Calcium nephrolithiasis and distal tubular acidosis in type 1 glycogen storage disease

A 36-year-old man was admitted to hospital due to right flank pain as a result of ureteral stones. He had been followed up for type 1 glycogen storage disease since the age of 11 years. He had four episodes of spontaneous stone birth during the previous 2 years, and each stone was composed mainly of calcium oxalate. Intravenous pyelography showed right hydronephrosis due to ureteral stones and bilateral multiple renal stones. We carried out transurethral ureterolithotripsy (TUL) on the right ureteral stones. The composition was a mixture of calcium oxalate and calcium phosphate. Laboratory evaluation demonstrated the association of distal renal tubular acidosis (RTA). These observations suggest that hypocitraturia and distal RTA are strongly correlated to recurrence of calcium nephrolithiasis. The patient's serum uric acid and urinary citrate excretion levels normalized after allopurinol and potassium citrate administration.     

Screening renal stone formers for distal renal tubular acidosis

A group of 110 consecutive renal stone formers were screened for distal renal tubular acidosis (RTA) using morning fasting urinary pH (mfUpH) levels followed by a short ammonium chloride loading test in patients with levels above 6.0. In 14 patients (12.7%) a renal acidification defect was noted; 13 had incomplete and 1 had complete distal RTA. Distal RTA was found particularly in recurrent stone formers (17%), and especially in those with bilateral stone disease, where a distal renal tubular acidification defect was found in 50%. We have been unable to differentiate primary from secondary RTA in renal stone formers. Regardless of whether the acidification defect is primary or secondary to stone formation, however, all renal stone formers with distal RTA can expect to benefit from prophylactic alkaline therapy and it is recommended that the screening procedure, which is easy to use in daily clinical practice, is applied to all stone formers and not restricted to patients with recurrent stone disease.     

Clinical experience of extracorporeal shock wave lithotripsy for cystine calculi

Thirty patients (16 men and 14 women) with cystine urinary stones were treated by extracorporeal shock wave lithotripsy (Dormer HM-3) from December 1984 through October 1989. The average patient age was 35.2 years with a range of 14 to 59 years. Seventy per cent of these subjects had had previous open surgical operations for stones. The cases consisted of 7 ureteral stones and 37 renal stones, including 15 staghorn calculi. An average of 1.3 session of ESWL was carried out to treat ureteral stones. Thirty-seven renal units with renal stone required 96 sessions of lithotripsy (average 2.6 sessions per unit). Seven patients with ureteral stones required auxiliary procedures, i.e., one transurethral lithotripsy (TUL), two percutaneous nephrostomies (PNS) and one open surgery. Thirty-seven renal stones, including staghorn calculi was treated by ESWL and auxiliary treatment of 21 TUL procedures, one PNS, 16 PNL procedures and one chemical chemolysis. Successful fragmentation (residual debris less than or equal to 4 mm) was achieved in 85.7% of ureteral stones, 90.9% of renal stones and 73.3% of staghorn calculi. The stone free rates of patients with ureteral stones, renal stones and staghorn calculi were 71.4%, 50.0% and 53.5%, respectively, at 3 months after ESWL. No serious complications were seen in this series. Fever above 38.5 degrees C was the most common complications (13.5%). Ureteral perforation was encountered once in TUL procedures. Transfusion and selective arterial embolization were needed for one case treated by PNL procedures. Although cystine stone is harder to be fragmented by ESWL than other stone composition, ESWL and endourology may be effective and safe procedures for cystine stone patients.(ABSTRACT TRUNCATED AT 250 WORDS)     

Ureteral stricture after ESWL for ureteral calculi

BACKGROUND: There are many reports on the effects of ESWL, but few reports on the complications, especially remaining ureteral stricture after this treatment. Therefore we have retrospectively reviewed our cases to define the predisposing factors of this complication. METHODS: Since 1991 we have treated urolithiasis with ESWL using a Siemens Lithostar for the first therapy. We had 16 cases of ureteral stricture after this treatment. Ureteral stricture is the most common complication after ESWL treatment. To define the risk factor of the stricture we have compared 549 cases that were successfully treated between 1994 and 1996 without this complication. In these two groups we examined ages, sexes, chief complaints, size, position and components of the calculi, the degree of hydronephrosis, the frequency of ESWL, the presence of urinary tract infection, the duration of stone impaction and the after endourological treatment using multiple logistic regression analysis. RESULTS: Patients with the stone incidentally found and those with the UTI seemed to be more frequently associated with ureteral stricture, however there was not a significant difference. The hydronephrosis more than grade 3 (p = 0.025), the frequency of ESWL (p = 0.0325) and the after endourological treatment, especially TUL (p = 0.0184) were statistically significant among the other factors. The stricture occurred in 5 out of 29 patients with the hydronephrosis of grade 4 and 5 between 1994 and 1996. CONCLUSION: We should carefully treat patients with grade 3 or more hydronephrosis with ESWL. We should not repeatedly treat the patients with ESWL. We should take care of TUL treatment after ESWL.     

