BASIM ALZOUBIThe worllds tallest man, Sultan Kosen (R) standing at 8 ft 1 in (246.5 m) !oses with shortest man in the world "e #ing!ing standing at 2 ft 5.$% in (%4.61 m) during ele&rate the launh of the 'uinness (orld Reords li)e roadshow in *stan&ul, on +anuar, 14, 2-1-. Kosen, 2%, was awarded the title of the world.s tallest man last Se!tem&er, sur!assing /hina.s 0ao 1ishun who stood in at a mere %2foot23. Kosen also holds the reords for the world.s largest hands and feet..Tur4e, +anuar, 2--3DefinitionHeight below 5th percentileHeight below 3d percentileMore than 2 SDs below the mean.Below-2.25 SDGrowth Velocit S"5RT ST6T7R8Growth VelocityA single point on a growth chart often does not define a worrisome growth pattern. Previous growth data should be plotted whenever available. Any suggestion of growth deceleration should be reviewed. Standard growth charts do not incorporatepubertal stage.Growth Chart for turner Syndrome compared to normalfemale growthCauses of Short StatureNormal Constitutional growth delay Genetic/familial short stature Combined constitutional growthdelay and familial short staturePathologicalNutritional Micronutrient defciency Zinc defciency Iron defciency Macronutrient defciency: decreased intake Hypocaloric diet Kwashiorkor Anorexia nervosa and other eating disorders Nutritional dwarfng (fear of obesity, fear ofhypercholesterolemia) Macronutrient defciency: decreased absorptionInfammatory bowel diseaseCeliac diseaseCystic fbrosisMalabsorptionEndocrine Hypothyroidism Isolated GH defciency Neurosecretory GH defciencyGH insensitivity (primary insulin-like growth factor defciency) HypopituitarismCushings syndromePrecocious puberty Chromosome defects!"rner sndrome#oonan SndromDown sndrome$rader%&illi sndromeIUGRSporadic'haracteristic appearance9("ssell%Sil)er sndromeDefects in bone DevelopmentAchondroplasia,hypochondroplasiaOther skeletal disordersMetabolicMucopolysaccharidosesOther storage disordersChronic diseaseChronic renal diseaseChronic liver diseaseCongenital heart disease (especially cyanoticconditions)Pulmonary (cystic fbrosis, bronchial asthma)Poorly controlled diabetes mellitusChronic infections (including human HIV, tuberculosis) associated with birth defects or mental retardationPsychosocial DeprivationChronic drug intakeGlucocorticoidsestrogens or androgensDIAGNOSIS OF SHORT STATUREMeasurementsIn a study of an academic pediatric clinic, 35% ofwell-child encounters failed to plot growth measurementsand/or document a growth abnormality. Observation of 44 pediatric and 11 family practices throughout the United States revealed that only 30% of measurements were accurate (within 0.5 cm); measurements difered by an average of 1.3 cm from measurements of the same children by study staf.Standing height is always shorterthan supine length, so plotting a standing height on the length chart creates an artifcial growth decelerationmid-parental target height (in cm):For males :(Mother +father height + 13 cm) divided by 2)For females :Mother +father height +13 )divided by 2Skeletal Maturation and Predicted Final Height1-Bayley and Pinneau method. bone age HeightThis correlation is more accurate after nine years of age.2-The TannerWhitehouse (TW) methodbone ageheightparental heightsin girls, the occurrence of menarche.Chronological appearance of osseous centersbirth to 5 years. Source: From Wilkins, Lawson. Diagnosis and Treatment of Endocrine Disorders in Childhood and Adolescence. Springfeld: Illinois, 1966Body ProportionsThe upper to lower body ratio starts at 1.7 atbirth, but as the legs grow, the ratio drops to 1.0 by 10 years of age. If the growth plates fuse early, as inprecocious puberty, the proportions remain childlike, with