Abstract: Topical use of corticosteroids may cause immunosuppression and iatrogenic Cushing’s syndrome via hypothalamic‐pituitary‐adrenal axis. We report two cases with iatrogenic Cushing’s syndrome with different clinical outcomes due to abuse of same potent topical steroid clobetasol propionate. One of them died because of fatal disseminated cytomegalovirus infection. The other patient recovered completely. Physicians and parents should be informed about the adverse effects of such potent topical corticosteroids and physicians should prescribe less potent agents, especially during infancy.
Acute lymphoblastic leukemia (ALL) is the most common malignancy of childhood.Central nervous system is the most frequent place of both at the onset and relapse of the disease, but orbital involvement is rarely seen.A seven-year-old girl diagnosed with high risk ALL and Turkish ALL-Berlin Frankfurt Münster (BFM)-2000 high risk group treatment was given.However, after induction and consolidation treatments, before the maintenance treatment she had blindness in her right eye.Ophthalmologic examination her visual acuity was fingers to count in two meters.In her right eye there was exudative retinal detachment and her orbital magnetic resonance imaging (MRI) showed that, there was a mass lesion in the posterior of right orbital globe, on the neighbourhood of optic disc, consistent with metastasis.After ALL-REZ-BFM-2002 relapse protocol treatments and radiotherapy her visual acuity was increased to 6/10.In control orbital MRI regression of mass lesion size was established.Bone marrow transplantation was planned.Orbital involvement is rarely seen in leukemia, but it is responsive to chemotherapy and radiotherapy combination.
AMAC: Hemofili hastalarinda, uygun proflaktik tedavi ve hasta-aile egitimine ragmen, acil servise en sik basvuru sebebi travmalar olarak bilinmektedir.YONTEMLER: Bu calismada bir universite hastanesi acil poliklinigine basvuran hemofili hastalarinin demografik verileri, infeksiyon kaynakli olmayan basvuru nedenleri, travma tipi, kanama odaklari retrospektif olarak incelenmistir. BULGULAR: Hemofili A hastalarinin ortalama yasi 16.6±9.1(1-32) yil iken, Hemofili B hastalarinin 17.1±7.1(5-27) yil idi. Hemofili A hastalarinin 1 tanesi orta tip, 1 tanesi hafif tip olup, digerleri agir tip idi. Hemofili B hastalarinin ise 2 tanesi orta tip olup digerleri agir tip idi. Yalniz 1 agir hemofili A hastasi proflaktik pihtilasma faktor tedavisi almakta idi. Hicbir hastada inhibitor pozitifligi yoktu. 2007-2013 yillari arasinda, acil poliklinigine 13 Hemofili A hastasinin 381(%70), 7 Hemofili B hastasinin 166 (%30) adet basvurusu olmustur. Basvurularin % 87 si agir tip hemofili, % 13 unu ise orta ve hafif tip hemofili hastalari olusturmakta idi. Toplam 547 basvurunun 311 (%57) i travma veya kanama, 236 (%43) si rutin pihtilasma faktor tedavisi alma amaci ile oldugu goruldu. Kanama yerleri incelendiginde, eklem ici kanama (n: 248), burun kanamasi (n: 18), agiz ici kanama (n: 14), hematuri (n: 14), iliopsoas kas ici kanama (n: 5), kafa ici kanama olmaksizin kafa travmasi (kafa-kafaya carpisma, basina televizyon ve dolap dusme n: 5), sinek-bocek isirigi sonrasi hematom (n: 4), bisiklete binme sonrasi anal-skrotal bolgede hematom (n: 3) oldugu saptandi.SONUC: Sonuc olarak, hemofili hastalarimizda eklem ici kanamalarin fazlaligina karsin kafa ici kanama olmamasi sevindiricidir. Diger yandan rutin pihtilasma faktor tedavisi icin hastane basvurusunun fazla olmasi bize aile ve/veya hastanin kendi kendine infuzyon egitiminin yeterli olmadigini gosterdi. Hemofili hastalarinda travma veya ev ici kazalardan korunmak icin hasta-aile egitimleri artirilmali ve surekli olmalidir.
