OGH doctors perform surgery on 3 children with rare disorder

Three children with a rare genetic liver disorder known as Progressive Familial Intrahepatic Cholestasis (PFIC) were operated on by specialist doctors at the Osmania General Hospital.

Usually, liver transplantation is recommended to treat the disorder. However, doctors from the government hospital used ileal exclusion surgery to operate on two of the patients. This procedure is rarely performed in India. The children were discharged a few days ago.

Head of Surgical Gastroenterology department at OGH, Ch. Madhusudhan said the method can be performed before the liver is damaged completely and helps avoid the need for transplantation.

The disorder is more common among children born to parents in consanguineous marriages (marriage between closely relatives).

Individuals with PFIC will not be able to drain bile from liver even though large bile ducts are open. This leads to liver damage, liver cancer, and deaths.

The doctors said that PFIC is an inherited disease where bile acid transport gene is defective thus leading to damage, fat malabsorption, deficiency of Vitamin A,D,E and K, cirrhosis and cancer.

“If PHIC is detected before developing cirrhosis, we can perform procedures such as ileal exclusion or partial external biliary drainage. Else, if detected in late stages, one will require transplantation,” said Dr Madhusudhan.

Some of the symptoms of the disorder are jaundice, itching all over body, and retarded growth. Two brothers, aged 14 and 16, from Dhanavari Palem of Khammam district, were brought to OGH with the symptoms. Other hospitals had suggested liver transplantation. The parents are daily wage earners.

The OGH team examined them found that their liver was not cirrhotic. Hence, they performed ileal exclusion surgery. Doctors Pandu Naik, Jyothi, Ramesh Kumar, Sudharshan, Venu, Varun were part of the team headed by Dr Madhusudan.

Another year-old child from Karimnagar who suffered from PHIC was detected with liver cirrhosis. Liver transplantation was performed on him. The toddler’s mother donated a portion of her liver.

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Printable version | May 27, 2022 4:33:55 am | https://www.thehindu.com/news/cities/Hyderabad/ogh-doctors-perform-surgery-on-3-children-with-rare-disorder/article38284277.ece