Periodic fever: a review on clinical, management and guideline for Iranian patients - part I

Periodic fever syndromes are a group of diseases characterized by episodes of fever with healthy intervals between febrile episodes. The first manifestation of these disorders are present in childhood and adolescence, but infrequently it may be presented in young and middle ages. Genetic base has been known for all types of periodic fever syndromes except periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis [PFAPA]. Common periodic fever disorders are Familial Mediterranean fever [FMF] and PFAPA. In each patient with periodic fever, acquired infection with chronic and periodic nature should be ruled out. It depends on epidemiology of infectious diseases. Some of them such as Familial Mediterranean fever and PFAPA are common in Iran. In Iran and other Middle East countries, brucellosis, malaria and infectious mononucleosis should be considered in differential diagnosis of periodic fever disorders especially with fever and arthritis manifestation. In children, urinary tract infection may be presented as periodic disorder, urine analysis and culture is necessary in each child with periodic symptoms. Some malignancies such as leukemia and tumoral lesions should be excluded in patients with periodic syndrome and weight loss in any age. After excluding infection, malignancy and cyclic neutropenia, FMF and PFAPA are the most common periodic fever disorders. Similar to other countries, Hyper IgD, Chronic Infantile Neurologic Cutaneous and Articular, TRAPS and other auto-inflammatory syndromes are rare causes of periodic fever in Iranian system registry. In part 1 of this paper we reviewed the prevalence of FMF and PFAPA in Iran. In part 2, some uncommon auto-inflammatory disorders such as TRAPS, Hyper IgD sydrome and cryopyrin associated periodic syndromes will be reviewed

Morbidity and mortality in Iranian children with juvenile systemic lupus erythematosus

Juvenile systemic lupus erythematosus [JSLE] is a common rheumatologic disorder that involves multi organ systems. Prognosis of lupus in children may be poorer than in adults. In this study, we determined mortality and morbidity rates in the pediatric SLE in Iran. In a cross sectional study, we evaluated 120 children with SLE who had registered in our pediatric rheumatology database within 2004-2010. Data including sex, age, remission, age at the time of diagnosis, age at the time of study, various types of organ involvement, mortality and remission were extracted from this database. From 120 cases, 77% [92 cases] were females and 23% [28 cases] males [F:M=3.3]. Mean follow up period was 56 +/- 32 months and mean age at the time of manifesting disease 10.34 +/- 2.9 years. Mortality rate was 10% [12 cases] in our investigation. Musculoskeletal involvement showed significant difference between various age groups [P<0.01], that was more frequent in group of 7 years and older. Frequency of oral ulcer and ophthalmic involvement in boys was significantly higher than in girls [P<0.05]. Frequency of cardiovascular involvement [P<0.01] and renal involvement [P<0.01] was significantly higher in the patients who had no remission. There was a significant association between mortality rate and cardiac [P<0.02, OR=4.9], pulmonary [P<0.01, OR=10.167] and liver [P<0.05, OR=1.19] involvement. In our investigation 1-year survival rate was 97% and 5-year survival rate 89%. Liver, cardiac and pulmonary involvements have an association with higher mortality in JSLE patients

Neonatal lupus erythematosus following rheumatoid arthritis: case report and literature review

Neonatal lupus erythematosus [NLE] is a transient autoimmune disease of developing fetus and neonate in mothers with systemic lupus erythematosus [SLE]. In this report we introduce an infant with NLE whose mother had rheumatoid arthritis. Our case was a 40 day old male infant with discoid-like and annular skin lesions over forehead and neck, irritability and low grade fever. There was a history of prematurity due to preeclampsia. There was no cytopenia or cardiac involvement but liver enzymes were more than 5-fold increased. FANA, Anti Ro and La were negative. The mother had a history of un-controlled rheumatoid arthritis for 12 years with deformity in metacarpal and PIP and ulnar deviation in hands. FANA=1/640 and anti-SSB/La was positive in the mother but there was no other clinical and paraclinical sign of SLE. Without any treatment and during months, the skin and mucosal lesions gradually disappeared without any scar and liver enzymes reached the normal level. After 6 months follow up, he was symptom free with normal growth and development. We recommend to check anti SSA/Ro and anti SSB/La antibodies in all pregnant women with connective tissue diseases to prevent life-threatening involvement of the infant

