[Survey of serum fat-soluble vitamins, zinc, copper and selenium levels in patients with cystic fibrosis]

Cystic fibrosis [CF] is a genetically disease with different respiratory and gastrointestinal organ involvement. Because of gastrointestinal tract involvement, patients have mal-absorption for vitamins and mineral elements; for example vitamin A, D, E and trace elements such as Selenium [Se], Zinc [Zn] and Copper [Cu] have low serum level in CF patients in different studies. The goal of this study is to define relation between serum level of this elements and severity of respiratory and gastrointestinal tract involvement in these patients. This cross sectional study performed on 39 cystic fibrosis patients admitted in pediatric ward of Masih Daneshvari hospital, Tehran, Iran. Serum levels of Vitamin A, D and E, Selenium, Zinc and Copper deficiency were studied. Serum level of Vitamin A and D were lower than normal values. Also, 3 cases [7.68%] had serum level of Zinc lower than normal values. CF patients are susceptible to have deficiencies of fat soluble vitamins and trace elements such as Selenium and Zinc because of mal-absorption. It seems that a perfect diet and nutritional components are helpful in prevention of these problems

Correlation of anxiety-depression and sleep quality in mothers of children with cystic fibrosis and asthma

Cystic fibrosis and asthma are considered among the chronic respiratory diseases. Taking care of the sick child by the mother-which is usually the main care taker- can be associated with high loads of stress and result in behavioral problems like anxiety, depression and change in sleep quality. This study aimed at evaluating the correlation between depression anxiety and sleep quality in mothers of children suffering from cystic fibrosis and asthma hospitalized in Masih Daneshvari Hospital. This was an analytical descriptive cross-sectional study conducted on 148 subjects [mothers of children with cystic fibrosis and asthma hospitalized in Masih Daneshvari Hospital] during 2008-2010. Data were collected using a questionnaire for demographic characteristics, sleep quality and Hospital Anxiety and Depression Scale [HADS]. Pittsburgh sleep quality index questionnaire [PQSI] was developed by Dr. Buysse and colleagues at the University of Pittsburgh's Western Psychiatric Institute and Clinic in the late 1980s. The PSQI was created after observing that most patients with psychiatric disorders had sleep disorders as well. Also, required data regarding the pulmonary function of patients was extracted from their medical records. In this study, high levels of anxiety and depression and poor sleep quality requiring clinical intervention were seen in 37.2%, 29.1% and 39% of mothers, respectively. A significant association was detected between sleep quality and depression-anxiety [P-value<0.005]. Also, depression-anxiety was significantly correlated with number of children and smoking [P-value<0.005]. A total of 20% of mothers suffering from depression and 14.3% of mothers with anxiety disorder had a history of cigarette smoking [P-value<0.005]. No significant association was found between substance abuse and occupation with depression-anxiety. Susceptibility was only correlated with anxiety [P-value<0.005]. Our study showed a significant association between sleep quality and depression-anxiety in mothers of children suffering from cystic fibrosis and asthma. Greater attention should be paid to prevent development and aggravation of these conditions in susceptible mothers who are the main care taker of their sick child with chronic diseases

Mediastinal lymphangioma in a child

We report a case of a male child with a cystic mass in his left side of the neck with extension to the mediastinum. This article highlights the clinical and paraclinical findings and management of these cases. In conclusion, it is necessary to evaluate the mediastinum for extension of the cyst in cases with cystic hygromas of the neck. Surgical resection of the tumor through a cervical incision can be considered

correlation of brody high resolution computed tomography scoring system with clinical status and pulmonary function test in patients with cystic fibrosis

To reduce the mortality and morbidity rates of cystic fibrosis [CF] patients, and to have an effective clinical management, it is important to monitor the progression of the disease. The aim of this study was to evaluate the progression of lung disease in CF patients by means of assessing the correlation of the CT scoring system with clinical status and pulmonary function test at the Pediatric Pulmonary Ward of Masih Daneshvari Hospital in 2008. Pulmonary high resolution computed tomography [HRCT] was performed in 23 CF patients using the Brody's scoring system. Morphologic signs as well as the extent and severity of each sign were scored, and the total sore was calculated. The correlation of HRCT scores[total score as well as the score for each parameter] with Shwachman Kuczycki scoring system and pulmonary function test were examined. The study included 9 female and 14 male patients with an age range of 5-23 years [mean: 13.42 years]. Bronchiectasis [100%] and peribronchial wall thickening [100%] were the most frequent CT abnormalities. Mucus plugging, air trapping and parenchymal involvements were respectively seen in 95.7%, 91.3% and 47.8% of patients. The overall CT score for all patients was 57.6 +/- 24.2 [means +/- SD]. The results of pulmonary function test showed a restrictive pattern; however, in 5.3% of the patients PFT was normal. The overall Shwachman-Kulczycki score was 53.48 +/- 13.8. There was a significantly [P=0.015] negative correlation between the total CT score and Shwachman-Kulczycki score; however, there was no significant correlation between total CT score and the results of PFT [P=0.481]. The Brody's scoring system for high resolution computed tomography seems to be a sensitive and efficient method to evaluate the progression of CF, and can be more reliable when we combine the CT scores with clinical parameters

