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Cystinuria is reportedly a rare condition affecting the stone patients in India. This paper presents the occurrence of cystine-related abnormality in the population of stone patients reporting to the hospitals in South India. Two thousand and eight hundred urine samples from 1,300 patients attending the urinary stone clinic during the period 2004-2008 were assessed for cystinuria by performing the nitroprusside test on the early morning urine and random samples on the day of attendance. Urinary deposits were also studied in all the patients. Stones retrieved from 800 stone patients were analysed qualitatively and by Fourier Transform infra red (FTIR) spectroscopy. Cystinuria was identified in only three patients. None of these patients showed cystine crystals. Three other patients out of the 1,300 showed presence of cystine crystals in the urine deposit. FTIR spectroscopy of the stones retrieved from the patients showed presence of cystine in 19 out of the 800 stones analysed (2.375%). None of the patients with cystine in the stones had either cystine crystals in the urine or positive nitroprusside test for cystine. All the patients who had positive cystine, cystine crystals or cystine in stone analysis had other biochemical abnormalities. They were medically managed with appropriate biochemical corrective chemotherapy and had control of stone disease process. All the patients were advised purine restriction in the diet. It is concluded from the study that cystinuria is a rare entity in South India. It, however, exists in a small percentage of stone patients. Specific treatment with D-penicillamine was not administered to the patients in view of the high cost, nonavailability and possible toxicity. The patients considered above did not have intractable stone disease which was not amenable to usual modalities of directed medical therapy.

This paper attempts to assess the current status of the various modalities of available treatment for urinary stone disease in the Kerala scenario. A total of 300 patients who attended the stone clinic with urinary stone disease and had stones retrieved by different means were selected for the study. Their clinical symptoms, demographic profile, size, number and position of stones, metabolic profiles, retrieval modalities and end result of treatment in terms of stone clearance were assessed. Instances of failure, incomplete clearance and complication events were noted. Based on the experiences, a flowchart was created for appropriate decision-making in urinary stone management. The modalities of retrieval included nephrectomy, nephrolithotomy, pyelo-nephrolithotomy, extended pyelolithotomy, pyelolithotomy, ureterolithotomy, cystolithotomy, urethrolithotomy, ESWL, PCNL, URS, cystolithotripsy, urethrolithotripsy and spontaneous passage. The clearance rate of stone was maximum in open surgery. The extent of stone clearance by ESWL depended on various factors. PCNL was mostly limited by the difficulties in achieving puncture at the stone site. Availability of a variety of flexible nephroscopes also altered the success rate of the procedure. There were good success rates in pushing stones from the ureter to the pelvis followed by PCNL. In patients who had successful PCNL, postoperative morbidity was significantly reduced in terms of the number of days of hospitalization, time taken for return to work, absence of urinary leak, site infection, urinoma formation and urinary tract infection. URS was performed in many patients and stones retrieved. However, the indication for the procedure remains doubtful as the size of most of the stones thus retrieved was less than 6 mm. These would have passed out spontaneously or with chemotherapeutic support. URS, lithotripsy and basketting were confronted by upward migration of stones to the kidney, requiring further procedures for retrieval. Introduction of double J stents helped in relieving urinary obstruction, particularly in patients presenting with anuria, but retained stents, forgotten stents and failed stone retrieval were common following the procedure. The procedure of URS was simplified by the presence of dilated ureter in spontaneous stone passers or those with distal obstruction and proximal dilatation. It is concluded from the study that open surgery still remains the sheet anchor of treatment of urinary stones in many patients in Kerala. Newer lesser invasive procedures should be ethically selected. Decisions should be patient based, taking into consideration the economic feasibility for the procedure proposed.