ABSTRACT
OBJECTIVES: Many congenital hydronephroses spontaneously resolve. This study evaluated a long-term follow-up of more than 4 years of patients with congenital hydronephrosis at a single center. METHODS: In total, 215 patients (286 kidneys) with congenital hydronephrosis were included. Hydronephrosis outcomes (resolution, improvement, and persistence) and time-to-outcome were evaluated. RESULTS: Fourteen patients underwent early surgical intervention until the age of 2 years. A total of 189 congenital hydronephrosis cases (66%) showed resolution at a median of 16 months (interquartile range: 7-21 months) and 169 (80%) of 210 kidneys with grade I to II hydronephrosis showed resolution at a median of 14 months (interquartile range: 6-23 months). Of 76 kidneys with grade III to IV hydronephrosis, 24 (32%) showed resolution at a median of 29 months (interquartile range: 24-41 months), and 56 (74%) showed improvement to grade II or less at a median of 12 months (interquartile range: 5-23 months). Of the 76 kidneys with grade III to IV hydronephrosis, five required delayed pyeloplasty at a median of 66 months (interquartile range: 42-89 months). One patient was asymptomatic, with a marked worsening of hydronephrosis and decreased renal function 6 years after the resolution of hydronephrosis. CONCLUSIONS: None of the patients with grade I to II hydronephrosis required surgical treatment, and a shorter follow-up may be sufficient. Grade III to IV severe hydronephrosis should be considered for a longer and more careful follow-up, given the possibility of asymptomatic exacerbation of hydronephrosis.
Subject(s)
Hydronephrosis , Humans , Hydronephrosis/congenital , Hydronephrosis/surgery , Hydronephrosis/diagnosis , Hydronephrosis/etiology , Hydronephrosis/complications , Follow-Up Studies , Male , Female , Infant , Child, Preschool , Kidney/abnormalities , Kidney/surgery , Infant, Newborn , Retrospective Studies , Time Factors , Remission, Spontaneous , Severity of Illness Index , Treatment Outcome , ChildABSTRACT
OBJECTIVES: Cryptorchidism (CO) diagnosis by palpation is challenging. Patients with suspected CO are primarily referred to pediatric urologists by general pediatricians and urologists. Currently, surgical treatment for CO is recommended earlier than in previous guidelines. In this study, we evaluated factors that lead to diagnosis discordance and delayed orchidopexy in patients referred with suspected CO in addition to timing of initial screening. METHODS: In total, 731 patients (1052 testes) with suspected CO were included. Risk factors for diagnostic discrepancy in CO diagnosis by pediatric urologists and risk of delayed orchiopexy were evaluated. RESULTS: Herein, 659 (90%) patients were diagnosed during routine public health checkups for infants and young children, and 419 (57%) patients were referred by pediatric practitioners. Of 1052 testes, 374 (36%) were diagnosed with CO by pediatric urologists. In multivariate analysis, risk factors of diagnostic discrepancy for CO diagnosis by pediatric urologists were bilateral testis (odds ratio [OR] = 9.17, p < 0.0001), >6 months old at initial diagnosis (OR = 1.036, p < 0.0001), and pediatric referral (OR = 4.60, p < 0.0001). In total, 296 patients underwent orchiopexy for CO. In multivariate analysis, risk factors for delayed orchiopexy were presence of comorbidities (OR = 3.43, p = 0.003) and >10 months old at referral (OR = 12.62, p < 0.0001). CONCLUSIONS: Pediatric referral is a risk factor for discordant CO diagnostics, and late age at referral brings a risk of delayed orchiopexy. It is necessary to enlighten pediatricians, who are mainly responsible for routine health checkups, in teaching CO diagnostic techniques to ensure early referral.
