RESUMEN
WHAT IS KNOWN AND OBJECTIVE: Adrenoleukodystrophy (ALD) is an X-linked disorder and characterized by the accumulation of saturated very long-chain fatty acids. Treatment is still unsatisfactory. Our objective is to report on the effect of the free-radical scavenger, edaravone, in a patient with ALD. CASE SUMMARY: The patient was given edaravone intravenously twice. D-ROM in cerebral spinal fluid decreased dramatically, and a shortening of neuronal transmission time as estimated on somatosensory evoked potential was observed. After terminating the treatment, his symptoms progressively reappeared. WHAT IS NEW AND CONCLUSION: This is the first report of the use of edaravone in ALD. The drug is apparently effective in improving symptoms of ALD and should be evaluated more formally.
Asunto(s)
Adrenoleucodistrofia/tratamiento farmacológico , Antipirina/análogos & derivados , Depuradores de Radicales Libres/uso terapéutico , Adrenoleucodistrofia/líquido cefalorraquídeo , Antipirina/efectos adversos , Antipirina/uso terapéutico , Niño , Edaravona , Potenciales Evocados Somatosensoriales/efectos de los fármacos , Depuradores de Radicales Libres/efectos adversos , Humanos , Masculino , Neuronas/efectos de los fármacos , Especies Reactivas de Oxígeno/líquido cefalorraquídeo , Transmisión Sináptica/efectos de los fármacos , Extremidad Superior/inervaciónRESUMEN
Duplications of the alimentary tract are very rare. A one-month-old female presented with symptoms of anorexia, vomiting and continuous watery diarrhea. The plain abdominal radiograph showed thickened intestinal wall and signs of small bowel obstruction. The fevers, vomiting, and continuous wartery diarrhea persisted despite antibiotics, and worsened. The patient failed to respond to medical managements, 27 hours after admission, the patient died due to multiple organ failures. The autopsy was performed, small bowel obstruction due to an ileocecal duplication cyst (3 × 3 cm) was recognized. The ileocecal duplication cyst was attached to the ileum which was changed edematous and necrotic. This potential diagnosis should be borne in mind for a patient who complains of abdominal symptoms with an unknown cause, and duplication cyst should be recognized as a fatal cause in infant.
RESUMEN
It has become evident that the number of patients with a new type of influenza-associated encephalopathy is increasing in Japan. Nitric oxide (NO), a simple free radical gas, elicits a wide range of physiological and pathophysiological effects. We measured the nitrite/nitrate (NO x ) levels in cerebral spinal fluid obtained from patients with influenza-associated encephalopathy in order to evaluate the correlation between the NO production and the process of influenza-associated encephalopathy. Fifteen children were enrolled, aged from 1 to 9 years. As control we used 14 cerebral spinal fluids obtained from patients with urinary tract infection, respiratory infection or mumps meningitis without any sequela. NO 3 in influenza-associated encephalopathy was significantly higher than that of control group. On the other hand NO 2 was not significantly higher than that of control group. In particular, 4 out of 5 fatal cases revealed high NO 2 or NO 3. One case having normal levels in NO 2 and NO 3 showed that NH 3 was high. These results revealed that NO plays a role in influenza-associated encephalopathy through indirect effects of NO.
