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Markus J Kemper

Publications and source records attributed to Markus J Kemper.

30 records · Page 2Linked to original sources
PubMed2004

Recurrence of severe steroid dependency in cyclosporin A-treated childhood idiopathic nephrotic syndrome.

BACKGROUND: In patients with steroid-dependent nephrotic syndrome (SDNS), long-term remission (LTR) can usually be achieved with cyclosporin A (CSA), after alternative treatment with cytotoxic drugs or levamisole has failed. Nevertheless, severe SDNS recurs in some patients despite CSA maintenance therapy. Few data are available on the clinical course and treatment strategies in these patients. METHODS: We carried out a retrospective chart analysis of 46 patients with SDNS treated with CSA, after failure of cytotoxic treatment with cyclophosphamide (CPO). Median age at primary manifestation was 3.0 years (range 0.8-6.9) and median current age is 20.4 years (range 8.6-29.1). Patients were recruited from three centres caring for a total of 186 patients with steroid-sensitive nephrotic syndrome. RESULTS: In 14 of the 46 patients (30%; 10 male), severe SDNS recurred again despite CSA maintenance therapy. Seven patients relapsed beyond the age of 18 years. Nine of 14 patients received a further course of cytotoxic treatment as first intervention: six were treated with chlorambucil (CLA) and three with CPO. Four of the CLA-treated patients remained in LTR in contrast to none after CPO. Five patients received levamisole after CSA: only one went into LTR, while in one other CSA could be discontinued although further relapses occurred. One further patient was switched to CLA after levamisole, finally inducing LTR. Overall, six patients required two or more drugs, and in four of these CSA maintenance ultimately had to be restarted. CONCLUSION: We conclude that SDNS can recur in patients despite CSA maintenance therapy. Treatment strategies for this subgroup of patients are complex and should be standardized to optimize long-term outcome. A subgroup of patients with childhood SDNS continues to relapse into adulthood.

Adrenal Cortex Hormones↗
PubMed2004

Health-related quality of life and psychosocial adjustment in steroid-sensitive nephrotic syndrome.

OBJECTIVES: To evaluate quality of life (QOL) and psychosocial adjustment by standardized tests in well-characterized patients with steroid-sensitive nephrotic syndrome. STUDY DESIGN: Forty-five patients were investigated at a median age of 9.8 years (range, 3.4 to 19.8) and 5.9 years (range, 0.1 to 16.3) since diagnosis. Child- and parent-rated QOL was evaluated by the The Netherlands Organization for Applied Scientific Research Academical Medical Center (TNO-AZL) Child Quality of life Questionnaire. Psychosocial adjustment was assessed by the Child Behavior Checklist and the Teacher Report Form. RESULTS: In patients, only the QOL subscale "social functioning" was impaired compared with that in control subjects, whereas parents rated 4 of a total of 7 subscales as abnormal. Psychosocial adjustment was impaired at home and at school. Steroid dependency and cytotoxic treatment had a significant negative impact on QOL only, whereas family climate, especially maternal distress, negatively affected both QOL and psychosocial adjustment. CONCLUSIONS: QOL and psychosocial adjustment are impaired in steroid-sensitive nephrotic syndrome. Both illness-related variables and family climate play an important role.

Adolescent↗
PubMed2004

Latex allergy in children with urological malformation and chronic renal failure.

PURPOSE: Children with spina bifida, bladder exstrophy and anorectal anomalies are at risk for latex allergy. Severe intraoperative anaphylaxis in a boy treated with kidney transplantation prompted this study to evaluate the prevalence of latex allergy in a cohort of children with chronic renal failure (CRF). MATERIALS AND METHODS: Between 1996 and 2002, 57 boys and 28 girls were investigated at a median age of 10.5 years (range 1.3 to 22.9). Urological malformations were the underlying cause of CRF in 33 patients (39%). Of the patients 39 were on conservative treatment, 20 were on dialysis and 26 had a functioning renal graft. Latex reaction was assessed by a careful history, specific serum latex IgE and skin prick test. RESULTS: A total of 19 patients (22%) showed latex reaction, of whom 8 had allergy (clinical symptoms included severe intraoperative anaphylaxis in 1) and 11 had sensitivity (positive IgE or prick test without symptoms). Of these 19 patients 11 had urological malformations. The number of surgical procedures, young age at operation and atopy were significant risk factors. When operations were analyzed separately, ie urological vs nonurological surgery, only urological surgery was significantly associated with latex reaction. A significant correlation was also found between the overall number of operations and latex radioallergosorbent class. CONCLUSIONS: All children with CRF who undergo early and multiple urological surgery are at high risk for latex reaction. Primary latex prevention, ie the routine use of latex-free gloves, tubes and catheters, should be implemented in all children with complex urological malformations.

