Refugee and asylum-seeker children and health problems in the city of Duzce, Türkiye.

INTRODUCTION: This study examines the health problems and healthcare needs of refugee and asylum-seeker children and aims to develop strategies for improvement. METHODS: Based on quantitative data from 448 refugee and asylum-seeker children and 222 non-refugee local children, this study was conducted at Düzce University, Department of Paediatrics, between 2010 and 2021. The refugee children originated from three countries: Iraq (n = 304), Syria (n = 101) and Afghanistan (n = 43). The data were analysed using the SPSS data analysis program. Ethical clearance was obtained from the Ethics Committee of Düzce Üniversity. RESULTS: The results suggest that refugee and asylum-seeker children have significantly higher rates of acute illness or infection, malnutrition (p < 0.001) and anaemia (p < 0.001) than local children as a result of living in overcrowded families (p = 0.017) and unhealthy conditions. Adolescent pregnancy (p = 0.049) emerges as an important social problem as a result of child marriage among refugee children, mostly in the form of consanguineous marriages (p < 0.001). The rate of having at least two adolescent pregnancies (under 18) was highest among Syrian refugee girls (p = 0.01). Although refugee and asylum-seeker children have higher rates of health insurance (between 74% and 95%), they have lower rates of insurance compared to local children. This research also compares the data from three nationalities, including Syria, Afghanistan and Iraq children; Iraqi and Afghan children under the international protection (IP) system with limited social support and rights had worse health conditions compared to other groups. Although Iraqi children had the highest rates of health insurance on admission (p < 0.001), they also had higher rates of chronic diseases (p = 0.001), infections (p = 0.004), allergic rhinitis (p = 0.001) and malnutrition (p < 0.001). The youngest age of admission (p = 0.006) and the shortest length of stay (p = 0.004) were for Afghan children who also had higher rates of upper respiratory infections (p = 0.021). CONCLUSIONS: This study highlights the urgent need for improved screening programmes and the importance of collaborative efforts to address the specific health needs of these populations. Addressing the health status of child refugees is a complex and multifaceted task that requires the active participation of healthcare professionals, policymakers and researchers, each of whom has a crucial role to play.

Renal involvement, presence of amyloidosis, and genotype-phenotype relationship in pediatric patients with Familial Mediterranean fever: a single center study.

The aim of the study is to investigate how renal involvement is correlated with frequency of amyloidosis, risk factors, and demographic and clinical characteristics in pediatric patients with Familial Mediterranean fever (FMF). Demographic and clinical characteristics and laboratory data of the pediatric patients diagnosed with FMF between 1990 and 2018 were recorded from their files. The diagnosis of patients with amyloidosis (AA) was proven by renal biopsy, and as for patients with non-amyloidosis renal involvement (RI wo AA), amyloidosis could not be detected but they were followed up with the diagnosis of proteinuria and/or hematuria. A total of 1929 FMF pediatric patients were included in the study. About 962 (49.9%) participants were male. There were 134 (6.9%) patients with RI wo AA and 23 (1.2%) patients with AA diagnosed by biopsy. The most common M694V heterozygous/homozygous(het/hom) (31%) mutation was observed. Delay in diagnosis and presence of colchicine resistance were more in patients with RI wo AA and AA (p < 0.05). M694V het/hom mutation was high in both RI wo AA and AA, while the presence of compound heterozygous with M694V mutation was high in RI wo AA (p < 0.01, p = 0.02, p = 0.048, respectively). There was a positive correlation between M694V mutation and monoarthritis/polyarthritis, between compound heterozygous with M694V mutations and presence of chest pain, and between V726A mutation and constipation. Also a negative correlation was found between E148Q and chest pain and between R202Q mutation and monoarthritis/polyarthritis. While M694V mutation increased the risk 2.6 times for AA and 1.7 times for RI wo AA, colchicine resistance increased the risk 33 times for AA and 25 times for RI wo AA.    Concluson: It was concluded in the present study that M694V mutation and colchicine resistance were two important risk factors for RI wo AA (6.9%) and amyloidosis (1.2%) in FMF patients. It should be kept in mind that compound heterozygous with M694V mutations may be associated with chest pain and R202Q mutation may be negatively correlated with arthritis, unlike M694V. The genetic results and clinical findings of the patients should be evaluated together and followed up closely. What is Known: • M694V mutation and colchicine resistance were two important risk factors for RI wo AA and amyloidosis in FMF patients. What is New: • Compound heterozygous with M694V mutations were associated with chest pain and may be more serious than thought. • Another point is that while R202Q mutations were negatively correlated with arthritis, M694V mutations were positively correlated.

