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1.
Kyobu Geka ; 77(7): 521-525, 2024 Jul.
Artículo en Japonés | MEDLINE | ID: mdl-39009550

RESUMEN

The remodeling of sinus of Valsalva( Yacoub operation) is being standardized by adding annular fixation and technique for cusp prolapse as a refined method of valve sparing aortic root repair. It is important to design whole configurations of Valsalva sinus( diameters of aortic annulus and sino-tubular junction) according to the patient's geometric height (geometric height-oriented strategy). In this paper, we summarized our tips of remodeling of sinus of Valsalva operation.


Asunto(s)
Seno Aórtico , Humanos , Seno Aórtico/cirugía , Válvula Aórtica/cirugía
2.
Surg Case Rep ; 10(1): 104, 2024 Apr 28.
Artículo en Inglés | MEDLINE | ID: mdl-38678483

RESUMEN

BACKGROUND: Kommerell diverticulum (KD) with right aortic arch and aberrant left subclavian artery (ALSCA) is a rare congenital aortic anomaly. To improve organ compression symptoms and avoid rupture of aneurysms in adulthood (19 years old-), surgical treatment is considered the only curative option. However, in childhood (-18 years old), several problems regarding approach and technique selection have been reported. Surgical treatment for KD in infancy (birth-2 years old) has been reported recently, but rarely in adolescence (13-19 years old). We herein report a case of KD in which the patient underwent graft replacement during adolescence. CASE PRESENTATION: A 13-year-old boy was admitted to our hospital presenting with dysphagia and body weight loss. Esophagography showed upper esophageal stenosis caused by extrinsic compression. Contrast-enhanced computer tomography showed saccular aneurysm formation of KD with right aortic arch (RAA) and ALSCA. Elective surgery including KD resection and graft replacement of the descending aorta was performed via right thoracotomy under partial extracorporeal circulation. The ALSCA was reconstructed by graft interposition. No postoperative complication was observed. Follow-up esophagography showed no residual stenosis. CONCLUSION: We experienced a case of KD with dysphagia and weight loss in adolescence, which was successfully treated with surgery. Graft replacement could be an effective treatment option, facilitating recovery even during the growth period.

3.
J Med Case Rep ; 18(1): 148, 2024 Mar 10.
Artículo en Inglés | MEDLINE | ID: mdl-38461309

RESUMEN

BACKGROUND: Rectus sheath hematoma is a rare presentation often associated with abdominal trauma and anticoagulant therapy. Here, we present a patient with severe rectus sheath hematoma accompanied by nephrotic syndrome who achieved significant clinical improvement without the need for invasive treatment. CASE PRESENTATION: A 72-year-old Japanese woman was referred to our hospital for the treatment of nephrotic syndrome. She was receiving steroid and anticoagulant therapy. Then she had abdominal pain and she was diagnosed with spontaneous rectus sheath hematoma by abdominal computed tomography. She received transfusion and was managed conservatively with bed rest, which led to improvement in abdominal pain. CONCLUSION: Despite the absence of trauma history, rectus sheath hematoma should be considered in patients at risk of vascular failure, including those receiving anticoagulant or steroid therapy, those who are elderly, and those with nephrotic syndrome.


Asunto(s)
Enfermedades Musculares , Síndrome Nefrótico , Femenino , Humanos , Anciano , Recto del Abdomen/diagnóstico por imagen , Síndrome Nefrótico/complicaciones , Anticoagulantes/efectos adversos , Hematoma/inducido químicamente , Hematoma/diagnóstico por imagen , Hematoma/terapia , Dolor Abdominal/inducido químicamente , Enfermedades Musculares/diagnóstico , Esteroides
4.
Ann Thorac Cardiovasc Surg ; 30(1)2024 Jan 26.
Artículo en Inglés | MEDLINE | ID: mdl-37779088

