Publications by authors named "Alicja Hubalewska-Dydejczyk"

170 Publications

Diversity of Pathological Conditions Affecting Pituitary Stalk.

J Clin Med 2021 Apr 14;10(8). Epub 2021 Apr 14.

Department of Endocrinology, Faculty of Medicine, Medical College, Jagiellonian University, 31-008 Krakow, Poland.

Pituitary stalk lesions (PSL) are a very rare pathology. The majority of conditions affecting the infundibulum do not present with clinically apparent symptoms, what makes the diagnosis difficult. The recognition might be also complicated by the non-specific and transient characteristics of hormonal insufficiencies. In our study, we retrospectively analysed demographic, biochemical, and clinical long-term data of 60 consecutive, unselected adult patients (34 women and 26 men) with PSL diagnosed in the Department of Endocrinology, Jagiellonian University in Krakow. The diagnosis of PSL were categorized as confirmed, probable, or undetermined in 26, 26 and 8 patients, accordingly. Given the possible aetiology congenital, inflammatory, and neoplastic stalk lesions were diagnosed in 17, 15 and 20 patients, accordingly. In eight cases the underlying pathology remained undetermined. The most common pituitary abnormality was gonadal insufficiency diagnosed in 50.8% of cases. Diabetes insipidus was detected in 23.3% of cases. In 5% of patients the pituitary function recovered partially over time. Stalk lesions were extensively discussed in the context of the current literature. Based on the published data and our own experience a diagnostic algorithm has been proposed to help physicians with the management of patients with this challenging condition.
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http://dx.doi.org/10.3390/jcm10081692DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8071026PMC
April 2021

Heterogeneity of the Clinical Presentation of the MEN1 LRG_509 c.781C>T (p.Leu261Phe) Variant Within a Three-Generation Family.

Genes (Basel) 2021 Mar 31;12(4). Epub 2021 Mar 31.

Department of Endocrinology, Jagiellonian University Medical College, 31-008 Cracow, Poland.

Multiple neuroendocrine neoplasia type 1 (MEN1) is a rare genetic disorder with an autosomal dominant inheritance, predisposing carriers to benign and malignant tumors. The phenotype of MEN1 syndrome varies between patients in terms of tumor localization, age of onset, and clinical aggressiveness, even between affected members within the same family. We describe a heterogenic phenotype of the variant c.781C>T (LRG_509t1), which was previously reported only once in a family with isolated hyperparathyroidism. A heterozygous missense variant in exon 4 of the gene was identified in the sequence of the gene, i.e., c.781C>T, leading to the amino acid change p.Leu261Phe in a three-generation family. In the screened family, 5/6 affected members had already developed hyperparathyroidism. In the index patient and two other family members, an aggressive course of pancreatic neuroendocrine tumor (insulinoma and non-functioning neuroendocrine tumors) with dissemination was diagnosed. In the index patient, late diagnosis and slow progression of the disseminated neuroendocrine tumor have been observed (24 years of follow-up). The very rare variant of , LRG_509t1 c.781C>T /p.Leu261Phe (LRG_509p1), diagnosed within a three-generation family has a heterogenic clinical presentation. Further follow-up of the family members should be carried out to confirm the spectrum and exact time of clinical presentation.
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http://dx.doi.org/10.3390/genes12040512DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8067145PMC
March 2021

Efficacy of multimodal anticancer therapy in the course of pancreatic neuroendocrine carcinoma.

Endokrynol Pol 2021 23;72(2):183-184. Epub 2021 Feb 23.

Jagiellonian University Medical College, Krakow, Poland.

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http://dx.doi.org/10.5603/EP.a2021.0005DOI Listing
February 2021

Graves' disease and exophthalmos - a mask for meningioma.

Endokrynol Pol 2021 23;72(2):179-180. Epub 2021 Feb 23.

Chair and Department of Endocrinology, Jagiellonian University Medical College, Krakow, Poland.

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http://dx.doi.org/10.5603/EP.a2021.0006DOI Listing
February 2021

Bilateral slipped capital femoral epiphysis as first manifestation of primary hyperparathyroidism in a 15-year-old boy.

Pediatr Endocrinol Diabetes Metab 2020 ;26(4):220-224

Department of Pediatric and Adolescent Endocrinology, University Children's Hospital of Krakow, Poland.

Abstract: Primary hyperparathyroidism (PHP) in children is a rare condition and has a very dynamic course with nonspecific symptoms, what complicates the diagnosis and delays PHP treatment.

Case Presentation: A 15-year-old boy was admitted to the Orthopedic Ward with the diagnosis of juvenile bilateral slipped capital femoral epiphysis and valgus deformities. Gait disturbances, limb pains and valgus knee deformities, polyuria, polydipsia and weight loss, have been increasing for 8 months. Despite the hypercalcemia found in laboratory tests and bone destruction demonstrated in computed tomography of the hips, orthopedic correction was performed. In histopathological examination -brown bone tumors. The PTH concentration was determined (PTH - 589.1 pg/ml; (N: 10-60) and the child was referred to the Department of Pediatric Endocrinology, where severe hypercalcemia (Ca - 4.07 mmol/l, N: 2.2-2.84) and hypophosphatemia (P - 0.68 mmol/l; N: 0.95-1.75) and adenoma of the left lower parathyroid gland was diagnosed. Forced diuresis, loop diuretics and pamidronic acid were used to obtain normocalcemia. The complications of hypercalcemia were excluded; diagnostic management excluding multiple endocrine neoplasia type 1 and 2a (MEN 1 and MEN 2A) syndrome were performed. The child was referred to the Department of Endocrinological Surgery, where the adenoma of the left inferior parathyroid gland was resected.

