RESUMO
Distinguishing autosomal-dominant polycystic kidney disease (ADPKD) from other inherited renal cystic diseases in patients with adult polycystic kidney disease and no family history is critical for correct treatment and appropriate genetic counseling. However, for patients with no family history, there are no definitive imaging findings that provide an unequivocal ADPKD diagnosis. We analyzed 53 adult polycystic kidney disease patients with no family history. Comprehensive genetic testing was performed using capture-based next-generation sequencing for 69 genes currently known to cause hereditary renal cystic diseases including ADPKD. Through our analysis, 32 patients had PKD1 or PKD2 mutations. Additionally, 3 patients with disease-causing mutations in NPHP4, PKHD1, and OFD1 were diagnosed with an inherited renal cystic disease other than ADPKD. In patients with PKD1 or PKD2 mutations, the prevalence of polycystic liver disease, defined as more than 20 liver cysts, was significantly higher (71.9% vs 33.3%, P = .006), total kidney volume was significantly increased (median, 1580.7 mL vs 791.0 mL, P = .027) and mean arterial pressure was significantly higher (median, 98 mm Hg vs 91 mm Hg, P = .012). The genetic screening approach and clinical features described here are potentially beneficial for optimal management of adult sporadic polycystic kidney disease patients.
Assuntos
Cistos/etiologia , Cistos/patologia , Rim/patologia , Fígado/patologia , Rim Policístico Autossômico Dominante/diagnóstico , Rim Policístico Autossômico Dominante/genética , Canais de Cátion TRPP/genética , Idoso , Feminino , Sequenciamento de Nucleotídeos em Larga Escala , Humanos , Testes de Função Renal , Testes de Função Hepática , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Mutação , Fenótipo , Rim Policístico Autossômico Dominante/complicações , Rim Policístico Autossômico Dominante/epidemiologia , Prevalência , Tomografia Computadorizada por Raios XRESUMO
UNLABELLED: Once-weekly 56.5-µg teriparatide treatment was significantly associated with the increase in lumbar spine bone mineral density at 48 weeks among hemodialysis patients with hypoparathyroidism and low bone mass; however, discontinuation of treatment because of adverse events was frequently observed. Careful monitoring for adverse events should be required. INTRODUCTION: Once-weekly 56.5-µg teriparatide is reportedly effective for treating osteoporotic patients without renal insufficiency. However, little is known about the efficacy and safety of once-weekly teriparatide in hemodialysis patients. METHODS: We conducted a 48-week prospective, observational cohort study including 22 hemodialysis patients aged 20 years or older with hypoparathyroidism and low bone mass who received once-weekly teriparatide at 56.5 µg at a tertiary care hospital between January 2013 and January 2015. Primary outcomes were within-subject percent changes of bone mineral density (BMD) at the lumbar spine, femoral neck, and distal one-third radius at 24 and 48 weeks. Secondary outcomes included percent changes of serum bone turnover markers (osteocalcin, bone-specific alkaline phosphatase (BAP), N-terminal propeptide of procollagen type 1 (P1NP), and tartrate-resistant acid phosphatase 5b (TRAP-5b)). Adverse events were evaluated. RESULTS: The BMD increased at the lumbar spine by 3.3 ± 1.9 % (mean ± SEM) and 3.0 ± 1.8 % at 24 and 48 weeks but not in the femoral neck and distal one-third radius. Serum osteocalcin, BAP, and P1NP increased significantly at 4 weeks, maintaining higher concentrations up to 48 weeks, although TRAP-5b decreased gradually during treatment. The baseline BAP was significantly associated with the 48-week percent change in lumbar spine BMD. Transient hypotension was the most common adverse event. Ten patients discontinued treatment because of adverse events. CONCLUSIONS: Once-weekly teriparatide was associated with increased lumbar spine BMD in hemodialysis patients with hypoparathyroidism and low bone mass. Careful monitoring should be required for treatment of such patients.
