scholarly journals Bilateral multiple cystic kidney disease and renal cortical abscess in a Boerboel

2011 ◽  
Vol 82 (2) ◽  
Author(s):  
A. M. Kitshoffa ◽  
V. McClure ◽  
C. K. Lim ◽  
R. M. Kirberger
Keyword(s):  
Left Kidney ◽  
Renal Cysts ◽  
Cystic Kidney Disease ◽  
Cystic Kidney ◽  
Ultrasonographic Diagnosis ◽  
Cellular Debris ◽  
Cystic Renal Disease ◽  
The Right ◽  
Portal Of Entry ◽  
Multiple Cysts

Cystic renal disease is rare in dogs and although infected renal cysts have been reported in humans, no report could be found in dogs. A 58 kg, 5-year-old, castrated, male Boerboel presented with weight loss, pyrexia, lethargy and vomiting, 20 months after an incident of haematuria was reported. The initial ultrasonographic diagnosis was bilateral multiple renal cysts of unknown aetiology. The cysts had significantly increased in size over the 20-month period and some contained echogenic specks which could be related to infection, normal cellular debris or haemorrhage. In both kidneys the renal contours were distorted (the left more than the right). The abnormal shape of the left kidney was largely due to multiple cysts and a large crescent-shaped septate mass on the cranial pole of the kidney. Aspirates of the septate mass were performed (left kidney) and the cytology and culture were indicative of an abscess. It is suggested that the previous incident of haematuria provided a portal of entry for bacteria into the cysts resulting in renal cortical abscess formation.

Phthalate Esters, Cystic Kidney Disease in Animals and Possible Effects on Human Health: A Review

1990 ◽  
Vol 9 (6) ◽  
pp. 397-401 ◽  
Author(s):  
K.N. Woodward
Keyword(s):  
Kidney Disease ◽  
Human Health ◽  
Phthalate Esters ◽  
Renal Cysts ◽  
Cystic Kidney Disease ◽  
Cystic Kidney ◽  
Peroxisome Proliferation ◽  
Peroxisome Proliferators ◽  
Prolonged Administration ◽  
Route Of Exposure

1 Phthalate esters are known to cause hepatic peroxisome proliferation in rodents and, after prolonged administration, hepatocarcinogenesis. Peroxisome proliferators as a group are hepatocarcinogenic. The mechanism is not known but it does not appear to involve a direct genotoxic element. 2 DEHP and DBP have been shown to cause renal cysts in rodents and they also produce renal peroxisome proliferation. There are no data to causally link the two phenomena. 3 Although renal cysts have been noted in haemodialysis patients and haemodialysis is a route of exposure to DEHP, there are no data to suggest a cause and effect relationship. 4 More studies are needed on the mechanism of renal cystogenesis.

scholarly journals New PAX2 Mutation Associated with Polycystic Kidney Disease: A Case Report

2021 ◽  
Vol 15 ◽  
pp. 117955652199235
Author(s):  
Jessica Maria Forero-Delgadillo ◽  
Vanessa Ochoa ◽  
Natalia Duque ◽  
Jaime Manuel Restrepo ◽  
Hernando Londoño ◽  
...  
Keyword(s):  
Kidney Disease ◽  
Novel Mutation ◽  
Clinical Manifestations ◽  
Renal Cysts ◽  
Renal Dysplasia ◽  
Cystic Kidney Disease ◽  
Cystic Kidney ◽  
Incomplete Penetrance ◽  
Wide Range ◽  
Stage Renal Disease

Background: Congenital anomalies of the kidney and urinary tract (CAKUT) are the leading cause of end stage renal disease in children. Diagnosis by genetic testing has proven challenging due to its genetic and phenotypic heterogeneity, as well as incomplete penetrance. We report a case on a 16-months old female with a history of renal cysts and a PAX2 mutation. Case presentation: The patient presented with a prenatal diagnosis of Potter sequence and a postnatal diagnosis of renal cysts. An ultrasound at 20 weeks gestation revealed right renal agenesis and possible left renal dysplasia. Post natal genetic analyses identified a novel mutation in PAX2. Conclusion: Cystic kidney disease is often underdiagnosed due to its variable expressivity and wide range of clinical manifestations; PAX2 genetic screening should be considered for all patients with CAKUT.

