Anxiety and pain during dental treatment among children with haemophilia.

AIM: Dental interventions are potentially overwhelming for children with hemophilia; the study was designed to assess the levels of dental anxiety related to the first dental intervention for these children. MATERIALS AND METHODS: Fifty-six boys with severe haemophilia A and B and 56 healthy peers between the ages of 7-12 in need of primary dental extraction were chosen for this study. Facial Analog Scale and Visual Analog Scale were applied to all participants. RESULTS: No significant differences among the groups were detected by means of the dental anxiety scores (FIS) and pain scores (VAS). The FIS scores of children who had experienced dental pain before the treatment were significantly higher regardless of the group they were part of (p=0.001). CONCLUSION: Children with haemophilia are not at an increased risk of dental anxiety using special precautions and with the help of adequate treatment regimens. Pain is a predictor for dental fear and anxiety on dental chair both for children with haemophilia and healthy ones.

Health-related quality of life in patients with haemophilia and inhibitors on prophylaxis with anti-inhibitor complex concentrate: results from the Pro-FEIBA study.

Patients with haemophilia A and inhibitors are at high risk for severe bleeding, progression of joint disease and deterioration of health-related quality of life (HRQoL). To determine the impact of prophylaxis with an activated prothrombin complex concentrate (aPCC) on HRQoL, HRQoL was assessed using the Short-Form (SF)-36 Health Survey and the EQ-5D questionnaire in subjects ≥ 14 years participating in a prospective, randomized, crossover study comparing 6 months of aPCC prophylaxis with 6 months of on-demand therapy. Eighteen of 19 patients completed the survey or questionnaire before and after the on-demand therapy and prophylaxis periods. A general trend towards improved HRQoL after prophylaxis was observed for the 18 evaluable patients in all SF-36 dimensions except for vitality/energy and physical functioning. After prophylaxis, 'good responders,' defined as patients experiencing ≥ 50% reduction in bleeding, exhibited statistically and clinically significant differences in the physical component score (P = 0.021), role - physical (P = 0.042), bodily pain (P = 0.015), and social functioning (P = 0.036). Similarly, the EQ-5D health profile showed a trend towards improvement after prophylaxis in all evaluable patients. Among the good responders, improvements did not differ from those observed after on-demand treatment. EQ visual analogue scale values were slightly improved following prophylaxis for all evaluable patients and the EQ-5D utility index improved in the good responders only. During prophylaxis, patients missed significantly fewer days from school or work because of bleeding than during on-demand treatment (P = 0.01). In conclusion, by significantly reducing bleeding frequency in good responders, aPCC prophylaxis improved HRQoL compared with on-demand treatment.

Circumcision and complications in patients with haemophilia in southern part of Turkey: Çukurova experience.

Circumcision is the oldest and most frequent surgical procedure in the world and especially in Turkey as is seen in the other Islamic countries because of religious and traditional pressures. In this study, we aim to report the experience of circumcision at Çukurova University in a total of 76 patients with haemophilia between 1990 and 2011. We retrospectively reviewed medical records of 69 haemophilia patients without inhibitors and seven haemophilia patients with inhibitors who had been circumcised. Before the year 2000, factor concentrates were given before and after circumcision for 6-7 days. After 2000, we used fibrin glue together with factor concentrates for only 3 days. By-passing agents were used for circumcision in haemophilia patients with inhibitors. Twelve of 69 patients without inhibitors were referred to our centre with bleeding after the circumcision before diagnosis of haemophilia. Nine of these twelve patients had severe life threatening bleeding and three of them had moderate bleeding. Sixty-four patients with haemophilia were circumcised in our centre under general anaesthesia except for three patients who were given local anaesthesia. Thirteen of 57 haemophilia patients (22.8%) without inhibitors had seven mild and six moderate bleeding complications. A few patients had significant bleeding, despite adequate factor replacement. Five of seven haemophilia patients with inhibitors had two moderate and three mild bleeding complications. Our experience showed that circumcision for patients with haemophilia should be carefully performed by surgeons together with paediatric haematologist, under appropriate conditions in haemophilia centres which has comprehensive coagulation lab.

The value of early treatment in patients with haemophilia and inhibitors.

Development of inhibitors to infused factor concentrates represents a major clinical and economic challenge in the treatment of haemophilic patients. It has been shown that a delay in initiation of treatment leads to requirement of a larger number of injections to stop the bleeding but this has never been formally linked to costs associated with the bleeding. The objectives of this study were to assess the relationship between time to initiation of NovoSeven and total costs, number of doses administered and time to bleeding resolution in mild to moderate bleeding episodes. Data on time to treatment initiation, time to bleeding resolution and on all resource use related to the bleeding were extracted from medical records in Turkey for 129 bleeding episodes. Regression analysis was used to assess the impact of time to treatment on outcomes. Longer time to treatment initiation increased both total costs associated with the bleeding, the number of doses needed and the time to bleeding resolution. Treatment in hospital was associated with significantly longer time to treatment, higher costs and longer time to bleeding resolution as compared with home treatment or outpatient treatment. When controlling for other bleeding characteristics, the cost of bleedings treated in hospital was more than 150% higher. This study shows that treatment with NovoSeven should be initiated as soon as possible after the onset of bleeding in order to minimize costs and optimize outcomes. Home treatment reduces time to treatment initiation and also reduces costs related to the bleeding.

