Clinical Presentation, Management and Materno-Fetal Outcome of Patients with HELLP Syndrome at the Yaoundé Gyneco-Obstetrics and Pediatric Hospital

Background: HELLP (Hemolysis, Elevated Liver enzymes and Low Platelets) syndrome is a common complication of severe preeclampsia, with a high maternal and perinatal mortality rate. Data on HELLP syndrome is scanty in our setting. We sought to study the clinical presentation, management, and materno-fetal outcome of patients with HELLP syndrome at the Yaoundé Gyneco-Obstetrics and Pediatric Hospital (YGOPH). Methods: This was a cross-sectional, analytical study with a retrospective data collection of patients, diagnosed with HELLP syndrome at the YGOPH between 1st January 2020 and 31st July 2021. Data were analyzed using the software R version 4.0.2. Results are presented as mean ± standard deviation, frequencies and percentages. P values < 0.05 were considered statistically significant. Results: Of 254 cases of severe preeclampsia, 17 developed HELLP syndrome, giving us a frequency of 6.7%. One patient who presented with hepatitis B was excluded. The mean age was 27.19 ± 6.44 years. Most patients (56.3%) had poor follow up during antenatal contacts. The most common symptom was headache (93.8%). Most cases (56.3%) were diagnosed during post-partum, prepartum (25.0%), and per partum (18.8%). Obstetrical management was done by cesarean section (62.5%) and vaginal delivery (37.5%). Post-partum management of HELLP syndrome consisted of antihypertensive medication, anticonvulsants, sedatives, transfusion of blood and blood products, and fluid resuscitation. Of the 50% of patients who presented with acute kidney injury, only 12.5% (1) were referred for dialysis. Over 50% of our study participants presented severe complications after delivery, but 43.8% recovered, while 56.2% finally died. The fetal outcome was: still birth (31.2%), intra-uterine growth restriction/low birth weight (12.5%), term delivery (≥37 weeks) (31.3%), and preterm deliveries (<37 weeks) (68.7%). Three (18.8%) of the neonates delivered alive presented with a poor Apgar score at the 5th minute. Conclusion: Although rare, HELLP syndrome exists in our setting. Proper management in appropriate centers is key to improving maternal and fetal outcomes.

A Typical Case of Posterior Reversible Encephalopathy Syndrome Associated with Postpartum Eclampsia-HELLP Syndrome

Background: Posterior reversible encephalopathy syndrome (PRES) is a reversible syndrome characterized by seizures, headache, altered mentation, and loss of vision associated with white matter changes on imaging. Case: A 37-year-old multigravida woman had a severe, immediate postpartum eclampsia-HELLP syndrome (hemolysis, elevated liver enzyme levels, low platelet count) with PRES characterized by generalized seizures and altered mental status. Magnetic resonance brain imaging showed high-intensity lesions in non-posterior portions including the frontal lobe and cingulated gyrus, which resolved completely after 2 weeks along with complete symptom regression. Conclusions: Cases of postpartum PRES without involvement of posterior brain regions after eclampsia-HELLP syndrome are very rare. Patients with PRES do not always show typical manifestations. The importance of a prompt diagnosis is emphasized, as is the crucial role of rapid blood pressure reduction.

Management of a Parturient with Preeclampsia and HELLP Syndrome Complicated by Gestational Diabetes Insipidus

HELLP syndrome (Hemolysis, Elevated Liver enzymes, Low Platelets) is considered to be a variant or severe form of pre-eclampsia, a life threatening complication of pregnancy. Gestational Diabetes Insipidus (GDI) can coexist with severe preeclampsia and HELLP syndrome. The combination of these two conditions presents a unique challenge to the anesthesiologist and the obstetric team, caring for this parturient. We present the case of a parturient with an unusual presentation of GDI, coexisting with severe preeclampsia and HELLP syndrome. She had two days history of polyuria and polydipsia as well as lethargy and rapidly rising serum sodium in addition to acute renal failure without any neurologic symptoms. Expeditious delivery of the baby and supportive management is essential for optimal outcomes. She underwent a repeat Cesarean section under combined spinal epidural (CSE) anesthesia. This patient was discharged on postoperative day five after clinical resolution of her signs and symptoms.

