Does Marine Lenhart Syndrome really exist?

Primary hyperthyroidism is the result of overproduction of thyroid hormone resulting in the classic symptoms of tachycardia, weight loss, diaphoresis, and hyperdefecation. There are multiple common causes to include Graves’ disease, toxic multinodular goiter, and solitary toxic adenomas. Marine Lenhart Syndrome (MLS) is a rare cause of hyperthyroidism, caused by a coexistence of constitutively active thyroid nodules and Graves’ disease. In the original document of Marine and Lenhart, there is no distinction made between the autoimmune phenomenon of Graves’ disease and the solitary toxic nodule of Plummer’s disease. Rather they are both considered to be the manifestation of the same disease. However, in the current era of radionuclide technology, a clear distinction of MLS can be seen with diffuse uptake in the thyroid gland and focused enhancement in the toxic nodules. Therefore what was previously described as one entity is now distinct as Graves’ disease and Plummer’s disease. It is also becoming increasingly clear within the literature that there is also a new phenomenon of post-radioiodine immunogenic hyperthyroidism in patients with toxic nodules and elevated autoantibodies. Therefore in order to properly treat and manage patients, a new definition of MLS may need to be proposed.

Frequency of Thyroid Dysfunction in Type 2 Diabetes Patients

Thyroid problems are more common in people with type 2 diabetes. They have microvascular problems as a result of hypothyroidism. Patients with diabetes and hypothyroidism have a higher risk of cardiovascular disease. Diabetic individuals who are screened for thyroid dysfunction will be able to receive early therapy for hypothyroidism. The goal of this study was to determine the extent of thyroid dysfunction in people with type 2 diabetes and the relationship between thyroid dysfunction and diabetic complications. This study is to be conducted at a tertiary care hospital in Chennai for a period of 1 year 200 patients were selected 100 were diabetics and 100 were controls (non-diabetic). 24 patients out of 100 subjects were detected to have thyroid dysfunction. 17 patients were detected to have SCH, 6 had primary hypothyroidism and I had primary hyperthyroidism. No case of subclinical hyperthyroidism was detected. TSH values were significantly higher in diabetics. Thyroid dysfunction especially SCH was prevalent in diabetics. There was a poor glycemic control in diabetics with thyroid dysfunction.

US-guided percutaneous microwave ablation for hyperthyroidism and immediate treatment response evaluation with contrast-enhanced ultrasound

Hyperthyroidism is a common disease mainly manifested by hyperexcitability of multiple systems and hypermetabolism. Currently, antithyroid drugs (ATDs), radioiodine therapy (RIT), and surgery are mainly used in the clinical treatment for primary hyperthyroidism. We reported a case of a 28-year-old female who received a novel treatment for primary hyperthyroidism. This patient had poor control of thyroid function while taking ATD, and her oral Methimazole (MMI) dose varied repeatedly between 20 mg qd and 15 mg qd, failing to maintain a stable status. To minimize the possible complication and to achieve drug reduction or withdrawal, she refused RIT and surgery and showed up in our department. The patient, diagnosed with Graves’ disease (GD) and met the surgical indication after systematic clinical evaluation, was subject to ultrasound-guided percutaneous microwave ablation (MWA) of the partial thyroid gland with continuous oral administration of 20 mg qd MMI. The post-ablation condition was stable and the patient was discharged 2 days after the operation. Thyroid ultrasound and serum thyroid function test were examined regularly after ablation and the MMI dosage was gradually reduced according to the results of the biochemical examination. Five weeks after the operation, the patient completely discontinued the medication. Ultrasound-guided percutaneous microwave ablation is minimally invasive, safe, and effective, and has potential to be an alternative treatment besides the 3 classical treatments of hyperthyroidism.

Efficacy of ultrasonography and Tc-99m MIBI SPECT/CT in preoperative localization of parathyroid adenomas causing primary hyperthyroidism

