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Abstracts of papers in this issue
--
Uroporphyrinogen
I synthase Sty-reductase
Pancreatic
beta-cell
adenomas
Continued
In a patient with hypoglycemia, detection and satisfactory removal of multiple insulin-secreting pancreatic tumors at the time of surgery were facilitated by the use of a feedbackcontrolled dextrose infusion system. Features of this instrument allow continuous monitoring of blood glucose as well as provision of needed quantities of dextrose to maintain euglycemia, by means of a computerized program. In addition to preventing hypoglycemia In the patient, this instrument indicated that the initial attempt at removal of the source of hyperinsulinism was inadequate and that further resection of the pancreas was indicated.
Karam JH, Lorenzi M. Young CW, Bums AD, Prosser PR, Grodsky GM, Galante M. Forsham PH: Programmed glucose infusion during surgical management of a patient with multiple insulinomas. Am J Med 66: 675-680, 1979.
lnsulinoma
of blood glucose
Biostator GCIIS
MonHoring
The natural history of acute intermittent porphyria (AIP) suggests that natural Steroids Strongly influence clinical expression of the disease. We examined So-reductive transformation of hormones in 21 subjects with clinlcally expressed AIP. 18 with clinically latent porphyria, and 20 normal subjects. Ratios of 5p to 5ru metabolites of [4-‘%]testosterone were substantially higher in those with clinically expressed AIP (3.4 f 0.5 mean f SE, range 1.1 to 9.0) as compared to those with latent AIP (1.0 & 0.1, range 0.5 to 2.3)or normal subjects (1.1 f 0.1. range 0.5 to 2.0). Similarly, per cent 5cu-metabolism of [ i,2-3H]-l 1-hydroxy-androstenedione was significantly lower in expressed AIP (46 f 3.6 per cent) as compared with that in latent AIP (71 f 2.4 per cent) or in normal subjects (73 f 2.3 per cent). Thus, although the groups with express& ar+d latent AlP were homogeneous with respect to eryfhrocyte UROS deficiency, impaired steroid %x-reduction was found only in the group with a history of exacerbations, supporting the view that Subtle endocrine abnormalities are involved in clinical expression Of AIP.
HL, Sassa S, Kappas A: Studies in porphyria. VIII. Relationship of the 5cY-reductive metabolism of steroid hormones to clinical expression Of the genetic defect in acute intermittent porphyria. Am J Med 66: 644-650, 1979.
AndersonKE, Bra&w
Acute intermittent porphyria
bypass grafting
RA, McIntosh with coronary
in the management 1979.
Coronary artery disease Angina pectoris
HD: The aortocoronary bypass grafting artery disease. Am J Med 66: 651-666,
revascularization
of
angioedema
Dana201 Complement
of hereditary
an-
on page A82
Danazol, an attenuated androgen, was shown to be effective in the prophylaxis of attacks of hereditary angioedema. Four adult patients with a history of monthly attacks had a cumulative total of six episodes during 60 patient months of treatment. Danazol appeared to act by increasing the level of the Cl esterase inhibitor through a mechanism which is still under investigation.
of attacks
Androgens
Rothbach C, Green RL. Levine MI, Fireman P: Prophylaxis gioedema. Am J Med 66: 681-683, 1979.
Cl esterase inhibitor
Hereditary
There is widespread agreement that aortocoronary bypass grafting generally lessens the symptoms and functional limitations of patients with angina pectoris. Evidence for prolongation Of fife or prevention of myocardial infarction, arrhythmias and ventricular dysfunction is inconCfUSiVe. Harmful effects associated with surgical management of coronary artery disease can be documented in terms of operative mortality, perioperative myocardial infarction, graft ocCfuSiOn and progression of occlusive disease in the native circulation. In this review of publish& experience, the accomplishments and the limitations of myocardial revascularization are considered in various clinical settings. Critical assessment of evolving information leads to the conclusion that widespread application of this procedure beyond the alleviation of symptoms refractory to medical therapy is not justified by present data.
