Discordant endocrinopathy in a sibling with shwachman-diamond syndrome.
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Adolescent↗
Biomedical subjects
Yasuyo Kashiwagi
Publications and source records attributed to Yasuyo Kashiwagi.
Combined treatment of steroids and cyclosporine in Kimura disease.
Kimura disease is a rare but distinctive chronic eosinophilic inflammatory disorder that is characterized by tumor-like lesions in the soft tissue and lymph nodes of the head and neck or parotid gland. Recently, many immunopathogenetic features of underlying T lymphocytes and related cytokines have been noted in Kimura disease. However, few previous studies have investigated the serial levels of cytokines in children. In this report we describe an 11-year-old Japanese boy with relapsing Kimura disease. Before the diagnosis of Kimura disease, the patient had a swelling on his left neck. Steroids were effective, but the tumor relapsed within a few months as the steroids were tapered. He was treated with steroids and cyclosporine. This treatment was done by measuring serial levels of serum soluble interleukin-2 receptor, interleukin-4, interleukin-5, and eosinophil cationic protein. These results suggest the activation of T-helper cells and T-helper 2 cytokines, that after activated B cells and eosinophilic infiltration play an important role in Kimura disease, and that cyclosporine suppresses the activity of this disease.
Angiolymphoid Hyperplasia with Eosinophilia↗
Primary biomarkers in cerebral spinal fluid obtained from patients with influenza-associated encephalopathy analyzed by metabolomics.
In order to search for the specific biomarkers of patients with influenza-associated encephalopathy this article analyzed all metabolites in cerebrospinal fluid (CSF) by using metabolome analysis. In all metabolites, the peaks of two molecular weights, 246.0092 and 204.0611, were significantly higher than those in other diseases including influenza without convulsion (p < .05). The peak of a molecular weight 228.0247 in all of the patients except one was less than that in other patients. These results indicate that the new metabolites detected in CSF would be primary markers for the diagnosis of influenza-associated encephalopathy.
Biomarkers↗
Therapy of childhood generalized morphea: case reports and reviews of the literature of Japanese cases.
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Child↗
Diagnosis and evaluation of febrile infants under 4 months of age in Japan by using RT-PCR for enterovirus.
OBJECTIVES AND METHODS: In order to establish an effective management for febrile infants under 4 months of age we analysed the causes of fever by using bacterial work up and a highly sensitive RT-PCR method during a 3-year-period. From February 1998 to January 2001, 263 infants under 4 months of age were admitted to our hospital because of fever (>38.0 degrees C) and enrolled in this study. RESULTS: Eighty-nine cases (33.8%) were diagnosed as lower respiratory infections, and 62 (23.5%) as urinary tract infections. Aseptic meningitis was found in 35 cases (13.2%) and fever of unknown origin in 22 (8.3%). Sepsis and purulent meningitis were found in three and two infants, respectively. Aseptic meningitis was the most common disease in infants below 30 days. Aseptic meningitis and fever of unknown origin revealed no seasonable tendency. Twelve cases out of 20 tested infants with aseptic meningitis showed positive results for enterovirus in CSF. CONCLUSION: Clinicians should consider age, sex and seasons when evaluating and treating infants with fever. Previously lumbar puncture, urinalysis and chest X-ray were recommended to avoid useless assays. These results also revealed that RT-PCR is a useful and significant assay to establish an exact diagnosis in very young infants.
Bacterial Infections↗
A case of Reye syndrome with rotavirus infection accompanied with high cytokines.
We report on a 23-month-old boy with a rare complication of rotavirus gastroenteritis. He was diagnosed as acute encephalopathy with DIC accompanied with high levels of cytokines. The liver pathology also revealed mild infiltration and fatty changes. He was suspected to be suffering from a cytokine storm followed by Reye syndrome.
Anticonvulsants↗
Nitrite/nitrate (NOx) and zinc concentrations in influenza-associated encephalopathy in children with different sequela.
NOx (NO2 and NO3) in CSF obtained from 22 patients with influenza-associated encephalopathy were higher than those of a control group. Within the different prognosis, there were no significant differences in NOx levels. By analyzing the serum obtained from patients infected with influenza, including encephalopathy, with others, the serum zinc levels did show marked differences between them. Four out of eleven patients with influenza-associated encephalopathy showed low zinc levels below the normal range. However, there were no significant differences in the zinc levels between the group with sequela and without sequela. These results indicate that the increase of NOx levels detected in influenza-associated encephalopathy relates to the low zinc levels, and both low molecules might play an important role for the cause of encephalopathy.
Brain Damage, Chronic↗
Efficacy of plasma exchange and methylprednisolone pulse therapy on influenza-associated encephalopathy.
Patients with a new type of influenza-associated encephalopathy with high mortality are increasing in Japan and the United States. We present three patients treated with methyprednisolone pulse treatment and plasma exchange to remove cytokines, and all three patients recovered without severe sequela. IL-6 decreased dramatically after the start of the plasma exchange and methyprednisolone. Therefore when influenza-associated encephalopathy is actually diagnosed, steroid pulse therapy should be started at an early stage, and when signs of DIC and/or MOF appear, plasma exchange is recommended to remove the cytokines and NOx.
