Browse

You are looking at 1 - 10 of 47 items

Tomomi Nakao First Department of Internal Medicine, Wakayama Medical University, Wakayama City, Wakayama, Japan

Search for other papers by Tomomi Nakao in
Google Scholar
PubMed
Close
,
Ken Takeshima First Department of Internal Medicine, Wakayama Medical University, Wakayama City, Wakayama, Japan

Search for other papers by Ken Takeshima in
Google Scholar
PubMed
Close
,
Hiroyuki Ariyasu First Department of Internal Medicine, Wakayama Medical University, Wakayama City, Wakayama, Japan

Search for other papers by Hiroyuki Ariyasu in
Google Scholar
PubMed
Close
,
Chiaki Kurimoto First Department of Internal Medicine, Wakayama Medical University, Wakayama City, Wakayama, Japan

Search for other papers by Chiaki Kurimoto in
Google Scholar
PubMed
Close
,
Shinsuke Uraki First Department of Internal Medicine, Wakayama Medical University, Wakayama City, Wakayama, Japan

Search for other papers by Shinsuke Uraki in
Google Scholar
PubMed
Close
,
Shuhei Morita First Department of Internal Medicine, Wakayama Medical University, Wakayama City, Wakayama, Japan

Search for other papers by Shuhei Morita in
Google Scholar
PubMed
Close
,
Yasushi Furukawa First Department of Internal Medicine, Wakayama Medical University, Wakayama City, Wakayama, Japan

Search for other papers by Yasushi Furukawa in
Google Scholar
PubMed
Close
,
Hiroshi Iwakura First Department of Internal Medicine, Wakayama Medical University, Wakayama City, Wakayama, Japan

Search for other papers by Hiroshi Iwakura in
Google Scholar
PubMed
Close
, and
Takashi Akamizu First Department of Internal Medicine, Wakayama Medical University, Wakayama City, Wakayama, Japan

Search for other papers by Takashi Akamizu in
Google Scholar
PubMed
Close

Summary

Thyroid storm (TS) is a life-threatening condition that may suffer thyrotoxic patients. Therapeutic plasma exchange (TPE) is a rescue approach for TS with acute hepatic failure, but it should be initiated with careful considerations. We present a 55-year-old male patient with untreated Graves’ disease who developed TS. Severe hyperthyroidism and refractory atrial fibrillation with congestive heart failure aggregated to multiple organ failure. The patient was recovered by intensive multimodal therapy, but we had difficulty in introducing TPE treatment considering the risk of exacerbation of congestive heart failure due to plasma volume overload. In addition, serum total bilirubin level was not elevated in the early phase to the level of indication for TPE. The clinical course of this patient instructed delayed elevation of bilirubin until the level of indication for TPE in some patients and also demonstrated the risk of exacerbation of congestive heart failure by TPE.

Learning points:

  • Our patient with thyroid storm could be diagnosed and treated promptly using Japan Thyroid Association guidelines for thyroid storm.

  • Delayed elevation of serum bilirubin levels could make the decision of introducing therapeutic plasma exchange difficult in cases of thyroid storm with acute hepatic failure.

  • The risk of worsening congestive heart failure should be considered carefully when performing therapeutic plasma exchange.

Open access
Agnieszka Łebkowska Department of Internal Medicine and Metabolic Diseases, Diabetology and Internal Medicine

Search for other papers by Agnieszka Łebkowska in
Google Scholar
PubMed
Close
,
Anna Krentowska Department of Internal Medicine and Metabolic Diseases, Diabetology and Internal Medicine

Search for other papers by Anna Krentowska in
Google Scholar
PubMed
Close
,
Agnieszka Adamska Department of Endocrinology, Diabetology and Internal Medicine

Search for other papers by Agnieszka Adamska in
Google Scholar
PubMed
Close
,
Danuta Lipińska Department of Endocrinology, Diabetology and Internal Medicine

Search for other papers by Danuta Lipińska in
Google Scholar
PubMed
Close
,
Beata Piasecka Department of Endocrinology, Diabetology and Internal Medicine

Search for other papers by Beata Piasecka in
Google Scholar
PubMed
Close
,
Otylia Kowal-Bielecka Department of Rheumatology and Internal Diseases, Medical University of Bialystok, Bialystok, Poland

Search for other papers by Otylia Kowal-Bielecka in
Google Scholar
PubMed
Close
,
Maria Górska Department of Endocrinology, Diabetology and Internal Medicine

Search for other papers by Maria Górska in
Google Scholar
PubMed
Close
,
Robert K Semple Centre for Cardiovascular Science, Queen’s Medical Research Institute, University of Edinburgh, Edinburgh, UK

