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1.
BMC Pregnancy Childbirth ; 21(1): 715, 2021 Oct 26.
Artigo em Inglês | MEDLINE | ID: mdl-34702216

RESUMO

BACKGROUND: Pregnancy in a rudimentary horn is an extremely rare type of ectopic pregnancy. A rudimentary uterine horn pregnancy is associated with a risk of spontaneous rupture and bleeding during surgery due to the increased uterine blood flow. Recent advances in imaging modalities have enabled laparoscopic surgery to be performed in cases without rupture in the early stages of pregnancy. However, there are few reports of successful pregnancies and deliveries after treatment of rudimentary horn pregnancies. We report the successful management of a case of non-communicating rudimentary horn pregnancy by local injection of methotrexate followed by complete laparoscopic excision along with a review of the literature. CASE PRESENTATION: The patient was a 29-year-old Japanese woman, gravida 2, nullipara. She was diagnosed with a left unicornuate uterus with a right non-communicating rudimentary horn on hysterosalpingography and magnetic resonance imaging. A gestational sac with a heartbeat was observed in the right rudimentary uterine horn at 6 weeks of gestation. A diagnosis of ectopic pregnancy in a non-communicating rudimentary horn was made. Color Doppler detected multiple blood flow signals around the gestational sac, which were clearly increased compared to the left unicornuate uterus. Her serum human chorionic gonadotropin level was 104,619 mIU/ml. A 100 mg methotrexate injection into the gestational sac was administered, and laparoscopic surgery was performed on day 48 after the methotrexate treatment. The right rudimentary horn and fallopian tube were successfully excised with minimal bleeding. A spontaneous normal pregnancy was established 6 months after the surgery. The pregnancy was uneventful, and a baby girl was born by elective cesarean section at 38w0d. CONCLUSION: Combined local methotrexate injection and laparoscopic surgery are safe treatment options for patients with a unicornuate uterus with a non-communicating rudimentary horn pregnancy.


Assuntos
Laparoscopia/métodos , Metotrexato/uso terapêutico , Gravidez Cornual/terapia , Adulto , Feminino , Humanos , Japão/epidemiologia , Gravidez , Resultado da Gravidez , Nascimento a Termo , Anormalidades Urogenitais/terapia , Útero/anormalidades
3.
Fertil Steril ; 115(5): 1347-1349, 2021 05.
Artigo em Inglês | MEDLINE | ID: mdl-33933179

RESUMO

OBJECTIVE: To report the utility of combined transvaginal and transabdominal oocyte retrieval in a patient with an ectopic ovary and unicornuate uterus. DESIGN: Video case report with demonstration of oocyte retrieval technique. SETTING(S): University-affiliated fertility center. PATIENT(S): A 35-year-old woman, gravida 0, with a 6-month history of infertility who presented to our center for fertility evaluation. Hysterosalpingography revealed a left unicornuate uterus and patent left fallopian tube magnetic resonance imaging and laparoscopy showed a right ectopic ovary located in the upper abdomen. Her partner was a 36-year-old male with isolated teratozoospermia. The couple did not conceive with intrauterine insemination. INTERVENTION(S): Ovarian stimulation for in vitro fertilization (IVF). Transvaginal retrieval of oocytes from the right ovary was not deemed possible due the anatomic location of the ovary, intervening blood vessels, and limited mobility of the ovary. Institutional review board approval was not required for this case report as per our institution's policy; patient consent was obtained for publication of the case. MAIN OUTCOME MEASURE(S): Transabdominal retrieval of oocytes from the right ovary and transvaginal retrieval of oocytes from the left ovary. RESULT(S): The couple underwent two IVF cycles. Nine oocytes were retrieved during the first IVF cycle: seven transabdominal (right ovary) and two transvaginal (left ovary). All oocytes were mature, and five blastocysts were cryopreserved. Eight oocytes were retrieved during the second IVF cycle, of which five oocytes were retrieved transabdominally from the right ovary, and three oocytes were retrieved transvaginally from the left ovary. All oocytes were mature, and four blastocysts were cryopreserved. A single thawed embryo was transferred in the natural menstrual cycle, which resulted in the live birth of a full-term baby boy weighing 2,410 grams. CONCLUSION(S): The current case highlights the safety and feasibility of combined transvaginal and transabdominal oocyte retrieval in patients with an ectopic ovary located in the upper abdomen.


