Your SlideShare is downloading. ×
Shortstature
Upcoming SlideShare
Loading in...5
×

Thanks for flagging this SlideShare!

Oops! An error has occurred.

×

Saving this for later?

Get the SlideShare app to save on your phone or tablet. Read anywhere, anytime - even offline.

Text the download link to your phone

Standard text messaging rates apply

Shortstature

573
views

Published on


0 Comments
0 Likes
Statistics
Notes
  • Be the first to comment

  • Be the first to like this

No Downloads
Views
Total Views
573
On Slideshare
0
From Embeds
0
Number of Embeds
0
Actions
Shares
0
Downloads
35
Comments
0
Likes
0
Embeds 0
No embeds

Report content
Flagged as inappropriate Flag as inappropriate
Flag as inappropriate

Select your reason for flagging this presentation as inappropriate.

Cancel
No notes for slide

Transcript

  • 1. Short StatureShort Stature Morning ReportMorning Report November 2005November 2005
  • 2. Normal GrowthNormal Growth The most critical factor in evaluatingThe most critical factor in evaluating growth is determining thegrowth is determining the growth velocitygrowth velocity Observation of childObservation of child’’s height patterns height pattern ““crossingcrossing”” percentile linespercentile lines on a linearon a linear growth curve can be the simplest methodgrowth curve can be the simplest method of identifying an abnormal growth velocityof identifying an abnormal growth velocity Repeated measurements are importantRepeated measurements are important with at least 3 and preferably 6 months ofwith at least 3 and preferably 6 months of observation in betweenobservation in between
  • 3. Normal GrowthNormal Growth Prenatal growth:Prenatal growth: Factors influencing growth are uterine function and size,Factors influencing growth are uterine function and size, maternal nutrition, insulin, and IGF/BPmaternal nutrition, insulin, and IGF/BP Postnatal growth:Postnatal growth: Growth hormone and thyroid hormone play major roleGrowth hormone and thyroid hormone play major role Rapid linear growth velocity that declines progressively afterRapid linear growth velocity that declines progressively after birth to 3 yearsbirth to 3 years 25 cm/yr25 cm/yr 12 cm/yr12 cm/yr 8 cm/yr8 cm/yr Age 3 to onset of pubertyAge 3 to onset of puberty Growth hormone and thyroid hormone play major roleGrowth hormone and thyroid hormone play major role Linear growth at constant rate in both sexesLinear growth at constant rate in both sexes 4 to 7 cm/yr4 to 7 cm/yr An abnormal growth velocity after 3 years of age isAn abnormal growth velocity after 3 years of age is almost always abnormal, children find their curve by thenalmost always abnormal, children find their curve by then
  • 4. PubertyPuberty Sex steroids (testosterone, estrogen) act in concert withSex steroids (testosterone, estrogen) act in concert with GH, thyroid hormone, and nutrition = acceleration ofGH, thyroid hormone, and nutrition = acceleration of growth = pubertal growth spurtgrowth = pubertal growth spurt Spontaneous GH levels increase in response toSpontaneous GH levels increase in response to estrogen and androgenestrogen and androgen First sign of puberty in females (breast enlargement)First sign of puberty in females (breast enlargement) precedes first sign in males (testicular enlargement) by 6precedes first sign in males (testicular enlargement) by 6 monthsmonths Pubertal growth spurt in girls is 2 years earlier than boysPubertal growth spurt in girls is 2 years earlier than boys (stage 3 in girls(stage 3 in girls vsvs stage 4 in boys)stage 4 in boys) Peak height velocity is slower in girls (8.3cm/yr) than inPeak height velocity is slower in girls (8.3cm/yr) than in boys (9.5cm/yr)boys (9.5cm/yr) Average 13 cm height difference between the 2 sexesAverage 13 cm height difference between the 2 sexes
  • 5. Normal GrowthNormal Growth Absolute height is also importantAbsolute height is also important Population based definitions of short staturePopulation based definitions of short stature includeinclude Less than 5Less than 5thth percentilepercentile Shorter than 2 SD below the mean (2.5%ile)Shorter than 2 SD below the mean (2.5%ile) Biologic definition is obtained by analysis of theBiologic definition is obtained by analysis of the childchild’’s height in the context of the expecteds height in the context of the expected genetic potential conferred by the parents or thegenetic potential conferred by the parents or the midmid--parental height or target heightparental height or target height
