Glycogen Storage Disease Type 1 / Von Gierke disease : Clinical Biochemistry by Dr Prakash Mungli
📌𝐅𝐨𝐥𝐥𝐨𝐰 𝐨𝐧 𝐈𝐧𝐬𝐭𝐚𝐠𝐫𝐚𝐦:- https://www.instagram.com/drgbhanuprakash
📌𝗝𝗼𝗶𝗻 𝗢𝘂𝗿 𝗧𝗲𝗹𝗲𝗴𝗿𝗮𝗺 𝗖𝗵𝗮𝗻𝗻𝗲𝗹 𝗛𝗲𝗿𝗲:- https://t.me/bhanuprakashdr
📌𝗦𝘂𝗯𝘀𝗰𝗿𝗶𝗯𝗲 𝗧𝗼 𝗠𝘆 𝗠𝗮𝗶𝗹𝗶𝗻𝗴 𝗟𝗶𝘀𝘁:- https://linktr.ee/DrGBhanuprakash
Glycogen Storage Disease Type 1/ Von Gierke disease : Clinical Biochemistry by Dr Prakash Mungli
von Gierke disease
-------------------------------
Email this page to a friend Print Facebook Twitter Pinterest
Von Gierke disease is a condition in which the body cannot break down glycogen. Glycogen is a form of sugar (glucose) that is stored in the liver and muscles. It is normally broken down into glucose to give you more energy when you need it.
Von Gierke disease is also called Type I glycogen storage disease (GSD I).
Causes
------------
Von Gierke disease occurs when the body lacks the protein (enzyme) that releases glucose from glycogen. This causes abnormal amounts of glycogen to build up in certain tissues. When glycogen is not broken down properly, it leads to low blood sugar.
Von Gierke disease is inherited, which means it is passed down through families. If both parents carry a nonworking copy of the gene related to this condition, each of their children has a 25% (1 in 4) chance of developing the disease.
Symptoms
------------------
These are symptoms of von Gierke disease:
Constant hunger and need to eat often
Easy bruising and nosebleeds
Fatigue
Irritability
Puffy cheeks, thin chest and limbs, and swollen belly
Exams and Tests
Your health care provider will perform a physical exam.
The exam may show signs of:
Delayed puberty
Enlarged liver
Gout
Inflammatory bowel disease
Liver tumors
Severe low blood sugar
Stunted growth or failure to grow
Children with this condition are usually diagnosed before age 1.
Tests that may be done include:
Biopsy of liver or kidney
Blood sugar test
Genetic testing
Lactic acid blood test
Triglyceride level
Uric acid blood test
If a person has this disease, test results will show low blood sugar and high levels of lactate (produced from lactic acid), blood fats (lipids), and uric acid.
Treatment
-----------------
The goal of treatment is to avoid low blood sugar. Eat frequently during the day, especially foods that contain carbohydrates (starches). Older children and adults may take cornstarch by mouth to increase their carbohydrate intake.
In some children, a feeding tube is placed through their nose into the stomach throughout the night to provide sugars or uncooked cornstarch. The tube can be taken out each morning. Alternatively, a gastrostomy tube (G-tube) can be placed to deliver food directly to the stomach overnight.
A medicine to lower uric acid in the blood and decrease the risk for gout may be prescribed. Your provider may also prescribe medicines to treat kidney disease, high lipids, and to increase the cells that fight infection.
People with von Gierke disease cannot properly break down fruit or milk sugar. It is best to avoid these products.
#type1glycogenstoragedisease #vongierkesdisease #usmle #usmlestep1 #glycogenstoragediseasetype1 #glycogenstoragediseases #usmlevideos #medicalbiochemistry
Видео Glycogen Storage Disease Type 1 / Von Gierke disease : Clinical Biochemistry by Dr Prakash Mungli канала Dr.G Bhanu Prakash Animated Medical Videos
📌𝗝𝗼𝗶𝗻 𝗢𝘂𝗿 𝗧𝗲𝗹𝗲𝗴𝗿𝗮𝗺 𝗖𝗵𝗮𝗻𝗻𝗲𝗹 𝗛𝗲𝗿𝗲:- https://t.me/bhanuprakashdr
📌𝗦𝘂𝗯𝘀𝗰𝗿𝗶𝗯𝗲 𝗧𝗼 𝗠𝘆 𝗠𝗮𝗶𝗹𝗶𝗻𝗴 𝗟𝗶𝘀𝘁:- https://linktr.ee/DrGBhanuprakash
Glycogen Storage Disease Type 1/ Von Gierke disease : Clinical Biochemistry by Dr Prakash Mungli
von Gierke disease
-------------------------------
Email this page to a friend Print Facebook Twitter Pinterest
Von Gierke disease is a condition in which the body cannot break down glycogen. Glycogen is a form of sugar (glucose) that is stored in the liver and muscles. It is normally broken down into glucose to give you more energy when you need it.
