Direct Pulp Capping of Primary Molars with Calcium Hydroxide or MTA Following Hemorrhage Control with Different Medicaments: Randomized Clinical Trial.

Purpose: This study aimed to evaluate the effects of different medicaments (sterile saline [SS]; ferric sulfate [FS]; or sodium hypochlorite [SH]) and pulp capping materials (calcium hydroxide [CH] or mineral trioxide aggregate [MTA]) on the success of direct pulp capping (DPC) in primary molars. Methods: The study was conducted with 55 children aged four to eight years. A total of 118 teeth, in which occlusal caries removal resulted in pulp exposure, were treated with DPC across six groups: SS+CH; FS+CH; SH+CH; SS+MTA; FS+MTA; and SH+MTA. Teeth were restored with Class I composite resin. Results: After two years, the overall clinical and radiographical success for DPC were 94.1 percent (111 out of 118 teeth) and 88.9 percent (105 out of 118 teeth), respectively. The clinical and radiographical success, respectively, for hemorrhage control medicaments were 92.1 percent and 89.5 percent for SS, 92.5 percent and 82.5 percent for FS, 97.5 percent, and 95.0 percent for SH (P>0.05). Internal resorption was significantly higher in the FS+CH group when compared to other groups (P<0.05). MTA had significantly higher success than CH for clinical (98.3 percent versus 89.7 percent) and radiographical success (98.3 percent versus 79.3 percent) (P<0.05, each comparison). Conclusions: For primary molars with occlusal caries and less than one-mm exposure sites, these findings suggest that direct pulp capping with MTA following hemorrhage control with the tested solutions offers a more predictable outcome compared to CH. Further, the findings of this study indicate an increased risk for internal resorption when FS and CH are used for DPC.

In Vitro Evaluation of Different Protocols for Preventing Microleakage of Fissure Sealants Placed Following Saliva Contamination.

PURPOSE: To evaluate the effect of different enamel conditioning protocols and their re-application on the microleakage of fissure sealants placed following saliva contamination. STUDY DESIGN: The study included 156 human third molars in 16 subgroups (2×4×2) under two main groups (sealant type): Group A- hydrophobic resin sealant, 3M Clinpro™ Sealant; Group B- hydrophilic resin sealant, Ultraseal XT Hydro. Each group was then divided according to the type of surface conditioning; 1- Er,Cr:YSSG laser etching, 2- acid-etching, 3- acid-etching+etch-and-rinse adhesive (Prime&Bond® One Select) and 4- self-etching adhesive (Clearfil™ SE Bond). After contaminating the conditioned occlusal enamel surfaces with artificial saliva, fissure sealant was applied in half of the specimens (a), whereas in the other half, (b) the respective surface conditioning was repeated and then fissure sealant was placed. Following thermocycling, the samples were immersed in basic fuchsin, sectioned, and dye penetration was quantitatively assessed with ImageJ. Two-way ANOVA and Bonferroni post-hoc tests were used for statistical analyses (p<0.05). RESULTS: The least microleakage was observed in A3b and A3a, whereas B4b and B4a were the subgroups with the highest microleakage. Following saliva contamination, when surface conditioning was not re-applied, the effects of fissure sealant types and surface conditioning were significant (p=0.005 and p<0.001, respectively). However, their interaction was insignificant (p=0.173). When surface conditioning was re-applied after saliva contamination, the effects of type of fissure sealant and surface conditioning (p=0.000, for both) and their interaction (p=0.004) were significant. CONCLUSIONS: 3M Clinpro™ Sealant was superior to Ultraseal XT Hydro. Re-application of Er,Cr:YSSG laser and the self-etching adhesive did not affect the microleakage of both fissure sealants. Without re-application, acid-etching+etch-and-rinse adhesive was superior to acid-etching only. However, both of them were similarly successful when they were re-applied following saliva contamination.

Turkish paediatric dentists' knowledge, experiences and attitudes regarding child physical abuse.

