Syndromes associated

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  • Tuyển tập các báo cáo nghiên cứu về y học được đăng trên tạp chí y học General Psychiatry cung cấp cho các bạn kiến thức về ngành y đề tài: Biomarker profiles in serum and saliva of experimental Sjögren's syndrome: associations with specific autoimmune manifestations...

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  • Tuyển tập các báo cáo nghiên cứu về y học được đăng trên tạp chí y học Critical Care giúp cho các bạn có thêm kiến thức về ngành y học đề tài: Video of dantrolene effectiveness on neuroleptic malignant syndrome associated muscular rigidity and tremor...

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  • Tuyển tập các báo cáo nghiên cứu về y học được đăng trên tạp chí y học quốc tế cung cấp cho các bạn kiến thức về ngành y đề tài: Systemic Capillary Leak Syndrome associated with hypovolemic shock and compartment syndrome. Use of transpulmonary thermodilution technique for volume management

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  • Tuyển tập báo cáo các nghiên cứu khoa học quốc tế ngành y học dành cho các bạn tham khảo đề tài: Immune reconstitution inflammatory syndrome associated with acquired immunodeficiency syndrome-related ...

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  • Tuyển tập báo cáo các nghiên cứu khoa học quốc tế ngành y học dành cho các bạn tham khảo đề tài: Mirizzi syndrome associated with hepatic artery pseudoaneurysm: a case report

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  • Tuyển tập các báo cáo nghiên cứu về hóa học được đăng trên tạp chí hóa học đề tài : Validity and reliability of the Spanish version of the DN4 (Douleur Neuropathique 4 questions) questionnaire for differential diagnosis of pain syndromes associated to a neuropathic or somatic component

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  • Tuyển tập các báo cáo nghiên cứu về y học được đăng trên tạp chí y học General Psychiatry cung cấp cho các bạn kiến thức về ngành y đề tài: Identification of possible candidate genes regulating Sjögren's syndrome-associated autoimmunity: a potential role for TNFSF4 in autoimmune exocrinopathy...

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  • Specific Paraneoplastic Neurologic Syndromes (Table 97-3) Table 97-3 Antibody-Associated Paraneoplastic and Nonparaneoplastic Syndromesa Antibodies Paraneoplastic Syndrome Frequent t Infrequen stic Nonparaneopla Limbic encephalitis Ma2, Hu, CV2/CRMP5, anti-NR1/NR2 of NMDA receptor Tr, VGKC VGKC Cerebellar degeneration Yo, Tr, mGluR1; Gliadin, GAD P/Q VGCC, Hu, MAZ Zic, CV2/CRMP5, Ma1-2 Ri, Hypothalamic, brainstem encephalitis Ma2, Hu P5 CV2/CRM Encephalomye litis Hu, Zic P5, CV2/CRM Ri, amphiphysin Chorea P5 CV2/CRM Opsoclonusmyoclonus Ri Yo, Hu, Ma2, Stif...

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  • Bone Marrow The bone marrow is usually normal or hypercellular, but in 20% of cases it is sufficiently hypocellular to be confused with aplasia. No single characteristic feature of marrow morphology distinguishes MDS, but the following are commonly observed: dyserythropoietic changes (especially nuclear abnormalities) and ringed sideroblasts in the erythroid lineage; hypogranulation and hyposegmentation in granulocytic precursors, with an increase in myeloblasts; and megakaryocytes showing reduced numbers or disorganized nuclei.

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  • Ectopic hormone production would only be an epiphenomenon associated with cancer if it did not result in clinical manifestations. Excessive and unregulated production of hormones such as ACTH, PTHrP, or vasopressin can lead to substantial morbidity and can complicate the cancer treatment plan. Moreover, the paraneoplastic endocrinopathies are sometimes the presenting feature of underlying malignancy and may prompt the search for an unrecognized tumor.

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  • Hematologic Syndromes: Introduction The elevation of granulocyte, platelet, and eosinophil counts in most patients with myeloproliferative disorders is caused by the proliferation of the myeloid elements due to the underlying disease rather than a paraneoplastic syndrome. The paraneoplastic hematologic syndromes in patients with solid tumors are less well characterized than the endocrine syndromes because the ectopic hormone(s) or cytokines responsible have not been identified in most of these tumors (Table 96-2).

