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2.
Artigo em Inglês | MEDLINE | ID: mdl-32612575

RESUMO

Background: The mutation frequencies of pituitary transcription factors genes in patients with combined pituitary hormone deficiencies (CPHD) vary substantially between populations. However, apart from PROP1 the mutation rate of other genes is low and for almost half of the patients with CPHD the routine sequencing of known genes is unsuccessful in the identification of genetic causes. Methods: A cohort of 66 sporadic and nine familial CPHD cases (80 patients in total) were subjected to initial testing of the genes PROP1, POU1F1, LHX3, LHX4, and HESX1 using a targeted gene panel and MLPA. In patients who tested negative, a whole exome sequencing approach was employed. Results: In nine of the familial cases and 32 of the sporadic patients mutations in the PROP1 gene were found (the common pathogenic variants included c.301_302delAG and c.150delA). Mutations were also found in genes so far not related directly to CPHD. A unique homozygous and clinically relevant variant was identified in the SEMA3A gene, which may contribute to neural development and his phenotypic spectrum including short stature and isolated hypogonadotropic hypogonadism (IHH). Another pathogenic variant p.A1672T was found in the IGSF10 gene reported to be responsible for delayed puberty and neuronal migration during embryogenesis. Several suspected novel but predicted benign variants were also identified for the CHD7, WDR11 and FGF17 genes. Conclusion: Although PROP1 defects account for a majority of CPHD patients, identification of rare, less frequent variants constitutes a big challenge. Multiple genetic factors responsible for CPHD are still awaiting discovery and therefore the usage of efficient genomic tools (i.e., whole exome sequencing) will further broaden our knowledge regarding pituitary development and function.

3.
J Clin Endocrinol Metab ; 105(3)2020 Mar 01.
Artigo em Inglês | MEDLINE | ID: mdl-31665449

RESUMO

CONTEXT: Urine steroid metabolomics, combining mass spectrometry-based steroid profiling and machine learning, has been described as a novel diagnostic tool for detection of adrenocortical carcinoma (ACC). OBJECTIVE, DESIGN, SETTING: This proof-of-concept study evaluated the performance of urine steroid metabolomics as a tool for postoperative recurrence detection after microscopically complete (R0) resection of ACC. PATIENTS AND METHODS: 135 patients from 14 clinical centers provided postoperative urine samples, which were analyzed by gas chromatography-mass spectrometry. We assessed the utility of these urine steroid profiles in detecting ACC recurrence, either when interpreted by expert clinicians or when analyzed by random forest, a machine learning-based classifier. Radiological recurrence detection served as the reference standard. RESULTS: Imaging detected recurrent disease in 42 of 135 patients; 32 had provided pre- and post-recurrence urine samples. 39 patients remained disease-free for ≥3 years. The urine "steroid fingerprint" at recurrence resembled that observed before R0 resection in the majority of cases. Review of longitudinally collected urine steroid profiles by 3 blinded experts detected recurrence by the time of radiological diagnosis in 50% to 72% of cases, improving to 69% to 92%, if a preoperative urine steroid result was available. Recurrence detection by steroid profiling preceded detection by imaging by more than 2 months in 22% to 39% of patients. Specificities varied considerably, ranging from 61% to 97%. The computational classifier detected ACC recurrence with superior accuracy (sensitivity = specificity = 81%). CONCLUSION: Urine steroid metabolomics is a promising tool for postoperative recurrence detection in ACC; availability of a preoperative urine considerably improves the ability to detect ACC recurrence.

4.
Pol Arch Intern Med ; 129(12): 874-882, 2019 12 23.
Artigo em Inglês | MEDLINE | ID: mdl-31808753

RESUMO

INTRODUCTION: Long­term glucocorticoid (GC) therapy is the most common cause of secondary adrenal insufficiency (AI), which undiagnosed may lead to life­threatening adrenal crisis. OBJECTIVES: The aim of the study was to evaluate AI in patients treated long­term with GCs, receiving a low maintenance dose (≤5 mg of prednisone or equivalent), namely, its prevalence and persistence, risk factors, and diagnostic accuracy of morning cortisol and dehydroepiandrosterone sulfate (DHEA­S) levels. PATIENTS AND METHODS: Adrenal function was evaluated in 40 patients before and after GC withdrawal and at least 1 year later. Based on morning cortisol levels and short Synacthen test, patients were divided into 3 groups: AI, intermediate (partial AI), and AS (adrenal sufficiency). Receiver operator characteristic curves were calculated to assess the diagnostic value of morning cortisol and DHEA­S levels before GC withdrawal. RESULTS: Before GC withdrawal, 42.5% of patients had AI or partial AI, which together persisted in 64.3% of those patients after withdrawal. After more than a year, the adrenal function returned to normal only in 14% of patients. Cushingoid feature occurred more often in the AI group compared with the AS group (60% vs 13%; P = 0.03). Morning cortisol levels of 14.91 µg/dl or higher (411 nmol/l) gave 100% negative predictive value to rule out AI. Morning cortisol of 6.51 µg/dl or less (179.6 nmol/l) gave 100% positive predictive value to rule in AI. DHEA­S proved to be a worse parameter for AI diagnosis. CONCLUSIONS: AI is common in patients treated with GCs and may persist for years after GC withdrawal. Cushingoid features are associated with a higher risk of AI. Morning cortisol levels may facilitate AI diagnosis.

