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1.
CEN Case Rep ; 2024 May 20.
Artigo em Inglês | MEDLINE | ID: mdl-38763967

RESUMO

Kidney transplantation is the encouraged kidney replacement therapy due to providing more prolonged survival with a better quality of life. Unfortunately, kidney transplant recipients are susceptible to infections because of long-term utilization of immunosuppression. Despite dermatophyte infections are generally not life-threatening, the clinical significance has been recently enhanced by an increasing number of immunocompromised patients. We have presented a rare dermatophytosis course, Majocchi's granuloma, that spreads to all extremities during the early post-transplant period. A young kidney transplant recipient was exposed to intensive immunosuppression therapy due to acute rejection in the early period of post-transplantation. After four months, numerous nodular skin lesions were raised on various body parts. An invasive fungal infection was identified in the skin biopsy. Also, Trichophyton rubrum was isolated in the tissue cultures. Consequently, the patient was diagnosed with Majocchi's granuloma. An effectual treatment was attained with an oral terbinafine tablet. Majocchi's granuloma is a distinct form of dermatophytosis characterized by the spreading of infection into the dermis. In this unexpected case, we alerted physicians to opportunistic infections in the kidney transplant recipient.

2.
J Pediatr Orthop ; 44(1): 15-21, 2024 Jan 01.
Artigo em Inglês | MEDLINE | ID: mdl-37909230

RESUMO

BACKGROUND: Although Dega acetabuloplasty is widely used for the treatment of developmental dysplasia of the hip, there is a paucity of data on long-term outcomes. The purpose of the study was to evaluate the rate of residual acetabular dysplasia after Dega acetabuloplasty. METHODS: Patients of a previously reported consecutive series of 35 patients (43 hips) operated by a single surgeon were recontacted for long-term follow-up. Of these, 25 patients (32 hips) consented, with a follow-up rate of 71% (74% of hips). The mean age at the time of surgery was 35 (18 to 65) months. The presence of residual dysplasia was noted according to the lateral center-edge angle of Wiberg, femoral head extrusion index, and Tönnis angle. The latest radiographic outcome was evaluated according to the Severin classification and patients were clinically evaluated according to the modified McKay criteria. RESULTS: The mean follow-up duration of 16.5 (12 to 20) years yielded an average age of 19.2 (14 to 23) years at the time of analysis. According to lateral center-edge angle, femoral head extrusion index, and Tönnis angle, 5 (15.6%) hips were dysplastic and 2 (6.3%) hips were reoperated for resubluxation. Thus, a total of 7 hips (21.9%) were considered to have residual dysplasia. With the exception of 2 hips that underwent further osteotomies, no other hips were re-subluxated or redislocated. Overcoverage was noted in 6 (18.7%) hips. There were 26 Severin group I and II (81.3%), 4 Severin group III (12.5%), and 2 Severin group IV (6.2%) hips. According to modified McKay criteria, 20 (62.5%) hips were excellent, 7 (21.9%) hips were good, and 5 (15.6%) were fair. Severin classification and modified McKay criteria were correlated with dysplasia ( P < 0.05). CONCLUSIONS: Seventy-eight percent of the hips treated by Dega acetabuloplasty for developmental dysplasia of the hip did not have acetabular dysplasia at a mean follow-up of 16 years. Even in well-treated asymptomatic hips, patients should be followed regularly, especially for residual dysplasia. LEVEL OF EVIDENCE: Level IV.


Assuntos
Acetabuloplastia , Displasia do Desenvolvimento do Quadril , Luxação Congênita de Quadril , Luxação do Quadril , Humanos , Adulto Jovem , Adulto , Pré-Escolar , Seguimentos , Displasia do Desenvolvimento do Quadril/diagnóstico por imagem , Displasia do Desenvolvimento do Quadril/cirurgia , Estudos Retrospectivos , Radiografia , Luxação Congênita de Quadril/diagnóstico por imagem , Luxação Congênita de Quadril/cirurgia , Luxação do Quadril/diagnóstico por imagem , Resultado do Tratamento , Acetábulo/diagnóstico por imagem , Acetábulo/cirurgia
3.
CEN Case Rep ; 2023 Oct 14.
Artigo em Inglês | MEDLINE | ID: mdl-37837535

