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Biomedical subjects

G Offner

Publications and source records attributed to G Offner.

At least 37 records · Page 2Linked to original sources
PubMed2000

Obstructive airway disease caused by Moraxella catarrhalis after renal transplantation.

We report a case of severe acute obstructive airway disease 2 months after renal transplantation in a 16-year-old patient with Biedl-Bardet syndrome who was transplanted for end-stage renal failure secondary to cystic kidney disease. Symptoms of severe obstructive airway disease developed 2 months after transplantation under immunosuppression with prednisone, azathioprine, and tacrolimus. The patient did not develop signs of infection; progressive shortness of breath remained the only symptom for several weeks. After extensive diagnostic evaluation, bronchoalveolar lavage revealed Moraxella catarrhalis as the single infectious agent. After 3 weeks of appropriate antibiotic therapy, symptoms of obstructive airway disease were completely relieved. This atypical presentation of Moraxella infection in an immunocompromised host represents a rare complication of renal transplantation, especially in young patients. Special aspects such as frequency, diagnosis, differential diagnosis, and management of this rare complication of renal transplantation in a pediatric patient are discussed.

Adolescent↗
PubMed2000

A stepwise approach to the treatment of early onset nephrotic syndrome.

We have retrospectively reviewed our single-center experience of the treatment of early onset nephrotic syndrome (NS). From 1991 to 1998, ten children with NS were treated. Kidney biopsy showed focal sclerosis (n=1), diffuse mesangial sclerosis (n=7), and congenital NS of the Finnish type (n=2). Associated conditions included incomplete Drash syndrome (n=1), Galloway-Mowat syndrome (n=1), and severe mental and motor retardation of unknown origin (n=3). From 1991 to 1997, five children with NS were treated. Bilateral nephrectomy (NX) was performed in three, one patient with severe retardation died at 4 years and NX was not performed in one patient who showed satisfactory growth and development. Three of these children were dialyzed and two were successfully transplanted. One patient was transplanted without previous dialysis. From 1997 to 1998, five children were treated with a regimen that included captopril and indomethacin (CAPTO/INDO). CAPTO/ INDO was successful in increasing serum protein in all patients and producing growth and development in four patients. In two patients CAPTO/INDO was successful only after unilateral NX. Our experience indicates that CAPTO/INDO may be a valuable treatment in patients with early onset NS. An individualized stepwise approach including unilateral NX should be considered to achieve optimal results.

Age of Onset↗
PubMed2000

Distal myopathy in nephropathic cystinosis.

In long-standing nephropathic cystinosis complications are observed in various organs. Distal myopathy was first described in detail in 1994. The prevalence was calculated to be 24%. We studied seven patients with nephropathic cystinosis with neurophysiological techniques. Only two patients complained of a distal muscle weakness but all showed signs of myopathy on electromyography, which was more pronounced in the distal muscles. Motor and sensory nerve conduction parameters were within normal ranges. One patient with the juvenile form of nephropathic cystinosis also had myopathy. We conclude that distal myopathy can be detected in nephropathic cystinosis even in the absence of clinically overt muscle weakness. Cystine-depleting therapy with cysteamine is recommended for all patients with cystinosis, even after renal transplantation, and the effect on the myopathy should be studied.

Adolescent↗
PubMed1999

Kidney transplanted children come of age.

BACKGROUND: The aim of renal replacement therapy in children is to restore their potential for normal growth and development in order to reach mature adulthood. Because pediatric kidney transplantation started in the late 1960s, it is now possible to document the progress and outcome of these patients from transplantation in childhood to survival into adulthood. METHODS: In this single-center study, all 150 children born before December 1977 and having received a kidney transplant between 1970 and 1993 were selected for long-term follow-up. The mean age at transplantation was 12.1 years (range 3.2 to 16.7), and the mean follow-up was 13.1 years (range 2.0 to 25.0). In December 1995, 124 grown-up patients with a mean age of 25.4 years (range 18.4 to 40.3) were alive, 89 with a functioning graft. Fifty had the first graft functioning longer than 10 years. The fate of all patients was traced, and those living were analyzed in regard to their somatic and socioeconomic states. RESULTS: The actuarial 25-year survival rate for the patients was 81%, and for the first graft it was 31%. The best graft survival rates were observed after living related donation, preemptive transplantation, and immunosuppression with cyclosporine. The latter benefit, however, vanished after eight years. The mean creatinine clearance declined over the years from 76 to 45 ml/min/1.73 m2, and the incidence of hypertension increased to more than 80% of the patients. Malignancies occurred in 2.6%. Final height was stunted in 44% of noncystinotic patients, whereas all patients with cystinosis were extremely growth retarded. Twenty-seven percent suffered from additional disabilities. A majority of adult patients were rehabilitated in regard to education and socioeconomic status, and 14% were unemployed. CONCLUSIONS: The results indicate that renal transplantation in children leads to a high degree of rehabilitation in adulthood. The life of a kidney transplant, however, is limited, which points out the need for more specific immunosuppression with fewer side-effects in order to reach the goal of lifelong graft function.

