The evaluation of maternal parameters at diagnosis may predict HELLP syndrome severity.

OBJECTIVE: Hemolysis, elevated liver enzymes, low platelets (HELLP) syndrome, a severe manifestation of pre-eclampsia and/or intrauterine growth restriction (IUGR) of the fetoplacental unit, is classified into three classes, according to the lowest platelet count observed during the course of the disease. The aim of our work was to analyze the levels of lactate dehydrogenase (LDH), aspartate transferase (AST), alanine transferase (ALT) and platelets at the time of HELLP syndrome diagnosis, to find possible cut-off values that could predict the severity of the syndrome from its early onset. METHODS: A retrospective analysis of the clinical records of 26 patients consecutively diagnosed with classes 1 and 2 HELLP syndrome was performed. Platelet count (x 1000/ml), LDH (IU/l), AST (IU/l), ALT (IU/l), hemoglobin (g/dl), hematocrit (%) and D-dimer (log of titer) were determined at admission and compared with the most severe peak values. Receiver operating characteristic (ROC) curves were used to calculate the best cut-off value at admission which correlated with the development of class 1 HELLP syndrome (the most severe condition). The post-test probability of developing class 1 severity was calculated. RESULTS: Mean gestational age at diagnosis was 33.4 weeks (range 23-40 weeks). Peak values of LDH, AST and ALT were significantly higher in class 1 HELLP syndrome patients. The platelet count at admission was not informative in differentiating patients who would later develop class 1 or class 2 HELLP syndrome. According to the best cut-off values at admission for LDH, AST and ALT, the post-test probability to predict patients with class 1 HELLP syndrome was 74%, 71% and 78%, respectively. If all the three parameters were above the cut-off value, the probability increased to 90%. CONCLUSIONS: The LDH, AST and ALT values at admission blood test, and to a greater extent the combination of all three abnormal tests, could predict the severity of HELLP syndrome.

[Familial transient red cell aplasia from parvovirus B-19 infection]. / Esordio familiare di crisi aplastica transitoria da Parvovirus B-19.

In our Paediatric Clinic we observed a case of transient aplastic crisis caused by Parvovirus B19 in a child and his mother, both affected by spherocytic haemolytic anemia. Anti-Parvovirus IgM antibody titre and viral search by PCR were positive. Anemia was treated with transfusion of concentrated red blood cells. In case of a family onset of hyperacute anemia it is necessary to consider a bone marrow aplastic crisis of the red series, induced by Parvovirus B19, especially if there is notice of an ongoing outbreak of erythema infectiosum.

HELLP syndrome and factor V Leiden.

The association of thrombophilia and obstetrical complications is documented and well consistent with the hypothesis of an insufficient placental perfusion due to fibrin deposition as a major underlying pathophysiological mechanism. Factor V Leiden is one of the most frequent thrombophilic mutations. A high prevalence of this mutation has recently been reported in a group of 21 German women with haemolysis, elevated liver enzymes, low platelets (HELLP) syndrome. In this respect, we studied the prevalence of factor V Leiden in 18 women who were consecutively diagnosed at our Department of Obstetrics and Gynaecology as having HELLP syndrome, between 1995 and 1999. Women were tested either at the time of diagnosis or months or years after delivery for coagulation parameters, protein C (PC), protein S (PS), antithrombin III, lupus-like anticoagulant, anticardiolipin antibodies (ACA), activated protein C (APC) resistance and detection of the G1691A mutation (factor V Leiden). In all women, the parameters studied were normal and in none of the investigated cases was the G1691A mutation found. HELLP being a severe form of preeclampsia, we think that the reported association between factor V Leiden and HELLP may reflect the well-known association with preeclampsia.

Soluble transferrin receptor in the study of fetal erythropoietic activity.