5.
Endokrynol Pol ; 70(6): 469-472, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31529458

RESUMO

INTRODUCTION: Extraperitoneal, abdominal paragangliomas most commonly originate from the sympathetic nervous system. Typical features include catecholamine overproduction and the potential for malignancy. Lesions are usually located paravertebrally, but when growing in a more expansive manner they may also appear between the inferior vena cava and aorta. In the authors' opinion this site excludes laparoscopic tumourectomy. MATERIAL AND METHODS: Twenty-eight patients were selected for surgical management of abdominal paragangliomas in the past eight years at our endocrine surgical centre. This group consisted of 21 (75%) women and seven (25%) men, aged 14 to 84 years (mean 47.9). In 13 (46.4%) cases paroxysmal hypertension was observed. Type 2 diabetes was noted in another 10 (35.7%) patients, and Takotsubo acute coronary syndrome in two (7.1%). Patients were preoperatively qualified for either open surgery or laparoscopic tumourectomy based on visualisation and location of the tumours in imaging studies. RESULTS: All patients were successfully operated. Eleven (39.3%) patients qualified for laparoscopy, while the remaining 17 (60.7%) were treated with an open surgical approach due to difficult access to the lesion. The mean operative time was 130 minutes for laparoscopy and 120 minutes for laparotomy (p = 0.2). There were no local or general complications after either type of procedure. CONCLUSIONS: The use of laparoscopic access is practically excluded in the treatment of paragangliomas located between the inferior vena cava and aorta, especially at the level of the renal vessels and extending superiorly to the diaphragm.

6.
J Clin Endocrinol Metab ; 104(2): 312-318, 2019 02 01.
Artigo em Inglês | MEDLINE | ID: mdl-30383267

RESUMO

Background: Up to 7% of all adrenal incidentalomas (AIs) are pheochromocytomas (PCCs). In the evaluation of AI, it is generally recommended that PCC be excluded by measurement of plasma-free or 24-hour urinary fractionated metanephrines. However, recent studies suggest that biochemical exclusion of PCC not be performed for lesions with CT characteristics of an adrenocortical adenoma (ACA). Aim: To determine the proportion of PCCs with ACA-like attenuation or contrast washout on CT. Methods: For this multicenter retrospective study, two central investigators independently analyzed the CT reports of 533 patients with 548 histologically confirmed PCCs. Data on tumor size, unenhanced Hounsfield units (HU), absolute percentage washout (APW), and relative percentage washout (RPW) were collected in addition to clinical parameters. Results: Among the 376 PCCs for which unenhanced attenuation data were available, 374 had an attenuation of >10 HU (99.5%). In the two exceptions (0.5%), unenhanced attenuation was exactly 10 HU, which lies just within the range of ≤10 HU that would suggest a diagnosis of ACA. Of 76 PCCs with unenhanced HU > 10 and available washout data, 22 (28.9%) had a high APW and/or RPW, suggestive of ACA. Conclusion: Based on the lack of PCCs with an unenhanced attenuation of <10 HU and the low proportion (0.5%) of PCCs with an attenuation of 10 HU, it seems reasonable to abstain from biochemical testing for PCC in AIs with an unenhanced attenuation of ≤10 HU. The assessment of contrast washout, however, is unreliable for ruling out PCC.