Periodic fever: a review on clinical, management and guideline for Iranian patients - part II

Periodic fever syndromes are a group of diseases characterized by episodes of fever with healthy intervals between febrile episodes. In the first part of this paper, we presented a guideline for approaching patients with periodic fever and reviewed two common disorders with periodic fever in Iranian patients including familial Mediterranean fever [FMF] and periodic fever syndromes except for periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis [PFAPA]. In this part, we review other autoinflammatory disorders including hyper IgD, tumor necrosis factor receptor-associated periodic syndrome [TRAPS], cryopyrin associated periodic syndromes, autoinflammatory bone disorders and some other rare autoinflammatory disorders such as Sweet's and Blau syndromes. In cryopyrin associated periodic syndromes group, we discussed chronic infantile neurologic cutaneous and articular [CINCA] syndrome, Muckle-Wells syndrome and familial cold autoinflammatory syndrome. Autoinflammatory bone disorders are categorized to monogenic disorders such as pyogenic arthritis, pyoderma ;gangraenosum and acne [PAPA] syndrome, the deficiency of interleukine-1 receptor antagonist [DIRA] and Majeed syndrome and polygenic background or sporadic group such as chronic recurrent multifocal osteomyelitis [CRMO] or synovitis, acne, pustulosis, hyperostosis and osteitis [SAPHO] syndrome are classified in sporadic group. Other autoinflammatory syndromes are rare causes of periodic fever in Iranian system registry

[Epidemiologic investigation of juvenile rheumatoid arthritis: a ten-year study]

Background: Juvenile Rheumatoid Arthritis [JRA] is the most common chronic pediat-ric rheumatologic disease. There is a wide range of variation in disease subgroup distribution and evolution according to different populations, environments and genetic predisposition. This study was designed to evaluate epidemiologic characteristics of JRA in Iran

Methods: A cross-sectional studies was performed on children suffering from JRA according to American College of Rheumatology during 10 year [2002-2011]

Patients' data was recorded in hospital questionnaires; Patients' age at the first presentation and time of diagnosis, sex, ethnicity, early clinical and constitutional presentations, joint involvement pattern and presence of associated diseases were evaluated

Results: In this study, 171 children [91 boys and 80 girls] were enrolled. The mean age of patients was 5.2 +/- 3.5 years at the time of presentation and 5.7+/-3.6 years when the diagnosis was confirmed

The most common type of disease was polyarticular arthritis [41.9%]

The prevalence of pauciarticular and systemic onset subtype were 31.6% and 19.3%, respectively

According to ethnicity, the most common incidence was seen in Pars and Turk populations respectively

The most common constitutional presentation was fever

Fever and morning stiffness was significantly higher in polyarticular patients. Extra articular manifestation was seen in 40.4% patients. Among extra-articular manifestations, skin involvement was seen more than others

There was no correlation between sex and type of chronic arthritis. Uveitis was detected in 5.8% of our cases

The mean age was significantly higher in polyarticular group [6.04 year] in comparison to pauciarticular and systemic group [4.4 year in both groups] [P= 0.005]

There was no correlation between sex and type of chronic arthritis

Conclusion: The prevalence of JRA subgroups was different in Iran comparing with western countries and polyarticular disease was the most common subgroup in this study. Uveitis was less common in this study in comparison to studies in other countries. There is no sex predilection in each subgroup of JR

effect of positive family history of autoimmunity in juvenile idiopathic arthritis characteristics; a case control study

