[Establishment of a Neonatal ECMO Programme on the Basis of a Special Training Programme--A 6-year Analysis]. / Etablierung neonataler ECMO bei Lungenversagen auf der Grundlage eines speziellen Trainingsprogramms--Eine 6-Jahres-Analyse.

BACKGROUND: Internationally the need for neonatal ECMO is decreasing and the Extracorporeal Life Support Organization (ELSO) recommends that centres providing neonatal ECMO should treat at least 6 children per year. METHOD: After a one-year training programme and preparation of the clinical application, neonatal ECMO was established and subsequently 41 infants [median age 1 day (1-172 days), median weight 3.25 kg (1.27-5.79 kg)] with severe respiratory failure have been treated within a 6-year period (fall 2008-fall 2014). For rescue therapy we provide inhaled nitric oxide, high-frequency oscillation and other differentiated ventilator strategies. Parallel to the clinical use of ECMO all employees have been trained in a special programme at 3-monthly intervals. RESULTS: By establishing an elaborate training programme and concentrating the treatment of critically ill newborns in one centre, the expertise of both running and preventing of neonatal ECMO due to pulmonary failure can be achieved. The diagnoses correlate to those of other centres which perform neonatal ECMO. 13 infants needed ECMO. The resulting overall survival rate was 11/12 (91.7%) infants treated with ECMO with a curative approach. All patients could be weaned from ECMO. CONCLUSION: In the context of a specialised university hospital with all treatment options for critically ill newborns and with the establishment of a specialised training programme, neonatal ECMO for pulmonary failure can achieve equally good results in comparison to those of national and international ECMO centres.

Poor sleep quality is associated with impaired glucose tolerance in women after gestational diabetes.

We analyzed the association of sleep quality and glucose metabolism in women after gestational diabetes (pGDM) and in women after normoglycemic pregnancy (controls). Data during pregnancy and a visit within the first 15 months after delivery were collected from 61 pGDM and 30 controls in a prospective cohort study. This included a medical history, physical examination, questionnaires (Pittsburgh Sleep Quality Index (PSQI), and Perceived Stress Scale (PSS)), and 5-point oral glucose tolerance test with insulin measurements to determine indices of insulin sensitivity and insulin secretion. We used Spearman correlation coefficients and multivariate regression models for analysis.9.3 ± 3.2 months after delivery, pGDM had significantly higher fasting and 2 h glucose levels and lower insulin sensitivity than controls. There was no significant difference in age, BMI and sleep quality as assessed with the PSQI between the two groups. The PSQI score correlated with the ogtt-2 h plasma glucose in pGDM (δ = 0.41; p = 0.0012), but not in controls. This association was confirmed with a multivariate linear regression model with adjustment for age, BMI and months post-delivery. Perceived stress was an independent risk factor (OR 1.12; 95% CI 1.02-1.23) for impaired sleep. Our findings suggest that post-delivery sleep quality significantly influences glucose tolerance in women after GDM and that impaired sleep is associated with increased stress perception. Measures to improve of sleep quality and reduce perceived stress should therefore be tested as additional strategies to prevent progression to type 2 diabetes after GDM.

Gestational diabetes in Turkmenistan: implementation of a screening program and first results.

