LIVIVO - The Search Portal for Life Sciences

zur deutschen Oberfläche wechseln
Advanced search

Search results

Result 1 - 10 of total 42

Search options

  1. Article ; Online: The ISAQ Score Does Not Predict Adrenal Crisis in Patients with Primary Adrenal Insufficiency.

    Quinkler, Marcus / Kienitz, Tina

    Experimental and clinical endocrinology & diabetes : official journal, German Society of Endocrinology [and] German Diabetes Association

    2022  Volume 130, Issue 8, Page(s) 554–560

    Abstract: Objective: This study aimed to investigate the ability of the immune system assessment questionnaire (ISAQ) to predict adrenal crisis (AC) and infectious events in patients with primary adrenal insufficiency (PAI).: Design: This was a prospective ... ...

    Abstract Objective: This study aimed to investigate the ability of the immune system assessment questionnaire (ISAQ) to predict adrenal crisis (AC) and infectious events in patients with primary adrenal insufficiency (PAI).
    Design: This was a prospective single-centre study over three years.
    Methods: Patients answered the ISAQ at baseline and were seen every 4-6 months in the endocrine outpatient clinic. At each visit previous infectious periods which required an increase in daily glucocorticoid dosage and AC were reported and documented. Seventy-five patients with PAI (53 women; 43 patients with autoimmune PAI, 20 patients with salt-wasting congenital adrenal hyperplasia and 12 patients who underwent bilateral adrenalectomy) were analysed. Due to the COVID-19 pandemic and consecutive lockdown measures, the data were analysed separately for March 2018 to March 2020 (period 1), and March 2020 to March 2021 (period 2).
    Results: During period 1 the ISAQ score significantly correlated with the number of reported infectious events (r=0.351; p<0.01), but not during period 2 (r=0.059, p=0.613), in which the number of infectious events per patient-year significantly decreased (1.1±0.1 vs 0.4±0.1; p<0.001). The frequency of AC decreased from 8.8 to 2.4 per 100 patient-years between the two study periods. The ISAQ score was not different between patients with or without AC.
    Conclusions: The ISAQ score does not identify patients prone to ACs. The COVID-19 pandemic and consecutive lockdown measures significantly diminished the frequency of infectious events and ACs.
    MeSH term(s) Addison Disease/epidemiology ; Adrenal Insufficiency/diagnosis ; Adrenal Insufficiency/epidemiology ; COVID-19 ; Communicable Disease Control ; Female ; Humans ; Pandemics ; Prospective Studies ; Surveys and Questionnaires
    Language English
    Publishing date 2022-03-03
    Publishing country Germany
    Document type Journal Article
    ZDB-ID 1225416-2
    ISSN 1439-3646 ; 0947-7349
    ISSN (online) 1439-3646
    ISSN 0947-7349
    DOI 10.1055/a-1734-2466
    Database MEDical Literature Analysis and Retrieval System OnLINE

    More links

    Kategorien

  2. Article ; Online: Mineralocorticoid replacement therapy in salt-wasting congenital adrenal hyperplasia.

    Lang, Katharina / Quinkler, Marcus / Kienitz, Tina

    Clinical endocrinology

    2023  

    Abstract: Patients with salt-wasting congenital adrenal hyperplasia (SW-CAH) usually show pronounced impairment of aldosterone secretion and, therefore, also require mineralocorticoid replacement. While a lot of research and discussion focusses on the ... ...

    Abstract Patients with salt-wasting congenital adrenal hyperplasia (SW-CAH) usually show pronounced impairment of aldosterone secretion and, therefore, also require mineralocorticoid replacement. While a lot of research and discussion focusses on the glucocorticoid therapy in SW-CAH to replace the missing cortisol and to control adrenal androgen excess, very little research is dealing with mineralocorticoid replacement. However, recent data demonstrated an increased cardiovascular risk in adult CAH patients urging to reflect also on the current mineralocorticoid replacement therapy. In this review, we explain the role and function of the mineralocorticoid receptor, its ligands and inhibitors and its relevance for the therapy of patients with SW-CAH. We performed an extensive literature search and present data on mineralocorticoid therapy in SW-CAH patients as well as clinical advice how to monitor and optimise mineralocorticoid replacement therapy.
    Language English
    Publishing date 2023-08-11
    Publishing country England
    Document type Journal Article ; Review
    ZDB-ID 121745-8
    ISSN 1365-2265 ; 0300-0664
    ISSN (online) 1365-2265
    ISSN 0300-0664
    DOI 10.1111/cen.14959
    Database MEDical Literature Analysis and Retrieval System OnLINE

