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1.
Adv Exp Med Biol ; 1232: 77-83, 2020.
Artículo en Inglés | MEDLINE | ID: mdl-31893397

RESUMEN

Instantaneous orthostatic hypotension (INOH) is one of the main types of orthostatic dysregulation in children and adolescents. In patients with INOH arterial pressure drops considerably after active standing and is slow to recover. We investigated changes in cerebral oxygenation in the bilateral prefrontal cortex during an active standing test in juvenile INOH patients to evaluate changes in cerebral oxygen metabolism. We enrolled 82 INOH patients (mean age 13.8 ± 2.2 years, 52 mild and 30 severe patients) at Nihon University Itabashi Hospital from October 2013 to April 2018. We measured cerebral oxygenated hemoglobin, deoxygenated hemoglobin, and total hemoglobin levels in the bilateral prefrontal cortex using near-infrared spectroscopy during an active standing test. In severe INOH patients, cerebral oxygenation of the right prefrontal cortex remained constant when blood pressure dropped; however, de-oxy-Hb significantly increased. These findings confirm that there is asymmetrical autoregulation between the right and left prefrontal cortex.


Asunto(s)
Circulación Cerebrovascular , Hipotensión Ortostática , Adolescente , Circulación Cerebrovascular/fisiología , Niño , Homeostasis , Humanos , Hipotensión Ortostática/fisiopatología , Oxihemoglobinas , Espectroscopía Infrarroja Corta
2.
Artículo en Inglés | MEDLINE | ID: mdl-30995366

RESUMEN

Objective: Orthostatic intolerance (OI) is an important health problem for children and adolescents. The onset and exacerbation of OI are strongly affected by psychosocial factors. Intestinal microbial deviations, which are affected by food and lifestyle factors, are an important risk factor for adult psychiatry patients, but their roles in pediatric patients are unclear. The objective of this study was to investigate the intestinal microbiota and its involvement in the mental health of children with OI. Methods: Fifty-six fecal samples from pediatric OI patients and 9 samples from healthy children were examined with terminal-restriction fragment length polymorphism analysis from July 2016 to January 2018 at Nihon University Itabashi Hospital, Tokyo, Japan. Bacterial diversity was analyzed using the Shannon-Wiener index and the Simpson index. All OI patients were assessed using 2 different psychological scales: the Children's Depression Inventory and the Children's Manifest Anxiety Scale. The patients were then divided into the following subgroups: depression or nondepression and anxiety or nonanxiety. Results: The mean proportion of Clostridium subcluster XIVa and/or Enterobacteriaceae (operational taxonomic unit [OTU] 940) in the OI patients was significantly higher than that in the healthy controls (P = .02). Among OI patients, Bifidobacterium (OTU 124) was less frequent in the depression group than in the nondepression group. However, depression and anxiety showed no correlation with bacterial diversity. Conclusions: Pediatric OI patients showed deviations in the intestinal bacterial flora. The intestinal flora can serve as a novel therapeutic target for the mental health management of intractable pediatric OI patients.


Asunto(s)
Ansiedad/microbiología , Depresión/microbiología , Heces/microbiología , Microbioma Gastrointestinal , Intolerancia Ortostática/microbiología , Adolescente , Ansiedad/complicaciones , Niño , Depresión/complicaciones , Femenino , Humanos , Masculino , Intolerancia Ortostática/complicaciones
3.
Brain Dev ; 41(6): 559-562, 2019 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-30772096

RESUMEN

BACKGROUND: Confusional migraine is a rare type of migraine presenting as an acute confusional state. However, the mechanism of this confusional state remains unclear. SUBJECT AND METHODS: We examined an 11-year-old girl with confusional migraine, using electroencephalography, brain magnetic resonance imaging, cerebrovascular magnetic resonance angiography, and single-photon emission computed tomography to investigate cerebral blood flow changes. RESULTS: Our findings revealed vessel narrowing in the left middle and posterior cerebral artery territory, indicating vasospasm and suggesting that the confusion was caused by hypoperfusion. However, abnormal increased cerebral blood flow in the left middle and posterior cerebral artery territory was observed during the non-confusional state. CONCLUSION: The recorded cerebral blood flow changes are similar to those associated with migraine attacks, gradually changing from abnormally low to abnormally high during the confusional and post-confusional state.


