Actinomycetes canaliculitis complicating congenital nasolacrimal duct obstruction in an infant

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Akshay Gopinathan Nair

2018-01-01

Full Text Available Actinomyces israelii is a Gram-positive anaerobic organism commonly associated with canaliculitis in adults. Pediatric canaliculitis is relatively rare, especially in infancy. We report the case of an 11-month-old boy who presented with co-existing canaliculitis and congenital nasolacrimal obstruction. The presenting signs included epiphora, discharge, conjunctival congestion, and matting of lashes. On examination, punctual pouting, regurgitation, and yellow canaliculiths were noted. A punctoplasty and canalicular curettage were performed along with nasolacrimal probing. Microbiological tests confirmed the organisms to be A. israelii. We discuss the clinical features and management of Actinomyces-associated canaliculitis and review the available literature on pediatric canaliculitis.

Role of TREM1-DAP12 in renal inflammation during obstructive nephropathy.

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Alessandra Tammaro

Full Text Available Tubulo-interstitial damage is a common finding in the chronically diseased kidney and is characterized by ongoing inflammation and fibrosis leading to renal dysfunction and end-stage renal disease. Upon kidney injury, endogenous ligands can be released which are recognized by innate immune sensors to alarm innate immune system. A new family of innate sensors is the family of TREM (triggering receptor expressed on myeloid cell. TREM1 is an activating receptor and requires association with transmembrane adapter molecule DAP12 (DNAX-associated protein 12 for cell signaling. TREM1-DAP12 pathway has a cross-talk with intracellular signaling pathways of several Toll-like receptors (TLRs and is able to amplify TLR signaling and thereby contributes to the magnitude of inflammation. So far, several studies have shown that TLRs play a role in obstructive nephropathy but the contribution of TREM1-DAP12 herein is unknown. Therefore, we studied TREM1 expression in human and murine progressive renal diseases and further investigated the role for TREM1-DAP12 by subjecting wild-type (WT, TREM1/3 double KO and DAP12 KO mice to murine unilateral ureter obstruction (UUO model. In patients with hydronephrosis, TREM1 positive cells were observed in renal tissue. We showed that in kidneys from WT mice, DAP12 mRNA and TREM1 mRNA and protein levels were elevated upon UUO. Compared to WT mice, DAP12 KO mice displayed less renal MCP-1, KC and TGF-β1 levels and less influx of macrophages during progression of UUO, whereas TREM1/3 double KO mice displayed less renal MCP-1 level. Renal fibrosis was comparable in WT, TREM1/3 double KO and DAP12 KO mice. We conclude that DAP12, partly through TREM1/3, is involved in renal inflammation during progression of UUO.

Oxidative stress in obstructive nephropathy

NARCIS (Netherlands)

Dendooven, Amelie; Ishola, David A.; Nguyen, Tri Q.; Van der Giezen, Dionne M.; Kok, Robbert Jan; Goldschmeding, Roel; Joles, Jaap A.

P>Unilateral ureteric obstruction (UUO) is one of the most commonly applied rodent models to study the pathophysiology of renal fibrosis. This model reflects important aspects of inflammation and fibrosis that are prominent in human kidney diseases. In this review, we present an overview of the

Transurethral incision of congenital obstructive lesions in the posterior urethra in boys and its effect on urinary incontinence and urodynamic study.

Science.gov (United States)

Nakamura, Shigeru; Kawai, Shina; Kubo, Taro; Kihara, Toshiharu; Mori, Kenichi; Nakai, Hideo

2011-04-01

Study Type--Therapy (case series) Level of Evidence 4. What's known on the subject? and What does the study add? We have clarified that there exist two types of voiding urodynamics (pressure-flow-study) for congenital urethral obstruction in boys; one is synergic pattern (SP) and the other is dyssynergic pattern (DP). In terms of daytime incontinence and nocturnal enuresis, the transurethral endoscopic incision of these obstructive lesions is only effective in the SP type, while never effective in the DP type. The synergic pattern (SP) seems to represent simple anatomical obstruction, while the dyssynergic pattern (DP) may represent anatomical obstruction complicated with functional obstruction. The efficacy of endoscopic incision to mild forms of congenital urethral obstruction has been controversial, especially in terms of nocturnal enuresis. One of the reasons for the controversy is due to the lack of pre-and post-operative urodynamic assessment with its linkage to symptomatic change. We have, for the first time in the world, systematically conducted voiding urodynamic study for those elusive lesions seen in enuretic boys. Conclusively, for simple mechanical obstruction (SP), we confirmed that some voiding urodynamic parameters improve after the endoscopic incision, parallel to symptomatic improvement, while in the rest (DP) endoscopic incision is never effective. The cause of this ineffectiveness seemed to be due to persistent functional obstruction having superimposed on mechanical obstruction. The result of the study urges us to be more keen to diagnose and treat the mild congenial urethral obstruction as well as the concomitant functional obstruction in boys with nocturnal enuresis. • To evaluate the clinical significance of congenital obstructive lesions of the posterior urethra in boys with refractory primary nocturnal enuresis. • VCUG was performed in 43 consecutive boys who visited our department from April 2004 to April 2009 who were unresponsive to

MR imaging appearance of laryngeal atresia (congenital high airway obstruction syndrome): unique course in a fetus

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Kuwashima, Shigeko; Kitajima, Kazuhiro; Kaji, Yasushi [Dokkyo Medical University, Department of Radiology, Mibu, Shimotsuga-gun, Tochigi (Japan); Watanabe, Hiroshi [Dokkyo Medical University, Department of Obstetrics and Gynecology, Mibu (Japan); Watabe, Yoshiyuki; Suzumura, Hiroshi [Dokkyo Medical University, Department of Pediatrics, Mibu (Japan)

2008-03-15

Congenital high airway obstruction syndrome (CHAOS) is a rare life-threatening syndrome. Most cases are diagnosed prenatally by US. We report a fetus with this syndrome that showed a unique course revealed on MRI. Ultrasonography at 22 weeks demonstrated that the fetus had ascites and bilaterally enlarged hyperechoic lungs. Congenital infection, congenital cystic adenomatoid malformation or CHAOS was suspected. Subsequent MRI performed at 24 weeks demonstrated bilaterally enlarged high-signal lungs, dilated bronchi, massive ascites, subcutaneous oedema and polyhydramnios. MRI confirmed the diagnosis of CHAOS. A second MRI at 35 weeks showed that the bilateral lung enlargement, ascites, oedema and polyhydramnios had resolved, but that the appearance of the airway was unchanged. The infant was delivered by caesarean section at 38 weeks of gestation and immediate tracheostomy was performed. This spontaneous regression was explained by a tracheo-oesophageal fistula that may have decreased the intrathoracic pressure. (orig.)

MR imaging appearance of laryngeal atresia (congenital high airway obstruction syndrome): unique course in a fetus

International Nuclear Information System (INIS)

Kuwashima, Shigeko; Kitajima, Kazuhiro; Kaji, Yasushi; Watanabe, Hiroshi; Watabe, Yoshiyuki; Suzumura, Hiroshi

2008-01-01

Congenital high airway obstruction syndrome (CHAOS) is a rare life-threatening syndrome. Most cases are diagnosed prenatally by US. We report a fetus with this syndrome that showed a unique course revealed on MRI. Ultrasonography at 22 weeks demonstrated that the fetus had ascites and bilaterally enlarged hyperechoic lungs. Congenital infection, congenital cystic adenomatoid malformation or CHAOS was suspected. Subsequent MRI performed at 24 weeks demonstrated bilaterally enlarged high-signal lungs, dilated bronchi, massive ascites, subcutaneous oedema and polyhydramnios. MRI confirmed the diagnosis of CHAOS. A second MRI at 35 weeks showed that the bilateral lung enlargement, ascites, oedema and polyhydramnios had resolved, but that the appearance of the airway was unchanged. The infant was delivered by caesarean section at 38 weeks of gestation and immediate tracheostomy was performed. This spontaneous regression was explained by a tracheo-oesophageal fistula that may have decreased the intrathoracic pressure. (orig.)

Congenital high airway obstruction syndrome: MR/US findings, effect on management, and outcome

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Mong, Andrew; Johnson, Ann M.; Kramer, Sandra S.; Jaramillo, Diego [Children' s Hospital of Philadelphia, Department of Radiology, Philadelphia, PA (United States); Coleman, Beverly G. [Hospital of the University of Pennsylvania, Department of Radiology, Philadelphia, PA (United States); Hedrick, Holly L.; Flake, Alan; Johnson, Mark; Wilson, R.D.; Adzick, N.S. [Children' s Hospital of Philadelphia, The Center for Fetal Diagnosis and Treatment, Philadelphia, PA (United States); Kreiger, Portia [Children' s Hospital of Philadelphia, Department of Pathology and Laboratory Medicine, Philadelphia, PA (United States)

2008-11-15

Congenital high airway obstruction syndrome (CHAOS) is a rare disorder defined as any fetal abnormality that obstructs the larynx or trachea. Prompt airway intervention at delivery after accurate prenatal diagnosis may allow survival of this otherwise fatal condition. To identify prenatal MRI findings in CHAOS, to compare these findings with those of fetal US, to determine if imaging alters diagnosis and management decisions, and to correlate prenatal with postnatal imaging findings. Records and MRI scans of ten fetuses with CHAOS were reviewed, and the findings correlated with outside and same-day fetal US and postnatal imaging findings. Fetal lung volumes were measured on MRI scans. Large lung volumes were found in 90% of the fetuses. Increased lung signal intensity, inverted diaphragm, and a dilated, fluid-filled lower airway were identified in all. The obstruction level was identified in 90%. MRI changed screening US diagnosis in 70%, but was concordant with the tertiary care US imaging in 90%. Seven fetuses were terminated or died in utero, and three fetuses survived after ex utero intrapartum tracheostomy placement. Autopsy or bronchoscopy performed in 60% confirmed CHAOS. Postnatal chest radiographs and CT showed hyperinflation, while US and fluoroscopy showed diminished diaphragmatic motion. MRI demonstrates large lung volumes, increased lung signal intensity, inverted diaphragm, and dilated fluid-filled lower airway, and usually identifies the obstruction level. The degree of correlation between MRI and tertiary prenatal US is high, but CHAOS is frequently misdiagnosed on screening US. Correct diagnosis may enable planned airway management. Voluminous lungs and diaphragmatic abnormalities persist on postnatal imaging. (orig.)

Congenital high airway obstruction syndrome: MR/US findings, effect on management, and outcome

International Nuclear Information System (INIS)

Mong, Andrew; Johnson, Ann M.; Kramer, Sandra S.; Jaramillo, Diego; Coleman, Beverly G.; Hedrick, Holly L.; Flake, Alan; Johnson, Mark; Wilson, R.D.; Adzick, N.S.; Kreiger, Portia

2008-01-01

Congenital high airway obstruction syndrome (CHAOS) is a rare disorder defined as any fetal abnormality that obstructs the larynx or trachea. Prompt airway intervention at delivery after accurate prenatal diagnosis may allow survival of this otherwise fatal condition. To identify prenatal MRI findings in CHAOS, to compare these findings with those of fetal US, to determine if imaging alters diagnosis and management decisions, and to correlate prenatal with postnatal imaging findings. Records and MRI scans of ten fetuses with CHAOS were reviewed, and the findings correlated with outside and same-day fetal US and postnatal imaging findings. Fetal lung volumes were measured on MRI scans. Large lung volumes were found in 90% of the fetuses. Increased lung signal intensity, inverted diaphragm, and a dilated, fluid-filled lower airway were identified in all. The obstruction level was identified in 90%. MRI changed screening US diagnosis in 70%, but was concordant with the tertiary care US imaging in 90%. Seven fetuses were terminated or died in utero, and three fetuses survived after ex utero intrapartum tracheostomy placement. Autopsy or bronchoscopy performed in 60% confirmed CHAOS. Postnatal chest radiographs and CT showed hyperinflation, while US and fluoroscopy showed diminished diaphragmatic motion. MRI demonstrates large lung volumes, increased lung signal intensity, inverted diaphragm, and dilated fluid-filled lower airway, and usually identifies the obstruction level. The degree of correlation between MRI and tertiary prenatal US is high, but CHAOS is frequently misdiagnosed on screening US. Correct diagnosis may enable planned airway management. Voluminous lungs and diaphragmatic abnormalities persist on postnatal imaging. (orig.)

Congenital Midureteric Stricture

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Singh Shalinder

2001-01-01

Full Text Available Congenital midureteric obstruction is a rare entity which can be caused by either ureteric valves or strictures. We report our experience with four patients with midureteric obstruction due to stricture over a six-year period. The condition needs to be differentiated from obstruction of the pelviureteric and vesicoureteric junctions. Obstruction can be initially screened by ultrasound and confirmed by a radionuclide scan with furosemide challenge. Retrograde ureteropyelography and/or intravenous urography may be required to define the level of the stricture.

Acute ciprofloxacin-induced crystal nephropathy with granulomatous interstitial nephritis

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R Goli

2017-01-01

Full Text Available Crystal-induced acute kidney injury (AKI is caused by the intratubular precipitation of crystals, which results in obstruction and kidney injury. Ciprofloxacin, a commonly used antibiotic, causes AKI secondary to immune-mediated interstitial injury. Rare mechanisms of ciprofloxacin-induced renal injury include crystalluria, rhabdomyolysis, and granulomatous interstitial nephritis. Clinical and experimental studies have suggested that crystalluria and crystal nephropathy due to ciprofloxacin occur in alkaline urine. Preexisting kidney function impairment, high dose of the medication, and advanced age predispose to this complication. We report a case of ciprofloxacin-induced crystal nephropathy and granulomatous interstitial nephritis in a young patient with no other predisposing factors. The patient responded to conservative treatment without the need for glucocorticoids.

Obstructive uropathy in childhood: A review | Eke | Port Harcourt ...

African Journals Online (AJOL)

Methods: Contemporary information on the management of obstructive uropathy was obtained by searching the Medline and adding information from the authors\\' experience. Results: Obstructive uropathy causes renal impairment in all age groups. The causes in children may be congenital or acquired. The congenital ...

Evaluation of reflux nephropathy, pyelonephritis and renal dysplasia

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Grattan-Smith, J.D. [Emory University School of Medicine, Children' s Healthcare of Atlanta, Department of Radiology, Atlanta, GA (United States); Children' s Healthcare of Atlanta, Department of Radiology, Atlanta, GA (United States); Little, Stephen B. [Children' s Healthcare of Atlanta, Department of Radiology, Atlanta, GA (United States); Jones, Richard A. [Emory University School of Medicine, Children' s Healthcare of Atlanta, Department of Radiology, Atlanta, GA (United States)

2008-01-15

MR urography has the potential to significantly improve our understanding of the relationship between reflux nephropathy, pyelonephritis, vesicoureteric reflux and renal dysplasia. MR urography utilizes multiple parameters to assess both renal anatomy and function and provides a more complete characterization of acquired and congenital disease. Pyelonephritis and renal scarring can be distinguished by assessing the parenchymal contours and signal intensity. Characteristic imaging features of renal dysplasia include small size, subcortical cysts, disorganized architecture, decreased and patchy contrast enhancement as well as a dysmorphic pelvicalyceal system. Because of its ability to subdivide and categorize this heterogeneous group of disorders, it seems inevitable that MR urography will replace DMSA renal scintigraphy as the gold standard for assessment of pyelonephritis and renal scarring. MR urography will contribute to our understanding of renal dysplasia and its relationship to reflux nephropathy. (orig.)

Evaluation of reflux nephropathy, pyelonephritis and renal dysplasia

International Nuclear Information System (INIS)

Grattan-Smith, J.D.; Little, Stephen B.; Jones, Richard A.

2008-01-01

MR urography has the potential to significantly improve our understanding of the relationship between reflux nephropathy, pyelonephritis, vesicoureteric reflux and renal dysplasia. MR urography utilizes multiple parameters to assess both renal anatomy and function and provides a more complete characterization of acquired and congenital disease. Pyelonephritis and renal scarring can be distinguished by assessing the parenchymal contours and signal intensity. Characteristic imaging features of renal dysplasia include small size, subcortical cysts, disorganized architecture, decreased and patchy contrast enhancement as well as a dysmorphic pelvicalyceal system. Because of its ability to subdivide and categorize this heterogeneous group of disorders, it seems inevitable that MR urography will replace DMSA renal scintigraphy as the gold standard for assessment of pyelonephritis and renal scarring. MR urography will contribute to our understanding of renal dysplasia and its relationship to reflux nephropathy. (orig.)

Congenital duodenal diaphragm in eight children

International Nuclear Information System (INIS)

Nawaz, Akhtar; Matta, Hilal; Jacobsz, Alic; Al-Salem, Ahmad H.; Trad, Omar

2004-01-01

Congenital duodenal obstruction (CDO) is common and usually easy to diagnose cause of intestinal obstruction in the newborn, except when the cause of the obstruction is duodenal diaphragm. We describe our experience with eight children who had intrinsic duodenal obstruction secondary to a duodenal diaphragm. The medical record of 22 children with the diagnosis of congenital intrinsic duodenal were reviewed for age, sex, gestation, birth weight, clinical features, associated anomalies, method of diagnosis, treatment and outcome. Operative findings and procedures were obtained from the operative notes. Eight of 22 children (36.4%) had congenital duodenal diaphragm (CDD). In all children, the diagnosis was made from the plain abdominal X-ray, which showed the classic double-bubble appearence, and barium meal, which showed duodenal obstruction. Four patients had associated anomalies, including two with Down's syndrome intraoperatively, five patients were found to have duodenal diaphragm with a central hole, while the other three had complete duodenal diaphragms. Postoperatively, all patients did well. Six required total parenteral nutrition. The 100% survival rate among these children is comparable to that in Western countries, and can be attributed to the lack of major associated abnormalities, good perioperative management, and the availability of total parenteral nutrition. (author)

All That Glitters Yellow Is Not Gold: Presentation and Pathophysiology of Bile Cast Nephropathy.

Science.gov (United States)

Pitlick, Mitchell; Rastogi, Prerna

2017-10-01

Acute kidney injury (AKI) often manifests in patients with liver disease because of a prerenal cause and presents as acute tubular necrosis or hepatorenal syndrome. Distinguishing between these entities is important for prognosis and treatment. Some patients may develop AKI related to their underlying liver disease: for example, membranoproliferative glomerulonephritis or IgA nephropathy. Bile cast nephropathy is an often ignored differential diagnosis of AKI in the setting of obstructive jaundice. It is characterized by the presence of bile casts in renal tubules, which can possibly cause tubular injury through obstructive and direct toxic effects. Thus, AKI in patients with liver disease may have a structural component in addition to a functional one. In this study, we describe 2 patients with severe hyperbilirubinemia who developed AKI and underwent a kidney biopsy that revealed bile casts in tubular lumens, consistent with bile cast nephropathy. One patient was treated aggressively for alcoholic hepatitis and required hemodialysis for AKI. The second patient was treated conservatively for drug-induced liver injury and did not require dialysis. Both patients saw a reduction in their bilirubin and creatinine toward baseline. Bile cast nephropathy is an important pathological entity that may account for the renal dysfunction in some patients with liver disease. It requires kidney biopsy for diagnosis and may often be overlooked given the scarcity of kidney biopsy in this particular clinical setting. The etiology is multifactorial, and it is often difficult to predict without the aid of a renal biopsy.

An experimental study on renal functional disturbance in rabbits after acute ureteral obstruction with dynamic Gd-DTPA enhanced MR imaging

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Shin, Kyoung Ja [Seoul Red Cross Hospital, Seoul (Korea, Republic of); Chung, Eun Chul; Rlee, Chung Sik [Ewha Womans University College of Medicine, Seoul (Korea, Republic of)

1994-05-15

To evaluate the anatomic and functional change in acutely obstructed kidneys, and probability of functional recovery after resolving the obstruction. There were 5 animal groups (6 rabbits in each group)-normal control group, 4 acute ureteral obstruction group. Each of the latter group was imaged with Gd-DTPA enhanced dynamic MR 2, 6, 24, and 48 hours after ligation of the left proximal urter, respectively. We compared the findings of dynamic MR imaging and histopathological findings. We could observe centripetal movement of dark band pattern which could be defined as 3 phases renal cortex, outer medulla, and inner medulla in normal rabbits. The appearance of the dark band pattern was delayed or absent in ureteral obstruction group with linear relationship to the duration of the obstruction. Gd-DTPA enhanced MR can be used to differentiate acute obstructive nephropathy from other causes of nephropathy and foretell the prognosis of the obstruction.

An experimental study on renal functional disturbance in rabbits after acute ureteral obstruction with dynamic Gd-DTPA enhanced MR imaging

International Nuclear Information System (INIS)

Shin, Kyoung Ja; Chung, Eun Chul; Rlee, Chung Sik

1994-01-01

To evaluate the anatomic and functional change in acutely obstructed kidneys, and probability of functional recovery after resolving the obstruction. There were 5 animal groups (6 rabbits in each group)-normal control group, 4 acute ureteral obstruction group. Each of the latter group was imaged with Gd-DTPA enhanced dynamic MR 2, 6, 24, and 48 hours after ligation of the left proximal urter, respectively. We compared the findings of dynamic MR imaging and histopathological findings. We could observe centripetal movement of dark band pattern which could be defined as 3 phases renal cortex, outer medulla, and inner medulla in normal rabbits. The appearance of the dark band pattern was delayed or absent in ureteral obstruction group with linear relationship to the duration of the obstruction. Gd-DTPA enhanced MR can be used to differentiate acute obstructive nephropathy from other causes of nephropathy and foretell the prognosis of the obstruction

Comparative analysis of diabetic nephropathy and non-diabetic nephropathy disease.

Science.gov (United States)

Chen, Qiuxiang; Zhu, Aimin; Wang, Junsheng; Huan, Xuelai

2017-12-01

Clinical symptoms of diabetic nephropathy patients and non-diabetic nephropathy are compared and analyzed, hemodialysis effect and quality of life of two kinds of nephrotic patients are analyzed. Respectively extract 1300 cases of diabetic nephropathy and non-diabetic nephropathy patients admitted to different hospitals during December 2011-December 2014. Based on whether the patient suffers from diabetes, they were divided into diabetic group and control group. Hemodialysis of two groups of patients were followed up to observe effectiveness of blood treatment, and complications were observed after one year of follow-up. Hematodialysis effectiveness of diabetic nephropathy patients is significantly lower than that of non-diabetic nephropathy group. After 1 year's follow-up, it can be found that survival rate of diabetic nephropathy patients is much lower than that of control group. In statistical comparison of data involved in the two groups of patients, P diabetic nephropathy patients is relatively poor compared to that of non-diabetic patients. In clinics, management and prevention of diabetic patients should be strengthened to avoid complication of nephropathy which brings serious injury to patients.

Sonographic findings in Gouty Nephropathy

International Nuclear Information System (INIS)

Kim, Mi Young; Jeon, Woo Ki; Kim, Ho Kyun; Kim, Yong Soo; Han, Chang Yul; Kim, Young Tong; Han, Sung Tag; Lee, Yoon Woo

1994-01-01

Ultrasound(US) findings of hyperechoic renal medulla in gouty nephropathy were compared with clinical features such as serum uric acid level to evaluate its usefulness in determination of the treatment and prognosis. A retrospective review of US of 36 cases of qouty arthritis was classified into four groups according to the medullary echogenicity (O :normal, grade I: renal medulla as isoechoic as renal cortex, grade II; heterogeneous increased echogenicity of renal medulla than that of renal cortex, grade III: the echogenicity of all renal medulla higher than that of renal cortex with renal contour deformity) which were compared with the serum urate level and associated conditions. Nephrocalcinosis and nephrolithiasis were analyzed through the KUB and the RGB. The degree of hyperechoic renal medulla was related to the level of serum uric acid, and in group IV, six cases of obstructive uropathy (nephrocalcinosis and nephrolithiasis) showed deformed renal contour. Associated conditions such as hypertension, alcoholism, diabetes mellitus and drug abuse were distributed in relation to the degree of hyperechoic renal medullas. US findings of hyperechoic renal mebulla was related with uric acid level in gouty nephropathy and thus could be valuable for treatment decision and prediction of prognosis

Sonographic findings in Gouty Nephropathy

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Kim, Mi Young; Jeon, Woo Ki; Kim, Ho Kyun; Kim, Yong Soo; Han, Chang Yul; Kim, Young Tong; Han, Sung Tag; Lee, Yoon Woo [Inje University College of Medicine, Seoul (Korea, Republic of)

1994-09-15

Ultrasound(US) findings of hyperechoic renal medulla in gouty nephropathy were compared with clinical features such as serum uric acid level to evaluate its usefulness in determination of the treatment and prognosis. A retrospective review of US of 36 cases of qouty arthritis was classified into four groups according to the medullary echogenicity (O :normal, grade I: renal medulla as isoechoic as renal cortex, grade II; heterogeneous increased echogenicity of renal medulla than that of renal cortex, grade III: the echogenicity of all renal medulla higher than that of renal cortex with renal contour deformity) which were compared with the serum urate level and associated conditions. Nephrocalcinosis and nephrolithiasis were analyzed through the KUB and the RGB. The degree of hyperechoic renal medulla was related to the level of serum uric acid, and in group IV, six cases of obstructive uropathy (nephrocalcinosis and nephrolithiasis) showed deformed renal contour. Associated conditions such as hypertension, alcoholism, diabetes mellitus and drug abuse were distributed in relation to the degree of hyperechoic renal medullas. US findings of hyperechoic renal mebulla was related with uric acid level in gouty nephropathy and thus could be valuable for treatment decision and prediction of prognosis.

Renal dynamic functional scintigraphy in children with congenital urinary tract anomalies

International Nuclear Information System (INIS)

Cui Ruixue; Zhou Qian

2000-01-01

Objective: To clarify the characteristics of renal scintigraphy in children with various congenital urinary tract anomalies and to evaluate their clinical significance. Methods: 51 children with congenital urinary tract anomalies were studied by 99 Tc m -DTPA renal dynamic functional scintigraphy (RDFS)). Among them, 8 cases were studied by diuretic renography in addition. Results: RDFS provided: 1) Both the morphological information of anomalies and the split renal function. 2) Localization of the site of urinary tract obstruction. In case of pelviureteric junction obstruction, the hydronephrosis was usually severe but with normal size ureter, whereas in ureterovesical junction obstruction the hydronephrosis was less severe but with dilatation of ureter. In case of lower urinary tract obstruction, the hydronephrosis and hydroureter were bilateral. Diuretic renography differentiated obstructive from non-obstructive hydronephrosis. By comparing pre-and post operative studies, it was easy to evaluate the recovery of renal function. Conclusions: RDFS is a valuable modality for giving both anatomical and functional information in children with congenital urinary tract anomalies and the authors suggest it should be used more popularly in Pediatrics

Pushed monocanalicular intubation versus probing as a primary management for congenital nasolacrimal obstruction

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Elsawaby EA

2016-09-01

Full Text Available Emad Abdelaal Elsawaby, Rania Asem El Essawy, Sameh Hassan Abdelbaky, Yomna Magdy Ismail Department of Ophthalmology, Faculty of Medicine, Cairo University, Cairo, Egypt Purpose: Evaluation of efficiency, complications, and advantages of pushed monocanalicular intubation using Masterka® tube versus simple probing in patients with congenital nasolacrimal duct obstruction (CNLDO.Patients and methods: This is a case-controlled study that included 60 eyes (of 53 patients; 30 eyes underwent probing and 30 eyes intubation using the Masterka tube as a primary treatment for CNLDO. The children were aged between 6 months and 36 months at the time of surgery, with no previous nasolacrimal surgical procedure, and had one or more of the following clinical signs of nasolacrimal duct obstruction: epiphora, mucous discharge, and/or increased tear lake.Results: We defined success by absence of epiphora, mucous discharge, or increased tear lake 1 month after tube removal. The overall success rate in the probing group was 80%, while it was 83.3% in the intubation group.Conclusion: Pushed monocanalicular intubation is an effective method for treatment of CNLDO; it requires only mask inhalation anesthesia and could be considered as an appropriate alternative procedure with imperceptible complications. Keywords: epiphora, probing, intubation, monocanalicular, nasolacrimal

Congenital segmental dilatation of the colon

African Journals Online (AJOL)

Congenital segmental dilatation of the colon is a rare cause of intestinal obstruction in neonates. We report a case of congenital segmental dilatation of the colon and highlight the clinical, radiological, and histopathological features of this entity. Proper surgical treatment was initiated on the basis of preoperative radiological ...

Congenital pyriform aperture stenosis

International Nuclear Information System (INIS)

Osovsky, Micky; Aizer-Danon, Anat; Horev, Gadi; Sirota, Lea

2007-01-01

Nasal airway obstruction is a potentially life-threatening condition in the newborn. Neonates are obligatory nasal breathers. The pyriform aperture is the narrowest, most anterior bony portion of the nasal airway, and a decrease in its cross-sectional area will significantly increase nasal airway resistance. Congenital nasal pyriform aperture stenosis (CNPAS) is a rare, unusual form of nasal obstruction. It should be considered in the differential diagnosis of any neonate or infant with signs and symptoms of upper airway compromise. It is important to differentiate this level of obstruction from the more common posterior choanal stenosis or atresia. CNPAS presents with symptoms of nasal airway obstruction, which are often characterized by episodic apnea and cyclical cyanosis. (orig.)

Experience of diagnosis of congenital arterial anomaly in a child with bronchial obstruction: clinical case and summary

Directory of Open Access Journals (Sweden)

L.V. Besh

2017-08-01

Full Text Available Congenital anomalies of the aortic arch are a large group of cardiovascular anomalies. Some of them are isolated; others are associated with congenital heart defects or genetic syndromes. There can be different clinical manifestations of these anomalies — from silent forms (accidentally discovered in the examination to a critical circulatory disturbance, requiring surgical intervention in the first hours after the birth of the baby. The article describes clinical search of double aortic arch in a child and successful surgical treatment of this anomaly. This congenital defect of the aorta usually causes the greatest compression of airways compared to other vascular abnormalities. Double aortic arch develops due to absence of involution of distal IV right aortic arch. As a result, two IV arches keep on developing and join together in the left-sided descending thoracic aorta, thus surrounding the trachea and esophagus. Smaller of the two arches is often subjected to atresia, but it is still connected to the descending aorta, creating a complete vascular ring. As a double aortic arch pressures on the trachea and esophagus, it can cause airway obstruction and dysphagia in a patient. Therapeutic tactic depends on the intensity of symptoms, presence of concomitant anomalies or diseases, and many additional factors, which are analyzed and considered within the council of physicians of different specialties.

Clinical and morphological characteristics of malformations in infants with congenital cytomegalovirus infection and congenital toxoplasmosis

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L. Yu. Barycheva

2015-01-01

Full Text Available The results of following up infants with intrauterine infections and malformations were retrospectively analyzed. Infants with malformations were diagnosed as having congenital cytomegalovirus infection and congenital toxoplasmosis in 127 and 69 cases, respectively. The aim of the study was to characterize malformations in infants with congenital cytomegalovirus and congenital Toxoplasma infections. The infants with malformations in congenital cytomegalovirus infection were found to have higher mortality rates (61,4% than those with congenital toxoplasmosis (34,8%. Postmortem analysis indicated that there was a predominance of embryopathies in infants with congenital cytomegalovirus infection and that of fetopathies in those with congenital toxoplasmosis. The dead infants with congenital cytomegalovirus infection had more commonly developed visceral defects, including heart diseases, pneumopathies, gastrointestinal and genitourinary abnormalities; fetopathies of the central nervous system and eye were prevalent in congenital toxoplasmosis. The surviving children with congenital toxoplasmosis were more frequently observed to have disabling CNS and ocular sequels as obstructive hydrocephalus, infantile cerebral palsy, complete or partial blindness, and cerebrasthenic disorders than those with congenital cytomegalovirus infection. 

Congenital Complete Tracheal Ring in a Neonate: A Case Report

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Esra ARUN ÖZER

2017-09-01

Full Text Available Abnormalities of the upper airway tract lead to congenital high airway obstruction and may complicate neonatal airway management in the delivery room. Congenital complete tracheal rings are a rare and unusual tracheal anomaly, usually presenting in the neonate or infant as respiratory distress. The clinical presentation can vary from almost asymptomatic patients to near-fatal airway obstruction. It may exist as an isolated entity, or in association with other congenital malformations, in particular, cardiac anomalies along with vascular rings and pulmonary slings. Other associated anomalies have also been reported, for example, chromosomal anomalies, malformation of other parts of the respiratory tract, esophagus and skeletal systems. Here, we report an extreme case of VACTERL/TACRD association presented with congenital complete tracheal ring, encephalocele, bilateral radial agenesis with absent thumbs, equinovalgus deformity on right foot, low-set ears and micrognathia.

Radiological evaluation of intestinal obstruction in neonate and infant

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Jeon, D. S.; Lim, K. Y.; Kim, S. J.; Kim, J. D.; Rhee, H. S. [Presbyterian Medical Center, Jeonju (Korea, Republic of)

1980-12-15

281 cases of neonatal and infantile intestinal obstruction confirmed by clinical and surgical procedure from 1975 till 1979 were reviewed radiologically. The result was as follows; 1. Intussusception was the most common cause of intestinal obstruction under one year of age (173/281: 61.56%), and other causes of descending order were infantile hypertrophic pyloric stenosis (20: 7.11%), congenital megacolon (19: 6.76%), anorectal malformation (17: 6.05%), meconium plug syndrome (17: 6.05%), hernia (12: 4.27%), band adhesion (8: 2.85%), rotation anomaly (5: 1.78%), small bowel atresia (3: 1.07%), Meckel's diverticulum (3: 1.07%), duodenal atresia (2: 0.71%), meconium ileus (1: 0.36%) and annular pancreas (1: 0.36%). Congenital type of intestinal obstruction (we classified intussusception acquired and others congenital) occupied 38.44%. 2. The ratio of male to female was 3: 1, congenital type 4.69 : 1 and acquired 2.39 : 1. 3. Vomiting, bloody stool, fever and abdominal distention were the most 4 symptoms. 4. The frequency of typical mechanical ileus pattern on plain abdomen films was 226 cases(80.43%) paralytic ileus or normal finding was 52 cases (18.51%) and pneumoperitoneum with ileus sign was 3 cases (1.06%). 5. Barium meal or enema was performed in 228 cases and narrowing or obstructed site was found in 213 cases of them (93.42%); intussusception 100% (173/173), infantile hypertrophic pyloric stenosis 93.33% (14/15) and congenital megacolon 82.35% (14/17). 6. Only 10 cases had associated disease such as Mongolism, thoracic kyphosis and scoliosis, microcolon, prematurity, ileocolic fistula, undescended testicle and hydrocele. 7. Meconium plug syndrome, duodenal atresia, small bowel atresia, mecondium ileus and annular pancreas were developed early after birth with average onset age of 3.6 days.

Radiological evaluation of intestinal obstruction in neonate and infant

International Nuclear Information System (INIS)

Jeon, D. S.; Lim, K. Y.; Kim, S. J.; Kim, J. D.; Rhee, H. S.

1980-01-01

281 cases of neonatal and infantile intestinal obstruction confirmed by clinical and surgical procedure from 1975 till 1979 were reviewed radiologically. The result was as follows; 1. Intussusception was the most common cause of intestinal obstruction under one year of age (173/281: 61.56%), and other causes of descending order were infantile hypertrophic pyloric stenosis (20: 7.11%), congenital megacolon (19: 6.76%), anorectal malformation (17: 6.05%), meconium plug syndrome (17: 6.05%), hernia (12: 4.27%), band adhesion (8: 2.85%), rotation anomaly (5: 1.78%), small bowel atresia (3: 1.07%), Meckel's diverticulum (3: 1.07%), duodenal atresia (2: 0.71%), meconium ileus (1: 0.36%) and annular pancreas (1: 0.36%). Congenital type of intestinal obstruction (we classified intussusception acquired and others congenital) occupied 38.44%. 2. The ratio of male to female was 3: 1, congenital type 4.69 : 1 and acquired 2.39 : 1. 3. Vomiting, bloody stool, fever and abdominal distention were the most 4 symptoms. 4. The frequency of typical mechanical ileus pattern on plain abdomen films was 226 cases(80.43%) paralytic ileus or normal finding was 52 cases (18.51%) and pneumoperitoneum with ileus sign was 3 cases (1.06%). 5. Barium meal or enema was performed in 228 cases and narrowing or obstructed site was found in 213 cases of them (93.42%); intussusception 100% (173/173), infantile hypertrophic pyloric stenosis 93.33% (14/15) and congenital megacolon 82.35% (14/17). 6. Only 10 cases had associated disease such as Mongolism, thoracic kyphosis and scoliosis, microcolon, prematurity, ileocolic fistula, undescended testicle and hydrocele. 7. Meconium plug syndrome, duodenal atresia, small bowel atresia, mecondium ileus and annular pancreas were developed early after birth with average onset age of 3.6 days

Inhibitory Effect of NH4Cl Treatment on Renal Tgfß1 Signaling Following Unilateral Ureteral Obstruction

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Martina Feger

2015-09-01

Full Text Available Background/Aims: Consequences of obstructive nephropathy include tissue fibrosis, a major pathophysiological mechanism contributing to development of end-stage renal disease. Transforming growth factor β 1 (Tgfβ1 is involved in the progression of renal fibrosis. According to recent observations, ammonium chloride (NH4Cl prevented phosphate-induced vascular remodeling, effects involving decrease of Tgfβ1 expression and inhibition of Tgfβ1-dependent signaling. The present study, thus, explored whether NH4Cl influences renal Tgfβ1-induced pro-fibrotic signaling in obstructive nephropathy induced by unilateral ureteral obstruction (UUO. Methods: UUO was induced for seven days in C57Bl6 mice with or without additional treatment with NH4Cl (0.28 M in drinking water. Transcript levels were determined by RT-PCR as well as protein abundance by Western blotting, blood pH was determined utilizing a blood gas and chemistry analyser. Results: UUO increased renal mRNA expression of Tgfb1, Tgfβ-activated kinase 1 (Tak1 protein abundance and Smad2 phosphorylation in the nuclear fraction of the obstructed kidney tissues, effects blunted in NH4Cl treated mice as compared to control treated mice. The mRNA levels of the transcription factors nuclear factor of activated T cells 5 (Nfat5 and SRY (sex determining region Y-box 9 (Sox9 as well as of tumor necrosis factor α (Tnfα, interleukin 6 (Il6, plasminogen activator inhibitor 1 (Pai1 and Snai1 were up-regulated in the obstructed kidney tissues following UUO, effects again significantly ameliorated following NH4Cl treatment. Furthermore, the increased protein and mRNA expression of α-smooth muscle actin (α-Sma, fibronectin and collagen type I in the obstructed kidney tissues following UUO were significantly attenuated following NH4Cl treatment. Conclusion: NH4Cl treatment ameliorates Tgfβ1-dependent pro-fibrotic signaling and renal tissue fibrosis markers following obstructive nephropathy.

Congenital Scaphoid Megalourethra: A Case Report

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Kenji Obara

2017-09-01

Full Text Available A congenital megalourethra is an enlargement of the pendulous urethra without evidence of distal obstruction. A 1-month-old boy presented to us with complaint of weak stream, ballooning of the penis before and during voiding and post voiding dribbling, since birth. Physical examination and cystourethroscope confirmed the diagnosis of congenital scaphoid megalourethra. He underwent reduction urethroplasty. During postoperative follow up, he had normal looking penis with good urinary stream.

Congenital high airway obstruction syndrome in the breech presentation managed by ex utero intrapartum treatment procedure after intraoperative external cephalic version.

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Miwa, Ichiro; Sase, Masakatsu; Nakamura, Yasuhiko; Hasegawa, Keiko; Kawasaki, Masahiro; Ueda, Kazuyuki

2012-05-01

Congenital high airway obstruction syndrome (CHAOS) caused by laryngeal atresia was diagnosed by prenatal ultrasound in a male fetus at 26 weeks of gestation. Findings included massive ascites, subcutaneous edema, enlarged hyperechogenic lungs with diaphragmatic inversion, dilated trachea, polyhydramnios, and breech presentation. Those findings of CHAOS spontaneously returned to normal by 33 weeks of gestation. However, the placenta was localized to the anterior uterine wall. In addition, the fetal position had been breech until delivery. At 36 weeks of gestation, a planned ex utero intrapartum treatment (EXIT) procedure was performed following intraoperative external cephalic version (ECV) in which the fetus was approached from the posterior wall of the uterus. Laryngoscopy revealed the predicted laryngeal obstruction, and tracheostomy was placed. Intraoperative ECV may be a useful technique in breech presentation before EXIT procedure. © 2012 The Authors. Journal of Obstetrics and Gynaecology Research © 2012 Japan Society of Obstetrics and Gynecology.

Comparative analysis of diabetic nephropathy and non-diabetic nephropathy disease

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Qiuxiang Chen

2017-12-01

Conclusion: Treatment effect of diabetic nephropathy patients is relatively poor compared to that of non-diabetic patients. In clinics, management and prevention of diabetic patients should be strengthened to avoid complication of nephropathy which brings serious injury to patients.

Obstructive renal injury: from fluid mechanics to molecular cell biology.

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Ucero, Alvaro C; Gonçalves, Sara; Benito-Martin, Alberto; Santamaría, Beatriz; Ramos, Adrian M; Berzal, Sergio; Ruiz-Ortega, Marta; Egido, Jesus; Ortiz, Alberto

2010-04-22

Urinary tract obstruction is a frequent cause of renal impairment. The physiopathology of obstructive nephropathy has long been viewed as a mere mechanical problem. However, recent advances in cell and systems biology have disclosed a complex physiopathology involving a high number of molecular mediators of injury that lead to cellular processes of apoptotic cell death, cell injury leading to inflammation and resultant fibrosis. Functional studies in animal models of ureteral obstruction using a variety of techniques that include genetically modified animals have disclosed an important role for the renin-angiotensin system, transforming growth factor-β1 (TGF-β1) and other mediators of inflammation in this process. In addition, high throughput techniques such as proteomics and transcriptomics have identified potential biomarkers that may guide clinical decision-making.

Transrectal Ultrasonographic Findings of Obstructive Male Infertility

International Nuclear Information System (INIS)

Kim, Chang Il; Kim, Yun Hyeon; Kang, Heoung Keun; Kim, Jae Kyu; Park, Jin Gyoon; Park, Heung Il; Park, Kwang Seong

1996-01-01

To evaluate the usefulness of the transrectal ultrasound in infertile men with suspected obstructive lesions of sperm transport system. We performed transrectal US in 26 infertile patients in whom obstructive lesions of sperm transport system were suspected in physical examination and laboratory data. 7.0 MHz transrectal transducer was used and the content of analysis of sonographic findings was the presence of vas deferens, seminal vesicle and ejaculatory duct. Also, we measured the width of seminal vesicle and diameter of ejaculatory duct. Transrectal US revealed an accurate diagnosis in 12 infertile men who had obstructive lesions and associated abnormalities in distal sperm transport system, two of congenital bilateral absence of the vasa deferentia and seminal vesicles, two of congenital unilateral absence of the vas deferens and seminal vesicle, three of dilatation of seminal vesicles, and five of dilatation of ejaculatory ducts and proximal ducts. The other 14 patients who had normal findings on transrectal US were revealed to have testicular failure (10 patients) and obstruction of proximal sperm transport system (4 patients) which were beyond the field-of-view of transrectal US. Transrectal US is the useful diagnostic method for the evaluation of infertile men with suspected obstructive lesions of sperm transport system. It can possibly decrease the need for the invasive vasography and may be helpful in the guidance of appropriate management of male infertility

Imaging of obstructive azoospermia

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Cornud, F. [Clinique Radiologique, 15 Avenue Robert Schuman, F-75 007 Paris (France)]|[Service de Radiologie, Hopital Necker, 169 rue de Sevres, F-75 015 Paris (France); Belin, X. [Clinique Radiologique, 15 Avenue Robert Schuman, F-75 007 Paris (France)]|[Service de Radiologie, Hopital Necker, 169 rue de Sevres, F-75 015 Paris (France); Delafontaine, D. [Medicine de la Reproduction, 8 rue Jean Richepin, F-75 016 Paris (France); Amar, T. [Uro-Andrologist, 19 Avenue Victor Hugo, F-75016, Paris (France); Helenon, O. [Service de Radiologie, Hopital Necker, 169 rue de Sevres, F-75 015 Paris (France); Moreau, J.F. [Service de Radiologie, Hopital Necker, 169 rue de Sevres, F-75 015 Paris (France)

1997-09-01

Obstructive azoospermia represents approximately 10 % of cases of male hypofertility. It is classified according to the volume of ejaculate. When the latter is normal a proximal obstruction is suspected. Scrotal sonography can help to detect dilation of the epididymal head when clinical findings are equivocal. Ejaculatory duct obstruction (EDO) is suspected when the volume of ejaculate is low. The use of transrectal ultrasonography (TRUS) plays a major role in the investigation of these patients, and endorectal MRI is a very useful adjunct in selected cases. The most common cause of EDO is congenital bilateral absence of vas deferens, which is now thought to be a genital form of cystic fibrosis in 80 % of cases. Consequently, a definitive diagnosis must be made before any attempt at in vitro fertilization. TRUS accurately visualizes abnormalities of the caudal junction of the vas deferens and seminal vesicles, yielding a definitive diagnosis without scrototomy. Other causes of EDO are congenital cysts compressing the distal part of the ejaculatory ducts and inflammatory distal stenosis. The former are accurately identified by TRUS, but the latter give more or less marked signs of obstruction which are only of value in azoospermic patients with a low-volume ejaculate. More invasive imaging is required to diagnose partial obstruction of the ED. Surgical vasography is still the reference, but puncture of the seminal vesicles under TRUS guidance is an attractive alternative, as it permits aspiration of seminal fluid (to seek motile sperm) and vasography without scrototomy. Lastly, endorectal MRI well assesses the relationships between the proximal prostatic urethra and the posterior wall of the ejaculatory ducts, which need to be precisely known when endoscopic resection of the ejaculatory ducts is planned. (orig.). With 9 figs.

Imaging of obstructive azoospermia

International Nuclear Information System (INIS)

Cornud, F.; Belin, X.; Delafontaine, D.; Amar, T.; Helenon, O.; Moreau, J.F.

1997-01-01

Obstructive azoospermia represents approximately 10 % of cases of male hypofertility. It is classified according to the volume of ejaculate. When the latter is normal a proximal obstruction is suspected. Scrotal sonography can help to detect dilation of the epididymal head when clinical findings are equivocal. Ejaculatory duct obstruction (EDO) is suspected when the volume of ejaculate is low. The use of transrectal ultrasonography (TRUS) plays a major role in the investigation of these patients, and endorectal MRI is a very useful adjunct in selected cases. The most common cause of EDO is congenital bilateral absence of vas deferens, which is now thought to be a genital form of cystic fibrosis in 80 % of cases. Consequently, a definitive diagnosis must be made before any attempt at in vitro fertilization. TRUS accurately visualizes abnormalities of the caudal junction of the vas deferens and seminal vesicles, yielding a definitive diagnosis without scrototomy. Other causes of EDO are congenital cysts compressing the distal part of the ejaculatory ducts and inflammatory distal stenosis. The former are accurately identified by TRUS, but the latter give more or less marked signs of obstruction which are only of value in azoospermic patients with a low-volume ejaculate. More invasive imaging is required to diagnose partial obstruction of the ED. Surgical vasography is still the reference, but puncture of the seminal vesicles under TRUS guidance is an attractive alternative, as it permits aspiration of seminal fluid (to seek motile sperm) and vasography without scrototomy. Lastly, endorectal MRI well assesses the relationships between the proximal prostatic urethra and the posterior wall of the ejaculatory ducts, which need to be precisely known when endoscopic resection of the ejaculatory ducts is planned. (orig.). With 9 figs

Fetal tracheolaryngeal airway obstruction: prenatal evaluation by sonography and MRI

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Courtier, Jesse; Poder, Liina; Wang, Zhen J.; Westphalen, Antonio C.; Yeh, Benjamin M.; Coakley, Fergus V. [University of California San Francisco, Department of Radiology, San Francisco, CA (United States)

2010-11-15

We reviewed the sonographic and MRI findings of tracheolaryngeal obstruction in the fetus. Conditions that can cause tracheolaryngeal obstruction include extrinsic causes such as lymphatic malformation, cervical teratoma and vascular rings and intrinsic causes such as congenital high airway obstruction syndrome (CHAOS). Accurate distinction of these conditions by sonography or MRI can help facilitate parental counseling and management, including the decision to utilize the ex utero intrapartum treatment (EXIT) procedure. (orig.)

[Myosin B ATPase activity of the intestinal smooth muscle in intestinal obstruction].

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Takamatsu, H

1983-06-01

Intestinal smooth myosin B was prepared from muscle layers around the lesion in dogs with experimental colonic stenosis and in patients with congenital intestinal obstruction. Mg2+-ATPase activity of the myosin B was compared between the proximal dilated segment and distal segment to obstruction. Experimental colonic stenosis: In early period after surgery, proximal colons showed higher activity of myosin B ATPase than distal colons, decreasing to less than distal colon as time passed. Congenital intestinal obstruction: In three cases, whose atresia might have occurred at earlier period of gestation, proximal bowels showed less activity of myosin B ATPase than distal bowels. However, in two cases, whose atresia might have occurred at later period of gestation, and two cases with intestinal stenosis, proximal bowels indicated higher activity of myosin B ATPase than distal bowels. These data suggested that the contractibility of the proximal intestine was depending on the duration of obstruction, and it was depressed in the former patients and was accelerated in the latter patients. These results suggested that the extensive resection of dilated proximal bowel in the congenital atresia is not always necessary to obtain good postoperative intestinal dynamics at the operation of the atresial lesions which may be induced at later period of gestation. They also suggested that surgery for intestinal obstruction should be performed before the depression of intestinal contractibility to get good bowel function.

Fetal tracheolaryngeal airway obstruction: prenatal evaluation by sonography and MRI

Science.gov (United States)

Courtier, Jesse; Poder, Liina; Wang, Zhen J.; Westphalen, Antonio C.; Yeh, Benjamin M.

2010-01-01

We reviewed the sonographic and MRI findings of tracheolaryngeal obstruction in the fetus. Conditions that can cause tracheolaryngeal obstruction include extrinsic causes such as lymphatic malformation, cervical teratoma and vascular rings and intrinsic causes such as congenital high airway obstruction syndrome (CHAOS). Accurate distinction of these conditions by sonography or MRI can help facilitate parental counseling and management, including the decision to utilize the ex utero intrapartum treatment (EXIT) procedure. PMID:20737145

Congenital milia En plaque on scalp

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Sangita Ghosh

2015-01-01

Full Text Available Milia en plaque is a rare disease entity characterized by confluence of multiple keratin-filled cysts resulting from the obstruction of hair follicle without any preceding primary dermatosis. Fewer than 40 cases have been reported so far in dermatological literature, and most cases are described to occur in adults and in the peri-auricular area. We describe a case of congenital MEP on scalp of a five-year-old boy with a blaschkoid extension into posterior nuchal area. This case report claims its uniqueness because of the unusual site and congenital presentation.

Ureteral valve masquerading as obstructive megaureter

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Sushmita Bhatnagar

2017-05-01

Full Text Available Primary obstructed megaureter is a common urological condition in the pediatric age group. It is one of the differentials for congenital anomalies of the kidney and urinary tract that include a myriad of structural anomalies of the urinary tract. The obstructive conditions are usually managed conservatively unless there is deterioration in renal function, repeated urinary tract infection, or any other symptom such as abdominal pain on ipsilateral side and hypertension. Presented here is a case of left lower ureteral valve that was diagnosed as primary obstructed megaureter with pain in abdomen and reduced ipsilateral renal function wherein ureteral valve was detected incidentally intraoperatively. Excision of the valve with end to end uretero-ureteral anastomoses over a D-J stent, without resorting to ureteric reimplantation was successful in relieving the obstruction and hydronephrosis

Dual Pathology Causing Congenital Bladder Outlet Obstruction.

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Kwong, Ruth; Johal, Navroop S; Upasani, Anand; Paul, Anu; Cuckow, Peter

2017-12-07

Anterior urethral syringocele is an uncommon congenital deformity characterised by cystic dilatation of bulbo-urethral gland ducts and is usually asymptomatic. We present a case on 4-day-old male neonate who presented with bilateral antenatal hydroureteronephrosis and renal impairment and found to have urethral syringocele and posterior urethral valves (PUV). Copyright © 2017. Published by Elsevier Inc.

Genetics of diabetic nephropathy

DEFF Research Database (Denmark)

Parving, H H; Tarnow, L; Rossing, P

1996-01-01

factor for cardiovascular disease in diabetic patients. However, a meta-analysis does not support the suggestion that this factor plays any role for the initiation of diabetic nephropathy. Similar negative results have been obtained in relation to polymorphisms of the genes encoding for angiotensinogen......Diabetic nephropathy is a clinical syndrome characterized by persistent albuminuria, a relentless decline in GFR, raised arterial blood pressure, and increased relative mortality for cardiovascular diseases. Diabetic nephropathy is a leading cause of end-stage renal failure. The pathogenesis...... of diabetic nephropathy is multifactorial, with contributions from metabolic abnormalities, hemodynamic alterations, and various growth factors and genetic factors. Epidemiologic and family studies have demonstrated that only a subset of the patients develop this complication that family clustering...

[Ultrasound and color Doppler applications in nephrology. The normal kidney: anatomy, vessels and congenital anomalies].

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Meola, Mario; Petrucci, Ilaria; Giovannini, Lisa; Samoni, Sara; Dellafiore, Carolina

2012-01-01

Gray-scale ultrasound is the diagnostic technique of choice in patients with suspected or known renal disease. Knowledge of the normal and abnormal sonographic morphology of the kidney and urinary tract is essential for a successful diagnosis. Conventional sonography must always be complemented by Doppler sampling of the principal arterial and venous vessels. B-mode scanning is performed with the patient in supine, prone or side position. The kidney can be imaged by the anterior, lateral or posterior approach using coronal, transverse and oblique scanning planes. Morphological parameters that must be evaluated are the coronal diameter, the parenchymal thickness and echogenicity, the structure and state of the urinary tract, and the presence of congenital anomalies that may mimic a pseudomass. The main renal artery and the hilar-intraparenchymal branches of the arterial and venous vessels should be accurately evaluated using color Doppler. Measurement of intraparenchymal resistance indices (IP, IR) provides an indirect and quantitative parameter of the stiffness and eutrophic or dystrophic remodeling of the intrarenal microvasculature. These parameters differ depending on age, diabetic and hypertensive disease, chronic renal glomerular disease, and interstitial, vascular and obstructive nephropathy.

Congenital Anomalies of the Kidney and the Urinary Tract (CAKUT)

Science.gov (United States)

2014-01-01

This article reviews the majority of Congenital Anomalies of the Kidney and Urinary Tract (CAKUT) with emphasis in Pediatric Pathology describing and illustrating lesions as varied as ureteral duplications, ureteropelvic junction obstruction, horseshoe kidney, posterior urethral valve and prune belly syndrome, obstructive renal dysplasia, nonmotile ciliopathies and several syndromes associated with renal malformations (Meckel–Joubert, short rib, Bardet–Biedl, asplenia/polysplenia, hereditary renal adysplasia, Zellweger, trisomies, VACTER-L, Potter, caudal dysplasia, and sirenomelia), as well as ADPK, and ARPK. The purpose of this review is not only to describe the congenital renal anomalies, but also to analyze the more recent therapeutic interventions that may modify the natural history of some of these severe conditions. PMID:25313840

ATP5B and ETFB metabolic markers in children with congenital hydronephrosis.

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Zhao, Qi; Yang, Yi; Wang, Changlin; Hou, Ying; Chen, Hui

2016-12-01

Congenital obstructive nephropathy is the primary cause of chronic renal failure in children. Disorders of mitochondrial energy metabolism may be a primary factor underlying tubular cell apoptosis in hydronephrosis. The β-F1-ATPase (ATP5B) and electron transfer flavoprotein β subunit (ETFB) metabolic markers are involved in mitochondrial energy metabolism in other diseases. The aim of the present study was to evaluate whether ATP5B and ETFB are represented in the hydronephrotic kidney, and whether they are associated with the progression of hydronephrosis. The cohort examined consisted of 20 children with hydronephrosis, graded III and IV using the Society for Fetal Urology grading system, and a control group consisting of 20 patients with nephroblastoma. Reverse transcription‑quantitative polymerase chain reaction and immunoblot analyses were used to investigate the differential expression of genes and proteins in the two groups. The gene and protein expression levels of ATP5B and ETFB were upregulated in the hydronephrosis group. Correlation analyses revealed negative correlations between ATP5B, ETFB protein and split renal function (SRF). Receiver‑operator curve analysis found a diagnostic profile of the ETFB protein in identifying children with hydronephrosis with abnormal SRF (hydronephrosis and require further detailed investigation.

MR imaging of congenital heart disease

International Nuclear Information System (INIS)

Kersting-Sommerhoff, B.A.; Diethelm, L.; Teitel, D.F.; Sommerhoff, C.P.; Higgins, C.B.

1988-01-01

Sensitivity and specificity of MR imaging for the evaluation of congenital heart disease was assessed in 51 patients (31 male and 20 female, aged 3-69 years), with a total of 115 congenital heart lesions. The true diagnosis was established by angiocardiography, catheterization, or surgery. Sensitivity at a specificity level of 90% was determined by means of receiver operating characteristic curves for great vessel relationships (100%), thoracic aorta anomalies (94%), atrial (91%) and ventricular (100%) septal defects, visceroatrial situs (100%), loop (100%), right ventricular outflow obstructions (95%), aortic valve (52%), mitral valve (62%), and tricuspid valve (76%). Spin-echo MR imaging is a reliable method for the noninvasive evaluation of congenital heart disease but is limited in the assessment of some valvular anomalies

Serious life threatening upper airway obstruction in congenital ...

African Journals Online (AJOL)

Reda A. Abolila

2012-07-15

Jul 15, 2012 ... hamartoma, multiple vascular leiomyoma of newborn, diffuse congenital fibromatosis ... in the literatures that about one third of such infants die in the first 4 months of life from .... Juvenile fibromatosis. Cancer 1954;7:953–78.

Diabetic nephropathy : pathology, genetics and carnosine metabolism

NARCIS (Netherlands)

Mooyaart, Antien Leonora

2011-01-01

My thesis concerns different aspects of diabetic nephropathy. A pathologic classification of diabetic nephropathy is developed, a meta-analyis of genes in diabetic nephropathy is developed and the other chapters are about the CNDP1 gene in relation to kidney disease, mainly diabetic nephropathy.

Fatal Airway Obstruction in a Man With a Cystic Hygroma.

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Wygant, Cassandra Maria; Cohle, Stephen D

2018-05-03

We describe a 24-year-old man with a cystic hygroma of the left side of the lower neck that led to sudden death. Cystic hygroma (cystic lymphangioma) is a congenital malformation of the lymphatic system. The patient, who had a tracheostomy because of airway obstruction from the cystic hygroma, was found dead with his tracheostomy tube on the floor next to him. Complications of cystic hygroma include infiltration of the neck causing airway obstruction, dysphagia, pain, and obstructive sleep apnea.

Deprivation amblyopia and congenital hereditary cataract.

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Mansouri, Behzad; Stacy, Rebecca C; Kruger, Joshua; Cestari, Dean M

2013-01-01

Amblyopia is a neurodevelopmental disorder of vision associated with decreased visual acuity, poor or absent stereopsis, and suppression of information from one eye.(1,2) Amblyopia may be caused by strabismus (strabismic amblyopia), refractive error (anisometropic amblyopia), or deprivation from obstructed vision (deprivation amblyopia). 1 In the developed world, amblyopia is the most common cause of childhood visual impairment, 3 which reduces quality of life 4 and also almost doubles the lifetime risk of legal blindness.(5, 6) Successful treatment of amblyopia greatly depends on early detection and treatment of predisposing disorders such as congenital cataract, which is the most common cause of deprivational amblyopia. Understanding the genetic causes of congenital cataract leads to more effective screening tests, early detection and treatment of infants and children who are at high risk for hereditary congenital cataract.

Long-term follow-up of reflux nephropathy in adults with vesicoureteral reflux - radiological and pathoanatomical analysis

International Nuclear Information System (INIS)

Koehler, J.; Thysell, H.; Tencer, J.; Forsberg, L.; Hellstroem, M.

2001-01-01

Purpose: To study the long-term development of urographic renal morphology in adults with vesicoureteral reflux, to investigate the relationship between renal damage and reflux grade, and to analyse the association between the long-term urographic outcome and the occurrence of acute pyelonephritis and reflux during follow-up. The purpose was also to try to distinguish between acquired and developmental renal damage, based on analyses of renal histological specimens and urographic features, and to analyse associated congenital urogenital abnormalities and family history of reflux, reflux nephropathy, urological malformation or death from end-stage renal disease. Material and Methods: Renal damage was identified in 100 (83 women) of 115 adults, selected because of documented reflux. Eighty-seven patients had two urographies done (median interval 14.3 years). The extent and progression of renal damage were assessed and features of developmental renal damage were determined. Histological renal specimens were available in 23 patients with renal damage. Results and Conclusions: The extent of renal damage correlated positively with the severity of reflux. No renal damage developed during the follow-up in 45 previously undamaged kidneys and progression of renal damage was rare (4 of 120 previously damaged kidneys), despite persisting reflux in half of the cases and episodes of acute pyelonephritis during follow-up. Thus, repeated renal imaging is rarely justified in adults with reflux nephropathy. Histological examination showed 'chronic pyelonephritis' in all 23 cases and co-existing renal dysplasia in 1 case. The detailed urographic analysis did not reveal support for developmental renal damage. High frequencies of associated congenital urogenital abnormalities and of a positive family history were found. Thus, congenital and/or hereditary factors cannot be discarded as background factors for the development of renal damage

Diuretic ultrasound - noninvasive method for assessment of congenital hydronephrosis in children

International Nuclear Information System (INIS)

Bueva, A.; Gaidarova, M.; Zlatanova, G.

2012-01-01

Ureteropelvic junction obstruction is the most common congenital abnormality of the urinary tract. Its incidence is 5 cases per 100 000 population annually. Several functional methods are available: diuretic renography, diuretic ultrasound and diuretic urography. The aim of this study is to compare the sensitivity and the specificity of the diuretic ultrasound and renography in the evaluation of upper urinary tract obstruction. (authors)

GENETICS ASPECTS OF DIABETIC NEPHROPATHY

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Oana-Elena Sauca

2010-09-01

Full Text Available Diabetic nephropathy is a clinical syndrome characterized by persistent albuminuria, a relentless decline in GFR, raised arterial blood pressure, and increased relative mortality for cardiovascular diseases. The pathogenesis of diabetic nephropathy is multifactorial, with contributions from metabolic abnormalities, hemodynamic alteration, and various growth and genetic factors. The identification of the main genes would allow the detection of those individuals at high risk for diabetic nephropathy and better understanding of its pathophysiologyas well.The present review discusses the main information available in literature regarding some genetic variants (involved in the renin-angiotensin system, glucose and lipid metabolism and some cytoskeleton proteins that reaffirms the importance of genetic factors in diabetic nephropathy.

Congenital heart surgery: what we do to our patients

African Journals Online (AJOL)

congenital heart surgery does not end at discharge at the door of the ... of obstructed right ventricular outflow, for example in tetralogy of. Fallot, or ... Patent ductus arteriosus (PDA). PDAs are commonly closed by transvascular catheter ...

Dusp16 Deficiency Causes Congenital Obstructive Hydrocephalus and Brain Overgrowth by Expansion of the Neural Progenitor Pool

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Ksenija Zega

2017-11-01

Full Text Available Hydrocephalus can occur in children alone or in combination with other neurodevelopmental disorders that are often associated with brain overgrowth. Despite the severity of these disorders, the molecular and cellular mechanisms underlying these pathologies and their comorbidity are poorly understood. Here, we studied the consequences of genetically inactivating in mice dual-specificity phosphatase 16 (Dusp16, which is known to negatively regulate mitogen-activated protein kinases (MAPKs and which has never previously been implicated in brain development and disorders. Mouse mutants lacking a functional Dusp16 gene (Dusp16−/− developed fully-penetrant congenital obstructive hydrocephalus together with brain overgrowth. The midbrain aqueduct in Dusp16−/− mutants was obstructed during mid-gestation by an expansion of neural progenitors, and during later gestational stages by neurons resulting in a blockage of cerebrospinal fluid (CSF outflow. In contrast, the roof plate and ependymal cells developed normally. We identified a delayed cell cycle exit of neural progenitors in Dusp16−/− mutants as a cause of progenitor overproliferation during mid-gestation. At later gestational stages, this expanded neural progenitor pool generated an increased number of neurons associated with enlarged brain volume. Taken together, we found that Dusp16 plays a critical role in neurogenesis by balancing neural progenitor cell proliferation and neural differentiation. Moreover our results suggest that a lack of functional Dusp16 could play a central role in the molecular mechanisms linking brain overgrowth and hydrocephalus.

Unusual cause of neonatal intestinal obstruction | Zikavska ...

African Journals Online (AJOL)

There are many causes of intestinal obstruction in the neonatal age. The most common types are mechanical and result from congenital malformations of the gastrointestinal tract. However, functional disorders also occur. In some cases, diagnosis can be made prenatally but in others manifestation occurs after birth. The aim ...

[Surgical treatment of congenital obstruction of the left ventricular outflow tract].

Science.gov (United States)

Biocina, B; Sutlić, Z; Husedinović, I; Letica, D; Sokolić, J

1993-01-01

This report presents the classification and all types of left ventricular outflow tract obstructions. The possibilities of operative therapies are surveyed as well. Results of surgical treatment in 34 patients with obstruction to left ventricular outflow are shown. The majority of patients underwent operation under extracorporeal circulation (84.4%), while the rest were operated by means of the inflow occlusion technique (14.7%). The obtained results were compared with those from the literature. The importance of echocardiographic evaluation of location of the left ventricular outflow tract obstruction and the appropriate choice of a surgical technique according to the patient's age are emphasized.

Acute bile nephropathy secondary to anabolic steroids.

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Alkhunaizi, Ahmed M; ElTigani, Mohamed A; Rabah, Rola S; Nasr, Samih H

2016-02-01

Renal dysfunction in cholestatic liver disease is multifactorial. Acute kidney injury may develop secondary to renal vasoconstriction in the setting of peripheral vasodilation and relative hypovolemia, tubular obstruction by bile casts, and direct tubular toxicity from bile. Anabolic steroids are frequently used by athletes to boost endurance and increase muscle mass. These agents are a recently recognized cause of hepatotoxicity and jaundice and may lead to acute kidney injury. To increase awareness about this growing problem and to characterize the pathology of acute kidney injury in this setting, we report on a young male who developed acute kidney injury in the setting of severe cholestatic jaundice related to ingestion of anabolic steroids used for bodybuilding. Kidney biopsy showed bile casts within distal tubular lumina, filamentous bile inclusions within tubular cells, and signs of acute tubular injury. This report supports the recently re-emerged concept of bile nephropathy cholemic nephrosis.

Diuretic renography in neonates and infants with congenital obstructive hydronephrosis for evaluating post-operative renal function

International Nuclear Information System (INIS)

Li Jianing; Yang Shurong; Fu Hongliang; Gu Zhenhui; Chen Fang; Xie Hua; Zou Renjian; Wu Jingchuan

2003-01-01

Objective: To study the value of diuretic renography (DR) in evaluation of renal function after operation in congenital obstructive hydronephrosis (HN) in neonates and infants. Methods: Thirty-two patients with the disease detected in their neonatal or infantile period were submitted to this study. DR was performed 1 to 4 times on all patients during follow-up. The qualitative evaluation of renal function after operation was combined with renal morphology, renogram curve and several other renal function indexes. The major index of quantitative evaluation of renal function is the change of renal blood perfusion rate (BPR). Results: The results of qualitative evaluation with 67 DR in the affected kidney (AK): 6 totally recovered, 35 significantly improved, 7 improved, 9 unchanged, 10 deteriorated. The changes of BPR of the groups with qualitative evaluation differed from each other significantly (F=7.77, P 0.05 and r=0.166, P>0.05, respectively). Conclusion: The change of BPR is a good index of renal function after operation, but only the quantitative evaluation being combined with qualitative evaluation can be considered a complete evaluation

Malrotation with transverse colon volvulus in early pregnancy: a rare cause for acute intestinal obstruction

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Sharma, Digvijoy; Parameshwaran, Rajesh; Dani, Tushar; Shetty, Prashanth

2013-01-01

Colonic volvulus is a relatively uncommon cause of large bowel obstruction, accounting for 10% of colonic obstructions. Volvulus of the transverse colon is quite rare, accounting for only 4–11% of all reported cases. We report an unusual case of documented volvulus of the transverse colon in a pregnant woman with intestinal malrotation and concomitant acute intestinal obstruction by congenital bands and adhesions. PMID:23964051

Congenital tracheal defects: embryonic development and animal models

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Zenab Arooj Sher

2016-03-01

Full Text Available Tracheal anomalies are potentially catastrophic congenital defects. As a newborn begins to breathe, the trachea needs to maintain an appropriate balance of elasticity and rigidity. If the tracheal cartilages are disorganized or structurally weak, the airways can collapse, obstructing breathing. Cartilage rings that are too small or too rigid can also obstruct breathing. These anomalies are frequently associated with craniofacial syndromes, and, despite the importance, are poorly understood. In this review, we summarize the spectrum of pathological phenotypes of the trachea and correlate them with the molecular events uncovered in mouse models.

Case Report: Congenital aganglionic megacolon in Nigerian adults ...

African Journals Online (AJOL)

Congenital aganglionic mega colon (Hirschsprung's disease) is a motor disorder in the gut, due to a defect in the craniocaudal migration of the neuroblast originating from the neural crest that occurs during the first twelve weeks of gestation, causing a functional intestinal obstruction, with its attendant complications, ...

