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Wahlberg, Jeanette
Alternative names
Publications (10 of 31) Show all publications
Svanberg, C., Norevall, L.-I., Ekman, B., Wahlberg Topp, J. & Bågesund, M. (2016). Cephalometric analysis of adults with Turner syndrome. Swedish Dental Journal, 40(1), 33-41
Open this publication in new window or tab >>Cephalometric analysis of adults with Turner syndrome
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2016 (English)In: Swedish Dental Journal, ISSN 0347-9994, Vol. 40, no 1, p. 33-41Article in journal (Refereed) Published
Abstract [en]

Turner syndrome (TS) is a genetic disorder of females with a prevalence of 1/2000-3000 live female births. The aim of this study was to compare cephalometric variables from adult women diagnosed with TS to a standardized reference group of 31-year old healthy women, and to evaluate the possible effects of human growth hormone (hGH) therapy in women with TS. Registered TS subjects in the Southeast region of Sweden were invited to take part in the study. Twenty-one women aged 36 +/- 13(18-57) years accepted participation. Lateral radiographs of the head were analyzed using standard cephalometric methods (Hasund analysis) and with the commercially available soft-ware program FACAD. Comparisons were made with roentgen-cephalometric standards from a reference group of nineteen 31-year old Swedish women. Analysis of the cephalometric radiographs from the TS subjects showed a more retrognathic maxilla (SNA 80.3 +/- 5.4) (p=0.0460) and mandible (SNB 77.0 +/- 5.2) (p=0.0014), and a correspondingly backward position of the chin (SN/Pg 78.9 +/- 5.5) (p=0.0046) as compared to the reference values of 31-year old women (SNA 83.2 +/- 3.0, SNB 81.5 +/- 2.3 and SNPg 83.0 +/- 2.3, respectively). In addition there was an increased posterior inclination of the maxilla (SN/NL 8.6 +/- 4.1), as compared to the reference values (SN/NL 5,3 +/- 2.7) (p=0.0048). There were no significant differences regarding sagittal or vertical jaw relations, mandibular inclination or cranial base angle between the TS-group and the 31-year olds with the reference values. No significant difference was seen in jaw relationship, as measured by the ANB value, however the Wits(index) (3.3 +/- 3.5) was higher (p=0.0001) than the reference values (-0.1 +/- 1.8). Subjects with or without previous hGH administration did not show any significant differences in cephalometric values. In conclusion, women with TS had a significantly more retrognathic maxilla (SNA) and mandible (SNB) and a correspondingly significantly posterior position of the chin (SN/Pg), a significantly increased posterior inclination of the maxilla (SN/NL) and a significantly increased Witsindex as compared to the reference group of 31-year old women. No craniofacial variables differed significantly between previously hGH-treated and not hGH-treated women with TS.

Place, publisher, year, edition, pages
SWEDISH DENTAL JOURNAL, 2016
Keywords
Turner syndrome; cephalometric analysis; growth hormone
National Category
Public Health, Global Health, Social Medicine and Epidemiology Dentistry
Identifiers
urn:nbn:se:liu:diva-128984 (URN)000374716300005 ()
Note

Funding Agencies|Folktandvarden Ostergotland; Medical Research Council of Southeast Sweden

Available from: 2016-06-09 Created: 2016-06-07 Last updated: 2018-03-19
Wahlberg Topp, J., Ekman, B., Nystrom, L., Hanson, U., Persson, B. & Arnqvist, H. (2016). Gestational diabetes: Glycaemic predictors for fetal macrosomia and maternal risk of future diabetes. Diabetes Research and Clinical Practice, 114, 99-105
Open this publication in new window or tab >>Gestational diabetes: Glycaemic predictors for fetal macrosomia and maternal risk of future diabetes
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2016 (English)In: Diabetes Research and Clinical Practice, ISSN 0168-8227, E-ISSN 1872-8227, Vol. 114, p. 99-105Article in journal (Refereed) Published
Abstract [en]

