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Biomedical subjects

A Barbato

Publications and source records attributed to A Barbato.

At least 73 records · Page 4Linked to original sources

Bernard-Soulier syndrome: diagnosis by an ELISA method using monoclonal antibodies in 2 new unrelated patients.

Two unrelated patients with Bernard-Soulier syndrome and their relatives were studied. The patients demonstrated severe bleeding diathesis, the relatives were asymptomatic. The propositi showed the characteristic abnormalities of the syndrome: thrombocytopenia, a percentage of giant platelets higher than 65%, prolonged bleeding time and defective platelet aggregation to ristocetin and bovine plasma. On the contrary, in the heterozygotes, the typical abnormalities were not fully evident. We introduce a simple ELISA method for the precise definition of both homozygous and heterozygous states for the syndrome by the quantitation of platelet glycoprotein (GP) Ib. Specific binding of monoclonal antibodies anti-platelet GPIb was performed both by direct binding of radioiodinated antibody and by ELISA. Comparable results were obtained. In fact, we demonstrated near absence of GPIb in the 2 propositi and about half the amount in the heterozygotes studied compared to normal platelets.

Adolescent↗

Influence of aeroallergens on bronchial reactivity in children sensitized to grass pollens.

Nine children, sensitized to grass pollens, with seasonal rhinitis and mild asthma underwent bronchial challenge with carbachol and EIA test repeatedly either during a pre-seasonal or in seasonal period. We failed to find any significant difference between pre-seasonal and seasonal values in both tests. We believe that atmospheric conditions are an important determinant of our results. It is our hypothesis that a threshold concentration in atmospheric pollen may exist which must be reached before an allergic reaction is evident.

Air Pollution↗

Localization of ectopic parathyroid adenomas by the technetium-thallium subtraction scan.

Among numerous methods utilized for preoperative localization of hyperfunctioning ectopic parathyroid tissue, the technetium-thallium scan appears to be a major advance. Eight patients are presented where this method located ectopic parathyroid tissue in the neck, in the mediastinum, within the pericardium, and within the thyroid gland. To date, these findings have been confirmed operatively in five of the eight patients. On the basis of our limited experience, the authors believe that this is a valuable method in the preoperative localization of ectopic parathyroid tissue, especially in patients with persistent or recurrent hyperparathyroidism following a thorough neck exploration.

Adenoma↗

Hyperthyroidism and Addison's disease in a patient with myotonic dystrophy.

A 53-year-old man had myotonic dystrophy, hyperthyroidism, and Addison's disease, an association not previously reported, to our knowledge. In the literature, at least five cases of hyperthyroidism associated with myotonic dystrophy have been described, but none also had Addison's disease. The presence of thyroid anti-microsomal antibodies and anti-adrenal antibodies suggests that the two endocrine disorders may be autoimmune. In our case, the treatment of the two endocrinopathies caused a reduction of myotonic symptoms.

Addison Disease↗

Spectrum of catecholamine-secreting tumors of the organ of Zuckerkandl.

In a series of 24 pheochromocytomas, four were tumors in the organ of Zuckerkandl. Their presenting symptoms of headache, palpitation, diaphoresis, and anxiety were similar to symptoms of adrenal pheochromocytomas. Two patients had paroxysmal and two patients sustained hypertension. All four had elevated urinary vanillylmandelic acid, metanephrine or catecholamine levels. Two of the tumors were localized with angiography before the availability of computerized tomography. Two patients underwent initial computerized tomography, which was nondiagnostic. Later scans looking specifically for organ of Zuckerkandl tumors were positive. Organ of Zuckerkandl tumors, like other ectopic pheochromocytomas, have a higher rate of malignancy. Two of the four were malignant tumors. One patient died 4 years after operation, but the other patient remains stable despite metastatic disease 8 years after operation. The two patients with benign tumors remain well 2 and 3 years after resection and treatment with alpha-blockade.

Adult↗

Hyperparathyroid crisis. Successful treatment of ten comatose patients.

