scholarly journals Deficiency in Six2 during prenatal development is associated with reduced nephron number, chronic renal failure, and hypertension in Br/+ adult mice

2009 ◽  
Vol 296 (5) ◽  
pp. F1166-F1178 ◽  
Author(s):  
Ben Fogelgren ◽  
Shiming Yang ◽  
Ian C. Sharp ◽  
Odaro J. Huckstep ◽  
Wenbin Ma ◽  
...  
Keyword(s):  
Renal Failure ◽  
Chronic Renal Failure ◽  
Prenatal Development ◽  
Mutant Mice ◽  
Renal Tubules ◽  
Nephron Number ◽  
Renal Hypoplasia ◽  
Adult Mice ◽  
Homeobox Transcription Factor ◽  
And Function

The Br/+ mutant mouse displays decreased embryological expression of the homeobox transcription factor Six2, resulting in hertitable renal hypoplasia. The purpose of this study was to characterize the renal physiological consequences of embryonic haploinsuffiency of Six2 by analyzing renal morphology and function in the adult Br heterozygous mutant. Adult Br/+ kidneys weighed 50% less than those from wild-type mice and displayed glomerulopathy. Stereological analysis of renal glomeruli showed that Br/+ kidneys had an average of 88% fewer glomeruli than +/+ kidneys, whereas individual glomeruli in Br/+ mice maintained an average volume increase of 180% compared with normal nephrons. Immunostaining revealed increased levels of endothelin-1 (ET-1), endothelin receptors A (ETA) and B (ETB), and Na-K-ATPase were present in the dilated renal tubules of mutant mice. Physiological features of chronic renal failure (CRF) including elevated mean arterial pressure, increased plasma creatinine, and dilute urine excretion were measured in Br/+ mutant mice. Electron microscopy of the Br/+ glomeruli revealed pathological alterations such as hypercellularity, extracellular matrix accumulation, and a thick irregular glomerular basement membrane. These results indicate that adult Br/+ mice suffer from CRF associated with reduced nephron number and renal hypoplasia, as well as glomerulopathy. Defects are associated with embryological deficiencies of Six2, suggesting that proper levels of this protein during nephrogenesis are critical for normal glomerular development and adult renal function.

scholarly journals Cutaneous Manifestations in Chronic Renal Failure Patients

1970 ◽  
Vol 9 (2) ◽  
pp. 13-16
Author(s):  
NB Shakya ◽  
SL Rajbhandari ◽  
A Sharma ◽  
RK Deo ◽  
SM Jha
Keyword(s):  
Renal Failure ◽  
Chronic Renal Failure ◽  
End Stage Renal Disease ◽  
Cutaneous Manifestation ◽  
Renal Diseases ◽  
Nephron Number ◽  
Cutaneous Manifestations ◽  
Stage Renal Disease ◽  
End Stage ◽  
And Function

Introduction: Chronic renal failure is a pathophysiologic process with multiple etiologies, resulting in the inexorable attrition of nephron number and function, and frequently leading to end stage renal disease. There are various cutaneous changes in chronic renal failure. Objectives: To observe the cutaneous manifestations in chronic renal falure and find out any difference in occurrence of cutaneous manifestation with modality of treatment of CRF.  Methods: The study was conducted in 50 (fifty) adult patients with chronic renal failure and another 50 (fifty) patients with similar age admitted with other renal diseases but not suffering from chronic  renal failure as control to facilitate comparison were considered in the Department of Dermatology and Venereology and the Department of Medicine, Shree Birendra Hospital, Chhauni from September 2008 to June 2010. Results: A significant occurrence of pruritus, xerosis and pallor in CRF patients: the highest being pruritus followed by xerosis and pallor. Occurrence of pruritus was found to be more in HD patients (68%) than in IPD patients (38%). No correlation was found between ages, sex, and duration of dialysis with complaint of pruritus. Skin xerosis is considered an important factor contributing or initiating pruritus. Conclusion: Pruritus is the commonest cutaneous manifestation of chronic renal failure. DOI: http://dx.doi.org/10.3126/mjsbh.v9i2.5020 Medical Journal of Shree Birendra Hospital Vol.9(2) 2010: 13-16

scholarly journals Effect of chronic renal failure on the expression and function of rat intestinal P‐glycoprotein in drug excretion

2001 ◽  
Vol 16 (8) ◽  
pp. 1607-1614 ◽  
Author(s):  
Céline Veau ◽  
Christine Leroy ◽  
Hélène Banide ◽  
Daniel Auchère ◽  
Sylviane Tardivel ◽  
...  
Keyword(s):  
Renal Failure ◽  
Chronic Renal Failure ◽  
Drug Excretion ◽  
P Glycoprotein ◽  
And Function

