CLASSICAL SEROLOGICAL MARKERS IN PEDIATRIC INFLAMMATORY BOWEL DISEASE IN BRAZIL.

Background: Perinuclear anti-neutrophil cytoplasmic antibodies (p-ANCA) and anti-Saccharomyces cerevisiae antibodies (ASCAs) have long been used to differentiate between Crohn's disease (CD) and ulcerative colitis (UC), more recently having been used as prognostic indicators.

Objective: To determine the diagnostic accuracy of serological markers in the identification of pediatric CD and UC in Sao Paulo, Brazil, as well as to correlate those markers with characteristics demographic and clinical of these two diseases.

Methods: Retrospective cross-sectional multi-center study involving pediatric patients with inflammatory bowel disease (IBD).

The first five-year evaluation of cystic fibrosis neonatal screening program in São Paulo State, Brazil.

The Hospital of the Ribeirão Preto Medical School, University of São Paulo is one of the three screening centers in São Paulo State, Brazil, and has included a test for cystic fibrosis (CF) since February 6, 2010, by a court order. We evaluated the first five years of this CF-newborn screening program. The original immunoreactive trypsinogen (IRT)/IRT screening protocol was adopted in Brazil.

ACRODERMATITIS ENTEROPATHICA: CLINICAL MANIFESTATIONS AND PEDIATRIC DIAGNOSIS.

Objective: To report a case of acrodermatitis enteropathica, a rare disease with autosomal recessive inheritance.

Case Description: An 11-month-old boy was presenting symmetrical erythematous and yellowish-brownish crusted lesions on his face, feet, hands and knees, intermittent diarrhea, fever, and recurrent infections since the age of six months. He was thin and had scarce hair on the scalp.

Nutritional status of adolescents with cystic fibrosis treated at a reference center in the southeast region of Brazil.

Background: Several factors can interfere with the full physical and emotional growth of adolescents, among them chronic diseases. The aim was to determine the nutritional status of adolescents and to associate it with puberty, pancreatic sufficiency, lung function and age range of Cystic Fibrosis (CF) diagnosis.

Methods: An observational, cross-sectional, retrospective and analytical study was conducted using the data of medical records.

The ontogeny of saliva secretion in infants and esophagoprotection.

Background: Several studies have reported that severe reflux esophagitis is rare in infants despite the well known high occurrence of regurgitation in early infancy. There is evidence of the importance of saliva for the pre-epithelial protection of the esophageal mucosa.

Results: A longitudinal study conducted on healthy infants indicated that the stimulated capacity of saliva secretion (saliva output per kg of body weight) was significantly higher during their first year of age compared to older children and adults.

[Early manifestations of cystic fibrosis in a premature patient with complex meconium ileus at birth].

Objective:: To report a case of a preterm infant with complex meconium ileus at birth and cystic fibrosis.

Case Description:: A male infant was born by vaginal delivery at 33 weeks and 5 days of gestational age with respiratory distress and severe abdominal distension. The exploratory laparotomy in the first day of life identified meconium ileus and secondary peritonitis.

[Newborn screening for galactosemia: a health economics evaluation].

This study assesses the efficiency of the galactosemia add-on test in neonatal screening performed on regular Guthrie card blood spots. Based on estimated average incidence of galactosemia (1:19,984 newborns) in São Paulo State, Brazil, the study develops a cost-benefit analysis model, using a B/C ratio and a 9.25% annual interest rate in order to decapitalize the results.

Clinical, nutritional and spirometric evaluation of patients with cystic fibrosis after the implementation of multidisciplinary treatment.

Objective: Cystic fibrosis (CF) is a chronic multisystemic hereditary disease for which a multidisciplinary approach must be taken. The objective of this study was to show the evolution of a group of patients with CF after the implementation of multidisciplinary treatment.

Methods: A retrospective study involving 19 patients (6-29 years of age) under clinical follow-up treatment at the University of São Paulo at Ribeirão Preto School of Medicine Hospital das Clínicas, located in the city of Ribeirão Preto, Brazil.

Impairment of bone mass development in children with chronic cholestatic liver disease.

Objective: To analyse aspects of mineral metabolism, bone mineral density (BMD), bone remodelling activity and serum IGF-1 levels in children with chronic cholestatic disease (CCLD).

Patients And Measurements: A total of 13 children with chronic cholestatic liver disease (CCLD; mean age 7.2 +/- 4.

Prevalence and demographic characteristics of celiac disease among blood donors in Ribeirão Preto, State of São Paulo, Brazil.

Celiac disease (CD) is an underdiagnosed disease occurring in different clinical forms. This study aimed to determine the prevalence of CD among blood donors from Ribeirão Preto, Brazil, and to study some demographic characteristics of celiac patients. Blood samples from 3000 blood donors were tested for the presence of tissue transglutaminase antibody and positive samples were tested for endomysial antibody.

Anorectal manometry in children with chronic functional constipation.

Background: The anorectal manometry is a very utilized and well recognized examination in children with chronic functional constipation. The major manometric findings in these children are: anal hypotonia, anal hypertonia, paradoxal contraction of the external anal sphincter, decreased ability of internal anal sphincter to relax during rectal distension and alterations in rectal contractility, sensibility and compliance.

Aims: To evaluate the anal basal pressure and the relaxation reflex before and after standard treatment for a better understanding of the physiopathologic mechanisms involved in pediatric chronic functional constipation.

Gastroesophageal reflux disease: its importance in ear, nose, and throat practice.

Objective: Gastroesophageal reflux disease (GERD) has been implicated in various extra-gastroesophageal diseases, especially in the upper and lower airways (atypical GERD). The objective of the present study was to determine the frequency of gastroesophageal and extra-gastroesophageal symptoms in pediatric patients with GERD and their response to the general treatment.

Material And Methods: A retrospective study was conducted on 45 children of both sexes aged 3 months-12 years with GERD diagnosed by clinical examination and 24h single-channel pHmetry.

[Clinical presentation of children with celiac disease attended at a Brazilian specialized university service, over two periods of time].

Background: Several studies have demonstrated changes in the forms of clinical presentation of celiac disease in children and adults, with an increased occurrence of atypical forms.

Aim: To determine this fact in Clinical Hospital, Ribeirão Preto, SP, Brazil.

Methods: Celiac patients were studied over two different periods of time, from January 1978 to December 1987 (group 1 = G1) and from January 1988 to December 1997 (group 2 = G2).

Adult-type hypolactasia: clinical, morphologic and functional characteristics in Brazilian patients at a university hospital.

Background: Adult-type hypolactasia (AH) is the most common form of disaccharidase deficiency in humans, with a prevalence that varies among ethnic groups. In Brazil, the few available studies suggest a high prevalence of this condition. The objective of this study was to determine the prevalence of AH in Brazilian patients at the Ribeirão Preto University Hospital, and to study its morphologic and functional expression.

[Prevalence of chronic constipation in children at a primary health care unit].

Objective: To study the frequency of chronic constipation in childhood at a Primary Health Care Unit and to observe its characteristics in this population.

Methods: The sample consisted of 313 children aged between one and 10 years treated at the Centro de Saúde Escola, in the district of Tibério, in the town of Ribeirão Preto. Among these, 84 children with chronic constipation were selected.

Triglyceride-induced diabetes mellitus in congenital generalized lipodystrophy.

High levels of triglycerides and free fatty acids have been implicated in the pathogenesis of type 2 diabetes mellitus (DM). Congenital generalized lipodystrophy (CGL) is an autosomal recessive syndrome characterized by intense whole body reduction of subcutaneous fat. Its clinical manifestations appear during the first years of life.