Hintergrund: Epidemiologische Daten zum ARDS sind unentbehrlich zur Planung entsprechender klinischer Studien und zur ökonomischen Allokation von Ressourcen (z.B. ECMO-Zentren). Inzidenz: Zugrunde liegt die Definition der Amerikanisch-Europäischen Konsensus-Konferenz von 1992. Die Inzidenz des ARDS bei Kindern jenseits des Neugeborenenalters liegt in drei populationsbezogenen Studien übereinstimmend bei 3/100000a für die Altersgruppe 0,1–18 Jahre. Dies entspricht deutschlandweit (2007: 13,97 Mio Einwohnern <18 Jahren) ca. 400–450 Fällen. Es besteht eine schiefe Altersverteilung, ca. 40% der Fälle treten im ersten Lebensjahr auf. Aetiologie und Grundkrankheiten: Etwa ein Drittel der Patienten weisen eine angeborene oder erworbene Störung des Immunsystems auf, nur ca. 40% erkranken ohne chronisches Grundleiden. Hier stellen die Meningokokkensepsis und Ertrinkungsunfälle die häufigsten Ursachen dar. Verlauf: Die Letalität liegt derzeit, zwischen ca. 30% in entwickelten Industrieländern und 60% in Schwellenländern. Das Letalitätsrisiko ist bei immunsupprimierten Patienten, Patienten mit akuter ZNS-Schädigung und Patienten mit Multiorganversagen besonders erhöht. Je nach Case-mix sterben bis zu 50% der Patienten an zerebralen Komplikationen. Ca 40% der Patienten, die einer ECMO-Therapie zugeführt werden, versterben. Hauptrisikofaktoren sind Schock und schwerste Hypoxämie vor ECMO-Beginn.

Epidemiologie: Beatmungs-assoziierte Pneumonien stellen die relativ häufigste „device-assoziierte“ infektiöse Komplikation in der Intensivmedizin dar. Die mittlere Inzidenz beträgt 5,5/1000 Anwendungstage in der Gesamtpopulation bzw. 1,7/1000 auf Kinder-Intensivstationen (KISS 2003–07). Sie verlängern signifikant die Beatmungsdauer, während eine kausal erhöhte Sterblichkeit nicht bei Kindern belegt ist. Definition und Diagnose: Die CDC-Definition fordert das neue Auftreten (mindestens 48 stündige Beatmung) klinischer Symptome (verändertes Trachealsekret, verschlechterter Gasaustausch) und einer systemischen Entzündungsreaktion zusammen mit pneumonischen Veränderungen im Röntgenbild. Die Spezifität der Diagnose und die Antibiotikatherapie können durch eine (i.d.R. nicht-bronchoskopische) broncho-alveoläre Lavage mit quantitativer Keimanalyse erhöht werden; „Protected-brush“-Proben sind für Kinder nicht validiert und insbesondere bei niedrigem Gewicht unpraktikabel. Erreger und Infektionswege: Häufigste Erreger sind S. aureus, Pseudomonas aeruginosa und Enterobacteriaceae. Die Infektion erfolgt über eine absteigende Besiedlung entlang der Tubusoberfläche, die Aspiration aus dem Hypopharynx, insbesondere bei gastroösophagealem Reflux, exogene Kontamination bei Eingriffen oder auch hämatogen. Risikofaktoren: Gesicherte Risikofaktoren sind eine neuromuskuläre Blockade, Intra- und Interhospital – Transporte, Immundefizienz, Bronchoskopie, Reintubationen, vorausgehende Antibiotikatherapie und kontinuierliche enterale Ernährung. Entsprechend ist die Inzidenz bei neurochirurgischen Intensivpatienten besonders erhöht. Präventionsmassnahmen: Allgemeine Hygienemassnahmen, bevorzugt nicht-invasive Beatmung, orale Intubation, Reduktion der pharyngealen Keimdichte (Verzicht auf großzügige Antazidatherapie, Oberkörper-Hochlagerung), evtl. Selektive Dekontamination des Darmtraktes (SDD) und bakterizide Tubusbeschichtungen.

