Level of agreement between children with asthma and their parents on quality of life

Background: Child-parent agreement is a controversial aspect of measuring health-related quality of life [HRQoL] in children and adolescents. The aim of this study was to assess the agreement between the child self-reports and parent proxy reports of the PedsQL 3.0 Asthma Module in Iranian children with asthma to evaluate HRQoL. Moreover, the psychometric properties of the child and parent reports of the PedsQL 3.0 Asthma Module were assessed in the present study

Methods: Participants were 112 children with asthma and their parents, who completed the Farsi version of the PedsQL 3.0 Asthma Module. The multitrait-multimethod correlation matrix and factor analysis were used to test whether the child self-reports and the parent proxy reports measured the same construct. Additionally, convergent and discriminant validity and internal consistency were assessed using the Pearson correlation

Results: The correlation between the child and parent HRQoL perceptions ranged between 0.13 and 0.36 across the same domains. Our factor analysis revealed that the child self-reports and the parent proxy reports measured 2 different constructs of HRQoL. Furthermore, our findings showed that both the child self-reports and the parent proxy reports of the PedsQL 3.0 Asthma Module had excellent internal consistency and acceptable convergent and discriminant validity

Conclusion: Although the child self-reports and the parent proxy reports of the Farsi version of PedsQL 3.0 Asthma Module showed good psychometric properties, they were not interchangeable. Our children with asthma and their parents evaluated child HRQoL from their own viewpoints

Clinical and genetic features in patients with cystic fibrosis in southwestern Iran

Cystic fibrosis [CF] is a common autosomal recessive genetic disease caused by a mutation in the CF transmembrane conductance regulatory [CFTR] gene. This study attempted to identify the most common CFTR mutations and any correlations between certain mutations and the clinical presentation of the disease in CF patients in southwestern Iran. Twenty nine common CFTR gene mutations were examined in 45 CF patients. Chronic cough, intestinal obstruction, dehydration, heat exhaustion and steatorrhea were the most common early clinical symptoms among our patients. The most common mutation was deltaF508, with an allele frequency of 21%. The homozygous deltaF508 mutation was observed in eight patients [18%], and three patients [7%] were deltaF508 carriers. The 2183AA>G mutation was observed in four patients, one of whom was also a deltaF508 carrier. The R1162X mutation was detected in two patients. The G542X, R334W and N1303K mutations were detected each in one patient, the first of whom was also a deltaF508 carrier. Out of 45 patients, 27 [60%] had none of the CFTR gene mutations we tested for. The most frequent mutations in southwestern Iranian patients with CF should be identified by sequencing the entire CFTR gene in order to optimize the design of a diagnostic kit for common regional mutations

Interstitial lung disease in a patient with chronic granulomatous disease

Chronic granulomatous disease [CGD] is an inherited phagocytes defect, characterized by defects of NADPH-oxidase and inability of bacterial killing, which leads to recurrent life-threatening infections. Respiratory problems, which are the major cause of morbidity in CGD, usually result from recurrent severe infections; however, vigorous inflammatory response could also cause respiratory diseases. Herein, an 11 year-old patient with CGD is presented who suffered from chronic cough and dyspnea for 7 years. Considering the results of chest X-ray, high-resolution computed tomography, and pulmonary function test, the diagnosis of interstitial lung disease was made. Early recognition of manifestations associated with CGD and appropriate treatment could prevent further complications and reduce morbidity and mortality in this group of patients

role of cow's milk allergy in pediatric chronic constipation: a randomized clinical trial

