Biomedical subjects
J Churg
Publications and source records attributed to J Churg.
Nomenclature of vasculitic syndromes: a historical perspective.
Since the original description of periarteritis nodosa (PAN) in 1866, the number of recognized forms of so-called idiopathic vasculitis has risen to about a dozen. Their relation to each other and their nature remain unclear, although the recent discovery of antineutrophil cytoplasmic autoantibodies (ANCA) promises a clue to the origin of at least some of them, particularly Wegener's granulomatosis (WG) and perhaps also Churg-Strauss syndrome (CSS). On the other hand, PAN appears to be a nonspecific syndrome of various etiologies.
Sudden death due to thrombotic thrombocytopenic purpura and HIV infection.
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Mesangiocapillary glomerulonephritis in Down's syndrome.
The clinical and pathologic features of progressive renal disease in 4 patients with Down's syndrome are described. All patients were male, between 20 and 30 years of age at the time of clinical presentation. Three out of 4 had proteinuria, and 2 had hematuria. Serologic tests for hepatitis B virus infection and antinuclear antibodies performed in 2 patients were negative. Examination of renal tissue from biopsy and/or from autopsy revealed mesangiocapillary glomerulonephritis (MCGN), type 1. While an increased incidence of congenital heart disease and acute leukemias has been documented in Down's syndrome, an association with MCGN has not been reported previously to our knowledge. This probably represents a form of idiopathic MCGN and may be related to the long survival of these individuals.
Correlation of glomerular basement membrane alterations with clinical data in progressive hereditary nephritis (Alport's syndrome).
Electron microscopic examination of glomerular basement membrane (GBM) was performed in 19 patients whose morphological changes as well as clinical features indicated the diagnosis of progressive hereditary nephritis (Alport's syndrome). The percentage of characteristically thickened and split and of thin GBM portions was determined in all the cases. The clinical course was more severe in males, which corresponded to higher rate of GBM alterations. In males, 58% of GBM was thickened and split and 24% was thin, while in females, the reverse was true, 28% was split and 48% of GBM was thin. There was a positive correlation of the split lesions and age in males, but not in females. The degree of splitting was directly proportional to the grade of proteinuria, while GBM thinning did not significantly correlate with proteinuria.
Acute hyperuricemic nephropathy and renal failure after transplantation.
This report describes a patient who was treated for rejection of a cadaveric renal allograft with a variety of drugs, including the continuous administration of ciclosporin over a period of 16 months. The patient developed hyperuricemia, attacks of gout and finally a rapidly progressing renal failure 17 months after transplantation. The removed transplanted kidney showed extensive tubular dilatation, intratubular deposits of uric acid crystals and characteristic granulomas. There was also morphologic evidence of transplant glomerulopathy, as well as scattered linear parenchymal (cortical?) scars of the type seen in mild chronic ciclosporin toxicity. Both of these changes undoubtedly contributed to the reduction of renal reserve. However, we propose that prolonged continuous use of ciclosporin was the main factor in the development of hyperuricemia and obstructive hyperuricemic nephropathy and renal failure in this patient. To our knowledge cases of this nature have not been previously reported.
Morphometric analysis of glomerular basement membranes (GBM) in thin basement membrane disease (TBMD).
We measured the thickness of glomerular basement membrane in 46 patients with thin basement membrane disease (TBMD), (age range 15-50 years, almost equal M:F ratio), and compared with that in a control group of 5 patients (age range 5-38 years) with normal glomerular morphology. The measurements of glomerular basement membrane taken from electron micrographs (magnification x 12,500) were analyzed using an interactive image analysis system assembled around an INTEL 10 microcomputer, with a high resolution touch sensitive screen as the interactive peripheral. Calculation was done by printing on an electron micrograph a grating replica (21,600 lines/cm), with the same magnification as the electron micrographs of the glomeruli and calibrating the arithmetic (AM) and harmonic (HM) mean for each case. Comparing the results of TBMD cases (AM 129-202 nm; HM 128-213 nm) with those of the control group consisting of 5 cases of "minimal change nephrotic syndrome" (AM 287-317 nm; HM 300-333 nm) it was found that GBM in TBMD is remarkably thin. The thinning was caused mainly by the decreased width of the lamina densa (TBMD group: 71.4-147.0 nm; HM 72.4-154.4 nm in comparison with the control group: AM 174.4-235.5 nm; HM 184.2-249.6 nm). This finding allows us to differentiate thin basement membrane disease from other glomerulopathies presenting primarily with isolated or recurrent hematuria.
