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Aim: To prospectively evaluate the potential role of 3DUS of the neonatal brain., Method: 60 patients from the NICU (gestational age: 25-42 weeks, mean: 31.6 weeks, age: 0 to 90 days, median: 10 days) underwent 2D- and 3D-neurosonography. Both studies were evaluated independently by two observers for comparison. Inter- and intraobserver variability were calculated., Results: All 3DUS were of diagnostic quality and could be performed without sedation. 3DUS missed no essential diagnosis as established by conventional ultrasound (2DUS). Diagnosis included normal or physiologically immature neonatal brains (n = 21), plexus cysts (n = 4), plexus bleedings (n = 10), intraventricular haemorrhages grade I-III (n = 8), periventricular pathology such as periventricular echodensities (n = 4) and periventricular haemorrhages or cerebral infarctions (n = 6), hydrocephalus (n = 4), widened subdural spaces (n = 2) and one suprasellar midline tumour. 3DUS imaging time at the patient (4.8 +/- 2.6 min) was significantly shorter than for 2DUS (9.1 +/- 6.1 min). The additional axial plane provided by 3DUS improved the sonographic potential for differential diagnosis. 3DUS allowed an improved standardisation and documentation potentially valuable for follow-up. No statistically significant differences in intra- and interobserver variability were noted compared to 2DUS. Restrictions of 3DUS were the lack of directional Doppler data and the lower resolution particularly of the purely reconstructed plane., Conclusion: Bedside neonatal 3D-neurosonography at the NICU is feasible with diagnostic quality without sedation. 3DUS improves comparison during follow-up, as well as standardisation and documentation, and can be considered a useful adjunct in neonatal 2D-neurosonography.

Unlabelled: Meningococcal disease may present as sepsis, meningitis or a combination of both. Impaired fibrinolysis and massive elevation of the plasminogen activator inhibitor-1 (PAI-1) is a characteristic feature of meningococcal sepsis. Previously, an association between mortality and the functional 4G/5G promoter polymorphism of the PAI-1gene in a cohort of UK and Dutch children with meningococcal sepsis was reported. We carried out a prospective, multicentre study to investigate the association of the 4G/5G PAI-1 polymorphism, diagnosis, and outcome in meningococcal disease in a Central European and UK population. Blood samples and clinical information of 347 previously healthy children with meningococcal infection were collected from 95 paediatric hospitals in Germany, Switzerland, Italy, the United Kingdom, and Austria from 2000 until 2002. Mortality was significantly associated with the 4G/4G genotype (12 of 90 (13%) vs. 15 of 240 (6%), P = 0.037), resulting in an odds ratio of 2.31. The diagnosis of sepsis (independent of symptoms of meningitis) was significantly more frequent in carriers of the 4G/4G genotype (P = 0.01), resulting in an odds ratio of 2.21 to develop sepsis. Meningitis was not associated with the PAI-1 4G/5G polymorphism, and allele frequencies were similar in patient and control groups., Conclusion: Our data show a correlation between the 4G/4G genotype in the plasminogen activator inhibitor-1 gene and poor outcome in children with meningococcal infection. In addition, 4G homozygous patients were prone to develop sepsis. We found no influence of the plasminogen activator inhibitor-1 polymorphism on the susceptibility to invasive meningococcal infection.

Background: To report immediate and long-term outcome in patients with surgical uterine devascularization for placenta accreta., Methods: Six patients with placenta accreta were treated conservatively during a cesarean section by a bilateral uterine and ovarian surgical devascularization procedure. Menstrual flow, imaging monitoring and further pregnancy were retrospectively reported., Results: Blood transfusion was necessary in five cases and a hysterectomy was performed in one patient with placenta previa accreta. All patients resumed menstruation without oral contraception but one of them reported temporary clinical symptoms of estrogen insufficiency. A chronic placental retention occurred in three patients with incomplete placenta removal. One patient with both bilateral uterine and ovarian arterial ligations had a subsequent pregnancy complicated by a recurrent placenta accreta that was subsequently treated conservatively., Conclusions: Surgical uterine devascularization for placenta accreta may be useful for uterine conservation. However, reproductive capacity may be altered by placental chronic retention and further pregnancies may be complicated by recurrent placenta accreta.