Assuntos
Conservadores da Densidade Óssea/administração & dosagem , Hipoparatireoidismo/complicações , Falência Renal Crônica/complicações , Osteoporose/tratamento farmacológico , Diálise Renal , Teriparatida/administração & dosagem , Idoso , Idoso de 80 Anos ou mais , Densidade Óssea/efeitos dos fármacos , Conservadores da Densidade Óssea/efeitos adversos , Conservadores da Densidade Óssea/uso terapêutico , Esquema de Medicação , Feminino , Colo do Fêmur/fisiopatologia , Humanos , Hipoparatireoidismo/fisiopatologia , Falência Renal Crônica/fisiopatologia , Falência Renal Crônica/terapia , Vértebras Lombares/fisiopatologia , Masculino , Pessoa de Meia-Idade , Osteoporose/etiologia , Osteoporose/fisiopatologia , Estudos Prospectivos , Rádio (Anatomia)/fisiopatologia , Teriparatida/efeitos adversos , Teriparatida/uso terapêuticoRESUMO
Helicobacter cinaedi can cause bacteremia mainly in immunocompromised patients. We present the clinical characteristics of H. cinaedi bacteremia in 4 renal transplant patients. Interestingly, all cases showed triggers of bacterial translocation: 2 cases developed after colonic perforation caused by diverticulitis, 1 case developed post cholecystectomy, and the remaining patient had chronic diarrhea. Accordingly, bacterial translocation caused by severe gastrointestinal complication could be a cause of H. cinaedi bacteremia.
Assuntos
Infecções por Helicobacter/tratamento farmacológico , Helicobacter/isolamento & purificação , Transplante de Rim/efeitos adversos , Idoso , Bacteriemia , Feminino , Infecções por Helicobacter/microbiologia , Humanos , Hospedeiro Imunocomprometido , Masculino , Pessoa de Meia-IdadeRESUMO
A bone biopsy specimen in a long-term hemodialysis patient with sarcoidosis coexisting with severe hypoparathyroidism has demonstrated that a persistent near physiological level of 1,25-dihydroxyvitamin D3 contributes to the preservation of bone remodeling and has the potential to retard the development of vascular calcification and atherosclerosis. Sarcoidosis-related hypercalcemia and hypoparathyroidism, which is characterized by 1,25-dihydroxyvitamin D3 (1,25(OH)2D3) overproduction, is rarely seen in hemodialysis patients. Herein, we describe a 60-year-old Japanese woman on hemodialysis for 35 years who presented with malaise and hypercalcemia. Severe hypoparathyroidism without parathyroidectomy and a preserved 1,25(OH)2D3 level were detected. Computed tomography showed bilateral axillary lymphadenopathy and minimal aortic and soft tissue calcification. The axillary node biopsy led to a definite diagnosis of sarcoidosis. A bone biopsy specimen obtained from the right iliac crest showed remodeling of normal lamellar bone with scalloped cement lines and clear double labeling by tetracycline on fluorescence microscopy. Histomorphometric analysis revealed that the bone formation rate was preserved (30.0 %/year), together with a decrease of osteoid volume (5.75 %) and fibrous volume (0 %), indicating that the patient did not have adynamic bone disease and only showed mild disease. This is the first documented case of sarcoidosis-related hypercalcemia associated with severe hypoparathyroidism in a long-term hemodialysis patient who underwent bone histomorphometry. Our findings suggest that, in hemodialysis patients with sarcoidosis coexisting with severe hypoparathyroidism, a persistent near physiological level of 1,25(OH)2D3 contributes to the preservation of bone remodeling and has the potential to retard the development of vascular calcification and atherosclerosis.
Assuntos
Osso e Ossos/patologia , Hipoparatireoidismo/etiologia , Diálise Renal/efeitos adversos , Sarcoidose/complicações , Remodelação Óssea/fisiologia , Feminino , Humanos , Hipercalcemia/etiologia , Pessoa de Meia-Idade , Vitamina D/análogos & derivados , Vitamina D/sangueRESUMO
AIMS: To investigate the relationship between the progression of anaemia and renal pathological findings in patients with diabetic nephropathy. METHODS: A total of 223 patients with diabetes underwent renal biopsy from 1985 to 2010 and were confirmed to have pure diabetic nephropathy according to the recent classification, of whom 113 (baseline haemoglobin ≥ 11 g/dl) were enrolled in the study. Linear regression analysis was used to estimate the changes in haemoglobin levels during the follow-up period. RESULTS: In a multivariate model adjusted for clinical and histopathological variables, higher interstitial fibrosis and tubular atrophy scores were more strongly associated with a decrease in haemoglobin levels than were lower scores. Compared with an interstitial fibrosis and tubular atrophy score of 0, the standardized coefficients for interstitial fibrosis and tubular atrophy scores of 1, 2 and 3 were 0.20 (95% CI -0.31 to 0.93), 0.34 (95% CI -0.22 to 1.34) and 0.47 (95% CI 0.07 to 1.96), respectively, whereas a higher glomerular class, a higher vascular lesion score and the presence of exudative lesions were not strongly correlated with the decrease in haemoglobin. CONCLUSIONS: Tubulointerstitial lesions that are more advanced are significantly associated with the progression of anaemia in patients with diabetic nephropathy after adjustment for numerous covariates. This finding suggests that tubulointerstitial lesions may be a useful prognostic indicator for anaemia in patients with diabetic nephropathy, and that decreased erythropoietin production attributable to the progression of tubulointerstitial lesions is a major cause of anaemia in these patients.
