DISEASES OF INFANCY & DISEASES OF INFANCY & CHILDHOOD ICHILDHOOD I

R. HUGO MARTÍNEZ LOZANO, M.D.R. HUGO MARTÍNEZ LOZANO, M.D.

Aug-28-2009.Aug-28-2009.

LEARNING OBJECTIVES: K & ULEARNING OBJECTIVES: K & U

GENERAL FEATURESGENERAL FEATURES CONGENITAL ANOMALIESCONGENITAL ANOMALIES BIRTH WEIGHT & GESTATIONAL AGEBIRTH WEIGHT & GESTATIONAL AGE BIRTH INJURIESBIRTH INJURIES PERINATAL INFECTIONSPERINATAL INFECTIONS NEONATAL RDSNEONATAL RDS NECROTIZING ENTEROCOLITISNECROTIZING ENTEROCOLITIS GM-VENTRICULAR HEMORRHAGESGM-VENTRICULAR HEMORRHAGES HYDROPS FETALISHYDROPS FETALISRef: pp. 470-486 & lecture’s informationRef: pp. 470-486 & lecture’s information

GENERAL FEATURES: GENERAL FEATURES: MORTALITY RATESMORTALITY RATES

< 1 YEAR 727.4< 1 YEAR 727.4 1-4 YEARS 32.61-4 YEARS 32.6 5-14 YEARS 18.55-14 YEARS 18.5 15-24 YEARS 80.715-24 YEARS 80.7Rates: per 100,000 population/year.Rates: per 100,000 population/year.

CONGENITAL ANOMALIES: CONGENITAL ANOMALIES: DEFINITIONSDEFINITIONS

MALFORMATIONSMALFORMATIONS DISRUPTIONSDISRUPTIONS DEFORMATIONSDEFORMATIONS SECQUENCESSECQUENCES SYNDROMESSYNDROMES

MALFORMATIONSMALFORMATIONS

PRIMARY ERROR OFMORPHOGENESIS

DISRUPTIONSDISRUPTIONS

SECONDARY DESTRUCTION (AMNIOTIC BANDS)

IDEM

DEFORMATIONSDEFORMATIONSEXTRINSIC DEFORMITY(BIOMECHANICAL CAUSES)

CLUBFEET

Deformation but no destruction

SEQUENCESSEQUENCES

POTTER’S FACIES (OLIGOHIYDRAMNIOS)

CLUB FEET (VARUS TYPE)

SEVERE PULMONARY HYPOPLASIA (OLIGOHYDRAMNIOS)

NORMAL

SYNDROMESSYNDROMES

MICROCEPHALY, CYCLOPIA, PROBOSCIS, POLYDACTILY AND ABNORMALKARIOGRAM NOT related to a single initiating defect

CONGENITAL ANOMALIES: CONGENITAL ANOMALIES: CAUSESCAUSES

GENETIC 12-25%GENETIC 12-25% ENVIRONMENTAL 10-13%ENVIRONMENTAL 10-13% MULTIFACTORIAL 20-25%MULTIFACTORIAL 20-25% IDIOPATIC 40-60%IDIOPATIC 40-60%

POLICYSTICKIDNEY SX.

FAS

NEUROAXIS DEFECT: Most likely environmental deficiency?

ANENCEPHALY

CONGENITAL TUMORS

MalformationMalformationFrequency per 10,000 Total Frequency per 10,000 Total BirthsBirths

Clubfoot without central nervous system Clubfoot without central nervous system anomaliesanomalies

25.725.7

Patent ductus arteriosusPatent ductus arteriosus 16.916.9

Ventricular septal defectVentricular septal defect 10.910.9

Cleft lip with or without cleft palateCleft lip with or without cleft palate 9.19.1

Spina bifida without anencephalusSpina bifida without anencephalus 5.55.5

Congenital hydrocephalus without Congenital hydrocephalus without anencephalusanencephalus

4.84.8

AnencephalusAnencephalus 3.93.9

Reduction deformity (musculoskeletal)Reduction deformity (musculoskeletal) 3.53.5

Rectal and intestinal atresiaRectal and intestinal atresia 3.43.4

CONGENITAL ANOMALIES: 3% 1st. CAUSE OF DEATH UNDER 1 YEAR.

BIRTH WEIGHT & GESTATIONAL BIRTH WEIGHT & GESTATIONAL AGEAGE

AGAAGA SGASGA LGALGA

37 W----------------42W37 W----------------42W

PRETERM FULL-TERM POST-TERMPRETERM FULL-TERM POST-TERM

SignSign 00 11 22

Heart rateHeart rate AbsenAbsentt

Below 100Below 100 Over 100Over 100

Respiratory effortRespiratory effort AbsenAbsentt

Slow, irregularSlow, irregular Good, Good, cryingcrying

Muscle toneMuscle tone LimpLimp Some flexion of Some flexion of extremitiesextremities

Active Active motionmotion

Response to catheter in nostril (tested Response to catheter in nostril (tested after oropharynx is clear)after oropharynx is clear)

No No responresponsese

GrimaceGrimace Cough or Cough or sneezesneeze

ColorColor Blue, Blue, palepale

Body pink, Body pink, extremities blueextremities blue

CompleteCompletely pinkly pink

APGAR SCORE

PREMATURITYPREMATURITY 2nd. IN NEONATAL MORALITY2nd. IN NEONATAL MORALITY RISK FACTORS: -PPROMRISK FACTORS: -PPROM -INTRAUTERINE INFECTION-INTRAUTERINE INFECTION -STRUCTURAL ABNORMAL.-STRUCTURAL ABNORMAL. -MULTIPLE GESTATIONS-MULTIPLE GESTATIONS COMPLICATIONS -RDSCOMPLICATIONS -RDS -NECROTIZING EC-NECROTIZING EC -SEPSIS-SEPSIS -BRAIN HEMORRAGHE-BRAIN HEMORRAGHE

KERNICTERUSKERNICTERUS

EXTREME PREMATURITY

CAPUT SUCCEDANUM

BIRTH INJURIES

PERINATAL INFECTIONSPERINATAL INFECTIONS

TRANSCERVICAL (ASCENDING):TRANSCERVICAL (ASCENDING):

- CHORIOAMNIONITIS- CHORIOAMNIONITIS

- HVS-II- HVS-II

- PAPILLOMATOSIS- PAPILLOMATOSIS

- CHLAMYDIASIS- CHLAMYDIASIS

HERPETIC ENCEPHALITIS

PERINATAL INFECTIONSPERINATAL INFECTIONS

TRANSPLACENTAL:TRANSPLACENTAL:

- TOXOPLASMOSIS- TOXOPLASMOSIS

- MALARIA- MALARIA

- RUBELLA- RUBELLA

- CMV- CMV

- HEPATITIS B- HEPATITIS B

- PARVOVIRUS B19- PARVOVIRUS B19

- BACTERIA (TREPONEMA, LISTERIA)- BACTERIA (TREPONEMA, LISTERIA)

CONGENITAL TOXOPLASMOSIS

TOMOGRAPHY MR

TOMOGRAPHY: DIAGNOSIS?

PARVOVIRUS B19

IDEM

RDS: ETIOPATHOGENESIS

MULTIPLE MICROATELECTASIS

EARLY STAGE

RDS

MICROATELECTASIS

LATE STAGE

N.E.

N.E.

GMH: TOPOGRAPHY & MORPHOLOGY

HYDROPSFETALIS

ERYTHROBLASTOSIS