RESUMO

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease and there is a distinct differentiation of clinical manifestations. Lupus nephritis (LN) is clinically apparent in approximately half of patients. A kidney biopsy is essential to define the kidney injury, exclude other injurious causes, and determine the histopathologic subtypes. Autoantibodies are crucial to the pathogenesis and the deposition of immune complexes in glomeruli is a hallmark of LN. The histopathology of LN is quite varied. Despite pauci-immune LN being an unexpected condition in SLE, it has been observed rarely with the presence of antineutrophil cytoplasmic autoantibodies (ANCA). We present a young male who was admitted to the emergency with syncope. The brain imaging revealed small infarct areas and signs of cerebral vasculitis. Also, he had elevated inflammatory markers, moderate proteinuria, and preserved kidney function. Anti-nuclear antibodies and anti-dsDNA were positive. Pauci-immune crescentic glomerulonephritis (PICGN) was observed in a kidney biopsy, however, ANCA was negative. SLE diagnosis was established by neurological manifestation, specific antibodies, proteinuria, and kidney biopsy findings. We administered a combination induction regimen, including pulse steroid and parenteral cyclophosphamide. The proteinuria was resolved in the follow-up. Our case highlights that SLE-associated ANCA-negative PICGN can be the initial presentation in the absence of typical manifestations. LN exhibits various pathological mechanisms in the kidney. As a consequence, SLE should be considered in the differential diagnosis of all forms of kidney injury.

5.
Children (Basel) ; 10(5)2023 May 13.
Artigo em Inglês | MEDLINE | ID: mdl-37238424

RESUMO

Since the natural course of obstetric brachial plexus palsy is variable, several problems are encountered. One important question, in considering patients with OBPP under observation in outpatient clinical settings, is whether children will have length discrepancies in their arms. The aim of this study was to determine differences in the length of the affected extremity, in comparison to the opposite upper extremity. As such, 45 patients, aged 6 months to 18 years, with unilateral brachial plexus palsy developed due to obstetric reasons, were included in the study. Affected and healthy side humerus, ulna, radius, 2nd metacarpal and 5th metacarpal lengths were evaluated according to gender, age, side, Narakas classification, primary and secondary surgery. Statistically significant differences were found in the change rates of affected/healthy humerus, radius, 2nd metacarpal and 5th metacarpal lengths according to age (93%, 95%, 92%, 90% and 90%, respectively). Affected/healthy change rates of ulna, radius, 2nd metacarpal and 5th metacarpal lengths were found to differ statistically (p < 0.05) according to the Narakas classification variable (94%, 92%, 95%, 94% and 94%, respectively). There were no statistically significant differences in the ratios of affected/healthy change in the lengths of the humerus, ulna, radius and 5th metacarpal compared to the primary surgery (p > 0.05). The ratios of affected/healthy change in ulna, radius and 5th metacarpal lengths were found to differ statistically (p < 0.05) according to secondary surgeries (93%, 91%, 91% and 92%, respectively). Joint and bone deformities and bone shortening were observed after changes that occurred in the postnatal and growing periods due to obstetric brachial plexus palsy. Every increase in function to be gained in the upper extremity musculature was also potentially able to reduce problems, such as shortness.

6.
Clin Exp Med ; 23(7): 3631-3640, 2023 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-36869968

RESUMO

Autosomal dominant polycystic kidney disease (ADPKD) has cancer-like pathophysiology. In this study, we aimed to investigate the phenotype of peripheral blood (PB) T cell subsets and immune checkpoint inhibitor expression of ADPKD patients across different chronic kidney disease (CKD) stages. Seventy-two patients with ADPKD and twenty-three healthy controls were included in the study. The patients were grouped into five different CKD stages, according to glomerular filtration rate (GFR). PB mononuclear cells were isolated and T cell subsets and cytokine production were examined by flow cytometry. CRP levels, height-adjusted total kidney volume (htTKV), rate of hypertension (HT) differed significantly across different GFR stages in ADPKD. T cell phenotyping revealed significantly elevated CD3+ T cells, CD4+, CD8+, double-negative, and double-positive subsets and significantly elevated IFN-γ and TNF-α producing subsets of CD4+, CD8+ cells. The expression of checkpoint inhibitors CTLA-4, PD-1, and TIGIT by T cell subsets was also increased to various extent. Additionally, Treg cell numbers and suppressive markers CTLA-4, PD-1, and TIGIT were significantly elevated in ADPKD patients' PB. Treg CTLA4 expression and CD4CD8DP T cell frequency in patients with HT were significantly higher. Lastly, HT and increased htTKV and higher frequency of PD1+ CD8SP were found to be risk factors for rapid disease progression. Our data provide the first detailed analyses of checkpoint inhibitor expression by PB T cell subsets during stages of ADPKD, and that a higher frequency of PD1+ CD8SP cells is associated with rapid disease progression.


