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Familial Pellagra-Like Skin Rash with Neurological Manifestations
Arch Dis Child: first published as 10.1136/adc.56.2.146 on 1 February 1981. Downloaded from 146 Freundlich, Statter, and Yatziv Familial pellagra-like skin rash with neurological manifestations E FREUNDLICH, M STATTER, AND S YATZIV Department ofPaediatrics, Government Hospital, Nahariya, Israel, and Paediatric Research Unit and Department ofPaediatrics, Hadassah University Hospital, Jerusalem, Israel development was normal, although the skin rash SUMMARY A 14-year-old boy of Arabic origin was noted several times. On one occasion a skin presented with a pellagra-like rash and neurological biopsy was performed and reported to be compatible manifestations including ataxia, dysarthria, nystag- with pellagra. A striking improvement was again mus, and coma. There was a striking response to oral noticed after nicotinamide administration. nicotinamide. The laboratory findings were not The last admission was at age 14 years in late typical of Hartnup disease: aminoaciduria and November (as were all previous admissions). At indicanuria were absent and there was no evidence this time, he presented with mild confusion, diplopia, of tryptophan malabsorption. Tryptophan loading dysarthria, ataxia, and a pellagroid skin rash. His did not induce tryptophanuria nor did it increase general condition deteriorated during the next few excretion of xanthurenic or kynurenic acids. These days; he became unable to walk or stand, and both findings support the possibility of a block in trypto- horizontal and vertical nystagmus were observed. phan degradation. The family history suggests a He became apathetic and entered into deep coma genetically-determined disorder. 4 days after admission. The electroencephalogram showed a markedly abnormal tracing with short We report a patient with familial pellagra-like skin bursts of high voltage 2 5 per second activity with manifestations and laboratory rash, with neurological superimposed sharp waves, mainly over the posterior copyright. -
Micronutrient Status and Dietary Intake of Iron, Vitamin A, Iodine
nutrients Review Micronutrient Status and Dietary Intake of Iron, Vitamin A, Iodine, Folate and Zinc in Women of Reproductive Age and Pregnant Women in Ethiopia, Kenya, Nigeria and South Africa: A Systematic Review of Data from 2005 to 2015 Rajwinder Harika 1,*, Mieke Faber 2 ID , Folake Samuel 3, Judith Kimiywe 4, Afework Mulugeta 5 and Ans Eilander 1 1 Unilever Research & Development, Vlaardingen, 3130 AC, The Netherlands; [email protected] 2 Non-communicable Diseases Research Unit, South African Medical Research Council, Cape Town 19070, South Africa; [email protected] 3 Department of Human Nutrition, University of Ibadan, Ibadan 200284, Nigeria; [email protected] 4 School of Applied Human Sciences, Kenyatta University, Nairobi 43844-00100, Kenya; [email protected] 5 Department of Nutrition and Dietetics, Mekelle University, Mekelle 1871, Ethiopia; [email protected] * Correspondence: [email protected]; Tel.: +31-101-460-5190 Received: 10 August 2017; Accepted: 28 September 2017; Published: 5 October 2017 Abstract: A systematic review was conducted to evaluate the status and intake of iron, vitamin A, iodine, folate and zinc in women of reproductive age (WRA) (≥15–49 years) and pregnant women (PW) in Ethiopia, Kenya, Nigeria and South Africa. National and subnational data published between 2005 and 2015 were searched via Medline, Scopus and national public health websites. Per micronutrient, relevant data were pooled into an average prevalence of deficiency, weighted by sample size (WAVG). Inadequate intakes were estimated from mean (SD) intakes. This review included 65 surveys and studies from Ethiopia (21), Kenya (11), Nigeria (21) and South Africa (12). -
THE PATHOLOGIC PHYSIOLOGY of PELLAGRA: V. the Circulating Blood Volume