Background Primary hyperparathyroidism (PHPT) results from an excess of parathyroid hormone (PTH) produced from an overactive parathyroid gland. The study aimed to explore the sonographic features of parathyroid adenomas and assess the diagnostic performance of ultrasonography (US) and Tc-99m MIBI SPECT/CT for preoperative localization of parathyroid adenomas. Methods A total of 107 patients were enrolled in this retrospective study who had PHPT and underwent parathyroidectomy. Of the 107 patients, 97 performed US and Tc-99m MIBI SPECT/CT examinations for preoperative localization of parathyroid nodules. The sensitivity and accuracy of each modality were calculated. Results In this study, residual parathyroid sign and polar vascular sign were identified as characteristic US features of parathyroid adenomas. These manifestations were closely related to the size of the abnormal parathyroid lesions. Among the 108 parathyroid nodules from 97 patients with PHPT, the sensitivity and accuracy of US for locating the parathyroid nodules were significantly higher than those of Tc-99m MIBI SPECT/CT (93.0% vs. 63.0% and 88.0% vs. 63.0% respectively; χ2 = 26.224, 18.227 respectively, P < 0.001). The differences between US + Tc-99m MIBI SPECT/CT and Tc-99m MIBI SPECT/CT-alone were statistically significant (χ2 = 33.410, 21.587 respectively, P < 0.001), yet there were no significant differences in the sensitivity or accuracy between US + Tc-99m MIBI SPECT/CT and US-alone (χ2 = 0.866, 0.187 respectively, P = 0.352 and 0.665). Conclusions US shows significantly better sensitivity and accuracy for localization of parathyroid adenomas than Tc-99m MIBI SPECT/CT. However, US combined with Tc-99m MIBI SPECT/CT is of great clinical value in the preoperative localization of parathyroid nodules in patients with PHPT.

Thyrotropin-Producing Pituitary Adenomas

Thyrotropin-producing pituitary adenomas are rare cause of hyperthyroidism and it compose about 0.5-3% of all functioning pituitary tumors. Diagnosis of thyrotropin-producing pituitary adenomas can be challenging because TSH concentrations can be normal with only elevated T4. As a result, patient would be simply treated as primary hyperthyroidism. The latency between onset of hyperthyroidism and diagnosis of pituitary adenoma was reported to be 4-6 years. Many patients had radioactive iodine treatment or thyroidectomy treatment for primary hyperthyroidism at the time of diagnosis. A 35-year-old male with history of intermittent FT4 elevation for the past 3 years presented at endocrine clinic for evaluation. Patient also had recent headache and dizziness. TSH was only marginally elevated once in the past 3 years. Alpha-subunit was found to be 2.6 ng/mL with negative heterophile antibody. MRI showed a 13.7 mm x 20.4mm x16.1mm sellar and suprasellar mass without cavernous sinus invasion but with chiasmal compression. Other pituitary hormone co-secretion was not found in this patient. Patient was treated with octreotide 20mg monthly for 3 month with normalization of T4 and size of the tumor also decreased on the medication. Patients was prepared for transsphenoidal surgery. Treatment for thyrotropin-producing pituitary adenomas is mainly surgery. However, medical therapy with somatostatin analogs does play an important part in terms of inducing euthyroid prior to surgery. There are also articles describing shrinkage of the tumor prior to surgery while on somatostatin analogs. There were also rare case reports of thyroid storm from thyrotropin-producing pituitary adenomas when patients were not treated with somatostatin analogs prior to surgery. The surgical outcome was determined by the size of the tumor. Transient hypothyroidism or hypopituitarism can happen after the surgery. However, it is more common in external beam radiotherapy or radiosurgery treatment. For thyrotropin-producing pituitary microadenomas, transsphenoidal surgery is the treatment of choice with high remission rate. In some difficult cases where octreotide was not controlling the hyperthyroidism, methimazole use in combination with octreotide after surgery was also documented in the literature. The idea of using somatostatin analogs as primary treatment of thyrotropin-producing pituitary adenomas due to the risk of hypopituitarism with transsphenoidal surgery was explored in some literature. But, no strong evidence of better outcome with medication treatment alone was found.

Immune Check Point Inhibitors Triggering Non Autoimmune Polyendocrinopathy

The superlative therapeutic response of cancer immunotherapy is activation of the immune system against cancer cells. Currently, one of the most considered immune system enhancer are the immunomodulatory antibodies well known as checkpoint inhibitor therapy. Among this type of treatment, programed cell death-1 receptor blocker, is one of the most sought-after therapies on demand now a day. Notwithstanding the important clinical benefits of this therapeutic modality, serial autoimmune adverse effects and variety of atypical presentations of life-threatening endocrinopathies are expected to occur. We present a case of a 78-year-old man with dyslipidemia and lung CA who was referred to our clinic after developing electrolyte disturbances with associated dizziness and fatigue one month after Pembrolizumab therapy initiation. Physical exam was unremarkable. Laboratory data was consistent with mild hyponatremia, hyperkalemia and adequate fasting blood sugar levels. Aldosterone levels were extremely low, ACTH levels were extremely high with inappropriate low total cortisol response and negative 21-Hydroxylase antibodies. Diagnosis of primary adrenal insufficiency was established and Fludrocortisone 0.05 mg PO daily therapy was started with further resolution of hyponatremia and initial symptoms. In addition, concurrent primary hyperthyroidism along with thyroid RAIU-Scan results were consistent with thyroiditis, but TSI and TPO’s antibody levels were unexpectedly negative. Eventually, a suspicious thyroid nodule was identified requiring biopsy. Initial FNA results showed a follicular lesion of undetermined significance followed by a benign finding when repeated after six months. During follow up, patient’s primary hyperthyroidism converted to severe primary hypothyroidism without any intervention for her prior hyperthyroidism. Patient’s TPO’s levels remain undetectable and his current status is post-thyroiditis with residual primary hypothyroidism. Primary adrenal insufficiency also persist and its antibodies have not yet been identified either. It is known that autoimmunity can predispose to the development of primary adrenal and thyroid disorders in patients undergoing PD-1 receptor blockers therapy against cancer. Both disorders are increasingly recognized and reported as one of the most common adverse effects presenting in patients treated with these agents. However, to our knowledge, cases of non-immune related adverse effects are barely documented. This case of uncommon endocrine manifestations related to checkpoint inhibitors therapy is meritorious of being reported since it should raise awareness in the medical community for prompt identification of signs and symptoms, as well as to offer adequate management, accurate treatment and provide a better standard of care.

Thyroid Storm: A Rare Paraneoplastic Syndrome in an Infant With Hepatoblastoma

Background: Graves disease (GD) is the most common cause of pediatric hyperthyroidism. Thyroid storm (TS) is a rare initial manifestation of GD and is typically triggered by an underlying stressor such as infection, trauma or surgery in a patient with underlying GD and poorly controlled hyperthyroidism. Clinical Case: A previously healthy 21-month-old Hispanic male presented to our ER due to concerns of acute abdominal pain. He was noted to have diffuse abdominal tenderness, unremitting anxiety and mild exophthalmos. Vital signs revealed tachycardia and hypertension. Initial lab evaluation was suggestive of primary hyperthyroidism (TSH&lt;0.02 mcU/mL, n 0.5-4.5 mcU/mL, and free T4 at 5.8 ng/dL, n 0.8-2 ng/dL). His Burch-Wartofsky point scale score was 45, indicating high likelihood of TS. He was aggressively treated with methimazole, potassium iodide and propranolol. Five days later, there was a significant improvement in symptoms and labs (TSH&lt;0.02 mcU/mL, n 0.5-4.5 mcU/mL, and free T4 2.3 ng/dL, n 0.8-2 ng/dL) and he was discharged home on methimazole and propranolol. Interestingly, all thyroid autoantibodies were negative including TSI, TRAb, anti-thyroglobulin and anti-TPO antibodies. His thyroid function continued to improve and propranolol was discontinued. Three weeks after his initial hospital admission, he developed a diffuse urticarial rash and the methimazole was held and propranolol restarted. Within a few days, he developed persistent fevers for which he was readmitted. His total T4 at that time was elevated at 23 mcg/dL (n 4.5-11 mcg/dL) and because the rash was improving, methimazole was restarted. In the following weeks, the patient continued to have intermittent fevers, diffuse waxing and waning rash, decreased activity, and reduced appetite. He was seen by his pediatrician who noted hepatomegaly. Abdominal CT and ultrasound revealed a liver mass (11 x 10 x 10 cm) and a 7 mm peripherally placed pulmonary nodule in the left lower lobe. The patient was admitted to the hematology/oncology unit for further evaluation. He was found to have an elevated alpha-fetoprotein level (AFP) of 43,051 ng/mL, n&lt;6 ng/mL, which was concerning for hepatoblastoma (HB) that was confirmed by tissue biopsy. He was subsequently initiated on neoadjuvant chemotherapy. Methimazole dose requirements gradually decreased with eventual discontinuation 1 week after initiation of chemotherapy. He has remained clinically euthyroid off methimazole for almost 3 months with normal thyroid function. Conclusion: We report an unusual case of transient hyperthyroidism that initially presented as TS in a 21 month old male who was subsequently diagnosed with HB. Paraneoplastic syndromes (PNS) may occur with any tumor. Hyperthyroidism is occasionally associated with non-seminomatous germ-cell tumors with elevated HCG. We believe this is the first report of pediatric thyroid storm as a PNS in HB.

