Potential pharmacotherapy for Zinner syndrome.
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ABSTRACT:
SUBMITTER: Takeshima T
PROVIDER: S-EPMC10406536 | biostudies-literature | 2023 Jul
REPOSITORIES: biostudies-literature
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Potential pharmacotherapy for Zinner syndrome.
Translational andrology and urology 20230713 7
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Project description:This is the case of a 45-year-old male who presented with dysuria and gross hematuria. Ultrasonography and computed tomography revealed an enormous pelvic cystic lesion and a right nephromegaly but an invisible left kidney. Using the imaging findings, the patient was diagnosed with Zinner syndrome. Transperitoneal laparoscopic excision of the cyst was performed for symptom relief. At post-treatment, the symptoms disappeared, and sexual function remained. Seminal vesicle cysts have been commonly reported in previous studies but rarely in this case. Furthermore, most previous studies have documented surgical excision of <10 cm cysts. However, a ∼12.5-cm seminal vesicle cyst was excised with the laparoscopic method without any perioperative complication in this case.
| S-EPMC10120364 | biostudies-literature
Project description:Introduction and importanceTo our knowledge, this is the first reported case of Robot-Assisted Radical Prostatectomy (RARP) in a patient with Zinner syndrome, a rare congenital condition characterized by unilateral renal agenesis, seminal vesicle cysts, and ejaculatory duct obstruction.Case presentationA 64-year-old male with elevated PSA was diagnosed with low-risk prostate cancer. Preoperative imaging confirmed Zinner syndrome, posing unique anatomical challenges. The procedure was successfully performed using the Da Vinci robotic system, allowing precise removal of the prostate, non-functioning kidney, and dilated ureter from the standard port setting without changing the patient's position. The surgery was completed with minimal complications, and the patient recovered well.Clinical discussionThe anatomical anomalies associated with Zinner syndrome complicate standard surgical approaches. Robotic-assisted techniques provide enhanced visualization and precision, facilitating safe and effective management of these cases.ConclusionThis urological procedure can be safely and efficiently performed on patients with Zinner Syndrome and synchronous prostate cancer by urological surgeons with extensive experience in robotic surgery in high-volume centers.
| S-EPMC11848090 | biostudies-literature
Project description:BackgroundZinner syndrome is a rare congenital malformation of the male genitourinary system, characterized by a triad: seminal vesicle cyst, unilateral renal agenesis, and ipsilateral ejaculatory duct obstruction. The etiology of this uncommon disease remains largely elusive; however, genetic mutations may contribute to its development. In this report, we present a case of symptomatic Zinner syndrome that was surgically treated, alongside an investigation into the potential genetic basis of the syndrome via whole exome sequencing.Case presentationWe report the case of an 18-year-old male presenting with urinary pain and was diagnosed with right renal agenesis and a left seminal vesicle cyst following comprehensive imaging. The patient also experienced perineal pain and urgency, without symptoms of frequent urination, dysuria, or hematuria, and no familial history of genitourinary anomalies was documented. He successfully underwent laparoscopic resection of a pelvic mass, with pathological examination confirming a seminal vesicle cyst. Postoperative recovery was uneventful. Whole exome sequencing of blood and tissue samples highlighted myeloma overexpressed gene (MYEOV), B melanoma antigen family member (BAGE), and N-acetylated-alpha-linked acidic dipeptidase 2 (NAALAD2) as potential mutated genes related to Zinner syndrome. Additionally, two predisposing genetic variants were identified.ConclusionsZinner syndrome is a rare condition commonly diagnosed via various imaging modalities. Surgical resection remains the most effective treatment for symptomatic cases. Gene sequencing provides valuable insights into the genetic etiology of Zinner syndrome, enhancing our understanding and potentially guiding future diagnostic approaches.
| S-EPMC11895205 | biostudies-literature
Project description:Congenital malformations of the seminal vesicle are uncommon, and most of them are cystic malformations. If an insult occurs during the first trimester of gestation, the embryogenesis of the kidney, ureter, seminal vesicle, and vas deferens could be altered. The mutual embryological origins of the seminal vesicle and ureteral bud from the mesonephric (Wolffian) duct result in association between ipsilateral renal agenesis and seminal vesical cysts. Zinner syndrome is a rare condition comprising a triad of unilateral renal agenesis, ipsilateral seminal vesicle obstruction and ipsilateral ejaculatory duct obstruction. This syndrome were first described by Zinner in 1914, and 200 cases have been reported in the literature. Most patients with this anomaly are asymptomatic until the second or third decade of life. Some cases have nonspecific symptoms such as prostatism, urinary urgency, dysuria, painful ejaculation, and perineal discomfort. In this paper, we present a uncommon case of a 21-year-old patient which the initial presentation of this condition was intermittent scrotal pain. A brief review of the literature is undertaken, regarding the main clinical, imaging implications, and the developmental anomalies that are involved in this congenital anomaly.
