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J Laparoendosc Adv Surg Tech A 2021 May 27. Epub 2021 May 27.

Division of Pediatric Surgery, Department of Surgery, Taichung Veterans General Hospital, Taichung, Taiwan, Republic of China.

Success rate of laparoscopic pyeloplasty for ureteropelvic junction obstruction (UPJO) in children is comparable with open pyeloplasty. Prolonged ileus and injury to adjacent viscera more often occurred in transperitoneal approach; however, longer operation time is noted in retroperitoneal approach. This study presented a hybrid retroperitoneoscopic pyeloplasty (HRP), for congenital UPJO in infants weighing <10 kg. Read More

IJU Case Rep 2021 May 1;4(3):155-158. Epub 2021 Mar 1.

Department of Urology Tokai University School of Medicine Isehara Kanagawa Japan.

Introduction: Ureteropelvic junction obstruction associated with ureteral duplication is rare, with prevalence reported to be around 2-7%. Ureteropelvic junction obstruction of the lower pole with both complete and incomplete duplex systems is a common cause of obstruction. Here, we report a case of ureteropelvic junction obstruction associated with incomplete ureteral duplication of the solitary kidney successfully treated by pyeloureterostomy. Read More

J Clin Med 2021 Apr 2;10(7). Epub 2021 Apr 2.

Core Unit Proteomics, Hannover Medical School, Institute of Toxicology, Carl-Neuberg-Strasse 1, 30625 Hannover, Germany.

Congenital anomalies of the urinary tract, and particularly of obstructive nephropathy such as ureteropelvic junction obstruction (UPJO) in infants, can later lead to chronic kidney disease and hypertension. Fundamental questions regarding underlying mechanisms remain unanswered. The aim of the present study was to quantitate the systemic amino acids metabolome in 21 UPJO infants requiring surgery (Group A) and 21 UPJO infants under conservative treatment (Group B). Read More

Int J Med Robot 2021 Jun 7;17(3):e2246. Epub 2021 Mar 7.

Department of Urology, Polytechnic University of Marche Region, "Ospedali Riuniti" Hospital, Ancona, Italy.

Objective(s): The incidence of urinary tract stone disease is steadily increasing in both adult and paediatric populations. This condition develops due to different factors: dietary or metabolic alterations, infection, and congenital anatomic malformations. Standard indications and treatments for children are analogous to the ones indicated for adults. Read More

Transl Androl Urol 2021 Jan;10(1):125-133

Department of Urology, Peking University First Hospital, Institute of Urology, Peking University, National Urological Cancer Center, Beijing, China.

Background: The aim of this study was to evaluate the effectiveness and safety of real-time surgical navigation by three-dimensional (3D) virtual reconstruction models in robot-assisted laparoscopic pyeloplasty (RALP).

Methods: Between November 2018 and January 2020, 38 patients with ureteropelvic junction obstruction (UPJO) who underwent RALP were retrospectively enrolled. The operations were assisted in real time by 3D models in 16 patients, while 22 patients underwent surgery without navigation. Read More

Radiographics 2021 Mar-Apr;41(2):462-486. Epub 2021 Jan 29.

From the Department of Radiology, Diagnósticos da América SA (DASA), Av Juruá 434, Alphaville Industrial, Barueri, SP 06455-010, Brazil.

The upper urinary tract is the most common human system affected by congenital anomalies. Congenital anomalies of the kidneys and ureters comprise a wide spectrum of disorders ranging from simple variants with no clinical significance to complex anomalies that may lead to severe complications and end-stage renal disease. They may be classified as anomalies of renal form, which are subclassified as structural anomalies (eg, persistent fetal lobulation, hypertrophied column of Bertin, and dromedary hump) and fusion anomalies (eg, horseshoe kidney and pancake kidney); anomalies of renal position (eg, renal malrotation, simple renal ectopia, and crossed renal ectopia) and renal number (eg, renal agenesis and supernumerary kidney); and abnormalities in development of the urinary collecting system (eg, pyelocaliceal diverticulum, megacalycosis, ureteropelvic junction obstruction, duplex collecting system, megaureter, ectopic ureter, and ureterocele). Read More

Case Rep Urol 2021 4;2021:6655813. Epub 2021 Jan 4.