Adolescent↗
PubMed2003

Neuropsychologic side-effects of tacrolimus in pediatric renal transplantation.

Calcineurin inhibition with tacrolimus has been used after renal transplantation (RTPL) as rescue therapy for insufficient immunological control or if cyclosporin A (CSA) toxicity occurred. Neurologic side-effects occur but are rare in children, usually presenting as tremor; however, serious complications, e.g. the posterior leukoencephalopathy syndrome are also documented. Twenty children (10 girls) were switched to tacrolimus: 11 (55%) for immunological reasons (n = 9: steroid-resistant rejection; n = 2: recurrent rejections) and nine for CSA side-effects. Tacrolimus was started at a median of 8 wk (range 10 d to 8.7 yr) after RTPL and was continued for a median of 2.5 yr (range 5 wk to 4.6 yr). Renal function significantly improved over a period of 12 months following conversion to tacrolimus (glomerular filtration rate 56 +/- 19 vs. 66 +/- 16 mL/min/1.73 m2; p < 0.03; n = 13). Fifteen of 20 (75%) patients tolerated tacrolimus well. The most frequent side-effects were neuropsychological and behavioral symptoms in three children, ranging from anorexia nervosa-like symptoms with weight loss, amenorrhea, depression and school problems to severe insomnia and to aggressive and anxious behavior in one child. Only the latter child was exposed to toxic tacrolimus blood levels. All side-effects were fully reversible after discontinuation of tacrolimus. In conclusion, tacrolimus had a beneficial effect on renal function and was well tolerated in the majority of pediatric patients. However, neuropsychologic and behavioral side-effects are important and maybe underrecognized in children.

Abdominal Pain↗
PubMed2002

Serum levels of immunoglobulins and IgG subclasses in steroid sensitive nephrotic syndrome.

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.

Adolescent↗
PubMed2002

Asymptomatic intracardiac thrombus in steroid-sensitive nephrotic syndrome.

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.

Child, Preschool↗
PubMed2002

Saliva IgM and IgA are a sensitive indicator of the humoral immune response to Escherichia coli O157 lipopolysaccharide in children with enteropathic hemolytic uremic syndrome.

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.

Adolescent↗
PubMedDate not supplied

Percutaneous endoscopic gastrostomy in children on peritoneal dialysis.

OBJECTIVE: Insertion of percutaneous endoscopic gastrostomies (PEG) in patients on chronic peritoneal dialysis (PD) has been reported to be contraindicated due to an increased risk of morbidity and mortality. However, no systematic survey on this topic has yet been published. DESIGN: Retrospective multicenter study. SETTING: 23 pediatric dialysis units associated with the working group Arbeitsgemeinschaft für Pädiatrische Nephrologie (APN). DATA SOURCE: A structured questionnaire on clinical details of PD patients who had undergone PEG insertion or open gastrostomy (OG) since 1994 was distributed to all pediatric dialysis units of the APN. RESULTS: 27 PD patients (20 males) from 12 centers in whom PEG insertion was performed after Tenckhoff catheter introduction were evaluated. Age at intervention ranged from 0.25 to 10.9 years (median 1.3 years). Most patients were malnourished, with standard deviation score (SDS) for body weight between -4.2 and -0.6 (median -2.2). Major complications were early peritonitis < 7 days after PEG in 10/27 (37%) patients, episodes of fungal peritonitis in 7/27 (26%) patients, 4 cessations of PD and change to hemodialysis, and 2 associated deaths. However, in 14 patients, no such problems were encountered and, in 4 patients, early peritonitis effectively treated with intraperitoneal antibiotics was the only major complication. Thus, in 18/27 (67%) patients, PD was successfully reinitiated shortly after PEG insertion. Among all participating centers, only two OG procedures were reported during the study period, illustrating a clear preference for the PEG over the OG procedure among members of the APN. CONCLUSION: PEG insertion following PD initiation carries a high risk for fungal peritonitis and potential PD failure; however, complication rates in this largest reported series were lower than previously described. Antibiotic and antifungal prophylaxis, withholding PD for 2 - 3 days, and gastrostomy placement by an experienced endoscopy team are suggested precautions for lowering the risk of associated complications. When gastrostomy placement does not occur prior to or at the time of initiating PD, the risks and benefits of percutaneous versus open placement must be carefully weighed.

Child↗