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Table of Contents
CLINICAL ARTICLE
Year : 2022  |  Volume : 23  |  Issue : 1  |  Page : 12-17

HELLP syndrome in pregnancy


Associate Professor, Department of Obstetrical and Gynaecological Nursing, College of Nursing, CIHSR, Dimapur, Nagaland, India

Date of Submission02-Feb-2022
Date of Decision17-May-2022
Date of Acceptance19-May-2022
Date of Web Publication05-Jul-2022

Correspondence Address:
Ms. Zuchanbeni Humtsoe
Associate Professor, Department of Obstetrical and Gynaecological Nursing, College of Nursing, CIHSR, Dimapur, Nagaland
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijcn.ijcn_6_22

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  Abstract 

HELLP is an acronym that refers to a syndrome characterised by haemolysis, elevated liver enzymes and a low platelet (HELLP) count. It is a rare and life-threatening condition that occurs during pregnancy. The most common symptoms of HELLP syndrome include headache, nausea, vomiting, upper right abdominal pain, tenderness and fatigue. Most symptoms and side effects subside within 2–3 days of delivery. Early identification and treatment is essential to prevent complications of HELLP syndrome.

Keywords: Eclampsia, haemolysis, elevated liver enzymes, and a low platelet syndrome, pre-eclampsia, pregnancy


How to cite this article:
Humtsoe Z. HELLP syndrome in pregnancy. Indian J Cont Nsg Edn 2022;23:12-7

How to cite this URL:
Humtsoe Z. HELLP syndrome in pregnancy. Indian J Cont Nsg Edn [serial online] 2022 [cited 2022 Aug 16];23:12-7. Available from: https://www.ijcne.org/text.asp?2022/23/1/12/349823


  Introduction Top


Haemolysis, elevated liver enzymes and a low platelet (HELLP) syndrome is a group of symptoms that occur in pregnant women who have: H-haemolysis (the breakdown of red blood cells [RBC]), EL-elevated liver enzymes and LP-low platelet count.[1] HELLP syndrome is a rare and life-threatening condition that occurs during pregnancy. It is a complication of the severe form of pre-eclampsia. Sometimes, HELLP may be a separate disorder from pre-eclampsia.[2] HELLP develops in 0.1%–1.0% of pregnant women. HELLP syndrome occurs in about 1–2 out of 1000 pregnancies. In women with pre-eclampsia or eclampsia, the condition develops in 10%–20% of pregnancies.[1] In HELLP syndrome, the diagnostic evaluation shows perinatal mortality close to 10%–60% and maternal mortality in 1.5%–5% of the situations.[3] The most common symptoms of HELLP syndrome include headaches, nausea and vomiting that continues to get worse, upper right abdominal pain or tenderness and fatigue or malaise. Treatment of HELLP syndrome is primarily based on the gestation of the pregnancy, but the delivery of the baby is the best way to stop this condition from causing any serious complications to both mother and baby. Most symptoms and side effects subside within 2–3 days of delivery.[4] Studies suggest that in about 70% of the cases, the HELLP syndrome develops antenatally with a peak frequency between the 27th and 37th gestational weeks; 10% occur before the 27th week and 20% beyond the 37th gestational week.[5] Early identification and treatment is essential to prevent complications of HELLP syndrome.


  Case Report Top


A case study of 40-year-old woman (Mrs. A) (G2PL1) at 33 weeks of gestation with HELLP syndrome is discussed with prior permission from her. She presented to the emergency department with a complaint of severe epigastric pain for about 8 h which did not subside by taking antacids. She did not have a history of per vaginal leaking or bleeding, no headache, vomiting, seizure or blurring of vision. There was no history of gestational diabetes, renal condition, jaundice, headache or fever. She developed hypertension (HTN) at around 3–4 months of pregnancy and was on antihypertensive drugs. She had a similar history of HTN in the first pregnancy which also started at around 3–4 months of pregnancy and subsided after delivery. There is no family history of HTN/pre-eclampsia.

At the time of her presentation to the emergency department, she was conscious and oriented. Her blood pressure (BP) was 230/140 mmHg, pulse – 90/min, respiration – 24/min, temperature – 98.5 F, oxygen saturation (SPO2) – 98% and foetal heart sound – 152/min. On abdominal assessment, there was an absence of uterine contraction.