Dilatação por cateter-balão (dacrioplastia para tratamento das obstruções congênitas do ducto lacrimonasal Balloon-catheter dilatation (dacrioplasty for congenital nasolacrimal duct obstruction treatment

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Roberto Murillo Limongi de Souza Carvalho

2009-10-01

Full Text Available OBJETIVO: Verificar o resultado da técnica, inédita no Brasil, de dilatação do ducto lacrimonasal usando cateter-balão para tratamento da obstrução congênita do ducto lacrimonasal. MÉTODOS: Foram tratadas crianças com idade entre dois e cinco anos, com obstrução congênita de vias lacrimais usando o cateter-balão (Lacricath® para dilatação das vias lacrimais. O cateter foi introduzido pelo ponto lacrimal superior, sob anestesia geral. RESULTADOS: Entre os 27 olhos tratados, 23 apresentaram desaparecimento dos sinais e sintomas de lacrimejamento (taxa de sucesso de 85,2%. Todos os procedimentos foram realizados sem intercorrências e o seguimento pós-operatório mínimo foi de um ano. CONCLUSÃO: A dilatação com balão mostrou-se segura e eficiente como primeiro procedimento para os casos de obstrução congênita das vias lacrimais em crianças com mais de dois anos.Purpose: To verify the results of balloon-catheter dilatation for the treatment of congenital lacrimal duct obstruction. To the best of author's knowledge this procedure has not been previously used in Brazil and deserves scientific studies. METHODS: Children between 2 and 5 years old, with congenital lacrimal duct obstruction, were treated with balloon-catheter dilatation (Lacricath®. Catheter was introduced by the superior lacrimal punctum, under general anesthesia. RESULTS: Twenty-seven eyes were treated and 23 presented total improvement of epiphora signs and symptoms (85.2% success rate. During a one year follow-up, all the procedures had good outcomes, without complications and all the children had 1 year of follow-up. CONCLUSION: Balloon-catheter dilatation is an efficient and safe procedure and might be used for congenital lacrimal duct obstruction treatment in children older than 2 years.

Congenital intracerebral teratoma: a rare differential diagnosis in newborn hydrocephalus

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Storr, U. [Landratsamt Neuburg-Schrobenhausen, Gesundheitsamt, Neuburg an der Donau (Germany)]|[Hospital for Sick Children, Erlangen-Nuernberg Univ., Nuernberg (Germany); Rupprecht, T. [Hospital for Sick Children, Erlangen-Nuernberg Univ., Nuernberg (Germany); Bornemann, A. [Inst. for General Pathology, Erlangen-Nuernberg Univ., Nuernberg (Germany); Ries, M. [Hospital for Sick Children, Erlangen-Nuernberg Univ., Nuernberg (Germany); Beinder, E. [Dept. of Obstetrics and Gynecology, Erlangen-Nuernberg Univ., Nuernberg (Germany); Boewing, B. [Hospital for Sick Children, Erlangen-Nuernberg Univ., Nuernberg (Germany); Harms, D. [Hospital for Sick Children, Erlangen-Nuernberg Univ., Nuernberg (Germany)

1997-03-01

Cogenital hydrocephalus is caused by a broad spectrum of underlying disorders. In the majority of cases it is due to aqueductal stenosis and other distinct congenital anomalies, like Arnold-Chiari malformation. Nevertheless, in the differential diagnosis rare conditions such as cerebral malignancies must also be considered. We present two cases of congenital intracerebral teratoma as a differential diagnosis in congenital obstructive hydrocephalus. A teratoma is suggested when a rapidly growing hydrocephalus with a central calcified and vascularized mass is found sonographically. Regular cerebral structures using cannot be detected. Early diagnosis in such cases is of clinical importance as the prognosis of congential intracerebral teratoma is generally very poor. (orig.)

Congenital intracerebral teratoma: a rare differential diagnosis in newborn hydrocephalus

International Nuclear Information System (INIS)

Storr, U.; Rupprecht, T.; Bornemann, A.; Ries, M.; Beinder, E.; Boewing, B.; Harms, D.

1997-01-01

Cogenital hydrocephalus is caused by a broad spectrum of underlying disorders. In the majority of cases it is due to aqueductal stenosis and other distinct congenital anomalies, like Arnold-Chiari malformation. Nevertheless, in the differential diagnosis rare conditions such as cerebral malignancies must also be considered. We present two cases of congenital intracerebral teratoma as a differential diagnosis in congenital obstructive hydrocephalus. A teratoma is suggested when a rapidly growing hydrocephalus with a central calcified and vascularized mass is found sonographically. Regular cerebral structures using cannot be detected. Early diagnosis in such cases is of clinical importance as the prognosis of congential intracerebral teratoma is generally very poor. (orig.)

The Endoscopic Morphological Features of Congenital Posterior Urethral Obstructions in Boys with Refractory Daytime Urinary Incontinence and Nocturnal Enuresis.

Science.gov (United States)

Nakamura, Shigeru; Hyuga, Taiju; Kawai, Shina; Kubo, Taro; Nakai, Hideo

2016-08-01

Purpose This study aims to evaluate the endoscopic morphological features of congenital posterior urethral obstructions in boys with refractory daytime urinary incontinence and/or nocturnal enuresis. Patients and Methods A total of 54 consecutive patients underwent endoscopy and were diagnosed with a posterior urethral valve (PUV) (types 1-4). PUV type 1 was classified as severe, moderate, or mild. A transurethral incision (TUI) was mainly performed for anterior wall lesions of the PUV. Voiding cystourethrography and pressure flow studies (PFS) were performed before and 3 to 4 months after TUI. Clinical symptoms were evaluated 6 months after TUI, and outcomes were assessed according to PFS waveform pattern groups (synergic pattern [SP] and dyssynergic pattern [DP]). Results All patients had PUV type 1 and/or 3 (i.e., n = 34 type 1, 7 type 3, and 13 types 1 and 3). There were severe (n = 1), moderate (n = 21), and mild (n = 25) cases of PUV type 1. According to PFS, SP and DP were present in 43 and 11 patients, respectively. TUI was effective in the SP group and symptoms improved in 77.4 and 69.3% of patients with daytime incontinence and nocturnal enuresis, respectively. Almost no effect was observed in the DP group. A significant decrease in the detrusor pressure was observed at maximum flow rate using PFS in the SP group. Conclusions PUV type 1 encompassed lesions with a spectrum of obstructions ranging from severe to mild, with mild types whose main obstructive lesion existed at the anterior wall of urethra occurring most frequently in boys with refractory daytime urinary incontinence and/or nocturnal enuresis. Georg Thieme Verlag KG Stuttgart · New York.

Chronic airway obstruction in children. Most common causes, diagnosis and Surgical and Endoscopic Treatment

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Ana Isabel NAVAZO-EGUÍA

2018-03-01

Full Text Available Introduction: Obstructive airway pathology in children may be congenital or acquired. Fortunately, it is a rare condition, but in most cases, it implies a diagnostic and therapeutic challenge. Endoscopic techniques have experienced in the last years a breakthrough in the treatment of these lesions, but open surgery still plays an important role. Objective: Our objective was to review the most frequent causes of obstruction of the central airway in the child, their diagnostic and current treatment status. Conclusions: Obstructive airway pathology in children requires immediate stabilization, detailed assessment, meticulous planning and individualized treatment. Management of patients with airway obstruction requires a close cooperation between specialists organized in multidisciplinary teams, whose main objective is the diagnosis and timely resolution of the various congenital malformations and acquired lesions that affect the larynx, trachea and bronchi. In many cases the decision of the treatment of choice is not easy. Selection of the most suitable treatment depends on the patient’s clinical situation and the anatomic type of stenosis. The surgeon must master all available techniques, to solve each specific situation.

Endoscopic third ventriculostomy for obstructive hydrocephalus

International Nuclear Information System (INIS)

Ali, M.; Usman, M.; Khan, Z.; Khan, K.M.; Hussain, R.; Khanzada, K.

2013-01-01

To determine the success rate of endoscopic third ventriculostomy (ETV) for treating obstructive hydrocephalus. Study Design: Cross-sectional observational study. Place and Duration of Study: The Neurosurgery Department of PGMI, Lady Reading Hospital, Peshawar, from May 2010 to November 2011. Methodology: Patients with obstructive hydrocephalus due to aqueductal stenosis, tectal and non-tectal tumour and already shunted patients for obstructive hydrocephalus presented with blocked shunt were included in the study. Patients with congenital hydrocephalus and secondary to meningitis were excluded. Endoscopic third ventriculostomy was performed. Success, complications and mortality was noted. Data was analyzed by descriptive statistics using SPSS software version 17. Results: There were 155 patients including 72 males and 83 females with ratio of 1: 1.33. Success rate was 71%. Indication of surgery was obstructive hydrocephalus due to aqueductal stenosis, posterior fossa tumour, brain stem and CP angle tumour. Complications were seen in 18 patients including mortality in 3 patients. Conclusion: ETV is effective, safe and successful procedure in patients with obstructive hydrocephalus. It may be used as replacement procedure of ventriculo-peritoneal shunt as initial line of management in selected patients. (author)

Anionic and cationic drug secretion in the isolated perfused rat kidney after neonatal surgical induction of ureteric obstruction.

NARCIS (Netherlands)

Gier, R.P.E. de; Feitz, W.F.J.; Masereeuw, R.; Wouterse, A.C.; Smits, D.; Russel, F.G.M.

2003-01-01

OBJECTIVE: To study the pathophysiological changes of renal tubular drug transport mechanisms in congenital renal obstruction, by developing a model for perfusing the isolated kidney (IPK) after neonatal surgical induction of partial ureteric obstruction in Hanover Wistar rats. MATERIAL AND METHODS:

Proliferative retinopathy predicts nephropathy

DEFF Research Database (Denmark)

Karlberg, Charlotte; Falk, Christine; Green, Anders

2012-01-01

We wanted to examine proliferative retinopathy as a marker of incident nephropathy in a 25-year follow-up study of a population-based cohort of Danish type 1 diabetic patients and to examine cross-sectional associations between nephropathy and retinopathy in long-term surviving patients of the same...... cohort. All type 1 diabetic patients from Fyn County, Denmark, were identified as of 1 July 1973. One hundred and eighty four patients were examined in 1981-1982 (baseline) and in 2007-2008 (follow-up). The level of retinopathy was graded by ophthalmoscopy at baseline and nine-field digital colour fundus...... and proliferative retinopathy, respectively. In conclusion, proliferative retinopathy is an independent marker of long-term nephropathy in type 1 diabetes. Upcoming studies should examine whether these microvascular complications are also causally linked in type 1 diabetes....

Kidney and Urinary Tract Congenital Malformations. Diagnosis and Evolution. 1999- 2005

International Nuclear Information System (INIS)

Aguilera Bauza, Mirna Pilar; Pena Perez, Raul; Ramirez Prieto, Juan Romelio; Martinez Feria, Rafael; Parra Cruz, Mariela; Pena Hernandez, Miguel Antonio

2008-01-01

A descriptive study in 351 patients at the Nephrology and Urology Services at 'Octavio de la Concepcion de la Pedraja' Teaching Pediatric Hospital from January 1999 to December 2005, was carried out. 535 kidney and urinary tract congenital malformations of 19 types were diagnosed. Primary vesicoureteral reflux and ureteropyelic stenosis were the most frequently malformations. The majority of these patients were diagnosed during the first year of the life. The most frequently clinic manifestation was urinary tract infection. Ultrasound study and cystouretrography were effective to diagnose these malformations. The majority of patients with prenatal diagnosis had congenital hydronephrosis. Posterior urethral valves were the principal cause of chronic renal failure. The I, II and III grades of Primary Vesicoureteral Reflux disappeared spontaneously with conservative treatment. There was a direct relationship between Reflux Nephropathy and the grade of these malformations

Clinicopathological analysis of biopsy-proven diabetic nephropathy based on the Japanese classification of diabetic nephropathy.

Science.gov (United States)

Furuichi, Kengo; Shimizu, Miho; Yuzawa, Yukio; Hara, Akinori; Toyama, Tadashi; Kitamura, Hiroshi; Suzuki, Yoshiki; Sato, Hiroshi; Uesugi, Noriko; Ubara, Yoshifumi; Hohino, Junichi; Hisano, Satoshi; Ueda, Yoshihiko; Nishi, Shinichi; Yokoyama, Hitoshi; Nishino, Tomoya; Kohagura, Kentaro; Ogawa, Daisuke; Mise, Koki; Shibagaki, Yugo; Makino, Hirofumi; Matsuo, Seiichi; Wada, Takashi

2018-06-01

The Japanese classification of diabetic nephropathy reflects the risks of mortality, cardiovascular events and kidney prognosis and is clinically useful. Furthermore, pathological findings of diabetic nephropathy are useful for predicting prognoses. In this study, we evaluated the characteristics of pathological findings in relation to the Japanese classification of diabetic nephropathy and their ability to predict prognosis. The clinical data of 600 biopsy-confirmed diabetic nephropathy patients were collected retrospectively from 13 centers across Japan. Composite kidney events, kidney death, cardiovascular events, all-cause mortality, and decreasing rate of estimated GFR (eGFR) were evaluated based on the Japanese classification of diabetic nephropathy. The median observation period was 70.4 (IQR 20.9-101.0) months. Each stage had specific characteristic pathological findings. Diffuse lesions, interstitial fibrosis and/or tubular atrophy (IFTA), interstitial cell infiltration, arteriolar hyalinosis, and intimal thickening were detected in more than half the cases, even in Stage 1. An analysis of the impacts on outcomes in all data showed that hazard ratios of diffuse lesions, widening of the subendothelial space, exudative lesions, mesangiolysis, IFTA, and interstitial cell infiltration were 2.7, 2.8, 2.7, 2.6, 3.5, and 3.7, respectively. Median declining speed of eGFR in all cases was 5.61 mL/min/1.73 m 2 /year, and the median rate of declining kidney function within 2 years after kidney biopsy was 24.0%. This study indicated that pathological findings could categorize the high-risk group as well as the Japanese classification of diabetic nephropathy. Further study using biopsy specimens is required to clarify the pathogenesis of diabetic kidney disease.

Update on Diabetic Nephropathy: Core Curriculum 2018.

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Umanath, Kausik; Lewis, Julia B

2018-06-01

Diabetic kidney disease and diabetic nephropathy are the leading cause of end-stage kidney disease in the United States and most developed countries. Diabetes accounts for 30% to 50% of the incident cases of end-stage kidney disease in the United States. Although this represents a significant public health concern, it is important to note that only 30% to 40% of patients with diabetes develop diabetic nephropathy. Specific treatment of patients with diabetic nephropathy can be divided into 4 major arenas: cardiovascular risk reduction, glycemic control, blood pressure control, and inhibition of the renin-angiotensin system (RAS). Recommendations for therapy include targeting a hemoglobin A 1c concentration diabetic nephropathy is therapy with a RAS-blocking medication. This Core Curriculum outlines and discusses in detail the epidemiology, pathophysiology, diagnosis, and management of diabetic nephropathy. Copyright © 2017 National Kidney Foundation, Inc. Published by Elsevier Inc. All rights reserved.

Preventing diabetic nephropathy

DEFF Research Database (Denmark)

Hansen, H P; Lund, S S; Rossing, P

2001-01-01

In type 1 diabetic patients with microalbuminuria not receiving antihypertensive treatment, an increase in urinary albumin excretion rate (AER) of 6% to 14%/year and a risk for the development of diabetic nephropathy of 3% to 30%/year have previously been reported. The aim of the present study...... was to audit the effect of angiotensin converting enzyme (ACE) inhibition on the progression of microalbuminuria and development of diabetic nephropathy. We consecutively identified 227 type 1 diabetic patients with persistent microalbuminuria (urinary AER between 30 and 300mg/24h, ELISA). According...... been reported in intervention trials....

Reversal of diabetic nephropathy by a ketogenic diet.

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Michal M Poplawski

Full Text Available Intensive insulin therapy and protein restriction delay the development of nephropathy in a variety of conditions, but few interventions are known to reverse nephropathy. Having recently observed that the ketone 3-beta-hydroxybutyric acid (3-OHB reduces molecular responses to glucose, we hypothesized that a ketogenic diet, which produces prolonged elevation of 3-OHB, may reverse pathological processes caused by diabetes. To address this hypothesis, we assessed if prolonged maintenance on a ketogenic diet would reverse nephropathy produced by diabetes. In mouse models for both Type 1 (Akita and Type 2 (db/db diabetes, diabetic nephropathy (as indicated by albuminuria was allowed to develop, then half the mice were switched to a ketogenic diet. After 8 weeks on the diet, mice were sacrificed to assess gene expression and histology. Diabetic nephropathy, as indicated by albumin/creatinine ratios as well as expression of stress-induced genes, was completely reversed by 2 months maintenance on a ketogenic diet. However, histological evidence of nephropathy was only partly reversed. These studies demonstrate that diabetic nephropathy can be reversed by a relatively simple dietary intervention. Whether reduced glucose metabolism mediates the protective effects of the ketogenic diet remains to be determined.

Reversal of Diabetic Nephropathy by a Ketogenic Diet

Science.gov (United States)

Poplawski, Michal M.; Mastaitis, Jason W.; Isoda, Fumiko; Grosjean, Fabrizio; Zheng, Feng; Mobbs, Charles V.

2011-01-01

Intensive insulin therapy and protein restriction delay the development of nephropathy in a variety of conditions, but few interventions are known to reverse nephropathy. Having recently observed that the ketone 3-beta-hydroxybutyric acid (3-OHB) reduces molecular responses to glucose, we hypothesized that a ketogenic diet, which produces prolonged elevation of 3-OHB, may reverse pathological processes caused by diabetes. To address this hypothesis, we assessed if prolonged maintenance on a ketogenic diet would reverse nephropathy produced by diabetes. In mouse models for both Type 1 (Akita) and Type 2 (db/db) diabetes, diabetic nephropathy (as indicated by albuminuria) was allowed to develop, then half the mice were switched to a ketogenic diet. After 8 weeks on the diet, mice were sacrificed to assess gene expression and histology. Diabetic nephropathy, as indicated by albumin/creatinine ratios as well as expression of stress-induced genes, was completely reversed by 2 months maintenance on a ketogenic diet. However, histological evidence of nephropathy was only partly reversed. These studies demonstrate that diabetic nephropathy can be reversed by a relatively simple dietary intervention. Whether reduced glucose metabolism mediates the protective effects of the ketogenic diet remains to be determined. PMID:21533091

Assessing Urinary Tract Junction Obstruction Defects by Methylene Blue Dye Injection.

Science.gov (United States)

Yun, Kangsun

2017-10-12

Urinary tract junction obstruction defects are congenital anomalies inducing hydronephrosis and hydroureter. Murine urinary tract junction obstruction defects can be assessed by tracking methylene blue dye flow within the urinary system. Methylene blue dye is injected into the renal pelvis of perinatal embryonic kidneys and dye flow is monitored from the renal pelvis of the kidney through the ureter and into the bladder lumen after applying hydrostatic pressure. Dye accumulation will be evident in the bladder lumen of the normal perinatal urinary tract, but will be constrained between the renal pelvis and the end point of an abnormal ureter, if urinary tract obstructions occur. This method facilitates the confirmation of urinary tract junction obstructions and visualization of hydronephrosis and hydroureter. This manuscript describes a protocol for methylene blue dye injection into the renal pelvis to confirm urinary tract junction obstructions.

Membranous nephropathy

Science.gov (United States)

... skin-lightening creams Systemic lupus erythematosus , rheumatoid arthritis, Graves disease, and other autoimmune disorders The disorder occurs at ... diagnosis. The following tests can help determine the cause of membranous nephropathy: Antinuclear antibodies test Anti-double- ...

Sarcopenia in diabetic nephropathy: a cross-sectional study.

Science.gov (United States)

Çeliker, Meral; Selçuk, Mustafa Yavuz; Olt, Serdar

2018-06-01

To investigate the relationship between sarcopenia and diabetic nephropathy. 56 diabetic patients without complications, 50 diabetic patients with nephropathy, 53 healthy controls included in this present study. Demographic characteristics such as sex, age, anthropometric measurements such as weight, body mass index [BMI], hip circumference, waist circumference and upper arm circumference were measured. Sarcopenia diagnosis was based on European Working Group on Sarcopenia in Older People [EWGSOP] criteria which consist of hand grip strength, 6-meter walking test and muscle mass. The frequency of sarcopenia increased gradually from 15.1% in healthy control group to 21.4% in the diabetes group, and 34% in diabetic nephropathy group (X2 for trend, p = 0.029). The frequency of sarcopenia was similar in diabetes and diabetic nephropathy group. However, the frequency of sarcopenia was higher in diabetic nephropathy than healthy controls (OR = 2.89, CI [1.11-7.51] in logistic regression). In the present study, the prevalence of sarcopenia was higher in patients with diabetic nephropathy compared to healthy controls.

Spectrum of Congenital Heart Diseases in Eastern Nepal: A tertiary care hospital experience

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Prashant Shah

2017-01-01

Full Text Available Background & Objectives: Congenital heart diseases are neglected especially in world’s poorest nations and appear to be ignored and unexplored dimension of health. The exact prevalence and spectrum of congenital heart diseases in Nepal is largely unknown. The aim of this study was to describe the local experience on the magnitude and the pattern of congenital heart disease in order to increase the awareness of the public and health policy makers on its burden in Nepal.Materials & Methods: This is an observational hospital based study carried out in a tertiary care hospital in Eastern Nepal. The duration of this study was from April 2015 to July 2016. The echocardiography reports of all patients clinically suspected of having congenital heart disease were retrieved, and their diagnostic details were extracted. Only patients of day one of life to 14 years of age were included. Congenital heart diseases like bicuspid aortic valve, mitral valve prolapse and various inherited cardiomyopathies were excluded.Results: A total of 330 echocardiograms were performed for clinically suspected congenital heart disease.  The mean age of study population was 22.31±34.08 months with male to female ratio of 1.2:1. 23% of clinically suspected congenital heart disease cases turned out to have normal echocardiography. Acyanotic congenital heart disease was most common (81.5% followed by cyanotic congenital heart disease (14.2% and obstructive congenital heart disease (4.3%. Atrial septal defect was found to be the most common form of acyanotic congenital heart disease (52% which was followed by ventricular septal defect (28.8% and patent ductus arteriosus (14.8%. Tetralogy of Fallot and double outlet right ventricle were the most common form of cyanotic CHD representing 44.4% of all cyanotic patients. Pulmonary stenosis was the most common obstructive congenital heart disease observed in this study population (63.6%. Rarer entities, like d-transposition of great

Radiological findings of congenital urethral valves

International Nuclear Information System (INIS)

Yeon, Kyung Mo; Kook, Shin Ho

1990-01-01

Congenital urethral valve is the common cause of hydronephrosis in newborn infants and the most common cause of bladder outlet obstruction in male children. We reviewed and analysed radiological findings and associated anomalies of 16 cases of congenital urethral valve which were examined during the period from January 1985 to December 1989. The most frequent age was under one year old (56%). The main symptoms were urinary dribbing (37.5%), weak stream (25%) and urinary frequency and incontinence (25%). Anterior urethral valve (AUV) was 5 cases (31%) and posterior urethral valve (PUV) was 11 cases(69%), in which 10 cases were Type I and one case was Type III. Bladder wall thickening was seen in all cases and its severity was partly correlated with the degree of vesicoureteral reflux (VUR). VUR was observed in 12 cases (75%), and relatively severe in older age group. The degree of VUR was milder in AUV than PUV. Hydronephrosis was more severe in PUV than in anterior one, and its degree was correlated with the severity of VUR. Associated anomalies were ectopic urethral opening (2 cases), PDA (1 case), congenital megacolon (1 case) and patent urachus (1 case) in PUV. So early diagnosis and treatment of congenital urethral valve is essential to the prevention of renal damage

Smoking in diabetic nephropathy: sparks in the fuel tank?

Science.gov (United States)

Chakkarwar, Vishal Arvind

2012-12-15

Diabetic nephropathy is associated with high morbidity and mortality and the prevalence of this disease is continuously increasing worldwide. Long-term diabetes increases the likelihood of developing secondary complications like nephropathy, the most common cause of end stage renal disease. Usually, other factors like hypertension, alcoholism and smoking also partly contribute to the progression of diabetic nephropathy. Among this, cigarette smoking in diabetes has been repeatedly confirmed as an independent risk factor for the onset and progression of diabetic nephropathy. Various studies suggest that smoking is a major fuel in the development of high oxidative stress and subsequently hyperlipidemia, accumulation of advanced glycation end products, activation of the renin angiotensin system and Rho-kinase, which are observed to play a pathogenic role in the progression of diabetic nephropathy. Furthermore, cigarette smoking in diabetic patients with vascular complications produces a variety of pathological changes in the kidney, such as thickening of the glomerular basement membrane and mesangial expansion with progression in glomerulosclerosis and interstitial fibrosis, which ultimately results in end stage renal failure. Strong associations are consistently found between chronic cigarette smoking and diabetic microvascular complications. A diverse group of studies unveil potential mechanisms that may explain the role of cigarette smoking in the progression of diabetic nephropathy. Tremendous efforts are being made to control smoking mediated progression of diabetic nephropathy, but no promising therapy is yet available. The present review critically discusses the possible detrimental role of chronic cigarette smoking in the progression of diabetic nephropathy and various possible pharmacological interventions to attenuate the exacerbation of diabetic nephropathy.

Managing neonatal bowel obstruction: clinical perspectives

Directory of Open Access Journals (Sweden)

Desoky SM

2018-02-01

Full Text Available Sarah M Desoky,1 Ranjit I Kylat,2 Unni Udayasankar,1 Dorothy Gilbertson-Dahdal1 1Department of Medical Imaging, University of Arizona College of Medicine, Tucson, AZ, USA; 2Division of Neonatal-Perinatal Medicine, Department of Pediatrics, University of Arizona College of Medicine, Tucson, AZ, USA Abstract: Neonatal intestinal obstruction is a common surgical emergency and occurs in approximately 1 in 2,000 live births. The causes of obstruction are diverse with varied embryological origins, and some underlying etiologies are not yet well described. Some findings of neonatal bowel obstruction can be detected prenatally on ultrasound imaging. The obstruction is classified as “high” when the level of obstruction is proximal to the ileum, and “low” when the level of obstruction is at the ileum or colon. Early diagnosis of the type of intestinal obstruction and localization of the obstructive bowel segment guides timely and appropriate management of the underlying pathologic entity. Neonatal bowel obstructions are ideally managed at specialized centers with a large volume of neonatal surgery and dedicated pediatric surgical and anesthesia expertise. Although surgical intervention is necessary in most cases, initial management strategies often target underlying metabolic, cardiac, or respiratory abnormalities. Imaging plays a key role in early and accurate diagnosis of the abnormalities. When bowel obstruction is suspected clinically, initial imaging workup usually involves abdominal radiography, which may direct further evaluation with fluoroscopic examination such as upper gastrointestinal (UGI contrast study or contrast enema. This article provides a comprehensive review of clinical and radiological features of common and less common causes of intestinal obstruction in the neonatal age group, including esophageal atresia, enteric duplication cysts, gastric volvulus, congenital microgastria, hypertrophic pyloric stenosis, duodenal atresia

Increased arterial stiffness in children with congenital heart disease.

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Häcker, Anna-Luisa; Reiner, Barbara; Oberhoffer, Renate; Hager, Alfred; Ewert, Peter; Müller, Jan

2018-01-01

Objective Central systolic blood pressure (SBP) is a measure of arterial stiffness and strongly associated with atherosclerosis and end-organ damage. It is a stronger predictor of cardiovascular events and all-cause mortality than peripheral SBP. In particular, for children with congenital heart disease, a higher central SBP might impose a greater threat of cardiac damage. The aim of the study was to analyse and compare central SBP in children with congenital heart disease and in healthy counterparts. Patients and methods Central SBP was measured using an oscillometric method in 417 children (38.9% girls, 13.0 ± 3.2 years) with various congenital heart diseases between July 2014 and February 2017. The test results were compared with a recent healthy reference cohort of 1466 children (49.5% girls, 12.9 ± 2.5 years). Results After correction for several covariates in a general linear model, central SBP of children with congenital heart disease was significantly increased (congenital heart disease: 102.1 ± 10.2 vs. healthy reference cohort: 100.4 ± 8.6, p congenital heart disease subgroups revealed higher central SBP in children with left heart obstructions (mean difference: 3.6 mmHg, p congenital heart disease have significantly higher central SBP compared with healthy peers, predisposing them to premature heart failure. Screening and long-term observations of central SBP in children with congenital heart disease seems warranted in order to evaluate the need for treatment.

Early release of neonatal ureteral obstruction preserves renal function

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Shi, Yimin; Pedersen, Michael; Li, Chunling

2004-01-01

was left in place or released after 1 or 4 wk. Renal blood flow (RBF) and kidney size were measured sequentially over 24 wk using MRI. In rats in which the obstruction was left in place, RBF of the obstructed kidney was progressively reduced to 0.92 ± 0.17 vs. 1.79 ± 0.12 ml·min−1·100 g body wt−1 (P ...The incidence of congenital hydronephrosis is ∼1% and is often associated with renal insufficiency. It is unknown whether early release is essential to prevent deterioration of renal function. Rats were subjected to partial unilateral ureteral obstruction (PUUO) on postnatal day 2. The obstruction...... downregulation of Na-K-ATPase to 62 ± 7%, aquaporin-1 to 53 ± 3%, and aquaporin-3 to 53 ± 7% of sham levels. Release after 1 wk completely prevented development of hydronephrosis, reduction in RBF and glomerular filtration rate, and downregulation of renal transport proteins, whereas release after 4 wk had...

Early pre-eclampsia unmasks underlying IgA nephropathy

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Mona Singh

2010-12-01

Full Text Available Mona Singh, Akhenaton Pappoe, Burl R DonDivision of Nephrology, University of California Davis Medical Center, Sacramento, CA, USAAbstract: Pre-eclampsia is the most ominous complication of pregnancy, and primary glomerular diseases can mimic pre-eclampsia in presentation. A patient presented at 21 weeks gestation with signs and symptoms of both pre-eclampsia and primary glomerular nephropathy. A critical clinical decision whether to continue or terminate the pregnancy was dependent on results of a renal biopsy. The biopsy noted the presence of both pre-eclampsia and immunoglobulin A (IgA nephropathy. Thus, the onset of pre-eclampsia unmasked the presence of unrecognized IgA nephropathy, and the IgA nephropathy was a risk factor for this patient developing pre-eclampsia. The results of a renal biopsy are key in distinguishing pre-eclampsia from other kidney diseases and instituting appropriate clinical management.Keywords: proteinuria, IgA nephropathy, renal biopsy, pre-eclampsia

Incipient and overt diabetic nephropathy in African Americans with NIDDM.

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Dasmahapatra, A; Bale, A; Raghuwanshi, M P; Reddi, A; Byrne, W; Suarez, S; Nash, F; Varagiannis, E; Skurnick, J H

1994-04-01

OBJECTIVE--To determine the prevalence of incipient and overt nephropathy in African-American subjects with non-insulin-dependent diabetes mellitus (NIDDM) attending a hospital clinic. Contributory factors, such as blood pressure (BP), duration and age at onset of diabetes, hyperglycemia, hyperlipidemia, and body mass index (BMI) also were evaluated. RESEARCH DESIGN AND METHODS--We recruited 116 African-American subjects with NIDDM for this cross-sectional, descriptive, and analytical study. BP, BMI, 24-h urine albumin excretion, creatinine clearance, serum creatinine, lipids, and GHb levels were measured. Albumin excretion rate (AER) was calculated, and subjects were divided into three groups: no nephropathy (AER 200 micrograms/min). Frequency of hypertension and nephropathy was analyzed by chi 2 testing, group means were compared using analysis of variance, and linear correlations were performed between AER and other variables. Multiple regression analysis was used to examine the association of these variables while controlling for the effects of other variables. RESULTS--Increased AER was present in 50% of our subjects; 31% had incipient and 19% had overt nephropathy. Hypertension was present in 72.4%; nephropathy, particularly overt nephropathy, was significantly more prevalent in the hypertensive group. Mean BP and diastolic blood pressure (dBP) were higher in the groups with incipient and overt nephropathy, and systolic blood pressure (sBP) was increased in overt nephropathy. Men with either form of nephropathy had higher sBP, dBP, and mean BP, whereas only women with overt nephropathy had increased sBP and mean BP. Subjects with incipient or overt nephropathy had a longer duration of diabetes, and those with overt nephropathy had a younger age at onset of diabetes. By multiple regression analysis, AER correlated with younger age at diabetes onset, but not with diabetes duration. No correlation with age, lipid levels, or GHb was noted. BMI correlated with AER

Annular pancreas causing extrahepatic biliary obstruction

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Ogulin, M.; Jamar, B.

2004-01-01

Background. Annular pancreas is an uncommon congenital abnormality, consisting of a flat band of pancreatic tissue, which encircles duodenum or extrahepatic biliary duct. We present a case of obstructive jaundice, caused by annular pancreas. Case report. A 46 years old female was admitted because of a sudden onset of abdominal pain, vomiting and jaundice. For the last six years she occasionally noticed her skin was light yellow, in the last year she felt distension in the upper abdomen, especially after fatty meals. Conclusions. Two US examinations, the first one six months before the admission, showed dilated hepatic ducts. The reason of dilatation was unclear, even after the endoscopic US examination. At operation an almost complete obstruction of the common hepatic duct was found, caused by a narrow band of pancreatic tissue. (author)

Clinical application of urodilatin in Type 2 diabetic nephropathy

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Zhu Yihua; Cao Xingjian; Chen Yuxiang; Zhang Kexia; Jin Yan

2011-01-01

Objective: To investigate the clinical application of urodilatin (URO) in tubular injury of DM2. Methods: 41 healthy controls, 33 type 2 diabetics without nephropathy, 37 patients with early stage of diabetic nephropathy and 26 patients with clinical diabetic nephropathy were enrolled in the study and categorized into four groups. Urodilatin was measured by radioimmunoassay (RIA). The changes of urodilatin levels among four groups were analyzed, and correlation analyses were performed between urodilatin and urinary micro-albumin/urine creatinine(mA/UCr). The efficiency index of URO were evaluated by receiver operation characteristic (ROC). Results: Compared with those in the controls,diabetics without nephropathy, early stage of diabetic nephropathy and clinical diabetic nephropathy, the urodilatin level decreased significantly in the course of diabetic nephropathy (P<0.001). The value of URO was significantly correlated with mA/UCr (r=-0.626, P<0.01). In early phase of DM2, The area under curve was 0.759. When the cut-off vaule of URO was ≤51.5 pg/ml, The sensitivity and specificity were 67.14% and 70.29%, respectively. Furthermore, Urodilatin had similar diagnosis efficiency with mA/UCr. Conclusion: The decrease of urodilatin level had clinical value in pristine tubular injury of DM2 and can serve as an evaluation parameter. (authors)

Acute postirradiation nephropathy

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Trojanowski, Z.

1982-01-01

The pathogenesis, morphological and clinical signs of acute postirradiation nephropathy are described with particular attention paid to the relationship between the clinical signs of renal involvement and the dose of radiation. (author)

Defining and predicting 'intrauterine fetal renal failure' in congenital lower urinary tract obstruction.

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Ruano, Rodrigo; Safdar, Adnan; Au, Jason; Koh, Chester J; Gargollo, Patricio; Shamshirsaz, Alireza A; Espinoza, Jimmy; Cass, Darrell L; Olutoye, Oluyinka O; Olutoye, Olutoyin A; Welty, Stephen; Roth, David R; Belfort, Michael A; Braun, Michael C

2016-04-01

The aim of this study was to identify predictors of 'intrauterine fetal renal failure' in fetuses with severe congenital lower urinary tract obstruction (LUTO). We undertook a retrospective study of 31 consecutive fetuses with a diagnosis of LUTO in a tertiary Fetal Center between April 2013 and April 2015. Predictors of 'intrauterine fetal renal failure' were evaluated in those infants with severe LUTO who had either a primary composite outcome measure of neonatal death in the first 24 h of life due to severe pulmonary hypoplasia or a need for renal replacement therapy within 7 days of life. The following variables were analyzed: fetal bladder re-expansion 48 h after vesicocentesis, fetal renal ultrasound characteristics, fetal urinary indices, and amniotic fluid volume. Of the 31 fetuses included in the study, eight met the criteria for 'intrauterine fetal renal failure'. All of the latter had composite poor postnatal outcomes based on death within 24 h of life (n = 6) or need for dialysis within 1 week of life (n = 2). The percentage of fetal bladder refilling after vesicocentesis at time of initial evaluation was the only predictor of 'intrauterine fetal renal failure' (cut-off <27 %, area under the time-concentration curve 0.86, 95 % confidence interval 0.68-0.99; p = 0.009). We propose the concept of 'intrauterine fetal renal failure' in fetuses with the most severe forms of LUTO. Fetal bladder refilling can be used to reliably predict 'intrauterine fetal renal failure', which is associated with severe pulmonary hypoplasia or the need for dialysis within a few days of life.

Autophagy: A Novel Therapeutic Target for Diabetic Nephropathy.

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Kume, Shinji; Koya, Daisuke

2015-12-01

Diabetic nephropathy is a leading cause of end stage renal disease and its occurance is increasing worldwide. The most effective treatment strategy for the condition is intensive treatment to strictly control glycemia and blood pressure using renin-angiotensin system inhibitors. However, a fraction of patients still go on to reach end stage renal disease even under such intensive care. New therapeutic targets for diabetic nephropathy are, therefore, urgently needed. Autophagy is a major catabolic pathway by which mammalian cells degrade macromolecules and organelles to maintain intracellular homeostasis. The accumulation of damaged proteins and organelles is associated with the pathogenesis of diabetic nephropathy. Autophagy in the kidney is activated under some stress conditions, such as oxidative stress and hypoxia in proximal tubular cells, and occurs even under normal conditions in podocytes. These and other accumulating findings have led to a hypothesis that autophagy is involved in the pathogenesis of diabetic nephropathy. Here, we review recent findings underpinning this hypothesis and discuss the advantages of targeting autophagy for the treatment of diabetic nephropathy.

Autophagy: A Novel Therapeutic Target for Diabetic Nephropathy

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Shinji Kume

2015-12-01

Full Text Available Diabetic nephropathy is a leading cause of end stage renal disease and its occurance is increasing worldwide. The most effective treatment strategy for the condition is intensive treatment to strictly control glycemia and blood pressure using renin-angiotensin system inhibitors. However, a fraction of patients still go on to reach end stage renal disease even under such intensive care. New therapeutic targets for diabetic nephropathy are, therefore, urgently needed. Autophagy is a major catabolic pathway by which mammalian cells degrade macromolecules and organelles to maintain intracellular homeostasis. The accumulation of damaged proteins and organelles is associated with the pathogenesis of diabetic nephropathy. Autophagy in the kidney is activated under some stress conditions, such as oxidative stress and hypoxia in proximal tubular cells, and occurs even under normal conditions in podocytes. These and other accumulating findings have led to a hypothesis that autophagy is involved in the pathogenesis of diabetic nephropathy. Here, we review recent findings underpinning this hypothesis and discuss the advantages of targeting autophagy for the treatment of diabetic nephropathy.

The Family Investigation of Nephropathy and Diabetes (FIND): design and methods.

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Knowler, William C; Coresh, Josef; Elston, Robert C; Freedman, Barry I; Iyengar, Sudha K; Kimmel, Paul L; Olson, Jane M; Plaetke, Rosemarie; Sedor, John R; Seldin, Michael F

2005-01-01

The Family Investigation of Nephropathy and Diabetes (FIND) is a multicenter study designed to identify genetic determinants of diabetic nephropathy. It is conducted in eight U.S. clinical centers and a coordinating center, and with four ethnic groups (European Americans, African Americans, Mexican Americans, and American Indians). Two strategies are used to localize susceptibility genes: a family-based linkage study and a case-control study using mapping by admixture linkage disequilibrium (MALD). In the family-based study, probands with diabetic nephropathy are recruited with their parents and selected siblings. Linkage analyses will be conducted to identify chromosomal regions containing genes that influence the development of diabetic nephropathy or related quantitative traits such as serum creatinine concentration, urinary albumin excretion, and plasma glucose concentrations. Regions showing evidence of linkage will be examined further with both genetic linkage and association studies to identify genes that influence diabetic nephropathy or related traits. Two types of MALD studies are being done. One is a case-control study of unrelated individuals of Mexican American heritage in which both cases and controls have diabetes, but only the case has nephropathy. The other is a case-control study of African American patients with nephropathy (cases) and their spouses (controls) unaffected by diabetes and nephropathy; offspring are genotyped when available to provide haplotype data. Identification of genes that influence susceptibility to diabetic nephropathy will lead to a better understanding of how nephropathy develops. This should eventually lead to improved treatment and prevention.

Congenital bronchopulmonary foregut malformations: concepts and controversies

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Newman, Beverley

2006-01-01

This article addresses the scope, etiology, important associations and imaging features of congenital bronchopulmonary foregut malformations. Etiologic concepts, including airway obstruction and vascular anomalies, are highlighted. Technical imaging advances, especially CT and MR, have greatly enhanced our diagnostic abilities in evaluating these lesions; however, thorough and careful assessment of all aspects of the malformation is still necessary. Several specific lesions are discussed in more detail, particularly regarding controversial issues in classification, understanding, imaging and management. (orig.)

Congenital bronchopulmonary foregut malformations: concepts and controversies

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Newman, Beverley [University of Pittsburgh School of Medicine and Children' s Hospital of Pittsburgh, Pittsburgh, PA (United States)

2006-08-15

This article addresses the scope, etiology, important associations and imaging features of congenital bronchopulmonary foregut malformations. Etiologic concepts, including airway obstruction and vascular anomalies, are highlighted. Technical imaging advances, especially CT and MR, have greatly enhanced our diagnostic abilities in evaluating these lesions; however, thorough and careful assessment of all aspects of the malformation is still necessary. Several specific lesions are discussed in more detail, particularly regarding controversial issues in classification, understanding, imaging and management. (orig.)

The Role of Autophagy in the Pathogenesis of Diabetic Nephropathy

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Kosuke Yamahara

2013-01-01

Full Text Available Diabetic nephropathy is a leading cause of end-stage renal disease worldwide. The multipronged drug approach targeting blood pressure and serum levels of glucose, insulin, and lipids fails to fully prevent the onset and progression of diabetic nephropathy. Therefore, a new therapeutic target to combat diabetic nephropathy is required. Autophagy is a catabolic process that degrades damaged proteins and organelles in mammalian cells and plays a critical role in maintaining cellular homeostasis. The accumulation of proteins and organelles damaged by hyperglycemia and other diabetes-related metabolic changes is highly associated with the development of diabetic nephropathy. Recent studies have suggested that autophagy activity is altered in both podocytes and proximal tubular cells under diabetic conditions. Autophagy activity is regulated by both nutrient state and intracellular stresses. Under diabetic conditions, an altered nutritional state due to nutrient excess may interfere with the autophagic response stimulated by intracellular stresses, leading to exacerbation of organelle dysfunction and diabetic nephropathy. In this review, we discuss new findings showing the relationships between autophagy and diabetic nephropathy and suggest the therapeutic potential of autophagy in diabetic nephropathy.

A comparison between monocanalicular and pushed monocanalicular silicone intubation in the treatment of congenital nasolacrimal duct obstruction

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Dima Andalib

2014-12-01

Full Text Available AIM: To compare the success rate of monocanalicular versus pushed monocanalicular silicone intubation (PMCI of the nasolacrimal duct for congenital nasolacrimal duct obstruction (CNLDO.METHODS: In a prospective randomized clinical trial 53 eyes of 49 patients with CNLDO underwent either monocanalicular silicone intubation (MCI (n=28 eyes or PMCI (n=25 eyes. All procedures were performed by 1 oculoplastic surgeon. Treatment success was defined as the complete resolution of epiphora at 3mo after tube removal.Results: The surgical outcome was assessed in 20 eyes with MCI and 20 eyes with PMCI. The mean age of treatment was 26.25±10.08mo (range, 13-49mo for MCI and 26.85±12.25mo (range, 16-68mo for PMCI. Treatment success was achieved in 18 of 20 eyes (90.0% in the MCI group compared with 10 of 20 eyes (50% in the PMCI group (P=0.01. In the PMCI group, the tube loss (30% was greater than the MCI group (5%, however the differences between the 2 groups proved to be not significant (P=0.91.CONCLUSION: Our results indicate that MCI has higher success rate in CNLDO treatment compared with PMCI in this small series of patients.

Lipid Abnormalities in Type 2 Diabetes Mellitus Patients with Overt Nephropathy

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Viswanathan, Vijay

2017-01-01

Background Diabetic nephropathy is a major complication of diabetes and an established risk factor for cardiovascular events. Lipid abnormalities occur in patients with diabetic nephropathy, which further increase their risk for cardiovascular events. We compared the degree of dyslipidemia among type 2 diabetes mellitus (T2DM) subjects with and without nephropathy and analyzed the factors associated with nephropathy among them. Methods In this retrospective study, T2DM patients with overt nephropathy were enrolled in the study group (n=89) and without nephropathy were enrolled in the control group (n=92). Both groups were matched for age and duration of diabetes. Data on total cholesterol (TC), triglycerides (TG), high density lipoprotein cholesterol (HDL-C), low density lipoprotein cholesterol (LDL-C), urea and creatinine were collected from the case sheets. TG/HDL-C ratio, a surrogate marker for small, dense, LDL particles (sdLDL) and estimated glomerular filtration rate (eGFR) were calculated using equations. Multivariate analysis was done to determine the factors associated with eGFR. Results Dyslipidemia was present among 56.52% of control subjects and 75.28% of nephropathy subjects (P=0.012). The percentage of subjects with atherogenic dyslipidemia (high TG+low HDL-C+sdLDL) was 14.13 among controls and 14.61 among nephropathy subjects. Though serum creatinine was not significantly different, mean eGFR value was significantly lower among nephropathy patients (P=0.002). Upon multivariate analysis, it was found that TC (P=0.007) and HDL-C (P=0.06) were associated with eGFR among our study subjects. Conclusion Our results show that dyslipidemia was highly prevalent among subjects with nephropathy. Regular screening for dyslipidemia may be beneficial in controlling the risk for adverse events among diabetic nephropathy patients. PMID:28447439

Spontaneous Perirenal Urinoma Associated with Ureteropelvic Junction Obstruction in a Child: A Case Report

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Po-Chang Tseng

2009-06-01

Full Text Available We report a case of a 10-year-old Taiwanese boy with a perinephric urinoma, whose health had previously been good, but who experienced a sudden onset of severe left flank pain. Radiological examination revealed ureteropelvic junction obstruction with grade IV hydronephrosis and perinephric urinoma of the left kidney. Percutaneous drainage was performed successfully to relieve these symptoms. Urinoma is a rare complication of congenital obstruction of the urinary tract, and it occurs most commonly following renal trauma.

Diabetic retinopathy in two patients with congenital IGF-I deficiency (Laron syndrome).

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Laron, Zvi; Weinberger, Dov

2004-07-01

Animal and clinical studies have shown that excessive amounts of growth hormone or insulin-like growth factor-I (IGF-I) promote the development of diabetes and diabetic retinopathy. Forthwith, we present two patients with congenital IGF-I deficiency who developed type II diabetes and subsequently retinopathy. Eighteen adult patients with classical Laron syndrome (8 males, 10 females, aged 20-62 years) were followed by us since childhood or underwent fundus photography with a Nikon NF 505 instrument. Three had been treated in childhood with IGF-I, the rest were never treated, including the two patients reported. Two never-treated patients were diagnosed with type II diabetes (DM) at ages 39 and 41 respectively. There was no diabetes in the families. Oral treatment was followed by insulin injections. Metabolic control was not optimal and one patient developed proliferative diabetic retinopathy, necessitating laser surgery. He also has nephropathy and severe neuropathy. The other patient has background diabetic retinopathy and has developed, progressively, exudates, microaneurisms, hemorrhages and clinically significant macular edema. He also has subacute ischemic heart disease. Our findings show that congenital IGF-I deficiency, similar to excess, causes vascular complications of DM, denoting also that vascular endothelial growth factor can induce neovascularization in the presence of congenital IGF-I deficiency.

Relationship between serum IV-C, β2-m levels and diabetic nephropathy

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Liu Lu; Zhang Mukun

2005-01-01

Objective: To investigate the relationship between the serum type IV collagen (IV-C), β 2 -micro globulin (β 2 -m) levels and diabetic nephropathy. Methods: Serum IV-C, β 2 -m levels were measured with RIA in 30 controls and 86 patients with type 2 diabetics mellitus (35 with diabetic nephropathy and 51 without nephropathy). Results: the serum levels of IV-C and β 2 -m in diabetic patients with nephropathy were significantly higher than those in controls (P 0.05). Conclusion: Serum IV-C and β 2 -m levels increased gradually as the diabetic nephropathy got more severe. They could be a sensitive marker for early diagnosis of development of diabetic nephropathy. (authors)

Congenital cytomegalovirus related intestinal malrotation: a case report.

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Colomba, Claudia; Giuffrè, Mario; La Placa, Simona; Cascio, Antonio; Trizzino, Marcello; De Grazia, Simona; Corsello, Giovanni

2016-12-07

Cytomegalovirus is the most common cause of congenital infection in the developed countries. Gastrointestinal involvement has been extensively described in both adult and paediatric immunocompromised patients but it is infrequent in congenital or perinatal CMV infection. We report on a case of coexistent congenital Cytomegalovirus infection with intestinal malrotation and positive intestinal Cytomegalovirus biopsy. At birth the neonate showed clinical and radiological evidence of intestinal obstruction. Meconium passed only after evacuative nursing procedures; stooling pattern was irregular; gastric residuals were bile-stained. Laparatomy revealed a complete intestinal malrotation and contextually gastrointestinal biopsy samples of the appendix confirmed the diagnosis of CMV gastrointestinal disease. Intravenous ganciclovir was initiated for 2 weeks, followed by oral valgancyclovir for 6 month. CMV-induced proinflammatory process may be responsible of the interruption of the normal development of the gut or could in turn lead to a disruption in the normal development of the gut potentiating the mechanism causing malrotation. We suggest the hypothesis that an inflammatory process induced by CMV congenital infection may be responsible, in the early gestation, of the intestinal end-organ disease, as the intestinal malrotation. CMV infection should always be excluded in full-term infants presenting with colonic stricture or malrotation.

Grave's disease associated with immunoglobulin A nephropathy: A rare association.

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Khan, I; Bhat, R A; Khan, I; Hameed, I

2015-01-01

Immunoglobulin A (Ig A) nephropathy is the most common form of primary glomerulonephritis. The association of Ig A nephropathy with Grave's disease has not been reported so far. We report a case of 20-year-old female with Grave's disease who presented with edema, facial puffiness, and decreased urine output. She was found to be hypertensive with renal failure and nephrotic range proteinuria. Renal biopsy revealed features of Ig A nephropathy. The patient was treated with oral corticosteroids (1 mg/kg/day). To our knowledge, this is the first case showing association of Grave's disease with Ig A nephropathy.

Axial torsion of meckel's diverticulum causing small bowel obstruction in adult: A case report

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Youn, In Kyung; Lee, Su Lim; Ku, Young Mi [Dept. of Radiology, Uijeongbu St. Mary' s Hospital, College of Medicine, The Catholic University of Korea, Uijeongbu (Korea, Republic of)

2016-02-15

Meckel's diverticulum (MD) is the most common congenital abnormality of the gastrointestinal tract that is prevalent in 2–3% of the population. The lifetime risk of complications is estimated at 4%. Small bowel obstruction is the second most common complication of MD. Among the causes of bowel obstruction, axial torsion of MD is the rarest complication. Urgent surgical treatment is needed in cases of small bowel obstruction associated with torsion of MD. Pre-operative diagnosis of MD as a cause of small bowel obstruction is difficult, because the diagnosis can be made only if the diverticulum is delineated at the site of obstruction. We reported a case of axial torsion of MD with necrosis that caused proximal small bowel perforation in a 21 year old male.

Contrast induced nephropathy

DEFF Research Database (Denmark)

Stacul, Fulvio; van der Molen, Aart J; Reimer, Peter

2011-01-01

PURPOSE: The Contrast Media Safety Committee (CMSC) of the European Society of Urogenital Radiology (ESUR) has updated its 1999 guidelines on contrast medium-induced nephropathy (CIN). AREAS COVERED: Topics reviewed include the definition of CIN, the choice of contrast medium, the prophylactic me...

Ureteropelvic junction obstruction and ureteral strictures treated by simple high-pressure balloon dilation

DEFF Research Database (Denmark)

Osther, P J; Geertsen, U; Nielsen, H V

1998-01-01

The long-term results of simple high-pressure balloon dilation in the treatment of ureteropelvic junction obstruction (UPJO) and ureteral strictures were evaluated. A total of 77 consecutive patients were treated: 40 had UPJO and 37 ureteral strictures. The etiology of the obstruction included...... years, success was achieved in only 25% of cases. There were no major complications. It was concluded that simple high-pressure balloon dilation is a safe and reasonably effective technique for the management of most ureteral strictures and congenital UPJO with symptom debut in adult life. Balloon...

Contrast-induced nephropathy after computed tomography

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Luciano da Silva Selistre

2015-03-01

Full Text Available Introduction: Contrast induced nephropathy is the third most prevalent preventable cause of acute kidney injury in hospitalized patients. It defined as an absolute increase in serum creatinine ≥ 0.5 mg/dL and relative ≥ 25% increase. Objective: We studied the risk factors to intravenous injection contrast nephropathy after computed tomography. Methods: We studied 400 patients prospectively. Results: The incidence of contrast induced nephropathy, with an absolute or a relative increase were 4.0% and 13.9%, respectively. Diabetes and cardiac failure were independent risk factors for CIN a relative increase de serum creatinine (O.R.: 3.5 [95% CI: 1.92-6.36], p < 0.01, 2.61 [95% CI: 1.14-6.03%], p < 0.05, respectively. Conclusions: We showed association between uses of intravenous injection contrast after computed tomography with acute injury renal, notably with diabetes and heart failure.

Long term ocular and neurological involvement in severe congenital toxoplasmosis.

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Meenken, C; Assies, J; van Nieuwenhuizen, O; Holwerda-van der Maat, W G; van Schooneveld, M J; Delleman, W J; Kinds, G; Rothova, A

1995-06-01

This study was set up to determine the long term ocular and systemic sequelae in patients with severe congenital toxoplasmosis. Cross sectional and retrospective study of 17 patients with severe congenital toxoplasmosis. In addition to chorioretinitis (100%), the most common abnormal ocular features were optic nerve atrophy (83%), visual acuity of less than 0.1 (85%), strabismus, and microphthalmos. In 50% of cases we observed iridic abnormalities and about 40% developed a cataract. Overt endocrinological disease, diagnosed in five of 15 patients, included panhypopituitarism (n = 2), gonadal failure with dwarfism (n = 1), precocious puberty with dwarfism and thyroid deficiency (n = 1), and diabetes mellitus and thyroid deficiency (n = 1). The observed endocrinological involvement was associated in all cases with obstructive hydrocephalus with a dilated third ventricle and optic nerve atrophy. The recognition of long term ocular, neurological, and endocrinological sequelae of congenital toxoplasmosis is important for medical management of these severely handicapped patients.

A complex microdeletion 17q12 phenotype in a patient with recurrent de novo membranous nephropathy.

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Hinkes, Bernward; Hilgers, Karl F; Bolz, Hanno J; Goppelt-Struebe, Margarete; Amann, Kerstin; Nagl, Sandra; Bergmann, Carsten; Rascher, Wolfgang; Eckardt, Kai-Uwe; Jacobi, Johannes

2012-05-14

Microdeletions on chromosome 17q12 cause of diverse spectrum of disorders and have only recently been identified as a rare cause of Mayer-Rokitansky-Kuester-Hauser-Syndrome (MRKH), which is characterized by uterus aplasia ± partial/complete vaginal aplasia in females with a regular karyotype. For the first time we report about a patient with a 17q12 microdeletion who is affected by MRKH in combination with a vascular and soft tissue disorder. Repeatedly she suffered from kidney transplant failure caused by consuming membranous nephropathy. A 38-year-old female patient had been diagnosed with right kidney aplasia, left kidney dysplasia and significantly impaired renal function during infancy. Aged 16 she had to start hemodialysis. Three years later she received her first kidney transplant. Only then she was diagnosed with MRKH. The kidney transplant was lost due to consuming nephrotic syndrome caused by de novo membranous nephropathy, as was a second kidney transplant years later. In addition, a hyperelasticity syndrome affects the patient with congenital joint laxity, kyphoscoliosis, bilateral hip dysplasia, persistent hypermobility of both elbows, knees and hips. Her clinical picture resembles a combination of traits of a hypermobile and a vascular form of Ehlers-Danlos-Syndrome, but no mutations in the COL3A1 gene was underlying. Instead, array-based comparative genomic hybridisation (CGH) detected a heterozygous 1.43 Mb deletion on chromosome 17q12 encompassing the two renal developmental genes HNF1β and LHX1. Deletions of HNF1β have recently drawn significant attention in pediatric nephrology as an important cause of prenatally hyperechogenic kidneys, renal aplasia and renal hypodysplasia. In contrast, membranous nephropathy represents an often-unaccounted cause of nephrotic syndrome in the adult population. A causative connection between theses two conditions has never been postulated, but is suggestive enough in this case to hypothesize it.

Decrease in toe pinch force in male type 2 diabetic patients with diabetic nephropathy.

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Kataoka, Hiroaki; Miyatake, Nobuyuki; Kitayama, Naomi; Murao, Satoshi; Tanaka, Satoshi

2018-06-01

The purpose of this cross-sectional study was to investigate the toe pinch force (TPF) of type 2 diabetic patients with diabetic nephropathy by disease stage, and to clarify the factors affecting the TPF. Seventy-four men with diabetic nephropathy (age: 62.7 ± 8.9 years, duration of diabetes: 14.2 ± 8.6 years) were enrolled. According to the staging of diabetic nephropathy, TPF and knee extension force (KEF) were compared among three groups: normoalbuminuria, microalbuminuria, and overt nephropathy. In addition, we investigated factors influencing TPF and KEF by performing multiple regression analysis. Normoalbuminuria group, microalbuminuria group, and overt nephropathy group included 26, 25, and 23 patients, respectively. The TPF of the overt nephropathy group (3.15 ± 0.75 kg) was significantly lower than that of the normoalbuminuria (4.2 ± 0.7 kg, p diabetic polyneuropathy (DPN) and diabetic nephropathy were determinant factors of the TPF; and age, body mass index, and diabetic nephropathy were determinant factors of the KEF. We found in male patients with diabetic nephropathy, the TPF and KEF decreased with progression of diabetic nephropathy. Furthermore, our findings suggest diabetic nephropathy and DPN are critically involved in the reduction of TPF and KEF.

Endemic Nephropathy Around the World.

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Gifford, Fiona J; Gifford, Robert M; Eddleston, Michael; Dhaun, Neeraj

2017-03-01

There have been several global epidemics of chronic kidney disease of unknown etiology (CKD u ). Some, such as Itai-Itai disease in Japan and Balkan endemic nephropathy, have been explained, whereas the etiology of others remains unclear. In countries such as Sri Lanka, El Salvador, Nicaragua, and India, CKD u is a major public health problem and causes significant morbidity and mortality. Despite their geographical separation, however, there are striking similarities between these endemic nephropathies. Young male agricultural workers who perform strenuous labor in extreme conditions are the worst affected. Patients remain asymptomatic until end-stage renal failure. Biomarkers of tubular injury are raised, and kidney biopsy shows chronic interstitial nephritis with associated tubular atrophy. In many of these places access to dialysis and transplantation is limited, leaving few treatment options. In this review we briefly describe the major historic endemic nephropathies. We then summarize the epidemiology, clinical features, histology and clinical course of CKD u in Mesoamerica, Sri Lanka, India, Egypt, and Tunisia. We draw comparisons between the proposed etiologies and supporting research. Recognition of the similarities may reinforce the international drive to establish causality and to effect prevention.

Endemic Nephropathy Around the World

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Fiona J. Gifford

2017-03-01

Full Text Available There have been several global epidemics of chronic kidney disease of unknown etiology (CKDu. Some, such as Itai-Itai disease in Japan and Balkan endemic nephropathy, have been explained, whereas the etiology of others remains unclear. In countries such as Sri Lanka, El Salvador, Nicaragua, and India, CKDu is a major public health problem and causes significant morbidity and mortality. Despite their geographical separation, however, there are striking similarities between these endemic nephropathies. Young male agricultural workers who perform strenuous labor in extreme conditions are the worst affected. Patients remain asymptomatic until end-stage renal failure. Biomarkers of tubular injury are raised, and kidney biopsy shows chronic interstitial nephritis with associated tubular atrophy. In many of these places access to dialysis and transplantation is limited, leaving few treatment options. In this review we briefly describe the major historic endemic nephropathies. We then summarize the epidemiology, clinical features, histology and clinical course of CKDu in Mesoamerica, Sri Lanka, India, Egypt, and Tunisia. We draw comparisons between the proposed etiologies and supporting research. Recognition of the similarities may reinforce the international drive to establish causality and to effect prevention.

Renal involvement in the antiphospholipid syndrome (APS)-APS nephropathy.

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Tektonidou, Maria G

2009-06-01

Although the kidney represents a major target organ in antiphospholipid syndrome (APS), renal involvement in APS was poorly recognized until recently. The most well-recognized renal manifestations of APS are the renal artery thrombosis/stenosis, renal infarction, hypertension, renal vein thrombosis, end-stage renal disease, increased allograft vascular thrombosis, some types of glomerular disease, and a small-vessel vaso-occlusive nephropathy, recently defined as APS nephropathy. APS nephropathy was first described in primary APS patients, characterized by acute thrombotic lesions in glomeruli and/or arterioles (thrombotic microangiopathy) and chronic vascular lesions such as fibrous intimal hyperplasia of arterioles and interlobular arteries, organized thrombi with or without recanalization, and fibrous arterial and arteriolar occlusions or focal cortical atrophy. APS nephropathy was also detected in further studies including patients with systemic lupus erythematosus (SLE)-related APS and SLE/non-APS patients with positive antiphospholipid antibodies, independently of lupus nephritis. The same histologic lesions, especially thrombotic mictroangiopathy, were also observed in patients with catastrophic APS. The most frequent clinical and laboratory characteristics of APS nephropathy in all the above groups of patients are hypertension (often severe), proteinuria (ranging from mild to nephrotic range), hematuria, and acute or chronic renal insufficiency.

Case Report - Analgesic nephropathy as a cause of end‑stage renal ...

African Journals Online (AJOL)

Analgesic nephropathy is a subtle but significant cause of chronic renal failure. There is paucity of data on analgesic nephropathy in Nigeria. This case presentation is to highlight the need to have high index of suspicion in patients at risk of developing analgesic nephropathy. In March 2009 a 55‑year‑old businessman was ...

Membranous Septal Aneurysm: An Unusual Case for Sub-Pulmonary Obstruction in cTGA

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Onur Isik

2013-10-01

Full Text Available Aneurysm of the membranous portion of the interventricular septum is an uncommon congenital cardiac malformation that is rarely diagnosed during life. Perimembranous ventricular septal defects are the most common type of the ventricular septal defects and originates from morphologically membranous interventricular septum. Surgical exposure and accurate closure of a ventricular septal defect with a membranous septal aneurysm beneath the tricuspid septal leaflet carries a risk of tricuspid valve insufficiency and conduction disturbances. The current study presents a case with membranous septal aneursym with congenitally corrected transposition of the great arteries and sub-pulmonary obstruction, which was surgically corrected.

Role of Nutrient-Sensing Signals in the Pathogenesis of Diabetic Nephropathy

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Shinji Kume

2014-01-01

Full Text Available Diabetic nephropathy is the leading cause of end-stage renal disease worldwide. The multipronged drug approach still fails to fully prevent the onset and progression of diabetic nephropathy. Therefore, a new therapeutic target to improve the prognosis of diabetic nephropathy is urgently required. Nutrient-sensing signals and their related intracellular machinery have evolved to combat prolonged periods of starvation in mammals; and these systems are conserved in the kidney. Recent studies have suggested that the activity of three nutrient-sensing signals, mTORC1, AMPK, and Sirt1, is altered in the diabetic kidney. Furthermore, autophagy activity, which is regulated by the above-mentioned nutrient-sensing signals, is also altered in both podocytes and proximal tubular cells under diabetic conditions. Under diabetic conditions, an altered nutritional state owing to nutrient excess may disturb cellular homeostasis regulated by nutrient-responsible systems, leading to exacerbation of organelle dysfunction and diabetic nephropathy. In this review, we discuss new findings showing relationships between nutrient-sensing signals, autophagy, and diabetic nephropathy and suggest the therapeutic potential of nutrient-sensing signals in diabetic nephropathy.

Correlation of secreted protein acidic and rich in cysteine with diabetic nephropathy

OpenAIRE

Li, Lei; Song, Hai-Yan; Liu, Kai; An, Meng-Meng

2015-01-01

To detect the serum concentrations of secreted protein acidic and rich in cysteine (SPARC) in patients with diabetic nephropathy and SPARC mRNA and protein expressions in renal tissue of db/db mice (C57BL/KsJ, diabetic nephropathy mice), thus preliminary exploration on the role of secreted protein acidic riches in cysteine in the development of diabetic nephropathy were carried out. Serum SPARC levels in normal subjects, patients with type 2 diabetes mellitus (without diabetic nephropathy), c...

Ectopic ureter associated with uterine didelphys and obstructed hemivagina: preoperative diagnosis by MRI

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Wang, Zhen J.; Daldrup-Link, Heike; Coakley, Fergus V.; Yeh, Benjamin M. [University of California, San Francisco (United States). Department of Radiology

2010-03-15

Uterine didelphys with obstructed hemivagina and ipsilateral renal anomalies is a rare congenital malformation of the female urogenital tract. While the urinary anomalies almost always involve renal agenesis, we report a rare case of a 17-year-old girl with the malformation associated with ectopic ureteral insertion into the obstructed hemivagina, which was diagnosed preoperatively by MR imaging. To the best of our knowledge, preoperative MR imaging diagnosis of the ectopic ureter associated with this syndrome has not been previously reported. Accurate preoperative diagnosis of ectopic ureteral insertion associated with this syndrome is important for surgical planning. (orig.)

Ectopic ureter associated with uterine didelphys and obstructed hemivagina: preoperative diagnosis by MRI

International Nuclear Information System (INIS)

Wang, Zhen J.; Daldrup-Link, Heike; Coakley, Fergus V.; Yeh, Benjamin M.

2010-01-01

Uterine didelphys with obstructed hemivagina and ipsilateral renal anomalies is a rare congenital malformation of the female urogenital tract. While the urinary anomalies almost always involve renal agenesis, we report a rare case of a 17-year-old girl with the malformation associated with ectopic ureteral insertion into the obstructed hemivagina, which was diagnosed preoperatively by MR imaging. To the best of our knowledge, preoperative MR imaging diagnosis of the ectopic ureter associated with this syndrome has not been previously reported. Accurate preoperative diagnosis of ectopic ureteral insertion associated with this syndrome is important for surgical planning. (orig.)

Congenital Midureteric Stricture: Challenges in Diagnosis and Management

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Raashid Hamid

2015-01-01

Full Text Available Background. Congenital midureteric stricture (MUS is a rare malformation. We report our experience with five cases seen over a period of 4 years from 2010 to 2014. Materials and Methods. The study was based on the retrospective analysis of five patients diagnosed as having MUS. Diagnosis was suspected after fetal ultrasonography (USG in one patient and magnetic resonance urography (MRU in four patients. Retrograde pyelography (RGP was performed on three patients. The final diagnosis was confirmed during surgical exploration in all the patients. Results. MRU was found to be a good investigation method. It showed the site of obstruction in the ureter in all instances. Intravenous urography detected proximal ureteric dilatation present in two of the patients. RGP delineates the level of stricture and the course of ureter, as shown in our cases. All patients had significant obstruction on the affected side. Four patients underwent ureteroureterostomy, all of whom had satisfactory results. In one patient, ureteric reimplantation was carried out due to distal small ureteric caliber. Conclusion. This rare entity is often misdiagnosed initially as pelviureteric junction obstruction. MRU is an excellent option for the anatomical location and functional assessment of the involved system. At the time of surgical correction of a ureteral obstruction, RGP is a useful adjunct for delineating the stricture level and morphology.

Acute oxalate nephropathy after ingestion of star fruit.

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Chen, C L; Fang, H C; Chou, K J; Wang, J S; Chung, H M

2001-02-01

Acute oxalate nephropathy associated with ingestion of star fruit (carambola) has not been reported before. We report the first two cases. These patients developed nausea, vomiting, abdominal pain, and backache within hours of ingesting large quantities of sour carambola juice; then acute renal failure followed. Both patients needed hemodialysis for oliguric acute renal failure, and pathologic examinations showed typical changes of acute oxalate nephropathy. The renal function recovered 4 weeks later without specific treatment. Sour carambola juice is a popular beverage in Taiwan. The popularity of star fruit juice is not compatible with the rare discovery of star fruit-associated acute oxalate nephropathy. Commercial carambola juice usually is prepared by pickling and dilution processes that reduce oxalate content markedly, whereas pure fresh juice or mild diluted postpickled juice for traditional remedies, as used in our cases, contain high quantities of oxalate. An empty stomach and dehydrated state may pose an additional risk for development of renal injury. To avoid acute oxalate nephropathy, pure sour carambola juice or mild diluted postpickled juice should not be consumed in large amounts, especially on an empty stomach or in a dehydrated state.

