title: [Two cases of autosomal dominant polycystic kidney diseases who presented bilateral enlarged kidneys and severe hypertension in the neonatal period].
abstract: Autosomal dominant polycystic kidney disease(ADPKD) is rarely observed in the neonatal period. We report 2 cases of ADPKD who showed bilateral enlarged, hyperechoic kidneys and severe hypertension. It is difficult to differentiate ADPKD from autosomal recessive polycystic kidney disease (ARPKD) based on the initial clinical presentations in this period. In both cases, bilateral enlarged kidneys and severe hypertension were detected without oligohydramnion and respiratory distress. The mother of case 1 has polycystic kidneys. The father of case 2 was diagnosed as ADPKD. Case 2 had heart failure due to hypertension. Angiotensin converting enzyme inhibitor (ACE-I) was administered to both patients and resulted in good control of blood pressure. ADPKD in the neonatal and very early infantile period has diverse clinical courses. In general, although severe cases are rare, some cases have renal failure and/or hypertension as we reported. We emphasize that both the prompt diagnosis of ADPKD and the start of medication are of great importance in the neonatal and very early infantile period. We recommend that neonates and infants with a family history of ADPKD undergo screening including physical examinations, blood pressure measurements and urinalysis.
authors.affiliation: Department of Pediatrics, Yokohama Municipal Citizen's Hospital, Kanagawa, Japan.
authors.forename: Hitoshi
authors.initials: H
authors.lastname: Wakaki
authors.forename: Masahiro
authors.initials: M
authors.lastname: Ikeda
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Hataya
authors.forename: Kenji
authors.initials: K
authors.lastname: Ishikura
authors.forename: Taiji
authors.initials: T
authors.lastname: Nakashima
authors.forename: Yutaka
authors.initials: Y
authors.lastname: Oosako
authors.forename: Masataka
authors.initials: M
authors.lastname: Honda
mesh_terms: D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D006977:Hypertension, Renal / Q000209:etiology*; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D016891:Polycystic Kidney, Autosomal Dominant / Q000150:complications* / Q000175:diagnosis