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.

Evaluation of facial anthropometric parameters in 11-17 year old boys.

BACKGROUND AND OBJECTIVE: Anthropometry is the study of qualitative specifications based on linear and angular measurements of human body. The aim of the present study was to determine anthropometric parameters of 11-17 year old boys of northeast Iran. METHOD: This cross-sectional analytical study was conducted on 583 boys of Fars ethnicity living in Mashhad with Class I skeletal and dental relationships. Digital photographs in natural head position were transferred to a computer and the desired anthropometric landmarks were traced on each image. Anthropometric parameters including the width of the forehead, the width of the face, the width of the cranial base, intergonial width, intercanthal width, binocular width, nasal width, mouth width, facial and nasal height and depth of superior, middle and inferior one-third of the face were measured by "Smile Analyzer" software. ANOVA, Tukey test, and linear regression were used for statistical analysis. RESULTS: Most of the parameters studied increased gradually with age. A growth spurt was evident at the ages of 15-16 years old for binocular width, nasal width, nasal height and depths of middle and inferior one-third of the face. Facial height was among some parameters which were found to increase slightly after 16. Unlike the other parameters, intercanthal width showed an irregular pattern of changes and statistical analysis did not show any significant differences among different age groups (P-value = 0.362). CONCLUSION: Aging of the face occurs in spurts and at different periods of life. During the studied time span, significant growth in most anthropometric parameters except intercanthal width was obvious.

Epidemiology and control of rabies in Iran.

Rabies is endemic in Iran. It is the most important zoonotic disease in the country. Based on studies undertaken during the past few decades, there is evidence that the main reservoir for rabies is wolves. The incidence of rabies in humans and animals is increasing each year. In 2006, more than 130,000 people received post-exposure prophylaxis. Similar figures apply regarding preventive vaccination in animals. Official data suggest that the majority of human exposures are due to biting episodes caused by dogs. However, investigations performed by this team showed that in many cases wolves were responsible for rabies transmission to humans. Because of the potential feasibility of oral rabies vaccination, this team has proposed a pilot study to evaluate the use of an oral rabies vaccine for vaccination of wildlife to control rabies in Iran.

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

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