Case Reports in Endocrinology http://www.hindawi.com The latest articles from Hindawi Publishing Corporation © 2016 , Hindawi Publishing Corporation . All rights reserved. Panhypopituitarism due to Absence of the Pituitary Stalk: A Rare Aetiology of Liver Cirrhosis Sun, 24 Apr 2016 06:54:44 +0000 http://www.hindawi.com/journals/crie/2016/9071097/ Studies have established a relationship between hypothalamic-pituitary dysfunction and the onset of liver damage, which may occasionally progress to cirrhosis. Patients with hypopituitarism can develop a metabolic syndrome-like phenotype. Insulin resistance is the main pathophysiological axis of metabolic syndrome and is the causal factor in the development of nonalcoholic fatty liver disease (NAFLD). We present the case of a young patient with liver cirrhosis of unknown aetiology that was finally attributed to panhypopituitarism. Marta Gonzalez Rozas, Lidia Hernanz Roman, Diego Gonzalez Gonzalez, and José Luis Pérez-Castrillón Copyright © 2016 Marta Gonzalez Rozas et al. All rights reserved. Gigantomastia and Macroprolactinemia Responding to Cabergoline Treatment: A Case Report and Minireview of the Literature Mon, 18 Apr 2016 09:52:52 +0000 http://www.hindawi.com/journals/crie/2016/3576024/ Background. Macroprolactinemia is defined as predominance of high molecular weight prolactin forms in the circulation. Although macroprolactin is considered as a biologically inactive molecule, some authorities suggest treatment in symptomatic cases. Gigantomastia is defined as excess breast tissue and most cases in the literature were treated by surgical intervention. Case. A 44-year-old woman was admitted to our clinic with gigantomastia and galactorrhea. The patient had a demand for surgical therapy. In laboratory examination, she had hyperprolactinemia and macroprolactinemia. Pituitary imaging revealed 6 mm microadenoma in right side of the hypophysis. Since she was symptomatic, cabergolin treatment was started. Macroprolactin became negative, breast circumference decreased significantly, and galactorrhea resolved after treatment. Conclusion. Gigantomastia might be the presenting symptom in patients with macroprolactinemia. In these patients medical treatment with cabergoline may be used initially as an alternative to surgical approach. Fatma Dilek Dellal, Didem Ozdemir, Cevdet Aydin, Gulfem Kaya, Reyhan Ersoy, and Bekir Cakir Copyright © 2016 Fatma Dilek Dellal et al. All rights reserved. Primary Mucosa-Associated Lymphoid Tissue Lymphoma of Thyroid with the Serial Ultrasound Findings Wed, 23 Mar 2016 13:55:39 +0000 http://www.hindawi.com/journals/crie/2016/5608518/ Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT) of the thyroid gland is uncommon. Even though its natural history is not well defined, it is known to be indolent course. We present a case of primary MALT thyroid lymphoma with the serial sonographic findings in the patient presenting as the focal nodule. A 45-year-old woman visited our hospital for neck examination. Initially, fine-needle aspiration cytology in the focal hypoechoic lesion in the left thyroid lobe on ultrasound sonography was performed and consistent with Hashimoto’s thyroiditis. However, the results of serial ultrasounds and core-needle biopsy revealed an extranodal marginal zone lymphoma of MALT on 4-year follow-up. Patients with a focal hypoechoic nodule with linear echogenic strands and segmental pattern in the background of Hashimoto’s thyroiditis on ultrasonography should undergo careful surveillance for malignancy. Serial sonographic features in this case are meaningful in the understanding of the natural history of the extranodal marginal zone lymphoma of MALT of the thyroid. Eon Ju Jeon, Ho Sang Shon, and Eui Dal Jung Copyright © 2016 Eon Ju Jeon et al. All rights reserved. Large Multinodular Toxic Goiter: Is Surgery Always Necessary? Wed, 23 Mar 2016 12:15:40 +0000 http://www.hindawi.com/journals/crie/2016/1320827/ Patients suffering from multinodular toxic goiter (MNTG) are candidates to thyroidectomy or radioiodine 131I (131I) therapy. Thyroidectomy may be preferable especially when the volume of hyperfunctioning tissue is so large that a single administration of 131I is unlikely to cure the patient in terms of nodule’s volume reduction and thyroid function. We describe the case of a 71-year-old man suffering from thyrotoxic state for the presence of two large hyperfunctioning thyroid nodules. As the patient refused surgery, at first we administered 600 MBq dose of 131I, which was unable to solve hyperthyroidism and local compressive symptoms. Then, before administering another 131I dose, the patient underwent a laser ablation treatment (LAT) in both nodules. After a significant shrinkage due to LAT, the patient received 400 MBq 131I. This procedure was able to definitely cure hyperthyroidism, to induce a significant reduction of nodules’ volume, and to render the patient asymptomatic for compressive symptoms. This case demonstrates that 131I preceded by LAT represents a valid alternative strategy to surgery, even in the presence of two large coexistent hot nodules. Roberto Negro and Gabriele Greco Copyright © 2016 Roberto Negro and Gabriele