Aim of the Study: To evaluate the influence of three common thrombophilic polymorphisms, factor V Leiden (FV), prothrombin G20210A (PT), and methylenetetrahydrofolate reductase (MTHFR) C677T mutations, on preterm birth of unknown cause., Patients and Methods: A single-centre case-control study of women with preterm infants < or =35 weeks of gestation, in whom obvious maternal, uterine, and fetal causes responsible for preterm birth were excluded (n = 35). The controls were 54 women with term infants hospitalised in the same ward., Results: There were no significant differences between the groups of mothers in history of fetal loss, venous or familial thrombosis, or previous preterm birth. FV was found in 8.6% of the cases, PT in 5.7%, and MTHFR mutation (homozygous) in 4.8% compared with 5.4% (p=0.292, OR 1.594, CI95% 0.303-8.384), 7.4% (p=0.379, OR 0.758, CI95% 0.131-4.374), and 4.5% (p = 0.485, OR 1.050, CI95% 0.090-12.276), respectively, in the controls. Differences in the three thrombophilic polymorphisms in the two groups of infants were also not significant., Conclusion: We could not demonstrate a distinct association between these thrombophilic polymorphisms and preterm birth.

Objective: To identify risk factors for the development of cystic periventricular leucomalacia (PVL) in twin gestation., Design: Retrospective case-control study., Setting: Tertiary care university hospital, Department of Paediatrics, Division of Neonatology, Graz, Austria., Patients: Preterm twin gestations with one sibling having developed cystic PVL, diagnosed by ultrasound scans, compared with their co-twins without PVL, in hospital between 1988 and 2000., Main Outcome Measures: Perinatal and postnatal risk factors for the development of PVL., Results: Eighteen preterm twin gestations were included. Monochorionicity was evident in 47% of the pregnancies, and twin to twin transfusion syndrome occurred in two cases (11%). Fetal distress correlated inversely with PVL (15% v 53%, p = 0.019, relative risk (RR) = 2.057, 95% confidence interval (CI) = 1.067 to 3.968). Hypocarbia with Pco(2) levels below 30 mm Hg (4 kPa) was diagnosed in 29% of the cases compared with 6% of the controls (p = 0.038, RR = 1.944, 95% CI = 1.113 to 3.396). There were no significant differences between groups with regard to premature rupture of the membranes, early onset infection, respiratory distress syndrome, mechanical ventilation, arterial hypotension, persistent ductus arteriosus, and hyperbilirubinaemia. Asphyxia was only evident in three controls. Three infants died and another three were lost to follow up. None of the cases compared with 62% of the controls were diagnosed as having developed normally (p < 0.001), and 14 cases (82%) compared with two controls (15%) developed cerebral palsy (p < 0.001)., Conclusion: Hypocarbia was the only risk factor strongly associated with cystic PVL. The general outcome of the infants was poor.

Objective: Update of knowledge on the various methods of management of intractable postpartum haemorrhage., Method: PubMed, MEDLINE were the electronic sources, in English and French languages, used for data retrieval. Uterine atony and abnormal placental insertions (placenta praevia or accreta) are the major causes of primary postpartum haemorrhages. To preserve fertility, we dispose of angiographic selective embolization or surgical vascular ligations. Embolization is a non-invasive method practicable by simple catheterization under local anesthesia. Vascular ligations of the uterine vessels or internal iliac arteries require mostly laparotomy. New and easier surgical methods, such as uterine compression or hemostatic suturing techniques have been described for which we lack experience., Results: For uterine atony, the success rate of arterial embolization and uterine artery ligations is close to 100%. Ligation of internal iliac arteries is a little less effective and technically more difficult to carry out. It remains interesting in obstetrical traumatic hurts, which do not concern the uterus. If bleeding from the lower segment occurs during caesarean section, low uterine artery ligatures are necessary. These methods are all the more effective than they are prematurely implemented before the rise of major coagulopathy. In this case, uterine devascularization has also to be applied to ovarian vessels. With placenta accreta, accreta portion of the placenta can be left in place and arterial embolization or vascular ligations can be done. Nevertheless the main cause of failure with conservative treatments is placenta accreta., Conclusion: The simplest and the least morbid methods must be retained. After vaginal birth, arterial embolization can be done, if there is no maternal haemodynamic disorder nor interventional vascular radiology unit nearby. During caesarean section, progressive uterine artery ligation can be done adapted to the bleeding cause. In case of failure of a conservative treatment, it would be dangerous to multiply techniques. Emergency peripartum then should remain the choice procedure.

The authors report on a fatal case of hemolytic disease of the newborn (HDN) due to anti-K antibodies with subsequent trilineage pancytopenia in a preterm infant of 28 weeks gestational age, with pronounced leukopenia and neutropenia. In addition, molecular typing of the Kk polymorphism was necessary to confirm HDN. This case of HDN associated with anti-K provides additional evidence that trilineage pancytopenia due to suppressed hematopoiesis is part of the disease. Therefore, antibodies against antigens of the Kell blood group system should be considered as a potential cause of unexplained inhibition of myelopoiesis.

