Kappa light chain nephropathy

Summary Percutaneous renal biopsies from 4 patients with clinically unsuspected kappa light chain nephropathy were studied using light, immunofluorescence, and electron microscopy. The diagnosis in each case was established by demonstrating monoclonal kappa light chain deposits in basement membranes and basement membrane-like structures of glomeruli, tubules, and blood vessels by immunofluorescence microscopy. Characteristic electron dense deposits occurred in every case but the intensity and distribution of electron densitites did not correlate with the immunofluorescence findings. When light chain aggregation occurred, as evidenced by the distribution of electron dense deposits, it was proportional to the amount of basement membrane-like material as if these immunoglobulins had a particular affinity for structures chemically related to basement membranes. Although active tubulointerstitial lesions were prominent in all biopsies, there was considerable variation in glomerular pathology with only 1 case exhibiting the typical nodular glomerulosclerosis. Correlation of the light, immunofluorescence, and electron microscopic findings in these cases suggests that the pathogenesis of kappa light chain nephropathy is related to light chain nephrotoxicity directed to basement membrane-like structures with subsequent alterations in hemodynamics and structural renal damage.     

Is Exercise-induced Blood Pressure Rise Predictive of Nephropathy in Insulin-dependent Diabetes?

ABSTRACT A follow-up study was performed in 48 male diabetics and 17 age-matched male controls, who in 1963 and 1971 participated in an exercise study to evaluate if the results could predict later development of diabetic nephropathy. The inclusion criteria were: Type I (insulin-dependent) diabetes with age below 40, onset of diabetes before age of 30, duration of diabetes more than 7 years and no proteinuria at the time for the first study. The diabetics demonstrated higher systolic blood pressure (BP) at work but as good physical condition as the controls. Thirteen developed nephropathy after 23 years (range 15–36) of diabetes duration. It was found that exercise-induced, abnormally raised systolic BP was not associated with later development of diabetic nephropathy.     

补肾化瘀解毒法治疗2型糖尿病肾病临床研究

目的 观察补肾化瘀解毒法对2型糖尿病肾病（DN）的临床疗效。方法 对78例DN患随机分为治疗组39例及对照组39例，采用饮食管理、降糖治疗，治疗组同时予以补肾化瘀解毒中药口服2个月。结果 治疗组疗效优于对照组（P＜0．01）。结论 补肾化瘀解毒法具有改善DN患临床症状、减少尿蛋白排泄、改善肾功能，以及延缓、控制DN的自然进程，提高DN患的生活质量等综合作用。     

The effects of stress management on symptoms of upper respiratory tract infection, secretory immunoglobulin A, and mood in young adults

Objective: To investigate the efficacy of a stress management programme on symptoms of colds and influenza in 27 university students before and after the examination period. Method: The incidence of symptoms, levels of negative affect, and secretion rate of secretory immunoglobulin A (sIgA) were recorded for 5 weeks before treatment, for the 4 weeks of treatment, and for 8 weeks after treatment in treated subjects and in 25 others who did not participate in stress management. Results: Symptoms decreased in treated subjects but not in controls during and after the examination period. Although sIgA secretion rate increased significantly after individual sessions of relaxation, resting secretion rate of sIgA did not increase over the course of the study. Negative affect decreased after examinations in both groups, but was not affected by treatment. Conclusion: Stress management reduced days of illness independently of negative affect and sIgA secretion rate. Although the component of treatment responsible for this effect has yet to be identified, psychological interventions may have a role in reducing symptoms of upper respiratory tract infection.     

特异性IgA抗体: 心脑血管病慢性肺炎衣原体感染的标志

目的：探索心脑血管病慢性肺炎衣原体（ＣＰ）感染的血清学证据。     

Presence of crystalline inclusions in the peripheral nerve of a patient with IgA lambda monoclonal gammopathy of undetermined significance