OBJECTIVES: Numerous studies have shown that the education of health professionals is essential to effectively respond to child abuse. The present study aimed to evaluate Turkish paediatric dentists' knowledge, experiences and attitudes regarding child physical abuse. MATERIALS AND METHODS: An electronic questionnaire was e-mailed to 518 paediatric dentists. Participants' knowledge of diagnostic indicators of child physical abuse, and their past experiences, attitudes and self-assessment of educational needs were evaluated. RESULTS: The response rate was 40.9% (n = 212). Participants who completed their undergraduate education before 1997 received significantly less education on child physical abuse than participants who completed their education more recently (P < 0.001). Although statistically insignificant, participants who completed their doctorate/specialty training after 2012 received increased education on child physical abuse (P = 0.06). Of the participants, 43.9% suspected physical abuse; however, only 12.7% reported it. 'I did not know where and how to report' was the most common reason for not reporting physical abuse. The rate of suspicion was higher among dentists from state hospitals or oral health centres (P < 0.05). Of the participants, 70.3% did not know about the legal sanctions for delay in or not reporting suspected cases. Only 15.6% assessed themselves as competent to diagnose and report physical abuse. Almost all of them acknowledged their need for more education on this topic. CONCLUSION: Turkish paediatric dentists' educational needs for diagnosing and reporting of child physical abuse cases should be met.

Primary molar pulpotomies with different hemorrhage control agents and base materials: A randomized clinical trial.

OBJECTIVE: To evaluate the clinical and radiographical success of primary molar pulpotomies which used 15.5% ferric sulfate (FS) or 1.25% sodium hypochlorite (NaOCl) for hemostasis and zinc oxide-eugenol (ZOE) and calcium hydroxide (CH) pastes as base materials. METHODS: In 29 healthy children, 80 primary molars were randomly allocated to one of the study groups: Group 1: FS-ZOE, Group 2: FS-CH, Group 3: NaOCl-ZOE, and Group 4: NaOCl-CH. After hemostasis with the respective solutions, pulp stumps and floor of the pulp chambers were covered with either ZOE or CH pastes. All teeth were restored with stainless steel crowns. Follow-up examinations were carried out at 1, 3, 6, and 12 months. RESULTS: One tooth in Group 1 and two teeth in Group 4 were extracted because of pain and periapial pathosis at sixth month. After 12 months, clinical success rates of pulpotomies in Groups 1-4 were 95%, 100%, 100%, and 89.5%, respectively. The differences were not significant (P = 0.548). Radiographic success rates for Groups 1-4 were 80%, 88.9%, 78.9%, and 84.2%, respectively. No statistically significant difference was found (P = 0.968). Pain on percussion was the most observed clinical finding. However, internal root resorption was the most common radiological finding and it was observed significantly more in mandibular primary molars (P < 0.05). CONCLUSION: Both ZOE and CH can be preferred as base materials after hemostasis achieved by the use of 15.5% FS or 1.25% NaOCl in primary tooth pulpotomy.

Partial Pulpotomy in Immature Permanent Molars After Carious Exposures Using Different Hemorrhage Control and Capping Materials.

PURPOSE: This study compared the outcome of partial pulpotomies with mineral trioxide aggregate (MTA) or calcium hydroxide (CH) following hemorrhage control with 2.5 percent sodium hypochlorite (SH) or 0.9 percent sterile saline (SS) solutions in cariously exposed immature permanent molars. METHODS: Following removal of two to three mm of the pulp at the exposure site, 80 Class I cavities were randomly allocated to four groups according to the hemorrhage control agent and pulp-capping material used: (1) group one-SH plus MTA; (2) group two-SS plus MTA; (3) group three-SH plus CH; (4) group four-SS plus CH. Glass ionomer cement was applied over the pulp-capping material, and the teeth were later restored with composite resin. The patients were recalled at six, 12, 18, and 24 months. RESULTS: After 24 months, simultaneous radiographic and clinical success rates were 94.4 percent, 100 percent, 95 percent, and 100 percent for groups one to four, respectively (P>.05). No significant correlation was found between marginal integrity scores of restorations and partial pulpotomy failure (P>.05). CONCLUSIONS: Partial pulpotomy, performed with MTA or CH used as the pulp-capping material following hemostasis with SH or SS solutions, provided comparable and favorable outcomes in carious pulp exposures of immature permanent teeth.

