不同年龄食管裂孔疝相关危险因素和常见误诊疾病分析

目的分析不同年龄食管裂孔疝的相关危险因素,并对其常见误诊疾病进行分析。 方法回顾性分析2012年7月至2016年12月新疆维吾尔自治区人民医院收治的食管裂孔疝患者168例,其中92例<60岁（中青年组）,76例≥60岁（老年组）。采用多因素Logistic回归分析筛选不同年龄段食管裂孔疝相关危险因素。同时采用χ²检验比较中青年组与老年组食管裂孔疝患者常见误诊疾病比例差异。 结果体重指数、进食过饱、睡前进食是中青年食管裂孔疝的独立危险因素（P=0.013、0.030、0.015）;睡前进食、习惯性便秘、慢性咳嗽是老年食管裂孔疝的独立危险因素（P=0.024、0.018、0.039）。中青年组和老年组食管裂孔疝患者误诊为冠状动脉粥样硬化性心脏病的比例最高［21.7% （20/92）、28.9 （22/76）］,但两者差异无统计学意义;中青年组食管裂孔疝患者误诊为咽喉炎、支气管哮喘的比例高于老年组患者［19.6% （18/92）vs 9.2% （7/76）、13.0% （12/92）vs 6.6% （5/76）］,老年组食管裂孔疝患者误诊为慢性支气管炎的比例高于中青年组患者［10.9% （10/92）vs 21.1% （17/76）］,且差异均有统计学意义（χ²=10.332,P=0.001;χ²=7.911,P=0.005;χ²=9.614,P= 0.002）。 结论不同年龄食管裂孔疝的危险因素不同,常见误诊疾病也有差异,要根据年龄进行针对性防治,同时减少食管裂孔疝的误诊。     

Hormone replacement and contraceptive therapy in autoimmune diseases

Sex hormones, including estrogens, influence the immune system in a complex manner, playing an important role in the pathophysiology of autoimmune diseases. Estrogen receptors can be found in almost all cells, including those of the adaptive and innate immune systems. Depending on the relative preponderance and stimulation of different receptors in various tissues, estrogens may demonstrate pro-inflammatory or anti-inflammatory properties. Traditionally, exogenous estrogens were considered to have the potential of worsening the autoimmune processes and hence were avoided in patients with rheumatic diseases. Recent studies have demonstrated the efficacy of exogenous hormone therapies, either in the form of oral contraceptives or post-menopausal hormonal replacement therapy, in the majority of patients with rheumatic diseases. However, caution needs to be exercised as a higher incidence of mild to moderate flares of systemic lupus erythematosus was noted with hormonal replacement therapy in the large randomized controlled trial, HRT-SELENA. Exogenous estrogens can also increase the risk of thrombosis in patients with positive antiphospholipid antibodies and should be avoided in this subgroup. This review will discuss the current evidence on the efficacy, safety and impact of exogenous sex hormone therapies in patients with autoimmune rheumatic diseases.     

Mineral Oil-induced Sclerosing Lipogranuloma of the Penis

Sclerosing lipogranuloma of the penis results from injection of high viscosity fluid for the purpose of penile augmentation and may have devastating cosmetic and sexual function consequences. Although rare, sclerosing lipogranuloma should be considered in the differential diagnosis of subcutaneous induration or nodules of the male genitalia as it may mimic carcinoma and poses a diagnostic challenge in patients reluctant to admit to injection therapy. Surgical excision with penile reconstruction is the mainstay of treatment. The authors present a case of a 35-year-old Myanmarese man with a sclerosing lipogranuloma of the penis due to injection of mineral oil successfully managed with penile biopsy and excision with split-thickness skin graft phalloplasty and provide a review of the current literature.Sclerosing lipogranulomas of the penis resulting from injection of high-viscosity, oil-based solutions have been sporadically reported in the international literature, most commonly in Asian, Russian, and Eastern European patients.^1–3 A variety of oils has been injected into the penis with the intent of penile augmentation.^3–5 Most commonly presenting with a gross deformity of the penis, sclerosing lipogranulomas of the penis, also commonly referred to as penile paraffinomas, are often associated with emotional distress, impotence, painful intercourse, or the inability to perform sexual activities.^2,4 Surgical treatment may be technically challenging, but excision of the foreign material with appropriate phalloplasty may give satisfactory long-term results.In this report, the authors describe a case of sclerosing lipogranulomas of the penis secondary to injection of mineral oil managed by wide excision of the skin and subcutaneous tissue infiltrated by the foreign material along with skin graft phalloplasty as well as provide a review of the current literature.     

Interferon-alpha treatment of children and young adults with chronic hepatitis delta virus (HDV) infection

Objective: To determine the response of one-year interferon-alpha therapy in hepatitis delta virus (HDV) infection in children and young adults at a tertiary care hospital, Karachi, Pakistan. Study Design: An observational study. Place and Duration of Study: Sarwar Zuberi Liver Centre (SZLC), Medical Unit IV, Civil Hospital, Karachi / Dow University of Health Sciences (DUHS), from June 2009 to July 2010. Methodology: Paediatric patients (< 18 years age) and young adults (18-35 years) presenting were screened for hepatitis B virus (HBV) and HDV sero-markers. HDV anti-body positive by ELISA were further screened for hepatitis D ribonucleic acid (HDV-RNA) by real time PCR. HDV RNA PCR positive patients were treated with INF-a (children 6 MIU/m2/day and adults 5 MIU/day) for a period of one year. Patients were assessed monthly. Haematological parameters and ALT were monitored during treatment. Clinical progress (side effects) and negative HDV RNA were used as response criteria. Results: Overall 49 patients were HDV RNA positive (children: n=15, mean age 15±2.92 years adults: n=34, mean age 27±4 years). Eighty percent were male. Treatment was given to 25 patients (children: n=11, adults: n=14). HBV genotype D was the predominant in all HDV RNA positive patients (73%). Eighty percent (20/25) were HDV-RNA negative after one year of treatment, and remaining patients are still under treatment. Side effects were tolerated well and children continued regular activity. Haematological parameters were unremarkable. Children maintained their pre-treatment centile for height and weight (growth parameters). ALT levels were significantly decreased post-treatment. Conclusion: Conventional INF-a was safe in children with HDV infection in terms of side effects and growth parameters. Eighty percent were HDV-RNA negative one year after treatment. Further follow-up 2 years post-treatment will give conclusive results.     

Wilkie's syndrome

Wilkie's syndrome is a rare variant of small intestinal obstruction resulting from compression of third part of the duodenum by the superior mesenteric artery. A girl 15 years of age, presented with postprandial abdominal pain, bilious vomiting and weight loss. Radiological imaging revealed vascular duodenal compression which was relieved by timely surgical intervention.