RESUMO
BACKGROUND: Protein-losing gastroenteropathy (PLGE) is a syndrome with a chief complaint of hypoalbuminemia, which occurs due to plasma protein leakage in the gastrointestinal tract, leading to general edema, ascites, and pleural effusions. CASE PRESENTATION: A 71-year-old woman visited another hospital for evaluation of hypoalbuminemia and systemic edema. She was hospitalized for a close inspection of hypoalbuminemia and was diagnosed with PLGE. Steroid and azathioprine therapy was prescribed; however, hypoalbuminemia did not improve, and the patient's condition worsened due to anasarca. As hospitalization was prolonged, the patient was transferred to our hospital. She was infected with Helicobacter pylori, and we performed H. pylori eradication. Following H. pylori eradication, her edema improved remarkably. CONCLUSION: We present the first case wherein H. pylori eradication successfully improved protein leakage in the lower gastrointestinal tract in a patient diagnosed with PLGE complicated with refractory to immunosuppressant treatment. H. pylori eradication should be considered in patients with PLGE complicated with H. pylori infection, without specific endoscopic finding or refractory to immunosuppressants.
Assuntos
Antibacterianos , Infecções por Helicobacter , Helicobacter pylori , Cirrose Hepática Biliar , Enteropatias Perdedoras de Proteínas , Idoso , Antibacterianos/uso terapêutico , Proteínas Sanguíneas/metabolismo , Feminino , Infecções por Helicobacter/sangue , Infecções por Helicobacter/complicações , Infecções por Helicobacter/diagnóstico , Infecções por Helicobacter/tratamento farmacológico , Helicobacter pylori/isolamento & purificação , Humanos , Imunossupressores/uso terapêutico , Cirrose Hepática Biliar/sangue , Cirrose Hepática Biliar/complicações , Cirrose Hepática Biliar/microbiologia , Enteropatias Perdedoras de Proteínas/sangue , Enteropatias Perdedoras de Proteínas/complicações , Enteropatias Perdedoras de Proteínas/tratamento farmacológico , Enteropatias Perdedoras de Proteínas/microbiologiaRESUMO
The purpose of this study was to determine the serum protein binding of tadalafil in children with protein-losing enteropathy (PLE) and to evaluate the specific binding of the drug to human serum-derived proteins in vitro. Seventeen serum samples from two PLE patients used after biochemical tests were collected, and the unbound fraction of tadalafil was determined by an ultrafiltration method. The serum albumin concentrations observed in patients #1 and #2 were 2.4-4.2 and 2.9-3.5 g/dL, respectively. The ranges of unbound fraction of tadalafil in patients #1 and #2 were 3.9-13 and 5.0-7.0%, respectively. This suggested that serum albumin was at least a binding carrier for tadalafil because the unbound fraction of tadalafil and serum albumin were slightly correlated. The unbound fraction of tadalafil at the total concentration of 300 ng/mL was negatively dependent on the serum albumin concentration (range: 1.0-5.0 g/dL) in vitro. In the presence of albumin, the additive effect of γ-globulin on the unbound fraction of tadalafil was marginal, but the addition of α1-acid glycoprotein to test samples decreased the unbound fraction of the drug. The decrease in the unbound fraction of tadalafil was greater at low albumin levels (2 g/dL). The addition of lipoprotein to test samples also decreased the unbound fraction of tadalafil, suggesting that lipoprotein was also a binding carrier of the drug. These results suggested that the disposition and/or response to tadalafil in PLE patients was altered by the change in protein bindings of the drug.
Assuntos
Proteínas Sanguíneas/metabolismo , Enteropatias Perdedoras de Proteínas/sangue , Tadalafila/metabolismo , Adolescente , Criança , Feminino , Humanos , Ligação Proteica/fisiologia , Enteropatias Perdedoras de Proteínas/diagnóstico , Albumina Sérica/metabolismo , Agentes Urológicos/metabolismoRESUMO
BACKGROUND: Hypovitaminosis D has previously been shown to be prevalent amongst dogs with protein losing enteropathy (PLE). The hypothesis of this study was that Low 25-hydroxyvitamin D (25(OH) D) serum concentrations could be a risk factor for negative outcome in dogs with PLE. Forty-three dogs diagnosed with PLE (2005-2014) and which serum Vitamin D serum concentrations were collected and archived at -80 Degrees C were analyzed. Post-diagnostic communication with referring veterinarians was made to determine outcome of PLE dogss: Dogs which died due to PLE within 4 months after diagnosis (negative outcome group, n = 22) and dogs alive or which died due to another disease at the end point of the study (1 year after diagnosis, good outcome group, n = 21). Serum samples taken at the time of diagnosis were analysed for ionized calcium (iCa) concentrations and serum 25(OH) D concentration. RESULTS: Clinical (CCECAI) scores, age at PLE diagnosis, and iCa concentrations were not significantly different between dog groups. A significantly greater (p < 0.001) number of PLE dogs treated with hydrolyzed or elimination diet alone showed good outcome as compared to the PLE negative outcome group. Median serum 25(OH) D concentration was significantly (p = 0.017) lower in dogs with negative outcome versus PLE dogs with good outcome. Using logistic regression analysis, 25(OH) D serum concentration was shown to be a statistically significant factor for outcome determination. Cox regression analysis yielded a hazard ratio of 0.974 (95% CI 0.949, 0.999) per each one nmol/l increase in serum 25(OH) D concentration. CONCLUSIONS: Low serum 25(OH) D concentration in PLE dogs was significantly associated with poor outcome. Further studies are required to investigate the clinical efficacy of Vitamin D (cholecalciferol) as a potential therapeutic agent for dogs with PLE.