    More links

    Kategorien

  3. Article ; Online: Neue Aspekte der Glukokortikoidsubstitution bei Nebennierenrindeninsuffizienz.

    Kienitz, Tina / Meyer, Gesine

    Der Internist

    2021  Volume 63, Issue 1, Page(s) 12–17

    Abstract: Background: Appropriate glucocorticoid dose adjustment in specific situations significantly impacts quality of life and performance of patients with adrenal insufficiency. It is also pivotal for the prevention of adrenal crisis.: Objectives: ... ...

    Title translation New aspects of glucocorticoid substitution in adrenal insufficiency.
    Abstract Background: Appropriate glucocorticoid dose adjustment in specific situations significantly impacts quality of life and performance of patients with adrenal insufficiency. It is also pivotal for the prevention of adrenal crisis.
    Objectives: Improving medical care for patients with adrenal insufficiency.
    Materials and methods: Selective literature research focussing on the most recent studies.
    Results: Optimal glucocorticoid substitution aims at closely mimicking physiological fluctuations of cortisol levels. In recent years glucocorticoid preparations with modified pharmacokinetics have expanded the therapeutic arsenal. Adrenal crises occur with an incidence of 4.8-9.3 crises per 100 patient years. With a mortality of 0.5 per 100 patient years adrenal crisis is a life-threatening event. Therefore, it is of the utmost importance to adjust glucocorticoid dose in situations with increased cortisol demand in order to prevent as well as appropriately treat adrenal crisis.
    Conclusions: To prevent life-threatening adrenal crisis, patients, their families and medical staff require training.
    MeSH term(s) Acute Disease ; Adrenal Insufficiency/diagnosis ; Adrenal Insufficiency/drug therapy ; Glucocorticoids/adverse effects ; Humans ; Hydrocortisone ; Quality of Life
    Chemical Substances Glucocorticoids ; Hydrocortisone (WI4X0X7BPJ)
    Language German
    Publishing date 2021-12-03
    Publishing country Germany
    Document type Journal Article ; Review
    ZDB-ID 2913-0
    ISSN 1432-1289 ; 0020-9554
    ISSN (online) 1432-1289
    ISSN 0020-9554
    DOI 10.1007/s00108-021-01209-4
    Database MEDical Literature Analysis and Retrieval System OnLINE

    More links

    Kategorien

  4. Article: [No title information]

    Quinkler, Marcus / Kienitz, Tina

    Experimental and Clinical Endocrinology & Diabetes

    2022  Volume 130, Issue 08, Page(s) 554–560

    Abstract: Objective: This study aimed to investigate the ability of the immune system assessment questionnaire (ISAQ) to predict adrenal crisis (AC) and infectious events in patients with primary adrenal insufficiency (PAI). ...

    Abstract Objective: This study aimed to investigate the ability of the immune system assessment questionnaire (ISAQ) to predict adrenal crisis (AC) and infectious events in patients with primary adrenal insufficiency (PAI).
    Design: This was a prospective single-centre study over three years.
    Methods: Patients answered the ISAQ at baseline and were seen every 4–6 months in the endocrine outpatient clinic. At each visit previous infectious periods which required an increase in daily glucocorticoid dosage and AC were reported and documented. Seventy-five patients with PAI (53 women; 43 patients with autoimmune PAI, 20 patients with salt-wasting congenital adrenal hyperplasia and 12 patients who underwent bilateral adrenalectomy) were analysed. Due to the COVID-19 pandemic and consecutive lockdown measures, the data were analysed separately for March 2018 to March 2020 (period 1), and March 2020 to March 2021 (period 2).
    Results: During period 1 the ISAQ score significantly correlated with the number of reported infectious events (r=0.351; p<0.01), but not during period 2 (r=0.059, p=0.613), in which the number of infectious events per patient-year significantly decreased (1.1±0.1 vs 0.4±0.1; p<0.001). The frequency of AC decreased from 8.8 to 2.4 per 100 patient-years between the two study periods. The ISAQ score was not different between patients with or without AC.
    Conclusions: The ISAQ score does not identify patients prone to ACs. The COVID-19 pandemic and consecutive lockdown measures significantly diminished the frequency of infectious events and ACs.
    Keywords Addison’s disease ; ISAQ score ; AddiQoL ; adrenal crisis ; COVID-19 ; congenital adrenal hyperplasia
    Language English
    Publishing date 2022-03-03
    Publisher Georg Thieme Verlag KG
    Publishing place Stuttgart ; New York
    Document type Article
    ZDB-ID 1225416-2
    ISSN 1439-3646 ; 0947-7349
    ISSN (online) 1439-3646
    ISSN 0947-7349
    DOI 10.1055/a-1734-2466
    Database Thieme publisher's database