PURPOSE: Data on the prevalence of gestational diabetes (GDM) is not available for Turkmenistan or any other central Asian country with large energy resources and rapidly increasing wealth and rates of obesity. We initiated a screening program to determine the prevalence of and the risk factors for GDM in Turkmenistan. METHODS: Between March 2008 and March 2011, all pregnant women presenting to the Ene-Maehri-Merkezi perinatal center in Ashgabat before week 34 of pregnancy received a glucose screening test (after 26 weeks of pregnancy; 50 g glucose). If 60-min glucose was ≥7.8 mmol/l, an oral glucose tolerance test (oGTT) (75gr) was performed. GDM was diagnosed if ≥1 glucose values were abnormal (≥5.0, ≥10.0, ≥8.0 mmol/l at 0-, 60-, 120-min, respectively). Birth weight, 30 min glucose, and APGAR (1, 5, and 10 min) were recorded for all newborns. RESULTS: Of 1,738 women, 22.7 % had a pathological screening test. 70 % of these, underwent an oGTT and of these, 39.5 % had GDM (overall prevalence 6.3 %). Age, BMI, parity, and blood pressure were associated with screening glucose (all p < 0.001). In a multivariate analysis, age, BMI, and family history for diabetes were associated with GDM. Newborns from affected mothers were heavier (3,622 ± 435 vs. 3,480 ± 464 g, p = 0.007) and developed postnatal hypoglycaemia more often (21.6 vs. 9.3 %, p = 0.001), while there was no difference in APGAR. CONCLUSIONS: GDM is a relevant problem in Turkmenistan and probably also in other central Asian countries. The prevalence is similar to other developing countries such as India or China. Risk factors are comparable to those determined in other parts of the world.

[Peridural anaesthesia with ropivacaine for a patient with Friedrich's ataxia. Caesarean section after dorsal stabilisation of the spinal column (Th5-L1)]. / Periduralanästhesie mit Ropivacain bei Friedreich-Ataxie. Sectio bei einer Patientin nach dorsaler Stabilisierung der Wirbelsäule (Th5-L1).

Friedreich's ataxia (FA) is a hereditary disease, which leads to degenerative changes in the spinal cord and cerebellum (incidence 1:50,000). These changes are caused by a defect in the gene that encodes a mitochondrial gene called frataxin and causes muscle weakness, scoliosis, cardiomyopathy and impaired glucose tolerance. Therefore, these patients require special care during anaesthesia. We report the case of a 25-year-old primigravida with a history of FA and dorsal stabilisation of the vertebral column, who was admitted to our hospital for elective caesarean section. Due to increased sensitivity to muscle relaxants, peridural anaesthesia with 8 ml 0.75% ropivacaine and 10 microg sufentanil was used in this case. The perioperative neurological consultation revealed no undue exacerbation of symptoms.

Three-dimensional pelvimetry by computed tomography.

PURPOSE: This study was undertaken to assess the agreement of computed tomography (CT) pelvimetry with different postprocessing techniques. MATERIALS AND METHODS: CT data sets of 25 patients were retrospectively analysed. There were no CT examinations performed solely for pelvimetry, and there was no radiation exposure for study purposes. Six pelvimetric measurements were obtained by two independent observers in four data sets of each patient, i.e. on biplanar topograms, multiplanar reconstructions of 1-mm slices, volume-rendered images of the same data and volume-rendered images based on 5-mm-thick images. Interobserver agreement and variability were determined by Bland-Altman analysis. A human skeleton was also scanned and measured with the same techniques and by ruler as reference. RESULTS: With a correlation coefficient of 0.98, interobserver agreement was best for assessing 3D volume-rendered images reconstructed from 1-mm-thick slices. Interobserver variability was very good for sagittal outlet and midpelvic diameter, transverse inlet diameter and obstetric conjugate (correlation coefficients 0.96-0.99) but limited for intertuberous and interspinous distance. CT and ruler measurements of the skeleton showed excellent agreement. CONCLUSIONS: Pelvimetry can be obtained with low interobserver variability on 3D volume-rendered CT reconstructions. Thus, CT pelvimetry is suitable to gain exact knowledge of pelvic anatomy to identify relevant parameters for dystocia in retrospective studies.

Crash emergency cesarean section: decision-to-delivery interval under 30 min and its effect on Apgar and umbilical artery pH.