Subject(s)
Cryptorchidism , Infant , Male , Child , Humans , Child, Preschool , Infant, Newborn , Cryptorchidism/diagnosis , Cryptorchidism/surgery , Orchiopexy/adverse effects , Orchiopexy/methods , Retrospective Studies , Age Factors , Risk FactorsABSTRACT
Acute pyelonephritis is the leading cause of bacterial infection among children. It can be difficult to diagnose early in the disease course owing to non-specific symptoms and physical findings. Recently, some cases of pediatric acute pyelonephritis with mild encephalitis/encephalopathy with a reversible splenial lesion (MERS) have been reported. We describe a case of a six-year-old boy who presented with a high fever and four episodes of cluster convulsions. Despite the absence of leukocyturia and hypo-inflammatory response in the blood, he was diagnosed with acute pyelonephritis by contrast-enhanced computed tomography seven days after onset. The convulsions were not simple febrile convulsions and suggested central nervous system (CNS) lesions, as the patient was older than the usual cut-off age of five years for febrile seizures. This case highlights an unusual presentation and clinical course of a case of pediatric acute pyelonephritis characterized by cluster convulsions and a poor inflammatory response. Furthermore, we strongly consider that the cause of the cluster convulsions may be related to MERS spectrum disorder and emphasize that pyelonephritis can be accompanied by CNS disturbances.
ABSTRACT
PURPOSE: The level of 6-sulfatoxy-melatonin (SaMT), a metabolite of melatonin, in first-void morning urine reflects blood melatonin levels from the previous night. We investigated the association between urine SaMT and sleep quality deterioration in patients with non-muscle invasive bladder cancer (NMIBC) treated with intravesical Bacillus Calmette-Guerin induction therapy (iBCG). METHODS: We enrolled 51 patients who received iBCG once weekly for 6 or 8 weeks. Patient-reported outcomes were assessed with questionnaires including the International Prostate Symptom Score (IPSS) and European Organization for Research and Treatment of Cancer Quality of Life Questionnaire Core 30 (QLQC30). Questionnaires were completed before (baseline), during, at completion, and 1 and 3 months after iBCG. Melatonin and SaMT levels at baseline were measured in serum and first-void morning urine samples, respectively. RESULTS: Based on changes in the QLQC30 insomnia subscale, 28 (55%) patients experienced sleep quality deterioration (deterioration group). Urine SaMT values in the deterioration group were lower than those in the non-deterioration group (P = 0.0015; 7.5 vs 15.4 ng/mg creatinine, respectively). Nocturia scores in the non-deterioration group decreased over time, while those of the deterioration group remained high after completion of iBCG. A binary logistic regression analysis revealed that low urine SaMT levels (≤ 9.6 ng/mg creatinine), high IPSS nocturia scores at baseline, and high IPSS storage subscores at baseline were associated with BCG-induced sleep quality deterioration. CONCLUSIONS: This study confirmed the association among urine SaMT levels, nocturia, and sleep disturbance in patients with NMIBC who receive iBCG. We should be aware of treatment-induced impairments to aid in appropriate decision-making.
Subject(s)
BCG Vaccine , Melatonin , Sleep Quality , Urinary Bladder Neoplasms , Administration, Intravesical , BCG Vaccine/therapeutic use , Creatinine , Humans , Male , Melatonin/urine , Neoplasm Invasiveness , Neoplasm Recurrence, Local/therapy , Nocturia , Quality of Life , Urinary Bladder Neoplasms/drug therapyABSTRACT
OBJECTIVES: Diagnosis of Hunner-type interstitial cystitis (HIC) relies on the ability to identify Hunner lesions endoscopically, which can lead to storage symptom misdiagnosis. Here, we examined serum biomarkers for HIC and verified their utility. METHODS: Based on the previous definition of the Japanese guidelines, which did not distinguish HIC and non-HIC diseases, we searched for serum biomarkers in 25 patients with interstitial cystitis (IC) and 25 control participants using metabolomics during 2013-2014. In 2019, we conducted a validation study in HIC and control groups. Serum samples were analyzed using liquid chromatography-tandem mass spectrometry, and candidate biomarker concentrations were compared between the groups using Mann-Whitney test. RESULTS: Metabolomics targeted 678 metabolites and revealed that the levels of 14 lysolipids, seven γ-glutamyl amino acids, and two monoacylglycerols were significantly different between the IC and control groups. The following metabolites were selected from each metabolite category as candidates: 1-linoleoylglycerophosphocholine (1-linoleloyl-GPC [18:2]), γ-glutamylisoleucine (γ-Glu-Ile), and 1-arachidonylglycerol (1-AG). The serum concentrations of 1-linoleoyl-GPC (18:2) in the HIC and control groups were 27 920 ± 6261 and 40 360 ± 1514 ng/mL (P = 0.0003), respectively. The serum concentrations of γ-Glu-Ile and 1-AG were not significantly different between the groups. When the cut-off value of 1-linoleoyl-GPC (18:2) was set at 28 400 ng/mL, the sensitivity and specificity were 68% and 84%, respectively. CONCLUSIONS: Serum 1-linoleoyl-GPC (18:2) is a candidate diagnostic biomarker for HIC. Additional studies on whether this biomarker can distinguish HIC from other diseases with high urination frequency are required for its clinical use.
