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1.
J Am Soc Nephrol ; 2024 Oct 09.
Artículo en Inglés | MEDLINE | ID: mdl-39382977

RESUMEN

BACKGROUND: Deep learning has great potential in digital kidney pathology. However, its effectiveness depends heavily on the availability of extensively labeled datasets, which are often limited due to the specialized knowledge and time required for their creation. This limitation hinders the widespread application of deep learning for the analysis of kidney biopsy images. METHODS: We applied self-distillation with no labels (DINO), a self-supervised learning method, to a dataset of 10,423 glomerular images obtained from 384 PAS-stained kidney biopsy slides. Glomerular features extracted from the DINO-pretrained backbone were visualized using principal component analysis (PCA). We then performed classification tasks by adding either k-nearest neighbor (kNN) classifiers or linear head layers to the DINO-pretrained or ImageNet-pretrained backbones. These models were trained on our labeled classification dataset. Performance was evaluated using metrics such as the area under the receiver operating characteristic curve (ROC-AUC). The classification tasks encompassed four disease categories (minimal change disease, mesangial proliferative glomerulonephritis, membranous nephropathy, and diabetic nephropathy) as well as clinical parameters such as hypertension, proteinuria, and hematuria. RESULTS: PCA visualization revealed distinct principal components corresponding to different glomerular structures, demonstrating the capability of the DINO-pretrained backbone to capture morphological features. In disease classification, the DINO-pretrained transferred model (ROC-AUC = 0.93) outperformed the ImageNet-pretrained fine-tuned model (ROC-AUC = 0.89). When the labeled data were limited, the ImageNet-pretrained fine-tuned model's ROC-AUC dropped to 0.76 (95% confidence interval [CI], 0.72-0.80), whereas the DINO-pretrained transferred model maintained superior performance (ROC-AUC 0.88, 95% CI 0.86-0.90). The DINO-pretrained transferred model also exhibited higher AUCs for the classification of several clinical parameters. External validation using two independent datasets confirmed DINO pre-training's superiority, particularly when labeled data were limited. CONCLUSIONS: The application of DINO to unlabeled PAS-stained glomerular images facilitated the extraction of histological features that can be effectively utilized for disease classification.

2.
Rinsho Ketsueki ; 64(3): 209-213, 2023.
Artículo en Japonés | MEDLINE | ID: mdl-37019675

RESUMEN

Chronic active Epstein-Barr virus (CAEBV) infection is characterized by persistent EBV infection and can lead to fatal conditions such as hemophagocytic syndrome and malignant lymphoma through the clonal expansion of EBV-infected T or natural killer (NK) cells. Hydroa vacciniforme lymphoproliferative disorder (HV) and hypersensitivity to mosquito bites (HMB) have been identified as skin diseases in EBV-associated T- or NK-cell lymphoproliferative diseases. We present the case of a 33-year-old man. The patient had frequent episodes of a facial rash for three years before he visited our hospital, he visited several dermatologists but did not receive a diagnosis of HV. He was referred to the hematology department of our hospital for assessment of atypical lymphocytes in peripheral blood. Based on routine blood and bone marrow test we were unable to diagnose HV. However, when the patient's liver function deteriorated six months later, we considered the possibility of HV after reevaluating the skin rash. After performing EBV-related tests, we were able to definitively diagnose CAEBV with HV. It is crucial to be able to connect clinical observations to EBV-related tests when diagnosing CAEBV. Hematologists must be knowledgeable of the EBV-associated skin conditions of HV and HMB.


Asunto(s)
Infecciones por Virus de Epstein-Barr , Exantema , Hidroa Vacciniforme , Trastornos Linfoproliferativos , Masculino , Humanos , Adulto , Hidroa Vacciniforme/patología , Herpesvirus Humano 4 , Diagnóstico Tardío
3.
Nephrol Dial Transplant ; 37(9): 1679-1690, 2022 08 22.
Artículo en Inglés | MEDLINE | ID: mdl-34499164

