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Biomedical subjects

E Leumann

Publications and source records attributed to E Leumann.

At least 37 records · Page 2Linked to original sources
PubMed1997

Pyelonephritis and vesicoureteral reflux after renal transplantation in young children.

PURPOSE: We assessed morbidity and risk factors of pyelonephritis in children after renal transplantation. MATERIALS AND METHODS: Between 1986 and 1995, 41 children underwent transplantation and all who had documented pyelonephritis were evaluated. RESULTS: Six children who underwent transplantation before age 7 years had 1 to 3 episodes of pyelonephritis with significant renal dysfunction and vesicoureteral reflux into the grafted system. An antireflux reimplantation procedure in 5 children was complicated by temporary functional obstruction in 3. No further infection occurred after correction of vesicoureteral reflux. After a median of 4.5 years post-transplantation all patients have a functioning graft. CONCLUSIONS: After renal transplantation vesicoureteral reflux and young recipient age are major risk factors for pyelonephritis with subsequent graft dysfunction.

Adolescent↗
PubMed1996

[25 years of dialysis and kidney transplantation in children and adolescents].

Between 1970 and 1994 84 children and adolescents with end stage renal failure (ESRF) were started on renal replacement therapy (RRT). Renal transplantation was the main goal from the beginning. The long term results were evaluated with emphasis on survival, development and social integration. RRT was started in nearly half of the children (45%) between the age of 10 and 15 years and in 14% before 5 years. 52 patients were first treated by hemodialysis (HD) and 27 by peritoneal dialysis (PD, since 1979). 5 patients underwent preemptive transplantation. By December 1994, 75 patients had received 99 grafts, i.e. 75 1st, 21 2nd and 3 3rd grafts; 3 kidneys were from living related donors and 8 patients were transplanted elsewhere. 7 patients were still on dialysis and 2 had died before transplantation. Actuarial patient survival (Kaplan-Meier) after start of RRT is 88% at 10 years and 75% at 17-25 years. Actuarial patient survival after first transplantation increased from 91% (1970-1984) at 5 years to 97% (1985-1994). 7 of the first 10 patients transplanted from 1970-1974 are alive, all with functioning grafts (4 with their first graft). 9 patients died after transplantation: 4 of recurrent disease, 2 of viral (CMV, EBV) infections and 1 each of spinalioma, allergic shock and traffic accident. First graft survival was 37% at 10 years. It increased from 53% (1970-1984) to 72% (1985-1994) at 5 years. The main causes of first graft loss (n = 33) were irreversible rejection (21) and recurrent disease (7). All patients aged > 22 years were further evaluated: patients with start of RRT 1970-1979 (group A, n = 18) were compared with those starting RRT from 1980-1987 (B, n = 19). Mean adult height in A was less than in B (163.9 cm vs 168.5 cm in men; 146.3 cm vs 156.5 cm in women). 50% in A vs 32% in B had a disability. Fewer patients in A (39%) than in B (62%) were fully employed. Considerably more patients in A (61%) than in B (37%) lived with their parents or siblings although the mean age in A was 31 years vs 25 years in B. 3 women were married (all in B), 2 of them gave birth to 3 healthy children and the third was pregnant. Long term patient and graft survival, somatic development and social integration have improved over the years due to a variety of factors. A comprehensive approach is necessary in treating children with ESRF.

Adolescent↗
PubMed1995

Prenatal sonographic diagnosis of autosomal recessive polycystic kidney disease (ARPKD) during the early second trimester.

Autosomal recessive polycystic kidney disease (ARPKD) is a rare hereditary disease with a high neonatal mortality. Currently, prenatal diagnosis is possible only during the second half of pregnancy, when bilaterally enlarged, echogenic kidneys are visible by ultrasound. We describe a case in which a diagnosis of ARPKD was sought in the first half of pregnancy. High-resolution ultrasonography revealed echogenic, normal-sized kidneys at 15 + 4 weeks. Microsatellite DNA analysis of a chorionic villus sample, parental blood, and blood of an affected sibling showed that the fetus had the maternal haplotype and a recombination of the paternal haplotype. Thus, no distinction between homo- and heterozygosity for the ARPKD mutation in the fetus was possible. A further ultrasound examination at 19 + 4 weeks confirmed the previous results, indicating that the fetus was likely to be affected. After termination of the pregnancy, the diagnosis was confirmed on microscopic examination.

