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    Disease Burden And Management Of Crigler-Najjar Syndrome: Report Of A World Registry
    (2022) Aronson, Sem J.; Junge, Norman; Trabelsi, Mediha; Kelmemi, Wided; Hubert, Aurelie; Brigatti, Karlla W.; Fox, Michael D.; de Knegt, Robert J.; Escher, Johanna C.; Ginocchio, Virginia M.; Iorio, Raffaele; Zhu, Yan; Ozcay, Figen; Rahim, Fakher; El-Shabrawi, Mortada H. F.; Shteyer, Eyal; Di Giorgio, Angelo; D'Antiga, Lorenzo; Mingozzi, Federico; Brunetti-Pierri, Nicola; Strauss, Kevin A.; Labrune, Philippe; Mrad, Ridha; Baumann, Ulrich; Beuers, Ulrich; Bosma, Piter J.; 35274801
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    Posterior Reversible Encephalopathy Syndrome in Childhood Hematological/Oncological Diseases: Multicenter Results
    (2021) Bilir, Ozlem A.; Dikme, Gurcan; Malbora, Baris; Evim, Melike S.; Sivis, Zuhal O.; Tufekci, Ozlem; Bahadir, Aysenur; Karaman, Serap; Vural, Sema; Bayhan, Turan; Yarali, Husniye N.; Celkan, Tiraje; Ozbek, Namik Y.; 33060391
    The aim of the study was to analyze the characteristics of posterior reversible encephalopathy syndrome (PRES) cases treated at 10 different institutions in our country. Fifty-eight patients diagnosed with PRES were included in this study. The data of PRES cases from 10 departments of pediatric hematology/oncology were analyzed. The mean age of the patients at the time of diagnosis of PRES was 8.95 +/- 3.66 years. Most patients (80.4%) had a primary diagnosis of acute leukemia. Patients received chemotherapy (71.4%) and/or used steroids within 14 days before the diagnosis of PRES (85.7%). Hypertension was found in 83.9% of the patients. Twenty-six patients had infections and 22 of them had febrile neutropenia. The most common electrolyte disorders were hypocalcemia, hypomagnesemia, and hypopotassemia. Six patients had tumor lysis syndrome and 4 had inappropriate antidiuretic hormone syndrome. Magnetic resonance imaging was used for diagnosis in all patients. The most commonly involved regions by magnetic resonance imaging were occipital (58%), parietal (51%), and frontal lobes (45%), respectively. Twenty-five patients required intensive care and 7 patients were intubated. In conclusion, PRES may develop during the follow-up and treatment of hematological diseases. In addition to steroid and intense combined chemotherapies, immunosuppressive agents and hypertension are also factors that may be responsible for PRES.
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    Wernicke Encephalopathy after Gastrointestinal Surgery
    (2015) Saygi, Semra; Savas, Tulin; Alkan, Ozlem; Erol, Ilknur
    We herein describe a child operated for acute abdomen who developed Wernicke's encephalopathy (WE) secondary to prolonged total parenteral nutrition (TPN) that lacked vitamin B1 supplementation. The author concluded that surgeons, child neurologists, pediatricians and radiologists need to be aware of the predisposing factors and symptoms of WE. Clinicians need to keep in mind that ophthalmoplegia, ataxia or altered mental status could be findings of WE.