Sanjiv Saxena

Treatment of Acute Rejection in Live Related Renal Allograft Recipients: A Comparison of Three Different Protocols

We present our experience on the comparison of three different modes of steroid therapy, oral prednisolone (OP), intravenous dexamethasone (IVDX) and intravenous methylprednisolone (IVMP) in the treatment of acute rejection (AR) in renal allograft recipients. Between January 1980 and January 1992, 206 patients underwent live related renal transplantation. Before 1990, all received prednisolone (PRED) and azathioprine (AZA) only. After 1990, patients were given PRED, AZA and cyclosporine (CsA). After 1 year, CsA was stopped and patients were converted to a two-drug regimen only. Of the 206 patients, 180 (87.4%) were male and mean age was 30.3+/-8.7 years (range 14-63). During the mean follow-up of 43.5 months, 178 episodes of AR were seen in 121 patients. Each episode was considered as a separate entrant in the study. Conventional immunosuppression was given in 151 episodes and 27 episodes were on triple-drug therapy. Diagnosis of AR was made by clinical, sonography, nuclear scan with or without graft biopsy evidence. Of the 178 AR, 110 (61.8%) were within 3 months, 36 (20.2%) were between 3 months and 1 year and 32 (18%) were after 1 year. OP was given in 11 cases while IVDX and IVMP were given in 48 and 119 cases respectively. Overall, 154 (86%) showed either a complete or partial response to antirejection therapy. Response to therapy was 91, 90 and 85% in OP, IVDX and IVMP groups respectively. There was no statistical difference in response rate in different groups. There was also no difference in side effects in three different groups. Our data suggest that it is the high dose of steroid rather than mode of therapy which is responsible for therapeutic benefit in treatment of AR.

VISCERAL LEISHMANIASIS : A RARE CAUSE OF UNEXPLAINED PYREXIA IN A RENAL ALLOGRAFT RECIPIENT

Dr. Sanjiv Saxena, Assist. Prof., Department of Nephrology, AIIMS, New Delhi (India) Dear Sir, Visceral leishmaniasis or kala-azar has not been recognised as a common infection in renal allograft recipients. However, immunodeficiency states can facilitate the reactivation of a dormant illness in infected patients or predispose such patients to infection in endemic areas. Indeed, following its first description in renal transplant recipients in 1979 [1], in recent years more case reports have described such an association [2-5]. In contrast to the nonimmunodefi-cient patients, the disease is often more fulminant and poorly responsive to therapy in these high risk patients [3]. We report here a case of visceral leishmaniasis in a renal allograft recipient who presented with pyrexia of undetermined origin and showed an excellent response to pentavalent antimonials. A 23-year-old young male, resident of Bihar, India, underwent a one-haplotype-matched live related renal transplantation for Alport’s syndrome with end stage renal disease at our centre in December 1992. Immunosuppression given comprised three drugs – azathioprine, prednisolone and cy-closporine, with a gradual tapering off from cyclosporine from 3 months onwards so as to be completely weaned off by 9 months. The early posttransplant period was uneventful. In June 1993 the patient was admitted for evaluation of his pyrexia of unknown origin of 3 weeks duration. The fever was high grade and remittent in nature without any localising symptoms. He had received an empirical course of antimalarials twice (chloroquine followed by a sulphamethoxa-zole and pyrimethamine combination) and also broad-spectrum antibiotics (ampicillin and norfloxacin) for his pyrexia in Bihar without response. Physical examination revealed a pale, anicteric, febrile patient with no significant peripheral lymphadenopathy. He was normotensive and the cardiovascular and respiratroy systems were normal. Abdominal examination showed a 2-cm hepatomegaly and a firm 3-cm splenomegaly with the allograft being normal and non-tender. Investigations on admission revealed a haemoglobin of 6.4 g/dl, reticulocyte count 1%, total leucocyte count 3,200/mm3, platelet count of 90,000/mm3 and ESR of 33 mm in the 1st h. Blood urea was 30 mg/dl, serum creatinine 1 mg/dl, serum bilirubin 0.9 mg/ dl, SGOT 86

