Abetalipoproteinemia (ABL) [अॅबेटिलीप्रोटीनेमिया] is a disorder that interferes with the normal absorption of fat and fat-soluble vitamins from food. It is caused by a mutation in microsomal triglyceride transfer protein resulting in deficiencies in the apolipoproteins B-48 and B-100, which are used in the synthesis and exportation of chylomicrons and VLDL respectively. It is a rare autosomal recessive disorder. Other names include Bassen-Kornzweig syndrome, Microsomal triglyceride transfer protein deficiency disease, MTP deficiency, Betalipoprotein deficiency syndrome. Siddha Spirituality of Swami Hardas Life System is of the opinion that our valuable readers to know about this unique and rarest disorder for well-being.
Abetalipoproteinemia Definition (अबेटीलिपोप्रोटीनेमिया की परिभाषा)
Abetalipoproteinemia is a condition characterized by the inability to fully absorb dietary fats, cholesterol, and fat-soluble vitamins. Signs and symptoms appear in the first few months of life and can include failure to thrive; diarrhea; acanthocytosis; and stool abnormalities.
Abetalipoproteinemia Symptoms (एबिटालिपोप्रोटीनेमिया के लक्षण)
Often symptoms will arise that indicate the body is not absorbing or making the lipoproteins that it needs. These symptoms usually appear en masse.
These symptoms come as follows:
- Failure to thrive/failure to grow in infancy
- Steatorrhea/fatty, pale stools
- Frothy stools
- Foul-smelling stools
- Protruding abdomen
- Intellectual disability/developmental delay
- Developmental coordination disorder, evident by age ten
- Muscle weakness
- Slurred speech i.e. dysarthria
- Scoliosis i.e. curvature of the spine
- Progressive decreased vision
- Balance and coordination problems
Abetalipoproteinemia Features (एबिटालिपोप्रोटीनेमिया की विशेषताएं)
Abetalipoproteinemia affects the absorption of dietary fats, cholesterol, and certain vitamins. People affected by this disorder are not able to make certain lipoproteins, which are molecules that consist of proteins combined with cholesterol and particular fats called triglycerides. This leads to multiple vitamin deficiencies, affecting the fat-soluble vitamin A, vitamin D, vitamin E, and vitamin K. However, many of the observed effects are due to vitamin E deficiency in particular.
The signs and symptoms of Abetalipoproteinemia appear in the first few months of life because pancreatic lipase is not active in this period. They can include:
- Weight gain failure and grow at the expected rate
- Abnormal spiny red blood cells i.e. acanthocytosis
- Fatty, foul-smelling stools i.e. steatorrhea
The stool may contain large chunks of fat and/or blood. Infants often present with gastrointestinal problems caused by poor fat absorption, which also contributes to steatorrhea. Other features of this disorder may develop later in childhood and often impair the function of the nervous system.
They can include:
- Poor muscle coordination
- Difficulty with balance and movement i.e. ataxia
- The progressive degeneration of the retina
Adults in their thirties or forties may have increasing difficulty with balance and walking. Many of the signs and symptoms of Abetalipoproteinemia result from a severe vitamin deficiency, especially vitamin E deficiency, which typically results in eye problems with degeneration of the spinocerebellar and dorsal column tracts.
Abetalipoproteinemia Genetics (एबिटालिपोप्रोटीनेमिया जेनेटिक्स)
The disease is extremely rare with approximately 100 reported cases worldwide since it was first identified by doctors Bassen and Kornzweig in 1950.
Abetalipoproteinemia Mechanism (एबिटालिपोप्रोटीनेमिया तंत्र)
Abetalipoproteinemia affects multiple physiological systems, the two most common being nervous and skeletal. Disruption of a nervous function includes:
- Loss of reflexes
- Speech impairments
- Tremors or involuntary motor tics
- Peripheral neuropathy (damage to the nerves outside of the brain and spinal cord)
Peripheral neuropathy causes:
- Loss of sensation
- Weakness, or numbness
- Pain in the extremities through stabbing
- Burning, or tingling sensations
Such symptoms are experienced by people affected by diabetes mellitus. Skeletal system developments often include lordosis, kyphoscoliosis, or pes cavus. Individuals often have abnormal bleeding due to the difficulty of forming clots.
Additional complications of the diseases if not properly treated include:
- Mental deterioration
- Loss of peripheral nerve function
Abetalipoproteinemia Diagnosis (एबिटालिपोप्रोटीनेमिया का निदान)
The initial workup of Abetalipoproteinemia typically consists of stool sampling, a blood smear, and a fasting lipid panel, though these tests are not confirmatory. As the disease is rare, though a genetics test is necessary for diagnosis, it is generally not done initially.
However, prenatal testing may be available for pregnancies identified to be at an increased risk.
The inability to absorb fat in the ileum will result in steatorrhea, or fat in the stool. As a result, this can be clinically diagnosed when foul-smelling stool is encountered. Low levels of plasma chylomicron are also characteristic.
There is an absence of apolipoprotein B. On intestinal biopsy, vacuoles containing lipids are seen in enterocytes. This disorder may also result in fat accumulation in the liver. Because the epithelial cells of the bowel lack the ability to place fats into chylomicrons, lipids accumulate at the surface of the cell, crowding the functions that are necessary for proper absorption.
Abetalipoproteinemia Treatment (एबिटालिपोप्रोटीनेमिया का उपचार)
Normally, treatment consists of rigorous dieting, involving massive amounts of vitamin E. High-dose Vitamin E therapy helps the body restore and produce lipoproteins, which people with Abetalipoproteinemia usually lack.
Vitamin E also helps keep skin and eyes healthy; studies show that many affected males will have vision problems later on in life. Common additional supplementation includes medium-chain fatty acids and linoleic acid. Treatments also aim to slow the progression of nervous system abnormalities.
