Autosomal dominant polycystic kidney disease: observations from a university hospital in saudi arabia.

Thirty cases of autosomal dominant polycystic kidney disease (ADPKD) seen at King Fahd Hospital of the University, Al-Khobar over a period of eight years, were analysed with respect to clinical features, laboratory investigations, radiological findings, complications and outcome. There were 13 males and 17 females with a mean age of 45 yrs + 10.1 (range 16-65 years). There was positive family history of renal disease in 17 cases. At the time of presentation, 27 cases had abdominal pain. The other features noted were hematuria (20 cases), polyuria (10 cases), urinary tract infection (22 cases), headache (9 cases), uremia (7 cases) and nephrolithiasis (5 cases). Bilaterally palpable kidneys were present in all cases. Hypertension (17 cases) was the next common clinical finding. Other clinical features noted were hepatomegaly (5 cases) and mitral valve prolapse (5 cases). Twenty-one patients had cysts in liver and five had cysts in spleen. Varying degrees of renal failure were seen in 15 cases. Six (20%) patients progressed to end stage renal disease during the period of observation.

Outcome of patients with pre-existing hepatitis B virus infection following renal transplant.

Twenty four patients who were HBsAg positive prior to renal transplantation were studied with respect to their hepatic and renal outcome. The kidneys in all patients were obtained from living related donors. The standard immunosuppressive therapy consisted of azathioprine and prednisolone. Post-renal transplant follow-up varied from 32-86 months (mean 55 +/- 9). Renal functions, liver functions, HBsAg and HBsAb status was closely monitored during follow-up. One patient developed acute hepatitis four months after transplantation; this resolved in 10 weeks. Four patients showed transient elevations of liver enzymes. The results of liver biopsy in 16 cases revealed, normal histology (8), virus in hepatocytes (5), and acute hepatitis (3). None of the patients had evidence of chronic liver disease. Two patients died due to chronic rejection/chronic renal failure and two patients died due to septicaemia. Five living patients have mild to moderate chronic rejection. It was concluded that pre-existing HBsAg carrier state in transplant patients had no deteriorating effect on the liver. The mortality was related to chronic rejection and septicaemia.

The effect of serum from patients of chronic renal failure on colony forming units (CFU-S) in mice.

Repopulating ability of mouse bone marrow stem cells, treated with pre-dialysis, post-dialysis and control sera was assessed by colony forming units (CFU-S). Significant lower colony counts were observed in pre-dialysis group as compared to control group. There was an improvement in the colony counts when the cells were treated with post-dialysis sera. The study suggests the presence of inhibitor/s of CFU-S in the uraemic sera which is/are partially removed by haemodialysis.

Emergency treatment with ketoconazole in disseminated intravascular coagulation due to metastatic prostatic carcinoma.

We report the use of ketoconazole to control disseminated intravascular coagulation due to prostatic carcinoma. Clinical improvement in the condition of the patient was noted in 48 hours and coagulation profile became normal in 10 days.

Incidence of reflux nephropathy (RN) in ESRD and effect of nephrectomy in RN on blood pressure and haematocrit following renal transplantation.

One hundred and fifty cases of end stage renal disease (ESRD) on pretransplant workup showed vesicoureteric reflux (VUR) in 21 (14%). Of these, 15 were primary reflux nephropathy (PRN) whereas 6 were secondary VUR. All patients in PRN group showed grade III to IV reflux while secondary VUR ranged between grade I to II. In the PRN group 38.5% had severe, 23% moderate and 23% mild hypertension; 53.3% were nonoliguric and 46.7% were oliguric. Mean plasma renin activity (PRA) was 6.6 and 2.85 ng/ml/hr in PRN and secondary VUR groups respectively. All cases of PRN and 2 from secondary VUR underwent nephrectomy and a month later renal transplantation. Following surgery blood pressure normalised without therapy in 50% and another 50% required lesser drugs in comparison to age matched non nephrectomised controls who showed hypertension in 100% cases, 60% requiring 3 to 4 drugs combination (p less than 0.05). Risk of low haematocrit in nephrectomised patients was insignificant.

Renal amyloidosis and non-specific aorto-arteritis--a hitherto undescribed association.

Two patients presented with the nephrotic syndrome complicating non-specific aorto-arteritis (Takayasu's arteritis). Histologically both had renal amyloidosis. On investigation there was no evidence of chronic infection or any immuno-inflammatory disease known to be associated with amyloidosis. These cases raise the possibility of a significant association between aorto-arteritis of the Takayasu type and systemic amyloidosis.

Spectrum of rapidly progressive (crescentic) glomerulonephritis in northern India.

36 consecutive patients whose biopsies showed significant extracapillary proliferation in the face of rapidly declining renal function were reviewed between 1967 and 1979. About 30% of the patients belonged to the pediatric age group with a male:female ratio of 2.5:1. Oliguria/anuria, hematuria, and progressive renal insufficiency were present in all cases. There was evidence of 9 cases being poststreptococcal, 2 SLE, 1 Henoch-Schönlein purpura, 2 possibly viral and 1 staphylococcal. Histologically, 29 cases had more than 60% crescents, 5 between 50 and 60% and 2 cases a little less than 50%. 28 cases were fatal in less than 10 months. 2 were lost of follow-up. 6 survivors with reversal of renal functions had 3 common factors to note, namely an antecedent disease, less crescents both in number and size, accompanied by fewer interstitial changes and early treatment. Rebiopsy in 2 survivors showed regression of histologic severity.