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1.
Arch Esp Urol ; 77(4): 331-337, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38840274

RESUMO

BACKGROUND: This study aimed to assess the feasibility, safety, and efficacy of an endoscopic parapelvic renal cyst (PRC) incision using flexible ureterorenoscopy (fURS). MATERIAL AND METHODS: We retrospectively reviewed data concerning 16 patients in whom PRC incisions had been performed using fURS between January 2016 and January 2022. Two patients were excluded from the study owing to a lack of follow-up information. The cysts of all the patients were evaluated preoperatively by computed tomography. The patients' age, gender, cyst size, presenting symptoms, postoperative complications, and pre- and post-treatment visual analogue scale (VAS) scores were evaluated. Surgical success was defined as a reduction of more than half of the cyst size in the sixth postoperative month. RESULTS: A total of 14 patients were included in this study. The patients' mean age was 52.6 ± 8.8 years, and the mean cyst size was 69.1 ± 15.5 mm. Twelve (85.7%) patients presented with flank pain. Clavien-Dindo grade 1 complications were observed in two patients (14.3%), and grade 2 complications were observed in one (7.1%). The median VAS scores were significantly lower after treatment than before in patients who presented with flank pain (2 (1-2.8) vs 8 (7-8), respectively; p = 0.002). Surgical success rate was detected in 11 patients (78.6%) six months after the treatment. CONCLUSIONS: Endoscopic incision of the PRC is a feasible treatment modality with high success rates and low complication rates. However, multicentre studies with larger populations and longer follow-ups are needed to evaluate the lasting effects.


Assuntos
Estudos de Viabilidade , Doenças Renais Císticas , Ureteroscopia , Humanos , Pessoa de Meia-Idade , Feminino , Masculino , Estudos Retrospectivos , Ureteroscopia/efeitos adversos , Ureteroscopia/métodos , Doenças Renais Císticas/cirurgia , Doenças Renais Císticas/diagnóstico por imagem , Resultado do Tratamento , Pelve Renal/cirurgia , Adulto , Ureteroscópios , Idoso , Desenho de Equipamento
2.
Urol Case Rep ; 53: 102682, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-38495852

RESUMO

We present a 64-year-old male who presented with right-sided flank pain secondary to a parapelvic cyst impinging on the proximal ureter. However, intraoperative findings showed that the ureter was encased within the parapelvic cyst and secondarily obstructed at the UPJ rather than extrinsically compressed by the cyst. This specific anatomic variant is exceedingly rare as no published cases with similar anatomy could be identified.

3.
Asian J Urol ; 10(2): 172-176, 2023 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-36942122

RESUMO

Objective: To explore the efficacy, safety, and feasibility of holmium laser flexible ureteroscopic intrapelvic drainage in the treatment of parapelvic renal cysts. Methods: From September 2012 to February 2019, a total of 18 patients, aged from 28 to 62 (mean±standard deviation [SD]: 46.50±9.14) years, were diagnosed with parapelvic renal cysts and treated by holmium laser flexible ureteroscopic intrapelvic drainage. There were 10 males and eight females. All of the parapelvic renal cysts were unilateral, and two cases were complicated with pyelolithiasis. The diameters of the cysts ranged from 4.1 cm to 8.2 cm. Results: All the patients completed the operation successfully in one stage without conversion to open surgery; in two cases, it was difficult to find the cysts during the operation, and the localization was completed by B-ultrasound and percutaneous injection of methylene blue. The mean operative time was 33.89 (SD: 9.68; range: 22-54) min, and the mean hospitalization time was 2.67 (SD: 0.91; range: 2-5) days. Three months and 6 months of follow-up were performed after surgery. The cysts disappeared in 13 (72%) cases, and the diameter of the cysts in five (28%) cases decreased by more than 50%. Conclusion: Holmium laser flexible ureteroscopic intrapelvic drainage in the treatment of parapelvic renal cysts is simple, safe, and effective, and can be used as the first choice for the treatment of parapelvic renal cysts.

