RESUMO
This trial evaluated the individual and interactional effects of diet and type of pregnancy (twin or single) on plasma metabolic response in ewes and their lambs from late pre-partum to late post-partum. Thus, a flock of 18 Ile de France breed sheep, consisting of 8 twin-bearing and 10 single-bearing ewes, were allocated to one of two groups according to their diet, either based on ad libitum naturalized pasture hay (NPH) or red clover hay (RCH), from d 45 pre-partum to d 60 post-partum. Plasma samples were collected at different times to determine albumin, cholesterol, total protein and urea, plus glucose and ß-hydroxybutyrate (BHB) concentration in ewes. The data was processed using the lme4 package for R, and SPSS Statistics 23.0 for Windows. The results showed that both diet and type of pregnancy influenced the metabolic profile in ewes, showing an inverse relationship between single- and twin-bearing ewes regarding glucose and especially BHB proportions from pre-partum to birth. During post-partum, higher urea concentrations were observed in twin- and single-bearing ewes fed RCH in contrast to those fed NPH, as a result of the higher-quality forage offered to ewes. Regarding lambs, the diet and type of pregnancy influenced the total protein and urea levels, where an inverse relationship at birth and early post-partum between albumin and cholesterol vs. total protein and urea was detected, reflecting a trend (P value between 0.06 and 0.07) to a better performance by groups of single lambs, especially those from single-bearing ewes fed RCH. Finally, under the conditions of this study, the maternal diet and type of pregnancy influenced the plasma metabolic response in ewes and their lambs, affecting the lamb performance especially at birth.
Assuntos
Ração Animal/análise , Dieta/veterinária , Fenômenos Fisiológicos da Nutrição Materna , Prenhez , Gravidez Múltipla , Ovinos/sangue , Animais , Feminino , Humanos , Gravidez , Ovinos/fisiologiaRESUMO
OBJECTIVES: To evaluate the association between learned helplessness (LH) and self-efficacy (SE) with disease activity, functional capacity, and level of pain in patients with rheumatoid arthritis (RA) and to compare LH and SE between patients in remission and patients with active disease. METHOD: This multicentre, cross-sectional study included consecutive patients (aged ≥ 18 years) with RA according to 2010 American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) criteria. LH was measured by the Rheumatology Attitude Index (RAI), Spanish version; SE with the Arthritis Self-efficacy Scale (ASES), Spanish version; functional capacity with the Health Assessment Questionnaire, Argentinian version (HAQ-A); and perceived pain by the visual analogue scale (VAS). Disease activity was measured by the Clinical Disease Activity Index (CDAI). RESULTS: A total of 115 patients (82% females) with a mean (± sd) age of 58 ± 13 years were included. We found a significantly positive correlation between LH and perceived pain (p < 0.001), HAQ-A score (p < 0.001), and CDAI (p < 0.001) and a significantly negative correlation between SE and perceived pain (p < 0.001), HAQ-A score (p < 0.001), and CDAI (p < 0.001). We found greater levels of SE and lower grades of LH in patients in remission compared to those with active disease (median 76 vs. 58; p < 0.001 and 6 vs. 11; p < 0.001, respectively). CONCLUSIONS: LH and SE correlated significantly with disease activity, functional capacity, and perceived pain. Levels of SE were higher in patients in remission compared to those with active disease as opposed to levels of LH, which were lower in patients in remission compared to those with active disease. These results show that cognitive factors are related to disease activity and their modifications may have importance in the management of RA.
Assuntos
Artrite Reumatoide/psicologia , Desamparo Aprendido , Percepção da Dor , Autoeficácia , Idoso , Argentina , Estudos Transversais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Índice de Gravidade de DoençaRESUMO
A study was undertaken to evaluate dietary glutamine supplementation effects on gilthead sea bream performance, intestinal nutrient absorption capacity, hepatic and intestinal glutamine metabolism and oxidative status. For that purpose gilthead sea bream juveniles (mean weight 13.0g) were fed four isolipidic (18% lipid) and isonitrogenous (43% protein) diets supplemented with 0, 0.5, 1 and 2% glutamine for 6weeks. Fish performance, body composition and intestinal nutrient absorption capacity were not affected by dietary glutamine levels. Hepatic and intestinal glutaminase (GlNase), glutamine synthetase (GSase), alanine aminotransferase, aspartate aminotransferase and glutamate dehydrogenase activities were also unaffected by dietary glutamine supplementation. In the intestine GlNase activity was higher and GSase/GlNase ratio was two-fold lower than in the liver, suggesting a higher use of glutamine for energy production by the intestine than by the liver. The liver showed higher catalase and glucose-6-phosphate dehydrogenase activities, while the intestine presented higher glutathione peroxidase and glutathione reductase activities and oxidised glutathione content, which seems to reveal a higher glutathione dependency of the intestinal antioxidant response. Total and reduced glutathione contents in liver and intestine and superoxide dismutase activity in the intestine were enhanced by dietary glutamine, though lipid peroxidation values were not affected. Overall, differences between liver and intestine glutamine metabolism and antioxidant response were identified and the potential of dietary glutamine supplementation to gilthead sea bream's antioxidant response was elucidated.
