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Titolo:
INTESTINAL, PANCREATIC AND HEPATIC INVOLVEMENT IN CARBOHYDRATE-DEFICIENT GLYCOPROTEIN SYNDROME TYPE-I
Autore:
KRISTIANSSON B; BORULF S; CONRADI N; ERLANSONALBERTSSON C; RYD W; STIBLER H;
Indirizzi:
SAHLGRENS UNIV HOSP,DEPT PEDIAT S-41685 GOTHENBURG SWEDEN GOTHENBURG UNIV,DEPT PEDIAT S-41124 GOTHENBURG SWEDEN GOTHENBURG UNIV,DEPT PATHOL S-41124 GOTHENBURG SWEDEN MALMO UNIV HOSP,DEPT PAEDIAT MALMO SWEDEN LUND UNIV,DEPT MOL & CELL BIOL LUND SWEDEN KAROLINSKA HOSP,DEPT NEUROL S-10401 STOCKHOLM SWEDEN
Titolo Testata:
Journal of pediatric gastroenterology and nutrition
fascicolo: 1, volume: 27, anno: 1998,
pagine: 23 - 29
SICI:
0277-2116(1998)27:1<23:IPAHII>2.0.ZU;2-Y
Fonte:
ISI
Lingua:
ENG
Soggetto:
DISORDERS; DISEASE;
Keywords:
CARBOHYDRATE-DEFICIENT GLYCOPROTEIN SYNDROME TYPE I; FAILURE TO THRIVE; INFANCY; INTESTINE; LIVER; PANCREAS;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Science Citation Index Expanded
Science Citation Index Expanded
Citazioni:
32
Recensione:
Indirizzi per estratti:
Citazione:
B. Kristiansson et al., "INTESTINAL, PANCREATIC AND HEPATIC INVOLVEMENT IN CARBOHYDRATE-DEFICIENT GLYCOPROTEIN SYNDROME TYPE-I", Journal of pediatric gastroenterology and nutrition, 27(1), 1998, pp. 23-29

Abstract

Background: Children with carbohydrate-deficient glycoprotein syndrome type I during infancy have gastrointestinal symptoms and growth impairment, the cause of which is largely unknown. Methods: Seven childrenwere investigated with small intestinal biopsy, liver biopsy, duodenal intubation with determination of lipolytic and proteolytic activity,and test meal. Weight, length-height, and head circumference were recorded regularly. Results: Growth was affected from early infancy, withan initial low rate of weight gain followed by impaired linear growth. Vomiting and diarrhea were dominant symptoms. Four of seven childrenhad abnormal findings in light microscopic examination of small intestinal biopsy specimens, with short villi and increased inflammatory cells in the stroma, that did not respond to elimination of such food proteins as cow's milk or gluten. Electron microscopic study showed dilatation of smooth endoplasmic reticulum and abnormal inclusions containing lipids. The liver was abnormal in all. Besides steatosis and fibrosis or cirrhosis, there was a remarkable increase of inflammatory cells in portal zones. Activity of lipolytic enzymes in duodenal juice waslow, except in one child, who no longer had growth problems or symptoms. Two of six had abnormal proteinolytic activity in duodenal juice. Digestion of triglycerides and absorption were within normal limits, as was the absorption of glucose and xylose. Conclusions: Inflammation of small intestine and liver may be the cause of gastrointestinal symptoms. In all Likelihood, the growth failure was because of low caloricintake and increased losses related to vomiting. Growth and gastrointestinal symptoms improved spontaneously as time elapsed. (C) 1998 Lippincott-Raven Publishers.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 02/12/20 alle ore 18:37:39