4.
BMC Surg ; 22(1): 315, 2022 Aug 13.
Artigo em Inglês | MEDLINE | ID: mdl-35964028

RESUMO

OBJECTIVE: This study aims to investigate the efficacy and safety of intraoperative real time ultrasound-assisted flexible ureteroscopic holmium laser incision and internal drainage in the treatment of parapelvic cysts, and to review recently published relevant literature. METHOD: This is a retrospective study in which the clinical data of 47 patients who underwent flexible ureteroscopic holmium laser incision and internal drainage of parapelvic cysts in our center from March 2017 to March 2021 were retrospectively analyzed. A literature search was conducted to review and summarize relevant reports on endoscopic treatment of parapelvic cysts published in the past 10 years. RESULTS: Among 47 patients with parapelvic cysts who underwent flexible ureteroscopic holmium laser incision and internal drainage, 12 (25.53%) cases had a typical cyst wall bulging into the collecting system under flexible ureteroscope. As the cyst wall was thin and translucent in these cases, ultrasound was not used during the operation. The cysts of the remaining 35 patients were located with the aid of intraoperative real time ultrasound, and all underwent successful operation. No serious surgical complications occurred after surgery. The patients were followed up for 12-24 months after operation. The cyst in one case was observed larger than its original size before operation, so recurrence was considered. In another two cases, the diameters of the cysts were more than half of their original diameters before operation. Thus, the efficacy was poor in the three cases. For the remaining 44 cases, there was no obvious cyst observed or the diameter of the cysts was less than half their preoperative level. CONCLUSION: The approach of ultrasound-assisted flexible ureteroscopic holmium laser incision and internal drainage in the treatment of parapelvic cysts is safe and effective, which helps to solve the problem of localization of atypical parapelvic cysts on endoscopic findings.


Assuntos
Cistos , Doenças Renais Císticas , Lasers de Estado Sólido , Cistos/cirurgia , Drenagem , Humanos , Doenças Renais Císticas/cirurgia , Lasers de Estado Sólido/uso terapêutico , Estudos Retrospectivos , Resultado do Tratamento , Ureteroscopia
5.
J Nephrol ; 35(8): 2035-2046, 2022 11.
Artigo em Inglês | MEDLINE | ID: mdl-35749008

RESUMO

Simple renal cysts are a common finding during abdominal imaging assessment. The incidence increases with age and it is higher in male gender. Parapelvic cysts are a subset of simple cysts that arise within the renal parenchyma, adjacent to the renal sinus, characterized by being generally single, larger, and incompletely surrounded by renal parenchyma. Noteworthy, parapelvic cysts are a rare and understudied condition which, although considered clinically insignificant due to the absence of influence on renal function, still have a controversial aetiopathogenesis. On the other hand, urological management and differential diagnosis have been thoroughly investigated. The aim of our review is to provide an overall vision on this rare condition, usually misdiagnosed and underestimated, on the basis of more recent data. An accurate differential diagnosis of parapelvic cysts can lead to the identification of treatable conditions such as Fabry disease, autosomal dominant polycystic kidney disease, polycystic liver disease and tuberous sclerosis complex disease.


Assuntos
Cistos , Neoplasias Renais , Hepatopatias , Rim Policístico Autossômico Dominante , Humanos , Masculino , Rim Policístico Autossômico Dominante/complicações , Rim Policístico Autossômico Dominante/diagnóstico por imagem , Rim Policístico Autossômico Dominante/genética , Cistos/diagnóstico por imagem , Cistos/terapia , Rim/patologia , Hepatopatias/diagnóstico , Hepatopatias/patologia , Neoplasias Renais/patologia , Biomarcadores , Doenças Raras/patologia
6.
J Clin Med ; 11(7)2022 Apr 05.
Artigo em Inglês | MEDLINE | ID: mdl-35407642

RESUMO

BACKGROUND: Symptomatic parapelvic cysts (PPC) are rare entities. Our objective is to highlight specific features of PPC to avoid a misdiagnosis of UPJ obstruction. METHODS: We retrospectively reviewed the records of children managed between 2012-2017. RESULTS: All four patients (18 months-8 years) presented with acute renal colic with a large intra-sinusal liquid mass (42-85 mm) on ultrasound, evoking a diagnosis of UPJ obstruction. On preoperative renal scintigraphy (n = 3) there was no dilatation of the renal pelvis and ipsilateral differential function was impaired in 2. Diagnosis of PPC was suspected preoperatively in three children (CT scan (n = 1); MRI (n = 2)) and made peri-operatively (n = 1). Preoperative retrograde pyelography (n = 3) and a further intraoperative retrograde pyelography with methylene blue (n = 1) did not identify communication with the cyst. No renal pelvis was identified in two patients. De-roofing of the cyst was curative in all cases at 5 years mean follow-up (no leakage, cyst recurrence or loss of function) and all 4 patients became asymptomatic after surgery. Histology demonstrated a single flat epithelial cell layer. Renal function normalized in one patient but remained impaired in the other. CONCLUSION: In case of symptoms of UPJ obstruction with a medial renal liquid mass on ultrasound, PPC should be considered when no dilatated pelvis on renal scan is identified. In such cases, a complementary imaging work-up is mandatory prior to surgery.