Bilateral congenital lacrimal fistulas in an adult as part of ectrodactyly-ectodermal dysplasia-clefting syndrome: A rare anomaly.

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Ghosh, Debangshu; Saha, Somnath; Basu, Sumit Kumar

2015-10-01

Ectrodactyly-ectodermal dysplasia and clefting syndrome or "Lobster claw" deformity is a rare congenital anomaly that affects tissues of ectodermal and mesodermal origin. Nasolacrimal duct (NLD) obstruction with or without atresia of lacrimal passage is a common finding of such a syndrome. The authors report here even a rarer presentation of the syndrome which manifested as bilateral NLD obstruction and lacrimal fistula along with cleft lip and palate, syndactyly affecting all four limbs, mild mental retardation, otitis media, and sinusitis. Lacrimal duct obstruction and fistula were managed successfully with endoscopic dacryocystorhinostomy (DCR) which is a good alternative to lacrimal probing or open DCR in such a case.

Bilateral congenital lacrimal fistulas in an adult as part of ectrodactyly-ectodermal dysplasia-clefting syndrome: A rare anomaly

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Debangshu Ghosh

2015-01-01

Full Text Available Ectrodactyly-ectodermal dysplasia and clefting syndrome or "Lobster claw" deformity is a rare congenital anomaly that affects tissues of ectodermal and mesodermal origin. Nasolacrimal duct (NLD obstruction with or without atresia of lacrimal passage is a common finding of such a syndrome. The authors report here even a rarer presentation of the syndrome which manifested as bilateral NLD obstruction and lacrimal fistula along with cleft lip and palate, syndactyly affecting all four limbs, mild mental retardation, otitis media, and sinusitis. Lacrimal duct obstruction and fistula were managed successfully with endoscopic dacryocystorhinostomy (DCR which is a good alternative to lacrimal probing or open DCR in such a case.

Concomitant macro and microvascular complications in diabetic nephropathy

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Alwakeel Jamal

2009-01-01

Full Text Available To determine the prevalence of concomitant microvascular and macrovascular complica-tions of diabetic nephropathy we retrospectively reviewed the medical records of all 1,952 type 2 dia-betic patients followed-up at Security Forces Hospital, Riyadh, Saudi Arabia from January 1989 to December 2004. There were 626 (32.1% patients (294 (47% were males who developed diabetic nephropathy. Their mean age was 66.9 ± 11.4 years, mean duration of diabetes was 15.4 ± 7.5 years, mean age at the onset of nephropathy was 61.5 ± 12.4 years, and mean duration of nephropathy was 3.9 ± 3.8 years. Concomitant diabetic complications included cataract (38.2%, acute coronary syndrome (36.1%, peripheral neuropathy (24.9%, myocardial infarction (24.1%, background retinopathy (22.4%, stroke (17.6%, proliferative retinopathy (11.7%, foot infection (7.3%, limb amputation (3.7% and blindness (3%. Hypertension was documented in 577 (92.2% patients, dyslipidemia in 266 (42.5% and mortality from all causes in 86 (13.7%. There were 148 (23.6% patients with one complication, 81 (12.9% with two, 83 (13.3% with three, and 61 (9.7% with four or more. Dete-rioration of glomerular filtration rate was observed in 464 (74% patients and doubling of serum creatinine in 250 (39.9%, while 95 (15.2% developed end-stage renal disease (ESRD at the end of study and 79 (12.6% required dialysis. Complications were significantly more prevalent among males with greater number reaching ESRD level than females (P< 0.05. Relative risks of developing com-plications were significant after the onset of nephropathy; ACS (1.41, MI (1.49, stroke (1.48, diabetic foot (1.6, amputation (1.58 and death (1.93. We conclude that complications of diabetes are aggre-ssive and progressive including high prevalence of diabetic nephropathy. Careful monitoring and proper institution of management protocols should be implemented to identify diabetic patients at high risk for complications and mitigate progression

Concomitant macro and microvascular complications in diabetic nephropathy

International Nuclear Information System (INIS)

Alwakeel, Jamal S; AlSuwaida, Abdulkareem; Isnani, Arthur C; AlHarbi, Ali; Alam Awatif

2009-01-01

To determine the prevalence of concomitant microvascular and macro vascular complications of diabetic nephropathy we retrospectively reviewed the medical records of all 1,952 type 2 dia-betic patients followed-up at Security Forces Hospital, Riyadh, Saudi Arabia from January 1989 to December 2004. There were 626 (32.1%) patients (294 (47%) were males) who developed diabetic nephropathy. Their mean age was 66.9 + -11.4 years, mean duration of diabetes was 15.4 + -7.5 years, mean age at the onset of nephropathy was 61.5 + - 12.4 years, and mean duration of nephropathy was 3.9 + - 3.8 years. Concomitant diabetic complications included cataract (38.2%), acute coronary syndrome (36.1%), peripheral neuropathy (24.9%), myocardial infarction (24.1%), background retinopathy (22.4%), stroke (17.6%), proliferative retinopathy (11.7%), foot infection (7.3%), limb amputation (3.7%) and blindness (3%). Hypertension was documented in 577 (92.2%) patients, dyslipidemia in 266 (42.5%) and mortality from all causes in 86 (13.7%). There were 148 (23.6%) patients with one complication, 81 (12.9%) with two, 83 (13.3%) with three, and 61 (9.7%) with four or more. Deterioration of glomerular filtration rate was observed in 464 (74%) patients and doubling of serum creatinine in 250 (39.9%), while 95 (15.2%) developed end-stage renal disease (ESRD) at the end of study and 79 (12.6%) required dialysis. Complications were significantly more prevalent among males with greater number reaching ESRD level than females (P< 0.05). Relative risks of developing complications were significant after the onset of nephropathy; ACS (1.41), MI (1.49), stroke (1.48), diabetic foot (1.6), amputation (1.58) and death (1.93). We conclude that complications of diabetes are aggressive and progressive including high prevalence of diabetic nephropathy. Careful monitoring and proper institution of management protocols should be implemented to identify diabetic patients at high risk for complications and mitigate

Concomitant macro and microvascular complications in diabetic nephropathy

Energy Technology Data Exchange (ETDEWEB)

Alwakeel, Jamal S; AlSuwaida, Abdulkareem [Div. of Nephrology, Dept. of Medicine, King Khalid Univ., Hospital, Riyadh (Saudi Arabia); Isnani, Arthur C [Dept. of Family and Community Medicine, King Khalid Univ. Hospital, Riyadh (Saudi Arabia); AlHarbi, Ali [Coll. of Medicine and Research Center, King Khalid Univ. Hospital, Riyadh (Saudi Arabia); Awatif, Alam [Div. of Nephrology, Dept. of Medicine, Security Forces Hospital, Riyadh (Saudi Arabia)

2009-07-01

To determine the prevalence of concomitant microvascular and macro vascular complications of diabetic nephropathy we retrospectively reviewed the medical records of all 1,952 type 2 dia-betic patients followed-up at Security Forces Hospital, Riyadh, Saudi Arabia from January 1989 to December 2004. There were 626 (32.1%) patients (294 (47%) were males) who developed diabetic nephropathy. Their mean age was 66.9 + -11.4 years, mean duration of diabetes was 15.4 + -7.5 years, mean age at the onset of nephropathy was 61.5 + - 12.4 years, and mean duration of nephropathy was 3.9 + - 3.8 years. Concomitant diabetic complications included cataract (38.2%), acute coronary syndrome (36.1%), peripheral neuropathy (24.9%), myocardial infarction (24.1%), background retinopathy (22.4%), stroke (17.6%), proliferative retinopathy (11.7%), foot infection (7.3%), limb amputation (3.7%) and blindness (3%). Hypertension was documented in 577 (92.2%) patients, dyslipidemia in 266 (42.5%) and mortality from all causes in 86 (13.7%). There were 148 (23.6%) patients with one complication, 81 (12.9%) with two, 83 (13.3%) with three, and 61 (9.7%) with four or more. Deterioration of glomerular filtration rate was observed in 464 (74%) patients and doubling of serum creatinine in 250 (39.9%), while 95 (15.2%) developed end-stage renal disease (ESRD) at the end of study and 79 (12.6%) required dialysis. Complications were significantly more prevalent among males with greater number reaching ESRD level than females (P< 0.05). Relative risks of developing complications were significant after the onset of nephropathy; ACS (1.41), MI (1.49), stroke (1.48), diabetic foot (1.6), amputation (1.58) and death (1.93). We conclude that complications of diabetes are aggressive and progressive including high prevalence of diabetic nephropathy. Careful monitoring and proper institution of management protocols should be implemented to identify diabetic patients at high risk for complications and mitigate

Congenital Treves' field transmesenteric hernia in children: A case series and literature review

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Ryuta Saka

2015-08-01

Full Text Available Transmesenteric hernia is abdominal visceral herniation through a congenital or acquired mesenteric defect. Treves' field is the area of terminal ileal mesentery circumscribed by the ileocolic artery and its last ileal branch. It is very susceptible to congenital defects. To clarify the clinical course of congenital Treves' field transmesenteric hernia (cTFTH, we retrospectively reviewed pediatric cases and conducted a literature review. Five consecutive pediatric cTFTH cases (one male, four females underwent emergency laparotomy at our institutions from April 2009 and December 2014. We analyzed their demographics, preoperative findings, surgical procedures, and outcomes, along with a literature review. Abdominal plain X-ray showed displaced intestinal gas with gas paucity in the center of the abdomen in two cases and diffusely increased intestinal gas in two others. Abdominal computed tomography (CT in four cases showed intestinal loop clusters, mesenteric vessel changes, small-bowel obstruction, and ascites. All cases underwent emergency laparotomy for ileal resection with ileocecal valve preservation. Simultaneous anastomoses were performed in four cases; the remaining case underwent ileostomy because of prematurity. Although one case needed reoperation for postoperative bowel obstruction, all patients survived. Literature review of pediatric cTFTH showed a high frequency of necrosis (70.5% and mortality (26.5%. Among pediatric cTFTH cases, 67.6% were aged <5 years. Mesenteric defect was <5 cm in 70% cases. We should be aware of the clinical importance and radiological features of pediatric cTFTH. Abdominal CT may provide useful information.

Sodium bicarbonate-based hydration prevents contrast-induced nephropathy: a meta-analysis

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Tamhane Umesh

2009-05-01

Full Text Available Abstract Background Contrast-induced nephropathy is the leading cause of in-hospital acute renal failure. This side effect of contrast agents leads to increased morbidity, mortality, and health costs. Ensuring adequate hydration prior to contrast exposure is highly effective at preventing this complication, although the optimal hydration strategy to prevent contrast-induced nephropathy still remains an unresolved issue. Former meta-analyses and several recent studies have shown conflicting results regarding the protective effect of sodium bicarbonate. The objective of this study was to assess the effectiveness of normal saline versus sodium bicarbonate for prevention of contrast-induced nephropathy. Methods The study searched MEDLINE, EMBASE, Cochrane databases, International Pharmaceutical Abstracts database, ISI Web of Science (until 15 December 2008, and conference proceedings for randomized controlled trials that compared normal saline with sodium bicarbonate-based hydration regimen regarding contrast-induced nephropathy. Random-effects models were used to calculate summary odds ratios. Results A total of 17 trials including 2,633 subjects were pooled. Pre-procedural hydration with sodium bicarbonate was associated with a significant decrease in the rate of contrast-induced nephropathy (odds ratios 0.52; 95% confidence interval 0.34–0.80, P = 0.003. Number needed to treat to prevent one case of contrast-induced nephropathy was 16 (95% confidence interval 10–34. No significant differences in the rates of post-procedure hemodialysis (P = 0.20 or death (P = 0.53 was observed. Conclusion Sodium bicarbonate-based hydration was found to be superior to normal saline in prevention of contrast-induced nephropathy in this updated meta-analysis.

Clustering of risk factors in parents of patients with type 1 diabetes and nephropathy.

Science.gov (United States)

Thorn, Lena M; Forsblom, Carol; Fagerudd, Johan; Pettersson-Fernholm, Kim; Kilpikari, Riika; Groop, Per-Henrik

2007-05-01

To assess the impact of parental risk factors for diabetic nephropathy. This cross-sectional study included 2,355 type 1 diabetic patients from the FinnDiane (Finnish Diabetic Nephropathy) study. Diabetic nephropathy was defined as macroalbuminuria (urinary albumin excretion rate >200 microg/min or >300 mg/24 h) or end-stage renal disease. Information was available from 4,676 parents. Parental scores were calculated based on the number of various traits in the parents. Patients with diabetic nephropathy, compared with those without diabetic nephropathy, had a higher prevalence of maternal (41 vs. 35%, P = 0.046) and parental (62 vs. 55%, P = 0.044) hypertension, maternal stroke (7.6 vs. 5.1%, P = 0.044), and maternal (1.4 vs. 0.7%, P = 0.058) and parental (4.3 vs. 2.9%, P = 0.030) type 1 diabetes. If both, compared with none, of the parents had hypertension, the adjusted odds ratio (OR) for diabetic nephropathy in offspring was 1.56 (95% CI 1.13-2.15). The adjusted OR for diabetic nephropathy was 2.13 (1.36-3.33) for the parental hypertension-diabetes score (3-4 vs. 0 points) and 2.13 (1.37-3.33) for the parental hypertension-cardiovascular disease (CVD)-diabetes score (4-6 vs. 0 points). Fathers of patients with diabetic nephropathy, compared with those without diabetic nephropathy, had reduced overall survival (log-rank P = 0.04) and reduced cardiovascular survival (log-rank P = 0.03). A cluster of parental hypertension, CVD, and diabetes is associated with diabetic nephropathy in type 1 diabetes, as is paternal mortality.

Angiotensinogen gene polymorphisms in IDDM patients with diabetic nephropathy

DEFF Research Database (Denmark)

Tarnow, L; Cambien, Francois; Rossing, P

1996-01-01

Genotypic abnormalities of the renin-ANG system have been suggested as a risk factor for the development of diabetic nephropathy. Cleavage of angiotensinogen is the rate-limiting step in the activation of the renin-ANG system. The TT genotype of a polymorphism encoding threonine instead of methio......Genotypic abnormalities of the renin-ANG system have been suggested as a risk factor for the development of diabetic nephropathy. Cleavage of angiotensinogen is the rate-limiting step in the activation of the renin-ANG system. The TT genotype of a polymorphism encoding threonine instead...... of methionine (M235T) has been associated not only with increased plasma angiotensinogen concentration but also with essential hypertension. In addition, a polymorphism in the angiotensinogen gene substituting methionine for threonine (T174M) has been associated with hypertension in nondiabetic populations. We...... studied the relationship between these polymorphisms in the angiotensinogen gene in IDDM patients with diabetic nephropathy (121 men, 74 women, age 40.9 +/- 10 years, diabetes duration 27 +/- 8 years). There was no difference in M235T genotype distribution between IDDM patients with diabetic nephropathy...

Nasopharyngeal encephalocele: a rare cause of upper airway obstruction.

Science.gov (United States)

Kalkan, Gokhan; Paksu, Sukru; Asilioglu, Nazik; Kiliç, Mehmet

2013-04-01

Nasopharyngeal encephalocele is a rare, benign congenital anomaly. It has the potential to be fatal due to airway obstruction. Here, we report on a 34-day-old infant with pneumonia who underwent mechanical ventilation. An upper airway evaluation was performed due to prolonged intubation, and revealed the presence of a nasopharyngeal encephalocele. The patient tolerated extubation and oral feeding after surgical resection of the lesion. Awareness of the condition can help clinicians arrive at an earlier diagnosis and enhance management.

Grave′s disease associated with immunoglobulin A nephropathy: A rare association

OpenAIRE

I Khan; R A Bhat; I Khan; I Hameed

2015-01-01

Immunoglobulin A (Ig A) nephropathy is the most common form of primary glomerulonephritis. The association of Ig A nephropathy with Grave's disease has not been reported so far. We report a case of 20-year-old female with Grave's disease who presented with edema, facial puffiness, and decreased urine output. She was found to be hypertensive with renal failure and nephrotic range proteinuria. Renal biopsy revealed features of Ig A nephropathy. The patient was treated with oral corticosteroids ...

IgA nephropathy enigma

Czech Academy of Sciences Publication Activity Database

Městecký, Jiří; Novák, J.; Moldoveanu, Z.; Raška, M.

2016-01-01

Roč. 172, NOV 2016 SI (2016), s. 72-77 ISSN 1521-6616 R&D Projects: GA MZd(CZ) NV15-33686A Institutional support: RVO:61388971 Keywords : IgA nephropathy * IgA subclasses * Autoimmunity Subject RIV: EE - Microbiology, Virology Impact factor: 3.990, year: 2016

Endothelial nitric oxide synthase gene haplotypes and diabetic nephropathy among Asian Indians

DEFF Research Database (Denmark)

Ahluwalia, Tarun Veer Singh; Ahuja, Monica; Rai, Taranjit Singh

2008-01-01

of the constitutive endothelial nitric oxide synthase gene (eNOS) polymorphisms with type 2 diabetic nephropathy. We genotyped three polymorphisms of eNOS (Two SNPs: -786T > C, 894G > T and one 27-bp repeat polymorphism in Intron 4 (27VNTR)) in type 2 diabetic nephropathy patients (cases: n = 195) and type 2 diabetic...... without nephropathy (controls: n = 255), using validated PCR-RFLP assays. We measured serum NO levels in these subjects and examined its correlation with diabetic nephropathy and eNOS genotypes. The frequency of CC (-786T > C), TT (894G > T) and aa genotypes (27VNTR) were significantly higher in diabetic...

Small bowel obstruction complicating colonoscopy: a case report

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Hunter Iain A

2008-05-01

Full Text Available Abstract Introduction This report describes a rare complication of colonoscopy and reviews the literature with regard to other rare causes of acute abdominal presentations following colonoscopy. Case presentation After a therapeutic colonoscopy a 60-year-old woman developed an acute abdomen. At laparotomy she was discovered to have small bowel obstruction secondary to incarceration through a congenital band adhesion. Conclusion Although there is no practical way in which such rare complications can be predicted, this case report emphasises the wide array of pathologies that can result in acute abdominal symptoms following colonoscopy.

Evolution of IgA nephropathy into anaphylactoid purpura in six cases--further evidence that IgA nephropathy and Henoch-Schonlein purpura nephritis share common pathogenesis.

Science.gov (United States)

Kamei, Koichi; Ogura, Masao; Sato, Mai; Ito, Shuichi; Ishikura, Kenji

2016-05-01

As the morphological and immunohistochemical manifestations of immunoglobulin A (IgA) nephropathy and Henoch-Schonlein purpura nephritis (HSPN) are very similar, they are considered to share a common pathogenesis. Although HSPN usually develops after the appearance of anaphylactoid purpura, we have encountered patients whose renal symptoms preceded purpura. We reviewed the clinical courses of patients who were first diagnosed with IgA nephropathy, but developed purpura later, at the National Center for Child Health and Development in Tokyo, Japan. Of the 53 patients who were diagnosed with primary IgA nephropathy at our institute during the study period (March 2002 to July 2015), six (11 %) developed anaphylactoid purpura after the diagnosis of primary IgA nephropathy and therefore met the inclusion criteria. Duration between the onset of nephritis and subsequent appearance of purpura ranged from 5 months to 14 years. One patient reached end-stage renal failure due to IgA nephropathy and developed purpura after renal transplantation. All renal biopsies performed before the appearance of purpura showed mesangial proliferation with predominant IgA deposits. Urinary findings deteriorated in three patients after the appearance of purpura, including one patient who developed rapidly progressive glomerulonephritis. Renal biopsy findings worsened in two patients. At the last observation, two patients showed mild renal insufficiency. Our clinical experience and previous reports support the argument that IgA nephropathy and HSPN are different manifestations of a single disease. Hence, it is acceptable to consider that they are variants of a single disease.

Dioxins, furans and dioxin-like PCBs in human blood: causes or consequences of diabetic nephropathy?

Science.gov (United States)

Everett, Charles J; Thompson, Olivia M

2014-07-01

Nephropathy, or kidney disease, is a major, potential complication of diabetes. We assessed the association of 6 chlorinated dibenzo-p-dioxins, 9 chlorinated dibenzofurans and 8 polychlorinated biphenyls (PCBs) in blood with diabetic nephropathy in the 1999-2004 National Health and Nutrition Examination Survey (unweighted N=2588, population estimate=117,658,357). Diabetes was defined as diagnosed or undiagnosed (glycohemoglobin ≥ 6.5%) and nephropathy defined as urinary albumin to creatinine ratio >30 mg/g, representing microalbuminuria or macroalbuminuria. For the 8 chemicals analyzed separately, values above the 75th percentile were considered elevated, whereas for the other 15 compounds values above the maximum limit of detection were considered elevated. Seven of 8 dioxins and dioxin-like compounds, analyzed separately, were found to be associated with diabetic nephropathy. The chemicals associated with diabetic nephropathy were: 1,2,3,6,7,8-Hexachlorodibenzo-p-dioxin; 1,2,3,4,6,7,8,9-Octachlorodibenzo-p-dioxin; 2,3,4,7,8-Pentachlorodibenzofuran; PCB 126; PCB 169; PCB 118; and PCB 156. Three of the 8 dioxins and dioxin-like compounds; 1,2,3,4,6,7,8,9-Octachlorodibenzo-p-dioxin; 2,3,4,7,8-Pentachlorodibenzofuran and PCB 118; expressed as log-transformed continuous variables; were associated with diabetes without nephropathy. When 4 or more of the 23 chemicals were elevated the odds ratios were 7.00 (95% CI=1.80-27.20) for diabetic nephropathy and 2.13 (95% CI=0.95-4.78) for diabetes without nephropathy. Log-transformed toxic equivalency (TEQ) was associated with both diabetic nephropathy, and diabetes without nephropathy, the odds ratios were 2.35 (95% CI=1.57-3.52) for diabetic nephropathy, and 1.44 (95% CI=1.11-1.87) for diabetes without nephropathy. As the kidneys function to remove waste products from the blood, diabetic nephropathy could be either the cause or the consequence (or both) of exposure to dioxins, furans and dioxin-like PCBs. Copyright © 2014

Uterine Artery Embolization for Ureteric Obstruction Secondary to Fibroids

International Nuclear Information System (INIS)

Mirsadraee, Saeed; Tuite, David; Nicholson, Anthony

2008-01-01

This case series examines the safety and efficacy of uterine artery embolization (UAE) in the treatment of obstructive nephropathy caused by large fibroids. Between 2004 and 2007, 10 patients referred with symptomatic uterine fibroids that were found to be causing either unilateral (7 patients) or bilateral (3 patients) hydronephrosis were treated by UAE. Presenting complaints included menorrhagia, dysmenorrhea, bulk symptoms, loin pain, postobstructive atrophy, and mild renal impairment. All had posterior intramural dominant fibroids >11 cm in maximum sagittal diameter and uterine volumes between 3776 and 15,625 ml. Outcome measures at between 12 and 36 months included procedural success, repeat intervention, relief of symptoms, resolution of hydronephrosis, stable renal function and size, and avoidance of hysterectomy. In all cases the cause of renal obstruction was confirmed to be a giant fibroid compressing the ureter at the pelvic brim. In all cases UAE was technically successful, though two patients required a repeat procedure. In eight patients hydronephrosis resolved and the obstruction was relieved, though two still had some bulk symptoms not requiring further treatment. Renal function improved or was stable in all cases. Renal size was stable in all cases. Where menorrhagia was part of the symptom complex it was relieved in all cases. Two patients diagnosed as having postobstructive atrophy of one kidney underwent retrograde ureteric stenting on the nonatrophied side prior to UAE. This was unsuccessful in one of the cases due to the distortion caused by the fibroid. Despite improvement in hydronephrosis this patient underwent hysterectomy at 7 months after a renogram demonstrated persistent obstruction at the pelvic brim. In the second patient a double pigtail stent was inserted with difficulty and eventually removed at 8 months. This patient has had stable renal function and size for 3 years post-UAE. We conclude that UAE is safe and effective in

Arctigenin suppresses renal interstitial fibrosis in a rat model of obstructive nephropathy.

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Li, Ao; Zhang, Xiaoxun; Shu, Mao; Wu, Mingjun; Wang, Jun; Zhang, Jingyao; Wang, Rui; Li, Peng; Wang, Yitao

2017-07-01

Renal tubulointerstitial fibrosis (TIF) is commonly the final result of a variety of progressive injuries and leads to end-stage renal disease. There are few therapeutic agents currently available for retarding the development of renal TIF. The aim of the present study is to evaluate the role of arctigenin (ATG), a lignan component derived from dried burdock (Arctium lappa L.) fruits, in protecting the kidney against injury by unilateral ureteral obstruction (UUO) in rats. Rats were subjected to UUO and then administered with vehicle, ATG (1 and 3mg/kg/d), or losartan (20mg/kg/d) for 11 consecutive days. The renoprotective effects of ATG were evaluated by histological examination and multiple biochemical assays. Our results suggest that ATG significantly protected the kidney from injury by reducing tubular dilatation, epithelial atrophy, collagen deposition, and tubulointerstitial compartment expansion. ATG administration dramatically decreased macrophage (CD68-positive cell) infiltration. Meanwhile, ATG down-regulated the mRNA levels of pro-inflammatory chemokine monocyte chemoattractant protein-1 (MCP-1) and cytokines, including tumor necrosis factor-α (TNF-α), interleukin-1β (IL-1β), and interferon-γ (IFN-γ), in the obstructed kidneys. This was associated with decreased activation of nuclear factor κB (NF-κB). ATG attenuated UUO-induced oxidative stress by increasing the activity of renal manganese superoxide dismutase (SOD2), leading to reduced levels of lipid peroxidation. Furthermore, ATG inhibited the epithelial-mesenchymal transition (EMT) of renal tubules by reducing the abundance of transforming growth factor-β1 (TGF-β1) and its type I receptor, suppressing Smad2/3 phosphorylation and nuclear translocation, and up-regulating Smad7 expression. Notably, the efficacy of ATG in renal protection was comparable or even superior to losartan. ATG could protect the kidney from UUO-induced injury and fibrogenesis by suppressing inflammation, oxidative

Mycotoxic nephropathy in Bulgarian pigs and chickens: complex aetiology and similarity to Balkan endemic nephropathy

CSIR Research Space (South Africa)

Stoev, SD

2009-01-01

Full Text Available picture of this nephropathy. A heavy contamination with Gibberella fujikuroi var. moniliformis (Fusarium verticillioides) and Penicillium aurantiogriseum complex (mainly Penicillium polonicum) was observed in almost all examined feed samples coming from...

A complex microdeletion 17q12 phenotype in a patient with recurrent de novo membranous nephropathy

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Hinkes Bernward

2012-05-01

Full Text Available Abstract Background Microdeletions on chromosome 17q12 cause of diverse spectrum of disorders and have only recently been identified as a rare cause of Mayer-Rokitansky-Kuester-Hauser-Syndrome (MRKH, which is characterized by uterus aplasia ± partial/complete vaginal aplasia in females with a regular karyotype. For the first time we report about a patient with a 17q12 microdeletion who is affected by MRKH in combination with a vascular and soft tissue disorder. Repeatedly she suffered from kidney transplant failure caused by consuming membranous nephropathy. Case presentation A 38-year-old female patient had been diagnosed with right kidney aplasia, left kidney dysplasia and significantly impaired renal function during infancy. Aged 16 she had to start hemodialysis. Three years later she received her first kidney transplant. Only then she was diagnosed with MRKH. The kidney transplant was lost due to consuming nephrotic syndrome caused by de novo membranous nephropathy, as was a second kidney transplant years later. In addition, a hyperelasticity syndrome affects the patient with congenital joint laxity, kyphoscoliosis, bilateral hip dysplasia, persistent hypermobility of both elbows, knees and hips. Her clinical picture resembles a combination of traits of a hypermobile and a vascular form of Ehlers-Danlos-Syndrome, but no mutations in the COL3A1 gene was underlying. Instead, array-based comparative genomic hybridisation (CGH detected a heterozygous 1.43 Mb deletion on chromosome 17q12 encompassing the two renal developmental genes HNF1β and LHX1. Conclusions Deletions of HNF1β have recently drawn significant attention in pediatric nephrology as an important cause of prenatally hyperechogenic kidneys, renal aplasia and renal hypodysplasia. In contrast, membranous nephropathy represents an often-unaccounted cause of nephrotic syndrome in the adult population. A causative connection between theses two conditions has never been postulated, but

Elevated vascular endothelial growth factor in type 1 diabetic patients with diabetic nephropathy

DEFF Research Database (Denmark)

Hovind, P; Tarnow, L; Oestergaard, P B

2000-01-01

patients with and without proliferative retinopathy were detected. CONCLUSIONS: Our data suggest that VEGF is elevated early in the course of diabetic nephropathy in men with type 1 diabetes mellitus. Baseline albuminuria, arterial blood pressure and male gender was predictors of diabetic nephropathy......BACKGROUND: Growth factors have been suggested to play a role in the development and progression of diabetic nephropathy. Vascular endothelial growth factor (VEGF) is a potent cytokine family that induces angiogenesis and markedly increases endothelial permeability. The aim of the present study...... was to investigate plasma levels of VEGF in a large cohort of type 1 diabetic patients with diabetic nephropathy and in long-standing type 1 diabetic patients with persistent normoalbuminuria, and to evaluate VEGF as a predictor of nephropathy progression. METHODS: We measured VEGF with an enzyme...

Canadian Cardiovascular Society 2009 Consensus Conference on the management of adults with congenital heart disease: Outflow tract obstruction, coarctation of the aorta, tetralogy of Fallot, Ebstein anomaly and Marfan’s syndrome

Science.gov (United States)

Silversides, Candice K; Beauchesne, Luc; Bradley, Timothy; Connelly, Michael; Niwa, Koichiro; Mulder, Barbara; Webb, Gary; Colman, Jack; Therrien, Judith

2010-01-01

With advances in pediatric cardiology and cardiac surgery, the population of adults with congenital heart disease (CHD) has increased. In the current era, there are more adults with CHD than children. This population has many unique issues and needs. Since the 2001 Canadian Cardiovascular Society Consensus Conference report on the management of adults with CHD, there have been significant advances in the field of adult CHD. Therefore, new clinical guidelines have been written by Canadian adult CHD physicians in collaboration with an international panel of experts in the field. Part II of the guidelines includes recommendations for the care of patients with left ventricular outflow tract obstruction and bicuspid aortic valve disease, coarctation of the aorta, right ventricular outflow tract obstruction, tetralogy of Fallot, Ebstein anomaly and Marfan’s syndrome. Topics addressed include genetics, clinical outcomes, recommended diagnostic workup, surgical and interventional options, treatment of arrhythmias, assessment of pregnancy risk and follow-up requirements. The complete document consists of four manuscripts that are published online in the present issue of The Canadian Journal of Cardiology. The complete document and references can also be found at www.ccs.ca or www.cachnet.org. PMID:20352138

Effective antihypertensive treatment postpones renal insufficiency in diabetic nephropathy

DEFF Research Database (Denmark)

Parving, H H; Smidt, U M; Hommel, E

1993-01-01

The effect of long-term, aggressive, antihypertensive treatment on kidney function in diabetic nephropathy was studied prospectively in 11 insulin-dependent diabetic patients (mean age, 30 years). Renal function was assessed every 4 months by measurement of glomerular filtration rate (GFR) (single...... infarction (GFR, 46 mL/min/1.73 m2). Effective antihypertensive treatment postpones renal insufficiency in diabetic nephropathy....

Urachal Cyst Causing Small Bowel Obstruction in an Adult with a Virgin Abdomen

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Michael P. O’Leary

2016-01-01

Full Text Available Introduction. A patent urachus is a rare congenital or acquired pathology, which can lead to complications later in life. We describe a case of urachal cystitis as the etiology of small bowel obstruction in an adult without prior intra-abdominal surgery. Case Report. A 64-year-old male presented to the acute care surgery team with a 5-day history of right lower quadrant abdominal pain, distention, nausea, and vomiting. He had a two-month history of urinary retention and his past medical history was significant for benign prostate hyperplasia. On exam, he had evidence of small bowel obstruction. Computed tomography revealed high-grade small bowel obstruction secondary to presumed ruptured appendicitis. In the operating room, an infected urachal cyst was identified with adhesions to the proximal ileum. After lysis of adhesions and resection of the cyst, the patient was subsequently discharged without further issues. Conclusion. Although rare, urachal pathology should be considered in the differential diagnosis when evaluating a patient with small bowel obstruction without prior intraabdominal surgery, hernia, or malignancy.

Laparoscopic versus open surgery in management of congenital duodenal obstruction in neonates: a single-center experience with 112 cases.

Science.gov (United States)

Son, Tran Ngoc; Kien, Hoang Huu

2017-12-01

A single center study was conducted to compare the short-term clinical outcome between laparoscopic surgery (LS) and open surgery (OS) repair for neonates with congenital duodenal obstruction (CDO). Medical records of all neonates with bodyweight at surgery over 1500g and without other gastrointestinal anomalies that underwent surgery (duodeno-duodenostomy or duodeno-jejunostomy) for CDO at our center between January 2009 and July 2015 were reviewed. The choice of OS or LS was surgeon-dependent. One hundred twelve patients were identified, with a median age and weight at surgery 8.5days and 2500g respectively. Forty-four patients underwent OS and 68 patients LS. There were no significant differences between the two groups regarding patient age, gender, weight at surgery, associated anomalies, and mean operative time. Compared to OS, the LS group had lower postoperative complications (5.9% vs 36.4%, p<0.0001), shorter mean time to initial oral feeding and mean postoperative hospital stay (3.9 vs. 7.1days and 8.6 vs. 12.9days respectively, p<0.0001) and better postoperative cosmesis. LS treatment for neonatal CDO is associated with lower postoperative morbidity, shorter recovery time and postoperative hospital stay and better postoperative cosmesis than OS. Retrospective Comparative Study. Level III. Copyright © 2017 Elsevier Inc. All rights reserved.

Contrast nephropathy in high-risk patients undergoing coronary angiography and intervention

International Nuclear Information System (INIS)

Uddin, M.A.; Rabbani, M.A.; Jafary, F.H.; Bhatti, M.A.; Islam, M.

2005-01-01

Objective: To determine the incidence of contrast nephropathy in high-risk patients undergoing coronary angiography and percutaneous coronary intervention (PCI), and to define the characteristics of this cohort. Design: Descriptive study. Place and Duration of Study: The Aga Khan University Hospital, Karachi from January to December 2002. Patients and Methods: One hundred and fifteen patients with serum creatinine greater than 1.4mg/dl who underwent coronary angiography or PCI were included. All patients received non-ionic contrast dye. Acute contrast nephropathy was defined as rise in serum creatinine of >0.5mg/dl within 48 hours following the index procedure. Means and standard deviations were calculated for continuous variables and frequencies for categorical variables. Results: Mean age of patients was 62.3 year + 8.83. Mean pre-contrast creatinine was 1.9+0.9mg/dl. Eleven (9.65%) patients developed contrast nephropathy. 4.4% of patients with serum creatinine 4.0(p-value 0.001). 11.9% diabetic patients developed nephropathy compared to 6.3% of non-diabetics (p-value 0.355). 11.4% of hypertensive and 3.7% of non-hypertensive patients developed contrast-nephropathy (p-value 0.454). 12.9% of low dose group ( 100ml) developed nephropathy (p-value 0.188). Mean serum creatinine in low dose group was higher (3.0mg/dl vs. 1.7 mg/dl). Conclusion: The incidence of contrast nephropathy in this study was similar to that reported in literature. Risk of CIN was found to be significantly proportional to the severity of baseline renal disease. Trends towards higher risk of CIN were seen in patients with diabetes and hypertension. Higher incidence of CIN in patients receiving low-dose contrast was confounded by higher baseline serum creatinine in that group. (author)

Prenatal diagnosis of congenital ranula: Case Report

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Aytül Çorbacıoğlu Esmer

2013-12-01

Full Text Available Congenital ranula is a mucous retention cyst which originates from the base of the oral cavity and is caused by sublingual or submandibular canal atresia or canalization defect. As with the other tumors in the oral cavity it may cause upper airway obstruction and hypoxia immediately after delivery. In the following stages of life, apart from the respiratory problems it may cause speach, chewing and swallowing disorders. The prenatal diagnosis of congenital sublingual ranula is very rare, and it presents as an avascular and anechoic cystic mass displacing the tongue upwards on fetal ultrasonography. Polyhydramnios can develop due to the obstruction and stomach may not be visualized. The differential diagnosis includes lymphatic malformations, epulis, epignatus, tyroglossal canal cyst and hemangioma. In this paper, we present a case of ranula which was diagnosed during prenatal ultrasonographic examination. The detailed fetal ultrasound scan performed at the 31th week of gestation revealed a non-septated anechoic cyst in the oral cavity measuring 20 x 17 x 15 mm. The cyst had well-defined regular borders with no solid component. Doppler ultrasonograhy did not show any vascularization within the cyst. After the delivery at the 38th week of gestation, a sublingual cystic mass with regular borders measuring 1.5 x1.5 cm was detected in the mouth of the neonate. Entubation was not required due to the absence of respiratory distress. In order to prevent difficutlies in feeding, the cystic mass was aspirated just after the delivery. A diagnosis of sublingual ranula was made based on the localization and the mucoid consistency of the cyst.

Fractalkine in type 2 Egyptian diabetics with and without nephropathy

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Ebtissam Zakaria

2013-01-01

Results and Conclusion Our study showed that the serum fractalkine concentration was significantly elevated in type 2 diabetic patients with nephropathy (1153.14±261.1 compared with type 2 diabetic patients without nephropathy (705.78±150.59 and the control group (251.5±64 (both P=0.000. There was a significant correlation between serum fractalkine level and 24-h UAE, HBA1C, and serum creatinine. Thus, this positive correlation between serum fractalkine level and UAE could be an early predictor of microvascular complications in diabetic patients. We can conclude that serum fractalkine plays a pathogenic role in the development of diabetic nephropathy.

Cobrotoxin from Naja naja atra Venom Ameliorates Adriamycin Nephropathy in Rats

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Shu-Zhi Wang

2015-01-01

Full Text Available Chronic kidney disease (CKD becomes a global health problem with high morbidity and mortality. Adriamycin- (ADR- induced rodent chronic nephropathy is a classic experimental model of human minimal lesion nephrotic syndrome. The present study investigated the effect of cobrotoxin (CTX on ADR-induced nephropathy. Rats were given 6 mg/kg ADR once through the tail vein to replicate ADR nephropathy model. CTX was administered to rats daily by placing a fast dissolving CTX membrane strip under the tongue starting from 5 days prior to ADR administration until the end of experiment. The results showed that CTX ameliorated the symptoms of ADR nephropathy syndrome with reduced body weight loss, proteinuria, hypoalbuminemia, dyslipidemia, serum electrolyte imbalance, oxidative stress, renal function abnormities, and kidney pathological lesions. Anti-inflammatory cytokine IL-10 expression was elevated after CTX administration in ADR nephropathy model. CTX inhibited the phosphorylation of IκB-α and NF-κB p65 nuclear translocation. Meanwhile, CTX upregulated the protein level of podocyte-specific nephrin and downregulated the level of fibrosis-related TGF-β. These findings suggest that CTX may be a potential drug for chronic kidney diseases.

Lithium clearance in chronic nephropathy

DEFF Research Database (Denmark)

Kamper, A L; Holstein-Rathlou, N H; Leyssac, P P

1989-01-01

1. Lithium clearance measurements were made in 72 patients with chronic nephropathy of different aetiology and moderate to severely reduced renal function. 2. Lithium clearance was strictly correlated with glomerular filtration rate, and there was no suggestion of distal tubular reabsorption...... of lithium or influence of osmotic diuresis. 3. Fractional reabsorption of lithium was reduced in most patients with glomerular filtration rates below 25 ml/min. 4. Calculated fractional distal reabsorption of sodium was reduced in most patients with glomerular filtration rates below 50 ml/min. 5. Lithium...... that lithium clearance may be a measure of the delivery of sodium and water from the renal proximal tubule. With this assumption it was found that adjustment of the sodium excretion in chronic nephropathy initially takes place in the distal parts of the nephron (loop of Henle, distal tubule and collecting duct...

Improved prognosis in type 1 diabetic patients with nephropathy

DEFF Research Database (Denmark)

Astrup, Anne Sofie; Tarnow, Lise; Rossing, Peter

2005-01-01

and aspirin were not prescribed routinely until April 2002. The primary end point was cardiovascular mortality and morbidity. Secondary end points were all-cause mortality and ESRD. RESULTS: During follow-up, 79 patients (40%) with nephropathy reached the primary end point versus 19 (10%) of normoalbuminuric...... patients, log rank test P blood pressure (1.13; 1.03 to 1.24). In the nephropathy group, 60 patients (30...

Bilateral congenital midureteric strictures associated with multicystic dysplastic kidney and hydronephrosis: evaluation with MR urography

Energy Technology Data Exchange (ETDEWEB)

Grattan-Smith, J.D.; Little, Stephen [Children' s Healthcare of Atlanta, Department of Radiology, Atlanta, GA (United States); Jones, Richard A. [Children' s Healthcare of Atlanta, Department of Radiology, Atlanta, GA (United States); Emory University School of Medicine, Department of Radiology, Atlanta, GA (United States); Kirsch, Andrew J. [Emory University School of Medicine, Department of Pediatric Urology, Atlanta, GA (United States)

2011-01-15

We report a case of bilateral congenital midureteric strictures diagnosed using MR urography. The severity of obstruction differed in the two ureters, resulting in a multicystic dysplastic kidney (MCDK) with an atretic ureter on one side and hydronephrosis that worsened over time due to progressive stenosis on the other. Although midureteric strictures are usually misdiagnosed as ureteropelvic junction (UPJ) or ureterovesical junction (UVJ) obstruction on conventional imaging, MR urography was able to clearly demonstrate both the anatomical and functional abnormalities. Additionally, because of the excellent anatomical resolution, similarities in the underlying pathological lesions could be contrasted with the severity of the pathophysiological impact upon each kidney. (orig.)

Predisposition to essential hypertension and development of diabetic nephropathy in IDDM patients

DEFF Research Database (Denmark)

Fagerudd, J A; Tarnow, L; Jacobsen, P

1998-01-01

Conflicting results have been reported on the relationship between familial predisposition to hypertension and development of diabetic nephropathy in IDDM. In our case-control study, we assessed the prevalence of hypertension among parents of 73 IDDM patients with diabetic nephropathy (DN......+; persistent albuminuria > 200 microg/min or > 300 mg/24 h) and 73 IDDM patients without diabetic nephropathy (DN-; urinary albumin excretion hypertension, defined as antihypertensive therapy or a 24-h ambulatory blood pressure (SpaceLabs 90207) > or = 135/85 mm...... for hypertension than were patients with DN+ and without parental treatment for hypertension (100 vs. 61%; P = 0.034; difference 39% [21-57%]). In conclusion, familial predisposition to essential hypertension increases the risk of diabetic nephropathy and may also contribute to the development of systemic...

The effect of CT urography in the diagnosis of non-calculous urinary obstruction diseases

International Nuclear Information System (INIS)

Zhang Zhuiyang; Shen Yuanliang; Li Xin; Cao Rongmei; Xu Xiqi; Dong Jian; Hua Dongying

2004-01-01

Objective: To evaluate the diagnostic effect of CT urography (CTU) in patients with non-calculous obstructive diseases of urinary tract. Methods: Forty cases with non-calculous urinary obstruction underwent helical CT during nephrographic and excretory phases. Three dimensional CTU was obtained with the reconstruction of excretory phase axial images through the workstation. Results: 24 of 40 cases with benign obstruction had a slight or severe urinary dilation. Among them,18 congenital urinary malformations showed specific findings. Four ureteric inflammations revealed a gradual taper of the lumen just like a beak, and another two prostatic hypertrophy appeared oval impression upon the middle of the posterior bladder. Six intrinsic and 10 extrinsic tumors were seen irregular filling defects of the urinary tracts as well as soft tissue mass surrounding the obstructive site, accompanied by the middle urinary dilation in 12 cases and severe in four. Meanwhile, bilateral ureterohydronephroses were also found in four extrinsic tumors. In six patients with unilateral nonfunctioning urinary tracts, CTU could be imaged with minimum intensity projection (MinIP) and then be merged with contralateral maximum intensity projection (MaxIP). Conclusion: CTU is an effective modality in the evaluation of non-calculous urinary obstruction diseases. (authors)

NEFROPATÍA OBSTRUCTIVA: SU FISIOPATOLOGÍA

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Musso CG

2011-01-01

Full Text Available Obstructive nephropathy is the functional and /or parenchymal renal damage secondary to the urinary tract occlusion at any part of it. The inducing urinary obstruction diseases can vary depending on the patient´s age and gender.There are many renal dysfunction inducing mechanisms involved in this entity: increase in the intra-luminal pressure, ureteral dilatation with ineffective ureteral peristalsis, glomerular ultrafiltration net pressure reduction, intra-renal glomerular blood flux reduction due to vasoconstriction, and local disease of chemotactic substances. Obstructive nephropathy can also lead to hypertension (vasoconstriction-hypervolemia, hyperkalemia, metabolic acidosis (aldosterone resistance, diabetes insipidus (vasopressine resistance.In conclusion, since obstructive nephropathy is a potentially reversible cause of renal dysfunction, it should always be taken into account among the differential diagnosis of renal failure inducing mechanisms.

Protease activated receptor 2 in diabetic nephropathy: a double edged sword

NARCIS (Netherlands)

Waasdorp, Maaike; Duitman, Janwillem; Florquin, Sandrine; Spek, Arnold C.

2017-01-01

Diabetic nephropathy is a major microvascular complication of diabetes mellitus, and the leading cause of end stage renal disease worldwide. The pathogenesis of diabetic nephropathy is complex, making the development of novel treatments that stop or reverse the progression of microalbuminuria into

Mitochondrial Reactive Oxygen Species and Kidney Hypoxia in the Development of Diabetic Nephropathy.

Science.gov (United States)

Schiffer, Tomas A; Friederich-Persson, Malou

2017-01-01

The underlying mechanisms in the development of diabetic nephropathy are currently unclear and likely consist of a series of dynamic events from the early to late stages of the disease. Diabetic nephropathy is currently without curative treatments and it is acknowledged that even the earliest clinical manifestation of nephropathy is preceded by an established morphological renal injury that is in turn preceded by functional and metabolic alterations. An early manifestation of the diabetic kidney is the development of kidney hypoxia that has been acknowledged as a common pathway to nephropathy. There have been reports of altered mitochondrial function in the diabetic kidney such as altered mitophagy, mitochondrial dynamics, uncoupling, and cellular signaling through hypoxia inducible factors and AMP-kinase. These factors are also likely to be intertwined in a complex manner. In this review, we discuss how these pathways are connected to mitochondrial production of reactive oxygen species (ROS) and how they may relate to the development of kidney hypoxia in diabetic nephropathy. From available literature, it is evident that early correction and/or prevention of mitochondrial dysfunction may be pivotal in the prevention and treatment of diabetic nephropathy.

Adaptive changes in renal mitochondrial redox status in diabetic nephropathy

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Putt, David A.; Zhong, Qing; Lash, Lawrence H., E-mail: l.h.lash@wayne.edu

2012-01-15

Nephropathy is a serious and common complication of diabetes. In the streptozotocin (STZ)-treated rat model of diabetes, nephropathy does not typically develop until 30 to 45 days post-injection, although hyperglycemia occurs within 24 h. We tested the hypothesis that chronic hyperglycemia results in a modest degree of oxidative stress that is accompanied by compensatory changes in certain antioxidants and mitochondrial redox status. We propose that as kidneys progress to a state of diabetic nephropathy, further adaptations occur in mitochondrial redox status. Basic parameters of renal function in vivo and several parameters of mitochondrial function and glutathione (GSH) and redox status in isolated renal cortical mitochondria from STZ-treated and age-matched control rats were examined at 30 days and 90 days post-injection. While there was no effect of diabetes on blood urea nitrogen, measurement of other, more sensitive parameters, such as urinary albumin and protein, and histopathology showed significant and progressive worsening in diabetic rats. Thus, renal function is compromised even prior to the onset of frank nephropathy. Changes in mitochondrial respiration and enzyme activities indicated existence of a hypermetabolic state. Higher mitochondrial GSH content and rates of GSH transport into mitochondria in kidneys from diabetic rats were only partially due to changes in expression of mitochondrial GSH carriers and were mostly due to higher substrate supply. Although there are few clear indicators of oxidative stress, there are several redox changes that occur early and change further as nephropathy progresses, highlighting the complexity of the disease. Highlights: ►Adaptive changes in renal mitochondrial and redox status in diabetic rats. ►Modest renal dysfunction even prior to onset of nephropathy. ►Elevated concentrations of mitochondrial GSH in diabetic kidneys. ►Change in GSH due partly to increased protein expression of transporter. �

Adaptive changes in renal mitochondrial redox status in diabetic nephropathy

International Nuclear Information System (INIS)

Putt, David A.; Zhong, Qing; Lash, Lawrence H.

2012-01-01

Nephropathy is a serious and common complication of diabetes. In the streptozotocin (STZ)-treated rat model of diabetes, nephropathy does not typically develop until 30 to 45 days post-injection, although hyperglycemia occurs within 24 h. We tested the hypothesis that chronic hyperglycemia results in a modest degree of oxidative stress that is accompanied by compensatory changes in certain antioxidants and mitochondrial redox status. We propose that as kidneys progress to a state of diabetic nephropathy, further adaptations occur in mitochondrial redox status. Basic parameters of renal function in vivo and several parameters of mitochondrial function and glutathione (GSH) and redox status in isolated renal cortical mitochondria from STZ-treated and age-matched control rats were examined at 30 days and 90 days post-injection. While there was no effect of diabetes on blood urea nitrogen, measurement of other, more sensitive parameters, such as urinary albumin and protein, and histopathology showed significant and progressive worsening in diabetic rats. Thus, renal function is compromised even prior to the onset of frank nephropathy. Changes in mitochondrial respiration and enzyme activities indicated existence of a hypermetabolic state. Higher mitochondrial GSH content and rates of GSH transport into mitochondria in kidneys from diabetic rats were only partially due to changes in expression of mitochondrial GSH carriers and were mostly due to higher substrate supply. Although there are few clear indicators of oxidative stress, there are several redox changes that occur early and change further as nephropathy progresses, highlighting the complexity of the disease. Highlights: ►Adaptive changes in renal mitochondrial and redox status in diabetic rats. ►Modest renal dysfunction even prior to onset of nephropathy. ►Elevated concentrations of mitochondrial GSH in diabetic kidneys. ►Change in GSH due partly to increased protein expression of transporter. �

Japan Diabetic Nephropathy Cohort Study: study design, methods, and implementation.

Science.gov (United States)

Furuichi, Kengo; Shimizu, Miho; Toyama, Tadashi; Koya, Daisuke; Koshino, Yoshitaka; Abe, Hideharu; Mori, Kiyoshi; Satoh, Hiroaki; Imanishi, Masahito; Iwano, Masayuki; Yamauchi, Hiroyuki; Kusano, Eiji; Fujimoto, Shouichi; Suzuki, Yoshiki; Okuda, Seiya; Kitagawa, Kiyoki; Iwata, Yasunori; Kaneko, Shuichi; Nishi, Shinichi; Yokoyama, Hitoshi; Ueda, Yoshihiko; Haneda, Masakazu; Makino, Hirofumi; Wada, Takashi

2013-12-01

Diabetic nephropathy, leading to end-stage renal disease, has a considerable impact on public health and the social economy. However, there are few national registries of diabetic nephropathy in Japan. The aims of this prospective cohort study are to obtain clinical data and urine samples for revising the clinical staging of diabetic nephropathy, and developing new diagnostic markers for early diabetic nephropathy. The Japanese Society of Nephrology established a nationwide, web-based, and prospective registry system. On the system, there are two basic registries; the Japan Renal Biopsy Registry (JRBR), and the Japan Kidney Disease Registry (JKDR). In addition to the two basic registries, we established a new prospective registry to the system; the Japan Diabetic Nephropathy Cohort Study (JDNCS), which collected physical and laboratory data. We analyzed the data of 321 participants (106 female, 215 male; average age 65 years) in the JDNCS. Systolic and diastolic blood pressure was 130.1 and 72.3 mmHg, respectively. Median estimated glomerular filtration rate (eGFR) was 33.3 ml/min/1.73 m(2). Proteinuria was 1.8 g/gCr, and serum levels of albumin were 3.6 g/dl. The majority of the JDNCS patients presented with preserved eGFR and low albuminuria or low eGFR and advanced proteinuria. In the JRBR and JKDR registries, 484 and 125 participants, respectively, were enrolled as having diabetes mellitus. In comparison with the JRBR and JKDR registries, the JDNCS was characterized by diabetic patients presenting with low proteinuria with moderately preserved eGFR. There are few national registries of diabetic nephropathy to evaluate prognosis in Japan. Future analysis of the JDNCS will provide clinical insights into the epidemiology and renal and cardiovascular outcomes of type 2 diabetic patients in Japan.

Polybezoar in a child with Down's Syndrome after corrective surgery of congenital duodenal stenosis

Energy Technology Data Exchange (ETDEWEB)

Arlart, I

1980-11-01

Case report of a mongoloid child (Trisomie 21), age 9, operated on in his neonatal period for congenital stenosis of the duodenum; now presenting with acute clinical signs of gastric outlet obstruction and increasing weight loss. Radiologic work-up showed this to be due to a large gastric and duodenal polybezoar, claimed to be due to emotional disturbance as part of this patients primary disease.

Polybezoar in a child with Down's Syndrome after corrective surgery of congenital duodenal stenosis

International Nuclear Information System (INIS)

Arlart, I.

1980-01-01

Case report of a mongoloid child (Trisomie 21), age 9, operated on in his neonatal period for congenital stenosis of the duodenum; now presenting with acute clinical signs of gastric outlet obstruction and increasing weight loss. Radiologic work-up showed this to be due to a large gastric and duodenal polybezoar, claimed to be due to emotional disturbance as part of this patients primary disease. (orig.) [de

Diabetic nephropathy: Time to withhold development and progression - A review

Directory of Open Access Journals (Sweden)

Usama A.A. Sharaf El Din

2017-07-01

Full Text Available The recent discoveries in the fields of pathogenesis and management of diabetic nephropathy have revolutionized the knowledge about this disease. Little was added to the management of diabetic nephropathy after the introduction of renin angiotensin system blockers. The ineffective role of the renin- angiotensin system blockers in primary prevention of diabetic nephropathy in type 1 diabetes mellitus necessitated the search for other early therapeutic interventions that target alternative pathogenic mechanisms. Among the different classes of oral hypoglycemic agents, recent studies highlighted the distinguished mechanisms of sodium glucose transporter 2 blockers and dipeptidyl peptidase-4 inhibitors that settle their renoprotective actions beyond the hypoglycemic effects. The introduction of antioxidant and anti-inflammatory agents to this field had also added wealth of knowledge. However, many of these agents are still waiting well-designed clinical studies in order to prove their beneficial therapeutic role. The aim of this review of literature is to highlight the recent advances in understanding the pathogenesis, diagnosis, the established and the potential renoprotective therapeutic agents that would prevent the development or the progression of diabetic nephropathy.

Clinical significance of determination of plasma endothelin (ET) and homocysteine (Hcy) levels in patients with diabetic nephropathy

International Nuclear Information System (INIS)

Zhang Aimin; Jin Ying; Zhou Xiu

2005-01-01

Objective: To determine the plasma levels of endothelin (ET) and homocysteine (Hcy) in patients with diabetic nephropathy. Methods: Plasma ET (with RIA) and Hcy( with electrochemiluminescence) contents were determined in 32 DM2 patients without nephropathy, 35 DM2 patients with nephropathy and 30 controls. Results: Endothelin and homocysteine levels were significantly higher in patients with diabetic nephropathy than those in patients without nephropathy and controls (P<0.05- 0.01). Conclusion: Endothelin and homocysteine were involved in the pathogenesis of diabetic nephropathy, and determination of which were of diagnostic and prognostic value in clinical practice. (authors)

Gene Expression Analysis in Tubule Interstitial Compartments Reveals Candidate Agents for IgA Nephropathy

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Jinling Wang

2014-09-01

Full Text Available Background/Aims: Our aim was to explore the molecular mechanism underlying development of IgA nephropathy and discover candidate agents for IgA nephropathy. Methods: The differentially expressed genes (DEGs between patients with IgA nephropathy and normal controls were identified by the data of GSE35488 downloaded from GEO (Gene Expression Omnibus database. The co-expressed gene pairs among DEGs were screened to construct the gene-gene interaction network. Gene Ontology (GO enrichment analysis was performed to analyze the functions of DEGs. The biologically active small molecules capable of targeting IgA nephropathy were identified using the Connectivity Map (cMap database. Results: A total of 55 genes involved in response to organic substance, transcription factor activity and response to steroid hormone stimulus were identified to be differentially expressed in IgA nephropathy patients compared to healthy individuals. A network with 45 co-expressed gene pairs was constructed. DEGs in the network were significantly enriched in response to organic substance. Additionally, a group of small molecules were identified, such as doxorubicin and thapsigargin. Conclusion: Our work provided a systematic insight in understanding the mechanism of IgA nephropathy. Small molecules such as thapsigargin might be potential candidate agents for the treatment of IgA nephropathy.

A review of congenital lung malformations with a simplified classification system for clinical and research use.

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Seear, Michael; Townsend, Jennifer; Hoepker, Amy; Jamieson, Douglas; McFadden, Deborah; Daigneault, Patrick; Glomb, William

2017-06-01

Congenital lung abnormalities are rare malformations increasingly detected early by prenatal ultrasound. Whether management of these frequently asymptomatic lesions should be surgical or conservative is an unresolved issue. The necessary prospective studies are limited by the absence of a widely accepted practical classification system. Our aim was to develop a simple, clinically relevant system for classifying and studying congenital lung abnormalities. We based our proposed grouping on a detailed analysis of clinical, radiological, and histological data from well-documented cases, plus an extensive review of the literature. The existence of hybrid lesions and common histological findings suggested a unified embryological mechanism-possibly obstruction of developing airways with distal dysplasia. Malformations could be classified by their anatomical and pathological findings; however, a system based on the prenatal ultrasound plus initial chest X-ray findings had greater clinical relevance: Group 1-Congenital solid/cystic lung malformation, Group 2-Congenital hyperlucent lobe, Group 3-Congenital small lung. Pathological classification is academically important but is unnecessarily complex for clinical and research use. Our simple radiological-based system allows unambiguous comparison between the results of different studies and also guides the choice of necessary investigations specific to each group.

A proton nuclear magnetic resonance-based metabonomics study of metabolic profiling in immunoglobulin a nephropathy

International Nuclear Information System (INIS)

Sui, Weiguo; Che, Wenti; Guimai, Zuo; Chen, Jiejing; Li, Liping; Li, Wuxian; Dai, Yong

2012-01-01

Objectives: Immunoglobulin A nephropathy is the most common cause of chronic renal failure among primary glomerulonephritis patients. The ability to diagnose immunoglobulin A nephropathy remains poor. However, renal biopsy is an inconvenient, invasive, and painful examination, and no reliable biomarkers have been developed for use in routine patient evaluations. The aims of the present study were to identify immunoglobulin A nephropathy patients, to identify useful biomarkers of immunoglobulin A nephropathy and to establish a human immunoglobulin A nephropathy metabolic profile. Methods: Serum samples were collected from immunoglobulin A nephropathy patients who were not using immunosuppressants. A pilot study was undertaken to determine disease-specific metabolite biomarker profiles in three groups: healthy controls (N = 23), low-risk patients in whom immunoglobulin A nephropathy was confirmed as grades I-II by renal biopsy (N = 23), and high-risk patients with nephropathies of grades IV-V (N = 12). Serum samples were analyzed using proton nuclear magnetic resonance spectroscopy and by applying multivariate pattern recognition analysis for disease classification. Results: Compared with the healthy controls, both the low-risk and high-risk patients had higher levels of phenylalanine, myo-inositol, lactate, L6 lipids ( CH-CH 2 -CH = O), L5 lipids (-CH 2 -C = O), and L3 lipids (-CH 2 -CH 2 -C = O) as well as lower levels of β-glucose, α-glucose, valine, tyrosine, phosphocholine, lysine, isoleucine, glycerolphosphocholine, glycine, glutamine, glutamate, alanine, acetate, 3-hydroxybutyrate, and 1-methylhistidine. Conclusions: These metabolites investigated in this study may serve as potential biomarkers of immunoglobulin A nephropathy. Point scoring of pattern recognition analysis was able to distinguish immunoglobulin A nephropathy patients from healthy controls. However, there were no obvious differences between the low-risk and high-risk groups in our research

A proton nuclear magnetic resonance-based metabonomics study of metabolic profiling in immunoglobulin a nephropathy

Energy Technology Data Exchange (ETDEWEB)

Sui, Weiguo; Che, Wenti; Guimai, Zuo; Chen, Jiejing [181st Hospital Guangxi, Central Laboratory, Laboratory of Metabolic Diseases Research, Guangxi Province (China); Li, Liping [Guangxi Normal University, The Life Science College, Guangxi Province (China); Li, Wuxian [Key Laboratory of Laboratory Medical Diagnostics of Education Ministry, Chongqiong Medical University, Chongqing (China); Dai, Yong [Clinical Medical Research Center, the Second Clinical Medical College of Jinan University (Shenzhen People' s Hospital), Shenzhen, Guangdong Province (China)

2012-07-01

Objectives: Immunoglobulin A nephropathy is the most common cause of chronic renal failure among primary glomerulonephritis patients. The ability to diagnose immunoglobulin A nephropathy remains poor. However, renal biopsy is an inconvenient, invasive, and painful examination, and no reliable biomarkers have been developed for use in routine patient evaluations. The aims of the present study were to identify immunoglobulin A nephropathy patients, to identify useful biomarkers of immunoglobulin A nephropathy and to establish a human immunoglobulin A nephropathy metabolic profile. Methods: Serum samples were collected from immunoglobulin A nephropathy patients who were not using immunosuppressants. A pilot study was undertaken to determine disease-specific metabolite biomarker profiles in three groups: healthy controls (N = 23), low-risk patients in whom immunoglobulin A nephropathy was confirmed as grades I-II by renal biopsy (N = 23), and high-risk patients with nephropathies of grades IV-V (N = 12). Serum samples were analyzed using proton nuclear magnetic resonance spectroscopy and by applying multivariate pattern recognition analysis for disease classification. Results: Compared with the healthy controls, both the low-risk and high-risk patients had higher levels of phenylalanine, myo-inositol, lactate, L6 lipids ( CH-CH{sub 2}-CH = O), L5 lipids (-CH{sub 2}-C = O), and L3 lipids (-CH{sub 2}-CH{sub 2}-C = O) as well as lower levels of {beta}-glucose, {alpha}-glucose, valine, tyrosine, phosphocholine, lysine, isoleucine, glycerolphosphocholine, glycine, glutamine, glutamate, alanine, acetate, 3-hydroxybutyrate, and 1-methylhistidine. Conclusions: These metabolites investigated in this study may serve as potential biomarkers of immunoglobulin A nephropathy. Point scoring of pattern recognition analysis was able to distinguish immunoglobulin A nephropathy patients from healthy controls. However, there were no obvious differences between the low-risk and high

Determinants of Intravascular Resistance in Indian Diabetic Nephropathy Patients: A Hospital-Based Study

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Anubhav Thukral

2011-01-01

Full Text Available Aims and Objectives. Metabolic dysregulation has failed to explain clinical variability of patients with diabetic nephropathy and hence a renewed interest emerged in haemodynamic factors as determinant of progression and development of diabetic nephropathy. We therefore studied for various factors which can correlate with raised renal vascular resistance in diabetic nephropathy. Material and Methods. Renal vascular resistance was measured in patients with established and incipient diabetic nephropathy and compared with controls using noninvasive color Doppler examinations of intrarenal vasculature. Results. Renal vascular resistance correlated with age, duration of disease, GFR, serum creatinine, and stage of retinopathy. Renal vascular resistance was significantly reduced in patients on treatment with RAAS inhibitors and insulin, than those on OHA and antihypertensives other than RAAS inhibitors. Conclusion. The study implies that renal vascular resistance may help identify diabetics at high risk of developing nephropathy, and these set of patients could be candidates for RAAS inhibition and early insulin therapy even in patients without albuminuria.

Contrast-induced nephropathy: risks, pathogenetic, prevention

International Nuclear Information System (INIS)

Paskalev, D.; Balev, B.

2006-01-01

Full text: The aim of the presentation is to review the contrast induced nephropathy ? nature, mechanisms of development, risk factors. Summary of the most important ways of prevention, diagnostics and treatment. The definition of CIN according the European Association of Urogenital Radiology is: 'A condition, in which renal function is impaired (elevation of serum creatinine with more than 25% or 44 μmol/l above the initial level) due to intravasal application of contrast media (CM) within 3 days following the application and when no other etiology factors are present'. We summarize the main risk factors of developing CIN - renal failure, diabetic nephropathy, dehydration, congestive heart failure, high blood pressure, age above 70 yrs, nephrotoxic medicines. The most effective ways of preventing CIN are the good hydratation of the patients and the usage of low-osmolar or iso-osmolar CM. Therapeutic treatment is with no proven preventive effect and currently is not routinely recommended. An early hem dialysis does not decrease the risk level of CIN development in patients with chronic renal failure (CRF). In such patients complete elimination of CM is achieved only after several hem dialyses. Hem filtration reliably decreases the risk of CIN in CRF patients, but is expensive and not widely available. We present a case from our hospital of a patient with diabetic nephropathy, who developed CIN following a coronary angiography

Silica Nephropathy

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N Ghahramani

2010-06-01

Full Text Available Occupational exposure to heavy metals, organic solvents and silica is associated with a variety of renal manifestations. Improved understanding of occupational renal disease provides insight into environmental renal disease, improving knowledge of disease pathogenesis. Silica (SiO2 is an abundant mineral found in sand, rock, and soil. Workers exposed to silica include sandblasters, miners, quarry workers, masons, ceramic workers and glass manufacturers. New cases of silicosis per year have been estimated in the US to be 3600–7300. Exposure to silica has been associated with tubulointerstitial disease, immune-mediated multisystem disease, chronic kidney disease and end-stage renal disease. A rare syndrome of painful, nodular skin lesions has been described in dialysis patients with excessive levels of silicon. Balkan endemic nephropathy is postulated to be due to chronic intoxication with drinking water polluted by silicates released during soil erosion. The mechanism of silica nephrotoxicity is thought to be through direct nephrotoxicity, as well as silica-induced autoimmune diseases such as scleroderma and systemic lupus erythematosus. The renal histopathology varies from focal to crescentic and necrotizing glomerulonephritis with aneurysm formation suggestive of polyarteritis nodosa. The treatment for silica nephrotoxicity is non-specific and depends on the mechanism and stage of the disease. It is quite clear that further research is needed, particularly to elucidate the pathogenesis of silica nephropathy. Considering the importance of diagnosing exposure-related renal disease at early stages, it is imperative to obtain a thorough occupational history in all patients with renal disease, with particular emphasis on exposure to silica, heavy metals, and solvents.

Cystatin-C and TGF-β levels in patients with diabetic nephropathy

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Mumtaz Takir

2016-11-01

Conclusions: Although urinary albumin excretion is recommended for the detection of type two diabetic nephropathy, there is a group of patients with decreased eGFR but without increased urinary albumin excretion, in which serum cystatin C level was indicated to be used as an early biomarker of diabetic nephropathy.

Metabolic nephropathies in children: Causes, clinical and laboratory manifestations

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E. A. Yuryeva

2016-01-01

Full Text Available In the regions polluted with industrial or agricultural toxicants, dysmetabolic nephropathy is detected in every 2 or 3 children and this rate increases with age. Exogenous intoxication is not the only cause of dysmetabolic nephropathy; of no less importance are endogenous toxicants, such as oxidative stress products, excess of usual metabolites or emergence of unusual products of abnormal metabolism. The toxicants are ascertained to be able to change the conformation of protein molecules to give rise to additional ligand loci ensuring the aggressive uptake of trace elements that fix changes in protein molecules, making them antigenically alien to the body. Low molecular weight proteins with their changed structure, which penetrate through the basement membrane, are unrecognized by the reabsorption systems of proximal tubules and excreted with urine, determining the appearance of the most steady and age-increasing sign of dysmetabolic nephropathy – microproteinuria or trace elementuria.