Background and objective: To evaluate the experience with 'balloon" gastrostomy buttons in pediatric patients. Distributions of the shaft lengths and the ongevity 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 2.3cm. 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 2.3cm. 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.

Objective: We give the first account of failure of extracorporeal membrane oxygenation therapy secondary to congenital cystic malformation of the lung (CCAM) type 0. Design: Case report. Setting: Pediatric intensive care unit. Patient: A female neonate, appropriate for gestational age, with respiratory failure immediately after delivery. Interventions: Cardiopulmonary support with venoarterial extracorporeal membrane oxygenation. Results: There was no improvement of pulmonary function, and the patient died. CCAM type 0 was diagnosed postmortem. Conclusions: CCAM type 0 should be considered as a rare differential diagnosis of irreversible lung pathologies leading to failure of extracorporeal membrane oxygenation therapy for neonatal respiratory failure.

Das klinische Bild der fulminanten Sepsis des Kindes stellt gerade für die Erstversorgung eine besondere Herausforderung dar. Neuere Erkenntnisse beim Waterhouse-Friedrichsen-Syndrom, also der fulminanten Meningokokkensepsis (die v. a. Kinder und Jugendliche betrifft), haben gezeigt, dass besonders in diesen Fällen eine frühe und effiziente Intervention zu einer dramatischen Besserung der Mortalitätszahlen führt. Diese frühe Intervention ist nur bei einem geschärften Bewusstsein für die Wichtigkeit der frühen, möglichst schon außerklinischen Diagnosestellung dieses Krankheitsbildes möglich. Der sich ergebende Interventionsablauf ist dann stufenweise rasch und am klinischen Effekt orientiert umzusetzen und beinhaltet insbesondere die frühe aggressive Volumengabe.

La coartación aórtica del neonato puede asociar en un porcentaje importante hipoplasia del arco aórtico, llegando en algunas series al 60%.Cuando existe hipoplasia del arco aórtico distal el tratamiento estándar consiste en la resección de la zona de coartación y anastomosis termino-terminal extendida.En casos de hipoplasia severa del arco aórtico distal y arco distal largo, podría no ser suficiente con la resección y anastomosis termino-terminal extendida, por lo que sería razonable realizar alguna técnica adicional para ampliar el arco aórtico distal, evitando así un abordaje anterior, el uso de parada circulatoria con o sin perfusión cerebral selectiva y el aumento de la morbimortalidad perioperatoria.Presentamos los resultados de 4 neonatos, a los que se les realizó una ampliación del arco aórtico distal, según técnica de Amato (anastomosis latero-lateral entre las arterias carótida y subclavia izquierdas), para posteriormente resecar la zona de coartación y anastomosar la aorta descendente al arco aórtico previamente ampliado.En todos los casos el ecocardiograma postoperatorio mostró arco reconstruido con flujo laminar. No se ha presentado ningún caso de recoartación durante un período de seguimiento medio de 12 meses.Consideramos que la técnica de elección en la coartación con hipoplasia de arco distal es la resección y anastomosis termino-terminal extendida.En casos seleccionados, con arco aórtico distal muy largo y severamente hipoplásico, la técnica de Amato es una alternativa atractiva, con el objeto de evitar un abordaje anterior y el uso de CEC. Además, puede realizarse en un primer tiempo, manteniendo perfusión sistémica ductus-dependiente.Neonatal aortic coarctation can be combined with a significant percentage of aortic arch hypoplasia, reaching 60% in some series.When there is hypoplasia of the distal aortic arch, the standard treatment consists of resection of the coarctation zone and extended end-to-end anastomosis.In cases of severe distal aortic arch hypoplasia and a long distal arch, resection and extended end-to-end anastomosis would not be sufficient, making it reasonable to perform an additional technique to widen the distal aortic arch, thus avoiding an anterior approach and interrupting the blood circulation with or without selective cerebral infusion, with the resulting risk of an increase in perioperative morbidity and mortality.The results are presented on 4 neonates on whom a widening of the distal aortic arch was performed using the Amato technique (side-to-side anastomosis between the left carotid and subclavian arteries), in order to subsequently resect the coarctation zone and perform an anastomosis of the descending aorta to the previously widened aortic arch.The post-operative echocardiogram showed a reconstructed arch with laminar flow in all cases. There has been no recurrence of coarctation in any of the cases during a mean follow-up of 12 months.We believe that resection with extended end-to-end anastomosis is the technique of choice in coarctation with distal arch hypoplasia.The Amato technique is an attractive alternative in selected cases with a very long and severely hypoplastic distal arch, with the aim of avoiding an anterior approach and the use of extracorporeal circulation. This could also be performed initially, maintaining ductal-dependent systemic perfusion.