Assuntos
Hipertensão , Rim Policístico Autossômico Dominante , Insuficiência Renal Crônica , Humanos , Rim Policístico Autossômico Dominante/complicações , Rim Policístico Autossômico Dominante/metabolismo , Antígeno CTLA-4 , Linfócitos T Reguladores/metabolismo , Receptor de Morte Celular Programada 1 , Rim , Insuficiência Renal Crônica/complicações , Progressão da Doença , Contagem de Células , Taxa de Filtração Glomerular
7.
Int Urol Nephrol ; 55(4): 975-982, 2023 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-36178610

RESUMO

PURPOSE: In our study, diagnostic and demographic characteristics of patients diagnosed with minimal change disease (MCD) by biopsy, clinical and laboratory findings in our country were investigated. METHODS: Data were obtained from the Turkish Society of Nephrology Glomerular Diseases (TSN-GOLD) Working Group database. Demographic characteristics, indications for biopsy, diagnosis of the glomerular diseases, comorbidities, laboratory and biopsy findings of all patients were recorded. The data presented are cross-sectional and includes application data for the biopsy period. RESULTS: Of 3875 patients, 233 patients with MCD (median age 35.0 years) were included in the study, which constitutes 6.0% of the total glomerulonephritis database. Renal biopsy was performed in 196 (84.1%) patients due to nephrotic syndrome. Median serum creatinine was 0.7 (0.6-1.0) mg/dl, mean eGFR was 104 ± 33 ml/min/1.73 m2 and median proteinuria 6000 mg/day. The number of patients under the age of 40 years was 139 (59.7%) (Group A), and the number of patients aged 40 years and over was 94 (40.3%) (Group B). Compared to Group A, global sclerotic glomeruli (24 vs. 43, p < 0.001) interstitial inflammation (15 vs. 34, p < 0.001), interstitial fibrosis (20 vs. 31, p = 0.001, vascular changes (10 vs. 25, p < 0.001) and tubular atrophy (18 vs. 30, p < 0.001) were found to be significantly higher in Group B. There was no difference in immunofluorescent staining properties between the two groups. CONCLUSION: Our data are generally compatible with the literature. Chronic histopathological changes were more common in patients aged 40 years and older than younger patients. Studies investigating the effects of these different features on renal survival are needed.


Assuntos
Nefropatias , Nefrologia , Nefrose Lipoide , Humanos , Adulto , Pessoa de Meia-Idade , Nefrose Lipoide/diagnóstico , Nefrose Lipoide/epidemiologia , Turquia/epidemiologia , Estudos Transversais , Nefropatias/patologia , Rim/patologia , Demografia , Biópsia , Estudos Retrospectivos
8.
BMC Nephrol ; 23(1): 326, 2022 10 05.
Artigo em Inglês | MEDLINE | ID: mdl-36199011

RESUMO

BACKGROUND: This study aimed to evaluate the etiologies, comorbidities, and outcomes of acute kidney injury (AKI) in Turkey and determine any potential differences among different geographical parts of the country. METHODS: This prospective observational study was conducted by the Acute Kidney Injury Working Group of the Turkish Society of Nephrology. Demographical and clinical data of patients with AKI at the time of diagnosis and at the 1st week and 1st, 3rd, and 6th months of diagnosis were evaluated to determine patient and renal survival and factors associated with patient prognosis. RESULTS: A total of 776 patients were included (54.7% male, median age: 67 years). Prerenal etiologies, including dehydration, heart failure, and sepsis, were more frequent than other etiologies. 58.9% of the patients had at least one renal etiology, with nephrotoxic agent exposure as the most common etiology. The etiologic factors were mostly similar throughout the country. 33.6% of the patients needed kidney replacement therapy. At the 6th month of diagnosis, 29.5% of the patients had complete recovery; 34.1% had partial recovery; 9.5% developed end-stage kidney disease; and 24.1% died. The mortality rate was higher in the patients from the Eastern Anatolian region; those admitted to the intensive care unit; those with prerenal, renal, and postrenal etiologies together, stage 3 AKI, sepsis, cirrhosis, heart failure, and malignancy; those who need kidney replacement therapy; and those without chronic kidney disease than in the other patients. CONCLUSION: Physicians managing patients with AKI should be alert against dehydration, heart failure, sepsis, and nephrotoxic agent exposure. Understanding the characteristics and outcomes of patients with AKI in their countries would help prevent AKI and improve treatment strategies.


