Cintia Marques dos Santos Silva

Pituitary apoplexy during treatment of cystic macroprolactinomas with cabergoline

Pituitary apoplexy is a rare and life-threatening clinical condition caused by hemorrhage and/or infarction of the pituitary gland or adenoma. Although pituitary apoplexy is usually spontaneous, it has been associated with numerous precipitating factors, such as bromocriptine use. However, reports of pituitary apoplexy during cabergoline therapy are scarce. We report three patients with cystic macroprolactinomas who developed pituitary apoplexy during cabergoline treatment.

Low Frequency of Cardiomyopathy Using Cardiac Magnetic Resonance Imaging in an Acromegaly Contemporary Cohort

CONTEXT Left ventricular hypertrophy (LVH) and myocardial fibrosis are considered common findings of the acromegaly cardiomyopathy in echocardiography studies. OBJECTIVE To evaluate the frequency of LVH, systolic dysfunction and myocardial fibrosis was undertaken in patients with acromegaly using cardiac magnetic resonance imaging (CMRi) before and after 12 months of octreotide long-acting repeatable treatment. PATIENTS AND METHODS Consecutive patients with active acromegaly submitted to biochemical analysis and CMRi before and after 12 months of treatment. Additionally, echocardiography was performed before treatment. RESULTS Forty consecutive patients were evaluated using CMRi at baseline and 30 patients were reevaluated after 12 months of treatment. Additionally, 29 of these patients were submitted to echocardiography. Using CMRi, the frequency of LVH was 5%. The mean left ventricular mass index (LVMi) was 61.73 ± 18.8 g/m(2). The mean left ventricular ejection fraction (LVEF) was 61.85 ± 9.2%, and all patients had normal systolic function. Late gadolinium enhancement was present in five patients (13.5%), and one patient (3.5%) had an increased extracellular volume. After treatment, 12 patients (40%) had criteria for disease control. No clinically relevant differences in cardiac variables before and after treatment were observed. Additionally, there was no difference in LVMi and LVEF among patients with and without disease control. Using echocardiography, 31% of the patients had LVH, mean LVMi was 117.8 ± 46.3 g/m(2) and mean LVEF was 67.3 ± 4.4%. All patients had normal systolic function. CONCLUSIONS We demonstrated by CMRi, the gold-standard method, that patients with active acromegaly might have a lower prevalence of cardiac abnormalities than previously reported.

Prostate cancer and acromegaly

Acromegalic patients have an increased prevalence of prostatic disorders compared to age-matched healthy subjects. Increased size of the whole prostate or the transitional zone, together with an elevated incidence of other structural changes, such as nodules, cysts, and calcifications, have been reported. Prostate enlargement in young acromegalic patients with low testosterone levels due to central hypogonadism supports the hypothesis that chronic GH and IGF-I excess cause prostate hyperplasia. The relationship between prostatic carcinoma and acromegaly is, until now, only circumstantial. Long-term follow-up of these patients is necessary since epidemiologic studies showed association between serum IGF-I levels in the upper normal limit and prostate cancer in the general population. This review approaches prostate diseases in patients with acromegaly.

Hipotiroidismo autoimune refratário a altas doses de levotiroxina e hipocalcemia grave

The factors that make difficult the normalization of TSH in hypothyroidism need special attention because some patients on thyroxine replacement do not maintain a normal TSH. We report a 50 year-old woman with autoimmune hypothyroidism of difficult compensation, associated with anemia, hypocalcemia with a previous episode of tetany, hypomagnesemia, psychologic alterations and important weight loss. After compensation of the hypothyroidism with doses of L-thyroxine as high as 325µg/day, the hypothesis of a malabsorptive syndrome was raised. Celiac disease was confirmed by elevated serum antigliadin antibody. A gluten-free diet was instituted which improved the symptoms associated with malabsorption and reduced the L-thyroxine requirement to 125µg/day. Because several studies have shown an association of both diseases, a routine screening for celiac disease has been widely proposed in patients with autoimmune thyroid disease.

Growth hormone isoforms in acromegalic patients before and after treatment with octreotide LAR