Greco. All rights reserved. Nonsuppressible Oral Dexamethasone Suppression Tests but Not Cushing Syndrome Tue, 22 Mar 2016 15:58:24 +0000 http://www.hindawi.com/journals/crie/2016/3684287/ In spite of the presence of definitive diagnostic criteria to diagnose Cushing syndrome diagnosis may become challenging. We report a young female with mild clinical features of Cushing syndrome, who had nonsuppressible oral dexamethasone suppression tests; also she had a suspicious pituitary lesion. She underwent pituitary surgery and a pituitary microadenoma (non-ACTH staining) was removed. Now she had come to us with similar complaints to those before. Again she had nonsuppressible oral dexamethasone suppression tests. As the diurnal variation of serum and salivary cortisol was maintained and urinary free cortisol was normal, further evaluation with IV dexamethasone suppression test was performed which clearly ruled out Cushing syndrome. The patient was not on any medicines known to alter dexamethasone metabolism. Fat malabsorption was also ruled out using appropriate tests. The reason for this discrepancy is thought to be altered (increased) metabolism of dexamethasone in this patient as it is widely variable in the general population. Abilash Nair, Atul Dhingra, Anjana Gopi, and Viveka P. Jyotsna Copyright © 2016 Abilash Nair et al. All rights reserved. Cervical Paraganglioma Mimicking Thyroid Nodule: A Rare Clinical Case Tue, 15 Mar 2016 12:01:43 +0000 http://www.hindawi.com/journals/crie/2016/8527279/ Objective. Paraganglioma is a rare neuroendocrine tumor. When it is located in the neck, it is commonly misdiagnosed as other thyroid neoplasms. Case Report. We report a case of cervical paraganglioma in a 55-year-old female. Patient was admitted to our clinic with goiter and neck pain. Thyroid ultrasonography revealed a 20 mm solitary, heterogeneous nodule located in the upper pole of left thyroid lobe. Fine needle aspiration cytology was nondiagnostic. She underwent left lobectomy and histopathology showed paraganglioma. Discussion. Cervical paragangliomas should be considered in the differential diagnosis of thyroid nodules. Berna İmge Aydoğan, Serpil Dizbay Sak, and Sevim Güllü Copyright © 2016 Berna İmge Aydoğan et al. All rights reserved. Amyloid Goiter Secondary to Ulcerative Colitis Tue, 08 Mar 2016 12:11:58 +0000 http://www.hindawi.com/journals/crie/2016/3240585/ Diffuse amyloid goiter (AG) is an entity characterized by the deposition of amyloid in the thyroid gland. AG may be associated with either primary or secondary amyloidosis. Secondary amyloidosis is rarely caused by inflammatory bowel diseases. Secondary amyloidosis is relatively more common in the patients with Crohn’s disease, whereas it is highly rare in patients with ulcerative colitis. Diffuse amyloid goiter caused by ulcerative colitis is also a rare condition. In the presence of amyloid in the thyroid gland, medullary thyroid cancer should be kept in mind in the differential diagnosis. Imaging techniques and biochemical tests are not very helpful in the diagnosis of secondary amyloid goiter and the definitive diagnosis is established based on the histopathologic analysis and histochemical staining techniques. In this report, we present a 35-year-old male patient with diffuse amyloid goiter caused by secondary amyloidosis associated with ulcerative colitis. Bunyamin Aydin, Yavuz Savas Koca, Tugba Koca, Ihsan Yildiz, Sevda Gerek Celikden, and Metin Ciris Copyright © 2016 Bunyamin Aydin et al. All rights reserved. Hashimoto’s Thyroiditis and Graves’ Disease in One Patient: The Extremes of Thyroid Dysfunction Associated with Interferon Treatment Wed, 02 Mar 2016 09:01:58 +0000 http://www.hindawi.com/journals/crie/2016/6029415/ Autoimmune thyroid disease associated with interferon therapy can manifest as destructive thyroiditis, Graves’ Hyperthyroidism, and autoimmune (often subclinical) hypothyroidism, the latter persisting in many patients. There are scare reports of a single patient developing extremes of autoimmune thyroid disease activated by the immunomodulatory effects of interferon. A 60-year-old man received 48 weeks of pegylated interferon and ribavirin therapy for chronic HCV. Six months into treatment, he reported fatigue, weight gain, and slowed cognition. Serum thyroid stimulating hormone (TSH) was 58.8 mIU/L [0.27–4.2], fT4 11.1 pmol/L [12–25], and fT3 4.2 pmol/L [2.5–6.0] with elevated anti-TPO (983 IU/mL [<35]) and anti-TG (733 U/mL [<80]) antibodies. He commenced thyroxine with initial clinical and biochemical resolution but developed symptoms of hyperthyroidism with weight loss and tremor 14 months later. Serum TSH was <0.02 mIU/L, fT4 54.3 pmol/L, and fT3 20.2 pmol/L, with an elevated TSH receptor (TRAb, 4.0 U/L [<1.0]), anti-TPO (1,163 IU/mL) and anti-TG (114 U/mL) antibodies. Technetium scan confirmed Graves’ Disease with bilateral diffuse increased tracer uptake (5.9% [0.5–3.5%]). The patient commenced carbimazole therapy for 6 months. Treatment was ceased following spontaneous clinical and biochemical remission (TSH 3.84 mIU/L, fT4 17pmol/L, fT3 4.5 pmol/L, and TRAb <1 U/L). This raises the need to monitor thyroid function closely in patients both