The life of the Ibrahim family from Iraq came to a standstill when they realised that their young children, Ibrahim, Sozan and Lozan, aged 4, 10 and 11 years respectively, were suffering from a rare genetic disorder called Progressive Familial Intrahepatic Cholestasis (PFIC), a class of chronic cholestasis disorder (a condition where bile cannot flow from the liver to the duodenum) that begins in infancy and usually progresses to liver cirrhosis within the first decade of life.

A team of surgeons, anaesthetists and 30 nurses led by Dr Vivek Vij, Director GI Surgery and Liver Transplant at Fortis Hospital, Noida persevered for nearly 12 hours for each liver transplant for the three Iraqi siblings, to save their precious life.

Dr Vivek Vij, Fortis Hospital Noida said, “This was a rare case and very few such cases have been reported in the medical literature. This is for the first time that we came across a family, wherein all their young children were suffering from PFIC, a rare genetic disorder which had led to stunted growth, low immunity, severe nutritional deficiency, progressive jaundice, bile excretion disorder and diseased liver. The children had recessive genes from both parents as they were born out of consanguine marriage. It also presented a challenge for finding three donors for recipients of the same family. Ideally, the sooner the transplant is performed in such cases, the better is the outcome. But since the two siblings, Lozan and Sozan, were already 11 years and 10 years old respectively, it made the surgery more challenging.”

Lozan (oldest sister) aged 11 years, weighed 24 kgs at the time of the surgery. Her aunt Sherivan was the donor. Sozan, (middle sister), aged 10 years weighed 18 kg. Her maternal uncle Nisrat was the donor. Ibrahim (youngest brother), aged 4 year old weighed only 12 kg at the time of surgery. His mother Bushra willingly agreed to donate a part of her liver.

Dr Vij further explained, “There are three types of PFIC (PFIC1, PFIC2 and PFIC3). Fewer than 200 patients with PFIC1 or PFIC2 are reported in the medical literature. PFIC 3 is even rarer.”

“It has been the most challenging phase of our life and I am glad that it has been a happy ending for our family. We are thankful to the doctors of Fortis Noida, who have miraculously saved all our three children”, said the elated mother Bushraabsullah Ahmed.

After the surgery, the patients were kept under close observation for a week. The children have been discharged and are ready to go back to Iraq.

Fortis Noida has performed more than 150 liver transplants till date.

Case Details

Lozan Ibrahim

Lozan Ibrahim, 11 years had progressive jaundice, pruritus and abdominal distension since the age of 2 years. She was evaluated for familial cholestasis and following liver biopsy, was diagnosed to have Progressive Familial Intrahepatic Cholestasis (PFIC). Her two siblings also had similar history. She also had difficulty in night vision due to deficiency of vitamin A, which occurs as a result of PFIC. Her vitamin deficiencies were corrected before surgery. Before surgery, she had fever for which she was evaluated and was treated with appropriate antibiotics and was taken for surgery once she was afebrile and urine and blood culture was negative. She underwent living related left lobe liver transplant on 26th June, 2014. Her aunt Sherivan was the donor. At time of surgery, she weighed 24 kg. Post operatively, her course was uneventful. At follow up, she is doing well with no complaints.

Sozan Ibrahim

Sozan Ibrahim, aged 10 years was presented with progressive jaundice, pruritus and abdominal distension started at the age of 2 1/2 years. She was evaluated for familial cholestasis as her sibling had similar history. She also had difficulty in night vision at presentation due to deficiency of vitamin A. She had severe ascites, which led to abdominal distension due to which she had difficulty in walking and breathing. She required removal of this fluid multiple times. She had severe protein deficiency leading to collection of fluid around her lungs too. Her protein and vitamin deficiencies were corrected before surgery. Before surgery, she had fever for which she was evaluated and was treated with appropriate antibiotics and was taken for surgery once she was afebrile and urine and blood culture was negative. She underwent living related left lateral segment liver transplant on 2nd July, 2014. Her uncle Nisrat was the donor. At time of surgery, she weighed 18 kg. Post operatively, she had persistently high drain output (ascitic). She had high grade fever during her post-operative period which responded to change of antibiotics. She is doing well with no complaints and has been discharged.

Ibrahim Ibrahim

Ibrahim, aged 4 years and the youngest of the three siblings, was brought with complaints of jaundice and severe itching. He developed jaundice, itching all over the body and abdominal distension which started at the age of 2 years. Jaundice was progressively increasing and associated with increased itching, loss of appetite and poor quality of life. He was evaluated for familial cholestasis as his siblings had similar history. He was diagnosed to have cirrhosis of liver due to Progressive Intrahepatic Familial Cholestasis (PFIC) Type – 3. His mother Bushra willingly agreed to donate a part of her liver and he underwent liver transplant using left lateral segment graft on 25th June 2014.

His surgery was challenging as he weighed only 12 kg at time of surgery, with small sized vessels available for anastomoses. His post-operative course remained uneventful with the exception of fever which occurred on 11th post-operative day. The cause was found to be a small intra-abdominal collection which was drained under local anaesthesia. He improved after that and was discharged and is healthy and active at follow up.

About Fortis Healthcare Limited

Fortis Healthcare Limited is a leading integrated healthcare delivery service provider in India. The healthcare verticals of the company primarily comprise hospitals, diagnostics and day care specialty facilities. Currently, the company operates its healthcare delivery services in IndiaSingaporeDubaiMauritius and Sri Lanka with 66 healthcare facilities (including projects under development), over 10,000 potential beds and approx. 280 diagnostic centres. In a global  study of the 30 most technologically advanced hospitals in the world, its flagship, the Fortis Memorial Research Institute’ (FMRI), was ranked No.2, by ‘topmastersinhealthcare.com’, and placed ahead of many other outstanding medical institutions in the world.

Article Source: https://www.prnewswire.com/in/news-releases/doctors-at-fortis-noida-perform-liver-transplants-on-three-iraqi-siblings-suffering-from-rare-genetic-disorder-269961981.html

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