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

Role of quantiFERON-TB test in detection of children infected with mycobacterium tuberculosis

Latent TB infection can persist for many years with about 10% lifetime risk of reactivation to active disease. However, in children with latent TB infection, disease develops within 2 years of infection. Recently, a new diagnostic test [QuantiFERON-TB Gold] which measures the production of interferon [IFN] gamma in whole blood upon stimulation with Mycobacterium tuberculosis has been introduced. The aim of this study is to compare the performance of the IFN-gamma assay with tuberculin skin test [TST] for the identification of latent TB infection in children in contact with active TB in the pediatric pulmonary ward. This cross-sectional study was conducted on 100 children, aged 2months-15 years admitted to the Pediatric Ward of Masih Daneshvari Hospital during 2007-2008. Whole blood was collected for measuring Interferongamma using QuantiFERON-TB Gold kit [QFT-Cellestis Comp]. In this procedure, Mycobacterium tuberculosis specific antigens [ESAT-6 and CFP-10] are used. In the present research, 100 children were studied and divided into 3 groups of case [TB], contact and control. PPD test was performed by injecting 0.1 ml of the 5 unit solution [Pasteur Institute of Iran] for all cases. Twenty-eight percent of the contacts, 60% of the cases and 10% of the controls were Afghans; the remaining were Iranians. Smear of the gastric washing [3X] was prepared in contact and case [TB] groups; 30% of the cases [TB] were AFB positive, while all of the contacts had negative smears. History of BCG vaccination during neonatal period and BCG scar were present in all cases. Positive PPD test [PPD >/= 10 mm] was observed in 90% of the cases and 24% of the contacts. PPD test was negative in the control group. Out of 50 contacts, 18 [36%] showed positive QFT test; and of 20 TB patients, 18 [90%] had positive tests. Regarding age, children with positive QFT test belonged to the older age group. To our knowledge, this is the first study to investigate the performance of the whole blood IFN-gamma assay in diagnosing latent TB infection in children in Iran. This study found a fair correlation between the TST and the whole blood IFN-gamma assay in children at high risk of latent TB infection. Our study also highlighted fair and moderate agreement in contact and TB groups respectively between the TST and QFT-TB test in children at high risk for latent TB infection. More studies are required to clarify this relationship

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

Bacteriological evaluation for diagnosis of tuberculosis in children

During the last decade of the 20th century, the number of new cases of tuberculosis [TB] in children increased worldwide. Pulmonary TB in adults is diagnosed by isolation of Mycobacterium tuberculosis .In children; the diagnosis is based on diagnostic criteria and characteristics of the disease. The aim of this study was to evaluate bacteriological specimens from children with TB hospitalized in the pediatric tuberculosis ward of Masih Daneshvari Hospital. We assessed the results of smears, cultures and polymerase chain reaction [PCR] of gastric aspirates for confirmation of clinical findings. A descriptive study was performed on126 medical records of children with TB during a 5-year period. Demographic data including age and gender, pulmonary and extra-pulmonary TB presentations and gastric washing smear, culture and PCR were collected and then analyzed by SPSS software. The study patients were divided into three groups of age: 0-5 yrs, 6-10 yrs and 11-15 yrs. The highest frequency [68.3%] was observed in the 11-15 years age group; 47.6% of the patients were males and 52.4% were females. The tuberculin skin test was positive in 73% of patients. Gastric aspirate smears, culture and PCR were positive for Mycobacterium tuberculosis in 55.6%, 58.7% and 53.2% of cases, respectively. Computed tomography [CT] scan showed evidence of TB in 94.4% of patients. In 34 patients, chest x-ray was normal and TB was diagnosed via CT-scan of the lung. The present study suggests that gastric lavage smears and cultures have high diagnostic value in TB diagnosis in children. In addition, chest CT-scan is recommended for diagnosis of TB in suspected children when other evaluations are normal

Efficacy and safety of inhaled steroids in children with asthma: a comparison of fluticasone propionate with beclomethasone

