[Laparoscopic pyeloplasty combined with ultrasonic lithotripsy via nephroscope for treatment of ureteropelvic junction obstruction with renal calculi].

OBJECTIVE: To investigate the efficacy and safety of laparoscopic pyeloplasty combined with ultrasonic lithotripsy via nephroscope in the treatment of ureteropelvic junction obstruction (UPJO) with renal calculi. METHODS: From June 2016 to January 2022, eight patients including five males and three females underwent laparoscopic pyeloplasty combined with ultrasonic lithotripsy via 19.5F(1F≈0.33 mm) nephroscope in Peking University People' s Hospital. The age ranged from 23-51 years (mean: 40.5 years) and the body mass index (BMI) ranged from 18.8-32.4 kg/m2 (mean 27.0 kg/m2). The lesion located on the left side in all of the eight patients. Two patients had solitary kidney and one patient had horseshoe kidney. Solitary stone was seen in one patient and the other seven patients suffered multiple stones, with two patients had staghorn stones. The largest diameter of stones ranged from 0.6-2.5 cm (mean: 1.5 cm). CT or ultrasound showed that moderate nephrosis was seen in five patients and severe nephrosis was seen in three patients. During surgery, after exposure of renal pelvis and proximal ureter, a small incision of 1.5 cm was performed in the anterior wall of the renal pelvis, and a 19.5F nephroscope was introduced into renal pelvis through laparoscopic trocar and renal pelvis incision. Stones were fragmented and sucked out by 3.3 mm ultrasonic probe placed through nephroscope. After stones were removed, modified laparoscopic pyeloplasty was performed. RESULTS: Surgery was successfully completed in all of the eight patients without conversion to open surgery. The operation time ranged from 160-254 min (mean 213 min) and the time of nephroscopic management time was 25-40 min (mean: 33 min). The hemoglobin was decreased by 3-21 g/L (mean: 10.3 g/L). The stone-free rate was 75% (6/8 cases), stones were incompletely removed in two patients due to abnormal intrarenal structure. The modified Clavien classification system (MCCS) grade ⅢA complication occurred in one patient postoperatively, which was nephrosis due to intrarenal bleeding, and nephrostomy was performed. With the mean follow-up of 30 months (ranged from 2-68 months), there was no evidence of obstruction in all the patients, and one patient underwent percutaneous nephrolithotomy to treat residual calculi. CONCLUSION: Laparoscopic pyeloplasty combined with ultrasonic lithotripsy via 19.5F nephroscope is feasible and safe, and could be a complementary method to treat UPJO and renal calculi.

HDR syndrome with a novel mutation in GATA3 mimicking a congenital X-linked stapes gusher: a case report.

BACKGROUND: Hypoparathyroidism, sensorineural hearing loss, and renal disease (HDR) syndrome, also known as Barakat syndrome, is a rare genetic disorder with high phenotypic heterogeneity caused by haploinsufficiency of the GATA3 gene on chromosome 10p14-p15. For these reasons, the diagnosis of HDR syndrome is challenging and requires a high index of suspicion as well as genetic analysis. CASE PRESENTATION: A 14-month-old boy, with sensorineural hearing loss in both ears, showed typical radiological features of X-linked stapes gusher on preoperative temporal bone computed tomography (CT) for cochlear implantations. Then after his discharge from hospital, he suffered a hypocalcemic seizure and we discovered a renal cyst during investigation of hypocalcemia. He was finally diagnosed with HDR syndrome by clinical findings, which were confirmed by molecular genetic testing. Direct sequencing of the GATA3 gene showed a heterozygous 2-bp deletion (c.1201_1202delAT), which is predicted to cause a frameshift of the reading frame (p.Met401Valfs*106). CONCLUSIONS: To our knowledge, this is the first case of HDR syndrome with a novel de novo variant mimicking a congenital X-linked stapes gusher syndrome. Novel mutations and the diversity of clinical manifestations expand the genotypic and phenotypic spectrum of HDR syndrome. Diagnosis of HDR syndrome is still challenging, but clinicians should consider it in their differential diagnosis for children with a wide range of clinical manifestations including hypocalcemia induced seizures and deafness. We hope that this case will contribute to further understanding and studies of HDR-associated GATA3 mutations.

Fetal Renal Stem Cell Transplant in Nephrotic and Nonnephrotic Glomerulonephritis with Stage 2-4 Chronic Kidney Disease: Potential Effect on Proteinuria and Glomerular Filtration Rate.

