Idiopathic short stature

Idiopathic short stature

DiseaseDisorder infobox
Name = Idiopathic short stature
ICD10 =
ICD9 = ICD9|783.43

Idiopathic short stature (ISS) refers to extreme short stature that does not have a diagnostic explanation ("idiopathic" designates a condition that is unexplained or not understood) after an ordinary growth evaluation. The term has been in use since at least 1975cite journal |author=Sizonenko PC, Rabinovitch A, Schneider P, Paunier L, Wollheim CB, Zahnd G |title=Plasma growth hormone, insulin, and glucagon responses to arginine infusion in children and adolescents with idiopathic short stature, isolated growth hormone deficiency, panhypopituitarism, and anorexia nervosa |journal=Pediatr. Res. |volume=9 |issue=9 |pages=733–8 |year=1975 |pmid=1105371 |doi=10.1203/00006450-197509000-00010] without a precise percentile or statistical definition of "extreme".

Definition

In 2003 Eli Lilly and Company offered a more precise definition of ISS when the pharmaceutical company submitted clinical trial data to the U.S. Food and Drug Administration (FDA) requesting approval to advertise their brand of growth hormone for the treatment of ISS. [cite journal |author=Leschek EW, Rose SR, Yanovski JA, "et al" |title=Effect of growth hormone treatment on adult height in peripubertal children with idiopathic short stature: a randomized, double-blind, placebo-controlled trial |journal=J. Clin. Endocrinol. Metab. |volume=89 |issue=7 |pages=3140–8 |year=2004 |pmid=15240584 |doi=10.1210/jc.2003-031457 |url=http://jcem.endojournals.org/cgi/pmidlookup?view=long&pmid=15240584] They proposed a definition of a height more than 2.25 standard deviations below mean, roughly equal to the shortest 1.2% of the population.

Other researchers have described a cutoff of 2.0 standard deviations.cite journal |author=Bonioli E, Tarò M, Rosa CL, "et al" |title=Heterozygous mutations of growth hormone receptor gene in children with idiopathic short stature |journal=Growth Horm. IGF Res. |volume=15 |issue=6 |pages=405–10 |year=2005 |pmid=16213173 |doi=10.1016/j.ghir.2005.08.004 |url=http://linkinghub.elsevier.com/retrieve/pii/S1096-6374(05)00107-3]

Controversies

Since 2003 this diagnosis has become controversial.
*Does it make sense to define a disease solely as a certain percentage of the population if there is little perceptible difference between a person just above the cutoff and another person just below the cutoff?

*How many of the people in the lowest 2% of the population are not simply "idiopathic" but rather have subtler disorders of growth hormone or IGF1 production or responsiveness or any of hundreds of other known or unknown conditions not readily detected by an "ordinary" growth evaluation?

*Should insurance or government (the other 99% of the population) pay US$100,000 or more (which is the estimated cost [cite journal |author=Lee JM, Davis MM, Clark SJ, Hofer TP, Kemper AR |title=Estimated cost-effectiveness of growth hormone therapy for idiopathic short stature |journal=Arch Pediatr Adolesc Med |volume=160 |issue=3 |pages=263–9 |year=2006 |pmid=16520445 |doi=10.1001/archpedi.160.3.263 |url=http://archpedi.ama-assn.org/cgi/pmidlookup?view=long&pmid=16520445] ) to treat someone to move them from the first percentile to perhaps the 10th? Would that just transfer the disadvantages to those in what was previously the "2nd" percentile?

*Even if treatment successfully changes height to the 5th or 10th percentile, does it actually improve the person's life in any measurable way? There is some evidence that hormone treatment may not result in a significant improvement in psychosocial functioning.cite journal |author=Visser-van Balen H, Sinnema G, Geenen R |title=Growing up with idiopathic short stature: psychosocial development and hormone treatment; a critical review |journal=Arch. Dis. Child. |volume=91 |issue=5 |pages=433–9 |year=2006 |pmid=16632673 |doi=10.1136/adc.2005.086942 |url=http://adc.bmjjournals.com/cgi/pmidlookup?view=long&pmid=16632673]

Treatment

The use of insulin-like growth factor 1cite journal |author=Savage MO, Camacho-Hübner C, David A, "et al" |title=Idiopathic short stature: will genetics influence the choice between GH and IGF-I therapy? |journal=Eur. J. Endocrinol. |volume=157 Suppl 1 |issue= |pages=S33–7 |year=2007 |pmid=17785695 |doi=10.1530/EJE-07-0292 |url=http://eje-online.org/cgi/pmidlookup?view=long&pmid=17785695] or aromatase inhibitorscite journal |author=Damiani D, Damiani D |title=Pharmacological management of children with short stature: the role of aromatase inhibitors |journal=J Pediatr (Rio J) |volume=83 |issue=5 Suppl |pages=S172–7 |year=2007 |pmid=17901908 |doi=10.2223/JPED.1699] have been proposed as an alternative to growth hormone.

See also

* growth hormone treatment

References

External links

* [http://www.fda.gov/bbs/topics/ANSWERS/2003/ANS01242.html Account of the FDA approval of Humatrope for ISS]
* [http://www.health.state.mn.us/htac/hgh.htm Excellent synopsis of GH use for ISS from the Minnesota Dept of Health]
* [http://www.nossaonline.org/ National Organization of Short Statured Adults]


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