| S-EPMC6148829 | biostudies-literature
Project description:Introductionand importance: Zinner syndrome is a rare congenital malformation of the seminal vesicles and the homolateral upper urinary tract. While the majority of patients remain asymptomatic and are discovered incidentally, others present symptoms such as micturition or ejaculatory difficulties, or pain. We report a case of Zinner syndrome in a 32-year-old patient with painful ejaculation and discuss the diagnosis and treatment difficulties.Case presentationA 32-year-old married patient was consulted for pelvic pain associated with painful ejaculation that had been evolving for six months. The clinical examination was normal. Routine laboratory studies of blood and urine were normal. The patient was explored by ultrasound which showed the absence of the right kidney and the presence of a 7 cm right lateral prostatic cystic mass. On MRI, the right kidney was not visualized. Multiple cysts were seen in the right seminal vesicle. Surgical excision of the cyst by laparotomy was performed. The patient had an uneventful recovery and was discharged on the third postoperative day.Clinical discussionCongenital malformations of the seminal vesicles are often associated with those of the ipsilateral upper urinary tract, as the ureteral and seminal vesicle buds originate from the mesonephric duct. The syndrome often occurs in the second and third decades of life, especially after the onset of sexual activity. The most common symptoms were dysuria, perineal pain, epididymitis, and painful ejaculation. Diagnostic modalities include ultrasound, MRI, and cystoscopy. In patients with symptoms, the therapeutic management of the cyst includes ultrasound-guided aspiration and laparoscopic or open surgical excision.ConclusionSeminal vesicle cysts associated with homolateral renal agenesis or hypoplasia are a rare urologic anomaly. The treatment depends on the patient's symptoms. surgical excision of seminal vesicle cysts may be needed for large cysts causing obstructive symptoms.
| S-EPMC9289330 | biostudies-literature
Project description:Hutchinson-Gilford progeria syndrome (HGPS), commonly called progeria, is an extremely rare disorder that affects only one child per four million births. It is characterized by accelerated aging in affected individuals leading to premature death at an average age of 14.5 years due to cardiovascular complications. The main cause of HGPS is a sporadic autosomal dominant point mutation in LMNA gene resulting in differently spliced lamin A protein known as progerin. Accumulation of progerin under nuclear lamina and activation of its downstream effectors cause perturbation in cellular morphology and physiology which leads to a systemic disorder that mainly impairs the cardiovascular system, bones, skin, and overall growth. Till now, no cure has been found for this catastrophic disorder; however, several therapeutic strategies are under development. The current review focuses on the overall progress in the field of therapeutic approaches for the management/cure of HGPS. We have also discussed the new disease models that have been developed for the study of this rare disorder. Moreover, we have highlighted the therapeutic application of extracellular vesicles derived from stem cells against aging and aging-related disorders and, therefore, suggest the same for the treatment of HGPS.
| S-EPMC7205988 | biostudies-literature
Project description:This case presents an unusual variation of Zinner syndrome with a patient found to have an ectopic ureteral insertion of the seminal vesicle from a supernumerary pelvic kidney and complete duplication of the ipsilateral, otherwise normal renal unit. The case offers an interesting variant of a previously established syndrome with successful treatment involving robotic resection of an ectopic ureter and seminal vesiculectomy, resulting in resolution of the patient's symptoms.
| S-EPMC7171451 | biostudies-literature
Project description:BackgroundZinner syndrome is a rare congenital anomaly featuring a unilateral seminal vesicle cyst and ipsilateral renal agenesis. While the majority of affected patients are asymptomatic and followed with conservative management, others have symptoms such as micturition, ejaculatory difficulties, and/or pain, thus may require treatment. These patients often undergo an invasive procedure as first-line treatment, such as transurethral resection of the ejaculatory duct, or aspiration and drainage, which reduces pressure within the seminal vesicle cyst, or surgical resection of the seminal vesicle. Reported here is a patient with ejaculation pain and pelvic discomfort associated with Zinner syndrome who was successfully treated in a non-invasive manner with silodosin, an α1-adrenoceptor antagonist.Case descriptionA 37-year-old Japanese male had ejaculation pain and pelvic discomfort associated with Zinner syndrome. Two months of treatment with silodosin, an α1-blocker, resulted in complete pain relief. Thereafter, conservative management with regular follow-up examinations has been conducted for five years, without recurrence of ejaculation pain or other symptoms associated with Zinner syndrome.ConclusionsThis is the first known published case report of a patient with Zinner syndrome treated with silodosin who was completely relieved from ejaculation pain. The effect of α1-adrenoceptor antagonists to inhibit seminal vesicle contraction, as well as cause relaxation of smooth muscles of the urethra and prostate may contribute to reduce pain associated with ejaculation. We concluded that silodosin treatment should be attempted in affected patients before considering surgical treatment.
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Project description:On 11 March 2020, the coronavirus disease (COVID-19) was defined by the World Health Organization as a pandemic. Severe acute respiratory syndrome-2 (SARS-CoV-2) is the newly evolving human coronavirus infection that causes COVID-19, and it first appeared in Wuhan, China in December 2019 and spread rapidly all over the world. COVID-19 is being increasingly investigated through virology, epidemiology, and clinical management strategies. There is currently no established consensus on the standard of care in the pharmacological treatment of COVID-19 patients. However, certain medications suggested for other diseases have been shown to be potentially effective for treating this infection, though there has yet to be clear evidence. Therapies include new agents that are currently tested in several clinical trials, in addition to other medications that have been repurposed as antiviral and immune-modulating therapies. Previous high-morbidity human coronavirus epidemics such as the 2003 SARS-CoV and the 2012 Middle East respiratory syndrome coronavirus (MERS-CoV) prompted the identification of compounds that could theoretically be active against the emerging coronavirus SARS-CoV-2. Moreover, advances in molecular biology techniques and computational analysis have allowed for the better recognition of the virus structure and the quicker screening of chemical libraries to suggest potential therapies. This review aims to summarize rationalized pharmacotherapy considerations in COVID-19 patients in order to serve as a tool for health care professionals at the forefront of clinical care during this pandemic. All the reviewed therapies require either additional drug development or randomized large-scale clinical trials to be justified for clinical use.
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