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History and findings A 24-year-old pregnant woman had to be hospitalized in the 33rd week of pregnancy because of premature contractions and clinical signs of pyelonephritis. She had a history of nephrolithiasis. Laboratory tests showed a total calcium of 3.6 mmol/l, hypophosphataemia of 0.59 mmol/l and an increased parathormone level of 420 ng/l. Ultrasonography demonstrated a large parathyroid adenoma, confirming the diagnosis of primary hyperparathyroidism. Course and treatment Despite several recommendations of conservative treatment in the literature it was decided to perform a parathyroidectomy, which was done without complication during the 35th week of pregnancy. A healthy, mature boy was born at the beginning ot the 41st week: at no time did he show any signs of hypoparathyroidism. Conclusions Surgical treatment of hyperparathyroidism is a reasonable and possible choice even in the 3rd trimester, because it allows regeneration of the fetal parathyroid. However, this decision must be individualized, in relation to the period of pregnancy and any progression of the disease.

The hypothalamic-pituitary-adrenal axis (HPA) was examined in 34 ventilated preterm infants weighing < or = 1250 g during the first week of life to evaluate the association between adrenal suppression and subsequent chronic lung disease. The second aim of the study was to detect perinatal and clinical differences between the infants with and without persistent suppression of the HPA after completion of dexamethasone treatment for chronic lung disease. To evaluate the HPA, the corticotropin-releasing hormone stimulation test was performed, and the cortisol and adrenocorticotropic hormone (ACTH) levels were measured by radioimmunoassay. No association could be found between the synthesis of cortisol and ACTH at the end of the first week of life and the development of chronic lung disease. After treatment with dexamethasone, baseline cortisol levels < 138 nmol l(-1) were found in 12 infants (46.2%), 8 of whom (30.8%) had cortisol values below 83 nmol l(-1). The perinatal data of these patients did not differ from infants without HPA suppression. However, the infants with cortisol levels < 83 nmol l(-1) after dexamethasone showed a significantly shorter need for mechanical ventilation and supplemental oxygen (p < 0.01) and a lower incidence of chronic lung disease (p < 0.05).

Congenital absence of the trachea is a rare anomaly that might confront the obstetrician or neonatologist with an unexpected emergency. These patients present with cyanosis, severe respiratory distress, insufficient gas exchange, absence of audible crying and difficult or impossible endotracheal intubation. In more than 90% it is associated with further congenital malformations. Adequate oxygenation depends on the existence of a tracheo- or bronchooesphageal fistula and the length of the proximal trachea. We present the cases of three neonates with tracheal agenesis with tracheooesophageal fistula. Two of the neonates died within the first hour of life because endotracheal intubation was impossible and oxygenation through an oesophageally placed tube was insufficient. The third infant could be oxygenated through a tracheooesophageal fistula. The ventilation was at least insufficient and no surgical intervention was made. The diagnosis of a congenital absence of the trachea usually is made after birth because of the clinical signs and the course within the first minutes of life. The only way that the diagnosis can be made prenatally is by magnetic resonance imaging (MRI). The knowledge of this clinical picture helps to make decisions in an unexpected emergency in the immediate postpartum period and also in patients whose ventilation is very difficult right from the start.

Background: Treatment with methadone during pregnancy may lead to severe neonatal abstinence syndrome. This is a retrospective study of the symptoms, duration, course and therapy in babies diagnosed as having neonatal abstinence syndrome due to maternal methadone substitution. We sought to find if there is a relationship between dose and duration of methadone substitution and the severity of neonatal abstinence syndrome. Patients and Methods: We evaluated the following data on preterm and term babies of mothers substituted with methadone between 1994 and 2001: maternal dose of methadone, duration of substitution during pregnancy, supplemental maternal drug use, maternal infections, perinatal complications, symptoms, duration, course and therapy of neonatal abstinence syndrome. Results: There were 34 babies (27 term and 7 preterm) with a mean gestational age of 38.5 weeks. The mean birthweight was 2808 grams. 97 percent of the babies developed symptoms of neonatal abstinence syndrome which required treatment. The mean duration of treatment was 36.5 days. Four children whose mothers had taken a dose of methadone greater that 100 grams per day throughout the whole or the last two month of pregnancy had a longer duration of neonatal abstinence syndrome (mean 64.5 days; Pearson correlation coefficient: r = 0.4725). Apart from common withdrawal symptoms such as shivering and excitability there were 9 babies who had seizures. There was one baby whose mother had been detoxified as an inpatient, who had no withdrawal symptoms. Conclusion: Maternal methadone dose and the duration of methadone ingestion during pregnancy correlated with the severity of the neonatal abstinence syndrome. Use of methadone in a pregnant woman requires careful thought.

Background: A number of severe gastrointestinal disorders in infancy and childhood may require the formation of an enterostomy as a crucial part of the treatment of the disease itself. This study reviews our pediatric patients with regard to the morbidity and mortality of enterostomy formation and closure over an 8-year period. Patients and methods: Sixty-eight enterostomies in infants and children and 60 consecutive enterostomy closures in retrospect are reported on. This includes colostomies, jejunostomies, ileostomies, and Mikulicz procedures. Results: In most instances, a transverse colostomy was performed. The most frequent indications were intestinal obstruction and necrotizing enterocolitis. More than half of the children were less than 1 month of age at the time of surgery. We observed an overall complication rate of 38.2% following enterostomy formation, with stoma prolapse being the most common, but faced major complications (such as sepsis, peritonitis, and enterocutaneous fistula) in only 10.3%. Complications after enterostomy closure were encountered in 20%. The overall mortality was 7%. Conclusion: Enterostomy formation and closure in the pediatric age group with severe underlying disease is still associated with substantial morbidity.