Purpose: To analyze the precision of cerebral blood flow (CBF) measurements made with continuous arterial spin labeling(CASL) perfusion magnetic resonance imaging (MRI) over experimentally relevant intervals., Materials and Methods: CASL perfusion MRI measurements of CBF on a 1.5-T GE Signa magnet were repeated in young healthy male and female subjects at one hour and one week. Precision of the measurement was evaluated at both time intervals., Results: CASL perfusion MRI measurements of CBF yielded within-subject coefficients of variation (wsCV) of 5.8% for global and 13% for individual vascular regions when measurements were repeated within one hour. Differences in these values represent the error in post-processing. Global and regional CBF measurements over one week yielded wsCVs of 13% and 14%, respectively. At one week, error secondary to physiologic variability affected global and regional measurements to the same degree and masked the software post-processing error seen at one hour. The magnitude of the difference in repeated measures correlated with the magnitude of the measurement., Conclusion: CASL perfusion MRI CBF measurements are accurate and precise. Variability over longer periods of time appears attributable to physiologic factors. Repeatability of the CASL measurement is sensitive to the magnitude of the measurement. This should be taken into account when studies requiring repeated measures involve subjects with significant variability in CBF., (Copyright 2003 Wiley-Liss, Inc.)

Background: Inhaled nitric oxide (iNO) is used as a vasodilator in pulmonary hypertension (PH) of the newborn infant., Patients and Methods: Retrospective analysis of patients, who were treated at our department with iNO in the period from 1994-2001. Response was defined as an increase of the paO (2)/FiO (2) Ratio > or = 20 % and/or a decrease of the oxygenation index (OI) >/= 20 % after 2 h (early response), and consecutively after 24 h (late response). The patients were divided into a) primary persistent pulmonary hypertension of the newbom (PPHN), or b) pulmonary hypertension secondary to meconium aspiration syndrome (MAS), sepsis or congenital diaphragmatic hernia (CDH)., Results: Between 1994 and 2001 we treated 47 patients with iNO at our neonatal intensive care unit. We included 16 (35 %) preterm infants (GA 34,5 [25 - 37] weeks, GG 2061 [680 - 3410] g) (Median/Range) and 31 (65 %) newbom (GA 40 [38 - 42] weeks, GG 3510 [2550 - 4560] g). 18 (38 %) patients suffered from primary PPHN, 29 (62 %) from secondary PPHN (14 MAS [30 %], 8 sepsis [17 %], 4 CDH [8 %]). 8 (50 %) preterm and 20 (64 %) term infants showed a positive iNO response after 2 h, again 8 (50 %) preterm and 20 (64%) term infants showed a positive iNO response after 24 h. There was neither a significant difference between term and preterm infants at 2 h, nor at 24 h. Between 2 h and 24 h 10 patients changed in their response to iNO. 5 (18 %) patients with early response showed a significant degradation after 24 h, whereas 5 (26 %) of the patients without early response showed a significant improvement of the oxygenation alter 24 h. Alltogether 13 (72 %) patients with PPHN, 8 (57 %) with MAS, 2 (50 %) with CDH, 4 (50 %) with sepsis showed a positive iNO response after 24 h. In regard to the oxygenation parameters at start of iNO-therapy, the patients with early response did not differ from the patient without response (median OI: 20,0 versus 21,8, median paO (2)/FiO (2) Ratio: 59,3 versus 55,0 mmHg at the start of the iNO therapy)., Conclusion: In regard to iNO response, there was no significant difference between term and preterm infants. Due to the changing response, a positive iNO-response after 2 h had no predictive value for the further prognosis of the oxygenation situation under iNO therapy.

The aim of this study was to describe the potential of three-dimensional ultrasound (3D US) in paediatric and neonatal neurosonography. The potential applications are illustrated based on our experience in 150 patients using three different 3D US techniques at two different sites. Various disease entities throughout the paediatric age have been evaluated. The potential of 3D US, including 3D US of the cerebral vessels based on colour Doppler data, is discussed based on comparison with conventional 2D US or other imaging (as available), and with regard to the literature. In our experience, 3D US is feasible in neonatal and paediatric neurosonography. It reduces imaging time, improves demonstration of complex anatomy and vasculature, and allows for evaluation of anatomy/pathology in any plane. The 3D US furthermore improves volume assessment (e.g. in hydrocephalus), and comparison with CT, MRI and during follow-up, with a potentially improved standardisation and documentation. The 3D US additionally offers an ideal modality for training and education, as the brain and the neonatal spine can be virtually rescanned at the workstation. Yet, limitations such as areas inaccessible to 2D US, limited resolution and motion artefacts have to be acknowledged. Three-dimensional US has the potential to become a valuable additional imaging tool in paediatric neurosonography.

