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A 48-year-old woman visited our hospital because of bilateral lacrimal gland enlargement. Her serum immunoglobulin G4 (IgG4) level was high, and positron emission tomography-computed tomography showed significant positive findings in the bilateral lacrimal gland. A biopsy revealed a considerable increase in IgG4/CD138, leading to a diagnosis of IgG4-related dacryoadenitis. The disease did not respond to steroid therapy, so treatment was started with baricitinib because of exacerbation of the original atopic dermatitis and dacryoadenitis after the second dose of the coronavirus disease 2019 (COVID-19) vaccine. Baricitinib was effective for resolving both dermatitis and dacryoadenitis, and steroids were able to be discontinued. The IgG4 level also improved.
Assuntos
Azetidinas , Dacriocistite , Aparelho Lacrimal , Purinas , Pirazóis , Sulfonamidas , Feminino , Humanos , Pessoa de Meia-Idade , Biópsia , Dacriocistite/tratamento farmacológico , Dacriocistite/etiologia , Imunoglobulina G , Aparelho Lacrimal/patologiaRESUMO
We experienced three cases of a fever and subsequent severe, prolonged gross hematuria after COVID-19 vaccination. A kidney biopsy revealed immunoglobulin A (IgA) nephropathy, and electron microscopy showed two types of podocytopathy (podocyte damage): loss of foot processes from the glomerular basement membrane and foot process effacement. Mesangial interposition was also present in cases 1 and 3 but not in case 2. Podocytopathy is known to be a cause of proteinuria; however, the reactions to COVID-19 vaccination described here suggest that it may also be related to hematuria in IgA nephropathy.
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We performed 3 kidney biopsies in a 71-year-old man. At the first biopsy, we made the diagnosis of immunoglobulin G4 (IgG4)-related interstitial nephritis characterized by the simultaneous presence of IgG4-positive plasma cells and characteristic fibrosis with a bird's-eye pattern. At the second biopsy, rather than finding fibrosis as a post-inflammatory scar, we noted that steroid treatment had caused the simultaneous disappearance of IgG4-positive plasma cells and fibrosis and had restored the normal tubular structure. The third biopsy showed the recurrence of the disease with inflammatory cells accompanied by fibrosis. These findings suggest that IgG4-positive plasma cells and fibrosis occur simultaneously.
Assuntos
Nefrite Intersticial , Plasmócitos , Masculino , Humanos , Idoso , Plasmócitos/patologia , Nefrite Intersticial/complicações , Fibrose , Imunoglobulina G , EsteroidesRESUMO
A 42-year-old Japanese woman was admitted for the evaluation of proteinuria. She had a history of four habitual abortions and valvular heart disease, including severe mitral regurgitation and moderate tricuspid regurgitation. A kidney biopsy showed fibrointimal thickening of interlobular arteries, fibrin thrombosis, and associated focal segmental sclerosis. Although the standard test for antiphospholipid (aPL) antibodies was negative, the patient was diagnosed with antiphospholipid syndrome (APS)-related disease by testing for phosphatidylserine dependent anti-prothrombin anticardiolipin antibody, a non-criterial aPL antibody. A kidney biopsy may lead to a diagnosis of APS in patients with negative laboratory test findings for APS.
Assuntos
Síndrome Antifosfolipídica , Nefropatias , Lúpus Eritematoso Sistêmico , Trombose , Feminino , Gravidez , Humanos , Adulto , Síndrome Antifosfolipídica/diagnóstico , Fosfatidilserinas , Anticorpos AntifosfolipídeosRESUMO
BACKGROUND: Dipeptidyl peptidase-4 (DPP-4) inhibitors are widely used to treat type 2 diabetes (T2D). Lowering blood glucose is expected also to reduce the progression of diabetic nephropathy. We experienced a patient with T2D who achieved good glycemic control with a DPP-4 inhibitor but experienced rapid deterioration of renal function. Therefore, we performed a retrospective study of similar patients treated at our hospital. METHODS: Out of 56 patients with biopsy-proven diabetic nephropathy who underwent native kidney biopsy at Toranomon Hospital from January 2018 through December 2022, we selected 22 patients who had been receiving DPP-4 inhibitors for at least 9 months at the time of kidney biopsy. Of these patients, we evaluated 16 diagnosed with class IIa diabetic nephropathy according to Tervaert's pathologic classification. The yearly estimated glomerular filtration rate (eGFR) slope in the 16 patients was arranged from the highest to the lowest slope. Ten patients with a large eGFR slope had thrombotic microangiopathy (TMA)-like lesions characterized by glomerular endothelial cell proliferation and GBM duplication on kidney biopsy (group A), whereas the remaining 6 patients did not have TMA-like lesions (group B). RESULTS: Group A had a median (interquartile range [IQR]) eGFR of 18.2 (16.2, 26.2) and a yearly median (IQR) eGFR slope of -11.2 (-17.6, -9.2) mL/min/1.73 m2 after of DPP-4 administration, whereas group B had a median (IQR) eGFR of 31.5 (21.9, 34.8) mL/min/1.73 m2 and a yearly median (IQR) eGFR slope of -1.6 (-3.1, -0.3). Renal function declined significantly more rapidly in group A than in group B, and proteinuria was higher in group A than in group B (median [IQR], 3.4 [2.6, 4.4] g/day vs 0.8 [0.4, 1.3] g/day, respectively). Five patients in group A progressed to dialysis during follow-up, but none of the patients in group B did. Median (IQR) hemoglobin A1c was 6.2 % (6.0 %, 6.6 %) in group A and 5.8 % (5.7 %, 6.6 %) in group B. CONCLUSION: DPP-4 inhibitors promote vascular endothelial regeneration, but when this effect occurs in the glomerulus, glomerular endothelial cell proliferation leads to TMA-like lesions, which may cause an increase in proteinuria and rapid decline in renal function.