Reporting the clinical spectrum of children with IgAV in a retrospective 24-year cohort: Influences of age and sex on clinical presentation.

Background/aim: Immunoglobulin A vasculitis (IgAV) is one of the most common types of vasculitis in children. The aims of this study were to investigate the clinical characteristics of the disease, and the effects of age and sex on the clinical course in children with IgAV. Materials and methods: This was a retrospective study including pediatric patients diagnosed with IgAV who attended follow-ups at the pediatric rheumatology department of a tertiary healthcare institution between January 1997 and December 2020. The patients were grouped and compared according to sex and age at diagnosis (<7 years vs. ≥7 years). Results: The study included 709 children with IgAV, 392 (55.3%) of whom were male. The mean age at diagnosis was 7.9 ± 3.2 years. The most common disease onset season was autumn (31.2%). Upper respiratory infections (27.8%) were the most common predisposing factors. Gastrointestinal system (GIS), joint, and renal involvement were observed in 52.8%, 47.5%, and 17.5% of patients, respectively. Renal involvement, GIS involvement, and disease relapse were significantly more common among those diagnosed after 7 years of age compared to those diagnosed before the age of 7 (p < 0.001, p = 0.033, and p < 0.001, respectively). Scrotal involvement and subcutaneous edema were more common among those diagnosed at younger than 7 years compared to those aged ≥7 years at diagnosis (p < 0.001 and p = 0.016, respectively). GIS involvement was more frequently seen in males compared to females (p = 0.046). Conclusion: It was demonstrated that being ≥7 years of age at diagnosis or being a male were associated with higher likelihood of renal and GIS involvement in children with IgAV.

Two randomized trials of canakinumab in systemic juvenile idiopathic arthritis.

BACKGROUND: Interleukin-1 is pivotal in the pathogenesis of systemic juvenile idiopathic arthritis (JIA). We assessed the efficacy and safety of canakinumab, a selective, fully human, anti-interleukin-1ß monoclonal antibody, in two trials. METHODS: In trial 1, we randomly assigned patients, 2 to 19 years of age, with systemic JIA and active systemic features (fever; ≥2 active joints; C-reactive protein, >30 mg per liter; and glucocorticoid dose, ≤1.0 mg per kilogram of body weight per day), in a double-blind fashion, to a single subcutaneous dose of canakinumab (4 mg per kilogram) or placebo. The primary outcome, termed adapted JIA ACR 30 response, was defined as improvement of 30% or more in at least three of the six core criteria for JIA, worsening of more than 30% in no more than one of the criteria, and resolution of fever. In trial 2, after 32 weeks of open-label treatment with canakinumab, patients who had a response and underwent glucocorticoid tapering were randomly assigned to continued treatment with canakinumab or to placebo. The primary outcome was time to flare of systemic JIA. RESULTS: At day 15 in trial 1, more patients in the canakinumab group had an adapted JIA ACR 30 response (36 of 43 [84%], vs. 4 of 41 [10%] in the placebo group; P<0.001). In trial 2, among the 100 patients (of 177 in the open-label phase) who underwent randomization in the withdrawal phase, the risk of flare was lower among patients who continued to receive canakinumab than among those who were switched to placebo (74% of patients in the canakinumab group had no flare, vs. 25% in the placebo group, according to Kaplan-Meier estimates; hazard ratio, 0.36; P=0.003). The average glucocorticoid dose was reduced from 0.34 to 0.05 mg per kilogram per day, and glucocorticoids were discontinued in 42 of 128 patients (33%). The macrophage activation syndrome occurred in 7 patients; infections were more frequent with canakinumab than with placebo. CONCLUSIONS: These two phase 3 studies show the efficacy of canakinumab in systemic JIA with active systemic features. (Funded by Novartis Pharma; ClinicalTrials.gov numbers, NCT00889863 and NCT00886769.).

