Lutz Bindl

High Plasma Levels of Phytosterols in Patients on Parenteral Nutrition: A Marker of Liver Dysfunction

Parenteral nutrition–associated cholestatic liver disease (PNAC) is considered to be multifactorial in origin, and its incidence has decreased during the past years (1). The pathogenesis of PNAC, however, remains poorly understood.Commercially available intravenous fat emulsions are soy-based preparations containing the phytosterols (PS) sitosterol, campesterol, and sigmasterol at a total concentration of approximately 1000 mol/L (40 mg/dL). The observation of high plasma levels of PS in patients with PNAC, receiving intravenous fat emulsions, led to the hypothesis that accumulation of these substances contributes to cholestasis (2–4). An animal model, disclosing decreased bile flow induced by infusion of PS (3), supported the assumption. In this study, we monitored the sitosterol plasma concentrations in a heterogeneous group of patients with liver dysfunction during parenteral nutrition (PN). Plasma concentrations of sitosterol were measured, using accurate gas-liquid chromatography-mass spectrometry techniques, as previously described (5). In total, we analyzed five patients on PN. Three of the five patients developed steatosis with non-icteric hepatic dysfunction. Their course improved with reduced glucose infusionrates while the dosage of lipid emulsions remained unchanged. Measurements of plasma sitosterol were continued in two patients with severe PNAC after stopping or reducing fat infusion.

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 ofHelicobacter 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 pyloripositive (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.

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.

Pulmonary Atresia with Intact Ventricular Septum and Right Ventricle—Coronary Artery Fistulae: Selection of Patients for Surgery

The anatomy of the coronary arteries and fistulae, filling pattern and evolution, right ventricular (RV) anatomy (as demonstrated by cineangiocardiography), hemodynamics, and various surgical procedures were evaluated with respect to their effect on coronary artery blood supply to provide guidelines for surgical management and candidate selection.

Cisapride reduces postoperative gastrocaecal transit time after cardiac surgery in children

ObjectiveTo investigate the influence of the prokinetic drug cisapride on gastrocaecal transit time (GCTT) in children after open heart surgery.DesignProspective, randomized and controlled study.SettingInterdisciplinary paediatric intensive care unit in a tertiary-care childrens hospital.PatientTwenty-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.InterventionsTen children were randomized to receive cisapride 0.2 mg/kg body weight, 30 min prior to measurement of GCTT.Measurements and resultsGCTT 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 noncisapride group achieved gastrocaecal transit. No adverse side-effects were observed.ConclusionsCisapride 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.

ARDS im Kindesalter Ergebnisse einer Umfrage in deutschen Kinderkliniken und gemeinsame Empfehlungen der „Arbeitsgemeinschaft ARDS im Kindesalter“ zur Beatmungstherapie