Tc-99m DTPA and Tc-99m DMSA renal scan findings in patients with congenital megacalyces and megaureter without urinary tract obstruction

International Nuclear Information System (INIS)

Ahn, Byeong Cheol; Bae, Jin Ho; Jeong, Sin Young; Lee, Jae Tae; Lee, Kyu Bo

2003-01-01

A 10 days old male infant with congenital megacalyces and megaureter, diagnosed by prenatal ultrasonographic screening, underwent Tc-99m DTPA renal scan for evaluation of urinary tract patency, Tc-99m DMSA scan for evaluation of renal cortical damage. He also underwent intravenous urography(IVU) and renal ultrasonography. Tc-99m DTPA renal scan demonstrates intense tracer accumulation in enlarged both renal pelvocalyses and ureters, which rapidly washout diuretics administration. Tc-99m DMSA renal cortical scan shows no remarkable photon defect in both renal cortices and visible tracer uptake in both megaureter areas. Ultasonographic and IVU studies show enlarged both renal calyses and bullously dilated ureters, but no dilatation in renal pelvis. Follow up Tc-99m DTPA renal scan, performed at one year later, also reveals intense tracer accumulation in enlarged both urinary tracts which rapidly washout without diuretics, and shows no significant change compare to the previous Tc-99m DTPA renal scan. Urinary tract obstruction and renal cortical damage can be easily evaluated with Tc-99m DTPA and Tc-99m DMSA scans in patients with megacalyces and megaureter

Tc-99m DTPA and Tc-99m DMSA renal scan findings in patients with congenital megacalyces and megaureter without urinary tract obstruction

Energy Technology Data Exchange (ETDEWEB)

Ahn, Byeong Cheol; Bae, Jin Ho; Jeong, Sin Young; Lee, Jae Tae; Lee, Kyu Bo [Kyungpook National University Medical School, Daegu (Korea, Republic of)

2003-06-01

A 10 days old male infant with congenital megacalyces and megaureter, diagnosed by prenatal ultrasonographic screening, underwent Tc-99m DTPA renal scan for evaluation of urinary tract patency, Tc-99m DMSA scan for evaluation of renal cortical damage. He also underwent intravenous urography(IVU) and renal ultrasonography. Tc-99m DTPA renal scan demonstrates intense tracer accumulation in enlarged both renal pelvocalyses and ureters, which rapidly washout diuretics administration. Tc-99m DMSA renal cortical scan shows no remarkable photon defect in both renal cortices and visible tracer uptake in both megaureter areas. Ultasonographic and IVU studies show enlarged both renal calyses and bullously dilated ureters, but no dilatation in renal pelvis. Follow up Tc-99m DTPA renal scan, performed at one year later, also reveals intense tracer accumulation in enlarged both urinary tracts which rapidly washout without diuretics, and shows no significant change compare to the previous Tc-99m DTPA renal scan. Urinary tract obstruction and renal cortical damage can be easily evaluated with Tc-99m DTPA and Tc-99m DMSA scans in patients with megacalyces and megaureter.

IgM nephropathy; can we still ignore it.

Science.gov (United States)

Vanikar, Aruna

2013-04-01

IgM nephropathy (IgMN) is a relatively less recognized clinico-immunopathological entity in the domain of glomerulonephritis , often thought to be a bridge between minimal change disease and focal segmental glomerulosclerosis. Directory of Open Access Journals (DOAJ), Google Scholar, Pubmed (NLM), LISTA (EBSCO) and Web of Science has been searched. IgM nephropathy can present as nephritic syndrome or less commonly with subnephrotic proteinuria or rarely hematuria. About 30% patients respond to steroids whereas others are steroid dependent / resistant. They should be given a trial of Rituximab or stem cell therapy. IgM nephropathy (IgMN) is an important and rather neglected pathology responsible for renal morbidity in children and adults in developing countries as compared to developed nations with incidence of 2-18.5% of native biopsies. Abnormal T-cell function with hyperfunctioning suppressor T-cells are believed to be responsible for this disease entity. Approximately one third of the patients are steroid responders where as the remaining two thirds are steroid resistant or dependent. Therapeutic trials including cell therapies targeting suppressor T-cells are required.

Prevention of contrast-induced nephropathy by use of bicarbonate solution: preliminary results and literature review.

Science.gov (United States)

Silva, Ricardo Gonçalves da; Silva, Nelson Gonçalves da; Lucchesi, Fabiano; Burdmann, Emmanuel A

2010-01-01

The incidence of contrast-induced nephropathy has increased simultaneously with the increase in contrast medium use in diagnostic and interventional procedures. The incidence of contrast-induced nephropathy in the general population is low, but increases exponentially in patients with risk factors, such as diabetes and chronic kidney disease. Several strategies have been used in order to prevent contrast-induced nephropathy. The most efficient strategies are saline hydration (0.9% or 0.45%), use of low-or iso-osmolality contrast medium, and sodium bicarbonate infusion. The aim of this study was to review the pertinent literature and to assess the efficacy of hydration with 1.3% sodium bicarbonate compared with hydration with 0.9% saline solution in preventing contrast-induced nephropathy in high-risk patients. A systematic search of the literature was conducted in PubMed by using the following keywords: bicarbonate, nephropathy, contrast medium, and acute kidney failure. In addition, 27 patients with diabetes and/or chronic kidney disease, diagnosed with some kind of cancer were randomized for study. None of the patients developed contrast-induced nephropathy characterized as a 0.5 mg/ dL-increase and/or a relative 25%-increase in baseline creatinine. The literature review strongly suggested that sodium bicarbonate is effective in preventing contrast-induced nephropathy. Regarding the randomized study, saline solution and bicarbonate solution had similar efficacy in preventing contrast-induced nephropathy. However, the small number of patients does not allow definite conclusions.

CT manifestation of congenital toxoplasmosis infection of the brain (report of 42 cases)

International Nuclear Information System (INIS)

Wang Zhenyu; Li Shuxin; Feng Kun

1997-01-01

To improve the recognition and diagnosis of congenital toxoplasmosis infection of the brain, forty-two cases of congenital toxoplasmosis infection of the brain verified by serological tests and initially investigated by CT were retrospectively studied. The main diagnostic feature of the entity included: (1) Widely scattered small nodular or curvilinear calcifications involving the basal ganglia, subependymal region and the frontal or parietal lobes; (2) Small patches of low density foci located at the paraventricular and gray-white matter junction area with some enhancement surrounding the foci after contrast media administration; (3) Evidence of obstructive hydrocephalus and (4) Complications of CNS malformation or developmental problems. Conclusion: CT was one of the best methods for the diagnosis of this entity, however, it should be closely correlated with the results from serological tests

Familial juvenile hyperuricemic nephropathy : report on a new mutation and a pregnancy

NARCIS (Netherlands)

Lhotta, Karl; Gehringer, A; Jennings, P; Kronenberg, F.; Brezinka, C; Andersone, I; Strazdins, V

BACKGROUND: Familial juvenile hyperuricemic nephropathy (FJHN) is a rare autosomal dominant disease caused by mutations in the uromodulin gene (UMOD) and leading to gout, tubulointerstitial nephropathy and end-stage renal disease. CASE REPORTS AND RESULTS: A Latvian family suffering from FJHN is

A story of microalbuminuria and diabetic nephropathy.

Science.gov (United States)

Roshan, Bijan; Stanton, Robert C

2013-10-01

It is estimated that more than 346 million people worldwide have diabetes mellitus . By the year 2030, it is predicted that diabetes will become the seventh leading cause of death in the world. Development of chronic kidney disease (CKD) in patients with diabetes adds significantly to the morbidity and mortality and significantly increases health care costs, even before the development of end stage renal disease (ESRD). Evidence  acquisitions: Directory of Open Access Journals (DOAJ), Google Scholar, Pubmed (NLM), LISTA (EBSCO) and Web of Science have been searched. Diabetic nephropathy (DN) is increasing rapidly worldwide. It is the leading cause of new cases of ESRD in the USA.  Interestingly, although DN is the most common cause of ESRD in diabetic patients, diabetes mellitus is also an independent and strong risk factor for ESRD ascribed to causes other than DN (e.g. hypertensive nephropathy). It is important to be aware of the pitfalls of using the urine albumin level in predicting development and progression of diabetic nephropathy in order to treat and advise the patients accurately.  Research into finding new markers is rapidly evolving but current progress makes it likely we will be using the urine albumin level for some years into the future.

Nephropathy in type 1 diabetes is associated with increased circulating activated platelets and platelet hyperreactivity

DEFF Research Database (Denmark)

Tarnow, Inge; Michelson, Alan D.; Barnard, Marc R.

2009-01-01

Patients with diabetes mellitus (DM) have increased platelet activation compared to non-diabetic controls. Platelet hyperreactivity has been associated with adverse cardiovascular outcomes in Type 2 DM, and with diabetic nephropathy. We investigated the relationship between platelet activation...... and nephropathy in Type 1 DM. Patients with Type 1 DM and diabetic nephropathy (n = 35), age- and sex-matched Type 1 DM patients with persistent normoalbuminuria (n = 51), and healthy age- and sex-matched controls (n = 30) were studied. Platelet surface P-selectin, platelet surface activated GPIIb/IIIa, monocyte...... controls (P = 0.0075). There were no differences between groups in activated GPIIb/IIIa or in response to TRAP at any end-point. More patients with nephropathy received aspirin (71.4%) compared to normoalbuminuric patients (27.4%) (P Type 1 diabetic nephropathy, as compared with normoalbuminuria...

Subclinical hypothyroidism and diabetic nephropathy in Iranian patients with type 2 diabetes.

Science.gov (United States)

Mansournia, N; Riyahi, S; Tofangchiha, S; Mansournia, M A; Riahi, M; Heidari, Z; Hazrati, E

2017-03-01

Association of subclinical hypothyroidism with type 2 diabetes and its complications has been previously documented. These reports were, however, inconclusive and mainly gathered from Chinese and East Asian populations. In this study, we aimed to determine the prevalence of subclinical hypothyroidism and its relationship with diabetic nephropathy in Iranian individuals with type 2 diabetes, drawn from a white Middle Eastern population with an increasing prevalence of diabetes. In this cross-sectional study, 255 Iranian participants with type 2 diabetes and without history of thyroid disorders were included. Patients with TSH > 4.2 mIU/L and normal T4 were classified as having subclinical hypothyroidism. Diabetic nephropathy was diagnosed based on abnormal 24-h urinary albumin or protein measurements (24-h urinary albumin ≥30 mg/day or 24-h urinary protein ≥150 mg/day). Multivariate logistic regression was employed to obtain the OR for the relationship between subclinical hypothyroidism and diabetic nephropathy. We found that subclinical hypothyroidism and diabetic nephropathy were as prevalent as 18.1 and 41.2 %, respectively, among the participants. We also found that subclinical hypothyroidism was independently associated with higher rates of diabetic nephropathy, after multivariable adjustment (OR [95 % CI] 3.23 [1.42-7.37], p = 0.005). We found that the prevalence of subclinical hypothyroidism in Iranian diabetic population was among the highest rates reported to date. Our data supported the independent association of subclinical hypothyroidism with diabetic nephropathy, calling for further investigations to evaluate their longitudinal associations.

Prognosis and treatment of diabetic nephropathy

DEFF Research Database (Denmark)

Rossing, Peter; Persson, Frederik; Frimodt-Møller, Marie

2018-01-01

cardiovascular morbidity and mortality. The natural course of classical diabetic nephropathy is initially microalbuminuria or moderately increased urine albumin excretion (30-300mg/g creatinine). Untreated microalbuminuria may then rise gradually, reaching severely increased albuminuric (macroalbuminuria) over 5...

Diabetic Nephropathy : Evaluation with Doppler Ultrasonography

International Nuclear Information System (INIS)

Sim, Jung Suk; Kim, Seung Hyup; Kang, Heung Sik; Park, Jae Hyung; Han, Man Chung

1996-01-01

To compare Doppler ultrasonography with laboratory tests in evaluation of diabetic nephropathy. Fifty-five patients (mean age = 60, M : F = 26 : 29) with diabetes mellitus underwent renal Doppler ultrasonography. Resistive indices were compared with degree of proteinuria, serum creatinine level, and creatinine clearance rate. Eighteen patients who showed no proteinuria or microscopic proteinuria had a mean resistive index (RI) of 0.72 (SD, 0.05), 16 patients with macroscopic proteinuria without nephrotic syndrome had a mean RI of 0.82 (SD, 0.13), and 21 patients with nephrotic syndrome had a mean RI of 0.90 (SD, 0.12). Renal RI correlated highly with serum creatinine level (r = 0.62) and creatinine clearance rate (r = -0.43). Renal Doppler ultrasonography provides a useful indication of renal function in diabetic nephropathy but cannot offer an advantage over conventional laboratory test

Improved prognosis of diabetic nephropathy in type 1 diabetes

DEFF Research Database (Denmark)

Andrésdóttir, Gudbjörg; Jensen, Majken L; Carstensen, Bendix

2015-01-01

previously 4.0 to 3.3 ml/min per 1.73 m2/year. During a median follow-up of 9.1 years, 29% of participants doubled their plasma creatinine or developed end-stage renal disease. Mortality risk was similar to our prior study (hazard ratio 1.05 (0.76-1.43). However, after age adjustment, as both diabetes......-term renin-angiotensin system inhibition), lipids, and glycemia, along with less smoking and other lifestyle and treatment advancements, is inadequately analyzed. To clarify this, we studied 497 patients with type 1 diabetes and diabetic nephropathy at the Steno Diabetes Center and compared them...... and nephropathy onset occurred later in life, mortality was reduced by 30%. Risk factors for decline in glomerular filtration rate, death, and other renal end points were generally in agreement with prior studies. Thus, with current treatment of nephropathy in type 1 diabetes, the prognosis and loss of renal...

Oxidative Stress/Angiotensinogen/Renin-Angiotensin System Axis in Patients with Diabetic Nephropathy

Directory of Open Access Journals (Sweden)

Masumi Kamiyama

2013-11-01

Full Text Available Although recent studies have proven that renin-angiotensin system (RAS blockades retard the progression of diabetic nephropathy, the detailed mechanisms of their reno-protective effects on the development of diabetic nephropathy remain uncertain. In rodent models, it has been reported that reactive oxygen species (ROS are important for intrarenal angiotensinogen (AGT augmentation in the progression of diabetic nephropathy. However, no direct evidence is available to demonstrate that AGT expression is enhanced in the kidneys of patients with diabetes. To examine whether the expression levels of ROS- and RAS-related factors in kidneys are increased with the progression of diabetic nephropathy, biopsied samples from 8 controls and 27 patients with type 2 diabetes were used. After the biopsy, these patients were diagnosed with minor glomerular abnormality or diabetes mellitus by clinical and pathological findings. The intensities of AGT, angiotensin II (Ang II, 4-hydroxy-2-nonenal (4-HNE, and heme oxygenase-1 (HO-1 were examined by fluorescence in situ hybridization and/or immunohistochemistry. Expression levels were greater in patients with diabetes than in control subjects. Moreover, the augmented intrarenal AGT mRNA expression paralleled renal dysfunction in patients with diabetes. These data suggest the importance of the activated oxidative stress/AGT/RAS axis in the pathogenesis of diabetic nephropathy.

The Oxford classification of IgA nephropathy: rationale, clinicopathological correlations, and classification

NARCIS (Netherlands)

Cattran, Daniel C.; Coppo, Rosanna; Cook, H. Terence; Feehally, John; Roberts, Ian S. D.; Troyanov, Stéphan; Alpers, Charles E.; Amore, Alessandro; Barratt, Jonathan; Berthoux, Francois; Bonsib, Stephen; Bruijn, Jan A.; D'Agati, Vivette; D'Amico, Giuseppe; Emancipator, Steven; Emma, Francesco; Ferrario, Franco; Fervenza, Fernando C.; Florquin, Sandrine; Fogo, Agnes; Geddes, Colin C.; Groene, Hermann-Josef; Haas, Mark; Herzenberg, Andrew M.; Hill, Prue A.; Hogg, Ronald J.; Hsu, Stephen I.; Jennette, J. Charles; Joh, Kensuke; Julian, Bruce A.; Kawamura, Tetsuya; Lai, Fernand M.; Leung, Chi Bon; Li, Lei-Shi; Li, Philip K. T.; Liu, Zhi-Hong; Mackinnon, Bruce; Mezzano, Sergio; Schena, F. Paolo; Tomino, Yasuhiko; Walker, Patrick D.; Wang, Haiyan; Weening, Jan J.; Yoshikawa, Nori; Zhang, Hong

2009-01-01

IgA nephropathy is the most common glomerular disease worldwide, yet there is no international consensus for its pathological or clinical classification. Here a new classification for IgA nephropathy is presented by an international consensus working group. The goal of this new system was to

Anterior urethral valves without diverticulum, a rare cause of infravesical obstruction and vesicoureteral reflux in children: Report of two cases and literature review

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Jyoti Bothra

2017-09-01

Full Text Available Congenital anterior urethral valve is a rare condition causing significant obstructive uropathy in pediatric age group. It is much rarer than posterior urethral valve. However, the clinical course is similar. We present two cases of anterior urethral valves in children

Contrast induced nephropathy

DEFF Research Database (Denmark)

Stacul, Fulvio; van der Molen, Aart J; Reimer, Peter

2011-01-01

PURPOSE: The Contrast Media Safety Committee (CMSC) of the European Society of Urogenital Radiology (ESUR) has updated its 1999 guidelines on contrast medium-induced nephropathy (CIN). AREAS COVERED: Topics reviewed include the definition of CIN, the choice of contrast medium, the prophylactic me....../min/1.73 m (2) is CIN risk threshold for intravenous contrast medium. • Hydration with either saline or sodium bicarbonate reduces CIN incidence. • Patients with eGFR = 60 ml/min/1.73 m (2) receiving contrast medium can continue metformin normally....

Alteration in serum osteocalcin levels in patients with diabetic nephropathy

International Nuclear Information System (INIS)

Salem, E.S.; Abdel-Messeih, Ph.L.; Mansour, H.H.

2013-01-01

The fact that bone mass density (BMD) is not useful for assessing fracture risk in diabetic patients (DM) seems problematic, because those populations are increasing in every country. Osteocalcin (OC) is synthesized by osteoblasts and is considered to be a marker of bone formation. The present study was carried out to evaluate the usefulness of OC as noninvasive biomarker of bone formation in diabetes mellitus type 2 (uncomplicated) and diabetic nephropathy. Immunoradiometric assay(IRMA) was used for the quantitative measurement of human intact OC both N-terminal and C-terminal fragments in the serum of the control and the studied groups. OC levels in the uncomplicated diabetic group were significantly lower while in the diabetic nephropathy group was significantly higher compared to control values . There was a weak negative correlation between OC and both fasting blood glucose and glycated Hb% in the diabetic group. In diabetic nephropathy patients, a weak positive correlation was observed between OC and protein creatinine ratio. The results concluded that changes in bone remodelling marker OC are present in both DM type 2 and diabetic nephropathy explaining osteopenia and osteoporosis observed in both cases.Therefore, an effective glycaemic control should be the hallmark of prevention and treatment of diabetes mellitus induced osteoporosis

Early extubation after congenital heart surgery

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Mirza Halimić

2014-12-01

Full Text Available Introduction: Despite recent advances in anesthesia, cardiopulmonary bypass and surgical techniques, children undergoing congenital heart surgery require postoperativemechanical ventilation. Early extubation was definedas ventilation shorter than 12 hours. Aim of this study is to identify factors associated with successful early extubation after pediatric cardiac surgery.Methods: The study was performed during period from January 2006 to January 2011 at Pediatric Clinic and Heart Center University Clinical center Sarajevo. One hundred children up to 5 years of age, who have had congenital heart disease, with left–right shunt and obstructive heart disease were included in the study. Patients were divided into two groups: Group I - patients extubated within 12 hours after surgery and Group II - patients extubated 12 or more hours after surgery. Results: The most frequently encountered preoperative variables were age with odds ratio 4% 95%CI (1-7%, Down's syndrome 8.5 95%CI (1.6-43.15, failure to thrive 4.3 95%CI( 1-18. Statistically significant postoperative data included lung disease (reactive airways, pneumonia, atelectasis, pneumothorax and with odds ratio 35.1 95 %CI (4-286 and blood transfusion with odds ratio 4.6 95%CI (2-12. Blood transfusion (p=0.002 (Wald=9.2 95%CI (2-12, during as well as after operation procedure has statistically significant influence on prediction time of extubation. Proven markers were age with cut of 21.5 months (sensitivity 74% and specificity 70% and extracorporeal circulation (ECC with cut-of 45.5 minutes (sensitivity 71% and specificity 65%.Conclusion: Early extubation is possible in many children undergoing congenital heart surgery. Younger age and prolonged ECC time are markers associated with prolonged mechanical ventilation.

The natural history of congenitally corrected transposition of the great arteries.

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Huhta, James

2011-01-01

The natural history of congenitally corrected transposition of the great arteries is of clinical/surgical importance once the fetus is born without heart block or signs of heart failure. Without significant tricuspid valve malformation, associated defects such as ventricular septal defect and left ventricular outflow obstruction can be repaired surgically. The mortality and long-term outcome appear to be linked strongly with the severity of tricuspid valve regurgitation. Some patients with an intact ventricular septum and no right ventricular dysfunction will live long lives without detection, and some women will successfully complete pregnancy.

Treatment of patients with a congenital transversal vaginal septum or a partial aplasia of the vagina. The vaginal pull-through versus the push-through technique.

NARCIS (Netherlands)

Bijsterveldt, C.D. van; Willemsen, W.N.P.

2009-01-01

STUDY OBJECTIVE: The aim of this study is to describe the different modalities of congenital obstructing vaginal malformations and the evaluation of techniques to solve the problem. DESIGN: A retrospective study. SETTING: The University Hospital Nijmegen, the Netherlands. PARTICIPANTS: The medical

Association of Intercellular Adhesion Molecule 1 (ICAM1 with Diabetes and Diabetic Nephropathy

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Harvest F Gu

2013-01-01

Full Text Available Diabetes and diabetic nephropathy are complex diseases affected by genetic and environmental factors. Identification of the susceptibility genes and investigation of their roles may provide useful information for better understanding of the pathogenesis and for developing novel therapeutic approaches. Intercellular adhesion molecule 1 (ICAM1 is a cell surface glycoprotein expressed on endothelial cells and leukocytes in the immune system. The ICAM1 gene is located on chromosome 19p13 within the linkage region of diabetes. In the recent years, accumulating reports have implicated that genetic polymorphisms in the ICAM1 gene are associated with diabetes and diabetic nephropathy. Serum ICAM1 levels in diabetes patients and the icam1 gene expression in kidney tissues of diabetic animals are increased compared to the controls. Therefore, ICAM1 may play a role in the development of diabetes and diabetic nephropathy. In this review, we present genomic structure, variation and regulation of the ICAM1 gene, summarized genetic and biological studies of this gene in diabetes and diabetic nephropathy and discussed about the potential application using ICAM1 as a biomarker and target for prediction and treatment of diabetes and diabetic nephropathy.

Choroidal thickness alterations in diabetic nephropathy patients with early or no diabetic retinopathy.

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Kocasarac, Can; Yigit, Yavuz; Sengul, Erkan; Sakalar, Yildirim Beyazit

2018-04-01

To assess changes in choroidal thickness (CT) in diabetes patients with and without diabetic nephropathy using enhanced depth imaging spectral domain optical coherence tomography (EDI-OCT). Thirty-five type 2 diabetes patients with a diagnosis of diabetic nephropathy (DNP) in nephrology department and 35 type 2 diabetes patients without nephropathy (non-DNP) were included in our prospective study consecutively. The control group comprised 34 healthy individuals. CT measurements were recorded under the fovea and at 1500 µm from the foveal center in the nasal and temporal sides. The study parameters also included age, refractive error, axial length, intraocular pressure, HbA1c, glomerular filtration rate and proteinuria amount. The subfoveal, temporal and nasal choroidal thickness was noted to be thinner in patients with DNP compared with non-DNP and normal subjects (p diabetic patients when diabetic nephropathy accompanies diabetes mellitus.

Radionuclide cisternography in diagnostics of obstruction hydrocephalus in introduced ventricculoperitoneal shunt

International Nuclear Information System (INIS)

Cernochova, I.; Lipina, R.

2004-01-01

To confirm or exclude a diagnosis of internal obstruction hydrocephalus in an 8-year old girl with an introduced ventriculoperitoneal (V-P) shunt Establishing the V-P shunt is indisputably the route of choice in non-communicating hydrocephalus. The existence of a V-P shunt, however, is connected with numerous risks, especially of injuries, infection as well as increased intraabdominal pressure - e.g. in pregnancy. The development of endoscopy in neurosurgery allows the creation of communication via the bottom of the third ventricle with basal cisterns, and the subsequent cancellation of a V-P shunt. We describe the case of an 8-year old girl with congenital internal hydrocephalus with an assumed obliteration of the Sylvian aqueduct with an established V-P shunt. An MR scan described the membrane in the area of the Sylvian aqueduct, but the disproportion between the dilatation of the lateral ventricles, third ventricle and fourth ventricle led the physician to doubt as to the accuracy of the diagnosis of internal obstruction hydrocephalus. Therefore we performed a radionuclide cisternography (in a modified manner), which proved an existing communication between the third and fourth cerebral ventricle and which contradicted the clinical diagnosis of obstruction hydrocephalus. (author)

The Congenital Heart Disease Genetic Network Study: Cohort description.

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Thanh T Hoang

Full Text Available The Pediatric Cardiac Genomics Consortium (PCGC designed the Congenital Heart Disease Genetic Network Study to provide phenotype and genotype data for a large congenital heart defects (CHDs cohort. This article describes the PCGC cohort, overall and by major types of CHDs (e.g., conotruncal defects and subtypes of conotrucal heart defects (e.g., tetralogy of Fallot and left ventricular outflow tract obstructions (e.g., hypoplastic left heart syndrome. Cases with CHDs were recruited through ten sites, 2010-2014. Information on cases (N = 9,727 and their parents was collected through interviews and medical record abstraction. Four case characteristics, eleven parental characteristics, and thirteen parent-reported neurodevelopment outcomes were summarized using counts and frequencies and compared across CHD types and subtypes. Eleven percent of cases had a genetic diagnosis. Among cases without a genetic diagnosis, the majority had conotruncal heart defects (40% or left ventricular outflow tract obstruction (21%. Across CHD types, there were significant differences (p<0.05 in the distribution of all four case characteristics (e.g., sex, four parental characteristics (e.g., maternal pregestational diabetes, and five neurodevelopmental outcomes (e.g., learning disabilities. Several characteristics (e.g., sex were also significantly different across CHD subtypes. The PCGC cohort is one of the largest CHD cohorts available for the study of genetic determinants of risk and outcomes. The majority of cases do not have a genetic diagnosis. This description of the PCGC cohort, including differences across CHD types and subtypes, provides a reference work for investigators who are interested in collaborating with or using publically available resources from the PCGC.

Recent Advances in the Pathogenesis and Management of Cast Nephropathy (Myeloma Kidney

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Stephanie Stringer

2011-01-01

Full Text Available Multiple myeloma is an incurable plasma cell malignancy that is often accompanied by renal failure; there are a number of potential causes of this, of which cast nephropathy is the most important. Renal failure is highly significant in myeloma, as patient survival can be stratified by the severity of the renal impairment. Consequently, there is an ongoing focus on the pathological basis of cast nephropathy and the optimal treatment regimens in this setting, including effective chemotherapy regimens to reduce light chain production and emerging extracorporeal techniques to remove circulating light chains. This paper bridges recent advances in the pathogenesis and management of cast nephropathy in multiple myeloma.

Congenital salivary gland anlage tumor - in utero and postnatal imaging

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Radhakrishnan, Rupa; Calvo-Garcia, Maria A.; Koch, Bernadette L. [Cincinnati Children' s Hospital Medical Center, Department of Radiology, Cincinnati, OH (United States); Lim, Foong-Yen [Cincinnati Children' s Hospital Medical Center, Division of Pediatric Surgery, Cincinnati, OH (United States); Elluru, Ravindhra G. [Cincinnati Children' s Hospital Medical Center, Division of Pediatric Otolaryngology, Cincinnati, OH (United States)

2015-03-01

We present a case of an infant with congenital salivary gland anlage tumor, with fetal and postnatal imaging. To the best of our knowledge, this is the first case describing the in utero imaging findings of salivary gland anlage tumor. A fetal MRI was performed secondary to the clinical finding of polyhydramnios, which identified a nasopharyngeal mass. Because findings were concerning for airway obstruction, the fetus was delivered by ex utero intrapartum treatment (EXIT) to airway procedure. A postnatal CT confirmed the findings of the fetal MRI. The lesion was resected when the baby was 4 days old and recovery was uneventful. (orig.)

Surgical treatment reduces blood pressure in children with unilateral congenital hydronephrosis.

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Al-Mashhadi, Ammar; Nevéus, Tryggve; Stenberg, Arne; Karanikas, Birgitta; Persson, A Erik G; Carlström, Mattias; Wåhlin, Nils

2015-04-01

Renal disorders can cause hypertension, but less is known about the influence of hydronephrosis on blood pressure. Hydronephrosis due to pelvo-ureteric junction obstruction (PUJO) is a fairly common condition (incidence in newborns of 0.5-1%). Although hypertensive effects of hydronephrosis have been suggested, this has not been substantiated by prospective studies in humans [1-3]. Experimental studies with PUJO have shown that animals with induced hydronephrosis develop salt-sensitive hypertension, which strongly correlate to the degree of obstruction [4-7]. Moreover, relief of the obstruction normalized blood pressure [8]. In this first prospective study our aim was to study the blood pressure pattern in pediatric patients with hydronephrosis before and after surgical correction of the ureteral obstruction. Specifically, we investigated if preoperative blood pressure is reduced after surgery and if split renal function and renographic excretion curves provide any prognostic information. Twelve patients with unilateral congenital hydronephrosis were included in this prospective study. Ambulatory blood pressure (24 h) was measured preoperatively and six months after surgery. Preoperative evaluations of bilateral renal function by Tc99m-MAG3 scintigraphy, and renography curves, classified according to O'Reilly, were also performed. As shown in the summary figure, postoperative systolic (103 ± 2 mmHg) and diastolic (62 ± 2 mmHg) blood pressure were significantly lower than those obtained preoperatively (110 ± 4 and 69 ± 2 mmHg, respectively), whereas no changes in circadian variation or pulse pressure were observed. Renal functional share of the hydronephrotic kidney ranged from 11 to 55%. There was no correlation between the degree of renal function impairment and the preoperative excretory pattern, or between the preoperative excretory pattern and the blood pressure reduction postoperatively. However, preoperative MAG3 function of the affected kidney correlated

Congenital heart disease in the newborn requiring early intervention

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Sin Weon Yun

2011-05-01

Full Text Available Although antenatal diagnostic technique has considerably improved, precise detection and proper management of the neonate with congenital heart disease (CHD is always a great concern to pediatricians. Congenital cardiac malformations vary from benign to serious conditions such as complete transposition of the great arteries (TGA, critical pulmonary and aortic valvular stenosis/atresia, hypoplastic left heart syndrome (HLHS, obstructed total anomalous pulmonary venous return (TAPVR, which the baby needs immediate diagnosis and management for survival. Unfortunately, these life threatening heart diseases may not have obvious evidence early after birth, most of the clinical and physical findings are nonspecific and vague, which makes the diagnosis difficult. High index of suspicion and astute acumen are essential to decision making. When patent ductus arteriosus (PDA is opened widely, many serious malformations may not be noticed easily in the early life, but would progress as severe acidosis/shock/cyanosis or even death as PDA constricts after few hours to days. Ductus dependent congenital cardiac lesions can be divided into the ductus dependent systemic or pulmonary disease, but physiologically quite different from each other and treatment strategy has to be tailored to the clinical status and cardiac malformations. Inevitably early presentation is often regarded as a medical emergency. Differential diagnosis with inborn error metabolic disorders, neonatal sepsis, persistent pulmonary hypertension of the newborn (PPHN and other pulmonary conditions are necessary. Urgent identification of the newborn at such high risk requires timely referral to a pediatric cardiologist, and timely intervention is the key in reducing mortality and morbidity. This following review deals with the clinical presentations, investigative modalities and approach to management of congenital cardiac malformations presenting in the early life.

Advanced diabetic nephropathy with “Clean” eyes: An extreme phenotype

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Debmalya Sanyal

2018-01-01

Full Text Available Introduction: It is generally accepted that renal and eye changes in diabetes are concordant. There are however a fair number of patients with diabetes who have end-stage renal disease (ESRD without any of the typical eye changes. The present study highlights the discordance between retinopathy and nephropathy and describes a series of patients of long-standing diabetes undergoing renal transplant who had little or no evidence of retinopathy. Methods: All patients with ESRD undergoing renal transplants underwent comprehensive fundus evaluation including dilated indirect ophthalmoscopy, slit lamp biomicroscopy, and fundus photography. The patients' age, gender, physical parameters (body mass index and blood pressure, duration of diabetes, glycosylated hemoglobin (HbA1c, albumin creatinine ratio, and presence of diabetic peripheral neuropathy (DPN were determined. Renal histopathology was reviewed, if available. Results: Five patients with diabetic nephropathy (DN underwent renal transplant and had no evidence of diabetic retinopathy (DR or up to two microaneurysms per fundus. All the patients were between 50 and 65 (mean ± standard deviation – 58.6 ± 4.67 years of age. The mean duration of diabetes was 16 ± 2.91 years. All had poor glycemic control with a mean HbA1c of 9.2 ± 0.837%. All had hypertension, macroalbuminuria, and DPN. Conclusion: There is a well-recognized association between retinopathy and nephropathy, in which nephropathy without retinopathy is rare but retinopathy without nephropathy is common. We have identified a subset of patients with kidney disease of sufficient severity to warrant renal transplant but who are protected from retinopathy. It is possible that there is an extreme phenotype of DN patients with unaffected eyes who carry genes protecting against DR.

Chronic nephropathies of cocaine and heroin abuse: a critical review.

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Jaffe, Jared A; Kimmel, Paul L

2006-07-01

Renal disease in cocaine and heroin users is associated with the nephrotic syndrome, acute glomerulonephritis, amyloidosis, interstitial nephritis, and rhabdomyolysis. The pathophysiologic basis of cocaine-related renal injury involves renal hemodynamic changes, glomerular matrix synthesis and degradation, and oxidative stress and induction of renal atherogenesis. Heroin is the most commonly abused opiate in the United States. Previous studies identified a spectrum of renal diseases in heroin users. The predominant renal lesion in black heroin users is focal segmental glomerulosclerosis and in white heroin users is membranoproliferative glomerulonephritis. Although the prevalence of heroin use in the United States has increased, the incidence of "heroin nephropathy" has declined. Because reports of heroin nephropathy predated the surveillance of hepatitis C virus and HIV, the varied findings might be related to the spectrum of viral illnesses that are encountered in injection drug users. Socioeconomic conditions, cultural and behavioral practices, or differences in genetic susceptibilities may be more associated with the development of nephropathy in heroin users than the drug's pharmacologic properties. Administration of cocaine in animal models results in nonspecific glomerular, interstitial, and tubular cell lesions, but there is no animal model of heroin-associated renal disease. The heterogeneity of responses that are associated with heroin is not consistent with a single or simple notion of nephropathogenesis. There are no well-designed, prospective, epidemiologic studies to assess the incidence and the prevalence of renal disease in populations of opiate users and to establish the validity of a syndrome such as heroin nephropathy. It is concluded although there is a paucity of evidence to support a heroin-associated nephropathy, the evidence from in vitro cellular and animal studies to support the existence of cocaine-induced renal changes is more convincing.

Effect of nephrotoxic drugs on the development of radiation nephropathy after bone marrow transplantation

International Nuclear Information System (INIS)

Lawton, C.A.; Fish, B.L.; Moulder, J.E.

1994-01-01

Chronic renal failure is a significant cause of late morbidity in bone marrow transplant patients whose conditioning regimen includes total body irradiation (TBI). Radiation is a major cause of this syndrome (bone marrow transplant nephropathy), but it may not be the only cause. These studies use a rat syngeneic bone marrow transplant model to determine whether nephrotoxic agents used in conjunction with bone marrow transplantation (BMT) could be enhancing or accelerating the development of radiation nephropathy. Rats received 11-17 Gy TBI in six fractions over 3 days followed by syngeneic bone marrow transplant. In conjunction with the bone marrow transplants, animals received either no drugs, cyclosporine, amphotericin, gentamicin, or busulfan. Drugs were given in schedules analogous to their use in clinical bone marrow transplantation. Drug doses were chosen so that the drug regimen alone caused detectable acute nephrotoxicity. Animals were followed for 6 months with periodic renal function tests. Gentamicin had no apparent interactions with TBI. Amphotericin increased the incidence of engraftment failure, but did not enhance radiation nephropathy. Cyclosporin with TBI caused late morbidity that appeared to be due to neurological problems, but did not enhance radiation nephropathy. Busulfan resulted in a significant enhancement of radiation nephropathy. Of the nephrotoxins used in conjunction with bone marrow transplantation only radiation and busulfan were found to be risk factors for bone marrow transplant nephropathy. 34 refs., 4 figs., 2 tabs

C1q Nephropathy: The Unique Underrecognized Pathological Entity

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Joe Devasahayam

2015-01-01

Full Text Available C1q nephropathy is a rare glomerular disease with characteristic mesangial C1q deposition noted on immunofluorescence microscopy. It is histologically defined and poorly understood. Light microscopic features are heterogeneous and comprise minimal change disease (MCD, focal segmental glomerulosclerosis (FSGS, and proliferative glomerulonephritis. Clinical presentation is also diverse, and ranges from asymptomatic hematuria or proteinuria to frank nephritic or nephrotic syndrome in both children and adults. Hypertension and renal insufficiency at the time of diagnosis are common findings. Optimal treatment is not clear and is usually guided by the underlying light microscopic lesion. Corticosteroids are the mainstay of treatment, with immunosuppressive agents reserved for steroid resistant cases. The presence of nephrotic syndrome and FSGS appear to predict adverse outcomes as opposed to favorable outcomes in those with MCD. Further research is needed to establish C1q nephropathy as a universally recognized distinct clinical entity. In this paper, we discuss the current understanding of pathogenesis, histopathology, clinical features, therapeutic options, and outcomes of C1q nephropathy.

Risk of congenital anomalies in the vicinity of municipal solid waste incinerators.

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Cordier, S; Chevrier, C; Robert-Gnansia, E; Lorente, C; Brula, P; Hours, M

2004-01-01

Although municipal solid waste incineration (MSWI) has contributed to increase the overall environmental load of particulate matter containing dioxins and metals, evidence of health consequences to populations is sparse. To assess at a regional level (in southeast France) the impact of these emissions on birth defect rates. Communities with fewer than 50 000 inhabitants surrounding the 70 incinerators that operated at least one year from 1988 to 1997 were studied. Each exposed community (n = 194) was assigned an exposure index estimated from a Gaussian plume model. Poisson models and a reference population of the 2678 unexposed communities in the region were used to calculate relative risks for congenital malformations, adjusted for year of birth, maternal age, department of birth, population density, average family income, and when available, local road traffic. The rate of congenital anomalies was not significantly higher in exposed compared with unexposed communities. Some subgroups of major anomalies, specifically facial clefts and renal dysplasia, were more frequent in the exposed communities. Among exposed communities, a dose-response trend of risk with increasing exposure was observed for obstructive uropathies. Risks of cardiac anomalies, obstructive uropathies, and skin anomalies increased linearly with road traffic density. Although both incinerator emissions and road traffic may plausibly explain some of the excess risks observed, several alternative explanations, including exposure misclassification, ascertainment bias, and residual confounding cannot be excluded. Some of the effects observed, if real, might be attributable to old-technology MSWIs and the persistent pollution they have generated.

Surgical indications for unilateral neonatal hydronephrosis in considering ureteropelvic junction obstruction

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Hong-Lin Cheng

2014-09-01

Full Text Available Prenatal hydronephrosis is one of the most common urological congenital abnormalities detected by ultrasound. The incidence ranges from 0.59% to 0.69%. Approximately 50% of these fetuses do not have hydronephrosis on postnatal examination, whereas 25–33% of the rest have persistent hydronephrosis leading to the diagnosis of ureteropelvic junction (UPJ obstruction. Renal ultrasonography and renal radionuclide scanning are the major modalities used for assessment and follow-up. Three main criteria used to determine the presence of obstruction are: (1 the magnitude of hydronephrosis present on ultrasound, (2 the relative renal function (RRF measured by renography, and (3 the response of radionuclide washout with furosemide. Unfortunately, it is not always easy to determine obstruction; different types of management have been developed. Without depending on the severity of renal pelvis dilation, percentage of RRF, and response of radionuclide washout in the initial presentation, early surgery to preserve renal function and aggressive observation to prevent unnecessary surgery are two extremes on the spectrum of management for neonatal UPJ obstruction. Relying on renal function in renography, <35–40% or 5–10% of a decrease in the percentage of RRF or on the enlarging of hydronephrosis, respectively, and parenchymal thinning on ultrasonography are the indications for the surgical management to recover renal function in time. In addition to renal function change and imaging progression, the follow-up protocol and family compliance are the other considerations in prevention of impaired renal function. Through more than 40 years of development in the field of UPJ obstruction in infants, there have been several advances in management but controversies remain to be resolved. In this review, we focus on the surgical indications for the UPJ obstruction in this cohort.

Polyoma Virus Nephropathy, First reported case in Saudi Arabia

International Nuclear Information System (INIS)

Siddiqui, N.A.; Hamid, M.H.; Bokhari, E.; El-Tayeb, A.

2006-01-01

Polyoma virus nephropathy (BK virus) is being recognized as an important cause of graft failure. It is usually confused with acute rejection. No cases have been reported from the kingdom of Saudi Arabia. We report a case of a Saudi gentleman, who was transplanted outside the country, with persistently elevated creatinine and urethral stenosis. He was treated for acute rejection on more than one occasion with no significant improvement in his renal function. Polyoma virus nephropathy was diagnosed by detecting the virus DNA by the polychain reaction techniques (PCR). The patient's renal function stabilized after the calcineurin inhibitors were discontinued. (author)

The use of expandable metallic airway stents for tracheobronchial obstruction in children.

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Filler, R M; Forte, V; Fraga, J C; Matute, J

1995-07-01

Expandable metallic angioplasty stents (Palmaz stent) have been implanted in the trachea and/or bronchi of seven children. Three children had severe tracheal stenosis after tracheoplasty for congenital tracheal stenosis repair, and four had tracheomalacia or bronchomalacia with or without vascular compression. The mean age at stenting was 9.7 months (range, 2 to 15 months). Balloon expandable stents were inserted into the trachea or bronchus through a 3.5-mm bronchoscope under fluoroscopic control. Initially a single tracheal stent was used for all patients except for one with obstruction in the trachea and both bronchi, in whom three stents were implanted. Three children had recurrent airway obstruction 1 month later; one was cured with a second stent; one child died 1 year later; and the other is being treated for heart disease. The others have no serious respiratory problems. The stents in all have been in place for 1 to 25 (mean, 11) months. No immediate complications were noted. Early and late bronchoscopy showed incomplete epithelialization of the stent and patches of granulation tissue on it. Two stents were removed bronchoscopically, one at the completion of treatment for tracheomalacia and the other at the time of recurrent airway obstruction. This preliminary experience indicates that expandable metallic stents have a useful role in the treatment of selected lower airway obstructions.

Urinary uromodulin excretion predicts progression of chronic kidney disease resulting from IgA nephropathy.

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Jingjing Zhou

Full Text Available BACKGROUND: Uromodulin, or Tamm-Horsfall protein, is the most abundant urinary protein in healthy individuals. Recent studies have suggested that uromodulin may play a role in chronic kidney diseases. We examined an IgA nephropathy cohort to determine whether uromodulin plays a role in the progression of IgA nephropathy. METHODS: A total of 344 IgA nephropathy patients were involved in this study. Morphological changes were evaluated with the Oxford classification of IgA nephropathy. Enzyme Linked Immunosorbent Assay (ELISA measured the urinary uromodulin level on the renal biopsy day. Follow up was done regularly on 185 patients. Time-average blood pressure, time-average proteinuria, estimated glomerular filtration rate (eGFR and eGFR decline rate were caculated. Association between the urinary uromodulin level and the eGFR decline rate was analyzed with SPSS 13.0. RESULTS: We found that lower baseline urinary uromodulin levels (P = 0.03 and higher time-average proteinuria (P = 0.04 were risk factors for rapid eGFR decline in a follow-up subgroup of the IgA nephropathy cohort. Urinary uromodulin level was correlated with tubulointerstitial lesions (P = 0.016. Patients that had more tubular atrophy/interstitial fibrosis on the surface had lower urinary uromodulin levels (P = 0.02. CONCLUSIONS: Urinary uromodulin level is associated with interstitial fibrosis/tubular atrophy and contributes to eGFR decline in IgA nephropathy.

Congenital chylous ascites in infants: another presentation of intestinal malrotation.

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Long, Li; Zhen, Chen; Yandong, Wei; Ning, Dong; Qi, Li; Qing, Gao

2018-03-01

The cause of the chylous ascites in infants isn't completely clear. The purpose of this study is to discuss our experience of recognition of intestinal malrotation as a cause of congenital chylous ascites in infants. Medical information of 10 infants with chylous ascites, who were admitted to the hospital between 2001 and 2014, was retrospective analyzed. Preoperatively, all patients underwent a period of conservative treatment. We found that nine of ten patients with intestinal malrotation, six of them underwent laparoscopic Ladd's procedure and three patients underwent open Ladd's procedure. The remaining one patient suffered from mesenteric lymph nodes rupture and laparoscopic resection was performed. The cylous ascites subsided in all patients after the surgery and no significant recurrence was encountered during follow-up time. Our study demonstrates that congenital chylous ascites could be caused by intestinal malrotation, causing the obstruction of the lymphatic flow in the mesenteric lymphatic channels. Ladd's procedure maybe a safe and effective treatment for infantile intractable chylous ascites. Treatment study. Level IV. Copyright © 2017 Elsevier Inc. All rights reserved.

Autosomal recessive polycystic kidney disease and congenital hepatic fibrosis (ARPKD/CHF)

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Turkbey, Baris; Choyke, Peter L. [National Institutes of Health, Molecular Imaging Program, National Cancer Institute, Bethesda, MD (United States); Ocak, Iclal [National Institutes of Health, Molecular Imaging Program, National Cancer Institute, Bethesda, MD (United States); University of Pittsburgh Medical Center, Department of Radiology, Pittsburgh, PA (United States); Daryanani, Kailash [National Institutes of Health, Clinical Center, Department of Radiology, Bethesda, MD (United States); Font-Montgomery, Esperanza; Lukose, Linda; Bryant, Joy; Tuchman, Maya; Gahl, William A. [National Institutes of Health, National Human Genome Research Institute, Medical Genetics Branch, Bethesda, MD (United States); Mohan, Parvathi [George Washington University, Department of Pediatric Gastroenterology, Washington, DC (United States); Heller, Theo [National Institutes of Health, National Institute of Diabetes and Digestive and Kidney Diseases, Bethesda, MD (United States); Gunay-Aygun, Meral [National Institutes of Health, National Human Genome Research Institute, Medical Genetics Branch, Bethesda, MD (United States); National Institutes of Health, Intramural Program, Office of Rare Diseases, Office of the Directors, Bethesda, MD (United States)

2009-02-15

ARPKD/CHF is an inherited disease characterized by non-obstructive fusiform dilatation of the renal collecting ducts leading to enlarged spongiform kidneys and ductal plate malformation of the liver resulting in congenital hepatic fibrosis. ARPKD/CHF has a broad spectrum of clinical presentations involving the kidney and liver. Imaging plays an important role in the diagnosis and follow-up of ARPKD/CHF. Combined use of conventional and high-resolution US with MR cholangiography in ARPKD/CHF patients allows detailed definition of the extent of kidney and hepatobiliary manifestations without requiring ionizing radiation and contrast agents. (orig.)

A comparison of spirapril and isradipine in patients with diabetic nephropathy and hypertension

DEFF Research Database (Denmark)

Nørgaard, K; Jensen, T; Christensen, P

1993-01-01

The effects of spirapril and isradipine on blood pressure, urinary albumin excretion and sodium-volume homeostasis in hypertensive insulin-dependent diabetic patients with nephropathy were assessed. Fifteen Type 1 diabetic patients aged 28-53 years with a diabetes duration of 19-37 years were...... studied. All had hypertension and diabetic nephropathy with a urinary albumin excretion of more than 300 mg/24 h. After a single blind placebo treatment period of 4 weeks the patients were randomly assigned to treatment with the calcium antagonist isradipine SRO 5 mg once daily or the ACE inhibitor...... vs 2636 +/- 194 meq/1.73 m2 (p diabetic nephropathy. Only the ACE inhibitor had demonstrable...

Reduction in albuminuria predicts a beneficial effect on diminishing the progression of human diabetic nephropathy during antihypertensive treatment

DEFF Research Database (Denmark)

Rossing, P; Hommel, E; Smidt, U M

1994-01-01

Diabetic nephropathy is the main cause of increased mortality and morbidity in IDDM patients. The effect of antihypertensive treatment on the progression of the nephropathy is highly variable. The aim of this study was to evaluate putative predictors of the progression in diabetic nephropathy dur...

Does the urinary protein pattern in AA-Amyloid nephropathy differ from that in other nephropathies?

International Nuclear Information System (INIS)

Teppo, A.M.; Maury, C.P.J.

1986-01-01

The urinary excretion of six plasma proteins was determined in reactive (secondary) amyloidosis, in rheumatoid arthritis, in systemic lupus erythematosus, in diabetic patients, in patients with chronic glomerulonephritis and in healthy controls. The type of proteinuria in patients with amyloidosis was compared with that of other patient groups and of nephropathies due to glomerulonephritis or diabetes. In amyloidosis the excretion of lambda light chains was slightly higher and that of kappa chains slightly lower than in other proteinurias, consequently the ratio lambda/kappa chains in patients with reactive amyloidosis was higher (p ≤ 0.01) than in other patient groups or in healthy controls. In patients with moderate/heavy proteinuria the excretion of IgG compared with that of albumin was in reactive amyloidosis as well as in diabetic nephropathy lower than in glomerulonephritis (p ≤ 0.05) and suggest the higher selectivity of protein excretion in these patients than in glomerulonephritis. The finding that the ratio of excreted lambda/kappa chains in reactive amyloidosis exceeds that of normal plasma indicates in these patients either increased plasma concentration and/or decreased reabsorption of lambda light chains

Gallic acid attenuates type I diabetic nephropathy in rats.

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Garud, Mayuresh Sudamrao; Kulkarni, Yogesh Anant

2018-02-25

Literature suggests that TGF-β1 has a central role in the progression of diabetic nephropathy and its down regulation can improve the disease condition. Oxidative stress, generation of advanced glycation end products and activation of renin angiotensin system are the connecting links between hyperglycemia and TGF-β1 over expression. Gallic acid is a phytochemical having wide range of biological activities. Gallic acid is reported to have antioxidant and advanced glycation inhibitory activity. It has also shown inhibitory effects on angiotensin converting enzyme. Gallic acid qualifies as a drug candidate to be tested in the diabetic nephropathy, one of the important complication of diabetes. Streptozotocin (55 mg/kg body weight, i.p.) induced diabetic nephropathy was used as an experimental model. Gallic acid was evaluated for its possible effect at the dose of 20 and 40 mg/kg body weight. Gallic acid treatment significantly lowered plasma levels of the creatinine and blood urea nitrogen and elevated the levels of the protein and albumin. Gallic acid also improved creatinine clearance. Determination of oxidative stress parameters showed that the oxidative stress in kidney tissues was reduced significantly in gallic acid treated animals. Results of the plasma, urine and oxidative stress parameters were also reflected in the histopathological evaluation showing improvement in kidney pathophysiology. ELISA assay for circulating TGF-β1 evaluation and immunohistochemical study for determination of kidney expression of TGF-β1 revealed that gallic acid significantly lowered both the circulating and tissue levels of TGF-β1. Results support the hypothesis that gallic acid can be effectively used in the treatment of diabetic nephropathy. Copyright © 2018 Elsevier B.V. All rights reserved.

Ichthyosiform mycosis fungoides with alopecia and atypical membranous nephropathy

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Qiang Zhou

2011-01-01

Full Text Available We describe here a rare case of variant of mycosis fungoides (MF: ichthyosiform MF with alopecia and atypical membranous nephropathy. The diagnosis was made based on the following findings: generalized ichthyosis-like eruption, alopecia, enlarged superficial lymph nodes, proteinuria, and hematuria, the histological features of the skin biopsy from both ichthyotic and alopecic lesions with immunohistochemical staining, and the renal biopsy examination with immunofluorescence. The histological examination of ichthyotic and alopecic lesions displayed a predominant infiltration of atypical lymphocytes in the upper dermis with the characteristics of epidermotropism and folliculotropism. Immunohistochemical studies demonstrated that most infiltrated atypical lymphocytes were CD3, CD4, and CD45RO positive, whereas negative for CD5, CD7, CD20, CD30, and CD56. A renal biopsy examination revealed atypical membranous nephropathy with deposition of immunoglobulin G (IgG, IgM, IgA, C1q, and C3. In this case atypical membranous nephropathy was involved, which is very uncommon and has never been presented in the literature to date. Although ichthyosiform MF usually features a relatively favorable course, diffuse alopecia and the renal involvement in this case might indicate aggressive disease and poor prognosis.

Vocal cord collapse during phrenic nerve-paced respiration in congenital central hypoventilation syndrome.

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Domanski, Mark C; Preciado, Diego A

2012-01-01

Phrenic nerve pacing can be used to treat congenital central hypoventilation syndrome (CCHS). We report how the lack of normal vocal cord tone during phrenic paced respiration can result in passive vocal cord collapse and produce obstructive symptoms. We describe a case of passive vocal cord collapse during phrenic nerve paced respiration in a patient with CCHS. As far as we know, this is the first report of this etiology of airway obstruction. The patient, a 7-year-old with CCHS and normal waking vocal cord movement, continued to require nightly continuous positive airway pressure (CPAP) despite successful utilization of phrenic nerve pacers. On direct laryngoscopy, the patient's larynx was observed while the diaphragmatic pacers were sequentially engaged. No abnormal vocal cord stimulation was witnessed during engaging of either phrenic nerve stimulator. However, the lack of normal inspiratory vocal cord abduction during phrenic nerve-paced respiration resulted in vocal cord collapse and partial obstruction due to passive adduction of the vocal cords through the Bernoulli effect. Bilateral phrenic nerve stimulation resulted in more vocal cord collapse than unilateral stimulation. The lack of vocal cord abduction on inspiration presents a limit to phrenic nerve pacers.

The association of congenital neuroblastoma and congenital heart disease

International Nuclear Information System (INIS)

Bellah, R.; D'Andrea, A.; Children's Hospital, Boston, MA; Darillis, E.; Fellows, K.E.

1989-01-01

Several authors have reported an association between neuroblastoma and congenital heart disease; others contend that, unlike specific wellknown associations between malignancy and congenital defects (Wilm's tumor and aniridia, leukemia and Down's syndrome), no real relationship exists. We present three cases of cyanotic congenital heart disease in which subclinical neuroblastoma was found. We speculate that abnormal neural crest cell migration and development may be a common link between cardiac malformations and congenital neuroblastoma. (orig.)

A Mass of Pancreatic and Gastric Heterotopia Causing a Small Bowel Obstruction in a 61-Year-Old Male.

Science.gov (United States)

Alfrejat, Majd; Khalil, Bassem; Jackobs, Jordan; Anderson, William; Eschbacher, Jennifer

2017-01-01

Heterotopic tissue is a congenital anomaly that has been previously reported. Gastric and pancreatic heterotopia are among the most studied ones. Herein, we describe a case of a combined pancreatic and gastric heterotopia that formed a mass and caused a small intestine obstruction in a 61-year-old male. We also did a brief literature review of cases with gastric and pancreatic heterotopia in adult patients.

Mast Cells and MCPT4 Chymase Promote Renal Impairment after Partial Ureteral Obstruction

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Maguelonne Pons

2017-05-01

Full Text Available Obstructive nephropathy constitutes a major cause of pediatric renal progressive disease. The mechanisms leading to disease progression are still poorly understood. Kidney fibrotic lesions are reproduced using a model of partial unilateral ureteral obstruction (pUUO in newborn mice. Based on data showing significant mast cell (MC infiltration in patients, we investigated the role of MC and murine MCPT4, a MC-released chymase, in pUUO using MC- (Wsh/sh, MCPT4-deficient (Mcpt4−/−, and wild-type (WT mice. Measurement of kidney length and volume by magnetic resonance imaging (MRI as well as postmortem kidney weight revealed hypotrophy of operated right kidneys (RKs and compensatory hypertrophy of left kidneys. Differences between kidneys were major for WT, minimal for Wsh/sh, and intermediate for Mcpt4−/− mice. Fibrosis development was focal and increased only in WT-obstructed kidneys. No differences were noticed for local inflammatory responses, but serum CCL2 was significantly higher in WT versus Mcpt4−/− and Wsh/sh mice. Alpha-smooth muscle actin (αSMA expression, a marker of epithelial–mesenchymal transition (EMT, was high in WT, minimal for Wsh/sh, and intermediate for Mcpt4−/− RK. Supernatants of activated MC induced αSMA in co-culture experiments with proximal tubular epithelial cells. Our results support a role of MC in EMT and parenchyma lesions after pUUO involving, at least partly, MCPT4 chymase. They confirm the importance of morphologic impairment evaluation by MRI in pUUO.

THE ROLE OF DURATION OF DIABETES IN THE DEVELOPMENT OF NEPHROPATHY

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Ishwar Sidappa Hasabi

2017-12-01

Full Text Available BACKGROUND Diabetes has now become the most common single cause of end-stage renal disease. Diabetic Kidney Disease (DKD is a lifethreatening and irreversible microvascular complication characterised by presence of persistent proteinuria, hypertension and progressive decline in renal function. Early detection and risk reduction measures can prevent diabetic nephropathy. Screening for microalbuminuria will allow early identification of patients with nephropathy provide an opportunity for early treatment, which has been shown to preserve renal function and thus prevent morbidity and mortality from diabetic nephropathy. The aim of the study is to study the relation between duration of diabetes and nephropathy. MATERIALS AND METHODS 120 patients with type 2 diabetes mellitus admitted to medical wards, KIMS, Hubli, over a period of one year satisfying the inclusion and exclusion criteria were enrolled for the study. 40 normal healthy adults were included in the control group. It’s a cross-sectional study and patients were enrolled by random sampling method. All the selected patients were subjected to detailed history and complete physical examination and data collected was noted in a predesigned pro forma. RESULTS Study participants were subdivided based on duration of diabetes into 10 years. Their mean age of onset of diabetes was 54.5 (± 10 years. Microalbuminuria was present in 45% (n=54 of diabetics, retinopathy 35.8% (n=43 and both increased with increase in duration of diabetes (p value 0.003 and 0.001, respectively (Table 3 and 4. Prevalence of hypertension was 51.7% in present study group and was significantly associated with duration of diabetes. CONCLUSION This study highlighted the prevalence of microalbuminuria and retinopathy in type 2 diabetes subjects. Microalbuminuria increases with increase in duration of diabetes. Screening for microalbuminuria will allow early detection of patients with nephropathy.

Non-Alcoholic Fatty Liver Disease Is not Related to the Incidence of Diabetic Nephropathy in Type 2 Diabetes

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Chun-Shan Bi

2012-11-01

Full Text Available To analyze the association between non-alcoholic fatty liver disease (NAFLD and the incidence of diabetic nephropathy in patients with type 2 diabetes, the incidence of diabetic nephropathy was assessed in 413 type 2 diabetic patients, by testing the 24 h urinary albumin excretion rate (UAER. The NAFLD was diagnosed based on patient’s medical history and liver ultrasound. The difference in diabetic nephropathy incidence between patients with and without NAFLD was tested by χ2. Multivariate logistic regression analysis was used to assess the factors associated with diabetic nephropathy among type 2 diabetic patients. Total 363 out of 413 type 2 diabetic patients were enrolled in this study. The incidences of NAFLD and diabetic nephropathy in participants were approximately 56% (202/363 and 38% (137/363 respectively, and there was no significant difference in the prevalence of diabetic nephropathy between patients with and without NAFLD (37.1% vs. 38.5%, p = 0.787. The duration of diabetes (odds ratio [OR] 1.065, 95% confidence interval [CI] 1.014–1.120, p = 0.012, waist circumference (OR 1.077, 95% CI 1.040–1.116, p = 0.000, and fasting blood glucose (FBG; OR 1.136, 95% CI 1.023–1.1262, p = 0.017 were significantly associated with diabetic nephropathy, whereas sex, high blood pressure, total cholesterol (TC, triglyceride (TG, and ankle brachial pressure index (ABI were not significantly associated with the disorder. The present results suggest that NAFLD is not related to the incidence of diabetic nephropathy in type 2 diabetes, but the duration of diabetes, waist circumference, and FBG are important factors for diabetic nephropathy in type 2 diabetes.

Transforming growth factor-β in diabetic nephropathy

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Karima Y. Ahmed

2013-01-01

Conclusion Serum TGF-β level increases in patients of both type 1 and type 2 diabetes and in those with diabetic nephropathy. TGF-β is considered one of the major mediators of diabetic renal fibrogenesis that Results in end-stage renal disease.

Contrast medium-induced nephropathy: Aspects on incidence ...

African Journals Online (AJOL)

Contrast media-induced nephropathy (CIN) is a well-known complication of radiological examinations employing iodine contrast media (I-CM). The rapid development and frequent use of coronary interventions and multi-channel detector computed tomography with concomitant administration of relatively large doses of ...

Management of airway obstruction and stridor in pediatric patients [digest].

Science.gov (United States)

Marchese, Ashley; Langhan, Melissa L; Pade, Kathryn H

2017-11-22

Stridor is a result of turbulent air-flow through the trachea from upper airway obstruction, and although in children it is often due to croup, it can also be caused by noninfectious and/or congenital conditions as well as life-threatening etiologies. The history and physical examination guide initial management, which includes reduction of airway inflammation, treatment of bacterial infection, and, less often, imaging, emergent airway stabilization, or surgical management. This issue discusses the most common as well as the life-threatening etiologies of acute and chronic stridor and its management in the emergency department. [Points & Pearls is a digest of Pediatric Emergency Medicine Practice].

Dynamic magnetic resonance imaging in the assessment of chronic medical nephropathies with impaired renal function

International Nuclear Information System (INIS)

Dalla-Palma, L.; Pozzi-Mucelli, R.S.; Cova, M.; Meduri, S.; Panzetta, G.; Galli, G.

2000-01-01

We examined the value of dynamic magnetic resonance imaging (MRI) in chronic renal disease with renal insufficiency. In 33 consecutive patients (21 vascular nephropathy, 12 glomerular nephropathy) MRI was performed using a 1.5-T unit and a body coil, with SE T1-weighted (TR/TE = 600/19 ms) and dynamic TFFE T1-weighted sequences (TR/TE = 12/5 ms, flip angle = 25 ) after manual bolus injection (via a cubital vein) of 0.1 mmol/kg Gd-DTPA-BMA. Morphological evaluation was performed in unblinded fashion by three radiologists, evaluating renal size, cortical thickness, and corticomedullary differentiation. Functional analysis was performed by one reviewer. Time-signal intensity curves, peak intensity value (P), time to peak intensity (T), and the P/T ratio were obtained at the cortex, medulla, and pyelocaliceal system of each kidney. The relationship of these parameters to serum creatinine and with creatinine clearance was investigated. A good correlation between morphological features of the kidneys and serum creatinine values was found. Morphological findings could not distinguish between vascular and glomerular nephropathies. A statistically significant correlation (P <0.01) between cortical P, cortical P/T, medullary P, and serum creatinine and creatinine clearance was found. A significant correlation (P <0.01) was also found between cortical T, medullary P/T, T of the excretory system, and creatinine clearance. The cortical T value was significantly higher (P <0.01) in vascular nephropathy than in glomerular nephropathy. Thus in patients with chronic renal failure dynamic MRI shows both morphological and functional changes. Morphological changes are correlated with the degree of renal insufficiency and not with the type of nephropathy; the functional changes seem to differ in vascular from glomerular nephropathies. (orig.)

CD44 expression in IgA nephropathy

NARCIS (Netherlands)

Florquin, Sandrine; Nunziata, Raffaele; Claessen, Nike; van den Berg, Frank M.; Pals, Steven T.; Weening, Jan J.

2002-01-01

Immunoglobulin A (IgA) nephropathy is a frequent, chronic renal disease characterized by a broad spectrum of clinical presentations and pathologic findings. CD44, a family of type I transmembrane glycoproteins: involved in cell-cell and cell-matrix interactions, may orchestrate partially the cascade

Mice deficient in PAPP-A show resistance to the development of diabetic nephropathy.

Science.gov (United States)

Mader, Jessica R; Resch, Zachary T; McLean, Gary R; Mikkelsen, Jakob H; Oxvig, Claus; Marler, Ronald J; Conover, Cheryl A

2013-10-01

We investigated pregnancy-associated plasma protein-A (PAPP-A) in diabetic nephropathy. Normal human kidney showed specific staining for PAPP-A in glomeruli, and this staining was markedly increased in diabetic kidney. To assess the possible contribution of PAPP-A in the development of diabetic nephropathy, we induced diabetes with streptozotocin in 14-month-old WT and Papp-A knockout (KO) mice. Renal histopathology was evaluated after 4 months of stable hyperglycemia. Kidneys from diabetic WT mice showed multiple abnormalities including thickening of Bowman's capsule (100% of mice), increased glomerular size (80% of mice), tubule dilation (80% of mice), and mononuclear cell infiltration (90% of mice). Kidneys of age-matched non-diabetic WT mice had similar evidence of tubule dilation and mononuclear cell infiltration to those of diabetic WT mice, indicating that these changes were predominantly age-related. However, thickened Bowman's capsule and increased glomerular size appeared specific for the experimental diabetes. Kidneys from diabetic Papp-A KO mice had significantly reduced or no evidence of changes in Bowman's capsule thickening and glomerular size. There was also a shift to larger mesangial area and increased macrophage staining in diabetic WT mice compared with Papp-A KO mice. In summary, elevated PAPP-A expression in glomeruli is associated with diabetic nephropathy in humans and absence of PAPP-A is associated with resistance to the development of indicators of diabetic nephropathy in mice. These data suggest PAPP-A as a potential therapeutic target for diabetic nephropathy.

Diabetic nephropathy. Is end-stage renal disease inevitable?

Science.gov (United States)

Bogusky, R T

1983-10-01

The appearance of proteinuria in an insulin-dependent diabetic patient is an ominous sign. Proteinuria heralds the presence of diabetic nephropathy and early death, or chronic renal failure requiring dialysis or transplantation, in 50% of patients. The pathogenesis of diabetic nephropathy is unknown. Adequate insulin administration is the most important preventive measure. Hypertension, if present, should be aggressively treated to delay progression of renal disease. Good nutrition, prompt treatment of urinary tract infections, and caution in the use of radiocontrast agents are other important preventive measures. Hemodialysis, peritoneal dialysis, and transplantation are options for patients with end-stage renal disease. No matter which is selected, the patient may still have multiple amputations, blindness, congestive heart failure, infections, and uncontrolled glycemia. Advancements are being made, however, that promise a better future for insulin-dependent diabetics.

Immunoglobulin A nephropathy in association with generalized inflammatory peeling skin syndrome.

Science.gov (United States)

Srinivasaraghavan, Rangan; Krishnamurthy, Sriram; Chandar, Rumesh; Mahadevan, Subramanian; Chandrashekar, Laxmisha; Rajesh, Nachiappa Ganesh

2015-01-01

We describe an 8-year-old girl born to second-degree consanguineous parents with complaints of recurrent episodes of hematuria for 6 months. She had generalized peeling of the skin since birth and recurrent purulent cutaneous infections. The clinical presentation and histopathology of the skin biopsy specimen were consistent with the inflammatory variant of peeling skin syndrome (PSS). She also had a single ventricle with pulmonary stenosis, for which a bidirectional Glenn shunt had been placed. The renal biopsy specimen showed immunoglobulin A (IgA) nephropathy. She responded well to enalapril and steroids, with a decrease in proteinuria. IgA nephropathy has not been previously reported in PSS. Complications such as IgA nephropathy in children with PSS would help to further delineate the diverse clinical presentations and the clinical course of this rare dermatosis. We discuss the mechanisms that could explain this hitherto unreported association. © 2014 Wiley Periodicals, Inc.

Diabetic nephropathy and arterial hypertension. The effect of antihypertensive treatment

DEFF Research Database (Denmark)

Parving, H H; Andersen, A R; Smidt, U M

1983-01-01

method for albumin determination. Our prospective studies in young insulin-dependent diabetics with diabetic nephropathy show that the rate of decline in glomerular filtration rate (GFR) varies considerably, with a mean of 0.75 ml/min/mo and a range from 0.1 to 1.50 ml/min/mo, and that an increase......Our longitudinal study of urinary albumin excretion rate in long-term insulin-dependent diabetics without proteinuria (negative albustix) suggests that early detection of patients at high and low risk of developing persistent proteinuria, i.e., diabetic nephropathy, is possible by using a sensitive...

Obstructive Sleep Apnea

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... find out more. Obstructive Sleep Apnea (OSA) Obstructive Sleep Apnea (OSA) Obstructive sleep apnea (OSA) is a ... find out more. Obstructive Sleep Apnea (OSA) Obstructive Sleep Apnea (OSA) Obstructive sleep apnea (OSA) is a ...

ACE Insertion/Deletion Polymorphism and Diabetic Nephropathy: Clinical Implications of Genetic Information

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Sung-Kyu Ha

2014-01-01

Full Text Available Approximately 20–40% of diabetic patients develop nephropathy which is the leading cause of ESRD in developed countries. The ACE I/D polymorphism is thought to be a marker for functional polymorphism which regulates circulating and tissue ACE activity. While the initial study found a protective effect of the II genotype on the development of nephropathy in IDDM patients, subsequent studies have addressed the role of ACE I/D polymorphism in the development and progression of diabetic nephropathy. RAAS blockers are the first line drugs for the treatment hypertension associated with diabetes and have been widely used in everyday clinical practice for the purpose of reducing proteinuria in patients with various renal diseases. However, the antiproteinuric effect of RAAS blockers is variable and the percentage of reducing proteinuria is in the range of 20–80%. The antiproteinuric effect of RAAS blockers may be related to a number of factors: the type or the dose of RAAS blockers, the duration of therapy, the level of sodium intake, and the type of patient’s ACE I/D genotype. Besides the nongenetic factors, drug responses, can be influenced by ACE gene polymorphism. In this review, we discuss the relationship between ACE I/D polymorphism and diabetic nephropathy and therapeutic response of RAAS blockers.

Obstructive Sleep Apnea

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Full Text Available ... find out more. Obstructive Sleep Apnea (OSA) Obstructive Sleep Apnea (OSA) Obstructive sleep apnea (OSA) is a ... find out more. Obstructive Sleep Apnea (OSA) Obstructive Sleep Apnea (OSA) Obstructive sleep apnea (OSA) is a ...

