Introduction
For a long time, midwifery experts have found that in pregnant mothers with preeclampsia, signs of hemolysis, increase of hepatic enzymes, and thrombocytopenia are reported [1]. Years later, these symptoms were recognized as a separate symptom of preeclampsia and, with regard to triple symptoms, it was called HELP syndrome [2, 3]. Currently, HELP syndrome is a potent variant of preeclampsia or its complication [4]. To identify the full type of HELP syndrome, the presence of all three symptoms is required. If the mother had only one or two signs, she is suffering from incomplete form of this disease [5, 6].
HELP syndrome is a severe complication that is associated with high mortality rates for both mother and fetus. A wide range of complications may occur and may cause serious challenges in diagnosing, treating and managing delivery patterns [7]. Rapid diagnosis and treatment of HELP syndrome are crucial and their outcome is reassuring in terms of maternal and fetal health.
 
Patient and methods
The pregnant woman, 33 years old, who was pregnant for the third time, was admitted to the emergency department at the 22nd week of pregnancy with a history of 2 days of abdominal pain, headache, blurred vision, and vomiting. The patient's mother stated that the patient had a seizure several hours ago. There was not a significant point in the patient's personal and family history. During the admission, the patient was agitated and suffered from disturbances. Blood pressure was 160/105 mmHg, heart rate was 107 beats per minute; respiratory rate was 18 per minute; body temperature  was 37 ° C, and the measured blood sugar with a glucometer was 62 mg / dL. There was no point in the examination of the heart and lungs. The examination of the abdomen was normal, the uterus was raised to around the umbilical cord. There was no vaginal bleeding. Jaundice was seen in the patient's eyes and face, and in the urine with +4 strip, the protein was observed. With the suspicion of Hell's syndrome, essential tests were requested. Lactate dehydrogenase (LDH) was greater than 700 U / L and aspartate aminotransferase (SGOT) was about 180 U / L. The platelet count in the peripheral blood dropped sharply and was less than 100,000 per ml of blood. To control blood pressure, magnesium and labetalol were injected intravenously and 2 units of the platelet were injected. The patient was monitored for 24 hours and, unfortunately, the next day, the embryo was aborted.
 
