Iranian Journal of Pediatrics
Volume:22 Issue: 1, 2012

Acute seizure attack is a stressful experience both for health care personnel and parents. These attacks might cause morbidity and mortality among patients, so reliable methods to control the seizure preferably at home should be developed. This study was performed to measure the time needed to control seizure attacks using intranasal midazolam compared to the common treatment (intravenous diazepam) and to evaluate its probable side effects. This study was conducted as a not blind randomized clinical trial among 60 patients coming to Imam Ali Hospital, Zahedan, Iran. The patients were 2 months to 15 years old children coming to our emergency department suffering from an acute seizure episode. Intranasal midazolam was administered 0.2 mg/kg equally dropped in both nostrils for case group and intravenous diazepam was administered 0.3mg/kg via IV line for control group. After both treatments the time needed to control the seizure was registered by the practitioner. Pulse rate and O2 saturation were recorded at patients’ entrance and in minutes 5 and 10 after drug administration. The time needed to control seizure using intranasal midazolam (3.16±1.24) was statistically shorter than intravenous diazepam (6.42±2.59) if the time needed to establish IV line in patients treated by intravenous diazepam is taken into account (P<0.001). The readings for O2 saturation or heart rate did not indicate a statistically significant difference between two groups of patients either at entrance or 5 and 10 minutes after drug administration. Considering the shorter time needed to control acute seizure episodes compared to intravenous diazepam and its safety record, intranasal midazolam seems to be a good candidate to replace diazepam, as the drug of choice, in controlling this condition.

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.

Obesity and overweight have shown an increasing trend in most developing countries. Childhood obesity would impose numerous health-related problems. This study was conducted to determine the correlation of obesity and overweight with emotional-behavioral problems in primary school age girls. In a cross-sectional study, 300 primary school girls (aged 7-11 years) were selected using a multi-staged sampling method, including randomized cluster and stratified method. For all students body mass index was measured and then based on BMI for age and sex, from each grade (1-5), 20 students were selected for each group of normal weight, overweight and obese. The emotional-behavioral problems were evaluated using child behavior checklist that consists of 113 items for childhood behavioral problems. Total behavioral problems were seen in 17%, 27%, and 2% in obese, overweight, and normal weight children, respectively. Internalizing problems (including Anxious/Depressed, Withdrawn, and Somatic Complaints) were seen in 11%, 15%, and 2% and externalizing problems (including Aggressive and Delinquent Behaviors) were observed in 8%, 17%, and 2% in obese, overweight, and normal weight children, respectively. The mean scores in all scales were higher in obese and overweight children in comparison with normal weight children and the emotional-behavioral problems had significant positive correlation with obesity and overweight (P<0.01). Despite the cultural differences between east and west, yet there are similarities in the most of the emotional-behavioral problems related to overweight and obesity. Also, it seems that the risk of behavioral-emotional problems in overweight girls is more than in obese girls. Overweight and Obesity prevention may be a primary preventive step for these problems in children.

Familial Mediterranan Fever is an hereditary autoinflammatory disease that presents with recurrent febrile attacks and poly serositis. Colchicine is the only known treatment in this diease. However, nearly 5-10% of patients are resistant to colchicines. There are many different modalities in colchicine resistant patients, biologic and immunosupressive drugs being the known ones. We studied the efficacy of Dapsone as an anti inflammatory drug in children with FMF who did not tolerate colchicine well. This is a case series study in 10 patients who had FMF on the base of Tel-Hashomer criteria and did not tolerate colchicine or did not respond to it well. Patients took 2mg/kg dapsone in single dose, during 6 months. In four patients episodic attacks returned after 27 days, so the drug was discontinued. One patient refused to continue the study; in five patients dapsone was taken in average for 8 months and 6 days, at least for 6 months. These five patients had no episodes of attack during the following observation. Dapsone could control episodic attacks of FMF in 50% of cases. It might be considered as an alternative therapy in FMF cases not responding to colchicine.