Potential mechanisms behind contrast medium-induced nephropathy

African Journals Online (AJOL)

How contrast medium-induced nephropathy (CIN) comes about is poorly understood, although CIN is a common cause of acute renal failure. Hitherto, the various studies performed have led to different interpretations and partially contradictory conclusions. This article aimed to review the mechanisms underlying CIN and to ...

A Mass of Pancreatic and Gastric Heterotopia Causing a Small Bowel Obstruction in a 61-Year-Old Male

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Majd Alfrejat

2017-01-01

Full Text Available Heterotopic tissue is a congenital anomaly that has been previously reported. Gastric and pancreatic heterotopia are among the most studied ones. Herein, we describe a case of a combined pancreatic and gastric heterotopia that formed a mass and caused a small intestine obstruction in a 61-year-old male. We also did a brief literature review of cases with gastric and pancreatic heterotopia in adult patients.

Aristolochic acid nephropathy: Harbinger of a global iatrogenic disease.

Science.gov (United States)

Grollman, Arthur P

2013-01-01

This review constitutes an overview of our investigations of aristolochic acid nephropathy, a chronic kidney disease associated with carcinomas of the upper urinary tract. Our studies began by confirming the hypothesis that chronic dietary poisoning by aristolochic acid was responsible for endemic (Balkan) nephropathy. A unique TP53 mutational signature in urothelial tumors and the presence of aristolactam-DNA adducts in the renal cortex, defined in the course of this research, proved to be robust biomarkers of exposure to this potent nephrotoxin and human carcinogen. Armed with this information, we used molecular epidemiologic approaches and novel mechanistic information to establish the causative role of aristolochic acid in upper urinary tract carcinoma in Taiwan, where one-third of the population had been prescribed herbal remedies containing Aristolochia, and the recorded incidence of upper urinary tract cancers is the highest in the world. As traditional Chinese medicine is practiced similarly in Taiwan and China, it is likely that upper urinary tract carcinomas and their attendant aristolochic acid nephropathy are prevalent in China and other Asian countries where Aristolochia herbs have been used for centuries in the treatment and prevention of disease, creating a potential public health problem of considerable magnitude. Copyright © 2012 Wiley Periodicals, Inc.

Diabetic Nephropathy in Women With Preexisting Diabetes

DEFF Research Database (Denmark)

Ringholm, Lene; Damm, Julie Agner; Vestgaard, Marianne

2016-01-01

In women with preexisting diabetes and nephropathy or microalbuminuria, it is important to deliver careful preconception counselling to assess the risk for the mother and the foetus, for optimizing glycaemic status and to adjust medical treatment. If serum creatinine is normal in early pregnancy,....... Supplementation with folic acid in early pregnancy and low-dose aspirin from 10 to 12 weeks reduces the risk of adverse pregnancy outcomes. During breastfeeding, several ACE inhibitors are considered safe.......In women with preexisting diabetes and nephropathy or microalbuminuria, it is important to deliver careful preconception counselling to assess the risk for the mother and the foetus, for optimizing glycaemic status and to adjust medical treatment. If serum creatinine is normal in early pregnancy......, kidney function is often preserved during pregnancy, but complications such as severe preeclampsia and preterm delivery are still common. Perinatal mortality is now comparable with that in women with diabetes and normal kidney function. Besides strict glycaemic control before and during pregnancy, early...

Changes of plasma levels of homocysteine (Hcy) and urinary albumin contents in patients with type 2 diabetes complicated with nephropathy

International Nuclear Information System (INIS)

Song Lili

2010-01-01

Objective: To study the changes of plasma levels of homocysteine (Hcy) and urinary albumin contents in patients with type 2 diabetes complicated with nephropathy. Methods: Plasma Hcy (with fluorescence immunoassay) fasting glucose, BUN, Cr (with biochemistry) levels and urinary albumin contents (with RIA) were determined in 36 DM2 patients without nephropathy, 30 DM2 patients with nephropathy and 30 controls. Results: The fasting blood glucose levels in the 2 groups of diabetic patients were not much different. Again, the BUN and Cr levels in the 3 groups of patients were about the same. The plasma Hcy levels in the group of patients with diabetic nephropathy were significantly higher than those in both controls and DM2 patients without nephropathy (all P<0.01). Conclusion: Hyperhomocysteinemia is a risk factor for nephropathy in DM2 patients. (authors)

Lack of serologic evidence to link IgA nephropathy with celiac disease or immune reactivity to gluten.

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Sina Moeller

Full Text Available IgA nephropathy is the most common form of primary glomerulonephritis worldwide. Mucosal infections and food antigens, including wheat gluten, have been proposed as potential contributing environmental factors. Increased immune reactivity to gluten and/or association with celiac disease, an autoimmune disorder triggered by ingestion of gluten, have been reported in IgA nephropathy. However, studies are inconsistent about this association. We aimed to evaluate the proposed link between IgA nephropathy and celiac disease or immune reactivity to gluten by conducting a comprehensive analysis of associated serologic markers in cohorts of well-characterized patients and controls. Study participants included patients with biopsy-proven IgA nephropathy (n = 99, unaffected controls of similar age, gender, and race (n = 96, and patients with biopsy-proven celiac disease (n = 30. All serum specimens were tested for IgG and IgA antibodies to native gliadin and deamidated gliadin, as well as IgA antibody to transglutaminase 2 (TG2. Anti-TG2 antibody-positive nephropathy patients and unaffected controls were subsequently tested for IgA anti-endomysial antibody and genotyped for celiac disease-associated HLA-DQ2 and -DQ8 alleles. In comparison to unaffected controls, there was not a statistically significant increase in IgA or IgG antibody reactivity to gliadin in individuals with IgA nephropathy. In addition, the levels of celiac disease-specific serologic markers, i.e., antibodies to deamidated gliadin and TG2, did not differ between IgA nephropathy patients and unaffected controls. Results of the additional anti-endomysial antibody testing and HLA genotyping were corroborative. The data from this case-control study do not reveal any evidence to suggest a significant role for celiac disease or immune reactivity to gluten in IgA nephropathy.

Lack of serologic evidence to link IgA nephropathy with celiac disease or immune reactivity to gluten.

Science.gov (United States)

Moeller, Sina; Canetta, Pietro A; Taylor, Annette K; Arguelles-Grande, Carolina; Snyder, Holly; Green, Peter H; Kiryluk, Krzysztof; Alaedini, Armin

2014-01-01

IgA nephropathy is the most common form of primary glomerulonephritis worldwide. Mucosal infections and food antigens, including wheat gluten, have been proposed as potential contributing environmental factors. Increased immune reactivity to gluten and/or association with celiac disease, an autoimmune disorder triggered by ingestion of gluten, have been reported in IgA nephropathy. However, studies are inconsistent about this association. We aimed to evaluate the proposed link between IgA nephropathy and celiac disease or immune reactivity to gluten by conducting a comprehensive analysis of associated serologic markers in cohorts of well-characterized patients and controls. Study participants included patients with biopsy-proven IgA nephropathy (n = 99), unaffected controls of similar age, gender, and race (n = 96), and patients with biopsy-proven celiac disease (n = 30). All serum specimens were tested for IgG and IgA antibodies to native gliadin and deamidated gliadin, as well as IgA antibody to transglutaminase 2 (TG2). Anti-TG2 antibody-positive nephropathy patients and unaffected controls were subsequently tested for IgA anti-endomysial antibody and genotyped for celiac disease-associated HLA-DQ2 and -DQ8 alleles. In comparison to unaffected controls, there was not a statistically significant increase in IgA or IgG antibody reactivity to gliadin in individuals with IgA nephropathy. In addition, the levels of celiac disease-specific serologic markers, i.e., antibodies to deamidated gliadin and TG2, did not differ between IgA nephropathy patients and unaffected controls. Results of the additional anti-endomysial antibody testing and HLA genotyping were corroborative. The data from this case-control study do not reveal any evidence to suggest a significant role for celiac disease or immune reactivity to gluten in IgA nephropathy.

Congenital Pyloric Atresia; a report of two cases

International Nuclear Information System (INIS)

Tayeb, Maaen; Khogeer, Suzie; Fallatah, Amna; Hamchou, Mustafa A.

2005-01-01

Congenital pyloric atresia (CPA) is a very rare malformation that constitutes less than 1% of all gas upper gastrointestinal atresias. It is a unique malformation that is commonly seen as an isolated lesion, but can also occur in association with other genetically determined conditions such as hereditary multiple intestinal atresias (HMIA). This is a report of two cases of isolated isolated CPA, outlining aspects of diagnosis and management. A 2-day-old female, a product of 35-weeks gestation via a low cesarean delivery due to a transverse lie to a 25-year-old mother who had gestational insulin dependent diabetes and polyhydraminos was referred because of non-bile stained vomiting. Her abdominal x-ray showed dilated stomach with no gas distally. Gastrograffin meal confirmed the diagnosis of gastric outlet obstruction. She was found to have pyloric artesia. This was excised via longitudinal incision in the pylorus, which was then closed transversely. Subsequently she did well. A 1-year -old female was evaluated because of persistent non-bile stained vomiting. Abdominal x-ray showed dilated stomach with no gas distally and barium meal confirmed the diagnosis of gastric outlet obstruction. She was operated and on during surgery was found to have congenital pyloric atresia. This was excised via longitudinal incsion in the pylorus. She did well. CPA is divided in three types: 1) Pyloric membrane 2) Pyloric artesia without a gap 3) Pyloric artesia with a gap. Both our patients have pyloric diaphagrams. The treatment of CPA is surgical and depends on the type of arteseia. For those with pyloric diaphagram or pyloric artesia without a gap the treatment is excision of diphagaram. This is also of importance in case there is more than one diaphagram. For those with pyloric artesia with a gap, if the gap is short, they should be treated with a Finny or Heineke-Mickulicz pyloroplasty, but if the gap is long then a gastroduodenostomy becomes the treatment of choice

Association of dioxins, furans and dioxin-like PCBs in human blood with nephropathy among US teens and young adults.

Science.gov (United States)

Everett, Charles J; Thompson, Olivia M

2016-06-01

We assessed the association of three chlorinated dibenzo-p-dioxins, a chlorinated dibenzofuran, and four dioxin-like polychlorinated biphenyls (PCBs) in human blood with nephropathy (microalbuminuria or macroalbuminuria) among teens and young adults (12-30 years old) having normal glycohemoglobin (A1c Dioxins, furans and dioxin-like PCBs in human blood: causes or consequences of diabetic nephropathy? Environ Res 2014;132:126-31), the cut-offs for these chemicals being considered elevated, were defined as the 75th percentile. Using these same cut-offs again, the proportion of those with one or more of the eight dioxin-like compounds elevated was 9.9%. The four chemicals associated with nephropathy were 1,2,3,6,7,8-hexachlorodibenzo-p-dioxin, PCB 126, PCB 169, and PCB 156. The proportion with one or more of these four dioxin-like chemicals elevated was 3.9% (unweighted n=46) and the odds ratio (OR) for nephropathy was 7.1 [95% confidence interval (CI) 1.8-28.1]. The association was strong among females (OR 17.4, 95% CI 3.4-88.6), but among males there were no cases of nephropathy when one or more of the four dioxin-like chemicals were elevated, and therefore no association. In a separate analysis, elevated toxic equivalency, defined using the eight dioxin-like chemicals (TEQ8), was associated with nephropathy. TEQ8 ≥50.12 fg/g included 2.6% of the sample (unweighted n=28) and had an OR of 5.8 (95% 1.3-25.9) for nephropathy. As found in the analysis of one or more of four dioxin-like chemicals elevated, TEQ8 ≥50.12 fg/g was associated with nephropathy among females (OR 11.9, 95% CI 1.6-87.2), but not males. Trends for least-squares means also differed by gender, but there were no significant differences in mean TEQ8 between normal subjects and those having nephropathy in either males or females. We also evaluated pre-diabetes (A1c 5.7-6.4%) without nephropathy and found no associations when one or more of four dioxin-like compounds were elevated, or when TEQ8 was

Preventive Nephrology - Proposed Options in Childhood Nephropathy

African Journals Online (AJOL)

Three children with renal disorders managed at the University of Ilorin Teaching Hospital are reported as case studies to underscore the need for preventive nephrology . The first case illustrates the inevitability of rapidly progressive renal failure when remedial management desired in the early stages of the nephropathy is ...

Digital video subtraction fluorography (DVSF) in the diagnosis of bronchial abnormality associated with congenital heart diseases

International Nuclear Information System (INIS)

Sano, Tetsuya; Arisawa, Jun; Nakajima, Toru

1990-01-01

To assess bronchial morphology and abnormality, 14 children with congenital heart diseases including 2 postoperative patients (age, 2 m.-4 yr) were studied by digital video subtraction fluorography (DVSF) using digital subtraction and image processing system (Philips, DVI-2). This newly developed technique clearly defined bronchial anatomy in all 14 patients. Bronchial situs could be determined in all 8 patients with complex heart anomalies. Out of 8 patients with respiratory distress in this study, obvious bronchial stenosis or obstruction was found by DVSF in 5 patients. Thus, DVSF image defined anatomies of main and lobar bronchi more clearly than previous noninvasive methods. Moreover, DVSF is noninvasive and easily performed even for small infants and critically ill patients. In conclusion, DVSF may be a useful technique to assess bronchial morphology and abnormality in patients with congenital heart diseases. (author)

Impaired autoregulation of glomerular filtration rate in type 1 (insulin-dependent) diabetic patients with nephropathy

DEFF Research Database (Denmark)

Parving, H H; Kastrup, Helge; Smidt, U M

1984-01-01

The effect of acute lowering of arterial blood pressure upon kidney function in nephropathy was studied in 13 patients with long-term Type 1 (insulin-dependent) diabetes. Ten normal subjects (six normotensive and four hypertensive) and five short-term Type 1 diabetic patients without nephropathy...

Coffin-Siris syndrome with the rarest constellation of congenital cardiac defects: A case report with review of literature

OpenAIRE

Nemani, Lalita; Barik, Ramachandra; Patnaik, Amar Narayana; Mishra, Ramesh C; Rao, Amaresh M; Kapur, Pragati

2014-01-01

We report a case of type-A Coffin-Siris syndrome (CSS) with a unique constellation of congenital heart defects. A 17-year-old Indian boy was referred to our hospital for central cyanosis with features of right heart failure. The cardiac abnormalities included biventricular outflow tract obstruction, small atrial septal defect (ASD), subaortic ventricular septal defect, drainage of left superior venacava to left atrial appendage, and aortic arch anomaly. Patient underwent successful right vent...

Neuroimaging findings in neonates and infants from superior vena cava obstruction after cardiac operation

International Nuclear Information System (INIS)

Karmazyn, Boaz; Horev, Gadi; Kornreich, Liora; Dagan, Ovdi; Vidne, Bernado A.

2002-01-01

Extraventricular obstructive hydrocephalus may develop after superior vena cava obstruction, an uncommon complication after cardiac surgery.Objective. To describe the neuroimaging findings in neonates and infants with superior vena cava thrombosis after cardiac surgery for congenital heart disease.Materials and methods. Between 1993 and 2001, 333 neonates and infants in our hospital underwent cardiac surgery, of whom 13 (3.9%) subsequently acquired superior vena cava syndrome. Eleven of these 13 children (7 boys, 4 girls) were evaluated by head ultrasound and computed tomography scans.Results. One child had normal findings on head ultrasound, and 10 children had extraventricular obstructive hydrocephalus (EVOH). In 6 children, aggravation of the hydrocephalus was noted up to 11.4 months after cardiac surgery; in 3 of them, the hydrocephalus was shunted to the peritoneum. One child had thrombosis of the dural sinuses, and 1 had hemorrhagic infarction. Two children died during follow-up.Conclusion. EVOH is a common complication of superior vena cava thrombosis, and head ultrasound should be performed in all neonates and infants with superior vena cava thrombosis after cardiac surgery. Long-term follow-up is needed, as the hydrocephalus may worsen even months after surgery. (orig.)

Fish Oil in Diabetic Nephropathy

DEFF Research Database (Denmark)

Rossing, Peter; Hansen, Birgitte V.; Nielsen, Flemming S.

1996-01-01

OBJECTIVE: Recent studies in nondiabetic kidney diseases suggest that dietary supplementation with n-3 polyunsaturated fatty acids (fish oil) may have beneficial effects on albuminuria, kidney function, arterial blood pressure, and dyslipidemia. Therefore, we evaluated the long-term effect of fish...... in the fish oil compared with the placebo group. CONCLUSIONS: Our study does not suggest that fish oil has beneficial effects on albuminuria, kidney function, blood pressure, and dyslipidemia in normotensive IDDM patients suffering from diabetic nephropathy....

Prevalence and Risk Factors of Diabetic Nephropathy in Omani Type 2 Diabetics in Al-Dakhiliyah Region

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Abdulhakeem Hamood Alrawahi

2012-05-01

Full Text Available Objective: To assess the prevalence and risk factors of diabetic nephropathy among Omani type 2 diabetics in Al-Dakhiliyah region of the Sultanate of Oman.Methods: A cross-sectional and a case control study designs were used to assess the prevalence and risk factors respectively. For the prevalence study a sample of 699 diabetic subjects were selected randomly from two polyclinics in Al-Dakhiliyah region; Sumail and Nizwa polyclinics. For the case control study, a sample consisting of 215 cases and 358 controls were randomly selected from those who were included in the cross-sectional study. A well designed questionnaire has been used to collect data regarding the disease and risk factors. Data was analyzed using SPSS19 statistical program.Results: Total prevalence of diabetic nephropathy was calculated as 42.5% (95% C.I: 38.83% - 46.15%. The difference in the prevalence in the two polyclinic catchment area was not significant. The prevalence was significantly higher among males (51.6% compared to females (36.5%. Crude analysis of the risk factors showed significant association between diabetic nephropathy and the following factors; male gender, decreased literacy, long duration of diabetes mellitus, hypertension, retinopathy, neuropathy, family history of diabetic nephropathy, poor glycemic control (high HbA1c, and hypertriglyceridemia. Multivariate analysis showed the following factors to be independent risk factors; male gender, decreased literacy, long duration of diabetes, family history of diabetic nephropathy and poor glycaemic control (high HbA1c.Conclusion: The prevalence of diabetic nephropathy in this study was 42.5% and the significant risk factors associated with it included male gender, decreased literacy, long duration of diabetes, family history of diabetic nephropathy and poor glycemic control (high HbA1c.

Congenital heart defects in molecularly proven Kabuki syndrome patients.

Science.gov (United States)

Digilio, Maria Cristina; Gnazzo, Maria; Lepri, Francesca; Dentici, Maria Lisa; Pisaneschi, Elisa; Baban, Anwar; Passarelli, Chiara; Capolino, Rossella; Angioni, Adriano; Novelli, Antonio; Marino, Bruno; Dallapiccola, Bruno

2017-11-01

The prevalence of congenital heart defects (CHD) in Kabuki syndrome ranges from 28% to 80%. Between January 2012 and December 2015, 28 patients had a molecularly proven diagnosis of Kabuki syndrome. Pathogenic variants in KMT2D (MLL2) were detected in 27 patients, and in KDM6A gene in one. CHD was diagnosed in 19/27 (70%) patients with KMT2D (MLL2) variant, while the single patient with KDM6A change had a normal heart. The anatomic types among patients with CHD included aortic coarctation (4/19 = 21%) alone or associated with an additional CHD, bicuspid aortic valve (4/19 = 21%) alone or associated with an additional CHD, perimembranous subaortic ventricular septal defect (3/19 = 16%), atrial septal defect ostium secundum type (3/19 = 16%), conotruncal heart defects (3/19 = 16%). Additional CHDs diagnosed in single patients included aortic dilatation with mitral anomaly and hypoplastic left heart syndrome. We also reviewed CHDs in patients with a molecular diagnosis of Kabuki syndrome reported in the literature. In conclusion, a CHD is detected in 70% of patients with KMT2D (MLL2) pathogenic variants, most commonly left-sided obstructive lesions, including multiple left-sided obstructions similar to those observed in the spectrum of the Shone complex, and septal defects. Clinical management of Kabuki syndrome should include echocardiogram at the time of diagnosis, with particular attention to left-sided obstructive lesions and mitral anomalies, and annual monitoring for aortic arch dilatation. © 2017 Wiley Periodicals, Inc.

Prevention of incipient diabetic nephropathy by high-dose thiamine and benfotiamine.

Science.gov (United States)

Babaei-Jadidi, Roya; Karachalias, Nikolaos; Ahmed, Naila; Battah, Sinan; Thornalley, Paul J

2003-08-01

Accumulation of triosephosphates arising from high cytosolic glucose concentrations in hyperglycemia is the trigger for biochemical dysfunction leading to the development of diabetic nephropathy-a common complication of diabetes associated with a high risk of cardiovascular disease and mortality. Here we report that stimulation of the reductive pentosephosphate pathway by high-dose therapy with thiamine and the thiamine monophosphate derivative benfotiamine countered the accumulation of triosephosphates in experimental diabetes and inhibited the development of incipient nephropathy. High-dose thiamine and benfotiamine therapy increased transketolase expression in renal glomeruli, increased the conversion of triosephosphates to ribose-5-phosphate, and strongly inhibited the development of microalbuminuria. This was associated with decreased activation of protein kinase C and decreased protein glycation and oxidative stress-three major pathways of biochemical dysfunction in hyperglycemia. Benfotiamine also inhibited diabetes-induced hyperfiltration. This was achieved without change in elevated plasma glucose concentration and glycated hemoglobin in the diabetic state. High-dose thiamine and benfotiamine therapy is a potential novel strategy for the prevention of clinical diabetic nephropathy.

Radon inhalation suppresses nephropathy in streptozotocin-induced type-1 diabetic mice

International Nuclear Information System (INIS)

Nishiyama, Yuichi; Kataoka, Takahiro; Yamato, Keiko; Etani, Reo; Taguchi, Takehito; Yamaoka, Kiyonori

2016-01-01

In this study, we investigated the suppressive effects of radon inhalation against nephropathy in C57BL/6J mice with type-1 diabetes induced by intraperitoneal injection of streptozotocin (50 mg/kg weight, given five times). Four weeks after diabetes induction, the diabetic mice were continuously treated with inhaled radon-222 of 2000 Bq/m3 or air only (sham) for four weeks. The results showed that radon inhalation did not affect type-1 diabetic symptoms such as body weight loss, hyperglycemia, and hypoinsulinemia. However, diabetic mice treated with radon showed lower urinary albumin excretion and fibrotic change in renal glomeruli compared with diabetic mice not treated with radon. Furthermore, renal superoxide dismutase activity and glutathione content were significantly higher in diabetic mice treated with radon than in diabetic mice not treated with radon. These findings suggested that radon inhalation enhanced renal antioxidants activities, resulting in the suppression of diabetic nephropathy. This study may contribute to the development of a novel approach in the treatment of nephropathy for diabetic patients. (author)

Endoscopic placement of ureteral stents for treatment of congenital bilateral ureteral stenosis in a dog.

Science.gov (United States)

Lam, Nathaniel K; Berent, Allyson C; Weisse, Chick W; Bryan, Christine; Mackin, Andrew J; Bagley, Demetrius H

2012-04-15

A 5-year-old 8.6-kg (18.9-lb) spayed female Pug was evaluated because of chronic hematuria and recurrent urinary tract infections. Excretory urography, ultrasonography, and excretory CT urography were performed. Results indicated that the dog had bilateral hydronephrosis and hydroureter and suspected proximal ureteral stenosis. Retrograde ureteropyelography confirmed the presence of stenosis at the ureteropelvic junction of each ureter, along with a large amount of endoluminal ureteral debris. Clinical findings suggested that the dog had a congenital bilateral anomaly of the upper urinary tract. The dog was anesthetized, and 2 double-pigtail ureteral stents were placed cystoscopically with fluoroscopic guidance for immediate relief of the ureteropelvic junction obstructions. Each stent extended from the left or right renal pelvis to the urinary bladder. The procedures and the patient's recovery from anesthesia were uncomplicated. Continuing improvements in severity of hydronephrosis, hydroureter, and dysuria were evident during routine follow-up examinations at 2, 4, 12, 16, and 45 weeks after stent placement. Over the subsequent 12 months, all clinical signs remained resolved other than a urinary tract infection that was successfully treated with antimicrobials. Ureteral stenosis should be considered as a differential diagnosis for hydronephrosis in dogs, particularly when urinary tract calculi or neoplasia is not present. Chronic hematuria and recurrent urinary tract infections can be associated with this condition. Placement of ureteral stents may be a successful treatment option for ameliorization of congenital ureteral obstructions.

Comprehensive approach to diabetic nephropathy

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Bancha Satirapoj

2014-09-01

Full Text Available Diabetic nephropathy (DN is a leading cause of mortality and morbidity in patients with diabetes. This complication reflects a complex pathophysiology, whereby various genetic and environmental factors determine susceptibility and progression to end-stage renal disease. DN should be considered in patients with type 1 diabetes for at least 10 years who have microalbuminuria and diabetic retinopathy, as well as in patients with type 1 or type 2 diabetes with macroalbuminuria in whom other causes for proteinuria are absent. DN may also present as a falling estimated glomerular filtration rate with albuminuria as a minor presenting feature, especially in patients taking renin–angiotensin–aldosterone system inhibitors (RAASi. The pathological characteristic features of disease are three major lesions: diffuse mesangial expansion, diffuse thickened glomerular basement membrane, and hyalinosis of arterioles. Functionally, however, the pathophysiology is reflected in dysfunction of the mesangium, the glomerular capillary wall, the tubulointerstitium, and the vasculature. For all diabetic patients, a comprehensive approach to management including glycemic and hypertensive control with RAASi combined with lipid control, dietary salt restriction, lowering of protein intake, increased physical activity, weight reduction, and smoking cessation can reduce the rate of progression of nephropathy and minimize the risk for cardiovascular events. This review focuses on the latest published data dealing with the mechanisms, diagnosis, and current treatment of DN.

Endothelin receptor a blockade is an ineffective treatment for adriamycin nephropathy.

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Roderick J Tan

Full Text Available Endothelin is a vasoconstricting peptide that plays a key role in vascular homeostasis, exerting its biologic effects via two receptors, the endothelin receptor A (ETA and endothelin receptor B (ETB. Activation of ETA and ETB has opposing actions, in which hyperactive ETA is generally vasoconstrictive and pathologic. Selective ETA blockade has been shown to be beneficial in renal injuries such as diabetic nephropathy and can improve proteinuria. Atrasentan is a selective pharmacologic ETA blocker that preferentially inhibits ETA activation. In this study, we evaluated the efficacy of ETA blockade by atrasentan in ameliorating proteinuria and kidney injury in murine adriamycin nephropathy, a model of human focal segmental glomerulosclerosis. We found that ETA expression was unaltered during the course of adriamycin nephropathy. Whether initiated prior to injury in a prevention protocol (5 mg/kg/day, i.p. or after injury onset in a therapeutic protocol (7 mg/kg or 20 mg/kg three times a week, i.p., atrasentan did not significantly affect the initiation and progression of adriamycin-induced albuminuria (as measured by urinary albumin-to-creatinine ratios. Indices of glomerular damage were also not improved in atrasentan-treated groups, in either the prevention or therapeutic protocols. Atrasentan also failed to improve kidney function as determined by serum creatinine, histologic damage, and mRNA expression of numerous fibrosis-related genes such as collagen-I and TGF-β1. Therefore, we conclude that selective blockade of ETA by atrasentan has no effect on preventing or ameliorating proteinuria and kidney injury in adriamycin nephropathy.

Total saponin of Dioscoreae hypoglaucae rhizoma ameliorates streptozotocin-induced diabetic nephropathy

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Guo C

2016-02-01

Full Text Available Changrun Guo,1 Gang Ding,2 Wenzhe Huang,2 Zhenzhong Wang,2 Zhaoqing Meng,1,2 Wei Xiao2 1State Key Laboratory of Natural Medicines, China Pharmaceutical University, Nanjing, People’s Republic of China; 2Jiangsu Kanion Pharmaceutical Co. Ltd, Lianyungang City, People’s Republic of China Background: Diabetic nephropathy has become the most common cause of morbidity and mortality in diabetic patients. Therefore, there is an urgent need for more effective and safer drugs for use in this condition.Purpose: The aims of this study were to investigate the ameliorative effects of total saponin of Dioscoreae hypoglaucae rhizoma (TSD on diabetic nephropathy and to explore the potential underlying mechanism(s.Methods: Rats with streptozotocin-induced diabetes were orally treated with TSD at 40, 80, and 160 mg/kg/d for 12 weeks. At the end of the treatment, blood, urine, and kidneys were collected for biochemical and histological examination.Results: The results demonstrated that TSD significantly decreased the fasting blood glucose, glycosylated hemoglobin, urinary protein, serum creatinine, and blood urea nitrogen levels in diabetic rats. The results of histological examinations showed that TSD ameliorated glomerular and tubular pathological changes in diabetic rats. Furthermore, TSD significantly prevented oxidative stress and reduced the renal levels of advanced glycation end products, transforming growth factor-β1, connective tissue growth factor, and tumor necrosis factor-α.Conclusion: This study demonstrated the renoprotective effects of TSD in experimental diabetic nephropathy via a number of different mechanisms. Keywords: total saponin of Dioscoreae hypoglaucae rhizoma, diabetic nephropathy, oxidative stress, AGEs, TGF-β1

BK Virus-Associated Nephropathy without Viremia in an Adolescent Kidney Transplant Recipient

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Kraisoon Lomjansook, M.D.

2017-09-01

Full Text Available BK virus can reactivate in kidney transplant recipients leading to BK virus-associated nephropathy (BKVAN and allograft dysfunction. Pathogenesis begins with viral replication, follows by viruria, viremia and nephropathy. Screening tools recommended for viral detection are urine and blood BK viral load. Viremia has higher positive predictive value than viruria, thus several guidelines recommend using viremia to determine whether renal biopsy, a gold standard for diagnosis of BKVAN is needed. We present a 16-year-old boy who developed BKVAN five months after deceased donor kidney transplantation. He had increased serum creatinine with negative blood BK viral load. BK nephropathy was diagnosed in kidney graft biopsy. The urine showed BK viruria. Immunosuppressant was reduced and ciprofloxacin given. Viruria disappeared and repeated graft biopsy was normal 4 months later. BK viremia was negative through 1 year follow up. We conclude that BKVAN may occur even without viremia and BK viruria may be considered for screening tool.

Clinical and pathological analysis of IgA nephropathy with chronic renal failure.

Science.gov (United States)

Liu, Yuyuan; Hu, Qinfeng; Shen, Ping; Tang, Li; Yuan, Gang; Zhou, Yongmei; Chai, Huaqi

2016-10-01

To investigative clinical and pathological characteristics of IgA nephropathy with chronic renal failure. Clinical and pathological findings from 65 cases of IgA nephropathy with chronic renal failure were reviewed. Pathological characteristics of all the cases were analyzed according to WHO definition and Oxford Classification. Evaluating the severity of pathological lesions by the Katafuchi R semiquantitative scoring system, and analyzing their relationship with clinical indexes of renal function. Of all 65 cases the male and female ratio was 1.4, and the mean age was 37 ± 13 years old. Levels of systolic pressure, mean arterial pressure (MAP), blood urea nitrogen (BUN), serum creatinine (Scr), uric acid (UA), album (Alb), serum IgG and 24 h urinary protein were related with eGRF level (p  0.05). IgA nephropathy with chronic renal failure usually occurred in young adults, and it had severe clinical condition and pathological changes, while there was no significant relationship between them.

Encountering Meckel's diverticulum in emergency surgery for ascaridial intestinal obstruction

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Amin Abid

2010-06-01

Full Text Available Abstract Background Meckel's diverticulum is the most common congenital anomaly of the gastrointestinal tract. In children with intestinal ascariasis, the diverticulum remains asymptomatic or rarely the Ascaris lumbricoides may lead to its complications in the presence of massive intestinal roundworm load. Given that preoperative diagnosis is seldom carried out, when Meckel's diverticulum is found at laparotomy for obstructive intestinal complications of roundworm, the diverticulum should be removed as complications may occur at any time. The aim of this study was to describe the findings of concomitant presence of Meckel's diverticulum who had surgical intervention in symptomatic intestinal ascariasis in children. Methods A retrospective case review study of 14 children who had surgical intervention for symptomatic intestinal ascariasis having the presence of concomitant Meckel's diverticulum was done. The study was done at SMHS Hospital Srinagar, Kashmir. Results A total of the 14 children who had ascaridial intestinal obstruction with concomitant presence of Meckel's diverticulum were studied. Age of children ranged from 4-12 years, male:female ratio was 1.8:1. Nine patients had asymptomatic Meckel's diverticulum, whereas 5 patients with symptomatic signs were found in the course of emergency surgery for ascaridial intestinal obstruction. Conclusion Meckel's diverticulum in intestinal ascariasis may pursue silent course or may be accompanied with complications of the diverticulitis, perforation or the gangrene. Incidental finding of the Meckel's diverticulum in the intestinal ascariasis should have removal.

Chemical substances as risk factors of nephropathy in diabetes mellitus

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Zofia Marchewka

2009-12-01

Full Text Available Although diabetes mellitus, a metabolic disease, does not fall into the group of diseases induced by toxic substances or environmental pollution, there is much evidence that some chemicals have considerable importance in its development. Exposure to substances with potential renal toxicity is especially dangerous for diabetics because it accelerates and intensifies diabetic nephropathy. This paper discusses the relationship between the xenobiotics and the development of diabetes mellitus and diabetic nephropathy with particular emphasis on those substances that causes the greatest damage to the kidneys. These are cadmium, iron, lead, arsenic, polychlorinated organic compounds, nitrogen compounds, and contrast agents. In addition, the mechanisms of diabetes mellitus induction or kidney damage by these xenobiotics are described.

Oxidative Stress in Diabetic Nephropathy with Early Chronic Kidney Disease

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Alejandra Guillermina Miranda-Díaz

2016-01-01

Full Text Available The increase in the prevalence of diabetes mellitus (DM and the secondary kidney damage produces diabetic nephropathy (DN. Early nephropathy is defined as the presence of microalbuminuria (30–300 mg/day, including normal glomerular filtration rate (GFR or a mildly decreased GFR (60–89 mL/min/1.73 m2, with or without overt nephropathy. The earliest change caused by DN is hyperfiltration with proteinuria. The acceptable excretion rate of albumin in urine is 300 mg/day. Chronic kidney disease (CKD is characterized by abnormalities in renal function that persist for >3 months with health implications. Alterations in the redox state in DN are caused by the persistent state of hyperglycemia and the increase in advanced glycation end products (AGEs with ability to affect the renin-angiotensin system and the transforming growth factor-beta (TGF-β, producing chronic inflammation and glomerular and tubular hypertrophy and favoring the appearance of oxidative stress. In DN imbalance between prooxidant/antioxidant processes exists with an increase in reactive oxygen species (ROS. The overproduction of ROS diminishes expression of the antioxidant enzymes (manganese superoxide dismutase, glutathione peroxidase, and catalase. The early detection of CKD secondary to DN and the timely identification of patients would permit decreasing its impact on health.

Prevalence of recurrent urinary tract infection in children with congenital anomalies of the kidney and urinary tract (CAKUT)

Science.gov (United States)

Ramayani, O. R.; Eyanoer, P. C.; Ritarwan, K.; Siregar, B.; Siregar, R. S.

2018-03-01

Prevalence of congenital abnormalities varies from 3.5% up to 43% in pediatrics.This wide interval is due to limited numbers of research. Limitation of data on recurrent urinary tract infection in CAKUT infants as well as symptoms which resemble other diseases makes it quite a challenge. A study of the prospective cohort was established to analyze the prevalence of recurrent UTI among CAKUT in children at Neonatal and Nephrology Paediatric Department of H.Adam Malik Hospital from 2016 to 2017.Urinalysis and urine culture were used to assess the presence of UTI. The result showed that the prevalence of UTI in CAKUT patients reached 64% in which 52% is the obstructive type and 12% non-obstructive type. Pelvic ureteric junction obstruction is the most common cause. Children with known urinary tract problems such as CAKUT are very prone to developing recurrent UTI. Due to a low survival rate of children with ESKD, new strategies are needed to prevent CAKUT, preserve renal function, and reduce associated cardiovascular morbidity. Meanwhile, children with CAKUT requires a multi-disciplinary and longer follow up.

Vorapaxar treatment reduces mesangial expansion in streptozotocin-induced diabetic nephropathy in mice.

Science.gov (United States)

Waasdorp, Maaike; Duitman, JanWillem; Florquin, Sandrine; Spek, C Arnold

2018-04-24

Twenty years after the onset of diabetes, up to 40% of patients develop diabetic nephropathy. Protease-activated receptor-1 (PAR-1) has recently been shown to aggravate the development of experimental diabetic nephropathy. PAR-1 deficient mice develop less albuminuria and glomerular lesions and PAR-1 stimulation induces proliferation and fibronectin production in mesangial cells in vitro . Vorapaxar is a clinically available PAR-1 inhibitor which is currently used for secondary prevention of ischemic events. The aim of this study was to investigate in a preclinical setting whether vorapaxar treatment may be a novel strategy to reduce diabetes-induced kidney damage. While control treated diabetic mice developed significant albuminuria, mesangial expansion and glomerular fibronectin deposition, diabetic mice on vorapaxar treatment did not show any signs of kidney damage despite having similar levels of hyperglycemia. These data show that PAR-1 inhibition by vorapaxar prevents the development of diabetic nephropathy in this preclinical animal model for type I diabetes and pinpoint PAR-1 as a novel therapeutic target to pursue in the setting of diabetic nephropathy. 22 C57Bl/6 mice were made diabetic using multiple low-dose streptozotocin injections (50 mg/kg) and 22 littermates served as non-diabetic controls. Four weeks after the induction of diabetes, 11 mice of each group were assigned to control or vorapaxar treatment. Mice were sacrificed after 20 weeks of treatment and kidney damage was evaluated.

Uric acid as a mediator of diabetic nephropathy

DEFF Research Database (Denmark)

Jalal, Diana I; Maahs, David M; Hovind, Peter

2011-01-01

Despite advances in the management of patients with diabetes, diabetic nephropathy (DN) remains the most common cause of end-stage renal disease in the United States and worldwide. Inflammation and endothelial dysfunction appear to play a central role in the onset and the progression of DN. Recen...

Transient mega-esophagus in a neonate with congenital diaphragmatic hernia

International Nuclear Information System (INIS)

Makhoul, I.R.; Smolkin, T.; Sujov, P.; Shoshany, G.; Epelman, M.

2001-01-01

Esophageal dilatation (ED) in neonates is rare. In the present case, ED was detected in a chest radiograph following repair of congenital diaphragmatic hernia (CDH) in a term neonate. A roentgenographic swallow study on the seventh day of life demonstrated ED and a sub-diaphragmatic stomach. The infant thrived adequately on enteral feeding. A swallow study on the twentieth day of life showed a normal-width esophagus with gastroesophageal reflux and small hiatus hernia. The longstanding herniated stomach in the fetus apparently caused kinking, edema, and obstruction of the gastroesophageal junction. This led to a significant ED and concealment of gastroesophageal reflux. We aim to arouse awareness about the occurrence of ED with CDH, and about its benign course under conservative management. (orig.)

Transient mega-esophagus in a neonate with congenital diaphragmatic hernia

Energy Technology Data Exchange (ETDEWEB)

Makhoul, I.R.; Smolkin, T.; Sujov, P. [Dept. of Neonatology, Rambam Medical Center and Rappaport Faculty of Medicine, Technion, Haifa (Israel); Shoshany, G. [Dept. of Pediatric Surgery, Rambam Medical Center and Rappaport Faculty of Medicine, Technion, Bat-Galim, Haifa (Israel); Epelman, M. [Dept. of Radiology, Rambam Medical Center and Rappaport Faculty of Medicine, Technion, Bat-Galim, Haifa (Israel)

2001-05-01

Esophageal dilatation (ED) in neonates is rare. In the present case, ED was detected in a chest radiograph following repair of congenital diaphragmatic hernia (CDH) in a term neonate. A roentgenographic swallow study on the seventh day of life demonstrated ED and a sub-diaphragmatic stomach. The infant thrived adequately on enteral feeding. A swallow study on the twentieth day of life showed a normal-width esophagus with gastroesophageal reflux and small hiatus hernia. The longstanding herniated stomach in the fetus apparently caused kinking, edema, and obstruction of the gastroesophageal junction. This led to a significant ED and concealment of gastroesophageal reflux. We aim to arouse awareness about the occurrence of ED with CDH, and about its benign course under conservative management. (orig.)

Increased left ventricular mass in normotensive type 1 diabetic patients with diabetic nephropathy

DEFF Research Database (Denmark)

Sato, A; Tarnow, L; Parving, H H

1998-01-01

in normotensive type 1 diabetic patients with and without nephropathy. RESEARCH DESIGN AND METHODS: M-mode and Doppler echocardiography was performed in 17 type 1 diabetic patients with nephropathy (albuminuria [median (range)], 345 (135-2,846) mg/24 h) and compared with 34 normotensive, normoalbuminuric (10 [3......-30] mg/24 h) type 1 diabetic patients matched for arterial blood pressure (mean +/- SD) ([134/77] +/- [13/7] vs. [129/78] +/- [12/7] mmHg), age (40 +/- 11 vs. 42 +/- 10 years), duration of diabetes (28 +/- 7 vs. 28 +/- 6 years), and BMI (24.2 +/- 4.2 vs. 24.6 +/- 2.4 kg/m2). RESULTS: Left ventricular......OBJECTIVE: Diabetic nephropathy increases the risk of premature cardiovascular disease and sudden death, particularly in type 1 diabetic patients. One possible mechanism for this risk may be left ventricular hypertrophy. In our study, we aimed to evaluate left ventricular structure and function...

Effects of nisoldipine and lisinopril on left ventricular mass and function in diabetic nephropathy

DEFF Research Database (Denmark)

Tarnow, L; Sato, A; Ali, S

1999-01-01

hypertensive type 1 diabetic patients with diabetic nephropathy enrolled in a 1-year, randomized, double-blind, parallel study of antihypertensive treatment with nisoldipine CC (20-40 mg/day) or lisinopril (10-20 mg/day). Ambulatory 24-h blood pressure was measured with the Takeda TM 2420 device (A & D, Tokyo......, respectively, and did not change during follow-up. CONCLUSIONS: Antihypertensive treatment with nisoldipine or lisinopril to bring diastolic blood pressure level within the normal target range does not hinder a rise in LVMI in type 1 diabetic patients with diabetic nephropathy.......OBJECTIVE: To compare the effects of the calcium channel blocker, nisoldipine, and the ACE inhibitor, lisinopril, on left ventricular mass (LVM) and systolic function in type 1 diabetic patients with diabetic nephropathy. RESEARCH DESIGN AND METHODS: M-mode echocardiography was performed in 50...

Left ventricular hypertrophy in non-insulin-dependent diabetic patients with and without diabetic nephropathy

DEFF Research Database (Denmark)

Nielsen, F S; Ali, S; Rossing, P

1997-01-01

patients with normoalbuminuria (42 males, 61 +/- 7 years, group 2), and 22 non-diabetic control subjects (15 males, 58 +/- 8 years, group 3) were investigated. Previous antihypertensive treatment was withdrawn 2 weeks before the study. Left ventricular mass index (LVMI) and systolic function were......(-2), respectively (p prevalence of LVH was 42% (95% CI, 23-63) and 14% (95% CI, 2-43) (p = 0.07) in these two groups, respectively. In conclusion, normotensive and hypertensive NIDDM patients with and without diabetic nephropathy frequently suffer from LVH and relatively reduced......The aim of our cross-sectional case-control study was to evaluate putative mechanisms of the increased cardiac morbidity and mortality in NIDDM patients with or without diabetic nephropathy. Fifty-one NIDDM patients with diabetic nephropathy (38 males, age 61 +/- 8 years, group 1), 53 NIDDM...

MR imaging features of the congenital uterine anomalies

International Nuclear Information System (INIS)

Hamcan, S.; Akgun, V.; Battal, B.; Kocaoglu, M.

2012-01-01

Full text: Introduction: Congenital uterine anomalies are common and usually asymptomatic. The agenesis, malfusion or deficient resorption of the Mullerian canals during embryogenesis may lead to these anomalies. Although ultrasonography (US) is the first step imaging technique in assessment of the uterine pathologies, it can be insufficient in differentiation of them. Magnetic resonance (MR) imaging is an adequate imaging technique in depicting pelvic anatomy and different types of uterine anomalies. Objectives and tasks: In this article, we aimed to present imaging features of the uterine anomalies. Material and methods: Pelvic MR scans of the cases who were referred to our radiology department for suspicious uterine anomaly were evaluated retrospectively. Results: We determined uniconuate uterus (type II), uterus didelphys (type III), bicornuate uterus (type IV), uterine septum (type V) and arcuate uterus (type VI) anomalies according to ASRM (American Society of Reproductive Medicine) classification. Conclusion: In cases with such pathologies leading to obstruction, dysmenorrhea or palpable pelvic mass in the puberty are the main clinical presentations. In cases without obstruction, infertility or multiple abortions can be encountered in reproductive ages. The identification of the subtype of the uterine anomalies is important for the preoperative planning of the management. MR that has multiplanar imaging capability and high soft tissue resolution is a non-invasive and the most important imaging modality for the detection and classification of the uterine anomalies

Congenital Hypothyroidism

Science.gov (United States)

... Disease Featured Resource Find an Endocrinologist Search Congenital Hypothyroidism March 2012 Download PDFs English Espanol Editors Rosalind S. ... Resources MedlinePlus (NIH) Mayo Clinic What is congenital hypothyroidism? Newborn babies who are unable to make enough ...

Mitochondrial disorders in congenital myopathies

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D. A. Kharlamov

2014-01-01

Full Text Available The literature review gives data on the role of mitochondrial disorders in the pathogenesis of congenital myopathies: congenital muscular dystrophies and congenital structural myopathies. It describes changes in congenital muscular dystrophies with type VI collagen, in myodystrophy with giant mitochondria, in congenital central core myopathies, myotubular myopathy, etc. Clinical and experimental findings are presented. Approaches to therapy for energy disorders in congenital myopathies are depicted.

Valsartan combined with clopidogrel and/or leflunomide for the treatment of progressive immunoglobulin A nephropathy.

Science.gov (United States)

Cheng, Genyang; Liu, Dongwei; Margetts, Peter; Liu, Limin; Zhao, Zhanzheng; Liu, Zhangsuo; Tang, Lin; Fang, Yudong; Li, Haijian; Guo, Yuanyuan; Chen, Fengmei; Liu, Fengxun

2015-02-01

The current standard treatment for IgA nephropathy relies on steroid and/or immunosuppressive therapy and angiotensin converting enzyme inhibitors (ACEI) or angiotensin receptor blocker (ARB). This study examines the benefits and safety of combining valsartan with clopidogrel and leflunomide as a treatment for progressive IgA nephropathy. Patients with primary IgA nephropathy, confirmed by renal biopsy, were recruited for this study. Patients were separated into four groups (n = 42 each) after 2 months of run-in period of valsartan treatment. All patients were treated with valsartan alone (Group 1) or valsartan and either clopidogrel (Group 2) or leflunomide (Group 3) or both clopidogrel and leflunomide (Group 4). Each group was followed up for their next 24 months for 24 h urinary protein excretion, serum creatinine and estimated glomerular filtration rate (eGFR) to assess the effect of the treatment. Adverse effects were recorded concurrently to evaluate the safety of the treatment. Of all 168 patients, 107 were males and 61 were females, with an average age of 33.8 ± 8.79 years. Baseline characteristics were comparable among the four groups (P > 0.05) prior to the experimental treatment. There was a significant (P Valsartan combined with Clopidogrel and Leflunomide can reduce the urinary proteins loss and renal function deterioration for IgA nephropathy patients and cause minimal adverse reactions. Our study suggests a new clinical treatment option for IgA nephropathy. © 2014 Asian Pacific Society of Nephrology.

Congenital anomalies of the urinary tract.

Science.gov (United States)

Pohl, Hans G; Belman, A Barry

2014-01-01

The upper urinary tract forms as a consequence of the reciprocal inductive signals between the metanephric mesenchyme and ureteric bud. A clue to the timing of events leading to an abnormality of the upper urinary tract can be the presence also of associated anomalies of internal genitalia since separation of these systems occurs at about the 10th week of gestation. Prenatal sonography has facilitated the detection of urological abnormalities presenting with hydronephrosis. Hydronephrosis suggests obstruction, but by itself cannot be equated with it. Instead, further radiographic imaging is required to delineate anatomy and function. Now, moreover, non-surgical management of CAKUT should be considered whenever possible. Despite the widespread use of prenatal screening sonography that usually identifies the majority of congenital anomalies of the urinary tract, many children still present with febrile urinary tract infection (UTI). Regardless of the etiology for the presentation, the goal of management is preservation of renal function through mitigation of the risk for recurrent UTI and/or obstruction. In the past many children underwent surgical repair aimed at normalization of the appearance of the urinary tract. Today, management has evolved such that in most cases surgical reconstruction is performed only after a period of observation - with or without urinary prophylaxis. The opinions presented in this section are not espoused by all pediatric urologists but represent instead the practice that has evolved at Children's National Medical Center (Washington DC) based significantly on information obtained by nuclear renography, in addition to sonography and contrast cystography.

Congenital esophageal stenosis associated with esophageal atresia/tracheoesophageal fistula: clinical and radiologic features

Energy Technology Data Exchange (ETDEWEB)

Yoo, Hye Jin; Kim, Woo Sun; Cheon, Jung-Eun; Shin, Su-Mi; Kim, In-One; Yeon, Kyung Mo [Seoul National University College of Medicine and the Institute of Radiation Medicine, Department of Radiology, Seoul (Korea); Yoo, So-Young [Seoul National University College of Medicine and the Institute of Radiation Medicine, Department of Radiology, Seoul (Korea); Sungkyunkwan University School of Medicine, Department of Radiology and Center for Imaging Science, Samsung Medical Center, Seoul (Korea); Park, Kwi-Won; Jung, Sung-Eun [Seoul National University Children' s Hospital, Department of Pediatric Surgery, Seoul (Korea)

2010-08-15

Congenital esophageal stenosis (CES) can be associated with esophageal atresia/tracheoesophageal fistula (EA/TEF). Because there are a variety of degrees of obstruction and symptoms of CES, it is frequently difficult to make a pre- and post-operative diagnosis of the distal CES associated with EA/TEF. To evaluate the clinical and radiologic features of congenital esophageal stenosis associated with esophageal atresia/tracheoesophageal fistula. We retrospectively reviewed postoperative esophagograms and medical records of 187 children (107 boys, 80 girls) who had primary repair of EA/TEF from 1992 to 2009 at our institution. We evaluated the incidence of CES, clinical findings, radiologic features and management of CES in these children. CES was diagnosed in 22 of 187 EA/TEF children (12%); one child had double CES lesions, for a total of 23 lesions. Ten of those 22 children (45%) had presented with significant symptoms of esophageal obstruction. The diagnosis of CES was delayed in 10 children (45%) until 1-10 years of age. On esophagogram, CES (n = 23) was located in the distal esophagus (n = 20, 87%) or mid-esophagus (n = 3, 13%). The degree of stenosis was severe (n = 6, 26%), moderate (n = 10, 43%), or mild (n = 7, 30%). Eight children, including two with unsuccessful esophageal balloon dilatation of CES, were treated surgically. Histologic examination revealed tracheobronchial remnant (n = 7) or fibromuscular hyperplasia (n = 1). One child with surgically treated CES developed achalasia at the age of 3 years 9 months. Esophagography after EA/TEF repair should be performed with a high index of suspicion for the presence of distal CES, because the diagnosis and adequate management of CES can often be delayed. (orig.)

Peculiarities of surgical treatment of gastrointestinal tract combined congenital malformations in newborns

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М. О. Makarova

2017-04-01

Full Text Available Congenital gastrointestinal (GI malformations make up 21–25 % of all congenital anomalies and require surgical correction in the neonatal period. The aim was to analyze the methods of operative treatment of hard composite congenital gastrointestinal malformations in infants. Materials and Methods: There were 13 newborns with gastroschisis, omphalocele and esophageal atresia combined with intestinal atresia, anal atresia and also with congenital heart defects in our study. Results. We have designed new preoperative care strategies for the newborns. All combined GI defects were corrected in one step. In gastroschisis and omphalocele in combination with small intestine atresia we made a plastic of anterior abdominal wall, bowel segment resection and anastomosis end-to-end. In esophageal atresia and atresia of the anus direct esophago-esophagoanastomosis was applied with suturing of tracheoesophageal fistula, also in two patients proctoplasty by Pena 2 was carried out, and in one patient with high anal atresia colostomy was applied, which was closed in the age of 6 months. In case of esophageal atresia combined with small intestine atresia direct esophago-esophagoanastomosis with tracheoesophageal fistula suturing was carried out, and resection of the bowel segment with anastomosis end-to-end was applied. In a patient with a combination of esophageal atresia and duodenal obstruction esophagoplasty and closure of tracheoesophageal fistula with anastomosis by Kimur was made. Preference was given to the combined anesthesia with neuraxial blockade. Postoperative care included prolonged artificial lung ventilation, anesthesia, parenteral nutrition, antibacterial and antifungal medicines. Conclusions. One-step correction of the congenital GI malformations in newborns is effective and it gives opportunity to achieve the best results with a single surgical intervention. Extremely important links of the combined GI defects therapy is timely and balanced

The influence of a single nucleotide polymorphism within CNDP1 on susceptibility to diabetic nephropathy in Japanese women with type 2 diabetes.

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Mahiro Kurashige

Full Text Available BACKGROUND: Several linkage analyses have mapped a susceptibility locus for diabetic nephropathy to chromosome 18q22-23, and polymorphisms within the carnosine dipeptidase 1 gene (CNDP1, located on 18q22.3, have been shown to be associated with diabetic nephropathy in European subjects with type 2 diabetes. However, the association of this locus with diabetic nephropathy has not been evaluated in the Japanese population. In this study, we examined the association of polymorphisms within the CNDP1/CNDP 2 locus with diabetic nephropathy in Japanese subjects with type 2 diabetes. METHODOLOGY/PRINCIPAL FINDINGS: We genotyped a leucine repeat polymorphism (D18S880 that is within CNDP1 along with 29 single nucleotide polymorphisms (SNPs in the CNDP1/CNDP2 locus for 2,740 Japanese subjects with type 2 diabetes (1,205 nephropathy cases with overt nephropathy or with end-stage renal disease [ESRD], and 1,535 controls with normoalbuminuria. The association of each polymorphism with diabetic nephropathy was analysed by performing logistic regression analysis. We did not observe any association between D18S880 and diabetic nephropathy in Japanese subjects with type 2 diabetes. None of the 29 SNPs within the CNDP1/CNDP2 locus were associated with diabetic nephropathy, but a subsequent sex-stratified analysis revealed that 1 SNP in CNDP1 was nominally associated with diabetic nephropathy in women (rs12604675-A; p = 0.005, odds ratio [OR] = 1.76, 95% confidence interval [CI], 1.19-2.61. Rs12604675 was associated with overt proteinuria (p = 0.002, OR = 2.18, 95% CI, 1.32-3.60, but not with ESRD in Japanese women with type 2 diabetes. CONCLUSIONS/SIGNIFICANCE: Rs12604675-A in CNDP1 may confer susceptibility to overt proteinuria in Japanese women with type 2 diabetes.

UPJ obstruction

Science.gov (United States)

... junction obstruction; Obstruction of the ureteropelvic junction Images Kidney anatomy References Elder JS. Obstruction of the urinary tract. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, St. Geme JW, Schor NF, eds. ... The Kidney . 10th ed. Philadelphia, PA: Elsevier; 2016:chap 38. ...

The role of hypertension in the development of nephropathy in type 1 (insulin-dependent) diabetes mellitus

DEFF Research Database (Denmark)

Feldt-Rasmussen, B; Nørgaard, K; Jensen, T

1990-01-01

Which comes first when developing clinical diabetic nephropathy, the blood pressure rise or the increasing urinary albumin excretion? This issue is discussed based on recent literature of studies in humans with Type 1 (insulin-dependent) diabetes mellitus. We conclude that hypertension has...... a central role in the progression of diabetic nephropathy and has deleterious effects on the life expectancy of patients who already have signs of diabetic renal disease in terms of elevated urinary albumin excretion. However, blood pressure is preceded by small increments of urinary albumin excretion rates......, an indicator of universally increased vascular leakiness, and thus does not seem to be the cause of diabetic nephropathy....

Rituximab for the treatment of refractory simultaneous anti-glomerular basement membrane (anti-GBM) and membranous nephropathy.

Science.gov (United States)

Bandak, Ghassan; Jones, Bruce A; Li, Jian; Yee, Jerry; Umanath, Kausik

2014-02-01

Antibody-mediated anti-glomerular basement membrane (anti-GBM) disease occurs rarely in the presence of another B-cell disorder, membranous nephropathy. The coexistence of these two autoimmune disorders would be anticipated to require differing, specific therapies targeted to each disease process. We describe a case of concomitant membranous nephropathy and anti-GBM disease in which conventional therapy, including steroids, plasmapheresis and cyclophosphamide, failed to attenuate the anti-GBM disease, yet responded to an alternative treatment of rituximab. This B-cell directed, monoclonal, chimeric antibody treatment substantially reduced anti-GBM antibody titers and led to discontinuation of plasmapheresis, while maintaining the remission of membranous nephropathy and anti-GBM disease.

Effect of pregnancy on diabetic nephropathy and retinopathy

International Nuclear Information System (INIS)

Irfan, S.; Arain, M.; Shahid, A.; Shaukat, A.

2004-01-01

Objective: To determine whether pregnancy worsens renal function in women with diabetic nephropathy and the effect of pregnancy on diabetic retinopathy. Subject and Methods: Thirty-five patients (aged 20-36 years) identified with diabetic nephropathy and moderate to severe renal dysfunction (creatinine Cr) - > 1.4 mg/dl) at pregnancy onset by retrospective chart review. Alterations in glomerular filtration rate (GFR) were estimated. An equal number of non-pregnant premenopausal type I diabetic women with similar degrees of renal dysfunction served as controls for non-pregnant rate of decline of renal function and potential contributing factors. Student's t-test and repeated measures analysis of variance were analyzed. Results: Mean serum Cr rose from 1.8 mg/dl pre pregnancy to 2.5 mg/dl in the third trimester. Renal function was stable in 27%, showed transient worsening in pregnancy in 27%, and demonstrated a permanent decline in 45%. Proteinuria increased in pregnancy in 79%. Exacerbation of hypertension or pre-eclampsia occurred in 73% and 71% of these showed acceleration of disease during the pregnancy. All the patients had diabetic retinopathy, though proliferative retinopathy was diagnosed and treated in only 54.5.% pre pregnancy. The retinopathy progressed, requiring laser therapy, in 45.4%. Macular edema was noted in 6 of the patients. Other diabetic complications included peripheral and autonomic neuropathy in 8 patients. Conclusion: Pregnancy induced progression is seen in the decline of renal functions. Patients with diabetic nephropathy were found to have a > 40% chance of accelerated progression of their disease as a result of pregnancy. Forty-five percent of the patients had permanent decline in GFR in association with pregnancy. (author)

Maturity-onset diabetes of the young with end-stage nephropathy

DEFF Research Database (Denmark)

Saudek, Frantisek; Pruhová, Stepánka; Boucek, Peter

2004-01-01

-onset diabetes of the young (MODY). SPK was performed in a 47-year old man who has MODY3 because of a Arg272His mutation in the hepatocyte nuclear factor-1alphagene. He developed overt diabetes mellitus at 19 years and end-stage diabetic nephropathy 26 years thereafter. Before SPK, the patient had measurable....... CONCLUSION: Identification of MODY3 among all C-peptide-positive patients with advanced diabetic nephropathy might help to select a specific group profiting from SPK.......BACKGROUND AND CASE: Simultaneous pancreas and kidney transplantation (SPK) is applied almost exclusively in C-peptide-negative type 1 diabetic patients, although some data on SPK in type 2 diabetes have been published as well. Nothing is known about SPK in the autosomal diabetes form, maturity...

Fcγ receptor deficiency attenuates diabetic nephropathy

OpenAIRE

López-Parra, Virginia; Mallavia, Beñat; López-Franco, Óscar; Ortiz-Muñoz, Guadalupe; Oguiza, Ainhoa; Recio, Carlota; Blanco, Julia A Parra; Nimmerjahn, F.; Egido de los Rios, J.; Gómez-Guerrero, Carmen

2012-01-01

Among patients with diabetes, increased production of immunoglobulins against proteins modified by diabetes is associated with proteinuria and cardiovascular risk, suggesting that immune mechanisms may contribute to the development of diabetes complications, such as nephropathy. We investigated the contribution of IgG Fcg receptors to diabetic renal injury in hyperglycemic, hypercholesterolemic mice. Weused streptozotocin to induce diabetes in apolipoprotein E–deficientmice and in...

Longitudinal Assessment of the Effect of Atrasentan on Thoracic Bioimpedance in Diabetic Nephropathy

DEFF Research Database (Denmark)

Webb, David J; Coll, Blai; Heerspink, Hiddo J L

2017-01-01

BACKGROUND: Fluid retention is a common adverse event in patients who receive endothelin (ET) receptor antagonist therapy, including the highly selective ETA receptor antagonist, atrasentan. OBJECTIVE: We performed longitudinal assessments of thoracic bioimpedance in patients with type 2 diabetes...... mellitus and nephropathy to determine whether a decrease in bioimpedance accurately reflected fluid retention during treatment with atrasentan. STUDY DESIGN: We conducted a randomized, double-blind, placebo-controlled study in 48 patients with type 2 diabetes mellitus and nephropathy who were receiving...

Congenital duodenal obstruction with situs inversus totalis: Report of a rare association and discussion

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Sharma Satendra

2008-01-01

Full Text Available This report is to present and discuss an extremely rare association of situs inversus with duodenal atresia in an 11-day-old male neonate born full term and weighing 1.9 kg. The baby presented with recurrent bilious vomiting. Babygram revealed situs inversus and duodenal obstruction. Echocardiography showed dextrocardia with a small ASD. Exploration confirmed a duodenal diaphragm with a central perforation between the third and fourth part of the duodenum and situs inversus. The literature search revealed 20 cases reported so far.

Cytomegalovirus Congenital Cataract

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Ridha Wahyutomo

2011-06-01

Full Text Available Cytomegalovirus congenital infection is an infection caused by the the subfamily â Herpesviridae, during pregnancy. The incidence of infections among newborn infants is 1 %. One of the effects of congenitally acquired infection is the congenital cataract. A 6-year-old child complained to have a blurred vision diagnosed with cytomegalovirus congenital cataract. The diagnosis was confirmed by a positive serology testing for Ig M and Ig G CMV. The laboratory test using Giemsa staining to find inclusion bodies and a faster PCR could not be carried out (Sains Medika, 3(1:84-88.

The Oxford IgA nephropathy clinicopathological classification is valid for children as well as adults

NARCIS (Netherlands)

Coppo, Rosanna; Troyanov, Stéphan; Camilla, Roberta; Hogg, Ronald J.; Cattran, Daniel C.; Cook, H. Terence; Feehally, John; Roberts, Ian S. D.; Amore, Alessandro; Alpers, Charles E.; Barratt, Jonathan; Berthoux, Francois; Bonsib, Stephen; Bruijn, Jan A.; D'Agati, Vivette; D'Amico, Giuseppe; Emancipator, Steven N.; Emma, Francesco; Ferrario, Franco; Fervenza, Fernando C.; Florquin, Sandrine; Fogo, Agnes B.; Geddes, Colin C.; Groene, Hermann J.; Haas, Mark; Herzenberg, Andrew M.; Hill, Prue A.; Hsu, Stephen I.; Jennette, J. Charles; Joh, Kensuke; Julian, Bruce A.; Kawamura, Tetsuya; Lai, Fernand M.; Li, Lei S.; Li, Philip K.; Liu, Zhi H.; Mezzano, Sergio; Schena, F. Paolo; Tomino, Yasuhiko; Walker, Patrick D.; Wang, Haiyan; Weening, Jan J.; Yoshikawa, Norishige; Zhang, Hong

2010-01-01

To study the predictive value of biopsy lesions in IgA nephropathy in a range of patient ages we retrospectively analyzed the cohort that was used to derive a new classification system for IgA nephropathy. A total of 206 adults and 59 children with proteinuria over 0.5 g/24h/1.73 m(2) and an eGFR of

Relationship between serum adiponectin concentration and diabetic nephropathy in patients with type 2 diabetes mellitus

International Nuclear Information System (INIS)

Zhu Wei; Yang Yuzhi; Li Xianhou; Feng Kun; Wang Dan

2007-01-01

Objective: To investigate the relationship between serum adiponectin concentration and diabetic nephropathy in patients with type 2 diabetes mellitus. Methods: The serum adiponectin concentrations were measured with RIA in 163 patients with type 2 diabetes mellitus and 50 controls. Results: In the diabetic patients, serum adiponectin concentrations were significantly higher in patients with macro albuminuria (n = 54) than those inpatients with microalbuminuria (n = 57) (P 0.05). Adiponectin concentrations were higher in women than in men, but there was no significant difference (P > 0.05). Conclusion: Serum adiponectin concentrations are increased in type 2 diabetic patients with advanced nephropathy. The kidney seems to be involved in the metabolism and excretion of adiponectin. Adiponectin may play important roles in the onset and development of diabetic nephropathy. (authors)

Glycosylated hemoglobin as a forecast factor of progressing of diabetic nephropathy in patients with diabetes type 1

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Pertseva N.O.

2017-12-01

Full Text Available The aim of the study was to propose a mathematical model for prediction of development of diabetic nephropathy in patients with diabetes mellitus by determining the level of glycosylated hemoglobin - as a factor in the development and progression of diabetic nephropathy. A survey of 136 patients with type 1 diabetes was performed in the endocrinology department of the OSH «Clinic of the Medical Academy», Dnipro in 2016-2017. Clinical laboratory examination included: determination of the level of glycosylated hemoglobin (HbA1c, level of blood creatinine, level of albuminuria. The GFR was calculated by the formula CKD-EPI. The obtained results of the study, using methods of correlation and regression analysis, show a clear correlation between the GFR score in patients with diabetes mellitus and the level of glycosylated hemoglobin. Statistical methods of analysis have shown that the level of glycosylated hemoglobin can be considered as an early predictor of development of diabetic nephropathy. The mathematical equation of prognosis for the onset of diabetic nephropathy can be used to determine the prognosis for the development of diabetic nephropathy in diabetes mellitus patients in clinical practice for the timely inclusion of patients with a high prognostic risk in a group requiring more stringent glycemic control.

Biotransformation effect of Bombyx Mori L. may play an important role in treating diabetic nephropathy.

Science.gov (United States)

Zhang, Lei; Zhang, La; Li, Yin; Guo, Xin-Feng; Liu, Xu-Sheng

2016-11-01

Compared with herbal drugs, medicine processed from animals (animal medicine) was thought to have more bioactive substances and higher activities. Biotransformation effect often plays an important role in their effect. However, researches about effect of animal medicine on diabetic nephropathy and applying animal medicine as natural bio-transformer were seldom reported. The purpose of this paper was to reveal the use of Bombyx Mori L. on diabetic nephropathy from ancient to modern times. The classical literature indicated that Saosi Decoction (), which contains Bombyx Mori L. or silkworm cocoon, was applied to treat disorders congruent with modern disease diabetic nephropathy from the Ming to Qing Dynasty in ancient China. Modern studies showed that Bombyx Mori L. contains four main active constituents. Among these, 1-deoxynojirimycin (1-DNJ) and quercetin showed promising potential to be new agents in diabetic nephropathy treatment. The concentrations of 1-DNJ and the activities of quercetin in Bombyx Mori L. are higher than in mulberry leaves, because of the biotransformation in the Bombyx Mori L. body. However, these specifific components need further human and mechanistic studies to determine their therapeutic potential for this challenging condition.

Targeted-release budesonide versus placebo in patients with IgA nephropathy (NEFIGAN)

DEFF Research Database (Denmark)

Fellström, Bengt C.; Barratt, Jonathan; Cook, Heather

2017-01-01

Background IgA nephropathy is thought to be associated with mucosal immune system dysfunction, which manifests as renal IgA deposition that leads to impairment and end-stage renal disease in 20–40% of patients within 10–20 years. In this trial (NEFIGAN) we aimed to assess safety and efficacy...... at 62 nephrology clinics across ten European countries. We recruited patients aged at least 18 years with biopsy-confirmed primary IgA nephropathy and persistent proteinuria despite optimised renin-angiotensin system (RAS) blockade. We randomly allocated patients with a computer algorithm, with a fixed...

Trends of diabetic nephropathy prevalence in Isfahan, Iran, during 1992-2010

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Tohid Jafari-Koshki

2015-01-01

Full Text Available Background: Diabetes mellitus is a metabolic disorder and its subsequent complications such as retinopathy, nephropathy, ulcers, disability, and amputation increase the burden of the disease. Patient knowledge-improving programs are employed to prevent disease progression and to improve the quality of life of the patients. In this way, we need to characterize the groups of patients in urgent need for more and rich-in-content programs. In the present study, we used piecewise regression to evaluate the trends of diabetic nephropathy prevalence in patients registered in the Sedigheh-Tahereh Research Center and to identify patients who were in need of more attention. Materials and Methods: Piecewise regression, used in this study, is a statistical method to identify change points, if any, in the trends of mortality rates, prevalence of a disease, or any other trends. Available information for 1,935 patients were retrieved from the database. Joinpoint program 3.5.3 and Statistical Package for the Social Sciences (SPSS 20 was used to fit piecewise regression and obtain descriptive statistics, respectively. Results: We assessed the trend of diabetic nephropathy in different groups of diabetic patients with respect to sex, blood pressure status, education, family history of diabetes, and age. The results showed an increasing trend in females, patients without family history of diabetes, and eover th recent years. The prevalence of diabetic nephropathy in patients with academic education was high. Conclusion: The groups with high prevalence or increasing trends need more preventive intervention and detailed assessment of the present trends. Exploring high-risk groups is beneficial for better policy-making in the future. However, discovering the reasons for the increased trend of the disease is really helpful in controlling diabetes complications.