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Encéfalo/metabolismo , Encéfalo/virología , Encefalitis Viral/líquido cefalorraquídeo , Encefalitis Viral/virología , Gripe Humana/líquido cefalorraquídeo , Gripe Humana/virología , Óxido Nítrico/líquido cefalorraquídeo , Encéfalo/diagnóstico por imagen , Niño , Preescolar , Electroencefalografía , Encefalitis Viral/mortalidad , Femenino , Humanos , Lactante , Masculino , Índice de Severidad de la Enfermedad , Tasa de Supervivencia , Tomografía Computarizada por Rayos XRESUMEN
BACKGROUND: In the USA, a high prevalence rate of cytomegalovirus (CMV) excretion among children in day-care centres was reported. However, there is no research about the prevalence rate of CMV among children in day-care centres in Japan. METHODS: The CMV excretion was studied in 54 children's saliva samples, collected from two different day-care centres in Tokyo. As a control, the prevalence of CMV was studied among 61 healthy children who did not attend any day-care centers. The CMV DNA in saliva were examined by polymerase chain reaction (PCR) analysis with one pair of primers for the immediate early region. The sequence of CMV genomes were examined in CMV PCR positive samples. RESULTS: Of the 54 saliva samples, 20.6% (6/29) and 24% (6/25) were CMV PCR positive in children at A and B day-care centres, respectively. The overall positivity of CMV PCR in saliva was 22.2% (12/54). Of the 61 saliva samples as the control study, 6.5% (4/61) were CMV PCR positive. There was a difference in the positivity in each age group of day-care centres and normal control. Each sample of the same day-care center gave conclusive and identical sequence results. CONCLUSION: We suspected that in each day-care center that there was one prevailing viral strain. We suppose that CMV infections were acquired inside the day-care centres. This is a first report which described viral transmission in day-care centres in Japan.
Asunto(s)
Guarderías Infantiles , Infecciones por Citomegalovirus/epidemiología , Citomegalovirus/aislamiento & purificación , ADN Viral/análisis , Saliva/microbiología , Preescolar , Citomegalovirus/genética , Infecciones por Citomegalovirus/transmisión , Infecciones por Citomegalovirus/virología , Transmisión de Enfermedad Infecciosa , Humanos , Lactante , Recién Nacido , Japón/epidemiología , Reacción en Cadena de la Polimerasa , PrevalenciaRESUMEN
The incidence of and mortality from colorectal cancer are increasing in Japan, as is its proportion among all malignant neoplasms. Thus, primary prevention of this cancer is crucial. Colorectal cancer is caused by interactions between host and environmental factors, with accumulation of gene alterations, such as activation of oncogenes and inactivation of suppressor genes, and generally involves an adenoma-carcinoma sequence. Carcinogenesis progresses with multi-factor, multi-hit and multi-stage mechanisms. According to the report by WCRF/AICR, convincing preventive factors include eating vegetables (not fruit) and physical activity (colon only), while probable risk factors are red meat and alcohol. Possible preventive factors include dietary fiber, starch and carotenoids, whereas possible risk factors include high body mass, fat and heavily cooked meat. Such preventive and risk factors for colorectal cancer are discussed in this review.
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Neoplasias Colorrectales/etiología , Neoplasias Colorrectales/epidemiología , Neoplasias Colorrectales/prevención & control , Femenino , Humanos , Japón/epidemiología , Masculino , Factores de RiesgoRESUMEN
We found ten cases of human cytomegalovirus (CMV) infection who were intractable stridor. Their symptoms were not improved by the treatment with aminophyllin nor beta stimulants. They were admitted repeatedly complaining of stridor, fever and diarrhea. In two cases, the immunological findings showed a decrease of bacterial sterilizing activity of the neutrophils. Additionally, blood count showed leukocytosis more than 15,000/ul in all cases. Total serum IgE and specific IgE antibodies to many antigens were not elevated. Transaminase was elevated. Chest X-p findings of interstitial pneumonia or atelectasis continued for a long time in some cases. Virological examinations revealed high concentrations of specific IgM or CF antibodies against CMV in all cases. CMV DNA in saliva were examined by polymerase chain reaction (PCR) with primer sets for the immediate early (IE) region of CMV and showed positive in seven cases. CMV in bronchoalveolar lavage (BAL) was isolated in two cases, and CMV PCR in BAL was positive in three cases. The sequence of the CMV-PCR products showed almost same sequence except one point mutation in bp 1203. We considered that CMV infections in infants may induce stridor for a long period.
