Normal human serum contains 18 +/-5 mg/100 ml of C1 esterase inhibitor (alpha-2 neuraminoglycoprotein) as estimated by immunochemical means. Of 118 patients with hereditary angioneurotic edema, the sera of 80, from 42 kindred, contained a mean concentration of 3.15 mg/100 ml or 17.5% of normal. The mean serum concentration in 35 patients in 7 other kindred was 20 mg/100 ml or 111% of normal, an...

We present the successful application of C1 esterase inhibitor (C1INH) concentrate to a patient with clinical amniotic fluid embolism (AFE).

In a previous publication from this laboratory (1), radioactive diisopropylfluorophosphate (DFP32), a potent and irreversible esterase inhibitor, was shown to be a suitable label for granulocytes. Evidence was presented that the label is firmly attached to granulocytes; it does not modify the viability of the cells, and the label is not reutilized when the cells are degraded. Experiments design...

The degradation of atracurium and the formation of laudanosine was examined in vitro in both Sorensen buffer and human plasma using sensitive, specific high pressure liquid chromatographic assays to determine drug concentrations. At normal physiological pH and temperature, the degradation of atracurium was threefold more rapid in plasma than in buffer. Laudanosine is the major end-product of at...

Patients with hereditary angioedema (HAE) need a special concern during pregnancy. Although, the disease has a relatively benign course during pregnancy, maternal mortality has been reported. We present a HAE patient with recurrent attacks during pregnancy, but uncomplicated labor under C1INH concentrate prophylaxis.

Hereditary angioedema (HAE) is a rare autosomal dominant disorder caused by reduced activity of the C1 esterase inhibitor. Clinically, angioedema most often involves the upper extremities, face, neck and larynx. The most common cause of death is asphyxia related to laryngeal oedema. Attacks are triggered by many factors such as trauma, stress, infections and hormonal fluctuations. C1 esterase i...

A spectrophotometric method using benzoyl arginine ethyl ester (BAEE) is described for the assay of serum and plasma trypsin-like esterase activity. The method was used to find activities in serum and plasma (anticoagulated with oxalate and heparin) from 12 healthy males at 3 different reaction pH’s. The effects of calcium, oxalate, heparin, soybean trypsin inhibitor, ovomucoid, and heat on thi...

The cerebral cholinergic system is centrally involved in memory formation. Studies in rodents suggest that cholinergic stimulation may facilitate encoding of new information but may interfere with retrieval. We investigated the effect of cholinergic stimulation on encoding and retrieval of episodic memory in humans. We also tested whether the putative benefit of cholinergic stimulation on memor...

BACKGROUND Hereditary angioedema (HAE) is a rare disease caused by C1INH gene mutations, which leads to a deficiency or dysfunction of C1 inhibitor (C1 INH), resulting in recurrent episodes of severe and potentially life-threatening edema. OBJECTIVE To evaluate the efficacy and safety of repeat use of nanofiltered C1 esterase inhibitor (human) (C1 INH-nf) for the short-term treatment of HAE a...