Primary immunodeficiency in children: report of seven years study

Primary immunodeficiency diseases [PIDs] are a genetically heterogeneous group of disorders that affect distinct components of the innate and adaptive immune system, such as neutrophils, macrophages, dendritic cells, complement proteins, natural killer cells, T and B lymphocytes. These disorders are rare, with an estimated prevalence of 1:10,000 live births. This study aimed at describing the clinical features, disease complications, treatment modalities and overall outcome of patients with Primary Immunodeficiency Diseases [PID] in Masih Daneshvari hospital during a 7-year period [2001-2008]. This was a retrospective study based on the review of patients' medical records. Clinical, laboratory, and epidemiological data including personal and family history were obtained by reviewing records of patients admitted to the Pediatric Pulmonary Ward of NRITLD, a referral center for tuberculosis and lung diseases. The diagnosis was made based on WHO criteria for primary immunodeficiency disorders. Data collected from 59 patients were evaluated and analyzed. There were 35 [59.3%] males and 24 [40.69%] females. The age of patients ranged from 6 months to 14.5 years and the mean age was 7.4 years. Positive family history was detected in 20 [33.9%] cases and parents of 36 patients [61.2%] were consanguineous. Twenty patients [33.9%] had a family history of PID. Phagocytic disorder [57.2%] was the most common form of PID, followed by antibody deficiency [33.7%] and T-cell or combined deficiency [8.2%]. No case of complement deficiency was detected. In this group of under study patients, 2 cases expired as the result of respiratory failure due to drug resistant pneumonia [chronic granulomatous disease cases]. Based on studied results, Phagocytic disorders [57.2%] were the most common disorders among our PID patients. This may be due to the large number of CGD patients referred with the pathologic finding of granuloma misdiagnosed with tuberculosis. Considering the high prevalence of PID in this study, cases with unusual, chronic, severe or recurrent infections should be evaluated for immunodeficiency disorders

Middle lobe syndrome in children

Atelectasis of the middle lobe or lingula of the lung is defined as middle lobe syndrome. On chest x-ray it is demonstrated as a wedge shaped density with anterior-inferior extension from the hilum. Although many etiologies have been implicated, this syndrome is one of the most common complications of asthma. A simple descriptive study was conducted on 11 patients with an age range of 0-18 yrs. They were admitted to Masih Daneshvari Hospital during 2000-2007 with the diagnosis of lingula or middle lobe atelectasis [of more than one month duration] and/ or recurrent consolidation [2 times or more]. The study group consisted of 6 boys [54.5%] and 5 girls [45.5%]. All patients were clinically symptomatic at the time of admission. Cough was the chief complaint [7patients, 63.6%]. The mean age at the time of initial diagnosis was 7.3 yrs [SD: 1.6].The most common findings on pulmonary CT-scan were infiltrations [3 cases, 27.3%] and atelectasis [3 cases, 27.3%]. Non-obstructive causes were the most frequent etiologies which included asthma [n=3, 27.3%], pneumonia [n=2, 18.2%] and bronchiectasis [n=2, 18.2%].Among the obstructive causes, an undefined tumor [1 case, 9.1%] was to mention. Nine cases [81.8%] had negative blood cultures and 9 cases [81.8%] had AFB negative sputum smears [3x]. Bronchoscopy was performed in 4 [36.4%]; which showed rapid improvement after fiberoptic bronchoscopy [FOB]. Medical treatment was planned for 9 children who demonstrated quick recovery. Surgery [lobectomy] was conducted in only 1 patient. Patients with right middle lobe syndrome [RMLS] had airway hypersensitivity, which is supported by the fact that asthma is very severe in this group of patients. Despite its low incidence, it should be considered very carefully and cautiously since it is associated with many severe complications. Therefore in undiagnosed suspected cases, in addition to a meticulous history taking, detailed diagnostic and therapeutic measures are recommended