Conclusions: 1) Patients with PHP should be diagnosed of the condition based on clinical symptoms. In patients with specific symptoms, it is necessary to determine serum Ca levels, especially prior to the surgical procedures. 2) In each case of PHP, determinations should be made of blood PTH, Ca and P and detection of MEN 1 and MEN 2A syndromes. 3) Patients with hyperparathyroidism require management of multiorgan complications of hypercalcemia. 4) Following surgical treatment of parathyroid adenoma, long-term endocrinological follow-up is necessary.
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http://dx.doi.org/10.5114/pedm.2020.100805DOI Listing
January 2020

Selection of the First Tc-Labelled Somatostatin Receptor Subtype 2 Antagonist for Clinical Translation-Preclinical Assessment of Two Optimized Candidates.

Pharmaceuticals (Basel) 2020 Dec 28;14(1). Epub 2020 Dec 28.

Department of Nuclear Medicine, Medical University Innsbruck, 6020 Innsbruck, Austria.

Recently, radiolabelled antagonists targeting somatostatin receptors subtype 2 (SST2) in neuroendocrine neoplasms demonstrated certain superior properties over agonists. Within the ERA-PerMED project "TECANT" two Tc-Tetramine (N4)-derivatized SST2 antagonists (TECANT-1 and TECANT-2) were studied for the selection of the best candidate for clinical translation. Receptor-affinity, internalization and dissociation studies were performed in human embryonic kidney-293 (HEK293) cells transfected with the human SST2 (HEK-SST2). Log , protein binding and stability in human serum were assessed. Biodistribution and SPECT/CT studies were carried out in nude mice bearing HEK-SST2 xenografts, together with dosimetric estimations from mouse-to-man. [Tc]Tc-TECANT-1 showed higher hydrophilicity and lower protein binding than [Tc]-TECANT-2, while stability was comparable. Both radiotracers revealed similar binding affinity, while [Tc]Tc-TECANT-1 had higher cellular uptake (>50%, at 2 h/37 °C) and lower dissociation rate (<30%, at 2 h/37 °C). In vivo, [Tc]Tc-TECANT-1 showed lower blood values, kidney and muscles uptake, whereas tumour uptake was comparable to [Tc]Tc-TECANT-2. SPECT/CT imaging confirmed the biodistribution results, providing the best tumour-to-background image contrast for [Tc]Tc-TECANT-1 at 4 h post-injection (p.i.). The estimated radiation dose amounted to approximately 6 µSv/MBq for both radiotracers. This preclinical study provided the basis of selection of [Tc]Tc-TECANT-1 for clinical translation of the first Tc-based SST2 antagonist.
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http://dx.doi.org/10.3390/ph14010019DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7824897PMC
December 2020

fT3:fT4 ratio in Graves' disease - correlation with TRAb level, goiter size and age of onset.

Folia Med Cracov 2020 09;60(2):15-27

Department of Endocrinology Jagiellonian University Medical College.

Introduction: Graves' Disease (GD) is an autoimmune hyperthyroidism occurring mostly in young women. The main pathogenic role of the disease is attributed to TSH receptor antibodies (TRAb), which stimulate the thyroid gland to increase production of the most active thyroid hormone- triiodothyronine (T3). High level of TRAb and a large goiter size are commonly known as poor prognostic factors for the disease and are used to predict relapse.

The Aim: The purpose of our study was to check the correlation between fT3:fT4 ratio with TRAb concentration, total volume of thyroid and age of GD onset.

Materials And Methods: 114 patients with onset or relapse of GD were analyzed. Those after thyroidectomy or radioiodine therapy were not taken into analysis. The data was retrospectively retrieved from the hospital's records consisting of patients' sex, age, level of TRAb, fT3, fT4 and thyroid volume on ultrasonography. The association between fT3:fT4 and TRAb concentration, thyroid volume and age was evaluated using Pearson correlation coefficient.

Results: The group was predominated by women (19.3% men, 80.7% women). The average age was 47.0. The analysis revealed positive correlation between: 1) fT3:fT4 ratio and total volume of thyroid (correlation ratio: 0.37; p <0.05) 2) fT3:fT4 ratio and level of TRAb (correlation ratio: 0.26; p or <0.05) 3) negative correlation between fT3:fT4 ratio and patient's age (correlation ratio: -0.14; p = 0.144).

Conclusions: Positive correlations between fT3:fT4 ratio and TRAb level and total volume of thyroid (poor predictors of GD) may confirm that high level of fT3 can also be a prognostic factor for GD severity.
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September 2020

Ectopic ACTH syndrome of different origin-Diagnostic approach and clinical outcome. Experience of one Clinical Centre.

PLoS One 2020 25;15(11):e0242679. Epub 2020 Nov 25.

Department of Endocrinology, Jagiellonian University, Krakow, Poland.