Assuntos
Neoplasias das Glândulas Suprarrenais/diagnóstico por imagem , Feocromocitoma/diagnóstico por imagem , Neoplasias das Glândulas Suprarrenais/patologia , Adulto , Idoso , Meios de Contraste , Diagnóstico Diferencial , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Feocromocitoma/patologia , Estudos Retrospectivos , Tomografia Computadorizada por Raios X/métodos
7.
Int J Endocrinol ; 2018: 1978590, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-30420883

RESUMO

Purpose: Intravenous methylprednisolone (IVMP) pulse therapy is the first-line treatment in active moderate-to-severe Graves' orbitopathy (GO) and dysthyroid optic neuropathy (DON). One of the adverse effects of this therapy is liver dysfunction that can be mild (ALT < 100 U/L), moderate (ALT: 100-300 U/L), and severe defined as acute liver injury (ALI) (ALT > 300 U/L). ALI can be irreversible and fatal. The aim of the study was to evaluate the influence of two different schemes of therapy with IVMP in moderate-to-severe GO and DON on biochemical liver parameters. Materials and Methods: 49 patients with moderate-to-severe GO were treated with IVMP in every week schedule (cumulative dose 4.5 g), and 19 patients with DON received 3.0 g IVMP (1.0 g/day for 3 consecutive days). AST, ALT, and total bilirubin were measured before treatment and after IVMP in the following selected pulses: after 0.5 g (A1), 3.0 g (A2), and 4.5 g (A3) in the group with moderate-to-severe GO and after 3.0 g IVMP in the group with DON (B1). Results: We observed a statistically higher level of AST and ALT after therapy with 3.0 g of IVMP (B1) than after 0.5 g (A1), 3.0 g (A2), and 4.5 g of IVMP (A3). Mild elevation of ALT was found in 4% and 11% of patients with moderate-to-severe GO and DON, respectively. Moderate elevation of ALT was found in 0% and 21% of patients with moderate-to-severe GO and DON, respectively. There were no cases of ALI. Conclusion: Therapy of GO with higher doses (1.0 g) of IVMP in consecutive days is associated with higher risk of liver damage than treatment with moderate doses (≤0.5 g) in every week schedule. This trial is registered with NCT03667157.

8.
Wideochir Inne Tech Maloinwazyjne ; 13(3): 283-287, 2018 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-30302139

RESUMO

Introduction: Lateral transabdominal adrenalectomy (LTA) is the most common minimally invasive technique used to treat patients with adrenal tumors. Aim: To analyze intra-operative and post-operative complications and reasons for conversion to open surgery in patients who underwent LTA and had previous abdominal surgery. Material and methods: Five hundred and nineteen patients underwent LTA in our center between 2005 and 2016. We identified a study group of 150 patients, with previous abdominal surgery. We analyzed the frequency of intra-operative and post-operative complications and the reasons for conversion from laparoscopic to open adrenalectomy. Results: The patients' mean age was 58; they underwent LTA due to hormonally active tumors (n = 79, 53%) and non-functioning adrenal tumors (n = 71, 47%). The size of adrenal lesions ranged from 20 mm to 90 mm. Seventy-eight (52%) adrenal lesions were found in the right adrenal gland, and 72 (48%) lesions in the left adrenal gland. The mean operating time was 130 min. The mean stay in hospital was five days. The intra-operative complications included blood pressure fluctuations (n = 32), abnormal vascular supply of the adrenal glands causing difficulties with dissections (n = 3), and respiratory problems (n = 1). Two (1.3%) patients had post-operative bleeding at the site of removed adrenal glands; 1 patient had an exacerbation of asthma postoperatively. Of the 150 patients analyzed, 3 (2%) required conversion to open adrenalectomy. The conversions were not caused by abdominal adhesions. Conclusions: Lateral transabdominal adrenalectomy is feasible and safe in patients with previous abdominal surgery. In our study, conversion from laparoscopic to open adrenalectomy was not caused by abdominal adhesions.

9.
Wideochir Inne Tech Maloinwazyjne ; 13(3): 288-291, 2018 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-30302140

RESUMO

Introduction: Adrenal cysts develop in up to about 0.2% of the overall population. They may account for up to 11% of all pathologies of adrenal glands. Aim: Is laparoscopic resection of adrenal cysts a method for the treatment of these pathologies? Material and methods: In the years 2010-2017, a total of 27 patients underwent surgery due to adrenal cysts; those included 18 (66.7%) women and 9 (33.3%) men aged 29 to 84 years (mean age: 42.7). Cyst diameter ranged from 55 to 130 mm. After exclusion of hormonal hyperactivity, parasitic cysts, or, to the best possible extent, cancer lesions, patients were qualified for adrenal-sparing laparoscopic surgery. Results: All patients were subjected to laparoscopic surgery. Cystic wall resection was performed in 15 (55.6%) patients while adrenalectomy was performed in the remaining 12 (44.4%) patients. The decision regarding the extent of the surgery was made intraoperatively. Histopathological assessment revealed pathological adrenal lesions in as few as 3 (11.1%) patients, with the rest of the study population, i.e. 24 (88.9%), presenting with normal adrenal tissue. Conclusions: Laparoscopic resection of adrenal cysts appears to be recommendable as a method for the treatment of these pathologies. It is simpler than adrenalectomy and associated with low risk of any pathological lesion remaining within the adrenal gland following careful intraoperative assessment by an experienced surgeon.