THE PATHOLOGIC PHYSIOLOGY OF PELLAGRA: V. The Circulating Blood Volume Roy H. Turner J Clin Invest. 1931;10(1):111-120. https://doi.org/10.1172/JCI100332. Find the latest version: https://jci.me/100332/pdf THE PATHOLOGIC PHYSIOLOGY OF PELLAGRA V. THE CIRCULATING BLOOD VOLUME By ROY H. TURNER (From the Department of Medicine, Tulane University of Louisiana School of Medicine, and the Medical Services of the Charity Hospital, New Orleans) (Received for publication October 27, 1930) Determination of circulating blood volume was undertaken as a part of a study of the disturbed physiology in pellagra, chiefly for the purpose of finding out whether shrinkage of plasma volume existed in patients who were suffering from a disease frequently characterized by severe diarrhea. Such a shrinkage would be of great importance of itself, and would obviously have a bearing upon the interpretation of the composition of the plasma determined at the same time. The existence of anemia can hardly be established nor its severity estimated -so long as we are in ignorance- of the plasma volume. It was also considered possible that the magnitude of the circulating blood volume might be correlated with certain of the features of the skin lesions, such as the degree of exudation of serum. I have used the dye method of Keith, Rowntree and Geraghty (1) modified as follows: A 3 per cent aqueous solution of brilliant vital red (National Analine Company) was made up 'the afternoon before use, and sterilized at 100°C. for 8 minutes. With a sterile calibrated pipette the quantity of this solution for each patient was placed in a sterile 50 cc. -
A Review of the Biochemistry, Metabolism and Clinical Benefits of Thiamin(E) and Its Derivatives
View metadata, citation and similar papers at core.ac.uk brought to you by CORE Advance Access Publication 1 February 2006 eCAM 2006;3(1)49–59provided by PubMed Central doi:10.1093/ecam/nek009 Review A Review of the Biochemistry, Metabolism and Clinical Benefits of Thiamin(e) and Its Derivatives Derrick Lonsdale Preventive Medicine Group, Derrick Lonsdale, 24700 Center Ridge Road, Westlake, OH 44145, USA Thiamin(e), also known as vitamin B1, is now known to play a fundamental role in energy metabolism. Its discovery followed from the original early research on the ‘anti-beriberi factor’ found in rice polish- ings. After its synthesis in 1936, it led to many years of research to find its action in treating beriberi, a lethal scourge known for thousands of years, particularly in cultures dependent on rice as a staple. This paper refers to the previously described symptomatology of beriberi, emphasizing that it differs from that in pure, experimentally induced thiamine deficiency in human subjects. Emphasis is placed on some of the more unusual manifestations of thiamine deficiency and its potential role in modern nutri- tion. Its biochemistry and pathophysiology are discussed and some of the less common conditions asso- ciated with thiamine deficiency are reviewed. An understanding of the role of thiamine in modern nutrition is crucial in the rapidly advancing knowledge applicable to Complementary Alternative Medi- cine. References are given that provide insight into the use of this vitamin in clinical conditions that are not usually associated with nutritional deficiency. The role of allithiamine and its synthetic derivatives is discussed. -
Is There an Ideal Diet to Protect Against Iodine Deficiency?
nutrients Review Is There an Ideal Diet to Protect against Iodine Deficiency? Iwona Krela-Ka´zmierczak 1,† , Agata Czarnywojtek 2,3,†, Kinga Skoracka 1,* , Anna Maria Rychter 1 , Alicja Ewa Ratajczak 1 , Aleksandra Szymczak-Tomczak 1, Marek Ruchała 2 and Agnieszka Dobrowolska 1 1 Department of Gastroenterology, Dietetics and Internal Diseases, Poznan University of Medical Sciences, Heliodor Swiecicki Hospital, 60-355 Poznan, Poland; [email protected] (I.K.-K.); [email protected] (A.M.R.); [email protected] (A.E.R.); [email protected] (A.S.-T.); [email protected] (A.D.) 2 Department of Endocrinology, Metabolism and Internal Medicine, Poznan University of Medical Sciences, 60-355 Poznan, Poland; [email protected] (A.C.); [email protected] (M.R.) 3 Department of Pharmacology, Poznan University of Medical Sciences, 60-806 Poznan, Poland * Correspondence: [email protected]; Tel.: +48-665-557-356 or +48-8691-343; Fax: +48-8691-686 † These authors contributed equally to this work. Abstract: Iodine deficiency is a global issue and affects around 2 billion people worldwide, with preg- nant women as a high-risk group. Iodine-deficiency prevention began in the 20th century and started with global salt iodination programmes, which aimed to improve the iodine intake status globally. Although it resulted in the effective eradication of the endemic goitre, it seems that salt iodination did not resolve all the issues. Currently, it is recommended to limit the consumption of salt, which is the main source of iodine, as a preventive measure of non-communicable diseases, such as hypertension or cancer the prevalence of which is increasing. -
