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Monoclonal gammopathy of undetermined significance (MGUS) is an asymptomatic premalignant plasma cell disorder that is characterized by the presence of serum M-protein less than 30 g/L or 3 g/dL, bone marrow (BM) clonal plasma cells less than 10%, absence of plasma cell myeloma (PCM) related end-organ damage (CRAB symptoms: hypercalcemia, renal insufficiency, anemia and, bone lesions) and absence of B-cell lymphoma or other disease known to produce an M-protein. MGUS is generally considered a preneoplastic disorder that does not always progress to overt malignancy (1, 2). Diverse endocrinopathies occur in patients with plasma cell disorders (3-6). One possible scenario is the rather rare POEMS syndrome, which is a paraneoplastic syndrome with key manifestations of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (7). We present a case study which emphasizes the importance of multidisciplinary evaluation of MGUS.

Epileptic seizures are defined as the transient appearance of signs or symptoms due to excessive or synchronous neuronal activity in the cerebral cortex. Pheochromocytomas and paragangliomas (PPGL) are tumors of chromaffin cells that arise from the medulla of the adrenal gland in 80-85% of patients and from the extra-adrenal sympathetic tissue of the abdomen, pelvis and chest in 10-20% of patients. The clinical picture of PPGL is variable and ranges from the absence of symptoms to severe clinical picture, depending on the biochemical profile. They are most often manifested by paroxysmal hypertension, followed by episodes of severe headache or diaphoresis, while epileptic attacks are rare. Neurological symptoms are present in many patients with PPGL. Also, paroxysmal neurological conditions such as vasodilating headache, intracranial tumors, diencephalic-autonomic epilepsy, hypertensive encephalopathy, focal arterial disease of the brain and anxiety state have been described, which may have similar clinical manifestations with pheochromocytomas. We present a 44-year-old woman, who has been diagnosed with pheochromocytoma as possible etiological basis of epileptic seizures. Pheochromocytoma, with its low incidence and "chameleon" clinical spectrum, should be considered as a potential etiological factor of convulsions.

Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis, is a necrotizing vasculitis of small and medium-sized blood vessels characterized by diffuse inflammation of vascular structures and perivascular and extravascular granulomatosis. In its systemic form, GPA predominantly affects the ear, nose and throat, with lung and kidney involvement with typically rapidly progressive necrotizing glomerulonephritis with extracapillary crescents, while the absence of kidney damage at the time of diagnosis is defined as a limited form of GPA with a more favorable prognosis (1, 2). Antineutrophil cytoplasmic antibodies (c-ANCA) with specificity for proteinase 3 (PR3) represent a biochemical diagnostic criterion. They are detected in 90% of generalized forms and in about 50% of limited forms of granulomatosis with polyangiitis (1, 2). In the absence of treatment, GPA is a disease of progressive evolution. Systemic corticosteroid therapy and immunosuppressive therapy significantly changed the prognostic aspect of the disease. Only a few sporadic observations have been published on endocrine disorders associated with GPA. We present a case of a man, 39 years old, with Wegener's granulomatosis who developed autoimmune thyroiditis 8 years after the initial diagnosis.

Background: Although thyroid hormones have significant effect on cardiovascular system, the impact of subtle thyroid dysfunction such as subclinical hypothyroidism (SCH) remains to be determined. We investigated coronary flow reserve (CFR) in patients with subclinical hypothyroidism. Methods: Thirty two subjects with SCH and eighteen control subjects with normal serum thyroid hormones and thyroid-stimulating hormone (TSH) levels were included in the study. TSH, free thyroxine, free triiodothyronine, glucose, insulin, HbA1c, cholesterol, triglyceride and plasma levels of C-reactive protein were measured. Coronary diastolic peak flow velocities in left anterior descending coronary artery were measured at baseline and after adenosine infusion. CFR was calculated as the ratio of hyperemic to baseline diastolic peak velocity. Results: CFR values were not significantly different between the two groups (SCH 2.76±0.35 vs controls 2.76±0.42). There was a significant correlation of CFR with waist to hip ratio, hypertension, smoking habits, markers of glucose status (glucose level, HbA1c, insulin level, HOMA IR), cholesterol, LDL-cholesterol and triglyceride levels in SCH group, whereas only cholesterol level showed significant correlation with CFR in controls. There was no correlation between CFR and thyroid hormones. Conclusions: We concluded that there is a different impact of cardiovascular risk factors on CFR in SCH patients compared to healthy control and that these two groups behave differently in the same circumstances under the same risk factors. The basis for this difference could be that the altered thyroid axis "set point" changes the sensitivity of the microvasculature in patients with SCH to known risk factors.