Cow's milk allergy has different presentations in children and can cause functional bowel symptoms such as chronic constipation. The aims of this study were to investigate the role of cow's milk allergy as a cause of chronic constipation and effect of cow's milk free diet [CMFD] on its treatment in children. We performed a randomized clinical study comparing CMFD with cow's milk diet [CMD] in two groups each consisting of 70 patients [age range, 1-13 years] with chronic functional constipation [defined as Rome III criteria]. All subjects had been referred to a pediatric gastroenterology clinic and had previously been treated with laxatives for at least 3 months without success; also all 140 patients performed skin prick test. The case group received CMFD for 4 weeks. After that they received CMD for 2 extra weeks. The control group received CMD for whole 6 weeks. A response was defined as decreased in signs and symptoms that not fulfilled Rome III criteria after 4 weeks of CMFD and came back to Rome III criteria after 2 weeks of CMD challenge. After 4 weeks 56 [80%] patients of the case group responded in comparison to 33 [47.1%] patients in the control group [P=0.0001]. In the case group after 2 weeks challenge 24 out of 56 [42.8%] responders developed constipation according to Rome III criteria. With other words, the frequency of cow's milk allergy among constipated patients was 80%. Only one patient had positive skin prick test. In children, chronic constipation can be a manifestation of cow's milk allergy. At present, although several aspects must be further investigated, a therapeutic attempt with elimination diet is advisable in all children with constipation unresponsive to correct laxative treatment

Specific IgE to common food allergens in children with atopic dermatitis

Atopic dermatitis is a major public health problem, often starting in early childhood and sometimes followed by other allergic diseases. Although hypersensitivity to foods is assumed to play an essential role in the development of atopic dermatitis in some patients, little is known about common food allergens in Iranian children with atopic dermatitis. This study was designed to identify probable food allergens in Iranian children with atopic dermatitis and find the relationship between food sensitization and the severity of atopic dermatitis. This study included 90 children aged 2-48 months with atopic dermatitis. Skin prick tests for cow's milk, hen's egg, almond, potato and soybean were done. Serum specific IgE to 20 food allergens was also screened. Among children with atopic dermatitis, the frequency of food sensitization was 40% by skin prick test and 51% by food-specific IgE. Children with atopic dermatitis were most commonly sensitized to cow's milk [31%], hen's egg [17.7%], tree nuts [17.7%], wheat [12.2%], potato [11.1%], tomato [8.8%] and peanut [8.8%]. In 42 children with moderate to severe eczema, sensitivity to food allergens was 78.5% by skin prick test and 88% by serum specific IgE evaluation. Our results showed that cow's milk, hen's egg and tree nuts were the most common food allergens in Iranian children with atopic dermatitis. Sensitization to foods was much higher in patients with moderate to severe atopic dermatitis. Determining specific IgE in children with atopic dermatitis can be helpful in managing these patients

Giant aneurysm of thoracic and proximal abdominal aorta in a patient with common variable immunodeficiency

Common variable immunodeficiency [CVID] is the most common symptomatic primary immunodeficiency disease, predisposing the patients to various tissue involvement and organ damage. Here a 16-year-old boy is presented who was referred to our center with cough, dyspnea, cyanosis, and history of recurrent pneumonia. The diagnosis of CVID was made according to reduction all serum immunoglobulin levels, normal numbers of T, B and NK lymphocyte subpopulations, poor antibodies responses. Considering abnormality in heart examination and chest X-ray, echocardiography and computed tomography angiography were performed which showed large thoraco-abdominal; aortic aneurysm in this patient. Although there are some reports of cardiovascular disease associated with primary antibody deficiencies, this is the first time that such large thoraco-abdominal aortic aneurysm is reported in CVID. This may be secondary to recurrent pulmonary infections or an unknown mutation process. Cardiovascular abnormalities are an entity that should be kept in mind in patients with primary immunodeficiency diseases

The Relationship Between Serum 25 Hydroxy Vitamin D Levels and Asthma in Children