Brain Diseases↗
Clinical application of rapid assay of interleukin-6 in influenza-associated encephalopathy.
The characteristics of influenza-associated encephalopathy is the high mortality and nimble progress with coma which appears in general cases within 48 hours. Most of patients show no abnormalities in the standard blood checks on admission or in early stage. In this study we investigated if a rapid assay of interleukin (IL)-6 is useful in influenza-associated encephalopathy in early stages. The levels of IL-6 in patients with influenza-associated encephalopathy did not show any significant difference compared with those in patients with febrile convulsion and rotavirus-associated convulsion. However the levels of IL-6 in severe cases were significantly higher than those of mild cases with influenza-associated encephalopathy. Consequently the rapid assay of serum IL-6 is useful to evaluate and decide the therapies.
Biomarkers↗
Serum and cerebrospinal fluid nitrite/nitrate levels in patients with rotavirus gastroenteritis induced convulsion.
Nitric oxide (NO) is a highly reactive free radical that is involved in a variety of different biological process. In recent reports, the putative role of NO in the neuropathogenesis of brain inflammation has been demonstrated. And then the relation between neuronal NO and convulsive seizures induced by virus has been suggested. However, there are few reports about NO in vivo under viral neurological infections. In order to evaluate the relation between NO production and neurological disorders induced by viral infection, sixty-six cases including 11 patients with rotavirus gastroenteritis admitted for convulsions were examined in this study. NO metabolites (NOx) levels in both serum and cerebrospinal fluid obtained from rotavirus gastroenteritis patients with convulsion were much higher than in those of patients with purulent meningitis, encephalitis, febrile convulsion or in the control group. There was a relative correlation between IL-6 and NOx in some cases. These results indicated that NO may have a pathophysiological role in convulsions associated by rotavirus infection either through indirect or direct effects of NO. Consequently, NOx inhibitors might be helpful for the treatment of rotavirus encephalopathy.
Child, Preschool↗
Virological and immunological characteristics of a 19-year-old Japanese female with fatal outcome with Epstein-Barr virus-associated hemophagocytic syndrome.
Hemophagocytic syndrome (HPS) is caused by the hyperactivation of T-cells and macrophages. The clinical characteristics associated with this disease result from overproduction of cytokines including interferon-gamma (INF-gamma), tumor necrosis factor-alpha (TNF-alpha), interleukin-6 (IL-6). HPS presents with fever, liver dysfunction, coagulation abnormalities, pancytopenia, and a benign histiocytic proliferation with prominent hemophagocytosis in bone marrow, lymph node, spleen, and liver. We describe a 19-year-old female with fatal HPS. She had been given corticosteroid every other day for systemic lupus erythematosus (SLE) without flare up. The causative underlying disease was acute primary Epstein-Barr virus (EBV) infection. EBV genomes were detected by the polymerase chain reaction (PCR). To measure the virus load we use a real-time PCR assay to quantify the amount of EBV DNA in peripheral blood lymphocytes, lung, kidney, brain and liver at autopsy. Further in situ hybridisation (ISH) and immunohistochemical studies demonstrated that Epstein-Barr virus encoded small RNA (EBER) was detected in CD8+ T-cells in bone marrow, lung, kidney, brain, liver and spleen. In each organ, mRNA levels of inflammatory cytokines (INF-gamma, TNF-alpha, IL-6) were highly detected compared with beta-Actin mRNA levels. These results suggest that EBV-infected CD8+ T-cells in each organ (peripheral blood lymphocytes, lung, kidney, brain and liver) may have an integral role in the pathophysiology of the HPS.
Adult↗
Sudden infant death syndrome due to parainfluenza virus 2 associated with hemophagocytic syndrome.
We report a child with Sudden Infant Death Syndrome (SIDS), aged 16 months. The histological findings of tonsils, spleen, and bone marrow revealed many hemophagocytic cells. Parainfluenza virus type 2 (PIV2) was cultured in the nasopharynx and detected by reverse-transcription (RT)-PCR in liver tissue and bone marrow. His laboratory data of elevated level of ferritin and IL-6 suggested hemophagocytic syndrome (HPS). It is suspected that PIV2 infection in infants is a risk factor for SIDS.
Histiocytosis, Non-Langerhans-Cell↗
Enterovirus-related type 1 diabetes mellitus and antibodies to glutamic acid decarboxylase in Japan.
