Adiponectin (ADPN) counteracts the inflammatory response of the endothelium, which plays an important role in the development of atherosclerosis in patients with chronic kidney disease (CKD). Data in children with CKD are scarce. We examined serum ADPN concentration in 90 children with various renal disorders: 28 with CKD on conservative treatment (CKD), 21 on regular dialysis treatment (D), and 41 after kidney transplantation (Tx); 27 age-matched healthy children served as controls (C). Body mass index (BMI), estimated glomerular filtration rate (eGFR), lipids, homocysteine, high sensitivity CRP (hsCRP), and systolic blood pressure (SBP) were also measured. Mean serum ADPN concentration was significantly higher in patients with CKD (27.3 μg/ml ±15.0), on D (34.2 μg/ml ±14.9), and after Tx (23.6 μg/ml ±9.5) compared with ADPN levels in C (13.5 μg/ml ±6.1) (p < 0.0001). Serum ADPN concentration was inversely related to BMI (p = 0.001) and SBP (p = 0.004). In the multiple linear regression analysis, only SBP remained independently associated with ADPN plasma levels. Data show that children with CKD have significantly higher serum ADPN, even after Tx. The protective antiarthrosclerotic effect of ADPN may be mediated by lower SBP, a finding that deserves further study.

BACKGROUND:Cure of the metabolic defect in primary hyperoxaluria type 1 (PH1) is possible with liver transplantation (LTx). Preemptive LTx (PLTx) was promoted to prevent chronic kidney disease due to nephrocalcinosis and urolithiasis. However, timing of this procedure is difficult in view of the heterogeneity of PH1 and effective conservative treatment. Combined liver-kidney transplantation (LKTx) is able to cure metabolic defect and replace renal function at the same time and is effective and indicated for patients with or approaching end-stage renal disease (ESRD). Sometimes a sequential approach for LKTx (first liver, then kidney) has been recommended. METHODS:We report on 13 patients with PH1 since 1995 who underwent transplantation procedures in our center for PH1 at a median age of 4.7 (range 1.4-8.9) years. RESULTS:The first two patients, planned for a sequential strategy, died early after LTx because of infectious complications. Four patients underwent PLTx at a median glomerular filtration rate of 65 (range 27-98) mL/min/1.73 m/day (Hoppe et al., Pediatr Nephrol 1996; 10: 488), and three patients still have sufficient residual renal function after a follow-up of median 11.6 years. Seven patients with ESRD received a combined LKTx, including four with infantile oxalosis, and three weighing less than 10 kg. There was no mortality and catch-up growth was observed in most patients. CONCLUSION:In summary and conclusion, transplantation procedures are challenging in PH1, but our results including growth data are encouraging. PLTx remains an option despite the difficulties in timing the procedure. LKTx is indicated for patients with ESRD and is possible even in patients with infantile oxalosis and may improve longitudinal growth.

The anti-CD20 antibody rituximab has been used successfully as a rescue therapy in some patients with therapy-refractory steroid-dependent nephrotic syndrome (SDNS), including both primary SDNS with minimal changes on biopsy and recurrent focal segmental glomerulosclerosis (FSGS) after renal transplantation. All patients showed remission from steroid dependency for at least 9 months concurring with the reappearance of B lymphocytes that had been eliminated by rituximab. The doses used so far vary between 375 mg/m(2) per dose at weekly intervals for 6 weeks and a single dose of 375 mg/m(2). Until now, with the limited information available, no substantial adverse effects have been reported. However, a recommendation to use rituximab instead of any other established treatment, such as cyclophosphamide, in SDNS cannot be given before clinical studies have been conducted, especially as publication bias cannot be excluded.

The insulin-like growth factors (IGF) -I and -II promote cellular growth and differentiation of various organs. Their growth-stimulating effects are modulated by a family of six IGF-binding proteins (IGFBPs). Altered patterns of intact and fragmented IGFBPs have been reported in serum and urine of children with chronic renal failure (CRF), and it has been suggested that this may contribute to the growth failure observed in these patients. In the present study, a rapid and comprehensive method is presented to analyze IGFBPs and IGFBP fragments in the plasma of a child with acute renal failure (ARF) who had undergone plasmapheresis. The plasma IGF-I and IGFBP-3 levels were drastically reduced. Plasmapheresis filtrate (3 l) was fractionated by cation-exchange chromatography and reversed-phase high-performance liquid chromatography. The fractions obtained were tested by ligand and immunoblotting. In addition to IGFBP-1 and -4 fragments, the majority of IGF-binding polypeptides were IGFBP-3 immunoreactive. N-terminal sequence analysis of a 17-kDa polypeptide revealed the isolation of a C-terminal fragment of IGFBP-3 starting with Lys 160. The IGF-II-binding polypeptide pattern in the ARF plasma resembles the pattern in hemofiltrate from CRF patients, suggesting that similar or identical proteases are involved in IGFBP-3 fragmentation and common mechanisms may lead to the accumulation of the fragments in both diseases.