Assuntos
Anemia/patologia , Diabetes Mellitus Tipo 1/patologia , Diabetes Mellitus Tipo 2/patologia , Nefropatias Diabéticas/patologia , Rim/patologia , Atrofia/patologia , Biópsia , Diabetes Mellitus Tipo 1/fisiopatologia , Diabetes Mellitus Tipo 2/fisiopatologia , Nefropatias Diabéticas/fisiopatologia , Progressão da Doença , Feminino , Fibrose , Taxa de Filtração Glomerular/fisiologia , Humanos , Masculino , Pessoa de Meia-Idade , Estudos RetrospectivosRESUMO
Cyst infection is a frequent and serious complication of autosomal dominant polycystic kidney disease (ADPKD). Lipid-soluble antibiotics like fluoroquinolones show good penetration into cysts and are recommended for cyst infection, but causative microorganisms are often resistant to these agents. This study investigated the profile of the microorganisms causing cyst infection in ADPKD, their susceptibility to lipid-soluble antibiotics, and clinical outcomes. This retrospective study reviewed all ADPKD patients admitted to Toranomon Hospital with a diagnosis of cyst infection from January 2004 to March 2014. All patients who underwent cyst drainage and had positive cyst fluid cultures were enrolled. Patients with positive blood cultures who satisfied our criteria for cyst infection or probable infection were also enrolled. There were 99 episodes with positive cyst fluid cultures and 93 episodes with positive blood cultures. The majority of patients were on dialysis. The death rate was high when infection was caused by multiple microorganisms or when there were multiple infected cysts. Gram-negative bacteria accounted for 74-79 % of the isolates in all groups, except for patients with positive hepatic cyst fluid cultures. The susceptibility of Escherichia coli to fluoroquinolones was very low in patients with hepatic cyst infection, especially those with frequent episodes and those with hepatomegaly. Fungi were detected in two episodes. Fluoroquinolone-resistant microorganisms showed a high prevalence in cyst infection. It is important to identify causative microorganisms to avoid the overuse of fluoroquinolones and to improve the outcome of cyst infection in ADPKD.
Assuntos
Infecções/etiologia , Rim Policístico Autossômico Dominante/complicações , Idoso , Antibacterianos/farmacologia , Antibacterianos/uso terapêutico , Resistência Microbiana a Medicamentos , Feminino , Bactérias Gram-Negativas/isolamento & purificação , Humanos , Infecções/diagnóstico , Infecções/tratamento farmacológico , Infecções/microbiologia , Infecções/cirurgia , Testes de Função Renal , Masculino , Testes de Sensibilidade Microbiana , Pessoa de Meia-Idade , Rim Policístico Autossômico Dominante/fisiopatologia , Rim Policístico Autossômico Dominante/terapiaRESUMO
We trace the 34-year history of a member of the first Japanese family in which lecithin-cholesterol acyltransferase (LCAT) deficiency was diagnosed. Marriage between cousins with low LCAT activity was responsible for familial LCAT deficiency (FLD). In 1976, a 27-year-old Japanese man was noted to have FLD based on proteinuria, hematuria, grayish corneal opacity and low LCAT activity (9.83%). Genetic analysis showed insertion of G-G-C coding glycine at codon 141. Total cholesterol (C) was low at 108 mg/dl and the ratio of C-ester to total C was very low (12%), while the lecithin (phosphatidylcholine) level was very high (97.3%). When his serum creatinine reached 2.6 mg/dl at the age of 41 years (in 1991), renal biopsy was performed. This showed expansion of the mesangial matrix and irregularly thickened capillary walls with a bubble-like appearance because of lipid deposits consisting of two components (partly lucent vacuolated areas and partly deeply osmiophilic areas). Magnification of the latter deposits showed curvilinear and serpiginous striated membranous structure. Hemodialysis was started in 1990 and has been continued for over 20 years until August 2010. Clinical problems have included AV shunt failure requiring 4 operations and 13 percutaneous transcatheter angioplasty procedures, as well as episodes of hemolytic anemia that subsided after infusion of fresh frozen plasma. Cardiovascular events have not yet occurred, although severe calcification of abdominal aorta has been detected by computed tomography.
