RESUMO
Planetary rings are observed not only around giant planets1, but also around small bodies such as the Centaur Chariklo2 and the dwarf planet Haumea3. Up to now, all known dense rings were located close enough to their parent bodies, being inside the Roche limit, where tidal forces prevent material with reasonable densities from aggregating into a satellite. Here we report observations of an inhomogeneous ring around the trans-Neptunian body (50000) Quaoar. This trans-Neptunian object has an estimated radius4 of 555 km and possesses a roughly 80-km satellite5 (Weywot) that orbits at 24 Quaoar radii6,7. The detected ring orbits at 7.4 radii from the central body, which is well outside Quaoar's classical Roche limit, thus indicating that this limit does not always determine where ring material can survive. Our local collisional simulations show that elastic collisions, based on laboratory experiments8, can maintain a ring far away from the body. Moreover, Quaoar's ring orbits close to the 1/3 spin-orbit resonance9 with Quaoar, a property shared by Chariklo's2,10,11 and Haumea's3 rings, suggesting that this resonance plays a key role in ring confinement for small bodies.
RESUMO
BACKGROUND: The ten-joint juvenile arthritis disease activity score (JADAS10) is designed to measure the level of disease activity in non-systemic juvenile idiopathic arthritis by providing a single numeric score. The clinical JADAS10 (cJADAS10) is a modification of the JADAS10 that excludes erythrocyte sedimentation rate (ESR). Three different sets of JADAS10/cJADAS10 cut-offs for disease activity states have been published, i.e., the Backström, Consolaro, and Trincianti cut-offs. The objective of this study was to investigate the performance of existing JADAS10 cut-offs in real-life settings using patient data from The Finnish Rheumatology Quality Register (FinRheuma). METHODS: Data were collected from the FinRheuma register. The proportion of patients with an active joint count (AJC) above zero when classified as being in clinically inactive disease (CID) or low disease activity (LDA) groups according to existing JADAS10/cJADAS10 cut-off levels were analyzed. RESULTS: A significantly larger proportion of the patients classified as being in CID had an AJC > 0 when using the JADAS10/cJADAS10 cut-offs by Trincianti et al. compared to those for the other cut-offs. In the LDA group, a significantly larger proportion of the polyarticular patients (35%/29%) had an AJC of two when Trincianti JADAS10/cJADAS10 cut-offs were used compared with when Backström (11%/10%) and Consolaro (7%/3%) JADAS10/cJADAS10 cut-offs were used. CONCLUSIONS: We found the cut-offs proposed by Consolaro et al. to be the most feasible, since these cut-off levels for CID do not result in the misclassification of active disease as remission, and the proportion of patients with AJC > 1 in the LDA group is lowest using these cut-offs.
Assuntos
Antirreumáticos , Artrite Juvenil , Reumatologia , Humanos , Artrite Juvenil/diagnóstico , Artrite Juvenil/tratamento farmacológico , Antirreumáticos/uso terapêutico , Finlândia , Estudos de ViabilidadeRESUMO
We aimed to study intestinal immune activation status in juvenile idiopathic arthritis (JIA) by assessing intestinal human leucocyte antigen (HLA) class II expression and the mRNA expression levels of the pro- and anti-inflammatory mediators and pattern recognition receptors. HLA-D-related (HLA-DR) expression was assessed using immunohistochemical staining of frozen sections in 11 children with JIA and 17 controls. The gene expression levels of the anti- and proinflammatory cytokines, lymphocyte recognition receptors and pattern recognition receptors were studied with reverse transcription-polymerase chain reaction (RT-PCR) in 14 children with JIA and 12 controls. All subjects had various gastrointestinal (GI) symptoms indicating endoscopic examinations, but eventually were not diagnosed with GI disease. In JIA patients, the expression of HLA-DR was increased in the crypt epithelial cells and in the epithelial basement membrane of the ileum when compared with the controls. Positive HLA-DR staining in the ileal mucosa was associated with the presence of high clinical disease activity of JIA and low mRNA expression of anti-inflammatory mediators, such as forkhead box protein P3 (FoxP3), glucocorticoid-induced tumour necrosis factor receptor-related protein (GITR) and transforming growth factor (TGF)-beta. Low ileal expression of interleukin (IL)-10, TGF-ß, FoxP3, Toll-like receptor 2 (TLR-2) and TLR-4 transcripts correlated significantly with a high clinical disease activity in the JIA patients. The increased HLA-DR expression suggests enhanced intestinal antigen presentation in JIA. A correlation between clinical disease activity and low gene expression of tolerogenic mediators in the ileum supports the hypothesis that a link exists between the gut immune system and JIA.
