Long-term follow-up of ninety eight Iranian patients with primary immune deficiency in a single tertiary centre.

PURPOSE: The aim was to describe the clinical manifestations, complications and long-term outcome of a cohort of Iranian patients with primary immune deficiency (PID). METHOD: We retrospectively studied the demographic, clinical and immunological characteristics of the PID patients in a single tertiary centre, from January 1989 to July 2014. The patients were classified according to the International Union of Immunological Societies Expert Committee on PID. RESULTS: 98 patients were diagnosed with and followed-up for 15 disorders. The mean age at onset and diagnosis and the diagnostic delay were 8±10, 14.2±13.1 and 6.1±7 years, respectively. Parental consanguinity rate was 57%. Predominantly Antibody Deficiency was the most common diagnosis (n=63), followed by congenital defects of phagocytes (n=16), combined immunodeficiencies (n=12), well defined syndromes (n=4) and defects in innate immunity (n=3). Recurrent sinopulmonary infection was the most common presentation. Active infections were treated appropriately, in addition to prophylactic therapy with IVIG and antimicrobials. Not all the patients were compliant with prophylactic regimens due to cost and unavailability. One SCID patient underwent successful bone marrow transplantation. The total mortality rate was 19% during the follow-up period (7.8±7.6 years). The mean age of living patients at the time of study was 23±11.7 years. CONCLUSIONS: Physicians awareness of PID has been rising dramatically in Iran, ensuring an increasing number of patients being diagnosed and treated. More effective treatment services, including health insurance coverage and drug availability are needed to improve the outcome of PID patients.

Auditory electrical tinnitus suppression in patients with and without implants.

The aim of this study was to evaluate the effectiveness of electrical tinnitus suppression in two groups of chronic severe tinnitus sufferers. Through standard tinnitus questionnaires, we compared the effectiveness of extratympanic and intratympanic auditory electrical stimulation (AES) by cochlear implants (CI) for the suppression or abolition of the perception of tinnitus and the decrease of its associated complaints. We made otolaryngological and comprehensive audiological assessment and also tinnitus measurement in each group of patients before and after AES and 50 days later. We investigated the dimensions of psychological complaints due to chronic and disabling tinnitus by means of the tinnitus questionnaire (TQ). The control examination during at least seven sessions (50 days) after AES in the group of patients without implants showed improvement in 20 of 32 patients (62.5%); 12 (37.5%) did not notice any change. In the comparative group of patients with implants, improvement occurred in 16 of 20 patients (75%); during the switch-on of the speech processor, these patients reported significant attenuation or complete suppression of their tinnitus. Complete suppression of the tinnitus after CI was observed for 11 patients (55%), and 5 patients (25%) demonstrated significant attenuation of tinnitus. Nonsuppression of tinnitus was observed for only 4 patients (25%). None of our patients was affected by an increment in the tinnitus owing to CI. The differences of means of scores in the standard TQ were significant in both groups of patients. A comparison of TQ score differences between patients with and without implants showed no significance. We concluded that AES is a useful and effective therapeutic intervention in patients with tinnitus. Extratympanic AES reduces the effects of the tinnitus but presents limitations, mainly owing to the short duration of the electrical residual inhibition of the tinnitus. CI is shown to be more efficient for the treatment of tinnitus, mainly because the electrical stimulation affects a wider area of the cochlea and is presented for longer sessions. Therefore, patients affected by incapacitating tinnitus should be considered for continuous use of electrical stimulation.