Assuntos
Doenças do Cão/sangue , Enteropatias Perdedoras de Proteínas/veterinária , Deficiência de Vitamina D/veterinária , Animais , Cálcio/sangue , Dieta/veterinária , Doenças do Cão/mortalidade , Cães , Feminino , Masculino , Enteropatias Perdedoras de Proteínas/sangue , Enteropatias Perdedoras de Proteínas/mortalidade , Estudos Retrospectivos , Vitamina D/análogos & derivados , Vitamina D/sangue , Deficiência de Vitamina D/sangueRESUMO
OBJECTIVE: To comprehensively characterize the immunologic characteristics of patients with protein-losing enteropathy (PLE) post-Fontan and compare them with patients without PLE post-Fontan. STUDY DESIGN: Patients with PLE post-Fontan and age-matched controls post-Fontan were prospectively studied with laboratory markers of immune function. Infectious history was obtained by interview and chart review. The groups' demographics, cardiac history, immune characteristics, and infection history were compared using appropriate 2-group statistics. RESULTS: A total of 16 patients enrolled (8 patients with PLE and 8 controls). All patients with PLE had lymphopenia compared with 25% of controls (P = .01). All patients with PLE had markedly depressed CD4 T cell counts (median 58 cells/µL) compared with controls (median 450 cells/µL, P = .0002); CD4% was also low in the PLE group (12.3%) and normal in control (36.9%, P = .004). Both groups had mildly depressed CD8 T cells and normal to slightly elevated natural killer and B-cell subsets. A majority of patients with PLE (62.5%) had negative titers to measles, mumps, and rubella vaccination, compared with no control Fontan with a negative titer (P = .03). Despite profoundly low CD4 counts, the frequency of infection was not different between groups with no reported opportunistic infections. CONCLUSIONS: Patients with Fontan-associated PLE have extensive quantitative immune abnormalities, particularly CD4 deficiency. These immune abnormalities are similar to those found in non-Fontan patients with PLE caused by intestinal lymphangiectasia.
Assuntos
Linfócitos T CD4-Positivos , Linfócitos T CD8-Positivos , Técnica de Fontan/efeitos adversos , Cardiopatias Congênitas/cirurgia , Linfopenia/epidemiologia , Enteropatias Perdedoras de Proteínas/imunologia , Contagem de Linfócito CD4 , Estudos de Casos e Controles , Criança , Pré-Escolar , Feminino , Cardiopatias Congênitas/sangue , Cardiopatias Congênitas/imunologia , Humanos , Isotipos de Imunoglobulinas/sangue , Lactente , Masculino , Estudos Prospectivos , Enteropatias Perdedoras de Proteínas/sangueRESUMO
BACKGROUND/AIMS: We classified intestinal lymphangiectasia (IL) into two categories, the white and non-white villi types, and evaluated their clinical characteristics and therapeutic responses. METHODS: Of the 988 patients who underwent double-balloon enteroscopy, 14 consecutive patients (7 men and 7 women, median age at onset 34 years) were enrolled with immunohistochemically confirmed IL with protein-losing enteropathy. RESULTS: Enteroscopically the white villi type (n = 8) showed white plaques and white-tipped villi were scattered in the small bowel, while non-white villi type (n = 6) showed that apparently normal but under more detailed observation, low and round villi with a normal color were diffused. The serum albumin levels and fecal α1-antitrypsin clearance before treatment were significantly worse in the non-white villi type (p = 0.017 and 0.039, respectively), whereas the serum immunoglobulin A and M levels were significantly lower in the white villi type (p = 0.010 and 0.046, respectively). At gastroscopy, a non-cirrhotic snakeskin appearance was significantly observed in the non-white villi type (p = 0.015). The corticosteroid response was better in the non-white villi type (p = 0.015). CONCLUSION: Two distinct subgroups were found in IL. This classification was useful in pathophysiological clustering and in predicting the therapeutic response.
Assuntos
Duodenopatias/patologia , Doenças do Jejuno/patologia , Linfangiectasia Intestinal/patologia , Enteropatias Perdedoras de Proteínas/patologia , Adolescente , Adulto , Idade de Início , Criança , Pré-Escolar , Enteroscopia de Duplo Balão , Duodenopatias/sangue , Duodenopatias/classificação , Duodenopatias/tratamento farmacológico , Duodenopatias/etiologia , Fezes/química , Feminino , Glucocorticoides/uso terapêutico , Humanos , Lactente , Recém-Nascido , Doenças do Jejuno/sangue , Doenças do Jejuno/classificação , Doenças do Jejuno/tratamento farmacológico , Doenças do Jejuno/etiologia , Linfangiectasia Intestinal/sangue , Linfangiectasia Intestinal/classificação , Linfangiectasia Intestinal/complicações , Linfangiectasia Intestinal/tratamento farmacológico , Masculino , Pessoa de Meia-Idade , Prednisolona/uso terapêutico , Prognóstico , Enteropatias Perdedoras de Proteínas/sangue , Enteropatias Perdedoras de Proteínas/tratamento farmacológico , Enteropatias Perdedoras de Proteínas/etiologia , alfa 1-Antitripsina/análiseRESUMO
A 7-year-old male patient who had abdominal swelling and eyelid oedema was diagnosed with restrictive cardiomyopathy. His serum albumin level was 2.3 g/dl. Protein-losing enteropathy due to restrictive cardiomyopathy was diagnosed and oral budesonide was started. His serum albumin level began to rise and ascites and peripheric oedema disappeared. The patient underwent a successful cardiac transplantation and budesonide was stopped. After the heart transplantation, the albumin level decreased to 2.3 g/dl, and therefore it was restarted. When the serum albumin level increased, the budesonide dose was tapered and stopped in 1 month. Budesonide may be an effective drug in patients with protein-losing enteropathy due to heart failure.