Frequency of pericardial effusion in childhood acute lymphoblastic leukemia and clinical features of these patients.

In this study, we aimed to determine the frequency of pericardial effusion (PE) in children diagnosed with acute lymphoblastic leukemia (ALL). Clinical features of patients with effusion were evaluated. For this purpose, we reviewed the medical records of ALL patients who had pretherapy echocardiograms. A total of 90 patients aged between 1.8 and 16.3 years were analyzed retrospectively. In 23 of 90 (25.6%) patients, PE was detected at initial diagnosis. The age of patients with PE ranged between 1.8 and 14.8 years (mean 5.05 ± 3.77 years). The female/male ratio was 9/14. Six (26.1%) patients were T-lineage and 17 (73.9%) were B-lineage ALL. Nine (39%) patients were in standard risk group, 13 (57%) were in median risk group, 1 (4%) patient was in high-risk group. Mean initial white blood cell count was 40.756 ± 38.653/mm(3) (range 23.000-130.000/mm(3)). Mean initial hemoglobin count was 7.3 ± 1.39 gr/dL (range 5.5-10.1 gr/dL), mean initial platelet count was 35.200 ± 26.300/mm(3) (range 4.000-118.000 mm(3)). Size of effusions was between 2 and 6 mm (mean size 3.3 ± 1.8 mm). All patients had normal left ventricular systolic function. In 87% of patients, effusions disappeared in the first 7 days and, in 13%, disappeared between 8th and 15th days of chemotherapy. None of the patients required pericardiocentesis. Cardiac dysfunction did not occur among any of these patients during chemotherapy. In conclusion, PE is not frequent in childhood ALL. It usually does not cause cardiac impairment. It responds to treatment of leukemia.

Bart's syndrome associated with pyloric and choanal atresia.

Bart's syndrome, first described by Bart in 1966, consists of congenital localized absence of skin, congenital epidermolysis bullosa, and associated nail abnormalities. A newborn infant with Bart's syndrome is reported since it is a very rare condition, especially when associated with pyloric and concomitant choanal atresia. To the best of our knowledge, this is the first report presenting a case of Bart's syndrome associated with choanal atresia.

Assessment of the changing trends in maternal knowledge about management of fever and antibiotic use in the last decade in Türkiye.

BACKGROUND: The wrong attitudes of parents on fever create a basis for unnecessary drug use and increased workload. The study was conducted to evaluate the knowledge and attitudes concerning fever and antibiotic use and demonstrate the changes in the last decade. METHODS: This cross-sectional study was composed of two parts, and a total of 500 participants were included. Group 1 (the new group, 50.0%) consisted of 250 participants who participated in the study between February 2020 and March 2020 and Group 2 (the old group, 50.0%) consisted of 250 participants who participated in the study between February 2010 and March 2010. All participants share the same ethnic properties and had been visiting the same center for similar reasons. A validated, structured questionnaire assessing the management of fever and antibiotic use was administered to all mothers. RESULTS: According to the fever assessment scoring, maternal knowledge of fever and its management in children significantly increased (p < 0.001). The antibiotic assessment score also increased in 2020 (p = 0.002). CONCLUSIONS: The public spotlight on the erroneous use of antibiotics and the management of febrile illnesses seems to be promising. Improving maternal/parental educational status and informational advertisements can enhance parental knowledge concerning fever and antibiotic use.