RESUMEN

BACKGROUND: The associations of focal segmental glomerulosclerosis (FSGS) histological variants with renal outcomes have rarely been investigated comprehensively by clinically relevant subgroups in this modern age. METHODS: Data on 304 (173 nephrotic and 131 non-nephrotic) patients with biopsy-confirmed FSGS from 2010 to 2013 were analyzed using the Japanese nationwide renal biopsy registry. The primary outcome was a composite of a 30% decline in estimated glomerular filtration rate or progression to end-stage kidney disease 5 years from the biopsy. We compared outcomes of FSGS variants according to the Columbia classification using survival analyses. Subgroup analyses were performed based on nephrotic syndrome (NS), immunosuppression and proteinuria remission (PR; proteinuria <0.3 g/day) during follow-up. Additionally, associations of NS, immunosuppression and PR with outcomes were examined for each variant. RESULTS: The distribution of variants was 48% (n = 145) FSGS not otherwise specified, 19% (n = 57) tip, 15% (n = 47) perihilar, 13% (n = 40) cellular and 5% (n = 15) collapsing. The outcome event occurred in 87 patients (29%). No significant differences in the outcome were found among the variants. Subgroup analyses yielded similar results. However, there was a trend toward improved outcome in patients with PR irrespective of variants [hazard ratio adjusted for histological variant and potential confounders (adjusted HR) 0.19 (95% confidence interval 0.10-0.34)]. NS was marginally associated with better outcome compared with non-NS [adjusted HR 0.50 (95% confidence interval 0.25-1.01)]. CONCLUSIONS: FSGS variants alone might not have significant impacts on the renal outcome after 5 years, while PR could be predictive of improved renal prognosis for any variant. Specific strategies and interventions to achieve PR for each variant should be implemented for better renal outcomes.


Asunto(s)
Glomeruloesclerosis Focal y Segmentaria , Síndrome Nefrótico , Glomeruloesclerosis Focal y Segmentaria/patología , Humanos , Terapia de Inmunosupresión , Síndrome Nefrótico/complicaciones , Síndrome Nefrótico/etiología , Proteinuria/patología , Estudios Retrospectivos
4.
Gan To Kagaku Ryoho ; 49(13): 1953-1955, 2022 Dec.
Artículo en Japonés | MEDLINE | ID: mdl-36733055

RESUMEN

A 54-year-old woman visited to a doctor nearby medical clinic complaining of loss of appetite. She was diagnosed with right hydronephrosis on abdominal ultrasonography, and referred to our hospital for further examination. Contrast abdominal computed tomography(CT)revealed that a 6.2 cm tumor with a contrast-enhancing effect inside in the retroperitoneum near the lower pole of the right kidney. She was diagnosed with hydronephrosis due to infiltration of the right kidney of a retroperitoneal tumor. The tumor was suspected of invading the duodenum and inferior vena cava, but no obvious lymph node or distant metastasis was observed. Abdominal MRI revealed a tumor showed hyperintensity on T2-weighted and diffusion-weighted images. We performed pancreaticoduodenectomy with inferior vena cava resection and right nephrectomy. The pathological diagnosis was leiomyosarcoma originating from retroperitoneum and pT2, pN0, pM0, pStage ⅢA. The postoperative course was good, and she was discharged 10 days after the operation. Thoracoabdominal CT showed a tumor 4 cm at the hepatic hilum three months after surgery, and EOB-MRI showed many tumors other than the same site, so multiple liver metastases were diagnosed as recurrence. Doxorubicin has been started and is still being treated.


Asunto(s)
Hidronefrosis , Leiomiosarcoma , Neoplasias Hepáticas , Neoplasias Retroperitoneales , Femenino , Humanos , Persona de Mediana Edad , Neoplasias Retroperitoneales/diagnóstico por imagen , Neoplasias Retroperitoneales/cirugía , Neoplasias Retroperitoneales/patología , Pancreaticoduodenectomía , Leiomiosarcoma/diagnóstico por imagen , Leiomiosarcoma/cirugía , Leiomiosarcoma/irrigación sanguínea , Neoplasias Hepáticas/cirugía , Vena Cava Inferior/cirugía , Vena Cava Inferior/patología
5.
Gan To Kagaku Ryoho ; 47(13): 2239-2241, 2020 Dec.
Artículo en Japonés | MEDLINE | ID: mdl-33468920

RESUMEN

A 60's woman was admitted to our hospital because of palpitations that occurred with exertion. Coronary angiography computed tomography(CT)of suspected angina detected a tumor in the pancreatic head region. Abdominal CT showed a poorly enhanced 40×32 mm solid tumor in the hepatoduodenal ligament that contained a fatty component and calcification. During surgery, the tumor was located in the hepatoduodenal ligament, adhered to the pancreatic head, common hepatic artery, gastroduodenal artery, portal vein and common bile duct. However, the tumor was resected by preserving them. The tumor contained stratified squamous epithelium, a sebaceous gland, nerve, a pancreatic gland, and an adrenal gland. The histological diagnosis was a mature cystic teratoma. The patient showed no recurrence in 2 years and 10 months post-surgery. Mature teratomas in the hepatoduodenal ligament are extremely rare. Some reports showed that combined resection was performed when the tumor was in contact with the common bile duct, portal vein, and arteries. However, in our case, the tumor was removed relatively safely without combined resection.


