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2.
Pan Afr Med J ; 33: 262, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31692740

RESUMO

Hyperbilirubinemia is one of the most widely seen cause of neonatal morbidity. Besides ABO and Rh isoimmunization, minor blood incompatibilities have been also been identified as the other causes of severe newborn jaundice. We report a newborn with indirect hyperbilirubinemia caused by minor blood group incompatibilities (P1, M, N, s and Duffy) whose hemolysis was successfully managed with intravenous immunoglobulin therapy. A thirty-two gestational weeks of preterm male baby became severely icteric on postnatal day 11, with a total bilirubin level of 14.66 mg/dl. Antibody screening tests revealed incompatibility on different minor groups (P1, M, N, s and Duffy (Fya ve Fyb)). On postnatal day thirteen, the level of bilirubin increased to 20.66 mg/dl although baby was under intensive phototherapy. After the administration of intravenous immunoglobulin and red blood cell transfusion, hemoglobin and total bilirubin levels became stabilised. Minor blood incompatibilities should be kept in mind during differential diagnosis of hemolytic anemia of the newborn. They share the same treatment algorithm with the other types hemolytic anemia. New studies revealed that intravenous immunoglobulin treatment in hemolytic anemia have some attractive and glamorous results. It should be seriously taken into consideration for treatment of minor blood incompatibilities.


Assuntos
Anemia Hemolítica/etiologia , Bilirrubina/metabolismo , Hiperbilirrubinemia/etiologia , Imunoglobulinas Intravenosas/administração & dosagem , Anemia Hemolítica/diagnóstico , Incompatibilidade de Grupos Sanguíneos/complicações , Diagnóstico Diferencial , Transfusão de Eritrócitos/métodos , Hemoglobinas/metabolismo , Humanos , Recém-Nascido , Recém-Nascido Prematuro , Icterícia Neonatal/etiologia , Masculino
3.
Braz J Med Biol Res ; 52(10): e8926, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31618370

RESUMO

Humoral immunological defects are frequent and important causes of hypogammaglobulinemia, leading to recurrent infections, autoimmunity, allergies, and neoplasias. Usually, its onset occurs in childhood or during the second and third decades of life; however, the diagnosis is made, on average, 6 to 7 years afterwards. As a consequence, antibody defects can lead to sequelae. Here we describe the clinical-laboratory characteristics, treatment, and prognoses of patients with hypogammaglobulinemia. An observational, cross-sectional, and retrospective study of patients attending the recently established outpatient group of Clinical Immunology between 2013 and 2018 was carried out. Patients with IgG levels below 2 standard deviations from the mean values for the age and/or impaired antibody response were included. Eight patients (3 F and 5 M; median age=41 years (16-65), average symptom onset at 25 years (1-59), and time to diagnosis of 10 years were included. The main infections were: sinusitis in 7/8, pneumonia in 6/8, otitis in 2/8, tonsillitis and diarrhea in 2/8, and diarrhea in 2/8 patients. Hypothyroidism was identified in 4/8 (50%) patients. Rhinitis was found in 7/8 (87.5%) and asthma in 3/8 (37.5%) patients. The tomographic findings were consolidations, atelectasis, emphysema, ground glass opacity, budding tree, bronchial thickening, and bronchiectasis. Immunoglobulin reposition was used between 466 and 600 mg/kg monthly (514.3 mg·kg-1·dose-1). Prophylactic antibiotic therapy was included in 7/8 (87.5%) patients. Airway manifestations prevailed in patients with hypogammaglobulinemia. There is a need for educational work to reduce the time of diagnosis and initiation of treatment, avoiding sequelae.


Assuntos
Agamaglobulinemia/diagnóstico , Imunoglobulinas Intravenosas/administração & dosagem , Adolescente , Adulto , Agamaglobulinemia/tratamento farmacológico , Idoso , Estudos Transversais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Fatores de Tempo , Adulto Jovem
4.
Orv Hetil ; 160(38): 1487-1494, 2019 Sep.
Artigo em Húngaro | MEDLINE | ID: mdl-31537095

