International Journal of Pediatrics
Volume:6 Issue: 49, Jan 2018

Congenital pulmonary malformations (CPM) comprise a group of anatomical abnormalities of the respiratory tree including congenital cystic malformations, bronchopulmonary sequestrations, bronchogenic cyst, bronchial atresia and congenital lobar emphysema.
Socioeconomic, demographic, and hospital factors were determinants of survival of CPM. Antenatal diagnosis should be developed in order to improve management and prognosis of CPM in country with low resource.

Chloramphenicol inhibits protein synthesis in bacteria and is usually bacteriostatic but is bactericidal against Haemophilus influenzae, Streptococcus pneumoniae, and Neisseria meningitis. Chloramphenicol penetrates all body tissues well. The cerebrospinal fluid concentration averages 60% of the serum level, while brain levels are 9 times higher because of high lipid solubility of this drug. Chloramphenicol acts primarily by binding reversibly to the 50S ribosomal subunit. This antibiotic is the drug of choice for the treatment of typhoid and paratyphoid fevers and bacterial meningitis. Chloramphenicol possesses a broad-spectrum of antimicrobial activity. Strains are considered sensitive if they are inhibited by chloramphenicol concentrations of ≤ 8 µg/ml. Neisseria gonorrhea, Brucella species, Bordetella pertussis, gram-positive cocci, Clostridium species, and gram-negative rods including Bacillus fragilis are inhibited by chloramphenicol. Most anaerobic bacteria including Mycoplasma, Chlamydia, Rickettsiae, Vibrio cholera, Escherichia coli and Klebsiella pneumoniae are inhibited by this antibiotic. The doses of chloramphenicol are 40.5 mg/kg/day for neonates and 75.5 mg/kg/day for older children. The therapeutic concentrations of chloramphenicol are 10-25 µg/ml. Peak therapeutic concentrations are obtained in 60% and therapeutic trough concentrations are found in 42% of children. Children affected by typhoid fever are cu red by chloramphenicol and the sensitivity to this antibiotic is 100%. Acute bacterial meningitis is the most dangerous infections disease in children. The causative organisms are gram-positive and gram-negative bacteria, and chloramphenicol is effective in killing these microorganisms. The aim of this study is to review the management of typhoid fever and bacterial meningitis in infants and children by chloramphenicol.

Trevor disease or dysplasia epiphysealis hemimelica (DEH) is an extremely rare condition with incidence of about 1:1,000,000. Male to female ratio of reporting case is 3:1, and usually diagnosed between two and eight years old. It usually affects the medial portion of the joint, but lateral involvement is not uncommon. Hip-joint was affected in less than 4% of existing cases in the literature. It would be very important to precisely mange the hip involvement to prevent from further articular cartilage destruction in this very young age.
We report an infant boy with isolated DEH of hip. We found a total of 271 cases of DEH that reported between 1926 and 2017.The most sites of involvement are ankle joint and around the knee. Our search reaches out to ten cases of hip involvement. Hip involvement needs a patient specified decision. We observed our patient for three years with a desirable hip joint function.

Urinary tract infection is a common disease in childhood. The aim of this study was to determine the diagnostic performance of urinary analysis, assess the role of urine culture in determining its necessity and evaluate etiologic agents and antimicrobial resistance patterns in children with urinary tract infection.
Our study was made by evaluating the patients who applied to the Antalya Research and Training Hospital- Turkey, between 2015 and 2017. A total 237 urine analysis and urine culture were retrospectively analyzed. Culture results were taken a reference for microscopic and chemical examination of urine and diagnostic accuracy of the test parameters, and the performance of urine analysis were calculated. The culture and antibiogram results were examined and antibiotic resistance with infectious agents frequency was evaluated.
The 42.4% of culture negative samples showed leukocyte esterase, nitrite, bacterial and leukocyte counts, which are indicative of infection in urine analysis, were found in normal range. The highest sensitivity (90%) was in the presence of leucocyte esterase and bacteria, while the highest specificity (99.4%) was in the presence of nitrite alone or with other components (leucocyte or leucocyte esterase). The highest antibiotic resistance was found in beta lactam antibiotics. The lowest antibiotic resistance was detected in the carbapenem followed by fluoroquinolone group antibiotics.
Microscopic and chemical examination of urine analysis can give us information about urine culture requirement. The observation of increasing overall resistance to antibiotics authorize further studies that lead to new recommendations to antibiotic use in children and adolescents.