PURPOSE: The objective of this study was to investigate the effect of decision-to-delivery interval of crash emergency cesarean section on Apgar and umbilical artery pH in a level-3 university hospital. MATERIALS AND METHODS: In a retrospective analysis, all women undergoing "crash" emergency cesarean section were evaluated. Emergency cesarean sections were performed in the delivery room. Data relating to indication, Apgar score, arterial cord pH, and time intervals between decision-to-deliver and actual delivery were collected retrospectively. RESULTS: All 109 crash emergency cesarean sections were performed within a decision-to-delivery time of 30 min. The median (with 10-90th percentile) time was 10 min (5-19). Thirty-three (30.3%) of the emergency cesarean sections had a gestational age below 32 weeks and 60 (55%) below 37 weeks. An abnormal fetal heart rate pattern was noted in most of the cases (91%). Prolapsed cord (21%) and placental abruption (20%) were the most frequent reasons for emergency cesarean section but in one-fourth (25.7%) no morphological reason could be identified. Very short decision-to-delivery times below 20 min were inversely correlated to fetal outcome, i.e., lower umbilical blood pH and Apgar scores (P < 0.01). CONCLUSION: The 30-min standard for the decision-to-delivery time interval set by Anglo-American countries may be a feasible guideline at least for level-3 hospitals. The 20-min interval set by the German Society of Gynecology and Obstetrics could not be achieved in all cases. The positive effect of very short intervals on neonatal outcome still needs to be proven.

Myomectomy as a pregnancy-preserving option in the carefully selected patient.

OBJECTIVES: To present the indications for myomectomy during pregnancy and to discuss complications possibly related and unrelated to the procedure. METHOD AND RESULTS: A 33-year-old patient at 18 weeks of gestation underwent removal of a 1,570-gram symptomatic fundic myoma. Histologically the patient had a leiomyomatous neoplasm of uncertain malignant potential. The pregnancy was continued under sequential observation with magnetic resonance imaging and ultrasound. At 36 weeks of gestation a healthy girl with an upper extremity limb defect was born via cesarean section. Follow-up of the mother and the child was uneventful. CONCLUSIONS: Certain known risk factors in pregnant women with myomas can predispose to complications during pregnancy. Women with such risk factors or women who have failed medical therapy should be offered the option of undergoing myomectomy as a pregnancy-preserving procedure.

[Paravaginal abscess in pregnancy]. / Paravaginaler Abszess in der Schwangerschaft.

Paravaginal abscess in pregnancy. We report the diagnosis and treatment of an infected Gartner's duct cyst during pregnancy. The patient presented with lower abdominal pain, fever (38.5 degrees C) and an elevated C-reactive Protein level. Pelvic examination revealed a painful paravaginal mass. Sonography was not able to detect the cranial border of the tumor. Magnetic resonance imaging (MRI) revealed fluid accumulation laterodorsal to the vagina without evidence of a connection with the retroperitoneal space. An infected Gartner's duct cyst with consecutive abscess formation along the mesonephric duct system, was diagnosed. Following transvaginal drainage, the remainder of the pregnancy was uneventful and the patient was delivered vaginally at 40 + 5 weeks without complications. - The rare clinical finding of a paravaginal abscess in pregnancy was treated without termination of the pregnancy. Preoperative planning of the surgical approach using MRI can be easier for pelvic processes extending out of the pelvis than using ultrasound and is less painful for the patient.

[Prune belly syndrome and congenital kidney tumors]. / Prune-Belly-Syndrom und kongenitale Nierentumoren.

