ISSN:0014-2972    

DOI:10.1111/j.1365-2362.1985.tb00298.x

出版商:Blackwell Publishing Ltd    

年代:1985

数据来源: WILEY

摘要:

Abstract.In a prospective investigation, a large kindred (twenty‐one subjects) with unexplained association of familial hypocalciuric hypercalcaemia and idiopathic interstitial lung disease was studied. Serum calcium was increased in fifteen patients (the youngest being 7 years old) and was associated with hypo‐ or normocalciuria. The abnormalities were not age‐dependent. The serum concentrations of parathyroid hormone, 25–hydroxyvitamin D3, 1,25‐dihydroxyvitamin D3and calcitonin were normal. In twelve patients the diffusing capacity (DLco) and/or DLco per unit lung volume was less than 75% predicted. This was often accompanied by a vital capacity of less than 80% predicted, an increased Tiffeneau index, and a reticulo‐micronodular pattern with high diaphragm on chest X‐ray. The decrease in DLco was more pronounced in older non‐smoking as well as smoking subjects (P<0·02) suggesting a progressing interstitial disease with age. The fibrosing alveolitis, which had been confirmed by open lung biopsy in three subjects, could not be attributed to sarcoïdosis, collagen‐vascular disease, or exogenous causes. The disturbances in the calcium homeostasis and in the diffusing capacity of the lung coexisted in seven of the twenty‐one patients. Apparently, both abnormalities were inherited following an autosomal‐dominant pattern but with a different penetration in each person, and seemed not be causall

ISSN:0014-2972    

DOI:10.1111/j.1365-2362.1985.tb00136.x

出版商:Blackwell Publishing Ltd    

年代:1985

数据来源: WILEY

摘要:

Abstract.Earlier we have shown that bone resorption is impaired in cultured calvaria from beige mice (an animal equivalent of the Chediak–Higashi syndrome in man). In the present study we have compared the concentrations of cyclic AMP and cyclic GMP in calvarial bones from beige mice with the nucleotide concentrations in bones from corresponding normal mice. In six independent experiments the basal concentrations of cyclic AMP was significantly (P<0·01) higher in bones from beige mice (on an average 50% augmented). The ratio of cyclic AMP/cyclic GMP was 2·43 times higher (P<0·01) in bones from beige mice. After stimulation with the phosphodiesterase inhibitor isobutylmethylxanthine and prostaglandin E2no significant differences of cyclic AMP concentrations between beige and control mice could be registered. The response to adenosine was significantly higher (P<0·005) in bones from beige mice (4·3 ± 0·4‐fold of basal cyclic AMP concentrations, mean ± SE) compared to control mice (1·9 ± 0·4‐fold of basal, mean ± SE). The increased basal concentration of cyclic AMP in calvaria from beige mice may be due to increased sensitivity to some agonists, such as adenosine, rather than simply being a func

ISSN:0014-2972    

DOI:10.1111/j.1365-2362.1985.tb00137.x

出版商:Blackwell Publishing Ltd    

年代:1985

数据来源: WILEY

摘要:

Abstract.The effects of dazoxiben on finger‐blood flow in response to cold challenge were studied in normal subjects and patients with Raynaud's phenomenon. In normal subjects concentrations of TXB2and 6‐oxo‐PGF1αwere measured in blood taken from dorsal hand veins following cold challenge. In a parallel multicentre study we examined the effects of dazoxiben on finger temperature and capillary blood cell velocity in patients with Raynaud's phenomenon. Dazoxiben did not affect finger arterial inflow at rest or during cold challenge in patients or controls. However in both groups, recovery was quicker after cold challenge on dazoxiben treatment. In patients median flow was 5 ml (100‐1ml) min‐1(range 1–10)v.2 (0·5–15),Plt; 0·05 dazoxibenv.placebo at 15 min after cold challenge. However, in normal subjects this did not prove to be statistically significant. In normal subjects there was a fall in TXB2concentrations and relative rise in 6‐oxo‐PGF1αfollowing dazoxiben treatment indicating redirection of prostaglandin endoperoxides towards synthesis of PGIi. Comparison of the sum‐total output of each eicosanoid following treatment with dazoxiben revealed a 65% reduction in TXB2concentrations (P<0·025 compared with placebo) and a 40% increase in 6‐oxo‐PGF1αconcentrations (P<0·05 compared with placebo). However a simultaneous increase in concentrations of FPA indicated generation of thrombin, probably at the needle tip. Long‐term treatment with dazoxiben resulted in no significant change in finger‐skin temperature or capillary blood cell velocity, duration, or severity

ISSN:0014-2972    

DOI:10.1111/j.1365-2362.1985.tb00138.x

出版商:Blackwell Publishing Ltd    

年代:1985

数据来源: WILEY

摘要:

