Metabolic alkalosis with hypo-electrolytaemia or pseudo-Bartter syndrome as a presentation of cystic fibrosis in infancy. Discription of three cases.
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Biomedical subjects
Publications and source records attributed to H Escobar Castro.
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Intestinal permeability was studied in a group of 10 children diagnosed of coeliac disease either when they presented an intestinal villi atrophy while receiving a gluten-containing diet and after, when they had a normal mucosa with the withdrawal of gluten from the diet. Ten healthy children served as controls. The 51 Cr-EDTA (51 Cr-ethylenediaminetetraacetate) was used as probe molecule and it was administered orally. Its urinary excretion showed clear differences between patients with an altered intestinal biopsy and those with a normal histopathology. The excretion of 51 Cr-EDTA remained elevated in celiac patients with a gluten-free diet and normal villi.
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Between 1980 and 1990, we have diagnosed fourteen cases of cholelithiasis in children at our hospital. Abdominal pain was the most common symptom. Diagnosis was made by abdominal ultrasonography in all of the cases. Risk factors were found in 4 children. Family history was present in four patients. The treatments performed included cholecystectomy in five cases, extracorporeal lithotripsy in one case and a dietary regimen with regular clinical reviews in eight patients.
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Treatment of caustic ingestion in children is a difficult and controversial problem which has not yet to be resolved. Here we report the evolution of fourteen children with esophageal injury after caustic ingestion that were treated with steroids at high-doses, nasogastric intubation and antibiotics. An early esophagoscopy was performed in all patients. Four patients developed esophageal strictures, three of which were mild and subsequent dilatation was successful. Only one patient was referred to surgery. We think that although it cannot be established, the use of high-dose of steroids and nasogastric intubation may be helpful for children with caustic esophagitis. We conclude that early endoscopy is useful to evaluate the severity of the lesions and to establish the necessity for treatment.
Autoimmune hemolytic anemia and severe giant cell hepatitis is an very uncommon association. Two patients aged 5 months and 2 years developed severe anemia, jaundice, hepatomegaly and splenomegaly. Laboratory tests demonstrated autoimmune hemolytic anemia and elevated amino-transferases. Diffuse giant cell transformation and distorted architecture was seen in liver biopsy. One patient had a cytomegalovirus infection. Early steroid therapy did not prevent fatal course with liver failure in one patient.
Over one half of the cases of chronic severe childhood asthma, refractory to conventional therapy and without other over aetiology, are associated to gastroesophageal reflux (GER). The aetiopathogenic role of GER in asthma is uncertain, and is only confirmed when therapy of the former induces evident improvement of the latter. We have instituted medical antireflux therapy over two years in 17 patients (17.6%) became asymptomatic after less than three months of therapy, and a further four (23.5%) by the sixth month. Seven patients showed clinical improvement by the third month (41%) and even a greater one by the sixth month, only very slight symptoms persisting thereafter. In three cases (17.6%) there was no improvement after two years of outcome of asthma and the persistence or not of pathological between the outcome of asthma and the persistence or not of pathological 24-hour pHmetry. On the contrary. macroscopic oesophagitis disappeared in the cases of asthma with good outcome, but persisted in all the cases who remained symptomatic. In conclusion, we consider that medical management of GER associated to asthma should be maintained for at least six months (if the clinical course so permits) before considering a surgical indication.
In order to determine the prevalence of hepatitis B virus (HBV) markers, we studied 375 healthy children, 242 males and 113 females, with age ranged between 6 to 14 years, from different schools at the Tetuán district (Madrid). We sent a questionnaire to the parents to investigate the presence of risk factor in the children and/or their parents. All children were screened for hepatitis B markers in serum. Our results shown the existence of HBV markers in 22 (5.9%) children. No major incidence of risk factors was found in the children with serologic evidence of HBV infection.
We present 28 children, 4 to 14 year-old, with duodenal ulcer; there were 21 males and 7 females. In 16 cases, after stimulation with pentagastrin, basal pepsinogen I (PG1), basal gastrinemia and basal acid output (BAO) and maximal acid output (MAO) were measured. Compared to controls, the mean levels of PG1 and gastrin were significantly higher in the patients; 12 children (80%) had high levels of PG1 and the remaining 3 (20%) had normal levels. The blood group O was the most prevalent: 64% of the cases.
A randomized, double-blind trial was performed to assess serological response and clinical protection for acute gastroenteritis due to rotavirus in 103 children aged 6 to 18 months, after a single dose of RIT 4237 live attenuated bovine rotavirus vaccine or placebo. Seroconversion, determined by enzyme-linked immunoabsorbent assay (ELISA), was significantly greater in initially seronegative vaccines compared with control group (p less than 0.0001); clinical protection rate was low in this study group and it is therefore concluded that seroconversion by itself is not sufficient to measure vaccine efficacy.
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A child with recurrent infections is presented. The frequent diarrhea leads to a severe malnutrition. The immunological work-up disclosed: absent IgA and low IgG serum levels, deficient cellular immunity and abnormal neutrophil chemotaxis. The cellular immunity and the neutrophil chemotaxis were improved with the nutritional status. At present the patient shows a partial combined immunodeficiency. The diagnosis of the case as a Nezelof's syndrome is discussed, as well as the addition of a secondary immunodeficiency caused by the recurrent infections and the malnutrition.
Zinc and copper seric values have been determined in 120 healthy children between the ages of 0 and 7 years. Percentile grafics of normal zinc, copper and copper/zinc ratio values, are also described.
In a boy initially diagnosed as X-linked hypogammaglobulinemia, the later clinical and analytical course and the application of new immunological techniques led to the new diagnosis of common variable immunodeficiency. The patient shows panhypogammaglobulinemia, a scarce number of plasma cells with a normal number of precursors and circulating B lymphocytes with membrane bound immunoglobulins and C3 receptors. Delayed hypersensitivity is absent despite normal amount of circulating T lymphocytes which were able to transform when stimulated by PHA. The anergy seems primary and not related to the clinical malnutrition. The authors make a differential diagnosis between both illnesses as a tentative pathogenetic interpretation of B cell development arrest.
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