Approach to short stature DR Arshad .ppt

Approach to short stature DR Arshad .ppt

• 1.
  DR. ARSHAD IQBAL (PGRPEDIATRICS)
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  Definition:  Height below3rd centile or less than 2 standard deviation below the median height for that age & sex according to the population standard. OR  Growth velocity consistently below 25th percentile or less than 5 cm / year.
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  Growth Chart Males Age (y) 30 34 38 42 46 50 54 58 62 66 70 74 78 H e ig h t (in ) H e ig h t (c m ) 24 6 8 10 12 14 16 18 20 70 80 90 100 110 120 130 140 150 160 170 180 190 200 0 +2 +1 -1 -2 -2.0 SD (2.3 percentile) Generally accepted definition of normal range
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  Growth Physiology Growth Environment Hormones Genetic factors Dietaryfactors •Growth hormone •Thyroid hormone •Gonadotrophins
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  Assessment of HeightVelocity YEAR INCREMENT (in cms) 1 25 2 10 3,4 7 5,6 6 7 – Puberty 5 Mid - puberty 9 – 10.3
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  CLASSIFICATION OF SHORTSTATURE Normal Variants – 1. Constitutional growth delay 2. Genetic/ Familial Short status Pathological – 1. Malnutrition 2. Endocrinal causes 3. Chromosomal defects 4. Genetic Disorders 5. Skeletal Dysplasia 6. Metabolic causes 7. Chronic diseases 8. Chronic drug intake
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  NORMAL VARIANT SHORTSTATURE
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  Short Short Stature Stature Proportionate Proportionate Normal Variants • Familial •Constitutional delay in growth Prenatal causes • IUGR • Intrauterine infections • Genetic Disorders Postnatal Causes • Nutritional dwarfism • Chronic visceral disease • Endocrine disorders • Emotional deprivation Short Limbed • Achandroplasia • Chondrodysplasias Short Trunk • Spondylo-epiphyseal dysplasia • MPS • Mucolipidoses • Caries spine • Hemivertebrae Disproportionate Disproportionate
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  C0nstitutional delaye ofgrowth in puberty  A family hx of growth in pubertal delay  Delayed bone age  Linear growth is below but parallel to lower percentile of growth  Growth harmones & thyroid studies are usualy normal  Normal final adult height  Its medical tx is not necessary but may be intitaed in adolecents experiencing psycosocial distress.
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   Boys withmore than two years of pubertal delay may benefit from short course of testosterone therap after 14yrs
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  APPROACH TO SHORTSTATURE Patient is evaluated in following four steps:-  History  Examination  Investigations  Management
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  History Antenatal pre-eclampsia, hypertension maternal historyof smoking, alcohol, infections Birth gestational age birth weight and length mode of delivery Apgar score neonatal complication Nutrition pattern of growth from birth Social Maternal and child relationship Family History short stature age of onset of puberty in family members diseases in the family. Past history Medical ,surgical and drug intake
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  Examination  Observation  Lookfor  Dismorphic facies  Nutritional status  Skin colour  Skeletal dysplasias
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  CLINICAL ASSESSMENT 1. Accurateheight measurement 2. Assessment of height velocity 3. Comparison with population norms 4. Comparison with child’s own genetic potential 5. Assessment of body proportion 6. Sexual maturity rating 7. Bone age
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  Assessment of Childwith Short Stature Accurate height measurement  Below 2 yrs- supine length with infantometer.  For older children - harpenden Stadiometer
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  Assessment of HeightVelocity YEAR INCREMENT (in cms) 1 25 2 10 3,4 7 5,6 6 7 – Puberty 5 Mid - puberty 9 – 10.3
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  Assessment of BodyProportion Proportionality is assessed by – Upper segment : Lower segment Ratio Comparison of arm span with height Based on this, short stature can be Normally US : LS Ratio – Birth: 1.7 3 years: 1.3 7 years:1 Adults: 0.9 Proportionate Disproportionate
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  Comparison with PopulationNorms  The height should be plotted on appropriate growth charts.  Any child who falls behind in growth across major percentiles in the chart should be evaluated.  Calculate mid-parental height.
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  Comparison with child’sown genetic potential  Mid Parent Height (MPH) gives an estimate of child’s genetically determined potential.  MPH for Boys = (Mother’s Height +13 cm) + Father’s Height / 2  MPH for Girls = Mother’s Height + (Father’s Height - 13cm) / 2
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  PERFORM SPECIFIC MANOEVOURS Screen for asymmetry of limbs  Screen for carrying angle  Screen of shortening of segments of limbs  Screen for hands  Inspection from front  Inspection from back
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  Pointers to etiologyof short stature Pointer Etiology Midline defects, micropenis, Frontal bossing, depressed nasal bridge, crowded teeth, GH deficiency Rickets Renal failure, RTA, malabsorption Pallor Renal failure, malabsorption, nutritional anemia Malnutrition PEM, malabsorption, celiac disease, cystic fibrosis Obesity Hypothyroidism, Cushing syndrome, Prader Willi syndrome Metacarpal shortening Turner syndrome, pseudohypoparathyroidism Cardiac murmur Congenital heart disease, Turner syndrome Mental retardation Hypothyroidism, Down/ Turner syndrome, pseudohypoparathyroidism
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  Stepwise Investigations
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  MANAGEMENT  Counselling ofparents (for physiological causes)  Dietary advice (Undernutrition, Celiac disease, RTA )  Limb lengthening procedures (Skeletal dysplasias )  Levothyroxine (In Hypothyroidism)  GH s/c injections (GH deficiency, Turner syndrome, SGA, CRF prior to transplant)
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  Indications of growth harmone FDA approved eight indications  GH deficiency  Turner syn  CH renal failure  SGA  Idiopathic short stature  Noonan syn  Prader willi syn
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  CRITERIA FOR STOPPINGGH TX  Growth rate is <1 inch / year  Bone age >14 yrs in girls and >16yrs in boys  Decesion by the patients that he or she is tall enough
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  Feature Familial ShortStature Constitutional Short Stature 1) Sex Both equally affected More common in boys 2) Length at Birth Normal( crosses percentile downwards by 3yrs) Normal (starts falling <5th centile in 1st 3yrs of life) 3) Family History Of short stature Of delayed puberty 4) Parents Stature Short (one or both) Average 5) Height Velocity Normal Normal 6) Puberty Normal Delayed 7) Bone Age & Chronological Age BA = CA > Height Age CA > BA = Height Age 8) Final Height Short, but normal for target height Normal due to normal growth in pre pubertal years.
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  Take Home Message •Take height by proper method and plot it on appropriate growth chart. • Use Growth Charts appropriately. • Every child must have proper growth monitoring so as to know whether the child is on his proper road to growth. • Any child with short stature is not always familial and should be evaluated completely.
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  THANK YOU