Association of a peripheral neuropathy with an IgA monoclonal gammopathy of undetermined significance (MGUS) is not commonly observed and is sometimes considered as coincidental. We present a case in which the nerve biopsy revealed the presence of crystalline inclusions in the endoneurium, a very unusual finding. A 75‐year‐old man complained of paresthesiae in both feet and unsteady gait for 6 months. He had no weakness, but deep tendon reflexes were absent and vibratory sensation distally diminished in both legs. An IgA lambda MGUS was evidenced in his serum at 10.2 g/L with 7% plasma cells in his bone marrow and no lytic lesion at skeletal examination. A superficial peroneal nerve biopsy was performed and showed numerous crystalline inclusions in the endoneurium. These were located in the cytoplasm of macrophagic histiocytes or free in the vicinity of nerve fibers. There was also a marked loss of myelinated nerve fibers and several “onion bulb” formations surrounding either isolated remyelinating fibers or small clusters of remyelinating fibers. Such crystalline inclusions have mainly been observed in the cytoplasm of plasma cells in cases of multiple myeloma, and correspond to non‐secreted IgA or IgG immunoglobulins with a kappa or rarely lambda light chain. Such inclusions have also been reported in the cytoplasm of the epithelial cells from corneal fragments, in patients with multiple myeloma or IgG MGUS, and in the tubular cells from the kidney of patients with multiple myeloma and a nephrotic syndrome. In the literature, there is only one very briefly mentioned case of neuropathy associated with a myeloma and with crystalline inclusions present in the epineurium. Thus, in dysglobulinemic neuropathy, nerve fibers can be damaged by three kinds of interstitial deposits, easily identified by immunohistochemistry and at ultrastructural examination: the well known amyloid fibrils, granulo‐fibrillar deposits and also crystalline inclusions.     

中西医结合治疗特发性膜性肾病临床观察

目的 :观察中西医结合治疗特发性膜性肾病临床疗效。方法 :对 6 8例特发性膜性肾病随机分为对照组 32例 ,治疗组 36例 ;对照组单纯用强的松和环磷酰胺、潘生丁、洛丁新 ,治疗组在用上述西药同时辨证加用中药治疗 ,观察 3年～ 6年。结果 :对照组完全缓解率为 2 8.1% ,总缓解率为 6 5 .6 % ;治疗组完全缓解率为 5 5 .6 % ,总缓解率为 86 .2 %。治疗组完全缓解率和总缓解率明显优于对照组 ,两组比较P <0 .0 5 ,具有显著性差异。结论 :中西医结合是治疗膜性肾病的有效方法。     

Circulating levels of matrix metalloproteinases MMP-3 and MMP-2 in renal transplant recipients with chronic transplant nephropathy.

BACKGROUND: Chronic transplant nephropathy remains the major cause of graft loss after the first year post transplant, with the exception of death with functioning graft. The histological hallmark of chronic kidney rejection is progressive fibrosis in which extracellular matrix turnover plays an important role. This turnover is regulated by several systems of connective tissue proteases, the matrix metalloproteinases family being one of them. Every metalloproteinase exerts a different function over extracellular matrix proteins and can contribute to the pathogenesis of several diseases, such as rheumatoid arthritis and glomerulonephritis. The role of metalloproteinases in the pathogenesis of chronic transplant nephropathy and in kidney transplantation has not yet been addressed. METHODS: We measured the serum levels of proMMP-1, proMMP-2 and proMMP-3 by ELISA in 40 patients with chronic transplant nephropathy, 30 with acute rejection, 30 with stable graft function for a time equivalent to chronic transplant nephropathy, 30 with stable graft function for a time equivalent to acute rejection, and 30 healthy age-paired blood donors. RESULTS: Serum proMMP-2 and proMMP-3 were significantly higher in patients with chronic transplant nephropathy than in patients with acute rejection, stable graft function and healthy donors. The most striking finding was the significant positive correlation observed between serum levels of proMMP-3 and serum creatinine, and between circulating levels of proMMP-2 and proteinuria. Serum concentration of proMMP-1 was increased in patients with acute rejection compared with those with stable graft function and healthy donors. CONCLUSIONS: Serum proMMP-2 and proMMP-3 reflect the changes of glomerular and interstitial extracellular matrix in chronic transplant nephropathy, suggesting that they could play a role in the pathogenesis of this condition. Acute rejection is associated with increased levels of proMMP-1, which could be a reflection of the stimulation induced by a number of inflammatory cytokines produced in such a process.     

Burn-induced linear IgA dermatosis

There have been several reports of linear IgA dermatosis (LAD) associated with drug exposure and lymphoproliferative malignancy, but trauma and burns have been suggested only in patients with bullous pemphigoid. We present a case of burn-induced LAD in a 48-year-old caucasian male presenting with a recent history of blistering eruption on the periphery of a cicatricial area caused by boiling methyl alcohol. Clinically, he presented a widespread bullous eruption. The direct immunofluorescence examination of a perilesional biopsy revealed an intense homogeneous linear pattern of IgA deposition consistent with the diagnosis of LAD. The patient responded to therapy with systemic steroids.