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  • Clinical Manifestations Patients with cancer who develop deep venous thrombosis usually develop swelling or pain in the leg, and physical examination reveals tenderness, warmth, and redness. Patients who present with pulmonary embolism develop dyspnea, chest pain, and syncope, and physical examination shows tachycardia, cyanosis, and hypotension. Some 5% of patients with no history of cancer who have a diagnosis of deep venous thrombosis or pulmonary embolism will have a diagnosis of cancer within 1 year.

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  • Pathogenesis Most PNDs are mediated by immune responses triggered by neuronal proteins (onconeuronal antigens) expressed by tumors. In PNDs of the central nervous system (CNS), many antibody-associated immune responses have been identified (Table 97-2). These antibodies usually react with the patient's tumor, and their detection in serum or cerebrospinal fluid (CSF) strongly predicts the presence of cancer. The target antigens are usually intracellular proteins with roles in neuronal development and function.

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  • Paraneoplastic Encephalomyelitis and Focal Encephalitis The term encephalomyelitis describes an inflammatory process with multifocal involvement of the nervous system, including brain, brainstem, cerebellum, and spinal cord. It is often associated with dorsal root ganglia and autonomic dysfunction. For any given patient, the clinical manifestations are determined by the area or areas predominantly involved, but pathology almost always reveals abnormalities (inflammatory infiltrates, neuronal loss, gliosis) beyond the symptomatic regions. ...

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  • Encephalitis and Encephalomyelitis: Treatment Most types of paraneoplastic encephalitis and encephalomyelitis respond poorly to treatment. Stabilization of symptoms or partial neurologic improvement may occasionally occur, particularly if there is a satisfactory response of the tumor to treatment. The roles of plasma exchange, IVIg, and immunosuppression have not been established. Approximately 30% of patients with anti-Ma2-associated encephalitis respond to treatment of the tumor (usually a germ-cell neoplasm of the testis) and immunotherapy.

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  • Paraneoplastic myelitis may present with upper or lower motor neuron symptoms, segmental myoclonus, and rigidity. This syndrome can appear as the presenting manifestation of encephalomyelitis and may be associated with SCLC and serum anti-Hu, anti-CV2/CRMP5, or anti-amphiphysin antibodies. Paraneoplastic myelopathy can also produce several syndromes characterized by prominent muscle stiffness and rigidity.

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  • Vasculitis of the nerve and muscle causes a painful symmetric or asymmetric distal sensorimotor neuropathy with variable proximal weakness. It predominantly affects elderly men and is associated with an elevated erythrocyte sedimentation rate and increased CSF protein concentration. SCLC and lymphoma are the primary tumors involved. Pathology demonstrates axonal degeneration and T cell infiltrates involving the small vessels of the nerve and muscle. Immunosuppressants (glucocorticoids and cyclophosphamide) often result in neurologic improvement.

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  • Aplastic Anemia Definition Aplastic anemia is pancytopenia with bone marrow hypocellularity. Acquired aplastic anemia is distinguished from iatrogenic marrow aplasia, marrow hypocellularity after intensive cytotoxic chemotherapy for cancer. Aplastic anemia can also be constitutional: the genetic diseases Fanconi's anemia and dyskeratosis congenita, while frequently associated with typical physical anomalies and the development of pancytopenia early in life, can also present as marrow failure in normal-appearing adults.

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  • Neuronal cell-surface antigens can be the target of antibodies in some patients with paraneoplastic encephalitis. A few of these antigens have been identified, including the NR1/NR2 subunits of NMDA receptors (Fig. 97-1) and voltage-gated potassium channels (VGKC). These disorders are more responsive to immunotherapy than those associated with immune responses to intracellular antigens. Figure 97-1 Antibodies to NR1/NR2 subunits of the NMDA receptor in a patient with paraneoplastic encephalitis and ovarian teratoma.

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  • Birthmarks are among the most common types of anomalies encountered by the pediatrician in practice. Moreover, they are a source of significant concern for parents regardless of whether they are associated with an underlying systemic abnormality. Pediatricians are often called upon in the neonatal period to establish the diagnosis and direct management. Recognition of the types of birthmarks that require additional evaluation or herald a potentially problematic course is essential when examining an infant.

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