HELLP Syndrome: Difference between revisions

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<div class="editorbox">'''Original Editors '''- Carolyn S. Furdek [[Pathophysiology of Complex Patient Problems|from Bellarmine University's&nbsp;Pathophysiology of Complex Patient Problems project.]]  
<div class="noeditbox">Welcome to [[Pathophysiology of Complex Patient Problems|PT 635 Pathophysiology of Complex Patient Problems]] This is a wiki created by and for the students in the School of Physical Therapy at Bellarmine University in Louisville KY. Please do not edit unless you are involved in this project, but please come back in the near future to check out new information!!</div><div class="editorbox">
'''Original Editors '''- Carolyn S. Furdek [[Pathophysiology of Complex Patient Problems|from Bellarmine University's&nbsp;Pathophysiology of Complex Patient Problems project.]]  


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== Definition/Description  ==


[[Image:Liver.jpg|border|left|230x200px|A liver affected by HELLP Syndrome]]  
== Introduction ==
[[File:Birth.jpeg|right|frameless|399x399px]]
HELLP syndrome is a life-threatening condition that can potentially complicate pregnancy. It is named for 3 features of the condition: Hemolysis, [[Liver Function Tests|Elevated Liver enzyme levels]], and Low Platelet levels. It typically occurs in the last 3 months of pregnancy but can also start soon after delivery.


<br>
* A wide range of non-specific symptoms may be present in women with HELLP syndrome. Symptoms may include fatigue; malaise; fluid retention and excess weight gain; [[headache]]; nausea and vomiting; pain in the upper right or middle of the abdomen; blurry vision; and rarely, nosebleed or seizures.
* The cause of HELLP syndrome is not known, but certain risk factors have been associated with the condition. It is most common in women with preeclampsia or eclampsia. If not diagnosed and treated quickly, HELLP syndrome can lead to serious complications for the mother and baby.
* The main treatment is to deliver the baby as soon as possible, even if premature, if there is distress of the mother or the baby. Treatment may also include medications needed for the mother or baby, and [[blood]] transfusion for severe bleeding problems.<ref>Rare Diseases hellp syndrome Available: https://rarediseases.info.nih.gov/diseases/8528/hellp-syndrome (accessed 5.9.2021)</ref>


HELLP syndrome is an acronym for several life-threatening symptoms that occur together in a woman’s pregnancy.
== Etiology/Causes ==
The aetiology and pathophysiology remain incompletely understood with multiple theories:


These symptoms are:  
* Immune-mediated: maternal acute rejection reaction to fetal antigens
* Placenta-mediated liver injury
* [[SIRS / Sepsis / Septic Shock|Systemic inflammatory response syndrome]] in the setting of pre-eclampsia<ref name=":0">Radiopedia [https://radiopaedia.org/articles/hellp-syndrome HELLP syndrome] Available:https://radiopaedia.org/articles/hellp-syndrome (accessed 6.10.2021)</ref>


*<span id="fck_dom_range_temp_1298593567296_714"></span>H – [http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0003024 '''h'''emolysis]
== Epidemiology ==
*EL – '''e'''levated '''l'''iver enzymes
HELLP syndrome has a prevalence of 0.5% to 0.9%. About 70% of cases occur in the third trimester of pregnancy, and the remainder occurs within 48 hours of delivery. The mortality rate of women with HELLP syndrome is 0 to 24%, with a perinatal death rate of up to 37%<ref name=":1">Khalid F, Tonismae T. [https://www.statpearls.com/articlelibrary/viewarticle/22684/ HELLP Syndrome]. StatPearls [Internet]. 2020 Jul 31.Available:https://www.statpearls.com/articlelibrary/viewarticle/22684/ (accessed 5.10.2021)</ref>
*LP – '''l'''ow [http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0004113 '''p'''latelet count].&nbsp;<ref name="PubMed">PubMed Health website. HELLP syndrome. Available at http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001892. Accessed February 18, 2011.</ref><sup></sup>


<br>
== Clinical Presentation ==
The presentation can be variable and can include malaise, epigastric and/or right upper quadrant pain, and nausea and vomiting. Some may have non-specific viral-like symptoms. The symptoms continuously progress.