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

[Occurrence pulmonary tuberculosis in a patient with Bernard soulier syndrome: a case report]

Bernard soulier syndrome [BSS] is a rare congenital bleeding disorder characterized by thrombocytopenia. BSS causes bruising, epistaxis, gingival bleeding, menorrhagia, post partum bleeding, gastrointestinal bleeding and post traumatic hemorrhage, but there is no report of hemoptysis in BSS. A 14 year-old girl was referred to our center due to massive hemoptysis. Her chest x-ray revealed complete collapse of the right lung. Rigid bronchoscopy was preformed and the intrabronchial clots were removed. Smear and culture of direct sputum was positive for Mycobacterium tuberculosis. She received anti-tuberculosis treatment. During treatment, she developed a massive vaginal bleeding, because of drug interaction between rifampin and low dose contraceptive [LD], which she had been taking for control of massive menstrual bleeding. Her vaginal bleeding was controlled by platelet infusion and recombinant factor 7 infusion. After 2 months of anti-TB treatment, sputum smear and culture for BK converted to negative. One year after treatment, the pulmonary tuberculosis was completely cured and no hemoptysis occurred. When hemoptysis occurs in patients with Bernard soulier syndrome, we should consider other differential diagnoses, and further diagnostic evaluation is recommended

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

[Pulmonary manifestations of pediatric hyper IgE syndrome]

Hyper IgE syndrome is a rare primary immune deficiency disorder characterized by pulmonary and cutaneous infection, eczema, and elevated serum IgE levels. In this article, 4 patients with hyper IgE syndrome and recurrent pulmonary infection were reported. Four girls with history of recurrent pulmonary infections were worked up and hyper IgE syndrome was diagnosed for them. All patients had recurrent pulmonary infections including pneumonia, bronchiectasis, pulmonary abscess, hydropneumothorax. Serum IgE levels were greater than 2000 Iu/ml in all cases. Microbial cultures showed staphylococcus aureas and pseudomans aeroginosa in two cases. All patients respond well to the wide spectrum intravenous antibiotics. Extra-pulmonary manifestations including purulent lymphadenitis, and skin and brain abcesses were observed. Hyper IgE syndrome causes recurrent pulmonary and extra-pulmonary infections which response fairly well to wide spectrum antibiotic therapy

relation between air pollution and cardiorespiratory admissions in Tehran

Air pollution is a major horror in many cities in Iran especially in Tehran. The cost of traffic congestion in the capital is put at two billion hours of time wasted each year. Tehran has also recorded SO[2] levels four times the standard prescribed by the World Health Organization. Tehran is the capital of the Islamic Republic of Iran with almost 11 million inhabitants [one sixth of the country's population], and is the most densely populated city of the country. The purpose of this research was to investigate the effects of air pollution on cardiorespiratory system. We assessed the relationship between the levels of air pollutants and emergency visits for asthma and cardiovascular diseases in Tehran, Iran. Two research questions investigated in this study were as follows: a] Which criteria elements of hazardous toxic air pollution were associated most strongly with the level of hospital admissions for cardiorespiratory conditions? b] What proportion of the variation in hospital admissions for cardiorespiratory conditions was explained by variations in levels of air pollution? During a 12-month period [from April 2004 to March 2005], the concentrations of 5 air pollutants [CO, NO[2], O[3], SO[2] and PM[10]] were measured in four stations located in north, west, south and central part of Tehran. The level of air pollution was calculated according to PSI [Pollution Standard Index]. Based on the results obtained during the study period, concentration of CO was reported as "above standard" on most of the days, leading to an "unhealthy" situation. 51.9% of measurements were made at PSI 300. For ozone [O[3]], all measurements were at standard conditions, PSI

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

Generalized lymphadenopathy: a case report of rosai-dorfman disease

Sinus histiocytosis with massive lymphadenopathy [SHML], Rosai-Dorfman Disease, is a rare histiocytic syndrome first described by Rosai and Dorfman, seen predominantly in childhood and early adulthood. Even though it is considered a benign disease, fatalities may occur due to cellular infiltrates of SHML. We report a 16-year-old boy with signs of polydypsia, polyuria, weight loss and generalized lymphadenopathy. He had been receiving corticosteroid following the diagnosis of histiocytosis X. Due to hyperglycemia, the patient was admitted with the primary diagnosis of diabetic ketoacidosis and medications were initiated. All paraclinical and immunologic examinations were negative. Axillary lymph node biopsy revealed the diagnosis of Rosai-Dorfman disease

