Glycogen storage disease type II, also called Pompe disease, is an autosomal recessive metabolic disorder which damages muscle and nerve cells throughout the body. It is caused by an accumulation of glycogen in the lysosome due to deficiency of the lysosomal acid alpha-glucosidase enzyme. It is the only glycogen storage disease with a defect in lysosomal metabolism, and the first glycogen storage disease to be identified, in 1932 by the Dutch pathologist J. C. Pompe. The build-up of glycogen causes progressive muscle weakness (myopathy) throughout the body and affects various body tissues, particularly in the heart, skeletal muscles, liver and the nervous system. Signs and symptoms
Newborn
The infantile form usually comes to medical attention within the first few months of life. The usual presenting features are cardiomegaly (92%), hypotonia (88%), cardiomyopathy (88%), respiratory distress (78%), muscle weakness (63%), feeding difficulties (57%) and failure to thrive (50%).The main clinical findings include floppy baby appearance, delayed motor milestones, and feeding difficulties. Moderate hepatomegaly may or may not be present. Facial features include macroglossia, wide open mouth, wide open eyes, nasal flaring (due to respiratory distress), and poor facial muscle tone.
Patients that have lost excessive amounts of blood may require a blood transfusion.The use of cyanoacrylate glue to prevent bleeding and seal battle wounds was designed and first used in the Vietnam War. Skin glue, a medical version of "super glue", is sometimes used instead of using traditional stitches used for small wounds that need to be closed at the skin level. Etymology
The word "Haemorrhage" (or hæmorrhage; using the æ ligature) comes from Latin haemorrhagia, from Ancient Greek αἱμορραγία (haimorrhagía, "a violent bleeding"), from αἱμορραγής (haimorrhagḗs, "bleeding violently"), from αἷμα (haîma, "blood") + -ραγία (-ragía), from ῥηγνύναι (rhēgnúnai, "to break, burst"). See also
Anaesthesia Trauma and Critical Care
Aneurysm
Autohemorrhaging
Anemia
Coagulation
Contusion
Exsanguination
Hemophage
Hemophilia
Hematoma
Istihadha

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