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1.
Indian J Pediatr ; 2021 Jan 05.
Artículo en Inglés | MEDLINE | ID: mdl-33400135

RESUMEN

OBJECTIVE: To clarify the pathogenesis of sudden unexpected natural death (SUD) as well as biomarkers to differentiate the underlying diseases, by performing cytokine analysis in the acute phase of pediatric patients in whom viral infection led to SUD. METHODS: An acute phase cytokine analysis of pediatric patients in whom viral infection led to SUD was performed, and the data obtained were compared with those from SUD patients not associated with viral infections. Subjects included 4 boys aged 1-16 mo who died of cardiopulmonary arrest associated with viral infections. The causative viruses were identified as enterovirus, parainfluenza virus, respiratory syncytial virus, and rotavirus. The 4 other infants/children (aged 2-12 mo) died of non-infectious episodes, i.e., 1, 2, and 1 died of drowning, falling, and a traffic accident, respectively. Cerebrospinal fluid samples (CSF) of the subjects were collected during cardiopulmonary resuscitation or within 24 h of the events. RESULTS: The infection-induced sudden death group showed elevated CSF levels of inflammatory cytokines and chemokines. No increase was observed in interleukin-10 levels. Furthermore, in the infection-induced sudden death group, platelet-derived growth factor levels correlated with inflammatory cytokine levels. CONCLUSIONS: Infection-associated SUD may be differentiated from noninfectious SUD by measuring the levels of acute phase-inflammatory cytokines and chemokines at the onset of SUD.

2.
Jpn J Infect Dis ; 73(6): 443-446, 2020 Nov 24.
Artículo en Inglés | MEDLINE | ID: mdl-32611972

RESUMEN

Low blood levels of vitamin D have been reported in children who have frequent respiratory tract infections. We measured serum concentrations of 25-hydroxy (OH) vitamin D in Japanese infants under 3 months of age who had respiratory syncytial virus (RSV) infection. Serum levels of 25-OH vitamin D in the 10 infants, excluding those with underlying diseases, were between < 4 and 29.8 ng/mL. In 8 out of 10 subjects (80.0%), serum 25-OH vitamin D levels were lower than 20 ng/mL. There was no statistically significant association between the levels of 25-OH vitamin D and age, duration of admission, respiratory severity score, white blood cell count, blood gas levels, and N-terminal pro-natriuretic peptide levels. Levels of serum 25-OH vitamin D in children who required hospitalization owing to RSV infection were low, indicating deficiency. These results suggest that vitamin D deficiency affects the susceptibility to RSV infection, but not the severity of the infection.

3.
Brain Dev ; 42(7): 523-528, 2020 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-32336482

RESUMEN

BACKGROUND: Riboflavin may prevent migraine episodes; however, there is limited evidence of its effectiveness in pediatric populations. This study investigated the effectiveness of riboflavin and clinical predictors of response in children with migraines. METHODS: We retrospectively reviewed data from 68 Japanese children with migraines, of whom 52 also exhibited another type of headache. Patients received 10 or 40 mg/day of riboflavin. We evaluated the average migraine frequency per month as a baseline and after 3 months of riboflavin therapy to determine the effectiveness and clinical predictors of response. RESULTS: The frequency of migraine episodes was significantly lower at 3 months than at baseline (median, [interquartile range], 5.2 (3-7) vs. 4.0 (2-5); p < 0.01). Twenty-five patients (36.7%) showed 50% or greater reduction in episode frequency (responders), while 18 (26.5%) showed a 25%-50% reduction. We compared responders (n = 25) and non-responders (n = 43) and found no significant differences in sex, familial history, riboflavin dose, migraine type (i.e., presence or absence of aura), age at headache onset, or age at consultation. However, non-responders were more likely to have co-morbid non-migraine headaches (odds ratio, 4.11; 95% confidence interval [CI], 1.27-13.33; p = 0.02); this variable was also significant in a multivariate analysis (adjusted odds ratio, 3.8; 95% CI, 1.16-12.6; p = 0.03). Of the co-morbid headache types, only tension headaches were significant (odds ratio, 0.176; 95% CI, 0.04-0.73; p = 0.013). No adverse effects of riboflavin were identified. CONCLUSIONS: Low-dose riboflavin is safe and modestly effective for migraines in children. It may be especially beneficial for children without other co-morbid headache types.