RESUMO

Immune status was investigated in 186 patients with chronic lymphoid leukaemia between January 2012 and March 2015. Incidences of infections and mortality were analysed in patients who did not receive prophylactic immunoglobulin therapy. Immunoglobulin G (IgG) levels were normal (7-17.8 g/L) or decreased in 62.37% and 35.48% of patients, respectively. We measured high immunoglobulin levels only in a few cases (2.15%). Immunoglobulin levels became increasingly lower in more advanced disease stages (Rai stages). The number of infections was inversely proportional to that. Hypogammaglobulinaemia proved to be more important than disease progression in terms of the development of infections. The most common infections were upper respiratory tract (33.07%) and sepsis (18.90%). Two months after chemotherapy, initially normal immunoglobulin levels decreased by an average of 21%, and at the same time the incidence of infections increased. The most common cause of death was sepsis: 30% occurred at low immunoglobulin levels, while 20% at normal immunoglobulin levels. According to literature, prophylactic immunoglobulin treatment is indicated in patients with chronic lymphoid leukaemia and immunodeficiency for decreasing both morbidity and mortality. According to recommendations in literature, replacement treatment must be administered in severe or moderately severe recurrent bacterial infections. Immunoglobulin prophylaxis may be provided as low dose (10 g), fix dose (18 g) or individually customized higher dose (300-400 mg/kg body weight) treatment. According to recommendations, higher dose immunoglobulin prophylaxis, administered every three weeks on six occasions, is more efficient when customized. With this dose, infection-free condition may be achieved in 50% of patients. Orv Hetil. 2019; 160(38): 1487-1494.


Assuntos
Agamaglobulinemia/tratamento farmacológico , Agamaglobulinemia/mortalidade , Imunoglobulinas Intravenosas/uso terapêutico , Imunoglobulinas/administração & dosagem , Leucemia Linfocítica Crônica de Células B/tratamento farmacológico , Sepse/mortalidade , Agamaglobulinemia/complicações , Relação Dose-Resposta a Droga , Feminino , Humanos , Hungria/epidemiologia , Imunoglobulina G/sangue , Imunoglobulinas Intravenosas/administração & dosagem , Controle de Infecções , Leucemia Linfocítica Crônica de Células B/complicações , Leucemia Linfocítica Crônica de Células B/mortalidade , Masculino , Sepse/diagnóstico , Resultado do Tratamento
5.
Medicine (Baltimore) ; 98(34): e16888, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31441865

RESUMO

RATIONALE: Facial nerve palsy (FNP) is one of the rare neurologic symptoms of Kawasaki disease (KD), associated with a higher incidence of coronary arteries lesions and may be an indicator of more severe disease. PATIENT CONCERNS: A 3-month-old male infant with persistent fever, irritability, and facial asymmetry. DIAGNOSES: KD with FNP. INTERVENTIONS: The infant received intravenous immunoglobulin (IVIG) (2 g/kg/16 hours) and aspirin (50 mg/kg/day) were started on the 8th day of illness. OUTCOMES: Fever and FNP resolved within 48 hours after IVIG treatment. The inflammatory markers all improved to normal or near-normal levels before discharge; all infectious studies returned negative. His left facial weakness was unappreciable at day of discharge. LESSONS: FNP associated with KD is an uncommon finding but may indicate an increased risk of coronary artery involvement. KD should always be kept in mind in the differential diagnosis of a child who presents with prolonged unexplained fever, even with incomplete diagnostic features, as well as the need to be aware of unusual manifestations, such as FNP.


Assuntos
Anomalias dos Vasos Coronários/etiologia , Assimetria Facial/etiologia , Paralisia Facial/etiologia , Síndrome de Linfonodos Mucocutâneos/diagnóstico , Anomalias dos Vasos Coronários/diagnóstico por imagem , Ecocardiografia , Assimetria Facial/tratamento farmacológico , Paralisia Facial/tratamento farmacológico , Humanos , Imunoglobulinas Intravenosas/administração & dosagem , Fatores Imunológicos/administração & dosagem , Lactente , Masculino , Síndrome de Linfonodos Mucocutâneos/complicações
6.
Medicine (Baltimore) ; 98(34): e16906, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31441871

RESUMO

RATIONALE: Brain abnormalities have frequently been reported in neuromyelitis optica spectrum disorders patients, but vertigo as an initial manifestation has rarely been described. PATIENT CONCERNS: A 64-year-old woman who initially presented with vertigo, then accompanied with other brainstem manifestations and spinal cord involvement. DIAGNOSES: MRI revealed medulla oblongata, cervical and thoracic spinal cord lesions. NMO-IgG antibody was seropositive. Taken her previous medical history and clinical manifestations into consideration, the patient was eventually diagnosed as neuromyelitis optica spectrum disorders. INTERVENTIONS: Before diagnosis, symptomatic treatment and acupuncture were adopted, whereas after diagnosis, steroid, intravenous immunoglobulin, and immunosuppressant were supplemented. OUTCOMES: Her dizziness, nausea and vomiting were gradually relieved by symptomatic treatment and acupuncture before the confirmed diagnosis and immunotherapy. After added treatment with steroid, immunosuppressant, especially intravenous immunoglobulin, diplopia and nystagmus disappeared, and superficial sensation was improving. She was fully recovered six months after admission. LESSONS: Vertigo as a rare prodrome of neuromyelitis optica spectrum disorders deserves attention. The symptoms and signs were improved by a combined treatment of steroid, immunosuppressant, acupuncture, and particularly intravenous immunoglobulin.


Assuntos
Neuromielite Óptica/diagnóstico , Vertigem/etiologia , Terapia por Acupuntura , Diplopia/etiologia , Diplopia/terapia , Feminino , Humanos , Imunoglobulinas Intravenosas/administração & dosagem , Fatores Imunológicos/administração & dosagem , Pessoa de Meia-Idade , Náusea/etiologia , Náusea/terapia , Neuromielite Óptica/tratamento farmacológico , Vértebras Torácicas/diagnóstico por imagem , Vertigem/terapia , Vômito/etiologia , Vômito/terapia
7.
Zhonghua Er Ke Za Zhi ; 57(7): 538-542, 2019 Jul 02.
Artigo em Chinês | MEDLINE | ID: mdl-31269554

RESUMO

Objective: To investigate the clinical manifestations, diagnosis, and treatment of H1N1 influenza A-associated encephalopathy (IAE) in children. Methods: The clinical manifestations, laboratory tests, cranial magnetic resonance imaging (MRI), electroencephalography (EEG) examinations and treatments of seven children with H1N1 IAE hospitalized in Guangzhou Women and Children's Medical Center from December 2018 to January 2019 were retrospectively analyzed. Results: Five of the seven children with H1N1 IAE were female. The age at admission was 4 years and 5 months (range 7 months-9 years). Neurological symptoms occurred simultaneously or early (0-3 days) after the flu-like symptom appeared. The main clinical manifestations of neurological symptoms were seizures (repeated seizures in five cases and status convulsion in two cases, including one case of unexpected fever and repeated seizures in a nine-year old girl) accompanied with altered consciousness (drowsiness in five cases and coma in two cases). Cranial MRI in three cases displayed multifocal lesions, mainly in the bilateral thalamus, brainstem and cerebellar hemisphere. MRI also showed reversible splenial lesion in the corpus callusumin in three cases. EEG tracings were characterized by diffuse slow wave activity in four cases, and status epilepticus was monitored in one case. All the 7 cases were treated with oral oseltamivir. Three cases were treated with pulsed methylprednisolone and intravenous immunoglobulin. One case was treated with intravenous immunoglobulin alone and all the patients received oral oseltamivir. All the patients survived, with three patients had minor neurological sequelae at discharge. Conclusions: The main clinical manifestations of H1N1 IAE are seizures and altered consciousness. Cranial MRI combined with EEG is helpful for early diagnosis. Intravenous immunoglobulin and (or) methylprednisolone should be considered for severe cases.


Assuntos
Encefalopatias/diagnóstico , Encefalopatias/tratamento farmacológico , Encéfalo/diagnóstico por imagem , Imunoglobulinas Intravenosas/administração & dosagem , Vírus da Influenza A Subtipo H1N1 , Influenza Humana/diagnóstico , Influenza Humana/tratamento farmacológico , Oseltamivir/uso terapêutico , Encefalopatias/virologia , Criança , Pré-Escolar , Eletroencefalografia , Encefalite Viral/complicações , Encefalite Viral/diagnóstico por imagem , Encefalite Viral/virologia , Feminino , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Vírus da Influenza A Subtipo H1N1/patogenicidade , Influenza Humana/complicações , Influenza Humana/virologia , Imagem por Ressonância Magnética , Masculino , Metilprednisolona , Estudos Retrospectivos , Resultado do Tratamento
8.
NeuroRehabilitation ; 44(4): 609-612, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31256085

RESUMO

BACKGROUND: Chronic inflammatory demyelinating polyneuropathy (CIDP) is a potentially disabling health condition. OBJECTIVE: To assess the effects of different pharmacological interventions used in CIPD. METHODS: To summarize and to discuss the rehabilitation perspective on the published Cochrane Overview "Treatments for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP): an overviewof systematic reviews" by Anne Louise Oaklander, et al., representing the Cochrane Neuromuscular Group. RESULTS: Five CSRs and 23 RCTs, reporting data on corticosteroids, plasma exchange and intravenous immunoglobulin, were considered in the overview. CONCLUSIONS: High quality trials investigating the combined effectiveness of drugs and exercise using ICF based outcomes should be encouraged.