<br>
* [[Hypertension in Pregnancy|Hypertension]] and proteinuria (classic symptoms of pre-eclampsia) may be absent or slight.<ref name=":0" />
* The average gestational age of presentation of HELLP syndrome is 34 weeks.
* Patients are usually multiparous and over the age of 35 years old.
* Patients are often overweight and have [[Oedema Assessment|edema]] in 50% of the cases.
* The condition exacerbates during the night. <ref name=":1" />


<br>[Image courtesy of [http://www.ispub.com/journal/the_internet_journal_of_anesthesiology.html The Internet Journal of Anesthesiology]]  
== Radiographic features ==
[[File:Liver gif.gif|right|frameless|200x200px]]
General features predominantly involve hepatic (liver) sequelae:


== Prevalence  ==
* hepatomegaly: especially the right lobe
* haemorrhage, subcapsular haematoma, rupture
* hepatic infarction<ref name=":0" />&nbsp;
Image 2: Liver position


As of Nov 2010, for every 1,000 pregnancies, 1 to 2 (0.5%-0.9%) women will be diagnosed with HELLP syndrome.&nbsp;&nbsp;Furthermore,&nbsp;10-20% of women diagnosed with severe&nbsp;[http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001900 preeclampsia]&nbsp;will be diagnsosed with HELLP.<ref name="PubMed" /> Mortality rates associated with HELLP syndrome have been reported as high as 25%.<ref name="Padden" />
== Treatment / Management ==
[[File:Neonatal intensive care.jpeg|right|frameless|399x399px]]
HELLP syndrome has the potential to quickly turn life-threatening for both mother and fetus. Therefore the recommendation is always to hospitalize patients for strict monitoring of laboratory values.  


Patients have a 19-27% chance of reoccurance on subsequent pregnancies.<ref name="Padden">Padden MO. HELLP Syndrome: Recognition and Perinatal Management. American Family Physician. September 1999. Available online at http://www.aafp.org/afp/990901ap/829.html. Accessed 1 March 2011.</ref>
* During hospitalization, patients should be treated as severely pre-eclamptic and should receive magnesium sulfate for seizure prophylaxis along with [[Blood Pressure|blood pressure]] control.
* Maternal-fetal monitoring should be performed throughout each step of management as typically, immediate delivery is recommended for true HELLP patients
* Those with stable maternal-fetal conditions between 24 to 34 weeks gestation a recommendation is to give [[Corticosteroid Medication|corticosteroids]] then deliver 24 hours after the last dose. See also [[Neonatal Respiratory Distress Syndrome]]. Steroid administration is not only beneficial to the fetus for lung maturity but also for improvement of [[Laboratory Tests|laboratory values]] in patients, particularly in elevating platelet counts.
* Some patients may benefit from transfusions of red cells, platelets, and plasma<ref name=":1" />.


== Characteristics/Clinical Presentation  ==
&nbsp;  
 
Approximately 7 out of 10 patients with HELLP syndrome will experience the symptoms prior to delivery between the 27th and 37th week of gestation. The remaining patients will develop the symptoms within 48 hours postpartum.<ref name="Haram" />
 
There are two classifications of HELLP Syndrome:<ref name="Padden">Padden MO. HELLP Syndrome: Recognition and Perinatal Management. American Family Physician. September 1999. Available online at http://www.aafp.org/afp/990901ap/829.html. Accessed 1 March 2011.</ref>
 
*Classification 1:&nbsp; Basis of 3 classic lab values
 
&nbsp;&nbsp;&nbsp;&nbsp;-&nbsp;Partial: one/two of the classic values present&nbsp;  


&nbsp;&nbsp;&nbsp; - Full:&nbsp; all three abnormalities present
[[Image:HELLP Chart.gif|center|Figure: Suggested protocol in treating pts with HELLP Syndrome|alt=|thumb|580x580px]]


(''Full HELLP syndrome classifications have a higher mortality rate and should be delivered within 48 hours'')  
[Chart courtesy of Journal of The American Family Physician: [http://www.aafp.org/afp/990901ap/829.html HELLP Syndrome]<ref>Padden MO. HELLP syndrome: recognition and perinatal management. American family physician. 1999 Sep;60(3):829-36.</ref>]


*Classification 2:&nbsp;&nbsp;Basis of platlet&nbsp;count
== Prognosis  ==


&nbsp;&nbsp;&nbsp; - Class 1:&nbsp; Platlet count &lt; 50,000 mm<sup>3</sup>
HELLP syndrome is a life-threatening condition. Early diagnosis and treatment, along with maternal and neonatal intensive care, can help to reduce the mortality in HELLP syndrome.