Assuntos
Deficiência da Lecitina Colesterol Aciltransferase/complicações , Diálise Renal , Adulto , Biópsia , Humanos , Rim/patologia , Lipídeos/sangue , Masculino , Fatores de TempoRESUMO
BACKGROUND: Although hepatitis C virus (HCV) infection is known to be associated with Type 2 cryoglobulinemic glomerulopathy (CG), only a few reports about other types of nephropathy have been published. METHODS: 68 HCV antibody positive patients in whom renal biopsy had been performed for persistent proteinuria, hematuria, and/or renal dysfunction between 1992 and 2008 at our institute were included. The histological, clinical and laboratory characteristics including the age, gender, hypertension, diabetes mellitus, liver histology (chronic hepatitis or liver cirrhosis), HCV-RNA, HCV genotype, splenomegaly, gastroesophageal varices, serum creatinine, hemoglobin, platelet count, rheumatoid factor, cryoglobulin, IgG, IgA, IgM, CH50, C3, C4, creatinine clearance, 24-h protein excretion, and hematuria, between their nephropathy with and without immune deposition were compared. RESULTS: Nephropathy was classified into two groups based on the detection of immune deposits by immunofluorescence microscopy: i.e., a positive group (n = 39) and a negative group (n = 29). The former group was further classified into three types of nephropathy: IgG dominant group (n = 10) (including membranous nephropathy (MN)), IgA dominant group (n = 20) (including IgA nephropathy (IgAN)), membranoproliferative glomerulonephritis (MPGN) (IgA type)), and IgM dominant group (n = 9) (MPGN apart from the IgA type). The latter group included diabetic nephropathy (n = 13), focal glomerular sclerosis (n = 4), and benign nephrosclerosis (n = 3), malignant nephrosclerosis (n = 1), tubulointerstitial nephritis (TIN) (n = 2), minimal change nephrotic syndrome (n = 1), cast nephropathy (n = 1), granulomatous TIN (n = 1), and others (n = 3). An increased serum IgM level, hypocomplementemia, splenomegaly, thrombocytopenia, liver cirrhosis, hematuria, and a high HCV RNA level were features of patients with MPGN of IgM dominant group (consistent with "CG"). CONCLUSIONS: Our results showed various histological patterns of HCV-related kidney disease and the specificity of CG, and revealed that a minority of HCV patients (n = 7) presented typical CG, while IgAN, MN, and diabetic nephropathy were more frequent.
Assuntos
Crioglobulinemia/patologia , Hepatite C/complicações , Nefropatias/patologia , Adulto , Idoso , Biópsia , Distribuição de Qui-Quadrado , Proteínas do Sistema Complemento/análise , Crioglobulinemia/imunologia , Crioglobulinemia/virologia , Nefropatias Diabéticas/patologia , Nefropatias Diabéticas/virologia , Feminino , Glomerulonefrite por IGA/patologia , Glomerulonefrite por IGA/virologia , Glomerulonefrite Membranoproliferativa/patologia , Glomerulonefrite Membranoproliferativa/virologia , Glomerulosclerose Segmentar e Focal/patologia , Glomerulosclerose Segmentar e Focal/virologia , Hematúria/patologia , Hematúria/virologia , Hepacivirus/genética , Hepacivirus/imunologia , Hepatite C/diagnóstico , Anticorpos Anti-Hepatite C/sangue , Humanos , Japão , Nefropatias/classificação , Nefropatias/imunologia , Nefropatias/terapia , Nefropatias/virologia , Masculino , Microscopia de Fluorescência , Pessoa de Meia-Idade , Nefrite Intersticial/patologia , Nefrite Intersticial/virologia , Nefrose Lipoide/patologia , Nefrose Lipoide/virologia , Valor Preditivo dos Testes , Proteinúria/patologia , Proteinúria/virologia , RNA Viral/sangue , Diálise Renal , Estudos Retrospectivos , Resultado do TratamentoRESUMO
BACKGROUND: Septic shock is associated with vasopressin deficiency and hypersensitivity to its exogenous administration. The aim of this study is to review the 28-day survival rate, hemodynamic and renal effects of vasopressin therapy in refractory septic shock Japanese patients. METHODS: 55 Japanese patients experiencing catecholamine-resistant septic shock were treated with vasopressin. Hemodynamic alterations and the serum concentrations of aspartate aminotransferase, total bilirubin and creatinine clearance were evaluated following vasopressin treatment. RESULTS: In both, survivors and non-surviving patients, treatment with vasopressin resulted in a significantly increase in mean arterial pressure, hourly urine output, and a significant decrease in heart rate and total pressor dosage requirements. Creatinine clearance was significantly increased only in survivors. There were no significant changes in the serum concentrations of aspartate aminotransferase and total bilirubin. The 28-day survival rate was 45% (25 patients). CONCLUSIONS: In Japanese septic shock patients, vasopressin infusion improved hemodynamic status and reduced catecholamine requirement, and 28-day survival rate was 45%.
