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Home , Hyperkalemia, Hypernatremia

Prabi Rajbhandari, MD, FAAP,​a,b​ Chetan Mandelia, MD,​b Halima S. Janjua, MD,b​ Praveen AKumar 9-Day-Old Conjeevaram Selvakumar, MD, b​ WithSangeeta Krishna, Weight MD, FAAPb Loss and abstract

A 9-day-old infant girl presented with diarrheaStreptococcus and weight loss of 19% since birth. She was born via spontaneous vaginal delivery at 39 weeks’ gestation to a mother positive for group B who received adequate intrapartum prophylaxis. The infant was formula-fed every ∼ 2 to 3 hours with no reported issues with feeding or swallowing. The infant had nonmucoid watery stools 5 to 15 times per day. Her family history was significant for hypertrophic cardiomyopathy in several of her family members. Her initial vital signs and physical examination were normal. Laboratory data on hospital admission showed a normal complete blood count, but her chemistry analysis revealed significant aAkron Children’s Hospital, Akron, Ohio; and bCleveland , , , and acute injury. Clinic Children’s, Cleveland, Ohio Her hypernatremia was resistant to fluid management. In this article, we Dr Rajbhandari conceptualized, designed, and discuss the infant’s hospital course, our clinical thought process, and how drafted the initial manuscript, and revised and we arrived at our final diagnosis. critically reviewed it. Dr Mandelia contributed to drafting of the manuscript, in addition to the Case History With Subspecialty acquisition, analysis, and interpretation of data; Input ∼ he also reviewed and revised the manuscript. other distant relatives. She had lost Drs Janjua, Selvakumar, and Krishna contributed Prabi Rajbhandari, MD, FAAP 19% of her birth weight by the day to subsequent drafts of the manuscript, interpreted (Pediatric Hospitalist [Moderator]): of admission (birth weight, 3.35 kg; the data, and reviewed and revised the manuscript. All authors approved the final manuscript as admission weight, 2.72 kg). Her submitted. rate on arrival to the floor was DOI: 10.1542/peds.2016-2953 A 9-day-old female infant was directly 165 beats/min, blood pressure was admitted from her pediatrician’s 94/67 mm Hg, respiratory rate was Accepted for publication Dec 14, 2016 office due to excessive diarrhea with 41 breaths/min, and oxygen saturation Address correspondence to Prabi Rajbhandari, MD, associated weight loss. She was born at was 96% on room air. On examination, FAAP, Akron Children’s Hospital, One Perkins Square, Akron, OH 44308. E-mail: [email protected] 39 weeks to parents who are of Amish she appeared malnourished but not PEDIATRICS (ISSN Numbers: Print, 0031-4005; Online, descent. Birth historyStreptococcus was significant dysmorphic. No other significant 1098-4275). for meconium-stained amniotic fluid findings were noted. Written consent and positive group B for this article was obtained from the Copyright © 2017 by the American Academy of Pediatrics status, which was adequately treated. family.What are the common causes for She was breastfed until day 5 of excessive weight loss in a newborn? FINANCIAL DISCLOSURE: The authors have life and was then switched to cow’s indicated they have no financial relationships Is there anything unusual in the relevant to this article to disclose. milk formula secondary to poor milk ∼ history that will point us to dig supply and poor latch. The infant deeper into her case? FUNDING: No external funding. was formula-fed 2 oz every 2 to 3 POTENTIAL CONFLICT OF INTEREST: The authors hours with no feeding or swallowing Sangeeta Krishna, MD (Pediatric have indicated they have no potential conflicts of ∼ interest to disclose. issues. Bowel movements were loose Hospitalist): to watery, 5 to 15 times daily, with no hematochezia or melena. Her To cite: Rajbhandari P, Mandelia C, Janjua HS, family history was significant for The most common causes of acute and et al. A 9-Day-Old With Weight Loss and Diarrhea. hypertrophic cardiomyopathy in excessive weight loss are inadequate Pediatrics. 