[Life-threatening macroglossia following cleft palate palatoplasty]. / Lebensbedrohliche Makroglossie nach Gaumenspaltenverschluss.

The case of a 13-month-old child who developed a life-threatening macroglossia with airway obstruction following palatoplasty for a cleft palate is reported. As direct laryngoscopy was not feasible a laryngeal mask (LM) was inserted to secure the airway. Under fiber optic guidance an endotracheal tube was then introduced via the LM. In this article the incidence, pathophysiology, clinical dynamics, options for emergency anesthesia management and organizational implications of this rare but typical complication in the field of oral and craniomaxillofacial surgery are reported.

Balloon gastrostomy buttons in pediatric patients: evaluation with respect to size, lifetime in patients, and parent acceptance.

BACKGROUND AND OBJECTIVE: To evaluate the experience with "balloon" gastrostomy buttons in pediatric patients. Distributions of the shaft lengths and the longevity of the balloon tubes were examined. Parents' and caregivers' opinion about this type of tube were analysed. METHODS AND PATIENTS: Retrospective chart review (n=38) and short questionnaire (n=21) during regular follow up visits. RESULTS: The mean longevity of balloon buttons was 193 days. 90.7% shaft lengths were from 1.5 to 2.3 cm. 100% of caregivers evaluated handling of the button tube as equal or better (81%) than a conventional PEG tube. "Overall" satisfaction was equal in 5% and better in 85% of cases. CONCLUSION: Button tubes have to be replaced about 2 times a year. In pediatrics typical shaft lengths are between 1.5 and 2.3 cm. A majority of care giving persons prefers the button tube in comparison with PEG-tubes. Thus, button tubes should be recommended for long-term enteral nutrition in order to improve the patients' quality of life.

Multiple types of cardiac arrhythmias in a child with head injury and raised intracranial pressure.

Arrhythmias occur as a life-threatening complication in adults with severe head injuries. A wide spectrum of brady- and tachyarrhythmias and different pathogenetic mechanisms have been described. We report an 8-year-old boy with traumatic brain injury who developed a variety of independent types of arrhythmias during the course of his illness, including supraventricular and ventricular extrasystoles, prolonged QT duration and ventricular fibrillation, accelerated junctional rhythm, and reentry tachycardia. Each arrhythmia may have had a distinct pathogenic pathway, and not all were associated with raised intracranial pressure.

[Management of septic shock and acquired respiratory distress syndrome in pediatric cancer patients]. / Intensivmedizinische Therapie infektiöser Komplikationen in der pädiatrischen Hämatologie und Onkologie.

Septic shock occurs in 6 % of paediatric cancer patients with neutropenia and fever. The mortality of the septic shock is 40 % in BMT patients and 5 % in others. One third of paediatric ARDS cases affect immunocompromised individuals with a total mortality of 45 % and 80 % after BMT. Septic shock is caused by gram-negative bacteria in more than 75 %. ARDS is due to pneumonia in more than 50 %, sepsis in about 25 %. This article provides the recommendations of the Infectious Diseases Working Party of the German Society for Pediatric Infectious Diseases (DGPI) and the German Society for Pediatric Hematology/Oncology (GPOH) for treatment of septic shock and ARDS. Therapy of septic shock includes early antibiotic therapy and volume expansion (> or = 40 ml/kg initially). Refractory shock requires vasopressors (noradrenaline), followed by a judicious circulatory management. Hydrocortisone is indicated in patients with high probability of adrenal insufficiency. Mainstay of ARDS therapy is ventilation with sufficient end-expiratory pressure (PEEP) to prevent loss of functional residual capacity and with limited tidal volumes (< or = 6 ml/kg) and limited inspiratory pressure (< 35 cm H(2)O) respectively, to minimize ventilator induced lung injury. Volume therapy consists of maintenance of sufficient preload to counteract the impaired venous return, induced by positive pressure ventilation. Diuretics and eventually veno-venous haemofiltration are used to reduce free lung water. Surfactant application may be considered in severe cases. Steroids are indicated in pneumocystis carinii pneumonia and in engraftment pneumonitis.