Purpose: The aim of this study was to assess the relationship between adrenal incidentaloma size, cortisol secretion suppressibility by Dexamethasone and relevant clinical and laboratory parameters. Methods: This retrospective study included all patients diagnosed with adrenal incidentaloma and admitted to the single department of the Clinic of endocrinology, Diabetes and Diseases of Metabolism during the period from 2012 till the end of 2019. The inclusion criterion was the presence of an asymptomatic adrenal mass on imaging not performed for suspected adrenal disease. The study group included 197 subjects. Adrenal masses were detected using CT or NMR scan. We analysed the correlation between tumour size and relevant hormonal and clinical parameters. Results: We found a significant positive correlation between incidentaloma size and morning cortisol, cortisol after overnight Dexamethasone, and a significant negative correlation between incidentaloma size and morning ACTH, and hip T score. Also, there was a weak but statistically significant correlation between incidentaloma size and systolic blood pressure. Conclusions: Our study shows that the prevalence of adrenal autonomous cortisol secretion increases with incidentaloma size, and that prevalence of hypertension and osteoporosis also increases with an increase of incidentaloma size.

There are three basic modalities for the treatment of thyrotoxicosis: thyrosuppresive drug therapy, ablation with radioactive iodine and surgical treatment. Patients who do not achieve adequate thyrotoxicosis control, as was the case of described patient, have a high mortality rate due to the possibility of developing a thyroid storm. The use of drug therapy for hyperthyroidism, as the first line of treatment, is associated with the appearance of various side effects, as was the case in our patient. Side effects of Methimazole are dose-dependent, while in the case of Propylthiouracil, the occurrence of side effects is not clearly dose-dependent. In the case of the described patient, all alternative, lesser known modalities for the treatment of hyperthyroidism were applied, after the occurrence of adverse reactions to thyrosuppressive therapy. Sodium perchlorate, ie. Sodium with perchloric acid is rarely used in the treatment of hyperthyroidism, as in cases of severe idiosyncratic reactions to thionamides, agranulocytosis or hepatitis, if the eumetabolic state is not achieved and the application of a therapeutic dose of radioiodine is not possible. It is applied in the form of a solution, usually 8%; In more severe forms of the disease, when hyperthyroidism is very pronounced, 10 to 15 drops a day are given 4 to 6 times and the dose is sometimes reduced to the minimum maintenance dose. After the adverse reaction even to sodium perchlorate therapy we were left with one more, last option-Plasma Therapy exchange. Plasma Therapy Exchange (TPE) is an out-of-body blood purification technique designed to remove high-molecular-weight substances bound to plasma proteins (autoantibody pathogens, immunocomplexes, cryoglobulins, myeloma light chains, endotoxins, lipoprotein-containing cholesterol, and thyroid). The effectiveness of treatment depends on the volume of blood being processed, the volume of plasma exchanged in each process, the number of procedures performed, the frequency of exchange and the rate of mobilization, stabilization and re-synthesis of cells or plasma components. TPE is an effective alternative treatment that provides an opportunity to prepare patients for definitive treatment: ablative therapy such as RAI ablation or thyroidectomy. Therapeutic plasmapheresis, if performed in specialist centers, is a safe, fast and effective method.