Parent's attachment to the child is an intimate,warm and continuous relationship which is the basis of the natural development of the child. Attachment starts long before birth, and is affected by a variety of factors that are not definitively recognized. Also, several interventions have been proposed for improving it that their effectiveness has not yet been determined. Given the evidence about the role of cultural and national differences, it is necessary to review existing studies in order to identify these factors and interventions in Iran.
Methods and Materials: In this review, Web of Science, Scopous, Proquest,Psycinfo, CINAHL and Pubmed databases and SID, Magiran, Irondoc, Barakat Knowledge Network System as Iranian databases were searched using English and Persian keywords such as prenatal attachment, relationship, maternal attachment between 2000 and 2017, to find articles related to prenatal attachment. The full text of the articles was studied by two reviewer and their main findings were extracted and categorized.
Factors and interventions associated with parental attachment summarized into 12 themes: parent education, culture, anxiety, family, planning for pregnancy, history of fetal loss, substance abuse, postpartum attachment, fetal anomaly, paternal attachment, attachment measurement tools, and effectiveness of education on prenatal attachment .
the effect of education and counseling on prenatal attachment in Iranian parents suggests the use of these methods in prenatal care. Parent’s education, social support and marital satisfaction were significant associated factors with increasing maternal attachment. History of fetal loss, anxiety and smoking was associated with the poor prenatal attachment .

Obesity constitutes a crucial health issue during preschool period and has an impact on children regardless their ethnic backgrounds. The purpose of the study was to examine the self-efficacy perceptions of mothers and potential differences and correlations with dietary behaviour and physical activity of their preschool children in Finland.
The sample for this study consisted of 154 mothers from nine private nurseries who lived in greater Helsinki and have Finnish or other nationality. There were 7 categories of geographical regions from which mothers came from, according to the nationality they declared. For the data collection the "Parental Self-Efficacy Questionnaire" was used, which evaluates the self-efficacy of parents regarding the dietary behaviour and physical activity of their children.
The age range of the children was between 3 up to 6 years of age (Mean=5.08 0.96), while the age range of mothers was between 25 up to 54 years of age (Mean=37.7 4.85). Positive correlations were found between maternal self-efficacy and children’s physical activity as well as between maternal self-efficacy and dietary behaviour. As it occurs from the analysis of the results from Pearson correlations: dietary behaviour had a positive correlation with physical activity r=0.583, p
Despite the non-statistically significant differences on these two factors, the results also showed high mean score values on maternal self-efficacy so in physical activity, as in dietary behaviour. Mothers who living in the same country which offers well structured guidelines about integrating nutrition and physical activity to help prevent lifestyle related diseases, possibly explains the fact that there are no differences related to ethnicity.

