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Biomedical subjects

A Bettinelli

Publications and source records attributed to A Bettinelli.

At least 37 records · Page 2Linked to original sources
PubMed1993

Long-term follow-up of a patient with Gitelman's syndrome.

The long-term follow-up (from age 6 to 20 years) of a girl with Gitelman's syndrome, who had four hypomagnesaemic-tetanic episodes associated with normal plasma calcium, hypokalemia and hypocalciuria, is presented. During and after puberty, hypomagnesaemia was of the order of 0.41-0.49 mmol/l and plasma potassium was at the lower reference limit. The long-term clinical course and growth of this patient appeared good, but, magnesium supplementation reduces the risk of tetanic crises.

Child↗
PubMed1993

Renal effects of cyclosporin A in children treated for idiopathic nephrotic syndrome.

Little data have been published on tubular renal function during cyclosporin A treatment in children without transplants. We studied 12 young subjects (mean age 10 years) with steroid-responsive idiopathic nephrotic syndrome and with signs of steroid toxicity. After achieving remission with prednisone 60 mg/m2, 8 children started cyclosporin A therapy (6 mg/kg/day) (group A) and 4 children were given cyclophosphamide 2.5 mg/kg/day (group B). The latter were considered as controls together with 10 other children with idiopathic nephrotic syndrome in complete remission and off therapy (group C). We monitored creatinine clearance and tubular handling of beta 2-microglobulin, sodium, phosphorus and uric acid for one year. Cyclosporin A induced a decrease in creatinine clearance with a decrease in fractional excretion of beta 2-microglobulin; sodium excretion was similar in the two treated groups and a transient decrease in fractional excretion of uric acid was seen only in patients receiving cyclosporin A. Both groups showed an increased renal threshold phosphate concentration. Our results suggest that in children, cyclosporin A therapy induces a decrease in glomerular filtration rate associated with increased reabsorption activity of proximal tubular cells.

Adolescent↗
PubMed1992

Energy and nutrient intake of patients with mild-to-moderate chronic renal failure compared with healthy children: an Italian multicentre study.

Nutritional counselling is important in the management of children with chronic renal failure (CRF). In 1988, a controlled European multicentre study was started to evaluate the effects of a low-protein diet on the progression of CRF in children. To assess the energy, macro- and micronutrient intake, 4-day weighed dietary records were obtained from 50 children with low to moderate CRF (creatinine clearance 65 to 15 ml/min per 1.73 m2) and from 93 healthy children. The mean energy intake was 90%-93% of the recommended dietary allowance for Italian children in controls and 76%-88% in CRF patients. The mean protein intake was 2.1-3.1 g/kg per day in controls and 1.6-2.7 g/kg per day in CRF patients. Overall, the energy intake was 10% and the protein intake 33% lower in CRF patients than in healthy children. Children with CRF consumed less cholesterol, calcium and phosphorus than healthy children. The lower spontaneous intake of energy, protein and other nutrients should be taken into account when planning the nutrition of children with CRF.

Child↗
PubMed1992

Use of calcium excretion values to distinguish two forms of primary renal tubular hypokalemic alkalosis: Bartter and Gitelman syndromes.

Clinical or biochemical findings were reevaluated in 34 pediatric patients with primary renal tubular hypokalemic metabolic alkalosis. The patients were subdivided into two groups. Bartter syndrome (primary renal tubular hypokalemic metabolic alkalosis with normocalciuria or hypercalciuria) was diagnosed in 18 patients with molar urinary calcium/creatinine ratios greater than 0.20, and Gitelman syndrome (primary renal tubular hypokalemic metabolic alkalosis with magnesium deficiency and hypocalciuria) was diagnosed in 16 patients with molar urinary calcium/creatinine ratios less than or equal to 0.20 and plasma magnesium levels less than 0.75 mmol/L. Some clinically important differences between the groups were observed. Patients with Bartter syndrome were often born after pregnancies complicated by polyhydramnios (8/18) or premature delivery (7/18) and had short stature (11/18) or polyuria, polydipsia, and a tendency to dehydration (16/18) during infancy (12/18) or before school age (18/18). Patients with Gitelman syndrome had tetanic episodes (12/16) or short stature (3/16) at school age (14/16). We conclude that the Bartter and Gitelman syndromes represent two distinct variants of primary renal tubular hypokalemic metabolic alkalosis and are easily distinguished on the basis of urinary calcium levels.

Alkalosis↗
PubMed1991

Acute effects of calcitriol and phosphate salts on mineral metabolism in children with hypophosphatemic rickets.