Von Gierke disease is also called Type I glycogen storage disease (GSD I).
Causes
------------
Von Gierke disease occurs when the body lacks the protein (enzyme) that releases glucose from glycogen. This causes abnormal amounts of glycogen to build up in certain tissues. When glycogen is not broken down properly, it leads to low blood sugar.
Von Gierke disease is inherited, which means it is passed down through families. If both parents carry a nonworking copy of the gene related to this condition, each of their children has a 25% (1 in 4) chance of developing the disease.
Symptoms
------------------
These are symptoms of von Gierke disease:
Constant hunger and need to eat often
Easy bruising and nosebleeds
Fatigue
Irritability
Puffy cheeks, thin chest and limbs, and swollen belly
Exams and Tests
Your health care provider will perform a physical exam.
The exam may show signs of:
Delayed puberty
Enlarged liver
Gout
Inflammatory bowel disease
Liver tumors
Severe low blood sugar
Stunted growth or failure to grow
Children with this condition are usually diagnosed before age 1.
Tests that may be done include:
Biopsy of liver or kidney
Blood sugar test
Genetic testing
Lactic acid blood test
Triglyceride level
Uric acid blood test
If a person has this disease, test results will show low blood sugar and high levels of lactate (produced from lactic acid), blood fats (lipids), and uric acid.
Treatment
-----------------
The goal of treatment is to avoid low blood sugar. Eat frequently during the day, especially foods that contain carbohydrates (starches). Older children and adults may take cornstarch by mouth to increase their carbohydrate intake.
In some children, a feeding tube is placed through their nose into the stomach throughout the night to provide sugars or uncooked cornstarch. The tube can be taken out each morning. Alternatively, a gastrostomy tube (G-tube) can be placed to deliver food directly to the stomach overnight.
A medicine to lower uric acid in the blood and decrease the risk for gout may be prescribed. Your provider may also prescribe medicines to treat kidney disease, high lipids, and to increase the cells that fight infection.
People with von Gierke disease cannot properly break down fruit or milk sugar. It is best to avoid these products.
#type1glycogenstoragedisease #vongierkesdisease #usmle #usmlestep1 #glycogenstoragediseasetype1 #glycogenstoragediseases #usmlevideos #medicalbiochemistry
Видео Glycogen Storage Disease Type 1 / Von Gierke disease : Clinical Biochemistry by Dr Prakash Mungli канала Dr.G Bhanu Prakash Animated Medical Videos
Показать
Комментарии отсутствуют
Информация о видео
28 августа 2021 г. 18:45:13
00:14:26
Другие видео канала
Migratory Conditions In Medicine - Quick BitesAction Of Lumbrical Muscles - Quick BitesRubella Virus Rash - Quick bitesPrimary Glaucoma 👁 : Ophthalmology LectureAssociation Between Hla And AutoimmunityHearing Loss Associated Conditions - Quick BitesDifferences Between Benign And Mlignant NeoplasmsRemarkable Rapid Revision Psychiatry || Dr Nadeem Zafar on 7th Dec #fmgejan2024 #fmgeexamDifferent Types Of CollagenPentad Of Thrombotic Thrombocytopenic Purpura - Quick BitesDifference Between Thromboxane A2 And Prostaglandin I2ENT Rapid Revision Part - 3 : Remarkable Rapid Revision series FMGE Jan 2023 #fmgejan2023Thiamine / Vitamin B1 (Animation) 🧠💔 : Medical Biochemistry USMLE Step 1Endometrial Cancer - OBG Live session By Dr AgalyaMeckel's Diverticulum - Quick Bites𝗥𝗲𝗺𝗮𝗿𝗸𝗮𝗯𝗹𝗲 𝗥𝗮𝗽𝗶𝗱 𝗥𝗲𝘃𝗶𝘀𝗶𝗼𝗻 𝗦𝘂𝗿𝗴𝗲𝗿𝘆Reye's syndrome - Quick bitesMuscle Physiology Part 2Rapid Review Physiology Part - 2 By Dr. Sree Teja : FMGE July 2023Metabolism of Tryptophan By Dr Pooja For MBBS 1st ProffNeuroanatomy for Mbbs 1st proff by Dr Abhijeet