Unlabelled: The reliability of procalcitonin (PCT) and interleukin-6 (IL-6) was determined and compared with that of C-reactive protein (CRP) in the diagnosis of early-onset sepsis of the neonate within the first 12 h of life. ROC analysis of values of 41 neonates with blood-cultures-positive and clinical sepsis compared with those of 27 uninfected neonates revealed sensitivities for PCT (> or = 6 ng/mL), IL-6 (> or = 60 pg/mL), and CRP (> or = 2.5 mg/L) of 77%, 54%, and 69% and specificities of 91%, 100% and 96%, respectively. Sensitivity of CRP at > or = 8 mg/L was 49% (p = 0.012 compared to PCT)., Conclusion: PCT was the most sensitive diagnostic parameter in the diagnosis of early-onset sepsis within 12 h of life.

Objective: The object of this study was to evaluate the potential of amplitude-coded colour Doppler sonography (aCDS) in paediatric neurosonography from the aspects of clinical value and impact., Patients and Methods: Sixty neonates, infants, and children underwent 72 aCDS investigations after conventional ultrasound (US) studies including conventional colour Doppler. Their diagnoses included tumours, hydrocephalus and cerebral fluid collections, cerebral vascular disorders, cerebral bleeds and hypoxic-ischaemic lesions. The results of aCDS were prospectively compared with the findings obtained with conventional US findings, with CT/MRI results when available (34 patients with 41 investigations), with operative findings (7 patients), and with clinical, laboratory, EEG and follow-up results., Results: Six patients showed normal findings. Fourteen cerebral bleeds were correctly recognised by both conventional US and aCDS; plexus haemorrhage was depicted only on aCDS in 4 additional neonates. In 4 of 6 patients with tumours and vascular malformations aCDS revealed additional information. aCDS improved early detection of focal ischaemic lesions in 10 of 13 cases and enabled Duplex sampling in 7 of 9 children with diffuse hypoxic brain injury. In 5 of 20 patients with hydrocephalus and/or fluid collections aCDS proved helpful (e.g. demonstration of liquor fistula), yielding a sensitivity of 93.75%, as against 43.75% for conventional US., Conclusion: aCDS is helpful in differentiating perfused structures such as a prominent ventricular plexus or tumours from structures without perfused vasculature, such as blood clots or ischaemic lesions. aCDS can be considered a valuable adjunct in the differential diagnosis of haemorrhage and ischaemic lesions as well as of vascular malformations and tumours.

The case of a 16-year-old woman with typical clinical features of acute vulval ulcer, Lipschutz's disease, is reported. The main differential diagnoses are discussed. Isolated vulval ulcer can reveal a recurrent aphthous ulcer, Behçet's disease or Crohn's disease. In contrast, acute vulval ulcer spontaneously heals with no recurrence, that must be taken into account for diagnosis.

Background: We analyzed the influence of respiratory syncytial virus (RSV) on the clinical course and management of infants hospitalized due to viral upper and lower respiratory tract infections (U/LRTI)., Patients and Methods: Infants younger than 2 years were prospectively tested for RSV infection by antigen detection in nasopharyngeal aspirates between November 1999 and October 2000., Results: Of 281 infants hospitalized during the study period, 58 (21%) tested RSV positive. Seasonal distribution of RSV infections showed a peak in March (45% of all U/LRTI). Infants with RSV infection (12% were preterm, 5% had congenital heart disease) were younger (p < 0.001), had more severe U/LRTI (p < 0.001), longer hospitalizations (p < 0.001), more days with oxygen requirement (p < 0.001) and respiratory support (p = 0016) and more frequent requirements for bronchodilators (p = 0.002) and corticosteroids (p = 0.02)., Conclusion: RSV contributed to prolonged hospitalizations and more severe clinical courses of disease both in very young term and preterm infants.

We report a case of congenital bilateral chylothorax, complicated by pulmonary eosinophilic vasculitis and transient eosinophilia. Excluding all known forms of eosinophilic pulmonary disease either histologically or clinically, possible mechanisms of its etiology are discussed. Treatment with corticosteroids was successful, and after a prolonged clinical course the patient recovered by age 2 years., (Copyright 2002 Wiley-Liss, Inc.)

Cystic periventricular leukomalacia refers to necrosis of the white matter in a characteristic distribution dorsal and lateral to the external angles of the lateral ventricles in preterm infants. The pathogenesis includes either hypoxic-ischaemic lesions resulting from impaired perfusion at the vascular border zones or the role of intra-amniotic infection with toxic effects of endotoxins and cytokines on oligodendrocytes. This overview illustrates the pathogenic theories, risk factors, diagnosis by cranial ultrasonography, and the actual classification. Cystic periventricular leukomalacia is the most severe and frequent cause of cerebral palsy in preterm infants and is almost constantly associated with serious subsequent neuromotor impairments such as diplegia or tetraplegia. Dependant on site and extension of the cysts additionally visual impairments, seizure disorders, hearing impairments, mental retardation, and microcephaly are observed.

A 27-year-old woman underwent surgery for an abdominal wall mass later confirmed to be a relapse of Stage I ovarian mucinous adenocarcinoma. The authors stress that caution should be observed during laparotomy to remove a malignant neoplasm in order to avoid parietal dissemination.