BACKGROUND: High end sonography allows the prenatal localization of the kidneys and the corresponding urine drainage system as early as 10-13 weeks of gestation. In mid second trimester, the voiding and filling of the urinary bladder can be demonstrated by ultrasound. Obstructions are the most common abnormalities of the urogenital tract. Though less frequent in incidence, more complex sequences of anomalies such as Prune Belly Syndrome or Megacystis-Microcolon-Intestinal-Hypoperistalsis-Syndrome (MMIHS) can also be detected in early gestational age. MATERIALS AND METHODS: Pathogenesis, prenatal diagnosis, pre- and postnatal treatment options and prognosis are discussed. RESULTS AND DISCUSSION: The same risk-adapted procedures aimed to protect the fetal urinary excretory function known in the therapeutic regimen of obstructive uropathy are available as treatment options. These range from non-invasive ultrasound for diagnosis and surveillance to needle procedures or even endoscopic interventions. Another rare entity of renal abnormalities are congenital neoplasm's--megaloblastic nephroma, nephroblastoma and neuroblastoma. CONCLUSION: Prognosis and obstetrical management are to be determined individually for each patient.

Growth and development of children to 4 years of age after repeated antenatal steroid administration.

UNLABELLED: It is common practice to repeat antenatal steroid administration after 7 to 10 days in women who continue to be at risk for preterm delivery. However, safety and efficacy of repeated courses have not been established. Mothers of singleton infants who had more than five courses of betamethasone (80-120 mg cumulative dose) were eligible for this cohort study. Index patients (IP) were compared to concurrent controls who had < or = 1 course but were matched for sex and gestational age. Of 35 IP born between 1986 and 1995 in a single perinatal centre, 28 were available for follow-up and could be matched. There was no difference between groups with respect to maternal age and gestational age at delivery. Median gestational age at initial treatment was 26.3 weeks (25th percentile 25.1 weeks, 75th percentile 27.2 weeks) in IP. There was no significant difference between groups in head circumference, length and body weight at birth and at age 4 years. The ability to sit and to walk without assistance and to use two-word phrases was attained at similar ages. The use of glasses or hearing aids, allergies, asthma or recurrent upper respiratory infections were not reported more frequently in IP. CONCLUSION: This study failed to ascertain adverse long-term effects of repeated antenatal steroid administration in infants and children to the age of 4 years. In contrast to a similar Australian study, we were unable to demonstrate a lower birth size in exposed infants even though our sample size for women with more than five courses and their cumulative doses were larger.

Telemedicine in ultrasound: new solutions.

The transport of dynamic ultrasound (US) pictures as video files or streams no longer presents a problem. Despite teleconferencing, this method of transfer provides no active influence on the data sent for the receiving expert. We are evaluating a software-based 3-D US system that provides the new opportunity of an active US re-evaluation of a virtual patient. The 3-D volumes can be reanalyzed, time- and examiner-independent, wherever the device is installed. To test the clinical feasibility of this virtual US investigation, we installed the device in a peripheral hospital and in our US unit. The transfer of the volume (about 10 to 15 MB) could be done with two parallel Integrated Services Digital Network (ISDN) lines with a conduction capacity of 128 KB/s. In this basic assessment, the transmission of US volumes of patients proved to be easy in the acquisition, quick in off-line transmission and reliable in off-line re-evaluation of the data.

S680N substitution of the follicle-stimulating hormone receptor is a common polymorphism not associated with spontaneous human twinning.

OBJECTIVE: To determine the influence of the A307/S680 and T307/N680 isoforms of the follicle-stimulating hormone receptor (FSHR) gene on the incidence of spontaneous human twinning. DESIGN: Case-control study. SETTING: Departments of Obstetrics and Gynecology and of Clinical Chemistry-Grosshadern, University Hospital Munich, Germany. PATIENT(S): Fifty-four mothers with dichorionic twin pregnancies and 92 singleton mothers as controls, who had conceived without assisted reproduction. INTERVENTION(S): Exon 10 of the FSHR gene was screened for the G2105A/S680N mutation. MAIN OUTCOME MEASURE(S): Amplification of genomic DNA by the polymerase chain reaction followed by restriction fragment length polymorphism analysis. RESULT(S): Allele frequencies for the G2105A/ S680N substitution of the FSHR in twin mothers were not different from those of controls (genotype (isoform) [twins vs. controls]: G/G (S/S) [24.1% vs. 22.3%]; A/G (N/S) [57.4% vs. 55.4%]; A/A (N/N) [18.5% vs. 22.3%]). Subgroup analysis of women with three or more successful pregnancies gave a similar result (G/G (S/S) [17.7% vs. 13.7%]; A/G (N/S) [64.7% vs. 63.6%]; A/A (N/N) [17.6% vs. 22.7%]). There was no correlation between FSHR isoform and twinning. CONCLUSION(S): The S680N substitution of the follicle-stimulating hormone receptor is a common polymorphism not associated with spontaneous human twinning.