BACKGROUND Human growth hormone (hGH) circulates as a mixture of different isoforms. It has been previously reported that the ratio of 20kDa to 20kDa plus 22kDa (%20kDa-hGH) is increased in patients with active acromegaly. OBJECTIVES To evaluate the GH isoforms (20kDa- and 22kDa-hGH) in acromegalic patients before and after six months of treatment with octreotide LAR, and to compare the results with those in healthy controls. In addition, the relationships between the %20kDa-hGH, tumor size and biochemical measurements were also investigated. DESIGN Random serum samples from 23 acromegalic patients evaluated before and after six months of treatment with octreotide LAR and from 23 matched healthy controls were studied. Growth hormone, IGF-I and prolactin (PRL) were measured by chemiluminescence immunometric assay and the 20kDa- and 22kDa-hGH isoforms were measured by specific time-resolved fluorescence immunoassays. RESULTS In acromegalic patients before treatment, there was a significantly higher median %20Da-hGH in comparison to healthy controls (14.31% vs. 9.59%, p<0.001). After six months of treatment, the median %20kDa-hGH was similar to the baseline values. Patients with GH<2.5ng/mL after six months of treatment had already lower GH and %20kDa-hGH at baseline (p<0.01). The IGF-I (SD-scores) was positively correlated to total GH levels in acromegalic patients after treatment. There was no correlation between the %20kDa-hGH and PRL levels or tumor size. CONCLUSIONS Our study confirmed that acromegalic patients have an increased proportion of circulating 20kDa-hGH isoform. Consequently, the use of a 22kDa-hGH specific assay may underestimate the tumor production of total GH. Although octreotide LAR promoted a significant decrease in the GH and IGF-I levels, it did not normalize the GH isoforms composition and suggests that the secretion of GH isoforms is equally inhibited by somatostatin analogues and that it is the disease control that normalizes the GH isoforms composition in acromegaly.

Experience with pegvisomant treatment in acromegaly in a single Brazilian tertiary reference center: efficacy, safety and predictors of response

Objective To describe the safety and efficacy of pegvisomant therapy and the predictors of treatment response in acromegaly patients at a single tertiary reference center in Brazil. Materials and methods We retrospectively reviewed the clinical, hormonal and radiological data of acromegaly patients treated with pegvisomant in our center. We also evaluated the presence of the d3 isoform of the growth hormone receptor (d3GHR). Results Twenty-seven patients were included (17 women). Pegvisomant was used in combination with octreotide LAR in 20 patients (74%), in combination with cabergoline in one (4%) and as monotherapy in six (22%). IGF-I normalization was achieved in 23 patients (85%). Mild and transitory elevation of liver enzymes was observed in two patients (7.4%), tumor growth in one (3.4%) and lipodystrophy in two (7.4%). One patient stopped the drug due to headaches. The GHR isoforms were evaluated in 14 patients, and the presence of at least one d3GHR allele was observed in 43% of them, but it was not a predictor of treatment response. Only pre-treatment IGF-I level was a predictor of treatment response. Conclusion Pegvisomant treatment was highly effective and safe in our series of Brazilian patients. A better chance of disease control can be expected in those with lower pre-pegvisomant IGF-I levels.

Transient central diabetes insipidus followed by pituitary apoplexy treated in a conservative way

A 49-year-old man, previously diagnosed with systemic ar-terial hypertension, presented to emergence room with severe headache. On physical examination blood pressure was 160x100 mm Hg. After 10 hours, despite of blood pressure normaliza-tion, the headache persisted and was followed by ophthalmo-plegia, meningeal irritation signs and consciousness impairment. A lumbar puncture was performed and meningitis was exclud-ed. Brain computed tomography was done and only maxillary sinusitis was detected. Three days after, the patient developed hypotension. Magnetic resonance imaging demonstrated a mac-roadenoma of 20x15x15 mm with supra-sellar extension and in-tratumoral hemorrhage (Figure). At that moment, pituitary func-tion tests were normal (thyrotropin: 1.46 mcUI/mL; RV: 0.3 a 5.0 mcUI/mL; free T4: 1.3 ng/dL; RV: 0.8-1.9 ng/dL; prolactin: 3.4 ng/dL; RV: 2,0 a 15,2 ng/mL) except for a low cortisol level (5.7

Two-dimensional speckle tracking echocardiography demonstrates no effect of active acromegaly on left ventricular strain

BackgroundSpeckle tracking echocardiography (STE) allows for the study of myocardial strain (ε), a marker of early and subclinical ventricular systolic dysfunction. Cardiac disease may be present in patients with acromegaly; however, STE has never been used to evaluate these patients.ObjectiveTo evaluate left ventricular (LV) global longitudinal strain in patients with active acromegaly with normal LV systolic function.DesignCross-sectional clinical study.MethodsPatients with active acromegaly with no detectable heart disease and a control group were matched for age, gender, arterial hypertension and diabetes mellitus underwent STE. Global LV longitudinal ε (GLS), left ventricular mass index (LVMi), left ventricular ejection fraction (LVEF) and relative wall thickness (RWT) were obtained via two-dimensional (2D) echocardiography using STE.ResultsThirty-seven patients with active acromegaly (mean age 45.6 ± 13.8; 48.6% were males) and 48 controls were included. The mean GLS was not significantly different between the acromegaly group and the control group (in %, −20.1 ± 3.1 vs. −19.4 ± 2.2, p = 0.256). Mean LVMi was increased in the acromegaly group (in g/m2, 101.6 ± 27.1 vs. 73.2 ± 18.6, p < 0.01). There was a negative correlation between LVMi and GLS (r = −0.39, p = 0.01).ConclusionsAcromegaly patients, despite presenting with a higher LVMi when analyzed by 2D echocardiography, did not present with impairment in the strain when compared to a control group; this finding indicates a low chance of evolution to systolic dysfunction and agrees with recent studies that show a lower frequency of cardiac disease in these patients.