OBJECTIVES: Proteinuria is a major cause of glomerulosclerosis progression in glomerular diseases, and the development of end-stage renal disease is more rapid in nephrotic patients than in nonnephrotic ones. The renal parenchyma is less regenerable because it is a tissue consisting of renal cells. Thus, stem cells obtained from fetal kidney tissue might be effective for reducing proteinuria and delaying glomerulosclerosis in these patients. MATERIALS AND METHODS: This report presents preliminary data from a prospective cohort study that included 17 patients with chronic glomerulonephritis in stage 2 to 4 chronic kidney disease who completed 3 visits during 1 year of follow-up. Fetal renal stem cells (multiple cells in suspension) were injected into the patient every 6 months. Patients were divided into 2 groups according to their nephrotic status, and 24-hour maximal proteinuria was recorded for at least 6 months (first group with proteinuria < 3.5 g/24 h, and second group with proteinuria > 3.5 g/24 h). RESULTS: During follow-up, group 1 was observed to have stable hemoglobin and total protein levels but significantly decreased albumin levels and glomerular filtration rates. In group 2, total protein with serum albumin significantly increased, and proteinuria and glomerular filtration rates significantly decreased. There was no significant difference in glomerular filtration rate decline between groups. CONCLUSIONS: Treatment with fetal renal stem cells significantly decreased proteinuria in nephrotic patients. However, this outcome also might have resulted from a reduction in glomerular filtration rate. Further studies with a larger number of patients and a control group would help to achieve better results that measure the efficacy of this treatment.

[Ultrasonographic finding and treatment of a cow with pyelonephrosis]. / Sonographische Befunde und Therapie bei einer Kuh mit Pyonephrose.

Ultrasonography of a six-year-old Simmental cow revealed an abscess like structure, approximately 15 cm in diameter, in place of the right kidney. The cow had a history of colic for 4 days and was referred to our clinic with a tentative diagnosis of caecal dilatation. The cow voided dark opaque urine with white floccules. Laboratory examination yielded increased haematocrit, leukocytosis with left shift, hyperbilirubinaemia and azotaemia. The diagnosis was confirmed, the caecum emptied surgically and the pus-filled structure at the site of the right kidney removed. A diagnosis of pyonephrosis was made after pathological examination of the pus-filled structure.

Renal pelvic villous adenoma presented with mucusuria: report of a case and literature review.

Villous adenomas of the urinary tract are an uncommon condition, and appear mostly in patients where the disease occurred in the lower urinary tract. In contrast, upper urinary tract villous adenomas are a rare condition. Currently, just three cases of villous adenoma in the renal pelvis have been published. Herein, we present the fourth case of a renal pelvic villous adenoma, along with muconephrosis and mucusuria. A 73-year-old man presented with abdominal discomfort and a palpable abdominal mass. He had a history of bilateral anatrophic nephrolithotomy, 8 years and 6 years earlier. The preoperative radiographic investigation showed severe right hydronephrosis. A right nephrectomy was carried out and the intraoperative finding showed severe perinephric adhesion and a great deal of mucus in the renal pelvis. The pathological examination showed a villous adenoma and urothelial metaplasia in the kidney.

Florid endometriosis in a postmenopausal woman.

OBJECTIVE: To report a case of florid endometriosis. DESIGN: Case report. SETTING: University hospital. PATIENT(S): A 53-year-old postmenopausal woman with a 10-cm retroperitoneal mass comprised of endometriosis causing hydroureteronephrosis and loss of ipsilateral kidney function. INTERVENTION(S): The patient underwent exploratory laparotomy with extensive lysis of adhesions, right nephrectomy, radical resection of the retroperitoneal mass including partial resection of the psoas muscle, dissection from the inferior vena cava, and resection of distal ileum, cecum, and appendix with a primary ileoascending colon reanastomosis. MAIN OUTCOME MEASURE(S): Postoperative symptom resolution. RESULT(S): The patient had widespread adhesive disease with a primary retroperitoneal endometriotic mass and a secondary mass involving the small bowel mesentery. Endometriomas were found in the right kidney and right distal ureter. Additional endometriotic implants were found at the right common iliac bifurcation, appendix, and in multiple mesenteric nodules. No residual ovarian tissue was identified, and preoperative FSH and estrogen (E) levels indicated no evidence of an ovarian remnant. CONCLUSION(S): Severe endometriosis caused ipsilateral renal failure despite postmenopausal levels of E and FSH, supporting the theory that endometriotic implants may have an autocrine function involving E biosynthesis or may respond to hormone production in adipose tissue.