2017;139(5):e20162953 her maternal grandfather, maternal feeding and/or acute changes in fluid uncle, maternal aunt, and several balance. Risk factors for inadequate Downloaded from www.aappublications.org/news by guest on September 27, 2021 PEDIATRICS Volume 139, number 5, May 2017:e20162953 DIAGNOSTIC DILEMMAS TABLE 1 Weight, Laboratory Values, and Fluid Management During the Hospitalization Variable HD 1 HD 3 HD 5 HD 7 HD 9 HD 11 feeding include lack of knowledge, improper formula mixing, delayed Weight, kg 2.72 2.9 2.97 3.03 3.05 3.15 , mmol/L 177 182 158 151 142 141 milk supply related to primipara , mmol/L 6.5 5.8 5.0 4.4 4.1 4.5 status, cesarean delivery/excessive Chloride, mmol/L 147 152 123 112 107 104 maternal pain or sedation, and CO2, mmol/L 10.6 10.9 19 23 20 23 older maternal age. Less common SUN, mg/dL 104 134 19 27 8 15 causes include increased metabolic , mg/dL 1.32 1.20 0.66 0.45 0.42 0.40 , mg/dL 78 156 103 92 103 123 demand such as that due to infections , mg/dL 11.8 11.6 10.7 10.8 10.4 9.3 (congenital or acquired), congenital Type of IV Fluids D10 NS + D5 1/2 D5 + 20 mEq No IV Fluids No IV Fluids No IV Fluids malformations such as heart disease, D10 Water + NS With NaHCO3 malrotation, pyloric stenosis, and Deficit 20 KCl PO feeds Cow’s Milk NPO Hypoallergenic Hypoallergenic CFF Plus CFF Plus malabsorption. A detailed history Formula Formula + Formula + Fructose Fructose and examination will likely point in Free Water Free Water the right direction. History should CFF, carbohydrate-free formula; CO2, carbon dioxide; D5, 5% dextrose; D10, 10% dextrose; IV, intravenous; KCl, potassium include details of pregnancy and chloride; NaHCO3, ; NS, 0.9% ; PO, oral; SUN, urea nitrogen. perinatal course, , tachypnea, diaphoresis, vomiting, diarrhea, , feeding behaviors, irritability, emesis, and social issues. The examination should focus on 10.6 mmol/L; , and ineffective breastfeeding. signs of , jaundice, 104 mg/dL; and creatinine, Other less common causes include 1.3 mg/dL. With these findings, congenital nephrogenic diabetes dysmorphism, any organomegaly, 2 3 she was transferred to the PICU insipidus,​ feeding goat’s or cow’s and evaluation of suck/swallow and 4 feeding aversions. A broad approach for management of hypernatremic Whatmilk,​ is and the malabsorption. initial management of hypernatremic dehydration? What to a more chronic failure to gain dehydration and acute kidney are the complications and what weight includes identifying causes Isinjury. this clinical picture typical of hypernatremic dehydration? Are precautions should be taken while of inadequate intake, inadequate correcting hypernatremia? absorption and assimilation, or there any other causes for a similar picture? excessive losses. Reviewing the Dr Janjua: newborn screen, obtaining a Halima Janjua, MD (Pediatric complete blood cell count, urinalysis, ): stool testing for fecal fat, reducing In any patient with hypernatremia, sugars, pH, if indicated, the goal is to identify the cause, and a renal and liver function test correct the fluid deficit, and correct Neonates with hypernatremic panel may be preliminary tests if a the hypertonicity along with any dehydration present with weight diagnosis is not clear with the initial other disturbances. loss. Median weight loss reported history and physical examination. Water deficit is calculated from with hypernatremic dehydration 1 the following formula: water The stooling pattern observed in is 19.5%,​ similar to the infant deficit = current total body water × this infant is not normal at this age. under discussion. Neonates with [(serum sodium/140) – 1]. In An infant should typically have 6 to hypernatremic dehydration can acute hypernatremia, the serum 8 soft transitional to seedy-looking present with irritability and agitation sodium level should be corrected yellow stools. Significant diarrhea leading to lethargy and . The at an initial rate of 2 to 3 mEq/L/h, and weight loss in this infant could be clinical picture of hypernatremic whereas chronic hypernatremia related to infection, inflammation, or dehydration differs from other (hypernatremia that has been malabsorption. states of dehydration. In this case, Dr Rajbhandari: present for >48 hours) should be fluids shift from the intracellular to corrected at a rate not to exceed the extracellular space keeping the 0.5 mEq/L/h and a total