RESUMO
PURPOSE: Ventilator-induced diaphragm dysfunction or damage (VIDD) is highly prevalent in patients under mechanical ventilation (MV), but its analysis is limited by the difficulty of obtaining histological samples. In this study we compared diaphragm histological characteristics in Maastricht III (MSIII) and brain-dead (BD) organ donors and in control subjects undergoing thoracic surgery (CTL) after a period of either controlled or spontaneous MV (CMV or SMV). METHODS: In this prospective study, biopsies were obtained from diaphragm and quadriceps. Demographic variables, comorbidities, severity on admission, treatment, and ventilatory variables were evaluated. Immunohistochemical analysis (fiber size and type percentages) and quantification of abnormal fibers (a surrogate of muscle damage) were performed. RESULTS: Muscle samples were obtained from 35 patients. MSIII (n = 16) had more hours on MV (either CMV or SMV) than BD (n = 14) and also spent more hours and a greater percentage of time with diaphragm stimuli (time in assisted and spontaneous modalities). Cross-sectional area (CSA) was significantly reduced in the diaphragm and quadriceps in both groups in comparison with CTL (n = 5). Quadriceps CSA was significantly decreased in MSIII compared to BD but there were no differences in the diaphragm CSA between the two groups. Those MSIII who spent 100 h or more without diaphragm stimuli presented reduced diaphragm CSA without changes in their quadriceps CSA. The proportion of internal nuclei in MSIII diaphragms tended to be higher than in BD diaphragms, and their proportion of lipofuscin deposits tended to be lower, though there were no differences in the quadriceps fiber evaluation. CONCLUSIONS: This study provides the first evidence in humans regarding the effects of different modes of MV (controlled, assisted, and spontaneous) on diaphragm myofiber damage, and shows that diaphragm inactivity during mechanical ventilation is associated with the development of VIDD.
Assuntos
Diafragma/patologia , Respiração Artificial/efeitos adversos , Respiração Artificial/métodos , Adulto , Idoso , Idoso de 80 Anos ou mais , Biópsia/métodos , Diafragma/anormalidades , Diafragma/fisiopatologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Músculo Quadríceps/anormalidades , Músculo Quadríceps/patologia , Músculo Quadríceps/fisiopatologiaRESUMO
BACKGROUND: Abdominal fat (both visceral and subcutaneous) accumulation is associated with an increased risk of developing insulin resistance. The latter stands as the basis upon which diabetes, hypertension, and atherogenic dyslipidemia tend to build up. Hence, abdominal liposuction (AL) could theoretically hold metabolic benefits. We undertook the present study to assess the effects of AL on carbohydrate and lipid metabolism. METHODS: This is a prospective study including 20 healthy volunteers (M2/F18) aged 39.6 +/- 7.7 years old (24-52), body mass index (BMI) = 25.3 +/- 4.7 kg/m(2) (19.8-36) who underwent AL. Before and 4 months after AL, we measured glucose and insulin concentrations, HOMA index [glucose (mM) x IRI (microUI/l)/22.5], free fatty acids (FFA), glycerol, total cholesterol and triglycerides, high-density lipoprotein (HDL)-cholesterol (HDL-c), low-density lipoprotein (LDL)-cholesterol (LDL-c), very low-density lipoprotein (VLDL)-cholesterol (VLDL-c) and apolipoproteins (apo) B, AI and AII, adiponectin (Adp), and ultra-sensitive C-reactive protein (CRP). RESULTS: Lipo-aspirate averaged 5.494 +/- 5.297 cc (600-19.000). Weight, BMI, and waist circumference decreased significantly 4 months after surgery by 4.6, 4.6 and 5.9%, respectively. There were significant decrements in FFA (-35%, p < 0.0001), glycerol (-63%, p < 0.0005), VLDL-c (-15.2%; p < 0.001), and triglycerides (-21.3%, p < 0.002), an increase in HDL-c (+10%, p < 0.03), Apo AI (+10.1%, p < 0.02), and Apo AII (+11.8%, p < 0.001). Total cholesterol, LDL-c, ApoB, and the LDL-c/ApoB ratio raised by +15% (p < 0.0005), +27.3% (p < 0.000), +15.1% (p < 0.008) and +2.76% (p < 0.008), respectively. Glucose, insulin, the HOMA index, Adp, and CRP were not significantly altered after AL. CONCLUSION: AL in healthy normal weight or slightly overweight subjects improves the major lipoprotein components of obesity-associated dyslipidemia. This improvement occurs independent of insulin sensitivity.