Assuntos
Ascite/tratamento farmacológico , Budesonida/administração & dosagem , Cardiomiopatia Restritiva/cirurgia , Edema/tratamento farmacológico , Glucocorticoides/administração & dosagem , Transplante de Coração , Enteropatias Perdedoras de Proteínas/tratamento farmacológico , Administração Oral , Ascite/etiologia , Cardiomiopatia Restritiva/complicações , Criança , Edema/etiologia , Humanos , Masculino , Enteropatias Perdedoras de Proteínas/sangue , Enteropatias Perdedoras de Proteínas/etiologia , Albumina Sérica , Resultado do TratamentoRESUMO
Disseminated mycobacterium avium complex (MAC) causing protein-losing enteropathy (PLE) due to intestinal lymphangiectasia (IL) in a non-HIV immunocompromised state is extremely rare. We present a case of 56-year-old male who was evaluated for worsening dyspnea and found to have right-sided chylous pleural effusion as well as worsening abdominal and retroperitoneal lymphadenopathy. He had a history of psoriasis for which hewas on etanercept and alefacept which were stopped two years prior to the presentation. The evaluation revealed a MAC infection in his lymph nodes--a low CD4 count but negative for HIV. He was started on MAC therapy. He subsequently developed noninfectious diarrhea, Hypoalbuminemia, recurrentpleural effusions, ascites, and Pneumocystis jiroveci pneumonia (PJP). Despite appropriate antibiotics and management--including total parental nutrition (TPN) with a medium-chain triglyceride enriched low fat diet--the patient's clinical condition deteriorated rapidly resulting in death.
Assuntos
Claritromicina/administração & dosagem , Etambutol/administração & dosagem , Linfangiectasia Intestinal , Infecção por Mycobacterium avium-intracellulare , Enteropatias Perdedoras de Proteínas , Rifampina/administração & dosagem , Antibióticos Antituberculose/administração & dosagem , Biópsia , Contagem de Linfócito CD4 , Ascite Quilosa/diagnóstico por imagem , Ascite Quilosa/etiologia , Evolução Fatal , Humanos , Linfangiectasia Intestinal/complicações , Linfangiectasia Intestinal/microbiologia , Linfangiectasia Intestinal/patologia , Masculino , Pessoa de Meia-Idade , Complexo Mycobacterium avium/isolamento & purificação , Infecção por Mycobacterium avium-intracellulare/complicações , Infecção por Mycobacterium avium-intracellulare/diagnóstico , Infecção por Mycobacterium avium-intracellulare/tratamento farmacológico , Infecção por Mycobacterium avium-intracellulare/fisiopatologia , Derrame Pleural/diagnóstico por imagem , Derrame Pleural/etiologia , Enteropatias Perdedoras de Proteínas/sangue , Enteropatias Perdedoras de Proteínas/diagnóstico , Enteropatias Perdedoras de Proteínas/tratamento farmacológico , Enteropatias Perdedoras de Proteínas/etiologia , Enteropatias Perdedoras de Proteínas/fisiopatologia , Albumina Sérica/análise , Tomografia Computadorizada por Raios XRESUMO
Protein-losing enteropathy is a rare manifestation of systemic lupus erythematosus. We report the case of an 18-year-old woman that presented initially with diarrhoea and anasarca. During evaluation, there was low serum albumin of 1.6 g/dl (3.5-5.2 g/dl) and a positive antinuclear antibody test (1:2560). Anti-Sm antibodies (ELISA) were positive in addition to low serum C3 of 35 mg/dl. A scintigraphy using 99mTc-labelled albumin was positive for abdominal protein loss. A diagnosis of systemic lupus erythematosus related protein-losing enteropathy was made. She was started on prednisolone 40 mg/day without amelioration; a month later, azathioprine (100 mg/day) was added, leading to normalization of serum albumin and resolution of symptoms within 4 months. After 1.5 years, the patient developed a 2.9 g 24-h proteinuria while still in remission of the protein-losing enteropathy, receiving 5 mg prednisone and 100 mg azathioprine daily.