Asunto(s)
Recurrencia Local de Neoplasia , Teratoma , Femenino , Humanos , Ligamentos/cirugía , Hígado , Epiplón , Teratoma/cirugía
6.
Biochem Biophys Res Commun ; 508(4): 1106-1112, 2019 01 22.
Artículo en Inglés | MEDLINE | ID: mdl-30553446

RESUMEN

IgA nephropathy (IgAN) is the most common form of primary glomerulonephritis, and disease recurrence often occurs after transplantation. On the other hands, Asymptomatic IgA deposition (IgAD) is occasionally observed in donated kidney. It is recognized that IgAD does not progress to IgAN, but the mechanism has not demonstrated yet. In IgAN, aberrant IgA1 O-glycan structure in the hinge region (HR) of serum IgA is suggested as one of the most convincing key mediators. However, little is known about IgA1 O-glycan structure in IgAD patients. Herein, we investigated the prevalence of IgAD in living renal transplant donors in our cohort. IgAD was observed in 21(13.0%) among 161 renal transplant donors and have statistically significant blood relationship with IgAN recipients (28.6% in relatives vs. 9.8% in non-relatives, respectively; p = 0.0073). Next, we evaluated the IgA1 O-glycan structure of serum IgA from IgAN recipients (n = 26), IgAD donors (n = 17), and non-IgAD helthy donors (n = 27) using matrix-assisted laser desorption ionization time-of-flight mass spectrometry (MALDI-TOF MS). The numbers of GalNAc and Gal and the Gal/GalNAc ratio in the HR of the IgAN recipients had significantly lower comparing to the IgAD and non-IgAD healthy donors. The decreased Gal/GalNAc ratio in IgAN recipients means the increased ratio of galactose-deficient IgA1. To the best of our knowledge, this is the first report to compare the O-glycan structures in IgAN recipients and IgAD donors using MALDI-TOF MS. We concluded that IgAD was more common in IgAN related donors. Overall, decreased GalNAc and Gal contents in HR could play a material pathogenic role in IgAN.


Asunto(s)
Glomerulonefritis por IGA/inmunología , Inmunoglobulina A/inmunología , Trasplante de Riñón , Adulto , Femenino , Galactosamina/metabolismo , Glomerulonefritis por IGA/sangre , Glomerulonefritis por IGA/epidemiología , Glicosilación , Humanos , Inmunoglobulina A/sangre , Inmunoglobulina A/química , Masculino , Polisacáridos/química , Polisacáridos/metabolismo , Prevalencia , Donantes de Tejidos
7.
Gan To Kagaku Ryoho ; 46(2): 380-382, 2019 Feb.
Artículo en Japonés | MEDLINE | ID: mdl-30914567

RESUMEN

We report a case of primary lymphoma of the breast complicated by heart failure and alcoholic-decompensated hepatic cirrhosis. The patient was a woman in her 60s who noticed a right breast tumor growing 3 months previously. The size of the tumor was approximately 5 cm, and the tumor had infiltrated the skin. There was no metastasis to the axillary lymph node or other organs by CT. We performed right breast mastectomy. Pathology indicated diffuse large B cell lymphoma(DLBCL). We considered chemotherapy, but her general condition was not good because of hepatic cirrhosis, so we administered palliative care. Although chemotherapy is the first choice of treatment for DLBCL, it is necessary to individually consider each patient's circumstances.


Asunto(s)
Neoplasias de la Mama , Cirrosis Hepática , Linfoma de Células B Grandes Difuso , Axila , Neoplasias de la Mama/complicaciones , Neoplasias de la Mama/diagnóstico , Neoplasias de la Mama/cirugía , Femenino , Humanos , Cirrosis Hepática/complicaciones , Cirrosis Hepática/diagnóstico , Linfoma de Células B Grandes Difuso/complicaciones , Linfoma de Células B Grandes Difuso/diagnóstico , Linfoma de Células B Grandes Difuso/cirugía , Mastectomía , Persona de Mediana Edad
8.
Hinyokika Kiyo ; 64(4): 175-179, 2018 Apr.
Artículo en Japonés | MEDLINE | ID: mdl-29772620