Assuntos
Resistência à Insulina/fisiologia , Lipectomia , Metabolismo dos Lipídeos/fisiologia , Sobrepeso/metabolismo , Sobrepeso/cirurgia , Gordura Subcutânea Abdominal/cirurgia , Adiponectina/sangue , Adulto , Glicemia/metabolismo , Proteína C-Reativa/metabolismo , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Estudos ProspectivosRESUMO
UNLABELLED: The recurrent chronic abdominal pain (RAP) is one of the most usual pathologies in pediatrics. It may appear in the form of periodical or continuous sharp crisis. In order to be accepted as chronic, the presence of this pain must last at least 3 months. In many of the reference publications, only between 5 and 1% of the cases are considered to have an organic ethiology and the rest are labelled as functional processes of psychological origin. The reason for our communication is to show our experience on 304 RAP patients from 1995 to 2001. We also want to highlight the usefulness of laparoscopy in order to diagnose and treat 14.45% (44) of cases of this group of patients. MATERIAL AND METHODOLOGY: 304 patients between 5 and 14 years old were studied from 1995 to 2001. We followed a protocol of: medical and personal medical record, specific anamnesis for RAP, detailed description of the diet and intestinal habits, physical exploration. Laboratory: 1) blood analysis, hepatic profile, glucose, cholesterol, creatinine, amylase, 2) urine sediment and may be urine culture, 3) faeces analysis and 4) vaginal flow analysis. Image diagnosis is: simple abdomen radiography, ultrasonography and intestinal Rx. Guided and specific determinations: spired urea test, gastric chemism, lactose test, gastroscopy and biopsy, colonoscopy and biopsy, laparoscopy. RESULTS: In short, we can say: 74 patients (23.4%) come with inappropiate feeding diets, 31 (10.1%) with helicobacter pilori, 20 (6.5%) with adenoids, pharynx and pharyngeal processes, the same figure from gynaecologic origin, 18 (5.9%) from psychological origin, and in 44 cases (14.45%) laparoscopy was indicated. CONCLUSIONS: The anatomopathological study in the cases in which we carried out laparoscopic appendicectomy does not allow us to accept the symptoms of chronic appendicitis. The exploratory laparoscopy and the appendectomy have meant benefits for the solution to this pathology in 14.45% of patients. It is essential to carry out a long-term follow-up, at least 12 months, to be sure RAP is cured. We must carry on looking for aetiological cause of RAP.