Assuntos
Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/diagnóstico , Enteropatias Perdedoras de Proteínas/diagnóstico , Enteropatias Perdedoras de Proteínas/etiologia , Adolescente , Anticorpos Antinucleares/sangue , Azatioprina/uso terapêutico , Biomarcadores/sangue , Complemento C3/análise , Diarreia/etiologia , Quimioterapia Combinada , Edema/etiologia , Ensaio de Imunoadsorção Enzimática , Feminino , Humanos , Hipoalbuminemia/etiologia , Imunossupressores/uso terapêutico , Lúpus Eritematoso Sistêmico/sangue , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Nefrite Lúpica/etiologia , Prednisolona/uso terapêutico , Prednisona/uso terapêutico , Enteropatias Perdedoras de Proteínas/sangue , Enteropatias Perdedoras de Proteínas/tratamento farmacológico , Proteinúria/etiologia , Albumina Sérica/análise , Fatores de Tempo , Resultado do TratamentoRESUMO
OBJECTIVE: The aim of our study was to investigate systemic lupus erythematosus (SLE) related protein-losing enteropathy (PLE) in the following areas: clinical features, laboratory, endoscopic and imaging characteristics, treatment and outcome. METHOD: A retrospective analysis was performed. RESULTS: From 2001 to 2010, 48 patients had SLE related PLE and their clinical characteristics were: age 40.8 ± 14.3 years, male-to-female ratio 1:8.6, mean symptom duration 4.3 ± 3.4 weeks, initial presentation and concomitant activity of SLE in 21(43.8%) and 37 (77.1%) patients, <20% patients developed gastrointestinal (GI) symptoms, mean serum albumin level 24.4 ± 5 g/L. Thirty (62.5%) patients had diffuse non-erosive erythematous GI mucosa with chronic inflammatory cells in lamina propria. Protein leakage was at the small bowel in 15 (31.3%) patients, terminal ileum/caecum in 16 (33.3%) patients and ascending colon in 11 (22.9%) patients. Thirty (62.5%) patients responded initially well to a combination of prednisolone and azathioprine (AZA) and 33 (68.8%) patients were maintained well by the above therapy. Higher potent induction and maintenance therapy were required in patients with: proteinuria (p < 0.01), history of previous immunosuppressive therapy (p < 0.02) and requirement of higher potent induction therapy (p < 0.01). PLE as initial SLE presentation was associated with better prognosis. Four reversible adverse events were reported: one had AZA-induced pancreatitis, two developed AZA-induced hypoplastic anemia and one developed steroid psychosis. One patient developed shingles in the fourth month and responded to oral acyclovir. No thromboembolic events were reported and one patient died of SLE nephropathy. CONCLUSION: There appears to be increasing prevalence of SLE related PLE. A diagnosis can be made using 99m Tc-labeled HSA scintigraphy. PLE generally responds well to immunosuppressive therapy.
Assuntos
Anti-Inflamatórios/uso terapêutico , Azatioprina/uso terapêutico , Imunossupressores/uso terapêutico , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Prednisolona/uso terapêutico , Enteropatias Perdedoras de Proteínas/tratamento farmacológico , Adolescente , Adulto , Idoso , Análise de Variância , Anti-Inflamatórios/efeitos adversos , Azatioprina/efeitos adversos , Sedimentação Sanguínea , Proteína C-Reativa , Ceco/patologia , Ceco/fisiopatologia , Distribuição de Qui-Quadrado , Colo Ascendente/patologia , Colo Ascendente/fisiopatologia , Endoscopia Gastrointestinal , Feminino , Hong Kong , Humanos , Imunossupressores/efeitos adversos , Quimioterapia de Indução , Intestino Delgado/patologia , Intestino Delgado/fisiopatologia , Lúpus Eritematoso Sistêmico/sangue , Lúpus Eritematoso Sistêmico/complicações , Quimioterapia de Manutenção , Masculino , Pessoa de Meia-Idade , Prednisolona/efeitos adversos , Enteropatias Perdedoras de Proteínas/sangue , Enteropatias Perdedoras de Proteínas/complicações , Enteropatias Perdedoras de Proteínas/diagnóstico por imagem , Cintilografia , Estudos Retrospectivos , Albumina Sérica/metabolismo , Estatísticas não Paramétricas , Tecnécio , Adulto JovemRESUMO
The purpose of this study was to measure serum hepatocyte growth factor (HGF) and elucidate the relationship between HGF and protein-losing enteropathy (PLE) after Fontan operation (FO). Ten patients with PLE (mean age 15.7 ± 8.7 years) who underwent FO were enrolled. Control group 1 comprised 20 patients without PLE after FO, and control group 2 comprised 10 patients with nephrotic syndrome (NS). Serum HGF, vascular endothelial growth factor, albumin, and random stool alpha-1 antitrypsin concentration were measured. Transthoracic echocardiography was completed. Serum HGF level was significantly greater in the PLE patients (0.61 ± 0.27 ng/ml) after FO than in the two control groups (0.41 ± 0.12 ng/ml [P = 0.024] for the Fontan group without PLE and 0.26 ± 0.12 ng/ml [P = 0.002] for the patients with NS). Serum albumin of patients with PLE (2.82 ± 0.96 g/dl) showed significantly lower levels compared with those of patients without PLE after FO (4.30 ± 0.37 g/dl, P < 0.001) but significantly greater levels compared with patients with NS (1.91 ± 0.33 g/dl, P = 0.019). Patients with greater serum HGF levels showed significant correlation with lower serum albumin level (P = 0.006, r = -0.495). Because serum HGF levels were significantly greater in patients with PLE after FO, HGF may play a role in the development of PLE after FO.