Objective: To evaluate the effects of 24 h partial liquid ventilation (PLV) with and without surfactant (S) treatment on gas exchange and lung injury in a newborn animal model of S deficiency.¶Design: A prospective, controlled, in vivo animal laboratory study.¶Setting: Research laboratory in a university setting.¶Subjects: Twenty-four pathogen-free, male piglets (mean weight 1.9 kg, age 1–3 days).¶Interventions: The animals were randomised in four groups: PLV with FC-77 combined with conventional ventilation (PLV/CV) versus S + PLV/CV and PLV combined with high frequency oscillatory ventilation (PLV/HFOV) versus S + PLV/HFOV. The piglets were anaesthetised, intubated and instrumented with vascular catheters. Thirty minutes after lung injury had been induced with repeated saline lavage, S animals received natural S. Thirty minutes after surfactant substitution PLV with FC-77 was started. The oxygenation index (OI), PaO2/FIO2 ratio, PaCO2 and the ventilatory efficacy index were determined before and during PLV. After 24 h the lungs were removed for histopathological examination.¶Measurements and main results: Within 60 min after the initiation of PLV, all animals demonstrated improvements of the OI and PaO2/FIO2 ratio compared to the values after lung injury. However, at 18 and 24 h of PLV, the OI and PaO2/FIO2 ratio were significantly worse in the S + PLV/CV and S + PLV/HFOV groups compared to the groups without S. PaCO2 was higher at 18 and 24 h when S was used in PLV/HFOV (p < 0.05). A semi-quantitative lung injury score revealed most severe lung damage in the S + PLV/HFOV group.¶Conclusion: The combination of S and PLV with FC-77 led to an impaired gas exchange and did not further protect the animal from lung injury.

The clinical outcome after inferior vena cava thrombosis in early infancy is unknown. We report the clinical long-term follow-up of 12 patients presenting inferior vena cava thrombosis within their first months of life (gestational age: 24-41 weeks; follow-up: 7+/-3 years). Accompanying renal venous thrombosis occurred in 9, and adrenal bleeding in 4 patients. A central venous catheter was related to the thrombosis in only four patients. Heterozygous factor V Leiden mutation was found in two of the eight infants without central venous catheter. Thrombolysis was performed in seven and effective in three infants; one infant required surgical thrombectomy. In three of eight infants with ineffective or with no therapy, spontaneous recanalization occurred during follow-up. No patient died of the thrombosis. Although no long-term anticoagulatory prophylaxis was performed, none of the children with persisting occlusion (n = 5) or stenosis (n = 1) of the inferior vena cava developed symptomatic thrombo-embolic complications. However, extensive internal collaterals (n = 6), visible varicosis (n = 5), pain in the legs (n = 3) and persisting renal disease (n = 3) with arterial hypertension (n = 2) were observed during follow-up.Inferior vena cava thrombosis of early infancy frequently persists and may cause considerable long-term morbidity. New strategies for early and long-term therapy are necessary.

The purpose of this controlled, prospective pilot study was to compare the short-and long-term efficacy of early versus late treatment with dexamethasone (Dex) in preterm infants at risk for chronic lung disease (CLD). Thirty ventilated premature infants with a birth weightor = 1250 g were randomized to receive Dex either from day 7 or from day 14. Dex was administered over 16 days tapering from 0.5 mg/kg per day to 0.1 mg/kg per day. The infants of the early treatment group could be weaned significantly earlier from the ventilator after 14 days (median; range 9-24) versus 24 days (median; range 8-44) in the late treatment group. The need for supplemental oxygen was shorter if Dex was started early - 24 days (median; range 10-57) versus 40 days (median; range 10-74). Oxygen dependency at 28 days of age was similar between the groups 6 out of 14 infants (42.9%) versus 10 out of 16 patients (62.5%). The long-term efficacy of the two Dex regimens on lung function was evaluated by body plethysmographic measurements made at the age of 3 months. Thoracic gas volume and airway resistance were measured and specific airway conductance calculated. No statistically significant differences between the groups were demonstrated.Early dexamethasone treatment led to earlier extubation in our study population, but was not associated with significant advantages regarding oxygen dependency at 28 days of life and pulmonary function test at 3 months of age.

The characteristic features of the Cantrell-sequence--anterior thoraco-abdominal wall defect with ectopia cordis and diaphragm, sternum, pericardium, and heart defects--have been observed in animals following maternal administration of beta-aminopropionitrile, a toxic amino-acid derivative. We report on an unusual case of the Cantrell-sequence in a premature infant with associated dysmelia, aplasia of the right kidney, cerebellar hypoplasia and circumscribed aplasia of the cutis, which has not been reported previously. Maternal history suggested an occupational exposure to aminopropionitriles prior to pregnancy. Prenatal ultrasound, differential diagnosis, perinatal management, and the teratogenic role of aminopropionitriles in this rare genetic disorder are discussed.

