2. GROWTH & GROWTH DISORDERS OLEH Dr. H. Hakimi SpAK Dr. Melda Deliana SpAK Dr. Siska Mayasari Lubis SpA Pediatric Endocrinology USU/ADAM MALIK HOSPITAL

3. Aim Understand stature and growth Growth assessment through growth chart Able to refer child with growth problems

4. Control of Growth

5. HYPOTHALAMUS PITUITARY Liver TARGET TISSUE IGF-1 GH-R IGF-R IGFBP GHBP GH-R GH GH RH SOMATOSTATIN +- PARACRINE AUTOCRINE ENDOCRINE IGF-1 IGFBP

6. PREREQUISITE KNOWLEDGE Growth Chart A chart that reflects the height and weight of a certain population which is specific for race, age, and sex. The curves represent the approximate normal growth patterns of the targeted population Most normal children growth patterns are parallel to one of the lines in the curve Weight assessment is important in determining etiologies of some growth failures

7. PREREQUISITE KNOWLEDGE Heights of normal group of children tend to follow a normal distribution  average height is at the 50th percentile Height less than 2SD (3rd percentile) or more than 2SD (97 th percentile) considered abnormal

8. 97 50 3

9. PREREQUISITE KNOWLEDGE Height velocity is increase of height between 2 measurements (preferably 6 months interval) Chronological age age according to birth date. Height age is the appropriate age for the height observed. Obtained by drawing a horizontal line from the observed height to cross the 50 th percentile of the growth chart. At that cutoff point draw a vertical line to see the age.

10. PREREQUISITE KNOWLEDGE Potential genetic height: The range of adult height that should be achieved the child regarding to the biological parents final height. Boys = (F h + 13) + M h  8.5 cm 2 Girls = (F h - 13) + M h  8.5 cm 2

11. 97 50 3 HACA Potential Genetic Height

12. PREREQUISITE KNOWLEDGE Bone Age: is the apparent age of the epiphyseal centers on roentgenograms as compared with normals. Bone age reflects the biological functioning of the body. Usually approximates height age. Useful in predicting final height (if bone age >6 years old) Methods GP, TWH, RWT

13. Infant Childhood Puberty Cm/ year GHSex steroid Growth Velocity Curve

14. Anthropometrics General –Height –Weight –Head circumference Body proportion –Sitting height –Arm span

15. Genetic factors Catch-up & catch down during the infant phase of growth Strong correlation between child’s height (older than 2 years) with mid parental height  shows PGH. If child height less than 2.5 SD of MPH high probability of growth disorders Potential genetic height constitutional delay of growth & puberty has a family history of delayed puberty and/or growth

16. Potential Genetic Height CanalizationCatch-down INFANT PERIOD 0123 year

17. PREREQUISITTE KNOWLEDGE Define –Normal growth –Abnormal growth Normal growth is indicated by heights that fall within 2 standard deviation of the mean and form a curve that parallels the 3rd, 50 th, or 97 th percentile on the growth chart Abnormal growth is growth curve that does not parallel the 3rd, 50 th, or 97 th percentile on the growth chart

18. PREREQUISITTE KNOWLEDGE Difference of –Growth –Size –Catch up growth Growth is a process of changes in anthropometrics measurements with time Size indicates height and weight at a point of time. For height it is identical to stature. Normalization of growth after a period of abnormal growth

19. Growth parameters 0-2 years –Length increases 50% during 1 st year & another 50% of the 1 st year growth during 2 nd year At 2 years old is + 50% of final height –Weight doubles birth weight at 4 – 5 months of age and triples birth weight at 1 year

20. Growth parameters Prepubertal years –Height increase 6 – 8 cm per year. Slight acceleration at 6-8 years old (mid child spurt) normal slowing of growth just before puberty Enters puberty with + 85% of final height –Weight doubles birth weight at 4 – 5 months of age and triples birth weight at 1 year

21. Growth parameters Pubertal years –Accelerated growth = growth spurt –Weight

22. Rules of Five’s for normal growth rates in children B irth 1yr 4yr 8yr 12yr Length (cm) 50 75 100 125 150 Growth velocity 25 8 6 6 (cm/yr)

23. Catch-up Growth A self-correcting response that restores an individual to his growth channel when favorable nutrition conditions are restored Complete catch-up growth is possible if adequate nutrition is available and if epiphyseal fusion has not occurred Skeletal maturity in inversely related to the likelihood of complete catch-up growth

24. Catch-up Growth Occurs Rapidly Catch-up weight gain in wasted children occurs 20 times the normal rate Catch-up height in stunted children occurs 3 times the normal growth rate A child who is both wasted and stunted will gain weight rapidly until his weight of height is appropriate, then growth will slow

25. SHORT STATURE YES PATHOLOGICPATHOLOGIC NORMAL GROWTH VELOCITY? NO NORMAL VARIANT BODY PROPORTION? DYSMORPHISMDYSMORPHISM W/H INDEX?

