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1.
An Pediatr (Barc) ; 70(2): 111-9, 2009 Feb.
Artigo em Espanhol | MEDLINE | ID: mdl-19217565

RESUMO

INTRODUCTION AND AIM: Weekly home-based subcutaneous immunoglobulin (SCIg) therapy is an alternative to intravenous immunoglobulin (IVIg) in the treatment of patients with primary antibody deficiencies. The objective of this study was to investigate the efficacy, safety, related quality of life and cost effectiveness of SCIg in our area. MATERIALS AND METHODS: Observational and descriptive study including paediatric patients with common variable immunodeficiency (CVID) receiving SCIg in our hospital (November 2006 to April 2008). Obtained data were compared with those from the last year with IVIg. RESULTS: Eleven patients with CVID were included. Median age was 15 years. The median trough serum IgG level was 622 mg/dl with IVIg. In patients in whom the SCIg dose was maintained or reduced compared to IVIg, the median trough serum IgG level was 850 mg/dl (p < 0.0005). Annual rate of infection was 2.22 per patient-year, without significant differences to IVIg (p = 0.212). There were 58 treatment-related adverse events (AE) reported with SCIg (45 local AE and 13 systemic AE). The most frequent treatment-related adverse event was infusion-site reaction. Switching to home-based subcutaneous IgG treatment led to significant improvements in quality of life and substantial cost savings. CONCLUSIONS: We conclude that subcutaneous administration of 16% SCIg is a safe and cost-effective alternative to IVIg for replacement therapy of primary antibody deficiencies. Median trough serum IgG levels were higher with SCIg. Local AE were common but mild and the incidence decreased over time. Quality of life is significantly improved.


Assuntos
Imunodeficiência de Variável Comum/tratamento farmacológico , Fatores Imunológicos/administração & dosagem , gama-Globulinas/administração & dosagem , Adolescente , Criança , Feminino , Humanos , Infusões Subcutâneas , Estudos Longitudinais , Masculino , Estudos Retrospectivos , Espanha
2.
An Pediatr (Barc) ; 68(3): 269-72, 2008 Mar.
Artigo em Espanhol | MEDLINE | ID: mdl-18358139

RESUMO

INTRODUCTION: Streptococcus pneumoniae is an infrequent casual agent of hemolytic uremic syndrome (HUS) with more severity than classic HUS. CASE REPORT: We present two patients with pneumococcal pneumonia and empyema who developed HUS. One patient the renal function returned to normal and the other needed a renal transplantation. CONCLUSION: Pneumococcal invasive disease may be a cause of severe HUS, so a high index of suspicion is mandatory to prompt appropriate diagnosis and management.


Assuntos
Síndrome Hemolítico-Urêmica/microbiologia , Infecções Pneumocócicas/complicações , Streptococcus pneumoniae/isolamento & purificação , Síndrome Hemolítico-Urêmica/diagnóstico por imagem , Humanos , Lactente , Transplante de Rim , Masculino , Insuficiência Renal/complicações , Insuficiência Renal/diagnóstico por imagem , Insuficiência Renal/cirurgia , Índice de Gravidade de Doença , Ultrassonografia
3.
An Pediatr (Barc) ; 64(4): 385-7, 2006 Apr.
Artigo em Espanhol | MEDLINE | ID: mdl-16606577

RESUMO

The short bowel syndrome is the result of a congenital or acquired loss of a large part of the small intestine. The most frequent causes of surgical resection of the intestine in infants are arterial or venous thrombosis, intestinal volvulus, necrotizing enterocolitis, and Crohn's disease. Symptoms include nutrient and electrolyte malabsorption, steatorrhea and diarrhea, which can result in failure to thrive. The consequences of extensive small bowel resections consist of nutritional deficiencies, gastric acid hypersecretion, nephrolithiasis, cholelithiasis and lactic acidosis. Of these, D-lactic acidosis is an infrequent but important complication because of the symptoms that it can produce. D-lactic acid in the human organism is generated by intestinal bacteria, D-lactate ingestion, or endogenous production in the methyl glycoxylase pathway. Neurological symptoms such as somnolence, ataxia or altered behavior in a patient with short bowel syndrome should make us think of D-lactic acidosis caused by bacterial overgrowth. We present the case of an 11-year-old boy with short bowel syndrome secondary to multiple resections during the postnatal period who was admitted to hospital for episodes of confusion and altered behavior. The diagnosis was lactic acidosis. Outcome was favorable due to prompt instauration of treatment.