Appendicitis is the most common indication for an emergency operation in children's age. Although none of the laboratory values has not high sensitivity and specificity for the diagnosis of appendicitis, leukocyte count and the percentage of segmented neutrophils are most commonly used. The aim of this study was to determine whether there is a statistically significant correlation between leukocyte count and the percentage of segmented neutrophils compared to the pathohistological finding of appendix in children.
We retrospectively analyzed the data in the period from 1 January 2016 to 31 December 2016. The analysis was made on 211 patients. Spearman's correlation coefficient (rs) was calculated. We determined the specificity and sensitivity of leukocyte count and the percentage of segmented neutrophils used in the calculation of Alvorado and Pediatric Appendicitis score.
The results of the research have shown that the correlation between leukocyte count and the pathohistological findings is weak (rs = 0.29, p = 3.61*10-8), while there is no correlation between the percentage of segmented neutrophils and pathohistological findings (rs = 0.18, p = 7.08 *10-5). The sensitivity of leukocyte count is 93% and the specificity is 30%, while the sensitivity to the percentage of segmented neutrophils is 71% and the specificity is 50%. ROC analysis for leukocytes shows area under the curve of 0.648, while for segmented neutrophils of 0.574.
Given the correlation results obtained, the clinical experience of physicians will still have one of the leading roles in diagnosing acute appendicitis in children.

The most common cause of admission to neonatal intensive care units (NICU) is respiratory distress syndrome. One of the respiratory assistance methods is using nasal continuous positive airway pressure (CPAP). Regarding the importance of pain control which is one of the major priorities in neonatal nursing care, this study aimed to evaluate the effect of body position on pain due to nasal CPAP in premature neonates.
In this cross-over clinical trial, 50 premature neonates who were receiving nasal CPAP admitted to the NICU of Imam Reza Hospital, Kermanshah, Iran, were included. The neonates were randomly placed at three body positions (fetal, supine, and prone positions). Pain was measured by Astrid Lindgren Children’s Hospital Pain Scale Neonates (ALPS-Neo) pain assessment scale. The collected data were analyzed using the SPSS software (Version 22.0).
Significant difference existed regarding pain of nasal CPAP among body positions (p Body positioning in premature neonates under nasal CPAP in NICU can be effective as a non-pharmacologic method in alleviating pain due to nasal CPAP. Among the studied positions, the lowest pain score was seen in fetal position.

Case Report: Two patients affected by complicated vascular anomalies were treated with sirolimus.
Case 1: fourteen-month-old male patient with primary congenital lymphedema in the right arm.
Case 2: boy aged two years and eight months, diagnosed with a non-respectable cervical aposiform hemangioendothelioma (KHE). Both patients received sirolimus at doses of 0.8 mg/m2/12 h during 12 and 13 months respectively. Monitoring plasma sirolimus concentrations made it possible to safely deal with problems associated with the medication. Both patients experiment an important improvement of their pathology, maintained after suspending treatment. No adverse effects related to treatment with sirolimus were observed.
Oral sirolimus proved to be an effective strategy in the treatment of complicated vascular anomalies in our pediatric population.

Despite of healthy nutrition recommendations have been offered in recent decades, researches show an increasing rate of unhealthy junk food consumption among primary school children. The aim of this study was to investigate the effects of health promotion intervention on the school food buffets and the changes in nutritional behaviors of the students.
In this Quasi-interventional study, eight schools agreed to participate in Tabriz city, Iran. The schools were randomly selected and divided into an intervention and a control group, and a pretest was given to both groups. A four weeks interventional program was conducted in eight randomly selected schools of the city based on the socio-ecological model. A check list was designed for the assessment of food items available at the schools’ buffets, a 60-item semi-quantitative food frequency questionnaire (FFQ) was used to assess the rate of food consumption and energy intake. Results evaluation and practice were analyzed using the Wilcoxon, Mann Whitney-U and Chi-square tests.
The findings revealed reduction in the intervention group between before and after intervention with regard the range of junk food consumption, except for the sweets consumption. The number of junk foods provided in the schools buffets reduced in the intervention group. After the intervention on the intervention group significant decreases were found in the intake of energy, fat and saturated fatty acids compared to the control group (p = 0.00).
In order to design effective school food environment promotion programs, school healthcare providers should consider multifaceted approaches.

