Quality of life in children with traumatic brain injury--basic issues, assessment, and recommendations.

An international Task Force was convened under the guidance of BMBF Conference so as to review the "State of the art" for measuring quality of life (QoL) in children who have suffered traumatic brain injury (TBI). After expert review of instruments and evaluation of two independent literature reviews this work group established "inclusion criteria" for the review of current tools that could contribute to the measurement of QoL in children with TBI. Six instruments were determined to meet all or most of the criteria required to be used in current clinical practice and research for children with TBI.

[Congenital Hyposplenia with multiple additional anomalies: a variant of the Ivemark Syndrome]. / Angeborene Hyposplenie mit multiplen Begleitfehlbildungen: Variante des Ivemark-Syndroms.

We report an 18-months-old boy with congenital spleen hypoplasia and cardiovascular defects. Besides, several minor clinical manifestations such as facial anomalies, hypospadia glans penis, agenesis of the corpus callosum and iris anomalies were observed. A partial or complete lateralisation defect could be excluded. The patient's phenotype comprises a previously undescribed combination of major and minor clinical features of the Ivemark syndrome. The case indicates that a common genetic defect might be the cause of this syndrome, which shows a variable expression.

[Effects of prepartum infusion solutions on glucose and bilirubin metabolism of mother and child in the prepartum and postpartum period]. / Auswirkungen von präpartal verabreichten Infusionslösungen auf den Zucker- und Bilirubinstoffwechsel von Mutter und Kind peri- und postpartal.

BACKGROUND: Administration of glucose 5% infusion is regularly used in obstetrics. The purpose of the study was to investigate the effects of glucose as compared to xylose and electrolyte solutions on parameters of maternal and fetal glucose and bilirubin metabolism during labour and after delivery. PATIENTS AND METHODS: 53 pregnant women (> or = 37 weeks of gestation, uncomplicated pregnancies) were randomised by entering the delivery ward. Under labour either glucose 5%, xylose 5% or electrolyte infusions were administered. Maternal serum glucose, serum osmolarity, insulin and glucagon were analysed before administration and 20 minutes after delivery. In the newborn blood osmolarity and serum glucose levels were analysed in the umbilical cord directly after birth and in capillary blood samplings 2 hours after birth. RESULTS: Maternal blood glucose levels 20 min. post partum were significantly different (p < 0.05). Maternal insulin and glucagon concentrations 20 min. pp showed similar trends with glucose levels but were not significantly different. Glucose levels in the umbilical cord were significantly higher in the glucose than in the electrolyte group, but not higher than in the Xylit group. In contrast, the glucose-levels in the newborns after 2 h were significantly higher in the glucose group compared with both other groups. No significant differences were observed in bilirubin levels. Osmolarity in the umbilical cord between groups differed significantly. CONCLUSIONS AND DISCUSSION: In conclusion, the administration of different solutions showed a distinct influence on the maternal and neonatal glucose metabolism. A significant impact on the bilirubin levels could not be shown in this study.

The effect of dexamethasone on respirator-dependent very-low-birth-weight infants is best predicted by chest X-ray.

BACKGROUND: Chronic lung disease (CLD) in premature infants shows a variable clinical course with different radiological manifestations. OBJECTIVE: To evaluate the correlation between parameters of trans-membrane permeability [albumin/secretory component (SC)] and oxidative stress [malondialdehyde (MDA)/SC] in tracheal aspirate fluid (TAF) and radiological findings with the effect of a 5-day course of dexamethasone (0.5 mg/kg per day). MATERIALS AND METHODS: Fifty ventilator-dependent premature infants with birth weights < 1,500 g (gestational ages 23-31 weeks) and radiological signs of early chronic lung disease (CLD) were treated with dexamethasone at day of life 5-27 (median 10 days) because of respiratory deterioration. TAF was collected serially. Chest X-rays taken before and 8-10 days after dexamethasone were scored for changes of opacification, consolidation and hyperinflation/emphysema, and classified into three groups. RESULTS: Twenty-four infants had a positive response to dexamethasone, defined as a reduction of the ventilation index FiO2 x mean airway pressure > 40% at day 5, compared to pretreatment values. About 80% of the responders showed homogeneous lung opacification on chest X-ray, reflecting leaky lung syndrome. In contrast, seven of eight infants with predominantly emphysema on radiology were non-responders; 80% of infants with a mixed radiological picture characterized by predominance of consolidations alternating with regions of emphysema were also non-responders. Ratios of albumin/SC and MDA/SC in TAF decreased significantly within 3 days after the onset of dexamethasone. However, MDA/SC was persistently higher in non-responders compared to responders. Opaque lungs were largely improved by dexamethasone, in contrast to streaky or patchy consolidations and emphysema. In a logistic regression model, radiographic classification was the most important factor influencing the response to dexamethasone with a positive predictive value of 86%, followed by albumin/SC ratio. CONCLUSIONS: The optimum timing of dexamethasone treatment may be determined by the stage of developing CLD and radiological findings rather than by the age of the premature infant.

