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- Malakoplakia
- Disrupted Development
Transitional cell carcinomainvades by extension - many carcinogenic causes - usually papillary grade - cytology / stage - invasion (inner third of muscle is still safe) smoking is a risk factor Treatment: ?resection, chemo Transitional cell papillomaseven or fewer layers Squamous cell carcinoma of the bladder associated with schistosomiasis (haematobium) Adenocarcinoma of the bladder Cystitis glandularis may resemble adenocarcinoma Malakoplakiainflammatory pattern showing michaelis-gutmann bodies (calcified nodules) Developmental / Pediatrics [Pediatric Infectious Disease] [vaccination] [Fluid Maintenance] Teratogenesis Maternal environment / normal morphogenesis / disrupted development Pulmonary lung malformations, neonatal lung Heme hyperbilirubinemia, ABO incompatibility GI Liver Congenital Heart Disease Bone Juvenile RA Derm atopic dermatitis, seborrheic dermatitis, miliaria rubra Neuro NEC, PVLM Childhood Tumors histiocytoses, renal tumors, neural tumors, other tumors Genetic Syndromes Down’s, Turner’s, etc. Metabolic Disorders Amino acids Homocystinuria, PKU, porphyria Other enzyme deficiencies Lysosomal storage disorders Mucopolysaccharidoses Hurler, Schei, Hunter Glycogen storage disorders hepatic hypoglycemia Von Gierke’s, Pompe’s muscle energy disorders McArdle’s Fetal Immune System Teratogenesis (see pharm) 1-2% live births have obvious congenital malformations (suspected 10%) / account for 30-40% hospitalizations organogenesis 14-56 days, after which teratogens cause deformation, retardation rubella – 1st trimester DES - vaginal glands, clear cell carcinoma AD - Marfan’s / neurofibromatosis AR - errors of metabolism XLR - imperforate anus / congenital cataract multifactorial (gen/env) - cleft lip / pyloric stenosis Maternal Environment transplacental - rubella, coxsackie, hepatitis, HSV, CMV, toxoplasmosis, other (malaria, listeria) delivery - HSV, syphilis, Group B Streptococcus perinatal: low virulence (E. Coli, Aerobacter, Alcaligenes, Proteus, Group B strep)
fetus swallows maternal PMN’s villitis: transplacental (may be asymptomatic) funisitis: inflammation of umbilical cord nutrition: malnutrition, obesity / endocrine: DM, hyperthyroid blood dyscrasias: hypochromic anemia (prematurity) / sickle cell / ITP early abortion: chromosome anomaly, cytotoxic (UV, methotrexate), implantation error, trauma
placenta accreta: absent decidua basalis, villi enter myometrium placenta abruptio: separation of placenta from uterus bilobate (succenturiate) placenta: marginal (Battledore) insertion: 1 in 5 / usu. asymptomatic velamentous insertion: placenta in membranes / bleeding risk at delivery single umbilical artery: 1/4 to ½ with malformations placenta extrachorialis: membranes insert on fetus rather than margins amnion nodosum: oligohydramnios causes squamous cell aggregates on fetal skin amniotic bands: adhere to fetus / compress, constrict IUGR: occurs with > 15% placental infarction Pre-eclampsia (see DIC) twins: 1/80 1/3 are monozygotic, 2/3 are dizygotic twin transfusion syndrome / fetus papyraceous Normal Morphogenesis embryo: up to 8 wks <30 mm lungs: embryonic 3-6 / pseudoglandular 6 - 16 / canalicular 16 - 24 / terminal sac 24 - / alveolar 40 kidneys: EC “cap” persists over glomeruli for 18-24 months postnatally liver: extramedullary hematopoeisis up to 14 days after birth lymph: primary follicles at 24 wks / germinal centers 4-6 wks after birth adrenals: large fetal cortex involutes at birth (produces DHEA precursor for placental estriol E-3) full term: 38-43 wks / 48 cm / 3200g prematurity: <37 wks gestation / <26 wks marginally viable / 7.5% of births / 64% neonatal death type 1 IUGR: symmetric / fetal abnormality type 2 IUGR: asymmetrical with CNS sparing / maternal and placental abnormality Disrupted Developmenthighest susceptibility 14 - 56 days dive reflex - pulmonary artery constriction to preserve brain perfusion RF: prematurity, DM, twins, C-section HMD (lack of surfactant) occurs at 4 hours / surfactant by 28 wks vascular spasm causes underperfusion / ischemia causes transudation / fluid and hyaline block respiration BPD follows barotrauma and high oxygen therapy results in retrolental fibroplasia