Assuntos
Técnica de Fontan/efeitos adversos , Cardiopatias Congênitas/cirurgia , Fator de Crescimento de Hepatócito/sangue , Enteropatias Perdedoras de Proteínas/sangue , Adolescente , Biomarcadores/sangue , Criança , Ecocardiografia , Feminino , Seguimentos , Cardiopatias Congênitas/sangue , Cardiopatias Congênitas/diagnóstico por imagem , Humanos , Masculino , Período Pós-Operatório , Prognóstico , Enteropatias Perdedoras de Proteínas/etiologia , Estudos RetrospectivosRESUMO
Fontan palliation has improved survival for single ventricle patients, but long-term complications persist including cardiovascular dysfunction, neurohormonal abnormalities, and protein-losing enteropathy (PLE). Although chronic inflammation contributes to morbidity, an association between inflammation and vascular dysfunction has not been studied. We assessed inflammation and vascular function in 31 Fontan-palliated patients (52% male, median age 14.3 years), including 10 PLE+. Fontan circulation was associated with altered inflammatory cytokines (TNF-α: mean 2.5 ± 1.4 vs. 0.7 ± 0.2 pg/ml, p < 0.0001; sTNFR2: 371 ± 108 vs. 2694 ± 884 pg/ml, p < 0.0001) and vascular dysfunction [log-transformed reactive hyperemia index (lnRHI) 0.28 ± 0.19 vs. 0.47 ± 0.26, p < 0.01; augmentation index (AI) -2.9 ± 13.8 vs. -16.3 ± 12.0, pâ¯=â¯0.001; circulating endothelial progenitor cells (cEPCs) 5.0 ± 8.1 vs. 22.8 ± 15.9, pâ¯=â¯0.0002)]. Furthermore, PLE+ patients showed greater inflammation (IFN-γ 6.3 ± 2.2 vs. 11.5 ± 7.9 pg/ml, pâ¯=â¯0.01; sTNFR1: 1181 ± 420 vs. 771 ± 350 pg/ml, pâ¯=â¯0.01) and decreased arterial compliance (AI: 5.4 ± 17.1 vs. -6.8 ± 10.2, pâ¯=â¯0.02) than PLE- patients. Circulating EPCs, but not inflammatory cytokines, were inversely associated with arterial stiffness in Fontan patients. In conclusion, chronic inflammation and vascular dysfunction are observed after Fontan operation, with greater inflammation and arterial stiffness in Fontan patients with active PLE. However, there is no clear association between inflammatory cytokines and vascular dysfunction, suggesting these pathophysiologic processes are not mechanistically linked.
Assuntos
Biomarcadores/sangue , Técnica de Fontan/efeitos adversos , Cardiopatias Congênitas/cirurgia , Complicações Pós-Operatórias/sangue , Enteropatias Perdedoras de Proteínas/sangue , Doenças Vasculares/sangue , Resistência Vascular/fisiologia , Adolescente , Adulto , Criança , Feminino , Seguimentos , Cardiopatias Congênitas/sangue , Humanos , Inflamação/sangue , Inflamação/etiologia , Masculino , Complicações Pós-Operatórias/etiologia , Estudos Prospectivos , Enteropatias Perdedoras de Proteínas/etiologia , Doenças Vasculares/etiologia , Doenças Vasculares/fisiopatologia , Adulto JovemRESUMO
BACKGROUND: Dogs with protein-losing nephropathy (PLN) are treated with antiplatelet drugs for thromboprophylaxis but no standardized method exists to measure drug response. It is also unknown if clopidogrel metabolite concentrations [CM] differ between healthy and PLN dogs. OBJECTIVES: Assess response to aspirin or clopidogrel in PLN dogs using platelet aggregometry (PA) and compare [CM] between healthy and PLN dogs. ANIMALS: Six healthy and 14 PLN dogs. METHODS: Platelet aggregometry using adenosine diphosphate (ADP), arachidonic acid (AA), and saline was performed in healthy dogs at baseline and 1-week postclopidogrel administration to identify responders or nonresponders. A decrease of ≥60% for ADP or ≥30% for AA at 1 or 3 hours postpill was used to define a responder. At 1 and 3 hours postclopidogrel, [CM] and PA were measured in healthy and PLN dogs. Platelet aggregometry was performed in PLN dogs at baseline, 1, 6, and 12 weeks after clopidogrel or aspirin administration. RESULTS: In PLN dogs receiving clopidogrel, PA differed from baseline at all time points for ADP but not for AA at any time point. Most dogs responded at 1 or both time points except for 1 dog that showed no response. For PLN dogs receiving aspirin, no differences from baseline were observed at any time point for either ADP or AA. No differences in [CM] were found at either time point between healthy and PLN dogs. CONCLUSIONS AND CLINICAL IMPORTANCE: Platelet aggregometry may represent an objective method to evaluate response to clopidogrel or aspirin treatment and PLN dogs appear to metabolize clopidogrel similarly to healthy dogs.
