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https://www.readbyqxmd.com/read/28735900/treatment-with-enzyme-replacement-therapy-during-pregnancy-in-a-patient-with-pompe-disease
#1
Merete Holbeck-Brendel, Birgitte Klindt Poulsen
Pregnancy is in general physically demanding, even more so for women with hereditary muscular diseases (HMDs). With increasing numbers of women with HMD reaching reproductive age, there is a growing need for research into what impact pregnancy can have on their clinical condition. A 25-year-old woman was diagnosed with Pompe disease at the age of 22 and began enzyme replacement therapy (ERT) right away. At the age of 25 she became pregnant. ERT was paused during the first trimester and recommenced throughout the second and third trimesters...
July 5, 2017: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/28734863/significant-linkage-evidence-for-interstitial-cystitis-painful-bladder-syndrome-on-chromosome-3
#2
Kristina Allen-Brady, Kerry Rowe, Melissa Cessna, Sara Lenherr, Peggy Norton
PURPOSE: Interstitial cystitis/painful bladder syndrome (IC/PBS) is a chronic pelvic pain condition with unknown etiology. We hypothesized that related IC/PBS cases were more likely to have a genetic etiology. The purpose of this study was to perform a genetic linkage analysis. MATERIALS AND METHODS: IC/PBS cases were identified using diagnostic codes linked to a population-based genealogy resource (Utah Population Database) and electronic medical records. For this analysis, 13 high-risk pedigrees (defined as having a statistical excess number of IC/PBS cases among descendants compared to matched hospital population rates) were used...
July 19, 2017: Journal of Urology
https://www.readbyqxmd.com/read/28729970/severe-cyclophosphamide-related-hyponatremia-in-a-patient-with-acute-glomerulonephritis
#3
Pasquale Esposito, Maria Valentina Domenech, Nicoletta Serpieri, Marta Calatroni, Ilaria Massa, Alessandro Avella, Edoardo La Porta, Luca Estienne, Elena Caramella, Teresa Rampino
Cyclophosphamide is frequently used to treat cancer, autoimmune and renal diseases, such as rapidly progressive glomerulonephritis. Its side effects are well-known, including bone marrow depression, infections, alopecia, sterility, bladder malignancy and hemorrhagic cystitis. Moreover, in some cases cyclophosphamide use has been related to the onset of hyponatremia, by development of a syndrome of inappropriate antidiuresis. Indeed, severe hyponatremia has been previously reported in patients treated with high-dose or moderate-dose of intravenous cyclophosphamide, while only few cases have been reported in patients treated with low dose...
July 6, 2017: World Journal of Nephrology
https://www.readbyqxmd.com/read/28729638/inhibition-of-microrna-214-promotes-epithelial-mesenchymal-transition-process-and-induces-interstitial-cystitis-in-postmenopausal-women-by-upregulating-mfn2
#4
Jian-Wei Lv, Wei Wen, Chen Jiang, Qi-Bo Fu, Yin-Jun Gu, Ting-Ting Lv, Zhen-Dong Li, Wei Xue
Our study aims to investigate the roles that microRNA-214 (miR-214) plays in the epithelial mesenchymal transition (EMT) process and the development of interstitial cystitis (IC) in postmenopausal women by targeting Mitofusin 2 (Mfn2). IC bladder tissues and adjacent normal bladder tissues were collected from postmenopausal women. Immunohistochemistry (IHC) staining was conducted. The target relationship between miR-214 and Mfn2 was determined by a dual luciferase reporter gene assay. Adipose-derived mesenchymal stem cells (ADMSCs) were extracted from postmenopausal rats and assigned to the blank, mimics, miR-214 inhibitors, mimics negative control (NC), inhibitors NC, Mfn2 siRNA, miR-214 inhibitors and Mfn2 siRNA groups...
July 21, 2017: Experimental & Molecular Medicine
https://www.readbyqxmd.com/read/28727973/pediatric-xanthogranulomatous-cystitis-the-first-described-case-in-a-pre-pubertal-male-and-review-of-the-literature
#5
Janae Preece, Vinay Prasad, Daryl McLeod
Xanthogranulomatous cystitis (XC) is a rare condition affecting the bladder thought to be caused by chronic inflammation. Fewer than 30 cases have been reported in the literature since being first described in 1932. We present the first reported case of XC in a pre-pubertal male and review the literature. A 10-year-old boy presenting with dysuria subsequently developed frequency. After failure of conservative management, an ultrasound of the bladder revealed a mass. The patient underwent partial cystectomy...