Assuntos
Injúria Renal Aguda , Insuficiência Cardíaca , Sepse , Injúria Renal Aguda/epidemiologia , Injúria Renal Aguda/etiologia , Injúria Renal Aguda/terapia , Idoso , Desidratação/complicações , Feminino , Insuficiência Cardíaca/complicações , Insuficiência Cardíaca/etiologia , Humanos , Unidades de Terapia Intensiva , Masculino , Prognóstico , Estudos Retrospectivos , Fatores de Risco , Sepse/complicações , Sepse/diagnóstico , Sepse/epidemiologia , Turquia/epidemiologia
9.
Ren Fail ; 44(1): 1048-1059, 2022 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-35786180

RESUMO

BACKGROUND: We aimed to evaluate the features of primary membranous nephropathy (MNP) in Turkish people. METHODS: This is a retrospective analysis of patients with biopsy-proven primary MNP. We obtained the data collected between 2009 and 2019 in the primary glomerulonephritis registry of the Turkish Society of Nephrology Glomerular Diseases Study Group (TSN-GOLD). Patients with a secondary cause for MNP were excluded. Clinical, demographic, laboratory, and histopathological findings were analyzed. RESULTS: A total of 995 patients with primary MNP were included in the analyses. Males constituted the majority (58.8%). The mean age was 48.4 ± 13.9 years. The most common presentation was the presence of nephrotic syndrome (81.7%) and sub nephrotic proteinuria (10.3%). Microscopic hematuria was detected in one-third of patients. The median estimated glomerular filtration rate (eGFR) was 100.6 mL/min/1.73 m2 (IQR, 75.4-116.3), and median proteinuria was 6000 mg/d (IQR, 3656-9457). Serum C3 and C4 complement levels were decreased in 3.7 and 1.7% of patients, respectively. Twenty-four (2.4%) patients had glomerular crescents in their kidney biopsy samples. Basal membrane thickening was detected in 93.8% of cases under light microscopy. Mesangial proliferation and interstitial inflammation were evident in 32.8 and 55.9% of the patients, respectively. The most commonly detected depositions were IgG (93%), C3 complement (68.8%), and kappa and lambda immunoglobulin light chains (70%). Although renal functions were normal at presentation, vascular, interstitial, and glomerular findings were more prominent on biopsy in hypertensive patients. No significant effect of BMI on biopsy findings was observed. CONCLUSIONS: Despite some atypical findings, the main features of primary MNP in Turkey were similar to the published literature. This is the largest MNP study to date conducted in Turkish people.


Assuntos
Glomerulonefrite Membranosa , Nefropatias , Nefrologia , Adulto , Glomerulonefrite Membranosa/patologia , Humanos , Nefropatias/patologia , Masculino , Pessoa de Meia-Idade , Proteinúria/complicações , Estudos Retrospectivos , Turquia/epidemiologia
10.
Hemodial Int ; 25(2): 164-172, 2021 04.
Artigo em Inglês | MEDLINE | ID: mdl-33155365

RESUMO

INTRODUCTION: Arteriovenous fistula (AVF) stenosis is one of the most important clinical problems in hemodialysis patients. The histopathological findings of neointimal hyperplasia and impaired angiogenesis have been well established in stenotic AVFs. Soluble vascular endothelial growth factor receptor-1 (sVEGFR-1) has been implicated in pathological angiogenesis. Thus, we aimed to investigate the association between sVEGFR-1 and AVF stenosis in hemodialysis patients. METHODS: This prospective cohort study included 70 patients with end-stage renal disease. Forty-five patients were included in the final analysis, and the median follow-up period was 36 months. Venous stenosis was detected by physical examination and documented by fistulography. Blood samples were analyzed a day before the fistula operation, and serum levels of sVEGFR-1 were measured. FINDINGS: The median sVEGFR-1 level was higher in the stenosis group than in the nonstenosis group (17 pg/mL [89.5%] vs. 5 pg/mL [19.2%], respectively; P < 0.001]. According to body mass index (BMI) categories, obese patients (BMI > 30 kg/m2 ) had the shortest stenosis-free survival (20 months [9.35-30.65]). Multivariate Cox analysis showed that sVEGFR-1, serum creatinine, and parathyroid hormone levels were associated with AVF stenosis risk. Kaplan-Meier survival curves showed that patients with less than the median value of sVEGFR-1 (<6093.07 pg/mL) had longer cumulative stenosis-free survival than patients with sVEGFR-1 levels above the median value (P < 0.001). DISCUSSION: Increased levels of sVEGFR-1 and obesity were found to be associated with AVF stenosis in hemodialysis patients.