Syndrome Fahr is described as calcification of the basal ganglia and is a rare inherited or sporadic neurological disease with a prevalence of 1:1000000 (1). For the first time described in 1930 in the works of Dr. Karl Theodor Fahr, it is characterized by calcium deposition in areas of the brain to control movement: basal gamma, thalamus, cerebral cortex, cerebellum, dentate nucleus, subcortical white matter and hippocampus (2). The diagnostic criteria were revised and modified by Moskowitz et al. in 1971., Ellie et al. in 1989. and Manyam in 2005. and include: bilateral calcifications of the basal ganglia confirmed by neuroradiological visualization, progressive neurological dysfunction and neuropsychiatric manifestations, absence of biochemical and somatic entities of mitochondrial disease, absence of infectious, traumatic, or toxic etiology, and autosomal dominant inheritance (2,3,4). Etiologically, endocrine diseases, and especially parathyroid gland dysfunction, are closely related to Farr's syndrome, including idiopathic hypoparathyroidism, secondary hypoparathyroidism, pseudohypoparathyroidism, pseudo-pseudohypoparathyroidism, and hyperparathyroidism. Pseudo and pseudo-pseudohypoparathyroidism are defined by a phenotypic spectrum induced by a mutation in RNA. They manifest on average at the age of 8 to 10, with clinical manifestations very similar to hypoparathyroidism, except for a slightly more pronounced intellectual declension. The term pseudohypoparathyroidism (PHP) for subtypes PHP type IA and PHP type 1C includes diseases that share the biochemical characteristics of hypoparathyroidism (hypocalcemia and hyperphosphatemia), and which are the consequences of resistance of target tissues to the action of parathyroid hormone. In some cases, resistance to TSH, gonadotropins, GHRH and calcitonin, which have receptors bound to the Gs Alpha subunit, is detected. Patients with type 1A and type 1C PHP show diverse phenotypic expression combined by the term Albright hereditary osteodystrophy (AHO), which includes premature closure of bone and short bone growth zones, low growth, ectopic calcifications. More specifically, based on the dissemination of ectopic calcifications, patients were classified into three categories: pseudohypoparathyroidism, progressive bone heteroplasia, and osteoma cutis. Our patient was diagnosed as pseudohypoparathyroidism at an early age based on the phenotype characteristics of AHO and the biochemical findings of hypocalcemia. Substitution therapy with vitamin D and calcium was introduced, and after pronounced changes in behavior, feelings of being followed and extreme anxiety, he was hospitalized in the Clinic for Neurology and in the Clinic for Endocrinology, Clinical Center of Serbia. Neurofunctional studies have shown progressive decline in intellectual function, neuroradiologically were detected zones of calcification of the basal ganglia and frontal subcortical white matter, as well as hypothyroidism as a consequence of TSH resistance. High concentrations of calcitonin were detected, also as a consequence of resistance of target tissues to calcitonin.

Monoklonalna gamopatija neutvrđenog značaja (engl. Monoclonal Gammopathy of Undetermined Significance, MGUS) je asimptomatski preneoplastični poremećaj plazma ćelija koji se karakteriše serumskim M-proteinom manjim od 30 g/L, klonalnim plazma ćelijama koštane srži manje od 10%, odsustvom oštećenja ciljnih organa povezanih sa mijelomom plazma ćelija (hiperkalcemija, bubrežna insuficijencija, anemija ili lezije kostiju) i odsustvom limfoma B-ćelija ili drugih bolesti sa visokom koncentracijom M-proteina. MGUS se generalno smatra preneoplastičnim poremećajem koji ne napreduje uvek u očigledan malignitet. Jedan od mogućih scenarija je veoma redak "POEMS" sindrom, koji se manifestuje polineuropatijom, organomegalijom, endokrinopatijom, monoklonalnom gamopatijom i promenama na koži. U ovoj publikaciji opisujemo patofiziologiju, prezentaciju, evaluaciju monoklonalne gamopatije neutvrđenog značaja kod pacijenta sa perifernom demijelinizirajućom polineuropatijom, simptomima hroničnog umora, erektilnom disfunkcijom i smanjenim libidom i ističemo ulogu multidisciplinarnog pristupa i endokrinoloških manifestacija MGUS. [ABSTRACT FROM AUTHOR]

The increased secretion of thyroid gland hormones affects the cardiovascular system by increasing heart rate and often by increasing systolic and diastolic blood pressure. We examined the influence of elevated thyroid hormone on blood pressure. Blood pressure monitoring was performed prior to the introduction of therapy in people with increased FT4 and on therapy when FT4 was in the normal range. We analyzed 32 people, of which 26 women had normal blood pressure values measured by blood pressure monitoring. Average age 45 and body mass index 27 kg/m2. Blood pressure was measured by monitoring blood pressure for 24 hours. On average, before the introduction of the therapy, it was 133/83 mmHg P 96 / min. The blood pressure on average on therapy with tireosuppressive was 128/82 mmHg P 74 / min. The Wilcoxon-Mann-Whitney paired test shows a significant P

