Can Propranolol be used as the first line treatment in infantile hemangioma?

Hemangiomas are the most common tumors of infancy. Most of them require no treatment, but treatment is needed if dramatic aesthetic, and/or functional impairment as visual or airway obstruction or ulceration arises. We reported a 6-month-old infant presented with a 6-week history of a rapidly growing cutaneus hemangioma on the right eyelid and caused visual impairment. The patient was successfully treated with the use of oral propranolol therapy. We suggest that propranolol can be considered as a first line treatment in a patient with infantil hemangioma

Evaluation of The Renal Function in The Intrauterine Growth Restricted Rats and The Effect of Maternal Glucocorticoids

The purpose of the study was to determine the effect of maternal glucocorticoids on experimental growth retarded rats and the effect of maternal undernutrition in different gestation periods for function of the kidney. This study had two sections. In the first section, 5 groups were formed. 10g/d diet was given in the 1st, 2nd, and 3rd trimester and throughout the pregnancy period. The control group was fed a normal diet. In the second section, 3 groups formed and all the rats take 10 g/d diet throughout pregnancy period. To determine the effect of endogenous glucocorticoids first group was treated with metyrapone, second group metyrapone+dexamethasone and the placebo injected saline until 14 days of pregnancy. The offsprings body and kidney weights were detected in the 0, 3rd and 20th weeks of age. Renal extraction functions and blood pressures from tail detected in the 20th week of age. Urinary excretion and glomerular filtration rate were low in rats that had dietary restriction in the last trimester. The glomerular filtration rates were found to be low in the group that had diet restriction during the whole pregnancy. Blood pressure values were found to be lower in the group that had diet restriction during their pregnancy compared to the control group. Kidney weights were similar in all groups in the first phase. It was observed that renal excretion functions were preserved in the group receiving metyrapone treatment, but there was no statistically significant difference between the results. Low blood pressures were normalized with metyrapone treatment. The kidney sizes at the 20th week of the rats which receiving metyrapone treatment were found to be smaller than those receiving physiological saline solution. Food restriction destroys renal functions but no effects with high blood pressure in adulthood. Glucocortiod exposure in pregnancy may reduce renal development

The ımpact of handicap severity on oral and periodontal status of patients with mental retardation

Aim: Mental retardation (MR) subjects comprise a considerable amount of the community and are susceptible to oral and periodontal problems due to insufficient oral care. The aim of this cross-sectional study was to determine the oral health and periodontal status of MR patients in Turkey with regard to periodontal indices and Decay missed filling teeth (DMFT) scores and compare findings according to severity of the MR. Materials and methods: One hundred and five MR patients were included to the study and divided into 3 groups according to MR severity diagnoses. Demographic variables like age, gender, disabled sibling, BMI, living an institution and clinical parameters like plaque index (PI), gingival index (GI), bleeding on probing (BOP), probing depth (PD), clinical attachment level (CAL), missing teeth and DMFT scores were recorded from all subjects. Appropriate statistical analyses were used to compare the findings. Results: There were no statistically significant differences between groups according to age, gender, disabled sibling, living status and DMFT scores. Clinical periodontal indices and the number of missing teeth were showed a statistically significant increasing trend with the severity of MR (p < 0.05). Correlation analysis showed significant positive correlations between PI and periodontal disease measures like GI, PD, CAL and BOP and also between PI and DMFT (p < 0.05). Conclusion: The deteriorated teeth condition and deprived periodontal health of MR patients may be most likely caused by the poor oral hygiene and may be worsen with the severity of the MR

Evaluation of the current disease severity scores in paediatric FMF: Is it necessary to develop a new one?

Objectives: Modified adult disease severity scoring systems are being used for childhood FMF. We aim to test the clinical consistency of two common severity scoring systems and to evaluate the correlation of scores with the type of FMF mutations in paediatric FMF patients since certain mutations are prone to severe disease.Methods. Two hundred and fifty-eight children with FMF were cross-sectionally studied. Assessment of the disease severity was performed by using the modified scoring systems of Mor et al. and Pras et al. Genetic analysis was performed using PCR and restriction endonuclease digestion methods for the presence of 15 FMF gene mutations. FMF mutations were grouped into three based on well-known genotypic-phenotypic associations. Correlation between the mutation groups and the severity scoring systems was assessed. The consistency of the severity scoring systems was evaluated.Results. The results of two scoring systems were not statistically consistent with each other (κ = 0.171). This inconsistency persisted even in a more homogeneous subgroup of patients with only homozygote mutations of M694V, M680I and M694I (κ = 0.125). There was no correlation between the mutation groups and either of the scoring systems (P = 0.002, r = 0,196 for scoring systems of Mor et al.; P = 0.009, r = 0.162 for Pras et al.).Conclusions. The inconsistency of the two scoring systems and lack of correlation between the scoring systems and mutation groups raises concerns about the reliability of these scoring systems in children. There is a need to develop a scoring system in children based on a prospective registry. © The Author 2011. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved

A validation of diagnostic score for molecular analysis of hereditary autoinflammatory syndromes with periodic fever in Turkish children

PubMe

Behçet disease: evaluation of clinical manifestations in Turkish children

PubMe

Comparison of the efficacy of once- and twice-daily colchicine dosage in pediatric patients with familial Mediterranean fever - a randomized controlled noninferiority trial

Background: In this study, we examined the efficacy and safety of a once-daily dosage schema of colchicine compared with a twice-daily dosage schema in pediatric patients with familial Mediterranean fever (FMF). Methods: In this 24-week, multicenter, randomized controlled noninferiority trial, pediatric patients newly diagnosed with FMF carrying a homozygous or compound heterozygous mutation and not receiving any treatment were included. Patients were randomly assigned using a block randomization method to receive treatment with a once- or twice-daily dosage. Clinical and laboratory characteristics and medication side effects were recorded and compared between groups. The study was carried out in compliance with Good Clinical Practice and the Consolidated Standards for Reporting of Trials (CONSORT) statement. Results: A total of 92 patients were selected, and 79 patients completed the study. There were 42 patients in the once-daily dosage group and 37 in the twice-daily dosage group. The results indicated that the once-daily dosage was not inferior to the twice-daily dosage regarding decrease in attack frequency and duration as well as improvement in clinical findings and Mor severity scores. Alterations in laboratory findings indicating inflammation, such as erythrocyte sedimentation rate, C-reactive protein, and serum amyloid A, were similar in both groups. The rates of drug side effects were similar between the once- and twice-daily dosage groups, implying comparable safety of colchicine, with the exception of diarrhea, which was slightly higher in the once-daily dosage group. Conclusions: Using colchicine with either a once- or twice-daily dosage provides similar clinical and laboratory improvements. Considering both efficacy and safety, colchicine can be prescribed with a once-daily dosage. Trial Registration ID: ClinicalTrials.gov identifier NCT02602028. Registered 5 November 2015