The risk factors of the patient are outlined along with the evidence from literature[6] in [Table 1].
Table 1: Comparison of risk factors of haemolysis, elevated liver enzymes and a low platelet count syndrome

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Studies suggest the instance of pre-eclampsia increases by 4% each year for every year over the age of 32. Other risk factors for HELLP syndrome include chronic HTN, smoking, assisted reproduction and multiple pregnancies.[6]

Pathophysiology

The pathophysiology of HELLP syndrome is ill-defined and includes many theories.[7],[8],[9] Some theorise that, because HELLP is a variant of pre-eclampsia, the pathophysiology stems from a common source. In pre-eclampsia, there is the presence of severe HTN, proteinuria and generalised oedema [Figure 1].
Figure 1: Pathophysiology of HELLP syndrome

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Laboratory investigations

Investigations for this patient included analysis of complete blood count and serum creatinine. The liver function test (LFT) as serum glutamic-oxaloacetic transaminase (SGOT), serum glutamic-pyruvic transaminase (SGPT), total bilirubin (BIL), prothrombin time, international normalised ratio (INR) and activated partial thromboplastin time. Urine analysis revealed RBC, pus cells, fine and coarse granular cast and blood in urine, protein and glucose. Blood and urine investigations were continued over the next few days. Ultrasonography revealed a single intrauterine live foetus with severe intrauterine growth retardation (IUGR). Electrocardiogram (ECG) and echocardiograph showed normal findings.

Since the BP was very high, and the LFT values were deranged, an emergency caesarean section was opted.

Diagnostic criteria of haemolysis, elevated liver enzymes and a low platelet syndrome

HELLP syndrome was considered when any one of the following was present.

  1. Haemolysis: BIL ≥1.2 mg/dl, haemoglobin slightly decreased, lactate dehydrogenase >600 U/L, broken RBC on blood smear
  2. Elevated liver enzymes: Alanine aminotransferase (ALT) ≥40 U/L or aspartate aminotransferase (AST) ≥70 U/L;
  3. Decreased platelets (PLT):

    PLT < 50×109 /L- Type I

    PLT 50-100 ×109 /L - Type II

    PLT 101-150×109 /L - Type III


Partial abnormality of the above three indicators was defined as partial HELLP syndrome, and all abnormality was defined as complete HELLP syndrome.[10] The confirmation diagnosis of HELLP syndrome is based upon the presence of all of the laboratory abnormalities comprising its name (haemolysis, elevated liver enzymes and low platelet count) in a pregnant/post-partum patient.[5]

Clinical presentation

The patient in the case report had the following laboratory values: total BIL – 1.9 mg/dl, AST/SGOT – 963 IU/L, ALT/SGPT – 680 IU/L and platelet – 114,000/mm3 which dropped to 52,000/mm3 within 12 h and therefore was diagnosed to have HELLP syndrome.

HELLP syndrome is a type of pre-eclampsia causing the elevation in liver enzymes and low platelet count.[11] Majority of the clinical symptoms are right upper abdominal quadrant or epigastric pain, nausea and vomiting. The upper abdominal pain may be fluctuating and is colic-like. Many patients report a history of malaise some days before presentation. Up to 30%–60% of women have headache; about 20% have visual symptoms.[5] The comparison of the patient along with the book picture is outlined in [Table 2].
Table 2: Comparison of clinical presentation of haemolysis, elevated liver enzymes and a low platelet count syndrome

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Management

The management of HELLP includes stabilising the woman and assessing foetal well-being. Due to the potential for severe maternal complications, which can develop rapidly, patients with HELLP are managed at a tertiary care centre with appropriate levels of maternal and neonatal intensive care. Delivery of the baby results in the resolution of HELLP syndrome while early identification and treatment is essential to prevent complications of HELLP syndrome.[11] Patients with HELLP syndrome should be treated prophylactically with magnesium sulphate to prevent seizures. Patients with HELLP syndrome should be routinely treated with corticosteroids. A high dose (10 mg) of intravenous dexamethasone every 12 h is reported to improve the laboratory abnormalities associated with HELLP syndrome.[12] Nowadays, betamethasone, instead of dexamethasone, has been recommended as a drug of choice for the promotion of foetal lung maturation in threatening preterm delivery.[13]

For Mrs. A, the treatment plan included Zuspan regimen of administration of MgSO4, followed by antihypertensives such as tablet Nicardia 10 mg, tablet labetalol 100 mg and tablet Minipress 1.5 mg. Injection of betamethasone 12 mg stat, injection of calcium gluconate 10 ml stat and injection of oxytocin 10 IU at the rate of 30d/m were administered. Eight units of fresh frozen plasma (FFP), 9 units of platelet and 3 units of whole blood were transfused. The woman underwent emergency caesarean section and a female baby weighing 1.3 kg was delivered with an Apgar score of 6/10 at 1 min.

