X Linked Intellectual Disability Hypogonadism Ichthyosis Obesity Short Stature Syndrome C0796264
A complex genetic syndrome affecting males, involving intellectual disability, hormonal problems, skin scaling, and metabolic issues. The severity varies among affected individuals. Management requires a multidisciplinary approach addressing each symptom.
Key facts at a glance
- ICD-10
- G40.909
- ICD-11
- 8A00.0
- Body system
- neurologic
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Generalized symptoms
(neurologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Intellectual disability
(neurologic)Obesity
(neurologic)Ichthyosis
(neurologic)Hypogonadism
(neurologic)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Drug therapy with clinical monitoring
Active treatment with regular monitoring by a healthcare provider is recommended.
Hormone replacement therapy
Testosterone replacement or other hormone therapies may be considered for individuals with hypogonadism to support pubertal development, bone health, and metabolic function. Treatment decisions should be individualized based on endocrine evaluation.
Metabolic and weight management program
Structured program addressing obesity through balanced nutrition, supervised physical activity, and behavioral modifications to improve overall metabolic health and reduce cardiovascular risk factors.
Additional treatment options
Nutritional optimization
Calorie-controlled diet with adequate protein and micronutrient intake to support growth in short stature patients while managing obesity. Consultation with a registered dietitian recommended.
dietaryCognitive and behavioral support
Psychological services addressing intellectual disability through adaptive skill training, behavioral interventions, and family support to maximize functional independence and quality of life.
psychologicalWhen to seek medical help
Recognize when you need immediate medical attention.
See a doctor within 24 hours
Moderate to severe symptoms that may indicate worsening of this condition. Prompt medical evaluation is recommended.
Contact your healthcare provider or seek urgent care if symptoms worsen rapidly. Go to the emergency department for life-threatening symptoms.
Frequently asked questions
Common questions about this topic.
What is X Linked Intellectual Disability Hypogonadism Ichthyosis Obesity Short Stature Syndrome C0796264?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Miller DT, Adam MP, Aradhya S, et al. Consensus statement: chromosomal microarray is a first-tier clinical diagnostic test for individuals with developmental disabilities or congenital anomalies. Am J Hum Genet. 2010.
Moeschler JB, Shevell M, Committee on Genetics. Comprehensive evaluation of the child with intellectual disability or global developmental delays. Pediatrics. 2014.
Rauch A, Hoyer J, Guth S, et al. Diagnostic yield of various genetic approaches in patients with unexplained developmental delay or mental retardation. Am J Med Genet A. 2012.
Kaufman L, Ayub M, Vincent JB. The genetic basis of non-syndromic intellectual disability: a review of X-linked conditions. Clin Genet. 2010.
Medication Comparisons
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy