Clinical presentation and magnetic resonance imaging characteristics of lymphocytic hypophysitis: a systematic review with meta-analysis

This meta-analysis was to analyze the clinical presentation, magnetic resonance imaging (MRI) characteristics, and the management of Lymphocytic hypophysitis (LYH). Meta-analysis showed that the percentage of women in LYH patients was 78%. LYH was associated with pregnancy in 15% of the female patients, with headache (49%) and symptoms of central diabetes insipidus (CDI) (45%) being the most frequent presentation. In 24 % of the LYH patients, there was an association with another autoimmune disease. The incidence of secondary hypogonadism, secondary hypoadrenalism, secondary hypothyroidism, and growth hormone deficit was 54%, 49%, 43%, and 22%, respectively. Pituitary contrast enhancement (63%), symmetrical pituitary enlargement (60%), thickening of the pituitary stalk (58%), sella mass or suprasellar extension (58%), and loss of posterior pituitary hyperintensity (50%) were typical MRI findings. Regarding LYH treatment, the percentage of patients who had observation or hormone replacement, steroid therapy, and surgery was 43%, 36%, and 34%, respectively.

A Nonfunctioning Pituitary Macroadenoma Presenting as Cyclical Vomiting and Severe Hyponatremia in a Young Female

Introduction. Recurrent vomiting is a commonly overlooked debilitating symptom which causes significant impact on the quality of life. There are several causes for vomiting, ranging from commonly known causes to rare causes. Nonfunctioning pituitary macroadenomas generally present with visual disturbances, headache, and symptoms due to anterior pituitary hormone deficiencies. This case report is about an atypical presentation of a nonfunctioning pituitary macroadenoma in which the patient presented with cyclical vomiting with severe hyponatremia. Case Report. A 23-year-old girl presented with four to five episodes of vomiting per day for two days duration. She had a history of similar episodes of vomiting since 2016, with each episode generally lasting for 4-5 days and occurring in every four to six months. All episodes exhibited similar symptomatology and she was free of symptoms in-between. Generalized body weakness, postural dizziness, reduced appetite, and secondary amenorrhea were other symptoms she has had since 2016. Examination findings showed a low body mass index (BMI) (16 kg/m2) with normal system examination. Investigations showed severe hyponatremia (110 mmol/L) with hypokalemia (3.2 mmol/L) and hypochloremia (74 mmol/L). Her urinary excretion of potassium, sodium, and serum osmolality was low. Urine osmolality was mildly elevated compared to serum osmolality. Blood urea was normal. Severe hyponatremia with minimal hyponatremic symptoms was suggestive of chronic hyponatremia, which was accentuated by ongoing vomiting and possible reduced intake of salt. Further investigations showed evidence of secondary hypoadrenalism, central hypothyroidism, hypogonadotropic hypogonadism, and mild hyperprolactinemia. Magnetic resonance imaging (MRI) revealed a pituitary macroadenoma with mass effect on the optic chiasma. Hydrocortisone and levothyroxine were started, and she underwent transsphenoidal resection of the pituitary tumor. She recovered from cyclical vomiting. Conclusion. There can be multiple overlapping aetiologies for every observed symptom, sign, and abnormal investigation finding. Therefore, aetiological diagnosis is challenging, especially in the presence of an atypical clinical presentation. Cyclical vomiting and severe hyponatremia are atypical presentations of nonfunctioning pituitary macroadenomas.

