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'Medullary Sponge Kidney' (596)


Jan
2017

Over 1%-15% of the population worldwide is affected by nephrolithiasis, which remains the most common and costly disease that urologists manage today. Identification of at-risk individuals remains a theoretical and technological challenge. The search for monogenic causes of stone disease has been largely unfruitful and a technological challenge; however, several candidate genes have been implicated in the development of nephrolithiasis.

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Oct
2017

To evaluate the effects of percutaneous nephrolithotomy (PNL) in the treatment of medullary sponge kidney with calculi.A total of 77 patients (91 renal units) of medullary sponge kidney with calculi (MSK group) and 77 patients (77 renal units) with common kidney stone (control group) received PNL at Department of Urology in Peking University People's Hospital from September 2006 to February 2016 were analyzed retrospectively. The MSK group included 33 males and 44 females with a mean age of (42.

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Feb
2017

Urinary proteomics is a promising tool for biomarker investigation, particularly in complex kidney diseases. Fabris and colleagues report that urinary laminin subunit alpha-2 is a potential diagnostic marker of medullary sponge kidney (MSK) disease by using a label-free quantitative proteomics platform and a clinically compatible enzyme-linked immunosorbent assay. The neglected issue of stone pathogenesis was also evidenced.

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Dec
2016

Patients with medullary sponge kidney (MSK) commonly encounter recurrent nephrolithiasis. The existing knowledge on safety of donors with MSK has not been studied.
We conducted a retrospective cohort study at a tertiary referral hospital to assess the outcomes of living kidney donors with MSK.

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Feb
2017

Medullary sponge kidney (MSK) disease, a rare kidney malformation featuring recurrent renal stones and nephrocalcinosis, continues to be diagnosed using expensive and time-consuming clinical/instrumental tests (mainly urography). Currently, no molecular diagnostic biomarkers are available. To identify such we employed a proteomic-based research strategy utilizing urine from 22 patients with MSK and 22 patients affected by idiopathic calcium nephrolithiasis (ICN) as controls.

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Aug
2017

Medullary sponge kidney (MSK) is a congenital renal disorder. Its association with several developmental abnormalities in other organs hints at the likelihood of some shared step(s) in the embryogenesis of the kidney and other organs. It has been suggested that the REarranged during Transfection (RET) proto-oncogene and the Glial cell line-Derived Neurotrophic Factor (GDNF) gene are defective in patients with MSK, and both RET and GDNF are known to have a role in the development of the central nervous system, heart, and craniofacial skeleton.

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Dec
2016

Characteristics of renal papillae in kidney stone formers.

Minerva Urol Nefrol 2016 Dec 21;68(6):496-515. Epub 2016 Jul 21.
Tracy P Marien, Nicole L Miller
The mechanism of kidney stone formation is not well understood. In order to better understand the pathophysiology for specific kidney stone compositions and systemic diseases associated with kidney stones, endoscopic papillary mapping studies with concurrent biopsies have been conducted. This review will summarize the findings of these studies and proposed mechanisms for thirteen disease processes associated with kidney stones.

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Jan
2016

Diseases of the kidney often have their names shortened, creating an arcane set of acronyms which can be confusing to both radiologists and clinicians. This review of renal pathology aims to explain some of the most commonly used acronyms within the field. For each entity, a summary of the clinical features, pathophysiology, and radiological findings is included to aid in the understanding and differentiation of these entities.

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Oct
2016

The purpose of this study was to review the safety and efficacy of the minimally invasive percutaneous nephrolithotomy in the treatment of medullary sponge kidney patients with complex renal calculi. Sixteen medullary sponge kidney patients with complex renal calculi underwent minimally invasive percutaneous nephrolithotomy procedures in our center were entered into this retrospective study. The data analyzed included patients' demographics, stone burden, operative time, operative blood loss, length of hospital stay, complications according to the modified Clavien system, and stone-free rate.

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Jul
2016

[Abnormal kidneys].

Rev Med Interne 2016 Jul 26;37(7):507-8. Epub 2015 Oct 26.
I En-Nafaa, T Ziadi, T Africha, B Zainoune, N Aziz, S M Drissi

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Jan
2016

Role of Osteogenesis in the Formation of Randall's Plaques.

Anat Rec (Hoboken) 2016 Jan 30;299(1):5-7. Epub 2015 Oct 30.
Saeed R Khan, Giovanni Gambaro

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Sep
2015

To evaluate the safety, efficacy, and durability of ureteroscopic laser papillotomy for the treatment of radiographically visible intraductal papillary calculi and/or free collecting system calculi associated with medullary sponge kidney (MSK).
The medical records of 25 MSK patients who underwent ureteroscopic laser papillotomy were reviewed at our institution from 2010 to 2013. The follow-up was made in our outpatient department.

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Nov
2015

Nephrocalcinosis is commonly present in primary hyperparathyroidism, distal renal tubular acidosis and medullary sponge kidney disease. To our knowledge it has not been studied in patients with calcium phosphate stones who do not have systemic disease.
We studied patients undergoing percutaneous nephrolithotomy who had calcium phosphate or calcium oxalate stones and did not have hyperparathyroidism, distal renal tubular acidosis or medullary sponge kidney disease.