Purpose: Ectopic Cushing Syndrome (EAS) is a rare condition responsible for about 5-20% of all Cushing syndrome cases. It increases the mortality of affected patients thus finding and removal of the ACTH-producing source allows for curing or reduction of symptoms and serum cortisol levels. The aim of this study is to present a 20-year experience in the diagnosis and clinical course of patients with EAS in a single Clinical Centre in Southern Poland as well as a comparison of clinical course and outcomes depending on the source of ectopic ACTH production-especially neuroendocrine tumors with other neoplasms.

Methods: Twenty-four patients were involved in the clinical study with EAS diagnosed at the Department of Endocrinology between years 2000 and 2018. The diagnosis of EAS was based on the clinical presentation, hypercortisolemia with high ACTH levels, high dose dexamethasone suppression test and/or corticotropin-releasing hormone tests. To find the source of ACTH various imaging studies were performed.

Results: Half of the patients were diagnosed with neuroendocrine tumors, whereby muscle weakness was the leading symptom. Typical cushingoid appearance was seen in merely a few patients, and weight loss was more common than weight gain. Patients with neuroendocrine tumors had significantly higher midnight cortisol levels than the rest of the group. Among patients with infections, we observed a significantly higher concentrations of cortisol 2400 levels in gastroenteropancreatic neuroendocrine tumors. Chromogranin A correlated significantly with potassium in patients with neuroendocrine tumors and there was a significant correlation between ACTH level and severity of hypokalemia.

Conclusion: EAS is not common, but if it occurs it increases the mortality of patients; therefore, it should be taken into consideration in the case of coexistence of severe hypokalemia with hypertension and muscle weakness, especially when weight loss occurs. Because the diagnosis of gastroenteropancreatic neuroendocrine tumor worsens the prognosis-special attention should be paid to these patients.
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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0242679PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7688153PMC
January 2021

Expert opinion on liquid L-thyroxine usage in hypothyroid patients and new liquid thyroxine formulation - Tirosint SOL [Opinia ekspertów dotycząca stosowania płynnej postaci lewotyroksyny oraz nowego preparatu Tirosint SOL u chorych na niedoczynność tarczycy].

Endokrynol Pol 2020 ;71(5):441-465

Department of Endocrinology, Medical Centre of Postgraduate Education, Warsaw, Poland.

Hypothyroidism is a common endocrine disorder affecting 3-15% of the adult population in subclinical form and 0.3-0.8% as overt disease. The mainstay of treatment is replacement monotherapy with levothyroxine (LT4). Currently several oral LT4 formulations including tablets, softgel capsules, and liquid formulations are available. Liquid LT4 is manufactured as LT4 solution in 85% glycerol and 96% ethanol and as LT4 solution in purified water and glycerol. The latest formulation, Tirosint SOL, gained FDA approval in 2017. To evaluate the clinical utility of liquid LT4 we reviewed the literature using three databases: PubMed/MEDLINE, Scopus, and Embase and found 405 articles among which 23 prospective and two retrospective studies were further evaluated. Finally, several case reports on rare clinical conditions were discussed. Our review demonstrated that liquid LT4 was more effective than tablet formulation in patients with malabsorption caused by interfering diseases, drugs, and bariatric surgery. The better pharmacokinetics of liquid LT4 was also confirmed in subjects without malabsorption: patients on replacement or suppressive therapy, who switched from tablet to liquid formulation in equivalent dose, gained better hormonal control, and required less frequent TSH measurements. The drug also appeared effective and easy to handle in patients fed by enteric tube. Liquid LT4 appeared equally effective whenever taken before or during breakfast. The analysis of the drug utility in particular populations including newborns, pregnant women, and the elderly confirmed the high value and safety of liquid LT4. However, in neonates the higher incidence of TSH suppression on liquid in comparison to tablet LT4 therapy was noted, and particular attention to avoid over-treatment must be paid. Concluding: the literature review revealed that liquid LT4 is especially advantageous in patients with malabsorption and the critically ill, but it seems also very promising in common therapy. The lack of alcohol content in the new formulation makes Tirosint SOL especially attractive.
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http://dx.doi.org/10.5603/EP.a2020.0065DOI Listing
January 2020

Acromegaly and late-onset primary hyperparathyroidism in a female with a rare MEN1 gene variant of yet undetermined clinical significance (p.Val167Ala).

Endokrynol Pol 2020 30;71(6):579-580. Epub 2020 Oct 30.

Department of Endocrinology, Jagiellonian University Medical College, Cracow, Poland.

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http://dx.doi.org/10.5603/EP.a2020.0063DOI Listing
October 2020

Comparison of PET/CT imaging with [F]FDOPA and cholecystokinin-2 receptor targeting [Ga]Ga-DOTA-MGS5 in a patient with advanced medullary thyroid carcinoma.

Eur J Nucl Med Mol Imaging 2021 Mar 3;48(3):935-936. Epub 2020 Aug 3.

Department of Nuclear Medicine, Medical University Innsbruck, Anichstrasse 35, 6020, Innsbruck, Austria.

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http://dx.doi.org/10.1007/s00259-020-04963-zDOI Listing
March 2021

Current iodine nutrition status in Poland (2017): is the Polish model of obligatory iodine prophylaxis able to eliminate iodine deficiency in the population?

Public Health Nutr 2020 10 1;23(14):2467-2477. Epub 2020 Jun 1.

Department of Endocrinology, Jagiellonian University Medical College, Kraków, Poland.