Carnosine Attenuates the Development of both Type 2 Diabetes and Diabetic Nephropathy in BTBR ob/ob Mice

NARCIS (Netherlands)

Albrecht, Thomas; Schilperoort, Maaike; Zhang, Shiqi; Braun, Jana D.; Qiu, Jiedong; Rodriguez, Angelica; Pastene, Diego O.; Kraemer, Bernhard K.; Koeppel, Hannes; Baelde, Hans; de Heer, Emile; Altomare, Alessandra Anna; Regazzoni, Luca; Denisi, Alessandra; Aldini, Giancarlo; van den Born, Jacob; Yard, Benito A.; Hauske, Sibylle J.

2017-01-01

We previously demonstrated that polymorphisms in the carnosinase-1 gene (CNDP1) determine the risk of nephropathy in type 2 diabetic patients. Carnosine, the substrate of the enzyme encoded by this gene, is considered renoprotective and could possibly be used to treat diabetic nephropathy (DN). In

Attenuation of diabetic nephropathy in streptozotocin-induced diabetic rats by Punica granatum Linn. leaves extract

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Snehal Nitin Mestry

2017-07-01

Full Text Available With an objective to develop Complementary and Alternative Medicine for the treatment of diabetic nephropathy, the present study investigated the protective effects of methanolic extract of Punica granatum leaves (MPGL in streptozotocin-induced diabetic nephropathy. Diabetic nephropathy has become a leading cause of end stage renal failure worldwide. P. granatum, due to its anti-diabetic, anti-inflammatory and antioxidant activities may retard the progression of diabetic nephropathy. In this study, diabetes was induced by a single injection of streptozotocin (STZ, 45 mg/kg, i.p. in rats. STZ-diabetic rats were treated with oral doses of MPGL (100, 200 and 400 mg/kg for 8 weeks. At the end of the experimental period, body and kidney weight and blood glucose levels were determined. Serum and urine parameters were investigated. Antioxidant enzymes and lipid peroxide levels were determined in the kidney along with histopathological examination of the same. MPGL significantly increased body weight, lowered blood glucose levels and ameliorated kidney hypertrophy index in the STZ-diabetic rats. The extract also decreased the levels of creatinine, blood urea nitrogen, total cholesterol, triglycerides, advanced glycation end products and albumin in serum and urine, respectively. MPGL significantly increased the antioxidant parameters in the kidney. Histological evaluation revealed that MPGL treated STZ-diabetic rats demonstrated reduced vacuolar degeneration of tubules; periodic acid Schiff base (PAS positivity staining intensity in glomeruli and basement membrane thickening. Present findings provide experimental evidence that MPGL has potential antioxidant, antihyperglycemic and anti-glycation activities which might be helpful in slowing the progression of diabetic nephropathy.

Attenuation of diabetic nephropathy in streptozotocin-induced diabetic rats by Punica granatum Linn. leaves extract.

Science.gov (United States)

Mestry, Snehal Nitin; Dhodi, Jayesh Bachu; Kumbhar, Sangita Balbhim; Juvekar, Archana Ramesh

2017-07-01

With an objective to develop Complementary and Alternative Medicine for the treatment of diabetic nephropathy, the present study investigated the protective effects of methanolic extract of Punica granatum leaves (MPGL) in streptozotocin-induced diabetic nephropathy. Diabetic nephropathy has become a leading cause of end stage renal failure worldwide. P. granatum , due to its anti-diabetic, anti-inflammatory and antioxidant activities may retard the progression of diabetic nephropathy. In this study, diabetes was induced by a single injection of streptozotocin (STZ, 45 mg/kg, i.p.) in rats. STZ-diabetic rats were treated with oral doses of MPGL (100, 200 and 400 mg/kg) for 8 weeks. At the end of the experimental period, body and kidney weight and blood glucose levels were determined. Serum and urine parameters were investigated. Antioxidant enzymes and lipid peroxide levels were determined in the kidney along with histopathological examination of the same. MPGL significantly increased body weight, lowered blood glucose levels and ameliorated kidney hypertrophy index in the STZ-diabetic rats. The extract also decreased the levels of creatinine, blood urea nitrogen, total cholesterol, triglycerides, advanced glycation end products and albumin in serum and urine, respectively. MPGL significantly increased the antioxidant parameters in the kidney. Histological evaluation revealed that MPGL treated STZ-diabetic rats demonstrated reduced vacuolar degeneration of tubules; periodic acid Schiff base (PAS) positivity staining intensity in glomeruli and basement membrane thickening. Present findings provide experimental evidence that MPGL has potential antioxidant, antihyperglycemic and anti-glycation activities which might be helpful in slowing the progression of diabetic nephropathy.

Acute and long-term effect of antihypertensive treatment on exercise-induced albuminuria in incipient diabetic nephropathy

DEFF Research Database (Denmark)

Christensen, Cramer; Mogensen, C E

1986-01-01

. In the acute study, using placebo/metoprolol 10 mg i.v. in patients with normal UAE, the maximal SBP at 600 kpm/min was reduced by 17 mmHg +/- 10 (SD) (2p less than 1.0%) and the maximal SBP at 600 kpm/min in the patients with incipient nephropathy was reduced by 15 mmHg +/- 11 (SD) (2p less than 1.......0%). However, no difference was observed in UAE, in patients with normal UAE or those with incipient nephropathy. Five of the patients with incipient nephropathy were followed with repeated exercise tests before and during 2.6 years of antihypertensive treatment, using metoprolol 200 mg/24 h and subsequently...

Congenital Abnormalities

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... tube defects. However, there is also a genetic influence to this type of congenital anomaly. Unknown Causes The vast majority of congenital abnormalities have no known cause. This is particularly troubling for parents who plan to have more children, because there is no way to predict if ...

Losartan and diabetic nephropathy: commentaries on the RENAAL study

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Tenenbaum Alexander

2002-04-01

Full Text Available Abstract The RENAAL (Reduction of Endpoints in NIDDM with the Angiotensin II Antagonist Losartan study is a multinational, double-blind, randomized, placebo controlled trial which was recently published. It was aimed to evaluate the effect of the angiotensin receptor blocker losartan in patients with diabetic nephropathy. The primary efficacy measure was the time to the first event of the composite end point of a doubling of serum creatinine, end-stage renal disease, or death. The conclusion was that losartan led to significant improvement in renal outcomes, that was beyond that attributable to blood pressure control in patients with type 2 diabetes and nephropathy. The perusal of the report raises concern, regarding to both the patient population as well as the outcome measures. At randomization, the placebo group included more patients with angina, myocardial infarction and lipid disorders than the losartan group. Information on glucose metabolism was disregarded, and data on antihyperglycemic therapy – which may have undesirable influences on cardiac performance – were not included in a multivariate analysis. In addition, only data on first hospitalization were reported, whilst information on total specific-cause hospitalizations was disregarded, thus potentially masking further unfavorable events. Furthermore, creatinine seems not to be a reliable surrogate end point. Based on its mechanism of action, losartan may possess favorable renoprotective properties. However, due to the methodological flaws and the incomplete data in the RENAAL study, the question of the effectiveness and safety of this drug in diabetic nephropathy remains yet unanswered.

Surgical Approach, Findings, and Eight-Year Follow-Up in a Twenty-Nine Year Old Female With Freeman-Sheldon Syndrome Presenting With Blepharophimosis Causing Near-Complete Visual Obstruction.

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Portillo, Augusto L; Poling, Mikaela I; McCormick, Rodger J

2016-07-01

The authors describe the surgical approach, findings, and 8-year follow-up in a 29-year-old woman, with severe Freeman-Sheldon syndrome, presenting with congenital blepharophimosis of both upper eyelids resulting in near-complete functional visual obstruction. To avoid possible Freeman-Sheldon syndrome-associated complications of malignant hyperthermia, difficult vascular access, and challenging endotracheal intubation, the surgery was completed under local anesthesia without sedation, and anatomical and functional correction was immediate and remained stable at 8-year follow-up. Unlike many congenital craniofacial syndromes, which frequently involve life-long impairments, important implications exist for plastic surgeons to facilitate opportunities for patients to overcome functional limitations.

Treatment of IgA nephropathy.

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Barratt, J; Feehally, J

2006-06-01

IgA nephropathy (IgAN) is an important cause of progressive kidney disease with 25-30% of patients developing end-stage renal disease within 20 years of diagnosis. There is still no treatment to modify mesangial IgA deposition and available treatments are those extrapolated from the management of other patterns of chronic glomerulonephritis. There remains no consensus on the use of immunosuppressive agents for treatment of progressive IgAN and this is compounded by the relative lack in IgAN of randomized controlled trials relevant to current clinical practice. Patients with recurrent macroscopic hematuria or isolated microscopic hematuria and proteinuria renal biopsy should be managed as for minimal change nephropathy. There is no evidence to support the use of corticosteroids for nephrotic IgAN outside this group of patients. Patients presenting with acute renal failure require evaluation to distinguish acute tubular necrosis, which requires supportive therapy only, from crescentic IgAN, for which treatment with cyclophosphamide and corticosteroids in a regimen similar to that for renal small vessel vasculitis is indicated in the absence of significant chronic histologic injury. Patients at greatest risk of progressive renal impairment are those with hypertension, proteinuria >1 g/24 h, and reduced glomerular filtration rate at diagnosis. All such patients should be treated to a blood pressure of 125/75 mm Hg with dual blockade of the renin-angiotensin system with angiotensin-converting enzyme inhibition and angiotensin receptor blockade. At present, there is insufficient evidence for the additional use of immunosuppressive agents, antiplatelet agents, or anticoagulants.

Congenital tuberculosis

African Journals Online (AJOL)

Prof Ezechukwu

2012-06-20

Jun 20, 2012 ... Key words: Congenital tuberculo- sis, case report, miliary tuberculosis. Introduction. Congenital tuberculosis defines tuberculosis in infants of .... tary TB and otitis media, resulting in seizures, deafness, and death. It is therefore not surprising that the index case who presented at twelve weeks of age, had ...

Influence of Enalapril on the progression of chronic renal failure in diabetic nephropathy and nephropathies of and other aethiology: A two-year study

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Trbojević Jasna

2002-01-01

Full Text Available Chronic renal failure (CRF is almost always associated with high arterial blood pressure. Adequate control of hypertension slows down the progression of the disease, Inhibitors of angiotenzin-converting enzyme (ACE inhibitors have proved to be very efficacious in decreasing high blood pressure. The aim of this study was to assess the influence of ACE inhibitor enalapril on the progression of CRF in patients with diabetic nephropathy and nephropathies of other origin. During 1998 and 1999 thirty patients (20 males and 10 females, aged 525+1.3 have been followed-up at the Department of Nephrology, Clinical Centre of Serbia. On regular monthly controls serum creatinine, urea, calcium and protein levels, creatinine clearance, and blood pressure, were measured. All patients were suggested a low protein diet. Progression of the disease was expressed by the slope of the regression line showing reciprocal serum creatinine values. Proteinaemia was significantly higher in diabetic patients after 12 months (p<0.35 but in the next 12 months the difference between groups disappeared. The same patients had significantly lower serum urea (p<0.05 after 24 months and creatinine values (p<0.05 dur ing the whole study. Other variables changed in the same manner and with similar progression in both groups. The direction of slope lines suggested recovery of kidney function in both examined groups. However, a smaller slope in patients with diabetic nephropathy together with other results showed that enalapril had better influence on slowing down the progression of CRF in this group of patients.

Congenital cervical bronchogenic cyst: A case report

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Kiralj Aleksandar

2015-01-01

Full Text Available Introduction. Bronchogenic cysts are rare congenital anomalies of the embryonic foregut. They are caused by abnormal budding of diverticulum of the embryonic foregut between the 26th and 40th day of gestation. Bronchogenic cysts can appear in the mediastinum and pulmonary parenchyma, or at ectopic sites (neck, subcutaneous tissue or abdomen. So far, 70 cases of cervical localization of bronchogenic cysts have been reported. Majority of bronchogenic cysts have been diagnosed in the pediatric population. Bronchogenic cysts of the cervical area are generally asymptomatic and symptoms may occur if cysts become large or in case of infection of the cyst. The diagnosis is made based on clinical findings, radiological examination, but histopathologic findings are essential for establishing the final diagnosis. Treatment of cervical bronchogenic cyst involves surgical excision. Case Outline. Authors present a case of a 6-year-old female patient sent by a pediatrician to a maxillofacial surgeon due to asymptomatic lump on the left side of the neck. The patient had frequent respiratory infections and respiratory obstructions. Magnetic resonance imaging (MRI of the neck was performed and a well-circumscribed cystic formation on the left side of the neck was observed, with paratracheal location. The complete excision of the cyst was made transcervically. Histopathological findings pointed to bronchogenic cyst. Conclusion. Cervical bronchogenic cysts are rare congenital malformations. Considering the location, clinical findings and the radiological features, these cysts resemble other cervical lesions. Surgical treatment is important because it is both therapeutic and diagnostic. Reliable diagnosis of bronchogenic cysts is based on histopathological examination.

Chronic myeloid leukemia in a child with IgA nephropathy.

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Udani, Amish; Vijayakumar, Mahalingam; Prahlad, Nageswaran; Ekambaram, Sudha

2012-08-01

We report an 11 year old boy with IgA nephropathy developing chronic myeloid leukemia on follow-up. This association suggests that a B cell defect might be involved in the pathogenesis of these two conditions.

Diabetic nephropathy and its risk factors in a society with a type 2 diabetes epidemic: a Saudi National Diabetes Registry-based study.

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Khalid Al-Rubeaan

Full Text Available The prevalence of diabetic nephropathy and its risk factors have not been studied in a society known to have diabetes epidemic like Saudi Arabia. Using a large data base registry will provide a better understanding and accurate assessment of this chronic complication and its related risk factors.A total of 54,670 patients with type 2 diabetes aged ≥ 25 years were selected from the Saudi National Diabetes Registry (SNDR and analyzed for the presence of diabetic nephropathy. The American Diabetes Association (ADA criterion was used to identify cases with microalbuminuria, macroalbuminuria and end stage renal disease (ESRD for prevalence estimation and risk factor assessment.The overall prevalence of diabetic nephropathy was 10.8%, divided into 1.2% microalbuminuria, 8.1%macroalbuninuria and 1.5% ESRD. Age and diabetes duration as important risk factors have a strong impact on the prevalence of diabetic nephropathy, ranging from 3.7% in patients aged 25-44 years and a duration of >5 years, to 21.8% in patients ≥ 65 years with a diabetes duration of ≥ 15 years. Diabetes duration, retinopathy, neuropathy, hypertension, age >45 years, hyperlipidemia, male gender, smoking, and chronologically, poor glycemic control has a significantly high risk for diabetic nephropathy.The prevalence of diabetic nephropathy is underestimated as a result of a shortage of screening programs. Risk factors related to diabetic nephropathy in this society are similar to other societies. There is thus an urgent need for screening and prevention programs for diabetic nephropathy among the Saudi population.

Diabetic Nephropathy and Its Risk Factors in a Society with a Type 2 Diabetes Epidemic: A Saudi National Diabetes Registry-Based Study

Science.gov (United States)

Al-Rubeaan, Khalid; Youssef, Amira M.; Subhani, Shazia N.; Ahmad, Najlaa A.; Al-Sharqawi, Ahmad H.; Al-Mutlaq, Hind M.; David, Satish K.; AlNaqeb, Dhekra

2014-01-01

Aims The prevalence of diabetic nephropathy and its risk factors have not been studied in a society known to have diabetes epidemic like Saudi Arabia. Using a large data base registry will provide a better understanding and accurate assessment of this chronic complication and its related risk factors. Methodology A total of 54,670 patients with type 2 diabetes aged ≥25 years were selected from the Saudi National Diabetes Registry (SNDR) and analyzed for the presence of diabetic nephropathy. The American Diabetes Association (ADA) criterion was used to identify cases with microalbuminuria, macroalbuminuria and end stage renal disease (ESRD) for prevalence estimation and risk factor assessment. Results The overall prevalence of diabetic nephropathy was 10.8%, divided into 1.2% microalbuminuria, 8.1%macroalbuninuria and 1.5% ESRD. Age and diabetes duration as important risk factors have a strong impact on the prevalence of diabetic nephropathy, ranging from 3.7% in patients aged 25–44 years and a duration of >5 years, to 21.8% in patients ≥65 years with a diabetes duration of ≥15 years. Diabetes duration, retinopathy, neuropathy, hypertension, age >45 years, hyperlipidemia, male gender, smoking, and chronologically, poor glycemic control has a significantly high risk for diabetic nephropathy. Conclusion The prevalence of diabetic nephropathy is underestimated as a result of a shortage of screening programs. Risk factors related to diabetic nephropathy in this society are similar to other societies. There is thus an urgent need for screening and prevention programs for diabetic nephropathy among the Saudi population. PMID:24586457

Increased sympathetic activity during sleep and nocturnal hypertension in Type 2 diabetic patients with diabetic nephropathy

DEFF Research Database (Denmark)

Nielsen, F S; Hansen, H P; Jacobsen, P

1999-01-01

AIMS: To elucidate the putative factors involved in the blunted nocturnal blood pressure reduction in hypertensive Type 2 diabetic patients with diabetic nephropathy. METHODS: Extracellular fluid volume and fluid shift from interstitial to plasma volume (haematocrit), sympathetic nervous activity...... (plasma noradrenaline and adrenaline) and the internal 'body clock' (serum melatonin) were investigated in 31 hypertensive Type 2 diabetes mellitus (DM) patients with diabetic nephropathy (24 males, age 60 (45-73) years). All variables, except extracellular volume, were measured repeatedly...... constant in both groups. Extracellular fluid volume and plasma melatonin levels were comparable in the two groups. CONCLUSION: Sustained adrenergic activity during sleep is associated with blunted nocturnal blood pressure reduction in hypertensive Type 2DM patients with diabetic nephropathy, probably...

ACE insertion/deletion (I/D) polymorphism and diabetic nephropathy.

Science.gov (United States)

Rahimi, Zohreh

2012-10-01

Angiotensin converting enzyme (ACE) gene encodes ACE, a key component of renin angiotensin system (RAS), plays an important role in blood pressure homeostasis by generating the vasoconstrictor peptide angiotensin II. Directory of Open Access Journals (DOAJ), Google Scholar, Pubmed (NLM), LISTA (EBSCO) and Web of Science have been searched. The presence of ACE insertion/deletion (I/D) polymorphism affects the plasma level of ACE. ACE DD genotype is associated with the highest systemic and renal ACE levels compared with the lowest ACE activity in carriers of II genotype. In this review focus has been performed on the study of ACE I/D polymorphism in various populations and its influence on the risk of onset and progression of diabetic nephropathy. Also, association between ACE I/D polymorphism and response to ACE inhibitor and angiotensin II receptor antagonists will be reviewed. Further, synergistic effect of this polymorphism and variants of some genes on the risk of development of diabetic nephropathy will be discussed.

Increased Granulocyte Heparanase Activity in Neutrophils from Patients with Lupus Nephritis and Idiopathic Membranous Nephropathy.

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Szymczak, Maciej; Kuźniar, Jakub; Kopeć, Wacław; Żabińska, Marcelina; Marchewka, Zofia; Kościelska-Kasprzak, Katarzyna; Klinger, Marian

2017-02-01

Heparanase is a β-glucuronidase that cleaves sugar chains of heparan sulfate proteoglycans. It is believed that heparanase may be involved in the pathogenesis of proteinuria. The aim of this study was to assess the significance of heparanase in the pathogenesis of particular glomerulonephritis types. The evaluation of heparanase activity in serum, urine, and granulocytes and superoxide dismutase (SOD) activity in granulocytes of patients with lupus nephritis (n = 17), membranous nephropathy (n = 11), IgA nephropathy (n = 12), focal and segmental glomerulosclerosis (n = 18), mesangiocapillary glomerulonephritis (n = 12) and in 19 healthy volunteers were performed. The heparanase activity in granulocytes of patients with lupus nephritis and membranous nephropathy was higher than heparanase activity in granulocytes in the control group (p = 0.02 in both cases). This is the first observation of this phenomenon. There was no difference between SOD activity in granulocytes of patients with all assessed types of glomerulonephritis and the control group. A positive correlation between heparanase activity in urine and double-strain DNA antibodies (r = 0.51; p = 0.04), and reverse correlations between heparanase in urine and hemolytic activity of the complement (r = -0.57; p = 0.03) in the lupus nephritis group, and between heparanase activity in granulocytes and serum total protein level (r = -0.69; p = 0.02) in membranous nephropathy were observed. Increase in heparanase activity without changes in superoxide dismutase activity in the granulocytes from patients with lupus nephritis and membranous nephropathy was observed. It may be used as one of the markers of these disease activities.

Attenuation of Diabetic Nephropathy by Carvacrol through Anti-oxidative Effects in Alloxan-Induced Diabetic Rats

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Hamid Reza Jamshidi

2018-03-01

Full Text Available Background and Objectives: Diabetes, a common metabolic disorder, is prevalent in many countries. Nephropathy is a main debate’s side effect. Role of oxidative stress is well known in induction of diabetic nephropathy while carvacrol is a potent anti-oxidant that might attenuate oxidative stress. The aim of this study was to explore the effect of carvacrol in decreasing nephropathy-induced oxidative damage in diabetic rats. Methods: Thirty five Wistar rats (200-250 g were divided to 7 groups. The rats received alloxan (i.p., 200 mg/kg for induction of diabetes. After one week, fasting blood sugar (FBS was assessed and the rats with FBS>250 mg/dL were considered as diabetic. Three weeks after alloxan injection, the blood urea (BUN and creatinine (Cr were determined for confirmation of inducing nephropathy. Then, the animals were treated with carvacrol for one week. Finally, they were anesthetized and blood was collected from animal’s heart for calculation of BUN and Cr. Furthermore, the kidneys were for oxidative stress markers such as glutathione capacity, protein carbonyl, lipid peroxidation and catalase activity. Results: Our results showed that glutathione level and catalase activity significantly increased after treatment with carvacrol. Same results were found in rats that received vitamin E. Also, lipid peroxidation, protein carbonyl content, BUN and Cr levels significantly decreased after treatment with carvacrol in comparison with diabetic rats. Conclusion: Our results showed that carvacrol improved nephropathy-induced oxidative damage similar to vitamin E. Therefore, it may be suggested that carvacrol can be suggested as a useful supplement in decreasing diabetic complaints along with anti-diabetic drugs.

 Uterus Didelphys with Obstructed Right Hemivagina, Ipsilateral Renal Agenesis and Right Pyocolpos: A Case Report

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Hansa Dhar

2011-11-01

Full Text Available  Uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis (OHVIRA Syndrome is a rare congenital anomaly of the female genital tract. Uterus didelphys occurs when the midline fusion of the mullerian ducts is arrested, either completely or incompletely. Women with didelphic uterus may be asymptomatic and unaware of having a double uterus. They may present with complaints of dysmenorrhoea and dyspareunia. A 25 year old woman attending the infertility clinic at Nizwa regional referral hospital presented with history of dysmenorrhoea and foul vaginal discharge with right cystic pelvic mass. She was diagnosed as a case of double uterus with obstructed right hemivagina and right pyocolpos with ipsilateral renal agenesis after routine ultrasonography in the clinic followed by MRI. Excision of the right vaginal septum with drainage of 200 ml of purulent discharge was performed. She was relieved of her symptoms and conceived promptly after the surgical excision of the partial vaginal septum.

Preproghrelin Leu72Met polymorphism predicts a lower rate of developing renal dysfunction in type 2 diabetic nephropathy.

Science.gov (United States)

Lee, Dae-Yeol; Kim, Sun-Young; Jo, Dae-Sun; Hwang, Pyoung Han; Kang, Kyung Pyo; Lee, Sik; Kim, Won; Park, Sung Kwang

2006-07-01

Ghrelin is a novel peptide hormone, which exerts somatotropic, orexigenic and adipogenic effects. Recent studies have shown that the preproghrelin Leu72Met polymorphism is associated with serum creatinine (Scr) concentration in type 2 diabetes; 72Met carriers exhibited lower Scr levels as compared with the 72Met non-carriers. We hypothesized that the preproghrelin Leu72Met polymorphism is associated with a lower rate of developing renal dysfunction in patients with type 2 diabetic nephropathy. The preproghrelin Leu72Met polymorphism was investigated using PCR techniques in 138 patients with diabetic nephropathy divided into two groups, one with normal renal function and the other with renal dysfunction. Determination of the frequency of the preproghrelin Leu72Met polymorphism was the main outcome measure. The frequency of the Leu72Met polymorphism in diabetic nephropathy was significantly lower in patients with renal dysfunction (15.9%, P polymorphism was also associated with serum total cholesterol levels in diabetic nephropathy patients with renal dysfunction; the 72Met carriers had lower total cholesterol levels than the 72Met non-carriers (P < 0.05). These data suggest that 72Met carrier status may be used as a marker predicting a lower chance of developing renal dysfunction in diabetic nephropathy.

Text Mining of the Classical Medical Literature for Medicines That Show Potential in Diabetic Nephropathy

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Lei Zhang

2014-01-01

Full Text Available Objectives. To apply modern text-mining methods to identify candidate herbs and formulae for the treatment of diabetic nephropathy. Methods. The method we developed includes three steps: (1 identification of candidate ancient terms; (2 systemic search and assessment of medical records written in classical Chinese; (3 preliminary evaluation of the effect and safety of candidates. Results. Ancient terms Xia Xiao, Shen Xiao, and Xiao Shen were determined as the most likely to correspond with diabetic nephropathy and used in text mining. A total of 80 Chinese formulae for treating conditions congruent with diabetic nephropathy recorded in medical books from Tang Dynasty to Qing Dynasty were collected. Sao si tang (also called Reeling Silk Decoction was chosen to show the process of preliminary evaluation of the candidates. It had promising potential for development as new agent for the treatment of diabetic nephropathy. However, further investigations about the safety to patients with renal insufficiency are still needed. Conclusions. The methods developed in this study offer a targeted approach to identifying traditional herbs and/or formulae as candidates for further investigation in the search for new drugs for modern disease. However, more effort is still required to improve our techniques, especially with regard to compound formulae.

Possible association of first and high birth order of pregnant women with the risk of isolated congenital abnormalities in Hungary - a population-based case-matched control study.

Science.gov (United States)

Csermely, Gyula; Susánszky, Éva; Czeizel, Andrew E; Veszprémi, Béla

2014-08-01

In epidemiological studies at the estimation of risk factors in the origin of specified congenital abnormalities in general birth order (parity) is considered as confounder. The aim of this study was to analyze the possible association of first and high (four or more) birth order with the risk of congenital abnormalities in a population-based case-matched control data set. The large dataset of the Hungarian Case-Control Surveillance of Congenital Abnormalities included 21,494 cases with different isolated congenital abnormality and their 34,311 matched controls. First the distribution of birth order was compared of 24 congenital abnormality groups and their matched controls. In the second step the possible association of first and high birth order with the risk of congenital abnormalities was estimated. Finally some subgroups of neural-tube defects, congenital heart defects and abdominal wall's defects were evaluated separately. A higher risk of spina bifida aperta/cystica, esophageal atresia/stenosis and clubfoot was observed in the offspring of primiparous mothers. Of 24 congenital abnormality groups, 14 had mothers with larger proportion of high birth order. Ear defects, congenital heart defects, cleft lip± palate and obstructive defects of urinary tract had a linear trend from a lower proportion of first born cases to the larger proportion of high birth order. Birth order showed U-shaped distribution of neural-tube defects and clubfoot, i.e. both first and high birth order had a larger proportion in cases than in their matched controls. Birth order is a contributing factor in the origin of some isolated congenital abnormalities. The higher risk of certain congenital abnormalities in pregnant women with first or high birth order is worth considering in the clinical practice, e.g. ultrasound scanning. Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.

Spectrum of steroid-resistant and congenital nephrotic syndrome in children: the PodoNet registry cohort.

Science.gov (United States)

Trautmann, Agnes; Bodria, Monica; Ozaltin, Fatih; Gheisari, Alaleh; Melk, Anette; Azocar, Marta; Anarat, Ali; Caliskan, Salim; Emma, Francesco; Gellermann, Jutta; Oh, Jun; Baskin, Esra; Ksiazek, Joanna; Remuzzi, Giuseppe; Erdogan, Ozlem; Akman, Sema; Dusek, Jiri; Davitaia, Tinatin; Özkaya, Ozan; Papachristou, Fotios; Firszt-Adamczyk, Agnieszka; Urasinski, Tomasz; Testa, Sara; Krmar, Rafael T; Hyla-Klekot, Lidia; Pasini, Andrea; Özcakar, Z Birsin; Sallay, Peter; Cakar, Nilgun; Galanti, Monica; Terzic, Joelle; Aoun, Bilal; Caldas Afonso, Alberto; Szymanik-Grzelak, Hanna; Lipska, Beata S; Schnaidt, Sven; Schaefer, Franz

2015-04-07

Steroid-resistant nephrotic syndrome is a rare kidney disease involving either immune-mediated or genetic alterations of podocyte structure and function. The rare nature, heterogeneity, and slow evolution of the disorder are major obstacles to systematic genotype-phenotype, intervention, and outcome studies, hampering the development of evidence-based diagnostic and therapeutic concepts. To overcome these limitations, the PodoNet Consortium has created an international registry for congenital nephrotic syndrome and childhood-onset steroid-resistant nephrotic syndrome. Since August of 2009, clinical, biochemical, genetic, and histopathologic information was collected both retrospectively and prospectively from 1655 patients with childhood-onset steroid-resistant nephrotic syndrome, congenital nephrotic syndrome, or persistent subnephrotic proteinuria of likely genetic origin at 67 centers in 21 countries through an online portal. Steroid-resistant nephrotic syndrome manifested in the first 5 years of life in 64% of the patients. Congenital nephrotic syndrome accounted for 6% of all patients. Extrarenal abnormalities were reported in 17% of patients. The most common histopathologic diagnoses were FSGS (56%), minimal change nephropathy (21%), and mesangioproliferative GN (12%). Mutation screening was performed in 1174 patients, and a genetic disease cause was identified in 23.6% of the screened patients. Among 14 genes with reported mutations, abnormalities in NPHS2 (n=138), WT1 (n=48), and NPHS1 (n=41) were most commonly identified. The proportion of patients with a genetic disease cause decreased with increasing manifestation age: from 66% in congenital nephrotic syndrome to 15%-16% in schoolchildren and adolescents. Among various intensified immunosuppressive therapy protocols, calcineurin inhibitors and rituximab yielded consistently high response rates, with 40%-45% of patients achieving complete remission. Confirmation of a genetic diagnosis but not the

Ameliorative effect of combination of benfotiamine and fenofibrate in diabetes-induced vascular endothelial dysfunction and nephropathy in the rat.

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Balakumar, Pitchai; Chakkarwar, Vishal Arvind; Singh, Manjeet

2009-01-01

The study has been designed to investigate the effect of benfotiamine and fenofibrate in diabetes-induced experimental vascular endothelial dysfunction (VED) and nephropathy. The single administration of streptozotocin (STZ) (50 mg/kg, i.p.) produced diabetes, which was noted to develop VED and nephropathy in 8 weeks. The diabetes produced VED by attenuating acetylcholine-induced endothelium dependent relaxation, impairing the integrity of vascular endothelium, decreasing serum nitrite/nitrate concentration and increasing serum TBARS and aortic superoxide anion generation. Further, diabetes altered the lipid profile by increasing the serum cholesterol, triglycerides and decreasing the high density lipoprotein. The nephropathy was noted to be developed in the diabetic rat that was assessed in terms of increase in serum creatinine, blood urea, proteinuria, and glomerular damage. The benfotiamine (70 mg/kg, p.o.) and fenofibrate (32 mg/kg, p.o.) or lisinopril (1 mg/kg, p.o., a standard agent) treatments were started in diabetic rats after 1 week of STZ administration and continued for 7 weeks. The treatment with benfotiamine and fenofibrate either alone or in combination attenuated diabetes-induced VED and nephropathy. In addition, the combination of benfotiamine and fenofibrate was noted to be more effective in attenuating the diabetes-induced VED and nephropathy when compared to treatment with either drug alone or lisinopril. Treatment with fenofibrate normalizes the altered lipid profile in diabetic rats, whereas benfotiamine treatment has no effect on lipid alteration in diabetic rats. It may be concluded that diabetes-induced oxidative stress, lipids alteration, and consequent development of VED may be responsible for the induction of nephropathy in diabetic rats. Concurrent administration of benfotiamine and fenofibrate may provide synergistic benefits in preventing the development of diabetes-induced nephropathy by reducing the oxidative stress and lipid

Congenital orbital teratoma

OpenAIRE

Aiyub, Shereen; Chan, Weng Onn; Szetu, John; Sullivan, Laurence J; Pater, John; Cooper, Peter; Selva, Dinesh

2013-01-01

We present a case of mature congenital orbital teratoma managed with lid-sparing exenteration and dermis fat graft. This is a case report on the management of congenital orbital teratoma. A full-term baby was born in Fiji with prolapsed right globe which was surrounded by a nonpulsatile, cystic mass. Clinical and imaging features were consistent with congenital orbital teratoma. Due to limited surgical expertise, the patient was transferred to Adelaide, Australia for further management. The p...

Contrast-medium-induced nephropathy: is there a new consensus? A review of published guidelines

International Nuclear Information System (INIS)

Thomsen, Henrik S.; Morcos, Sameh K.

2006-01-01

The interest in contrast-medium-induced nephropathy has increased considerably during the last few years. Various guidelines regarding identifying patients at risk and measures to reduce the incidence of this complication have been proposed. The aim of this review was to analyse whether there is some consistency amongst these guidelines. A Medline search for the keyword ''contrast medium induced nephropathy'' during the period from the beginning of 2003 through the end of September 2005 was carried out. Only papers in English were reviewed. Thirteen guidelines were identified. Inconsistency was observed regarding advise on the prophylactic use of drugs and the isoosmolar dimer to reduce the incidence of contrast-medium-induced nephropathy. Consistency was found in relation to the importance of hydration, cessation of intake of nephrotoxic drugs and administration of the lowest possible dose of contrast medium. No new consensus has been observed in comparison to the European Society for Urogenital Radiology (ESUR) guidelines, which were published in 1999. (orig.)

Remission of secondary membranous nephropathy in a patient with Kimura disease after surgical resection

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Sunhwa Lee

2014-09-01

Full Text Available Kimura disease (KD is an eosinophilic, granulomatous, benign, chronic inflammatory disease with an unknown etiology. A 33-year-old woman visited our hospital because of a palpable, left subclavian mass, a left scapulo-anterior pseudoaneurysm, and nephrotic syndrome. Her subclavian lymph node biopsy examination result was consistent with KD, and results of a renal biopsy indicated secondary membranous nephropathy. After renal histological examination confirmed nephropathy, treatment with prednisolone and cyclosporine was initiated, which was maintained for over 1 year. However, this therapy only provided a transient improvement in proteinuria. One year after commencing the treatment, both proteinuria and azotemia aggravated as the left axillary mass doubled in size. Finally, the mass was surgically excised, following which the azotemia rapidly normalized and proteinuria resolved within 1 month. This case shows that tumor resection in a patient with KD with secondary nephropathy may resolve secondary renal manifestations. Furthermore, reversible renal dysfunction may be caused by unknown secreted molecules.

Clinical significance of determination of changes of serum visfatin and adiponectin levels in patients with type 2 diabetic nephropathy

International Nuclear Information System (INIS)

Xu Ning

2009-01-01

Objective: To study the clinical significance of changes of serum visfatin and adiponectin levels in patients with type 2 diabetic nephropathy (DN). Methods: Serum visfatin (with ELISA) and serum adiponectin (with RIA) levels were determined in 41 cases of DM2 without nephropathy, 32 cases of DN and 35 controls. Results: Serum visfatin levels in the diabetic patients were significantly higher than those in controls (P<0.01), while the serum adiponectin levels were significantly lower than those in controls (P<0.01). Serum visfatin levels were significantly negatively correlated with those of serum adiponectin (r=-0.4108, P<0.05). The levels of serum adiponectin in patients with DN is higher than those in patients with DM2 but without nephropathy. Conclusion: The development of type 2 diabetic nephropathy might be related to the levels of visfatin and adipone. (authors)

Improved pregnancy outcome in type 1 diabetic women with microalbuminuria or diabetic nephropathy: effect of intensified antihypertensive therapy?

DEFF Research Database (Denmark)

Nielsen, Lene Ringholm; Damm, Peter; Mathiesen, Elisabeth R

2009-01-01

To describe pregnancy outcome in type 1 diabetic women with normoalbuminuria, microalbuminuria, or diabetic nephropathy after implementation of an intensified antihypertensive therapeutic strategy.......To describe pregnancy outcome in type 1 diabetic women with normoalbuminuria, microalbuminuria, or diabetic nephropathy after implementation of an intensified antihypertensive therapeutic strategy....

Diagnosis of secondary pulmonary lymphangiectasia in congenital heart disease: a novel role for chest ultrasound and prognostic implications.

Science.gov (United States)

Lam, Christopher Z; Bhamare, Tanmay Anant; Gazzaz, Tamadhir; Manson, David; Humpl, Tilman; Seed, Mike

2017-10-01

Secondary pulmonary lymphangiectasia is a complication of congenital heart disease that results from chronic pulmonary venous obstruction. We aimed to evaluate the performance of chest ultrasound (US) in diagnosing secondary pulmonary lymphangiectasia and to review the clinical course of children with secondary pulmonary lymphangiectasia. Chest US was performed on 26 children with hypoplastic left heart syndrome, total anomalous pulmonary venous connection or cor triatriatum in a prospective observational study. Thirteen children had pulmonary venous obstruction (62% male; median age: 17 days old, range: 1-430 days old) and 13 children did not have obstruction (62% male; median age: 72 days old, range: 4-333 days old). US features of secondary pulmonary lymphangiectasia were documented and diagnostic performance was determined. Clinical course of patients with secondary pulmonary lymphangiectasia was reviewed. Eleven of 13 (84.6%) patients in the obstructed group had a clinical and/or biopsy diagnosis of secondary pulmonary lymphangiectasia. Statistically significant chest US criteria for diagnosis were presence of irregular lung surface (likelihood ratio [LR] 6.8, 95% confidence interval [CI] 1.9-25.1), subpleural cystic appearing structures (LR 3.6, 95% CI 1.2-10.7), and combination of subpleural cystic appearing structures and surface irregularity together (LR 10.9, 95% CI 1.6-75.0). Seven of 11 (63.6%) patients with secondary pulmonary lymphangiectasia died during follow-up, the majority due to cardiopulmonary failure or complications. Chest US is an accurate and reproducible bedside method for diagnosing secondary pulmonary lymphangiectasia in patients with pulmonary venous obstruction. These patients may have worse prognoses.

Clinical significance of determination of serum collagen type IV (IV-C) and transforming growth factor beta1(TGF-β1) levels in patients with diabetic nephropathy

International Nuclear Information System (INIS)

Xie Hongfang; Peng Liang

2006-01-01

Objective: To investigate the clinical significance of determination of serum collagen type IV (IV-C) and transforming growth factor beta 1 (TGF-β 1 ) levels in patients with diabetic nephropathy. Methods: Serum IV-C levels ( with RIA) and TGF-β 1 levels (with ELISA) were determined in 30 controls and 105 patients with type II diabetis mellitus (45 with diabetic nephropathy and 60 without nephropathy). Results: The serum levels of IV-C and TGF-β 1 in diabetic patients with nephropathy were significantly higher than those in controls (P 0.05). Conclusion: Serum IV-C and TGF-β 1 , levels increased gradually as the diabetic nephropathy got more severe, they could be used as sensitive markers for early diagnosis of development of diabetic nephropathy. (authors)

[Preclinical diagnostics and correction of the disturbed renal blood flow in the children presenting with diabetic nephropathy].

Science.gov (United States)

Aver'ianov, A P; Tkacheva, E N; Bolotova, N V; Filina, N Iu; Ivanova, Iu V; Nikolaeva, N V; Tikhonova, L A

2011-01-01

The present study included 86 children aged between 7 and 17 years with type 1 diabetes mellitus from 1 to 15 years in duration. In all the patients, renal blood flow was investigated with the use of ultrasonic dopplerography. The results of the study suggest disturbances of intrarenal hemodynamics that manifested themselves as enhanced resistance of renal arteries from periphery to the centre in the patients at the hyperfiltration stage of diabetic nephropathy (DN) in conjunction with the reduced velocity of blood flow in inter-lobular and segmental arteries. In contrast, the patients at the microalbuminuric stage of diabetic nephropathy exhibited increased resistance and reduced velocity of blood flow in the main renal veins. In 35 patients presenting with diabetic nephropathy, hemodynamic correction was achieved by the application of the traveling pulsed magnetic field (TP-MF) to the renal region using an AMO-ATOS-E apparatus (Russia). This treatment resulted in normalization of the characteristics of renal blood flow. It is concluded that TPMF has good prospects for the use as a component of the combined treatment of diabetic nephropathy.

Anti-glomerular basement membrane disease superimposed on membranous nephropathy: a case report and review of the literature

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Nivera Noel

2010-08-01

Full Text Available Abstract Introduction Anti-glomerular basement membrane disease is a rare autoimmune disorder characterized by pulmonary hemorrhage, crescentic glomerulonephritis and the presence of circulating anti-glomerular basement membrane antibodies. The simultaneous occurrence of both anti-glomerular basement membrane disease and membranous nephropathy is rare. Case presentation A 59-year-old Hispanic man presented with acute onset of nausea and vomiting and was found to have renal insufficiency. Work-up included a kidney biopsy, which revealed anti-glomerular basement membrane disease with underlying membranous nephropathy. He was treated with emergent hemodialysis, intravenous corticosteroids, plasmapheresis, and cyclophosphamide without improvement in his renal function. Conclusion Simultaneous anti-glomerular basement membrane disease and membranous nephropathy is very rare. There have been 16 previous case reports in the English language literature that have been associated with a high mortality and morbidity, and a very high rate of renal failure resulting in hemodialysis. Co-existence of membranous nephropathy and anti-glomerular basement membrane disease may be immune-mediated, although the exact mechanism is not clear.

Effects of vitamin E and its derivativeson diabetic nephropathy in Ratsand identification of diacylglycerol kinase subtype involved in the improvement of diabetic nephropathy

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Tomoko Kakehi

2017-10-01

Full Text Available Background: Diabetes is a significant social issue. Controlling diabetic complications such as nephropathy is very important for QOL of diabetic patients. One of the mechanisms which causes diabetic complications is the abnormal activation of protein kinase C (PKC by increased diacylglycerol (DG from hyperglycemia. Diacylglycerol kinase (DGK can attenuate PKC activity by converting DG to phosphatidic acid. Thus far, d-a-tocopherol (VtE treatment has been shown to prevent early changes of diabetic renal dysfunctions by activating DGK. However, it is still unknown whether VtE derivatives improve diabetic nephropathy similarly to VtE, and which DGK subtype is activated by VtE and/or the derivatives. Objective: The purpose of the study was to investigate effects of VtE and its derivatives on diabetic nephropathy in rats, in addition to identifying the DGK subtype involved in the improvement of nephropathy in vivo. Methods: To induce diabetes in rats, six weeks old male Sprague-Dawley rats were intraperitonealy administrated 65 mg/kg streptozocin (STZ in 20 mM citrate buffer. For two or eight weeks, 40 mg/kg VtE, 44 mg/kg acetate VtE (aVtE or 49.3 mg/kg succinate VtE (sVtE was intraperitonealy administrated every other day after STZ administration. The blood glucose level, body weight, and kidney weight, in addition to urinary volume, albumin, and BUN were measured every week after STZ administration. Additionally, in order to identify the DGK subtype activated by VtE and aVtE, the DGK subtype expressed in the rat glomerulus was checked by RT-PCR and western blotting, and the activity in the glomerulus from the rats before and after the VtE and aVtE treatments were measured in the presence or absence of EGTA. Results: Averages of kidney weight and BUN of rats treated with VtE, aVtE and sVtE for 8 weeks were reduced, compared to the control. However, the intraperitoneal administration of sVtE was toxic. Additionally, the amount of urine volume and

Obstructive Sleep Apnoea

African Journals Online (AJOL)

Cheyne-Stokes respiration), obstructive sleep apnoea and mixed or complex sleep apnoea.1. Obstructive sleep apnoea (OSA) is the most common of these three disorders and is defined as airway obstruction during sleep, accompanied by at least ...

Comparing Life Quality Strategies and Emotion Regulation in People with Congenital and Non-Congenital Motor Disability

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Seyedeh Zeynab Miraghaei

2017-10-01

Full Text Available Objective: The purpose of the present study was to compare emotion regulation strategies and life quality of people with congenital and non-congenital motor disabilities. Method: This study is a casual-comparative study and its population consisted of all people with congenital and non-congenital motor disability in Kahrizak Charity Foundation in Tehran in 2016. To conduct the study, available sampling method was used, and congenital and non-congenital disabled people were selected (200 people. To collect data, Cognitive Emotion Regulation Scale by Granovsky and life quality questionnaire were used. Research hypotheses were tested using multivariate analysis of variance. Results: The findings of this study showed that there is a significant difference between emotion regulation components in people with congenital and non-congenital disabilities (p<0.05. Also, according to the findings, a significant difference was observed between life quality dimensions (physical and mental health in people with congenital and non-congenital disabilities (p<0.05. Conclusion: According to the significant difference between two groups of subjects, necessary measures regarding consultation and psychotherapy should be taken into consideration to let people benefit from desirable mental health level.

Key aspects congenital infection

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Yu. V. Lobzin

2014-01-01

Full Text Available The key questions to solve the problem of congenital infection in the Russian Federation are: using in national practice over world accepted terminology adapted to the recommendations of the World Health Organization; representation of the modern concepts of an infectious process in the classification of congenital infections; scientific development and introducing in clinical practice the «standard case definitions», applied to different congenital infections; optimization of protocols and clinical guidelines for diagnosis, treatment and prevention of congenital infections; improvement a knowledge in the infectious disease for all  pecialists involved in the risk assessment of congenital infections, manage pregnancy and children. Based on our experience and analysis of publications, the authors suggest possible solutions.

ESSENTIAL ARTERIAL HYPERTENSION AND RISK FACTORS ASSOCIATED WITH HYPERTENSIVE NEPHROPATHY

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Boban Milojković

2014-12-01

Full Text Available Arterial hypertension is a major risk factor that predisposes to cardiovascular disorders and is responsible for most of the morbidity and mortality in patients. Hypertension is closely associated with the kidney, because kidney disease can be both the cause and consequence of increased blood pressure. Elevation of blood pressure is a strong independent risk factor for hypertensive nephropathy and development of ESRD. The pathogenesis of ischemic hypertensive nephropathy (IHN is multifactoral, and in addition to blood pressure other factors contribute to the development of this renal pathology and its progression to end-stage renal disease. These include obesity, smoking, male gender and other still unknown risk factors. The aim of this paper was to analyse the association between essential arterial hypertension and renal hypertensive disease and prevalence of other atherosclerotic risk factors in patients with developed hypertensive renal disease. In this prospective cross sectional study 283 patients of both genders with diagnosed essential hypertension and hypertensive renal disease were analysed. The anamnestic data related to age, duration of hypertension, history of smoking, presence of hypertensive retinopathy, hypertrophy of the left chamber and data about previous renal diseases were collected through conversation and medical documentation. The clinical examination comprise determination of blood pressure, body mass index (BMI, lipid parameters (total cholesterol, LDL cholesterol, HDL cholesterol and triglycerides, serum urea and creatinine, urine, albumin and protein concentration. The total number of 283 patients (185 males and 98 females with HN was analyzed. The analysis revealed significantly higher proportion of males aged over 60 years with IHN. The mean age of examined hypertensive patients with IHN is 62.6±8.8 years with duration of hypertension 19.8±5.9 years. All examined patients had hypertensive retinopathy and

Long-term renoprotective effect of nisoldipine and lisinopril in type 1 diabetic patients with diabetic nephropathy

DEFF Research Database (Denmark)

Tarnow, L; Rossing, P; Jensen, C

2000-01-01

). Two patients in the lisinopril group and three patients in the nisoldipine group entered therapy for end-stage renal failure. CONCLUSIONS: Long-term treatment with lisinopril or nisoldipine has similar beneficial effects on progression of diabetic nephropathy in hypertensive type 1 diabetic patients.......OBJECTIVE: To compare the long-term effect on kidney function of a long-acting calcium antagonist (nisoldipine) versus a long-acting ACE inhibitor (lisinopril) in hypertensive type 1 diabetic patients with diabetic nephropathy. RESEARCH DESIGN AND METHODS: We performed a 4-year prospective......, randomized, double-dummy controlled study comparing nisoldipine (20-40 mg once a day) with lisinopril (10-20 mg once a day). The study was double-blinded for the first year and single-blinded thereafter. The study included 51 hypertensive type 1 diabetic patients with diabetic nephropathy. Three patients...

Combination therapy with rituximab, low-dose cyclophosphamide, and prednisone for idiopathic membranous nephropathy: a case series.

Science.gov (United States)

Cortazar, Frank B; Leaf, David E; Owens, Charles T; Laliberte, Karen; Pendergraft, William F; Niles, John L

2017-02-01

Membranous nephropathy is a common cause of the nephrotic syndrome. Treatment with standard regimens fails to induce complete remission in most patients. We evaluated the efficacy of combination therapy with rituximab, low-dose, oral cyclophosphamide, and an accelerated prednisone taper (RCP) for the treatment of idiopathic membranous nephropathy. We analyzed 15 consecutive patients with idiopathic membranous nephropathy treated with RCP at Massachusetts General Hospital. Seven patients (47%) received RCP as initial therapy, and the other eight patients (53%) received RCP for relapsing or refractory disease. All patients had at least 1 year of follow-up. The co-primary outcomes were attainment of partial and complete remission. Partial remission was defined as a urinary protein to creatinine ratio (UPCR) RCP resulted in high rates of complete remission. Larger studies evaluating this regimen are warranted.

Common variants of inflammatory cytokine genes are associated with risk of nephropathy in type 2 diabetes among Asian Indians

DEFF Research Database (Denmark)

Ahluwalia, Tarun Veer Singh; Khullar, Madhu; Ahuja, Monica

2009-01-01

Inflammatory cytokine genes have been proposed as good candidate genes for conferring susceptibility to diabetic nephropathy. In the present study, we examined the combined effect of multiple alleles of pro inflammatory cytokine genes for determining the risk of nephropathy in type 2 diabetic...

Congenital symmastia revisited

DEFF Research Database (Denmark)

Sillesen, Nanna H; Hölmich, Lisbeth R; Siersen, Hans Erik

2012-01-01

Symmastia is defined as medial confluence of the breast. The term 'symmastia' is modified from Greek (syn meaning 'together', and mastos meaning 'breast') and was first presented by Spence et al. in 1983. Two forms of symmastia exist: an iatrogenic and a congenital version. Congenital symmastia...... is a rare condition in which web-like soft tissue traverses the sternum to connect the breasts medially. The literature on congenital symmastia is limited, few cases have been published, and knowledge about ideal treatment is still insufficient....

An Unusual Case of Recurrent Pyocolpos Following Midtrimester Miscarriage Revealed as Obstructed Hemivagina with Ipsilateral Renal Agenesis (OHVIRA Syndrome

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Haldar M

2014-08-01

Full Text Available Obstructed hemivagina with ipsilateral renal agenesis (OHVIRA Syndrome is a rare congenital anomaly. It mostly presents with severe dysmenorrhea and a palpable mass due to unilateral hematocolpos. Sometime it presents in unusual way with prolonged vaginal bleeding and profuse vaginal discharge. Here we report case of an 18 years old married female with OHVIRA syndrome presenting late with symptoms of recurrent pyocolpos following dilatation and evacuation for midtrimester miscarriage that was diagnosed on the basis of MRI and managed by vaginal septotomy.

Diagnostic value of 64-slice spiral computed tomography imaging of the urinary tract during the excretory phase for urinary tract obstruction.

Science.gov (United States)

Zhao, De-Li; Jia, Guang-Sheng; Chen, Peng; Liu, Xin-Ding; Shu, Sheng-Jie; Ling, Zai-Sheng; Fan, Ting-Ting; Shen, Xiu-Fen; Zhang, Jin-Ling

2017-11-01

The present study aimed to assess the diagnostic value of 64-slice spiral computed tomography (CT) imaging of the urinary tract during the excretory phase for urinary tract obstruction. CT imaging of the urinary tract during the excretory phase was performed in 46 patients that had been diagnosed with urinary tract obstruction by B-mode ultrasound imaging or clinical manifestations. It was demonstrated that out of the 46 patients, 18 had pelvic and ureteral calculi, 12 cases had congenital malformations, 3 had ureteral stricture caused by urinary tract infection and 13 cases had malignant tumors of the urinary tract. The average X-ray dose planned for the standard CT scan of the urinary tract group 1 was 14.11±5.45 mSv, while the actual X-ray dose administered for the CT scan during the excretory phase group 2 was 9.01±4.56 mSv. The difference between the two groups was statistically significant (t=15.36; Purinary tract during the excretory phase has a high diagnostic value for urinary tract obstruction.

Congenital Intrahepatic Portosystemic Shunts

Energy Technology Data Exchange (ETDEWEB)

Lee, Woong Hee; Kim, Young Tong; Jou, Sung Shick; Shin, Hyeong Cheol [Soonchunhyang University, Asan (Korea, Republic of)

2008-12-15

Intrahepatic portosystemic shunts are an anomalous connection between the portal vein and hepatic vein/IVC, which may be either congenital or acquired secondary to liver cirrhosis or portal hypertension. Cases of congenital intrahepatic shunts are usually encountered in children and may spontaneously resolve. We report 5 cases of congenital intrahepatic portosystemic shunts in neonates and an adult

Is low birth weight a risk factor for the development of diabetic nephropathy in patients with type 1 diabetes?

DEFF Research Database (Denmark)

Eshoj, O; Vaag, A; Borch-Johnsen, K

2002-01-01

OBJECTIVES: To investigate if low birth weight as a consequence of intrauterine malnutrition is a risk factor for the later development of diabetic nephropathy. DESIGN AND SUBJECTS: In a case-control set-up a group of type 1 diabetic subjects with diabetic nephropathy (n = 51) and a matched control...... group with normal kidney function (n = 51) were compared. Diabetic nephropathy and normal kidney function were defined as urinary albumin excretion rate above 200 microg min-1 and below 20 microg min-1, respectively. The birth weights were all obtained from the midwives' original records. SETTING......: The patients were identified from a population-based study of chronic diabetic complications in the Funen County, Denmark. MAIN OUTCOMES: Birth weights according to the presence of diabetic nephropathy. RESULTS: The median (10-90 percentile) birth weights were 3,600 g (2,960-4,274) in the group with diabetic...

Staged surgical management of gastroschisis with severe viscero-abdominal disproportion complicated by membranous intestinal obstruction

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Sliepov O.

2017-01-01

Full Text Available Introduction. Incidence of gastroschisis (GS complicated by intestinal atresia is 4.5–12.6% of cases. 80% of this obstructions are observed in jejunoileal segment. Association of GS with intestinal atresia (GIA is usually diagnosed well, but at birth, diagnosis of atresia is often controversial. There is misdiagnosis of atresia during the initial surgery in about 12% of cases. The ideal treatment model of GIA had not already been set. Different surgical approaches were described in literature: abdominal closure modalities, terms of intestinal anastomoses creation and intestinal stomas formation remain outstanding issues. the severity of congenital defects may vary thus the tactics and strategy of surgical management as well as the ability to create primary intestinal anastomosis should be considered individually. Clinical case. GS with severe viscero-abdominal disproportion (VAD in fetus was diagnosed prenatally by ultrasonography (US at 21 week of gestation. Prenatal US follow-up monitoring and examinations were conducted. The presence of concomitant intestinal obstruction was suspected at 35 week of gestation. Regarding diagnosed malformations, according to developed in our clinic "First Minutes Surgery" tactics, operation was performed in 15 minutes after birth. During the initial surgery intestinal obstruction was not confirmed. Staged surgical management of GS was conducted. Intestinal obstruction was diagnosed and confirmed by radiography only after 21 days of age, after increasing of oral intakes volume to 15.0 ml. Jejunal web was found on surgery. Following web removal diamond-shaped anastomosis was created. During post-op period the recurrence of intestinal obstruction was not observed, full enteral nutrition was reached. The good outcome after exhibited surgical management was achieved. Conclusions. The proposed strategy and tactics of surgical management found to be effective and can be applied in the case of GS with severe VAD

Membranous nephropathy in the older adult: epidemiology, diagnosis and management.

NARCIS (Netherlands)

Deegens, J.K.J.; Wetzels, J.F.M.

2007-01-01

Membranous nephropathy is the most important cause of the nephrotic syndrome in elderly patients (aged >65 years). The clinical presentation is similar in older and younger patients, although elderly patients more often present with renal failure. Notably, glomerular filtration rate (GFR) is usually

Congenital heart disease

Science.gov (United States)

Congenital heart disease (CHD) is a problem with the heart's structure and function that is present at birth. ... Fraser CD, Kane LC. Congenital heart disease. In: Townsend CM Jr, ... Sabiston Textbook of Surgery: The Biological Basis of Modern ...

Genetics Home Reference: congenital hypothyroidism

Science.gov (United States)

... Facebook Twitter Home Health Conditions Congenital hypothyroidism Congenital hypothyroidism Printable PDF Open All Close All Enable Javascript to view the expand/collapse boxes. Description Congenital hypothyroidism is a partial or complete loss of function ...

[Neonatal tumours and congenital malformations].

Science.gov (United States)

Berbel Tornero, O; Ortega García, J A; Ferrís i Tortajada, J; García Castell, J; Donat i Colomer, J; Soldin, O P; Fuster Soler, J L

2008-06-01

The association between pediatric cancer and congenital abnormalities is well known but, there is no exclusive data on the neonatal period and the underlying etiopathogenic mechanisms are unknown. First, to analyze the frequency of neonatal tumours associated with congenital abnormalities; and second, to comment on the likely etiopathogenic hypotheses of a relationship between neonatal tumours and congenital abnormalities. Historical series of neonatal tumours from La Fe University Children's Hospital in Valencia (Spain), from January 1990 to December 1999. Histological varieties of neonatal tumours and associated congenital abnormalities were described. A systematic review of the last 25 years was carried out using Medline, Cancerlit, Index Citation Science and Embase. The search profile used was the combination of "neonatal/congenital-tumors/cancer/neoplasms" and "congenital malformations/birth defects". 72 neonatal tumours were identified (2.8% of all pediatric cancers diagnosed in our hospital) and in 15 cases (20.8%) there was some associated malformation, disease or syndrome. The association between congenital abnormalities and neonatal tumours were: a) angiomas in three patients: two patients with congenital heart disease with a choanal stenosis, laryngomalacia; b) neuroblastomas in two patients: horseshoe kidney with vertebral anomalies and other with congenital heart disease; c) teratomas in two patients: one with cleft palate with vertebral anomalies and other with metatarsal varus; d) one tumour of the central nervous system with Bochdaleck hernia; e) heart tumours in four patients with tuberous sclerosis; f) acute leukaemia in one patient with Down syndrome and congenital heart disease; g) kidney tumour in one case with triventricular hydrocephaly, and h) adrenocortical tumour: hemihypertrophy. The publications included the tumours diagnosed in different pediatric periods and without unified criteria to classify the congenital abnormalities. Little data

The effects of vitamin-E on oxidative stress and metabolic imbalance induced by acute unilateral ureteral obstruction in anaesthetized rats

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Shirazi M

2008-12-01

Full Text Available "nBackground: Obstructive nephropathy has been associated with disorders in metabolism state and oxidative balance of kidney. Stress oxidative play a key role in the pathophysiological processes of renal diseases. The objective of this study was to investigate effects of vitamin-E, as a powerful antioxidant, on renal oxidative stress and metabolism defect induced by 24-hr unilateral ureteral obstruction (UUO. "nMethods: Anesthetized male Sprague-Dawley rats (n=10 in each group were sterilely operated to occlude the left ureter. In UUO+NS, we had a single dose normal saline injection and in UUO+VitE and UUO+OO groups, D-α-tocopherol (50 mg/kg, the main component of vitamin-E, and its vehicle (Olive Oil, respectively, were twicely infused I.P. before and after UUO-induction. There were also sham-operated and control groups. 24-hr after of UUO-induction, both kidneys were removed and stored in -70°C. To determine metabolism condition, the levels of ATP and ADP; and to evaluate redox state, the levels of malondialdehyde (MDA and ferric reducing/antioxidant power (FRAP of kidneys were assessed. "nResults: The comparisons between UUO+NS and sham groups indicated that UUO increased MDA (p<0.001 and ADP (p<0.05, but decreased FRAP, and ATP/ADP ratio in obstructed kidney (all p<0.001. In UUO+VitE group, MDA and FRAP were equal to their levels in sham group, while ATP, ADP and ATP/ADP ratio were not different from those of UUO+NS group in obstructed kidney. "nConclusion: Twenty four hour of UUO caused renal reduction in oxidative metabolism and elevations in reactive oxygen species; and administration of vitamin-E, although considerably ameliorated the oxidative stress, could not improve the defected metabolism.

XbaI GLUT1 Gene Polymorphism and the Risk of Type 2 Diabetes with Nephropathy

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Ioannis Stefanidis

2009-01-01

Full Text Available Altered expression of the facilitated glucose transporter GLUT1 affects pathways implicated in the pathogenesis of diabetic nephropathy. There is indication that variation of GLUT1 gene (SLC2A1 contributes to development of microangiopathy in diabetes mellitus type 2 (DM patients. A genetic association study involving Caucasians was carried out to investigate the role of XbαI polymorphism in the GLUT1 gene in diabetic nephropathy (DN. Study population (n = 240 consisted of 148 unrelated patients with DM (92 cases with diabetic nephropathy (DN, and of 92 matched healthy control subjects. Diabetic nephropathy was defined as persistent albuminuria (> 300 mg/24 h and/or renal failure, in the absence of non-diabetes induced renal disease. The analysis showed that the risk of developing DM and DN in XbaI(− carriers, when healthy individuals were considered as controls, was two-fold: odds ratio (OR 2.08 [95% confidence interval (1.14–3.79]. However, there was no evidence of association between XbaI(− and DN when patients with DM and without DN were considered as controls: OR = 1.12 (0.55–2.26. Thus, the GLUT1 XbaI(− allele is associated with DM, and possibly with a more severe form of the disease that can lead to development of DN.

ACE serum level and I/D gene polymorphism in children with obstructive uropathies and other congenital anomalies of the kidney and urinary tract.

Science.gov (United States)

Kostadinova, Emilya Stambolova; Miteva, Lyuba Dineva; Stanilova, Spaska Angelova

2017-08-01

The aim of this study was to investigate the association of an insertion/deletion (I/D) polymorphism in angiotensin-converting enzyme (ACE) gene with serum ACE level in relation to the type and severity of malformations from congenital anomalies of the kidney and urinary tract (CAKUT) spectrum. A group of 134 Bulgarian children with CAKUT divided into four subgroups according to the leading malformation and 109 controls were genotyped by classical polymerase chain reaction. The quantitative determination of serum ACE was performed by ELISA method. A significant elevation of DD-genotype was observed in high-grade hydronephrosis compared to low-grade (43% vs. 9%). The carrying of DD-genotype was associated with higher risk for severe hydronephrosis with OR = 7.5 (95% CI: 1.242÷45.278; P = 0.028). Also, elevated serum ACE concentrations in patients with high-grade compared to low-grade hydronephrosis (237.4 ± 45 ng/mL vs 180.5 ± 64 ng/mL; P = 0.0065) were found. ACE level was significantly lower in patients with unilateral renal agenesis; hypo/dysplasia and multicystic dysplastic kidney (156.6 ± 54 ng/mL) than controls (200.6 ± 56.7 ng/mL; P = 0.005) and the remaining CAKUT subgroups. The DD genotype of I/D ACE polymorphism encodes the highest serum ACE level may be an additional genetic risk factor contributing to the severe hydronephrosis in Bulgarian patients with obstructive uropathies in contrast to other investigated categories of CAKUT malformations. © 2016 Asian Pacific Society of Nephrology.

Fetomaternal outcome in obstructed labor in a peripheral tertiary care hospital

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Sabyasachi Mondal

2013-01-01

Full Text Available Background: Obstructed labor is the leading cause of hospitalization, comprising up to 39% of all obstetric patients in developing countries. It is the single most important cause of maternal death and is one of the three leading causes of perinatal mortality. Objectives: To study the different parameters of obstructed labor cases, like its frequency, etiology, management, and complications. This will help to formulate a positive strategy to prevent obstructed labor and its consequences. Materials and Methods: 313 patients admitted with features of obstructed labor were studied. By clinical examination, diagnosis of cephalopelvic disproportion, malposition, malpresentation, obstruction in birth canal, overdistended bladder, hematuria, rupture of uterus, and supermolding of fetal head was done. Screening for maternal exhaustion, dehydration, genital sepsis, pyrexia, rupture of uterus, postpartum hemorrhage, vesico-vaginal fistula, and shock was carried out. Deaths as a consequence of obstructed labor were noted. Diagnosis of asphyxiated or dead fetus or neonatal death was done. Mode of delivery and time interval between referral, admission, intervention done, and related feto-maternal outcome were noted. Statistical analysis was done using Epi Info software. Results: Majority of the patients (87.86% were from low socioeconomic group, 88.82% were from rural areas, 16.16% were illiterate, and 27.79% were unbooked. The commonest cause of obstructed labor was cephalopelvic disproportion (55.59%. Other causes were malposition (23%, malpresentation (18.21%, fetal congenital abnormality (1.28%, myoma (0.32%, and non-dilatation of cervix (1.60%. The commonest mode of delivery was cesarean section (85.94%. Instrumental deliveries formed 9.58%. Destructive operation was done in 1.92% cases. Rupture uterus was seen in 8 (2.56% cases, out of which hysterectomy was done in 5 cases and repair in 3 cases. The common maternal complications were sepsis [pyrexia

Congenital heat disease

International Nuclear Information System (INIS)

Higgins, C.B.; Silverman, N.H.; Kersting-Somerhoff, B.A.

1990-01-01

The book covers the tomographic anatomy of the normal and congenitally malformed heart and tomographic imaging of the normal heat. It then compares echocardiographic evaluation and the use of MR imaging in the diagnosis and evaluation of individual congenital cardiac malformations

Antiphospholipid syndrome (APS) nephropathy in catastrophic, primary, and systemic lupus erythematosus-related APS.

Science.gov (United States)

Tektonidou, Maria G; Sotsiou, Flora; Moutsopoulos, Haralampos M

2008-10-01

Renal involvement in antiphospholipid syndrome (APS) has been poorly recognized. A renal small-vessel vasculopathy, defined as APS nephropathy, has recently been observed in small series of patients with primary APS (PAPS) and systemic lupus erythematosus (SLE)-APS. We examined the renal histologic, clinical, and laboratory characteristics of different groups of patients with APS including catastrophic APS (CAPS). Our study included all CAPS (n=6), PAPS (n=8), and SLE-APS (n=23) patients with biopsy-proven renal involvement who were referred to our departments. The kidney biopsy specimens were retrospectively examined by the same renal pathologist. APS nephropathy was diagnosed as previously described. Demographic, clinical, and laboratory data were recorded. All patients with CAPS had acute and chronic renal vascular lesions compatible with diagnosis of APS nephropathy. Thrombotic microangiopathy (TMA), the acute lesion, was observed in all CAPS patients. Fibrous intimal hyperplasia of interlobular arteries (FIH) and focal cortical atrophy (FCA) were the most common chronic vascular lesions, occurring in 4 of 6 (66.7%) and 3 of 6 (50%) patients with CAPS, respectively. TMA was detected in 3 of 8 (37.5%) patients with PAPS and in 8 of 23 (35%) patients with SLE-APS, while FIH and FCA were found with similar frequencies in all 3 groups. Hypertension, proteinuria, hematuria, and renal insufficiency were the most common renal manifestations of all APS groups. Acute and chronic APS nephropathy lesions were detected in all 3 APS groups. Acute lesions were more prominent in CAPS, while chronic lesions were found with similar frequencies in all groups. Hypertension, proteinuria, hematuria, and renal insufficiency were the most common renal manifestations of all APS groups.

[Severe iatrogenic airway obstruction due to lingual lymphangioma].

Science.gov (United States)

Segado Arenas, A; Flores González, J-C; Rubio Quiñones, F; Quintero Otero, S; Hernández González, A; Pantoja Rosso, S

2011-09-01

Lymphangioma of the tongue is a rare and benign tumour involving congenital and cystic abnormalities derived from lymphatic vessels. Treatment modalities include surgery and a large number of different intralesional injections of sclerosing agents. Presently, OK-432 (Picibanil(®)) is the preferred sclerosant and when administered intralesionally will result in inflammation, sclerosis, and cicatricial contraction of the lesion. We report a case of microcystic lymphangioma of the tongue in a 5-year-old boy treated with an intralesional injection of OK-432. In the immediate postoperative period, the patient suffered severe diffuse swelling, progressive upper airway obstruction with inspiratory stridor, and respiratory distress requiring emergency fiberoptic nasotracheal intubation. Although OK-432 injections are found to be safe and effective as a first line of treatment for lymphangiomas, local swelling with potentially life-threatening airway compromise should be anticipated, especially when treating lesions near the upper airway. Copyright © 2011 Elsevier Masson SAS. All rights reserved.

