History of haemophilia

Summary.  Haemophilia, an ancient disease, now has sophisticated methods for diagnosis and treatment. The genetically missing factors can now be supplied by fractionation of human-derived (HD) plasma or with recombinant technology (r). Making therapeutic choices is complicated by past transfusion-transmitted diseases. HD and r products now have similar safety profiles. Several diseases have only HD products for treatment. These products remain important in our treatment armamentarium.     

Thrombophilic risk factors and unusual clinical features in three Italian CADASIL patients

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a genetically transmitted cerebrovascular disease. Typically, the first clinical manifestation is migraine and the full clinical spectrum of the disease with recurrent strokes of the subcortical type, cognitive, and mood disorders is seen during the fourth and fifth decades of life. Vascular risk factors are usually absent in CADASIL patients and the diagnosis of the disease is particularly suspected in young adults with cerebrovascular events of unknown cause, diffuse leukoencephalopathy on computed tomography or magnetic resonance imaging, and a history of cerebrovascular diseases or dementia in many family members. We describe three Italian CADASIL patients who presented to medical attention for cerebrovascular events occurred after the age of 55 and had, in addition to hypertension and hyperlipidemia, thrombophilic risk factors such as hyperhomocysteinemia, elevated levels of lipoprotein(a), and antiphospholipid antibodies. Symptoms possibly related to cortical involvement, such as dysphasia and visual field deficits, were reported by two of these patients. We conclude that a diagnosis of CADASIL should not be disregarded in patients with vascular risk factors and presenting with symptoms not immediately referable to subcortical damage at ages more advanced than commonly reported.     

影响下尿路尿动力学检查准确性的因素及护理措施

笔者通过对270例患者进行尿动力学检查,认为环境、病人的配合程度、检查体位、导管的型号、膀胱灌注情况及护士操作技术、服务态度对尿动力学检查有一定的影响。针对影响因素采取护理措施:积极创造良好的检查环境,加强对患者的宣教与沟通,正确放置体位和选择导管、冲洗液的速度,操作护士熟练的技术和人性化服务,是获得准确检查结果的关键。     

Endocrinology: Predictive value of serum oestradiol concentrations and oocyte number in severe ovarian hyperstimulation syndrome

Ovarian hyperstimulation syndrome (OHSS) is a serious complicationof gonadotrophin usage but it is difficult to accurately predictits occurrence. Previous investigators have identified the combinationof high oestradiol concentrations and oocyte number as beingpredictive in 80% of cases. In this study we sought to identifythe incidence of severe OHSS in patients with high oestradiolconcentrations and large numbers of oocytes and to evaluatethe importance of pregnancy in the development of OHSS. Between1990 and 1993, we studied 139 cycles using two assisted reproductivetechniques [oocyte donor, n =72; in-vitro fertilization (IVF),n = 67] in which either oestradiol (>4000 pg/ml), oocytenumber (>25), or both were elevated. OHSS was diagnosed bystandard criteria. There were no cases of severe OHSS in theoocyte donor group and six in the IVF group. Among 10 patientswith oestradiol concentration >6000 pg/ml and >30 oocytes,only one had OHSS (10%). The relative risk of OHSS with pregnancywas 12 (confidence interval 2.18–66.14). We conclude thatthe risk of OHSS even at high levels of stimulation is lowerthan previously believed. Secondly, donors have a very low riskof OHSS, probably because of the absence of pregnancy. As such,cryopreservation of all oocytes in IVF cycles is a reasonablealternative to cycle cancellation or use of adjunctive medication.     

Medical education for minorities at a US medical school

The University of Washington School of Medicine (UWSM) has initiated new efforts to build a regional minority applicant pool and to expand its educational programmes to accommodate students from disadvantaged backgrounds. Specific interventions include: establishment of medical career planner position to coordinate region-wide outreach; pre-entry education; and support activities once enrolled. This study describes specific services and presents sociodemographic and performance data on 56 minority and 280 majority students entering the UWSM between 1981 and 1985. Economic status and educational background of minority students were significantly below that of majority students, several flexible academic policies enabled most students to achieve mastery in courses and to progress through the curriculum. The educational data base utilized in this study, and those at other institutions, can assume important roles in the identification of problem areas in the education of disadvantaged students and in evaluation of the interventions attempted.     