Subject(s)
Cystitis, Interstitial , Biomarkers , Cystitis, Interstitial/diagnosis , Cystitis, Interstitial/pathology , Humans , Urinary Bladder/pathologyABSTRACT
PURPOSE: The treatment landscape for advanced, unresectable, or metastatic urothelial carcinoma (aUC) has shifted substantially since the advent of immune checkpoint inhibitors (ICIs). We investigated the extent to which pembrolizumab therapy is superior to conventional chemotherapy as a second-line treatment. PATIENTS AND METHODS: A multicenter-derived database registered 454 patients diagnosed with aUC between 2008 and 2020. Of these, 94 patients (21%) who received second-line pembrolizumab and 75 (17%) who received second-line chemotherapy but never received third-line or later ICI therapy were included. We compared overall survival (OS) from the initial date of first-line chemotherapy between two groups by adjusting for prognostic factors through propensity score matching (PSM) and inverse probability of treatment weighting (IPTW). The IPTW-adjusted hazard ratio and 95% confidence interval were estimated using a multivariate Cox regression analysis. To identify patients who were more likely to benefit from second-line pembrolizumab than from chemotherapy, we performed a subgroup analysis for OS with an IPTW-adjusted model. RESULTS: The PSM-adjusted comparison showed a significant improvement in the prognosis with second-line pembrolizumab use (P = 0.01). The OS benefit with the advent of pembrolizumab was 8 months (18 months vs 26 months). Multivariable analyses using IPTW adjustment demonstrated that lymph node metastasis (P = 0.001), lung metastasis (P = 0.013), and bone metastasis (P = 0.003) were poor independent prognostic factors, and pembrolizumab use (P = 0.021) was a favorable independent prognostic factor. Subgroup analyses revealed that pembrolizumab was associated with survival benefits over chemotherapy in all subgroups, including young patients (age <70 years), those who received radical surgery, and those without visceral metastasis. CONCLUSION: We demonstrated a significant improvement in prognosis after the advent of pembrolizumab for patients with aUC. ICIs should not be restricted based on patient characteristics.
ABSTRACT
PURPOSE: We investigated sleep parameters and patient-reported outcomes before, during, and after induction Bacillus Calmette-Guerin therapy using questionnaires and actigraphy in patients with non-muscle invasive bladder cancer. METHODS: We investigated 10 patients who received Bacillus Calmette-Guerin therapy once weekly for 8 weeks. The International Prostate Symptom Score, European Organization for Research and Treatment of Cancer Quality of Life Questionnaire Core 30, Functional Assessment of Cancer Therapy-Bladder, and multi-item Short Form-8 tools were used to assess patient-reported outcomes. Participants completed all questionnaires before (baseline), at the 4th and 8th doses, and 1 month after the last Bacillus Calmette-Guerin dose. The MotionWatch8 was fastened to patients' waist throughout the study. Composite sleep quality was determined based on sleep duration, efficiency, and fragmentation. RESULTS: We observed a transient increase in frequency/nocturia subscores and the insomnia subscore. The number of patients with poor sleep quality increased from 0 (0%) at baseline to 7 (70%) at the 4th dose and to 6 (60%) patients at the 8th dose. Among 10 patients, 6 (60%) were assigned to the sleep deterioration group and 4 (40%) to the non-deterioration group. Sleep quality was restored to baseline levels in 5 of 6 patients (83%) within 1 month after the last dose in the sleep deterioration group, and the nocturia subscore of the International Prostate Symptom Score was significantly increased only in this group (P=0.03). CONCLUSIONS: This is the first study that confirms intravesical Bacillus Calmette-Guerin-induced sleep quality deterioration based on a questionnaire survey and actigraphy.