Prevention of recurrent calcium stone formation with potassium citrate therapy in patients with distal renal tubular acidosis

Distal renal tubular acidosis is a common cause of intractable calcium nephrolithiasis. We examined the effect of oral potassium citrate therapy in 9 patients with incomplete distal renal tubular acidosis diagnosed on the basis of an abnormal response to an oral ammonium chloride load. Patients were studied during a control phase and after 3 months of potassium citrate treatment (60 to 80 mEq. daily). Potassium citrate caused a significant increase in urinary pH and urinary citrate, and a decrease in urinary calcium. The urinary relative saturation ratio of calcium oxalate significantly decreased during treatment, while that of brushite did not change. Potassium citrate also was shown to inhibit new stone formation. During a mean treatment period of 34 months none of the 9 patients had new stones, although 39.3 plus or minus 79.7 (standard deviation) stones per patient formed during the 3 years preceding treatment. The results support the potential clinical advantage of potassium citrate therapy in patients with distal renal tubular acidosis and recurrent calcium nephrolithiasis.     

Two years clinical experiences with extracorporeal shock-wave lithotripsy and transurethral ureterolithotripsy for ureteral stones at Osaka City University Hospital

From July 1985 to June 1987, 303 patients with ureteral stones were treated by either extracorporeal shock-wave lithotripsy (ESWL) or transurethral ureterolithotripsy (TUL). The ureteral stones were classified into two groups, upper and lower ureteral stones. The upper ureteral stone was defined as a stone located above the pelvic brim in radiological examinations. ESWL was performed using a Dornier lithotriptor HM-3. For TUL, following the insertion of a guide wire and dilatation of the intramural ureter by ureteral bougie, a ureteroscope was introduced into the ureter. The success rate included both patients who became stone free and patients whose stones were disintegrated into less than 4 mm. The success rate of ESWL for upper ureteral stones was 90%, and 8.5% were treated subsequently by TUL. The success rate of TUL for upper ureteral stones was 42%, and the remaining required another session of TUL or another procedure, mainly ESWL. On the other hand, the success rate of TUL for lower ureteral stones was 71%, and the remainder also required another session of TUL or another procedure, mainly ESWL. The efficacy of TUL for stone street was comparably low by the evaluation done at 5 days after the procedure. However, almost all patients with stone street, which had developed after ESWL treatment, became stone free several weeks after TUL and insertion of a stent catheter. Major complications or side effects for ESWL were fever of more than 37.5 degrees C (7.5%) and pain attacks (8.9%).(ABSTRACT TRUNCATED AT 250 WORDS)     

Alkali action on the urinary crystallization of calcium salts: contrasting responses to sodium citrate and potassium citrate

Alkali therapy is used commonly to prevent recurrent stone formation in patients with distal renal tubular acidosis. We compared the effects of potassium citrate to those of sodium citrate in 6 well defined cases of incomplete distal renal tubular acidosis. The patients were studied during a control phase, during potassium citrate treatment (80 mEq. per day) and during sodium citrate treatment (80 mEq. per day) chosen in random order. Potassium citrate caused a decrease in urinary calcium and a significant increase in urinary citrate that resulted in a significant decrease in the urinary saturation of calcium oxalate. It did not alter the saturation of brushite and sodium urate. However, while sodium citrate also was able to increase the urinary citrate level, there was no decrease in the urinary calcium (owing to the increased sodium load). Thus, the urinary saturation of calcium oxalate did not decrease as much as with potassium citrate and the saturation of brushite increased significantly. Moreover, the urinary saturation of sodium urate increased significantly owing to the enhanced sodium excretion. The results suggest that potassium citrate therapy may retard the crystallization of calcium oxalate and may not cause calcium phosphate crystallization. In contrast, sodium citrate may have no effect or it sometimes may accentuate the crystallization of calcium salts. Thus, our study supports the potential clinical advantage of potassium citrate therapy over sodium alkali treatment in patients with incomplete distal renal tubular acidosis and recurrent calcium nephrolithiasis.     