Pregnant-Postpartum Women with Severe Epigastric Pain and HELLP Syndrome: A Highly Dangerous Combination

OBJECTIVE: HELLP syndrome is a severe preeclampsia spectrum disorder diagnosed when laboratory evidence of hemolysis, liver dysfunction and thrombocytopenia are present. The presence of epigastric pain with laboratory criteria for HELLP syndrome may indicate higher maternal-fetal risk. Thus we explored maternal outcomes in 42 pregnant/postpartum women that had HELLP syndrome by laboratory criteria in addition to sudden severe epigastric pain. METHODS: A database was constructed from the medical files of all patients with medical/hypertensive disorders evaluated by the first author from 1986-2015 for medicolegal purposes. All patient files of women who presented to their physicians with a diagnosis of presumptive HELLP syndrome were examined. RESULTS: Fifty-eight pregnant/postpartum women fulfilled study inclusion criteria;they presented to physicians for evaluation usually in non-tertiary care hospital settings. Clinical presentation and care including lab data and details of pregnancy outcome were evaluated. A correct diagnosis of HELLP syndrome was verified for 42 women (72.4%);others were determined to have AFLP-Acute Fatty Liver of Pregnancy (n = 8) or TTP-aHUS-Thrombotic thrombocytopenic purpura-adult/atypical hemolytic uremic syndrome (n = 8). All 42 HELLP women (100%) in our cohort had severe epigastric pain. Treatment modalities for maternal HELLP syndrome included magnesium sulfate (n = 35, 83%), antihypertensives (n = 17, 40%);corticosteroids (CORT) for HELLP were not utilized. Major maternal morbidity (21 strokes, 9 liver ruptures/hematomas) affected 41 HELLP women (98%);22 died, 7 were permanently disabled. CONCLUSION: HELLP syndrome patients presenting with sudden, severe epigastric pain in this highly selected cohort experienced very high maternal morbidity and mortality. Laboratory evidence of HELLP syndrome in association with epigastric pain is a dangerous combination that portends great danger to safe motherhood. We speculate that the absence of CORT use in this cohort contributed to poor maternal outcome (word count = 280 as modified for reviewers).

Clinical diagnosis and treatment of “atypical” HELLP syndrome

HELLP syndrome is regarded as one of the most serious manifestations of preeclampsia,but approximately 15%of patients with HELLP syndrome have no clinical manifestations of preeclampsia;furthermore,although most cases of HELLP syndrome occur after 34 weeks,some cases occur at a much earlier gestational stage,even before 20 weeks of gestation,the underlying pathogenesis of“atypical”HELLP syndrome with very early onset or the basis of non-preeclampsia manifestations should be more actively explored.Obstetricians should carefully identify the potential etiology of“atypical”HELLP syndrome in order to provide a reasonable treatment and improve maternal and fetal prognosis.

Acute fatty liver of pregnancy:An update on pathogenesis and clinical implications

Acute fatty liver of pregnancy (AFLP) is a serious maternal illness occurring in the third trimester of pregnancy with significant perinatal and maternal mortality. Till recently, it has been considered a mysterious illness. In this editorial, we review the recent advances in understanding the pathogenesis of AFLP and discuss the studies documenting a fetal-maternal interaction with a causative association between carrying a fetus with a defect in mitochondrial fatty acid oxidation and development of AFLP. Further, we discuss the impact of these recent advances on the offspring born to women who develop AFLP, such that screening for a genetic defect can be life saving to the newborn and would allow genetic counseling in subsequent pregnancies. The molecular basis and underlying mechanism for this unique fetal-maternal interaction causing maternal liver disease is discussed.

Investigation of diagnosis and treatment of hemolysis-elevated liver enzymes-low platelet counts （HELLP） syndrome： clinical analysis of 59 cases

Background Hemolysis-elevated liver enzymes-low platelet counts （HELLP） syndrome is a clinical condition occurring in middle and late stage pregnancy. It is characterized by hemolysis, elevated liver enzymes and low platelet counts. This study involves the analysis of the diagnosis, clinical characteristics and treatment of 59 cases of HELLP syndrome as well as the clinical classification, method of delivery and gestational age at delivery. Methods Clinical data from 59 cases of HELLP syndrome occurring from January 2000 to December 2009 were analyzed retrospectively. Thirty-five cases were classified as complete HELLP syndrome and 24 cases were considered partial HELLP syndrome. Results Twenty-six of the 59 analyzed patients （44%） with complete HELLP syndrome showed rapid onset, severe signs, symptoms, and complications in addition to a poor clinical outcome. Complications included multiple organ dysfunction syndrome （MODS） occurring in 18 cases, eclampsia （3 cases）, placental abruption （3 cases）, and perinatal death （4 cases）. The remaining 33 cases （24 with partial and 9 with complete HELLP） were characterized by less severe signs, symptoms, complications and progression of the condition. Two of these cases were complicated with MODS （6.1%）, and 1 with perinatal death （3.0%）.Twelve non-radical-type cases received conservative treatment. The remaining 4 patients had recurring HELLP syndrome （6.78%）. Conclusions HELLP syndrome is classified as the radical type and non-radical-type according to clinical characteristics and outcome. Classification of HELLP syndrome cases according to clinical features can help in the monitoring and treatment of the disease. Active termination of pregnancy should be considered for radical-type cases. Non-radical-type cases can undergo conservative treatment with close monitoring in an attempt to improve perinatal outcome without increasing maternal morbidity.