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Infecciones por Citomegalovirus/complicaciones , Ruidos Respiratorios/etiología , Femenino , Humanos , Lactante , MasculinoRESUMEN
A hospital-acquired outbreak with febrile illness and/or rash occurred in our neonatal special care nursery (SCN) from September 1995 to September 1996. A total of 23 infants developed symptoms. We could not detect the etiological agents by routine virus isolation. In a retrospective study, however, enterovirus RNA was detected by reverse transcriptase-polymerase chain reaction (RT-PCR) from four out of six cerebrospinal fluid (CSF) samples and from two of 12 sera. Thus six out of 16 patients from whom samples were obtained were diagnosed retrospectively as having enterovirus infection. Furthermore, we detected the enterovirus genome from four of 20 serum samples obtained from patients who had other clinical symptoms, and from infants hospitalized without noticeable clinical illness during the same periods. This outbreak was caused by two different enteroviruses, which we assumed were echovirus type 7 (Echo 7) and coxsackievirus B3 (Cox B3), because of the sequence results. We demonstrated the clinical advantage of the analysis of nucleotide sequencing as supportive evidence of transmission.
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Infección Hospitalaria/diagnóstico , Transmisión de Enfermedad Infecciosa , Infecciones por Enterovirus/diagnóstico , Enterovirus/aislamiento & purificación , Secuencia de Bases , Infección Hospitalaria/epidemiología , Infección Hospitalaria/transmisión , ADN Complementario/química , Brotes de Enfermedades , Enterovirus/genética , Infecciones por Enterovirus/epidemiología , Infecciones por Enterovirus/transmisión , Femenino , Humanos , Recién Nacido , Unidades de Cuidado Intensivo Neonatal , Japón/epidemiología , Masculino , Datos de Secuencia Molecular , ARN Viral/sangre , ARN Viral/líquido cefalorraquídeo , ARN Viral/genética , Estudios Retrospectivos , Reacción en Cadena de la Polimerasa de Transcriptasa Inversa , Alineación de Secuencia , Análisis de Secuencia de ADNRESUMEN
Ultrasonic evaluation of the intima-media thickness in the common carotid artery (IMT-CCA) has been widely used as a marker of atherosclerosis. However, the definition of IMT-CCA is not uniform and it includes two quite different pathological changes; a general intima-media thickening and a local atherosclerotic change (plaque formation). The aim of this paper was to evaluate the IMT-CCA and local atherosclerosis separately, and to clarify how the IMT-CCA itself changes with age and how local plaques influence the relationship between the IMT-CCA and age. The subjects were 979 men and women aged 40 to 79 years who participated in the first wave examination of the National Institute for Longevity Sciences--Longitudinal Study of Aging (NILS-LSA). The IMT-CCA measured at the thinnest point was significantly higher in men (0.61 +/- 0.15 mm) than in women (0.58 +/- 0.14 mm, p < 0.01) and it increased with age (trend p < 0.0001) in both genders. The IMT-CCA was higher with the presence of plaque in the bulbs (PLQ-BLB) than without PLQ-BLB (p < 0.0001). Although this was a cross-sectional study, the IMT-CCA increased 0.06 mm/10 years with PLQ-BLB and 0.04 mm/10 years without PLQ-BLB. The IMT-CCA could be partially explained by age, gender and PLQ-BLB (r2 = 0.317). In this middle-aged and elderly population, an increase in the IMT-CCA showed a moderate relationship with local atherosclerosis and age.
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Envejecimiento/patología , Enfermedades de las Arterias Carótidas/patología , Arteria Carótida Común/diagnóstico por imagen , Arteria Carótida Común/patología , Adulto , Factores de Edad , Anciano , Enfermedades de las Arterias Carótidas/epidemiología , Femenino , Humanos , Japón/epidemiología , Estudios Longitudinales , Masculino , Persona de Mediana Edad , Análisis Multivariante , Variaciones Dependientes del Observador , Prevalencia , Análisis de Regresión , Factores Sexuales , UltrasonografíaRESUMEN
The purpose of this study was to investigate age and gender differences in current perception thresholds (CPT) in Japanese citizens. CPT values at frequencies of 2000, 250 and 5 Hz reflect different types of peripheral sensory nerve functions. Since there have been only a limited number of reports which investigated CPT values in community-dwelling people, little is known about variations with age and gender. The present study therefore concentrated on a large population of 1632 individuals (men; 818 mean age +/- standard deviation 59.4 +/- 10.9, women; 814, 59.4 +/- 11.1) in a Japanese community. Significant gender differences in CPT values at 250 and 5 Hz were observed. Multiple comparisons among 4 age groups (40s, 50s, 60s and 70s) showed age-related differences in CPT values at 2000 and 250 Hz in both genders. However, age and gender interactions with reference to CPT values appeared to be different between these latter two frequencies. At 5 Hz, only men showed age-related variations. These results indicated gender differences in fiber-specific aging changes.
