Proteinuric glomerular disease in adults: cumulative life tables over twenty years

Outcome in 425 patients with persistent proteinuria has been assessed over a period of five to 20 years. Nephrotics and non-nephrotics are analysed separately. Clinical and laboratory findings do not correlate with renal histology. Seventy-eight patients had minimal histological lesions and four died (only one in renal failure). Fifty-one patients had endothelial cell proliferation, and 20-year survival was 70 per cent; renal failure occurred within five years in all 17 who progressed, and was independent of nephrotic status. In both membrano-proliferative disease (98 patients) and membranous glomerulopathy (59 patients) 20-year survival was 20 to 30 per cent. Epithelial cell proliferation (85 patients) had the worst prognosis, and survival after 12 years was negligible. In these last three groups decay in survival was almost linear against time and independent of nephrotic status. In focal proliferative disease 20-year survival in nephrotics (31 patients) was only 30 per cent, contrasting with non-nephrotics (23 patients) with 80 per cent survival. Of 105 patients presenting with proteinuria without symptoms, in 30 the proteinuria was postural and biopsies were not done. The other 75 showed a range of histological changes and prognosis similar to the group with symptoms.     

Minimal Change Glomerular Disease in Malaysian Adults and use of Alternate Day Steroid Therapy

One hundred and forty-eight patients over the age of 12 yearsseen from July 1972 through December 1980 were accepted forthis review of minimal change glomemlar disease (MCGD). Thediagnosis was based on a typical clinical presentation, andrenal biopsy findings MCGD forms 43.5 per cent of our patients with idiopathic nephroticsyndrome. The patients were predominantly young adults (82 percent) and the onset of nephrotic syndrome (NS) occurred belowthe 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-daysteroid (LASt) therapy. Four had cyclophosphamide alone, 21patients had cyclophosphamide after a trial of LASt Eighty-fourpatients (62.7 per cent) were initial res ponders: 17 of theseafter cyclophosphamide, 26 (19.4 per cent) were initial non-responders:five cyclophosphamide, 19 were late responders. Nine patientswere partial responders, six were non-responders and one wentinto renal failure. Forty-one patients defaulted, emigratedor were seen only locally at the time of study. Ninety-ninepatients were followed for three to 102 months (mean 23.3 months).Thirty-seven patients were followed for 36 to 102 months. Relapseswere infrequent but occurred as late as 60 months.     

我国康复医学事业二十年

80年代初 ,在党的十一届三中全会精神指引下 ,我国引进了现代康复医学 ,至今已近 2 0年。本文作者将简述党和政府对康复医学事业的有关方针、政策和重要举措 ,以及医务人员开辟、创造的部分业绩 ,留下一些历史资料 ,以供借鉴。康复医学是一门新兴学科 ,它是由医学与残疾学、心理学、社会学、工程学等相互渗透而成的一门跨科性学科 ,具体地说 ,康复医学是一门关于残疾和功能障碍的预防、评估诊断、治疗和处理的医学学科 ,其目的是减轻、消除功能障碍 ,帮助伤病员和残疾者根据其实际需要和身体潜力 ,最大限度地恢复其生理上、心理上、职业和社…     

The Nephrotic Syndrome in Adults with 'Minimal Change' Glomerular Lesions

We describe 49 patients who developed a nephrotic syndrome afterthe age of 15 years, and who showed a ‘minimal change’pattern in their renal biopsies. Patients with sclerosing lesionsaffecting only part of the glomerulus were excluded, but 39biopsies showed some minor changes. Glomerulosclerosis affectingwhole glomeruli was present in 21 biopsies, and related stronglyto increasing age. Focal tubular atrophy and vascular changeswere also common but less related to age. Mesangial matrix increasewas not age-related, and presumably is a component of the glomerulardisease. At onset, these patients showed features differing from thosefound in nephrotic children with similar histology: the sexincidence of the adults was equal, non-selective differentialprotein clearances (51 per cent) hypertension (31 per cent)and diminished renal function (70 per cent) were all more common.No clinical tests distinguished these patients clearly fromother adult nephrotics, and renal biopsy remains essential intheir management. The subsequent behaviour of the adult patientshowever, resembled that of the childhood group very closely.Forty four patients were treated with prednisolone for at leasteight weeks, and 82 per cent responded with early loss of proteinuria.Of the 36 responding patients, 70 per cent later relapsed, 63per cent repeatedly. Five corticosteroid-resistant, and 12 corticosteroidintolerantpatients were treated with cyclophosphamide: one died aftera pulmonary embolus, but 14 lost their proteinuria and 11 remainin remission. Follow-up data were available for all 49 patients for up to19 years (mean 4.1 years). Nine patients were dead, only onein uraemia but three from probable complications of treatment.Ten still relapse and require corticosteroid treatment, butonly one now has persistent symptomless proteinuria. Twenty-nineare well, off all treatment.     

