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1.
J Biol Regul Homeost Agents ; 34(4 Suppl. 2): 47-53. SPECIAL ISSUE: FOCUS ON PEDIATRIC CARDIOLOGY, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-33000600

RESUMO

Kawasaki disease (KD) is an acute systemic vasculitis of unknown etiology. It has a self-limiting course and so far, represents the most common cause of coronary heart disease acquired in children aged between 6 months and 5 years. The inflammatory process can involve the coronary arteries with the formation of aneurysms and thrombotic occlusions with the risk of sudden death, especially in infants. Myocardial inflammation and abnormalities of cardiac contractility can occur acutely or many years after the disease onset. Therapy must be started within 10 days after the onset of symptoms to reduce the risk of heart complications. Immunoglobulin and aspirin treatment are effective in reducing heart complications. Recent studies have shown new therapeutic strategies (corticosteroids, immunosuppressive and biological drugs) in case of ineffectiveness of treatment with immunoglobulins.


Assuntos
Cardiopatias , Síndrome de Linfonodos Mucocutâneos , Pré-Escolar , Vasos Coronários , Cardiopatias/etiologia , Humanos , Lactente , Síndrome de Linfonodos Mucocutâneos/complicações , Síndrome de Linfonodos Mucocutâneos/diagnóstico , Síndrome de Linfonodos Mucocutâneos/tratamento farmacológico
2.
Magn Reson Chem ; 58(9): 902-912, 2020 09.
Artigo em Inglês | MEDLINE | ID: mdl-32526806

RESUMO

Cellulose-based artefacts are highly prone to degradation, especially in the presence of acidic compounds, which trigger the depolymerization of cellulose chains and lead to a loss in the original mechanical resistance of the material. Calcium hydroxide nanoparticles dispersed in organic solvent have been recently proposed for the deacidification of cellulose-based artworks. In this work, changes induced on paper by a deacidification treatment, following an acidification bath, were studied by nuclear magnetic resonance (NMR) relaxometry and by the so-called NMR diffraction of water trapped in the cellulose network. The deacidification treatment modifies intrachain and interchain bonds in hydrolyzed and degraded cellulose, leading to a buffered cellulose network configuration, which is similar to that characterizing the untreated reference sample in terms of relaxation parameters. Overall, calcium hydroxide nanoparticles are demonstrated effective in hindering the degradation of cellulose induced by acids and ageing in strong environmental conditions, even from the standpoint of cellulose network arrangement. It is worth noting, too, that the unilateral NMR device used for the relaxation measurements may represent a powerful tool for the preservation of cellulose-based artworks because it allows for the monitoring of the conservation status of cellulose in a completely non-invasive manner.

3.
J Nematol ; 522020.
Artigo em Inglês | MEDLINE | ID: mdl-33829207

RESUMO

During a field survey for entomopathogenic nematodes in Georgia, in the territory of Borjomi-Kharagauli, a nematode population was isolated from the body of single dead beetle of Lucanus ibericus Motschulsky 1845 (Coleoptera: Lucanidae). Based on morphological characters and molecular analyses, the nematode species is identical to Pelodera strongyloides (Schneider, 1860, 1866), being the first record of this species in Georgia. Morphometrics of the Georgian population agree with the original description, and females differ from males by very few characters. The Georgian population of P. strongyloides was molecularly characterized by sequencing the D2 to D3 expansion domains of the 28S rRNA gene and two new molecular markers as the ITS and the mitochondrial COI gene. Phylogenetic analyses revealed that all P. strongyloides sequences grouped together along with all other Pelodera species.

4.
Clin Exp Immunol ; 195(1): 74-85, 2019 01.
Artigo em Inglês | MEDLINE | ID: mdl-29920643

RESUMO

The microbiome is represented by microorganisms which live in a symbiotic way with the mammalian. Microorganisms have the ability to influence different physiological aspects such as the immune system, metabolism and behaviour. In recent years, several studies have highlighted the role of the microbiome in the pathogenesis of autoimmune diseases. Notably, in systemic lupus erythematosus an alteration of the intestinal flora (lower Firmicutes/Bacteroidetes ratio) has been described. Conversely, changes to the gut commensal and periodontal disease have been proposed as important factors in the pathogenesis of rheumatoid arthritis. At the same time, other autoimmune diseases (i.e. systemic sclerosis, Sjögren's syndrome and anti-phospholipid syndrome) also share modifications of the microbiome in the intestinal tract and oral flora. Herein, we describe the role of the microbiome in the maintenance homeostasis of the immune system and then the alterations of the microorganisms that occur in systemic autoimmune diseases. Finally, we will consider the use of probiotics and faecal transplantation as novel therapeutic targets.


