P1016 - RENAL MANIFESTATIONS IN MICROVILLUS INCLUSION DISEASE PATIENTS ON TOTAL PARENTERAL NUTRITION
P1016
RENAL MANIFESTATIONS IN MICROVILLUS INCLUSION DISEASE PATIENTS ON TOTAL PARENTERAL NUTRITION
M. Al-Fraihat1,*, B. Almurbati1, M. Bitzan2, H. Obeidat3, E. Elyyan4, A. kader1
1pediatric gastroentrology, 2pediatric nephrology, 3clinical pharmacy, 4nursing, Al jalila children hospital , dubai, United Arab Emirates
Rationale: Microvillus Inclusion Disease is a rare congenital enteropathy in children, often requiring lifelong total parenteral nutrition . While gastrointestinal features are well-documented, renal involvement in MVID remains poorly understood. This study aims to describe renal complications in a pediatric MVID cohort, focusing on both biochemical abnormalities and structural findings.
Methods: A retrospective descriptive study analyzing renal manifestations in a cohort of pediatric patients diagnosed with microvillus inclusion disease (MVID) who are dependent on total parenteral nutrition (TPN). Data were collected from five genetically confirmed MVID patients receiving long-term TPN at a tertiary pediatric care center.
Results: Renal abnormalities were present in all five pediatric MVID patients on long-term TPN. Nephrocalcinosis or nephrolithiasis occurred in 3 patients. Proteinuria and tubular dysfunction (e.g., Fanconi syndrome or potassium-wasting) were seen in 2 patients each. One patient had a congenital anomaly (solitary kidney). Serum creatinine was normal in most, with one mild elevation. Two patients had elevated protein/creatinine ratios (>1.0 mg/g), suggesting glomerular involvement. Urinary calcium was low in most, yet nephrocalcinosis was still observed. Urinary potassium ranged from low to high; one patient had elevated urinary phosphate. Renal imaging showed structural abnormalities in 4 patients
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Conclusion: This study highlights the importance of early renal monitoring in MVID patients. Nephrocalcinosis and proteinuria are common, and careful management of electrolyte disturbances and renal protection is essential. Further research is needed to understand the long-term renal prognosis in MVID and the potential role of genetic factors in renal manifestations.
Disclosure of Interest: None declared