Pentalogy of Cantrell: two patients and a review to determine prognostic factors for optimal approach

Two patients with incomplete pentalogy of Cantrell are described. The first was a girl with a large omphalocele with evisceration of the heart, liver and intestines with an intact sternum. Echocardiography showed profound intracardiac defects. The girl died 33 h after birth. The second patient was a female fetus with ectopia cordis (EC) without intracardiac anomalies; a large omphalocele with evisceration of the heart, stomach, spleen and liver; a hypoplastic sternum and rib cage; and a scoliosis. The pregnancy was terminated. A review of patients described in the literature is presented with the intention of finding prognostic factors for an optimal approach to patients with the pentalogy of Cantrell. In conclusion the prognosis seems to be poorer in patients with the complete form of pentalogy of Cantrell, EC, and patients with associated anomalies. Intracardial defects do not seem to be a prognostic factor

Serum screening with Down's syndrome markers to predict pre-eclampsia and small for gestational age: Systematic review and meta-analysis

<p>Abstract</p> <p>Background</p> <p>Reliable antenatal identification of pre-eclampsia and small for gestational age is crucial to judicious allocation of monitoring resources and use of preventative treatment with the prospect of improving maternal/perinatal outcome. The purpose of this systematic review was to determine the accuracy of five serum analytes used in Down's serum screening for prediction of pre-eclampsia and/or small for gestational age.</p> <p>Methods</p> <p>The data sources included Medline, Embase, Cochrane library, Medion (inception to February 2007), hand searching of relevant journals, reference list checking of included articles, contact with experts. Two reviewers independently selected the articles in which the accuracy of an analyte used in Downs's serum screening before the 25^thgestational week was associated with the occurrence of pre-eclampsia and/or small for gestational age without language restrictions. Two authors independently extracted data on study characteristics, quality and results.</p> <p>Results</p> <p>Five serum screening markers were evaluated. 44 studies, testing 169,637 pregnant women (4376 pre-eclampsia cases) and 86 studies, testing 382,005 women (20,339 fetal growth restriction cases) met the selection criteria. The results showed low predictive accuracy overall. For pre-eclampsia the best predictor was inhibin A>2.79MoM positive likelihood ratio 19.52 (8.33,45.79) and negative likelihood ratio 0.30 (0.13,0.68) (single study). For small for gestational age it was AFP>2.0MoM to predict birth weight < 10^thcentile with birth < 37 weeks positive likelihood ratio 27.96 (8.02,97.48) and negative likelihood ratio 0.78 (0.55,1.11) (single study). A potential clinical application using aspirin as a treatment is given as an example.</p> <p>There were methodological and reporting limitations in the included studies thus studies were heterogeneous giving pooled results with wide confidence intervals.</p> <p>Conclusion</p> <p>Down's serum screening analytes have low predictive accuracy for pre-eclampsia and small for gestational age. They may be a useful means of risk assessment or of use in prediction when combined with other tests.</p

Evaluation of the long-term effects of oleum origani on the toxicity induced by administration of streptozotocin in rats

WOS: A1997YJ03700021PubMed ID: 9401956Oleum origani, the essential oil of Origanum onites L., is a traditional plant material used in Turkey for the treatment of several diseases, including diabetes mellitus. This study has evaluated the effect of oleum origani on streptozotocin-induced tissue injury and haematological changes. The effect of oleum origani on glycaemia was also studied. Long-term administration of oleum origani resulted in significant improvement of tissue injury induced by streptozotocin treatment. No effect on blood glucose levels was detected. In addition, any visible toxicity or disturbance of haematological parameters and tissue structure attributable to the long-term use of oleum origani were not established in normal rats. The data indicate that long-term use of oleum origani might be effective in preventing or at least in retarding the development of some complications of diabetes mellitus. Further investigation is required to determine the underlying mechanism(s) of the protective effect against tissue injury induced by streptozotocin-treatment of rats

Prenatal diagnosis of Cantrell's pentalogy: a case report

A 23 year-old nulliparous woman was admitted to the obstetrics clinic in the 12(th) week of her pregnancy. Following the first trimester scanning, the fetus was diagnosed as having a large omphalocele and ectopia cordis. it was thought to be a thoracoabdominal wall defect and a possible case of Cantrell's pentalogy. Amniocentesis was performed and at the 16(th) week, the pregnancy was terminated because of karyotype revealing trisomy 21 and the serious structural defects. Autopsy demonstrated an ectopia cordis without pericardium and an abdominal wall defect with an omphalocele. Fetus had no diaphragma or sternum, and pulmonary and extremity anomalies were also present. With these findings, this case is suggested to be a variant of Cantrell's pentalogy

The megacystis-microcolon-intestinal hypoperistalsis syndrome - Report of a case and review of the literature

The megacystis-microcolon-intestinal hypoperistalsis syndrome is part of a spectrum of intestinal motility disorders and is characterized by abdominal distension, lax abdominal musculature, incomplete intestinal rotation, microcolon, megacystis, bilious vomiting and decreased or absent intestinal peristalsis. In this report a newborn girl with megacystis-microcolon-intestinal hypoperistalsis syndrome is reported

Fetal Splenic Size in Anemia Due to Rh-alloimmunization

To determine whether fetal splenic enlargement predicts anemia in Rh-alloimmunized nonhydropic singleton fetuses. Splenic circumference was measured before funipuncture in 21 singleton pregnancies on 47 occasions. The spleen was imaged in an axial section of the fetal abdomen close to the level used for measurement of the abdominal circumference. The splenic length and width were measured and the circumference calculated by the formula (length and width x 1.57). One measurement per patient was used for each analysis. Splenic circumference was measured and expressed as multiples of the normal median (MoM) for gestational age. One hundred twenty-one cases were used to provide cross-sectional normative data. The expected median splenic circumference values were derived from a normal group. Fetal anemia was defined as hemoglobin deficit, ie, mean hemoglobin concentration for gestation minus the measured value. Anemia was defined as hemoglobin deficit exceeding 2 g/dL, and severe anemia as hemoglobin deficit exceeding 5 g/dL. Receiver operator characteristics curves for the prediction of anemia using different splenic circumference (MoM) values were constructed. Splenic circumference was an excellent predictor of severe anemia in cases with no prior transfusion: sensitivity 100% and specificity 94.7% (area under the curve = .97, P < .03). The measurement did not correlate significantly with severe anemia in the group with prior transfusion (area under the curve = .73, P = .19). Splenomegaly is sensitive for the detection of severe anemia in nonhydropic Rh sensitized cases without prior transfusion. The splenic enlargement could be explained by extramedullary erythropoiesis