3 shortness*)).mp. (9321) 964 (severe adj3 paradoxical respiration).mp. (3) 965 (cardiopulmonary adj3 (arrest or insufficency)).mp. (2454) 966 (lung adj3 insufficiency).mp. (212) 967 or/947-966 (221698) 968 946 and 967 (2352)

969 limit 968 to "all child (0 to 18 years)" (1350) 970 (infan* or neonat* or newborn* or baby or babies or toddler* or p?ediatric* or child* or teen* or adoles* or youth*).mp. (3616267) 971 968 and 970 (1534) 972 969 or 971 (1534)

Constipation Symptom Search

947 Constipation/ (11576) 948 Defecation/ (6117) 949 Feces/ (76546) 950 Fecal Impaction/ (962) 951 Gastrointestinal Motility/ (19976) 952 constipat*.mp. (22050)

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Pawliuk C, et al. BMJ EBM 2020;0:1–5. doi: 10.1136/bmjebm-2020-111452 BMJ Publishing Group Limited (BMJ) disclaims all liability and responsibility arising from any reliance Supplemental material placed on this supplemental material which has been supplied by the author(s) BMJ EBM

953 ((hard or impact*) adj3 stool*).mp. (328) 954 (bowel* adj3 (symptom* or habit or evacuat* or movement* or void* or strain)).mp. (6300) 955 (f#eces adj3 (impact* or evacuate*)).mp. (13) 956 defecate*.mp. (725) 957 (intestin* adj3 (motility adj3 dysmotility)).mp. (1) 958 (gastrointestinal adj3 (motility or dysmotility)).mp. (21902) 959 (colon* adj3 (transit or inertia)).mp. (1701) 960 (bowel* adj3 (function* or habit* or symptom* or evacuat* or movement or void* or strain*)).mp. (9540) 961 (f#eces adj3 (evacuat* or impacted)).mp. (14) 962 (fecalith or dyschezia).mp. (335) 963 (abdominal adj3 disten*).mp. (5586) 964 or/947-963 (135641) 965 946 and 964 (1028)

966 limit 965 to "all child (0 to 18 years)" (732) 967 (infan* or neonat* or newborn* or baby or babies or toddler* or p?ediatric* or child* or teen* or adoles* or youth*).mp. (3616267) 968 965 and 967 (800) 969 966 or 968 (800)

Feeding Difficulties Symptom Search

947 gastroesophageal reflux/ or laryngopharyngeal reflux/ (23165) 948 (feeding adj3 (difficult* or problem*)).mp. (3327) 949 Regurgitation.mp. (30040) 950 Vomiting/ (20773) 951 (vomit* or emesis).mp. (68022) 952 retch*.mp. (940) 953 Nausea/ (13833) 954 nausea.mp. (54095) 955 (feeding adj3 intolerance*).mp. (496)

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Pawliuk C, et al. BMJ EBM 2020;0:1–5. doi: 10.1136/bmjebm-2020-111452 BMJ Publishing Group Limited (BMJ) disclaims all liability and responsibility arising from any reliance Supplemental material placed on this supplemental material which has been supplied by the author(s) BMJ EBM

956 (salivary adj3 secretion*).mp. (2622) 957 Dyspepsia/ (7862) 958 (Dyspepsia* or indigestion*).mp. (12613) 959 Deglutition Disorders/ (16424) 960 aspiration.mp. (68047) 961 ((Gastric or Gastroesophageal or gastric acid or gastro oesophageal or esophageal) adj3 reflux).mp. (29691) 962 (swallowing adj3 (difficulty or disorder* or incoordination)).mp. (2423) 963 ( or (deglutition adj2 disorder*)).mp. (28288) 964 ((oromotor or oral motor) adj3 skill*).mp. (88) 965 or/947-964 (247301) 966 946 and 965 (1983)

967 limit 966 to "all child (0 to 18 years)" (1085) 968 (infan* or neonat* or newborn* or baby or babies or toddler* or p?ediatric* or child* or teen* or adoles* or youth*).mp. (3616267) 969 966 and 968 (1221) 970 967 or 969 (1221)

Sleep Symptom Search

947 Sleep Disorders/ (17223) 948 / or sleep deprivation/ or sleep disorders, circadian rhythm/ or sleep disorders, intrinsic/ (9760) 949 "Sleep Initiation and Maintenance Disorders"/ (9937) 950 (sleep adj3 (disturbanc* or disorder or problem* or disturbance* or dysfunction*)).mp. (19385) 951 ((short sleeper or long sleeper or subwakefullness) adj3 syndrome*).mp. (0) 952 (neurogenic tachypneas adj3 sleep-related).mp. (0) 953 *.mp. (414) 954 (nocturnal adj3 "eating-drinking syndrome").mp. (4) 955 ((extrinsic or environmental or adjustment or limit-setting) adj3 sleep).mp. (344) 956 *.mp. (14655) 957 (sleep adj3 (initiation or maintenance)).mp. (10607)