Opioid-induced hypoadrenalism resulting in fasting hypoglycaemia

An 18-year-old woman with a history of hollow visceral myopathy presented with a small-bowel obstruction. High-dose opioid analgesia was required subsequently during hospital admission. She suffered two episodes of documented fasting hypoglycaemia, despite adjustment of parenteral carbohydrate administration. Investigations for non-insulin-mediated hypoglycaemia revealed a low morning cortisol of 109 nmol/L and an inappropriately low Adrenocorticotropic hormone (ACTH) level of 2.2 pmol/L. A diagnosis of secondary adrenal insufficiency was confirmed on repeat cortisol and ACTH testing. The 250 µg short Synacthen test cortisol response was normal, suggestive of acute rather than chronic ACTH deficiency. This pattern was consistent after further opioid exposure. Adrenal recovery occurred shortly after opioid cessation. Opioid-induced hypoadrenalism is likely an under-recognised clinical entity with potentially serious adverse patient outcomes. There are reported cases involving commonly prescribed opioids including fentanyl and tramadol. However, we believe this is the first reported clinical case of acute transient opioid-induced secondary hypoadrenalism associated with fasting hypoglycaemia.

Endocrine disorders with craniopharyngioma in adults before and after surgery

Background. Craniopharyngiomas (CF) - a benign tumor of the embryologic origin. The surgical method of treatment is basic.Aim: to estimate dynamics of endocrine disorders before and after surgical treatment of CF at different topographical variations.Methods.The research involved 42 patients older than 18 years (25 women and 17 men) with a mean age - 41 [21; 69] with a verified diagnosis of CF. All patients were operated in 28 cases – total resection, in 7 - the pituitary stalk was saved. Before and 6 months after the surgery all patients were examined by means of hormonal blood tests (TSH, free T4, cortisol, prolactin, LH , FSH, estradiol, testosterone, IGF-1). By tumor localizing patients were divided into 4 groups: 1 – intra-suprasellar (2) 2 – at location of the pituitary stalk (15), 3 – combined «the pituitary stalk» and ventricular (10), 4 –intra-ventricular (15).Results. In group 1: panhypopituitarism -2 (100%), diabetes insipidus (DI) – 0. Both patients had subtotal ablation. After the surgery the nature of disturbance has not changed. In group 2: secondary hypoadrenalism - 9 (60%), hypothyroidism - 11 (73%), hypogonadism - 12 (80%), DI - 7 (46%), hyperprolactinemia - 9 (60%). After the surgery panhypopituitarism - 15 (100%), DI - 14 (93%), hyperprolactinemia - 4 (26 %). In group 3: secondary hypoadrenalism - 3 (30%), hypothyroidism - 6 (60%), hypogonadism - 6 (60%), DI - 2 (20%), hyperprolactinemia - 3 (30%). After the surgery panhypopituitarism - 10 (100%), DI - 10 (100%), hyperprolactinemia - 3 (30 %). In group 4: secondary hypoadrenalism - 6 (40%), hypothyroidism - 9 (60%), hypogonadism - 12 (80%), DI - 5 (33%), hyperprolactinemia - 8 (53%). After the surgery panhypopituitarism - 10 (71%), secondary hypoadrenalism - 12 (85%), hypothyroidism - 13 (92%), hypogonadism - 11 (79%), DI - 11 (78%), hyperprolactinemia - 8 (53%).Conclusion. The high incidence of endocrine disorders is caused by the localization of the CF with predominance of secondary hypogonadism and hypothyroidism. After the surgery worsening hormone deficiency was mentioned, also while preserving the pituitary stalk. Non-radical ablation of ventricular CF can partially maintain endocrine function.

Symptomatic empty sella syndrome: an unusual manifestation of Erdheim–Chester disease