Unusual clinical scenarios in Urology and Andrology

This collection includes some unusual cases and how they were diagnosed and treated. Case 1: A case of a patient with primary hyperthyroidism presenting with a submucosal ureteral stone after endoscopic lithotripsy was described. After multiple endoscopic treatment, the stone was successfully removed by open ureterolithotomy recovering ureteral patency and normal renal function. Case 2: A case of burned-out testicular cancer with atypical lymphatic spread (stage II A) was presented. After right orchiectomy and complete remission of tumor markers, due to atypical metastases location and uncertain histology, the patient was treated with systemic therapy based on bleomycin, etoposide and cisplatin (PEB). At re-staging after chemotherapy computed tomography showed reduction of all node metastases and an observation protocol was proposed. Case 3: A patient was readmitted to hospital after 12 days from an uneventful Robot-Assisted Radical Prostatectomy (RARP) for prostate cancer due to lower abdominal pain plus abdominal distension, nausea and constipation not responsive to medical therapy. Computed Tomography showed colon and small bowel dilatation without any evidence of anatomical or mechanical obstruction. Laparoscopic abdominal exploration confirmed bowel distension without evidence of obstructing lesions. Ogilvie’s Syndrome or acute colonic pseudo-obstruction (ACPO) was diagnosed. The patient fully recovered and was discharged six days after the procedure. Case 4: A case of recurrent Acute Idiopathic Scrotal Edema (AISE) was diagnosed on clinical signs together with the decisive help of pathognomonic ultrasound findings as the “fountain sign”. Case 5: Small bilateral testicular nodules were diagnosed in a 30-years old patient undergoing scrotal ultrasound in follow up of acute lymphoblastic leukemia. Ultrasound guided testis sparing surgery was performed demonstrating Leydig cell tumors.

Central Hyperthyroidism due to Thyroid-Stimulating Hormone-Secreting Pituitary Microadenoma in an Adolescent Boy: Case Report and Review of the Literature

Thyroid-stimulating hormone- (TSH-) secreting pituitary adenoma (TSH-oma) is a rare cause of secondary hyperthyroidism and can be misdiagnosed as primary hyperthyroidism. We report a case of a 15-year-old male patient who was one of two monozygotic twins and exhibited hyperthyroidism syndrome. The laboratory results showed secondary hyperthyroidism, with increased levels of free T3 (FT3) and free T4 (FT4) and no TSH inhibition. Magnetic resonance imaging (MRI) and histopathological examination of the pituitary gland confirmed pituitary microadenoma. The patient was treated with methimazole, propranolol, and somatostatin analogs to restore euthyroidism before undergoing an endoscopic transsphenoidal resection of the pituitary tumor. After surgery, the hyperthyroidism symptoms improved, thyroid hormones normalized, and MRI of the pituitary gland showed the complete removal of the tumor with no recurrence after 2 years of follow-up.

Efficacy of Ultrasonography and Tc-99m MIBI SPECT/CT in Preoperative Localization of Parathyroid Adenomas Causing Primary Hyperthyroidism

Background: Primary hyperparathyroidism (PHPT) results from an excess of parathyroid hormone (PTH) produced from an overactive parathyroid gland. The study aimed to explore the sonographic features of parathyroid adenomas and assess the diagnostic performance of ultrasonography (US) and Tc-99m MIBI SPECT/CT for preoperative localization of parathyroid adenomas. Methods: A total of 107 patients were enrolled in this retrospective study who had PHPT and underwent parathyroidectomy. Of the 107 patients, 97 performed US and Tc-99m MIBI SPECT/CT examinations for preoperative localization of parathyroid nodules. The sensitivity and accuracy of each modality were calculated. Results: In this study, residual parathyroid sign and polar vascular sign were identified as characteristic US features of parathyroid adenomas and these manifestations are closely related to the size of the abnormal parathyroid lesions. Using 108 parathyroid nodules from 97 patients with PHPT, the sensitivity and accuracy of US in locating parathyroid nodules were significantly higher than those of Tc-99m MIBI SPECT/CT (93.0% vs. 63.0% and 88.0% vs. 63.0%), and the difference was statistically significant (c²=26.224, 18.227, P<0.001). The difference between US combined with Tc-99m MIBI SPECT/CT and Tc-99m MIBI SPECT/CT-alone was statistically significant (c²=33.410, 21.587, P<0.001), yet there was no significant difference compared with US alone (c²=0.866, 0.187, P=0.352 and 0.665). Conclusions: US shows significantly better sensitivity and accuracy for localization of parathyroid adenomas than Tc-99m MIBI SPECT/CT. However, US combined with Tc-99m MIBI SPECT/CT is of great clinical value in the preoperative localization of parathyroid nodules in patients with PHPT.