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Brachymetacarpalia
Joseph Junewick, MD FACR
over 7 years ago
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Medullary Nephrocalcinosis

Case Detail

Anatomy: Genitourinary
Junewick
Joseph Junewick, MD FACR
Diagnostic Category: Metabolic
Created: over 8 years ago
Updated: over 7 years ago
Tags: PEDS
Modality/Study Types: US
Activities:
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History

Child with seizure disorder and hematuria.


Case Images


Diagnosis

Topomax (topiramate) induced medullary nephrocalcinosis

Clinical Notes

Taking topiramate for seizure control.

Findings

US – Medullary nephrocalcinosis with twinkle color artifact; calcification at ureterovesical junction.

Discussion

Topomax is used in the management of seizures refractory to conventional therapy. A side-effect is hyperchloremic non-anion gap acidosis (decreased serum bicarbonate). Renal disease, respiratory insufficiency, status epilepticus, diarrhea, ketogenic diet or certain medications can potentiate the acidosis. Metabolic acidosis can cause nephrolithiasis, osteomalacia (rickets), and reduce growth rates.

Medullary nephrocalcinosis accounts for about 90% of nephrocalcinosis in children. Anything that causes hypercalcemia and hypercalciuria can lead to medullary nephrocalcinosis. Other causes of medullary nephrocalcinosis include endocrine dysfunction (hyperparathyroidism, Cushing disease, diabetes insipidus, hyperthyroidism), metabolic (hypervitaminosis D, milk-alkalai syndrome, cystonosis, hyperoxaluria, hyperuricosuria), renal tubular acidosis, drugs (furosemide, steroids), and urinary stasis (obstruction, medullary sponge kidney).

On US, loss of normal hypoechogenicity of the papillae is the earliest sign of medullary nephrocalcinosis. As the disease progresses, hyperechogenicity coalesces and gives rise to posterior acoustical shadowing.



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