As per the Zuspan regimen, magnesium sulphate was administered to prevent seizures and betamethasone was administered to improve outcomes. Along with the antihypertensive drugs, steroids (injection betamethasone 12 mg stat) were given as gestation was only 33 weeks. She was also started on antibiotics (Injection ceftriaxone 1 g and Injection Cefoperazone + Sulbactam 1.5 g).

Zuspan Regimen for administration of MgSO4

  1. Loading dose: MgSO4 4 g in 100 ml normal saline over 20 m
  2. Maintenance dose: MgSO4 12 g in 60 ml normal saline at 5 ml/h for 12 h.


She had reduced urine output (438 ml in 12 h) which reduced to 5 ml (<0.5 mg/kg) in 1 h after starting MgSO4. Injection of calcium gluconate 10 ml was administered as STAT dose and MgSO4 was stopped. Foley's catheterisation was done. Intravenous fluids such as Ringer's lactate at 50 ml/h were given. The urine output improved gradually.

In the emergency unit, after LSCS, to prevent post-partum haemorrhage, blood and blood components were transferred as replacement within 12 h. Carboxymethylcellulose ointment was instilled for complaints of blurring vision and photophobia. Haemodynamic stability was achieved with the help of consultation and advice by physicians from general medicine.

Prognosis

Patient with pregnancies complicated by pre-eclampsia, growth restriction, preterm delivery, abruptio placentae and stillbirth are at increased risk of recurrence, risk for related obstetric complications in future pregnancies and risk for cardiovascular and renal disease in later life. The patient underwent an emergency caesarean section and delivered a preterm baby weighing 1.3 kg. Her condition gradually improved after the delivery of the baby and she got discharged on 7th day in stable condition.

Complications

In women with post-partum HELLP syndrome, risk of renal failure and pulmonary oedema is significantly increased compared to those with an antenatal onset.[14] Rarely, HELLP syndrome can be complicated by the development of hepatic infarction as well as subcapsular haematoma or hepatic rupture. These potentially fatal conditions each manifest with severe right upper quadrant abdominal pain and marked elevations in serum aminotransferases.[15]

Nursing management


  1. Nursing Diagnosis Top


Risk for maternal injury related to potential organ damage secondary to HELLP syndrome.

Expected outcome

The patient will maintain safety and be able to actively participate in safeguarding herself and her foetus in the treatment.

Intervention

  • Patient level of consciousness was assessed using GCS score and the score was 15/15
  • Mental status assessment was done to determine the level of risk for maternal injury-irritability was not present. she had no seizures
  • Vital signs were monitored every 30 min
  • Strict monitoring of input and output was maintained
  • Patient was on strict bed rest, and both mother and foetus were continuously monitored till the time of delivery
  • Antihypertensive drugs were administered as advised.
  • Magnesium sulphate (MgSO4) following Zuspan regimen was administered for a total duration of 12 h as advised
  • Injection of calcium gluconate 10 ml in 10 ml sterile water over 10 min was administered as advised since the patient developed reduced urine output
  • Carboxymethylcellulose ointment 1 drop 4 hourly was administered as advised
  • A total of 8 units FFP, 9 units platelet and 3 units' whole blood were transfused as advised using strict asepsis
  • Liver function studies were followed up every day. During the initial 2 days, it showed a decline in liver function as evidenced by increasing values of liver enzymes. However, following the treatment, there was a steady improvement in liver function.


Evaluation

The patient's level of consciousness remained intact and urine output gradually returned to normal. She did not develop any adverse events. Her condition was stabilised and BP was controlled to certain extent from 230/140 mm Hg to 190/127 mm Hg.


  2. Nursing Diagnosis Top


Decreased cardiac output related to increased systemic vascular resistance secondary to HELLP syndrome as evidenced by the presence of fatigue and decreased activity tolerance.

Expected outcome

Patient maintains adequate cardiac output as evidenced by normal vital parameters – BP, pulse rate and rhythm, activity tolerance and less fatigue.