RESUMEN

A 74-year old man presented to us with difficulty urinating. The laboratory examinations revealed an elevated prostate-specific antigen (PSA) level. A prostate biopsy revealed prostate adenocarcinoma. The computed tomography scan showed metastasis to lymph nodes in the pelvic cavity and both adrenal glands. Combined androgen blockade therapy was started, and PSA levels began to decrease gradually. However, 2 months later, he was hospitalized for treatment of cellulitis and was found to have hypertension, hyperglycemia, and hypokalemia. Endocrinological studies showed elevated serum adrenocorticotropic hormone (ACTH) and serum and urinary cortisol levels. The plasma cortisol level was not reduced by either the 1 mg or 8 mg dexamethasone suppression test, and the plasma cortisol and ACTHlevels did not respond to the corticotropin-releasing hormone test. Computed tomography indicated increased metastasis, and endocrinological studies and other examinations suggested a diagnosis of ectopic ACTHsyndrome. Combined androgen blockade therapy was continued, and inhibitors of adrenocortical synthesis were also administered. However, 16 weeks later, he died of prostate cancer. On autopsy, the histological examination revealed small cell carcinoma in the prostate and adrenal glands and other sites of metastasis, while immunohistochemistry findings revealed that the tumors in the prostate and adrenal glands were strongly positive for ACTH.


Asunto(s)
Síndrome de ACTH Ectópico , Antagonistas de Andrógenos , Carcinoma de Células Pequeñas , Neoplasias de la Próstata , Síndrome de ACTH Ectópico/etiología , Hormona Adrenocorticotrópica , Anciano , Antagonistas de Andrógenos/uso terapéutico , Andrógenos , Carcinoma de Células Pequeñas/diagnóstico , Humanos , Masculino , Neoplasias de la Próstata/complicaciones , Neoplasias de la Próstata/tratamiento farmacológico
9.
Nihon Jinzo Gakkai Shi ; 57(8): 1369-75, 2015.
Artículo en Japonés | MEDLINE | ID: mdl-26817168

RESUMEN

Here we report a rare case of Merkel cell carcinoma complicated with nephrosis and malignant lymphoma. A 79-year-old male, who had undergone rectectomy due to colorectal cancer about 10 years previously, was diagnosed as Merkel cell carcinoma of the left ear lobe with lymph node metastases. Tumor resection and lymph node dissection were performed. A year later, follow-up PET-CT revealed a small hot spot at the ileocecum without apparent tumor formation based on examination by colonoscopy. The patient received 56 Gy of radiation. Two months later, he developed new-onset nephrosis followed by renal failure, and was referred to our hospital (Cr 4.26 mg/dL, UA 13.5 mg/dL, Alb 2.1 g/dL). Further examination negated the possibility of vasculitis, collagen disease, or myeloma kidney. Since his renal function continued to decline, causing uremic symptoms, he was hospitalized and underwent hemodialysis soon after referral. Abdominal CT scan revealed an ileocecal mass with multiple abdominal lymphadenopathy, which was later diagnosed as diffuse large B-cell lymphoma (stage IV) by tumor biopsy. Corticosteroid therapy (prednisolone 60 mg/day) was soon initiated with no response. Local skin redness and blister formation at the left shoulder emerged gradually, which strongly suggested a local recurrence of Merkel cell carcinoma. Despite the use of rituximab, the patient's general condition deteriorated without any sign of recovery. Three months after the start of dialysis, we discontinued dialysis therapy due to his poor health status, and eventually he died of cachexia. Autopsy revealed triple cancers: rectal cancer, Merkel cell carcinoma, and malignant lymphoma. In addition to the case report, we will summarize and discuss former similar case reports in the literature.


Asunto(s)
Carcinoma de Células de Merkel/diagnóstico , Neoplasias del Oído/diagnóstico , Linfoma/diagnóstico , Nefrosis/complicaciones , Neoplasias Cutáneas/diagnóstico , Anciano , Autopsia , Carcinoma de Células de Merkel/complicaciones , Neoplasias del Oído/complicaciones , Resultado Fatal , Humanos , Linfoma/complicaciones , Masculino , Diálisis Renal , Neoplasias Cutáneas/complicaciones
10.
Clin Exp Nephrol ; 17(2): 155-73, 2013 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-23385776