Asunto(s)
Circulación Cerebrovascular/fisiología , Trastornos Migrañosos/fisiopatología , Niño , Confusión/fisiopatología , Electroencefalografía/métodos , Femenino , Humanos , Angiografía por Resonancia Magnética/métodos , Imagen por Resonancia Magnética/métodos , Trastornos Migrañosos/sangre , Trastornos Migrañosos/complicaciones , Tomografía Computarizada de Emisión de Fotón Único/métodos
5.
Pediatr Emerg Care ; 33(6): 418-421, 2017 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-27228148

RESUMEN

Acute focal bacterial nephritis (AFBN) is a localized bacterial infection of the kidney presenting as an inflammatory mass without frank abscess formation. In children, most patients with AFBN present with nonspecific conditions, such as fever, vomiting, and abdominal pain. A small number of reported cases are accompanied by neurological symptoms, including meningeal irritation, unconsciousness, and seizures. We experienced 2 rare cases of AFBN associated with central nervous system lesions. The first case was a 3-year-old girl who had neurological symptoms, including unconsciousness and seizures, with AFBN associated with acute reversible encephalopathy. The second case was a 5-year-old girl who had neurological symptoms, including unconsciousness, with AFBN accompanied by clinically mild encephalitis/encephalopathy with a reversible splenial lesion.


Asunto(s)
Infecciones Bacterianas/complicaciones , Encefalopatías/complicaciones , Sistema Nervioso Central/patología , Encefalitis/complicaciones , Nefritis/microbiología , Antibacterianos/uso terapéutico , Infecciones Bacterianas/tratamiento farmacológico , Encefalopatías/tratamiento farmacológico , Sistema Nervioso Central/diagnóstico por imagen , Líquido Cefalorraquídeo/química , Líquido Cefalorraquídeo/citología , Preescolar , Imagen de Difusión por Resonancia Magnética/métodos , Femenino , Humanos , Nefritis/diagnóstico , Tomografía Computarizada por Rayos X/métodos , Resultado del Tratamiento
6.
Pediatr Int ; 57(5): 922-9, 2015 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-25922882

RESUMEN

BACKGROUND: The aim of this preliminary study was to investigate the clinical use of acute-stage susceptibility-weighted imaging (SWI) in children with prolonged convulsive disorder. METHODS: Ten children with prolonged convulsive disorder who underwent SWI within 2 h after termination of seizure (acute-stage SWI group) and 15 control children who underwent SWI > 2 h after their seizures terminated or for other purposes were enrolled. The cerebral venous vasculature was compared between the groups. The acute-stage SWI group was further divided into three subgroups: normal group, those with regional low signals in the cerebral veins (regional group) and those with diffuse low signals in the cerebral veins (generalized group). Inter-ictal electroencephalography (EEG) and venous blood gas findings during seizure activity were compared between these subgroups. RESULTS: All patients in the acute-stage SWI group had low cerebral vein signal. Four patients were assigned to the regional group and six patients to the generalized group. Decrease of venous pH and the increase of venous pCO2 during seizure activity was more prominent in the regional group than in the generalized group. In the regional group, low-signal areas in the cerebral veins were consistent with abnormal areas on EEG; these low-signal areas resolved completely in all patients on follow-up SWI. Ten patients in the control group had normal SWI, and five had a generalized low signal. CONCLUSIONS: Acute-stage SWI may be a useful alternative for identifying lateralization of seizures in children with prolonged convulsive disorder.


Asunto(s)
Encéfalo/patología , Imagen de Difusión por Resonancia Magnética/métodos , Convulsiones/diagnóstico , Adolescente , Niño , Preescolar , Electroencefalografía , Femenino , Estudios de Seguimiento , Humanos , Lactante , Recién Nacido , Masculino , Reproducibilidad de los Resultados , Estudios Retrospectivos
7.
Obes Res Clin Pract ; 9(1): 31-4, 2015.
Artículo en Inglés | MEDLINE | ID: mdl-25660172

RESUMEN

To investigate the effects of n-3 polyunsaturated fatty acids on stearoyl-CoA desaturase (SCD) activity, we treated 10 obese children (mean age: 12.9 years) with cod liver oil once daily for 12 weeks. The effects of cod liver oil supplementation on SCD activity, as estimated by the palmitoleate/palmitate ratio, depended on the docosahexaenoic acid (DHA) contents at baseline. Baseline DHA contents were negatively correlated with baseline SCD activity. After the treatment, baseline DHA contents were found to be significantly associated with the reduction of SCD activity. Cod liver oil supplementation may be a complementary treatment for obese children with low baseline contents of DHA.


