| Abstract|| |
A total of 52 patients were referred to our center from gynecology and obstetric units in our area with acute renal failure during the last two years. Seven patients were found to have so called syndrome of hemolysis (H), elevated liver enzymes (EL) and low platelets (LP) associated with acute renal failure. The syndrome can easily be confused with other diagnoses like hemolytic uremic syndrome, idiopathic thrombotic thrombocytopenic purpura and disseminated intravascular hemolysis. Six patients had renal biopsies to confirm the diagnosis, while one did not consent for biopsy. Four patients were found to have acute tubular necrosis, one had acute cortical necrosis and one was not enough for interpretation. We conclude that the patients can easily be misdiagnosed if we are not familiar with the diagnosis and that the overall prognosis is good if the patient survives the acute stage.
Keywords: Acute renal failure, HELLP syndrome, Saudi Arabia.
|How to cite this article:|
Sheikh IA, Shaheen FA. Acute Renal Failure and HELLP Syndrome: A Single Center's Experience. Saudi J Kidney Dis Transpl 1998;9:290-3
|How to cite this URL:|
Sheikh IA, Shaheen FA. Acute Renal Failure and HELLP Syndrome: A Single Center's Experience. Saudi J Kidney Dis Transpl [serial online] 1998 [cited 2020 Oct 24];9:290-3. Available from: https://www.sjkdt.org/text.asp?1998/9/3/290/39272
| Introduction|| |
Since the description of HELLP syndrome by Weinstein in 1982  and addition of this syndrome as a sixth criterion to diagnose severe preeclampsia, many reports were published from different parts of the world ,,, . Some authors reported this syndrome in women after delivery , . The cause of renal failure is said to be involvement of the kidneys in the generalized microangiopathic process. In general, the prognosis is reported to be good, with improvement in laboratory results over 48-72 hours after delivery  . We are reporting here seven cases that fulfilled the criteria of HELLP syndrome.
| Patients and Methods|| |
History and physical examination were performed on the patients. Complete blood count and chemistry were checked daily. Hemolysis was diagnosed by the presence of anemia, mild elevation of bilirubin, high LDH and moderate hyperkalemia. A two to three fold elevation of liver enzymes was taken as elevated liver enzymes and a platelet less than 100,000 at least two times was accepted as thrombocytopenia.
Renal biopsies were performed on all the patients except one. The patients received replacement therapy in the form of hemodialysis or slow continuous hemodiafiltration only when absolutely indicated. Also, the patients received fresh frozen plasma, packed RBC, diuretics and antiplatelet agents. None of the patients received plasmapheresis or platelet concentrates.
| Results|| |
[Table - 1] summerizes the history of the patients in our study, and outcome of their pregnancies. All patients delivered prematurely. Only two patients delivered viable babies. At presentation, the main complaints of the patients were nausea, vomiting and epigastric pain.
All patients had oligo-anuria. All patients had elevated blood pressure. Four patients developed generalized convulsions either before referral, or during their stay in our center. Mild to moderate pedal oedema was present in four patients. Only one patient had signs of generalized bleeding manifested as hematuria post renal biopsy and some vaginal bleeding. All the patients had mild jaundice.
All the patients had evidence of hemolysis with moderate anemia, mild elevation of bilirubin, high LDH and moderate hyperkalemia on admission. All the patients had two to three folds increase of transaminases above the normal levels. Varying degrees of thrombocytopenia was present on admission which persisted for a while. All but one patient had normal pro-thrombin time, partial thromboplastin time (PTT) and fibrinogen. Peripheral blood was positive for fragmented red blood cells in two patients.
The results of the renal biopsies showed acute tubular necrosis in four patients and acute cortical necrosis in one patient. In another patient, the biopsy specimen was insufficient for interpretation. The seventh patient did not consent for biopsy. These two patients responded to the treatment in the similar way of the other four patients with acute tubular necrosis. No platelet thrombi of fibrin clots were found in the renal biopsies.
| Discussion|| |
The combination of hemolytic anemia, thrombocytopenia and acute renal failure are features of serious conditions such as disseminated intravascular coagulation, (DIC), systemic vasculitis and hemolytic uremic syndromes (HUS). The three syndromes thrombotic thrombocytopenic purpura (TTP), childhood HUS and adult HUS are now considered to be variable expressions of a single entity because of many common features  .
There are few clinical parameters by which one can differentiate between these diseases. In DIC usually history of some severe insult like shock is available and general condition is poor. Hematological examination usually shows very high fibrinogen degradation products (FDP), a low serum fibrinogen, prolonged prothrombin time (PT) and partial thromboplastin time (PTT). Vasculitis has more musculoskeletal and cutaneous features with less prominent thrombocytopenia. In HUS, there is a history of some viral infection followed by severe renal failure, a moderate rise in FDP, slight decrease in serum fibrinogen but normal PT and PTT.