Aims: To investigate how glucose levels at diagnosis of gestational diabetes (GDM) are associated with infant birth weight and long-term risk of manifest diabetes mellitus in the mother. Methods: In a case control study GDM pregnancies (n = 2085) were compared with non-GDM pregnancies matched for day of delivery and obstetric unit (n = 3792). GDM was defined as capillary blood glucose (cB-glucose) >9.0 mmol/l (plasma glucose >10.0 mmol/l) after a 75 g oral glucose tolerance test (OGTT). The GDM cohort were followed up 8.5-13.5 yrs after initial diagnosis with a questionnaire, answered by 1324 GDM women (65%). Results: GDM women had higher mean infant birth-weight compared with controls (3682 g vs. 3541 g, P < 0.001). In multiple linear regression analysis, birth weight was positively correlated to fasting cB-glucose at GDM diagnosis (P < 0.001), increased week of gestation (P < 0.001) and BMI before pregnancy (P < 0.003), while 2 h OGTT cB-glucose values >= 9.0 mmol/l were not related. Infants born to mothers with fasting cB-glucose >= 4.5 mmol/l had no increased mean birth-weight or macrosomia (>= 4500 g) compared to controls. In the follow up 334/1324 women (25%) of the GDM women had developed diabetes, 215 type 2 diabetes, 46 type 1 diabetes and 72 unclassified diabetes. In logistic regression fasting cB-glucose and 2 h OGTT cB-glucose at diagnosis of GDM as well as BMI >25 and origin outside Europe were risk factors for manifest diabetes. Conclusions: Fasting blood glucose at diagnosis of GDM gives important information besides 2 h OGTT glucose about pregnancy outcome and future risk for maternal diabetes. (C) 2016 Elsevier Ireland Ltd. All rights reserved.

Place, publisher, year, edition, pages
ELSEVIER IRELAND LTD, 2016
Keywords
Birth weight; Pregnancy; GDM; Blood glucose; OGTT
National Category
Clinical Medicine
Identifiers
urn:nbn:se:liu:diva-128751 (URN)10.1016/j.diabres.2015.12.017 (DOI)000375129600015 ()26818892 (PubMedID)
Note

Funding Agencies|Swedish Diabetes Association; Medical Research Council of Southeast Sweden (FORSS); Linkoping University, Sweden

Available from: 2016-05-31 Created: 2016-05-30 Last updated: 2017-04-24
Burman, P., Eden-Engstrom, B., Ekman, B., Anders Karlsson, F., Schwarcz, E. & Wahlberg Topp, J. (2016). Limited value of cabergoline in Cushings disease: a prospective study of a 6-week treatment in 20 patients. European Journal of Endocrinology, 174(1), 17-24
Open this publication in new window or tab >>Limited value of cabergoline in Cushings disease: a prospective study of a 6-week treatment in 20 patients
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2016 (English)In: European Journal of Endocrinology, ISSN 0804-4643, E-ISSN 1479-683X, Vol. 174, no 1, p. 17-24Article in journal (Refereed) Published
Abstract [en]

Context and objective: The role of cabergoline in Cushings disease (CD) remains controversial. The experience is limited to case reports and few open studies that report the effects determined after >= 1 month of treatment. In prolactinomas and dopamine-responsive GH-secreting tumours, effects of cabergoline are seen within days or weeks. Here, we searched for short-term effects of cabergoline in CD. Design: Twenty patients (19 naive and one recurrent) were included in a prospective study. Cabergoline was administered in increasing doses of 0.5-5 mg/week over 6 weeks. Methods: Urinary free cortisol (UFC) 24 h, morning cortisol and ACTH, and salivary cortisol at 0800, 1600 and 2300 h were determined once weekly throughout. Diurnal curves (six samples) of serum cortisol were measured at start and end. Results: At study end, the median cabergoline dose was 5 mg, range 2.5-5 mg/week. The prolactin levels, markers of compliance, were suppressed in all patients. During the treatment, hypercortisolism varied, gradual and dose-dependent reductions were not seen. Five patients had a >50% decrease of UFC, three had a >50% rise of UFC. Salivary cortisol at 2300 h showed a congruent >50% change with UFC in two of the five cases with decreased UFC, and in one of the three cases with increased UFC. One patient with decreases in both UFC and 2300 h salivary cortisol also had a reduction in diurnal serum cortisol during the course of the study. Conclusions: Cabergoline seems to be of little value in the management of CD. Only one patient had a response-like pattern. Given the known variability of disease activity in CD, this might represent a chance finding.