7.
Urologiia ; (1): 61-66, 2022 Mar.
Artigo em Russo | MEDLINE | ID: mdl-35274861

RESUMO

INTRODUCTION: Parapelvic renal cysts are very common. Indications for surgical treatment are upper urine tract obstruction, pain and recurrent gross hematuria. AIM: To analyze the efficiency and safety of endoscopic transurethral and percutaneous laser marsupialization of parapelvic renal cysts. MATERIALS AND METHODS: A total of 9 patients were undergone to transurethral intrarenal marsupialization of parapelvic renal cysts from March 2016 to February 2021 (4 men, 5 women, aged 42-78 years). Another 2 patients (2 men, aged 46 and 52 years) were treated by percutaneous approach. The average size of the cyst according to contrast-enhanced multi-slice computed tomography (MSCT) was 3.1+/-1.8 cm. In two cases, papillary tumor of the pelvis was suspected. The anteroposterior diameter of the pelvis was 2.6+/-1.3 cm; 9 patients had pain in the loin area, while in 7 patients recurrent gross hematuria was also an indication for surgical treatment. For marsupialization, a holmium (Ho:YAG) laser Auriga XL (Boston Scientific, USA) was used in 4 patients, and in other cases (n=7) a procedure was performed using a thulium fiber laser (Tm Fiber) Fiberlase U1 (IRE-Polus, Russia). In 3 patients, to clarify the site of incision of the cyst, intraoperative ultrasound was used. In all cases, after draining the cyst, an internal stent was placed inside the cyst for a period of 4-6 weeks. RESULTS: The duration of transurethral surgery was 26+/-11 minutes, while percutaneous marsupialization of the cyst, which was performed in combination with percutaneous nephrolithotomy, took 10 and 18 minutes, respectively. The average catheterization time was 12+/-8 hours. Nephrostomy tube was removed on the 2nd day. The length of stay was 4+/-2 days. Febrile fever was noted in 1 patient (9%), which required a change in antibiotic therapy. During ultrasound control at discharge, the dilatation of the collecting system was not detected in any cases, while the residual cavity was found in 2 patients (18%). Follow-up contrast-enhanced MSCT and ultrasound within 3-30 months in all patients (n=11) showed no dilatation of the collecting system. In 1 (9%) patient, the residual cavity was preserved with a decrease in size to 1.2 cm without signs of upper urinary tract obstruction; the initial diameter of the cyst in this patient was 4.9 cm. There was no recurrence of gross hematuria. CONCLUSIONS: In our opinion, transurethral and percutaneous laser marsupialization of parapelvic renal cysts is an effective and safe method that allows definitive treatment for cysts up to 4 cm in size. If the cyst is larger than 4 cm, endoscopic removal should be balanced with the possibility of preserving the residual cavity. The most common complication of endoscopic treatment of intrarenal cysts is acute pyelonephritis with a rate of 9%.


Assuntos
Doenças Renais Císticas , Nefrostomia Percutânea , Adulto , Idoso , Feminino , Humanos , Doenças Renais Císticas/diagnóstico por imagem , Doenças Renais Císticas/cirurgia , Masculino , Pessoa de Meia-Idade , Resultado do Tratamento , Ultrassonografia , Ureteroscopia/métodos
8.
BMC Urol ; 22(1): 7, 2022 Jan 24.
Artigo em Inglês | MEDLINE | ID: mdl-35073883

