RESUMEN
OBJECTIVE: Limited data regarding adrenal involvement in multiple endocrine neoplasia type 1 (MEN1) is available. We describe the characteristics of MEN1-associated adrenal lesions in a large cohort to provide a rationale for their management. METHODS: Analysis of records from 715 MEN1 patients from a multicentre database between 1956 and 2008. Adrenal lesions were compared with those from a multicentre cohort of 144 patients with adrenal sporadic incidentalomas. RESULTS: Adrenal enlargement was reported in 20.4% (146/715) of patients. Adrenal tumours (>10âmm in size) accounted for 58.1% of these cases (10.1% of the whole patient cohort). Tumours were bilateral and >40âmm in size in 12.5 and 19.4% of cases respectively. Hormonal hypersecretion was restricted to patients with tumours and occurred in 15.3% of them. Compared with incidentalomas, MEN1-related tumours exhibited more cases of primary hyperaldosteronism, fewer pheochromocytomas and more adrenocortical carcinomas (ACCs; 13.8 vs 1.3%). Ten ACCs occurred in eight patients. Interestingly, ACCs occurred after several years of follow-up of small adrenal tumours in two of the eight affected patients. Nine of the ten ACCs were classified as stage I or II according to the European Network for the Study of Adrenal Tumors. No evident genotype/phenotype correlation was found for the occurrence of adrenal lesions, endocrine hypersecretion or ACC. CONCLUSIONS: Adrenal pathology in MEN1 differs from that observed in sporadic incidentalomas. In the absence of relevant symptoms, endocrine biology can be restricted to patients with adrenal tumours and should focus on steroid secretion including the aldosterone-renin system. MEN1 is a high-risk condition for the occurrence of ACCs. It should be considered regardless of the size of the tumour.
Asunto(s)
Neoplasias de las Glándulas Suprarrenales/epidemiología , Bases de Datos como Asunto/estadística & datos numéricos , Estudios Multicéntricos como Asunto , Neoplasia Endocrina Múltiple Tipo 1/epidemiología , Feocromocitoma/epidemiología , Adolescente , Neoplasias de las Glándulas Suprarrenales/genética , Neoplasias de las Glándulas Suprarrenales/patología , Adulto , Anciano , Bélgica/epidemiología , Estudios de Casos y Controles , Niño , Preescolar , Estudios de Cohortes , Análisis Mutacional de ADN , Femenino , Francia/epidemiología , Humanos , Masculino , Persona de Mediana Edad , Estudios Multicéntricos como Asunto/estadística & datos numéricos , Neoplasia Endocrina Múltiple Tipo 1/genética , Neoplasia Endocrina Múltiple Tipo 1/patología , Feocromocitoma/genética , Feocromocitoma/patología , Proteínas Proto-Oncogénicas/genética , Carga Tumoral , Adulto JovenRESUMEN
Total T4, total T3 and TSH were measured in blood, urine and dialysate of 13 patients on CAPD. Iodine was measured in blood and urine. Free T4 and T3 were measured in blood only. Serum measurements revealed one true hypothyroidism, normal total T4, T3 and TSH for 12 patients, low free T4 and T3 with normal TSH for 9 patients. Iodine was slightly elevated for 3 patients. Urinary T4 and T3 were low. Dialysate T4 and T3 were low (T4 = 13, 6 nmol/24 H). Low free thyroid hormones are not relevant to iodine excess, nor to dialysate losses. Low free thyroid hormones may be considered as an endocrine dysfunction of chronic illnesses.
Asunto(s)
Yodo/análisis , Diálisis Peritoneal Ambulatoria Continua , Tirotropina/sangre , Tiroxina/sangre , Triyodotironina/sangre , Adulto , Anciano , Femenino , Humanos , Yodo/sangre , Yodo/orina , Masculino , Persona de Mediana Edad , Tirotropina/orina , Tiroxina/orina , Triyodotironina/orinaRESUMEN
Micro-albuminuria, glycosylated haemoglobin and creatinine clearance were measured in 28 insulin dependent and 8 insulin requiring diabetic patients (17 males aged 45 +/- 14.4 and 19 females aged 40 +/- 17 years). Twelve had retinopathy and six had high blood pressure. There was no statistical difference of duration of diabetes, creatinine clearance and glycosylated haemoglobin between patients with normal microalbuminuria and patients with hyper microalbuminuria. There was no correlation between glycosylated haemoglobin and micro-albuminuria, between creatinine clearance and micro-albuminuria, and between creatinine clearance and glycosylated haemoglobin. Patients with retinopathy did not have significantly higher levels of micro-albuminuria. Hypertensive patients had a significantly higher micro-albuminuria (p = 0.03, m = 45.10 +/- 56) compared with normotensive patients (m = 15.49 +/- 17.54). Glycosylated haemoglobin did not differ whether patients were normo or hypertensive. Hypertensive patients were significantly older (p = 0.01) than normotensive. The lack of difference in glycemic control between normo and hypertensive diabetics suggests that diabetes and hypertension are two independent cumulative risk factors of micro-albuminuria. Hypermicro-albuminuria may be secondary to microangiopathy in young diabetics and secondary to hypertension in old diabetics.