10.
Gynecol Endocrinol ; 34(12): 1022-1026, 2018 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-30129786

RESUMO

Hypercortisolemia in females may lead to menstrual cycle disturbances, infertility, hirsutism and acne. Herewith, we present a 18-year-old patient, who was diagnosed due to weight gain, secondary amenorrhea, slowly progressing hirsutism, acne and hot flashes. Thorough diagnostics lead to a conclusion, that the symptoms was the first manifestation of primary pigmented nodular adrenocortical disease (PPNAD). All symptoms of Cushing syndrome including hirsutism and menstrual disturbances resolved after bilateral adrenalectomy. Our report indicates that oligo- or amenorrhea, hirsutism, acne in combination with weight gain, growth failure, hypertension and slightly expressed cushingoid features in a young woman requires diagnostics towards hypercortisolemia. Despite PPNAD is a very rare cause of ACTH-independent Cushing syndrome, it has to be taken into consideration, especially when adrenal glands appear to be normal on imaging and paradoxical rise in cortisol level in high-dose dexamethasone test is observed. Unlike in our patient, in vast majority of patients, PPNAD is associated with Carney complex (CC). Therefore, these patients and their first-degree relatives should be always carefully screened for symptoms of PPNAD, CC and genetic mutations of PRKAR1A, PDE11A, and PDE8B genes.


Assuntos
Doenças do Córtex Suprarrenal/diagnóstico , Amenorreia/etiologia , Hirsutismo/etiologia , Adolescente , Doenças do Córtex Suprarrenal/complicações , Doenças do Córtex Suprarrenal/patologia , Doenças do Córtex Suprarrenal/cirurgia , Glândulas Suprarrenais/patologia , Adrenalectomia , Feminino , Humanos
11.
Endokrynol Pol ; 69(4): 411-415, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-29952409

RESUMO

INTRODUCTION: To assess the effectiveness of early unilateral laparoscopic adrenalectomy in ACTH-independent and subclinical hypercor-tisolaemia. MATERIAL AND METHODS: We conducted a unicentric, retrospective study. Between 2010 and 2015, 356 laparoscopic adrenalectomies were performed in the Department of General and Endocrine Surgery of the MUW. Hypercortisolaemia was found in 50 (14%) patients, while overt hypercortisolaemia was found in 31 patients. In the hypercortisolaemia group, ACTH-dependent hypercortisolaemia was diagnosed in five (10%) and ACTH-independent hypercortisolaemia in 25 patients (50%). One patient with overt hypercortisolaemia had cancer of the adrenal cortex. The remaining 19 (38%) patients had subclinical Cushing's syndrome. For our study, we compared patients with ACTH-independent hypercortisolaemia (n = 25) with those with Cushing's syndrome (n = 19). Patients with ACTH-dependent hyper-cortisolaemia (n = 5) and the patient with cancer of the adrenal cortex (n = 1) were excluded. RESULTS: Patients from both groups (n = 44) underwent a unilateral transperitoneal adrenalectomy. Good early outcomes were observed in 42 patients (93.3%). In one patient, an additional laparoscopic surgery was necessary on postoperative day 0 due to bleeding. In another patient, on day 22 post-surgery, we found an abscess in the site of the excised adrenal gland, which was drained under laparoscopic guid-ance. In three patients (6.8%) with substantial obesity, temporary respiratory insufficiency of varying degrees occurred. We did not observe any thromboembolic complications. All patients with overt hypercortisolaemia and nine patients with subclinical hypercortisolaemia had secondary adrenal insufficiency postoperatively. CONCLUSIONS: Transperitoneal unilateral laparoscopic adrenalectomy is an efficient and safe treatment option in patients with ACTH- -independent hypercortisolaemia, both overt and subclinical.