Assuntos
Doenças Autoimunes/microbiologia , Disbiose/imunologia , Microbioma Gastrointestinal/imunologia , Microbiota/imunologia , Probióticos/uso terapêutico , Animais , Doenças Autoimunes/imunologia , Transplante de Microbiota Fecal , Homeostase , Humanos , Imunidade Inata
5.
J Helminthol ; 90(2): 152-65, 2016 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-25647151

RESUMO

Morphological and molecular analyses of a stem nematode causing a severe disease on infected sowthistle (Sonchus bulbosus) plants, involving the formation of gall-like structures on infected leaves and stems, have led to the description of a new species named Ditylenchus oncogenus n. sp. Morphologically, the new species is characterized by a medium to large body size (all adults more than 1 mm in length); a delicate stylet (9.0-11.0 µm long) with minute, rounded knobs; a long post-vulval uterine sac (c. 65% of the vulva-anus distance); six incisures at the lateral fields and characteristic D. destructor-pattern of spicules (with pronounced ventral tumulus and anteriorly pointed, less sclerotized, cuticle parts present within the lamina). The results of molecular analysis of rRNA gene sequences, including the D2-D3 expansion regions of 28S rRNA, internal transcribed spacer (ITS) rRNA, partial 18S rRNA gene, the protein-coding mitochondrial gene, cytochrome oxidase c subunit I (COI), and the heat-shock protein 90 (hsp90) gene, support the new species status. The results of a host-suitability test indicated that the new species does not parasitize potato (Solanum tuberosum) tubers and broad bean (Vicia faba) seedlings. Histopathological observations on naturally infected sowthistle tissues revealed that D. oncogenus n. sp. causes floral stem neoplasia and midrib leaf gall formation on the type, and to date only known, host. The galls were characterized by extensive hyperplasia, where several necrotic cells in the neoplasic area were directly damaged by feeding of the nematode, whereas a number of adjacent cells showed typical cytological changes, such as granulated cytoplasm with hypertrophied nuclei and nucleoli.


Assuntos
Doenças das Plantas/parasitologia , Sonchus/parasitologia , Tylenchida/classificação , Tylenchida/genética , Animais , Feminino , Itália , Masculino , Filogenia , Tylenchida/ultraestrutura
6.
J Endocrinol Invest ; 37(2): 195-202, 2014 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-24497219

RESUMO

BACKGROUND: Since the original description, there have been only few epidemiological studies of Wolfram syndrome (WS). AIM: Aims of the present paper are to ascertain WS prevalence and expression in a district of North-eastern Sicily, i.e. a geographic area where consanguineous unions are not very unusual. MATERIALS AND METHODS: Prevalence rates of WS in the Messina district were calculated by taking into consideration both the total population (653,737) and the populations included within the 0-30 year age range (202,681). We estimated the relative prevalence of WS among patients with youth-onset insulin-dependent diabetes mellitus (DM) who are currently aged under 30 years (256). RESULTS: Global WS prevalence in our district is 1:54,478, whereas prevalence among individuals under 30 is 1:16,890 and relative prevalence among patients with juvenile-onset insulin-dependent DM is 1:22.3. When compared with the patients with insulin-dependent DM of Messina district, WS patients did not exhibit significant differences in terms of biochemical features at DM onset, whereas age at DM diagnosis was significantly earlier in WS group. CONCLUSIONS: (a) WS prevalence is not so infrequent as generally expected; (b) in our series, DM presented before 10 years in 11/12 patients and ten cases have already developed all the four peculiar manifestations of WS by 26 years; (c) 9/12 patients exhibited a homozygous frameshift/truncation mutation (Y454_L459del_fsX454), which is the one most frequently found also in patients from other Italian regions; (d) age at DM diagnosis was significantly earlier in WS group than in the patients with insulin-dependent DM of Messina district.