Does hypereosinophilic syndrome precede common B acute lymphoblastic leukaemia in childhood? A case report.

Hypereosinophilic syndrome (HES) and the association of hypereosinophilia with acute lymphoblastic leukaemia (ALL) are both rare in children. Some acute myelogenous leukaemias can present with eosinophilia, but the relationship between HES and ALL is not well known and is rarer than the relationship between HES and acute myelogenous leukaemia. Patients are diagnosed with HES when no cause is found to explain the eosinophilia leading to end organ damage. For this reason, it is recommended that patients presenting with hypereosinophilia be carefully assessed to exclude any malignant clonal proliferation. HES may present with severe clinical manifestations such as high leucocyte count, anaemia, thrombocytopaenia, hepatosplenomegaly or cardiac and neurological involvement, all of which are primarily features of myeloproliferative disorders. Some patients with HES can develop chronic eosinophilic leukaemia. Successful treatment of HES with agents used in chronic myeloid leukaemia supports the idea that HES can be a chronic myeloid disorder. There are few cases reporting an association between ALL and hypereosinophilia that precedes or is concomitant with ALL. Here we report the case of a 14-year-old girl who developed common B ALL 7 months after diagnosis and treatment of HES. Interestingly, eosinophilia was not concomitant with the diagnosis of ALL.

The epidemiology and economic impact of varicella-related hospitalizations in Turkey from 2008 to 2010: a nationwide survey during the pre-vaccine era (VARICOMP study).

Varicella can cause complications that are potentially serious and require hospitalization. Our current understanding of the causes and incidence of varicella-related hospitalization in Turkey is limited and sufficiently accurate epidemiological and economical information is lacking. The aim of this study was to estimate the annual incidence of varicella-related hospitalizations, describe the complications, and estimate the annual mortality and cost of varicella in children. VARICOMP is a multi-center study that was performed to provide epidemiological and economic data on hospitalization for varicella in children between 0 and 15 years of age from October 2008 to September 2010 in Turkey. According to medical records from 27 health care centers in 14 cities (representing 49.3% of the childhood population in Turkey), 824 children (73% previously healthy) were hospitalized for varicella over the 2-year period. Most cases occurred in the spring and early summer months. Most cases were in children under 5 years of age, and 29.5% were in children under 1 year of age. The estimated incidence of varicella-related hospitalization was 5.29-6.89 per 100,000 in all children between 0-15 years of age in Turkey, 21.7 to 28 per 100,000 children under 1 year of age, 9.8-13.8 per 100,000 children under 5 years of age, 3.96-6.52 per 100,000 children between 5 and 10 years of age and 0.42 to 0.71 per 100,000 children between 10 and 15 years of age. Among the 824 children, 212 (25.7%) were hospitalized because of primary varicella infection. The most common complications in children were secondary bacterial infection (23%), neurological (19.1%), and respiratory (17.5%) complications. Secondary bacterial infections (p < 0.001) and neurological complications (p < 0.001) were significantly more common in previously healthy children, whereas hematological complications (p < 0.001) were more commonly observed in children with underlying conditions. The median length of the hospital stay was 6 days, and it was longer in children with underlying conditions (<0.001). The median cost of hospitalization per patient was $338 and was significantly higher in children with underlying conditions (p < 0.001). The estimated direct annual cost (not including the loss of parental work time and school absence) of varicella-related hospitalization in children under the age of 15 years in Turkey was $856,190 to $1,407,006. According to our estimates, 882 to 1,450 children are hospitalized for varicella each year, reflecting a population-wide occurrence of 466-768 varicella cases per 100,000 children. In conclusion, this study confirms that varicella-related hospitalizations are not uncommon in children, and two thirds of these children are otherwise healthy. The annual cost of hospitalization for varicella reflects only a small part of the overall cost of this disease, as only a very few cases require hospital admission. The incidence of this disease was higher in children <1 year of age, and there are no prevention strategies for these children other than population-wide vaccination. Universal vaccination is therefore the only realistic option for the prevention of severe complications and deaths. The surveillance of varicella-associated complications is essential for monitoring of the impact of varicella immunization.

