ABSTRAK
Latar belakang : Purpura Henoch-Schӧnlein (PHS) merupakan sindrom klinisyang disebabkan vaskulitis akut sistemik pada pembuluh darah kecil yang palingsering pada anak. Manifestasi klinis PHS sering melibatkan berbagai organ sepertikulit, sendi, gastrointestinal, dan ginjal. Rekurensi terjadi pada hampir 50 % kasusdan memengaruhi prognosis PHS. Sejauh ini belum ada publikasi penelitian PHSyang meliputi manifestasi klinis, laboratorium, serta rekurensi di Indonesia.Tujuan : Mengetahui manifestasi klinis, laboratorium serta rekurensi PHS anak diIndonesia.Metode : Penelitian deskriptif retrospektif. Data diperoleh dari rekam medispasien anak berusia 0-18 tahun dengan diagnosis PHS selama periode 1 Januari2009 hingga 31 Desember 2012 di Rumah Sakit Cipto Mangunkusumo, Jakarta.Hasil : Terdapat 71 kasus PHS dengan rentang usia 2 sampai 16 tahun dan usiatersering pada kelompok 6-8 tahun. Proporsi anak wanita lebih tinggi dibandinglelaki dengan rasio 1,2:1. Semua pasien mengalami purpura palpabel danmanifestasi tersering lainnya adalah gangguan gastointestinal (79 %), artritis atauartralgia (68 %), dan keterlibatan ginjal (41 %), sedangkan yang jarang adalahgangguan neurologis (1 %), dan edema skrotum (4 %). Riwayat infeksi yangmendahului gejala PHS didapatkan pada 56 % kasus. Peningkatan laju endapdarah (88 %) dan trombositosis (60 %) merupakan kelainan laboratorium yangpaling sering ditemukan, diikuti dengan hematuria (41 %), leukositosis (32 %),dan anemia (31 %). Penurunan fungsi ginjal ditemukan pada 6/42 kasus.Perbaikan gejala klinis terlihat dalam waktu kurang dari 4 minggu untukmanifestasi kulit, gastrointestinal, dan persendian. Sebanyak 18/24 subjek denganhematuria mengalami perbaikan dalam waktu 6 bulan. Penurunan fungsi ginjalmenetap tidak ditemukan dalam penelitian ini. Rekurensi didapatkan pada 5/57subjek yang memiliki data pemantauan.Simpulan : Manifestasi klinis tersering pada PHS adalah purpura palpabel,gangguan gastrointestinal, artritis atau artralgia, dan keterlibatan ginjal, sedangkanyang jarang adalah gangguan neurologis dan edema skrotum. Pemeriksaan darahperifer lengkap dan urinalisis sebaiknya dilakukan pada semua pasien PHS untukmendukung diagnosis dan menilai keterlibatan ginjal. Pada semua pasien PHSsebaiknya dilakukan pemantauan minimal selama 6 bulan untuk menilaiketerlibatan ginjal yang mungkin timbul terlambat serta rekurensi
ABSTRACT
Background : Henoch-Schӧnlein purpura (HSP) is a clinical syndrome whichcaused by systemic acute vasculitis in small vessel. Henoch-Schӧnlein purpura isthe most common etiology of vasculitis in children. Clinical manifestationsusually involved several organs, such as skin, joint, gastrointestinal, and kidney.Recurrency occured in almost 50 % cases, and lead to poor prognosis. Up to now,there was no publications of HSP study in Indonesia regarding in clinical profiles,laboratory, and recurrency.Objective : To investigate the clinical characteristics, laboratory, and recurrencyof HSP in Indonesian’s children.Method : A retrospective descriptive study was conducted from medical recordsof children up to 18 years, in Cipto Mangunkusumo Hospital (CMH). Ourparticipants were children diagnosed as having HSP from January 1st 2009 toDecember 31st 2012.Results : There were 71 cases of HSP, with the range of age from 2 years old to16 years old. Mostly subjects were at group age between 6 and 8 years old. Girlwas commonly affected compared to boy (1.2:1). All patients had palpablepurpura, other clinical symptoms that usually occured were gastrointestinal(79 %), arthritis or arthralgia (68 %), and kidney disorder (41 %). Neurologicsymptoms (1 %) and scrotal edema (4 %) were the least found. 56 % of HSPpatient was preceeded by infection history. Laboratory results that commonlyfound were increasing of ESR (88 %), thrombocytosis (60 %), hematuria (41 %),and anemia (31 %), respectively. Kidney function impairment was occured in 6/42cases. Clinical symptoms improvement had shown in less than 4 weeks for skin,gastrointestinal, and joint disorder. Eighteen of twenty four subjects withhematuria had recovery within 6 months. There were no cases of persistent kidneyfunction impairment. Recurrency occured in 5/57 subjects.Conclusion : Clinical manifestations that commonly found in HSP patients werepalpable purpura, gastrointestinal disorder, arthritis or arthralgia, and kidneyinvolvements. Neurological disorder and scrotal edema were less found. Routineblood and urine examination should be done in all HSP patients to confirm thediagnosis and evaluate kidney involvement. In all HSP patients, we suggest to dofollow up on evaluating late kidney involvement and recurrency minimally in 6months period. |
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