The molecular basis of ciliopathies and cyst formation

2015 ◽  
Author(s):  
Wolfgang Kühn ◽  
Gerd Walz
Keyword(s):  
Planar Cell Polarity ◽  
Critical Role ◽  
Mammalian Target Of Rapamycin ◽  
Renal Cysts ◽  
Cyst Formation ◽  
Cystic Kidney Disease ◽  
Cystic Kidney ◽  
Convergent Extension ◽  
Diverse Range ◽  
Cell Intercalation

Abnormalities of the cilium, termed ‘ciliopathies’, are the prime suspect in the pathogenesis of renal cyst formation because the gene products of cystic disease-causing genes localize to them, or near them. However, we only partially understand how cilia maintain the geometry of kidney tubules, and how abnormal cilia lead to renal cysts, and the diverse range of diseases attributed to them. Some non-cystic diseases share pathology of the same structures. Although still incompletely understood, cilia appear to orient cells in response to extracellular cues to maintain the overall geometry of a tissue, thereby intersecting with the planar cell polarity (PCP) pathway and the actin cytoskeleton. The PCP pathway controls two morphogenetic programmes, oriented cell division (OCD) and convergent extension (CE) through cell intercalation that both seem to play a critical role in cyst formation. The two-hit theory of cystogenesis, by which loss of the second normal allele causes tubular epithelial cells to form kidney cysts, has been largely borne out. Additional hits and influences may better explain the rate of cyst formation and inter-individual differences in disease progression. Ciliary defects appear to converge on overlapping signalling modules, including mammalian target of rapamycin and cAMP pathways, which can be targeted to treat human cystic kidney disease irrespective of the underlying gene mutation.

scholarly journals Pelvic Kidney Mimicking Skeletal Metastasis on Bone Scan- Interesting Image

2021 ◽  
Vol 22 (2) ◽  
pp. 155-156
Author(s):  
Mohammed Mehedi Al Zahid Bhuiyan ◽  
Azmal Kabir Sarker ◽  
Hongyoon Choi ◽  
Minseok Suh ◽  
Gi Jeong Cheon
Keyword(s):  
Bone Scan ◽  
Left Kidney ◽  
Renal Cysts ◽  
Skeletal Metastasis ◽  
Breast Conserving Surgery ◽  
Left Breast ◽  
Lymph Node Biopsy ◽  
Hepatic Cyst ◽  
Large Area ◽  
The Right

A 57-year-old female patient underwent left breast-conserving surgery with sentinel lymph node biopsy for Left breast carcinoma (stage IIA). The patient had hypertension and diabetes mellitus. Other findings include multiple hepatic cyst, bilateral renal cysts and uterine myoma. She had no significant renal symptoms and her liver &renal function test were normal.She was sent for Technetium-99m-methylene diphosphonate (99mTc-MDP) bone scan. There was a large area of intense tracer concentration in the region of right sacro-iliac (SI) joint which appeared like an osteoblastic metastasis at first glance. However, absence of uptake in the right renal fossa with the left kidney being normal in position contemplated the probability of right-sidedpelvickidneywhich was confirmed later by a contrast enhanced computerized tomography (CT) scan of abdomen that showed a pelvic right kidney overlying the sacrum. Bangladesh J. Nuclear Med. 22(2): 155-156, Jul 2019

scholarly journals Uncontrolled hypertension and hyperreninemia after hemorrhage in a patient with end-stage renal disease and acquired renal cysts.