Saliva antibodies to Escherichia coli O157 were investigated as markers of the immune response in children with enteropathic hemolytic uremic syndrome (HUS). Paired serum and saliva samples were collected from 22 children with HUS during acute disease and convalescence and were tested for E. coli O157 lipopolysaccharide (LPS)-specific IgM and IgA antibodies by ELISA. Serum and saliva samples from 44 age-matched controls were used to establish the cut-off values. Elevated levels of IgM and/or IgA antibodies to O157 LPS were detected in saliva of 13/13 HUS patients with Shiga toxin-producing E. coli (STEC) O157 in stool culture and from 4 of 5 HUS patients in whom STEC were not detected. These results closely mirrored the results obtained with paired serum samples. In contrast, saliva and serum samples from four children with STEC isolates belonging to O-groups O26, O145 (n = 2), and O165 lacked detectable O157 LPS-specific antibodies. The specificity of the ELISA was confirmed by western blotting. In STEC O157 culture-confirmed cases, the sensitivity of the ELISA was 92% for saliva IgM and IgA, based on the first available sample, and 100% and 92%, respectively, when subsequent samples were included. The specificity was 98% for IgM and 100% for IgA. Children with E. coli O157 HUS demonstrate a brisk, easily detectable immune response as reflected by the presence of specific antibodies in their saliva. Saliva-based immunoassays offer a reliable, noninvasive method for the diagnosis of E. coli O157 infection in patients with enteropathic HUS.

Alterations of serum immunoglobulins, especially hypogammaglobulinemia (HG), are a frequent finding in steroid sensitive nephrotic syndrome (SSNS). The exact mechanisms are unclear, especially the persistence of HG into remission. Therefore we studied serum immunoglobulins M, A and G including IgG subclasses 1–4 in 44 children with SSNS; 14 were studied during relapse (RL) and 30 in remission (RM). Data were compared with those of 23 healthy controls. In a subgroup of 23 patients (12 in RM and 11 in RL) we also studied IgG-1 specific antibodies to tetanus toxoid and IgG-2 specific antibodies to pneumococcus antigen. Increased serum concentrations of IgM in RL and reduction of serum IgG in RL and RM were confirmed. During relapse, HG was characterized to result from deficiency of IgG-1–3, whereas in early phases of relapse the reduction was due to low IgG-1 only. In RM the deficiency of IgG-2 persisted for 12 months and correlated strongly with the duration of remission (R=0.60, P<0.0001). IgG-4 levels were not altered in SSNS. In addition, IgG-2 specific antibodies to pneumococcus antigen were significantly reduced only in RL compared to RM (P<0.05). In conclusion, hypogammaglobulinemia of SSNS is characterized by a different constitution of IgG subclasses. In RL a reduction of serum levels of IgG-1–3 occurs, while low concentrations of IgG-2 might be the explanation for HG in remission of SSNS.

Arterial and venous thrombotic events can lead to severe complications in the nephrotic syndrome, but may remain clinically silent in a substantial proportion of patients. Intracardiac thrombi associated with multiorgan thrombosis have been described in autopsy cases of the earlier literature, but have never been documented in vivo. We here report an asymptomatic intracardiac thrombus in a child with frequently relapsing steroid-sensitive nephrotic syndrome and a ventricular septal defect.

