Heinz-Günter Sieberth

Cerebral tuberculoma 11 years after renal transplantation

A case of cerebral tuberculoma in a 39-year-old patient who had received a renal graft from a living related donor 11 years previously is reported. The patient had a major seizure, progressive psychiatric signs and fever 5 days prior to admission. The clinical history suggested a neurological cause and rapid diagnosis of a cerebral tuberculoma was made by a computed tomography-guided stereotactic puncture of a space-occupying cerebral lesion. The aspirated pus contained Mycobacterium tuberculosis. Anti-tuberculous therapy with isoniacid, rifampicin, ethambutol and pyracinamide was administered. Transplant function deteriorated and the patient died due to intractable septicemia with multiorgan failure from pulmonary infection dissemination and additional urinary tract infection with atypical mycobacteria. The chance for a benign clinical course necessitates vigorous procedures for an early diagnosis of cerebral infections in renal transplant recipients with neurological/psychiatric signs.

Long-Term Extracorporeal Bilirubin Elimination: A Case Report on Cascade Resin Plasmaperfusion

Acute hepatic failure develops as a disease entity of rather diverse origin. With disease progression, toxic bilirubin levels may cause severe complications which include AV-nodal blockage, cardiac arrhythmia, impaired consciousness, generalized seizures, and status epilepticus. Treatment choices to prevent clinical deterioration comprise of costly and limited available orthotopic liver transplantation, utilization of extracorporeal bioartificial liver support devices and haemoperfusion/plasmaperfusion treatment with activated charcoal/anion exchange filters. Here, we present a patient with acute drug-induced cholestatic hepatitis. Excessively elevated bilirubin levels were accompanied by cardiac and cerebral complications. Extracorporeal resin perfusion treatment (Plasorba, BR–350) was successfully performed over a 50-day period without activation of the coagulation system or side effects. Bilirubin levels were lowered to a minimum of 225 μmol/l, with concurrent clinical improvement. In conclusion, extracorporeal anion exchange plasmaperfusion may be a viable long-term treatment for hyperbilirubinaemic side effects in overt cholestatic hepatitis.

Renal Oncocytoma of the Native Kidney after Renal Transplantation

Patients with acquired cystic kidney disease (ACKD) are at an increased risk of renal neoplasms. Frequent tumors are adenomas and renal cell carcinomas. However, renal oncocytomas may occur in patients with ACKD. Little is known about oncocytomas of the native kidney following renal transplantation. By means of B scan ultrasonography, a solid and echo-inhomogeneous renal mass was incidentically observed in the right native kidney of a 28-year-old female patient with ACKD 4 years following renal transplantation. A nephrectomy was performed. The histological examination revealed a renal oncocytoma. The increased prevalence of neoplasms in the case of ACKD and following renal transplantation requires careful monitoring of the patients concerned. In very rare cases a renal oncocytoma may develop in the native kidney after renal transplantation.

Hereditäre Osteoonychodysplasie mit renaler Osteopathie

Die hereditare Osteo-Onychodysplasie (HOOD) ist eine autosomal dominant vererbliche Fehlbildung von Geweben mesodermalen und ektodermalen Ursprungs, die in einer Haufigkeit von 22 Fallen auf 1 Mio. Einwohner und mit einer Penetranz von 100% auftritt (Valdueza 1973). Seit der Erstbeschreibung 1820 durch de Chatelain sind uber 200 Falle dieses Syndroms unter 22 Synonyma (Nail-patella syndrome, Fong’s syndrome, Turner-Kieser-Syndrom, Onychomesodysplasie u.a.) publiziert worden (Vogel und Wiegers 1980). Als charakteristisches Erkennungsmerkmal gilt die Tetralogie von Patellahypoplasie, Ellenbogendysplasie, symmetrischen Beckenhornern und Nageldystrophie. Daruber hinaus zeigen etwa 30-60% der betroffenen Patienten eine renale Beteiligung mit unterschiedlich stark ausgepragter Proteinurie und, fakultativ, eine Einschrankung der glomerularen Filtrationsrate, die mitunter bis zur dialysepflichtigen Niereninsuffizienz fortschreitet. Obwohl das verantwortliche Gen auf dem 9. Chromosom in enger Beziehung zu den Loci der ABO-Blutgruppen und der erythrozytaren Adenylatkinase lokalisiert werden konnte, ist ein zugrunde liegender metabolischer oder struktureller Defekt bisher nicht definiert worden (Ferguson-Smith et al. 1976).