Sonographic appearances of the abdominal manifestations of hereditary angioedema.

Hereditary angioedema (HAE) is the autosomal dominant deficiency of C1-esterase inhibitor. There have hitherto been no reports on the US appearances of HAE. The unique case of a 12-year-old girl with recurrent abdominal pain is reported, in whom HAE was diagnosed by US and family history of paroxysmal dyspnoea, cutaneous swelling and attacks of abdominal pain. Pertinent US features were intestinal oedema and ascites. Sonographic evidence of intestinal swelling was only seen on the initial day of an episode of abdominal pain. Oedema, as demonstrated by MRI the following day, regressed rapidly, whereas ascites persisted for at least 3 days. It is therefore important to perform imaging in the acute phase to demonstrate the massive intestinal oedema, which is characteristic for the disease.

Endobronchial juvenile hemangioma--a case report of a neonate including immunohistochemical monitoring and nuclear, cellular, and vascular morphometry.

A 3-month-old female child suffered from tachypnea and dyspnea with abnormal blood gas values. Chest X-rays revealed an increased transparency of the left lung and a mediastinal shift to the right side. High resolution computed tomography (CT) documented a narrowing of the left upper stem bronchus. Ensuing endoscopy detected an occlusive endobronchial tumor mass that did not infiltrate the bronchial cartilage as confirmed with endobronchial ultrasonic monitoring. Based on gross histological examination of the surgical specimen obtained using sleeve resection, the highly vascularized tumor exhibited an adenomatoid growth pattern with a rather homogeneous population of nuclei. The light microscopical presentation was consistent with a juvenile (infantile) hemangioma, which was confirmed using immunohistochemical examinations despite the display of neuroendocrine features. Although endobronchial juvenile hemangiomas are an extremely rare event in early childhood, this case underscores the necessity to not neglect its occurrence in differential diagnosis.

Lethal pneumococcal infection in an 18-month-old girl with splenic hypoplasia and dysgammaglobulinemia.

We report the case of an 18-month-old girl who died of overwhelming pneumococcal sepsis. Autopsy revealed a small spleen with unusual architecture. There was a marked rarefaction of the white pulp with only very few but florid germinal centers. Immunohistochemical staining showed a low number of T and B lymphocytes in the spleen, whereas normal numbers and distribution of lymphocytes were found in all other primary and secondary lymphatic organs. Whereas levels of IgM were normal, IgA and IgG levels were significantly lower than in age-matched controls. Consistent with serological data, B cells mainly expressed IgM and IgD, whereas IgG expression was lower than expected. Additionally, intestinal immunoglobulin distribution in B-cell areas of lymphofollicular hyperplasia showed normal expression of IgM, but almost no expression of IgA. A review of the literature failed to disclose a similar case of dysgammaglobulinemia associated with isolated structural spleen anomalies. We propose that the patient suffered from a defect of the B-cell differentiation pathway.

Risk factors associated with early-onset sepsis in premature infants.

OBJECTIVE: To define perinatal factors associated with early-onset neonatal sepsis. STUDY DESIGN: Maternal and neonatal variables were analysed retrospectively in 343 infants born before 35 weeks using univariate and multivariate statistical analysis. RESULTS: Logistic regression analysis identified risk factors for probable neonatal sepsis: gestational age at delivery (odds ratio 0.9, 95% confidence interval (CI) 0.91-0.96), premature rupture of the membranes (odds ratio 2.9, 95% CI 1.004-8.56), Apgar score after 1 min (odds ratio 0.7, 95% CI 0.53-0.96), and histological chorioamnionitis and/or funisitis (odds ratio 4.1, 95% CI 1.36-12.12). There was a strong association between probable sepsis and intracranial haemorrhage of the infant (odds ratio 4.3, 95% CI 1.07-17.40). Funisitis had a high specificity (91%) and positive predictive value (82%) for the detection of neonatal sepsis < or =32 weeks. CONCLUSIONS: Independent obstetrical risk factors for early-onset neonatal sepsis in premature infants may help to identify newborns who benefit from maternal antibiotic prophylaxis before birth. The histological examination of the umbilical cord can be used as an additional diagnostic test to detect newborns at risk of infection.