Assuntos
Dopamina/farmacologia , Resistência a Medicamentos , Infecções por Bactérias Gram-Negativas/mortalidade , Infecções por Bactérias Gram-Positivas/mortalidade , Norepinefrina/farmacologia , Choque Séptico/mortalidade , Vasopressinas/administração & dosagem , Pressão Sanguínea/efeitos dos fármacos , Pressão Sanguínea/fisiologia , Cardiotônicos/farmacologia , Relação Dose-Resposta a Droga , Quimioterapia Combinada , Seguimentos , Infecções por Bactérias Gram-Negativas/tratamento farmacológico , Infecções por Bactérias Gram-Negativas/fisiopatologia , Infecções por Bactérias Gram-Positivas/tratamento farmacológico , Infecções por Bactérias Gram-Positivas/fisiopatologia , Humanos , Infusões Intravenosas , Japão/epidemiologia , Pessoa de Meia-Idade , Estudos Prospectivos , Choque Séptico/tratamento farmacológico , Choque Séptico/fisiopatologia , Taxa de Sobrevida/tendências , Fatores de Tempo , Resultado do Tratamento , Vasoconstritores/administração & dosagemRESUMO
Because pregnancy is rare in women with end-stage renal disease, dialysis patients have not been reported to present with acute abdominal symptoms related to pregnancy including ectopic pregnancy. A 41-year-old woman treated with hemodialysis for over 18 years was brought to the emergency room at our institution because of acute abdominal pain. Ultrasonography detected an abdominal fluid collection, and her anemia had worsened (hematocrit 18%). Emergency laparoscopic exploration disclosed a hemorrhagic corpus luteum of pregnancy, causing ovarian bleeding on the left. Coagulation of bleeding points was carried out. At this time, pregnancy at 7 weeks of gestation was discovered. After the procedures, hemodialysis frequency was increased to 5 times weekly, and an erythropoietin derivative was administered to maintain a hematocrit above 30%. The patient developed no hypertension. At 33 weeks of gestation, cesarean section was performed because of a decrease in amniotic fluid and frequent late deceleration of the fetal heart rate. A live baby girl weighing 1,422 g was born. The successful pregnancy reflects remarkable progress in dialysis technology. Pregnancy, then, can underlie an acute abdomen in childbearing-age women (14 - 44 years old) undergoing long-term dialysis.
Assuntos
Abdome Agudo/etiologia , Corpo Lúteo , Hemorragia/complicações , Diálise Renal , Abdome Agudo/diagnóstico , Abdome Agudo/cirurgia , Adulto , Cesárea , Diagnóstico Diferencial , Endossonografia , Feminino , Hemorragia/diagnóstico , Hemorragia/cirurgia , Humanos , Falência Renal Crônica/terapia , Laparoscopia , Gravidez , Resultado da Gravidez , Tomografia Computadorizada por Raios XRESUMO
Multicentric Castleman disease is a systemic lymphoproliferative disease with incomplete understood etiology. The various renal complications of this disease may include minimal change disease, mesangial proliferative glomerulonephritis, membranous glomerulonephritis and nephrotic syndrome, caused by secondary amyloidosis. In several reported cases of localized Castleman disease associated with renal amyloidosis and nephrotic syndrome, resection of organs involved by lymphoid proliferation resulted in complete remission. However, therapy of multicentric Castleman disease with renal amyloidosis is not well-established. We treated a case of a 39-year-old woman with multicentric Castleman disease complicated by nephrotic syndrome caused by secondary AA amyloidosis. The patient underwent autologous peripheral blood stem cell transplantation (auto-PBSCT), achieving complete remission. Autologous stem cell transplantation may be an attractive choice in therapy for refractory multicentric Castleman disease.