ZusammenfassungDas akute Atemnotsyndrom (ARDS) tritt jährlich bei 3–6/100 000 Kindern und Jugendlichen auf. Ursächlich sind verschiedenste direkte und indirekte Lungenschäden, wobei Pneumonien und Sepsis, gefolgt von Polytraumata und Ertrinkungsunfällen, die häufigsten sind. Zu 60% sind chronisch kranke Kinder betroffen, darunter 35% mit Störungen der Immunabwehr. Die Sterblichkeit beträgt ca. 25–30%, bei immundefizienten Patienten ca. 50%. Eine Störung der Immunität ist der wichtigste unabhängige Risikofaktor für die Entwicklung und das Überleben eines ARDS. Die dabei auftretende Hypoxämie ist Folge einer Schädigung der alveolokapillären Grenzmembran und des Kollapses der Alveolen bzw. der Ausbildung eines nichtkardiogenen Lungenödems. Durch effektive Beseitigung der auslösenden Ursache, Veränderungen der Beatmungstherapie mit niedrigen Atemzugvolumina und einem endexspiratorischen Druck (PEEP), der ausreicht, den Kollaps eröffneter Alveolarbezirke zu verhindern, und eine differenzierte Kreislauftherapie hat sich die Sterblichkeit durch Hypoxie bei ARDS drastisch vermindert. Aufwändige Therapien wie die Gabe von Surfactant oder die extrakorporale Membranoxygenierung (ECMO) sind selten gerechtfertigt.

We describe a fatal case of encephalitis that might be correlated with primary human metapneumovirus (HMPV) encephalitis. Postmortem HMPV RNA was detected in brain and lung tissue samples from the patient. Furthermore, HMPV RNA was found in culture fluids from cells coincubated with lung tissue.

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.

Der enterale Eiweißverlust (EEWV) kann als Begleitsymptom bei einer Vielzahl systemischer Erkrankungen und lokaler Erkrankungen des Gastrointestinaltraktes auftreten. Leitsymptom sind die Eiweißmangelödeme, gastrointestinale Symptome sind nicht obligat. Die Diagnose wird durch den Ausschluss anderer Proteinverlustquellen und den Nachweis erhöhter Alpha-1-Antitrypsin-Konzentrationen im Stuhl (> 320 mg/L) gesichert. In der Mehrzahl der Fälle ist die Klärung der Ursache über die Diagnose einer Grundkrankheit leicht möglich. Die diagnostische Aufarbeitung muss in unklaren Fällen zunächst nach dem Pathomechanismus (Zirkulationsstörung vs. Entzündung vs. Störung der Basalmembran) differenzieren. Davon ausgehend ist die gezielte Diagnostik der Grundkrankheit möglich. Ein gestuftes Vorgehen, orientiert an Invasivität und Kosteneffektivität wird vorgestellt. Die Lokalisationsdiagnostik erfordert bildgebende Verfahren (CT-Abdomen, Sonographie, Endoskopie und Szintigraphie). Bei umschriebener Verlustquelle muss eine intraoperative Enteroskopie erwogen werden. Kann der Proteinverlust nicht kausal behandelt werden, so wird eine Substitution von Albumin und Immunglobulinen in Abständen von 1-4 Wochen erforderlich. Die Prognose wird durch eine evtl. Grundkrankheit bestimmt und ist bei Proteinverlust als einzigem Symptom relativ gut.

