An analysis of the teaching of intravenous self-administration in patients with hereditary angio-oedema.

BACKGROUND: Hereditary angio-oedema (HAE), C1 inhibitor HAE (C1-INH-HAE) type I and C1-INH-HAE type II, are inherited disorders characterised by potentially life-threatening recurrent swellings, caused by a deficiency of C1 inhibitor. Management includes attack treatment or prevention using prophylaxis/routine prevention. AIM: To evaluate the success of self-administration training as part of a home care programme for treatment of HAE using intravenous C1 inhibitor. METHODS: In total, 18 patients (7 men, 11 women; aged 18-72 years) were trained to self-administer a plasma-derived C1 inhibitor concentrate for acute treatment or routine prevention of HAE attacks. The number of training sessions needed to learn intravenous self-administration, delay in time to treatment and reduction in attack frequency (per month) were evaluated after completion of the training. RESULTS: All patients successfully completed training. The median number of training sessions required to be capable of unassisted/independent self-injection was 5 (range 2-30). Time to treatment was reduced from a median of 4.5 h (270 min) by medical professionals to 15 min by patients after self-administration training). Using the treatment as routine prevention resulted in a reduction of median frequency of attacks from 8 to 0.5 attacks/month. CONCLUSION: C1 inhibitor self-administration for the treatment of HAE allows patients to quickly treat attacks at home, potentially reducing attack severity. The results also demonstrate the benefit of self-administered routine prevention therapy in a real-world patient population. Self-administered therapy potentially allows patients to gain greater control over their attacks, resulting in a reduction in healthcare utilization.

[Progressive necrotizing otitis externa in an elderly diabetic with cranial nerve paralysis]. / Progrediente nekrotisierende Otitis externa des älteren Diabetikers mit Hirnnervenparesen.

On the basis of an own observation the rare clinical picture of an external otitis with pareses of the brain nerves is casuistically described. Up to now it was observed only in elder persons with diabetes mellitus. The pathogenic agent is always Pseudomonas aeruginosa. The inflammatory process, issuing from the external auditory passage and under circumvention of the tympanum, spreads to the skull base and according to the localisation causes adequate pareses of the brain nerves. Early operation and aimed antibiotic therapy is the therapy of choice.

[Demonstration of ECG-triggered, two-dimensional ultrasonic cross-sectional pictures of the heart]. / Darstellung Ekg-getriggerter zweidimensionaler Ultraschallschnittbilder des Herzens.

It is reported on experiences with the demonstration of twodimensial cut pictures of the heart by means of the ultrasound device "Vidoson". To receive temporarily exactly defined picture, a trigger mechanism for releasing the camera dependent on the R-lead of the ECG is described as supplementation for the device. The advantages and possibilities of such defined cut pictures of the heart are shown.

[Blood circulation disturbances of the upper extremities as a principle symptom of pseudoxanthoma elasticum]. / Durchblutungsstörungen der oberen Extremitäten als Leitsymptom bei Pseudoxanthoma elasticum

The pseudoxanthoma elasticum is a rare disease of the connective tissue which coincides with typical symptoms of the skin, eyes and vessels. It is reported on a 37-year-old female patient that showed disturbances of the blood circulation in the upper extremities as a main symptom.

[Vasculitis nodularis with participation of inner organs]. / Vasculitis nodularis mit Beteiligung innerer Organe

The vasculitis nodularis belongs to the group of the allergic-hyperergic vascular diseases of the skin, which above all the small veins are affected at. The participation of internal organs is unusual. It is reported on a 31-year-old Syrian patient in whom a participation of the liver in form of a granulomatous hepatitis and of the kidneys appeared. A hypertension might be attributed to the participation of the kidneys. The incorporation of a thrombosis of the central vein of the right eye in this disease is probable. The main symptoms of the disease were nodes of the size of a bean occurring in batches, lying subcutaneously, and were paraclinical signs for inflammation. The permanently increased absorption-antistreptolysin titre spoke for an allergic-hyperergic vascular process induced by streptococci.