RESUMEN

PURPOSE: Coronary anastomosis is the most key factor to accomplish coronary artery bypass grafting, which is one of the largest areas in cardiovascular surgery. Although we have organized on-site simulator training courses of coronary anastomosis using BEAT YOUCAN, it became difficult to continue it because of COVID-19. Therefore, we established a real-time evaluation sheet instead of an Objective Structured Assessment of Technical Skills (OSATS) evaluation sheet. The purposes of this study was to develop the real-time assessment system and to prove the correlation between the score obtained by the OSATS and the score obtained by the real-time evaluation system. SUBJECTS AND METHODS: A total of 22 videos from the qualifying round of real-time coronary anastomosis competition evaluated by both the modified OSATS and the real-time evaluation system were utilized in this study. The global rating score of OSATS was compared with the global rating score of real-time evaluation system. RESULTS: When examined the relationship between the OSATS total score and the real-time total score, there was a significant correlation (R = 0.752, p <0.001). The OSATS general definition score and the real-time total score also showed a strong correlation (R = 0.733, p <0.001). CONCLUSIONS: We developed a real-time assessment sheet to evaluate coronary anastomosis. This assessment sheet had a good correlation with the OSATS evaluation sheet.


Asunto(s)
Internado y Residencia , Humanos , Vasos Coronarios/diagnóstico por imagen , Vasos Coronarios/cirugía , Competencia Clínica , Resultado del Tratamiento , Anastomosis Quirúrgica
5.
Nat Commun ; 14(1): 8199, 2023 Dec 11.
Artículo en Inglés | MEDLINE | ID: mdl-38081837

RESUMEN

Morphometric studies have revealed the existence of simple geometric relationships among various animal shapes. However, we have little knowledge of the mathematical principles behind the morphogenetic dynamics that form the organ/body shapes of different species. Here, we address this issue by focusing on limb morphogenesis in Gallus gallus domesticus (chicken) and Xenopus laevis (African clawed frog). To compare the deformation dynamics between tissues with different sizes/shapes as well as their developmental rates, we introduce a species-specific rescaled spatial coordinate and a common clock necessary for cross-species synchronization of developmental times. We find that tissue dynamics are well conserved across species under this spacetime coordinate system, at least from the early stages of development through the phase when basic digit patterning is established. For this developmental period, we also reveal that the tissue dynamics of both species are mapped with each other through a time-variant linear transformation in real physical space, from which hypotheses on a species-independent archetype of tissue dynamics and morphogenetic scaling are proposed.


Asunto(s)
Organogénesis , Animales , Morfogénesis , Xenopus laevis
6.
SAGE Open Med Case Rep ; 11: 2050313X231220801, 2023.
Artículo en Inglés | MEDLINE | ID: mdl-38149120

RESUMEN

Kidney involvement in systemic sclerosis occurs in about 20% of cases, with scleroderma renal crisis as a significant complication. However, cases of glomerular disease with massive proteinuria are rare. We present a unique case of systemic sclerosis with the development of nephrotic syndrome. The report provides clinical details and podocyte pathological findings. A 40-year-old male with prior skin sclerosis was diagnosed with systemic sclerosis. Treatment with oral prednisone led to gradual improvement, but a year later, he experienced a systemic sclerosis renal crisis. Using the angiotensin converting enzyme (ACE) inhibitors improved kidney function. However, 3 months later, nephrotic syndrome was diagnosed. Despite an increased prednisolone dose, proteinuria persisted. A kidney biopsy revealed glomerular sclerosis and characteristic vascular changes. Immunofluorescent studies showed no deposits. Electron microscopy confirmed podocyte abnormalities.