Asunto(s)
Aceite de Hígado de Bacalao/uso terapéutico , Ácidos Docosahexaenoicos/sangre , Síndrome Metabólico/dietoterapia , Obesidad Pediátrica/dietoterapia , Fosfolípidos/sangre , Estearoil-CoA Desaturasa/sangre , Niño , Suplementos Dietéticos , Femenino , Humanos , Masculino , Síndrome Metabólico/sangre , Obesidad Pediátrica/sangre , Fosfolípidos/química , Proyectos Piloto , Estearoil-CoA Desaturasa/efectos de los fármacos , Resultado del Tratamiento
8.
Pediatr Neurol ; 51(4): 550-2, 2014 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-25266617

RESUMEN

BACKGROUND: Wolf-Hirschhorn syndrome is a congenital malformation syndrome resulting from deletion of the short arm of chromosome 4. Individuals with Wolf-Hirschhorn syndrome may have a "Greek warrior helmet" appearance, growth retardation, developmental delay, muscular hypotonia, epilepsy, and difficulty with language including verbal communication. An affinity for music has not previously been reported in these patients. PATIENTS: We describe two patients with Wolf-Hirschhorn syndrome who both have a strong affinity for music. One patient is a 20-year-old woman who likes to listen to music all day and can hum many tunes. The other patient is a 9-year-old girl who is calmed by music and received music therapy, with subsequent improvement in her communication skills (eye contact, joint attention, and vocalizations to request music). CONCLUSIONS: Individuals with Wolf-Hirschhorn syndrome may have a strong affinity for music and may benefit from music therapy. Additional studies are needed to investigate the interest in music in individuals with Wolf-Hirschhorn syndrome.


Asunto(s)
Música/psicología , Síndrome de Wolf-Hirschhorn/psicología , Adulto , Niño , Femenino , Humanos , Musicoterapia , Síndrome de Wolf-Hirschhorn/terapia , Adulto Joven
9.
Adv Exp Med Biol ; 812: 253-261, 2014.
Artículo en Inglés | MEDLINE | ID: mdl-24729241

RESUMEN

Orthostatic dysregulation (OD) has been classified into subtypes by heart rate and blood pressure; however, the hemodynamics of brains have not yet been revealed. Therefore, we investigated changes in cerebral blood flow and oxygenation during an active standing test to clarify the pathophysiology of two subtypes: postural tachycardia syndrome (POTS) and neurally mediated syncope (NMS). We studied 31 children (15 boys, 16 girls; mean age, 14.0 ± 1.7 years) who presented with OD at the Department of Pediatrics and Child Health, Nihon University School of Medicine between 2009 and 2011. OD was diagnosed using the Japanese clinical guidelines for juvenile orthostatic dysregulation. After a 10-min resting period in the supine position, patients were asked to quickly stand up and keep upright for 10 min. Cerebral blood flow and cerebral oxygenation were measured using transcranial Doppler sonography and near-infrared spectroscopy. POTS showed a significant decrease of oxy-Hb and resistance index (RI), suggesting transient ischemia with maintainable cerebral autoregulation. NMS showed a decrease of oxy-Hb and an increase of RI, suggesting ischemia and impairment of autoregulation.


