Secondary pseudohypoaldosteronism associated with double renal pelvis and ureter in an infant

DOI 11 References
  • Sugimoto Satoru
    Department of Pediatrics, Kyoto Prefectural University of Medicine Graduate School of Medical Science Department of Pediatrics, Maizuru Medical Center, The National Hospital Organization
  • Mori Jun
    Department of Pediatrics, Maizuru Medical Center, The National Hospital Organization Department of Pediatrics, University of Alberta, Canada
  • Morita Takashi
    Department of Pediatrics, Kyoto Prefectural University of Medicine Graduate School of Medical Science Department of Pediatrics, Maizuru Medical Center, The National Hospital Organization
  • Tanda Koichi
    Department of Pediatrics, Maizuru Medical Center, The National Hospital Organization Department of Neonatology, Kyoto First Red Cross Hospital
  • Komatsu Hiroshi
    Department of Pediatrics, Maizuru Medical Center, The National Hospital Organization
  • Nakajima Hisakazu
    Department of Pediatrics, Kyoto Prefectural University of Medicine Graduate School of Medical Science
  • Kosaka Kitaro
    Department of Pediatrics, Kyoto Prefectural University of Medicine Graduate School of Medical Science

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Other Title
  • 重複腎盂尿管に合併した続発性偽性低アルドステロン血症の乳児例

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We report an eight-month-old girl who developed hyponatremia and hyperkalemia, accompanying poor sucking and poor weight gain. Endocrinological findings demonstrated the high values of serum aldosterone and plasma renin activity. Three dimension computed tomography revealed double renal pelvis and ureter of right kidney. Urinary tract infection was denied. Diagnosis was confirmed as secondary pseudohypoaldosteronism (secPHA) associated with urinary tract malformation. The treatment of NaCl supplementation was successful. Some cases with secPHA have demonstrated life-threatening symptoms such as cardiac arrest, convulsion and consciousness disturbance. When adequate treatment of sodium supplementation is performed for secPHA, clinical outcome is favorable. Early diagnosis and treatment for secPHA are very important. We recommend that endocrinological examination and investigation for kidney and urinary tract should be performed if an infant with hyponatremia is encountered. The onset of secPHA might be influenced by the aldosterone resistance due to premature renal tubules complicated by urinary tract malformation and urinary tract infection. However, the etiology of secPHA, including molecular biology, is not clarified. Further basic research is expected for elucidation of pathology of secPHA.

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