We investigated the acute effects of oral administration of 1,25-dihydroxyvitamin D (1,25-(OH)2D) and phosphate on the major mineral metabolism indexes in six children with vitamin D-resistant rickets treated with a long-term regimen of phosphate and calcitriol. Two acute tests were performed in which plasma calcium, phosphate, immunoreactive parathyroid hormone (iPTH) (intact molecule), 25-hydroxyvitamin D (25-OHD), and 1,25-(OH)2D levels were measured: the first after an oral phosphate load (20 mg/kg) was administered after calcitriol had been discontinued for 10 days, and the second after a calcitriol load (0.03 microgram/kg) plus the same phosphate load but with the children receiving the usual combination treatment. There were no significant differences in basal levels of calcium, phosphate, iPTH, 25-OHD, or 1,25-(OH)2D between the two tests, nor were delta percent calcium and 25-OHD values significantly different. The delta percent plasma phosphate concentration at 60 minutes was significantly higher during test 2 than during test 1 (p less than 0.01) and delta percent iPTH concentration at 60 minutes was significantly higher during test 1 than during test 2 (p less than 0.01). In test 2 the iPTH level returned to baseline at 180 minutes. Higher delta percent 1,25-(OH)2D values at 60 minutes were observed in test 2 than in test 1 (p less than 0.01). Furthermore, the delta percent 1,25-(OH)2D levels were still higher at 180 minutes in test 2 than during test 1 (p less than 0.01). Our study indicates that oral calcitriol has an inhibitory effect on iPTH secretion in the hours immediately after oral phosphate administration in children with vitamin D-resistant rickets.

25-Hydroxyvitamin D 2↗
PubMed1991

Seizures and transient blindness following intravenous pulse methylprednisolone in children with primary glomerulonephritis.

Two children, 1 with idiopathic nephrotic syndrome and 1 with endo-extracapillary glomerulonephritis, presented an episode of seizures and transient blindness at different times after i.v. pulse methylprednisolone (IVPMP) treatment. Neurological manifestations in patient 1 could be due to hypertension secondary to IVPMP, while the pathogenesis of such manifestations remained difficult to clarify in patient 2. The severity of uremia in patient 2 could be one of the conditions that, in association with the abrupt changes obtained with IVPMP, predispose to neurological manifestations. Careful clinical and biochemical monitoring seems necessary in children with primary glomerulonephritis, other than those transplanted, after IVPMP, and factors predisposing to neurologic sequelae should be further defined.

Blindness↗
PubMed1989

Ex novo episodes of acute glomerulonephritis and Guillain-Barré syndrome: a case report.

A nine-year-old girl had several ex novo episodes of acute glomerulonephritis with clinical evidence of rapid progression in two of them. Guillain-Barré syndrome was diagnosed 10 days after the second episode of acute glomerulonephritis. Two renal biopsies (performed at about a four-year interval) gave morphological evidence of new episodes of acute glomerulonephritis. To our knowledge this is the first report of an association between ex novo episodes of acute glomerulonephritis and Guillain-Barré syndrome.

Acute Disease↗
PubMed1986

Effects of 1,25-dihydroxyvitamin-D3 treatment on mineral balance in children with end stage renal disease undergoing chronic hemofiltration.

Ten children with end stage renal disease on chronic hemofiltration (HF) were studied for a 1-yr period to evaluate the efficacy of 1,25-dihydroxyvitamin-D3 (1,25(OH)2D3) therapy on biohumoral parameters of renal osteodystrophy and bone mineral content. In six of these children an acute study was done of the direct effect of the HF procedure on calcium and phosphate balance during 12 HF sessions. During the first 6 months of the study all children were treated with 1,25(OH)2D3 (0.25-0.50 microgram/day) to maintain plasma calcium at 9.5-11.0 mg/dl. There was a significant increase in plasma calcium (p less than 0.05) and a significant decrease in plasma phosphate (p less than 0.01) and alkaline phosphatase concentrations (p less than 0.05). The circulating levels of NH2 immunoreactive parathyroid hormone did not change, remaining at the upper limits of reference values. Immunoreactive parathyroid hormone-COOH terminal fragment levels decreased significantly (p less than 0.05). Bone mineral content rose significantly (p less than 0.01). During the last 6 months of the study, to evaluate the possibility that HF alone might control secondary hyperparathyroidism, 1,25(OH)2D3 treatment was discontinued in five children; plasma calcium and phosphate were well controlled whereas hyperparathyroidism worsened in all five, and one also developed intense pruritus and hypertension. The other five children remained on 1,25(OH)2D3 treatment; two of these were transplanted, and the other three continued to show an improvement of mineral balance. The results of the acute study showed that calcium balance was positive with a mean Ca++ gain of 140 mg/HF session. The mean total phosphate removed per HF run was 574 mg.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1986

Mineral metabolism during a hemofiltration session in children.