Intervention

  • Assessed BP, heart rate and SPO2 and Capillary refill time (CRT)
  • The presence of irritability, restlessness and difficulty concentration was assessed
  • Respiration – rate, rhythm and breath sounds were assessed and found normal
  • Assessed for reduction of pitting oedema of lower extremities
  • Assessed for peripheral pulses, cold and clammy skin and extremities, increased respiratory rate, the presence of orthopnoea, increased heart rate, neck vein distension and decreased level of consciousness
  • Supplemental oxygen was administered at 3–5 L/min
  • Strict bed rest was provided and fatigue and activity intolerance monitored
  • Catheterisation was done, and urine output was monitored
  • Elevation of the lower extremities was maintained and encouraged
  • Antihypertensive drugs were administered as advised
  • ECG was performed in bed, and findings were normal.


Evaluation

Patient's BP was controlled (140/87 mm Hg) and all vitals remained within normal parameters.


  3. Nursing Diagnosis Top


Ineffective uteroplacental tissue perfusion is related to decreased oxygen-carrying capacity of the maternal blood/vasoconstriction as evidenced by IUGR and preterm delivery.

Expected outcome

The baby will maintain FHR within normal baseline of 110–160 bpm.

Intervention

  • Continuous monitoring of foetal well-being was maintained using electronic foetal monitor (EFM) – FHR, tone, rhythm and deviations from the normal were observed.
  • Assessed for the presence of uterine contraction which was absent. Bleeding and tenderness were also assessed
  • Oxygen was administered at 3–5 L/min
  • Strict bed rest was observed.
  • Monitoring of daily foetal movement count (DFMC) was taught and encouraged to report, if any deviations are observed.
  • Injection betamethasone 12 mg stat was administered as advised as assessing gestational age which was found to be 33 weeks
  • Probable prognosis of both the mother and baby was explained and they were encouraged to ask questions and were kept updated throughout the treatment process.


Evaluation

The foetus maintained heart rate within the normal baseline, but the baby was delivered prematurely due to the presence of severe HELLP. Baby was shifted to the neonatal intensive care unit for necessary care.


  4. Nursing Diagnosis Top


Excess fluid volume is related to possible renal insufficiency, decreased cardiac output and liver dysfunction as evidenced by the presence of peripheral oedema and oliguria.

Expected outcome

Patient maintains heart rate of 60–100 beats/min, urine output ≥30 mL/h and oedema will be resolved.

Intervention

  • Assessed for oedema and weight gain. Monitored daily weight
  • Vital signs, especially BP and heart rate, were frequently monitored and recorded
  • Assessed for crackles, respiratory pattern, shortness of breath, orthopnoea and distended neck vein
  • Foley's catheterisation was done, and urine output was monitored every one hourly.
  • Extremities were kept elevated.
  • Injection Lasix 20 mg stat was administered as advised to reduce oedema.


Evaluation

Patient's heart rate was maintained between 60 and 100 beats/min, the urine output improved gradually with gradual subsidence of oedema.


  5. Nursing Diagnosis Top


Anxiety related to the uncertainty of prognosis perceived threat to self and baby evidenced by restlessness and verbalisation of nervousness.

Expected outcome

The patient verbalises reduced level of anxiety, maintains normal heart rate and utilise support effectively.

Intervention

  • Anxiety of the patient was assessed. She had mild anxiety
  • Heart rate was monitored for any changes associated with anxiety
  • Assessed for physical reactions to anxiety such as nausea, pain, dizziness and weakness
  • Encouraged to verbalise concerns, ask questions and clarify doubts
  • Encouraged to ask for help/assistance as required. All interactions were carried out in a calm and assuring manner as far as possible
  • Explained about the condition and probable prognosis
  • Environment stimuli were kept minimal to some extend by minimising noises, keeping peaceful environment
  • Assessed her past coping strategies and found talking to her mother reduces her stress
  • Assisted in developing/reinforcing anxiety-reducing strategies such as listening to calming music, relaxation, deep breathing and positive visualisation
  • All care activities, procedures and issues involving the patient were communicated to the patient and her attendants
  • Need for adherence to the medications as prescribed after discharge was explained
  • Encouraged mother to regularly monitor her BP
  • Follow-up after 1 week or to come immediately to the emergency department in case of dizziness/nausea/fainting/cold clammy skin.


Evaluation

The patient appears calm and verbalised reduced feeling of nervousness, and understanding of the condition involving her and the baby. She was cooperative throughout the care and expressed confidence in self-care and was motivated to perform home care.