Intermittent or daily administration of 1-alpha calcidol for nephrectomised infants on peritoneal dialysis?

Secondary hyperparathyroidism and renal osteodystrophy are major problems in patients with end-stage renal failure and may result in poor growth in children on dialysis. Whether vitamin D sterols should be given intermittently or daily remains a controversial issue. We studied 16 bilaterally nephrectomised infants with congenital nephrosis of the Finnish type (median age 0.54 years), all on peritoneal dialysis. Nine of them were receiving intermittent 1-alpha calcidol therapy and seven daily 1-alpha calcidol therapy. The target serum parathyroid hormone (PTH) level was 2-3 times the upper limit of normal (ULN). There were no statistically significant differences in PTH values between the groups (1.7-times vs 0.5-times the ULN at 3 months and 3.1-times vs 3.4-times the ULN at 6 months, respectively). The required weekly doses of 1-alpha calcidol were low, and there were no significant differences between the intermittent and daily groups (0.06 microg/kg vs 0.04 microg/kg at 3 months and 0.09 microg/kg vs 0.05 microg/kg at 6 months, respectively). The infants on intermittent 1-alpha calcidol showed significant catch-up growth during dialysis after nephrectomy relative to the infants on daily 1-alpha calcidol (-1.6 SD to -0.7 SD vs -1.4 SD to -1.0 SD, respectively; P < 0.05). Our results indicate that either intermittent or daily vitamin D analogue therapy, if started early, will prevent secondary hyperparathyroidism equally well in children on peritoneal dialysis (PD), but intermittent therapy might be more favourable for growth.

Upregulation of ADAM19 in chronic allograft nephropathy.

ADAM19 (a disintegrin and metalloproteinase 19) is involved in cell-cell and cell-matrix interactions and tumor necrosis factor (TNF)-alpha shedding. We studied ADAM19 in chronic allograft nephropathy (CAN) nephrectomies and in normal human kidneys. Reverse transcriptase (RT) PCR revealed an upregulation of ADAM19 mRNA in CAN when compared with control kidneys (p = 0.002). Using RNA in situ hybridization (ISH), we detected moderate ADAM19 mRNA expression in vascular smooth muscle cells (SMCs) and distal tubuli of control kidneys. In CAN, massive ADAM19 expression was detected in SMCs, distal tubuli, glomerular sclerotic lesions and inflammatory CD4+ cells. To determine whether ADAM19 is specifically related to CAN, we studied transplant biopsies with and without CAN, acute rejection and non-transplant-related kidney diseases: interstitial fibrosis (IF), interstitial atrophy, glomerular fibrosis and interstitial inflammation. In various renal structures, ADAM19 mRNA was significantly higher in CAN when compared with renal allografts without CAN or acute rejection. ADAM19 expression in renal endothelium was significantly higher in acute rejection when compared with renal allografts without CAN. When compared to CAN, ADAM19 was expressed to a similar extent in non-transplant-related interstitial and glomerular fibrosis, interstitial atrophy and inflammation. Although these observational data do not establish a cause and effect relationship, ADAM19 may have a modulatory role in the dysfunctional renal allograft state.

Percutaneous ureterostomy as an effective diversion in a newborn.

Ectopic ureter in a duplicated system in men is rare and rarely causes bilateral obstructive symptoms. The tendency of the ureter to dilate more than the caliceal system is unique to neonates and makes upper urinary diversions more challenging. However, alternative percutaneous diversions other than nephrostomy might be beared in mind in such cases with huge dilatation in ureters in suffering neonates. As discussed in this case percutaneous ureterostomy may be very effective and have a role in diagnosis and management of neonatal hydroureteronephrosis.

[Clinical case of the month. Peritonitis after spontaneous rupture of pyonephrosis. A case report]. / Le cas clinique du mois. Péritonite secondaire à la rupture spontanée d'une pyonéphrose dans la grande cavité péritonéale. A propos d'un cas.

Peritonitis after spontaneous rupture of pyonephrosis is a rare complication, usually diagnosed intraoperatively. We report the case of a 35 year old woman who was admited for an acute abdomen. Ultrasound showed features of acute peritonitis, with left pyonephrosis, confirmed by a CT-SCAN. Treatment consisted of laparotomy with nephrectomy and abdominal washing and draining. The outcome was favourable.

Laparoscopic-assisted upper pole ureterocalicostomy using renal inversion and autotransplantation.

