Septic shock in children in an urban area in Western Germany--outcome, risk factors for mortality and infection epidemiology.

BACKGROUND: Only sparse data exist about children with septic shock in Europe. The present study aimed to evaluate demographics, treatment, outcome and risk factors for mortality in Western Germany. PATIENTS: Children with septic shock aged 2 months to 17 years. METHODS: In a multi-center retrospective study of 20 children's hospitals data were obtained and analyzed by chart review. Risk factors for mortality were identified and assessed by multivariate regression analysis. RESULTS: Overall mortality in 83 cases with septic shock was 25% (21 patients). Significant risk factors were high PRISM III score, low pH, low arterial systolic blood pressure, presence of disseminated intravascular coagulation and extent of multi-organ failure, but not lactate (p=0.05) and base excess (p=0.065). Mortality in hospitals which treated 10 or more patients (category 1) was 17% and increased to 22% in hospitals which treated 3-6 patients (category 2). In hospitals with only 1 or 2 patients (category 3) mortality rate was 61% (p<0.01 when compared to category 1 or 2). A stepwise increase was also seen in the severely sick patients according to PRISM III (>19): category 1: 23%, category 2: 40%, category 3: 62.5% (p<0.05 for comparison of category 1 and 3). Multivariate analysis of significant risk factors revealed low number of treated patients as the only individual risk factor for mortality. CONCLUSION: Mortality from pediatric septic shock in an urban area in Western Germany is high. Disease severity and treatment in a department with few cases were associated with increased mortality.

Pulmonary sarcoidosis in a 14-year-old boy diagnosed by low-dose CT-guided transthoracic lung biopsy.

Pulmonary sarcoidosis is a rare disease in the pediatric age group, characterized by the presence of epitheloid-cell granulomas. In stage 3 sarcoidosis, pulmonary infiltrates without hilar lymphadenopathy occur. Definitive diagnosis requires a histopathological specimen, which might be difficult to obtain by transbronchial biopsy. Multidetector computed tomography (MDCT)-guided transthoracic lung biopsy (TLB) is a well-established procedure in adults, but has only rarely been applied in children.A 14-year-old boy was admitted to hospital for evaluation of a chronic systemic disease with severe pulmonary manifestation. All investigations, including bronchosopy and bronchoalveolar lavage with microbiological and virological testing, had been negative. MDCT-guided TLB was performed on a 16-section scanner with a low-dose protocol (single slices, 120 kV, 20 mAs), using a 16-gauge biopsy device. The total effective dose was 0.4 mSv for the biopsy procedure. Histopathological examination revealed multiple epitheloid-cell granulomas with giant cells in the absence of microbiological or virological abnormalities. A diagnosis of stage 3 pulmonary sarcoidosis was made and systemic anti-inflammatory therapy was administered, which led to complete remission within weeks. MDCT-guided TLB can be a valuable instrument in assessing pulmonary manifestations of pediatric sarcoidosis, enabling precise histopathological diagnosis and adequate therapy. The use of low-dose protocols can substantially reduce radiation exposure without relevant loss of image information. MDCT-guided lung biopsy should be considered prior to open-lung surgery in selected patients with unclear pulmonary disease.

Hydrocephalus internus in two patients with 5,10-methylenetetrahydrofolate reductase deficiency.

Hydrocephalus internus (HCI) of all four ventricles in association with early neurological abnormalities is described as the presenting symptom in two patients with 5,10-methylenetetrahydrofolate reductase (MTHFR) deficiency. Decreased activity of MTHFR leads to reduction of 5-methyltetrahydrofolate, the main methyl donor for methionine synthesis necessary for synthesis of S-adenosyl-methionine (SAM). Demyelination in MTHFR deficiency has been attributed to low SAM levels in the brain. The biochemical hallmarks of the disorder are hyperhomocystinemia, homocystinuria and low levels of plasma methionine. Hydrocephalus internus requiring neurosurgical intervention has to our knowledge not been reported as a presenting feature of homocystinuria due to deficiency of MTHFR so far. The surprising finding of HCI of all four ventricles in MTHFR deficiency must be kept in mind when evaluating patients with hydrocephalus of unknown origin.

