Immunotactoid glomerulopathy.

We present 11 patients with immunotactoid glomerulopathy, a new syndrome characterized clinically by proteinuria (11/11), microscopic hematuria (9/11) and hypertension (9/11). The patients consisted of six females and five males, aged 25 to 59 years (mean, 44.6). Proteinuria was the presenting feature and the reason for renal biopsy in all patients. The diagnosis of immunotactoid glomerulopathy was established at renal biopsy by the presence of glomerular extracellular microtubules composed of immune reactants. All the biopsies studied by immunofluorescence (10 cases) had glomerular deposits of IgG and C3. In three biopsies studied with IgG subclass specific antisera, only one patient had monoclonal immunoglobulin deposits (IgG3 kappa). In six cases the glomerular deposits were analyzed for light chains. In three the deposits contained kappa only, and three consisted of both kappa and lambda. In two cases the immune aggregates were confined to the mesangium, and in the remaining eight cases, the deposits were present in the mesangium and the glomerular basement membranes. Electron-dense deposits composed of microtubules were present in the same distribution within the glomerulus as the immune reactants. The microtubules had a uniform diameter in each biopsy, but they varied in size from case to case. They were approximately the same size in eight cases (mean, 22.3 +/- 3 [SD] nm). Three cases had much larger microtubules: 34.2 nm, 35.4 nm, and 48.9 nm in diameter. Although the 22.3-nm microtubules resembled amyloid in their appearance, glomerular distribution and random orientation in the tissue, they were more than twice the diameter of amyloid (8.9 nm), and Congo red and thioflavin T stains for amyloid were negative. Similar microtubular structures have been described in patients with cryoglobulinemia, SLE and paraproteinemia, but these diseases were excluded in our patients on clinical, serologic and in some cases histologic grounds. More important, none of our patients had clinical or histochemical evidence of amyloidosis, an entity which may be confused with immunotactoid glomerulopathy on a morphologic basis. Follow-up, from 22 to 94 months (mean, 52.6) was obtained in all 11 patients, and 2 clinical courses were noted. Six patients had progressive deterioration of renal function, with five requiring dialysis. This group had severe hypertension (4/6) and nephrotic-range proteinuria (5/6) at some point in their course. The remaining five patients with stable renal function had proteinuria of less than 2.0 g/24 hr in most cases (4/5), and none had severe hypertension. This dichotomy correlated with the distribution of immunotactoids.(ABSTRACT TRUNCATED AT 400 WORDS)

Duodenal gangliocytic paragangliomas: a study of 10 cases with immunocytochemical neuroendocrine markers.

Ten cases of duodenal paraganglioma were studied by conventional histologic and immunocytochemical techniques at both light and electron microscopic levels. Histologically, mixtures of epithelial, ganglion, and spindle cells were seen. In all of the cases immunoreactivity for neuron-specific enolase (NSE) and protein gene product (PGP) 9.5 was seen in each component. Pancreatic polypeptide immunoreactivity was detected in eight cases, mainly in epithelial cells. Somatostatin immunoreactivity was present in epithelial and ganglion cells in nine cases. In seven cases immunoreactivity for neurofilaments, a marker for neurons, was seen in ganglion and spindle cells. However, immunoreactivity for chromogranin, a protein found in endocrine storage granules, was found in only two cases, and the staining was confined to well-granulated epithelial cells. The spindle cells were immunostained only for neuronal markers, NSE and neurofilaments, and the glial marker S-100 protein.

Minimal renal amyloidosis with nephrotic syndrome.

Two patients older than age 50 years had minimal amyloidosis in association with the nephrotic syndrome. The amyloid deposits in each case were so inconspicuous as to have been initially overlooked, and both biopsy specimens were thought to show minimal glomerular changes. A few, widely scattered, silver-positive, epimembranous spicules were found on reexamination by light microscopy, and Congo red and thioflavin T stains and electron microscopy confirmed the presence of small glomerular amyloid deposits. Both patients have since died of renal failure. In our series of biopsy specimens, amyloidosis was found in 14.2% of patients older than age 50 years presenting with nephrotic syndrome or severe proteinuria, over 1 1/2 times the frequency of minimal-change nephrotic syndrome. We therefore urge careful examination for amyloid deposits of all kidney biopsy specimens with the appearance of minimal-change nephrotic syndrome in older patients. Fluorescence microscopy of Congo red- and thioflavin T-stained sections is very helpful in the detection of small deposits.

Mesangiolysis: an important glomerular lesion in thrombotic microangiopathy.