Dental caries-associated microorganisms in asthmatic children

According to several studies, asthma medications especially beta2-agonists and corticosteroids have harmful effects on the dentition. This study was conducted to evaluate the prevalence of dental caries in asthmatic children in comparison with healthy controls. Some potential confounders of oral health were also evaluated. Asthmatic children aged 5-15 years under the care of the Pediatric Pulmonary Clinic of Masih Daneshvari Hospital were studied. DMFT index [decay, missing, filling teeth] was assessed in them by using the visual-tactile technique. Also, saliva samples were taken from each child and the number of Streptococcus mutans and Lactobacilli colonies in the samples was counted. Similar data were collected from the healthy controls. Forty-five asthmatic [mean age 10.90 +/- 3.16 yrs] and 46 healthy children [mean age 11.03 +/- 0.59 yrs] were studied. Mean DMFT was 3.98 +/- 2.53 in the control group and 4.30 +/- 2.81 in the study group which revealed a significant difference between the two groups regarding DMFT index. The number of Lactobacilli colonies was 8171.3 +/- 11956.0 and 16078.4 +/- 24305.5 in asthmatic and non-asthmatic groups, respectively which demonstrated no significant difference in this regard. Whereas, the number of Streptococcus mutans colonies was significantly different between the two groups [32331.7 +/- 46258.9 colonies in the control group versus 80883.4 +/- 74799.9 colonies in the study group; p-value < 0.05]. Multivariable analysis revealed that asthmatic children receiving anti-asthmatic medication including beta2-agonists and corticosteroids had a higher DMFT index. According to our study the prevalence of dental caries was higher in asthmatic children as compared to the healthy controls. Also, a significant correlation was detected between the saliva pathogens and dental caries. Dental caries were more prevalent in children receiving beta2-agonists alone than in those receiving both corticosteroids and beta2-agonists. Our study concluded that a more comprehensive precise oral health training program needs to be established by complete evaluation of the dental caries status in asthmatic children and also by training them regarding the technique of using inhalers with a spacer to lower the complications and costs of dental caries

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

Clinical and radiological aspects of chronic granulomatous disease in children: a case series from Iran

Chronic granulomatous disease [CGD] is a rare disorder of phagocytes, predisposes patients to bacterial and fungal infections. The main purpose of this study was to determine the clinical, radiological, pathologicial features, outcome and response to treatment of children with CGD. Thirteen patients with CGD, who had been referred to National Research Institute of Tuberculosis and Lung Disease [NRITLD], were reviewed during a 6 year period [1999-2005]. There were 10 [76%] male and 3[24%] female cases. The median age of the patients was 9 years [1 month-12 years].Family history of CGD was reported by 7 patients. The median diagnostic age was 8 years, with a diagnostic delay of 4.5 years. The most common manifestations of CGD were pulmonary infections and skin involvement, followed by generalized lymphadenopathy. The most common radiological findings were multiple lymphadenopathy in mediastinal region and fibrotic changes in lung fields. Two patients died of pulmonary infections. Based on the results of this research, immunologic evaluations especially evaluation for CGD is highly recommended in children suffering from recurrent pulmonary infections, cutaneous or hepatic abscesses, or infections caused by uncommon pathogens. Early diagnosis and prophylactic treatment both, prevent further development of the lesions, irreversible complications and decreasing mortality and morbidity rates in children suffering from CGD

Transmission of Mycobacterium tuberculosis to households of tuberculosis patients: a comprehensive contact tracing study

Tuberculosis [TB] continues to be a major health problem in developing countries. Contact investigation is the most appropriate strategy to interrupt transmission and subsequent development of TB. This cross-sectional study was conducted to assess the impact of contact screening on case-finding by using tuberculin skin test chest radiography. Contacts of smear-positive patients with pulmonary TB [index cases] were diagnosed and registered in our center during 2002 - 2004. Contacts, defined as household members living with index cases for >30 days, were screened by sputum examination, tuberculin skin test, and chest radiography. Sixty-eight patients with smear-positive pulmonary TB were considered as index cases. A total of 224 close contacts with index cases [an average of 3 contacts for each index case] were detected. Age among contacts ranged from 6 months to 74 years. Eighty-three percent of contacts were Iranians and 17% were Afghans. Abnormal radiographs were seen in 49.6% of contacts. Sixteen point five percent of contacts had a positive tuberculin skin test of >10 mm; 7.6% had a positive sputum smear. The mean - SD age of Iranian contacts [29.1 +/- 16.6 years] was significantly [P < 0.001] higher than that of Afghans [18.6 +/- 14.1 years]. Cavitary formation, nodular pattern, and infiltration were found to have a strong association with a positive sputum smear for acid fast bacilli [100%, 100%, and 87%, respectively]. The rate of TB in contacts was higher than other similar studies. Earlier detection and treatment of adults with TB could interrupt transmission and be a step towards eliminating childhood TB. Contact control and source-case investigations should be emphasized for TB control. Novel strategies are needed to maximize the number of contacts who are not only identified and evaluated, but also completely treated