Assuntos
Fístula Arteriovenosa , Derivação Arteriovenosa Cirúrgica , Fístula Arteriovenosa/etiologia , Derivação Arteriovenosa Cirúrgica/efeitos adversos , Constrição Patológica , Humanos , Estudos Prospectivos , Diálise Renal/efeitos adversos , Fator A de Crescimento do Endotélio Vascular , Receptor 1 de Fatores de Crescimento do Endotélio Vascular
11.
BMC Nephrol ; 21(1): 525, 2020 12 03.
Artigo em Inglês | MEDLINE | ID: mdl-33272229

RESUMO

BACKGROUND: Tunneled catheters can be used as an alternative vascular access in patients with limited health expectancy,vascular access problems and several comorbidities. We aimed to present a patient with venous stenosis related- reversible acute Budd-Chiari syndrome after catheter malposition. CASE PRESENTATION: After changing of tunneled catheter insertion, 36-year old man was admitted to our hospital with sudden onset of nausea, fever, chills and worsening general condition In computed tomography (CT) imaging, a hypodense thrombus was observed in which the distal end of the catheter is at the level of drainage of the hepatic veins in the inferior vena cava and that blocked hepatic vein drainage around the catheter. The catheter was removed and a new catheter was inserted in the same session. Because patient's general condition was good and without fever, he was discharged with advices on the 9th day of hospitalization. CONCLUSION: Although catheter malposition and thrombosis are not a common complication, clinicians should be alert of these complications.


Assuntos
Síndrome de Budd-Chiari/diagnóstico por imagem , Cateterismo Venoso Central/efeitos adversos , Veias Hepáticas/diagnóstico por imagem , Veias Jugulares , Falência Renal Crônica/terapia , Diálise Renal/métodos , Veia Cava Inferior/diagnóstico por imagem , Trombose Venosa/diagnóstico por imagem , Doença Aguda , Adulto , Amiloidose/complicações , Síndrome de Budd-Chiari/etiologia , Febre Familiar do Mediterrâneo/complicações , Humanos , Falência Renal Crônica/etiologia , Fígado/diagnóstico por imagem , Masculino , Proteína Amiloide A Sérica , Tomografia Computadorizada por Raios X , Trombose Venosa/complicações
12.
Eur J Clin Invest ; 50(12): e13350, 2020 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-32652532

RESUMO

BACKGROUND: Neointimal hyperplasia is the main cause of arteriovenous fistula (AVF) failure. Hypoxia-inducible factors (HIFs) factors are associated with neointimal hyperplasia. Thus, we investigated the association between HIF-2 alpha (HIF-2α) and AVF maturation in end-stage kidney disease (ESKD) patients. METHODS: This prospective cohort study was conducted in 21 voluntary healthy subjects and 50 patients with ESKD who were eligible for AVF creation. Inclusion criteria were being ESKD patients without a history of AVF surgery and dialysis. Eight patients excluded from the study due to having unavailable veins six patients were excluded due to acute thrombosis after surgery. One patient lost to follow-up. A total of 35 patients were included in final analysis. The blood samples were collected a day before the AVF surgery for biochemical parameters and HIF-2α measurement. HIF-2α levels were measured by the ELISA method. RESULTS: Compared with healthy subjects, ESKD patients had a significantly higher level of HIF-2α. [1.3 (1.0-1.9) vs 2.2 (1.6-3.0)] (P = .002). Patients were divided into two groups after the evaluation of AVF maturation, as the mature group (n = 19) and the failure group (n = 16). Serum HIF-2α level was 1.7 (1.1-1.8) in the mature group; however, it was 3.1 (2.8-3.3 in failure group (P < .001). Multiple logistic regression analyses showed that HIF-2α independently predicted AVF maturation. The ROC curve analysis showed that HIF-2α > 2.65 predicted AVF maturation failure with the 87% sensitivity and 94% specificity [AUC:0.947, 95% CI (0.815-0.994), P < .001]. CONCLUSIONS: HIF-2-α levels were higher in ESKD patients than healthy subjects. HIF-2-α could be a marker of AVF maturation failure.


Assuntos
Derivação Arteriovenosa Cirúrgica , Fatores de Transcrição Hélice-Alça-Hélice Básicos/sangue , Falência Renal Crônica/terapia , Neointima/sangue , Complicações Pós-Operatórias/sangue , Adulto , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Neointima/epidemiologia , Complicações Pós-Operatórias/epidemiologia , Diálise Renal
13.
J Oral Maxillofac Surg ; 78(7): 1171-1182, 2020 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-32275899

