Nevoid Basal Cell Carcinoma Syndrome (NBCCS) is an autosomal-dominant disorder characterized by multiple basal cell carcinomas, jaw cysts, palmar/plantar pits, calcification of the falx cerebri, and spine and rib anomalies. The combination of clinical, imaging, and histological findings is helpful in identifying NBCCS patients. Imaging plays a crucial role in evaluation of these patients. We present a wide variety of clinical and radiological findings characteristic of this disease.

Midazolam/ketamine sedation has been used successfully in children undergoing painful invasive procedures. The authors prospectively assessed inter- and intra-individual variability in ketamine dosage for sedation in repetitive invasive procedures in children with malignancies. A total of 92 invasive procedures (58 lumbar punctures, 34 bone marrow biopsies; range: 2-9 procedures/patient) were performed on 25 children (median age: 12 years). Intravenous sedation consisted of 0.1 mg midazolam/kg and 0.5-1.0 mg ketamine/kg. Incremental dosages of ketamine (0.33 mg/kg) were given if necessary to achieve or maintain deep sedation. Primary outcome measure was the inter- and intraindividual ketamine dosage required to achieve adequate sedation; secondary outcome measures were the number of procedures with adequate sedation (Ramsay score of > 4), the number of adverse side effects, and the need for therapeutic interventions. All 92 invasive procedures were completed with satisfactory sedation levels in 88 procedures (95.7%). There was a great inter- and intraindividual variability in ketamine dosage required to achieve or maintain adequate sedation. In 12% of procedures side effects were seen, which required no or only minor interventions. Due to great inter- and intraindividual differences, ketamine dosage should be titrated toward the desired level of sedation. Thus, ketamine can be adjusted to the individual's need while achieving adequate sedation.

Background: Enteroviruse belongs to the family of picornaviruses. They can be devided in 4 groups: polioviruses (Typ 1-3) enteric cythopathogenic human orphan (ECHO)- viruses, unclassified enteroviruses 68-71 and coxsackie viruses. Coxsackie virus type B can cause pharyngitis, myalgia, myocarditis and meningitis as well as severe neonatal infections.Case report: We report a patient with coxsackie type B 4 encephalitis. A 7 year old boy had 2 episodes of mental dizziness and consciousness disturbance. Because of he presented a visus of 70 %, headaches and vomiting he was admitted to our hospital. In the cerebrospinal fluid a coxsackie virus type B 4 could be identified. With nonspecific therapy the boy recovered.Conclusion: We concluded that coxsackie virus B4 caused acute encephalitis with ophthalmological and mental symptoms and good prognosis. Coxsackie virus B4 should be considered in patients with encephalitis.

PURPOSE:In the Society of Pediatric Oncology (SIOP) 93-01 study, 30 patients older than 16 years were found to have Wilms' tumor. They were treated according to the pediatric protocol and were analyzed for clinical presentation, stage distribution, and prognosis.PATIENTS AND METHODS:Patient age ranged from 16 to 62 years (median, 25.4 years). Tumor stages were defined according to SIOP, and treatment was risk-adapted according to SIOP 93-01/Society for Pediatric Oncology and Hematology (GPOH) protocol. The patients were evaluated with regard to response, toxicity, and prognosis. Specimens of all tumors were centrally reviewed.RESULTS:Ten patients (33%) had metastatic disease at the time of diagnosis (liver, four patients; lung, three patients; liver and lung, three patients). The local stage distribution showed a predominance of higher stages (stage I, eight patients; stage IIN-, three patients; stage IIN+, four patients; stage III, 15 patients). Histologic studies revealed intermediate-risk in 23 of 30 tumors; two tumors were classified as high-risk; and three tumors were clear-cell sarcomas. Two of 30 patients showed a nephroblastoma and a renal cell carcinoma simultaneously in the same kidney. A complete remission was achieved in 24 patients; four patients relapsed after complete remission; and three of them reached a second remission with further treatment. Event-free survival was 57%, with an overall survival of 83% (median observation time, 4 years).CONCLUSION:Adults can be cured in a high percentage by a multimodal treatment according to pediatric protocols. Toxicity is higher than in children, but acceptable in view of the high remission rate.