Objective: The monitoring of the populations' iodine status is an essential part of successful programmes of iodine deficiency elimination. The current study aimed at the evaluation of current iodine nutrition in school children, pregnant and lactating women as a marker of the effectiveness and sustainability of mandatory iodine prophylaxis in Poland.

Design: The following iodine nutrition indicators were used: urinary iodine concentration (UIC) (all participants) and serum thyroglobulin (pregnant and lactating women).

Setting: The study was conducted in 2017 within the National Health Programme in five regions of Poland.

Participants: The research included 300 pregnant women, 100 lactating women and 1000 school children (aged 6-12 years).

Results: In pregnant women, median UIC was 111·6 µg/l; there was no significant difference in median UIC according to the region of residence. In 8 % of pregnant women, thyroglobulin level was >40 ng/ml (median thyroglobulin 13·3 ng/ml). In lactating women, median UIC was 68·0 µg/l. A significant inter-regional difference was noted (P = 0·0143). In 18 % of breastfeeding women, thyroglobulin level was >40 ng/ml (median thyroglobulin 18·5 ng/ml). According to the WHO criteria, the investigated sample of pregnant and lactating women was iodine-deficient. Median UIC in school children was 119·8 µg/l (with significant inter-regional variation; P = 0·0000), which is consistent with iodine sufficiency. Ninety-four children (9·4 %) had UIC < 50 µg/l.

Conclusions: Mandatory iodisation of household salt in Poland has led to a sustainable optimisation of iodine status in the general population. However, it has failed to assure adequate iodine nutrition during pregnancy and lactation.
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http://dx.doi.org/10.1017/S1368980020000403DOI Listing
October 2020

Standardized Map of Iodine Status in Europe.

Thyroid 2020 09 15;30(9):1346-1354. Epub 2020 Jul 15.

Department of Public Health, Dokuz Eylul University Medical Faculty, İzmir, Turkey.

Knowledge about the population's iodine status is important, because it allows adjustment of iodine supply and prevention of iodine deficiency. The validity and comparability of iodine-related population studies can be improved by standardization, which was one of the goals of the EUthyroid project. The aim of this study was to establish the first standardized map of iodine status in Europe by using standardized urinary iodine concentration (UIC) data. We established a gold-standard laboratory in Helsinki measuring UIC by inductively coupled plasma mass spectrometry. A total of 40 studies from 23 European countries provided 75 urine samples covering the whole range of concentrations. Conversion formulas for UIC derived from the gold-standard values were established by linear regression models and were used to postharmonize the studies by standardizing the UIC data of the individual studies. In comparison with the EUthyroid gold-standard, mean UIC measurements were higher in 11 laboratories and lower in 10 laboratories. The mean differences ranged from -36.6% to 49.5%. Of the 40 postharmonized studies providing data for the standardization, 16 were conducted in schoolchildren, 13 in adults, and 11 in pregnant women. Median standardized UIC was <100 μg/L in 1 out of 16 (6.3%) studies in schoolchildren, while in adults 7 out of 13 (53.8%) studies had a median standardized UIC <100 μg/L. Seven out of 11 (63.6%) studies in pregnant women revealed a median UIC <150 μg/L. We demonstrate that iodine deficiency is still present in Europe, using standardized data from a large number of studies. Adults and pregnant women, particularly, are at risk for iodine deficiency, which calls for action. For instance, a more uniform European legislation on iodine fortification is warranted to ensure that noniodized salt is replaced by iodized salt more often. In addition, further efforts should be put on harmonizing iodine-related studies and iodine measurements to improve the validity and comparability of results.
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http://dx.doi.org/10.1089/thy.2019.0353DOI Listing
September 2020

Traumatic brain injuries induced pituitary dysfunction: a call for algorithms.

Endocr Connect 2020 May;9(5):R112-R123

Chair and Department of Endocrinology, Jagiellonian University Medical College, Krakow, Poland.

Traumatic brain injury affects many people each year, resulting in a serious burden of devastating health consequences. Motor-vehicle and work-related accidents, falls, assaults, as well as sport activities are the most common causes of traumatic brain injuries. Consequently, they may lead to permanent or transient pituitary insufficiency that causes adverse changes in body composition, worrisome metabolic function, reduced bone density, and a significant decrease in one's quality of life. The prevalence of post-traumatic hypopituitarism is difficult to determine, and the exact mechanisms lying behind it remain unclear. Several probable hypotheses have been suggested. The diagnosis of pituitary dysfunction is very challenging both due to the common occurrence of brain injuries, the subtle character of clinical manifestations, the variable course of the disease, as well as the lack of proper diagnostic algorithms. Insufficiency of somatotropic axis is the most common abnormality, followed by presence of hypogonadism, hypothyroidism, hypocortisolism, and diabetes insipidus. The purpose of this review is to summarize the current state of knowledge about post-traumatic hypopituitarism. Moreover, based on available data and on our own clinical experience, we suggest an algorithm for the evaluation of post-traumatic hypopituitarism. In addition, well-designed studies are needed to further investigate the pathophysiology, epidemiology, and timing of pituitary dysfunction after a traumatic brain injury with the purpose of establishing appropriate standards of care.
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http://dx.doi.org/10.1530/EC-20-0117DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7274553PMC
May 2020

Difficulties in the diagnosis and treatment of malignant paraganglioma of the urinary bladder.