RESUMO

PURPOSE: The main disadvantage of distraction osteogenesis is the prolonged treatment protocol. Recently, oxytocin (OT) has been found to have anabolic effects on bone metabolism. In this experimental study, the effects of OT on the mandibular distraction gap in rabbits at 2 different distraction rates were evaluated. MATERIALS AND METHODS: This experimental study was conducted on 28 male New Zealand white rabbits. The animals were divided into 3 experimental groups and 1 control group. Group A (control group, n = 7) consisted of animals with distraction at a rate of 1 mm/day, and group B (n = 7) consisted of animals with a distraction rate of 2 mm/day; groups A and B received postoperative saline solution injection. Group C (n = 7) consisted of animals with distraction at a rate of 1 mm/day, and group D (n = 7) consisted of animals with a distraction rate of 2 mm/day; postoperative OT injection was performed in groups C and D. RESULTS: Both histomorphologic and micro-computed tomography evaluations showed increased bone healing in the OT-treated groups. CONCLUSIONS: On the basis of the evaluation of both the histomorphometric and micro-computed tomographic data, systemic OT administration was found to increase new bone formation and bone healing with distraction osteogenesis.


Assuntos
Osteogênese por Distração , Ocitocina , Animais , Densidade Óssea , Regeneração Óssea , Masculino , Mandíbula , Osteogênese , Coelhos , Microtomografia por Raio-X
14.
World J Clin Cases ; 7(16): 2309-2315, 2019 Aug 26.
Artigo em Inglês | MEDLINE | ID: mdl-31531324

RESUMO

BACKGROUND: Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated condition that consisted of disorders that share particular clinical, serologic and pathologic properties. The common presentation of disease includes tumor-like swelling of involved organs and the histopathological findings are a lymphoplasmacytic infiltrate enriched with IgG4-positive plasma cells, and a variable degree of fibrosis that has a characteristic "storiform" pattern in biopsy specimens of tumor-like masses. Major presentations of this disease, which often affects more than one organ, include autoimmune pancreatitis, salivary gland disease (sialadenitis), orbital disease and retroperitoneal fibrosis. The steroid treatment is essential for the treatment of the disease however, other immunosuppressive drugs including cyclophosphamide or rituximab could be an option in resistant cases. CASE SUMMARY: Herein, we reported a 34-year-old woman whom previously had diagnosed with asthma, rheumatoid arthritis and Sjögren's syndrome (SS) referred our nephrology department due to acute kidney failure development at the last rheumatology visit. After kidney biopsy she has been diagnosed with IgG4-RD and tubuluointerstitial nephritis. She had been accepted resistant to steroid, mycophenolate mofetil, methotrexate and azathioprine therapies due to receiving in last two years. She refused to receive cyclophosphamide due to potential gonadotoxicity of the drug. Thus, rituximab therapy was considered. She received 1000 mg infusion, 15 d apart and 6 mo later it has been administered same protocol. After one year from the last rituximab dose serum creatinine decreased from 4.4 mg/dL to 1.6 mg/dL, erythrocyte sedimentation rate decreased from 109 mm/h to 13 mm/h [reference range (RR) 0-20], and C-reactive protein decreased from 55.6 mg/L to 5 mg/L (RR 0-6). All pathologic lymph nodes and masses were also disappeared. CONCLUSION: Patients with IgG4-RD usually misdiagnosed with rheumatologic diseases including systemic lupus erythematous or SS and also they were screened for the presence of malignancy. Rituximab could be an important treatment option in cases with steroid resistant tubulointerstitial nephritis in IgG4-RD.

15.
Cardiorenal Med ; 9(6): 370-381, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31319406

RESUMO

BACKGROUND AND OBJECTIVES: Cyst pressure induces renin-angiotensin-aldosterone system activation and kidney hypoxia in autosomal dominant polycystic kidney disease (ADPKD). Lipopolysaccharide-induced Toll-like receptor activation causes metabolic disturbances that are triggered by increased succinate levels and hypoxia inducible factors, which results in inflammation via IL-1ß activation. Since we aimed to investigate the role of both inflammation and hypoxia in the clinical course of ADPKD, via succinate levels from sera samples, HIF-1α gene expression from whole blood and urine samples and IL-1ßgene expression from whole blood were measured. METHODS: One hundred ADPKD patients and 100 matched healthy controls were enrolled to this cross-sectional study. Twenty-four-hour ambulatory blood pressure monitoring was conducted in all participants. Blood, serum, and urine samples were taken after 12-h fasting for the measurement of biochemical parameters and succinate levels. Whole blood and urine samples were used for HIF-1α and IL-1ß geneexpression by using quantitative real-time PCR. RESULTS: There were significant differences in whole blood HIF-1α, IL-1ß geneexpression, and serumsuccinate levels between the ADPKD patients and the control subjects. Whole blood HIF-1αgene expression, IL-1ß geneexpression, and serumsuccinate levels were also significantly different in ADPKD patients with hypertension in comparison with normotensive ones (p < 0.05). Serum succinate levels and blood IL-1ß geneexpression were increased in ADPKD patients with high levels of HIF-1α geneexpression (p = 0.018 and p = 0.029, respectively). CONCLUSIONS: Increased age,low eGFR, and HIF-1α and IL-1ß geneexpressions were also independently associated with hypertension in ADPKD patients. Inflammation and hypoxia are both relevant factors that might be associated with hypertension in ADPKD.