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958 ((lethal or severe or obstructive) adj3 sleep apn#ea).mp. (3344) 959 (sleep disordered adj3 breathing).mp. (4691) 960 or/947-959 (59359) 961 946 and 960 (704)

962 limit 961 to "all child (0 to 18 years)" (263) 963 (infan* or neonat* or newborn* or baby or babies or toddler* or p?ediatric* or child* or teen* or adoles* or youth*).mp. (3616267) 964 961 and 963 (308)

Temperature Regulation Symptom Search

947 body temperature regulation/ or sweating/ or thermogenesis/ or torpor/ (28408) 948 (cold adj3 (hands or feet)).mp. (258) 949 Hypothermia/ (12765) 950 hypothermia*.mp. (39844) 951 fever/ or "fever of unknown origin"/ (37761) 952 (fever* or hyperthermia* or pyrexia*).mp. (206630) 953 (diaphoresis or sweating or perspiration).mp. (12577) 954 Shivering/ (1676) 955 shiver*.mp. (3949) 956 Primary Dysautonomias/ (185) 957 Dysautonomia*.mp. (2551) 958 (body temperature adj3 regulation*).mp. (21848) 959 (thermoregulation* or heat loss*).mp. (8002) 960 or/947-959 (279889) 961 946 and 960 (1838)

962 limit 961 to "all child (0 to 18 years)" (836) 963 (infan* or neonat* or newborn* or baby or babies or toddler* or p?ediatric* or child* or teen* or adoles* or youth*).mp. (3616267) 964 961 and 963 (965)

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965 962 or 964 (965)

Tone and Motor Difficulties Symptom Search

947 Muscle / (7830) 948 Spasm/ (6764) 949 / or torticollis/ (8535) 950 Dystonic Disorders/ (2019) 951 Muscle Tonus/ (6457) 952 ataxia/ or cerebellar ataxia/ or gait ataxia/ (11026) 953 muscle hypertonia/ or muscle rigidity/ (2971) 954 neuromuscular manifestations/ or muscle / (2919) 955 / (1892) 956 hyperkinesis/ or / (5051) 957 Contracture/ (7298) 958 ((tone adj3 (problem* or difficult*)) or (tone adj3 (problem* or difficult*))).mp. (101) 959 (spastic* or spasm).mp. (43371) 960 (muscle* adj3 tonus*).mp. (6739) 961 (dystonia* or toticollis).mp. (13301) 962 (ataxia* or ataxy).mp. (36497) 963 (rubral adj3 ).mp. (4) 964 (coordination adj3 (impairment* or lack of)).mp. (926) 965 (dyscoordination or dyssynergia).mp. (1386) 966 (muscl* adj3 (tone or hypertonicity or atonic or flaccid or poor or increase* or decrease* or spasm)).mp. (36354) 967 (((abnormal or involuntary) adj3 movement) or asterixis or or hemiballism* or ballismus).mp. (17182) 968 (hyperkinesi* or hypokinesia).mp. (8122) 969 (muscle* adj3 contracture*).mp. (1278) 970 (immobil* or (reduced adj3 mobilit*)).mp. (112004) 971 ((awkward or spastic) adj3 gait).mp. (362) 972 (muscular adj3 (hypotrophy or rigidity or hypertonia)).mp. (552)

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Pawliuk C, et al. BMJ EBM 2020;0:1–5. doi: 10.1136/bmjebm-2020-111452 BMJ Publishing Group Limited (BMJ) disclaims all liability and responsibility arising from any reliance Supplemental material placed on this supplemental material which has been supplied by the author(s) BMJ EBM

973 hypotonia.mp. (7868) 974 dyskinesia.mp. (14787) 975 or/947-973 (283784) 976 946 and 975 (8395)

977 limit 976 to "all child (0 to 18 years)" (2634) 978 (infan* or neonat* or newborn* or baby or babies or toddler* or p?ediatric* or child* or teen* or adoles* or youth*).mp. (3616629) 979 976 and 978 (3083) 980 977 or 979 (3083)

Urinary Incontinence Symptom Search

947 Enuresis/ or Diurnal Enuresis/ or Nocturnal Enuresis/ (4496) 948 urinary incontinence/ or Urinary Incontinence, Stress/ or Urinary Incontinence, Urge/ or urinary retention/ (31813) 949 (urinary adj3 (incontinence or retention)).mp. (42020) 950 bedwetting.mp. (283) 951 enuresis.mp. (5649) 952 or/947-951 (47042) 953 946 and 952 (230)

954 limit 953 to "all child (0 to 18 years)" (93) 955 (infan* or neonat* or newborn* or baby or babies or toddler* or p?ediatric* or child* or teen* or adoles* or youth*).mp. (3616629) 956 953 and 955 (101) 957 954 or 956 (101)

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Pawliuk C, et al. BMJ EBM 2020;0:1–5. doi: 10.1136/bmjebm-2020-111452