Perianal Paget’s disease: case report and review of the literature

A peri-anal skin lesion, often eczema-like and with symptoms of pruritus, that does not resolve after classical local therapy should be biopsied. We present a case of peri-anal extramammary Paget’s disease (EMDP) and associated anal adenocarcinoma. Reviewing the literature, more than 30% of patients with EMDP present a second primary tumour in their past, present or future history. In Europe, the risk of developing a new primary tumour in patients with this condition is increased compared with the standard population. In cases of peri-anal Paget’s disease (PPD), specific histochemical markers allow us to differentiate between a primary and a secondary form, the secondary one is strongly associated with colorectal and anal tumours. We provide information about the most commonly suggested therapy for PPD with or without associated malignancy and about the recommended follow-up.     

原发性干燥综合征合并肺动脉高压的临床研究

目的探讨原发性干燥综合征(pSS)并发肺动脉高压(PAH)的发生率、临床特点及筛查方法。方法对61例临床确诊为pSS的患者行彩色多普勒超声心动检查,应用三尖瓣反流速度来估测肺动脉收缩压(PASP),依据PAH诊断标准将其分为PAH组(10例)和非PAH组(51例),收集2组的临床资料、实验室检查、胸CT扫描和超声心动的检测结果并进行比较。结果 (1)61例pSS患者中并发PAH者占16.4%,PAH组中PASP为31~64mmHg,平均(40±9)mmHg,其中4例无任何与PAH相关的临床表现,PAH的发生与患者年龄、性别、病程无关。(2)患者的主要症状为发热、干咳、胸闷或胸痛、心悸、进行性呼吸困难、雷诺现象、胸腔积液和肺间质病变,PAH组雷诺现象、心悸、肺间质病变的发生率高于非PAH组(P<0.05),PAH组的补体C3水平低于非PAH组(P<0.05)。(3)PAH组瓣膜受累达100%,其中2例仅伴有右心系统扩大,3例同时出现左心和右心系统扩大,PAH组左房内径、右房内径、右室内径、肺动脉内径高于非PAH组(P<0.05或P<0.01)。结论 pSS并发PAH并不少见,患者即使无相应临床症状,也应行超声心动筛查,这对PAH的临床早期诊断和治疗十分重要。     

Keratoacanthoma formation after skin grafting: A brief report and pathophysiological hypothesis

Keratoacanthoma formation after skin grafting is rare. We report the third case in the literature of multiple keratoacanthomas developed at both split‐thickness skin graft donor and recipient sites. We provide possible explanations for this poorly understood phenomenon and highlight its implications on treatment options.     

Antisynthetase syndrome: two cases presenting orofacial manifestations

Antisynthetase syndrome is a rare autoimmune disease that is characterised by inflammatory myositis, and interstitial lung disease or chronic arthropathy, or both. To the best of our knowledge, orofacial manifestations have not previously been reported. We present 2 patients with orofacial disease: one with aphthous-like oral ulceration and the other with hyposalivation.     

Successful management of steroid‐resistant vascular tumors associated with the Kasabach–Merritt phenomenon using sirolimus

Vascular tumors associated with Kasabach–Merritt phenomenon (KMP) are life‐threatening and the mortality is as high as 10–30%. Steroids are considered the primary choice for drug therapy. However, there are many steroid‐resistant cases. In the present study, analyzed data are presented to support the use of sirolimus in clinical practise for the treatment of corticosteroid‐resistant vascular tumors with KMP in eight infants between June 2015 and April 2017 in a single hospital. The time to initial response was 6.8 ± 2.7 days. The average stabilization time for the platelet count was 19.1 ± 8.5 days. At the time of publication, the average duration of sirolimus treatment was 14.1 ± 4.0 months, and the average time for sirolimus treatment as a single agent was 12.6 ± 4.2 months. The side‐effects were tolerable and included oral ulcer, fever, pain, skin rash and transient ascension of serum transaminase and cholesterol. Our study indicated that sirolimus therapy is an effective and safe method for the treatment of corticosteroid resistant vascular tumors associated with KMP in infants.     

Kinetics of the TL phenomenon in LiF:Mg irradiated to different dose of gamma radiation of 60Co using two different programs of deconvolution

In this work, the results on determination of kinetic parameters using the method of deconvolution of glow curves obtained from the LiF doped with Mg²⁺ ions, after having been irradiated to 5, 10 and 25 Gy of ⁶⁰Co gamma radiation, corresponding to the range of TL-vs-dose response linearity, are presented. The deconvolution is performed using two functions obtained by the method of asymptotic series and by the method based on the approximation of continued fractions. The glow curve of the studied material shows a dosimetric glow peak at 518 K and three peaks of very low intensity at 383, 423 and 463 K, all of a general-order kinetics. Kinetic parameters obtained with both methods showed that the values of kinetics, b, the energy of the traps, E, and the factor of frequency, s, do not differ significantly. However, the figure of merit, FOM, and the second derivative criteria indicate that the accuracy of the calculations with the second method improves considerably.