Hybrid Classes
We provide offline, online and recorded lectures in the same amount.
Personalised Mentoring
Every aspirant is unique and the mentoring is customised according to the strengths and weaknesses of the aspirant
Topicwise Mindmaps
In every Lecture. Director Sir will provide conceptual understanding with around 800 Mindmaps.
Quality Content
We provide you the best and Comprehensive content which comes directly or indirectly in UPSC Exam.
DAILY NEWS ANALYSIS
PRINT
06 May, 2020
5 Min Read
Maple syrup urine disease (MSUD) is a rare genetic disorder characterized by a deficiency of certain enzymes (branched-chain alpha-keto acid dehydrogenase complex) required to break down (metabolize) the three branched-chain amino acids (BCAAs) [Leucine, Isoleucine and Valine] in the body.
The result of this metabolic failure is that all three BCAAs, along with their various byproducts, accumulate abnormally throughout the body.
In the classic, severe form of MSUD, the plasma concentrations of the BCAAs begin to rise within a few hours of birth. If untreated, symptoms begin to emerge, often within the first 24-48 hours of life.
Symptoms of MSUD
The “non-specific” symptoms are those of increasing neurological dysfunction and include lethargy, irritability and poor feeding, followed soon by focal neurological signs such as abnormal movements and increasing spasticity, and shortly thereafter, by convulsions and deepening coma.
If untreated, progressive brain damage is inevitable and death ensues usually within weeks or months. The finding that is unique to MSUD is the emergence of a characteristic odor, reminiscent of maple syrup that can most readily be detected in the urine and earwax and may be smelled within a day or two of birth.
The disorder can be successfully managed through a specialized diet. However, even with treatment, both affected children and adults patients with MSUD remain at high risk for developing episodes of acute illness (metabolic crises) often triggered by infection, injury, failure to eat (fasting) or even by psychological stress. During these episodes there is a rapid, sudden spike in amino acid levels necessitating immediate medical intervention.
Types of MSUD
Source: TH
06 Feb, 24
Recently, Prime Minister announced Pradhan Mantri Suryodaya Yojana under which 1 crore households will get rooftop solar power systems. India’s Status of Current Solar Capacity India currently stands at 4th place globally in solar power capacity. As per Ministry of New an
06 Feb, 24
The Foreign Contribution Regulation Act, 2010 (FCRA) registration of two prominent non-governmental organisations (NGOs) — Centre for Policy Research (CPR) and World Vision India (WVI) have been cancelled this month. What is FCRA? Key provisions of FCRA, 2010 Key aspects Description
06 Feb, 24
Voice clone fraud has been on the rise in India. AI voice cloning – It is the process of creating a synthetic replica of a person’s voice through machine learning and speech synthesis technology.It is called as voice deepfakesor audio deepfakes. Objective – To achieve a high level of na
06 Feb, 24
Steps taken by India to promote Science Communication Publications and Information Directorate (PID) - An organisation under Council of Science and Industrial Research (CSIR) established in 1951 for publishing and disseminating scientific information in India. National science magazines- The PI
05 Feb, 24
Universal Basic Income (UBI) can strengthen welfare architecture and unlock the nation’s latent demographic potential. UBI - It is an income support mechanism typically intended to reach all or a very large portion of the population regardless of their earnings or employment status. Objective- To provide enough to co
06 Feb,2024
Our Popular Courses
Module wise Prelims Batches
Mains Batches
Test Series
Copyright 2023 CLT TECHNOLOGIES AND EDU-PUBLISHERS PRIVATE LIMITED. All rights reserved.
24 × 7 Support : 8287714422
Whatsapp : 8010068998
Email Us: query@aspireias.com
My Notes
Geography And Environment