Search for other papers by Robert K Semple in
Google Scholar
PubMed
Close
, and
Irina Kowalska Department of Internal Medicine and Metabolic Diseases, Diabetology and Internal Medicine

Search for other papers by Irina Kowalska in
Google Scholar
PubMed
Close

Summary

Type B insulin resistance syndrome (TBIR) is characterised by the rapid onset of severe insulin resistance due to circulating anti-insulin receptor antibodies (AIRAs). Widespread acanthosis nigricans is normally seen, and co-occurrence with other autoimmune diseases is common. We report a 27-year-old Caucasian man with psoriasis and connective tissue disease who presented with unexplained rapid weight loss, severe acanthosis nigricans, and hyperglycaemia punctuated by fasting hypoglycaemia. Severe insulin resistance was confirmed by hyperinsulinaemic euglycaemic clamping, and immunoprecipitation assay demonstrated AIRAs, confirming TBIR. Treatment with corticosteroids, metformin and hydroxychloroquine allowed withdrawal of insulin therapy, with stabilisation of glycaemia and diminished signs of insulin resistance; however, morning fasting hypoglycaemic episodes persisted. Over three years of follow-up, metabolic control remained satisfactory on a regimen of metformin, hydroxychloroquine and methotrexate; however, psoriatic arthritis developed. This case illustrates TBIR as a rare but severe form of acquired insulin resistance and describes an effective multidisciplinary approach to treatment.

Learning points:

  • We describe an unusual case of type B insulin resistance syndrome (TBIR) in association with mixed connective tissue disease and psoriasis.

  • Clinical evidence of severe insulin resistance was corroborated by euglycaemic hyperinsulinaemic clamp, and anti-insulin receptor autoantibodies were confirmed by immunoprecipitation assay.

  • Treatment with metformin, hydroxychloroquine and methotrexate ameliorated extreme insulin resistance.

Open access
Daramjav Narantsatsral Division of Endocrinology and Metabolism, Department of Internal Medicine

Search for other papers by Daramjav Narantsatsral in
Google Scholar
PubMed
Close
,
Takagi Junko Division of Endocrinology and Metabolism, Department of Internal Medicine

Search for other papers by Takagi Junko in
Google Scholar
PubMed
Close
,
Iwayama Hideyuki Department of Pediatrics, Aichi Medical University School of Medicine, Nagakute, Japan

Search for other papers by Iwayama Hideyuki in
Google Scholar
PubMed
Close
,
Inukai Daisuke Department of Otolaryngology, Aichi Medical University School of Medicine, Nagakute, Japan

Search for other papers by Inukai Daisuke in
Google Scholar
PubMed
Close
,
Takama Hiroyuki Department of Dermatology, Aichi Medical University School of Medicine, Nagakute, Japan

Search for other papers by Takama Hiroyuki in
Google Scholar
PubMed
Close
,
Nomura Yuka Division of Endocrinology and Metabolism, Department of Internal Medicine

Search for other papers by Nomura Yuka in
Google Scholar
PubMed
Close
,
Hirase Syo Division of Endocrinology and Metabolism, Department of Internal Medicine

Search for other papers by Hirase Syo in
Google Scholar
PubMed
Close
,
Morita Hiroyuki Division of Endocrinology and Metabolism, Department of Internal Medicine

Search for other papers by Morita Hiroyuki in
Google Scholar
PubMed
Close
,
Otake Kazuo Division of Endocrinology and Metabolism, Department of Internal Medicine

Search for other papers by Otake Kazuo in
Google Scholar
PubMed
Close
,
Ogawa Tetsuya Department of Otolaryngology, Aichi Medical University School of Medicine, Nagakute, Japan

Search for other papers by Ogawa Tetsuya in
Google Scholar
PubMed
Close
, and
Takami Akiyoshi Department of Hematology, Aichi Medical University School of Medicine, Nagakute, Japan

Search for other papers by Takami Akiyoshi in
Google Scholar
PubMed
Close

Summary

Dupilumab an inhibitor of the interleukin (IL)-4R-alpha subunit is used for the treatment of allergic diseases. The patient was a 49-year-old man who received dupilumab for the treatment of severe atopic dermatitis. He presented hyperthyroidism with elevated thyroglobulin and anti-thyroid antibody negativity at 4 months after the initiation of therapy. On scintigraphy, the thyroid radioiodine uptake was low. Ultrasonography showed a diffuse hypoechoic area in the thyroid gland. A pathological study revealed lymphocytic infiltration. The administration of dupilumab was continued because of his atopic dermatitis that showed an excellent response. The patient`s hyperthyroidism changed to hypothyroidism 3 weeks later. Six months later his thyroid function normalized without any treatment. We herein describe the case of a patient with atopic dermatitis who developed painless thyroiditis under treatment with dupilumab. To the best of our knowledge, this is the first report of this event in the literature.

