Acquired cutis laxa and multiple myeloma.
We report a patient with acquired cutis laxa and multiple myeloma, and the possible association between the two conditions is discussed.
Biomedical subjects
Publications and source records attributed to F Wang.
We report a patient with acquired cutis laxa and multiple myeloma, and the possible association between the two conditions is discussed.
Thirty-one patients with systemic lupus erythematosus had membranous lupus nephropathy (MLN). They were divided into two groups. Group I consisted of 13 patients who had pure MLN but the patients in Group 2 had segmental proliferation in up to 35 per cent of their glomeruli. The rest of the glomeruli had purely membranous change. The patients of Group 2 were no different from the other MLN patients in terms of age, sex and race. The extrarenal disease in both groups was extensive and severe. The renal disease was usually associated with the nephrotic syndrome or oedema but was asymptomatic throughout in one patient. Both renal and extrarenal features responded to treatment initially but relapses were frequent and often severe. Relapses often occurred as treatment was discontinued or medication reduced. Survival at six years in Group I was 62 per cent and in Group 2 was 50 per cent. Only one patient died with renal failure although five patients had impaired renal function at death. The chief causes of death were disease of the central nervous system and infection.
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There is little information in the literature concerning scabies in patients with systemic lupus erythematosus. Scabies in our five patients appeared to be more severe than usual, and two of them developed crusted scabies, probably on an immunologic basis.
Both the mammalian thick ascending limb of Henle's loop and the shark rectal gland actively transport Cl against an electrochemical gradient by mechanisms involving hormone-sensitive NaCl transport. In contrast to mammalian renal tubules, individual tubules of the shark rectal gland previously have not been perfused in vitro. Using a combination of renal slice and microdissection techniques we were able to isolate and perfuse single rectal gland tubules without the use of enzyme treatment. Single tubules consistently generated lumen-negative transepithelial voltages (Vt) of -1.8 mV when perfused and bathed with identical shark Ringer's solution. The addition of cyclic AMP, vasoactive intestinal peptide (VIP), and adenosine to the bath increased Vt to -7.5, -9.0, and -4.3 mV, respectively (all P less than 0.02 compared with paired controls). Each stimulation could be reversed by addition by furosemide to the bath. The adenosine response was inhibited by theophylline, a specific inhibitor of adenosine receptors. The tubules had a low transepithelial electrical resistance of 12-26 omega X cm2 and exhibited a transepithelial permselectivity for small cations. These results indicate that tubules of the rectal gland can be perfused in vitro and have receptors for VIP and adenosine. Cyclic AMP and secretagogues hyperpolarize the membrane consistent with electrogenic chloride transport, and these effects are reversed by furosemide, an inhibitor of coupled sodium-potassium-chloride co-transport. The response of Vt to cyclic AMP and furosemide, the transepithelial electrical resistance, and the cation selective permeability of tubules are remarkably similar to measurements in perfused mammalian thick ascending limbs.
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One hundred and forty-eight patients over the age of 12 years seen from July 1972 through December 1980 were accepted for this review of minimal change glomerular disease (MCGD). The diagnosis was based on a typical clinical presentation, and renal biopsy findings. MCGD forms 43.5 per cent of our patients with idiopathic nephrotic syndrome. The patients were predominantly young adults (82 per cent) and the onset of nephrotic syndrome (NS) occurred below the age of 30 years. Most patients had severe oedema and hypoalbuminaemia. Few patients recovered spontaneously. One hundred and thirty patients were given long-term alternate-day steroid (LASt) therapy. Four had cyclophosphamide alone, 21 patients had cyclophosphamide after a trial of LASt. Eighty-four patients (62.7 per cent) were initial responders: 17 of these after cyclophosphamide, 26 (19.4 per cent) were initial non-responders: five cyclophosphamide, 19 were late responders. Nine patients were partial responders, six were non-responders and one went into renal failure. Forty-one patients defaulted, emigrated or were seen only locally at the time of study. Ninety-nine patients were followed for three to 102 months (mean 23 . 3 months). Thirty-seven patients were followed for 36 to 102 months. Relapses were infrequent but occurred as late as 60 months.
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Twenty-seven patients with histologically confirmed lupus nephritis were studied to identify the incidence of lupus band and its significance to histological patterns of nephritis and complement levels. It was found that the kidney involvement is 2.5 times more frequent in lupus band positive patients. The association of a positive lupus and low C3 level signifies the presence of diffuse proliferative glomerulonephritis rather than membranous glomerulonephritis. The results, together with earlier reports, are discussed.
A patient with idiopathic inhibitor to factor VIII is reported. Treatment with cyclophosphamide and factor VIII (given simultaneously) produced a good clinical response with disappearance of the inhibitor.
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