[Protein-loosing enteropathy: report of four cases and review of etiology, diagnostic work-up and treatment]. / Enteraler Eiweisserlust: Pathophysiologie, Ursachen, Diagnostik und Therapie mit Fallbeispielen.

The protein-loosing enteropathy (PLE) may result from a broad variety of underlying diseases. These conditions are of systemic nature or locally affecting the gastrointestinal tract. Major symptoms are oedema due to low plasma protein levels. Gastrointestinal symptoms are not necessarily present. The diagnosis is confirmed by the finding of increased faecal concentrations of Alpha-1-Antitrypsin (> 320 mg/L). In the majority of cases, in which underlying diseases are present, the etiology is obvious. In unclear cases the differentiation into inflammatory or circulatory disturbances or alterations of the architecture of the basal membrane is helpful. An economic, staged approach is presented. To localize the site of protein loss imaging is required (abdominal ultrasound, CT-scan, endoscopy and Technetium-Scan). If a circumscribed intestinal source of protein loss is suspected which may be amenable to surgery, intraoperative enteroscopy should be considered. If causal treatment is impossible; intravenous replacement of albumin and immunoglobulines in intervals from 1 to 4 weeks will be necessary. The prognosis in patients with isolated PLE is good. Otherwise it depends on the underlying disease.

Plasma levels of 24S-hydroxycholesterol reflect the balance between cerebral production and hepatic metabolism and are inversely related to body surface.

We have previously presented evidence that most of the 24S-hydroxycholesterol present in the circulation originates from the brain and that most of the elimination of this oxysterol occurs in the liver. Plasma 24S-hydroxycholesterol levels decline by a factor of about 5 during the first decades of life. The concentration of the enzyme cholesterol 24S-hydroxylase in the brain is, however, about constant from the first year of life, and reduced enzyme levels thus cannot explain the decreasing plasma levels during infancy. In the present work we tested the hypothesis that the plasma levels of 24S-hydroxycholesterol may reflect the size of the brain relative to the capacity of the liver to eliminate the substance. It is shown here that the age-dependent changes in absolute as well as cholesterol-related plasma level of 24S-hydroxycholesterol closely follow the changes in the ratio between estimated brain weight and estimated liver volume. The size of the brain is increased only about 50% whereas the size of the liver is increased by about 6-fold after the age of 1 year. Liver volume is known to be highly correlated to body surface, and in accordance with this the absolute as well as the cholesterol-related plasma level of 24S-hydroxycholesterol was found to be highly inversely correlated to body surface in 77 healthy subjects of varying ages (r(2) = 0.74). Two chondrodystrophic dwarves with normal size of the brain but with markedly reduced body area had increased levels of 24S-hydroxycholesterol when related to age but normal levels when related to body surface. It is concluded that the balance between cerebral production and hepatic metabolism is a critical determinant for plasma levels of 24S-hydroxycholesterol at different ages and that endocrinological factors are less important. The results are discussed in relation to the possibility to use 24S-hydroxycholesterol in the circulation as a marker for cholesterol homeostasis in the brain.

[Surveillance of nosocomial infections: prospective study in a pediatric intensive care unit. Background, patients and methods]. / Surveillance nosokomialer Infektionen: Prospektive gezielte Erfassung von nosokomialen Infektionen auf einer pädiatrischen Intensivstation.

UNLABELLED: BACKGROUND, PATIENTS AND METHODS: From November 1997 through May 1998, the incidence of nosocomial infections was studied prospectively in a 10-bed multidisciplinary pediatric intensive care unit in Germany. A standardized surveillance [SEKI] system based on the National Nosocomial Infection Surveillance [NNIS] System of the Centers for Disease Control and Prevention [CDC] was used. The CDC definitions for nosocomial infections were adapted to the current practice of pediatric intensive care in Germany. Infection rates were calculated as infections per 100 patients, per 1000 patient-days, and per 1000 device-days (central venous catheters, urinary-catheters, and mechanical ventilation). RESULTS: Fifteen nosocomial infections were recorded in 201 patients during 1035 patient-days. The overall nosocomial infection rates were 7.5/100 patients and 14.5/1000 patient-days. Device-associated nosocomial infection rates for urinary-catheters and mechanical ventilation were 7.2/1000 utilization-days and thus below the 75th percentile of the last NNIS report. Central line infection rates were 10.7/1000 utilization days and therefore above the 75th percentile of the NNIS data (10.2/1000). The median length-of-stay was 5.1 days. CONCLUSIONS: Surveillance data are indispensable for internal and external quality control, and prospective surveillance of nosocomial infections should become an essential component of hospital infection control programs in pediatric intensive care in Germany. The standardized calculation of (device utilization ratios and) device-specific infection rates yields results which can be compared with national and international surveillance data. SEKI meets the criteria of a practice oriented, prospective and standardized surveillance system. Considerable efforts for collecting and interpreting the required data should be balanced against the benefit of prevention of nosocomial infections in this population of critically ill persons.

