Clinical and demographical characteristics of primary progressive multiple sclerosis in Isfahan, Iran

Primary progressive multiple sclerosis (PPMS) is an uncommon form of multiple sclerosis (MS) in which the course of disease is progressive from onset. In a retrospective study amongst 1606 MS patients registered in Isfahan MS Society (IMSS) from April 2003 to 31 December 2005, 92 PPMS cases were identified. That means, the frequency of PPMS amongst all included MS patients would be 5.7% (95% CI: 6.7% and 4.7%). The mean expanded disability status scale (EDSS) for the group was 5.09 ± 1.3. The commonest mode of presentation was motor disturbance in 55 (59.8%), other modes of presentation were, vertigo in 15 (16.3%), visual problems in 12 (13%), sensory disturbances in six (6.5%), and diplopia in four (4.3%). The current existing symptoms were motor problems in all 92 (100%), cerebellar symptoms in 46 (50%), and cognitive impairment in only six (6.5%). Interestingly, two (2%) were affected by poliomyelitis during childhood and presenting symptom in both was limb weakness. Primary progressive form of MS is less common in Persian population and some of the rates observed in PPMS patients differ from those in other regions, these differences may be due to different ethnicity of Persian patients or to geographical differences.     

对原发性骨质疏松诊断的一些看法

原发性骨质疏松的诊断首先要除外继发性骨质疏松。后者包括的范围很广，内容也很多，需要广博的内科学基础和辨证的逻辑思维才能予以正确鉴别。骨密度检查只能作为一种参考，而不能作为诊断原发性骨质疏松的唯一依据。     

胆源性感染性休克兔血栓素、前列环素与血小板聚集率的变化

动态观察胆总管急性完全性梗阻并感染、继发休克兔的血浆血栓素(TXA_2)和前列环素(PGI_2)的稳定代谢产物 TXB_2和6-keto-PGF_(1α)含量及血小板聚集率的变化。结果,血浆 TXB_2呈进行性升高,20h 后(休克时)呈下降趋势,血浆6-keto-PGF_(1α)6h 升高,随后回降;血小板聚集率呈进行性下降。提示血栓素和前列环素、血小板聚集率的变化在重症急性胆管炎、胆源性感染性休克发病过程中起重要病理生理作用,与病情和预后转归有重要联系。     

Depression Scale (DEPS) among suicide attempters

The Depression Scale (DEPS), a new screening instrument for detecting depression in primary health care, was compared with the Hamilton Depression Scale (HDS) among 50 suicide attempters. Using the HDS as a gold standard, the positive and negative predictive values of the DEPS for the diagnosis of depression were 98% and 20%, respectively. The correlation between the total HDS scores and the total DEPS scores was 0.60. The DEPS may help general practitioners to detect depression among suicide attempters, but it should not be used to exclude depression.     

Sustained good glycaemic control in NIDDM patients by implementation of structured care in general practice: 2-year follow-up study

