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Etiology of nephrocalcinosis in northern Indian children
Mukta Mantan1, Arvind Bagga, Virenderjeet Singh Virdi
1Department of Pediatrics, Maulana Azad Medical College and Associated Hospitals, New Delhi, India.
Insights
Nephrocalcinosis (NC) in children is often caused by distal renal tubular acidosis (RTA). Early diagnosis and treatment are crucial, though NC extent may not change, and kidney function can decline over time.
Area of Science:
- Pediatric Nephrology
- Renal Medicine
- Clinical Etiology
Background:
- Nephrocalcinosis (NC) is a significant renal condition in children.
- Understanding the diverse etiologies of NC is critical for effective management.
- Previous studies highlight various contributing factors, necessitating comprehensive evaluation.
Purpose of the Study:
- To investigate the etiological spectrum of nephrocalcinosis in a pediatric cohort.
- To identify the most common causes of NC in the studied population.
- To assess the long-term renal function outcomes in patients with NC.
Main Methods:
- Retrospective analysis of 40 pediatric patients diagnosed with NC over an 8-year period.
- Clinical data collection including age at onset, presenting symptoms, and biochemical parameters.
- Etiological investigation using available diagnostic facilities, including assessment for RTA, hypercalciuria, and hyperoxaluria.
Main Results:
- Distal renal tubular acidosis (RTA) was the most frequent cause of NC, identified in 50% of patients.
- Idiopathic hypercalciuria and hyperoxaluria each accounted for 7.5% of cases.
- A significant decline in glomerular filtration rate (GFR) was observed during follow-up (P = 0.001).
Conclusions:
- Protocol-based evaluation can determine the etiology of NC in most pediatric cases, even with limited resources.
- While specific therapies may improve biochemical markers, they do not reverse established NC.
- Long-term monitoring is essential due to the potential for progressive decline in renal function.
Abstract:
This retrospective survey examines the etiology of nephrocalcinosis (NC) in 40 patients (26 boys), over an 8-year period. The median age at onset of symptoms and presentation was 36 months and 72 months, respectively. Clinical features included marked failure to thrive (82.5%), polyuria (60%) and bony deformities (52.5%). The etiology of NC included distal renal tubular acidosis (RTA) in 50% patients and idiopathic hypercalciuria and hyperoxaluria in 7.5% each. Other causes were Bartter syndrome, primary hypomagnesemia with hypercalciuria, severe hypothyroidism and vitamin D excess. No cause for NC was found in 12.5% patients. Specific therapy, where possible, ameliorated the biochemical aberrations, although the extent of NC remained unchanged. At a median (range) follow up of 35 (14-240) months, glomerular filtration rate (GFR) had declined from 82.0 (42-114) ml/min per 1.73 m2 body surface area to 70.8 (21.3-126.5) ml/min per 1.73 m2 body surface area (P = 0.001). Our findings confirm that, even with limited diagnostic facilities, protocol-based evaluation permits determination of the etiology of NC in most patients.
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Proximal Convoluted Tubule (PCT):
The PCT is the initial segment of the renal tubule, extending from the Bowman's capsule that encloses the glomerulus. Its convoluted structure and microvilli-lined cells increase the surface area for reabsorption. The PCT reabsorbs glucose, amino acids, sodium, and water from the filtrate, ensuring essential...