Patients with and without family history of autoimmune disease with respect to clinical features and laboratory data. Sixteen JIA patients with family history of autoimmune disease were identified during study, 32 patients were chosen for comparative group from referred patients to the rheumatology clinic according to the date of referral. Two groups were compared with respect to age of onset, sex, subtype, disease activity, duration of active disease and laboratory variables. The age of onset was significantly lower in JIA patients with family history of autoimmunity [4.7 years vs. 7.0 years; P=0.02], polyarthicular subtype was more frequent in patients with positive family history [50% vs.25%; P=0.04] most of JIA patients with positive family history were in the active phase at the time of study [64% vs 25%; P=0.02] and had a longer duration of active disease [21.0 months vs 12.3 months; P=0.04]. Patients with positive family history had more positive ANA [43.5%% vs 12.5%; P=0.01] and also more positive ADA [75% vs 20.8%; P=0.002]. Two groups were similar according to sex, and other laboratory variables. JIA patients with family history of autoimmune disease seem to have a more severe disease than patients without such family history, they are younger at the onset, and have mostly poyarthicular subtype. They also have more ANA and ADA positivity. These findings are different from familial JIA case-control studies according to active disease duration, subtype, and ANA positivity

Prevalence of fungal skin infections in Iranian wrestlers

Fungal infections are one of the most common skin infections. Athletes, especially in contact sports such as wrestlers are the group at risk of fungal skin infections [FSI]. The aim of this study was to determine prevalence of FSI and some effective factors in wrestlers in Tehran, Iran. This study was a cross-sectional descriptive study which was conducted in 2009 and subjects were wrestlers of wrestling clubs of Tehran, Iran. In this study the prevalence of FSI and its related factors was collected based on clinical history, physical examination and laboratory tests. We also determined the rate of dermatophytic contamination of wrestling mats. The relationship between independent variables and incidence of fungal infection analyzed by Chi square test and regression analysis. In this study, out of 454 wrestlers, 111 [24.5%] subjects had suspicious fungal skin lesions on physical examination. The Prevalence of FSI was 8.2% in all of the wrestlers [34.2% of the wrestlers with suspicious lesion]. Malassezia furfur [50%] and trichophyton tonsurans [30%] were the most common causative agents. Epidermophyton floccosum [7.5%], Trichophyton rubrum [5%], Candida albicans [5%] and Trichophyton mentagrophytes [2.5%] were other isolated fungi. Eleven wrestling mats [44%] were contaminated with different fungal organisms. The prevalence of fungal skin infection in Iranian wrestlers is relatively high. Appropriate preventive measures need to be prepared and implemented to reduce incidence of FSI in wrestlers

effects of SPARK physical education program on fundamental motor skills in 4-6 year-old children

The purpose of this study was to investigate the effect of SPARK Physical Education [PE] program on fundamental motor skills in 4-6 year children. SPARK [Sports, Play, and Active Recreation for Kids] is an evidence based PE program designed in order to promote the lifelong wellbeing. In total, 90 children aged 4 to 6 years were selected randomly. The children were allocated into 3 groups with separate PE programs: 1-SPARK, 2-Gymnastics and 3-Routine activity. Using the Test of Gross Motor Development [TGMD-2], a pretest was done in all groups. Afterwards, SPARK and Gym PE programs were performed for 8 weeks and 3 sessions each week. The third group used to do the routine physical education program in their daycare. After 8 weeks [24 sessions], the post tests were done for all groups with the same scoring system as the pretest. The results showed that the SPARK program had a higher efficacy on the promotion of the fundamental motor skills comparing to the routine physical education programs or gymnastics PE group. SPARK can be used as an appropriate alternative in order to promote the children's motor skills

Anger in adolescent boy athletes: a comparison among judo, karate, swimming and non athletes

