WordNet

wipe with a sponge, so as to clean or moisten
a porous mass of interlacing fibers that forms the internal skeleton of various marine animals and usable to absorb water or any porous rubber or cellulose product similarly used
primitive multicellular marine animal whose porous body is supported by a fibrous skeletal framework; usually occurs in sessile colonies (同)poriferan, parazoan
erase with a sponge; as of words on a blackboard
gather sponges, in the ocean
soak up with a sponge
of or relating to the medulla of any body part
containing or consisting of or resembling bone marrow
of or relating to the medulla oblongata
either of two bean-shaped excretory organs that filter wastes (especially urea) from the blood and excrete them and water in urine; "urine passes out of the kidney through ureters to the bladder"
the inner part of an organ or structure in plant or animal

PrepTutorEJDIC

〈C〉『海綿動物』 / 〈C〉〈U〉『海綿』,スポンジ;(一般に)海綿(スポンジ)状の物 / 〈C〉《比喩(ひゆ)的に》(海綿のように)何かをふんだんに吸収する人(物) / 〈C〉《話》いそうろう,食客 / 〈C〉〈U〉(外科手術用の)ガーゼ帯 / …‘を'海綿(スポンジ)で洗う(ぬらす,ふく) / 〈液体〉‘を'海綿(スポンジなど)で吸い取る《+『up』+『名,』+『名』+『up』》 / 《話》…‘を'せびり取る;…‘に'せびる / 〈海綿などが〉液体を吸収する / 《話》(人に)たかる《+『on』(『upon, off』)+『名』〈人〉》
腎臓;(食品としての)羊(豚など)の腎臓 / 《文》気質,性質,たち(nature)
〈U〉骨髄 / =medulla oblongata

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/07/19 14:04:04」(JST)

wiki en

Medullary Sponge Kidney (also known as Cacchi–Ricci disease) is a congenital disorder of the kidneys characterized by cystic dilatation of the collecting tubules in one or both kidneys. Individuals with medullary sponge kidney are at increased risk for kidney stones and urinary tract infection. Patients with MSK typically pass twice as many stones per year as do other stone formers without MSK. While described as a "benign" disorder with a low morbidity rate, as many as 10% of patients with MSK have an increased risk of morbidity associated with frequent stones and UTIs. While some patients report increased chronic kidney pain, the source of the pain, when a UTI or blockage is not present, is unclear at this time. Renal colic (flank and back pain) is present in 55% of patients. Women with MSK experience more stones, UTIs and complications than men. MSK was previously believed not to be hereditary but there is more evidence coming forth that may indicate otherwise.^[1]^[2]

Etiology

In recent studies, insight has been obtained on the genetic basis of this disease, supporting the hypothesis that MSK is due to a disruption at the 'ureteric bud-metanephric mesenchyme' interface. This explains why so many tubular defects coexist in this disease, and particularly a distal tubular acidification defect of which the highly prevalent metabolic bone disease is one very important consequence. In addition to the typical clinical phenotype of recurrent stone disease, other clinical profiles have now been recognized, that is, an indolent, almost asymptomatic MSK, and a rare form characterized by intractable, excruciating pain.^[3] It was previously believed that most cases of Medullary Sponge Kidney were sporadic however, recent studies show familial clustering of MSK is common, and has an autosomal dominant inheritance, a reduced penetrance, and variable expressivity.^[4]^[5] Other theories suggest that dilatation of a collecting duct may occur, caused by occlusion by uric acid during fetal life or resulting from tubular obstruction due to calcium oxalate calculi secondary to infantile hypercalciuria.^[6]

A rare, autosomal recessive form is associated with Caroli disease.^[6]

Epidemiology

In the general population, the frequency of medullary sponge kidney disease is reported to be 0.02–0.005%; that is, 1 in 5000 to 1 in 20,000. The frequency of medullary sponge kidney has been reported by various authors to be 12 – 21% in patients with kidney stones (nephrolithiasis).^[7] The disease is bilateral in 70% of cases.

