Chapter 10 Part 3 Flashcards
Sudden Infant Death Syndrome definition
sudden death of an infant under 1 yr 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; diagnosis of EXCLUSION
Examples of SIDS risk factors
young maternal age, drug use, low socioeconomic group, male sex, SIDS in prior sibling, prematurity, hyperthermia, co-sleeping, child abuse, long QT syndrome, etc.
Common postmortem morphological findings
petechiae, congested lungs, vascular engorgement, astrogliosis of brainstem, hypoplasia of dentate nucleus
Key risk factors for SIDS
vulnerable infant, critical developmental period in homeostatic control, exogenous stressor
Leading hypothesis for SIDS pathogenesis
delayed development of “arousal” and cardioresp control (medulla oblongata), defective serotonin pathway
Function of laryngeal chemoreceptors as they relate to SIDS
Typically elicit a cardiorespiratory inhibitory effect, stimulation augmented by resp infection and prone position
Common environmental stressors that are associated with SIDS
maternal smoking, thermal stress, sleeping on soft surfaces, prone or side sleeping
Definition of benign tumor
gross and microscopic appearances considered innocent, meaning it will remain localized, not spread, and is amenable to surgical removal
Definition of heterotopia
microscopically normal cells or tissues that are present in abnormal locations
Definition of hamartoma
excessive focal overgrowth of cells and tissues native to the organ (ex. hemangioma, lymphangioma, adenoma of the liver)
Hemangioma
most common tumor of infancy, cavernous or capillary, most will spontaneously regress; can be indicative of von Hippel-Lindau disease
Sturge-Weber Syndrome
sporadic congenital neurocutaneous disorder characterized by port-wine stain affecting skin in ophthalmic branch of CNV, abnormal capillary venous vessels in leptomeninges of brain and choroid, glaucoma, seizures, stroke, intellectual disability
Lymphatic tumors
lymphangiomas (hamartomatous) or lymphangiectasis; often occurs in neck, axilla, mediastinum
Fibrous tumor genetic anomaly
can look like adult fibrosarcomas; chromosomal translocation t(12;15)(p13;q25) resulting in ETV6-NTRK3 transcript causing stimulation of oncogenic RAS and PI3K/AKT pathway
Teratoma
can be benign or malignant, peak incidence at 2 yrs of age or late adolescence/early adulthood; more mature=better prognosis
Sacrococcygeal teratoma
congenital anomalies in hindgut, cloacal region and midline defects
Characteristics of pediatric neoplasms
histologically unique, primitive, sheets of cells with small, round nuclei, organogenesis specific to site of tumor
In what tissues do most pediatric cancers arise?
hematopoietic system, soft tissues, nervous system, bone, kidney
Most common neoplasms in children under 10
leukemia, neuroblastoma, wilms tumor, hepatoblastoma, retinoblastoma, rhabdomyosarcoma, teratoma, ewing sarcoma, astrocytoma, medulloblastoma, ependymoma
Neuroblastic tumors
most common extracranial solid tumor of childhood, occur sporadically but 1-2% are familial (ALK gene), tumors of sympathetic ganglia and adrenal medulla arising from neural crest population
Key characteristics of neuroblastomas
spontaneous or therapy-induced differentiation of primitive neuroblasts into mature elements, spontaneous regression, wide range of clinical behavior and prognosis
Clinical presentation of neuroblastoma
<2 yo with large abdominal mass, fever, with or without weight loss, cutaneous metastases; >2yo presents with mets (periorbital region, lungs, blood, liver, bones)
Key diagnostic feature of neuroblastoma
catecholamines increased in blood, VMA and HVA increase in urine
Gross appearance of neuroblastoma
soft, gray-tan tissue, areas of necrosis, cystic softening, hemorrhage, calcification