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Envejecimiento/fisiología , Umbral Sensorial/fisiología , Fenómenos Fisiológicos de la Piel , Adulto , Distribución por Edad , Anciano , Estimulación Eléctrica , Femenino , Humanos , Japón , Estudios Longitudinales , Masculino , Persona de Mediana Edad , Caracteres Sexuales , Distribución por SexoRESUMEN
It has been reported that measles virus may be present in the intestine of patients with Crohn's disease. Additionally, a new syndrome has been reported in children with autism who exhibited developmental regression and gastrointestinal symptoms (autistic enterocolitis), in some cases soon after MMR vaccine. It is not known whether the virus, if confirmed to be present in these patients, derives from either wild strains or vaccine strains. In order to characterize the strains that may be present, we have carried out the detection of measles genomic RNA in peripheral mononuclear cells (PBMC) in eight patients with Crohn's disease, three patients with ulcerative colitis, and nine children with autistic enterocolitis. As controls, we examined healthy children and patients with SSPE, SLE, HIV-1 (a total of eight cases). RNA was purified from PBMC by Ficoll-paque, followed by reverse transcription using AMV; cDNAs were subjected to nested PCR for detection of specific regions of the hemagglutinin (H) and fusion (F) gene regions. Positive samples were sequenced directly, in nucleotides 8393-8676 (H region) or 5325-5465 (from noncoding F to coding F region). One of eight patients with Crohn disease, one of three patients with ulcerative colitis, and three of nine children with autism, were positive. Controls were all negative. The sequences obtained from the patients with Crohn's disease shared the characteristics with wild-strain virus. The sequences obtained from the patients with ulcerative colitis and children with autism were consistent with being vaccine strains. The results were concordant with the exposure history of the patients. Persistence of measles virus was confirmed in PBMC in some patients with chronic intestinal inflammation.
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Trastorno Autístico/virología , ADN Viral/química , Enfermedades Inflamatorias del Intestino/virología , Vacuna Antisarampión/efectos adversos , Virus del Sarampión/aislamiento & purificación , Monocitos/virología , Vacuna contra la Parotiditis/efectos adversos , Vacuna contra la Rubéola/efectos adversos , Adulto , Secuencia de Bases , Estudios de Casos y Controles , Colitis Ulcerosa/virología , Enfermedad de Crohn/virología , Femenino , Humanos , Masculino , Virus del Sarampión/genética , Vacuna contra el Sarampión-Parotiditis-Rubéola , Datos de Secuencia Molecular , Vacunas Combinadas/efectos adversosRESUMEN
Casein a component of milk is used for food additives, industrial materials and drugs. However casein is known to be a main allergen in milk allergy. Recently several cases of anaphylaxis to antibiotics including casein have been reported. In this study we investigated casein allergy in milk allergy. 6 out of 8 patients who were positive for milk RAST were also positive for casein RAST. In these positive cases only 3 out of 6 patients had some allergic symptoms after taking antibiotics. In 3 patients DLST was also positive to casein. There was one patient who was positive in DLST without any symptoms after taking the same antibiotics. It is needed to pay attention to casein allergy when giving the medication which includes casein.
