Striking and widespread microglial activation in the brains of Southdown lambs with type II glucocerebrosidosis (neuronopathic Gaucher disease).

Glucocerebrosidosis (termed Gaucher disease in humans) is a lysosomal storage disease, caused by a deficiency of the enzyme glucocerebrosidase, which results in accumulation of the glycolipid substrate glucocerebroside in the macrophage-monocyte system. Three principal forms are recognized in humans, two being neuronopathic and resulting in neurodegeneration. Only two spontaneously arising cases have been described in domestic animals, one in a dog and the other in a flock of Southdown sheep.

Large Multinucleated Variant Endothelial Cells in Allograft Kidney Microvasculature: A Biopsy Series.

There are few published studies examining cytomorphologic alterations in endothelial cells in human tissue. One fascinating but largely unexplored endothelial morphologic variant is large multinucleated variant endothelial cells (MVECs). To our knowledge, there are no published reports of MVECs identified in the kidney.

Atypical Nodular Pulmonary Kappa Light-Chain Deposition.

We report a case of an incidental positron emission tomography avid right middle lobe lesion which was increasing in size. Due to concerns regarding malignancy, the patient underwent right middle lobectomy. Microscopic examination showed a 12 × 10 × 10 mm poorly circumscribed lesion composed of eosinophilic material.

A case of monoclonal gammopathy of renal significance presenting as atypical amyloidosis with IgA lambda paraproteinemia.

Monoclonal gammopathy of renal significance is defined as any B cell or plasma cell clonal lymphoproliferation which neither causes tumor complications nor meets any current hematological criteria for specific therapy, with one or more kidney lesions related to the produced monoclonal immunoglobulin, such as amyloidosis. A 50-year-old male presented with heavy proteinuria and blood tests showing IgA and Lambda paraproteinemia. Light microscopy showed mesangial eosinophilic ground substance extending into the capillary loops, and positive staining within the glomeruli and vessel walls for amyloid P immunohistochemistry was also noted.

Ultrastructural identification of a proximal tubulopathy without crystals in a relapsed multiple myeloma patient.

A multiple myeloma patient, who had been treated with a hematopoietic stem cell transplant, underwent a renal biopsy for investigation of a possible relapse of disease as indicated by increased serum creatinine and positive urinary Bence-Jones protein containing increased kappa light chain. Paraprotein-related renal disease was not evident by light microscopy or immunofluorescence microscopy however electron microscopy demonstrated a proximal tubulopathy with intracytoplasmic non-crystalline inclusions. The ultrastructural findings suggested possible end-organ involvement by the disease and follow-up studies subsequently revealed a relapsed multiple myeloma in the patient.

An unusual pattern of peritubular capillary injury involving apoptosis in a renal transplant patient.

Microvascular injury is an important factor in renal allograft survival. Repeated episodes of endothelial injury from chronic antibody-mediated rejection typically manifest at the ultrastructural level as circumferential multilayering of remodeled glomerular basement membrane material and peritubular capillary basal lamina. In contrast to this typical pattern of microvascular injury, a renal transplantation case is presented in which focally dilated and multilayered segments of peritubular capillary basal lamina bearing lipid droplets were interspersed with ultrastructurally normal unilayered segments of basal lamina devoid of lipid droplets.

Structure and Function of the Kidney in Septic Shock. A Prospective Controlled Experimental Study.

Rationale: It is unclear how septic shock causes acute kidney injury (AKI) and whether this is associated with histological change.

Objectives: We aimed to determine the nature and extent of changes in renal structure and function over time in an ovine model of septic shock.

Methods: Fifteen sheep were instrumented with a renal artery flow probe and renal vein cannula.

Electron-dense deposit in renal transplant patients on eculizumab may be drug-derived.

Eculizumab is a monoclonal antibody that inhibits the conversion of complement protein C5 to C5a and C5b. Eculizumab has been used to treat some disorders of complement regulation owing to its ability to inhibit terminal complement activation. The efficacy of eculizumab in reducing complement-mediated microvascular injury in renal allografts is currently the subject of trials.

Angiotensin II type 1 receptor antibody precipitating acute vascular rejection in kidney transplantation.

Atypical non HLA antibodies are increasingly recognised as causes of immunological injury in allotransplantation. In this report we describe a non HLA sensitized male renal allograft recipient who developed acute vascular rejection on a "for cause" biopsy (Banff v2, g2, ptc 3) at day 4 post first renal allograft in the presence of elevated angiotensin II type 1 receptor antibodies (AT1R-Ab level 14.1).

Spontaneous glomerular mesangial lesions in common marmoset monkeys (Callithrix jacchus): a benign non-progressive glomerulopathy.

Background: Common marmosets are known to develop an IgM glomerulopathy, which has been linked with 'wasting marmoset' syndrome. This study investigated renal pathology in a colony of marmosets, with and without weight loss.

Methods: Renal histology, immunofluorescence, and electron microscopy were performed on marmosets euthanized for research or for weight loss.

Increased expression of the glucose-responsive gene, RCAN1, causes hypoinsulinemia, β-cell dysfunction, and diabetes.

RCAN1 is a chromosome 21 gene that controls secretion in endocrine cells, regulates mitochondrial function, and is sensitive to oxidative stress. Regulator of calcineurin 1 (RCAN1) is also an endogenous inhibitor of the protein phosphatase calcineurin, the inhibition of which leads to hypoinsulinemia and diabetes in humans and mice. However, the presence or the role of RCAN1 in insulin-secreting β-cells and its potential role in the pathogenesis of diabetes is unknown.

Disseminated microsporidiosis with Encephalitozoon species in a renal transplant recipient.

To our knowledge, 5 cases of disseminated microsporidiosis with Encephalitozoon species have been reported worldwide in transplant recipients. George et al. present the first such case in Australia, to be reported and treated with good clinical recovery.

Ultrastructural analysis, zinc transporters, glucose transporters and hormones expression in New world primate (Callithrix jacchus) and human pancreatic islets.

The New world primates (NWP) Callithrix jacchus separated from man approximately 50 million years ago and is a potential alternative small non-human primate model for diabetes research. Ultrastructure, and gene expression of pancreatic islets and the recently described diabetes auto antigenic zinc transporters families in human, NWP and pig pancreas were studied. Morphologically NWP islets were larger than pig islets and similar in size to human islets.

Ultrastructural observations in a case of BK virus nephropathy with viruses in glomerular subepithelial humps.

BK virus nephropathy is a known cause of renal transplant dysfunction and failure. The disease is identified by examination of kidney biopsy tissue utilizing histopathological techniques. Ultrastructural examination of two glomeruli revealed pathology within one glomerulus.