Adult↗
PubMed1995

Changing pattern of primary hyperoxaluria in Switzerland.

BACKGROUND: The clinical course of primary hyperoxaluria (PH) is greatly variable and diagnosis is often delayed. Little is known about the overall occurrence and current prognosis. METHODS: We evaluated all known patients with PH residing and observed in Switzerland during the last 15 years with the help of a survey among Swiss nephrologists. RESULTS: Of the 25 patients observed between 7/79 and 6/94 in Switzerland, 18 were alive in 1994-14 on conservative therapy and four on renal replacement therapy (RRT). Twenty-two patients had PH type 1; the exact type was not determined in three. The estimated prevalence of PH (type 1) is 2 per million population; the minimal incidence is 1 per 100,000 live births. Diagnosis was delayed by 8 years (median) except in infants. Five patients were pyridoxine sensitive. According to life table analysis, 20% of patients were in end-stage renal failure (ESRF) and 10% had died by the age of 15 years, and 50% were in ESRF and 20% dead at 25 years. Prognosis has improved: Five of 13 patients died during the first half of the observation period as opposed to two of 20 in the second part. CONCLUSIONS: Overall prognosis appears better than hitherto believed considering the large clinical spectrum of PH. Greater awareness of PH is needed to improve further long-term prognosis.

Adolescent↗
PubMed1995

Efficacy of oral citrate administration in primary hyperoxaluria.

Urinary citrate is a potent inhibitor of calcium oxalate (CaOx) crystallization, but oral citrate has rarely been used in patients with primary hyperoxaluria (PH). We studied the effect of sodium citrate administration (0.1-0.15 g/kg/day) on urinary citrate excretion and CaOx saturation in seven paediatric patients and the clinical response to long-term treatment (average 4 years) in five patients. Urinary citrate increased from 0.73 to 2.54 mmol/24 h/1.73 m2 and urinary saturation for CaOx (calculated by equil 2) decreased from 11.41 to 6.79 (for both, p < 0.02). Long-term administration of alkali citrate [0.15 g (0.5 mmol)/kg/day] resulted in stable or improved renal function in three and slow deterioration in two partially non-compliant patients. Alkali citrate is effective in patients with PH.

Administration, Oral↗
PubMed1994

[Therapy with growth hormone in pediatric patients with chronic kidney insufficiency].

Statural growth in children with chronic renal failure (CRF) is often delayed. Several studies have shown that one of the main causes is partial resistance to growth hormone (GH), which can be overcome by supraphysiologic doses of recombinant human (rh) GH. Since August 1990 we have been treating 8 boys and 1 girl (ages 1.7-12.7, mean 5.3 yrs) with rhGH (4 IU/m2 s.c. daily). All patients were prepubertal and were 2.4-4.5 (mean 3.2) SDS below the mean normal height for age and sex. 5 patients were on dialysis. Mean growth velocity was -2.0 SDS (5.4 cm/yr) before and +2.6 SDS (9.5 cm/yr) during the first year of therapy with rhGH. Mean height increased from -3.2 to -2.5 SDS at 1 year. Height in the 4 boys treated > 2 yrs with rhGH improved from -3.3 SDS (before rhGH) to -2.4 (1st yr) and to -2.0 SDS (2nd yr); their height increased by 9.6 cm (1st yr) and 7.8 cm (2nd yr) as compared to 4.6 cm in the year before treatment. The response to rhGH was better in the 4 patients treated conservatively than in those on dialysis. Side effects did not occur. Plasma insulin increased but the oral glucose tolerance test remained normal. We conclude that treatment with rhGH represents an important step forward in selected pediatric patients with CRF. The excellent acceptance reflects the high expectations of the patients and their families.

Child↗
PubMed1994

[Cyclosporin in autoimmune diseases].

At a symposium held in Zurich in November 1993, a series of presentations covered the optimal use of cyclosporin A (CsA) in autoimmune diseases. Besides its immunosuppressive effects in organ transplantation, CsA appears to be active in a variety of autoimmune disorders. Its most pronounced and beneficial clinical effects are seen in patients with severe rheumatoid arthritis. New clinical studies include the nephrotic syndrome in children and patients with systemic lupus erythematodes. The main concern remains CsA induced nephrotoxicity. For the treatment of autoimmune disorders CsA can be used at lower doses (< or = 5 mg/kg per day) than in transplantation medicine. Thus, monitoring of CsA blood concentrations is not usually necessary. The galenics of CsA has been optimized recently, an achievement which will further improve the clinical use of CsA. Due to increased knowledge of intracellular CsA binding proteins and of effects of CsA on T-lymphocytes and cytokine production, it can be expected that novel immunosuppressive compounds will be developed.