Subject(s)
BCG Vaccine , Urinary Bladder Neoplasms , Actigraphy , BCG Vaccine/adverse effects , Humans , Male , Neoplasm Invasiveness , Outcome Assessment, Health Care , Quality of Life , Surveys and Questionnaires , Urinary Bladder Neoplasms/drug therapyABSTRACT
INTRODUCTION: Response to pembrolizumab after first-line chemotherapy is vital to prolonged survival in advanced, unresectable, and/or metastatic urothelial carcinoma (aUC). However, there are sparse clinical data on host-tumor immune modification by first-line platinum-based chemotherapy. This study investigated the association between response to first-line gemcitabine plus cisplatin (GC) or carboplatin (GCarbo) chemotherapy and response to subsequent pembrolizumab treatment. PATIENTS AND METHODS: A multicenter-derived database registered 454 patients diagnosed with aUC between 2008 and 2020. Of these, 108 patients who received first-line GC or GCarbo followed by second-line or later pembrolizumab were eligible for investigation and were classified into 3 groups: 48 receiving full-dose GC, 21 receiving dose-reduced GC, and 39 receiving GCarbo. Overall survival (OS) was calculated using the Kaplan-Meier method and compared using the log-rank test. Possible factors associated with the response to pembrolizumab were evaluated using binary logistic regression methods. RESULTS: The rate of patients undergoing surgical removal of the primary organ was higher and creatinine clearance was lower in the dose-reduced GC and GCarbo groups than in the full-dose GC groups. Pembrolizumab responders had significantly better survival benefits than nonresponders. The rate of pembrolizumab responders was much higher in first-line chemotherapy responders than in first-line chemotherapy nonresponders. In contrast to the full-dose GC and GCarbo groups, the pembrolizumab responder rate was lower, and no association was observed between response to first-line chemotherapy and response to pembrolizumab in the dose-reduced GC group. CONCLUSION: Cisplatin and carboplatin may play an important role in the antitumor immune response, which could impact the outcome of subsequent pembrolizumab treatment. Given that the rate of response to pembrolizumab after dose-reduced GC chemotherapy was relatively low, this regimen is not recommended for cis-unfit patients with aUC. Further studies are required to understand the mechanisms responsible for the cross-reactivity of platinum and immune checkpoint inhibitors.
Subject(s)
Carcinoma, Transitional Cell , Urinary Bladder Neoplasms , Antibodies, Monoclonal, Humanized , Antineoplastic Combined Chemotherapy Protocols , Carboplatin , Carcinoma, Transitional Cell/drug therapy , Carcinoma, Transitional Cell/pathology , Cisplatin , Deoxycytidine/analogs & derivatives , Humans , Urinary Bladder Neoplasms/pathology , GemcitabineABSTRACT
OBJECTIVE: To investigate useful objective variables and factors supporting the diagnosis of retractile testis and cryptorchidism by primary care providers, including urologists. METHODS: This retrospective study included 512 boys diagnosed with retractile testis or cryptorchidism at our institute. Boys with retractile testis were followed up and underwent orchiopexy once the testis became undescended, while boys with cryptorchidism underwent orchiopexy immediately. We investigated trends in the prevalence of testicular malposition and explored useful diagnostic factors for retractile testis and cryptorchidism. RESULTS: Of 512 boys, 199 were diagnosed with retractile testis and 313 were diagnosed with cryptorchidism. Comparison of clinical information between retractile testis and cryptorchidism showed that age at diagnosis, laterality, and location of the testis were significantly different between the groups (P < 0.0001, P < 0.0001, and P < 0.0001, respectively). The comparison of surgical information also showed that epididymal abnormality and state of processus vaginalis is patency or closure were significantly different between the groups (P = 0.0088 and P = 0.0003, respectively). Multivariate analysis showed that diagnosis at age 0-1 years, unilaterality, and canalicular testis were predictive factors for cryptorchidism (P = 0.001, P < 0.0001, and P < 0.0001, respectively). CONCLUSIONS: Age at diagnosis, laterality, and location of the testis could be factors to aid the diagnosis of retractile testis and cryptorchidism.