Assuntos
Aminoácidos/metabolismo , Antioxidantes/farmacologia , Suplementos Nutricionais , Alimentos , Glutamina/farmacologia , Absorção Intestinal/efeitos dos fármacos , Dourada/metabolismo , Animais , Arginina/metabolismo , Dieta , Glucose/metabolismo , Glutamina/metabolismo , Glutationa/metabolismo , Intestinos/efeitos dos fármacos , Intestinos/enzimologia , Peroxidação de Lipídeos/efeitos dos fármacos , Fígado/efeitos dos fármacos , Fígado/metabolismo , Estresse Oxidativo/efeitos dos fármacos , Dourada/crescimento & desenvolvimentoRESUMO
During winter, low temperatures induce a direct metabolic depression in gilthead sea bream, without any significant compensatory effect below 13 degrees C. The present study therefore focused on how to improve response to cold in these fish, looking specifically at the two factors of diet (high energy, HiE, and low energy, LoE) and activity (normal, -SW, and sustained activity, +SW) prior to exposure to cold. Following a preparatory period of 75 days water was adjusted to 10 degrees C and kept for 40 days. Enzymatic activities and store deposition revealed that the HiE-SW group had acquired an energy surplus whilst the LoE+SW group exhibited an energy deficit. Liver enzyme activities evidenced diet dependence: LoE groups showed greater glucose-6-phosphate dehydrogenase activity and HiE groups showed greater lipoprotein lipase and hepatic lipase activities. Moreover, the HiE-SW group's lower citrate synthase/cytochrome-c-oxidase ratio reflected the energy surplus available. Perivisceral fat mobilisation caused by cold stress affected liver integrity, resulting in a pre-steatotic condition for the HE-SW group. The differences in liver enzyme activities produced by pre-cold conditions disappeared at low temperatures and enzymatic activities did not compensate. Therefore any improvement that would enable gilthead sea bream to face up to winter must be achieved prior to the appearance of low temperatures.
Assuntos
Aclimatação/fisiologia , Temperatura Baixa , Metabolismo Energético/fisiologia , Dourada/fisiologia , Animais , Dieta , Lipídeos/sangue , Fígado/citologia , Fígado/enzimologia , Dourada/sangue , Natação/fisiologiaRESUMO
We present 2 cases of systemic cryptococcosis with cutaneous involvement in patients with acquired immunodeficiency syndrome (AIDS). Both patients were male, intravenous drug abusers, 31 and 35 years old, with severe immunodepression and a CD4+ lymphocyte count of 10/ml and 1/ml, respectively. They both had papular lesions reminiscent of molluscum contagiosum and in one patient with concomitant systemic leishmaniasis, there were spores of Cryptococcus neoformans coexisting with the leishmanias in the cutaneous lesions, constituting the first reported case of this particular association. Both patients responded well to amphotericin B followed by fluconazole.
Assuntos
Infecções Oportunistas Relacionadas com a AIDS/microbiologia , Criptococose/complicações , Dermatopatias/complicações , Adulto , Criptococose/tratamento farmacológico , Criptococose/patologia , Cryptococcus neoformans , Humanos , Masculino , Dermatopatias/tratamento farmacológico , Dermatopatias/patologiaRESUMO
We report an 82-year-old man who presented with a tumor which had developed over the previous year on the right nasal ala of a rhinophyma. Histopathological, immunohistochemical, and electron microscopic study confirmed the diagnosis of angiosarcoma on the head and neck. He was treated with radiotherapy of the tumor and cervical adenopathy, which developed later. The possible etiological and pathogenetic role of lymphedema due to inflammatory flares of rosacea on the nose is discussed, together with the histological and immunohistochemical data leading to the diagnosis of this tumor.