The forgotten chamber: right-ventricular functions in juvenile idiopathic arthritis.

Cardiac involvement, such as pericarditis, myocarditis, and endocarditis, is seen in juvenile idiopathic arthritis. Although there have been some reports about right-ventricular systolic and diastolic functions of adults with rheumatoid arthritis and left-ventricular systolic and diastolic functions of children with JIA, there have been no studies about RV functions of children with JIA. The aim of this study was to determine RV functions in children with JIA. We performed conventional echocardiography and tissue Doppler imaging measurements of the right ventricle of patients with JIA. All patients were in sinus rhythm at the time of examination without overt LV heart failure and with normal LV ejection fraction. Fifty-five children with the diagnosis of JIA and 33 healthy control subjects were included in the study. Peak systolic, early, and late diastolic tricuspid annular velocities were significantly decreased in JIA patients compared with healthy controls (p < 0.05). Isovolumic accelaration (IVA), as a measure of myocardial acceleration during isovolumic contraction of the right ventricle, was also significantly lower in JIA patients than in healthy controls (p < 0.05). RV systolic and diastolic functions, in addition to the previously shown LV functions, are affected in JIA patients. IVA decreases in JIA patients and may be used as an alternative, noninvasive parameter for the assessment of RV systolic function in children with JIA.

The distribution of juvenile idiopathic arthritis in the eastern Mediterranean: results from the registry of the Turkish Paediatric Rheumatology Association.

OBJECTIVES: To analyse the demographics, main clinical and laboratory features and subtype distribution of juvenile idiopathic arthritis (JIA) in an eastern Mediterranean country, based on a multicentre registry. METHODS: Between March 2008 and February 2009 with this cross-sectional study, consecutive patients seen with JIA in selected centres were registered through a web-based registry. All patients were classified according to the International League of Associations for Rheumatology (ILAR) criteria. RESULTS: There were 634 patients with a mean age of 11.84 ± 4.66 years and the female/male ratio was 1.2. The distributions of JIA patients according to onset of disease were as follows: systemic 92 (14.5%), oligoarticular extended 26 (4.1%), oligoarticular persistent 234 (36.9%), rheumatoid factor (RF) positive polyarthritis 20 (3.2%), RF negative polyarthritis 129 (20.3%), enthesitis-related 120 (18.9%), psoriatic 13(2.1%). The frequency of uveitis was 15.7% among all of the oligoarthritis patients. Anti-nuclear antibody (ANA) was positive mainly among the oligoarticular onset patients. Twenty-one patients also had Familial Mediterranean fever (FMF). Among systemic JIA patients, the frequency of macrophage activation syndrome (MAS) was 15.2% (n=14). At the end of the mean follow-up of 7.6 ± 4.4 years, 305 (48.1%) patients were defined to have inactive disease on medication, and 106 (16.7%) were completely free of any disease symptoms without medication. CONCLUSIONS: Enthesitis related arthritis had a high frequency whereas psoriatic arthritis was very rare compared to other series. We suggest that there are certain differences in the characteristics of JIA in our eastern Mediterranean population. Thus, genetic studies need to be assessed in these populations separately and findings of genome wide association studies need to be confirmed in different populations.

Status epilepticus in children: causes, clinical features and short-term outcome.

