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SETTING: Diagnosis of Mycobacterium avium complex pulmonary disease (MAC-PD) requires positive culture of expectorated sputum or specimens acquired by bronchoscopy. Whether patients diagnosed using bronchoscopy have milder disease and milder progression than those diagnosed using sputum remains uncertain.OBJECTIVE: To clarify whether disease severity and progression differ according to the diagnostic method.METHODS: We retrospectively analysed 92 patients with MAC-PD. We compared characteristics of patients and disease progression according to the diagnostic methods used: sputum or bronchoscopy. Additionally, we investigated the impact of these methods on disease progression using multivariate analysis.RESULTS: Patients diagnosed using sputum were younger than those diagnosed using bronchoscopy; however, there were small differences from the viewpoint of clinical practice in disease severity, and estimated progression-free survival rate did not differ significantly. The predictors of disease progression were disease forms other than non-cavitary nodular/bronchiectatic disease, hypoalbuminemia and severe radiographic scores.CONCLUSION: The diagnostic methods had no significant impact on disease severity and disease progression of MAC-PD. If the diagnosis cannot be established by sputum culture or if sputum cannot be obtained in the patients with risk factors for disease progression, bronchoscopy would be useful to provide opportunity of treatment for MAC-PD.

CONTEXTUALIZAÇÃO: Em função da complexidade das manifestações clínicas da paralisia cerebral (PC) e das dificuldades na sua classificação baseada apenas nos tipos motores e topografia de distribuição corporal, estudiosos canadenses propuseram o Gross Motor Function Classification System (GMFCS). Apesar de esse sistema de classificação estar sendo bastante utilizado no Brasil, ele ainda não havia sido adapatado transculturalmente para tal. OBJETIVOS: Realizar a adaptação transcultural para o Brasil do Sistema de Classificação da Função Motora Grossa (GMFCS) para a PC e verificar a confiabilidade entre observadores do instrumento adaptado com crianças brasileiras. MÉTODOS: Este estudo consistiu em duas etapas, sendo a primeira relacionada com o processo de adaptação transcultural, e a segunda referente à testagem do instrumento. A adaptação transcultural do instrumento foi feita por meio da tradução, retrotradução, análise semântica, análise de conteúdo, retrotradução da versão final e aprovação dos autores do instrumento. A testagem do instrumento ocorreu junto a 40 crianças com PC, as quais foram avaliadas por dois examinadores para verificar a confiabilidade entre observadores. RESULTADOS: Os resultados demonstram que as etapas de tradução e retrotradução não apresentaram dificuldades, e a equivalência semântica e a conceitual foram obtidas. A confiabilidade entre examinadores demonstrou que as avaliações quase não diferiam e que havia excelente correlação e consistência interna do constructo, com coeficiente de correlação intraclasse (ICC) de 0,945 (com intervalo de 0,861 a 0,979) e a de Cronbach de 0,972. CONCLUSÕES: A versão final do GMFCS mostrou bom potencial de aplicabilidade por graduandos e profissionais da área de neuropediatria.BACKGROUND: Due to the complexity of clinical symptoms of cerebral palsy and the difficulties in classifying it based upon the motor types and the topography of the body distribution only, Canadian researchers have proposed the Gross Motor Function Classification System (GMFCS). Although this classification system has been largely used in Brazil, it has not been cross culturally adapted yet. OBJECTIVES: To perform the cross adaptation of the Gross Motor Function Classification System for the Cerebral Palsy (GMFCS) into Brazilian-Portuguese and to verify the reliability among observers of the adapted instrument in Brazilian children. METHODS: This study was performed in two stages; the first stage was related to the process of cross-cultural adaptation and the second stage tested the instrument. Translation, back-translation, semantic and content analysis, back-translation of the final version and the approval of the authors were used for the cross-cultural adaptation. The test of the instrument was performed in 40 children with cerebral palsy, who were evaluated by two raters to verify the reliability among the observers. RESULTS: The results showed that the stages of translation and back-translation did not present any difficulties and the semantic and conceptual equivalence was achieved. The reliability among the observers showed that the evaluations do not differ and that there is an excellent correlation and internal consistency of the construct with an ICC of 0.945 (95% CI 0.861 to 0.979) and a Cronbach a of 0.972. CONCLUSIONS: The final version of the GMFCS showed good potential of applicability for undergraduate students and professionals of the neuropediatric area.

Eighteen Swedish patients with familial amyloidotic polyneuropathy were tested for the met30 mutation of the transthyretin (TTR) (prealbumin) gene by RFLP analysis of genomic DNA using the restriction enzyme NsiI. The results confirmed previous findings that the Swedish variant of familial polyneuropathy has the same valine by methionine substitution at position 30, as seen in patients with FAP from Japan, Portugal or patients of Swedish descent from USA. However, two of the patients were homozygous, totally lacking the wild type allele. Measurable serum values for the variant transthyretin (TTR) was detected with a RIA-method in all the 18 Swedish FAP patients studied with a mean concentration of 12.1 +/- 5.1 (SD) mg/100 ml, (11.0 +/- 4.1 when the two homozygotes were excluded). In 45 Japanese patients the mean was 9.2 +/- 2.7 mg/100 ml. The variant TTR was not detected in the healthy controls. The value for the variant TTR was nearly twice that high in the two homozygous patients, 21.14 and 21.16 mg/100 ml, respectively. There was no correlation between the serum levels of variant TTR and the duration of disease or levels of serum albumin in the FAP-patients.

O objetivo deste trabalho consistiu em verificar o caráter das relações entre os membros das equipes de saúde da família e sua forma de atuação, por meio da identificação de temáticas que promovam convergência e divergência entre os profissionais de nível universitário e os de nível médio. Foi desenvolvido um estudo qualitativo, com a aplicação da técnica de grupo focal, para identificar a opinião e a percepção dos profissionais das equipes do PSF sobre os cursos de capacitação de que participaram e sobre as atividades desenvolvidas nas Unidades de Saúde da Família. Foram cinco os temas abordados: capacitação da equipe, responsabilidades na equipe, retaguarda ao PSF, relação com a comunidade e o PSF como modelo assistencial. As temáticas que geraram mais consenso entre os dois grupos, profissionais universitários e profissionais de nível médio, foram: a capacitação da equipe e a retaguarda ao PSF. Os demais temas - responsabilidades na equipe, relação com a comunidade e o PSF como modelo assistencial - geraram divergências. A pesquisa também possibilitou identificar a existência de dificuldades nas relações entre os membros da equipe e de limitações no sentido de uma atuação integrada e interdisciplinar.The objective of this work is to verify the nature of the relationship between the members of family health teams and also how they perform at work. This was done by identifying themes that promote convergence and divergence between professionals that were divided, for this purpose, into two groups: those with high education and those with secondary education. Methodologically, we developed a qualitative study, using focal group technique, to determine the views and perceptions of the PSF team members about the training courses they attended and about the activities developed in the Family Health Units. The themes covered were five, namely: team's training, team's responsibilities, stand-by support for the PSF, relationship with the community and the PSF as a care model. The themes that led to greater consensus between the two groups were team's training and stand-by support for the PSF. The other themes - team's responsibilities, relationship with the community and PSF as a care model - led to disagreements. The research also enabled us to verify that are some problems in the relationship between team members and limitations in their performance with respect to integrated and interdisciplinary actions.

