PFIC as the name suggests progressive familial intrahepatic cholestasis is a progressive disorder which leads to liver failure. In this disease liver cells fail to secrete enough bile and bile salts. It is a rare disease which equally affects both genders. PFIC is a class of chronic cholestasis disorder which consists of various genetic diseases. Mostly it develops in infancy at the age of three months. Liver plays very specific roles in our body as in fat metabolism, production of proteins, provides immunity, etc. There are three known types of PFIC- PFIC1, PFIC2, and PFIC3. Its symptoms are related to bile buildup and liver disease, especially itching and yellowish skin. It is responsible for 10-15% of children for cholestasis liver disease. In this article we will discuss about progressive familial intrahepatic cholestasis in detail, its causes, symptoms and management according to classical Ayurveda texts.
PFIC is heterogeneous and autosomal recessive disorder during childhood which affects bile formation. Impaired production of bile from liver cells results in cholestasis liver disease. Bile plays a very important role to remove toxins and waste material from our body. It also helps in digesting fat in food we eat. This condition leads to deposition of bile pigments in skin, sclera and mucous membrane. It is characterized by cholestasis during infancy (onset) which progresses rapidly leads to liver cirrhosis and ends into liver failure. This disease causes pruritus and malabsorption. Through radiological examination PFIC is properly diagnosed and then managed accordingly. Most signs and symptoms resemble like liver diseases. PFIC is managed by monitoring, medications and surgically. PFIC is a class of chronic cholestasis disorder which contains a variety of genetic diseases.
The primary reason behind progressive familial intrahepatic cholestasis is “gene mutation”. There is mutation in ATP8B1, ABCB11 and ABCB4 genes.
These genes make protein which helps in regulating bile salts and its composition. It is autosomal recessive disorder so one of the parents acts as carrier without any symptoms of PFIC disorder. Some causes can be as follows
- Genetic Defect
The signs and symptoms for progressive familial intra hepatic cholestasis are as following
- Yellowing of skin
- Itching of skin
- Yellow eyes (sclera)
- Weight loss
- Diarrhea ( lasting for a week)
- Hepatomegaly( enlarged liver)
- Splenomegaly ( enlarged spleen)
- Portal hypertension
- Clay colored stools
- Peripheral edema
- Short stature
- Bleeding ( if vit.k deficiency)
The diagnosis for progressive familial intrahepatic cholestasis are as follows
- Clinical manifestations
- In Ayurveda darshan, prashan and sparshan (medical history and examination)
- Liver ultrasonography
- Liver histology
- Genetic testing
- Liver biopsy
The management for progressive familial intrahepatic cholestasis depends on medical and surgical approach, some methods are as follows
- Dietary changes ( medium chain triglycerides)
- Vitamins supplement (fat soluble- Vit. A,D,K,E)
- Exposure to sunlight
- Calcium intake
- Naso biliary drainage
- Surgical treatment
- Liver transplant
Ayurvedic Point of View Regarding Progressive Familial Intrahepatic Cholestasis
In classical texts, liver/ hepatic disease is known as “yakrit” and disorders related to liver are known as “yakrit vikar”. According to all Acharya’s, the liver is formed from ‘rakta dhatu’ (blood). Liver disorders in Ayurveda are tridoshaja but there is predominance of pitta dosha.
The causative factors for liver disorders in Ayurveda are as follows
- Spicy food
- Intake of alcohol
- Taking preservatives
- Night time awakening
- Day time sleeping
- Suppression of urges like urination, hunger, etc.
Clinical features of liver disorders according to Ayurveda are as follows
- Constipated stool
- Anemia excessive thirst
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Treatment of liver disorders according to Ayurveda are as follows
- Based on balancing kapha and pitta dosha
- Deepan (increase digestive fire)
- Paachan (increase digestion)
- Pitta virechan (laxatives or cholagogues)
- Vaman (induced vomiting)
- Virechan (induced motions)
There are some herbs can be used to correct liver disorders
- Bhumi amalaki (Phyllanthus niruri)
- Haridra (Curcuma longa)
- Loha bhasma ( iron ash)
- Kumari (aloe vera)
- Draksha (Vitis vinifera)
- Kutki (Picrorhiza kurroa)
Herbal Remedies for Progressive Familial Intrahepatic Cholestasis by Planet Ayurveda
Planet Ayurveda is a large manufacturing unit of natural and herbal medicines. It is a well authorized company and certified to GMP and ISO. All the medicinal formulations prepared there are from classical Ayurveda text. They are well known for their purity and drug effectiveness. All products are free from chemicals, any artificial preservative, color, yeast, starch, etc. The best combination provided for Progressive Familial Intrahepatic Cholestasis (PFIC) by planet Ayurveda are as following
- Yakrit Plihantak Churna
- Sanjivani Vati
- Kalmegh Ghan Vati
- Bhoomi Amla Swaras
- Liv Support
1. Yakrit Plihantak Churna
It is standardized powder formulation prepared from extract of bhumi amla (Phyllanthus niruri), bhringraj (Eclipta alba), kalmegh (Andrographis paniculata), Sharpunkha (Tephrosia purpurea), etc. This powder plays a very important role in liver and spleen functioning. It helps in the liver to remove toxins and repair damaged liver cells than grow new liver cells. It helps in maintaining jaundice, liver damage, and fatty liver.
Dosage- One tablespoon twice daily with plain water after meals.
2. Sanjivani Vati
It is a well-known tablet formulation from our ancient classical texts. These tablets are prepared from extract of vidanga (Emblica ribes), ginger root (Zingiber officinalis), pippali (Piper longum), haritaki (Terminalia chebula), etc. It acts as an antioxidant and analgesics properties. It helps in digestion as it acts as an appetizer (increases hunger). It balances kapha and pitta dosha. It removes all the accumulated toxins from the liver.
Dosage- Two tablets twice daily with lukewarm water after meals.
3. Kalmegh Ghan Vati
It is standardized tablet preparation from our classical text and it is prepared from an extract of kalmegh (Andrographis paniculata). This tablet helps in improving the properties of bile and increases bile amount also. It improves digestion due to its laxative property. Kalmegh has antioxidant, anti-inflammatory and hepatic protective properties. These tablets are very useful in management of liver disorders.
Dosage- Two tablets twice daily with plain water after meals.
4. Bhoomi Amla Swaras
This is standardized juice (swaras) formulation prepared from bhoomi amla (Phyllanthus niruri). It acts as antioxidant and anti-viral to liver diseases. It is hepato protective and reverts back if any damage to the liver. It mainly controls kapha and pitta dosha to its normal level. This formulation also has blood purifying properties.
Dosage- Two tablespoons twice daily with equal amounts of water, 15 minutes before meals.
4. LIV Support
It is standardized capsule formulation prepared from extract of kalmegh (Andrographis paniculata), bhringraj (Eclipta alba), saral (Pinus roxburghii) and kaasni (Cichorium intybus). These capsules help to maintain a healthy liver and digestive system.It helps in improving properties of bile and helps in digestion. It has antioxidant, anti-inflammatory and hepato protective property.
Dosage- One capsule twice daily with plain water after meals.
Contact Planet Ayurveda Support Team to provide you the costing / ordering and delivery information at – email@example.com or Call at 0172-521-4030 (India), +91-172-521-4030 (Outside India) or Whatsapp at (+91) 9915-593-604
PFIC is a neonatal liver disease due to inherited molecular defects, when a child is unable to drain bile (cholestasis). It is a progressive disease which leads to liver failure but it can be managed according to symptoms. Above mentioned management can be followed but for any serious condition you must immediately concern the physician.