Management of fetal hyperthyroidism caused by persistent autoimmune antibodies in a case of previously treated maternal Graves' disease.

Maternal Graves' autoantibodies are well known to cause fetal and neonatal thyroid disturbances. Despite radioiodine therapy, Graves' autoantibodies are known to persist, which can cross the placenta and cause hyperthyroidism in the fetus. We present the case of a 26-year-old woman in her first pregnancy, clinically and biochemically euthyroid with history of treated Graves' disease, where the fetus showed signs of hyperthyroidism on antenatal scans. This was confirmed by amniotic fluid testing as fetal blood sampling was not feasible and successfully treated with maternal carbimazole whilst continuing thyroxine for the mother (block-replacement). We discuss the challenges in the diagnosis of fetal hyperthyroidism and treatment whilst maternal thyroid status is maintained on thyroxine.

Primary Adrenal Lymphoma as a Rare Cause of Primary Adrenal Insufficiency: Challenges in Management and a Review of the Literature.

Background/Objective: Primary adrenal lymphoma (PAL) is an aggressive form of lymphoma associated with adrenal insufficiency (AI) in most cases. It requires a histologic confirmation unlike other cases of primary AI. Case Report: We report a case of a 66-year-old man who presented with AI with symptomatic hypotension and hypo-osmolar hyponatremia. Ultrasound and computed tomography scans revealed bilateral bulky adrenal masses that were avid on fluorodeoxyglucose positron emission tomography scan. The diagnosis of PAL was confirmed with adrenal biopsy. He was treated with rituximab-based chemotherapy, which was complicated by several endocrine challenges, including worsening diabetes, multiple adrenal crises, prolonged hyponatremia, and refractory hypokalemia requiring spironolactone. He eventually developed central nervous system disease and was treated with palliative intent. Discussion: AI in the setting of PAL can constitute both diagnostic and therapeutic challenges, including significant electrolyte imbalances as discussed in this case report. Conclusion: It is important to have a high suspicion for PAL, especially in the presence of bilateral adrenal masses and AI. Early adrenal biopsy is required for diagnosis. Multidisciplinary care is vital to manage complications that arise during the disease course and treatment.

A Case of Burnt-Out Langerhans Cell Histiocytosis Presenting as Postpartum Hypopituitarism.

OBJECTIVE: To evaluate the case of a woman who presented with central hypogonadism and diabetes insipidus and further developed a persistent cough leading to an unexpected diagnosis of burnt-out Langerhans cell histiocytosis (LCH). METHODS: Clinical and laboratory endocrine evaluation, magnetic resonance imaging, high-resolution computed tomography, and open-lung biopsy results are discussed. RESULTS: A 28-year-old woman presented at 10 months postpartum with polydipsia, polyuria, and amenorrhea for 3 months. Her results showed a prolactin level of 25 µg/L (reference,<23.5 µg/L), estrogen level of 91 pmol/L (reference, 110-180 pmol/L), follicle-stimulating hormone level of 6 IU/L (reference, 2-20 IU/L), and luteinizing hormone level of 6 IU/L (reference, 2-70 IU/L). A water-deprivation test found a sodium concentration of 148 mmol/L (reference, 135-145 mmol/L), serum osmolality of 310 mmol/kg (reference, 275-295 mmol/kg), and urine osmolality of 107 mmol/kg (reference, 50-1450 mmol/kg) that improved to 142 mEq/L, 295 mmol/kg, and 535 mmol/kg, respectively, after desmopressin administration. Gadolinium-enhanced pituitary magnetic resonance imaging demonstrated a markedly thickened stalk with uniform enhancement. Chest high-resolution computed tomography confirmed bilateral upper-zone cystic lung disease suggestive of either pulmonary lymphangioleiomyomatosis or LCH. Eventual histology showed CD1a-positive burnt-out LCH. This differentiation was crucial as pulmonary lymphangioleiomyomatosis exacerbates with estrogen therapy and pregnancy, which the patient was able to successfully pursue without disease exacerbation. CONCLUSION: The patient's initial presentation was considered as lymphocytic hypophysitis, but subsequent cystic changes on high-resolution computed tomography led to a unifying definitive diagnosis of burnt-out LCH. This case highlights the importance of investigating for uncommon secondary causes of hypophysitis.

Bilateral adrenal gland haemorrhage: an unusual cause.

