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1.
Ann Plast Surg ; 92(2): 208-211, 2024 Feb 01.
Artigo em Inglês | MEDLINE | ID: mdl-38170969

RESUMO

ABSTRACT: Klippel-Trenaunay syndrome (KTS) is characterized by port-wine stains, mixed vascular malformations, and soft tissue and bone hypertrophy. Klippel-Trenaunay syndrome is occasionally complicated by chyluria, for which there is no effective treatment currently. We report a case of KTS complicated by intractable chyluria and hematuria due to a lymphatic-ureteral fistula. The patient was successfully treated with multiple lymphaticovenular anastomoses (LVAs).A 66-year-old woman with an enlarged left lower extremity since childhood was diagnosed with KTS. At 60 years of age, she developed chyluria (urine albumin, 2224 µg/mL) and hematuria. Lymphoscintigraphy showed a lymphatic-ureteral fistula near the ureterovesical junction. Conservative treatment was ineffective. She also developed left lower extremity lymphedema, which gradually worsened. Leg cellulitis and purulent pericarditis developed because of hypoalbuminemia (minimum serum albumin level, 1.3 g/dL).We performed 14 LVAs in 2 surgeries to reduce lymphatic fluid flow through the lymphatic-ureteral fistula. The chyluria and hematuria resolved soon after the second operation, and the urine albumin level decreased (3 µg/mL). After 28 months, she had no chyluria or hematuria recurrence and her serum albumin level improved (3.9 g/dL). Multiple LVAs can definitively treat chyluria caused by a lymphatic-ureteral fistula in patients with KTS.


Assuntos
Fístula , Síndrome de Klippel-Trenaunay-Weber , Linfedema , Humanos , Feminino , Criança , Idoso , Síndrome de Klippel-Trenaunay-Weber/complicações , Síndrome de Klippel-Trenaunay-Weber/cirurgia , Síndrome de Klippel-Trenaunay-Weber/diagnóstico , Hematúria/complicações , Extremidade Inferior/irrigação sanguínea , Linfedema/cirurgia , Linfedema/complicações , Fístula/complicações , Albumina Sérica
2.
Nephrology (Carlton) ; 29(2): 65-75, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-37871587

RESUMO

AIM: Among patients with Immunoglobulin A (IgA) nephropathy, we aimed to identify trajectory patterns stratified by the magnitude of haematuria and proteinuria using repeated urine dipstick tests, and assess whether the trajectories were associated with kidney events. METHODS: Using a nationwide multicentre chronic kidney disease (CKD) registry, we analysed data from 889 patients with IgA nephropathy (mean age 49.3 years). The primary outcome was a sustained reduction in eGFR of 50% or more from the index date and thereafter. During follow-up (median 49.0 months), we identified four trajectories (low-stable, moderate-decreasing, moderate-stable, and high-stable) in both urine dipstick haematuria and proteinuria measurements, respectively. RESULTS: In haematuria trajectory analyses, compared to the low-stable group, the adjusted hazard ratios (HRs) (95% confidence interval [CI]) for kidney events were 2.59 (95% CI, 1.48-4.51) for the high-stable, 2.31 (95% CI, 1.19-4.50) for the moderate-stable, and 1.43 (95% CI, (0.72-2.82) for the moderate-decreasing groups, respectively. When each proteinuria trajectory group was subcategorized according to haematuria trajectories, the proteinuria group with high-stable and with modest-stable haematuria trajectories had approximately 2-times higher risk for eGFR reduction ≥50% compared to that with low-stable haematuria trajectory. CONCLUSION: Assessments of both haematuria and proteinuria trajectories using urine dipstick could identify high-risk IgA nephropathy patients.


Assuntos
Glomerulonefrite por IGA , Insuficiência Renal Crônica , Humanos , Pessoa de Meia-Idade , Glomerulonefrite por IGA/complicações , Glomerulonefrite por IGA/diagnóstico , Hematúria/etiologia , Hematúria/complicações , Japão/epidemiologia , Rim , Proteinúria/etiologia , Proteinúria/complicações , Insuficiência Renal Crônica/complicações , Insuficiência Renal Crônica/diagnóstico , Insuficiência Renal Crônica/epidemiologia , Taxa de Filtração Glomerular
3.
J Wound Ostomy Continence Nurs ; 50(6): 504-511, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-37966080