Case of hypoparathyroidism, deafness and renal dysplasia (HDR) syndrome associated with nephrocalcinosis and distal renal tubular acidosis

Hypoparathyroidism, deafness and renal dysplasia (HDR) syndrome is an autosomal dominant disorder characterized by hypoparathyroidism, sensorineural deafness and renal dysplasia. Herein, we report a case of HDR syndrome associated with nephrocalcinosis and distal renal tubular acidosis. A 34-year-old woman was admitted to investigate recurrent stone formation and bilateral nephrocalcinosis. As a 3-year-old child, she had been diagnosed with HDR syndrome without chromosome evaluation. She had spontaneous stone passages on several occasions. On laboratory examination, serum calcium and intact parathyroid hormone at lower levels. Urinary citrate excretion was extremely low at 51.6 mg/day. On an ammonium chloride loading test, complete distal renal tubular acidosis was proved. To prevent the nephrocalcinosis from deteriorating, she was given potassium-sodium citrate. Since administration, she has not experienced spontaneous stone passage or renal colic. Nephrocalcinosis and recurrent urolithiasis will strongly affect renal prognosis in this case and we consider that citrate medication is an effective therapy in avoiding progress of her nephrocalcinosis.     

Renal tubular acidosis in recurrent renal stone formers

Renal tubular acidosis (RTA) is a well-known metabolic disturbance that may promote recurrent renal stone formation. However, its incidence, screening criteria and association with other lithogenic metabolic abnormalities are not established in recurrent nephrolithiasis. 10 of 50 consecutive recurrent renal stone formers had a persistent fasting morning urinary pH above 6.0 and/or a basal plasma bicarbonate concentration below 20.0 mM. Acid and alkaline loads disclosed RTA in 3 patients: 1 patient had incomplete type-1 distal RTA in addition to hyperoxaluria; a second patient showed complete type-2 proximal RTA, hyperoxaluria and renal hypercaliuria; and a third patient had incomplete proximal RTA without any other metabolic derangement. These results reinforce the importance of RTA as an isolated metabolic abnormality among recurrent renal stone formers. In addition, RTA appears to be more commonly associated with other lithogenic metabolic derangements than has been previously suspected. The extensive metabolic protocol used in this study provides a useful tool in the diagnosis and therapeutic considerations of recurrent nephrolithiasis.     

Endoscopic treatment for severe encrusted ureteral stent left in place for 3 years

A 52-year-old man had bilateral ureteral stents placed before treatment for ureteral and renal stones, but did not return for treatment and follow-up. Three years later, he complained of hematuria and vertigo. An abdominal X-ray revealed large renal and ureteral stones rising from and enveloping the stent. A bilateral percutaneous nephrostomy was placed. The right ureteral stent was easily removed with a cystoscope. The left ureteral stone was separated from the stent by ureteroscopic lithotripsy (TUL) and percutaneous nephroscopic lithotripsy (PNL). The left stent was torn off and difficult to remove because of encrustation. It was finally removed through an endoscopic procedure. Right PNL and extracorporeal shock wave lithotripsy (ESWL) were performed and all stones and stents were extracted. He was stone-free at 4 months.     

A case of nephrocalcinosis due to Sj?gren syndrome associated with renal tubular acidosis

A 39-year-old women was admitted to the hospital because of fever, lumbago and recurrent history of spontaneous stone discharge. An abdominal X-ray film demonstrated multiple calculi in the medullary positions of both kidneys and right multiple ureteral stones. Laboratory examinations showed hypergammaglobulinemia and the urine pH level was fixed around 7; nevertheless general metabolic acidosis existed. These laboratory data indicated nephrocalcinosis due to Sj?gren syndrome associated with renal tubular acidosis. Right multiple ureteral stones were removed by transurethral ureteroscopy. After starting alkali therapy, neither increased nor recurrent stone formation was recognized.     

Changes in the management of urolithiasis: endourologic treatment of calculi in the upper urinary tract

Three hundred seventy cases with calculi in upper urinary tract were treated by endourology, 210 cases were subjects of percutaneous nephrolithotripsy (PNL) and 160 cases of transurethral ureterolithotripsy (TUL), in Nara Medical University Hospital and 13 affiliated hospitals. Of the PNL cases, PNL was completed in 184 cases (87.6%) and not completed in 16 cases (12.4%). 168 cases (80.0%) had successful results by PNL alone, stone-free or stone fragments less than 5 mm, in spite of the stone location and size. No serious adverse effect was experienced throughout the study. However, changes like a scar formation or fibrosis around the nephrostomy tract were revealed as a late complication by excretory urography, computed tomography and renal scanning. Of the 160 TUL cases, TUL was successful in 132 cases (82.5%) and unsuccessful in 28 cases (17.5%). The results of TUL for the calculi above the upper iliac cresta were not favorable, success rate for stone 6-10 mm in diameter was 70.3% and that for stones larger than 11 mm 37.5%. However, the results of TUL for the calculi below the upper ileac cresta were favorable, the success rate for the 6-10 mm calculi was 91.5% and for the calculi larger than 11 mm 88.4%. Of 28 TUL failures, 7 cases underwent PNL and 21 cases received open surgery. Throughout our endourologic treatments, the most problematic stone was calculi impact in the ureter. The indication of endourology for the calculi in upper urinary tract is discussed with consideration of ESWL which is becoming the main stream of stone treatment.     