7.
J Nephrol ; 36(8): 2257-2267, 2023 11.
Artículo en Inglés | MEDLINE | ID: mdl-37597092

RESUMEN

BACKGROUND: The Japan Renal Biopsy Registry (J-RBR), a nationwide, web-based, registry system, started in 2007. This study aimed to summarise the epidemiology of biopsy-diagnosed kidney disease in Japan over 10 years. METHODS: We analysed the J-RBR database, from 2007 to 2017. Patients' clinical data collected at the time of biopsy and histopathological diagnoses were used for epidemiological and clinicopathologic analyses. RESULTS: The predominant renal biopsy diagnoses were immunoglobulin A nephropathy (39.2%), lupus nephritis (6.5%) and minimal change disease (6.0%) in younger adults (19-64 years), and membranous nephropathy (17.4%), antineutrophil cytoplasmic antibody-associated vasculitis or anti-glomerular basement membrane glomerulonephritis (13.0%), and immunoglobulin A nephropathy (12.5%) in older adults (≥ 65 years). The percentages of patients diagnosed with membranoproliferative glomerulonephritis and immunoglobulin A nephropathy decreased, whereas those with immunoglobulin A vasculitis and diabetic nephropathy increased over the decade. In paediatric patients (< 19 years), immunoglobulin A nephropathy (36.1%), minimal change disease (17.6%), and immunoglobulin A vasculitis (8.6%) were the predominant diagnoses. The percentage of patients diagnosed with immunoglobulin A vasculitis increased over the decade. Based on the sex distribution, minimal change disease and membranous nephropathy were predominant in men aged < 20 and > 40 years, respectively, whereas immunoglobulin A vasculitis and antineutrophil cytoplasmic antibody-associated vasculitis or anti-glomerular basement membrane glomerulonephritis were predominant in women in their 20s and 30s and aged < 50 years, respectively. Immunoglobulin A nephropathy was predominant in men at most ages and in women in their 20s to 40s. CONCLUSIONS: This study describes the distribution and changes in kidney biopsy diagnoses over 10 years in Japan and paves the way for future research on kidney diseases in adults and children.


Asunto(s)
Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos , Glomerulonefritis por IGA , Glomerulonefritis Membranosa , Glomerulonefritis , Vasculitis por IgA , Nefrosis Lipoidea , Adolescente , Adulto , Anciano , Niño , Femenino , Humanos , Masculino , Persona de Mediana Edad , Adulto Joven , Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/diagnóstico , Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/epidemiología , Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/patología , Anticuerpos Anticitoplasma de Neutrófilos , Biopsia , Glomerulonefritis/epidemiología , Glomerulonefritis/patología , Glomerulonefritis por IGA/patología , Glomerulonefritis Membranosa/diagnóstico , Glomerulonefritis Membranosa/epidemiología , Glomerulonefritis Membranosa/patología , Inmunoglobulina A , Japón/epidemiología , Riñón/patología , Nefrosis Lipoidea/patología , Sistema de Registros
8.
J Diabetes Investig ; 14(11): 1268-1278, 2023 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-37483063

RESUMEN

AIMS/INTRODUCTION: This multicenter cohort study retrospectively assessed the association between polar vasculosis and the progression of diabetic kidney disease (DKD) in type 2 diabetes. MATERIALS AND METHODS: We enrolled 811 patients with type 2 diabetes, biopsy-proven DKD, and proteinuria (≥0.15 g/g creatinine [g/day]). The association between polar vasculosis and other kidney lesions was explored. The outcome was DKD progression defined as a composite of renal replacement therapy initiation or 50% decline in estimated glomerular filtration rate (eGFR) from baseline. RESULTS: Of the 811 cases, 677 (83.5%) had polar vasculosis. In multivariate logistic regression analysis, subendothelial widening of the glomerular basement membrane, glomerulomegaly, glomerular class in the Renal Pathology Society classification ≥IIb, vascular lesions, age, eGFR, and hemoglobin A1c were positively associated with polar vasculosis, whereas interstitial fibrosis and tubular atrophy (IFTA) was negatively associated with polar vasculosis. During a median follow-up of 5.2 years, progression of DKD occurred in 322 of 677 (7.4 events/100 person-years) and 79 of 134 (11.4 events/100 person-years) cases with and without polar vasculosis, respectively. Kaplan-Meier analysis showed that polar vasculosis was associated with lower cumulative incidences of DKD progression. Multivariate Cox regression analyses showed that polar vasculosis was associated with a lower risk of DKD progression, regardless of eGFR or proteinuria subgroups. These associations between polar vasculosis and better kidney outcome were unchanged considering all-cause mortality before DKD progression as a competing event. CONCLUSIONS: This study showed that polar vasculosis of DKD was associated with less advanced IFTA and a better kidney outcome in type 2 diabetes with proteinuria.