The relationship between the latency period, infection markers, and oxidant and antioxidant states in women with preterm premature rupture of membranes.

BACKGROUND: A major cause of perinatal morbidity and mortality has been reported to be preterm premature rupture of membranes (PPROM). Our objective was to evaluate oxidant-antioxidant balance, infection parameters, time interval between rupture of membranes and delivery (latency period), and the relationship among all these parameters. METHODS: Seventy-five cases with PPROM between 24 and 34 gestational weeks were included in the study. A control group of 41 women who gave birth at term were considered as the control group. The relationship among maternal plasma total oxidative stress (TOS), malondialdehyde (MDA), total antioxidant status (TAS), leukocyte counts, CRP, vitamin C and E levels, gestational week, neonatal birthweight, and latency period was evaluated. RESULTS: In cases with PPROM, rupture occurred at an average of 29.4 gestational weeks and premature babies were born at an average of 31.6 gestational weeks. The mortality rate of babies born to PPROM women was 18.7% (14/75) died at or following birth. In the PPROM group, TOS, MDA, and leukocyte counts were found to be significantly higher compared to the control group (p < 0.001). Besides, a significant negative correlation was detected among the latency period, TOS, CRP, and leukocyte counts (p < 0.05). CONCLUSIONS: Appropriate treatment protocols that strengthen antioxidant defense systems and taking into consideration the signs of infection can decrease the incidence of PPROM and/or mortality rates of babies born to PPROM women.

Influence of influenza vaccination on recurrent hospitalization in patients with heart failure.

BACKGROUND: The current study aimed to evaluate the influence of regular annual influenza vaccinations on cardiovascular (CV) death and heart failure-related hospitalizations (HFrH) in stable outpatients with heart failure with reduced ejection fraction. METHODS: The Turkish research team-HF (TREAT-HF) is a network undertaking multicenter, observational cohort studies in HF. This study is a subgroup analysis of TREAT-HF outpatient cohorts who completed a questionnaire on influenza vaccination status and for whom follow-up data were available. A total of 656 patients with available follow-up data for CV death and HFrH including recurrent hospitalization were included in the study. Patients were classified into two groups: those who received regular influenza vaccination (40 %) and those who did not receive vaccination. RESULTS: During a mean follow-up of 15 ±6 months, 113 (18 %) patients had CV death and 471 (72 %) patients had at least one HFrH. The CV death rate was similar in both groups of patients (16 vs. 19 %, p = 0.37), whereas, HFrH and recurrent HFrH were significantly less frequently encountered in patients who received regular influenza vaccination than in those who did not receive vaccination (43 vs. 92 % and 16 vs. 66 %, p < 0.001, respectively). In a multivariate Cox proportional hazards model - in addition to a few clinical factors - vaccination status (HR = 0.30, 95 % CI = 0.17-0.51, p < 0.001) and graduation from university (HR = 0.35, 95 % CI = 0.17-0.72, p = 0.004) remained independently associated with the risk of recurrent HFrH. CONCLUSION: Regular influenza vaccination does not influence CV deaths; however, it decreases HFrH including recurrent episodes of HFrH in outpatients with heart failure with reduced ejection fraction.

Assessing the effectiveness of sustainable land management policies for combating desertification: A data mining approach.

This study investigates the relationship between fine resolution, local-scale biophysical and socioeconomic contexts within which land degradation occurs, and the human responses to it. The research draws on experimental data collected under different territorial and socioeconomic conditions at 586 field sites in five Mediterranean countries (Spain, Greece, Turkey, Tunisia and Morocco). We assess the level of desertification risk under various land management practices (terracing, grazing control, prevention of wildland fires, soil erosion control measures, soil water conservation measures, sustainable farming practices, land protection measures and financial subsidies) taken as possible responses to land degradation. A data mining approach, incorporating principal component analysis, non-parametric correlations, multiple regression and canonical analysis, was developed to identify the spatial relationship between land management conditions, the socioeconomic and environmental context (described using 40 biophysical and socioeconomic indicators) and desertification risk. Our analysis identified a number of distinct relationships between the level of desertification experienced and the underlying socioeconomic context, suggesting that the effectiveness of responses to land degradation is strictly dependent on the local biophysical and socioeconomic context. Assessing the latent relationship between land management practices and the biophysical/socioeconomic attributes characterizing areas exposed to different levels of desertification risk proved to be an indirect measure of the effectiveness of field actions contrasting land degradation.