4.
J Infect Chemother ; 26(4): 393-396, 2020 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-31879187

RESUMEN

We report a first case of respiratory syncytial virus (RSV) infection-associated encephalopathy in which RS virus was detected in the patient's intratracheal aspiration and cerebrospinal fluid despite negative rapid test results of the nasal swab. The patient's findings and clinical course coincided with those of acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) with severe subsequent sequelae. Our case indicates that clinicians should consider RSV infection when patients have AESD with unknown etiology.

5.
J Pediatr Hematol Oncol ; 41(5): e325-e328, 2019 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-30130274

RESUMEN

Therapeutic phlebotomy is recommended for treating hereditary hemochromatosis. However, the procedure and its efficacy for children remain unclear. We describe a young female patient with ferroportin disease, which was confirmed from excess iron deposition within hepatocytes and by identifying a heterozygous variant p.Cys326Phe in SLC40A1. She had been followed without phlebotomy. Liver histology at age 13 years revealed iron deposition progression. Phlebotomy was initiated and her iron markers and imaging findings improved without severe adverse effects. Therapeutic phlebotomy for children is effective and well-tolerated and should be considered as early as possible after a hemochromatosis diagnosis.


Asunto(s)
Proteínas de Transporte de Catión/deficiencia , Hemocromatosis/diagnóstico , Hemocromatosis/terapia , Mutación , Flebotomía/métodos , Adolescente , Proteínas de Transporte de Catión/genética , Femenino , Hemocromatosis/genética , Heterocigoto , Humanos , Resultado del Tratamiento
8.
Clin Rheumatol ; 36(6): 1433-1435, 2017 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-28321568

RESUMEN

Sjögren syndrome (SS) is characterized by lymphocytic infiltration of exocrine glands, mainly the lacrimal and salivary glands, leading to keratoconjunctivitis sicca and xerostomia. SS is one of the most common autoimmune rheumatic diseases in adults; however, few cases of primary childhood SS with gastrointestinal and liver lesions have been reported in the literature. We report five cases of primary childhood SS with gastrointestinal and liver lesions. Multiple gastric biopsies in four cases revealed atrophic gastritis in the antrum of the stomach or chronic gastritis. Liver biopsies in two cases revealed nonalcoholic steatohepatitis. Careful clinical approach and follow-up for gastrointestinal and liver lesions are required.


Asunto(s)
Hígado/patología , Síndrome de Sjögren/patología , Estómago/patología , Adolescente , Niño , Femenino , Humanos , Masculino
9.
Arthritis Rheumatol ; 69(2): 447-459, 2017 02.
Artículo en Inglés | MEDLINE | ID: mdl-27788288

RESUMEN

OBJECTIVE: To elucidate the genetic background of a patient with neonatal-onset multisystem inflammatory disease (NOMID) with no NLRP3 mutation. METHODS: A Japanese male child diagnosed as having NOMID was studied. The patient did not have any NLRP3 mutation, even as low-frequency mosaicism. We performed whole-exome sequencing on the patient and his parents. Induced pluripotent stem cells (iPSCs) were established from the patient's fibroblasts. The iPSCs were then differentiated into monocyte lineage to evaluate the cytokine profile. RESULTS: We established multiple iPSC clones from a patient with NOMID and incidentally found that the phenotypes of monocytes from iPSC clones were heterogeneous and could be grouped into disease and normal phenotypes. Because each iPSC clone was derived from a single somatic cell, we hypothesized that the patient had somatic mosaicism of an interleukin-1ß-related gene. Whole-exome sequencing of both representative iPSC clones and the patient's blood revealed a novel heterozygous NLRC4 mutation, p.T177A (c.529A>G), as a specific mutation in diseased iPSC clones. Knockout of the NLRC4 gene using the clustered regularly interspaced short palindromic repeat/Cas9 system in a mutant iPSC clone abrogated the pathogenic phenotype. CONCLUSION: Our findings indicate that the patient has somatic mosaicism of a novel NLRC4 mutation. To our knowledge, this is the first case showing that somatic mutation of NLRC4 causes autoinflammatory symptoms compatible with NOMID. The present study demonstrates the significance of prospective genetic screening combined with iPSC-based phenotype dissection for individualized diagnoses.