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Apr
2015

Medullary nephrocalcinosis is a hallmark of medullary sponge kidney (MSK). We had the opportunity to study a spontaneous calcification process in vitro by utilizing the renal cells of a patient with MSK who was heterozygous for the c.-27 + 18G>A variant in the GDNF gene encoding glial cell-derived neurotrophic factor.

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May
2015

Medullary sponge kidney (MSK) is associated with recurrent stone formation, but the clinical phenotype is unclear because patients with other disorders may be incorrectly labeled MSK. We studied 12 patients with histologic findings pathognomonic of MSK. All patients had an endoscopically recognizable pattern of papillary malformation, which may be segmental or diffuse.

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Dec
1969

Percutaneous nephrolithotomy (PCNL) was mostly performed with fluoroscopy and/or ultrasonography. The safety and feasibility of PCNL performed totally under ultrasound are not clearly defined. Therefore, we introduce the 9-year experience of 8 025 ultrasound guided PCNL procedures from multiple centers in China performed by the same surgeon, to evaluate the feasibility and security of this technique.

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May
2015

Calcium stones represent 85% to 90% of all urinary calculi, including various crystalline compositions and etiological conditions. Calcium phosphate accounts for 10% to 15% of cases. These stones are mainly related to 3 groups of risk factors, including calcium or phosphate metabolism disturbance, renal acidification defects and urinary tract infection.

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Oct
2014

Nephrocalcinosis consists of the deposition of calcium salts in the renal parenchyma and is considered the mixed form when it involves the renal cortex and medulla. The main etiological agents of this condition are primary hyperparathyroidism, renal tubular acidosis, medullary sponge kidney, hyperoxaluria and taking certain drugs. These factors can lead to hypercalcemia and/or hypercalciuria, which can give rise to nephrocalcinosis.

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Jul
2014

The term hemihyperplasia refers to an enlargement of body parts beyond the normal asymmetry. Hemihyperplasia can be isolated or associated with various well-described malformation syndromes. Medullary sponge kidney (MSK) has been described with isolated and syndromic hemihyperplasia; the actual prevalence is not known The hemi hypertrophy can be so subtle that it may be easily overlooked.

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Dec
1969

Inherited renal diseases.

Curr Pediatr Rev 2014 ;10(2):95-100
Jocelyn C Leung
Genetic disorders of the kidney include cystic diseases, metabolic diseases and immune glomerulonephritis. Cystic diseases include autosomal dominant and recessive polycystic kidney disease (ADPKD, ARPKD, respectively). Neonates with enlarged, cystic kidneys should be evaluated for PKD.

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Dec
1969

The medullary sponge kidney is a malformation characterized by Bellini ducts' ectasias and often by nephrocalcinosis. Scientific literature demonstrates and emphasizes how the Rx-urography plays a primary role to achieve diagnostic certainty in cases of suspected MSK. Pathognomonic urographic aspects in cases of medullary sponge kidney are defined as "bouquet of flowers", "bunch of grapes" or "brush".

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Jan
2015

We assessed whether a combination of the fibrin tissue adhesive Tisseel® (human fibrinogen and thrombin) plus the hemostatic matrix FloSeal® (bovine derived gelatin matrix/human thrombin) could safely replace the conventional deep medullary suture without compromising outcomes.
Laparoscopic mid pole and one-third partial nephrectomy was performed on the right kidney of 12 female pigs. The only difference between the 2 groups of 6 pigs each was the use of a fibrin tissue adhesive plus hemostatic matrix combination in group 2 instead of the deep medullary running suture in control group 1.

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Dec
1969

The remarkable progresses of imagistic and interventional techniques that have been implemented during the last decades facilitated the diagnostic and allowed the treatment indication changes for numerous renal disorders. The purpose of the present lecture was to outline a data review concerning a renal anomaly first described one century ago as well as to evaluate the impact of endourologic technical progresses over the therapeutic management of the respective disease. The medullary sponge kidney (MSK) or Cacchi-Ricci disorder represents a disturbance in the renal development characterized by the cystic type dilation and diffuse precalyceal ducts ectasias.

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Apr
2014

Medullary sponge kidney on retrograde pyelography.

Chonnam Med J 2014 Apr 22;50(1):21-2. Epub 2014 Apr 22.
Tsung-Yi Huang, Jih-Pin Lin, Shu-Pin Huang
A woman aged 31 had recurrent urinary tract infection with bloody urine. A series image of medullary sponge kidney presented by intravenous urography (IVU) was detected dynamically by retrograde pyelography (RP). Other than ultrasonography and IVU, RP is also a reliable method to detect medullary sponge kidney.

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Dec
1969

The medullary sponge kidney is also known as Lenarduzzi's kidney or Cacchi and Ricci's disease from the first Italian authors who described its main features. A review of the scientific literature underlines particular rarity of the association of MSK with developmental abnormalities of the lower urinary tract and genital tract such as hypospadias and bilateral cryptorchidism. The work presented is the only one in the scientific literature that shows the association between the medullary sponge kidney and the testicular dysgenesis syndrome.

