Surgery to remove gallstones can give rise to aHUS: Study
Teenage girl developed condition following ERCP surgery
Atypical hemolytic uremic syndrome (aHUS) can be a rare complication of a procedure to remove gallstones, according to a report in the U.S. that describes the case of a teenage girl who developed the condition secondary to post-endoscopic retrograde cholangiopancreatography (ERCP) pancreatitis.
The study, “Atypical hemolytic uremic syndrome: a rare complication of postendoscopic retrograde cholangiopancreatography pancreatitis,” was published in the Journal of Pediatric Gastroenterology and Nutrition.
Bile is a fluid produced in the liver and stored in the gallbladder that normally helps break down fats during digestion. Gallstones are solid deposits that can form in the gallbladder or bile ducts, a series of tubes that carry bile from the liver to the intestines. The largest bile duct into which all the other bile ducts converge is called the common bile duct and ERCP, where a thin tube is threaded through the bile ducts, is the primary procedure to treat gallstones that form in it. Inflammation of the pancreas, called pancreatitis, is a complication of ERCP, however.
In aHUS, blood clots form in small blood vessels and can arise following certain events, including infections, pregnancy, and autoimmune diseases.
aHUS develops after ERCP procedure
The girl, 16, was admitted to the hospital three months after giving birth due to abdominal pain, which had begun in her third trimester of pregnancy, but didn’t abate after her baby was born. Imaging of her bile ducts revealed gallstones, and she was scheduled for an ERCP.
The procedure was performed successfully, but shortly after it the girl developed pancreatitis, with abdominal pain and increased levels of pancreatic enzymes.
At the same time, lab tests showed signs of red blood cell destruction and kidney dysfunction indicative of thrombotic microangiopathy (TMA), a group of disorders marked by the formation of blood clots in small blood vessels that leads to organ damage. aHUS is a relatively rare form of TMA.
“Prompt diagnosis and treatment of TMA are crucial, as delays can cause irreversible renal failure potentially leading to death,” the researchers wrote.
A battery of diagnostic tests to look for various types of TMA led to a diagnosis of aHUS. While the diagnosis was being worked out, the girl received multiple blood transfusions and underwent plasma exchange to stabilize her condition. Once aHUS was confirmed, she started treatment with the approved therapy Soliris (eculizumab), which improved her kidney function, and was discharged from the hospital shortly thereafter.
The girl switched a few months later to Ultomiris (ravulizumab-cwvz), another approved aHUS therapy that works the same way as Soliris, but is administered less often. So far, her kidney function has remained normal and she hasn’t shown signs of red blood cell destruction.
The researchers said their report shows aHUS may be a rare complication of post-ERCP pancreatitis that clinicians should be aware of, but noted that the specific mechanisms that lead to the onset of aHUS in that context still aren’t fully understood.
“We intend to bring awareness among the readers about this rare complication that can lead to devastating patient outcomes without early recognition and appropriate management,” the researchers wrote.
Soliris and Utlomiris are both marketed by Alexion, AstraZeneca Rare Disease, which wasn’t involved in this study.
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