We studied the effects of one session of hemofiltration on the mineral metabolism in a group of uremic children. We observed an increase of calcemia, accompanied by positive calcium balance, and a decrease of plasma phosphate and PTH-MM. No substantial loss of plasma 25-OH D was found. It is concluded that hemofiltration has a positive effect on calcemia and is very effective in removing phosphate, but it acts only temporarily on PTH removal. An adequate calcium concentration must be provided in the substitution fluid.

Adolescent↗
PubMed1985

The influence of hyperparathyroidism on glucose metabolism in uremia.

We studied glucose metabolism in a group of adolescents and young adults with uremia using the hyperglycemic clamp technique. In eight adolescent patients, the glucose metabolic rate correlated negatively with PTH levels and positively with the glomerular filtration rate. Six patients, one adolescent and five adults on regular hemodialysis with severe hyperparathyroidism, had low glucose metabolic rates and reduced insulin sensitivity compared to normal subjects. After parathyroidectomy, the glucose metabolic rate improved by 47%; plasma insulin concentrations during hyperglycemia increased by 37%, and insulin sensitivity did not change significantly. Thus, correction of hyperparathyroidism was associated with normalization of glucose metabolic rates and increased insulin secretion, but insulin resistance did not change.

Adolescent↗
PubMed1985

Nitrogen balance: an adequate treatment prescription index for children on haemofiltration.

We studied 12 children (10.6 +/- 2.5 years; 21 +/- 4.8 kg body weight) with end-stage renal disease on chronic haemofiltration prescribed using a urea kinetic model. After 12 months, urea generation rate, protein catabolic rate and nitrogen balance were assessed in each patient. Pre-treatment BUN was adequate in all 12 patients but in three nitrogen balance and growth rate were unsatisfactory. These three children were studied for a second year in which plasma water cleared/week was increased; during this year a significant increase in nitrogen balance and growth rate was obtained.

Blood↗
PubMed1985

Intraplatelet serotonin (I5HT) in children with the hemolytic uremic syndrome.

I5HT levels were investigated in 14 children with Hemolytic Uremic Syndrome (HUS). Low levels of I5HT were demonstrated in all the children studied during the early phase of the disease, indicating reversible platelet aggregation and recirculation of degranulated platelets. Three months after the onset of HUS, only 2 children with a pathological picture of cortical necrosis and a mild degree of chronic renal failure had low I5HT levels. On the contrary, normal I5HT levels were present in the other children completely recovered from HUS and with a renal biopsy picture of thrombotic microangiopathy with predominant glomerular involvement. We conclude that normal values of I5HT, after the acute stage of HUS, are a good index of complete recovery from the disease. Further observations are required to assess the prognostic value of low I5HT levels in children with chronic renal failure secondary to HUS.

Adolescent↗
PubMed1984

[Radiotherapy of brain tumors in childhood].

External radiotherapy with high energy photons in children's brain tumors improves results of surgical treatment according to their histology. Radiotherapy treatment is a decisive factor in survival time and quality of life. Undifferentiated tumors with high radiosensitivity are treated when radiotherapy is the only means for recovery; tumors presenting histologically low malignancy in which effective radiotherapy treatment is uncertain and the risk of radiotherapy damage is the predominant factor. Radiotherapists who treat children's brains which in many cases are not yet totally myelinized , must have good technical ability. Literature and personal experience show that in medulloblastomas irradiation of the entire central nervous system associated with chemotherapy gives a survival time of 5 years in 50% of the cases; in glioblastomas and anaplastic astrocytomas, fortunately rare in children, results are poor; in brainstem tumors the association of the extracranial shunt to radiotherapy has improved results of survival time at 5 years to 50%. A good quality of life is frequent and radiological effects are rare. It is clear that radiotherapy has reached its maximum. Progress is still to be made thanks to discoveries of more effective radiosensitizer or radioprotector compounds.

Adolescent↗
PubMed1983

Use of urea kinetic model and haemofiltration to improve nitrogen balance in haemodialysed children.

To improve nitrogen balance and nutritional status, 10 paediatric patients (11.2 +/- 2.7 years; 28.5 +/- 5.1 kg) on regular haemodialysis (HD) for 25.3 +/- 1.2 months were treated with haemofiltration (HF) for 12.2 +/- 3.4 months. HF was prescribed using a urea kinetic model to ensure a pre-treatment blood urea nitrogen (BUN) of 130 mg/100ml. Urea generation rate (Gu) and protein catabolic rate (PCR) were computed by urea kinetics. Protein and calorie intake was assessed in the children on an unrestricted diet by analysis of 10 days' dietary records obtained every two months. During HF treatment a significant decrease of Gu and PCR was obtained and nitrogen balance increased in all the children.

Adolescent↗