Assuntos
Coristoma/cirurgia , Recuperação de Oócitos/métodos , Ovário , Doenças Peritoneais/cirurgia , Anormalidades Urogenitais/cirurgia , Útero/anormalidades , Abdome/cirurgia , Adulto , Coristoma/complicações , Coristoma/terapia , Feminino , Fertilização in vitro , Humanos , Recém-Nascido , Infertilidade/terapia , Nascido Vivo , Masculino , Doenças Peritoneais/terapia , Gravidez , Teratozoospermia/complicações , Teratozoospermia/terapia , Anormalidades Urogenitais/complicações , Anormalidades Urogenitais/terapia , Útero/cirurgia
4.
Reprod Sci ; 28(6): 1596-1611, 2021 06.
Artigo em Inglês | MEDLINE | ID: mdl-33826100

RESUMO

Impairment of uterine structure and function causes infertility, pregnancy loss, and perinatal complications in humans. Some types of uterine impairments such as Asherman's syndrome, also known as uterine synechiae, can be treated medically and surgically in a standard clinical setting, but absolute defects of uterine function or structure cannot be cured by conventional approaches. To overcome such hurdles, partial or whole regeneration and reconstruction of the uterus have recently emerged as new therapeutic strategies. Transplantation of the whole uterus into patients with uterine agenesis results in the successful birth of children. However, it remains an experimental treatment with numerous difficulties such as the need for continuous and long-term use of immunosuppressive drugs until a live birth is achieved. Thus, the generation of the uterus by tissue engineering technologies has become an alternative but indispensable therapeutic strategy to treat patients without a functional or well-structured uterus. For the past 20 years, the bioengineering of the uterus has been studied intensively in animal models, providing the basis for clinical applications. A variety of templates and scaffolds made from natural biomaterials, synthetic materials, or decellularized matrices have been characterized to efficiently generate the uterus in a manner similar to the bioengineering of other organs and tissues. The goal of this review is to provide a comprehensive overview and perspectives of uterine bioengineering focusing on the type, preparation, and characteristics of the currently available scaffolds.


Assuntos
Engenharia Tecidual/tendências , Anormalidades Urogenitais/terapia , Útero/anormalidades , Células-Tronco Adultas , Animais , Bioengenharia/métodos , Matriz Extracelular Descelularizada , Células-Tronco Embrionárias , Feminino , Doenças dos Genitais Femininos/terapia , Ginatresia/terapia , Humanos , Células-Tronco Pluripotentes Induzidas , Engenharia Tecidual/métodos , Alicerces Teciduais , Anormalidades Urogenitais/patologia , Anormalidades Urogenitais/fisiopatologia , Útero/citologia , Útero/patologia , Útero/fisiopatologia
5.
Clin Exp Nephrol ; 25(6): 633-640, 2021 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-33616778

RESUMO

BACKGROUND: Turner syndrome (TS) is a chromosomal disorder with various complications, including congenital anomaly of the kidney and urinary tract (CAKUT). However, its renal function remains poorly known. Therefore, this study aimed to evaluate renal function in TS of various ages from childhood to adulthood. METHODS: We retrospectively analyzed 63 patients with TS who visited our hospital between 1989 and 2020, examined their renal morphology, and analyzed renal function by calculating the estimated glomerular filtration rate (eGFR) using formulas applicable for Japanese populations. RESULTS: Renal morphological abnormality was observed in 22 cases (35.0%) (horseshoe kidney, 7 [11.1%]; hydronephrosis, 11 [17.5%]; duplex collecting system, 3 [4.8%]; and single unilateral kidney, 1 [1.6%]). We evaluated the eGFR of 47 subjects aged 2.8-39.3 years and classified them into Group 1 (with CAKUT, n = 15) and Group 2 (without CAKUT, n = 32). The eGFR at the first visit and the final follow-up was not statistically different between these groups. In Group 1 with CAKUT, the eGFR was not significantly different between that at the first visit and that at the final follow-up (p = 0.21). During the observation period (median, 7.9 years), the eGFR of all individuals in both groups gradually decreased with age, but did not fall < 60 mL/min/1.73 m2, which defines chronic kidney disease (CKD). CONCLUSIONS: The renal function of TS remained normal in all cases during our investigation period, and no one developed CKD by the age of 40 years.


Assuntos
Taxa de Filtração Glomerular , Rim/fisiopatologia , Síndrome de Turner/fisiopatologia , Anormalidades Urogenitais/fisiopatologia , Refluxo Vesicoureteral/fisiopatologia , Adolescente , Adulto , Fatores Etários , Criança , Pré-Escolar , Progressão da Doença , Feminino , Humanos , Japão , Rim/anormalidades , Prognóstico , Estudos Retrospectivos , Síndrome de Turner/diagnóstico , Síndrome de Turner/terapia , Anormalidades Urogenitais/diagnóstico , Anormalidades Urogenitais/terapia , Refluxo Vesicoureteral/diagnóstico , Refluxo Vesicoureteral/terapia , Adulto Jovem
6.
Semin Ultrasound CT MR ; 42(1): 37-45, 2021 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-33541588