BACKGROUND: We aimed to evaluate the cause, clinical profile, and short-term outcome of status epilepticus cases that were admitted to our pediatric emergency unit between 1 January and 31 December 2008. METHODS: We studied the clinical features of 59 seizures that occurred in 56 patients aged between 3 months and 15 years with the diagnosis of status epilepticus. We observed the clinical course and outcome of 53 cases for 6 to 18 months. The correlation between the cause of the seizure and the patient's age at the time of status epilepticus was evaluated as well as the correlation between the risk of seizure recurrence and family history of seizure, the neurological status of the patient prior to seizure and the presence of epilepsy. RESULTS: The most common cause of status epilepticus is febrile illness in children younger than 2 years and idiopathic/cryptogenic and remote symptomatic causes in children older than 2 years. The rate of recurrence of seizure was significantly higher in cases with existing neurological abnormalities, prior epilepsy and seizures with remote symptomatic causes. The most common triggering factors of status epilepticus development in cases with epilepsy were noncompliance for anti-epileptic drugs and infectious fever. CONCLUSIONS: In our study, the risk factors for seizure recurrence were the presence of prior epilepsy, existence of neurological abnormalities and remote symptomatic causes. We argue that improving the compliance of patients and their families to take medicine appropriately and training them in how to cope with febrile illnesses may decrease the recurrence of seizures.

Psychomotor development in children with iron deficiency and iron-deficiency anemia.

BACKGROUND: Iron deficiency and iron-deficiency anemia are the most common nutritional deficiencies in children, especially in developing countries. Iron-deficiency anemia in infancy is associated with impaired neurodevelopment. Studies have shown an association between iron deficiency without anemia and adverse effects on psychomotor development. OBJECTIVE: To determine the effects of iron deficiency and iron-deficiency anemia on psychomotor development in childhood. METHODS: . We evaluated psychomotor development in healthy children with iron deficiency and iron-deficiency anemia with the use of the Denver II Developmental Screening Test (DDST-II). If the child score was more than 90th percentile compared to children in the same age group, the test was scored as "delay" it was scored as a "caution" if the child score was between the 75th and 90th percentiles. The test result was interpreted as "normal," if there was no delay and only one "caution" for any item. If the child had one or more "delays" or more than two "cautions," the result was classified as "abnormal." RESULTS: DDST-II scores were abnormal in 67.3% of subjects with iron-deficiency anemia, 21.6% of those with iron deficiency, and 15.0% of control subjects. The difference from the control group in the percentage of abnormal scores was significant for subjects with iron-deficiency anemia (p < .01) but not for those with iron deficiency (p = 0.203); p > .05. (p-value, post-hoc comparison of 2 groups.) CONCLUSIONS: Iron-deficiency anemia impaired psychomotor development during childhood. However, the evidence on the adverse effects of iron deficiency remains controversial. The Denver II Developmental Screening Test is a valuable test to detect early developmental delays, especially in infants with risk factors.

Obesogenic Environment in Childhood: Implications of High Socioeconomic Level in a Developing Country.

OBJECTIVE: Today, obesity in childhood is considered as an important health problem, especially in developed countries. This study aims to determine the prevalence of obesity in children and to reveal the relationship between television watching habit, computer use and socioeconomic status in children. METHOD: A cross-sectional study was performed in 11 public schools selected with random sampling in Istanbul. A total of 1479 children (767 males (51.9%)) were included in the study. Their ages ranged from 10-15, and the mean age was 11.95±1.36 years for girls and 12.06±1.39 years for boys. Demographic characteristics and factors affecting obesity were inquired with the structured questionnaire form.The height, and body weights of the students were measured, and their body mass indexes were calculated. Those with a BMI above the 95th percentile were considered obese, and those between the 85th and 95th percentiles were considered as overweight. RESULTS: In the study group, the obesity rate was calculated as 5.3%, and the mild obesity rate as 14.9 percent. The total prevalence of overweight and obese children was 20.2%. The frequency of obesity in the high socioeconomic level group was significantly higher than low one (p<0.01). The average daily screen time was 3.74±1.58 hours. The obesity risk was found to increase three times when this period exceeded five hours. In the logistic regression analysis, while high socioeconomic level was found as the main determinant among the risk factors for obesity, the effects of daily screen time were found to be marginally significant (p<0.045). CONCLUSION: The lifestyle of the families with high socioeconomic status is the main determinant of obesity in adolescents. Nutrition with higher calorie foods, more frequent media use and a more sedentary life brought about is an obesogenic environment created by a high socioeconomic status. Environmental risk factors for obesity should be assessed.