Deficiency of cartilage in the segmental bronchi of a child: congenital or acquired?

The case of a 4-year-old girl suffering from a severe bronchopneumonia of the left lower lobe is presented. Microscopically no cartilage was found in the resected lobe distal to the lobar bronchus. The case is unusual because a congenital lobar emphysema associated with an aplasia of bronchial cartilage has to be considered as well as secondary cartilage destruction due to chronic relapsing inflammations.

[Effects of amrinone in myocardial insufficiency following heart surgery in children]. / Wirkungen von Amrinon bei Myokardinsuffizienz nach Herzoperationen im Kindesalter.

11 infants and children with severe congestive heart failure after cardiac surgery were treated with Amrinone. Cardiac and circulatory effects were monitored by quantitative echocardiography and blood pressure measurements. Within 1 day after the initial dose ventricular ejection fractions (EF) increased. Left ventricle: EF = 0.35 +/- 0.06 to EF = 0.49 +/- 0.06; right ventricle: EF = 0.30 +/- 0.12 to EF = 0.56 +/- 0.12. Vascular resistance dropped from R = 1527 +/- 517 dyn.s.cm-5.m2 to R = 1071 +/- 399 dyn.s.cm-5.m2. Blood pressure and heart rate remained almost unchanged. It is concluded, that Amrinone acts as a positive inotropic and vasodilating drug with the potential for effective treatment in children where conventional therapy has failed.

Verapamil-induced ventricular tachycardia in hypertrophic cardiomyopathy.

A 17-year old boy with hypertrophic obstructive cardiomyopathy (HOCM) developed repeated short runs of self terminating ventricular tachycardia 12 hours after starting oral verapamil, which was substituted for propranolol therapy. It is suggested that alterations in sympathetic tone induced by changing from a beta-blocker to a calcium antagonist might have increased myocardial irritability thus favouring genesis of severe ventricular arrhythmias. Verapamil is often considered to be more effective than propranolol in the treatment of HOCM. However, if propranolol is replaced by verapamil one has to take account of the possibility of serious arrhythmias as demonstrated by our case.

Value of image enhancement and injection of contrast medium for right ventricular volume determination by two-dimensional echocardiography in congenital heart disease.

To determine factors that influence the accuracy of echocardiographically estimated right ventricular volume and to improve the echocardiographic input information by applying image enhancement techniques, quantitative contrast echocardiography (4-chamber view) and biplane angiocardiography were performed in 23 children during routine diagnostic cardiac catheterization. Volumes calculated on the basis of unprocessed and processed echocardiographic cross sections (area-length method and sphere model) underestimated angiocardiographic volumes significantly (p less than 0.01), and more so in end-diastole (50.6%) than in end-systole (35.9%). Thus, ejection fraction was significantly (p less than 0.01) underestimated; mean values were 0.48 +/- 0.12 and 0.60 +/- 0.08, respectively. The best comparison between echocardiography and angiocardiography at end-diastole was achieved with the sphere model using image enhancement techniques and injection of contrast media, where y = 0.54x - 6.8, r = 0.97, sy.x = 7.3. Correlations, however, in which unprocessed echocardiograms were used showed only slightly less good correlations. With the 6 image-enhancement techniques, a more homogeneous structure of the image and a more distinct outline of the internal surface was achieved. The statistical error improved only slightly. The echocardiographic 4-chamber view allows right ventricular volume determination with an acceptable accuracy. Its underestimation is related to inadequate visualization of trabeculations and mainly to the models used. Application of image enhancement techniques allows easier outlining of the internal cavity surface. The advantage gained by the combination of contrast infection and image enhancement techniques does not warrant the routine central injection of available contrast material.