Subject(s)
Cryptorchidism , Testis , Cryptorchidism/diagnosis , Cryptorchidism/epidemiology , Cryptorchidism/surgery , Humans , Infant , Infant, Newborn , Male , Prevalence , Retrospective Studies , Testis/surgeryABSTRACT
OBJECTIVES: The bladder urothelium is not always impermeable. During sleep, the bladder might absorb urine in healthy individuals who sleep through the night. This study aimed to determine whether the bladder absorbs urine by using a method other than ultrasonic scanning and to simultaneously evaluate sleeping conditions. METHODS: Eleven participants (five males, six females) aged 20 to 49 years without lower urinary tract symptoms or urination while sleeping were enrolled. Bladder volume was estimated by studying the relationship between dilution and absorbance of indigo carmine dissolved in urine. A 12F Foley catheter was inserted into the bladder before sleep. Urine samples (5 mL) were extracted at 2, 3, 4, 5, and 6 am sleep stages were monitored with a single-channel portable electroencephalograph device. RESULTS: The estimated bladder volume at 6 am and voided volume immediately after rising were significantly correlated (Spearman's ρ = 0.62, P = .046). Eight participants (three males, five females) showed an absorption pattern of the estimated bladder volume change. In a male participant, the blue dye's strength gradually decreased until 4 am (estimated 859 mL) and increased from 5 am (estimated 455 mL). In another, the blue dye's strength increased at 4 am (estimated 449 mL) vs at 3 am (estimated 757 mL). In all participants, electroencephalograph data demonstrated that sleep was maintained despite having a full bladder. CONCLUSIONS: The bladder absorbs urine and maintains an approximate volume of functional bladder capacity during sleep to avoid incontinence and maintain sleep in adults due to an urge to void urine during the sleep cycle.
Subject(s)
Brain Waves , Nocturia , Urinary Incontinence , Female , Humans , Male , Sleep , Urinary Bladder/diagnostic imaging , UrinationABSTRACT
OBJECTIVES: This study aimed to determine whether Dahl salt-sensitive rats fed a high-salt diet would show features of nocturia due to nocturnal polyuria and to examine the efficacy of choreito (CRT) on nocturnal polyuria. METHODS: Dahl salt-sensitive rats were divided into three groups. Group A was fed a 4% salt diet, group B a 2% salt diet, and group C a normal 0.3% salt diet. In groups α and ß, other rats were further divided into two groups: The rats in group α were fed a 2% salt plus 3% CRT diet, and those in group ß, were fed a 2% salt diet. Each rat was placed in an individual metabolic cage for 24 hours every week for 6 weeks. Water intake, urine production, voiding frequency, and voided volume per micturition were recorded. RESULTS: The systolic blood pressure increased in the group fed a 4% salt diet compared to groups fed with a 2% and 0.3% salt diet. The urinary volume was higher in the groups fed with 4% and 2% salt than in the group fed with 0.3% salt. Further, water intake in the group fed a 2% salt plus 3% CRT diet was significantly lower than that in the group fed with a 2% salt diet. CONCLUSIONS: Dahl salt-sensitive rats fed a 2% salt diet were candidates for a model of nocturnal polyuria. Using this model, we suggest that CRT reduces water intake in the active phase and contributes to water restriction in the treatment of nocturnal polyuria.
