Natural products, PGC-1 , and Duchenne muscular dystrophy

Peroxisome proliferator-activated receptor (PPAR) is a transcriptional coactivator that binds to a diverse range of transcription factors. PPAR coactivator 1 (PGC-1) coactivators possess an extensive range of biological effects in different tissues, and play a key part in the regulation of the oxidative metabolism, consequently modulating the production of reactive oxygen species, autophagy, and mitochondrial biogenesis. Owing to these findings, a large body of studies, aiming to establish the role of PGC-1 in the neuromuscular system, has shown that PGC-1 could be a promising target for therapies targeting neuromuscular diseases. Among these, some evidence has shown that various signaling pathways linked to PGC-1 are deregulated in muscular dystrophy, leading to a reduced capacity for mitochondrial oxidative phosphorylation and increased reactive oxygen species (ROS) production. In the light of these results, any intervention aimed at activating PGC-1 could contribute towards ameliorating the progression of muscular dystrophies. PGC-1 is influenced by different patho-physiological/pharmacological stimuli. Natural products have been reported to display modulatory effects on PPAR activation with fewer side effects in comparison to synthetic drugs. Taken together, this review summarizes the current knowledge on Duchenne muscular dystrophy, focusing on the potential effects of natural compounds, acting as regulators of PGC-1.

[Prevalence of atopic eczema in infants under two years referring to the vaccination ward of the Hazrat Rasoul Akram Hospital, Tehran, Iran]

Atopic dermatitis is a common allergic disease recognized as a threat to public health. Atopic dermatitis is often observed in people with individual or family history of allergic diseases. Due to the importance of this disease and its early diagnosis, and the very limited number of studies on this topic, the current study was performed on the prevalence of atopic dermatitis on infants under 2 years of age. This was an analytic cross-sectional study from January 2010 to February 2011. All infants referred to Hazrat Rasoul Akram Hospital [Tehran, Iran] for vaccination were enrolled in the study. Patients with atopic dermatitis, by permission of their parents, underwent Prick test for identification of the allergy type. In the present study, 380 infants were enrolled with mean age of 9.9 +/- 6.6 months [217 boys and 163 girls]. Patients were classified into two groups based on atopic dermatitis [48 positive for eczema and 332 negative for it]. Of the 48 subjects of the positive group, 31 agreed to take the Prick test. The results showed that 11 patients [35.5%] were allergic to cow milk, 6 patients [19.3%] to almond, 4 patients [12.9%] to rice and egg's yolk, 3 patients [9.7%] to egg white, 2 patients [6.5%] to fish, soybean, potato, and sesame, and 1 patient [3.2%] to wheat flour. It seems that the outbreak of atopic dermatitis in children is relatively high, and avoidable factors play a role in its occurrence. Hence, based on the examined Prick tests, it seems that the occurrence and exacerbation of the disease can be controlled and its progression into asthma prevented by decreasing or removing food allergens and factors involved in the patient's disease process

Development of a high-resolution melting analysis method based on SYBR green-I for rs7216389 locus genotyping in asthmatic child patients

Asthma is caused by the combination of different factors. Current concepts of asthma pathogenesis emphasize on gene-environment interactions. Mega-genome scanning projects revealed that different Single Nucleotide Polymorphisms [SNPs] are related to asthma susceptibility. rs7216389-T is one of them that is related to childhood asthma and its effect on childhood asthma severity has been proved in different nations, however no study has been performed in Eastern Mediterranean and Middle East countries yet. To perform population genetic studies, a rapid and high-throughput screening method is necessary. High-resolution melting analysis is a rapid, powerful and accurate method, which is suitable for this type of studies. Therefore, it has been decided to develop a high-resolution melting method for rs7216389 locus genotyping in Iranian asthmatic children. In the current study, a high-resolution melting analysis method based on SYBR Green-I was developed to check the frequency of rs7216389-T mutation in Iranian asthmatic children for the first time. Second and third classes of intercalating dyes are commonly used for high-resolution melting method. However, in this study, SYBR Green-I was used for rs7216389 locus genotyping for the first time. Our results for 60 samples showed that SYBR Green-I has good efficacy for rs7216389 locus genotyping through high-resolution melting method in comparison with PCR-RFLP and sequencing. Comparison of our results based on HRM analysis with PCR-RFLP showed that our developed method is rapid, accurate, high-throughput and economic to study the rs7216389 locus in asthmatic children and it is applicable for other similar population genetic studies

[Prevalence of food allergy in asthmatic children under 18 yeasts of age in Semnan-Iran in 2007-2008]

