[Early manifestations of Tangier disease].

Background: The diagnosis of Tangier disease in childhood is based on the specific aspect of tonsils or by screening relatives of affected subjects.

Case Report: A moderately enlarged liver associated with splenomegaly was found upon routine physical examination of a 3 month-old breast-fed boy, born in Turkey from consanguineous parents. Laboratory studies disclosed moderate increase in serum alanine aminotransferase activity (ALAT 52 UI/l, N < 30).

[Percutaneous endoscopic gastrostomy in pediatric practice].

Percutaneous endoscopic gastrostomy (PEG) has become a good alternative to nasogastric tube feeding and surgical gastrostomy. The procedure requires two practitioners, one of them being an endoscopist. The technique can be performed either under local or general anesthesia, at bedside or in an operating room or endoscopic room, using a one step button or first setting of a catheter and secondly a button, by pull technique in which the button is pulled through esophagus or push technique were the button is pushed through the parietal wall.

Familial autoimmune enteropathy with circulating anti-bullous pemphigoid antibodies and chronic autoimmune hepatitis.

In a family of four children (two boys and two girls), the two brothers had severe, protracted watery diarrhea beginning at 2 and 3 weeks of life, respectively. Duodenal mucosa in both patients showed total villous atrophy and severe inflammatory infiltration of the entire bowel. The first patient also had lymphoid cell infiltration of the pancreas and died at 6 weeks of age.

CD23/CD21 interaction is required for presentation of soluble protein antigen by lymphoblastoid B cell lines to specific CD4+ T cell clones.

Previous studies have documented a role for membrane-bound CD23 (the low affinity Fc epsilon RII) in presentation of alloantigens by B cells. The aim of the present study was to examine the involvement of cell surface CD23 in presentation of more conventional soluble protein antigens to T cells. We show that antibodies to CD23 and to its lymphocyte-associated second ligand, CD21, inhibit presentation of the cow's milk allergen casein, by autologous CD23+CD21+ B-EBV cell lines to casein-specific HLA-DP-restricted CD4+ T cell clones obtained from patients with either reaginic or enterophatic forms of cow's milk protein intolerance.

[Surveillance of children with liver transplantation].

The benefit of liver transplantation in children with end-stage liver disease is now well established. About 80% of the children are alive 5 years after liver transplantation. These good results are obtained not only because of the improvements of the surgical techniques, but also secondary to the reffinements of the follow-up of the patients.

[Home parenteral nutrition in children: practical modalities].

Today, the duration of parenteral nutrition (PN) is unlimited. PN is used in digestive tract chronic disease (the digestive tract is either unusable or is at rest) or in oncology, hematology and renutrition before transplantation. Thanks to technical advances, PN, although sophisticated, may be applied at home if an active involvement of one parent is obtained.

[Common bilio-pancreatic channel. Apropos of a case with intermittent dilatation of the biliary tracts].

Background: A common biliopancreatic channel can be revealed by cholestatic episodes. Its early removal avoids the development of liver cirrhosis and the risk of carcinomatous change.

Case Report: A 2 year 9 month-old girl had suffered from jaundice plus dark urine and pale stools for 2 months.

Recombinant human G-CSF (Lenograstim) for infectious complications in glycogen storage disease type Ib. Report of 7 cases.

Seven patients with glycogen storage disease type Ib suffering from severe and/or recurrent bacterial infections were treated with glycosylated recombinant G-CSF (Lenograstim). Mean follow up was 20.8 months (range 9-30 months).

Treatment with lenograstim (glycosylated recombinant human granulocyte colony-stimulating factor) and orthotopic liver transplantation for glycogen storage disease type Ib.

A 7 1/2-year-old patient with glycogenosis type lb had chronic neutropenia, recurrent bacterial infections, and severe hypoglycemia in spite of continuous nasogastric feeding. She was treated with lenograstim (glycosylated recombinant human granulocyte colony-stimulating factor) and orthotopic liver transplantation. Absolute neutrophil counts and platelet counts rapidly reached normal values, and infectious episodes were reduced.

Intestinal epithelial cells express the CD23/Fc epsilon RII molecule: enhanced expression in enteropathies.

Immunohistochemical analysis of normal human intestine revealed that two anti-CD23 monoclonal antibodies (mAb), EBVCS 1 and EBVCS 2, reacted with human intestinal epithelial cells. Both mAb exhibited an exclusive reactivity with epithelial cells of the small and large bowels. Staining with both EBVCS 1 and EBVCS 2 was localized on the apical and basal sides of enterocytes.

[CD23: structures, functions and practical perspectives in allergy reactions].

CD23, the low-affinity receptor for IgE, is a surface cell marker and surface CD23 is cleaved into soluble fragments by an autocatalytic mechanism. cDNA clone encoding for CD23 has been isolated and structural studies realized. CD23 is expressed by B and T lymphocytes and by other cells including tumor cells.

[Benign recurrent cholestasis. Apropos of a case with normal serum gamma-glutamyl transpeptidase level].

We report a case of intrahepatic cholestatis with normal serum levels of gamma GT who was similar to the benign recurrent cholestasis (BRC). The diagnosis of cholestasis in infants may be difficult and the distinction between BRC and Byler's disease should be made as early as possible.

[Neonatal gastric necrosis].

Background: Neonatal necrotizing gastritis is rare. It is usually a complication of acute anoxia or shock and its diagnosis is essentially radiological.

Case Reports: Case no.

Benign recurrent cholestasis with normal gamma-glutamyl-transpeptidase activity.

We report two observations of intrahepatic cholestasis with normal serum levels of gamma-glutamyl-transpeptidase. These cases fit the diagnostic criteria of benign recurrent cholestasis and show that it, like Byler disease, is another form of pediatric intrahepatic cholestasis with a normal gamma-glutamyl-transpeptidase level in the infant.

[Familial infantile cortical hyperostosis (Caffey's disease) with osteolytic lesions of the skull].

Background: The parietal and frontal bones are rarely affected in infantile cortical hyperostosis.

Case Reports: Case n. 1: A 14-day-old boy developed a swelling of the left eyelid that extended to the face in a few days.