of 8 to intravascular volume somewhat The patient’s initial evaluation 10 mEq/d. This slow correction is steady. This shift of fluids gives the included a complete blood cell count, chosen to avoid the risk of cerebral skin a “doughy” feel rather than the which was within normal limits. edema during rapid correction. Acute loss of skin turgor that is seen in A metabolic panel was obtained, hypernatremia causes osmotic water other forms of dehydration. which showed the following: sodium, movement out of cells, resulting 177 mmol/L; potassium, 6.5 mmol/L; The 2 most common causes of in shrinkage of these cells. After chloride, 147 mmol/L; bicarbonate, neonatal hypernatremia are diarrhea 2 to 3 days, the brain adjusts and Downloaded from www.aappublications.org/news by guest on September 27, 2021 2 Rajbhandari et al mitigates chronic hypernatremia by Due to resistant hypernatremia, the typically present with vomiting, increasing the intracellular content infant was made nil per oral (NPO) diarrhea, abdominal distension, of organic osmolytes. If extracellular on hospital day (HD) 3 and was and/or bloody stools with associated tonicity is rapidly decreased, water treated with 5% dextrose water with weight loss, although some patients will move into the brain cells, 20 mEq/L of sodium bicarbonate that have a combination of cutaneous, producing , which was later changed to 5% dextrose gastrointestinal, and respiratory may lead to herniation, permanent water to provide additional free symptoms. neurologic deficits, and myelinolysis. water. This approach reduced the CMPA should be considered in Serum electrolyte levels should be frequency of her diarrhea to 4 to this neonate with weight loss and measured frequently, and serial 6 times per day compared with 6 diarrhea. Diagnosis of CMPA is neurologic examinations should be to 12 times while being fed orally. usually made with clinical resolution performed to detect any neurologic Frequent plasma sodium monitoring of symptoms on switching to partially changes. was continued, and her sodium or completely hydrolyzed formula. levels started trending down. Given If the diarrhea continues after In neonates, severe hypernatremic her significant family history, an switching to hypoallergenic formula, dehydration can lead to cerebral echocardiogram was obtained, which other causes for neonatal diarrhea edema, cerebral venous thrombosis, showed normal cardiac systolic should be sought. Our patient did not cerebral hemorrhage, and 5,6​ function with a mildly dilated aortic improve after undergoing the switch infarction. ‍ Nonfocal neurologic root. She was transferred to the in formula; hence, we proceeded with signs or in the setting of regular nursing floor on HD 4. Upon Drfurther Rajbhandari: investigation. hypernatremic dehydration in a arrival to the regular nursing floor, neonate should raise the suspicion the infant’s comprehensive metabolic of cerebral venous thrombosis. The panel was as follows: sodium, 159 What other gastrointestinal causes patient can also have decreased mmol/L; potassium, 4.3 mmol/L; should be taken into consideration levels of consciousness or focal chloride, 126 mmol/L; bicarbonate, when presented with diarrhea with neurologic . 17 mmol/L; blood urea nitrogen, Drweight Selvakumar: loss in a 9-day-old infant? Hypernatremic dehydration can 19 mg/dL; and creatinine, 0.68 also cause aortic and/or renal mg/dL. Although failure to thrive arterial thrombosis. Renal venous can be a manifestation of heart thrombosis, acute renal failure, failure, this factor was unlikely to Etiologies of protracted diarrhea in a newborn are vast, ranging disseminated7 intravascular be the case considering her normal coagulation,​ and thrombosis of echocardiogram. The infant was from self-limited pathologies such as infectious causes (mostly viral extremities8 have also been restarted on oral feedings with cow’s reported. such as rotavirus, adenovirus, or Dr Rajbhandari: milk–based formula; however, her sodium levels started rising again sapovirus) or CMPA to persistent with persistent diarrhea. A possibility diarrheal illnesses such as congenital of cow’s milk protein allergy (CMPA) diarrheal disorders (CDDs). CDDs