Developmental coordination disorder and muscle weakness are usually treated with physiotherapy or occupational therapy. Dietary restriction of triglycerides has also been useful. Nutritionists often work with medical professionals to design appropriate dietary treatments for their patients.
Abetalipoproteinemia Prognosis (एबिटालिपोप्रोटीनेमिया का निदान)
If treatment is initiated early in disease the neurologic sequelae may be reversed and further deterioration can be prevented. The long-term outlook is reasonably good for most people when diagnosed and treated early.
A case study presented a female patient diagnosed at the age of 11. Despite the relatively late diagnosis, the patient married and at the age of 34, gave birth to a full-term healthy infant. Her medication included vitamin K 10 mg twice a week, beta-carotene 40,000 IU daily, vitamin A 10,000 IU daily, vitamin E 400 IU daily, vitamins B6 and B12, calcium, magnesium, and eye drops.
The best treatment for Abetalipoproteinemia (एबिटालिपोप्रोटीनेमिया का सबसे अच्छा उपचार)
A common remedy for Abetalipoproteinemia is high dosages of vitamins that are fat-soluble. You can also obtain different supplements, along with linoleic acid, that’s an omega-6 fatty acid. Diet may be a critical part of curing Abetalipoproteinemia. The doctor may additionally suggest speaking to a specialist who’ll let you reduce your fat consumption. This could contain altering to skim milk or ingesting smaller servings fatty ingredients and the meats.
Abetalipoproteinemia and Siddha remedies (एबिटालिपोप्रोटीनेमिया और सिद्ध उपचार)
1. Siddha preventive measures (सिद्ध निवारक उपाय)
Everybody must practice Siddha preventive measures, whether a person is affected with abetalipoproteinemia or not, but preventive measures are the primary steps for switching on to any other Siddha remedies, and hence they are important. It helps in one’s capability, effectiveness, and productivity, decision making power, intellectuality, and removing minor health problems. There are three types of preventive measures:
- Earthing – performed for earthing the negativity of our body
- Field Cleaning – cleans energy field (Aura) of our body
- Siddha Brain Exercise/Energizing – energizes our brain for proper functionality
Everybody’s tendency is to get attracted to the word ‘free‘, however, don’t neglect even these Siddha preventive measures are free. Avail the benefits by practicing them sincerely, and regularly. For the ease of understanding what Siddha preventive measures are, please watch a video for a live demonstration.
2. Siddha Shaktidata Yog (सिद्ध शक्तीदाता योग)
This unique Siddha Shaktidata Yog of Siddha Spirituality can solve the problems related to abetalipoproteinemia with Siddha remedies. There is no compulsion of training of ‘Swami Hardas Life System’ methods. This not only gives benefits to self but also it can be used for other affected persons, whether a person is in the same house, distantly available in the same city, same nation or might be in any corner of the world, however, both the procedures have been explained here.
3. Siddha Kalyan Sadhana (सिद्ध कल्याण साधना)
Recite this Sadhana with a Sankalp “My problems of abetalipoproteinemia are solved as early as possible and I should gain health” (जितनी जल्दी हो सके अॅबेटिलीप्रोटीनेमिया की मेरी समस्याओं का निवारण हो और मुझे स्वास्थ्य हासिल हो ), which should be repeated in mind 3 – 3 times every after each stanza. Any person irrespective of caste, creed, religion, faith, sex, and age can recite this Sadhana, which should be repeated at least twice in a day. To know more, please click on this link.
4. CCPE products (CCPE उत्पाद)
These products work on the concepts of ‘Conceptual Creative Positive Energy’ (CCPE) within the provisions of the ‘CCPE Life System’ and the theory of Quantum Technology to a certain extent. However, the products get activated only whenever touched by a human and then they become capable of solving the problem and achieving health. However, please use these products for abetalipoproteinemia as mentioned below:
CCPE Extractor: The CCPE Extractor should be gently moved over the Agya Chakra in a circular motion at least for 30 to 60 seconds, thereafter, follow the same process on the head, heart, and naval for another 30 to 60 seconds, which finishes within almost 2 – 4 minutes.
CCPE Booster: Keep one Booster over the Agya Chakra and another one each over the head, heart, and naval for 3 – 6 minutes. You may need to have 4 Boosters, which establishes positivity.
5. A daily routine (एक दैनिक दिनचर्या)
In general, a daily routine may look like this:
- Increase physical activities e.g. exercise, walking, swimming, and consume Sattvik diet, etc
- Apply free Siddha remedies minimum 3 times a day, as explained above
- In case, if someone wishes to learn advanced methods of Swami Hardas Life System, undergo unique training
- Perform Swayamsiddha Agnihotra daily, if feasible
Ensure to sprinkle in some fun during the day: Don’t forget to relax and laugh in between. Laughing is a great way to boost your immune system and help you.
Along with all the above activities, apply above explained free Siddha remedies minimum 3 times a day, the more is good. Just try the methods of Siddha Spirituality of Swami Hardas Life System. I am confident that you will surely find improvements within 3 days.
Training of Swami Hardas Life System (स्वामी हरदास लाइफ सिस्टम का प्रशिक्षण)
Any problem with regard to health, peace, and progress can be solved independently without money and medicines by undergoing training of Swami Hardas Life System. Any person irrespective of religion, caste, creed, faith, sex, and age can undergo this unique training.
In view of the above, I am confident that you have learned about abetalipoproteinemia, definition, symptoms, features, genetics, mechanism, causes, diagnosis, prognosis, treatment, best treatment, and Siddha remedies. Now, after reading this article you have become self-sufficient, hence its right time to use your acquired knowledge for solving problems as per the provision available in Siddha Spirituality of Swami Hardas Life System.
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