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Mucormycosis is a rare acute opportunistic infection caused by a saprophytic fungus, which belongs to the order Mucorales. This report describes intraoral mucormycosis in 2 children with acute leukemia who were undergoing chemotherapy and had febrile neutropenia. A 7-year-old boy with acute myeloid leukemia and a 9-year-old boy with acute lymphoblastic leukemia were referred to the Department of Pediatric Dentistry at Cukurova University for their intraoral soft tissue lesions, which were diagnosed as mucormycosis by histologic examination. While, for the first case, the lesion was debrided under general anesthesia and medical antifungal therapy was performed, only medical management was done, without any debridement, for the second case. Early recognition of mucormycosis is necessary to limit the spread of infection, which can lead to high morbidity and mortality. Therefore, health practitioners should be familiar with the signs and symptoms of the disease. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

. 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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

: We investigated serum levels of interleukin (IL)-1β, IL-6, IL-8, IL-12 and tumour necrosis factor (TNF)-α in JRA patients during both active and inactive phases of the disease. The systemic JRA patients had the highest IL-1β and IL-6 levels during both active and inactive periods. In the systemic group IL-1β, 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-α levels during both active and inactive periods were comparable to controls. IL-1β 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 Il-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-1β, IL-6 and IL-12 may play an important role in JRA and may be used as a marker of disease activity. [ABSTRACT FROM AUTHOR]

A letter to the editor regarding devastative tongue bleeding due to self-bite after dental extraction in a haemophilia is presented.

A 4-year-old girl presented with fever, coughing, and vomiting; followed by unconsciousness. Magnetic resonance imaging showed hyperintense changes in the thalami bilaterally, brain stem, cerebellum, and subcortical cortex. Novel influenza A (H1N1) virus was identified by polymerase chain reaction in patient's nasopharyngeal swab specimen. We reported a rare case of clinically severe, novel influenza A-associated encephalitis. Novel influenza A should be considered in the differential diagnosis in patients with seizures and mental status changes, especially during an influenza outbreak. [ABSTRACT FROM AUTHOR]

The article presents a case depicting the successful use of recombinant factor VIIa during bilateral cataract operation and concurrent circumcision performed on hemophilia A patient with inhibitors. The approaches in treating hemophilia includes inducing immune tolerance with high doses and long-term use and the use of prothrombin complex concentrates. Risks in performing surgical procedures include contaminating with viruses such as hepatitis B, C and HIV.