Critical illness neuropathy in pediatric intensive care patients.

Critical illness neuropathy is an axonal polyneuropathy recognized more frequently in adult intensive care patients with sepsis and multiple organ dysfunction. In children the diagnosis is rarely made. Within 1 year the authors observed two children with critical illness neuropathy. Both patients, a male 6 years, 6 months of age with a brain contusion and a male 2 years, 6 months of age who underwent craniectomy for Crouzon's disease, required prolonged mechanical ventilation and developed sepsis with multiple organ dysfunction. Three to 4 weeks after successful treatment of the sepsis, a flaccid tetraparesis was noticed in both patients. Laboratory investigations of blood and cerebrospinal fluid and spinal magnetic resonance imaging revealed normal results. Electrophysiologic examinations were indicative of an axonal polyneuropathy. Spontaneous improvement occurred within several months. It is likely that critical illness neuropathy occurs more often in critically ill children than previously thought. Careful neurologic examination and early electrophysiologic investigations are necessary to establish the diagnosis. Important differential diagnoses of acquired lower motor neuron weakness in pediatric intensive care medicine are discussed.

Possible role of uric acid as an antioxidant in premature infants.

Uric acid (UA) is a water-soluble antioxidant in human body fluids. In umbilical cord blood of 172 neonates with gestational ages between 22 and 42 weeks, UA concentrations were between 50 and 990 mumol/l. There was no correlation with gestational age. Infants with an umbilical cord pH < 7.0 had markedly lower UA concentrations in cord blood than neonates without severe acidosis. UA levels increased during the first 24 h of life and subsequently declined within 2 weeks to lower levels than in older children. In tracheal aspirates (TA), UA at concentrations of about 10(-4) mol/l was found capable of effectively scavenging free oxygen radicals. Luminol-dependent chemiluminescence activity of isolated TA phagocytes or alveolar macrophages was suppressed by UA dose dependently, with a 100% reduction at UA concentrations of 10(-3) mol/l. On the assumption that oxidative stress contributes to the development of chronic lung disease (CLD), in premature infants the ratio of the antioxidant UA and malondialdehyde (MDA) as a marker of oxidative injury was measured in serially obtained TA of 102 ventilated premature infants with birth weights < 1,500 g. At the age of 3-14 days, infants who later developed severe CLD had significantly lower TA UA/MDA ratios than infants without CLD development. In infants with moderate CLD, UA/MDA ratios were comparable to those in the non-CLD group until day 5. During the next 2 weeks, decreasing ratios of UA/MDA paralleled lung injury with increased oxygen requirement and microvascular permeability. Serum UA concentrations did not differ between groups. It is speculated that UA may be physiologically important as an antioxidant in the epithelial lining fluid of the respiratory tract in neonates during the 1st week of life.

Isolated dihydroxyacetonephosphate-acyl-transferase deficiency in rhizomelic chondrodysplasia punctata: clinical presentation, metabolic and histological findings.

UNLABELLED: Rhizomelic chondrodysplasia punctata (RCDP) is clinically characterized by symmetrical shortening of the proximal limbs, contractures of joints, a characteristic dysmorphic face, and cataracts. In the classical form an impairment of several peroxisomal functions and enzymes (plasmalogen synthesis, phytanic acid oxidation, 3-oxoacyl-CoA thiolase) has been repeatedly shown. Recently a variant involving only the peroxisomal dihydroxyacetonephosphate acyltransferase (DHAP-AT) has been described. We present a patient with isolated DHAP-AT deficiency and all clinical, radiological and pathological features of classical RCDP. For the first time, microscopy and immunocytochemistry of hepatocytes could be performed. CONCLUSION: In contrast to studies on classical rhizomelic chondrodysplasia punctata which have shown enlarged peroxisomes in numbers varying from hepatocyte to hepatocyte, the peroxisomes in our patient seem to be normal in size, number and shape.

Chemiluminescence activity of phagocytes from tracheal aspirates of premature infants after surfactant therapy.

The effect of surfactant on the respiratory burst of phagocytic cells was studied in the tracheobronchial tract of 40 mechanically ventilated neonates (gestational age 24-37 weeks) over the first week of life. We measured the luminol-dependent chemiluminescence (CL) activity of granulocytes and macrophages isolated from tracheal aspirates in 23 preterm infants 1-6 days after administration of bovine surfactant and in 17 untreated controls. Following stimulation by the chemotactic peptide N-formylmethionylleucylphenylalanine, CL activity was not or only slightly impaired in the surfactant group. In contrast, treatment with exogenous surfactant significantly reduced CL response to opsonized zymosan (OPZ), which involves phagocytosis, for up to 6 days (p < 0.05). The impairment of CL activity seemed to be dose dependent, as repeated surfactant applications (cumulative phospholipid dose of 200 mg/kg) reduced OPZ-elicited CL activity to a greater extent than application of a single dose of 100 mg/kg. In agreement with in vitro studies, our data suggest that high-dose application of exogenous surfactant may affect the antibacterial function of phagocytic cells in the lung.

