Synkopen nach Belastung

Vascular airway compression syndromes have to be differentiated from bronchial asthma. We report the case of an 11-year-old girl who had been treated for bronchial asthma since childhood and presented with recurrent syncope after exercise. A double aortic ring caused severe tracheal stenosis with reflex ventilation depression and CO2 retention documented by spiroergometry. After transsection of the right dorsal arch the symptoms resolved and flow-volume charts and gas exchange were normal.     

Epstein-Barr-Virus-Infektionen nach Stammzelltransplantation

EBV associated lymphoproliferative disease (PTLD) after stem cell transplantation (SCT) is linked with an often fulminate course and a high mortality. Main risk factors are EBV primary infection after transplantation,T cell depleted graft,T cell antibodies (ATG,ALG, OKT3) and unrelated or HLA-mismatched donors.Besides some limitations, EBV viral load has proven to be an effective tool in early diagnosis and monitoring of therapy. Therapeutic options are mainly based on monoclonal anti-CD20 antibodies (Rituximab) and the adoptive T cell transfer,which proved to be effective in prophylaxis as well as in therapy.Future perspectives are standardizing of EBV viral load measurement, establishing of immunological diagnostics as well as the prophylaxis of PTLD.     

Herzversagen nach Appendektomie

We report on a 5-year-old girl who developed heart failure after an initially uneventful clinical course following a noncardiac surgical procedure. Echocardiographic examination showed severe myocardial dysfunction of both ventricles. The patient died on the 3rd postoperative day in low output failure and electromechanical dissociation. At autopsy total absence of myocardial tissue of the right ventricular free wall with severe lipomatous transformation was found. This abnormality appears clinically as an "arrhythmogenic right ventricle" in most of the cases. Therefore, severe dysrhythmia or myocardial dysfunction may have been the leading factors in the development of congestive heart failure with a deleterious outcome.     

Krankheiten nach Nierentransplantation

Kidney transplantation is the therapy of choice in children with end-stage renal disease. There are myriad problems that can arise after renal transplantation, and they are individually very variable. Recurrence of the original underlying disease is of concern in focal segmental glomerulosclerosis, in other forms of glomerulonephritis, and in primary hyperoxaluria. The progression of extrarenal manifestations such as hepatic fibrosis with the development of portal hypertension is observed in autosomal recessive polycystic kidney disease. Liver involvement in nephronophthisis is also clinically significant. Long-term side effects of steroids can be stressful and demand that the medication be tapered off. Nephrotoxic side-effects of calcineurin inhibitors can require an individually tailored drug combination strategy. EBV-associated lymphoproliferation is a consequence of exaggerated immune suppression. In many patients creeping creatinine build-up is ongoing. The most important risk factors for life expectancy are cardiovascular complications of chronic renal insufficiency and of medicamentous treatment.     

Harnwegsinfektionen nach Nierentransplantation

Febrile urinary tract infections after renal transplantation (transplant pyelonephritis) are a relevant clinical problem. They cause significant morbidity and even urosepsis and are usually associated with acute graft dysfunction. Risk factors include baseline immunosuppression, anatomical and functional factors (vesicoureteric reflux into the kidney graft, bladder dysfunction), underlying diagnosis, urinary tract surgery before transplantation as well as gender (increased incidence in girls). Meticulous diagnosis and treatment are important to minimize compromise of long-term graft function. Future prospective studies are warranted to increase the knowledge of risk factors and acute and chronic graft dysfunction. This will ultimately lead to improved prophylactic and therapeutic measures and further optimization of long-term renal graft survival.     

Augensymptome nach Aortenisthmusstenosenresektion

Horner??s syndrome (miosis, ptosis, enophthalmus) is caused by an irritation or damage of the stellate ganglion. After correction of coarctation of the aorta followed by a left-sided chest tube, a toddler developed Horner??s syndrome on the third day after surgery. Chest tubes causing Horner??s syndrome are rarely described in the literature. In cases of sudden appearance the position of the chest tube should be examined immediately and corrected if necessary.     