Place, publisher, year, edition, pages
BIOSCIENTIFICA LTD, 2016
National Category
Clinical Medicine
Identifiers
urn:nbn:se:liu:diva-124115 (URN)10.1530/EJE-15-0807 (DOI)000366401700007 ()26582653 (PubMedID)
Note

Funding Agencies|Swedish Association of Local Authorities and Regions to the Swedish Pituitary Study Group; University of Lund; Linkoping; Uppsala

Available from: 2016-01-22 Created: 2016-01-19 Last updated: 2017-04-24
Landegren, N., Sharon, D., Freyhult, E., Hallgren, A., Eriksson, D., Edqvist, P.-H., . . . Kampe, O. (2016). Proteome-wide survey of the autoimmune target repertoire in autoimmune polyendocrine syndrome type 1. Scientific Reports, 6(20104)
Open this publication in new window or tab >>Proteome-wide survey of the autoimmune target repertoire in autoimmune polyendocrine syndrome type 1
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2016 (English)In: Scientific Reports, ISSN 2045-2322, E-ISSN 2045-2322, Vol. 6, no 20104Article in journal (Refereed) Published
Abstract [en]

Autoimmune polyendocrine syndrome type 1 (APS1) is a monogenic disorder that features multiple autoimmune disease manifestations. It is caused by mutations in the Autoimmune regulator (AIRE) gene, which promote thymic display of thousands of peripheral tissue antigens in a process critical for establishing central immune tolerance. We here used proteome arrays to perform a comprehensive study of autoimmune targets in APS1. Interrogation of established autoantigens revealed highly reliable detection of autoantibodies, and by exploring the full panel of more than 9000 proteins we further identified MAGEB2 and PDILT as novel major autoantigens in APS1. Our proteome-wide assessment revealed a marked enrichment for tissue-specific immune targets, mirroring AIREs selectiveness for this category of genes. Our findings also suggest that only a very limited portion of the proteome becomes targeted by the immune system in APS1, which contrasts the broad defect of thymic presentation associated with AIRE-deficiency and raises novel questions what other factors are needed for break of tolerance.

Place, publisher, year, edition, pages
NATURE PUBLISHING GROUP, 2016
National Category
Health Sciences
Identifiers
urn:nbn:se:liu:diva-125308 (URN)10.1038/srep20104 (DOI)000368996700001 ()26830021 (PubMedID)
Note

Funding Agencies|Swedish Research Council; Formas Research Council; Torsten Soderberg Foundation; Ragnar Soderberg Foundation; Novonordisk Foundation; Intramural Research Program, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland, USA

Available from: 2016-02-24 Created: 2016-02-19 Last updated: 2017-04-24
Bergthorsdottir, R., Nilsson, A. G., Gillberg, P., Ekman, B. & Wahlberg, J. (2015). Health-Related Quality of Life In Patients With Adrenal Insufficiency Receiving Plenadren Compared With Immediate-Release Hydrocortisone.. Value in Health, 18(7), A616
Open this publication in new window or tab >>Health-Related Quality of Life In Patients With Adrenal Insufficiency Receiving Plenadren Compared With Immediate-Release Hydrocortisone.
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2015 (English)In: Value in Health, ISSN 1098-3015, E-ISSN 1524-4733, Vol. 18, no 7, p. A616-Article in journal, Meeting abstract (Refereed) Published
Abstract [en]

Background

Previous studies in patients with primary adrenal insufficiency (PAI) on conventional replacement therapy suggest decreased health-related quality of life (HRQoL), and that patients report more frequently fatigue, increased anxiety and inability to work compared to background population.