Asunto(s)
Albuminuria/sangre , Diabetes Mellitus Tipo 1/sangre , Angiopatías Diabéticas/sangre , Hipertensión/sangre , Adolescente , Adulto , Anciano , Glucemia/metabolismo , Creatina/orina , Femenino , Hemoglobina Glucada/análisis , Humanos , Masculino , Persona de Mediana EdadRESUMEN
A cross-sectional study was performed in 19 patients on haemodialysis and in 11 on continuous ambulatory peritoneal dialysis (CAPD) in order to investigate the relationship between thyroid hormones and nutritional status. T4, T3 resin uptake, T3, rT3 and TSH were measured by radio-immunoassay and compared with controls. Nutritional status was assessed by measurements of blood proteins, albumin, gamma-globulin, transferrin, arm muscle circumference and triceps skinfold thickness. In haemodialysis, T4, FTI, T3 and rT3 were significantly decreased. TSH was normal. In CAPD, thyroid hormones were normal. In both groups, proteins, albumin, gamma-globulin and transferrin were normal. Triceps skinfold thickness was normal in males and females, whereas arm muscle area was dramatically reduced in males and normal in females. In haemodialysis, a negative correlation was found between T3 and proteins (p less than 0.01), rT3 versus proteins (p less than 0.01) and versus gamma-globulin (p less than 0.01). In CAPD, a positive correlation was found between T3 and triceps skinfold thickness (p less than 0.05). We suggest that the overload of carbohydrate might normalize the thyroid hormones in patients on CAPD. The relationship between thyroid hormones and nutritional status in patients treated by dialysis suggests a putative protective effect of low T3 levels against protein breakdown.
Asunto(s)
Fallo Renal Crónico/sangre , Estado Nutricional , Diálisis Peritoneal Ambulatoria Continua , Hormonas Tiroideas/sangre , Adulto , Anciano , Estudios Transversales , Femenino , Humanos , Fallo Renal Crónico/fisiopatología , Masculino , Persona de Mediana Edad , Tirotropina/sangre , Tiroxina/sangre , Triyodotironina/sangre , Triyodotironina/fisiologíaRESUMEN
Three diabetic patients presenting with foot ulcers and arteriopathy were treated with continuous subcutaneous insulin infusion. Glycemic control was excellent and only minimal amputation was performed in two patients. Strict glycemic control improves healing of wounds even in diabetics suffering from chronic arteriopathy.
Asunto(s)
Complicaciones de la Diabetes , Enfermedades del Pie/tratamiento farmacológico , Sistemas de Infusión de Insulina , Úlcera Cutánea/tratamiento farmacológico , Angiopatías Diabéticas/tratamiento farmacológico , Enfermedades del Pie/etiología , Humanos , Masculino , Persona de Mediana Edad , Úlcera Cutánea/etiología , Cicatrización de Heridas/efectos de los fármacosRESUMEN
Thyroid hormone levels were measured in 12 patients on continuous ambulatory peritoneal dialysis and in 18 patients on haemodialysis, all in stable clinical status. Values in patients on peritoneal dialysis were not different from those found in controls, whereas a highly significant decrease in T3 and r T3 was observed in patients on haemodialysis. These results are in contrast with the low T3 syndrome and the high r T3 levels of systemic diseases and malnutrition. The difference between the two groups of patients on dialysis suggests that thyroid abnormalities do not result from renal failure alone, nor from malnutrition, but from a disturbance in the calorie/carbohydrate ratio of the ingesta.
Asunto(s)
Carbohidratos de la Dieta/administración & dosificación , Fallo Renal Crónico/metabolismo , Diálisis Renal/métodos , Triyodotironina Inversa/sangre , Triyodotironina/sangre , Adulto , Ingestión de Energía , Femenino , Glucosa/administración & dosificación , Humanos , Fallo Renal Crónico/terapia , Masculino , Persona de Mediana Edad , Diálisis Peritoneal Ambulatoria Continua , Hormonas Tiroideas/metabolismoRESUMEN
The first patient had Hashimoto's thyroiditis and developed membranous glomerulonephritis with subepithelial deposits of IgG, C3, and thyroglobulin; the second patient had Graves' disease and developed, after the second administration of 131 I 2, rapidly progressive glomerulonephritis with epithelial crescents; in the remaining patient, Hashimoto's thyroiditis was diagnosed at the time of renal insufficiency due to crescentic glomerulonephritis. All three patients had circulating antithyroglobulin antibodies, with high titers in patients I and III; in every patient, the search for circulating immune complexes was negative at the time of the renal biopsy. Apart from the classical membranous type, other glomerular lesions may be associated with thyroiditis. The frequent clinical latency of Hashimoto's disease warrants systematically testing for circulating antithyroglobulin antibodies in women presenting with apparently idiopathic glomerulonephritis.