Kidney biopsy in SLE. I. A clinical-morphologic evaluation.
The relationship between renal morphology and clinical disease was analysed in 148 patients with SLE attending a lupus clinic. Patients were not selected for renal disease. Renal tissue was assessed according to the World Health Organization classification of lupus nephritis, the presence of active and chronic lesions was recorded and disease activity was measured according to a standard protocol. All sections of the classification were represented in this group of patients. Active and chronic lesions were more likely to occur among patients with Class III/IV (proliferative glomerulonephritis), than in any other category. Patients with Class III/IV biopsy were more likely to have evidence of clinical renal disease than patients in Class II (mesangial). However, almost half of the Class II patients had some evidence of renal disease, including elevated serum creatinine, as well as important non-glomerular lesions. Without biopsy they might have been thought to have proliferative lesions and been treated more aggressively. Two patients with proliferative glomerulonephritis had no clinical evidence of renal disease. Thus, at the time of biopsy results renal histological examination did not uniformly correlate with clinical renal disease.
Idiopathic and secondary vasculitis: a review.
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Immunohistochemical localization of renin in end-stage kidneys.
Hypertension in chronic renal failure is usually due to excessive accumulation of salt and water. In some cases, sodium and volume depletion by dialysis fail to reduce the high BP, and plasma renin activity tends to be higher. We performed a semiquantitative analysis of the immunohistochemical distribution of renin in the kidneys of ten patients with end-stage renal disease and hypertension using a specific antihuman renin antibody and a peroxidase-antiperoxidase technique on paraffin sections of nephrectomy and/or autopsy specimens. In five cases with severe, dialysis-resistant hypertension, the degree of immunoreactivity was most striking, exceeding that found in renovascular hypertension and present in arterioles at a distance from the glomeruli. Three cases of advanced diabetic glomerulosclerosis consistently showed minimal immunoreactivity. We conclude that renin often can be detected immunologically in the kidney of patients with chronic renal failure and hypertension, but its pathophysiological role will require further study.
Nephropathy after combination chemotherapy of lymphoma.
Two patients with malignant (Hodgkin's and non-Hodgkin's lymphoblastic type) lymphoma developed chronic renal failure following prolonged combination chemotherapy and a course of radiotherapy to the para-aortic lymph nodes. The individual drugs used in both patients are believed to have very low or no nephrotoxic potential. The kidneys were shielded in a standard manner and no more than 200 rads reached the organs. On biopsy (1 patient) and at autopsy (both patients), the kidneys demonstrated diffuse tubular atrophy, extensive interstitial fibrosis and focal glomerular changes with crescents. There were also severe sclerotic changes in the arteries. The changes bore some similarity to, but were not identical with those seen with radiation nephritis. They probably represent a radiomimetic effect produced by prolonged and repeated combination chemotherapy, perhaps potentiated by the small amount of ionizing radiation.
Case report of congo red-negative amyloidosis-like glomerulopathy.
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Renal papillary necrosis: relapsing form associated with alcoholism.
Described is a patient with recurrent renal papillary necrosis and chronic alcoholism as a possible precipitating factor. The case also demonstrates excellent recovery of renal function, despite considerable scarring of the parenchyma secondary to repeated episodes of sloughing of the papillae.
Use of lectins in the study of histogenesis of renal cysts.
Two peroxidase-labeled lectins, from Tetragonolobus lotus and from peanut (Arachis hypogaea), were used in the study of five cases of polycystic kidney of different types. The two lectins bind with high selectivity of proximal tubules and collecting ducts, respectively. It was found that in infantile polycystic kidneys the cyst walls consistently stained with peanut lectin but not with Tetragonolobus lectin, confirming their origin from collecting ducts only. In adult polycystic kidneys there was mixture of two types of cysts: one positive for peanut, the other positive for Tetragonolobus. The two lectins were, however, mutually exclusive. Moreover, some cysts did not stain for either lectin. The findings suggest that in this type of disease a cyst can originate from any portion of the nephron. The data are also consistent with reports in the literature which used microdissection techniques or chemical analysis of the cyst content. The use of lectins will probably be useful in investigating other types of cystic kidneys, such as dysplastic kidneys and experimental models. The technique is relatively easy to use, but must be applied under strictly controlled conditions, especially when using surgical or postmortem material.
Renal involvement in thrombotic microangiopathies.
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Diffuse collagen disease.
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