Endokrynol Pol 2020 15;71(2):196-197. Epub 2020 Apr 15.

Clinical Department of Endocrinology, University Hospital, Jagiellonian University, Medical College, Krakow, Poland.

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http://dx.doi.org/10.5603/EP.a2019.0068DOI Listing
January 2021

Decreased Immunoglobulin G Core Fucosylation, A Player in Antibody-dependent Cell-mediated Cytotoxicity, is Associated with Autoimmune Thyroid Diseases.

Mol Cell Proteomics 2020 05 5;19(5):774-792. Epub 2020 Feb 5.

Faculty of Pharmacy and Biochemistry, University of Zagreb, Zagreb, Croatia; Genos, Glycoscience Research Laboratory, Zagreb, Croatia. Electronic address:

Autoimmune thyroid diseases (AITD) are the most common group of autoimmune diseases, associated with lymphocyte infiltration and the production of thyroid autoantibodies, like thyroid peroxidase antibodies (TPOAb), in the thyroid gland. Immunoglobulins and cell-surface receptors are glycoproteins with distinctive glycosylation patterns that play a structural role in maintaining and modulating their functions. We investigated associations of total circulating IgG and peripheral blood mononuclear cells glycosylation with AITD and the influence of genetic background in a case-control study with several independent cohorts and over 3,000 individuals in total. The study revealed an inverse association of IgG core fucosylation with TPOAb and AITD, as well as decreased peripheral blood mononuclear cells antennary α1,2 fucosylation in AITD, but no shared genetic variance between AITD and glycosylation. These data suggest that the decreased level of IgG core fucosylation is a risk factor for AITD that promotes antibody-dependent cell-mediated cytotoxicity previously associated with TPOAb levels.
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http://dx.doi.org/10.1074/mcp.RA119.001860DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7196582PMC
May 2020

Decision-Analytic Modeling Studies in Prevention and Treatment of Iodine Deficiency and Thyroid Disorders: A Systematic Overview.

Thyroid 2020 05 26;30(5):746-758. Epub 2020 Feb 26.

Institute of Public Health, Medical Decision Making and Health Technology Assessment, Department of Public Health, Health Services Research and Health Technology Assessment, UMIT-University for Health Sciences, Medical Informatics and Technology, Hall in Tirol, Austria.

Prevention and treatment of iodine deficiency-related diseases remain an important public health challenge. Iodine deficiency can have severe health consequences, such as cretinism, goiter, or other thyroid disorders, and it has economic implications. Our aim was to give an overview of studies applying decision-analytic modeling to evaluate the effectiveness and/or cost-effectiveness of iodine deficiency-related prevention strategies or treatments related to thyroid disorders. We performed a systematic literature search in PubMed/MEDLINE (Medical Literature Analysis and Retrieval System Online), EMBASE (Excerpta Medica Database), Tuft's Cost-Effectiveness Analysis Registry, and National Health System Economic Evaluation Database (NHS EED) to identify studies published between 1985 and 2018 comparing different prevention or treatment strategies for iodine deficiency and thyroid disorders by applying a mathematical decision-analytic model. Studies were required to evaluate patient-relevant health outcomes (e.g., remaining life years, quality-adjusted life years [QALYs]). Overall, we found 3950 studies. After removal of duplicates, abstract/title, and full-text screening, 17 studies were included. Eleven studies evaluated screening programs (mainly newborns and pregnant women), five studies focused on treatment approaches (Graves' disease, toxic thyroid adenoma), and one study was about primary prevention (consequences of iodine supplementation on offspring). Most of the studies were conducted within the U.S. health care context ( = 7). Seven studies were based on a Markov state-transition model, nine studies on a decision tree model, and in one study, an initial decision tree and a long-term Markov state-transition model were combined. The analytic time horizon ranged from 1 year to lifetime. QALYs were evaluated as health outcome measure in 15 of the included studies. In all studies, a cost-effectiveness analysis was performed. None of the models reported a formal model validation. In most cases, the authors of the modeling studies concluded that screening is potentially cost-effective or even cost-saving. The recommendations for treatment approaches were rather heterogeneous and depending on the specific research question, population, and setting. Overall, we predominantly identified decision-analytic modeling studies evaluating specific screening programs or treatment approaches; however, there was no model evaluating primary prevention programs on a population basis. Conclusions deriving from these studies, for example, that prevention is cost-saving, need to be carefully interpreted as they rely on many assumptions.
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http://dx.doi.org/10.1089/thy.2018.0776DOI Listing
May 2020

Pregnancy, thyroid, and the potential use of selenium.

Hormones (Athens) 2020 Mar 13;19(1):47-53. Epub 2019 Nov 13.

Chair and Department of Endocrinology, Jagiellonian University Medical College, Kopernika 17 Str, 31-501, Cracow, Poland.

The management of pregnant women is a major concern of health care around the world. There is growing evidence regarding the influence of selenium (Se) on pregnancy and fetus outcomes. However, due to as yet insufficient evidence, lack of measurable markers to assess the effect of Se supplementation on the human metabolism, and Se's narrow therapeutic index, the majority of experts and the current guidelines published by several scientific societies do not recommend the use of Se in pregnancy and in women of childbearing age. Further research based on well-designed studies, including assessment of the complex interactions between different micronutrients and individual response to different doses of Se, is needed.
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http://dx.doi.org/10.1007/s42000-019-00144-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7033058PMC
March 2020

Radiological and hormonal improvements in a 22-year-old patient with lymphocytic hypophysitis - the watchful waiting approach.