Our patient was initially treated was considered, and she was then are inherited enteropathies mostly with 10% dextrose in 0.9% saline switched over to hypoallergenic related to genetic defects with an autosomal recessive trait. These calculated for maintenance along Isformula hypernatremia on HD 5 ( Tablecommon 1). in CMPA? enteropathies lead to severe with replacement of deficit over How strongly should we consider 72 hours. The goal was to reduce CMPA in a clinical scenario such as diarrhea with massive dehydration the plasma sodium level by 10 to this one? and metabolic acidosis sometimes 12 mmol/L in 24 hours. The infant necessitating total parenteral was monitored frequently, with Praveen Kumar Conjeevaram nutrition. CDDs are broadly classified laboratory values checked every Selvakumar, MD (Pediatric into 4 groups in relation to the 4 hours. After 48 hours, fluids were Gastroenterology): defect: absorption and transport of changed to 5% dextrose in 0.45% nutrients and electrolytes, enterocyte saline with 20 mmol/L of potassium differentiation and polarization, chloride along with formula. She CMPA is the most common food enteroendocrine cell differentiation, and modulation of the intestinal did not experience vomiting; allergy in early childhood, with a 9 ∼ 4 however, she continued to have loose prevalence of 2% to 7.5%. Most immune response (Table 2). stools 6 to 12 times per day. Her infants develop symptoms within Diarrhea can be osmotic in hypernatremia persisted at 160 to 1 week after introduction of cow’s case of malabsorption, in which 180 mmol/L. milk–based formula. Children excess of nutrients in the bowel Downloaded from www.aappublications.org/news by guest on September 27, 2021 PEDIATRICS Volume 139, number 5, May 2017 3 TABLE 2 Differential Diagnoses for Protracted Diarrhea in a Newborn Differential Diagnoses trichohepatoenteric syndrome, or fecal sodium levels >140 mmol/L, infections such as cholera. Lysinuric metabolic acidosis and alkaline stools Infectious Rotavirus protein intolerance, caused by a are suggestive of congenital sodium Adenovirus defect in the cationic amino acid diarrhea. These children may need Sapovirus transporter, can cause chronic an esophagogastroduodenoscopy or CMPA diarrhea and failure to thrive in colonoscopy to assess for mucosal Defect in absorption of carbohydrates addition to other manifestations pathologies such as inflammatory Congenital sucrase-isomaltase deficiency GGM such as hyperammonemia, infiltrate (immune dysregulation, Congenital lactase deficiency osteopenia, and renal disease. polyendocrinopathy, enteropathy, Defect in absorption and transport of amino Disorders of lipid trafficking X-linked syndrome) or lipid-filled acids such as chylomicron retention enterocytes (lipid-trafficking Lysinuric protein intolerance disease, hypolipoproteinemia, diseases) or villous atrophy Defect in lipid trafficking Chylomicron retention disease or abetalipoproteinemia can (microvillus inclusion disease), as Hypolipoproteinemia also cause chronic steatorrhea well as to assess disaccharidase Abetalipoproteinemia in addition to severe fat-soluble activities in the small intestine Pancreatic insufficiency vitamin deficiencies. Pancreatic (congenital sucrase-isomaltase Cystic fibrosis insufficiency such as cystic fibrosis, deficiency). Molecular testing might Shwachman-Diamond syndrome Johanson-Blizzard syndrome Shwachman-Diamond syndrome, be needed to confirm the diagnosis Congenital lipase Johanson-Blizzard syndrome, and in some of the CDDs. Management Enterokinase deficiency congenital lipase or enterokinase strategies depend on the etiology, Defect in absorption and transport of deficiency should also be considered which includes carbohydrate-free electrolytes in young infants with chronic formula, parenteral nutrition in Congenital sodium diarrhea Congenital chloride diarrhea diarrhea. Immune dysregulation, secretory diarrheas, and vitamin or polyendocrinopathy, enteropathy, pancreatic enzyme supplementation. Defect in immune dysregulation Dr Rajbhandari: IPEX syndrome X-linked (IPEX) syndrome is Defect in enterocyte differentiation an X-linked disease that causes Microvillus inclusion disease