Assuntos
Artrite Juvenil/genética , Artrite Juvenil/imunologia , Regulação da Expressão Gênica , Antígenos HLA-DR/genética , Mucosa Intestinal/imunologia , Mucosa Intestinal/metabolismo , Transdução de Sinais , Adolescente , Artrite Juvenil/patologia , Estudos de Casos e Controles , Criança , Pré-Escolar , Citocinas/genética , Citocinas/imunologia , Feminino , Humanos , Íleo/imunologia , Íleo/metabolismo , Íleo/patologia , Mediadores da Inflamação/imunologia , Mediadores da Inflamação/metabolismo , Mucosa Intestinal/patologia , MasculinoRESUMO
Up-regulation of interleukin (IL)-17 in small intestinal mucosa has been reported in coeliac disease (CD) and in peripheral blood in type 1 diabetes (T1D). We explored mucosal IL-17 immunity in different stages of CD, including transglutaminase antibody (TGA)-positive children with potential CD, children with untreated and gluten-free diet-treated CD and in children with T1D. Immunohistochemistry was used for identification of IL-17 and forkhead box protein 3 (FoxP3)-positive cells and quantitative polymerase chain reaction (qPCR) for IL-17, FoxP3, retinoic acid-related orphan receptor (ROR)c and interferon (IFN)-γ transcripts. IL-1ß, IL-6 and IL-17 were studied in supernatants from biopsy cultures. Expression of the apoptotic markers BAX and bcl-2 was evaluated in IL-17-stimulated CaCo-2 cells. The mucosal expression of IL-17 and FoxP3 transcripts were elevated in individuals with untreated CD when compared with the TGA-negative reference children, children with potential CD or gluten-free diet-treated children with CD (P < 0·005 for all IL-17 comparisons and P < 0·01 for all FoxP3 comparisons). The numbers of IL-17-positive cells were higher in lamina propria in children with CD than in children with T1D (P < 0·05). In biopsy specimens from patients with untreated CD, enhanced spontaneous secretion of IL-1ß, IL-6 and IL-17 was seen. Activation of anti-apoptotic bcl-2 in IL-17-treated CaCo-2 epithelial cells suggests that IL-17 might be involved in mucosal protection. Up-regulation of IL-17 could, however, serve as a biomarker for the development of villous atrophy and active CD.
Assuntos
Doença Celíaca/imunologia , Diabetes Mellitus Tipo 1/imunologia , Duodeno/imunologia , Interleucina-17/biossíntese , Regulação para Cima , Adenocarcinoma/patologia , Apoptose/genética , Atrofia , Autoanticorpos/sangue , Autoanticorpos/imunologia , Doença Celíaca/dietoterapia , Doença Celíaca/metabolismo , Doença Celíaca/patologia , Linhagem Celular Tumoral/metabolismo , Criança , Pré-Escolar , Neoplasias do Colo/patologia , Diabetes Mellitus Tipo 1/metabolismo , Dieta Livre de Glúten , Duodeno/metabolismo , Duodeno/patologia , Feminino , Fatores de Transcrição Forkhead/biossíntese , Fatores de Transcrição Forkhead/genética , Proteínas de Ligação ao GTP , Humanos , Lactente , Interleucina-17/genética , Interleucina-17/fisiologia , Masculino , Microvilosidades/ultraestrutura , Mucosa/imunologia , Mucosa/metabolismo , Mucosa/patologia , Proteína 2 Glutamina gama-Glutamiltransferase , RNA Mensageiro/biossíntese , Linfócitos T Reguladores/imunologia , Transglutaminases/imunologiaRESUMO
It has been suggested that the incidence of herpes zoster may increase due to lack of natural boosting under large-scale vaccination with the varicella vaccine. To study the possibility and magnitude of such negative consequences of mass vaccination, we built a mathematical model of varicella and zoster epidemiology in the Finnish population. The model was based on serological data on varicella infection, case-notification data on zoster, and new knowledge about close contacts relevant to transmission of infection. According to the analysis, a childhood programme against varicella will increase the incidence of zoster by one to more than two thirds in the next 50 years. This will be due to increase in case numbers in the 35 years age groups. However, high vaccine coverage and a two-dose programme will be very effective in stopping varicella transmission in the population.