Six cases of malignancy-associated thrombotic microangiopathy and eight cases of idiopathic microangiopathy have been studied by renal biopsy. All patients of both groups had mild to severe renal impairment and microangiopathic hemolytic anemia. The renal lesions were histopathologically identical in the two groups. The most characteristic abnormalities were glomerular mesangiolysis and glomerular and arteriolar thrombosis. Subendothelial widening, presumably due to entrapment of blood components, and the formation of capillary and arteriolar thrombi may be attributed to endothelial damage. Glomerular fibrinogen was demonstrated by immunofluorescence in a majority of cases. Immunofluorescence also showed glomerular immunoglobulin M (IgM) and Clq in a majority of cases and C3 in slightly less than half. Mesangiolysis, present in every case, resulted in coalescence of capillary lumina, but mesangiolysis is a bland process, easily overlooked. The mesangial waists of the glomerular tufts seemed to unravel and come apart, with no inflammatory reaction or fibrin deposition on the luminal surface. The presence of capillary enlargement was confirmed morphometrically as an increased proportion of glomerular sectional area. In what appeared to be a late stage of mesangiolysis, the mesangium was thickened by pale fibrillary material, producing a lobulated glomerular tuft and eventual glomerular solidification. The early stages of mesangiolysis may be reflected only in glomerular capillary ectasia, whereas the late stages produce a distinctive form of glomerular sclerosis.

Clinical and imaging features of rhabdoid tumor of the kidney.

Rhabdoid tumor of the kidney (RTK) is a rare, highly malignant neoplasm of childhood. The clinical profile of this neoplasm differs from that of Wilms' tumor. We present two cases of RTK. In both our cases, large bulky masses with poorly defined margins and calcifications were demonstrated. The clinical and imaging findings are compared with other childhood renal neoplasms.

Lecithin cholesterol acyltransferase deficiency: ultrastructural examination of sequential renal biopsies.

We present the renal biopsy findings in two brothers with nephrotic syndrome and lecithin:cholesterol acyltransferase deficiency. Mesangial expansion and capillary wall thickening were accompanied by numerous extracellular osmiophilic membranes within round lucent spaces, by round lamellar deposits, and by amorphous deposits containing thread-like structures with cross-striations. A second biopsy in one of the brothers, when he had developed renal insufficiency 8 yr later, showed increased mesangial and capillary wall thickening. The deposits, which had been in predominantly subepithelial and intramembranous locations, were now in a more prominent subendothelial location. The subepithelial localization of the deposits seen on initial examination may be related to their cationic charge, perhaps with binding to glomerular polyanion, and to uptake by glomerular epithelial cells. The accumulation of lipid is associated with progressive mesangial and glomerular sclerosis.

Further observations in the ultrasound evaluation of renal allograft rejection.

In order to determine whether sonographic parenchymal changes and volume changes are reliable predictors of acute transplant rejection, 36 recipients of renal allografts were studied. The significance of graft volume changes was evaluated, since most previous studies have not utilized routine immediate postoperative baseline scans for comparison to follow-up scans. All patients had baseline scans within 24 hours of the transplant. All 24 acute rejection patients had biopsy proof of rejection within three days of the follow-up scans. Seven clinically normal and five clinically designated acute tubular necrosis (ATN) patients were studied for comparison. A retrospective blind review utilizing three sonologists and previously described criteria combined with calculated volume changes demonstrated an overall sensitivity of 85 per cent and specificity of 94 per cent for the detection of acute rejection. The kidneys with acute rejection had an average volume increase of 73 per cent vs. 27 per cent for ATN patients and 24 per cent for normal patients, a statistically significant difference.

Osteolytic monostotic Paget's disease of the fifth lumbar vertebra. A case report.

Osteolytic monostotic Paget's disease or osteitis deformans of the fifth lumbar vertebra occurred in a 55-year-old woman. An isolated lytic process involving the entire vertebral body and posterior elements and an open biopsy showed extensive remodeling with cement lines, myelofibrosis, and osteoclastic resorption typical of Paget's disease.

Endorectal sonography in the evaluation of rectal and perirectal disease.

Endorectal sonography initially was developed for evaluation of the prostate and now has been adapted for evaluation of rectal and perirectal disease. We used endorectal sonography to evaluate a spectrum of diseases, including primary and recurrent rectal carcinoma, metastases, villous adenoma, leiomyosarcoma, endometriosis, sacrococcygeal teratoma, chordoma, retroperitoneal cystic hamartoma, pelvic lipomatosis, diverticulitis, and perirectal abscess. The technique has been useful in localization of perirectal abscesses and in sonographically guided biopsy of perirectal masses. Knowledge of normal sonographic anatomy of the rectum is essential in the evaluation of rectal and perirectal disease. In this essay, we describe the technique of endorectal sonography and illustrate the sonographic findings in a variety of diseases.