Krankheiten nach Lebertransplantation

Liver transplantation represents a very successful curative therapy for end-stage liver disease in children and adolescents. Despite good overall outcome there are several surgical, medical and pharmacokinetic complications, which should be considered in the interdisciplinary challenge of long-term follow-up. This paper describes typical complications following liver transplantation with respect to different postoperative time periods with the aim to aid physicians in clinics and private practice in follow-up examinations of children following liver transplantation.     

Zur Masernprophylaxe nach Degkwitz

Ohne Zusammenfassung     

Exanthem nach abdomineller Schmerzsymptomatik

Ohne Zusammenfassung     

Komplikationen nach operierter Oesophagusatresie

Zusammenfassung Bei längerdauernder Nachbehandlung von 15 Kindern, die die Operation einer Oesophagusatresie unter 70 Neugeborenen mit dieser Mißbildung überlebten, konnten im wesentlichen 4 Arten von Komplikationen beobachtet werden, die sich oft langsam oder erst nach einiger Zeit bemerkbar machten. Am häufigsten sahen wir die Behinderung der Nahrungsaufnahme durch eine Striktur im Bereich der Anastomose, die durch eine Schrumpfung der Nahtstelle entsteht. Auch narbige Verziehungen des Oesophagus verursachen Trinkschwierigkeiten. Am gefährlichsten erwies sich die Nahtinsuffizienz, die vor allem dann droht, wenn die Anastomose unter Spannung steht, wenn also ein größerer Defekt überbrückt werden mußte. Es bilden sich Fisteln oder Pseudodivertikel zum Mediastinum oder zur Lunge aus mit nachfolgender Pleuritis und hartnäckiger Pneumonie; von 4 Kindern starben allein 2 an dieser Komplikation. Zum Schluß wird noch auf Passagehindernisse durch Fremdkörper hingewiesen. An Hand von Beispielen und Röntgenbildern werden diese Komplikationen näher erläutert.     

Benigne Myositis nach Influenzainfektion

The clinical course of a 5.5-year-old boy is presented; he suddenly complained of severe calf pain 11 days after the start of an airway infection accompanied by cough, cold, and fever. Due to the pain, he was no longer able to walk. Furthermore, the boy exhibited fever around 39°C despite the continuous administration of antipyretic drugs and antibiotics. Comprehensive diagnostics were performed (laboratory analyses, magnetic resonance imaging of the skeletal muscle, bone marrow puncture, x-ray, autoimmunological analyses, exclusion of muscle dystrophy, ultrasonic surveys, search for pathogenic agents via serology and nasopharyngeal secretions, electrocardiography, echocardiography, metabolic analyses). Finally, based on all clinical findings and laboratory results, the patient was diagnosed with benign acute childhood myositis. A connection with a previous influenza infection was likely, based on relevant laboratory findings (leucopenia, no increase in C-reactive protein, no evidence for further inflammation mediators) and positive influenza serology. The patient recovered completely despite the prolonged process. A new aspect of this form of myositis, first described in 1957 by Lundberg, was established in the form of a clearly increased number of circulating immune complexes with complementary factors C3 and C1. To the author’s knowledge, this has not been previously described.     

Reaktivierung nach einer Herpes-simplex-Enzephalitis

Background

In case of relapse after Herpes simplex encephalitis (HSE) a clear distinction between recurrent virus infection and autoimmune disease is frequently not possible and both pathophysiological mechanisms can occur simultaneously.

Case

We report on a 4 year old boy who was admitted in stupor 10 days after HSE.

Treatment and follow-up

Treatment with aciclovir, immunoglobulins, phenobarbital und tiapridex had no effect. The child developed severe neurological deficits. After 6 months of inpatient rehabilitation his motor status returned to normal while substantial impairment of speech and attention remained.

Conclusions

Early relapse after HSE occurs within 2–4 weeks after the initial disease. Clinical presentation includes various neurological symptoms in combination with fever and impairment of consciousness. We recommend CSF testing and MRI after completion of the initial antiviral therapy.