RESUMO

PURPOSE: To assess the performance of a new urinary intermittent catheter (IC) prototype designed with a micro-hole drainage zone compared to a conventional eyelet catheter (CEC) in terms of flow-stop, bladder emptying, and hematuria. DESIGN: Randomized controlled crossover studies. SUBJECT AND SETTING: The sample comprised 15 male healthy volunteers (HV) and 15 IC users, along with 15 female HV and 15 IC users. The age range was lower for HV participants than for IC users (range: 20-57 years for HV vs 21-82 years for IC users). The study setting was the Department of Urology, located in Rigshospitalet, Copenhagen. METHODS: Number of flow-stop incidents, residual urine volume at first flow-stop (RV1), and dipstick hematuria were measured during and after catheterization by a health care professional (HV) and by self-catheterisation (IC-users). Results from the 3 studies were combined for HV and IC users on RV1 and number of flow-stop incidents but separated on sex. For incidents of hematuria, an effect of underlying condition was assumed, and a combined analysis on sex was performed, separating HV and IC users. RESULTS: When compared to the micro-hole drainage zone design, catheterizations with CEC resulted in a significantly higher mean RV1 (mean difference: 49 mL in males and 32 mL in females, both P < .001) and average number of flow-stop incidents (8 and 21 times more frequent for males and females, respectively, both P < .001). The likelihood for hematuria was 5.84 higher with CEC than with micro-hole drainage hole design, P = .053, during normal micturition in HV postcatheterization. No serious adverse events were reported. CONCLUSION: The micro-hole drainage zone catheter provides IC users fewer premature flow-stops. This design feature reduces modifiable urinary tract infection risk factors, such as residual urine and micro-trauma; additional research is needed to determine its effects on bladder health.


Assuntos
Cateterismo Urinário , Infecções Urinárias , Adulto , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Adulto Jovem , Hematúria/complicações , Tecnologia , Bexiga Urinária , Cateterismo Urinário/métodos , Cateteres Urinários/efeitos adversos , Infecções Urinárias/etiologia , Estudos Cross-Over
4.
Recenti Prog Med ; 114(12): 749-754, 2023 Dec.
Artigo em Italiano | MEDLINE | ID: mdl-38031858

RESUMO

A healthy 9-years-old boy was brought to the Emergency Department for widespread abdominal pain associated with bloody diarrhoea and significant tenesmus, in the absence of fever. Blood tests were compatible with an acute gastroenteritis, even though microbiological tests on stools resulted negative. Given the haemorrhagic dysentery, the boy was hospitalized to start empiric antibiotic therapy and intravenous rehydration. Abdominal ultrasound showed a thickening of colonic walls, mimicking an inflammatory intestinal disease at the onset (subsequently denied by gastro-colonoscopy). Seven days after the onset of symptoms, blood tests revealed microangiopathic anaemia with negative Coombs test, associated with thrombocytopenia. Urine dipstick revealed haematuria and proteinuria in nephritic range. No contraction of diuresis or alteration of renal function were observed (being creatinine values always within the normal range). Laboratory tests were consistent with the diagnosis of Haemolytic Uremic Syndrome (Hus) at the onset. Approximately 1% of paediatric patients with bloody diarrhoea can develop Hus. Positivity for Escherichia coli is not always evident in the stools. Thus, the triad of haemolytic anaemia, thrombocytopenia and renal failure could be present in only 60% of Hus at the onset. The finding of haematuria and/or proteinuria on the urine dipstick may be indicative of early kidney damage, allowing for careful monitoring and a rehydration program that can prevent progression of kidney damage and extrarenal complications.


Assuntos
Síndrome Hemolítico-Urêmica , Trombocitopenia , Masculino , Humanos , Criança , Hematúria/complicações , Síndrome Hemolítico-Urêmica/complicações , Síndrome Hemolítico-Urêmica/diagnóstico , Síndrome Hemolítico-Urêmica/terapia , Diarreia/complicações , Diarreia/terapia , Trombocitopenia/complicações , Hemorragia Gastrointestinal/etiologia , Proteinúria/complicações , Rim
5.
Zhongguo Dang Dai Er Ke Za Zhi ; 25(7): 732-738, 2023 Jul 15.
Artigo em Chinês | MEDLINE | ID: mdl-37529956

RESUMO

OBJECTIVES: To investigate the genotypes of the pathogenic gene COL4A5 and the characteristics of clinical phenotypes in children with Alport syndrome (AS). METHODS: A retrospective analysis was performed for the genetic testing results and clinical data of 19 AS children with COL4A5 gene mutations. RESULTS: Among the 19 children with AS caused by COL4A5 gene mutations, 1 (5%) carried a new mutation of the COL4A5 gene, i.e., c.3372A>G(p.P1124=) and presented with AS coexisting with IgA vasculitis nephritis; 3 children (16%) had large fragment deletion of the COL4A5 gene, among whom 2 children (case 7 had a new mutation site of loss51-53) had gross hematuria and albuminuria at the onset, and 1 child (case 13 had a new mutation site of loss3-53) only had microscopic hematuria, while the other 15 children (79%) had common clinical phenotypes of AS, among whom 7 carried new mutations of the COL4A5 gene. Among all 19 children, 3 children (16%) who carried COL4A5 gene mutations also had COL4A4 gene mutations, and 1 child (5%) had COL4A3 gene mutations. Among these children with double gene mutations, 2 had gross hematuria and proteinuria at the onset. CONCLUSIONS: This study expands the genotype and phenotype spectrums of the pathogenic gene COL4A5 for AS. Children with large fragment deletion of the COL4A5 gene or double gene mutations of COL4A5 with COL4A3 or COL4A4 tend to have more serious clinical manifestations.