Abstract.An oral water load of 20 ml (kg body wt)‐1was given to seventeen patients with the nephrotic syndrome and fifteen healthy control subjects. Diuresis (D), free water clearance (CH2O), plasma concentrations of arginine vasopressin (AVP), angiotensin II (AII) and aldosterone (Aldo) were determined before and 3 times during the first 4 h after loading. In the nephrotic syndrome D was significantly lower 1–2 h after loading than in the control subjects, predominantly due to a lower CH2O(2·61 and 7·01 ml min‐1(medians),P<0·01).Creatinine clearance and the maximum increase in CH2Owere significantly correlated in patients with the nephrotic syndrome (ρ= 0·721,n= 17,P<0·01) and the control subjects (ρ= 0·596,n= 15,P<0·01). AVP was reduced in both groups during loading, but AVP was clearly elevated in the patients with the nephrotic syndrome when compared to the control subjects both before (3·0 and 1·9 pmol 1‐1,P<0·01) and during loading. There was a significantly negative correlation between CH2Oand AVP in both groups. AII and Aldo were reduced during loading, but the levels were the same in the patients and in the control group, and AII and Aldo were not correlated to CH2O. It is concluded that patients with the nephrotic syndrome excrete an oral water load more slowly than healthy control subjects, and that this phenomenon partly is due to reduced glomerular filtration rate and part

ISSN:0014-2972    

DOI:10.1111/j.1365-2362.1985.tb00139.x

出版商:Blackwell Publishing Ltd    

年代:1985

数据来源: WILEY

摘要:

Abstract.The effects of adrenaline, noradrenaline, and of the α2‐ and β‐selective agonists clonidine and isoproterenol were studied in fifteen obese subjects before and after 4 weeks of caloric restriction (300 cal day‐1). Basal glycerol release averaged 1·4 μmol (106cells)‐1(180 min)‐1before starvation and 2·8 μmol (106cells)‐1(180 min)‐1during starvation (P≤ 0·1). Before starvation adrenaline and noradrenaline caused a 2–3‐fold increase of glycerol release. This lipolytic effect disappeared during starvation. An inhibitory effect of adrenaline was observed instead which was maximal at an adrenaline concentration of 1 μmol 1‐1(P≤ 0·05). The dose‐response relationships of the α2and β‐selective agents clonidine and isoproterenol were not appreciably changed by caloric restriction.The increase of basal lipolytic rate and the reversal of adrenaline action seen during caloric restriction could be mimicked by removal of endogenous adenosine using adenosine deaminase (1·6 μg ml‐1). In addition, inclusion of N6‐phenylisopropyladenosine (1 μmol 1‐1) into the medium reverted the adrenaline‐induced inhibition seen during caloric restriction.The results suggest that local modulators such as adenosine are of primary importance for the apparent change of responsiveness to adrenaline and noradrenaline

ISSN:0014-2972    

DOI:10.1111/j.1365-2362.1985.tb00140.x

出版商:Blackwell Publishing Ltd    

年代:1985

数据来源: WILEY

摘要:

Abstract.The effect of renal function on the cyclic AMP (cAMP) response to exogenous parathyroid hormone (PTH) was examined in patients with chronic renal failure (n= 22) and primary hyperparathyroidism (n= 19). In the patients with chronic renal failure there was marked resistance to the effect of exogenous PTH. In primary hyperparathyroidism the cAMP responses were variable; most of the patients with an abnormally small response having impaired renal function. After parathyroidectomy, responsiveness improved to varying degrees. In three patients repeatedly tested up to several months after parathyroidectomy, the recovery of responsiveness was a gradual process which began within days but did not, however, return to normal. Thus, there was an irreversible component to the resistance to PTH in these patients. A strong negative correlation between plasma creatinine and the cAMP response to PTH (P<0·001) was found in a group of patients, some with treated primary hyperparathyroidism and some with chronic renal failure. Thus, renal impairment is an important, but probably not the sole, contributory factor involved in the irreversible resistance to the action of PTH in hyperparathyroidism

ISSN:0014-2972    

DOI:10.1111/j.1365-2362.1985.tb00141.x

出版商:Blackwell Publishing Ltd    

年代:1985

数据来源: WILEY

摘要:

Abstract.In twenty‐one patients (sixteen male, five female) with various kidney diseases including primary hyperoxaluria type I (four patients), the plasma oxalate level was calculated from the isotopically determined oxalate clearance and the chemically determined urinary oxalate excretion. The apparent oxalate distribution volume was assessed as well.In patients with impaired kidney function (n= 12), the oxalate clearance was lower and the biological half‐life and plasma concentration were higher than in patients with normal kidney function (n= 10). No differences were found in the oxalate‐to‐creatinine clearance ratio (mean value 1·93), urinary oxalate excretion and apparent oxalate distribution volume. A linear relation was found between the oxalate and creatinine clearance, while the clearance ratio was independent of the degree of renal failure. The apparent oxalate distribution volume was 1·45 times the estimated extracellular fluid volume.Because the isotopically determined plasma oxalate levels are lower than chemically measured ones, a quick and better estimation of plasma oxalate can be made from the urinary oxalate excretion and the creatinine

ISSN:0014-2972    

DOI:10.1111/j.1365-2362.1985.tb00142.x

出版商:Blackwell Publishing Ltd    

年代:1985

数据来源: WILEY