Congenital orbital teratoma.

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Aiyub, Shereen; Chan, Wengonn; Szetu, John; Sullivan, Laurence J; Pater, John; Cooper, Peter; Selva, Dinesh

2013-12-01

We present a case of mature congenital orbital teratoma managed with lid-sparing exenteration and dermis fat graft. This is a case report on the management of congenital orbital teratoma. A full-term baby was born in Fiji with prolapsed right globe which was surrounded by a nonpulsatile, cystic mass. Clinical and imaging features were consistent with congenital orbital teratoma. Due to limited surgical expertise, the patient was transferred to Adelaide, Australia for further management. The patient underwent a lid-sparing exenteration with frozen section control of the apical margin. A dermis fat graft from the groin was placed beneath the lid skin to provide volume. Histopathology revealed mature tissues from each of the three germ cell layers which confirmed the diagnosis of mature teratoma. We describe the successful use of demis fat graft in socket reconstruction following lid-sparing exenteration for congenital orbital teratoma.

Congenital orbital teratoma

Directory of Open Access Journals (Sweden)

Shereen Aiyub

2013-01-01

Full Text Available We present a case of mature congenital orbital teratoma managed with lid-sparing exenteration and dermis fat graft. This is a case report on the management of congenital orbital teratoma. A full-term baby was born in Fiji with prolapsed right globe which was surrounded by a nonpulsatile, cystic mass. Clinical and imaging features were consistent with congenital orbital teratoma. Due to limited surgical expertise, the patient was transferred to Adelaide, Australia for further management. The patient underwent a lid-sparing exenteration with frozen section control of the apical margin. A dermis fat graft from the groin was placed beneath the lid skin to provide volume. Histopathology revealed mature tissues from each of the three germ cell layers which confirmed the diagnosis of mature teratoma. We describe the successful use of demis fat graft in socket reconstruction following lid-sparing exenteration for congenital orbital teratoma.

Multidetector row computed tomography in bowel obstruction. Part 2. Large bowel obstruction

Energy Technology Data Exchange (ETDEWEB)

Sinha, R. [Department of Radiology, Glenfield Hospital, Leicester (United Kingdom)]. E-mail: rakesh.sinha@uhl-tr.nhs.uk; Verma, R. [Department of Radiology, Glenfield Hospital, Leicester (United Kingdom)

2005-10-01

Large bowel obstruction may present as an emergency as high-grade colonic obstruction and can result in perforation. Perforated large bowel obstruction causes faecal peritonitis, which can result in high morbidity and mortality. Multidetector row computed tomography (MDCT) has the potential of providing an accurate diagnosis of large bowel obstruction. The rapid acquisition of images within one breath-hold reduces misregistration artefacts than can occur in critically ill or uncooperative patients. The following is a review of the various causes of large bowel obstruction with emphasis on important pathogenic factors, CT appearances and the use of multiplanar reformatted images in the diagnostic workup.

Radiographic features of congenital segmental dilation of the intestine in a german shepherd dog.

Science.gov (United States)

Johnson, Laura; Simone, Kristina; Cullen, John; Talley, Ashley; Cohen, Eli B

2017-12-04

A 10-week-old spayed female German Shepherd Dog was presented for acute vomiting and diarrhea. There was no reported foreign body or toxin ingestion. Radiographs showed a severely (∼11 × 7 cm), focally distended right abdominal intestinal segment containing gas and soft tissue material. Other small intestinal segments were segmentally gas distended. Celiotomy identified a ∼9 cm focally dilated segment at the jejunoileal junction with no aborad luminal obstruction. Resection and anastomosis of the dilation was performed. Histopathology showed mild mucosal inflammation, but otherwise normal wall layering and autonomic ganglia. Radiographic and histopathologic findings were consistent with congenital segmental dilation of the intestine. © 2017 American College of Veterinary Radiology.

Burden and impact of congenital syndromes and comorbidities among adults with congenital heart disease.

Science.gov (United States)

Bracher, Isabelle; Padrutt, Maria; Bonassin, Francesca; Santos Lopes, Bruno; Gruner, Christiane; Stämpfli, Simon F; Oxenius, Angela; De Pasquale, Gabriella; Seeliger, Theresa; Lüscher, Thomas F; Attenhofer Jost, Christine; Greutmann, Matthias

2017-08-01

Our aim was to assess the overall burden of congenital syndromes and non-cardiac comorbidities among adults with congenital heart disease and to assess their impact on circumstances of living and outcomes. Within a cohort of 1725 adults with congenital heart defects (65% defects of moderate or great complexity) followed at a single tertiary care center, congenital syndromes and comorbidities were identified by chart review. Their association with arrhythmias, circumstances of living and survival was analyzed. Within the study cohort, 232 patients (13%) had a genetic syndrome, 51% at least one comorbidity and 23% ≥2 comorbidities. Most prevalent comorbidities were systemic arterial hypertension (11%), thyroid dysfunction (9%), psychiatric disorders (9%), neurologic disorders (7%), chronic lung disease (7%), and previous stroke (6%). In contrast to higher congenital heart defect complexity, the presence of comorbidities had no impact on living circumstances but patients with comorbidities were less likely to work full-time. Atrial arrhythmias were more common among patients with moderate/great disease complexity and those with comorbidities but were less common among patients with congenital syndromes (pCongenital syndromes and comorbidities are highly prevalent in adults with congenital heart disease followed at specialist centers and add to the overall complexity of care. The presence of these additional factors has an impact on living circumstances, is associated with arrhythmias and needs to be further explored as prognostic markers. Copyright © 2017 Elsevier B.V. All rights reserved.

Congenital cytomegalovirus infection

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Katarina Rednak-Paradiž

2006-11-01

Full Text Available Background: CMV is the most common agent that causes congenital virus infection. Only 10 % of infected children have symptomatic infection immediately after birth. Signs of central nervous system damage, neurosensory deafness and delayed psychomotor development may manifest as a result of asimptomatic congenital infection later in childhood. In the article we present basic properties of CMV; we describe clinical picture of the congenital infection and possibilities of diagnose and its treatment. We present five children with symptomatic congenital CMV infection that were hospitalized for the period 1992–2002 at the Neonatal department in the University Children’s Hospital in Ljubljana.Conclusions: Identification of infected neonates, especially those with asimptomatic congenital CMV infection, is difficult. Latest incidence of infection in Slovenia is unknown. With new investigations the efficiency of antiviral therapy was discovered but exact indications for therapy are not yet known. CMV vaccine, once available, may ultimately be the best control strategy for this important public health problem. Proper educating women in childbearing age about the risks of CMV and how to avoid disease transmission during pregnancy (hand washing, avoiding mouth-to-mouth contact with preschool children, usage of gloves especially when handling dipers or respiratory secretions are the only control strategies available.

Is renal medullary carcinoma the seventh nephropathy in sickle cell ...

African Journals Online (AJOL)

Introduction: Previous studies had enlisted renal medullary carcinoma (RMC) as the seventh nephropathy in sickle cell disease (SCD). Clinical experience has contradicted this claim and this study is targeted at refuting or supporting this assumption. Objective: To estimate the prevalence of RMC and describe other renal ...

Changes of platelet GMP-140 in diabetic nephropathy and its multi-factor regression analysis

International Nuclear Information System (INIS)

Wang Zizheng; Du Tongxin; Wang Shukui

2001-01-01

The relation of platelet GMP-140 and its related factors with diabetic nephropathy was studied. 144 patients of diabetic mellitus without nephropathy (group without DN, mean suffering duration of 25.5 +- 18.6 months); 80 with diabetic nephropathy (group DN, mean suffering duration of 58.7 +- 31.6 months) and 50 normal controls were chosen in the research. Platelet GMP-140, plasma α 1 -MG, β 2 -MG, and 24 hour urine albumin (ALB), IgG, α 1 -MG, β 2 -MG were detected by RIA, while HBA 1 C via chromatographic separation and FBG, PBG, Ch, TG, HDL, FG via biochemical methods. All the data had been processed with software on computer with t-test and linear regression, and multi-factor analysis were done also. The levels of platelet GMP-140, FG, DBP, TG, HBA 1 C and PBG in group DN were significantly higher than those of group without DN and normal control (P 0.05), while they were higher than those of normal controls. Multi-factor analysis of platelet GMP-140 with TG, DBP and HBA 1 C were performed in 80 patients with DN (P 1 C are the independent factors enhancing the activation of platelets. The disturbance of lipid metabolism in type II diabetic mellitus may also enhance the activation of platelets. Elevation of blood pressure may accelerate the initiation and deterioration of DN in which change of platelet GMP-140 is an independent factor. Elevation of HBA 1 C and blood glucose are related closely to the diabetic nephropathy

Prevalence of congenital amusia.

Science.gov (United States)

Peretz, Isabelle; Vuvan, Dominique T

2017-05-01

Congenital amusia (commonly known as tone deafness) is a lifelong musical disorder that affects 4% of the population according to a single estimate based on a single test from 1980. Here we present the first large-based measure of prevalence with a sample of 20 000 participants, which does not rely on self-referral. On the basis of three objective tests and a questionnaire, we show that (a) the prevalence of congenital amusia is only 1.5%, with slightly more females than males, unlike other developmental disorders where males often predominate; (b) self-disclosure is a reliable index of congenital amusia, which suggests that congenital amusia is hereditary, with 46% first-degree relatives similarly affected; (c) the deficit is not attenuated by musical training and (d) it emerges in relative isolation from other cognitive disorder, except for spatial orientation problems. Hence, we suggest that congenital amusia is likely to result from genetic variations that affect musical abilities specifically.

Effect of antihypertensive treatment on progression of incipient diabetic nephropathy

DEFF Research Database (Denmark)

Christensen, Cramer; Mogensen, C E

1985-01-01

of urinary albumin excretion before and during 2.6 years +/- 1.0 (SD) of treatment. The blood pressure was depressed by the treatment (systolic blood pressure from 135 mm Hg +/- 8.6 to 124 mm Hg +/- 6.2, NS; mean blood pressure from 107 mm Hg +/- 7.6 to 97 mm Hg +/- 3.4, 2p less than 0.05; diastolic blood......The aim of the study was to clarify whether antihypertensive treatment with a selective beta blocker would have an effect on the progression rate of kidney disease in patients with incipient diabetic nephropathy. Six male patients with juvenile-onset diabetes with incipient nephropathy (urinary...... albumin excretion above 15 micrograms/min and total protein excretion below 0.5 g/24 hr) were treated with metoprolol (200 mg daily). At the start of the antihypertensive treatment the mean age was 32 years +/- 4.2 (SD). The patients were followed a mean 5.4 years +/- 3.1 (SD) with repeated measurements...

Congenital Leukemia in Down's syndrome

International Nuclear Information System (INIS)

Iqbal, W.; Khan, F.; Muzaffar, M.; Khan, U. A.; Rehman, M. U.; Khan, M. A.; Bari, A.

2006-01-01

Congenital Leukemia is a condition and often associated with fatal outcome/sup 1/. Most of the neonatal cases reported have acute non-lymphoblastic leukemia, in contrast to the predominance of acute lymphoblastic leukemia found in later childhood. congenital leukemia is occasionally associated with number of congenital anomalies and with chromosomal disorders such as Down's syndrome. Subtle cytogenetic abnormalities may occur more commonly in the affected infants and their parents, when studied with newer cytogenetic techniques/sup 2/. Inherent unstable hematopoieses resulting from chromosomal aberration in children with Downs's syndrome can present with transient myeloproliferative disorder, mimicking leukemia which undergoes spontaneous recovery/sup 3/. Only few cases of congenital leukemia with Downs syndrome, presented as congenital leukemia. (author)

[Congenital cystic lung lesions--review of the literature with three clinical cases].

Science.gov (United States)

Slancheva, B; Hitrova, S; Markov, D; Vakrilova, L; Pramatarova, T; Yarukova, N; Brankov, O

2013-01-01

Congenital cystic lung lesions are rare. Mainly affects the lower respiratory patishta.i are congenital cystic malformation and adematozna bronchopulmonary sequestration (BPS). The pathogenesis of the occurrence of these malformations is not clear but they have a common clinical course. In most cases, the anomaly is asymptomatic and occurs with infections of the lung during the first year of life. Currently congenital lung lesions were classified into five types and is considered by most authors. The anomaly is due to the abnormal proliferation of terminal bronchioles accompanied by inhibition of alveolar development between 7-17 weeks, obstructed airway dysplasia and metaplasia of normal lung tissue. Early diagnosis is vital in making a medical decision on how to treat CCAM. Associated with abnormalities of the urinary tract, cardiovascular system, gastrointestinal atresia, diaphragmatic hernia skeletal abnormalities. In pregnancies in which prenatal lung lesions weighs registered necessary series of ultrasound examinations to track finding and using the Doppler to assess how the blood supply of the fault. The clinical presentation of malformations is respiratory distress, respiratory infection, and dyspnea. The use of CT and MRA allows better visualization of the pulmonary lesions. With its combination with arteriography and bronchoscopy are used to differentiate CCAM and pulmonary sequestration. We present three cases with lung lesions were born in Neonatologia clinic at the University Hospital of Obstetrics and Gynecology "Maternity" Sofia for the period 2010-2012 three cases CCAMs type 1, operated by 5 meters after birth with a good final outcome without complications in the postoperative period and lack of pulmonary symptoms up to 1 year after birth.

Prevalence of congenital anomalies in newborns with congenital heart disease diagnosis

International Nuclear Information System (INIS)

Egbe, Alexander; Lee, Simon; Ho, Deborah; Uppu, Santosh; Srivastava, Shubhika

2014-01-01

There is a known association between congenital heart disease (CHD) and other congenital anomalies (CA). These associations have been altered by changes in prenatal factors in recent time. We reviewed the largest database of inpatient hospitalization information and analyzed the current association between common CHD diagnoses and other congenital anomalies. Case-control study design. We reviewed the Nationwide Inpatient Sample (NIS) database from 1998 to 2008 and identified all live births with CHD diagnosis (case) and live births without CHD diagnosis (control). We compared prevalence of associated congenital anomalies between the case and control groups. Our cohort consisted of 97,154 and 12,078,482 subjects in the case and control groups, respectively. In the CHD population, prevalence of non-syndromic congenital anomaly (NSCA), genetic syndrome (GS), and overall extra-cardiac congenital anomaly (CA) were 11.4, 2.2, and 13.6%, respectively. In the control group, prevalence of NSCA, GS, and CA were 6.7, 0.3, and 7.0%, respectively. NSCA (odds ratio (OR): 1.88, confidence interval (CI): 1.73-1.94), GS (OR 2.52, CI 2.44-2.61), and overall CA (OR: 2.01, CI: 1.97-2.14) were strongly associated with CHD. Prevalence of GS and multiple organ-system CA decreased significantly over the study period. This is the largest and most comprehensive population-based study evaluating association between CHD and extra-cardiac malformation (ECM) in newborns. There was significant decrease in prevalence of GS and multiple CA over the study period

Congenital heart defects and extracardiac malformations.

Science.gov (United States)

Rosa, Rosana Cardoso M; Rosa, Rafael Fabiano M; Zen, Paulo Ricardo G; Paskulin, Giorgio Adriano

2013-06-01

To review the association between congenital heart defects and extracardiac malformations. Scientific articles were searched in the Medline, Lilacs, and SciELO databases, using the descriptors "congenital heart disease," "congenital heart defects," "congenital cardiac malformations," "extracardiac defects," and "extracardiac malformations." All case series that specifically explored the association between congenital heart defects and extracardiac malformations were included. Congenital heart diseases are responsible for about 40% of birth defects, being one of the most common and severe malformations. Extracardiac malformations are observed in 7 to 50% of the patients with congenital heart disease, bringing a greater risk of comorbidity and mortality and increasing the risks related to heart surgery. Different studies have attempted to assess the presence of extracardiac abnormalities in patients with congenital heart disease. Among the changes described, those of the urinary tract are more often reported. However, no study has evaluated all patients in the same way. Extracardiac abnormalities are frequent among patients with congenital heart disease, and patients with these alterations may present an increased risk of morbimortality. Therefore, some authors have been discussing the importance and cost-effectiveness of screening these children for other malformations by complementary exams.

Congenital Heart Defects and CCHD

Science.gov (United States)

... and more. Stony Point, NY 10980 Close X Home > Complications & Loss > Birth defects & other health conditions > Congenital heart defects and ... in congenital heart defects. You have a family history of congenital heart ... syndrome or VCF. After birth Your baby may be tested for CCHD as ...

Congenital amusias.

Science.gov (United States)

Tillmann, B; Albouy, P; Caclin, A

2015-01-01

In contrast to the sophisticated music processing reported in the general population, individuals with congenital amusia show deficits in music perception and production. Congenital amusia occurs without brain damage, sensory or cognitive deficits, and has been suggested as a lifelong deficit with genetic origin. Even though recognized for a long time, this disorder has been systematically studied only relatively recently for its behavioral and neural correlates. The currently most investigated hypothesis about the underlying deficits concerns the pitch dimension, notably with impaired pitch discrimination and memory. Anatomic and functional investigations of pitch processing revealed that the amusic brain presents abnormalities in the auditory and inferior frontal cortices, associated with decreased connectivity between these structures. The deficit also impairs processing of pitch in speech material and processing of the time dimension in music for some of the amusic individuals, but does not seem to affect spatial processing. Some studies suggest at least partial dissociation in the disorder between perception and production. Recent studies revealed spared implicit pitch perception in congenital amusia, supporting the power of implicit cognition in the music domain. Current challenges consist in defining different subtypes of congenital amusia as well as developing rehabilitation programs for this "musical handicap." © 2015 Elsevier B.V. All rights reserved.

Assessment for markers of nephropathy in newly diagnosed type 2 ...

African Journals Online (AJOL)

Objective: To assess for markers of nephropathy in newly diagnosed type 2 diabetics, using blood pressure levels, endogenous creatinine clearance and urinary protein excretion as markers of renal disease. Study design: Ninety newly diagnosed type 2 diabetics were studied within 6 weeks of diagnosis. They were in ...

Attenuation of Diabetic Nephropathy in Otsuka Long-Evans Tokushima Fatty (OLETF Rats with a Combination of Chinese Herbs (Tangshen Formula

Directory of Open Access Journals (Sweden)

Haojun Zhang

2011-01-01

Full Text Available Diabetic nephropathy is one of the most significant microvascular complications in patients with type 2 diabetics. The concise mechanism of diabetic nephropathy is unknown and there is no successful treatment. The objective of study was to investigate effects of Chinese herbs (Tangshen Formula on diabetic nephropathy in Otsuka Long-Evans Tokushima Fatty (OLETF rats. OLETF rats and LETO rats were divided into four groups: LETO control, OLETF diabetics, OLETF diabetics treated with Tangshen Formula, and OLETF diabetics treated with Monopril. Body weight, blood glucose, and 24 h urinary proteins were measured once every four weeks. Blood samples and kidney tissues were obtained for analyses of total cholesterol, triglyceride, whole blood viscosity, plasma viscosity, and pathohistological examination at 36 and 56 weeksrespectively. Untreated OLETF rats displayed diabetic nephropathy over the study period. Treatment of OLETF rats with Tangshen Formula attenuated the increases in blood glucose, body weight, 24 h urinary protein content, serum total cholesterol, whole blood viscosity and plasma viscosity at certain time. Treatment with Tangshen Formula also reduced glomerulosclerotic index and interstitial fibrotic index seen in OLETF rats. In conclusion, Tangshen Formula could attenuate the development of diabetic nephropathy in OLETF rat diabetic model.

Lisinopril Protects Against the Adriamycin Nephropathy and Reverses the Renalase Reduction: Potential Role of Renalase in Adriamycin Nephropathy

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Pengxun Han

2013-09-01

Full Text Available Aims: To investigate the potential role of renalase in adriamycin nephropathy and the effect of lisinopril on the regulation of renalase. Methods: Adriamycin nephropathy was induced in male Wistar rats (n=12 by a single injection of adriamycin at 2 mg/kg body weight. Rats were then randomly assigned to a model group or a treatment group, to which were administered distilled water or the angiotensin converting enzyme inhibitor lisinopril, respectively, for 12 weeks. Six normal rats served as controls. At the end of study, physiological parameters and systolic blood pressure were measured. Glomerulosclerosis and tubulointerstitial injury were assessed by histopathology Renalase protein expression in kidney was quantified by immunohistochemistry and immunoblotting. The serum concentration and urinary excretion of renalase were determined by enzyme-linked immunosorbent assay. Results: In model group rats, proteinuria and systolic blood pressure were elevated. Increased serum renalase concentration was observed; however, renalase protein expression in the kidney was significantly decreased. Compared with the model group, decreased proteinuria, lower systolic blood pressure, and fewer morphologic lesions were detected in the treatment group. Although levels of serum renalase were similar, accumulation of renalase in urine and kidney tissue increased notably in the treatment group compared with the model group. Conclusions: This study suggests that renalase may be involved in the process of adriamycin-induced renal injuries. Lisinopril may attenuate adriamycin-induced kidney injury by controlling blood pressure, which may be partially attributed to the renalase expression and secretion.

Coffin-Siris syndrome with the rarest constellation of congenital cardiac defects: A case report with review of literature

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Lalita Nemani

2014-01-01

Full Text Available We report a case of type-A Coffin-Siris syndrome (CSS with a unique constellation of congenital heart defects. A 17-year-old Indian boy was referred to our hospital for central cyanosis with features of right heart failure. The cardiac abnormalities included biventricular outflow tract obstruction, small atrial septal defect (ASD, subaortic ventricular septal defect, drainage of left superior venacava to left atrial appendage, and aortic arch anomaly. Patient underwent successful right ventricular infundibular resection, subaortic membrane resection, closure of atrial and ventricular septal defect, rerouting left superior vena cava to left pulmonary artery and aortic valve replacement.

Coffin-Siris syndrome with the rarest constellation of congenital cardiac defects: A case report with review of literature.

Science.gov (United States)

Nemani, Lalita; Barik, Ramachandra; Patnaik, Amar Narayana; Mishra, Ramesh C; Rao, Amaresh M; Kapur, Pragati

2014-09-01

We report a case of type-A Coffin-Siris syndrome (CSS) with a unique constellation of congenital heart defects. A 17-year-old Indian boy was referred to our hospital for central cyanosis with features of right heart failure. The cardiac abnormalities included biventricular outflow tract obstruction, small atrial septal defect (ASD), subaortic ventricular septal defect, drainage of left superior venacava to left atrial appendage, and aortic arch anomaly. Patient underwent successful right ventricular infundibular resection, subaortic membrane resection, closure of atrial and ventricular septal defect, rerouting left superior vena cava to left pulmonary artery and aortic valve replacement.

In Acute IgA Nephropathy, Proteinuria and Creatinine Are in the Spot, but Podocyturia Operates in Silence: Any Place for Amiloride?

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H. Trimarchi

2017-01-01

Full Text Available IgA nephropathy is the most frequent cause of primary glomerulonephritis, portends erratic patterns of clinical presentation, and lacks specific treatment. In general, it slowly progresses to end-stage renal disease. The clinical course and the response to therapy are usually assessed with proteinuria and serum creatinine. Validated biomarkers have not been identified yet. In this report, we present a case of acute renal injury with proteinuria and microscopic hematuria in a young male. A kidney biopsy disclosed IgA nephropathy. Podocyturia was significantly elevated compared to normal subjects. Proteinuria, renal function, and podocyturia improved promptly after steroids and these variables remained normal after one year of follow-up, when steroids had already been discontinued and patient continued on valsartan and amiloride. Our report demonstrates that podocyturia is critically elevated during an acute episode of IgA nephropathy, and its occurrence may explain the grim long-term prognosis of this entity. Whether podocyturia could be employed in IgA nephropathy as a trustable biomarker for treatment assessment or even for early diagnosis of IgA nephropathy relapses should be further investigated.

Carnosine as a protective factor in diabetic nephropathy - Association with a leucine repeat of the carnosinase gene CNDP1

NARCIS (Netherlands)

Janssen, B; Hohenadel, D.; Brinkkoetter, P.; Peters, V.; Rind, N.; Fischer, C.; Rychlik, I.; Cerna, M.; Romzova, M.; de Heer, E.; Baelde, H.; Bakker, Stephan; Zirie, M.; Rondeau, E.; Mathieson, P.; Saleem, M.A.; Meyer, J.; Koppel, H.; Sauerhoefer, S.; Bartram, C.R.; Nawroth, P.; Hammes, H.P.; Yard, B.A.; Zschocke, J.; van der Woude, F.J.

The risk of diabetic nephropathy is partially genetically determined. Diabetic nephropathy is linked to a gene locus on chromosome 18q22.3-q23. We aimed to identify the causative gene on chromosome 18 and to study the mechanism by which the product of this gene could be involved in the development

A case of palpable purpura and nephropathy: Occam's Razor or Hickam's Dictum.

Science.gov (United States)

Mandhadi, Ranadeep; Kodumuri, Vamsi; Arora, Rohit; Puneet Singh, Param; Adigopula, Shashi; Chua, Serafin

2013-01-01

Vasculitis causing palpable purpura, nephropathy, and hematologic abnormalities is a well-known entity. However, sometimes, vasculitis may not be the primary cause but is part of a systemic disease. Literature suggests that infections like HIV can induce nephropathy and antineutrophilic cytoplasmic antibody-positive vasculitis, which is different from the well-known entity of "antineutrophilic cytoplasmic antibody-associated vasculitis." We present a 46-year-old female patient with a history of intravenous drug abuse who reported with a rash, swelling, and palpable purpura of the lower extremities. Peripheral smear showed no evidence of disseminated intravascular coagulation or thrombotic thrombocytopenic purpura; metabolic profile showed acute kidney injury. She was found to be HIV- and hepatitis C-positive. Immunologic workup was positive for both MPO and PR3 antineutrophilic cytoplasmic antibodies and negative for cryoglobulins; complement levels were low. Skin biopsy showed leukocytoclastic vasculitis but kidney biopsy was negative for any immunologic involvement; it showed only glomerulosclerosis. Thus, it was thought that nephropathy and vasculitis, in this case, are two distinct pathologic processes, both induced by infection (HIV and/or hepatitis C). The patient responded to low-dose steroid therapy. She was later started on the definitive therapy, the highly active antiretroviral therapy regimen. This case illustrates the fact that low-dose steroids can still be a good alternative in acute situations in patients at risk from immunosuppression.

A case of membranous nephropathy as a manifestation of graft-versus-host disease

Directory of Open Access Journals (Sweden)

Jae Hyun Han

2013-03-01

Full Text Available Nephrotic syndrome (NS rarely occurs after hematopoietic stem cell transplantation (HSCT as a late manifestation of graft-versus-host disease (GVHD. Herein, we report a case of HSCT-associated membranous nephropathy in a female patient with aplastic anemia. The patient received an allogeneic HSCT from her human leukocyte antigen-identical brother following myeloablative conditioning chemotherapy. NS occurred 21 months after HSCT without any concurrent features of chronic GVHD. The patient was treated with prednisolone and cyclosporine after renal biopsy confirmed membranous nephropathy, and achieved complete remission. Our report contradicts previous assumptions that concomitant chronic GVHD is responsible for the development of NS, suggesting that NS can develop as a new, independent manifestation of GVHD.

Congenital malaria in China.

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Zhi-Yong Tao

2014-03-01

Full Text Available BACKGROUND: Congenital malaria, in which infants are directly infected with malaria parasites from their mother prior to or during birth, is a potentially life-threatening condition that occurs at relatively low rates in malaria-endemic regions. It is recognized as a serious problem in Plasmodium falciparum-endemic sub-Saharan Africa, where recent data suggests that it is more common than previously believed. In such regions where malaria transmission is high, neonates may be protected from disease caused by congenital malaria through the transfer of maternal antibodies against the parasite. However, in low P. vivax-endemic regions, immunity to vivax malaria is low; thus, there is the likelihood that congenital vivax malaria poses a more significant threat to newborn health. Malaria had previously been a major parasitic disease in China, and congenital malaria case reports in Chinese offer valuable information for understanding the risks posed by congenital malaria to neonatal health. As most of the literature documenting congenital malaria cases in China are written in Chinese and therefore are not easily accessible to the global malaria research community, we have undertaken an extensive review of the Chinese literature on this subject. METHODS/PRINCIPAL FINDINGS: Here, we reviewed congenital malaria cases from three major searchable Chinese journal databases, concentrating on data from 1915 through 2011. Following extensive screening, a total of 104 cases of congenital malaria were identified. These cases were distributed mainly in the eastern, central, and southern regions of China, as well as in the low-lying region of southwest China. The dominant species was P. vivax (92.50%, reflecting the malaria parasite species distribution in China. The leading clinical presentation was fever, and other clinical presentations were anaemia, jaundice, paleness, diarrhoea, vomiting, and general weakness. With the exception of two cases, all patients

[Identification of risk factors for congenital malformations].

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Canals C, Andrea; Cavada C, Gabriel; Nazer H, Julio

2014-11-01

The relative importance of congenital malformations as a cause of death in the first year of life is increasing along with the control of preventable causes of perinatal mortality. To identify risk factors for congenital malformations. Retrospective case-control study of births registered in the database of The Latin American Collaborative Study of Congenital Malformations (ECLAMC), in the period 2001-2010. Birth weight and gestational age were significantly lower in cases than controls, behaving as risk factors and associated with a greater severity of congenital malformations. The risk and severity of congenital malformations increased along with mother's age. Fetal growth retardation, a history of congenital malformations in the family, physical factors and acute illnesses of the mother in the first trimester of pregnancy were also significant risk factors for congenital malformations and their severity. The educational level of the mother was a protective factor for congenital malformations and their severity. Variables previously identified as risk factors for congenital malformations, were significantly related with the occurrence of congenital malformations and their severity.

Serum Levels of Vaspin and Its Correlation with Nitric Oxide in Type 2 Diabetic Patients with Nephropathy.

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Mihanfar, Aynaz; Rahmati-Yamchi, Mohammad; Mota, Ali; Abediazar, Sima; Pilehvar-Soltanahmadi, Younes; Zarghami, Nosratollah

2018-01-01

Diabetic Nephropathy (DN), a serious and prevalent complication of diabetes, has been rapidly raising worldwide. Vaspin, as an adipokine with anti-diabetic effects, is predominantly released from visceral adipose tissue. Moreover, vaspin has the stimulatory effect on nitric oxide (NO) bioavailability through the activation of NO synthase. The aim of the present study was to investigate the serum levels of vaspin and their correlation with NO metabolite in diabetic patients with normal renal function and renal insufficiency. Volunteers patients with non-nephropathy Type 2 Diabetic Mellitus (T2DM) as control (n=40, age= 56.95±6.11 years) and patients with diabetic nephropathy (DN) (n=40, age=57.85±5.63 years) as case group were enrolled in this study, and serum samples were collected for the measurement of vaspin levels by ELISA technique. Also, serum levels of NO metabolites were calorimetrically assessed. We found that vaspin levels significantly decreased in diabetic patients with nephropathic condition as compared with diabetic patients with normal renal function (p diabetic patients with nephropathy in comparison with non-nephropathic diabetics (pdiabetic patients and increased levels of NO may be a defensive mechanism in the DN. Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.org.

Taurine Alleviates the Progression of Diabetic Nephropathy in Type 2 Diabetic Rat Model

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Jang Hyun Koh

2014-01-01

Full Text Available The overexpression of vascular endothelial growth factor (VEGF is known to be involved in the pathogenesis of diabetic nephropathy. In this study, the protective effects of taurine on diabetic nephropathy along with its underlying mechanism were investigated. Experimental animals were divided into three groups: LETO rats as normal group (n=10, OLETF rats as diabetic control group (n=10, and OLETF rats treated with taurine group (n=10. We treated taurine (200 mg/kg/day for 20 weeks and treated high glucose (HG, 30 mM with or without taurine (30 mM in mouse cultured podocyte. After taurine treatment, blood glucose level was decreased and insulin secretion was increased. Taurine significantly reduced albuminuria and ACR. Also it decreased glomerular volume, GBM thickness and increased open slit pore density through decreased VEGF and increased nephrin mRNA expressions in renal cortex. The antioxidant effects of taurine were confirmed by the reduction of urine MDA in taurine treated diabetic group. Also reactive oxygen species (ROS levels were decreased in HG condition with taurine treated podocytes compared to without taurine. These results indicate that taurine lowers glucose level via increased insulin secretion and ameliorates the progression of diabetic nephropathy through antifibrotic and antioxidant effects in type 2 diabetes rat model.

Study on Association of Pentraxin 3 and Diabetic Nephropathy in a Rat Model

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Xuehai Chen

2018-01-01

Full Text Available Diabetic nephropathy (DN is a serious microvascular complication of diabetes. Compared with other therapies for diabetic patients, islet transplantation can effectively prevent and reverse diabetes-induced microvascular disease, such as diabetic retinopathy and nephropathy. PTX3 is the only long pentraxin that can be detected in renal tissue. In this study, we investigated the expression of PTX3 when early DN was reversed after islet transplantation. Methods. Diabetes was induced in rats by injecting streptozotocin (STZ. Twelve weeks later, the diabetic rats were divided into 2 groups: the islet transplantation group (IT and the diabetic nephropathy group (DN. Renal injury, renal function, and the expression of PTX3 in the plasma and the kidneys were assessed with urinalysis, immunohistochemical staining, and Western blot, respectively. Results. The expression of PTX3 in the kidney was significantly decreased in the DN group but increased in the IT group because of the reversal of DN. Conclusions. Our data showed that the level of PTX3 in renal tissue is closely related to renal injury in DN. This may be used to quantify the extent of renal injury in DN, provide a potential early indicator of renal tubular injury in early DN patients, and assess DN clinical progression.

IgA nephropathy in systemic lupus erythematosus patients: case report and literature review

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Leonardo Sales da Silva

2016-06-01

Full Text Available Abstract Systemic erythematosus lupus (SLE is a multisystemic autoimmune disease which has nephritis as one of the most striking manifestations. Although it can coexist with other autoimmune diseases, and determine the predisposition to various infectious complications, SLE is rarely described in association with non‐lupus nephropathies etiologies. We report the rare association of SLE and primary IgA nephropathy (IgAN, the most frequent primary glomerulopathy in the world population. The patient was diagnosed with SLE due to the occurrence of malar rash, alopecia, pleural effusion, proteinuria, ANA 1: 1,280, nuclear fine speckled pattern, and anticardiolipin IgM and 280 U/mL. Renal biopsy revealed mesangial hypercellularity with isolated IgA deposits, consistent with primary IgAN. It was treated with antimalarial drug, prednisone and inhibitor of angiotensin converting enzyme, showing good progress. Since they are relatively common diseases, the coexistence of SLE and IgAN may in fact be an uncommon finding for unknown reasons or an underdiagnosed condition. This report focus on the importance of the distinction between the activity of renal disease in SLE and non‐SLE nephropathy, especially IgAN, a definition that has important implications on renal prognosis and therapeutic regimens to be adopted in the short and long term.

Congenital platelet function defects

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... pool disorder; Glanzmann's thrombasthenia; Bernard-Soulier syndrome; Platelet function defects - congenital ... Congenital platelet function defects are bleeding disorders that cause reduced platelet function. Most of the time, people with these disorders have ...

Complex etiology and pathology of mycotoxic nephropathy in South African pigs

CSIR Research Space (South Africa)

Stoev, SD

2009-01-01

Full Text Available Spontaneous nephropathy in pigs seen in South Africa was found to have multi-mycotoxic etiology involving several mycotoxins such as ochratoxin A (OTA), penicillic acid (PA) and fumonisin B1 (FB1) in addition to a not yet identified mycotoxin...

Number and Frequency of Albuminuria Measurements in Clinical Trials in Diabetic Nephropathy

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Kröpelin, Tobias F; de Zeeuw, Dick; Andress, Dennis L

2015-01-01

. CONCLUSIONS: Increasing the number of urine collections per study visit and the number of visits over time does not change the average drug effect estimate but markedly increases the precision, thereby enhancing statistical power. Thus, clinical trial designs in diabetic nephropathy using albuminuria...... data from three randomized intervention trials (Aliskiren Combined with Losartan in Type 2 Diabetes and Nephropathy, Selective Vitamin D Receptor Activation for Albuminuria Lowering, and Residual Albuminuria Lowering with Endothelin Antagonist Atrasentan) including patients with type 2 diabetes...... of urine collections per visit were increased. Using all albuminuria measurements at all study visits led to a 4- to 6-fold reduction in sample size to detect a 30% albuminuria-lowering treatment effect with 80% power compared with using baseline and end-of-treatment albuminuria measurements alone...

General Concepts in Adult Congenital Heart Disease.

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Mutluer, Ferit Onur; Çeliker, Alpay

2018-01-20

Congenital heart disease in adults (adult congenital heart disease) is a growing burden for healthcare systems. While infant mortality due to congenital heart disease in the last four decades decreased by almost 3-fold, adult congenital heart disease prevalence increased by more than 2-fold in United States. Adult congenital heart disease prevalence is expected to increase steadily until 2050 in projections. Adult congenital heart disease is a multifaceted problem with many dimensions. This manuscript aims to provide an overview of the common adult congenital heart diseases and summarize important points in management of these diseases with possible problems and complications that the patients and the physicians face.

General Concepts in Adult Congenital Heart Disease

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Ferit Onur Mutluer

2018-02-01

Full Text Available Congenital heart disease in adults (adult congenital heart disease is a growing burden for healthcare systems. While infant mortality due to congenital heart disease in the last four decades decreased by almost 3-fold, adult congenital heart disease prevalence increased by more than 2-fold in United States. Adult congenital heart disease prevalence is expected to increase steadily until 2050 in projections. Adult congenital heart disease is a multifaceted problem with many dimensions. This manuscript aims to provide an overview of the common adult congenital heart diseases and summarize important points in management of these diseases with possible problems and complications that the patients and the physicians face

Congenital cholesteatoma of the infratemporal fossa with congenital aural atresia and mastoiditis: a case report

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Abdel-Aziz Mosaad

2012-06-01

Full Text Available Abstract Background Congenital cholesteatoma may be expected in abnormally developed ear, it may cause bony erosion of the middle ear cleft and extend to the infratemporal fossa. We present the first case of congenital cholesteatoma of the infratemporal fossa in a patient with congenital aural atresia that has been complicated with acute mastoiditis. Case presentation A sixteen year old Egyptian male patient presented with congenital cholesteatoma of the infratemporal fossa with congenital aural atresia complicated with acute mastoiditis. Two weeks earlier, the patient suffered pain necessitating hospital admission, magnetic resonance imaging revealed a soft tissue mass in the right infratemporal fossa. On presentation to our institute, Computerized tomography was done as a routine, it proved the diagnosis of mastoiditis, pure tone audiometry showed an air-bone gap of 60 dB. Cortical mastoidectomy was done for treatment of mastoiditis, removal of congenital cholesteatoma was carried out with reconstruction of external auditory canal. Follow-up of the patient for 2 years and 3 months showed a patent, infection free external auditory canal with an air-bone gap has been reduced to 35db. One year after the operation; MRI was done and it showed no residual or recurrent cholesteatoma. Conclusions Congenital cholesteatoma of the infratemporal fossa in cases of congenital aural atresia can be managed safely even if it was associated with mastoiditis. It is an original case report of interest to the speciality of otolaryngology.

Evaluation of adriamycin nephropathy by an in vivo electron paramagnetic resonance

International Nuclear Information System (INIS)

Oteki, Takaaki; Nagase, Sohji; Yokoyama, Hidekatsu; Ohya, Hiroaki; Akatsuka, Takao; Tada, Mika; Ueda, Atsushi; Hirayama, Aki; koyama, Akio

2005-01-01

A rat model for human minimal change nephropathy was obtained by the intravenous injection of adriamycin (ADR) at 5 mg/kg. By using an in vivo electron paramagnetic resonance (EPR) spectrometer operating at 700 MHz, the temporal changes in signal intensities of a nitroxide radical, 4-hydroxyl-2,2,6,6-tetramethylpiperidine-1-oxyl (TEMPOL), in the kidneys of rats with ADR nephropathy were investigated. The decay rate of the EPR signal intensity obtained in the kidney is indicative of the renal reducing ability. It was found that the reducing ability in the kidney declined on the 7th day after ADR administration and recovered after the 14th day. Impairment of the reducing ability occurred before the appearance of continuous urinary protein. The in vitro EPR study showed that this impairment of in vivo renal reducing ability is related to impairment of the reducing ability in the mitochondria

Lycium chinense leaves extract ameliorates diabetic nephropathy by suppressing hyperglycemia mediated renal oxidative stress and inflammation.

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Olatunji, Opeyemi Joshua; Chen, Hongxia; Zhou, Yifeng

2018-06-01

Diabetic nephropathy is one of the most serious and most frequently encountered diabetic complication, accounting for the highest cause of end-stage renal disease. This present study was aimed at exploring the protective/attenuative effect of Lycium chinense leaf extract (MELC) on streptozotocin induced diabetic nephropathy in experimental Sprague Dawley rats. The oral administration of diabetic rats with MELC markedly ameliorated renal dysfunction as observed in the significant reduction in the serum levels of creatinine, blood urea nitrogen (BUN), albumin and TGF-β1 as compared to the untreated diabetic control rats. In addition, the elevated levels of renal oxidative stress markers and pro-inflammatory parameters (GSH, SOD, CAT, MDA, TNF-α, IL-6 and IL-1β) were significantly reduced in MELC treated diabetic rats. The results obtained in this study suggests that L. chinense leaf might have the potential as possible pharmacological agent against diabetic nephropathy by suppressing renal oxidative stress and inflammation. Copyright © 2018. Published by Elsevier Masson SAS.

Aortic obstruction: anatomy and echocardiography

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Keirns Candace

2006-09-01

Full Text Available Abstract Echocardiography is a valuable non-invasive technique for identifying the site and type of aortic obstruction. Knowledge of the morphological details of each type of obstruction is the basis for correct interpretation of the diagnostic images and clinical decisions. This study was undertaken to correlate the echocardiographic images with anatomic specimens of equivalent valvular and supravalvular aortic obstruction. Specimens were part of the collection of the Department of Embryology. Fifty six patients were studied, and forty specimens with aortic obstruction were analyzed. Echocardiographic characteristics: Thirty one (55.3% patients were women and twenty five (44.7% men. Valvular aortic obstruction was found in Thirty six patients (64.3 % and supravalvular aortic obstruction in twenty (35.7%. Anatomic characteristics: Of the forty specimens examined, twenty one (52.5% had valvular aortic obstruction and nineteen (47.5% supravalvular aortic obstruction. The anatomoechocardiographic correlation clearly showed that the anatomic findings of the specimen hearts and aortas corresponded to echocardiographic images of valvular and supravalvular aortic obstruction and provided solid corroboration of echocardiographic diagnoses.

Left-Sided Congenital Diaphragmatic Hernia with Multiple Congenital Cardiac Anomalies, Hernia Sac, and Microscopic Hepatic Heterotopia: A Case Report

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Maria Arafah

2011-01-01

Full Text Available Congenital diaphragmatic hernia is a common congenital anomaly of uncertain etiology. Its association with multiple congenital anomalies in various organs is well recognized and antenatal radiological evidence of congenital diaphragmatic hernia warrants thorough evaluation to detect other anomalies, some of which can be life threatening. Rarely, heterotopic hepatic tissue is identified in the hernia, a rare pathological finding, exhibiting more than one macroscopic and microscopic characteristics, and always associated with cardiac congenital anomalies. Herein, we report a case of left-sided microscopic heterotopic hepatic tissue in a congenital diaphragmatic hernia in an infant with multiple cardiac congenital anomalies, but with preserved pericardium.

Congenital Heart Information Network

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... heart defects. Important Notice The Congenital Heart Information Network website is temporarily out of service. Please join ... and Uwe Baemayr for The Congenital Heart Information Network Exempt organization under Section 501(c)3. Copyright © ...

Application of low dose radiation and low concentration contrast media in enhanced CT scans in children with congenital heart disease.

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Liu, Zhimin; Song, Lei; Yu, Tong; Gao, Jun; Zhang, Qifeng; Jiang, Ling; Liu, Yong; Peng, Yun

2016-09-01

The aim of this study was to explore the feasibility of using low dose radiation and low concentration contrast media in enhanced CT examinations in children with congenital heart disease. Ninety patients with congenital heart disease were randomly divided into three groups of 30 patients each who underwent contrast-enhanced cardiac scans on a Discovery CT750 HD scanner. Group A received 270 mg I/mL iodixanol, and group B received 320 mg I/mL iodixanol contrast media and was scanned with prospective ECG triggering mode. Group C received 320 mg I/mL iodixanol and was scanned with conventional retrospective ECG gating mode. The same weight-based contrast injection protocol was used for all three groups. Images were reconstructed using a 30% adaptive statistical iterative reconstruction (ASIR) algorithm and a 50% ASIR in groups A and B and a 30% ASIR in group C. The subjective and objective image quality evaluations, diagnostic accuracies, radiation doses and amounts of contrast media in the three groups were measured and compared. All images in the three groups met the diagnostic requirements, with the same diagnostic accuracy and image quality scores greater than 3 in a 4-point scoring system. However, ventricular enhancement and the objective noise, signal-to-noise ratio, contrast-to-noise ratio and subjective image quality scores in group C were better than those in groups A and B (all Pcontrast dose (14% lower than that of groups B and C). Enhanced CT scan images with low dose radiation and low concentration contrast media can meet the diagnostic requirements for examining children with congenital heart disease while reducing the potential risk of radiation damage and contrast-induced nephropathy. © 2016 John Wiley & Sons Ltd.

Acute GI obstruction.

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Hucl, Tomas

2013-10-01

Acute gastrointestinal obstruction occurs when the normal flow of intestinal contents is interrupted. The blockage can occur at any level throughout the gastrointestinal tract. The clinical symptoms depend on the level and extent of obstruction. Various benign and malignant processes can produce acute gastrointestinal obstruction, which often represents a medical emergency because of the potential for bowel ischemia leading to perforation and peritonitis. Early recognition and appropriate treatment are thus essential. The typical clinical symptoms associated with obstruction include nausea, vomiting, dysphagia, abdominal pain and failure to pass bowel movements. Abdominal distention, tympany due to an air-filled stomach and high-pitched bowel sounds suggest the diagnosis. The diagnostic process involves imaging including radiography, ultrasonography, contrast fluoroscopy and computer tomography in less certain cases. In patients with uncomplicated obstruction, management is conservative, including fluid resuscitation, electrolyte replacement, intestinal decompression and bowel rest. In many cases, endoscopy may aid in both the diagnostic process and in therapy. Endoscopy can be used for bowel decompression, dilation of strictures or placement of self-expandable metal stents to restore the luminal flow either as a final treatment or to allow for a delay until elective surgical therapy. When gastrointestinal obstruction results in ischemia, perforation or peritonitis, emergency surgery is required. Copyright © 2013. Published by Elsevier Ltd.

Circadian rhythm of arterial blood pressure and albuminuria in diabetic nephropathy

DEFF Research Database (Denmark)

Hansen, H P; Rossing, P; Tarnow, L

1996-01-01

The aim of our study was to evaluate the diurnal relationship between arterial blood pressure and albuminuria, and some potential mechanisms responsible for impaired nocturnal blood pressure reduction (non-dippers, groups I and II) in diabetic nephropathy (DN). Twenty-four-hour ambulatory blood p...

Plasma lipoproteins and renal function during simvastatin treatment in diabetic nephropathy

DEFF Research Database (Denmark)

Hommel, E; Andersen, P; Gall, M A

1992-01-01

The aim of this study was to assess the effect of simvastatin on plasma lipoproteins and renal function in hypercholesterolaemic Type 1 (insulin-dependent) diabetic patients with diabetic nephropathy. Twenty-six hypercholesterolaemic (total cholesterol greater than or equal to 5.5 mmol/l) Type 1...

MRI in the assessment of congenital vaginal anomalies

International Nuclear Information System (INIS)

Humphries, P.D.; Simpson, J.C.; Creighton, S.M.; Hall-Craggs, M.A.

2008-01-01

Aim: To assess accuracy of magnetic resonance imaging (MRI) for the delineation of morphological abnormalities of the vagina in patients with congenital anomalies of the genito-urinary tract. Materials and methods: Fifty-one patients (median age 19 years; range 12-40 years) were studied. All were consecutively referred for MRI to assess genital tract anatomy, between 1996 and 2004, from a clinic specializing in congenital abnormalities of the urogenital tract. All patients were assessed clinically and underwent MRI. Images were reviewed retrospectively by an experienced radiologist. Where there was discordance between clinical and radiological findings a consensus diagnosis was achieved by the gynaecologists and radiologists reviewing all of the clinical and radiological evidence together, including assessment of vaginal length. Results: The clinical data were incomplete for five women and the images non-diagnostic in two cases; consequently, 44 of 51 women had complete datasets and could be evaluated. Vaginas were abnormal in 30 of the 44 patients. There was discordance between the clinical and imaging findings at the initial review in three of the 44 cases (6.8%). After consensus review, and with the inclusion of measurement of the vaginal length on MRI, the MRI and clinical findings were concordant in all cases. The initial discordance was due to two vaginal dimples not being appreciated on MRI and one case in which presence of vaginal tissue proximal to a mid-segment obstruction was not appreciated clinically. Conclusion: MRI is an accurate method of imaging vaginal anomalies. However, to achieve reliable results the radiologist requires details of previous surgery and the vaginal length must be measured

MRI in the assessment of congenital vaginal anomalies

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Humphries, P.D. [Department of Radiology, University College Hospital, London (United Kingdom); Simpson, J.C.; Creighton, S.M. [Department of Obstetrics and Gynaecology, University College Hospital, London (United Kingdom); Hall-Craggs, M.A. [Department of Radiology, University College Hospital, London (United Kingdom)], E-mail: margaret.hall-craggs@uclh.nhs.uk

2008-04-15

Aim: To assess accuracy of magnetic resonance imaging (MRI) for the delineation of morphological abnormalities of the vagina in patients with congenital anomalies of the genito-urinary tract. Materials and methods: Fifty-one patients (median age 19 years; range 12-40 years) were studied. All were consecutively referred for MRI to assess genital tract anatomy, between 1996 and 2004, from a clinic specializing in congenital abnormalities of the urogenital tract. All patients were assessed clinically and underwent MRI. Images were reviewed retrospectively by an experienced radiologist. Where there was discordance between clinical and radiological findings a consensus diagnosis was achieved by the gynaecologists and radiologists reviewing all of the clinical and radiological evidence together, including assessment of vaginal length. Results: The clinical data were incomplete for five women and the images non-diagnostic in two cases; consequently, 44 of 51 women had complete datasets and could be evaluated. Vaginas were abnormal in 30 of the 44 patients. There was discordance between the clinical and imaging findings at the initial review in three of the 44 cases (6.8%). After consensus review, and with the inclusion of measurement of the vaginal length on MRI, the MRI and clinical findings were concordant in all cases. The initial discordance was due to two vaginal dimples not being appreciated on MRI and one case in which presence of vaginal tissue proximal to a mid-segment obstruction was not appreciated clinically. Conclusion: MRI is an accurate method of imaging vaginal anomalies. However, to achieve reliable results the radiologist requires details of previous surgery and the vaginal length must be measured.

Early detection of congenital syphilis

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Nagalakshmi Chowdhary

2014-01-01

Full Text Available Late congenital syphilis is a very rare clinical entity, and its early diagnosis and treatment is essential. Dental findings often provide valuable evidence for the diagnosis of late congenital syphilis. It occurs due to the transmission of the disease from an infected mother to her fetus through placenta. This long forgotten disease continues to effect pregnant women resulting in perinatal morbidity and mortality. Congenital syphilis is a preventable disease, and its presence reflects a failure of prenatal care delivery system, as well as syphilis control programs. We are reporting a case of late congenital syphilis with only Hutchinson′s teeth.

Quantitative GC-MS assay of citric acid from humans and db/db mice blood serum to assist the diagnosis of diabetic nephropathy.

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Gao, Haoxue; Yu, Xiaoyi; Sun, Runbin; Yang, Na; He, Jun; Tao, Mingxue; Gu, Huilin; Yan, Caixia; Aa, Jiye; Wang, Guangji

2018-03-01

The early diagnosis of diabetic nephropathy (DN) is rather challenging. Our previous study suggested that citric acid is a potential marker for the early diagnosis of diabetic nephropathy in db/db mice. For the first time, in this study, a surrogate analyte of 13 C 6 -citric acid was employed to generate calibration curves for the quantitative measurement of the endogenous citric acid in the sera of db/db mice and diabetic nephropathy patients by GC/MS after the analytes were extracted, methoximated and trimethylsilylated. The constant response factor of 13 C 6 -citric acid versus citric acid over the linear range indicated the identical ionization efficiency of these two compounds. The full validation assessments suggested that the method is sensitive, specific, reliable, reproducible and has acceptable parameters. Statistical analysis revealed cut-off citric acid concentrations of 29.24 μg/mL with a 95% confidence interval between 32.75 and 39.16 μg/mL in the diabetic nephropathy patients and 16.74 and 22.57 μg/mL in the normal controls. The areas under the receiver operating characteristic curves indicated accuracies of over 90% for the diagnoses of early diabetic nephropathy in both humans and db/db mice, which suggests that the serum citric acid level is potentially a biomarker that could assist in the diagnosis of diabetic nephropathy. Copyright © 2018 Elsevier B.V. All rights reserved.

The diagnosis value of endothelin, calcitonin gene-related peptide and the ratio in diabetic nephropathy

International Nuclear Information System (INIS)

Li Lusheng; Zhao Xin; Chi Liuying; Yang Xixiu; Mao Hongyu

2007-01-01

Objective: To evaluate the diagnosis value of Endothelin (ET), Calcitonin gene-related peptide(CGRP) and theratio in diabetic nephropathy(DN). Methods: To choose 54 healthy as the control group and 124 patients with diabetes or DN as test group. According to urinary albumin excretion rate (uAER), the test group was divided into three groups, which were diabetes group of normalalbuminmia (group A), early DN (group B) and clinical DN and renal failure group( group C ). Plasma concentration of ET and CGRP were measured with radioimmunossay for those in the Control group and patients with diabetes or DN. Results: The level of ET was significantly higher in diabetic nephropathy than that in the control group (P<0.01), and there was positive correlation between ET and uAER(r=0.591, P<0.01). The plasma level of CGRP was significantly lower in diabetic nephropathy than that in the control group (P<0.01), and there was negative correlation between CGRP and uAER(r-0.389, P<0.05). The level of ET, CGRP and ET/CGRP ratio have evidently changed with the uAER rised. Conclusion: (1)The level of ET, CGRP and type 2 diabetic nephropathy are closely related, and which probably plays a role in the development of DN. (2)ET/CGRP ratio, as a new way for diagnosis, can even more reflecte the seriousness of DN. (3)This experiment provide us a new way for the prevention and treatment of DN with extrinsic CGRP. (authors)

Genetics Home Reference: critical congenital heart disease

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... Facebook Twitter Home Health Conditions Critical congenital heart disease Critical congenital heart disease Printable PDF Open All Close All ... for Disease Control and Prevention: Congenital Heart Defects Disease InfoSearch: Congenital Heart Defects KidsHealth from Nemours Lucile Packard Children's ...

AN ANALYTICAL STUDY IN ADHESIVE BOWEL OBSTRUCTION

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Gerald Anand Raja

2017-04-01

Full Text Available BACKGROUND Peritoneal adhesions can be defined as abnormal fibrous bands between organs or tissues or both in the abdominal cavity that are normally separated. Adhesions may be acquired or congenital; however, most are acquired as a result of peritoneal injury, the most common cause of which is abdominopelvic surgery. Less commonly, adhesions may form as the result of inflammatory conditions, intraperitoneal infection or abdominal trauma. The extent of adhesion formation varies from one patient to another and is most dependent on the type and magnitude of surgery performed as well as whether any postoperative complications develop. Fortunately, most patients with adhesions do not experience any overt clinical symptoms. For others, adhesions may lead to any one of a host of problems and can be the cause of significant morbidity and mortality. MATERIALS AND METHODS This is a retrospective study of 50 patients admitted in Government Royapettah Hospital with adhesive bowel obstruction between September 2008 to September 2010. All patients were admitted and managed either conservatively or surgically. RESULTS 1. Adhesive bowel disease is the most common cause for bowel obstruction followed by hernias. 2. Increased incidence is noted in females. 3. Increased incidence of adhesions was documented in gynaecological and colorectal surgeries. 4. Below umbilical incisions have higher propensity for adhesion formation. 5. Laparotomies done for infective aetiology have higher adhesion risks. 6. Most of adhesive obstructions can be managed conservatively. 7. Adhesiolysis preferably laparoscopic can be done. For gangrenous bowel resection and anastomosis or ostomy done. 8. Given the above risk factors, adhesive bowel disease can be prevented to a certain extent. CONCLUSION The formation of peritoneal adhesions continues to plague patients, surgeons and society. Although, research in this area is ongoing, there is currently no method that is 100% effective in

Congenital Anorectal Malformation Severity Does Not Predict Severity of Congenital Heart Defects

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Jonker, Jara E.; Liem, Eryn T.; Elzenga, Nynke J.; Molenbuur, Bouwe; Trzpis, Monika; Broens, Paul M. A.

2016-01-01

Objective To determine the prevalence of congenital heart defects (CHDs) in patients with mild or severe congenital anorectal malformations (CARMs), and whether all patients with CARM need pediatric cardiology screening. Study design We included 129 patients with CARM born between 2004 and 2013, and

Randomized control trial for the assessment of the anti-albuminuric effects of topiroxostat in hyperuricemic patients with diabetic nephropathy (the ETUDE study).

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Kato, Sawako; Ando, Masahiko; Mizukoshi, Toshihiro; Nagata, Takanobu; Katsuno, Takayuki; Kosugi, Tomoki; Tsuboi, Naotake; Maruyama, Shoichi

2016-05-01

Proteinuria is an established risk factor for diabetic nephropathy. Recent studies indicate that some xanthine oxidase inhibitors have a renoprotective effect. The aim of this study was to assess whether topiroxostat reduces albuminuria in hyperuricemic patients with diabetic nephropathy and overt proteinuria. The ETUDE study is an ongoing 24-week, multicenter, open-label, randomized (1:1), parallel group study involving hyperuricemic patients with diabetic nephropathy (estimated glomerular filtration rate [eGFR] ≥ 20 mL/min/1.73 m(2)) and overt proteinuria (0.3 ≤ urine protein to creatinine ratio (UPCR) ETUDE trial is the first randomized controlled study of topiroxostat in hyperuricemic patients with diabetic nephropathy and overt proteinuria. We will clarify the pleiotropic function of topiroxostat including an anti-albumiuric effect as well as its effects on safely decreasing serum uric acid levels.

G/T substitution in intron 1 of the UNC13B gene is associated with increased risk of nephropathy in patients with type 1 diabetes

DEFF Research Database (Denmark)

Tregouet, D.A.; Groop, P.H.; McGinn, S.

2008-01-01

for association with diabetic nephropathy (persistent albuminuria >/=300 mg/24 h) in a large type 1 diabetes case/control (1,176/1,323) study from three European populations. RESULTS: Only one SNP, rs2281999, located in the UNC13B gene, was significantly associated with nephropathy after correction for multiple...... was 1.68 (95% CI 1.29-2.19) (P = 1.0 x 10(-4)). This association was replicated in an independent population of 412 case subjects and 614 control subjects (combined OR of 1.63 [95% CI 1.30-2.05], P = 2.3 x 10(-5)). CONCLUSIONS: We identified a polymorphism in the UNC13B gene associated with nephropathy......OBJECTIVE: Genetic and environmental factors modulate the susceptibility to diabetic nephropathy, as initiating and/or progression factors. The objective of the European Rational Approach for the Genetics of Diabetic Complications (EURAGEDIC) study is to identify nephropathy susceptibility genes...

SGLT2 Protein Expression Is Increased in Human Diabetic Nephropathy

Science.gov (United States)

Wang, Xiaoxin X.; Levi, Jonathan; Luo, Yuhuan; Myakala, Komuraiah; Herman-Edelstein, Michal; Qiu, Liru; Wang, Dong; Peng, Yingqiong; Grenz, Almut; Lucia, Scott; Dobrinskikh, Evgenia; D'Agati, Vivette D.; Koepsell, Hermann; Kopp, Jeffrey B.; Rosenberg, Avi Z.; Levi, Moshe

2017-01-01

There is very limited human renal sodium gradient-dependent glucose transporter protein (SGLT2) mRNA and protein expression data reported in the literature. The first aim of this study was to determine SGLT2 mRNA and protein levels in human and animal models of diabetic nephropathy. We have found that the expression of SGLT2 mRNA and protein is increased in renal biopsies from human subjects with diabetic nephropathy. This is in contrast to db-db mice that had no changes in renal SGLT2 protein expression. Furthermore, the effect of SGLT2 inhibition on renal lipid content and inflammation is not known. The second aim of this study was to determine the potential mechanisms of beneficial effects of SGLT2 inhibition in the progression of diabetic renal disease. We treated db/db mice with a selective SGLT2 inhibitor JNJ 39933673. We found that SGLT2 inhibition caused marked decreases in systolic blood pressure, kidney weight/body weight ratio, urinary albumin, and urinary thiobarbituric acid-reacting substances. SGLT2 inhibition prevented renal lipid accumulation via inhibition of carbohydrate-responsive element-binding protein-β, pyruvate kinase L, SCD-1, and DGAT1, key transcriptional factors and enzymes that mediate fatty acid and triglyceride synthesis. SGLT2 inhibition also prevented inflammation via inhibition of CD68 macrophage accumulation and expression of p65, TLR4, MCP-1, and osteopontin. These effects were associated with reduced mesangial expansion, accumulation of the extracellular matrix proteins fibronectin and type IV collagen, and loss of podocyte markers WT1 and synaptopodin, as determined by immunofluorescence microscopy. In summary, our study showed that SGLT2 inhibition modulates renal lipid metabolism and inflammation and prevents the development of nephropathy in db/db mice. PMID:28196866

The effects of atrasentan on urinary metabolites in patients with type 2 diabetes and nephropathy

DEFF Research Database (Denmark)

Pena, Michelle J; de Zeeuw, Dick; Andress, Dennis

2018-01-01

We assessed the effect of atrasentan therapy on a pre-specified panel of 13 urinary metabolites known to reflect mitochondrial function in patients with diabetic kidney disease. This post-hoc analysis was performed using urine samples collected during the RADAR study which was a randomized, double......-blind, placebo-controlled trial that tested the effects of atrasentan on albuminuria reduction in patients with type 2 diabetes and nephropathy. At baseline, four of the 13 metabolites, quantified by gas-chromatography mass spectrometry, were below detectable levels, and six were reduced in patients with e...... diabetes, nephropathy, and eGFR

The Effect of Hyperuricemia on the Rate of Contrast-Induced Nephropathy in Patients with Coronary Angiography

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Hossein Vakili

2016-10-01

Full Text Available Introduction: There is little information about the relationship between hyperuricemia and contrast induced nephropathy. The present study aimed to evaluate the relationship between hyperuricemia and contrast induced nephropathy among patients, who had undergone coronary angiography.Methods: In the current study, 200 consecutive patients with coronary artery disease, who underwent coronary angiography in Modarres hospital, were enrolled. According to the available data, the upper limit normal level of uric acid was defined as 7 mg/dl in males and 6.5 mg/dl in females. By increasing level of serum creatinine to 0.5 mg/dl (or 25% enhancement from basic level of creatinine during 48 hours of introduction of contrast agent, diagnosis of Contrast Induced Nephropathy (CIN was established. The relationship between hyperuricemia and CIN was then assessed.Results: There is a significant difference between normouricemic patients and hyperuricemic patients, in aspect of weight (P = 0.011 and uric acid (P = 0.001; however, other quantitative and qualitative variables including age, volume of contract agent, creatinine level after angiography, hemoglobin level, gender, arterial access type, number of involved vessels, were insignificant between the two groups (P > 0.05. Moreover, as an essential finding, CIN was shown in 9% of normouricemic patients and 10% of hyperuricemic  patients with no significant difference between the two groups (P = 0.6.Conclusions: Our study suggests that hyperuricemia may not significantly increase the rate of the contrast-induced nephropathy in patients, who had undergone angiography

Early diagnostic predictors: useful in treatment and progression of diabetes associated nephropathy

International Nuclear Information System (INIS)

Nawab, S.N.; Shahid, S.M.; Azhar, A.; Ahmed, N.

2013-01-01

Diabetic nephropathy (DN) is one of the major complications of type 2 diabetes mellitus (T2DM) characterized by frequent microalbuminuria, elevated arterial blood pressure, persistent decline in glomerular filtration rate and high risk of morbidity and mortality. It encompasses long-term duration of diabetes, which has an effect on the minute blood vessels of kidney. The biochemical parameters play a key role in the prediction of nephropathy in T2DM patients. Therefore, the present study was conducted to investigate the role of biochemical markers in the prediction of DN in T2DM patients. The aim of this study was addressed in case-control setting, 230 T2DM, 200 DN patients and 110 non diabetic healthy individuals were included in order to assess the biochemical parameters and risk of DN. Patients were recruited according to WHO's criteria from various hospitals of Karachi, Pakistan. After getting informed consent from patients and control subjects, clinical data was recorded. Five hundred and forty (n=540) samples were studied for their serum blood glucose, blood pressure, glycosylated hemoglobin (HbAlc), serum creatinine, serum urea, lipid profile and urinary albumin levels. The analysis showed that incidence and the progression of the DN increased with hyperglycemia, longer duration of diabetes, dyslipidemia, elevated level of serum urea, creatinine and urinary albumin levels in patients with T2DM. Therefore, these biochemical predictors can anticipate the occurrence of nephropathy in later stages of diabetes. (author)

The role of oxidative stress in streptozotocin-induced diabetic nephropathy in rats.

Science.gov (United States)

Fernandes, Sheila Marques; Cordeiro, Priscilla Mendes; Watanabe, Mirian; Fonseca, Cassiane Dezoti da; Vattimo, Maria de Fatima Fernandes

2016-10-01

The objective of this study was to evaluate the role of oxidative stress in an experimental model of streptozotocin-induced diabetic nephropathy in rats. Wistar, adult, male rats were used in the study. Animals were divided in the following groups: Citrate (control, citrate buffer 0.01M, pH 4.2 was administrated intravenously - i.v - in the caudal vein), Uninephrectomy+Citrate (left uninephrectomy-20 days before the study), DM (streptozotocin, 65 mg/kg, i.v, on the 20th day of the study), Uninephrectomy+DM. Physiological parameters (water and food intake, body weight, blood glucose, kidney weight, and relative kidney weight); renal function (creatinine clearance), urine albumin (immunodiffusion method); oxidative metabolites (urinary peroxides, thiobarbituric acid reactive substances, and thiols in renal tissue), and kidney histology were evaluated. Polyphagia, polydipsia, hyperglycemia, and reduced body weight were observed in diabetic rats. Renal function was reduced in diabetic groups (creatinine clearance, p < 0.05). Uninephrectomy potentiated urine albumin and increased kidney weight and relative kidney weight in diabetic animals (p < 0.05). Urinary peroxides and thiobarbituric acid reactive substances were increased, and the reduction in thiol levels demonstrated endogenous substrate consumption in diabetic groups (p < 0.05). The histological analysis revealed moderate lesions of diabetic nephropathy. This study confirms lipid peroxidation and intense consumption of the antioxidant defense system in diabetic rats. The association of hyperglycemia and uninephrectomy resulted in additional renal injury, demonstrating that the model is adequate for the study of diabetic nephropathy.