Female pseudohermaphroditism represents discrepancy between karyotype and gonadal features on one side and a psychogenic phenotype on the other. Congenital adrenal hyperplasia is the part of the spectrum of female pseudohaermaofroditism and is due to an enzyme deficiency in steroidogenesis. More than 95% of patients have 21 hydroxylase deficiency which leads to a lack of cortisol and ACTH hypersecretion of pituitary, overproduction of 17 hydroxy progesterone (17OHP) and androgens and adrenal cortex hyperplasia. The clinical phenotype is classified as classical and nonclassical (aka. 'Late onset' form). The classic form is represented as salt-waisting and simply virilizing, depending on the degree of lack of aldosterone. Pathophysiology of CAH due to the lack of 21α hydroxylase is closely associated with the degree of enzyme deficiency. Overproduction of androgens is leading to accelerated virilisation. Classical form is manifested in childhood and is characterized by the overproduction of cortisol precursors and adrenal androgens. In the most severe form, co-aldosterone deficiency leads to loss of salt with all the complications. Girls with the classical form of CAH typically have am ambiguous genitals at birth due to high concentrations of androgens in utero. CAH due to 21OH deficiency is the most common cause of ambiguous genitals in 46XX newborns. Characteristically, the clitoris is enlarged, partially fused labia maiora and a common urogenital sinus at the site of the urethra and vagina. The uterus, Fallopian tubes and ovaries are present and normal, a structures of Wolfian duct are absent. When diagnosed in childhood 46XX CAH patients has been assigned female gender so far, even in fully expressed in virilised external genitalia. This dogmatic approach is based on preserving fertility, and if there was at least uterus, opting for female sex was considered justified. Only about 5% of 46 patients with XX KAH has a psychogenic male gender, as described patient. In addition to mutations of CYP21A2 and effects of high concentration of androgen in the CAH, in the phenotypic expression of CAH different length of sequence of CAG androgen receptor could have an impact, which modulates the effect of androgens on the periphery. Also, while the cases of adaptation of sexual identity and the external genitalia from female to male were documented, not a single case of questionable male sexual identity is noted. In the presented patient testosterone levels are at the upper limit for men, which may be due to enzymatic block and synthesis of cortisol precursor, which is further metabolized to the active androgen: testosterone and dihydrotestosterone. High concentrations of T prenatally and in infancy has led to premature fusion epiphysis and low growth, and high concentrations of DHT to clitoromegaly. The LHRH test showed no stimulatory response of LH, which is probably suppressed by high concentrations of testosterone , but there still needs to be done endocraniaol MNR to rule out other possible etiologies of gonadotroph suppression. Dexamethasone suppression test with measurement of testosterone could give an answer. The treatment of adult patients is based on hydrocortisone in dosages that provide permanent substitution of cortisol and suppress ACTH hypersecretion. In 46 XX patients who are phenotypically male, and whose full identity is male, reconstructive surgical procedures are needed to enable adequate quality of life.

OBJECTIVE: The aim of the study was to assess whether sudden change in blood glucose concentration in healthy subject would produce insulin secretory pattern similar to one in type 2 diabetic subjects (T2D). DESIGN: The concept of the study was to 'reset' the pancreas and empty the stored insulin pool, so that subtle derangements of the pulsatile insulin secretion could be studied. METHODS: The study group included 6 control and 7 T2D subjects. For insulin and glucose determination, blood was sampled for 60 minutes with 2 minutes inter-sample interval. To increase blood glucose an intravenous glucose bolus was given (0.3 g/kg body weight). RESULTS: The average glucose level in the T2D group was significantly higher, but the average insulin concentration was not different between the groups. Insulin half-life, pulse amplitude, number of pulses and interpulse interval did not differ between the groups. CONCLUSIONS: Therefore, the sudden increase in blood glucose causes insulin secretory pattern to become similar in the healthy and in the T2D subjects. However, the defect in the insulin secretion or glucose sensing inherent to β-cell exists, as the insufficient quantity of insulin is secreted in response to the increased glucose level.

