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1.
Food Chem ; 302: 125296, 2020 Jan 01.
Artigo em Inglês | MEDLINE | ID: mdl-31400698

RESUMO

The process of manufacturing infant milk formulas (IMFs) involves heat treatments that can lead to whey protein denaturation. The objective of the study was to determine how protein composition affects the denaturation kinetics of the whey proteins within IMFs. Three model IMFs (1.3% of cow's milk protein) were produced with a caseins: whey proteins ratio of 40:60, differing only by the whey protein composition. The kinetics of heat-induced denaturation of α-lactalbumin, ß-lactoglobulin and lactoferrin were investigated between 67.5 °C and 80 °C by chromatographic quantification of the residual native proteins. Results showed that the heat-denaturation of α-lactalbumin was reduced when ß-lactoglobulin was absent. The heat-denaturation of lactoferrin was not affected by the composition of the IMFs but its presence enhanced the heat-denaturation of ß-lactoglobulin. This study revealed that, for higher heat treatments (90 °C/15 s, 75 °C/15 min), IMF containing α-lactalbumin and lactoferrin preserved a higher proportion of native whey proteins than IMFs containing ß-lactoglobulin.


Assuntos
Temperatura Alta , Fórmulas Infantis/química , Desnaturação Proteica , Proteínas do Soro do Leite/química , Animais , Bovinos , Humanos , Lactente , Cinética
2.
Rev. Esc. Enferm. USP ; 53: e03486, Jan.-Dez. 2019. tab
Artigo em Inglês, Português | LILACS, BDENF - Enfermagem | ID: biblio-1020393

RESUMO

RESUMO Objetivo Avaliar o impacto das Infecções Relacionadas à Assistência à Saúde no custo da hospitalização de crianças. Método Estudo de coorte, prospectivo, quantitativo, envolvendo crianças admitidas nas Unidades de Internação e de Terapia Intensiva Pediátrica de um hospital universitário público. Os dados foram analisados por meio do software SPSS por distribuição de frequências, medidas de tendência central e dispersão. Para todas as análises foi considerado o nível de significância estatística de p<0,05. Resultados A amostra foi composta de 173 crianças, destas, 18,5% desenvolveram infecção relacionada à assistência à saúde, que aumentou em 4,2 vezes (p<0,001) o custo da hospitalização. Maior impacto de custo foi observado entre pacientes com dois ou mais sítios infecciosos (R$ 81.037,57, p=0,010) e sepse (R$ 46.315,63 p<0,001). Crianças colonizadas por microrganismos multirresistentes, com prevalência de E. coli e A. baumannii ESBL, geraram custos maiores, R$ 35.206,15 e R$ 30.692,52, respectivamente. Conclusão As infecções relacionadas à assistência à saúde aumentaram significativamente os custos da hospitalização de crianças, em especial entre aquelas com mais de dois sítios infecciosos, que desenvolveram sepse e colonizadas por microrganismos multirresistentes.


RESUMEN Objetivo Evaluar el impacto de las Infecciones Relacionadas con la Asistencia Sanitaria en el costo de la hospitalización de niños. Método Estudio de cohorte, prospectivo, cuantitativo, involucrando a niños ingresados en las Unidades de Hospitalización y de Cuidados Intensivos Pediátricos de un hospital universitario público. Los datos fueron analizados mediante el software SPSS por distribución de frecuencias, medidas de tendencia central y dispersión. Para todos los análisis, se consideró el nivel de significación estadística de p<0,05. Resultado La muestra estuvo compuesta de 173 niños, de estos el 18,5% desarrollaron infección relacionada con la asistencia sanitaria, que aumentó 4,2 veces (p<0,001) el costo de la estancia hospitalaria. Mayor impacto de costo fue observado entre pacientes con dos o más sitios infecciosos (R$ 81.037,57, p=0,010) y sepsis (R$ 46.315,63 p<0,001). Niños colonizados por microorganismos multirresistentes, con prevalencia de E. coli y A. baumannii ESBL, generaron costos mayores, R$ 35.206,15 y R$ 30.692,52, respectivamente. Conclusión Las infecciones relacionadas con la asistencia sanitaria aumentaron significativamente los costos de la hospitalización de niños, en especial entre los con más de dos sitios infecciosos, que desarrollaron sepsis y colonizados por microorganismos multirresistentes.


ABSTRACT Objective To evaluate the impact of Healthcare-Associated Infections on the hospitalization cost of children. Method A prospective, quantitative cohort study involving children admitted to the Inpatient and Pediatric Intensive Care Units of a public university hospital. The data were analyzed through SPSS software by frequency distribution, central tendency measures and dispersion. The level of statistical significance was set at p<0.05 for all analyzes. Results The sample consisted of 173 children, of whom 18.5% developed Healthcare-Associated Infections, which increased the hospitalization costs 4.2 times (p<0.001). A greater cost impact was observed among patients with two or more infectious sites (R$81,037.57; p=0.010) and sepsis (R$46,315.63; p<0.001). Children colonized by multiresistant microorganisms with a prevalence of E. coli and A. baumannii ESBL also generated higher costs of R$35,206.15 and R$30,692.52, respectively. Conclusion Healthcare-Associated Infections significantly increased the hospitalization costs for children, especially among those with more than two infectious sites, who developed sepsis or were colonized by multiresistant microorganisms.