The term ‘child development’ is used to describe the skills acquired by children between birth and the age of 6 years. Development is rather an integrated process and impairment in one developmental milestone can affect other areas too. The goal of the present study was preparation and determination of validity and reliability of the "Newsha Developmental Scale" as an integrated scale for assessing developmental skills of Persian speaking children up to the age of six years. The Newsha developmental scale was created to thoroughly evaluate seven developmental domains in 13 age groups from birth to 72 months. It was based on the concerns of parents and nursery teachers about child development. In order to obtain the scores of normal population, 593 children in nurseries and health centers of Tehran were assessed. The children had no medical concern or confirmed disorders. With consideration of the age of each child, the score of minimum or higher in each developmental milestone was considered as the normal range. The test-retest reliability and inter-rater reliability were measured. In addition, the content validity was assessed by ideas taken from 10 specialists in the field of child development and the content validity Index (CVI) was calculated. Also construct validity was considered by establishing the effect of age on test results. A correlation of more than 95% was shown in both test-retest and inter-rater reliabilities (P<0.001). The CVI of various skills of the age groups was between 0.8 and 1, and construct validity revealed the effect of age on the test results. The gender difference was shown only in 6 items among 646 items of the scale (P<0.02). The "Newsha Developmental Scale" is an integrated and comprehensive scale for evaluating the developmental process of children and identification of any probable delay in developmental abilities of children with Persian language up to 6 years of age.

Overweight among adolescents is not only an important public health problem but also a problem affecting economic growth in developing countries. The aim of this study was to investigate the parental determinants of overweight and obesity in Iranian adolescents at national level. This cross sectional study was conducted within the framework of the Comprehensive Study on Household Food Consumption Patterns and Nutritional Status of IR Iran during 2001-2003. In adolescents, anthropometric indices were defined based on the CDC 2000 cut-off points for age and gender-specific body mass index (BMI). Parental characteristics were collected by questionnaire. Among the 7158 participating households, data on 7908 adolescents aged 11-19 years (3750 girls and 4158 boys) was analyzed. The prevalence of overweight (85–94th percentile) in boys and girls was 6.2%, and 8.7%, respectively. The prevalence of obesity (≥95th percentile) among boys and girls was similar (3.3%). Parents’ weight status, father’s job and parents’ education showed significant association with weight status in adolescents. Logistic regression analysis showed that parental overweight and obesity, parental education and father’s job were the main parental determinants of overweight and obesity in Iranian adolescents. Parental overweight and obesity, parental education and father’s job seem to be the major parental determinants of overweight in Iranian adolescents. Future prevention programs must take these risk factors into account.

To investigate the molecular mechanism underlying T-bet mediated anti-neoplastic effects of cytokine induced killer (CIK) cells. Lymphocytes isolated from peripheral blood of leukemic children were induced with γ- interferon (IFN-γ), CD3McAb and interluki-2 (IL-2), and co-cultured with dendritic cells (DCs) to generate DC-CIK cells. The morphology and immunophenotype of these cells were determined by a light microscopy and flow cytometry, respectively. IL-2 and IFN-γ levels released by DC-CIK cells were quantified by ELISA. Cytotoxicity of DC-CIK cells against leukemia cell lines was measured by MTT assay. FCM was used to detect CD4+CD25+Treg cells, while RT-PCR and Western blot were used to determine mRNA and protein expressions of Foxp3 and GATA3 in DC-CIK cells treated with T-bet monoclonal antibody. Induced DC-CIK cells were regular, round and transparent with variable cell volume and cellular aggregation. The main effector cells in this population were CD3+CD8+ cells and CD3+CD56+ cells. We demonstrated a time dependent increase in IL-2 and IFN-γ levels after induction. DC-CIK cells were cytotoxic to B95 cells, Jhhan cells and M07e cells, with the highest cytotoxicity towards B95 cells. Treatment with mouse anti-human T-bet monoclonal antibody resulted in an increase in the proportion of CD4+CD25+Treg cells and elevation of Foxp3 and GATA3 mRNA and protein levels. DC-CIK cells induced with cytokines were strongly cytotoxic towards a number of cancer cell lines. Foxp3 and GATA3 were implicated in the T-bet mediated anti-neoplastic effects of DC-CIK cells via activation of the Th1 pathway and suppression of the Th2 and Treg pathways.