Assuntos
Nefrite Hereditária , Humanos , Nefrite Hereditária/genética , Nefrite Hereditária/complicações , Nefrite Hereditária/patologia , Hematúria/genética , Hematúria/complicações , Estudos Retrospectivos , Colágeno Tipo IV/genética , Genótipo , Mutação
6.
AIDS Res Ther ; 20(1): 49, 2023 07 15.
Artigo em Inglês | MEDLINE | ID: mdl-37454082

RESUMO

BACKGROUND: Kidney disease is an important comorbidity in people living with HIV(PLWH), and is associated with poor outcomes. However, data on renal function of PLWH are limited in China so far. In this study we assessed the prevalence of kidney disease in patients either on antiretroviral therapy (ART) or not respectively in a single center in China and explored the possible risk factors associated. METHODS: In the cross-sectional study, we recruited hospitalized adult PLWH. Demographic characteristics, clinical information and laboratory variables were collected. Kidney disease was defined as estimated glomerular filtration rate (eGFR) < 60 mL/min/1.73 m2, and/or isolated hematuria, proteinuria, microalbuminuria. We calculated the prevalence of kidney disease and used logistic regression to assess its associated risk factors. RESULTS: A total of 501 adult PLWH were enrolled, 446 (89.0%) males and 55 (11.0%) females. The median age was 39 (IQR 30-50) years old. The prevalence of kidney disease was 19.0%, 22 (4.4%) patients with eGFR < 60 mL/min/1.73 m2, 53 (10.6%) patients with hematuria, 11 (2.2%) patients with proteinuria, and 40 (8.0%) patients with microalbuminuria. 297 (59.3%) patients were receiving ART. The patients on ART had a higher prevalence of renal disease than those had not been administrated with ART (22.6% vs. 13.7%, P = 0.013). On the multivariate logistic regression analysis among patients not on ART, lower haemoglobin (OR 0.994, 95%CI: 0.902-0.988, P = 0.013) were significantly associated with kidney disease. While among those on ART, older age (OR 1.034, 95%CI: 1.003-1.066, P = 0.032), lower haemoglobin (OR 0.968, 95%CI: 0.948-0.988, P = 0.002) and lower albumin (OR 0.912, 95%CI: 0.834-0.997, P = 0.044) were significantly associated with kidney disease. CONCLUSIONS: The prevalence of kidney disease among hospitalized PLWH in China is high, especially in patients on ART. A larger scale study on Chinese outpatient PLWH should be conducted, so as to precisely assess prevalence of kidney disease in general Chinese PLWH.


Assuntos
Infecções por HIV , Nefropatias , Adulto , Masculino , Feminino , Humanos , Pessoa de Meia-Idade , Infecções por HIV/complicações , Infecções por HIV/tratamento farmacológico , Infecções por HIV/epidemiologia , Prevalência , Hematúria/epidemiologia , Hematúria/complicações , Estudos Transversais , Fatores de Risco , Nefropatias/epidemiologia , Taxa de Filtração Glomerular , Proteinúria/epidemiologia , Proteinúria/complicações , China/epidemiologia
7.
BMC Pediatr ; 23(1): 377, 2023 07 26.
Artigo em Inglês | MEDLINE | ID: mdl-37495962

RESUMO

BACKGROUND: There is no standard recommendation for IgA nephropathy treatment in children. METHODS: This is a retrospective study. From 2012 to 2020, newly diagnosed primary IgAN followed up for at least 1 year were enrolled. The correlation of MESTC scores and clinical index including proteinuria, gross hematuria and renal dysfunction was analyzed. Treatment and clinical response of 6 month, 1year and 3 year at follow up were also analyzed. Complete renal remission was calculated with Kaplan-Meier analysis. RESULTS: The median follow up was 36 months, from 12 months to 87months in 40 IgAN children. Angiotensin-converting enzyme inhibitor (ACEI) was applied to all patients. 30% received ACEI alone; 15% received glucocorticoids; 37.5% received glucocorticoids plus cyclophosphamide, 17.5% received glucocorticoids plus mycophenolate mofetil. Individuals with diffuse mesangial hypercellularity (M1) were more likely to have nephrotic range proteinuria compared to patients with M0 (80% vs. 20%, P < 0.01). Complete renal remission at 6-month, 1-year and 3-year follow up is 50.25%, 70% and 87.5% respectively. Five-year complete renal remission calculated by Kaplan-Meier analysis is 58.4%. Although without significant difference, there is trend of better survival with complete renal remission in group of nephrotic range proteinuria onset. There is no severe adverse effect. CONCLUSION: This study supports the use of glucocorticoids plus immunosuppressive in addition to ACEI in IgA nephrology pediatric patients with proteinuria. We suggest proactive immunosuppressive treatment in IgA nephropathy in children. This is from a single center in China as may not same results in other population.