Asunto(s)
Diabetes Mellitus Tipo 2 , Nefropatías Diabéticas , Humanos , Biopsia , Estudios de Cohortes , Diabetes Mellitus Tipo 2/patología , Nefropatías Diabéticas/epidemiología , Nefropatías Diabéticas/etiología , Nefropatías Diabéticas/patología , Progresión de la Enfermedad , Riñón , Proteinuria/complicaciones , Estudios Retrospectivos
9.
Fukushima J Med Sci ; 69(2): 151-155, 2023 Aug 10.
Artículo en Inglés | MEDLINE | ID: mdl-37225454

RESUMEN

Vascular prosthesis replacement and thoracic endovascular repair (TEVAR) are used to treat patients with enlarged chronic type B aortic dissection. A case in which thrombosis of the false lumen was achieved by the staged combination of these two methods is presented. A 41-year-old woman with a thoracoabdominal aortic aneurysm (maximum short diameter 44 mm) identified 5 years earlier was being monitored as an outpatient in our department when she presented with back pain. Computed tomography (CT) showed acute type B aortic dissection (DeBakey type IIIa), which was managed conservatively. When CT showed an aortic dissection with a patent false lumen immediately below the left subclavian artery bifurcation, one-debranching TEVAR was performed to close the entry, along with right axillary artery to left axillary artery bypass surgery. Outpatient CT at 3 months postoperatively showed rapid enlargement in the vicinity of the celiac artery. Thoracoabdominal aortic replacement to prevent rupture was performed, and the patient was then monitored as an outpatient. CT at age 43 years showed enlargement of the residual false lumen. Additional TEVAR was successfully performed. Thus, three-stage treatment was conducted to enlarge the residual false lumen, causing successful thrombosis of the false lumen.


Asunto(s)
Aneurisma de la Aorta Torácica , Disección Aórtica , Implantación de Prótesis Vascular , Procedimientos Endovasculares , Trombosis , Femenino , Humanos , Adulto , Aneurisma de la Aorta Torácica/cirugía , Aneurisma de la Aorta Torácica/complicaciones , Implantación de Prótesis Vascular/efectos adversos , Resultado del Tratamiento , Procedimientos Endovasculares/efectos adversos , Estudios Retrospectivos , Disección Aórtica/cirugía , Trombosis/etiología , Trombosis/cirugía , Stents/efectos adversos
10.
Dev Biol ; 500: 22-30, 2023 08.
Artículo en Inglés | MEDLINE | ID: mdl-37247832

RESUMEN

Xenopus young tadpoles regenerate a limb with the anteroposterior (AP) pattern, but metamorphosed froglets regenerate a hypomorphic limb after amputation. The key gene for AP patterning, shh, is expressed in a regenerating limb of the tadpole but not in that of the froglet. Genomic DNA in the shh limb-specific enhancer, MFCS1 (ZRS), is hypermethylated in froglets but hypomethylated in tadpoles: shh expression may be controlled by epigenetic regulation of MFCS1. Is MFCS1 specifically activated for regenerating the AP-patterned limb? We generated transgenic Xenopus laevis lines that visualize the MFCS1 enhancer activity with a GFP reporter. The transgenic tadpoles showed GFP expression in hoxd13-and shh-expressing domains of developing and regenerating limbs, whereas the froglets showed no GFP expression in the regenerating limbs despite having hoxd13 expression. Genome sequence analysis and co-transfection assays using cultured cells revealed that Hoxd13 can activate Xenopus MFCS1. These results suggest that MFCS1 activation correlates with regeneration of AP-patterned limbs and that re-activation of epigenetically inactivated MFCS1 would be crucial to confer the ability to non-regenerative animals for regenerating a properly patterned limb.