Adolescent↗
PubMed1998

Does tacrolimus cause more severe anemia than cyclosporine A in children after renal transplantation?

Initial reports indicated the possibility of severe anemia associated with tacrolimus (TC) therapy. We investigated the degree of anemia under TC treatment in comparison to cyclosporine A (CsA) treatment in children after renal transplantation. A cross-sectional analysis of 95 children successfully transplanted for at least 3 months was performed. Eighty-five children received CsA and 10 TC. TC-treated patients were compared with CsA-treated patients who were matched according to age, gender, creatinine clearance, and time after transplantation. No patient received additional therapy with mycophenolate mofetil or azathioprine. The creatinine clearance of the whole group of transplanted children was 58 ml/min per 1.73 m2. The patients within the matched-pair analysis had a lower creatinine clearance (TC 46 and CsA 48 ml/min per 1.73 m2). The hemoglobin was 10.3 g/dl for the TC-treated children and 10.4 g/dl among the CsA-treated patients. Numerically, EPO was higher and iron lower in the TC group than in the CsA group. Among children with functioning renal grafts, a correlation exists between Hb and creatinine clearance. A significant difference in the degree of anemia between TC- and CsA-treated children could not be found.

Adolescent↗
PubMed1998

End-stage renal failure in children younger than 6 years: renal transplantation is the therapy of choice.

UNLABELLED: Between 1975 and 1994, 46 children under 6 years of age received a total of 52 renal transplants. Obstructive uropathy and dysplasia accounted for most causes of terminal renal failure (17 and 12 cases respectively). Four patients required a second, 1 patient a third transplantation. Cadaveric organs were used on 33 occasions; 19 patients received a living-related donor kidney. Immunosuppression was performed with azathioprine in 5, with cyclosporine A in 21 and combined azathioprine/cyclosporine therapy in 20 cases. After 1 year, graft survival was 81%, and after 5 years 78%. Creatinine clearance declined slightly between 1 and 5 years from 69 to 56 ml/min per 1.73 m2. Main causes of graft failure were thrombotic complications in 6 cases and death with functioning graft in 5 cases. Graft thrombosis occurred only in grafts from young donors under the age of 7 years and after vascular anastomosis to the iliac vessels. Only two transplants were lost in rejection episodes. Patient survival was 94% after 1 and 90% after 5 years. Two patients died due to septiacemia, 1 died of a ruptured aortic aneurysm, 1 of cerebral ischaemia and 1 suddenly of unknown cause. Patient and graft survival was not different compared with 204 patients aged 6-16 years who received a renal transplantation during the same time period at our institution. After transplantation the patients receiving cyclosporine A showed a marked catch-up growth in the 1st year. The median standard deviation score (SDS) of body length improved from -2.63 to -1.39 standard deviations. CONCLUSION: Renal transplantation is the treatment of choice in end-stage renal failure in children under 6 years.

Actuarial Analysis↗
PubMed1998

Effect of a silver device in preventing catheter-related infections in peritoneal dialysis patients: silver ring prophylaxis at the catheter exit study.