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Caseínas/inmunología , Hipersensibilidad a la Leche/inmunología , Adolescente , Anafilaxia/etiología , Anafilaxia/inmunología , Antibacterianos/efectos adversos , Niño , Preescolar , Femenino , Humanos , Lactante , Masculino , Prueba de RadioalergoadsorciónRESUMEN
Polyarteritis nodosa (PAN) is a multisystem inflammatory disease associated with necrosis of small and medium arteries. It can occur in a systemic form with manifestations in skin, joints, heart, nervous system, gastrointestinal tracts, lungs and kidneys. It is relatively rare in adults and even more in children. It may be divided into a cutaneous form (C-PAN) and a more generalized form (G-PAN). Usually C-PAN is thought to run a benign course. But some cases were reported to run chronic courses and be diagnosed G-PAN. It has been reported that some adults with PAN are related to HBV infection. On the other hand most children with PAN had upper respiratory infections some with streptococcal infections. We described a 5-year-old boy who was suffering from high fever, joint pain, subcutaneous nodular and livedo reticularis. We diagnosed him C-PAN by examination and clinical course. Anti-streptolysin O (ASO) and anti-streptokinase (ASK) increased and we suspected that C-PAN followed after streptococcal infection. We reviewed the literatures of C-PAN and streptococcal infection in childhood. Streptococcal infection has been implicated by a positive throat swab or an increase in ASO. There were 25 cases (75.7%) which were thought to be related with prior streptococcal infection in 33 cases. We thought that C-PAN may be associated with streptcoccal infection in childhood.
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Poliarteritis Nudosa/etiología , Infecciones del Sistema Respiratorio/complicaciones , Infecciones Estreptocócicas/complicaciones , Preescolar , Humanos , Masculino , Poliarteritis Nudosa/tratamiento farmacológico , Infecciones del Sistema Respiratorio/tratamiento farmacológico , Infecciones Estreptocócicas/tratamiento farmacológico , Resultado del TratamientoRESUMEN
BACKGROUND: Non-polio enterovirus infections are recognized in children during summer-fall seasons and they sometimes cause large outbreaks. We experienced a nosocomial infection in the neonatal nursery and echovirus type 7 was isolated from samples of four patients. OBJECTIVES: We diagnosed the horizontal infection of four neonates by reverse transcriptase-nested polymerase chain reaction (RT-nested PCR) and the nucleotide sequence. STUDY DESIGN: Total RNA was extracted from clinical isolates, serum samples and cerebrospinal fluid (CSF). We amplified enterovirus genome in the 5'-noncoding region by nested PCR and determined the nucleotide sequences. RESULTS: Enterovirus genome was detected in all isolates, in the acute-phase sera in all four patients and in the CSF in one patient by the first PCR. By using nested PCR, the genome was detected from convalescent-phase sera in two patients. All enterovirus genome obtained from the nursery outbreak showed the same sequences with 100% homology. CONCLUSION: We demonstrated the clinical advantages of RT-nested PCR from serum samples and the analysis of nucleotide sequencing gave the supportive evidence of identification of transmission pathway.
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Infección Hospitalaria , Brotes de Enfermedades , Infecciones por Echovirus/virología , Enterovirus Humano B/aislamiento & purificación , Reacción en Cadena de la Polimerasa de Transcriptasa Inversa/métodos , Regiones no Traducidas 5'/genética , Secuencia de Bases , Líquido Cefalorraquídeo/virología , ADN Complementario , Infecciones por Echovirus/epidemiología , Enterovirus Humano B/clasificación , Enterovirus Humano B/genética , Humanos , Recién Nacido , Epidemiología Molecular , Datos de Secuencia Molecular , Salas Cuna en Hospital , Faringe/virología , ARN Viral/sangre , Recto/virología , Sensibilidad y Especificidad , Análisis de Secuencia de ADNRESUMEN
We reported a 13-year-old boy diagnosed as multiple sclerosis associated with narcolepsy. He had suffered from retrobulbar optic neuritis at the age of 11 years which was improved gradually by prednisolone. Four months later he was admitted because of excessive somnolence. The diagnosis of narcolepsy was made based on hypnagogic hallucination, sleep paralysis, changes of personality and the sleep onset sREM (SOREM). The elevation of anti-measles antibody and a positive oligoclonal band in CSF, low density areas in the bilateral internal capsule on CT scan, and high signal areas in the same region on T2-weighted MRI confirmed the diagnosis of multiple sclerosis. An abnormal arousal response occurred occasionally in sleep stage 2 and 4, which started with electrical silence followed by a period with irregular high voltage slow waves and repetitive focal spike train. Those clinical symptoms and abnormalities of biochemical and electrophysiological studies normalized after treatment with prednisolone. However, abnormalities on MRI showed no improvement even after long term administration of prednisolone (2.5 mg/day).