Learning points:

  • Dupilumab, a fully human monoclonal antibody that blocks interleukin-4 and interleukin-13, has been shown to be effective in the treatment atopic dermatitis and asthma with eosinophilia.

  • Painless thyroiditis is characterized by transient hyperthyroidism and hypothyroidism and recovery without anti-thyroid treatment.

  • This is the first report of painless thyroiditis as an adverse effect of dupilumab, although conjunctivitis and nasopharyngitis are the main adverse effects of dupilumab.

Open access
Sara Lomelino-Pinheiro Endocrinology Department, Instituto Português de Oncologia de Lisboa Francisco Gentil, Lisboa, Portugal

Search for other papers by Sara Lomelino-Pinheiro in
Google Scholar
PubMed
Close
,
Bastos Margarida Endocrinology, Diabetes and Metabolism Department, Centro Hospitalar Universitário de Coimbra, Coimbra, Portugal

Search for other papers by Bastos Margarida in
Google Scholar
PubMed
Close
, and
Adriana de Sousa Lages Endocrinology, Diabetes and Metabolism Department, Centro Hospitalar Universitário de Coimbra, Coimbra, Portugal

Search for other papers by Adriana de Sousa Lages in
Google Scholar
PubMed
Close

Summary

Familial hypomagnesemia with secondary hypocalcemia (FHSH) is a rare autosomal recessive disorder (OMIM# 602014) characterized by profound hypomagnesemia associated with hypocalcemia. It is caused by mutations in the gene encoding transient receptor potential cation channel member 6 (TRPM6). It usually presents with neurological symptoms in the first months of life. We report a case of a neonate presenting with recurrent seizures and severe hypomagnesemia. The genetic testing revealed a novel variant in the TRPM6 gene. The patient has been treated with high-dose magnesium supplementation, remaining asymptomatic and without neurological sequelae until adulthood. Early diagnosis and treatment are important to prevent irreversible neurological damage.

Learning points:

  • Loss-of-function mutations of TRPM6 are associated with FHSH.

  • FHSH should be considered in any child with refractory hypocalcemic seizures, especially in cases with serum magnesium levels as low as 0.2 mM.

  • Normocalcemia and relief of clinical symptoms can be assured by administration of high doses of magnesium.

  • Untreated, the disorder may be fatal or may result in irreversible neurological damage.

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

Search for other papers by Kaja Grønning in
Google Scholar
PubMed
Close
,
Archana Sharma Department of Endocrinology, Akershus University Hospital, Lorenskog, Norway

Search for other papers by Archana Sharma in
Google Scholar
PubMed
Close
,
Maria Adele Mastroianni Department of Haematology, Akershus University Hospital, Lorenskog, Norway

Search for other papers by Maria Adele Mastroianni in
Google Scholar
PubMed
Close
,
Bo Daniel Karlsson Department of Radiology, Akershus University Hospital, Lorenskog, Norway

Search for other papers by Bo Daniel Karlsson in
Google Scholar
PubMed
Close
,
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

Search for other papers by Eystein S Husebye in
Google Scholar
PubMed
Close
,
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

Search for other papers by Kristian Løvås in
Google Scholar
PubMed
Close
, and
Ingrid Nermoen Department of Endocrinology, Akershus University Hospital, Lorenskog, Norway
Institute of Clinical Medicine, Faculty of Medicine, University of Oslo, Oslo, Norway

Search for other papers by Ingrid Nermoen in
Google Scholar
PubMed
Close

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
Yuri Tanaka Division of Neurology, Respirology, Endocrinology and Metabolism, Department of Internal Medicine, Faculty of Medicine, University of Miyazaki, Miyazaki, Japan

Search for other papers by Yuri Tanaka in
Google Scholar
PubMed
Close
,
Taisuke Uchida Division of Neurology, Respirology, Endocrinology and Metabolism, Department of Internal Medicine, Faculty of Medicine, University of Miyazaki, Miyazaki, Japan

Search for other papers by Taisuke Uchida in
Google Scholar
PubMed
Close
,
Hideki Yamaguchi Division of Neurology, Respirology, Endocrinology and Metabolism, Department of Internal Medicine, Faculty of Medicine, University of Miyazaki, Miyazaki, Japan

Search for other papers by Hideki Yamaguchi in
Google Scholar
PubMed
Close
,
Yohei Kudo Division of Neurology, Respirology, Endocrinology and Metabolism, Department of Internal Medicine, Faculty of Medicine, University of Miyazaki, Miyazaki, Japan