O objetivo deste trabalho consistiu em verificar o caráter das relações entre os membros das equipes de saúde da família e sua forma de atuação, por meio da identificação de temáticas que promovam convergência e divergência entre os profissionais de nível universitário e os de nível médio. Foi desenvolvido um estudo qualitativo, com a aplicação da técnica de grupo focal, para identificar a opinião e a percepção dos profissionais das equipes do PSF sobre os cursos de capacitação de que participaram e sobre as atividades desenvolvidas nas Unidades de Saúde da Família. Foram cinco os temas abordados: capacitação da equipe, responsabilidades na equipe, retaguarda ao PSF, relação com a comunidade e o PSF como modelo assistencial. As temáticas que geraram mais consenso entre os dois grupos, profissionais universitários e profissionais de nível médio, foram: a capacitação da equipe e a retaguarda ao PSF. Os demais temas - responsabilidades na equipe, relação com a comunidade e o PSF como modelo assistencial - geraram divergências. A pesquisa também possibilitou identificar a existência de dificuldades nas relações entre os membros da equipe e de limitações no sentido de uma atuação integrada e interdisciplinar.

A 19-year-old man was admitted to our hospital for delayed puberty. At birth, he had macrocephalia and showed delayed physical and mental development. At 9 years of age, right cryptorchism was diagnosed. His parents had noticed that he could not recognize any smells since his infancy. Physical examination on admission revealed ocular hypertelorism, high myopia, high arched palate, and intermittent external strabismus. Sense of smell was scaled out by olfactometry. External genitalia were infantile. Neurological examination showed on IQ of 83, and mild truncal ataxia. Magnetic resonance imaging (MRI) showed a cystic distension of the IV ventricle, partial aplasia of the cerebellar vermis, elevation of the tentorium cerebelli, enlargement of the III ventricle, and agenesis of the corpus callosum. These findings revealed that the patient had Dandy-Walker malformation. The basal FSH, LH, and testosterone levels were all low compared with normal adult reference values. The serial LH-RH provocation tests showed stepwise LH and FSH elevation. After the fifth day of LH-RH administration, both LH and FSH responses clearly improved. Olfactory tracts were defective in MRI findings. These findings were consistent with hypogonadotropic hypogonadism of hypothalamic origin with anosmia, and the patient was therefore diagnosed with Kallmann syndrome. Sequence analysis of the KAL1 gene showed no mutation in the coding region. To our knowledge, this is the first case report of the coexistence of Kallmann syndrome and Dandy-Walker malformation in the same patient.

Com o objetivo de realizar a versão e estudos psicométricos do Questionário de Suporte Social de Sarason, aplicou-se o instrumento em 113 mulheres-mães. Para a análise, utilizou-se a estatística Kappa, o Índice de Concordância Bruta e a Correlação de Spearman. A consistência interna do instrumento foi avaliada. Os resultados indicam uma fidedignidade teste-reteste aceitável e um alto nível de consistência interna. Observaram-se associações estatísticas entre os índices de suporte social e as variáveis trabalho, gravidez e renda. Apresentam-se informações sobre fontes de suporte social e extensão da rede as quais sugerem uma participação maior da família próxima como fornecedora de suporte.Con el objetivo de realizar la versión y estudios psicométricos del Cuestionario del Apoyo Social de Saranson, se empleó el instrumento en 113 mujeres-madres. Para la análisis se utilizó la estatística Kappa, el Índice de Concordancia Bruta y la Correlación de Spearman. Se estimó la consistencia interna del instrumento. Los resultados muestran una fidelidad test-retest acetable y un elevado nivel de consistencia interna. Se observaron asociaciones estadísticas entre los índices de apoyo social y las variables trabajo, gestación y renta. Se presentan informaciones sobre fuentes de soporte social y extensión de la red¿, las cuales sugieren una mayor participación de la familia como proveedora del apoyo.With the aim of developing psychometrical studies using the Saranson's Social Support Questionnaire, an adapted version of the instrument was applied to 113 mothers. The Kappa Statistical Parameter, the Index of Gross Agreement and Spearman's Correlation were used to the analysis. The analysis of the internal consistency instrument showed a high level of it. In addition, the results indicated an acceptable test-retest reliability as well as statistical relationships between the social support indexes and variables related to work, pregnancy and income. Information on social support sources and the extension of the network suggest a greater participation of the family as the provider of the support.

An acromegalic patient with a pituitary somatotroph adenoma associated with an extremely elevated plasma GHRH concentration is presented. The preoperatively high concentration of plasma GHRH returned to the normal level after successful removal of the adenoma. GHRH production and GHRH gene expression were confirmed in the adenoma by studies including immunohistochemistry and in situ hybridization. Expression of GHRH receptor messenger ribonucleic acid was verified by in situ hybridization. Immunohistochemical double staining for GH and GHRH revealed their colocalization in single adenoma cells. These findings confirmed the autocrine or paracrine regulation of GH production by endogenous GHRH from the adenoma cells. GHRH synthesis in the pituitary gland has recently been demonstrated, however, there have been no previous reports of a GHRH-producing pituitary somatotroph adenoma associated with an elevated plasma GHRH concentration. The existence of this GHRH-producing adenoma suggests a possible role of locally generated GHRH in the progression of somatotroph adenomas, i.e. the monoclonally established somatotroph adenomas develop further under the influence of locally produced GHRH. The demonstration of GHRH production by this somatotroph adenoma is of importance in clarifying the autocrine or paracrine regulation of GH production and the progression of human somatotroph adenomas.