Endokrynol Pol 2020 4;71(1):104-105. Epub 2019 Nov 4.

Chair and Department of Endocrinology, Jagiellonian University Medical College, Krakow, Poland.

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http://dx.doi.org/10.5603/EP.a2019.0046DOI Listing
November 2020

Pituitary tumours in MEN1 syndrome - the new insight into the diagnosis and treatment.

Endokrynol Pol 2019 ;70(5):445-452

Endocrinology Department, University Hospital in Krakow, Krakow, Poland.

Pituitary tumours are a common pathology affecting 15-20% of the population. Only about 1‰ of adenomas are clinically manifested; among them, about two/thirds are hormonally active, most often secreting prolactin or growth hormone. Pituitary tumours are mainly an isolated pathology, without any genetic background. However, the latest studies pay special attention to the possibility of developing an adenoma as a result of genetic mutation. Among pituitary adenomas, the leading group of genetically determined lesions is related to a mutation in AIP or MEN1, followed by PRKAR1A, GRP101, DICER, and SDHx. The genetic basis of these pituitary tumours is related to positive family history, young age of the patient, aggressive clinical process, and resistance to treatment. Pituitary tumours occur in over 40% of patients with MEN1 syndrome - often in women, they are more than 1 cm in diameter, and secrete prolactin. They are usually diagnosed in the fourth decade of life and show a worse response to pharmacotherapy than sporadic ones. Confirmation of the genetic background of the pituitary tumour implies measurable implications; it might help to direct the diagnosis in patients' family members, partially predict the development of the disease, and, above all, extend patients' life expectancy.
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http://dx.doi.org/10.5603/EP.a2019.0026DOI Listing
July 2020

The effectiveness of a community-based, type 2 diabetes prevention programme on health-related quality of life. The DE-PLAN study.

PLoS One 2019 11;14(10):e0221467. Epub 2019 Oct 11.

First Department of Propaedeutic Medicine, Diabetes Center, National and Kapodistrian University of Athens, Laiko General Hospital, Athens, Greece.

Background And Aims: The DE-PLAN was a European multicenter study, with the primary objective of testing whether a community-based lifestyle modification programme could serve as a means of primary prevention for type 2 diabetes (T2D) in high-risk individuals (based on the FINDRISC questionnaire). The aim of this study was to examine the impact of a 1-year community-based lifestyle intervention on health-related quality of life (HRQOL) in individuals from four participating European centers (Athens, Barcelona, Krakow, Kaunas), through a post-hoc analysis.

Materials And Methods: Each center was allowed to implement different intervention strategies specifically tailored to the needs of their corresponding population sample. Before and after the intervention, participants underwent clinical evaluation, anthropometric measurements, an oral glucose tolerance test and lipid profile measurements. Health-related quality of life was assessed using the validated HRQOL-15D questionnaire. A difference of ±0.015 in the 15D questionnaire score was set as the threshold of clinically meaningful change.

Results: Data from 786 participants (67% females, mean age 59.7±9.4 years, BMI 31.5±4.5 kg/m2) with complete data regarding the HRQOL were analyzed (Athens: 104, Barcelona: 434, Krakow: 175, Kaunas: 70). After 1 year, a significant overall improvement in HRQOL was shown, as depicted by a change of 15D score from baseline value (0.88±0.9) to post-intervention (0.90±0.87, P<0.001), achieving the threshold of clinically meaningful change. A significant weight reduction was also observed (-0.8±4.0 kg, P<0.001). In multivariate analysis, improvement in HRQOL was independently associated with lower 15D score at baseline (P<0.001) and self-reported increase in overall exercise time (P<0.001) as assessed through specifically designed trial questionnaires.

Conclusion: A community-based lifestyle intervention programme aiming at T2D prevention, applied on a heterogeneous population and with varied methods, was shown to improve overall health-related quality of life to a clinically meaningful degree.
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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0221467PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6788719PMC
March 2020

Optimization of parathyroid C-choline PET protocol for localization of parathyroid adenomas in patients with primary hyperparathyroidism.

EJNMMI Res 2019 Jul 31;9(1):73. Epub 2019 Jul 31.

University of Groningen, University Medical Center Groningen, Department of Nuclear Medicine and Molecular Imaging, P.O. Box 30 001, 9700, RB, Groningen, The Netherlands.

Purpose: To evaluate the optimal tracer uptake time, the minimal amount of radioactivity and the inter-observer agreement for C-choline positron emission tomography/computed tomography (PET/CT) in patients with primary hyperparathyroidism (pHPT).

Methods: Twenty-one patients with biochemically proven pHPT were retrospectively studied after injection of 6.3 ± 1.2 MBq/kg C-choline. PET data of the first nine patients, scanned for up to 60 min, were reconstructed in 10-min frames from 10- to 60-min postinjection (p.i.), mimicking varying C-choline uptake times. Parathyroid adenoma to background contrast ratios were calculated and compared, using standardized uptake values (SUVs). Data was reconstructed with varying scan durations (1, 2.5, 5, and 10 min) at 20-30-min p.i. (established optimal uptake time), mimicking less administered radioactivity. To establish the minimal required radioactivity, the SUVs in the shorter scan durations (1, 2.5, and 5 min) were compared to the 10-min scan duration to determine whether increased variability and/or statistical differences were observed. Four observers analyzed the C-choline PET/CT in four randomized rounds for all patients.

