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1.
Ann Bot ; 124(5): 849-860, 2019 11 15.
Artigo em Inglês | MEDLINE | ID: mdl-31361802

RESUMO

BACKGROUND AND AIMS: At the rear edge of the distribution of species, extreme isolation and small population size influence the genetic diversity and differentiation of plant populations. This may be particularly true for Arctic-alpine species in mid-latitude mountains, but exactly how peripherality has shaped their genetic and reproductive characteristics is poorly investigated. The present study, focused on Salix herbacea, aims at providing new insights into the causes behind ongoing demographic dynamics and their consequences for peripheral populations of Arctic-alpine species. METHODS: We performed a whole-population, highly detailed sampling of the only two S. herbacea populations in the northern Apennines, comparing their clonal and genetic diversity, sex ratio and spatial genetic structure with a reference population from the Alps. After inspecting ~1800 grid intersections in the three populations, 563 ramets were genotyped at 11 nuclear microsatellite markers (nSSRs). Past demography and mating patterns of Apennine populations were investigated to elucidate the possible causes of altered reproductive dynamics. KEY RESULTS: Apennine populations, which experienced a Holocene bottleneck and are highly differentiated (FST = 0.15), had lower clonal and genetic diversity compared with the alpine population (RMLG = 1 and HE = 0.71), with the smaller population exhibiting the lowest diversity (RMLG = 0.03 and HE = 0.24). An unbalanced sex ratio was found in the larger (63 F:37 M) and the smaller (99 F:1 M) Apennine population. Both were characterized by the presence of extremely large clones (up to 2500 m2), which, however, did not play a dominant role in local reproductive dynamics. CONCLUSIONS: Under conditions of extreme isolation and progressive size reduction, S. herbacea has experienced an alteration of genetic characteristics produced by the prevalence of clonal growth over sexual reproduction. However, our results showed that the larger Apennine population has maintained levels of sexual reproduction enough to counteract a dramatic loss of genetic and clonal diversity.


Assuntos
Salix , Regiões Árticas , Variação Genética , Genética Populacional , Genótipo , Repetições de Microssatélites , Reprodução
2.
Pediatr Med Chir ; 34(3): 133-42, 2012.
Artigo em Inglês | MEDLINE | ID: mdl-22966726

RESUMO

BACKGROUND: Caudal block with levobupivacaine or ropivacaine is the most commonly used regional anaesthesia in children. METHODS: The aim of study was to compare the cardiocirculatory profile induced in two matched groups of young patients, submitted to caudal anaesthesia with levobupivacaine or ropivacaine for an elective subumbilical surgery. Sixty children were enrolled: thirty received levopubivacaine 0.25% and thirty ropivacaine 0.2%. Intraoperative heart rate (HR), systolic blood pressure (SBP), diastolic blood pressure (DBP) were monitored at following times: Ta0 (after anaesthesia induction), Tal (after caudal anaesthesia), Ta2 (five minutes later), Ta3 (ten minutes later), Ts1 (at surgical incision), Ts2, Ts3, Ts4, Ts5 (every 10 minutes during surgery), Taw (at the awakening). RESULTS: In both groups the cardiocirculatory trend remained within normal ranges at all times considered, demonstrating the safety of the method with both drugs. Both groups showed a similar trend at the different monitoring times: low decrease in HR, SBP and DBP after caudal block, slight increase in parameters after skin incision, slight decrease during surgery, increase at awakening. Regarding SBP and DBP, the levobupivacaine group children generally showed higher levels compared to the ropivacaine group, especially for DBP. CONCLUSIONS: Paediatric caudal anaesthesia is an effective method with an very infrequent complication rate. Possible hypotheses for differing haemodynamic behaviour could include a stronger vasoconstriction reflex of innervated areas during caudal anaesthesia with levobupivacaine and a lower levobupivacaine induced block of the sympathetic fibers, related to different pharmacokinetic profile of low concentrations of the local anaesthetics used in paediatric epidural space.


