1-deamino-8-d-arginine vasopressin: a new pharmacological approach to the management of haemophilia and von willebrand's disease

Zollinger-Ellison syndrome.

ASH ISTH NHF WFH 2021 guidelines on the management of von Willebrand disease.

https://onlinelibrary.wiley.com/doi/pdf/10.1046/j.1538-7836.2003.00230.x

Intensive exposure to factor VIII is a risk factor for inhibitor development in mild hemophilia A.

Inhibitors are an uncommon complication of mild haemophilia, occurring in 3-13% of patients and usually arising during adulthood. The risk of inhibitor development in this group appears to be associated with relatively few high-risk factor VIII genotypes clustered in the A2 and C2 domains, especially the Arg593-Cys and the Trp2229-Cys mutations. Kindreds with these mutations have an inhibitor incidence of up to 40%. These mutations may induce a stable conformational change in the factor VIII molecule rendering it antigenically distinct from wild-type factor VIII. Inhibitors in mild haemophilia usually cross-react with endogenous factor VIII reducing the basal VIIIC to < 0.01 IU/ml, and causing spontaneous bleeding. This bleeding is sometimes severe and life-threatening, two-thirds of patients developing a pattern of soft tissue, gastrointestinal (GI) and urinogenital bleeding reminiscent of acquired haemophilia. Bleeding has been treated with human and porcine factor VIII, bypass therapy and DDAVP. Recombinant factor VIIa and DDAVP have the advantage that they do not induce an anamnestic rise in inhibitor titre. About 60% of these inhibitors disappear in the remainder over a median of 9 months. Few of these inhibitors recur, suggesting that most such patients have become tolerant. The inhibitors persist long-term and remain troublesome in about 40% of patients. The limited data available on immune tolerance induction in this group indicate a generally poor response to this approach. Two of nine achieved tolerance, with a partial response in a further four. Inhibitors are an uncommon but life-threatening complication of haemophilia. This complication should be considered when selecting the treatment modality for patients with a family history of inhibitors, and DDAVP used whenever possible.

Factor VIII inhibitors in mild and moderate‐severity haemophilia A

Gastric secretion in duodenal ulcer, with particular reference to the diagnosis of Zollinger-Ellison syndrome.

Summary. Two patients with mild haemophilia developed inhibitors of Factor VIII during replacement therapy to cover major surgery. The inhibitors inactivated not only transfused antihaemophilic factor but also the patient's own Factor VIII and temporarily converted them to severe haemophiliacs. The activity of the inhibitors rapidly declined, possibly as a result of steroid therapy, and both patients eventually regained their previously mild haemophilic state.

Inhibitor of factor viii in mild haemophilia

A controlled single-blind trial has been carried out to determine the value of long-term anticholinergic therapy in duodenal ulcer. Of 106 male patients with symptomatic and radiologically proven duodenal ulcer admitted to the trial, 91 completed the study. Patients were divided randomly into three groups. They received either glycopyrronium, or 1-hyoscyamine in a sustained-release form, or inert tablets for one year. Progress was judged on the basis of frequency and severity of symptoms, monthly assessments by patients, antacid consumption, and radiology. By all criteria, glycopyrronium and 1-hyoscyamine were not significantly superior to placebo. Symptomatic improvement, both subjective and objective, occurred in approximately 80% of all patients during the year of observation; there was no significant difference between the groups.

https://gut.bmj.com/content/gutjnl/11/7/559.full.pdf

A controlled trial of glycopyrronium and l-hyoscyamine in the long-term treatment of duodenal ulcer.

Ninety patients with duodenal ulcer, divided randomly into three groups, were treated continuously for one year with either glycopyrronium, 1-hyoscyamine (as a sustained-release preparation) or inert tablets. Dosage with active tablets was so adjusted that the patient experienced definite but tolerable side-effects. Basal and maximal gastric acid secretion were measured immediately before and one week after cessation of treatment. There was no significant change in the means of these measurements in patients who received placebo or 1-hyoscyamine. In those given glycopyrronium, mean basal output was significantly increased. Mean maximal acid output in this group fell, but not significantly. Individual measurements of maximal acid output showed quite marked fluctuations in all groups. It is concluded that spontaneous changes in parietal cell mass may occur in patients with duodenal ulcer, and that prolonged anticholinergic therapy does not reduce parietal cell mass.

https://gut.bmj.com/content/gutjnl/10/10/774.full.pdf

Gastric acid secretion in patients with duodenal ulcer treated for one year with anticholinergic drugs

Coagulation studies were performed on haemophilic patients during a controlled trial of oral contraceptive therapy. Treatment with a combined oestrogen-progestogen preparation was associated with a significant rise in the factor VII-X complex. This change was not observed during treatment with progestogen alone. The highest levels of factor IX and fibrinogen and the lowest levels of factor X were observed during the first period of the trial but these effects were independent of the type of treatment. The results suggest that the effects of oral contraceptives on blood coagulation in haemophilic males are similar to those observed in normal females and that the changes are due to the oestrogenic component.

https://jcp.bmj.com/content/jclinpath/24/1/23.full.pdf

Coagulation studies in haemophilic patients taking oral contraceptives

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