Catheter-related infections remain a significant cause of method failure in chronic peritoneal dialysis (PD) therapy. Given the increasing antibiotic resistance, such nonpharmacological strategies as local silver devices attract more interest. To establish whether a silver ring device (designed by Grosse-Siestrup in 1992) mounted onto the PD catheter and placed at the exit site at skin level is effective in preventing exit-site and other catheter-related infections, a prospective 12-month, multicenter, controlled study stratified by diabetes status was conducted. The study subjects were assessed by an extensive structured inventory, including a broad spectrum of control variables, such as age, body mass index (BMI), Staphylococcus aureus carrier status, catheter features, mode and quality of PD therapy, comorbidity, and psychosocial rehabilitation. Ten experienced German outpatient dialysis centers (seven adult, three pediatric) participated in the trial. All eligible patients (n=195) from the study area without catheter-related infections during the ascertainment period were included (incidental subjects undergoing PD therapy for at least 3 months). The main outcome measures were the occurrence of first exit-site infections (primary study end point), sinus tract/tunnel infection, and peritonitis. Ninety-seven patients were assigned to the silver ring and 98 patients to the control group. Baseline characteristics of age, sex, proportion of pediatric and incidental patients, S aureus carrier status, and other variables were similar in both groups. The incidence of infections in the silver ring group versus the control group was as follows: 23 of 97 versus 16 of 98 patients had exit-site infections, 12 of 97 versus 12 of 98 patients had sinus tract/tunnel infections, 16 of 97 versus 18 of 98 patients had peritonitis, respectively. Kaplan-Meier analysis for the probability of an infection-free interval showed no statistical difference (log-rank test) between the two groups. Displacement of the silver ring contributed to study termination in 6% of the study group patients, including two patients with catheter loss. Univariate analysis and multiple logistic regression identified younger age (<50 years), low serum albumin level (<35 g/L), number of previously placed PD catheters, short cuff-exit distance (<2 cm), and S aureus nasal carriage as risk factors for the development of exit-site infections. In conclusion, our study does not show any benefit of the silver ring in preventing catheter-related infections in PD patients. Thus, prevention of infection-related method failure in PD still has to rely on conventional antibiotic treatment strategies and less so on alternative methods.

Adult↗
PubMed1998

Treatment with recombinant human growth hormone in short children with nephropathic cystinosis: no evidence for increased deterioration rate of renal function. The European Study Group on Growth Hormone Treatment in Short Children with Nephropathic Cystinosis.

To evaluate the effect of long-term treatment with recombinant human GH (rhGH) on renal function in short children with nephropathic cystinosis with and without concomitant cysteamine treatment, 36 growth-retarded children with nephropathic cystinosis (age 7.3+/-2.7 y; creatinine clearance [C(CR)] 50+/-27 mL (min x 1.73 m2)(-1) were treated with 1 IU rhGH/kg/wk for up to 5 y. The rise in serum creatinine before and during rhGH treatment was compared with that in a historical control group of cystinotic patients. The effect of concomitant cysteamine treatment on the evolution of renal function before and after the start of rhGH was evaluated separately in patients without (group A) and with cysteamine treatment (group B). The decline of C(CR) was also compared with that in noncystinotic patients with chronic renal failure with and without rhGH treatment. At study entry, serum creatinine values in group A were similar to those in the historical controls, whereas group B had significantly lower serum creatinine values. Treatment with rhGH did not accelerate the rise in creatinine independently of cysteamine treatment. There were no significant differences in the mean decline of C(CR) per year in cystinotic compared with noncystinotic patients with chronic renal failure with or without rhGH treatment. rhGH therapy for up to 5 y does not accelerate the deterioration of renal function. This justifies the continuation of controlled studies of rhGH treatment in these patients. The study also provides further evidence that cysteamine therapy reduces the progression of renal failure in children with cystinosis.

Child↗
PubMed1998

Recurrence of renal disease after kidney transplantation in children: 24 years of experience in a single center.

The aim of this study was to assess the frequency and clinical implications of a recurrence of the original renal disease in children after kidney transplantation. Thus, the records of patients with immunological and metabolic diseases transplanted between 1970 and 1994 were retrospectively analyzed. There were 113 renal transplantations in 99 patients, who had the following original diseases: focal segmental glomerulosclerosis (FSGS), membrano-proliferative glomerulonephritis type I and type II (MPGN I, II), Henoch-Schoenlein nephritis, IgA-nephropathy, hemolytic uremic syndrome (HUS) and hyperoxaluria type I (PH I) and other rare diseases. Recurrences were observed in FSGS, MPGN II, HUS and PH I but not in the other diseases. In FSGS, the recurrence rate was 20% with graft failure in 5 of 6 grafts. No specific risk factors for recurrent FSGS could be determined. In MPGN II, the recurrence was 60% but the loss of grafts occurred at the same rate as in the non-recurrence group. In HUS, recurrence was seen in 4 out of 24 renal grafts (16.6%) with subsequent graft loss in all cases. All cases had suffered from an atypical HUS. PH I recurred in 4 of 5 allografts with graft loss in all patients. The remaining graft was transplanted after a liver transplantation and graft function was well preserved for 4 years. We confirm that the risk of recurrence with loss of the graft is high in a certain group of renal diseases. In these the indication for transplantation, particularly with living related donor kidneys, needs special evaluation. A better understanding of the pathomechanism of the diseases should lead to prevention of recurrence, as in PH I in which a liver transplant is now the primary option.