persistent infantile diarrhea, Tufting enteropathy Trichohepatoenteric syndrome endocrinopathy, and dermatitis. Our patient was started on a trial Defect in enteroendocrine cell differentiation Enteroendocrine cell differentiation of hypoallergenic formula. Her Enteric anendocrinosis disorders that could cause chronic diarrhea continued while she was ∼ Mitchell-Riley syndrome diarrhea are enteric anendocrinosis, on this formula, and her sodium IPEX, immune dysregulation, polyendocrinopathy, Mitchell-Riley syndrome, or level persisted at 150 mmol/L. enteropathy, X-linked. proprotein convertase 1/3 A trial of NPO was given on HD 8 deficiency; most of these disorders to determine if the diarrhea was are associated with endocrinopathies. secretory or osmotic in nature. Intravenous fluids were adjusted and lumen increases the osmolarity, Initial investigations in a newborn electrolyte levels were monitored thereby leading to increased or young infant with protracted closely during this time. The patient water absorption in the colon, or diarrhea should include assessment showed improvement while being secretory, in which electrolyte of stool electrolytes, pH, fat, and NPO, suggesting osmotic diarrhea. absorption or secretion is impaired. reducing substances. A trial of fasting Additional stool studies, including Osmotic diarrhea in a newborn or can be performed to determine if the stool pH, electrolytes, and reducing young infant is usually caused by diarrhea is secretory or osmotic, and substances, were obtained. Results of disorders involving malabsorption this trial was done for our patient. the stool studies revealed low fecal of carbohydrates such as congenital A marked decrease in stool output sodium levels and a high osmolar gap, sucrase-isomaltase deficiency, during fasting and high stool osmolar confirming osmolar diarrhea; stool- glucose-galactose malabsorption gap (>100 mosm/kg) suggests an reducing substances were positive. (GGM), or congenital lactase osmotic diarrhea. In particular, low An esophagogastroduodenoscopy deficiency; secretory diarrheas stool pH, high stool osmolarity, and with biopsy was performed on HD 9; include conditions such as congenital the presence of reducing substances the results were visually normal with chloride diarrhea, congenital sodium in the stool indicate carbohydrate normal histologic findings. Duodenal diarrhea, disorders of villous malabsorption. Fecal chloride levels biopsy specimens were also sent out architecture such as microvillus >90 mmol/L, metabolic alkalosis, for assessment of disaccharidase inclusion disease, intestinal epithelial and acidic stools might indicate activity, which was later reported as dysplasia (tufting enteropathy), congenital chloride diarrhea, whereas normal. Downloaded from www.aappublications.org/news by guest on September 27, 2021 4 Rajbhandari et al TABLE 3 Patient’s Weight, Percentile, and Feedings on Follow-up Visits Age Weight (Percentile) Feeds On HD 9, the patient was switched to a carbohydrate-free formula with Birth weight 3.35 kg (60th percentile) Breastfeeding + cow’s milk formula 1–3 wk 2.72–3.15 kg (5–15th percentile) (Hospitalization period), discharged on carbohydrate- additional fructose. She improved free formula with this regimen, and her sodium 3 mo 4.9 kg (7th percentile) Carbohydrate-free formula + fructose (20–24 oz/d) Whylevel and normalized. how did we decide on 6 mo 6.6 kg (22nd percentile) Solids added + carbohydrate-free formula continued carbohydrate-free formula? 9 mo 7.7 kg (40th percentile) Solids added + carbohydrate-free formula continued 1 y 9.4 kg (60th percentile) Regular table foods + carbohydrate-free formula Dr Selvakumar:

Deficiency of enzymes in cases This variant has been previously10 improves upon cessation of formula of congenital sucrose-isomaltase reported in patients with GGM. containing glucose, galactose, deficiency or congenital lactase Final Diagnosis and Discussion sucrose, and lactose but resumes deficiency or defective transporter when these sugars are reintroduced. such as GGM can cause carbohydrate Diarrhea can also worsen with the malabsorption in young infants. use of oral rehydration solution Because of the child’s Amish descent, GGM is a rare autosomal recessive because it contains glucose. normal duodenal histology, and disorder caused by a defect in the positive stool-reducing substances, SLC5A1 gene encoding the intestinal The diagnosis of GGM can be we suspected GGM in this child. We sodium/glucose11,12​ transporter reliably established with resolution therefore initiated carbohydrate- SGLT1. ‍ This transporter defect of diarrhea after switching to a free formula, which resulted in results in defective sodium-coupled carbohydrate-free formula such resolution of the diarrhea. Fructose transport of glucose and galactose as RCF formula (Abbott Nutrition, was added to this regimen to across the intestinal brush border, Columbus, OH). Fortification of meet energy requirements, which leading to malabsorption of glucose calories can be accomplished by she tolerated well. Therefore, a and galactose. Since the first report adding fructose because absorption presumptive diagnosis of GGM was of GGM in 1962,12, >4013​ mutations have of fructose is normal in these Drmade. Rajbhandari: been described. ‍ There is no children. The hydrogen breath test established prevalence because there can also be used in the diagnosis are only a few reported cases in the of GGM. Oral administration of After starting the carbohydrate-free literature. This rarity of occurrence glucose or galactose (2 g/kg) formula, the patient’s sodium level with possible severe clinical picture results in elevation of breath normalized along with significant at presentation makes it a challenging hydrogen to >20 ppm above 15 improvement in diarrhea. She was entity for physicians with regards to baseline in patients with GGM. observed in the hospital for another diagnosis and management. A fairly However, this test is less practical 2 days and was discharged from the large cohort of patients with GGM10 to administer in young infants. A hospital on the same formula have been14 reported from Amish flat serum glucose response curve (12 kcal/oz) with added fructose and Arab populations. to oral administration of glucose (total 20 kcal/oz). The patient or galactose can also16 be used in the continued to do well and caught The clinical presentation is usually diagnosis of GGM. Diagnosis can be up on her growth curve well. with protracted diarrhea leading confirmed with molecular analysis Electrolyte levels and renal function to dehydration, metabolic acidosis, of the SLC5A1 gene. were within normal limits at hypernatremia, and weight loss follow-up visits (Table 3). within the first week of life. Patients with GGM are treated with Genetic testing was sent which later Diarrhea usually begins on day lifetime restriction of glucose and confirmed the diagnosis. Sequence 2 to 3 of life once breastfeeding galactose, with only a marginal analysis of the SLC5A1 gene or formula feeding is started. improvement in tolerance at older identified a homozygous c.1673G>A Although symptoms begin early ages. Calories should be provided (p. R558H) variant in our patient. in life, diagnosis may be delayed. mostly in the form of fats, proteins, This variant was a guanine to adenine Studies have reported a mean and fructose. These patients should single nucleotide substitution in exon age of diagnosis of 4.5 months. also receive adequate calcium and 14 of the SLC5A1 gene, resulting in The diarrhea in GGM is osmotic, vitamin D supplementation due replacement of an codon caused by accumulation of to lack of dairy intake. Glucose- (CGT) with a histidine codon (CAT) at unabsorbed glucose and galactose containing suspensions should be amino acid position 558 (p.R558H). in the intestinal lumen. Diarrhea avoided in these children. Downloaded from www.aappublications.org/news by guest on September 27, 2021 PEDIATRICS Volume 139, number 5, May 2017 5 5. Staub E, Wilkins B. A fatal case of of Old Order Amish. Clin Genet. Abbreviations hypernatraemic dehydration in a 2011;79(1):86–91 neonate. J Paediatr Child Health. 