Inhaled corticosteroids are indicated in children who have mild persistent asthma. Fluticasone propionate is a newer corticosteroid agent with higher potency compared with previous generations. However, still few dose-ranging studies have been investigated for optimal dosing of inhaled corticosteroids particularly in children with regard to the tolerability and safety of the drug. The primary purpose of this study was to compare and evaluate the efficacy and safety of fluticasone with beclomethosone in the treatment of childhood asthma unresponsive to non-steroidal medications and also in persistent, moderate and severe asthma. Seventy children, aged 6 to 14 years were enrolled in an open randomized trial with a parallel group design. Fiftytwo children with moderate, severe or persistent asthma received fluticasone 100 micro g twice daily for 12 weeks compared with 18 asthmatic children on beclomethasone 200 micro g daily. The outcome was assessed by data on questionnaires, changes in clinical symptoms, and results of peak flowmetery [PEFR]. Moreover, safety was assessed by 24 hour urinary cortisol measurement at the beginning of the study and comparison of the data with urinary cortisol at the end of 12 weeks. A total of 70 children between 6 to 14 years [33 girls and 37 boys] were randomized to start treatment with fluticasone or beclomethasone. From 70 children 13[18.6%] had a history of contact with pets during their life. At the beginning in beclomethasone group: 88.9% had cough, 88.9% had post exercise cough, 66.7% had dyspnea and 72.2% had wheezing. In Fluticasone group: 75% had cough,76.9% had post exercise cough, 46.2% had dyspnea and 59.6% had wheezing. After 3 months of therapy in beclomethasone group: cough was seen in 16.7%, post exercise cough in 11.1%, dyspnea in 11.1%, wheezing in 16.7% and in fluticasone group: cough in 15.4%, post exercise cough in 11.1%, dyspnea in 1.9% and wheezing in 3.8%.Data showed a better improvement in clinical signs of patients with fluticasone [p < 0.05]. Pulmonary function tests revealed better lung function in fluticasone group [p < 0.05]. In addition, 24 hours urinary cortisol level was measured at the beginning and after 12 weeks of therapy and it was within the normal range for both drugs. Fluticasone produced significantly greater improvement in lung function and control of asthma symptoms compared to beclomethasone and is efficient in the treatment of persistent, moderate and severe asthma in children. In addition these improvements were achieved with no greater degree of cortisol suppression compared with beclomethasone

Prevalence of tuberculosis in close contacts smear positive TB patients

Tuberculosis [TB] is considered as one of the main causes of mortality and morbidity in developing countries. At present, extensive contact investigation among households is not a routine part of TB control efforts in most countries. The investigation of contacts of TB cases is an essential part of TB control program. In this study, we have evaluated the active contact tracing among close contacts of smear positive TB patients. The main aim of this research was to demonstrate the prevalence of TB and to fully screen the close contacts of TB patients in order to detect active and infected TB cases. Close contacts of newly diagnosed smear positive pulmonary TB patients were identified. The information and data of the contacts including history, clinical examination, history of BCG vaccination, results of tuberculin skin test [TST], and bacteriological and radiological manifestations were collected on special questionnaires. Out of the total 147 close contacts of 34 index cases, 81 [55.1%] were female and 66 [44.9%] were male. Based on the nationality, there were 38 [25.9%] Afghan refugees and 109 [74.1%] Iranian cases. The duration of contact was as follows: in 30 cases the contact time period was less than 1 yr., in 75 individuals it was more than 1 yr., while 42 individuals had a persistent contact. In 61.8% of the cases the induration of TST was less than 15 mm, while in 38.2% it was more than 15 mm. Abnormal radiological manifestations were detected in 33.3% of close contacts including calcification [25.17%], parenchymal infiltration [4.08%], cavity [2.04%] and nodular lesions [2.04%]. According to the diagnostic findings, out of 147 contacts, 7 [4.8%] cases of pulmonary TB were detected. In our study no significant statistical difference was found regarding the prevalence of TB among the Iranian and Afghan close contacts. Also there was no significant statistical difference in the duration of contact time of the TB cases. Furthermore, positive radiological findings were detected in 33.3% of the close contacts. In 25.7% of the cases, the pattern was calcification indicating primary TB infection. According to the results of this research the rate of TB in contacts is 400 times higher than the society [4800/100,000 versus 12/100,000]. This study points out the importance of systematic investigation of contacts to discover new cases of TB. It also demonstrates the significant role of radiology as a major tool in diagnosing both TB infection and disease