Assuntos
Acidose Láctica/etiologia , Síndrome do Intestino Curto/complicações , Acidose Láctica/diagnóstico , Criança , Humanos , Masculino
4.
An Pediatr (Barc) ; 84(2): 70-8, 2016 Feb.
Artigo em Espanhol | MEDLINE | ID: mdl-26033741

RESUMO

INTRODUCTION: Selective immunoglobulin A deficiency (SIgAD), the most common primary immunodeficiency, is often asymptomatic. High rates of familial clustering have been described in SIgAD, but the causative genetic defect and mechanism of inheritance are unknown. OBJECTIVES: To determine whether familial SIgAD cases show more severe clinical and immunological characteristics than sporadic ones; to investigate the utility of screening first-degree relatives (FDRs) of these patients, and to determine whether symptoms in affected family members are important enough to justify screening. PATIENTS AND METHODS: Descriptive, cross-sectional study (October 2010-September 2011) of all patients with SIgAD and followed up in our center. Demographic, clinical, and analytical data were reviewed. A familial case was defined as an SIgAD patient with at least one affected FDR. RESULTS: Of the 130 participants, 42 were SIgAD patients and 88 FDR. There were 13 (31%) familial cases and and 14 (16%) affected FDRs. Six family members had to be analyzed in order to detect one affected one. There were no clinical differences between familial and sporadic SIgAD cases. The percentages of intestinal disease (p=001, OR=9.57, 95%CI 2.59-35.3), hospitalizations (p=045, OR=4.01; 95%CI 1.10-14.67], and need for chronic treatment (p=006, OR=5.5; 95%CI 1.57-19.54) were higher in affected FDRs than in unaffected ones. CONCLUSIONS: The symptoms were not more severe in familial than sporadic SIgAD cases. Nonetheless, the elevated prevalence of affected FDRs with significant morbidity may justify routine screening of close family members of these patients.


Assuntos
Saúde da Família , Deficiência de IgA/diagnóstico , Estudos Transversais , Humanos , Imunoglobulina A/sangue , Prevalência
5.
An Pediatr (Barc) ; 81(2): 69-76, 2014 Aug.
Artigo em Espanhol | MEDLINE | ID: mdl-24246235

RESUMO

INTRODUCTION: An Imported Diseases Clinic was created in the hospital in 2009. The aim of this study was to asses its contribution in terms of capacity, quality of care and teaching offered. PATIENTS AND METHODS: A retrospective study was conducted from 2009 to 2011, analyzing: A) development of knowledge by means of protocols and publications created, and subject taught; B) capacity and quality of care offered by the analysis of patients seen, the adequacy of the protocols and accessibility. The patients were classified into 3 groups. Group 1: immigrant patient screening, group 2: patient consultation after tropical or sub-tropical travel, group 3: screening of vertical transmission of imported disease. RESULTS: Six protocols have been developed and disseminated on the unit website, as well as 5 scientific publications. A total of 316 patients were evaluated: 191 included in group 1 (29 Adopted and 162 Immigrants), 57 in group 2 (94.7% Visiting Friends and Relatives and 81.5% without a pre-travel consultation). They consulted due to, gastrointestinal symptoms (52.6%) and fever (43.8%), with 68 included in group 3 at risk of imported disease by vertical transmission (62 Trypanosoma cruzi, 1 Human T Lymphotropic Virus and 5 Plasmodium spp.). The overall adherence to the protocols was about 77.1%. DISCUSSION: Infectious Diseases Units must adapt to the reality of the population and be flexible in its structure. Periodic assessment of the quality of care offered is essential, as well as an evaluation on the need for additional studies.


