Enterostomy complications in infancy and childhood.

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.

Partial liquid ventilation with surfactant: effects on gas exchange and lung pathology in surfactant-depleted piglets.

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), PaO(2)/FIO(2) ratio, PaCO(2) 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 PaO(2)/FIO(2) ratio compared to the values after lung injury. However, at 18 and 24 h of PLV, the OI and PaO(2)/FIO(2) ratio were significantly worse in the S + PLV/CV and S + PLV/HFOV groups compared to the groups without S. PaCO(2) 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. CONCLUSIONS++: 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.

[Prevention of neonatal anemia with recombinant human erythropoietin: a cost-benefit analysis]. / Die Prophylaxe der Frühgeborenenanämie mit rekombinantem humanen Erythropoietin: eine Kosten-Nutzen-Analyse.

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 (p < 0.05). 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.

[Combination therapy of high frequency oscillatory ventilation, NO inhalation and surfactant replacement in a child with acute respiratory distress syndrome]. / Kombinierter Einsatz von Hochfrequenzoszillationsbeatmung, NO-Applikation und Surfactantsubstitution beim kindlichen ARDS.

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.

[Fiberoptic measurement of arterial oxygen saturation in premature and term neonates]. / Fiberoptische Messung der arteriellen Sauerstoffsättigung bei Früh- und Neugeborenen.

BACKGROUND: 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. METHODS: In ventilated premature infants (FiO2 > 0.4) 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. RESULTS: 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; p < 0.0001). CONCLUSION: In critically ill patients, if non-invasive oxygen monitoring fails, a fiberoptic catheter offers the possibility of continuous and reliable measurement of oxygen saturation.

The clinical outcome after inferior vena cava thrombosis in early infancy.

UNLABELLED: 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. CONCLUSION: 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.

Early versus late dexamethasone treatment in preterm infants at risk for chronic lung disease: a randomized pilot study.

UNLABELLED: Abstract 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 weight < or = 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. CONCLUSION: 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.

Inhaled budesonide in ventilator-dependent preterm infants: a randomized, double-blind pilot study.

The aim of this randomized, double-blind pilot study was to evaluate the short-term efficacy of early inhalation therapy with budesonide in ventilator-dependent preterm infants. The primary outcome variable was the duration of artificial ventilation; secondary outcome variables were the need for supplemental oxygen and the release of several inflammatory mediators in the tracheobronchial aspirate fluid. The infants of the budesonide group could not be weaned earlier from the ventilator. The ventilatory parameters on day 14 of life and the need for supplemental oxygen were similar in both groups. The release of inflammatory mediators was not reduced in the budesonide group. No adverse side effects were observed in either group. In conclusion, aerosolized budesonide failed to demonstrate significant short-term pulmonary improvement in ventilator-dependent preterm infants.

[Selenium status and bronchopulmonary dysplasia in premature infants <1,500 g]. / Selen-Status und Bronchopulmonale Dysplasie bei Frühgeborenen < 1500 g.

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 infants < 1500 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) (p < 0.001). 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).

Pharmacokinetics of oral fluconazole in premature infants.

UNLABELLED: 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 in preterm infants weighing < or = 1250 g: association with perinatal data and chronic lung 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).

The Cantrell-sequence: a result of maternal exposure to aminopropionitriles?

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.

Thoracic sequelae after surgical closure of the patent ductus arteriosus in premature infants.

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.

[Function of the hypothalamo-hypophyseal-adrenal axis to corticotropin-releasing hormone test in premature infants with bronchopulmonary dysplasia]. / Funktion der Hypothalamus-Hypophysen-Nebennierenrinden-Achse im Corticotropin-Releasing-Hormontest bei Frühgeborenen mit Bronchopulmonaler Dysplasie.

BACKGROUND: 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. METHODS: 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. RESULTS: 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. CONCLUSION: 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.

[Neonatal, neurologic and psychosocial findings in higher order multiple births. Follow-up for 3 to 10 years]. / Neonatologische, neurologische und psychosoziale Befunde bei höhergradigen Mehrlingen. Nachuntersuchungen über 3 bis 10 Jahre.

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.

Inflammatory reaction and capillary leak syndrome related to cardiopulmonary bypass in neonates undergoing cardiac operations.

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-alpha, 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 microgram/L vs 0.9 +/- 0.2 microgram/L) (mean +/- standard error of the mean) (p < 0.05) and higher leukocyte counts (leukocytes, 17.9 +/- 2.1 X 10(3) cells/ml versus 11.7 +/- 0.8 X 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 alpha 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-alpha 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.

Effect of sodium nitroprusside on complement activation induced by cardiopulmonary bypass: a clinical and experimental study.

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 o 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.