Herbal Remedies for Progressive Familial Intrahepatic Cholestasis (PFIC)

ABSTRACT

Progressive familial intrahepatic cholestasis (PFIC) is a term used to describe a diverse set of juvenile autosomal recessive diseases that affect bile production and manifest as cholestasis of hepatocellular origin. People with conditions like portal hypertension, hepatosplenomegaly, jaundice, or acute itching are specifically targeted. This illness primarily results in cirrhosis and an urgent need for a liver transplant. Progressive familial intrahepatic cholestasis comes in three different forms: PFIC1, PFIC2, and PFIC3. In this article, we will read briefly about Progressive familial intrahepatic cholestasis.

INTRODUCTION OF PFIC

Progressive familial intrahepatic cholestasis is a genetic illness that causes bile to be secreted improperly. It causes progressive liver disease, and at the end, liver failure occurs as a result of the progressive liver disease. Liver disease is caused by clinical signs that first appear in childhood. Typically, this causes cirrhosis, a failure to thrive, and the requirement for liver transplantation.

TYPES OF PFIC

Progressive familial intrahepatic cholestasis is divided into three types:

1. PFIC1
2. PFIC2
3. PFIC3

PFIC1 and PFIC2 typically show up in the first few months of life, while PFIC3 can appear later on, throughout early childhood, young adulthood, or even in infancy. Jaundice, pruritus, and cholestasis are the main clinical symptoms. Patients with PFIC typically experience fibrosis and end-stage liver disease before reaching adulthood.

CAUSES OF PFIC

1. PFIC1 is caused by a gene mutation in ATP8B1.
2. PFIC2 is caused by a gene mutation in ABCB11.
3. PFIC3 is caused by a gene mutation in ABCB4.

PATHOGENESIS OF PFIC

1. PFIC1

This condition is brought on by numerous changes to the P-type ATPase protein (ATP8B1), which is responsible for the movement of phospholipids across membranes. Additionally known as Byler’s illness.

Symptoms

1. Watery stools
2. Deafness
3. Pancreatitis
4. Small stature

2. PFIC2

This condition is brought on by numerous changes to ABCB11, which codes for the bile salt export pump. Hepatocellular carcinoma is a risk factor for those with PFIC2.

Symptoms: Clinical characteristics are more severe than PFIC1.

3. PFIC3

This condition is caused by a number of changes to ABCB4, which is responsible for the translocation of phosphatidylcholine. It damages the biliary epithelium and results in phosphatidylcholine being absent from the bile.

Symptoms: At this stage, liver disease is the primary clinical concern. The majority of symptoms are not revealed until later in early childhood.

SIGNS AND SYMPTOMS

1. Splenomegaly
2. Portal hypertension
3. Hepatomegaly
4. Jaundice
5. Intense pruritus
6. Failure to thrive
7. Bleeding
8. Cholestasis
9. Fat malabsorption
10. Poor body weight gain
11. Vomiting
12. Osteopenia
13. Weakness in the body
14. Nausea

DIAGNOSIS OF PFIC

1. Genetic Testing
2. Bile analysis
3. Electron microscopy
4. Liver function test
5. Ultrasound
6. Liver immunostaining
7. Molecular analysis

TREATMENT OF PFIC

1. Drug therapy
2. Liver transplantation

AYURVEDIC ASPECT OF PROGRESSIVE FAMILIAL INTRAHEPATIC CHOLESTASIS

According to Ayurveda, this disease is associated with the yakrit vikara, in which the pitta dosha predominates. Yakrit vikar results from pitta dosha dysfunction. Agni is symbolised by pitta in the body, with both positive and negative impacts according to its normal or pathological state, such as digestion or indigestion, vision or loss of eyesight, normal or abnormal temperature, and other dual functions. Various diseases are caused by the vitiated pitta dosha.

HERBAL REMEDIES FOR PROGRESSIVE FAMILIAL INTRAHEPATIC CHOLESTASIS BY PLANET AYURVEDA

Planet Ayurveda is a GMP-certified Ayurvedic clinic that formulates various medicines to prevent various health problems and provide relief to sick people. All preparations that are formulated by Planet Ayurveda do not contain any preservatives, additives, or resins. These remedies are 100% pure, are formulated under the strict guidance of Ayurvedic MD practitioners, and do not cause any side effects. For the Treatment of Progressive Familial Intrahepatic Cholestasis, Planet Ayurveda provides many herbal supplements for this disease that do not cause any side effects.