OCULAR MANIFESTATIONS of VITAMIN a DEFICIENCY*T
Br J Ophthalmol: first published as 10.1136/bjo.51.12.854 on 1 December 1967. Downloaded from Brit. J. Ophthal. (1967) 51, 854 OCULAR MANIFESTATIONS OF VITAMIN A DEFICIENCY*t BY G. VENKATASWAMY Department of Ophthalmology, Madurai Medical College, Madurai, India NUTRITIONAL deficiencies are a frequent cause of serious eye disease in India. Oomen (1961) reported a mortality of nearly 30 per cent. in young children with keratomalacia and an even higher proportion in those with protein malnutrition; about 25 per cent. of the survivors became totally blind, and about 60 per cent. were left with reduced vision in one or both eyes. Deficiency diseases revealed by dietary surveys have included xerophthalmia, Bitot's spots, angular stomatitis, and phrynoderma. Gilroy (1951) observed xerophthalmia in 250 out of 4,191 children from 44 estates in Assam. Sundararajan (1963) found signs of vitamin A deficiency in 35 to 45 per cent. of schoolchildren in Calcutta. Chandra, Venkatachalam, Belavadi, Reddy, and Gopalan (1960) reported that lack of protein and vitamin A was the most frequent cause of nutritional deficiency disorders in India; out of copyright. 14,563 children examined in a 5-year period, 2,245 showed malnutrition, 551 vitamin A deficiency, and 157 keratomalacia. Rao, Swaminathan, Swarup, and Patwardhan (1959) observed two to five cases ofvitamin A deficiency for every case ofkwashiorkor. A world-wide survey ofxerophthalniia carried out in nearly fifty countries (including countries in Asia) by WHO in 1962-1963 revealed that this was often the most important cause of blindness in young children. Scrimshaw (1959), McLaren (1963), and UNICEF (1963) concluded that vitamin A deficiency was one of the http://bjo.bmj.com/ main nutritional problems in tropical and subtropical areas. -
Scurvy Due to Selective Diet in a Seemingly Healthy 4-Year-Old Boy Andrew Nastro, MD,A,G,H Natalie Rosenwasser, MD,A,B Steven P
Scurvy Due to Selective Diet in a Seemingly Healthy 4-Year-Old Boy Andrew Nastro, MD,a,g,h Natalie Rosenwasser, MD,a,b Steven P. Daniels, MD,c Jessie Magnani, MD,a,d Yoshimi Endo, MD,e Elisa Hampton, MD,a Nancy Pan, MD,a,b Arzu Kovanlikaya, MDf Scurvy is a rare disease in developed nations. In the field of pediatrics, it abstract primarily is seen in children with developmental and behavioral issues, fDivision of Pediatric Radiology and aDepartments of malabsorptive processes, or diseases involving dysphagia. We present the Pediatrics and cRadiology, NewYork-Presbyterian/Weill Cornell Medical Center, New York, New York; bDivision of case of an otherwise developmentally appropriate 4-year-old boy who Pediatric Rheumatology and eDepartment of Radiology, developed scurvy after gradual self-restriction of his diet. He initially Hospital for Special Surgery, New York, New York; and d presented with a limp and a rash and was subsequently found to have anemia Division of Neonatal-Perinatal Medicine, University of Michigan, Ann Arbor, Michigan gDepartment of Pediatrics, and hematuria. A serum vitamin C level was undetectable, and after review of NYU School of Medicine, New York, New York hDepartment of the MRI of his lower extremities, the clinical findings supported a diagnosis of Pediatrics, Bellevue Hospital Center, New York, New York scurvy. Although scurvy is rare in developed nations, this diagnosis should be Dr Nastro helped conceptualize the case report, considered in a patient with the clinical constellation of lower-extremity pain contributed to writing the introduction, initial presentation, hospital course, and discussion, and or arthralgias, a nonblanching rash, easy bleeding or bruising, fatigue, and developed the laboratory tables while he was anemia. -
Nutrition 102 – Class 3