Despite various described methods of reconstruction after upper ureteral injury, many cases are complex and remain a surgical challenge. Careful preoperative evaluation and planning are crucial in the selection of the appropriate procedure, particularly in patients in whom preservation of the renal mass is imperative. We report a case of severe upper ureteral injury and subsequent fibrosis, with no usable renal pelvis and focal upper pole dilation, that was managed with renal inversion and upper pole ureterocalicostomy. The option of autotransplantation was provided by laparoscopic nephrectomy and ex vivo reconstruction, minimizing patient morbidity and maximizing a successful outcome. This case illustrates the expansion of laparoscopy from the mere extirpative to a role in complex reconstruction. To our knowledge, this reconstructive strategy has never been previously reported and may be applicable in a limited number of situations.

Unilateral ureteroperitoneostomy in the management of hypoproteinemia in nephrotic rats with normal renal function.

Maintenance of serum albumin levels within normal limits is difficult to achieve in nephrotic children with normal renal functions who are unresponsive to specific treatment. One approach in such children is unilateral nephrectomy with rapid progression to renal failure. Peritoneal membrane is permeable to fluids, electrolytes and proteins, and peritoneal space has been used for total parenteral alimentation. Experimental ureteroperitoneostomy has been reported not to cause any significant side effect. The aim of this study was to evaluate the effects of unilateral ureteroperitoneostomy on serum and urine protein levels in rats with adriamycin-induced nephrosis. Adriamycin nephrosis was induced in 45 male Wistar rats. After two weeks, unilateral nephrectomy (Nx), unilateral ureteroperitoneostomy (Up) and sham operated (Sh) groups, each including 15 rats were formed. Serum creatine (S(Cr)) and albumin (S(alb)), and daily urinary protein excretion (U(pro)) were determined before adriamycin injection (week 0), before operations (week 2) and at the end of 6th week in all rats. In addition, percent change in serum albumin (deltaS(alb)) and urine protein levels (deltaU(pro)) between weeks 0-2, 0-6 and 2-6 were calculated for each group (e.g.; deltaS(alb) 0-2 = [S(alb) week 2-S(alb) week 0]/S(alb) week 0 x 100). Then, these parameters were compared within and between the groups. Furthermore, peritoneal tissue samples were obtained from the rats in Sh and Up groups to be examined for pathological changes. S(Cr) did not change within and in between the groups during the study period. S(alb) decreased significantly at weeks 2 and 6 with respect to week 0 in all three groups. In addition, although S(alb) tended to decrease at week 6 with respect to week 2 in all groups, this was significant only in Sh group. U(pro) increased significantly at weeks 2 and 6 with respect to week 0, and at week 6 with respect to week 2 in all groups. However, S(alb) and U(pro) were not different between the three groups at weeks 0.2 and 6. On the other hand, deltaS(alb) and deltaU(pro) were not different between Sh vs. Nx and Nx vs. Up rats, but deltaS(alb) 0-6, deltaS(alb) 2-6 and deltaU(pro) 0-6 were significantly lower in Up group compared to Sh group. Histopathological examination of peritoneal samples revealed significantly higher fibrosis score in Up group compared to Sh group. In conclusion, unilateral ureteroperitoneostomy may one important therapeutic selection in the treatment of intractable nephrotic syndrome. However, peritoneal fibrosis could be a concern for further use of peritoneum in case of end stage renal failure.

Muconephrosis.

We report a case of a mucus-filled kidney (muconephrosis) encountered during laparoscopic nephrectomy for presumed xanthogranulomatous pyelonephritis. Conversion to open nephrectomy and en-bloc right hemicolectomy were necessary because of severe perinephric fibrosis and suspected renal-enteral fistula. Pathologic examination revealed a renal pelvic villous adenoma and diffuse intestinal metaplasia of the urothelium; no enteral communication or gastrointestinal pathologic features were found. Urothelial villous adenoma is extremely rare, and one should consider neoplastic etiologies, including appendiceal mucinous cystadenocarcinoma and mucus-secreting adenocarcinoma of the renal pelvis, in directing the appropriate management of muconephrosis.

Laparoscopic nephrectomy in children: two case reports.

We successfully performed a laparoscopic nephrectomy on 2 young girls in March 1994 and August 1994. The first patient was a 9-year-old girl with a hypoplastic kidney associated with an ectopic ureterocele, and the other was a 3-year-old girl with a nonfunctioning hydronephrotic kidney caused by stricture of the pyeloureteral junction. The operating times were 153 and 183 minutes, respectively. No complications occurred intraoperatively or postoperatively in either patient. The 9-year-old girl resumed normal daily activities by postoperative day 2, and the 3-year-old girl by postoperative day 3. Both girls were discharged on day 4.