In order to evaluate fetal erythropoiesis we measured red blood cells, hemoglobin, hematocrit, serum transferrin receptor (sTfR), and iron status parameters in fetuses undergoing percutaneous umbilical blood sampling, and in normal newborns at term. We found high levels of sTfR in fetuses and newborns as compared with normal adults (3,149 +/- 181 vs. 1,881 +/- 137 ng/ml, P < 0.00001). Concentrations of sTfR correlate with gestational age and red blood cell numbers (r = 0.441, P < 0.001; r = 0.366, P = 0.06). sTfR concentrations do not show correlation with iron status parameters. The increased sTfR concentration is consistent with the fact that fetal life is characterized by cell proliferation and tissue growth. sTfR concentration correlates with gestational age and numbers of red blood cells, and can therefore be considered a good indicator of fetal erythropoiesis. It is conceivable that, during intrauterine life, sTfR expression is independent from iron status. sTfR determination will help in reaching a better understanding of some aspects of fetal physiology, and will help elucidate the physiopathology of fetal hematological diseases.

Sickle cell thalassemia in pregnancy: a case report.

A 31-year-old pregnant woman at 20 weeks' gestation was sent to our hospital for fever, anemia, and arthralgias. As she was known to be a double heterozygote for beta-thalassemia and hemoglobin S, a diagnosis of hemolytic anemia caused by sickled red cells vasocclusive crises was made. The patient was submitted to partial exchange transfusion (PET) with a complete recovery. A second PET was performed at 36 weeks' gestation, and elective cesarean section was performed at 37 weeks, with the birth of a normal female neonate.

Red cell and plasma ferritin in a group of normal fetuses at different ages of gestation.

Plasma and cell ferritin were determined in 47 normal fetuses at different gestational ages in order to evaluate fetal iron status. Plasma ferritin shows an increase during pregnancy (17.7 micrograms/l-mean geometrical value of fetuses between 18 and 20 weeks; 56.8 micrograms/l mean geometrical value of fetuses between 32 and 35 wk) and significantly correlates to hemoglobin and number of red blood cells. Red cell ferritin, too, increases throughout pregnancy (from 92.7 ag/cell to 265 ag/cell) and shows a better correlation to hemoglobin and number of red blood cells. Experimental evidence has been provided showing that the placenta takes up ferritin and is important in iron storage and transport (even against a concentration gradient). Our data support this theory and suggest a situation of positive balance between iron uptake and utilization during intrauterine life.

[Postoperative hyperamylasemia: the evaluation of the efficacy of ranitidine in prevention]. / Iperamilasemia postoperatoria: valutazione dell'efficacia della ranitidina nella prevenzione.

The paper examines the role of ranitidine in the prevention of postoperative hyperamylasemia, a complication following major biliary tract surgery, especially if associated with intraoperative cholangiography. Forty patients underwent cholecystectomy and intraoperative cholangiography: calculosis of the choledochus was observed in 11 patients and a revision of the VBP was therefore carried out involving the transcystic insertion of Kehr's tube. Patients were randomly subdivided into two groups: group A (treated with 50 mg ranitidine 4 times a day e.v.) and group B (no anti H2 treatment). No significant differences were noted between the two groups with regard to the onset of hyperamylasemia, although it is worth reporting that ranitidine virtually eliminated all hemorrhagic complications due to ulcer or postoperative gastritis.

Periodic acid Schiff (PAS) positivity in fetal erythroblasts.

The smears of 26 fetal blood samples taken for prenatal diagnosis were stained for the PAS reaction. Twelve out of 26 cases were subsequently found to be normal and 14 were found to be affected by different abnormalities; 83.4% of normal cases and 92.8% of abnormal cases showed moderate positivity to the PAS reaction. A higher percentage of PAS positive erythroblasts was found in the abnormal cases (p less than 0.01). Five cases with a percentage greater than 10% also showed a very high percentage of erythroblasts. No linear correlation was found between PAS positivity and erythroblastosis either in the normal or the abnormal cases, but pooling them resulted in a correlation between PAS positivity and erythroblastosis (r = 0.536; p less than 0.005). PAS positivity was most often diffuse and scored 1+. Since erythroblastosis is considered an indirect sign of fetal liver erythropoiesis, the hepatic environment and its peculiar hemopoiesis could play a role in producing a cohort of PAS positive erythroblasts.

