Nephrotic syndrome

The nephrotic syndrome is a common disorder with many causes. The pathology of nephrotic syndrome resides chiefly in the renal glomerulus. Regardless of etiology, an abnormality of the glomerular wall is always found. Electron microscopic studies of needle biopsy specimens have shown the basic lesion to be an alteration of the epithelial cell layer food processThe pathogenesis of the principal features of the syndrome were discussed briefly. The mechanism(s) of proteinuria, hypercholesterolemia and to some extent, of edema, remain obscure. The significance of urinary doubly refactile lipid bodies exhibiting the "Maltese cross" phenomenon in the diagnosis of nephrotic syndrome was emphasizedRemediable cause of nephrotic syndrome should be carefully eliminated before embarking on any course of therapy aimed principally towards relieving the edema. The introduction of cortisone in the 1950s has brightened the outlook of patients with the idiopathic nephrotic syndrome. (Summary)

Adrenal cortical steroid therapy of primary nephrotic syndrome among adult Filipinos: clinical, biochemical and histologic studies

An analysis of the response to corticosteroid therapy of 66 patients with primary nephrotic syndrome was presented in this paper. A summary of the clinical status and biochemical data before and after the initial course of corticosteroid therapy is shown in TABLE VIII. Forty three patients followed for 1 to 48 months was likewise analyzed; of these 44 patients, 26 were on intermittent steroids and 18 were not on the drugThe type of response obtained was classified into Grade A, B, C and D. Remission after the initial course was obtained in 62% of the cases treated. In the intermittent-treated group, 58% were in remission at the time of this study in contrast to 41% remission in patients who were not maintained on the drug. There were 15 deaths in this series, 7 occurred in the initial course of treatment and 8 in the follow-up period. All fatalities during the initial course were among uremic patients with endstage kidneys. In the follow-up period deaths were also due to uremia and were on patients who responded poorly do the initial courseCorticosteroids remain the drug of choice in the management of primary nephrotic syndrome and should be used whenever indicated. ACTH therapy is more expensive and requires the parenteral route for its administration. The routine administration of ACTH in the course of corticosteroid therapy was not utilized in this study as this has been shown to be of no value in preventing adrenal insufficiencyKidney biopsies are useful in diagnosis, prognosis and guide to therapy of nephrotic syndrome but should not supersede the clinical features and common laboratory procedures in the choice of patients for corticosteroid therapy as suggested by other authors. Eighteen patients in this study have kidney biopsies. Remissions were obtained in patients with "normal" and minimal degree of glomerular involvement; however; poor results were also seen in patients with minimal changesThe need to follow patients closely and with periodic determination of the common laboratory procedures as a guide to therapy was stressedFinally, corticosteroid therapy of primary nephrotic syndrome is most beneficial in patients ill with the disease for less than 6 months duration, without or with minimal azotemia, hypertension and hematuria, and in cases with "normal" and minimal glomerular changes. Adequate dosage and longer initial course of therapy of at least 6 weeks and possibly more in some cases will insure a higher incidence of remission. The duration of intermittent steroids therapy has not been conclusively settled but it is the general felling that it should be continued for 6 months to 1 year after a successful initial course of treatment in patients who remained in remission. (Summary and Conclusions)

Correlative studies on the occurrence of urinary anisotropic lipid bodies in conditions associated with Proteinuria

The incidence of anisotropic lipid material in the urine increases significantly with the degree of proteinuria. Lipid crystals were seen in all cases of nephrotic syndrome studied and in 11 of 28 non-nephrotic subjects with abnormal amounts of protein excretion per day. In 10 subjects without or with normal amounts of protein excretion per day, anisotropic lipid particles were not seenWhen the clinical diagnosis of all the subjects studied were divided into 2 groups as to whether they are known or not known causes of nephrotic syndrome, it was found that anisotropic lipid bodies were seen significantly more often in the former group. This observation among the non-nephrotic subjects inevitably leads to the question whether they can be regarded as pre-nephroticsThere was no correlation seen with serum albumin, non-protein nitrogen and total cholesterol with the excretion of urinary anisotropic lipid material. The lack of correlation with plasma levels of total cholesterol was surprising in view of the fact that Maltese crosses are composed of cholesterol ester and free cholesterol. This finding strengthens the observation that a renal factor possibly the presence of glomerular damage is a requirement for the occurrence of cholesteroluria. (Summary)

Correlative studies on pyuria, quantitative urine culture, and renal function tests

In the study 74% of urine specimens with pyuria grew signfificant colony counts. In specimens with "normal" numbers of white blood cells in the positive urine cultures were obtained in 20%. The latter observation has been occasionally reported in the medical literature as instances of bacteriuria without pyuria. Further analysis of cases with "normal" leucocyturia with the use of the Sternheimer-Malbin stain revealed that positive urine cultures were obtained in those with pale blue type of white blood cells. The importance of this observation in the diagnosis of asymptomatic urinary tract infection was stressedTwo important parameters of renal function in correlation with pyuria were also assessed in this study: glomerular filtration rate by B.U.N., serum creatinine and endogenous creatinine clearance and concentrating ability by Fishberg concentration test. The limitation of B.U.N. and serum creatinine in estimating G.F.R. was pointed out since the values of these end products of protein metabolism do not go up unless G.F.R. is reduced by over 50%. The presence of normal creatinine clearance and Fishberg concentration test in patients with pyuria and bacteriuria are strong evidences favoring the confinement of the urinary infection to the lower tract. Impaired creatinine clearance and concentration tests are observed in patients with pyelonepthritis and other underlying renal diseases with or without superimposed infection.(Summary and Conclusions)

Kidney and liver biopsy studies in systemic lupus erythematosus

A case of a 30-year-old Filipina housewife with systemic lupus erythematosus studied by percutaneous needle biopsy of the kidney and liver was reported. (Summary)