Dual versus triple therapy of Helicobacter pylori infection: results of a multicentre trial.

OBJECTIVE: To compare dual therapy (omeprazole and amoxicillin) with triple therapy (omeprazole, amoxicillin, and clarithromycin) in the treatment of Helicobacter pylori infection. The efficacy of 1 mg/kg/day omeprazole was randomly compared with 2 mg/kg/day. STUDY DESIGN: 252 patients (median age, 11.0 years; range, 3-18) presenting with chronic abdominal pain underwent endoscopy and a 13C-urea breath test. Gastric biopsy specimens were taken for histological examination and for the rapid urease test. Patients were treated for two weeks: group A (n = 63) received amoxicillin (50 mg/kg; maximum, 2 g/day), group B (n = 73) received amoxicillin and clarithromycin (20 mg/kg; maximum, 1 g/day). Both groups were randomly treated with either 1 or 2 mg/kg omeprazole (maximum, 80 mg/day). Diagnostic procedures were repeated four weeks after the end of treatment. RESULTS: 11 patients were excluded; 136 patients were H pylori positive (56%), 105 of whom were re-examined after treatment. Helicobacter pylori was eradicated in 52% of group A and 83% of group B. The dose of omeprazole had no influence on the eradication rate. Specificity and sensitivity of the rapid urease test were 94% and 93%, respectively. Specificity and sensitivity of the 13C-urea breath test were 93% and 95%, respectively. CONCLUSIONS: Dual therapy can no longer be recommended. Triple therapy is more effective than dual therapy in the eradication of H pylori infection. The lower dose of 1 mg/kg omeprazole was as effective as 2 mg/kg.

[Peliosis hepatis with hepato-pulmonary syndrome]. / Peliosis Hepatis with Hepato-Pulmonary Syndrome.

Sinusoidal dilatation is a hallmark of peliosis hepatis, a rare vascular disorder that can be either drug induced or of infectious origin. Here we report a patient with peliosis hepatis of unknown etiology. An hepato-pulmonary syndrome developed which was reversible following liver transplantation.

A new mutation of the glucose-6-phosphatase gene in a 4-year-old girl with oligosymptomatic glycogen storage disease type 1a.

A 4-year-old German girl was diagnosed as having glycogen storage disease type la and showed no other marked symptoms except hepatomegaly. The glucose-6-phosphatase activity in the liver was approximately 1.5% to 5.0% of normal values, and molecular analysis revealed compound heterozygosity for R83C and the novel mutation N264K. This result indicates that there is a wide clinical variation of glucose-6-phosphatase deficiency. DNA analysis is helpful for confirmation of the diagnosis, as well as establishment of the genotype and phenotype correlation in glycogen storage disease type 1a.

Effect of a German Poison Control Center on health care cost reductions in harmless exposure cases.

To evaluate the effect of a German Poison Control Center (PCC) service on health care cost reduction in cases of harmless exposures, a prospective telephone survey of 200 callers were asked which medical aid would have been sought if no PCC service had been available. Calculation of savings was based on the total number of calls and standardized fees for medical treatment. Savings in costs for health insurance in these cases was 20 to 50% of the annual PCC budget. The cost effectiveness of this German PCC for this patient group was lower than for North American PCCs. Cost effectiveness might be improved by making efforts to increase the number of public calls.

Cisapride reduces postoperative gastrocaecal transit time after cardiac surgery in children.