Objectives

To study self-reported health status with EQ-5D in patients with PAI. Patients treated with Plenadren (modified-release hydrocortisone) were compared with patients treated with immediate release hydrocortisone (IRHC) replacement therapy.

Methods

This was a cross-sectional, multi-centre, non-interventional survey of patients with PAI receiving Plenadren or immediate release hydrocortisone (IRHC) replacement.

Subjects

One hundred thirty-four adult patients with PAI of whom 36 (19 females [53%]) were treated with Plenadren and 98 (77 females [79%]) were treated with IRHC, were included.

MAIN OUTCOME MEASURE

HRQoL described by the EQ-5D, a generic preference-based measure of health.

RESULTS

Patients on Plenadren and on IRHC had a mean ± SD age of 53.1 ± 12.7 years and 48.0 ± 13.1 years, respectively (P=0.043). The majority of the patients were diagnosed more than 5 years ago (69%). The mean ± SD daily Plenadren and IRHC doses were 27.0 ± 6.8 mg and 26.6 ± 10.9 mg, respectively (P=0.807). 47% of the Plenadren patients had been receiving Plenadren and 82% of the IRHC patients had been receiving IRHC for more than 3 years. Patients receiving Plenadren had better HRQoL measured by the EQ-5D questionnaire compared to patients replaced with IRHC (0.76 ± 0.18 vs 0.68 ± 0.18, respectively [P=0.040]).

CONCLUSIONS

Replacement therapy with Plenadren in patients with PAI confers measurable benefit on HRQoL relative to IRHC as estimated by the EQ-5D questionnaire, and may therefore be advantageous when compared to IRHC substitution.

National Category
Health Care Service and Management, Health Policy and Services and Health Economy Surgery
Identifiers
urn:nbn:se:liu:diva-124656 (URN)10.1016/j.jval.2015.09.2145 (DOI)26533455 (PubMedID)
Available from: 2016-02-09 Created: 2016-02-08 Last updated: 2017-11-30
Ekman, B., Wahlberg Topp, J. & Landberg, E. (2015). Urine oligosaccharide pattern in patients with hyperprolactinaemia. Glycoconjugate Journal, 32(8), 635-641
Open this publication in new window or tab >>Urine oligosaccharide pattern in patients with hyperprolactinaemia
2015 (English)In: Glycoconjugate Journal, ISSN 0282-0080, E-ISSN 1573-4986, Vol. 32, no 8, p. 635-641Article in journal (Refereed) Published
Abstract [en]

Free milk-type oligosaccharides are produced during pregnancy and lactation and may have an impact on several cells in the immune system. Our aim was to investigate if patients with isolated hyperprolactinaemia, not related to pregnancy, also have increased synthesis and urinary excretion of milk-type oligosaccharides and to compare the excretion pattern with that found during pregnancy. Urine samples were collected as morning sample from 18 patients with hyperprolactinaemia, 13 healthy controls with normal prolactin levels and four pregnant women. After purification, lactose and free oligosaccharides were analysed and quantified by high-performance anion-exchange chromatography with pulsed amperometric detection. The identity of peaks was confirmed by exoglycosidase treatment and comparison with oligosaccharide standards. Prolactin was measured in serum collected between 09 and 11 a.m. by a standardized immunochemical method. Patients with hyperprolactinaemia had higher urinary excretion of lactose than normoprolactinemic controls and urinary lactose correlated positively to prolactin levels (r = 0.51, p less than 0.05). Increased levels of the fucosylated oligosaccharides 2-fucosyl lactose and lacto-di-fucotetraose were found in urine from three and two patients, respectively. The acidic oligosaccharide 3-sialyl lactose was found in high amount in urine from two patients with prolactin of greater than 10,000 mU/l. However, pregnant women in their third trimester had the highest concentration of all these oligosaccharides and excretion increased during pregnancy. This study is first to show that both lactose and certain fucosylated and sialylated milk-type oligosaccharides are increased in some patients with hyperprolactinaemia. It remains to elucidate the functional importance of these findings.

