RESUMO
Arabinoxylans (AX) microspheres with different insulin/AX mass ratio were prepared by formation of phenoxy radical issued from the ferulic acid by enzymatic oxidation (entrapped in situ of insulin). Phenolic acid content and FT-IR spectrum of unloaded and insulin-loaded AX microspheres revealed that the phenoxy radical issued from the ferulic acid by enzymatic oxidation did not interact covalently with insulin. The microspheres showed a spherical shape, smooth surface and an average diameter of particles of 320⯵m. In vitro control release found that AX microspheres minimized the insulin loss in the upper GI tract, retaining high percentage (~75%) of insulin in its matrix. The stability of the secondary structure of insulin was studied by dichroism circular (CD). The CD spectra of insulin released from AX microspheres did not change according to the insulin/AX mass ratio of the microsphere. Significant hypoglycemic effects with improved insulin-relative bioavailability tested on an in vivo murine model revealed the efficacy of these enzymatically cross-linked arabinoxylans microspheres as a new oral insulin carrier.
Assuntos
Reagentes de Ligações Cruzadas/química , Sistemas de Liberação de Medicamentos , Insulina/administração & dosagem , Lacase/metabolismo , Microesferas , Xilanos/química , Administração Oral , Animais , Glicemia/metabolismo , Liberação Controlada de Fármacos , Humanos , Masculino , Ratos Wistar , Reologia , Espectroscopia de Infravermelho com Transformada de Fourier , Distribuição TecidualRESUMO
INTRODUCTION: Envenomation by black widow spiders manifests clinically with signs of neurotoxicity in paediatric patients. OBJECTIVE: Identify typical neurological signs and symptoms in paediatric patients of different ages, and describe treatment and outcomes in a paediatric hospital in northwest Mexico. MATERIAL AND METHODS: We reviewed 70 clinical records of patients hospitalised due to black widow spider bite between 1978 and 2014. We divided the total into 2 groups: Group 1, infants and preschool children; and Group 2, school-age children and adolescents. The demographic variables were age, sex, birthplace, place where envenomation occurred, body part(s) affected, degree of envenomation according to signs and symptoms, treatment, clinical outcome, and statistical differences. RESULTS: Boys accounted for 61.4% of all cases, and infants younger than one year old made up 14.2%. Most patients (70%) were bitten by the spider at home; the anatomical areas most frequently affected were the legs, neck, thorax, and abdomen. The neurological signs and symptoms displayed by Group 1 were irritability, constant crying, sialorrhoea, nausea, tachycardia, arrhythmias, fatigue when walking, agitation, muscle spasms paraesthesia, tetany, seizures, and nystagmus. Signs in Group 2 included localized pain, headache, sialorrhoea, paraesthesia, profuse sweating, anxiety, muscle weakness, muscle spasms, and fine tremor. The predominant autonomic sign in Group 1 was sialorrhoea (P<.0001) and in Group 2, paraesthesia (P<.0001). Patients who received Fab antivenom treatment displayed better outcomes and shorter hospital stays than those who did not. No deaths were reported. CONCLUSIONS: The neurological signs and symptoms caused by black widow spider bite are predominantly autonomic, and identifying them permits early diagnosis and more effective treatment.