Asunto(s)
Circulación Cerebrovascular , Oxígeno/sangre , Síndrome de Taquicardia Postural Ortostática/sangre , Postura , Síncope/sangre , Niño , Femenino , Humanos , Masculino
10.
Adv Exp Med Biol ; 812: 271-278, 2014.
Artículo en Inglés | MEDLINE | ID: mdl-24729243

RESUMEN

Near-infrared spectroscopy enables recognition of various brain conditions based on certain factors, such as oxygenated hemoglobin (oxy-Hb). Since July 2012, we have been trying to determine the mechanisms of autonomic function in Japanese children with orthostatic intolerance (also called orthostatic dysregulation) in Nihon University Itabashi Hospital in Tokyo, Japan. A total of 23 children aged 7-16 years diagnosed with postural tachycardia syndrome (POTS), a subtype of orthostatic dysregulation, were enrolled in the study. We evaluated the relation between asymmetry in frontal cortex activity and the automatic nervous system and compared oxy-Hb changes in the right and left frontal cortices during an active standing test. We observed that during active standing oxy-Hb decreased in the frontal cortex. The oxy-Hb changes were asymmetrical, with a significantly larger decrease in the left frontal cortex than in the right frontal cortex, suggesting that tachycardia during active standing in POTS patients might be caused by activation of the right frontal cortex, which induces sympathetic nervous system activity.


Asunto(s)
Encéfalo/irrigación sanguínea , Oxígeno/sangre , Síndrome de Taquicardia Postural Ortostática/sangre , Postura , Adolescente , Niño , Femenino , Humanos , Masculino
11.
Ann Nucl Med ; 28(5): 498-503, 2014 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-24647992

RESUMEN

The Japanese Society of Nuclear Medicine has recently published the consensus guidelines for pediatric nuclear medicine. This article is the English version of the guidelines. Part 1 proposes the dose optimization in pediatric nuclear medicine studies. Part 2 comprehensively discusses imaging techniques for the appropriate conduct of pediatric nuclear medicine procedures, considering the characteristics of imaging in children.


Asunto(s)
Consenso , Diagnóstico por Imagen/métodos , Medicina Nuclear/métodos , Pediatría/métodos , Dosis de Radiación , Radiofármacos/administración & dosificación , Anestesia , Peso Corporal , Humanos , Japón , Restricción Física , Micción
12.
No To Hattatsu ; 46(1): 16-21, 2014 Jan.
Artículo en Japonés | MEDLINE | ID: mdl-24620425

RESUMEN

OBJECTIVE: We investigated the clinical efficacy and pharmacokinetics of lamotrigine (LTG) as an add-on therapy in childhood-onset intractable epilepsy. METHODS: We reviewed the charts of 28 outpatients who had received LTG as an add-on therapy. The data collected included epilepsy type, seizure frequency, concomitant anti-epileptic drugs, dosage of LTG and LTG serum levels. Furthermore, we reviewed the relationship between the LTG serum levels (microg/ml) and dosage of LTG (mg/kg/day), as well as the relationship between the LTG serum levels (microg/ml) and clinical efficacy in the following 2 groups:the valproate sodium (VPA) combination group and the non-VPA combination group. RESULTS: A reduction of 50% or more in seizure frequency was observed in 10 patients. In addition, there was a high correlation between the LTG serum levels and the dosage of LTG in each group. In the VPA combination group, the average of LTG serum levels in patients with adequate therapeutic response (50% reduction in seizure frequency) was higher than that in patients without adequate therapeutic response. In the non-VPA combination group, the average LTG serum level in adequate response patients was lower than that in patients without adequate therapeutic response. However, the epilepsy types of adequate response patients differed in the two groups. CONCLUSIONS: The LTG serum level is predictable based on the dosage of LTG. It was judged that the effective blood concentration of LTG differed when used with VPA, although factors other than the combined use of VPA should have been taken into consideration also.


Asunto(s)
Convulsiones/tratamiento farmacológico , Triazinas/farmacocinética , Adolescente , Edad de Inicio , Niño , Quimioterapia Combinada/métodos , Femenino , Humanos , Lamotrigina , Masculino , Resultado del Tratamiento , Triazinas/uso terapéutico , Ácido Valproico/administración & dosificación , Ácido Valproico/uso terapéutico
13.
J Infect Chemother ; 20(2): 134-8, 2014 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-24462436