July 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/28727444/fosfomycin-biosynthesis-via-transient-cytidylylation-of-2-hydroxyethylphosphonate-by-the-bifunctional-fom1-enzyme
#6
Su-Hee Cho, Seung-Young Kim, Takeo Tomita, Taro Shiraishi, Jin-Soo Park, Shusuke Sato, Fumitaka Kudo, Tadashi Eguchi, Nobutaka Funa, Makoto Nishiyama, Tomohisa Kuzuyama
Fosfomycin is a wide-spectrum phosphonate antibiotic that is used clinically to treat cystitis, tympanitis, etc. Its biosynthesis starts with the formation of a carbon-phosphorus bond catalyzed by the phosphoenolpyruvate phosphomutase Fom1. We identified an additional cytidylyltransferase (CyTase) domain at the Fom1 N-terminus in addition to the phosphoenolpyruvate phosphomutase domain at the Fom1 C-terminus. Here, we demonstrate that Fom1 is bifunctional and that the Fom1 CyTase domain catalyzes the cytidylylation of the 2-hydroxyethylphosphonate (HEP) intermediate to produce cytidylyl-HEP...
July 20, 2017: ACS Chemical Biology
https://www.readbyqxmd.com/read/28727330/-lower-urinary-tract-infections-in-men-urethritis-cystitis-and-prostatitis
#7
Marie-Céline Zanella, Fabian Schoofs, Benedikt Huttner, Angela Huttner
Recommendations for the treatment of lower non-catheter-related urinary tract infection (UTI) in men are rarely evidence-based. Their management requires the localization of the site of infection, whether it be the urethra, bladder or prostate, and includes antibiotic therapy and in most cases urological assessment. They are often associated with urinary tract procedures or anatomical or functional abnormalities. Nearly 80 % of male UTIs are caused by Enterobacteriaceae. The prevalence of broad-spectrum beta-lactamase-producing strains (ESBL) and quinolone-resistant strains is increasing...
April 12, 2017: Revue Médicale Suisse
https://www.readbyqxmd.com/read/28725616/chronic-orchialgia-epidemiology-diagnosis-and-evaluation
#8
REVIEW
John T Sigalos, Alexander W Pastuszak
Chronic orchialgia is a vexing condition defined as chronic or intermittent scrotal pain lasting at least three months that significantly interferes with daily activities. There are currently no guidelines regarding the diagnosis and management of this condition despite it being the cause of 2.5-4.8% of urologic clinic visits. Men often present with chronic orchialgia in their mid to late 30s, although the condition can present at any age. A broad differential diagnosis of chronic orchialgia includes epididymitis, testicular torsion, tumors, obstruction, varicocele, epididymal cysts, hydrocele, iatrogenic injury following vasectomy or hernia repair, and referred pain from a variety of sources including mid-ureteral stone, indirect inguinal hernia, aortic or common iliac artery aneurysms, lower back disorders, interstitial cystitis, and nerve entrapment due to perineural fibrosis; approximately 25-50% of chronic orchialgia is idiopathic in nature...
May 2017: Translational Andrology and Urology
https://www.readbyqxmd.com/read/28717279/cystitis-glandularis-management-and-challenges-in-a-renal-transplant-recipient
#9
Himanshu Agarwal, Mukund G Andankar, Viswanath Billa
Cystitis cystica or glandularis is a clinical and pathological entity of the bladder mucosa occurring secondary to inflammation or chronic obstruction. Its premalignant nature remains controversial, especially in an immunocompromised transplant recipient. We present a rare case where a chronic kidney disease patient was found to have cystitis glandularis while being worked up for living-related donor renal transplant and describe its subsequent management.