Recurrent bacterial meningitis.

UNLABELLED: To characterize recurrent bacterial meningitis in children, we reviewed the charts of all patients treated for more than one episode of bacterial meningitis at the Würzburg University Children's Hospital from 1980 to June 1995. Twenty-five children suffered 2-13 episodes of bacterial meningitis. Most patients were referred from other hospitals to our paediatric neurosurgical service. No immunodeficiency was found. In all patients, the cause of recurrent meningitis was an anatomical lesion with 13 intracranial defects including encephaloceles, skull fractures, Mondini dysplasias, neurenteric cyst, fibrous dysplasia, persistent craniopharyngeal duct, and 12 lumboscral defects with a dermoid cyst within the lumbosacral spine. A first episode of meningitis at school age did not exclude a congenital defect. In total, 84 episodes of meningitis were treated, a pathogen was isolated in 77%. The most common pathogen was Streptococcus pneumoniae, followed by Escherichia coli, Staphylococci and others. The pathogen isolated often gave a clue to the location of the defect. Personal history was often unrewarding and in some cases the search for the anatomical lesion required repeated imaging and explorative surgery. In 24 of 25 cases, final treatment of recurrent meningitis was by surgical intervention. CONCLUSION: In recurrent bacterial meningitis, excessive diagnostic and therapeutic procedures are indicated. An anatomical defect is a very probable cause.

Immunologic parameters in cord blood indicating early-onset sepsis.

Different immunologic parameters were measured in cord blood to test their usefulness in the early diagnosis of early onset sepsis. Cord blood levels of circulating intercellular adhesion molecule-1 (cICAM-1), interleukin-6 (IL-6) and interleukin-8 (IL-8) were significantly elevated in septic compared to nonseptic neonates. No significant difference between either population was seen for cord blood C3a and elastase-alpha 1-proteinase inhibitor complex (E alpha 1 PI). Measured concentrations of cICAM-1, IL-6 and IL-8 in fetal and maternal blood did not correlate, indicating that the neonate's response to sepsis is clearly different from the mother. Our data suggest that cord blood measurements of cICAM-1, IL-6 and IL-8 might be useful in identifying neonates with early-onset sepsis.

[Antepartum prevention and postnatal therapy of respiratory distress syndrome]. / Präpartale Prophylaxe und postnatale Therapie des Atemnotsyndroms.

The introduction of surfactant in the therapy of respiratory distress syndrome (RDS) reduced mortality and long term complications in very premature infants. Nevertheless, the obstetric management influences critically the outcome. In a prospective study of 116 premature infants with RDS treated with natural surfactant preparations after birth, mortality was significantly reduced by antepartum corticosteroid therapy suggesting a synergistic effect of corticosteroids and surfactant on the immature lung. It is assumed that a preventive administration of surfactant immediately after birth would benefit neonates at risk for RDS more than a delayed surfactant replacement after the development of RDS. But without a reliable assessment of fetal lung maturity before birth more than 50% of our premature infants with birth weights less than 1500 g would be exposed to surfactant unnecessarily. It is important that fetal asphyxia is avoided. Acquired respiratory distress syndrome occur even in premature infants after shock or meconium aspiration and may respond poorly to surfactant replacement. This is also the case in lung hypoplasia or perinatal infection, where the combined efforts of obstetricians and neonatologists are needed to attain better results.

[Effect of body position and positioning changes on lung function of ventilated premature and newborn infants]. / Einfluss von Körperposition und Lageveränderungen auf die Lungenfunktion beatmeter Früh- und Neugeborener.

We studied 21 intubated premature infants (wts 800-2800 g) with respiratory distress syndrome between day 2 and 10 to evaluate the effect of body position on lung mechanics and gas exchange. The dynamic compliance of the total respiratory system was similar in the prone and supine position. When the infant was turned from the supine or the prone position to the other one, a significant improvement of oxygenation was seen temporarily. Positioning did not significantly affect the dynamic compliance, the minute volume or pCO2. In circulatory stable premature infants a change of the body position probably alters the regional ventilation to perfusion ratio and leads to a reduction of intrapulmonary venous admixture.