oxalates and Arrhythmias--Cardiac

This 48-year-old male developed renal insufficiency, peripheral sensitivity to cold, occasional ventricular extrasystoles and atrio-ventricular block. He died suddenly after ventricular fibrillation. Autopsy showed extensive deposits of oxalate crystals in the kidney, myocardium, arterial walls of the extremities, and in many other visceral organs. The atrio-ventricular (A-V) node was also severely involved and marked fibrosis was found in the common A-V bundle (His) and in both bundle branches. The A-V node artery showed mural crystals and the lumen was narrowed by 50 per cent. Features supporting the diagnosis of primary oxalosis with cardiac manifestations include the onset of urolithiasis at 10 years of age, and 5 other siblings all having died of "renal disease" in infancy.

Topics: Arrhythmias, Cardiac; Cardiac Complexes, Premature; Heart Block; Heart Conduction System; Humans; Kidney; Kidney Calculi; Male; Middle Aged; Myocardium; Oxalates

Topics: Arrhythmias, Cardiac; Barbiturates; Butyrylcholinesterase; Drug Synergism; Drug Tolerance; Dysautonomia, Familial; Genetic Diseases, Inborn; Humans; Hyperbilirubinemia, Hereditary; Jaundice; Kidney Calculi; Malignant Hyperthermia; Metabolism, Inborn Errors; Narcotics; Nephrocalcinosis; Osteogenesis Imperfecta; Oxalates; Paralyses, Familial Periodic; Pharmacogenetics; Porphyrias; Succinylcholine

The present status of regular dialysis and renal transplantation in patients with end-stage renal disease secondary to primary hyperoxaluria is reported. Clinical studies include one personal case with an 18-month period of follow-up and data concerning thirteen patients treated in 10 centres in Europe which have been collected through a cooperative survey carried out with the assistance of Registry of the EDTA. On January 1 st, 1974, mean survival of patients with oxalosis treated by RDT was 30.4 months (range 6 to 102 months). Five cadaveric renal transplants have been performed in four patients; two patients are surviving with grafts functioning for 18 and 45 months. Dialysis and/or transplantation should be performed in patients with oxalosis early enough to prevent ischaemic, cardiac and neuromusclar complications which occur at the end-stage of the disease. Evidence for blood coagulation disorders, particularly chronic consumption coagulopathy, should be investigated for with adequate laboratory methods and long-term heparin therapy instituted if necessary. No convincing reports concerning the efficiency of the various drugs which have been tried out to reduce the biosynthesis of oxalic acid in patients with oxalosis have been issued to this date.

Topics: Acute Kidney Injury; Adolescent; Adult; Arrhythmias, Cardiac; Child; Child, Preschool; Disseminated Intravascular Coagulation; Extremities; Female; Humans; Ischemia; Kidney Calculi; Kidney Transplantation; Male; Metabolic Diseases; Middle Aged; Neuromuscular Diseases; Oxalates; Renal Dialysis

Topics: Adolescent; Adult; Arrhythmias, Cardiac; Autopsy; Coronary Vessels; Female; Heart Diseases; Humans; Kidney; Male; Metabolic Diseases; Middle Aged; Myocarditis; Necrosis; Oxalates; Raynaud Disease