A family with a complete deficiency of lactate dehydrogenase M-subunit was investigated. The propositus was an 18-year-old male who complained of exertional pigmenturia and easy fatigue. Marked discrepancy was observed in the ratio between creatine kinase and lactate dehydrogenase (CK/LDH). Electrophoretic analysis of serum LDH isoenzymes of the propositus demonstrated only one activity band of LDH H4. A complete lack of the LDH M-subunit was similarly demonstrated in erythrocytes, leukocytes and in the intermediate vastus muscle. LDH levels in the muscle specimen were markedly decreased in the patient, whereas CK and aspartate aminotransferase were almost the same as in a control subject. LDH isoenzymes of erythrocytes were analyzed in 5 siblings and in the parents. This demonstrated a complete lack of LDH M-subunit in 3 siblings. The ratio between H-subunit and M-subunit (H/M) in erythrocyte LDH suggested a partial absence of the M-subunit in two siblings and in the parents. An abortive increase of blood lactate and a marked increase in blood pyruvate were observed immediately after ischemic work of the forearm, accompanied by an increase in serum creatine kinase and myoglobinuria. The present case represents a newly described form of genetically determined myopathy.

Adolescent↗

PubMed1980

Mechanisms of altered renal perfusion in the early stage of obstructive jaundice.

Mechanisms responsible for renal hemodynamic alterations were studied in jaundiced (by bile-duct ligation) rabbits (BDL) 10 days after ligation of the biliary tract, in comparison with sham-operated rabbits (SO). Arterial hematocrit, plasma volume, blood pressure, abdominal inferior vena cava pressure, and heart rate were not significantly different between the BDL and SO groups. Cardiac output in BDL rabbits decreased to approximately 73% of the value for SO rabbits. Renal blood flow and GFR were reduced to 64 and 61%, respectively. Reductions in blood pressure and renal blood flow, caused by bleeding (8 ml/kg of body wt), were more marked in the BDL group than they were in the SO group. In the BDL group, the recovery of blood pressure following blood infusion was slower and the mortality was higher, There was no significant increase in the renovascular sensitivity to exogenous noradrenaline or angiotensin II in the BDL group. The findings indicate that the early stage of obstructive jaundice in rabbits was characterized by altered renal perfusion partly due to reduced cardiac output and by incrased liability to hemorrhagic hypotension.

Animals↗

PubMed1980

Protein permeability and immune complex deposition in glomerular capillary walls of rats.

The effect of reduced glomerular anionic charge on immune complex deposition in the glomerular basement membrane was studied in rats with Heymann nephritis. A reduction of the anionic charge was induced by lysozyme administration. The animals were divided into the following four groups: normal controls (group A), group B treated with lysozyme, group C treated with lysozyme and immunized with autologous tubular antigen, and group D immunized with the antigen. Proteinuria was marked in groups B, C and D as compared with group A, especially in group D. Development of Heymann nephritis by the tubular antigen was suppressed in group C. Stainings of anionic substances in the glomerular capillary walls by cationic probes were weakened in groups B, C and D. Immune complex deposited in the subepithelial area of the capillary wall was also stained by cationic probes. The findings suggest that reduced glomerular anionic charge by polycations results in suppressed immune complex deposition in the basement membrane, partially due to increased permeation of macromolecules across the glomerular capillary walls in rats.

Animals↗

PubMed1980

Renal hemodynamics in oliguric and nonoliguric acute renal failure of rabbits.

The role of renal hemodynamic alterations in the curtailment of renal functions was studied in uranyl acetate-induced oliguric (ORF) and nonoliguric (NORF) renal failures of rabbits. 5 days after drug injection, renal functional and morphological changes were most remarkable. A depression of Cin and elevation of serum creatinine concentration were more marked in ORF than in NORF. Renal blood flow was high as compared to controls. Cortical blood flow redistributed to the inner cortex. There was no significant difference in renal blood flow or cortical flow distribution between renal failure models. The findings suggest the minor roles of renal blood flow and cortical flow distribution in maintaining renal failure in these nephrotoxic models. Prominent tubular necrosis was found in ORF but not in NORF. Arterial inulin concentration during retrograde ureteral infusion of 14C-inulin solution was significantly high in ORF as compared to controls and NORF. However, this inulin leakage was too small to explain severely curtailed inulin clearance.

Acute Kidney Injury↗

PubMed1980