&nbsp;&nbsp;&nbsp; - Class 2:&nbsp; Platlet count 50,000-100,000 mm<sup>3</sup>
* The mortality rate of women with HELLP syndrome is 0%-24%, with a perinatal death rate of up to 37% (poor perinatal prognosis is because of placental abruption, intrauterine hypoxia and asphyxia, prematurity, and low birth weight).


&nbsp;&nbsp;&nbsp;&nbsp;-&nbsp;Class 3:&nbsp; Platlet count 100,000-150,000 mm<sup>3</sup>
Maternal death occurs due to disseminated intravascular coagulation (DIC), placental abruption, postpartum hemorrhage, or acute renal failure.


(''Class 1 pts have a higher maternal morbidity and mortality rate'')
# DIC occurs in 15% to 62.5% of the cases.
# Placental abruption occurs in 11% to 25% of women with HELLP syndrome.
# Postpartum hemorrhage occurs in 12.5% to 40%
# Acute renal failure in 36% to 50% of the cases.


Clinical symptoms of HELLP include discomfort in the upper right quadrant of the abdomen, pain in the epigastric area, vomiting, and nausea.<ref name="Haram">Haram, K. Svendsen, E. Abildgaard, U. The HELLP syndrome: Clinical issues and management. A Review. BMC Pregnancy Childbirth [serial online]. 2009; 9:8.</ref> The abdominal discomfort can increase and decrease throughout the day.<ref name="Sibai">Sibai BM. Diagnosis, controversies, and management of the syndrome of hemolysis, elevated liver enzymes, and low platelet count. Obstetrics and Gynecology [serial online]. 2004;103:981–991.</ref> Patients can report extreme fatigue prior to presentation or ‘feeling unwell’.<ref name="Sibai" /><ref name="PubMed" /> Other symptoms include headache, fluid retention, excess weight gain, blurry vision, nosebleeds (or bleeding that does not stop easily), seizures/convulsions.<ref name="PubMed" />
Patients with HELLP syndrome have a 19%-27% risk of developing HELLP syndrome in subsequent pregnancies<ref name=":1" />.  


== Associated Co-morbidities  ==
== Physical Therapy Management ==


HELLP syndrome can lead patients to be at a higher risk for the following conditions:<ref name="Svenningsen">Svenningsen R, Morken NH, Kahn JA. Corticosteroids in the treatment of HELLP-syndrome? Tidsskr Nor Laegeforen. 2006;126(17):2253–2256.</ref><ref name="Vigil">Vigil-De Gracia PE, Tenorio-Marañón RF, Cejudo-Carranza E, Helguera-Martinez A, García-Cáceres E. Difference between pre-eclampsia, HELLP syndrome and eclampsia, maternal evaluation. Ginecol Obstet Mex. 1996;64:337–382.</ref>  
Due to the severity and risk of maternal mortality, conservative management is not recommended in the treatment of HELLP syndrome.<ref name="Haram">Haram, K. Svendsen, E. Abildgaard, U. The HELLP syndrome: Clinical issues and management. A Review. BMC Pregnancy Childbirth [serial online]. 2009; 9:8.</ref>