during and following completion of interferon treatment. R. H. Bishay and R. C. Y. Chen Copyright © 2016 R. H. Bishay and R. C. Y. Chen. All rights reserved. A Case Report of Dramatically Increased Thyroglobulin after Lymph Node Biopsy in Thyroid Carcinoma after Total Thyroidectomy and Radioiodine Mon, 29 Feb 2016 18:46:56 +0000 http://www.hindawi.com/journals/crie/2016/6471081/ Thyroglobulin (Tg) is an important modality for monitoring patients with thyroid cancers, especially after thyroidectomy followed by radioiodine (RAI). It is also used as a marker for burden of thyroid tissue whether malignant or benign. Although there have been several reports of rising serum Tg transiently after thyroid biopsy in intact glands and following palpation or trauma, there are no reports in the literature of elevation in Tg after biopsy of suspicious lesions in thyroidectomized patients. In this paper we report a fascinating case of a considerable and initially worrying, although ultimately transient, rise in Tg in a patient 2 years after total thyroidectomy and RAI ablation after fine needle aspiration (FNA) of a suspicious thyroid bed nodule that was proven positive. Mandana Moosavi and Stuart Kreisman Copyright © 2016 Mandana Moosavi and Stuart Kreisman. All rights reserved. Thyroid Ultrasound Pitfalls: Esophageal Fibrovascular Polyp Mimicking Thyroid Nodule Sun, 28 Feb 2016 08:14:21 +0000 http://www.hindawi.com/journals/crie/2016/3601508/ Background. Ultrasound (US) is the most accurate tool in the diagnosis of thyroid nodules if performed by expert physician. Misdiagnosis due to extrathyroidal lesions mimicking thyroid nodules is reported in literature. We describe the first case of an esophageal fibrovascular polyp misdiagnosed as a thyroid nodule on US examination. Patient Findings. A 54-year-old woman presented to emergency department for headache and underwent carotid Doppler extended to neck ultrasound with incidental finding of a nodule in the posterior side of the left thyroid lobe. A following thyroid US performed by an endocrinologist allowed the characterization of the lesion as an esophageal pathology, considering the extrathyroidal position, the typical peripheral hyperechoic spots and hypoechoic rim, the connection to the esophagus, and the swallowing connected movement. The patient was addressed to further investigations and finally to anterior pharyngotomy with histological diagnosis of esophageal fibrovascular polyp. Summary. Differential diagnosis between thyroid nodules and other neck lesions is important to prevent an unnecessary fine needle aspiration biopsy and to treat the extrathyroidal pathology. In this case, an US performed by an expert endocrinologist allowed detecting an esophageal fibrovascular polyp requiring surgical removal. In conclusion, the possibility of an esophageal pathology, and even fibrovascular polyp, should be considered during US thyroid examination. Anna Ansaloni, G. Brigante, and B. Madeo Copyright © 2016 Anna Ansaloni et al. All rights reserved. Mifepristone Improves Octreotide Efficacy in Resistant Ectopic Cushing’s Syndrome Wed, 17 Feb 2016 11:40:08 +0000 http://www.hindawi.com/journals/crie/2016/8453801/ A 30-year-old Caucasian man presented with severe Cushing’s syndrome (CS) resulting from ectopic adrenocorticotropin syndrome (EAS) from a metastatic pancreatic neuroendocrine tumor. The patient remained hypercortisolemic despite treatment with steroidogenesis inhibitors, chemotherapy, and octreotide long-acting release (LAR) and was enrolled in a 24-week, phase 3 clinical trial of mifepristone for inoperable hypercortisolemia. After mifepristone was added to ongoing octreotide LAR treatment, EAS symptoms essentially resolved. Cortisol decreased dramatically, despite mifepristone’s competitive glucocorticoid receptor antagonist effects. The clinical and biochemical effects reversed upon mifepristone discontinuation despite the continued use of octreotide LAR therapy. Substantial improvement in octreotide LAR efficacy with mifepristone use was noted in this patient with ectopic CS, consistent with upregulation of somatostatin receptors previously downregulated by hypercortisolemia. Andreas G. Moraitis and Richard J. Auchus Copyright © 2016 Andreas G. Moraitis and Richard J. Auchus. All rights reserved. Acute Kidney Injury Associated with Linagliptin Sun, 14 Feb 2016 13:52:14 +0000 http://www.hindawi.com/journals/crie/2016/5695641/ Linagliptin is a dipeptidyl peptidase-IV (DPP-IV) inhibitor that is approved for the treatment of type 2 diabetes mellitus. About 5% of linagliptin is eliminated by the kidneys and no dose adjustment is recommended in kidney impairment. We report a first case of linagliptin-associated acute kidney injury (AKI) in a patient with preexisting chronic kidney disease (CKD). We hypothesize that AKI was due to renal hypoperfusion from linagliptin-induced natriuresis and intravascular volume contraction in the setting of concomitant lisinopril use, which is known to impair autoregulation and potentiate hypotension-induced AKI. It may be prudent to exert caution and closely monitor kidney function when initiating linagliptin in combination with ACE-inhibitors in CKD