Karate and judo are originally Japanese martial arts which may have different influences on adolescents' behavior. This study was conducted to examine the total anger rate and its subscale-reactive anger, instrumental anger, and anger control-rates in young karateka and judoka. A cross-sectional study was carried out in 11 to 19-year old boys. Adolescents included in the study were judoka [n=70], karateka [n=66], swimmers [n=59], and non athletes [n=96]. One stage cluster sampling method was used to select judoka, karateka, and swimmers from sport clubs in Tehran. Students of governmental schools at the same area were chosen as the non-athletes group. The "Adolescent Anger Rating Scale" questionnaire was utilized to assess the anger rate. The mean age of participants was 12.90[ +/- 2.06] years. The total anger rates were 45.40 [ +/- 5.61] in judoka, 41.53[ +/- 5.63] in karateka, 41.19[ +/- 5.33] in swimmers, and 45.44 [ +/- 8.58] in non athletes. In total anger scale karateka and swimmers had a significantly lower score compared to judoka and non athletes. In instrumental anger subscale the difference was significant just between karateka and non athletes. In reactive anger subscale judoka showed higher scores than swimmers. In anger control subscale the difference was significant between judoka and swimmers and also judoka and karateka. The difference of anger control between karateka and non athletes was significant. The findings of this study propose a difference in the anger rate between judoka and karateka. In contrary to the results of previous studies, judo training may have no influence on anger control, while karate training could be beneficial

Characteristics of headache at altitude among Trekkers; a comparison between acute mountain sickness and non- acute mountain sickness headache

Headache at altitudes has had an incidence of 25-62% through many related studies. Many reasons are identified concerning headache at altitudes such as acute mountain sickness [AMS], sinus headache, migraine, tension type headache, and frontal tension headache. This study tried to compare different types of headache among trekkers on Mount Damavand, a 5671m mountain, Iran, to find their incidence and related symptoms and signs. Through a cross-sectional study, we evaluated headache incidence and its correlation to AMS among people who climbed Mount Damavand. Lake Louise Score, a self-report questionnaire, was applied to make AMS diagnosis through three separate stages of trekking program. Chi-square test was employed as the main mean of analysis. Totally, 459 between 13-71 years old participated in the study among which females were 148 [32.1%] and males 311 [67.8%]. Headache was found in 398 [86.7%] among whom 279 [70%] were proved as AMS. Investigating the types of headache in the cases of AMS showed 64.5% steady, 31% throbbing, and 4.5% stabbing characters which had significant differences with a P value = 0.003. The majority of headaches were stated as frontal [38.9%] and the least prevalence belonged to parietal area [4.4%], while global headache was reported 27%. This study specifies the exact location of headaches at altitude in cases of AMS and non-AMS headaches. Many cases of high altitude non-AMS headache are resulted by tension and light reflection at altitude

Dental problems in hypophosphatemic rickets, a cross sectional study

Hypophosphatemic rickets is an uncommon metabolic bone disorder which affects all ages and both sexes. It is characterized by low concentration of serum phosphate levels, impairment of mineralization of bone matrix and teeth with variable etiology. Dental problems in this disorder have not been described well in previous studies. All hypophosphatemic rickets patients who came to a referral clinic during 2008-2010 enrolled in this study. All patients had low phosphorous and high ALP, normal PTH and 25-hydroxy-vitamin D and normal or low level of serum calcium. After diagnosis all patients were examined by a dentist for enamel hypoplasia, taurodontism, dental abscess, gingivitis, dental caries, and dentition delay. Nineteen patients were enrolled in this study. The average age of the patients was 10 [ +/- 4.23] years [range 3-17]. Seventy-nine percent of patients had regular follow-up after diagnosis of background disease. Dental caries and delay in the dentition were most prevalent [each one 47.7%] followed by enamel hypoplasia in 42.1% of the patients. Other problems were taurodontism in 15.8% patients, dental abscess and gingivitis in 10.9%. Hypophosphatemic rickets is a disease with different clinical features; one of them is dental problem, dental caries is the most common problem

Effects of selected exercises on elementary school third grade girl students' motor development

The aim of this study was to examine the influence of selected exercises on motor development in elementary school thirds grade girl students in Ahwaz City, Iran. The present study was semi-experimental, performed as field and using experimental and control groups. The statistical population included all third grade girl students of elementary schools of Ahvaz city [10,500 subjects]. Forty of whom were randomly selected [cluster stage] and then were divided randomly into two experimental [N=20, Mean age=8.9 +/- 0.49] and control [N=20, Mean age= 8.9 +/- 0.48] groups. Experimental group was given training for eight weeks, 3 sessions per week and each session lasting 45 minutes. To measure motor development, the Test of Gross Motor Development, edition 2 [TGMD-2; 2002] was applied. Statistical analysis was performed using independent t-test and paired t-test. The results showed statistically significant differences between control and experimental groups regarding locomotion skill [8.433, P<0.05], manipulation skills [10.95, P<0.001] and overall motor development [13.203, P<0.001]. In fact, selected exercises impacted on the motor development of subjects and led to their motor development progress. The selected exercises can be used as an appropriate plan to improve motor skill development in third grade of elementary school