Place, publisher, year, edition, pages
SPRINGER, 2015
Keywords
Prolactin; Prolactinoma; Urine; Oligosaccharides; Lactose
National Category
Clinical Medicine
Identifiers
urn:nbn:se:liu:diva-122650 (URN)10.1007/s10719-015-9610-x (DOI)000363488300006 ()26275984 (PubMedID)
Note

Funding Agencies|FORSS (Medical Research Council of Southeast Sweden) [4065]; Faculty of Health and Sciences, Linkoping University, Sweden

Available from: 2015-11-16 Created: 2015-11-13 Last updated: 2017-12-01
Ekman, B., Alstrand, N., Bachrach-Lindström, M., Jenmalm, M. C. & Wahlberg, J. (2014). Altered Chemokine Th1/Th2 Balance in Addison's Disease: Relationship with Hydrocortisone Dosing and Quality of Life. Hormone and Metabolic Research, 46(1), 48-53
Open this publication in new window or tab >>Altered Chemokine Th1/Th2 Balance in Addison's Disease: Relationship with Hydrocortisone Dosing and Quality of Life
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2014 (English)In: Hormone and Metabolic Research, ISSN 0018-5043, E-ISSN 1439-4286, Vol. 46, no 1, p. 48-53Article in journal (Refereed) Published
Abstract [en]

The adrenalitis found in autoimmune Addison’s disease (AAD) is considered having a Th1-driven pathogenesis. Circulating Th1- and Th2-associated chemokines responsible for the trafficking of leukocytes to inflammatory sites are markers for the Th1/Th2 balance. The aim of the study was to assess if the same daily hydrocortisone dose of 30 mg given in either 2 or 4 doses to patients with AAD could affect the Th1/Th2 balance of circulating chemokines.

Fifteen patients (6 women) with AAD were included in this randomised, placebo controlled, double blind cross-over study. Samples for chemokines, Th1-associated (CXCL10, CXCL11) and Th2-associated (CCL17, CCL22), were drawn 5 times during a 24-h period at the end of each treatment period and analysed with Luminex. Seven control subjects did the same diurnal blood sampling once. Subjects with AAD had higher median diurnal levels of the Th1-associated chemokines than controls, CXCL10 [43 (33–56) pg/ml vs. 22 (19–34) pg/ml, p<0.01] and CXCL11 [37 (29–48) pg/ml vs. 16 (9–24) pg/ml, p<0.001], whereas no significant difference was found regarding the Th2-related chemokines. Similar chemokine levels were found when the same hydrocortisone dose of 30 mg was divided in 2 or 4 doses. Levels of CXCL11 correlated negatively with scores of SF-36 domains (high score indicate better health) of General Health (GH) and total score for Physical Component Summary (PCS), and these negative correlations were most pronounced at 04:00 h on the 2-dose regimen. Patients with AAD have a dominant Th1 chemokine profile that partially correlates to reduced quality of life.

National Category
Endocrinology and Diabetes
Identifiers
urn:nbn:se:liu:diva-104003 (URN)10.1055/s-0033-1351291 (DOI)000329563500004 ()
Available from: 2014-02-06 Created: 2014-02-06 Last updated: 2019-01-09
Mitchell, A. L., Macarthur, K. D. R., Gan, E. H., Baggott, L. E., Wolff, A. S. B., Skinningsrud, B., . . . Pearce, S. H. S. (2014). Association of Autoimmune Addisons Disease with Alleles of STAT4 and GATA3 in European Cohorts. PLoS ONE, 9(3), 0088991
Open this publication in new window or tab >>Association of Autoimmune Addisons Disease with Alleles of STAT4 and GATA3 in European Cohorts
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2014 (English)In: PLoS ONE, ISSN 1932-6203, E-ISSN 1932-6203, Vol. 9, no 3, p. 0088991-Article in journal (Refereed) Published
Abstract [en]

