No abstract available.
Melanoma*

No abstract available.
Heart Diseases* ; Heart*

Ganglioneruroblastoma and neuroblastoma are among commonest types of childhood malignancy and a number of unique paraneoplastic syndromes have associated with both localized and disseminated neuroblastoma. The coincidence of neuroblastoma and myoclonic encephalopathy or other paraneoplastic syndromes occurs relatively rare, and therefore, failure to recognize this association could result in delays in both diagnosis and treatment, and the result could prove to be unfortunately fatal. The mechanism which underlies the remote damaging effect of neural crest tumor, especially neuroblastoma, on the nervous system resulting in myoclonic encephalopathy is by no means clear. In addition the nature and the extent of the pathologic lesion are inconsistent. We experienced a case of myoclonic encephalopathy associated with an occult mediastinal ganglioneuroblastoma in a 22-month-old girl who was hospitalized for inability to walk without support and tilting of the head to the left side. She became increasingly ataxic, and during the hospitalization myoclonic jerks of upper extremities and head along with chaotic, rapidly flickering, multidirectional spontaneous eye movements, were noted. Laboratory data included normal complete blood count, urinalysis, BUN and creatinine, electrolytes and bone marrow. Chest X-ray and chest CT revealed a relatively well marginated right posterior mediastinal mass. In a 24 hours urine excretion test, VMA and catecholamines were increased. Over the next 2 weeks, a surgical exploration revealed a right posterior mediastinal mass. Microscopically the mass proved to be a ganglioneuroblastoma, extending to right innominate artery and right axillary lymph nodes. Within 2 weeks after the surgery, radiotherapy (2,400 rads) and chemotherapy (CTX, DTIC, VCR) were started, but corticosteroid was not used. She has been free of tumor and abnormal neurological systemic symptoms and signs for 1 1/2 year since the completion of chemotherapy. In the 3 1/2 years follow-up period, her neurologic symptoms has completely resolved by the completion of 2 years chemotherapy. We report a case of mycoclonic encephalopathy associated with hidden ganglioneuroblastoma in 22-month-old girl.
Blood Cell Count ; Bone Marrow ; Brachiocephalic Trunk ; Catecholamines ; Creatinine ; Dacarbazine ; Diagnosis ; Drug Therapy ; Electrolytes ; Epilepsies, Myoclonic* ; Eye Movements ; Female ; Follow-Up Studies ; Ganglioneuroblastoma* ; Head ; Hospitalization ; Humans ; Infant ; Lymph Nodes ; Myoclonus ; Nervous System ; Neural Crest ; Neuroblastoma ; Neurologic Manifestations ; Paraneoplastic Syndromes* ; Radiotherapy ; Thorax ; Tomography, X-Ray Computed ; Upper Extremity ; Urinalysis

No abstract available.
Echocardiography* ; Echocardiography, Doppler, Pulsed ; Heart Diseases* ; Heart*

No abstract available.
Arteries* ; Cardiomyopathy, Hypertrophic* ; Humans ; Infant* ; Mothers*

No abstract available.

Pulmonary Fibrosis*

Extranodal marginal zone B-cell lymphoma of mucosa associated lymphoid tissue (MALT lymphoma) is a low-grade B-cell lymphoma that presents with an indolent clinicopathologic nature. Although this tumor can occur in various sites, including the gastrointestinal, skin, salivary gland and ocular adnexa, radiation therapy shows high local control in that disease. We report a case of recurrent MALT lymphoma presenting a huge cheek mass after radiotherapy on ocular adnexal lymphomas. An 65-year-old man had a history of treatments on MALT lymphomas of ocular adnexa came again with a huge cheek mass, not recurred and not originated from salivary gland, skin, other related mucosa tissues. He got excisional biopsy and was confirmed immunohistochemically as MALT lymphoma. After diagnosis, he was sent urgently to a radiotherapist for further treatment. He responded well to added radiotherapy with 36 Gy dose and has remained well at 6 months after his initial presentation.
Aged ; Biopsy ; Cheek ; Head and Neck Neoplasms ; Humans ; Lymphoid Tissue ; Lymphoma ; Lymphoma, B-Cell ; Lymphoma, B-Cell, Marginal Zone ; Mucous Membrane ; Porphyrins ; Salivary Glands ; Skin

STUDY DESIGN: A case of brain infarction due to occlusion of vertebral artery after cervical spine fracture is reported. OBJECTIVES: Documentation of possibility and clinical significances of brain infarction as one of grave complications after cervical spine injury. SUMMARY OF LITERATURE REVIEW: Occlusion of vertebral artery and consequent brain infarction can be associated with cervical spine injuries because vertebral arteries course through the transverse foramina from sixth to second corvical vertebrae. Infarction of vertebrobasilar system may cause impairment of cerebral, cerebellar, or brain stem function and can occasionally bring grave functional loss, even death. MATERIALS AND METHODS: A case of occlusion of vertebral artery and consequent cerebral infarction after cervical spine fracture in a 66 year-old man. Brain CT and angiogram were performed. He was managed with anticoagulants. RESULTS: Neurologic deficits from brain infarction disappeared after 2 weeks. CONCLUSIONS: Attention to the possibility of these complications and awareness of their clinical features seem to be mandatory in managing cervical spine injury patients.
Aged ; Anticoagulants ; Brain ; Brain Infarction ; Brain Stem ; Cerebral Infarction* ; Humans ; Infarction ; Neurologic Manifestations ; Spine* ; Vertebral Artery*

Immunoglobulin A (IgA) nephropathy in patients with the clinical findings of rapidly progressive glomerulonephritis usually demonstrate crescentic glomerulonephritis (CrGN). It is characterized by mesangial immune complex deposits containing IgA and is rarely associated with ANCA. The following case report illustrates a CrGN with mesangial & capillary wall IgA deposits by immunofluorescence (IF) and mesangial & endocapillary electron dense deposits by electron microscopy (EM) in patient with positive ANCA serology. A 54-year-old male visited due to the discomfort of right flank. Blood pressure was 150/90 mmHg. BUN and serum Creatinine was 40 mg/dL, 4.4 mg/dL respectively. Urinalysis revealed protein 2+, >30 RBC's/ HPF (dysmorphic), 24hr urine protein 1,612 mg/day and creatinine clearance 19 mL/min/1.73m2. Serological P-ANCA was positive by indirect immunofluorescence & ELISA. The histologic findings showed global scleorsis and fibrocelluar crescent without hypercellularity on LM, mesangial IgA deposition on IF, mesangial and subendothelial electrone dense material depositions on EM. After treatment (Methylprednisolon 500 mg/day for 3 days and then prednisolone 60 mg/day), Serum creatinin level, proteinuria and ANCA titer were decreased.
Antibodies ; Antibodies, Antineutrophil Cytoplasmic* ; Antigen-Antibody Complex ; Blood Pressure ; Capillaries ; Creatinine ; Enzyme-Linked Immunosorbent Assay ; Fluorescent Antibody Technique ; Fluorescent Antibody Technique, Indirect ; Glomerulonephritis ; Glomerulonephritis, IGA* ; Humans ; Immunoglobulin A* ; Male ; Microscopy, Electron ; Middle Aged ; Prednisolone ; Proteinuria ; Urinalysis