Assuntos
Aspirina/uso terapêutico , Clopidogrel/uso terapêutico , Doenças do Cão/tratamento farmacológico , Enteropatias Perdedoras de Proteínas/veterinária , Animais , Estudos de Casos e Controles , Clopidogrel/metabolismo , Cães , Feminino , Masculino , Agregação Plaquetária , Inibidores da Agregação Plaquetária/metabolismo , Inibidores da Agregação Plaquetária/uso terapêutico , Enteropatias Perdedoras de Proteínas/sangue , Enteropatias Perdedoras de Proteínas/patologiaRESUMO
A 24-year-old woman with a medical history of chronic lower extremity oedema, abdominal pain, diarrhoea and recurrent pulmonary infections presented with sepsis from right lower extremity cellulitis. Blood cultures grew Morganella morganii Laboratory evaluation revealed lymphopaenia, hypogammaglobulinaemia, a low CD4+ T-cell count and nutritional deficiencies resulting from protein-losing enteropathy (PLE). CT showed small bowel wall thickening in the jejunum and ileum. Primary intestinal lymphangiectasia (PIL) was the likely diagnosis that explained her PLE and immunodeficiencies. Video capsule endoscopy is an important diagnostic tool for distal small bowel pathology and confirmed patchy areas of lymphangiectasia of the jejunum and ileum. Secondary causes of lymphangiectasia were ruled out. Clinically significant immunodeficiency from PIL has not been frequently documented, and this case adds to the literature of rare infections associated with PIL. Treatment with intravenous antibiotics resolved her septicaemia, while dietary modifications improved her oedema, abdominal pain and diarrhoea.
Assuntos
Agamaglobulinemia/imunologia , Bacteriemia/imunologia , Infecções por Enterobacteriaceae/imunologia , Linfangiectasia Intestinal/diagnóstico , Morganella morganii/isolamento & purificação , Enteropatias Perdedoras de Proteínas/imunologia , Administração Intravenosa , Agamaglobulinemia/sangue , Agamaglobulinemia/diagnóstico , Antibacterianos/administração & dosagem , Bacteriemia/diagnóstico , Bacteriemia/tratamento farmacológico , Bacteriemia/microbiologia , Biópsia , Contagem de Linfócito CD4 , Endoscopia por Cápsula , Infecções por Enterobacteriaceae/diagnóstico , Infecções por Enterobacteriaceae/tratamento farmacológico , Infecções por Enterobacteriaceae/microbiologia , Feminino , Humanos , Íleo/diagnóstico por imagem , Íleo/patologia , Imunoglobulina A/sangue , Imunoglobulina A/imunologia , Imunoglobulina G/sangue , Imunoglobulina G/imunologia , Imunoglobulina M/sangue , Imunoglobulina M/imunologia , Jejuno/diagnóstico por imagem , Jejuno/patologia , Linfangiectasia Intestinal/sangue , Linfangiectasia Intestinal/complicações , Linfangiectasia Intestinal/imunologia , Morganella morganii/imunologia , Enteropatias Perdedoras de Proteínas/sangue , Enteropatias Perdedoras de Proteínas/diagnóstico , Tomografia Computadorizada por Raios X , Adulto JovemAssuntos
Lúpus Eritematoso Sistêmico/complicações , Enteropatias Perdedoras de Proteínas/etiologia , Fator de Crescimento Transformador alfa/sangue , Fator A de Crescimento do Endotélio Vascular/sangue , Adulto , Feminino , Humanos , Lúpus Eritematoso Sistêmico/sangue , Enteropatias Perdedoras de Proteínas/sangue , Enteropatias Perdedoras de Proteínas/fisiopatologiaRESUMO
This retrospective study was designed to evaluate the effectiveness of subcutaneous heparin therapy for the treatment of protein-losing enteropathy (PLE) associated with single-ventricle palliation and to evaluate the side effects of long-term heparin use. PLE affects 4% to 13% of Fontan operative survivors. Five-year survival after onset of PLE is only 46% to 59%. We studied a cohort of patients with single-ventricle palliation who developed PLE and were treated with subcutaneous heparin. Seventeen patients were included in the study. Symptoms of PLE appeared on average 43 months after surgical palliation. At diagnosis of PLE, mean albumin level was 2.0 +/- 0.4 g/dl. At cardiac catheterization, mean systemic venous pressure was 11.6 mm Hg. Subjective symptomatic improvement on heparin therapy occurred in 13 patients (76%). Three patients (18%) went into clinical remission. Compared with the period before initiation of heparin, there was no significant difference in the number of hospital admissions (p = 0.99) or albumin infusions (p = 0.88) during the first year of heparin therapy. Five patients had x-rays of their thoracolumbar spine, and 9 patients had bone mineral analyses; all scans were grossly abnormal. In conclusion, subcutaneous heparin therapy leads to subjective improvement of PLE symptoms in most patients; however, it does not change the need for frequent albumin infusions and does not increase the rate of remission above that for standard medical therapy.