Asunto(s)
Proteínas Adaptadoras de Señalización CARD/genética , Proteínas de Unión al Calcio/genética , Síndromes Periódicos Asociados a Criopirina/genética , Mutación , Humanos , Células Madre Pluripotentes Inducidas , Recién Nacido , Masculino , Fenotipo
11.
J Clin Lab Anal ; 29(4): 328-33, 2015 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-25130628

RESUMEN

Rotavirus is the most common cause of severe gastroenteritis in young children; however, its pathogenesis and immunity are not completely understood. Even less well recognized is rotavirus-induced central nervous system (CNS) involvement, which has been associated with seizure, encephalopathy and death, among others. To elucidate the host response to rotavirus infection, we retrospectively examined neurotransmitter amino acids in the cerebrospinal fluid (CSF) of 19 children with CNS involvement associated with rotavirus infection. Subjects were classified into two groups: those with encephalopathy followed by prolonged seizure (encephalopathy group) and those who had experienced afebrile, brief cluster of seizures without encephalopathy (cluster group). The levels of glutamate, glycine, and taurine in the encephalopathy group were significantly higher than those in the cluster group. Increased levels of excitatory amino acids in the CSF may induce neurological disorders and be related to disorder severity. To the best of our knowledge, this is the first report regarding amino acids in the CSF obtained from patients with rotavirus-induced CNS involvement. Further study is necessary to elucidate the role of CSF amino acid levels in rotavirus-induced CNS involvement.


Asunto(s)
Encefalopatías/líquido cefalorraquídeo , Encefalopatías/virología , Aminoácidos Excitadores/líquido cefalorraquídeo , Infecciones por Rotavirus/líquido cefalorraquídeo , Infecciones por Rotavirus/virología , Rotavirus/fisiología , Niño , Preescolar , Femenino , Ácido Glutámico/líquido cefalorraquídeo , Humanos , Lactante , Masculino
12.
World J Diabetes ; 5(6): 917-23, 2014 Dec 15.
Artículo en Inglés | MEDLINE | ID: mdl-25512797

RESUMEN

Various pathological conditions can cause fatty liver in children. Nonalcoholic steatohepatitis (NASH) in children has been known since 1983. However, NASH diagnosed in childhood does not have a favorable outcome. The pathological characteristics of NASH are significantly different between children and adults. Nonalcoholic fatty liver disease (NAFLD)/NASH is accompanied by insulin resistance, which plays a pivotal role in its pathophysiology in both children and adults. In NASH, a "two-hit" model involving triglyceride accumulation (first hit) and liver damage (second hit) has been accepted. Insulin resistance was found to correlate with changes in fat levels; however, it did not correlate with fibrosis or NAFLD activity score in children. Therefore, insulin resistance may be important in the first hit. Because there is obvious familial clustering in NASH, genetic predisposition as well as environmental factors including diet might be the second hit of NAFLD/NASH.

13.
J Infect Chemother ; 20(11): 661-5, 2014 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-25179394

RESUMEN

There are currently very few English reports about Japanese pediatric human immunodeficiency virus (HIV). In this study, we introduce our experience with pediatric HIV in a single hospital, and review the present status of HIV infections in children in Japan. In Japan, the main infection routes of HIV include sexual activity, mother-to-child transmission (MTCT), blood or blood product transfusion, and drug use. Most pediatric HIV patients have been infected by MTCT in recent years. One survey showed that in Japan, 52 babies were infected by MTCT between 1984 and 2011. Only 2 cases of pediatric HIV infection have been reported since 2010. The MTCT rate has decreased to 0.5% owing to several preventive interventions. In addition, the HIV antibody test is now performed in more than 98.3% of pregnant women in Japan.


