Screening for Lysosomal Acid Lipase Deficiency in a Lipid Clinic.

Background: Lysosomal acid lipase deficiency (LAL-D) is a rare autosomal recessive disease, with massive accumulation of cholesteryl esters and triglycerides in many organs, leading to hepatosplenomegaly, microvesicular steatosis, cirrhosis and premature death. Early recognition is crucial for timely enzyme replacement therapy.

Objectives: To screen for LAL-D in subjects with dyslipidemias and/or liver disease at an outpatient lipid clinic.

Lysosomal Acid Lipase Deficiency: Report of Five Cases across the Age Spectrum.

Lysosomal acid lipase (LAL) deficiency is an autosomal recessive lysosomal storage disorder caused by mutations in the gene that leads to premature organ damage and mortality. We present retrospective data from medical records of 5 Brazilian patients, showing the broad clinical spectrum of the disease.

HISTOLOGICAL FEATURES OF EOSINOPHILIC ESOPHAGITIS IN CHILDREN AND ADOLESCENTS.

Background: Eosinophilic esophagitis is an emerging disease featured by eosinophilic esophageal infiltrate not responsive to proton pump inhibitors.

Objective: To characterize histological features of children and adolescents with eosinophilic esophagitis.

Methods: Cross-sectional study in a tertiary hospital.

Diagnosis of Helicobacter pylori infection by means of reduced-dose ¹³C-urea breath test and early sampling of exhaled breath.

Objective: The aim of this study was to evaluate the accuracy of reduced-dose ¹³C-urea breath test (¹³C-UBT) and early sampling of exhaled breath for the detection of Helicobacter pylori infection in children and adolescents.

Methods: Patients up to 20 years old that underwent upper gastrointestinal endoscopy with gastric biopsies were included. The ¹³C-UBT was performed after a 4-hour fasting period with 4 points of collection: baseline (T₀), and at 10, 20, and 30 minutes (T₁₀, T₂₀, and T₃₀) after ingestion of 25 mg ¹³C-urea diluted in 100 mL of apple juice.

Anti-tissue transglutaminase antibodies (IgA and IgG) in both Crohn´s disease and autoimmune diabetes.

Objective: a strong association has been observed between celiac disease, generally its silent clinical form, and autoimmune disorders. A potential correlation with inflammatory bowel disease has also been suggested. Anti-tissue transglutaminase antibodies have been detected in Crohn´s disease.

Morphometrical and immunohistochemical study of intrahepatic bile ducts in biliary atresia.

Background: Extrahepatic biliary atresia results from a progressive destruction of the bile ducts by an inflammatory fibrosing process which leads ultimately to cirrhosis of biliary type. The etiology of the disorder remains unknown. The histological features include cholestasis, ductular proliferation, eventual loss of intrahepatic bile ducts, and ducts with primitive embryonic shape (ductal plate malformation).

Gastric emptying evaluation in children with erosive gastroesophageal reflux disease.

Background/aims: Delayed gastric emptying may be an important contributing factor to gastroesophageal reflux disease (GERD) in children, but there are limited data on its evaluation in children with erosive-GERD. This study aims to evaluate the gastric emptying of a solid meal in patients with erosive-GERD.

Methods: Nineteen patients (age range 8.

Intraepithelial lymphocytes in duodenum from Brazilian adolescents with type 1 diabetes. Influence of Helicobacter pylori.

Background: An increased number of intraepithelial lymphocytes (IELs) can be the only histological feature in early stages of celiac disease (CD). This is also presented in duodenum of patients with Helicobacter pylori-associated gastritis and in autoimmune diseases. Because CD is frequently associated with type 1 diabetes mellitus, we analyzed the density of IELs in the distal duodenum of non-celiac diabetic patients associated or not with H.

Gastric emptying of solids is slower in functional dyspepsia unrelated to Helicobacter pylori infection in female children and teenagers.

Aim: To evaluate gastric emptying of solids in children and adolescents with functional dyspepsia with and without Helicobacter pylori infection.