Juvenile Churg-Strauss syndrome as an etiology of myocarditis and ischemic stroke in adolescents; a case report

Churg-Strauss syndrome [CSS], a systemic vasculitis accompanied by asthma and eosinophilia, almost invariably affects the lung and is frequently associated with cutaneous involvement It rarely has cardiac involvement. We report an unusual case of CSS with myocardial. A 16-year old female suffered of allergic asthma for 4 years. She was under treatment with oral prednisolone and seretide inhalation. After CSS diagnosis, she developed paroxysmal atrial tachycardia. Serum levels of Troponin I and Troponin T were increased indicating massive myocardial damage probably due to myocarditis. After 5 months she developed acute hemiparesis without any evidence of ischemic or hemorrhagic event. She was treated with IVIg, intravenous pulses of methylprednisone and cyclophosphamide for each complication. Myocarditis and stroke may also complicate CSS which should be taken in consideration for better management

Pleuritic chest pain; where should we search for?

Pleuritic pain is not an unusual problem in children. Other concomitant symptoms should be considered for diagnostic approach in a child with pleuritic chest pain. In this report we discuss chest pain in a 6-year-old child with regard to other signs and symptoms. Finally, we found a rare life-threatening complication of juvenile systemic lupus erythematosus [JSLE] in our patient

Bilirubin/albumin ratio for predicting acute bilirubin-induced neurologic dysfunction

The aim of this study was to evaluate the bilirubin albumin [B/A] ratio in comparison with total serum bilirubin [TSB] for predicting acute bilirubin-induced neurologic dysfunction [BIND]. Fifty two term and near term neonates requiring phototherapy and exchange transfusion for severe hyperbilirubinemia in Children's Medical Center, Tehran, Iran, during September 2007 to September 2008, were evaluated. Serum albumin and bilirubin were measured at admission. All neonates were evaluated for acute BIND based on clinical findings. Acute BIND developed in 5 [3.8%] neonates. B/A ratio in patients with BIND was significantly higher than in patients without BIND [P<0.001]. Receiver operation characteristics [ROC] analysis identified a TSB cut off value of 25 mg/dL [area under the curve [AUC] 0.945] with a sensitivity of 100% and specificity of 85%. Also, according to the ROC curve, B/A ratio cut off value for predicting acute BIND was 8 [bil mg/al g] [AUC 0.957] with sensitivity of 100% and specificity of 94%. Based on our results, we suggest using B/A ratio in conjunction with TSB. This can improve the specificity and prevent unnecessary invasive therapy such as exchange transfusion in icteric neonates

Acute hemorrhagic edema of infancy; a report of five Iranian infants and review of the literature

Acute hemorrhagic edema of infancy [AHEI] is a benign self limiting leukocytoclastic vasculitis in young children. Serious complications, e.g. renal and gastrointestinal involvement, are not usually detected in AHEI patients. We report five patients with AHEI. Our patients were 17 to 21 months old. One patient presented with gastrointestinal bleeding due to this syndrome, the other one experienced second attack and scrotal edema due to epididymo-orchitis, while the third patient had renal involvement as hematuria and the other one had bilateral auricular chondritis. One of our cases was a typical case of AHEI without any complications, so a skin biopsy was not necessary. In this study, we describe the symptoms, probable triggering factors and treatment of choice for each patient. Although AHEI is a childhood vasculitis with no impairment of the general condition, some organ involvements such as gastrointestinal, renal or scrotal lesions and rarely chondritis are probable in these patients