Asthma is the most common chronic disorder in children and is more prevalent in children 5-10 years of age. The prevalence of childhood asthma has increased in recent years. Food allergy has been implicated as a risk factor for asthma. In this study, we determined the prevalence of food allergy and associated factors in asthmatic children less than 18 years of age. In this Cross-sectional study, we studied 289 asthmatic children less than 18 years of age in Semnan [Iran] in 2007-2008, who were diagnosed according to physical examination and history. Skin pick test was performed for all of participants and a questionnaire was filled out. Wheals of 3 millimeter and flares of 10 millimeters larger than negative control was regarded as a positive test. 35.9% [95% Confidence Interval: 30.5-41.3%] of asthmatic children showed sensitization to at least one of the principal allergenic foods [wheat, rice, peanut, egg, soya and cow milk]. Prevalence of food allergy is asthmatic children under 18 years of age had significant association with gender, birth weight, history of other allergies, family history of allergy, type of coexistent allergy and age of initiation of symptoms, age of introduction of complementary feeding, consumption of cow milk before one year of age and also duration of breast feeding [P<0.05]. These finding showed that the prevalence of food allergy in asthmatic children is high. There is significant association between food allergy and use of cow's milk before one year of age, low birth weight, short beat feeding period, male gender, history of other allergies in the patient and close relatives, early age of initiation of symptoms and also introduction of complementary foods before six months of age. Thus, we should have particular attention on food allergens in such king of patients, until they outgrown their food allergies. This is the optimum way for controlling asthma in these people

[ incidence of complications of vaccination in children and infants of Semnan, Iran]

Vaccination has achieved significant and cost effective success in prevention of infectious disease. Although the current vaccines used are very effective and their side effects are minimal, generally no vaccine is not free from side effects. Incidence of adverse reactions after immunization may discourage people for further immunization of their children. The aim of this work was to determine the incidence of complications of vaccination in children and infants of Semnan, Iran. In a longitudinal study [prospective], all vaccinated children between September 2006 and March 2007 in 11 vaccination centers were studied. A specific questionnaire, including vaccine recipient profile, type of vaccine, birth weight, feeding and 46 adverse reactions were completed immediately after and 2,4,6,12, 18 months later. 5776 children were studied. 29% of the children showed at least one adverse reaction of vaccination. The most common adverse reactions were: fever [24%], pain at injection site [3.8%], swelling [2.5%], erythema [2.5%], induration [2.1%], and ulceration at injection site [2.1%]. Incidence of other complications was below 1%. The most dangerous complication of the vaccine was encephalitis [one case] and two cases had febrile seizures. The most cause of hospitalization was adverse reaction of MMR vaccine. Finally, of every four children, one child showed at least one complication that fever was the most common. In general, routine immunization program of Iran country against nine common infectious diseases has lower complications. This complication is mainly mild and transient and do not need any intervention by drugs. However, among these may be rare and dangerous complications such as seizures and encephalitis occur. Hence, a careful follow-up program is required to report complications of immunization

Antioxidant and free radical scavenging activity of h. officinalisl. var. angus tifolius, v. odorata, b. hyrcana and c. speciosum

Extracts of 4 medicinal and aromatic plants were investigated for their antioxidant potency employing six various established in vitro system: H. officinalis L. var. angustifolius aerial parts, C. speciosum flowers, V. odorata and B. hyrcana leaves.With regard to IC5o values [micro g/ m], the order in DPPH radical-scavenging were CS [585.6] > HO [311] > VO [245.1] > and BH [113.1]. Effectiveness in reducing powers were high and in a descending order of HO > CS > BH > VO [at the concentrations of 25-800 micro g/ ml]. IC[50] for Fe[2+] chelating ability were 188, 750 and 980 micro g/ ml for VO, CS and HO, respectively. BH extract has shown only 38% inhibition at 800 micro g/ ml. The extracts showed weak nitric oxide-scavenging activity. All extracts exhibited very low and moderate concentration-dependent antioxidant activity in FTC methods. IC[50] for scavenging of H[2]O[2] were 169 for BH, 175 for CS, 640 for VO and 663 microg/ ml for HO. The content of total phenolic compounds and flavonoids were measured in plant extracts. The data obtained in the in vitro models clearly establish the antioxidant potency of all extracts

[Prevalence of obesity and some related factors among students aged 7 to 12 in Semnan, Iran]

Obesity is one of the most important public health problems. During recent decades, prevalence of obesity among children has increased and this is also observed among Iranian children. This study is aimed to determine the prevalence of obesity and some related factors among students aged 7 to 12 in Semnan. This was a cross sectional study. 400 students aged between 7 to 12, were selected by randomized sampling from 20 elementary school. We measured height and weight of children and their parents to calculate BMI. They also completed a questionnaire which was designed to collect some interested variables. 14/3% of children were [obese] and 18/8% of them were categorized as [over weight]. There was a significant association between obesity and gender, so a higher rate of obesity in boys and overweight in girls was observed. Also getting older per each year, the obesity became higher 1.26 times. A significant relationship was observed between obesity of father, mother and children, so an increase of each unit of BMI their father and mother, the obesity chance in children became equal to 1.18 and 1.15 respectively. There was no significant association between obesity and other parameter studied such as consuming of fast food, watching TV and working with computer, daily commute to school, rank of birth, number of families, or parent's educational levels. The prevalence of obesity during childhood is increasing, in Semnan and it correlates with obesity of parents. Therefore training of parents and also their children might be an effective way to decrease obesity among children

