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Biomedical subjects

U Querfeld

Publications and source records attributed to U Querfeld.

68 records · Page 4Linked to original sources

Excessive urinary oxalate excretion after combined renal and hepatic transplantation for correction of hyperoxaluria type 1.

A 4.5-year-old boy received a combined liver and kidney transplant for correction of hyperoxaluria type 1. Both organs were from the same donor and functioned primarily. Three months after transplantation, urine oxalate excretion reached a maximum of 10,500 mumol/24 h and remained above 2300 mumol/24 h for the next 2 months. Two months later, oxalate excretion decreased to about 565 mumol/24 h, indicating exhaustion of a large oxalate pool. Six months after transplantation plasma oxalate is near normal (4.9 mumol/l). With the exception of one episode of acute rejection of the renal transplant, both organs were tolerated well and continue to have a unimpaired function 9 months after transplantation. However, there is increased echogenity on renal ultrasound, indicating oxalate deposits in the grafted kidney. This case illustrates that successful combined transplantation of both liver and kidney can be performed in infants, resulting in cure of the metabolic defect. The prolonged or acute excretion of oxalate may lead to oxalate deposition in the grafted kidney without impaired graft function or early graft loss.

Child, Preschool↗

Effects of cytokines on the production of lipoprotein lipase in cultured human macrophages.

Macrophages are important cells in the pathogenesis of atherosclerosis because of their tendency to accumulate lipid and become transformed into foam cells. Cultured human monocyte-derived macrophages spontaneously secrete lipoprotein lipase (LPL), and LPL has been linked to increased lipid uptake by these cells. Because secretion of various macrophage products depends on activation by lymphokines, we studied the effects of immunoregulatory lymphokines on LPL secretion by cultured human macrophages. After culturing cells in RPMI 1640 medium with 20% fetal calf serum, recombinant human gamma-interferon (gamma-INF), interleukin-1 (IL-1), and interleukin-2 (IL-2) were added to the medium and LPL secretion was assessed by measuring LPL activity and/or LPL mass in the medium. Gamma-INF suppressed LPL production both when added to freshly plated cultures of human blood monocytes, as well as when added to monocyte/macrophages from mature cultures (day 6) that were producing large amounts of LPL. IL-1 inhibited medium LPL when added to freshly plated cultures, but not when added to mature cultures. On the other hand, IL-2 did not inhibit LPL in freshly plated cultures, but produced a dose-dependent suppression of LPL from mature cultures. None of the cytokines were cytotoxic to macrophages, and cells that were cultured in gamma-INF demonstrated partial recovery from LPL-suppressive doses of the cytokine. After exposure of cells to 50 U/ml of gamma-INF and 50 U/ml of IL-2 for 3 days, LPL mRNA levels, when expressed as LPL/gamma-actin ratios, were 42% and 53% of controls, respectively. Thus, activation of human macrophages in vitro by gamma-INF resulted in a suppression of LPL production.(ABSTRACT TRUNCATED AT 250 WORDS)

Cells, Cultured↗

A new percutaneous renal biopsy device for pediatric patients.

A new automated biopsy technique is described for performing percutaneous renal biopsies in pediatric patients. The biopsy device (Bard Biopty Instrument) employs a relatively small needle (18 gauge). We believe this procedure offers a safer and more effective means of obtaining adequate renal tissue for pathological assessment in pediatric patients.

Adolescent↗

Lipoprotein profiles at different stages of the nephrotic syndrome.

We investigated lipoprotein profiles in 24 children with normal renal function at different stages of the idiopathic nephrotic syndrome (NS). Four groups of patients were studied: (I) steriod-resistant NS with persistent proteinuria; (II) untreated steroid-sensitive NS during a relapse; (III) steroid-sensitive NS in remission induced by steroid-treatment; (IV) steroid-sensitive NS in long-term remission without therapy. Triglycerides (TG), cholesterol (CHOL), and phospholipids (PLP) were measured in plasma as well as in the lipoprotein fractions of very low (VLDL), intermediate (IDL), low (LDL) and high density (HDL). Apoproteins (Apo) AI, AII, B and C-apoproteins were measured in patients of groups I and IV. Results were compared to those obtained in 24 healthy control subjects. All patients with active NS (groups I-III) had significantly elevated CHOL levels. TG and CHOL in the VLDL, IDL, LDL, and CHOL in HDL2, but not HDL3 were inversely correlated with the serum albumin level. Patients with active NS had increased concentrations of TG and CHOL in lipoprotein fractions of lower density. Total and fractionated HDL-CHOL was not significantly different from control levels in any group. Patients in group I had significantly reduced Apo AI levels, whereas an increase of Apo AI and Apo AII in HDL3 and of most C-apoproteins in both HDL fractions was observed in patients of group IV. While changes in HDL apoprotein composition during long-term remission are of yet unknown clinical significance, our data indicate an increased risk of atherosclerosis only in those paediatric patients with persistent steroid-resistant NS.

Adolescent↗

Hyperlipidemia in pediatric patients undergoing peritoneal dialysis.

