THE HELLP SYNDROME, A PROSPECTIVE STUDY<sup>*</sup>

Abraham, Kottarathil A.; Connolly, Geraldine; Farrell, John; Walshe, J. Joseph

doi:10.1081/jdi-100107367

Cited by 35 publications

(23 citation statements)

References 18 publications

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“…However, acute renal dysfunction is frequent in HELLP syndrome, reported in a minimum of 3% to 8% of patients and up to 36% to 53% of cases. [20][21][22][23] Moreover, the patients included in our study had clinical and biologic features, including liver involvement, similar to those previously reported in patients with HELLP syndrome. 24,25 Finally, in contrast to the HELLP syndrome, liver involvement, a hallmark of HELLP syndrome, is uncommon in patients with aHUS.…”

Section: Clinical Characteristic Of Patients With Hellp Syndrome and supporting

confidence: 68%

Factor H, membrane cofactor protein, and factor I mutations in patients with hemolysis, elevated liver enzymes, and low platelet count syndrome

Fakhouri

Jablonski

Lepercq

et al. 2008

Blood

111

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The HELLP syndrome, defined by the existence of hemolysis, elevated liver enzymes, and low platelet count, is a serious complication of pregnancy-related hypertensive disorders and shares several clinical and biologic features with thrombotic microangiopathy (TMA). Several recent studies have clearly shown that an abnormal control of the complement alternative pathway is a major risk for the occurrence of a peculiar type of TMA involving mainly the kidney. The aim of this study was to screen for complement abnormalities in 11 patients with HELLP syndrome and renal involvement. We identified 4 patients with a mutation in one of the genes coding for proteins involved in the regulation of the alternative pathway of complement. Our results suggest that an abnormal control of the complement alternative pathway is a risk factor for the occurrence of HELLP syndrome. (Blood. 2008;112:4542-4545)

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Section: Clinical Characteristic Of Patients With Hellp Syndrome and supporting

confidence: 68%

Factor H, membrane cofactor protein, and factor I mutations in patients with hemolysis, elevated liver enzymes, and low platelet count syndrome

Fakhouri

Jablonski

Lepercq

et al. 2008

Blood

111

View full text Add to dashboard Cite

show abstract

“…[55577677] Dialysis is needed in approximately 10%–46% of patients with HELLP syndrome associated AKI during acute phase. However, even dialysis-requiring AKI has excellent prognosis with complete recovery of renal function in almost all cases.…”

Section: Causes Of Acute Kidney Injury In Pregnancy With Respect To Tmentioning

confidence: 99%

Acute kidney injury in pregnancy-specific disorders

Prakash

Ganiger

2017

Indian J Nephrol

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The incidence of acute kidney injury in pregnancy (P-AKI) has declined significantly over the last three decades in developing countries. However, it is still associated with significant fetomaternal mortality and morbidity. The diagnosis of P-AKI is based on the serum creatinine increase. The usual formulas for estimating glomerular filtration rate (GFR) are not validated in this population. The incidence of P-AKI with respect to total AKI cases has decreased in the last three decades from 25% in 1980s to 9% in 2000s at our centre. During the first trimester of gestation, AKI develops most often due to septic abortion or hyperemesis gravidarum. Septic abortion related AKI with respect to total AKI decreased from 9% to 5% in our study. Prevention of unwanted pregnancy and avoidance of septic abortion are keys to eliminate abortion associated AKI in early pregnancy. However, we have not seen AKI on account of hyperemesis gravidarum over a period of 33 years at our center. In the third trimester, the differential diagnosis of AKI in association with pregnancy specific conditions namely preeclampsia/HELLP syndrome, acute fatty liver of pregnancy and thrombotic microangiopathies of pregnancy (P-TMA) is more challenging, because these 3 conditions share several clinical features of thrombotic microangiopathy which makes the diagnosis very difficult on clinical grounds. It is imperative to distinguish these conditions to make appropriate therapeutic decisions. Typically, AFLP and HELLP syndrome improve after delivery of the fetus, whereas plasma exchange is the first-line treatment for pregnancy associated thrombotic microangioathies (P-TMA). We observed that preclampsia/eclampsia is the most common cause of AKI in late third trimester and postpartum periods followed by puerperal sepsis and postpartum hemorrhage. Pregnancy-associated thrombotic microangiopathies (aHUS/TTP) and AFLP are rare causes of AKI during pregnancy in developing countries.

