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 immunocompromised patients, fungal infections may cause nasal septum perforation and treatment could be achieved by antifungal therapy and debridement of necrotic tissue.

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 otitis 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.

Can Rh antigens be a risk factor in noise-induced hearing loss?

Noise-induced hearing loss (NIHL) is one of the most common occupational problems and is one of the main causes of deafness. Many factors cause NIHL. Individual susceptibility is one of them. Rhesus (Rh) antigens and ABO blood groups can be factors in determining individual susceptibility. We aim to investigate the relationship between the Rh antigens and NIHL. The study was conducted in 438 factory workers who had been exposed to a noise level more than 85 dB for 8 h a day for a period of >/=15 years. The audiologic results and blood groups were obtained from the individual health records of the factory workers. We determined NIHL in 236 (53.9%) workers. Two hundred and nineteen (55.4%) of Rh-positive workers and seventeen (39.5%) of Rh-negative workers have NIHL, and the difference between the two groups was statistically significant (P < 0.05), whereas no statistically significant difference was determined between the NIHL and ABO blood groups. In conclusion, we suggest that the people with Rh-positive blood group are more prone to develop NIHL.

Laryngeal melanosis: a case report and review of the literature.

We present a case with laryngeal melanosis and discuss the clinical significance of this condition. A 58-year-old male patient was referred to the otolaryngology department with a 2-month history of hoarseness. He had a history of smoking 20 cigarettes a day for 40 years. Videolaryngosgopic examination showed chronic laryngitis findings with multifocal pigmented pachydermic areas. Multiple biopsies were performed by laryngomicrosurgery. Pathological examination revealed laryngeal melanosis. As the number is not much enough, the association of laryngeal melanosis and carcinogenesis seems to be controversial. In the point of management, we have to be aware of the risks of laryngeal melanosis, such as developing neoplasm, and monitor the patients closely by periodical examinations and biopsies and advise the patients to stop smoking.