*'''Renal Failure''' -&nbsp;loss of the kidney’s ability to function properly. The body will no longer be capable of filtering excess fluid, waste, and salts from the blood. This leads to dangerous levels in the system.<ref name="Mayo">Mayo Clinic web site. Acute Kidney Failure. Available at: http://www.mayoclinic.com/health/kidney-failure/DS00280. Accessed February 22, 2011.</ref>
* [[Abdominal Muscles|Abdominal]] weakness and deconditioning may be seen in patients after a [[Cesarean Section|caesarean]] section.&nbsp;  
*'''Consumptive coagulopathy'''&nbsp; - (also known as disseminated intravascular coagulation (DIC)) - clotting factors reduced<ref name="MEDLINE">Definitions, Online - Medline Plus. Available online at http://www.nlm.nih.gov/medlineplus/. Accessed 1 March 2011.</ref>
* Most patients are advised to wait&nbsp;4-6 weeks before resuming [[Physical Activity|physical activity]] and [[Therapeutic Exercise|exercise]].<ref name="C-section">Cesarean Birth; Post Partum Patient Education Material: Ohio State University Medical Center. Can be located online at http://medicalcenter.osu.edu/PatientEd/Materials/PDFDocs/women-in/post-par/cesarean.pdf. Accessed 25 March 2011.</ref>&nbsp;&nbsp;&nbsp;  
*'''Abruptio placentae - '''the placenta nourishing the fetus abruptly separates from the uterine wall prior to delivering the baby<ref name="MEDLINE" />
*'''Pulmonary edema - '''fluid build up in the&nbsp;lungs -&nbsp;can lead to shortness of breath.<ref name="MEDLINE" /><br>
*'''Cerebral edema''' - build up of fluid around the brain<ref name="MEDDIC">Definitions, Online – Medical Dictionary. Available online at http://www.medterms.com. Accessed 7 March 2011.</ref> <br>
*'''Subcapsular liver hematoma - '''pooling of blood just outside of the liver<ref name="MEDLINE" /><br>
*'''Hypovolemic shock - '''excessive fluid and blood loss that can lead to organ failure<ref name="MEDLINE" /><br>
 
== Diagnostic Tests/Lab Tests/Lab Values  ==
 
Lab Tests and Values:<ref name="Padden">Padden MO. HELLP Syndrome: Recognition and Perinatal Management. American Family Physician. September 1999. Available online at http://www.aafp.org/afp/990901ap/829.html. Accessed 1 March 2011.</ref>
 
Hemolysis:&nbsp;
 
*Low haptoglobin concentration (&lt; 1 g/L – &lt; 0.4 g/L)&nbsp; - ''more specific indicator ''<ref name="Haram" />
*High LDH<ref name="Haram" />
*presence of unconjugated bilirubin<ref name="Haram" />
 
&nbsp;&nbsp; (if Hematocrit normal: decreased serum&nbsp;haptoglobin levels may be present indicating HELLP)<ref name="Padden" />
 
Liver Enzymes: As high as 4,000 U per L
 
Platelets: As low as 6,000 per mm<sup>3</sup>&nbsp;(<span id="fck_dom_range_temp_1298996872834_120"></span><span id="fck_dom_range_temp_1298996872835_607"></span>anything less than 150,000&nbsp;per mm<sup>3</sup> should be of concern)
 
Plasma fibrogen: levels less than 300 mg per dL ([http://www.webmd.com/a-to-z-guides/disseminated-intravascular-coagulation-dic-topic-overview DIC] suspected)&nbsp;
 
== Etiology/Causes  ==
 
In 1982 L Weinstein identified cardinal signs and symptoms that were a variant of severe preeclampsia and named the condition HELLP.<ref name="Weinstein">Weinstein L. Syndrome of hemolysis elevated liver enzymes, and low platelet count: a severe consequence of hypertension in pregnancy. American Journal of Obstetrics and Gynecology. 1982. 142(2): 159-67.</ref>
 
The overall cause of HELLP syndrome in pregnant women is unknown at this time. However, researchers do have a better understanding of the three main characteristics that are known to occur with HELLP. These symptoms are: haemolysis, elevation of liver enzymes, and thrombocytopenia.
 
*Haemolysis occurs due to [http://www.nlm.nih.gov/medlineplus/ency/article/000571.htm microangiopathic haemolytic anaemia] (MAHA). Blood smears have shown contracted red cells w/ spicula, polychromatic red cells, and increased reticulocyte counts. All these findings lead researches to suspect the development of MAHA. Increased [http://www.nlm.nih.gov/medlineplus/ency/article/003471.htm LDH] levels and decreased [http://www.nlm.nih.gov/medlineplus/ency/article/003677.htm haemoglobin] concentrations further show Haemolysis.<ref name="Haram" /> <br>
*Elevated liver enzymes indicates the involvement of the liver as well as reflecting the haemolytic process.<ref name="Haram" />
 
<br>
 
<br>
 
<br>
 
A recent study out of Turkey (published in March 2011) found that Homocysteine levels were significantly higher and a increased likelihood of deficiency in antithrombin III were found in women diagnosed with HELLP.<ref name="Dogan">Dogan OO, Simsek Y, Celen S, Danisman N., Frequency of herediatary thrombophilia, anticoagulant activity, and homocysteine levels in patients with hemolysis, elevated liver functions and low thrombocyte count (HELLP) syndrome. Journal of Obstetrics and gynaecology research, March 6 2011 (epub ahead of print). Available at: http://www.ncbi.nlm.nih.gov/pubmed/21375667. Accessed 8 March 2011.</ref> <br>
 
