RESUMO
Neuroendocrine Tumors (NETs) encompass a wide variety of tumors arising from neuroendocrine cells, which produce bioactive substances. The incidence of NETs increased significantly lately, becoming one of the most common tumors of the digestive tract. Their clinical presentation is as diverse as their capacity for hormone production. Carcinoid syndrome is the most common hormonal syndrome produced by NETs and is characterized by diarrhea, flushing and cardiac valvular lesions. New research brought multiple changes in the classification of these neoplasms and a new understanding about their diagnosis and treatment, promoting a multidisciplinary approach. Somatostatin analogues, radiation, biological, and cytotoxic drugs have improved the prognosis of these patients, which entails a great challenge for healthcare providers.
Assuntos
Antineoplásicos , Tumores Neuroendócrinos , Antineoplásicos/uso terapêutico , Diarreia , Humanos , Tumores Neuroendócrinos/diagnóstico , Tumores Neuroendócrinos/terapia , Somatostatina/uso terapêuticoRESUMO
Neuroendocrine Tumors (NETs) encompass a wide variety of tumors arising from neuroendocrine cells, which produce bioactive substances. The incidence of NETs increased significantly lately, becoming one of the most common tumors of the digestive tract. Their clinical presentation is as diverse as their capacity for hormone production. Carcinoid syndrome is the most common hormonal syndrome produced by NETs and is characterized by diarrhea, flushing and cardiac valvular lesions. New research brought multiple changes in the classification of these neoplasms and a new understanding about their diagnosis and treatment, promoting a multidisciplinary approach. Somatostatin analogues, radiation, biological, and cytotoxic drugs have improved the prognosis of these patients, which entails a great challenge for healthcare providers.
Assuntos
Humanos , Tumores Neuroendócrinos/diagnóstico , Tumores Neuroendócrinos/terapia , Antineoplásicos/uso terapêutico , Somatostatina/uso terapêutico , DiarreiaRESUMO
Neuroendocrine tumors (NETs) are relatively rare and highly heterogeneous neoplasms. Despite this, recent studies from North America and Central Europe have suggested an increase in incidence. In Latin America, NET data are scarce and scattered with only a few studies reporting registries. Our goal was to establish a NET registry in Chile. Here, we report the establishment and our first 166 NET patients. We observed a slight preponderance of males, a median age at diagnosis of 53 years and a median overall survival of 110 months. As anticipated, most tumors were gastroenteropancreatic (GEP). Survival analyses demonstrated that non-GEP or stage IV tumors presented significantly lower overall survival (OS). Similarly, patients with surgery classified as R0 had better OS compared to R1, R2, or no surgery. Furthermore, patients with elevated chromogranin A (CgA) or high Ki67 showed a trend to poorer OS; however, these differences did not reach statistical significance (log-rank test p = 0.07). To the best of our knowledge, this is the first report of a NET registry in Chile. Median OS in our registry (110 months) is in line with other registries from Argentina and Spain. Other variables including age at diagnosis and gender were similar to previous studies; however, our data indicate a high proportion of small-bowel NETs compared to other cohorts, reflecting the need for NET regional registries. Indeed, these registries may explain regional discrepancies in incidence and distribution, adding to our knowledge on this seemingly rare, highly heterogeneous disease.
Assuntos
Tumores Neuroendócrinos/diagnóstico , Tumores Neuroendócrinos/epidemiologia , Sistema de Registros , Adulto , Idoso , Idoso de 80 Anos ou mais , Chile/epidemiologia , Cromogranina A/sangue , Feminino , Humanos , Ácido Hidroxi-Indolacético/sangue , Incidência , Neoplasias Intestinais/diagnóstico , Neoplasias Intestinais/epidemiologia , Neoplasias Intestinais/mortalidade , Estimativa de Kaplan-Meier , Antígeno Ki-67/análise , Masculino , Pessoa de Meia-Idade , Tumores Neuroendócrinos/mortalidade , Neoplasias Pancreáticas/diagnóstico , Neoplasias Pancreáticas/epidemiologia , Neoplasias Pancreáticas/mortalidade , Serotonina/sangue , Neoplasias Gástricas/diagnóstico , Neoplasias Gástricas/epidemiologia , Neoplasias Gástricas/mortalidade , Resultado do Tratamento , Adulto JovemRESUMO
Congenital hyperinsulinism (CH) is the most frequent cause of persistent hypoglycemia in the newborn and it is characterized by an inappropriately elevated insulin level in presence of hypoglycemia. Initial management is medical treatment, but if it fails, partial pancreatectomy is the surgical procedure of choice. OBJECTIVE: To report a case of a newborn with CH to aware on this condition and update its multidisciplinary management. CASE REPORT: The case of a full term newborn with severe symptomatic hypoglycemia unresponsive to medical treatment is reported . Ultrasonography and abdominal MRI were normal, Gallium 68 PET/CT showed uptake in the body of the pancreas. Laparoscopic corporocaudal pancreatectomy was performed. The patient resolved hypoglycemia and was discharged without incidents. Genetic study was consistent with focal CH, confirmed later by biopsy. DISCUSSION: This case report exposes the clinical presentation of CH and its diagnosis. It emphasizes on the surgical treatment and the advantages of minimally invasive technique.