Assuntos
Amidas/farmacologia , Anestesia Caudal/métodos , Anestésicos Locais/farmacologia , Pressão Sanguínea/efeitos dos fármacos , Frequência Cardíaca/efeitos dos fármacos , Monitorização Intraoperatória , Bupivacaína/análogos & derivados , Bupivacaína/farmacologia , Pré-Escolar , Feminino , Humanos , Levobupivacaína , Masculino , Ropivacaina
3.
Pediatr Med Chir ; 31(3): 130-3, 2009.
Artigo em Inglês | MEDLINE | ID: mdl-19739493

RESUMO

PURPOSE: Colonic atresia and stenosis are rare causes of intestinal obstruction in the infant. Only 1.8%-15% of intestinal atresias occur in the colon. Congenital colonic stenosis is even less common than colonic atresia. Only 10 cases have been reported in Literature since 1966 and only one late-onset case has been reported in Literature until now. We describe the case of a 4-month-old baby coming to our attention because of an intestinal subocclusion due to a congenital colonic stenosis of the ascending colon. CASE REPORT: A 4-month-old baby came to our attention for persistent abdominal distension, reduction of bowl function and decaying of overall clinical conditions. A plain abdominal radiograph showed distended intestinal loops with air-fluid levels and no gas in the rectum. During the barium enema the contrast medium appeared to completely fill the lumen of the colon up to the ileo-cecal valve and Cecum appearing higher than normal. Beyond the ileo-cecal valve, the contrast medium showed an abnormal hypotonic dilatation of the small intestinal loops. Suspecting an organic intestinal obstruction, an explorative laparotomy was deemed necessary and at halfway in the ascending colon a stenosis was found. RESULTS: The post-operative course was uneventful and the patient is currently in good clinical conditions, has a normal diet and is thriving. CONCLUSION: Considering both the Literature and our own experience, it is wise to reckon the congenital colonic stenosis as a rare but possible cause of complete or partial intestinal obstruction not only in the newborn but also throughout the first year of life.


Assuntos
Doenças do Colo/congênito , Obstrução Intestinal/congênito , Fatores Etários , Anastomose Cirúrgica , Doenças do Colo/diagnóstico por imagem , Doenças do Colo/cirurgia , Constrição Patológica/congênito , Humanos , Lactente , Obstrução Intestinal/diagnóstico por imagem , Obstrução Intestinal/cirurgia , Laparotomia , Masculino , Radiografia Abdominal , Resultado do Tratamento
4.
J Matern Fetal Neonatal Med ; 28(13): 1602-7, 2015 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-25234103

RESUMO

OBJECTIVE: We examined the reliability of the main prenatal and postnatal prognosis-related indexes that can be used to evaluate congenital diaphragmatic hernia (CDH) outcome. METHODS: Seventy-seven neonates with CDH were analyzed according to CDH prognosis-related factors, divided into prenatal findings, postnatal clinical values and postnatal predictive outcome scores applied at birth and within the first 12-24 h. The data are compared between two groups: survivors and non-survivors. RESULTS: During prenatal age, major associated anomalies, intrathoracic stomach, diagnosis prior to 25 weeks of gestational age and lung-to-head ratio < 0.6 were statistically significant, demonstrating their greater incidence in non-survivors. The majority of postnatal values at PICU admission were found to be reliable in identifying the CDH outcome: paO2/FiO2, oxygenation index, alveolar-arterial-O2 gradient, arterial-alveolar-O2 tension ratio, pH, mean blood pressure, body temperature. All the postnatal predictive outcome scores (Apgar 1' and 5', CDH-Study-Group equation, Score for Neonatal-Acute-Physiology II, SNAP-Perinatal-Extension II, Pediatric Risk of Mortality III and Wilford-Hall/Santa-Rosa formula) were statistically significant with more favorable values for prognosis in the survivors group. CONCLUSION: The chances of predicting CDH outcome are fairly high. During prenatal age, only a few findings may be obtained. Conversely, many postnatal indexes and scores can reliably predict such outcome.


Assuntos
Indicadores Básicos de Saúde , Hérnias Diafragmáticas Congênitas/diagnóstico , Resultado da Gravidez , Diagnóstico Pré-Natal/métodos , Gasometria , Feminino , Idade Gestacional , Hérnias Diafragmáticas Congênitas/sangue , Hérnias Diafragmáticas Congênitas/mortalidade , Hérnias Diafragmáticas Congênitas/terapia , Humanos , Recém-Nascido , Masculino , Valor Preditivo dos Testes , Gravidez , Resultado da Gravidez/epidemiologia , Prognóstico , Reprodutibilidade dos Testes
5.
Clin Pharmacol Ther ; 57(4): 446-54, 1995 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-7712674