Epileptic seizures are defined as the transient appearance of signs or symptoms due to excessive or synchronous neuronal activity in the cerebral cortex. Pheochromocytomas and paragangliomas (PPGL) are tumors of chromaffin cells that arise from the medulla of the adrenal gland in 80­85% of patients and from the extra ­adrenal sympathetic tissue of the abdomen, pelvis and chest in 10­20% of patients. The clinical picture of PPGL is variable and ranges from the absence of symptoms to severe clinical picture, depending on the biochemical profile. They are most often manifested by paroxysmal hypertension, followed by episodes of severe headache or diaphoresis, while epileptic attacks are rare. Neurological symptoms are present in many patients with PPGL. Also, paroxysmal neurological conditions such as vasodilating headache, intracranial tumors, diencephalic ­autonomic epilepsy, hypertensive encephalopathy, focal arterial disease of the brain and anxiety state have been described, which may have similar clinical manifestations with pheochromocytomas. We present a 44­year­old woman, who has been diagnosed with pheochromocytoma as possible etiological basis of epileptic seizures. Pheochromocytoma, with its low incidence and “chameleon” clinical spectrum, should be considered as a potential etiological factor of convulsions. [ABSTRACT FROM AUTHOR]

Granulomatosis with polyangiitis (GPA), formerly known as Wegener’s granulomatosis, is a necrotizing vasculitis of small and medium-sized blood vessels characterized by diffuse inflammation of vascular structures and perivascular and extravascular granulomatosis. In its systemic form, GPA predominantly affects the ear, nose and throat, with lung and kidney involvement with typically rapidly progressive necrotizing glomerulonephritis with extracapillary crescents, while the absence of kidney damage at the time of diagnosis is defined as a limited form of GPA with a more favorable prognosis (1, 2). Antineutrophil cytoplasmic antibodies (c-ANCA) with specificity for proteinase 3 (PR3) represent a biochemical diagnostic criterion. They are detected in 90% of generalized forms and in about 50% of limited forms of granulomatosis with polyangiitis (1, 2). In the absence of treatment, GPA is a disease of progressive evolution. Systemic corticosteroid therapy and immunosuppressive therapy significantly changed the prognostic aspect of the disease. Only a few sporadic observations have been published on endocrine disorders associated with GPA. We present a case of a man, 39 years old, with Wegener’s granulomatosis who developed autoimmune thyroiditis 8 years after the initial diagnosis. [ABSTRACT FROM AUTHOR]

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There are three basic modalities for the treatment of thyrotoxicosis: thyrosuppresive drug therapy, ablation with radioactive iodine and surgical treatment. Patients who do not achieve adequate thyrotoxicosis control, as was the case of described patient, have a high mortality rate due to the possibility of developing a thyroid storm. The use of drug therapy for hyperthyroidism, as the first line of treatment, is associated with the appearance of various side effects, as was the case in our patient. Side effects of Methimazole are dose-dependent, while in the case of Propylthiouracil, the occurrence of side effects is not clearly dose-dependent. In the case of the described patient, all alternative, lesser known modalities for the treatment of hyperthyroidism were applied, after the occurrence of adverse reactions to thyrosuppressive therapy. Sodium perchlorate, ie. Sodium with perchloric acid is rarely used in the treatment of hyperthyroidism, as in cases of severe idiosyncratic reactions to thionamides, agranulocytosis or hepatitis, if the eumetabolic state is not achieved and the application of a therapeutic dose of radioiodine is not possible. It is applied in the form of a solution, usually 8%; In more severe forms of the disease, when hyperthyroidism is very pronounced, 10 to 15 drops a day are given 4 to 6 times and the dose is sometimes reduced to the minimum maintenance dose. After the adverse reaction even to sodium perchlorate therapy we were left with one more, last option- Plasma Therapy exchange. Plasma Therapy Exchange (TPE) is an out-of-body blood purification technique designed to remove high-molecular-weight substances bound to plasma proteins (autoantibody pathogens, immunocomplexes, cryoglobulins, myeloma light chains, endotoxins, lipoprotein-containing cholesterol, and thyroid). The effectiveness of treatment depends on the volume of blood being processed, the volume of plasma exchanged in each process, the number of procedures performed, the frequency of exchange and the rate of mobilization, stabilization and re-synthesis of cells or plasma components. TPE is an effective alternative treatment that provides an opportunity to prepare patients for definitive treatment: ablative therapy such as RAI ablation or thyroidectomy. Therapeutic plasmapheresis, if performed in specialist centers, is a safe, fast and effective method. [ABSTRACT FROM AUTHOR]