Assuntos
Vacina contra Varicela/administração & dosagem , Vacina contra Varicela/imunologia , Varicela/prevenção & controle , Herpes Zoster/prevenção & controle , Herpesvirus Humano 3/imunologia , Vacinação/métodos , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Varicela/epidemiologia , Criança , Pré-Escolar , Simulação por Computador , Coleta de Dados/métodos , Feminino , Herpes Zoster/epidemiologia , Humanos , Lactente , Recém-Nascido , Masculino , Pessoa de Meia-Idade , Modelos Teóricos , Adulto JovemRESUMO
Supported by an economic evaluation, rotavirus vaccine is introduced into the national immunisation schedule in Finland. The vaccination programme has been estimated to be reasonably cost-effective. Given at the age of two, three and five months, the vaccine is expected to prevent annually in Finland among children under the age of five years approximately 2,000 rotavirus diarrhoea episodes needing hospitalisation, and over 10,000 outpatient visits. The impact of the programme will be evaluated in 2011 by repeating the economic analysis and carefully monitoring adverse events.
Assuntos
Programas de Imunização , Infecções por Rotavirus/prevenção & controle , Vacinas contra Rotavirus/uso terapêutico , Análise Custo-Benefício , Efeitos Colaterais e Reações Adversas Relacionados a Medicamentos , Finlândia , Humanos , Programas de Imunização/economia , Avaliação de Programas e Projetos de SaúdeRESUMO
UNLABELLED: Dental evidence, especially from radiographs, has been found to be an effective method in personal identification. Previously, it has been shown that wireless personal digital assistants (PDA) can be used to transfer digitized radiographs. The purpose of this study was to set up a secure and reliable mobile connection for transferring dental digital images for disaster victim identification, and to test this new way of working in Phuket, Thailand, following the December 2004 Asian Tsunami disaster. MATERIAL AND METHODS: Digital dental radiographs and clinical images were transferred in two separate sets using secured data transmission from a server in Finland to PDA terminals in Thailand. The mean size of the images in test 1 and test 2 were 90.7 kB and 88.1 kB, respectively. RESULTS: The mean speed of the transmission was 3.7 kB/s with the Nokia 9500 and 3.4 kB/ s with the Qtek 2020i. The quality of all the pictures was found to be good enough for dental identification purposes. CONCLUSIONS: Wireless personal digital assistants (PDA) together with data secure transmission of digital clinical information could be used in order to assist in disaster victim identification in areas where GSM cellular networks are available.
Assuntos
Computadores de Mão , Desastres , Odontologia Legal/métodos , Radiografia Dentária Digital , Humanos , TailândiaRESUMO
The purpose of this study was to study neurocognitive performance as a predictor of outcomes in midlife schizophrenia. There is a lack of studies with unselected samples and a long follow-up. The study is based on the prospective, unselected population-based Northern Finland Birth Cohort 1966. The study includes 43 individuals with schizophrenia and 73 controls, whose neurocognitive performance was assessed twice, at 34 and 43 years. At both time points we used identical neurocognitive tests to assess verbal and visual memory and executive functions. Our main aim was to analyse neurocognitive performance at 34 years as a predictor of clinical, vocational and global outcomes at 43 years. Additionally, the analysis addressed cross-sectional associations between cognitive performance and clinical, vocational and global measures at 43 years. The assessment of outcomes was performed in the schizophrenia group only. In the longitudinal analysis poorer visual memory predicted poorer vocational outcome and poorer long-term verbal memory predicted poorer global outcome. In the cross-sectional analysis poorer visual memory and lower composite score of neurocognition were associated with poorer global outcome. No individual neurocognitive test or the composite score of these predicted remission. These data indicate that neurocognition, especially memory function, is an important determinant of long-term functional outcome in midlife schizophrenia.