Buccino patients
Myocardial
Aortocoronary
Uroporphyrinogen
I synthase Sty-reductase
Pancreatic
beta-cell
adenomas
Continued
In a patient with hypoglycemia, detection and satisfactory removal of multiple insulin-secreting pancreatic tumors at the time of surgery were facilitated by the use of a feedbackcontrolled dextrose infusion system. Features of this instrument allow continuous monitoring of blood glucose as well as provision of needed quantities of dextrose to maintain euglycemia, by means of a computerized program. In addition to preventing hypoglycemia In the patient, this instrument indicated that the initial attempt at removal of the source of hyperinsulinism was inadequate and that further resection of the pancreas was indicated.
Karam JH, Lorenzi M. Young CW, Bums AD, Prosser PR, Grodsky GM, Galante M. Forsham PH: Programmed glucose infusion during surgical management of a patient with multiple insulinomas. Am J Med 66: 675-680, 1979.
lnsulinoma
of blood glucose
Biostator GCIIS
MonHoring
The natural history of acute intermittent porphyria (AIP) suggests that natural Steroids Strongly influence clinical expression of the disease. We examined So-reductive transformation of hormones in 21 subjects with clinlcally expressed AIP. 18 with clinically latent porphyria, and 20 normal subjects. Ratios of 5p to 5ru metabolites of [4-‘%]testosterone were substantially higher in those with clinically expressed AIP (3.4 f 0.5 mean f SE, range 1.1 to 9.0) as compared to those with latent AIP (1.0 & 0.1, range 0.5 to 2.3)or normal subjects (1.1 f 0.1. range 0.5 to 2.0). Similarly, per cent 5cu-metabolism of [ i,2-3H]-l 1-hydroxy-androstenedione was significantly lower in expressed AIP (46 f 3.6 per cent) as compared with that in latent AIP (71 f 2.4 per cent) or in normal subjects (73 f 2.3 per cent). Thus, although the groups with express& ar+d latent AlP were homogeneous with respect to eryfhrocyte UROS deficiency, impaired steroid %x-reduction was found only in the group with a history of exacerbations, supporting the view that Subtle endocrine abnormalities are involved in clinical expression Of AIP.
HL, Sassa S, Kappas A: Studies in porphyria. VIII. Relationship of the 5cY-reductive metabolism of steroid hormones to clinical expression Of the genetic defect in acute intermittent porphyria. Am J Med 66: 644-650, 1979.
AndersonKE, Bra&w
Acute intermittent porphyria
bypass grafting
RA, McIntosh with coronary
in the management 1979.
Coronary artery disease Angina pectoris
HD: The aortocoronary bypass grafting artery disease. Am J Med 66: 651-666,
revascularization
of
angioedema
Dana201 Complement
of hereditary
an-
on page A82
Danazol, an attenuated androgen, was shown to be effective in the prophylaxis of attacks of hereditary angioedema. Four adult patients with a history of monthly attacks had a cumulative total of six episodes during 60 patient months of treatment. Danazol appeared to act by increasing the level of the Cl esterase inhibitor through a mechanism which is still under investigation.
of attacks
Androgens
Rothbach C, Green RL. Levine MI, Fireman P: Prophylaxis gioedema. Am J Med 66: 681-683, 1979.
Cl esterase inhibitor
Hereditary
There is widespread agreement that aortocoronary bypass grafting generally lessens the symptoms and functional limitations of patients with angina pectoris. Evidence for prolongation Of fife or prevention of myocardial infarction, arrhythmias and ventricular dysfunction is inconCfUSiVe. Harmful effects associated with surgical management of coronary artery disease can be documented in terms of operative mortality, perioperative myocardial infarction, graft ocCfuSiOn and progression of occlusive disease in the native circulation. In this review of publish& experience, the accomplishments and the limitations of myocardial revascularization are considered in various clinical settings. Critical assessment of evolving information leads to the conclusion that widespread application of this procedure beyond the alleviation of symptoms refractory to medical therapy is not justified by present data.
Buccino patients
Myocardial
Aortocoronary