Latar belakang. Demam tifoid masih merupakan masalah kesehatan masyarakat yang penting di Indonesia. Gambaran klinis tifoid sangat bervariasi, seperti gejala klinis ringan hingga berat yang disertai komplikasi. Tujuan. Untuk mengetahui gambaran manifestasi klinis, laboratorium, dan tata laksana DT anak yang dirawat di RSUP Dr. Sardjito. Metode. Penelitian retrospektif ini dilakukan menggunakan data rekam medis pasien DT anak yang menjalani rawat inap sejak Januari 2011 hingga Mei 2016. Hasil. Ditemukan 158 kasus DT yang terbukti secara serologis (96,8%), kultur darah (1,3%), maupun keduanya (1,9%). Demam merupakan gejala utama pada seluruh pasien. Gejala penyerta lain adalah anoreksia (54,4%), mual (49,4%), muntah (41,8%), nyeri kepala (37,3%), batuk (37,3%), nyeri perut (34,2%), konstipasi (30,4%), dan diare (29,7%). Tanda yang paling sering didapatkan adalah pembesaran hati (29,7%), nyeri tekan abdomen (20,3%), limfadenopati (13,3%), letargi (13,3%), lidah kotor (12,0%), pembesaran limpa (6,3%) dan penurunan kesadaran (4,4%). Rerata lama rawat inap adalah 8,4±6,2 hari, mortalitas 2,5% diakibatkan oleh sepsis berat. Respon pasien 81,0% terhadap pemberian antibiotik, terbanyak diresepkan adalah ceftriaxone (45,6%). Komplikasi berupa sepsis (10,1%), hepatitis (8,9%), perdarahan saluran cerna (5,7%), ensefalopati (4,4%), dan perforasi usus (0,6%). Kesimpulan. Profil klinis anak dengan DT serupa dengan gambaran umum pasien di daerah endemis. Penegakan diagnosis pasti dengan menggunakan kultur darah masih belum banyak membantu di rumah sakit kami. Mortalitas yang masih cukup tinggi perlu diteliti lebih lanjut untuk perbaikan prognosis pasien.

McCune-Albright syndrome (MAS) is a rare disease characterized by a triad of fibrous dysplasia, cafe-au-lait spots and peripheral precocious puberty. We reported a 5-year-8-month old girl with MAS who has been followed-up for 2 years and 8 months. She was referred to pediatric endocrinology clinic in our hospital for vaginal bleeding at age of 2 years 11 months. She had peripheral precocious puberty, i.e. increased estrogen level associated with very low gonadotropins, and cafe-au-lait spots on her face and was diagnosed as MAS. The patient was treated with estrogen receptor blocker (tamoxifen). She had no menses during the 2 years and 8 months of tamoxifen treatment. Her growth rate and bone maturation were also in normal ranges. However, at the end of tamoxifen treatment she had an episode of vaginal bleeding so that we had to change to other treatment modalities. ABSTRAK Sindrom McCune-Albright merupakan penyakit langka yang ditandai dengan trias displasia fibrosa, makula cafe-au-lait dan pubertas prekok perifer. Kami melaporkan anak perempuan usia 5 tahun 8 bulan yang telah kami amati selama 2 tahun 8 bulan. Pasien datang ke poliklinik endokrinologi di rumah sakit kami dengan keluhan perdarahan pervaginam pada usia 2 tahun 11 bulan. Ia mengalami pubertas prekok perifer yang ditandai dengan peningkatan kadar estrogen dan rendahnya kadar gonadotropin,, makula cafe-au-lait, dan didiagnosis dengan sindrom McCune-Albright. Pasien diterapi dengan penghambat reseptor estrogen (tamoxifen). Selama terapi, siklus menstruasi terhenti, kecepatan pertumbuhan dan maturasi tulang dalam batas normal. Namun pada akhir terapi dengan tamoxifen, pasien kembali mengalami perdarahan pervaginam sehingga kami harus mengganti dengan modalitas terapi yang lain.

McCune-Albright syndrome (MAS) is a rare disease characterized by a triad of fibrous dysplasia, cafe-au-lait spots and peripheral precocious puberty. We reported a 5-year-8-month old girl with MAS who has been followed-up for 2 years and 8 months. She was referred to pediatric endocrinology clinic in our hospital for vaginal bleeding at age of 2 years 11 months. She had peripheral precocious puberty, i.e. increased estrogen level associated with very low gonadotropins, and cafe-au-lait spots on her face and was diagnosed as MAS. The patient was treated with estrogen receptor blocker (tamoxifen). She had no menses during the 2 years and 8 months of tamoxifen treatment. Her growth rate and bone maturation were also in normal ranges. However, at the end of tamoxifen treatment she had an episode of vaginal bleeding so that we had to change to other treatment modalities. ABSTRAK Sindrom McCune-Albright merupakan penyakit langka yang ditandai dengan trias displasia fibrosa, makula cafe-au-lait dan pubertas prekok perifer. Kami melaporkan anak perempuan usia 5 tahun 8 bulan yang telah kami amati selama 2 tahun 8 bulan. Pasien datang ke poliklinik endokrinologi di rumah sakit kami dengan keluhan perdarahan pervaginam pada usia 2 tahun 11 bulan. Ia mengalami pubertas prekok perifer yang ditandai dengan peningkatan kadar estrogen dan rendahnya kadar gonadotropin,, makula cafe-au-lait, dan didiagnosis dengan sindrom McCune-Albright. Pasien diterapi dengan penghambat reseptor estrogen (tamoxifen). Selama terapi, siklus menstruasi terhenti, kecepatan pertumbuhan dan maturasi tulang dalam batas normal. Namun pada akhir terapi dengan tamoxifen, pasien kembali mengalami perdarahan pervaginam sehingga kami harus mengganti dengan modalitas terapi yang lain.

Sepsis is associated with increased morbidity and mortality in children worldwide, mainly in developing countries. This fatal risk emphasizes the importance of finding accessible and inexpensive parameters to be used as predictors for mortality in children with sepsis. The aim of this study was to determine the role of increased mean platelet volume (MPV) as a predictor for mortality in children with sepsis.  A case control study was applied using medical records of all in-patients aged 1 mo -18 y diagnosed with sepsis at Dr. Sardjito General Hospital, Yogyakarta from January 2015-December 2016. Bivariate and multivariate analyses by Chi-square and logistic regression to evaluate the correlations between increased MPV within the first 24-72 h (ΔMPV>0) and mortality were applied. Eighty-one eligible subjects met the inclusion/exclusion criteria with the mortality was 52%. Chi-square analysis showed significant correlations between increased MPV and mortality (p=0.005). Multivariate analysis showed increased MPV within the first 24-72 h after sepsis diagnosis as a predictor for mortality after controlling for sex and AKI (adjusted OR 3.851; 95% CI:1.354-10.948; p= 0.011). In conclusion, an increase in MPV within the first 24-72 h after diagnosed is an independent predictor for mortality in children with sepsis.