A hemophagocytic lymphohistiocytosis case with newly defined UNC13D (C.175G>C; p.Ala59Pro) mutation and a rare complication

Hemophagocytic lymphohistiocytosis (HLH) represents a severe hyperinflammatory condition with cardinal symptoms of prolonged fever, cytopenias, hepatosplenomegaly, and hemophagocytosis by activated, morphologically benign macrophages with impaired function of natural killer cells and cytotoxic T lymphocytes. A 2-month-old girl, who was admitted with fever, was diagnosed with HLH and her genetic examination revealed a newly defined mutation in the UNC13D (c.175G>C; p.Ala59Pro) gene. She was treated with dexamethasone, etoposide, and intrathecal methotrexate. During the second week of treatment, after three doses of etoposide, it was noticed that there was a necrotic plaque lesion on the soft palate. Pathologic examination of debrided material in PAS and Grocott staining revealed lots of septated hyphae, which was consistent with aspergillosis infection. Etoposide was stopped and amphotericin B treatment was given for six weeks. HLH 2004 protocol was completed to eight weeks with cyclosporine A orally. There was no patient with invasive aspergillosis infection as severe as causing palate and nasal septum perforation during HLH therapy. In immuncompromised patients, fungal infections may cause nasal septum perforation and treatment could be achieved by antifungal therapy and debridement of necrotic tissue. © 2015 Turkish Society of Hematology. All rights reserved

The effects of caspofungin and voriconazole in an experimental fungal infection of the ear due to Aspergillus

The purpose of the study was to evaluate the effectiveness of caspofungin and voriconazole in the treatment of experimental Aspergillus otits media in an experimental rabbit model. A total of 30 New Zealand white rabbits were divided into four treatment groups and one control group. The rabbits were immunosuppressed by cyclophosphamide and triamcinolone acetonide. The right ear of each rabbit was infected by an injection of the inoculum of 0.1 ml (8.6 x 103 CFU/0.1 ml) of Aspergillus fumigatus into the middle ear cavity. At 72 h after the inoculation, amphotericin B 1 mg/kg per day (n = 6), itraconazole 10 mg/kg per day (n = 6), voriconazole 10 mg/kg per day (n = 6) and caspofungin 5 mg/kg per day (n = 6) were injected to each treatment group. No antifungal drug was administered to the control group (n = 6). Clinical and histopathological examination scores and microbiological analysis of middle ear mucosa were compared.There was statistically significant difference in the clinical scores, histopathological scores, and mean CFU/g between the treatment and control groups (P < 0.05). There was no statistically significant difference among the treatment groups in the clinical and histopathological scores, whereas there was statistically significant difference in the mean CFU/g (P < 0.05). The mean CFU/g of amphotericin B and caspofungin groups were similar and both were lower than the itraconazole and voriconazole groups. Also, the mean CFU/g of voriconazole group was lower than the itraconazole group (P < 0.05). Caspofungin and voriconazole were demonstrated at least as effective as amphotericin B and itraconazole. We suggest that caspofungin and voriconazole may be considered for the treatment of fungal infection of the ear

The influence of iris color and retina pigment epithelium melanin on allergic rhinitis

Objective: The purpose of this case control study is to establish the influence of iris color and retina pigment epithelium melanin on allergic rhinitis.Methods: Ninety-nine subjects diagnosed prospectively as allergic rhinitis and 85 control subjects were included in the study. Color of iris was recorded after ophthalmic examination. Electro-oculographic measurement was done after pupil dilatation and Arden ratios were recorded.Results: Mean age was 29.44 +/- 9.8 years in subject with allergic rhinitis and 32.67 +/- 12.9 years in control subjects (p>0.05). Measured mean Arden ratio was 158.22 +/- 29.4 in subjects with allergic rhinitis and 179.34 +/- 29.3 in normal subjects (p<0.05). In control group, Arden ratio was significantly higher in eyes with brown iris (p<0.05). In subjects with allergic rhinitis, Arden ratio was significantly lower in brown eyes (p<0.05).Conclusion: An association between allergic rhinitis and melanin content of iris and retina pigment epithelium was found. It can be speculated that pigment-producing system may play a role in the pathogenesis of the allergic rhinitis

Frontal sinus mucocele: A case report

Mukoseller, epitelyal sınırlı, paranazal sinüs içini tamamen dolduran ve mukus içeren yapılardır. En sık frontal daha sonra etmoid, maksiller ve sfenoid sinüste görülürler. Büyüdükçe çevreye bası yaparak kemik erozyonuna ve sinüs dışına taşarak değişik semptomlara neden olurlar. Yedi aydır sol gözde giderek artan şişlik şikayeti ile kliniğimize başvuran 43 yaşında erkek hastanın yapılan muayenesinde sol gözde ağrı, periorbital ödem ve propitozis saptandı. Paranazal sinüs tomografisinde sol frontal sinüsü tamamen dolduran ve inferior duvarını erode ederek orbita içerisine uzanım gösteren, göz küresine üstten bası yapan 3x1, 5x3,5 cm boyutunda mukosel saptandı. Frontal sinüs mukoseli endoskopik sinüs cerrahisiyle nazal kaviteye marsupiyalize edildi.Mucoceles are the structures that are limited in the epithelium and are filling in the paranasal sinuses with mucus content. They are most frequently seen in the frontal sinus and then in ethmoid, maxillary and sphenoid sinuses, by decreasing rate. They cause bone erosion as they enlarge and different symptoms when they run over the sinus. Left orbital pain, periorbital edema and proptosis was determined in the examination of the 43 years old male patient; who was referred to our clinic with progressively enlarging left orbital tumour for seven months. In the paranasal sinus computerized tomography, a mucocele; approximately with the diameter of 3x1, 5x3,5 cm, that fills in the whole frontal sinus and reachs the orbita by eroding the inferior sinus wall and compresses the orbita superiorly, was determined. This frontal sinus mucocele was marsupialized to the nasal cavity by endoscopic sinus surgery

The effect of radiofrequency thermal ablation method on nasal mucociliary activity in patients with inferior turbinate hypertrophy

OBJECTIVESThis study aims to investigate the effect of radiofrequency thermal ablation (RFTA) treatment on nasal mucociliary activity before and after treatment in inferior turbinate hypertrophy.PATIENTS AND METHODSThirty-nine patients (21 males, 18 females; mean age 36.3±13.9 years; range 16 to 67 years) admitted to our clinic with complaint of nasal obstruction and diagnosed with stromal inferior turbinate hypertrophy were included in this study. Effect of RFTA treatment on nasal mucociliary activity was investigated by saccharine test before treatment and two months after treatment. Grade of nasal obstruction was evaluated by visual analog scale (VAS) before treatment and two months after treatment. Results were compared with paired t-test.RESULTSMean mucociliary activity times were 9.8±4.4 minutes before treatment and 9.6±4.1 minutes two months after treatment, and the difference between two tests was not statistically significant (p=0.3). Mean VAS scores for nasal obstruction were 6.5±1.2 before treatment and 3.8±1.0 two months after treatment, and the difference between two results was statistically significant (p=0.001). Difference between sexes in terms of saccharine transit time and VAS values before and after treatment was not statistically significant (p>0.05).CONCLUSIONIt was detected that RFTA has no adverse effect on mucociliary activity in the treatment of inferior turbinate hypertrophy, and this method is notably effective in relieving the nasal obstruction caused by inferior turbinate hypertrophy