Subject(s)
Hypertension , Nocturia , Animals , Blood Pressure , Drugs, Chinese Herbal , Hypertension/complications , Nocturia/etiology , Polyuria/complications , Rats , Rats, Inbred DahlABSTRACT
OBJECTIVES: To evaluate the efficacy, safety and tolerability of vibegron for the treatment of antimuscarinic-resistant neurogenic bladder dysfunction in patients with spina bifida. METHODS: In this retrospective study, 15 patients with antimuscarinic-resistant neurogenic bladder dysfunction due to spina bifida underwent a video-urodynamic study before and during the administration of vibegron 50 mg once daily instead of antimuscarinic agents from February 2019 through April 2021. The video-urodynamic study was carried out to evaluate bladder compliance, maximum cystometric bladder capacity, detrusor overactivity, detrusor leak point pressure and vesicoureteral reflux before and >3 months after the beginning of vibegron administration. RESULTS: Treatment with vibegron significantly improved bladder compliance and maximum cystometric bladder capacity compared with antimuscarinic agents, respectively (7.4 ± 4.2 vs 30.4 ± 48.2 mL/cmH2 O, P = 0.0001; 231.4 ± 81.2 vs 325.2 ± 106.5 mL, P = 0.0005). Detrusor overactivity did not change after the administration of vibegron. Bladder deformity, which was confirmed in 12 patients, improved in half of the patients after taking vibegron. Vesicoureteral reflux, which was confirmed in two patients, was extinguished after taking vibegron. Newly occurring adverse events were not observed, and all patients continued to take vibegron during the treatment period. CONCLUSIONS: Favorable efficacy of vibegron for antimuscarinic-resistant neurogenic bladder dysfunction due to spina bifida was shown video-urodynamically without apparent adverse events. Vibegron is a favorable option for the treatment of antimuscarinic-resistant neurogenic bladder dysfunction in patients with spina bifida.
Subject(s)
Spinal Dysraphism , Urinary Bladder, Neurogenic , Humans , Muscarinic Antagonists/adverse effects , Pyrimidinones , Pyrrolidines , Receptors, Adrenergic , Retrospective Studies , Urinary Bladder, Neurogenic/drug therapy , Urinary Bladder, Neurogenic/etiology , UrodynamicsABSTRACT
Patients who contract severe renal infections often suffer from urosepsis. Therefore, early diagnosis and treatment are required. Sometimes, the treatment with antibiotics is not enough for control of the infections. Most of the patients also require surgical interventions including transurethral drainage and nephrectomy. Twenty-two patients with severe renal infections treated between April 2010 and October 2019 at our institute were evaluated retrospectively. Eleven patients had undergone nephrectomy. Open nephrectomy was performed on 10 patients. Laparoscopic nephrectomy was attempted in the other patient but was converted to open nephrectomy because severe adhesion was found around the tissues. Nephrectomy was performed by the retroperitoneal approach on 9 patients and by the transperitoneal approach on 2 patients. The reteroperitoneal approach was used on two patients who suffered postoperative colon perforation. Inflammatory involvement of renal pelvis, hilum and adjacent structures leads to dense fibrotic reaction and obliteration of tissue planes, which makes the nephrectomy procedure challenging. Therefore, it is important to choose the most appropriate nephrectomy procedure for each patient when they have contracted severe renal infections.
Subject(s)
Kidney Neoplasms , Laparoscopy , Humans , Kidney , Kidney Neoplasms/surgery , Nephrectomy , Retrospective Studies , Treatment OutcomeABSTRACT
OBJECTIVES: To investigate the natural course of retractile testis by analyzing its prevalence and outcomes. METHODS: This retrospective study included 215 boys in whom retractile testis was diagnosed after reviewing the medical history and physical examinations of the patients. Orchiopexy was performed once the testis became undescended. We investigated the trends in the prevalence and outcomes of retractile testis and compared clinical factors between cases that resolved spontaneously and those that required orchiopexy. RESULTS: Of 215 boys, 145 were finally evaluated. The mean age at diagnosis was 2 years, and 100 boys were aged ≤2 years when they were hospitalized. Seventy-three boys were referred to our institution through health examinations as babies. The condition improved spontaneously in 89 boys, while 43 boys underwent orchiopexy, and 13 boys remained under follow-up. The follow-up period between diagnosis and resolution was significantly longer in the spontaneous resolution group than in the surgical intervention group (P = 0.011). Bilateral retractile testis improved spontaneously in significantly more boys compared to unilateral retractile testis (P = 0.0010). Spontaneous resolution was observed in boys of all ages, but those diagnosed at ≤3 years of age had a significantly higher rate of spontaneous resolution compared to those who were diagnosed at >3 years of age (P = 0.0019). CONCLUSIONS: Our findings suggest that retractile testis cannot be affirmed as a variant of normal testis. Performing examinations at a young age is critical for preventing misdiagnosis, screening failures, and unnecessary surgery.