Assuntos
Corticosteroides/administração & dosagem , Imunoglobulinas Intravenosas/administração & dosagem , Troca Plasmática/tendências , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/terapia , Corticosteroides/efeitos adversos , Terapia por Exercício/tendências , Humanos , Imunoglobulinas Intravenosas/efeitos adversos , Troca Plasmática/efeitos adversos , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/diagnóstico , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/fisiopatologia , Resultado do Tratamento
9.
Transplant Proc ; 51(6): 1732-1738, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31301858

RESUMO

Plasma cell-rich acute rejection (PCAR) and antibody-mediated rejection (ABMR), for which a standard treatment has not yet been established, are associated with poor graft survival after kidney transplantation. Here, we report a case series of 3 Japanese patients diagnosed with PCAR accompanied by ABMR. Steroid pulse therapy and rabbit antithymocyte globulin, plasma exchange, intravenous immunoglobulin, and rituximab therapies were sequentially performed in the first case. A graft biopsy after each treatment showed that plasma cell infiltration persisted. Five months after the initiation of rejection therapy, the patient was subjected to bortezomib therapy, which led to the partial elimination of plasma cells from the graft. However, the graft function gradually deteriorated, and hemodialysis treatment was warranted. In the other 2 cases, the patients received the same combination of therapy including bortezomib within a short period. Graft biopsies performed subsequently showed a marked decrease in the number of infiltrated plasma cells, and stabilization of renal graft function was achieved in both cases. Bortezomib, which targets plasma cells, is a potent drug that eliminates infiltrated plasma cells from the graft in PCAR. Thus, in addition to conventional therapy comprising plasma exchange, intravenous immunoglobulin, and rituximab against ABMR, bortezomib may be necessary to administer without any delay to control PCAR.


Assuntos
Bortezomib/uso terapêutico , Terapia Combinada/métodos , Rejeição de Enxerto/tratamento farmacológico , Plasmócitos/efeitos dos fármacos , Inibidores de Proteassoma/uso terapêutico , Adolescente , Corticosteroides/administração & dosagem , Pré-Escolar , Feminino , Rejeição de Enxerto/imunologia , Rejeição de Enxerto/patologia , Sobrevivência de Enxerto , Humanos , Imunoglobulinas Intravenosas/administração & dosagem , Transplante de Rim/efeitos adversos , Masculino , Plasmócitos/patologia , Plasmaferese , Estudos Retrospectivos , Rituximab/administração & dosagem , Adulto Jovem
10.
Int J Med Sci ; 16(4): 576-582, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31171909

RESUMO

Background: Kawasaki disease (KD) is the most common acute coronary vasculitis to occur in children. Although we have uncovered global DNA hypomethylation in KD, its underlying cause remains uncertain. In this study, we performed a survey of transcript levels of DNA methyltransferases and demethylases in KD patients. Materials and Methods: We recruited 145 participants for this study. The chip studies consisted of 18 KD patients that were analyzed before undergoing intravenous immunoglobulin (IVIG) treatment and at least 3 weeks after IVIG treatment, as well as 36 control subjects, using Affymetrix GeneChip® Human Transcriptome Array 2.0. An additional study of 91 subjects was performed in order to validate real-time quantitative PCR. Results: In our microarray study, the mRNA levels of DNMT1 and DNMT3A were significantly lower while TET2 was higher in acute-stage KD patients compared to the healthy controls. Through PCR validation, we observed that the expression of DNMT1 and TET2 are consistent with the Transcriptome Array 2.0 results. Furthermore, we observed significantly lower DMNT1 mRNA levels following IVIG treatment between those who developed CAL and those who did not. Conclusion: Our findings provide an evidence of DNA methyltransferases and demethylases changes and are among the first report that transient DNA hypomethylation is induced during acute inflammatory phase of Kawasaki disease.