RESUMO
Six healthy male subjects were given in a crossover fashion medium molecular weight (HES 125) and low molecular weight (HES 40) hydroxyethyl starch, dextran, and balanced salt solution by intravenous infusion. The plasma volumes were determined using labeled albumin and plasma protein measurements. Three properties of factor VIII protein complex and indices of blood coagulation and hemostasis were measured before and after the infusions. Both the salt solution and HES 40 increased plasma volume, but their effect wore off within 3 hours. Dextran and HES 125 increased plasma volume significantly (P less than 0.001) more than the salt solution did, and the expansion was maintained for 24 hours. Plasma volume increases (dextran and HES 125) were associated with high nonglucose carbohydrate levels in plasma and low levels in urine. No or slight increases in plasma volumes (HES 40), on the other hand, were associated with low and high carbohydrate levels in plasma and urine, respectively. Serum alpha-amylase activity increased significantly after both HES preparations as compared to salt solution. Dextran and HES 125 decreased all the three values of factor VIII, these decreases being maximal 3 to 6 hours after administration and highest (about 25 per cent) for F VIII R:Ag and F VIII R:cof. It is concluded that HES 125 and dextran are equally effective plasma expanders.
Assuntos
Coagulação Sanguínea/efeitos dos fármacos , Dextranos/farmacologia , Hemostasia/efeitos dos fármacos , Derivados de Hidroxietil Amido/farmacologia , Volume Plasmático/efeitos dos fármacos , Amido/análogos & derivados , Adulto , Metabolismo dos Carboidratos , Fator VIII/metabolismo , Humanos , Masculino , Peso Molecular , Distribuição Aleatória , Fatores de TempoRESUMO
The effects of a single dose of ultraviolet B (UVB) radiation (0.4 J/cm2) on immunological functions by blood leukocytes and on hematological parameters was studied in roach (Rutilus rutilus), a teleostean fish. The respiratory burst of phorbol 12-myristate 13-acetate stimulated whole blood phagocytes increased significantly after UVB irradiation but spontaneous cytotoxicity of blood leukocytes toward 51chromium-labeled K562 target cells was not markedly altered. Differential cell counting revealed that UVB exposure significantly increased the proportion of granulocytes and significantly decreased the proportion of lymphocytes in the peripheral blood, whereas hematocrit and the total number of white and red blood cells were unchanged. Plasma cortisol concentration increased in UVB-exposed fish. Severe handling stress caused similar, although not as potent, effects on the measured parameters of fish blood as UVB irradiation. These observations suggest that in fish UVB brings about a stress response, which may account for the observed alterations in the immune parameters and leukocyte composition of blood. Exposure of fish to strong visible light induced no alterations in immunological or hematological parameters, making it unlikely that ultraviolet radiation mediates its effects through visual perception.
Assuntos
Sistema Imunitário/efeitos da radiação , Animais , Cyprinidae/sangue , Cyprinidae/imunologia , Citotoxicidade Imunológica/efeitos dos fármacos , Citotoxicidade Imunológica/efeitos da radiação , Humanos , Sistema Imunitário/efeitos dos fármacos , Células K562 , Explosão Respiratória/efeitos dos fármacos , Explosão Respiratória/efeitos da radiação , Acetato de Tetradecanoilforbol/farmacologia , Raios UltravioletaRESUMO
The effects of short-term exposure to ultraviolet B (UVB) radiation on lymphocyte-related parameters were studied under controlled laboratory conditions using roach (Rutilus rutilus), a cyprinid teleost, as the model fish. In vitro lymphoproliferative responses stimulated with a T-cell-specific mitogen, concanavalin A (ConA), or a B-cell-specific activator, lipopolysaccharide (LPS), were decreased in exposed fish. Also nonstimulated proliferation was lower than in unexposed fish. ConA-activated responses returned to normal levels within 7 days after exposure, but LPS-activated responses were reduced throughout the 14 day follow-up. The capability of UVB-exposed fish to produce an antibody response was studied by intraperitoneal immunization with bovine gamma-globulin (BGG). The concentration of anti-BGG antibodies in plasma as well as the number of anti-BGG-specific antibody-secreting cells in the spleen or blood were not decreased in fish exposed either to a single dose of UVB prior to immunization, or to single dose of UVB prior to immunization followed by three additional doses after immunization. Immunoglobulin M (IgM) production, when assayed as plasma IgM level or as the number of IgM-secreting cells in the spleen or blood, was not suppressed after exposure to UVB irradiation. These results indicate that a single dose of UVB or short-term exposure to UVB irradiation has no negative effects on IgM production or reactivity against antigen administered via the intraperitoneal route. However, the suppression of in vitro lymphoproliferative responses suggest that exposure to UVB has the potential to interfere with lymphocyte-related functions in fish.