    More links

    Kategorien

  5. Article ; Online: Correction: Adrenal Crisis - Definition, Prevention and Treatment: Results from a Delphi Survey.

    Kienitz, Tina / Bechmann, Nicole / Deutschbein, Timo / Hahner, Stefanie / Honegger, Jürgen / Kroiss, Matthias / Quinkler, Marcus / Rayes, Nada / Reisch, Nicole / Willenberg, Holger Sven / Meyer, Gesine

    Hormone and metabolic research = Hormon- und Stoffwechselforschung = Hormones et metabolisme

    2023  

    Language English
    Publishing date 2023-08-25
    Publishing country Germany
    Document type Journal Article ; Published Erratum
    ZDB-ID 80125-2
    ISSN 1439-4286 ; 0018-5043
    ISSN (online) 1439-4286
    ISSN 0018-5043
    DOI 10.1055/a-2157-2308
    Database MEDical Literature Analysis and Retrieval System OnLINE

    More links

    Kategorien

  6. Article: Glucocorticoid Replacement for Adrenal Insufficiency and the Development of Non-Alcoholic Fatty Liver Disease.

    Meyer, Gesine / Gruendl, Madeleine / Chifu, Irina / Hahner, Stefanie / Werner, Johanna / Weiß, Johannes / Kienitz, Tina / Quinkler, Marcus / Badenhoop, Klaus / Herrmann, Eva / Friedrich-Rust, Mireen / Bojunga, Joerg

    Journal of clinical medicine

    2023  Volume 12, Issue 19

    Abstract: Glucocorticoid excess is a known risk factor for non-alcoholic fatty liver disease (NAFLD). Our objective was to analyse the impact of glucocorticoid replacement therapy on the development of NAFLD and NAFLD-related fibrosis and, therefore, on ... ...

    Abstract Glucocorticoid excess is a known risk factor for non-alcoholic fatty liver disease (NAFLD). Our objective was to analyse the impact of glucocorticoid replacement therapy on the development of NAFLD and NAFLD-related fibrosis and, therefore, on cardiovascular as well as hepatic morbidity in patients with adrenal insufficiency. Two hundred and fifteen individuals with primary (
    Language English
    Publishing date 2023-10-06
    Publishing country Switzerland
    Document type Journal Article
    ZDB-ID 2662592-1
    ISSN 2077-0383
    ISSN 2077-0383
    DOI 10.3390/jcm12196392
    Database MEDical Literature Analysis and Retrieval System OnLINE

    More links

    Kategorien

  7. Article ; Online: Therapeutic Patient Education for Adrenal Insufficiency under COVID-19 Pandemic Conditions.

    Kienitz, Tina / Hahner, Stefanie / Burger-Stritt, Stephanie / Quinkler, Marcus

    Experimental and clinical endocrinology & diabetes : official journal, German Society of Endocrinology [and] German Diabetes Association

    2020  Volume 129, Issue 3, Page(s) 241–249

    Abstract: Adrenal insufficiency (AI) requires life-long treatment with glucocorticoid replacement therapy. Over- or under-substitution carries the risk of increased morbidity in the form of side effects or adrenal crises. Glucocorticoid replacement therapy needs ... ...