  • 6. Target HeightTarget Height Boys:Boys: [father[father’’s height (cm) + (mothers height (cm) + (mother’’s height (cm)s height (cm) ++ 1313)] divided by 2 OR)] divided by 2 OR [father[father’’s height (in) + mothers height (in) + mother’’s height (in)s height (in) + 5+ 5 in]in] divided by 2divided by 2 Girls:Girls: [(father[(father’’s height (cm)s height (cm) –– 1313) + mother) + mother’’s heights height (cm)] divided by 2 OR(cm)] divided by 2 OR [father[father’’s height (in) + mothers height (in) + mother’’s height (in)s height (in) –– 55 in]in] divided by 2divided by 2 Target Height =Target Height = midparentalmidparental height +/height +/-- 2 SD2 SD
  • 7. Skeletal MaturationSkeletal Maturation Growth = increase in length of the bones + rate ofGrowth = increase in length of the bones + rate of skeletal maturationskeletal maturation Bone age radiographyBone age radiography Method of assessing skeletal maturation by examiningMethod of assessing skeletal maturation by examining epiphysealepiphyseal maturation of the hand/wristmaturation of the hand/wrist < 18 months: use< 18 months: use hemiskeletonhemiskeleton xx--ray due to immature hand/wristray due to immature hand/wrist growth platesgrowth plates Delayed bone age indicates that the short stature isDelayed bone age indicates that the short stature is partially reversiblepartially reversible because linear growth continuesbecause linear growth continues untiluntil epiphysealepiphyseal fusion is completefusion is complete A short child with nonA short child with non--delayed bone age is of muchdelayed bone age is of much greater concerngreater concern
  • 8. Body ProportionsBody Proportions UpperUpper--toto--lower body segment (U/L) ratiolower body segment (U/L) ratio Short stature: proportionate (involves trunk andShort stature: proportionate (involves trunk and lower extremities) or disproportionate (one morelower extremities) or disproportionate (one more than other)than other) Lower segment is measured by the distanceLower segment is measured by the distance between upper border of thebetween upper border of the symphysissymphysis pubispubis and the floor while standingand the floor while standing Upper segment is determined byUpper segment is determined by substractingsubstracting the lower segment from the standing heightthe lower segment from the standing height InIn achondroplasiaachondroplasia, the U/L ratio will be increased, the U/L ratio will be increased
  • 9. Short StatureShort Stature Normal VariantsNormal Variants Constitutional Growth DelayConstitutional Growth Delay Familial Short StatureFamilial Short Stature PathologicPathologic Decreased Weight for Height:Decreased Weight for Height: •• MalnutritionMalnutrition •• Chronic DiseaseChronic Disease •• Chromosomal abnormalitiesChromosomal abnormalities Increased Weight for Height:Increased Weight for Height: •• HypothyroidismHypothyroidism •• GlucocorticoidGlucocorticoid excessexcess •• GH deficiencyGH deficiency DisproportionateDisproportionate (U/L ratio(U/L ratio ↑↑oror↓↓)) •• SkeletalSkeletal dysplasiasdysplasias •• Clinical syndromesClinical syndromes
  • 10. Copyright ©2005 American Academy of Pediatrics Rose, S. R. et al. Pediatrics in Review 2005;26:410-420 Schematic demonstration of height and weight growth curves in various conditions associated with short stature
  • 11. Normal Variations in GrowthNormal Variations in Growth Familial Short StatureFamilial Short Stature Genetic or intrinsicGenetic or intrinsic Cross percentilesCross percentiles downward during first 3downward during first 3 yearsyears Eventually find theirEventually find their geneticgenetic--appropriate linearappropriate linear growth percentilegrowth percentile Normal bone ageNormal bone age Normal timing of pubertyNormal timing of puberty + Family History+ Family History Adult height: shortAdult height: short Constitutional DelayConstitutional Delay Slowing growth velocitySlowing growth velocity during first 3 years,during first 3 years, crosses percentilescrosses percentiles Normal growth velocityNormal growth velocity duringduring prepubertalprepubertal yearsyears Delayed bone ageDelayed bone age Delayed timing of pubertyDelayed timing of puberty ““Late BloomerLate Bloomer”” + Family History+ Family History Adult height: normalAdult height: normal
  • 12. www.mead johnson.com
  • 13. www.mead johnson.com
  • 14. Increased Weight for HeightIncreased Weight for Height Growth Hormone DeficiencyGrowth Hormone Deficiency HypothyriodismHypothyriodism GlucocorticoidGlucocorticoid excessexcess