PRODUCT LIST

1. YAKRIT PLIHANTAK CHURNA
2. SANJIVANI VATI
3. KALMEGH GHAN VATI
4. BHOOMI AMLA SWARAS
5. LIV SUPPORT

Product Description

1. YAKRIT PLIHANTAK CHURNA

Yakrit plihantak churna is a multi-herbal churna that is formulated by Planet Ayurveda with the help of Tephrosia purpurea (Sharpunkha), Eclipta alba (Bhringraj), Phyllanthus niruri (Bhumiamla), Andrographis paniculata (Kalmegha), and Cichorium intybus (Kaasni). It is a powerful antioxidant and helps remove the toxin from the body. It helps strengthen the liver and spleen. It is quite effective in protecting liver cells from damage caused by free radicals due to its antioxidant and anti-inflammatory activities. And it also helps boost immunity, antimicrobial properties, and lowers the level of sugar in the blood.

Dosage: Take one tablespoon twice daily.

2. SANJIVANI VATI

Sanjivani vati is a multi-herbal formulation that is formulated by Planet Ayurveda with the help of Ginger Root (Zingiber officinale), Vidanga (Embelia ribes), Amalaki (Emblica officinalis), Guduchi herb (Tinospora cordifolia), and some other herbs. It is beneficial in treating all autoimmune conditions as well as eliminating toxins from the body, increasing immunity, and strengthening the body. It is beneficial for chronic fever, colds, coughs, respiratory tract infections, and other viral diseases.

Dosage: Take 2 tablets twice daily.

3. KALMEGH GHAN VATI

Kalmegh ghan vati is a single herbal formulation that is formulated by Planet Ayurveda with the help of Andrographis paniculata (Kalmegh). It contains liver-protecting properties, antimalarial, antithrombotic, and antibiotic properties. It helps with lack of appetite and blood purification. There are also antipyretic, analgesic, and antiviral effects of kalmegh. It prevents blood from clotting and protects the liver from toxins.

Dosage: Take 2 tablets twice daily.

4. BHOOMI AMLA SWARAS

Bhoomi amla swaras is a single herbal formulation that is formulated by Planet Ayurveda with the help of bhoomi amla extract (Phyllanthus niruri extract). It is hepatoprotective in nature and helps in the treatment of liver conditions. It contains antioxidants that eliminate toxins from the body, clean out all the channels of the body, and prevent ulcers by lowering gastric acid levels. It is indicated in various diseases like indigestion, sore throat, cough, vomiting, peptic ulcer, hyperacidity, anaemia, etc.
Dosage: Take 2 tablespoons twice daily.

5. LIV SUPPORT

Liv support is a multi-herbal formulation that is formulated by Planet Ayurveda with the help of Eclipta alba (Bhringraj), Andrographis paniculata (Kalmegh), Kasani (Cichorium intybus), and Pinus roxburghii (Saral). It is pungent and bitter in taste and dry, light, and hot in effect. It aggravates pitta and pacifies vata and kapha. It is beneficial for the liver. It contains diuretic, digestive, and laxative properties. It is used to treat anaemia, hyperacidity, migraines, ophthalmic conditions, and skin conditions. It is used for splenomegaly, hepatomegaly, and jaundice. It also enhances appetite and digestion.

Dosage: Take 1 capsule twice daily.

CONCLUSION

Progressive familial intrahepatic cholestasis (PFIC) is a term used to describe a diverse set of juvenile autosomal recessive diseases that affect bile production and manifest as cholestasis of hepatocellular origin. Progressive familial intrahepatic cholestasis comes in three different forms: PFIC1, PFIC2, and PFIC3. It causes progressive liver disease, and at the end, liver failure occurs as a result of the progressive liver disease. According to Ayurveda, this disease is associated with the yakrit vikara, in which the pitta dosha predominates. Yakrit vikar results from pitta dosha dysfunction. In Planet Ayurveda, there are some herbal remedies that are beneficial for the treatment of Progressive familial intrahepatic cholestasis without causing any side effects.