Assuntos
Inibidores da Enzima Conversora de Angiotensina , Glomerulonefrite por IGA , Glucocorticoides , Imunossupressores , Estudos Retrospectivos , Glomerulonefrite por IGA/sangue , Glomerulonefrite por IGA/complicações , Glomerulonefrite por IGA/tratamento farmacológico , Glomerulonefrite por IGA/patologia , Humanos , Masculino , Feminino , Criança , Biópsia , Proteinúria/complicações , Estimativa de Kaplan-Meier , Glucocorticoides/administração & dosagem , Glucocorticoides/efeitos adversos , Glucocorticoides/uso terapêutico , Pressão Intraocular/efeitos dos fármacos , Linfócitos T CD4-Positivos/citologia , Linfócitos T CD4-Positivos/efeitos dos fármacos , Linfócitos T CD4-Positivos/imunologia , Resultado do Tratamento , Seguimentos , Hematúria/complicações , Nefropatias/complicações , Fatores de Tempo , Inibidores da Enzima Conversora de Angiotensina/administração & dosagem , Inibidores da Enzima Conversora de Angiotensina/uso terapêutico , Ciclofosfamida/uso terapêutico , Ácido Micofenólico/uso terapêutico , Análise de Sobrevida , Imunossupressores/administração & dosagem , Imunossupressores/uso terapêutico , China , População do Leste Asiático
8.
Lupus ; 32(9): 1084-1092, 2023 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-37480363

RESUMO

BACKGROUND: This study aimed to explore risk factors for lupus nephritis (LN) in systemic lupus erythematosus (SLE) patients and establish a Nomogram prediction model based on LASSO-logistic regression. METHODS: The clinical and laboratory data of SLE patients in Meishan People's Hospital from July 2012 to December 2021 were analyzed retrospectively. All SLE patients were divided into two groups with or without LN. Risk factors were screened based on LASSO-logistic regression analysis, and a Nomogram prediction model was established. The receiver operating characteristic curve, calibration curves, and decision curve analysis were adopted to evaluate the performance of the Nomogram model. RESULTS: A total of 555 SLE patients were enrolled, including 303 SLE patients with LN and 252 SLE patients without LN. LASSO regression and multivariate logistic regression analyses showed that ESR, mucosal ulcer, proteinuria, and hematuria were independent risk factors for LN in SLE patients. The four clinical features were incorporated into the Nomogram prediction model. Results showed that calibration curve was basically close to the diagonal dotted line with slope 1 (ideal prediction case), which proved that the prediction ability of the model was acceptable. In addition, the decision curve analysis showed that the Nomogram prediction model could bring net clinical benefits to patients when the threshold probability was 0.12-0.54. CONCLUSION: Four clinical indicators of ESR, mucosal ulcer, proteinuria, and hematuria were independent risk factors for LN in SLE patients. The predictive power of the Nomogram model based on LASSO-logistic regression was acceptable and could be used to guide clinical work.


Assuntos
Lúpus Eritematoso Sistêmico , Nefrite Lúpica , Humanos , Nefrite Lúpica/complicações , Lúpus Eritematoso Sistêmico/complicações , Estudos Retrospectivos , Nomogramas , Hematúria/complicações , Úlcera , Biomarcadores , Proteinúria/complicações , Fatores de Risco
9.
BMC Nephrol ; 24(1): 156, 2023 06 05.
Artigo em Inglês | MEDLINE | ID: mdl-37277729

RESUMO

BACKGROUND: Arteriovenous fistula (AVF) due to renal allograft biopsy is mechanical trauma resulting from the penetration of small arteries and veins by a core needle. Most AVFs are reported to resolve asymptomatically and spontaneously. This report presents a patient with acute kidney injury (AKI) due to urinary tract obstruction caused by a bleeding AVF in a renal allograft. CASE PRESENTATION: A 22-year-old Japanese woman who underwent living-donor kidney transplantation (KT) at 3 years due to end-stage renal disease caused by focal segmental glomerulosclerosis (FSGS) presented with a renal transplant AVF (gourd-shaped; 42 × 19 × 20 mm). The AVF was unexpectedly discovered by ultrasound before a surveillance biopsy at 10 years after KT. The patient had a history of recurrent FSGS, had undergone several renal allograft biopsies after KT, and did not experience symptoms or growth of the AVF for years. Nineteen years after KT, the patient developed AKI with sudden, asymptomatic, gross hematuria and anuria. Plain computed tomography revealed a hematoma in the pelvis of the renal allograft and bladder tamponade. The AVF was successfully treated by coil embolization. Hemodialysis was performed for AKI, and graft function was gradually recovered. CONCLUSIONS: Unexpected bleeding from a renal transplant AVF may lead to transplant dysfunction. Angiographic embolization against the ruptured renal transplant AVF may prevent rebleeding and rescue the renal allograft.