Assuntos
Humanos , Masculino , Feminino , Recém-Nascido , Lactente , Pré-Escolar , Criança , Criança Hospitalizada , Infecção Hospitalar , Custos de Cuidados de Saúde , Resistência Microbiana a Medicamentos , Unidades de Terapia Intensiva Pediátrica , Estudos de Coortes , Hospitais Universitários
3.
Asunción; s.n; Nov 2019. (1-67) p.
Tese em Espanhol | LILACS, BDNPAR | ID: biblio-1021733

RESUMO

La infancia es considerada como una etapa trascendental en el proceso evolutivo del hombre, caracterizada por dos fenómenos: crecimiento y desarrollo, por tanto es fundamental una adecuada nutrición. Con relación a este aspecto, el programa de complementación nutricional denominado PANI (Programa Ampliado Nutricional Integral), se implementa, para garantizar la atención oportuna de niños desnutridos y en riesgo de desnutrición y evitar otros factores de riesgo que puedan poner en peligro a esta población vulnerable. Objetivo: Analizar el impacto de la implementación del programa PANI en niños menores de 5 años desnutridos y con riesgo de desnutrición en la Unidad de Salud Familiar ItáKa´aguy, durante el periodo 2015 a 2017. Metodología: Se realizó un estudio descriptivo, retrospectivo, transversal, en donde se evaluó a 74 niños menores de 5 años desnutridos y con riesgos de desnutrición, que fueron atendidos en la Unidad de Salud Familiar ItáKa´aguy, (Fernando de la Mora, XIa Región Sanitaria - Central). Se implementó con ellos el Programa de Asistencia Nutricional y luego de 2 años se compararon los resultados de la evaluación inicial, con las actuales. Resultados: Se resalta que, previo a la intervención sólo 4 niños tuvieron un peso adecuado; 19 niños se encontraban en estado de desnutrición; 6 niños se encontraban en el nivel de peso no adecuado y 40 niños se encontraban en "Riesgo de desnutrición". Mientras que posterior a la implementación del programa, 62 niños obtuvieron el peso adecuado a su edad; 3 niños se mantuvieron con signos clínicos de desnutrición y 4 niños manifestaron riesgo de desnutrición. Conclusión: se encontró que el PANI ha contribuido a mejorar de la desnutrición en los niños y niñas, como en el peso acorde con la edad, pero no así en aspectos como talla y el edema, por lo que se concluye que el impacto ha sido medio. Palabras Clave: Nutrición de los Grupos de Riesgo, Programas y Políticas de Nutrición y Alimentación,


The childhood is considered as a transcendental stage in the evolutionary process of man, characterized by two phenomena: growth and development, therefore proper nutrition is essential. In relation to this aspect, the nutritional complementation program called PANI (Comprehensive Nutritional Extended Program), is implemented to ensure timely care of malnourished children at risk of malnutrition and avoid other risk factors that may endanger this population vulnerable. Objective: To analyze the impact of the implementation of the PANI program in children under 5 years of age undernourished and at risk of malnutrition in the ItáKa'aguy Family Health Unit, during the period 2015 to 2017. Methodology: A descriptive, retrospective study was conducted, cross-sectional, in which 74 under-5 undernourished children with risk of malnutrition were evaluated, who were attended in the ItáKa'aguy Family Health Unit (Fernando de la Mora, XIa Sanitary - Central Region). The Nutritional Assistance Program was implemented with them and after 2 years the results of the initial evaluation were compared with the current ones. Results: It is highlighted that, prior to the intervention, only 4 children had an adequate weight, 19 children were in malnutrition, 6 children were at the unsuitable weight level and 40 children were at risk of malnutrition; while after the implementation of the program, 62 children obtained the appropriate weight at their age, 3 children remained with clinical signs of malnutrition and 4 children showed risk of malnutrition. Conclusion: it was found that PANI has contributed to improve malnutrition in boys and girls, as in weight according to age, but not in aspects such as height and edema, so it is concluded that the impact has been medium.


Assuntos
Humanos , Masculino , Feminino , Recém-Nascido , Lactente , Pré-Escolar , Programas de Nutrição , Transtornos da Nutrição Infantil/epidemiologia , Transtornos da Nutrição do Lactente/epidemiologia , Políticas Públicas de Saúde , Paraguai/epidemiologia , Transtornos da Alimentação e da Ingestão de Alimentos , Estudos Transversais , Estudos Retrospectivos , Fatores de Risco , Nutrição da Criança , Nutrição do Lactente , Classificação Internacional de Atenção Primária
4.
BMJ ; 367: l4993, 2019 11 04.
Artigo em Inglês | MEDLINE | ID: mdl-31685481

RESUMO

The studyGilbert R, Brown M, Rainford N et al. Antimicrobial-impregnated central venous catheters for prevention of neonatal bloodstream infection (PREVAIL): an open-label, parallel-group, pragmatic, randomised controlled trial. Lancet Child Adolesc Health 2019;3:381-90.The study was funded by the NIHR Health Technology Assessment programme (project number 12/167/02).To read the full NIHR Signal, go to https://discover.dc.nihr.ac.uk/content/signal-000782/antimicrobial-central-venous-catheters-for-pre-term-babies-do-not-reduce-infections.


Assuntos
Anti-Infecciosos , Bacteriemia , Cateteres Venosos Centrais , Antibacterianos , Criança , Humanos , Lactente , Recém-Nascido , Avaliação da Tecnologia Biomédica
5.
Zhonghua Er Ke Za Zhi ; 57(11): 837-843, 2019 Nov 02.
Artigo em Chinês | MEDLINE | ID: mdl-31665837

