대한신장학회

Skip Navigation: Skip to contents

KOR
ENG
전체메뉴보기

간행물 검색

현재 페이지 경로

HOME
간행물
간행물 검색

논문분류	춘계학술대회 초록집
제목	Unusual Renal Presentation of an Autoimmune Disease
저자	Dina Sallam
출판정보	2024; 2024(1):
키워드
초록	Renal tubular acidosis (RTA) is common in adults with primary Sjogren syndrome, but up to our knowledge, it is reported in very few pediatric patients. Here we report a case of a female patients who presented with bilateral recurrent parotitis, unexplained attacks of quadriparesis & polyurea, that proved to be distal type RTA with hypokalemia induced paresis, that was associated with dry mouth and eyes, where clinical and investigations criteria confirmed the diagnosis of Juvenile Sjögren Syndrome with 2ry d-RTA. A 13-year-old female, presented with bilateral recurrent painful facial swelling at angle of the mouth, raising ear lobules, making a facial disfigurement, in addition to repeated attacks of quadriparesis, polyurea. Her history revealed dysphagia to solid food, where she needed to drink plenty of water to swallow solid food, with eye burning sensation. Examinations revealed bilateral parotitis, with was non obstructing, she had dry mouth Repetitive saliva swallowing test & dry eye by Schirmer's test, proximal muscle weakness and polyurea. Investigations revealed hypokalemia, which was the cause of her quadriparesis, normal anion gap metabolic acidosis, and medullary nephrocalcinosis, where she was diagnosed as distal type Renal tubular acidosis (RTA). Immunological markers: high titers of anti-Ro/SSA & anti-La/SSB antibodies, ANA titer ≥1:320 with a positive rheumatoid factor. So, the diagnosis of Juvenile Sjögren Syndrome with distal RTA was confirmed & treatment was started in the form of Corticosteroids + mycophenolate mofetil in addition to the symptomatic treatment of hypokalemia, distal RTA, dry mouth & eye, with marked improvement of the patient’s condition. Conclusion: Juvenile Sjögren Syndrome should be considered in adolescent female with otherwise unexplained RTA. A more in-depth investigations of other renal manifestations is needed, where renal biopsy may be of a great importance in excluding renal damage and the need for immunosuppressive medications.
원문(PDF)	PDF 원문보기

목록

위로가기