[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 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

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 hyper IgE syndrome: case series and literature review

This study aimed to present four children suffering from recurrent pulmonary infections in the context of hyper lgE syndrome. All patients had recurrent pulmonary infections including pneumonia, bronchiectasis, pulmonary abscess and hydropneumothorax. Serum IgE level was greater than 2000 IU/ml in all cases. Microbial cultures showed Staphylococcus aureus and Pseudomonas aeruginosa in two cases. All responded well to the wide spectrum intravenous antibiotics. Extra-pulmonary manifestations included purulent lymphadenitis as well as skin and brain abscesses. Hyper IgE syndrome causes recurrent pulmonary and extra-pulmonary infections which respond fairly well to wide spectrum antibiotic therapy

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

Ascorbic acid concentration in plasma and white blood cells of patients with bronchial asthma

Asthma is an inflammatory disease of the airways. Oxidants are of the important factors damaging the airways. Moreover, an inappropriate correlation exists between oxidants and antioxidants in asthma. Vitamin C is one of the major protective antioxidants of the airways. Thus, we evaluated the concentration of Vit C in plasma and white blood cells of asthmatic patients. A case-control study was performed on 50 asthmatic patients referred to Masih Daneshvari Hospital. Data were collected through the general information and 24h dietary recall questionnaires and then the effect of independent variables on plasma and WBC ascorbic acid concentrations was evaluated by statistical analyses using biochemical tests. Data showed that plasma and WBC ascorbic acid deficiency exists in 38% and 92% of asthmatic patients, respectively. There was a significant difference in plasma and WBC Vit C concentrations between case and control groups [p < 0.0001]. There was a positive significant correlation between the level of plasma ascorbic acid and dietary Vit C intake [p=0.0001]. The present study showed a relationship between asthma and ascorbic acid levels in plasma and in WBCs. More precise studies are recommended for better determination of asthma and vitamin C correlation

Diagnostic value of lung CT-scan in childhood tuberculosis

Many different methods and approaches have been applied for confirmation of tuberculosis in children. The diagnostic criteria being currently used for detection of childhood TB consist of clinical symptoms, history of close contact, radiological findings, PPD test and positive bacteriologic or pathologic findings. Since each of these methods may have false positive or negative results, it is necessary to find a better method for prompt diagnosis. This study was performed to determine the value of CT-scan as a sensitive method in detecting hilar, parenchymal and mediastinal involvements in early diagnsis of childhood TB and compare it with other diagnostic criteria. This cross-sectional comparative study was carried out on 100 children, suspicious of having primary pulmonary TB between September 1999 and September 2000 in Masih Daneshvari Hospital. All patients had prior history of close contact with smear-positive patients having active pulmonary TB. Epidemiological factors as well as radiological and microbiological findings were evaluated. Of total 100 patients, 43 were female and 57 were male. The mean age was 7.5 +/- 3.6 yrs ranging from 4 months to 14 years. Forty two were Iranian and 58 were Afghan. Thirty nine children had a positive PPD test. Bacteriological and simple chest x ray findings compatible with TB were positive in 11 and 36 patients, respectively. Pulmonary CT-scan without contrast revealed lung lesions in 55 patients while 25 of them [45.4%] had normal chest x rays. In 12 patients positive CTscan was the only positive diagnostic finding. Our results show the value of pulmonary CT-scan as a diagnostic criterion in pediatiric tuberculosis and we recommend it for early diagnosis in suspicious cases with no other positive findings

Primary and acquired drug resistance in childhood tuberculosis

Background: Increased rates of multidrug-resistant tuberculosis [MDR-TB] have been reported from developing countries. We evaluated the incidence of drug resistance in children in order to determine the magnitude of the problem, in our region

Objective: To determine the resistance pattern of Mycobacterium tuberculosis to four anti-tuberculosis drugs in childhood pulmonary tuberculosis at National Research Institute of Tuberculosis and Lung Disease [NRITLD] which is a referral centre in Tehran. Treatment of the patients was based on the DOTS strategy according to the WHO protocols since 1989

Materials and Methods: Retrospective analysis of all cases of pulmonary tuberculosis with positive M. tuberculosis culture who had referred to paediatrics ward from January 1999 to August 2004. M. tuberculosis sensitivity testing was performed by the Lowenstein-Jensen medium for isoniazid [INH], rifampicin [RMP], streptomycin [SM], and ethambutol [EMB]

Results: Among 350 children [0-15years] with confirmed tuberculosis, 7 children had resistance to at least one of the four anti-TB drugs. Out of the 7 patients, 6 were Afghan refugees and one patient was Iranian. Among those 85.7% had resistance to RMP, 71.4% to INH, 57.1% to SM, and 28.6% to EMB .In addition, 28.5% of patients had resistance to all four drugs [RMP, INH, SM, EMB], 14.2% to INH, RMP, SM, 28.5% to INH, RMP and 14.2% had resistance to each of SM and RMP. In this study 2% of children with TB had resistance out of which primary resistance was detected in 57.1%. Secondary resistance was found in 42.9% of cases who had previous history of anti-TB therapy

Conclusion: According to 2% prevalence of drug resistance in children and high resistance to RMP in our study, more aggressive interventions should be considered. Further management and supervision in DOTS implementation is highly recommended to prevent transmission of resistant tuberculosis