Relation between microalbuminuria and gene mutations in familial Mediterranean fever.

We aimed to investigate the urinary microalbumin level, which is a sensitive marker of glomerular function for establishing probable renal involvement in early stages of the disease in patients with familial Mediterranean fever (FMF), and to determine the relation between gene mutations of these cases and urinary microalbumin levels. Fifty patients with FMF who were admitted to our department and had been followed up in the pediatric rheumatology outpatient clinic for five years were included in the study. Diagnosis was based on Tel-Hashomer criteria. Gene mutations (M694V, V726A, M680I) and acute phase reactants were determined as supportive findings. Routine renal function tests with 24 hour urinary microalbumin levels and urinary microalbumin/creatinine ratios were evaluated. There was a statistically significant difference between the study and control groups in terms of microalbumin/creatinine ratios, whereas no difference was observed with respect to the other parameters. Comparison of subgroups (gene mutations) in terms of all parameters (age, age at diagnosis, duration of delay in treatment, glomerular filtration rate, tubular reabsorption of phosphorus, and microalbumin/creatinine ratios) showed no difference. We suggest measurement of urinary microalbumin levels at regular intervals in order to establish renal injury early and decrease related complications.

Chylous ascites after lymphadenectomy in a Wilms` tumor patient.

Yörük MA, Erat-Nergiz M, Timur Ç, Canbolat-Ayhan A, Ergüven M. Chylous ascites after lymphadenectomy in a Wilms` tumor patient. Turk J Pediatr 2018; 60: 436-438. Wilms` tumor is the most common renal malignancy in children and the fourth most common childhood cancer. It accounts 6-7% of all childhood malignancies. Surgical resection is an important therapy option and transabdominal or transperitoneal resection with lymph node sampling is preferred. Development of chylous ascites following intraabdominal or retroperitoneal resection in pediatric age group generally results from extensive lymph node dissection, accidental ligation or interruption of lymphatic ducts. Diseases or conditions affecting abdominal and/or retroperitoneal lymph nodes may cause chylous ascites. Postoperative chylous ascites is associated with significant morbidity and may cause mechanic, nutritional and immunological complications. In the present study, a 16-month-old infant with Stage IV Wilms` tumor who developed chylous ascites after left nephrectomy and lymphadenectomy will be presented; chylous ascites treatment with enteral nutrition and surgical treatment approach for Wilms` tumor will be reviewed.

A case of early diagnosed carnitine deficiency presenting with respiratory symptoms.

INTRODUCTION: Carnitine deficiency is an autosomal recessively inherited disease characterized by a low carnitine concentration in plasma and tissues. Primary carnitine deficiency (PCD) is caused by a deficiency in the plasma membrane carnitine transporter, with urinary carnitine wasting causing systemic carnitine depletion. The most common presentation of PCD is hypoketotic hypoglycemic encephalopathy. Cardiomyopathy can also be seen. CASE REPORT: A 9-month-old girl was admitted to our clinic with wheezing, respiratory distress and nighttime cough. She was pale, expirium was prolonged, breath sounds were coarse bilaterally and were increased in the right hemithorax. RESULTS: She had hypochromic microcytic anemia and the serum CPK level was elevated. Cardiothoracic index was increased (0.62). In the chest X-ray there was hyperaeration especially in the upper regions of the left lung, and paracardiac infiltration in the right lung. The echocardiogram showed dilated cardiomyopathy. In pulmonary perfusion scintigraphy, perfusion of the right lung was 26% and of the left lung 74%. Cardiomegaly and dilatation in main the pulmonary artery was detected in the MR angiogram. Plasma carnitine and acylcarnitine levels were found to be significantly low. Fat accumulation in myocytes and rare atrophic fibers were detected in a muscle biopsy. Oral carnitine supplementation was started at a dose of 100 mg/kg. All the symptoms and findings regressed within a short period of time. DISCUSSION: This case was presented to emphasize that carnitine deficiency can present with respiratory tract symptoms like wheezing and recurrent respiratory tract infections. Although PCD usually presents with hypoketotic hypoglycemia in infants, it also has to be suspected in the etiology of dilated cardiomyopathy. Treatment is very easy and lifesaving once the correct diagnosis is made, and the prognosis is excellent with lifelong carnitine supplementation.