Asunto(s)
Epigénesis Genética , Extremidades , Animales , Xenopus laevis/genética , Animales Modificados Genéticamente , Extremidades/fisiología , Factores de Transcripción/genética
11.
Sci Rep ; 13(1): 5123, 2023 03 29.
Artículo en Inglés | MEDLINE | ID: mdl-36991090

RESUMEN

We analyzed the seasonal variations in the number of renal biopsies and clinical characteristics of primary glomerular disease in Japan using the Japan Renal Biopsy Registry (J-RBR). We retrospectively collected clinical and pathological data of patients with primary glomerular disease who were registered in the J-RBR between 2007 and 2018. Immunoglobulin A nephropathy (IgAN), minimal change nephrotic syndrome (MCNS), membranous nephropathy (MN), and postinfectious acute glomerulonephritis (PIAGN) constituted the four major glomerular disorders included in this study (total, 13,989; IgAN, 9121; MCNS, 2298; MN, 2447; and PIAGN, 123). The number of patients with IgAN or MCNS was higher during summer. However, no overt seasonal variations were observed in patients with MN or PIAGN. Subgroup analyses suggested that in the patients with IgAN, more renal biopsies of severe cases were performed during winter, probably owing to age and blood pressure. Furthermore, more renal biopsies of severe cases were performed during spring and winter in patients with MCNS even after adjusting for the abovementioned host factors. This study suggests that seasonal factors influence the decision to perform renal biopsy as well as the pathogenesis of primary glomerular disease. Thus, our findings may provide important insights regarding the pathophysiology of primary glomerular disease.


Asunto(s)
Glomerulonefritis por IGA , Glomerulonefritis Membranosa , Glomerulonefritis , Nefrosis Lipoidea , Humanos , Riñón/patología , Estaciones del Año , Estudios Retrospectivos , Japón/epidemiología , Glomerulonefritis por IGA/patología , Glomerulonefritis/patología , Glomerulonefritis Membranosa/patología , Nefrosis Lipoidea/patología , Sistema de Registros , Biopsia
12.
Dev Growth Differ ; 65(2): 86-93, 2023 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-36680534

RESUMEN

Amphibians and fish often regenerate lost parts of their appendages (tail, limb, and fin) after amputation. Limb regeneration in adult amphibians provides an excellent model for appendage (limb) regeneration through 3D morphogenesis along the proximodistal, dorsoventral, and anteroposterior axes in mammals, because the limb is a homologous organ among amphibians and mammals. However, manipulating gene expression in specific appendages of adult amphibians remains difficult; this in turn hinders elucidation of the molecular mechanisms underlying appendage regeneration. To address this problem, we devised a system for appendage-specific gene induction using a simplified protocol named the "agarose-embedded heat shock (AeHS) method" involving the combination of a heat-shock-inducible system and insertion of an appendage in a temperature-controlled agarose gel. Gene expression was then induced specifically and ubiquitously in the regenerating limbs of metamorphosed amphibians, including a frog (Xenopus laevis) and newt (Pleurodeles waltl). We also induced gene expression in the regenerating tail of a metamorphosed P. waltl newt using the same method. This method can be applied to adult amphibians with large body sizes. Furthermore, this method enables simultaneous induction of gene expression in multiple individuals; further, the data are obtained in a reproducible manner, enabling the analysis of gene functions in limb and tail regeneration. Therefore, this method will facilitate elucidation of the molecular mechanisms underlying appendage regeneration in amphibians, which can support the development of regenerative therapies for organs, such as the limbs and spinal cord.


Asunto(s)
Pleurodeles , Médula Espinal , Animales , Xenopus laevis/genética , Pleurodeles/genética , Sefarosa , Expresión Génica , Mamíferos
13.
Blood Purif ; 52(4): 373-381, 2023.
Artículo en Inglés | MEDLINE | ID: mdl-36521435

