Journal of Clinical Pediatrics ›› 2019, Vol. 37 ›› Issue (8): 601-.doi: 10.3969/j.issn.1000-3606.2019.08.011

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Clinical analysis of an infant case with transient pseudohypoaldosteronism—salt-losing crisis

ZHU Yan, KUANG Xinyu, KANG Yulin, ZHU Guanghua, HUANG Wenyan, WU Ying   

  1. Department of Nephrology and Rheumatology, Shanghai Children's Hospital Affiliated to Shanghai Jiao Tong University, Shanghai 200062, China
  • Published:2019-08-09

Abstract: Objective To explore the diagnosis and treatment of transient pseudohypoaldosteronism. Methods Clinical data including clinical manifestations, laboratory findings, diagnosis and treatments of a case with transient pseudohypoaldosteronism were retrospectively analyzed, and related literatures were reviewed. Results A female infant, 3 months of age, was hospitalized due to electrolyte disturbances. Laboratory findings showed hyponatremia, hyperkalemia, metabolic acidosis, elevated plasma aldosterone and renin, normal 17 hydroxyprogesterone, and ultrasonography showed urinary tract abnormalities. The child was diagnosed as congenital adrenal hyperplasia and then given the hormone therapy. When she was 6 months old, she was hospitalized due to electrolyte disturbances again, subsequent laboratory findings showed hyponatremia, hyperkalemia, metabolic acidosis, elevated plasma aldosterone and renin, normal 17 hydroxyprogesterone, and urine culture confirmed pyelonephritis caused by Klebsiella pneumonia >105 CFU/mL and urinary tract abnormalities. After prompt correction of electrolyte disturbances and anti-infection, the electrolyte, plasma aldosterone and renin levels quickly returned to normal. Related literatures review was performed using PubMed, and there has no case reported in China but some foreign literatures, we considered the child as a transient pseudohypoaldosteronism caused by urinary tract abnormalities or/and urinary tract infections. Therefore, we stopped the hormone therapy and then gave anti-infection treatment followed by surgical procedures for the treatment of urinary tract anomalies. Conclusions Diagnosis of congenital adrenal disorders is essential in a baby who develops a salt-losing crisis in the first few weeks of life. However, urinary tract abnormalities and urinary tract infection should be considered and rapidly excluded in any infant presenting with a salt-losing crisis with hyponatremia and hyperkalemia in particular in the late neonatal period. Key

Key words: salt-losing crisis; pseudohypoaldosteronism; urinary tract infection; urinary tract abnormalities