Center for Anatomical Science and Education, Department of Surgery, Saint Louis University School of Medicine, Saint Louis, MO 63104, USA.

. Urogenital and vascular anomalies, including a left duplex kidney and a left aberrant renal artery that gave rise to the left ovarian artery, were observed in a 77-year-old female cadaver during a routine dissection. . Read More

Case Rep Womens Health 2021 Jan 27;29:e00275. Epub 2020 Nov 27.

Urology, Hospital Selayang, Malaysia.

Adult giant hydronephrosis in a normally sited kidney is unusual during pregnancy. The most frequent cause is congenital obstruction at the ureteropelvic junction. Ultrasound accompanied by magnetic resonance imaging (MRI) are valuable in reaching the diagnosis, especially when clinical assessment of an abdominal mass is inconclusive regarding aetiology. Read More

Urol Clin North Am 2021 Feb 5;48(1):113-125. Epub 2020 Nov 5.

Division of Urology, Children's Hospital of Philadelphia, Perelman School of Medicine, University of Pennsylvania, 3401 Civic Center Boulevard, Philadelphia, PA 19104, USA.

Almost 30 years have passed since the inception of minimally invasive surgery in urology and specifically in pediatric urology. Laparoscopy has now become an essential tool in the pediatric urologic armamentarium. The application of robot-assisted surgery in pediatrics has allowed for widespread utilization for common reconstructive procedures such as pyeloplasty and ureteral reimplantation. Read More

Medicine (Baltimore) 2020 Nov;99(45):e23072

Department of Ultrasound, The Maternal and Child Health Care Hospital of Hunan Province.

Rationale: Hydronephrosis, mostly caused by ureteropelvic junction obstruction, rarely occurs in infants. However, imperforate hymen atresia in female infants may cause hydronephrosis, even though it is rare.

Patient Concerns: A 3-month-old female infant was admitted to our hospital for frequent crying. Read More

Int J Surg Case Rep 2020 23;75:513-516. Epub 2020 Sep 23.

Prince Sultan Military Medical City, Urology Department, Riyadh, Saudi Arabia. Electronic address:

Introduction: Giant hydronephrosis (GH) is a rare urological entity, described as more than 1 L of fluid contained in the renal collecting system. Ureteropelvic junction obstruction (UPJO) is the most common cause. GH if not discovered and managed early can result in long term complications. Read More

Proteomics Clin Appl 2020 11 19;14(6):e2000030. Epub 2020 Oct 19.

Department of Pediatric Urology, Xin hua Hospital Affiliated to Shanghai Jiaotong University School of Medicine, 1665 Kongjiang Road, Shanghai, 200092, China.

Purpose: Ureteropelvic junction obstruction (UPJO) is the most frequent cause of congenital hydronephrosis in child. To better investigate the molecular mechanisms of this pathological process, the stenotic ureter proteome of UPJO in infants is compared with their own normal pre-stenotic segments.

Experimental Design: Data independent acquisition-based proteomics are performed to compare proteome between pre-stenotic and stenotic ureter from nine UPJO infants. Read More

J Indian Assoc Pediatr Surg 2020 Jul-Aug;25(4):231-235. Epub 2020 Jun 24.

Department of Pathology, AFMC, Pune, Maharashtra, India.

Background: The etiopathogenesis of congenital ureteropelvic junction obstruction (UPJO) has been inconclusive in spite of the numerous studies carried out to find the possible causative factor. The results of different studies have been conflicting and contradictory. It has been postulated that the interstitial cells of Cajal (ICC) are the pacemaker cells located in the ureteropelvic junction (UPJ) and regulate the peristalsis in this region. Read More

Am J Med Genet C Semin Med Genet 2020 09 5;184(3):644-655. Epub 2020 Sep 5.

Division of Human Genetics, Department of Pediatrics, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA.

Mosaic genetic mutations may be somatic, germline, or "gonosomal" and have the potential to cause genetic syndromes, disorders, or malformations. Mutations can occur at any point in embryonic development and the timing determines the extent of distribution of the mutation throughout the body and different tissue types. The eye and visual pathway offer a unique opportunity to study somatic and gonosomal mosaic mutations as the eye consists of tissues derived from all three germ layers allowing disease pathology to be assessed with noninvasive imaging. Read More

J Endourol Case Rep 2019 30;5(3):128-130. Epub 2019 Aug 30.