Patients And Methods: The study included 27 female patients (mean age 13.38 +/- 2.

[Functional, microbiological and morphological intestinal findings among human immunodeficiency virus infected children].

Background: [corrected] Gastrointestinal tract disorders are frequent among human immunodeficiency virus infected children, with important repercussions on nutrition and survival. Most studies related to this subject were restricted to adults, being less investigated the problem in the children.

Aims: To study intestinal digestion, absorption, microbiological and morphological findings among human immunodeficiency virus infected children.

High prevalence of celiac disease in Brazilian blood donor volunteers based on screening by IgA antitissue transglutaminase antibody.

Objective: To study the prevalence of celiac disease among blood donor volunteers based on screening by IgA antitissue transglutaminase antibody, followed by a confirmatory small intestine biopsy.

Methods: The transversal study involved 3000 potential blood donors, residing in the city of Sao Paulo, Brazil. The participants were gender divided into 1500 men and 1500 women, with an average age 34.

[Analysis of clinicopathological, tumor cell proliferation and apoptosis parameters in adenocarcinoma of the gastric cardia].

Background/aims: In view of the increased incidence of carcinoma of the cardia over recent years, this work had the aim of studying the clinicopathological aspects, cell proliferative and tumor apoptotic indices of this neoplasm, their interrelations and possible influences on the prognosis.

Material And Methods: Forty cases of adenocarcinoma of the cardia were studied between 1988 and 2001, with a minimum clinical follow-up of 3 years. Patients were excluded if they had previous chemotherapy or radiotherapy treatment, presented early neoplasia, or died during the operations or for other reasons unrelated to cancer.

Urease activity does not reflect the degree of colonization by Helicobacter pylori in children.

Background: Urea breath test is an accurate method for the diagnosis of Helicobacter pylori infection in children. This test could estimate the bacterial load by measuring the urease activity in gastric mucosa. The aim of the study was to correlate the result of 13C-urea breath test (13C-UBT) and histological estimative for bacterial colonization and severity of inflammatory infiltrate.

Glycine reduces tissue lipid peroxidation in hypoxia-reoxygenation-induced necrotizing enterocolitis in rats.

Purpose: To assess the protective effect of glycine in an experimental model of Neonatal Necrotizing Enterocolitis (NEC).

Methods: Fifty (50) neonatal Wistar rats, from a litter of six female rats and weighing 4 to 6 grams, were used. Five animals were cannibalized and the 45 remaining were distributed into three groups: the G1 normal control group (n=12); the G2 Group (n=16), of animals that underwent hypoxia-reoxygenation (HR); the G3 Group of animals (n=17) that underwent HR following a 5% intraperitoneal glycine infusion.

[Evaluation of an experimental model of necrotizing enterocolitis in rats].

Objective: To evaluate an experimental model of necrotizing enterocolitis in rats proposed by OKUR e col. in 1995.

Methods: On their first day of life, 28 EPM-Wistar rats weighing between 4 and 6 grams were submitted to hypoxia (H) by placing them in a CO2 gas chamber for rodents' sacrifice, where they received a 100% CO2 air flow for 5 minutes.

13C-urea breath test to diagnose Helicobacter pylori infection in children aged up to 6 years.

Background: 13C-urea breath test (13C-UBT) is an accurate noninvasive tool for diagnosis of Helicobacter pylori infection. It is considered the best method for epidemiological studies, but there are few studies to evaluate the 13C-UBT in infants and toddlers.

Aim: To evaluate the 13C-UBT performed with infrared spectroscopy in children aged up to 6 years.

Cell-detaching Escherichia coli (CDEC) strains from children with diarrhea: Identification of a protein with toxigenic activity.

Twenty-four strains of cell-detaching Escherichia coli (CDEC) isolated from stool specimens in different cities in Brazil were examined for virulence properties. Aerobactin production and multiple antibiotic resistance were observed in most of the isolates. In hybridization studies, the alphahly, pap, and cnf sequences, common properties of this category of E.