The appendix is one of the most common sites for carcinoid tumors. Most carcinoids are found in appendices removed incidentally at laparotomy for conditions unrelated to acute appendicitis. We describe the case of a 13-year-old female who presented with abdominal pain in the right lower quadrant (RLQ), with nausea and decreased appetite for the previous 2 days. A physical examination favoreda diagnosis of acute appendicitis. A carcinoid tumor was diagnosed based on the histological examination of the removed appendix. The patient underwent an isolated appendectomy due to the small size of the lesion.

Postoperative intussusception is a rare complication after various kinds of operations, the incidence of POI is 0.01–0.25 % of children following laparotomy. We reported and analyzed a case of a patient who developed postoperative intussusception following laparotomy for Nissen fundoplication.
Postoperative intussusception differs from the other cases of invagination with respect to the pathogenesis, clinical presentation, localization, and therapeutic approach. Diagnosis must be suspected in patient who had occlusion symptoms after abdominal surgery and ultrasound was highly accurate in diagnosing postoperative intussusception in children.

Hypohidrotic ectodermal dysplasia (HED) is a rare genetic disorder, distinguished by hypotrichosis, hypohidrosis, and hypodontia. HDE can be inherited in X-linked recessive manner as a result of mutations in the ectodysplasin A (EDA) gene as well as autosomal dominant and autosomal recessive manners both of them caused by mutations in EDA receptor (EDAR) and EDAR-associated death domain (EDARADD) genes.
Findings: In this report, we investigated a consanguineous Iranian family with autosomal recessive form of HED. A homozygous missense mutation was detected in exon 1 of EDAR gene in the proband (c.278C>G) resulting in p.C93S that alters the sequence of the EDAR protein.
We facilitated the effective genetic counseling and prenatal diagnosis in this family through detection of the disease causing mutation.

Septicemia is a serious condition in pediatric population which highly depends on immediate diagnosis and treatment in terms of mortality and morbidity rate. Developing adjunctive laboratory tests to help clinicians make appropriate decision is of great priority. In this study we aimed to evaluate the predictive value of each biomarker to find the most valuable one based of which a reasonable empiric therapy would be initiated prior to definite diagnosis by blood culture.
Hospital records of Patients who referred to the Hazrat-Masumeh hospital of Qom province were reviewed between March 2013 and March 2017. Diagnostic tests including blood culture and hematologic indices (WBC, ANC, ABC and Platelets) as well as inflammatory biomarkers (ESR, CRP) were measured for all participants. Patients over 6 year-old and those received antibiotic within a week prior to admission were excluded from the study.
Total number of 378 patients was enrolled in this retrospective study, of which 200 (52.91%) were boys. Positive blood culture was achieved in 171 (45.23%) patients. Significant increase observed in ANC, ABC, Platelets, ESR and CRP levels among patients with positive blood culture compared with negative blood culture (P-value of 0.0012, Measuring the ANC, ABC, Platelet, ESR, CRP levels as initial evaluation in adjunction to confirmative blood culture is reasonable approach with acceptable diagnostic value for patients suspected to septicemia.

The prevalence of hepatitis B and C in Iranian children with chronic kidney disease is limited. Therefore, the present study intends to assess the prevalence of hepatitis B and C in children with chronic kidney disease (CKD).
The present study is a historical cohort study which was conducted in a window period of 25 years in Children’s Medical Center in Tehran, 1991-2016. Data (age, gender, duration of hemodialysis, kidney transplant, and severity of CKD) were extracted from hospital profiles of admitted patients. Infection with hepatitis B or C viruses was considered as primary outcome. At the end, results were reported as odds ratio (OR) with a confidence interval of 95 per cent (95% CI).
Three hundred and fifty five children (50.1% boys, mean age of 54.5±89.0 months) were assessed. Hepatitis B and hepatitis C were detected in 9 (2.5%) and 5 (1.4%) children, respectively. Ten children had either hepatitis B or C infection with a prevalence of 2.8% (95% confidence interval: 1.4% to 5.1%). Multivariable analyses showed that association between the need for hemodialysis (OR=13.52; p=0.083) and severity of chronic kidney disease (OR=0.28; p=0.072) with incidence of hepatitis infection was borderline. However, risk of hepatitis B or C infection was 5.9-fold greater in girls compared to boys (OR=5.94; p=0.047).
The present study showed that the prevalence of hepatitis B and C were 2.5% and 1.4%, in children with chronic kidney disease, respectively. The prevalence of mentioned infections was significantly higher in girls compared to boys.