Assuntos
Hiperinsulinismo Congênito/diagnóstico , Hiperinsulinismo Congênito/cirurgia , Humanos , Recém-Nascido , Masculino , Pancreatectomia/métodosRESUMO
El hiperinsulinismo congénito (HC) es la causa más frecuente de hipoglicemia persistente del recién nacido (RN), y se caracteriza por secreción no regulada de insulina frente a hipoglicemia. El manejo inicial es médico, pero en caso de fracaso, la pancreatectomía parcial es el tratamiento quirúrgico de elección. Objetivo: Comunicar el caso de un RN con HC con énfasis en el estudio genético, de imágenes y manejo quirúrgico, incluyendo la cirugía laparoscópica. Caso clínico: RN de término, con hipoglicemia prolongada por HC que no respondió al tratamiento médico. La ultrasonografía y resonancia nuclear magnética abdominal fueron normales. PET/CT Galio 68 evidenció aumento de metabolismo glucídico focalizado en el cuerpo pancreático. Se realizó pancreatectomía córporo-caudal laparoscópica sin incidentes, con mejoría de la hipoglicemia. El estudio genético fue compatible con HC focal, confirmado luego por biopsia. Conclusión: Se expone acerca de las características clínicas y diagnóstico del HC, en particular el rol de la cirugía y ventajas del abordaje laparoscópico.
Congenital hyperinsulinism (CH) is the most frequent cause of persistent hypoglycemia in the newborn and it is characterized by an inappropriately elevated insulin level in presence of hypoglycemia. Initial management is medical treatment, but if it fails, partial pancreatectomy is the surgical procedure of choice. Objective: To report a case of a newborn with CH to aware on this condition and update its multidisciplinary management. Case report: The case of a full term newborn with severe symptomatic hypoglycemia unresponsive to medical treatment is reported . Ultrasonography and abdominal MRI were normal, Gallium 68 PET/CT showed uptake in the body of the pancreas. Laparoscopic corporocaudal pancreatectomy was performed. The patient resolved hypoglycemia and was discharged without incidents. Genetic study was consistent with focal CH, confirmed later by biopsy. Discussion: This case report exposes the clinical presentation of CH and its diagnosis. It emphasizes on the surgical treatment and the advantages of minimally invasive technique.