== Systemic Involvement  ==
 
add text here
 
== Medical Management (current best evidence)  ==
 
In patients diagnosed with HELLP syndrome prior to delivery, the immediate treatment is delivery of the fetus.<ref name="Bacq">Bacq Y. Liver diseases unique to pregnancy: A 2010 update. Clinics and Research in Hepatology and Gastroenterology. 2011; 20: (Article in Press) Available at http://www.ncbi.nlm.nih.gov/pubmed/21310683.</ref> If the fetus is earlier than 34 weeks gestation, steroid injections and close monitoring for 24-48 hours may be provided to allow the fetus’ lungs to mature.<ref name="Haram" />&nbsp;
 
[[Image:HELLP Chart.gif|frame|center|Figure: Suggested protocol in treating pts with HELLP Syndrome]]
 
[Chart courtesy of Journal of The American Family Physician: [http://www.aafp.org/afp/990901ap/829.html HELLP Syndrome]]<br>
 
'''Maternal medications<ref name="Padden" />'''&nbsp;
 
*Magnesium sulfate – anticonvulsant to prevent seizures
*Antihypertensive medications – high blood pressure
*Blood product – if necessary
*Dexamethasone – corticosteroid used for fetus lung maturity <br>
 
== Physical Therapy Management (current best evidence)  ==
 
Due to the severity and risk of maternal mortality, conservative management is not recommended in the treatment of HELLP syndrome.<ref name="Haram" />&nbsp;  
 
== Alternative/Holistic Management (current best evidence)  ==
 
Due to the severity and risk of maternal mortality, conservative management is not recommended in the&nbsp;treatment of HELLP syndrome.<ref name="Haram" />&nbsp;  


== Differential Diagnosis  ==
== Differential Diagnosis  ==
Line 135: Line 80:
HELLP Syndrome my be misdiagnosed as any of the below&nbsp;conditions:<ref name="Haram" />  
HELLP Syndrome my be misdiagnosed as any of the below&nbsp;conditions:<ref name="Haram" />  


*Viral Hepatitis  
*Viral [[Hepatitis A, B, C|Hepatitis]]
*Cholangitis  
*Cholangitis  
*Acute fatty liver of pregnancy  
*Acute fatty liver of pregnancy  
*Haemolytic uremic syndrome  
*Haemolytic uremic syndrome  
*Thrombotic thrombocytopenic purpura  
*Thrombotic thrombocytopenic purpura  
*Systemic lupus erythematosus
*[[Systemic Lupus Erythematosus|Systemic lupus erythematosus]]