RESUMO

OBJECTIVE: To study the possibility that the penetration of the antibiotic ciprofloxacin into polymorphonuclear leukocytes (PMN) may be associated with some changes in cell reactivity. DESIGN: Superoxide anion and chemiluminescence generation induced by formyl-methionyl-leucyl-phenylalanine (fMLP) and platelet-activating factor (PAF) were studied ex vivo in 12 healthy volunteers (mean age, 53.15 +/- 16.3 years; mean body weight, 71.23 +/- 6.9 kg) at fixed intervals up to 72 hours from the administration of a single oral dose of 250 mg ciprofloxacin. Cytosolic free calcium levels ([Ca2+]i) in resting and stimulated cells were also evaluated. The dynamic parameters of the effects on PMNs were compared with the kinetic profile of the drug in plasma and in PMNs. RESULTS: Superoxide generation induced by the stimulating agents increased significantly, reaching a peak after 12 hours (+116% [p < 0.001] for fMLP and +66% [p < 0.05] for PAF). Similarly, chemiluminescence production showed a threefold increase in the response to the stimulating agents 12 hours after drug administration (p < 0.001). The increase in [Ca2+]i in stimulated PMNs was significantly potentiated (p < 0.001). The mathematic analysis of the effects of ciprofloxacin showed that time to maximal activity was between 10.4 hours (PAF-dependent [Ca2+]i increase), and 15 hours (fMLP-induced superoxide anion and chemiluminescence production). The ratio of PMNs to plasma ciprofloxacin concentration increased progressively, from 0.5 at 30 minutes to 10.4 after 24 hours. In addition, time to maximal activity and half-life differed in PMNs and in plasma (4.66 versus 1.90 hours and 13.03 versus 7.28 hours, respectively). CONCLUSIONS: Ciprofloxacin administration induced a long-lasting enhancement of PMN reactivity to fMLP and PAF. The levels of the drug in the cells were greater and more sustained in the time than those in plasma.


Assuntos
Ciprofloxacina/farmacologia , Neutrófilos/metabolismo , Adulto , Idoso , Análise de Variância , Ciprofloxacina/sangue , Ciprofloxacina/farmacocinética , Feminino , Meia-Vida , Humanos , Medições Luminescentes , Masculino , Pessoa de Meia-Idade , N-Formilmetionina Leucil-Fenilalanina/farmacologia , Neutrófilos/efeitos dos fármacos , Fator de Ativação de Plaquetas/farmacologia , Fatores de Tempo
6.
Eur J Cancer Prev ; 9(1): 35-9, 2000 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-10777008

RESUMO

Populations resident in the historical town of Venice and in the inland industrial city of Mestre are at different risk of exposure to environmental pollutants. This case-control study compares the risk of developing lung cancer in the two populations in relation to known risk factors for this neoplasm. A retrospective study of 305 incident cases of lung and 447 frequency-matched population controls was conducted through a standard questionnaire on main risk factors for lung cancer. Completeness of cases was checked against the Venetian Cancer Registry files. The results indicate that lung cancer risk associated with tobacco smoking was high in both areas, although more elevated in Venice islands among heavy smokers. An elevation of risk was associated with housing without a heating system, possibly suggesting a role of worse hygienic conditions. An increased risk associated with exposure to occupational carcinogens was detected in the inland area. In conclusion, lung cancer risk due to tobacco smoking largely affects both the populations, while other risks such as occupation or housing conditions appear to be more population-specific.


Assuntos
Poluição do Ar em Ambientes Fechados/efeitos adversos , Neoplasias Pulmonares/etiologia , Fumar/efeitos adversos , Adulto , Idoso , Carcinógenos/efeitos adversos , Estudos de Casos e Controles , Feminino , Habitação , Humanos , Incidência , Itália/epidemiologia , Neoplasias Pulmonares/epidemiologia , Masculino , Pessoa de Meia-Idade , Exposição Ocupacional , Estudos Retrospectivos , Medição de Risco , Ventilação
7.
Chemosphere ; 44(8): 1723-36, 2001 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-11534904

RESUMO

The genotoxic effects of different size fractions of airborne particulate (Total, PM10 and PM25), extracted with acetone or toluene, were evaluated by: the Ames plate test (TA98 and TA100 strains, w/o S9), gene conversion and reversion (w/o endogenous metabolic activation) in the Saccharomyces cerevisiae D7 strain, and the comet assay on human leukocytes. The data on human leukocytes confirm the sensitivity of the comet assay and its applicability to assess genotoxicity in environmental samples. The PM2.5 fraction of airborne particulate generally shows the highest concentration of DNA-damaging compounds. Genotoxic response, in all the test systems applied, is highly dependent on extraction solvent used. Acetone seems to extract compounds with more similar genotoxic responses in the three test systems used than toluene extracts. Toluene appears to extract air pollutants genotoxic on yeast and leukocytes but is mainly cytotoxic on Salmonella.