Thrombophilic gene mutations and recurrent spontaneous abortion: prothrombin mutation increases the risk in the first trimester.

PROBLEM: Thrombophilic predisposition may be one of the underlying causes of recurrent spontaneous abortions (RSA). We studied the prevalence of five thrombophilic gene mutations in patients with RSA. METHOD OF STUDY: 102 patients with two or more consecutive abortions and 128 women without miscarriage were analyzed for factor V Leiden mutation (FVL), prothrombin G20210A mutation (PTM), C677T mutation in the 5,10-methylenetetrahydrofolate reductase (MTHFR) gene, glycoprotein IIIa (GPIIIa) C1565T polymorphism, and beta-fibrinogen G-455A polymorphism by polymerase chain reaction (PCR) techniques. RESULTS: No differences in the prevalence of FVL, MTHFR T/T, GPIIIa and 1-fibrinogen polymorphism were detected. Heterozygous PTM occurred more often in patients with RSA. This effect was significant in a subgroup with abortions exclusively in the first trimester (6.7%, vs. 0.8%, P = 0.027, OR 8.5). CONCLUSIONS: In contrast to the other mutations and polymorphisms, heterozygous PTM is more common in patients with abortions in the first trimester. This might reflect an influence of PTM on pathogenesis of early pregnancy loss.

[Fetal obstructive uropathy--diagnosis and possible treatments]. / Fetale obstruktive Uropathie--Diagnose- und Behandlungsmöglichkeiten.

BACKGROUND: Abnormalities of the urinary tract are found in up to 5% of newborns. They account for 25% of all prenatally diagnosed congenital defects and contribute 4% to perinatal mortality. When considering the prognosis of these anomalies, association with other pathological conditions has to be taken into account. MATERIALS AND METHODS: We discuss the state of the art in detection and management of fetal urinary tract abnormalities as are standard at this tertiary center of fetal medicine. RESULTS AND DISCUSSION: Posterior urethral valve, megaureter or ureteropelvic junction obstruction represent one end of a wide spectrum of obstructive uropathies, ranging from pathophysiologically minor deviations to severe impairment of renal function. Organ development, physiology of urine production and excretion as well as their potential disorders are presented. CONCLUSION: Sonographic and invasive diagnostic options in the evaluation of the urogenital tract are discussed. Careful assessment of each individual case and prognosis determine pre- and postnatal treatment.

[Prenatal diagnosis of kidney parenchyma diseases]. / Vorgeburtliche Diagnostik von Nierenparenchymerkrankungen.

BACKGROUND: Aside from congenital anomalies of the urogenital tract, fetal renal tissue itself can be dysplastic. Prenatal ultrasound allows the differential diagnosis of simple cysts, different degrees of renal dysplasia and agenesis of the kidneys. MATERIALS AND METHODS: This article focuses on the multifactorious complex of parenchymal renal disease. According to the classification of Potter Syndrome and cystic renal dysplasia/Potter Sequences I-IV, pathogenesis, prenatal diagnosis, pre- and postnatal treatment options and prognosis are discussed. RESULTS AND DISCUSSION: Concomitant absence of amniotic fluid frequently refutes diagnosis until artificial amniotic fluid infusion has been performed. Although intrauterine therapy is rarely possible, the frequent association with other abnormalities and fetal syndromes may be of consequence. The sonographic appearance of parenchymal renal disease is heterogeneous. This inconsistency has lead to different classifications. This study uses the "modified Potter Classification" of parenchymal disease in kidneys (Zerres).