Presentation

Most cases are asymptomatic or are discovered during investigation of hematuria. Symptomatic patients typically present as middle aged adults with renal colic, nephrolithiasis, nephrocalcinosis and/or recurrent urinary tract infections; however, MSK also may affect children very rarely. In addition to the typical clinical phenotype of recurrent stone disease, other clinical profiles have now been recognized, that is, an indolent, almost asymptomatic MSK, and a rare form characterized by intractable pain.^[8]

Diagnosis

Classically, MSK is seen as hyperdense papillae with clusters of small stones on renal ultrasonography or abdominal x-ray. The irregular (ectatic) collecting ducts often seen in MSK, which are sometimes described as having a "paintbrush-like" appearance, are best seen on intravenous urography. However, IV urography has been largely replaced by contrast-enhanced, high-resolution helical CT with digital reconstruction.^[9]

Treatment

Often, aggressive treatment is unnecessary for people with disease that does not cause any symptoms (asymptomatic).^[9] In such cases, treatment may consist of maintaining adequate fluid intake, with the goal of decreasing the risk of developing kidney stones (nephrolithiasis).^[9] Cases of recurrent kidney stone formation may warrant evaluation for possible underlying metabolic abnormalities.^[9]

In patients with low levels of citrate in the urine (hypocitraturia) and incomplete distal renal tubular acidosis, treatment with potassium citrate helps prevent the formation of new kidney stones.^[9] Urinary tract infections, when they occur, should also be treated.^[9]

Patients with the more rare form of MSK marked by chronic pain typically require pain management. Non-obstructing stones in MSK can be associated with significant and chronic pain even if they're not passing. The pain in this situation can be constant. It is not certain what causes this pain but researchers have proposed that the small numerous stones seen in MSK may cause obstruction of the small tubules and collecting ducts in the kidney which could lead to the pain. This pain can often be debilitating and treatment is challenging. Narcotic medication even with large quantities is sometimes not adequate. Some success with pain control has been reported using laser lithotripsy (called “ureteroscopic laser papillotomy”).^[10]

Complications

Complications associated with medullary sponge kidney include the following:

Renal stones^[6]
Urinary tract infection (UTI)^[6]
Hematuria^[6]
Distal renal tubular acidosis (Type 1 RTA)^[6]
Renal insufficiency (rarely)^[6]
Marked chronic pain^[6]
Chronic kidney failure^[6]

Support & Awareness

Many patients with MSK find support in online support groups.

MSK awareness colors are purple, teal and green.

MSK Ribbon

References

^ "Medullary Sponge Kidney". Retrieved 22 August 2012.
^ "Evidence for inheritance of medullary sponge kidney". Retrieved 15 February 2013.
^ Gambaro, Giovanni; Danza, Francesco M.; Fabris, Antonia (July 2013). "Medullary sponge kidney". Current Opinion in Nephrology and Hypertension 22 (4): 421–426. doi:10.1097/MNH.0b013e3283622b86.
^ Fabris, Antonia; Lupo, Antonio; Ferraro, Pietro M; Anglani, Franca; Pei, York; Danza, Francesco M; Gambaro, Giovanni. "Familial clustering of medullary sponge kidney is autosomal dominant with reduced penetrance and variable expressivity". Kidney International 83 (2): 272–277. doi:10.1038/ki.2012.378.
^ Goldfarb, David S (February 2013). "Evidence for inheritance of medullary sponge kidney". Kidney International 83 (2): 193–196. doi:10.1038/ki.2012.417.
^ ^a ^b ^c ^d ^e ^f ^g ^h ⁱ Ghosh, Amit K. "Medullary Sponge Kidney". Medscape Reference. Retrieved 1 January 2013.
^ Ginalski, JM; Portmann L; Jaeger P (1990). "Does medullary sponge kidney cause nephrolithiasis?" (PDF). American Journal of Roentgenology 155 (2): 299–302. doi:10.2214/ajr.155.2.2115256. PMID 2115256.
^ "Medullary Sponge Kidney".
^ ^a ^b ^c ^d ^e ^f Salant DJ]; Gordon CE (2012). "Chapter 284. Polycystic Kidney Disease and Other Inherited Tubular Disorders". Harrison's Principles of Internal Medicine. (18th ed.). New York: McGraw-Hill. ISBN 978-0071748896.
^ "What is Medullary Sponge Kidney?". Retrieved 21 May 2014.