Two cases of ammonium acid urate urinary stones related to anorexia nervosa and laxative abuse

We report two cases of urolithiasis related to anorexia nervosa and laxative abuse. Case 1: A 21-year-old woman was referred to our hospital because of left flank pain. A left ureteral stone, 10 x 6 mm in size, was successfully fragmented by extracorporeal shock-wave lithotripsy (ESWL), but she experienced repetitive formation of bilateral urinary stones and double J stent encrustation which required 13 sessions of ESWL, one session of transurethral ureterolithotripsy and one session of cystolithotripsy over a period of 5 years. All stones were comprised of pure ammonium acid urate. It was later revealed that she was diagnosed with anorexia nervosa at 15 years old and had suffered from laxative abuse (bisacodyl, 300-500 mg/day) ever since. Case 2: A 18-year-old woman was referred to our hospital because of left lower abdominal pain. A left renal stone, 15 x 10 mm in size, was successfully fragmented by ESWL, but she had double J stent encrustation which was managed by cystolithotripsy. All stones were comprised of pure ammonium acid urate. She was later diagnosed with anorexia nervosa and it turned out that she had suffered from an eating disorder and laxative abuse (bisacodyl, 200 mg/day) since the age of 15 years. Both patients had marked decrease in urine volume, hyponatremia and hypokalemia. Anorexia nervosa and laxative abuse should be suspected whenever a woman has an ammonium acid urate stone in sterile urine because the treatment of these disorders is crucial to the prevention of repetitive formation of urinary stones.     

Extracorporeal shock wave lithotripsy for ureteral stones and cystine stones]

We report our experience with extracorporeal shock wave lithotripsy (ESWL) in the treatment of ureteral and cystine stones, which are known to be difficult to treat by this method. First, in order to determine the effectiveness of the ureteral catheter in the destruction of ureteral stones, we compared the clinical results of 121 patients treated without the catheter and 141 patients inserted with the catheter. There was no significant difference in the success rate between the two groups regardless of stone size, which indicates that the use of the ureteral catheter had no effect on the outcome of treatment. We then studied the clinical results of impacted ureteral stones which are especially difficult to destroy. Excretory urography was performed to non-invasively diagnose these stones, and those without visualization in the ureter below the stone were diagnosed as impacted stones and treated by ESWL without the ureteral catheter. Among the stones with a diameter of 1 to 2 cm, the success rate was significantly lower in impacted stones compared to non-impacted stone. These findings suggest that ESWL treatment without the ureteral catheter may be effective for ureteral stones with a diameter of less than 1 cm and non-impacted stones with a diameter of 1 to 2 cm, while combination therapy with other methods such as TUL may be better for other stones. We also performed ESWL on 6 patients with renal stones and 2 patients with ureteral stones which were cystine stones. Renal stones required an average 4.1 treatment with an average of 1,875 shocks per treatment, and ureteral stones required 1.5 treatment with an average of 1,833 shocks.(ABSTRACT TRUNCATED AT 250 WORDS)     

ESWL治疗复杂上尿路结石的体会

目的探讨ESWL治疗复杂上尿路结石的效果。方法总结自1993年以来行ESWL治疗的所有上尿路结石,对其中较大的肾、输尿管结石、肾鹿角形结石及多发性结石的碎石效果进行分析。结合文献对石街的防治和双“J”管的应用进行讨论。结果ESWL治疗上尿路结石总有效率96．4％。治愈率92．1％。较大肾结石有效率93．9％,治愈率84．8％,石街形成率63．6％。鹿角形结石治愈率40％。较大输尿管结石有效率96．6％,治愈率91．5％,石街形成率23．7％。单侧多发结石治愈率95．7％。双侧多发结石治愈率100％。结论ESEL治疗较大的上尿路结石及多发性结石均有很好的效果。对鹿角形结石应与其它治疗方法联合应用。应及时发现并治疗碎石后形成的石街,双“J”管的应用并非必要。     