RESUMO

Uterine arteriovenous malformations are rare but may represent a life-threatening cause of vaginal bleeding. The typical patient affected is a multiparous woman during her thirties. The origin can be congenital or acquired, with the latter being more common after uterine surgery and presenting mainly as arteriovenous fistulous connections into the myometrium supplied by uterine arteries. The correct diagnosis of uterine arteriovenous malformations requires imaging findings of tubular and tortuous structures with mixed signal from arterial and venous flows; transvaginal color-Doppler ultrasound is the initial technique applied, then integrated with contrast-enhanced magnetic resonance or computed tomography. Multiple treatment approaches are available, including conservative-medical, endovascular embolization and surgery. Transarterial embolization represents the most applied, preserving childbearing capacity with negligible procedural complications; clinical and technical success rates are elevated, up to 90%. The goal of embolization is to occlude the point of fistula or the nidus and the application of multiple embolizing agents has been reported: despite there is no clear superiority of one over the others, liquids, especially those related to the dymethil-sulfoxide family, present relevant technical advantages. Surgery is nowadays to be considered when the endovascular approach fails and in these cases hysterectomy remains the common recommendation.


Assuntos
Malformações Arteriovenosas/diagnóstico por imagem , Malformações Arteriovenosas/terapia , Embolização Terapêutica/métodos , Ultrassonografia Doppler em Cores/métodos , Anormalidades Urogenitais/diagnóstico por imagem , Anormalidades Urogenitais/terapia , Útero/anormalidades , Malformações Arteriovenosas/cirurgia , Feminino , Humanos , Anormalidades Urogenitais/cirurgia , Artéria Uterina/diagnóstico por imagem , Artéria Uterina/cirurgia , Útero/diagnóstico por imagem , Útero/cirurgia
7.
Curr Urol Rep ; 22(3): 15, 2021 Feb 03.
Artigo em Inglês | MEDLINE | ID: mdl-33534013

RESUMO

PURPOSE OF REVIEW: Patients with congenital urologic conditions present unique challenges as adults. Herein, we review the literature relevant to the adult reconstructive urologist confronted with complex surgical concerns affecting their patients with a history of hypospadias, spina bifida, and other syndromes affecting the genitourinary tract. RECENT FINDINGS: Urethral stricture disease related to hypospadias is complex, but successful urethroplasty and penile curvature correction can be achieved with an anatomically minded approach. Multiple urinary diversion techniques can be considered in a patient-centered approach to bladder management in the adult spina bifida patient, but complications are common and revision surgeries are frequently required. Strong evidence is lacking for most surgical techniques in this population, but experiences reported by pediatric and adult urologists with genitourinary reconstruction training can help foster consensus in decision-making. Urologists trained in genitourinary reconstruction may be uniquely positioned to care for the transitional urology patient as they enter adolescence and adulthood.


Assuntos
Papel do Médico , Procedimentos de Cirurgia Plástica , Disrafismo Espinal/complicações , Transição para Assistência do Adulto , Anormalidades Urogenitais/cirurgia , Procedimentos Cirúrgicos Urológicos , Adolescente , Adulto , Criança , Feminino , Humanos , Hipospadia/cirurgia , Masculino , Reoperação , Estreitamento Uretral/etiologia , Estreitamento Uretral/cirurgia , Sistema Urinário/cirurgia , Anormalidades Urogenitais/terapia , Doenças Urológicas/etiologia , Doenças Urológicas/cirurgia , Urologistas
8.
World J Urol ; 39(4): 993-1001, 2021 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-32076821

RESUMO

PURPOSE: Transition in urology is defined by the process that allows an adolescent or a young adult with a congenital or acquired urogenital anomaly to assume increasing responsibility for their own health care and to become the primary decision maker in their care. METHODS: A review of the literature regarding transitional care for lifelong urologic congenital anomalies was performed with the aim of reporting expert opinion when data are non-existent. This review focuses on special considerations for adolescents and young adults with spina bifida, bladder exstrophy, anorectal malformations and differences of sexual development. RESULTS: Urologic goals during the transition from childhood to adulthood continue to include attention to the preservation of renal function and optimization of lower urinary tract function. Additional concerns include care to decrease long-term surgical complications (especially after augmentation cystoplasty), to monitor for malignancy, to prepare for sex activity and fertility, and to help the adult patient in decision making. Transition aims to maximize quality of life and independence by ensuring uninterrupted appropriate care through a multidisciplinary approach which varies by geographical location and healthcare setting. Barriers include patient and family factors as well as provider and system related factors. A dedicated team is an important element of successful transition.


