Characteristics of mutant mice (ICGN) with spontaneous renal lesions: a new model for human nephrotic syndrome

1989 ◽  
Vol 23 (2) ◽  
pp. 169-174 ◽  
Author(s):  
A. Ogura ◽  
T. Asano ◽  
J. Matsuda ◽  
K. Takano ◽  
M. Nakagawa ◽  
...  

Spontaneous nephrotic mice (ICGN mice), a new mutant strain of mouse from outbred ICR, were clinically, macroscopically, histologically and immunohistochemically studied to establish their value as a model for human nephrotic syndrome. Most of the affected mice developed proteinuria, hypoproteinaemia and hypercholesterolaemia, and some of them developed systemic oedema. A high concentration of blood urea nitrogen (BUN) and a low haematocrit value were also observed. The kidneys of severe cases showed a decrease in size and had a yellowish granular surface. These findings indicated that the mice were terminally affected by chronic renal in-sufficiency. Histopathology demonstrated glomerular lesions consisting of thickened basement membranes of the capillary loops with irregular spike-like protrusions and enlargement of the mesangium unaccompanied by cellular proliferation. The immunofluorescence technique revealed positive granular staining for IgA, IgG and IgM and to a lesser extent for C3 along the capillary loops in affected mice. The similarity between this spontaneous disease and human nephrotic syndrome caused by idiopathic glomerular lesions is discussed. ICGN mice may be a useful animal model for this human disease.

1959 ◽  
Vol 109 (6) ◽  
pp. 633-648 ◽  
Author(s):  
Sidney Rothbard ◽  
Robert F. Watson

Renal glomerular lesions were induced by rabbit serum containing antibody to rat collagen injected intravenously into rats prepared with subcutaneously administered Freund adjuvant. Neither the anti-collagen serum nor the adjuvant alone induced the lesion. The lesions were characterized by diffuse glomerular injury with swelling, shredding, and fusion of the basement membranes, crescent formation, cellular proliferation, numerous multinuclear giant cells, and capillary hyaline thrombi. Various rabbit antisera, including those against fish collagen or rat serum failed to induce the renal lesion when substituted for anti-rat collagen serum. Also, anti-rat collagen serum absorbed with its homologous antigen, native rat collagen, failed to induce the lesion. Although complete adjuvant, i.e. with mycobacteria, in which normal serum was incorporated enhanced the glomerular lesion which resulted from intravenous injection of anti-collagen serum, the incomplete adjuvant without serum was sufficient. Comparison of the renal lesions induced by anti-collagen serum with nephrotoxic nephritis induced in rats by rabbit anti-kidney serum showed that they differ histologically. Also the antisera used to produce these two renal lesions differ immunologically. Antibodies to normal rabbit serum developed in rats injected intravenously with rabbit anti-rat collagen serum after preparation with adjuvant, but not when adjuvant was omitted. The pathogenesis of the renal injury is discussed as a manifestation of an antigen-antibody reaction, with nephritis occurring only after the adjuvant-stimulated antibody to the rabbit globulin has been formed in the rat and has reacted with the rabbit anti-rat collagen already fixed by its homologous antigen in the kidney.


1986 ◽  
Vol 23 (6) ◽  
pp. 718-733 ◽  
Author(s):  
R. S. Jaenke ◽  
T. A. Allen

Membranous nephropathy, a disease syndrome characterized by severe proteinuria and often accompanied by the nephrotic syndrome, was identified in 29% of a population of 46 proteinuric dogs. Renal lesions were characterized by the presence of subepithelial immunoglobulin deposits distributed diffusely along the glomerular capillary wall. Advanced stages were associated with progressive thickening of capillary basement membranes and incorporation of the immune deposits. These changes were followed by either glomerulosclerosis or recovery. Characteristic morphologic stages were correlated with clinical pathologic findings which showed that the level of proteinuria, hypoalbuminemia, and consequent nephrotic syndrome was most severe in the initial stages of membranous nephropathy while the level of azotemia increased in the more advanced stages of the syndrome.


Medicines ◽  
2021 ◽  
Vol 8 (2) ◽  
pp. 12
Author(s):  
Arianna A. Tovar ◽  
Ian A. White ◽  
Alfonso L. Sabater

Blood derived products have become a valuable source of tissue for the treatment of ocular surface diseases that are refractory to conventional treatments. These can be obtained from autologous or allogeneic sources (patient’s own blood or from healthy adult donors/umbilical cord blood, respectively). Allogeneic cord blood demonstrates practical advantages over alternatives and these advantages will be discussed herein. Umbilical cord blood (UCB) can be divided, generally speaking, into two distinct products: first, mononuclear cells, which can be used in regenerative ophthalmology, and second, the plasma/serum (an acellular fraction), which may be used in the form of eyedrops administered directly to the damaged ocular surface. The rationale for using umbilical cord serum (UCS) to treat ocular surface diseases such as severe dry eye syndrome (DES), persistent epithelial defects (PED), recurrent epithelial erosions, ocular chemical burns, graft versus host disease (GVHD), among others, is the considerably high concentration of growth factors and cytokines, mimicking the natural healing properties of human tears. Allogeneic serum also offers the opportunity for therapeutic treatment to patients who, due to poor heath, cannot provide autologous serum. The mechanism of action involves the stimulation of endogenous cellular proliferation, differentiation and maturation, which is highly efficient in promoting and enhancing corneal epithelial healing where other therapies have previously failed.


