ABSTRACT
BACKGROUND: Autoimmune hepatitis (AIH) and cryptogenic chronic hepatitis (CCH) are important causes of liver failure in children, frequently necessitating orthotopic liver transplantation (OLT). The aim of this study is to review disease progression and potential differences between subgroups of children with AIH and CCH. METHODS: The medical records of 65 children diagnosed with AIH or CCH between 1980 and 1998 were evaluated. RESULTS: The median age at presentation was 9 years, 8 months (range 4 months-19 years), and the median follow-up period was 8 years (range 3 months-18 years, 10 months). Forty-one patients (63%) were female. Twenty-eight patients were Hispanic, 28 were Caucasian, 8 were African-American, and 1 was Asian. Forty-three patients (66%) were diagnosed with type 1 AIH, 8 (12%) with type 2 AIH, and 14 (22%) with CCH. Forty patients (62%) underwent OLT (51% of those with type 1 AIH, 75% of those with type 2 AIH, and 86% of those with CCH). Thirteen (33%) of the transplanted patients experienced disease recurrence. African-American patients experienced a significantly higher rate of disease recurrence post-OLT than did Hispanic patients. Seven patients (11%) died, two without OLT, and five posttransplantation. CONCLUSIONS: AIH and CCH frequently necessitate OLT in children. CCH is a more aggressive disease than Type 1 AIH among children with these disorders. Ethnicity influences the rate of disease recurrence after liver transplantation.
Subject(s)
Hepatitis, Autoimmune/surgery , Hepatitis, Chronic/surgery , Liver Transplantation , Adolescent , Adult , Child , Child, Preschool , Ethnicity , Female , Hepatitis, Autoimmune/classification , Humans , Infant , Male , Recurrence , Treatment OutcomeABSTRACT
The interstitial cells of Cajal complex within the gut wall function as a pacemaker to direct peristalsis. Their neoplastic counterpart is the gastrointestinal pacemaker cell tumor, a spindle and/or epithelioid cell mesenchymal tumor previously known as gastrointestinal stromal tumor or incorrectly called leiomyosarcoma in some cases of older reports. Although numerous cases of gastrointestinal leiomyosarcomas have been documented in the English-language literature, no pediatric case of gastrointestinal stromal tumor or gastrointestinal pacemaker cell tumor has, to our knowledge, been recorded. Herein, we report a case of congenital gastrointestinal pacemaker cell tumor confirmed by immunohistochemistry and electron microscopy in a full-term male newborn.
Subject(s)
Biological Clocks , Jejunal Neoplasms/congenital , Jejunal Neoplasms/pathology , Stromal Cells/pathology , Biomarkers, Tumor/metabolism , Humans , Immunohistochemistry , Infant, Newborn , Jejunal Neoplasms/metabolism , Male , Microscopy, ElectronABSTRACT
Biopsies of the jejunum from 61 children, 6 months-4 years of age, including 35 with allergy to corn protein, 18 with celiac disease and 8 children without stomach and intestine pathology were studied. Biopsies were fixed in 10% formalin or in Carnois fixative, the sections were stained with hematoxylin and eosin, 0.5% toluidine blue (pH 0.5), and immunoperoxidase method was used for revealing IgA. The structure of the mucosa was not changed in the allergic patients, the content of the granulated and ungranulated labrocytes was increased, their number being decreased in the elimination diet. The number of plasma cells was diminished, and in only few of them IgA was found in children with severe alimentary malabsorption. A selective deficiency of IgA was diagnosed which determines the therapy of such children. Hyperregenerative atrophy of the mucous membrane and predominance of degranulated cells over undergranulated were noted in celiac disease.
Subject(s)
Dietary Proteins/adverse effects , Edible Grain/adverse effects , Food Hypersensitivity/pathology , Intestinal Mucosa/pathology , Plant Proteins/adverse effects , Biopsy , Celiac Disease/etiology , Celiac Disease/pathology , Child, Preschool , Humans , Immunoenzyme Techniques , Immunoglobulin A/analysis , Infant , Jejunum/pathology , Malabsorption Syndromes/immunology , Malabsorption Syndromes/pathologyABSTRACT
Children with food allergy underwent allergologic and gastroenterologic examinations using esophagogastroduodenojejunoscopy. Impairment of the jejunum in the form of jejunitis associated with function abnormality as shown by the loading tests was revealed. It is concluded that the given patients: group should be examined for the morphofunctional status of the upper gastrointestinal tract.
Subject(s)
Food Hypersensitivity/pathology , Jejunal Diseases/pathology , Jejunum/pathology , Adolescent , Child , Child, Preschool , Enteritis/etiology , Enteritis/pathology , Enteritis/physiopathology , Female , Food Hypersensitivity/complications , Food Hypersensitivity/physiopathology , Humans , Infant , Intestinal Absorption/physiology , Jejunal Diseases/etiology , Jejunal Diseases/physiopathology , Jejunum/physiopathology , MaleABSTRACT
Assessment of the clinico-anamnestic and morphofunctional parameters in children with celiac disease made it possible to define criteria for the acuity of the pathological process, enabling the diagnosis of the disease phase. The data obtained as a result of morphometric examination of the biopsy material withdrawn from the small intestinal mucosa turned out to be of paramount importance.
Subject(s)
Celiac Disease/diagnosis , Child, Preschool , Diagnosis, Differential , Endoscopy, Gastrointestinal , Female , Humans , Infant , Jejunum/pathology , Lactose Intolerance/diagnosis , Male , Severity of Illness IndexABSTRACT
The possibilities of combined enteroscopy in children with suspected surgical diseases of different parts of the small intestine are evaluated. Examination of the jejunum 40-150 cm below the Treitz ligament by means of a standard pediatric fibroscope followed by retrograde ileoscopy allowed an exhaustive diagnosis to be established (including localization of the pathological process) in 18 of 19 children who were operated on. No hyperdiagnosis of surgical diseases occurred. Complications of enteroscopy were not registered.
