Correction to: Sudden onset of syncope and disseminated intravascular coagulation at 14 weeks of pregnancy: a case report.

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Sudden onset of syncope and disseminated intravascular coagulation at 14 weeks of pregnancy: a case report.

BACKGROUND: Amniotic fluid embolism (AFE), also known as anaphylactoid syndrome of pregnancy (ASP), typically occurs during labor and may result in cardiorespiratory collapse and disseminated intravascular coagulation (DIC). There are reports describing less typical presentations of AFE/ASP in which patients do not necessarily have the classic triad of hypoxia, hypotension, and coagulopathy. AFE/ASP rarely occurs in the absence of labor, but such cases may involve medical or surgical abortion, spontaneous miscarriage, or obstetrical procedures including amniocentesis and amnioinfusion. There are, however, no previously reported cases of AFE/ASP with sudden loss of consciousness and disseminated intravascular coagulation occurring during early pregnancy, in the absence of any intervention or obstetric event. CASE PRESENTATION: A 32-year-old G3P2 Japanese woman had sudden-onset syncope at 14 weeks' gestation. On arrival at our hospital, her level of consciousness was severely disturbed as determined by the Glasgow Coma Scale. Although her vital signs were initially stable, blood samples collected intravenously and by femoral artery puncture did not coagulate. A subchorionic hematoma with active extravasation of blood was apparent on contrast-enhanced computed tomography. Two hours after her arrival, she developed hypovolemic shock with progression of DIC, presumably due to intrauterine and retroperitoneal bleeding. After transfusion of blood products; treatments for DIC including the use of recombinant human soluble thrombomodulin, ulinastatin, and corticosteroids; and hysterectomy, her level of consciousness and physical condition improved remarkably. Later investigation of preoperative blood samples revealed that serum levels of AFE/ASP-associated markers were elevated. Immunohistochemical studies on the excised, unruptured uterus showed that amniotic fluid components were present inside a uterine blood vessel. CONCLUSIONS: This is the first reported patient with sudden-onset syncope and DIC, but without apparent cardiorespiratory collapse, with the highly likely etiology of AFE/ASP occurring at the beginning of the second trimester of pregnancy and in the absence of intervention or delivery. Maternal collapse with DIC during any stage of pregnancy should be considered an AFE/ASP-associated event, even in the absence of labor or obstetric procedures. This event may occur in the presence of subchorionic hematoma alone.

Vitamin K prophylaxis and late vitamin K deficiency bleeding in infants: fifth nationwide survey in Japan.

BACKGROUND: In 1980, the first nationwide survey on late vitamin K deficiency bleeding (VKDB) in infants was conducted in Japan, and it was followed by the second, third and fourth nationwide surveys in 1985, 1988 and 1991, respectively. The fifth nationwide survey was designed to ascertain the epidemiology of late VKDB between January 1999 and December 2004. PATIENTS AND METHODS: Questionnaires were sent to 2161 hospitals in Japan that employed members of the Japan Pediatric Society in March 2005. Responses were received from 1373 hospitals, for a response rate of 63.5%. RESULTS: The total number of reported cases was 71, including 21 idiopathic type and 16 secondary type. The incidence of late VKDB was estimated to be 1.9 cases per 100,000 births (95% confidence interval: 1.2-3.0) during this survey period. In 34 cases, the presence or absence of any underlying disease was not clarified. A total of 67/71 infants were entirely breast-fed. Intracranial hemorrhaging was observed in 26 (63.4%) out of 41 infants whose bleeding sites were described in the questionnaires. In 63 cases (88.7%) of late VKDB found in the present survey, however, vitamin K had been given at least once either during or after the neonatal period. CONCLUSIONS: A reevaluation of the current prophylaxis strategy for late VKDB in infants is necessary.

Diagnosis of Persistent Cloaca by Ultrasonography and MRI: A Case Report.

BACKGROUND Persistent cloacal malformations are rare anomalies that are anorectal malformations occurring in females. In cases of persistent cloaca, prenatal ultrasonography shows fetal ascites, cystic tumor in the abdomen, oligohydramnios, and hydronephrosis. There are various types of persistent cloaca, and symptoms vary. CASE REPORT A 38-year-old pregnant woman was referred to our hospital because of suspected fetal expansion of the intestinal tract. Prenatal ultrasonography revealed a fetal growth restriction, oligohydramnios, fetal abdominal cyst, and bilateral hydronephrosis, and persistent cloaca was suspected. Also, magnetic resonance imaging (MRI) revealed a double uterus and bilateral hydronephrosis, hydrocolpos; as such, persistent cloaca was diagnosed. Cesarean section was performed at 36 weeks+3 days gestation and delivered a female infant weighing 1957 g, with Apgar scores of 9 (1 min)/9 (5 min). CONCLUSIONS We report a case of persistent cloaca detected in the prenatal ultrasonography and MRI examination. Prenatal diagnosis is important because it can lead to a better outcome for infants with persistent cloaca. In the image inspection in persistent cloaca, there are characteristic findings such as ascites, cystic tumor in the abdomen, difficulty in visualizing the bladder, oligohydramnios, and hydronephrosis. So, if persistent cloaca is suspected, use of ultrasonography and MRI will allow its diagnosis.