Nutrition 102 – Class 3 Angel Woolever, RD, CD 1 Nutrition 102 “Introduction to Human Nutrition” second edition Edited by Michael J. Gibney, Susan A. Lanham-New, Aedin Cassidy, and Hester H. Vorster May be purchased online but is not required for the class. 2 Technical Difficulties Contact: Erin Deichman 574.753.1706 [email protected] 3 Questions You may raise your hand and type your question. All questions will be answered at the end of the webinar to save time. 4 Review from Last Week Vitamins E, K, and C What it is Source Function Requirement Absorption Deficiency Toxicity Non-essential compounds Bioflavonoids: Carnitine, Choline, Inositol, Taurine, and Ubiquinone Phytoceuticals 5 Priorities for Today’s Session B Vitamins What they are Source Function Requirement Absorption Deficiency Toxicity 6 7 What Is Vitamin B1 First B Vitamin to be discovered 8 Vitamin B1 Sources Pork – rich source Potatoes Whole-grain cereals Meat Fish 9 Functions of Vitamin B1 Converts carbohydrates into glucose for energy metabolism Strengthens immune system Improves body’s ability to withstand stressful conditions 10 Thiamine Requirements Groups: RDA (mg/day): Infants 0.4 Children 0.7-1.2 Males 1.5 Females 1 Pregnancy 2 Lactation 2 11 Thiamine Absorption Absorbed in the duodenum and proximal jejunum Alcoholics are especially susceptible to thiamine deficiency Excreted in urine, diuresis, and sweat Little storage of thiamine in the body 12 Barriers to Thiamine Absorption Lost into cooking water Unstable to light Exposure to sunlight Destroyed -
Guidelines on Food Fortification with Micronutrients
GUIDELINES ON FOOD FORTIFICATION FORTIFICATION FOOD ON GUIDELINES Interest in micronutrient malnutrition has increased greatly over the last few MICRONUTRIENTS WITH years. One of the main reasons is the realization that micronutrient malnutrition contributes substantially to the global burden of disease. Furthermore, although micronutrient malnutrition is more frequent and severe in the developing world and among disadvantaged populations, it also represents a public health problem in some industrialized countries. Measures to correct micronutrient deficiencies aim at ensuring consumption of a balanced diet that is adequate in every nutrient. Unfortunately, this is far from being achieved everywhere since it requires universal access to adequate food and appropriate dietary habits. Food fortification has the dual advantage of being able to deliver nutrients to large segments of the population without requiring radical changes in food consumption patterns. Drawing on several recent high quality publications and programme experience on the subject, information on food fortification has been critically analysed and then translated into scientifically sound guidelines for application in the field. The main purpose of these guidelines is to assist countries in the design and implementation of appropriate food fortification programmes. They are intended to be a resource for governments and agencies that are currently implementing or considering food fortification, and a source of information for scientists, technologists and the food industry. The guidelines are written from a nutrition and public health perspective, to provide practical guidance on how food fortification should be implemented, monitored and evaluated. They are primarily intended for nutrition-related public health programme managers, but should also be useful to all those working to control micronutrient malnutrition, including the food industry. -
Nl Nov14 Web.Indd
IDD NEWSLETTER NOVEMBER 2014 ID IN CANADA 15 Severe iodine deficiency in a Canadian boy with food allergies Excerpted from: Pacaud D et al. A third world endocrine disease in a 6-year-old North American boy. Journal of Clinical Endocrinology and Metabolism. 1995; 80(9): 2574–2576 A 6-year-old French-Canadian boy was seen for symptoms of goiter and hypothyroidism of acute onset. He was referred to the endo- crinology clinic with a 3-month history of fatigue. Severe asthma and atopic dermatitis had started during infancy. The boy had multiple food allergies. His diet was very restricted and consisted of oat cereal, horse meat, broccoli, sweet potatoes, cauliflower, grapes, apples, and water. His thyroid was diffusely increased in size. Thyroid function tests revealed severe primary hypothyroidism with undetectable antibodies. Physical exam showed several classic signs of hypothyroi- dism: facial edema was noticeable, and the skin felt very dry and was eczematous. Investigations and treatment liz west/flickr, 2012; CC BY 2.0 Normal bone age and growth rate suggested Cruciferous vegetables contain thiocyanate which together with iodine deficiency may lead to goiter that the hypothyroidism was of acute onset. A nutritional investigation showed low Discussion In conclusion, this boy suffered from caloric intake and low urinary iodine