Short Stature: Difference between revisions
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*If the [[short stature]] is caused due to a [[hormonal]] [[deficiency]] it should be managed with [[hormonal]] [[treatment]].<ref name="pmidPMID: 32310491">{{cite journal| author=| title=StatPearls | journal= | year= 2020 | volume= | issue= | pages= | pmid=PMID: 32310491 | doi= | pmc= | url= }} </ref> | *If the [[short stature]] is caused due to a [[hormonal]] [[deficiency]] it should be managed with [[hormonal]] [[treatment]].<ref name="pmidPMID: 32310491">{{cite journal| author=| title=StatPearls | journal= | year= 2020 | volume= | issue= | pages= | pmid=PMID: 32310491 | doi= | pmc= | url= }} </ref> | ||
*These include treating growth hormonal deficiencies and constitutional growth delays with gonadotropin-releasing hormone analogues (GnRHa), aromatase inhibitors, recombinant human insulin-like growth factor- 1 (RhIGF-1), low-dose androgen therapy, recombinant human growth hormone (rhGH) etc. <ref name="pmidPMID: 28688585">{{cite journal| author=Lanes R, González Briceño LG| title=Alternatives in the Treatment of Short Stature. | journal=Adv Pediatr | year= 2017 | volume= 64 | issue= 1 | pages= 111-131 | pmid=PMID: 28688585 | doi=10.1016/j.yapd.2017.03.004 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=28688585 }} </ref> | *These include treating [[growth hormonal]] [[deficiencies]] and constitutional [[growth]] delays with [[gonadotropin-releasing hormone]] [[analogues]] ([[GnRHa]]), aromatase inhibitors, recombinant human insulin-like growth factor- 1 (RhIGF-1), low-dose androgen therapy, recombinant human growth hormone (rhGH) etc. <ref name="pmidPMID: 28688585">{{cite journal| author=Lanes R, González Briceño LG| title=Alternatives in the Treatment of Short Stature. | journal=Adv Pediatr | year= 2017 | volume= 64 | issue= 1 | pages= 111-131 | pmid=PMID: 28688585 | doi=10.1016/j.yapd.2017.03.004 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=28688585 }} </ref> | ||
* In addition to [[treating]] the underlying [[cause]], [[patients]] should be provided with [[psychosocial]] [[counselling]] and [[support]] in order to help them cope with [[psychosocial]] [[distress]] as a result of their [[short stature]].<ref name="pmidPMID: 32310491">{{cite journal| author=| title=StatPearls | journal= | year= 2020 | volume= | issue= | pages= | pmid=PMID: 32310491 | doi= | pmc= | url= }} </ref> | |||
* In addition to treating the underlying cause, patients should be provided with psychosocial counselling and support in order to help them cope with psychosocial distress as a result of their short stature.<ref name="pmidPMID: 32310491">{{cite journal| author=| title=StatPearls | journal= | year= 2020 | volume= | issue= | pages= | pmid=PMID: 32310491 | doi= | pmc= | url= }} </ref> | |||
=== Surgery === | === Surgery === | ||
Revision as of 21:42, 10 November 2020
| Short stature | |
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| ICD-10 | E34.3 |
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| ICD-9 | 783.43 |
| DiseasesDB | 18756 |
| MedlinePlus | 003271 |
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ayesha Javid, MBBS[2]
Overview
Short stature is defined as a condition in which the height of an individual is in the 3rd percentile for the mean height of a given age, sex, and population group. According to Ranke (1996), “Short stature is defined as a condition in which the height of an individual is two standard deviations (SD) below the corresponding mean height of a given age, sex and population group.” [1]
Historical Perspective
Classification
There are two types of short stature,
Proportionate short stature (PSS)
Proportionate short stature (PSS) is when the person is small, but limbs and trunk height are in proportions. [2]
Disproportionate short stature (DSS)
Whereas in disproportionate short stature (DSS), the normal proportion of limbs and trunk height is absent, and the individual shows a great difference in their sitting and standing height. [2]
Causes
PHYSIOLOGICAL CAUSES OF SHORT STATURE OR NORMAL VARIANTS OF GROWTH
Familial short stature
Constitutional delay of growth and puberty
Idiopathic short stature
Small for gestational age infants with catch-up growth
PATHOLOGIC CAUSES OF GROWTH FAILURE
Systemic disorders with secondary effects on growth
- Undernutrition
- Glucocorticoid therapy
- Gastrointestinal disease
- Rheumatologic disease
- Chronic kidney disease
- Cancer
- Pulmonary disease
- Cardiac disease
- Immunologic disease
- Metabolic diseases
Genetic Causes
Several genetic disorders have prominent effects on growth.
- Turner's Syndrome
- SHOX gene variant
- Prader-Willi Syndrome
- Noonan syndrome
- Silver-Russell syndrome
- Skeletal dysplasias/growth plate abnormalities
Endocrine Causes
- Cushing syndrome
- Hypothyroidism
- Growth hormone deficiency
Other causes of short stature
Psychosocial short stature
It is a growth disorder that is observed between the ages of 2 and 15, caused by extreme emotional deprivation or stress.