Thirty-six children (median chronological age 6 years 1 month) who had undergone surgical closure of a patent ductus arteriosus through a left posterolateral thoracotomy in the neonatal period (median gestational age 32 weeks) were investigated prospectively with respect to anatomical and functional changes of the chest. At follow-up examination, residual or recurrent patent ductus arteriosus was not observed. Three patients had chronic bronchial obstruction. Two patients showed pathological musculoskeletal thoracic sequelae that did not require any treatment at the time of follow-up; persistence of immediate postoperative left phrenic palsy (n = 1) and thoracic scoliosis (n = 1). Twenty of the 27 patients in whom chest X-ray was performed had minor radiological skeletal anomalies in the form of rib deformation or fusion related to the thoracotomy, lesions which have a potential to induce thoracic scoliosis. Left shoulder elevation at chest X-ray and isolated left arm dysfunction at clinical examination were not observed. Despite the low incidence of scoliosis and the absence of left arm dysfunction observed at mid-term follow-up in our series, the incidence of minor rib deformations with a potential to induce severe anomalies such as scoliosis should motivate late follow-up examination at adolescence to definitively assess the prevalence of thoracic sequelae after surgical closure of the patent ductus arteriosus in premature infants.

Complement activation and leukocyte stimulation were prospectively studied during and after cardiopulmonary bypass in 16 children receiving sodium nitroprusside—a nitrovasodilator releasing nitric oxide—for vasodilation during the cooling and rewarming periods of extracorporeal circulation. Results were compared with those in 29 patients who were not treated with sodium nitroprusside during the operation. Patients treated with sodium nitroprusside had significantly less C3 conversion during cardiopulmonary bypass as measured by the ratio C3d/C3 (p < 0.05) and significantly less C5a liberation immediately after cardiopulmonary bypass (p < 0.005) than patients not treated with sodium nitroprusside. C4 was not overtly consumed in our series. Leukocyte count during the rewarming period of cardiopulmonary bypass, but not leukocyte elastase release during cardiopulmonary bypass, was significantly reduced in patients treated with sodium nitroprusside (p < 0.05). In vitro experiments were conducted to analyze the effect of sodium nitroprusside on complement hemolytic activity initiated by the classic and the alternate pathways and on zymosan-induced C3 conversion by the activation of the alternate pathway. The in vitro experiments clearly demonstrate inhibition of complement hemolytic activity by sodium nitroprusside in the sera tested. The 50% inhibitory concentration of sodium nitroprusside on the available complement hemolytic activity was less through the alternate pathway than through the classic one (4.2 ± 0.8 mmol/L and 14.0 ± 2.88 mmol/L, respectively). The decrease of complement hemolytic activity measured was dose-dependent and was enhanced by the sodium nitroprusside preincubation of the sera tested. This effect was related to the duration of preincubation. Sodium nitroprusside photodegradation (enhancing nitric oxide release) increased the anticomplementary effect of the drug, reducing the 50% inhibitory concentration on complement hemolytic activity to 0.24 to 0.02 mmol/L for the alternate pathway and 2.74 to 0.3 mmol/L for the classic pathway. The zymosan-induced C3 conversion was inhibited by sodium nitroprusside. Nitroglycerin and isosorbide dinitrate (other nitric oxide donors) had in vitro effects on complement hemolytic activity similar to those of nonphotodegraded sodium nitroprusside at similar concentrations (1 mmol/L). Our results suggest that sodium nitroprusside, both in vitro and in vivo, has an inhibiting effect on complement activation initiated by both classic and alternate pathways and that this effect is mediated by nitric oxide release from sodium nitroprusside. This is the first report on the anticomplementary effect of sodium nitroprusside by nitric oxide release. (J THORAC CARDIOVASC SURG 1996;111:882-92)

Between January 1985 and March 1989 we retrospectively observed multiple system organ failure (MSOF) in 16 of 460 children (3.5%) who underwent cardiac surgery with cardiopulmonary bypass for congenital heart disease. MSOF was arbitrarily defined as a clinical entity with failure of two or more vital organ systems associated with high fever, thrombocytopenia, and cardiocirculatory insufficiency and occurring within the first postoperative week. In 13 children the first clinical manifestations of MSOF were evident on the first post-operative day and in the other 3 on the second or third post-operative day. All children showed acute renal failure, acute hepatic failure, high fever, and thrombocytopenia. Most of them showed respiratory insufficiency and neurological involvement. Seven of the 16 children died. Four of the 9 surviving patients had neurological sequelae still present 6 months after the operation, and the others recovered completely.

Two different ventilation techniques were compared in a seven-centre, randomised trial with 181 preterm infants up to and including 32 completed weeks gestational age, who needed mechanical ventilation because of lung disease of any type. Technique A used a constant rate (60 cycles/min), inspiratory time (IT) (0.33s) and inspiratory: expiratory ratio (I:E) (1:2). The tidal and minute volume was only changed by varying peak inspiratory pressure until weaning via continuous positive airway pressure. Technique B used a lower rate (30 cycles/min) with longer IT (1.0 s). The I:E ratio could be changed from 1:1 to 2:1 in case of hypoxaemia. Chest X-rays taken at fixed intervals were evaluated by a paediatric radiologist and a neonatologist unaware of the type of ventilation used in the patients. A reduction of at least 20% in extra-alveolar air leakage (EAL) or death prior to EAL was supposed in infants ventilated by method A. A sequential design was used to test this hypothesis. The null hypothesis was rejected (P = 0.05) when the 22nd untied pair was completed. The largest reduction in EAL (-55%) was observed in the subgroup 31-32 weeks of gestation and none in the most immature group (28 weeks). We conclude that in preterm infants requiring mechanical ventilation for any reason of lung insufficiency, ventilation at 60 cycles/min and short IT (0.33 s) significantly reduces EAL or prior death compared with 30 cycles/min and a longer IT of 1 s. We speculate that a further increase in rate and reduction of IT would also lower the risk of barotrauma in the most immature and susceptible infants.