Assuntos
Anticoagulantes/uso terapêutico , Ventrículos do Coração/patologia , Heparina/uso terapêutico , Enteropatias Perdedoras de Proteínas/tratamento farmacológico , Enteropatias Perdedoras de Proteínas/mortalidade , Anticoagulantes/administração & dosagem , Anticoagulantes/efeitos adversos , Criança , Estudos de Coortes , Feminino , Heparina/administração & dosagem , Heparina/efeitos adversos , Humanos , Masculino , Prontuários Médicos , Michigan/epidemiologia , Enteropatias Perdedoras de Proteínas/sangue , Estudos Retrospectivos , Análise de SobrevidaRESUMO
BACKGROUND: Certain amino acids are decreased in humans with inflammatory bowel disease (IBD) and supplementation with the same amino acids has shown beneficial effects in animal models of IBD. Currently, the amino acid status of dogs with protein-losing enteropathy (PLE) is unknown. HYPOTHESIS/OBJECTIVE: To determine if serum amino acid concentrations are abnormal in dogs with PLE and correlated with clinical and laboratory variables and outcome. ANIMALS: Thirty client-owned dogs diagnosed with PLE and 12 apparently healthy dogs seen at Bristol Veterinary School. METHODS: Retrospective study using stored residual serum from fasted dogs with PLE, collected at the time of diagnostic investigation and from apparently healthy dogs. Serum was analyzed for 30 amino acids using an automated high-performance liquid chromatography amino acid analyzer. RESULTS: Serum tryptophan concentrations were significantly decreased in dogs with PLE (median, 22 nmol/mL; range, 1-80 nmol/mL) compared with apparently healthy control dogs (median, 77.5 nmol/mL; range, 42-135 nmol/mL, P < .001). There were no significant differences in the remaining 29 serum amino acids between dogs with PLE and apparently healthy. Serum tryptophan concentrations were also significantly correlated with serum albumin concentrations in dogs with PLE (P = .001, R2 = 0.506). CONCLUSIONS AND CLINICAL IMPORTANCE: Decreased serum tryptophan concentration might play a role in the pathogenesis of canine PLE or be a consequence of the disease.
Assuntos
Aminoácidos/sangue , Doenças do Cão/sangue , Enteropatias Perdedoras de Proteínas/veterinária , Animais , Estudos de Casos e Controles , Cromatografia Líquida de Alta Pressão/veterinária , Cães , Feminino , Masculino , Enteropatias Perdedoras de Proteínas/sangue , Estudos Retrospectivos , Albumina Sérica/análise , Triptofano/sangueRESUMO
RATIONALE: Although systemic lupus erythematosus (SLE) can be complicated by various gastrointestinal tract diseases, it is rarely associated with lupus enteritis and protein-losing enteropathy (PLE). We report here the successful surgical treatment of lupus enteritis and therapy-resistant and refractory PLE in a patient with SLE. We also provide a review of relevant literature. PATIENT CONCERNS: A 16-year-old girl presenting with polyarthritis, malar rash, and palmar erythema was indicated for steroid therapy on the basis of positive results for antinuclear, anti-Smith, and antiphospholipid antibodies, which confirmed the diagnosis of SLE. During the course of steroid therapy, the patient developed acute abdomen and hypoalbuminemia. DIAGNOSES: Computed tomography and Tc-labeled human serum albumin scintigraphy revealed abnormal findings, and a diagnosis of lupus enteritis and PLE was made. Steroid treatment was continued but no significant improvement was observed, and the patient was referred and admitted to our hospital. Double-balloon enteroscopy revealed multiple ischemic stenoses and mucosal necroses in the small intestine, suggesting that PLE was associated with ischemic enteritis due to antiphospholipid syndrome. The patient received steroids, immunosuppressive drugs, and antithrombotic therapy, with no improvement in symptoms. Thus, the disease was judged to be refractory and resistant to medical therapy, and the patient was indicated for surgical treatment. INTERVENTIONS: Partial small intestinal resection was performed by removing the segment of the small intestine presenting PLE lesions, and a double-end ileostomy was created. OUTCOMES: Multiple stenotic lesions were confirmed in the resected segment. Histopathology evaluation revealed marked inflammatory cell infiltration in the intestinal tract wall and recanalization of the vessels, suggesting a circulatory disorder caused by vasculitis and antiphospholipid syndrome. Postoperatively, the clinical course was good. Serum albumin levels and body weight increased as nutritional status improved significantly. Secondary enteroenterostomy with ileostomy closure could be performed at 2 months after the initial surgery. LESSONS: Timely surgical treatment can be successful in managing therapy-resistant and refractory PLE in patients with SLE.
Assuntos
Procedimentos Cirúrgicos do Sistema Digestório/métodos , Hipoalbuminemia , Intestino Delgado/cirurgia , Lúpus Eritematoso Sistêmico , Enteropatias Perdedoras de Proteínas/cirurgia , Adolescente , Dissecação/métodos , Feminino , Humanos , Hipoalbuminemia/diagnóstico , Hipoalbuminemia/etiologia , Testes Imunológicos/métodos , Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/imunologia , Masculino , Enteropatias Perdedoras de Proteínas/sangue , Enteropatias Perdedoras de Proteínas/diagnóstico , Enteropatias Perdedoras de Proteínas/etiologia , Cintilografia/métodos , Albumina Sérica/análise , Resultado do TratamentoRESUMO
Intestinal lymphangiectasia is a disease characterized by hypoproteinemia and edema resulting from protein-losing gastroenteropathy secondary to abnormal intestinal lymphatics. Immunologic abnormalities associated with this disease include hypogammaglobulinemia, lymphocytopenia, skin anergy, and impaired allograft rejection. In the present study, the in vitro blastogenic transformation of lymphocytes from 12 patients with intestinal lymphangiectasia was assessed in order to gain insight into the mechanism of the cellular immune defect in this disease. Peripheral blood lymphocytes from patients with intestinal lymphagiectasia showed impaired in vitro transformation to nonspecific mitogens, specific antigens, and allogeneic cells when compared to equal numbers of cells from normal individuals. Patients with the most deficient in vitro reactivity tended to have the lowest serum albumin concentration and the lowest absolute lymphocyte count. Lymphocytes obtained from chylous effusions in each of the four patients studied transformed more vigorously than peripheral blood cells from the same patients. These results may be explained by the loss of recirculating, long-lived lymphocytes into the gastrointestinal tract, resulting in a relative depletion of the population of lymphocytes necessary for in vitro blast transformation. This disease thus represents a clinical analogue of animals with experimental thoracic duct drainage, and provides evidence for the existence, in man, of two functionally distinct lymphocyte populations. In addition, these findings establish a new mechanism of impaired delayed hypersensitivity and defective in vitro lymphocyte transformation, i.e. the gastrointestinal loss and consequent depletion of the long-lived, recirculating population of lymphocytes from the peripheral lymphocyte pool.