Hyperparathyroid crisis is a rare disease manifested by elevated serum calcium, weakness, nausea and vomiting, altered states of consciousness, and elevated circulating parathormone. This hypercalcemic state is noted for a frequently acute presentation and associated high mortality rate, approaching 60% in some series. Ten patients in parathyroid crisis were observed in a consecutive personal series of 325 cases of operatively proved hyperparthyroidism. All 10 patients were successfully treated. Each patient remained or lapsed into persistent coma despite extensive medical management and normalization of serum calcium in some instances. An emergency parathyroidectomy was performed in all cases. Reversal of the comatose state was noted in all patients within 24 hours, followed by gradual normalization of serum calcium. Serum calcium ranged from 15 to 19.6 mg/dl. The blood urea nitrogen level was elevated in six patients. A single adenoma was found in nine patients and multiglandular disease involving the neck and the mediastinum in one. All patients survived. The successful treatment of this disease demands prompt and accurate diagnosis coupled with vigorous medical therapy and emergency parathyroidectomy if the patient's status continues to deteriorate.

Acute Disease↗

Primary intestinal lymphangiectasia. Comparison between endoscopic and radiological findings.

Two children with primary intestinal lymphangiectasia (PIL) are presented, both of whom had been prescribed an MCT supplemented diet low in long chain fatty acids. The hypoproteinemic edema improved in one patient, but persisted in the other. Following several years of dietary therapy, both subjects were re-evaluated, and a duodenoscopy and radiography of the digestive tract were performed. The duodenoscopic procedure evidenced two types of lymphangiectatic plaques on the surface of the duodenal mucosa: one form had a diameter of less than 1 mm, while the other exceeded 3 mm. The smaller lesions were seen in the child with the more favorable clinical course, whereas both types were observed in the other patient. Radiological examination disclosed the typical anomalies of intestinal lymphangiectasia only in the subject who had not responded to the dietary regimen. These results suggest that endoscopy affords a more precise assessment of the anatomic injury and could play an important role in formulating an accurate clinical diagnosis.

Child↗

Immune competence assessment in hyperbilirubinemic newborns before and after phototherapy.

Tests for immune competence in normal and hyperbilirubinemic newborns--before and after phototherapy--were assessed. The lymphoproliferative response to PHA-M in hyperbilirubinemic infants was inhibited in the presence of autologous plasma in comparison with normal infants and normal adults. The phototherapy does not seem to inhibit the lymphoproliferative response. After phototherapy, an increased level of IgM and a slight decrease in IgG concentration were found. These data support the possibility that phototherapy is not contraindicated during septic hyperbilirubinemia.

B-Lymphocytes↗

Pancreatic ascites in the first year of life.

A case of pancreatic ascites in a 4-month-old baby girl is reported. To the best of our knowledge only 4 previous cases occurring in the first year of life have been reported in the pediatric literature. In patients with unexplained ascites, amylase determination should be performed on ascitic fluid and serum. The baby was first treated by external drainage and later by internal drainage of the pseudocyst which had formed along the fistula.

Ascites↗

Sodium cromoglycate and carbachol-induced bronchoprovocation in asthmatic children.

Ten children with mild bronchial asthma underwent bronchial challenge with carbachol on three consecutive days; using a double-blind technique, they were given inhaled saline placebo on the first day and placebo or sodium cromoglycate (SCG) on the second and third days 15 min prior to the bronchial provocation test with carbachol. We did not find any significant difference between the baseline and placebo values, but we did obtain a statistically significant difference when comparing baseline with post-SCG and placebo with post-SCG values. These results demonstrate a protective effect of SCG on carbachol-induced bronchospasm in asthmatic children.

Adolescent↗

Relationships between salt sensitivity of blood pressure and sympathetic nervous system activity: a short review of evidence.

Experimental and clinical studies provided evidence in favor of complex relationships between sympathetic nervous system activity and salt-sensitivity of blood pressure. Genetic and acquired metabolic alterations associated with a tendency to retain salt and water may generate salt-sensitivity of blood pressure and shift the pressure-natriuresis curve to the right, promoting an increase in blood pressure. Sympathetic activation is a factor contributing to this result. Chronic high dietary salt intake is followed by a derangement in mechanisms of central sympathetic inhibition and then by an enhanced peripheral sympathetic tone. This, in turn, may generate salt-sensitivity of blood pressure by affecting renal hemodynamics, tubular sodium and water handling. Insulin resistance and sodium and water retention are prompted by high-fat (as well as high carbohydrate) diets, and by an increase in body fat mass. Also, aging is a condition of impaired interactions of the above factors. A gain in weight due to reduced physical activity, not followed by a parallel decrease in calorie intake, brings to a fall in insulin sensitivity. In many cases, the natural age-related decline of renal function is associated with a reduced physical exercise, hyperinsulinemia and sodium retention; sympathetic nervous system activity is enhanced and causes an increase in blood pressure.

Aging↗