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6. sudden infant death syndrome (sids); pediatric pathology


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6. sudden infant death syndrome (sids); pediatric pathology

  1. 1. Pediatric Pathology Pediatric Pathology 1 Dr. Krishna Tadepalli, MD,
  2. 2. 6. Sudden Infant Death Syndrome 6. Sudden Infant Death Syndrome 2 Dr. Krishna Tadepalli, MD,
  3. 3. Sudden Infant Death Syndrome (SIDS) • • • • • • • • • Definition = "the sudden death of an infant under 1 year of age which remains unexplained after a thorough case investigation, including performance of a complete autopsy, examination of the death scene, and review of the clinical history” Usually dies while asleep, mostly in the prone or side position (pseudonyms of crib death or cot death) Cause =unknown Epidemiology Leading cause of death between age 1 month and 1 year in USA Third leading cause of infant deaths 90% of deaths occur during the first 6 months of life (peak- 2 and 4 months); this is independent of risk factors & geographic locale "apparent life-threatening event" (ALTE),= apnea + marked change in color or muscle tone+ choking or gagging Risk Factors: table in next slide 3 Dr. Krishna Tadepalli, MD,
  4. 4. Table 10-7. Risk Factors and Postmortem Findings Associated with SIDS Table 10-7. Risk Factors and Postmortem Findings Associated with SIDS PARENTAL Young maternal age (age <20 years) Maternal smoking during pregnancy Drug abuse in either parent, specifically paternal marijuana and maternal opiate, cocaine use Short intergestational intervals Late or no prenatal care Low socioeconomic group African-American and American Indian ethnicity (? socioeconomic factors) INFANT Brain stem abnormalities, associated with delayed development of arousal and cardiorespiratory control Prematurity and/or low birth weight Male sex Product of a multiple birth SIDS in a prior sibling Antecedent respiratory infections Germline polymorphisms in autonomic nervous system genes ENVIRONMENT Prone or side sleep position Sleeping on a soft surface Hyperthermia Co-sleeping in first 3 months of life 4 Dr. Krishna Tadepalli, MD,
  5. 5. Table 10-7. Risk Factors and Postmortem Findings Associated with SIDS Table 10-7. Risk Factors and Postmortem Findings Associated with SIDS POSTMORTEM ABNORMALITIES DETECTED IN CASES OF SUDDEN UNEXPECTED INFANT DEATH* Infections • Viral myocarditis • Bronchopneumonia Unsuspected congenital anomaly • Congenital aortic stenosis • Anomalous origin of the left coronary artery from the pulmonary artery Traumatic child abuse • Intentional suffocation (filicide) Genetic and metabolic defects • Long QT syndrome (SCN5A and KCNQ1 mutations) • Fatty acid oxidation disorders (MCAD, LCHAD, SCHAD mutations) • Histiocytoid cardiomyopathy (MTCYB mutations) • Abnormal inflammatory responsiveness (partial deletions in C4a and C4b) 5 Dr. Krishna Tadepalli, MD,
  6. 6. Sudden Infant Death Syndrome (SIDS) • • • • • • • • Morphology Multiple petechiae =MC finding (seen in thymus, pleura, and epicardium) Lungs =congested with vascular engorgement ± pulmonary edema CNS = fails to provide a clear cause of death (hypoplasia of arcuate nucleus, astrogliosis of the brain stem and cerebellum) Hepatic extramedullary hematopoiesis and periadrenal brown fat Pathogenesis (table 10.7) Multifactorial A "triple-risk" model – vulnerable infant – critical developmental period in homeostatic control – exogenous stressor • Most compelling hypothesis =delayed development of "arousal" and cardiorespiratory control (which in turn under control of serotonergic (5-HT) system of the medulla of brainstem Traumatic child abuse must be carefully excluded under these circumstances SIDS = Diagnosis of exclusion, requiring careful examination of the death scene 6 and a complete postmortem examination. Dr. Krishna Tadepalli, MD,
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