Assuntos
Emigrantes e Imigrantes , Infecções , Viagem , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Infecções/epidemiologia , Infecções/etiologia , Infecções/terapia , Masculino , Estudos Retrospectivos , Centros de Atenção Terciária
6.
An Pediatr (Barc) ; 74(2): 74-83, 2011 Feb.
Artigo em Espanhol | MEDLINE | ID: mdl-21168377

RESUMO

INTRODUCTION: More than 200 primary immunodeficiencies (PID) have been described and about 60% present during childhood. Early diagnosis and treatment have been shown to improve patient outcome. AIM: Analysis of patients with a PID diagnosed in a paediatric tertiary care hospital-referral centre over a period of 10 years. PATIENTS AND METHODS: Medical records of all paediatric patients followed up in our unit were retrospectively reviewed. Clinical and epidemiological features, laboratory tests, therapy and outcome were analysed. RESULTS: One hundred and eighty nine patients were followed up in this period of time. Antibody disorders were the most common diagnosis. In our series, clinical presentation at diagnosis were: recurrent respiratory infections in selective IgA deficiency and common variable immunodeficiency (CVID) patients, failure to thrive and opportunistic infections (mainly viral infections) in patients with severe combined immunodeficiency (SCID), skin abscesses (Staphylococcus aureus, Serratia spp.) and complicated pneumonia (Aspergillus spp., Rhodococcus equi) in chronic granulomatous disease, congenital heart disease and consistent phenotype in 22q11 deletion syndrome, skin abscesses and ecthyma gangrenosum in severe congenital neutropenia and opportunistic infections and sepsis (Pseudomonas aeruginosa) in children with X-linked agammaglobulinaemia (XLA). Lymphoproliferative disorders were common in CVID. No malignancies were observed during this period. One patient with XLA developed chronic encephalitis. All patients with CVID and XLA were receiving immunoglobulin replacement therapy (8 intravenous and 14 (since 2006) subcutaneous route) and in all but two SCID patients, stem cell transplantation was performed. Outcome was good in most of them except 8 SCID (2 prior and 6 after transplantation), 3 Wiskott-Aldrich syndrome, 1 complete DiGeorge, 1 chronic granulomatous disease and 1 ataxia-telangiectasia patients who died during follow-up. CONCLUSION: The vast majority of patients included in this series presented with typical clinical features; therefore, basic knowledge of these entities in primary care and collaboration with hospital referral centres should allow a large number of PID in children to be diagnosed at an early stage, leading to proper treatment and monitoring, and therefore improvement of patient prognosis.


Assuntos
Síndromes de Imunodeficiência , Adolescente , Criança , Pré-Escolar , Feminino , Hospitais , Humanos , Síndromes de Imunodeficiência/diagnóstico , Síndromes de Imunodeficiência/epidemiologia , Síndromes de Imunodeficiência/terapia , Lactente , Recém-Nascido , Masculino , Estudos Retrospectivos , Fatores de Tempo
7.
An Pediatr (Barc) ; 75(6): 380-95, 2011 Dec.
Artigo em Espanhol | MEDLINE | ID: mdl-21757410

RESUMO

INTRODUCTION: Poor adherence to antiretroviral treatment (ART) is the commonest cause of treatment failure in children and adults living with HIV, and this is especially important during adolescence. Therefore, any analysis of ART effectiveness in children should include an evaluation of adherence to ART. The aim of this study is to assess the usefulness of an ART adherence monitoring program in an HIV-infected paediatric population. PATIENTS AND METHODS: An observational and cross-sectional study was performed, within the framework of the "Health Education Program for Optimising Adherence in Paediatric Patients with HIV", which is part of the "I am not alone" project. Adherence was assessed simultaneously by different methods: personal interview, therapeutic drug monitoring, pharmacy dispensing records and evolution of viral load and T CD4+ lymphocyte count. RESULTS: Twenty patients were included (50% female, median age 14.5 years). Percentage of self-reported full adherence was 90% (95% CI: 70-97.2%); however, the median adherence percentage according to pharmacy dispensing records was significantly lower (83.3%, SD=32.88). The average of drugs and dosage forms per day were 3.5 (SD=0.83) and 5.5 (SD=2.72), respectively. There was an inverse relationship between the number of dosage forms per day and adherence scores (F=13.8; P=.002). No single method was statistically related to adherence, although therapeutic drug monitoring showed a trend towards significance. CONCLUSIONS: Global adherence to ART was high and was easier with simpler regimens. Self-reported adherence overestimated real adherence to ART in our cohort. The simultaneous use of different methods to assess adherence is recommended in HIV-infected children.