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Esclerosis Múltiple/complicaciones , Narcolepsia/etiología , Adolescente , Humanos , Masculino , Esclerosis Múltiple/diagnósticoRESUMEN
A simple and sensitive method was developed for the differentiation of the Hoshino vaccine strain from wild strains with a restriction fragment length polymorphism (RFLP) analysis in the part of hemagglutinin-neuraminidase (HN) gene. The virus genome was amplified by using a reverse transcriptase-polymerase chain reaction (RT-PCR) directly from clinical samples. The PCR product of the Hoshino vaccine strain was cleaved into 2 fragments after digestion with Sca I and Afl II. All wild strains showed 2 RFLP profiles, A and B, different from that of vaccine strain. Wild A strains were cut into 2 fragments after digestion with Sca I only, while wild B strains were cleaved neither with Sca I nor Afl II. This molecular approach provides an effective method for differentiation of the Hoshino vaccine strain from wild strains of mumps virus in patients after vaccination.
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Genoma Viral , Proteína HN/genética , Vacuna contra la Parotiditis/aislamiento & purificación , Virus de la Parotiditis/aislamiento & purificación , Paperas/virología , Animales , Chlorocebus aethiops , Humanos , Meningitis Aséptica/líquido cefalorraquídeo , Meningitis Aséptica/virología , Paperas/patología , Vacuna contra la Parotiditis/clasificación , Vacuna contra la Parotiditis/genética , Virus de la Parotiditis/clasificación , Virus de la Parotiditis/genética , Polimorfismo de Longitud del Fragmento de Restricción , Células VeroAsunto(s)
Meningoencefalitis/etiología , Tifus por Ácaros/complicaciones , Anciano , Humanos , MasculinoRESUMEN
We described a 10-year-old girl with SLE complicated with anticardiolipin antibody, who showed bleeding tendency since infant. Her platelet was 6 x 10(3)/microliter. The APTT was remarkably prolonged. The platelet count was normalized after steroid treatment. A false positive Venereal Disease Research Laboratory (VDRL) test, lupus anticoagulants and anticardiolipin antibodies (IgG and IgM) were positive. Her symptoms and the laboratory data fulfilled the criteria of antiphospholipid syndrome. Renal biopsy was performed because of proteinuria, hematuria and the positive of anti-nuclear antibody. The pathology of kidney showed that of lupus nephritis (diffuse membranous glomerulonephritis), and she was diagnosed SLE. She showed no laboratory findings of hyperfibrinolysis and no symptoms of thrombosis under the steroid treatment for 3 years.
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Anticuerpos Anticardiolipina/análisis , Síndrome Antifosfolípido/complicaciones , Síndrome Antifosfolípido/diagnóstico , Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/diagnóstico , Niño , Femenino , Hemorragia/etiología , HumanosRESUMEN
We report a rare case of intractable frontal lobe epilepsy with mental deterioration, in which the measles virus gene was detected from the cerebrospinal fluid (CSF) and peripheral mononuclear cells (PBMC) obtained 9 years after the first epileptic episode using reverse transcriptase-polymerase chain reaction (RT-PCR). The patient had been immunized with an attenuated measles vaccine and had no history of clinically apparent acute measles infection. However the analysis of the sequence of the PCR product from CSF showed the circulating wild strain genotype at the time when the patient complained of his first epileptic episode.