  Conclusion Top


HELLP syndrome is a rare condition and life-threatening complication of pre-eclampsia. It requires immediate identification and intervention to reduce maternal morbidity and mortality and to improve neonatal outcomes. There is no therapy to prevent HELLP, but some suggest low-dose aspirin to reduce the risk of pre-eclampsia. Regular antenatal check-ups must be advocated for all antenatal mothers to reduce complications associated with pre-eclampsia. Pregnant women and post-partum women with clinical manifestations and/or signs suggestive of this obstetric complication require intense care and special procedures, alerting health professionals to the meticulousness of the drug therapy and the accomplishment of a laboratorial examination as early as possible.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
White CD. HELLP Syndrome; 2022. Available from: http://www.nlm.nih.gov/medlineplus/ency/article/000890.htm. [Last accessed on 2022 May 09].  Back to cited text no. 1
    
2.
Dutta DC, Dutta's Textbook of Gynecology. New Delhi: Jaypee Brothers Medical Publisher; 2018.  Back to cited text no. 2
    
3.
Ribeiro JF, Silva Melo SS, Costa Silva C, Simone Vieira Carvalho Guimarães SV, Guimarães dos Santos M. HELLP syndrome: Obstetric characterization and treatment modality. J Nurs UFPE 2017;3:1343-8  Back to cited text no. 3
    
4.
HELLP Syndrome. American Pregnancy Association; 2018. Available from: http://americanpregnancy.org/pregnancy-complications/hellp- syndrome. [Last accessed on 2022 May 09].  Back to cited text no. 4
    
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Haram K, Svendsen E, Abildgaard U. The HELLP syndrome: Clinical issues and management. A Review. BMC Pregnancy Childbirth 2009;9:8.  Back to cited text no. 5
    
6.
Malmström O, Morken NH. HELLP syndrome, risk factors in first and second pregnancy: A population-based cohort study. Acta Obstet Gynecol Scand 2018;97:709-16.  Back to cited text no. 6
    
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Phipps E, Prasanna D, Wunnie B, Belinda J. Preeclampsia: Updates in Pathogenesis, Definitions, and Guidelines; 2022. Available from: https://cjasn.asnjournals.org/content/11/6/1102. long [Last accessed on 2022 May 09].  Back to cited text no. 7
    
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Burton GJ, Redman CW, Roberts JM, Moffett A. Pre-eclampsia: Pathophysiology and clinical implications. BMJ 2019;366:l2381.  Back to cited text no. 8
    
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Uzan J, Carbonnel M, Piconne O, Asmar R, Ayoubi JM. Pre-eclampsia: Pathophysiology, diagnosis, and management. Vasc Health Risk Manag 2011;7:467-74.  Back to cited text no. 9
    
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Huang H, Liu B, Gao X, Wang Y. Clinical classification, pregnancy outcomes and risk factors analysis of severe preeclampsia complicated with HELLP syndrome. Front Surg 2022;9:859180.  Back to cited text no. 10
    
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Sibai BM. HELLP Syndrome (Hemolysis, Elevated Liver Enzymes, and Low Platelets). UpToDate; 2022. Available from: https://www.uptodate.com/contents/preeclampsia-clinical-features-and-diagnosis. [Last accessed on 2022 May 09].  Back to cited text no. 11
    
12.
Padden MO. HELLP syndrome: Recognition and perinatal management. Am Fam Physician 1999;60:829-36, 839.  Back to cited text no. 12
    
13.
Jobe AH, Soll RF. Choice and dose of corticosteroid for antenatal treatments. Am J Obstet Gynecol 2004;190:878-81.  Back to cited text no. 13
    
14.
Drakeley AJ, Le Roux PA, Anthony J, Penny J. Acute renal failure complicating severe preeclampsia requiring admission to an obstetric intensive care unit. Am J Obstet Gynecol 2002;186:253-6.  Back to cited text no. 14
    
15.
Mikolajczyk AE, Renz J, Diaz G, Alpert L, Hart J, Te HS. Massive hepatic infarction caused by HELLP syndrome. ACG Case Rep J 2017;4:e81.  Back to cited text no. 15
    


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    Tables

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  In this article
Abstract
Introduction
Case Report
1. Nursing Diagnosis
2. Nursing Diagnosis
3. Nursing Diagnosis
4. Nursing Diagnosis
5. Nursing Diagnosis
Conclusion
References
Article Figures
Article Tables

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