Elevation Of Antithrombin III By Sulfinpyrazone Therapy In Chronic Renal Failure

1981 ◽  
Author(s):  
M Bern ◽  
J Green
Keyword(s):  
Renal Failure ◽  
Chronic Renal Failure ◽  
Antithrombin Iii ◽  
Chronic Hemodialysis ◽  
Protective Effects ◽  
Platelet Factor ◽  
At Iii ◽  
Before And After ◽  
Artery Disease ◽  
And Function

Sulfinpyrazone can reduce the incidence of thrombosis of A-V shunts in chronic renal failure. The drug is also reported to prevent acute deaths from coronary artery disease. This study was to determine mechanisms for these protective effects.Patients on chronic hemodialysis served as the study models. Six patients on dialysis three times per week for 6 or more months received sulfinpyrazone 200 mgm t.i.d. p.o. for 14 days. Blood samples were obtained before dialysis was begun before and after the 14 days of drug therapy.Results are shown as mean ± standard error of mean.AT III levels rose significantly by functional and immune assays. Functional levels (by von Kaulla technique) rose 24.5 ± 3.1 sec. to 47.3 + 5.5 sec. (P>.005) Plasma protein AT III (by radial immunodiffusion) rose 31.2 ± 2.17 mg/dl to 37.9 ± 2.1 mgm/dl. (P>.01) Platelet factor 4 (by Abbot radioimmunology assay) fell from 46.4 + 13.6 ngm/ml to 9.5 ± 1.1 ngm/ml.(P>.005) The concentration of thrombin-anti-thrombin complex (by R. Rosenberg, Harvard Medical School, Boston) rose from 4.2 ± .09 to 8.4 ± 1.0 (P>.005)Thus it appears that sulfinpyrazone elevates antithrombin concentration and function while simultaneously suppressing platelet release. These two effects may or may not be mutually dependent. The clinical efficacy of sulfinpyrazone may relate in part to the elevation of antithrombin III, probably by inhibiting its consumption, while also inhibiting platelet function.

scholarly journals Congenital uronephropathy pattern in children

2001 ◽  
Vol 41 (5) ◽  
pp. 241
Author(s):  
Husein Alatas ◽  
Natalina Soesilawati ◽  
Bambang Madiyono
Keyword(s):  
Renal Failure ◽  
Urinary Tract Infection ◽  
Acute Renal Failure ◽  
Urinary Tract ◽  
Chronic Renal Failure ◽  
Tract Infection ◽  
Teaching Hospitals ◽  
Affecting Factors ◽  
Cross Sectional ◽  
Renal Hypoplasia

To obtain the basic data of congenital uronephropathy pattern and the affecting factors in children, we conducted a cross-sectional study at the Department of Child Health Cipto Mangunkusumo (CM) Hospital Jakarta from 1995 to 1999 and 9 teaching hospitals throughout Indonesia. During the study period 134 patients were obtained, 116 patients from the CM Hospital and 18 patients from other teaching hospitals. Most patients (48.8%) were below 1 year of age; male were affected more than female (2.4:1). The disorder was classified into two groups, i.e., congenital nephropathy and uropathy. There were 10 children with nephropathy, i.e., 4 with unilateral renal hypoplasia, 3 with polycystic kidney, and 3 with renal agenesis. In the uropathy group, 43 were with hypospadia, 22 with primary reflux vesicoureter, 18 with neurogenic bladder, and 17 with ureteropelvic junction obstruction. The complications found were urinary tract infection (71.2%), chronic renal failure (15.7%), hypertension (3.7%), and acute renal failure (1.5%). Consanguinity, familial disorders, maternal diseases, x-ray exposure and abortion efforts were found in a small proportion of patients. History of drug or herbs use in the first trimester of pregnancy was found in a large proportion of patients, mostly took analgesics (especially acetaminophen). In conclusion, uropathy disorders were much more common than congenital nephropathy. The most common complication was urinary tract infection, followed by chronic renal failure, hypertension, and acute renal failure.

Old age and kidneys

2008 ◽  
Vol 149 (17) ◽  
pp. 789-794 ◽  
Author(s):  
Endre Balázs ◽  
Andrea Ruszwurm ◽  
Miklós Székely ◽  
István Wittmann ◽  
Judit Nagy
Keyword(s):  
Renal Failure ◽  
Chronic Renal Failure ◽  
Interstitial Fibrosis ◽  
The Elderly ◽  
Tubular Atrophy ◽  
Water Excretion ◽  
Age Related ◽  
Renal Morphology ◽  
And Function ◽  
Age Related Changes