IPEX (immune-dysregulation, polyendocrinopathy, enteropathy, X-linked) syndrome is an autoimmune disorder with an often lethal outcome in spite of immunosuppressive therapy. We report the successful use of sirolimus in 3 patients with IPEX. The efficacy of sirolimus is probably due to its different mode of action compared to calcineurin-dependent agents.

Objective: The objective of this study was to determine the epidemiology of acute respiratory distress syndrome (ARDS) in children and adolescents aged 1 mo to 18 yrs. Design: The authors conducted a population-based prospective multicenter survey from February 1 to 28,1997, June 1 to 30, 2001, and April 1 to 30, 2004. Setting: This study was conducted at 94 intensive care units (ICUs) in 1997, 92 ICUs in 2001 and 2004 in the district of Cologne, Germany with a population of 4.15 (1997), 4.28 (2001), and 4.35 million (2004), respectively. The survey was not confined to children's hospitals, but addressed all ICUs. Patients: This study consisted of children and adolescents aged 1 mo to 18 yrs. Term neonates and premature babies with a corrected age below 43 gestational weeks were excluded. ARDS was defined according to the consensus criteria (acute-onset pulmonary process of noncardiogenic origin, Pao2/Fio2 ratio <200, bilateral alveolar infiltration in chest radiograph). Results: All ICUs at pediatric hospitals and all but seven adult ICUs collaborated. Incidence of ARDS was assessed as the number of patients entering ARDS criteria within the three study periods divided by the total population in this age group. During the study period, 12 pediatric patients were diagnosed as having ARDS. In five cases, onset was before the study period, representing a calculated prevalence of 5.5 × 10 −5 [3.1 × 10−5; 9.6 × 10−5] cases per year and an incidence of 3. 2 × 10−5 [1.5 × 10−5; 6.6 × 10−5] cases per year in the age group under investigation. Conclusion: This is the first population-based evaluation of the incidence of ARDS in the pediatric age group. It shows that the incidence of ARDS in this age group is low. This makes randomized studies on pediatric ARDS aiming on the end point “outcome” nearly impossible.

Autoimmune enteric leiomyositis is an extraordinary rare cause of acquired chronic intestinal pseudo-obstruction in children. We report a 5-year-old girl who developed chronic intestinal pseudo-obstruction 3 years after an autoimmune hepatitis. Mucosal biopsies of the upper gastrointestinal tract and colon showed minimal inflammatory changes. On full-thickness biopsies of the small intestine, a dense lymphocytic infiltrate of the muscularis propria was seen, mainly consisting of cytotoxic T lymphocytes. Smooth muscle fibers were degenerated and diminished, but the myenteric plexus was intact. The coexistence of an autoimmune hepatitis in our case indicates an expansion of autoreactive T cells to homologous self-antigens. It is of practical importance for histopathological diagnosis that inflammation in autoimmune enteric leiomyositis affects the muscularis propria of the small intestine, whereas mucosa and submucosa do not show severe inflammatory changes. Therefore, correct diagnosis may be missed in peroral and peranal mucosal biopsies, but full-thickness biopsies are required.

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.