Background: Gene variants known to contribute to Autoimmune Addisons disease (AAD) susceptibility include those at the MHC, MICA, CIITA, CTLA4, PTPN22, CYP27B1, NLRP-1 and CD274 loci. The majority of the genetic component to disease susceptibility has yet to be accounted for. Aim: To investigate the role of 19 candidate genes in AAD susceptibility in six European case-control cohorts. Methods: A sequential association study design was employed with genotyping using Sequenom iPlex technology. In phase one, 85 SNPs in 19 genes were genotyped in UK and Norwegian AAD cohorts (691 AAD, 715 controls). In phase two, 21 SNPs in 11 genes were genotyped in German, Swedish, Italian and Polish cohorts (1264 AAD, 1221 controls). In phase three, to explore association of GATA3 polymorphisms with AAD and to determine if this association extended to other autoimmune conditions, 15 SNPs in GATA3 were studied in UK and Norwegian AAD cohorts, 1195 type 1 diabetes patients from Norway, 650 rheumatoid arthritis patients from New Zealand and in 283 UK Graves disease patients. Meta-analysis was used to compare genotype frequencies between the participating centres, allowing for heterogeneity. Results: We report significant association with alleles of two STAT4 markers in AAD cohorts (rs4274624: P = 0.00016; rs10931481: P = 0.0007). In addition, nominal association of AAD with alleles at GATA3 was found in 3 patient cohorts and supported by meta-analysis. Association of AAD with CYP27B1 alleles was also confirmed, which replicates previous published data. Finally, nominal association was found at SNPs in both the NF-kappa B1 and IL23A genes in the UK and Italian cohorts respectively. Conclusions: Variants in the STAT4 gene, previously associated with other autoimmune conditions, confer susceptibility to AAD. Additionally, we report association of GATA3 variants with AAD: this adds to the recent report of association of GATA3 variants with rheumatoid arthritis.

Place, publisher, year, edition, pages
Public Library of Science, 2014
National Category
Medical and Health Sciences
Identifiers
urn:nbn:se:liu:diva-106092 (URN)10.1371/journal.pone.0088991 (DOI)000332839300009 ()
Available from: 2014-04-25 Created: 2014-04-24 Last updated: 2017-12-05
Nilsson, A. G., Marelli, C., Fitts, D., Bergthorsdottir, R., Burman, P., Dahlqvist, P., . . . Johannsson, G. (2014). Prospective evaluation of long-term safety of dual-release hydrocortisone replacement administered once daily in patients with adrenal insufficiency. European Journal of Endocrinology, 171(3), 369-377
Open this publication in new window or tab >>Prospective evaluation of long-term safety of dual-release hydrocortisone replacement administered once daily in patients with adrenal insufficiency
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2014 (English)In: European Journal of Endocrinology, ISSN 0804-4643, E-ISSN 1479-683X, Vol. 171, no 3, p. 369-377Article in journal (Refereed) Published
Abstract [en]

Objective: The objective was to assess the long-term safety profile of dual-release hydrocortisone (DR-HC) in patients with adrenal insufficiency (AI). Design: Randomised, open-label, crossover trial of DR-HC or thrice-daily hydrocortisone for 3 months each (stage 1) followed by two consecutive, prospective, open-label studies of DR-HC for 6 months (stage 2) and 18 months (stage 3) at five university clinics in Sweden. Methods: Sixty-four adults with primary AI started stage 1, and an additional 16 entered stage 3. Patients received DR-HC 20-40 mg once daily and hydrocortisone 20-40 mg divided into three daily doses (stage 1 only). Main outcome measures were adverse events (AEs) and intercurrent illness (self-reported hydrocortisone use during illness). Results: In stage 1, patients had a median 1.5 (range, 1-9) intercurrent illness events with DR-HC and 1.0 (1-8) with thrice-daily hydrocortisone. AEs during stage 1 were not related to the cortisol exposure-time profile. The percentage of patients with one or more AEs during stage 1 (73.4% with DR-HC; 65.6% with thrice-daily hydrocortisone) decreased during stage 2, when all patients received DR-HC (51% in the first 3 months; 54% in the second 3 months). In stages 1-3 combined, 19 patients experienced 27 serious AEs, equating to 18.6 serious AEs/100 patient-years of DR-HC exposure. Conclusions: This long-term prospective trial is the first to document the safety of DR-HC in patients with primary AI and demonstrates that such treatment is well tolerated during 24 consecutive months of therapy.