Assuntos
Adrenalectomia , Síndrome de Cushing/cirurgia , Laparoscopia , Hiperfunção Adrenocortical/cirurgia , Adulto , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Segurança do Paciente , Estudos Retrospectivos , Resultado do Tratamento
12.
Pol Arch Intern Med ; 128(6): 371-378, 2018 06 30.
Artigo em Inglês | MEDLINE | ID: mdl-29726479

RESUMO

Introduction Adrenocortical carcinoma (ACC) is a rare malignancy, associated with poor outcome and few therapeutic options. Despite increasing attention, the knowledge about the clinical course and treatment of these tumors is limited. Objectives Survival rates in ACC are still low and the percentage of relapse is high. Thus, it is crucial to identify the prognostic factors of overall survival (OS) and recurrence­free survival (RFS). Patients and methods This was a retrospective analysis of 66 patients diagnosed with ACC between 2002 and 2015. Results The median OS was 43.5 months, 78.19 months for stage I + II, 22.95 months for stage III, and 19.54 months for stage IV ACC. Older age, stage IV ACC, margin status R2, and no mitotane treatment were associated with poor OS. Low Ki67 and mitotic indices were related to improved OS in a univariate analysis. The median RFS was 101.1 months. Disease recurrence after potentially curative surgery was reported in 1 patient (25%) with stage I, 12 patients (46%) with stage II, and 9 patients (45%) with stage III ACC. Male sex and no mitotane treatment were associated with a reduced RFS in a multivariate analysis and higher Ki67 and mitotic indices in the univariate analysis. Conclusions Ki67 and mitotic indices should be considered as prognostic factors when planning the adjuvant treatment of ACC. Mitotane treatment may be independently associated with better outcomes regardless of the tumor stage.


Assuntos
Carcinoma Adrenocortical/cirurgia , Índice Mitótico , Carcinoma Adrenocortical/tratamento farmacológico , Carcinoma Adrenocortical/terapia , Adulto , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Mitotano/uso terapêutico , Polônia , Prognóstico , Estudos Retrospectivos , Taxa de Sobrevida
13.
J Obstet Gynaecol Res ; 44(6): 1118-1125, 2018 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-29517119

RESUMO

AIM: The diagnosis of biochemical hyperandrogenism is still challenging because a set of appropriate, recommended diagnostic tests has not been established. In our study, we aimed to answer the question of whether salivary testosterone is a reliable test to establish the diagnosis of biochemical hyperandrogenism as compared to serum total testosterone (TT) measured either by liquid chromatography-tandem mass spectrometry (LC-MS/MS) or immunoassay and to assess which set of biochemical tests would be the most appropriate for the identification of biochemical hyperandrogenism. METHODS: A total of 39 women, aged 18-45 years, with clinical or biochemical hyperandrogenism and 41 healthy individuals, aged 19-45 years, were enrolled in the study. Salivary testosterone was measured using the Salimetrics test. Serum TT was measured either using the LC-MS/MS method or immunoassay, and dehydroepiandrosterone sulphate (DHEA-S) and androstenedione were measured using LC-MS/MS. RESULTS: In 15 of 17 (88%) patients with elevated serum TT measured by LC-MS/MS and in 14 of 16 (87%) measured with immunoassay, salivary testosterone showed normal levels. In 11 of 39 women (28%) with normal serum testosterone levels, DHEA-S was elevated. All patients with elevated androstenedione presented with an elevated concentration of either serum testosterone or DHEA-S. CONCLUSION: Salivary testosterone measurement may lead to the underdiagnosis of biochemical hyperandrogenism. Both serum testosterone and DHEA-S should be measured in the endocrine work-up toward biochemical hyperandrogenism.


Assuntos
Sulfato de Desidroepiandrosterona/metabolismo , Hiperandrogenismo/diagnóstico , Hiperandrogenismo/metabolismo , Saliva/metabolismo , Testosterona/metabolismo , Adolescente , Adulto , Cromatografia Líquida , Sulfato de Desidroepiandrosterona/sangue , Feminino , Humanos , Hiperandrogenismo/sangue , Pessoa de Meia-Idade , Espectrometria de Massas em Tandem , Testosterona/sangue , Adulto Jovem
14.
Pol Merkur Lekarski ; 43(255): 129-132, 2017 Sep 29.
Artigo em Polonês | MEDLINE | ID: mdl-28987046