Assuntos
Humanos , Masculino , Recém-Nascido , Hiperinsulinismo Congênito/diagnóstico , Pancreatectomia/métodos , Hiperinsulinismo Congênito/cirurgiaRESUMO
BACKGROUND: Ventricular dyssynchrony is a common finding in patients with heart failure (HF), especially in the presence of conduction delays. The loss of ventricular synchrony leads to progressive impairment of contractile function, which may be explained in part by segmental abnormalities of myocardial metabolism. However, the association of these metabolic disarrangements with parameters of ventricular dyssynchrony and electrocardiography (ECG) findings has not yet been studied. METHODS: Our aim was to determine the correlation between the presence of left bundle branch block (LBBB) with left ventricular (LV) mechanical synchrony assessed by multiple-gated acquisition scan (MUGA) and with patterns of 18-fluorodeoxyglucose (18FDG) uptake in patients with non-ischemic heart failure. Twenty-two patients with non-ischemic cardiomyopathy, LV ejection fraction (LVEF) ≤45% and New York Heart Association (NYHA) Functional Class II or III symptoms under standard medical therapy were included, along with 10 healthy controls matched for age and gender. A 12-lead ECG was obtained to measure the length of the QRS. Mechanical LV synchrony was assessed by MUGA using phase analysis. All patients and controls underwent positron emission tomography with 18FDG to determine the distribution of myocardial glucose uptake. The standard deviation of peak (18)FDG uptake was used as an index of metabolic heterogeneity. Student's t-test and Pearson's correlation were used for statistical analysis. RESULTS: The mean age of the patients with HF was 54 ± 12 years and 72% were male. The length of the QRS was 129 ± 31 milliseconds and LBBB was present in 9 patients. Patients with HF had decreased LV 18FDG uptake compared with controls (7.56 ± 3.36 vs. 11.63 ± 4.55 standard uptake value; p = 0.03). The length of the QRS interval correlated significantly with glucose uptake heterogeneity (r = 0.62; p = 0.002) and mechanical dyssynchrony (r = 0.63; p = 0.006). HF patients with LBBB showed marked glucose uptake heterogeneity compared with HF patients without LBBB (41.4 ± 10 vs 34.7 ± 4.9 ml/100 g/min, respectively; p = 0.01). CONCLUSIONS: Patients with non-ischemic heart failure exhibit a global decrease in myocardial glucose uptake. Within this group, subjects who also have LBBB exhibit a marked heterogeneity in segmental glucose uptake, which directly correlates with QRS duration.
Assuntos
Bloqueio de Ramo/metabolismo , Bloqueio de Ramo/fisiopatologia , Insuficiência Cardíaca/metabolismo , Insuficiência Cardíaca/fisiopatologia , Disfunção Ventricular Esquerda/metabolismo , Disfunção Ventricular Esquerda/fisiopatologia , Adulto , Idoso , Estudos de Casos e Controles , Eletrocardiografia , Feminino , Fluordesoxiglucose F18/metabolismo , Glucose/metabolismo , Humanos , Masculino , Pessoa de Meia-Idade , Tomografia por Emissão de Pósitrons , Volume Sistólico/fisiologiaRESUMO
It is unknown why heart failure progresses even when patients are treated with the best therapy available. Evidences suggest that heart failure progression is due to loss of neurohumoral blockade in advanced stages of the disease and to alterations in myocardial metabolism induced, in part, by this neurohumoral activation. Alterations in cardiac energy metabolism, especially those related to substrate utilization and insulin resistance, reduce the efficiency of energy production, causing a heart energy reserve deficit. These events play a basic role in heart failure progression. Therefore, modulation of cardiac metabolism has arisen as a promissory therapy in the treatment of heart failure. This review describes myocardial energy metabolism, evaluates the role of impaired energy metabolism in heart failure progression and describes new therapies for heart failure involving metabolic intervention.
Assuntos
Progressão da Doença , Metabolismo Energético/fisiologia , Insuficiência Cardíaca/tratamento farmacológico , Insuficiência Cardíaca/fisiopatologia , Miocárdio/metabolismo , HumanosRESUMO
It is unknown why heart failure progresses even when patients are treated with the best therapy available. Evidences suggest that heart failure progression is due to loss of neurohumoral blockade in advanced stages of the disease and to alterations in myocardial metabolism induced, in part, by this neurohumoral activation. Alterations in cardiac energy metabolism, especially those related to substrate utilization and insulin resistance, reduce the efficiency of energy production, causing a heart energy reserve deficit. These events play a basic role in heart failure progression. Therefore, modulation of cardiac metabolism has arisen as a promissory therapy in the treatment of heart failure. This review describes myocardial energy metabolism, evaluates the role of impaired energy metabolism in heart failure progression and describes new therapies for heart failure involving metabolic intervention.
