Resection of an elusive parathyroid adenoma in the aortopulmonary window.

Primary hyperparathyroidism (PHPT) is the most common cause of hypercalcaemia and is caused by a single parathyroid adenoma in the neck in 85% or more of patients. National Institute for Health and Care Excellence (NICE) guidelines published in May 2019 advise that preoperative imaging is offered prior to parathyroid surgery for PHPT if it will inform surgical approach. If imaging, typically a neck ultrasound and a second modality that may use ionising radiation, does not identify the adenoma or is discordant, it is advised that surgery is performed by a surgeon with expertise in the management of unlocalised parathyroid disease.The cure rate in such cases is over 96%. Occasionally, however, PHPT cure can be challenging.A woman in her late 40s presented with bilateral renal calculi and was found to have PHPT. Following false positive imaging and three non-curative surgical procedures, removal of a supernumerary ectopic parathyroid adenoma in the aortopulmonary window via sternotomy achieved definitive cure.

Hypertriglyceridaemia in pregnancy: an unexpected diagnosis and its management.

A woman in her 30s with gestational diabetes presented at 36 weeks' gestation with reduced fetal movements and diminishing insulin requirements. In view of her gestation, she was induced and incidentally found to have profound hyponatraemia. Further biochemical investigations confirmed severe hypertriglyceridaemia and hypercholesterolaemia. This raises the possibility of secondary causes such as familial dysbetalipoproteinemia and polygenetic hypertriglyceridaemia. She was successfully managed by aggressive dietary modification. This involved a supervised fast followed by a fat-free diet. A fenofibrate was proposed but declined due to our patient's wish to breastfeed. Management required considerable input from the multidisciplinary team. Treatment options to consider are aggressive dietary restriction of fat or the addition of a cholesterol-lowering medication, such as a fibrate. In refractory cases, a supervised fast may be required or, in cases where complications have arisen, apheresis. The patient and her baby made a good recovery with no long-lasting health implications.

Sarcoid granulomas in the parathyroid gland - a case of dual pathology: hypercalcaemia due to a parathyroid adenoma and coexistent sarcoidosis with granulomas located within the parathyroid adenoma and thyroid gland.

We present a highly unusual and interesting case of coexistent hyperparathyroidism and sarcoidosis leading to hypercalcaemia. A 70 year old female presented with weight loss, constipation and dehydration. Investigations revealed marked hypercalcaemia with a non-suppressed PTH. In view of the degree of hypercalcaemia as well as the unintentional weight loss, investigations for malignancy were conducted -these were negative. Parathyroid imaging was then requested and an adenoma was identified. Surprisingly, surgery revealed the coexistence of a parathyroid adenoma with the unexpected finding of sarcoid granulomas within the parathyroid and thyroid glands. To our knowledge, this is the first such case reported. Further imaging confirmed pulmonary sarcoidosis and a serum ACE was elevated. Serum calcium levels did not respond to parathyroidectomy but eventually fell with steroid therapy.

A Complicated Pregnancy in an Adult with HNF4A p.R63W-Associated Fanconi Syndrome.

Renal Fanconi syndrome (RFS) is characterised by generalised dysfunction of the proximal renal tubules, resulting in excessive urinary loss of solutes, most notably bicarbonate, and type II (proximal) renal tubular acidosis. It is a rare condition, and literature around its management through pregnancy is limited. We present the management of a 37-year-old woman with RFS secondary to the HNF4A p.R63W mutation, through her third pregnancy. She presented at 28 + 5 weeks with dehydration, low serum bicarbonate, and profound metabolic acidosis. Daily infusions of sodium bicarbonate were necessary, and the requirements increased throughout the pregnancy. She also demonstrated both fasting hypoglycaemia and episodes of postprandial hyperglycaemia which required complex management. Due to concerns around fetal health, an elective caesarean section was performed at 34 weeks, delivering a healthy baby girl. This case highlights the potential complexity of pregnancy in patients with RFS and the need for a multidisciplinary approach to its management.

Renin-Angiotensin System Blockade Improves Cardiac Indices in Acromegaly Patients.

Blockade of the angiotensin-renin system, with angiotensin converting enzyme inhibitors (ACEi) and angiotensin receptor blockers (ARBs), has been shown to improve cardiac outcomes following myocardial infarction and delay progression of heart failure. Acromegaly is associated with a disease-specific cardiomyopathy, the pathogenesis of which is poorly understood.The cardiac indices of patients with active acromegaly with no hypertension (Group A, n=4), established hypertension not taking ACEi/ARBs (Group B, n=4) and established hypertension taking ACEi/ARBs (Group C, n=4) were compared using cardiac magnetic imaging.Patients taking ACEi/ARBs had lower end diastolic volume index (EDVi) and end systolic volume index (ESVi) than the other 2 groups ([C] 73.24 vs. [A] 97.92 vs. [B] 101.03 ml/m2, ANOVA p=0.034, B vs. C p<0.01). Groups A and B had EDVi and ESVi values at the top of published reference range values; Group C had values in the middle of the range.Acromegaly patients on ACEi/ARBs for hypertension demonstrate improved cardiac indices compared to acromegaly patients with hypertension not taking these medications. Further studies are needed to determine if these drugs have a beneficial cardiac effect in acromegaly in the absence of demonstrable hypertension.

A seven-day diabetes service for inpatients and the emergency department in an acute hospital setting - the East and North Herts Diabetes Outreach Team (DOT).

There is evidence that all hospital-based care needs to improve across 7 days. Inpatients with diabetes require better specialist attention and improved clinical outcomes. The East and North Herts inpatient diabetes service has responded to this challenge with care now delivered by consultants and diabetes nurses, 365 days per year. We set out to provide a prospectively measurable improvement in ascertainment of appropriate patients alongside a 'care bundle' to ensure they receive a better quality experience. We also set out to document quantifiable changes in clinical data. A seven-day service is now in place and provides a variety of benefits to both professionals and patients alike.