Growth disturbances

Knut Dahl-JørgensenUnit 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 agePrematurityFetal alcohol syndrome

Bone development disordersRicketsSceletal dysplasias

MetabolicKidney failureHypoxic, CardiacLiver diseasesInborn errors of metabolism

SyndromesNoonansAarskog

Routine examinations 1

Family history growth and puberty, growth treatment, syndromes

NeonatalGetational age, birth weight, birth length, forceps, Hypoglycemia, hyperbilirubinemia, mikropenis

Gatrointestinal symptomsNeurological symptomsPsycosocial 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 predictionClinical 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 radiologistsGreat SD in reference materialTotal variability: Young child + 5 cm, Late puberty + 3 cmIf unpredicted early puberty: Height prediction will decrease.

Other method: Tanner Whitehouse

Normal growth patterns

Normal early pubertyNormal late pubertyFamilial (genetic) short statureConstitutional delay of growth and pubertyObesity

Pathological growth patterns

Growth hormone deficiency

Clinical appearancePuppy childHypoplastic midface ?Evt. Hypoglycemia, hyperbilirubinemia, mikropenis

AuxologyDrop in height percentileGrowth velocity (cm per year) < 10 perc.Delayed Bone Age

Causes of Growth hormone deficiency

CNS malformations (midline defects)HydrocephalusCNS injuries (birth, forceps)Meningitis, brain edemaCongenital infectionsHypothalamic or hypophyseal tumors Cranial radiationCongenital, genetic

Indications for Growth hormone treatment

Main indicationGrowth Hormone Deficiency

Other indications:Turner SyndromeKidney failurePrader-Willi SyndromeSmall 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 pensDisposable prefilled syringesAutoinjection systems

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