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“…AKI occurs in 3%-15% of cases of HELLP syndrome, and HELLP syndrome accounts for 40% of all cases of P-AKI and up to 60% of severe cases (13,(63)(64)(65). AKI associated with HELLP syndrome, even in its severe forms requiring dialysis, usually retains a favorable renal outcome.…”

Section: Tma In Pregnancy: the Great Paradigm Shiftmentioning

confidence: 99%

Obstetric Nephrology

Fakhouri

Vercel

Frémeaux‐Bacchi

2012

Clinical Journal of the American Society of Nephrology

145

121

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Summary AKI in pregnancy remains a cause of significant fetomaternal mortality and morbidity, particularly in developing countries. Hypertensive complications of pregnancy (preeclampsia/eclampsia or hemolysis, elevated liver enzymes, and low platelets count syndrome) are the leading cause of AKI in pregnancy worldwide. Thrombotic microangiopathy is another peculiar and devastating cause of AKI in pregnancy. During the last decade, our understanding, and in some cases, our management, of these causes of AKI in pregnancy has dramatically improved. For instance, convincing data have linked pre-eclampsia/eclampsia to an increase in circulating antiangiogenic factors soluble Flt 1 and endoglin, which induce endothelial cell dysfunction, hypertension, and proteinuria. Several distinct pathogenic mechanisms underlying thrombotic microangiopathy, including thrombotic microangiopathy occurring during pregnancy, have been established. Thrombotic microangiopathy, which can present as hemolytic uremic syndrome or thrombotic thrombocytopenic purpura, can be reclassified in four potentially overlapping subtypes: disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 deficiency-related thrombotic microangiopathy, complement alternative pathway dysregulation-related thrombotic microangiopathy, secondary thrombotic microangiopathy (verotoxin and antiangiogenic drugs), and thrombotic microangiopathy of undetermined mechanism. In most cases, pregnancy is only a precipitating factor for thrombotic microangiopathy. Treatment of thrombotic microangiopathy occurring during pregnancy should be tailored to the underlying pathogenic mechanism: (1) restoration of a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 serum activity in the setting of thrombotic thrombocytopenic purpura through plasma exchanges and in some cases, B cell-depleting therapy and (2) inhibition of complement alternative pathway activation in atypical hemolytic uremic syndrome using antiC5 blocking antibody (eculizumab).

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THE HELLP SYNDROME, A PROSPECTIVE STUDY^*

Abstract: HELLP syndrome is a rare but potentially serious complication of pregnancy. Correlation with laboratory data and early intervention are vital in achieving a favorable outcome for both mother and fetus.

Cited by 35 publications

References 18 publications

Factor H, membrane cofactor protein, and factor I mutations in patients with hemolysis, elevated liver enzymes, and low platelet count syndrome

Factor H, membrane cofactor protein, and factor I mutations in patients with hemolysis, elevated liver enzymes, and low platelet count syndrome

Acute kidney injury in pregnancy-specific disorders

Obstetric Nephrology

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THE HELLP SYNDROME, A PROSPECTIVE STUDY*

Abstract: HELLP syndrome is a rare but potentially serious complication of pregnancy. Correlation with laboratory data and early intervention are vital in achieving a favorable outcome for both mother and fetus.

Cited by 35 publications

References 18 publications

Factor H, membrane cofactor protein, and factor I mutations in patients with hemolysis, elevated liver enzymes, and low platelet count syndrome

Factor H, membrane cofactor protein, and factor I mutations in patients with hemolysis, elevated liver enzymes, and low platelet count syndrome

Acute kidney injury in pregnancy-specific disorders

Obstetric Nephrology

Contact Info

Product

Resources

About

THE HELLP SYNDROME, A PROSPECTIVE STUDY^*