Antiphospholipid syndrome: characteristics and obstetrical management.

Antiphospholipid syndrome (APS) is characterized by a combination of clinical features consisted of thrombotic or pregnancy-related events and autoimmune antiphospholipid antibodies. In the 1998 International Consensus Preliminary Criteria, APS is defined by the concomitant presence of these clinical features and laboratory tests, including solid immunoassay and lupus anticoagulant (LAC). Current concept of antiphospholipid antibodies directed against plasma proteins with affinity for negatively charged phospholipids, mainly beta2-glyoprotein 1 (beta2GP1) and prothrombin, that is, anti-beta2GP1 and anti-prothrombin antibodies have been shown as a key antibody in the APS, while the pathophysiological mechanisms remain still uncertain. In the recent investigations potential mechanisms, such as endothelial activation induced by bivalent-formed antiphospholipid antibodies and complement activation, have emerged. Recurrent pregnancy losses or fetal death, and increased rates of preeclampsia and placental insufficiency are the clinical features in the APS. It is now accepted that unfractionated or low-molecular-weight heparin in combination with low-dose aspirin represents the current standard treatment for pregnant women with anti-phospholipid antibodies, and high-dose immunoglobulin is considered as a salvage therapy for refractory APS. This review highlights the characteristics of APS and the recent consensus for obstetrical managements in APS.

Unilateral pulmonary agenesis associated with oesophageal atresia and tracheoesophageal fistula: A case report with prenatal diagnosis.

We describe herein a case of unilateral pulmonary agenesis (PA) with oesophageal atresia (EA)/tracheoesophageal fistula (TEF) that was diagnosed prenatally and repaired by esophagoesophagostomy with stable postoperative course. The patient was born at 34 weeks gestation, after ultrasonography at 22 weeks gestation showed possible right-sided diaphragmatic eventration or PA and EA was subsequently suspected due to hydramnios. The initial X-ray showed mediastinal shift to the right, and coil up sign of the nasogastric tube, without intracardiac anomaly. Immediately after the diagnosis of EA/TEF and unilateral PA on day 0, the patient was intubated in the operating room, and a gastrostomy tube was placed. After pulmonary status stabilized, at 4 days old, EA/TEF was repaired through a thoracotomy in the right 4 th intercostal space. The right main bronchus was noted to continue into the distal oesophagus; this fistula was ligated and divided, and a single-layer esophagoesophagostomy was performed under mild tension with one vertebral gap. The neonate was maintained on mechanical ventilation and gradually weaned to extubation at 7 days old. The postoperative course was uneventful, with the exception of prolonged jaundice that emerged at 3 months old. Laparoscopic cholangiography at that time excluded biliary atresia, and jaundice resolved spontaneously. The patient has not shown any respiratory symptoms or feeding difficulties as of the 12-month follow-up.

Des-gamma-carboxyprothrombin (PIVKA-II) levels in maternal serum throughout gestation.

The status of vitamin K in pregnant women was investigated using the highly sensitive method for des-gamma-carboxyprothrombin (protein induced by vitamin K absence [PIVKA-II]), electrochemiluminescence immunoassay. A gradual elevation of PIVKA-II related to gestational weeks was observed in healthy pregnant women, suggesting that a modest vitamin K deficiency takes place in gestation. Furthermore, throughout gestation the majority of pregnant women exceeded the healthy adult levels in PIVKA-II. Among complicated gestations of preeclampsia, a remarkable elevation of PIVKA-II was observed in severe preeclampsia, in which a high correlation between PIVKA-II level and coagulation parameters, including thrombin-antithrombin (TAT) complexes, was revealed. These data were suggestive that the vitamin K status readily decreased into a deficient status in hypercoagulative conditions. Among other complicated gestations, a moderate elevation of PIVKA-II was demonstrated in hyperemetic conditions, and, if at all, only a slight elevation of PIVKA-II was observed in other maternal diseases. The present study is the first report regarding the changes of PIVKA-II in pregnant women.