== Case Reports/ Case Studies  ==
== Case Report ==


*HELLP syndrome in a pregnant patient with a past history of splenectomy for idiopathic thrombocytopenic purpura. Case Report.<ref name="Yamamoto">Yamamoto H. Yamazaki K. Nishikawa S. Hayashi T. Hayakawa O. Kudo R., HELLP syndrome in a pregnant patient with a past history of splenectomy for idiopathic thrombocytopenic purpura. Case Report. Gynecology and Obstetrics. 1997. 259(2), 105-107.</ref> [[http://www.springerlink.com/content/y5770048k31230k6/export-citation/ view article in Journal of Gynecology and Obstetrics]]&nbsp;&nbsp;
*Effect of corticosteroids on HELLP syndrome: a case report.<ref name="Schlembach">Schlembach D. Munz W. Fischer T., Effect of corticosteroids on HELLP syndrome: a case report. Journal of Perinatal Medicine, November 20, 2000. 28(6), 502-505.</ref> [[http://www.reference-global.com/doi/abs/10.1515/JPM.2000.068 view article in Journal of Perinatal Medicine]]
*Subscapular Liver Hematoma in HELLP Syndrome: Case Report.<ref name="Kapan">Kapan M. Evsen MS. Gumas M.. Onder A. Tekbas G., Subscapular Liver Hematoma in HELLP Syndrome: Case Report. Gastroenterology Research. June 2010. 3(3). 144-146.</ref> [[http://www.gastrores.org/index.php/Gastrores/article/viewArticle/205/246 view article in Gastroenterology Research]]
*Subscapular Liver Hematoma in HELLP Syndrome: Case Report.<ref name="Kapan">Kapan M. Evsen MS. Gumas M.. Onder A. Tekbas G., Subscapular Liver Hematoma in HELLP Syndrome: Case Report. Gastroenterology Research. June 2010. 3(3). 144-146.</ref> [[http://www.gastrores.org/index.php/Gastrores/article/viewArticle/205/246 view article in Gastroenterology Research]]
*Case Report: post partum class 1 HELLP syndrome.<ref name="Basama">Basama FM. Granger K, Case Report: post partum class 1 HELLP syndrome. Gynecology and Obstetrics. 2007. 275(3) 187-189.</ref>&nbsp; [[http://www.springerlink.com/content/y42078717x684301/ view article in Journal of Gynecology and Obstetrics]]
== <br>Resources  ==
*American Pregnancy Association - [http://www.americanpregnancy.org www.americanpregnancy.org]
*Preeclampsia&nbsp;Foundation&nbsp;- [http://www.preeclampsia.org www.preeclampsia.org]
*HELLP Syndrome Society - [http://www.preeclampsia.org www.preeclampsia.org]&nbsp; (merged with preeclampsia foundation)
== Recent Related Research (from [http://www.ncbi.nlm.nih.gov/pubmed/ Pubmed])  ==
<div class="researchbox">
<rss>http://eutils.ncbi.nlm.nih.gov/entrez/eutils/erss.cgi?rss_guid=12Su8VomptfJPpE_rTFsJEzX3BaJXtAe95ks9RBwz0MZBjlEnU|charset=UTF-8|short|max=10</rss>
</div>
== References  ==
== References  ==


<references />
<references />  


[[Category:Bellarmine_Student_Project]]
[[Category:Bellarmine_Student_Project]]
[[Category:Womens_Health]]
[[Category:Pregnancy]]
[[Category:Postpartum]]
[[Category:Pelvis]] [[Category:Conditions]] [[Category:Pelvis - Conditions]]

Latest revision as of 11:45, 3 August 2022

Original Editors - Carolyn S. Furdek from Bellarmine University's Pathophysiology of Complex Patient Problems project. Top Contributors - Carolyn Furdek, Nicole Hills, Lucinda hampton, Admin, Elaine Lonnemann, Kim Jackson, 127.0.0.1, Wendy Walker and WikiSysop  

Introduction[edit | edit source]

Birth.jpeg

HELLP syndrome is a life-threatening condition that can potentially complicate pregnancy. It is named for 3 features of the condition: Hemolysis, Elevated Liver enzyme levels, and Low Platelet levels. It typically occurs in the last 3 months of pregnancy but can also start soon after delivery.

  • A wide range of non-specific symptoms may be present in women with HELLP syndrome. Symptoms may include fatigue; malaise; fluid retention and excess weight gain; headache; nausea and vomiting; pain in the upper right or middle of the abdomen; blurry vision; and rarely, nosebleed or seizures.
  • The cause of HELLP syndrome is not known, but certain risk factors have been associated with the condition. It is most common in women with preeclampsia or eclampsia. If not diagnosed and treated quickly, HELLP syndrome can lead to serious complications for the mother and baby.
  • The main treatment is to deliver the baby as soon as possible, even if premature, if there is distress of the mother or the baby. Treatment may also include medications needed for the mother or baby, and blood transfusion for severe bleeding problems.[1]

Etiology/Causes[edit | edit source]

The aetiology and pathophysiology remain incompletely understood with multiple theories:

Epidemiology[edit | edit source]

HELLP syndrome has a prevalence of 0.5% to 0.9%. About 70% of cases occur in the third trimester of pregnancy, and the remainder occurs within 48 hours of delivery. The mortality rate of women with HELLP syndrome is 0 to 24%, with a perinatal death rate of up to 37%[3]

Clinical Presentation[edit | edit source]

The presentation can be variable and can include malaise, epigastric and/or right upper quadrant pain, and nausea and vomiting. Some may have non-specific viral-like symptoms. The symptoms continuously progress.