Zusammenfassung Das Acute respiratory distress syndrome (ARDS) ist auch in der pädiatrischen Intensivmedizin angesichts der hohen Letalität eine therapeutische Herausforderung. 1992 haben sich deshalb etwa 50 deutsche Kinderkliniken in einer informellen Arbeitsgemeinschaft zusammengeschlossen, deren Ziel die Erarbeitung gemeinsamer therapeutischer Strategien ist. Um Daten über Epidemiologie, Risikofaktoren und Verlauf zu gewinnen, führten wir 1993 anhand eines standardisierten Fragebogens für die Jahre von 1991–1993 eine prospektive und retrospektive Erhebung in deutschen Kinderkliniken durch. 112 Kinder wurden gemeldet. Die Inzidenz entsprach 7 Fällen auf 1 000 pädiatrische Intensivpatienten. Die Hälfte der Kinder war jünger als 2 Jahre alt. In 43 % der Fälle entwickelte sich das ARDS aus einer pulmonalen, in 39 % aus einer systemischen Grundkrankheit, 18 % ließen sich nicht in diese Kategorien klassifizieren. Die Letalität lag bei 46 % ohne Zusammenhang mit Alter, Geschlecht oder auslösender Ursache. Die Anzahl der begleitenden Organversagen hatte dagegen einen signifikanten Einfluß auf die Prognose. Die Übersicht über die Behandlungsmaßnahmen zeigte, daß einheitliche therapeutische Strategien derzeit nicht existieren und andererseits eine Reihe bisher ungeprüfter Therapieverfahren eingesetzt wurde. Die in dieser Untersuchung gewonnenen Informationen stellen eine wichtige Grundlage für die Planung prospektiver multizentrischer Studien beim ARDS im Kindesalter dar. Die vorgestellten Empfehlungen zur Beatmungsgtherapie wurden unter diesem Gesichtspunkt erstellt.Summary Acute respiratory distress syndrome (ARDS) is a therapeutic challenge in pediatric intensive care in view of its high mortality. In 1992, about 50 German pediatric intensive care unit doctors founded a working group with the aim of collaborating in clinical research. The objectives of both a prospective and retrospective survey conducted in German pediatric intensive care units in 1993 was to accumulate data on the epidemiology, risk factors and natural history in a large group of pediatric ARDS patients. 112 patients were reported, an incidence of 7 cases per 1 000 admissions to pediatric intensive care units. Half of the children were less than 2 years old. In 43 % of the cases, ARDS was associated with a pulmonary, in 39 % with a underlying systemic disease. Mortality was 46 % and independent of age, sex and triggering event. The number of associated organ failures, however, strongly influenced mortality. The analysis of treatment modalities employed in the patients revealed a lack of uniform therapeutic strategies. On the other hand, the patients were exposed to interventions not yet supported by controlled trials. The data gathered in this survey provide the basis for the design of prospective multicenter studies urgently needed to evaluate innovative treatment modalities in pediatric ARDS. Recommendations on ventilatory management and patient monitoring are included for this purpose.

Bakterielle Dünndarmüberwucherung Klinik, Diagnose und Therapie

ZusammenfassungBei 3 Patienten im Alter von 3 Monaten bis 5 Jahren, die zur Abklärung einer Gedeihstörung und rezidivierender Durchfälle vorgestellt wurden, konnte mittels einer Kombination von typischer Anamnese, laborchemischer, radiologischer, endoskopischer und histologischer Methoden und des H2-Atemtests die Diagnose einer bakteriellen Dünndarmüberwucherung gestellt werden. Von besonderer Bedeutung ist, daß bei allen Patienten eine Darmerkrankung vorbestand: Zwei Patienten, ehemalige Frühgeborene, waren im Rahmen einer nekrotisierenden Enterokolitis mehrfach laparotomiert worden, es bestand die Notwendigkeit zu teilweise ausgedehnten Darmresektionen. Beim 3. Patienten wurde ein langstreckiger Morbus Hirschsprung gesichert. Eine antibiotische Therapie mit Metronidazol führte in allen 3 Fällen zu einer Gewichtszunahme und Besserung der klinischen Beschwerden. Diskussion: Die bakterielle Dünndarmüberwucherung ist eine wichtige Differentialdiagnose der Gedeihstörung, insbesondere bei vorbestehender Darmerkrankung. Die Diagnose ergibt sich aus der Kombination verschiedener indirekter Untersuchungsverfahren. Ein direkter mikrobiologischer Nachweis ist schwierig. Eine antibiotische Therapie führt in der Regel zur raschen Gewichtszunahme.SummaryEvaluation of three patients, aged between three months and five years because of failure to thrive and persistent diarrhea revealed the diagnosis of small bowel bacterial overgrowth. A combination of a typical history, endoscopy, laboratory, radiological and histological methods as well as the breath hydrogen test were used. All patients had preexisting intestinal disease: two (former premature infants) had been operated several times because of necrotizing enterocolitis including resection of larger parts of small bowel. The third patient was suffering from pancolonic Hirschsprungs disease. All patients gained weight and reported less complaints after antibiotic treatment with metronidazole. Discussion: Small bowel bacterial overgrowth has to be considered in the differential diagnosis of failure to thrive, especially in cases with preexisting intestinal disease. The diagnosis may be substantiated by a combination of indirect methods. Direct bacteriologic analysis is difficult. Usually, antibiotic treatment leads to a good weight gain after a short period of time.