Family coping is an active process in which the family and each member of it use the available resources to decrease or control the demand. Using effective coping strategy, a family can react to stresses and adapt better to its situation. The purpose of this study was to examine psychometric properties of a Persian version of Family Crisis Oriented Personal Evaluation Scales (F-COPES) in a population of Iranian mothers with cerebral palsy children.
F-COPES was translated into Persian following Wild et al.’s model, face and content validities of the translated version were determined by 12 faculty board members in different nursing fields, psychology, and tool development. Afterward, to fill out the scale, 208 mothers with cerebral palsy children who had referred to Kermanshah based rehabilitation centers were selected through convenient sampling. Construct validity of the tool was determined by using confirmatory factor analysis. To examine internal consistency, Cronbach’s alpha and to examine reliability, intraclass correlation coefficient (ICC) (15 participants with 2 weeks interval) was used. The collected data was analyzed in SPSS (version 16.0) and EQS6.1.
Confirmatory factor analysis supported validity of the whole tool and its five subscales. Cronbach’s alpha of the whole tool was obtained alpha=0.84 and the alpha of the five subscales ranged from 0.76 to 0.94. In addition, ICC was obtained equal with 0.89.
The Persian version of F-COPES was featured with acceptable psychometric properties so that it can be used to survey family coping in Iranian mothers with cerebral palsy child.

The prevalence of burn in children is more than other age groups. This study aimed to "investigate the factors associated with the burn degree and total body surface area (TBSA) in under five-year-old children of Kermanshah ".
We studied under five-year-old children hospitalized in the burn center of Imam Khomeini hospital in Kermanshah from September 2014 until March 2016 for 18 months. Required Information extracted by trained staff based on existing data. Researcher-made checklist was used to obtain information. The ordinal regression has been applied to evaluate factors related to TBSA and burn degree in children.
In general, 262 children were input of the study so that 226 cases (86.3%) were in under two-year-old group. The majority of cases were boys by 66.8% (175 cases). Hot liquid was the most reported cause of burns by 68.7% of all cases. Most of the burn incidents (43.1%) with high burn degree occurred at 7 am to 2 pm. Girls were exposed to higher burn degrees more than boys. Factors such as being boy (OR=2.83), less than 2 years old age (OR=4.91) significantly increased TBSA. Also, living in rural (OR=5.17) and delay of treatment (OR=41.35) significantly increased burn degree.
To reduce the incidence and complications of burns in children, interventions should be considered to change the environmental and individual factors.

Adolescence pregnancy is high risk both for mother and child. This systematic review aimed to determine the effect of home visiting on maternal and neonatal outcomes in adolescent mothers.

This systematic review was performed by searching English databases including Cochran library, PubMed, Google scholar, Scopus, web of science, Embase, Ovid and Persian databases including SID, Magiran, and Barakat Knowledge Network System without time limitation. The search terms included "adolescent or teen pregnancy", "adolescent or teen mothers", "home visiting", "home visitation" and "home visit".

According to databases search, 967 papers were found that among them 913 papers were not related. Among 54 related papers, 44 abstracts and 10 full texts were studied. At the end, 7 RCT included in this systematic review. The meta-analysis result done on 375 person indicated that mental health in the home visiting group was significantly better than the control group (routine care or cares except considered intervention) (standard mean difference: -0.33; 95%CI: -0.57 to -0.10; p=0.006, I2=0%). Also, meta-analysis done on 185 persons showed that there was no significant difference between two groups in terms of repeat pregnancy (odds ratio: 0.83; 95% CI: 0.33 to 2.03; p=0.67; I2=50%) and repeat birth (odds ratio: 0.90; 95%CI: 0.35 to 2.31; P= 0.820, I2=0%).

