A Case of Langerhans Cell Histiocytosis With Multifocal, Single-System GI Tract Involvement and Literature Review.

Langerhans cell histiocytosis (LCH) is the most common histiocytic disorder, characterized by the pathologic clonal proliferation and accumulation of immature Langerhans cells within organs. Multiple organ systems can be affected, resulting in a spectrum of clinical manifestations. Isolated gastrointestinal involvement in LCH is rare and usually presents in childhood as a multisystem disease and usually has poor outcomes.

Do mast cells play a pathogenetic role in neurofibromatosis type 1 and ulcerative colitis?

Concurrent association of neurofibromatosis type I and ulcerative colitis has been reported in one clinical case (Tavakkoli et al., 2009). Although this association may represent a casual finding, a common pathophysiology is postulated.

Mutant neurogenin-3 in congenital malabsorptive diarrhea.

Background: Neurogenin-3 (NEUROG3) is expressed in endocrine progenitor cells and is required for endocrine-cell development in the pancreas and intestine. The NEUROG3 gene (NEUROG3) is therefore a candidate for the cause of a newly discovered autosomal recessive disorder characterized by generalized malabsorption and a paucity of enteroendocrine cells.

Methods: We screened genomic DNA from three unrelated patients with sparse enteroendocrine cells for mutations of NEUROG3.

Role of macrophages and stellate cells in the pathogenesis of veno-occlusive disease: an electron microscopic case study.

Veno-occlusive disease (VOD) is an entity described as a triad of pathologic findings including ascites, tender hepatomegaly, and elevated liver enzymes. The prognosis of patients suffering from VOD is highly variable, ranging from slow resolution to the need for liver transplant. The histopathology of VOD has been described by light and electron microscopy.

Laparoscopic and thoracoscopic esophagomyotomy for children with achalasia.

Background: Minimally invasive esophagomyotomy, consisting of a laparoscopic or thoracoscopic approach, has become a preferred surgical treatment for adults with achalasia. This multicenter study reports on the clinical status of children who have undergone minimally invasive esophagomyotomy for achalasia.

Methods: Symptomatology for achalasia was assessed in 22 pediatric patients who underwent minimally invasive esophagomyotomy for achalasia between 1995 and 2000.

Outcome after intestinal transplantation: results from one center's 9-year experience; discussion 1031-2.

Hypothesis: Outcomes after intestinal transplantation have improved during the past decade with refinements in surgical techniques as well as advances in immunosuppression and antimicrobial therapy.

Design: Retrospective analysis.

Setting: Tertiary care medical center, August 1991 through December 2000.

Orthotopic liver transplantation for autoimmune hepatitis and cryptogenic chronic hepatitis in children.

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.

Pediatric upper gastrointestinal endoscopy: state of the art.

Upper gastrointestinal (GI) endoscopy has become a routine procedure in pediatrics. It has been proven safe, highly informative and cost effective when used in the outpatient setting by experienced endoscopists. This review summarizes the experience in pediatric upper GI endoscopy over the last 30 years.

Congenital gastrointestinal pacemaker cell tumor.

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.

[Mucous membrane of the jejunum in allergy to cereal protein].

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.

[Morphofunctional changes in the small intestine of children with food allergy].

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.

[A comparative evaluation of the results of studies of multi-moment chromatic intubation and cholecystography].

These methods were employed in examinations of 55 patients with various gastrointestinal diseases. The findings of both methods coincided in assessment of gallbladder kinetic function in 36 (66%) patients. In 19 (34%) patients the results did not coincide, in 9 of them multi-moment chromatic duodenal intubation with xylite stimulation has yielded overstated results, and in the remaining 10 patients gallbladder hyperfunction was detected by x-ray examination after egg yolk stimulation.

[Criteria of severity of the pathological process in children with celiac disease].

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.

[Enteroscopy in surgical diseases of the small intestine in children].

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.