Adult↗
PubMed1994

Paediatric nephrology in countries with limited resources.

This paper summarises a symposium concerned with the provision of care for children with kidney disease in developing countries. Better organisation of services is required to prevent waste of resources, with the emphasis on team work between professionals, shared care with local health care personnel remote from the paediatric nephrology unit and good communications. Families need to be educated and provided with appropriate information so that they can care for their child at home. Technology should be simple and robust and the staff using it should be fully trained to maintain it in use. Therapies should be definitive where possible, because long-term supervision of treatment is often difficult. Effective but inexpensive medications should be used where possible. Twinning of developing and richer countries is valuable to transfer technology, help with training and assist in care through the development of personal contacts.

Child, Preschool↗
PubMed1994

From dialysis to basic paediatric nephrology: an unorthodox project applied in Yerevan, Armenia.

Following the earthquake in December 1988, a long-term project was developed: starting from haemodialysis, it was extended to basic nephrology and urology, with a programme for schooling and psychosocial care. Medical training had first priority. The programme relied on robust and often second-hand equipment and voluntary work. The approach chosen (an inverse help programme, from sophisticated to basic medicine) strongly stimulated motivation and proved to be practicable.

Adolescent↗
PubMed1994

The role of surgery and lithotripsy in childhood urolithiasis.

From 1982 through 1993 36 interventions in 32 infants and children with urolithiasis have been performed in our department. There were 22 boys and 10 girls. The main cause for the interventions have been congenital malformations in 9, followed by recurrent urinary tract infections in 6, previous operations in 5 and hypercalciuria in 5 patients. In 7 children the cause of the urolithiasis remained unknown. As a therapeutic option extracorporeal shock-wave lithotripsy (ESWL) was available after 1988. The malformations as well as the postoperative conditions have been corrected surgically and the calculi were removed at the same time. For post-infectious, idiopathic or hypercalciuric calculi ESWL was used in the majority of patients after 1988. Therefore a total of 26 calculi were removed by operation and 13 by ESWL. Only 1 calculus was extracted endoscopically. There were no complications either after ESWL or after open surgery. Our results show that ESWL gains more and more importance in infants and children. Calculi in congenital malformations or as result of operations are still removed by open surgery. In the other cases ESWL is the method of choice.

Adolescent↗
PubMed1993

Management of primary hyperoxaluria: efficacy of oral citrate administration.

The prognosis of primary hyperoxaluria (PH) is not only related to endogenous oxalate production and the response (if any) to pyridoxine (in type I), but is greatly influenced by extrarenal factors like dehydration. The earlier the diagnosis of PH, the better the chances of improving the prognosis in individual patients. Measures to enhance the solubility of calcium oxalate are important. Besides ensuring at all times a generous fluid intake (> 2 l/m2), administration of alkali citrate (0.15 g/kg), which has not been advocated so far in PH, appears very promising. We studied the effect of sodium citrate in six patients with PH. Mean urinary citrate excretion (mmol/day per 1.73 m2) without oral citrate was very low (0.57) and rose to 2.49 with citrate administration. This was accompanied by a significant decrease in the calcium oxalate saturation (calculated by equil 2) from 11.7 to 6.9 (P < 0.05). Treatment in five patients over 10-36 months resulted in improved (1) or stabilized (4) renal function and reduced passage of stones. Additional measures include restriction of salt and of oxalate-rich food. We conclude that long-term administration of alkali citrate is beneficial in patients with PH.

Administration, Oral↗
PubMed1993

Urinary saturation and nephrocalcinosis in preterm infants: effect of parenteral nutrition.

