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Anne Cathrine Parelius Wammer Department of Endocrinology, Akershus University Hospital, Lørenskog, Norway

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Ingrid Nermoen Department of Endocrinology, Akershus University Hospital, Lørenskog, Norway
Institute of Clinical Medicine, University of Oslo, Oslo, Norway

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Per Medbøe Thorsby Institute of Clinical Medicine, University of Oslo, Oslo, Norway
Hormone Laboratory, Department of Medical Biochemistry and Biochemical Endocrinology and Metabolism Research Group, Oslo University Hospital, Aker, Norway

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Nils Bolstad Department of Medical Biochemistry, Oslo University Hospital, Oslo, Norway

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Kari Lima Department of Endocrinology, Akershus University Hospital, Lørenskog, Norway

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Hoa Tran Department of Haematology, Akershus University Hospital, Lørenskog, Norway

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Ivar Følling Department of Endocrinology, Akershus University Hospital, Lørenskog, Norway
Institute of Clinical Medicine, University of Oslo, Oslo, Norway

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Summary

We present a young woman with treatment resistant insulin autoimmune syndrome (IAS) with a protracted course. Her serum insulin level was 6945 pmol/l (<160), C-peptide 4042 pmol/L (<1480), anti-insulin antibodies 5305 U/mL (<0.4) were monoclonal IgG kappa. After 12 h of fasting, her blood glucose fell to 1.2 mmol/L. Post-meal blood glucose peaked at 12.2 mmol/L with reactive hypoglycaemia below 2 mmol/L. Frequent meals and continuous blood glucose monitoring were helpful, but further treatments advocated in the literature with prednisolone, rituximab, plasmapheresis, cyclophosphamide and ciclosporin were without beneficial effect.

Based on this case and a review of the literature, we propose that IAS is not one but two different diseases with different therapeutic strategies. The first disease, polyclonal IAS, predominates in Asia and is characterized by polyclonal anti-insulin antibodies, association with certain HLA genotypes and other autoimmune conditions, medications and viral infections possibly triggering the disease, a possible female predominance among young patients and a tendency towards spontaneous remission. The other disease, monoclonal IAS, predominates in Caucasians. Typical features are monoclonal anti-insulin antibodies, only weak HLA association, no drug predisposition, no sex difference, rare remission and conventional therapy often being without any clinical effect. We suggest that monoclonal IAS with IgG or IgA anti-insulin antibodies should receive therapy targeting plasma cells rather than lymphocytes.

Learning points

  • IAS may be considered as two separate diseases, polyclonal and monoclonal.

  • The presence of either polyclonal or monoclonal antibodies should determine the choice of treatment for IAS.

  • In polyclonal IAS, discontinuation of a triggering medication and treatment of triggering conditions should be the backbone of therapy.

  • Monoclonal IAS should receive treatment targeting plasma cells.

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Elinor Chelsom Vogt Department of Medicine, Haukeland University Hospital, Bergen, Norway
Department of Clinical Science and K.G. Jebsen-Center for Autoimmune Diseases, University of Bergen, Bergen, Norway

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Kathrin Hammerling Department of Oncology, Haukeland University Hospital, Bergen, Norway

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Halfdan Sorbye Department of Oncology, Haukeland University Hospital, Bergen, Norway
Department of Clinical Science and K.G. Jebsen-Center for Autoimmune Diseases, University of Bergen, Bergen, Norway

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Anette Heie Department of Surgery, Haukeland University Hospital, Bergen, Norway

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Andre Sulen Department of Clinical Science and K.G. Jebsen-Center for Autoimmune Diseases, University of Bergen, Bergen, Norway

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Grethe Ueland Department of Medicine, Haukeland University Hospital, Bergen, Norway

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Eystein Husebye Department of Medicine, Haukeland University Hospital, Bergen, Norway
Department of Clinical Science and K.G. Jebsen-Center for Autoimmune Diseases, University of Bergen, Bergen, Norway

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Paal Methlie Department of Medicine, Haukeland University Hospital, Bergen, Norway

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Summary

Feminizing estrogen-secreting adrenocortical carcinomas (ACCs) are exceedingly rare and carry a poor prognosis. The most common presenting trait is gynecomastia, but enlarged breasts are also a frequent clinical finding in healthy men. Biochemical evaluation may be challenging. As such, there is a high risk of delayed diagnosis and treatment opportunity. Here, we present a case with an estrogen-producing ACC where the abnormal steroid profile obtained at the time of initial workup was essential for the prompt diagnosis. Wider adoption of liquid chromatography mass spectrometry-based steroid assays has potential to improve early diagnosis of feminizing estrogen-secreting ACC.