Previous studies established that variable degrees and types of glycosylation can account for differences in the ability of CD44 to function as a receptor for hyaluronic acid. We have now used neuraminidase treatment to conclude that sialylation negatively regulates CD44 on the human monocytic cell line THP-1 and peripheral blood monocytes. Both of these cell types displayed increased receptor activity after overnight culture with LPS. Of particular interest, the sialidase inhibitor 2-deoxy-2,3-dehydro-N-acetylneuraminic acid completely blocked the LPS induced recognition of hyaluronic acid by THP-1 cells. Furthermore, acquisition of this characteristic paralleled induction of one type of sialidase activity. Monocytes may be capable of enzymaticly remodeling cell surface CD44, altering their ability to interact with the extracellular matrix.

A double-blind, multi-center study was performed on patients with HTLV-I-associated myelopathy (HAM) to evaluate the therapeutic effect of treatment with natural interferon-alpha (HLBI). Forty-eight HAM patients were enrolled and treated with either 0.3 MU (n = 15), 1.0 MU (n = 17), or 3.0 MU (n = 16) of HLBI for 28 days. Clinical evaluation included motor dysfunction, urinary disturbances, and changes of neurologic signs. The frequency of therapeutic response judged as excellent to good 4 weeks after starting therapy and 4 weeks after completion of therapy were 7.1% (1 of 14) and 8.3% (1 of 12) in the 0.3-MU group, 23.5% (4 of 17) and 26.7% (4 of 15) for the 1.0-MU group, and 66.7% (10 of 15) and 61.5% (8 of 13) for the 3.0-MU group. The therapeutic benefit in the 3.0-MU group was significantly higher than in the 0.3-MU group. There was no significant difference in the incidence of symptomatic side effects between groups. Abnormal laboratory data were obtained for some patients in the 1.0-MU and 3.0-MU groups; however, the treatment schedule could be continued in most patients. These results suggest that HAM patients may be safely treated with HLBI 3.0 MU every day for 4 weeks with favorable clinical effects.

Human neutrophil peptides (HNPs) 1, 2 and 3, also called defensins, have high antimicrobial activity and are cytotoxic to both normal and transformed human cells. The localization of HNPs 1–3 and their mRNAs in cells of normal human bone marrow and peripheral blood was investigated. Immunoreactivity for HNPs 1–3 appeared in the promyelocyte stage and increased with the maturation of the neutrophil series. HNPs 1–3 were absent from such mononuclear phagocyte systems as peripheral monocytes, alveolar macrophages, and Kupffer cells in the liver. HNP 1–3 mRNAs were detected in bone marrow cells by Northern blot analysis and in peripheral leukocytes by the reverse transcription-polymerase chain reaction (RT-PCR). HNPs 1–3 are restricted to cells of neutrophil lineage and seem to function as microbicidal agents in the neutrophil-mediated defense system.

Increased expression of IL-17F has been noted in the airway of asthmatic patients, but its role in asthma has not been fully elucidated. Insulin-like growth FACTOR-I (IGF-I) is known to be involved in airway remodelling and inflammation, while its regulatory mechanisms remain to be defined.To further clarify the biological function of IL-17F, we investigated whether IL-17F is able to regulate the expression of IGF-I in bronchial epithelial cells.Bronchial epithelial cells were stimulated with IL-17F in the presence or absence of T-helper type 2 cytokines. Various kinase inhibitors were added to the culture to identify the key signalling events leading to the expression of IGF-I, in conjunction with the use of short interfering RNAs (siRNAs) targeting mitogen- and stress-activated protein kinase (MSK) 1, p90 ribosomal S6 kinase (p90RSK), and cyclic AMP response element-binding protein (CREB).IL-17F significantly induced IGF-I gene and protein expression, and co-stimulation with IL-4 and IL-13 augmented its production. MAP kinase kinase (MEK) inhibitors and the Raf1 kinase inhibitor significantly inhibited IGF-I production, and the combination of PD98059 and Raf1 kinase inhibitor showed further inhibition. Overexpression of Raf1 and Ras dominant-negative mutants inhibited its expression. MSK1 inhibitors significantly blocked IL17F-induced IGF-I expression. Moreover, transfection of the siRNAs targeting MSK1, p90RSK, and CREB blocked its expression.In bronchial epithelial cells, IL-17F is able to induce the expression of IGF-I via the Raf1-MEK1/2-ERK1/2-MSK1/p90RSK-CREB pathway in vitro.

Due to the complexity of clinical symptoms of cerebral palsy and the difficulties in classifying it based upon the motor types and the topography of the body distribution only, Canadian researchers have proposed the Gross Motor Function Classification System (GMFCS). Although this classification system has been largely used in Brazil, it has not been cross culturally adapted yet.To perform the cross adaptation of the Gross Motor Function Classification System for the Cerebral Palsy (GMFCS) into Brazilian-Portuguese and to verify the reliability among observers of the adapted instrument in Brazilian children.This study was performed in two stages; the first stage was related to the process of cross-cultural adaptation and the second stage tested the instrument. Translation, back-translation, semantic and content analysis, back-translation of the final version and the approval of the authors were used for the cross-cultural adaptation. The test of the instrument was performed in 40 children with cerebral palsy, who were evaluated by two raters to verify the reliability among the observers.The results showed that the stages of translation and back-translation did not present any difficulties and the semantic and conceptual equivalence was achieved. The reliability among the observers showed that the evaluations do not differ and that there is an excellent correlation and internal consistency of the construct with an ICC of 0.945 (95% CI 0.861 to 0.979) and a Cronbach a of 0.972.The final version of the GMFCS showed good potential of applicability for undergraduate students and professionals of the neuropediatric area.

O objetivo deste trabalho consistiu em verificar o caráter das relações entre os membros das equipes de saúde da família e sua forma de atuação, por meio da identificação de temáticas que promovam convergência e divergência entre os profissionais de nível universitário e os de nível médio. Foi desenvolvido um estudo qualitativo, com a aplicação da técnica de grupo focal, para identificar a opinião e a percepção dos profissionais das equipes do PSF sobre os cursos de capacitação de que participaram e sobre as atividades desenvolvidas nas Unidades de Saúde da Família. Foram cinco os temas abordados: capacitação da equipe, responsabilidades na equipe, retaguarda ao PSF, relação com a comunidade e o PSF como modelo assistencial. As temáticas que geraram mais consenso entre os dois grupos, profissionais universitários e profissionais de nível médio, foram: a capacitação da equipe e a retaguarda ao PSF. Os demais temas - responsabilidades na equipe, relação com a comunidade e o PSF como modelo assistencial - geraram divergências. A pesquisa também possibilitou identificar a existência de dificuldades nas relações entre os membros da equipe e de limitações no sentido de uma atuação integrada e interdisciplinar. The objective of this work is to verify the nature of the relationship between the members of family health teams and also how they perform at work. This was done by identifying themes that promote convergence and divergence between professionals that were divided, for this purpose, into two groups: those with high education and those with secondary education. Methodologically, we developed a qualitative study, using focal group technique, to determine the views and perceptions of the PSF team members about the training courses they attended and about the activities developed in the Family Health Units. The themes covered were five, namely: team's training, team's responsibilities, stand-by support for the PSF, relationship with the community and the PSF as a care model. The themes that led to greater consensus between the two groups were team's training and stand-by support for the PSF. The other themes - team's responsibilities, relationship with the community and PSF as a care model - led to disagreements. The research also enabled us to verify that are some problems in the relationship between team members and limitations in their performance with respect to integrated and interdisciplinary actions.