Twenty years of functional MRI: the science and the stories

Since its inception over twenty years ago, the field of functional magnetic resonance imaging (fMRI) has grown in usage, sophistication, range of applications, and impact. After twenty years, it's useful to briefly look back as well as forward - to size up just how far we have come and speculate just how far we may go. This is an introduction to the special issue of "Twenty years of fMRI: the science and the stories." The one-hundred and three papers in this special issue highlight the major methodological developments and controversies of fMRI from a first person perspective over the past twenty years. The growth of this field is not just fascinating from a science and technology perspective, but also from a human perspective. Most who were fortunate enough to be part of this effort at the beginning, as well as those who jumped in along the way have their fair share of interesting stories consisting of top rate science as well as intense thought and effort, good or bad fortune, and some claim to a contribution. These stories are in the following papers, written by the current leaders in the field and the innovators throughout the twenty year history. The categories, designed to cover every aspect of the emergence and development of fMRI, include: pre-fMRI; the first BOLD brain activation results; developments in pulse sequences, imaging methods, and hardware for fMRI; methodological developments, issues, and mechanisms; new paradigm designs; education; and the future. Within this issue, we have a collage of overlapping, complementary, yet sometimes contradictory accounts of what happened during the breathtakingly diverse and intense development of this still growing field over the past twenty years.     

Variables Discriminating Between Cryptogenic Glomerular Lesions in Adults with the Nephrotic Syndrome

A univariate analysis of individual clinical and biochemicalvalues of adult patients with cryptogenic nephrotic syndromehas shown that significant differences exist between patientswith proliferative glomerulonephritis, ‘minimal change’nephritis and membranous nephropathy. For any given adult patient with the condition, the most likelyclinical diagnosis is proliferative disease and the next, minimalchange. These two diagnoses together account for most cases.The best clinical discriminants between them are the systolicblood pressure and plasma cholesterol concentration. If thesystolic pressure is greater than 145 mm Hg proliferative diseaseis more likely, but if the cholesterol is greater than 530 mgm/100ml,a minimal change lesion is more likely. A scattergram for combiningthese variables in clinical practice is given, showing a zoneof uncertainty where renal biopsy would be indicated. Although single variables do not permit discrimination betweenmembranous nephropathy and the other two groups, it is suggestedthat analytical techniques where combinations of variables areused may be helpful, and should be developed.     

Glycogen Storage Disease Diagnosed in Adults

Glycogen storage diseases are usually identified in childhood.We present the clinical, biochemical and histological featuresof 10 patients first diagnosed in adult life. Five had glycogenstorage disease type 1a, one type 1e, two type IX, and in twopatients there were previously unreported abnormalities of hepaticglucose-6-phosphatase system activity. Of the latter, one patienthad an inhibitor of liver glucose-6-phosphatase (pseudo-1b glycogenstorage disease) the other having abnormal glucose-6-phosphataseactivity and microsomal pyrophosphate transport. A glucagontest is suggested as a useful screening procedure. Glycogenstorage disease should be considered in adults with symptomssuggesting hypoglycaemia.     

Glomerular Hyperfiltration and Renal Disease Progression in Type 2 Diabetes

OBJECTIVE To describe the prevalence and determinants of hyperfiltration (glomerular filtration rate [GFR] ≥120 mL/min/1.73 m(2)), GFR decline, and nephropathy onset or progression in type 2 diabetic patients with normo- or microalbuminuria. RESEARCH DESIGN AND METHODS We longitudinally studied 600 hypertensive type 2 diabetic patients with albuminuria <200 μg/min and who were retrieved from two randomized trials testing the renal effect of trandolapril and delapril. Target blood pressure (BP) was <120/80 mmHg, and HbA(1c) was <7%. GFR, albuminuria, and glucose disposal rate (GDR) were centrally measured by iohexol plasma clearance, nephelometry in three consecutive overnight urine collections, and hyperinsulinemic euglycemic clamp, respectively. RESULTS Over a median (range) follow-up of 4.0 (1.7-8.1) years, GFR declined by 3.37 (5.71-1.31) mL/min/1.73 m(2) per year. GFR change was bimodal over time: a larger reduction at 6 months significantly predicted slower subsequent decline (coefficient: -0.0054; SE: 0.0009), particularly among hyperfiltering patients. A total of 90 subjects (15%) were hyperfiltering at inclusion, and 11 of 47 (23.4%) patients with persistent hyperfiltration progressed to micro- or macroalbuminuria versus 53 (10.6%) of the 502 who had their hyperfiltration ameliorated at 6 months or were nonhyperfiltering since inclusion (hazard ratio 2.16 [95% CI 1.13-4.14]). Amelioration of hyperfiltration was independent of baseline characteristics or ACE inhibition. It was significantly associated with improved BP and metabolic control, amelioration of GDR, and slower long-term GFR decline on follow-up. CONCLUSIONS Despite intensified treatment, patients with type 2 diabetes have a fast GFR decline. Hyperfiltration affects a subgroup of patients and may contribute to renal function loss and nephropathy onset or progression. Whether amelioration of hyperfiltration is renoprotective is worth investigating.     