Systemic and local reactions of bee venom immunotherapy in Iran

Severe allergic reactions during specific immunotherapy may occur in the treatment of hymenoptera sting allergy. The objective of the present study was to examine the characteristics of allergic reactions during specific immunotherapy in patients with allergy towards hymenoptera venom in the Iranian population. A prospective study was performed using the clinical reports of 27 patients with anaphylaxis to bee venom [Apis melifera, Geupes vespula and Geupes Polites]. Ten patients treated with Cluster protocol during 2002 and 2006 After diagnosis of hymenoptera sting allergy according to history and intradermal tests, the patient were treated with Cluster protocol immunotherapy. The protocol lasted 6 weeks with an increase in the concentration of venom from 0.01? g/ml to 100 ? g/ml. None of the patient received premedication. All patients with hymenoptera venom allergy received 120 injections. Anaphylactic reactions were classified according to the Mueller-classification. The frequencies of systemic reactions during Cluster protocol were 8.33% and 5% for yellow jacket and honey bee venom respectively. No patient experienced severe systemic reaction. Cluster protocol for hymenoptera immunotherapy is a reliable method for the treatment of anaphylactic reactions to bee venom. It is safe with low cost and do not need hospitalization

Autoimmune progesterone anaphylaxis

Progesterone induced dermatitis is a rare disorder. It typically occurs in females due to an autoimmune phenomenon to endogenous progesterone production, but can also be caused by exogenous intake of a synthetic progestin. Here in, we present a case of autoimmune progesterone anaphylaxis [AIPA] observed in an adolescent female. The patient is an 18-year-old Caucasian female with no significant past medical history and no prior exogenous hormone use, who presented to her primary care physician complaining of cyclic skin eruptions with dyspnea, cough and respiratory distress. She noted that her symptoms occurred monthly, just prior to her menses. An intradermal skin test using 0.1 cml of progesterone was performed. The patient developed a 15mm wheal after 15 minutes, confirming the diagnosis of AIPA. The patient was started on a continuous regimen of an oral conjugated estrogen [0.625mg]. The skin eruptions and respiratory symptoms have not returned since the initiation of this therapy. Autoimmune progesterone dermatitis manifests via the occurrence of cyclic skin eruptions. Women with the disorder commonly present with dermatologic lesions in the luteal phase of the menstrual cycle, if there are any other organ involvement in addition to skin [e.g. lung, GI] the reaction should be called as autoimmune progesterone anaphylaxis. Diagnosis of AIPA is confirmed by performing a skin allergen test using progesterone

Prevalence and symptoms of idiopathic hypercalciuria in primary school children of Tehran

Determining prevalence of idiopathic hypercalciuria [IH] in primary school children of Tehran. We evaluated 778 primary school children [age 6-11, mean 9.1 years] in two steps: first [Screening test], we measured urine calcium to urine creatinine ratio [UCa/UCr] and in the second step [Definitive test], for those children who had UCa/UCr ratio more than 0.21 mg/mg we measured 24 hours urine calcium excretion. Children with secondary forms of hypercalciuria were excluded from the study. Children with idiopathic hypercalciuria were evaluated for manifestations of IH. Among 778 children, 195 [25.1%] had UCa/UCr ratio more than 0.21 mg/mg, but from these 195 children only 128 children delivered 24 hours urine samples. Among these 128 children for whom 24 hours urine calcium measurements were done 28 children excreted more than 4mg/kg/day calcium without hypercalcemia or any other known causes of hypercalciuria and we defined them as having idiopathic hypercalciuria. If all 195 suspicious cases of IH had delivered 24-hrs urine samples we would have 42 cases of IH. Prevalence of IH in our children was 5.4% and its manifestations were: hematuria, dysuria, recurrent abdominal pain, urinary incontinence, urgency, urinary tract infections and urolithiasis

High doses intravenous immunoglobulin versus oral cyclosporine in the treatment of severe atopic dermatitis