Injury rates in Iranian Taekwondo athletes; a prospective study

Taekwondo, as the most popular martial art among Iranian sportsmen, might lead to injury for the athletes of this sport during competitions. We decided to report the incidence rate of injuries sustained by the athletes of this sport during national competitions. All competitions of Iran national championship taekwondo league in 2006-2007 with 204 athletes were observed prospectively to detect the occurrence of injuries. The severity of injuries was classified into four groups [mild, moderate, severe, and critical] according to the involvement of medical care team in the contest, ability of the athletes to resume and duration of probable absence of injured athletes from future competitions and training sessions. Athlete-Exposure [A-E] was defined as the number of competitions multiplied by two. On this base, the rate of injury incidence per 1000 A-Es, the rate of injuries per time unit and the rate of injury occurred for each 100 athletes were considered as the major outcomes of this study. Of totally 1,338 A-Es, 93 injuries were recorded during the competitions. The rate of injury incidence was found to be 69.5 injuries per 1000 A-Es and the rate of injuries per minute of competitions was 0.023 which corresponded to 23.3 injuries per 1000 minutes of competitions. 45.6 injuries were occurred for each 100 athletes during the course of competition. The most frequent injuries were mild [68.8%] and critical injuries [24.7%], followed by moderate and severe injuries; 4.3% and 2.1%, respectively. The rate of injury we found was lower than that of western countries. In spite of finding the lower limbs as the most frequent place of injuries in other studies, we found the upper limbs as the most predisposed place of injuries which might be due to difference in the method of combat of Iranian athletes with other athletes

Injury rates in Iranian Taekwondo athletes; a prospective study

The objective of this study was to compare the Iranian taekwondo-in statistically in terms of total injury rates to international counterparts as gleaned from the extant literature. The Iranian sample consisted of 204 male taekwondo-in participating in the national championship. The international sample included the participants in national and international tournaments. Validated standard questionnaires were employed at all tournaments to collect injury data that were always diagnosed by the respective tournament physicians. An injury was defined as any circumstance for which assistance was sought from the medical personnel. In addition to injury rates, 95% confidence intervals [CIs] around the rates were computed. To assess which group was at higher risk, odds ratios were calculated, including the 95% CIs. Compared to Greek counterparts, the injury rate for the Iranian taekwondo-in was statistically significantly higher. The Iranians were also at a higher risk to incur an injury: OR =11.2 [95%CI: 6.60 - 18.88, P<0.001, CLR=2.86]. When comparing the Iranian taekwondo-in to their colleagues competing at the 1999 World Championships, the former recorded a statically significantly lower injury rate but the latter were not at a higher risk [OR=0.61, 95%CI: 0.41 - 0.91, P==0.014, CLR=2.20]. A statistical comparison of total injury rates in Iranian and international taekwondo-in revealed no difference between the two groups. However, what is of concern is that the total injury rate across taekwondo studies is significantly higher than those reported for American football

national experience on physical activity initiatives for adolescent girls and their mothers: CASPIAN study

To provide a low- cost and simple model of culturally-appropriate and low cost facilities for improvement of physical activity for girls and their mothers through an after-school program and to determine the changes in anthropometric indexes after this trial. This national study was conducted in 2006-2007 in 7 provinces with different socioeconomic situations in Iran. Female students who studied in the 7[th] through 10[th] grade and their mothers were selected by random cluster sampling. In each province, 24 sessions of after-school aerobic physical activity were held for 90 minutes, two days a week, and 3 months long at school sites in the afternoon. The study comprised 410 participants [204 mothers and 206 daughters], with a mean age of 15.86 +/- 1.01 and 40.71 +/- 6.3 years in girls and their mothers, respectively. The results of the focus group discussions showed that in general, both mothers and daughters of generalized and abdominal obesity improved significantly both in girls and in their mothers [P-value <0.0001 for weight, body mass index and waist circumference]. Our findings may provide a low-cost and simple effective model of motivation for physical activity with targeted interventions for girls and their mothers. We suggest that the success of this trial might be a result of bonding and accompaniment of mothers and daughters. Such model can be integrated in the existing health and education systems to increase the physical activity level