Assuntos
Injúria Renal Aguda , Fístula Arteriovenosa , Glomerulosclerose Segmentar e Focal , Transplante de Rim , Feminino , Humanos , Adulto Jovem , Adulto , Transplante de Rim/efeitos adversos , Hematúria/complicações , Glomerulosclerose Segmentar e Focal/patologia , Rim/patologia , Fístula Arteriovenosa/complicações , Fístula Arteriovenosa/diagnóstico por imagem , Injúria Renal Aguda/terapia , Injúria Renal Aguda/complicações , Aloenxertos
10.
Transfus Apher Sci ; 62(5): 103732, 2023 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-37263885

RESUMO

BACKGROUND: Actinic cystitis is a severe complication after radiotherapy for prostate cancer. It is a chronic inflammatory process that leads to an alteration of bladder mucosa with formation of petechiae and subsequently hematuria. Actinic cystitis responds poorly to medical treatment, with a heavy burden on patients' quality of life. Patients with refractory hematuria may undergo cystectomy in the attempt to control bleeding. We conducted a prospective study to evaluate the effectiveness of the allogeneic platelet growth factors for actinic cystitis. METHODS AND MATERIAL: Nine patients with actinic cystitis were enrolled in this study. The primary outcome measures were the effects of the platelet growth factors on the injury of the bladder mucosa. The secondary outcome was the change in quality of life RESULTS: A total of 9 patients, mean age 68 (range 59-81) underwent a therapeutic program of bladder instillation with allogeneic platelets growth factors for 3 months. Of the 9 patients, all (100 %) had complete resolution of hematuria and urinary symptoms. After three months cystoscopy showed regeneration of the normal bladder mucosa. Biopsies allowed histological confirmation of the finding. DISCUSSION: The instillation of allogeneic platelet growth factors in actinic cystitis is a new treatment that in this setting of patients appears promising in promoting a resolution of urinary symptoms, hematuria and avoiding a disabling surgery such as cystectomy.


Assuntos
Cistite , Transplante de Células-Tronco Hematopoéticas , Masculino , Humanos , Pré-Escolar , Criança , Hematúria/complicações , Estudos Prospectivos , Qualidade de Vida , Cistite/tratamento farmacológico , Cistite/etiologia , Transplante de Células-Tronco Hematopoéticas/efeitos adversos
11.
Beijing Da Xue Xue Bao Yi Xue Ban ; 55(3): 553-557, 2023 Jun 18.
Artigo em Chinês | MEDLINE | ID: mdl-37291934

RESUMO

OBJECTIVE: To compare the safety and effectiveness of active migration technique and in situ lithotripsy technique in the treatment of 1-2 cm upper ureteral calculi by retrograde flexible ureteroscopy. METHODS: A total of 90 patients with 1-2 cm upper ureteral calculi treated in the urology department of Beijing Friendship Hospital from August 2018 to August 2020 were selected as the subjects. The patients were divided into two groups using random number table: 45 patients in group A were treated with in situ lithotripsy and 45 patients in group B were treated with active migration technique. The active migration technique was to reposition the stones in the renal calyces convenient for lithotripsy with the help of body position change, water flow scouring, laser impact or basket displacement, and then conduct laser lithotripsy and stone extraction. The data of the patients before and after operation were collected and statistically analyzed. RESULTS: The age of the patients in group A was (51.6±14.1) years, including 34 males and 11 females. The stone diameter was (1.48±0.24) cm, and the stone density was (897.8±175.9) Hu. The stones were located on the left in 26 cases and on the right in 19 cases. There were 8 cases with no hydronephrosis, 20 cases with grade Ⅰ hydronephrosis, 11 cases with grade Ⅱ hydronephrosis, and 6 cases with grade Ⅲ hydronephrosis. The age of the patients in group B was (51.8±13.7) years, including 30 males and 15 females. The stone diameter was (1.52±0.22) cm, and the stone density was (964.6±214.2) Hu. The stones were located on the left in 22 cases and on the right in 23 cases. There were 10 cases with no hydronephrosis, 23 cases with grade Ⅰ hydronephrosis, 8 cases with grade Ⅱ hydronephrosis, and 4 cases with grade Ⅲ hydronephrosis. There was no significant diffe-rence in general parameters and stone indexes between the two groups. The operation time of group A was (67.1±16.9) min and the lithotripsy time was (38.0±13.2) min. The operation time of group B was (72.2±14.8) min and the lithotripsy time was (40.6±12.6) min. There was no significant difference between the two groups. Four weeks after operation, the stone-free rate in group A was 86.7%, and in group B was 97.8%. There was no significant difference between the two groups. In terms of complications, 25 cases of hematuria, 16 cases of pain, 10 cases of bladder spasm and 4 cases of mild fever occurred in group A. There were 22 cases of hematuria, 13 cases of pain, 12 cases of bladder spasm and 2 cases of mild fever in group B. There was no significant difference between the two groups. CONCLUSION: Active migration technique is safe and effective in the treatment of 1-2 cm upper ureteral calculi.