Assuntos
Dor Abdominal/etiologia , Apendicectomia/métodos , Apendicite/complicações , Apendicite/cirurgia , Laparoscopia/métodos , Adolescente , Criança , Pré-Escolar , Doença Crônica , Feminino , Humanos , MasculinoRESUMO
INTRODUCTION: The disease of Von Hippel Lindau (VHL) is hereditary and causes a predisposition to the development of tumours. Organs such as the cerebellum, the pancreas, the kidney, the suprarenal glands and the retina are more usually affected by this disease. CLINICAL CASE: We present the case of a 5-year-old patient who suffers from asiymptomatic high blood pressure. In the family antecedents, it is relevant the case of the father, with pheocromocytoma bilateral, which led us to carry out a genetic study of his two sons. Our patient, the younger; presented a mutation of the VHL gene in the short arm of the chromosome 3. In one of the periodic controls, it could be detected high blood pressure of 160/100 mm. Hg, clinically asymptomatic. The other child did not present a genetic mutation and has no disease. The presence of high catecholamines, the detection of a 3 cm left suprarenal mass through the ecography, the TAC that did not show a right suprarenal pathology and the MBIG scintigraphy confirmed the diagnostic of pheocromocytoma. The RNM showed another 0.8-cm mass which confirmed a pheocromocytoma bilateral. We started the treatment against high blood pressure with fenoxibenzamine and diltiazem, and we controlled this problem. We also prepared the pre-and-post operation anesthetic strategy, which is so important for the surgical success. The operation started by a laparoscopic, we made left adrenalectomy and we had to reconvert to laparotomy to make partial right adrenalectomy. Six months after the operation, the patient is free from symptomatology and follows a treatment with glucocorticoides with smaller and smaller doses. COMMENTS: The case is exceptional because it embodies the following characteristics: early diagnostic age, family affectation and discovery of asymptomatic high blood pressure. It needed an appropriate preanesthetic and anesthetic preparation, which gave way to an operation without complications. The postoperation was also stable and presented no complications.
Assuntos
Neoplasias das Glândulas Suprarrenais/cirurgia , Neoplasias Primárias Múltiplas/cirurgia , Feocromocitoma/cirurgia , Doença de von Hippel-Lindau/complicações , Neoplasias das Glândulas Suprarrenais/complicações , Pré-Escolar , Humanos , Masculino , Neoplasias Primárias Múltiplas/complicações , Feocromocitoma/complicaçõesRESUMO
The myothonic dystrophy or Steinert's disease is a congenital, autosomal, dominant disorder which seriously affects the striated muscle and also to a certain extent, several organs and systems and on rare occasions, the intestinal smooth muscle. In the case, we treated a four years old girl in whom Steinert's disease was diagnosed when she was born and who developed the characteristics of severe constipation after a few months of life. The ano-rectal manometry showed a paradoxical reaction of external Sphincter with a normal inhibitory reflex. The rectal biopsy revelated a miophathy which affected the muscularis propia with the normal neuronal innervation. The X-ray studies showed that motility disorder was stopped in the distal area of the left colon. A colostomy in the healthy zone worked extremely well. Six months later, the normal colon was brought down retro-rectal. Only a partial incontinence remained in the external sphincter caused by Steinert's disease. The interest of this case lies in the pathological association, striade muscle and smooth muscle, in such an early age of life. It is a new contribution to the complex chapter of the intestinal pseudo-obstruction.
Assuntos
Pseudo-Obstrução Intestinal/complicações , Pseudo-Obstrução Intestinal/cirurgia , Distrofia Miotônica/complicações , Pré-Escolar , Feminino , Humanos , Músculo Liso/patologiaRESUMO
INTRODUCTION: Between the external genital exploration and the internal genital direct vision, through laparoscopia, there is a black point which is the pelvic floor; we have to know whether there is a vagina or not, what it is like and where it arrives at. The reason for this research is to present our experience with the transrectal ecography, which allows us a very good exploration of the pelvic floor. MATERIAL AND METHODS: We present 6 patients, the youngest is 16 months old and the oldest is 19 years old, who have a diagnostic of 3 congenital adrenal hyperplasia (HSC), 2 gonadal dysgenesis and 1 vaginal agenesis (S. Rokitanski). Under sedation, we carried out a transrectal ecography with Aloka SSD650 ecograph and 7.5 MHz vaginal scanner. Ultrasone. RESULTS: In case of HSC (16 months old), the transrectal ecography showed the vaginal arrival at the urethra and we were able to measure the distance from the external sphincter. In the other two HSC, which had been surgically corrected, the transrectal ecography clearly showed the vagina (length and calibre). In the two gonadal dysgenesis (two 11-and-19-year-old sisters, the first of whom had undergone vulvo-vaginoplasty, we appreciated the length of the vagina and, in the case of the sister with a relatively normal vagina, we confirmed the presence of the vagina connected to the uterus. In the vaginal agenesis, in which a neovagina with amnion membrane had been carried out, which, in its turn, ended in a situation of hematometra due to a stenosis, the transrectal ecography was really helpful to obtain vaginal dilatations. CONCLUSIONS: Ecography is an easily available technique and provides both through and detailed information of the genital structures going through the pelvic floor, a zone which is otherwise difficult to explore. It was done under sedation for the patient's age and idiosyncrasy. The experience has just started but we are sure that in the future it will replace the genitograma.