Urinary lithogenic and inhibitory factors were studied in 27 preterm infants; 16 had total parenteral nutrition (TPN) and 11 had breastmilk with an additional glucose-sodium chloride infusion. Urines were collected for 24 hours on day 2 (period A), day 3 (B), and once between days 4 and 10 (C). Urinary calcium oxalate saturation was calculated by the computer program EQUIL 2. Renal ultrasonography was performed every second week until discharge. The calcium/creatinine ratio increased in infants on TPN (A 0.91; C 1.68 mol/mol) and was significantly higher at period C than that in infants on breastmilk/infusion (A 0.52; C 0.36). The oxalate/creatinine ratio was persistently higher with TPN (203 mmol/mol) than with breastmilk/infusion (98; 137). The citrate/creatinine remained constant with TPN (0.44 mol/mol), whereas it increased significantly with breastmilk/infusion (0.26; 0.49). Calcium/citrate rose considerably with TPN, but decreased with breastmilk/infusion to a significantly lower level than with TPN. The urinary calcium oxalate saturation increased with TPN (2.4; 4.5) and decreased with breastmilk/infusion (2.1; 1.5) to a significantly lower value than with TPN. Nephrocalcinosis developed in two infants on TPN. Mean daily calcium intake was similar in both groups, whereas protein, sodium, and phosphorus intake were significantly higher on TPN. It is concluded that the increase in urinary calcium oxalate saturation observed with TPN is due to the combined effect of an increased urinary calcium excretion and higher urinary oxalate/creatinine and calcium/citrate ratios. The changes observed are likely to be caused by TPN itself, which differs in several respects from breastmilk feeding.

Breast Feeding↗
PubMed1993

Hypercalciuria and nephrocalcinosis, a feature of Wilson's disease.

Hypercalciuria and nephrocalcinosis are not uncommon in patients with Wilson's disease but have only once been reported as the presenting sign. We diagnosed Wilson's disease in a 17-year-old male patient 6 years after his first episode of gross hematuria and 2 years after detection of hypercalciuria and nephrocalcinosis. Therapy with penicillamine resulted only in a moderate reduction of urinary calcium excretion but oxalate excretion increased.

Adolescent↗
PubMed1992

[Vitamin D poisoning in infants: a preventable cause of hypercalciuria and nephrocalcinosis].

The established prophylaxis for vitamin D-deficient rickets today is 400 IU vitamin D3 given daily during the first year of life. With this regimen, vitamin D intoxication is a rare event. Nevertheless, we have recently seen 4 infants with vitamin D intoxication after a so called "stoss" prophylaxis, i.e. twice 300,000 units (7.5 mg) vitamin D3 orally within 4 weeks. One patient presented with failure to thrive due to marked hypercalcemia (3.9 mmol/l) and nephrocalcinosis, 2 patients showed medullary nephrocalcinosis on ultrasonography and one patient had gross hematuria and spontaneous passage of a calculus. Three patients had massive hypercalciuria (calcium/creatinine ratio 1.8-4.8 mol/mol, normal less than 1). The 25 (OH) vitamin D3 plasma levels, measured only in 2 patients, were strikingly increased (270 and 158 nmol/l, respectively, normal 25-80). Urinary calcium excretion slowly decreased to normal values on a low calcium diet and high fluid intake. Nephrocalcinosis, however, persisted in 2 patients and showed a slight progression ultrasonographically in one patient. The short time interval between vitamin D administration and onset of symptoms and the subsequent clinical course provide strong evidence that hypercalciuria and nephrocalcinosis were due to vitamin D "stoss" prophylaxis in all four cases. In conclusion, there is no indication for vitamin D "stoss" prophylaxis for vitamin D-deficient rickets in infants. Vitamin D intoxication still has to be considered as a possible cause of hypercalciuria.

Calcifediol↗
PubMed1991

Unknown syndrome: ischiadic hypoplasia, renal dysfunction, immunodeficiency, and a pattern of minor congenital anomalies.

We report a 6 year old male with a pattern of malformations and anomalies including intrauterine growth retardation, microcephaly, psychomotor retardation, a pattern of craniofacial anomalies (flat face, hypertelorism, epicanthic folds, strabismus, short nose, low set ears), hypospadias and cryptorchidism, bilateral partial cutaneous syndactyly between fingers 2 to 5 and toes 2 to 4, postaxial polydactyly of the fingers and toes, severe conductive hearing loss, hypoplasia of the ischiadic bones, complex renal dysfunction, hypogammaglobulinaemia with proneness to bacterial infections of the upper and lower respiratory tract, and recurrent pseudomembranous enterocolitis. The parents are cousins of Turkish origin.

Abnormalities, Multiple↗