UpToDate Contents

全文を閲覧するには購読必要です。 To read the full text you will need to subscribe.

1. 海綿腎 medullary sponge kidney
2. 腎石灰沈着症 nephrocalcinosis
3. 成人におけるカルシウム結石の危険因子 risk factors for calcium stones in adults
4. ベックウィズ・ヴィーデマン症候群 beckwith wiedemann syndrome
5. 鎮痛剤性腎症の症状および診断 manifestations and diagnosis of analgesic nephropathy

English Journal

Biopsy proven medullary sponge kidney: clinical findings, histopathology, and role of osteogenesis in stone and plaque formation.

Evan AP1, Worcester EM, Williams JC Jr, Sommer AJ, Lingeman JE, Phillips CL, Coe FL.
Anatomical record (Hoboken, N.J. : 2007).Anat Rec (Hoboken).2015 May;298(5):865-77. doi: 10.1002/ar.23105. Epub 2015 Feb 17.
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 recognizabl
PMID 25615853

Calcium phosphate stone morphology can reliably predict distal renal tubular acidosis.

Dessombz A1, Letavernier E2, Haymann JP2, Bazin D3, Daudon M4.
The Journal of urology.J Urol.2015 May;193(5):1564-9. doi: 10.1016/j.juro.2014.12.017. Epub 2014 Dec 10.
PURPOSE: 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 distu
PMID 25498572

Spontaneous calcification process in primary renal cells from a medullary sponge kidney patient harbouring a GDNF mutation.

Mezzabotta F1, Cristofaro R, Ceol M, Del Prete D, Priante G, Familiari A, Fabris A, D'Angelo A, Gambaro G, Anglani F.
Journal of cellular and molecular medicine.J Cell Mol Med.2015 Apr;19(4):889-902. doi: 10.1111/jcmm.12514. Epub 2015 Feb 18.
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-derive
PMID 25692823

Japanese Journal

海綿腎

鈴木清澄,権寧博,大塚博雅,林悠太,蓮見禎行,須崎愛,山中弥太郎,矢内充,高橋悟,相馬正義
日大醫學雜誌 71(6), 365-366, 2012-12-01
NAID 10031146214

MR Imaging of Focal Medullary Sponge Kidney : Case Report

HIDA Tomoyuki,NISHIE Akihiro,ASAYAMA Yoshiki,ISHIGAMI Kousei,FUJITA Nobuhiro,INOKUCHI Junichi,NAITO Seiji,ANDO Sadamu,HONDA Hiroshi
Magnetic resonance in medical sciences : MRMS 11(1), 65-69, 2012-03-01
… We present a case of focal medullary sponge kidney (MSK) that mimicked a renal tumor. …
NAID 10030284856

経皮的に切除した海綿腎に合併した腎孟Fibroepithelial polypの1例

田代和也,岩室紳也,波多野孝,簗田周一,古田昭,尾山博則,滝沢明利
泌尿器科紀要 45(8), 535-537, 1999-08
… We report a case of fibroepithelial polyp in the renal pelvis complicated with medullary sponge kidney successfully treated by percutaneous resection. … The patient had recurrent bilateral renal stones because of medullary sponge kidney. …
NAID 120002141785

Related Pictures

★リンクテーブル★

リンク元	「慢性間質性腎炎」「海綿腎」「renal insufficiency」「MSK」「髄質海綿腎」
関連記事	「sponge」「medullary」「medulla」「kidney」

「慢性間質性腎炎」

Medullary sponge kidney
	Medullary spongeas seen on an intravenous pyelogram
Classification and external resources
ICD-10	Q61.5
ICD-9-CM	753.17
DiseasesDB	7915
eMedicine	article/379323
MeSH	D007691