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Biomedical subjects

D Yeste Fernández

Publications and source records attributed to D Yeste Fernández.

10 recordsLinked to original sources

[Glycogen storage disease type IX presenting as abdominal distention, hepatomegaly and hypoglycemia during infancy].

Glycogen storage diseases are a rare group of disorders in daily pediatric practice but must be taken into account when a patient presents with poor physical growth, hepatomegaly, hypoglycemia, hypotonia and/or other metabolic disturbances. Early diagnosis allows treatment that might improve the patient's outcome to be started or, at the very least, genetic counseling to be given to the parents. We present a 10-month-old boy who presented with growth retardation, abdominal distention and hepatomegaly and who was finally diagnosed with glycogenosis type IX. Definitive diagnosis was obtained by demonstrating the enzyme defect (phosphorylase beta-kinase) in affected tissues. Enteral nutrition was started using a diurnal high-carbohydrate diet with frequent feedings and nocturnal nasogastric continuous feeding, achieving optimal growth parameters and clinical response.

Glycogen Storage Disease↗

[Biliary atresia and polysplenia syndrome: outcome and growth post-transplantation].

Biliary atresia (BA) is the most common indication for liver transplantation in children. Approximately 7-10% of these patients have the associated polysplenia syndrome (PS). The prognosis of patients with BA and PS has been reported to be poorer than that in patients with BA without PS. All patients who underwent liver transplantation for BA and who still attend periodic controls at the outpatient clinic were considered. A retrospective study of outcome and growth in children with BA was made, and compared with a subgroup of patients with BA and PS. There were no significant differences on complications, liver and renal function tests, lipids and growth data. We concluded that BA and PS do not preclude successful liver transplantation.

Biliary Atresia↗

[Renovascular hypertension due to unilateral renal artery stenosis with hypokalemic alkalosis, the salt-losing syndrome and reversible hyperechogenicity of the contralateral kidney. A study of 2 infants].

This report describes two infants with severe arterial hypertension secondary to unilateral renal artery stenosis which was manifested by polyuria, polydipsia, hypokalemic alkalosis, hyponatremia, increased natriuresis and increased plasma values of rennin and aldosterone. On sonographic examination, the contralateral non-stenotic kidney of both patients appeared enlarged and hyperechogenic mimicking parenchymal lesion. When the patients became normotensive, their sodium and potassium balance became normal and their contralateral non-stenotic kidney also became normal in size and echogenicity. The increase of the filtration and the natriuresis observed in the contralateral non-stenotic kidney of the patients with renovascular hypertension due to renal artery stenosis might be responsible for the hyperechogenicity. When the patients became normotensive, the filtration and excretion of sodium of the contralateral kidney also became normal and the increase of echogenicity also disappeared. The reversibility of the sonographic findings suggest a functional origin.

Alkalosis↗

[Abnormal origin of the left coronary artery of the pulmonary artery trunk. Report of 2 cases].

Two cases are presented with anomalous origin of the left coronary artery from the pulmonary artery. Both cases initiate clinical symptoms in the first three months of life, defined by angina and congestive heart failure. In both cases cardiac catheterization showed the existence of anomalous origin of the left coronary artery and underdeveloped collateral circulation. Reimplantation of the anomalous coronary to ascending aorta on the two patients. Has excellent results on the first patient, but not in the second one who died during surgery.

Coronary Circulation↗

[Congenital mesoblastic nephroma].

Authors report three cases of congenital mesoblastic nephroma. In two of them, diagnosis was neonatal, while the third was made at the 3rd-month. They discuss diagnostic procedures employed as well as pathological findings. Presence of a mass was the first clinical sign in all three cases. Nephrectomy was the only treatment employed, with favourable outcome.

Female↗