Asunto(s)
Infecciones por VIH/transmisión , Transmisión Vertical de Enfermedad Infecciosa , Terapia Antirretroviral Altamente Activa , Niño , Preescolar , Femenino , Infecciones por VIH/diagnóstico , Infecciones por VIH/tratamiento farmacológico , Infecciones por VIH/epidemiología , Infecciones por VIH/etiología , Humanos , Lactante , Recién Nacido , Japón/epidemiología , Masculino , Inhibidores de la Transcriptasa Inversa/uso terapéutico , Conducta Sexual , Abuso de Sustancias por Vía Intravenosa/complicaciones , Reacción a la Transfusión
14.
J Infect Chemother ; 20(7): 443-5, 2014 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-24793175

RESUMEN

BACKGROUND: There is currently no consensus treatment for children non-responsive to peginterferon (Peg-IFN) and ribavirin. CASE PRESENTATION: Here, we present a Japanese child with chronic hepatitis C with fibrosis, who did not respond to Peg-IFN α-2b but responded to Peg-IFN α-2a with ribavirin, accompanied with fluvastatin. To date, there has been no reported case of re-treatment in children. The early viral response occurred soon after starting treatment using Peg-IFN α-2a/ribavirin plus fluvastatin. CONCLUSION: Our result indicates that when treatment by Peg-IFN α-2b/ribavirin combination therapy is not efficient, combination therapy using Peg-IFN α-2a/ribavirin plus fluvastatin should be considered in children with advanced liver change.


Asunto(s)
Antivirales/uso terapéutico , Hepatitis C Crónica/tratamiento farmacológico , Interferón-alfa/uso terapéutico , Polietilenglicoles/uso terapéutico , Ribavirina/uso terapéutico , Niño , Quimioterapia Combinada/métodos , Ácidos Grasos Monoinsaturados/uso terapéutico , Fluvastatina , Humanos , Indoles/uso terapéutico , Interferón alfa-2 , Masculino , Proteínas Recombinantes/uso terapéutico , Resultado del Tratamiento
15.
Pathol Int ; 64(2): 75-80, 2014 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-24629175

RESUMEN

Shwachman-Diamond syndrome, which is characterized by pancreatic fatty degeneration, skeletal growth retardation, and hematological dysfunction, is a congenital disease caused by SBDS gene mutations. Although hematological disorders often accompany this syndrome, carcinomas associated with this syndrome have not been reported except in one breast cancer and one moderately differentiated pancreatic cancer case. We report on an autopsy of a 24-year-old case of pancreatoduodenal carcinoma in Shwachman-Diamond syndrome. The histology of the tumor was undifferentiated carcinoma, which seems to have originated from either the pancreatic duct or the duodenal epithelium. The tumor was intermingled with two pathological changes characteristic of Shwachman-Diamond syndrome: fatty degeneration of the pancreas and inflammation of the villous stroma of the duodenum. Considering that SBDS protein regulates mitosis and its suppression causes genomic instability, this case might provide an example of carcinogenesis based on genomic instability, together with degenerative changes and chronic inflammation, at a very young age.


Asunto(s)
Enfermedades de la Médula Ósea/patología , Carcinoma/patología , Neoplasias Duodenales/patología , Insuficiencia Pancreática Exocrina/patología , Lipomatosis/patología , Neoplasias Pancreáticas/patología , Enfermedades de la Médula Ósea/complicaciones , Carcinoma/complicaciones , Neoplasias Duodenales/complicaciones , Insuficiencia Pancreática Exocrina/complicaciones , Resultado Fatal , Humanos , Lipomatosis/complicaciones , Masculino , Neoplasias Pancreáticas/complicaciones , Síndrome de Shwachman-Diamond , Adulto Joven
17.
Nihon Rinsho ; 70(8): 1376-80, 2012 Aug.
Artículo en Japonés | MEDLINE | ID: mdl-22894076

RESUMEN

Rotavirus is one of the universal viruses that causes diarrhea(acute gastroenteritis) in the worldwide, and the infections are sporadically accompanied with critical illness. Ten percent of affected individuals are admitted to hospitals. The complications are commonly serious dehydration, several diseases of central nervous system, sepsis and postrenal insufficiency accompanied with renal stone. Two safety and effective vaccinations were implemented to prevent infections. According to the overseas guideline management for estimations for the severity and treatment by oral rehydration solution are recommended.