26. SHORT STATURE PATHOLOGICPATHOLOGIC PROPORTIONALPROPORTIONAL W/H  ENDOKRIN GH DEFICIENCYGH DEFICIENCY HYPOTHYROIDHYPOTHYROID CORTISOL EXCESSCORTISOL EXCESS PSEUDOHYPOPARATHYR OIDPSEUDOHYPOPARATHYR OIDENDOKRIN GH DEFICIENCYGH DEFICIENCY HYPOTHYROIDHYPOTHYROID CORTISOL EXCESSCORTISOL EXCESS PSEUDOHYPOPARATHYR OIDPSEUDOHYPOPARATHYR OID W/H  MALNUTRITIONMALNUTRITION CHRONIC INFECTIONCHRONIC INFECTION CHRONIC DISEASE (ORGANIC)CHRONIC DISEASE (ORGANIC) PSYCHOSOCIALPSYCHOSOCIAL IUGRIUGR MALNUTRITIONMALNUTRITION CHRONIC INFECTIONCHRONIC INFECTION CHRONIC DISEASE (ORGANIC)CHRONIC DISEASE (ORGANIC) PSYCHOSOCIALPSYCHOSOCIAL IUGRIUGR DYSMORPHICDYSMORPHIC DYSPROPORTIONATEDYSPROPORTIONATE

27. SHORT STATURE PATHOLOGICPATHOLOGIC DYSMORPHICDYSMORPHIC DYSPROPORTIONATEDYSPROPORTIONATE SKELETAL DYSPLASIASKELETAL DYSPLASIA A/HYPO CHONDROPLASIA METABOLIC DISEASESMETABOLIC DISEASESRICKETS SPINAL DISORDERSSPINAL DISORDERS CRANIOSPINAL IRRADIATION SPONDYLODYSPLASIA SKELETAL DYSPLASIASKELETAL DYSPLASIA A/HYPO CHONDROPLASIA METABOLIC DISEASESMETABOLIC DISEASESRICKETS SPINAL DISORDERSSPINAL DISORDERS CRANIOSPINAL IRRADIATION SPONDYLODYSPLASIA CHROMOSOMECHROMOSOME TRISOMI 21TRISOMI 21 TURNER SYNDROME SYNDROMES (  IUGR)SYNDROMES (  IUGR) FETAL ALCOHOL, RUSSELL- SILVER, PRADER-WILLI, NOONAN, SECKEL, de LANGE, LARON, COCKAYNE dll CHROMOSOMECHROMOSOME TRISOMI 21TRISOMI 21 TURNER SYNDROME SYNDROMES (  IUGR)SYNDROMES (  IUGR) FETAL ALCOHOL, RUSSELL- SILVER, PRADER-WILLI, NOONAN, SECKEL, de LANGE, LARON, COCKAYNE dll

28. SHORT STATURE NORMAL VARIANT FAMILIAL SS CONSTITUTIONAL DELAY OF GROWTH AND PUBERTY  BA = CA  FINAL HEIGHT < 3 RD PERCENTILE  APPROPRIATE WITH PGH  BA = CA  FINAL HEIGHT < 3 RD PERCENTILE  APPROPRIATE WITH PGH  BA < CA  FINAL HEIGHT =NORMAL  APPROPRIATE WITH PGH  POSITIVE FAMILY HISTORY OF CDGP  BA < CA  FINAL HEIGHT =NORMAL  APPROPRIATE WITH PGH  POSITIVE FAMILY HISTORY OF CDGP

29. Familial Short Stature Constitutional Delay of Growth & Puberty

30. Stature and Growth Which child needs more attention?

31. Quiz Data of a boy –Age 7 yrs and 4 mos : 110 cm; BA 5 yrs –Age 8 yrs : 114 cm; BA 6 yrs –Age 9 yrs 6 mos : 122 cm; BA 7 yrs Father 172 cm, Mother 166 cm Menarche (mother) 15 yrs WHAT IS YOUR ASSESSMENT?

32. GROWTH CHART  STATURE  Patient 1 ()  Patient 1 (  )  Patient 2 ()  Patient 2 (  )  GROWTH  NORMAL ()  NORMAL (  )  ABNORMAL ()  ABNORMAL (  )  CATCH UP ()  CATCH UP (  )     

33. Penutup Mengapa Tinggi Akhir anak dengan CDGP dapat mencapai normal sedangkan pada FSS tidak? Hormon apa yang menyebabkan terjadinya penutupan epifisis?