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The case of a 37-year-old male suffering from Gottron's acrogeria and cutaneous sarcoidosis is presented together with a thorough review of the literature. So far about 40 cases of Gottron's acrogeria have been described. An association of sarcoidosis and acrogeria has not been observed to date.

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Intravenous administration of pentamidine is known to cause long-QT syndrome (Torsade de pointes tachycardias and large QT prolongation) in rare cases and to cause small QT prolongation regularly. A similar pattern is seen with other drugs known to cause a long-QT syndrome. Pentamidine aerosol prophylaxis is commonly used to prevent Pneumocystis carinii pneumonia in HIV-infected persons. The goal of this study was to clarify whether pentamidine aerosol prophylaxis induces QT prolongation. We examined 100 patients receiving pentamidine aerosol prophylaxis at a rather high dose (300 mg biweekly) for at least 1 month (range 1-24) by determining the QT interval corrected for heart rate (QTc), blind for treatment. In a cross-sectional study, QTc was not different in 50 HIV-infected patients with chronic pentamidine aerosol prophylaxis (413 ms), 50 similar HIV-infected patients without pentamidine (407 ms), and 50 similar patients without HIV-infection and without pentamidine (407 ms). In a longitudinal study in another 50 HIV-infected patients, QTc was the same before (414 ms) and on long-term (median 9-month) pentamidine aerosol prophylaxis (414 ms). In contrast to the case with intravenous pentamidine, we found no QT prolongation and thereby no risk of long-QT syndrome with pentamidine aerosol prophylaxis.

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In a family with synovial chondromatosis affecting at least three, presumably five members, the articular disorder was combined with dwarfism. The persons with joint disorders were below the third percentile in body height, but family members with normal articular function had normal height. We believe this to be the first description of a combination of synovial chondromatosis with genetically caused dwarfism.

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A 20-year-old woman developed multifocal bone pain. Diagnostic radiology revealed thickened periosteum, and syphilis serology was positive (T. pallidum haemagglutination test 1:5120). She also had discrete signs of congenital syphilis, with retinitis pigmentosa and Hutchinson's incisors. Skeletal scintigraphy demonstrated increased uptake even in radiologically unremarkable bones. The bone pain decreased after four-week administration of 1 M units of penicillin G daily. Scintigraphy one year later showed regression of the increased uptake, skeletal radiology did not reveal significant changes. This case shows that late manifestations of congenital syphilis still occur today. The stigmata of such an infection can be minute and easily missed. Skeletal scintigraphy is better for demonstrating active bone involvement than conventional radiology.

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False positive tests for autonomic neuropathy in HIV-infection and AIDS? Case-control-study of heart rate variation in 62 cases Autonomic neuropathy is said to be common in AIDS. In a cross section study we tested heart rate variability, a simple and sensitive test, in relation to disease severity. The expiratory-inspiratory RR-interval-ratio (RRR) was normal in HIV-infected clinically healthy persons (mean 1.45; n = 10), but progressively lowered in LAS (1.24; n = 12), ARC (1.20; n = 7), AIDS (1.13; n = 20), AIDS with pneumocystis carinii pneumonia (1.07; n = 7) and patients who died within the four month study (1.06; n = 6). The progressive decrease of heart rate variability (from 45% to 6% for group means) is in accord with previous reports. - In a simultaneous case-control study we compared RRR of these 62 patients with those of 62 not HIV infected patients matched for age, sex, resting heart rate and presence of pneumonia. Neither in the six subgroups nor in the total group we found a difference between HIV infected patients and their controls (1.20 +/- 0.18 SD and 1.22 +/- 0.17, respectively; 62 pairs). The decrease of RRR with increasing disease severity in HIV-infected patients was mainly dependent upon increasing heart rate and to a lesser degree upon increasing age. Therefore, a decreased heart rate variability in AIDS is not a specific sign of autonomic neuropathy and likely to be a false positive test result.