Search for other papers by Yohei Kudo in
Google Scholar
PubMed
Close
,
Tadato Yonekawa Division of Neurology, Respirology, Endocrinology and Metabolism, Department of Internal Medicine, Faculty of Medicine, University of Miyazaki, Miyazaki, Japan

Search for other papers by Tadato Yonekawa in
Google Scholar
PubMed
Close
, and
Masamitsu Nakazato Division of Neurology, Respirology, Endocrinology and Metabolism, Department of Internal Medicine, Faculty of Medicine, University of Miyazaki, Miyazaki, Japan

Search for other papers by Masamitsu Nakazato in
Google Scholar
PubMed
Close

Summary

We report the case of a 48-year-old man with thyroid storm associated with fulminant hepatitis and elevated levels of soluble interleukin-2 receptor (sIL-2R). Fatigue, low-grade fever, shortness of breath, and weight loss developed over several months. The patient was admitted to the hospital because of tachycardia-induced heart failure and liver dysfunction. Graves’ disease with heart failure was diagnosed. He was treated with methimazole, inorganic iodide, and a β-blocker. On the day after admission, he became unconscious with a high fever and was transferred to the intensive care unit. Cardiogenic shock with atrial flutter was treated with intra-aortic balloon pumping and cardioversion. Hyperthyroidism decreased over 10 days, but hepatic failure developed. He was diagnosed with thyroid storm accompanied by fulminant hepatitis. Laboratory investigations revealed elevated levels of sIL-2R (9770 U/mL). The fulminant hepatitis was refractory to plasma exchange and plasma filtration with dialysis, and no donors for liver transplantation were available. He died of hemoperitoneum and gastrointestinal hemorrhage due to fulminant hepatitis 62 days after admission. Elevated circulating levels of sIL-2R might be a marker of poor prognosis in thyroid storm with fulminant hepatitis.

Learning points:

  • The prognosis of thyroid storm when fulminant hepatitis occurs is poor.

  • Liver transplantation is the preferred treatment for fulminant hepatitis induced by thyroid storm refractory to plasma exchange.

  • Elevated levels of soluble interleukin-2 receptor might be a marker of poor prognosis in patients with thyroid storm.

Open access
Mohammed Faraz Rafey Galway University Hospitals, Galway, Ireland
HRB Clinical Research Facility, National University of Ireland Galway, Galway, Ireland

Search for other papers by Mohammed Faraz Rafey in
Google Scholar
PubMed
Close
,
Arslan Butt Galway University Hospitals, Galway, Ireland

Search for other papers by Arslan Butt in
Google Scholar
PubMed
Close
,
Barry Coffey Galway University Hospitals, Galway, Ireland

Search for other papers by Barry Coffey in
Google Scholar
PubMed
Close
,
Lisa Reddington Galway University Hospitals, Galway, Ireland

Search for other papers by Lisa Reddington in
Google Scholar
PubMed
Close
,
Aiden Devitt Galway University Hospitals, Galway, Ireland

Search for other papers by Aiden Devitt in
Google Scholar
PubMed
Close
,
David Lappin Galway University Hospitals, Galway, Ireland

Search for other papers by David Lappin in
Google Scholar
PubMed
Close
, and
Francis M Finucane Galway University Hospitals, Galway, Ireland
HRB Clinical Research Facility, National University of Ireland Galway, Galway, Ireland

Search for other papers by Francis M Finucane in
Google Scholar
PubMed
Close

Summary

We describe two cases of SGLT2i-induced euglycaemic diabetic ketoacidosis, which took longer than we anticipated to treat despite initiation of our DKA protocol. Both patients had an unequivocal diagnosis of type 2 diabetes, had poor glycaemic control with a history of metformin intolerance and presented with relatively vague symptoms post-operatively. Neither patient had stopped their SGLT2i pre-operatively, but ought to have by current treatment guidelines.

Learning points:

  • SGLT2i-induced EDKA is a more protracted and prolonged metabolic derangement and takes approximately twice as long to treat as hyperglycaemic ketoacidosis.

  • Surgical patients ought to stop SGLT2i medications routinely pre-operatively and only resume them after they have made a full recovery from the operation.

  • While the mechanistic basis for EDKA remains unclear, our observation of marked ketonuria in both patients suggests that impaired ketone excretion may not be the predominant metabolic lesion in every case.

  • Measurement of insulin, C-Peptide, blood and urine ketones as well as glucagon and renal function at the time of initial presentation with EDKA may help to establish why this problem occurs in specific patients.

Open access