Age-related changes in renal morphology and function cannot be regarded physiological. The number of glomeruli falls, sclerotic glomeruli and aglomerular arterioles develop. Besides tubular atrophy interstitial fibrosis is often seen, and the age-related vascular changes strongly affect the kidneys. Renal blood flow and GFR decrease, without concomitant changes in se-creatinine. Disorders of tubular transport manifest mainly in salt- and water-excretion and lead to hyposthenuria. The pathogenesis of these age-related changes is not fully understood. Nevertheless, such changes impair the excretory functions and the pharmacokinetics of drugs. In real chronic renal failure other functions (erythropoietin production, vitamin-D, Ca and P metabolism) are also impaired. Due to more frequent occurrence of systemic diseases (diabetes, hypertension, etc.) in the elderly, real chronic renal failure is also more common, and various forms of acute renal failure develop more easily.

scholarly journals Gata3Hypomorphic Mutant Mice Rescued with a Yeast Artificial Chromosome Transgene Suffer a Glomerular Mesangial Cell Defect

2016 ◽  
Vol 36 (17) ◽  
pp. 2272-2281 ◽  
Author(s):  
Takashi Moriguchi ◽  
Lei Yu ◽  
Akihito Otsuki ◽  
Keiko Ainoya ◽  
Kim-Chew Lim ◽  
...  
Keyword(s):  
Kidney Development ◽  
Yeast Artificial Chromosome ◽  
Mesangial Cells ◽  
Critical Role ◽  
Artificial Chromosome ◽  
Mutant Mice ◽  
Renal Tubules ◽  
Glomerular Mesangial Cells ◽  
Renal Hypoplasia ◽  
Transgenic Mouse Line

GATA3 is a zinc finger transcription factor that plays a crucial role in embryonic kidney development, while its precise functions in the adult kidney remain largely unexplored. Here, we demonstrate that GATA3 is specifically expressed in glomerular mesangial cells and plays a critical role in the maintenance of renal glomerular function. Newly generatedGata3hypomorphic mutant mice exhibited neonatal lethality associated with severe renal hypoplasia. Normal kidney size was restored by breeding the hypomorphic mutant with a rescuing transgenic mouse line bearing a 662-kbGata3yeast artificial chromosome (YAC), and these animals (termed G3YR mice) survived to adulthood. However, most of the G3YR mice showed degenerative changes in glomerular mesangial cells, which deteriorated progressively during postnatal development. Consequently, the G3YR adult mice suffered severe renal failure. We found that the 662-kbGata3YAC transgene recapitulatedGata3expression in the renal tubules but failed to direct sufficient GATA3 activity to mesangial cells. Renal glomeruli of the G3YR mice had significantly reduced amounts of platelet-derived growth factor receptor (PDGFR), which is known to participate in the development and maintenance of glomerular mesangial cells. These results demonstrate a critical role for GATA3 in the maintenance of mesangial cells and its absolute requirement for prevention of glomerular disease.

scholarly journals Fatal Paratanaisia bragai (Digenea: Eucotylidae) infection in scarlet macaws (Ara macao) in Costa Rica

2015 ◽  
Vol 60 (3) ◽  
Author(s):  
Alejandro Alfaro-Alarcón ◽  
Juan Alberto Morales ◽  
Vincenzo Veneziano ◽  
Mario Santoro
Keyword(s):  
Renal Failure ◽  
Chronic Renal Failure ◽  
Costa Rica ◽  
Interstitial Fibrosis ◽  
Renal Tubules ◽  
Urinary System ◽  
Severe Dehydration ◽  
White Spots ◽  
Ara Macao ◽  
Renal Infection

AbstractWe report on four fatal cases of renal infection due to Paratanaisia bragai in scarlet macaws (Ara macao) from two rescue centres in Costa Rica. At necropsy, birds had severe dehydration and cachexia. Two birds had hydropericardium, oedematous lungs, and liver lipidic degeneration. All birds had enlarged kidneys with brown pale discoloration and diffuse white spots on the cortical and sliced surfaces. Ureters were filled with many specimens of P. bragai. Histopathologically, the urinary system revealed multifocal interstitial lymphocytic-plasmacytic nephritis, multifocal mineralization of renal tubules, and interstitial fibrosis associated with flukes. Death of all scarlet macaws was related to severe nephritis leading to chronic renal failure due to P. bragai infection. It is plausible that P. bragai infection of scarlet macaws was accidental due to ingestion of the gastropod intermediate host inside the cages during the rainy season when humidity is higher and gastropods are more active. This represents the second report of parasitism by Eucotylidae digeneans in birds of Psittaciformes and the first in scarlet macaws.

Ultrastructural and Biosynthetic Changes in Epitheliocytes of Renal Tubules during the Development of Chronic Renal Failure

2005 ◽  
Vol 139 (3) ◽  
pp. 369-373
Author(s):  
L. M. Nepomnyashchikh ◽  
N. L. Tov ◽  
S. V. Aidagulova ◽  
D. V. Nesterov
Keyword(s):  
Renal Failure ◽  
Chronic Renal Failure ◽  
Renal Tubules
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