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This Concept Map, created with IHMC CmapTools, has information related to: Childhood Dz II, Infancy, Childhood II NEONATAL RDS AKA Hyaline Memb Dz Membranes form in periph airspaces Clinical (w/out Tx): preterm, AGA, often DM in mom, C-section 30min-retraction of ribs, sternum on insp -expiratory grunt few hrs-cyanosis, fine rales ETIOLOGY-immature lungs- in 60% of 28wk gest, 20% of 32-36wk, 5% after 37 wks -deficiency in pulm surfactant (type II) -made after 35th wk gest -w/out surf-lungs collapse every exhalation -soft thoracic wall pulled in as diaph descends ->progressive atelectasis->protein exudate ->hyaline membranes->hypoxemia Surfactant suppressed by high insulin (DM mom) Enhanced by corticosteroids MORPH-solid, airless, liver-colored lungs on gross -necrotic cell debris(type II) in airways, membs -fibrin in membranes, little inflam -repair after 48 hrs CLINICAL-try to to delay labor-monitor amn PLs -prophylactic surfactant ដ-28 wks -Tx based on Sx after that-high-freq vent -Recovered pts risk: PDA, intravent hemorrh, necrotizing enterocolitis, Membranes form in periph airspaces Clinical (w/out Tx): preterm, AGA, often DM in mom, C-section 30min-retraction of ribs, sternum on insp -expiratory grunt few hrs-cyanosis, fine rales ETIOLOGY-immature lungs- in 60% of 28wk gest, 20% of 32-36wk, 5% after 37 wks -deficiency in pulm surfactant (type II) -made after 35th wk gest -w/out surf-lungs collapse every exhalation -soft thoracic wall pulled in as diaph descends ->progressive atelectasis->protein exudate ->hyaline membranes->hypoxemia Surfactant suppressed by high insulin (DM mom) Enhanced by corticosteroids MORPH-solid, airless, liver-colored lungs on gross -necrotic cell debris(type II) in airways, membs -fibrin in membranes, little inflam -repair after 48 hrs CLINICAL-try to to delay labor-monitor amn PLs -prophylactic surfactant ដ-28 wks -Tx based on Sx after that-high-freq vent -Recovered pts risk: PDA, intravent hemorrh, necrotizing enterocolitis O2 Tx risk RISK-oxygen toxicity-retrolental firboplasia (retinopathy)-dec'd VEGF in hyperoxia->apop -proliferation w/ room air Bronchopulm dysplasia-O2 dependence at 28days -resp distress up to 3-6mo -epith hyperplasia, squamous metaplasia, alv wall thickening, fibrosis -major problem w/ very low birth weight (gm), Infancy, Childhood II Tumors, Tumor-like Lesions HAMARTOMA -excessive, focal growth of NL cells -in correct organ, but too much -incorrect architecture, Infancy, Childhood II Tumors, Tumor-like Lesions HETEROTOPIA (CHORISTOMA) -NL cells in ABNL location eg-panc tissue in sm. int -adrenal cells in kidney, lung, ov -can be origin of true neoplasm -paradoxical presentation!, Infancy, Childhood II SIDS Unexplained sudden death of əyo -usually while asleep (crib/cot death) -6000 US deaths/yr-most common COD -90% ɞmo, esp 2-4 mo -most w/ URI preceding death Risk factors: sleeping prone, thermal stress, heavy wrapping, overnight heating Mom: ងyo, unmarried, low SES, smoking, short time b/w pregs, drugs (methadone) Infant: low birth wt, male, premie, mult birth not first sibling, SIDS in prior sibling MORPH-CNS astrogliosis of stem -EMH in liver, periadrenal BROWN FAT -increased chromaffin cell volume -bundle of His, SA node ABNL, RVHypertrophy -hypoxia->pulm congestion, edema -bottom line - NONSPECIFIC PATHOGENESIS-10% due to IEM -'idiopathic'-apnea and ABNL temp control b/c premie, low birth wt -underlying defect may arise in utero, Infancy, Childhood II Causes of Death ə yo Congenital anomalies, prematurity, SIDS 1-4yo Injuries, congen anomalies, malignancies 5-14yo Injuries from accidents, maligs, Homicide, Infancy, Childhood II ERYTHROBLASTOSIS FETALIS Hemolytic Dz of the Newborn BLOOD GROUP INCOMPATIBILITY-ABO or Rh-D -leakage of fetal RBCs to mom (əmL) -mom sensitized during 3rd tri or delivery ->ABs cross placenta to kid (only IgG cross) -thus uncommon in 1st preg-IgG after