Different pharmacological agents have been used for sedation in children undergoing invasive procedures. The authors prospectively evaluated the efficacy, the occurrence of adverse side effects, and cardiovascular parameters in midazolam and ketamine sedation for invasive procedures in children with malignancies and hematological disorders. A total of 183 invasive procedures were performed on 63 children (mean age 9.2 +/- 5.2 years). Intravenous sedation consisted of 0.1 mg midazolam/kg and 1.0 mg ketamine/kg. Incremental dosages of ketamine (0.33 mg/kg) were given if necessary to maintain deep sedation. Systolic and diastolic blood pressure, heart rate, and oxygen saturation were recorded. All 183 invasive procedures were successfully completed with satisfactory sedation levels in 170 procedures (92.9%; 95% CI:88.2-96.2%). In 33 procedures (18%; 95% CI: 12.8-24.4%) sedation was associated with side effects, the most common being oxygen desaturation. One patient experienced a transient episode of laryngospasm. There was a significant increase in both systolic and diastolic blood pressure and heart rate after ketamine medication (p <.01). Procedure and recovery time were correlated to ketamine dosage (p <.01). The combination of midazolam and ketamine is efficacious in achieving deep sedation for painful invasive procedures. Considering the possibility of potentially serious respiratory complications it should be performed only by physicians who are trained in advanced airway management and life support. As opposed to many other sedative drugs with cardio-depressant properties, ketamine causes a rise in both systolic and diastolic blood pressure, and heart rate.

Medical and Pediatric OncologyVolume 40, Issue 4 p. 266-267 Medulloblastoma with extensive nodularity in nevoid basal cell carcinoma syndrome Schahin Aliani MD, Corresponding Author Schahin Aliani MD [email protected] Department of Pediatrics, Faculty of Medicine, Homburg, GermanyUniversitätsklinik für Kinder-und, Jugendmedizin, Kirrbergerstr., 66421 Homburg, Germany.Search for more papers by this authorJuergen Brunner MD, Juergen Brunner MD Department of Pediatrics, Faculty of Medicine, Homburg, GermanySearch for more papers by this authorNorbert Graf MD, Norbert Graf MD Department of Pediatrics, Faculty of Medicine, Homburg, GermanySearch for more papers by this authorKatrin Altmeyer MD, Katrin Altmeyer MD Department of Radiology, Faculty of Medicine, Homburg, GermanySearch for more papers by this authorIsolde Niedermayer MD, Isolde Niedermayer MD Department of Neuropathology, Faculty of Medicine, Homburg, GermanySearch for more papers by this authorMartin Strowitzki MD, Martin Strowitzki MD Department of Neurosurgery, Faculty of Medicine, Homburg, GermanySearch for more papers by this author Schahin Aliani MD, Corresponding Author Schahin Aliani MD [email protected] Department of Pediatrics, Faculty of Medicine, Homburg, GermanyUniversitätsklinik für Kinder-und, Jugendmedizin, Kirrbergerstr., 66421 Homburg, Germany.Search for more papers by this authorJuergen Brunner MD, Juergen Brunner MD Department of Pediatrics, Faculty of Medicine, Homburg, GermanySearch for more papers by this authorNorbert Graf MD, Norbert Graf MD Department of Pediatrics, Faculty of Medicine, Homburg, GermanySearch for more papers by this authorKatrin Altmeyer MD, Katrin Altmeyer MD Department of Radiology, Faculty of Medicine, Homburg, GermanySearch for more papers by this authorIsolde Niedermayer MD, Isolde Niedermayer MD Department of Neuropathology, Faculty of Medicine, Homburg, GermanySearch for more papers by this authorMartin Strowitzki MD, Martin Strowitzki MD Department of Neurosurgery, Faculty of Medicine, Homburg, GermanySearch for more papers by this author First published: 10 February 2003 https://doi.org/10.1002/mpo.10163Citations: 6AboutPDF 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. REFERENCES 1 Gorlin RJ, Goltz RW. Multiple basal-cell epithelioma, jaw cysts and bifid rib: A syndrome. N Engl J Med 1960; 262: 908–912. 2 Kimonis VE, Goldstein AM, Pastakia B, et al. Clinical manifestations in 105 persons with nevoid basal cell carcinoma syndrome. Am J Med Genet 1997; 69: 299–308. 3 Evans DG, Farndon PA, Burnell LD, et al. The incidenceof Gorlin syndrome in 173 consecutive cases of medulloblastoma. Br J Cancer 1991; 64: 959–961. 4 Schofield D, West DC, Anthony DC, et al. Correlation ofloss of heterozygosity at chromosome 9q with histological subtype in medulloblastomas. Am J Pathol 1995; 146: 472–480. 5 Giangaspero F, Perilongo G, Fondelli MP, et al. Medulloblastom a with extensive nodularity: A variant with favorable prognosis. J Neurosurg 1999; 91: 971–977. 6 Johnson RL, Rothman AL, Xie J, et al. Human homolog of patched, a candidate gene for the basal cell nevus syndrome. Science 1996; 272: 1668–1671. Citing Literature Volume40, Issue4April 2003Pages 266-267 ReferencesRelatedInformation