Assuntos
Hemangiossarcoma/etiologia , Neoplasias Nasais/etiologia , Rinofima/complicações , Idoso , Idoso de 80 Anos ou mais , Biópsia por Agulha , Diagnóstico Diferencial , Seguimentos , Hemangiossarcoma/diagnóstico , Hemangiossarcoma/radioterapia , Humanos , Imuno-Histoquímica , Masculino , Neoplasias Nasais/diagnóstico , Neoplasias Nasais/radioterapia , Rinofima/diagnósticoRESUMO
BACKGROUND: Although radiofrequency ablation (RFA) is promising for the local treatment of breast cancer, burns are a frequent complication. The safety and efficacy of a new technique with a bipolar RFA electrode was evaluated. METHODS: Dosimetry was assessed ex vivo in bovine mammary tissue, applying power settings of 5-15 W with 10-20 min exposure and 3.0-12.0 kJ to a 20-mm active length bipolar internally cooled needle-electrode. Subsequently, in 15 women with invasive breast carcinoma ≤2.0 cm diameter ultrasound-guided RFA was performed followed by immediate resection. RESULTS: An ablation zone of 2.5 cm was reached in the ex vivo experiments at 15 W at 9.0 kJ administered energy. Histopathology revealed complete cell death in 10 of 13 patients (77%); in 3 patients partial ablation was due to inaccurate probe positioning. In 1 patient a pneumothorax was caused by the probe placement, treated conservatively. No burns occurred. CONCLUSIONS: Ultrasound-guided RFA with a bipolar needle-electrode appears to be a safe local treatment technique for invasive breast cancer up to 2 cm. Ways to improve placement of the probe and direct monitoring of the ablation-effect should be the aim of further research.
Assuntos
Neoplasias da Mama/cirurgia , Queimaduras/prevenção & controle , Carcinoma Ductal de Mama/cirurgia , Ablação por Cateter/métodos , Idoso , Animais , Neoplasias da Mama/patologia , Carcinoma Ductal de Mama/patologia , Ablação por Cateter/instrumentação , Bovinos , Eletrodos , Desenho de Equipamento , Feminino , Humanos , Glândulas Mamárias Animais , Pessoa de Meia-IdadeRESUMO
BACKGROUND: We evaluated whether performing contrast-enhanced breast MRI in addition to mammography and/or ultrasound in patients with nonpalpable suspicious breast lesions improves breast cancer management. METHODS: The MONET - study (MR mammography of nonpalpable breast tumours) is a randomised controlled trial in patients with a nonpalpable BIRADS 3-5 lesion. Patients were randomly assigned to receive routine medical care, including mammography, ultrasound and lesion sampling by large core needle biopsy or additional MRI preceding biopsy. Patients with cancer were referred for surgery. Primary end-point was the rate of additional surgical procedures (re-excisions and conversion to mastectomy) in patients with a nonpalpable breast cancer. FINDINGS: Four hundred and eighteen patients were randomised, 207 patients were allocated to MRI, and 211 patients to the control group. In the MRI group 74 patients had 83 malignant lesions, compared to 75 patients with 80 malignant lesions in the control group. The primary breast conserving surgery (BCS) rate was similar in both groups; 68% in the MRI group versus 66% in the control group. The number of re-excisions performed because of positive resection margins after primary BCS was increased in the MRI group; 18/53 (34%) patients in the MRI group versus 6/50 (12%) in the control group (p=0.008). The number of conversions to mastectomy did not differ significantly between groups. Overall, the rate of an additional surgical intervention (BCS and mastectomy combined) after initial breast conserving surgery was 24/53 (45%) in the MRI group versus 14/50 (28%) in the control group (p=0.069). INTERPRETATION: Addition of MRI to routine clinical care in patients with nonpalpable breast cancer was paradoxically associated with an increased re-excision rate. Breast MRI should not be used routinely for preoperative work-up of patients with nonpalpable breast cancer.
Assuntos
Neoplasias da Mama/cirurgia , Mastectomia Segmentar/métodos , Biópsia por Agulha/métodos , Neoplasias da Mama/patologia , Feminino , Humanos , Imageamento por Ressonância Magnética , Mamografia/métodos , Mastectomia/estatística & dados numéricos , Pessoa de Meia-Idade , Cuidados Pré-Operatórios , Reoperação/estatística & dados numéricosRESUMO
The present study demonstrates the presence of different amino acid carriers in the membrane of trout red cells. Most glycine is taken up through the Na(+)-dependent system ASC, although the nearly specific Gly system is also active. Besides these carriers, glycine is taken up by means of Na(+)-independent transporters, system L being the most important. A system asc of high affinity and low capacity has been found, and band 3 is unable to transport glycine under physiological conditions. These results suggest that although all these carriers are already present in primitive vertebrates, several differences exist in their properties with respect to those found in mammalian cells.