We evaluated serial measurements of serum lipid levels in 68 patients aged 12.6 +/- 4.7 years undergoing treatment with continuous ambulatory peritoneal dialysis/continuous cycling peritoneal dialysis (CAPD/CCPD). Fasting mean levels of triglycerides (TG) and cholesterol (C) were elevated above the 95th percentile of published normal values by 102% and 19%, respectively, at the start of dialysis. Except for a short-term decrease in TG levels at 6 and 9 months, no significant change in mean lipid levels was observed during a follow-up period of 2 years. At initiation of dialysis, elevated TG and C levels were present in 90% and 69% of the patients, respectively. The prevalence of hyperlipidemia (HL) varied between 63% and 88% (TG) and 61% and 93% (C), respectively, during the follow-up period. TG and C levels were not correlated with caloric intake (evaluated in 17 patients), serum albumin levels, treatment modality (CAPD or CCPD), a history of the nephrotic syndrome, or previous treatment with hemodialysis or transplantation. However, a significant inverse correlation was observed between age and serum lipids at the initiation of dialysis treatment and after 1 year (TG: r = -0.40; C: r = -0.44). Our data indicate a high prevalence of HL but no significant change of serum lipid levels during 2 years of treatment with CAPD/CCPD.

Adolescent↗

Magnetic resonance imaging of iron overload in children treated with peritoneal dialysis.

The ability of magnetic resonance imaging (MRI) to detect iron overload in children with end-stage renal disease (ESRD) was studied in 18 multiply transfused patients, aged 15.5 +/- 4.8 years, and 5 nontransfused children without evidence of renal disease. In the transfused patients, the serum ferritin (SF) level was compared to (a) a subjective rating of signal intensity of MRI images (scale of 0-10), (b) mean T1 values of liver and spleen, and (c) computer-assisted measurements of spin echo intensity (SEI) of liver, spleen, muscle and fat tissue. On subjective evaluation, the mean signal intensity was significantly lower in transfused patients than in controls and a significant correlation with the SF levels was observed for ratings of both liver and spleen. Mean T1 values of liver and spleen did not correlate with the SF levels. On computer analysis, the ratios of SEI of fat/liver, fat/spleen, muscle/liver and muscle/spleen were significantly correlated with the SF levels as well as the subjective evaluation sources. These data indicate that MRI is a suitable technique of documenting the presence and degree of iron overload in multiply transfused children with ESRD.

Adolescent↗

"Partial trisomy 22 and 11" due to a paternal 11;22 translocation associated with Hirschsprung disease.

The 11;22 translocation seems to be the most frequent, non-Robertsonian, translocation in man. Approximately 50 cases with an unbalanced karyotype 47,XX (or XY),+der(22), t(11q;22q), due to a 3:1 meiotic disjunction in the parental translocation carrier, have been reported in the literature. We present an additional patient with that chromosome aberration, whose father was shown to be the translocation carrier. He presented with many of the more or less typical signs of the syndrome, but had an extraordinary additional finding, namely Hirschsprung disease. Although anal stenosis is a rather frequent finding in the syndrome, Hirschsprung disease has never been described in the literature. Furthermore the most important genetic and cytogenetic data on that chromosome aberration are given, including implications for genetic counselling.

Chromosomes, Human, Pair 11↗

Generalized amyloidosis secondary to xanthogranulomatous pyelonephritis.

Unilateral nephrectomy in an 8-year-old male with chronic renal failure and urosepsis revealed a combined histologic lesion of xanthogranulomatous pyelonephritis and amyloidosis. Amyloid deposits were found also in the rectum and liver. On clinical grounds xanthogranulomatous pyelonephritis was also suspected in the remaining kidney. Common causes of systemic amyloidosis were excluded. After operation the signs of amyloidosis diminished, probably due to removal of the infected kidney, but renal function deteriorated rapidly.

Amyloidosis↗

The significance of focal global sclerosis in idiopathic nephrotic syndrome. Long-term clinical observations.

The clinical course of five pediatric patients with idiopathic nephrotic syndrome associated with focal global sclerosis in renal biopsy was analyzed retrospectively. The condition was defined as complete obsolescence of more than 15% of glomeruli associated with tubular atrophy and interstitial fibrosis. At onset, all children had a steroid-sensitive relapsing nephrotic syndrome. Two became steroid-dependent and one steroid-resistant. Cyclophosphamide or chlorambucil treatment was initially followed by remission in all patients; a second course was successful in one of two patients. After 12 to 18 years of observation, four patients are in remission for periods of 2 to 12 years. Glomerular filtration rate is slightly reduced in only one patient. This study suggests that the clinical course of children with idiopathic nephrotic syndrome associated with focal global sclerosis is similar to that observed in patients with minimal glomerular changes but different from that in patients with focal segmental sclerosis.

Antineoplastic Agents↗

[Iatrogenic hydrothorax in a premature infant and its treatment].

Iatrogenic hydrothorax in a premature infant of 36 weeks' gestation was caused by misplacement of a central venous catheter leading to massive pleural effusion in the left hemithorax. Subsequently respiratory insufficiency developed which could be treated successfully within 24 h by complete removal of the i.v. fluid via the misplaced catheter and simultaneous mechanical ventilation.

Catheterization↗

Renal tubular dysgenesis: a report of two cases.

Renal tubular dysgenesis, a congenital disorder of renal tubular development, was diagnosed in two newborns with oligohydramnios and Potter phenotype. Renal tubular dysgenesis (RTD) is a recently recognized congenital disorder of renal tubular development associated with oligohydramnios, Potter phenotype, and neonatal respiratory and renal failure. We report two newborn siblings with typical clinical and anatomic features of RTD. The diagnosis was proven by autopsy in one child. The pediatrician should consider the diagnosis of RTD in a child with congenital anuria and structurally normal kidneys on ultrasound, especially if a maternal history of late trimester oligohydramnios is present.

Anuria↗