Assuntos
Cyprinidae/imunologia , Linfócitos/efeitos da radiação , Animais , Imunoglobulina M/biossíntese , Imunoglobulina M/sangue , Ativação Linfocitária/efeitos dos fármacos , Ativação Linfocitária/efeitos da radiação , Linfócitos/efeitos dos fármacos , Linfócitos/imunologia , Mitógenos/farmacologia , Raios UltravioletaRESUMO
Roach (Rutilus rutilus) were irradiated with a single dose of ultraviolet B (UVB) radiation (0.4 J/cm2) in order to study the effects of UVB on the nonspecific immune defense mechanisms of fish. Neutrophils and macrophages were isolated from the head kidney of fish on days 1-14 postirradiation. Both random and directed migration of neutrophils, studied by migration under agarose assay, were suppressed on day 1 after UVB irradiation. The respiratory burst of phorbol 12-myristate 13-acetate-stimulated neutrophils and macrophages was also suppressed at days 1 and 2 after UVB irradiation. The suppression of migration and respiratory burst were restored or the responses were even enhanced later, but on the other hand spontaneous cytotoxicity of neutrophils toward 51chromium-labeled K562 target cells stayed suppressed throughout the 14 day follow-up. This study indicates that UVB radiation has the potential to suppress the functioning of phagocytes and to compromise the immune system of fish.
Assuntos
Quimiotaxia de Leucócito/efeitos da radiação , Peixes/imunologia , Neutrófilos/efeitos da radiação , Fagócitos/efeitos da radiação , Raios Ultravioleta , Animais , Carpas/imunologia , Tecido Linfoide/efeitos da radiação , Macrófagos/imunologia , Macrófagos/efeitos da radiação , Neutrófilos/fisiologia , Fagócitos/efeitos dos fármacos , Fagócitos/imunologia , Explosão Respiratória/efeitos dos fármacos , Explosão Respiratória/efeitos da radiação , Acetato de Tetradecanoilforbol/farmacologiaRESUMO
Ultraviolet B radiation penetrates into water and can affect fish health and the immune system, as is the case with mammals. Teleost fish, the roach, were exposed to UVB irradiation in aquariums and a panel of immune parameters was determined. In addition to altered blood picture and respiratory burst by blood leukocytes, changes were noted also in major lymphatic organs. Respiratory burst and natural cytotoxicity activity of head kidney granulocytes and mitogen-activated proliferation of splenic lymphocytes were suppressed. Although mostly transitory, some parameters remained suppressed for the following 2 weeks. Ultraviolet A radiation had only minor effects. The stress induced by UVB may be involved in the modulation of immune parameters.
Assuntos
Peixes , Sistema Imunitário/efeitos da radiação , Raios Ultravioleta/efeitos adversos , Animais , Peixes/imunologia , Granulócitos/efeitos da radiação , Cabeça/efeitos da radiação , Humanos , Rim/efeitos da radiação , Linfócitos/efeitos da radiação , Explosão Respiratória/efeitos da radiaçãoRESUMO
Tobacco smoke is highly genotoxic and produces chromosomal damage in several experimental systems. Active smokers have been shown to have an increased prevalence of somatic chromosome damage in their peripheral blood lymphocytes: this is seen in most cases as an increased sister-chromatid exchange (SCE) frequency and often also as increased structural chromosome aberrations (CAs). Among passive smokers, in association with exposure to environmental tobacco smoke, no such induction of chromosomal damage has been documented. In the present paper we report negative results on induction of chromosomal damage in 2 separate groups of intensive involuntary exposure to tobacco smoke, non-smoking restaurant personnel and newborn children of smoking mothers. While significant exposure in these groups is clearly seen in biochemical intake markers, e.g. cotinine and thiocyanate values in plasma, the conventional cytogenetic parameters, structural chromosome aberrations and sister-chromatid exchanges, are unable to detect the low exposures of involuntary smokers.
