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What are cholecystitis and PNH?

Q: What are cholecystitis and PNH? I am suffering from both. As PNH is a blood-related disorder, is it risky to have a gall bladder operation (laparoscopy)?

A:Paroxysmal nocturnal haemoglobinuria (PNH) is a disease caused by a defect in the bone marrow stem cells causing a membrane abnormality in blood cells. It is characterised by red cell breakdown (haemolysis) resulting in the release of haemoglobin into the urine imparting a dark colour to it. The term ‘nocturnal’ and ‘paroxysmal’ are imprecise as haemolysis occurs throughout the day but the urine concentrated overnight produces the prominently dark-coloured urine in the morning. The disease is due to a defect in the haematopoietic stem cell which results in lack of certain surface proteins in the membranes of red blood cells, white blood cells and platelets caused by a genetic mutation. The patient may present with a haemolytic anaemia, thrombotic episodes in large veins or bone marrow suppression causing pancytopaenia (i.e. anaemia, decrease in white cells [leukopaenia] and reduced platelets [thrombocytopaenia]). The condition is insidious and follows a chronic course with morbidity depending on the degree of haemolysis, thrombophilia and bone marrow failure. Gallstones are a common problem in people with PNH due to prolonged and recurrent red cell breakdown resulting in increased bilirubin levels in the bile, which is stored in the gall bladder. The excess bilirubin can precipitate and form bilirubin stones. Gallstones can become a problem when they block the flow of bile into the intestines. Symptoms of a gall bladder attack include severe abdominal pain, especially on the right side of the abdomen, fever, nausea, vomiting, and chills. The preferred surgery for gall stones is laparoscopic unless there are contraindications to it. It is the anaesthetic risk in these patients which requires attention. Preoperative treatment of precipitating factors, such as sepsis, avoidance of drugs and techniques activating the complement, and prevention of thrombotic episodes are necessary. Nitrous oxide is generally avoided in those with hypoplastic anaemia, especially if liver function is deranged. The use of volatile anaesthetics for induction is preferable to IV anaesthetics because of their association with a high frequency of anaphylactoid reactions. Haemoglobinuria can occur after the induction of anaesthesia in a proportion of patients. Maintenance of adequate hydration is important in preventing thrombotic episodes during surgery and postoperative use of heparin is advocated by some. In case of blood transfusion, fresh packed cells or saline-washed cells are used. Please discuss with your doctor as he would be best placed to advise you.

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