Assuntos
Doença da Artéria Coronariana/genética , DNA (Citosina-5-)-Metiltransferase 1/genética , DNA (Citosina-5-)-Metiltransferases/genética , Proteínas de Ligação a DNA/genética , Síndrome de Linfonodos Mucocutâneos/genética , Proteínas Proto-Oncogênicas/genética , Adolescente , Criança , Pré-Escolar , Doença da Artéria Coronariana/complicações , Doença da Artéria Coronariana/patologia , Vasos Coronários/metabolismo , Vasos Coronários/patologia , Metilação de DNA/genética , Feminino , Humanos , Imunoglobulinas Intravenosas/administração & dosagem , Lactente , Recém-Nascido , Inflamação/genética , Inflamação/patologia , Masculino , Síndrome de Linfonodos Mucocutâneos/complicações , Síndrome de Linfonodos Mucocutâneos/patologia , RNA Mensageiro/genética , Transcriptoma/genética
11.
Pediatrics ; 143(6)2019 06.
Artigo em Inglês | MEDLINE | ID: mdl-31048414

RESUMO

BACKGROUND: Coronary artery aneurysms (CAA) are a serious complication of Kawasaki disease. Treatment with intravenous immunoglobulin (IVIg) within 10 days of fever onset reduces the risk of CAA from 25% to <5%. Corticosteroids and infliximab are often used in high-risk patients or those with CAA at diagnosis, but there are no data on their longer-term impact on CAA. METHODS: Retrospective multicenter study including children who had CAA with a z score ≥2.5 and <10 at time of diagnosis and who received primary therapy with IVIg alone or in combination with either corticosteroids or infliximab within 10 days of onset of fever. RESULTS: Of 121 children, with a median age of 2.8 (range 0.1-15.5) years, 30 (25%) received primary therapy with corticosteroids and IVIg, 58 (48%) received primary therapy with infliximab and IVIg, and 33 (27%) received primary therapy with IVIg only. Median coronary z scores at the time of diagnosis did not differ among treatment groups (P = .39). Primary treatment intensification with either corticosteroids or infliximab were independent protective factors against progression of coronary size on follow-up (coefficient: -1.31 [95% confidence interval: -2.33 to -0.29]; coefficient: -1.07 [95% confidence interval: -1.95 to -0.19], respectively). CONCLUSIONS: Among a high-risk group of patients with Kawasaki disease with CAA on baseline echocardiography, those treated with corticosteroids or infliximab in addition to IVIg had less progression in CAA size compared with those treated with IVIg alone. Prospective randomized trials are needed to determine the best adjunctive treatment of patients who present with CAA.


Assuntos
Aneurisma Coronário/tratamento farmacológico , Aneurisma Coronário/epidemiologia , Imunoglobulinas Intravenosas/administração & dosagem , Infliximab/administração & dosagem , Síndrome de Linfonodos Mucocutâneos/tratamento farmacológico , Síndrome de Linfonodos Mucocutâneos/epidemiologia , Adolescente , Antirreumáticos/administração & dosagem , Criança , Pré-Escolar , Aneurisma Coronário/diagnóstico por imagem , Feminino , Seguimentos , Humanos , Lactente , Masculino , Síndrome de Linfonodos Mucocutâneos/diagnóstico por imagem , Estudos Retrospectivos , Fatores de Risco , Resultado do Tratamento
12.
Pediatrics ; 143(6)2019 06.
Artigo em Inglês | MEDLINE | ID: mdl-31048415

RESUMO

OBJECTIVES: Patients with Kawasaki disease can develop life-altering coronary arterial abnormalities, particularly in those resistant to intravenous immunoglobulin (IVIg) therapy. We tested the tumor necrosis factor α receptor antagonist etanercept for reducing both IVIg resistance and coronary artery (CA) disease progression. METHODS: In a double-blind multicenter trial, patients with Kawasaki disease received either etanercept (0.8 mg/kg; n = 100) or placebo (n = 101) subcutaneously starting immediately after IVIg infusion. IVIg resistance was the primary outcome with prespecified subgroup analyses according to age, sex, and race. Secondary outcomes included echocardiographic CA measures within subgroups defined by coronary dilation (z score >2.5) at baseline. We used generalized estimating equations to analyze z score change and a prespecified algorithm for change in absolute diameters. RESULTS: IVIg resistance occurred in 22% (placebo) and 13% (etanercept) of patients (P = .10). Etanercept reduced IVIg resistance in patients >1 year of age (P = .03). In the entire population, 46 (23%) had a coronary z score >2.5 at baseline. Etanercept reduced coronary z score change in those with and without baseline dilation (P = .04 and P = .001); no improvement occurred in the analogous placebo groups. Etanercept (n = 22) reduced dilation progression compared with placebo (n = 24) by algorithm in those with baseline dilation (P = .03). No difference in the safety profile occurred between etanercept and placebo. CONCLUSIONS: Etanercept showed no significant benefit in IVIg resistance in the entire population. However, preplanned analyses showed benefit in patients >1 year. Importantly, etanercept appeared to ameliorate CA dilation, particularly in patients with baseline abnormalities.


