NORMAL AND ABNORMAL GROWTH IN CHILDREN

Question 1

growth

Answer 1

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

Question 2

3 phases of somatic growth in children

Answer 2

infancy
childhood
puberty

Question 3

most important determinants in the pahses

Answer 3

infancy - nutrition
childhood - growth hormone
puberty - sex steroids and growth hormone

Question 4

growth patterns in each growth phase

Answer 4

infancy - deceleration of rapid foetal growth rate
childhood - slow decoration except mid childhood adrenal spurt
puberty - pubertal growth spurt

Question 5

measuring growth

Answer 5

growth chart

BMI

Question 6

growth chart

Answer 6

heigth
weight
head circumference
- on chart demonstrates percentile

Question 7

BMI

Answer 7

• 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

Question 8

bone age

Answer 8

bone is degree of maturity of a Childs bone

Question 9

how is bone age calculated

Answer 9

• 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

Question 10

causes of short stature

Answer 10

post natally most common nutrition

• consittutional
• intra uterine growth retardation
• systemic disease
• iatrogenic
• inherited

Question 11

polygenetic disorder

Answer 11

• where there is an integration in the genetic pathway and the environmental defects
• eg cardiac disease, neural tube defects, chronic cancer

Question 12

environmental factors

Answer 12

• 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)

Question 13

birth defect types

Answer 13

single/isolated defects

multiple defects

Question 14

single birth defect

Answer 14

• not the same as single gene defect
• only one part of the developing fetus has been affected
• common and mulit factoral

Question 15

multiple birth defects

Answer 15

• associations
• sequences
• field complexes
• syndromes

Question 16

association

Answer 16

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

Question 17

sequences

Answer 17

potters sequnce

Pierre robin sequence

Question 18

potters sequence

Answer 18

• oligohydramnios
• insufficient amniotic fluid in uterus, fetus is squashed
  can lead to
  • squashed facies
  • joint contractures
  • bowed limbs
  • lung hypoplasia

Question 19

Pierre robin sequence

Answer 19

• early mandibular hypoplasia
• glossoptosis
• failed palatal closure
  Management implications
• breathing difficulties
• feeding difficulties
• cleft palate
• speech therapy
• dental care
• possible learning difficulties

Question 20

field complexes

Answer 20

Insult to a localised part of an embryo resulting in abnormalities in adjacent structures of disparate embryonic origin

Question 21

cause of Hemifacial microsomia

Answer 21

• Disturbance of blood supply to the first and second branchial arches in first 6 weeks of pregnancy leads to hemifacial microsomia

Question 22

result of hemifacial microsomia

Answer 22

• localised underdevelopment of the face

- one side does not grow, affecting cheek lower jaw, muscles, nerevs ect

Question 23

Trisomy 21 cause and effects

Answer 23

• genetic disorder due to 3rd copy of chromosome 21

- associated with growth delay, intellectual intelligence lowers, facial features

Question 24

what is trisomy 21

Answer 24

down syndrome

Question 25

facial features and intraorally of down sydrnome trisomy 21

Answer 25

``` 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 ```

Question 26

disorders of growth and dvleopemnt

Answer 26

* Osteogenesis imperfecta * Down’s syndrome * X-linked Vitamin D resistant rickets * Cleidocranial dysostosis * Achondroplasia * Gigantism * Acromegaly

Question 27

dentinogenetic imperfecta

Answer 27

- Autosomal dominant condition - disorder of dentine - hypo mineralisation - can be associated with OI? - affects 1:8000

Question 28

what can cause dentinogenetic imperfect

Answer 28

Mutations of dentine sialophosphoprotien

Question 29

classifications of dentine disroders

Answer 29

dentinogenetis imperfecta radicular dentine dysplasia DI with osteogenesis imperfecta

Question 30

pathology of the dentine disorders

Answer 30

dentinogenetis imperfecta (DSPP mutation affecting non collagenous protiens) radicular dentine dysplasia (radicular pathology) DI with osteogenesis imperfecta (collagenous proteins affected)

Question 31

dental features of DI

Answer 31

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

Question 32

osteogenetic imperfecta symtoms

Answer 32

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

Question 33

Hypophosphatemic rickets and | cuase

Answer 33

AKA X linked vitamin D resisitat rickets | - failure of distal tubular phosphate reabsorption resulting in lower serum phosphate, increase in alkaline phosphatase

Question 34

symptoms of hypophosphatemic rickets

Answer 34

- skeletal deformities - short stature - bowing of legs - frontal bossing - large pulp chambers and elongated pulp horns - abcesses in the abceses of caries

Question 35

achondroplasia

Answer 35

dwarfism is a consequence sporadic mutation defect in FGFR gene

Question 36

achrnodplasa symtoms

Answer 36

- 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

Question 37

cleidocranal dysostkosis and symtoms

Answer 37

``` 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 ```

Question 38

gigantism

Answer 38

overproduction of pituriaty growth hormoen | usually due to ademona

Question 39

gigantism after fusion of epiphyses

Answer 39

acromegaly

Question 40

gigantism before fusion of epiphyses

Answer 40

gigantism of whole skeleton

Question 41

acromegaly

Answer 41

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