RESUMO

Objective: To summarize the clinical features of leukoencephalopathy with vanishing white matter disease (VWM) in children. Methods: A retrospective cohort study was performed on 54 genetically diagnosed VWM patients in Peking University First Hospital from January 2007 to March 2019. Paper registration form and electronic medical record system were used to collect the data,and the children were divided into five groups according to the age of disease onset:<1 year, 1-<2 years, 2-<4 years, 4-<8 years and 8-<18 years respectively. The progression of motor function, episodic aggravation, epileptic seizures, survival time, brain magnetic resonance imaging (MRI) and genotype features were analyzed and compared. Non-parametric test, χ(2) test or Fisher's exact test were used for comparison among groups; Kaplan-Meier survival curve was adopted to delineate the survival status of the children. Results: Fifty-four VWM patients were included in the study, including 34 males and 20 females.The age of disease onset was 2 years and 8 months (ranged from 6 months to 9 years and 7 months). Onset age was less than 1 year in 5 cases; onset age was 1-<2 years in 12 cases; onset age was 2-<4 years in 25 cases; onset age was 4-<8 years, in 10 cases; onset age was 8-<18 years in 2 cases; 94% (51/54) of patients had complaint of motor regression at the first visit; 87% (47/54) of patients suffered from episodic aggravation. Episodic seizures occurred in 43% (23/54) patients. In survivors with disease durations of 1-3 years, in 38% (9/24) patients the disease was classified as grades Ⅳ-Ⅴ by gross motor function classification system (GMFCS). For the onset age 1-<2 years group, 1 patient was classified as GMFCS Ⅳ among 3 survivors with disease durations of 1-3 years. As for the 2-<4 years group, 6 patients were classified as GMFCS Ⅳ-Ⅴ among 15 patients with disease durations of 1-3 years, whereas 1 patient was classified as GMFCS Ⅳ-Ⅴ among 4 patients with disease durations of 1-3 years in the 4-<8 years group. Lesions, liquefaction and diffusion restriction in brain MRI were compared among different groups, and it was revealed that the earlier the age of disease onset was, the more likely the subcortical white matter (frontal lobe P<0.01,temporal and parieto-occipital lobe both P=0.002), internal capsule (anterior limb P<0.01, posterior limb P=0.00) and brain stem (midbrain P=0.001, pons P<0.01) were to be involved. In addition, internal capsule (anterior limb P=0.002, posterior limb P=0.005) and brain stem (midbrain P=0.001, pons P=0.003) showed more diffuse restricted diffusion. Moreover, the subcortical white matter (frontal and parieto-occipital lobe both P<0.01, temporal lobe P=0.005) showed earlier rarefaction. The 1-year and 2-year survival rates of the overall patients were 81% and 75% respectively, while the 15-year survival rate was 45%. EIF2B5 gene variation was the most common, which accounts for 43% (23/54), followed by EIF2B3 (22%, 12/54). Conclusions: The majority of VWM patients complained of motor regression at the first visit, episodic aggravation and epileptic seizures are common in the course. Earlier age at onset is associated with more rapid clinical progression, shorter survival time as well as more extensive lesions, liquefaction and diffusion restriction in brain MRI. The most common variant gene is EIF2B5, followed by EIF2B3.


Assuntos
Encéfalo/diagnóstico por imagem , Leucoencefalopatias/patologia , Adolescente , Tronco Encefálico , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Imagem por Ressonância Magnética , Masculino , Transtornos dos Movimentos/fisiopatologia , Estudos Retrospectivos
6.
Zhonghua Er Ke Za Zhi ; 57(11): 830-836, 2019 Nov 02.
Artigo em Chinês | MEDLINE | ID: mdl-31665836

RESUMO

Objective: To summarize the clinical and genetic features of ß-propeller protein-associated neurodegeneration (BPAN). Methods: The clinical data of 17 patients with BPAN with WDR45 gene variants were retrospectively collected at Children's Hospital of Fudan University, Peking University First Hospital, Capital Institute of Pediatrics, Shengjing Hospital of China Medical University and Shanghai Children's Hospital from June 2016 to December 2018, and their clinical manifestations, electroencephalogram, neuroimaging and genetics were analyzed. Results: Seventeen cases (13 females, 4 males), aged 1.1-8.8 years, were included. The median age of seizure onset was 14.5 months, from 3 months to 24 months of age, manifested with epileptic spasm in 6 cases and focal seizures in 5 cases. Eight patients had only one seizure type and 8 patients had two or more seizure types. Nine patients had complete remission of seizures. All 16 patients with seizures had developmental delay before the seizure onset, of whom 13 patients had moderate to severe seizures. The brain magnetic resonance imaging (MRI) was abnormal in 13 patients, including cerebral atrophy (10 cases) and thinning of the corpus callosum (9 cases). The brain magnetic susceptibility weighted imaging (SWI) in preschool stage showed prominent T2 hypointense signals in bilateral globus pallidus and brainstem ventral in two cases. Five seizure types (spasm, focal, absence, myodonic and generalized tonic clonic seizures)were found on ictal electroencephalogram(EEG) recordings. Compared to female patients(17(6-24) months of ege), male cases had earlier seizure onset (3, 4, 5, 18 months of age) . All patients had de novo variations in WDR45(6 nonsense, 4 frameshift, 3 missense and 4 splicing variations), with hemizygous variants in 3 males, mosaic variants in a male and heterozygous variants in 13 females, within which 5 variations had not been reported (c.977-1C>T,c.976+1G>C,c.10C>T,c.806del and c.110T>C). Conclusions: The patients with BPAN have profound developmental delay and are vulnerable to seizures. The male patients with BPAN tend to have more severer clinical phenotype than females. Early brain SWI could facilitate the timely diagnosis of this disease.


Assuntos
Proteínas de Transporte/genética , Epilepsia/genética , Doenças Neurodegenerativas/genética , China , Eletroencefalografia , Epilepsia/diagnóstico , Feminino , Humanos , Lactente , Masculino , Doenças Neurodegenerativas/diagnóstico por imagem , Estudos Retrospectivos , Convulsões
7.
Zhonghua Er Ke Za Zhi ; 57(11): 857-862, 2019 Nov 02.
Artigo em Chinês | MEDLINE | ID: mdl-31665840