  • Hypertension and proteinuria (classic symptoms of pre-eclampsia) may be absent or slight.[2]
  • The average gestational age of presentation of HELLP syndrome is 34 weeks.
  • Patients are usually multiparous and over the age of 35 years old.
  • Patients are often overweight and have edema in 50% of the cases.
  • The condition exacerbates during the night. [3]

Radiographic features[edit | edit source]

Liver gif.gif

General features predominantly involve hepatic (liver) sequelae:

  • hepatomegaly: especially the right lobe
  • haemorrhage, subcapsular haematoma, rupture
  • hepatic infarction[2] 

Image 2: Liver position

Treatment / Management[edit | edit source]

Neonatal intensive care.jpeg

HELLP syndrome has the potential to quickly turn life-threatening for both mother and fetus. Therefore the recommendation is always to hospitalize patients for strict monitoring of laboratory values.

  • During hospitalization, patients should be treated as severely pre-eclamptic and should receive magnesium sulfate for seizure prophylaxis along with blood pressure control.
  • Maternal-fetal monitoring should be performed throughout each step of management as typically, immediate delivery is recommended for true HELLP patients
  • Those with stable maternal-fetal conditions between 24 to 34 weeks gestation a recommendation is to give corticosteroids then deliver 24 hours after the last dose. See also Neonatal Respiratory Distress Syndrome. Steroid administration is not only beneficial to the fetus for lung maturity but also for improvement of laboratory values in patients, particularly in elevating platelet counts.
  • Some patients may benefit from transfusions of red cells, platelets, and plasma[3].

 

Figure: Suggested protocol in treating pts with HELLP Syndrome

[Chart courtesy of Journal of The American Family Physician: HELLP Syndrome[4]]

Prognosis[edit | edit source]

HELLP syndrome is a life-threatening condition. Early diagnosis and treatment, along with maternal and neonatal intensive care, can help to reduce the mortality in HELLP syndrome.

  • The mortality rate of women with HELLP syndrome is 0%-24%, with a perinatal death rate of up to 37% (poor perinatal prognosis is because of placental abruption, intrauterine hypoxia and asphyxia, prematurity, and low birth weight).

Maternal death occurs due to disseminated intravascular coagulation (DIC), placental abruption, postpartum hemorrhage, or acute renal failure.

  1. DIC occurs in 15% to 62.5% of the cases.
  2. Placental abruption occurs in 11% to 25% of women with HELLP syndrome.
  3. Postpartum hemorrhage occurs in 12.5% to 40%
  4. Acute renal failure in 36% to 50% of the cases.

Patients with HELLP syndrome have a 19%-27% risk of developing HELLP syndrome in subsequent pregnancies[3].

Physical Therapy Management[edit | edit source]

Due to the severity and risk of maternal mortality, conservative management is not recommended in the treatment of HELLP syndrome.[5]

Differential Diagnosis[edit | edit source]

HELLP Syndrome my be misdiagnosed as any of the below conditions:[5]

Case Report[edit | edit source]

References[edit | edit source]

  1. ↑ Rare Diseases hellp syndrome Available: https://rarediseases.info.nih.gov/diseases/8528/hellp-syndrome (accessed 5.9.2021)
  2. ↑ 2.0 2.1 2.2 Radiopedia HELLP syndrome Available:https://radiopaedia.org/articles/hellp-syndrome (accessed 6.10.2021)
  3. ↑ 3.0 3.1 3.2 3.3 Khalid F, Tonismae T. HELLP Syndrome. StatPearls [Internet]. 2020 Jul 31.Available:https://www.statpearls.com/articlelibrary/viewarticle/22684/ (accessed 5.10.2021)
  4. ↑ Padden MO. HELLP syndrome: recognition and perinatal management. American family physician. 1999 Sep;60(3):829-36.
  5. ↑ 5.0 5.1 Haram, K. Svendsen, E. Abildgaard, U. The HELLP syndrome: Clinical issues and management. A Review. BMC Pregnancy Childbirth [serial online]. 2009; 9:8.
  6. ↑ Cesarean Birth; Post Partum Patient Education Material: Ohio State University Medical Center. Can be located online at http://medicalcenter.osu.edu/PatientEd/Materials/PDFDocs/women-in/post-par/cesarean.pdf. Accessed 25 March 2011.
  7. ↑ Kapan M. Evsen MS. Gumas M.. Onder A. Tekbas G., Subscapular Liver Hematoma in HELLP Syndrome: Case Report. Gastroenterology Research. June 2010. 3(3). 144-146.
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