The number of dichorionic twin pregnancies is reduced by the common MTHFR 677C-->T mutation.

In multiple pregnancies, demands for folic acid are considerably increased. The most common inborn error of folate metabolism is mild methylenetetrahydrofolate reductase (MTHFR) deficiency due to the synthesis of a thermolabile variant of the enzyme with impaired catalytic activity which leads to reduced 5-methyltetrahydrofolate (5-methyl-THF) and mildly elevated homocysteine plasma concentrations when folate status is inadequate. To investigate whether the number of offspring is influenced by this mutation, we determined the frequency of the 677C-->T substitution in 156 singleton and 40 twin mothers with dichorionic placentation. The T allele frequency in singleton (0.30) and twin mothers (0.16) was significantly different (P = 0.011). Mothers with the 677C-->T mutation had a 2.28 times lower risk of having a twin pregnancy than those without (95% confidence interval = 1.18-4.66; P = 0.008). Our observation would explain, at least in part, the hereditary trait of multiple gestations and is in agreement with the ethnic distribution pattern of the T allele which has been found to be inversely correlated with the incidence of dichorionic twins. Our findings suggest that the MTHFR 677C-->T mutation interferes with human brood size, probably by influencing the proliferation of rapidly dividing embryonic and maternal cells.

Obstetric risks and vertical transmission of hepatitis C virus infection in pregnancy.

BACKGROUND: Reports of obstetric complications of mothers infected with hepatitis C virus (HCV) are limited and the risk of mother-to-infant transmission varies widely. We assessed the course of pregnancy in HCV-infected women and the rate of vertical transmission. METHODS: Between October 1992 and December 1996, 3712 pregnant patients of the university hospital Grosshadern Munich, Germany, were screened for anti-HCV and analyzed for HCV-RNA by polymerase chain reaction. Clinical and biochemical parameters were monitored. Children born to HCV-positive women were followed up at 6, 12 and 18 month intervals and screened for anti-HCV and HCV-RNA. RESULTS: Thirteen (42%) of 31 anti-HCV positive patients had a cesarean section which was twice the rate of that in the HCV-negative group (p=0.004). None of the cesarean deliveries was due to complications directly caused by HCV infection. Nine (29%) of 31 anti-HCV positive women had preterm delivery compared to 19% in the anti-HCV negative patients, the difference being statistically not significant. Fetal outcome parameters such as APGAR score, umbilical pH and birth weight of HCV infected pregnancies were not impaired. All 29 babies tested for anti-HCV were seropositive after birth. Between 12 and 18 months of age, 10% of the infants still were anti-HCV positive, whereas only one baby was HCV-RNA positive beyond 12 months yielding a vertical transmission rate of 5% among HCV-RNA positive mothers. CONCLUSION: Anti-HCV positive pregnancies have an increased risk of cesarean delivery, probably due to the high-risk collective of anti-HCV positive mothers. The mother-to-child transmission rate is low and linked to maternal HCV-RNA positivity.

Intra-uterine fetal demise caused by amniotic band syndrome after standard amniocentesis.

The amniotic band syndrome represents a prime example of exogenous disruption of an otherwise normal fetal development. It may be a sequel of invasive diagnostic procedures such as amniocentesis or fetal blood sampling. A 38-year-old gravida II, para II delivered a morphologically normal male stillborn at term. The pregnancy history had been unremarkable but for an early 2nd-trimester amniocentesis. Cause of the intra-uterine fetal demise was noted to be an amniotic band constricting the umbilical cord. An amniotic band is a rare but potentially fatal condition which may be induced by, e.g., invasive prenatal procedures. Such bands are not usually diagnosed prenatally; however, selected patients with augmented risk may profit from intensive ultrasound evaluation including Doppler studies.