RESUMEN

The Japan Renal Biopsy Registry (J-RBR) was started in 2007 and the Japan Kidney Disease Registry (J-KDR) was then started in 2009 by the Committee for Standardization of Renal Pathological Diagnosis and the Committee for the Kidney Disease Registry of the Japanese Society of Nephrology. The purpose of this report is to describe and summarize the registered data from 2009 and 2010. For the J-KDR, data were collected from 4,016 cases, including 3,336 (83.1 %) by the J-RBR and 680 (16.9 %) other cases from 59 centers in 2009, and from 4,681 cases including 4,106 J-RBR cases (87.7 %) and 575 other cases (12.3 %) from 94 centers in 2010, including the affiliate hospitals. In the J-RBR, 3,165 native kidneys (94.9 %) and 171 renal grafts (5.1 %) and 3,869 native kidneys (94.2 %) and 237 renal grafts (5.8 %) were registered in 2009 and 2010, respectively. Patients younger than 20 years of age comprised 12.1 % of the registered cases, and those 65 years and over comprised 24.5 % of the cases with native kidneys in 2009 and 2010. The most common clinical diagnosis was chronic nephritic syndrome (55.4 % and 50.0 % in 2009 and 2010, respectively), followed by nephrotic syndrome (22.4 % and 27.0 %); the most frequent pathological diagnosis as classified by the pathogenesis was IgA nephropathy (31.6 % and 30.4 %), followed by primary glomerular diseases (except IgA nephropathy) (27.2 % and 28.1 %). Among the primary glomerular diseases (except IgA nephropathy) in the patients with nephrotic syndrome, membranous nephropathy was the most common histopathology in 2009 (40.3 %) and minor glomerular abnormalities (50.0 %) were the most common in 2010 in native kidneys in the J-RBR. Five new secondary and longitudinal research studies by the J-KDR were started in 2009 and one was started in 2010.


Asunto(s)
Biopsia , Enfermedades Renales/patología , Riñón/patología , Sistema de Registros/normas , Adulto , Factores de Edad , Anciano , Anciano de 80 o más Años , Biopsia/normas , Femenino , Glomerulonefritis por IGA/patología , Humanos , Japón/epidemiología , Enfermedades Renales/diagnóstico , Enfermedades Renales/epidemiología , Estudios Longitudinales , Masculino , Persona de Mediana Edad , Pacientes , Estándares de Referencia , Factores Sexuales , Adulto Joven
11.
Hinyokika Kiyo ; 59(3): 175-7, 2013 Mar.
Artículo en Japonés | MEDLINE | ID: mdl-23633633

RESUMEN

Endocervicosis/ endosalpingiosis of the bladder is a very rare benign condition, with a total of 34 documented cases found in the literature. Herein, we report a 35-year-old woman with suspected bladder cancer following a cystoscopic examination, which revealed a mass on the right posterior bladder wall. Transurethral resection of the lesion was performed, and both pathological and immunohistochemical findings confirmed a diagnosis of endocervicosis/ endosalpingiosis. Recurrence was seen 6 months later in a follow-up cystoscopic examination and a partial cystectomy was performed. Since endocervicosis/ endosalpingiosis tends to recur, a partial cystectomy is the best therapeutic option for patients with persistent symptoms or recurrence.


Asunto(s)
Coristoma/patología , Conductos Paramesonéfricos , Enfermedades de la Vejiga Urinaria/patología , Adulto , Coristoma/cirugía , Femenino , Humanos , Enfermedades de la Vejiga Urinaria/cirugía
12.
Case Rep Hematol ; 2023: 4747989, 2023.
Artículo en Inglés | MEDLINE | ID: mdl-37408875

RESUMEN

IgE plasma cell neoplasm is the rarest subtype of plasma cell neoplasms and is known for its poor prognosis and high incidence of t(11;14). However, t(11;14) has been classified as a standard-risk rather than high-risk cytogenetic abnormality in multiple myeloma. We have been unable to explain the discrepancy that the hallmark of IgE plasma cell neoplasm with a poor prognosis is a standard-risk cytogenetic abnormality. Here, we report a case of IgE primary plasma cell leukemia with extramedullary lesions of the liver, stomach, and lymph nodes. Plasma cell infiltration was pathologically confirmed in each organ. Cytogenetic analysis of plasma cells revealed t(11;14) and amplification of 1q21. Chemotherapy, with immunomodulatory imide drugs, proteasome inhibitors, and CD38 antibodies, was unsuccessful. In IgE plasma cell neoplasm, coexistence of other cytogenetic abnormalities with t(11;14) may be important. Investigating the presence of cytogenetic abnormalities coexisting with t(11;14) is not only useful for evaluating prognosis but also important for understanding the pathogenesis of the disease. Recently, venetoclax, an oral BCL2 inhibitor, has demonstrated promising efficacy in plasma cell neoplasm patients harboring t(11;14). Development of an effective venetoclax-based regimen for treating aggressive IgE plasma cell neoplasm with t(11;14) is expected.

13.
Case Rep Hematol ; 2023: 3628712, 2023.
Artículo en Inglés | MEDLINE | ID: mdl-36879893

RESUMEN

Mixed phenotype acute leukemia (MPAL) is characterized by leukemic blasts that express markers of multiple lineages. Compared with acute myeloid leukemia (AML) and acute lymphoblastic leukemia (ALL), MPAL is considered to have a poor treatment outcome. We report a case of MPAL T/myeloid not otherwise specified that was initially presented as multilineage lymphoblastic lymphoma and subsequently developed into leukemic MPAL. An acute lymphoblastic leukemia-based treatment regimen was ineffective, but azacitidine and venetoclax therapy resulted in hematological complete remission. Our case suggests that multilineage lymphoblastic lymphoma should be considered to be the same disease as MPAL, albeit with different clinical presentations. Optimal treatment for MPAL has not been established yet, but azacitidine and venetoclax therapy may be a potential approach.