Assuntos
Hipertensão/complicações , Subunidade alfa do Fator 1 Induzível por Hipóxia/metabolismo , Interleucina-1beta/metabolismo , Rim Policístico Autossômico Dominante/genética , Adulto , Hipóxia Celular/genética , Estudos Transversais , Feminino , Expressão Gênica/fisiologia , Humanos , Hipertensão/sangue , Subunidade alfa do Fator 1 Induzível por Hipóxia/genética , Interleucina-1beta/genética , Masculino , Pessoa de Meia-Idade , Nefrite/genética , Rim Policístico Autossômico Dominante/sangue , Rim Policístico Autossômico Dominante/complicações , RNA Mensageiro/metabolismo , Sistema Renina-Angiotensina/genética
16.
CEN Case Rep ; 8(4): 227-232, 2019 11.
Artigo em Inglês | MEDLINE | ID: mdl-31089951

RESUMO

Secondary and tertiary hyperparathyroidism is an important problem of chronic kidney disease. Brown tumor is a benign, unusual, reactive lesion as a result of disturbed bone remodeling, from long-standing increase in parathyroid hormone level. Brown tumors may cause morbidity due to pressure symptoms on neural structures and spontaneous bone fractures. Herein, we presented a peritoneal dialysis patient with tertiary hyperparathyroidism under calcand calcitriol treatment for 4 years due to refusing of the parathyroidectomy operation. She admitted to hospital for sudden onset back pain with difficulty in gait and walking, and imaging studies showed an expansile mass lesion in the thoracic spine. She was operated for mass and diagnosed with brown tumor. After operation, she lost the ability of walking than become paraplegic and she underwent rehabilitation program. Preventive measures including calcitriol and cinacalcet may cause a modest decrease in parathyroid hormone levels but it should be remembered for the development of bone complications such as brown tumor formation in patients with moderate elevated PTH levels, especially those with tertiary hyperparathyroidism. Parathyroidectomy should be performed without delay in these cases.


Assuntos
Hiperparatireoidismo/complicações , Osteíte Fibrosa Cística/complicações , Osteoclastos/patologia , Paraplegia/etiologia , Diálise Peritoneal/efeitos adversos , Adulto , Calcitriol/uso terapêutico , Agonistas dos Canais de Cálcio/uso terapêutico , Feminino , Humanos , Hiperparatireoidismo/tratamento farmacológico , Osteíte Fibrosa Cística/diagnóstico por imagem , Osteíte Fibrosa Cística/patologia , Osteíte Fibrosa Cística/cirurgia , Paraplegia/reabilitação , Paratireoidectomia/normas , Insuficiência Renal Crônica/terapia
17.
Int Urol Nephrol ; 50(11): 2067-2072, 2018 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-30251011

RESUMO

PURPOSE: Renal infarction is a clinical condition which is caused by renal artery occlusion and leads to permanent renal parenchymal damage. In the literature, there are generally case reports on this subject, and few studies that include a large group of patients. Therefore, we aimed to present the data of a large group of patients who were diagnosed with acute renal infarction in our country in this retrospective study. METHODS: The data of patients who were diagnosed with acute renal infarction according to clinical and radiological findings in Turkey in the last 3 years were examined. For this purpose, we contacted with more than 40 centers in 7 regions and obtained support from clinically responsible persons. Demographic data of patients, laboratory data at the time of diagnosis, tests performed for etiologic evaluation, given medications, and patients' clinical status during follow-up were obtained from databases and statistical analysis was performed. RESULTS: One-hundred and twenty-one patients were included in the study. The mean age was 53 ± 1.4 (19-91) years. Seventy-one (58.7%) patients were male, 18 (14.9%) had diabetes, 53 (43.8%) had hypertension, 36 (30%) had atrial fibrillation (AF), and 6 had a history of lupus + antiphospholipid syndrome (APS). Forty-five patients had right renal infarction, 50 patients had left renal infarction, and 26 (21.5%) patients had bilateral renal infarction. The examinations for the ethiologies revealed that, 36 patients had thromboemboli due to atrial fibrillation, 10 patients had genetic anomalies leading to thrombosis, 9 patients had trauma, 6 patients had lupus + APS, 2 patients had hematologic diseases, and 1 patient had a substance abuse problem. Fifty-seven (57%) patients had unknown. The mean follow-up period was 14 ± 2 months. The mean creatinine and glomerular filtration rate (GFR) values at 3 months were found to be 1.65 ± 0.16 mg/dl and 62 ± 3 ml/min, respectively. The final mean creatinine and GFR values were found to be 1.69 ± 0.16 mg/dl and 62 ± 3 ml/min, respectively. CONCLUSIONS: Our study is the second largest series published on renal infarction in the literature. More detailed studies are needed to determine the etiological causes of acute renal infarction occurring in patients.


