MyPACS.net: Radiology Teaching Files > Case 299306

never visited BARTTER'S SYNDROME
Contributed by: Faculty and residents Children's Hospital, Radiologist, Children's Health System, Birmingham, Alabama., USA.
Patient: 5 year old female
History: history of Bartter's syndrome since infancy
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Findings: Hyperechoic renal medullary pyramids bilaterally.
Diagnosis: Medullary nephrocalcinosis due to Bartter's syndrome.
Discussion:

The differential for hyperechoic medullary pyramids includes medullary nephrocalcinosis, protein deposition (e.g. Tamm-Horsfall protein), crystal deposition (e.g. sodium urate), autosomal recessive polycystic kidney disease, and dystrophic calcification (e.g. due to renal papillary necrosis).

The most common cause is medullary nephrocalcinosis. The differential includes hypercalcemic states (hyperparathyroidism, hypervitaminosis D, sarcoid, metastasis), hypercalciuric states (renal tubular acidosis, furosemide therapy), and medullary sponge kidney.

This particular patient has Bartter's syndrome. This is a renal tubular disorder which results in the inabilty to resord sodium at the loop of Henle. This autosomal recessive disorder leads to a hypokalemic metabolic alkalosis. The patients are hypocalcemic and hypercalciuric secondary to defective renal tubular transporters and an inappropriate ion gradient for calcium reabsorption. This leads to the medullary nephrocalcinosis demonstrated on ultrasound.

References: Bartter Syndrome on eMedicine.com by Lynda A. Frasetto, M.D.
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Additional Details:

Case Number: 299306Last Updated: 05-25-2004
Anatomy: Genitourinary (GU)   Pathology: Metabolic
Modality: USExam Date: 01-14-2004Access Level: Readable by all users
Keywords: bartter's syndrome, medullary nephrocalcinosis

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