Cutaneous anthrax of the hand and its reconstruction with a reverse-flow radial forearm flap

Bacillus anthracis infection can lead to necrosis in tissues and may manifest as a fatal disease in human beings. The authors present a patient with a large area of skin necrosis on the dorsum of the hand that was reconstructed with a reverse flow-through radial forearm flap, and they discuss the relevant literature. To the authors' knowledge, this is the first published report of such extensive necrosis resulting from anthrax limited to the extensor retinaculum of the hand.     

Primary amyloidosis presenting with massive generalized lymphadenopathy

Systemic amyloidosis is an unusual cause of generalized massive lymphadenopathy. In such cases the clinical picture may mimic lymphoma. We report a case of generalized massive lymphadenopathy caused by amyloidosis. The 55-year old female patient was admitted to our hospital with dyspnea and edema in the lower extremities. These were diminished breath sounds with bilateral basal congestion hepatosplenomegaly and generalized massive lymphadenopathy in axillary, cervical, and inguinal areas. The diagnosis was made by excisional biopsy of one of the involved lymph nodes. Amyloidosis (AL type) was shown and treatment with melphalan and prednisone was initiated. Unfortunately the patient died after 51 days in hospital.     

High dose rate endobronchial brachytherapy effectively palliates symptoms due to inoperable lung cancer

BACKGROUND: Intraluminal brachytherapy has become an established treatment for major airway occlusion by relapsed or persistent inoperable endobronchial tumors. The aim of this study was to compare the palliation improvement pre- and post-radiotherapy. METHODS: The study group was 95 patients with the diagnosis of inoperable lung cancer who were eligible for HDR brachytherapy. Fiber-optic bronchoscopy was performed and the level and degree of endobronchial obstruction were estimated in terms of bronchial obstruction index. Endobronchial irradiation was delivered using remote HDR afterloading brachytherapy with iridium-192. Brachytherapy was delivered at weeks 1, 2 and 3 at 7.5 Gy per fraction or at weeks 1 and 2 at 10 Gy per fraction. All patients were evaluated at the beginning and at the third month of therapy. Using Speiser's symptomatic scoring criteria, the severity of symptoms (dyspnea, cough, hemoptysis and postobstructive pneumonia) was weighted. Bronchoscopic findings at the initial evaluation and at the third month were also scored. Surviving patients were followed up for a minimum of 3 months with a mean of 7.5 +/- 5.35 months (median: 6 months). RESULTS: All the symptoms and bronchial obstruction improved significantly after brachytherapy (P < 0.05). The most responding symptoms were dyspnea and hemoptysis. The factors determining the complete response were evaluated; age, staging, histological type, lesion localization and previous history of radiotherapy did not seem to determine the complete response (P > 0.05). CONCLUSIONS: All the symptoms and bronchial obstruction index seemed to improve after brachytherapy. However, it is difficult to predict the response before the therapy.     

MPO-ANCA-associated pulmonary-renal vasculitis in a patient with diabetes mellitus

Diabetes mellitus is one of the major causes of chronic renal failure. Typical findings of diabetic nephropathy are early hyperfiltration followed by microalbuminuria and overt proteinuria, resulting in a progressive decrease in glomerular filtration rate. Rapidly progressive glomerulonephritis has rarely been reported in patients with diabetes mellitus. Here, we describe a patient with MPO-ANCA-associated vasculitis, presenting with pulmonary-renal syndrome. Immunosuppressive treatment, including pulse methyl-prednisolone and cyclophosphamide, was administered and the disease was resolved.     

双料喉风散治疗疱疹性咽峡炎疗效观察

目的 观察双料喉风散治疗疱疹性咽峡炎的疗效。方法 198例均为我院留观治疗疱疹性咽峡炎忠儿，随机分为对照组和治疗组。对照组选用青霉素＋病毒唑静滴治疗，治疗组在此基础上加用双料喉风散直接喷雾患处，3-5d为1疗程，于治疗开始和结束时进行血常规化验。结果 两组的治疗效果经统计学处理（P〈0．05），差异有显著性。结论 双料喉风散治疗疱疹性咽峡炎可以明显缩短病程、效果好。     

Diurnal variation of intraocular pressure and its correlation with retinal nerve fiber analysis in Turkish patients with exfoliation syndrome