RESUMEN

INTRODUCTION: Treating diabetic nephropathy with low-density lipoprotein (LDL) apheresis reduces proteinuria and improves prognosis. However, its impact on patients' quality of life (QoL) is unclear. This study evaluated the effect of LDL apheresis on QoL in patients with diabetes, proteinuria, and hypercholesterolemia. METHODS: In this nationwide multicenter prospective study, we enrolled 40 patients with diabetes. Inclusion criteria were proteinuria (defined as an albumin/creatinine ratio ≥3 g/g), serum creatinine levels <2 mg/dL, and serum LDL ≥120 mg/dL despite drug treatment. LDL apheresis was performed 6-12 times within 12 weeks. The 36-item Short Form Health Survey (SF-36) was used to analyze QoL. RESULTS: The study enrolled 35 patients (27 men and 8 women; mean age 58.9 ± 11.9 years). A comparison of baseline SF-36 values with those at the end of the course of apheresis found an improvement in the mean physical component summary (37.9 ± 11.4 vs. 40.6 ± 10.5, p = 0.051) and a significant increase in the mean mental component summary (MCS) (49.4 ± 8.4 vs. 52.5 ± 10.9, p = 0.026). A multivariable linear regression analysis revealed a history of coronary heart disease negatively correlated with the MCS increase at the end of the course of apheresis (ß coefficient -6.935, 95% confidence interval, 13.313 to-0.556, p = 0.034). CONCLUSION: Our results suggest that LDL apheresis may improve the mental and physical QoL in patients with diabetes, proteinuria, and hypercholesterolemia.


Asunto(s)
Eliminación de Componentes Sanguíneos , Diabetes Mellitus , Nefropatías Diabéticas , Hipercolesterolemia , Masculino , Humanos , Femenino , Persona de Mediana Edad , Anciano , Calidad de Vida , Estudios Prospectivos , Eliminación de Componentes Sanguíneos/métodos , Lipoproteínas LDL , Proteinuria/terapia , Nefropatías Diabéticas/terapia , Resultado del Tratamiento , Diabetes Mellitus/terapia
14.
Clin Exp Nephrol ; 27(2): 141-150, 2023 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-36329296

RESUMEN

BACKGROUND: Fabry disease (FD) is an X-linked inherited disease where renal complications are associated with a poor prognosis. However, little is known about the prevalence of Fabry nephropathy (FN) in patients with chronic kidney disease (CKD). We extracted FN data from the Japan Renal Biopsy Registry, analyzed the prevalence of FN, and examined the correlation between clinical characteristics and renal involvement according to sex differences and hemi- and heterozygosity in patients with FD. METHODS: A total of 38,351 participants who underwent renal biopsy were retrospectively enrolled, and FN was determined. The clinical characteristics of FD patients were examined based on sex differences. RESULTS: Twenty-nine patients (0.076%) (19 males and 10 females, mean age: 43.7 ± 15.5 years old) were diagnosed with FN. Median estimated urinary protein (UP) and mean eGFR levels were 0.9 [interquartile range (IQR) [0.7-1.6] g/gCr and 67.1 ± 36.8 mL/min/1.73 m2, respectively. Mean systolic blood pressure (SBP) was 126.4 ± 17.1 mmHg and diastolic blood pressure was 76.1 ± 12.6 mmHg. An inverse correlation between eGFR and logarithm UP levels was observed (r2 = 0.23, p = 0.02), SBP was positively associated with logarithm UP (r2 = 0.34, p = 0.004) overall and inversely associated with eGFR (r2 = 0.25, p = 0.007) regardless of sex, and SBP was an independent determinant of proteinuria (p = 0.004) and eGFR (p = 0.007). CONCLUSIONS: The prevalence of biopsy-proven FN was 0.076%. Since SBP is associated with eGFR regardless of zygosity, strict SBP control might be necessary to prevent progression to end-stage kidney disease in both male and female patients with FN.