Introduction: Only four children with chronic Intestinal Pseudoobstruction (CIPO) due to myositis have been reported, all requiring TPN. Autoimmune hepatitis (AIH) was not associated. Clinical case report History: Admission of a 5,5 yr old girl with paralytic ileus. Medication with prednisone (alternating doses of 0.3 and 0.5mg/kg) and azathioprine (3mg/kg) because of AIH diagnosed at 20 month of age. Examination: Short stature (104 cm), abdominal distension, liver 3 cm below RCM.Abnormal laboratory findings: ESR 22mm/1hr, LDH 357 U/l, CRP 35mg/l, IgG 34g/l, IgM 2,2g/l, albumine 29g/l, SMA 1:160. Methods: Further immunologic studies No detectable further autoantibodies (ANA, ANCA, AMA, AMA-M2, LKM, parietal cells, reticuline fibers, enterocytes and entric nerves. HLA- class I: HLA: A2, A24(9); HLA-B: b51(5), B44(12) HLA-C CW4,-HLA-class II:DR B 1*07, DR B 1*15, DR B 4*, DR B 5*, DR B 1*02, DR B 1*06 Results: Histology of whole mount intestinal biopsies: Lymphoid infiltrations, predominating on the mucosa and on the muscularis of the bowel with disappearnace of the smooth muscle. The intraepithelial lymphocytes (CD3/CD8) enhanced in number, but also some follicules with lymphocytes (CD20). Well preserved myenteric plexus.Course: Remission of the intestinal pseudoobstruction with prednisone 2mg/kg and tacrolimus (trough levels of 6–10 ng/ml). Serum IgG levels declined to 15g/l. At 6.5 yrs. relaps of CIPO, after tapering prednisone from 5mg to alternating doses of 2,5 and 5mg. Abnormal serum levels for IgG (20g/l), IgM (2,35g/l), LDH (323 U/l), CRP (30mg/l).Within 10 days after increasing the prednisone dose to 2 mg/kg remission was achieved, which was maintained with prednisone 0,2mg/kg, tacrolimus and azathioprine (3mg/kg) for meanwhile 5 months. Conclusion: This is the first report of AIH and autoimmune intestinal leiomyositis. Intestinal findings resemble the cases of idiopathic intestinal myositic previously reported. In contrast to uncomplicated cases of AIH there was a persistent and marked elevation of IgG, which declined with effective immunosuppressive tretment of the myositis. The only abnormal serologic marker of myositis was a slight increase in LDH.

Journal of Pediatric Gastroenterology and NutritionVolume 39, Issue S1 p. S390-S390 ABSTRACTS: Poster Session Abstracts P0875 ATYPICAL AUTOIMMUNENTEROPATHY WITH UNCOMMON FOXP-3 MUTATION: SUCCESSFUL TREATMENT WITH SIROLIMUS L. Bindl, L. Bindl Childrens Hospital, RFWU, Bonn, GermanySearch for more papers by this authorF. Ruemmele, F. Ruemmele Gastroenterologie, Hopital Necker, Paris, FranceSearch for more papers by this authorL. Perroni, L. Perroni Genetica Humana, Ospedali Galliera, Genova, ItalySearch for more papers by this authorM. J. Lentze, M. J. Lentze Childrens Hospital, RFWU, Bonn, GermanySearch for more papers by this author L. Bindl, L. Bindl Childrens Hospital, RFWU, Bonn, GermanySearch for more papers by this authorF. Ruemmele, F. Ruemmele Gastroenterologie, Hopital Necker, Paris, FranceSearch for more papers by this authorL. Perroni, L. Perroni Genetica Humana, Ospedali Galliera, Genova, ItalySearch for more papers by this authorM. J. Lentze, M. J. Lentze Childrens Hospital, RFWU, Bonn, GermanySearch for more papers by this author First published: 01 June 2004 https://doi.org/10.1002/j.1536-4801.2004.tb13305.x Submitted by: [email protected] Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onEmailFacebookTwitterLinkedInRedditWechat No abstract is available for this article. Volume39, IssueS1June 2004Pages S390-S390 RelatedInformation