patients. Deepak K. Nandikanti, Elvira O. Gosmanova, and Aidar R. Gosmanov Copyright © 2016 Deepak K. Nandikanti et al. All rights reserved. Pegvisomant-Induced Cholestatic Hepatitis in an Acromegalic Patient with UGT1A128 Mutation Tue, 09 Feb 2016 14:06:46 +0000 http://www.hindawi.com/journals/crie/2016/2087102/ Pegvisomant (PEGv) is a growth hormone receptor antagonist approved for the treatment of acromegaly; one of its documented adverse effects is reversible elevation of hepatic enzymes. We report a 39-year-old male acromegalic patient with a pituitary macroadenoma who underwent transsphenoidal surgery. The patient’s condition improved but GH and IGF-I levels did not normalize; as a consequence, we first administered dopamine agonists and then somatostatin receptor ligands (SRLs) with poor response. PEGv 15 mg every other day was added to lanreotide 120 mg monthly. The patient developed a severe hepatitis five months after starting the combination therapy. Elevated ferritin, iron, and transferrin saturation suggested probable hepatitis due to haemochromatosis. We performed a liver biopsy which showed an acute cholestatic hepatitis consistent with toxic etiology. A heterozygous genotype UGT1A128 polymorphism associated with Gilbert’s syndrome was also found in this Argentine patient. The predominant clinical presentation resembled an acute cholestatic hepatitis associated with severe hemosiderosis, a different and new pattern of PEGv hepatotoxicity. Maria Susana Mallea-Gil, Ignacio Bernabeu, Adriana Spiraquis, Alejandra Avangina, Lourdes Loidi, and Carolina Ballarino Copyright © 2016 Maria Susana Mallea-Gil et al. All rights reserved. Thyroid Swelling and Thyroiditis in the Setting of Recent hCG Injections and Fine Needle Aspiration Thu, 28 Jan 2016 08:59:46 +0000 http://www.hindawi.com/journals/crie/2016/2915816/ A 60-year-old woman presented with a neck mass and underwent fine needle aspiration of a left thyroid nodule. During this time, she had been injected with hCG for weight loss. Soon after, she developed rapid diffuse thyroid growth with pain. She was ultimately diagnosed with thyrotoxicosis due to postaspiration subacute thyroiditis and subsequently became hypothyroid. This condition is rare in the nonpregnant state in noncystic nodules with a smaller needle gauge approach. The incidence of thyroid nodule discovery and evaluation is increasing. As more procedures are undertaken, understanding of potential complications is important. This case highlights potential complications of thyroid fine needle aspiration including diffuse thyroid swelling and thyroiditis. The role of hCG injections is speculated to have potentially stimulated thyroid follicular epithelium via cross-reactivity with the TSH receptor and contributed to the acute inflammatory response after fine needle aspiration. Elizabeth M. Lamos and Kashif M. Munir Copyright © 2016 Elizabeth M. Lamos and Kashif M. Munir. All rights reserved. An Ectopic ACTH Secreting Metastatic Parotid Tumour Wed, 20 Jan 2016 09:19:12 +0000 http://www.hindawi.com/journals/crie/2016/4852907/ A 60-year old woman presented with features of Cushing’s syndrome (CS) secondary to an ectopic adrenocorticotropic hormone (ACTH) secreting metastatic parotid tumour 3 years after excision of the original tumour. She subsequently developed fatal intestinal perforation and unfortunately died despite best possible medical measures. Ectopic ACTH secretion accounts for 5–10% of all patients presenting with ACTH dependent hypercortisolism; small cell carcinoma of lung (SCLC) and neuroendocrine tumours (NET) account for the majority of such cases. Although there are 4 previous case reports of ectopic ACTH secreting salivary tumours in literature, to our knowledge this is the first published case report in which the CS developed after 3 years of what was deemed as a successful surgical excision of primary salivary tumour. Our patient initially had nonspecific symptoms which may have contributed to a delay in diagnosis. Perforation of sigmoid colon is a recognised though underdiagnosed complication associated with steroid therapy and hypercortisolism. This case demonstrates the challenges faced in diagnosis as well as management of patients with CS apart from the practical difficulties faced while trying to identify source of ectopic ACTH. Thomas Dacruz, Atul Kalhan, Majid Rashid, and Kofi Obuobie Copyright © 2016 Thomas Dacruz et al. All rights reserved. DKA with Severe Hypertriglyceridemia and Cerebral Edema in an Adolescent Boy: A Case Study and Review of the Literature Wed, 20 Jan 2016 09:07:40 +0000 http://www.hindawi.com/journals/crie/2016/7515721/ A 13-year-old adolescent boy with type 1 diabetes mellitus (1b) presented with diabetic ketoacidosis (DKA) and cerebral edema. Grossly lipemic serum and lipemia retinals due to extremely high triglyceride (TG) level were observed without evidence of xanthoma or xanthelasma. Cerebral edema was treated by appropriate ventilation and mannitol administration. Normal saline was carefully given and regular insulin was titrated according to blood sugar levels. Triglyceride levels were reduced from 9,800 mg/dL to normal range within 9 days after conventional