Granulocytic sarcoma of gingiva: an unusual case with aleukemic presentation.

BACKGROUND: Granulocytic sarcoma (GS) is an uncommon extramedullary tumor composed of dense aggregates of immature myeloid precursor cells, which is usually associated with acute or chronic myeloid leukemia. The tumor may also be a predecessor to acute myeloid leukemia (AML). It may be found in any location throughout the body; however, intraoral occurrence is extremely rare. This report describes a case of gingival granulocytic sarcoma that developed prior to AML in a 12-year-old female. METHODS AND RESULTS: The patient, who had a 3-month history of gingival enlargement, was diagnosed as having granulocytic sarcoma based on clinical, radiological, and histological findings. Although the tumor regressed significantly after two induction chemotherapy courses, the patient subsequently died due to pneumococcal sepsis and pleurisy. CONCLUSION: This case report affirms the importance of granulocytic sarcoma in the differential diagnosis of gingival enlargements, since the tumor may occur before bone marrow involvement by leukemic cells.

Severe alveolar bone loss and gingival hyperplasia as initial manifestation of Burkitt cell type acute lymphoblastic leukemia.

BACKGROUND: The purpose of this case report is to present severe alveolar bone destruction and gingival enlargement as initial manifestation of Burkitt cell type acute lymphoblastic leukemia (ALL-L3) in a 14-year-old boy. METHODS: The patient was referred to the periodontology department with a 4-week history of gingival enlargement and loosening of teeth. The clinical examination revealed gingival enlargement and expansion of alveolar mucosa particularly in molar regions of both jaws. Almost all teeth had deep periodontal pockets and severe mobility. While the radiographs showed severe alveolar bone loss which extended to apical thirds of many teeth, the microbiologic analysis revealed that the patient did not harbor major periodontopathogenic bacteria species. The results of blood tests and bone marrow aspiration were compatible with ALL-L3. RESULTS: Remission-induction treatment with BFM-90 ALL chemotherapy protocol was started; however, the patient died 4 weeks after the diagnosis due to neutropenic sepsis. CONCLUSIONS: Although no biopsy was performed, it is possible that the severe periodontal destruction and gingival enlargement in this case may have been due to the infiltration of leukemic cells in gingiva, periodontal ligament, and alveolar bone. The similarities of these findings with numb chin syndrome (NCS) and Burkitt's lymphoma (BL) are discussed in this report.

Erythrocyte pyruvate kinase activity during chemotherapy in children with leukemia and lymphoma.

OBJECTIVES: To determine both erythrocyte pyruvate kinase activity (ePKA) at the time of diagnosis of patients with acute leukemia or lymphoma and the differences in the ePKA profiles during the malignant disease and its chemotherapy. METHODS: A prospective, longitudinal clinical study was performed involving 57 patients, 10 were the ones with relapse of acute lymphoblastic leukemia, 32 were the ones with acute lymphoblastic leukemia (ALL) and 15 were the ones with lymphoma. None of the subjects in this study group received treatment or blood transfusion before the study, except the ones diagnosed with relapse of ALL. Forty two healthy children were also selected to form the control group. In order to measure ePKA, blood samples were taken for five times, with 1.5 months apart between each other during the study. Statistical analysis were done by using Wilcoxon's signed rank test, Kruskall-Wallis with Mann-Whitney U Test and Spearman rank correlation coefficient test. RESULTS: The ePKA of the patients with relapse of ALL, and ALL, but not the patients with lymphoma, at the time of diagnosis were found to be lower compared to the one's in the control group (respectively p = 0.001, p = 0.003). The comparison between the first ePKA samples and the third ePKA samples of the patients with both ALL and lymphoma showed a significant increase (respectively p = 0.006, and p = 0.047). CONCLUSION: The measurement of ePKA can be considered for follow-up the neoplastic treatment due to the fact that it is detected to be low in leukemia and relapse of ALL and in normal values after chemotherapy. However, more long-term studies, including more number of cases, are required to be carried out in order to prove the accuracy of this hypothesis. (Tab. 2, Fig. 1, Ref. 28.)

Cytokine levels in serum of patients with juvenile rheumatoid arthritis.