RESUMO

BACKGROUND: Flexible ureteroscopic incision and drainage is a relatively new surgical method for treating parapelvic cysts. Considering that the intraoperative localization of the cyst may fail with a flexible ureteroscope, we use an innovative ultrasound-guided method to locate the cystic wall during flexible ureteroscopic surgery. METHODS: We retrospectively reviewed 17 consecutive cases of parapelvic renal cysts treated by ultrasound-guided flexible ureteroscopy between March 2017 and May 2020. The differences between the simple flexible ureteroscopic technique and ultrasound-guided flexible ureteroscopic technique were compared. The surgical procedures, postoperative complications, results and patient follow-ups were evaluated. RESULTS: The cyst wall was seen clearly in 10 patients with ureteroscopic vision. Another 7 patients underwent ultrasound-guided flexible ureteroscopic surgery since it was difficult to identify the cyst wall. The mean operative time was 25.9 ± 8.7 min and 37.1 ± 10.1 min for the conventional and modified techniques, respectively (P = 0.004); the mean time to search for cysts was 17.6 ± 5.8 min and 26.5 ± 8.4 min, respectively (P = 0.002); and the mean incision time was 7.1 ± 4.9 min and 12.1 ± 5.6 min, respectively (P = 0.000). All of the patients were followed-up for 12 months, and no serious complications or recurrence were observed. CONCLUSIONS: We demonstrated that it is feasible and safe to treat parapelvic renal cysts by ultrasound-guided flexible ureteroscopic incision and drainage. The small sample size and need for further studies were the limitations of our work.


Assuntos
Doenças Renais Císticas/cirurgia , Pelve Renal/cirurgia , Cirurgia Assistida por Computador , Ultrassonografia de Intervenção , Ureteroscópios , Ureteroscopia/métodos , Adulto , Idoso , Desenho de Equipamento , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos
9.
J Pediatr Urol ; 17(6): 864-865, 2021 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-34479807

RESUMO

INTRODUCTION: We report a case of a right parapelvic renal cyst causing intermittent ureteropelvic junction obstruction (UPJO). DIAGNOSTIC EVALUATION: A 13-year-old male was referred for right flank pain. Stone protocol CT revealed renal pelvis dilation with punctate stones. Due to concern for intermittently obstructive calculi, he underwent ureteroscopy, which was unremarkable. A diuretic renogram showed symmetric uptake with partial emptying on the right with pain after diuretic administration. In office, we potentiated a Dietl's crisis with ultrasound obtained before and after fluid intake. Comparison of ultrasounds revealed a parapelvic cyst causing calyceal dilation. He was counseled for robotic cyst decortication and possible pyeloplasty. SURGICAL CONSIDERATIONS: A robotic cyst decortication was performed. Once decorticated, the cyst base was fulgurated and pararenal fat was interposed into the cyst base. Console time was 70 min with minimal blood loss. The patient was discharged post-operative day 1. Follow-up renal ultrasound at 4 months demonstrated resolution of hydronephrosis and parapelvic cyst. CONCLUSION: Parapelvic renal cysts causing intermittent UPJO is a rare entity that may be missed on a diuretic renogram. Clinical suspicion and appropriate imaging with ultrasound or magnetic resonance imaging are useful. Robotic cyst decortication is a technically feasible approach to treat this condition.


Assuntos
Cistos , Hidronefrose , Procedimentos Cirúrgicos Robóticos , Obstrução Ureteral , Adolescente , Criança , Cistos/complicações , Cistos/diagnóstico por imagem , Cistos/cirurgia , Humanos , Pelve Renal/diagnóstico por imagem , Pelve Renal/cirurgia , Masculino , Estudos Retrospectivos , Obstrução Ureteral/diagnóstico por imagem , Obstrução Ureteral/etiologia , Obstrução Ureteral/cirurgia
10.
Exp Ther Med ; 21(2): 172, 2021 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-33456539

