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Diagnosis and Treatment > Signs and Symptoms

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Tzy Harn Chua Department of Endocrinology, Changi General Hospital, Singapore

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Wann Jia Loh Department of Endocrinology, Changi General Hospital, Singapore

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Summary

Severe hyponatremia and osmotic demyelination syndrome (ODS) are opposite ends of a spectrum of emergency disorders related to sodium concentrations. Management of severe hyponatremia is challenging because of the difficulty in balancing the risk of overcorrection leading to ODS as well as under-correction causing cerebral oedema, particularly in a patient with chronic hypocortisolism and hypothyroidism. We report a case of a patient with Noonan syndrome and untreated anterior hypopituitarism who presented with symptomatic hyponatremia and developed transient ODS.

Learning points:

  • Patients with severe anterior hypopituitarism with severe hyponatremia are susceptible to the rapid rise of sodium level with a small amount of fluid and hydrocortisone.

  • These patients with chronic anterior hypopituitarism are at high risk of developing ODS and therefore, care should be taken to avoid a rise of more than 4–6 mmol/L per day.

  • Early recognition and rescue desmopressin and i.v. dextrose 5% fluids to reduce serum sodium concentration may be helpful in treating acute ODS.

Taxonomy:
Clinical Overview, Gland/Organ, Pituitary, Topic, Pituitary, Hormone, ACTH, Cortisol, FSH, GH, IGF1, LH, Testosterone, Thyroxine (T4), Triiodothyronine (T3), TSH, Condition/ Syndrome, Hyponatraemia, Hypopituitarism, Microadenoma, Noonan syndrome, Pituitary adenoma, Patient Demographics, Age, Adult, Gender, Male, Ethnicity, Asian - other, Country of Treatment, Singapore, Diagnosis and Treatment, Signs and Symptoms, Abdominal pain, Aphasia, Dizziness, Ears - low set, Gynaecomastia, Headache, Hypogonadism, Hyponatraemia, Hypopituitarism, Hypotension, Hypothyroidism, Hypotonia, Insomnia, Kyphoscoliosis, Nausea, Neck - loose skin (nape), Neck - short, Osteoporosis, Seizures, Short stature, Vomiting, Investigation, 25-hydroxyvitamin-D3, ACTH, Blood pressure, Bone mineral density, Chloride, Cortisol, Cortisol (9am), Cortisol (serum), FSH, FT3, FT4, GH, Haemoglobin, IGF1, LH, MRI, Serum osmolality, Sex hormone binding globulin, Sodium, Testosterone, TSH, Urine osmolality, X-ray, Intervention, Fluid repletion, Medication, Desmopressin, Glucocorticoids, Glucose, Hydrocortisone, Levothyroxine, Saline, Valproic acid, Related Disciplines, Neurology, Publication Details, Case Report Type, Unique/unexpected symptoms or presentations of a disease
DOI:
https://doi.org/10.1530/EDM-20-0039
Volume/Issue:
Volume 2020: Issue 1
Open access
Hui Yi Ng Department of Clinical Medicine, Level 4, Macquarie University, 2 Technology Place, Macquarie University, New South Wales, Australia

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Divya Namboodiri Department of Clinical Medicine, Level 4, Macquarie University, 2 Technology Place, Macquarie University, New South Wales, Australia

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Diana Learoyd University of Sydney, Faculty of Medicine and Health, Northern Clinical School, Reserve Road St Leonards, New South Wales, Australia

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Andrew Davidson Department of Neurosurgery, Level 2, Macquarie University, 2 Technology Place Macquarie University, New South Wales, Australia

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Bernard Champion Department of Clinical Medicine, Level 4, Macquarie University, 2 Technology Place, Macquarie University, New South Wales, Australia

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Veronica Preda Department of Clinical Medicine, Level 4, Macquarie University, 2 Technology Place, Macquarie University, New South Wales, Australia

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Summary

Co-secreting thyrotropin/growth hormone (GH) pituitary adenomas are rare; their clinical presentation and long-term management are challenging. There is also a paucity of long-term data. Due to the cell of origin, these can behave as aggressive tumours. We report a case of a pituitary plurihormonal pit-1-derived macroadenoma, with overt clinical hyperthyroidism and minimal GH excess symptoms. The diagnosis was confirmed by pathology showing elevated thyroid and GH axes with failure of physiological GH suppression, elevated pituitary glycoprotein hormone alpha subunit (αGSU) and macroadenoma on imaging. Pre-operatively the patient was rendered euthyroid with carbimazole and underwent successful transphenoidal adenomectomy (TSA) with surgical cure. Histopathology displayed an elevated Ki-67 of 5.2%, necessitating long-term follow-up.

Learning points:

  • Thyrotropinomas are rare and likely under-diagnosed due to under-recognition of secondary hyperthyroidism.

  • Thyrotropinomas and other plurihormonal pit-1-derived adenomas are more aggressive adenomas according to WHO guidelines.

  • Co-secretion occurs in 30% of thyrotropinomas, requiring diligent investigation and long-term follow-up of complications.

Taxonomy:
Clinical Overview, Gland/Organ, Pituitary, Topic, Pituitary, Hormone, GH, IGF1, Prolactin, Thyroxine (T4), Triiodothyronine (T3), TSH, Condition/ Syndrome, Acromegaly, Goitre (multinodular), Hyperthyroidism, Hypogonadism, Pituitary adenoma, Plurihormonal pituitary adenoma, Thyrotrophic adenoma, Patient Demographics, Age, Adult, Gender, Male, Ethnicity, White, Country of Treatment, Australia, Diagnosis and Treatment, Signs and Symptoms, Anxiety, Dizziness, Goitre, Headache, Heat intolerance, Hypertension, Hyperthyroidism, Hypogonadism, Insomnia, Osteopenia, Palpitations, Tachycardia, Ventricular hypertrophy, Investigation, Angiography, Blood pressure, BMI, Bone mineral density, DEXA scan, Echocardiogram, FT3, FT4, GH, GH suppression, Haematoxylin and eosin staining, Heart rate, Histopathology, IGF1, Immunostaining, MRI, Pituitary function, Sex hormone binding globulin, SHBG, Thyroid function, Thyroid ultrasonography, TRH stimulation, Triiodothyronine (T3) suppression, TSH, Intervention, Resection of tumour, Transsphenoidal surgery, Medication, Aspirin, Beta-blockers, Carbimazole, Metoprolol, Statins, Related Disciplines, Surgery, Publication Details, Case Report Type, Unique/unexpected symptoms or presentations of a disease
DOI:
https://doi.org/10.1530/EDM-19-0068
Volume/Issue:
Volume 2019: Issue 1
Open access