Alloveda Liver Update: A case report of HELLP syndrome in a 26-year-old woman with cystic fibrosis |
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Alloveda Liver Update: A case report of HELLP syndrome in a 26-year-old woman with cystic fibrosis

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The microangiopathic hemolysis, elevated liver enzymes, and low platelet(HELLP) syndrome is a potentially fatal complication of pregnancy. It has arare occurrence and the current study reports a case of HELLP syndrome in a 26-year-oldwoman with mild cystic fibrosis bronchiectasis in the third trimester ofpregnancy The woman was affected with the triad of microangiopathic hemolysis,elevated liver enzymes, and low platelet count. Her baby was delivered successfully after a semi-elective caesareansection.

 Although pregnancies in patients with cystic fibrosis arecorrelated to an increased rate of complication, the reported case is highlyimportant as this for the second time thesuccessful delivery of a baby occurred in a women with cystic fibrosis. Thispregnancy became a challenge because of the presence of microangiopathichemolysis, elevated liver enzymes, and low platelet syndrome. 

HELLP syndrome is known to complicate approximately one to two in every1000 pregnancies. Although certain researches consider this a severe variant ofthe pre-eclamptic spectrum, in few cases, it occurs in absence of otherpathological hallmarks of the disease, as in this case HELLP was presentwithout proteinuria. The pathophysiology of pre-eclampsia is yet controversial,it is supposed to arise as a result of deficient placentation, followed by theliberation of vasoactive mediators and subsequent maternal endothelialdysfunction. Risk factors for the development of HELLP syndrome include multiparity,age over 25years,Caucasian ethnicity, and a history of poor pregnancy outcome. 

The main line treatment for both pre-eclampsia and HELLP syndrome isdelivery of the baby, and plethora of evidence supports the expedition ofdelivery, if the pregnancy is at or beyond 34weeks of gestation and particularly. Although thepresence of cystic fibrosis and HELLP syndrome are not directly associated toeach other in this case, the diagnosis of HELLP syndrome was obscured.Moreover, individuals with cystic fibrosis commonly develop abnormal liverfunction tests when unwell or dehydrated due to biliary sludging. The patientstarted making a recovery before acutely deteriorating, which confirmed thediagnosis.

Although evidences demonstrated that were no adverse maternal outcomesfor patients with relatively preserved lung function (FEV1>60%), in regard to cystic fibrosis lung disease andpregnancy, rates of prematurity would appear to be increased. The manifestationof HELLP syndrome insignificantly affected included patient’s lung functionnegatively.  Moreover, the outcome was favorable in the case of thepatient used in the study, however, cystic fibrosis increased complexity toacute management as her lung function had deteriorated. In addition, people withcystic fibrosis are more at risk during general anesthetic procedures,specifically if a peri-operative inability to clear airway secretions isenhanced by post-operative abdominal pain.

The main differential diagnoses in this case were thrombotic thrombocytopenicpurpura and the hemolytic uremic syndrome. Nevertheless, the presence ofhypertension before the development of severe microangiopathic hemolysis andplatelet destruction, along with absence of any associated renal dysfunction,prompt resolution of clinical and biochemical derangements upon delivery of thebaby rule out other diagnoses.

Source: Geake J, Dabscheck E, Reid D. Hemolysis, elevated liver enzymes,and low platelet (HELLP) syndrome in a 26-year-old woman with cystic fibrosis:a case report. J Med Case Rep. 2012 May 23;6:134. 

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