Discussion
Hell's syndrome is a multi-systemic disease, with its distinct trait, Hemolytic anemia, impaired liver function with elevated liver enzymes and low platelet count.
Hell's syndrome was first described in 1982 by Weinstein [2]. The characteristics of the disease are morbidity and mortality rate of mothers and infants and their prevalence in pregnancies, and especially its association with preeclampsia and eclampsia. It is common in the third trimester of pregnancy, especially in white women over 25 years of age.
There is no detailed information on the etiology and pathogenesis of the disease. It is thought that abnormal formation of placenta causes ischemia in the placenta and the production of toxic substances in the bloodstream, which also causes damage to endothelial cells in the bloodstream. These injuries may lead to stenosis in various organs, activation of the coagulation system, increased capillary permeability, activation of platelets and their consumption in fine vessels, thereby leading to signs of hypertension, proteinuria, edema and thrombocytopenia.
Differential diagnosis of HELP syndrome: HELP syndrome may be mistaken for viral hepatitis, cholangitis and some other acute illnesses, such as cholecystitis, urinary tract infections, especially pyelonephritis, gastritis, gastric ulcer and acute pancreatitis.
Other illnesses such as immune thrombocytopenic purpura (ITP), acute fatty liver of pregnancy (AFLP), hemolytic urine syndrome (HUS), thrombotic thrombotocytopein purpura (TTP) and systemic lupus erythematosus (SLE) are less common but may impede HELP syndrome. . Often, these diseases are associated with high mortality in mothers and severe and prolonged cycles. These diseases may be mistaken for Hell syndrome. Careful examination is essential for proper diagnosis because they have different treatments.
Some AFLP clinical symptoms, although overlapping with Hell syndrome, are two completely different clinical and biochemical conditions. AFLP usually occurs between weeks 30 to 38 of gestation. Patients have the history of 1 to 2 weeks of bruising, abdominal distension, nausea, vomiting, and pain in the epigastric or right upper abdomen, headache and jaundice. High blood pressure and proteinuria are commonly absent. In further studies, increased blood concentrations, metabolic acidosis, acute liver failure, and some degrees of diffuse dystonia (DIC) with platelet counts reduced to normal or slightly, prolonged prothrombin time (PT) and prolonged blood clotting (PTT), decreased Concentrations of serum fibrinogen and antithrombin, leukocytosis, increased creatinine and uric acid, ammonia, liver enzymes such as alkaline phosphatase, aspartate aminotransferase (AST), alanine aminotransferase (ALT), and bilirubin are complementary findings. Reducing blood glucose and prolonging the prothrombin time may differentiate AFLP from Hell's syndrome. Liver ultrasound indicates increased liver ecchymosis. If the blood coagulation condition allows, liver biopsy has indication [8].
Major complications of AFLP include gastrointestinal bleeding, acute renal failure and pancreatitis. Most pregnant mothers recover from one to four weeks postpartum, but the disease may recur in the next pregnancy.
Complications of HELP syndrome: This syndrome is associated with a variety of complications in the mother, fetus and baby. Common complications in the mother include preeclampsia and eclampsia, placental decompression, disseminated intravascular coagulation (DIC), acute renal failure, pulmonary edema, liver subcapsular hematoma or rupture, retinal detachment, brain hemorrhage, and sometimes death of the pregnant mother. Common complications in embryos and newborns include intrauterine growth retardation, premature delivery, respiratory distress syndrome (RDS), thrombocytopenia, and perinatal death.
Treatment for pregnant mothers with HELP syndrome: Generally speaking, for the management of pregnant mothers with severe preeclampsia and Hell syndrome , the most appropriate option is delivery and termination of pregnancy if the fetal age is 34 weeks or older. If the age of the fetus is between 27 and 34 weeks, after 48 hours of the initial assessment and stabilization of the mother's condition and the treatment of short-term corticosteroid therapy, delivery should be taken. If the age of the fetus is less than 27 weeks, conservative treatment (longer than 48 to 72 hours) is usually considered.
The treatment of pregnant mothers with Hell syndrome: The first step is to accurately assess the conditions of the patient, and then examine the clinical conditions of the mother, the age of gestational age with ultrasound. After the above, the conditions for the onset of labor and the state of the cervix, should be examined.
The second step is to conduct laboratory tests including complete blood count, platelet count, critical prothrombin time tests, and blood clotting time, liver enzymes, LDH and Haptoglobin measurement, and urinalysis, blood pressure measurement, embryonic ultrasound and cardiopulmonary and cardiopulmonary Doppler examination. , administration of fluids and electrolytes, antihypertensive drugs (labatolol or nifedipine), and administration of magnesium sulfate to prevent seizure.
In the third step, controlling vital signs and observing fluid balance is vital.
Often immediate delivery is not recommended, but almost everyone agrees that delivery should be done 24 to 48 hours after treatment with steroids, preferably by natural or cesarean delivery. If the HELP syndrome is present before the 24th week of pregnancy, it is best to terminate the pregnancy.
The relationship between fatty acid oxidation disorders (FAODs) and HELP syndrome: FAODs in mitochondria are a group of genetic and metabolic diseases that are transmitted through autosomal recessive inheritance patterns. Infants with FAODs usually have symptoms of hypoglycemia, metabolic acidosis, liver failure, hepatomegaly, cardiomyopathy, and heart failure. Late symptoms of these diseases include episodic myopathies, neuropathy, retinopathy, and arrhythmia.
One of the most important symptoms of this disease is sudden and unexplained death in all ages. Some FAODs are associated with interruptions in intrauterine growth (IUGR) and premature birth. In mothers who are heterozygote for these diseases, important complications such as severe preeclampsia, acute fatty liver transplantation (AFLP) and HELP syndrome are very common.
These diseases (FAODs) are fairly common. Diagnosis is especially important before symptoms and complications occur, and can prevent maternal and infant mortality. Currently, the majority of these diseases can be diagnosed with neonatal metabolic screening by the Tandem Copper Spectrometry (MS / MS) technique. From this group of diseases, especially the disturbance of Long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD), Short-chain acyl-CoA dehydrogenase (SCAD) deficiency or mitochondrial trifunctional protein (MTP) deficiency, are more likely to cause HELP syndrome in pregnant mothers [9].
The heterozygous mothers of the homozygous embryo who carry one of these diseases may have symptoms of syndrome due to the passage of maternal toxin metabolites. The cause of this phenomenon in the fetus is that incomplete fatty acids are metabolized due to homozygous mutations, and since the mother is the carrier of the heterozygote of the same mutation, she cannot completely dispose the accumulated metabolites. Therefore, these substances apply their toxic effects to the liver and other tissues.
 
Conclusion
Fatty Acid Oxidation Disorders (FAODs) in mitochondria are a group of genetic and metabolic diseases that are transmitted through the autosomal recessive inheritance pattern. One of the most important symptoms of this disease is sudden and unexplained death in all ages. Some of the oxidation disorders of fatty acids are associated with the interruption of intrauterine growth (IUGR) and prematurity. In mothers who are heterozygote for these diseases, important complications such as severe preeclampsia, acute fatty liver in pregnancy (AFLP) and HELP syndrome are very common. These diseases (FAODs) are fairly common. Therefore, in pregnant women at high risk, special attention is needed to this complication.

Acknowledgments
The case was not found by the authors.
Ethical permission
The case was not found by the authors.
Conflict of Interest
The case was not found by the authors.
Financial support
This study was supported by Sarem Fertility and Infertility Research Center.
Contribution of authors
 Youssef Shafeghati (First author), all affairs of the article has been done by him (100%).