6 results on '"Ralf Knöfler"'
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2. Akute zentralnervöse Symptomatik bei einem Kind mit Down Syndrom und akuter Leukämie – An was muss man denken?
- Author
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Gabriele Hahn, Björn Sönke Lange, Nora Benecke, Meinolf Suttorp, and Ralf Knöfler
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Pediatrics, Perinatology and Child Health - Published
- 2017
- Full Text
- View/download PDF
Catalog
3. Duodenocolonic Fistula As A Rare Complication of Intestinal Burkitt Lymphoma in a Three-Year-Old Boy
- Author
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Meinolf Suttorp, Ralf Knöfler, Björn Sönke Lange, Guido Fitze, Wilhelm Woessmann, Martin W. Laass, Katrin Schuchardt, and Kenichi Vinzenz Okuda
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Diarrhea ,Male ,medicine.medical_specialty ,Ileus ,Colon ,medicine.medical_treatment ,Fistula ,Biopsy ,03 medical and health sciences ,0302 clinical medicine ,Antineoplastic Combined Chemotherapy Protocols ,Gastroscopy ,medicine ,Intestinal Fistula ,Humans ,Stage (cooking) ,Duodenoscopy ,Neoplasm Staging ,Ultrasonography ,Chemotherapy ,business.industry ,medicine.disease ,Burkitt Lymphoma ,Magnetic Resonance Imaging ,Lymphoma ,Surgery ,Treatment Outcome ,030220 oncology & carcinogenesis ,Child, Preschool ,Pediatrics, Perinatology and Child Health ,030211 gastroenterology & hepatology ,Segmental resection ,medicine.symptom ,Complication ,business - Abstract
Burkitt lymphoma (BL) in children often presents with abdominal localization. Intestinal perforations have been described mainly during treatment. We report on a three-year-old patient with abdominal BL who was diagnosed with a duodenocolonic fistula.A three-year-old boy presented with diarrhea, crampy abdominal pain, and a four-week history of loss of appetite and weight. Ultrasound and MRI detected a colonic tumor forming a duodenocolonic fistula which was verified by gastroduodenoscopy. A surgical biopsy revealed BL. The stage III BL with low LDH was treated with four courses of BFM-type short-pulse chemotherapy. After two courses of chemotherapy the patient developed a mechanic ileus. A segmental resection of a short segment of the colon at the right flexure carrying the residual tumor mass with cicatricial stenosis and fistula followed by colonic end to end anastomosis and covering of the fistula by omentum major were carried out without complication. 15 days after surgery, two additional courses of chemotherapy could be administrated and the boy is in ongoing remission and free of any symptoms with a follow-up interval of 18 months.Duodeonocolonic fistula at presentation in a child with abdominal BL is extremely rare. Delayed surgery after size of the tumor bulk has been reduced by chemotherapy might represent a risk adapted approach. However, due to limited experience with duodenocolonic fistulas even in larger pediatric lymphoma trials any decision has to be based on the problems to be faced in individual cases.Ein Burkitt-Lymphom (BL) im Kindesalter ist oft im Abdomen lokalisiert. Intestinale Perforationen wurden in der Literatur zumeist als während der Chemotherapie auftretende Komplikation beschrieben. Wir berichten über einen Patienten mit abdominellen BL, welcher bei Diagnosestellung eine duodenokolische Fistel aufwies.Ein 3jähriger Junge wurde vorgestellt mit rezidivierenden Diarrhoen, krampfartigen Bauchschmerzen, Appetitlosigkeit und Gewichtsverlust über einen 4wöchigen Zeitraum. Die radiologische Diagnostik mittels Abdomensonographie und MRT ergab das Bild eines Tumors, welcher eine duodenokolonische Fistel ausgebildet hatte, was mittels Ösophagogastroduodenoskopie bestätigt wurde. Die chirurgische Tumorbiopsie sicherte den Befund eines BL. Es handelte sich um das Stadium III mit einer leichten LDH-Erhöhung. Nach 2 Blöcken der Chemotherapie entwickelte der Patient einen mechanischen Ileus. Eine operative kurzstreckige Segementresektion des stenosierenden und fistelbildenden Tumors im Bereich des Colons ascendens wurde mit einer End-zu-End-Anastomose und Abdecken der Fistel durch Omentum majum ohne Komplikationen durchgeführt. Am 15. postoperativen Tag konnten anschließend 2 weitere Chemotherapie-Blöcke verabreicht werden. Der Patient befindet sich 16 Monaten später beschwerdefrei in Remission.Eine duodenokolonische Fistel als Komplikation eines abdominellen BL ist eine extreme Rarität. Die verzögerte Resektion nach Tumorverkleinerung durch Chemotherapie stellt möglicherweise eine risiko-adaptierte Vorgehensweise dar. Jedoch sollte jede Entscheidung zur Vorgehensweise bei duodenokolonischen Fisteln aufgrund der fehlenden Erfahrungen im Kindesalter auch innerhalb großer Lymphomstudien immer individuell angepasst sein. more...