RESUMEN

Mycoplasma pneumoniae is a common cause of respiratory tract illness in children. Among the most common extrapulmonary manifestations are disorders of the central nervous system, including meningitis, meningoencephalitis, cerebellitis, polyneuropathy, acute disseminated encephalomyelitis, and Guillain-Barré syndrome. Guillain-Barré syndrome, also known as acute inflammatory demyelinating polyradiculoneuropathy, is an acute-onset, immune-mediated disorder of the peripheral nervous system. The central nervous system is usually intact in patients with Guillain-Barré syndrome. However, there have been some reports of an association of Guillain-Barré syndrome with central nervous system involvement in children. We report a 3-year-old boy with M. pneumoniae infection associated with Guillain-Barré syndrome and encephalitis. Both serum anti-GM1 ganglioside (IgG and IgM) and anti-galactocerebroside IgG antibodies were detected in our patient: the former in the earlier stage of the disease, and the latter in the later stage. We speculate that anti-GM1 ganglioside was associated more with encephalitis, and anti-galactocerebroside antibody was associated more with GBS in our case. Our patient is the youngest report of Guillain-Barré syndrome with central nervous system involvement, and the first report of a pediatric patient with associated M. pneumoniae infection. Such cases are rarely reported, but highlight the need for awareness of the association of the infection with Guillain-Barré syndrome with central nervous system involvement.


Asunto(s)
Encefalitis/microbiología , Síndrome de Guillain-Barré/microbiología , Neumonía por Mycoplasma/complicaciones , Preescolar , Humanos , Masculino
14.
Pediatr Neurol ; 50(2): 171-6, 2014 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-24262343

RESUMEN

BACKGROUND: Susceptibility-weighted imaging is a novel high-spatial-resolution three-dimensional gradient-echo magnetic resonance imaging technique with phase postprocessing that accentuates the paramagnetic properties of blood products. The use of susceptibility-weighted imaging for epileptic focus localization in the acute stage of encephalopathy in a child has not been documented. PATIENTS: We report three pediatric patients with status epilepticus in the setting of fever, in whom susceptibility-weighted imaging showed transient prominence of the focal venous vasculature. RESULTS: Conventional cranial T1- and T2-weighted images and diffusion-weighted images showed no abnormalities. The prominence of the focal venous vasculature in these patients, as demonstrated by susceptibility-weighted imaging, was consistent with the epileptic focuses suggested by both clinical symptoms and electroencephalograph findings and resolved completely without neurological sequelae in all patients. CONCLUSIONS: Susceptibility-weighted imaging may facilitate assessing epileptic focus localization in the acute stage of encephalopathy in children.


Asunto(s)
Encéfalo/patología , Imagen por Resonancia Magnética/métodos , Estado Epiléptico/patología , Enfermedad Aguda , Encéfalo/fisiopatología , Niño , Preescolar , Imagen de Difusión por Resonancia Magnética , Electroencefalografía , Encefalitis Viral/patología , Encefalitis Viral/fisiopatología , Femenino , Humanos , Masculino , Infecciones por Roseolovirus/patología , Infecciones por Roseolovirus/fisiopatología , Convulsiones Febriles/patología , Convulsiones Febriles/fisiopatología , Estado Epiléptico/fisiopatología
15.
J Infect Chemother ; 19(1): 149-53, 2013 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-22569794

RESUMEN

Rotavirus is a common cause of severe gastroenteritis in children. It is known that rotavirus gastroenteritis may be accompanied by neurological manifestations, including encephalitis/encephalopathy and seizures. We report a case of a 4-year-old girl with clinically mild encephalopathy with a reversible splenial lesion associated with rotavirus infection. She was admitted to our hospital because of reduced level of consciousness, seizures, diarrhea, and vomiting. Fecal rotavirus antigen testing was positive. Cell counts in the cerebrospinal fluid (CSF) were normal. She had a normal serum sodium level on admission. Brain computed tomography showed no cerebral edema. However, electroencephalography showed generalized high-voltage slow waves, and diffusion-weighted magnetic resonance imaging demonstrated a transient abnormality in the splenium of the corpus callosum. We diagnosed clinically mild encephalopathy with a reversible splenial lesion associated with rotavirus infection. She recovered well and exhibited no neurological sequelae. Rotavirus RNA and antigen were not detected in the CSF, suggesting that the reversible splenial change was caused by indirect effects on the central nervous system subsequent to viral infection. Her normal serum sodium level indicates that this change can occur without hyponatremia.


