TY  - JOUR
TT  - X-Linked Myotubular Myopathy and Chylothorax
AU  - Durankuş, Ferit
AU  - Ayvaz, Adnan
AU  - Bozdağ, Şenol
AU  - Abdullah Göl, Canan
AU  - Şenkal, Evrim
PY  - 2018
DA  - September
JF  - International Anatolia Academic Online Journal Health Sciences
JO  - IAAOJH
PB  - Abdülkadir IŞIK
WT  - DergiPark
SN  - 2148-3159
SP  - 40
EP  - 47
VL  - 4
IS  - 2
LA  - en
KW  - Chylothorax
KW  - X-linked myotubular myopathy
KW  - Neonate
N2  - Myotubular myopathy, also known as centronuclear myopathy, is acongenital muscle disease. There are three main types according to itsinheritance, which differ in clinical severity, age of onset, and prognosis.The most common and most severe type is X- linked myotubular myopathy. X-linkedmyotubular myopathy usually manifests with hypotonia and respiratory distressat birth. Patients with severe types of the disease are often lost during theneonatal period due to respiratory failure. In muscle histopathology, centralnucleation is typical in the majority of muscle fibers, and pleural effusionsare frequently described in congenital myopathies. A 20-day-old male neonateborn via C-section at 36 gestation weeks and 2400 g was referred to ourhospital due to hypotony, edema, and respiratory distress. In this article, wepresent a severe form of the disease in a neonate with chylothorax.
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UR  - https://dergipark.org.tr/tr/pub/iaaojh/article/453045
L1  - https://dergipark.org.tr/tr/download/article-file/545659
ER  -