Diagnostic Approach of Thrombocytopenia in Pregnancy: A Review

Thrombocytopenia (defined as platelet count 9/L) is present in 7% - 12% of pregnant women at delivery. Although there are mild etiologies of this condition that are often diagnosed incidentally, there are more severe causes that can be life threating. Thrombocytopenia also has a great implication in surgical risk and regional anesthesia. A structured evaluation of thrombocytopenia is necessary to allow an adequate diagnostic approach. Here we summarized the current knowledge of thrombocytopenia in pregnancy.

A case of aggravation of hemolytic anemia, elevated liver enzymes and low platelet count syndrome after delivery

Background Hemolytic anemia, elevated liver enzymes and low platelet count （HELLP） syndrome is a severe obstetric complication which usually resolves in most patients after delivery.Methods We report a rare case of aggravation of HELLP syndrome after delivery.Results The patient underwent the treatment for HELLP syndrome,.including glucocorticoid therapy. The symptoms of HELLP syndrome reappeared and became more severe than before the termination of pregnancy. The patient also had severe and persistent hypoproteinemia, hyponatremia and hypocalcemia.Conclusions HELLP syndrome is an acute and critical obstetric syndrome which can have heterogeneous presentations and variable prognosis. We should be fully aware of the diverse clinical characteristics of this condition.

Thrombotic Thrombocytopenic Purpura in a Parturient Leading to Life-Threatening Thrombocytopenia and Neonatal Demise—A Case Report

Thrombocytopenia is common (up to 12%) in pregnancy;thrombotic thrombocytopenia is a rare cause of thrombocytopenia;however,it is immediately life-threatening to both mother and fetus and requires immediate intervention.This is compounded by the need to act on a presumptive diagnosis with high level of suspicion given the relatively long laboratory confirmation time.A 31-year-old gravida 3,para 1 parturient at 26 weeks of gestation presented to outside hospital with recent onset of easy bruising and blurry vision.A blood count was drawn and showed a platelet count of 19,000/μL.She was transferred to our institution for management where an initial diagnosis of preeclampsia with severe features was made based on thrombocytopenia and fetal growth restriction (<1%).Platelet count nadired at 14,000/μL and a blood smear showed schistocytes,suggesting microangiopathic hemolysis,prompting discussion of urgent cesarean delivery and anesthesia consult.An urgent hematology consult led to presumptively diagnosing thrombotic thrombocytopenic purpura and cesarean delivery was deferred after discussion with the patient and team.Plasma exchange and steroid therapy were started promptly,and her platelet count improved within 12 hours.Unfortunately,the patient experienced neonatal demise.Undetectable ADAMTS13 levels confirmed diagnosis of thrombotic thrombocytopenia.She was transitioned to rituximab,platelets recovered to baseline,and she continues to do well.Thrombotic thrombocytopenia is a rare,life-threatening cause of thrombocytopenia in pregnancy.Despite grim fetal prognosis,especially in early pregnancy,low threshold of suspicion,early anesthesia involvement,and multi-disciplinary approach can facilitate diagnosis and timely intervention.In our case,it was likely lifesaving.

Imaging of pregnancy-related liver diseases

Pregnancy-related liver diseases are the most frequent cause of liver dysfunction in pregnancy and pose a real threat to fetal and maternal survival.Although hepatobiliary disorders are uncommon in pregnancy,they may be severe enough to cause risk to both the mother and fetus.There are five different types of liver disease that are unique to pregnancy,including hyperemesis gravidarum,intrahepatic cholestasis of pregnancy,preeclampsia,hemolysis,elevated liver enzyme,and low platelet count syndrome,and acute fatty liver of pregnancy.Ultrasound,computed tomography,and magnetic resonance imaging play important roles in the rapid diagnosis and evaluation of complications.An accurate diagnosis can guide further treatment or the induction of labor to prevent intrauterine fetal death.This review highlights the pathogenesis,clinical features,imaging features,and treatment of pregnancy-related liver diseases with the aim of enhancing awareness of these rare but potentially life-threatening diseases of pregnancy.