July 2017: Indian Journal of Urology: IJU: Journal of the Urological Society of India
https://www.readbyqxmd.com/read/28717088/long-term-effects-of-intravenous-cyclophosphamide-in-combination-with-mesna-provided-intravenously-and-via-bladder-perfusion-in-a-patient-with-severe-multifocal-motor-neuropathy
#10
Eri Iwasawa, Takuya Ohkubo, Tadashi Kanouchi, Takashi Kanda, Hidehiro Mizusawa, Takanori Yokota
A 25-year-old woman presenting with progressive muscle weakness in the distal extremities in the absence of sensory involvement for 2 years was diagnosed with multifocal motor neuropathy (MMN). Her disease was difficult to manage with various immunosuppressants, and the muscle weakness eventually progressed to involve the respiratory muscles, necessitating mechanical ventilation. Intravenous cyclophosphamide (CY) dramatically improved her symptoms, and she has since maintained her ambulatory status for 18 years with intermittent CY therapy...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28713429/treatment-of-severe-refractory-hematuria-due-to-radiation-induced-hemorrhagic-cystitis-with-dexamethasone
#11
José Carlos Rodrigues Nascimento, Márcio Wilker Soares Campelo, Iuri Arruda Aragão, José Fernando Bastos de Moura, Lúcio Flávio Gonzaga Silva, Reinaldo Barreto Oriá
Treatment of pelvic neoplasms with radiotherapy may develop sequelae, especially RHC. An 85-year-old male patient was admitted to a hospital emergency with gross hematuria leading to urinary retention and was diagnosed with RHC. The urinary bladder was probed, unobstructed, and maintained in continuous three-way saline irrigation. During 45 days of hospitalization, the patient underwent two cystoscopic procedures for urinary bladder flocculation, whole blood transfusions, and one platelet apheresis. None of these interventions led to clinical resolution...
2017: Case Reports in Medicine
https://www.readbyqxmd.com/read/28713083/regional-features-of-the-microbial-landscape-of-urine-and-comparative-evaluation-of-antimicrobial-therapy-in-children-with-cystitis
#12
Tetyana O Kryuchko, Vira P Kharshman, Inna M Nesina, Nataliia V Kuzmenko, Jaroslava M Oleksiienko
INTRODUCTION: Growing resistance of pathogens to antibiotics, including cross-resistance to other antimicrobial classes that are used in the treatment of recurrent infections of the lower urinary system in children demands constant control of issues of regional antibiotic resistance. In the present days, in the empirical treatment of such patients physicians still choose medications with preserved activity in relation to E. coli. The aim of our study was to investigate the regional features of microbial landscape of urine in children with cystitis and study the efficacy of 7-day administration of Furamag medicinal drug for the treatment of recurrent episodes of this disease in children...
2017: Wiadomości Lekarskie: Organ Polskiego Towarzystwa Lekarskiego
https://www.readbyqxmd.com/read/28707385/risk-factors-of-lower-urinary-tract-syndrome-among-ketamine-users
#13
I-Chun Chen, Ming-Huei Lee, Wei-Chih Chen, Tsung-Ching Hu, Hsiu-Ying Lin
OBJECTIVE: This study investigated the risk factors of ketamine associated-lower urinary tract symptoms (LUTS), such as duration of use, dosage of ketamine, co-occurring substance use of other psychoactive drugs, comorbidities, and depression. METHODS: This study was a cross-sectional survey. LUTS was assessed with the O'Leary symptom and problem index (OSPI) scores. We included the comorbidities of interstitial cystitis/painful bladder syndrome (IC/PBS) as comorbid factors...
July 13, 2017: Lower Urinary Tract Symptoms
https://www.readbyqxmd.com/read/28702363/a-case-of-bacillus-calmette-gu%C3%A3-rin-cystitis-diagnosed-with-a-novel-loop-mediated-isothermal-amplification-method
#14
Kazuyoshi Tachi, Akinori Sato, Yuji Kouzaki, Takuya Maeda, Akihiko Kawana, Tomohiko Asano
Intravesical bacillus Calmette-Guérin (BCG) instillation is broadly used to prevent bladder cancer recurrence or to treat carcinoma in situ. BCG infection is rare but can cause serious problems because this strain has intrinsic resistance to pyrazinamide, a first-line anti-tuberculosis drug. Furthermore, there had been no specific and easy procedure accurately diagnosing BCG infection. In this case report we present the first case of BCG cystitis diagnosed with a newly developed easy-to-use diagnostic procedure using the loop-mediated isothermal amplification method...
September 2017: Urology Case Reports
https://www.readbyqxmd.com/read/28702160/bk-virus-associated-hemorrhagic-cystitis-in-patients-with-allogeneic-hematopoietic-cell-transplantation-report-of-three-cases
#15
Duygu Mert, Hikmetullah Batgi, Alparslan Merdin, Sabahat Çeken, Mehmet Sinan Dal, Emre Tekgündüz, Fevzi Altuntaş, Mustafa Ertek
BK viras is a human polyoma viras. It is acquired in early childhood and remains life-long latent in the genitourinary system. BK virus replication is more common in receiving immunosuppressive therapy receiving patients and transplant patients. BK virus could cause hemorrhagic cystitis in patients with allogeneic stem cell transplantation. Hemorrhagic cystitis is a serious complication of hematopoietic stem cell transplantation. Hemorrhagic cystitis could cause morbidity and long stay in the hospital. Diagnosis is more frequently determined by the presence of BK virus DNA detected with quantitative or real-time PCR testing in serum or plasma and less often in urine...