Assuntos
Eritrócitos/metabolismo , Glicina/metabolismo , Truta/metabolismo , Ácido 4-Acetamido-4'-isotiocianatostilbeno-2,2'-dissulfônico/farmacologia , Animais , Transporte Biológico , Células Cultivadas , Membrana Eritrocítica/metabolismo , Membrana Eritrocítica/ultraestrutura , Eritrócitos/ultraestrutura , Sódio/farmacologia , Canais de Sódio/metabolismo , TrítioRESUMO
The present study shows that in trout red blood cells the activity of some amino acid carriers, not directly involved in cell volume regulation, is affected by external osmolality. Glycine uptake has been used as the experimental approach because it was shown previously that it is effected by different carriers, namely the Na+-dependent ASC and Gly systems, as well as the Na+-independent asc and L systems. An increase in the uptake through the Gly system and the two Na+-independent carriers was found, while the ASC system appeared to be downregulated. Those systems whose activities were increased by hypo-osmolality did not share the mechanism by which this increase was obtained. Thus, the Gly system was sensitive to intracellular ionic changes, while the Na+- independent systems were mechanically stimulated, as assessed by the iso-osmotic swelling caused by ammonium chloride. On the other hand, a volume-sensitive transporter may be present in trout red blood cells, which is involved in the swelling-induced glycine movement, as can be deduced from the effect of some inhibitors such as pyridoxal phosphate, DIDS (4,4'-diisothiocyanate-stilbene-2,2'-disulfonic acid) and quinine.
Assuntos
Proteínas de Transporte/fisiologia , Eritrócitos/fisiologia , Glicina/metabolismo , Animais , Calcimicina/farmacologia , Cálcio/metabolismo , Eritrócitos/química , Concentração Osmolar , Pressão Osmótica , Truta/metabolismoRESUMO
Evidence is presented that the high levels of internal L-glutamic and L-aspartic acid in frog (Rana esculenta) red blood cells are due to the existence of a specific carrier for acidic amino acids of high affinity (Km = 3 microM) and low capacity (Vmax 0.4 mumol L-Glu.Kg-1 dry cell mass.10 min-1). It is Na+ dependent and the incorporation of L-glutamic acid can be inhibited by L- and D-aspartate and L-cysteic acid, while D-glutamic does not inhibit. Moreover, this glutamic uptake shows a bell-shaped dependence on the external pH. All these properties show that this carrier belongs to the system X-AG family. Besides the incorporation through this system, L-glutamic acid is also taken up through the ASC system, although, under physiological conditions, this transport is far less important, since it has relatively low affinity (Km 39 microM) but high capacity (Vmax 1.8 mumol L-Glu.Kg-1 dry cell mass.10 min-1).
Assuntos
Proteínas de Transporte/sangue , Eritrócitos/metabolismo , Glicoproteínas/sangue , Rana esculenta/sangue , Sistema X-AG de Transporte de Aminoácidos , Sistemas de Transporte de Aminoácidos , Aminoácidos/sangue , Animais , Ácido Aspártico/sangue , Transporte Biológico , Glutamatos/sangue , Ácido Glutâmico , Ácido gama-Aminobutírico/sangueRESUMO
The uptake of l-leucine by trout red blood cells and peripheral lymphocytes has been analyzed. The present study shows two functionally different Na+-independent systems for apolar branched-chain amino acids. They are designated as L systems because they share some properties with the mammalian L system. The carrier present in red blood cells has low Km values, is trans-stimulable and not stereospecific for leucine uptake; on the other hand, the system present in lymphocytes is stereospecific for leucine uptake and trans-inhibitable. Both carriers are pH sensitive in a similar fashion at low pHs, but there are important differences at higher pH values (above neutrality). These properties are compared with these of the asc systems previously reported in these cells.