Assuntos
Transtornos do Desenvolvimento Sexual/diagnóstico por imagem , Endossonografia , Diafragma da Pelve/diagnóstico por imagem , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Reto , Vagina/anormalidades , Vagina/diagnóstico por imagemRESUMO
We evaluated an immunochromatographic assay detecting pneumococcal antigen in urine samples from children diagnosed with pneumococcal pneumonia. The sensitivity and specificity of the immunochromatographic test with nonconcentrated urine (NCU) were 86.7 and 62.9%, respectively; with concentrated urine (CU), they were 100 and 11.7%, respectively. Pneumococcal antigen was also detected in 42.5% of NCU and 87.1% of CU samples from nasopharyngeal carriers. This is a nonspecific test for the diagnosis of pneumococcal pneumonia in children, particularly the very young.
Assuntos
Antígenos de Bactérias/urina , Imunoensaio/métodos , Pneumonia Pneumocócica/diagnóstico , Streptococcus pneumoniae/imunologia , Adolescente , Portador Sadio/diagnóstico , Portador Sadio/microbiologia , Criança , Pré-Escolar , Cromatografia/métodos , Cromatografia/estatística & dados numéricos , Feminino , Humanos , Imunoensaio/estatística & dados numéricos , Lactente , Masculino , Nasofaringe/microbiologia , Pneumonia Pneumocócica/microbiologia , Sensibilidade e Especificidade , Streptococcus pneumoniae/isolamento & purificaçãoRESUMO
STUDY DESIGN: A prospective evaluation of the smoking habits of new spine patients was performed during a 4-year interval. OBJECTIVE: To assess what effect the spine health care provider has on a patient's ongoing nicotine addiction. SUMMARY OF BACKGROUND DATA: The negative effects of smoking and nicotine for the spine patient are well defined. METHODS: Spine patients (N = 10,901) were queried as to their smoking habits during their new patient consultation; 3041 current smokers were identified. Data were obtained during 20,835 follow-up visits. Some of the smokers (n = 1632) were seen in follow-up visits and evaluated for their cigarette usage. Two different approaches were applied to assist the patient to stop smoking: 1) chart identification, reinforcement at each visit, continued education, written handout, practitioner assigning high priority, and 2) occasional mentioning, lower priority. RESULTS: The number of smokers who quit smoking was 35.6% in the high priority group versus 19.5% in the lower priority group. There was also a difference in the number of patients who decreased their smoking (67% vs. 38%). The fear that confronting a patient who smoked would cause the patient to leave the practice was not realized. Other factors that predicted successful cessation were fewer packs per day, fewer years smoking, and older age. The effect of the practitioner was independent of these other variables as determined by a logistic regression analysis. CONCLUSION: Patients will respond better if the practitioner assigns a priority and works with the patient to educate them about ways to address this particular substance abuse. Just asking about the patient's smoking status had a dramatic effect on smoking status. All spine practitioners should do all in their power to help their patients overcome their nicotine addiction.