Persistent pulmonary hypertension of the newborn (PPHN), initially described by Gersony et al as persistent foetal circulation (PFC syndrome), results from a flawed transition from foetal to extrauterine pulmonary circulation. It is characterised by the maintenance of a high pulmonary vascular resistance and right-to-left shunting through the ductus arteriosus and foramen ovale. Infants with a wide variety of underlying clinical conditions develop PPHN. According to Rudolph three main anatomic types of PPHN can be identified: normal pulmonary vascular development increased pulmonary vascular smooth muscle development decreased cross-sectional area of pulmonary vascular bed. It is important to realize that several pathophysiologic mechanisms may coexist and interact. Besides metabolic and respiratory acidosis, hypercapnia and hypoxaemia some other factors induce pulmonary vasoconstriction. Thromboxane, leukotrienes and prostaglandins play a decisive role. Since PPHN can be associated with a broad spectrum of clinical conditions, a specific clinical picture is lacking. The baby is usually term or post-term, cyanotic immediately after birth or some hours later. Birth asphyxia, hyperviscosity, sepsis and aspiration of meconium have been recognized as predisposing factors. The diagnosis can be confirmed by echocardiography. Contrast echo will indicate right-to-left shunting with normal anatomy. Currently hyperventilation, tolazolin, chlorpromazin and dopamine/dobutamine have been advocated as central foci for clinical therapy. Recently prostacyclin was introduced as a specific pulmonary vasodilatator.(ABSTRACT TRUNCATED AT 250 WORDS)

We report about a child with severe ARDS after burning trauma who did not respond to conventional treatment with controlled pressure ventilation under conditions of permissive hypercapnia and changing of the infants's body position. A combined treatment with high frequency oscillatory ventilation, inhalation of nitric oxide and surfactant replacement improved the pulmonary status. Twelve days after the accident the boy could be extubated and 5 weeks later he could be discharged without any pulmonary and neurologic handicap. The use of these therapeutic tools may help to avoid the necessity of the invasive extracorporeal life support.

Einleitung: Angeborene Hirntumoren sind selten, sie machen etwa 0,5 bis 2 Prozent aller Tumoren des ZNS im Kindesalter aus. Darunter stellen die histologisch gutartigen Teratome mit etwa 40% die groste Gruppe dar. Die Prognose ist bei Inoperabilitat des Tumors infaust. Die Erkrankung wird anhand einer Kasuistik vorgestellt. Kasuistik: Aufgrund respiratorischer Anpassungsstorungen ubernahmen wir am ersten Lebenstag ein reifes, weibliches Neugeborenes aus der Geburtsklinik. Klinisch imponierte ein Makrozephalus, der Kopfumfang lag mit 38cm oberhalb der 97. Perzentile. In der Sonographie des ZNS sowie in der nachfolgend durchgefuhrten MR-Tomographie stellte sich die Raumforderung als 6×8cm groser, inhomogener, teils zystischer, teils solider Tumor der rechten Hemisphare dar, welcher das Hirnparenchym diffus durchsetzte. Die Grose des Tumors bedingte eine Mittellinienverlagerung mit Kompression der kontralateralen Hirnhemisphare, ferner eine Kompression der Ff. Monroi sowie des Aquaeductus cerebri und konsekutiv eine Erweiterung insbesondere des linken Seitenventrikels. Der Tumor wurde seitens der Kollegen der Neurochirurgischen Klinik als in der Neugeborenen-Periode inoperabel eingestuft. Klinisch bot die Patienten an den Folgetagen eine rasche Zunahme des Kopfumfangs, sowie eine progrediente Verschlechterung des Trinkverhaltens und der Vigilanz. Sie verstarb am 9. Lebenstag an respiratorischer Insuffizienz infolge des Hirndrucks. Die neuropathologische Untersuchung zeigte analog zur bildgebenden Diagnostik einen von der rechten Hirnhemisphare ausgehenden, teils zystischen, teils soliden Tumor. Konventionell-histologisch und immunhistochemisch lies sich unreifes Gewebe aller drei Keimblatter nachweisen. Der Tumor wurde als immatures Teratom klassifiziert. Schlussfolgerungen: Konnatale, intrakranielle Teratome entstehen aus Keimzellgewebe, welches im Zuge der fetalen Keimwanderung vom Dottersack aus die primordiale Keimanlage nicht erreicht hat. Die Tumoren werden haufig bereits durch pranatale Ultraschalldiagnostik festgestellt, klinisch imponieren Makrozephalie durch den Tumor selbst und durch Liquorzirkulationsstorungen sowie Hirndruck-bedingte Auffalligkeiten der Atmung, des Trinkverhaltens, motorische Ausfalle und ein Sonnenuntergangs-Phanomen der Augen. Anders als die im spateren Lebensalter uberwiegenden malignen Keimzelltumoren des ZNS sind die histologisch benignen, differenzierten kongenitalen Teratome koservativen Therapiemodalitaten schlecht zugangig. Einzig kurativer Ansatz ist eine zumindest subtotale Resektion des Tumors. Wie auch in dem hier vorgestellten Fall ist eine chirurgische Entfernung aber typischerweise aufgrund der Grosenausdehnung unmoglich. Dementsprechend ist die Mortalitat hoch: Rund 90% der Patienten versterben noch intrauterin bzw. innerhalb weniger Tage bis Wochen nach der Geburt.