short limbs compared to the trunk. On the other hand, if growth is prolonged as in hypogonadism, a long-limbed shape results . The lower body segment is measured by measuring the distance between the upper border of the symphysis pubis and the foor in a patient who is standing.Preferably, the sitting height can be measuredto represent the upper segment. The trunk may be disproportionately shorter due to scoliosis, or spinal injury from total body irradiation.Arm span is another helpful measurement indetermining body proportions. The distance between the distal ends of both middle phalanges is measured to determine the arm span. Normally, the arm span is shorter than theheight in boys before age 10 to 11 years and girlsbefore 11 to 14 years, after which the arm span exceedsthe height. *#V+S!*G,!*-#SGeneral Screening TSH, FT4 general chemistries including !", creatinine liver function tests urinalysis complete blood count with differential blood gas sedimentation rate and #$reactive protein tissue transglutaminase antibody or antiendomyseal antibodysweat testing for cystic fibrosis % &aryotype ingirls bone age'ndocrine Thyroid function tests. A#TH and cortisol level in blood and urinewhen adrenal disease is suspected. '(amination of the eye grounds and visual fields )*+ scanif hypopituitarism is suspected .:anagement of short statureTreatment of the ause'"*';1 '"asal 0ridge, @ela,ed @entition, "igh2!ithed Eoie@ela,ed /losure 5f The 6nterior ;ontanel:iro!enis. @ela,ed #u&ert,GH defciencyRandom GH levels are useless except for the frst few months of life .Random IGF-I and IGFBP-3Provocative GH testing to directly assess GH secretory capacity.S"bstances "sed in growth hormone stim"lating tests/.*ns"lin2.'lonidine3.,rginine5.:e)o dopa5.Gl"cagonGH INSENSITIVITY autosomal recessive syndrome caused by defects oftheGH receptor. elevated serum concentrations of GH low serum concentrations of IGF-I, IGFBP3, and GHBP.HYPOTHYROIDISMThe growth velocity is slow and BA is delayed relative to CA. The risks of mental retardation and learning defcits are related directly to the length of delay in diagnosis. GLUCOCORTICOID EXCESSIatrogenicadrenal adenoma ACTH secreting pituitary adenoma. Growth velocity is slow, BA is delayed relative to the CA, and weight gain and elevated blood pressure typicallyare present. IDIOPATHIC SHORT STATURECriteria for Diagnosis of ISSHeight more than 2.0 SDS below the meanormal si!e for gestational age at birthormal bo"# $ro$ortionso e%i"en&e of &hroni& organi& "iseaseo $s#&hiatri& "isease or se%ere emotional "ist'rban&eormal foo" inta(eo e%i"en&e of en"o&rine "efi&ienSlow or normal tem$o of growthIn&l'"e" within this &onsens's "efinition are &hil"ren with &onstit'tionalgrowth "ela# an" those with familial short stat're.>oonan S@ela,ed !u&ert,@own2slanting or wide2set e,esFow2set or a&normall, sha!ed ears:ild mental retardationShort StatureSmall !enis7ndesended testiles7nusual hest sha!e (!etus e=a)atum)(e&&ed and short2a!!earing ne4FDA approved GH Treatment1. Growth hormone defciency 2.Turner syndrome,3. End-stage renal failure beforekidney transplantation4. Prader-Willi syndrome5.SGA6.idiopathic short stature. 7.Noonan Syndrome8.Adult with growth hormone defciency"ow *s '" 'i)enGSu&utaneous dail, '" administrationis urrentl, !referred, with the a)erage '"@ hildin the 7nited States reei)ing initial treatmentwith -.$ mgH4gHw4 di)ided into si= to se)en doses.ADVERSE EFFECTS OF hGH TREATMENTGrowth hormone treatment does not increase the risk for recurrence of brain tumors or leukemiaslipped capital femoral epiphysispseudotumor cerebriworsening of scoliosisgynecomastiaincrease in total body water during the frst 2 wk of treatmentDM ?$han% !ou