Effect of meconium ileus on the clinical prognosis of patients with cystic fibrosis

The objective of the present study was to determine the possible prognostic factors which may explain the difference in the survival of patients with cystic fibrosis (CF) with and without meconium ileus. Over a period of 20 years, 127 patients with CF, whose diagnosis was confirmed by typical clinical characteristics and altered sweat chloride levels, were studied retrospectively. The patients were divided into two groups: group 1 consisted of patients who presented CF and meconium ileus (N = 9), and group 2 consisted of patients with CF without meconium ileus (N = 118). The characteristics studied were based on data obtained upon admission of the patients using a specific protocol. Demographic, clinical, nutritional and laboratory data were obtained. The genotype was determined in 106 patients by PCR. Survival was analyzed using the Kaplan-Meier method. The median follow-up period was 44 months. A statistically significant difference was observed between the groups studied regarding the following variables: age at diagnosis and weight and height z scores. The presence of meconium ileus was associated with an earlier diagnosis; these patients had greater deficits in height and weight at the time of diagnosis and at the end of the study. The estimated probability of survival for patients with CF without meconium ileus was 62 ± 14 percent and for those with meconium ileus 32 ± 18 percent. Patients with CF and meconium ileus presented a poor nutritional status at diagnosis and a lower survival rate compared to the general CF population

Serum antigliadin antibody levels as a screening criterion before jejunal biopsy indication for celiac disease in a developing country

The objective of the present study was to determine the efficacy of detection of antigliadin immunoglobulins G and A (IgG and IgA) for the diagnosis of celiac disease in a developing country, since other enteropathies might alter the levels of these antibodies. Three groups were studied: 22 patients with celiac disease (mean age: 30.6 months), 61 patients with other enteropathies (mean age: 43.3 months), and 46 patients without enteropathies (mean age: 96.9 months). Antigliadin IgG and IgA ELISA showed sensitivity of 90.9 and 95.5 percent, respectively. With the hypothetical values of prevalence ranging from 1:500 to 1:2000 liveborns, the positive predictive value varied from 8.5 to 2.3 percent for IgG and from 4.8 to 1.1 percent for IgA. Considering the patients without enteropathies, specificity was 97.8 and 95.7 percent for IgG and IgA, respectively. In patients with other enteropathies, specificity was 82.0 and 84.1 percent, respectively. When patients with and without other enteropathies were considered as a whole, specificity was 88.8 and 91.6 percent, respectively. The specificity of positive IgG or IgA was 93.5 percent in children without enteropathies and 78.7 percent in the presence of other enteropathies. The negative predictive value for hypothetical prevalences varying from 1:500 to 1:2000 liveborns was 99.9 percent. Thus, even in developing countries where the prevalence of non-celiac enteropathies is high, the determination of serum antigliadin antibody levels is a useful screening test prior to the jejunal biopsy in the investigation of intestinal malabsorption

Dietary fiber intake, stool frequency and colonic transit time in chronic functional constipation in children

The objective of the present study was to evaluate associations between fiber intake, colonic transit time and stool frequency. Thirty-eight patients aged 4 to 14 years were submitted to alimentary evaluation and to measurement of colonic transit time. The median fiber intake of the total sample was age + 10.3 g/day. Only 18.4 percent of the subjects presented a daily dietary fiber intake below the levels recommended by the American Health Foundation. In this group, the median left colonic transit time was shorter than in the group with higher dietary fiber intake (11 vs 17 h, P = 0.067). The correlation between stool frequency and colonic transit time was negative and weak for left colon (r = -0.3, P = 0.04), and negative and moderate for rectosigmoid and total colon (r = -0.5, P<0.001 and r = -0.5, P<0.001, respectively). The stool frequency was lower in the group with slow transit time (0.8 vs 2.3 per week, P = 0.014). In conclusion, most patients with chronic functional constipation had adequate dietary fiber intake. The negative correlation between stool frequency and colonic transit time increased progressively from proximal segments to distal segments of the colon. Patients with normal and prolonged colonic transit time differ in terms of stool frequency

Quadro clínico e nutricional de pacientes com fibrose cística: 20 anos de seguimento no HC-UFMG / Clinical and nutritional aspects of a Center of Cystic Fibrosis-HC-UFMG: 20 years of follow-up

OBJETIVOS: A fibrose cística (FC) é a doença genética letal, de herança autossômica recessiva, mais comum entre pacientes de cor branca. O presente estudo foi realizado com o objetivo de identificar o quadro clínico e nutricional à admissäo dos pacientes no Centro de Tratamento de FC do HC-UFMG e avaliar a sobrevida a longo prazo. PACIENTES E MÉTODOS: Em um período de 20 anos, 127 pacientes portadores de FC foram acompanhados longitudinalmente e submetidos a protocolo previamente estabelecido, após confirmaçäo do diagnóstico pelo teste do suo...