Assuntos
Anti-Inflamatórios não Esteroides/administração & dosagem , Etanercepte/administração & dosagem , Imunoglobulinas Intravenosas/administração & dosagem , Síndrome de Linfonodos Mucocutâneos/diagnóstico por imagem , Síndrome de Linfonodos Mucocutâneos/tratamento farmacológico , Doença Aguda , Pré-Escolar , Método Duplo-Cego , Resistência a Medicamentos/efeitos dos fármacos , Resistência a Medicamentos/fisiologia , Quimioterapia Combinada , Feminino , Humanos , Lactente , Masculino
13.
Rinsho Shinkeigaku ; 59(6): 360-364, 2019 Jun 22.
Artigo em Japonês | MEDLINE | ID: mdl-31142711

RESUMO

An 84-year-old woman developed blepharoptosis, diplopia, weakness of extremities, and dysphagia with elevation of serum CK levels after treatment with nivolumab against renal cell carcinoma. 3 Hz repetitive stimulation showed waning in the trapezius muscle, leading to the diagnosis of myasthenia gravis. Laboratory examination showed that anti-acetylcholine receptor antibody was negative. We performed IVIg and steroid therapy. However, her symptoms did not improve, and she died of respiratory failure, although serum CK levels ameliorated to the normal range. The results of autopsy showed atrophy of muscle fibers and massive infiltration of inflammatory cells in the endomysium of the iliopsoas muscle and diaphragm, indicating occurrence of myositis. Immunohistochemical analysis showed that CD8-positive T cells mainly infiltrates in the endomysium with a small number of CD4-potive T cells. Here, we report an autopsy case of nivolumab-induced myasthenia gravis and myositis.


Assuntos
Antineoplásicos Imunológicos/efeitos adversos , Miastenia Gravis/induzido quimicamente , Miastenia Gravis/patologia , Miosite/induzido quimicamente , Miosite/patologia , Nivolumabe/efeitos adversos , Idoso de 80 Anos ou mais , Antineoplásicos Imunológicos/uso terapêutico , Autopsia , Linfócitos T CD4-Positivos/patologia , Linfócitos T CD8-Positivos/patologia , Carcinoma de Células Renais/tratamento farmacológico , Creatina Quinase/sangue , Evolução Fatal , Feminino , Humanos , Imunoglobulinas Intravenosas/administração & dosagem , Neoplasias Renais/tratamento farmacológico , Miastenia Gravis/diagnóstico , Miastenia Gravis/tratamento farmacológico , Miosite/diagnóstico , Miosite/tratamento farmacológico , Nivolumabe/uso terapêutico , Insuficiência Respiratória/induzido quimicamente
16.
Chin Med Sci J ; 34(1): 60-64, 2019 Mar 30.
Artigo em Inglês | MEDLINE | ID: mdl-30961783

RESUMO

Heparin-induced thrombocytopenia (HIT) is a relatively infrequent complication of heparin administration. HIT can cause devastating thrombosis, making it one of the most serious adverse drug reactions encountered in clinical practice. We successfully treated a case of severe HIT presenting with thrombosis and life-threatening bleeding complications with intravenous immunoglobulin (IVIG), platelet transfusion and oral anticoagulant Rivaroxaban. In this case, we considered that IVIG played the most important role by preventing further thrombosis, increasing the platelet count, and ensuring the efficacy of Rivaroxaban. We therefore suggest that IVIG might be the optimal treatment for patients with this urgent condition.