treatment was commenced without antilipid medication. Based on our review of the literature, this is the first reported case of confirmed pediatric DKA with severe hypertriglyceridemia and cerebral edema. In patients with DKA and hypertriglyceridemia, clinicians should be mindful of the possibility of associated acute pancreatitis and cerebral edema. Tansit Saengkaew, Taninee Sahakitrungruang, Suttipong Wacharasindhu, and Vichit Supornsilchai Copyright © 2016 Tansit Saengkaew et al. All rights reserved. Pseudohypoaldosteronism in a Neonate Presenting as Life-Threatening Hyperkalemia Tue, 19 Jan 2016 14:19:30 +0000 http://www.hindawi.com/journals/crie/2016/6384697/ Context. Pseudohypoaldosteronism type 1 (PHA1) is a life-threatening disease that causes severe hyperkalemia and cardiac arrest if not treated appropriately or if diagnosis is missed. Objective. To report a case of a newborn with vomiting and lethargy, ultimately diagnosed with pseudohypoaldosteronism. Patient. This case presented to the ED at an age of 14 days in hypovolemic shock. There was a family history of sudden infant death, her sister who was diagnosed with CAH and passed away at 3 months of age despite regular hormone replacement. Our patient had cardiac arrest in ED, due to hyperkalemia; while receiving fluid boluses, cardiopulmonary resuscitation was initiated. After stabilization, diagnostic workup demonstrated persistently low sodium, acidosis, and high potassium, which required peritoneal dialysis. Based on these findings, the patient was diagnosed with CAH. It turned out later that the patient had PHA1. Two years later, the patient had a new sibling with the same disease diagnosed at birth and started immediately on treatment without any complication. Conclusions and Outcome. This case highlights the significant diagnostic and therapeutic challenges in treating children with PHA1. Adrenal crisis is not always CAH; delayed diagnosis can lead to complication and even death. The presence of high plasma renin activity, aldosterone, and cortisol, along with the presence of hyponatremia and hyperkalemia, established the diagnosis of PHA type 1 and ruled out CAH. Najya A. Attia and Yousef I. Marzouk Copyright © 2016 Najya A. Attia and Yousef I. Marzouk. All rights reserved. Treatment of Ipilimumab Induced Graves’ Disease in a Patient with Metastatic Melanoma Mon, 11 Jan 2016 06:06:58 +0000 http://www.hindawi.com/journals/crie/2016/2087525/ Objective. Thyroid disease has been reported among the endocrinopathies that can occur after treatment with ipilimumab. Graves’ disease, however, has been rarely reported with this medication. Here we report a case of Graves’ disease diagnosed after initiation of ipilimumab in a patient with melanoma. Methods. We present the clinical presentation and management course of this patient followed by a related literature review. Results. A 67-year-old male with metastatic melanoma was started on ipilimumab. He developed hyperthyroidism after two doses of ipilimumab. The cause of hyperthyroidism was determined to be Graves’ disease. Ipilimumab was held and the patient was started on methimazole with return to euthyroid status. Ipilimumab was resumed and the patient continued methimazole during the course of ipilimumab therapy, with controlled hyperthyroidism. Restaging studies following four cycles of ipilimumab showed complete response in the lungs, with residual melanoma in the neck. The patient then underwent total thyroidectomy and left neck dissection as a definitive treatment for both hyperthyroidism and residual melanoma. Conclusion. Graves’ disease can develop after starting ipilimumab and methimazole can be an effective treatment. For patients whose hyperthyroidism is well-controlled on methimazole, ipilimumab may be resumed with close monitoring. Umal Azmat, David Liebner, Amy Joehlin-Price, Amit Agrawal, and Fadi Nabhan Copyright © 2016 Umal Azmat et al. All rights reserved. Measurement of Serum Free Thyroxine Index May Provide Additional Case Detection Compared to Free Thyroxine in the Diagnosis of Central Hypothyroidism Tue, 08 Dec 2015 07:37:55 +0000 http://www.hindawi.com/journals/crie/2015/965191/ The diagnosis of central hypothyroidism is often suspected in patients with hypothalamic/pituitary pathology, in the setting of low, normal, or even slightly elevated serum TSH and low free thyroxine (FT4). We present four cases of central hypothyroidism (three had known pituitary pathology) in whom central hypothyroidism was diagnosed after the serum free thyroxine index (FTI) was found to be low. All had normal range serum TSH and free thyroxine levels. This report illustrates that the assessment of the serum FTI may be helpful in making the diagnosis of central hypothyroidism in the appropriate clinical setting and when free T4 is in the low-normal range, particularly in patients with multiple anterior pituitary hormone deficiencies and/or with symptoms suggestive of hypothyroidism. Kevin M. Pantalone, Betul Hatipoglu, Manjula K. Gupta, Laurence Kennedy, and Amir H. Hamrahian Copyright © 2015 Kevin M. Pantalone et al. All rights reserved. Megace Mystery: A Case of Central Adrenal Insufficiency Sun, 06 Dec 2015 