Assuntos
Transição para Assistência do Adulto/normas , Anormalidades Urogenitais/terapia , Adolescente , Humanos , Adulto Jovem
9.
J Matern Fetal Neonatal Med ; 34(3): 360-365, 2021 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-30983458

RESUMO

Objective: The aim of this prospective observational study was to establish a suitable model for the postnatal follow-up and management of prenatal renal and urinary tract anomalies in Shanghai, China.Methods: Minhang and Changning maternal child health care hospitals were selected to establish the integrated management model. Newborns with prenatal renal and urinary tract anomalies in these two centers were eligible to participate in the study from 2015 to 2017. All newborns were classified into three groups based on prenatal findings: (1) severe/complex urinary tract dilatation (UTD) with ureterectasia, (2) other renal and urinary tract abnormalities, and (3) isolated mild to moderate UTD. The newborns underwent their first postnatal ultrasound and follow-up according to the presumed management strategy. Demographic and clinical data were collected from all institutes.Results: A total of 129 newborns fulfilled the study criteria, and 121 completed the postnatal evaluation. Ten newborns in group 1 (n = 13) were diagnosed with obstructive uropathy, including 9 with ureteropelvic junction obstruction (UPJO) and one with megaureter. All 13 newborns in group 2 had consistent postnatal results and were followed under previously established procedures. Sixty-seven cases in group 3 (n = 95) had a UTD at their first scan at 42 postnatal days, and two were diagnosed with UPJO. A total of 2 infants with UPJO underwent surgery, and 71 (65.7%, 71/108) of the UTD cases were resolved.Conclusions: The majority of the patients had a favorable outcome. Close multidisciplinary collaboration among obstetricians, neonatologists, pediatricians, and pediatric nephrologists and urologists is mandatory.


Assuntos
Sistema Urinário , Anormalidades Urogenitais , Doenças Urológicas , Criança , China/epidemiologia , Feminino , Humanos , Lactente , Recém-Nascido , Rim/diagnóstico por imagem , Gravidez , Sistema Urinário/diagnóstico por imagem , Anormalidades Urogenitais/terapia
10.
Best Pract Res Clin Endocrinol Metab ; 34(6): 101476, 2020 12.
Artigo em Inglês | MEDLINE | ID: mdl-33353780

RESUMO

Congenital absence of the vas deferens (CAVD) is a rare genetic condition first discovered in the mid-18th century related to mutations in the cystic fibrosis transmembrane regulatory genes. The condition is typically found during work-up of male infertility, and the majority of cases can be diagnosed with complete history and physical examination and pertinent investigations. The condition can be separated into three subcategories, and genetic advances have led to a much better understanding behind the disease, its pathogenesis, and options for treatment. In this review, we discuss the genetics, pathogenesis, embryology, and diagnosis of treatment of CAVD. Future work in this area likely will aim to better understand the epigenetic factors that influence the development of the condition in order to identify potential upstream therapeutic targets.


Assuntos
Regulador de Condutância Transmembrana em Fibrose Cística/genética , Infertilidade Masculina/genética , Mutação , Anormalidades Urogenitais/genética , Ducto Deferente/anormalidades , Humanos , Infertilidade Masculina/diagnóstico , Infertilidade Masculina/embriologia , Infertilidade Masculina/terapia , Masculino , Anormalidades Urogenitais/diagnóstico , Anormalidades Urogenitais/embriologia , Anormalidades Urogenitais/terapia , Ducto Deferente/diagnóstico por imagem , Ducto Deferente/embriologia , Ducto Deferente/patologia
11.
Semin Pediatr Surg ; 29(6): 150985, 2020 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-33288133

RESUMO

The treatment of patients with colorectal disorders and their associated urologic, gynecologic, gastrointestinal, spinal, and orthopedic anomalies requires care from various medical and surgical specialties over the course of their lifetime. This is ideally handled by a collaborative center which facilitates the assessment and development of a long-term patient care plan among multiple specialties which can enhance the quality of care, improve communication among different specialties, and improve patient satisfaction and outcomes. We describe the process, as well as lessons learned in developing such a center.


Assuntos
Anormalidades Múltiplas/terapia , Malformações Anorretais/terapia , Doença de Hirschsprung/terapia , Hospitais Especializados/organização & administração , Anormalidades Musculoesqueléticas/terapia , Desenvolvimento de Programas/métodos , Anormalidades Urogenitais/terapia , Adolescente , Criança , Pré-Escolar , Cirurgia Colorretal/organização & administração , Humanos , Lactente , Recém-Nascido , Colaboração Intersetorial , Planejamento de Assistência ao Paciente/organização & administração , Equipe de Assistência ao Paciente/organização & administração , Pediatria/organização & administração , Encaminhamento e Consulta/organização & administração , Transição para Assistência do Adulto/organização & administração
12.
Semin Pediatr Surg ; 29(6): 150987, 2020 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-33288143

RESUMO

Anorectal malformations (ARM) are well recognized to be associated with anomalies in other organ systems. The introduction of screening protocols has increased the diagnosis of these anomalies and greater collaboration with other specialties has influenced the treatment and follow-up of patients with ARMs. Much of the medical literature regarding the treatment of anorectal malformations has focused on technical details of operations and early post-surgical outcomes. Recently, an increase in published data regarding the long-term sequelae of an ARM diagnosis has resulted in an emphasis extended follow up in this population. Patient support groups have highlighted complex issues in ARM patients persist into adulthood have advocated for improved transitional care. This article describes the benefits of long-term follow-up and identifies key issues in ARM patients with respect to urologic and gynecologic health. A collaborative model of care is outlined and suggested timings of screening for potential problems is described.