OBJECTIVES: In order to clarify the relationship between enteroviruses and type 1 diabetes mellitus in Japan we investigated enteroviral RNA in serum from children with type 1 diabetes mellitus. METHODS: We investigated enteroviral RNA in serum from children with type 1 diabetes mellitus by using highly sensitive RT-PCR. Additionally the sequences and viral loads were determined and compared with anti-coxsackie virus antibodies and anti-glutamic acid decarboxylase (GAD) antibodies. RESULTS: RT-PCR for enterovirus was positive in 23 (37.7%) from 61 samples. The positivity had no disparity of age, but decreased by aging after the occurrence of type 1 diabetes mellitus. The sequences of the positives were similar as those of coxsackie B4. The viral loads revealed that there was no positive patient with high titers of anti-GAD antibodies. CONCLUSION: In Japan there is some correlation with type 1 diabetes mellitus and enterovirus. The pathophysiology of type 1 diabetes mellitus seems to consist of a direct destruction by persistent coxsackie virus and the autoimmune mechanism through autoantibodies against beta-cells.
Adolescent↗
A case report of Kabuki make-up syndrome with cytomegalovirus infection.
An 8-month-male infant was admitted to our institute in order to investigate his developmental delay. He had facial features-long palpebral fissures with eversion of the lower lateral eyelids, arched eyebrows with lateral sparseness, depressed nasal tip, large, prominent and cupped ears. From these characteristical features, he was diagnosed as having Kabuki make-up syndrome (KS). When he was 2 months old, he was admitted to our institute because of intractable stridor and liver dysfunction associated with cytomegalovirus (CMV) infection. In KS, increased susceptibility to infection is described. We suspected persistent CMV infection because of an increased susceptibility to infection in KS. Recently acute idiopathic thrombocytopenic purpura (ITP) was diagnosed. According to the correlation between the number of CMV DNA copies and his platelet count, it is speculated that ITP would occur when the number of CMV DNA copies was elevated.
Abnormalities, Multiple↗
Extraordinary changes in excitatory amino acid levels in cerebrospinal fluid of influenza-associated encephalopathy of children.
The correlation between the glutamate-glutamine cycle and nitric oxide (NO) production in the central nervous system (CNS) of a new type of influenza-associated encephalopathy in children is discussed. When measurements of several amino acids and NOx (nitrite/nitrate) levels in the cerebrospinal fluid (CSF) using HPLC-fluorescence and -UV methods, respectively, were made. the CSF glutamate levels of patients with the new type of encephalitis were significantly lower, and both glutamine and NOx levels were significantly higher than those of the control group and the patients of the meningitis group. Results indicate that the turnover rate of glutamate in CNS, particularly in the brain, increases in the influenza-associated encephalopathy. The high mortality in the disease may correlate with the hyperactivity of supra-spinal glutamate neurons and the subsequent high activity levels of NOx in CNS.
Brain Diseases↗
Enteroviral hepatitis in children.
BACKGROUND: The pathogen causing enteroviral hepatitis is often not found despite careful examination. METHODS: This study investigates the enterovirus genome in serum and liver tissue obtained from patients who showed abnormal liver function without negative data of usual studies and cytomegalovirus (CMV) serologically positive cases.' RESULTS: Nine out of 21 serum samples were positive by using reverse transcriptase-polymerase chain reaction (RT-PCR) for enterovirus. The 21 samples had CMV-IgM antibodies in five cases. These CMV serologically positive cases were all negative for enterovirus using RT-PCR. Therefore, nine out of 16 (60%) were of unknown etiology. Some cases showed liver dysfunction over a period of more than 6 months. The liver function revealed that all cases finally improved. The sequences coincided with those of Coxsackie B5 or B6 with the highest score by gene homology search. The liver pathology revealed that two of three subjected cases had mild fibrosis and small cell infiltration. RT-PCR of liver tissue for enterovirus were positive in all three cases comparing the house keeping gene. The viral load was high in acute phase and low in convalescent phase. CONCLUSIONS: In more than half of children with illnesses of unknown etiology, the pathogen was found to be enteroviruses, and RT-PCR and quantification of serum is an easy method to identify these diseases.
Child, Preschool↗
Rapid diagnosis of bacterial meningitis by using multiplex PCR and real time PCR.
BACKGROUND: The purpose of the present study was to improve a method for a rapid identification of bacteria in bacterial meningitis by using multiplex polymerase chain reaction (PCR). METHODS: Ten species of bacteria which cause meningitis in children were investigated, and cerebrospinal fluid from patients with purulent meningitis was studied. The ribosomal RNA genes of bacteria are essential, and are highly conserved in the bacterial kingdoms with consensus region. The 23S rRNA region shows a larger variation among species than in the 16S rRNA region. The authors set primers in the universal region and specific region of 23S rRNA, then amplified these regions by multiplex PCR and real-time PCR. RESULTS: All species of bacteria showed one band by PCR using universal primer. Haemophilus influenzae and Streptococcus pneumoniae showed two bands by multiplex PCR using a combination of universal primers and specific primers. The authors detected H. influenzae within 15 min by using real-time PCR. CONCLUSION: It was possible to identify clinically significant bacterial species in cerebrospinal fluid by multiplex PCR, and to identify H. influenzae by real-time PCR within a short period.
Cerebrospinal Fluid↗
[Mumps virus].
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Diagnosis, Differential↗