Assuntos
Antirretrovirais/uso terapêutico , Infecções por HIV/tratamento farmacológico , Adesão à Medicação , Inquéritos e Questionários , Adolescente , Criança , Pré-Escolar , Estudos Transversais , Feminino , Humanos , Lactente , Masculino , Estudos Retrospectivos
9.
Acta Paediatr ; 92(5): 631-3, 2003 May.
Artigo em Inglês | MEDLINE | ID: mdl-12839298

RESUMO

UNLABELLED: Lemierre syndrome is a severe postanginal sepsis complicated by internal jugular thrombophlebitis. We report on a 14 y-old girl affected by high fever, shivering chills, headache, severe lateral neck pain, left ocular proptosis and general malaise. Magnetic resonance imaging of the head and neck showed right internal jugular vein and sigmoid sinus thrombosis. Fusobacterium sp. was identified in the blood culture. CONCLUSION: Our report is a reminder that Lemierre syndrome still exists and remains potentially life threatening. A high index of suspicion is necessary to prompt diagnosis and treatment.


Assuntos
Infecções por Fusobacterium/diagnóstico , Infecções por Fusobacterium/terapia , Fusobacterium/isolamento & purificação , Veias Jugulares/diagnóstico por imagem , Sepse/diagnóstico , Sepse/terapia , Tromboflebite/diagnóstico , Tromboflebite/terapia , Adolescente , Feminino , Infecções por Fusobacterium/microbiologia , Humanos , Veias Jugulares/microbiologia , Radiografia , Sepse/microbiologia , Síndrome , Tromboflebite/microbiologia
10.
Acta Paediatr ; 91(4): 383-90, 2002.
Artigo em Inglês | MEDLINE | ID: mdl-12061352

RESUMO

UNLABELLED: The aim of this multicentre, double-blind, randomized study was to assess the paediatric antipyretic efficacy of a new ibuprofen formulation containing L-arginine for gastric protection, compared with the efficacy of paracetamol. For this purpose 100 patients were given ibuprofen-arginine (1 drop/kg: 6.67 mg/kg) and 99 paracetamol (4 drops/kg: 10.65 mg/kg). The main efficacy endpoint was the mean change in tympanic temperature 4 h after drug intake. Twelve patients were excluded because of early vomiting or spitting out the medication. The resulting efficacy analysis population included a total of 88 patients treated with ibuprofen-arginine and 87 with paracetamol. Mean change in tympanic temperature (degrees C) showed no difference between groups (p = 0.527) but more patients in the ibuprofen-arginine group attained a temperature reduction greater than 2 degrees C (p = 0.043). A total of 107 patients required antipyretic rescue medication, with a smaller proportion in the ibuprofen-arginine group. Although this was not statistically significant, a trend towards improved activity was observed (p = 0.100). Overall efficacy was judged from the recovery or improvement in 68.8% of patients in the ibuprofen-arginine group compared with 65.5% in the paracetamol group. Nineteen patients reported adverse events, with vomiting being the most common complaint, but no differences were detected between treatments. CONCLUSION: Based on the present results, ibuprofen-arginine oral drops have shown to be a safe, well-tolerated and potent paediatric antipyretic agent. Hence, ibuprofen-arginine should be considered as an adequate choice for the control of paediatric fever of likely infectious aetiology.


Assuntos
Acetaminofen/uso terapêutico , Analgésicos não Narcóticos/uso terapêutico , Arginina/uso terapêutico , Febre/tratamento farmacológico , Ibuprofeno/uso terapêutico , Acetaminofen/administração & dosagem , Acetaminofen/efeitos adversos , Analgésicos não Narcóticos/administração & dosagem , Analgésicos não Narcóticos/efeitos adversos , Arginina/administração & dosagem , Arginina/efeitos adversos , Criança , Pré-Escolar , Método Duplo-Cego , Combinação de Medicamentos , Determinação de Ponto Final , Feminino , Humanos , Ibuprofeno/administração & dosagem , Ibuprofeno/efeitos adversos , Masculino
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