scholarly journals Focal Xanthogranulomatous Pyelonephritis in Brachydactyly Mental Retardation Syndrome (2q37 Deletion Syndrome)

2019 ◽  
Vol 09 (02) ◽  
pp. 114-116
Author(s):  
Esra Nagehan Akyol Onder ◽  
Mine Ozkol ◽  
Nalan Nese ◽  
Can Taneli ◽  
Osman Orkun Cankorur ◽  
...  
Keyword(s):  
Mental Retardation ◽  
Renal Fibrosis ◽  
Granulomatous Inflammation ◽  
Rare Condition ◽  
Renal Parenchyma ◽  
Xanthogranulomatous Pyelonephritis ◽  
Deletion Syndrome ◽  
Inflammatory Infiltration ◽  
Small Deletion ◽  
Foamy Macrophages

AbstractXanthogranulomatous pyelonephritis (XGP) is characterized by destruction of the renal parenchyma and granulomatous inflammation with lipid-laden foamy macrophages as well as inflammatory infiltration and intensive renal fibrosis. It generally occurs in adults, especially those in the fifth and sixth decades of life, but is occasionally seen in children as well. Brachydactyly mental retardation (BDMR) syndrome (OMIM 600430) is caused by a small deletion of chromosome 2q37 and is a rare condition, with roughly 100 cases reported worldwide. Here, we describe the case of a patient with deletion of chromosome 2q37, which is known as the BDMR syndrome, and XGP.

scholarly journals Xanthogranulomatous Pyelonephritis Associated with Hepatic Dysfunction in Pregnancy

2015 ◽  
Vol 2015 ◽  
pp. 1-4
Author(s):  
L. Ferreira ◽  
C. Oliveira ◽  
C. Cruz ◽  
A. Pacheco
Keyword(s):  
Pregnant Woman ◽  
Rare Disease ◽  
Hepatic Dysfunction ◽  
Rare Event ◽  
Renal Parenchyma ◽  
Xanthogranulomatous Pyelonephritis ◽  
Normal Renal Parenchyma ◽  
Foamy Macrophages ◽  
In Pregnancy ◽  
Total Nephrectomy

Xanthogranulomatous pyelonephritis is a rare disease characterised by the replacement of normal renal parenchyma by foamy macrophages. The only treatment for this type of pyelonephritis is of a surgical nature with partial or total nephrectomy. The occurrence of xanthogranulomatous pyelonephritis during pregnancy is a rare event (with only 6 cases described in the literature). We report a case of xanthogranulomatous pyelonephritis in a 32-week pregnant woman associated with hepatic dysfunction.

Xanthogranulomatous pyelonephritis due to Aspergillus in a non-diabetic older patient

2021 ◽  
Vol 14 (9) ◽  
pp. e245067
Author(s):  
Neha Khilar ◽  
Navneet Gupta ◽  
Prabha Adhikari ◽  
Abhijith Rajaram Rao
Keyword(s):  
Organ Transplant ◽  
Granulomatous Inflammation ◽  
Rare Condition ◽  
Xanthogranulomatous Pyelonephritis ◽  
Transplant Recipients ◽  
Older Patient ◽  
Unique Case ◽  
Left Flank ◽  
Long Duration ◽  
Left Flank Pain

Xanthogranulomatous pyelonephritis is a rare condition characterised by destructive granulomatous inflammation of renal parenchyma. Primary renal Aspergillosis has been reported in patients with immunocompromised states such as diabetes, retroviral disease, organ transplant recipients, etc. We present a unique case of an older adult in his early 60s, presenting with fever and left flank pain with renal angle tenderness, diagnosed with primary renal aspergillosis with xanthogranulomatous pyelonephritis. These symptoms resolved with a long duration of antifungal (itraconazole) therapy and nephrectomy. The unique features are the development of fungal pyelonephritis in the absence of any immunocompromising conditions and the development of xanthogranulomatous changes with no risk factors.

scholarly journals Cellular and Molecular Mechanisms of Kidney Injury in 2,8-Dihydroxyadenine Nephropathy