Unlabelled: The aim of the study was to determine risk factors for the development of cystic periventricular leucomalacia (PVL) and to correlate ultrasound findings with neurodevelopmental outcome. By means of a retrospective case-control study (matched for gestational age, birth weight, sex, and year of birth) and a cohort analysis of all preterm infants with cystic PVL documented by ultrasound scans hospitalised at a local tertiary care centre between 1988 and 1998, 98 preterm infants with a gestational age ranging from 26 to 35 weeks were diagnosed as having cystic PVL. The mean day of diagnosis of periventricular echodensities was 3 +/- 2 days (range 1-11 days), and of cystic PVL 21 +/- 8 days (range 2-47 days). Of 79 infants (1988-1997) eligible for neurodevelopmental follow-up (91%), hemi-, di-, or tetraplegia was diagnosed in 61 (77%), normal mental outcome in 22 (28%), associated visual disorders in 41 (52%) and seizure disorders in 12 (15%) infants. Significant risk factors associated with the development of cystic PVL were premature rupture of membranes, chorioamnionitis, and hyperbilirubinaemia (odds ratios 4.665, 6.026, and 2.460 respectively). Subgroup analysis according to gestational age (26-28, 29-32, 33-35 weeks) revealed similar results despite spontaneous labour (26-28 weeks; odds ratio 4.808) and pre-eclampsia (33-35 weeks; odds ratio 3.517). Multiple pregnancy was associated with a twofold increased risk (odds ratio 2.075). The white matter damage probably accounted for the significantly higher prevalence of apnoeas (P < 0.001) and neonatal seizures (P < 0.001). Cysts located bilateral or parieto-occipital were associated with a higher risk of cerebral palsy (odds ratios 6.933 and 4.327 respectively). Solely anterior located cysts were associated with normal neurological outcome. Increasing size of the cysts was associated with increasing risk of cerebral palsy with a cut-off value of 10 mm (odds ratio 3.300 and above) and all infants with cysts of more than 20 mm diameter had cerebral palsy., Conclusion: The high prevalence of premature rupture of the membranes and chorioamnionitis further supports the role of intra-uterine infection in the pathogenesis of periventricular leucomalacia. The overall prognosis of cystic periventricular leucomalacia is poor.

Background: Rh system antibodies are commonly encountered in blood bank practice as well as during pregnancy. Nevertheless, no examples of anti-Ce (RH7) have been reported as a cause of HDN that requires exchange transfusion., Case Report: A 38-year-old woman in her fourth pregnancy was typed as blood group O D+, C-, c+, E+, e-. Anti-C and anti-e were detected in her serum during a routine prenatal work-up. Further evaluation, including flow cytometric analysis, revealed the presence of a strong anti-Ce and a weak anti-e. Her partner was typed as group A D+, C+, c-, E-, e+. A seemingly healthy male infant was delivered at 40 weeks of gestation. The infant's RBCs were typed as group O D-, C+, c+, E+, e+ with a positive DAT (titer 128). Twenty-five hours after birth, the baby had to be transferred to the neonatal intensive care unit because of rapidly rising total serum bilirubin. Despite intensive treatment, including double phototherapy, albumin infusion, and the administration of furosemide and IVIG, the total serum bilirubin level increased during the following day and exchange transfusion with 2 units of type O D-, C-, c+, E+, e- had to be performed; this resulted in a prompt decrease in total serum bilirubin without relapse., Conclusion: Anti-Ce caused severe HDN requiring exchange transfusion. This highlights the need for a close follow-up throughout pregnancy if unexpected RBC antibodies are present, to permit the provision of compatible blood in case of a rare antibody.

Respiratory syncytial virus (RSV) causes seasonal epidemics between December and March (April) and remains the main agent that causes severe lower respiratory tract infections in young infants. Children with bronchopulmonary dysplasia up to 24 months of age and preterm infants with a gestational age of 32 weeks and below, who are less than six months of age, are at highest risk for severe RSV infection. RSV-IGIV has been demonstrated to reduce significantly RSV associated hospitalizations, RSV associated hospital days and the incidence of severe RSV lower respiratory tract infections. Monthly infusions during RSV season were safe and well tolerated. Adverse events related to the hyperimmune globulin infusion were generally mild (< 3%) including fluid overload, decreased oxygen saturation and fever. Palivizumab, an intramuscularly administered humanized monoclonal antibody (RSV-glycoprotein-F antibody), will be preferable for the future because of ease of administration and comparable reduction in the risk of hospitalization. RSV-IGIV and palivizumab are both cost expansive and prophylaxis should be limited to high-risk infants.