Asunto(s)
Encefalopatías/patología , Cuerpo Calloso/patología , Gastroenteritis/complicaciones , Infecciones por Rotavirus/complicaciones , Rotavirus/aislamiento & purificación , Encefalopatías/virología , Preescolar , Imagen de Difusión por Resonancia Magnética , Electroencefalografía , Femenino , Gastroenteritis/virología , Humanos , Rotavirus/genética , Infecciones por Rotavirus/virología
16.
Pediatr Emerg Care ; 28(10): 998-1002, 2012 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-23023467

RESUMEN

OBJECTIVES: In April 2009, a novel influenza A (H1N1) pdm virus was identified in Mexico and spread quickly around the world. However, the clinical features of acute encephalopathy associated with 2009 pandemic influenza have not yet been elucidated. METHODS: We treated 8 patients (3 boys and 5 girls) aged 4 to 11 years (average age, 8 y 3 months) with influenza virus-associated encephalopathy, who presented at our 2 hospitals between July 2009 and March 2010. We investigated the clinical characteristics, treatments, and outcomes in the patients. RESULTS: In all patients, brain computed tomography showed mild to severe diffuse cerebral edema, and electroencephalography revealed diffuse high-voltage slow waves. They were all treated with oseltamivir and methylprednisolone pulse therapy. Six patients recovered without any sequelae; however, the remaining 2 had residual neurological sequelae. These 2 patients presented with severe disturbance of consciousness, and their central nervous system symptoms appeared within 12 hours after the onset of fever. One patient had periventricular leukomalacia and symptomatic epilepsy by perinatal brain hypoxia, and the other patient had 1 complex febrile and 2 febrile seizures. CONCLUSIONS: This study showed that patients with influenza-associated encephalopathy caused by influenza A (H1N1) pdm infection were all older than those with seasonal influenza. Underlying neurological disease or history may be associated with poor prognosis.


Asunto(s)
Encefalitis Viral/epidemiología , Subtipo H1N1 del Virus de la Influenza A/genética , Gripe Humana/complicaciones , Pandemias , Niño , Preescolar , ADN Viral/análisis , Electroencefalografía , Encefalitis Viral/diagnóstico , Encefalitis Viral/virología , Femenino , Humanos , Gripe Humana/epidemiología , Gripe Humana/virología , Japón/epidemiología , Imagen por Resonancia Magnética , Masculino , Estudios Retrospectivos , Tomografía Computarizada por Rayos X
17.
Pediatr Emerg Care ; 28(2): 153-7, 2012 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-22307182

RESUMEN

BACKGROUND: Posterior reversible encephalopathy syndrome (PRES) is a recently described disorder with typical radiological findings of bilateral gray and white matter abnormalities in the posterior regions of the cerebral hemispheres and cerebellum. Its clinical symptoms include headache, decreased alertness, mental abnormalities such as confusion, diminished spontaneity of speech, and changed behavior ranging from drowsiness to stupor, seizures, vomiting, and abnormalities of visual perception such as cortical blindness. In this study, the clinical and radiological findings of 4 children with this syndrome due to a variety of conditions are reported. METHODS: The records of 4 children with a diagnosis of PRES were retrospectively analyzed. RESULTS: PRES is associated with a disorder of cerebrovascular autoregulation of multiple etiologies. Four patients with PRES who had primary diagnoses of severe aplastic anemia, nephritic syndrome, Henoch-Schönlein purpura, and acute poststreptococcal glomerulonephritis are presented. This syndrome has been described in numerous medical conditions, including hypertensive encephalopathy, eclampsia, and with the use of immunosuppressive drugs. CONCLUSIONS: Early recognition of PRES as a complication during different diseases and therapies in childhood may facilitate precise diagnosis and appropriate treatment.


