59-year-old female with PMH of Bipolar disorder and Pituitary macroadenoma presented to the ER yesterday for worsening Headache associated with nausea, vomiting and double vision. Detailed imaging of the pituitary gland demonstrated a hypo enhancing sellar and suprasellar mass extending asymmetric to the right, appears to fill and expand the right cavernous sinus. This was associated with T2 hypo intensity. The hypo enhancing mass measured around 2.9 cm AP by 2.7 cm transverse by 3.5 cm CC, previously 2.9×2.4×3.1 cm. Of note, she was found to have this pituitary macroadenoma non-functional on prior testing. Important to note that she had history of bipolar disorder and it was refractory to many medications and was started on Lithium 10 months prior to this presentation. She underwent Image Guided Endoscopic Trans nasal Transsphenoidal Resection of Pituitary Tumor with reconstruction with Nasoseptal Flap, Lumbar Drain Placement, and Injection of Intrathecal Fluorescein Dye. Her postoperative course was complicated by Central DI and was started on DDAVP treatment. Eventually she developed Panhypopituitarism and needed Levothyroxine and Hydrocortisone replacement therapy. She had a prolonged hospital course due to post-surgical meningitis resulting in fever and encephalopathy. She had persistent hypernatremia and lithium was discontinued given the fact she was found to be in Nephrogenic DI and her hypernatremia requiring intravenous D5 water fluid treatment for a brief period.
Presentation: Monday, June 13, 2022 12:30 p.m. - 2:30 p.m.
Introduction: Thyroid storm is a rare but life-threatening emergency. Multi-organ failure has been recognized as the most common cause of death, but conventional therapies can be limited depending on the clinical presentation. We present a case of a patient in thyroid storm who rapidly developed multi-organ failure, preventing her from obtaining potentially life-saving treatment. Case Presentation: A 68-year-old female with a past medical history of hypertension, hyperlipidemia, and Grave’s disease, who was non-compliant with medications, presented to a facility for shortness of breath after the unexpected death of her husband. She was diagnosed with a non-ST elevation myocardial infarction and new onset heart failure. At that time, her TSH level was <0.010 uIU/mL and Free T4 was 1.80 ng/dL. Imaging revealed a significantly enlarged thyroid gland measuring 8cm by 6.6cm. She was started on methimazole and discharged home. A few days after discharge, she underwent a cardiac catheterization and was found to have Takotsubo cardiomyopathy. On presentation to our facility 2 weeks later, the patient was experiencing worsening shortness of breath and anxiety. She was found to have new-onset uncontrolled atrial fibrillation with rapid ventricular response and a blood pressure of 77/38 mmHg. The Burch-Wartofsky Point Scale was calculated to be 55 points, highly suggestive of thyroid storm. TSH was < 0.010 uIU/mL, total T4 was 16.63 ug/dL, and free T4 was 3.28 ng/dL. She was initiated on propylthiouracil, cholestyramine, hydrocortisone, and esmolol. Within 12 hours, she developed fulminant multi-organ failure requiring ventilatory support and vasopressors. She also developed ischemic hepatitis and propylthiouracil was discontinued. Urgent therapeutic plasma exchange (TPE) and continuous renal replacement therapy (CRRT) were later attempted but both therapies were not initiated due to severe hemodynamic instability. A bedside echocardiogram revealed an estimated ejection fraction of 20-25%. Due to worsening cardiogenic shock, she was evaluated for extracorporeal membrane oxygenation (ECMO) but was not a candidate. She instead underwent an emergent Impella device implantation. Despite this intervention, the patient’s clinical condition did not improve after multiple vasopressors, and the patient’s family opted for comfort-focused measures. The patient died after 1 day of hospitalization. Conclusion: A multimodality approach to treatment is recommended for patients with thyroid storm but underlying conditions such as Takotsubo cardiomyopathy and fulminant multi-organ failure may complicate the treatment plan. The complexity of this case highlights the need to understand relative contraindications to salvage therapies, such as TPE, and the role for other treatment options when patients present with co-existing multi-organ failure.
Background: Hypophosphatasia is a rare metabolic bone disease caused by mutations in the tissue-nonspecific alkaline (TNSALP) phosphatase gene. The disease is typically diagnosed in children but may be seen in adults with premature loss of adult teeth, poor bone healing, recurrent bone fractures, and limited mobility due to chronic pain. Clinical case: A 48-year-old female with Juvenile Onset hypophosphatasia and diabetes mellitus type 2 who presented to the endocrine clinic for diabetes mellitus. She had a telemedicine visit because she was not able to come to the office due to her recent rib fracture and pain. She had multiple fractures since she was 16-years-old with multiple fractures in her toes. She later developed a femur fracture when 20 years old and second at 28-years-old in the other femur. She was tested for juvenile hypophosphatasia and the diagnosis was confirmed. She received no treatment at that time. She subsequently had recurrent non-traumatic rib fractures that interfered with daily life. She is unable to exercise due to her frequent fractures and consequently having poor diabetic control. She has family history of brother with femur fracture. Labs showed decreased alkaline phosphatase (<5 normal: 38–126 U/L), elevated vitamin B6 levels (2296.2: normal 20–125 nmol/L) normal vitamin D (33: normal 30–100 ng/mL) and calcium levels. Chest x-ray showed multiple rib fractures with different healing stages. Dexa scan was also done and was normal to age with lowest Z-score being 8.2. Patient was started on Asfotase Alfa (an enzyme replacement therapy that contains the active site of TNSALP). Conclusion: This case illustrates the debilitating effects of hypophosphatasia and importance of recognizing previously untreated adult patients with hypophosphatasia. Many patients have very late diagnosis and suffer severe complications in their adulthood. Although this disease is rare, recognizing the symptoms of juvenile onset hypophosphatasia can help initiate early treatment to decrease these complications. Hypophosphatasia should be considered as a differential diagnosis in adults with multiple fractures and decreased alkaline phosphatase.
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