Login to MyKarger

New to MyKarger? Click here to sign up.



Login with Facebook

Forgot your password?

Authors, Editors, Reviewers

For Manuscript Submission, Check or Review Login please go to Submission Websites List.

Submission Websites List

Institutional Login
(Shibboleth or Open Athens)

For the academic login, please select your country in the dropdown list. You will be redirected to verify your credentials.

Original Paper

Chronic Granulomatous Disease: A Clinical Survey of 41 Patients from the Iranian Primary Immunodeficiency Registry

Movahedi M.a · Aghamohammadi A.a · Rezaei N.a · Shahnavaz N.a · Babaei Jandaghi A.a · Farhoudi A.a · Pourpak Z.a · Moin M.a · Gharagozlou M.a · Mansouri D.b

Author affiliations

aImmunology, Asthma and Allergy Research Institute, Children’s Medical Center, Tehran University of Medical Sciences, and bMasih Daneshvari Hospital, Beheshti University of Medical Sciences, Tehran, Iran

Related Articles for ""

Int Arch Allergy Immunol 2004;134:253–259

Do you have an account?

Login Information





Contact Information











I have read the Karger Terms and Conditions and agree.



Login Information





Contact Information











I have read the Karger Terms and Conditions and agree.



To view the fulltext, please log in

To view the pdf, please log in

Buy

  • FullText & PDF
  • Unlimited re-access via MyKarger
  • Unrestricted printing, no saving restrictions for personal use
read more

CHF 38.00 *
EUR 35.00 *
USD 39.00 *

Select

KAB

Buy a Karger Article Bundle (KAB) and profit from a discount!

If you would like to redeem your KAB credit, please log in.


Save over 20% compared to the individual article price.
Learn more

Rent/Cloud

  • Rent for 48h to view
  • Buy Cloud Access for unlimited viewing via different devices
  • Synchronizing in the ReadCube Cloud
  • Printing and saving restrictions apply

Rental: USD 8.50
Cloud: USD 20.00


Select

Subscribe

  • Access to all articles of the subscribed year(s) guaranteed for 5 years
  • Unlimited re-access via Subscriber Login or MyKarger
  • Unrestricted printing, no saving restrictions for personal use
read more

Subcription rates


Select

* The final prices may differ from the prices shown due to specifics of VAT rules.

Article / Publication Details

First-Page Preview
Abstract of Original Paper

Received: November 03, 2003
Accepted: February 25, 2004
Published online: June 28, 2004
Issue release date: July 2004

Number of Print Pages: 7
Number of Figures: 2
Number of Tables: 0

ISSN: 1018-2438 (Print)
eISSN: 1423-0097 (Online)

For additional information: https://www.karger.com/IAA

Abstract

Background: Chronic granulomatous disease (CGD) represents a group of inherited disorders of the phagocytic system, involving recurrent infections at different sites, especially the respiratory system. The present study was accomplished in order to determine the clinical spectrum of Iranian patients with CGD. Methods: Forty-one patients (29 males and 12 females) with CGD, who had already been referred to two immunodeficiency referral centers in Iran, were reviewed during a 22-year period (1980–2002). Results: These patients belonged to 34 families, and 56% of them were consanguineous. The median age at the time of study was 12 years (3 months to 22 years). The median age at onset of symptoms was 4 months (1 month to 12 years), and the median diagnostic age was 5.5 years (2 months to 20 years), with a diagnostic delay of 3 years on average. The most common presenting complaint in our CGD patients was lymphadenopathy (seen in 11 patients, 26.8%). The most common manifestations of CGD (in descending order) were lymphadenopathy (75.6%), pulmonary infections (65.9%) and skin involvement (63.4%) during their illness, followed by gastrointestinal (56.1%), skeletal (29.3%), upper respiratory tract (26.8%) and central nervous system (2.4%) involvement. Conclusions: Early diagnosis of the disease is crucial. In view of the possibility of timely treatment, i.e. prophylactic treatment of infection, CGD should be excluded in any patient with unexplained infections or granulomas.

