1/8. Congenital intractable diarrhea with possible defective crypt regeneration: report of a case.A male infant, born uneventfully from a consanguinous marriage, presented with intractable watery diarrhea from his third day of life, with subsequent malnutrition and failure to thrive. He received central parenteral nutrition beginning at three months of age after a poor response to a semielemental diet and peripheral parenteral nutrition. He was totally dependent on central parenteral nutrition thereafter. Although diarrhea disappeared with strict bowel rest, intolerance to minimal enteral feedings persisted throughout his 2 years 4 months of life. Investigations including stool examinations and repeated cultures, immune function studies, radiologic studies of the small bowel and screening for galactosemia and cystic fibrosis could not demonstrate a specific cause for the diarrhea. Repeated small intestinal biopsies at 1 month, 4 months and 1 year 5 months of age showed persistent villous atrophy with crypt hypoplasia and a low crypt mitotic index. Electron microscopic examination revealed normal-appearing microvilli. This child may have had a congenital enteropathy due to an inborn crypt regeneration defect causing lifelong intolerance to enteral feedings.- - - - - - - - - - ranking = 1keywords = malnutrition (Clic here for more details about this article) |
2/8. Necrotizing enterocolitis: an overlooked life-threatening complication of acute diarrhoeal illness in infants and children.A 7-month-old girl developed necrotizing enterocolitis with extensive pneumatosis intestinalis following an acute diarrhoeal illness. This was preceded by the use of an anti-motility drug and repeated attacks of paralytic ileus. It is noteworthy that all the published cases of necrotizing enterocolitis following acute diarrhoeal illness in infants and children are from tropical countries. This might be related to the use of anti-diarrhoeal drugs and associated malnutrition resulting in hypokalaemia and hypoalbuminaemia.- - - - - - - - - - ranking = 1keywords = malnutrition (Clic here for more details about this article) |
3/8. Congenital diabetes mellitus and fatal secretory diarrhea in two infants.Two unrelated male infants presented with brittle insulin-dependent diabetes mellitus in the first days of life. Subsequently they each developed severe secretory diarrhea, with stool volumes of more than 100 ml/kg/day. Extensive biochemical and serological investigation failed to reveal the etiology of the diarrhea. The infants, cared for at different institutions, underwent therapeutic trials of various agents including loperamide, cholestyramine, prednisone, indomethacin, and somatostatin analogue, without response. Both infants succumbed to septicemia and malnutrition related to diarrhea and poor control of glycemia. At autopsy, both were found to have absence of islets of langerhans in the pancreas, and diffuse dysplastic changes in small and large intestinal mucosae. In particular, the entire alimentary tract in each case was lined by epithelia most typical of foregut mucosa: secretory-type glands, absent crypts of Lieberkuhn, and absent villi. These cases are contrasted with previously-reported infants with congenital diabetes mellitus, and the possible interrelation of these two highly unusual findings, congenital diabetes mellitus and diffuse intestinal dysplasia, is examined.- - - - - - - - - - ranking = 1keywords = malnutrition (Clic here for more details about this article) |
4/8. aluminum hydroxide in the symptomatic treatment of infants with chronic diarrhea.Two neonates with milk intolerance and two older infants, one with acute gastroenteritis and the other with prolonged malnutrition, developed chronic diarrhea. Despite bowel rest, total parenteral nutrition, and alterations in the protein and carbohydrate content of the milk preparations used, every attempt to feed them resulted in diarrhea. All four patients were given aluminum hydroxide because of its bile salt-binding activity and its tendency to cause constipation. Coincident with its administration the diarrhea stopped and the enteral feedings were successfully reinstituted. The only complication seen was the development of constipation in two of the infants; this responded to lowering of the dose of the aluminum hydroxide.- - - - - - - - - - ranking = 1keywords = malnutrition (Clic here for more details about this article) |