Adolescent↗
PubMed1997

Response to diphtheria and tetanus booster vaccination in pediatric renal transplant recipients.

BACKGROUND: Although inactivated vaccines are recommended for immunocompromized patients, efficacy and safety of diphtheria and tetanus immunization in renal transplant recipients have received little attention so far. The aim of the study was to investigate the response to a standard diphtheria and tetanus booster vaccination in pediatric renal transplant recipients. METHODS: Forty-two children, median age 13.2 years (range, 7.8-18.9 years) with complete primary immunization 9.2 years (0.9-15.4 years) before transplantation were enrolled. Immunosuppression consisted of cyclosporine plus prednisolone in 15 (36%), cyclosporine, azathioprine, and prednisolone in 24 (57%), and tacrolimus plus prednisolone in 3 (7%). Antibodies were measured by enzyme-linked immunosorbent assay before and 1, 6, and 12 months after vaccination. RESULTS: Before vaccination, protective antibody concentrations exceeding 0.1 IU/ml against diphtheria were found in 16 children (38%). Thirty-eight (90%) had protective antibody concentrations against tetanus. After booster immunization, the protection rate against diphtheria rose to 95% at 1 month with a decline to 93% at 6 and 76% at 12 months. Protection against tetanus was complete after vaccination and persisted over the observation. Antibody concentrations were comparable to those reported for healthy children. Statistical analysis showed no influence of allograft function, immunosuppressive regimen, previous cytotoxic therapy, or time between primary immunization and end-stage renal failure on antibody response. Immunization was well tolerated and kidney function remained unaffected in patients with stable allograft function. CONCLUSIONS: Diphtheria and tetanus vaccination can be performed effectively and safely in renal transplant recipients as generally recommended.

Adolescent↗
PubMed1997

[Severe stenosis of the right coronary artery in a 15-year-old girl with type IIa hypercholesterolemia: successful treatment with stent implantation].

Coronary artery stenosis with need for therapy is rarely seen in childhood. A 15-year-old girl with hypercholesterinaemia type II a was undergoing lipid aphereses therapy (once or twice a week) since she was 6 years old. The girl was seen in our hospital with stenocardia and depression of the ST-segment in the inferior ECG leads at rest. Myocardial scintigraphy with technetium 99 showed an ischemia of the infero-lateral left ventricular myocardium. During selective coronary angiography a 90% stenosis of the proximal right coronary artery over a distance of approximately 5 mm close to the ostium was found. Post stenotic dilatation of the vessel was obvious. In addition a diffuse 10% stenosis in the proximal and middle part of the right coronary artery was found. The left coronary artery appeared angiographically normal. After balloon dilatation, stent implantation was performed without complications with a 6 mm microstent. Reduction of the stenosis from 90% to 40% could be achieved. Ticlopidin 2 x 250 mg was started for thrombocyte aggregation inhibition. During the following 12 months the patient has been free of symptoms. Lipidaphareses has been continued as before. Stent implantation seems to be a successful treatment for coronary artery stenosis also in young patients.

Adolescent↗
PubMed1997

Nitric oxide and gallbladder motility in prairie dogs.

In this study we evaluated the role of nitric oxide (NO) on gallbladder motility in the normal prairie dog by 1) immunohistochemistry, 2) an enzymatic assay for NO synthase (NOS), and 3) an in vivo model to measure whole gallbladder tone and contractility. NOS was localized to gallbladder mucosal cells by NADPH-diaphorase and polyclonal antibodies to a constitutive brain NOS. Gallbladder mucosal homogenates demonstrated total NOS activity in the range of 578 +/- 115 pmol x mg protein(-1) x 30 min(-1). Blockade of NOS activity in vivo using N(omega)-nitro-L-arginine methyl ester resulted in an up to 80% increase in gallbladder tone from basal. A 40% increase in tone was seen with methylene blue, suggesting that tone was maintained by both NO activation of guanylate cyclase and possibly direct effects on Ca2+ channels. An exogenous nitrosothiol, S-nitroso-N-acetyl-cysteine, abolished cholecystokinin (CCK) octapeptide and bethanechol-stimulated gallbladder contraction. We conclude that the prairie dog gallbladder contains constitutive NOS and synthesizes NO, which is important for the maintenance of basal gallbladder tone and is an inhibitor of the contractile response of the gallbladder to agonists such as CCK and bethanechol.