Wilson's disease (WD) is an autosomal recessive disease with genetic abnormality on chromosome 13 causing defect in copper metabolism and increased copper concentration in liver, central nervous system and other organs, which causes different clinical manifestations. The aim of this study was to determine the sensitivity of different clinical and paraclinical tests for diagnosis of Wilson’s disease. Paraffin blocks of liver biopsy from 41 children suspicious of WD were collected. Hepatic copper concentrations were examined with atomic absorption spectrophotometry (Australian GBC, model: PAL 3000). Fifteen specimens had hepatic copper concentration (dry weight) more than 250μg/g. Clinical and laboratory data and histologic slides of liver biopsies of these 15 children were reviewed retrospectively. Liver tissue was examined for staging and grading of hepatic involvement and also stained with rubeonic acid method for copper. Patients were 5-15 years old (mean age=9.3 years, standard deviation=2.6) with slight male predominance (9/15=60%). Five (33%) patients were 10 years old. Three (20%) of them were referred for icterus, 8 (54%) because of positive family history, 2 (13%) due to abdominal pain and 2 (13%) because of hepatosplenomegaly and ascites. Serum AST and ALT levels were elevated at the time of presentation in all patients. In liver biopsy, histological grade and stage was 0-8 and 0-6 respectively, 2 (13%) had cirrhosis, 1 (7%) had normal biopsy and 12 (80%) showed chronic hepatitis. Hepatic copper concentrations were between 250 and 1595 μg/g dry weight. The sensitivity of various tests were 85% for serum copper, 83% for serum ceruloplasmin, 53% for urinary copper excretion, 44% for presence of KF ring and 40% for rubeonic acid staining on liver biopsies. None of the tests stated in the article were highly sensitive for diagnosis of WD, so we suggest that diagnosis should be based on combination of family history, physical examination and different tests.

Urinary tract infection (UTI) is one of the most common causes of febrile pediatric diseases. Also, vesicoureteral reflux (VUR) is a significant risk factor for UTI. Voiding cystourethrography (VCUG) is the method of choice for evaluation of VUR. This study was done to predict VUR by DMSA scan (technetium 99 m-labeled dimercaptosuccinic acid) and ultrasonography (US). In a prospective study, all children with first time acute pyelonephritis were selected and evaluated by DMSA scan and US. Then VCUG was done with negative urine culture. All children with final diagnosis of obstructive congenital anomaly were excluded. The sensitivity, specifity, positive predictive values, negative predictive values, Confidence Interval of DMSA scan and US were calculated for prediction or exclusion of VUR. Among 100 children with UTI diagnosis, VUR was detected in 39 children and 63 (31.5%) kidneys. DMSA scan was abnormal in 103 (51.5%) units, 45 units had VUR (PPV=44%), 79 units with normal DMSA scan had no VUR (NPV=81%). Of kidney units that were abnormal by DMSA or US, 51 units had VUR. PPV and NPV were 44% and 56%, respectively. DMSA scan alone or with US cannot predict VUR (especially low grade VUR). But according to NPV, it seems that absence of VUR can be predicted. So, more studies are needed to determine the usefulness of DMSA scan and US instead of VCUG for detection of VUR.

This study was conducted to evaluate the prevalence of rotavirus disease and to investigate the genotypes of rotavirus strains causing acute gastroenteritis among children aged <5 years old in Marvdasht, Iran. One hundred and forty-one children, aged 1 month to 5 years, afflicted with severe diarrhea were enrolled during January 2007 to December 2008. Their stool samples were studied with enzyme immunoassays (EIA) for group A rotaviruses. Rotavirus-positive specimens were genotyped by the Nested RT-PCR using different types of specific primers. Out of total collected samples rotavirus infection was detected in 40 (28.37%). Of the rotavirus episodes, 72.91% occurred during the first 2 years of life (P=0.038). The highest prevalence of infection was identified in summer (52.50%) and the lowest in winter (7.50%). The most common clinical features included diarrhea (96.25%), vomiting (82.50%) and fever (45.0%). Mixed genotypes were the predominant G type (60.0%), followed by non-typeable (12.50%), G2 (12.50%), G4 (10.0%) and G1 (5.0%) genotypes. G3/G8 mixed infection is the first of these rotavirus genotypes to be reported in Iran. Regarding high frequency of rotavirus infection, continuous surveillance is needed to inform diarrhea prevention programs as well as to provide information about the occurrence of new rotavirus strains. This will assist policy makers in decision making on rotavirus vaccine introduction.