Assuntos
Síndrome de Wolfram/epidemiologia , Síndrome de Wolfram/genética , Adolescente , Adulto , Criança , Estudos de Coortes , Consanguinidade , Diabetes Insípido/epidemiologia , Diabetes Insípido/genética , Diabetes Mellitus Tipo 1/epidemiologia , Diabetes Mellitus Tipo 1/genética , Feminino , Genótipo , Humanos , Masculino , Linhagem , Fenótipo , Prevalência , Sicília/epidemiologia , Síndrome de Wolfram/complicações , Adulto Jovem
7.
J Endocrinol Invest ; 37(3): 303-8, 2014 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-24474680

RESUMO

BACKGROUND: Due to the lack of specific pediatric studies, no data are available about natural history of endogenous subclinical hyperthyroidism (SH) in childhood. AIMS: (a) To investigate for the first time the natural history of SH [suppressed thyrotropin (TSH) and normal free thyroxine free thyroxine (FT4) levels] when presenting as initial manifestation of Hashimoto's thyroiditis (HT) in childhood (group A); (b) to compare spontaneous evolution of HT-related SH with that observed in age-matched patients with HT-related frank hyperthyroidism (suppressed TSH and elevated FT4 levels), i.e., Hashitoxicosis Htx (group B). RESULTS: In the 11 patients of group A, TSH normalization spontaneously occurred 1-24 months after diagnosis, while in the 10 patients of group B it occurred 3-9 months after diagnosis, with no differences between the 2 groups in terms of time interval from entry to TSH normalization. In group A, this time interval was related to baseline thyroid peroxidase antibodies (r=0.78, p = 0.04). During follow-up, eight patients of each group remained euthyroid, whereas two became hypothyroid (in both groups) and one developed Graves' disease (in group A). CONCLUSION: (a) HT should be included among the causes of endogenous SH in pediatric age; (b) in children with HT-related SH, spontaneous normalization of TSH levels occurs within the first 24 months after diagnosis, as well as in age-matched patients with Htx; (c) in both these conditions, a further deterioration of thyroid function might re-present in some patients during follow-up; (d) Ht-related SH and Htx might be possibly seen as different biochemical stages along the same continuum.


Assuntos
Doença de Hashimoto/complicações , Hipertireoidismo/diagnóstico , Adolescente , Autoanticorpos/sangue , Criança , Estudos Transversais , Feminino , Seguimentos , Humanos , Hipertireoidismo/etiologia , Masculino , Prognóstico , Estudos Prospectivos , Hormônios Tireóideos/sangue
8.
Pediatr Med Chir ; 36(5-6): 104, 2014 Dec 30.
Artigo em Inglês | MEDLINE | ID: mdl-25669895

RESUMO

BACKGROUND: Numerous studies have shown that GH, in addition to promoting linear growth, exerts a key role in many metabolic processes. However, there are only few studies aiming at evaluating the metabolic panel of children with GH deficiency (GHD). The aims of the study were: to verify the presence of metabolic alterations in GHD children in comparison with age-matched controls and to check the possible effects of two year GH therapy on the metabolic parameters in GHD. STUDY DESIGN: cross-sectional and prospective; one center experience POPULATION AND METHODS: We enrolled 32 pediatric GHD patients (group A) and 33 sex- and age-matched healthy controls (group B). Baseline serum assays (lipid, insulin, glucose) were performed in both groups. GHD children underwent replacement therapy with GH. The same assays were repeated after 12 and 24 months of GH treatment. RESULTS: No differences, in basal assays were found between the two groups. In group A, after initiation of GH, there was a significant increase of basal insulin and HOMA- insulin resistance (IR) index (p <0.001). In children with severe GHD (peak GH <3 ng / ml), after beginning of GH therapy a significant improvement in the lipid profile was found (p < 0.05). CONCLUSIONS: a) At the time of diagnosis GHD children had a metabolic picture that was not different from non- GHD group; b) in children with severe GHD, the metabolic profile showed a trend towards at improvement after the initiation of replacement therapy with GH, with beneficial effects in terms of total cholesterol, LDL cholesterol and cardiovascular risk indices; c) GHD patients with unfavorable metabolic profile (high BMI and hypercholestorolemia) need a monitoring of glucose metabolism by periodical evaluations of insulin and HOMA - IR.