Animals↗
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↗
PubMed1997

Assessment of long-term risks for living related kidney donors by 24-h blood pressure monitoring and testing for microalbuminuria.

Our aim was to assess the long-term risks for kidney donors of developing arterial hypertension and hypertension-associated diseases or hyperfiltration injury of the remaining solitary kidney. We conducted a cross-sectional study in which 29 donors who were nephrectomized at our center between October 1973 and March 1990 were enrolled. At the time of evaluation median age was 54 (37-70) yr. Since kidney donation an average time interval of 11.1 +/- 3.8 yr had elapsed. Body weight, casual blood pressure, S-creatinine and proteinuria were recorded. In 28/29 donors 24-h blood pressure monitoring was performed; all 29 were tested for microalbuminuria (MAU). The patient history was checked for treatment with antihypertensives, coronary heart disease and diabetes mellitus. From all 29 kidney donors surveyed up to 19.8 yr none developed marked renal insufficiency: median S-creatinine was 1.0 mg/dl (range 0.7-1.6), only 3 donors had a S-creatinine > 1.3 mg/dl. Glomerular filtration rate (GFR) decreased in an age-dependent manner. While all donors had been normotensive without an antihypertensive treatment at time of nephrectomy, actually 29% proved to be hypertensive with average values > 130/80 mmHg in the 24-h assessment. 5/29 donors received antihypertensives, 3 of whom nevertheless were hypertensive. Day-night profile was lost in 2 patients. After donation one patient developed coronary heart disease. 7/29 donors (24%) displayed positive testing for MAU, furthermore one had proteinuria (approx. 300 mg/l). MAU was associated in one case with slightly elevated S-creatinine (1.3 mg/dl) and in 3 cases with arterial hypertension. In the long-term course living related kidney donors do not seem to be at risk for developing hypertensive disorders more often than the general population. The prevalence of MAU in the 29 cases studied was higher than so far described for healthy subjects. This may reflect subclinical hyperfiltration damage of the glomerulus. Progressive renal insufficiency with clinical relevant function loss of the remaining solitary organ, however, was not observed up to 19.8 yr after kidney donation.

Adult↗
PubMed1996

Retroperitoneal placement of living related adult renal grafts in children less than 5 years of age--a feasible technique?

The extraperitoneal approach was used for transplantation of adult renal grafts in eight children under 5 years, three of them weighing less than 10 kg. Usually, children of this size are approached transperitoneally. The modified procedure included partial mobilization of the right liver to provide enough space for retroperitoneal graft placement and positioning of the vascular anastomoses, invariably to the aorta and caval vein. With this regimen, no significant space problems were encountered in recipients as small as 9 kg, and particularly, neither arterial nor venous complications occurred. Currently, at a mean follow-up of 2.4 years, all patients are alive with well-functioning grafts. Thus, the extraperitoneal approach represents a feasible and successful procedure in the pediatric living related renal transplantation setting for recipients under 10 kg of weight.

Adult↗
PubMed1996

Oxalate elimination via hemodialysis or peritoneal dialysis in children with chronic renal failure.

Oxalate elimination and oxalate dialysance via hemodialysis (HD) or peritoneal dialysis (CAPD) has not been studied in detail in pediatric patients. We studied plasma oxalate, oxalate elimination, and oxalate dialysance in 15 infants and children undergoing CAPD (9 female, 6 male, aged 9 months to 18 years) and in 10 children on HD (4 female, 6 male, aged 7-18 years). Two children in each group had primary hyperoxaluria (PH). The mean duration of dialysis prior to examination was 12 +/- 11 months in CAPD and 31 +/- 23 months in HD patients. Bicarbonate HD was performed 5 h three times a week, CAPD consisted of five daily exchanges in 5 patients and four changes in the remaining 10 children (dwell volume 40 ml/kg body weight, 2.3 g/l glucose). Although oxalate dialysance was significantly higher in HD (mean 115.6 ml/ min per 1.73 m2 in HD versus 7.14 ml/min in CAPD), mean oxalate elimination per week was not different between both renal replacement therapies (3,478 mumol/1.73 m2 surface area/week in CAPD versus 3,915 mumol/1.73 m2 per week in HD). Oxalate elimination in patients with PH was between 6,650 and 9,900 mumol/week. Plasma oxalate remained elevated in both procedures [28-84 mumol/l in CAPD (92/148 in PH) and 33-101 mumol/l in HD (70/93 in PH)]. Oxalate elimination can be increased by a more frequent hemodialysis regimen.

Adolescent↗