2020 ◽  
Vol 31 (4) ◽  
pp. 799-816 ◽  
Author(s):  
Barbara Mara Klinkhammer ◽  
Sonja Djudjaj ◽  
Uta Kunter ◽  
Runolfur Palsson ◽  
Vidar Orn Edvardsson ◽  
...  
Keyword(s):  
Clinical Relevance ◽  
Molecular Mechanisms ◽  
Granulomatous Inflammation ◽  
Kidney Injury ◽  
Rare Condition ◽  
Therapeutic Interventions ◽  
Renal Tubules ◽  
Rodent Models ◽  
Adenine Phosphoribosyltransferase ◽  
Reparative Process

BackgroundHereditary deficiency of adenine phosphoribosyltransferase causes 2,8-dihydroxyadenine (2,8-DHA) nephropathy, a rare condition characterized by formation of 2,8-DHA crystals within renal tubules. Clinical relevance of rodent models of 2,8-DHA crystal nephropathy induced by excessive adenine intake is unknown.MethodsUsing animal models and patient kidney biopsies, we assessed the pathogenic sequelae of 2,8-DHA crystal-induced kidney damage. We also used knockout mice to investigate the role of TNF receptors 1 and 2 (TNFR1 and TNFR2), CD44, or alpha2-HS glycoprotein (AHSG), all of which are involved in the pathogenesis of other types of crystal-induced nephropathies.ResultsAdenine-enriched diet in mice induced 2,8-DHA nephropathy, leading to progressive kidney disease, characterized by crystal deposits, tubular injury, inflammation, and fibrosis. Kidney injury depended on crystal size. The smallest crystals were endocytosed by tubular epithelial cells. Crystals of variable size were excreted in urine. Large crystals obstructed whole tubules. Medium-sized crystals induced a particular reparative process that we term extratubulation. In this process, tubular cells, in coordination with macrophages, overgrew and translocated crystals into the interstitium, restoring the tubular luminal patency; this was followed by degradation of interstitial crystals by granulomatous inflammation. Patients with adenine phosphoribosyltransferase deficiency showed similar histopathological findings regarding crystal morphology, crystal clearance, and renal injury. In mice, deletion of Tnfr1 significantly reduced tubular CD44 and annexin two expression, as well as inflammation, thereby ameliorating the disease course. In contrast, genetic deletion of Tnfr2, Cd44, or Ahsg had no effect on the manifestations of 2,8-DHA nephropathy.ConclusionsRodent models of the cellular and molecular mechanisms of 2,8-DHA nephropathy and crystal clearance have clinical relevance and offer insight into potential future targets for therapeutic interventions.

scholarly journals DIFFUSE XANTHOGRANULOMATOUS PYELONEPHRITIS OCCURRING IN A NON-FUNCTIONAL KIDNEY - A REPORT OF TWO CASES

2012 ◽  
Vol 02 (02) ◽  
pp. 54-56
Author(s):  
Harish S. Permi ◽  
Rajeev T. P. ◽  
Prajwal Ravinder ◽  
Sajitha N. ◽  
Panna Hegde
Keyword(s):  
Urinary Tract Obstruction ◽  
Renal Calculus ◽  
Xanthogranulomatous Pyelonephritis ◽  
Renal Neoplasm ◽  
Radiographic Appearance ◽  
Formidable Challenge ◽  
Adjacent Structures ◽  
Immunocompromised State ◽  
Foamy Macrophages ◽  
Functioning Kidney

AbstractXanthogranulomatous pyelonephritis is a rare debilitating illness resulting in focal or diffuse renal destruction. It is characterized pathologically by lipid-laden foamy macrophages which share many characteristics with true renal neoplasm in terms of its radiographic appearance and ability to involve adjacent structures or organs. It affects non-functioning kidney and is often associated with urinary tract obstruction, infection, nephrolithiasis, diabetes, and immunocompromised state. The treatment is almost universally extirpative and can pose a formidable challenge to the surgeon. We report two cases of diffuse Xanthogranulomatous pyelonephritis occurring in a non-functioning kidney of 38 year old diabetic male and 51 year old male with renal calculus.

scholarly journals Renal Collision Tumor in Association with Xanthogranulomatous Pyelonephritis