Assuntos
Amiloidose/etiologia , Hiperplasia do Linfonodo Gigante/complicações , Hiperplasia do Linfonodo Gigante/terapia , Falência Renal Crônica/etiologia , Síndrome Nefrótica/etiologia , Adulto , Amiloidose/terapia , Feminino , Humanos , Falência Renal Crônica/terapia , Melfalan/administração & dosagem , Agonistas Mieloablativos/administração & dosagem , Síndrome Nefrótica/terapia , Transplante de Células-Tronco de Sangue PeriféricoRESUMO
Antineutrophil cytoplasmic antibody-(ANCA) associated glomerulonephritis usually shows histopathologic features of pauciimmune crescentic glomerulonephritis and occurs late in life. We report a 14-year-old Japanese girl presenting with proteinuria, hematuria and mildly elevated serum creatinine. A renal biopsy specimen demonstrated crescentic glomerulonephritis, immunofluorescence showed mesangial IgA staining. Electron microscopic examination disclosed paramesangial deposits. Serum ANCA against myeloperoxidase (MPO) were detected at high titers. Myeloperoxidase-ANCA-related nephritis accompanied by IgA nephropathy is considered rare in childhood and teen years. Yet, if ANCA assays and detailed electron microscopic examination of renal specimens were performed routinely in patients with rapidly progressive glomerulonephritis, the diagnosis might be more frequent in young patients.
Assuntos
Anticorpos Anticitoplasma de Neutrófilos , Mesângio Glomerular , Glomerulonefrite/imunologia , Glomerulonefrite/patologia , Imunoglobulina A , Adolescente , Feminino , Mesângio Glomerular/química , Mesângio Glomerular/patologia , Humanos , Imunoglobulina A/análiseAssuntos
Injúria Renal Aguda/diagnóstico por imagem , Colangite/diagnóstico por imagem , Colestase/diagnóstico por imagem , Rabdomiólise/diagnóstico por imagem , Doença Aguda , Injúria Renal Aguda/etiologia , Idoso , Colangite/complicações , Colestase/complicações , Humanos , Masculino , Radiografia , Rabdomiólise/etiologiaRESUMO
Colovesical fistula is a relatively rare condition that is primarily related to diverticular disease. There are few reports of colovesical fistula after renal transplantation. We report of a 53-year-old man who was diagnosed with colovesical fistula after recurrent urinary tract infection, 5 months after undergoing cadaveric renal transplantation. Laparoscopic partial resection of the sigmoid colon with the use of the Hartmann procedure was performed. Six months after that surgery, there was no evidence of recurrent urinary tract infection and the patient's renal graft function was preserved. Physicians should keep colovesical fistula in mind as a cause of recurrent urinary tract infection in renal transplant recipients, especially in those with a history of diverticular disease.
Assuntos
Fístula Intestinal/diagnóstico , Transplante de Rim/efeitos adversos , Laparoscopia/efeitos adversos , Insuficiência Renal/cirurgia , Infecções Urinárias/complicações , Biópsia , Colo Sigmoide/cirurgia , Humanos , Fístula Intestinal/diagnóstico por imagem , Rim/cirurgia , Masculino , Pessoa de Meia-Idade , Complicações Pós-Operatórias , Recidiva , Insuficiência Renal/complicações , Tomografia Computadorizada por Raios X , TransplantadosRESUMO
Many patients with type II mixed cryoglobulinemia have been shown to be infected with hapatitis C virus (HCV). Therefore, interferon-alfa has become the first choice of treatment for patients with HCV-associated cryoglobulinemia. However, the disease often relapses after the discontinuation of interferon therapy. The long-term effect of interferon therapy is controversial. Therefore, a more effective therapy needs to be developed. A 62-year-old Japanese woman was admitted to our hospital for the examination of abnormal liver function tests, severe edema, and purpura in her lower extremities. Glomerulopathy secondary to HCV-related cryoglobulinemia was suspected. Her serum creatinine was increased to 2.1 mg/dL. Interferon therapy was considered initially. However, because of pancytopenia caused by liver cirrhosis and splenomegaly, splenectomy was performed in February 1997, before the start of interferon therapy. Renal biopsy specimen taken at the time of the splenectomy showed typical cryoglobulinemic glomerulonephritis. Gradually, after surgery, the patient's thrombocytopenia and anemia improved, her proteinuria and hematuria were decreased, her cryocrit dropped from 15% to 5%, the Ccr increased from 21.1 mL/min to 48.8 mL/min, and the purpura in her lower extremities disappeared. A repeat renal biopsy performed in May 1998 showed marked histological improvement. Splenectomy is not widely accepted as a treatment for cryoglobulinemia. Our case suggests the possibility that the monoclonal-IgM component of the type II cryoglobulin may be formed in the spleen. In conclusion, splenectomy may be an effective therapy for cryoglobulinemia in patients with HCV-positive liver cirrhosis and pancytopenia secondary to splenomegaly.