14.
Clin Transplant ; 26 Suppl 24: 20-4, 2012 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-22747471

RESUMEN

BK virus nephropathy (BKVN) is one of the factors that reduces renal graft function after transplantation. However, BKVN and rejection present similar pathological findings, as both are accompanied by cellular infiltration to the interstitium and tubulitis, thus they are difficult to distinguish for diagnosis and medical treatment. In the present study, we examined immunohistochemical pathological features of BKVN in four cases treated in our hospital from 2007 to 2010. Common immunohistological finding is that tubulitis in these cases was primarily EMA and 34ßE12-positive and existed predominantly from the collecting duct to the distal convoluted tubule. The majority of infected cells existed in EMA and 34ßE12-positive tubules, which were also located mainly from the collecting duct to the distal tubule. In addition, a large number of SV40-positive infected cells were similarly seen. Dylon staining clearly revealed eosinophils. We concluded that the main pathological features of BKVN are the presence of tubulitis and infected cells predominantly from the collecting duct to distal tubule and the appearance of eosinophils.


Asunto(s)
Virus BK/patogenicidad , Biomarcadores/metabolismo , Enfermedades Renales/etiología , Enfermedades Renales/patología , Túbulos Renales/patología , Infecciones por Polyomavirus/complicaciones , Infecciones Tumorales por Virus/complicaciones , Adulto , Femenino , Rechazo de Injerto , Humanos , Técnicas para Inmunoenzimas , Enfermedades Renales/metabolismo , Trasplante de Riñón , Túbulos Renales/metabolismo , Túbulos Renales/virología , Masculino , Persona de Mediana Edad , Infecciones por Polyomavirus/metabolismo , Infecciones por Polyomavirus/virología , Estudios Retrospectivos , Infecciones Tumorales por Virus/metabolismo , Infecciones Tumorales por Virus/virología
15.
Clin Exp Nephrol ; 16(6): 903-20, 2012 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-23053590

RESUMEN

BACKGROUND AND OBJECTIVES: Data regarding renal disease in the elderly (age ≥65 years old) and very elderly (age ≥80 years old) Japanese are extremely limited. The aim of this study was to examine the causes of renal disease and their clinical presentations in elderly patients who underwent renal biopsy. DESIGN, SETTING, PARTICIPANTS, AND MEASUREMENTS: From July 2007 to November 2011, all of the elderly native renal biopsy patients who had been registered in the Japan Renal Biopsy Registry (J-RBR; 2802 including 1596 males and 1206 females) were identified. Their data were compared with a control group of 7416 patients who ranged in age from 20 to 64 years old and were registered on the J-RBR over the same period. In addition, the clinical and pathological classifications of 276 very elderly patients were also analyzed. RESULTS: The indications for biopsy were nephrotic syndrome (NS) in 36.2 and 50.7 % of the elderly and the very elderly patients, chronic nephritic syndrome in 31.8 and 17.4 %, and acute kidney injury including rapidly progressive glomerulonephritis in 18.6 and 22.5 %, respectively. Primary glomerular disease was the most frequent diagnosis, followed by MPO-ANCA-positive nephritis, IgA nephropathy (IgAN), and diabetic nephropathy. In primary GN including IgAN, membranous nephropathy (MN) was the most frequent histological type, followed by IgAN and minor glomerular abnormalities. A comparison with the control group showed that MN, MPO-ANCA-positive nephritis, and amyloid nephropathy were more common in the elderly (P < 0.001), and IgAN was less common (P < 0.001). As for nephrotic syndrome in the elderly, MN was the most common histological type, followed by minimal change NS, diabetic nephropathy, amyloid nephropathy, and focal segmental glomerulosclerosis. There was a significant discrepancy between the urinary protein/creatinine ratio and daily proteinuria after the 7th decade of life. CONCLUSIONS: Renal biopsy is a valuable diagnostic tool, even in elderly and very elderly Japanese patients. In the future, modified clinical guidelines for elderly renal disease should be developed.