Assuntos
Humanos , Progressão da Doença , Metabolismo Energético/fisiologia , Insuficiência Cardíaca/tratamento farmacológico , Insuficiência Cardíaca/fisiopatologia , Miocárdio/metabolismoRESUMO
BACKGROUND: In 2005, 80% of cardiovascular disease (CVD) deaths occurred in low- to middle-income countries (i.e., developing nations). Cardiovascular imaging, such as myocardial perfusion SPECT, is one method that may be applied to detect and foster improved detection of at-risk patients. This document will review the availability and utilization for nuclear cardiology procedures worldwide and propose strategies to devise regional centers of excellence to achieve quality imaging around the world. METHODS: As a means to establish the current state of nuclear cardiology, International Atomic Energy Agency member and non-member states were queried as to annual utilization of nuclear cardiology procedures. Other sources for imaging statistics included data from medical societies (American Society of Nuclear Cardiology, European Society of Cardiology, and the European Association of Nuclear Medicine) and nuclear cardiology working groups within several nations. Utilization was calculated by dividing annual procedural volume by 2007 population statistics (/100,000) and categorized as high (>1,000/100,000), moderate-high (250-999/100,000), moderate (100-249/100,000), low-moderate (50-99/100,000) and low (<50/100,000). RESULTS: High nuclear cardiology utilization was reported in the United States, Canada, and Israel. Most Western European countries, Australia, and Japan reported moderate-high utilization. With the exception of Argentina, Brazil, Colombia and Uruguay, South America had low usage. This was also noted across Eastern Europe, Russia, and Asia. Utilization patterns generally mirrored each country's gross domestic product. However, nuclear cardiology utilization was higher for developing countries neighboring moderate-high "user" countries (e.g., Algeria and Egypt); perhaps the result of accessible high-quality training programs. CONCLUSIONS: Worldwide utilization patterns for nuclear cardiology vary substantially and may be influenced by physician access to training and education programs. Development of regional training centers of excellence can guide utilization of nuclear cardiology through the application of guideline- and appropriateness-driven testing, training, continuing education, and quality assurance programs aiding developing nations to confront the epidemics of CVD.
Assuntos
Cardiologia/estatística & dados numéricos , Diagnóstico por Imagem/estatística & dados numéricos , Acessibilidade aos Serviços de Saúde/estatística & dados numéricos , Avaliação das Necessidades , Medicina Nuclear/estatística & dados numéricos , Países DesenvolvidosRESUMO
We report an 11 year-old boy who presented with difficulty in swallowing without symptoms of hypothyroidism. The physical examination revealed a mass at the base of the tongue. The thyroid hormone profile showed a primary hypothyroidism (a serum TSH of 10.8 IU/mL with normal-low thyroxin of 6.0 microg/dL and low triiodothyronine of 57.8 ng/dL). Antithyroid antibodies were negative. The fiberoptic endoscopy showed a reddish mass, without evidence of haemorrhage or ulceration, confirmed as a well circumscribed, hypodense mass in the base of the tongue by computed tomography of the oropharynx and neck. Tc-99-pertechnetate scanning showed an abnormal area of uptake at the base of the tongue and no uptake in the normal thyroid location, concordant with an ectopic lingual thyroid gland. Levothyroxine in a suppressive dose was started that resulted in a reduction of the size of the mass and disappearance of dysphagia.
Assuntos
Transtornos de Deglutição/etiologia , Tireoide Lingual/complicações , Criança , Transtornos de Deglutição/diagnóstico , Diagnóstico Diferencial , Humanos , Tireoide Lingual/diagnóstico , Masculino , Testes de Função Tireóidea , Hormônios Tireóideos/sangue , Tireotropina/sangue , Tomografia Computadorizada por Raios XRESUMO
Objective: Determine differences in the epidemiology, clinical features and diagnosis of Graves-Basedow disease (GBD) in prepubertal and pubertal patients. Method: Retrospective study analyzing medical records of 38 patients with GBD at Pontificia Universidad Católica de Chile between 1992-2007. Statistical analysis was performed with non parametric test of Mann-Whitney U and proportions difference with Fisher Test (SPSS 10.0 for Windows and Graphpad Prism 4). Results: 21 patients were prepubertal and 17 were pubertal, with ages between 3 and 15,9 years. There were more girls than boys in both groups (5:2 and 15:2, respectively; p = 0.2). The most common clinical presentations were diffuse goiter, hyperactivity, frequent bowel movements, insomnia and heat intolerance. The prepubertal group had a taller stature (+2.4 SDS) compared with the pubertal group (+0.2 SDS; p = 0.03) and the most frequent ocular manifestation was exophthalmus in both groups. Conclusions: We did not find any differences in the clinical presentations of Graves-Basedow disease among prepubertal and pubertal patients. Neuropsychiatric symptoms such as hyperactivity and insomnia, together with tall stature are common features in children with GBD.