Effects of prophylactic insertion of Double-J stents to decrease episodes of renal colic in patients with recurrent ureteral stones

PURPOSE: To evaluate the hypothesis that prophylactic insertion of Double-J stents after uncomplicated transurethral lithotripsy (TUL) can decrease the number of episodes of renal colic and their intensity in patients with recurrent ureteral stones (those with three or more episodes of stone formation). PATIENTS AND METHODS: During a prospective randomized clinical trial from May 1999 to January 2004, 195 patients with recurrent ureteral stones were included in our study; 94 had stents in place for 4 weeks, and 101 patients remained stentless (control group). A few patients in each group had residual stone disease. All patients were followed-up for a mean period of 24 months and questioned about the number and intensity of their episodes of renal colic, and were also evaluated for their rates of spontaneous stone passage. RESULTS: Spontaneous passage of stones was seen in 43 patients (45.7%; CI 95%, 35.7, 55.8) who underwent stenting, and 35 patients (34.7%; CI 95%, 25.4, 43.9) in the stentless group (P > 0.05). The number of episodes of renal colic was significantly lower in the stented group (P < 0.001). CONCLUSION: Insertion of Double-J stents for 4 weeks after uncomplicated TUL in patients with recurrent ureteral stones significantly decreases the number of episodes of ureteral colic, although it does not decrease the rate of stone formation.     

Clinical experiences of renal and upper ureter stone by extracorporeal shock wave lithotripsy (ESWL). II: 3-month follow-up of cases treated with ESWL

Fifty three out of 60 cases treated with Extracorporeal Shock Wave Lithotripsy (ESWL), were followed up for 3 months. Forty four of the patients had renal stones (2 had bilateral renal stones, 3 had staghorn calculi, 2 had incomplete staghorn calculi, 10 had multiple renal stones), 2 had the unilateral renal and ureteral stones and 16 had ureteral stone. The stone had been discharged completely within 3 months in 47 cases (86.8%), and residual stones were noticed in 6 cases (11%), two of which had stone discharge after retreatment with ESWL. The other cases are being followed up without further treatment, because the residual sandy stones are thought to be able to be discharged spontaneously.     

远端肾小管酸中毒致泌尿系结石的诊治(附20例报告)

目的：探讨远端肾小管酸中毒(dRTA)引起泌尿系结石患者的诊断和治疗方法。方法：回顾性分析20例dRTA引起泌尿系结石患者的临床资料。结果：结石均取出或排除，术后继续使用相应药物治疗，防止结石复发。结论：dRTA引起的泌尿系结石需要相应药物治疗，同时需取出或排除结石，解除梗阻，保护肾功能。     

Extracorporeal shock wave lithotripsy for upper urinary tract stone using piezoelectric lithotripter (Piezolith 2200)

The results of clinical trial using the second generation extracorporeal shock wave lithotripter (Piezolith 2200, Wolf, West Germany) were presented. The treatments were performed between December 1987 and March 1988 at the University of Tokyo. In total 59 ESWL sessions were carried out on 32 patients with 48 upper urinary tract stones. The treatment could be performed without anesthesia or analgesic agent in every case. A double-J ureteral stent was indwelling in 6 patients with ureteral stones or large stones before ESWL treatment, and transurethral lithotripsy (TUL) was performed in two patients after the treatment. On the X-ray film obtained three weeks after the final ESWL treatment, 13 cases (40.6%) were completely free from stone fragments, while 9 cases (28.2%) had stone fragments less than or equal to 5 mm. Others had fragments greater than 5 mm. ESWL using this device was clinically useful in 71.9% in the three weeks follow-up period. No serious complication was observed after treatment except macroscopic hematuria for a few days in all cases, pyrexia in 4 cases (12.5%) and flank pain in 7 cases (21.9%). There were slight and transient changes in the laboratory data after the treatment, but these changes were milder than those with spark gap lithotripters. It is concluded that Piezolith 2200 is useful in the management of patients with upper urinary tract stones, and it is applicable more safely than the first generation lithotripters.     

Renal acidification defects in medullary sponge kidney

Thirteen patients with medullary sponge kidney underwent a short ammonium chloride loading test to investigate their renal acidification capacity. All but 1 presented with a history of recurrent renal calculi and showed bilateral widespread renal medullary calcification on X-ray examination. Nine patients had some form of renal acidification defect; 8 had the distal type of renal tubular acidosis, 2 the complete and 6 the incomplete form. One patient had proximal renal tubular acidosis. These findings, which suggest that renal acidification defects play an important role in the pathogenesis of renal calculi in medullary sponge kidney, have considerable therapeutic implications.