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Completing previous studies in patients with sinus bradycardia (Med. Klin. 82 [1987], 647-650) we compared metoprolol with carteolol and pindolol, pindolol with carteolol, no treatment with carteolol (in two groups) in five series of the paired comparisons of Holter-ECG each. With change from metoprolol to carteolol or pindolol (dose ratio 10:1) lowest heart rate on Holter-ECG increased by 28 or 29% without change of exercise heart rate. Direct comparison of pindolol and carteolol revealed a very similar heart rate profile, indicating equipotent beta blockade and ISA. In patients with previous beta blocker induced bradycardia, carteolol did not change a normal resting heart rate off treatment. However, in patients with spontaneous sinus bradycardia carteolol increased lowest heart rate (+14%, due to overriding ISA) and lowered exercise heart rate (-15%, due to overriding beta blockade). A beta blocker induced sinus bradycardia consistently improved with change of treatment to carteolol and pindolol. With caution carteolol and pindolol may also be used despite spontaneous sinus bradycardia.

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The effect of an acute endogeneous catecholamine stimulation on the regulation of lymphocyte beta-adrenoceptor activity was studied in a patient with pheochromocytoma. Baseline blood pressure, heart rate, adrenoceptor density, and plasma concentrations of epinephrine, norepinephrine, and cyclic adenosine monophosphate were normal. Excessive spontaneous increases of catecholamine concentrations were accompanied by a rise in blood pressure, bradycardia, and an acute up-regulation of beta-adrenoceptors. Plasma concentrations of cyclic adenosine monophosphate paralleled the increase in receptor density and blood pressure. After normalization of catecholamine plasma levels, blood pressure, and beta-adrenoceptor density returned to baseline values. This observation adds support to the theory that an acute catecholamine stimulation leads to an acute sensitization of the beta-adrenoceptor-adenylatecyclase-cyclic-adenosine-monophosphate system leading to blood pressure elevation.

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Hypersensitivity reactions to heparin preparations with a wide spectrum of clinical manifestations have been reported frequently in the past, but are a rarity now. A 88 year old man was admitted for physical therapy of a collum femoris fracture. Treatment with a diuretic, Reserpine and Verapamil was continued. Chest x-ray revealed a large thoracic aortic aneurysm. From the 12th to the 18th day of low dose heparin prophylaxis with calcium heparin, 7500 U twice daily, at least eight attacks of asthma or cyanosis were observed, starting about two hours after heparin injection. The last attack began suddenly with wheezing, tachypnoea and cough and was associated with apprehension, a sudden blood pressure increase and severe cyanosis. Ventilation improved with oxygen and a beta 2-stimulator, but hypertension and cyanosis lasted for three hours. After discontinuation of heparin no further attacks occurred. Causes other then heparin could not be found. Despite the use of porcine mucosa heparin, avoidance of preservatives and use of low doses a hypersensitivity reaction occurred in our case. The delayed onset after preceding subcutaneous application as well as difficulties in separating the reaction from complications of underlying disease may delay heparin discontinuation.

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Mother, father (26 y.o.) and their only child (5 y.o.) developed nonproductive cough, fever (39.5 to 40.4 degrees C) and bilateral pulmonary infiltrates within three weeks. In addition the mother developed a small left pleural effusion and a pericardial effusion, a relative bradycardia, a pruritic vesicular exanthem of the extremities and the trunk, an erythema nodosum and arthritis of the tarsal joints. The father's coulter counter red blood count was distorted by microagglutination at room temperature (hemoglobin 13.2 gr/dl; erythrocytes 1,91 X 10(6) mm-3 and MCH 69.1 pg; MCV 120 fl and hematocrit 23.8%) but not at 37 degrees C (13.2; 4.15 and 31.8; 92 and 39.3, respectively). In the daughter myringitis, pharyngitis, cervical lymphadenopathy and splenomegaly were observed. Cold agglutinins and serologic evidence for mycoplasma pneumoniae infection were demonstrable in all three. Treatment with Tetracycline (parents) and Erythromycin (child) was effective.

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In patients dying in adolescence with familial hypercholesterolemia a combination of xanthomata of the skin and tendons, atheromatous plaques on the aortic and mitral valves, the coronary artery outlets and in the proximal aorta is frequently found. However, little is known of the sequence of these changes or their clinical equivalents. For this reason findings are described in two patients whose homozygous familial hypercholesterolemia is confirmed by studies of the cell biochemistry of the dermal fibroblasts after an observation period of eight and twelve years.

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