HELLP syndrome also enters in the differential diagnosis and is closer to HUS rather than DIC. The general condition of the patient in HELLP syndrome usually remains fair. There is often significant renal failure and laboratory investigations show elevated transaminases, normal fibrinogen FDP, PT and PTT.
The patients in our study also had normal bleeding profile. The patients studied seem to have developed HELLP syndrome before delivery but they were not diagnosed to have this syndrome, this could be the reason for high mortality of the neonates. Five of the patients were primigravida.
Renal biopsies of our patients did not show platelet or fibrin thrombi in small vessels which is a common feature in HUS. HELLP developed during pregnancy and improved after delivery.
Treatment of HUS includes fresh frozen plasma and plasmapheresis in an attempt to replace the "missing factor" or remove the substances causing enhanced platelet aggregations , . None of our patients received plasmapheresis. No platelet transfusion was given as the decrease in platelets is considered to be due to consumption and if more fuel is added, it might increase the severity of the disease. Hemodialysis treatment was given only when an absolute indication became evident. One patient, who was started on continuous arteriovenous hemofiltration (CAVH) had a significant sudden drop in platelet count.
Transfusion of fresh frozen plasma and antiplatelet agent remain main lines of treatment  . Four patients developed attacks of generalized convulsions. Two patients experienced the seizures before transfer to this center immediately after delivery while one patient developed seizures after two sessions of hemodialysis. One patient had confusion followed by attack of generalized convulsions while she was on conservative treatment. The causes of convulsions could be due to eclampsia, uncontrolled hypertension, sensitivity to a moderate rise in uremic toxins and post dialysis disequilibrium syndrome. None of the patients had any neurological sequellae. The rise of liver enzymes, which is caused by liver distension proved to be benign. It is demonstrated to be due to fiber deposits in the hepatic sinusoids by flourescent antibody technique  . No special treatment was required. Serum bilirubin came decreased to normal levels within 3-5 days in all the patients followed by normalization of transaminases. The patient who had acute cortical necrosis, renal biopsy reported a rim of viable glomeruli and this remaining part was proved to be enough in maintaining the serum creatinine level around 450 : 550 µmol/L. This patient is still being followed up in nephrology clinic.
| References|| |
|1.||Weinstein L. Syndrome of hemolysis, elevated liver enzymes and low platelet count: a severe consequence of hypertension in pregnancy. Am J Obstet Gynecol 1982;142 (2): 159-67. |
|2.||Baca L, Gibbons RB. The HELLP syndrome:a serious complication of pregnancy with hemolysis, elevated levels of liver enzymes, and low platelet count. Am J Mcd 1986; 85:590-1. |
|3.||Rath W, Loos W, Kuhn W, Graeff H.Significance of early laboratory diagnosis for obstetrical procedures in severe gestoses and the HELLP syndrome. Geburtshilfe Frauenheilkd 1988;48(3):127-33. |
|4.||Nisescrt S, Dribusch E, Bellmann O, Kauhausen H. Disorders of liver function, thrombopenia and hemodialysis in a special clinical form of hypertension in pregnancy (the so-called HELLP syndrome) Geburtshilfe Frauenheilkd 1989. |
|5.||Estcbe JP, Mallcdant Y, Miras A, et al. Two cases of the HELLP (Hemodialysis Elevated Liver Low Platelet) syndrome. Rev Fr Gynecol Obstet 1989;84(4):359-62. |
|6.||Bischofberger K, Diehl S. HELLP syndrome-postpartum Geburtshilfe Frauenheilkd. 1991;51(9):753-5. |
|7.||Schaldach P, Halle H, Birnbaum M. HELLP syndrome a serious complication in pregnancy. Zentralbl Gynakol 1989; 111(16): 1132-4. |
|8.||Martin JN Jr, Blake PG, Perry KG Jr, et al. The natural history of HELLP Syndrome: patterns of disease progression and regression:Am J Obstet Gynecol 1991;164:1500-9. |
|9.||Remuzzi G. HUS and TTP: variable expression of a single entity. Kidney Int 1987;32:292-308. [PUBMED] |
|10.||Myers TJ; Wakem CJ, Ball ED, Tremont SJ. Thrombotic thrombocytopenic purpura: combined treatment with plasmapheresis and antiplatelct agents. Ann Intern Med 1980;92:149-55. |
|11.||Egerman, RS; Witlm AG, Friedman SA, Sibai BM. Thrombotic thrombocytopenic purpura and hemolytic uremic syndrome in pregnancy review of 11 cases. Am J Obstet Gynecol 1996:175:950. |
|12.||Arias F, Mancilla-Jimencz R. Hepatic fibrinogen deposits in pre-eclampsia. Immunoflourescent evidence. N Engl J Med 1976;295:578-82. |
Iftikhar A Sheikh
Department of Nephrology, King Fahd Hospital, Jeddah Kidney Center, P.O. Box 11076, Jeddah 21453
[Table - 1]