Fetal malnutrition (FM) implies soft tissue wasting at birth with significant postnatal consequences and morbidity, and is identified by clinical assessment (CAN score) and anthropometry. No previous studies have been done to study all these parameters and evolve a screening method. The aim of this study was identifying the incidence of FM using CAN score and compare the nutritional assessment with anthropometry and evolve a screening tool for rapid assessment of FM. Prospective study in Government district maternity hospital. 300 term newborns were assessed by CAN score and anthropometry recorded. The newborns were classified as per weight for age. Ponderal index (PI), Body mass index (BMI) and midarm circumference/head circumference ratio (MAC/HC) calculated and compared to CAN Score for accuracy in identifying FM. Incidence of FM was 24%. Newborns identified malnourished by PI, BMI, MAC/HC were evaluated by CAN score and significant number of them (31/78 in PI, 60/121 in BMI, 51/81 in MAC/HC) were found well nourished. Similarly those recognized as normal by PI, BMI, MAC/HC were malnourished by CAN score(25/222 in PI, 11/179 in BMI, 42/219 in MAC/HC) with statistical significance(0.0001). BMI had the highest sensitivity and 11 neonates with normal BMI had low CAN score ann 9 of them had normal PI also making a combination of BMI and PI a good indicator of normal nutrition. FM is best identified by CAN Score. BMI is the best screening tool for malnutrition and when coupled with PI will identify most normally nourished newborns.

The aim of this study was to evaluate the prevalence and risk factors of anemia among adolescents in Denizli where Mediterranean cuisine (fresh fruit and vegetables) is adopted. We accepted hemoglobin values below 12 g/dl for girls and 13 g/dl for boys as the criteria of anemia. We recorded a detailed history including nutritional habits, consumption of animal source foods especially red meat, consumption of fresh fruit and vegetables, presence of parasitic infestation, psychosocial status, school success and any materials consumed except ordinary food. We investigated the prevalence of anemia in 1120 children (672 girls and 448 boys), aged 12 to 16 years. We found that the overall prevalence of anemia was 5.6%. 8.3% of the girls and 1.6% of the boys were anemic. We diagnosed iron deficiency anemia in 37(59%) anemic patients and combined iron deficiency and vitamin B12 deficiency anemia in 26 (41%) anemic patients. None of the patients had folic acid deficiency. Our results suggest that the socioeconomic status of the family, traditional eating habits of the region, the fear of gaining weight and irregular eating habits are of great importance in the development of adolescent anemia in Denizli.

Few reports are available on some benefits, such as shortened duration of diarrhea and better weight gain, for lactose-free over lactose-containing formula in acute childhood diarrhea. We evaluated the effects of lactose-free formula in dietary management of acute diarrhea in formula-fed children. This controlled-clinical trial was conducted on formula-fed children, aged 1 to 24 months, referring with acute non-bloody diarrhea (≤2 weeks). Those who had major systemic illness, severe malnutrition, severe dehydration, severe vomiting, or history of antibiotic therapy were not included. Children were allocated to receive lactose-free formula (intervention, n=37) or lactose-containing formula (control, n=34). Time to diarrhea relief and weight change were compared between the two groups after one week. During the study, 32 male and 39 female children (7.1±3.7 months) were included. Those who received lactose-free formula had a significantly shorter time to diarrhea relief compared with the controls (1.7±0.7 vs. 2.6±0.7 days, P<0.001). Weight significantly increased in both groups, but there was no difference between the two groups in weight change (37±100 vs. 38±77 gr, P=0.673). Multivariate analysis showed that receiving lactose-free formula significantly predicted time to diarrhea relief (95% CI: 1.5 to 3.9, P<0.001) controlling for baseline characteristics. Early administration of lactose-free formula for formula-fed children presenting with acute diarrhea can result in a more rapid relief of acute diarrhea and thus perhaps less mortality and morbidity. Trials with longer follow-ups are warranted to better evaluate long-term results such as weight change and feeding problems in this regard.

