Weeping sponge kidney: an unusual phenomenon that should be considered in cases of severe renal haemorrhage.

AIM: To describe the clinical presentation, imaging evaluation, endovascular management, and outcomes of multifocal renal capsular haemorrhage, "weeping sponge kidney", and to identify associated risk factors and the pathophysiological mechanism behind this condition. MATERIALS AND METHODS: This is a case series in which clinical information for each of the cases was collected retrospectively from electronic patient notes as well as the radiology information and picture archiving and communication systems. RESULTS: Four consecutive cases were included in the series. All of the cases were treated successfully with embolisation. Three of the four patients had chronic renal failure with renal atrophy, which are patient factors that appear to be associated with multifocal renal capsular haemorrhage. Based on the procedural findings and the published literature, a pathophysiological mechanism is described to explain this condition and the relevance of the collateral arterial supply to the kidney in such cases is discussed. CONCLUSION: Small subcapsular haematomas are usually self-limiting but in patients with renal failure, there may be an increased risk of developing a weeping sponge kidney, which can be life-threatening. The endovascular treatment for multifocal haemorrhage differs from that for a single bleeding point, especially if preservation of renal function is not a priority.

Detailed Nephro-urological Management of a Case of Medullary Sponge Kidney with Distal Renal Tubular Acidosis and Obstructive Uropathy.

Medullary sponge kidney (MSK) is a rare renal malformation characterized by precalyceal tubular ectasia of the renal collecting ducts that clinically manifests as recurrent renal stones, distal renal tubular acidosis (dRTA), osteoporosis, and nephrocalcinosis. In this case report, we present the case of a woman with a severe form of MSK associated with dRTA. She had extensive nephrocalcinosis and obstructive uropathy caused by a large upper ureteric stone in the left kidney. The stone was disintegrated by flexible ureteroscopic laser lithotripsy. Her initial biochemical derangements were identified and corrected with a Polycitra-K solution and hydrochlorothiazide, leading to reduced stone load and osteopenia 1 year later.

[Effects of percutaneous nephrolithotomy in the treatment of medullary sponge kidney with calculi].

Objective: To evaluate the effects of percutaneous nephrolithotomy (PNL) in the treatment of medullary sponge kidney with calculi. Methods: 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 (42.1±13.2) years, the mean stone burden was (3.9±1.8) cm. The control group included 36 males and 41 females with a mean age of (45.3±13.0) years, the mean stone burden was (3.6±1.5) cm. The numbers of tracts, the time of operation, the drop of hemoglobin, the change of creatine, the time of hospitalization, the stone free rate and major complications were compared between the two groups. The measurement data and numeration data were compared with t test and χ(2) test. Results: There were no significant differences in sex, age, preoperative urinary tract infection, stone type, and stone burden between the two groups (all P>0.05). The proportion of bilateral renal calculus in MSK group was higher (18.2% vs. 0, χ(2)=15.400, P=0.000). There were 159 percutaneous channels were established in MSK group while 90 percutaneous channels were established in control group. Compared with the control group, the operation time ((88.1±37.5) minutes vs. (68.5±30.1) minutes, t=3.543, P=0.000) and hospitalization time ((15.1±8.3) days vs. (10.1±3.6) days, t=4.816, P=0.000) were longer, the creatinine level increased ((101.2±62.6) µmol/L vs. (71.3±23.6) µmol/L, t=3.777, P=0.000), the rate of stone free decreased (27.5% vs. 83.1%, χ(2)=51.840, P=0.000) and the rate of complications increased (29.9% vs. 11.7%, χ(2)=8.114, P=0.004) in MSK group. There was no statistically difference in hemoglobin drop ((12.5±13.2) g/L vs. (13.0±10.9) g/L, t=-0.260, P=0.795). Conclusions: Using PNL for patients of MSK with calculi has a lower stone free rate and a higher complications. It is an effective method for patients of MSK with large and complex calculi.

Medullary sponge kidney.

PURPOSE OF REVIEW: 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. RECENT FINDINGS: 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. SUMMARY: Findings suggest the need for a more comprehensive clinical characterization of MSK patients. The genetic grounds for the condition warrant further investigation, and reliable methods are needed to diagnose MSK.

