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==Natural History==
==Natural History==
A wide range of clinical variability is observed among individuals with Alström syndrome, including among siblings. The first clinical presentation of Alström syndrome is usually [[nystagmus]] caused by cone-rod dystrophy and resulting in childhood [[blindness]]. Disease characteristics that are later in onset include truncal [[obesity]] that manifests during the first year of life, progressive [[sensorineural hearing loss]], infantile-onset [[dilated cardiomyopathy]] or later-onset [[restrictive cardiomyopathy]], insulin-resistant [[type 2 diabetes mellitus]], and hepatic, pulmonary, and [[renal dysfunction]].
A wide range of clinical variability is observed among individuals with Alström syndrome, including among siblings. The first clinical presentation of Alström syndrome is usually [[nystagmus]] caused by cone-rod dystrophy and resulting in childhood [[blindness]]. Disease characteristics that are later in onset include truncal [[obesity]] that manifests during the first year of life, progressive [[sensorineural hearing loss]], infantile-onset [[dilated cardiomyopathy]] or later-onset [[restrictive cardiomyopathy]], insulin-resistant [[type 2 diabetes mellitus]], and hepatic, pulmonary, and [[renal dysfunction]].
{| class="wikitable" border="2"
|-style="background:silver; color:black" align="center"
| '''Manifestation''' || '''Range of Age Onset''' || '''Incidence'''
|-style="background:white; color:black"
| Cone-rod dystrophy || Birth - 15 mos || 100%
|-style="background:white; color:black"
| Obesity || Birth - 5 years || 98%
|-style="background:white; color:black"
| Developmental delay || Birth-adolescence || 25%-30%
|-style="background:white; color:black"
| Dilated cardiomyopathy || 2 wks - 4 mos || 42%
|-style="background:white; color:black"
| Progressive sensorineural hearing loss || 2-25 yrs || 88%
|-style="background:white; color:black"
| Restrictive cardiomyopathy || Juvenile - late 30s || 18%
|-style="background:white; color:black"
| Insulin resistance / type 2 diabetes mellitus || 4-30 yrs / 8-40 yrs || 92% / 68%
|-style="background:white; color:black"
| Short stature || Puberty - adult || 98%
|-style="background:white; color:black"
| Hepatic disease || 8-30 yrs || 23%-92%
|-style="background:white; color:black"
| Hypogonadotropic hypogonadism || 10+ yrs || 78% of males
|-style="background:white; color:black"
| Urologic disease || Adolescence - adult || 48%
|-style="background:white; color:black"
| Renal disease || Adolescence - adult || Variably progressive with age
|}


==Complications==
==Complications==

Revision as of 19:21, 25 February 2013

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1], Associate Editor(s)-in-Chief: Aarti Narayan, M.B.B.S [2]; Raviteja Guddeti, M.B.B.S. [3]

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Natural History

A wide range of clinical variability is observed among individuals with Alström syndrome, including among siblings. The first clinical presentation of Alström syndrome is usually nystagmus caused by cone-rod dystrophy and resulting in childhood blindness. Disease characteristics that are later in onset include truncal obesity that manifests during the first year of life, progressive sensorineural hearing loss, infantile-onset dilated cardiomyopathy or later-onset restrictive cardiomyopathy, insulin-resistant type 2 diabetes mellitus, and hepatic, pulmonary, and renal dysfunction.


Manifestation Range of Age Onset Incidence
Cone-rod dystrophy Birth - 15 mos 100%
Obesity Birth - 5 years 98%
Developmental delay Birth-adolescence 25%-30%
Dilated cardiomyopathy 2 wks - 4 mos 42%
Progressive sensorineural hearing loss 2-25 yrs 88%
Restrictive cardiomyopathy Juvenile - late 30s 18%
Insulin resistance / type 2 diabetes mellitus 4-30 yrs / 8-40 yrs 92% / 68%
Short stature Puberty - adult 98%
Hepatic disease 8-30 yrs 23%-92%
Hypogonadotropic hypogonadism 10+ yrs 78% of males
Urologic disease Adolescence - adult 48%
Renal disease Adolescence - adult Variably progressive with age

Complications

References

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