Assuntos
Hidronefrose , Litotripsia a Laser , Litotripsia , Cálculos Ureterais , Masculino , Feminino , Humanos , Adulto , Pessoa de Meia-Idade , Idoso , Cálculos Ureterais/cirurgia , Hematúria/complicações , Hematúria/terapia , Ureteroscopia/efeitos adversos , Ureteroscopia/métodos , Litotripsia/efeitos adversos , Litotripsia/métodos , Litotripsia a Laser/métodos , Hidronefrose/terapia , Hidronefrose/complicações , Dor , Resultado do Tratamento , Estudos Retrospectivos
12.
BMC Nephrol ; 24(1): 124, 2023 05 03.
Artigo em Inglês | MEDLINE | ID: mdl-37138249

RESUMO

BACKGROUND: IgA nephropathy (IgAN) is universally recognized as one of the most common primary glomerular diseases in all ages. Cyclic neutropenia (CN) is a rare haematologic disorder that is associated with mutations of the ELANE gene. The co-occurrence of IgAN and CN is extremely rare. This is the first case report of a patient with IgAN and genetically confirmed CN. CASE PRESENTATION: We report a case of a 10-year-old boy who presented with recurrent viral upper respiratory tract infections accompanied by several episodes of febrile neutropenia, haematuria, proteinuria and acute kidney injury. Upon first admission, his physical examination was unremarkable. His kidney function was impaired, whereas his urine microscopy showed evidence of macroscopic haematuria and proteinuria. Further workup showed elevated IgA. The renal histology was consistent with mesangial and endocapillary hypercellularity with mild crescentic lesions, while immunofluorescence microscopy showed IgA-positive staining, which was characteristic of IgAN. Moreover, genetic testing confirmed the clinical diagnosis of CN, therefore Granulocyte colony-stimulating factor (G-CSF) was initiated to stabilize the neutrophil count. Regarding proteinuria control, the patient was initially treated with an Angiotensin-converting-enzyme inhibitor for approximately 28 months. However, due to progressive proteinuria (> 1 g/24 h), Corticosteroids (CS) were added for a period of 6 months according to the revised 2021 KDIGO guidelines with favorable outcome. CONCLUSIONS: Patients with CN are more susceptible to recurrent viral infections, which can trigger IgAN attacks. In our case CS induced remarkable proteinuria remission. The use of G-CSF contributed to the resolution of severe neutropenic episodes, viral infections and concomitant AKI episodes, contributing to better prognosis of IgAN. Further studies are mandatory to determine whether there is a genetical predisposition for IgAN in children with CN.


Assuntos
Glomerulonefrite por IGA , Masculino , Criança , Humanos , Glomerulonefrite por IGA/complicações , Glomerulonefrite por IGA/diagnóstico , Glomerulonefrite por IGA/patologia , Hematúria/complicações , Microscopia , Urinálise , Proteinúria/complicações , Corticosteroides/uso terapêutico , Imunoglobulina A
13.
Urology ; 175: 187-189, 2023 05.
Artigo em Inglês | MEDLINE | ID: mdl-36758730

RESUMO

The prostatic utricle cyst is usually an asymptomatic enlargement of the persistent remnant of Mullerian duct in males, especially seen associated with hypospadias and usually needs no active intervention. However occasionally they can become symptomatic and can present with complaints of impotence, ejaculatory pain, difficult voiding, urinary retention and abdominal pain. Hematuria is a rare symptom in such patients and is usually due to infection. However rarely it can be a harbinger of a more sinister complication like a malignancy. Herein, we present an adolescent boy with hematuria due to malignant degeneration in a long-standing prostatic utricle cyst.


Assuntos
Cistos , Lesões Pré-Cancerosas , Neoplasias da Próstata , Masculino , Humanos , Adolescente , Hematúria/complicações , Próstata/patologia , Neoplasias da Próstata/patologia , Uretra , Cistos/complicações , Cistos/diagnóstico , Cistos/patologia , Lesões Pré-Cancerosas/patologia
14.
Int J Med Robot ; 19(3): e2508, 2023 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-36808427

RESUMO

BACKGROUND: Describe the outcomes and safety of robotic-assisted kidney auto-transplantation (RAKAT) in the treatment of nutcracker syndrome (NCS) and loin pain haematuria syndrome (LPHS). METHODS: This retrospective study included 32 cases of NCS and LPHS seen during December 2016 to June 2021. RESULTS: Three (9%) patients had LPHS and 29 (91%) NCS. All were non-Hispanic whites, and 31 (97%) women. The mean age was 32 years (SD = 10) and the BMI 22.8 (SD = 5). The RAKAT was completed in all patients, 63% had a total improvement of pain. According to the Clavien-Dindo classification, 47% presented with type 1, and 9% with type 3 complications with a mean follow-up of 10.9 months. The incidence of acute kidney injury in post-procedure was 28%. No one required blood transfusions, and there were no deaths during the follow-up. CONCLUSION: RAKAT was a feasible procedure with a similar complication rate to those reported for other surgical techniques.