PURPOSE: Asthma and other allergic disorders have increased over the past decades in nearly all nations. Many studies have suggested the role of vitamin D deficiency in both T-helper1 and T-helper2 diseases; however, the association between vitamin D, allergy, and asthma remains uncertain. In this study, the associations of 25-hydroxy vitamin D3 levels with asthma and with the severity of asthma were evaluated. METHODS: This cross-sectional study was conducted on 50 asthmatic children and 50 healthy controls aged 6-18 years. Serum 25-hydroxy vitamin D3 levels were determined and compared between the two groups. The relationship between serum vitamin D levels and pulmonary function test outcomes and eosinophil counts were examined in asthmatic patients. RESULTS: Univariate analysis of the relationship between asthma and vitamin D showed that decreased vitamin D levels were associated with significantly increased odds of asthmatic state (P=0.002). In a multivariate analysis after adjustment for age, body mass index, and sex, the relationship between vitamin D and asthma increased. In asthmatic patients, 25-hydroxy vitamin D levels had direct and significant correlations with both predicted FEV1 (R2=0.318; P=0.024) and FEV1/FVC (R2=0.315; P=0.026). There were no associations between vitamin D level and eosinophil counts, duration of disease, and the number of hospitalization or unscheduled visits in the previous year (P>0.05). CONCLUSIONS: These results showed that serum 25-hydroxy vitamin D levels were inversely associated with asthma, and there was a direct and significant relationship between vitamin D levels and pulmonary function test outcomes in asthmatic children. An interventional study in asthmatic patients with low serum vitamin D concentration may establish a causal relationship between asthma and vitamin D.

Para-aortic lymphadenopathy associated with Kawasaki disease

Kawasaki disease is an acute vasculitis that occurs mainly in children. Cervical lymphadenopathy is one of the major presenting manifestations of Kawasaki disease. We report a case of Kawasaki disease with para aortic lymphadenopathy, as an unusual feature in this disease. This 2.5 year old girl presented with persistent high grade fever, erythematous rash, bilateral non prurulent conjunctivitis, red lips, and edema of extremities. Laboratory results included an elevated erythrocyte sedimentation rate, leukocytosis, anemia, and positive C-reactive protein. On second day after admission she developed abdominal pain. Ultrasonography of abdomen revealed multiple lymph nodes around para aortic area, the largest measuring 12mmx6mm. Treatment consisted of aspirin and high dose intravenous gamma- globulin. Ultrasonography and CT scan of abdomen performed one week later showed disappearance of the lymph nodes. There are few previous reports of lymphadenopathy in unusual sites such as mediastinum in Kawasaki disease. Para aortic lymph nodes enlargement might be an associated finding with acute phase of Kawasaki disease. In these patients a close observation and ultrasonographic follow up will prevent unnecessary further investigation

Predictive value of the cow's milk skin prick test in infantile colic

Infantile colic is a common problem among young infants. Cow's milk allergy has been suggested as one of the causes. We aimed to investigate the value of the cow's milk skin test for the diagnosis of cow's milk allergy in exclusively breast-fed infants with infantile colic. Exclusively breast-fed infants with infantile colic were enrolled in this study. On the first visit, the average hours of crying of the infant in a 24-h period were recorded and the cow's milk skin test was performed. If the infant had a positive skin test, elimination of cow's milk from the mothers' diet was advised. Infants with negative skin tests were divided into case and control groups. Cow's milk was eliminated from the diet of mothers in the case group. After 2 weeks, the number of hours of crying were recorded again. The reduction in the crying hours was compared between the two groups using the chi-square test. Skin tests were positive in 3 of 114 cases [2.6%] of infantile colic. All three cases recovered completely following elimination of cow's milk from the mothers' diet. Among the 111 patients with negative skin tests, 77 patients completed the study: 35 in the case group and 42 in the control group. The reduction in crying hours in infants in the case group was not significantly different from that in the control group. Elimination of cow's milk from the mothers' diet is not beneficial for infants with a negative skin test. Infants with a positive skin test may benefit from this management

Antigen-specific antibody response in juvenile-onset SLE patients following routine immunization with tetanus toxoid

