Late onset central hypoventilation syndrome.
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Biomedical subjects
Publications and source records attributed to J Lopez-Herce.
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A newborn female diagnosed with transposition of the great vessels with restrictive ventricular septal defect presented left facial peripheral nerve paralysis following anatomical surgery correction (arterial switch) by cardiopulmonary bypass. We have not found any causal factor either in the anesthesia or postoperative period. The electromyogram presented signs of peripheral nerve impairment, and the cerebral echography and electroencephalogram were normal. The facial nerve paralysis was almost recovered seven weeks after surgery. This is the first pediatric patient reported with peripheral facial nerve paralysis after cardiac surgery.
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The levels of prostaglandin (PG) E2 and 6-keto-PGF1 alpha (stable metabolite of prostacyclin) in plasma and gastric juice were determined in 113 critically ill children and adolescent, and compared to those registered in a plasma control group of 24 children and a gastric juice control group of 15. The gastric juice concentration of PGE2 is our patients [9.2 +/- 3.1 (SEM) pg/ml] was significantly lower (p = 0.001) than in the control group [81.1 +/- 18.1 (SEM) pg/ml]. There were no differences in plasma levels of PGE2 and plasma gastric juice levels of 6-keto-PGF1 alpha between the patients and the control groups. Children who died had lower plasma levels of PGE2 [6.2 +/- 2.2 (SEM) pg/ml] and gastric juice levels of PGE2 [2.3 +/- 0.8 (SEM) pg/ml] than the survivors (p less than 0.05). The gastric juice concentration of PGE2 was also lower in children who suffered important upper gastrointestinal bleeding, although the difference did not reach statistical significance.
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Continuous arteriovenous haemofiltration (CAVH) was used in 7 critically ill children: a premature neonate with hypervolaemia secondary to hydrops foetalis and six children aged 9 days to 7 years with acute oliguric renal failure. Biospal 0.5 m2, Renaflo 0.25 m2, Gambro 0.15 m2 and Amicon 0.015 m2 filters were used according to the weights and ages of the patients. Adequate removal of water and solutes was obtained in 6 patients. One of the patients with the smallest filter needed a change to a filter with a larger surface area to improve water and solute removal. Haemofiltration was maintained for between 17 hours and 31 days and was well tolerated. CAVH was discontinued because of recovery of renal function in three patients, improvement of the hypervolaemic state in one, death in three, and transfer to continuous ambulatory peritoneal dialysis because of chronic renal failure in one patient. CAVH is a useful technique for the treatment of acute renal failure and hypervolaemia in critically ill children.
Eight hypertensive crises (HC) were treated with 2.5 mg of sublingual nifedipine in three children with weights below 10 kg (group A); 16 HC in 6 children between 10 and 20 kg with 5 mg (group B); and 40 HC in 10 children over 20 kg with 10 mg of nifedipine (group C). The relative decrease in both systolic and diastolic blood pressure was similar in all groups. The decrease was more rapid in groups A and B when the contents were extracted from the capsule and given directly. The hypotensive effect lasted 4 h. There were no side effects. The effective and safe single dose of nifedipine has been established to be 2.5 mg for children weighing less than 10 kg, and 5 mg for children weighing between 10 and 20 kg.
We report 31 episodes of hypertensive crises in children, managed with sublingual nifedipine at the following dosages: 10 mg in children with body weight (BW) higher than 20 kg, 5 mg in children with BW between 10 and 20 kg, and 2.5 mg in children with BW below 10 kg. The mean initial blood pressures were 161.41 mm Hg for the systolic pressure (mSBP) and 111.25 mm Hg for the diastolic pressure (mDBP). After nifedipine, both the mSBP and the mDBP decreased, with onset of effect five minutes after dosage and maximum decrease at 60 min (mSBP 134.93 mm Hg, mDBP 79.23 mm Hg, for decreases of 16.4 and 28.7%, respectively), and this effect persisted for 180 min. Blood pressure increased again from min 240 to min 360, yet without reaching the initial levels. One case did not respond to the first dose of nifedipine and required a second one. The effect of nifedipine was more pronounced on the DBP than on the SBP, and greater reductions of both pressures were achieved in the cases with higher initial readings. No side of medication were observed in our patients.
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A female infant with an intermediate variant of maple syrup urine disease is described. The patient had psychomotor retardation and high plasma levels of branched chain aminoacids. Leucine decarboxylation rate in leukocytes was diminished. Cranial computed tomography showed decreased density in the cerebral white matter. After starting dietary treatment the infant resumed her psychomotor development and the abnormal images previously seen on computed tomography disappeared.
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