A few years ago recombinant human erythropoietin (rh-EPO) has been introduced for the prophylaxis of anaemia of prematurity. Aim of this controlled study was a cost-effectiveness analysis of the prophylaxis with rh-EPO versus sole transfusion with packed red blood cells. In the study group 33 infants (gestational age 30 +/- 2 weeks, birthweight 1217 g +/- 244 g) were treated with rh-EPO beginning on the fifth day of life for a six week period. They received 750 IE rh-EPO/kg/week and transfusion with packed red blood cells when indicated. In the historic control group 33 infants (gestational age 29.2 +/- 1.9 weeks, birthweight 1181 g +/- 205 g) did not receive rh-EPO, patients were only transfused. Indication and guidelines for transfusion were identical for both groups. The number of transfusions was registered after 2 and 4 weeks of life and by the time of hospital discharge. The cost analysis was carried out by using current prices for packed red blood cells including material and processing and prices for rh-EPO (Neo-Recormon, Boehringer Mannheim). Infants in the study group received 1.39 +/- 1.94 transfusions per patient while patients in the control group needed 2.7 +/- 1.93 transfusions per patient (p0.05). Cost for treatment was slightly increased in the study group (DM 536,- vs. DM 459,-). Prophylaxis of anaemia of prematurity with recombinant human erythropoietin proved to be effective. Compared with sole blood transfusion treatment, expenses for the prophylaxis with rh-EPO were only little higher.

Non-invasive oxygen monitoring with pulse oximetry or transcutaneous monitoring has gained widespread use in neonatology. Different factors like arterial hypotension, peripheral vasoconstriction and edema adversely affect the accuracy of both methods. To ensure reliable monitoring of oxygen saturation in critically ill patients we measured oxygen saturation with a fiberoptic catheter via umbilical artery.In ventilated premature infants (FiO20.4) a 4F-fiberoptic catheter (Oximetrix)-3, Abbott) was inserted to the descending aorta (Th 6-8). Simultaneously pulse oximetry (SaPO2) was performed with the Ohmeda Biox 3700. To compare the reliability of both methods, blood was analysed for arterial partial oxygen pressure (PaO2), fetal hemoglobin (HbF) and arterial oxygen saturation (SaO2) by complete co-oximetry (Radiometer Copenhagen OSM3) as reference.In 10 premature infants (median gestational age 30.5 weeks; median birth weight 1360 g) oxygen saturation was measured with the fiberoptic catheter (SaFO2) over a total period of 935 hours. In all, 137 blood samples were analysed for arterial saturation (SaO2) by co-oximetry. The mean difference between the SaO2 and SaFO2 was -1.89% (+/- 1.53); the mean difference between SaO2 and the values obtained by pulse oximetry (SaPO2) was -3.09% (+/- 2.33). The SaFO2 results correlated closely with the co-oximetry values (r = 0.97; p0.0001).In critically ill patients, if non-invasive oxygen monitoring fails, a fiberoptic catheter offers the possibility of continuous and reliable measurement of oxygen saturation.

We report about a child with severe ARDS after burning trauma who did not respond to conventional treatment with controlled pressure ventilation under conditions of permissive hypercapnia and changing of the infants's body position. A combined treatment with high frequency oscillatory ventilation, inhalation of nitric oxide and surfactant replacement improved the pulmonary status. Twelve days after the accident the boy could be extubated and 5 weeks later he could be discharged without any pulmonary and neurologic handicap. The use of these therapeutic tools may help to avoid the necessity of the invasive extracorporeal life support.

Selenium is an essential component of the antioxidant enzyme glutathione peroxidase that protects tissues against oxidative injury by detoxifying peroxides. In preterm infants the risk for selenium deficiency is increased due to insufficient selenium uptake. Low selenium uptake and as a consequence decreased glutathione peroxidase activity may result in an elevated risk for the development of bronchopulmonary dysplasia (BPD). The aim of this prospective study was to investigate the relationship between the selenium status of preterm infants1500 g and the incidence of BPD. We determined the selenium plasma levels by means of atomic absorption spectrometry in 34 VLBW infants (mean birth weight 1075 +/- 249 g; mean gestational age 28.6 +/- 2.5 weeks) within the first 5 days of life and later in the age of 4 weeks. The infants received mainly parenteral nutrition and were not specifically supplied with selenium. Postnatally, the selenium plasma level was 34.2 micrograms/l (17.3/50) [median (25/75% quantil)] and dropped after 4 weeks to a median value of 16.1 micrograms/l (5.2/38.4) (p0.001). In the infants with BPD (n = 12) the selenium concentration within the first week of life was 45.0 micrograms/l (31.5/55.6) versus 33.2 micrograms/l (20.2/42.4) in the infants without BPD. In the age of 4 weeks of life the median selenium level was not significantly different between the infants with and without BPD - 17.2 micrograms/l (10.3/22.5) versus 14.8 micrograms/l (8.8/22.6).