Infection is now the most common cause of morbidity in Systemic Lupus Erythematosus [SLE]. There is lack of information regarding the specific antibody formation in response to vaccines in young SLE patients. To determine the efficacy of anti-tetanus antibody response in young patients with SLE. Forty SLE patients with mean age of 14.1 years [range: 7-21] and 60 age and sex matched normal controls were enrolled in this study over a period of one year. Diagnosis was made according to the ACR criteria and disease activity was determined based on SLE Disease Activity Index [SLEDAI]. All patients and controls had received the complete schedule of tetanus vaccinations consisting of three primary doses and two boosters by the age of six. Serum immunoglobulins and anti-tetanus antibody titers were determined by Nephelometry and ELISA. Anti-tetanus antibody levels greater than 0.1 IU/ml have been suggested as protective. In all of the patients and controls anti-tetanus antibody titer was > 0.1 IU/ml. IgG, IgA, and IgM levels were in the normal range for their age. Mean disease activity score was 4.9 [range: 0-16]. There was no association between SLEDAI score and anti-tetanus antibody response. School age onset and immunosuppressive therapy does not seem to interfere with development of consistent immunity to tetanus vaccine in young SLE patients

Agreement rate of skin prick test with tissue eosinophil count in patients with nasal polyps

The pathogenetic mechanism of nasal polyps remains unknown, although allergy has been cited as an important factor in the etiology of nasal polyposis. Currently there is no definite histological criterion for differentiation of allergic from inflammatory nasal polyp. However, in a few studies, tissue eosinophil count has been used for this. This study aimed to find out the agreement rate of skin prick test and tissue eosinophil count in patients with nasal polyposis. Twenty five patients [18 males, 7 females] with nasal polyp were enrolled in this study. For each patient tissue sample from polyp material was taken for histopathological investigation. Moreover, skin prick test was performed for each patient using eleven common aeroallergens. Skin prick test was positive in 48% of the patients. Tissue eosinophil count of more than 50% was found in 75% of skin prick positive and in 69.2% of skin prick negative patients. Also tissue eosinophil count of more than 50% was found in 69.2% of patients with typical allergic symptoms as well as 75% of patients without allergic symptoms. No agreement was found between skin prick tests and tissue eosinophil counts in patients with nasal polyp. Also no difference was found between the tissue eosinophil counts in allergic and non allergic patients. Considering these results, it can be concluded that having a high tissue eosinophil count in patients with nasal polyp does not indicate that the polyp is allergic

Bone mineral density in children with systemic lupus erythematosus and juvenile rheumatoid arthritis

Although there is increasing in bone metabolism in patients with rheumatic disorders, few data exist on bone mineral density [BMD] in children with rheumatic disorders or on the association of BMD with disease-related variables. We determined BMD in Iranian children with systemic lupus erythematosus [SLE] and juvenile rheumatoid arthritis [JRA] to evaluate the relationship between disease-related variables and BMD. Twenty patients [13 girls and 7 boys] with SLE [n=15] and JRA [n=5] with a mean age of 13.10 +/- 3.29 years [range, 6-17 years], attending a pediatric rheumatology clinic and 20 healthy controls [matched for age and sex with each patient] were enrolled in a cross-sectional study between 2001 and 2003. BMD [g/cm[2]] of the femoral neck [BMD-F] and lumbar vertebrae [BMD-L] were measured by dual energy X-ray absorptiometry [DEXA]. The correlation between BMD and cumulative dose of steroids, daily dose of steroid, disease duration, disease activity, height, weight, and age was investigated. BMD in the patients [BMD-F=0.72 +/- 0.15, BMD-L=0.70 +/- 0.19] was significantly lower than controls [BMD-F=0.95 +/- 0.17, BMD-L=0.98 +/- 0.20, P=<0.001]. The severity of descreased BMD was more prominent in lumbar vertebrae than the femoral neck [P=0.04]. None of the variables were consistently related to a decrease in BMD. BMD was significantly lower in patients compared with controls. It was more prominent in lumbar vertebrae [trabecular bone]. Although cumulative dose of steroids and diseaese appeared to have some influence on BMD, none were independently correlated with BMD