RESUMO

The present study aimed to compare the efficacy and safety of a flexible ureteroscopic holmium laser incision with flexible ureteroscopic 1470-nm diode laser incision for the treatment of parapelvic renal cysts. The current study collected and analysing the clinical data of 90 independent renal cysts cases retrospectively, including 43 renal cysts cases that received holmium laser surgery (holmium laser group) and 47 renal cysts cases that received 1470-nm diode laser surgery (1470-nm diode laser group). Each group was divided into a thin-walled cyst subgroup and thick-walled cyst subgroup according to cyst wall thickness. Intracapsular hematoma was significantly lower in the 1470-nm diode laser group compared with the holmium laser group (0/47 vs. 4/43; P=0.048). The incision diameter in the 1470-nm diode laser group was significantly larger than the holmium laser group in the thick-walled parapelvic renal cysts subgroup [1.70(1.50,1.90) vs. 1.30(1.25,1.70) cm; P=0.007]. The renal cystic diameter of the two groups was markedly reduced one and six months after surgery. The difference was non-significant in the diameter of the renal cyst in the thin-walled cysts subgroups between the two laser groups 6 months after surgery (1.01±0.38 vs. 1.03±0.53 cm; P=0.454). However, the diameter of the renal cyst in the thick-walled cysts subgroup treated with the 1470-nm diode laser was significantly lower compared with the thick-walled cysts subgroup treated with the holmium laser 6 months after surgery (1.21±0.57 vs. 1.88±0.94 cm; P=0.002). The results demonstrated that the use of a 1470-nm diode laser or holmium laser surgery under a flexible ureteroscope is a safe and effective treatment for parapelvic renal cysts. For thick-walled parapelvic renal cysts, the 1470-nm diode laser appears to exhibit a lower postoperative recurrence rate and better long-term postoperative effects due to its improved haemostatic effect and larger intraoperative incision diameter.

11.
J Endourol ; 35(4): 466-472, 2021 04.
Artigo em Inglês | MEDLINE | ID: mdl-33050738

RESUMO

Objective: To investigate the outcomes of retrograde flexible ureteroscopy in managing parapelvic renal cysts and speculate the factors affecting therapeutic efficacy. Methods: Thirty-eight patients with parapelvic renal cysts were recruited and underwent retrograde flexible ureteroscopy using holmium laser. Parapelvic cysts were divided into peripheral type and central type based on the position of cyst convex to the perirenal tissue. Feasibility and safety were retrospectively evaluated, and cases were analyzed to detect their distinctive characteristics. Independent-sample t-test and chi-square test were undertaken for continuous variables and categorical variables, respectively. Results: Radiologic evidence of success was achieved in 31 (81.58%) cases after a mean follow-up of 14.4 months (range 6-26 months). No significant perioperative complications were identified. There were seven cases with features of peripherally located parapelvic cyst. Four cysts shown as irregular protrusion were unable reduce to less half of previous size. Reductions were recorded in the other three patients with spherically peripheral protrusion. There was significant difference between these two types (p = 0.029). Among the 31 patients with centrally located parapelvic cyst, 28 of these have simple cysts that achieved radiologic success and 3 of the 31 patients were identified as failed cases indicated by renal pelvis enveloped by cyst on radiologic investigation. The success rate of simple cysts was significantly higher than that of the later type (p < 0.001). Conclusion: The location and shape of parapelvic cyst may play a critical role in the radiologic outcome of internal incision and patients with simple central or spherical peripheral cyst may benefit more from retrograde flexible ureteroscopy combined with laser incision.


Assuntos
Doenças Renais Císticas , Ureteroscopia , Humanos , Doenças Renais Císticas/diagnóstico por imagem , Doenças Renais Císticas/cirurgia , Estudos Retrospectivos , Resultado do Tratamento , Ureteroscópios
12.
Urol Case Rep ; 34: 101454, 2021 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-33194553

RESUMO

Pelviureteric junction obstruction can be attributed to intrinsic and extrinsic pathologies. We report an unusual cause of pelviureteric junction obstruction due to a large parapelvic cyst in a malrotated kidney. The patient presented with intermittent flank pain. The diagnosis was arrived at following imaging. The cyst was managed by open surgery.

13.
West Indian med. j ; 69(6): 459-460, 2021. graf
Artigo em Inglês | LILACS-Express | LILACS | ID: biblio-1515684
14.
Chinese Journal of Urology ; (12): 574-577, 2019.
Artigo em Chinês | WPRIM (Pacífico Ocidental) | ID: wpr-755489