Asunto(s)
Enfermedad de Fabry , Insuficiencia Renal Crónica , Adulto , Femenino , Humanos , Masculino , Persona de Mediana Edad , Biopsia , Estudios Transversales , Enfermedad de Fabry/complicaciones , Enfermedad de Fabry/diagnóstico , Enfermedad de Fabry/epidemiología , Tasa de Filtración Glomerular , Japón/epidemiología , Sistema de Registros , Insuficiencia Renal Crónica/diagnóstico , Insuficiencia Renal Crónica/epidemiología , Insuficiencia Renal Crónica/etiología , Estudios Retrospectivos
15.
Cureus ; 15(12): e49892, 2023 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-38174198

RESUMEN

Recently, the association between membranous nephropathy (MN) and malignancy has been recognized in about 30% of epidermal growth factor-like 1 (NELL-1) positive cases. However, the mechanism of association with MN and malignancy remains under search. In this report, we present a unique case of MN with positive staining for both thrombospondin type-1 domain-containing 7A (THSD7A) and NELL-1. An 80-year-old Japanese woman with nephrotic syndrome (NS) was diagnosed as an immunoglobulin (Ig)G1 subclass predominant secondary MN with weakly positive for THSD7A staining. Then, advanced cancer in the sigmoid colon was found during screening tests for malignancy. After the removal of colon carcinoma, complete remission was achieved at 28 weeks follow-up after operation. Five years later, she remained in remission and passed without recurrence. Thereafter, we examined again newly reported NELL-1 in renal biopsy specimens and found very strong staining along the glomerular capillary walls. Moreover, in resected tumor tissues, NELL-1 was strongly positive at the basal side of adenocarcinoma cells, but THSD7A staining was negative. This case report provides clinical details and highlights the utility of autoantibodies, especially NELL-1, in the diagnosis and treatment of secondary MN with malignancy.

17.
Int J Nephrol ; 2022: 2718810, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-35983504

RESUMEN

This retrospective exploratory study aimed to identify early clinical indicators of kidney prognosis in primary nephrotic syndrome (NS). Univariate Cox proportional hazards regression analysis identified clinical parameters in the 2-month period after initiating immunosuppressive therapy (IST); it predicted 40% reduction in the estimated glomerular filtration rate (eGFR) in 36 patients with primary NS. Time-dependent receiver operating characteristic curve analysis was used to evaluate the performance of the predictors for the cumulative incidence of 40% reduction in the eGFR up to 8 years after initiating IST. The mean follow-up period was 71.9 months. The eGFR was reduced by 40% in four patients. Significant predictors for time to 40% reduction in the eGFR were as follows: an increase in the serum soluble urokinase plasminogen activator receptor (s-suPAR) 2 months after initiating IST (Δs-suPAR (2M); hazard ratio (HR) for every 500 pg/mL increase: 1.36, P=0.006), s-suPAR at 2 months after initiating IST (s-suPAR (2M); HR for every 500 pg/mL increase: 1.13, P=0.015), urinary protein-to-creatinine ratio (u-PCR) (u-PCR (2M); HR for every 1.0 g/gCr increase: 2.94, P=0.003), and urinary liver-type fatty acid-binding protein (u-L-FABP) (u-L-FABP (2M); HR for every 1.0 µg/gCr increase: 1.14, P=0.006). All four factors exhibited high predictive accuracy for cumulative incidence of 40% reduction in the eGFR up to 8 years after initiating IST, with areas under the receiver operating characteristic curve of 0.92 for Δs-suPAR (2M), 0.87 for s-suPAR (2M), 0.93 for u-PCR (2M), and 0.93 for u-L-FABP (2M). These findings suggest that Δs-suPAR (2M), s-suPAR (2M), u-PCR (2M), and u-L-FABP (2M) could be useful indicators of initial therapeutic response for predicting kidney prognosis in primary NS.