Assuntos
Crioglobulinemia/classificação , Glomerulonefrite/terapia , Esplenectomia , Anemia/terapia , Anticorpos Monoclonais/imunologia , Biópsia , Creatinina/sangue , Crioglobulinemia/virologia , Crioglobulinas/análise , Feminino , Glomerulonefrite/etiologia , Hematúria/terapia , Hepatite C/complicações , Humanos , Imunoglobulina M/imunologia , Cirrose Hepática/etiologia , Pessoa de Meia-Idade , Pancitopenia/etiologia , Proteinúria/terapia , Baço/imunologia , Esplenomegalia/etiologia , Trombocitopenia/terapiaRESUMO
We report autopsy findings of a 69-year-old man on long-term CAPD therapy for 13 years who showed linear peritoneal calcification. Continuous ambulatory peritoneal dialysis (CAPD) was started in 1982. He has been administered excessive amounts of vitamin D(3) derivatives (VitD) (2.0 to 2.5 microg daily) and calcium carbonate (4 g daily) for secondary hyperparathyroidism since initiation of CAPD. In May 1995, his intact parathyroid hormone (PTH) level increased over 1,000 pg/mL. Immediately after VitD was changed from pill to liquid, the dose was increased to 5 microg daily. Although the serum calcium level remained between 4.5 and 4.9 mEq/L, and serum phosphate level was 5.0 to 7.2 mg/dL, plain abdominal radiography and computed tomography showed continuous calcification along the intestinal wall in October 1995. In spite of the continuation of CAPD therapy, he remained asymptomatic until he died of congestive heart failure in January 1997. He experienced eight episodes of peritonitis during his clinical course. Autopsy showed that numerous calcified plaques were present on the submucosal portion between the thickened serosa and the longitudinal layer of the muscularis externa. The remainder of the subserosa was fibrotic, and the small arteries had markedly thickened intima and severely narrowed lumina.
Assuntos
Músculos Abdominais/patologia , Calcinose/etiologia , Diálise Peritoneal Ambulatorial Contínua/efeitos adversos , Músculos Abdominais/diagnóstico por imagem , Cálcio/sangue , Carbonato de Cálcio/uso terapêutico , Colecalciferol/uso terapêutico , Humanos , Hiperparatireoidismo Secundário/tratamento farmacológico , Masculino , Pessoa de Meia-Idade , Fosfatos/sangue , Fatores de Tempo , Tomografia Computadorizada por Raios XRESUMO
Little attention has been paid to nephropathies and proteinuria in renovascular hypertension (RVH). Recently there has been a growing interest in the conditions induced by RVH. 10 cases of RVH were diagnosed by angiography and renin sampling from renal veins in the last 6 years in our hospital. The patients were all male and mean age was 64 +/- 8 (SD) years. Data were as follow: protein excretion was 3.8 +/- 2.2 g/day (> or = 3.5 g/day in 8 patients), sBP 202 +/- 24 mmHg, dBP 113 +/- 17 mmHg, serum renin concentration 64 +/- 45 pg/ml, and ipsilateral/contralateral renal vein renin ratio 3.3 +/- 1.0. RVH was treated by nephrectomy in 3 patients, percutaneous transluminal renal angioplasty (PTA) in 2, and angiotensin converting enzyme inhibitors (ACE-I) administration in 8. Biopsies were performed on contralateral kidney in 4 patients. Focal segmental glomerulosclerosis (FGS) was found in 3 patients, and nephrosclerosis in 1, whereas only nephrosclerosis was found in nephrectomized kidneys in all 3 patients. After nephrectomy, PTA and the treatment by ACE-I, not only blood pressure but also proteinuria was markedly reduced. These findings suggest that severe stenosis of the renal artery led to renal ischemia, which activated renin excretion, to cause glomerular hyperfiltration through vasoconstriction of the efferent arterioles in the contralateral kidney. FGS-like lesion thus induced appeared to have caused massive proteinuria.