Assuntos
Heparina/efeitos adversos , Imunoglobulinas Intravenosas/administração & dosagem , Transfusão de Plaquetas , Rivaroxabana/administração & dosagem , Trombocitopenia/induzido quimicamente , Trombocitopenia/terapia , Idoso de 80 Anos ou mais , Feminino , Heparina/administração & dosagem , Humanos
17.
Int J Immunopathol Pharmacol ; 33: 2058738419843381, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30968712

RESUMO

Common variable immunodeficiency disorders (CVIDs) represent a group of primary immunodeficiency diseases characterized by hypogammaglobulinemia and dysfunctional immune response to invading pathogens. Previous studies have indicated that CVID is associated with microbial translocation and systemic myeloid cell activation. The goal of this study was to determine whether patients with CVID display elevated systemic levels of markers of granulocyte activation and whether the levels are further influenced by intravenous immunoglobulin (IVIg) infusions. The plasma levels of granulocyte activation markers elastase and myeloperoxidase were determined using enzyme-linked immunosorbent assay (ELISA) in 46 CVID patients and 44 healthy controls. All CVID patients were in a stable state with no apparent acute infection. In addition, granulocyte activation markers' plasma levels in 24 CVID patients were determined prior to and 1 h following IVIg administration. Neutrophil elastase and myeloperoxidase plasma levels were significantly higher in CVID patients than in healthy controls. Systemic elastase levels were further increased following IVIg administration. In vitro stimulation of 13 CVID patients' whole blood using IVIg in a therapeutically relevant dose for 2 h resulted in a significant increase in plasma elastase levels compared to unstimulated blood. The data presented here indicate that CVID is associated with chronic granulocytic activation which is further exacerbated by administering IVIg. Increased myeloperoxidase and elastase levels may contribute to associated comorbidities in CVID patients.


Assuntos
Imunodeficiência de Variável Comum/sangue , Imunodeficiência de Variável Comum/diagnóstico , Elastase de Leucócito/sangue , Peroxidase/sangue , Adulto , Idoso , Idoso de 80 Anos ou mais , Biomarcadores/sangue , Imunodeficiência de Variável Comum/tratamento farmacológico , Feminino , Humanos , Imunoglobulinas Intravenosas/administração & dosagem , Masculino , Pessoa de Meia-Idade , Adulto Jovem
18.
Pediatr Rheumatol Online J ; 17(1): 13, 2019 Apr 03.
Artigo em Inglês | MEDLINE | ID: mdl-30943984

RESUMO

BACKGROUND: Kawasaki disease (KD) is the most common acute coronary vasculitis disease to occur in children. Its incidence has been attributed to the combined effects of infection, genetics, and immunity. Although the etiopathogenesis of KD remains unknown, we have performed a survey of global genetic DNA methylation status and transcripts expression in KD patients in order to determine their contribution to the pathogenesis of KD. METHODS: We recruited 148 participants for this case-control study. The chip studies consisted of 18 KD patients that were analyzed both before undergoing intravenous immunoglobulin (IVIG) treatment and at least 3 weeks afterward, as well as 36 non-KD control subjects, using Illumina HumanMethylation450 BeadChip and Affymetrix GeneChip® Human Transcriptome Array 2.0. We then carried out real-time quantitative PCR on a separate cohort of 94 subjects for validation. RESULTS: According to our microarray study, CD177, a neutrophil surface molecule, appeared to be significantly upregulated in KD patients when compared to controls with epigenetic hypomethylation. After patients received IVIG treatment, CD177 mRNA levels decreased significantly. PCR validation indicated that the CD177 expression is consistent with the Transcriptome Array 2.0 results. Furthermore, the area under the curve values of CD177 between KD patients and controls is 0.937. We also observed significantly higher CD177 levels in typical KD than in incomplete presentation or KD with IVIG resistance. CONCLUSION: In this study, we have demonstrated the epigenetic hypomethylation and increased expression of CD177 during the acute stage of KD. Furthermore, a higher expression of CD177 in KD patients with typical presentation was associated with IVIG resistance.


Assuntos
Isoantígenos/metabolismo , Síndrome de Linfonodos Mucocutâneos/metabolismo , Receptores de Superfície Celular/metabolismo , Estudos de Casos e Controles , Criança , Pré-Escolar , Metilação de DNA , Feminino , Proteínas Ligadas por GPI/sangue , Proteínas Ligadas por GPI/metabolismo , Humanos , Imunoglobulinas Intravenosas/administração & dosagem , Isoantígenos/sangue , Masculino , Síndrome de Linfonodos Mucocutâneos/terapia , Análise de Sequência com Séries de Oligonucleotídeos , Reação em Cadeia da Polimerase em Tempo Real , Receptores de Superfície Celular/sangue , Sensibilidade e Especificidade , Transcriptoma/genética
19.
Vox Sang ; 114(3): 237-246, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30883804

