growth disturbances knut dahl-jørgensen unit for endocrinology and diabetes pediatric dept....

Growth disturbances

Knut Dahl-Jørgensen

Unit for Endocrinology and Diabetes Pediatric Dept.

Ullevål University Hospital

Simple guidelines by short stature

• Examined by the doctor:– All children below 2.5 height percentile– All children decreasing more than two centile chanals

• Refer to Pediatric Dept. by increasing deviation• Enclose by referal :

– Family history (growth and puberty)– History of birth, development and diseases– Growth chart– Eventually Bone age and laboratory test results

Differential diagnoses by short stature

Constitutional Growth DelayFamily Short StatureCombination of the previousNutritional

HypocaloricChronic inflamatory bowel diseaseMalabsorption , Coeliac disease

EndocrineHypothyroidismGrowth Hormone DeficiencyHypopituitarismExcessive cortisolPrecosious puberty

Chromosome defectsTurner Syndrome

Maternal deprivation

Low birth weightSmall for gestagional age

Prematurity

Fetal alcohol syndrome

Bone development disordersRickets

Sceletal dysplasias

MetabolicKidney failure

Hypoxic, Cardiac

Liver diseases

Inborn errors of metabolism

SyndromesNoonans

Aarskog

Routine examinations 1

Family history growth and puberty, growth treatment, syndromes

NeonatalGetational age, birth weight, birth length, forceps,

Hypoglycemia, hyperbilirubinemia, mikropenis

Gatrointestinal symptoms

Neurological symptoms

Psycosocial problems

Routine examinations 2

• Physical examination– Height

– Weight

– Growth velocity (cm per year)

– Tanner stages

– Teticular volume

– Dysmorphology (evt. sitting height, arm span)

– Blood pressure

– General physical examination

– Neurological examination

Routine examinations 3

Bone age and final height prediction

Clinical chemistry– TSH and free thyroxin

– GH, IGF-1, IGF-BP3

– At puberty: LH, FSH, estradiol or testosteron

– Coeliac screening

– Hemoglobin, ferritin, CRP

Chromosomes (girls)Evt. Metabolic screen, liver, kidney, bone (PTH, Vit.D)

Prediction of final height

Bone age estimation: Greulich & Pyle Atlas

Prediction: Bailly and Pinneau Tables

Causion ! Variability in bone age estimation by different radiologists

Great SD in reference material

Total variability: Young child + 5 cm, Late puberty + 3 cm

If unpredicted early puberty: Height prediction will decrease.

Other method: Tanner Whitehouse

Normal growth patterns

Normal early puberty

Normal late puberty

Familial (genetic) short stature

Constitutional delay of growth and puberty

Obesity

Pathological growth patterns

Growth hormone deficiency

Clinical appearancePuppy child

Hypoplastic midface ?

Evt. Hypoglycemia, hyperbilirubinemia, mikropenis

AuxologyDrop in height percentile

Growth velocity (cm per year) < 10 perc.

Delayed Bone Age

Causes of Growth hormone deficiency

CNS malformations (midline defects)

Hydrocephalus

CNS injuries (birth, forceps)

Meningitis, brain edema

Congenital infections

Hypothalamic or hypophyseal tumors

Cranial radiation

Congenital, genetic

Indications for Growth hormone treatment

Main indicationGrowth Hormone Deficiency

Other indications:Turner Syndrome

Kidney failure

Prader-Willi Syndrome

Small for gestagional age without catch-up growth ?

Testing Growth hormone secretion

Physiological testsOne random samplePhysical activityContinuous overnight samplingContinuous 24 hours sampling

Stimulation tests (allways two tests)Insulin iv (hypoglycemia)Arginine ivGlucagone iv or imClonidine (oral)GHRH

IGF-1, IGF-BP3

Growth hormone treatment

Daily subcutaneous injections

Injection pens

Disposable prefilled syringes

Autoinjection systems

Dosage: 0.033 mg/kg/day (0.1 U/kg/day)