Assuntos
Ortopedia/tendências , Educação de Pacientes como Assunto/tendências , Relações Médico-Paciente , Abandono do Hábito de Fumar/psicologia , Doenças da Coluna Vertebral/complicações , Humanos , Ortopedia/estatística & dados numéricos , Aceitação pelo Paciente de Cuidados de Saúde , Educação de Pacientes como Assunto/normas , Educação de Pacientes como Assunto/estatística & dados numéricos , Estudos Prospectivos , Abandono do Hábito de Fumar/métodos , Abandono do Hábito de Fumar/estatística & dados numéricos , Doenças da Coluna Vertebral/psicologiaRESUMO
Radio-guided surgery is a new technique which can provide benefits for pediatric oncology, as in our patient with neuroblastoma in stage IV, that after a chemotherapy, surgical, radiotherapy and autologous bone marrow transplant treatment kept showing, at 2 years, residual tumoral fragments and increase of catecholamines. Radio-guided surgery allowed an easy and exact location. This technique decreases surgery time and let us find residual tumoral tissue no matter how small. With radio-guided surgery we can obtain higher survival and even cure the patient.
Assuntos
3-Iodobenzilguanidina , Neoplasias Abdominais/diagnóstico por imagem , Neoplasias Abdominais/cirurgia , Neuroblastoma/diagnóstico por imagem , Neuroblastoma/cirurgia , Compostos Radiofarmacêuticos , Neoplasias Abdominais/sangue , Catecolaminas/sangue , Pré-Escolar , Feminino , Seguimentos , Humanos , Neuroblastoma/sangue , Cintilografia , Fatores de TempoRESUMO
OBJECTIVE: Apolipoprotein(B) [apo(B)] reflects the total mass of atherogenic particles (VLDL, IDL, and LDL), and its increase is associated with cardiovascular disease independently of LDL cholesterol (LDLc) levels. Apo(B) determination has been recently standardized, but attention to regional reference limits is advisable. Our aim was to analyze the frequency of dyslipidemic phenotypes, including those dependent on increased apo(B) in normocholesterolemic type 2 diabetic patients. RESEARCH DESIGN AND METHODS: A total of 100 consecutively seen type 2 diabetic patients (63 men, 37 women; aged 59 +/- 11 years) were included, after excluding those on lipid-lowering therapy. Apo(B) cutoff (1.1 g/l) was obtained from a group of normolipidemic (47 men, 21 women) control subjects, and LDLc, triglycerides, and HDL cholesterol (HDLc) cutoff points were those from the National Cholesterol Education Program guidelines. LDLc levels were obtained by ultracentrifugation if triglyceride levels were > 3.45 mmol/l; otherwise, they were calculated (Friedwald). Apo(B) levels were measured by immunoturbidimetry. RESULTS: Normocholesterolemia (LDLc < 4.13 mmol/l) appeared in 75 of the 100 patients, of whom 55 were normo- and 20 hypertriglyceridemic. Hyperapolipoprotein(B) [hyperapo(B)] was the most frequent lipid disorder, present in 34 (45%) of the normocholesterolemic patients (22 normo- and 12 hypertriglyceridemic). Low HDLc levels were more prevalent (53%) in patients with hyperapo(B) than in the rest (24%). CONCLUSIONS: Hyperapo(B) was found in almost half of the normocholesterolemic type 2 diabetic patients and was frequently associated with low HDLc levels and hypertriglyceridemia. Thus, given its independent association with cardiovascular disease and that it identifies high-risk phenotypes in normocholesterolemic diabetic patients apo(B) should be used to evaluate the lipidic pattern of these patients.