Purpose The purpose was to evaluate the diurnal variation (DV) of intraocular pressure (IOP) in patients with exfoliation syndrome (XS), to measure retinal nerve fiber layer (RNFL) thickness by using scanning laser polarimetry, and to compare these measurements with those of normal subjects. Methods Forty-five subjects with XS and 40 healthy, age/sex matched subjects were recruited into the study. A detailed ophthalmologic examination was performed. IOP measurements were obtained at 08:00 am, 12:00 pm, 03:00 pm, and 06:00 pm. The XS group was further divided into DV ≥5 mmHg and DV<5 mmHg groups and also according to the existence of IOP fluctuation. The IOP measurements and RNFL thickness measurements were compared between the groups. Results The mean IOP value was found to be highest in the morning both in the XS and control groups. IOP showed a gradual decrease from 8.00 am to 6.00 pm in the control group, whereas a second peak at 03:00 pm was observed in the XS group. There was a fluctuation in 53.3% of the XS group, while none of the healthy subjects showed fluctuation. Superior and inferior ratios were statistically lower in XS patients than those in control subjects (p<0.05). Moreover, in patients with XS showing a DV ≥5 mmHg and/or a fluctuation, the superior ratio, inferior ratio, the number, superior average and superior integral were significantly different (all p values <0.05) from those of control subjects. Conclusions As the XS patients with high diurnal IOP variation and fluctuating pattern of IOP had lower RNFL thickness measurements, it is crucial to follow up these patients by performing scanning laser polarimetry in order to discover any possible glaucomatous damage at an earlier stage than with the use of conventional visual field analysis.     

Cranial Nerve Root Entry Zone Primary Cerebellopontine Angle Gliomas: A Rare and Poorly Recognized Subset of Extraparenchymal Tumors

With the exception of patients with neurofibromatosis type II, pediatric extraparenchymal cerebellopontine angle (CPA) tumors of any sort are extremely rare. Most gliomas encountered in the CPA in either children or adults involve the CPA as exophytic extensions of primary brain stem and/or cerebellar tumors. We encountered an unusual case of a giant CPA pilocytic astrocytoma arising from the proximal trigeminal nerve, completely separate from the brain stem. A nine-year-old girl with no evidence for any neurocutaneous syndrome, presented with headaches, mild obstructive hydrocephalus, trigeminal hypesthesia and a subtle peripheral facial paresis. Pre-operative neuroimaging suggested a petroclival meningioma. The tumor was completely resected via a right pre-sigmoid, retro-labyrinthine, sub-temporal, transtentorial ('petrosal') approach, using intraoperative neurophysiological monitoring, with minimal morbidity. This appears to be the first reported case of a pediatric primary CPA glioma and the seventh reported case of primary CPA glioma, overall. It represents the second reported case of a primary CPA pilocytic astrocytoma. Given the findings in this case and the six other cases of primary CPA gliomas reported in the literature, as well as the results of histological studies of normal cranial nerves, we hypothesize that the point of origin of these rare and unusual tumors is the root entry zone of the involved cranial nerves. The differential diagnosis of primary CPA tumors should be expanded to include cranial nerve root entry zone primary CPA gliomas.     

The <Emphasis Type="Italic">TP53</Emphasis> mutational spectrum and frequency of <Emphasis Type="Italic">CHEK2*1100delC</Emphasis> in Li–Fraumeni-like kindreds

Li–Fraumeni syndrome (LFS) is a dominantly inherited cancer predisposition syndrome characterized by a wide spectrum of neoplasms occurring at young age. Germline mutations in the TP53 tumor suppressor gene have been identified in approximately 71 of LFS patients and 22 of Li–Fraumeni-like (LFL) patients. Mutations within the cell cycle checkpoint gene CHEK2 have also been reported in some patients with LFS, LFL, and phenotypically suggestive of LFS (PS-LFS) not carrying a TP53 mutation. In this study, we show that 7 of the 23 patients with LFS/LFL tested positive for deleterious mutations in p53. Fifteen of the remaining sixteen were not found to carry the CHEK2* 1100delCmutation. These results indicate that CHEK2*1100delC is not a common cause of LFS, LFL, or PS-LFS in North American kindreds not carrying a TP53 mutation. Of note, two patients were found to carry p53* R72P, which is of unknown clinical significance. Lack of segregation of this allele in one of these kindreds provides strong evidence that the R72P allele is not disease-causing. While mutations in p53 account for a proportion of patients with LFS/LFL, future studies are needed to determine if other genes are responsible for LFS/LFL families not carrying germline p53 mutations.