Assuntos
Anormalidades Múltiplas/terapia , Assistência ao Convalescente , Malformações Anorretais/terapia , Relações Interprofissionais , Equipe de Assistência ao Paciente , Anormalidades Urogenitais/terapia , Anormalidades Múltiplas/diagnóstico , Assistência ao Convalescente/métodos , Assistência ao Convalescente/organização & administração , Malformações Anorretais/diagnóstico , Comportamento Cooperativo , Gastroenterologia , Ginecologia , Humanos , Equipe de Assistência ao Paciente/organização & administração , Pediatria , Anormalidades Urogenitais/diagnóstico , Urologia
13.
Early Hum Dev ; 150: 105189, 2020 11.
Artigo em Inglês | MEDLINE | ID: mdl-32978001

RESUMO

Fetal lower urinary tract obstruction (LUTO) is classically based on prenatal ultrasound identification of a dilated/ thick-walled bladder, bilateral hydronephrosis, dilated ureters and a dilated posterior urethra (also known as the "keyhole sign") in a male fetus. Although the most common underlying diagnosis is posterior urethral valves, the prenatal appearance may be similar with urethral atresia or stenosis, the Prune-Belly Syndrome, or even a cloacal anomaly in a female. These conditions form part of the Congenital Anomalies of Kidney and Urinary Tract (CAKUT) spectrum, which is the commonest cause of end-stage renal disease in children. Although it is difficult to predict postnatal renal function from the prenatal appearance, studies have recently identified predictive features (based on ultrasound findings and fetal biochemistry), and established staging systems to assist with counselling, and, where indicated, patient selection for in-utero intervention. Current in-utero therapy includes amnio-infusion, vesico-amniotic shunting, and fetal cystoscopy with valve ablation or urethral stenting. Postnatal survival and renal functional outcomes, complications and management uncertainties are described, highlighting areas of future development.


Assuntos
Ultrassonografia Pré-Natal/métodos , Obstrução do Colo da Bexiga Urinária/diagnóstico por imagem , Anormalidades Urogenitais/diagnóstico por imagem , Refluxo Vesicoureteral/diagnóstico por imagem , Feminino , Humanos , Recém-Nascido , Gravidez , Prognóstico , Ultrassonografia Pré-Natal/normas , Obstrução do Colo da Bexiga Urinária/diagnóstico , Obstrução do Colo da Bexiga Urinária/terapia , Anormalidades Urogenitais/diagnóstico , Anormalidades Urogenitais/terapia , Refluxo Vesicoureteral/diagnóstico , Refluxo Vesicoureteral/terapia
14.
Pediatr Nephrol ; 35(5): 851-860, 2020 05.
Artigo em Inglês | MEDLINE | ID: mdl-32020338

RESUMO

BACKGROUND: Congenital anomalies of the kidney and urinary tract (CAKUT) is associated with a slower progression to end-stage renal disease (ESRD) in pre-dialysis patients. However, little is known about the associated mortality risks after transitioning to dialysis. METHODS: This retrospective cohort study included 0-21 year-old incident dialysis patients from the United States Renal Data System starting dialysis between 1995 and 2016. We examined the association of CAKUT vs. non-CAKUT with all-cause mortality, using Cox regression adjusted for case mix variables. We also examined the mortality risk associated with 14 non-CAKUT vs. CAKUT ESRD etiologies and under stratification by estimated glomerular filtration rate (eGFR). RESULTS: Among 25,761 patients, the median (interquartile range) age was 17 (11-19) years, and 4780 (19%) had CAKUT. CAKUT was associated with lower mortality, with an adjusted hazard ratio (aHR) of 0.72 (95%CI, 0.64-0.81) (reference: non-CAKUT). In age-stratified analyses, CAKUT vs. non-CAKUT aHRs (95%CI) were 0.66 (0.54-0.80), 0.56 (0.39-0.80), 0.66 (0.50-0.86), and 0.97 (0.80-1.18) among patients < 6, 6-< 13, 13-< 18, and ≥ 18 years at dialysis initiation, respectively. Among non-CAKUT ESRD etiologies, the risk of mortality associated with primary glomerulonephritis (aHR, 0.93; 95%CI 0.80-1.09) and focal segmental glomerulosclerosis (aHR, 0.89; 95%CI, 0.75-1.04) were comparable or slightly lower compared to CAKUT, whereas most other primary causes were associated with higher mortality risk. While the CAKUT group had lower mortality risk compared to the non-CAKUT group patients with eGFR ≥5 mL/min/1.73m2, CAKUT was associated with higher mortality in patients with eGFR < 5 mL/min/1.73 m2. CONCLUSIONS: CAKUT is associated with lower mortality among children < 18 years old, but showed comparable mortality with non-CAKUT among patients ≥ 18 years old. ESRD etiology should be considered in risk assessment for children initiating dialysis.