OBJECTIVE: To investigate the influence of the prokinetic drug cisapride on gastrocaecal transit time (GCTT) in children after open heart surgery. DESIGN: Prospective, randomized and controlled study. SETTING: Interdisciplinary paediatric intensive care unit in a tertiary-care children's hospital. PATIENT: Twenty-one children with a median age of 6.2 years on day 1 after uncomplicated open heart surgery for isolated septal defects, acquired mitral or aortic valve disease or tetralogy of Fallot. Control group consisting of 10 healthy children with a median age of 8.1 years. INTERVENTIONS: Ten children were randomized to receive cisapride 0.2 mg/kg body weight, 30 min prior to measurement of GCTT. MEASUREMENTS AND RESULTS: GCTT was measured using hydrogen breath testing with a test solution containing lactulose and mannitol (0.4 g/kg and 0.1 g/kg body weight respectively). GCTT was markedly delayed in all patients compared to the control group. Within 8 h 8/10 patients in the treatment group versus 4/11 patients in the non-cisapride group achieved gastrocaecal transit. No adverse side-effects were observed. CONCLUSIONS: Cisapride accelerates gastrocaecal transit after open heart surgery in children. In intensive care patients on inotropic support or opioid medication, it may facilitate the earlier reintroduction of enteral feeding.

[Cerebral nodular and laminar heterotopic tissue. 2 case reports and review of the literature]. / Cerebrale noduläre und laminare Heterotopie. 2 Fallberichte und Literaturübersicht.

Cerebral heterotopia indicating disturbance of neuronal migration is very rare. There are two kinds of cerebral heterotopia, the laminar and the nodular. The localisation may be diffuse or focal. Not infrequently isolated cerebral heterotopias have no clinical relevance. Typical symptoms on the other hand are psychomotoric retardation and epilepsy.

Aerosolised prostacyclin for pulmonary hypertension in neonates.

Aerosolised prostacyclin (PGI2) was administered to two neonates with pulmonary hypertension to dilate the pulmonary vessels selectively. The alveolar-arterial oxygen gradient fell from 73.2 (patient 1) and 71.8 kPa (patient 2) to 33.8 and 26.7 kPa respectively. Systemic blood pressure remained stable while pulmonary blood pressure declined in patient 1. When inhaled, PGI2 seems to improve oxygenation, mainly due to reduction of intrapulmonary shunting.

[Transfusion-associated acute pulmonary insufficiency: a rare, but life-threatening transfusion reaction]. / Die transfusionsassoziierte akute Lungeninsuffizienz: eine seltene, aber lebensbedrohliche Transfusionsreaktion.

Two patients developed within several hours after blood transfusion severe shortness of breath which required temporary artificial ventilation. X-ray pictures of the chest showed pulmonary edema in both patients. No cardiac causes for edema were found. Detection of granulocyte antibodies in the sera of one patient and one blood donor confirmed the suspected diagnosis of transfusion-related acute lung injury.

[Transfusion associated acute pulmonary insufficiency. Diagnostic confirmation by the demonstration of granulocytic antibodies]. / Transfusionsassoziierte akute Lungeninsuffizienz. Diagnosesicherung durch den Nachweis granulozytärer Antikörper.

In two patients, a 50-year-old woman (case 1) and a 2-year-old girl (case 2) acute shortness of breath requiring artificial ventilation developed 3-5 hours after infusion of two erythrocyte concentrates in case 1 and unfiltered platelet-enriched plasma (20 ml/kg) in case 2. The chest radiograph showed diffuse infiltrations in the lungs of both patients. After administration of catecholamines and respirator therapy, extubation was possible in the first patient after two days, in the second after five days. In neither case had there been any evidence of hypervolaemia, heart failure or infection to explain the lung findings. However, the serum of case 1 and the serum of the platelet donor had antibodies against granulocytes. The granulocyte-compatibility test (between patient serum and donor granulocytes) was positive. This confirmed the clinical suspicion of transfusion-related acute lung injury (TRALI). However, demonstration of antibodies was not only important for the diagnosis, but made it possible to recognize a blood donor whose serum contained antibodies against granulocytes which can provoke TRALI.