- Published
- 2018
4. Acute Cerebral Symptoms in a Child with Down Syndrome and Acute Leukemia - What has to be Considered?
- Author
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Nora, Benecke, Björn Sönke, Lange, Meinolf, Suttorp, Gabriele, Hahn, and Ralf, Knöfler
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Leukemia, Myeloid, Acute ,Acute Disease ,Humans ,Infant ,Down Syndrome ,Child - Published
- 2017
5. Inherited Disorders of Platelet Function in Pediatric Clinical Practice: A Diagnostic Challenge
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Werner Streif, Ralf Knöfler, and W. Eberl
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Male ,Pediatrics ,medicine.medical_specialty ,Platelet Aggregation ,Platelet Function Tests ,Platelet disorder ,Hemorrhagic Disorders ,Diagnosis, Differential ,Thrombasthenia ,medicine ,Von Willebrand disease ,Humans ,Medical history ,Platelet ,Cooperative Behavior ,Child ,Purpura ,Mucous Membrane ,business.industry ,Mucous membrane ,Perioperative ,medicine.disease ,Clinical Practice ,Epistaxis ,medicine.anatomical_structure ,Pediatrics, Perinatology and Child Health ,Immunology ,Female ,Interdisciplinary Communication ,Blood Platelet Disorders ,business - Abstract
Hereditary disorders of platelet function are a heterogeneous group of diseases that are often complex and tend to go undetected until clinically relevant bleeding occurs. Hallmarks are epistaxis, easy bruising, mucous membrane bleeding, perioperative bleeding and menorrhagia. Bleeding may be intermittent and unpredictable. After decades of successful research on platelet biology and genetics, research findings have not been satisfactorily translated to clinical practice. The lack of robust and well- standardized test systems continues to make the diagnosis of platelet defects cumbersome for the practising clinician. Patient history and description of clinical bleeding symptoms are essential. Exclusion of von Willebrand disease, platelet count and investigation of blood smears may provide a tentative diagnosis. Light transmission aggregometry is still considered the gold standard for assessing platelet function. Due to the wide range of possible genetic defects molecular biological analyses can complement but do not substitute for other tests. The true incidence of inherited disorders of platelet function is unknown. A survey in Germany revealed that receptor-defects including Glanzmann's thrombasthenia and Bernard-Soulier syndrome and aspirin-like defects were the most frequently diagnosed platelet disorders. Of affected children 60% presented with mild and 40% with moderate to severe bleeding tendency. Epistaxis, cutaneous and mucous membrane bleeding were the most frequent symptoms. The paediatric competence network of the GTH e.V. comprises 44 collaborating centres that are caregivers to over 150 children with well-defined inherited platelet defects. A major goal of this network is to promote diagnosis of children with inherited disorders of platelet function. more...
- Published
- 2010
- Full Text
- View/download PDF
6. Evaluation of desmopressin effect on primary haemostasis in pediatric patients with aspirin-like defect as hereditary thrombocytopathy
- Author
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A. Gneuß, Ralf Knöfler, J. T. Tauer, Judith Lohse, and T. Jürgens
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Blood Platelets ,Male ,medicine.medical_specialty ,Adolescent ,Platelet Aggregation ,Platelet Function Tests ,urologic and male genital diseases ,Hemostatics ,chemistry.chemical_compound ,Thrombin ,Adenosine Triphosphate ,hemic and lymphatic diseases ,Internal medicine ,von Willebrand Factor ,medicine ,Humans ,Platelet ,Deamino Arginine Vasopressin ,Thrombocytopathies ,Desmopressin ,Ristocetin ,Child ,Infusions, Intravenous ,Whole blood ,Aspirin ,Hemostasis ,business.industry ,Primary haemostasis ,Adenosine Diphosphate ,Endocrinology ,chemistry ,Child, Preschool ,Pediatrics, Perinatology and Child Health ,Female ,Blood Platelet Disorders ,business ,hormones, hormone substitutes, and hormone antagonists ,medicine.drug - Abstract
OBJECTIVES Despite about 3 decades of clinical experience with the therapy of inherited thrombocytopathies (HTP) with desmopressin (DDAVP) the mechanisms of haemostatic effects of DDAVP in these diseases remain unclear. Therefore platelet function diagnostics was carried out in whole blood (WB) from children with aspirin-like defect as one of the clinically mild forms of HTP after DDAVP administration. DESIGN AND METHODS 11 children (age range: 3-16 years) were treated with DDAVP i.v. (0.3 μg/kg as short infusion). Before, after 120, and 240 min of DDAVP administration the following parameters were measured: platelet aggregation (PA) and ATP release induced by ADP, collagen, ristocetin and thrombin; PFA-100 closure times (CT), factor VIII activity (FVIII:C), Von Willebrand factor antigen (VWF:Ag), collagen binding activity (VWF:CB) and blood count. RESULTS PA, ATP release and blood count were not influenced by DDAVP administration. PFA-100 CTs were markedly reduced at 120 and 240 min after DDAVP, respectively. FVIII:C, VWF:Ag and VWF:CB were increased after 120 min. CONCLUSION The DDAVP-induced improvement of primary haemostasis in patients with aspirin-like defect is mainly due to the marked increase of the VWF. For the evaluation of the clinical effect of DDAVP administration in patients with aspirin-like defect the investigation of a larger group of patients is needed. more...
- Published
- 2011
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