RESUMO

Objective: To analyze the clinical characteristics of patients with PCDH19-female limited epilepsy (PCDH19-FE). Methods: The clinical data of 60 female epilepsy patients with PCDH19 gene heterozygous variations at the Department of Pediatrics, Peking University First Hospital from October 2007 to December 2018 were collected and analyzed retrospectively, their clinical manifestations, accessory examination and follow-up treatment were summarized. Results: Data of a total of 60 cases of PCDH19-FE were collected. The seizure onset occurred between 4 and 42 months of age (median: 11 months of age). Focal seizures occurred in 47 patients (78%), generalized tonic-clonic seizures (GTCS) occurred in 30 patients (50%), and other rare types of seizures included atypical absence, myoclonic, clonic, tonic, and atonic seizures. Two or more seizures types existed in 24 patients (40%), and seven patients (12%) had attacks of status epilepticus. Sensitivity to fever was observed in 47 out of them (78%) and clustering of seizures as found in all patients. During the interictal phase, focal discharges were monitored in 22 cases (22/45, 49%), multifocal discharges in 12 cases (12/45, 27%), widely discharging in 2 cases (4%), and both focal and widely discharging in 9 cases (20%). Clinical seizures were detected in 30 patients during the electroencephalogram (EEG) recording, including focal seizures in 22 cases, GTCS seizures in 8 cases, tonic seizure in three cases, myoclonic seizure followed by GTCS in one case, and two types of seizures in four cases. Before seizure onset, 57 patients had normal development and three patients had delayed language development. After seizure onset, varied degrees of intelligence disability were present in 38 cases (63%), language delay in 36 cases (60%), and gait instability in 10 cases (17%). Autistic features occurred in 17 cases (28%); and other behavioral problems like learning difficulties, personality, or emotional disorders existed in 33 cases (55%). Age at last follow-up ranged from one year and 3 months to 22 years and 3 months of age, 17 patients (28%) were seizure-free for more than 2 years (5 to 22 years at the last follow-up). The efficiency of antiepileptic drugs were 65% (33/51) in sodium valproate, 63% (27/43) in levetiracetam and 59% (20/34) in topiramate. Conclusions: The clinical features of PCDH19-FE are characterized by clustering of seizures, focal seizures in most cases, sensitivity to fever mostly, focal discharges principally in EEG, varied degrees of intellectual disability or movement disorder, combined with autism spectrum disorders in partial and high efficiency in sodium valproate or levetiracetam treatment.


Assuntos
Caderinas/genética , Epilepsias Mioclônicas/genética , Epilepsia/genética , Convulsões/genética , Adolescente , Transtorno do Espectro Autista , Criança , Pré-Escolar , Eletroencefalografia , Epilepsia/fisiopatologia , Feminino , Humanos , Lactente , Mutação , Estudos Retrospectivos , Convulsões/fisiopatologia , Adulto Jovem
8.
Zhonghua Er Ke Za Zhi ; 57(11): 863-869, 2019 Nov 02.
Artigo em Chinês | MEDLINE | ID: mdl-31665841

RESUMO

Objective: To analyze the clinical characteristics of newly treated high-risk group neuroblastoma (NB) patients with bone marrow metastasis and to explore the prognostic factors. Methods: The clinical features (sex, age, stage, risk group, pathological type, metastatic site, etc.) of 203 newly treated high-risk NB patients with bone marrow metastasis admitted to Hematology Oncology Center, Beijing Children's Hospital from January 2007 to December 2016 were analyzed retrospectively. There were 118 males (58.1%) and 85 females (41.9%). Kaplan-Meier method was used for survival analysis and Cox regression was used to analyze the prognostic factors. Results: The age at onset of the 203 patients was 41 months (9-147 months). The metastatic sites at diagnosis were as follows: bone in 195 cases (96.1%), distant lymph nodes in 104 cases (51.2%), skull and endomeninx in 61 cases (30.0%), orbit in 30 cases (14.8%), pleura in 16 cases (7.9%), liver in 13 cases(6.4%), canalis spinalis in 13 cases (6.4%), other sites in 11 cases (5.4%) and skin and soft tissue in 10 cases (4.9%). In all, 194 cases were enrolled for prognostic analysis. The follow-up time was 36 months (1 day-138 months) , and the 5-years event free survival (EFS) and overall survival (OS) were 36.1% and 39.7%, respectively. A total of 118 patients (60.8%) had events (first relapse or death) with the time to event occurrence was 15 months (1 day-72 months), whereas 112 patients (57.7%) died with the event occurrence to death time was 3 months (1 day-21 months). There was no significant difference in 5-years OS between radiotherapy group and non-radiotherapy group (42.3% vs. 38.3%, χ(2)=3.671, P=0.055). The 5-years OS in transplantation group was significantly better than the non-transplantation group (44.3% vs. 35.5%, χ(2)=8.878, P=0.003), and the radiotherapy combined transplantation group also had a better 5-years OS rate than the non-radiotherapy combined transplantation group (45.8% vs. 37.3%, χ(2)=5.945, P=0.015). Univariate survival analysis showed lactate dehydrogenase ≥ 1 500 U/L, the amplification of MYCN, the metastatic sites of orbit, canalis spinalis and pleura were associated with poor prognosis of newly diagnosed high-risk NB patients (χ(2)=21.064, 13.601, 3.998, 6.183, 15.307, all P<0.05). The amplification of MYCN and the metastatic sites of pleura were risk factors for prognosis of newly diagnosed high-risk NB patients by Cox regression models (HR=1.896,1.100, 95%CI: 1.113-3.231, 1.020-1.187, both P<0.05). Conclusions: The prognosis is unfavorable in high-risk group NB patients with BM metastasis. Radiotherapy combined with transplantation can further improve the prognosis of these patients. The amplification of MYCN and the metastatic sites of pleura were the poor prognostic factors for high-risk NB patients with bone marrow metastasis.


Assuntos
Neoplasias da Medula Óssea/patologia , Neuroblastoma/patologia , Neoplasias da Medula Óssea/mortalidade , Neoplasias da Medula Óssea/radioterapia , Criança , Pré-Escolar , Intervalo Livre de Doença , Feminino , Humanos , Lactente , Masculino , Metástase Neoplásica , Neuroblastoma/mortalidade , Neuroblastoma/radioterapia , Prognóstico , Estudos Retrospectivos , Taxa de Sobrevida , Resultado do Tratamento
9.
Medicine (Baltimore) ; 98(44): e17744, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31689826

RESUMO

Congenital syphilis (CS) can cause serious impact on the fetus. However, congenital syphilis presenting as sepsis is a critical condition but hardly identified by the clinic for the first time. In this study, we aimed to identify the benefit of earlier and accurate diagnosis for the infants who suffer congenital syphilis presenting as sepsis.A retrospective study was performed with patients diagnosed of congenital syphilis presenting as sepsis who were the inpatients in the West China Second Hospital between 2011 and 2018. The control group was collected in the neonatal sepsis patients whose blood culture are positive.Fifty-eight patients were included in the study. In the congenital syphilis group, one patient died and 12 (41.3%) patients get worse to MODS (multiple organ dysfunction syndrome). Symptoms, signs, and lab examinations are found to be significantly different (P < .05) between two groups as below, including rash, palmoplantar desquamation, abdominal distension, splenomegaly, hepatomegaly, etc. And, at the aspect of Hb, PLT, WBC, CRP, ALT, AST, these differences occurred in the different groups. It is obvious that the prognosis of children with syphilis is worse. According to a comparison between the different outcomes in the CS, the worse outcome subgroup of patients is significantly younger and have more severely impaired liver function.Because of the high mortality of these infants, pediatricians should improve awareness of CS. Syphilis screening is recommended for pregnant women.


