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F Alonso Falcón

Publications and source records attributed to F Alonso Falcón.

5 recordsLinked to original sources

Study of gastrointestinal polypeptides controlling gastric acid secretion in patients with primary antibody deficiency.

BACKGROUND: Gastric abnormalities are a common feature in patients with primary antibody deficiency. The most important problem is the high incidence of stomach cancer found in these patients. Chronic atrophic gastritis with pernicious anemia is also a common finding that predisposes to gastric adenocarcinoma. The aim of the present study was to identify factors predictive of high risk for developing gastric cancer in patients with primary antibody deficiency. PATIENTS AND METHODS: We studied gastric hormones (gastrin, somatostatin and gastrin-releasing peptide, GRP) in 47 patients (23 children and 24 adults) with primary antibody deficiency. In accordance with the World Health Organization (WHO) classification, patients were diagnosed as having X-linked agammaglobulinemia (Bruton disease) in 13 cases, common variable immunodeficiency in 28, and hypogammaglobulinemia with hyperIgM in 6. Gastric biopsy was performed in 22 patients (16 children and 6 adults). Hormone determinations were carried out by radioimmunoassay. RESULTS: Baseline serum gastrin levels were normal or increased compared with controls, but the response to stimulation with a hyperproteic diet was delayed in 18 patients and lower than in controls in 7. In 4 adult patients, all with pernicious anemia, gastric biopsy revealed chronic atrophic gastritis involving the stomach corpus and antrum (type B gastritis). The absence of a normal response of gastrin secretion to stimulation with a hyperproteic diet may be explained by this finding. Serum somatostatin and GRP levels were higher than in controls. No correlations were found between these findings and patient age, type of immunodeficiency or duration of clinical manifestations.

Adolescent

[Radiologic digestive manifestations in patients with antibody deficiency].

UNLABELLED: In order to establish a relationship between radiological and clinical data and/or early diagnosis of the complications frequently found in patients with hypogammaglobulinemia, we carried out gastrointestinal series (GIS) in 47 patients mean age 22.5 years with hypogammaglobulinemia or primary antibody deficiencies. RESULTS: 15 patients did not show any radiological abnormalities. Nodular lymphoid hyperplasia (NLH) was found in 23 cases. None of the patients with NLH had gastrointestinal symptoms. Radiologic signs of malabsorption were present in 15 cases. 2 patients with radiologic signs of malabsorption (mean age 12 years) did have clinical or laboratory finding suggesting a malabsortive syndrome. Moreover, 2 patients showing clinical manifestations of malabsorption did not show any significant radiological findings. 4 patients were diagnosed of chronic atrophic gastritis (CAG) by means of endoscopy and gastric biopsy, whereas only in one of these cases GIS showed gastric fold atrophy, compatible with the pathologic diagnosis of CAG; 2 of these patients progressed to gastric cancer, a diagnosis that was done by endoscopy. CONCLUSIONS: We have observed that NLH is more frequent in patients with hypogammaglobulinemia than in the normal population, it is asymptomatic and may be diagnosed by radiological methods. The radiological findings of malabsorption do not correlate with the clinical data. A combination of endoscopy and gastric biopsy is the method of choice for the early diagnosis of atrophic gastritis and gastric cancer, since the radiological alterations found in GIS appear at late times. We conclude that GIS supplies few data to the study of digestive manifestations in patients with hypogammaglobulinemia, except in the case of NLH.

Adolescent

[GM1 gangliosidosis, type I, Landing's disease. Presentation of a case and review of the literature].

A case of type 1 GM1 gangliosidosis, also called Norman-Landing disease is reported. The patient' was a nine months old boy who presented psychomotor retardation since birth, coarse facies, hepatomegaly and macular cherry red spot. Roentgenographic findings were those of dysostosis multiplex. Bone marrow smear showed type 1 Gasser's cells, as it occurs in the storage diseases. The infant presented a severe B-galactosidase deficiency and died at the age of ten months. Recent advances in pathogenesis, diagnosis and future therapy are discussed.

G(M1) Ganglioside