Case Report: Churg-Strauss Syndrome in an 8-year-old girl

Churg-Strauss Syndrome [CSS] is a small vessel granulomatous vasculitis that characteristically affects the middle age group and is extremely rare in children. We describe an 8-year-old girl with a prolonged history of cough and eosinophila that initially was labelled as pulmonary tuberculosis, but after complementary assays, associated with skin biopsy, eosinophilic fibrinoid vasculitis, CSS was eventually diagnosed. In this case, due to her young age and more prevalence of infectious lung diseases in our area, there was a few months delay for correct diagnosis and treatment. After meeting the CSS criteria the appropriate treatment was started and her condition improved

Synergistic effect of intravenous immunoglobulins and iodinated contrast media on renal function

Acute renal failure is one of the side effects while using intravenous immunoglobulins. This complication is also observed with iodinated contrast media. Herein, we describe a patient with acute renal failure who received intravenous immunoglobulins and iodinated contrast media concomitantly. Both drugs are responsible for osmotic nephrosis. The same effect on renal cells may explain a synergistic effect on renal function

Anticardiolipin antibodies in juvenile rheumatoid arthritis and systemic lupus erythematosus

Antiphospholipid antibody syndrome [APS] can either occur as a primary syndrome or associated with other autoimmune diseases such as systemic lupus erythematosus [SLE]. Anticardiolipin antibody [aCL] of IgG and/or IgMisotype in blood, measured by a standardized ELISAis the most acceptable laboratory criteria. APS IgGisotype, particularly IgG2 subclass is more strongly associated with thrombosis. This study was done to determine the prevalence of IgG aCL and its subclasses in relation to APS symptoms, in a group of juvenile rheumatoid arthritis [JRA] and juvenile systemic lupus erythematosus [SLE] patients. In this prospective study, 28 JRAand 16 SLE patients, aged 3-18 years, were enrolled. IgG aCLwas assayed by standard aCL ELISA. IgG subclasses were also assayed by ELISA on sera with medium to high titers of aCL.ACL assay was performed on at least two occasions for each patient, over 3-6 months period of follow up. 29% [8/28] of JRApatients and 44% [7/16] of SLE patients had aCL. Six of SLE patients displayed APS related manifestations: hemolytic anemia, thrombocytopenia, arterial occlusion, valvular heart disease, livedo reticularis and pulmonary hypertension, but none of them had persistant medium or high titer of aCL. The lack of association of high titer of aCL with APS related symptoms was observed in two patients. The IgG subclasses were primarily IgG1 and IgG3. The prevalence of IgG aCL in this group of pediatric SLE and JRA is not uncommon but it's relation to clinical manifestations is not clear. IgG1 and IgG3 subclasses were not associated with thrombosis, which is in agreement with previous studies

Prevalence of aeroallergens in allergic rhinitis in Shiraz

Allergic rhinitis is an extremely common disease worldwide. Aeroallergens are very often involved in allergic rhinitis and their prevalence may vary in different regions. The causative allergens of allergic rhinitis in our area are unknown. The purpose of this study was to determine the prevalence of skin reactivity to different aeroallergens in patients with allergic rhinitis in the city of Shiraz, Iran. A total of 212 patients who were referred to Motahari Allergy Clinic with chronic rhinitis were subjected to skin prick test [SPT] with a series of common allergenic extracts including grasses, weeds, trees, house dust mites and moulds. One hundred and thirty two subjects [62.2%] had positive SPT to at least one aeroallergen. Male to female ratio was 1.2 and mean age was 18.2 years. The prevalence rates for allergen groups were: pollens [92.4%], mites [22.7%] and moulds [8.3%]. Among 122 patients reactive to pollens, 92 [75.4%] showed skin reactivity to weeds, 78 [63.9%] to grasses and 68 [55.7%] to trees. Polysensitization was common, with 75.7% of all sensitized patients being positive to more than one aeroallergen. Pollens are the main sensitizing allergens among patients with allergic rhinitis in Shiraz. This pattern of prevalence was expected based on herbal geography, climate and also found to be compatible with the results from studies carried out in places with the same habitat