Assuntos
Sepse Neonatal/diagnóstico , Complicações Infecciosas na Gravidez/diagnóstico , Sepse/diagnóstico , Sífilis Congênita/diagnóstico , Sífilis/diagnóstico , Estudos de Casos e Controles , China , Diagnóstico Diferencial , Diagnóstico Precoce , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Insuficiência de Múltiplos Órgãos/diagnóstico , Insuficiência de Múltiplos Órgãos/microbiologia , Insuficiência de Múltiplos Órgãos/mortalidade , Sepse Neonatal/microbiologia , Sepse Neonatal/mortalidade , Gravidez , Complicações Infecciosas na Gravidez/microbiologia , Complicações Infecciosas na Gravidez/mortalidade , Estudos Retrospectivos , Sepse/microbiologia , Sepse/mortalidade , Sífilis/microbiologia , Sífilis/mortalidade , Sorodiagnóstico da Sífilis , Sífilis Congênita/mortalidade
10.
Medicine (Baltimore) ; 98(44): e17749, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31689829

RESUMO

RATIONALE: Early infantile epileptic encephalopathy (EIEE) 65 was recently shown to be caused by the cytoplasmic FMRP interacting protein 2 (CYFIP2) mutation. To date, only 5 cases have been reported in two articles, and all the outcomes in all cases were poor. PATIENT CONCERNS: In this study, we reported an 8-month-old girl with a 1 month-long history of seizures and developmental delay. Over 1 month later, she developed epileptic spasms in clusters with hypsarrhythmia on electroencephalography. DIAGNOSIS: The patient was diagnosed with EIEE 65 and trio-based whole-exome sequencing revealed a causative de novo CYFIP2 mutation c.260G >T (p.Arg87Leu). INTERVENTIONS: The proband was successively treated with multiple antiepileptic drugs, including levetiracetam, phenobarbital, VitB6, topiramate, methylprednisolone, prednisone, valproic acid and vigabatrin. OUTCOMES: After resistance to multiple anti-epileptic drugs over 2 months of treatment, she finally achieved seizure-free several days after vigabatrin administration and her developmental delay steadily improved. LESSONS: OUR: case confirmed that CYFIP2 was the pathogenic gene of EIEE 65. We also first demonstrated vigabatrin might be effective for control of seizures and helpful for the improved outcomes of these patients.


Assuntos
Proteínas Adaptadoras de Transdução de Sinal/genética , Mutação/genética , Espasmos Infantis/genética , Anticonvulsivantes/uso terapêutico , Diagnóstico Precoce , Feminino , Humanos , Lactente , Espasmos Infantis/diagnóstico , Espasmos Infantis/tratamento farmacológico , Resultado do Tratamento
11.
Medicine (Baltimore) ; 98(44): e17751, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31689830

RESUMO

Maternal bacterial sepsis during pregnancy and the postpartum period is a common cause of maternal mortality and fetal morbidity and mortality. This study was performed to analyze perinatal prognosis and related factors of maternal bacterial sepsis in west China.We conducted a cross-sectional study of pregnant women with bacterial sepsis who were admitted into a tertiary care center in western China between 2011and 2015. Data from these cases were collected and analyzed.Eighty six women were identified with bacterial sepsis in our hospital, and the incidence of maternal bacterial sepsis was 1.7 per 1000 maternities, the incidence of septic shock was 1.8 per 10,000 maternities, and 1 maternal death occurred. Among the 86 pregnant women with bacterial sepsis, genital tract infection was the most common source of infection (41/86, 47.7%). The most common bacteria in the Gram-positive bacteria group was Listeria monocytogenes and in the Gram-negative bacteria group was Escherichia coli. The premature delivery rate (65.7%) was substantially higher in the Gram-negative bacteria group (P = .011), and the miscarriage rate (31.3%) was higher in the Gram-positive bacteria group (P = .042). The fetal/neonatal mortality rate was 20% (21/105) and higher in the Gram-positive bacteria group (P = .008), and the infant mortality rate in 1 year was 7.1% (6/84).Bacterial sepsis remains an alarming cause of both maternal and fetal morbidity and mortality, and infant mortality. Key treatment involves a multi-disciplinary group of clinicians with experience in all aspects of the care of pregnant women with sepsis and early initiation of appropriate antibiotics according to the type of bacterial infection. The effect of maternal sepsis on long-term fetal outcome should be investigated.


Assuntos
Bactérias Gram-Negativas , Bactérias Gram-Positivas , Complicações Infecciosas na Gravidez/mortalidade , Infecções do Sistema Genital/mortalidade , Sepse/mortalidade , Aborto Espontâneo/microbiologia , Aborto Espontâneo/mortalidade , Adulto , China/epidemiologia , Estudos Transversais , Feminino , Humanos , Incidência , Lactente , Mortalidade Infantil , Recém-Nascido , Mortalidade Materna , Gravidez , Complicações Infecciosas na Gravidez/microbiologia , Infecções do Sistema Genital/microbiologia , Sepse/microbiologia
12.
Anticancer Res ; 39(11): 6389-6392, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31704872

RESUMO

BACKGROUND/AIM: Neurofibromatosis type 1 (NF1) is characterized by the occurrence of multisystem tumors, among which the most characteristic are optic pathway gliomas (OPGs) and plexiform neurofibromas (PNFs). With the development of new anticancer drugs targeting the immune system, it is important to examine the immunological status of patients with NF1. Furthermore, the immune system has been suggested as a probable modulator of NF1-associated phenotypes. The objective of this study was the analysis of lymphocyte subset populations with respect to the presence of PNFs and OPGs. PATIENTS AND METHODS: Fifty-three patients with NF1 diagnosed with OPG/PNF were analyzed for lymphocyte subpopulations. RESULTS: Significantly lower levels of B-cells, T-cells and natural killer (NK) cells were observed in the group of patients with PNFs compared to those with OPG. CONCLUSION: Our observation may indicate a correlation between weakened functioning of the immune system and the formation of PNFs.