Early diagnostic predictors: useful in treatment and progression of diabetes associated nephropathy

Energy Technology Data Exchange (ETDEWEB)

Nawab, S. N.; Shahid, S. M.; Azhar, A. [University of Karachi, Karachi (Pakistan). Dept. of Biotechnology and Genetic Engineering; Ahmed, N. [University of Karachi, Karachi (Pakistan). Dept. of Biochemistry

2013-06-15

Diabetic nephropathy (DN) is one of the major complications of type 2 diabetes mellitus (T2DM) characterized by frequent microalbuminuria, elevated arterial blood pressure, persistent decline in glomerular filtration rate and high risk of morbidity and mortality. It encompasses long-term duration of diabetes, which has an effect on the minute blood vessels of kidney. The biochemical parameters play a key role in the prediction of nephropathy in T2DM patients. Therefore, the present study was conducted to investigate the role of biochemical markers in the prediction of DN in T2DM patients. The aim of this study was addressed in case-control setting, 230 T2DM, 200 DN patients and 110 non diabetic healthy individuals were included in order to assess the biochemical parameters and risk of DN. Patients were recruited according to WHO's criteria from various hospitals of Karachi, Pakistan. After getting informed consent from patients and control subjects, clinical data was recorded. Five hundred and forty (n=540) samples were studied for their serum blood glucose, blood pressure, glycosylated hemoglobin (HbAlc), serum creatinine, serum urea, lipid profile and urinary albumin levels. The analysis showed that incidence and the progression of the DN increased with hyperglycemia, longer duration of diabetes, dyslipidemia, elevated level of serum urea, creatinine and urinary albumin levels in patients with T2DM. Therefore, these biochemical predictors can anticipate the occurrence of nephropathy in later stages of diabetes. (author)

Alterations of urinary metabolite profile in model diabetic nephropathy

Energy Technology Data Exchange (ETDEWEB)

Stec, Donald F. [Vanderbilt Institute of Chemical Biology, Vanderbilt University Medical Center, Nashville, TN 37232 (United States); Wang, Suwan; Stothers, Cody [Department of Medicine, Vanderbilt University Medical Center, Nashville, TN 37232 (United States); Avance, Josh [Berea College, 1916 CPO, Berea, KY 40404 (United States); Denson, Deon [Choctaw Central High School, Philadelphia, MS 39350 (United States); Harris, Raymond [Department of Medicine, Vanderbilt University Medical Center, Nashville, TN 37232 (United States); Voziyan, Paul, E-mail: paul.voziyan@vanderbilt.edu [Department of Medicine, Vanderbilt University Medical Center, Nashville, TN 37232 (United States)

2015-01-09

Highlights: • {sup 1}H NMR spectroscopy was employed to study urinary metabolite profile in diabetic mouse models. • Mouse urinary metabolome showed major changes that are also found in human diabetic nephropathy. • These models can be new tools to study urinary biomarkers that are relevant to human disease. - Abstract: Countering the diabetes pandemic and consequent complications, such as nephropathy, will require better understanding of disease mechanisms and development of new diagnostic methods. Animal models can be versatile tools in studies of diabetic renal disease when model pathology is relevant to human diabetic nephropathy (DN). Diabetic models using endothelial nitric oxide synthase (eNOS) knock-out mice develop major renal lesions characteristic of human disease. However, it is unknown whether they can also reproduce changes in urinary metabolites found in human DN. We employed Type 1 and Type 2 diabetic mouse models of DN, i.e. STZ-eNOS{sup −/−} C57BLKS and eNOS{sup −/−} C57BLKS db/db, with the goal of determining changes in urinary metabolite profile using proton nuclear magnetic resonance (NMR). Six urinary metabolites with significantly lower levels in diabetic compared to control mice have been identified. Specifically, major changes were found in metabolites from tricarboxylic acid (TCA) cycle and aromatic amino acid catabolism including 3-indoxyl sulfate, cis-aconitate, 2-oxoisocaproate, N-phenyl-acetylglycine, 4-hydroxyphenyl acetate, and hippurate. Levels of 4-hydroxyphenyl acetic acid and hippuric acid showed the strongest reverse correlation to albumin-to-creatinine ratio (ACR), which is an indicator of renal damage. Importantly, similar changes in urinary hydroxyphenyl acetate and hippurate were previously reported in human renal disease. We demonstrated that STZ-eNOS{sup −/−} C57BLKS and eNOS{sup −/−} C57BLKS db/db mouse models can recapitulate changes in urinary metabolome found in human DN and therefore can be

Alterations of urinary metabolite profile in model diabetic nephropathy

International Nuclear Information System (INIS)

Stec, Donald F.; Wang, Suwan; Stothers, Cody; Avance, Josh; Denson, Deon; Harris, Raymond; Voziyan, Paul

2015-01-01

Highlights: • 1 H NMR spectroscopy was employed to study urinary metabolite profile in diabetic mouse models. • Mouse urinary metabolome showed major changes that are also found in human diabetic nephropathy. • These models can be new tools to study urinary biomarkers that are relevant to human disease. - Abstract: Countering the diabetes pandemic and consequent complications, such as nephropathy, will require better understanding of disease mechanisms and development of new diagnostic methods. Animal models can be versatile tools in studies of diabetic renal disease when model pathology is relevant to human diabetic nephropathy (DN). Diabetic models using endothelial nitric oxide synthase (eNOS) knock-out mice develop major renal lesions characteristic of human disease. However, it is unknown whether they can also reproduce changes in urinary metabolites found in human DN. We employed Type 1 and Type 2 diabetic mouse models of DN, i.e. STZ-eNOS −/− C57BLKS and eNOS −/− C57BLKS db/db, with the goal of determining changes in urinary metabolite profile using proton nuclear magnetic resonance (NMR). Six urinary metabolites with significantly lower levels in diabetic compared to control mice have been identified. Specifically, major changes were found in metabolites from tricarboxylic acid (TCA) cycle and aromatic amino acid catabolism including 3-indoxyl sulfate, cis-aconitate, 2-oxoisocaproate, N-phenyl-acetylglycine, 4-hydroxyphenyl acetate, and hippurate. Levels of 4-hydroxyphenyl acetic acid and hippuric acid showed the strongest reverse correlation to albumin-to-creatinine ratio (ACR), which is an indicator of renal damage. Importantly, similar changes in urinary hydroxyphenyl acetate and hippurate were previously reported in human renal disease. We demonstrated that STZ-eNOS −/− C57BLKS and eNOS −/− C57BLKS db/db mouse models can recapitulate changes in urinary metabolome found in human DN and therefore can be useful new tools in

Relationship between changes of plasma endothelin (ET) level, ATPase activity of erythrocyte membrane and development of nephropathy in patients with pregnancy induced hypertension

International Nuclear Information System (INIS)

Qin Lin; Lu Beiyi

2008-01-01

Objective: To investigate the possible role played by alteration of plasma ET levels and activities of Na + - K + -APT ase and Ca 2+ -Mg 2+ -ATPase of erythrocyte membrane in patients with nephropathy pregnancy induced hypertension. Methods: The concentrations of plasma ET was detected with RIA and erythrocyte membrane ATPase activities were determined with Reilni method in 32 pregnant women with PIH complicated with nephropathy and 70 women with PIH but no nephropathy and 35 normal pregnant women as controls. Results: The plasma ET levels in patients with PHI (both with and without nephropathy) were significantly higher than those in normal preganat women (P + -K + -ATPase and Ca 2+ -Mg 2+ -ATPase levels were significantly de- creased (P + -K + -ATPase and Ca 2+ -Mg 2+ -ATPase activity of erythrocyte membrane. (authors)

Association Between CNDP1 Genotype and Diabetic Nephropathy Is Sex Specific

NARCIS (Netherlands)

Mooyaart, Antien L.; Zutinic, Ana; Bakker, Stephan J. L.; Grootendorst, Diana C.; Kleefstra, Nanne; van Valkengoed, Irene G. M.; Bohringer, Stefan; Bilo, Henk J. G.; Dekker, Friedo W.; Bruijn, Jan Anthonie; Navis, Gerjan; Janssen, Bart; Baelde, Hans J.; De Heer, Emile

OBJECTIVE-The 5-5 homozygous CNDP1 (carnosinase) genotype is associated with a reduced risk of diabetic nephropathy. We investigated whether this association is sex specific and independent of susceptibility for type 2 diabetes. RESEARCH DESIGN AND METHODS-Three separate groups of 114, 90, and 66

The role of theophylline in prevention of radiocontrast media-induced nephropathy

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Malhis Mahmoud

2010-01-01

Full Text Available Contrast media induced nephropathy (CIN results in significant morbidity and mortality. We therefore investigated whether theophylline (adenosine antagonist reduces the inci-dence of contrast media induced nephropathy. Two hundred and eighty patients were randomly assigned to prophylactic administration of hydration with sodium bicarbonate plus theophylline (either orally or intravenously (n=128 or hydration with sodium bicarbonate only (n=152. Blood Urea, creatinine, and glomerular filtration rate (MDRD were measured before and after administration of contrast media. Both groups were similar in clinical characteristics and amount of contrast used. Theophylline prophylaxis significantly reduced the incidence of CIN (1.6% vs 7.9%; P= 0.015. Compared to low-risk patients, Theophylline prophylaxis significantly reduced the incidence of CIN in moderate and high-risk patients (0% vs 8.8%; P= 0.022 and 9.1% vs 42.1%; P= 0.014 respectively. In conclusion, prophylactic administration of theophylline re-duces the incidence of CIN in moderate and high-risk patients for CIN.

Hypertension in diabetes as related to nephropathy. Early blood pressure changes

DEFF Research Database (Denmark)

Feldt-Rasmussen, B; Borch-Johnsen, K; Mathiesen, E R

1985-01-01

We measured the blood pressure under standardized conditions in three groups of patients with type I (insulin-dependent) diabetes: group 1, patients with Albustix-negative urine and normal urinary albumin excretion rate below 20 micrograms/min; group 2, patients with Albustix-negative urine and e...... that arterial hypertension is an early feature in the developing of diabetic nephropathy, with blood pressure rising before the presence of clinical proteinuria.......We measured the blood pressure under standardized conditions in three groups of patients with type I (insulin-dependent) diabetes: group 1, patients with Albustix-negative urine and normal urinary albumin excretion rate below 20 micrograms/min; group 2, patients with Albustix-negative urine...... and elevated urinary albumin excretion rate 20 to 200 micrograms/min; and group 3, patients with Albustix-positive urine at the time of diagnosis of diabetic nephropathy, that is, proteinuria greater than 0.5 g/24 hr on four consecutive visits with an interval of more than 1 month. We also studied blood...

Complement factor H-related proteins in IgA nephropathy-sometimes a gentle nudge does the trick.

Science.gov (United States)

Thurman, Joshua M; Laskowski, Jennifer

2017-10-01

Complement activation probably contributes to glomerular inflammation and damage in IgA nephropathy. In this issue, 2 groups report that levels of factor H-related protein 1 are elevated in patients with IgA nephropathy and correlate with disease progression. These studies provide new evidence that the complement cascade is important to the pathogenesis of this disease. These results also suggest that factor H-related protein 1 levels may be useful for identifying those patients at high risk of disease progression. Copyright © 2017 International Society of Nephrology. Published by Elsevier Inc. All rights reserved.

Fetal chromosome abnormalities and congenital malformations: an ...

African Journals Online (AJOL)

The results also showed that Multiple congenital anomalies (MCA) represented among 42.2%, congenital malformation of CNS represents 26.6%, congenital malformation of the skeletal system 20%, congenital polycystic kidney 8.8% and pyloric stenosis in 2.2%. Among the 21 women with abnormal karyotype of amniotic ...

Familial Screening for Left-Sided Congenital Heart Disease: What Is the Evidence? What Is the Cost?

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Daniel J. Perry

2017-12-01

Full Text Available Since the American Heart Association’s recommendation for familial screening of adults with congenital heart disease for bicuspid aortic valve, similar recommendations for other left-sided heart defects, such as hypoplastic left heart syndrome (HLHS, have been proposed. However, defining at-risk populations for these heart defects based on genetics is less straightforward due to the wide variability of inheritance patterns and non-genetic influences such as environmental and lifestyle factors. We discuss whether there is sufficient evidence to standardize echocardiographic screening for first-degree relatives of children diagnosed with HLHS. Due to variations in the inclusion of cardiac anomalies linked to HLHS and the identification of asymptomatic individuals with cardiac malformations, published studies are open to interpretation. We conclude that familial aggregation of obstructive left-sided congenital heart lesions in families with history of HLHS is not supported and recommend that additional screening should adopt a more conservative definition of what truly constitutes this heart defect. More thorough consideration is needed before embracing familial screening recommendations of families of patients with HLHS, since this could inflict serious costs on healthcare infrastructure and further burden affected families both emotionally and financially.

Hennekam syndrome presenting as nonimmune hydrops fetalis, congenital chylothorax, and congenital pulmonary lymphangiectasia

NARCIS (Netherlands)

Bellini, Carlo; Mazzella, Massimo; Arioni, Cesare; Campisi, Corradino; Taddei, Gioconda; Tomà, Paolo; Boccardo, Francesco; Hennekam, Raoul C.; Serra, Giovanni

2003-01-01

We report a female infant with congenital lymphedema, facial anomalies, intestinal lymphangiectasia consistent with a diagnosis of Hennekam syndrome. At birth the patient presented with severe respiratory distress due to nonimmune hydrops fetalis, a congenital chylothorax (CC), and pulmonary

The role of Toll-like receptor 2 in inflammation and fibrosis during progressive renal injury.

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Jaklien C Leemans

Full Text Available Tissue fibrosis and chronic inflammation are common causes of progressive organ damage, including progressive renal disease, leading to loss of physiological functions. Recently, it was shown that Toll-like receptor 2 (TLR2 is expressed in the kidney and activated by endogenous danger signals. The expression and function of TLR2 during renal fibrosis and chronic inflammation has however not yet been elucidated. Therefore, we studied TLR2 expression in human and murine progressive renal diseases and explored its role by inducing obstructive nephropathy in TLR2(-/- or TLR2(+/+ mice. We found that TLR2 is markedly upregulated on tubular and tubulointerstitial cells in patients with chronic renal injury. In mice with obstructive nephropathy, renal injury was associated with a marked upregulation and change in distribution of TLR2 and upregulation of murine TLR2 danger ligands Gp96, biglycan, and HMGB1. Notably, TLR2 enhanced inflammation as reflected by a significantly reduced influx of neutrophils and production of chemokines and TGF-beta in kidneys of TLR2(-/- mice compared with TLR2(+/+ animals. Although, the obstructed kidneys of TLR2(-/- mice had less interstitial myofibroblasts in the later phase of obstructive nephropathy, tubular injury and renal matrix accumulation was similar in both mouse strains. Together, these data demonstrate that TLR2 can initiate renal inflammation during progressive renal injury and that the absence of TLR2 does not affect the development of chronic renal injury and fibrosis.

Prolonged Ileus in an Infant Presenting with Primary Congenital Hypothyroidism

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Caroline Chua

2015-01-01

Full Text Available Congenital hypothyroidism (CH is the most common endocrine disorder affecting the newborn. Universal newborn screening (NBS has virtually eliminated the static encephalopathy and devastating neurodevelopmental syndrome known as cretinism. This report describes the presentation of an infant referred by the primary pediatrician to our hospital at 12 days of age for confirmatory testing after the NBS was consistent with CH. The infant had hypoglycemia secondary to lethargy and poor feeding and required transfer to the neonatal intensive care unit for worsening abdominal distension despite normalization of serum thyroid function tests following hormone replacement. In particular, the recalcitrant ileus and secondary bowel obstruction resulted in an additional diagnostic workup and lengthened hospital day. Our report highlights the acute gastrointestinal consequences of hypothyroidism despite evidence of effective treatment. We believe that the preclinical detection and immediate therapy for CH have lessened the prevalence of this presentation in general practice, and hence practitioners are less likely to be familiar with its natural history and management.

Contrast induced nephropathy after transcatheter aortic valve implantation

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D. L. Kranin

2017-01-01

Full Text Available Background: Aortic stenosis ranks the third in the structure of all cardiovascular diseases, conceding only to arterial hypertension and coronary heart disease. Transcatheter aortic valve implantation (TAVI is a promising area of interventional endovascular surgery that enables to provide surgical care to a significant group of the patients with severe aortal stenosis.Aim: To assess the efficacy of prevention of the contrast induced nephropathy (CIN in patients who underwent TAVI under general anesthesia.Materials and methods: We evaluated incidence of CIN in 19 patients who underwent surgery for aortic valve stenosis under general anesthesia with hemodilution and intravenous magnesium sulfate 1 g before administration of the contrast.Results: Laboratory signs of nephropathy within the first 72 hours after the intervention were found in 8/19 (42.1% of patients. In 4 (50% of patients with CIN, its risk had been very high, in 3 (38%, high, and in 1 (12%, moderate. The results obtained are compatible with the contrast-induced acute kidney injury risk estimated from the Mehran-Barrett-Parfrey scale.Conclusion: The used technique of hemodilution and magnesium-based prevention can be considered a safe method of CIN prophylaxis in TAVI patients.

Amadori albumin in diabetic nephropathy

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Km. Neelofar

2015-01-01

Full Text Available Nonenzymatic glycation of macromolecules in diabetes mellitus (DM is accelerated due to persistent hyperglycemia. Reducing sugar such as glucose reacts non enzymatically with free €- amino groups of proteins through series of reactions forming Schiff bases. These bases are converted into Amadori product and further into AGEs. Non enzymatic glycation has the potential to alter the biological, structural and functional properties of macromolecules both in vitro and in vivo. Studies have suggested that amadori as well as AGEs are involved in the micro-macro vascular complications in DM, but most studies have focused on the role of AGEs in vascular complications of diabetes. Recently putative AGE-induced patho-physiology has shifted attention from the possible role of amadori-modified proteins, the predominant form of the glycated proteins in the development of the diabetic complications. Human serum albumin (HSA, the most abundant protein in circulation contains 59 lysine and 23 arginine residues that could, in theory be involved in glycation. Albumin has dual nature, first as a marker of intermediate glycation and second as a causative agent of the damage of tissues. Among the blood proteins, hemoglobin and albumin are the most common proteins that are glycated. HSA with a shorter half life than RBC, appears to be an alternative marker of glycemic control as it can indicate blood glucose status over a short period (2-3 weeks and being unaffected by RBCs life span and variant haemoglobin, anemia etc which however, affect HbA1c. On the other hand, Amadori albumin may accumulate in the body tissues of the diabetic patients and participate in secondary complications. Amadori-albumin has potential role in diabetic glomerulosclerosis due to long term hyperglycaemia and plays an important role in the pathogenesis of diabetic nephropathy. This review is an approach to compile both the nature of glycated albumin as a damaging agent of tissues and as an

Membrane targeting and secretion of mutant uromodulin in familial juvenile hyperuricemic nephropathy

NARCIS (Netherlands)

Jennings, Paul; Aydin, Sonia; Kotanko, Peter; Lechner, Judith; Lhotta, Karl; Williams, Sian; Thakker, Rajesh V; Pfaller, Walter

Familial juvenile hyperuricemic nephropathy (FJHN) is an autosomal dominant genetic disorder that is characterized by hyperuricemia, gout, and tubulointerstitial nephritis. FJHN is caused by mutations in the UMOD gene, which encodes for uromodulin, the most abundant urinary protein. Herein is

Epidemiology of contrast material-induced nephropathy in the era of hydration.

NARCIS (Netherlands)

Balemans, C.E.A.; Reichert, L.J.M.; Schelven, B.I. van; Brand, A. van den; Wetzels, J.F.M.

2012-01-01

PURPOSE: To evaluate the incidence of contrast material-induced nephropathy (CIN) in patients with an estimated glomerular filtration rate (eGFR) of less than 60 mL/min/1.73 m(2) who received intravenous contrast media and underwent treatment in accordance with current guidelines and to determine

Low birth weight. A risk factor for development of diabetic nephropathy?

DEFF Research Database (Denmark)

Rossing, P; Tarnow, L; Nielsen, F S

1995-01-01

for expression of renal disease after exposure to potentially injurious renal stimuli. The aim of this study was to determine if low birth weight is a risk factor for development of diabetic nephropathy. In a case-control study, we investigated 184 (110 men) insulin-dependent diabetes mellitus (IDDM) patients...

Congenital chylous ascites

International Nuclear Information System (INIS)

Romanska-Kita, J.; Borszewska-Kornacka, M. K.; Rudzinska, I.; Wawrzoniak, T.; Dobrzanska, A.; Czech-Kowalska, J.

2011-01-01

Congenital chylous ascites is a rare entity, conditioned by numerous factors and with changing dynamics of the disease. Because of the lack of therapeutic and diagnostic standards, this disease constitutes to be a medical challenge. This article presents current knowledge on pathogenesis, diagnostics and management of this disease, as well as a case of a newborn with primary congenital chylous ascites in the abdominal cavity. (authors)

Congenital nystagmus and negative electroretinography

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Roussi M

2011-04-01

Full Text Available Mirella Roussi, Hélène Dalens, Jean Jacques Marcellier, Franck BacinDepartment of Ophthalmology, Clermont-Ferrand University, Clermont-Ferrand, FranceAbstract: Congenital nystagmus is a pathologic oculomotor state appearing at about three to four months of age. The precise diagnosis requires detailed clinical examination and electrophysiological findings. This case report presents two male patients with congenital nystagmus examined longitudinally from the age of six months until 17-18 years of age. Clinical and electrophysiological protocols were detailed. The first results showed electronegative electroretinography in the two cases and examination combined with electroretinographic findings helped us to make the diagnosis of Congenital Night Stationary Blindness (CSNB. This diagnosis was confirmed by genetic studies. CSNB is interesting to study because through electrophysiological findings, it enables a better understanding of the physiology of neural transmission in the outer part of the retina.Keywords: Congenital nystagmus, negative electroretinography, congenital night stationary blindness

[Congenital diaphragmatic hernia: respiratory and vascular outcomes].

Science.gov (United States)

Pennaforte, T; Rakza, T; Sfeir, R; Aubry, E; Bonnevalle, M; Fayoux, P; Deschildre, A; Thumerelle, C; de Lagausie, P; Benachi, A; Storme, L

2012-02-01

Congenital diaphragmatic hernia (CDH) is a life-threatening anomaly associated with a variable degree of pulmonary hypoplasia (PH) and persistent pulmonary hypertension (PPH). Despite remarkable advances in neonatal resuscitation and intensive care, and new postnatal treatment strategies, the rates of mortality and morbidity in the newborn with CDH remain high as the result of severe respiratory failure secondary to PH and PPH. Later, lung function assessments show obstructive and restrictive impairments due to altered lung structure and lung damage due to prolonged ventilatory support. The long-term consequences of pulmonary hypertension are unknown. Other problems include chronic pulmonary aspiration caused by gastro-oesophageal reflux and respiratory manifestations of allergy such as asthma or rhinitis. Finally, failure to thrive may be caused by increased caloric requirements due to pulmonary morbidity. Follow-up studies that systematically assess long-term sequelae are needed. Based on such studies, a more focused approach for routine multidisciplinary follow-up programs could be established. It is the goal of the French Collaborative Network to promote exchange of knowledge, future research and development of treatment protocols. Copyright © 2012. Published by Elsevier Masson SAS.

Rice bran protein hydrolysates attenuate diabetic nephropathy in diabetic animal model.

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Boonloh, Kampeebhorn; Lee, Eun Soo; Kim, Hong Min; Kwon, Mi Hye; Kim, You Mi; Pannangpetch, Patchareewan; Kongyingyoes, Bunkerd; Kukongviriyapan, Upa; Thawornchinsombut, Supawan; Lee, Eun Young; Kukongviriyapan, Veerapol; Chung, Choon Hee

2018-03-01

Diabetic nephropathy (DN) is an important microvascular complication of uncontrolled diabetes. The features of DN include albuminuria, extracellular matrix alterations, and progressive renal insufficiency. Rice bran protein hydrolysates (RBPs) have been reported to have antihyperglycemic, lipid-lowering, and anti-inflammatory effects in diabetic rats. Our study was to investigate the renoprotective effects of RBP in diabetic animals and mesangial cultured cells. Eight-week-old male db/m and db/db mice were orally treated with tap water or RBP (100 or 500 mg/kg/day) for 8 weeks. At the end of the experiment, diabetic nephropathy in kidney tissues was investigated for histological, ultrastructural, and clinical chemistry changes, and biomarkers of angiogenesis, fibrosis, inflammation, and antioxidant in kidney were analyzed by Western blotting. Protection against proangiogenic proteins and induction of cytoprotection by RBP in cultured mesangial cells was evaluated. RBP treatment improved insulin sensitivity, decreased elevated fasting serum glucose levels, and improved serum lipid levels and urinary albumin/creatinine ratios in diabetic mice. RBP ameliorated the decreases in podocyte slit pore numbers, thickening of glomerular basement membranes, and mesangial matrix expansion and suppressed elevation of MCP-1, ICAM-1, HIF-1α, VEGF, TGF-β, p-Smad2/3, and type IV collagen expression. Moreover, RBP restored suppressed antioxidant Nrf2 and HO-1 expression. In cultured mesangial cells, RBP inhibited high glucose-induced angiogenic protein expression and induced the expression of Nrf2 and HO-1. RBP attenuates the progression of diabetic nephropathy and restored renal function by suppressing the expression of proangiogenic and profibrotic proteins, inhibiting proinflammatory mediators, and restoring the antioxidant and cytoprotective system.

APOL1 Nephropathy: A Population Genetics and Evolutionary Medicine Detective Story.

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Kruzel-Davila, Etty; Wasser, Walter G; Skorecki, Karl

2017-11-01

Common DNA sequence variants rarely have a high-risk association with a common disease. When such associations do occur, evolutionary forces must be sought, such as in the association of apolipoprotein L1 (APOL1) gene risk variants with nondiabetic kidney diseases in populations of African ancestry. The variants originated in West Africa and provided pathogenic resistance in the heterozygous state that led to high allele frequencies owing to an adaptive evolutionary selective sweep. However, the homozygous state is disadvantageous and is associated with a markedly increased risk of a spectrum of kidney diseases encompassing hypertension-attributed kidney disease, focal segmental glomerulosclerosis, human immunodeficiency virus nephropathy, sickle cell nephropathy, and progressive lupus nephritis. This scientific success story emerged with the help of the tools developed over the past 2 decades in human genome sequencing and population genomic databases. In this introductory article to a timely issue dedicated to illuminating progress in this area, we describe this unique population genetics and evolutionary medicine detective story. We emphasize the paradox of the inheritance mode, the missing heritability, and unresolved associations, including cardiovascular risk and diabetic nephropathy. We also highlight how genetic epidemiology elucidates mechanisms and how the principles of evolution can be used to unravel conserved pathways affected by APOL1 that may lead to novel therapies. The APOL1 gene provides a compelling example of a common variant association with common forms of nondiabetic kidney disease occurring in a continental population isolate with subsequent global admixture. Scientific collaboration using multiple experimental model systems and approaches should further clarify pathomechanisms further, leading to novel therapies. Copyright © 2017 Elsevier Inc. All rights reserved.

Association of chronic obstructive pulmonary disease and obstructive sleep apnea consequences

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Carlos Zamarrón

2009-01-01

Full Text Available Carlos Zamarrón1, Vanesa García Paz1, Emilio Morete1, Felix del Campo Matías21Servicio de Neumología, Hospital Clínico Universitario de Santiago, Santiago, Spain; 2Servicio de Neumologia, Hospital Universitario Rio Ortega de Vallaclolid, Vallaclolid, SpainAbstract: Obstructive sleep apnea syndrome (OSAS and chronic obstructive pulmonary disease (COPD are two diseases that often coexist within an individual. This coexistence is known as overlap syndrome and is the result of chance rather than a pathophysiological link. Although there are claims of a very high incidence of OSAS in COPD patients, recent studies report that it is similar to the general population. Overlap patients present sleep-disordered breathing associated to upper and lower airway obstruction and a reduction in respiratory drive. These patients present unique characteristics, which set them apart from either COPD or OSAS patients. COPD and OSAS are independent risk factors for cardiovascular events and their coexistence in overlap syndrome probably increases this risk. The mechanisms underlying cardiovascular risk are still unclear, but may involve systemic inflammation, endothelial dysfunction, and tonic elevation of sympathetic neural activity. The treatment of choice for overlap syndrome in stable patients is CPAP with supplemental oxygen for correction of upper airway obstructive episodes and hypoxemia during sleep.Keywords: chronic obstructive pulmonary disease, obstructive sleep apnea syndrome, overlap syndrome, sleep, cardiovascular disease

Congenital heart defects: the 10-year experience at a single center.

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Aydin, Emine; Aypar, Ebru; Oktem, Ahmet; Ozyuncu, Ozgur; Yurdakok, Murat; Guvener, Murat; Demircin, Metin; Beksac, M Sinan

2018-06-18

We aimed to evaluate congenital heart disease (CHD) cases according to EUROCAT subgroup classification that were diagnosed during the prenatal period in our center. CHDs that were prenatally diagnosed using ultrasonography and confirmed by fetal echocardiography were reviewed over a 10-year period. Subgroup classification was finalized at the post-partum period in terms of the EUROCAT guide 1.3. Congenital heart defect subtypes and obstetric outcomes (gestational week at delivery, birth weight, gender, extracardiac structural abnormalities, karyotype results if performed) were analyzed. The data of 180 cases with CHD was examined. Left ventricular outflow tract obstruction (LVOT) was the most common CHD subtype (57/180; 31.6%), which included 48, five, and four cases of hypoplastic left heart syndrome (HLHS), coarctation of the aorta, and aortic valve atresia/stenosis, respectively. Eighteen pregnancies were terminated; the most common CHD subtype among patients of terminated pregnancies was hypoplastic left heart syndrome (HLHS) (n = 7, 38.8%). The most common extracardiac malformations were a single umbilical artery, esophageal atresia, and situs inversus in our study group. Eighteen of the 96 (18.75%) neonates with CHD died during the neonatal period. The most common CHD subtype was HLHS (7/18; 38%) among the newborns who died after birth. Prenatal diagnosis of a CHD and subgroup classification is very important for clinical decision making, including prenatal management, recommendations for termination of the pregnancy, postnatal management of the patient, and for early referral to pediatric cardiology and cardiovascular surgery centers.

Outcome after prenatal diagnosis of congenital anomalies of the kidney and urinary tract.

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Nef, Samuel; Neuhaus, Thomas J; Spartà, Giuseppina; Weitz, Marcus; Buder, Kathrin; Wisser, Josef; Gobet, Rita; Willi, Ulrich; Laube, Guido F

2016-05-01

Congenital anomalies of the kidney and urinary tract are common findings on fetal ultrasound. The aim of this prospective observational study was to describe outcome and risk factors in 115 patients born 1995-2001. All prenatally diagnosed children were stratified into low- and high-risk group and followed postnatally clinically and by imaging at defined endpoints. Risk factors were evaluated using odds ratios. Neonatal diagnosis included pelvi-ureteric junction obstruction (n = 33), vesicoureteral reflux (n = 27), solitary mild pelvic dilatation (postnatal anteroposterior diameter 5-10 mm; n = 25), and further diagnosis as primary obstructive megaureter, unilateral multicystic dysplastic kidney, renal dysplasia and posterior urethral valves. In 38 children with prenatal isolated hydronephrosis, ultrasound normalized at median age of 1.2 years (range 0.1-9). Surgery was performed in 34 children at median age of 0.4 years (0.1-10.8). Persistent renal anomalies without surgery were present in 43 children and followed in 36 for median time of 16 years (12.2-18). Oligohydramnios and postnatal bilateral anomalies were significantly associated with surgery and impaired renal function. The majority of children had a favourable postnatal outcome, in particular children with prenatally low risk, i.e. isolated uni- or bilateral hydronephrosis. Oligohydramnios and postnatal bilateral anomalies were risk factors for non-favourable outcome. • In congenital anomalies of the kidney and urinary tract significantly poorer outcome is known in patients with bilateral renal hypoplasia or solitary kidney associated with posterior urethral valves. • Other factors as proteinuria and vesicoureteral reflux were associated with a higher risk of progression to chronic renal failure in these patients. What is New: • Unlike other studies giving us above-mentioned information, we included all patients with any kind of prenatally diagnosed congenital anomalies of the kidney

Within-breath respiratory impedance and airway obstruction in patients with chronic obstructive pulmonary disease.

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Silva, Karla Kristine Dames da; Faria, Alvaro Camilo Dias; Lopes, Agnaldo José; Melo, Pedro Lopes de

2015-07-01

Recent work has suggested that within-breath respiratory impedance measurements performed using the forced oscillation technique may help to noninvasively evaluate respiratory mechanics. We investigated the influence of airway obstruction on the within-breath forced oscillation technique in smokers and chronic obstructive pulmonary disease patients and evaluated the contribution of this analysis to the diagnosis of chronic obstructive pulmonary disease. Twenty healthy individuals and 20 smokers were assessed. The study also included 74 patients with stable chronic obstructive pulmonary disease. We evaluated the mean respiratory impedance (Zm) as well as values for the inspiration (Zi) and expiration cycles (Ze) at the beginning of inspiration (Zbi) and expiration (Zbe), respectively. The peak-to-peak impedance (Zpp=Zbe-Zbi) and the respiratory cycle dependence (ΔZrs=Ze-Zi) were also analyzed. The diagnostic utility was evaluated by investigating the sensitivity, the specificity and the area under the receiver operating characteristic curve. ClinicalTrials.gov: NCT01888705. Airway obstruction increased the within-breath respiratory impedance parameters that were significantly correlated with the spirometric indices of airway obstruction (R=-0.65, pdisease patients presented significant expiratory-inspiratory differences (p90%). We conclude the following: (1) chronic obstructive pulmonary disease introduces higher respiratory cycle dependence, (2) this increase is proportional to airway obstruction, and (3) the within-breath forced oscillation technique may provide novel parameters that facilitate the diagnosis of respiratory abnormalities in chronic obstructive pulmonary disease.

Within-breath respiratory impedance and airway obstruction in patients with chronic obstructive pulmonary disease

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Karla Kristine Dames da Silva

2015-07-01

Full Text Available OBJECTIVE: Recent work has suggested that within-breath respiratory impedance measurements performed using the forced oscillation technique may help to noninvasively evaluate respiratory mechanics. We investigated the influence of airway obstruction on the within-breath forced oscillation technique in smokers and chronic obstructive pulmonary disease patients and evaluated the contribution of this analysis to the diagnosis of chronic obstructive pulmonary disease. METHODS: Twenty healthy individuals and 20 smokers were assessed. The study also included 74 patients with stable chronic obstructive pulmonary disease. We evaluated the mean respiratory impedance (Zm as well as values for the inspiration (Zi and expiration cycles (Ze at the beginning of inspiration (Zbi and expiration (Zbe, respectively. The peak-to-peak impedance (Zpp=Zbe-Zbi and the respiratory cycle dependence (ΔZrs=Ze-Zi were also analyzed. The diagnostic utility was evaluated by investigating the sensitivity, the specificity and the area under the receiver operating characteristic curve. ClinicalTrials.gov: NCT01888705. RESULTS: Airway obstruction increased the within-breath respiratory impedance parameters that were significantly correlated with the spirometric indices of airway obstruction (R=−0.65, p90%. CONCLUSIONS: We conclude the following: (1 chronic obstructive pulmonary disease introduces higher respiratory cycle dependence, (2 this increase is proportional to airway obstruction, and (3 the within-breath forced oscillation technique may provide novel parameters that facilitate the diagnosis of respiratory abnormalities in chronic obstructive pulmonary disease.

Contrast media induced nephropathy: a literature review of the available evidence and recommendations for practice.

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Deek, Hiba; Newton, Phillip; Sheerin, Noella; Noureddine, Samar; Davidson, Patricia M

2014-11-01

Contrast media induced nephropathy (CIN) is a sudden compromise of renal function 24-48 h after administering contrast medium during a CT scan or angiography. CIN accounts for 10% of hospital acquired renal failure and is ranked the third cause of acquiring this condition. Identifying patients at risk through proper screening can reduce the occurrence of this condition. This review paper aims to critique current evidence, provide a better understanding of CIN, inform nursing practice and make recommendations for bedside nurses and future research. An integrative review of the literature was made using the key terms: "contrast media", "nephritis", "nephropathy", "contrast media induced nephropathy scores", "acute kidney failure", "acute renal failure" and "acute kidney injury". MeSH key terms used in some databases were: "prevention and control", "acute kidney failure" and "treatment". Databases searched included Medline, CINAHL and Academic Search Complete, and references of relevant articles were also assessed. The search included all articles between the years 2000 and 2013. Sixty-seven articles were obtained as a result of the search, including RCTs, systematic reviews, and retrospective studies. Contrast media induced nephropathy is an iatrogenic complication occurring secondary to diagnostic or therapeutic procedures. At times it is unavoidable but a systematic method of risk assessment should be adopted to identify high risk patients for tailored and targeted approaches to management interventions. As the use of contrast media is increasing for diagnostic purposes, it is important that nurses be aware of the risk factors for CIN, identify and monitor high risk patients to prevent deterioration in renal function when possible. Copyright © 2014 Australian College of Critical Care Nurses Ltd. Published by Elsevier Ltd. All rights reserved.

Effect of different blood purification methods on adipokines and flammatory factor in patients with diabetic nephropathy

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Chun Zhong

2016-02-01

Full Text Available Objective: To investigate the effect of in different type hemopurification techniques on adipokine and flammatory factor in diabetic nephropathy with maintenance hemodialysis (MHD. Methods: A total of 81 diabetic nephropathy patients were randomly divided into on hemodialysis group (HD, n=27, hemodialysis with hemoperfusion group (HD+HP, n=27, online hemodiafiltration group (OL-HDF, n=27. Levels of adiponectin, resistin, interleukin-6 (IL-6, and tumor necrosis factor (TNF-α were tested and compared before and after treatment. Results: Indicators of HD group have no statistical significance before and after treatment. HD+HP and OL-HDF group adiponectin level increased significantly, resistin, IL-6 and TNF-α level decreased significantly before and after treatment; OL-HDF group resistin, IL-6 and TNF-α level decreased more significantly than HD+HP group after treatment. Conclusion: HD+HP and OL-HDF treatments have reliable curative effect on diabetic nephropathy patients, which can regulate adiponectin, resistin, IL-6 and TNF-α level. Effect of OL-HDF treatment eliminates resistin, IL-6 and TNF-α more markedly than HD+HP treatment.

Congenital and perinatal cytomegalovirus infection

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Chun Soo Kim

2010-01-01

Full Text Available Cytomegalovirus (CMV is currently the most common agent of congenital infection and the leading infectious cause of brain damage and hearing loss in children. Symptomatic congenital CMV infections usually result from maternal primary infection during early pregnancy. One half of symptomatic infants have cytomegalic inclusion disease (CID, which is characterized by involvement of multiple organs, in particular, the reticuloendothelial and central nervous system (CNS. Moreover, such involvement may or may not include ocular and auditory damage. Approximately 90% of infants with congenital infection are asymptomatic at birth. Preterm infants with perinatal CMV infection can have symptomatic diseases such as pneumonia, hepatitis, and thrombocytopenia. Microcephaly and abnormal neuroradiologic imaging are associated with a poor prognosis. Hearing loss may occur in both symptomatic and asymptomatic infants with congenital infection and may progress through childhood. Congenital infection is defined by the isolation of CMV from infants within the first 3 weeks of life. Ganciclovir therapy can be considered for infants with symptomatic congenital CMV infection involving the CNS. Pregnant women of seronegative state should be counseled on the importance of good hand washing and other control measures to prevent CMV infection. Heat treatment of infected breast milk at 72?#608;for 5 seconds can eliminate CMV completely.

Carvedilol Protects against Cyclosporine Nephropathy in Rats

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H. Kotolová

2006-01-01

Full Text Available The aim of our experimental work was to study whether carvedilol is able to protect renal tissue from cyclosporine toxic effect in animal model of cyclosporine nephropathy. The study was performed on twenty Wistar rats divided in two experimental groups: control (treated with placebo and carvedilol (treated with p.o. dose 10mg/kg/day in 1 ml solution. Cyclosporine in oral dose of 15 mg/kg/day was administered to all animals during 15 days of experiment. Urine was collected daily for the assessment of diuresis, proteinuria, and determination of urea and creatinine levels. Serum collected at the end of the experiment (day 15 was used for the determination of urea and transferrin levels. The level of renal tissue damage was evaluated by the Jones method for basal membranes, glomeruli and tubuli impregnation, and by the Kossa method for calcium impregnation. For the determination of paranuclear inclusions presence we used chromanilinblue (CAB method. Statistically significant differences between total protein levels in urine on day 7 of the experiment and urea levels in serum at the end of the experiment in the control group and the carvedilol-treated group indicate a protective effect of carvedilol on renal tissue, which is supported also by the results of a histological examination of renal tissue. Significant increase in the serum transferrin level was registered in the carvedilol-treated group and no significant changes were noted in ceruloplasmin serum levels. In conclusion, our pilot study showed that carvedilol has the ability to protect renal tissue from cyclosporine induced nephropathy in rats.

Role of metabolic control on diabetic nephropathy

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Macedo Célia Sperandéo

2002-01-01

Full Text Available OBJECTIVE: The aim of this investigation was studying the influence of glucose metabolic control on diabetic nephropathy. The authors observed the effect of acarbose, insulin, and both drugs on the metabolic control and development of mesangial enlargement of kidney glomeruli in alloxan-diabetic rats. METHODS: Five groups of Wistar rats were used: normal rats (N, non-treated alloxan-diabetic rats (D, alloxan-diabetic rats treated with acarbose (AD, alloxan-diabetic rats treated with insulin (ID, and alloxan-diabetic rats treated with insulin plus acarbose (IAD. The following parameters were evaluated: body weight; water and food intake; diuresis; blood and urine glucose levels; and the kidney lesions: mesangial enlargement and tubule cell vacuolization. Renal lesions were analysed using a semi-quantitative score 1, 3, 6, 9, and 12 months after diabetes induction. RESULTS: Diabetic rats showed a marked increase of glycemia, urinary glucose levels, diuresis, water and food intake, and weight loss, while the treated diabetic rats showed significant decreased levels of these parameters. The most satisfactory metabolic control was that of diabetic rats treated with acarbose + insulin. There was a significant mesangial enlargement in diabetic rats compared to normal rats from the third up to the 12th month after diabetes induction, with a significant difference between the animals treated with acarbose + insulin and non-treated diabetic rats. A difference between the animals treated with acarbose or insulin alone and non-treated diabetics rats was not seen. CONCLUSIONS: The authors discuss the results stressing the role of diabetic metabolic control in the prevention of diabetic nephropathy.

Diabetic nephropathy and antioxidants.

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Tavafi, Majid

2013-01-01

Oxidative stress has crucial role in pathogenesis of diabetic nephropathy (DN). Despite satisfactory results from antioxidant therapy in rodent, antioxidant therapy showed conflicting results in combat with DN in diabetic patients. Directory of Open Access Journals (DOAJ), Google Scholar,Pubmed (NLM), LISTA (EBSCO) and Web of Science have been searched. Treatment of DN in human are insufficient with rennin angiotensin system (RAS) blockers, so additional agent ought to combine with this management. Meanwhile based on DN pathogenesis and evidences in experimental and human researches, the antioxidants are the best candidate. New multi-property antioxidants may be improved human DN that show high power antioxidant capacity, long half-life time, high permeability to mitochondrion, improve body antioxidants enzymes activity and anti-inflammatory effects. Based on this review and our studies on diabetic rats, rosmarinic acid a multi-property antioxidant may be useful in DN patients, but of course, needs to be proven in clinical trials studies.

Oral contraceptives, angiotensin-dependent renal vasoconstriction, and risk of diabetic nephropathy

DEFF Research Database (Denmark)

Ahmed, Sofia B; Hovind, Peter; Parving, Hans-Henrik

2005-01-01

OBJECTIVE: Diabetes, the leading cause of end-stage renal disease in the U.S., is believed to involve activation of the renin angiotensin system (RAS) as a risk factor for nephropathy. RAS activation occurs in healthy women using oral contraceptives (OCs), but the effects of OC use on the diabeti...

Remote Ischemic Preconditioning To Reduce Contrast-Induced Nephropathy: A Randomized Controlled Trial

NARCIS (Netherlands)

Menting, T.P.; Sterenborg, T.B.; Waal, Y.R. de; Donders, R.; Wever, K.E.; Lemson, M.S.; Vliet, J.A. van der; Wetzels, J.F.M.; Schultze Kool, L.J.; Warle, M.C.

2015-01-01

BACKGROUND: Despite the increasing use of pre- and post-hydration protocols and low osmolar instead of high osmolar iodine containing contrast media, the incidence of contrast induced nephropathy (CIN) is still significant. There is evidence that contrast media cause ischemia reperfusion injury of

Multifocal Congenital Hemangiopericytoma.

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Robl, Renata; Carvalho, Vânia Oliveira; Abagge, Kerstin Taniguchi; Uber, Marjorie; Lichtvan, Leniza Costa Lima; Werner, Betina; Mehrdad Nadji, Mehrdad

2017-01-01

Congenital hemangiopericytoma (HPC) is a rare mesenchymal tumor with less aggressive behavior and a more favorable prognosis than similar tumors in adults. Multifocal presentation is even less common than isolated HPC and hence its clinical and histologic recognition may be challenging. A newborn infant with multifocal congenital HPC causing severe deformity but with a favorable outcome after chemotherapy and surgical removal is reported. © 2016 Wiley Periodicals, Inc.

Apolipoprotein(a) in insulin-dependent diabetic patients with and without diabetic nephropathy

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Gall, M A; Rossing, P; Hommel, E

1992-01-01

Insulin-dependent diabetic patients with diabetic nephropathy have a highly increased morbidity and mortality from cardiovascular diseases. To determine whether altered levels of apolipoprotein(a) (apo(a)), the glycoprotein of the potentially atherogenic lipoprotein(a) (Lp(a)), contribute...... to the increased risk of ischaemic heart disease, apo(a) was determined in 50 insulin-dependent diabetic patients with diabetic nephropathy (group 1), in 50 insulin-dependent diabetic patients with microalbuminuria (group 2), in 50 insulin-dependent diabetic patients with normoalbuminuria (group 3), and in 50...... healthy subjects (group 4). The groups were matched with regard to sex, age and body mass index. The diabetic groups were also matched with regard to diabetes duration. The level of apo(a) was approximately the same in the four groups, being: 122 (x/ divided by 4.2) U l-1, 63 (x/ divided by 4.4) U l-1...

A Meta-Analysis of Randomized Controlled Trials of Yiqi Yangyin Huoxue Method in Treating Diabetic Nephropathy

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Jiao Ying Ou

2016-01-01

Full Text Available Objective. The purpose of this systematic review is to evaluate the evidence of Yiqi Yangyin Huoxue Method for diabetic nephropathy. Methods. 11 electronic databases, through September 2015, were searched to identify randomized controlled trials of Yiqi Yangyin Huoxue Method for diabetic nephropathy. The quality of the included trials was assessed using the Jadad scale. Results. 26 randomized controlled trials were included in our review. Of all the included trials, most of them were considered as high quality. The aggregated results suggested that Yiqi Yangyin Huoxue Method is beneficial to diabetic nephropathy in bringing down the microalbuminuria (SMD = −0.98, 95% CI −1.22 to −0.74, serum creatinine (SMD = −0.56, 95% CI −0.93 to −0.20, beta-2 microglobulin (MD = 0.06, 95% CI 0.01 to 0.12, fasting plasma glucose (MD = −0.35, 95% CI −0.62 to −0.08, and 2-hour postprandial blood glucose (MD = 1.13, 95% CI 0.07 to 2.20, but not in decreasing blood urea nitrogen (SMD = −0.72, 95% CI −1.47 to 0.02 or 2-hour postprandial blood glucose (SMD = −0.48, 95% CI −1.01 to 0.04. Conclusions. Yiqi Yangyin Huoxue Method should be a valid complementary and alternative therapy in the management of diabetic nephropathy, especially in improving UAER, serum creatinine, fasting blood glucose, and beta-2 microglobulin. However, more studies with long follow-up are warrant to confirm the current findings.

Albumin Antioxidant Response to Stress in Diabetic Nephropathy Progression

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Medina-Navarro, Rafael; Corona-Candelas, Itzia; Barajas-González, Saúl; Díaz-Flores, Margarita; Durán-Reyes, Genoveva

2014-01-01

Background A new component of the protein antioxidant capacity, designated Response Surplus (RS), was recently described. A major feature of this component is the close relationship between protein antioxidant capacity and molecular structure. Oxidative stress is associated with renal dysfunction in patients with renal failure, and plasma albumin is the target of massive oxidation in nephrotic syndrome and diabetic nephropathy. The aim of the present study was to explore the albumin redox state and the RS component of human albumin isolated from diabetic patients with progressive renal damage. Methods/Principal Findings Serum aliquots were collected and albumin isolated from 125 diabetic patients divided into 5 groups according to their estimated glomerular filtration rate (GFR). In addition to clinical and biochemical variables, the albumin redox state, including antioxidant capacity, thiol group content, and RS component, were evaluated. The albumin antioxidant capacity and thiol group content were reciprocally related to the RS component in association with GFR reduction. The GFR decline and RS component were significantly negatively correlated (R = –0.83, palbumin to stress in relation to the progression of diabetic renal disease was evaluated. The findings confirm that the albumin molecular structure is closely related to its redox state, and is a key factor in the progression of diabetes nephropathy. PMID:25187963

Angiotensin converting enzyme 2 amplification limited to the circulation does not protect mice from development of diabetic nephropathy

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Wysocki, Jan; Ye, Minghao; Khattab, Ahmed M.; Fogo, Agnes; Martin, Aline; David, Nicolae Valentin; Kanwar, Yashpal; Osborn, Mark; Batlle, Daniel

2016-01-01

Blockers of the renin-angiotensin system are effective in the treatment of experimental and clinical diabetic nephropathy. An approach different from blocking the formation or action of angiotensin II(1-8) that could also be effective involves fostering its degradation. Angiotensin converting enzyme 2 (ACE2) is a monocarboxypeptidase than cleaves angiotensin II (1-8) to form angiotensin (1-7). Therefore, we examined the renal effects of murine recombinant ACE2 in mice with streptozotocin-induced diabetic nephropathy as well as that of amplification of circulating ACE2 using minicircle DNA delivery prior to induction of experimental diabetes. This delivery resulted in a long-term sustained and profound increase in serum ACE2 activity and enhanced ability to metabolize an acute angiotensin II (1-8) load. In mice with streptozotocin-induced diabetes pretreated with minicircle ACE2, ACE2 protein in plasma increased markedly and this was associated with a more than 100-fold increase in serum ACE2 activity. However, minicircle ACE2 did not result in changes in urinary ACE2 activity as compared to untreated diabetic mice. In both diabetic groups, glomerular filtration rate increased significantly and to the same extent as compared to non-diabetic controls. Albuminuria, glomerular mesangial expansion, glomerular cellularity and glomerular size, were all increased to a similar extent in minicircle ACE2-treated and untreated diabetic mice, as compared to non-diabetic controls. Recombinant mouse ACE2 given for 4 weeks by intraperitoneal daily injections in mice with streptozotocin-induced diabetic nephropathy also failed to improve albuminuria or kidney pathology. Thus, a profound augmentation of ACE2 confined to the circulation failed to ameliorate the glomerular lesions and hyperfiltration characteristic of early diabetic nephropathy. These findings emphasize the importance of targeting the kidney rather than the circulatory renin angiotensin system to combat diabetic

Value of adding the renal pathological score to the kidney failure risk equation in advanced diabetic nephropathy.

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Masayuki Yamanouchi

Full Text Available There have been a limited number of biopsy-based studies on diabetic nephropathy, and therefore the clinical importance of renal biopsy in patients with diabetes in late-stage chronic kidney disease (CKD is still debated. We aimed to clarify the renal prognostic value of pathological information to clinical information in patients with diabetes and advanced CKD.We retrospectively assessed 493 type 2 diabetics with biopsy-proven diabetic nephropathy in four centers in Japan. 296 patients with stage 3-5 CKD at the time of biopsy were identified and assigned two risk prediction scores for end-stage renal disease (ESRD: the Kidney Failure Risk Equation (KFRE, a score composed of clinical parameters and the Diabetic Nephropathy Score (D-score, a score integrated pathological parameters of the Diabetic Nephropathy Classification by the Renal Pathology Society (RPS DN Classification. They were randomized 2:1 to development and validation cohort. Hazard Ratios (HR of incident ESRD were reported with 95% confidence interval (CI of the KFRE, D-score and KFRE+D-score in Cox regression model. Improvement of risk prediction with the addition of D-score to the KFRE was assessed using c-statistics, continuous net reclassification improvement (NRI, and integrated discrimination improvement (IDI.During median follow-up of 1.9 years, 194 patients developed ESRD. The cox regression analysis showed that the KFRE,D-score and KFRE+D-score were significant predictors of ESRD both in the development cohort and in the validation cohort. The c-statistics of the D-score was 0.67. The c-statistics of the KFRE was good, but its predictive value was weaker than that in the miscellaneous CKD cohort originally reported (c-statistics, 0.78 vs. 0.90 and was not significantly improved by adding the D-score (0.78 vs. 0.79, p = 0.83. Only continuous NRI was positive after adding the D-score to the KFRE (0.4%; CI: 0.0-0.8%.We found that the predict values of the KFRE and the D

Value of adding the renal pathological score to the kidney failure risk equation in advanced diabetic nephropathy.

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Yamanouchi, Masayuki; Hoshino, Junichi; Ubara, Yoshifumi; Takaichi, Kenmei; Kinowaki, Keiichi; Fujii, Takeshi; Ohashi, Kenichi; Mise, Koki; Toyama, Tadashi; Hara, Akinori; Kitagawa, Kiyoki; Shimizu, Miho; Furuichi, Kengo; Wada, Takashi

2018-01-01

There have been a limited number of biopsy-based studies on diabetic nephropathy, and therefore the clinical importance of renal biopsy in patients with diabetes in late-stage chronic kidney disease (CKD) is still debated. We aimed to clarify the renal prognostic value of pathological information to clinical information in patients with diabetes and advanced CKD. We retrospectively assessed 493 type 2 diabetics with biopsy-proven diabetic nephropathy in four centers in Japan. 296 patients with stage 3-5 CKD at the time of biopsy were identified and assigned two risk prediction scores for end-stage renal disease (ESRD): the Kidney Failure Risk Equation (KFRE, a score composed of clinical parameters) and the Diabetic Nephropathy Score (D-score, a score integrated pathological parameters of the Diabetic Nephropathy Classification by the Renal Pathology Society (RPS DN Classification)). They were randomized 2:1 to development and validation cohort. Hazard Ratios (HR) of incident ESRD were reported with 95% confidence interval (CI) of the KFRE, D-score and KFRE+D-score in Cox regression model. Improvement of risk prediction with the addition of D-score to the KFRE was assessed using c-statistics, continuous net reclassification improvement (NRI), and integrated discrimination improvement (IDI). During median follow-up of 1.9 years, 194 patients developed ESRD. The cox regression analysis showed that the KFRE,D-score and KFRE+D-score were significant predictors of ESRD both in the development cohort and in the validation cohort. The c-statistics of the D-score was 0.67. The c-statistics of the KFRE was good, but its predictive value was weaker than that in the miscellaneous CKD cohort originally reported (c-statistics, 0.78 vs. 0.90) and was not significantly improved by adding the D-score (0.78 vs. 0.79, p = 0.83). Only continuous NRI was positive after adding the D-score to the KFRE (0.4%; CI: 0.0-0.8%). We found that the predict values of the KFRE and the D-score were

Congenital left paraduodenal hernia causing chronic abdominal pain and abdominal catastrophe.

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Shi, Yan; Felsted, Amy E; Masand, Prakash M; Mothner, Brent A; Nuchtern, Jed G; Rodriguez, J Ruben; Vasudevan, Sanjeev A

2015-04-01

Paraduodenal hernias are the most common type of congenital internal hernia. Because of its overall rare incidence, this entity is often overlooked during initial assessment of the patient. Lack of specific diagnostic criteria also makes diagnosis exceedingly difficult, and the resulting diagnostic delays can lead to tragic outcomes for patients. Despite these perceived barriers to timely diagnosis, there may be specific radiographic findings that, when combined with the appropriate constellation of clinical symptoms, would aid in diagnosis. This patient first presented at 8 years of age with vague symptoms of postprandial emesis, chronic abdominal pain, nausea, and syncope. Over the span of 6 years he was evaluated 2 to 3 times a year with similar complaints, all of which quickly resolved spontaneously. He underwent multiple laboratory, imaging, and endoscopic studies, which were nondiagnostic. It was not until he developed signs of a high-grade obstruction and extremis that he was found to have a large left paraduodenal hernia that had volvulized around the superior mesenteric axis. This resulted in the loss of the entire superior mesenteric axis distribution of the small and large intestine and necrosis of the duodenum. In cases of chronic intermittent obstruction without clear etiology, careful attention and consideration should be given to the constellation of symptoms, imaging studies, and potential use of diagnostic laparoscopy. Increased vigilance by primary care and consulting physicians is necessary to detect this rare but readily correctable condition. Copyright © 2015 by the American Academy of Pediatrics.

Assessment of glomerular filtration rate in diabetic nephropathy using the plasma clearance of 51Cr-EDTA

DEFF Research Database (Denmark)

Hansen, H P; Rossing, P; Mathiesen, E R

1998-01-01

Plasma clearance of 51Cr-EDTA is widely used to assess the glomerular filtration rate (GFR) in diabetic nephropathy. Originally, the ratio between the intravenously injected amount of tracer and the total area under the plasma concentration curve was used for the calculation of total 51Cr...... and rate of decline in GFR based upon these three methods. Bland & Altman plots were used to illustrate the range of agreement. We investigated 76 insulin-dependent diabetic (IDDM) patients with microalbuminuria or diabetic nephropathy. GFR was measured after a single intravenous injection of 3.7 MBq 51Cr...

Sodium bicarbonate versus isotonic saline solution to prevent contrast-induced nephropathy : a systematic review and meta-analysis.

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Zapata-Chica, Carlos Andres; Bello Marquez, Diana; Serna-Higuita, Lina Maria; Nieto-Ríos, John Fredy; Casas-Arroyave, Fabian David; Donado-Gómez, Jorge Hernando

2015-09-30

Contrast-induced nephropathy is one of the main causes of acute kidney injury and increased hospital-acquired morbidity and mortality. The use of sodium bicarbonate for nephroprotection has emerged as a preventative strategy; however, its efficacy is controversial compared to other strategies, such as hydration using 0.9% saline solution. To compare the effectiveness of sodium bicarbonate vs. hydration using 0.9% saline solution to prevent contrast-induced acute kidney injury. A systematic review of studies registered in the COCHRANE, PUBMED, MEDLINE, LILACS, SCIELO and EMBASE databases was conducted. Randomized controlled studies that evaluated the use of 0.9% saline solution vs. sodium bicarbonate to prevent contrast-induced nephropathy were included. A total of 22 studies (5,686 patients) were included. Sodium bicarbonate did not decrease the risk of contrast-induced nephropathy (RD= 0.00; 95% CI= -0.02 to 0.03; p= 0.83; I(2)= 0%). No significant differences were found in the demand for renal replacement therapy (RD= 0.00; 95% CI= -0.01 to 0-01; I(2)= 0%; p= 0.99) or in mortality (RD= -0.00; 95% CI= -0.001 to 0.001; I(2)= 0%; p= 0.51). Sodium bicarbonate administration is not superior to the use of 0.9% saline solution for preventing contrast-induced nephropathy in patients with risk factors, nor is it better at reducing mortality or the need for renal replacement therapy.

Cathepsin L is crucial for the development of early experimental diabetic nephropathy

NARCIS (Netherlands)

Garsen, M.; Rops, A.; Dijkman, H.B.; Willemsen, B.K.; Kuppevelt, T.H. van; Russel, F.G.M.; Rabelink, T.J.; Berden, J.H.; Reinheckel, T.; Vlag, J. van der

2016-01-01

Proteinuria is one of the first clinical signs of diabetic nephropathy and an independent predictor for the progression to renal failure. Cathepsin L, a lysosomal cysteine protease, can be involved in the development of proteinuria by degradation of proteins that are important for normal podocyte

Complex Etiology, Prophylaxis and Hygiene Control in Mycotoxic Nephropathies in Farm Animals and Humans

Directory of Open Access Journals (Sweden)

Stoycho D. Stoev

2008-04-01

Full Text Available Various etiological factors contributing to the development of mycotoxic nephropathy in farm animals and humans are reviewed. The possible synergistic effect between ochratoxin A (OTA and other mycotoxins, as penicillic acid (PA and fumonisin B1 (FB1, contributing to this nephropathy is also considered and discussed. The most convenient ways of prophylaxis and various preventive measures against OTA contamination of feeds or foods are reviewed. A reference is made concerning the most successful methods of veterinary hygiene control in the slaughterhouses in order to prevent the entering of OTA in commercial channels with a view to human health. The economic efficacy of these prophylactic procedures is also considered. An evaluation of human exposure to OTA is made.

The endothelin antagonist atrasentan lowers residual albuminuria in patients with type 2 diabetic nephropathy

DEFF Research Database (Denmark)

de Zeeuw, Dick; Coll, Blai; Andress, Dennis

2014-01-01

Despite optimal treatment, including renin-angiotensin system (RAS) inhibitors, patients with type 2 diabetic nephropathy have high cardiorenal morbidity and mortality related to residual albuminuria. We evaluated whether or not atrasentan, a selective endothelin A receptor antagonist, further...... reduces albuminuria when administered concomitantly with maximum tolerated labeled doses of RAS inhibitors. We enrolled 211 patients with type 2 diabetes, urine albumin/creatinine ratios of 300-3500 mg/g, and eGFRs of 30-75 ml/min per 1.73 m(2) in two identically designed, parallel, multinational, double...... parameters returned to pretreatment levels. In conclusion, atrasentan reduced albuminuria and improved BP and lipid spectrum with manageable fluid overload-related adverse events in patients with type 2 diabetic nephropathy receiving RAS inhibitors....

Reduced upper obstructions in N3 and increased lower obstructions in REM sleep stage detected with manometry.

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Wirth, Markus; Schramm, Juliane; Bautz, Maximilian; Hofauer, Benedikt; Edenharter, Günther; Ott, Armin; Heiser, Clemens

2018-01-01

In obstructive sleep apnea (OSA), airway obstruction occurs at different anatomic levels. The frequency and location of obstructions play a crucial role in the planning of surgical treatment. The aim of this study was to evaluate the pharyngeal obstruction levels in different sleep stages with manometry in OSA patients. In addition, the manometry results were compared with drug-induced sleep endoscopy (DISE). Forty-one patients with OSA received manometry measurements during one night of sleep. All patients were simultaneously evaluated with polysomnography. The frequency of obstructions in different sleep stages was assessed. Twenty patients were additionally studied with DISE. Obstruction levels detected with manometry were compared with DISE. The frequency of upper and to a lesser extent lower obstructions decreased in sleep stage N3. In rapid eye movement (REM) sleep, lower obstructions increased. The overall proportion of upper and lower obstructions detected with manometry corresponded with DISE in 13 of 20 cases. A significant change in the obstruction levels was detected with manometry in N3 and REM sleep. The reduction of both upper and to a lesser extent lower obstructions in N3 suggests more stable airways in slow-wave sleep. Relevant lower obstructions were not detected in DISE compared to manometry in 5 out of 20 examinations. This could be a potential reason for treatment failure of site-specific surgical OSA treatment when only performing DISE preoperatively. Therefore, manometry could be a useful complementary tool in the preoperative evaluation for OSA.

Mesoamerican nephropathy: a neglected tropical disease with an infectious etiology?

Science.gov (United States)

Murray, Kristy O; Fischer, Rebecca S B; Chavarria, Denis; Duttmann, Christiane; Garcia, Melissa N; Gorchakov, Rodion; Hotez, Peter J; Jiron, William; Leibler, Jessica H; Lopez, Job E; Mandayam, Sreedhar; Marin, Alejandro; Sheleby, Jessica

2015-10-01

An outbreak of unexplained and severe kidney disease, "Mesoamerican Nephropathy," in mostly young, male sugar cane workers emerged in Central America in the late 1990's. As a result, an estimated 20,000 individuals have died, to date. Unfortunately, and with great consequence to human life, the etiology of the outbreak has yet to be identified. The sugarcane fields in Chichigalpa, Chinandega, Nicaragua, have been involved in the outbreak, and during our initial investigation, we interviewed case patients who experienced fever, nausea and vomiting, arthralgia, myalgia, headache, neck and back pain, weakness, and paresthesia at the onset of acute kidney disease. We also observed a heavy infestation of rodents, particularly of Sigmodon species, in the sugarcane fields. We hypothesize that infectious pathogens are being shed through the urine and feces of these rodents, and workers are exposed to these pathogens during the process of cultivating and harvesting sugarcane. In this paper, we will discuss the epidemic in the Chichigalpa area, potential pathogens responsible for Mesoamerican Nephropathy, and steps needed in order to diagnose, treat, and prevent future cases from occurring. Copyright © 2015 Institut Pasteur. Published by Elsevier Masson SAS. All rights reserved.

Genetics Home Reference: congenital mirror movement disorder

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... Health Conditions Congenital mirror movement disorder Congenital mirror movement disorder Printable PDF Open All Close All Enable ... view the expand/collapse boxes. Description Congenital mirror movement disorder is a condition in which intentional movements ...

Congenital anterior urethral valves and diverticula: Diagnosis and management in six cases

Directory of Open Access Journals (Sweden)

Rawat Jiledar

2009-01-01

Full Text Available Background: Anterior urethral valves (AUVs are rare congenital anomalies causing lower urinary tract obstruction in children. Although they are referred to as valves, these obstructive structures often occur in the form of a diverticulum. The urethra in these cases shows saccular or bulbar dilatation known as anterior urethral diverticulum (AUD. They typically occur where there is a defect in the corpus spongiosum, leaving a thin-walled urethra. This segment of the urethra balloons out during voiding, simulating a mass that is sometimes visible along the ventral wall of the penis. The swelling is fluctuant and urine dribbles from the meatus on compression. The present study highlights the clinical approach in identifying the condition and its treatment options, especially for those, presenting with urethral diverticula. Materials and Methods: We have studied children with congenital anterior urethral valves and diverticula. Six patients of AUVs with diverticula were admitted during the period of 2000-2007 and were prospectively evaluated. The mean age of presentation was 16 months (15 days to 4 years. Straining at micturition and a palpable penile swelling were the most common presenting features. The diagnosis was established by voiding cystourethrogram (VCUG and supported by ultrasonography (USG. All patients were treated with single-stage open surgical excision except one who died preoperatively due to urosepsis. Initial lay opening of the penoscrotal urethra and delayed repair were done in one patient. Results: The surgical outcome was successful in all but one patient, who died of delayed presentation with severe back pressure changes, urinary ascitis and urosepsis. On long-term follow-up, all patients demonstrated good stream of urine. The renal functions were normal and the patients had no evidence of urinary infections. Conclusion: We propose that, the patients of AUVs, if not excessively delayed for treatment are otherwise well in terms

Radiology of congenital heart disease

International Nuclear Information System (INIS)

Amplatz, K.

1986-01-01

This is a text on the radiologic diagnosis of congenital heart disease and its clinical manifestations. The main thrust of the book is the logical approach which allows an understanding of the complex theory of congenital heart disease. The atlas gives a concise overview of the entire field of congenital heart disease. Emphasis is placed on the understanding of the pathophysiology and its clinical and radiological consequences. Surgical treatment is included since it provides a different viewpoint of the anatomy

A Multicenter Randomized Controlled Trial of Rituximab versus Cyclosporine in the Treatment of Idiopathic Membranous Nephropathy (MENTOR).

Science.gov (United States)

Fervenza, Fernando C; Canetta, Pietro A; Barbour, Sean J; Lafayette, Richard A; Rovin, Brad H; Aslam, Nabeel; Hladunewich, Michelle A; Irazabal, Maria V; Sethi, Sanjeev; Gipson, Debbie S; Reich, Heather N; Brenchley, Paul; Kretzler, Matthias; Radhakrishnan, Jai; Hebert, Lee A; Gipson, Patrick E; Thomas, Leslie F; McCarthy, Ellen T; Appel, Gerald B; Jefferson, J Ashley; Eirin, Alfonso; Lieske, John C; Hogan, Marie C; Greene, Eddie L; Dillon, John J; Leung, Nelson; Sedor, John R; Rizk, Dana V; Blumenthal, Samuel S; Lasic, Lada B; Juncos, Luis A; Green, Dollie F; Simon, James; Sussman, Amy N; Philibert, David; Cattran, Daniel C

2015-01-01

Idiopathic membranous nephropathy remains the leading cause of nephrotic syndrome in Caucasian adults. Immunosuppressive therapy with cyclosporine (CSA) is often successful in reducing proteinuria, but its use is associated with a high relapse rate. Rituximab, a monoclonal antibody that specifically targets CD20 on the surface of B-cells, is effective in achieving a complete remission of proteinuria in patients with idiopathic membranous nephropathy. However, whether rituximab is as effective as CSA in inducing and maintaining complete or partial remission of proteinuria in these patients is unknown. The membranous nephropathy trial of rituximab (MENTOR) hypothesizes that B-cell targeting with rituximab is non-inferior to CSA in inducing long-term remission of proteinuria. Patients with idiopathic membranous nephropathy, proteinuria ≥5 g/24 h, and a minimum of 3 months of Angiotensin-II blockade will be randomized into a 12-month treatment period with i.v. rituximab, 1,000 mg (2 infusions, 14 days apart; repeated at 6 months if a substantial reduction in proteinuria (equal to or >25%) is seen at 6 months) or oral CSA 3.5-5 mg/kg/day for 6 months (continued for another 6 months if a substantial reduction in proteinuria (equal to or >25%) is seen at 6 months). The efficacy of treatment will be assessed by the remission status (based on changes in proteinuria) at 24 months from randomization. Patient safety will be assessed via collection of adverse event data and evaluation of pre- and posttreatment laboratory data. At the 6-month post-randomization visit, patients who have been randomized to either CSA or rituximab but who do not have a reduction in proteinuria ≥25% (confirmed on repeat measurements within 2 weeks) will be considered treatment failures and exit the study. This study will test for the first time whether treatment with rituximab is non-inferior to CSA in inducing long-term remission (complete or partial) of proteinuria in patients with idiopathic

Obstructive Sleep Apnea

Medline Plus

Full Text Available ... can also invite bacteria that lead to gum disease. Click here to find out more. Obstructive Sleep Apnea (OSA) Download Download the ebook for further information Obstructive sleep ... high blood pressure, heart disease and decreased libido. In addition, OSA causes daytime ...

Impaired EphA4 signaling leads to congenital hydronephrosis, renal injury, and hypertension

DEFF Research Database (Denmark)

Sällström, Johan; Peuckert, Christiane; Gao, Xiang

2013-01-01

Experimental hydronephrosis induced by partial ureteral obstruction at 3 wk of age causes hypertension and renal impairment in adult rats and mice. Signaling by Ephrin receptors (Eph) and their ligands (ephrins) importantly regulates embryonic development. Genetically modified mice, where...... the cytoplasmic domain of the EphA4 receptor has been substituted by enhanced green fluorescent protein (EphA4(gf/gf)), develop spontaneous hydronephrosis and provide a model for further studies of the disorder. The present study aimed to determine if animals with congenital hydronephrosis develop hypertension...... and renal injuries, similar to that of experimental hydronephrosis. Ultrasound and Doppler techniques were used to visualize renal impairment in the adult mice. Telemetric blood pressure measurements were performed in EphA4(gf/gf) mice and littermate controls (EphA4(+/+)) during normal (0.7% NaCl)- and high...

A Randomized, Controlled Trial of Rituximab in IgA Nephropathy with Proteinuria and Renal Dysfunction.

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