With the aim of developing psychometrical studies using the Saranson's Social Support Questionnaire, an adapted version of the instrument was applied to 113 mothers. The Kappa Statistical Parameter, the Index of Gross Agreement and Spearman's Correlation were used to the analysis. The analysis of the internal consistency instrument showed a high level of it. In addition, the results indicated an acceptable test-retest reliability as well as statistical relationships between the social support indexes and variables related to work, pregnancy and income. Information on social support sources and the extension of the network suggest a greater participation of the family as the provider of the support.

An improved hardware architecture for high-speed data processing with a high-speed update rate of 60 displays/s is described. This technique is applied to a general purpose digital oscilloscope, the DL 1200. An overview is provided of the technique and the new product. >

We assessed the clinical features in twenty-three cases diagnosed as paragonimiasis westermani from April 1988 to May 2000 in our clinic at Miyazaki Medical College Hospital. Twenty-one patients had eaten wild boar or crab meat. Their symptoms included productive cough, chest pain and hemosputum. The findings of radiography and computed tomography (CT) were nodules, masses, infiltrative shadows and pleural effusion. The percentages of eosinophils in the peripheral blood were elevated in 16 of 23 patients, and the serum IgE values were elevated in 12 of 18 patients. Serologic tests, which are very helpful for diagnostic purposes were performed on the blood sera of all patients, and on the pleural effusions of five patients. It is often difficult to diagnose paragonimiasis westermani in patients in whom eosinophilia or increased serum IgE is not present. However, paragonimiasis westermani should also be considered as a possible diagnosis when abnormal shadows are found in chest radiographs and chest CT.

A 50-year-old woman who had been treated for Parkinson's disease was admitted to our hospital because of exertional dyspnea and weight loss. Her chest computed tomography showed diffuse ground glass opacities in both lung fields. Bronchofiberscopy was performed, and the bronchoalveolar lavage fluid showed an elevated lymphocyte count. The serum Ouchterlony test yielded positive results for parrot droppings as well as for pigeon serum and droppings. She had had a parrot for several years, but no pigeon, and so we diagnosed bird fanciers' disease associated with parrot droppings. This responded well to steroid therapy. This case of bird fanciers' disease was caused by the droppings of only one parrot.

Respiratory virus is one of the most common causes of airway inflammation, but its pathogenic mechanisms are not well understood. Eotaxin is a potent eosinophil chemoattractant and is a selective agonist for C-C chemokine receptor 3 (CCR3). Although it has recently been demonstrated that epithelial cells express eotaxin, both in vivo and in vitro, there are few data concerning the expression in viral infection.We hypothesized that eotaxin may play an important role in attracting inflammatory cells into the airway after viral infection and analysed whether viral infection induces eotaxin in nasal epithelial cells in vitro.Nasal epithelial cells obtained from polypectomy for nasal polyp were infected with influenza virus A (subtype H3N2). The cells and supernatants were collected 8, 24 and 48 h after infection. Eotaxin mRNA was analysed by RT-PCR. Eotaxin concentration in the supernatants was analysed by enzyme-linked immunosorbent assay. We also examined a blocking assay to analyse the intervention of pro-inflammatory cytokines, TNF-alpha and IL-1beta in eotaxin production induced by influenza virus.The results showed that eotaxin was expressed constitutively in uninfected cells, but was up-regulated for both mRNA and protein levels in infected cells. Blocking experiments using anti-TNF-alpha and anti-IL-1beta antibodies showed no effects of these agents on the level of eotaxin. In addition, UV-inactivated virus did not enhance the expression of eotaxin.These results suggest that influenza virus A infection in nasal epithelial cells stimulates the expression of eotaxin, and may play an important role in the pathogenesis of airway inflammation by inducing eotaxin.

We measured the levels of beta-defensin 1 and 2 (HBD-1, 2), novel antimicrobial peptides in plasma and in bronchoalveolar lavage fluid (BALF) from patients with with non-tuberculous mycobacterium infection (NTM). Plasma HBD-2 levels in NTM patients before treatment were higher than those in the controls, while the HBD-1 levels were similar to the control levels. High levels of HBD-2, but not of HBD-1, in BALF were also observed in NTM patients. In NTM, a positive correlation was found between HBD-2 levels in BALF and plasma, and also between HBD-2 and IL-1 beta levels in BALF. NTM patients with cavities or ectasia on chest radiography had higher HBD-2 levels in BALF than those without. Plasma HBD-2 levels in NTM patients were markedly decreased after successful treatment, while those of patients with an intractable mycobacterium infection maintained the same high plasma HBD-2 levels as those before treatment. These findings suggest that HBD-2 may participate in the host defense and plasma HBD-2 levels may reflect disease activity in pulmonary NTM.

The hypothalamus is the most important region in the control of food intake and body weight. The ventromedial 'satiety center' and lateral hypothalamic 'feeding center' have been implicated in the regulation of feeding and energy homeostasis by various studies of brain lesions. Orexins(orexin A and orexin B) and melanin-concentrating hormone (MCH), whose intracerebroventricular injections increase food intake, are localized in the lateral hypothalamus and provide diffuse projections throughout the brain. Orexins and MCH neurons have a coextensive distribution, but are not colocalized. Orexins and MCH may affect feeding behavior through distinct neuronal pathways. We here describe the effect of orexins and MCH on feeding behavior from the physiological, neuroanatomical and molecular studies.