Assuntos
Infarto/etiologia , Obstrução da Artéria Renal/etiologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Taxa de Filtração Glomerular , Humanos , Infarto/diagnóstico , Infarto/terapia , Masculino , Pessoa de Meia-Idade , Obstrução da Artéria Renal/diagnóstico , Obstrução da Artéria Renal/terapia , Estudos Retrospectivos , Fatores de Risco , Resultado do Tratamento , Turquia , Adulto Jovem
18.
Semin Dial ; 31(3): 268-277, 2018 05.
Artigo em Inglês | MEDLINE | ID: mdl-29664556

RESUMO

Autosomal dominant polycystic kidney disease (ADPKD) is a common monogenic disease characterized by massive enlargement of fluid-filled cysts in the kidney. Due to its genetic pattern, the disease differs from other CKD. ADPKD is a multi-system, progressive disorder which is frequently complicated with hypertension, cardiovascular events and cerebrovascular disease. Thus, there are many clinical problems specific to ADPKD. In this article, we reviewed these clinical problems and their management in ADPKD with hemodialysis patients.


Assuntos
Falência Renal Crônica/etiologia , Falência Renal Crônica/terapia , Transplante de Rim/métodos , Rim Policístico Autossômico Dominante/complicações , Diálise Renal/efeitos adversos , Doenças Cardiovasculares/etiologia , Doenças Cardiovasculares/fisiopatologia , Transtornos Cerebrovasculares/etiologia , Transtornos Cerebrovasculares/fisiopatologia , Progressão da Doença , Feminino , Humanos , Falência Renal Crônica/fisiopatologia , Masculino , Nefrectomia/métodos , Rim Policístico Autossômico Dominante/diagnóstico , Rim Policístico Autossômico Dominante/mortalidade , Diálise Renal/métodos , Medição de Risco , Análise de Sobrevida , Resultado do Tratamento
20.
Perit Dial Int ; 38(1): 57-62, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-29097487

RESUMO

BACKGROUND: The aim of the study is to evaluate the long-term protective effect of N-acetylcysteine (NAC), an antioxidant agent, against aminoglycoside (AG)-induced ototoxicity. METHODS: A total of 40 patients receiving continuous ambulatory peritoneal dialysis (CAPD) and having their first peritonitis attacks and planned to be treated with AGs were enrolled in the study. They were randomized into 2 groups: 1 group received additional NAC and the other did not. All patients underwent hearing tests with pure tone audiometry (PTA) after the diagnosis, at 1 month and 12 months and at the same time the tumor necrosis factor (TNF)-α and interleukin (IL)-6 levels were measured. RESULTS: Patients taking NAC had better hearing test results in both ears at 1 month except 2,000 Hz for the left ear, which wasn't significantly different between the 2 groups. Although patients taking NAC had generally better PTA results at 12 months, differences between the 2 groups were not statistically significant. Baseline IL-6 level was significantly higher in the NAC group than the control group. Both TNF-α and IL-6 levels at 1 month were significantly lower in the NAC group than in the control group. On the other hand, there was no significant difference between the 2 groups in terms of TNF-α and IL-6 levels at 12 months. CONCLUSIONS: The results of the current study showed that NAC, a potent anti-inflamatory drug, may be otoprotective, but that the effect is not long-lasting.


Assuntos
Acetilcisteína/uso terapêutico , Amicacina/efeitos adversos , Otopatias/prevenção & controle , Sequestradores de Radicais Livres/uso terapêutico , Peritonite/tratamento farmacológico , Adulto , Audiometria de Tons Puros/métodos , Otopatias/induzido quimicamente , Humanos , Interleucina-6/sangue , Falência Renal Crônica/terapia , Pessoa de Meia-Idade , Diálise Peritoneal Ambulatorial Contínua/efeitos adversos , Peritonite/etiologia , Estudos Prospectivos , Fator de Necrose Tumoral alfa/sangue
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