Neue Behandlungsverfahren werfen ein neues Licht auf noch immer ungelöste Fragen zu seit langem bekannten Krankheiten. Die in diesem Heft von Helling et al. vorgestellte Arbeit “Die Lasertonsillotomie bei der Tonsillenhyperplasie des Kleinkindes” auf den Seiten 470–478 ist Anlass, erneut über die Frage nach den Auswirkungen der operativen Behandlung der Tonsilla palatina auf die Immunkompetenz des Heranwachsenden nachzudenken.

Solid-pseudopapillary tumor of the pancreas constitutes a very rare benign or low-grade malignant lesion occurring most commonly in young women and girls. It was First described by Frantz [1]. Local infiltration, distant metastasis and recurrence are very rare. Until today, the histogenetic origin of the tumor cell remains to be elucidated [4,6]. In 1996, solid-pseudopapillary tumor of the pancreas was introduced in the World Health Organization (WHO) classification of tumors of the exocrine pancreas [3]. Our case report - like a recently published work by Lange et al. [5] - intends to underline the significance of solid-pseudopapillary tumor in the differential diagnosis of a pancreatic mass.

Solid-pseudopapillary tumor of the pancreas constitutes a very rare benign or low-grade malignant lesion occurring most commonly in young women and girls. It was first described by Frantz [1]. Local infiltration, distant metastasis and recurrence are very rare. Until today, the histogenetic origin of the tumor cell remains to be elucidated [4,6]. In 1996, solid-pseudopapillary tumor of the pancreas was introduced in the World Health Organization (WHO) classification of tumors of the exocrine pancreas [3]. Our case report - like a recently published work by Lange et al. [5] - intends to underline the significance of solid-pseudopapillary tumor in the differential diagnosis of a pancreatic mass.

Ganglioneuroma constitutes a benign and surgically treatable tumor. The authors studied 4 patients with histopathologically proven ganglioneuroma focusing on radiological and metabolic features. The results confirm previous investigations that have shown metabolic activity in ganglioneuroma and characteristic patterns in imaging studies. Although for definite diagnosis tissue investigation is required, certain clinical and radiological features are suggestive of ganglioneuroma.

BACKGROUND:Cholestasis following bone marrow transplantation is not rare, but should always be a warning signal. The main causes of cholestasis following bone marrow transplantation are hepatotoxic effects of drugs, venoocclusive disease (VOD), early graft-versus-host disease (GvHD), total parenteral nutrition, viral hepatitis, sepsis and opportunistic infections. Vanishing bile duct syndrome represents a very rare cause for cholestatic symptoms.CASE REPORT:We report on a 8-year-old boy suffering from myelodysplastic syndrome, who underwent allogeneic bone marrow transplantation and developed biliary ductopenia in terms of a vanishing bile duct syndrome.CONCLUSION:Differential diagnosis for cholestasis following bone marrow transplantation should include the vanishing bile duct syndrome.