A 49-year-old man was admitted to our hospital with a productive cough and bloody sputum. Peripheral blood eosinophil counts and serum IgE values were elevated. Chest radiography and chest computed tomography revealed a solitary nodule with cavitation and satellite lesions in the right middle lobe, in contact with the pleura. The diagnosis of paragonimiasis westermani was confirmed by detection of ova in the sputum and bronchoalveolar lavage fluid (BALF) samples, and by immunoserological examination. The patient was treated with praziquantel for 3 days at a daily dosage of 75 mg/kg. After treatment, his clinical symptoms and radiological abnormalities disappeared. The IL-5 level was high in BALF, but it was undetectable in the plasma, suggesting that IL-5 in inflammatory lesions may be particularly important in mediating eosinophilia in the blood and BALF in paragonimiasis westermani.

We performed electrophysiologic tests on two patients with digitalis toxicity who first had photophobia and xanthopsia and revealed reversible reduced visual acuity and binocular central scotoma.The patients were a 72-year-old male and a 54-year-old male who had symptoms of digitalis toxicity.The corrected visual acuity was severely decreased during digitalis toxicity, 0.02 oculus dexter (OD) and 0.1 oculus sinister (OS) in case 1 and 0.04 OD and 0.2 OS in case 2. But visual acuity recovered as the blood levels of digitalis decreased to the normal level and the corrected visual acuity was 0.7 OD and 0.8 OS in case 1 and 0.8 OD and 0.9 OS in case 2. We recorded 30 Hz-flicker electroretinogram (ERG), single flash ERG, photopic ERG, and scotopic ERG when digitalis blood levels were elevated and normal. Decreased amplitudes of 30 Hz-flicker ERG and photopic ERG suggested that photoreceptor function was disturbed at digitalis toxicity and cone dysfunction was more severely disturbed than rod dysfunction.30 Hz-flicker ERG, as well as electrocardiogram and digitalis blood level, is a relatively convenient and useful measure of digitalis toxicity. It is necessary consiler toxicity when severe visual dysfunction is observed in patients with digitalis therapy.

A 69-year-old woman visited her physician on October 1 complain of dry cough. However, the chest radiograph revealed no abnormalities. She was later admitted to our hospital because a radiograph taken by another physician on November 26 revealed a massive lesion in the right upper mediastinum. Computed tomographic findings showed a massive lesion containing a branching structure with a few calcifications, suggesting a case of atelectasis of the left upper lobe with mucus plug. Bronchoscopic examination revealed complete obstruction of the orifice of the left upper lobe bronchus, and so a diagnosis of bronchial atresia was made. However, since the patient had a history of tuberculous peritonitis and the mass lesion was somewhat calcified, the possibility that this was an acquired case could not be ruled out. After treatment with oral antibiotics, the size of the atelectasis was decreased. Therefore, we considered that the expansion of the atelectasis could have been due to superimposed bacterial infection.

To study whether abnormal secretion of islet amyloid polypeptide is involved in the development of insulin resistance and impaired insulin secretion in Type 2 (non-insulin-dependent) diabetes mellitus, we measured islet amyloid polypeptide concentrations in 56 first-degree relatives of Type 2 diabetic subjects and in 10 healthy control subjects. Fasting islet amyloid polypeptide concentrations were similar in control subjects, glucose-tolerant and glucose-intolerant relatives (8 +/- 1, 9 +/- 1 and 11 +/- 2 fmol/ml; p = NS). The area under the islet amyloid polypeptide curve measured during an oral glucose load was larger in glucose-intolerant relatives (115 +/- 13 fmol/ml) compared to glucose tolerant relatives and control subjects (88 +/- 3 and 79 +/- 12 fmol/ml; p less than 0.05). The insulin response during the oral glucose load was inversely correlated with the rate of glucose disposal measured during a euglycaemic hyperinsulinaemic clamp (r = -0.725; p less than 0.01), while no significant correlation was observed between the corresponding values for islet amyloid polypeptide and glucose disposal (r = -0.380; p = NS). Hypersecretion of islet amyloid polypeptide is observed in glucose-intolerant first-degree relatives of patients with Type 2 diabetes. Since these patients are characterized by insulin resistance and abnormal first-phase insulin secretion, the putative role of islet amyloid polypeptide in the development of these abnormalities remains to be established. It is however, unlikely that islet amyloid polypeptide is involved in the development of insulin resistance as insulin-resistant relatives with normal glucose-tolerance showed normal islet amyloid polypeptide concentrations.

Ghrelin, a novel GH-releasing acylated peptide, was recently isolated from rat stomach. It stimulated the release of GH from the anterior pituitary through the GH secretagogue receptor (GHS-R). Ghrelin messenger RNA and the peptide are present in rat stomach, but its cellular source has yet to be determined. Using two different antibodies against the N- and C-terminal regions of rat ghrelin, we identified ghrelin-producing cells in the gastrointestinal tracts of rats and humans by light and electron microscopic immunohistochemistry and in situ hybridization combined with immunohistochemistry. Ghrelin-immunoreactive cells, which are not enterochromaffin-like cells, D cells, or enterochromaffin cells, accounted for about 20% of the endocrine cell population in rat and human oxyntic glands. Rat ghrelin was present in round, compact, electron-dense granules compatible with those of X/A-like cells whose hormonal product and physiological functions have not previously been clarified. The localization, population, and ultrastructural features of ghrelin-producing cells (Gr cells) indicate that they are X/A-like cells. Ghrelin also was found in enteric endocrine cells of rats and humans. Using two RIAs for the N- and C-terminal regions of ghrelin, we determined its content in the rat gastrointestinal tract. Rat ghrelin was present from the stomach to the colon, with the highest content being in the gastric fundus. Messenger RNAs of ghrelin and GHS-R also were found in these organs. Ghrelin probably functions not only in the control of GH secretion, but also in the regulation of diverse processes of the digestive system. Our findings provide clues to additional, as yet undefined, physiological functions of this novel gastrointestinal hormone.

A 61-year-old man visited a community hospital because of hemosputum. A solitary nodule in the left lower lung field was pointed out on a chest roentgenogram. The patient was treated with antibiotics, but the solitary nodule increased in size. He was referred to our hospital because of high fever and observations of cavity formation and pleural effusion on a chest roentgenogram. The pleural effusion showed no cytologic evidence of malignancy, and cultures were also negative for bacteria. An increased percentage of lymphocytes was detected in the pleural effusion, but slight eosinophilia was found in blood samples. Dot enzyme-linked immunosorbent assay and Ouchterlony's double-diffusion test yielded a diagnosis of pulmonary dirofilariasis. After drainage of the pleural effusion by thoracentesis, spontaneous regression was observed. Cavity formation, pleural effusion, and spontaneous regression are in general rare in patients with pulmonary dirofilariasis.