Neonatal malaria is a serious cause of morbidity and mortality in sub-Saharan Africa. Diagnosis of neonatal malaria is difficult because of the similarity in clinical presentation with other neonatal infections. This study aim to highlight the clinical presentations and high mortality still associated with neonatal malaria. Twenty four out of 41 neonates seen during a 6 months period were studied. Gestational age, age at presentation, birth weight and other clinical symptoms were documented. Questionnaires were used to collect pertinent pregnancy and perinatal history from the mothers. Data was analyzed using SPSS v18 and results expressed in tables using means, frequencies and percentages. All 24 neonates, 50% of whom were males, had a positive smear for malaria parasite. 29.2% were preterm, 17(70.8%) had congenital malaria, 18(75.0%) mothers used intermittent preventive treatment (IPT) of malaria prophylaxis in the index pregnancy and 1(4.2%) mother had HIV in pregnancy. Fever was the principal presenting symptom and 83.0% responded to treatment with amodiaquine. Neonatal malaria is still an important cause of mortality, a more effective malaria prophylaxis program and routine malaria parasite screening for neonates is recommended.

Thyroid hormone is necessary for normal development of the auditory system. The aim of this study was to investigate the rate of hearing impairment in congenitally hypothyroid (CH) patients, and its relation with factors such as CH severity and age at starting treatment, during CH screening program in Isfahan. Hearing acuity was assessed in two groups of children with (94 patients aged 4 months – 3 years) and without CH (450), between 2000-2006. Otoacostic emission (OAE) was performed by a two step method. After two tests without OAE signals bilaterally, they were referred for auditory brainstem response (ABR) test. Subjects with both OAE and ABR abnormal test results were considered to have hearing problem. Obtained data was compared in case and control group and also CH patients with and without hearing impairment. Three (3.2%) of patients and 1 of control group (0.2%) were diagnosed with sensorineural hearing loss. The rate of hearing loss was not different significantly in two studied groups (P>0.05). There was no difference between age of starting treatment and first T4 and TSH level in CH patients with and without hearing loss (P>0.05). CH neonates with hearing impairment had thyroid dyshormonogenesis according to the follow up results. The rate of hearing loss was low among our studied CH patients. It may be due to proper management of CH patients. In view of the fact that all CH neonates were dyshormonogentic and considering the relation between certain gene mutations and hearing impairment in CH patients, further studies with larger sample size, with regard to different etiologies of CH should be investigated to indicate the possible gene mutations related to hearing loss in CH.

To develop a simple clinical scoring system for severity of illness to help prioritize care and predict outcome in emergency department. Prospective hospital based observational study. Out of a total of 874 children who attended emergency department in one year, 777 were included in the study. Data was collected at the time of admission in emergency department. The baseline information like age, gender, etc and variables of ‘toprs’ score viz temperature, oxygen saturation, pulse rate, respiratory rate, sensorium and seizures were recorded. Variables were categorized as normal (score zero) or abnormal (score 1) based on systemic inflammatory response syndrome (SIRS) criteria and criteria mentioned in advanced pediatric life support (APLS) and the total scores were computed for each child. The outcome (death/discharge) was correlated with the study variables and total score. The predictive ability of score was calculated using receiver operating characteristic (ROC) curve analysis. Of the six variables, temperature, oxygen saturation and respiratory rate were found to be significantly associated with mortality. Mortality increased with the increase in the number of abnormal variables. Based on the regression coefficients, maximum possible score was 6.68. The predictive ability of score was 81.7 calculated using ROC curve. Maximum discrimination was observed at a score of 2.5. For triage in emergency, any patient with 2 or more abnormal variables should be closely monitored and evaluated. These patients require admission as they have a potential risk of death.