Assuntos
Viúva Negra , Síndromes Neurotóxicas/etiologia , Síndromes Neurotóxicas/fisiopatologia , Picada de Aranha/complicações , Picada de Aranha/fisiopatologia , Adolescente , Animais , Antivenenos/uso terapêutico , Doenças do Sistema Nervoso Autônomo/etiologia , Doenças do Sistema Nervoso Autônomo/fisiopatologia , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , México/epidemiologia , Doenças do Sistema Nervoso/etiologia , Síndromes Neurotóxicas/epidemiologia , Picada de Aranha/epidemiologia , Resultado do TratamentoRESUMO
BACKGROUND: Celiac disease (CD) is an autoimmune enteropathy induced by dietary wheat gluten that can have serious consequences if not diagnosed and treated early. It is important to be familiar with other alterations associated with gluten ingestion due to the multiplicity of clinical presentations. OBJECTIVES: To describe the most common CD presentation patterns and alterations associated with gluten in children from the northwest region of Mexico, with an incipient knowledge of its prevalence. PATIENTS AND METHODS: Age, sex, family history, and gastrointestinal and extraintestinal symptoms were recorded in 24 patients within the time frame of 2006 to 2010. Biochemical and hematologic data were collected. Anti-gliadin and anti-transglutaminase antibodies were analyzed in all the cases, and haplotypes (HLA-DQ2/DQ8) and duodenal biopsy were evaluated in some of the cases. RESULTS: Of the 24 patients (14 girls and 10 boys), 13 presented with typical CD with symptoms of poor gastrointestinal absorption; 7 patients with a mean age of 5 years presented with atypical CD; 2 had disease onset with gastrointestinal and extraintestinal (neurologic) problems; and 2 with other gluten-related disorders. All of the patients had positive serology; 11/15 presented with HLA-DQ2/DQ8 and 4 with at least one allele; damaged mucosa was observed in the 6 biopsies taken. A third of the patients were anemic, 6 presented with an albumin value of<3.5g/dL, and 4 with mineral deficiencies. A total of 83% of the patients improved with a gluten-free diet. CONCLUSIONS: The presentation patterns were: 1) typical CD, 2) atypical CD, 3) CD with gastrointestinal and extraintestinal (neurologic) symptoms, and 4) gluten-related disorders other than CD.
Assuntos
Doença Celíaca/epidemiologia , Adolescente , Doença Celíaca/patologia , Doença Celíaca/terapia , Pré-Escolar , Feminino , Glutens/imunologia , Antígenos HLA-DQ/análise , Haplótipos , Humanos , Lactente , Absorção Intestinal , Mucosa Intestinal/patologia , Masculino , México/epidemiologiaRESUMO
Human nephrotic syndrome has been related to mutations in glomerular proteins. Mutations in the NPHS2 gene that encodes podocin have been described as responsible for steroid-resistant nephrotic syndrome. It has been advised to test for NPHS2 mutations in parallel or before giving steroid treatment in nephrotic syndrome patients in order to avoid unnecessary therapy. We identified NPHS2 mutations in Mexican children with nephrotic syndrome. The study included 13 children with nephrotic syndrome and 2 healthy control individuals; 8 patients were steroid-resistant and 5 were steroid-sensitive. We analyzed the 3rd exon of NPHS2 by DNA sequencing. Podocin heterozygous missense mutations L139R and L142P were found; the former was found in both steroid-sensitive and steroid-resistant children, while the latter was found in a steroid-resistant child. We conclude that NPHS2 mutations should be investigated to help decide the course of treatment in nephrotic syndrome patients.
Assuntos
Peptídeos e Proteínas de Sinalização Intracelular/genética , Proteínas de Membrana/genética , Síndrome Nefrótica/congênito , Prednisona/administração & dosagem , Estudos de Casos e Controles , Pré-Escolar , Esquema de Medicação , Evolução Molecular , Éxons , Humanos , Peptídeos e Proteínas de Sinalização Intracelular/química , Peptídeos e Proteínas de Sinalização Intracelular/metabolismo , Proteínas de Membrana/química , Proteínas de Membrana/metabolismo , Mutação de Sentido Incorreto , Síndrome Nefrótica/tratamento farmacológico , Síndrome Nefrótica/genética , Prednisona/uso terapêutico , Alinhamento de Sequência , Análise de Sequência de DNARESUMO
There were reviewed 145 cases of children in which hepatic biopsy was done at the Hospital Infantil del Estado de Sonora, from 1978 to 1990. The larger age group were infants and preschool children (74.3 percent) males being predominant; signs and symptoms were related with hepatic illness, as well as the admission diagnoses. The indication of biopsy was for confirmation of liver disease in more than 50 percent, 37.1 percent for unknown diagnoses and 20.6 percent to look for liver disease by a systemic illness. The most usual procedure was percutaneous biopsy with Vim-Silverman needle in 111 cases (76.5 percent), in 23 percent, the biopsy was done by major surgical method. Nine percent of the children needed open surgical method after percutaneous biopsy. The time from the admission to biopsy performance in patients with neonatal hepatitis vs biliary atresia was 14 days. In other type of illness the time was 25 days. The morbidity was 1 percent. There was no mortality. The histopathologic diagnosis of liver diseases was done in 96 cases (66.7 percent) by this method in 31 children (21.3 percent) with investigation of jaundice (neonatal hepatitis vs biliary atresia). The diagnostic mistake in tissues obtained by percutaneous needle, was statistically significant (p < 0.05). Average hospitalization stay was less than two months in 70 percent of the cases.