Schizencephaly is an extremely rare congenital disease caused by abnormal neuronal migration. The etiology of schizencephaly is not established but vascular disturbance in early childhood could cause this condition. We have cared of a patient with schizencephaly. The patient was 47 year old male. He had focal motor seizure with secondary generalization. Neurological examination revealed, mild left hemiparesis, left pyramidal signs with no sensory impairment, left hemiatrophy, and mirror movement. MRI findings showed schizencephaly, open lip type(type II) in right cerebral hemisphere. His epileptic seizure was controlled by administration of sodium valproate. The possible mechanism of this mirror movement in his left hand and leg could be reorganization of non-affected brain and disinhibition on homolateral pyramidal tract in non-affected left cerebral hemisphere by the transcallosal inhibitory pathyway from affected right cerebral hemisphere. Sodium valpronate can not suppress this mirror movement.

Our previous study showed that treatment of cultured bovine adrenal chromaffin cells with phorbol 12, 13-dibutyrate (PDBu) or 12-O-tetradecanoylphorbol 13-acetate (TPA) caused a rapid (15 min) and persistent (15 h) translocation of both conventional (c) protein kinase C-alpha (PKC-alpha) and novel PKC-epsilon (but not atypical PKC-zeta) from cytosol to membranes, whereas thymeleatoxin (TMX) increased the similar but selective membrane association of only cPKC-alpha. In the present study, chronic (/=12 h) treatment of chromaffin cells with PDBu raised cell surface (125)I-insulin binding without altering the K(D) value; it developed in a concentration (EC(50) = 1.9 nM)-and time (t(1/2) = 14.6 h)-dependent manner, reaching its maximum 115% increase at 48 h. Either TPA (30 nM) or TMX (EC(50) = 6.4 nM) also increased (125)I-insulin binding by 97 or 88%, whereas the biologically inactive 4alpha-TPA had no effect. The increasing effect of PDBu (30 nM for 24 h) on (125)I-insulin binding was significantly blocked, even when H7, an inhibitor of PKC, was added at 8 h after the initiation of PDBu treatment. Concurrent treatment with brefeldin A, an inhibitor of vesicular transport from the trans-Golgi network, cycloheximide, an inhibitor of protein synthesis, or 5,6-dichlorobenzimidazole riboside, an inhibitor of RNA synthesis, abolished the PDBu-induced increment of (125)I-insulin binding. Western blot analysis, using antibody against the beta-subunit of the insulin receptor, showed that treatment with PDBu (30 nM) or TMX (EC(50) = 2.3 nM) increased levels of insulin receptor precursor (approximately 190 kDa; t(1/2) = 7.1 h) and insulin receptor beta-subunit (t(1/2) = 15.4 h), causing their almost maximum 52 and 59% rises, respectively, at 24 h. Northern blot analysis revealed that PDBu or TMX increased levels of insulin receptor mRNAs by approximately 35% as soon as 3 h, producing its monophasic peak approximately 76% increases at 24 h. All of these increasing effects of PDBu and TMX on (125)I-insulin binding and insulin receptor beta-subunit and insulin receptor mRNA levels were entirely prevented by simultaneous treatment with Gö6976, a selective inhibitor of cPKC. These results suggest that long-term activation of cPKC-alpha up-regulates the density of the cell surface insulin receptor via transcriptional/translational events.

To clarify the function of ascorbate free radical (AFR) reductase in the lens antioxidation mechanism, we investigated the difference among species in AFR reductase activity in different vertebrate lenses.Soluble and insoluble fractions were prepared from the lenses of frogs, guinea pigs, rats, rabbits, pigs, and calves. AFR reductase and diaphorase activity of each fraction was determined.AFR reductase activity in the lens soluble fraction was the highest in frogs. That of guinea pigs and rabbits was at the next level; there was only a little activity in rats and pigs, and none was detected in calves. Membrane-bound AFR reductase in the lens insoluble fraction was extracted by 0.3% Triton X-100. The membrane-bound enzyme activity was almost at the same level in frogs, rats, rabbits, and calves, and a little higher in guinea pigs and pigs. However, such species-specificity of AFR reductase activity as in the soluble fraction was not observed in 0.3% Triton X-100 extracts. Diaphorase activity was 3 to 9 times as much as AFR reductase activity in the soluble fractions of frogs, guinea pigs, and rabbits, but in 0.3% Triton X-100 extracts of all vertebrate species used, it was very high, 108 to 311 times the AFR reductase activity.These results suggest that the lens soluble and membrane-bound AFR reductases are individual enzyme molecules and have different antioxidative functions. The lenses of frogs, guinea pigs, and rabbits contain a near-ultraviolet (UV) light absorbing compound, reduced pyridine nucleotide at a high concentration. Therefore, the soluble AFR reductase activity may be high in the vertebrate lenses with a near-UV light filter, and enhance the antiphotoxidation of ascorbic acid.

Viral infection is one of important factors to cause the exacerbation of bronchial asthma. We have investigated 167 adults of asthmatics to clarify the correlation between viral infection and exacerbation of asthma. Patients were classified to four group by the symptoms of common cold and asthma attack. Furthermore, we have examined Picornavirus and Human rhino virus RNA from throat gargles of patients using RT-PCR (reverse transcription--polymerase chain reaction) method. Forty of 65 (61.5%) asthmatics with common cold revealed asthma attack and common cold was significantly associated with acute exacerbation of asthma (p0.01). We identified Picornavirus RNA, which include 113 of Human rhino virus serotypes and enterovirus, from the samples of 16 of 52 (30.8%) patients who had acute exacerbation. It was significantly higher than the detection rate of viral RNA from patient without asthma attack. Furthermore, we analyzed Human rhino virus RNA from the same samples by RT-PCR and 93.7% of Picornavirus were identified as Human rhino virus. Taken together, these findings suggest that common cold is significantly associated with the exacerbation of bronchial asthma. Human rhino virus infection might be one of important virus in this procedure.

A 24-year-old man was admitted to our hospital in June 1996 with complaints of anterior chest discomfort. Chest X-ray films on admission showed an abnormal mediastinal shadow with well-defined margin. Chest X-ray examinations about 6 weeks earlier had not detected any abnormalities. Laboratory tests on admission showed a high serum concentration of Siaryl Lewis X-i antigen (SLX). A computed tomographic scan of the chest showed a large (6 x 6 x 12 cm) homogeneous mass in the right anterior mediastinum. The mass was removed completely and histologically diagnosed as a thymic cyst. Biochemical analysis of fluid from the cyst revealed remarkably high levels of SLX, CA 19-9, and CEA. In immunohistochemical studies, epithelial cells from the cystic walls stained positive for SLX, CA 19-9, and CEA. After the operation, the level of serum SLX returned almost to normal.