Assuntos
Glomerulonefrite/mortalidade , Glomerulosclerose Segmentar e Focal/mortalidade , Falência Renal Crônica/mortalidade , Diálise Renal/estatística & dados numéricos , Anormalidades Urogenitais/mortalidade , Refluxo Vesicoureteral/mortalidade , Adolescente , Causas de Morte , Criança , Pré-Escolar , Progressão da Doença , Feminino , Taxa de Filtração Glomerular , Glomerulonefrite/complicações , Glomerulonefrite/terapia , Glomerulosclerose Segmentar e Focal/complicações , Glomerulosclerose Segmentar e Focal/terapia , Humanos , Lactente , Recém-Nascido , Falência Renal Crônica/etiologia , Falência Renal Crônica/terapia , Masculino , Estudos Retrospectivos , Medição de Risco/estatística & dados numéricos , Fatores de Risco , Estados Unidos/epidemiologia , Anormalidades Urogenitais/complicações , Anormalidades Urogenitais/terapia , Refluxo Vesicoureteral/complicações , Refluxo Vesicoureteral/terapia , Adulto Jovem
15.
J Neonatal Perinatal Med ; 13(1): 91-96, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-31609706

RESUMO

BACKGROUND: Renal pelvic dilatation (RPD) is a frequent finding in fetal ultrasound. The aim of the study is to correlate the prenatally detected moderate and severe pyelectasis with the postnatal outcome. METHODS: A retrospective analysis involving 90 cases of prenatally detected moderate and severe RPD referred to our prenatal diagnosis centre with 18 months of urological follow-up. Prenatal ultrasound was correlated with postnatal renal function, assessed by plasmatic creatinine and/or renal scintigraphy performed before surgery. RESULTS: Cases were divided between two groups according to postnatal management: group A including 35 newborns (38.9%) that needed surgical treatment and group B with 55 patients (61.1%) who were managed conservatively. The group A presented higher median RPD (18 mm, IQR 12-25 mm) compared to the group B (11 mm, IQR 10-14 mm). The most common anomaly detected within group A was pelvi-ureteric junction (PUI) obstruction (43%). Within group B 32 cases (58%) showed spontaneous resolution of hydronephrosis during postnatal follow up. In case of moderate pyelectasis the risk of postnatal surgery was 25% and raised to 60% for severe RPD. In our study, 29 newborns showed pathologic scintigraphies: 25 required surgery while 4 did not find indication for surgery due to ipsilateral renal function irreversible damage. 6 patients had high creatinine level (>0.6 mg/dl). 35 cases out of 90 (39%) developed monolateral irreversible renal function impairment. CONCLUSION: Moderate and severe RPD are often correlated with postnatal renal damage, therefore a close multidisciplinary follow-up is required. Prenatal scanning is highly predictive of postnatal outcome and can address properly the prenatal counseling.


Assuntos
Tratamento Conservador , Hidronefrose/terapia , Pielectasia/terapia , Obstrução Ureteral/cirurgia , Procedimentos Cirúrgicos Urológicos , Refluxo Vesicoureteral/terapia , Creatinina/metabolismo , Feminino , Humanos , Hidronefrose/complicações , Hidronefrose/congênito , Hidronefrose/diagnóstico por imagem , Recém-Nascido , Pelve Renal/cirurgia , Masculino , Gravidez , Pielectasia/diagnóstico por imagem , Pielectasia/metabolismo , Cintilografia , Remissão Espontânea , Insuficiência Renal/congênito , Insuficiência Renal/etiologia , Insuficiência Renal/metabolismo , Estudos Retrospectivos , Índice de Gravidade de Doença , Rim Único , Ultrassonografia Pré-Natal , Ureter/cirurgia , Obstrução Ureteral/congênito , Obstrução Ureteral/diagnóstico por imagem , Estreitamento Uretral/diagnóstico por imagem , Estreitamento Uretral/metabolismo , Estreitamento Uretral/terapia , Anormalidades Urogenitais/diagnóstico por imagem , Anormalidades Urogenitais/metabolismo , Anormalidades Urogenitais/terapia , Refluxo Vesicoureteral/diagnóstico por imagem , Refluxo Vesicoureteral/metabolismo
16.
Minerva Med ; 111(1): 33-49, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31755672