Assuntos
Transtornos do Crescimento/tratamento farmacológico , Terapia de Reposição Hormonal/métodos , Hormônio do Crescimento Humano/uso terapêutico , Adolescente , Doenças Cardiovasculares/etiologia , Estudos de Casos e Controles , Criança , Pré-Escolar , Colesterol/sangue , LDL-Colesterol/sangue , Estudos Transversais , Feminino , Hormônio do Crescimento Humano/deficiência , Hormônio do Crescimento Humano/farmacologia , Humanos , Masculino , Estudos Prospectivos , Fatores de Risco , Fatores de Tempo , Resultado do Tratamento
9.
J Biol Regul Homeost Agents ; 27(1): 259-66, 2013.
Artigo em Inglês | MEDLINE | ID: mdl-23489706

RESUMO

Obesity is a state of chronic inflammation. Data on IGF system are often discrepant, and their relationships with mediators of inflammation are unknown. Furthermore, changes in thyroid function have been reported. We aimed at investigating the changes in these systems, and verify any relationships among cytokines, IGF system, thyroid function and insulin-insensitivity. Fifty obese pre-pubertal children, and 55 normal-weight subjects comparable for age and sex were enrolled. Serum IGF-I, IGF-II, IGFBP-1, IGFBP-2, IGFBP-3, IL-6 and TNF-alpha were assayed. In obese children insulin, TSH and FT4 were measured also, and the HOMA-IR index was calculated. Increased IGF-II, IL-6 and TNF-alpha, and decreased IGFBP-1 and IGFBP-2 concentrations were found in obese compared to normal-weight children. The IGF-I/IGFBP-3 molar ratio was also reduced in the obese subjects. In the obese children with high HOMA-IR index, IGFBP-1 and -2 serum concentrations were significantly decreased compared with those with normal insulin sensitivity, and in the obese subjects with increased TSH, IGFBP-2 concentrations were lower, and IGFBP-3 levels were higher compared to their counterparts with normal TSH levels. Among the significant correlations, BMISDS was correlated with IGF-II, and TSH. IGF-II concentrations showed a positive relationship with IL-6. TSH was correlated with IGFBP-2 also. The data showed interactions among IL-6, IGF system, insulin sensitivity, and thyroid function with changes being related to the degree of obesity. Chronic inflammation in obese children was confirmed. Some of the changes in the IGF system could be a consequence of insulin resistance and could account also for later complications in obese subjects.


Assuntos
Citocinas/sangue , Mediadores da Inflamação/sangue , Obesidade/sangue , Obesidade/fisiopatologia , Puberdade/sangue , Somatomedinas/metabolismo , Glândula Tireoide/fisiopatologia , Índice de Massa Corporal , Peso Corporal , Criança , Feminino , Humanos , Resistência à Insulina , Proteína 1 de Ligação a Fator de Crescimento Semelhante à Insulina/sangue , Proteína 2 de Ligação a Fator de Crescimento Semelhante à Insulina/sangue , Proteína 3 de Ligação a Fator de Crescimento Semelhante à Insulina/sangue , Fator de Crescimento Insulin-Like I/metabolismo , Fator de Crescimento Insulin-Like II/metabolismo , Interleucina-6/sangue , Masculino
12.
J Endocrinol Invest ; 36(1): 12-5, 2013 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-22189488

RESUMO

BACKGROUND: Increased artery intima-media thickness (IMT) was found in adults with classical congenital adrenal hyperplasia (CAH). No data are available in patients with non-classical (NC) CAH. AIMS: To evaluate IMT in adolescents with classical and NC CAH and to compare the results with those recorded in a control population. PATIENTS AND METHODS: Eighteen adolescents with either classical (Subgroup A1) or NC CAH (Subgroup A2) were compared with 16 controls (Group B). All subjects underwent IMT ultrasonography measurement at different sites; results were correlated with clinical, metabolic, and insulin resistance (IR) data. RESULTS: When compared with Group B, both subgroups exhibited higher IMT values at all sites. No differences were found between classical and NC CAH. Univariate analysis of factors impacting on IMT of CAH patients demonstrated that: a) abdominal aorta (AA) IMT was positively correlated with cumulative glucocorticoid doses, triglyceride serum levels, and diastolic blood pressure SD score and negatively with androstenendione and ACTH levels; b) common carotid (CC) IMT was positively associated with triglycerides and triglyceride/HDL ratio. At multiple regression analysis, the independent positive predictors of AA and CC IMT were respectively triglyceride levels and triglyceride/HDL ratio. CONCLUSIONS: a) Even adolescents with NC CAH and not only those with classical form may be at higher risk of artery alterations; b) this risk is not necessarily associated with either obesity or waist/height ratio or dyslipidemia; c) an important role in the pathogenesis of artery alterations in CAH may be played by intermittent iatrogenic hypercortisolism and secondary IR.