Assuntos
Subpopulações de Linfócitos B/citologia , Células Matadoras Naturais/citologia , Neurofibroma Plexiforme/imunologia , Neurofibromatose 1/imunologia , Glioma do Nervo Óptico/imunologia , Subpopulações de Linfócitos T/citologia , Adolescente , Adulto , Idoso , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Contagem de Linfócitos , Masculino , Pessoa de Meia-Idade , Neurofibroma Plexiforme/etiologia , Neurofibromatose 1/complicações , Glioma do Nervo Óptico/etiologia
13.
Anticancer Res ; 39(11): 6413-6416, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31704875

RESUMO

BACKGROUND/AIM: The aim of this study was to present the clinical characteristics, natural history and survival outcomes of primary gastrointestinal non-Hodgkin lymphomas (PGINHL) in the pediatric population. PATIENTS AND METHODS: Surveillance, Epidemiology, and End Results (SEER) database was queried for patients aged 0 to 19 years with PGINHL between 1973 and 2014. RESULTS: A total of 452 cases were identified [mean age 11.0 (±5.1)] years, whites 84.1%, males (76.5%). The majority of tumors were noted in the small bowel (SB) (47.6%), followed by large bowel (LB) (28.5%) and the stomach (10.0%). Overall, the most common histological subtype was Burkitt lymphoma (51.8%), followed by diffuse large B-cell lymphoma (DLBCL) (26.1%). Mean overall survival (OS) of the entire cohort was 33,33 years with a 5-yr, 10-yr and 30-yr survival rate of 86%, 86% and 79%, respectively. Large bowel tumors had the best long-term survival rates whereas; gastric tumors had the worst with 30-yr survival rate 84% and 74%, respectively. Overall, 328 (72.6%) patients received surgery. No significant survival difference was noted between patients who underwent surgery and those who did not. CONCLUSION: This study presents the largest dataset of pediatric PGINHL and describes the clinical features and outcomes of these patients in addition to summarizing the literature.


Assuntos
Neoplasias Gastrointestinais , Linfoma não Hodgkin , Adolescente , Linfoma de Burkitt/mortalidade , Linfoma de Burkitt/patologia , Linfoma de Burkitt/cirurgia , Neoplasias do Ceco/mortalidade , Neoplasias do Ceco/patologia , Neoplasias do Ceco/cirurgia , Criança , Pré-Escolar , Feminino , Neoplasias Gastrointestinais/mortalidade , Neoplasias Gastrointestinais/patologia , Neoplasias Gastrointestinais/cirurgia , Humanos , Neoplasias do Íleo/mortalidade , Neoplasias do Íleo/patologia , Neoplasias do Íleo/cirurgia , Lactente , Recém-Nascido , Linfoma Difuso de Grandes Células B/mortalidade , Linfoma Difuso de Grandes Células B/patologia , Linfoma Difuso de Grandes Células B/cirurgia , Linfoma não Hodgkin/mortalidade , Linfoma não Hodgkin/patologia , Linfoma não Hodgkin/cirurgia , Masculino , Programa de SEER , Neoplasias Gástricas/mortalidade , Neoplasias Gástricas/patologia , Neoplasias Gástricas/cirurgia , Taxa de Sobrevida , Adulto Jovem
14.
Hua Xi Kou Qiang Yi Xue Za Zhi ; 37(5): 505-508, 2019 Oct 01.
Artigo em Chinês | MEDLINE | ID: mdl-31721498

RESUMO

OBJECTIVE: To study the clinical features and treatments of congenital submandibular duct dilatation. METHODS: Seven children with congenital submandibular duct dilatation from January 2008 to March 2018 were included in this study, whose average age was 5 months and 22 days. The clinical manifestations are unilateral swelling of the mouth floor. All seven children underwent sublingual gland resection, submandibular gland dilatation catheter resection, and catheter reroute under general anesthesia. Intraoperatively, the orifice of the submandibular gland was constricted and part of the catheter was dilated. RESULTS: All seven patients had good healing without swelling or cyst formation. CONCLUSIONS: Congenital submandibular duct dilatation occurs at a young age. Early diagnosis and treatment can help prevent further expansion of the catheter and avoid gland atrophy, feeding difficulty, and breathing obstruction. Simultaneous excision of the sublingual gland can avoid the formation of postoperative sublingual cyst.


Assuntos
Rânula , Ductos Salivares , Criança , Dilatação , Humanos , Lactente , Glândula Sublingual , Glândula Submandibular
15.
Pan Afr Med J ; 33: 254, 2019.
Artigo em Francês | MEDLINE | ID: mdl-31692805

RESUMO

Introduction: Postoperative complications are common; some are transient, others may be serious, but they are all important to patients. One of the most important gaps in surgical research is the lack of consensus over the preferred result, the type of measurement or of evaluation. Methods: We conducted a retrospective study of postoperative complications at the Surgery Department of the National Hospital Center of Nouakchott. Eight hundred thirty-four patients underwent surgery over a seven-month period (1 January 2017-31 July 2017). The demographic and clinical parameters were studied and analyzed statistically using SPSS software 20. Results: The study involved 834 patients, of whom 426 (51.1%) were men. The average age of patients was 34.81 years (1-90 years). Four hundred thirty-two (51.2%) patients underwent emergency surgery. The sex ratio (M/F) was 1.04. Arab-Berbers race accounted for 77.8%. Appendicular disease accounted for 41.12%, hepatobiliary disease accounted for 17.76%, and abdominal wall disease accounted for 16.1%). Thyroid disease accounted for 5.6%. One hundred eighty-three (21.94%) patients developed postoperative complications, including 4 (2.1%) deaths. Clavien-Dindo grade II was the most represented with 82.5% of complications and accounted for 17.91% of all operated patients. Surgical site infection accounted for 62.8% of all complications. Conclusion: This study shows that the Clavien-Dindo classification can be applied to patients who have undergone elective surgery and emergency surgery. We think that the lack of follow-up and the lack of means to fight the infection and the non-rigorous respect of asepsis and antisepsis procedures would play an important role.