Assuntos
Poluentes Atmosféricos/toxicidade , Dano ao DNA , Acetona/química , Cidades , Ensaio Cometa , Humanos , Leucócitos/efeitos dos fármacos , Testes de Mutagenicidade , Tamanho da Partícula , Saccharomyces cerevisiae/efeitos dos fármacos , Saccharomyces cerevisiae/genética , Salmonella typhimurium/efeitos dos fármacos , Salmonella typhimurium/genética , Tolueno/química
8.
Acta Cytol ; 32(1): 75-8, 1988.
Artigo em Inglês | MEDLINE | ID: mdl-2447724

RESUMO

The cytologic picture in fine needle aspirates from a unique type of breast tumor, with stromal proliferation of osteoclastlike giant cells, cartilaginous metaplasia and metaplastic spindle-shaped carcinomatous cells, is described. In this case, an erroneous cytologic diagnosis of fat necrosis associated with a rich component of reactive fibroblasts was made. This false-negative report, which was mainly due to lack of an obvious carcinomatous component in the aspirated material, is discussed with emphasis placed on the need to exclude spindle cell metaplasia in a breast carcinoma whose aspirates are characterized by a rich component of spindle-shaped cells.


Assuntos
Neoplasias da Mama/patologia , Idoso , Neoplasias da Mama/diagnóstico , Diagnóstico Diferencial , Feminino , Humanos , Metaplasia , Coloração e Rotulagem
9.
Pediatr Med Chir ; 18(5): 487-92, 1996.
Artigo em Italiano | MEDLINE | ID: mdl-9053888

RESUMO

NEC represents the most common gastrointestinal disorder in newborn. Its range varies from 1% to 7.7% and is frequently associated with factors such as intestinal ischaemia, prematurity, gastrointestinal infection and early and rapid enteral feeding. Between 15/1/1990 and 15/6/1995, 129 critically ill newborns were admitted in NICU of Policlinico S. Orsola-Bologna. We examined only 93 patients, hospitalized for over 48 hours, presenting one or more risk factors for the development of NEC, such as birthweight < 2000 gm, respiratory distress, gastrointestinal bacterial colonization, sepsis, PDA and use of umbilical catheters. The aim of the study was to evaluate NEC incidence in newborns exposed to this complication and the analysis of risk factors associated with the elements of prevention and protection. No cases of NEC were observed despite the high incidence of risk factors. The newborns studied were divided in six different groups with increasing risk factors. Among the prevention elements of NEC, every patient was treated by nutrition, at first exclusively by TPN followed by careful enteral feeding (< 20 ml/kg/die) and the improvement of mesenteric blood flow by dopamine (2-3 mcg/kg/min); other preventive treatments were given according to clinical condition: dobutamine (5-10 mcg/kg/min in 51 ps.) to improve the cardiovascular function, gastrointestinal decontamination (8 ps.), antibiotic therapy (81 ps.), in cases of diagnosed infection and intravenous immunoglobulin (25 ps.) after discovering low ematic values. Analyzing the treatments and their day numbers in the 6 groups of patients no statistically significant differences were evident. On the contrary, dividing the patients into 3 groups according to GA (< 30 w, 30-35 w, > 35 w) an extension in treatment time is more evident in the group of GA < 30 weeks. Our therapeutic behaviour, based on respect of gastrointestinal blood flow, careful and gradual enteral feeding and prevention, constant monitoring and infection treatment, has been useful to stop the NEC incidence.