1994 ◽  
Vol 5 (1) ◽  
pp. 22-26
Author(s):  
S Bongu ◽  
P F Faubert ◽  
J G Porush ◽  
F Gulmi
Keyword(s):  
Kidney Disease ◽  
Esrd Patient ◽  
Renal Cysts ◽  
Cystic Kidney Disease ◽  
Uncontrolled Hypertension ◽  
Cystic Kidney ◽  
First Case ◽  
Acquired Cystic Kidney Disease ◽  
Perinephric Hematoma ◽  
Stage Renal Disease

Acquired cystic kidney disease occurs in over 74% of patients with ESRD on hemodialysis for more than 4 yr. A variety of complications have been associated with these cysts including bleeding, lithiasis, infection, obstruction, and malignant transformation. An ESRD patient who developed accelerating hypertension secondary to an acute perinephric hematoma due to a bleeding-acquired renal cyst is described. The hypertension, which was refractory to aggressive drug therapy, was controlled only after the involved kidney was removed, after the demonstration of an elevated ipsilateral renal vein renin level. This is the first case reported in which worsening hypertension, apparently due to the "Page Kidney," developed as a complication of perinephric bleeding in an ESRD patient with acquired cystic kidney disease.

Case 7.8

2014 ◽  
pp. 322-323
Author(s):  
Christine U. Lee ◽  
James F. Glockner
Keyword(s):  
Renal Failure ◽  
Chronic Renal Failure ◽  
Left Kidney ◽  
Renal Cysts ◽  
Renal Capsule ◽  
Axial Diffusion ◽  
Diffusion Weighted ◽  
Renal Hilum ◽  
The Right ◽  
Perinephric Fat

85-year-old man with acute on chronic renal failure Coronal SSFSE (Figure 7.8.1), axial FSE (Figure 7.8.2), and axial diffusion-weighted (b=100 s/mm2) (Figure 7.8.3) images reveal a small left kidney with hydronephrosis of upper pole calyces. Nodular thickening involves the renal capsule and Gerota fascia, with larger nodules adjacent to the renal hilum and within the perinephric fat. Note also the right-sided renal cysts....

scholarly journals Ttc30a affects tubulin modifications in a model for ciliary chondrodysplasia with polycystic kidney disease

2021 ◽  
Vol 118 (39) ◽  
pp. e2106770118
Author(s):  
Maike Getwan ◽  
Anselm Hoppmann ◽  
Pascal Schlosser ◽  
Kelli Grand ◽  
Weiting Song ◽  
...  
Keyword(s):  
Kidney Disease ◽  
Renal Cysts ◽  
Cystic Kidney Disease ◽  
Cystic Kidney ◽  
Cartilage Defects ◽  
Disease Genes ◽  
Embryonic Mouse ◽  
Cystic Kidneys ◽  
Jeune Syndrome ◽  
Sensenbrenner Syndrome

Skeletal ciliopathies (e.g., Jeune syndrome, short rib polydactyly syndrome, and Sensenbrenner syndrome) are frequently associated with nephronophthisis-like cystic kidney disease and other organ manifestations. Despite recent progress in genetic mapping of causative loci, a common molecular mechanism of cartilage defects and cystic kidneys has remained elusive. Targeting two ciliary chondrodysplasia loci (ift80 and ift172) by CRISPR/Cas9 mutagenesis, we established models for skeletal ciliopathies in Xenopus tropicalis. Froglets exhibited severe limb deformities, polydactyly, and cystic kidneys, closely matching the phenotype of affected patients. A data mining–based in silico screen found ttc30a to be related to known skeletal ciliopathy genes. CRISPR/Cas9 targeting replicated limb malformations and renal cysts identical to the models of established disease genes. Loss of Ttc30a impaired embryonic renal excretion and ciliogenesis because of altered posttranslational tubulin acetylation, glycylation, and defective axoneme compartmentalization. Ttc30a/b transcripts are enriched in chondrocytes and osteocytes of single-cell RNA-sequenced embryonic mouse limbs. We identify TTC30A/B as an essential node in the network of ciliary chondrodysplasia and nephronophthisis-like disease proteins and suggest that tubulin modifications and cilia segmentation contribute to skeletal and renal ciliopathy manifestations of ciliopathies in a cell type–specific manner. These findings have implications for potential therapeutic strategies.