RESUMO

INTRODUCTION: Endometriosis is a common disease in women of reproductive age. In addition to causing pain, it may also reduce fertility. The coexistence of endometriosis and congenital uterine anomalies (CUA) has been frequently reported in the published literature. The present report is a review of existing studies on the subject and our own hitherto unpublished data. EVIDENCE ACQUISITION: The electronic search was conducted using the Pubmed database with specific keyword combinations including endometriosis, adenomyosis, infertility, Müllerian malformations/anomalies, and septate uterus. The principal aspects addressed in the present study were: diagnosis, management, and classification of CUA, their impact on fertility and coexistence with endometriosis. EVIDENCE SYNTHESIS: Endometriosis and CUA are frequently detected in the exploration of infertility, because both of these are liable to impair fertility. Endometriosis is associated with obstructive anomalies and nonobstructive malformations, especially those concerning the septate uterus. The diagnosis and management of CUA have been discussed for several years. Various classification systems have been proposed. CONCLUSIONS: The analysis of the existing literature has revealed the absence of any consensus about the management, diagnosis, and classification of CUA, especially with regard to the septate uterus. We need to find and speak a common language in order to avoid inappropriate or unnecessary surgery and optimize the individual patient's treatment. The combined presence of endometriosis or adenomyosis and CUA is a reason to perform precise diagnostic imaging investigations and early surgery for the purpose of enhancing the chances of pregnancy in infertile patients. Further research is needed on the subject.


Assuntos
Endometriose/complicações , Anormalidades Urogenitais/complicações , Útero/anormalidades , Adenomiose/complicações , Adenomiose/patologia , Endometriose/patologia , Feminino , Humanos , Infertilidade Feminina/etiologia , Ultrassonografia , Anormalidades Urogenitais/classificação , Anormalidades Urogenitais/diagnóstico , Anormalidades Urogenitais/terapia
17.
J Pediatr Surg ; 55(7): 1377-1380, 2020 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-31266634

RESUMO

INTRODUCTION: Herlyn-Werner-Wunderlich syndrome (HWWS) is a rare variant of Mullerian ductal anomaly associated with ipsilateral renal agenesis. Most patients are diagnosed after menarche with complications of uterovaginal obstruction, genitourinary infection and pelvic adhesions. Patients often undergo additional operations for misdiagnosis or treatment of complications. Our institution manages several HWWS patients diagnosed before symptoms by screening for antenatally-diagnosed renal agenesis. This study aims to improve the presymptomatic management of HWWS patients. METHODS: We carried out retrospective case review of patients diagnosed with HWWS from 2010 to 2017 on patient demographics, symptoms, clinical course and operative management and summarize the sparse literature published to date. RESULTS: There were 8 patients with HWWS but only 2 symptomatic patients presented acutely with hematocolpos requiring urgent vaginal surgery. The other six patients had early diagnosis through postnatal ultrasound screening. No patient required further operation for diagnosis or complications related to obstructed hemivagina. CONCLUSION: Our case series and literature review show that the majority of prepubertal patients with HWWS do not require early gynecological surgery. We recommend that female babies with renal agenesis should be screened for HWWS syndrome with ultrasound. Early diagnosis and presymptomatic elective surgery may prevent urogynecological complications that cause fertility and renal impairment. STUDY DESIGN: Case series, level IV evidence.


Assuntos
Rim/anormalidades , Ductos Paramesonéfricos/anormalidades , Anormalidades Urogenitais , Adolescente , Adulto , Criança , Pré-Escolar , Erros de Diagnóstico , Feminino , Hematocolpia/etiologia , Hematocolpia/cirurgia , Humanos , Lactente , Estudos Retrospectivos , Síndrome , Anormalidades Urogenitais/complicações , Anormalidades Urogenitais/diagnóstico , Anormalidades Urogenitais/terapia , Vagina/anormalidades , Vagina/cirurgia , Adulto Jovem
18.
Abdom Radiol (NY) ; 44(12): 3978-3989, 2019 12.
Artigo em Inglês | MEDLINE | ID: mdl-31478084

RESUMO

Urachal anomalies are classified into four types depending on the level of persistence of the embryonic urachal remnants between the urinary bladder and the umbilicus: patent urachus, umbilical-urachal sinus, urachal cyst, and vesico-urachal diverticulum. Due to the increasing use of cross-sectional imaging, urachal anomalies are frequently detected as incidental findings. Imaging plays a pivotal role in the initial diagnosis, evaluation of complications, treatment follow-up, and long-term surveillance of patients with urachal anomalies. Different urachal anomalies demonstrate characteristic imaging features that aid in a timely diagnosis and guide treatment. A patent urachus is visualized as an elongated tubular structure between the umbilicus and the urinary bladder. While umbilical-urachal sinus appears as focal dilatation at the umbilical end of the urachal remnant, the vesico-urachal diverticulum presents as a focal outpouching of the urinary bladder at anterosuperior aspect. Urachal cysts are identified as midline fluid-filled sacs most frequently located near the dome of the urinary bladder. Untreated urachal anomalies could progress into potential complications, including infection and malignancy. Knowledge regarding imaging features of urachal anomalies helps in timely diagnosis, treatment, follow-up, and early detection of complications.