© 2004 S. Karger AG, Basel


References

  1. Berendes H, Bridges RA, Good RA: A fatal granulomatosus of childhood: The clinical study of a new syndrome. Minn Med 1957;40:309–312.
  2. Lekstrom-Himes JA, Gallin JI: Immunodeficiency diseases caused by defects in phagocytes. N Engl J Med 2000;343:1703–1714.
  3. Curnutte JT: Chronic granulomatous disease: The solving of a clinical riddle at the molecular level. Clin Immunol Immunopathol 1993;67(3 Pt 2):S2–S15.
  4. Forehand JR, Nauseef WM, Curnutte JT, Johnston RB Jr: Inherited disorders of phagocytic killing; in Scriver CR, Beaudet AL, Sly WS, Valle D (eds): The Metabolic and Molecular Bases of Inherited Disease. New York, McGraw-Hill, 1995, vol 7, pp 3995–4026.
  5. Gallin JI, Buescher ES, Seligmann BE, Nath J, Gaither T, Katz P: NIH Conference. Recent advances in chronic granulomatous disease. Ann Intern Med 1983;99:657–674.
  6. Johnston RB Jr, Newman SL: Chronic granulomatous disease. Pediatr Clin North Am 1977;24:365–376.
  7. Quie PG, White JG, Holmes B, Good RA: In vitro bactericidal capacity of human polymorphonuclear leukocytes: Diminished activity in chronic granulomatous disease of childhood. J Clin Invest 1967;46:668–679.
  8. Segal BH, Leto TL, Gallin JI, Malech HL, Holland SM: Genetic, biochemical, and clinical features of chronic granulomatous disease. Medicine (Baltimore) 2000;79:170–200.
  9. Babior BM, Takeuchi C, Ruedi J, Gutierrez A, Wentworth P Jr: Investigating antibody-catalyzed ozone generation by human neutrophils. Proc Natl Acad Sci USA 2003;100:3031–3034.
  10. Winkelstein JA, Marino MC, Johnston RB Jr, Boyle J, Curnutte J, Gallin JI, et al: Chronic granulomatous disease: Report on a national registry of 368 patients. Medicine 2000;79:155–169.
  11. Aghamohammadi A, Moin M, Farhoudi F, Pourpak Z, Rezaei N, Abolmaali K, et al: Primary immunodeficiency in Iran: First report of national registry of PID in children and adults. J Clin Immunol 2002;22:375–380.
  12. Johnston RB Jr: Clinical aspects of chronic granulomatous disease. Curr Opin Hematol 2001;8:17–22.
  13. Barbouche MR, Sghiri R, Mellouli F, Boukhdir Y, Dellagi K, Bejaoui M: Chronic septic granulomatous disease. 14 cases. Presse Méd 1999;28:2034–2036.
  14. Hasui M: Chronic granulomatous disease in Japan: Incidence and natural history. The Study Group of Phagocyte Disorders of Japan. Pediatr Int 1999;41:589–593.
  15. Roos D, de Boer M, Kuribayashi F, Meischl C, Weening RS, Segal AW, et al: Mutations in the X-linked and autosomal recessive forms of chronic granulomatous disease. Blood 1996;87:1663–1681.
  16. Johnston RB Jr, Baehner RL: Chronic granulomatous disease: Correlation between pathogenesis and clinical findings. Pediatrics 1971;48:730–739.
  17. Ahlin A, De Boer M, Roos D, Leusen J, Smith CIE, Sundin U, et al: Prevalence, genetics and clinical presentation of chronic granulomatous disease in Sweden. Acta Paediatr 1995;84:1386–1394.
  18. Mouy R, Fischer A, Vilmer E, Seger R, Griscelli C: Incidence, severity and prevention of infections in chronic granulomatous disease. J Pediatr 1989;114(4 Pt 1):555–560.
  19. Weening RS, Adriaansz LH, Weemaes CMR, Lutter R, Roos D: Clinical differences in chronic granulomatous disease in patients with cytochrome b-negative or cytochrome b-positive neutrophils. J Pediatr 1985;107:102–104.
  20. Quie PG, Mills EL, Roberts RL, Noya FJD: Disorders of the polymorphonuclear phagocytic system; in Stiehm ER (ed): Immunologic disorders in infants and children. Philadelphia, Saunders, 1996, pp 453–462.
  21. Tauber AI, Borregaard N, Simons E, Wright J: Chronic granulomatous disease: A syndrome of phagocyte oxidase deficiencies. Medicine (Baltimore) 1983;62:286–309.
  22. Finn A, Hadzic N, Morgan G, Strobel S, Levinsky RJ: Prognosis of chronic granulomatous disease. Arch Dis Child 1990;65:942–945.