Assuntos
Procedimentos Cirúrgicos Eletivos/estatística & dados numéricos , Emergências , Complicações Pós-Operatórias/epidemiologia , Infecção da Ferida Cirúrgica/epidemiologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Pessoa de Meia-Idade , Complicações Pós-Operatórias/classificação , Estudos Retrospectivos , Adulto Jovem
16.
Pan Afr Med J ; 33: 253, 2019.
Artigo em Francês | MEDLINE | ID: mdl-31692839

RESUMO

Introduction: sickle cell disease is a genetic disease with autosomal inheritance associated with haemoglobin structure abnormality which causes the formation of hemoglobin S. The purpose of our study was to collect data on digestive diseases in patients with sickle cell disease in Lubumbashi and to highlight their epidemiological and clinical features. Methods: We conducted a retrospective, descriptive, cross-sectional study at the Research Center for Sickle Cell Disease in Lubumbashi. All the records of patients on follow-up for sickle cell disease with digestive disease during our 3-year period (January 2015 to December 2017) were analyzed. Data were collected using a survey taking into account different study parameters including: age, sex, the reason for consultation, diagnosis, the type of vaso-occlusive crisis, the paraclinical examinations made, hydroxyurea treatment. Results: out of a total of 403 medical records examined we found 206 cases (n=206) of sickle cell disease associated with digestive disease, accounting for a rate of 51,11% of patients with sickle cell disease who suffered from digestive diseases. Both sexes were represented with a slight female predominance (51.94%) and a sex ratio M/F of 0.92. The most represented age ranges 1-6 years (32.52%), the average age was 11.8 years; the standard deviation was 21.9; the extreme ages were 13 months and 38 years. The reason for consultation was dominated by fever (60,67%), abdominal pain (44.66%) and digestive disorders (30,09%). Vaso-occlusive abdominal crises were found in 65 patients (31.55%) among whom 36 had only 1 crisis, 24 had 2 crises and 5 had 3 crises. Intestinal diseases were found in 121 patients (69,41%) dominated by intestinal parasites (found in 58 patients whose collection of stool samples showed 4 parasites: Yersinia enterocolitis, Entamoeba histolytica, Giardia intestinalis and Clostridium difficile). Gastric diseases were found in 105 patients ( 50,97%) divided into peptic ulcer (45 patients) and gastritis (60 patients); biliary vesicular disease was found in 40 patients (19.41%) including vesicular lithiasis without cholecystitis (32 patients), lithiasic cholecystitis (5 patients) and lithiasis in the main biliary tract (3 cases); there was 1 single case diagnosed with acute pancreatitis. The most common associated diseases in our study were respiratory diseases (169 cases;82,03%), oto-rhino-laryngological diseases (157 cases;76.21%), bony, vaso-occlusive crises (146 cases; 70,87%), urogenital diseases (64 cases; 31.06%) and malaria (51 patients; 24.75%). Hepatic diseases and diseases of the spleen were found in 18 cases (8.73%) and 47 cases (22,81%) respectively. Ultrasound was requested in 79 patients but only 31 of them underwent it because of the lack of financial means (it costs 20 U.S. dollars). In the case of clinically obvious splenomegaly, the search for Howell-Jolly bodies was requested in 23 patients but it was only performed in 2 patients because it costs 10 U.S. dollars). Routine blood count, hemoglobin, hematocrit, inflammatory assessment and thick drop examination were performed in all our patients but liver assessment, tests done on stool samples, urine test were recommended based on patient's complaint. Out of 206 patients, only 60 were under hydroxyurea treatment (29,16%). Conclusion: digestive diseases are common in patients with sickle cell disease and account for almost half of patients with diagnosed sickle cell disease. Unfortunately, best management is limited by poverty leading to less very useful paraclinical examinations in patients with digestive diseases resulting from sickle cell disease.


Assuntos
Anemia Falciforme/complicações , Doenças do Sistema Digestório/epidemiologia , Hidroxiureia/uso terapêutico , Dor Abdominal/epidemiologia , Dor Abdominal/etiologia , Adolescente , Adulto , Anemia Falciforme/tratamento farmacológico , Criança , Pré-Escolar , Estudos Transversais , República Democrática do Congo/epidemiologia , Doenças do Sistema Digestório/etiologia , Doenças do Sistema Digestório/fisiopatologia , Feminino , Humanos , Lactente , Masculino , Estudos Retrospectivos , Esplenomegalia/epidemiologia , Esplenomegalia/etiologia , Adulto Jovem
17.
Pan Afr Med J ; 33: 276, 2019.
Artigo em Francês | MEDLINE | ID: mdl-31692842

RESUMO

Cystic malformations of the bile ducts are rare congenital disorders, with an incidence of 1/2000000 live births. Complication including the angiocholitis, chronic pancreatitis, progressive biliary cirrhosis, portal hypertension or gallbladder lithiases can reveal severe disorder. Spontaneous perforation is one of the rare complications described for the first time in 1934 by Weber. We report the case of an 18-month old baby admitted with subocclusive syndrome with biliary peritonitis. Ultrasound was performed showing abdominal effusion with cystic formation communicating with the bile ducts associated with subcapsular effusion of the liver confirmed by a scanner. Treatment was based on peritoneal toilet with redon drain at the level of the perforation and subhepatic drain without cyst excision. The patient was re-admitted 6 months after this incident to be definitively treated.


