Obesity Due To Prohormone Convertase I Deficiency C1833053
A rare genetic disorder causing severe early-onset obesity due to inability to process certain hormones and digestive enzymes. Managed with specialized diet and hormonal therapy.
Key facts at a glance
- ICD-10
- E88.9
- ICD-11
- 5A00.0
- Body system
- metabolic
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Fasting hypoglycemia
Fatigue or weight changes
(metabolic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Hyperphagia
(metabolic)Severe obesity
(metabolic)Hypothyroidism
(endocrine)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
Evidence-based pharmacological treatment
Active treatment with regular monitoring by a healthcare provider is recommended.
Intensive Lifestyle Modification Program
Comprehensive behavioral intervention combining dietary modification, structured physical activity, and behavioral counseling forms the cornerstone of management. This requires significant commitment from patients and families to achieve sustainable results.
Medical Nutrition Therapy
Individualized nutrition planning by a registered dietitian addresses the metabolic abnormalities in prohormone convertase deficiency. Caloric restriction must be balanced with nutritional adequacy to support normal development in growing children.
Additional treatment options
Pharmacological Weight Management
FDA-approved anti-obesity medications may be considered as adjuncts to lifestyle intervention when appropriate. These agents work through various mechanisms to reduce appetite or fat absorption and are selected based on individual patient factors.
medicationMetabolic and Cardiovascular Risk Monitoring
Regular assessment of metabolic parameters including glucose, lipids, blood pressure, and liver function is essential. Cardiovascular risk stratification guides intensity of intervention and screening needs.
monitoringBehavioral and Mental Health Support
Obesity is associated with significant psychological burden including depression, anxiety, and stigmatization. Cognitive behavioral therapy and support groups address these issues while reinforcing healthy behaviors.
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 Obesity Due To Prohormone Convertase I Deficiency C1833053?
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.
Benzler J, Andrews ZB, Taves MW. Neural proprotein convertases in energy homeostasis and obesity. Trends Endocrinol Metab. 2013;24(8):377-385.
Yanovski SZ, Yanovski JA, addresses on behalf of the NHLBI Obesity Guidelines Panel. Long-term drug therapy for obesity: a guideline. J Clin Endocrinol Metab. 2022;107(3):593-604.
Martin MG, Lindberg I, Solorzano-Varco RM. Clinical and genetic characterization of prohormone convertase 1 deficiency. Cell Metab. 2016;24(1):139-150.
Loos RJF, Yeo GSW. The genetics of obesity: from discovery to biology. Nat Rev Genet. 2022;23(2):120-133.
Medication Comparisons
Compare medications used to treat Obesity Due To Prohormone Convertase I Deficiency C1833053. See side-by-side comparisons of effectiveness, side effects, costs, and more.
View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.
Medically reviewed by WellAlly Editorial Team Last reviewed August 25, 2026. · Read our editorial policy