Page 16 - Journal of Special Operations Medicine - Winter 2015

never had a sense of urgency and his bowel habits were Disclosures
not expected to interfere with Special Operations duty, The authors have nothing to disclose. This article repre-
and because his levels of fat-soluble vitamins were sents the views of the authors and in no way represents
within normal limits, it was not thought that this would the views of the United States Department of Defense,
preclude him from special warfare service.
Department of the Navy, or the Department of Veterans
Affairs.
The patient’s neurologic examination findings were
normal. Specifically, his gait and speech were normal,
his cranial nerves were intact, there was no finger-to- References
nose ataxia, he had full appreciation of light touch and 1. Turk U, Basol G, Barutcuoglu B, et al. A 54-year-old dia-
pinprick, his strength was normal throughout, and his betic man with low serum cholesterol. Clin Chem. 2012;58:
DTRs were not diminished. 826–829.
2. Schonfeld G. Familial hypobetalipoproteinemia: a review. J
He denied difficulties with night vision. His color vision Lipid Res. 2003;44:878–883.
was intact and his visual acuity was excellent. While 3. Schonfeld G. The hypobetalipoproteinemias. Annu Rev Nutr.
1995;15:23–34.
ophthalmologic disease is unlikely to develop in het- 4. Tarugi P, Averna M. Hypobetalipoproteinemia: genetics, bio-
erozygous FHBL, we thought that it was important to chemistry, and clinical spectrum. Adv Clin Chem. 2011;54:
ensure that his retinas did not show evidence of subclini- 81–107.
cal disease. Findings on a retinal examination by an eye 5. Tarugi P, Averna M, Leo ED, et al. Molecular diagnosis of
specialist also were normal. hypobetalipoproteinemia: and ENID review. Atherosclerosis.
2007;195:e19–27.
6. Lee J, Hegele RA. Abetalipoproteinemia and homozygous hy-
Acanthocytosis is a distinguishing feature on a periph- pobetalipoproteinemia: a framework for diagnosis and man-
eral blood smear in patients with ABL and homozygous agement. J Inherit Metab Dis. 2014;37:333–339.
FHBL. 9,10 This is due to vitamin E deficiency, which is 7. Sen D, Dagdelen S, Erbas T. Hepatosteatosis with hypobetali-
poproteinemia. J Natl Med Assoc. 2007;99:284–286.
10
vital to the integrity of the RBC membrane. As vitamin 8. Kwiterovich PO. Diagnosis and management of familial dys-
E deficiency is of special concern in the development of lipoproteinemias. Curr Cardiol Rep. 2013;15:371.
symptoms, a peripheral smear was obtained and exam- 9. Welty FK. Hypobetalipoproteinemia and abetalipoprotein-
ined for the presence of acanthocytes. No acanthocytes emia. Curr Opin Lipidol. 2014;25:161–168.
were seen, lending further support to our presumptive 10. Peretti N, Sassolas A, Roy CC, et al. Guidelines for the di-
diagnosis of heterozygous FHBL. agnosis and management of chylomicron retention disease
based on a review of the literature and the experience of two
centers. Orphanet J Rare Dis. 2010;5:24.
In addition, the patient was counseled to avoid lifestyles 11. Zamel R, Khan R, Pollex RL, et al. Abetalipoproteinemia:
that may contribute to the development of fatty liver, as two case reports and literature review. Orphanet J Rare Dis.
he may already be at increased risk. Due to the risks in- 2008;3:19.
volved if he were to develop fat-soluble vitamin deficien- 12. Heeks LV, Hooper AJ, Adams LA, et al. Non-alcoholic
steatohepatitis related cirrhosis in a patient with APPOB
cies and the sequelae of such, we thought he should have L343V familial hypobetalipoproteinaemia. Clin Chim Acta.
the levels of fat-soluble vitamins checked at his yearly 2013;421:121–125.
physical examinations. If they remained stable over a 13. Ballestri S, Lonardo A, Losi L, et al. Do diabetes and obesity
period of years, then it may be reasonable to lengthen promote hepatic fibrosis in familial heterozygous hypobetali-
poproteinemia? Intern Emerg Med. 2009;4:71–73.
the frequency at which they are tested. While this pa- 14. Brosnahan DM, Kennedy SM, Converse CA, et al. Pathology
tient may benefit from further elucidation of his true of hereditary retinal degeneration associated with hypobetali-
genotype through genetic testing, it was not thought poproteinemia. Ophthalmology. 1994;101:38–45.
that it would alter the patient’s management and dispo-
sition regarding Special Operations duty.

LT Strain is with the Captain James A. Lovell Federal Health
Conclusion Care Center (FHCC), Department of Undersea Medicine,
Patients desiring participation in Special Operations Chicago, Illinois. Dr Strain graduated from the University of
duty who are found to have hypolipidemia should have Kansas School of Medicine in 2013 and then completed an
a thorough medical history, laboratory evaluation, and Internal Medicine internship at Naval Medical Center Ports-
imaging studies. If available, data on family members’ mouth. Following completion of the Undersea Medical Offi-
cer (UMO) course in December 2014, he was assigned to the
lipid levels should also be obtained to guide diagnosis. Captain James A. Lovell FHCC, where he has served as a staff
If the preponderance of evidence suggests a diagnosis of UMO since. E-mail: jonathan.strain@med.navy.mil.
heterozygous FHBL and if the workup results are nega-
tive, it seems reasonable to clear these individuals for LCDR (SEL) Vigilante is with the Captain James A. Lovell
participation in military Special Operations. Federal Health Care Center (FHCC), Department of Undersea

4 Journal of Special Operations Medicine Volume 15, Edition 4/Winter 2015