    Abstract Adrenal insufficiency (AI) requires life-long treatment with glucocorticoid replacement therapy. Over- or under-substitution carries the risk of increased morbidity in the form of side effects or adrenal crises. Glucocorticoid replacement therapy needs to be flexible with dose adaptation in special situations. This could not be managed by medical personnel on a daily basis, but requires an educated patient who has a good knowledge of the disease, understands his medical therapy and is able to perform situational dose adaptation. The rarity of the disease in combination with the need to respond to stressful situations with rapid glucocorticoid dose adjustment underlines that a well-trained patient is crucial for optimal management of the disease.In this literature review we provide background information further clarifying the need of education in patients with AI including the current shortcomings of medical therapy and of the treatment of patients with AI. We outline the aims of therapeutic patient education, present the concept of structured patient education in Germany, and discuss available results of patient group education programs. Furthermore, we propose strategies how therapeutic patient education for adrenal insufficiency can be organized under COVID-19 pandemic conditions.
    MeSH term(s) Adrenal Insufficiency/drug therapy ; COVID-19 ; Glucocorticoids/administration & dosage ; Health Knowledge, Attitudes, Practice ; Hormone Replacement Therapy/standards ; Humans ; Patient Education as Topic ; Self-Management
    Chemical Substances Glucocorticoids
    Keywords covid19
    Language English
    Publishing date 2020-08-06
    Publishing country Germany
    Document type Journal Article ; Review
    ZDB-ID 1225416-2
    ISSN 1439-3646 ; 0947-7349
    ISSN (online) 1439-3646
    ISSN 0947-7349
    DOI 10.1055/a-1217-7208
    Database MEDical Literature Analysis and Retrieval System OnLINE

    More links

    Kategorien

  8. Article ; Online: Central Diabetes Insipidus Caused by Arginine Vasopressin Gene Mutation: Report of a Novel Mutation and Review of Literature.

    Feldkamp, Lara L I / Kaminsky, Elke / Kienitz, Tina / Quinkler, Marcus

    Hormone and metabolic research = Hormon- und Stoffwechselforschung = Hormones et metabolisme

    2020  Volume 52, Issue 11, Page(s) 796–802

    Abstract: Familial neurohypophyseal diabetes insipidus (FNDI) is an autosomal dominant hereditary disorder characterized by severe polydipsia and polyuria that usually presents in early childhood. In this study, we describe a new arginine vasopressin (AVP) gene ... ...

    Abstract Familial neurohypophyseal diabetes insipidus (FNDI) is an autosomal dominant hereditary disorder characterized by severe polydipsia and polyuria that usually presents in early childhood. In this study, we describe a new arginine vasopressin (AVP) gene mutation in an ethnic German family with FNDI and provide an overview of disease-associated AVP-gene mutations that are already described in literature. Three members of a German family with neurohypophyseal diabetes insipidus were studied. Isolated DNA from peripheral blood samples was used for mutation analysis by sequencing the whole coding region of AVP-NPII gene. Furthermore, we searched the electronic databases MEDLINE (Pubmed) as well as HGMD, LOVD-ClinVar, db-SNP and genomAD in order to compare our cases to that of other patients with FNDI. Genetic analysis of the patients revealed a novel heterozygote missense mutation in exon 2 of the AVP gene (c.274T>G), which has not yet been described in literature. We identified reports of more than 90 disease-associated mutations in the AVP gene in literature. The novel mutation of the AVP gene seems to cause FNDI in the presented German family. Similar to our newly detected mutation, most mutations causing FNDI are found in exon 2 of the AVP gene coding for neurophysin II. Clinically, it is important to think of FNDI in young children presenting with polydipsia and polyuria.
    MeSH term(s) Adult ; Diabetes Insipidus, Neurogenic/etiology ; Diabetes Insipidus, Neurogenic/pathology ; Female ; Genetic Testing ; Humans ; Male ; Middle Aged ; Mutation ; Neurophysins/genetics ; Pedigree ; Prognosis ; Protein Precursors/genetics ; Vasopressins/genetics ; Young Adult
    Chemical Substances AVP protein, human ; Neurophysins ; Protein Precursors ; Vasopressins (11000-17-2)
    Language English
    Publishing date 2020-07-06
    Publishing country Germany
    Document type Case Reports ; Journal Article ; Review
    ZDB-ID 80125-2
    ISSN 1439-4286 ; 0018-5043
    ISSN (online) 1439-4286
    ISSN 0018-5043
    DOI 10.1055/a-1175-1307
    Database MEDical Literature Analysis and Retrieval System OnLINE

    More links

    Kategorien

  9. Article: Giant bilateral adrenal lipoma in a patient with congenital adrenal hyperplasia.

    Kienitz, Tina / Schwander, Jörg / Bogner, Ulrich / Schwabe, Michael / Steinmüller, Thomas / Quinkler, Marcus

    Endocrinology, diabetes & metabolism case reports

    2021  Volume 2021

    Abstract: Summary: Apart from adrenal myelolipomas, adrenal lipomatous tumors are rare and only seldom described in the literature. We present the case of a 50-year-old man, with a classical form of congenital adrenal hyperplasia (CAH), which was well treated ... ...