scholarly journals Autosomal dominant polycystic kidney disease

2008 ◽  
Vol 136 (Suppl. 4) ◽  
pp. 340-347
Author(s):  
Steva Pljesa
Keyword(s):  
Kidney Disease ◽  
Autosomal Dominant ◽  
Renal Cysts ◽  
Diagnostic Methods ◽  
Cystic Kidney Disease ◽  
Cystic Kidney ◽  
Na Channel ◽  
Somatostatin Analogue ◽  
Ca Channel ◽  
Angiotensin Ii Receptor

Autosomal dominant polycystic kiney disease is a hereditary systemic disorder, characterized by the developement of cysts, mainly in the kidney and liver, also with gastrointestinal and cardiovascular abnormalities. It affects 4 to 6 million people wordwide and accounts for end-stage renal disease in 7-10% of dialysis patients. The genetic penetrance is 100%, all affected individuals develop renal cysts until 70 years of age, and because of a great renal function reserve only about 50% of patients develop some degree of renal failure until the age of 60. Autosomal dominant polycystic kiney disease is a heterogeneous disorder, from a clinical as well as from a genetic point of view. There are at least three genes responsible for the disease: PKD-1 gene localized on chromosome 16p in the 16p13.3 segment which encodes Polycystin 1 protein similar to membrane receptor, PKD-2 gene localized on chromosome 4q in 4q13-23 segment which encodes Polycystin 2 protein wery similar to voltage L type Ca++ channel as well as Na+ channel and PKD-3 gene of unknown localization. Specific proteins participate in regulation od cell proliferation, apoptosis, secretion, polarity, cell-matrix interactions as cell-cell interactions and lead to the developement of cystic kidney disease. Renal manifestations of disease include structural (cyst development), functional (concentration alility falls), endocrine (renin erythropoietin) abnormalities and extra- renal manifestations. A routine diagnostic methods are good case-history about cystic kidney disease in family, ultrasonographic examination of kidneys and computerized tomography. In therapy of autosomal dominant polycystic kiney disease, low protein diets may help, treatment of arterial hypertension with ACE inhibitors and angiotensin II receptor blockers, the vasopressin V2 antagonists (VSR), rapamycin and long-acting somatostatin analogue may have some benefit.

scholarly journals Renal Involvement in Linear Nevus Sebaceous Syndrome—An Underrecognized Feature

2021 ◽  
Vol 13 (2) ◽  
pp. 203-209
Author(s):  
Chon-In Kuok ◽  
Winnie-Kwai-Yu Chan
Keyword(s):  
Renal Involvement ◽  
Renal Cysts ◽  
Celiac Trunk ◽  
Renal Arteries ◽  
Mesenteric Arteries ◽  
Cystic Kidney Disease ◽  
Neurological Deficits ◽  
Cystic Kidney ◽  
Bilateral Renal Artery Stenosis ◽  
Nevus Sebaceous

Linear nevus sebaceous syndrome (LNSS) is a rare neurocutaneous disorder. It is characterized by the presence of nevus sebaceous, ocular anomalies, neurological deficits, and convulsion. Renal involvement was not commonly reported. We report a 10-year-old girl with LNSS who had concomitant cystic kidney disease and diffuse aortopathy with bilateral renal artery stenosis, leading to hypertension requiring oral anti-hypertensive medications. The girl presented with chorioretinal coloboma and multiple nevus sebaceous at birth. She had aortic coarctation and received surgical repair at one week of life. She had persistent hypertension during her follow-up. Further investigations were performed to look for causes of hypertension apart from possible re-coarctation. Her magnetic resonance angiogram revealed diffuse aortopathy, which extended from the aortic arch to the abdominal aorta. Branches of the aorta, including the celiac trunk, superior mesenteric arteries, and renal arteries, were also narrowed. Multiple renal cysts were also identified in her right kidney. Interventional angioplasty over the renal arteries was not feasible due to diffuse narrowing of the aorta, especially at the origins of renal arteries. The blood pressure was controlled with oral anti-hypertensive medications. Our case illustrated that pediatricians should be aware of the possible renal involvements in LNSS, which impose a significant impact on the management and long-term prognosis of these patients.

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