Podocyte Infolding Glomerulopathy Masquerading as Membranous Nephropathy - A Shared Pathogenesis?

Podocyte infolding glomerulopathy (PIG) is a rare pathological entity, diagnosed by electron microscopic demonstration of diffuse infolding of the podocytes into the glomerular basement membranes. We report the first case from United Kingdom exhibiting typical ultrastructural features of PIG in a male with Type II diabetes mellitus, hypertension and common variable immune deficiency. Renal biopsy revealed phospholipase A2 receptor (PLA2R) immunostain positive membranous nephropathy (MN) but no serum PLA2R antibodies.

XFVM modelling of fracture aperture induced by shear and tensile opening.

In reservoir simulation, it is important to understand the mechanical behaviour of fractured rocks and the effect of shear and tensile displacements of fractures on their aperture. Tensile opening directly enhances the fracture aperture, whereas shear of a preexisting rough-walled fracture creates aperture changes dependent on the local stress state. Since fracture dilatation increases reservoir permeability, both processes must be included in a realistic and consistent manner into the mechanical reservoir simulation model.

Extra-corporeal non-liver transplant therapies for acute liver failure: Focus on plasma exchange and continuous renal replacement therapy.

The acute inflammatory milieu in patients with acute liver failure (ALF) results in 'toxic' blood in these patients. In vitro experiments have shown that the plasma obtained from ALF patients is toxic to rabbit hepatocytes and inhibits regeneration of rat hepatocytes. Treatments such as plasma exchange and continuous renal replacement therapy to cleanse the blood have improved survival in ALF patients.

How to use the Bristol Stool Chart in childhood constipation.

Constipation in children is common and is a frequent cause for healthcare attendances in both primary and secondary care. The Bristol Stool Chart has become ubiquitous as an aid in the diagnosis and treatment of constipation, but many clinicians may not be aware of its origins or strengths and weaknesses. In this article, we outline the history and rationale behind the development of the Bristol Stool Chart and how it should be used in childhood constipation.

Crystals, crystals everywhere but not a clue till late… Light chain crystalline proximal tubulopathy with concomitant myeloma cast nephropathy.

The renal diseases commonly associated with myeloma include primary amyloidosis, cast nephropathy, and light chain deposition disease. Less frequent forms of renal involvement encountered in the course of myeloma are crystalline and non-crystalline proximal tubulopathies, neoplastic plasma cell infiltration, and immunoglobulin crystallization in interstitial histiocytes and glomerular cells including podocytes. Light chain proximal tubulopathy (LCPT) caused by aggregation of non-crystalline and rarely crystalline deposits of monoclonal light chains in the cytoplasm of proximal tubular epithelial cells, accounts for less than 5% of monoclonal gammopathy-associated kidney diseases.

Transitions between nanomechanical and continuum mechanical contacts: new insights from liquid structure.

The use of continuum mechanics to describe contacts involving nanoscale and atomic interactions has been one of the key controversies in nanoscience, tribology, and petrophysical and geological studies. By applying a novel nonequilibrium molecular dynamics scheme to wet quartz contacts, this study revealed the key transitions between continuum electrostatic, nanomechanical and Hertzian contact behaviors at around one nm of surface separation, which results in critical contact pressure fluctuations between -30 and 100 MPa. Using a novel liquid-structure analysis scheme based on the spatial distribution of water molecules, the nanomechanical behavior was found to originate from the collapse and localization of layers of water molecules.

Nephropathic cystinosis presenting with uveitis: Report of a "Can't See, Can't Pee" situation.

Nephropathic cystinosis is a rare autosomal recessive lysosomal disease characterized by accumulation of pathognomonic cystine crystals in renal and other tissues of the body. Cystinosis is caused by mutant cystinosin, the cystine transport protein located in lysosomal membranes, leading to systemic deposits of cystine and resultant end organ damage. Cystinosis is rarer in Asians than Caucasians with only a handful of cases reported from India to date.

Protocol and rationale for the first South Asian 5-year prospective longitudinal observational cohort study and biomarker evaluation investigating the clinical course and risk profile of IgA nephropathy: GRACE IgANI cohort.

IgA nephropathy (IgAN) is the most common primary glomerulonephritis and an important cause of end-stage kidney disease. Unlike the slowly progressive course seen among Caucasian and East Asian subjects (actuarial survival 80-85% over 10 years), in India about 30-40% of patients have nephrotic syndrome and renal dysfunction at presentation and a 10-year renal survival of 35%, as reported from a retrospective registry. These observations cannot be entirely attributed to a lack of uniform screening protocols or late referral and attest to the probability that IgAN may not be the same disease in different parts of the world.

Fibrillary glomerulonephritis in a human immunodeficiency virus-positive, hepatitis C-negative Indian patient: Expanding the profile of renal involvement in human immunodeficiency virus infection.

Highly active anti retroviral therapy (HAART) has dramatically improved life expectancy of human immunodeficiency virus (HIV) infected patients, converting HIV infection into a chronic illness with associated changes in its attendant renal complications. The past two decades have witnessed a decrease in the prevalence of HIV associated nephropathy (HIVAN), traditionally considered to be the hall mark of renal involvement in HIV infection. Simultaneously a host of other glomerular and tubulo-interstitial diseases have emerged, expanding the spectrum of HIV associated renal diseases, predominant among which is HIV associated immune complex mediated kidney diseases (HIVICK).