Objetivo: Determinar si existen diferencias en las características epidemiológicas y clínicas al momento del diagnóstico de hipertiroidismo por Basedow Graves (BG) en sujetos pre-púberes y púberes. Pacientes y Método: Estudio descriptivo y retrospectivo. Universo: Pacientes con diagnóstico de BG en control en endocrinología pediátrica en la Pontificia Universidad Católica de Chile, entre 1991 y abril 2007 (n = 38). Se registraron los hallazgos clínicos y de laboratorio. Se evaluó las diferencias entre los grupos con pruebas no paramétricas (Mann-Whitney U), las diferencias de proporciones con la Prueba de Fisher (SPSS 10.0 para Windows y graphpad Prism 4). Resultados: El rango de edad fue 3 a 15,9 años. Veintiún sujetos eran pre-púberes y 17 eran púberes; hubo más mujeres que hombres (5: 2 y 15: 2, respectivamente; p = 0,2). Los síntomas y signos más frecuentes fueron bocio difuso, hiperactividad, polidefecación, insomnio e irradiación de calor. No hubo diferencias entre los pre-púberes y púberes. El grupo pre-púber tenía talla más alta que su carga genética (+2,4 DS) comparados con los púberes (+0,2 SDS; p = 0,03). El compromiso ocular más frecuente fue el exoftalmo. Conclusión: No encontramos diferencias en la forma de presentación del BG entre los niños pre-púberes y púberes. Los síntomas neuropsiquiátricos tales como hiperactividad e insomnio, y una talla mayor a la esperada para la diana familiar, fueron hallazgos frecuentes en los niños con BG.
Assuntos
Humanos , Masculino , Feminino , Pré-Escolar , Criança , Adolescente , Doença de Graves/diagnóstico , Doença de Graves/epidemiologia , Antropometria , Autoanticorpos/análise , Chile/epidemiologia , Doença de Graves/imunologia , Doença de Graves/terapia , Hormônios Tireóideos/análise , Medicina Nuclear , Estudos Retrospectivos , Interpretação Estatística de DadosRESUMO
We report all year-old boy who presented with difficulty in swallowing without symptoms of hypothyroidism. The physical examination revealed a mass at the base of the tongue. The thyroid hormone profile showed a primary hypothyroidism (a serum TSH of 10.8 IU/mL with normal-low thyroxin of 6.0 fig/dL and low triiodothyronine of 57.8 ng/ dL). Antithyroid antibodies were negative. The fiberoptic endoscopy showed a reddish mass, without evidence of haemorrhage or ulceration, confirmed as a well circumscribed, hypodense mass in the base of the tongue by computed tomography of the oropharynx and neck. Tc-99-pertechnetate scanning showed an abnormal area of uptake at the base of the tongue and no uptake in the normal thyroid location, concordant with an ectopic lingual thyroid gland. Levothyroxine in a suppressive dose was started that resulted in a reduction of the size of the mass and disappearance of dysphagia.