Recurrence of de novo membranous glomerulonephritis on renal grafts.

An updated study of the glomerular lesions found in renal grafts in children showed 3 features of interest. 1) In our experience, the incidence of the occurrence of de novo membranous glomerulonephritis (MGN) remains around 9% (48 of 530 grafts). 2) Of the 29 patients we reported in a previous study [Antignac et al. 1988], 18 lost their grafts and 7 received a second graft. Four of these 7 patients recurred de novo MGN on their second graft. Their clinical course is reported in detail. 3) In our population of transplanted children, of the 55 patients who received a second graft, the only recipients who developed a de novo MGN were the 4 patients who had already developed de novo MGN on their first graft. The various factors possibly involved in the pathogenesis of de novo MGN are reviewed. The high incidence of recurrence of de novo MGN indicates that host factors play a major role in the development of this nephropathy.

Renal transplantation in 22 children with nephropathic cystinosis.

In 1989, 22 children (11 boys, 11 girls aged 8-23 years) with nephropathic cystinosis, who had received a total of 28 renal allografts over the previous 14 years, were reviewed. Nineteen were alive, of whom 17 had functioning grafts 5 months to 13 years after transplantation. The mean serum creatinine level in these 17 was 135 mumol/l. Patient and graft survival did not differ from non-cystinotic children. Persistent hypothyroidism was found in 3 patients, transient diabetes mellitus in 1, severely disturbed vision in 1 and brain atrophy in 11. Arterial hypertension was present in 16 patients. Growth retardation was universal, although in 4 patients on cyclosporin A post-transplant catch-up growth occurred. Five patients over 15 years completed puberty. Readjustment in terms of school performance was good but was less good for psychosocial development. None of the patients had ever been treated with cystine-depleting agents; the data will therefore provide a historical control group with which to compare the results from a group treated with these agents.

[Results of 310 cadaver kidney transplants in children and adolescents]. / Résultats de 310 transplantations de rein de cadavre chez l'enfant et l'adolescent.

This study reports the results of 310 cadaver kidney transplantations in 295 children and adolescents performed from 1973 to 1983. The actuarial survival of patients was 97% at one year and 92% at 5 years; that of grafts was 79% at 1 year and 65% at 5 years, these rates having improved during the last years. Results were similar and even better for the 18 second transplantations. Among the causes of failure, rejection comes first (65%), then thromboses of renal artery (13%) and relapses of oxalosis or steroid resistant nephrosis (12%). Patients with cytotoxic antibodies have a less good survival of grafts, especially after 5 years. HLA A and B compatibility is a factor of success. Among complications hypertension is frequent, 53% of patients receiving antihypertensive treatment after 1 year. It is sometimes severe and was responsible for death in 5 cases. Growth was variable after transplantation: 25% of children before puberty had a catch-up curve, 25% had an unchanged growth and 50% an increased retardation. The average standard deviation was near zero but it was -0.49 SD/year in patients with creatinine level greater than or equal to 150 mumol and +0.24 SD in children under alternate day steroid therapy. Rehabilitation was excellent, less than 3% of patients not being engaged in any activity 1 year after transplantation.

Pyonephrosis: imaging and intervention.

A series of 70 pyonephrotic kidneys drained by percutaneous nephrostomy tube was examined to evaluate the contribution of radiologic imaging to the diagnosis of pyonephrosis and to assess the diagnostic and therapeutic role of drainage by percutaneous nephrostomy catheter. The diagnosis of pyonephrosis is suspected when the clinical symptoms of fever and flank pain are combined with the radiologic evidence of obstruction to the urinary tract. Sonography gives a prompt diagnosis of hydronephrosis, and needle puncture of the kidney yields pus and establishes the presence of pyonephrosis. A percutaneous nephrostomy catheter is then inserted and serves for initial drainage of infected urine and for evaluation of residual kidney function before definitive surgery. The nephrostomy catheter is used for diagnostic nephrostograms, ureteral perfusions, therapeutic dissolution of stones, and indefinite drainage of the kidney. In 10 azotemic patients, the blood urea nitrogen and serum creatinine values returned to normal levels after antibiotic therapy and nephrostomy drainage of infection. Long-term evaluation of residual renal function by means of an excretory urogram or a renogram was available in another 26 patients and 25 of them showed function of the previously pyonephrotic kidney.