Assuntos
Hiperplasia Suprarrenal Congênita/complicações , Espessura Intima-Media Carotídea , Adolescente , Hiperplasia Suprarrenal Congênita/sangue , Hiperplasia Suprarrenal Congênita/patologia , Adulto , Estudos de Casos e Controles , Feminino , Humanos , Masculino , Prognóstico , Fatores de Risco , Índice de Gravidade de Doença , Adulto Jovem
13.
Pediatr Med Chir ; 35(4): 183-6, 2013.
Artigo em Italiano | MEDLINE | ID: mdl-24245101

RESUMO

UNLABELLED: Klinefelter syndrome (KS) is the most frequent chromosomal aneuploidy with a prevalence of 1:500 men but it often remains a largely undiagnosed condition and only 10% of cases are identified in childhood and adolescence. We report the anamnestic, clinical and auxological findings of 14 KS patients diagnosed in paediatric age. 3/14 patients (21%) with KS were diagnosed in prenatal age by amniocentesis, 1 patient was diagnosed at birth due to genital ambiguity and the remaining 10/14 (71.4%) were diagnosed at a chronological age younger than 15 years old for a clinical picture characterized by a peculiar cognitive and behavioral pattern or genital anomalies and abnormalities of pubertal development. The classical karyotype 47 XXY was present in 10/14 subjects (72%), a mosaic form (46 XY/47 XXY) was present in 2/14 (14%) and a complex aneuploidy (48 XXYY and 48 XXXY)was present in the remaining 2/14 (14%) patients. All KS patients diagnosed in childhood and adolescence (10/14 =71.4 %) showed a stature taller than the respective target height and also the predicted final height (calculated from a chronological age older than 7 years old) and the reached final height were significantly taller than target height. CONCLUSION: according to our retrospective data we can assert that KS in paediatric age is characterized by a stature taller than target


Assuntos
Síndrome de Klinefelter/diagnóstico , Adolescente , Criança , Pré-Escolar , Crescimento , Humanos , Recém-Nascido , Síndrome de Klinefelter/fisiopatologia , Masculino , Estudos Retrospectivos
14.
Pediatr Med Chir ; 35(4): 187-90, 2013.
Artigo em Italiano | MEDLINE | ID: mdl-24245102

RESUMO

UNLABELLED: Rapid-onset Obesity with Hypothalamic Dysfunction, Hypoventilation, and Autonomic Dysregulation (ROHHAD) is a rare and complex pediatric disorder. Children typically show ROHHAD after the first years of life with rapid weight gain and subsequently autonomic nervous system dysregulation (altered pain perception, pupillary dysfunction, hypothermia and bradycardia); alveolar hypoventilation with risk of cardiorespiratory arrest and hypothalamic dysfunction (central diabetes insipidus, hypothyroidism, growth hormone and corticotrophin deficiency). Tumours of neural crest origin, such as ganglioneuroblastoma and ganglioneuronoma, are reported in 33% of the patients and may be found in the chest or abdomen. Here we describe two girls who presented with rapid weight gain, at the age of 5 and 9 years respectively. The first was admitted due to obesity and central hypothyroidism. After two months she rapidly developed a clinical picture characterized by thermal dysregulation, hypodipsia and severe hypernatriemia, hypertrigliceridemia, alveolar hypoventilation supported by mechanical ventilation. The second presented with rapid-onset obesity and a mild hyperprolactinemia. After three months of follow-up she was admitted due to a clinical picture of hypothermia, seizures and hyponatremia. Subsequentely she developed altered water balance (severe hypernatremia) and severe hypoventilation. Chest CT and MR imaging showed a posterior mediastinal mass. Endocrinological investigation showed corticotrophin deficiency and central hypothyroidism treated with specific replacement therapies. CONCLUSIONS: On the basis of our experiences we can infer that it is necessary perform specific further investigations of hypothalamic function in all the children with rapid onset obesity in order to early prevent the catastrophic consequences that may occur in this syndrome.