We investigated serum levels of interleukin (IL)-1beta, IL-6, IL-8, IL-12 and tumour necrosis factor (TNF)-alpha in JRA patients during both active and inactive phases of the disease. The systemic JRA patients had the highest IL-1beta and IL-6 levels during both active and inactive periods. In the systemic group IL-1beta, IL-6 and IL-12 levels during the active period were elevated compared to the inactive period (p = 0.0173, p = 0.0359 and p = 0.0117, respectively). Levels of these cytokines during the inactive stage were still greater than those of controls. IL-8 and TNF-alpha levels during both active and inactive periods were comparable to controls. IL-1beta correlated strongly with CRP and ESR (p = 0.008 and p = 0.031, respectively). IL-6 correlated significantly with CRP (p = 0.002). IL-12 levels were found to be correlated with ESR and CRP (p = 0.03 and p = 0.04, respectively). In active polyarticular JRA patients, IL-6 levels were elevated compared to the inactive phase, and the control (p = 0.001) IL-12 levels decreased significantly with clinical remission (p = 0.018). There was a strong correlation between 11-12 levels and number of joint with limited motion (p = 0). In oligoarticular JRA patients, IL-12 levels during active period were greater than in the controls and there was a marked decrease in IL-12 levels when the patients entered the inactive phase (p = 0.001) In conclusion, IL-1beta, IL-6 and IL-12 may play an important role in JRA and may be used as a marker of disease activity.

Oral Health Status in Children with Acute Lymphoblastic Leukemia and Lymphoma.

We evaluated the oral health status of 85 acute lymphoblastic leukemia (ALL)/lymphoma pediatric patients who received remission-induction and maintenance chemotherapy and 85 age and sex-matched healthy children with the criteria of World Health Organization (WHO) and to determine the prevalence and distribution of dental problems in order to constitute preventive dentistry precautions in this study. The gingival tissues were scored with Community Index of Periodontal Treatment Necessity (CPITN) and dmf-t and DMF-T indices were used for caries evaluation. In the study group, malocclusion was found in 24 patients (28.2%). CPITN was scored as follows in the study group; 11% of the patients had healthy gingiva (Grade 0), the presence of plaque (Grade I) 79% of the patients, the presence of calculus (Grade II) 10% of patients were observed. Nevertheless, mucositis was found with various grades in 9 patients who received chemotherapy. Decayed teeth were found in the 76 patients and in 45 healthy children. 91.7% of patients and 52.9% of children needed dental treatment were determined. The DMF-T and dmf-t scores showed that ALL/lymphoma patients had more decayed and needed more dental treatment, missing or filled teeth both in their deciduous (p< 0.001) and permanent (p< 0.05) dentition when compared to systemically healthy children.

Prevalence of GB virus C/hepatitis G virus infection in pediatric patients receiving multiple transfusions in Southern Turkey.

The aim of this study was to determine the prevalence of GB virus C (GBV-C) infection in pediatric patients receiving multiple blood transfusions in Turkey where HBV and HCV infections are common. Sera of a total of 148 children, of whom 85 had cancer and 63 hemoglobinopathies, were tested for GBV-C RNA and HCV RNA by RT-PCR and for antibodies to HBV and HCV. Demographic and clinical information as well as laboratory results were recorded for the patients (81 boys, 67 girls, aged 1-19 years). HBsAg positivity was found in 23 (15.5%) patients, HBV DNA positivity in 12 (8.1%), HCV RNA positivity in 9 (6.7%), and GBV-C RNA positivity in 4 (2.7%). There was no significant difference in the GBV-C RNA positivity between patients with cancer (3.2%) and patients with hemoglobinopathies (2.4%) (p > 0.05). GBV-C RNA was found in 4 (3.1%) out of 127 patients who had received transfusions, but it was not found in any of 21 patients who had not received transfusions. However, there was no relationship between GBV-C RNA positivity and the number of transfusions. Two of the patients with GBV-C RNA had high levels of ALT (ALT > 40 IU). In these two patients, neither HBV DNA nor HCV RNA were detected by PCR, and serological tests were also negative for these agents. We concluded that pediatric patients who had multiple transfusions in Turkey are at risk of being infected with GBV-C, in addition to HBV and HCV. Investigation of GBV-C RNA in patients with high ALT levels in the absence of other viral markers may be useful.

The frequency of IgG subclass deficiency in children with recurrent respiratory infections.

We studied the frequency of IgG subclass deficiency in 61 children (ages 1-7 years, mean 3.8 years) with recurrent respiratory tract infections by using the radial immunodiffusion method. The overall frequency of IgG subclass deficiency was found to be 39.3 percent (24 patients). Among 26 children who had major immunoglobulin abnormalities, we found IgG subclass deficiency in 11 patients (42.3%). In children with normal immunoglobulin levels, the frequency of IgG subclass deficiency was 37.1 percent (13 patients).

Influence of smoke exposure on serum IgE levels of atopic patients.

The effect of environmental tobacco smoke on serum total immunoglobulin E (IgE) of respiratory allergic patients was evaluated in this study. A total of 558 patients with respiratory allergies (311 boys and 248 girls) aged between 1 and 17 years (mean 6.92 +/- 3.81 years) were included. None of the subjects were active smokers. IgE levels were significantly higher in children with smoking parents compared with non-smokers (P < 0.05). Children with smoking mothers were found to have higher levels of IgE than children with smoking fathers. The highest level of IgE was found in children when both parents were smokers (P < 0.005).