NORMAL AND ABNORMAL GROWTH IN CHILDREN Flashcards
growth
A complex biological phenomenon that starts at conception and is regulated by nutritional, hormonal and genetic factors
- reaches its peak in the second trimester of pregnancy
3 phases of somatic growth in children
infancy
childhood
puberty
most important determinants in the pahses
infancy - nutrition
childhood - growth hormone
puberty - sex steroids and growth hormone
growth patterns in each growth phase
infancy - deceleration of rapid foetal growth rate
childhood - slow decoration except mid childhood adrenal spurt
puberty - pubertal growth spurt
measuring growth
growth chart
BMI
growth chart
heigth
weight
head circumference
- on chart demonstrates percentile
BMI
- BMI = Mass (kg)/Height (m) squared
- proxy for body fat
- BMI for children is plotted on chart for gender and age
- online calculator for children and adults
BMI applications - identification of children above/below a healthy weight is the first step
- used in schools and hospitals
bone age
bone is degree of maturity of a Childs bone
how is bone age calculated
- uses standardised x rays
- estimate the maturity of each epiphyseal centre of the LH wrist to derive a score
- The age at which the score is on the 50th centile is the bone age of the individual
- can be used to predict a childs future height
causes of short stature
post natally most common nutrition
- consittutional
- intra uterine growth retardation
- systemic disease
- iatrogenic
- inherited
polygenetic disorder
- where there is an integration in the genetic pathway and the environmental defects
- eg cardiac disease, neural tube defects, chronic cancer
environmental factors
- drugs/chemicals (thalidomide, epanutin, warfarin, alcohol, fluoride)
- radiation
- infection
- metabolic
- hyperthermia
- vascular
- amniotic bands (Where the inner layer of the placenta is damaged during pregnancy, thin strands of bands become tangled around the body restricting blood flow)
birth defect types
single/isolated defects
multiple defects
single birth defect
- not the same as single gene defect
- only one part of the developing fetus has been affected
- common and mulit factoral
multiple birth defects
- associations
- sequences
- field complexes
- syndromes
association
Combination of at least 3 anomalies which are associated statistically but underlying mechanism not clear V Vertebral anomalies A Anal atresia C Cardiac defect T Tracheo – E Esophageal atresia R Radial and Renal anomalies L Limb abnormality
sequences
potters sequnce
Pierre robin sequence
potters sequence
- oligohydramnios
- insufficient amniotic fluid in uterus, fetus is squashed
can lead to
• squashed facies
• joint contractures
• bowed limbs
• lung hypoplasia
Pierre robin sequence
- early mandibular hypoplasia
- glossoptosis
- failed palatal closure
Management implications - breathing difficulties
- feeding difficulties
- cleft palate
- speech therapy
- dental care
- possible learning difficulties
field complexes
Insult to a localised part of an embryo resulting in abnormalities in adjacent structures of disparate embryonic origin
cause of Hemifacial microsomia
- Disturbance of blood supply to the first and second branchial arches in first 6 weeks of pregnancy leads to hemifacial microsomia
result of hemifacial microsomia
- localised underdevelopment of the face
- one side does not grow, affecting cheek lower jaw, muscles, nerevs ect
Trisomy 21 cause and effects
- genetic disorder due to 3rd copy of chromosome 21
- associated with growth delay, intellectual intelligence lowers, facial features
what is trisomy 21
down syndrome
facial features and intraorally of down sydrnome trisomy 21
Facial eatures - small neck - small ears - eyes slanted upwards Intraorally - class 3 mallocclusion - Large, fissured tongue - Periodontal disease - Hypodontia - Microdontia - Enamel defects - Class 3 malocclusion due to maxillary retrognathia
disorders of growth and dvleopemnt
- Osteogenesis imperfecta
- Down’s syndrome
- X-linked Vitamin D resistant rickets
- Cleidocranial dysostosis
- Achondroplasia
- Gigantism
- Acromegaly
dentinogenetic imperfecta
- Autosomal dominant condition
- disorder of dentine
- hypo mineralisation
- can be associated with OI?
- affects 1:8000
what can cause dentinogenetic imperfect
Mutations of dentine sialophosphoprotien
classifications of dentine disroders
dentinogenetis imperfecta
radicular dentine dysplasia
DI with osteogenesis imperfecta
pathology of the dentine disorders
dentinogenetis imperfecta (DSPP mutation affecting non collagenous protiens)
radicular dentine dysplasia (radicular pathology)
DI with osteogenesis imperfecta (collagenous proteins affected)
dental features of DI
1) Teeth have an amber, grey to purple discoloration
2) pulpal obliteration
3) relatively bulbous crowns
4) short narrow roots
5) enamel may be lost following tooth eruption, exposing dentine which wears rapidly
6) normal mantel dentine
7) rest of the dentine has non ordered dentinal tubules
8) affects primary >permanent
osteogenetic imperfecta symtoms
1) Bone fragility
- multiple fractures
- unstable vertebral column
2) Connective tissue disease with thin blood vessels (bruise easily)
3) poor muscle
4) loose joints
5) hearing loss
6) blue schelra
7) progressive hearing loss
8) dentinal changes
9) Bisphosphonates – risk of osteonecrosis
Hypophosphatemic rickets and
cuase
AKA X linked vitamin D resisitat rickets
- failure of distal tubular phosphate reabsorption resulting in lower serum phosphate, increase in alkaline phosphatase
symptoms of hypophosphatemic rickets
- skeletal deformities
- short stature
- bowing of legs
- frontal bossing
- large pulp chambers and elongated pulp horns
- abcesses in the abceses of caries
achondroplasia
dwarfism is a consequence
sporadic mutation
defect in FGFR gene
achrnodplasa symtoms
- shortened arms and legs(upper arms and thigs larger than forarms and lower legS)
- large head size and frontal bossing
- flattened nasal bridge
- crowding due to small jaws especially maxilla
cleidocranal dysostkosis and symtoms
Autosomal dominant pathway • Defective development of intramembranous ossification • Short in stature • Absent clavicles • Delayed closure of sutures Frontal and parietal bossing - hypoplastic maxilla and zygoma - delayed exfoliation of primary teeth - multiple supernumeraries - multiple unerupted teeth and associated cysts
gigantism
overproduction of pituriaty growth hormoen
usually due to ademona
gigantism after fusion of epiphyses
acromegaly
gigantism before fusion of epiphyses
gigantism of whole skeleton
acromegaly
continued froth at md condyle- gross prognathism
- macroglossia
- spacing of dentition
- thickened facial soft tissue
- growth of hands and points
- joint pain
- headaches
- problems with vision