Assuntos
Doenças do Sistema Nervoso Autônomo/diagnóstico , Doenças Hipotalâmicas/diagnóstico , Hipoventilação/diagnóstico , Obesidade/diagnóstico , Criança , Pré-Escolar , Feminino , Humanos , Síndrome
15.
J Chem Phys ; 137(9): 094908, 2012 Sep 07.
Artigo em Inglês | MEDLINE | ID: mdl-22957595

RESUMO

Structure and dynamics of water confined in channels of diameter of few nanometer in size strongly differ from the ones of water in the bulk phase. Here, we present radiowave dielectric relaxation measurements on water-filled single-walled carbon nanotubes, with the aim of highlighting some aspects on the molecular electric dipole organization of water responding to high spatial confinement in a hydrophobic environment. The observed dielectric spectra, resulting into two contiguous relaxation processes, allow us to separate the confined water in the interior of the nanotubes from external water, providing support for the existence in the confinement region of water domains held together by hydrogen bonds. Our results, based on the deconvolution of the dielectric spectra due to the presence of a bulk and a confined water phase, furnish a significantly higher Kirkwood correlation factor, larger than the one of water in bulk phase, indicating a strong correlation between water molecules inside nanotubes, not seen in bulk water.

16.
Radiol Med ; 117(6): 979-91, 2012 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-22580809

RESUMO

PURPOSE: The aim of this study was to assess the baseline computed tomography (CT) attenuation of acute and chronic pulmonary thromboemboli, their contrast enhancement (CE), correlation with haematocrit (Ht) levels and the presence of hypertrophic bronchial arteries. MATERIALS AND METHODS: From January 2006 to October 2009, we measured the baseline and postcontrast attenuation values of acute pulmonary thrombi emboli on CT angiograms of 86 patients with acute pulmonary embolism (PE) and those of chronic thrombi in 29 patients with pulmonary hypertension of various origins. The attenuation of acute thrombi was correlated with Ht and CE of chronic thrombi with the presence of hypertrophic bronchial arteries. RESULTS: Acute emboli had a mean baseline attenuation of 54.9 Hounsfield units (HU) and showed no CE. The attenuation of acute thrombi was not dependent on Ht. Chronic thrombi had a mean baseline attenuation of 33.8 HU, and 54% of thrombi showed significant CE. In 57% of cases, a collateral circulation had developed. In 76.5% of cases, CE and hypertrophic bronchial arteries coexisted (p=0.026). Neither thrombotic CE nor bronchial artery hypertrophy predominated in any one of the diseases associated with chronic thrombosis. CONCLUSIONS: Before contrast administration, acute emboli coare prevalently hyperattenuating and therefore more conspicuous. Only chronic thrombi exhibit CE, and CE is significantly associated with the development of collateral circulation, which may be involved in the process of thrombotic recanalisation.


Assuntos
Artéria Pulmonar/diagnóstico por imagem , Embolia Pulmonar/diagnóstico por imagem , Tomografia Computadorizada por Raios X/métodos , Adulto , Idoso , Idoso de 80 Anos ou mais , Artérias Brônquicas/diagnóstico por imagem , Artérias Brônquicas/fisiopatologia , Meios de Contraste , Feminino , Humanos , Hipertensão Pulmonar/diagnóstico por imagem , Hipertensão Pulmonar/fisiopatologia , Iopamidol/análogos & derivados , Masculino , Pessoa de Meia-Idade , Artéria Pulmonar/fisiopatologia , Embolia Pulmonar/fisiopatologia , Estatísticas não Paramétricas
17.
J Endocrinol Invest ; 34(4): 292-5, 2011 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-20543555

