eMedicine Specialties > Dermatology > Pediatric Diseases

Menkes Kinky Hair Disease: Follow-up

Author: Suguru Imaeda, MD, Chief of Dermatology, Yale University Health Services; Chief of Dermatology, West Haven Veterans Affairs Medical Center; Assistant Professor, Department of Dermatology, Yale University School of Medicine
Contributor Information and Disclosures

Updated: Nov 13, 2009

Follow-up

Further Outpatient Care

  • Seizure management is the most important aspect of outpatient care in Menkes kinky hair syndrome.

Complications

  • Complications of Menkes kinky hair syndrome include seizures and pneumonia.

Prognosis

  • The prognosis for Menkes kinky hair syndrome is poor.
  • Patients experience rapid, progressive neurologic deterioration.
  • Death typically occurs by the time the patient is aged 3 years.

Patient Education

  • Genetic counseling is indicated in Menkes kinky hair syndrome.

Miscellaneous

Medicolegal Pitfalls

  • Genetic counseling is important in the management of future pregnancies.

Special Concerns

  • Patients with Menkes kinky hair syndrome frequently have multiple issues that complicate anesthetic management.23,24 Physical characteristics (eg, small chin, prominent upper incisors) alter airway anatomy, and marked dental fragility make airway management potentially difficult. Alternatives to traditional direct laryngoscopy may be more suitable for these patients. The propensity for tooth fracture during laryngoscopic manipulation may be related to the defective collagen and connective tissue formation that are characteristic of Menkes kinky hair syndrome.25
 
Acknowledgments

The authors and editors of eMedicine gratefully acknowledge the contributions of previous Chief Editor, William D. James, MD, to the development and writing of this article.



More on Menkes Kinky Hair Disease

Overview: Menkes Kinky Hair Disease
Differential Diagnoses & Workup: Menkes Kinky Hair Disease
Treatment & Medication: Menkes Kinky Hair Disease
Follow-up: Menkes Kinky Hair Disease
References
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References

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Further Reading

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Keywords

Menkes kinky hair disease, Menkes kinky hair syndrome, steely hair syndrome, trichopoliodystrophy, copper metabolism, copper deficiency, pili torti, hair-shaft abnormality

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Contributor Information and Disclosures

Author

Suguru Imaeda, MD, Chief of Dermatology, Yale University Health Services; Chief of Dermatology, West Haven Veterans Affairs Medical Center; Assistant Professor, Department of Dermatology, Yale University School of Medicine
Suguru Imaeda, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, Connecticut State Medical Society, Sigma Xi, and Society for Investigative Dermatology
Disclosure: Nothing to disclose.

Medical Editor

Mark A Crowe, MD, Assistant Clinical Instructor, Department of Medicine, Division of Dermatology, University of Washington School of Medicine
Mark A Crowe, MD is a member of the following medical societies: American Academy of Dermatology and North American Clinical Dermatologic Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Michael J Wells, MD, Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center
Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and Texas Medical Association
Disclosure: Nothing to disclose.

Managing Editor

Robert A Schwartz, MD, MPH, Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School
Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and Sigma Xi
Disclosure: Nothing to disclose.

CME Editor

Catherine M Quirk, MD, Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania
Catherine M Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology
Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

 
 
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