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Article
February 1953

ERUPTIVE XANTHOMA AND HYPERLIPEMIA IN GLYCOGEN STORAGE DISEASE (VON GIERKE'S DISEASE)

AMA Arch Derm Syphilol. 1953;67(2):146-151. doi:10.1001/archderm.1953.01540020024005
Abstract

SINCE von Gierke1 reported his two cases of glycogen storage disease in 1929, fewer than 100 cases have been reported thus far in the medical literature. Because of the rarity of the condition and the fact that we could not find any case reports in the dermatologic literature, this case of eruptive xanthoma and hyperlipemia in glycogen storage disease is deemed worthy of publication.

REPORT OF A CASE

V. K., a 4-month-old white girl, was born after a full term pregnancy and a normal delivery. Her birth weight was 2,975 gm. (6 lb., 9 oz.). During her neonatal period, the infant had diarrhea and frequent vomiting until these were stopped by change of formula. The infant then appeared to thrive up to 2 months of age. It was then noticed that she had a constantly distended abdomen and frequent stools. About two weeks prior to the admission of

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    EXPAND ALL
    Fish Oil to Prevent Psychosis
    Scott W Woods, MD | Yale University
    Amminger et al(1) have made a valuable contribution to the prevention of psychotic disorders. Three additional points are worthy of discussion. First, the authors studied only one dose of the ?-3 preparation, and other doses or other preparations may not be as effective. From April 2002 to November 2003, we enrolled 7 subjects meeting criteria for risk syndromes for psychosis(2) similar to those used by Amminger et al in an 12-week, open - label study of an ?-3 preparation. None were receiving antipsychotic medication. Visits were weekly for 8 weeks and then biweekly; extension out to one year was permitted for patients who improved. Response was defined based on achieving no positive symptoms in the at-risk range on the Scale of Psychosis-risk Symptoms (SOPS).(3) In our sample (5M, 2F, 18.1±3.6 years), 2 patients completed at least 12 weeks and responded (at 4 and 16 weeks); however, 2 patients converted to psychosis while on protocol (to schizophreniform disorder at 2 weeks and to psychotic disorder NOS at 6 weeks), and a third converted (to schizophrenia at 6 weeks) one week after dropping out due to worsening depressive symptoms. The remaining two patents dropped out, one due to improvement after 3 weeks (a responder) and one after 6 weeks for reasons related to leaving college. Based on the three conversions among seven patients, we suspended the study. Our ?-3 preparation was purified ethyl-eicosapentaenoic acid (EPA) 500 mg, two tablets twice daily, the same preparation and dose that produced mixed findings in the authors’ previous first-episode antipsychotic augmentation study.(4) It is possible that the 700 mg/d of EPA that Amminger et al delivered in the current study to the at-risk patients is effective on average but the 2000 mg/d we gave is not. Also possible is that docosohexaenoic acid and other ?-3 fatty acids in the Amminger et al mixture are required for efficacy, a suggestion recently put forth in a systematic review to explain mixed findings with ?-3 fatty acids in bipolar disorder.(5) Other possibilities for the discrepant findings are that that we discontinued our study prematurely, given that 3/7 patients responded to treatment, or that we recruited a more severe and perhaps a “later” at-risk sample: SOPS total scores at baseline were 45±18, compared to 40±16 in our previous trial.(6) Second, it is worth noting that the signature “fish burp” side effect of ?-3 fatty acids, which can occur in as many as 75% of subjects,(7) risks compromising the blind.(8) Assessment of the blind through patient “guesses” is worthwhile to incorporate into future studies. Newer, more highly refined fish oil preparations may minimize this issue. Third, the authors emphasize that therapeutic effects persisted after cessation of the 12-week intervention. Given the ready availability of ?-3 preparations over-the-counter and the resourcefulness of patients and their families, the possibility that some or many patients continued ?-3 preparations on their own during follow-up should not be discounted.
    Scott W. Woods Thomas H. McGlashan Yale University School of Medicine
    Dr. Woods received a small grant from Laxdale Limited to conduct the open label trial. In the past ten years he has received investigator-initiated grant support from Eli Lilly, Janssen, Bristol Myers-Squib, Pfizer, Schering Plough, and Glytech Inc and speaking or consultant fees from Schering Plough and Otsuka. Dr. McGlashan has received investigator-initiated grant support from Eli Lilly.
    References
    1. Amminger GP, Schafr MR, Papageorgiou K, Klier CM, Cotton SM, Harrigan SM, Mackinnon A, McGorry PD, Berger GE. Long-chain ?-3 fatty acids for indicated prevention of psychotic disorders: A randomized, placebo-controlled trial. Arch Gen Psychiatry. 2010;67(2):146-154.
    2. Woods SW, Addington J, Cadenhead KS, Cannon TD, Cornblatt BA, Heinssen R, Perkins DO, Seidman LJ, Tsuang MT, Walker EF, McGlashan TH. Validity of the prodromal risk syndrome for psychosis: findings from North American Prodrome Longitudinal Study. Schizophrenia Bulletin. 2009;35:894-908.
    3. Miller TJ, McGlashan TH, Woods SW, Stein K, Driesen N, Corcoran CM, Hoffman R, Davidson L. Symptom assessment in schizophrenic prodromal states. Psychiatr Q. 1999;70(4):273-87.
    4. Berger GE, Proffitt TM, McConchie M, Yuen H, Wood SJ, Amminger GP, Brewer W, McGorry PD. Ethyl-eicosapentaenoic acid in first-episode psychosis: a randomized, placebo-controlled trial. J Clin Psychiatry. 2007;68(12):1867-75.
    5. Turnbull T, Cullen-Drill M, Smaldone A. Efficacy of omega-3 fatty acid supplementation on improvement of bipolar symptoms: a systematic review. Arch Psychiatr Nurs. 2008;22(5):305-11.
    6. McGlashan TH, Zipursky RB, Perkins D, Addington J, Miller T, Woods SW, Hawkins KA, Hoffman RE, Preda A, Epstein I, Addington D, Lindborg S, Trzaskoma Q, Tohen M, Breier A. Randomized, double-blind trial of olanzapine versus placebo in patients prodromally symptomatic for psychosis. Am J Psychiatry. 2006;163(5):790-9.
    7. Marangell LB, Martinez JM, Zboyan HA, Kertz B, Kim HF, Puryear LJ. A double-blind, placebo-controlled study of the omega-3 fatty acid docosahexaenoic acid in the treatment of major depression. Am J Psychiatry. 2003;160(5):996-8.
    8. Stoll AL, Severus WE, Freeman MP, Rueter S, Zboyan HA, Diamond E, Cress KK, Marangell LB. Omega 3 fatty acids in bipolar disorder: a preliminary double-blind, placebo-controlled trial. Arch Gen Psychiatry. 1999;56(5):407- 12.
    CONFLICT OF INTEREST: None Reported
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