Asunto(s)
Factores de Edad , Enfermedades Renales/epidemiología , Enfermedades Renales/patología , Riñón/patología , Adulto , Anciano , Anciano de 80 o más Años , Biopsia , Femenino , Glomerulonefritis/epidemiología , Glomerulonefritis/patología , Glomerulonefritis por IGA/epidemiología , Glomerulonefritis por IGA/patología , Humanos , Incidencia , Japón/epidemiología , Masculino , Persona de Mediana Edad , Síndrome Nefrótico/epidemiología , Síndrome Nefrótico/patología , Sistema de Registros
16.
Clin Exp Nephrol ; 15(4): 493-503, 2011 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-21437579

RESUMEN

BACKGROUND: The Committee for the Standardization of Renal Pathological Diagnosis and the Working Group for Renal Biopsy Database of the Japanese Society of Nephrology started the first nationwide, web-based, and prospective registry system, the Japan Renal Biopsy Registry (J-RBR), to record the pathological, clinical, and laboratory data of renal biopsies in 2007. METHODS: The patient data including age, gender, laboratory data, and clinical and pathological diagnoses were recorded on the web page of the J-RBR, which utilizes the system of the Internet Data and Information Center for Medical Research in the University Hospital Medical Information Network. We analyzed the clinical and pathological diagnoses registered on the J-RBR in 2007 and 2008. RESULTS: Data were collected from 818 patients from 18 centers in 2007 and 1582 patients from 23 centers in 2008, including the affiliated hospitals. Renal biopsies were obtained from 726 native kidneys (88.8%) and 92 renal grafts (11.2%) in 2007, and 1400 native kidneys (88.5%) and 182 renal grafts (11.5%) in 2008. The most common clinical diagnosis was chronic nephritic syndrome (47.4%), followed by nephrotic syndrome (16.8%) and renal transplantation (11.2%) in 2007. A similar frequency of the clinical diagnoses was recognized in 2008. Of the native kidneys, the most frequent pathological diagnosis as classified by pathogenesis was immunoglobulin (Ig) A nephropathy (IgAN) both in 2007 (32.9%) and 2008 (30.2%). Among the primary glomerular diseases (except IgAN), membranous nephropathy (MN) was the most common disease both in 2007 (31.4%) and 2008 (25.7%). CONCLUSIONS: In a cross-sectional study, the J-RBR has shown IgAN to be the most common disease in renal biopsies in 2007 and 2008, consistent with previous Japanese studies. MN predominated in the primary glomerular diseases (except for IgAN). The frequency of the disease and the clinical and demographic correlations should be investigated in further analyses by the J-RBR.


Asunto(s)
Enfermedades Renales/patología , Riñón/patología , Sistema de Registros , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Biopsia , Niño , Preescolar , Estudios Transversales , Femenino , Glomerulonefritis por IGA/epidemiología , Glomerulonefritis por IGA/patología , Glomerulonefritis Membranosa/epidemiología , Glomerulonefritis Membranosa/patología , Humanos , Lactante , Internet , Japón/epidemiología , Enfermedades Renales/epidemiología , Fallo Renal Crónico/epidemiología , Fallo Renal Crónico/patología , Trasplante de Riñón , Masculino , Persona de Mediana Edad , Síndrome Nefrótico/epidemiología , Síndrome Nefrótico/patología
17.
Sci Rep ; 11(1): 2602, 2021 01 28.
Artículo en Inglés | MEDLINE | ID: mdl-33510182

RESUMEN

Focal segmental glomerulosclerosis (FSGS) is a serious condition leading to kidney failure. We aimed to investigate the clinical characteristics of FSGS and its differences compared with minimal change disease (MCD) using cross-sectional data from the Japan Renal Biopsy Registry. In Analysis 1, primary FSGS (n = 996) were stratified by age into three groups: pediatric (< 18 years), adult (18-64 years), and elderly (≥ 65 years), and clinical characteristics were compared. Clinical diagnosis of nephrotic syndrome (NS) was given to 73.5% (97/132) of the pediatric, 41.2% (256/622) of the adult, and 65.7% (159/242) of the elderly group. In Analysis 2, primary FSGS (n = 306) and MCD (n = 1303) whose clinical diagnosis was nephrotic syndrome (NS) and laboratory data were consistent with NS, were enrolled. Logistic regression analysis was conducted to elucidate the variables which can distinguish FSGS from MCD. On multivariable analysis, higher systolic blood pressure, higher serum albumin, lower eGFR, and presence of hematuria associated with FSGS. In Japanese nationwide registry, primary FSGS patients aged 18-64 years showed lower rate of NS than those in other ages. Among primary nephrotic cases, FSGS showed distinct clinical features from MCD.