Assuntos
Criança , Humanos , Masculino , Transtornos de Deglutição/etiologia , Tireoide Lingual/complicações , Transtornos de Deglutição/diagnóstico , Diagnóstico Diferencial , Tireoide Lingual/diagnóstico , Testes de Função Tireóidea , Hormônios Tireóideos/sangue , Tireotropina/sangue , Tomografia Computadorizada por Raios XRESUMO
El Megauréter Primario no Refluyente (MPNR) es una patología con alta resolución espontánea. El presente trabajo pretende estudiar como diferentes factores clínicos e imagenológicos objetivados en la evaluación inicial predicen aquellos pacientes que van a requerir conducta quirúrgica. Materiales y Métodos: Se realizó un análisis retrospectivo de pacientes con diagnóstico de MPNR diagnosticados entre el año 1995 y 2005. Se utilizó estadística descriptiva y test de chi-cuadrado. Resultados: Se reclutaron 44 pacientes, incluyendo 8 casos bilaterales (52 megauréteres). El tiempo promedio de seguimiento fue de 23,2 +/- 20,1 meses. La edad mediana de diagnóstico fue de 7,5 meses (rango 1-155 meses), con un 77 por ciento de pacientes masculinos. Un 32 por ciento de los pacientes recibieron tratamiento quirúrgico. No hubo asociación entre la conducta y el género o la forma de presentación. Los pacientes con megauréter izquierdo tuvieron mayor tendencia a ser manejados quirúrgicamente (0,01< p< 0,05), mientras que los bilaterales resolvieron todos médicamente (0,05< p< 0,1). La presencia de ITU durante el seguimiento fue un fenómeno infrecuente (9 por ciento), teniendo una asociación intermedia con la necesidad de tratamiento quirúrgico (0,01< p< 0,05). Una Función Renal Relativa Cintigráfica (FRR) inicial menor a 40 por ciento presentó un VPP=0,67 y un VPN=0,81 para predecir cirugía, con una probabilidad de asociación intermedia (0,01 < p < 0,05). Una Curva de Excreción Cintigráfica Obstructiva (CECO) inicial presentó un VVP=0,82 y un VPN=0,89 para predecircirugía, con una alta probabilidad de asociación (p < 0,01). Una Dilatación Pieloureteral Ecográfica Severa (DPUES) inicial presentó un VVP=0,67 y un VPN=0,89 para predecir cirugía, con una alta probabilidad de asociación (p < 0,01). Al presentarse estas condiciones en conjunto, se obtuvo una mejora importante de los VPP para predecir cirugía, con altas probabilidades de asociación...
Introduction: Primary non refluxing megaureter (PNM) has a high index of spontaneous resolution. The present study evaluates different clinical and imaging factors that may predict the need for surgical management. Material and methods: A retrospective analysis of PNM patients diagnosed between 1995 and 2005was performed. Data was described and analyzed with chi-square test. Results: A total of 44 patients, 8 bilateral cases (52 megaureters total), were analyzed. Mean follow up time was 23,2 +/- 20,1 months. Mean age was 7,5 months (range 1-155 months) with 77 percent of patients being male. A total of 32 percent patients underwent surgical treatment. There was no relation between medical conduct and form of presentation. Left megaureters were mostly treated surgically(0,01< p < 0,05) while bilateral ones were managed expectantly (0,05 < p < 0,1). Urinary infection during follow-up was infrequent (only 9 percent) having an intermediate association with surgical treatment (0,01< p < 0,05). Relative Renal Cintigram Function (RRCF) under 40 percent presented a PPV=0,67 and NPV=0,81 for surgery with and intermediate association probability (0,01< p< 0,05).Obstructive Renal Cintigram Curve (ORCC) presented a PPV=0,82 and a NPV=0,89 for surgery with a high association probability (p < 0,01). Initial Severe Pelvic Hydronefrosis (ISPH) presented PPV=0,67and NPV=0,89 for surgery with a high association probability (p < 0,01). All these conditions put together presented an increased in the PPV for predicting surgery with high association probabilities. Conclusions: Surgical management of PNM is infrequent. Gender and form of presentation were not significantly associated with management. Side and the presence of UTI during follow-up could be used to prognosticate the need for surgery. Prognostic value of RRCF, ORCC and ISPH is defined by their NPV. Renal ultrasound and cintigram are useful in predicting future therapeutic conduct.
Assuntos
Humanos , Masculino , Feminino , Lactente , Pré-Escolar , Criança , Doenças Ureterais , Doenças Ureterais , Dilatação Patológica , Dilatação Patológica , Estudos Retrospectivos , Seguimentos , Prognóstico , Rim , Rim , Sensibilidade e Especificidade , Valor Preditivo dos TestesRESUMO
La consulta por cojera en un niño es frecuente, en ella el médico debe realizar una completa anamnesis y exhaustivo examen físico para el diagnóstico diferencial, pero en la mayoría de los casos este se define por los hallazgos en los estudios por imágenes. Este trabajo orienta a reconocer qué estudio solicitar de acuerdo a la patología que se sospecha como diagnóstico.