Assuntos
Apolipoproteínas B/sangue , Doenças Cardiovasculares/epidemiologia , Colesterol/sangue , Diabetes Mellitus Tipo 2/genética , Hiperlipidemias/genética , Apolipoproteínas B/genética , HDL-Colesterol/sangue , LDL-Colesterol/sangue , VLDL-Colesterol/sangue , Diabetes Mellitus Tipo 2/sangue , Diabetes Mellitus Tipo 2/complicações , Angiopatias Diabéticas/epidemiologia , Feminino , Humanos , Hiperlipidemias/epidemiologia , Masculino , Pessoa de Meia-Idade , Fenótipo , Valores de Referência , Fatores de RiscoRESUMO
OBJECTIVE: To determine the influence of glycemic control improvement with intensive therapy on lipoprotein(a) [Lp(a)] concentrations in type 1 diabetic patients. RESEARCH DESIGN AND METHODS: A total of 105 poorly controlled type 1 diabetic patients (60 men, 45 women) without diabetic complications participated in a longitudinal study performed in a tertiary referral center, to compare lipid, lipoprotein, and Lp(a) levels before and after 3 months of intensive therapy with multiple insulin doses. Lp(a) levels were measured by the Terumo method. Differences between the two periods were assessed by the paired t test and Wilcoxon's test. RESULTS: After 3 months of intensive therapy, all patients exhibited improved glycemic control. HbA1c decreased from 8.9 +/- 2.4 to 6.5 +/- 1.6% (P < 0.0001), being < or =6% in 47% of patients. However, although a more favorable lipoprotein profile was obtained, no changes in Lp(a) concentrations were observed in the whole group of patients (16.7 +/- 17.3 vs. 17.2 +/- 17.7 mg/dl) or in patients with baseline Lp(a) levels above 30 mg/dl (47.1 +/- 14.8 vs. 47.4 +/- 18.9 mg/dl) or below 30 mg/dl (9.6 +/- 7.3 vs. 10.2 +/- 6.7 mg/dl). In addition, patients reaching HbA1c < or =6 or >6% presented similar Lp(a) levels (19.7 +/- 18.0 vs. 15.0 +/- 17.4 mg/dl), and changes in Lp(a) did not correlate with those observed in HbA1c. CONCLUSIONS: These data demonstrate that the improvement of glycemic control does not influence plasma Lp(a) concentrations in type 1 diabetic patients independently of baseline Lp(a) levels and the degree of glycemic control.
Assuntos
Glicemia/metabolismo , Diabetes Mellitus Tipo 1/sangue , Lipoproteína(a)/sangue , Adulto , Colesterol/sangue , Diabetes Mellitus Tipo 1/tratamento farmacológico , Esquema de Medicação , Feminino , Frutosamina/sangue , Hemoglobinas Glicadas/metabolismo , Humanos , Hipoglicemiantes/administração & dosagem , Hipoglicemiantes/uso terapêutico , Insulina/administração & dosagem , Insulina/uso terapêutico , Estudos Longitudinais , Masculino , Triglicerídeos/sangueRESUMO
Nowadays, the implantation of coronary endoprosthesis within the left main coronary artery is not considered as an absolute contraindication. Here, we show a case of acute occlusion within the left main coronary artery. This was resolved by implanting a stent during a programmed cardiac catheterization. It should be stressed that this problem was occurred without manipulating the left coronary tree. In addition, the patient was in cardiac arrest when the stent was implanted. Cardiopulmonary resuscitation was applied because of this condition.
Assuntos
Angioplastia Coronária com Balão , Vasos Coronários , Isquemia Miocárdica/terapia , Stents , Cateterismo Cardíaco , Angiografia Coronária , Ecocardiografia , Emergências , Humanos , Masculino , Pessoa de Meia-Idade , Isquemia Miocárdica/diagnósticoRESUMO
OBJECTIVE: To evaluate the effect of glycemic control improvement with insulin therapy on lipoprotein(a) [Lp(a)] levels in patients with NIDDM. RESEARCH DESIGN AND METHODS: We performed a longitudinal study in a tertiary referral center to compare lipid and Lp(a) levels before and after 3 months of insulin therapy in 60 poorly controlled NIDDM patients (32 men, 28 women). Patients previously treated with oral hypoglycemic agents (n = 50) received one to two insulin doses, and those previously treated with insulin (n = 10) received multiple insulin doses. Lp(a) levels were measured by the Terumo method. Differences between the two periods were assessed by the paired t test and Wilcoxon's test. RESULTS: After 3 months of insulin therapy, HbA1c decreased from 9.6 +/- 1.9 to 6.0 +/- 1.4% (P < 0.0005) in all patients and from 9.1 +/- 2.1 to 6.1 +/- 2.9% (P < 0.05) in patients under multiple insulin doses, being < or = 6.0% in 59% of patients. Total triglyceride and VLDL cholesterol levels decreased (P < 0.01) and HDL cholesterol increased significantly (P < 0.0005). However, no changes in Lp(a) levels were observed in all patients (25.3 +/- 25.0 vs 25.7 +/- 27.2% mg/dl) and in patients with baseline Lp(a) levels above (63.5 +/- 15.5 vs. 65.1 +/- 23.1 mg/dl) or below 30 mg/dl (11.5 +/- 7.5 vs. 11.5 +/- 7.3 mg/dl). In addition, patients reaching HbA1c levels < or = 6.0% or > 6.0% presented similar Lp(a) levels (26.0 +/- 29.1 vs 25.3 +/- 25.0 mg/dl). Moreover, no correlations were observed between changes in Lp(a) levels and in the glycemic control parameters. CONCLUSIONS: This study shows that the improvement of glycemic control by insulin therapy does not influence plasma Lp(a) levels, measured by the Terumo method, in NIDDM patients, independently of baseline values and the degree of glycemic control reached.