RESUMO

Immunoglobulin G4-related disease (IgG4-RD) is a comparatively new condition that may involve more than one organ. The lack of characteristic, pathognomonic clinical symptoms may delay the diagnosis of this disease. The diagnosis is based upon clinical manifestation, elevated serum levels of IgG4 and histopathologic examination with immunohistochemical staining to reveal infiltration of IgG4-positive plasma cells. The first line treatment is oral glucocorticoids. A CASE REPORT: 38-year-old woman with Hashimoto disease, chronic sinusitis and chronic hepatitis of unknown etiology was admitted to the Department of Endocrinology because of moderate eyelids swelling accompanied by redness for 3 years. Graves' orbitopathy and systemic vasculitis were suspected, however both were excluded (negative antibodies results: anty-TSHR, ANCA, ANA). Serologic investigation of Sjögren's syndrome was also negative. In Magnetic Resonance Imaging (MRI) of orbits there were described bilateral mild extension of lateral rectus muscles, normal signal of adipose tissue and bilateral lacrimal glands enlargement. Moreover, increased IgG4 serum levels were detected. The material derived from perinasal sinuses surgery was analyzed in histopathology examination with immunohistochemical staining, which revealed characteristic features of chronic inflammatory process and increased numbers of IgG4 - positive plasma cells (>50 in a large field of view). The diagnosis of IgG4-RD was established. Because of non-effective oral methylprednisolone therapy in the past, the patient was referred to Clinic of Rheumatology for further treatment. After the therapy with methylprednisolone and azathioprine there were observed the significant reduction of symptoms. CONCLUSIONS: Because of lack of characteristic symptoms of IgG4- RD, it should be always considered in differential diagnosis of chronic inflammatory diseases of various organs.


Assuntos
Doenças Autoimunes/complicações , Doença de Hashimoto/etiologia , Hepatite/etiologia , Imunoglobulina G , Sinusite/etiologia , Adulto , Doenças Autoimunes/diagnóstico , Doenças Autoimunes/tratamento farmacológico , Doenças Autoimunes/imunologia , Azatioprina/uso terapêutico , Quimioterapia Combinada , Feminino , Humanos , Metilprednisolona/uso terapêutico
15.
J Trace Elem Med Biol ; 44: 186-191, 2017 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-28965575

RESUMO

OBJECTIVES: Selenium (Se) deficiency is related to an increased risk of preterm labor, miscarriage, preeclampsia, gestational diabetes, and other obstetric complications. As the Se status declines during pregnancy, we hypothesized that the decline may be exacerbated in women with autoimmune thyroid disease (AITD). MATERIAL AND METHODS: Pregnant women (n=74; 30 [23-38] years) were consecutively recruited from the district of Warsaw, Poland, and divided into healthy subjects (HS, n=45), and women with a diagnosis of AITD (AITD, n=29). Thyroglobulin antibodies (TG-aAb), thyroid peroxidase antibodies (TPO-aAb), TSH, free T3, free T4, total T3, and total T4, as well as urine iodine excretion were determined. Se status was assessed by serum Se and selenoprotein P (SELENOP) concentrations. Thyroid volume was evaluated by ultrasonography. RESULTS: Serum Se and SELENOP concentrations were relatively low in both control and AITD women. A Se deficit according to WHO definition (<45µg/l) was observed in 0%, 3.4%, 28.6% and 4.5%, 18.2%, 35.5% of women in the AITD and HS group, respectively, during the 1st, 2nd, and 3rd trimester. From first to third trimester, TPO-aAb and TG-aAb declined in AITD by 71% and 60%, respectively. The decline in TPO- and TG-aAb was unrelated to the Se status. CONCLUSIONS: In this area of habitual low Se intake, a high proportion of women developed a severe Se deficit during pregnancy, irrespective of AITD status. This decline must be considered as a preventable risk factor for pregnancy complications of relevance to both the unborn child and the pregnant mother.


Assuntos
Doenças Autoimunes/sangue , Selênio/deficiência , Doenças da Glândula Tireoide/sangue , Adulto , Estudos de Casos e Controles , Feminino , Humanos , Recém-Nascido , Polônia , Gravidez , Trimestres da Gravidez/sangue , Selênio/sangue , Selenoproteína P/metabolismo
16.
Kardiol Pol ; 75(11): 1192-1198, 2017.
Artigo em Inglês | MEDLINE | ID: mdl-28715066