  • 15. GHDGHD CongenitalCongenital PerinatalPerinatal asphyxiaasphyxia CNS malformation (CNS malformation (septosepto--opticoptic dysplasiadysplasia)) •• MRI shows interruption of pituitary stalk, small gland or emptyMRI shows interruption of pituitary stalk, small gland or empty sellasella •• Prolonged jaundice, hypoglycemia, FTT with normal growth velocitProlonged jaundice, hypoglycemia, FTT with normal growth velocityy •• Subsequent linear growth deceleration with normal weight seenSubsequent linear growth deceleration with normal weight seen after age 1after age 1 AcquiredAcquired IdiopathicIdiopathic •• Usually hypothalamic deficiency of GHRH, rather than pituitaryUsually hypothalamic deficiency of GHRH, rather than pituitary deficiency of GHdeficiency of GH Tumors (Tumors (craniopharyngiomacraniopharyngioma,, gliomaglioma,, germinomagerminoma)) Traumatic head injuryTraumatic head injury CNS infection or irradiationCNS infection or irradiation Surgical damage to pituitary or hypothalamusSurgical damage to pituitary or hypothalamus
  • 16. GHDGHD Short statureShort stature Slow growth velocitySlow growth velocity Facial phenotype of younger childFacial phenotype of younger child Delayed bone ageDelayed bone age Low IGFLow IGF--1 and IGFBP3 values1 and IGFBP3 values Abnormal stimulation testAbnormal stimulation test Peak stimulated GH concentration < 10Peak stimulated GH concentration < 10 ng/mLng/mL inin response to 2 GHresponse to 2 GH stimstim tests (tests (argininearginine, insulin,, insulin, clonidineclonidine,, glucagonglucagon) or < 18) or < 18 ng/mLng/mL in response to ain response to a combinedcombined argininearginine--GHRHGHRH stimstim testtest Must evaluate other pituitary hormones!Must evaluate other pituitary hormones!
  • 17. http://www.mead johnson.com/pro fessional/newsle tters/v4n2pp/Fig- 10.gif
  • 18. GHDGHD Metabolic effects include increases in body fatMetabolic effects include increases in body fat and cholesterol; reductions in lean body mass,and cholesterol; reductions in lean body mass, bone mineral density, cardiac function, andbone mineral density, cardiac function, and staminastamina GH currently approved by FDA for:GH currently approved by FDA for: GH DeficiencyGH Deficiency Turner syndromeTurner syndrome Renal insufficiencyRenal insufficiency PraderPrader WilliWilli syndromesyndrome Normal children with height < 2.4 SDNormal children with height < 2.4 SD SGA who have not reached 5th %SGA who have not reached 5th %ileile by age 2by age 2
  • 19. HypothyroidismHypothyroidism Congenital (untreated)Congenital (untreated) Slow growth velocitySlow growth velocity Delayed bone ageDelayed bone age ConstipationConstipation Mentally delayed unless detected before age 2Mentally delayed unless detected before age 2--3 months3 months Acquired (untreated)Acquired (untreated) Delayed growthDelayed growth ConstipationConstipation Dry skinDry skin AsymptomaticAsymptomatic Normal IQ (if developed after age 2 years)Normal IQ (if developed after age 2 years) Regardless of symptoms, all children with significantRegardless of symptoms, all children with significant short stature should be screened for hypothyroidism!short stature should be screened for hypothyroidism!
  • 20. www.mead johnson.com
  • 21. GlucocorticoidGlucocorticoid ExcessExcess IatrogenicIatrogenic Pharmacologic treatmentPharmacologic treatment Endogenous steroid productionEndogenous steroid production Adrenal adenomaAdrenal adenoma ACTH producing pituitary adenomaACTH producing pituitary adenoma Slow growth velocitySlow growth velocity Delayed bone ageDelayed bone age Weight gain and elevated blood pressureWeight gain and elevated blood pressure Weight gain in obesity usually has increasedWeight gain in obesity usually has increased growth velocitygrowth velocity
  • 22. Decreased Weight for HeightDecreased Weight for Height Systemic illnessSystemic illness MalnutritionMalnutrition MalabsorptionMalabsorption Inflammatory bowel diseaseInflammatory bowel disease Social neglect (psychosocial dwarfism)Social neglect (psychosocial dwarfism)
  • 23. Decreased Weight for HeightDecreased Weight for Height Weight loss or decline in weight gain isWeight loss or decline in weight gain is more pronounced than the decline in linearmore pronounced than the decline in linear growthgrowth Delayed sexual developmentDelayed sexual development Delayed skeletal maturation proportionateDelayed skeletal maturation proportionate to the severity of the underlyingto the severity of the underlying malnutrition or systemic disease activitymalnutrition or systemic disease activity