Asunto(s)
Gastroenteritis/virología , Infecciones por Rotavirus , Enfermedad Aguda , Enfermedades del Sistema Nervioso Central/etiología , Deshidratación/etiología , Deshidratación/terapia , Femenino , Fluidoterapia , Gastroenteritis/complicaciones , Gastroenteritis/prevención & control , Gastroenteritis/terapia , Humanos , Cálculos Renales/etiología , Masculino , Sepsis/etiología , Índice de Severidad de la Enfermedad , Vacunación
18.
Clin Exp Nephrol ; 16(4): 656-7, 2012 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-22752353

RESUMEN

Acute focal bacterial nephritis (AFBN) is a localized, interstitial bacterial infection of the renal parenchyma. In this study, we measured the serum levels of several cytokines in patients with AFBN. A total of 11 children were enrolled in the study and classified into two groups of patients: an AFBN group and a control group. There was no significant difference in the serum levels of interleukin (IL)-1ß, IL-2, IL-4, IL-6, IL-8, IL-10, IL-12, IL-17, granulocyte-colony stimulating factor, or tumor necrosis factor-α among the patients in the two groups. However, the serum levels of interferon-γ among the patients in the AFBN group were significantly higher than those among the patients in the control group. The current results suggest that the bacterial kidney infection in the AFBN group is localized and that interferon-γ may be produced locally in response to the infection.


Asunto(s)
Interferón gamma/sangre , Nefritis/sangre , Nefritis/microbiología , Enfermedad Aguda , Estudios de Casos y Controles , Niño , Preescolar , Enterococcus faecalis/aislamiento & purificación , Escherichia coli/aislamiento & purificación , Femenino , Factor Estimulante de Colonias de Granulocitos y Macrófagos/sangre , Humanos , Lactante , Interleucinas/sangre , Masculino , Factor de Necrosis Tumoral alfa/sangre
20.
Pediatr Int ; 54(1): 86-8, 2012 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-22136601

RESUMEN

BACKGROUND: Children infected with rotavirus often show increased levels of transaminase, and symptoms are characterized by white stool, similar to biliary atresia. Rotavirus infections are also sporadically accompanied with convulsions, encephalopathy and Reye syndrome. The aim of the present study was therefore to investigate transaminase and interleukin (IL)-6 levels in rotavirus infection, in order to better understand their clinical significance. METHODS: Results of liver function tests, mainly the elevation of transaminase and IL-6 in rotavirus gastroenteritis with or without convulsions, were evaluated. RESULTS: Aspartate aminotransferase (AST) and alanine aminotransferase (ALT) levels were high in 23 of 26 samples (88.5%), and in three of 26 samples (11.5%), respectively. No significant differences in liver function tests could be found between the groups with or without convulsions. Three patients whose direct bilirubin levels were above the upper normal limit were all classified into the group without convulsions. Spearman's correlation coefficient was 0.89 between increasing AST levels and IL-6 levels. CONCLUSION: Rotavirus infection is occasionally accompanied with hepatitis, but only in a mild form, and does not correlate with neurological complications. High levels of transaminase might reflect high IL-6.


Asunto(s)
Alanina Transaminasa/sangre , Aspartato Aminotransferasas/sangre , Gastroenteritis/virología , Interleucina-6/sangre , Infecciones por Rotavirus/sangre , Convulsiones/sangre , Preescolar , Femenino , Gastroenteritis/sangre , Humanos , Lactante , Pruebas de Función Hepática , Masculino , Estudios Retrospectivos , Infecciones por Rotavirus/complicaciones , Convulsiones/virología
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