Assuntos
Humanos , Adolescente , Pré-Escolar , Criança , Claudicação Intermitente/diagnóstico , Claudicação Intermitente/etiologia , Artrite Infecciosa/complicações , Diagnóstico Diferencial , Doença de Legg-Calve-Perthes/complicações , Extremidade Inferior , Osteomielite/complicações , Sinovite/complicações , Traumatismos da Perna/complicaçõesRESUMO
Arterial hypertension of adrenal etiology is mainly attributed to primary hyperaldosteronism. However, subtle expressions of hyperadrenergic or glucocorticoid excess can also generate arterial hypertension. The present report describes two hypertensive patients cataloged as resistant essential hypertensives, in whom adrenal masses were found incidentally, who highlight the need to recognize these tenuous clinical or laboratory presentations. Case 1 was a 50-yr-old female with hyperadrenergic hypertension associated to a left adrenal node, normal cortisol and aldosterone:renin ratio, marginally increased urinary normetanephrine, and a positive 131I MIBG radioisotope scan. Adrenalectomy normalized blood pressure and urinary metanephrines. Pathology showed a hyperplastic adrenal medulla associated to a multinodular cortical hyperplasia. Case 2 was a 62- yr-old female with progressive hypertension, a slight Cushing phenotype, non-suppressible hypercortisolism, normal urinary metanephrines, and bilateral adrenal nodes. Bilateral adrenalectomy and subsequent replacement normalized blood pressure and phenotypic stigmata. Pathology demonstrated bilateral cortical multinodular hyperplasia and medullary hyperplasia. The clinical study in both patients was negative for MEN. The apparently rare association of cortical and medullary lesions presented by both patients is probably overlooked in routine pathology exams, but should be meticulously searched since the crosstalk between the adrenal cortex and medulla may prompt dual abnormalities.
Assuntos
Doenças das Glândulas Suprarrenais/complicações , Glândulas Suprarrenais/patologia , Hipertensão/etiologia , Doenças das Glândulas Suprarrenais/diagnóstico , Neoplasias das Glândulas Suprarrenais/complicações , Neoplasias das Glândulas Suprarrenais/diagnóstico , Feminino , Humanos , Hiperplasia/complicações , Hiperplasia/diagnóstico , Pessoa de Meia-Idade , Feocromocitoma/complicações , Feocromocitoma/diagnósticoRESUMO
Acute pyelonephritis (APN) may produce permanent renal damage (PRD), which can subsequently lead to diverse complications. We prospectively evaluated 147 females and 122 males (mean age 3.5 years) with APN in order to analyze the relationship between the presence of PRD, at the time of cortical renal scintigraphy, and age, gender, episodes of urinary tract infection (UTI), and presence of vesicoureteral reflux (VUR). There were 152 children studied after the first proven UTI. VUR was present in 150 children. PRD was observed in 170 children. There were no significant differences between boys and girls. PRD was found in 36.4% of children younger than 1 year and in 70.1% of those older than 1 year ( P<0.0001). Of children with VUR, 72% had PRD compared with 52% of children without VUR ( P<0.0001). Of children with a first episode of UTI, 55.9% developed PRD as did 72.6% of those with recurrent UTI ( P=0.004). Our results showed that PRD in children with APN is important, especially in the presence of VUR, recurrent UTI, and older age.
Assuntos
Pielonefrite/etiologia , Insuficiência Renal/epidemiologia , Infecções Urinárias/complicações , Refluxo Vesicoureteral/complicações , Doença Aguda , Adolescente , Criança , Pré-Escolar , Cicatriz/etiologia , Demografia , Feminino , Humanos , Incidência , Lactente , Masculino , Estudos Prospectivos , Insuficiência Renal/etiologiaRESUMO
Renovascular hypertension (RVH) is responsible for 10% of arterial hypertension in children. The early diagnosis of RVH permits specific treatment leading to the cure of hypertension and avoidance of parenchymal damage. Captopril renal scintigraphy (CRS) provides information on the renovascular cause of the arterial hypertension. To validate the usefulness of CRS in hypertensive children, clinical, scintigraphic, and radiological data from 20 patients (mean age 6.1+/-5.5 years) were reviewed. Two patients were newborns. All had renal ultrasound scans and 9 had aortograms. In 7 children, RVH was confirmed by angiography, and CRS was positive for RVH in 6 of these. CRS was negative for RVH in 12 of 13 children without RVH. CRS was non-diagnostic in 3 children with abnormal baseline renal scintigraphy and severely decreased relative renal function ( <35%), 1 of whom had RVH. No side effects of captopril renography were observed. Captopril renography provides a logical, non-invasive, safe, and cost-effective approach in the evaluation of children suspected of having RVH.