RESUMO

Objective To evaluate the efficacy and safety of tip-flexible ureterorenoscope (tf-URS) with holmium laser for one-stage management of parapelvic cyst.Methods The clinical data of 26 patients treated with tf-URS incision and drainage using holmium laser from February 2016 to August 2018 were reviewed.The study were including 15 male and 11 female patients,ranging from 32 to 68 years old,with an average of 53.5 years old.There were 24 cases of unilateral single renal parapelvic cyst and 2 cases of bilateral single renal parapelvic cyst.There were 4 cases in 26 cases with unilateral single renal parapelvic cyst and contralateral single renal cyst,2 cases with unilateral single renal parapelvic cyst and ipsilateral kidney of stones.The diameter of parapelvic cyst was 3.4-5.6 cm,average 4.8 cm.All patients had undergone holmium laser endo-decortication of parapelvic cyst by tf-URS.With general anesthesia,tf-URS accessed pelvis retrogradely and decorticated parapelvic cyst with 200 μm Holium laser to drainage the cyst to pelvis.If the tf-URS was not placed successfully for the first time,Double-J tubes were retained for 1-2 weeks before treatment.The operative time,hospitalization time,blood loss,postoperative complications and clinical symptoms were collected and analyzed.Results The one-time access success rate of insertion of ff-URS was 88.5% (23/26).All operations were successful without severe complications.The average time of operation was 17.2 min,ranging from 11 to 25 min.In 3-30 months follow-up,the cysts disappeared in 22 patients and reduced by more than one half in 1 patients.Flank pain relieved in 19 patients.Conclusions Holmium laser endo-decortication of parapelvic cyst by tf-URS could be a simple,minimally invasive,safe and effective method for parapelvic cyst,which is worthy of further promotion and application in clinical practice.

15.
F1000Res ; 7: 356, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-29770213

RESUMO

Fabry disease is an X-linked genetic deficiency in the alpha-galactosidase enzyme resulting in intracellular accumulation of glycosphingolipids and multisystem organ dysfunction. Typically 50% of males and 20% of affected females have renal involvement, ranging from proteinuria or reduced renal function, renal parapelvic cysts and progressive renal disease ultimately requiring transplantation or dialysis. The phenotypic presentation of Fabry disease is incredibly varied and will even vary between family members with the same confirmed genetic mutation. In a cohort of patients affected by Fabry disease in the North East of England we examine the different phenotypic presentations of eight index cases (6 male, 2 female) with predominantly renal disease and the renal manifestations within their family members. The mean age of presentation was 40 years of age (range 23-59 years). Various multisystem manifestations were observed including cardiac, neurological, cerebrovascular and skin involvement. Two of the male index patients reached end stage renal disease (ESRD) requiring renal replacement therapy. Two female index patients had phenotypes limited to hypertension and proteinuria at presentation and the remaining patients had either stable or progressive chronic kidney disease at the time of diagnosis. We demonstrate the need for a high index of suspicion in order to consider Fabry disease as a diagnosis and the importance of cascade genetic screening to identify affected family members so that treatment can be initiated in a timely fashion.

16.
Urol Int ; 101(3): 366-368, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-28494441

RESUMO

Parapelvic cysts originate in the renal parenchyma and extend into the renal sinus. A series of 3 patients with symptomatic obstructing parapelvic cysts is described, 2 with acute presentations, and 1 with chronic symptoms. In 2 of the 3 cases, there was a significant delay in establishing a diagnosis. Although one individual was successfully managed by image-guided cyst aspiration, the second patient required repeated aspiration due to cyst re-accumulation. A high index of clinical suspicion and a combination of imaging modalities, including serial ultrasound, excretory-phase CT, and MAG3 renogram, are necessary to establish the diagnosis and monitor response to treatment.


Assuntos
Doenças Renais Císticas/terapia , Pelve Renal/patologia , Tomografia Computadorizada por Raios X , Ultrassonografia , Doenças Ureterais/terapia , Cistos/terapia , Humanos , Hidronefrose/patologia , Inflamação , Rim/imunologia , Rim/patologia , Doenças Renais Císticas/diagnóstico , Masculino , Dor/diagnóstico , Renografia por Radioisótopo , Resultado do Tratamento , Doenças Ureterais/diagnóstico , Urografia
17.
Nephrol Dial Transplant ; 33(2): 318-323, 2018 02 01.
Artigo em Inglês | MEDLINE | ID: mdl-28371803