Assuntos
Aberrações Cromossômicas , Poluição por Fumaça de Tabaco/efeitos adversos , Células Cultivadas , Sangue Fetal/citologia , Humanos , Recém-Nascido , Ocupações , Troca de Cromátide Irmã , Fumar/genéticaRESUMO
Aquatic organisms can be harmed by the current levels of solar ultraviolet radiation. We have recently shown that exposure of fish to UVB irradiation alters the functioning of the fish immune system, but the effects of UVA radiation are unknown. The present study continues this work by characterizing UVA irradiation-induced immunological changes in fish. Roach, a cyprinid fish, were exposed to a single dose of either UVA (3.6 J/cm2) or UVB (0.5 J/cm2) irradiation. Both irradiations suppressed transiently mitogen-stimulated proliferation of blood lymphocytes. UVA, but not UVB, decreased hematocrit, plasma protein, and plasma immunoglobulin levels and increased the proportions of blood cells classified as unidentified leukocytes, possibly consisting of UVA-damaged lymphocytes. UVB, but not UVA, altered the functioning of head kidney and blood phagocytes, induced granulocytosis and lymphocytopenia in the blood and increased plasma cortisol concentration. These results imply that both UVA and UVB are potent modulators of the immune defence of fish.
Assuntos
Cyprinidae/imunologia , Ativação Linfocitária/efeitos da radiação , Linfócitos/efeitos da radiação , Raios Ultravioleta , Animais , Proteínas Sanguíneas/efeitos da radiação , Cyprinidae/sangue , Contagem de Eritrócitos , Hematócrito , Imunoglobulinas/sangue , Imunoglobulinas/efeitos da radiação , Contagem de Leucócitos , Linfócitos/citologia , Linfócitos/imunologiaRESUMO
The present study was designed to examine, whether, effluents from a modern pulp and paper mill using elemental chlorine-free/total chlorine-free (ECF/TCF) bleaching, exert effects on the immune system of fish and, in addition, to relate these findings to physiological parameters known to be affected by bleached kraft-mill effluents (BKME). Roach (Rutilus rutilus) were exposed in laboratory conditions to primary- or secondary-treated effluent from a pulp and paper mill. In order to study their capability to respond to foreign antigens they were immunised with bovine gamma-globulin (BGG) prior to exposure. The number of anti-BGG antibody-secreting cells (ASC) and the number of immunoglobulin-secreting cells (ISC) in the spleen and blood as well as the level of anti-BGG specific antibodies and concentration of plasma immunoglobulin (IgM) were studied. Phagocytosis and migration of granulocytes of the head kidney were also determined. In addition to the immunological parameters, the activity of hepatic biotransformation enzymes, the carbohydrate metabolism and osmoregulation were examined. Exposure of roach for 21 days to BKME affected several immunological parameters. Both effluents, primary- and secondary-treated, impaired the immunoreactivity of the fish. Sex-related differences in the immune responses were evident in many parameters e.g. in the number of blood ISC and splenic ASC. Sex also had effects on cortisol levels and in the induction of 7-ethoxyresorufin O-deethylase (EROD). These results demonstrate that both primary- and secondary-treated effluent from a pulp and paper mill using ECF/TCF bleaching have effects on fish immune functions. Further, these findings suggest that steroids may contribute to immunomodulation in fish.
Assuntos
Peixes/imunologia , Sistema Imunitário/efeitos dos fármacos , Resíduos Industriais/efeitos adversos , Papel , Poluentes Químicos da Água/toxicidade , Adenosina Trifosfatases/metabolismo , Animais , Metabolismo dos Carboidratos , Movimento Celular/efeitos dos fármacos , Cloro/toxicidade , Citocromo P-450 CYP1A1/biossíntese , Ensaio de Imunoadsorção Enzimática , Água Doce/análise , Brânquias/efeitos dos fármacos , Brânquias/enzimologia , Hidrocortisona/sangue , Imunoglobulina M/biossíntese , Resíduos Industriais/análise , Glicogênio Hepático/metabolismo , Linfócitos/efeitos dos fármacos , Linfócitos/imunologia , Neutrófilos/efeitos dos fármacos , Neutrófilos/imunologia , Explosão Respiratória/efeitos dos fármacos , Poluentes Químicos da Água/análise , Equilíbrio Hidroeletrolítico/efeitos dos fármacosRESUMO
The characteristic, bloodhound-like appearance, which degenerates gradually, of patients with primary hereditary systemic amyloidosis, also called Meretoja's syndrome (MS), is attributable to amyloid degeneration of the craniofacial skin and peripheral facial nerves, but apparently also to amyloid deposits in the muscles; a finding not previously described. A material of five patients treated with plastic surgery is presented, and the peculiarities and differences of this rare disease in comparison with other peripheral neuropathies is discussed from a reconstructive viewpoint.