11:59:17 +0000 http://www.hindawi.com/journals/crie/2015/147265/ Megestrol acetate (MA) is a synthetic progestin with both antineoplastic and orexigenic properties. In addition to its effects on the progesterone receptor, MA also binds the glucocorticoid receptor. Some patients receiving MA therapy have been reported to develop clinical features of glucocorticoid excess, while others have experienced the clinical syndrome of cortisol deficiency—either following withdrawal of MA therapy or during active treatment. We describe a patient who presented with clinical and biochemical features of central adrenal insufficiency. Pituitary function was otherwise essentially normal, and the etiology of the isolated ACTH suppression was initially unclear. The use of an exogenous glucocorticoid was suspected but was initially denied by the patient; ultimately, the culprit medication was uncovered when a synthetic steroid screen revealed the presence of MA. The patient’s symptoms improved after she was switched to hydrocortisone. Clinicians should be aware of the potential effects of MA on the hypothalamic-pituitary-adrenal (HPA) axis. Kunal Mehta, Irene Weiss, and Michael D. Goldberg Copyright © 2015 Kunal Mehta et al. All rights reserved. Thyroid Sporadic Goiter with Adult Heterotopic Bone Formation Tue, 01 Dec 2015 12:41:51 +0000 http://www.hindawi.com/journals/crie/2015/806864/ Thyroid heterotopic bone formation (HBF) in goiter is a rare finding. Five thyroid resection specimens were analyzed for HBF. The results were correlated with clinicomorphological features. All patients were women (33–82 years). The preoperative diagnosis was thyroid goiter or nodule. Treatment consisted in thyroidectomy and lobectomy (3 and 2, resp.). Microscopy showed sporadic nodular goiter. Malformative blood vessels and vascular calcifications were seen in intra- and extrathyroid location (5 and 3, resp.). The number and size of HBFs (total: 28) ranged between 1 and 23/thyroid gland (one bilateral) and 1 and 10 mm, respectively. Twelve HBFs were in contact with the thyroid capsule. Most were extranodular (21, versus 6 intranodular). The medical history was positive for dyslipidemia, hyperglycemia, renal dysfunction, and hyperuricemia (2, 3, and 3 cases and 1 case, resp.) without any parathyroid abnormality. In conclusion, thyroid HBF may be characterized by subcapsular or extranodular location, various size (usually ≥2 mm), and vascular calcifications and malformations. Features of metabolic syndrome and renal dysfunction may be present, but their exact role in the pathogenesis of HBFs remains to be elucidated. Adriana Handra-Luca, Marie-Laure Dumuis-Gimenez, Mouna Bendib, and Panagiotis Anagnostis Copyright © 2015 Adriana Handra-Luca et al. All rights reserved. Congenital Bands with Intestinal Malrotation after Propylthiouracil Exposure in Early Pregnancy Wed, 18 Nov 2015 07:27:13 +0000 http://www.hindawi.com/journals/crie/2015/789762/ Exposure to propylthiouracil in early pregnancy may be associated with an increased risk of birth defects. But the spectrum of associated congenital anomalies is not yet well defined. While preliminary reports suggest that most cases of propylthiouracil-associated birth defects are restricted to the preauricular and urinary systems, careful consideration should be given to other possible manifestations of teratogenicity. We propose that congenital bands may potentially represent a rare yet serious complication of propylthiouracil exposure in early pregnancy, possibly arising from an early mesenteric developmental anomaly. We report a case of a 17-day-old girl that presented with acute small bowel obstruction associated with intestinal malrotation arising from several anomalous congenital bands. Her mother was treated for Graves’ disease during pregnancy with first trimester exposure to propylthiouracil but remained clinically and biochemically euthyroid at conception and throughout the duration of pregnancy. This case suggests that the use of propylthiouracil in early pregnancy may be associated with congenital bands and intestinal malrotation. More reports are needed to further support this association. Alexander A. Leung, Jennifer Yamamoto, Paola Luca, Paul Beaudry, and Julie McKeen Copyright © 2015 Alexander A. Leung et al. All rights reserved. Hypoglycemia Secondary to Sulfonylurea Ingestion in a Patient with End Stage Renal Disease: Results from a 72-Hour Fast Tue, 17 Nov 2015 12:26:51 +0000 http://www.hindawi.com/journals/crie/2015/742781/ Insulin, proinsulin, and C-peptide levels increase with sulfonylurea exposure but the acuity of increase has not been described in dialysis patients. We present a case of a dialysis patient who presented with hypoglycemia and was found to have accidental sulfonylurea ingestion. This is a 73-year-old man with ESRD on peritoneal dialysis, without history of diabetes, who presented with hypoglycemia. Past medical history includes multiple myeloma, congestive heart failure, and hypertension. At initial presentation, his blood glucose was 47 mg/dL, with concomitant elevations in the following: C-peptide 30.5 (nl: 0.8–3.5 ng/mL), insulin 76 (nl: 3–19 μIU/mL), and proinsulin 83.3 (nl: ≤8.0 