Systemic juvenile idiopathic arthritis as a fever of unknown origin.

Juvenile idiopathic arthritis (JIA) is a rare inflammation with still unidentified cause. It can also be cause of fever of unknown origin. Diagnosis is made by eliminating infection, malignancy, and rheumatological diseases. In this report, case of a 5-year-old patient with symptoms of intermittent fever, areas of rash on the body, itching, and swelling, redness, and pain in the right and left ankle is described. Serological test results were negative for infectious agents, and malignancy was excluded. Patient was diagnosed with systemic JIA associated with intermittent fever, negative rheumatological markers and negative serology test results. Treatment with methylprednisolone and methotrexate yielded positive clinical response. Diagnosis of systemic JIA can be challenging, and must be made by eliminating other diseases.

Carotid sheath abscess caused by a tooth decay infection on the opposite side.

Deep neck infections are mortal diseases that need emergency treatment. It can occur at any age but usually in pediatric ages. In this report, a left cervical carotid space abscess of a pediatric patient was discussed. It was interesting that the only origin of the left carotid sheath abscess was right inferior first molar tooth decay. Right neck spaces were all clean. Patient had no immunosupression and also there were no congenital masses such as branchial cleft cysts, foreign bodies, or masses suspicious for malignancies in cervical ultrasound and MRI. We discussed this rare condition under the light of the literature.

Comparison of transcutaneous and total serum bilirubin measurement in Turkish newborns.

Severe neonatal hyperbilirubinemia can be prevented by screening for neonatal jaundice. Transcutaneous bilirubin (TcB) measurement is a noninvasive method for screening neonates. The aim of this study was to examine the correlation between TcB measurement (using the JM-103 bilirubinometer) and total serum bilirubin (TSB) measurement. To our knowledge, this is the first study evaluating the usefulness of the JM-103 bilirubinometer in Turkish neonates. Two hundred and fifty healthy infants in our well-baby nurseries and follow-up clinic with a gestational age of ≥36 weeks who were ≤15 days old were enrolled in this study. TcB measurements were taken usinng the JM-103; almost simultaneously, TSB was checked using a spectrophotometric method. The mean±SD TSB level was 11.2±4.6 mg/dl (range, 0.9-27.0 mg/dl); 17.2% of cases had TSB>15 mg/dl. There was good correlation between transcutaneous bilirubin and total serum bilirubin measurements (Pearson's correlation coefficient 0.87 for TcB from the forehead, 0.88 for TcB from the sternum; p<0.001). The transcutaneous bilirubin measurement tended to underestimate the value with increasing discrepancy at higher TSB values. The mean difference between serum bilirubin and transcutaneous (from the sternum and forehead) bilirubin values was significantly lower in cases not requiring phototherapy than in those requiring phototherapy [2.6 mg/dl (sternum) vs 4.8 mg/dl, 2.9 mg/dl (forehead) vs. 5.2 mg/dl, respectively; p<0.001] Although the JM-103 bilirubinometer tends to underestimate serum bilirubin, especially in patients with high bilirubin levels, it is a suitable screening tool to identify jaundiced infants that require a serum bilirubin check and may reduce the need for TSB measurements.