RESUMO

BACKGROUND: Adrenalectomy with preoperative pharmacological preparation is strongly recommended in patients diagnosed with pheochromocytoma, in order to prevent perioperative complications. AIM: To compare phenoxybenzamine (PhB) and doxazosin (DOX) in terms of perioperative haemodynamic status in patients with pheochromocytoma, who have been prepared for adrenalectomy. METHODS: Retrospective analysis of 44 patients with pheochromocytoma (aged 16-80 years, 29 females) who underwent adrenalectomy. Patients were divided into two groups: 35 patients on DOX and nine patients on PhB. RESULTS: Mean time of preparation for surgery was 38.8 days in the DOX group and 18.3 days in the PhB group (p = 0.04). No statistically significant differences between the DOX and PhB groups in intraoperative blood pressure (BP) fluctuations were found: < 170/100 mm Hg (34% vs. 44%, respectively, p = 0.42), ≥ 200/110 mm Hg (40% vs. 22%, respectively, p = 0.28). Mean greatest intraoperative systolic BP (195 ± 53 vs. 166 ± 42 mm Hg, p = 0.21) and diastolic BP (98 ± 20 vs. 89 ± 46 mm Hg, p = 0.21), and mean lowest intraoperative systolic BP (87 ± 13 vs. 79 ± 17 mm Hg, p = 0.25) and diastolic BP (49 ± 8 vs. 46 ± 12 mm Hg, p = 0.60) were not different between the DOX and PhB groups, respectively. Sodium nitroprusside was administrated in 30% DOX vs. 11% PhB patients (p = 0.25). Laparoscopic surgery was conducted in 97% DOX vs. 89% PhB patients (p = 0.64). Postoperative BP drop below 90/60 mm Hg was noted in 48% of the DOX vs. 43% of the PhB group (p = 0.56). Negative correlation was found between the length of DOX administration with maximal intraoperative systolic BP (r = -0.45, p = 0.006) and diastolic BP (r = -0.39, p = 0.019). CONCLUSIONS: There are no clinically relevant differences between patients with pheochromocytoma, who have been prepared for adrenalectomy with DOX or PhB.


Assuntos
Pressão Sanguínea/efeitos dos fármacos , Gerenciamento Clínico , Doxazossina/uso terapêutico , Fenoxibenzamina/uso terapêutico , Feocromocitoma/cirurgia , Complicações Pós-Operatórias/prevenção & controle , Adrenalectomia , Adulto , Idoso , Idoso de 80 Anos ou mais , Anti-Hipertensivos , Doxazossina/farmacologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Fenoxibenzamina/farmacologia , Estudos Retrospectivos , Adulto Jovem
17.
Endokrynol Pol ; 68(4): 430-433, 2017.
Artigo em Inglês | MEDLINE | ID: mdl-28604944

RESUMO

INTRODUCTION: Graves' orbitopathy (GO) is the commonest extrathyroidal feature of Graves' disease. For active, moderate to severe GO intravenous glucocorticoid pulse therapy (ivGCS) is the first-line treatment, which might be followed by oral glucocorticoid therapy. Glucocorticoid treatment, especially in the time of withdrawal, carries a risk of adrenal insufficiency. The aim of this study was to assess the pituitary-adrenal axis function in patients with GO before and at the cessation of ivGCS, and after further oral glucocorticoid therapy. MATERIAL AND METHODS: Twenty patients received treatment in accordance with the EUGOGO protocol (500 mg methylprednisolone once a week for six weeks, then 250 mg once a week for another six weeks) followed by oral prednisone at a gradually decreasing dose from 30 mg/day over a three-month period. Adrenal function was evaluated directly before the ivGCS, before the last pulse, and after oral glucocorticoid intake. The assessment consisted of clinical evaluation, and measurements of morning total serum cortisol (TSC) and plasma adrenocorticotropic hormone (ACTH). Patients with decreased cortisol level underwent ACTH stimulation test with TSC measurements before, and 30 minutes and 60 minutes after the administration of synthetic ACTH. RESULTS: All patients before and at the cessation of ivGCS treatment demonstrated intact adrenal function. One patient after prednisone therapy presented decreased adrenal reserve. TSC concentration was significantly lower after the ivGCS (p = 0.025) and oral glucocorticoid therapy (p = 0.0006) in comparison to evaluation before therapy. CONCLUSIONS: Therapy with ivGCS for GO does not lead to secondary adrenocortical insufficiency. Further low-dose oral glucocorticoid therapy may result in secondary adrenocortical insufficiency in some patients.


Assuntos
Insuficiência Adrenal/induzido quimicamente , Glucocorticoides/efeitos adversos , Oftalmopatia de Graves/tratamento farmacológico , Metilprednisolona/efeitos adversos , Prednisona/efeitos adversos , Administração Intravenosa , Insuficiência Adrenal/sangue , Hormônio Adrenocorticotrópico/sangue , Hormônio Adrenocorticotrópico/efeitos dos fármacos , Adulto , Idoso , Quimioterapia Combinada , Feminino , Glucocorticoides/administração & dosagem , Glucocorticoides/uso terapêutico , Humanos , Hidrocortisona/sangue , Masculino , Metilprednisolona/administração & dosagem , Metilprednisolona/uso terapêutico , Pessoa de Meia-Idade , Sistema Hipófise-Suprarrenal/efeitos dos fármacos , Prednisona/administração & dosagem , Prednisona/uso terapêutico , Adulto Jovem
18.
Endokrynol Pol ; 68(4): 407-410, 2017.
Artigo em Inglês | MEDLINE | ID: mdl-28585682

