Lívia Carvalho Galvão

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. Donors positive to both tests were referred for clinical evaluation and for a jejunal biopsy. Twenty-four samples were moderately/strongly positive for transglutaminase, with 9 of them being endomysial negative and 15 positive. Of the 13 biopsies obtained from 12 females and 1 male, 1 was classified as Marsh grade IV, 4 as grade III, 2 as grade II, 4 as grade I, and 2 as grade 0. Estimated prevalence was therefore 1:273 (0.33%; 95% CI, 0.127 to 0.539). The 1:273 estimated prevalence of CD detected indicates that the disease is not rare in Brazil. The frequency was higher among females and among individuals of European descent, with a significantly higher frequency for a family history of digestive tract cancer or epilepsy.

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. The chi-squared test was used to compare the groups of constipated and nonconstipated children (p=0.01). RESULTS The prevalence of chronic constipation was 26.8%. In the group of chronic constipation, 85.7% of the children had hardened stools, 25% showed reduction in fecal mass, 17.9% had an interval between evacuations longer than 2 days (constipated children), 14.3% revealed fecal soiling and 21.4% presented blood in stools. CONCLUSION The prevalence of chronic constipation was high and the interval between evacuations was as an important diagnostic criterion for the selection of constipated children.

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).

Human leukocyte antigen class II alleles in white Brazilian patients with celiac disease.

Background Celiac disease (CD) is a permanent gluten intolerance disorder characterized by malabsorption, intestinal mucosa villus atrophy, and crypt hyperplasia. Clinical and histologic features improve in persons consuming a gluten free diet. The pathogenesis of CD involves environmental, genetic, and immunologic factors. Methods The frequencies of human leukocyte antigen (HLA) class II alleles were evaluated in white Brazilian patients who had CD and compared with those observed in healthy individuals from the same geographical area (Ribeirão Preto, São Paulo) and of similar ethnic background. Twenty-five patients with CD, 11 females and 14 males, and 91 control individuals were studied. The HLA class II alleles were typed using amplified DNA hybridized with sequence-specific primers. Statistical analysis was performed using the two-tailed Fisher exact test. The relative risk (RR), etiologic fraction (EF), and preventive fraction (PF) were also estimated. The EF represents the attributable risk for the development of CD at the population level, whereas PF represents the protective risk. Results The frequency of the HLA-DRB1*03, HLA-DRB1*07, and HLA-DQB1*02 alleles was significantly increased in patients. The RR conferred by these alleles was 5.35, 7.15, and 10.6, respectively, and the EF was 48.7%, 44.7%, and 76%, respectively. The frequency of HLADQB1*06 alleles was significantly decreased in CD patients, conferring an RR of 0.08 and a PF of 48%. Conclusions The results show that HLA-DRB1*03, HLA-DRB1*07, and HLA-DQB1*02 alleles conferred susceptibility to CD in Brazilian patients. In contrast, HLADQB1*06 alleles conferred protection against development of the disease.

Apresentação clínica de doença celíaca em crianças durante dois períodos, em serviço universitário especializado

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). Time of disease, age at diagnosis and nutritional status and clinical forms were investigated. The typical form was considered to be present if the patient had at least two of the following signs/symptoms: diarrhea, abdominal distention, and weight loss, associated or not with others. RESULTS Clinical forms (%) - G1: typical 57.14 and atypical 42.85. G2: typical 55.18 and atypical 44.82. Median age at diagnosis: 23 months (G1) and 21 months (G2). Interval between the onset of symptoms and the diagnosis: 13 months (G1) and 11 months (G2). Gomez nutritional status G1: Eutrophy = 23.8, DI = 47.61, DII = 23.8 and DIII = 4.76. G2: Eutrophy = 20.68, DI = 48.27, DII = 27.58 and DIII = 3.44. Waterloo G1: Eutrophy = 23.8, Wasting = 14.28, Stunting = 28.57 and Chronic = 33.33. G2: Eutrophy = 20.68, Wasting = 13.79, Stunting = 34.48 and Chronic = 31.03. No statistically significant differences occurred between the groups for any of these parameters. CONCLUSIONS No change in the forms of presentation or remaining clinical characteristics of celiac disease occurred in our service. This may be due to the population characteristics or to differences among health services, or to different periods of observation.

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. METHODS Anorectal manometry was performed before treatment on 20 children with chronic functional constipation aged 4 to 12 years and the results were compared to those obtained after standard treatment, with a good outcome. RESULTS There was a reduction in anal basal pressure after treatment, but no differences were detected between the anorectal manometries performed before and after treatment in terms of amplitude and duration of relaxation, residual pressure, latency time, or descent and ascent angle. CONCLUSIONS We conclude that the anal basal pressure decreased in children recovering from chronic functional constipation, but the standard treatment did not provide all the conditions necessary for the relaxation reflex of constipated children to return to the values described in normal children.

