Evaluation of Patient Follow-up with Transient Hypogammaglobulinemia in Infancy Diagnosis

Öz

Introduction: In this study, we aimed to investigate the clinical, laboratory and demographic characteristics of patients with THI who were admitted with the suspicion of recurrent infection and immunodeficiency.

Methods: The study included 287 patients who were followed up with the diagnosis of transient hypogammaglobulinemia in infancy at Selçuk University Meram Faculty of Medicine, Child Health and Diseases Department and Department of Pediatric Allergy and Immunology, between November 2001 and November 2006. The age, gender, the age of the complaints and diagnosis, clinical features, family history and laboratory findings of the patients were examined. The data of the patients were collected by using the hospital file and the records in the Pediatric Immunology cards.

Results: 195 (67.9%) of the patients were male and 92 (32.1%) were female (p <0.05). The M/F ratio was 2.1. The age of diagnosis ranged from 8 months to 48 months with a mean of 24.6±11.3 months. The duration between the complaints and the age of diagnosis was between 1 and 63 months and the mean age was 13.5±8.6 months. Complaints of the patients were recurrent upper respiratory tract infection (URTI) 54.35% (n:156), lower respiratory tract infection (LRTI) 50.52% (n:145), sinusitis 41.26% (n:118), recurrent otitis, 28.22% (n:81), asthma finding 12.89% (n=37), allergic skin and rhinitis findings 10.45% (n:30), gastroenteritis 6.96% (n:20), urinary tract infection 4.18% (n:12), and moniliazis was 3.83% (n:11).

Conclusion: THI is usually a self-recovering disease around three years of age. In order to prevent complications such as chronic lung disease and to reduce morbidity, it is important to follow up the cases regularly.

Anahtar Kelimeler

• Transient hypogammaglobunemia,immunodeficiency

Kaynakça

1. REFERENCES1. Gitlin D, Janeway CA: Agammaglobulinemia: Congenital, acquired and transient forms. Prog Hematol 1956; 1: 318.
2. 2. Ochs HD, Stiehm ER, Winkelstein JA. Antibody deficiency. In: Stiehm ER, Ochs HD, Winkelstein JA, (eds). Immunologic Disorders in Infants and Children. 5th edition: Philadelphia. Elsevier Saunders;2004.p.357-73.
3. 3. Wilson CB, Lewis DB, Penix LA: The physiologic immunodeficiency of immaturity. In Stiehm ER(ed): Immunologic Disorders in Infants and Children. Philadelphia, WB Saunders, 1996,p:253.
4. 4. Dalal I, Reid B, Nisbet-Brown E, et al: The outcome of patients with hypogammaglobulinemia in infancy and early childhood. J Pediatr 1998; 133: 144-6.
5. 5. McGeady SJ: Transient hypogammaglobulinemia of infancy: Need to reconsider name and definition. J Pediatr 1987; 47: 110.
6. 6. Tiller TL, Buckley RH: Transient hypogammaglobulinemia of infancy: Review of the literature, clinical and immunologic features of 11 new cases, and long-term follow-up. J Pediatr 1978; 92: 347.
7. 7. Primary immunodeficiency diseases. Report of an IUIS Scientific Committee. International Union of Immunological Societies. Clin Exp Immunol 1999; 118 (Suppl 1): 1-28.
8. 8. Luzi G, Pesce AM, Rinaldi S: Primary immunodeficiencies in Italy. Data revised from the Italian Register of Immunodeficiencies-IRID (1977-1988). Immunol Clin 1989; 8(1): 45-54.

9. 9. Ryser O, Morell A, Hitzig WH: Primary immunodeficiencies in Switzerland: First report of the national registry in adults and children. J Clin Immunol 1988,8(6): 479-85.
10. 10. Affentranger P, Morell A, Spath P, Seger P: Registry of primary immunodeficiencies in Switzerland. Immunodeficiency 1993; 4: 193-5.
11. 11. Siegel RL, Issekutz T, Schwaber J, et al: Deficiency of T helper cells in transient hypogammaglobulinemia of infancy. N Engl J Med 1981; 305: 1307.
12. 12. Kowalczyk D, Mytar B, Zembala M: Cytokine production in transient hypogammaglobulinemia and isolated IgA deficiency. J Allergy Clin Immunol 1997;100: 556.
13. 13. Francisco A, et al. Update on primary immundeficiency diseases. J Allergy Clin Immun 2006;117: 435-41.
14. 14. Stiehm ER, Ochs HD, Winkelstein JA: Immunologic disorders: general consideration. In Immunologic Disorders in Infants and Children, ER Stiehm, HD Ochs, JA Winkelstein (eds). USA, Elsevier Saunders, 2005, pp 289–355.
15. 15. Bittles AH, Mason WM, Greene J, Rao NA: Reproductive behavior and health in consanguineous marriages. Science 1991; 252: 789-94.
16. 16. Bittles A: Consanguinity and its relevance to clinical genetics. Clin Genet 2001;60: 89-98.
17. 17. Dressler F, Peter HH, Muller W, et al: Transient hypogammaglobulinemia of infancy: Five new cases, review of the literature and redefinition. Acta Paediatr Scand 1989;78: 767.
18. 18. Walker AM, Kemp AS, Hill DJ, et al: Features of hypogammaglobulinemia in infants screened for immunological abnormallities. Arch Dis Child 1994;70: 183.
19. 19. Kılıç SS, Tezcan I, Sanal Y, Metin A, Ersoy F. Transient hypogammaglobulinemia of infancy: clinical and immunological features of 40 new cases. Pediatr Int 2000;42: 647-50.
20. 20. Kutukculer N, Aksu G. Frequency of primary immundeficiencies diagnosed in 10 years in a pediatric immunology department in turkey (480 cases). XIIth Meeting of the European Society for Immunodeficiencies (ESID). 4-7 october 2006, Budapest, Hungary. p:229.
21. 21. Dogu F, İkinciogulları A, Babacan E. Transient hypogammaglobulinemia of infancy and early childhood: outcome of 30 cases. Turk J Pediatr 2004;46: 120-4.
22. 22. Fineman SM, Rosen FS, Geha RS: Transient hypogammaglobulinemia, elevated immunglobulin E levels and food allergy. J Allergy Clin Immunol 1979;64: 216.
23. 23. Vetrie D, Vorechovsky I, Sideras P, et al: The gene involved in X-linked agammaglobulinemia is a member of the src family of protein-tyrosine kinases. Nature 1993;361: 226.
24. 24. Cano F, Mayo DR, Ballow M: Absent specific viral antibodies in patients with transient hypogammaglobulinemia of infancy. J Allergy Clin Immunol 1990;85: 510.