Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0027819 (neuroblastoma)
 27,800 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Unexpected sudden cardiac death among children with a history of Kawasaki disease has come to be reported in Japan. Death occurred between 2 months and 8 years after complete recovery from Kawasaki disease according to our study material. To study the lesions of Kawasaki disease sequelae we examined 61 cases of Kawasaki disease which came to autopsy. In 17 of these the deaths apparently to be due to sequelae of this disease, were characterized by cardiac insufficiency caused by ancient coronary aneurysm with organized thrombotic occlusion and superimposed acute ischemic myocardial degeneration and/or necrosis. The age of the lesions appears to correlate with the interval period between complete recovery from this disease and death. Six cases succumbed incidentally of other causes: one traffic accident, one hemophilus meningitis, one chronic myeloid leukemia, one neuroblastoma, one meningeal hemorrhage due to rupture of basilar arterial aneurysm, and one acute lymphatic leukemia. Even in these cases, definite sequelae of arteritis were detected. A surgically resected coronary aneurysm and a ventricular aneurysm were also examined. It was disclosed by mass physical check-up of school children that 0.1% had a history of Kawasaki disease, among which 5-6% showed cardiac and/or coronary abnormality including aneurysms. The high incidence of cardiac involvement in Kawasaki disease has been proved in this study and this kind of lesion has the possibility of resulting in unexpected cardiac death of children.
 ...
PMID:Pathological study of sequelae of Kawasaki disease (MCLS). With special reference to the heart and coronary arterial lesions. 379 88

Neuroblastoma is the third most common pediatric cancer and can mimic many other diseases. However, clinicians need to be aware about the possibility of neuroblastoma, when other more obvious clinical conditions such as the Kawasaki disease (KD) are present. KD is an acute febrile vasculitis of childhood with coronary complications. There were a few reports for autopsy of KD patients with neuroblastoma. A 2-y-old girl was admitted for definite KD with five clinical signs. Her fever did not respond to retreatment with intravenous high dose immunoglobulin. Later a chest computerized tomography scan revealed a mass lesion in the right lower lung field, and ultimately diagnosed as neuroblastoma on the paravertebral area of thoracic spine levels T4 to T10. The second patient was a 3-y-old boy who was diagnosed as incomplete KD with left coronary ectasia. His fever also did not respond to retreatment with immunoglobulin, and the abdominal computerized tomography scan with biopsy confirmed neuroblastoma of the right adrenal gland. We report two patients with KD who were additionally diagnosed as having neuroblastoma.
 ...
PMID:Two cases of Kawasaki disease with hidden neuroblastoma. 2325 Jul 63

Kawasaki disease (KD) is a systemic vasculitis of unknown etiology and a leading cause of acquired heart disease. It is assumed that there is an activation of the immune system by an infectious trigger in a genetically susceptible host. Neuroblastoma is the most common extracranial solid tumor in young children. It mainly originates from primordial neural crest cells that generate the adrenal medulla and sympathetic ganglia. A diagnosis of concurrent KD and neuroblastoma in a living child has been made in only one previous report. We report the second case and review the literature.
 ...
PMID:Concurrent adrenal neuroblastoma and kawasaki disease: a report of a rare case. 2347 67

The approach to the child with ataxia requires a detailed history and careful general and neurological examination as well as selected blood work and brain imaging and increasingly available genetic testing for inherited ataxias that usually have an episodic or progressive presentation. The differential of acute and recurring ataxia covered in this chapter includes intoxication (e.g., antiepileptics, lead, alcohol), postinfectious cerebellitis, hemorrhage, ischemic stroke, tumor (posterior fossa or cerebellum), brainstem encephalitis, occult neuroblastoma, Miller Fisher syndrome, conversion reaction, multiple sclerosis, epileptic pseudoataxia, vasculitis (e.g., Kawasaki), metabolic etiologies (e.g., maple syrup urine disease, pyruvate dehydrogenase deficiency, ornithine transcarbamylase deficiency, biotinidase deficiency, Hartnup disease, and argininosuccinic aciduria), migraine, migraine equivalents (benign paroxysmal positional vertigo), autosomal dominant episodic ataxias (with seven types currently identified), and hypothyroidism. Cooperation with therapists and providers from other specialties including ophthalmology and genetics and metabolism is essential to caring for these children and their families.
 ...
PMID:Ataxia. 2362 31

Langerhans cell histiocytosis (LCH) is a rare proliferative disease accompanied by the accumulation of pathological Langerhans cells, which often spreads into multi-site and multi-organ systems. We here describe a girl with a history of Kawasaki disease and cervical lymphadenopathy who presented with occipital LCH. Adrenal tumor was detected on staging evaluation of LCH and was diagnosed as neuroblastoma on resection using laparoscopic surgery. Neither tumor relapsed following chemotherapy for LCH and resection of neuroblastoma. Although LCH often spreads into multi-organ lesions, invasive biopsy may be needed for tumors with atypical localization for LCH in consideration of the synchronous occurrence of malignancies.
...
PMID:Coexistence of neuroblastoma detected on staging of Langerhans cell histiocytosis. 2525 48