Assuntos
Ativação Linfocitária , Linfócitos , Enteropatias Perdedoras de Proteínas/imunologia , Adolescente , Adulto , Idoso , Candida albicans , Células Cultivadas , Criança , Quilo , Toxoide Diftérico , Meia-Vida , Humanos , Hipersensibilidade Tardia/etiologia , Técnicas In Vitro , Lectinas , Pessoa de Meia-Idade , Enteropatias Perdedoras de Proteínas/sangue , Enteropatias Perdedoras de Proteínas/fisiopatologia , Toxoide Estafilocócico , Estimulação Química , Estreptodornase e Estreptoquinase , Estreptolisinas , Toxoide TetânicoRESUMO
The guanido carbon of hepatic arginine is the common precursor of urea and of the arginine of plasma proteins synthesized in the liver. It is possible to measure the momentary synthetic rates of plasma proteins by "pulse labeling" this arginine pool with bicarbonate-(14)C. In the current study, this method has been adapted in order to use urinary urea data and was applied to control subjects and patients with gastrointestinal protein loss. The assumptions required for this determination are discussed. There was close agreement between albumin synthetic rates measured by this method and albumin catabolic rates derived from simultaneous albumin-(131)I studies, supporting the validity of the method and suggesting that there is relatively little fluctuation in the rate of albumin synthesis from time to time. The albumin synthetic rates in six control subjects averaged 5.8 mg/kg per hr, while those of five patients with gastrointestinal protein loss averaged 7.2 mg/kg per hr. Thus in these patients, there was relatively little acceleration of albumin synthesis in response to continued loss of plasma proteins into the gastrointestinal tract. Fibrinogen synthetic rates averaged 1.9 mg/kg per hr in five control subjects and 3.2 mg/kg per hr in five patients with gastrointestinal protein loss. Transferrin synthetic rates exhibited considerable individual variation in both groups and averaged 0.24 mg/kg per hr in four control subjects and 0.31 mg/kg per hr in five patients with gastrointestinal protein loss. The method employed in this study offers several advantages in studying plasma protein metabolism. It provides a direct measurement of protein synthesis, applicable to several proteins simultaneously, does not require a long-term steady state in the metabolism of the proteins, and is capable of measuring short-term fluctuations in synthetic rates. Therefore, this approach is applicable to the investigation of the physiological factors controlling the rates of synthesis for plasma proteins.
Assuntos
Proteínas Sanguíneas/biossíntese , Enteropatias Perdedoras de Proteínas/sangue , Transferrina/análise , Ureia/urina , Esclerose Lateral Amiotrófica/sangue , Arginina , Bicarbonatos , Isótopos de Carbono , Doença de Crohn/sangue , Fibrinogênio/análise , Humanos , Leucemia Linfoide/sangue , Leucemia Mieloide/sangue , Soroalbumina RadioiodadaRESUMO
OBJECTIVES: To describe the clinical outcome of dietary management of Yorkshire terriers with protein-losing enteropathy without immunosuppressive/anti-inflammatory medications. METHODS: Records were searched for Yorkshire terriers with hypoalbuminaemia and a clinical diagnosis of protein-losing enteropathy that were managed with diet and without immunosuppressive/anti-inflammatory medications. Serum albumin changes were compared using a one-way repeated measures ANOVA. Canine chronic enteropathy clinical activity index scores were compared using a Wilcoxon signed-rank test. RESULTS: Eleven cases were identified. Clinical signs were variable including: diarrhoea, respiratory signs, vomiting, lethargy and weight loss. Diets fed included home cooked (n=5); Royal Canin Gastrointestinal Low Fat (n=4); Hill's Prescription Diet i/d Low Fat (n=1); or Purina HA Hypoallergenic (n=1). Clinical signs resolved completely in eight dogs, partially resolved in two dogs and failed to respond in one dog. In dogs that responded, albumin significantly improved from baseline (mean 14·9 g/L, sd ±3·7), at 2 to 4 weeks (mean 24·2 g/L, sd ±5·5, P=0·01), and at 3 to 4 months (mean 27·0 g/dL, sd ±5·9, P=0·01). CLINICAL SIGNIFICANCE: These results indicate that dietary management of protein-losing enteropathy is a potential management strategy in Yorkshire terriers. Randomised clinical trials in Yorkshire terriers with protein-losing enteropathy are necessary to compare success rate, survival and quality of life with dietary management versus combined dietary and immunosuppressive/anti-inflammatory therapy.