Mycobacterium thermoresistibile infection in a child

Mycobacterium thermoresistibile was first reported in 1981 as a human pathogen. Several studies have reported pulmonary infection and cutaneous lesions due to this type of mycobacterium. A five-year-old boy with cough, fever, and abdominal pain was referred to Masih Daneshvari Hospital. He had been treated with diagnosis of histiocytosis x. Gastric lavage was performed and examined by polymerase chain reaction [PCR] and Mycobacterium thermoresistibile was found. It seems that this case is the first report of an atypical tuberculosis caused by Mycobacterium thermoresistibile in a child

Renal tuberculosis in a four-year-old child

Tuberculosis of the genitourinary tract is one of the late reactivation or complications of pulmonary tuberculosis which is mostly prevalent in young adults and middle-aged people. Renal tuberculosis is rare in children. We present a 4 year-old Afghan girl suffering from pulmonary and renal tuberculosis. The child presented to the hospital with severe malnutrition, fever and cough which initiated a month earlier. She had a history of close contact with her mother who was a smear positive TB patient. Radiologic findings in her chest x-ray were pulmonary infiltration in the right lower lobe along with right hilar adenopathy. The diagnosis of pulmonary tuberculosis was made based on the systemic and pulmonary signs and symptoms as well as the bacteriologic examinations. Anti-tuberculosis drugs were given to the child and after obtaining intravenous pyelography [IVP], voiding cystourethrogram [VCUG] and kidney CT-scan; the diagnosis of renal tuberculosis in addition to pulmonary tuberculosis was made. Urinary symptoms were treated by anti-tuberculous therapy

Foreign body in children's airways: a five -year study

Foreign body aspiration continues to be a major problem and one of the most important reasons for mortality and morbidity particularly sudden death among children. Therefore, this study was conducted to evaluate the clinical and paraclinical features and also the treatment of children who admitted in this center as the result of foreign body aspiration and underwent bronchoscopy to remove aspirated material. This was a cross sectional study conducted on children under the age of 15 who admitted and registered in NRITLD with the diagnosis of foreign body aspiration and underwent bronchoscopy. Children were analyzed based on their age, gender, primary diagnosis, characteristic of the foreign body, the interval between aspiration and starting treatment, radiological findings, and the severity of airway injuries. Forty-seven children under the age 15 were evaluated in this study in a five-year period between 1998 and 2004. Sixty-three percent of them were boy and 37% were girl. Thirty [63%] children were found to be younger than 3 years old. Moreover in 63% of the cases the primary diagnosis before referring to this center was not foreign body aspiration. Hence, as the result of misdiagnosis and delay, 50% of children had injuries in their airways. The most common aspirated foreign body was organic materials [82%] and the most common radiological finding was hyperinflation in the chest x-ray. This study showed that early diagnosis and treatment of foreign body is a critical factor to prevent further airway complications in children. According to the results of this study ragid bronchoscopy is the most effective procedure for treatment of foreign body aspiration in children

Infantile histiocytosis X: Case reoprt

Langerhans cell histiocytosis [LCH] is a disease of unknown etiology that presents in three forms: Letterer- Siwe disease, Hand-Schuller-Christian disease, and Eosinophilic Granuloma. It is a multifocal disease that usually affects several organs and unifocal forms are rare. Pulmonary involvement occurs in approximately 40% of cases and almost always in young adults. Lungs are rarely affected in infant patients. This report presents a 15-month-old male infant that was admitted for cyanosis and respiratory distress. Diagnostic work up revealed a primary pulmonary histiocytosis and the treatment made a significant improvement of signs and symptoms by the time of discharge