Morphological changes of the jejunal mucosa in protracted diarrhea and their correlation with disease duration, weight loss and serum albumin levels

The pathogenesis of protracted diarrhea is multifactorial. In developing countries, intestinal infectious processes seem to play an important role in triggering the syndrome. Thirty-four children aged 1 to 14 months, mean 6.5 months, with protracted diarrhea were studied clinically and in terms of small intestinal mucosal morphology. Mild, moderate or severe hypotrophy of the jejunal mucosa was detected in 82 percent of cases, and mucosal atrophy was observed in 12 percent. The intensity of the morphological changes of the jejunal mucosa correlated negatively with serum albumin levels. No correlation was detected between mucosal grading and duration of diarrhea or between mucosal grading and weight reported as percentile. After nutritional support was instituted, serial jejunal biopsies were obtained from 12 patients: five patients submitted to parenteral nutrition for 7 to 38 days, mean 17 days, and 7 patients reveiving a hypoallergenic oral diet (semi-elemental formula, 3; chicken formula, 3; human milk, 1). In seven cases (58 percent) a progressive increase in villus height and a decrease in the number of inflammatory cells were noted. Recovery of the morphologic pattern was accompanied by clinical improvement in all patients.

Prevalence of anemia among school-children from Rio-Acima (State of Minas Gerais, Brazil): use of the standardized prevalence method and evaluation of iron deficiency

The prevalence of anemia and iron deficiency was investigated in 332 children aged 7 to 15 years, 156 (47 per cent) boys and 176 (53 per cent) girls enrolled in the schools of the municipality of Rio Acima, MG. Seventy-four children were white (22.3 per cent), 218 were mulatto (65.7 per cent), and 40 were black (12 per cent). Mean hemoglobin level was 12.75 ñ 0.75 g/ dl. Lower values were determined for black children (12.32 + 0.87 g/dl) compared to white (l2.76 ñ 0.99 g/dl) and mulatto (12.81 ñ 0.94 g/dl) children. The prevalence of anemia was 16.6 per cent when determined on the basis of the percentage of children with hemoglobin values lower than the 3rd percentile for age and sex (standard method), and 36.2 per cent when determined by the standardized prevalence method for the evaluation of the prevalence of malnutrition in populations. Depletion of iron reserves was 8.13 per cent for the population in general and 20 per cent for the anemic children. This low prevalence of iron deficiency may have been the result of the value adopted as the lower normal limit (10 ng/ml) for serum ferritin values. The small percentage of anemic children with iron depletion may also be justified by the standard of normality adopted for hemoglobin values which was originally elaborated for the white population of North America and Finland and therefore may be inadequate for the population studied here, of diverse racial composition.

Fecal loss and clearance of alpha-1-antitrypsin in children with persistent diarrhea

1. Daily fecal loss and daily clearance of alpha-1-antitrypsin were determined in 30 infants without intestinal disorders and in 21 with persistent diarrhea. 2. Stools were collected during a 48-h period and a randomly obtained single sample was also collected. Blood samples were also collected from the infants, and alpha-1-antitrypsin was measured by radial immunodiffusion in both stool and serum. 3. No difference in daily fecal loss (mg/d) of alpha-1-antitrypsin was detected between the control group and the group with persistent diarrhea (11 +/- 9.3 vs 18.5 +/- 20 mg/d). No difference in daily alpha-1-antitrypsin clearance (ml/d) was detected between the control group and the group with persistent diarrhea (4.3 +/- 3.6 vs 5.2 +/- 4.8 ml/d). 4. There was a strong correlation between daily fecal loss and daily clearance of alpha-1-antitrypsin (N = 50). There was a weak correlation between the concentrations of alpha-1-antitrypsin in randomly obtained single samples and daily fecal loss of the antiprotease (N = 25; r = -0.183; P < 0.01). 5. We conclude that: a) there is no increased fecal loss of alpha-1-antitrypsin persistent in diarrhea; b) fecal alpha-1-antitrypsin clearance is not necessary to estimate the enteric loss of the antiprotease; c) the determination of alpha-1-antitrypsin in random samples of feces is not a reliable method.