Subject(s)
Cryptorchidism , Testis , Aged , Child, Preschool , Cryptorchidism/epidemiology , Cryptorchidism/surgery , Humans , Infant , Japan/epidemiology , Male , Retrospective Studies , Testis/surgery , Treatment OutcomeABSTRACT
INTRODUCTION: Hypoparathyroidism, sensorineural deafness, and renal dysplasia syndrome is an autosomal dominant rare genetic disease. Some patients with hypoparathyroidism, sensorineural deafness, and renal dysplasia syndrome may present with renal calcification (nephrocalcinosis) and disorder. We report the first case of living-donor kidney transplantation for a patient with hypoparathyroidism, sensorineural deafness, and renal dysplasia syndrome. CASE PRESENTATION: This case pertains to a 26-year-old woman who was diagnosed with congenital hypoparathyroidism 1 month after birth, following which vitamin D supplementation was initiated. In 20XX, she developed nephrocalcinosis and was confirmed to have a GATA3 mutation; hence, she was diagnosed with hypoparathyroidism, sensorineural deafness, and renal dysplasia syndrome. In 20XX + 7, ABO-incompatible living-donor kidney transplantation was performed. Her renal function improved, and graft calcification was not observed. CONCLUSION: Over intake of vitamin D caused nephrocalcinosis. The renal function was improved after living-donor kidney transplantation and the patient's serum calcium levels normalized without vitamin D supplementation. Therefore, kidney transplantation should be considered a treatment option for patients with hypoparathyroidism, sensorineural deafness, and renal dysplasia syndrome.
ABSTRACT
PURPOSE: Cryptorchidism is one of the most common congenital abnormalities in pediatric urology, and orchiopexy is performed for the prevention of testicular damage and malignant transformation. We examined the distribution and outcomes of cryptorchidism under a single investigator at our institute. PATIENTS AND METHODS: This retrospective study included 283 boys diagnosed with cryptorchidism at our institute. Cryptorchidism was diagnosed based on the medical history and physical examination findings. Boys without spontaneous resolution after 6 months of age were indicated for orchiopexy. We investigated the 12-year trend in the distribution and outcomes of cryptorchidism at the institute. RESULTS: The mean age at diagnosis, gestational age, and birth weight were 2 years, 37 weeks, and 2740 g, respectively. A total of 170 boys underwent orchiopexy under 2 years of age, and 136 boys underwent orchiopexy under the age of 1 year, while 62 boys underwent orchiopexy over the age of 3 years. Abnormalities of the epididymis and disclosure of the processus vaginalis were observed in 44 (25%) and 72 boys (41%), respectively. Comparison of boys with or without hypospadias showed that the age at orchiopexy was higher in boys with hypospadias than in those without hypospadias (P=0.028). In addition, boys without hypospadias had a higher rate of abnormality of the epidermis than those with hypospadias (P=0.024). CONCLUSION: Our findings suggest that most boys with cryptorchidism are treated under the age of 2 years and the incidence of epididymal abnormality is relatively high, especially in boys with hypospadias. An understanding of the natural features of cryptorchidism could lead to better management and outcomes. Further research is warranted to develop an appropriate treatment timeline in boys with cryptorchidism.
ABSTRACT
PURPOSE: To determine the clinical efficacy of endoscopic transurethral incision (TUI) for boys with refractory daytime incontinence due to a posterior urethral valve with or without nocturnal enuresis. PATIENTS AND METHODS: A total of 20 boys with daytime incontinence were assessed. Twelve boys underwent TUI (TUI+ group) and eight boys continued receiving oral drugs (TUI- group). The primary endpoint was the cure rate associated with TUI or NE in both groups. RESULTS: Only two boys achieved daytime continence 6 months after TUI, but no boys were cured of nocturnal enuresis 6 months after TUI. The median time to daytime continence was significantly longer in the TUI+ than in the TUI- group (52 vs 27 months, respectively; log rank P = 0.041) and the median time to dry nights was significantly longer in the TUI+ than in the TUI- group (56 vs 36 months, respectively; log rank P = 0.021). CONCLUSION: TUI might be not effective in boys with refractory daytime incontinence.