Assuntos
Úraco/anormalidades , Anormalidades Urogenitais/diagnóstico por imagem , Meios de Contraste , Diagnóstico Diferencial , Humanos , Achados Incidentais , Anormalidades Urogenitais/complicações , Anormalidades Urogenitais/terapia
19.
Prenat Diagn ; 39(7): 495-504, 2019 06.
Artigo em Inglês | MEDLINE | ID: mdl-30957256

RESUMO

OBJECTIVES: Cardiac remodeling due to renal dysfunction may have an impact on myocardial function (MF) of fetuses with lower urinary tract obstruction (LUTO). The aim was to identify possible differences in MF in LUTO fetuses compared with healthy controls and to look for interactions between urine biochemistry and MF indices. METHODS: This is a cohort study consisting of 31 LUTO fetuses and 45 healthy controls. Subgroups were generated according to intrauterine therapy (group 1: LUTO after therapy, group 2: LUTO without therapy at the time of examination, and group 3: controls). MF indices were measured using pulsed wave tissue Doppler imaging and M-mode. Furthermore, results of fetal urine biochemistry were gathered retrospectively. RESULTS: Among other findings, right ventricular (RV) e'/a' ratio was lower in group 1 compared with group 3 (p = .050). According to gestational age (GA) level-dependent analysis, RV isovolumetric relaxation time was significantly longer in group 2 compared with group 1 and group 3 at GA level 1 (19 wk of gestation). A significant positive correlation between RV e'/a' ratio and ß-2-microglobulin as well as α-1-microglobulin and potassium could be observed. CONCLUSION: We observed differences in MF and an association between ventricular filling pattern and renal protein secretion in LUTO fetuses. This can be interpreted as a sign of intrauterine cardiac remodeling.


Assuntos
Doenças Fetais/fisiopatologia , Feto/fisiologia , Coração/fisiologia , Obstrução Uretral/fisiopatologia , Estudos de Casos e Controles , Estudos de Coortes , Ecocardiografia Doppler , Feminino , Doenças Fetais/terapia , Doenças Fetais/urina , Fetoscopia , Idade Gestacional , Testes de Função Cardíaca , Humanos , Recém-Nascido , Masculino , Gravidez , Estudos Retrospectivos , Ultrassonografia Pré-Natal , Obstrução Uretral/congênito , Obstrução Uretral/terapia , Obstrução Uretral/urina , Anormalidades Urogenitais/fisiopatologia , Anormalidades Urogenitais/terapia , Anormalidades Urogenitais/urina , Função Ventricular Esquerda/fisiologia , Remodelação Ventricular/fisiologia
20.
Indian J Pediatr ; 86(4): 354-359, 2019 04.
Artigo em Inglês | MEDLINE | ID: mdl-30796705

RESUMO

OBJECTIVES: To study the etiology and clinical profile of congenital obstructive uropathy in children, renal status and growth at diagnosis and at follow-up and to determine the predictors for development of chronic kidney disease (CKD). METHODS: An observational (retrospective-prospective) study was conducted at a tertiary care hospital in South India from September 2014 through September 2016. Sixty children diagnosed to have congenital obstructive uropathy with a minimum follow-up period of 5 y were included and followed up prospectively for 2 more years during the study period. The data of the children at admission and follow-up was obtained from the medical records and analyzed. RESULTS: Congenital uretero-pelvic junction obstruction followed by Posterior urethral valve were the most common etiologies identified. Male preponderance (88.3%) was observed with poor urinary stream being the most common presentation (36.6%). Forty percent of the population had elevated creatinine. Fifteen percent were hypertensive and 25% had growth failure at diagnosis. However, there was a reduction in the number of children with poor estimated glomerular filtration rate (eGFR), hypertension and growth faltering during follow-up. Among the risk factors, hypertension at diagnosis [O.R-12.8 (2.21-74.22) and p value <0.05] and frequent urinary tract infection (UTI) [O.R-14.06 (2.32-85.42) and p value <0.05] were the most important factors for CKD progression. Children with low eGFR (< 60 ml/min/1.73m2) had more height faltering and hypertension at follow-up (p value <0.05). CONCLUSIONS: Hypertension and frequent UTI were observed to be strongly associated with progression of CKD. Estimated GFR was found to be significantly associated with faltering of height and hypertension. Preserving the renal function prevents growth faltering and development of hypertension at follow-up thereby ensuring a better quality of life.


Assuntos
Anormalidades Urogenitais/patologia , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Fatores Sexuais , Obstrução Ureteral/congênito , Obstrução Ureteral/patologia , Anormalidades Urogenitais/terapia
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