Abstract Ref Number = APCP1089
OUTCOME OF TUBULOPATHY CHILDREN IN DR SOETOMO HOSPITAL SURABAYA
Ratih Dwi Andini,Chairunisa Harahap,Risky Vitria Prasetyo,Ninik Asmaningsih,Mohammad Sjaifullah Noer
Department of Child Health Faculty of Medicine Universitas Airlangga Dr Soetomo Hospital Surabaya Indonesia
Background : Tubulopathy is selective loss of one or more tubulus cell functions caused by natrium reabsorption disorder in proximal tubulus until ductus coledocus. Without early, adequate, and sustainable treatment of the medication, children with tubulopathy would potentially undergo growth problems, nefrocalcynosis, rachitis, kidney failure, or even mortality. The aim of this study is to determine the outcome of children with tubulopathy.
Material : A retrospective study was performed in 1-18 years-old children with tubulopathy at Dr. Soetomo Hospital from January 2014 to March 2018. There were Bartter syndrome, Gitelman syndrome, and renal tubular acidosis (RTA) groups. Clinical symptoms, physical examination, laboratory, and ultrasonography result were obtained from medical record. The last follow up conditions were noted.
Results : A total of 26 children was included in the study, dominated by male (17/26) children with mean age of 7.1 (SD 5.01) years. RTA was noted in 6/26 children, 9/26 with Bartter syndrome, and 11/26 with Gitelman syndrome. Gitelman syndrome manifestated as polyuria (5/11), reccurent hypokalemia (3/11) and muscle weakness (3/11). RTA manifestated as failure to thrive (3/6), polyuria (2/6) and vomiting (1/6). Bartter syndrome manifestated as polyuria (4/9), reccurent hypokalemia (3/9) and muscle weakness (2/9). From the last follow up, 2/11 children with Gitelman syndrome underwent musculoskeletal symptom, 1/6 children with RTA died, and 9/9 children with Bartter syndrome without any symptom.
Conclusions : Bartter syndrome has better outcome than Gitelman syndrome and RTA
Keywords: Tubulopathy in children outcome Soetomo Hospital Surabaya