The symptoms include decreased growth hormone (GH) and somatomedin secretion, very short stature, weight that is inappropriate for the height, and immature skeletal age. This disease is a progressive one, and as long as the child is left in the stressing environment, their cognitive abilities continue to degenerate. Though rare in the population at large, it is common in feral children and in children kept in abusive, confined conditions for extended lengths of time. It can cause the body to completely stop growing but is generally considered to be temporary; regular growth will resume when the source of stress is removed
Differential Diagnosis
- Endocrine disorders: Growth hormone deficiency (GHD), insulin-like growth factor-1 (IGF-1), growth failure, constitutional growth delay, growth failure, stunted growth, structural brain abnormalities, or pituitary lesions, pituitary microadenomas, congenital hypothyroidism, idiopathic short stature, intrauterine growth deficiency, etc.[3]
- Genetic disorders: Down syndrome, Turner syndrome, 3M syndrome, Noonan syndrome, Prader-Willi syndrome, Aarskog syndrome, Silver-Russell syndrome, etc.
- Bone diseases: Dwarfism, Achondroplasia (short-limbed dwarfism), diastrophic dysplasia (short-limbed dwarfism), spondylo-epiphyseal dysplasia (short-trunk dwarfism), rickets, etc
- Chronic disorders: Cystic fibrosis, Crohn disease, juvenile idiopathic arthritis (JIA), anemia, chronic renal insufficiency, inflammatory bowel disorder, chronic malnutrition, etc
- Psychological distress [2]
Epidemiology and Demographics
In the United States, 2.5% of the population is short. A study conducted on the school children age 4-16 years in the South Indian Population shows the overall prevalence of short stature was 2.86%.
Gender
- People of both sexes are affected, but
- A retrospective study in South China showed that more boys were admitted than girls. This could be due to the reason that males are more likely to be brought to medical attention because of the prevalence of social expectations and pressures as compared to the females.
- On the other hand, in Rosario, Argentina, the higher prevalence was found in females (16.4%) than males (8.4%) (p<0.001).[21] The short stature in females was related to age, weight, and abdominal obesity.
Age
- Different ethnic groups have different average statures, which are essential to take into consideration while comparing mean parameters.
- Any individual who has not attained the union of epiphyseal plates, can get affected.
Treatment
Medical Therapy
- The primary management of short stature should be to treat the underlying cause.[2]
- If the short stature is caused due to a hormonal deficiency it should be managed with hormonal treatment.[2]
- These include treating growth hormonal deficiencies and constitutional growth delays with gonadotropin-releasing hormone analogues (GnRHa), aromatase inhibitors, recombinant human insulin-like growth factor- 1 (RhIGF-1), low-dose androgen therapy, recombinant human growth hormone (rhGH) etc. [4]
- In addition to treating the underlying cause, patients should be provided with psychosocial counselling and support in order to help them cope with psychosocial distress as a result of their short stature.[2]
Surgery
- Bone lengthening surgery is being performed not only for the treatment of dwarfism and/or skeletal deformities caused by congenital abnormalities, tumors and infections.[5] [6]
- The surgery is performed using Ilizarov method with circular external fixation. This is called cosmetic leg lengthening or symmetrical extended limb lengthening. [7] [8]
Prevention
- There are no primary preventive measures available for short stature.
References
- ↑ "StatPearls". 2020. PMID 32310491 Check
|pmid=value (help). - ↑ 2.0 2.1 2.2 2.3 2.4 2.5 "StatPearls". 2020. PMID 32310491 PMID: 32310491 Check
|pmid=value (help). - ↑ Derrick KM, Gomes WA, Gensure RC (2018). "Incidence and Outcomes of Pituitary Microadenomas in Children with Short Stature/Growth Hormone Deficiency". Horm Res Paediatr. 90 (3): 151–160. doi:10.1159/000489456. PMID 30261514 PMID: 30261514 Check
|pmid=value (help). - ↑ Lanes R, González Briceño LG (2017). "Alternatives in the Treatment of Short Stature". Adv Pediatr. 64 (1): 111–131. doi:10.1016/j.yapd.2017.03.004. PMID 28688585 PMID: 28688585 Check
|pmid=value (help). - ↑ Cattaneo R, Villa A, Catagni M, Tentori L (1988). "Limb lengthening in achondroplasia by Ilizarov's method". Int Orthop. 12 (3): 173–9. doi:10.1007/BF00547160. PMID 3182120 PMID: 3182120 Check
|pmid=value (help). - ↑ Ottaviani G, Randelli P, Catagni MA (2005). "Segmental cement extraction system (SEG-CES) and the Ilizarov method in limb salvage procedure after total knee cemented prosthesis removal in a former osteosarcoma patient". Knee Surg Sports Traumatol Arthrosc. 13 (7): 557–63. doi:10.1007/s00167-004-0575-8. PMID 15660273 PMID: 15660273 Check
|pmid=value (help). - ↑ Stathis SL, O'Callaghan MJ, Williams GM, Najman JM, Andersen MJ, Bor W (1999). "Behavioural and cognitive associations of short stature at 5 years". J Paediatr Child Health. 35 (6): 562–7. doi:10.1046/j.1440-1754.1999.00427.x. PMID 10634984 PMID: 10634984 Check
|pmid=value (help). - ↑ Kranzler JH, Rosenbloom AL, Proctor B, Diamond FB, Watson M (2000). "Is short stature a handicap? A comparison of the psychosocial functioning of referred and nonreferred children with normal short stature and children with normal stature". J Pediatr. 136 (1): 96–102. doi:10.1016/s0022-3476(00)90057-x. PMID 10636982 PMID: 10636982 Check
|pmid=value (help).