RESUMO

INTRODUCTION: Adrenal neoplasms comprise about 10% of all tumours affecting this organ and constitute a significant, at first diagnostic and subsequently therapeutic, problem, especially since a relatively high proportion of neoplastic lesions are asymptomatic. The number of diagnosed metastases to adrenal glands is increasing. Surgical treatment involves both open surgery as well as laparoscopy. MATERIAL AND METHODS: There were 245 adrenalectomies performed at our centre due to various indications over the past four years. In 27 (11.5%) cases neoplasms were diagnosed in the final histopahtological examination. In 11 (40.7%) cases primary adrenal cortex tumours were diagnosed, metastases from other solid organ tumours were identified in another 12 (44.4%) patients, and rarer neoplasms were diagnosed in the remaning 4 (14.8%) subjects. Cases of malignant pheochromocytoma were not included in this report. RESULTS: Laparoscopic adrenalectomy was performed in 23 (85.2%) subjects, while the ramaining 4 (12.9%) patients were subject to open adrenalectomy (conversion to open procedure in one case). There were no deaths or significant complications in the perioperative period. Comparing mean duration of open (140 minutes) and laparoscopic (190 minutes) procedures yielded a statistically significant difference (p = 0.02). There was no statistically significant difference found in the duration of operation with regard to laparoscopic adrenalectomies of tumours less than 50 mm and over 55 mm in diameter (p = 0.16). CONCLUSIONS: Laparoscopic adrenalectomy is a safe and effective method of treatment of adrenal tumours. Its oncological completeness is comparable (to open surgery) when performed by experienced surgical teams. Laparoscopy is the reason for the smooth postoperative course observed in most patients.


Assuntos
Neoplasias das Glândulas Suprarrenais/cirurgia , Adrenalectomia , Laparoscopia , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Segurança do Paciente , Resultado do Tratamento
20.
Ginekol Pol ; 88(1): 5-8, 2017.
Artigo em Inglês | MEDLINE | ID: mdl-28157253

RESUMO

OBJECTIVES: Total testosterone/dihydrotestosterone ratio (TT/DHT) was found to determine metabolic risk in polycystic ovary syndrome (PCOS). The aim of this study was to analyze whether (TT/DHT) may be helpful in predicting metabolic risk not only in PCOS patients but also in healthy women. MATERIAL AND METHODS: Total testosterone (TT), dihydrotestosterone (DHT), androstendione and dehydroepiandrosterone sulphate (DHEA-S) were measured by LC-MS/MS in 36 women with PCOS and in 29 age-matched controls without clinical hyperandrogenism. In all participants, anthropometric data, lipids, adipose tissue percent (%fat), HOMA-IR were also assessed. RESULTS: The studied groups were not different in terms of age, BMI, waist circumference, %fat and HOMA-IR. In the patients group, mean TT and androstendione levels were significantly higher as compared to controls (1.4 nmol/L vs. 1.0 nmol/L, P < 0.001) and (6.6 nmol/L vs. 4.9 nmol/L, P < 0.01), respectively. In the patients group, mean TT/DHT ratio was significantly higher compared to controls (3.6 vs. 2.7, P < 0.01) and correlated with BMI (r = 0.37, P < 0.05), waist circumference (r = 0.44, P < 0.01), %fat (r = 0.30, P < 0.05), as well as with insulin levels (r = 0.38, P < 0.05) and HOMA-IR (r = 0.44, P < 0.05). The association between TT/DHT ratio and unfavorable metabolic parameters was also seen in controls. CONCLUSION: Total testosterone/dihydrotestosterone ratio assessed by LC-MS/MS correlates with a worse metabolic profile not only in PCOS patients, but also in healthy women.


Assuntos
Tecido Adiposo , Androstenodiona/sangue , Sulfato de Desidroepiandrosterona/sangue , Di-Hidrotestosterona/sangue , Resistência à Insulina , Insulina/sangue , Síndrome do Ovário Policístico/sangue , Testosterona/sangue , Adolescente , Adulto , Composição Corporal , Índice de Massa Corporal , Estudos de Casos e Controles , Cromatografia Líquida , Feminino , Humanos , Prognóstico , Espectrometria de Massas em Tandem , Circunferência da Cintura , Adulto Jovem
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