Learning points

  • Feminizing estrogen-secreting adrenocortical carcinomas (ACCs) are a rare, but an important differential diagnosis in men with rapidly developing gynecomastia.

  • Biochemical evaluation is essential for a prompt diagnosis.

  • Steroid hormone profiling using liquid chromatography mass spectrometry technology has the potential to improve early diagnosis of feminizing estrogen-secreting ACC.

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Ann-Elin Meling Stokland Department of Endocrinology, Stavanger University Hospital, Stavanger, Norway

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Anne Lise Dahle Department of Internal Medicine, Haugesund Hospital, Haugesund, Norway

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Vidar Laurits Kloster Department of Radiology, Haugesund Hospital, Haugesund, Norway

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Torbjørn Nedrebø Department of Anaesthesia, Haraldsplass Deaconess Hospital, Bergen, Norway
Department of Clinical Science, University of Bergen, Bergen, Norway

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Bjørn Gunnar Nedrebø Department of Internal Medicine, Haugesund Hospital, Haugesund, Norway
Department of Clinical Science, University of Bergen, Bergen, Norway

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Summary

Myxedema coma is an important differential diagnosis in critically ill patients. Early diagnosis and treatment are paramount but challenging due to a lack of diagnostic criteria. We report a case about a patient who suffered from untreated hypothyroidism for several years. Before the correct diagnosis was made, he was admitted three times due to severe constipation. Eventually, he developed myxedema coma in connection with a urinary tract infection. The course was complicated by recurrent seizures, and neuroimaging showed bilateral hygromas. Hormone replacement therapy resulted in complete recovery and regression of hygromas. To the best of our knowledge, this is the first time hygroma is reported in association with myxedema coma.

Learning points

  • Myxedema coma is a difficult diagnosis to make due to a lack of diagnostic criteria.

  • Cardinal features include hypothermia, bradycardia, gastrointestinal symptoms, pericardial/pleural effusions and affection of CNS. Anemia and hyponatremia are common.

  • In case of suspected myxedema coma, neuroimaging should be a part of the evaluation in most cases.

  • There is a possible association between longstanding/severe hypothyroidism and hygroma.

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Kaja Grønning Department of Endocrinology, Akershus University Hospital, Lorenskog, Norway

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Archana Sharma Department of Endocrinology, Akershus University Hospital, Lorenskog, Norway

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Maria Adele Mastroianni Department of Haematology, Akershus University Hospital, Lorenskog, Norway

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Bo Daniel Karlsson Department of Radiology, Akershus University Hospital, Lorenskog, Norway

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Eystein S Husebye Department of Clinical Science and K.G. Jebsen Center of Autoimmune Disorders, University of Bergen, Bergen, Norway
Department of Medicine, Haukeland University Hospital, Bergen, Norway

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Kristian Løvås Department of Clinical Science and K.G. Jebsen Center of Autoimmune Disorders, University of Bergen, Bergen, Norway
Department of Medicine, Haukeland University Hospital, Bergen, Norway

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Ingrid Nermoen Department of Endocrinology, Akershus University Hospital, Lorenskog, Norway
Institute of Clinical Medicine, Faculty of Medicine, University of Oslo, Oslo, Norway

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Summary

Primary adrenal lymphoma (PAL) is a rare cause of adrenal insufficiency. More than 90% is of B-cell origin. The condition is bilateral in up to 75% of cases, with adrenal insufficiency in two of three patients. We report two cases of adrenal insufficiency presenting at the age of 70 and 79 years, respectively. Both patients had negative 21-hydroxylase antibodies with bilateral adrenal lesions on CT. Biopsy showed B-cell lymphoma. One of the patients experienced intermittent disease regression on replacement dosage of glucocorticoids.

Learning points:

  • Primary adrenal lymphoma (PAL) is a rare cause of adrenal insufficiency.

  • Bilateral adrenal masses of unknown origin or in individuals with suspected extra-adrenal malignancy should be biopsied quickly when pheochromocytoma is excluded biochemically.

  • Steroid treatment before biopsy may affect diagnosis.

  • Adrenal insufficiency with negative 21-hydroxylase antibodies should be evaluated radiologically.

Open access