Asunto(s)
Glomeruloesclerosis Focal y Segmentaria/epidemiología , Nefrosis Lipoidea/epidemiología , Sistema de Registros , Adolescente , Adulto , Factores de Edad , Anciano , Niño , Preescolar , Estudios Transversales , Femenino , Humanos , Japón/epidemiología , Masculino , Persona de Mediana Edad
18.
Int J Hematol ; 114(5): 630-635, 2021 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-34328633

RESUMEN

A previously healthy 49-year-old Japanese woman presented with cervical lymph node swelling and tenderness. Lymph node biopsy revealed reactive lymphadenitis without granulomas. No malignant cells were found, and no acid-fast positive bacilli were identified by Ziehl-Neelsen staining. She was treated unsuccessfully with various antibiotics, and it was very challenging to reach a diagnosis. 18F-Fluorodeoxyglucose (18F-FDG) uptake in bones was evaluated using positron emission tomography-computed tomography (PET-CT), and disseminated mycobacterial infection was suspected. The interferon-gamma (IFN-γ) release assays QuantiFERON (QFT) and T-SPOT were used to diagnose tuberculosis infection. On testing, a difference in mitogen response was found between these assays. The response was low for QFT but adequate for T-SPOT, suggesting the presence of anti-IFN-γ antibodies. This difference depended on whether the patient's plasma (including anti-IFN-γ antibodies) was used within the assay system. Mycobacterium abscessus was isolated from lymph node cultures, and plasma anti-IFN-γ antibodies were confirmed. The patient was diagnosed with disseminated M. abscessus infection with underlying adult-onset immunodeficiency caused by anti-IFN-γ antibodies. Granulomas are a pathological hallmark of mycobacterial infection, but may not fully form in immunodeficient patients. Clinicians should be aware of the possibility of mycobacterial infection without granuloma formation due to anti-IFN-γ antibodies.


Asunto(s)
Anticuerpos/inmunología , Granuloma/diagnóstico , Granuloma/etiología , Interferón gamma/inmunología , Linfadenitis/diagnóstico , Linfadenitis/etiología , Infecciones por Mycobacterium no Tuberculosas/diagnóstico , Infecciones por Mycobacterium no Tuberculosas/microbiología , Anticuerpos/sangre , Manejo de la Enfermedad , Susceptibilidad a Enfermedades , Femenino , Humanos , Inmunohistoquímica , Persona de Mediana Edad , Tomografía de Emisión de Positrones , Evaluación de Síntomas
19.
Urology ; 140: e12-e13, 2020 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-32171696

RESUMEN

A 74-year-old woman presented to the urology clinic with a protruding mass in her urinary bladder detected by ultrasonography. She had no symptoms. Cystoscopy revealed a 1-cm lesion with a lock of 1-cm-long white hair-like structures on the right side of the bladder. White plaques were also noted covering some areas of the bladder. Transurethral resection of the lesion and biopsy of the white plaques were performed. Pathological examination confirmed a diagnosis of keratinizing squamous metaplasia of the bladder with no evidence of malignancy.


Asunto(s)
Neoplasias de la Vejiga Urinaria/patología , Vejiga Urinaria/patología , Anciano , Femenino , Humanos , Metaplasia
20.
Gan To Kagaku Ryoho ; 36(1): 123-5, 2009 Jan.
Artículo en Japonés | MEDLINE | ID: mdl-19151577

RESUMEN

A 58-year-old man who complained of an abdominal tumor was admitted to our hospital. Abdominal CT scan showed that a 15-cm tumor occupied the entire right upper abdomen and that there were ascites and liver metastases. A liver biopsy was performed. The liver biopsy showed a small cell carcinoma pattern, but no definitive origin of the tumor was determined. Considering the extensive peritoneal invasion and multiple liver metastases, he received 2 / courses of cisplatin/etoposide chemotherapy, but his tumor became larger with concomitant abdominal pain and nausea. The patient suddenly died due to multiple organ failure caused by tumor necrosis. The autopsy revealed a pathological diagnosis of primary small cell carcinoma of the pancreas.


Asunto(s)
Carcinoma de Células Gigantes/patología , Carcinoma de Células Pequeñas/patología , Neoplasias Pancreáticas/patología , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Autopsia , Carcinoma de Células Gigantes/diagnóstico por imagen , Carcinoma de Células Gigantes/tratamiento farmacológico , Carcinoma de Células Pequeñas/diagnóstico por imagen , Carcinoma de Células Pequeñas/tratamiento farmacológico , Resultado Fatal , Humanos , Masculino , Persona de Mediana Edad , Metástasis de la Neoplasia/diagnóstico por imagen , Metástasis de la Neoplasia/patología , Neoplasias Pancreáticas/diagnóstico por imagen , Neoplasias Pancreáticas/tratamiento farmacológico , Radiografía
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