Assuntos
Glomerulosclerose Segmentar e Focal/etiologia , Hipertensão Renovascular/complicações , Rim/patologia , Proteinúria/etiologia , Idoso , Arteriosclerose/complicações , Biópsia , Pressão Sanguínea , Humanos , Hipertensão Renovascular/etiologia , Masculino , Pessoa de Meia-Idade , Renina/sangueRESUMO
Renal diseases of mixed connective tissue disease (MCTD) are not unusual. Although most of them are SLE-like renal impairment with immune complex deposits, systemic sclerosis- (SSc) like renal impairments with intimal thickening of interlobular arteries or arterioles are also encountered. Several cases of SSc complicated with MPO-ANCA-related necrotizing glomerulonephritis (nGN) are reported. Here we report a case which developed MPO-ANCA-related nGN 16 years after the diagnosis of MCTD. She exhibited pauci-immune focal nGN and significantly high titer of MPO-ANCA. She was successfully treated with prednisolone and cyclophosphamide. We believe this is the first case in which MPO-ANCA-related nGN was demonstrated in a patient with MCTD.
Assuntos
Anticorpos Anticitoplasma de Neutrófilos/imunologia , Glomerulonefrite/complicações , Doença Mista do Tecido Conjuntivo/complicações , Peroxidase/imunologia , Feminino , Glomerulonefrite/imunologia , Glomerulonefrite/patologia , Humanos , Glomérulos Renais/patologia , Pessoa de Meia-Idade , Doença Mista do Tecido Conjuntivo/imunologiaRESUMO
In Japan there is a steady increase in patients who have been on dialysis therapy for more than 10 years. Bone lesions could emerge as a serious problem during this dialysis period. From 1986 to 1993, bone lesions were examined by histomorphometry of the bone tissues (40 biopsis, 17 autopsies) in fifty seven patients who have undergone a long-term hemodialysis treatment (37 males, 20 females) at Toranomon hospital. Mean age of the patients was 56 +/- 11 (SD) years (range; 22 to 74) and mean dialysis period, 14.5 +/- 11 (SD) years (range; 10 to 28). The results were compared with biochemical and endocrinological data. The subjects were classified into osteitis fibrosa group (33%), osteomalacia group (12%), mixed group (7%) and mild group (48%). Intact parathyroid hormone (PTH) and alkaline phosphatase (Al-P) value were significantly higher in osteitis fibrosa and mixed groups, all cases showing intact-PTH values greater than 500 pg/ml (normal value; 20-53), but not in osteomalcia and mild groups. The analysis of 32 cases with intact-PTH higher than 500 pg/ml showed osteitis fibrosa in 59.4% mixed in 12.5%, osteomalacia in 3.1% and mild in 25%. Aluminum was positive in 41% for osteitis fibrosa, 25% for mixed, 60% for osteomalacia and 52% for mild group. In summary, cases having undergone hemodialysis more than 10 years showed higher frequency of osteitis fibrosa when intact-PTH exceeded 500 pg/ml. Al-P values in such cases were high in association with the intact-PTH values. There was no significant correlation between aluminum deposition and respective bone tissue types.
Assuntos
Osso e Ossos/patologia , Osteíte/etiologia , Terapia de Substituição Renal/efeitos adversos , Adulto , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Osteíte/patologia , Hormônio Paratireóideo/sangue , Fatores de TempoRESUMO
Diabetic nephropathy can be regarded mainly as a type of microangiopathy, but is a disease that may also include aspects of macroangiopathy. This is especially true of renal disease in non-insulin dependent diabetes mellitus (NIDDM), which is characterized not only by diabetic glomerulosclerosis, but also by atherosclerosis. We performed morphological studies on the kidney, using computed tomography (CT), focusing on such points as: (1) abdominal aortic calcifications at the level of kidney, (2) calcifications in the renal artery, and (3) wedge-shaped defects on the renal surface. We noted that these findings became more prominent in NIDDM patients during end-stage renal failure than during normal renal function, and were significantly more common in those two NIDDM groups than in age-matched nondiabetic patients without hypertension, hyperlipidemia or gout. NIDDM patients exhibited these features more frequently than IDDM patients.