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Biomedical subjects

J Rodríguez Soriano

Publications and source records attributed to J Rodríguez Soriano.

At least 19 recordsLinked to original sources

[Long-term prognosis of childhood IgA nephropathy in adult life].

BACKGROUND: To evaluate long-term prognosis in a group of children with IgA nephropathy and to analyse which clinical factors were associated with progression to chronic renal failure in adulthood. PATIENTS AND METHOD: Retrospective study. 58 young adults with IgA nephropathy diagnosed at 10.6(SD 2.9) years old and studied after a follow-up of 11.8 (SD 2.9) years. RESULTS: Relapses of macroscopic hematuria and proteinuria were the most frequent symptoms at onset (75.9%). In 25.9% of patients high plasmatic IgA levels were also detected. Most cases had grade I (44.8%)or grade II (44.8%) histological lesions at diagnosis. At the last control, clinical remision was observed in 21 patients (36.2%) and 50% of the whole group remained with abnormal urine. 8 patients(13.8%) reached terminal renal failure. Mean renal survival (defined as glomerular filtration rate above 50 ml/min/1.73 m2)was 100, 93.3 and 81.1% at 5, 10 and 15 years of evolution, respectively. CONCLUSIONS: About 14% of children with IgA nephropathy had long-term renal bad prognosis. Hypertension at onset, plasma creatinine elevation and proteinuria during adolescence were significant risk factors associated with chronic renal failure during adulthood. Minimal lesions at IgA nephropathy diagnosis in children did not exclude long-term poor prognosis.

Adult↗

[Treatment of idiopathic nephrotic syndrome with cyclosporin A].

OBJECTIVE: Cyclosporin A (CyA) has been used in steroid-dependent and steroid-resistant nephrotic syndrome (NS) with the aim to prolong or to induce remission, respectively. PATIENTS AND METHODS: The efficacy and side-effects of CyA therapy were evaluated in 25 children with idiopathic NS. Twelve patients had steroid-dependent NS and 13 patients had steroid-resistant NS. In all cases, CyA was given as a third alternative drug, once therapies with prednisone and alkylating agents had failed. In steroid-resistant patients CyA administration was always associated with low-dose prednisone. RESULTS: All 12 patients with steroid-dependent NS entered into remission during CyA administration, but 7 patients relapsed when the drug was withdrawn or tapered and 7 of 8 patients requiring long-term therapy continued to present new relapses. Prednisone requirement was lower and growth velocity higher during the year on CyA therapy than during the year preceding CyA therapy. Only 5 of the 13 patients with steroid-resistant NS had a complete remission. Three of these patients relapsed upon cessation of therapy, but these relapses became steroid-sensitive. Clinical side-effects (hirsutism, gum hyperplasia, arterial hypertension) were only observed in a few patients. Biochemical side-effects (hyperuricemia, hypomagnesemia) were more frequently observed, but always reverted upon cessation of therapy. The development of osteosarcoma in one patient may represent a coincidental finding. CONCLUSIONS: The results suggest that CyA therapy is capable of inducing remission in all patients with steroid-dependent NS and in about one third of patients with steroid-resistant NS. However, most patients relapse when the CyA is stopped and require long-term therapy, often associated with administration of predisone.

Adolescent↗

Polyunsaturated fatty acid status in patients with phenylketonuria.

To determine whether the n-6 and n-3 polyunsaturated fatty acid levels can be affected by the PKU diet, fatty acid composition was studied in PKU subjects and controls together with their lipid intake. The study population consisted of 40 PKU patients treated with a phenylalanine-restricted diet. Controls were 50 children on whom preoperative tests had been carried out for minor surgery. Methyl esters were obtained from plasma and red blood-cells and separated by gas chromatography. PKU patients showed lower docosahexaenoic acid levels (22: 6 n-3) (p < 0.001) and higher n-6 series levels in plasma (18:2 n-6) (p < 0.001) and in red blood-cells (20: 4 n-6) (p < 0.001) than the control population. Since docosahexaenoic and arachidonic acids are fundamental structural components for normal brain development, it seems necessary to consider a dietary adjustment in these patients.

Adolescent↗

[Cardiovascular risk in the pediatric population. Plasma fatty acid levels].