2011 ◽  
Vol 1 ◽  
pp. 9 ◽  
Author(s):  
Jennifer Rothschild ◽  
Shweta Bhatt ◽  
Vikram S. Dogra
Keyword(s):  
Squamous Cell Carcinoma ◽  
Cell Carcinoma ◽  
Squamous Cell ◽  
Osteogenic Sarcoma ◽  
Rare Condition ◽  
Collision Tumor ◽  
Xanthogranulomatous Pyelonephritis ◽  
General Area

Collision tumor is a rare condition in which two neoplasms (usually benign and malignant), both growing in the same general area, collide with each other and become intermingled. We present histopathology and imaging correlation of xanthogranulomatous pyelonephritis coexistent with squamous cell carcinoma and osteogenic sarcoma of the kidney.

scholarly journals Lymphocytes: Versatile Participants in Acute Kidney Injury and Progression to Chronic Kidney Disease

2021 ◽  
Vol 12 ◽  
Author(s):  
Chujin Cao ◽  
Ying Yao ◽  
Rui Zeng
Keyword(s):  
Chronic Kidney Disease ◽  
Acute Kidney Injury ◽  
Kidney Disease ◽  
Renal Injury ◽  
Renal Fibrosis ◽  
Kidney Injury ◽  
Renal Parenchyma ◽  
Health Concern ◽  
High Morbidity ◽  
Parenchyma Cells

Background: Acute kidney injury (AKI) remains a major global public health concern due to its high morbidity and mortality. The progression from AKI to chronic kidney disease (CKD) makes it a scientific problem to be solved. However, it is with lack of effective treatments.Summary: Both innate and adaptive immune systems participate in the inflammatory process during AKI, and excessive or dysregulated immune responses play a pathogenic role in renal fibrosis, which is an important hallmark of CKD. Studies on the pathogenesis of AKI and CKD have clarified that renal injury induces the production of various chemokines by renal parenchyma cells or resident immune cells, which recruits multiple-subtype lymphocytes in circulation. Some infiltrated lymphocytes exacerbate injury by proinflammatory cytokine production, cytotoxicity, and interaction with renal resident cells, which constructs the inflammatory environment and induces further injury, even death of renal parenchyma cells. Others promote tissue repair by producing protective cytokines. In this review, we outline the diversity of these lymphocytes and their mechanisms to regulate the whole pathogenic stages of AKI and CKD; discuss the chronological responses and the plasticity of lymphocytes related to AKI and CKD progression; and introduce the potential therapies targeting lymphocytes of AKI and CKD, including the interventions of chemokines, cytokines, and lymphocyte frequency regulation in vivo, adaptive transfer of ex-expanded lymphocytes, and the treatments of gut microbiota or metabolite regulations based on gut-kidney axis.Key Message: In the process of AKI and CKD, T helper (Th) cells, innate, and innate-like lymphocytes exert mainly pathogenic roles, while double-negative T (DNT) cells and regulatory T cells (Tregs) are confirmed to be protective. Understanding the mechanisms by which lymphocytes mediate renal injury and renal fibrosis is necessary to promote the development of specific therapeutic strategies to protect from AKI and prevent the progression of CKD.

scholarly journals Dyke–Davidoff–Masson syndrome with crossed cerebellar atrophy

2017 ◽  
Vol 21 (1) ◽  
Author(s):  
Sanjay M. Khaladkar ◽  
Shishir Chauhan ◽  
Abhijit M. Patil ◽  
Siddappa G. Gandage ◽  
Surbhi Chauhan Kalra
Keyword(s):  
Mental Retardation ◽  
Middle Cerebral Artery ◽  
Cerebellar Atrophy ◽  
Facial Asymmetry ◽  
Rare Condition ◽  
Female Child ◽  
Left Middle Cerebral Artery ◽  
Cerebral Hemiatrophy ◽  
Radiological Changes ◽  
Left Middle

Dyke–Davidoff–Masson syndrome is a rare condition with classical, clinical and radiological changes – mental retardation, hemiparesis, facial asymmetry, seizures and cerebral hemiatrophy with calvarial changes. Contralateral cerebellar atrophy is rare and occurs if insult occurs after 1 month of age. We report a case of a 6-year-old female child presenting with right-sided hemiparesis, convulsions and left cerebral hemiatrophy with an old infarct in left middle cerebral artery (MCA) territory, ipsilateral calvarial thickening and right (crossed) cerebellar atrophy.

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