18.
Case Rep Nephrol Dial ; 12(2): 96-104, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-35950048

RESUMEN

Recently, several cases of novel apolipoprotein E (apoE)-related glomerular disease known as membranous nephropathy (MN)-like apoE deposition disease with apoE Toyonaka (Ser197Cys) and homozygous apoE2/2 have been reported. However, the clinical and pathological characteristics are uncertain due to the small number of reports. Here, we report an additional case with various clinical and pathological characteristics. A 28-year-old Japanese man with mild proteinuria and hematuria underwent a kidney biopsy. Examination under a light microscope revealed mesangial proliferation, mesangial matrix expansion, and segmental spike lesion. An immunofluorescence study showed no immunoglobulin or complement depositions. In the electron microscopic (EM) examination, massive deposits with various electron densities in the subepithelial, subendothelial, and paramesangial areas were more prominent than those reported in previous cases, which resembled microbubbles or microcysts on higher magnification. The glomerular basement membrane (GBM) structure was partly degenerated by these deposits. Serum triglyceride and cholesterol levels were within the normal range. However, the serum apoE concentration was significantly high, and glomerular apoE accumulation was detected in immunohistochemistry. The DNA sequence revealed apoE Toyonaka and homozygous apoE2/2 similar to that of the previous cases with MN-like apoE deposition disease. MN-like apoE deposition disease can manifest as only mild hematuria and proteinuria without dyslipidemia. Various characteristic deposits associated with GBM degeneration can be observed in the EM study.

19.
Clin Exp Nephrol ; 26(12): 1170-1179, 2022 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-35962244

RESUMEN

BACKGROUND: Prognosis of nephrotic syndrome has been evaluated based on pathological diagnosis, whereas its clinical course is monitored using objective items and the treatment strategy is largely the same. We examined whether the entire natural history of nephrotic syndrome could be evaluated using objective common clinical items. METHODS: Machine learning clustering was performed on 205 cases from the Japan Nephrotic Syndrome Cohort Study, whose clinical parameters, serum creatinine, serum albumin, dipstick hematuria, and proteinuria were traceable after kidney biopsy at 5 measured points up to 2 years. The clinical patterns of time-series data were learned using long short-term memory (LSTM)-encoder-decoder architecture, an unsupervised machine learning classifier. Clinical clusters were defined as Gaussian mixture distributions in a two-dimensional scatter plot based on the highest log-likelihood. RESULTS: Time-series data of nephrotic syndrome were classified into four clusters. Patients in the fourth cluster showed the increase in serum creatinine in the later part of the follow-up period. Patients in both the third and fourth clusters were initially high in both hematuria and proteinuria, whereas a lack of decline in the urinary protein level preceded the worsening of kidney function in fourth cluster. The original diseases of fourth cluster included all the disease studied in this cohort. CONCLUSIONS: Four kinds of clinical courses were identified in nephrotic syndrome. This classified clinical course may help objectively grasp the actual condition or treatment resistance of individual patients with nephrotic syndrome.


Asunto(s)
Aprendizaje Profundo , Síndrome Nefrótico , Humanos , Síndrome Nefrótico/tratamiento farmacológico , Creatinina , Estudios de Cohortes , Hematuria , Japón , Proteinuria/etiología
20.
JTCVS Open ; 10: 75-84, 2022 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-36004215

RESUMEN

Objectives: The aim of this study was to assess potential predictors of aortic events after an emergency surgery for acute type A aortic dissection, especially paying attention to the findings of computed tomography (CT) performed immediately after the surgery. Methods: Between January 2001 and December 2015, 72 patients, who were diagnosed as having Stanford type A acute aortic dissection with a patent false lumen in the descending thoracic aorta, survived the emergency operation, and had postoperative CT scan data, were included in this study (mean follow-up, 8.2 ± 3.8 years; range 0.8-17.4 years). From the CT scan data, the diameter of the false lumen (FL-D) and true lumen (TL-D) were measured, and the FL-D:TL-D ratio was calculated. Long-term outcomes of the FL-D > TL-D group (n = 30) and the FL-D < TL-D group (n = 42) were compared. Results: In the late follow-up, 17 aortic events in the downstream aorta were observed. The FL-D:TL-D ratio (P = .01) was an adjusted risk of aortic events in multivariable analysis. The rates of freedom from aortic events at 5 and 9 years were superior in the FL-D < TL-D group than in the FL-D > TL-D group (92.0% and 88.6% vs 81% and 60.7%; log rank P < .05). Conclusions: Our results suggest that the false lumen:true lumen ratio predicts long-term prognosis after surgical repair of acute type A aortic dissection.

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