5/8. Pneumococcal meningitis following parenteral alimentation in infants.Three young infants with protracted diarrhea and malnutrition were successfully treated by means of intravenous nutrition, which included infusions of fat emulsion (Intralipid) and of fresh frozen plasma. Three to five weeks after termination of intravenous feeding, and after full recovery, they developed pneumococcal septicemia and meningitis. One infant died, and postmortem examination showed diffuse deposition of "intravenous fat pigment" in hepatocytes and in reticuloendothelial cells. In the two infants who recovered, follow-up studies did not disclose any primary derangement of immunologic function. We propose that the infused fat may have caused a temporary depression of immunologic defense mechanisms, predisposing these infants to the pneumococcal infection; the delay in onset of the infection might be attributed to a short-lasting protective effect of fresh frozen plasma which was included in the intravenous feeding regimen.- - - - - - - - - - ranking = 1keywords = malnutrition (Clic here for more details about this article) |
6/8. salmonella gastroenteritis in the first three months of life. A review of management and complications.Salmonellosis in older children and adults is usually a self-limited disease, but the risk of complications in infants is not well-defined. We performed a retrospective review of 52 patients. 90 days of age or less, seen at the St. Louis Children's Hospital between 1975 and 1981 with stool cultures positive for salmonella. Sixteen were 30 days old or less (neonates), 21 were 31- 60 days of age, and 15 were 61-90 days old. Among patients in whom blood cultures were done initially, bacteremia was most frequent in neonates: 5/11 (45%), compared to 2/18 (11%) in older infants. All seven infants presenting with bacteremia received 10 or more days of antibiotic therapy: yet complications (osteomyelitis, fatal meningitis or chronic diarrhea) developed in three of five neonates and one of two older infants. Complications also developed in seven of 22 patients who initially had negative blood cultures, including two infants in whom sepsis later developed and two infants who required intravenous hyperalimentation because of chronic diarrhea and malnutrition. The group of 23 patients who did not have blood cultures all did well. Salmonellosis is not necessarily a self-limited infection in young infants. Even in the absence of bacteremia, clinicans would appear to be justified in using antimicrobial therapy in infants 3 months of age or les with salmonella gastroenteritis, particularly neonates of older infants with symptoms of dysentery or failure to thrive.- - - - - - - - - - ranking = 1keywords = malnutrition (Clic here for more details about this article) |
7/8. Increase in T cells bearing the gamma/delta receptor associated with lymphoproliferative disease of granular lymphocytes in an infant with intractable diarrhea.A two-year-old infant with intractable diarrhea and lymphoproliferative disease of granular lymphocytes attributed to a persistent cytomegalovirus infection showed an increase in cells bearing the gamma/delta T-cell receptor (TCR), which accounted for approximately 20% of total peripheral blood lymphocytes and 40% of CD3 T cells. Of the gamma/delta TCR cells, two-thirds were double negative (CD4-/CD8-) and the other one-third CD8 positive. The majority of gamma/delta cells were delta TCS 1 positive. The predominance of delta TCS 1 positive cells was also confirmed on biopsy of lymphoid tissues from the colon. After improvement of watery diarrhea and malnutrition following three-month hyperalimentation, the number of gamma/delta TCR cells decreased. The patient subsequently died of pneumonia at the age of 2 years and 11 months. A possible site-specific role for the gamma/delta TCR cells, particularly delta TCS 1 cells, in the human intestine is discussed.- - - - - - - - - - ranking = 1keywords = malnutrition (Clic here for more details about this article) |
8/8. Congenital enterocyte heparan sulphate deficiency with massive albumin loss, secretory diarrhoea, and malnutrition.BACKGROUND: The molecular basis of protein-losing enteropathy is unknown. However it has been shown that sulphated glycosaminoglycans may be important in regulating vascular and renal albumin loss. methods: We describe three baby boys who presented within the first weeks of life with massive enteric protein loss, secretory diarrhoea, and intolerance of enteral feeds. All required total parenteral nutrition and repeated albumin infusions. No cause could be found in any case despite extensive investigations, including small intestinal biopsy sampling, which were repeatedly normal. FINDINGS: By specific histochemistry, we detected gross abnormality in the distribution of small intestinal glycosaminoglycans in all three infants, with complete absence of enterocyte heparan sulphate. The distribution of vascular and lamina propria glycosaminoglycans was, however, normal. INTERPRETATION: The presentation of these infants suggests that enterocyte heparan sulphate is important in normal small intestinal function.- - - - - - - - - - ranking = 4keywords = malnutrition (Clic here for more details about this article) |