Assuntos
Diabetes Mellitus Tipo 2/tratamento farmacológico , Hipoglicemiantes/uso terapêutico , Insulina/uso terapêutico , Lipoproteína(a)/sangue , Idoso , Diabetes Mellitus Tipo 2/sangue , Diabetes Mellitus Tipo 2/metabolismo , Feminino , Hemoglobinas Glicadas/análise , Hemoglobinas Glicadas/efeitos dos fármacos , Hemoglobinas Glicadas/metabolismo , Humanos , Lipídeos/sangue , Lipoproteína(a)/efeitos dos fármacos , Lipoproteína(a)/metabolismo , Estudos Longitudinais , Masculino , Pessoa de Meia-Idade , Fatores de TempoRESUMO
The Mixed Gonadal Dysgenesis represents the 7.6% of all our patients with intersexual states. We report 14 patients who present Mixed Gonadal Dysgenesis. We have studied: diagnosis age; external genitalia description; sex assigned in birth and if has changed; the karyotype; sex chromatine; hormonal study; genitography; internal genitalia and internal Mullerians ducts structures; gonadal histologycal study; surgical treatment and hormonal treatment. The results show that 50% of the cases presents a 46XY karyotype and the other 50% mosaicisme 45XO/46XY. The histological study is very distinctive. A vulvovagynoplasty and clitoroplasty was made in all the cases. Four patients must follow an hormonal treatment after reaching puberal age. Summing up, with patients having ambiguous genitalia we can suspect it consists of a Mixed Gonadal Dysgenesis. The diagnosis must be precocious. And this diagnosis will be based in an ambiguous genitalia, with a karyotype 46XY or 45XO/46XY, the persistence of the internal Müllerian duct structures, and the histological study with a dysgenetic testis. These patients should be raised as females because they can obtain a good morphological and functional development like a normal female.
Assuntos
Disgenesia Gonadal/tratamento farmacológico , Disgenesia Gonadal/cirurgia , Hormônios Esteroides Gonadais/uso terapêutico , Pré-Escolar , Terapia Combinada , Feminino , Identidade de Gênero , Genitália/cirurgia , Gonadoblastoma/patologia , Gonadoblastoma/cirurgia , Humanos , Lactente , CariotipagemAssuntos
Neoplasias das Glândulas Suprarrenais/diagnóstico por imagem , Doenças Fetais/diagnóstico por imagem , Neuroblastoma/diagnóstico por imagem , Ultrassonografia Pré-Natal , Neoplasias das Glândulas Suprarrenais/patologia , Neoplasias das Glândulas Suprarrenais/cirurgia , Feminino , Humanos , Recém-Nascido , Neuroblastoma/patologia , Neuroblastoma/cirurgia , GravidezRESUMO
14 boys with pubertal gynecomastia, aged 9 to 16 years, were presented. Physical assessment, including pubertal maturation, height, weight, bone age, pubic hair stage, testicular and penis calibre and degree of gynecomastia was determined. Sexual steroids, LH-FSH, prolactin, were studied. All patients had psychological stress. 11 patients had been treated surgically by mastectomy with success.