Assuntos
Hematúria , Procedimentos Cirúrgicos Robóticos , Humanos , Feminino , Adulto , Masculino , Hematúria/cirurgia , Hematúria/complicações , Procedimentos Cirúrgicos Robóticos/métodos , Estudos Retrospectivos , Dor/complicações , Rim/cirurgia
15.
Jt Dis Relat Surg ; 34(1): 3-8, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-36700257

RESUMO

OBJECTIVES: This study aims to increase the awareness of the association between lateralized overgrowth (LO) and abdominal tumor among the pediatric orthopedic community and to evaluate its incidence in our center. PATIENTS AND METHODS: Between January 1997 and December 2021, a total of 166 patients with Wilms tumors and hepatoblastomas were retrospectively analyzed. Data including age, sex, initial clinical signs (hematuria, abdominal mass with or without general discomfort), type of asymmetric regional body overgrowth (isolated or in relation with any syndrome), and tumor stage at diagnosis were recorded. In addition, age at which asymmetric regional body overgrowth was described and age at the time of tumor diagnosis were noted. RESULTS: Of a total of 166 patients, 133 were diagnosed with Wilms tumors (nephroblastomas) and 33 were diagnosed with hepatoblastomas. In 94% of the cases, the initial clinical signs were an abdominal mass and/or hematuria. Overall, five (3%) patients presented with LO. Four patients with Wilms tumor presented it at the initial clinical examinations. In three of these cases (2.3%), we found it isolated and, in the remaining patient (0.75%), it was associated with Beckwith-Wiedemann spectrum. Only one patient affected from hepatoblastoma (3%) presented with an isolated LO at the time of tumor diagnosis. CONCLUSION: Our study results show an incidence of LO in relation to intra-abdominal tumors of 3%. The latest updates recommend genetic testing to identify subgroups with a higher risk for tumor development that are more likely to benefit from tumor protocol surveillance.


Assuntos
Neoplasias Abdominais , Síndrome de Beckwith-Wiedemann , Hepatoblastoma , Neoplasias Renais , Neoplasias Hepáticas , Cirurgiões Ortopédicos , Tumor de Wilms , Criança , Humanos , Hepatoblastoma/diagnóstico , Hepatoblastoma/epidemiologia , Hepatoblastoma/complicações , Síndrome de Beckwith-Wiedemann/complicações , Síndrome de Beckwith-Wiedemann/diagnóstico , Síndrome de Beckwith-Wiedemann/genética , Estudos Retrospectivos , Hematúria/complicações , Tumor de Wilms/diagnóstico , Tumor de Wilms/epidemiologia , Tumor de Wilms/etiologia , Neoplasias Abdominais/epidemiologia , Neoplasias Abdominais/complicações , Neoplasias Renais/epidemiologia , Neoplasias Renais/complicações , Neoplasias Hepáticas/diagnóstico , Neoplasias Hepáticas/epidemiologia , Neoplasias Hepáticas/complicações
16.
J Bras Nefrol ; 45(1): 111-115, 2023.
Artigo em Inglês, Português | MEDLINE | ID: mdl-34328493

RESUMO

Tuberous sclerosis complex (TSC) and autosomal dominant polycystic kidney disease (ADPKD) are conditions related to renal failure that can rarely occur in association as a contiguous gene syndrome. Angiomyolipomas (AMLs) are renal tumors strongly related to TSC that may rupture and cause life-threatening bleedings. We present a patient with TSC, ADPKD, and renal AMLs with persistent hematuria requiring blood transfusion. The persistent hematuria was successfully treated through endovascular embolization, a minimally invasive nephron sparing technique.


Assuntos
Angiomiolipoma , Neoplasias Renais , Doenças Renais Policísticas , Rim Policístico Autossômico Dominante , Esclerose Tuberosa , Humanos , Angiomiolipoma/complicações , Angiomiolipoma/terapia , Rim Policístico Autossômico Dominante/complicações , Esclerose Tuberosa/genética , Hematúria/complicações , Doenças Renais Policísticas/complicações , Doenças Renais Policísticas/terapia , Neoplasias Renais/complicações , Hemorragia/etiologia , Hemorragia/terapia
17.
Intern Med J ; 53(10): 1837-1845, 2023 10.
Artigo em Inglês | MEDLINE | ID: mdl-36305476

RESUMO

BACKGROUND: Fibrillary (FGN) and immunotactoid (IT) glomerulonephritis are uncommon. AIMS: To evaluate the prevalence, clinicopathological correlations and outcomes of FGN and IT in our regional centre in Australia. METHODS: We interrogated a renal biopsy database for cases of FGN and IT from 2000 to 2020. Data included demographics, serum creatinine, haematuria status, proteinuria, comorbidities and histopathological findings. RESULTS: We had 14 cases of FGN and t of IT. The mean presenting age was 59.8 years, and 42.9% were males. No patients with FGN had dysproteinaemia, whereas both patients with IT had chronic lymphocytic leukaemia. At presentation, 75% of patients with FGN and both patients with IT had haematuria; all had proteinuria. Mean albumin-creatinine ratio at presentation was 254 mg/mmol for FGN and 604 mg/mmol for IT. Mean presenting serum creatinine was 149 µmol/L for FGN and 95 µmol/L for IT. Four patients with FGN (28.6%) received immunomodulatory therapy. The prognosis of FGN was poor, with six patients (46.2%) reaching end-stage kidney disease after a median of 42 months (range 1-96 months). All patients presenting with proteinuria <30 mg/mmol entered complete remission; patients with higher-grade proteinuria exhibited progressive chronic kidney disease. Patients with IT had complete remission with treatment of underlying haematological disease. CONCLUSION: FGN is rare, with poor response to immunomodulatory therapy. It carries poor renal prognosis. Less proteinuria at diagnosis may predict a more benign disease course. IT is associated with haematological malignancy and carries better prognosis and response to treatment.