SITUS INVERSUS : A RARE EXTRARENAL ASSOCIATION OF ALPORT'S SYNDROME

Dr. Sanjiv Saxena, Asstt. Prof., Department of Nephrology, AIIMS, 110029 New Delhi (India) Dear Sir, Alport’s syndrome is a form of hereditary nephritis characterised by progressive hema-turic nephritis and sensorineural hearing loss occurring in successive generations in a family [1]. A spectrum of ocular, hematological and other extra-renal abnormalities have been reported in patients with Alport’s syndrome [2–4]. Only 2 cases of Alport’s syndrome with situs inversus have been reported in the literature so far [4,5]. We now report the third case of Alport’s syndrome with situs inversus. A 26-year-old male presented to the Nephrology services of All India Institute of Medical Sciences, New Delhi in April 1992 with a history of pedal edema and facial puffiness off and on for 3 years prior to admission. About 4 months prior to admission, he was found by his local doctors to have moderate hypertension and moderately severe azotemia and was put on conservative management for uremia. The patient gave a history of decreased hearing since the age of 10 years and of decreased visual acuity in both eyes for the last 8 years. He denied any history of gross hematuria in the past. The patient was the eldest of 6 sibs and his 2 younger brothers aged 14 and 16 years also complained of decreased hearing and visual acuity for the last 7-8 years but had not been investigated till date for the same. However, they denied any history of hematuria or anasarca. No other family members had any history of renal disease. On examination, the patient was found to be very pale and had features of chronic renal failure. There was evidence of situs inversus on clinical examination in the form of dextrocardia and abdominal viscerae on the opposite side. Ocular examination revealed the presence of bilateral anterior and posterior lenticonus with the oil-droplet sign being positive on distant direct ophthalmological examination. The audiometry revealed evidence of bilateral moderate sensorineural deafness. Laboratory investigations confirmed the presence of severe anemia Hb being 0.65 mmol/l (4.5 mg/dl) with a normal total and differential leucocyte count. The platelets were normal in number and structure. There was evidence of severe azotemia on admission with a serum creatinine of 1056 mmol/l (12 mg/dl). Urine examination revealed 3+ proteinuria and 15-20 RBCs per high power field. Proteinuria was 3.6 g/24 h and the creatinine clearance was 3 ml/min. Skeletal survey

Bilateral Renal Stones Associated with Nail-Patella Syndrome

Dr. Sanjiv Saxena, Assistant Professor, Department of Nephrology, AIIMS, New Delhi-110029 (India) Dear Sir, The nail-patella syndrome (hereditary osteo-onychodysplasia or HOOD) is a well-recognized autosomal dominant disorder characterized by nail, skeletal and renal abnormalities. A spectrum of renal involvement has been described in patients with the nail-patella syndrome [1,2]. Although Glassock et al. [3] mention that a disproportionate number of these patients may have renal stones (references not quoted), on review of the literature, we came across only three case reports describing such an association [2, 4, 5]. However, all the above three cases had evidence of unilateral calculus disease. A 23-year-old male was referred to the Nephrology Services of All India Institute of Medical Sciences, New Delhi in June 1993 for the management of chronic renal failure. He had a history of recurrent bilateral renal colics for the preceeding 3 years and had undergone pyelolithotomy on the right side and ureteroli-thotomy on the left side for bilateral calculus disease 2 years earlier in a district hospital. The status of his renal functions at the time of surgery was not known. The patient had no subsequent colics and repeated skiagrams revealed no radiopaque calculi. However, for the preceding 4 months, he started having anasarca and was found to have mild hypertension and moderately severe azotemia. There was no history of renal calculus disease or any other renal illness in the family. Clinical examination revealed severe pallor, mild hypertension and changes of chronic azotemia. In addition, the patient had dystro-phic nails in both upper and lower limbs, fixed flexion deformity of the right elbow, bilaterally small hypoplastic patellae and bilaterally palpable iliac horns. There were surgical scars of pyelolithotomy and ureteroli-thotomy on the right side and left side of the abdomen, respectively. Neither of the kidneys was palpable. Laboratory evaluation confirmed the presence of severe anemia with Hb 0.70 mmol/l (4.7g/dl) and severe azotemia with serum cre-atinine of 1,408 μmol/l (16.0 mg/dl). Urine examination revealed 2+proteinuria and 8-10 WBCs per high power field on microscopy. 24-hour urine examination showed 1.98g proteinuria and a creatinine clearance of 4 ml/ min. Skeletal survey revealed bilateral iliac horns, bilaterally hypoplastic patellae and an ill-developed capitellum of right radius. Ultra-sonography of the abdomen revealed bilateral normal-sized kidneys with a