Assuntos
Biópsia por Agulha , Hepatopatias/patologia , Fígado/patologia , Adolescente , Biópsia por Agulha/instrumentação , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Estudos RetrospectivosRESUMO
We have reviewed sixty three cases of patients with hepatosplenomegaly admitted at the internal medicine service at the Hospital Infantil del Estado de Sonora over a period of the ten years. We didn't found differences in the number of male and female children. The larger groups were from 1 month to 6 years old age. The nutrition status were normal in more of 80% of the patients. The time that children spent at the hospital for diagnosis was less than 15 days in more than half of the cases. The symptoms predominating and clinical features were paleness fever and adenomegalies. Basic laboratory and other studies were made in a initial period, phase I (CBS, urinalysis serum, urea serum creatinine, serum glutamic oxalacetic and piruvic transaminases, and coccidioidine skin test, monotest, ASL, cytomegalovirus and rubella antibodies, Sabin-Feldman test, VDRL and chest, abdominal and paranasal sinus x-Ray) we reached a definitive diagnosis with this laboratory methods in 41.2% of the cases. A percutaneous hepatic biopsy was a rewarding accurate diagnostic procedure in 19 of 21 patients. The diagnosis that we established in general groups were: infections diseases, oncologic disease, metabolic disturbances, primary unknown hepatic disease, hematologic disorders and congestive diseases. In this letter we want to show our experience in a second level pediatric hospital in a mexican province so that this work can be reviewed by other Physicians and they can offer a better attention for this patients.
Assuntos
Hepatomegalia/diagnóstico , Esplenomegalia/diagnóstico , Criança , Pré-Escolar , Diagnóstico Diferencial , Feminino , Hepatomegalia/epidemiologia , Hepatomegalia/etiologia , Humanos , Lactente , Masculino , México/epidemiologia , Estudos Retrospectivos , Esplenomegalia/epidemiologia , Esplenomegalia/etiologiaRESUMO
The Pelger-Huet anomaly is a benign disorder which affects the morphology of the granulocytes and is namely inherited as an autosomic dominant trait. The frequency of this anomaly varies from country to country and in México this constitutes the sixth family reported. This is a case of a 21-month-old boy whose anomaly was detected during the study of his ferropenic anemia with which he arrived at the hospital. During the study of his nine family members, the anomaly was found to have also been present in his father. An electron microscopy study of the subject, his father's and a normal control's neutrophils was conducted. The patient's and his father's leucocyte granules were found to be normal but decreased in number. This work includes some discussion on inherited traits, their presentation, morphology, differential diagnosis and emphasizes the importance in recognizing this anomaly and the promotion of genetic counselling.
Assuntos
Anomalia de Pelger-Huët/diagnóstico , Adulto , Núcleo Celular/ultraestrutura , Grânulos Citoplasmáticos/ultraestrutura , Diagnóstico Diferencial , Humanos , Lactente , Masculino , México , Microscopia Eletrônica , Neutrófilos/ultraestrutura , Linhagem , Anomalia de Pelger-Huët/sangue , Anomalia de Pelger-Huët/genéticaAssuntos
Atrofia Muscular/complicações , Doenças da Medula Espinal/complicações , Células do Corno Anterior/patologia , Atrofia , Diagnóstico Diferencial , Feminino , Humanos , Lactente , Masculino , Hipotonia Muscular/diagnóstico , Atrofia Muscular/diagnóstico , Doenças da Medula Espinal/diagnóstico , Doenças da Medula Espinal/patologia , SíndromeRESUMO
La atrofia espinal infantil progresiva o enfermedad de Werdnig-Hoffmann (EWH) es un desorden hereditario que afecta nucleos motores del asta anterior de la medula; se distinguen tres tipos clinicos con comportamiento especifico. Sin embargo los ninos con este padecimiento son frecuentemente diagnosticados como portadores de miopatias congenitas o paralisis cerebral infantil. Hasta ahora no existen metodos especificos de diagnostico, pues las alteraciones que se detectan son compartidas por diversas enfermedades.En la observacion de nuestros casos y por lo referido en la literatura medica, es fundamental el juicio clinico para considerar esta entidad. A la fecha, no existen perspectivas de tratamiento y los pacientes llegan inexorablemente a la muerte. El tiempo de sobrevida depende tanto del tipo clinico como de las medidas de sosten para prolongar la existencia