1985 ◽  
Vol 89 (2) ◽  
pp. 69-73 ◽  
Author(s):  
Erna Alture-Werber ◽  
Stephen C. Edberg
Keyword(s):  

1996 ◽  
Vol 49 (2) ◽  
pp. 370-373 ◽  
Author(s):  
Norishi Ueda ◽  
Radhakrishna Baliga ◽  
Sudhir V. Shah

2020 ◽  
Vol 20 (4) ◽  
pp. e385-389
Author(s):  
Areeba Ejaz ◽  
Meher B. Ali ◽  
Fatima Siddiqui ◽  
Mashal B. Ali ◽  
Ammarah Jamal

Pierson syndrome is caused by mutations in the laminin β2 gene causing absent β2 laminin, which is a normal component of the basement membranes of the mature glomerulus, structures in the anterior eye and neuromuscular junctions. The mutations manifest as congenital nephrotic syndrome and microcoria which are characteristic ocular features of this disease. These mutations may also result in neurological abnormalities such as hypotonia and psychomotor retardation. We report a two-month old boy who presented to the Pediatrics Department of Dr. Ruth K. M. Pfau Civil Hospital, Karachi, Pakistan, in 2015, with the typical features of microcoria and congenital nephrotic syndrome. The hypocalcaemia, hypoproteinaemia and probable immunocompromised state consequent to nephrotic syndrome resulted in seizures, hypothyroidism and urosepsis. Despite being treated aggressively with high dose antibiotics, ionotropic support, angiotensin-converting enzyme inhibitors, thyroxine replacement and nutritional support, the infant died due to significant multiorgan disease including renal failure and septic shock. Keywords: Pierson Syndrome; Microcoria and Congenital Nephrotic Syndrome; Congenital Microcoria; Hypothyroidism; Septic Shock; Case Report; Pakistan.


2021 ◽  
pp. 13-14
Author(s):  
VPS Punia ◽  
Apoorva Shetty ◽  
Prashant Prashant ◽  
Akash Bharti ◽  
Praveen Raman Mishra ◽  
...  

Psoriasis is known to cause chronic inammatory disorder of the skin through an immune mediated mechanism, it may be complicated by different types of glomerular lesions. Three different mechanisms have been implicated by which psoriasis can cause renal damage: immune-mediated renal damage, drug-related renal damage and chronic renal damage. This report presents a case of 35 years old male patient with extensive psoriasis, who presented to our hospital with nephrotic syndrome


PEDIATRICS ◽  
1960 ◽  
Vol 25 (6) ◽  
pp. 967-976
Author(s):  
R. A. Parker ◽  
Carolyn F. Piel

The clinical course of nephrosis in five infants with onset of disease before 7 months of age is presented, together with evaluation of renal lesions seen at necropsy. The problems of the management of nephrosis susceptibility to infection and water and electrolyte imbalance were found to be exaggerated by the young age of the patients. The renal pathology observed in these five infants consisted of persistence of immature glomeruli and dilatation of the tubules in the cortical area. Later, the immature glomeruli and associated tubules appear to atrophy and the remaining glomeruli to hypertrophy. Long-term adrenocorticosteroid therapy seems to be contraindicated, not only on the basis of the pathologic changes, but because it greatly exaggerates the problems of management and does not effect a remission of the disease.


1981 ◽  
Vol 18 (1) ◽  
pp. 92-109 ◽  
Author(s):  
A. J. Spencer ◽  
N. G. Wright ◽  
I. Macmillan

The role of fibrin in the pathogenesis of renal glomerular scarring in the dog was studied. Fibrin deposition, resulting from disseminated intravascular coagulation, was induced by intravenous injection of Liquoid (sodium polyanetholsulphonate). Thirty-eight puppies were killed from 30 minutes to 39 days after treatment, and the renal lesions examined by light, electron and immunofluorescence microscopy. The major acute lesions in the glomeruli were capillary thrombosis, mesangial and endothelial cell swelling and phagocytosis of fibrin, polymorphonuclear leukocyte infiltration and necrosis. Animals that recovered from this acute phase had focal glomerular scars. Affected glomeruli showed combinations of mesangial enlargement, focal tuft hypercellularity, collagen formation, thickening, wrinkling and duplication of the glomerular basement membranes, and some capsular adhesions. These observations indicate that fibrin deposition can be an important mechanism in glomerular scarring in the dog.


1974 ◽  
Vol 11 (6) ◽  
pp. 506-514 ◽  
Author(s):  
C. F. Simpson ◽  
B. M. Gebhardt ◽  
R. E. Bradley ◽  
R. F. Jackson

The kidneys of seven dogs with natural infections of heartworm disease, four dogs with experimental infections, and six control (uninfected) dogs raised in isolation were examined by light, fluorescent, and electron microscopy. Swelling and fragmentation of the basement membranes accompanied by denudations of endothelial cells and fusion and atrophy of foot processes occurred in the glomeruli of dogs with heartworm disease, but not in control dogs. The severity of tissue damage correlated with the degree of microfilaremia. There was no evidence that the glomerular lesions were caused by immune complexes deposited in the basement membranes; and the alterations of glomerular capillaries were probably caused by motile microfilariae in the capillaries.


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