Assuntos
Eritrócitos/metabolismo , Leucina/farmacocinética , Linfócitos/metabolismo , Truta/sangue , Animais , Proteínas de Transporte/metabolismo , Concentração de Íons de HidrogênioRESUMO
The Na(+)-independent uptake of L-alanine has been studied in trout red blood cells, isolated hepatocytes and peripheral blood lymphocytes. The present study shows the existence of two functionally different Na(+)-independent systems for short chain neutral amino acids in these cells. They are designated as asc systems because of their resemblance to systems described in other cell types. Besides their independence of sodium and a rough similarity in substrate preference, the most important property shared by the two carriers is a lack of trans-stimulation, allowing further differentiation from system L. One of them is an unusually stereospecific carrier present in red blood cells, the other is less restrictive and present in hepatocytes and peripheral blood lymphocytes. Extracellular acid pH increases the incorporation to red blood cells, while it slightly depresses the uptake in the other cells. From the data presented, it is not possible, at first, to classify these carriers as asc1 or asc2 systems. Moreover, the system present in red cells resembles that found in the nonerythroid cells, BSC-1, while there is no clear parallelism between the system found in hepatocytes/lymphocytes and any of those described previously.
Assuntos
Alanina/metabolismo , Eritrócitos/metabolismo , Fígado/metabolismo , Linfócitos/metabolismo , Sódio/farmacologia , Alanina/farmacologia , Aminoácidos/farmacologia , Animais , Transporte Biológico , Células Cultivadas , Eritrócitos/efeitos dos fármacos , Fígado/efeitos dos fármacos , Linfócitos/efeitos dos fármacos , TrutaRESUMO
Brush border membrane vesicles (BBMV) enriched in sucrase, maltase and alkaline phosphatase, and impoverished in Na(+)-K(+)-ATPase, were isolated from proximal and distal intestine of the gilthead sea bream (Sparus aurata) by a MgCl(2) precipitation method. Vesicles were suitable for the study of the characteristics of D-glucose apical transport. Only one D-glucose carrier was found in vesicles from each intestinal segment. In both cases, the D-glucose transport system was sodium-dependent, phlorizin-sensitive, significantly inhibited by D-glucose, D-galactose, alpha-methyl-D-glucose, 3-O-methyl-D-glucose and 2-deoxy-D-glucose, and showed stereospecificity. Apparent affinity constants of D-glucose transport (K(t)) were 0.24 +/- 0.03 mM in proximal and 0.18 +/- 0.03 mM in distal intestine. Maximal rate of influx (Jmax) was 47.3 +/- 2.2 pmols. mg(-1) protein for proximal and 27.3 +/- 3.6 pmols. mg(-1) protein for distal intestine. Specific phlorizin binding and relative abundance of an anti-SGLT1 reactive protein were significantly higher in proximal than in distal BBMV. These results suggest the presence of the same D-glucose transporter along the intestine, with a higher density in the proximal portion. This transporter is compatible with the sodium-dependent D-glucose carrier described for other fish and with the SGLT1 of higher vertebrates.
Assuntos
Glucose/metabolismo , Intestinos/fisiologia , Microvilosidades/metabolismo , Dourada/fisiologia , ATPase Trocadora de Sódio-Potássio/metabolismo , Sódio/metabolismo , Animais , Transporte Biológico Ativo/fisiologiaRESUMO
Glucose metabolism has been studied in Salmo trutta red blood cells. From non-metabolizable analogue (3-O-methyl glucose and L-glucose) uptake experiments it is concluded that there is no counterpart to the membrane transport system for glucose found in mammalian red blood cells. Once within the cells, glucose is directed to CO2 and lactate formation through both the Embden-Meyerhoff and hexose monophosphate shunts; lactate appears as the most important end-product of glucose metabolism in these cells. From experiments under anaerobic conditions, and in the presence of an inhibitor of pyruvate transfer to mitochondria, most of the CO2 formed appears to derive from the hexose monophosphate pathway. Appreciable O2 consumption has been detected, but there is no clear relationship between this and substrate metabolism. Key enzymes of glucose metabolism, hexokinase, fructose-6-phosphate kinase and, probably, pyruvate kinase are out of equilibrium, confirming their regulatory activity in Salmo trutta red blood cells. The presence of isoproterenol, a catecholamine analogue, induces important changes in glucose metabolism under both aerobic and anaerobic conditions, and increases the production of both CO2 and lactate. From the data presented, glucose appears to be the major fuel for Salmo trutta red blood cells, showing a slightly different pattern of glucose metabolism from rainbow trout red blood cells.