Tx-RhIg-anti-Rh ABs - at 28 wks and before deliv ABO incomp in 25% of pregs-hemolytic in 10% -most anti-A,B are IgM - don't cross -mostly happens in O moms -no protection against ABO rxns-severe in 0.5% Consequences - ANEMIA, JAUNDICE (bilirubin) -hep'splen'meg w/ extramed hemato. -unconj bili binds lipids in brain-kernicterus -anemia can->heart, liver failure->hypoproteinemia -anasarca (hydrops fetalis) - only 10% from EF (CV malformations the highest cause of HF) MORPH-range from stillborn to recovery -kernicteric brain-enlarged, edematous, yellow -esp in BASAL GANG, THALAMUS, CBLM, CEREBRAL GRAY MATTER, CORD -dmg rare if bili ងmg/dL -hyperactive RBC series-inc'd reticulocytes, normos -EMH in liver, spleen, LNs, kidneys, lungs, heart -edema in peritoneal, pleural, pericardial cavities CLINICAL-poss pallor, jaundice, megaly, edema Tx-photoTx-light oxidizes unconj bili -total exchange transfusion if severe -high dose IV Ig, Infancy, Childhood II Tumors, Tumor-like Lesions WILMS' TUMOR -most common primary renal tumor -2-5yo PATHOGENESIS- 1-WAGR Syndrome: aniridia, genital anoms, MR -33%->wilms' -delete WT-1 on chrom 11 p13 2-Denys-Drash Syn: gonadal dysgenesis, nephropathy->renal failure -mutated WT-1, not deleted 3-Beckwith-Wiedemann Syn: organomeg, hemihypertrophy, large ad cortex cells -WT-2 Chrom 11 p15.5 (distal to WT-1) -UNimprinted maternal allele also->hep'blastoma, AdCort tumors, rhabdos, panc tumors Some familial Wilms' tumors: WT-3(?) Nephroblastomatosis-immature kidney elements in otherwise NL kidney - premalignant lesion! MORPH-large, solitary, well-circumscribed Triphasic- blastemic, stromal, epith cells- in most -can have lots of different tissue-fat, mm, gland Anaplasia-worse prognosis Epithelial diff'tion - BETTER PROGNOSIS CLINICAL-large abd mass, asymmetrical, HTN, hematuria, pain -often w/ lung mets at presentation Tx-CTX+XRT+surgery-if used, 90% survival -can->secondary tumors-bone, soft t, leuk, lymphoma, brain, GU, Infancy, Childhood II Tumors, Tumor-like Lesions BENIGN Hemangiomas-MOST COMMON INFANT TUMOR -same as in adults-can enlarge w/ growth -can also spon. regress -skin, face - rarely becomes malignant -larger = port-wine stains -can signal von Hippel-Lindau dz Lymphatic Tumors- -lymphangiomas-cystic, cavernous spaces -often deep in neck, axilla, MS, retroperi -bigger after birth->encroachment problems -lymphangiectasis-dilations of lymph channels ->diffuse swelling of body part/organ -UGLY, but doesn't spread Fibrous Tumors-some spon regress -myofibromatoses w/ mm-specific actin -usually solitary-multifocal=>worse outcome -fibromatosis-spindly cells -infantile fibrosarcoma Teratomas-benign, cystic or solid, malignant Peaks-2 yo AND late adolescence-early adult -most in sacrococcygeal region gonads, midline -more in girls-can be w/ hydrops -12% malignant-lethal - often w/ older kids -benign in younger kids, Infancy, Childhood II Tumors, Tumor-like Lesions MALIGNANT-leading dz COD 4-14 yo -close relationship b/w terato-, oncogenesis -can regress or cytodifferentiate -mostly hematopoietic system, nervous tissue, soft tissue, bone, kidney ALL>Neuroblastoma>Wilms' tumor> hepatoblastoma>retinoblastoma>rhabdomyo> teratoma>Ewing sarcoma>juv astrocytoma> medulloblastoma>ependymoma '-blastoma'-b/c more embryonal features -small round blue cell tumors, Infancy, Childhood II Tumors, Tumor-like Lesions Neuroblastoma-most common in əyo -mostly sporadic occurence MORPH-25-35% in adrenal medulla -sympa chain -mostly regress spon'ly -some invasive, some not, some w/ capsule -brainlike tissue-small, dark cells in sheets -Homer-Wright pseudorosettes Ganglioneuroma-most well-diff'd NB-no blasts -wide, early mets CLINICAL- ɚyo-wt loss, large abd mass, fever -older not as evident->mets əyo excellent prognosis Ploidy of tumor cells->good outcome 70-80% w/ deletion on Chrom 1 band p36 -worse prognosis (also w/ Chrom 14 deletion) -amplified N-myc in 25%->worse prog -High TrkA->good prognosis