The C-C chemokine eotaxin is a potent chemoattractant for eosinophils and probably plays an important role in the pathogenesis of asthma, although the mechanisms of its regulation are not well known. Airway epithelial cells express eotaxin mRNA and protein after stimulation with a variety of cytokines. We focused on the molecular mechanisms of eotaxin gene regulation by TNF-alpha and IL-4 in the airway epithelial cell line, BEAS-2B. Cells were transfected with luciferase reporter plasmids, which contained up to 1363 bp of the eotaxin promoter. Eotaxin promoter activity was increased by TNF-alpha (2.5-fold) and IL-4 (1.5-fold), respectively. The combination of TNF-alpha and IL-4 produced 3.6-fold activation of the eotaxin promoter. The eotaxin promoter contains overlapping consensus binding sites for transcription factors, NF-kappa B and STAT6, which are known to mediate responses to TNF-alpha and IL-4, respectively. Electrophoretic mobility shift assays revealed NF-kappa B binding after TNF-alpha stimulation and STAT6 binding after IL-4 stimulation using a DNA probe derived from the eotaxin promoter. Mutant plasmids were generated to define the roles of these transcription factors in eotaxin promoter activity. TNF-alpha stimulation, but not IL-4 stimulation, was lost in plasmids mutated at the NF-kappa B binding site, whereas IL-4 stimulation, but not TNF-alpha stimulation, was lost in plasmids mutated at the STAT6 binding site. When both sites were mutated, all transcriptional activation was lost. These results imply that TNF-alpha and IL-4 stimulate expression of the eotaxin gene by activating NF-kappa B and STAT6.

A 47-year-old woman was referred to our hospital because of cough and an abnormal shadow in the left lung field. The infiltrate reduced without therapy and another infiltrate appeared in the right lung field. Bronchiolitis obliterans organizing pneumonia was clinically suspected due to the absence of signs of eosinophilia in peripheral blood and bronchoalveolar lavage fluid (BALF). Open lung biopsy specimens disclosed alveolitis with mononuclear cell infiltration and organization within the air spaces of bronchioli and alveolar ducts. The observation of pronounced eosinophil infiltration in the alveolar spaces of some specimens yielded a diagnosis of eosinophilic pneumonia. After steroid therapy, the abnormal shadows disappeared. BALF lymphocyte surface marker analysis detected no decrease in the CD4/CD8 ratio; activated CD4 and CD8 lymphocytes were notably higher than the corresponding levels in peripheral blood. IL-5, IL-3, and GM-CSF values in BALF were not significantly elevated. This was a case of borderline eosinophilic pneumonia that was difficult to diagnose on the basis of clinical parameters alone.

Bronchial asthma is characterized as a chronic inflammation of the airway infiltrated by eosinophils, lymphocytes, and neutrophils. ICAM-1 expression on airway epithelium facilitates adhesion between these inflammatory cells and bronchial epithelial cells, and induces the activation of inflammatory cells. ICAM-1 expression was affected by various cytokines, such as IL-17. IL-17 is a novel cytokine released by CD4+ activated memory T cells. In this study, we examined the effect of IL-17 on ICAM-1 expression by RT-PCR and flow cytometry. Human bronchial epithelial cells, NCI-H 292 cells, were stimulated with IL-17 (100 ng/ml) and/or IFN-gamma (100 U/ml). ICAM-1 was expressed constitutively. IL-17 alone did not enhance ICAM-1 expression on NCI-H 292 cells. However, IL-17 synergistically enhanced ICAM-1 expression induced by IFN-gamma. These results suggest that IL-17 has an effect on ICAM-1 expression of bronchial epithelial cells in airway inflammation.

Uroguanylin, a member of the guanylin peptide family, acts on guanylyl cyclase C (GC-C) to regulate intestinal and renal fluid and electrolyte transport through the second messenger, cGMP. Using an antiserum raised against synthetic rat uroguanylin, we established an RIA and identified three endogenous molecular forms in the intestine and kidney: a 15-amino acid uroguanylin, an 18-amino acid uroguanylin that is a monobasic processing product, and a 9.4-kDa prouroguanylin. Prouroguanylin is the major molecular form in these two tissues, whereas only 15-amino-acid uroguanylin is present in the urine. Rat uroguanylin is most abundant in the proximal small intestine, its content decreasing toward the colon. Uroguanylin is present immunohistochemically in the endocrine cells in the intestine and stomach, B cells in the pancreatic islets, and tubular epithelial cells in the kidney. Uroguanylin has a widespread tissue distribution and is located in cells that function in an endocrine, paracrine, and/or luminocrine (luminal secretion) fashion. Uroguanylin may have physiological functions other than the regulation of fluid and electrolyte transport in the intestine and kidney.

We present a rare case of chronic active Epstein-Barr virus (EBV) infection showing various clinical outcomes. A 26-year-old man was admitted to our hospital due to persistent fever and dyspnea. Serologic response of the patient to EBV indicated chronic active infection. He showed pleuritis, parotitis, chronic hepatic dysfunction, disseminated intravascular coagulation, virus associated hemophaghocytic syndrome, acute rhabdomyolysis, acute renal failure, acute cerebellar ataxia, encephalitis and multiple brain abscesses. None of acyclovir, gancyclovir, prednisolone or interleukin-2 was effectual to abolish those abnormalities. This is the first report of transient cerebellar ataxia which aggravated to panencephalitis associated with chronic EBV infection.

We report the efficacy of oral clarithromycin and inhaled beclomethasone against severe bronchorrhea in a patient with alveolar cell carcinoma. A 54-year-old man produced about 500 to 900 ml of clear and egg-white-like sputum each day. Anti-cancer chemotherapy and erythromycin therapy did not reduce the volume of sputum. After administration of clarithromycin and inhaled beclomethasone, sputum volume decreased to about 300 nl each day and the patient's ability to perform daily activities improved. Two months later, clarithromycin was stopped and the patient was treated with inhaled beclomethasone alone. Sputum volume did not increase for 6 months, although the chestroentgenographic findings gradually worsened. Then the sputum volume gradually increased. Five months after the sputum volume began to increase, he was producing about 2 liters of sputum each day and died of respiratory failure. Although the levels of CA 19-9, SLX, and CEA in serum were all within the normal range, the sputum contained high levels of CA 19-9 (1,133,620 U/ml), SLX (3,000 U/ml), and CEA (283 ng/ml). In patients with bronchorrhea, measurement of tumor markers in sputum may be useful for the diagnosis of alveolar cell carcinoma.