pmol/L). During the 72-hour fast, which he completed without hypoglycemia, insulin declined to be within normal limits (to 12 μIU/mL); proinsulin (to 12.1 pmol/L) and C-peptide (to 7.2 ng/mL) levels decreased but remained elevated. The sulfonylurea screen ultimately returned positive for glipizide, clinching the diagnosis. This is the first reported case which characterizes the chronic elevation of proinsulin in a patient with ESRD, as well as its dramatic increase after a presumed solitary exposure to sulfonylurea. The 72-hour fast conducted gives insight into the clearance of insulin, proinsulin, and C-peptide after sulfonylurea ingestion in ESRD. Alice Abraham, Mishaela Rubin, Domenico Accili, John P. Bilezikian, and Utpal B. Pajvani Copyright © 2015 Alice Abraham et al. All rights reserved. Rare Skeletal Complications in the Setting of Primary Hyperparathyroidism Tue, 17 Nov 2015 06:25:19 +0000 http://www.hindawi.com/journals/crie/2015/139751/ Parathyroid carcinoma represents an extremely rare neoplasm with diverse clinical manifestations which vary from asymptomatic patients to severe complications of hypercalcemia or parathyrotoxicosis while skeletal involvement is rather common. Herein we aimed at presenting a unique case of a young patient with rare aggressive skeletal complications of parathyroid cancer that initially were misdiagnosed. Ossification of the cervical ligamentum flavum and skull tumor illustrates erosive bonny lesions of hyperparathyroidism that in association with previous medical history of recurrent nephrolithiasis and biochemical findings guide the diagnosis. We suggest that increased awareness and holistic approach are needed in order to recognize and further investigate signs and symptoms of hyperparathyroidism. Nikos Sabanis, Eleni Gavriilaki, Eleni Paschou, Asterios Kalaitzoglou, Dimitrios Papanikolaou, Pinelopi Ioannidou, and Sotirios Vasileiou Copyright © 2015 Nikos Sabanis et al. All rights reserved. Atypical Parathyroid Adenoma Complicated with Protracted Hungry Bone Syndrome after Surgery: A Case Report and Literature Review Thu, 12 Nov 2015 07:03:54 +0000 http://www.hindawi.com/journals/crie/2015/757951/ Hungry Bone Syndrome refers to the severe and prolonged hypocalcemia and hypophosphatemia, following parathyroidectomy in patients with hyperparathyroidism. We present the case of an eighteen-year-old woman with a four-year history of hyporexia, polydipsia, weight loss, growth retardation, and poor academic performance. The diagnostic work-up demonstrated primary hyperparathyroidism with hypercalcemia of 13.36 mg/dL, a PTH level of 2551 pg/mL, bone brown tumors, and microcalcifications within pancreas and kidneys. Neck ultrasonography revealed a parathyroid adenoma of 33 × 14 × 14 mm, also identified on 99Tc-sestamibi scan. Bone densitometry showed decreased -Score values (total lumbar -Score of −4.2). A right hemithyroidectomy and right lower parathyroidectomy were performed. Pathological examination showed an atypical parathyroid adenoma, of 3.8 g of weight and 2.8 cm in diameter. After surgery she developed hypocalcemia with tetany and QTc interval prolongation. The patient required 3 months of oral and intravenous calcium supplementation due to Hungry Bone Syndrome (HBS). After 42 months, she is still under oral calcium. Usually HBS lasts less than 12 months. Therefore we propose the term “Protracted HBS” in patients with particularly long recovery of 1 year. We present a literature review of the diagnosis, pathophysiology, and treatment of HBS. Óscar Alfredo Juárez-León, Miguel Ángel Gómez-Sámano, Daniel Cuevas-Ramos, Paloma Almeda-Valdés, Manuel Alejandro López-Flores A La Torre, Alfredo Adolfo Reza-Albarrán, and Francisco Javier Gómez-Pérez Copyright © 2015 Óscar Alfredo Juárez-León et al. All rights reserved. Adrenal Lymphangioma Masquerading as a Catecholamine Producing Tumor Thu, 05 Nov 2015 09:54:02 +0000 http://www.hindawi.com/journals/crie/2015/380151/ Objective. To report the unusual case of an adrenal lymphangioma presenting in a patient with an adrenal cystic lesion and biochemical testing concerning for pheochromocytoma. The pertinent diagnostic and imaging features of adrenal lymphangiomas are reviewed. Methods. We describe a 59-year-old patient who presented with hyperhidrosis and a 2.2 by 2.2 cm left adrenal nodule. Biochemical evaluation revealed elevated plasma-free normetanephrine, urine normetanephrine, urine vanillylmandelic acid, and urine norepinephrine levels. Elevated plasma norepinephrine levels were not suppressed appropriately with clonidine administration. Results. Given persistent concern for pheochromocytoma, the patient underwent adrenalectomy. The final pathology was consistent with adrenal lymphangioma. Conclusions. Lymphangiomas are benign vascular lesions that can very rarely occur in the adrenal gland. Imaging findings are generally consistent with a cyst but are nonspecific. Excluding malignancy in patients presenting with adrenal cysts can be difficult. Despite its benign nature, the diagnosis of adrenal lymphangioma may ultimately require pathology. Israel Hodish, Lindsay Schmidt, and Andreas G. Moraitis Copyright © 2015 Israel Hodish et al. All