Purpose: Our purpose was to measure sperm creatine phosphokinase (CK) activity, which reflects cytoplasmic retention in immature spermatozoa, in normospermic and oligozospermic Hungarian men., Methods: A study of 109 randomly selected men in a university-based andrology laboratory was done., Results: CK activity differed between normospermic and oligozospermic men (0.21 +/- 0.02 vs. 1.19 +/- 0.15 CK IU/10(8) sperm; n = 56 and n = 53; mean +/- standard error of the mean, respectively). There was an inverse correlation between sperm concentration and CK activity (r = -0.70; n = 109). However, 28% of men in the range with less than 10 million sperm/ml had normal sperm CK activity (below the mean + 2 standard deviations of the group with greater than 30 x 10(6) sperm/ml), whereas 36% of men in the group with 20-30 million sperm/ml and 5% in the group with greater than 30 million sperm/ml had elevated CK activities, indicating that the incidence of mature and immature spermatozoa in specimens is independent from the sperm concentrations., Conclusions: The improved facility of sperm CK activity measurements, compared with sperm concentrations, in the assessment of sperm maturity was confirmed in a Hungarian population. The CK measurements aid the selection of the most efficient treatment for couples with male-factor or unexplained infertility, particularly when considering the options of intrauterine insemination, varicocelectomy followed by a waiting period, or ovulation workup/induction in wives of men who are oligozospermic but may have fertile sperm.

Bronchopulmonary dysplasia (BPD) is a chronic lung disease that develops in preterm infants treated with oxygen and positive-pressure ventilation for respiratory distress syndrome. Despite the introduction of new treatment modalities (surfactant therapy, high-frequency oscillation) and improvements in the outcome of critically ill preterm infants, BPD has become an extremely important complication of neonatal intensive care and the most common form of chronic lung disease in infants. Specific pathogenesis, treatment modalities, prognosis, and multidisciplinary approaches to the prevention of BPD are described in detail. Extrinsic allergic alveolitis ("hypersensitivity pneumonitis") is a rare pulmonary disease in childhood due to inhaled organic dust, containing fungal antigens, thermophilic actinomycetes, or avian proteins. Diagnosis is often difficult, but it should be considered in every child with persistent and otherwise unexplained respiratory symptoms.

Over a 6-year period (1989-1995), gastrointestinal (GI) perforation was diagnosed in nine preterm infants (mean gestational age 27 weeks, mean birth weight 872 g). Three presented with necrotizing enterocolitis (NEC), two with indwelling-tube-induced perforation of the stomach, one with small-left-colon syndrome, and another with meconium ileus. Spontaneous intestinal perforation occurred in two similar very-low-birth-weight (VLBW) infants, in the distal ileum, on days 8 and 9 of life, respectively. The only clinical sign was extensive abdominal distension, and abdominal X-ray studies revealed free peritoneal air. All findings were distinct from those associated with NEC. Their further clinical course was complicated by reperforation on day 32 and 39, respectively. They subsequently recovered and presented without GI problems at the corrected ages of 4 and 2 months, respectively. In contrast to high mortality of 57% in the group with non-spontaneous intestinal perforations, spontaneous perforation seems to have a good prognosis even in VLBW infants if diagnosed and treated promptly.

The spectrum of chronic interstitial lung disease in children includes a large and heterogeneous group of rare disorders. This paper reviews these disorders by focussing on basic pathophysiological mechanisms, and by discussing the difficulties in the classification of these diseases. Diagnostic and therapeutic approaches are also listed. The overall prognosis is dubious and mortality remains high.

We report the case of 17-year-old male adolescent immunocompetent patient with an operated transposition of the great arteries after the Mustard technique admitted to our hospital because of a cough and hemoptysis. Two nodules and an area of ground glass appearance located in the lower lobe of the left lung were diagnosed by ultrafast computed tomography (UF-CT) after ruling out cardiovascular complications. The gastric aspirate revealed acid-fast bacilli despite a repeatedly negative tuberculin skin test identified as Mycobacterium gordonae by the Gen-Probe Rapid Diagnostic Test. After an initial standard antimycobacterial therapy with isoniazid, rifampin and pyrazinamide the therapy was changed to clarithromycin and after a treatment course of 14 days, the UF-CT revealed a normal scan of both lungs. The case described suggests that one has to consider M. gordonae as a rare cause of infection even in immunocompetent patients.