RESUMO

Background: Fabry's disease (FD) is a rare, multi-organ lysosomal disease, caused by the deficiency of the enzyme α-galactosidase A, and is difficult to diagnose. Although parapelvic cysts (PC) were previously associated with FD, their prevalence and significance are unclear. Methods: The present study aimed to: (i) evaluate, by renal ultrasound, the real prevalence of PC and of their determinants in a multicentre, nationwide cohort of FD patients (n = 173, Study 1) and (ii) ascertain whether a greater accuracy of PC detection improved their identification, in FD patients from a single centre (n = 67, Study 2). In both studies, for each FD patient, an age- and renal function-matched subject was selected for comparison (1:1). Results: In Study 1, PC were detected in 28.9% of FD subjects and in only 1.1% of control subjects (P < 0.001). The presence of other renal abnormalities did not differ between the groups, nor differences exist in the main demographic and laboratory parameters between the groups. In Study 2, the greater accuracy of ultrasound increased PC prevalence from 29.8% to 43.3% in the same subjects (P < 0.05). In both studies, no correlation was detected between PC and the main demographic, clinical and biochemical parameters, including use of enzyme replacement therapy (P < 0.1, minimum value). Finally, no difference existed between FD patients with and without PC. Conclusions: The present study suggests that the presence of PC in renal patients should alert physicians to consider the diagnosis of FD, primarily in subjects with an unclear family history of renal disease and in the presence of other stigmata of the disease.


Assuntos
Doença de Fabry/fisiopatologia , Doenças Renais Císticas/diagnóstico , Adulto , Estudos Transversais , Doença de Fabry/diagnóstico por imagem , Feminino , Humanos , Itália/epidemiologia , Doenças Renais Císticas/diagnóstico por imagem , Doenças Renais Císticas/epidemiologia , Masculino , Pessoa de Meia-Idade , Prevalência , Prognóstico , Estudos Retrospectivos , Ultrassonografia/métodos , alfa-Galactosidase/metabolismo
18.
China Journal of Endoscopy ; (12): 96-99, 2017.
Artigo em Chinês | WPRIM (Pacífico Ocidental) | ID: wpr-613602

RESUMO

Objective To observe ransurethral flexible ureteroscopey for two-step precise method treatment of parapelvic cyst. Methods From May 2014 to November 2015, 23 patients of parapelvic cyst underwent a ransurethral flexible ureteroscopey for two-step precise method treatment, then analyze the clinical data of patients, observe the therapeutic effect. Results All patients underwent primary surgery successfully while 4 cases among them were confirmed as urine level puncture calyceal diverticulum treated with flexible ureteroscopy diverticulum expand drainage, the other 19 cases were confirmed as renal cyst and treated with cyst incision drainage successfully. The hemoglobin before and after surgery showed no changes. With no blood transfusion, the average postoperative hospitalization time was 2 d. 1 day after surgery, KUB was to locate the DJ tube position; 1 month later, ultrasound or CT check show that the cyst volume was significantly reduced, postoperative follow-up range from 6 months to 1 year. All the patients had no long-term complications. Conclusion Ransurethral flexible ureteroscopey for two-step precise method treatment of parapelvic cyst and renal pelvis lamp beside diverticulum provide a clear direction for the next operation. It's simple operation with no significant complications and recovered quickly, which is an ideal method for the treatment of renal pelvic.

19.
Nefrologia ; 36(3): 310-2, 2016.
Artigo em Inglês, Espanhol | MEDLINE | ID: mdl-27061865

RESUMO

Fabry disease is an inherited, X-linked lysosomal storage disorder caused by deficiency of the enzyme alpha galactosidase A (alpha-GLA A), which leads to glycosphingolipid accumulation, mainly globotriaosylceramide, in tissues. Disease prevalence and the index of suspicion are both low, which tends to result in delayed diagnosis and treatment. We present the case of a male Fabry disease patient who manifested no angiokeratoma lesions but presented multiple parapelvic cysts and renal failure. The genetic study revealed an alpha-GLA A gene mutation that had not been recorded in the mutations registry. The de novo mutation was not found in his relatives and it was not transmitted to his offspring. The large number and peculiar appearance of the parapelvic cysts led to the diagnosis.


Assuntos
Doença de Fabry/complicações , Doenças Renais Císticas/etiologia , Éxons/genética , Doença de Fabry/diagnóstico , Doença de Fabry/diagnóstico por imagem , Humanos , Doenças Renais Císticas/diagnóstico por imagem , Falência Renal Crônica/etiologia , Pelve Renal/patologia , Masculino , Pessoa de Meia-Idade , Deleção de Sequência , alfa-Galactosidase/genética
20.
Clin Kidney J ; 6(2): 238-9, 2013 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-26019858
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