Assuntos
Lactente , Humanos , Masculino , Feminino , Doenças Desmielinizantes , Doenças da Medula Espinal , Neurônios Motores , Atrofia Muscular , ParalisiaRESUMO
La hemosiderosis pulmonar idiopatica (HPI) es una entidad clinica de presentacion poco frecuente; tiene estigmas clinicos que en el curso de su evolucion pocas veces tomamos en cuenta, a pesar de que estos son senalados insistentemente en las diversas publicaciones de la literatura medica; nos referimos basicamente a la presencia de tos cronica, anemia hipocromica ferropenica, disnea, expectoracion hemoptoica e infiltrado pulmonar difuso, con imagenes de condensacion del espacio aereo, rapidamente cambiantes y evolucion a fibrosis intersticial.Esta situacion nos motivo a revisar el numero de casos existentes en el Hospital del Nino del Noroeste DIF, en sus primeros cinco anos de labores (1977-1982). Encontramos tres casos de pacientes que reunieron los requisitos minimos para catalogarlos bajo el diagnostico de HPI, descartandose previamente patologias tales como cardiopatias congenitas, lupus eritematoso, anemias hemoliticas talasemias e infecciones cronicas. En este trabajo informamos los rasgos mas sobresalientes, sobre evolucion, estudios para diagnostico y tratamiento, a la vez sue se vierten los conceptos mas aceptables acerca de la etiopatogenia
Assuntos
Pré-Escolar , Adolescente , Humanos , Feminino , Hemossiderose , Prednisona , Fibrose PulmonarRESUMO
Se presenta un caso de enfermedad de Gaucher estudiado en el Hospital del Nino del Noroeste, D.I.F. Las caracteristicas clinicas mas sobresalientes en este paciente de 15 anos de edad fueron: dolor articular con remisiones y exacerbaciones que cedia a la administracion de analgesicos, acompanandose de aumento de volumen moderado y deformidad progresiva de ambas rodillas a lo largo de 8 anos. El nino fue internado para estudios encontrando hepatosplenomegalia; se le practicaron diversos examenes incluyendo biopsia hepatica, la cual mostro celulas de Gaucher tipicas y la ultrastructura comprobo la presencia de depositos caracteristicos de la enfermedad de Gaucher. En el presente articulo se vieron los conceptos actuales del padecimiento en relacion a aspectos clinicos, fisiopatogenia, alteraciones enzimaticas, histologia, ultrastructura celular y tratamiento
Assuntos
Adolescente , Humanos , Doença de GaucherRESUMO
Se presentan dos casos de hiperbilirrubinemia familiar no conjugada (enfermedad de Gilbert), detectada en dos hermanos, uno del sexo masculino de 12 anos de edad, con manifestacion clinica (ictericia) apreciada desde la primera semana de vida, con remision y reaparicion a los 5 anos, cursando posteriormente asignologico y asintomatico hasta su admision al hospital; el otro, una hermana de 15 anos de edad cuyos sintomas se iniciaron a los 12 anos. Se practicaron diversos examenes de laboratorio tendientes a descartar padecimientos tales como anemias hemoliticas, hepatitis persistente, enfermedad de Crigler-Najjar, Dubin-Johnson y sindrome de Rotor.Se practico biopsia hepatica y estudio de medula osea resultado normales. El unico parametro de laboratorio alterado fue elevacion de bilirrubina no conjugada de manera moderada hasta 6 mg/dl; por exclusion se diagnostico enfermedad de Gilbert, instalandose prueba terapeutica con excelentes resultados
Assuntos
Criança , Adolescente , Humanos , Masculino , Feminino , Doença de GilbertRESUMO
Se presentan dos casos de pacientes que cursaron con carcada leucocitosis y linfocitosis extrema. Los estudios iniciales, especificamente la biometria hematica, hizo sospechar leucemia aguda; sin embargo, los examenes tendientes a demostrarla, condujeron al diagnostico de linfocitosis infecciosa aguda. La evolucion clinica observada en los dos ninos fue buena, apreciandose normalizacion en los conteos hematicos (leucocitos, linfocitos), en un periodo de seis semanas