An unexpected cause of anaphylaxis: potato.

Immediate reactions against contact to raw potato has been reported in adults with generally being in the form of an oral contact dermatitis or contact urticaria, but it may also manifest as rhinitis symptoms, wheezing or even anaphylaxis. Cooked or raw potato allergy has been rarely reported in children as some is being immediate and others being late reactions, and it usually results from ingestion. Herein, we report two cases with a background of allergic diseases developed anaphylaxis one with cooked potato and the other one with raw potato.

CLINICAL FEATURES AND GENETIC ANALYSIS OF SIX PATIENTS WITH WISKOTT-ALDRICH SYNDROME REPORTING TWO NOVEL MUTATIONS: EXPERIENCE OF ERCIYES UNIVERSITY, KAYSERI, TURKEY.

AIM: The Wiskott-Aldrich syndrome (WAS) is an X-linked immunodeficiency characterized by micro-thrombocytopenia, eczema, and recurrent infections. We aimed to share our experience with six children with WAS, including two patients with two novel mutations. MATERIAL AND METHOD: We present phenotypical and laboratory description of six patients with WAS. The initial clinical presentation, biochemical and radiological features, molecular diagnosis together with long-term follow-up data are provided. RESULTS: The patients showed increased serum levels of IgE; otherwise the serum levels of IgM were decreased. The percentages of CD3+ T cells were decreased or within lower limit. Four patients underwent molecular genetics analysis and Western blot studies; two of them showed unpublished mutations: a hemizygous splice site mutation in intron 8 (c.778-2A>T), and a hemizygous deletion in exon10 of the WASP gene (c.1017delT; p.S339fsX444) were detected. Western blot studies confirmed the reduced WAS protein expression in peripheral mononuclear blood cells in four studied patients. CONCLUSIONS: The major characteristics of patients were thrombocytopenia with decreased mean platelet volume and bleeding. All patients had been previously misdiagnosed as idiopathic thrombocytopenic purpura, demonstrating the importance of a careful differential diagnosis, and intense evaluation.

Increased Postexercise Lipoxin A4 Levels in Exhaled Breath Condensate in Asthmatic Children With Exercise-Induced Bronchoconstriction.

BACKGROUND: Lipoxins could be potential modulators of inflammation in the lungs. To our knowledge, the role of exhaled breath condensate (EBC) lipoxin A4 (LXA4) in asthmatic children with exercise-induced bronchoconstriction (EIB) has not been investigated. OBJECTIVE: The aim of our study was to determine the involvement of EBC LXA4 in EIB. METHODS: Forty-five patients aged between 5 and 17 years were included in the study. Patients were divided into 2 groups: asthmatic children with a positive response to exercise (n = 17) and asthmatic children with a negative response to exercise (n = 28). Levels of LXA4 were determined in EBC before and immediately after the exercise challenge using ELISA. RESULTS: EBC LXA4 levels were significantly increased immediately after exercise in asthmatic children with a positive response to the exercise challenge (P = .05). No significant differences were observed in children with a negative response to exercise (P > .05). There was an inverse correlation between LXA4 levels and the percent degree of reduction in forced expiratory volume in the first second (FEV1%) postexercise in children with a positive exercise challenge (P = .05, r = -0.50). No significant differences were observed in LXA4 levels between atopic and nonatopic asthmatics (P > .05, Mann-Whitney U test). CONCLUSIONS: Levels of EBC LXA4 increased immediately after exercise in asthmatic children with a positive exercise challenge response. We hypothesize that airway LXA4 levels increase to compensate bronchoconstriction and suppress acute inflammation, and that spontaneous bronchodilatation after EIB may be due to LXA4.

