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Cystinuria: the South Indian experience
1Department of Surgery, Zensa Hospital, Trivandrum, 695009, India. fazilmarickar@hotmail.com
Cystinuria is a rare urinary stone condition in South India, affecting a small percentage of patients. This study found cystine in stones of 2.375% of patients, often without typical cystinuria indicators.
Area of Science:
- Nephrology
- Urology
- Biochemistry
Background:
- Cystinuria, a rare metabolic disorder, is characterized by excessive cystine excretion in urine, leading to stone formation.
- Its prevalence in Indian stone patients is not well-documented, necessitating localized epidemiological studies.
Purpose of the Study:
- To investigate the occurrence of cystine-related abnormalities in urinary stone patients in South India.
- To correlate urinary findings, stone composition, and clinical presentation in affected individuals.
Main Methods:
- Analysis of 2,800 urine samples from 1,300 patients using the nitroprusside test and urinary deposit examination.
- Qualitative and Fourier Transform Infrared (FTIR) spectroscopy analysis of 800 retrieved urinary stones.
- Assessment for co-existing biochemical abnormalities.
Main Results:
- Cystinuria (positive nitroprusside test) was identified in only 3 patients; none had cystine crystals.
- Cystine crystals were observed in 3 other patients.
- FTIR spectroscopy revealed cystine in 19 stones (2.375%), with no correlation to positive urine tests or crystals.
- Patients with cystine in stones often presented with other biochemical abnormalities and were managed medically.
Conclusions:
- Cystinuria is a rare but present condition among South Indian stone patients.
- Cystine stones can occur without overt cystinuria or urinary cystine crystals.
- Medical management focusing on biochemical correction and dietary purine restriction is effective for these patients.
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