Sweat chloride measurement is considered a standard diagnostic tool for cystic fibrosis (CF). This study was performed to compare sweat chloride values obtained by quantitative pilocarpine iontophoresis (classic test) with sweat crystallization detected by direct observation of a drop of perspiration under light microscopy in patients with and without CF. The tests using both techniques were performed simultaneously in patients with and without CF. Cutoff values of ≥60 mmol/L of chloride concentration for the classic sweat test was considered for diagnosis of CF. In crystallization method, observation of typical dendritic forms of salt crystals under light microscopy was interpreted positive. Sixty patients suspected to CF (31 males and 29 females) with age range of 9 months to 2 years underwent the sweat test using both techniques. Median sweat chloride values was 26.13+10.85 in group with negative and 72.76+12.78 mmol/L in group with positive sweat test, respectively. All the patients who had positive sweat test in classic method showed typical dendritic forms of salt crystal in sweat crystallization test, which provided the test with 100% sensitivity (95%CI: 93.1-100). Only one of the 31 subjects with negative results for classic sweat test had positive result for crystallization sweat test, which provided the test with 96.7% specificity (95%CI: 92.9-100). Time spent to perform the crystallization test was significantly shorter than the classic method whereas its cost was also lower than the second method. There was a good correspondence between two studied methods of sweat test. These results suggested the sweat crystallization test as an alternative test for detecting CF disease with high sensitivity and specificity.

In Iran thyroid-stimulating hormone (TSH) based neonatal screening program is included in health care services from 2005 for detection of patients with primary congenital hypothyroidism (CH). This study was performed for a critical evaluation of the screening program primary congenital hypothyroidism in Fars province, Iran. From November 2006 to September 2007, TSH serum concentrations of 63031 newborns, 3 to 5 days old born in Fars province, were measured by heel prick. The newborns with TSH 5mIU/L were recalled for measurement of serumT4 and TSH in venous blood samples Of 127 recalled subjects, 43 were confirmed to be hypothyroid, showing a prevalence of 1:1465 with F:M ratio of 1.05:1. The most common clinical and radiological findings were prolonged jaundice (73%), large anterior fontanel (56%), wide posterior fontanel (55%), absence of distal femoral epiphysis (20%), and umbilical hernia (11%). Scintigraphy of the thyroid with 99mTC revealed eutopia (67.4%), hypoplasia (23.3%), agenesis (4.7%) and ectopia (2.3%). It is concluded that a cut off value of TSH≥5mIU/L overestimates recalling the number of patients with CH. The most common cause of congenital hypothyroidism is not dysgenesis of the gland and perhaps dyshormonogenesis in Iran is more common than what is reported in other countries.

To investigate the correlation between hypoglycemia and positive rate of inborn error of metabolism (IEM) in neonatal intensive care unit. 160 patients from a neonatal intensive care unit were enrolled. Blood glucose was measured by Roche Modular chemistry. The dry blood on filter papers, collected from 160 patients, was tested by tandem mass spectrometry to detect 35 inborn errors of metabolism. Clinical follow-up of all the patients was at least in an interval of 12 months. The mean observation period was 13.5 months per child. Based on the ROC curve, the optimal cut-off value of hypoglycemia as an indicator for screening for IEMs was projected to be 2.8 mmol/L, which yielded a sensitivity of 71.4% and a specificity of 76.5%. The patients were divided into two groups: hypoglycemia group (48 cases) and the control group (112 cases). 5(10.4%) of the 48 patients in the hypoglycemia group were positive, while only 2(1.8%) of the 112 patients in the control group were positive. The difference of the positive rate in the screening for IEMs between the two groups was significant (c2=4.10, P<0.05); the relative risk (RR) was 5.83 (95% CI: 1.06-32.12). The risk of patients with hypoglycemia suffering from IEMs was significantly higher than that of the non-hypoglycemia patients in NICU, based on cut-off value of 2.8mmol/L.