A premarital screening program aiming at reducing the incidence of thalassemia major was started under the auspices of the Regional Health Administration in 1995 in the city of Denizli in the Aegean region of Turkey. In this report we assessed the 4-year results of the screening program. All couples who applied for marriage procedures were screened for β-thalassemia trait by automatic red cell indices and Hb A<sub>2</sub> determination. The couples at risk were counseled and offered prenatal diagnosis and termination of pregnancy in case of an affected fetus. From October 1995 to August 1999, a total of 19,804 subjects (9,902 couples) were recruited for this study. The prevalence of β-thalassemia trait with increased Hb A<sub>2</sub> was found to be 2.6% (514/19,804). In addition to the thalassemia trait, 22 patients (0.11%) had sickle trait. In 15 of the 9,902 couples, both partners were found to be carriers of the β-thalassemia trait. After genetic counseling, 2 of the 15 planned carrier marriages were canceled. Seven couples declared that they do not want to have a child at present. Prenatal diagnosis was sought by 6 couples. One fetus was found to be normal, 4 had thalassemia minor and 1 had thalassemia major; this pregnancy was terminated by elective abortion. This study indicated that premarital screening is a very useful tool for detecting carrier couples and an effective way of controlling thalassemia major.
ÖZLeishmaniasis, sıklıkla 5 yaş altında görülen retiküloendotelyal sistemi tutan enfeksiyon hastalığıdır.Visseral enfeksiyon, uzun süreli ateş, kilo kaybı, halsizlik, pansitopeni, hepatosplenomegaliye sebep olabilir.Türkiye'de Visseral Leishmaniasis'ten (VL) sorumlu etken Leishmania infantum'dur.Bu yazıda İndirekt Floresan
Hemophagocytic lymphohistiocytosis (HLH) represents a severe hyperinflammatory condition with cardinal symptoms of prolonged fever, cytopenias, hepatosplenomegaly, and hemophagocytosis by activated, morphologically benign macrophages with impaired function of natural killer cells and cytotoxic T lymphocytes. A 2-month-old girl, who was admitted with fever, was diagnosed with HLH and her genetic examination revealed a newly defined mutation in the UNC13D (c.175G>C; p.Ala59Pro) gene. She was treated with dexamethasone, etoposide, and intrathecal methotrexate. During the second week of treatment, after three doses of etoposide, it was noticed that there was a necrotic plaque lesion on the soft palate. Pathologic examination of debrided material in PAS and Grocott staining revealed lots of septated hyphae, which was consistent with aspergillosis infection. Etoposide was stopped and amphotericin B treatment was given for six weeks. HLH 2004 protocol was completed to eight weeks with cyclosporine A orally. There was no patient with invasive aspergillosis infection as severe as causing palate and nasal septum perforation during HLH therapy. In immunocompromised patients, fungal infections may cause nasal septum perforation and treatment could be achieved by antifungal therapy and debridement of necrotic tissue.Hemofagositik lenfohistiositoz (HLH) uzamış ateş, sitopeni, hepatosplenomegali semptomları ile seyreden, active olmuş, morfolojik olarak benign makrofaj ve doğal öldürücü hücreler ile sitotosik T lenfosit fonksiyon bozukluğu sonucu gelişen hiperenflamatuvar bir durumdur. İki aylık düşmeyen ateş yakınması ile başvuran hasta HLH tanısı aldı ve hastanın genetik incelemesinde UNC13D (c.175G>C; p.Ala59Pro) geninde yeni tanımlanan bir mutasyon saptandı. Hastaya deksamatazon, etopozit ve intratekal metotreksat tedavileri başlandı. Tedavinin 2. haftasında, üç doz etopozit aldıktan sonra, yumuşak damakta plak lezyonu fark edildi ve bu nekrotik lezyon debride edildi. Debridman materyalinin patolojik incelemesinin PAS, Grocott boyamasında aspergilloz enfeksiyonu ile uyumlu olarak çok sayıda septalı hif görüldü. Etopozid tedavisi sonlandırılarak altı hafta boyunca amphotericin B tedavisi verildi. HLH 2004 tedavi protokolü oral siklosporin ile sekiz haftaya tamamlandı. HLH tedavisi sırasında yumuşak damak perforasyonuna neden olacak kadar ağır aspergilloz enfeksiyonu geçiren bir olgu bildirilmemiştir. İmmünyetmezlikli hastada mantar enfeksiyonları nazal septum perforasyonuna neden olabilmektedir ve tedavi nekrotik dokunun debridmanı ve antifungal tedavi ile sağlanabilmektedir.
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