Haemophilia care in China: achievements of a decade of World Federation of Hemophilia treatment centre twinning activities

Summary. The World Federation of Hemophilia (WFH) began its involvement in China in 1993, supporting Chinese haemophilia centre twinning programmes in 1997. Although only three Chinese centres were in a twinning relationship (Tianjin/Calgary 1997–2004, Guangzhou/Ottawa 2000–2006, Shanghai/Ottawa, Calgary 2002–2007), WFH helped establish a collaborative network of six Chinese centres (Tianjin, Guangzhou, Shanghai, Beijing, Hefei, Jinan) in 2004. These network centres, in collaboration with haemophilia patients, shared and coordinated several successful ongoing priority projects (Registry, Nursing, Laboratory Diagnosis, Physiotherapy and Prophylaxis). A number of physicians, nurses and technologists have received WFH fellowships for training internationally. They are becoming haemophilia‐care leaders and are key personnel working towards comprehensive care in China. National and regional workshops/conferences in addition to educational material development have been successful in reaching out to healthcare professionals and persons with haemophilia across the country. Key elements of success include the enthusiasm and commitment of the Chinese professionals and patient leaders in their desire to advance haemophilia care and their commitment to work collaboratively in a coordinated manner. They are beginning to gain national and international recognition and are launching outreach education and care programmes to cover other parts of China. WFH is now transitioning support in China from a twinning program to country programme (Global Alliance for Progress). Haemophilia work in China is still in the beginning, but there is now a solid foundation for future progress.     

INDUCTION OF ALCOHOL DEHYDROGENASE IN LIVER AND GASTRO-INTESTINAL TRACT

Experiments were designed to determine whether the increased rate of ethanol clearance previously demonstrated in alcoholics, in normal humans and in rats following prolonged alcohol ingestion is due to an increase in activity of enzymes involved in its metabolism. Alcohol was given in various doses to 56 rats, and alcohol dehydrogenase (ADH) and lactic dehydrogenase (LDH) assayed in liver, bile, colon, stomach and small intestine. Results were compared with 13 controls and rats given either actinomycin D or cycloheximide. Results of our experiments clearly show that the activity of ADH and LDH in liver, stomach and small intestine significantly increases with administration of single and repeated doses of alcohol, and the effect persists for a significant time. The effect of alcohol appears to be specific, as increased activity in enzymes not involved in its metabolism was not found. Increased ADH activity could be blocked by actinomycin and cycloheximide which suggests that alcohol induced de novo synthesis of this enzyme protein. The increase in activity of the rate-limiting enzyme, ADH, by its substrate ethanol, provides a plausible explanation for the observed tolerance in both rats and man conditioned by prior administration of alcohol. The increase in LDH, a terminal enzyme involved in ethanol metabolism, provides further indirect evidence that ethanol can be metabolized at a faster rate through the normal pathway. The finding of both ADH and LDH in significant amounts in stomach and small intestine in both normal and alcohol-fed rats suggests that ethanol can be metabolized to a significant extent in extrahepatic sites. This is contrary to the current view that alcohol is metabolized entirely by the liver, and that the multiple metabolic derangements in the alcoholic are the consequence of its obligatory metabolism in that organ.     

Advanced interventional pulmonology procedures: Training guidelines from the Thoracic Society of Australia and New Zealand

Training in interventional pulmonology procedures is increasing in popularity. However, the nature of training is difficult to define, particularly with respect to an adequate number of cases. These guidelines approach training not just from a modest number of supervised cases, but also from a range of educational and outcome targets which give a rounded approach to the issue. These include prerequisite skills from basic procedures, the place of simulated training, formal simulation testing, modest procedural outcome and side effect targets, audit presentations, ongoing reading, and hands‐on training expectations. All of this would still be under the supervision of an experienced trainer.     

