Nonobstructive Hydronephrosis with Secondary Polycythemia  

Oleh:

Sung, Chih-Chien ; Lin, Shih-Hua

Jenis: Article from Journal - ilmiah internasional
Dalam koleksi: The New England Journal of Medicine (keterangan: ada di Proquest) vol. 365 no. 01 (Jul. 2011), page 365:e1.
Topik: Polycythemia; Nephrogenic Diabetes Insipidus
Fulltext: Nonobstructive Hydronephrosis.pdf (429.29KB)

Ketersediaan

Perpustakaan FK Nomor Panggil: N08.K.2011.02 Non-tandon: 1 (dapat dipinjam: 0) Tandon: tidak ada

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Isi artikel

A 34-year-old nonsmoking man was referred for evaluation of a 3-year history of polycythemia that required monthly phlebotomy. His medical history was significant for congenital nephrogenic diabetes insipidus, with a urine output of approximately 12 to 15 liters per day. Physical examination revealed euvolemia and massive, palpable kidneys. The results of laboratory tests showed a hemoglobin level of 20.2 g per deciliter (reference range, 13.5 to 18.0) and a serum creatinine level of 1.6 mg per deciliter (141 µmol per liter) (reference range, 0.7 to 1.2 mg per deciliter [62 to 106 µmol per liter]). The patient showed no shortness of breath and was not hypoxic. Before the initiation of phlebotomy, his red-cell mass was elevated. An elevated serum erythropoietin level of 49.1 U per liter (reference range, 3.7 to 31.5) and the absence of a V617F mutation in the Janus kinase 2 (JAK2) gene suggested secondary polycythemia. Computed tomographic imaging of the abdomen without the use of contrast material revealed a markedly distended urinary bladder (dagger), severe bilateral megaureter (arrows), and extreme bilateral hydronephrosis with paper-thin renal cortices (arrowheads). Uncontrolled nephrogenic diabetes insipidus may result in marked nonobstructive hydronephrosis, which may subsequently cause local renal hypoxia, increased erythropoietin production, and polycythemia. For this patient, the prescription of captopril and hydrochlorothiazide decreased urinary output to 7 to 9 liters per day and permitted the frequency of his therapeutic phlebotomy to be reduced to once every 3 months.

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