Assuntos
Enterocolite Pseudomembranosa/prevenção & controle , Emergências , Enterocolite Pseudomembranosa/etiologia , Enterocolite Pseudomembranosa/terapia , Humanos , Recém-Nascido , Recém-Nascido Prematuro , Tempo de Internação , Nutrição Parenteral Total , Fatores de Risco
10.
Pediatr Med Chir ; 15(2): 145-50, 1993.
Artigo em Italiano | MEDLINE | ID: mdl-8321716

RESUMO

It is a matter of discussion if bladder augmentation should or should not utilize in the treatment of exstrophy-epispadias complex. When staged functional bladder reconstruction is adopted in the most difficult cases, or when one desires to avoid early and/or subsequent urinary diversions, sometimes a bladder enhancement could be necessary. The indications should be: a progressive damage to the upper urinary tract, after bladder closure at birth, due to a very small, no compliant detrusor plate (this is a rare condition: only one case in our series); a bladder capacity < or = 80 mls at the time of bladder neck reconstruction (this is a frequent but questionable condition: three cases in our series); a progressive damage of the upper urinary tract after bladder neck reconstruction (this is an unexpected, but not rare condition: 3 cases in our series); during every undiversion procedure (bladder augmentation is nearly mandatory during undiversion in cloacal or bladder exstrophies previously diverted: 4 cases in our series). In our experience, 12 bladder augmentations (in 11 patients, over 85 cases we observed) were done at median age of 8 years and 5 months. Different intestinal segments have been used: the sigmoid colon, the ileo-cecal portion, an ileal tract and the stomach. Follow-up ranges from 11 years to 12 months; until now, we observed few surgical complications: a bowel obstruction in one patient, a left ureteral partial stenosis at level of the anastomosis with the gastric patch wall in another patient and bladder lithiasis in 5 patients.(ABSTRACT TRUNCATED AT 250 WORDS)


Assuntos
Extrofia Vesical/cirurgia , Ceco/transplante , Colo Sigmoide/transplante , Epispadia/cirurgia , Íleo/transplante , Estômago/transplante , Bexiga Urinária/cirurgia , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Métodos , Complicações Pós-Operatórias/epidemiologia
11.
Pediatr Med Chir ; 25(5): 341-4, 2003.
Artigo em Italiano | MEDLINE | ID: mdl-15058832

RESUMO

Between 0,7-3% of pediatric patients may require resuscitation during hospital stay. The physicians of the Pediatric Intensive Care Unit of the C.O.U. Anesthesia and Intensive Care-Baroncini developed a plan for the management of pediatric emergencies inside the Department of Pediatric Medical and Surgical Sciences. The plan consisted of: the drawing up of a PI 34-Procedure; the preparation and implementation of a training course for doctors and nurses; the purchase of 12 emergency-trolleys according to the Broselow Pediatric Resuscitation Measuring Tape and the implementation of a specific system for the emergency-call. Precise duty for anesthesiologists and intensivists is the emergency- planning and management, in order to diffuse the medical knowledge needed to assist patients requiring vital functions support. The management of the intra-hospital pediatric emergencies is strictly dependent on the training of the staff, based on a specific support algorithm, and specific equipment for the different ages.


Assuntos
Reanimação Cardiopulmonar/métodos , Hospitalização , Reanimação Cardiopulmonar/normas , Criança , Humanos
18.
Radiol Med ; 87(6): 758-62, 1994 Jun.
Artigo em Italiano | MEDLINE | ID: mdl-8041928

RESUMO

Stereotaxis is a well-known technique allowing fine-needle aspiration cytology and preoperative localization of the nonpalpable breast lesions seen on mammograms, permitting early diagnosis and easy surgical ablation. The authors report on their experience (126 cases) with stereotactic aspiration (85 cases) and preoperative localization (41 cases) of nonpalpable lesions. The technique allowed to hit the lesion in 115/126 cases (91.3%). Cytology showed 24 cases of cancer and 38 benign lesions (3 of them were false negatives); in 8 cases cytology was suspect (7 cancers and 1 sclerosing adenosis) and in 15 inadequate (17.7%). Sensitivity and specificity were confirmed by histology or follow-up (6 months + 6 months); sensitivity was 91.2% and specificity 97.2% (excluding inadequate cases). The 41 needle localizers were useful to detect 37 malignancies. Fifty-one lesions were biopsied: 37 of them were malignant and 14 benign (benign/malignant ratio = 0.38/1).