Systemic infections with Candida albicans in neonates are a frequent and well recognized problem. The therapeutic gold standard in this situation is the combined intravenous antimycotic treatment with amphotericin B and flucytosine. Potential adverse effects of this regimen have encouraged the search for desirable alternatives. We report on the use of oral fluconazole in neonates with Candida albicans septicaemia. Three premature infants were treated with four courses of therapy. Pharmacokinetic studies were performed during each course. At oral doses of 4.5–6 mg/kg once a day, serum levels of fluconazole were within the therapeutic range during the entire dosage interval. Follow up showed microbiological and clinical cure in all patients with no side-effects. In one patient a dosage of 4 mg/kg per day lead to a microbiological relapse with sub-therapeutic serum levels. Conclusions Oral fluconazole seems to be a safe and effective treatment for Candida albicans septicaemia even in premature infants.

The hypothalamic-pituitary-adrenal axis (HPA) was examined in 34 ventilated preterm infants weighingor = 1250 g during the first week of life to evaluate the association between adrenal suppression and subsequent chronic lung disease. The second aim of the study was to detect perinatal and clinical differences between the infants with and without persistent suppression of the HPA after completion of dexamethasone treatment for chronic lung disease. To evaluate the HPA, the corticotropin-releasing hormone stimulation test was performed, and the cortisol and adrenocorticotropic hormone (ACTH) levels were measured by radioimmunoassay. No association could be found between the synthesis of cortisol and ACTH at the end of the first week of life and the development of chronic lung disease. After treatment with dexamethasone, baseline cortisol levels138 nmol l(-1) were found in 12 infants (46.2%), 8 of whom (30.8%) had cortisol values below 83 nmol l(-1). The perinatal data of these patients did not differ from infants without HPA suppression. However, the infants with cortisol levels83 nmol l(-1) after dexamethasone showed a significantly shorter need for mechanical ventilation and supplemental oxygen (p0.01) and a lower incidence of chronic lung disease (p0.05).

The purpose of this prospective study was to examine the hypothalamic-pituitary-adrenal axis using the corticotropin-releasing hormone stimulation test in 24 preterm infants (mean gestational age 27.4 +/- 1.4 weeks, mean birth weight 997 +/- 166 g) with bronchopulmonary dysplasia after completion of dexamethasone treatment.The CRH stimulation test was performed before and two days after the course of dexamethasone therapy in a dosage of 1 microgram/kg body weight. Blood samples were obtained before and 30 minutes after application of CRH. The blood values for cortisol and ACTH were measured by radioimmunoassay.The mean basal cortisol level was significantly reduced from 338 +/- 283 nmol/l before dexamethasone treatment to 153 +/- 102 nmol/l after dexamethasone therapy. The site of HPA suppression was located to the pituitary gland as the mean basal ACTH level dropped from 9.5 +/- 5.2 pmol/l to 5.6 +/- 2.0 pmol/l after the dexamethasone course. In 9 infants there was only an insufficient increase of cortisol level after application of CRH as a possible sign of a reduced adrenal response. The patients with HPA-suppression did not differ in clinical aspects from infants without suppression of the HPA.The results demonstrate a significant suppression of the adrenal and pituitary gland in very low birth weight infants with bronchopulmonary dysplasia after dexamethasone treatment. Before stressful situations like surgery we therefore recommend an investigation of the HPA.

We examined the outcome of 9 triplet, 3 quadruplet, 1 quintuplet and 1 sixtuplet pregnancies delivered between 1979-1989 at the perinatal center of the RWTH Aachen. The course of pregnancy and neonatal period were retrospectively analysed. The follow-up program covered at least 3, up to a maximum of 10 years. 12 families could be interviewed concerning psychosocial effects. The neonatal mortality was 4%. Neonatal morbidity; hyaline membrane disease (n = 18), intraventricular hemorrhage (n = 9), pneumothorax (n = 7), patent ductus arteriosus (n = 7), bronchopulmonary dysplasia (n = 8). At the age of 2 years 63% of the children were considered to be normal on developmental assessment, 17% showed mild, 20% severe developmental delay. With 3 to 10 years 83% were normal, 17% severely handicapped. In total 20% of the children died or showed severe handicap. Higher order multiple pregnancies make great demands on the perinatal medicine and lead in spite of an improved prognosis to a remaining burden for the children and their parents.