Atopic dermatitis is one of the most common allergic diseases that almost always respond to conventional therapies with topical emollient, topical corticosteroids, systemic antihistamines and allergic abstinence. However few cases of atopic dermatitis with severe course do not respond to conventional therapies and high dose of intravenous immunoglobulin or cyclosporine are recommended for them. This clinical trial study has been done to compare the last two regimens in patients with severe atopic dermatitis, Scoring Atopic Dermatitis [SCORAD] > 70. We included 14 patients in two groups. In group 1, eight patients were randomly selected and received 4mg/kg cyclosporine daily for 3 months and in group 2, six patients received 2g/kg Intravenous Immunoglobulin [IVIG] as stat infusion. All patients were followed on days 15, 30, 60 and 90 after starting the therapy. About 75% and 62.5% of patients had positive skin tests to egg and to milk respectively. Six patients out of 14 patients did not have skin test, so specific IgE by Radioallergosobent tests [RAST] was used for them. All of these patients had positive RAST to egg and 66.6% against cow's milk. There was a significant difference in the clinical outcomes of these two groups with a marked reduction in SCORAD of day 90[th] in group 1 in comparison to group 2 [Pvalue = 0.005]. No significant adverse drug reaction was seen in these two groups

Descriptive study of 226 patients with allergic rhinitis and asthma

The prevalence of allergic diseases such as asthma and allergic rhinitis is high in general population and aeroallergens are the most common allergens that cause air way inflammation. The aim of this study was an evaluation of clinical and laboratory findings in allergic patients and identification of the most common aeroallergen in these patients. A cross–sectional retrospective study was conducted on 226 allergic patients who were referred to allergic clinic of Karaj city, and skin prick test response to aeroallergens were studied. The most common risk factors in these patients were total IgE more than 100 IU/ml and a positive family history of atopy. Skin prick testing results showed that the most common aeroallergens were: herbacee II [62%], sycamore [57%], chenopodium [53%], tree mix [50%], herbacee III [47%], grass [43%], ash [40%], herbacee I [37%], cedar [27%], cockroach [25%], and mite D.P [19%], D.F [18%]. High prevalence of skin reactivity to weeds [chenopodium and herbacee] and sycamore indicates variation in the prevalence of aeroallergen reactivity in different regions with different climates

Neutropenia in patients with primary antibody deficiency disorders

Neutropenia is characterized by decrease in the absolute number of circulating neutrophils and an increase susceptibility to infections. The current study was performed in order to explain the clinical and laboratory findings of patients with antibody deficiency disorders associated neutropenia. The patients' records of 19 neutropenic cases out of 207 patients with antibody deficiencies, who had been referred to Children's Medical Center and enrolled in Iranian primary immunodeficiency registry, were reviewed. Nineteen cases [14 male and 5 female], with a mean age of 10.7 +/- 5.7 years, were associated with neutropenia [9.2%]. The disorders with associated neutropenia were Hyper IgM syndromes [3 of 8], Common variable immunodeficiency [13 of 109], and X-linked agammaglobulinemia [3 of 45]. The median age for the onset of disease and diagnosis age were 15 months [1-134] and 3.8 years [6 months-13 years], respectively. The most common infections during the course of illness were pneumonia [13 cases], diarrhea [12 cases], oral candidiasis [9 cases], otitis media [6 cases], sinusitis [6 cases], cutaneous infections [5 cases], and abscess [5 cases]. Other less frequent infections were: conjunctivitis, oral ulcers, meningitis, and osteomyelitis. Three neutropenic patients died because of recurrent infections. Neutropenia may occur in any of the primary immunodeficiency disorders. Persistent or severe infections always pose a supposition, which deserves further evaluation for detecting an underlying immune deficiency syndrome and neutropenia, since a delay in diagnosis may result in a serious organ damage or even death of the patient

Gastrointestinal manifestations of patients with Chronic Granulomatous Disease

Chronic Granulomatous Disease [CGD] represents a group of inherited disorders of phagocytic system, manifesting recurrent infections at different sites. The present study was accomplished in order to determine the gastrointestinal manifestations of CGD patients. Fifty-seven patients [38 males and 19 females] with CGD, who had been referred to three immunodeficiency referral centers in Iran, were studied during a 24-year period [1980-2004]. The median age at the time of study was 14.5 years old [1-56 years]. The median onset age of symptoms was 5 months [1 month - 13.75 years], and that of diagnostic age was 5 years [2 months- 54.1 years], with a diagnostic delay of 33 months, on average. Seven patients were presented with acute diarrhea, 3 with oral candidiasis, and 2 with liver abscesses as the first chief complaints. Twenty-four cases [42.1%] had been complicated by gastrointestinal manifestations during their course of the disease. Of those, 12 cases [21.1%] had diarrhea, 7 [12.3%] oral candidiasis, 5 [8.8%] hepatitis, 4 [7.0%] hepatic abscess, and 2 cases [3.5%] gastric outlet obstruction. Also, failure to thrive was detected in 6 patients [10.5%]. Four patients died [7%]. CGD should be excluded in any patient with gastrointestinal manifestations especially chronic diarrhea, hepatic abscess, and gastric outlet obstruction