[A woman with urinary tract infections and flank pain]. / Een vrouw met urineweginfecties en flankpijn.

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.

Medullary sponge kidney: causes and treatments.

Medullary sponge kidney is a little known and little understood disease. A patient with a medullary sponge kidney may undergo decades of suffering in the form of infections and pain before any diagnosis is even made. When a diagnosis is made, it is more than likely to be an incidental finding from a test for another problem. However, on diagnosis there are a number of options available to treat this condition. These include non-invasive treatments such as drug therapy, diet or extracorporeal shock wave lithotripsy, to invasive therapies such as percutaneous nephrolithotomy, ureteroscopy and more advanced surgery. The aim of this article is to highlight this little known condition, outline the effects, and discuss the treatment options available to patients.

Coexistence of medullary sponge kidney and ulcerative colitis in the same patient: long-term follow-up.

We describe a female patient with ulcerative colitis since the age of 17, who was accidentally diagnosed as having medullary sponge kidney 3 years after the establishment of diagnosis of inflammatory bowel disease. The diagnosis of renal disease was based on the typical appearance of both kidneys on abdominal ultrasound examination and on IV pyelography findings. All other well-known causes of medullary sponge kidney were excluded on the basis of the relevant laboratory investigation. So far, the patient experienced only one episode of urinary infection but no renal colic. Since the time of diagnosis of ulcerative colitis her renal function tests are perfectly normal. She is under maintenance treatment with mesalazine. The benign nature of the situation was explained to her. She was advised to drink at least one and a half litter of water daily, in order to reduce the risk of nephrolithiasis. The combination of the two disorders in our patient is probably the result of a chance. However, taking into account the potentially dangerous long-term results of medullary sponge kidney, we suggest that patients with ulcerative colitis must have a careful ultrasound examination of both kidneys at least at the time of diagnosis of the bowel disease, in order to exclude the possibility of medullary sponge kidney, as conservative measures could result in avoidance of potentially dangerous complications, such as renal stones and urinary infections.

Medullary sponge kidney and urinary calculi: aeromedical concerns.

Medullary sponge kidney (MSK) is a benign disorder associated with a lifetime risk of renal stones in 60% of patients. Patients frequently have episodic painless hematuria, but are often otherwise asymptomatic unless renal calculi or infections complicate the disease. Nephrolithiasis is a relative, but frequently enforced, contraindication to space or other high-performance flight. Two case reports of asymptomatic NASA flight crew with MSK and three cases of United States Air Force (USAF) military aviators diagnosed with MSK are reviewed. All cases resulted in waiver and return to flight status after treatment and a vigorous followup and prophylaxis protocol. MSK in aviation and spaceflight necessitates case-by-case evaluation and treatment to rule out other potential confounding factors that might also contribute to stone formation and in order to requalify the aviator for flight duties.

beta(2)-Microglobulin amyloidosis caused spinal cord compression in a long-term haemodialysis patient.

STUDY DESIGN: A case report of cervical myelopathy caused by epidural beta (2)-microglobulin (beta2m) amyloid deposits in a 50-year-old woman with haemodialysis treatment. OBJECTIVE: Long-term haemodialysis in patients with end-stage renal disease leads to several complications based on beta2m deposits, which can affect, in the cervical spine, the intervertebral disk, and in rare cases, they may compress the spinal cord and nerves. The objective of this report is to describe the clinical and radiological follow-up preceding the indispensable surgical excision of an amyloid mass in a 50-year-old woman with haemodialysis treatment. Long-term postoperative cervicalgia owing to subcondylian bone cyst-associated atlanto-occipital instability is also described and discussed. SETTING: Department of Neurosurgery A, Hop Pellegrin, Bordeaux, France. CASE REPORT: We present a clinical case of a patient with spinal cord compression. The patient was treated by surgical excision of an amyloid mass subsequent to a C2-C3 laminectomy. The patient experienced clinical improvement with a regression of all of her neurological symptoms. Histological findings confirm the diagnosis of beta2m amyloid deposition. However, 5 years after surgery the subcondylian bone cysts were still observed and atlanto-occipital instability required her to wear a minerva. CONCLUSION: Our case report confirms that surgical excision of beta2m epidural deposits is necessary and relevant when neurological prognosis is discussed, and that pain is still the major symptom of disease evolution. The use of high-flux synthetic membranes could decrease the beta2m blood level and early renal graft is the only method to prevent such complications.