A 1.5-year-old girl was referred to our centre because of microscopic haematuria. This was discovered by chance at the age of 12 months by a general paediatrician during an upper respiratory tract infection. Physical examination at presentation revealed no abnormalities. Her body weight was at the 50th and her length at the 97th percentile. Her blood pressure was 85/60 mmHg. The family history was negative regarding renal disease and deafness, and testing of the patient’s urine revealed no abnormality. The serum creatinine was 0.4 mg/dl and repeated testing for serum C3, C4, AST, ANA, ds-DNA, pand c-ANCA antibodies was normal. The urine contained 200 erythrocytes/ml (69% dysmorphic), red blood cell or white cell casts could not be demonstrated. There was no proteinuria initially. The urinary calcium/ creatinine ratio (mg/mg) was 0.04. Renal ultrasound revealed no abnormality. A hearing test was normal and an ophthalmologic investigation showed no lenticonus anterior or cataracts. During follow-up the patient developed macroscopic haematuria during infections and mild glomerular proteinuria (221 mg/m2/day). Renal biopsy was recommended but refused by the parents. At the age of 5 years she developed difficulties swallowing food but not liquids (one episode of dysphagia about every 3 weeks) for which she was investigated at another hospital. Achalasia of the oesophagus was diagnosed Fig. 1. Barium swallow of esophagus showing an achalasia like on barium swallow and a significant obstruction of the picture but also demonstrating indents from leiomyoma (arrows). distal oesophagus was excluded by endoscopy.

Development of steroid dependency represents a significant therapeutic challenge in steroid-sensitive nephrotic syndrome. Previous studies have shown conflicting results concerning the benefit of a 12-week treatment with cyclophosphamide (CPO), with 24%–67% of patients achieving long-term remission. We therefore analyzed the clinical response of 20 consecutive children with steroid-dependent nephrotic syndrome (SDNS) (12 male, median age at start of treatment 5.9 years, range 3.2–14.7 years) treated at our institution with CPO (2 mg/kg per day) for 12 weeks since 1989. Median duration of follow-up was 5.8 (range 1.1–9.25) years. Only 6 of 20 children (30%) showed a long-term remission of >2 years, while 14 of 20 (70%) developed relapses again. Of these, 12 patients (86%) again developed steroid dependency, requiring further alternative treatment. Our data show that a 12-week course of CPO leads to unfavorable results in the majority of patients with SDNS. We therefore conclude that there is a need for further optimization of therapy in SDNS.

Background: We studied biochemical parameters of iron metabolism in INS, to elucidate the consequences of urinary transferrin and iron loss in relapse of INS.

Seminars in DialysisVolume 7, Issue 6 p. 413-420 Use of Recombinant Human Erythropoietin in Children Undergoing Dialysis Dirk E. Müller-Wiefel, Corresponding Author Dirk E. Müller-Wiefel Department of Pediatrics, Division of Pediatric Nephrology, University Children's Hospital, Hamburg, GermanyHead of Pediatric Nephrology, University Children's Hospital Hamburg, Martinistrasse 52 D-20246 Hamburg GermanySearch for more papers by this authorOliver Amon, Oliver Amon Department of Pediatrics, Division of Pediatric Nephrology, University Children's Hospital, Hamburg, GermanySearch for more papers by this author Dirk E. Müller-Wiefel, Corresponding Author Dirk E. Müller-Wiefel Department of Pediatrics, Division of Pediatric Nephrology, University Children's Hospital, Hamburg, GermanyHead of Pediatric Nephrology, University Children's Hospital Hamburg, Martinistrasse 52 D-20246 Hamburg GermanySearch for more papers by this authorOliver Amon, Oliver Amon Department of Pediatrics, Division of Pediatric Nephrology, University Children's Hospital, Hamburg, GermanySearch for more papers by this author First published: November 1994 https://doi.org/10.1111/j.1525-139X.1994.tb00805.xCitations: 3AboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat Citing Literature Volume7, Issue6November 1994Pages 413-420 RelatedInformation

Abstract. The long‐term results of four different regimens of prednisone therapy were compared in 32 children with steroid sensitive, frequently relapsing idiopathic nephrotic syndrome with minimal glomerular lesions on renal biopsy. Prednisone was adminstered according to the following dosage schedules: 1) long‐term daily, 2) standard intermittent, 3) standard alternate‐day, and 4) short‐term daily. Over a mean observation period of 7 years patients without steroid dependency received a cumulative dosage of prednisone of 10 mg/m2/day and those with steroid dependency received 19 mg/m2/day. Relapse free intervals were the longest with long‐term daily prednisone therapy compared to the other three regimens. In frequently relapsing patients without steroid dependency the relapse free intervals were similar with either intermittent or alternate‐day prednisone therapy (median 75 d); however, they were significantly shorter with short‐term prednisone therapy (median 33 d). In frequently relapsing patients with steroid dependency the time of remission was generally shorter than in patients without steroid dependency (median 25d vs. 69d) with no benefit of any of the different forms of short‐term treatment.