We studied the inflammatory reaction related to cardiopulmonary bypass in 24 neonates (median age 6 days) undergoing the arterial switch operation for simple transposition of the great arteries, with respect to the development of postoperative capillary leak syndrome. Complement proteins, leukocyte count, tumor necrosis factor-α, and histamine levels were determined before, during, and after cardiopulmonary bypass. Additionally, protein movement from the intravascular into the extravascular space during cardiopulmonary bypass was assessed by the measurement of plasma concentrations of proteins with molecular weights ranging from 21,200 to 718,000. Capillary leak syndrome developed in 13 of the 24 neonates. Patients with capillary leak syndrome, as compared with those without, had preoperatively higher C5a levels (C5a, 3.0 ± 0.6 μg/L vs 0.9 ± 0.2 μg/L) (mean ± standard error of the mean) ( p < 0.05) and higher leukocyte counts (leukocytes, 17.9 ± 2.1 × 103 cells/ml versus 11.7 ± 0.8 × 10 3 cells/ml) ( p < 0.05), suggesting in these neonates a preoperative inflammatory state. Preoperative clinical and operative data were identical in both patient groups. Before cardiopulmonary bypass, serum protein concentrations were similar in all patients. Ten minutes after institution of cardiopulmonary bypass, protein concentrations fell to significantly lower values in patients with capillary leak syndrome than in those without: albumin (19% ± 1.5% vs 30% ± 6% of the prebypass value, p < 0.05), immunoglobulin G (17% ± 1.5% vs 29% ± 5.5%, p < 0.001), and α 2 -macroglobulin (15% ± 1.2% vs 25% ± 4%, p < 0.02). During cardiopulmonary bypass, albumin concentrations remained significantly lower in patients with capillary leak syndrome than in those without, whereas hematocrit values were similar in both groups. During cardiopulmonary bypass, patients with capillary leak syndrome also had lower concentrations of complement proteins C3 and C4 but not C1 inhibitor. C3d/C3 ratio and C5a levels were similar in both patient groups. In contrast, histamine liberation during cardiopulmonary bypass was significantly more pronounced in patients with capillary leak syndrome than in those without (725.2 ± 396.7 pg/ml vs -54.1 ± 58.4 pg/ml, p < 0.05). Tumor necrosis factor-α levels after protamine administration were also significantly higher in patients with capillary leak syndrome (38.1 ± 10.0 pg/ml vs 15.3 ± 3.4 pg/ml, p < 0.05). Leukocyte count during and after cardiopulmonary bypass was similar in both patient groups. This study demonstrates increased protein leakage as early as 10 minutes after initiation of cardiopulmonary bypass in patients having clinical signs of postoperative capillary leak syndrome. Patients with capillary leak syndrome displayed more pronounced histamine liberation and tumor necrosis factor-α liberation than patients without capillary leak syndrome, suggesting a relationship between cardiopulmonary bypass–related inflammatory reaction and perioperative capillary damage. A preoperative inflammatory state in patients with capillary leak syndrome could have enhanced the inflammatory response to cardiopulmonary bypass. (J THORAC CARDIOVASC SURG 1996;112:687-97)

Computerized monitoring in a neonatal intensive care unit allows continuous registration and complete storage of vital parameters. The stored data can be used for detailed retrospective analysis of critically ill patients and the graphical presentation of the vital parameters may lead to an earlier recognition of clinical deterioration. This computer system which can be established in any intensive care unit demonstrates the improvement in neonatal intensive care monitoring at low financial costs.

A 24-year-old pregnant woman had to be hospitalized in the 33rd week of pregnancy because of premature contractions and clinical signs of pyelonephritis. She had a history of nephrolithiasis. Laboratory tests showed a total calcium of 3.6 mmol/l, hypophosphataemia of 0.59 mmol/l and an increased parathormone level of 420 ng/l. Ultrasonography demonstrated a large parathyroid adenoma, confirming the diagnosis of primary hyperparathyroidism.Despite several recommendations of conservative treatment in the literature it was decided to perform a parathyroidectomy, which was done without complication during the 35th week of pregnancy. A healthy, mature boy was born at the beginning ot the 41st week: at no time did he show any signs of hypoparathyroidism.Surgical treatment of hyperparathyroidism is a reasonable and possible choice even in the 3rd trimester, because it allows regeneration of the fetal parathyroid. However, this decision must be individualized, in relation to the period of pregnancy and any progression of the disease.

Primary hyperparathyroidism has to be accused to cause serious morbidity during pregnancy not only on the maternal, but also on the fetal side: the fetus is threatened by prematurity, dystrophy and an increased risk of stillbirth. Postpartually hypocalcaemia and tetany may be observed as the result of neonatal hypoparathyroidism caused by maternal and thus also fetal hypercalcaemia. We report the case of a 32-year-old pregnant woman suffering from a severe form of primary hyperparathyroidism caused by an adenoma of the parathyroidea. The tumor was removed in the 34. week of pregnancy. Six weeks later the patient delivered a healthy boy (birth weight 3450 g). A survey is given of the therapeutical procedures that should be arranged individually by interdisciplinary consulting depending on the degree of maternal disease and on the gestational age.

As part of a multicenter surfactant rescue study, the chest X-rays of 239 preterm and term infants were analyzed. To study the influence of surfactant administration on radiographic appearance, 130 patients with a clinical and radiological diagnosis of typical respiratory distress syndrome were selected, in whom adequate chest x-rays before and within 48 h after treatment were available. Median gestational age was 30 weeks (range 25-38 weeks), median birth weight was 1335 g (range 625-3450). The time of surfactant application ranged between 90 min and 24 h after birth (median 6 h). The most common finding after surfactant administration was uniform (n = 47) or disproportionate (n = 46) improvement of pulmonary aeration, which showed a significant correlation to posttreatment reduction of oxygen requirement (p less than 0.0001). Asymmetric clearance was more often localized on the right side and usually disappeared within two to five days. Only in 13 patients no change of ventilation was found. Development of interstitial emphysema (n = 24, including three patients with pneumothorax) after surfactant treatment was an unfavourable prognostic sign. 54% of these patients (13 of 24) died within the first month of life, compared to 8% (7 of 93) in the group of patients with initial improvement of ventilation.