Retinoic acid induces liver/bone/kidney-type alkaline phosphatase gene expression in F9 teratocarcinoma cells.

All-trans retinoic acid (RA) induces alkaline phosphatase (ALP) activity by 3-8-fold in murine F9 teratocarcinoma cells, in parallel with their differentiation towards primitive endoderm. The elevation of ALP activity is associated with increases in the amounts of liver/bone/kidney-type ALP protein and the respective transcript. These effects of RA are due to activation of ALP gene transcription rather than to an increase in the half-life of the mRNA. Induction of ALP mRNA does not require de novo protein synthesis, since it is not blocked by treatment with cycloheximide. Dibutyryl cyclic AMP, which is known to induce further differentiation of F9 cells from the primitive to the parietal endoderm, blocks the induction of ALP mRNA by RA.

Purification and characterization of mouse liver xanthine oxidase.

Xanthine oxidase (EC 1.1.3.22) is purified to homogeneity from mouse liver after induction with bacterial lipopolysaccharide. The enzyme has an apparent molecular weight of 300,000 in its native state and it is suggested to be constituted of two identical subunits of Mr 150,000 each. The isoelectric point is 6.7 and the apparent Km value for xanthine is 3.4 microM. The amino acid composition of mouse xanthine oxidase is quite similar to that of Drosophila xanthine dehydrogenase.

T lymphocyte subpopulations in myelodysplastic syndromes.

T cell subpopulations, defined by monoclonal antibodies (OKT3, OKT4 and OKT8), were assessed on 13 patients with myelodysplasia (MDS). The percentage and numbers of OKT3- and OKT4-positive lymphocytes were significantly lower (p less than 0.025) than in normal controls, whereas those of OKT8 were not. In the group of patients with refractory anemia with excess of blasts (RAEB) and in those with chronic myelomonocytic leukemia, the percentage and absolute numbers of OKT8 lymphocytes were significantly lower (p less than 0.025) than in patients with refractory anemia or with primary acquired sideroblastic anemia, while those of OKT3 and OKT4 did not differ significantly. Quantitative impairment of T cell subpopulations may be part of the myelodysplastic situation as a result of the dyslymphopoiesis, according to the hypothesis that MDS originate from pluripotent stem cells. The decrease of OKT8 in RAEB and chronic myelomonocytic leukemia could be related to the previously shown insufficient erythropoietic activity in these patients.

Antioxidant system and serum trace elements in alpha-thalassaemia and Hb Lepore trait.

Erythrocyte antioxidant enzymes, superoxide dismutase, catalase and glutathione peroxidase were found to be significantly high in subjects with alpha-thalassaemia and Hb Lepore trait, as a consequence of the increased oxidant stress which is known to exist in these conditions. Among the serum trace elements present in these enzymes, selenium was increased in subjects with Hb Lepore trait and significantly low in those with alpha-thalassaemia trait, while selenium erythrocyte content was significantly increased in alpha-thalassaemic subjects.

One-stage operation for diverticular disease.

The experience with 59 patients affected by diverticular disease who underwent surgery after failure of medical treatment, is reported. 40 patients showed one or more postoperative complications, 19 none. The operations performed were: in 46 cases one-stage resection with anastomosis, Hartmann's operation in 8 and other procedures in 5. Six patients died: 3 after Harmann's operation, 2 after colostomy and one after anastomosis. The mean stay in bed for complicated cases was 24 days after anastomosis and 36 after Hartmann's operation. Therefore anastomosis is preferred in all cases including those with stenosis, fistula or abscess provided that peritonitis is not present. The anastomosis is performed away from the site of abdominal sepsis. In cases with peritonitis the selected surgical procedure is usually Hartmann's operation.