Department of Urology, Loma Linda University Health, Loma Linda, California.

Autosomal dominant polycystic kidney disease is the most prevalent hereditary renal disease, associated with progressive renal insufficiency, usually leading to dialysis. It is rarely diagnosed with other renal abnormalities. We present a case of a 35-year-old woman with a duplicated left polycystic kidney, who had recurrent pain and pyelonephritis because of ureteropelvic junction (UPJ) obstruction of the upper moiety. Read More

Bosn J Basic Med Sci 2021 Jun 1;21(3):313-322. Epub 2021 Jun 1.

Department of Pediatric General Thoracic Surgery, Affiliated Hospital of Zunyi Medical University, Zunyi, China.

Congenital hydronephrosis caused by ureteropelvic junction obstruction (UPJO) eventually leads to renal interstitial fibrosis and atrophy, after a series of pathophysiological problems. Renal repair after injury depends on renal stem cells. This study aimed to determine the expression of renal stem cell marker CD133 in children of different ages and the regulatory effect of stem cell microenvironment. Read More

Dis Markers 2020 29;2020:9520309. Epub 2020 Jun 29.

Department of Pediatric Nephrology Medical University of Lublin, Gębali 6, 20-093 Lublin, Poland.

Renal tubulointerstitial fibrosis caused by congenital ureteropelvic junction obstruction (UPJO) may lead to the development of obstructive nephropathy (ON) and the impairment of kidney function. Hence, the identification of early biomarkers of this condition might be of assistance in therapeutic decisions. This study evaluates serum and urinary metalloproteinases MMP-1, MMP-2, and MMP-9 and tissue inhibitors of metalloproteinases TIMP-1 and TIMP-2 as potential biomarkers of ON in children with congenital unilateral hydronephrosis (HN) caused by UPJO. Read More

Ital J Pediatr 2020 Jul 9;46(1):95. Epub 2020 Jul 9.

Department of Pediatric, The Affiliated Hospital of Qingdao University, Qingdao, 266003, Shandong, China.

Background: MED13L-related intellectual disability is a new syndrome that is characterized by intellectual disability (ID), motor developmental delay, speech impairment, hypotonia and facial dysmorphism. Both the MED13L haploinsufficiency mutation and missense mutation were reported to be causative. It has also been reported that patients carrying missense mutations have more frequent epilepsy and show a more severe phenotype. Read More

Front Pediatr 2020 27;8:242. Epub 2020 May 27.

University of Texas Southwestern Medical Center, Dallas, TX, United States.

In the last decade, management of congenital UPJ obstruction has become progressively observational despite the lack of precise predictors of outcome. While it is clear that many children will have resolution of their hydronephrosis and healthy kidneys, it is equally clear that there are those in whom renal functional development is at risk. Surgical intervention for the young infant, under 6 months, has become relatively infrequent, yet can be necessary and poses unique challenges. Read More

Exp Cell Res 2020 08 13;393(1):112086. Epub 2020 May 13.

Department of Urology, Nanfang Hospital, Southern Medical University, China. Electronic address:

Objective: Ureteropelvic junction obstruction (UPJO) is a common renal obstructive disorder, but its pathogenic mechanisms remain largely unclear. We aimed to investigate the potential involvement of the renin-angiotensin system in congenital UPJO pathogenesis.

Methods: Differentially expressed proteins in exosomes isolated from amniotic fluid of patients with congenital UPJO were characterized using iTRAQ (isobaric tags for relative and absolute quantification)-based proteomics. Read More

J Pediatr Urol 2020 Oct 10;16(5):597.e1-597.e6. Epub 2020 Apr 10.

Pediatric Urology The Children's Hospital of Michigan 3901 Beaubien Detroit, MI 48201, USA. Electronic address:

Background: In December 2014, Nguyen et al. introduced the Upper Tract Dilation (UTD) classification scheme, hoping to unify multiple disciplines when describing ultrasound imaging of congenital hydronephrosis. We hypothesized that the academic community has been slow to adopt its use in publications. Read More

J Int Med Res 2020 Apr;48(4):300060520918781

Department of Nephrology, Children's Hospital of Nanjing Medical University, Nanjing, China.

Objectives: We aimed to investigate the safety concerns associated with placing double-J ureteric stents post-laparoscopic pyeloplasty surgery for congenital ureteropelvic junction obstruction (UPJO) and hydronephrosis.