A 60-year-old man, in who idiopathic interstitial pneumonia (IIP) was diagnosed in 1990, was admitted to our hospital in March 1995 with exertional dyspnea, hemosputum and fever. On chest X ray, new infiltrates in the bilateral middle lung fields were seen, in addition to reticulonodular shadows in the bilateral lower lung fields, which had been noted in 1990. Intubation was reformed because of hypoxemia, and bronchoalveolar lavage (BAL) was performed in right B6. The BAL fluid was bloody, and a diagnosis of alveolar hemorrhage was made based on the presence of many hemosiderin-laden macrophages in the fluid. After pulse therapy with methylprednisolone, the hypoxemia and chest X ray findings improved, and he was extubated. Alveolar hemorrhage may be one possible complication leading to an exacerbation of IIP.

We studied bilateral facial nerve conduction velocity distribution (FNCVD) of 8 Bell's palsy patients in their paretic and recovered state and unilateral FNCVD of 13 normal control using collision method. Dual supramaximal stimuli were adapted to marginalis mandibulae branch of facial nerve and compound muscle action potential (CMAP) was recorded from the surface of mentalis muscles. Peak of FNCVD in normal control was present at 22-23 m/sec. In Bell's palsy patients, there was no significant change in FNCVD either at paretic or recovery state. Peak of involved side FNCVD of Bell's palsy patient was almost normal but its amplitude was low. However, in healthy side of Bell's palsy patient, peak of FNCVD was present at 18-19 m/sec. This finding shows that facial nerve diameter distribution in the healthy side of Bell's palsy patient has smaller peak than that of normal control.

The siblings of Sanfilippo syndrome type A (MPS III A) have been reported. The relationship of their parents was the first cousins. Case 1: A 30-year-old Japanese man was hospitalized because of gait disturbance and mental impairment. His early somatic and mental development was normal until 9 years of age when mental deterioration had developed. Speech and gait disturbances and double incontinence occurred at 18 years of age. He could not walk at 21 years of age. Those symptoms were slowly progressive. Case 2: A 32-year-old Japanese man, the elder brother of case 1, had a similar clinical history to that of case 1. Their neurological findings revealed mental impairment, coarse face, positive forced grasp and sucking reflexes, and pyramidal signs. Lumbar X-ray showed platyspondylitis, compression fracture of L 1 and osteoporotic changes. Brain MRI of both cases showed brain atrophy, ventricular dilatation and abnormal high intensity signals near the posterior horn of the lateral ventricles on T2 weighted image. Low perfusion images of fronto-parietal regions were seen in the early phase of SPECT using 123I-IMP. This siblings were diagnosed as Sanfilippo syndrome type A because of heparan sulfaturia and deficiency of heparan sulfate sulfamidase of the lymphocytes. Average life span of Sanfilippo syndrome type A is not so long but the age of our cases is over 30 years of age.

We measured serum soluble adhesion molecules levels in patients with sarcoidosis. The serum levels of soluble ICAM-land L-, E-, and P-selectin were significantly elevated in patients with sarcoidosis compared with healthy volunteers. However, there was no significant difference in serum soluble VCAM-1 levels between the patients and healthy volunteers. A significant correlation was observed between serum soluble L-selectin levels and the number of lymphocytes in the bronchoalveolar lavage fluid of patients with sarcoidosis. Although higher levels of serum soluble adhesion molecules were present in accordance with the clinical stage of sarcoidosis, the differences were not statistically significant. There was a significant correlation between serum ACE and soluble ICAM-1 or VCAM-1 levels. These findings suggest that soluble adhesion molecules may play an important role in the pathogenesis of sarcoidosis.

Lesch-Nyhan syndrome is associated with complete deficiency of hypoxanthine-guanine phosphoribosyltransferase (HPRT), characterized by hyperuricemia and severe neurological signs. The HPRT gene has been mapped to the q26 region on the long arm of the X-chromosome. We are taking care of a family of Lesch-Nyhan syndrome. A 14-year-old male was noted the growth disturbance at the age of 7 months and self-mutilation behavior characterized by compulsive biting of his lip and fingers at the age of 18 months. In 1987, at the age of 4, he was diagnosed as Lesch-Nyhan syndrome from neurologic signs and hyperuricemia (9.8 mg/dl). Neurological examination revealed mild mental and growth retardation, spasticity and hyperreflexia of lower extremities, choreoathetoid movements of extremities, and compulsive self-mutilation. The HPRT activity in erythrocytes of this patient was 0.02 nmol/min/mg hemoglobin (control value 1.76 +/- 0.06), and adenine phosphoribosyltransferase (APRT) activity was 1.08 nmol/min/mg hemoglobin (control value 0.43 +/- 0.06). Using polymerase chain reaction (PCR) method coupled with direct sequencing, we analyzed the nucleotide sequences of each exon from the genomic DNA as well as the entire HPRT coding region of the cDNA by RT-PCR method. In the HPRT gene from the patient, a guanine to adenine substitution at base position 209 in exon 3 was identified, which resulted in a single amino acid substitution of glycine with glutamic acid at codon 70. The family studies indicated that his mother, sister and grandmother were heterozygotes. PCR-restriction fragment length polymorphism (RFLP) utilizing Mnl I site which created by the mutation, was useful for detection of the mutant gene. We have identified a new missense mutation of the HPRT gene in a Japanese patient. This mutation was reported at the same codon as foreign mutants and mighty be indicative of a location of mutation activity in the HPRT gene.

We measured the concentrations of soluble adhesion molecules in serum obtained from healthy volunteers and from patients with idiopathic pulmonary fibrosis (IPF) and the acute respiratory distress syndrome (ARDS). The concentrations of soluble intercellular adhesion molecule 1 (ICAM-1), vascular cell adhesion molecule 1 (VCAM-1), E-selectin, and P-selectin were significantly higher in serum from the patients than in serum from the healthy volunteers. In patients with IPF, the concentration of soluble ICAM-1 in serum was inversely related to the vital capacity (expressed as a percent of the predicted value). The concentrations of L-selectin in serum from patients with ARDS were significantly lower than those in serum from healthy volunteers and from patients with IPF. These data suggest that serum-soluble adhesion molecules may be useful as markers of disease activity, severity, and prognosis in ARDS and IPF.