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  • Le Syndrome de Klinefelter
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Le devenir du Klinefelter

The future of Klinefelter's syndrome

Resume

Le devenir du Syndrome de Klinefelter (SK) au delà de l'âge du dépistage dans l'enfance ou lors de l'incorporation au Service Militaire est mal connu.

33 observations réunies dans des services divers chez des sujets de 20 à 65 ans servent de support à une revue de la littérature; celle-ci permet d'identifier deux groupes de données.

Le premier groupe concerne des facteurs de risque indéterminés car les obervations rapportées restent isolées (hémopathies, processus tumoraux). Les troubles de la régulation glucidique et un hyperinsulinisme paraissent néanmoins plus apparents.

Le deuxième groupe de faits correspond à un risque relatif plus significatif. On y retient le cancer du sein, l'ostéoporose, les ulcères de jambes. Une pathologie dysimmunitaire pourraît être un facteur déterminant de plusieurs circonstances pathologiques.

Les tumeurs testiculaires que l'on pouvait craindre sont en fait des événements rares.

Abstract

Medical prognosis of Klinefelter's Syndrome (KS) has to been elucidate. our knowledge about the future of KS beyond infancy or military enrolement is poor.

We report 33 cases of KS issued from various departments, aged from 20 to 65 years, who served as a support for study of the litterature. It was then possible to determine two groups of risk.

The first group relates undetermined risk factors because reported cases are rare (Various Tumors; Leukemia); however these facts appear surpass a mere coincidence. Abnormal glucid regulation and hyperinsulinism are also described.

The second group of facts relates more meaningful risks as breast carcinoma, osteoporosis and lower leg ulcers. Since patients develop frequently autoimmune diseases, this would be the missing link between some pathologies and the sex anomaly.

Suprisingly testicular tumors are rare in spite of numerous intrinseque factors of risk.

References

  1. ALARCON-SEGOVIA D., SAUZA J.: Systemic Lupus Erythematosus and KLINEFELTER's Syndrome in “KLINEFFELTER's Syndrome Vol 1, Ed. Springer-Verlag. 1984; 109–114.

  2. BAUDUCEAU-POPELIER M., NIZOU C., REBOUL P., BERTIN E., FLAGEAT J., GAUTIER D.: Ostéoporose précoce et Syndrome de KLINEFELTER. 10ème Congrès Français d'Endocrinologie 26 Septembre 1991, Ann. Endocrinol. 1991; 52: 188.

    Google Scholar 

  3. BREIT R.: Lower leg Ulcers in KLINEFELTER's Syndrome in „KLINEFELTER's Syndrome Vol. 1, Ed. Springer-Verlag, 1994; 71–79.

  4. GAUTIER D., BAUDUCEAU B., ZIDI E., LEFEBVRE P., SCHOONBERG C., FROMANTIN M.: Les multiples visages du Syndrome de KLINEFELTER. Rev. Franç. Endocrinol. Clin. 1991; 31: 89–96.

    Google Scholar 

  5. GAUTIER D., NIZOU C., BAUDUCEAU B., CLEMENT R., SCHOONBERG C., LARROQUE P.: Pathologie veineuse et syndrome de KLINEFELTER. Rev. Franç. Endocrinol. Clin. 1991; 32: 189–195.

    Google Scholar 

  6. HESSE S., BERBIS Ph., JUHAN-VAGUE J., BONERANDI J.J., PRIVAT Y.: Syndrome de KLINEFELTER et Ulcères de Jambe: Place des troubles de l'hémostase. Ann. Dermatol. Venereol. 1992; 119: 951–957.

    PubMed  CAS  Google Scholar 

  7. HOROWITZ M., NORDIN B.E.C., AARON J., STEURER T., FRANCIS R.M., PHILCOX J.C., SEAMARK R.: Osteoporosis and KLINEFELTER's Syndrome. in „KLINEFELTER's Syndrome Vol 1, Ed. Springer-Verlag, 1984; 51–61.

  8. HOROWITZ M., NISHART J.M., O'LOUGHLIN P.D., MORRIS H.A., NEED A.G., NORDIN B.E.C.: Osteoporosis and KLINEFELTER's Syndrome. Clin. Endocrinol. 1992; 36: 113–118.

    Article  CAS  Google Scholar 

  9. KOBAYASH S., SHIMAMOTO T., TANIGUCHI O., HASHIMOTO H., HIROSE S.: KLINEFELTER's Syndrome associated with progressive systemic sclerosis: report of a case and review of the litterature. Clin. Rheumatol. 1991; 10: 84–86.

    Article  Google Scholar 

  10. KOYAMA M., UEJIMA K., KONISHI T., ISHIDA M., KATO M., NISHIGAKI M., MIZUTANI H. MATSUMOTO K., KAWASE M., TAMAKI T.: A case of malignant lymphoma accompanied by KLINEFELTER's Syndrome. Intern. Med. 1992; 31: 496–499.

    Article  PubMed  CAS  Google Scholar 

  11. MEISTER P.: KLINELFTER's Syndrome and testicular tumors in „KLINEFELTER's Syndrome 1 Vol Ed. Springer-Verlag, 1984; 115–117.

  12. MUTS-HOMSMA S.J.M., GERAEDTS J.P.M.: KLINEFELTER's Syndrome and leukemia: coïncidence an Association in „KLINEFELTER's Syndrome 1 Vol Ed. Springer-Verlag, 1984; 127–130.

  13. ROUFFY J., PESTEL M., CORTOT A., SIKORAV H., MICHAUX R., JULIEN R.: Syndrome de KLINEFELTER, hypertriglycéridémie endogène et artériopathie des membres inférieurs. Ann. Med. Int. 1973; 124: 201–206.

    CAS  Google Scholar 

  14. SCHEIKE O., VISFELDT J., PETERSEN B.: Breast Carcinoma in Association with KLINEFELTER's Syndrome in „KLINEFELTER's Syndrome 1 Vol Ed. Springer-Verlag, 1984; 118–126.

  15. WONG F.H.W., PUN K.K., WANG C.: Loss of Bone Mass in Patients with KLINEFELTER's Syndrome Despite Sufficient Testosterone Replacement. Osteoporosis Int. 1993; 3: 3–7.

    Article  CAS  Google Scholar 

  16. ZUPPINGER K., ENGEL E., FORBES A.P., MANTOOTH L., CLAFFEY J.: KLINEFELTER's Syndrome — a clinical and cytogenetic Study in 24 cases. Acta. Endocrinol. 1967; 54: Suppl. 113. 0652

    Google Scholar 

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Gasnault, J.P., Dewailly, D. & Leroy-Billard, M. Le devenir du Klinefelter. Androl. 4, 303–311 (1994). https://doi.org/10.1007/BF03034319

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