Coagulation index to distinguish severe preeclampsia from normal pregnancy.

The purpose of this study was to establish a new coagulation index to distinguish severe preeclampsia from normal pregnancy using optimal coagulation parameters. The difference between platelet counts in early gestation and before delivery (DeltaPlt = [platelet counts before delivery] - [platelet counts in early gestation]) was calculated as an index of changes in platelet counts. Antithrombin (AT) activity, thrombin-antithrombin (TAT) complex, fibrin degradation products (FDP) D-dimer, and DeltaPlt were investigated in 72 cases of normal pregnant women in the third trimester of pregnancy and 56 cases of severe preeclampsia. The new coagulation index was calculated using multivariate logistic regression analysis. As a result, in a case using four parameters, the following formula was obtained: Y = (-0.019 x AT activity) + (0.067 x TAT) + (0.067 x D-dimer) + (-0.064 x DeltaPlt) + 0.706. According to this formula, coagulation indices in normal pregnant women and in patients with severe preeclampsia were -0.77 +/- 0.51 and 1.41 +/- 1.56, respectively (p < 0.0001). Among patients with severe preeclampsia, coagulation and fibrinolysis disorders before delivery were typical in patients terminated by cesarean section (coagulation index = 1.62 +/- 1.66) compared with those with successful vaginal delivery (coagulation index = 0.52 +/- 0.34) (p < 0.05). These facts suggest that an excessive hypercoagulable state is associated with the termination of pregnancy resulting from the aggravation of preeclampsia. From the viewpoint of coagulation and fibrinolysis disorders, the predictive value in order to decide the optimal time for the termination of pregnancy could be recommended when the coagulation index exceeded 1.20.

Anticholinergics induce eclamptic seizures.

Severe preeclampsia is a pathophysiological disorder specific to pregnancy and characterized by vasoconstriction and hypercoagulability. Eclampsia (convulsion associated with preeclampsia) and hemolysis, elevated liver enzymes, and low platelet count associated with preeclampsia (HELLP syndrome) are serious complications in patients with severe preeclampsia. They are thought to be characterized by generalized vasoconstriction and reduction in blood flow to various organs that may be explained by increased sensitivity of the vascular smooth muscles and increased vasopressors. Liver involvement in eclampsia and preeclampsia is referred to as HELLP syndrome, and epigastric and right upper quadrant pain is often a symptom of severe preeclampsia and may be indicative of imminent convulsions. In addition, marked dilatation of the stomach and the colon is often demonstrated in these patients. These phenomena result from the hyperactivity of the sympathetic nervous system but are not caused by the hyperactivity of the parasympathetic nervous system. The authors experienced two cases of eclamptic seizures after the administration of an anticholinergic (scopolamine butylbromide) in patients with severe preeclampsia complicated by HELLP syndrome. Anticholinergics, blocking agents of the parasympathetic nervous system, can enhance the hyperactivity of the sympathetic nervous system; therefore, vasospasms of the vessels may be easily aggravated, and eclamptic seizures may be induced in patients with severe preeclampsia, especially in those complicated by HELLP syndrome. The administration of anticholinergics should be avoided in patients with severe preeclampsia, especially when there is epigastralgia.

Improvement of vitamin K status of breastfeeding infants with maternal supplement of vitamin K2 (MK40).

The present study is aimed at evaluating the efficacy of maternal vitamin K2 supplementation on the vitamin K status of newborn infants using the measurement of des-gamma-carboxyprothrombin (PIVKA-II [protein induced by vitamin K absence]) and the hepaplastin test (HPT). PIVKA-II and HPT were measured at the 1st month of age in two groups: 31 infants with maternal vitamin K supplementation (15 mg/d Menatetrenone since the 14th day after parturition) (group 1) and 46 without maternal supplementation (group 2). All infants received vitamin K2 syrup twice within the 1st week of life. The PIVKA-II levels of 31 infants (group 1) were 23.6 mAU/mL (standard deviation [SD] 5.8), showing extremely low levels, and close to healthy adult levels, with a smaller deviation than what was seen in group 2. The levels of the 46 infants in group 2 were 27.8 (SD 16.0). This does not differ significantly from group 1, but a small number of infants showed a modestly high level in PIVKA-II. There also was no significant difference between the two groups in the HPT. These data would indicate that maternal vitamin K supplementation can maintain the vitamin K status throughout the late neonatal period and prevent an onset of vitamin K-deficient hemorrhage.