RESUMO

BACKGROUND: GH-IGF-I axis is mainly involved in the complex process of somatic growth but emerging evidence suggests that it also influences hypothalamic-pituitary-gonadal (HPG) function. SUBJECTS: We report some data regarding long-term auxological and pubertal outcome of five female patients with hereditary forms of GH-IGF-I deficiency (Laron and GH-gene deletion syndrome) and a mean age of 23.4±5.3 yr (range 19-32). METHODS: All the patients received recombinant human IGF-I (rhIGF-I, Pharmacia and Upjohn, Stockholm, Sweden, and rhIGF-I, Genentech, San Francisco, CA, USA) from a mean age of 8.6 yr (range 3.2-14.2) up to the final height. RESULTS: Final height was very disappointing (≤ -5.0 SD scores) and lower than target height in all the patients. Pubertal onset was delayed in most of them but menarche occurred spontaneously in all the patients. Median age at menarche was 15.1 yr. Menstrual cycles were regular for several years. Median duration of gynecological follow- up was 8.3 yr with the longest span of 17.2 yr. CONCLUSION: We can assert that GH-IGF-I axis has an essential role in promoting linear growth in humans and its physiological action cannot be replaced by pharmacological treatment in most patients with hereditary forms of IGF-I insufficiency as demonstrated by their subnormal final height. Our clinical observations can also support an essential role of IGF-I in genitalia growth but not in the function of HPG axis as demonstrated by the maintenance of regular menstrual cycles in the presence of subnormal levels of IGF-I after treatment discontinuation.


Assuntos
Hormônio do Crescimento Humano/genética , Fator de Crescimento Insulin-Like I/deficiência , Fator de Crescimento Insulin-Like I/uso terapêutico , Síndrome de Laron/fisiopatologia , Puberdade/fisiologia , Adolescente , Adulto , Criança , Pré-Escolar , Feminino , Hormônio do Crescimento Humano/deficiência , Hormônio do Crescimento Humano/uso terapêutico , Humanos , Síndrome de Laron/genética , Ciclo Menstrual/fisiologia , Proteínas Recombinantes/uso terapêutico , Adulto Jovem
18.
Radiol Med ; 116(2): 230-45, 2011 Mar.
Artigo em Inglês, Italiano | MEDLINE | ID: mdl-21311989

RESUMO

PURPOSE: Evaluation of computed tomography (CT) pulmonary angiography parameters revealing pulmonary embolism (PE) severity with particular attention to pulmonary obstruction indexes. Comparison with clinical and hemodynamic data and determination of predictive role in the development of chronic pulmonary heart disease. MATERIALS AND METHODS: This retrospective study analyzes 45 not consecutive patients from November 2007 to December 2008 with CT angiography diagnosis of acute PE. Included in the study are patients at the first documented episode of acute PE, with 6 month follow-up. Patients with severe pre-existent cardiopulmonary pathology or neoplastic diseases were excluded from the study. CT angiography evaluated right ventricular (RV)/left ventricular (LV) ratio, obstruction index according to Qanadli and Total Clot Burden (Ghanima score). PE indexes were compared with Troponin I measurement and echocardiography result; at last hospitalization and intensive care time were reported. RESULTS: A significant association was found between Ghanima and Qanadli score: the two indexes are equivalent in quantification of pulmonary arterial obstruction (p<0.001). Among others CT parameters, the new Ghanima score evidenced the best accuracy to detect patients evolving to chronic pulmonary heart disease (76%). This value is higher than that of echocardiography (71%). Troponins showed highest accuracy (82%). CONCLUSIONS: Ghanima score can be used in emergency CT angiography diagnosis as prognostic marker for a quickly risk stratification of pulmonary heart disease or death in patients with acute PE. This approach allows to obtain, with just one test, both the diagnosis and a rather accurate acute PE risk stratification.


Assuntos
Artéria Pulmonar/diagnóstico por imagem , Embolia Pulmonar/diagnóstico por imagem , Doença Cardiopulmonar/diagnóstico por imagem , Tomografia Computadorizada por Raios X/métodos , Doença Aguda , Adulto , Idoso , Idoso de 80 Anos ou mais , Angiografia/métodos , Distribuição de Qui-Quadrado , Meios de Contraste , Feminino , Humanos , Iopamidol/análogos & derivados , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Prognóstico , Artéria Pulmonar/patologia , Doença Cardiopulmonar/patologia , Curva ROC , Estudos Retrospectivos , Sensibilidade e Especificidade , Índice de Gravidade de Doença , Estatísticas não Paramétricas
19.
Sci Rep ; 10(1): 2814, 2020 02 18.
Artigo em Inglês | MEDLINE | ID: mdl-32071361