    Abstract Summary: Apart from adrenal myelolipomas, adrenal lipomatous tumors are rare and only seldom described in the literature. We present the case of a 50-year-old man, with a classical form of congenital adrenal hyperplasia (CAH), which was well treated with prednisolone and fludrocortisone. The patient presented with pollakisuria and shortness of breath while bending over. On MRI, fat-equivalent masses were found in the abdomen (14 × 19 × 11 cm on the right side and 10 × 11 × 6 cm on the left side). The right adrenal mass was resected during open laparotomy and the pathohistological examination revealed the diagnosis of an adrenal lipoma. Symptoms were subdued totally postoperatively. This is the first report of a bilateral adrenal lipoma in a patient with CAH that we are aware of.
    Learning points: Macronodular hyperplasia is common in patients with congenital adrenal hyperplasia (CAH). Solitary adrenal tumors appear in approximately 10% of adult CAH patients and are often benign myelolipomas. The Endocrine Society Clinical Practice Guideline does not recommend routine adrenal imaging in adult CAH patients. Adrenal imaging should be performed in CAH patients with clinical signs for an adrenal or abdominal mass. Adrenal lipoma is rare and histopathological examinations should rule out a differentiated liposarcoma.
    Language English
    Publishing date 2021-03-31
    Publishing country England
    Document type Journal Article
    ZDB-ID 2785530-2
    ISSN 2052-0573
    ISSN 2052-0573
    DOI 10.1530/EDM-20-0204
    Database MEDical Literature Analysis and Retrieval System OnLINE

    More links

    Kategorien

  10. Article ; Online: Adrenal Crisis - Definition, Prevention and Treatment: Results from a Delphi Survey.

    Kienitz, Tina / Bechmann, Nicole / Deutschbein, Timo / Hahner, Stefanie / Honegger, Jürgen / Kroiss, Matthias / Quinkler, Marcus / Rayes, Nada / Reisch, Nicole / Willenberg, Holger Sven / Meyer, Gesine

    Hormone and metabolic research = Hormon- und Stoffwechselforschung = Hormones et metabolisme

    2023  Volume 56, Issue 1, Page(s) 10–15

    Abstract: Based on recent data, a total number of about 29 000 patients with adrenal insufficiency can be calculated for Germany, and about 1500 fatalities due to adrenal crises have to be expected within the next decade. Management of adrenal crises is still ... ...

    Abstract Based on recent data, a total number of about 29 000 patients with adrenal insufficiency can be calculated for Germany, and about 1500 fatalities due to adrenal crises have to be expected within the next decade. Management of adrenal crises is still unsatisfactory. The objectives of this study were to establish consensus for diagnostic criteria, prevention strategies, and treatment recommendations for adrenal crises. The study was conducted from January 2022 to April 2023, using Delphi technique. Four rounds of questionnaires were sent to 45 experts, selected by a coordinating group on behalf of the adrenal section of the German Society of Endocrinology. The survey was implemented online using the REDCap web application. Responses were captured anonymously. During the Delphi process the expert panel developed diagnostic criteria to identify patients likely to have an adrenal crisis. Education about adrenal insufficiency among patients as well as non-endocrine medical personnel were regarded as highly important. It was suggested that recommendations for the management of adrenal insufficiency have to be simplified and made widely available. This study provides pragmatic strategies to identify and treat patients prone to adrenal crisis, thereby highlighting the need for an improved management of patients with adrenal insufficiency.
    MeSH term(s) Humans ; Adrenal Insufficiency/diagnosis ; Adrenal Insufficiency/prevention & control ; Surveys and Questionnaires ; Endocrinology ; Germany/epidemiology
    Language English
    Publishing date 2023-08-10
    Publishing country Germany
    Document type Journal Article
    ZDB-ID 80125-2
    ISSN 1439-4286 ; 0018-5043
    ISSN (online) 1439-4286
    ISSN 0018-5043
    DOI 10.1055/a-2130-1938
    Database MEDical Literature Analysis and Retrieval System OnLINE

    More links

    Kategorien

To top