Thirty-seven offspring of patients who had acute myocardial infarctions were investigated. Serum fatty acids of the whole plasma lipids were evaluated within the frame of a full biochemical assessment. Baseline values were obtained and the children were subsequently put on appropriate diets and advised to cut-out smoking and alcohol. The same values were obtained following one year on such a regimen. Results were compared with those found in a control group. Baseline palmitoleic and dihomogammalinolenic acid serum levels were significantly raised in the study group. Values of palmitoleic, but not of dihomogammalinolenic acid, had returned to normal by the reassessment one year later.

Adolescent↗

[Other indications for growth hormone treatment: chronic kidney failure and Turner's syndrome].

Recently, it has been demonstrated that treatment with growth hormone (GH) can accelerate height velocity in children with chronic renal insufficiency (CRI), after kidney transplantation and in Turner syndrome. The pathogenesis of growth retardation in CRI is complex. Possibly the most important factor involved is the presence of peripheral resistance to insulin-like growth factors. The administration of GH in high doses may restore catch-up growth, both in children with CRI and in kidney post-transplant patients. After 12 months of treatment height velocity increased from 4.3 to 6.6 cm/year in 4 children with CRI, and from 2.5 to 7.4 cm/year in 3 patients with kidney transplant. GH therapy alone, but even more in combination with oxandrolone increases the growth rate in Turner syndrome and may increase the final height, but the long-term results of this treatment are awaited. No undesirable collateral effects have been reported.

Body Height↗

[Intolerance to cow's milk proteins. An epidemiologic study].

The incidence of cow's milk protein intolerance (CMPI) varies depending on the series. The aim of the present investigation was to determine the incidence of this disease in our population and its change over the past the years. Over the period January 1977 to December 1986, 217 patients suspected to have the disease were submitted to a cow's milk challenge test. Diagnosis was confirmed clinically and/or pathologically in 121 patients and was ruled out in 96 patients. The incidence for the whole period was 0.78 cases/1,000 alive newborn infants. However, it fell significantly over the ten years, with a maximum of 1.36/1,000 alive newborn infants in 1979 and a minimum of 0.17/1,000 alive newborn infants in 1986 (r = 0.76, p less than 0.001). We conclude that, similarly to the observation in other diseases of nutritional origin, the incidence in our population of CMPI has clearly decreased, probably as a consequence of changes in the dietary habits.

Cohort Studies↗

[Restricted intake of sodium in newborn infants with very low birth weight in the first days of life].

A prospective study on the amount of sodium administered in the first five days of life in a group of 18 low-birth-weight infants is presented. Their mean birth weight, and gestational age were 1.150 gm., and 28.8 weeks. Patients were cared for in radiant warmers, and protected with a plastic blanket. Minimum amount of sodium necessary to maintain a normal plasma sodium concentration was given. Mean daily sodium and free water given was 0.96 mEq/kg/day, and 108.4 ml/kg/day, respectively. In day 5, there was a mean accumulated body weight loss of ten per cent. This group of patients were compared with a similar group in whom plastic blanket was not used, and who received a sodium intake of 1.5 mEq/kg/day. In this latter group of patients there was a higher incidence of hypernatremia (p less than 0.005). The incidence of hyponatremia was similar in both groups. Daily sodium intake might be very low, in infants of very-low-birth-weight if a negative weight balance is allowed in the first days of life.

Drinking↗

[Regional organization of a neonatal transportation system].

Organization, operational plans, and initial experience of a Regional Neonatal Transport System is described. The system covers provinces of Bizcaia, and Alava in Northern Spain, with a total of 17,500 annual deliveries. In the first 10 months 31 newborn infants were transported. Their mean birth weight was 2,066 +/- +/- 931 Gm. Ten infants had a birth weight less than 1,500 Gm, and in 22 (71%) of them gestational age was under 37 weeks. Some types of respiratory distress was present in 71% of them, and 25.8% required mechanical ventilation during transport. Hypothermia was present in 36% of the infants at referral center, percentage that decreased to only 9% at their arrival to the NICU. During transport mean body temperature increased from 35.33 +/- 1.07 degrees to 35.84 +/- 0.9 degrees C (p less than 0.01). Infants condition was judged to improve in 13% of them. Overall survival rate was 74.2%, being 80.9% in those weighting from 1,000 to 1,500 Gm. Infants who were hypothermic had a lower survival (45.5%) than those who were not (90%) (p less than 0.05).

Birth Weight↗