Results indicates that home visiting can improve mental health but does not have any effect on repeat pregnancy and repeat birth. Clinical trials with accurate methodology by controlling effect of number and duration of home visiting are recommended.

Fetus-In-Fetu (FIF) is a rare congenital malformation, which consists of a fetus mixed with a distinct tissue that is from the other fetus of twin. FIF is defined as a mass containing a vertebral axis often associated with other organs or limbs around this central axis.
Case report: The patient was a two day old girl infant who due to a mass in a sacrum area measuring 8.5 x 12.5 cm with soft consistency containing a normal skin color fluid with blood streaks and hemorrhagic and bruise colored patches since birt, was admitted at the Taleghani children's Pediatric Center of Gorgan. The results of the mother's ultrasound in the fourth month of pregnancy had identified the cyst for the fetus. In the primary results of imaging, the first the diagnosis was type 2 sacrocoxigeal teratoma. However, results of post-birth graphy confirmed the presence of fetal bone structure in the infants's sacrum, so FIF was diagnosed for the infant.
In the present study, FIF has been seen in sacrum, which is a very rare case in FIF.

Self-extubation is a problem in pediatric intensive care unit (PICU) and can be harmful for children. Level of sedation is a determining factor for self-extubation. The aim of this study was to compare thr rate of self-extubation and duration of ventilation following different sedative modalities.
This prospective and randomized study was done in Mofid Children Hospital, Tehran, Iran from October 2015 to September 2016. One hundred and fifty seven children from 1 month to 15 years of age with normal consciousness level requiring mechanical ventilation were included.The rate of self-extubation, and duration of ventilation were compared among 3 groups; Midazolam group (n= 50) 0.1 mg/kg/hr, Fentanyl group (n= 50) 2 mcg/kg/hr., and Midazolam-Fentanyl group (n= 57); midazolam 0.05 mg/kg/hr and fentanyl 1 mcg/kg/hr. All administrations were IV. Optimal level of sedation was 2-3 using Ramsay score.
One hundred and fifty seven children were studied. The mean age of patients was 4.5 ± 1.5 years with an excess of boys (57.9% vs. 42.1%). Rate of self-extubation was lower in Midazolam-Fentanyl group; the difference was significant among 3 groups (p This study showed that combination therapy with Fentanyl Midazolam was associated with lower rate of self-extubation and shorter duration of ventilation. In addition, this combination therapy seems safe.

Ecballium elaterium is the only species belonging to the genus Ecballium of Cucurbitaceae family. It is native to the Mediterranean region. Despite its side effects, E. elaterium has still been being used as an alternative treatment agent for sinusitis, cirrhosis, rheumatic diseases and hemorrhoids for its anti-inflammatory and cathartic actions. Herein we discuss a pediatric case showing gastrointestinal side effects after ingesting E. elaterium.
Case Report: A six-year-old boy admitted to Çukurova University Medical Faculty Pediatric Emergency Department, Turkey, for vomiting one hour after ingesting a green plant which he had tasted to satisfy his curiosity. Physical examination, vital signs and laboratory tests revealed normal. The ingested plant was defined to be E. elaterium. Maintenance fluid infusion, 1 mg/kg ranitidine and sucralfate medications were commenced. During the follow-up, the patient developed numbness of the tongue and hyper-salivation, without any signs of uvular edema or other system findings. Further follow-up showed stable vital signs within the normal range with no additional complications. The patient was sent home with the cure and recommendations.
The plants and herbs that are used as agents of alternative or complementary medicine may as well be accidentally or curiously taken by children leading to unwanted intoxication cases. Pre-encounter actions to prevent such cases are as important as any post-exposure clinical interventions to impede the unwanted consequences. One such measure might be a more intensive public information policy underscoring the fact that plants have the potential to be noxious and may cause serious side effects and even death.