Place, publisher, year, edition, pages
BioScientifica, 2014
National Category
Clinical Medicine
Identifiers
urn:nbn:se:liu:diva-112489 (URN)10.1530/EJE-14-0327 (DOI)000343670900015 ()24944332 (PubMedID)
Note

Funding Agencies|ViroPharma SPRL, Maidenhead, UK

Available from: 2014-11-28 Created: 2014-11-28 Last updated: 2017-12-05
Wahlberg, J. & Ekman, B. (2013). Atypical or typical adrenocorticotropic hormone-producing pulmonary carcinoids and the usefulness of 11C-5-hydroxytryptophan positron emission tomography: two case reports. Journal of Medical Case Reports, 7, 80
Open this publication in new window or tab >>Atypical or typical adrenocorticotropic hormone-producing pulmonary carcinoids and the usefulness of 11C-5-hydroxytryptophan positron emission tomography: two case reports
2013 (English)In: Journal of Medical Case Reports, ISSN 1752-1947, E-ISSN 1752-1947, Vol. 7, p. 80-Article in journal (Refereed) Published
Abstract [en]

Introduction

Pulmonary carcinoids associated with ectopic adrenocorticotropic hormone secretion have a good prognosis if histological examination shows typical pulmonary carcinoid and low proliferation, whereas a poor outcome is linked to atypical pulmonary carcinoid and high proliferation. Here we describe the diagnostic challenges to find the tumor in Cushing’s syndrome secondary to ectopic adrenocorticotropic hormone secretion in two cases with an atypical and a typical pulmonary carcinoid, respectively.     

Case presentation

A 63-year-old Caucasian woman presented with aggressive clinical features related to Cushing’s syndrome, having very high levels of urinary cortisol and circulating adrenocorticotropic hormone and cortisol. Magnetic resonance imaging showed no pituitary tumor, and bilateral inferior petrosal sinus sampling revealed no central peripheral ratio of adrenocorticotropic hormone. Computed tomography and 111Indium-pentetreoide somatostatin receptor scintigraphy could not visualize any ectopic tumor. The patient was referred for an 11C-5-hydroxytryptophan positron emission tomography, and a small 8mm nodule in her left lung was found. The tumor was removed via a lateral thoracic incision and wedge excision. The histological examination showed an atypical carcinoid with Ki-67 index of 9 to 10%, and an additional lobectomy was performed.     

The second patient, a 22-year-old Caucasian man, also presented with aggressive Cushing’s syndrome, with very high urinary cortisol levels and increased circulating cortisol as well as adrenocorticotropic hormone levels. A magnetic resonance imaging scan of the pituitary showed no tumor, whereas a 12×9×14mm tumor was detected in the right lung on the primary computed tomography scan and no further investigation was performed. The tumor was removed via a lateral thoracic incision and wedge excision. A typical carcinoid with Ki-67 index of 1 to 2% was found and no further surgery was performed.     

After surgical removal, the biochemical disturbances resolved and significant clinical improvement were achieved in both patients after 24 months of follow up.     

Conclusions

Diagnostic evaluation time is limited due to the aggressive course in ectopic adrenocorticotropic hormone-dependent Cushing’s syndrome. We suggest that 11C-5-hydroxytryptophan positron emission tomography could be considered early as a secondary diagnostic tool when primary computed tomography and/or magnetic resonance imaging scans fail to show any tumor.

Place, publisher, year, edition, pages
BioMed Central, 2013
Keywords
ACTH; ACTH syndrome; Cortisol; Cushing’s syndrome; Ectopic; Pulmonary carcinoid
National Category
Medical and Health Sciences
Identifiers
urn:nbn:se:liu:diva-102327 (URN)10.1186/1752-1947-7-80 (DOI)
Available from: 2013-12-05 Created: 2013-12-05 Last updated: 2017-12-06Bibliographically approved
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