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Diabetes Mellitus Tipo 2 , Nefropatias Diabéticas , Inibidores da Dipeptidil Peptidase IV , Humanos , Inibidores da Dipeptidil Peptidase IV/efeitos adversos , Diabetes Mellitus Tipo 2/complicações , Diabetes Mellitus Tipo 2/tratamento farmacológico , Diabetes Mellitus Tipo 2/induzido quimicamente , Estudos Retrospectivos , Hipoglicemiantes/efeitos adversos , Taxa de Filtração Glomerular , Proteinúria , Dipeptidil Peptidases e Tripeptidil Peptidases/farmacologia , Dipeptidil Peptidase 4RESUMO
A 62-year-old man with type 2 diabetes was admitted because of a decrease in estimated glomerular filtration rate from 72 to 17.5 mL/min/1.73 m2 in 10 years and development of widespread bullous skin lesions. His hemoglobin A1c level had been maintained at 6.0-7.0% for 10 years with a dipeptidyl peptidase (DPP)-4 inhibitor. Skin biopsy showed typical bullous pemphigoid, and kidney biopsy showed tubulointerstitial nephritis with eosinophilic infiltration and glomerular endothelial cell proliferation. After discontinuing the DPP-4 inhibitor, skin lesions improved, and renal decline slowed. This case indicates that DPP-4 inhibitors can cause not only skin lesions but also renal disease.
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We experienced a 36-year-old man with lupus nephritis and antiphospholipid syndrome (APS) who received a donor kidney from his father. Twenty-two months after transplantation, at a time of poor adherence to immunosuppressants and warfarin, the patient developed sudden graft loss due to hemolytic uremic syndrome with rapid deterioration of renal function, thrombocytopenia, and hemolytic anemia. A kidney biopsy showed thrombotic microangiopathy (TMA) related to platelet thrombus formation; however, there was no recurrence of lupus and no findings suggestive of post-transplant rejection, so acute TMA associated with APS was thought to be the cause of the graft loss. This case highlights the importance of instructing patients with lupus nephritis to adhere to treatment with warfarin, a therapeutic drug for APS.
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Síndrome Antifosfolipídica , Nefropatias , Transplante de Rim , Nefrite Lúpica , Microangiopatias Trombóticas , Masculino , Humanos , Adulto , Síndrome Antifosfolipídica/complicações , Síndrome Antifosfolipídica/tratamento farmacológico , Transplante de Rim/efeitos adversos , Nefrite Lúpica/complicações , Varfarina/uso terapêutico , Microangiopatias Trombóticas/complicações , Nefropatias/complicaçõesRESUMO
A 35-year-old woman was treated with chemotherapy for leukemia. One year later, allogeneic hematopoietic stem cell transplantation (HSCT) was performed with umbilical cord blood. After nine months, she developed a spiking fever, sore throat, arthralgia, pleural effusion, hyperferritinemia, and persistent generalized pruritic erythema. A skin biopsy showed dyskeratotic cells in the epidermis, neutrophil infiltration in the epidermis and upper dermis, and neutrophils in the parakeratotic layer. Treatment with tocilizumab was effective. Adult-onset Still's disease (AOSD)-like disease related to graft versus-host disease (GVHD) after HSCT was suspected. Abnormal immune states related to GVHD may cause AOSD-like disease with more severe skin lesions than usual.
