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Diagnosis and Treatment > Medication

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Huilin Koh Department of Endocrinology, Singapore General Hospital, Singapore, Singapore

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Manish Kaushik Department of Renal Medicine, Singapore General Hospital, Singapore, Singapore

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Julian Kenrick Loh Department of Cardiology, National Heart Centre Singapore, Singapore, Singapore

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Chiaw Ling Chng Department of Endocrinology, Singapore General Hospital, Singapore, Singapore

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Summary

Thyroid storm with multi-organ failure limits the use of conventional treatment. A 44-year-old male presented with thyroid storm and experienced cardiovascular collapse after beta-blocker administration, with resultant fulminant multi-organ failure requiring inotropic support, mechanical ventilation, extracorporeal membrane oxygenation (ECMO) and continuous renal replacement therapy. Hepatic and renal failure precluded the use of conventional thyroid storm treatment and early plasma exchange was instituted. The patient underwent emergency thyroidectomy after four effective exchanges, with subsequent rapid reversal of multi-organ failure. The challenges of institution of plasma exchanges with ongoing ECMO support, dialysis and timing of thyroidectomy are discussed. This case highlights the important role of early therapeutic plasma exchange (TPE) as an effective salvage therapy for lowering circulating hormones and stabilization of patients in preparation for emergency thyroidectomy in patients with thyroid storm and fulminant multi-organ failure.

Learning points:

  • Administration of beta-blockers in thyroid storm presenting with congestive cardiac failure may precipitate cardiovascular collapse due to inhibition of thyroid-induced hyperadrenergic compensation which maintains cardiac output.

  • TPE can be an effective bridging therapy to emergency total thyroidectomy when conventional thyroid storm treatment is contraindicated.

  • End-organ support using ECMO and CRRT can be combined with TPE effectively in the management of critically ill cases of thyroid storm.

  • The effectiveness of plasma exchange in lowering thyroid hormones appears to wane after 44–48 h of therapy in this case, highlighting the importance early thyroidectomy.

Taxonomy:
Clinical Overview, Gland/Organ, Thyroid, Topic, Thyroid, Hormone, Thyroxine (T4), Triiodothyronine (T3), TSH, Condition/ Syndrome, Hyperthyroidism, Liver failure, Thyroid storm, Patient Demographics, Age, Adult, Gender, Male, Ethnicity, Asian - Chinese, Country of Treatment, Singapore, Diagnosis and Treatment, Signs and Symptoms, Atrial fibrillation, Breathing difficulties, Coagulopathy, Coughing, Diarrhoea, Heart failure, Hyperthyroidism, Hypotension, Hypoxia, Pyrexia, Renal failure, Sore throat, Thyrotoxicosis, Vomiting, Investigation, Alkaline phosphatase, Ammonia, Blood pressure, Brain natriuretic peptide, Creatinine (serum), Electrocardiogram, FT3, FT4, Haemoglobin, Heart rate, Lactate, Platelet count, Procalcitonin, Thyroid antibodies, Troponin, TSH, TSH receptor antibodies, Urea and electrolytes, White blood cell count, X-ray, Intervention, Dialysis, Plasma exchange, Plasmapheresis, Thyroidectomy, Medication, Albumin, Beta-blockers, Carbimazole, Digoxin, Epinephrine, Glucocorticoids, Heparin, Hydrocortisone, Levothyroxine, Norepinephrine, Propranolol, Related Disciplines, Cardiology, Nephrology, Publication Details, Case Report Type, Novel treatment
DOI:
https://doi.org/10.1530/EDM-19-0051
Volume/Issue:
Volume 2019: Issue 1
Open access
Jin-Ying Lu Division of Endocrinology and Metabolism, Department of Internal Medicine, National Taiwan University Hospital, Taipei, 100, Taiwan

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Po-Ju Hung Division of Endocrinology and Metabolism, Department of Internal Medicine, National Taiwan University Hospital, Taipei, 100, Taiwan