Increased postexercise lipoxin A4 levels in exhaled breath condensate in asthmatic children with exercise-induced bronchoconstriction

Background: Lipoxins could be potential modulators of inflammation in the lungs. To our knowledge, the role of exhaled breath condensate (EBC) lipoxin A4 (LXA4) in asthmatic children with exercise-induced bronchoconstriction (EIB) has not been investigated. Objective: The aim of our study was to determine the involvement of EBC LXA4 in EIB. Methods: Forty-five patients aged between 5 and 17 years were included in the study. Patients were divided into 2 groups: asthmatic children with a positive response to exercise (n=17) and asthmatic children with a negative response to exercise (n=28). Levels of LXA4 were determined in EBC before and immediately after the exercise challenge using ELISA. Results: EBC LXA4 levels were significantly increased immediately after exercise in asthmatic children with a positive response to the exercise challenge (P=.05). No significant differences were observed in children with a negative response to exercise (P>.05). There was an inverse correlation between LXA4 levels and the percent degree of reduction in forced expiratory volume in the first second (FEV1%) postexercise in children with a positive exercise challenge (P=.05, r=-0.50). No significant differences were observed in LXA4 levels between atopic and nonatopic asthmatics (P>.05, Mann-Whitney U test). Conclusions: Levels of EBC LXA4 increased immediately after exercise in asthmatic children with a positive exercise challenge response. We hypothesize that airway LXA4 levels increase to compensate bronchoconstriction and suppress acute inflammation, and that spontaneous bronchodilatation after EIB may be due to LXA4 (AU)

Introducción: Las lipoxinas pueden actuar potencialmente como inmunomoduladores de la actividad inflamatoria en el pulmón. A nuestro entender, el papel de la lipoxina A4 (LXA4), determinada en condensado de aire exhalado (EBC) en niños asmáticos con broncoconstricción inducida por el ejercicio (BEI) no ha sido previamente investigado. Objetivo: El objetivo de nuestro estudio fue determinar la implicación de la LXA4 determinada en EBC, en el broncoespasmo inducido por ejercicio. Métodos: Se incluyeron en el estudio un total de cuarenta y cinco pacientes de edades comprendidas entre 5 y 17 años. Los pacientes se dividieron en dos grupos: niños asmáticos con respuestas positivas (n = 17) y negativas (n = 28) a la provocación bronquial con ejercicio. Los niveles de LXA4 en EBC se determinaron inmediatamente antes y después de la provocación bronquial mediante un método ELISA. Resultados: Los niveles de LXA4 en EBC aumentaron significativamente tras la provocación con ejercicio en aquellos niños asmáticos con respuestas positivas en la provocación (p = 0,05). Sin embargo, no pudimos encontrar ninguna diferencia estadísticamente significativa en pacientes con respuesta negativa al ejercicio (p > 0,05). Hubo una correlación inversa entre el incremento de los niveles de LXA4 y el grado de reducción porcentual del volumen espiratorio forzado en un segundo (FEV1%) en los pacientes con respuesta positiva a la provocación (p = 0,05, r = -0,50). No se observaron diferencias significativas en los niveles de LXA4 entre asmáticos alérgicos y no alérgicos (p> 0,05, prueba de Mann-Whitney). Conclusiones: Los niveles de EBC LXA4 se incrementan inmediatamente después de la broncoconstricción inducida por el ejercicio en niños asmáticos. Se postula que los niveles de las vías respiratorias aumentan LXA4 para suprimir la inflamación aguda en la vía respiratoria, y podrían ser responsables de la inducción de broncodilatación espontánea que aparece tras el EIB (AU)

The effects of dentin adhesives and liner materials on the microleakage of class II resin composite restorations in primary and permanent teeth.