Sex Differences in Children and Young Adults With Bicuspid Aortic Valve Disease in First Two Decades of Life

ObjectiveTo elucidate sex differences in valve morphology, disease phenotype, progression, and outcomes among children and young adults with bicuspid aortic valve (BAV).Patients and MethodsThis is a retrospective cohort study examining all children and young adults (aged ≤22 years) with isolated BAV diagnosed, by excluding patients with concomitant congenital heart defects or genetic syndromes, from January 1, 1990, through December 1, 2016, at Mayo Clinic in Rochester, Minnesota.ResultsOf 1010 patients with BAV, 558 had isolated BAV. Distributions of morphology were right-left in 65.8% (n=367), right-noncoronary in 34% (n=190), and left-noncoronary cusp fusion in 0.2% (n=1) of patients; with no sex differences. Male to female ratio was 3:1. At the first echocardiographic evaluation in the study, there were no sex differences in terms of frequency of aortic valve stenosis or regurgitation. However, males had significantly higher grades of aortic valve regurgitation at 17 years of age onward (P<.0001). Males had significantly larger mid-ascending aorta (P=.01) and sinus of Valsalva dimensions (z score; P=.0001) as compared with females, with a novel finding of peak aortic dimensions around 8 years of age. Males also had more than 2-fold higher risk for sinus of Valsalva dilation (z score >2) as compared with females (odds ratio, 2.3; 95% CI, 1.2 to 4.2; P=.01). There were no significant sex differences in the primary cardiac outcomes of interventions on aortic valve and/or aorta, aortic dissection, or death.ConclusionIn children and young adults with BAV, males have a higher grade of aortic regurgitation in late adolescence, significantly larger aortic dimensions, different patterns of aortic growth, and more frequent sinus of Valsalva dilation as compared with females. Overall, the rate of primary cardiac events is lower in young patients, with no significant sex differences.     

Degrees of Kidney Disease in Nigerian Adults with Sickle-Cell Disease

Objective

To study degrees of chronic kidney disease (CKD) using creatinine clearance in adult Nigerian patients with sickle-cell disease (SCD).

Methods

One hundred SCD patients, made up of 79 HbSS (homozygous haemoglobin S) patients and 21 HbSC (heterozygous haemoglobins S and C) patients, were investigated prospectively, along with 50 normal controls. Their sociodemographic data, weight and drug history were documented. Each participant underwent dipstick urinalysis, and creatinine clearance was calculated following a 24-hour urine collection and serum creatinine measurement. They were categorized into stages of CKD based on the creatinine clearance.

Results

Of the 79 HbSS patients, 14 (18%), 28 (35%), 33 (42%) and 4 (5%) had stage 1, 2, 3 and 4 CKD, respectively. In the HbSC group, 3 (14%), 9 (43%) and 9 (43%) patients had stage 1, 2 and 3 CKD, respectively. Proteinuria was noted in 16 (20%) HbSS patients but not in any of the HbSC patients. Of the subjects aged ≤24 years (n = 49), 9 (18%), 18 (37%), 21 (43%) and 1 (2%) had stage 1, 2, 3 and 4 CKD, respectively. Of those aged >24 years (n = 51), 8 (16%), 19 (37%), 21 (41%) and 3 (6%) had stage 1, 2, 3 and 4 CKD, respectively. None of the subjects had stage 5 CKD.

Conclusion

In this study, the adult subjects with SCD had various degrees of CKD. Adequate follow-up and active intervention are advocated to delay the onset of end-stage nephropathy.Key Words: Chronic kidney disease, Creatinine clearance, Sickle-cell disease