Over a 5-year period (1984-1988) intra- and periventricular hemorrhage (IVH/PVH) was observed in 299 preterm infants. Sixty-eight infants developed posthemorrhagic hydrocephalus (PH); of these, 23 infants died and 40 infants could be followed up for assessment of neurological development (5 patients were lost to follow-up). At 1 year of corrected age 15% (25% at 5 year follow-up) of the infants were determined to have developed normally, 35% (25% at 5-year follow-up) showed mild neurological symptoms and/or slight developmental delay, 32.5% (28% at 5-year follow-up) had handicaps and/or moderate mental retardation, and 17.5% (22% at 5-year follow-up) had severe handicaps and/or severe mental retardation. There was a significantly worse outcome in infants with grade 4 IVH/PVH (P < 0.05) and a significantly worse outcome in the group requiring ventriculoperitoneal (VP) shunt (P < 0.05). The results at 1 year of corrected age proved to be a quite realistic predictor of neurological functioning at 5 years of age (80% predicted correctly in the non-shunted-group--one patient lost to follow-up; 95% predicted correctly in the shunted group--four patients lost to follow-up). Cystic periventricular leukomalacia had been diagnosed in 7 (10%) patients and was associated with poor neurodevelopmental outcome. Gestational age, birth weight, time of shunt placement, and peripartum asphyxia had no significant influence on neurodevelopmental outcome. Infants with shunt infections and a high number of shunt revisions were found to have a significantly worse neurodevelopmental outcome (P < 0.01).

Between July 1993 and December 1994 five term infants of mothers with Graves' disease were hospitalised at the Frühgeburtenstation of the Univ.-Frauenklinik Graz. Four Mothers had elevated TSH-receptor-antibody (TRAb)--levels during pregnancy, one had normalised TRAb-titers. In one case hyperthyroidism was first diagnosed during pregnancy. Three newborns had elevated TRAb-titers; in one of them thyrotoxicosis was diagnosed clinically and biochemically at the second day of life, one newborn had mild hyperthyroidism with tachycardia at the end of the first week of life and one newborn was asymptomatic by immediately initiated therapy. The two other newborns had normal thyroid hormone and antibody levels and no symptoms or signs of hyperthyroidism. The cases are reported and discussed in detail and our overall approach to diagnosis and treatment of neonatal hyperthyroidism in case of maternal Graves' disease will be given.

Two children with Ph+ chronic myelogenous leukemia (CML) relapsed in the chronic phase after allogeneic bone marrow transplantation (BMT). They were treated with transfusions of peripheral blood mononuclear cells (PBMC) obtained from the former bone marrow donors in combination with interferon alfa-2. In one child, CML was successfully controlled as shown by disappearance of Ph+ metaphases as well as negativity for BCR-ABL fusion gene transcripts demonstrated by polymerase chain reaction (PCR) analysis. The patient has remained in complete remission without evidence of disease for 12 months after donor PBMC transfusions. The other child showed disappearance of BCR-ABL gene transcripts by PCR analysis only in peripheral blood cells, but PCR positivity persisted in bone marrow samples. These results indicate that adoptive immunotherapy may be a further alternative in children with relapse of CML after allogeneic BMT as previously described for adult patients.

Prostaglandins (PGs) are of primary importance in the initiation and maintenance of labor in women. A major intrauterine source of prostaglandins is the amnion, which synthesizes increased amounts of PGE2 at term labor. Because PG endoperoxide-H synthase (PGHS) catalyzes the rate-limiting step of PG synthesis from arachidonic acid, we investigated the changes in amniotic PGHS specific activity during gestation and at term and preterm labor. Also, we determined the level of immunoreactive PGHS protein in the amnion to evaluate the mechanisms by which PGHS activity may be regulated. PGHS specific activity, measured at the amount of PGE2 produced by amnion microsomes under optimal conditions, was 18.2 +/- 3.7 pg PGE2/micrograms protein.min (mean +/- SE; n = 19) at term (37-42 weeks gestation) before the spontaneous onset of labor. PGHS specific activity was significantly higher after spontaneous term labor (38.9 +/- 6.0 pg PGE2/micrograms protein.min; n = 19; P < 0.05). Amnion samples from preterm (< 36 weeks gestation) nonlaboring patients contained low levels of PGHS specific activity (5.9 +/- 1.8 pg PGE2/micrograms protein.min; n = 9), which increased significantly with spontaneous preterm labor (28.3 +/- 6.8 pg PGE2/micrograms protein.min; n = 10; P < 0.05). Longitudinal analysis of the data showed that PGHS specific activity was low in the first and second trimesters of gestation, but increased dramatically before labor onset at term. We detected PGHS protein in all microsomal samples, with an antiovine PGHS antibody recognizing both PGHS-1 and -2 isoforms of the enzyme. However, there was no correlation between PGHS specific activity and the amount of immunoreactive PGHS protein. Using an antibody specific for PGHS-2, we detected immunoreactive protein in only 9 of the 25 tissues examined and found no correlation between PGHS specific activity and the amount of PGHS-2 protein. These results suggest that 1) PGHS specific activity in the amnion increases sharply before the onset of labor at term; 2) further increases in specific activity occur during term and preterm labor; and 3) the specific activity of PGHS in the amnion is not related directly to the amount of immunoreactive enzyme protein.