PURPOSE: To compare the occlusal and gingival microleakage of Class-II composite restorations utilizing etch-and-rinse and self-etch adhesives and different liner materials in primary and permanent teeth. STUDY DESIGN: Standardized class-II cavities were prepared in freshly-extracted sound primary and permanent molars (n = 80/each), with all cavosurface margins involving enamel. The main experimental groups were; A. Single Bond 2/primary teeth; B. Adper SE Plus/primary teeth; C. Single Bond 2/permanent teeth; and D. Adper SE Plus/permanent teeth. Each group comprised 4 subgroups (n = 10/each) with respect to the liner material employed (n = 10/subgroup): 1. Fuji VII; 2. Fuji Triage; 3. Filtek Supreme XT Flowable Composite, and 4. No liner All teeth were restored with Filtek Supreme XT Universal Nanofilled Composite. Following thermocycling and immersion in basic fuchsin, the extent of microleakage was measured on crown sections using image analysis. The data were analyzed with Wilcoxon Signed Ranks Test, Mann-Whitney U-Test and Kruskal-Wallis One-Way ANOVA at alpha = 0.05. RESULTS: In both primary and permanent teeth the use of etch-and-rinse adhesive resulted in similar occlusal and gingival microleakage values (p > 0.05). As for the self-etch adhesive, similar results were observed (p > 0.05) with the exception of significantly less occlusal leakage in the Fuji Triage VII and Fuji Triage subgroups of primary teeth than those of permanent teeth (p < 0.05). When the effects of liner material and the type of adhesive were disregarded, significantly more gingival microleakage was observed in primary teeth than in permanent teeth (p < 0.01), while the occlusal microleakage values were similar (p > 0.05). Irrespective of the tooth type and adhesive material, comparison of subgroups containing a liner material with those without one revealed no significant differences for both occlusal and gingival microleakage values (p > 0.05). CONCLUSIONS: Occlusal microleakage was similar in both primary and permanent teeth, while a lesser extent of gingival seal was observed in primary teeth. Overall, placement of a liner material did not improve resistance to microleakage.

Evaluation and selection of indicators for land degradation and desertification monitoring: methodological approach.

An approach to derive relationships for defining land degradation and desertification risk and developing appropriate tools for assessing the effectiveness of the various land management practices using indicators is presented in the present paper. In order to investigate which indicators are most effective in assessing the level of desertification risk, a total of 70 candidate indicators was selected providing information for the biophysical environment, socio-economic conditions, and land management characteristics. The indicators were defined in 1,672 field sites located in 17 study areas in the Mediterranean region, Eastern Europe, Latin America, Africa, and Asia. Based on an existing geo-referenced database, classes were designated for each indicator and a sensitivity score to desertification was assigned to each class based on existing research. The obtained data were analyzed for the various processes of land degradation at farm level. The derived methodology was assessed using independent indicators, such as the measured soil erosion rate, and the organic matter content of the soil. Based on regression analyses, the collected indicator set can be reduced to a number of effective indicators ranging from 8 to 17 in the various processes of land degradation. Among the most important indicators identified as affecting land degradation and desertification risk were rain seasonality, slope gradient, plant cover, rate of land abandonment, land-use intensity, and the level of policy implementation.

Evaluation and selection of indicators for land degradation and desertification monitoring: types of degradation, causes, and implications for management.

Indicator-based approaches are often used to monitor land degradation and desertification from the global to the very local scale. However, there is still little agreement on which indicators may best reflect both status and trends of these phenomena. In this study, various processes of land degradation and desertification have been analyzed in 17 study sites around the world using a wide set of biophysical and socioeconomic indicators. The database described earlier in this issue by Kosmas and others (Environ Manage, 2013) for defining desertification risk was further analyzed to define the most important indicators related to the following degradation processes: water erosion in various land uses, tillage erosion, soil salinization, water stress, forest fires, and overgrazing. A correlation analysis was applied to the selected indicators in order to identify the most important variables contributing to each land degradation process. The analysis indicates that the most important indicators are: (i) rain seasonality affecting water erosion, water stress, and forest fires, (ii) slope gradient affecting water erosion, tillage erosion and water stress, and (iii) water scarcity soil salinization, water stress, and forest fires. Implementation of existing regulations or policies concerned with resources development and environmental sustainability was identified as the most important indicator of land protection.

Transthoracic echocardiographic and cardiopulmonary exercise testing parameters in Eisenmenger's syndrome. Association with six-minute walk test distance.