11. Mart n MG, Turk E, Lostao MP, Kerner 2012;48(9):859–862 í C, Wright EM. Defects in Na+/glucose CDD: congenital diarrheal 6. Hbibi M, Abourazzak S, Babakhouya cotransporter (SGLT1) trafficking disorder A. Severe hypernatremic dehydration and function cause glucose- CMPA: cow’s milk protein allergy associated with cerebral venous and galactose malabsorption. Nat Genet. GGM: glucose-galactose aortic thrombosis in the neonatal 1996;12(2):216–220 malabsorption period. BMJ Case Rep. 2012;2012 12. Lam JT, Mart n MG, Turk E, et al. HD: hospital day í Missense mutations in SGLT1 cause NPO: nil per os 7. Unal S, Arhan E, Kara N, Uncu N, Aliefendioğlu D. Breast-feeding- glucose-galactose malabsorption by associated hypernatremia: trafficking defects. Biochim Biophys References retrospective analysis of 169 Acta. 1999;1453(2):297–303 term newborns. Pediatr Int. 1. Oddie SJ, Craven V, Deakin K, 13. W right EM, Turk E, Martin MG. 2008;50(1):29–34 Westman J, Scally A. Severe neonatal Molecular basis for glucose-galactose hypernatraemia: a population based 8. Amitai I, Goder K, Husseini N, Rousso malabsorption. Cell Biochem Biophys. study. Arch Dis Child Fetal Neonatal Ed. M. Hypernatremic dehydration 2002;36(2–3):115–121 2013;98(5):F384–F387 complicated by peripheral 14. Saadah OI, Alghamdi SA, Sindi HH, 2. Linshaw MA. Back to basics: congenital gangrene in infancy. Isr J Med Sci. Alhunaitti H, Bin-Taleb YY, Alhussaini nephrogenic . 1983;19(6):538–540 BH. Congenital glucose-galactose malabsorption: a descriptive study of Pediatr Rev. 2007;28(10):372–380 9. berni Canani R, Terrin G, Cardillo G, clinical characteristics and outcome 3. basnet S, Schneider M, Gazit A, Tomaiuolo R, Castaldo G. Congenital from Western Saudi Arabia. Arab J Mander G, Doctor A. Fresh goat’s milk diarrheal disorders: improved Gastroenterol. 2014;15(1):21–23 for infants: myths and realities—a understanding of gene defects is review. Pediatrics. 2010;125(4):www.​ leading to advances in intestinal 15. W right EM. I. Glucose galactose pediatrics.​org/​cgi/​content/​full/​125/​4/​ physiology and clinical management. malabsorption. Am J Physiol. e973 J Pediatr Gastroenterol Nutr. 1998;275(5 pt 1):G879–G882 4. Vandenplas Y, Koletzko S, Isolauri E, 2010;50(4):360–366 16. Pahari A, Milla PJ, van’t Hoff WG. et al. Guidelines for the diagnosis and 10. Xin B, Wang H. Multiple sequence Neonatal nephrocalcinosis in management of cow’s milk protein variations in SLC5A1 gene are association with glucose-galactose allergy in infants. Arch Dis Child. associated with glucose-galactose malabsorption. Pediatr Nephrol. 2007;92(10):902–908 malabsorption in a large cohort 2003;18(7):700–702

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Updated Information & including high resolution figures, can be found at: Services http://pediatrics.aappublications.org/content/139/5/e20162953 References This article cites 15 articles, 4 of which you can access for free at: http://pediatrics.aappublications.org/content/139/5/e20162953#BIBL Subspecialty Collections This article, along with others on similar topics, appears in the following collection(s): Fetus/Newborn Infant http://www.aappublications.org/cgi/collection/fetus:newborn_infant_ sub Gastroenterology http://www.aappublications.org/cgi/collection/gastroenterology_sub Permissions & Licensing Information about reproducing this article in parts (figures, tables) or in its entirety can be found online at: http://www.aappublications.org/site/misc/Permissions.xhtml Reprints Information about ordering reprints can be found online: http://www.aappublications.org/site/misc/reprints.xhtml

Downloaded from www.aappublications.org/news by guest on September 27, 2021 A 9-Day-Old With Weight Loss and Diarrhea Prabi Rajbhandari, Chetan Mandelia, Halima S. Janjua, Praveen Kumar Conjeevaram Selvakumar and Sangeeta Krishna Pediatrics 2017;139; DOI: 10.1542/peds.2016-2953 originally published online April 11, 2017;

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Pediatrics is the official journal of the American Academy of Pediatrics. A monthly publication, it has been published continuously since 1948. Pediatrics is owned, published, and trademarked by the American Academy of Pediatrics, 345 Park Avenue, Itasca, Illinois, 60143. Copyright © 2017 by the American Academy of Pediatrics. All rights reserved. Print ISSN: 1073-0397.

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