Results: SUVpeak of the adenoma decreased from 30 to 40 p.i. onwards. All adenoma/background contrast ratios did not differ from 20- to 30-min p.i. onwards. The SUVs of adenoma in the scan duration of 1, 2.5, and 5 min all differed significantly from the same SUV in the 10-min scan duration (all p = 0.012). However, the difference in absolute SUV adenoma values was well below 10% and therefore not considered clinically significant. The inter-observer analysis showed that the Fleiss' kappa of the 1-min scan were classified as "moderate," while these values were classified as "good" in the 2.5-, 5-, and 10-min scan duration. Observers scored lower certainty scores in the 1- and 2.5-min scans compared to the 5- and 10-min scan durations.

Conclusion: The optimal time to start PET/CT scanning in patients with pHPT is 20 min after mean injection of 6.3 MBq/kg C-choline, with a recommended scan duration of at least 5 min. Alternatively, the radioactivity dose can be lowered by 50% while keeping a 10-min scan duration without losing the accuracy of C-choline PET/CT interpretation.
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http://dx.doi.org/10.1186/s13550-019-0534-5DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6669228PMC
July 2019

Hypophysitis - new insights into diagnosis and treatment.

Endokrynol Pol 2019 ;70(3):260-269

Department of Endocrinology, University Hospital in Krakow, Krakow, Poland.

Hypophysitis is a broad term used to describe conditions leading to inflammation of the pituitary gland and the pituitary stalk. It may develop as a primary condition or secondary to other diseases. Hypophysitis is classified based on aetiological, anatomical, and histological criteria. Clinical symptoms result from enlargement of the pituitary gland, hormonal deficiencies, diabetes insipidus, and hyperprolactinemia. Histopathological verification of tissue samples from a pituitary biopsy remains the gold standard in diagnosing hypophysitis. However, due to the invasiveness and risk of the procedure it is rarely performed. The diagnosis is based mainly on clinical presentation, laboratory tests, and imaging. The rarity of the disease and the deficit in reliable data result in a lack of clear guidelines in the treatment of hypophysitis. The basic therapy relies on hormonal replacement. High doses of steroids are the first-line treatment of symptoms caused by mass effect in sella and compression of surrounding structures. In steroid-resistant patients or in cases of inacceptable sides effects, treatment with other immunosuppressant drugs was administered with success. The course of the disease varies: some patients present remission, in other cases hypophysitis leads to fibrosis and atrophy of the pituitary gland, which is reflected in persistent hormonal deficiencies and images of an empty sella. The objective of this article is to present the most important information: the epidemiology, clinical image, diagnostic procedures, and treatment of primary hypophysitis, in order to allow better understanding of this disease and implementation of proper management. Posttraumatic and immunotherapyrelated hypophysitis are also briefly characterised.
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http://dx.doi.org/10.5603/EP.a2019.0015DOI Listing
December 2019

Superior Diagnostic Performance of the GLP-1 Receptor Agonist [Lys(AhxHYNIC-[Tc]/EDDA)NH]-Exendin-4 over Conventional Imaging Modalities for Localization of Insulinoma.

Mol Imaging Biol 2020 02;22(1):165-172

Department of Nuclear Medicine, University Medical Centre Ljubljana, Zaloska 7, 1525, Ljubljana, Slovenia.

Purpose: Insulinomas are the most common functioning neuroendocrine neoplasms of the pancreas, typically diagnosed due to characteristic symptoms. In the vast majority, the treatment is surgical and curative, requiring accurate localization of the tumour; conventional imaging, including somatostatin receptor molecular imaging, is negative in up to 10 % of cases. Recently, labelled glucagon-like peptide receptor (GLP-1R) analogues were introduced as a sensitive diagnostic method for localization of insulinomas. The aim of this study was to assess the diagnostic accuracy of a Tc-99m-labelled GLP-1R agonist [Lys(AhxHYNIC-[Tc]EDDA)NH]-exendin-4 for localization of occult insulinoma.

Procedures: Eight patients (all females; age range 35-75 years) with biochemically proven insulinoma and with negative or inconclusive conventional imaging (consisting of somatostatin receptor scintigraphy, computed tomography, endoscopic ultrasound and magnetic resonance imaging) were enrolled. Whole-body single-photon emission tomography/computed tomography (SPECT/CT) imaging was performed 4 h post-injection of 740 MBq of [Lys(AhxHYNIC-[Tc]EDDA)NH]-exendin-4. Surgical treatment was performed based on imaging findings. Histology of the removed lesions and biochemical and clinical symptom resolution was considered as the gold standard for analysis of the imaging results.

Results: Focal uptake of [Lys(AhxHYNIC-[Tc]EDDA)NH]-exendin-4 was found in all patients, leading to successful removal of the offending lesion and complete biochemical and symptomatic resolution. Histological analysis confirmed insulinoma in all included patients.