OBJECTIVE: The six-minute walk test (6MWT) evaluates the functional exercise capacity in patients with cardiopulmonary disease. We aimed to investigate the association between 6MWT distance and transthoracic echocardiographic (TTE) findings as well as cardiopulmonary exercise testing (CPET) parameters in Eisenmenger's syndrome (ES) patients waiting for heart-lung transplantation on their initial admission to our center. PATIENTS AND METHODS: A total of 23 patients with ES (12 women, 11 men; mean age, 28.2 ± 8.1 years) were included in the study. The correlation between 6MWT distance and CPET and TTE findings was retrospectively analyzed. RESULTS: The most frequent underlying heart diseases were ventricular septal defect (VSD) with complex congenital heart disease (n = 10, 43 %) and isolated VSD (n = 7, 30 %). The 6MWT distance was 349.7 ± 77.4 m in the study group. An inverse correlation was found between 6MWT distance and systolic pulmonary arterial pressure (SPAP) measured with TTE (r = - 0.445; p = 0.03). All patients underwent CPET at the first visit. Mean VO2 max was 14.9 ± 3.3 ml/kg/min and the VE/VCO2 rate was 50.4 ± 9.2 %. No significant correlation was observed between 6MWT and CPET findings. SPAP, which did not display any correlation with CPET findings, was the only independent predictor of 6MWT distance. CONCLUSION: We suggest that 6MWT distance may be more suitable than CPET in the follow-up of ES patients. Further prospective, randomized, controlled trials are necessary to make more robust interpretations of this issue.

Severe congenital neutropenia in two siblings related to HAX1 mutation without neurodevelopmental disorders.

Severe congenital neutropenia (SCN) is a rare primary myelopoiesis disorder, characterized by reduced absolute neutrophil counts from birth, increased susceptibility to recurrent and life-threatening infections, and a preleukemic predisposition. Herein, we describe two siblings with SCN born from consanguineous parents who were referred for complaints of recurrent cutaneous infections, gingivitis, purulent otitis media, and both lower and upper respiratory tract infections. Bone marrow aspiration of one patient demonstrated a maturation arrest in the myeloid lineage at the promyelocyte-myelocyte stages. Genetic analysis revealed a homozygous mutation in exon 2 c.130-131insA; p.W44X in the HAX1 gene. Although identical mutations were detected in both siblings, there was a clear discrepancy between the clinical course of the brother, who eventually required granulocyte colony stimulating factor (G-CSF) therapy, and the sister, who did not. Although SCN is a rare disorder, the early onset of recurrent infections and severe neutropenia, especially in children born from consanguineous parents, should always raise suspicion and warrant further evaluation.

The clinical features and genetic mutations of chronic granulomatous disease: results from a reference centre at middle Anatolia.

AIM: Chronic granulomatous disease (CGD) is a rare innate immune deficiency with neutrophil function disorder. In this retrospective study, we aimed to evaluate the clinical features of the patients with CGD. MATERIAL AND METHOD: We presented eight patients (6 boys, 2 girls) with CGD which were evaluated at Erciyes University Medical Faculty hospital between 1996 and 2012. The initial complaints, age at diagnosis, consanguinity of the parents, similar disease history or death of the siblings, physical examination, diagnostic tests, clinical courses, and genetic characteristics were analyzed. RESULTS: The initial complaints were started before the age of one in four patients; whereas only two patients diagnosed before the first birth day. Lymphadenomegally, suppurative infections, pneumonia, diarrhea were the most noted initial complaints. All parents were consanguineous. The clinical features were mild; and the ages of diagnosis were late in patients with p47 and p67 defect. The patient with X linked CGD was diagnosed when he was 3 months old; his clinical course was complicated with chronic otitis media, zygomatic abscess, lung abscess, and facial paralysis. The patient with p22 defect was diagnosed at two months of age; and gastric wall granuloma, inflammation in proximal femur was detected. CONCLUSIONS: The awareness of the clinicians about CGD will result in early diagnosis and consequently reduce the mortality and morbidity of this disease.

Preserving alveolar bone growth following cervical root fracture: a case report.

Root fracture injuries affect 0.5%-7% of permanent teeth. Although cervical root fractures are less frequent in children, their serious consequences and poor prognosis may lead to tooth loss. In this case presentation, we discuss a treatment approach chosen to preserve alveolar bone growth following a cervical root fracture in an 8-Year-old boy.