Summary Erdheim–Chester disease (ECD) is a potentially fatal condition characterized by infiltration of multiple organs by non-Langerhans histiocytes. Although endocrine dysfunction has been reported in association with ECD, to date, there have been no previous reports of empty sella syndrome (ESS) associated with it. We report the case of a patient with ECD who had symptomatic ESS. A 55-year-old man of Chinese ethnicity initially presented with symptoms of heart failure, fatigue and knee joint pain. Physical examination revealed xanthelasma, gynaecomastia, lung crepitations, hepatomegaly and diminished testicular volumes. He had laboratory evidence of hypogonadotrophic hypogonadism, secondary hypoadrenalism and GH deficiency. Imaging studies showed diffuse osteosclerosis of the long bones on X-ray, a mass in the right atrium and thickening of the pleura and of the thoracic aorta on fusion positron emission tomography–computed tomography. Magnetic resonance imaging (MRI) of the brain showed an empty sella. The diagnosis of ECD was confirmed by bone biopsy. Learning points ECD is a multisystemic disease that can affect the pituitary and other organs. The diagnosis of ECD is based on clinical and radiological features and histology, showing lipid-laden CD68+ CD1a− S100− histiocytes surrounded by fibrosis. The finding of xanthelasmas especially in the presence of normal lipid levels in the presence of a multisystem infiltrative disorder should raise the suspicion of ECD. Systemic perturbation of autoimmunity may play a role in the pathogenesis of ECD and is an area that merits further research.

Addisonian Crisis Precipitated By Thyroxine Therapy In A Patient With Type 2 Autoimmune Polyglandular Syndrome

Primary hypoadrenalism(Addison’s disease) refers to glucocorticoid deficiency occurring in the setting of adre­nal disease(mostly due to autoimmune adrenalitis), whereas secondary hypoadrenalism arises because of deficiency of ACTH(mostly due to pituitary disease) . A major distinction between these two is that mineralocorticoid deficiency invari­ably accompanies primary hypoadrenalism, but this does not occur in secondary hypoadrenalism because only ACTH is de­ficient; the renin-angiotensin-aldosterone axis is intact and in primary hypoadrenalism skin pigmentation is always present due to increased ACTH secretion(unless of short duration) but it is absent in secondary hypoadrenalism. Addison’s disease or primary adrenocortical failure was first described by English physician Thomas Addison, who found it in six patients with adrenal tuberculosis in 1855(1). Addisonian crisis is a potentially fatal condition associated mainly with an acute defi­ciency of the glucocorticoid cortisol and, to a lesser extent, the mineralocorticoid aldosterone. This is a rare condition with an estimated incidence in the developed world of 0.8 cases per 100,000 and prevalence of 4 to 11 cases/100,000 population. Despite optimised life-saving glucocorticoid replacement and mineralocorticoid-replacement therapy, it is life threatening if overlooked(2-3). Hypothyroidism may mask the addison’s disease so in patients with panhypopituitarism and autoim­mune polyglandular syndrome type 2 thyroxine replacement without adequate steroid replacement may precipitate acute addisonian crisis. Journal of Chitwan Medical College 2013; 3(4); 54-56 DOI: http://dx.doi.org/10.3126/jcmc.v3i4.9557

A Patient with Postpartum Hypopituitarism (Sheehan's Syndrome) Developed Postpartum Autoimmune Thyroiditis (Transient Thyrotoxicosis and Hypothyroidism): A Case Report and Review of the Literature

A 36-year-old woman with postpartum hypopituitarism (Sheehan's syndrome: SS) developed postpartum autoimmune thyroiditis (PPAT). She delivered a baby by Caesarean section (620 mL blood loss). At 1 month post partum, she developed thyrotoxicosis due to painless thyroiditis (autoimmune destructive thyroiditis). She was positive for antithyroid antibodies. Postpartum and hypoadrenalism-induced exacerbation of autoimmune thyroiditis caused the thyrotoxicosis due to autoimmune destructive thyroiditis. ACTH was undetectable. She had ACTH deficiency and secondary hypoadrenalism. Hydrocortisone was started. At 6 months post partum, she was referred to us with hypothyroidism. Thyroxine was administered. She had thyrotoxicosis at 1-2 months post partum and then hypothyroidism. She was diagnosed with PPAT. She had hypopituitarism, ACTH deficiency (secondary hypoadrenalism), low prolactin with agalactia, and low LH with failure to resume regular menses. She had empty sella on MRI. She was diagnosed with SS. Three cases with SS have been reported to develop PPAT. Postpartum immunological rebounds and hypoadrenalism-induced immunological alterations (or a combination of the two) might have been responsible for the PPAT.