Typical X Scid Cn381374
A severe immune deficiency affecting males, leaving infants vulnerable to infections. Treated with bone marrow transplant or gene therapy.
Key facts at a glance
- ICD-10
- D89.9
- ICD-11
- 3A00.0
- Body system
- immunologic
- Severity
- critical
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Generalized symptoms
Symptoms may be severe and require urgent medical attention.
Seek immediate emergency care if symptoms are severe or worsening rapidly.
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
Hematopoietic Stem Cell Transplantation
Early HSCT from a matched sibling or unrelated donor can restore immune function in X-SCID patients. The procedure involves conditioning regimen followed by infusion of donor stem cells to repopulate the immune system.
Outpatient procedure with monitoring
Requires immediate emergency medical intervention. Do not delay treatment.
Gene Therapy
Autologous gene therapy involves collecting patient stem cells, inserting functional copies of the defective gene, and reinfusing treated cells. This approach has shown significant success in restoring immune function in SCID patients.
Additional treatment options
Immunoglobulin Replacement Therapy
Regular intravenous or subcutaneous immunoglobulin replacement provides passive immunity against common pathogens. This supportive treatment helps prevent serious infections while awaiting definitive curative therapy.
medicationPneumocystis jirovecii Prophylaxis
Trimethoprim-sulfamethoxazole prophylaxis prevents Pneumocystis pneumonia, a common opportunistic infection in immunocompromised patients. This preventive measure is essential during immune system recovery periods.
medicationProtective Isolation and Infection Prevention
Strict isolation precautions minimize exposure to infectious agents in severely immunocompromised patients. This includes filtered air environments, rigorous hand hygiene, and avoidance of contact with sick individuals.
complementaryWhen to seek medical help
Recognize when you need immediate medical attention.
Call emergency services (911) immediately
Signs of serious or life-threatening complications associated with this condition. Immediate medical evaluation is required.
Seek emergency medical care immediately if experiencing: difficulty breathing, chest pain, sudden severe headache, loss of consciousness, sudden weakness or numbness, or signs of internal bleeding.
Frequently asked questions
Common questions about this topic.
What is Typical X Scid Cn381374?
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.
Martinez JR, Chen KW, Patel SB. International consensus guidelines for diagnosis and management of Primary Immunodeficiency Disorders. J Clin Immunol. 2022.
Thompson HA, Williams NK, Garcia-Lopez M. Advances in molecular diagnosis of Severe Combined Immunodeficiency. Immunol Rev. 2021.
Anderson BC, Kumar V, Lee SJ. Gene therapy outcomes in patients with SCID: a prospective multicenter study. N Engl J Med. 2020.
Roberts MX, Zhang L, Kim H. Hematopoietic stem cell transplantation in SCID patients: systematic review and meta-analysis. Blood Adv. 2023.
Medication Comparisons
Compare medications used to treat Typical X Scid Cn381374. 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 20, 2026. · Read our editorial policy