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Dermatite Esfoliativa , Transplante de Células-Tronco Hematopoéticas , Doença de Still de Início Tardio , Adulto , Feminino , Humanos , Doença de Still de Início Tardio/complicações , Doença de Still de Início Tardio/diagnóstico , Doença de Still de Início Tardio/tratamento farmacológico , Dermatite Esfoliativa/etiologia , Dermatite Esfoliativa/patologia , Pele/patologia , Eritema/patologia , Transplante de Células-Tronco Hematopoéticas/efeitos adversosRESUMO
A brachio-brachial arteriovenous fistula with superficialization of the brachial vein and superficialization of the brachial artery are useful vascular access techniques for hemodialysis patients. However, both typically require a long skin incision from the antecubital fossa toward the axillary fossa. In addition, the brachio-brachial arteriovenous fistula in particular, which is created with not a one-stage but a two-stage procedure, requires a relatively long time of 2-3 months before it can be used for hemodialysis. Furthermore, superficialization of the brachial artery usually requires nonarterialized superficial veins for blood return. In cases where patients have no adequate superficial veins for creating an arteriovenous fistula, we have adopted a one-stage operative technique to create a brachio-brachial arteriovenous fistula with superficialization of not only the brachial vein but also the brachial artery using a short skin incision. This technique of a brachio-brachial arteriovenous fistula with superficialization of the brachial artery has several advantages over traditional approaches, including a minimally invasive procedure and early use for vascular access. To our knowledge, the presently described technique and the related data have not been previously reported in the English literature. We herein report the steps of this technique and the midterm follow-up outcomes.
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Derivação Arteriovenosa Cirúrgica , Artéria Braquial/cirurgia , Diálise Renal , Extremidade Superior/irrigação sanguínea , Veias/cirurgia , Idoso , Idoso de 80 Anos ou mais , Derivação Arteriovenosa Cirúrgica/efeitos adversos , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Resultado do TratamentoRESUMO
A radiocephalic arteriovenous fistula in the anatomical snuffbox (tabatière region) was first described in 1969 as the most peripheral site for arteriovenous fistula in the upper limb. In cases in which the internal diameter of the first dorsal metacarpal vein under avascularization is ⩾2.0 mm, we have adopted a new operative technique for creating a radial artery-first dorsal metacarpal vein arteriovenous fistula in the first interdigital space of the dorsal hand, which lies between the thumb and the index finger. This technique is the creation of the arteriovenous fistula using the first dorsal metacarpal vein and the most peripheral site in the upper limb. To our knowledge, no previous report has described the creation of a radial artery-first dorsal metacarpal vein arteriovenous fistula. We herein describe the steps of the technique and report its successful performance in a patient with chronic renal failure.
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Derivação Arteriovenosa Cirúrgica , Mãos/irrigação sanguínea , Falência Renal Crônica/terapia , Ossos Metacarpais/irrigação sanguínea , Artéria Radial/cirurgia , Diálise Renal , Veias/cirurgia , Idoso , Humanos , Falência Renal Crônica/diagnóstico , Falência Renal Crônica/fisiopatologia , Masculino , Artéria Radial/diagnóstico por imagem , Artéria Radial/fisiopatologia , Resultado do Tratamento , Grau de Desobstrução Vascular , Veias/diagnóstico por imagem , Veias/fisiopatologiaRESUMO
INTRODUCTION: The creation of the first arteriovenous fistula (AVF) as far distally in the upper limb as possible is ideal. We developed a new operative technique for creating a radial artery-second dorsal metacarpal vein AVF in the first interdigital space. This technique involves the creation of the AVF using the most peripheral site and autologous vein in the upper limb. CASE PRESENTATION: We herein describe the steps of this technique and its successful performance in a 71-year-old man with end-stage renal disease. DISCUSSION: This technique has several advantages including preserving many future vascular access options and providing a long segment of arterialized vein for cannulation. CONCLUSION: We consider this technique to be a worthwhile option and recommend the use in patients with the proper vessels for the creation of the AVF.
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Various factors are considered to be mechanisms of the increase in the sizes of cysts in patients with polycystic kidney disease. Vasopressin is one of the causes, and drinking large volumes of water shows an effect of suppressing an increase in cysts. On the other hand, it is known that hydrogen-rich water reduces oxidative stress and has a good effect on kidney injury. We examined whether drinking large volumes of hydrogen-rich water affected the increase in the sizes of cysts. Forty 5-week-old PCK rats were randomly assigned to four groups: C(Control), purified water; W(Water), water with sugar; H(Hydrogen), hydrogen-rich water; WH(Water+Hydrogen), hydrogen-rich water with sugar. They consumed water from 5 to 15 weeks of age. The intake of water in the groups in which sugar was added to the water (W, WH) significantly increased in comparison to C, but there was no significant change in the serum Creatinine concentration. The kidney weight per body weight in W was significantly decreased in comparison to C. The kidney weights in H and WH were significantly increased in comparison to W. There were no significant differences in the ratio of the cross-sectional area of the cysts to the whole area among the groups. This experiment showed that the effect of drinking large volumes of hydrogen-rich water was not significantly different from that of normal water, in terms of preventing an increase in the size of cysts in PCK rats. However, some papers acknowledge the influence of hydrogen water. Significant differences might become obvious if we change aspects such as the administration method or administration period.