embryonic 3-6 wks pseudoglandular 6 - 16 - cartilage, cilia, goblet cells, glands canalicular 16 - 28 - acinae, septa, type I and II pneumocytes terminal sac saccular 28 - 34 - surfactant alveolar 40 - 2 yrs Respiratory Distress Syndrome Prematurity PDA – pulmonary edema – capillary leak (see cardiac) surfactant deficiency - immature lung structure Surfactant – phosphatidylcholine (lecithin) 70%, phosphatidylglycerol 10%, phosphatidylethanolamine 5%, sphingomyelin 2%, other 3%, proteins 5%, SP A-D 5% Presentation: tachypnea, nasal flaring, subcostal and intercostal retractions, expiratory grunt, cyanosis, diminished air entry CXR: symmetric, homogeneous, ground glass appearance (not well aerated) – also decreased pneumothorax mortality Diagnosis: evidence of prematurity, signs and lab, other causes (group B streptococcus), radiologic evidence of HMD (reticulo-granular pattern, air bronchograms [because collapsed lung tissue allows dark bronchi to be seen]) Complications: ICH, PDA, BPD, ROP, Secondary infection, Rupture of Lungs Treatment: supportive care: O2 and/or ventilation, intubation Drug therapy: natural surfactant from cow’s lungs - human amniotic fluid (not practical, requires 10 C-sections to get one therapy), alveolar washing and lung extracts from cows, pigs, etc. synthetic surfactant (no protein) used to tremendous success, 30% decrease in mortality, 1% decreased in overall infant mortality in US) Prevention: distributes better in liquid interface, may treat when not necessary (40%) (one study shows advantage to prevention therapy before 26 weeks gestation) Congenital Malformations of the Lungs Hypoplastic lung compression, oligohydramnios, obstruction, vascular, CNS Brachial plexus injury, phrenic nerve injury One lung is small on CXR because diaphragm is not functional Pulmonary sequestration acessory lung blood supply systemic, not pulmonary artery extralobar sequestration mass of lung separated, systemic blood supply, diaphragmatic hernia (left) intralobar sequestration more common / systemic blood supply / posterior-basal lung cystic or solid inflammation bronchial connection to foregut predisposes to infection Cystic disease bronchogenic lymphangiectasis may accompany HMD / venous return obstruction / Turner’s syndrome cystic adenomatoid malformation gastric epithelium (type 1) / unilobar / associated with heart and kidney malformations / prematurity / type 3 is fatal congenital lobar emphysema hyperexpansion due to bronchus collapse / unilobar / normal septa (not true emphysema) Neonatal Pulmonary Disease Hyaline membrane disease insulin retards surfactant production / thyroxine, glucocorticoids promote surfactant / appears after 4 hours of life / macrophages present after 24 hrs. Bronchopulmonary Dysplasia BPD artificial ventilation, oxygen therapy - may lead to cor pulmonale Stage 1 (2-3 days) - acute, exudative / hyaline membranes, atelectasis, lymphatic dilatation Stage 2 (4-10 days) - regeneration / necrosis, repair, hyperaeration Stage 3 (10-20days) - transition / bronchiolitis obliterans, histiocytes Stage 4 (1 month) - honeycombing, peribronchial muscle hypertrophy, squamous metaplasia Pneumonia (see other) true pneumonia (fibrinopurulent, hyaline exudate) v. aspiration of maternal WBC’s Meconium aspiration intrauterine distress causes excess meconium release into amniotic sac marked abnormalities and other complications as in BPD Hx: term or post-term, asphyxia CXR: hyperinflation (lowered diaphragm), clumpy distribution of lung abnormalities Treatment: mechanical ventilation, lavage techniques being investigated Download 3.95 Mb. Share with your friends:
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