UNLABELLED: Our patient had drainage of a large amoebic liver abscess. This got complicated by a severe degree of hypotension, which required aggressive fluid resuscitation and hydrocortisone support. Computerised tomography (CT) of the abdomen revealed bilateral adrenal gland haemorrhage (BAH) resulting in primary adrenal gland failure, which was the cause for hypotension. Patient was on long-term warfarin for provoked deep vein thrombosis of lower limb, which was discontinued before the procedure. Thrombophilia profile indicated the presence of lupus anticoagulant factor with prolonged activated partial thromboplastin time (aPTT). Patient was discharged on lifelong warfarin. This case emphasises the need for strong clinical suspicion for diagnosing BAH, rare but life-threatening condition, and its association with amoebic liver abscess and anti-phospholipid antibody syndrome (APLS). LEARNING POINTS: Recognition of BAH as a rare complication of sepsis.APLS can rarely cause BAH.

Ipilimumab-induced autoimmune hypophysitis: a differential for sellar mass lesions.

UNLABELLED: Autoimmune hypophysitis (AH) has been previously described in a typical demographic population, primarily women in the reproductive age group and perinatal period. The era of immune modulation using anti-cytotoxic T-lymphocyte-associated antigen 4 biological therapy (ipilimumab) against advanced cancers like metastatic melanomas has now resulted in a new form of hypophysitis being increasingly recognised under a spectrum of immune-related adverse events. Drug-related AH often presents with subtle symptoms and a pituitary mass, with the potential for fatality necessitating wide awareness and a high index of clinical suspicion given that it is usually treatable. We describe below two cases of AH within the last three months at our centre, which were treated with different regimens and produced good endocrine outcomes. LEARNING POINTS: AH is a new and defined clinical entity occurring as a side effect of ipilimumab, which enhances immune-mediated destruction of metastatic melanoma.It can present insidiously and have life-threatening complications related to hypocortisolism, hence a high index of clinical suspicion must be exerted by treating physicians, and seems to result in resolution of pituitary masses and variable improvements of pituitary function.Clinical improvement, radiological resolution of pituitary masses and variable normalisation of pituitary function are possible with early treatment with high-dose oral or i.v. steroids and hormone replacement therapy, although duration and dosing protocols are unclear at this stage.Ipilimumab should continue to be prescribed as treatment for metastatic melanoma; however, close clinical observation of patient's progress must be maintained while they are on this drug.Predictive factors for onset of AH remain unclear and it is imperative that AH is distinguished from pituitary metastases.Further studies are required to determine the safety of continuing therapy with ipilimumab in patients who have developed AH while on treatment.

Extreme hypertriglyceridemia managed with insulin.

Extreme hypertriglyceridemia can lead to acute pancreatitis and rapid lowering of serum triglycerides (TG) is necessary for preventing such life-threatening complications. However, there is no established consensus on the acute management of extreme hypertriglyceridemia. We retrospectively reviewed 10 cases of extreme hypertriglyceridemia with mean serum TG on presentation of 101.5 ± 23.4 mmol/L (8982 ± 2070 mg/dL) managed with insulin. Serum TG decreased by 87 ± 4% in 24 hours in those patients managed with intravenous insulin and fasting and 40 ± 8.4% in those managed with intravenous insulin alone (P = .0003). The clinical course was uncomplicated in all except 1 patient who subsequently developed a pancreatic pseudocyst. Thus, combination of intravenous insulin with fasting appears to be an effective, simple, and safe treatment strategy in immediate management of extreme hypertriglyceridemia.

Neonatal hypothyroidism despite maternal tri-iodothyronine toxicosis: a management problem?

A primigravida was referred with hyperthyroidism in early pregnancy from longstanding Graves' disease treated with propylthiouracil. She had selective elevation of free tri-iodothyronine (fT3) levels, low normal free tetra-iodothyronine (fT4) and suppressed thyroid-stimulating hormone (TSH). Given her symptoms of thyrotoxicosis and elevated TSH receptor antibodies, therapy was tailored towards maintaining clinical and biochemical euthyroidism. However the fetus developed a goitre secondary to hypothyroidism. This case highlights the dilemmas in managing maternal T3 toxicosis while aiming for a high normal fT4 to prevent fetal hypothyroidism including the role of fetal ultrasound monitoring and amniocentesis.