Assuntos
Neoplasias da Mama/diagnóstico por imagem , Mamografia/métodos , Radiografia Intervencionista/métodos , Adulto , Idoso , Biópsia por Agulha/instrumentação , Biópsia por Agulha/métodos , Biópsia por Agulha/estatística & dados numéricos , Mama/patologia , Neoplasias da Mama/epidemiologia , Neoplasias da Mama/patologia , Citodiagnóstico/instrumentação , Citodiagnóstico/métodos , Citodiagnóstico/estatística & dados numéricos , Feminino , Seguimentos , Humanos , Mamografia/instrumentação , Mamografia/estatística & dados numéricos , Pessoa de Meia-Idade , Radiografia Intervencionista/instrumentação , Radiografia Intervencionista/estatística & dados numéricos , Sensibilidade e Especificidade , Técnicas Estereotáxicas/instrumentação , Técnicas Estereotáxicas/estatística & dados numéricos
19.
Acta Anaesthesiol Scand ; 48(1): 61-8, 2004 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-14674975

RESUMO

BACKGROUND: Optimum transfer of energy from the left ventricle to the arterial circulation requires appropriate matching of these mechanical systems. Left ventricular-arterial coupling describes this relationship between the ventricular elastance (Ees) and arterial elastance (Ea). The ratio of these elastances defines the efficiency of myocardium and provides in our study a useful technique for assessment of the actions of remifentanil. The purpose of this study was to evaluate the effects of remifentanil on ventriculo-arterial coupling in cardiac surgery in patients with coronary artery disease. METHODS: Fourteen patients with coronary artery disease, submitted intraoperatively to cardiac anesthesia for myocardial revascularization, were examined prospectively. With the use of transesophageal echocardiography (TEE) and different dicrotic arterial pressures, we determined the ventricle elastance (Ees), the arterial elastance (Ea) and myocardial efficiency before and after administration of a slow-bolus of remifentanil (1 micro kg(-1)). RESULTS: Remifentanil decreases significantly the ventricular elastance (from 6.09 mmHg ml-1 m(-2) to 4.88) (P < 0.05), with a less, but however, significant decrease of arterial elastance (from 3.68 mmHg ml(-1) m(-2) to 3.13) (P < 0.05). Despite causing simultaneous declines, maintains a good myocardial efficiency (0.64-0.68) with no significant difference. CONCLUSION: Although remifentanil depresses ventricular and arterial elastance, preserves a good left ventricular-arterial coupling and mechanical efficiency, despite a little increase of coupling. However, these effects are maintained only during a slow intravenous infusion and are dose-dependent with impairment of coupling, that may contribute to decline in overall cardiovascular performance, at higher anesthetic dose and rapid infusion in patients with a severe myocardial dysfunction.


Assuntos
Anestésicos Intravenosos , Artérias/fisiologia , Doença da Artéria Coronariana/fisiopatologia , Coração/fisiologia , Contração Miocárdica/fisiologia , Piperidinas , Idoso , Artérias/efeitos dos fármacos , Pressão Sanguínea/fisiologia , Débito Cardíaco/fisiologia , Ecocardiografia Transesofagiana , Feminino , Coração/efeitos dos fármacos , Ventrículos do Coração/efeitos dos fármacos , Hemodinâmica/efeitos dos fármacos , Humanos , Masculino , Pessoa de Meia-Idade , Revascularização Miocárdica , Miocárdio/metabolismo , Consumo de Oxigênio/efeitos dos fármacos , Remifentanil , Volume Sistólico/efeitos dos fármacos , Função Ventricular
20.
Artigo em Inglês | MEDLINE | ID: mdl-6159266

RESUMO

A new family with May-Hegglin anomaly is presented. 9 patients were found to be affected, namely to present thrombocytopenia, giant platelets, and leukocytes inclusion bodies. A mild to moderate hemorrhagic diathesis was present in 8 patients (easy bruising, excessive bleeding after tooth extraction, menomethrorrhagia). One patient was asymptomatic. The bleeding tendency seemed to be relatively more pronounced in those patients who have larger platelets. Bleeding time was slightly prolonged in 4 of the affected patients. Platelet aggregation to Ristocetin and serotonin release was normal; on the contrary, platelet adhesiveness was slightly decreased in all patients. Plasma Btg was investigated in 7 patients, found to be normal in 5 and elevated in 2. Platelet Btg was found to be increased in all patients investigated. The ratio between Btg and platelet number was elevated in every instance. The circulating platelet mass (Btg platelet mass microgram/ml) was investigated in 7 patients, found normal in two and decreased in the remaining three. The disorder is transmitted as an autosomal dominant trait but there seems to be a variable phenotypic expression from one patient to the other.


Assuntos
Plaquetas/patologia , Trombocitopenia/genética , Adolescente , Adulto , Idoso , Criança , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Adesividade Plaquetária , Agregação Plaquetária , Contagem de Plaquetas , Trombocitopenia/sangue , Trombocitopenia/patologia
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