  • 24. MalnutritionMalnutrition Most common cause of short stature in the worldMost common cause of short stature in the world Can be :Can be : SingleSingle--nutrient malnutrition (calcium, vitamin)nutrient malnutrition (calcium, vitamin) Disinterest in eatingDisinterest in eating Anorexia nervosaAnorexia nervosa With restoration of adequate nutrition, growthWith restoration of adequate nutrition, growth usually accelerates (catchusually accelerates (catch--up)up) Adult height may be compromised if malnutritionAdult height may be compromised if malnutrition is profoundis profound
  • 25. Systemic DiseaseSystemic Disease Growth delay depends on severityGrowth delay depends on severity Inflammatory bowel disease and CeliacInflammatory bowel disease and Celiac disease can be silent and cause growthdisease can be silent and cause growth delay before onset of GI symptomsdelay before onset of GI symptoms Renal tubular acidosis orRenal tubular acidosis or nephrogenicnephrogenic DIDI may present from birth with FTTmay present from birth with FTT Growth delay associated with otherGrowth delay associated with other chronic diseases are usually evidentchronic diseases are usually evident clinically (cardiac, pulmonary)clinically (cardiac, pulmonary)
  • 26. Social NeglectSocial Neglect Usually nutritional and related directly to inadequateUsually nutritional and related directly to inadequate caloric intakecaloric intake Typically present with FTTTypically present with FTT Occasionally, a neglected child may present in the sameOccasionally, a neglected child may present in the same way as GHD, known as psychosocial dwarfismway as GHD, known as psychosocial dwarfism Psychosocial dwarfismPsychosocial dwarfism Disturbed parentDisturbed parent--child relationshipchild relationship Characteristic bizarre behavior pattern (aggressive food/waterCharacteristic bizarre behavior pattern (aggressive food/water seeking habits)seeking habits) Slow growth velocitySlow growth velocity Delayed bone ageDelayed bone age Abnormal pituitary responses toAbnormal pituitary responses to stimstim testingtesting Resume normal growth when removed from environmentResume normal growth when removed from environment
  • 27. DisproportionateDisproportionate ShorteningShortening SkeletalSkeletal dysplasiasdysplasias Abnormal U/L segment ratio for ageAbnormal U/L segment ratio for age Relative shortening of limbs compared toRelative shortening of limbs compared to spine (spine (achondroplasiaachondroplasia)) Relative shortening of spine compared toRelative shortening of spine compared to limbs (limbs (spondylodysplasiaspondylodysplasia)) Some may affect spine and limbs equallySome may affect spine and limbs equally Normal bone ageNormal bone age Diagnosis made by radiography or geneticsDiagnosis made by radiography or genetics
  • 28. Clinical SyndromesClinical Syndromes Turner syndromeTurner syndrome PraderPrader WilliWilli syndromesyndrome Down syndromeDown syndrome Evaluate forEvaluate for dysmorphicdysmorphic features for cluesfeatures for clues to diagnosisto diagnosis
  • 29. Turner SyndromeTurner Syndrome Short stature may be only clinical manifestationShort stature may be only clinical manifestation KaryotypeKaryotype should be considered in any short girl,should be considered in any short girl, especially if puberty is delayedespecially if puberty is delayed SHOSHOXX gene is essential for normal growth and Turnergene is essential for normal growth and Turner patients have only half the normal dosepatients have only half the normal dose May have normal appearance or have features such asMay have normal appearance or have features such as webbed neck, short metacarpals, shieldwebbed neck, short metacarpals, shield--shaped chest,shaped chest, hyperconvexhyperconvex fingerfinger-- and toenails,and toenails, cubituscubitus valgusvalgus (wide(wide carrying angle of arms)carrying angle of arms) GonadalGonadal dysgenesisdysgenesis results in an incomplete or absentresults in an incomplete or absent puberty, no pubertal growth spurtpuberty, no pubertal growth spurt Bone age is minimally delayedBone age is minimally delayed
  • 30. http://www.magi cfoundation.org/ downloads/turn ersgrowthchart2 19yearsmed545 .pdf
  • 31. ReferencesReferences Rose, S. R. et al. Pediatrics in Review 2005;26:410-420 ZitelliZitelli et al. Atlas of Pediatric Physicalet al. Atlas of Pediatric Physical Diagnosis.Diagnosis. ww.meadjohnson.comww.meadjohnson.com http://www.magicfoundation.org