Assuntos
Glicemia/metabolismo , Eritrócitos/metabolismo , Truta/sangue , 3-O-Metilglucose , Anaerobiose , Animais , Transporte Biológico Ativo/efeitos dos fármacos , Eritrócitos/efeitos dos fármacos , Técnicas In Vitro , Isoproterenol/farmacologia , Cinética , Metilglucosídeos/sangueRESUMO
L-Alanine uptake has been studied in frog red blood cells. The present study shows the presence of different carriers for this amino acid in these cells. In the physiological concentration range, most L-alanine is taken up through the Na(+)-dependent system ASC, although the sodium-independent systems asc and L are also active. The competitive inhibition data obtained makes difficult to differentiate the two Na(+)-independent activities in a clear contrast with data from fish or mammalian erythrocytes, indicating that despite its widespread occurrence in vertebrates, these carriers show characteristics that are species specific.
Assuntos
Alanina/sangue , Aminoácidos Cíclicos , Eritrócitos/metabolismo , Rana esculenta/sangue , Aminoácidos/farmacologia , Animais , Ligação Competitiva , Transporte Biológico Ativo/efeitos dos fármacos , Cisteína/farmacologia , Eritrócitos/efeitos dos fármacos , Feminino , Peixes , Técnicas In Vitro , Cinética , Masculino , Mamíferos , Sódio/sangueRESUMO
The present study shows that the capacity of trout (Salmo trutta) red blood cells (RBCs) and freshly isolated hepatocytes to take up short-chain neutral amino acids changes according to a seasonal pattern. Maximal amino acid uptake rates in RBCs were obtained in winter and spring, while minima were seen in summer and autumn. In contrast, the maximal rates for the freshly isolated hepatocytes were obtained in autumn and winter, and the minima were seen in spring and summer. In addition, by studying the uptake of glycine, evidence was found that the activities of the amino acids carriers ASC, asc and Gly in RBCs varied according to a seasonal rhythm. The activity of the ASC and asc systems changed in parallel with the global uptake of amino acids. Moreover, the RBC:plasma concentration ratio for certain substrates of these carriers (alanine, serine and glycine) varied accordingly. In contrast, the activity of the Gly system was modified inversely with respect to the overall amino acid uptake. The activity of the ASC system in freshly isolated hepatocytes was also seasonally modified, reaching a maximum in autumn, shortly before the reproductive period.
Assuntos
Aminoácidos/metabolismo , Eritrócitos/fisiologia , Fígado/fisiologia , Truta/fisiologia , Animais , Estações do AnoRESUMO
Bacillary angiomatosis is an unusual systemic vascular proliferation seen predominantly in patients with the acquired immunodeficiency syndrome. These vascular lesions are due to infection with a Bartonella species, most commonly B. henselae, but sometimes B. quintana. It is treatable and often curable, but without therapy may be life-threatening. Clinically, the disorder often resembles several different vascular disorders, particularly pyogenic granuloma and Kaposi's sarcoma. We now report a clinically typical patient with bacillary angiomatosis who was HIV seronegative, but who had idiopathic thrombocytopenic purpura, was status-post splenectomy and to whom long-term systemic prednisone had been administered.
Assuntos
Angiomatose Bacilar/diagnóstico , Glucocorticoides/efeitos adversos , Soronegatividade para HIV , Prednisolona/efeitos adversos , Púrpura Trombocitopênica Idiopática/complicações , Adulto , Angiomatose Bacilar/tratamento farmacológico , Angiomatose Bacilar/patologia , Antibacterianos/uso terapêutico , Azitromicina/uso terapêutico , Glucocorticoides/uso terapêutico , Humanos , Masculino , Prednisolona/uso terapêutico , Púrpura Trombocitopênica Idiopática/tratamento farmacológico , Púrpura Trombocitopênica Idiopática/patologia , Pele/patologiaRESUMO
BACKGROUND: The development of squamous cell carcinomas is the main cause of death of patients with Dystrophic epidermolysis bullosa. We think it is of interest to know their clinical characteristics and the treatment difficulties they cause. METHODS: We followed the clinical evolutions and carried out histopathological studies of eight primary cutaneous squamous cell carcinomas on three males and one female with recessive dystrophic epidermolysis bullosa. Patient ages ranged from 16 to 34 years (mean 27). RESULTS: Two patients had two tumours and one three, all were in characteristically scarred skin; four on upper limbs; four on lower. Maximum tumour dimensions ranged from 2 to 28 cm (mean 13), and represented 6 or more months of evolution. In three cases the affected limb must be amputated. Five tumours were well differentiated, three were moderately differentiated. CONCLUSIONS: The patients consulted too late and their large lesions needed aggressive treatment. Appropriate information and regular examination of patients with RDEB helps early diagnosis of tumours and may avoid disabling operations.