June 1, 2017: Hematology Reports
https://www.readbyqxmd.com/read/28698872/proliferation-of-interstitial-cells-in-the-cyclophosphamide-induced-cystitis-and-the-preventive-effect-of-imatinib
#16
Maria Sancho, Domingo Triguero, Aranzazu Lafuente-Sanchis, Angeles Garcia-Pascual
Cyclophosphamide- (CYP-) induced cystitis in the rat is a well-known model of bladder inflammation that leads to an overactive bladder, a process that appears to involve enhanced nitric oxide (NO) production. We investigated the changes in the number and distribution of interstitial cells (ICs) and in the expression of endothelial NO synthase (eNOS) in the bladder and urethra of rats subjected to either intermediate or chronic CYP treatment. Pronounced hyperplasia and hypertrophy of ICs were evident within the lamina propria and in the muscle layer...
2017: BioMed Research International
https://www.readbyqxmd.com/read/28687365/efficacy-of-botulinum-toxin-a-for-the-treatment-of-bladder-pain-syndrome-a-systematic-review
#17
D C Ochoa Vargas, H A García Perdómo
OBJECTIVES: To determine the efficacy and safety of BTX-A, compared with other interventions for the treatment of BPS to improve quality of life. METHODS: This systematic review fulfils all the requirements of the Cochrane manual and PRISMA reporting guidelines. The PROSPERO registration number is: CRD42016039480.Clinical trials without language discrimination were included. BPS patients over 18 y/o that were treated with BTX-A were included. Studies were searched in published databases and no published literature from inception to the present day...
July 4, 2017: Actas Urologicas Españolas
https://www.readbyqxmd.com/read/28679185/the-role-of-an-herbal-agent-in-treatment-for-escherichia-coli-induced-bacterial-cyctitis-in-rats
#18
Murat Tuken, Mustafa Zafer Temiz, Emrah Yuruk, Asuman Orcun Kaptanagasi, Kayhan Basak, Fehmi Narter, Ahmet Yaser Muslumanoglu, Kemal Sarica
OBJECTIVES: The aim of this study is to evaluate the effects of the herbal agent in the prevention and treatment of bacterial cystitis in a rat model. MATERIAL AND METHODS: A total of twenty-eight male Sprague- Dawley rats were divided into four groups. Group-1 constituted the control group (operated and normal saline injected into the bladder, received only drinking water for 7 days); Group-2 constituted the no-treatment group (operated, E.coli J96 strain injected into the bladder, received only drinking water for 7 days); Group-3 constituted the short-term treatment (operated, E...
June 30, 2017: Archivio Italiano di Urologia, Andrologia
https://www.readbyqxmd.com/read/28654851/a-colovesical-fistula-with-a-persistent-descending-mesocolon-due-to-partial-situs-inversus-a-case-report
#19
Tetsuya Mochizuki, Hirofumi Tazawa, Yuzo Hirata, Yoshio Kuga, Tomohiro Miwata, Sotaro Fukuhara, Kouki Imaoka, Seiji Fujisaki, Mamoru Takahashi, Saburo Fukuda, Toshihiro Nishida, Hideto Sakimoto
INTRODUCTION: Situs inversus viscerum, a congenital condition in which the visceral organs are a mirror image of their normal physiological positions, could be total or partial. Persistent descending mesocolon (PDM) is a congenital anomaly that is asymptomatic because of its short length. PDM causing intestinal obstruction is a known clinical complication. PRESENTATION OF CASE: A 74-year-old woman presented with pneumaturia and enteruria for two months, and recurrent cystitis for a month...
June 17, 2017: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/28652164/schistosoma-haematobium-a-delayed-cause-of-hematuria
#20
Wei Phin Tan, Thomas Hwang, Ji-Weon Park, Lev Elterman
A 22-year-old African-American man with recurrent episodes of gross hematuria for 6 months presented to the clinic for evaluation. A thorough history revealed that the patient emigrated from Mozambique to the United States 12 years ago. Urine culture was negative for a urinary tract infection. Cystoscopy revealed 4 lesions in the bladder. Biopsy of the bladder lesion revealed severe cystitis and Schistosoma Haematobium. The patient later confirmed that he used to swim in rivers and streams back in Africa. He completed a course of praziquantel and his gross hematuria resolved...
June 23, 2017: Urology
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