[Kidney sponge--treatment--case report]. / Nerka gabczasta--postepowanie terapeutyczne--opis przypadku.

Medullary sponge kidney (MSK) is a benign asymptomatic developmental anomaly of the kidney mostly seen in adult females. Typical for this morphological abnormality is dilation of the collecting ducts. Intravenosus pyelogram shows accumulation of contrast in dilated ducts giving to the papillae the appearance of a bouquet flowers, characteristic for MSK. Urinary tract infections, nephrolithiasis, hematuria and hyperkalciuria are the common complications of the kidney sponge. We present a case of a 29-year-old female who suffers from recurrent urinary tract infection, nephrolithiasis and distal tubular acidosis. This kind of tubular acidosis is specific for kidney sponge clinical picture.

Management of lithiasis in medullary sponge kidneys.

Twenty-four patients with medullary sponge kidneys (MSKs), diagnosed on a recent IVU, and renal lithiasis were treated by extracorporeal shock wave lithotripsy (ESWL). A detailed history regarding frequency of renal colic and urinary tract infection (UTI) was recorded and compared to the post-ESWL frequency of the symptoms. Our results show that the stone clearance rate is similar to that of non-MSK patients but there is a great reduction in the frequency of renal colic and UTI.

Hipercalciuria: Presentación clínica, laboratorio y tratamiento / Hypercalciuria: clinic presentation, laboratory and treatment

El propósito del presente trabajo es el de reportar la experiencia de la consulto del servicio de nefrología del Hospital "J.M delos Ríos" en el diagnóstico y manejo de esta entidad, desde enero de 1991 hasta diciembre de 1991, de un total de 4.800 pacientes evaluados, se obtuvieron 653 pacientes con una relación calcio/creatinina en orina mayor de 0,11. El antecedente familiar más importante que se encontró fue litiasis renal (39,5 por ciento). Las formas de presentación clínica más frecuentes fueron: Infección Urinaria (56,6por ciento), dolor abdominal (56,8por ciento), hematuria microscopica (43 por ciento), hematuria macroscopica (31,8 por ciento) y retardo del crecimiento (12,9 por ciento). Dentro de las patologías renales asociadas predominaron las siguientes: Litiasis Renal (39,8 por ciento) y riñon de esponja (29,5 por ciento). El manejo médico incluyó: Regulación dietética, tiazidas, citrato de potasio o bicarbonato de sodio, segun las necesidades de cada caso

Prophylactic role of extracorporeal shock wave lithotripsy in the management of nephrocalcinosis.

Electromagnetic extracorporeal shock wave lithotripsy (ESWL) was performed with a standard Siemens Lithostar on parenchymal calcifications in 10 medullary sponge kidneys in order to evaluate the eventual benefit of preventive lithotripsy. The results of shock wave lithotripsy on precaliceal calcifications were disappointing and have been related to the lack of expansion during shock wave interaction and to the impaired drainage of particles. Three patients also had large impacted ureteric stones and in such cases ESWL remains the treatment of choice.

The use of extracorporeal shock wave lithotripsy for medullary sponge kidneys.

A number of patients with medullary sponge kidney recurrently form and pass stones with the risk of developing an obstructive nephropathy. These patients may benefit from extracorporeal shock wave lithotripsy to the medullary collections, as this appears to reduce the frequency of symptomatic stone passage.

Combined treatment of medullary sponge kidney by EDTA potassium citrate and extracorporeal shock wave lithotripsy.

A case of successful renal calculus dissolution by the combined treatment which consists of irrigation with ethylenediaminetetraacetic acid (EDTA), potassium citrate, and extracorporeal shock-wave lithotripsy (ESWL) is described here. Renal irrigation via nephrostomy, which was the main treatment, was attempted on a 34-year-old Japanese male who had bilateral nephrocalcinosis caused by type 1 renal tubular acidosis associated with an impacted calculus in the right ureter. Finally, most of the calculi have been dissolved within 1 year.