Acute lymphoblastic leukemia (ALL) is the most common malignancy diagnosed in children. Extramedullary leukemia including hepatosplenomegaly, lymphadenopathy, and mediastinal mass are the clinical presentations of T-cell ALL. Pericardial effusion and atrial mass are rare presentations of pediatric leukemia. We report a 15-year-old girl with T-cell ALL, who presented initially with pericardial effusion and cardiac tamponade and subsequently developed right atrial mass. The mediastinal and right atrial masses were excised by surgical procedure as a treatment strategy for prevention of arrhythmia and sudden death. T-cell ALL can present with pericardial and mediastinal involvement as an initial manifestation. Pericardiocenthesis and systemic anticancer chemotherapy without local chemotherapy should be considered in these cases.

Achondroplasia is a relatively frequent genetic disorder that may lead to limb weakness, motor-mental retardation, hydrocephaly, and respiratory disorders. In this pathology, foramen magnum stenosis and accompanying disorders like respiratory depression is well documented. A 2.5 year-old child with the diagnosis of achondroplasia admitted to our clinic with severe respiratory depression, limb weakness, and motor mental retardation as well as sympathetic dysfunction. In radiologic evaluations, severe foramen magnum stenosis was detected. The patient was operated and posterior fossa decompression was accomplished to prevent compression of respiratory centers and neurons. This case is unique with the narrowest foramen magnum reported up to date and the sympathetic dysfunction which is not reported as a complication in achondroplasic patients. The authors review the relevant literature, focusing on the indications for cervicomedullary decompression in infants with achondroplasia.

Type 1 diabetes mellitus (T1DM) is the most common form of diabetes in young children. Serious optic complications, e.g. diabetic retinopathy and diabetic cataract involvement, are not usually detected in T1DM patients at the onset of the disease. Two girls aged 11 years and 9 years were hospitalized in our unit in 2008 and 2009. They presented cataracts 1 and 6 months before the diagnosis of T1DM, respectively. After blood glucose level was controlled by insulin therapy, the cataract was resolved, totally in one and partly in the other girl. Meanwhile, visual acuity of both cases recovered, closely associated with fluctuation of plasma glucose level. In this study, we describe the symptoms, probable mechanism and treatment of diabetic cataract. Early antihyperglycemic therapy and maintenance of stable blood glucose level may reverse acute diabetic cataract or prevent it from getting worse.

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.

Penile duplication (diphallus) is an extremely rare disorder. It is almost always associated with other malformations like double bladder, exstrophy of the cloacae, imperforate anus, duplication of the rectosigmoid and vertebral deformities. Meanwhile anal canal duplication, the most distal and least common duplication of the digestive tube and is a very rare congenital malformation. A 21 days old Egyptian neonate is reported with complete penile duplication and two scrotums with each one carrying two palpable testes. Both penises have normal shaft with normally located meatus. Clear urine voids from both meati spontaneously. The child had also a fold of redundant skin about 4×5 cm at the anal region in which two separate anal openings are present. In rectal examination we found two normal anuses passing stool spontaneously. Ascending (voiding) cystourethrography revealed two penises with two separate meatuses and one bladder from which the two urethras go out separately. Intravenous pyelogram (IVP) revealed two normal kidneys and ureters. Barium study revealed duplication of rectum and colon, otherwise normal GIT. In our review of the literature, we did not come across any other case of this variety of the penile duplication and congenital presence of two anuses. Unfortunately the patient expired before any surgical correction.

Klippel-Trenaunay-Weber Syndrome (KTWS) is a rare neurocutaneous syndrome. Hemimegalencephaly (HME) and seizure episodes have been reported previously in a few cases with KTWS. We report here a 3 day-old girl with partial motor seizures, extensive port-wine staining and mild structural deformities in the feet, and a hemimegalencephaly. Occurrence of partial motor seizures in addition to bilateral lower extremities extensive port-wine staining is a unique feature seen in our case.