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Apr
2014

Since the publication of the latest recommendations of the Lithiasis Comity of the French Association of Urology (CLAFU) on the management of ureteral and renal stones, practices have evolved. This text provides an update for the treatment of kidney stones. It is especially the important advances in the field of laser-ureterorenoscopy that changed practices.

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Dec
1969

Medullary sponge kidney (MSK) is characterized by malformation of the terminal collecting ducts and is associated with an increased risk of nephrolithiasis, nephrocalcinosis, urinary tract infections, renal acidification defects, and reduced bone density. It has been historically diagnosed with intravenous pyelography (IVP), which is falling out of favor as an imaging modality. CT urography (CTU) performed with multidetector CT (MDCT) has been shown to create images of the renal collecting system with similar detail as IVP; however, its utility in diagnosing MSK has not been defined.

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Dec
1969

Distal renal tubular acidosis combined with medullary sponge kidney (MSK) is not uncommon in adults, but is rare in infants. We report a 13-month-old boy with MSK who had features of distal renal tubular acidosis (nephrocalcinosis, hypercalciuria, hypocitraturia) and failed to thrive. Renal ultrasound revealed bilateral increased medullary echogenicity and nephrocalcinosis.

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Jul
2013

Medullary sponge kidney.

Curr Opin Nephrol Hypertens 2013 Jul;22(4):421-6
Giovanni Gambaro, Francesco M Danza, Antonia Fabris
After it was first described in 1939, medullary sponge kidney (MSK) received relatively little attention. This was because it was believed to have a low prevalence and because it was considered a benign condition. Studies in recent years have been changing these convictions however, hence the present review.

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Jul
2013

Medullary sponge kidney and von Meyenburg complex.

Acta Clin Belg 2013 Jan-Feb;68(1):73-4
C-T Chao

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May
2013

Re: medullary sponge kidney: state of the art.

J Urol 2013 May 22;189(5):1770. Epub 2013 Feb 22.
Dean G Assimos

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Jan
2013

Rabson-Mendenhall Syndrome (RMS) is a rare form of severe insulin resistance due to a recessive mutation of the insulin receptor. Associated manifestations include facial dysmorphism, skin abnormalities, and renal anomalies.
We report a case of a 13 year old African female with RMS, severe insulin resistance, and a cluster of renal pathologies including nephromegaly, nephrolithiasis, hydronephrosis, and medullary sponge kidney.

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Feb
2013

Medullary sponge kidney (MSK) is associated with recurrent calcium stones. Gambaro's group evaluated the relatives of probands with MSK. When prior imaging was not available, they performed renal ultrasounds.

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May
2013

Medullary sponge kidney: state of the art.

Nephrol Dial Transplant 2013 May 9;28(5):1111-9. Epub 2012 Dec 9.
Antonia Fabris, Franca Anglani, Antonio Lupo, Giovanni Gambaro
Medullary sponge kidney (MSK) is a kidney malformation that generally manifests with nephrocalcinosis and recurrent renal stones; other signs may be renal acidification and concentration defects, and pre-calyceal duct ectasias. MSK is generally considered a sporadic disorder, but an apparently autosomal dominant inheritance has also been observed. As MSK reveals abnormalities in both the lower and the upper nephron and is often associated with urinary tract developmental anomalies, its pathogenesis should probably be sought in one of the numerous steps characterizing renal morphogenesis.

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Feb
2013

Medullary sponge kidney (MSK) is a renal malformation typically associated with nephrocalcinosis and recurrent calcium nephrolithiasis. Approximately 12% of recurrent stone formers have MSK, which is generally considered a sporadic disorder. Since its discovery, three pedigrees have been described in which an apparently autosomal dominant inheritance was suggested.

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Dec
1969

A 52-year-old woman presented with recurrent urinary tract infections and flank pain. Both an abdominal CT-scan and a plain abdominal X-ray showed bilateral nephrocalcinosis and a kidney stone in the left ureter. These findings are suggestive of medullary sponge kidneys.

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Dec
1969

Rabson-Mendenhall syndrome (RMS) is a genetic disorder characterized by severe insulin resistance and somatic characteristics. Recombinant insulin-like growth factor 1 (r-IGF-1) is used to treat RMS, as the IGF-1 and insulin receptors share homology. However, the effect of r-IGF-1 varies in patients and it is difficult to manage metabolic status appropriately in r-IGF-1 resistant cases.

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Apr
2012

Congenital hepatic fibrosis is an exceedingly rare disease in China, where only very few cases with sufficient evidences and clinical data have been reported up to now. Here we reported a young patient, onset of hematemesis and melena, who had striking portal hypertension but without liver function damage. Computer tomography scans showed hepatosplenomegaly, intra-hepatic bile ducts dilation, thickening portal vein and tortuous spleen vein, and medullary sponge kidney.

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