The ultrastructural organization and some histochemical characteristics of the enteric nervous system (ENS) were investigated in 10- and 18-week-old human fetuses. In the 10-week-old human fetus immature myoblasts, and mostly neuroblasts were found in the ganglia. Simple, undifferentiated neuropil was observed among neuronal cells. The neuropil generally did not contain synapses; however axosomatic synapse was registered rarely on the surface of certain neurons. Neuromuscular junctions were common, both axons and neurons were in close contact with the sarcolemma. In the 18-week-old human fetus the fine-structural characteristics of the intestinal smooth muscle cells were the same as in the adult. Nerve profiles were frequently found among the muscle cells. NADH-diaphorase histochemistry revealed the presence of numerous ganglia but solitary neurons still occurred. Differentiated neurons and neuroblasts could be distinguished in the myenteric ganglia. Synapses were often detected in the neuropil. Thick nerve plexuses were frequently found in the proximity of smooth muscle cells, forming "distant" and "close" myoneural contacts. Well-defined fluorescent network and several fluorescent nerve cell bodies were demonstrated by glyoxylic acid. The above organization may provide a satisfactory basis for an integrated peristaltic movement in the gut of the 18-week-old human fetus.

Prenatal diagnosis of fetal rubella infection was reported. Maternal infection took place at the 13th week of gestation. Percutaneous umbilical fetal blood sampling was performed at the 22nd week of gestation. 1:400 titre of rubella specific immunoglobulin in fetal sera confirmed the rubella infection. A normal baby was born by cesarean section at term and subsequent ophthalmologic, cardiologic, audiologic follow-up have not revealed any alteration in infant's development. Clinical management of fetal rubella infection was discussed.

Between January 1984 and March 1989 twenty-seven low birth weight infants (mean birth weight 1351 gm, mean gestational age 30 weeks) required shunts after development of a posthaemorrhagic hydrocephalus. Revision of the shunt occurred in 78% of the patients with a range of 1 to 11 revisions. Obstruction of the ventricular catheter was the main cause of mechanical complications that occurred in 75%. Preterm infants weighing less than 1000 gm revealed an enormous infection rate of 71%. Initial shunt placement in the first 8 weeks of life was more likely to need shunt revision (94%) than that placed at older age (44%). There was no difference between the type of shunt and percentage of shunt revision, but the Heyer-Schulte system in comparison with the Codman Uni shunt was more likely to have mechanical complications. Infants with Grade IV haemorrhage required the same percentage of revisions as those with Grade III haemorrhage. There was no association between preoperative therapy and the need for shunt revision. A great amount of erythrocytes and very low glucose levels in the preoperative CSF were more likely to predict shunt revision than predicted by the CSF protein.

The contraceptive effect of d-norgestrel given immediately after copulation in various quantities was investigated in rabbits. It was established that the effect is dose-dependent. A correlation was found between the amount of dose administered and the changes taking place on the surface of endometrium. It can be suggested that d-norgestrel alters the surface of the endometrium to such an extent that nidation is unable to take place; therefore, it can be used for postcoital contraception at any time.

Total phospholipid contents and the individual phospholipid components of human adult and fetal spleens from 17--18 and 23--24 week's pregnancies composition of sphingomyelin, phosphatidylcholine, phosphatidylethanolamine, phosphatidylinositol, phosphatidylserine, diphosphatidylglycerol and phosphatidic acid studied in human adult, 17--18 and 23--24 week fetal spleens.

We measured the cross reactivity of two medications--digitoxin and spironolactone--in two digoxin radioimmunoassay (liquid and solid-phase) kit procedures. Both tests showed similar average percentages of cross reactivity with digitoxin (7.2 and 8.9% for intravenous, and 11.9 and 10.9% for oral administration), but no cross reactivity with spironolactone or its metabolites after equal intravenous or oral doses.

The PGE2-analogue Sulproston (16-phenoxy-omega-17,18,19,20-tetranor-PGE2-mythylsulfonylamide) was administered to 200 medically and gynecologically normal women who were 17 +/- 0.4 days beyond their expected menstrual period and who had a positive pregnancy test. The intramuscular impact dose (500 micrograms repeated after 4 hours) caused an immediate tonic uterine contraction which compromised the estradiol 17 beta, progesterone and chorionic gonadotropin production within the fetoplacental unit, and thereby allowed the evolution of cyclic uterine activity, cervical dilatation and tissue expulsion. Pregnancy termination was complete in 92% of women, 5.5% required surgical curettage and 2.5% were given a second Sulproston treatment 2-3 weeks after the first to remove retained tissue from the uterus. The medical induction of menstruation was preferred by 83% of the women who had previously experienced surgical termination of pregnancy. Normal menstruation resumed in all women after 36 +/- 0.9 days. The majority of 42 women questioned found Sulproston a satisfactory, safe, simple and effective drug regimen for "menstrual induction".