Methods: A total of 1349 patients with postoperative double-J stent placement at our center were included. Clinical variables for enrolled patients were collected by two independent authors. Read More

Pediatr Med Chir 2019 Dec 19;41(2). Epub 2019 Dec 19.

Pediatric Surgery and Urologic Unit, Local Health Unit 8, S. Bortolo Hospital, Vicenza.

The hydronephrosis, characterized by the dilation of the renal pelvicalyceal system with possible functional damage to the renal parenchyma, is the most common congenital abnormality of the urinary system detected in utero through the prenatal ultrasound screening. (.. Read More

Front Pediatr 2020 7;8:137. Epub 2020 Apr 7.

Gaziantep Maternity and Children's Hospital, Pediatric Urology, Gaziantep, Turkey.

Kidney parenchyma and collecting system arise from two different embryologic units as a result of a close interaction between them. Therefore, their congenital abnormalities are classified together under the same heading named CAKUT (congenital abnormalities of the kidney and urinary tract). The pathogenesis of CAKUT is thought to be multifactorial. Read More

Int J Urol 2020 May 11;27(5):369-376. Epub 2020 Mar 11.

Committee for the Formulation of Medical Management Guide for Pediatric Congenital Hydronephrosis (ureteropelvic junction obstruction), Academic Committee, Japanese Society of Pediatric Urology, Osaka, Japan.

The prevalence of asymptomatic hydronephrosis, now detected by ultrasonography, has increased. However, definitive management guidelines for the management of congenital hydronephrosis have not been established. The Japanese Society of Pediatric Urology created a "medical management guide" based on new findings for physicians practicing pediatric urology. Read More

Ultrasound Q 2020 Mar;36(1):20-23

Department of Pediatric Surgery, The First Affiliated Hospital of Sun Yat-Sen University, Guangzhou, China.

Objective: The aims of the study were to measure renal hemodynamic indexes and to evaluate postsurgical recovery in children diagnosed with obstructive hydronephrosis using color Doppler ultrasound.

Methods: This study enrolled 36 patients with ureteropelvic junction obstruction who underwent Anderson-Hynes pyeloplasty. The peak systolic velocity (PSV) and the resistive index (RI) of the main renal arteries (MRAs) and interlobar renal arteries (IRAs) were measured using color Doppler ultrasound. Read More

J Pediatr Surg 2020 Aug 11;55(8):1621-1625. Epub 2020 Feb 11.

National Children's Research Centre, Our Lady's Children's Hospital, Dublin, Ireland; School of Medicine and Medical Science and Conway Institute of Biomolecular and Biomedical Research, University College Dublin, Ireland. Electronic address:

Purpose: Ureteropelvic junction (UPJ) obstruction is the most common cause of congenital hydronephrosis in children. The pathophysiology of UPJ obstruction and the exact mechanism of pelviureteral peristalsis are poorly understood. Anoctamin-1 (ANO1), a Ca-activated chloride channel, has been shown to play a key role in muscle wall contractions in the gastrointestinal tract. Read More

Mol Genet Genomic Med 2020 04 14;8(4):e1183. Epub 2020 Feb 14.

Norton Children's Urology, Norton Healthcare, Louisville, KY, USA.

Background: Congenital aniridia involves total or partial hypoplasia of the iris and is due to a deficiency in PAX6 gene expression. WAGR syndrome is comprised of Wilms tumor, aniridia, genitourinary abnormalities, and intellectual disability. Numerous genitourinary pathologies may be associated with WAGR syndrome, necessitating an evaluation of the genitourinary anatomy. Read More

Clin Nephrol 2020 Apr;93(4):195-202

Aim: By observing the expression and distribution of platelet-derived growth factor receptor α-positive (PDGFRα) cells in ureteropelvic junction obstruction (UPJO), to explore their role in the pathogenesis of children with congenital hydronephrosis.

Materials And Methods: The control group involved specimens of the normal ureter (nephrectomy for tumor; n = 10), and the UPJO group contained specimens of ureteropelvic junction (UPJ) segment excised during pyeloplasty (n = 30). The specimens were investigated using immunofluorescence for the expression and distribution of PDGFRα cells in each group by light microscopy with computerized image analysis. Read More