Conclusions: [Lys(AhxHYNIC-[Tc]EDDA)NH]-exendin-4 SPECT/CT appears to be an excellent molecular imaging method for preoperative localization of an occult insulinoma, surpassing conventional imaging methods. If routinely available, it could be considered as a method of choice due to its favorable combination of imaging characteristics.
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http://dx.doi.org/10.1007/s11307-019-01372-zDOI Listing
February 2020

Consolidation with Y ibritumomab tiuxetan radioimmunotherapy in mantle cell lymphoma patients ineligible for high dose therapy: results of the phase II multicentre Polish Lymphoma Research Group trial, after 8-year long follow-up.

Leuk Lymphoma 2019 11 9;60(11):2689-2696. Epub 2019 Apr 9.

Policlinico "Sant'Orsola-Malpighi" - University of Bologna Institute of Hematology "Seràgnoli", Bologna, Italy.

Polish Lymphoma Research Group performed a phase-II trial to test whether Y ibritumomab tiuxetan radioimmunotherapy (Y90) may constitute an alternative consolidation for mantle cell lymphoma patients unfit for high-dose therapy. Forty-six patients were consolidated with Y90 following response to the 1st ( = 34) or 2nd line ( = 12) (immuno)chemotherapy. Majority of the patients had advanced disease (stage IV and presence of B-symptoms in 85% and 70%, respectively) and high MIPI (5.8, range 4-7). Consolidation with Y90 increased the complete remission (CR) rate obtained by the 1st line therapy from 41% to 91% and allowed for median PFS of 3.3 and OS of 6.5 years. In the first relapse, CR rate increased from 16% to 75%, while median PFS and OS totaled 2.2 and 6.5 years, respectively. At 8 years, 30% of patients, consolidated in the 1st line CR were alive, without relapse. Toxicity associated with Y90 is manageable, more severe after fludarabine-based regimens.
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http://dx.doi.org/10.1080/10428194.2019.1602261DOI Listing
November 2019

The value of the free androgen index depends on the phenotype of polycystic ovary syndrome - a single-centre experience.

Endokrynol Pol 2019 2;70(4):330-335. Epub 2019 Apr 2.

Department of Gynaecological Endocrinology and Gynaecology, Jagiellonian University Medical College, Cracow, Poland.

Introduction: The free androgen index (FAI) values differ among patients with polycystic ovarian syndrome; however, the differences are not fully understood or known. The aim of the study was to evaluate FAI in women with polycystic ovary syndrome (PCOS) in regard to the phenotype of the PCOS and insulin resistance status.

Material And Methods: Anthropometric, hormonal, and biochemical parameters were assessed in 312 recruited women with PCOS. The FAI values were calculated in the reproductive and metabolic phenotypes of PCOS in groups of insulin resistance status based on the homeostasis model assessment-insulin resistance (HOMA-IR) > 2.0 or fasting insulin (FI) > 10 mmol/L. To test the relationship between individual variables, Spearman's correlation analysis, the Kolmogorov-Smirnov test, and Student's t-test were used.

Results: The correlation between FAI values and HOMA-IR and FI was 0.42 and 0.47, respectively, in PCOS patients. A two fold higher FAI value was observed in metabolic PCOS phenotype when compared to the reproductive one (8.51 ± 5.56 vs. 4.40 ± 2.45 for HOMA-IR and 8.73 ± 6.09 vs. 4.31 ± 3.39 for FI, respectively; p < 0.05).

Conclusions: PCOS patients are not a homogenous group in terms of FAI value. Patients with metabolic PCOS phenotype are characterised by two-fold higher FAI values compared with reproductive PCOS phenotype. Further studies on the metabolic and androgenic status of different types of PCOS phenotypes should be carried out.
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http://dx.doi.org/10.5603/EP.a2019.0017DOI Listing
February 2020

Comment on Sundin et al.: ENETS Consensus Guidelines.

Neuroendocrinology 2019 27;108(4):365. Epub 2019 Mar 27.

Chair and Department of Endocrinology, Jagiellonian University, Medical College, Cracow, Poland.

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http://dx.doi.org/10.1159/000497418DOI Listing
March 2019

Diagnostics and treatment of acromegaly - updated recommendations of the Polish Society of Endocrinology.

Endokrynol Pol 2019 ;70(1):2-18

Department of Endocrinology and Metabolic Disorders, Medical University, Lodz, Poland.

Acromegaly is a rare disease caused by excessive production of growth hormone (GH), typically by a pituitary tumour. The diagnosis is usually delayed, and patients frequently develop various complications that cause premature mortality. In patients with hypertension, heart failure, diabetes, and arthropathies that are not age-specific, attention should be paid to signs of acromegaly. Insulin-like growth factor 1 (IGF-1) assay should be used as a screening test whenever acromegaly is suspected. Further diagnostic investigations and treatment should be carried out at specialist centres. First-line treatment involves selective excision of pituitary adenoma using transsphenoidal access. Patients with chances of cure with surgical removal of the pituitary tumour should be referred to centres that have experience in this type of procedure, following pharmacological preparation. Other patients, as well as patients after failed neurosurgical treatment, should first receive chronic treatment with first-generation somatostatin analogues. For second-line treatment, pasireotide, pegvisomant, cabergoline, or combinations thereof should be considered. In every case, acromegaly sequelae require life-long monitoring and active treatment. Current recommendations, being an updated version of the recommendations published in Endokrynologia Polska in 2014, which take into account the Polish situation, should prove useful in the management of patients with acromegaly.
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http://dx.doi.org/10.5603/EP.a2018.0093DOI Listing
July 2019