RESUMO

BACKGROUND AND OBJECTIVE: Immunoglobulin replacement therapy (IRT) is often used to support patients with primary immunodeficiency disease (PID) and secondary immunodeficiency disease (SID). Home-based subcutaneous immunoglobulin (SCIg) is reported to be a cheaper and more efficient option compared to hospital-based intravenous immunoglobulin (IVIg) for PID. In contrast, there is little information on the cost-effectiveness of IRT in SID. However, patients who develop hypogammaglobulinaemia secondary to other conditions (SID) have different clinical aetiology compared to PID. This study assesses whether SCIg provides a good value-for-money treatment option in patients with secondary immunodeficiency disease (SID). METHODS: A Markov cohort simulation model with six health states was used to compare cost-effectiveness of IVIg with SCIg from a healthcare system perspective. The costs of treatment, infection and quality-adjusted life years (QALYs) for IVIg and SCIg treatment options were modelled with a time horizon of 10 years and weekly cycles. Deterministic and probabilistic sensitivity analyses were performed around key parameters. RESULTS: The cumulative cost for IVIg was A$151 511 and for SCIg A$144 296. The QALYs with IVIg were 3·07 and with SCIg 3·51. Based on the means, SCIg is the dominant strategy with better outcomes and at lower cost. The probabilistic sensitivity analysis shows that 88·3% of the 50 000 iterations fall below the nominated willingness to pay threshold of A$50 000 per QALY. Therefore, SCIg is a cost-effective treatment option. CONCLUSION: For SID patients in Queensland (Australia), the home-based SCIg treatment option provides better health outcomes and cost savings.


Assuntos
Análise Custo-Benefício , Imunização Passiva/economia , Imunoglobulinas Intravenosas/economia , Austrália , Feminino , Custos Hospitalares , Humanos , Imunoglobulinas Intravenosas/administração & dosagem , Imunoglobulinas Intravenosas/uso terapêutico , Síndromes de Imunodeficiência/terapia , Masculino
20.
Transpl Infect Dis ; 21(3): e13074, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30868720

RESUMO

Intravenous immunoglobulin (IVIg) therapy is increasingly used in the pediatric population, in particular among children with immune-compromising conditions. Pooled immunoglobulin products are routinely tested for hepatitis B surface antigen (HBsAg) and nucleic acid; however, screening for hepatitis B core antibody (anti-HBc) is not commonly performed. Thus, the administration of IVIg containing anti-HBc to children with immune-compromising conditions may complicate the interpretation of hepatitis B serologic testing in that a positive anti-HBc test may represent passive transfer of antibody from IVIg or may indicate resolved or chronic hepatitis B infection. Due to the risk of hepatitis B reactivation in immunocompromised patients, a positive anti-HBc test must be carefully considered. As part of a quality improvement initiative, we identified and reviewed the records of all pediatric patients at our institution who tested positive for anti-HBc over an 18-month period. Of 44 total patients with positive anti-HBc tests, we found that 22 (50%) had previously received IVIg in the preceding 4 months. All but one of these, 21/22 (95%), went on to receive immunosuppressive therapy (IS). Among the patients who received IS, 19 (86%) had not undergone hepatitis B serologic testing prior to IVIg administration and 16 (73%) did not have subsequent testing to distinguish between passive acquisition of anti-HBc from IVIg and chronic hepatitis B infection. Our single-center experience reveals that a high proportion of positive anti-HBc tests in children are presumed to be because of the passive antibody transfer from IVIg. However, a low proportion of patients undergo confirmatory testing, despite the risk of hepatitis B reactivation during IS. We thus propose a risk-based algorithm for interpretation and monitoring of hepatitis B testing in immunocompromised children.


Assuntos
Anticorpos Anti-Hepatite B/sangue , Antígenos do Núcleo do Vírus da Hepatite B/imunologia , Hepatite B/sangue , Hospedeiro Imunocomprometido , Ativação Viral , Adolescente , Algoritmos , Criança , Estudos de Coortes , DNA Viral , Feminino , Hepatite B/diagnóstico , Hepatite B Crônica/sangue , Hepatite B Crônica/diagnóstico , Humanos , Imunização Passiva , Imunoglobulinas Intravenosas/administração & dosagem , Masculino , Programas de Rastreamento , Fatores de Risco
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