ABSTRACT
INTRODUCTION: Spina bifida is a congenital anomaly caused by a neural tube closure defect that may result in sexual dysfunction. Sexual dysfunction and infertility are prevalent in spina bifida patients but have been scarcely reported. CASE PRESENTATION: We report the case of a 36-year-old man with spina bifida and mild-moderate erectile dysfunction. He could experience erection and ejaculation. He got married at the age of 28 years, but his wife was unable to conceive for 2 years thereafter. Semen analyses revealed that semen volume, sperm density, and sperm motility rate were below normal levels. It was concluded that natural conception would be difficult, and assisted reproductive strategies were planned. After 4 years, his wife conceived through intracytoplasmic sperm injection and gave birth to a healthy baby. CONCLUSION: Fertility treatment, including intracytoplasmic sperm injection, is a useful therapeutic method for male patients with spina bifida who desire to father a child.
ABSTRACT
OBJECTIVES: To investigate trends in treatment outcomes of surgical intervention versus observation for pediatric hydrocele. METHODS: This retrospective study included 175 patients diagnosed with hydrocele at our institution. Hydrocele was diagnosed based on medical history, physical examination and ultrasonography findings. The treatment for these patients was divided into two options: surgical intervention or careful follow up; the outcomes were investigated. RESULTS: The median age at diagnosis was 3 months, and a total of 11 patients (6%) were premature at birth. Hydrocele was diagnosed on the right side, the left side and bilaterally in 106 (61%), 46 (26%) and 23 (13%) patients, respectively. A total of 136 patients showed spontaneous improvement at the median 7 months after diagnosis, and 54 patients underwent surgical intervention. The rate of spontaneous resolution deceased with age, but spontaneous resolution was observed in patients aged >2 years. CONCLUSIONS: Our findings suggest that spontaneous resolution can be observed in patients aged >2 years, and surgical intervention can be carried out effectively and safely. Infant hydrocele should be followed up carefully for at least 1 year without surgical intervention since diagnosis. Investigation of the optimal timing of and appropriate reason for surgical intervention can lead to better management and outcomes in patients with hydrocele. Further research is warranted to support the current clinical practice.
Subject(s)
Testicular Hydrocele , Aged , Child , Child, Preschool , Humans , Infant , Japan/epidemiology , Male , Retrospective Studies , Testicular Hydrocele/diagnostic imaging , Testicular Hydrocele/epidemiology , Treatment OutcomeABSTRACT
BACKGROUND: Nocturia is one of the most bothersome lower urinary tract symptoms and often impairs sleep quality in the elderly. Although previous studies on nocturia have indicated that the successful treatment of nocturia improves sleep quality, most used questionnaires and activity devices to analyze sleep/wake patterns. Therefore, there is little information about the treatment effects of desmopressin on objective sleep quality. The aim of the DISTINCT study is to investigate the change in subjective and objective sleep quality using electroencephalography (EEG) and the Pittsburgh Sleep Quality Index (PSQI) after the administration of desmopressin in patients with nocturia due to nocturnal polyuria. METHODS: A total of 20 male patients, ≥65 years old, with nocturnal polyuria, defined as a nocturnal polyuria index (NPi) (nocturnal urine volume / 24 h urine volume) value ≥0.33, will participate in this study. The participants must have a nocturnal frequency of ≥2 and the first uninterrupted sleep period (FUSP) must occur within < 2.5 h. Desmopressin 50 µg per day will be orally administered before going to bed for 4 weeks. Urinary frequency volume charts (FVC) and EEG will be recorded prior to treatment and at 1 week and 4 weeks after the initiation of treatment. The PSQI will be completed before and 4 weeks after treatment. The primary endpoint is the change from baseline in the mean time of slow-wave sleep (sleep stages N3 and N4) at 4 weeks. The secondary endpoints include the change in the mean value of each sleep variable, the mean delta power during the FUSP, the correlation between nocturnal urinary frequency and slow-wave sleep time, and the change in PSQI score before and after treatment. DISCUSSION: The DISTINCT study will provide valuable evidence to indicate that oral desmopressin treatment for nocturnal polyuria prolongs the FUSP, resulting in the extension of slow-wave sleep time associated with sleep quality. TRIAL REGISTRATION: The Japan Registry of Clinical Trials ( jRCTs051190080 ). Registered 9 December, 2019.