Persistent pulmonary hypertension of the newborn (PPHN), initially described by Gersony et al as persistent foetal circulation (PFC syndrome), results from a flawed transition from foetal to extrauterine pulmonary circulation. It is characterised by the maintenance of a high pulmonary vascular resistance and right-to-left shunting through the ductus arteriosus and foramen ovale. Infants with a wide variety of underlying clinical conditions develop PPHN. According to Rudolph three main anatomic types of PPHN can be identified: normal pulmonary vascular development increased pulmonary vascular smooth muscle development decreased cross-sectional area of pulmonary vascular bed. It is important to realize that several pathophysiologic mechanisms may coexist and interact. Besides metabolic and respiratory acidosis, hypercapnia and hypoxaemia some other factors induce pulmonary vasoconstriction. Thromboxane, leukotrienes and prostaglandins play a decisive role. Since PPHN can be associated with a broad spectrum of clinical conditions, a specific clinical picture is lacking. The baby is usually term or post-term, cyanotic immediately after birth or some hours later. Birth asphyxia, hyperviscosity, sepsis and aspiration of meconium have been recognized as predisposing factors. The diagnosis can be confirmed by echocardiography. Contrast echo will indicate right-to-left shunting with normal anatomy. Currently hyperventilation, tolazolin, chlorpromazin and dopamine/dobutamine have been advocated as central foci for clinical therapy. Recently prostacyclin was introduced as a specific pulmonary vasodilatator.(ABSTRACT TRUNCATED AT 250 WORDS)

In a retrospective study performed at the university department of pediatrics at Aachen the development of the mortality rate of extreme low birth weight infants (less than or equal to 1000 grams) born from 1976 to 1987 has been under investigation. During this period a decline of the neonatal mortality rate from 79.5% to 29.8% could be observed. Sex, gestational age, birthweight, place of birth and delivery mode were important factors influencing the outcome of the patients. Premature babies who were born by caesarean section had a significantly higher survival rate than babies who had been delivered vaginally. Premature babies born in the university hospital at Aachen (inborns) had a remarkably lower neonatal mortality rate than infants who postpartum had been transferred to the intensive care unit from surrounding hospitals (outborns). A second aspect of this study was the development of neonatal morbidity. For some diseases like intracranial hemorrhage a decrease in frequency could be noticed. Especially high-graded intraventricular hemorrhage (IVH greater than or equal to III degrees) occurred significantly less frequent between 1985 and 1987. The comparison of morbidity rates between inborns and outborns revealed a lower incidence of hyaline membrane disease, pneumothorax and intracranial hemorrhage in inborn patients. The results of this study imply that the intensive care of extreme low birth weight infants is justified. To achieve even lower mortality and morbidity rates the importance of regionalization has to be emphasized.

Repeated doses of Perfluorocarbon in saline-lavaged piglets: Effects on gas exchange and lung pathology

Combination of surfactant and partial liquid ventilation (PLV) in newborn surfactant depleted piglets

22 premature infants with a birth weight less than 1500 g were studied by computed tomography. All of them needed intensive care therapy and had symptoms, which may have been caused by cerebral haemorrhage. In 15 cases (= 68%) we found subependymal and intraventricular bleeding. Clinical data showed that ventilated premature infants with subependymal and intraventricular haemorrhage needed respiratory support in the first hours p.p. because of hyaline membrane and aspiration syndroms. Premature infants without cerebral haemorrhage were ventilated much later (mean 96 h. p.p.). All patients with intraventricular haemorrhage and ventricular enlargement with and without parenchymal haemorrhage (grade III and IV) died. Prematures with subependymal haemorrhage (grad I) and intraventricular haemorrhage without ventricular dilation (grade II) may survive, depending on eventually present pulmonary complications. The introduction of computed tomography in the examination of the CNS on prematures can therefore help to give an early prognosis.

Human fetal cells from 10 prematures and newborn infants (28–38 weeks of gestational age) and isolated or non-isolated fetal cells circulating in the blood of 9 primigravidae were studied in their ability to respond to phytohemagglutinin, pokeweed, dextran sulfate and lipopolysaccharide. An age-dependent responsiveness of fetal cells obtained from the prematures to all mitogens tested was detected as well as a clear graduation of mitogenic capacity with phytohemagglutinin to produce the highest stimulation. Though a moderate mitogenic response to lipopolysaccharide and dextran sulfate was noted in the blood cultures of the infants, LPS and in part DS transformation of fetal cells obtained from maternal blood appeared to be reduced or absent. A selective stimulation of fetal cells occurring in the circulation of primiparae sufficient for prenatal diagnosis could not be achieved with the mitogens tested. The findings suggest that fetal cells crossing to the mother are different from normal fetal lymphocytes. The present study was performed to elucidate in as quantitative a manner as possible the responses of human fetal cells to different T- and B-cell mitogens. Cells were obtained from various sources for comparing the mitogenic responses of isolated and non-isolated fetal cells. Our results demonstrate that mitogenic responses depend on the gestational age of the fetal cells, the source of the cells and on experimental conditions.