Assuntos
Cisto do Colédoco/diagnóstico , Peritonite/diagnóstico , Cisto do Colédoco/terapia , Drenagem/métodos , Humanos , Lactente , Masculino , Peritonite/etiologia , Ultrassonografia
18.
Pan Afr Med J ; 33: 269, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31693717

RESUMO

Rotaviruses are one of the leading etiological agents of gastroenteritis in young children, for which a monovalent G1P(8) vaccine has been provided for free in Kenyan since July 2014. The main objective was to estimate the post vaccine prevalence and seasonal distribution of rotavirus diarrhea in children less than 5 years in Nairobi County, Kenya. Rotavirus positive samples were collected from children below 5 years of age in two hospitals within Nairobi County where vaccination status was card-confirmed. The children were examined and the demographic and clinical profiles of the children were recorded. Fecal specimens were analyzed for rotavirus antigen using an ELISA kit, followed by characterization by PAGE. Out of the total 323 samples, 49 had detectable rotavirus infection, representing 15.2% prevalence. Age distribution of rotavirus prevalence was as follows: ≤ 6 months-8.5%, 7-12 months-27.4%, 13-24 months - 41.4%, 25-36 months - 16.4% while 36-65 months had 6.3%. Rotavirus diarrhea was more common in wet and cold months of the year, the highest prevalence being observed in August (24.5%), 12.3% in both July and March, while April scored a prevalence of 10.2%. Out of the 49 rotavirus positive children, 48 had vomiting and abdominal cramps while all had fever and watery stool. The prevalence of Rotaviral diarrhea in children less than 5 years in Nairobi County Kenya has greatly reduced following the vaccine introduction and is more common during the wet and cold seasons of the year.


Assuntos
Diarreia/epidemiologia , Gastroenterite/epidemiologia , Infecções por Rotavirus/epidemiologia , Vacinas contra Rotavirus/administração & dosagem , Distribuição por Idade , Pré-Escolar , Diarreia/virologia , Feminino , Gastroenterite/virologia , Humanos , Lactente , Quênia/epidemiologia , Masculino , Prevalência , Infecções por Rotavirus/prevenção & controle , Estações do Ano
19.
Pan Afr Med J ; 33: 330, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31692697

RESUMO

Introduction: Globally, 50 million children under 5 were wasted; of which 16 million were severely wasted. A severely wasted child is at a nine times higher risk of dying. To prevent this problem, it is necessary to determine the magnitude and factors associated with childhood wasting. In Ethiopia specifically Wukro town, Tigray regional state there is no clear information regarding under five wasting. Therefore, the study assessed the prevalence and associated factors of wasting among under five children in Wukro town, North Ethiopia. Objective: to assess the prevalence of wasting and associated factors among under five children of Wukro town, Tigray, North Ethiopia, 2017/2018. Methods: Community based cross-sectional study design with a single population proportion formula was used with a total sample size of 400 children. Wukro town has three kebele, two kebelle were included in the study through simple random sampling method. There was proportional allocation of subjects to each kebelle and final study subject was selected using systematic method. In case there were more than one child in the household one child was selected randomly. The data were collected by face to face interview and measuring of weight and height after the instrument was pre-tested. The anthropometric results were entered in to Emergency nutritional assessment (ENA) to calculate Z-Score. The collected data and result of Z-score were entered in to Statistical package for social science (SPSS) version 20. Finally, results were presented in texts, graphs and tables. Results: A total of 394 under five children were participated in this study, which gave a response rate of 98.5%. The respondents were females 222 (56.3%) and 106(26.95%) were in the age group of 12-23 month. The overall prevalence of wasting was 28 (7.2%). Out of this 14 (3.6%) were wasted and 14 (3.6%) were severely wasted. Under five children those, whose family does not live together were 3.086 times more likely to be wasted compared to under five children those, whose family live together (P=.038, OR=3.086, & 95% CI= (1.061, 8.970). Under five children those, whose mother did not taken family planning were 2.530 times more likely to be wasted compared to under five children those, whose mother take family planning (P=.038, OR=2.530, & 95% CI= (1.054, 6.074)). Conclusion: Significant numbers of mothers were not taken extra food during pregnancy and lactation. There was significant prevalence of wasting of under five children in the study area. Living condition of family and usage of family planning were associated with increased risk of wasting.


Assuntos
Características da Família , Serviços de Planejamento Familiar/estatística & dados numéricos , Fenômenos Fisiológicos da Nutrição Pré-Natal , Síndrome de Emaciação/epidemiologia , Pré-Escolar , Estudos Transversais , Dieta , Etiópia/epidemiologia , Feminino , Humanos , Lactente , Recém-Nascido , Lactação , Masculino , Gravidez , Fatores de Risco
20.
Pan Afr Med J ; 33: 304, 2019.
Artigo em Francês | MEDLINE | ID: mdl-31692745

RESUMO

Vesicoureteral reflux is very common in children, requiring a long follow-up period to reduce its progression toward chronic renal failure. This study aims to analyze the epidemiological diagnostic features, the management of vesicoureteral reflux and long term course of patients with this disease. We conducted a retrospective study of 42 patients with suspected vesicoureteral reflux hospitalized in the Department of Visceral Paediatric Surgery at the Hassan II University Hospital of Fez over a period of 6 years from January 2010 to December 2015. Mean age of patients at diagnosis was 3 years and 2 months. The boy is to girl sex ratio was 1.8. Vesicoureteral reflux was isolated in 81% of patients and secondary or associated in 19% of patients. The most common initial manifestation was urinary tract infection (90.4%). Renal function was impaired in 54.8% of children. The treatment was based on antibiotics against diagnosed UTI (90.4%), antibiotic prophylaxis in the case of recurrence and Cohen reimplantation (97.62%). Surgical indication readily concerned all patients with grades IV and V vesicoureteral reflux (73.9%) as well as patients with impaired kidneys and 26.1% of patients after medical treatment. Early and late postoperative course was in general satisfactory: disappearance of vesicoureteral reflux in 92.68% of cases. Upper urinary tract regressed except for 9.52% of patients; 95.23% of patients had complete recovery of renal function. There was a reduction in UTI recurrence (in 19.04% of cases after surgery). The majority of parents judged positively the clinical course of their children (54.76%) after surgery.


Assuntos
Antibacterianos/administração & dosagem , Infecções Urinárias/etiologia , Refluxo Vesicoureteral/diagnóstico , Adolescente , Antibioticoprofilaxia/métodos , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Recidiva , Estudos Retrospectivos , Infecções Urinárias/epidemiologia , Infecções Urinárias/terapia , Refluxo Vesicoureteral/fisiopatologia , Refluxo Vesicoureteral/cirurgia
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