Assuntos
Glomerulonefrite , Hematúria , Masculino , Humanos , Pessoa de Meia-Idade , Feminino , Hematúria/diagnóstico , Hematúria/epidemiologia , Hematúria/complicações , Creatinina , Estudos Retrospectivos , Glomerulonefrite/diagnóstico , Glomerulonefrite/epidemiologia , Glomerulonefrite/terapia , Proteinúria/diagnóstico , Proteinúria/epidemiologia , Proteinúria/complicações
18.
Mod Rheumatol Case Rep ; 7(2): 335-339, 2023 06 19.
Artigo em Inglês | MEDLINE | ID: mdl-36508304

RESUMO

Ureteritis associated with the immunological disorder is rarely reported, and most cases in this category are small vessel vasculitis and immunoglobulin G4-related disease. Rheumatoid arthritis (RA)-associated ureteritis is uncommon, and underlying aetiology is unclear. We present a patient with ureteritis who had a medical history of RA and was successfully treated with steroids and immunosuppressant. A 49-year-old woman who had been treated for RA and atopic dermatitis suffered from gross haematuria for 5 successive days. Contrast-enhanced computed tomography (CT) showed right-dominant upper urinary tract dilatation with enhanced thickened wall. The haematuria continued accompanied with intermittent right back and lower abdominal pain, and the following CT image taken after 3 months presented the progression to bilateral hydronephrosis. Ureteral stents were placed, and antibiotic therapy was introduced for obstructive pyelonephritis. Ureterocystoscopy and following biopsy from the upper ureteral tract showed a chronic inflammatory change in the histopathology, and we finally considered the stenosing ureteritis to be caused by immune-mediated mechanism related to RA. After starting steroid therapy with methotrexate, therapeutic response was obtained to remove the stents. In the cases of ureteritis or ureteral stenosis of unknown aetiology with a medical history of immunological disorders, we should consider the underlying immune-activated state and try to test contrast-enhanced CT and histological examination before performing a surgical procedure. After excluding the common causes of ureteritis or ureteral stenosis, these tests would support the appropriate diagnosis.


Assuntos
Artrite Reumatoide , Doenças Ureterais , Feminino , Humanos , Pessoa de Meia-Idade , Constrição Patológica/complicações , Doenças Ureterais/complicações , Doenças Ureterais/diagnóstico , Hematúria/complicações , Artrite Reumatoide/complicações , Artrite Reumatoide/diagnóstico , Tomografia Computadorizada por Raios X
20.
Altern Ther Health Med ; 29(1): 104-110, 2023 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-36350322

RESUMO

Purpose: Our study aims to investigate the long-term survival and prognostic factors of patients after laparoscopic radical nephrectomy. Methods: Totally, 245 patients with renal cell carcinoma in our hospital from January 2015 to February 2017 were analyzed retrospectively. The 5-year survival status of patients with renal cell carcinoma was under analysis and further based on univariate analysis, and its influencing factors were analyzed by Cox regression. Results: The average 5-year follow-up time of 245 patients with renal cell carcinoma was (4.88 ± 0.52) years. The mortality of 1 year, 3 years and 5 years were 2.45% (5/245), 6.35% (16/245) and 9.80% (24/245), respectively. The survival rates were 97.55% (239/245), 93.06% (228/245) and 90.61% (222/245). Univariate analysis showed that age, tumor diameter, hematuria, TNM stage and postoperative recurrence may be the influencing factors of 5-year survival of patients with renal cell carcinoma (P < .05). However, the following parameters, including gender, course of disease, and other clinical complications were not related to the 5-year survival of patients with renal cell carcinoma (P > .05). the influencing factors of 5-year survival status of patients with renal cell carcinoma were age, tumor diameter, hematuria, TNM stage, and postoperative recurrence. Conclusion: The study revealed the long-term survival of patients with renal cell carcinoma may be associated with age, tumor diameter, hematuria, TNM stage and postoperative recurrence.


Assuntos
Carcinoma de Células Renais , Neoplasias Renais , Laparoscopia , Humanos , Carcinoma de Células Renais/cirurgia , Carcinoma de Células Renais/patologia , Neoplasias Renais/cirurgia , Neoplasias Renais/patologia , Prognóstico , Estudos Retrospectivos , Hematúria/complicações , Hematúria/cirurgia , Nefrectomia
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