The present study describes the morphometry of a unilateral ossified sacrotuberous ligament. It aims to discuss its Anatomical and clinical implications.The pudendal nerve, internal pudendal artery, nerve to obturator internus and coccygeal branch of inferior gluteal artery, are the important structures related to sacrotuberous ligament. An ossified sacrotuberous ligament may be an important etiological factor in neurovascular compression syndromes and its anatomical knowledge may help in the development of new treatment strategy for this common clinical problem. The ossified sacrotuberous ligament in the present case exhibits, a characteristic anterior and posterior segment, a base at the ischial tuberosity and an apex attached to alae of sacrum. The ossified sacrotuberous ligament may be important in differential diagnosis of soft tissue pain and tenderness after trauma. It may be a challenging puzzle for the present day surgeon and radiologist in interpretation of radiological problems.

Exposure to cigarette smoke has numerous health risks for infants and children. Home smoking ban is a simple way to reduce the detrimental impacts of cigarette smoke. This study aimed to determine the prevalence of cigarette smoking bans in homes as well as associated factors in families with infants in Tehran, Iran.
This study was a population-based cross-sectional survey on 1,112 families with infants, selected through stratified-cluster sampling. In this study, a researcher made questionnaire including four parts: sociodemographic characteristics, smoking status, smoking restrictions, and parental awareness and belief, was completed. The multivariable logistic regression was used to investigate the relationship between smoking bans in homes and associated factors.
Complete smoking ban in homes was 37.3%. In the multivariable logistic regression, cigarette smoking ban in homes was significantly higher for employed mothers (odds ratio [OR] = 3.03, 95% confidence interval [CI]: 1.2–7.2, P This study indicates that complete smoking ban is not established in many households with infants in Iran. Furthermore, it is necessary to perform health interventions for reducing infant exposure to cigarette smoke, while considering the factors associated with smoking ban.

Hypotrichosis with juvenile macular dystrophy (HJMD) is a rare genetic disorder caused from mutations in the Cadherin 3 (CDH3) gene.
In the present study, we reported an Iranian family with three affected members born to a consanguineous parent. Mutational analysis using whole exome sequencing has revealed a nucleotide change in CDH3 gene (NM_001793:exon8:c.830delG) which leads to a frame-shift mutation (p.G277Afs*20). No intra-familial phenotypic variation was found.
Identification of disease-causing mutation in this family facilitated the effective genetic counseling and prenatal diagnosis.

Eczema or atopic dermatitis (AD) is one of the most prevalent skin diseases in the world. Although, the disorder is not fatal, it can cause life quality reduction. The aim of the current study was to investigate the impact of atopic dermatitis on life quality of 1-6-year-old children.
The current study is a descriptive and analytical one designed to assess quality of life (QOL) in 1-6-year-old children with atopic dermatitis in Kurdistan province (West of Iran). All the children who attended skin clinic of Besat Hospital, Sanandaj- Iran, during 2014 and 2016, participated in the study. Quality of life questionnaires were used to obtain data. Parents of the participating children were asked to complete the questionnaire. Index of Scoring Atopic Dermatitis (SCORAD) was used to determine the severity of the disease. The study data were analysis using Stata-12 software.
During the study, 53 children with atopic dermatitis were identified and 66.04% were male. According to the classification of SCORAD index, 54.36% of the children (19 subjects) were included in the moderate group (SCORAD 14-40) and 63.46% (33 persons) in the severe group (SCORAD> 40). Mean of life quality score was 9.24 ± 10.48 (range 0-30) and there was no statistically significant difference between the genders (P >0.05).
There was a positive correlation between the quality of life and pain severity in AD children; and children with atopic dermatitis had low quality of life and itching, wound, discomfort and sleep disorder, were the factors that mainly impact on their life quality.