Assuntos
Ginecomastia/cirurgia , Adolescente , Mama/patologia , Mama/cirurgia , Criança , Ginecomastia/diagnóstico , Ginecomastia/patologia , Humanos , Masculino , Mastectomia Simples , Esteroides/análiseRESUMO
Fifty-two posterior spinal fusions were performed for pediatric idiopathic, congenital, and neuromuscular scoliotic curves. Cotrel-Dubousset instrumentation was used in all patients. Nine had prior anterior spinal releases and fusions. The patterns were mixed, with a predominance of right thoracic curvatures. The average preoperative curve measured 60.6 degrees, with correction to 29. Seven patients required revision surgery, and 17 wore orthoses after operation. There were 17 complications in this group, including hook pullout, prominent hardware, infection, pseudarthrosis, and two cases of broken Cotrel-Dubousset instrumentation rods. Fatigue failure of this instrumentation, secondary to pseudarthrosis, has not been reported previously, and these two cases are presented in detail. The operative morbidity and difficulty were increased in the larger idiopathic curves and in neuromuscular and congenital scoliosis. Cotrel-Dubousset instrumentation is an overall excellent tool for the multiplanar correction of scoliosis and is amenable to revision surgery.
Assuntos
Dispositivos de Fixação Ortopédica , Escoliose/cirurgia , Adolescente , Adulto , Criança , Desenho de Equipamento , Falha de Equipamento , Feminino , Seguimentos , Humanos , Masculino , Doenças Neuromusculares/complicações , Dispositivos de Fixação Ortopédica/efeitos adversos , Radiografia , Estudos Retrospectivos , Escoliose/congênito , Escoliose/etiologia , Coluna Vertebral/diagnóstico por imagemRESUMO
Ten patients with complete or incomplete androgens insensitivity syndrome (A.I.S.) is reported. Diagnosis of A.I.S. is presented on the basis: clinical, phenotype, studies of sexual steroids specially androgens, gonadotropins, 5-alpha-reductase activity and possible abnormality of androgen target cells from the genital skin. Histology of the testes is shown. Surgical treatment of these patients is reported, depending of the abnormality of the genitalia.
Assuntos
Androgênios/fisiologia , Transtornos do Desenvolvimento Sexual/fisiopatologia , Transtornos do Desenvolvimento Sexual/cirurgia , Adolescente , Criança , Pré-Escolar , Tolerância a Medicamentos , Feminino , Humanos , Lactente , MasculinoRESUMO
Twenty-seven cases are presented of neovaginoplasty and four methods used by the authors to construct an artificial vagina are described. The advantages and disadvantages of the surgical techniques used in vaginoplasty and long-term results are discussed.
Assuntos
Cirurgia Plástica/métodos , Vagina/anormalidades , Adolescente , Adulto , Colo Sigmoide/cirurgia , Feminino , Seguimentos , Humanos , Mucosa/cirurgia , Peritônio/cirurgia , Transplante de Pele , Stents , Fatores de Tempo , Bexiga Urinária/cirurgia , Vagina/cirurgiaRESUMO
We report an anesthetic technique to control hypertension in a Wilms' tumor in stage I in a 25-month-old child weighing 10 kg. He was treated with actinomycin D and vincristine during 4 weeks before surgical resection, developing inadequate ADH secretion due to vincristine. General anesthesia was used, with atropine, thiopental and atracurium as muscular relaxant. Systemic intraoperative anesthesia with narcotics was not given. A catheter was placed in the epidural space, with entrance through L3-L4 and its extreme in L1. Radiological control of the distal end of the catheter was carried out with iohexol before the administration of two 0.3% bupivacaine doses without adrenaline of 3 ml each, and intraoperative normal blood pressure was achieved. It was concluded that combined superficial general anesthesia techniques, associated with continuous epidural blockade, can be useful to control hypertension in Wilms' tumor.