Unmasking and successful management of light chain deposition disease of kidney in pregnancy: a complex case, mirroring the complex needs of pregnancy with kidney disease in India.

Pregnancy offers a precious window of opportunity to diagnose previously undetected or new onset kidney diseases in emerging countries like India, where access to medical, educational and health care facilities are not equitably distributed across varied sections of society. We report a case of a 33 year-old primi gravida who had a successful pregnancy following what was initially considered to represent preeclampsia at 38 weeks of gestation, in whom a subsequent kidney biopsy for persistence of pregnancy-related acute kidney injury (Pr-AKI) revealed light chain deposition disease (LCDD). The etiological evaluation of LCDD led to the detection of an underlying plasma cell dyscrasia which was treated effectively with chemotherapy and autologous stem cell transplant.

Immunoglobulin G4-related tubulointerstitial nephritis: A not to be missed diagnosis.

Immunoglobulin G4-related tubulointerstitial nephritis (IgG4-TIN) is a newly recognized clinicopathological entity characterized by a dense interstitial infiltrate of IgG4-positive plasma cells accompanied by fibrosis and obliterative phlebitis causing acute or chronic renal dysfunction amenable to corticosteroid therapy. IgG4-TIN is the dominant manifestation of renal involvement in IgG4-related disease (IgG4-RD) which is a novel, immune-mediated, fibroinflammatory and multiorgan disorder. We describe a case of IgG4-TIN with isolated renal involvement in an elderly male patient with poor response to corticosteroid therapy.

Spectrum of biopsy proven renal disease in South Asian children: Two decades at a tropical tertiary care centre.

Aim: We report findings from a large single centre paediatric renal biopsy cohort in South Asia.

Methods: We analyzed all renal biopsies performed on children aged ≤18 years between 1996 and 2015 at our centre. The clinical characteristics and histological diagnosis pertaining to each case, distribution of renal diseases in children with various clinical presentations, and changes in the pattern of kidney disease during the study period were analyzed.

Cancer stem cells in hepatocellular carcinoma--an immunohistochemical study with histopathological association.

Background & Objectives: Cancer stem cells (CSCs) may be responsible for tumour recurrence and resistance to chemotherapy in hepatocellular carcinoma (HCC). This study was carried out to evaluate the association between histological parameters and liver CSCs (LCSC) in HCC, and to compare distribution of liver CSCs in HCC associated with and without hepatitis B virus (HBV) infection.

Methods: Seventy nine tumours (49 surgical resections from 46 patients, and 30 from autopsy) were reviewed.

Immunoglobulin G4-related tubulointerstitial nephritis associated with interstitial pulmonary disease: Report of a case with review of literature.

Immunoglobulin G4-related disease (IgG4-RD) is an emerging clinicopathological entity. Renal involvement is dominated by tubulointerstitial nephritis (TIN) with IgG4-positive plasma cells and fibrosis. IgG4-RD commonly affects middle-aged to elderly men with accompanying extra-renal lesions such as sialadenitis, lymphadenopathy, or type 1 autoimmune pancreatitis, all of which respond favorably to corticosteroid therapy.

Collapsing glomerulopathy following anabolic steroid use in a 16-year-old boy with IgA nephropathy.

Collapsing glomerulopathy (CG) is a proliferative podocytopathy, increasingly recognized in a variety of disease conditions. We report a case of CG in a 16-year-old boy with IgA nephropathy (IgAN) who presented with acute kidney injury, marked proteinuria and hypertension following a short period of anabolic steroid use. Although CG has been associated with long-term anabolic steroid use among body builders, there is no data on the effect of anabolic steroid use in persons with underlying renal disease like IgAN.

Recurrent episodic acute kidney injury as presenting manifestation of mitochondrial myopathy.

Mitochondrial cytopathies (MC) are a rare heterogenous group of disorders with frequent multisystem involvement including uncommon renal manifestations. Acute kidney injury (AKI) as the primary manifestation of MC is extremely rare. Here, we report a case of recurrent episodic AKI in an adult male who was subsequently diagnosed to have mitochondrial disease.

Role of geomechanically grown fractures on dispersive transport in heterogeneous geological formations.

A second order in space accurate implicit scheme for time-dependent advection-dispersion equations and a discrete fracture propagation model are employed to model solute transport in porous media. We study the impact of the fractures on mass transport and dispersion. To model flow and transport, pressure and transport equations are integrated using a finite-element, node-centered finite-volume approach.

Numerical study of the effects of particle shape and polydispersity on permeability.

We study through numerical simulations the dependence of the hydraulic permeability of granular materials on the particle shape and the grain size distribution. Several models of sand are constructed by simulating the settling under gravity of the grains; the friction coefficient is varied to construct packs of different porosity. The size distribution and shapes of the grains mimic real sands.

Electrokinetic coupling in unsaturated porous media.

We consider a charged porous material that is saturated by two fluid phases that are immiscible and continuous on the scale of a representative elementary volume. The wetting phase for the grains is water and the nonwetting phase is assumed to be an electrically insulating viscous fluid. We use a volume-averaging approach to derive the linear constitutive equations for the electrical current density as well as the seepage velocities of the wetting and nonwetting phases on the scale of a representative elementary volume.