RESUMO
A previously healthy 49-year-old male presented with abdominal pain, constitutional syndrome, paroxysmal palpitations and diaphoresis. Full-body CT scan showed a large malignant adrenal mass with abdominal lymph node and pulmonary metastasis. Biochemical studies revealed hypersecretion of catecholamines, cortisol, sexual steroids and steroid precursors; ACTH was not suppressed, and chromogranin A was negative. 18F-fluorodeoxyglucose PET/CT showed intense tracer uptake in the adrenal mass and abdominal lymph nodes. He was placed under adrenergic blockade and offered cytoreductive surgery, but his evolution was unfavorable with rapid clinical deterioration due to compressive abdominal symptoms, uncontrolled pain, peripheral oedema, cachexia and severe dilated cardiomyopathy. 123I-metaiodobenzylguanidine scintigraphy was negative. Poor clinical status precluded any surgical or systemic treatments. A biopsy of the adrenal mass suggested adrenocortical carcinoma. Two weeks later he developed recurrent level 3 non-insulin mediated hypoglycemias, with suppressed levels of insulin, C-peptide, IGF-1 and IGF-BP3. He responded poorly to palliative measures and died within a week, four months after the initial diagnosis. We present a puzzling case of an aggressive stage IV adrenal malignancy with bizarre secretory profile. Although we could not obtain a surgical specimen, combined available data suggested adrenocortical carcinoma. The pathophysiology is uncertain, and we explored exceedingly rare scenarios, including adrenocortical carcinoma masquerading as pseudo-pheochromocytoma; synchronous adrenocortical carcinoma and pheochromocytoma; adrenal mixed corticomedullary tumor; and ACTH-producing pheochromocytoma. The presence of ectopic ACTH-dependent hypercortisolism, discordant plasma and urinary metanephrine levels and IGF-2 mediated hypoglycemias were also quite perplexing. To our knowledge, this is the first report of a malignant adrenal tumor co-secreting steroid hormones with ACTH-dependent hypercortisolism, catecholamines and IGF-2. We faced obvious diagnostic and therapeutic challenges and encourage future studies to explore the complex interactions between cortical and chromaffin cells of the adrenal gland, that may have bidirectionally contributed to this patient's condition.
RESUMO
BACKGROUND: New glucose-lowering drugs have shown benefits regarding cardiovascular events, heart failure, and kidney-related outcomes in type 2 diabetes (T2D). This study aimed to estimate the adequacy of SGLT2 inhibitors (SGLT2i) and GLP-1 receptor agonists (GLP-1 RA) prescription to people living with T2D with established atherosclerotic cardiovascular disease (ASCVD) or heart failure (HF). MATERIAL AND METHODS: This was a cross-sectional study based on adults with T2D in a Portuguese local health unit between January 2019 and January 2020. Subjects with ASCVD were compared with subjects without ASCVD, and subjects with HF were compared with subjects without HF regarding clinical and demographic characteristics. RESULTS: Our study included 13,869 adults with T2D, among whom 5.9% were coded for HF and 20.4% were defined as having ASCVD. SGLT2i were prescribed to 36.0% of subjects with HF. SGLT2i and/or GLP-1 RA were prescribed to 36.1% of patients with ASCVD. When comparing with subjects without ASCVD, subjects with ASCVD were significantly older (70.8 vs. 66.5 years, p<0.001), had lower estimated glomerular filtration rate (68.2 vs. 74.6 mL/min/1.73 m2, p<0.001), and higher rates of prescription of SGLT2i and/or GLP-1 RA (36.1 vs. 31.4%, p<0.001). When comparing with subjects without HF, subjects with HF were significantly older (74.6 vs. 66.9 years, p<0.001), had lower estimated glomerular filtration rate (59.6 vs. 74.1, mL/min/1.73 m2, p<0.001), and higher rates of prescription of SGLT2i (36.0 vs. 30.3%, p<0.001). CONCLUSION: SGLT2i and GLP-1 RA are underprescribed in T2D, with almost two-thirds of patients not being prescribed these agents despite being strongly advised by current guidelines. These findings highlight the need for specific actions to improve T2D management at primary care level.
RESUMO
Objectives. Hungry bone syndrome (HBS) is a severe and underdiagnosed complication of parathyroidectomy in the treatment of primary hyperparathyroidism (PHP) and secondary hyper-parathyroidism to chronic kidney disease (SHP-CKD).Methods. A longitudinal study was conducted to compare the postoperative outcomes of patients who developed HBS in two different time frames: before and after implementing a protocol with an intensive electrolytic monitoring and an algorithm regarding electrolytic supplementation.Results. Overall, 77 parathyroidectomies were included. In PHP, a protocol implementation led to an increased admission of patients in the Intermediate Care Unit for intensive electrolytic monitoring (p<0.001) and an increased rate of oral calcium replacement during hospital stay (p=0.013) compared to pre-protocol era. In SHP-CKD, duration of intravenous calcium replacement was reduced (p=0.010). The prevalence of HBS (9.8% in PHP and 58.3% in SHP-CKD) was similar between the two periods, although its diagnosis had an increased trend in PHP since the protocol implementation. None of the diagnosis of HBS was established due to hypocalcemic symptoms in the post-protocol era (contrary to pre-protocol period, p=0.021). Both hypocalcemia length and duration of surgical ward hospitalization were reduced (p=0.047 and p=0.042, respectively).Conclusions. An improved assessment of hyperparathyroidism and a decrease in HBS severity were noted in the post-protocol era. We strongly recommend the implementation of a standardized protocol with an intensive phosphocalcium monitoring in the high-risk patients who undergo parathyroidectomy due to hyperparathyroidism as it improves the health care and management of HBS.