levels Since the introduction of iodized table salt goitrous hypothyroidism secondary to severe indicative of severe iodine deficiency. Initial in North America, severe iodine deficiency iodine deficiency and compounded by thi- treatment with levothyroxine resulted in has been practically eradicated. The boy’s ocyanate overload. Thus, even in our envi- the resolution of clinical hypothyroidism, a severe goitrous hypothyroidism was the ronment of relative iodine abundance, IDD reduction of thyroid volume, and normali- result of an extremely restricted diet used and possibly other nutritional deficiencies zation of thyroid function tests, but urinary to control severe atopy. -
Rickets: Not a Disease of the Past LINDA S
Rickets: Not a Disease of the Past LINDA S. NIELD, M.D., West Virginia University School of Medicine, Morgantown, West Virginia PRASHANT MAHAJAN, M.D., M.P.H., Wayne State University School of Medicine, Detroit, Michigan APARNA JOSHI, M.D., Children’s Hospital of Michigan, Detroit, Michigan DEEPAK KAMAT, M.D., PH.D., Wayne State University School of Medicine, Detroit, Michigan Rickets develops when growing bones fail to mineralize. In most cases, the diagnosis is established with a thorough his- tory and physical examination and confirmed by laboratory evaluation. Nutritional rickets can be caused by inadequate intake of nutrients (vitamin D in particular); however, it is not uncommon in dark-skinned children who have limited sun exposure and in infants who are breastfed exclusively. Vitamin D–dependent rickets, type I results from abnor- malities in the gene coding for 25(OH)D3-1-a-hydroxylase, and type II results from defective vitamin D receptors. The vitamin D–resistant types are familial hypophosphatemic rickets and hereditary hypophosphatemic rickets with hyper- calciuria. Other causes of rickets include renal disease, medications, and malabsorption syndromes. Nutritional rickets is treated by replacing the deficient nutrient. Mothers who breastfeed exclusively need to be informed of the recom- mendation to give their infants vitamin D supplements beginning in the first two months of life to prevent nutritional rickets. Vitamin D–dependent rickets, type I is treated with vitamin D; management of type II is more challenging. Familial hypophosphatemic rickets is treated with phosphorus and vitamin D, whereas hereditary hypophosphatemic rickets with hypercalciuria is treated with phosphorus alone. -
341 Nutrient Deficiency Or Disease Definition/Cut-Off Value
10/2019 341 Nutrient Deficiency or Disease Definition/Cut-off Value Any currently treated or untreated nutrient deficiency or disease. These include, but are not limited to, Protein Energy Malnutrition, Scurvy, Rickets, Beriberi, Hypocalcemia, Osteomalacia, Vitamin K Deficiency, Pellagra, Xerophthalmia, and Iron Deficiency. Presence of condition diagnosed, documented, or reported by a physician or someone working under a physician’s orders, or as self-reported by applicant/participant/caregiver. See Clarification for more information about self-reporting a diagnosis. Participant Category and Priority Level Category Priority Pregnant Women 1 Breastfeeding Women 1 Non-Breastfeeding Women 6 Infants 1 Children 3 Justification Nutrient deficiencies or diseases can be the result of poor nutritional intake, chronic health conditions, acute health conditions, medications, altered nutrient metabolism, or a combination of these factors, and can impact the levels of both macronutrients and micronutrients in the body. They can lead to alterations in energy metabolism, immune function, cognitive function, bone formation, and/or muscle function, as well as growth and development if the deficiency is present during fetal development and early childhood. The Centers for Disease Control and Prevention (CDC) estimates that less than 10% of the United States population has nutrient deficiencies; however, nutrient deficiencies vary by age, gender, and/or race and ethnicity (1). For certain segments of the population, nutrient deficiencies may be as high as one third of the population (1). Intake patterns of individuals can lead to nutrient inadequacy or nutrient deficiencies among the general population. Intakes of nutrients that are routinely below the Dietary Reference Intakes (DRI) can lead to a decrease in how much of the nutrient is stored in the body and how much is available for biological functions.