Zusammenfassung Bei der Meningeoangiomatose handelt es sich um eine seltene, gutartige, hamartomartige Läsion. Diese ist histopathologisch durch umschriebene transkortikale und leptomeningeale meningovaskuläre Proliferate mit fokalen Verkalkungen charakterisiert, wobei sich Varianten mit vorherrschenden zellulären oder vaskulären Merkmalen unterscheiden lassen. Die Meningeoangiomatose kann in Assoziation mit einer Neurofibromatose, am häufigsten mit der vom Typ 2, auftreten, sie wird aber zumeist als eigenständige Krankheitsentität beschrieben. Bei sporadischen Fällen manifestiert sie sich oft erstmalig durch einen fokalen Krampfanfall. Selten ist eine Meningeoangiomatose mit einem darüber liegenden Meningeom assoziiert. Diese Läsionen sind in der Regel gutartig und müssen von einem invasiven, anaplastischen Meningeom differenziert werden. Wir berichten über ein 4-jähriges Mädchen mit zellulärer Variante einer spontanen Meningeoangiomatose mit einem assoziierten Meningeom vom fibroblastischen Typ. Die fokal deutliche Immun-Positivität der Meningeoangiomatose mit einem Antikörper gegen CD34 war hilfreich zum Ausschluss eines invasiven Meningeoms.

We report two cases of inflammatory pseudotumors of the urinary bladder, one case of a chronic granulomatous pseudotumor (CGT) and one case of a pseudosarcomatous myofibroblastic (fibromyxoid) tumor (PMT). Both tumors resembled malignancies such as rhabdomyosarcomas regarding clinical appearance and imaging findings and represent rare urinary bladder tumors. The imaging findings on unenhanced and contrast-enhanced MRI as well as histological specimen are presented. Final diagnosis was made following elective surgery. Differential diagnosis of urinary bladder tumors as well as the imaging findings of these clinically comparable cases are discussed. Awareness of these benign lesions may prevent patients from inappropriate therapies such as chemotherapy or radiation therapy.

1 Current Knowledge about the Pathogen 1.1 Characteristics of Hepatitis A Virus The clinical features of hepatitis A have been known for a long time. However, the pathogen (hepatitis A virus (HAV)) was firstly identified in 1973 with the aid of immune electron microscopy [1]. HAV is classified in the family of Picornaviridae , genus Hepatovirus .

Background: High-dose chemotherapy according to recent protocols causes a high rate of bone marrow aplasia. Platelet transfusions in immunocompromised patients need a consequent leukocyte depletion to prevent infections, alloimmunization and immunomodulation. Leukocyte depletion can either be done as inline filtration or as bedside filtration. To prevent graft-versus-host disease platelet concentrates are generally irradiated before transfusion. Methods: From June 1999 to May 2000 we administered 235 platelet concentrates in 38 pediatric patients with malignancies. The number of transfusions per patient varied from 1 to 28. In all patients the absolute platelet count was determined before and after transfusion. 118 concentrates were bedside-filtered, in 117 concentrates inline filtration was done. All concentrates were irradiated with 30 Gy before transfusion. In 116 platelet concentrates we detected the concentration before transfusion and calculated the corrected count increment (CCI). Half of these concentrates was bedside-filtered, the other half was inline-filtered. Results: The mean platelet count of the patients before transfusion was 13,500/mul and that after transfusion 52,000/mul. In the group treated with bedside-filtered concentrates the platelet count increased to 36,000/mul, and in the group treated with inline-filtered concentrates it rose to 68,000/mul. The difference is statistically significant (p < 0.0001). By detecting the platelet count in the concentrate directly after production and again after bedside filtration, a significant platelet loss could be shown. Conclusion: Inline-filtered platelet concentrates should be preferred for the transfusion in thrombocytopenic patients.