Asunto(s)
Encefalopatía Hipertensiva/diagnóstico , Imagen por Resonancia Magnética , Síndrome de Leucoencefalopatía Posterior/diagnóstico , Adolescente , Anemia Aplásica/cirugía , Anticonvulsivantes/uso terapéutico , Antihipertensivos/uso terapéutico , Niño , Ciclosporina/efectos adversos , Diagnóstico Precoz , Epilepsia Tónico-Clónica/tratamiento farmacológico , Epilepsia Tónico-Clónica/etiología , Femenino , Glomerulonefritis/complicaciones , Humanos , Encefalopatía Hipertensiva/tratamiento farmacológico , Encefalopatía Hipertensiva/patología , Inmunosupresores/efectos adversos , Discapacidad Intelectual/etiología , Imagen por Resonancia Magnética/métodos , Masculino , Síndrome Nefrótico/complicaciones , Síndrome de Leucoencefalopatía Posterior/inducido químicamente , Síndrome de Leucoencefalopatía Posterior/tratamiento farmacológico , Síndrome de Leucoencefalopatía Posterior/etiología , Síndrome de Leucoencefalopatía Posterior/patología , Complicaciones Posoperatorias/inducido químicamente , Complicaciones Posoperatorias/diagnóstico , Púrpura de Schoenlein-Henoch/complicaciones , Trasplante de Células Madre , Infecciones Estreptocócicas/complicaciones , Trastornos de la Visión/etiología
18.
Brain Dev ; 34(2): 115-7, 2012 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-21441007

RESUMEN

We report on a 4-year-old boy who died from influenza encephalopathy. The clinical course and microscopic findings of the autopsied liver were compatible with Reye's syndrome. We examined the mitochondrial respiratory chain function by blue native polyacrylamide gel electrophoresis (BN-PAGE), western blotting, and respiratory chain enzyme activity assays. The activity of liver respiratory chain complex (CO) I was markedly decreased (7.2% of the respective control activity); whereas, the other respiratory chain complex activities were substantially normal (CO II, 57.9%; CO III, 122.3%; CO IV, 161.0%). The activities of CO I-IV in fibroblasts were normal (CO I, 82.0%; CO II, 83.1%; CO III, 72.9%; CO IV, 97.3%). The patient was diagnosed with liver-specific complex I deficiency. This inborn disorder may have contributed to the fatal outcome. We propose that relying only on fibroblast respiratory chain complex activities may lead to the misdiagnosis of liver-specific complex I deficiency.


Asunto(s)
Complejo IV de Transporte de Electrones/metabolismo , Virus de la Encefalitis/patogenicidad , Gripe Humana/complicaciones , Hígado/enzimología , Enfermedades Mitocondriales , Preescolar , Complejo I de Transporte de Electrón/deficiencia , Complejo I de Transporte de Electrón/metabolismo , Humanos , Masculino , Enfermedades Mitocondriales/complicaciones , Enfermedades Mitocondriales/metabolismo , Enfermedades Mitocondriales/patología
19.
Clin Chim Acta ; 413(1-2): 109-12, 2012 Jan 18.
Artículo en Inglés | MEDLINE | ID: mdl-21963460

RESUMEN

BACKGROUND: Late preterm infants (LPIs; 34-37 gestational weeks at birth) have higher risk for several morbidities than do term infants (TIs). It has been suggested that a cholesterol and fatty acid supply may improve their outcomes. We investigated the lipoprotein subclass profile in LPIs to evaluate their early postnatal lipid metabolism. METHODS: Eighty-one infants (25 LPIs, 56 TIs) were included. Cholesterol and triglyceride (TG) concentrations in 12 lipoprotein subclasses were measured at birth and at 1 month using HPLC. RESULTS: In LPIs, the cord blood exhibited higher cholesterol concentrations in medium and large subclasses of very low-density lipoprotein (VLDL), low-density lipoprotein (LDL) and high-density lipoprotein (HDL) compared to the values in TIs. During the first month of life, LPIs had greater increases in cholesterol concentrations of medium and large subclasses of VLDL than TIs, whereas postnatal increases in cholesterol concentrations of medium and large subclasses of LDL and HDL were smaller. TG concentrations were not different in each VLDL subclass at birth and at 1 month. CONCLUSIONS: In LPIs, cord blood lipoprotein subclass profiles and the early postnatal change exhibited different, especially in cholesterol concentrations.


Asunto(s)
Recien Nacido Prematuro , Lipoproteínas/sangre , Colesterol/sangre , Cromatografía Líquida de Alta Presión , Femenino , Humanos , Recién Nacido , Masculino , Triglicéridos/sangre
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