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Pei-Lung Chen Division of Endocrinology and Metabolism, Department of Internal Medicine, National Taiwan University Hospital, Taipei, 100, Taiwan
Department of Medical Genetics, National Taiwan University Hospital, Taipei, 100, Taiwan
Graduate Institute of Medical Genomics and Proteomics, National Taiwan University, Taipei, 100, Taiwan
Graduate Institute of Clinical Medicine, National Taiwan University, Taipei, 100, Taiwan
Research Center for Developmental Biology and Regenerative Medicine, National Taiwan University, Taipei, 100, Taiwan

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Ruoh-Fang Yen Department of Nuclear Medicine, National Taiwan University, Taipei, 100, Taiwan

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Kuan-Ting Kuo Graduate Institute of Pathology, College of Medicine, National Taiwan University, Taipei, 100, Taiwan
Department of Pathology, National Taiwan University Hospital, Taipei, 100, Taiwan

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Tsung-Lin Yang Department of Otolaryngology, National Taiwan University Hospital, Taipei, 100, Taiwan

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Chih-Yuan Wang Division of Endocrinology and Metabolism, Department of Internal Medicine, National Taiwan University Hospital, Taipei, 100, Taiwan

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Tien-Chun Chang Division of Endocrinology and Metabolism, Department of Internal Medicine, National Taiwan University Hospital, Taipei, 100, Taiwan
Department of Medicine, College of Medicine, National Taiwan University, Taipei, 100, Taiwan

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Tien-Shang Huang Division of Endocrinology and Metabolism, Department of Internal Medicine, National Taiwan University Hospital, Taipei, 100, Taiwan
Department of Medicine, College of Medicine, National Taiwan University, Taipei, 100, Taiwan
Department of Social Medicine, College of Medicine, National Taiwan University, Taipei, 100, Taiwan
Department of Medicine, Cathay General Hospital, Taipei, 106, Taiwan

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Ching-Chung Chang Division of Endocrinology and Metabolism, Department of Internal Medicine, National Taiwan University Hospital, Taipei, 100, Taiwan
Department of Medicine, College of Medicine, National Taiwan University, Taipei, 100, Taiwan
Department of Internal Medicine, China Medical University Hospital, Taichung, 404, Taiwan
Department of Internal Medicine, China Medical University, Taichung, 404, Taiwan

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Summary

We report a case of follicular thyroid carcinoma with concomitant NRAS p.Q61K and GNAS p.R201H mutations, which manifested as a 13.5 cm thyroid mass with lung, humerus and T9 spine metastases, and exhibited good response to radioactive iodine treatment.

Learning points

  • GNAS p.R201H somatic mutation is an activating or gain-of-function mutation resulting in constitutively activated Gs-alpha protein and downstream cAMP cascade, independent of TSH signaling, causing autonomously functioning thyroid nodules.

  • NRAS p.Q61K mutations with GNAS p.R201H mutations are known for a good radioactive iodine treatment response.

  • Further exploration of the GNAS-activating pathway may provide therapeutic insights into the treatment of metastatic follicular carcinoma.

Taxonomy:
Clinical Overview, Gland/Organ, Thyroid, Topic, Thyroid, Hormone, Thyroxine (T4), TSH, Condition/ Syndrome, Follicular thyroid cancer, Graves' disease, Metastatic carcinoma, Patient Demographics, Age, Adult, Gender, Male, Ethnicity, Asian - Chinese, Country of Treatment, Taiwan, Province of China, Diagnosis and Treatment, Signs and Symptoms, Dysphonia, Goitre, Hepatic cysts, Vocal cord paresis, Investigation, CT scan, DNA sequencing, Endoscopy, Fine needle aspiration biopsy, FT4, Genetic analysis, MRI, Radionuclide imaging, SPECT scan, Thyroglobulin, TSH, TSH receptor antibodies, Ultrasound scan, Intervention, Diet, Lymph node dissection, Radionuclide therapy, Thyroidectomy, Medication, Albumin, Levothyroxine, Thyrotropin alpha, Related Disciplines, Otolaryngology, Publication Details, Case Report Type, Insight into disease pathogenesis or mechanism of therapy
DOI:
https://doi.org/10.1530/EDM-15-0067
Volume/Issue:
Volume 2016: Issue 1
Open access