Diagnosis and distribution of Helicobacter pylori in the gastric mucosa of symptomatic children

The demonstration of the association of H, pylori with gastritis and peptic ulcer has been of increasing interest to gastroenterologists, microbiologists, and histopathologists. In this study, the presence of H, pylori in the gastric mucosa of children was investigated by culture, preformed urease test, and carbolfuchsin staining of biopsy smears. The organism was detected in 44,9% of the children studied, and found to be distributed equaly on the antral and fundic mucosa. Compared to culture, the urease test and carbolfuchsin staining proved to be of higher sensitivity and specificity in detecting H, pylori

Influence of nutritional status, age and sex on infant hair zinc concentration

1. Zinc concentration was measured in hair samples from 57 infants (27 boys and 30 girls) aged 7 to 24 months. Tyenty-eight infants were considered eutrophic and 29 chronic and severe malnutrition. 2. Hair segments less than 3-cm long were close scalp in the occipital area and washed in deionized water and acetone. Zinc levels were measured by neutron-activation. 3. Hair zinc concentration decreased with age in both eutrophic and malnourished infants from 160 microng/g at 7 months to 90 microng/g at 24 months. 4. No statistically significant difference in hair zinc concentration was detected between eutrophic an malnourished infants (148 ñ 60 vs 128 ñ 57 vs microng/g hair, mean ñ SD) or between sexes

Chronic calcifying pancreatitis in children: a report of six cases

1. Study of six patients with chronic calcifying pancreatitis (CCP) occurring over a 10 year period, representing an incidence rate of approximately 1:1,000 of the impatients in the children's ward of a general hospital in central Brazil, is reported. 2. Major clinical manifestations as well as therapeutic management are described. 3. The possible relationship between CCP and primary protein-calorie malnutrition is discussed and the importance of a CCP diagnosis when dealing malnourished children who do not respond satisfactorily to common therapy is emphasized

Doenca celiaca:aspectos atuais e nossa experiencia. / Celic disease: current aspects and our experience

Neste trabalho sao feitas consideracoes sobre a doenca celiaca. Uma casuistica e apresentada, e a necessidade de se estabelecer criterios bem definidos da doenca e discutida. Enfatiza-se o risco de complicacoes que correm os celiacos em uso de gluten. Manifestacoes clinicas pouco comuns e outras menos evidentes sao abordadas no trabalho o efeito protetor do leite humano e enfatizado

Sindrome de Chilaidite na infancia (interposicao hepato-diafragmatica do colo) - Relato de cinco casos. / Chilaidite syndrome in infancy (hepatodiaphragmatic interposition of the colon) - Report of 5 cases

Os autores relatam cinco casos de interposicao do colo entre o figado e o diafragma (Sindrome de Chilaidite) em criancas. A distensao das alcas do colo por gas, devido a aerofagia e diarreia, foi um achado comum. O estudo radiologico do abdome em posicao vertical foi sempre necessario para o diagnostico definitivo. O diagnostico diferencial foi feito com pneumotorax, pneumoperitonio e abscesso subfrenico principalmente. O tratamento foi expectante em quatro pacientes e cirurgico em um

Duodenal intubation in the differential diagnosis of obstructive jaundice in infants.

Os autores estudaram a validade da intubacao duodenal no diagnostico diferencial da ictericia em criancas. Trinta e seis pacientes foram estudados, com idades variando entre 1 e 5 meses (media - 2,4), sendo 21 meninos e 15 meninas, todos com colestase caracterizada por ictericia, coluria e acolia fecal. Em todos os 15 casos que apresentaram intubacoes negativas, a cirurgia confirmou o diagnostico de atresia biliar. Os 21 pacientes restantes, com intubacao duodenal positiva, foram rotulados, naquela oportunidade, de portadores de "sindrome de hepatite neonatal". Devido a sua eficiencia, baixo custo e carater inofensivo, a intubacao duodenal revelou-se um metodo util para discriminar quais as criancas cuja colestase requer tratamento medico ou cirurgico

Uso da dieta de frango no tratamento da diarreia protraida do lactante. / Use of chicken diet in the treatment of protracted infantile diarrhea

Os autores apresentam a sua experiencia com o uso de carne de peito de frango como fonte proteica no tratamento dietetico da diarreia protraida do lactente. Os bons resultados obtidos sao motivo de sugestao para o uso desta formula