Surgical treatment for carcinoma of the papilla of vater

Eighty of 89 patients who underwent radical resection (resectability 89.9%) for carcinoma of the papilla of Vater between 1976 and 1992 were retrospectively reviewed. Seventy-three patients underwent pancreaticoduodenectomy (PD) and 7 underwent pylorus-preserving pancreaticoduodenectomy (PPPD). The postoperative mortality rate was only 3.8% (3 patients). The 3- and 5-year survival rates were 63.6% and 57.4%, respectively. Important factors influencing long-term survival were Stage (clinical stage = Stage), microscopic lymph node metastasis (n), duodenal wall invasion (d), vascular invasion (v), and the epithelium of origin. Early carcinoma of the papilla of Vater is defined as tumor in which invasion is limited within the papilla of Vater; in particular, carcinomatous invasion is within the muscle of Oddi (d₀) with n₀. PD and/or PPPD with radical lymph node dissection should be performed for carcinoma of the papilla of Vater, as these procedures can be performed with low morbidity and mortality.     

Clinical characteristics of rapidly progressive leuko-araiosis

Introduction – 38 patients found to have either pure leuko-araiosis (LA) or LA combined with infarction(s) on computer tomography (CT) in 1989 were re-examined in 1992 in order to evaluate the progression of LA. The follow-up period averaged 3.2 years. Material and methods - The clinical and radiological data on patients in 1989 were collected from hospital records and re-evaluated. The patients were re-examined clinically (including 24 hour ambulatory blood pressure measurement), and neuroradiologically (CT) in 1992 for this study. Results – 11 (29%) patients were found to have significant (rapid) progression of the extent of LA on CT during the follow-up. At baseline, there was no significant difference in the mean number of brain infarctions between the groups with progressing (prLA) and non-progressing LA (nprLA) or between the number of cortical and central infarctions within these groups. At follow-up, the total number of infarctions had increased significantly in both groups, but it was mostly because of the increase in cortical infarctions in the prLA group (p = 0.043) and, conversely, the central ones in the nprLA group (p = 0.011). prLA was found to be related to heart failure (82% vs 37%, p = 0.029) and atrial fibrillation (55% vs 19%, p = 0.047), whereas nprLA was strongly associated with a sudden onset of symptoms (78% vs prLA 18%, p = 0.001) like a-true brain infarction. Other clinical factors, including mean blood pressure and heart rate, did not clearly differentiate between the groups. Conclusion - The results suggest that there are different subgroups of patients with LA associated with various vascular factors. The occurrence of LA is not related to the distribution of infarctions. The progression of LA is not related to the number of brain infarctions or to the simultaneous increase of infarctions on CT.     

Interactions between epidermal growth factor-mediated autocrine regulation and linoleic acid-stimulated growth of a human prostate cancer cell line.

Human prostate cancer (PC) cell lines possess epidermal growth factor (EGF) receptors and secrete EGF-related polypeptides. We used an EGF receptor-blocking antibody (anti-EGF.R) to demonstrate a functional autocrine loop, as well as the interaction between this and the effects of linoleic acid (LA), an omega-6 fatty acid, on PC cell growth. The anti-EGF.R competed effectively with [125I]EGF for receptors on DU145 PC cells, and on a high-passage DU145 variant (DU145M); when added to the culture medium, it suppressed both DU145 and DU145M cell growth in a dose-dependent manner. LA, a precursor for eicosanoid synthesis, had little effect on DU145 cell growth rate but stimulated DU145M growth in a concentration-related manner over a range of 0.25-2.0 micrograms/ml. anti-EGF.R (10(-9) M) caused suppression of LA-stimulated growth of DU145M cells in serum-free medium, which was prevented by the addition of 2 nM EGF. We conclude that an EGF.R-mediated autocrine loop is involved in PC cell growth regulation and that at least one site of action may be the synthesis of eicosanoids from their LA precursor.     

Wives prefer reading: Stress and female sexuality

A clinical discussion is presented of sources of sexual stress for women and how they might interfere with healthy sexual functioning. Several therapeutic techniques to reduce sexual stress are described and clinical case examples are given to clarify their use in clinical practice.     

Adolescents who make suicide attempts repeatedly

Four hundred and six consecutive suicide attempts made by 15-19 year-old adolescents in 1973-1982 were examined. Two hundred and twenty-six cases were first, 180 repeated suicide attempts. Repeaters came from poorer social situations and less well integrated families than first-timers. Repeaters had many adapting problems typical of personality disorders and had previously been in psychiatric treatment. Their level of adaptive functioning (GAS) and overall functioning (DSM III: s axis V) were poorer than within first-timers. They were not psychotic more often than first-timers and did not make more difficult suicide attempts. Their psychiatric after-care was more intensive and prognosis with regard to subsequent suicide was poorer than within first-timers. At the end of follow-up time (approx. 5 years) 1% of first-timers and 4% of repeaters had committed suicide. Observed-expected ratio for first-timers was 0.58 and for repeaters 1.73.