RESUMO

Autoantibodies, which are antibodies that target self-epitopes, have considerable diagnostic, prognostic and predictive value in specific autoimmune diseases. Various infectious agents have been linked via numerous mechanisms to the formation of different autoantibodies. Therefore, estimating the prevalence of autoantibodies and anti-infectious antibodies in different populations is of high importance. Different genetic and environmental pressures, such as these found in Ghana's different geographical provinces, may affect the prevalence of autoantibodies. In this study, we assessed the seroprevalence of a diverse panel of autoantibodies and anti-infectious antibodies among the healthy Ghanaian population and investigated possible environmental and genetic predispositions for autoantibodies and autoimmunity. The sera of 406 healthy individuals were obtained from Greater Accra, Upper West, Eastern and Volta regions. Multiplexed assay and chemiluminescent immunoassay techniques were utilized to assess the presence of a panel of autoantibodies and anti-infectious antibodies. We found a high prevalence of anti-HSV-1 IgG (91-100%), anti-EBNA IgG (81-93%) and anti-EBV-VCA IgG (97-100%) antibodies. The prevalence of ANA (at least one of: anti-dsDNA; anti-chromatin; anti-ribosomal-P; anti-Ro/SSA; anti-La/SSB; anti-centromere B; anti-Sm; anti-Sm/RNP; anti-Scl-70; anti-Jo1; anti-DFS70) was estimated at 14%. An inverse association between anti-HSV-2 antibodies and ANA (p = 0.044; adjusted OR = 0.398; CI [0.162-0.975]) was found, after adjusting for differences in gender, age, and familial history of autoimmune diseases. A trend towards reduced seroprevalence of anti-dsDNA antibodies among subjects who were positive for anti-HSV-2 antibodies was also noted (p = 0.1). In conclusion, the inverse association between anti-HSV-2 antibodies and ANA positivity suggests a possible protective role of HSV-2 infection against autoimmunity.


Assuntos
Anticorpos Antinucleares/sangue , Anticorpos Antifosfolipídeos/sangue , Anticorpos Antivirais/sangue , Doenças Autoimunes/epidemiologia , Infecções por Vírus Epstein-Barr/epidemiologia , Herpes Simples/epidemiologia , Adolescente , Adulto , Idoso , Doenças Autoimunes/imunologia , Infecções por Vírus Epstein-Barr/imunologia , Feminino , Gana , Herpes Simples/imunologia , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Soroepidemiológicos , Adulto Jovem
20.
J Endocrinol Invest ; 32(6): 519-24, 2009 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-19474521

RESUMO

BACKGROUND: Few papers focus on exercise-related blood glucose (BG) in patients on continuous sc insulin infusion (CSII) or multiple daily injections (MDI) with glargine. AIM: The main objective was to evaluate the degree of glycemic control in Type 1 diabetes mellitus adolescents on CSII doing physical activity with pump switched on or off. These findings were also compared with a small group of patients on MDI with glargine. SUBJECTS AND METHODS: Eight patients on CSII (basal rate continued or turned off in alternating sessions) and 5 on MDI joined 4 sessions of moderate-severe exercise. RESULTS: Post-exercise BG significantly increased with the pump off and was unchanged/decreased with the pump on and MDI groups vs baseline. The hypoglycemia rate was not different among the 3 groups at any time. Pump on: hypoglycemias more frequent both at bedtime (p=0.031) and at awakening (p<0.001) than before dinner and at awakening than at bed-time (p=0.044). Pump off: hypoglycemias more frequent both at bed-time (p=0.010) and at awakening (p=0.031) than before dinner. MDI: no differences. CONCLUSIONS: Glargine is safe and reducing the pre-lunch insulin is unnecessary. Subjects on insulin pump should not stop the basal rate. If they stop the pump, some actions are advisable: pre-exercise insulin bolus, pre-sleeping snack rich in carbohydrates, slight reduction of the overnight basal rate. On the other hand, if the basal rate is unmodified, the ingestion of sugary drinks during the exercise, the reduction of the overnight basal rate, a reduction of the pre-dinner insulin bolus and/or a pre-sleeping snack should be considered.


Assuntos
Glicemia/metabolismo , Diabetes Mellitus Tipo 1/sangue , Diabetes Mellitus Tipo 1/tratamento farmacológico , Sistemas de Infusão de Insulina , Insulina/análogos & derivados , Aptidão Física/fisiologia , Adolescente , Criança , Feminino , Índice Glicêmico , Humanos , Insulina/administração & dosagem , Insulina Glargina , Insulina Lispro , Insulina de Ação Prolongada , Masculino , Estatísticas não Paramétricas
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