Pancytopenia in Propionic Acidemia: Hematologic Evaluation and Studies of Hematopoiesis in Vitro
نویسندگان
چکیده
منابع مشابه
Optic neuropathy in methylmalonic acidemia and propionic acidemia.
BACKGROUND Methylmalonic acidemia (MMA) and propionic acidemia (PA) are rare hereditary disorders of protein metabolism, manifesting early in life with ketoacidosis and encephalopathy and often resulting in chronic complications. Optic neuropathy (ON) has been increasingly recognised in both conditions, mostly through isolated case reports or small cases series. We here report the clinical feat...
متن کاملSecondary Hemophagocytosis in Propionic Acidemia
1Department of Pediatric Metabolism and Nutrition, Diyarbakir Children’s Hospital, Diyarbakir, Turkey 2Pediatric Intensive Care Unit, Diyarbakir Children’s Hospital, Diyarbakir, Turkey 3Department of Pediatric Hematology and Oncology, Diyarbakir Children’s Hospital, Diyarbakir, Turkey 4Department of Pediatric Endocrinology, Diyarbakır Children’s Hospital, Diyarbakir, Turkey 5Department of Pedia...
متن کاملevaluation of sadr eminence in safavid period
چکیده: یکی از دوره های مهم تاریخی ایران به لحاظ تأمین استقلال ملی مذهبی و حتی تأثیر آن بر فرهنگ و مذهب ایرانیان، دوره صفویه است. رسمیت دادن و رواج مذهب شیعه توسط شاه اسماعیل اول، یکی از مهمترین اقدامات این دولت محسوب می شود. بنابراین برای اجرای این سیاست، وی منصب صدارت را به عنوان منصبی مذهبی- حکو متی ایجاد کرد .این منصب از دوره ی تیموریان ایجاد شده بود ولی در اواخر این دوره اهمیت بیشتری یافت...
15 صفحه اولPropionic acidemia in the newborn.
Propionic acidemia is a rare, autosomal, recessively inherited inborn error of propionate metabolism. It presents most often as a neonatal life threatening emergency with metabolic acidosis, hyperammonemia, hyperglycinemia and hyper gylcinuria. Since its first description in a male infant with episodic metabolic acidosis and hyperglycinemia(l), more than 100 cases have been reported. The presen...
متن کاملEmerging trends in management of propionic acidemia.
OBJETIVO To evaluate the therapeutic agents used during metabolic crises and in long-term management of patients with propionic acidemia (PA). MATERIALS AND METHODS The records of PA patients were retrospectively evaluated. RESULTS The study group consisted of 30 patients with 141 admissions. During metabolic crises, hyperammonemia was found in 130 (92%) admissions and almost all patients w...
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ژورنال
عنوان ژورنال: Pediatric Research
سال: 1986
ISSN: 0031-3998,1530-0447
DOI: 10.1203/00006450-198608000-00017