  23. Kobayashi Y, Komazawa Y, Kobayashi M, Matsumoto T, Sakura N, Ishikawa K, Usui T: Presumed BCG infection in a boy with chronic granulomatous disease. Clin Pediatr (Phila) 1984;23:586–589.
  24. Smith PA, Wittenberg DF: Disseminated BCG infection in a child with chronic granulomatous disease. S Afr Med J 1984;65:821–822.
  25. Lau YL, Wong SN, Lawton JW, Chow CB: Chronic granulomatous disease: A different pattern in Hong Kong? J Paediatr Child Health 1991;27:235–239.
  26. Gonzalez B, Moreno S, Burdach R, et al: Clinical presentation of Bacillus Calmette-Guérin infections in patients with immunodeficiency syndromes. Pediatr Infect Dis J 1989;8:201–206.
  27. Pogrebmoal JW, Gallin JI, Malech HL, Baker AR, Moskaluk CA, Travis WD, Pass HI: Surgical management of pulmonary infections in chronic granulomatous disease. Ann Thorac Surg 1993;55:844–849.
  28. Global Tuberculosis Control. WHO Reports 1996–2002. TB Situation in the Region: Islamic Republic of Iran. Presentation during the National Managers of the Tuberculosis Control Programmes in the Eastern Mediterranean Region. Rabat, June 17–19, 2003.
  29. Chudwin DS, Wara DW, Cowan MJ, Ammann AJ: Aspergillus pneumonia in chronic granulomatous disease: Recurrence and long-term outcome. Acta Paediatr Scand 1982;71:915–917.
  30. Cohen MS, Isturiz RE, Malech HL, Root RK, Wilfert CM, Gutman L, Buckley RH: Fungal infection in chronic granulomatous disease. The importance of the phagocyte in defense against fungi. Am J Med 1981;71:59–66.
  31. Eppigner TM, Greenberger PA, White DA, Brown AE, Cunningham-Rundles C: Sensitization to Aspergillus species in the congenital neutrophil disorders in chronic granulomatous disease and hyper IgE syndrome. J Allergy Clin Immunol 1999;104:1265–1272.
  32. Perry HB, Boulanger M, Pennoyer D: Chronic granulomatous disease in an adult with recurrent abscesses. Arch Surg 1980;115:200–202.
  33. Lublin M, Bartlett DL, Danforth DN, Kauffman H, Gallin JI, Malech HL, et al: Hepatic abscess in patients with chronic granulomatous disease. Ann Surg 2002;235:383–391.
  34. Dickerman JD, Colletti RB, Tampas JP: Gastric outlet obstruction in chronic granulomatous disease of childhood. Am J Dis Child 1986;140:567–570.
  35. Johnson FE, Humbert JR, Kusela DC, Todd JK, Lilly JR: Gastric outlet obstruction due to X-linked chronic granulomatous disease. Surgery 1975;78:217–223.
  36. Margolis DH, Melnick DA, Alling DW, Gallin JI: Trimethoprim-sulfamethoxazole prophylaxis in the management of chronic granulomatous disease. J Infect Dis 1990;162:723–726.
  37. Weening RS, Kabel P, Pijman P, Roos D: Continuous therapy with trimethoprim-sulfamethoxazole in patients with chronic granulomatous disease. J Pediatr 1983;103:127–130.
  38. The International Chronic Granulomatous Disease Cooperative Study Group: A controlled trial of interferon gamma to prevent infection in chronic granulomatous disease. N Engl J Med 1991;324:509–516.
  39. Gallin JI, Alling DW, Malech HL, Wesley R, Koziol D, Marciano B, et al: Itraconazole to prevent fungal infections in chronic granulomatous disease. N Engl J Med 2003;348:2416–2422.
  40. Liese JG, Jendrossek V, Jansson A, Petropoulou T, Kloos S, Gahr M, Belohradsky BH: Chronic granulomatous disease in adults. Lancet 1996;347:220–223.

Article / Publication Details

First-Page Preview
Abstract of Original Paper

Received: November 03, 2003
Accepted: February 25, 2004
Published online: June 28, 2004
Issue release date: July 2004

Number of Print Pages: 7
Number of Figures: 2
Number of Tables: 0

ISSN: 1018-2438 (Print)
eISSN: 1423-0097 (Online)

For additional information: https://www.karger.com/IAA


Copyright / Drug Dosage / Disclaimer

Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.