100+ Pediatric Liver transplants during the COVID Pandemic at GGHC
The Institute of liver disease and transplantation at Gleneagles Global Health City (GGHC) has performed 100+ pediatric liver transplants during the pandemic, with a 100% success rate. These pediatric liver transplant surgeries were performed on babies as young as four months in age, using organs obtained from both living and cadaveric donors.
The team of doctors performed a unique procedure on a 10-year-old boy who was suffering from a rare genetic disorder known as primary hyperoxaluria type 2. To our knowledge, this was the first time this one-of-a-kind procedure was attempted in the world on a child. During this critical and highly intricate surgery, the kidney and liver of the patient were transplanted together on the same day. The kidney was donated by patient’s mother and a part of liver was donated by paternal aunt. Today, the boy is doing perfectly fine, living a normal, healthy life and attending school.
What is Primary Hyperoxaluria Type 2 (PH2)?
Primary hyperoxaluria type 2 is a rare genetic disorder. This disorder is characterized by an overproduction of oxalate (or oxalic acid). Oxalate molecules combine with calcium in the kidney to form calcium oxalate which forms kidney stones.
These calcium oxalate deposits can damage the kidneys, cause kidney failure, and also cause injury to other organs. In primary hyperoxaluria type 2, there is a deficiency of the enzyme glyoxylate reductase/hydropxypyruvate (GRHPR), which is based in the liver. The function of this enzyme is to prevent the deposition of oxalate in the body.
Findings from relevant studies show that primary hyperoxaluria type 2 has an autosomal recessive mode of inheritance. This implies that a child inherits one copy of a mutated gene from each parent.
Types of Primary Hyperoxaluria
Primary Hyperoxaluria can be of three types, each differing in severity and genetic cause.
- Primary hyperoxaluria type 1: kidney stones usually begin to appear from childhood to early adulthood. In this condition, the patient can develop end-stage renal disease at any age.
- Primary hyperoxaluria type 2: this form of the disease is similar to type 1 primary hyperoxaluria. The only difference is that the patient develops end-stage renal disease later in life.
- Primary hyperoxaluria type 3: in this form of the disease, affected individuals develop kidney stones in early childhood, but the additional signs and symptoms of the disease are unclear.
How Do We Diagnose this Rare Disease?
People with primary hyperoxaluria usually have recurrent kidney and bladder stones. The symptoms of this condition vary, with some experiencing mild to moderate or severe symptoms. Besides kidney (renal) stones, here are a few other common symptoms of primary hyperoxaluria:
- Excessive calcium deposition in the kidney
- Kidney stones that block the ureters, causing the blockade of urine flow
- Repeated and frequent urinary tract infections
- Declining kidney performance (also known as renal insufficiency)
The symptoms of this condition may manifest at a specific age or may continue to appear throughout an individual's lifetime.
The diagnosis is usually confirmed by genetic studies.
Treatment and Prognosis of Primary Hyperoxaluria Type 2
The treatment for primary hyperoxaluria type 2 is based on preventing the formation and deposition of calcium oxalate. Increasing fluid intake is the first medical advice. Administering calcium-oxalate crystallization inhibitor medications is also included in the first line of treatment. People with primary hyperoxaluria type 2 must avoid the intake of vitamin C in high doses because oxalate is formed from the degradation of ascorbic acid (or vitamin C). Foods that are naturally high in oxalates, such as chocolate, starfruit, and rhubarb, should also be avoided.
The prognosis of an individual with primary hyperoxaluria type 2 depends upon the severity of the condition at the time of diagnosis, how early the disease was diagnosed, and the treatment that was administered. For some people, medication and hydration are adequate for managing symptoms and controlling the disease. Some individuals, however, may eventually develop kidney failure. In such cases, a combined kidney and liver transplant is recommended.
For more information on Pediatric liver transplants and consultation with our expert hepatologists, contact GGHC at +91 44 44777000 today!