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. Methods: One hundred fifteen patients between 5 and 60 years undergoing upper gastrointestinal endoscopy were included. Mucosal biopsy specimens were obtained from the second portion of the duodenum. AH was defined by the disaccharidase activity (lactase/sucrase ratio) of the duodenal mucosa. The morphologic expression of lactase was studied by immunohistochemistry. Results: The mean age of the subjects was 28.8 ± 14.8 years. Seventy of the 115 subjects (60.8% prevalence) had AH by enzyme activity measurements. Milk drinking was common and similar in patients with and without AH. Among the patients, 91.3% of the nonwhite and 53.2% of the white individuals had hypolactasia (P = 0.002). Immunohistochemistry revealed the presence of lactase in 73.3% of individuals with normal lactase activity. Two different expression patterns were found in patients with AH. Conclusions: The prevalence of AH was high in our subjects and similar to that reported in other Brazilian studies. Hypolactasia was more common among nonwhites. Immunohisto-chemistry permitted the identification of two phenotypes of AH, the first characterized by the absence of both stainable lactase and lactase activity, and the second by the presence of stainable lactase without significant activity.

Sobrevida e processos infecciosos em pacientes com AIDS: análise de acordo com os níveis séricos iniciais de vitamina A

Patients with Aids (n = 39) were followed up for a maximum period of 36 weeks, after which the types and topographies of infectious complications presented and patient survival were analyzed and correlated with the vitamin A levels presented by the patients at the beginning of clinical follow-up. Twenty-one (53,8%) patients presented serum retinol levels below 1.6mmol/L, 12 (57%) of whom had values lower than 1.05mmol/L. There was no correlation between low serum vitamin A levels and the types or topographies of the infectious complications that occurred during the follow-up period. Although mean survival at the end of the 36 months follow-up period was similar for the two groups, patients with retinol deficiency presented a lower probability of survival during the first 24 months of follow-up compared to patients without hypovitaminosis A (8.44 x 1.42 months; p = 0.003).Patients with Aids (n = 39) were followed up for a maximum period of 36 weeks, after which the types and topographies of infectious complications presented and patient survival were analyzed and correlated with the vitamin A levels presented by the patients at the beginning of clinical follow-up. Twenty-one (53,8%) patients presented serum retinol levels below 1.6 micromol/L, 12 (57%) of whom had values lower than 1.05 micromol/L. There was no correlation between low serum vitamin A levels and the types or topographies of the infectious complications that occurred during the follow-up period. Although mean survival at the end of the 36 months follow-up period was similar for the two groups, patients with retinol deficiency presented a lower probability of survival during the first 24 months of follow-up compared to patients without hypovitaminosis A (8.44 x 1.42 months; p = 0.003).

Study of lung involvement in patients with cystic fibrosis

OBJECTIVE: To characterize the involvement of the respiratory apparatus of patients with cystic fibrosis in order to obtain a comprehensive view of their pulmonary picture.METHODS: Data were obtained retrospectively from the medical records of 16 patients with cystic fibrosis; arterial gas and spirometry data were obtained prospectively for the same patients, who were not in an acute pulmonary situation. The patients were subjects of both sexes aged 6 years or older who were followed up at the Pediatrics Outpatient Clinic of the University Hospital, Faculty of Medicine of Ribeirão Preto, USP.RESULTS: Median patient age was 114 months (9 years and 6 months) ranging between 72 - 360 months, and 68.75% were males. Productive cough was the most frequent symptom observed in 75% of the population studied. All patients had positive sputum culture obtained at least one year before, with Pseudomonas aeruginosa being detected in 81.25% of the cases. Arterial gases revealed some abnormalities in 81.25% of the patients and spirometry revealed abnormalities in 56.25%.CONCLUSION: All patients presented at least one type of pulmonary alteration. Measurement of arterial gases detected a larger number of patients with altered pulmonary function than did spirometry, but the two examinations complemented each other for a good evaluation of pulmonary function.

[Neonatal cholestasis and cytomegalovirus infection: clinical and histopathologic forms].

Cytomegalovirus infection is symptomatic in only 10% of cases. The most frequent findings are cholestasis and hepatosplenomegaly. Ten patients who presented neonatal cholestasis associated with cytomegalovirus infection were studied. The majority had elevation of serum aminotransferases and mild abnormality of hepatic function. The histopathologic findings were: normal, giant cell hepatitis, bile duct proliferation (confused with extrahepatic biliary atresia) and ductopenia. The clinical course of the patients varied from disappearance of the symptoms (2 cases) to death (2 cases). Because of the possibility of confusing the histologic findings with extrahepatic biliary atresia, the etiology of neonatal cholestasis, including cytomegalovirus infection, should be determined as soon as possible.