Summary In primary care it is difficult to treat the growing number of non-insulin-dependent diabetic (NIDDM) patients according to (inter)national guidelines. A prospective, controlled cohort study was designed to assess the intermediate term (2 years) effect of structured NIDDM care in general practice with and without ’diabetes service' support on glycaemic control, cardiovascular risk factors, general well-being and treatment satisfaction. The ’diabetes service', supervised by a diabetologist, included a patient registration system, consultation facilities of a dietitian and diabetes nurse educator, and protocolized blood glucose lowering therapy advice which included home blood glucose monitoring and insulin therapy. In the study group (SG; 22 general practices), 350 known NIDDM patients over 40 years of age (206 women; mean age 65.3 ± SD 11.9; diabetes duration 5.9 ± 5.4 years) were followed for 2 years. The control group (CG; 6 general practices) consisted of 68 patients (28 women; age 64.6 ± 10.3; diabetes duration 6.3 ± 6.4 years). Mean HbA_{1 c} (reference 4.3–6.1 %) fell from 7.4 to 7.0 % in SG and rose from 7.4 to 7.6 % in CG during follow-up (p = 0.004). The percentage of patients with poor control (HbA_{1 c} > 8.5 %) shifted from 21.4 to 11.7 % in SG, but from 23.5 to 27.9 % in CG (p = 0.008). Good control (HbA_{1 c} < 7.0 %) was achieved in 54.3 % (SG; at entry 43.4 %) and 44.1 % (CG; at entry 54.4 %) (p = 0.013). Insulin therapy was started in 29.7 % (SG) and 8.8 % (CG) of the patients (p = 0.000) with low risk of severe hypoglycaemia (0.019/patient year). Mean levels of total and HDL-cholesterol (SG), triglycerides (SG) and diastolic blood pressure (SG + CG) and the percentage of smokers (SG) declined significantly, but the prevalence of these risk factors remained high. General well-being (SG) did not change during intensified therapy. Treatment satisfaction (SG) tended to improve. Implementation of structured care, including education and therapeutic advice, results in sustained good glycaemic control in the majority of NIDDM patients in primary care, with low risk of hypoglycaemia. Lowering cardiovascular risk requires more than reporting results and referral to guidelines. [Diabetologia (1997) 40: 1334–1340] Received: 5 February 1997 and in revised form: 22 May 1997     

原发性贲门失弛缓症的外科治疗(附10例报告)

作者对10例原发性贲门失弛级症进行手术治疗．采用Heller术8例，食管下段贲门切除术3例，其中再手术3例，附加幽门成形术4例。本组资料表明，Olsen分期Ⅰ～Ⅲ期行Heller术效果满意，不需附加手术，Ⅲ期需行食管下段贲门切除术。作者对手术方法、手术径路、附加手术与手术效果的关系等进行探讨。     

Primary Adenocarcinoma in the Urethrorectal Fistula

A 60-year-old Japanese man was hospitalized because of urinary leakage from the anus on October 3, 1994. Retrograde urethrography detected a fistula between the bulbous urethra and the rectum. Urethrocystoscopy revealed a tumor on the urethrorectal fistula. Tumor biopsy showed a well differentiated adenocarcinoma. Cystourethrectomy with fistulectomy, and ileal conduit urinary diversion were performed. Pathological examination revealed primary adenocarcinoma in the fistula with invasion to the prostatic urethra and bladder wall. The patient showed no evidence of a recurrence as of August, 1996.     

Bile duct injury repair: when? what? who?

Laparoscopic cholecystectomy is associated with a two-to-four times higher risk of bile duct injury (BDI) than open cholecystectomy. BDI can lead to significant morbidity and even mortality. The first priority in BDI is to control peritoneal and biliary sepsis and to convert an acute BDI to a controlled external biliary fistula (EBF) — this can be achieved by endoscopic and/ or radiological intervention in most cases. This should be followed by assessment of the extent of injury — both biliary and vascular. Immediate management of BDI recognized during cholecystectomy depends on the type of injury, the condition of the patient, and the experience of the surgeon. For BDI recognized after cholecystectomy, early repair is not recommended, as the results are poor. The EBF may evolve into a benign biliary stricture (BBS), which should be electively repaired by a Roux-en-Y hepatico-jejunostomy. The use of an endoscopic stent as definitive management of BDI is not recommended. Long-term follow-up is essential after the repair of a BBS, as recurrence can occur several years after repair. Recurrent BBS is best treated with endoscopic balloon dilatation. Excellent early and long-term results can be obtained in specialized units at tertiary care referral centers.     

Cerebral primitive neuro-ectodermal tumour following treatment of a unilateral retinoblastoma

Summary Cerebral primitive neuro-ectodermal tumour (PNET) occurring as a second primary malignancy in childhood is exceedingly rare. We present a 7-year-old boy who developed a proven supratentorial PNET five years after enucleation and radio-/chemotherapy for a sporadic, unilateral retinoblastoma with optic nerve invasion. The association with this malignant eye disease as well as the effect of irradiation and multi-agent chemotherapy on second tumour induction are evaluated.