Factors Affecting the Prognosis in Acute Renal Failure: A SURVEY OF 251 CASE

A survey has been made of 251 cases of acute renal failure (acutetubular necrosis). The over-all mortality was 44 per cent. Despiteincreasing expertise in the management of this complicationof surgical, medical, and obstetric disorders, considerabletechnical advances in dialysis, and increasing recognition andimproved management of incipient renal failure, there was nodecrease in mortality over the 11-year period of the survey.Four major factors have been identified as having an adverseinfluence on prognosis, namely age, infection, surgical gastrointestinallesions, and gastrointestinal haemorrhage. Failure to improvethe survival figures despite increasing experience in managementis due to a shift in the spectrum of severity of the patientsencountered, in that a larger number of older patients withmore critical illnesses are being referred to renal-failureunits. Although the acute renal failure may be controlled inthe earlier stages of the illness, many of these patients dielater of overwhelming infection or other complications.     

Influence of carbamoylation on some analytical properties of basic polypeptides

The effect of carbamoylation on the assay or identification of histones and polylysine was investigated. Incubation with sodium cyanate decreased the positive charge on these polypeptides as judged by changes in the binding of methyl orange or the electrophoretic mobility. Histones in chromatin appeared less accessible to carbamoylation than isolated histones. Carbamoylation of proteins under conditions in which there was little or no effect on the Lowry procedure could affect their assay by methods utilizing metachromasia with Coomassie Blue G. The Bradford assay has low sensitivity for HI histone and polylysine but this can be increased by preincubation with sodium cyanate. More extensive carbamoylation of polylysine caused decreased sensitivity which was the only response seen with core nucleosomal histones and bovine serum albumin when preincubated with sodium cyanate. It was concluded that the sensitivity for HI histone and polylysine in assays dependent on metachromasia with Coomassie Blue G may be changed by factors which decrease the positive charge on these polypeptides.     

RESPONSE OF PLASMA ALDOSTERONE TO FLUDROCORTISONE IN PRIMARY HYPERALDOSTERONISM AND OTHER FORMS OF HYPERTENSION

The response of plasma aldosterone to fludrocortisone administration (400 mug 12-hourly for 3 days) was studied in twenty-two patients with primary hyperaldosteronism. No difference was observed in the response between those patients with an adrenal adenoma and those with bilateral adrenocortical hyperplasia, there being no significant change in plasma aldosterone levels across the test period. No separation between the groups was seen when basal plasma renin concentration was related to the aldosterone level following fludrocortisone. It is concluded that the test is of little value in the pre-operative differentiation of these conditions. Twenty-three patients with no demonstrable cause for their hypertension and four with elevated levels of plasma deoxycorticosterone were similarly studied for comparison. These groups demonstrated a normal fall in plasma aldosterone levels following fludrocortisone.     

Hardness evaluation of penile prostheses

AIM: This work investigates the hardness and buckling force of penile prostheses to further understand the rigidity of penile prostheses before and after implantation. METHODS: Evaluated herein are four prosthetic samples (three inflatable, one semi-rigid), five real prostheses (one inflatable, four semi-rigid), and one prosthesis after implantation. The hardness is measured with a Shore Durimeter by pressing the tester's indentor to the surface of the specimen. A volunteer with inflatable prosthesis implantation is evaluated with respect to penile hardness versus various numbers of pumping. The buckling force of the prosthesis is also determined by a push-pull gauge and a specially designed sampling table. RESULTS: Results in this study demonstrate that although the inflatable prosthesis could only be pumped to a certain amount of hardness, hardness and buckling force correlate well with each other. After reaching the hardness limit, prostheses can even be pumped a further few times. However, continuous pumping only puts more tension on the prosthetic material without increasing hardness and could induce mechanical failure of the prosthesis. Results also indicate that the buckling force decreases with increasing length of the semirigid prostheses, and increases when the prosthesis has a larger diameter. CONCLUSION: This mechanical measurement of rigidity in penile prostheses could provide more information to clinicians about the penile prosthesis before surgical implantation, and to patients about prosthesis usage after implantation.