rights reserved. Weekly Intramuscular Injection of Levothyroxine following Myxoedema: A Practical Solution to an Old Crisis Thu, 05 Nov 2015 06:39:16 +0000 http://www.hindawi.com/journals/crie/2015/169194/ An 82-year-old female with known hypothyroidism was admitted to hospital after being found on the floor. On examination, she was unkempt, confused, bradycardic, hypothermic, and barely arousable. Initial biochemistry revealed a thyroid stimulating hormone (TSH) of >100 mU/L and free thyroxine (FT4) level of 1.5 pmol/L which supported a diagnosis of myxoedema coma. She was resuscitated and commenced on liothyronine, levothyroxine, and hydrocortisone and some improvement was made. It became apparent that she was hiding and spitting out her oral levothyroxine including levothyroxine elixir. Given the need for prompt alternative control, we sought advice from international experts where intramuscular levothyroxine was recommended. She was managed from day 50 onwards with intramuscular levothyroxine 200 mcg once a week, which was subsequently increased to 500 mcg. Thyroid function normalized and she made continual cognitive and physical progress and was discharged to a rehabilitation hospital. Her intramuscular levothyroxine was stopped and she was subsequently restarted on oral levothyroxine, with a plan for on-going close monitoring of her thyroid function. This report highlights the potential to use intramuscular levothyroxine in individuals with severe hypothyroidism arising from poor compliance with levothyroxine treatment or other potential causes such as impaired absorption. Peter N. Taylor, Arshiya Tabasum, Gina Sanki, David Burberry, Brian P. Tennant, James White, Onyebuchi Okosieme, Andrew Aldridge, and Gautam Das Copyright © 2015 Peter N. Taylor et al. All rights reserved. Primary Adrenal Failure due to Antiphospholipid Syndrome Sun, 25 Oct 2015 11:16:39 +0000 http://www.hindawi.com/journals/crie/2015/161497/ Background. Antiphospholipid syndrome (APS) characterized by thrombosis and abortus may rarely cause primary adrenal failure. Case Presentations. A 34-year-old male presented with hypotension, hypoglycemia, hyperpigmentation on his skin and oral mucosa, scars on both legs, and loss of consciousness. In laboratory examinations, hyponatremia (135 mmol/L), hyperpotassemia (6 mmol/L), and thrombocytopenia (83 K/µL) were determined. Cortisol (1.91 µg/dL) and adrenocorticotropic (550 pg/mL) hormone levels were also evaluated. The patient was hospitalized with a diagnosis of acute adrenal crisis due to primary adrenal insufficiency. A Doppler ultrasound revealed venous thrombosis. The patient was diagnosed with antiphospholipid syndrome after the detection of venous thrombosis, thrombocytopenia, elevated aPTT, and anticardiolipin antibody levels. Anticoagulation treatment was started for antiphospholipid syndrome. The patient is now following up with hydrocortisone, fludrocortisone, and warfarin sodium. Conclusion. Antiphospholipid syndrome is a rare reason for adrenal failure. Antiphospholipid syndrome should be suspected if patients have morbidity secondary to venous-arterial thrombosis. Murat Sahin, Ayten Oguz, Dilek Tuzun, Serife Nur Boysan, Bülent Mese, Hatice Sahin, and Kamile Gul Copyright © 2015 Murat Sahin et al. All rights reserved. Nonclassical Congenital Adrenal Hyperplasia and Pregnancy Thu, 08 Oct 2015 09:43:26 +0000 http://www.hindawi.com/journals/crie/2015/296924/ Objective. The most common form of congenital adrenal hyperplasia (CAH) is 21-hydroxylase (21-OH) deficiency due to mutation of the CYP21A2 gene. Patients with nonclassical CAH (NC-CAH) are usually asymptomatic at birth and typically present in late childhood, adolescence, or adulthood with symptoms of excessive androgen secretion. Subfertility is relative in NC-CAH, but the incidence of spontaneous miscarriage is higher. Here, we report a previously undiagnosed female who gave birth to a normal male child and is planning to become pregnant again. Case Report. A 32-year-old female was referred to our clinic for obesity. Her medical history revealed that she had had three pregnancies. She was planning to become pregnant again. Her laboratory results revealed that she had NC-CAH. Since her husband is the son of her aunt and she had miscarriages and intrauterin exitus in her history, their genetic analyses were performed. Conclusion. Since most patients with NC-CAH have a severe mutation, these patients may give birth to a child with the classical CAH (C-CAH) if their partner is also carrying a severe mutation. Females with NC-CAH who desire pregnancy must be aware of the risk of having an infant with C-CAH. Neslihan Cuhaci, Cevdet Aydın, Ahmet Yesilyurt, Ferda Alpaslan Pınarlı, Reyhan Ersoy, and Bekir Cakir Copyright © 2015 Neslihan Cuhaci et al. All rights reserved. Management of Refractory Noninsulinoma Pancreatogenous Hypoglycemia Syndrome with Gastric Bypass Reversal: A Case Report and Review of the Literature Wed, 07 Oct 2015 11:19:08 +0000 http://www.hindawi.com/journals/crie/2015/384526/ Background. Roux-en-Y gastric bypass (RYGB) is a commonly performed, effective bariatric procedure; however, rarely, complications such as postprandial hypoglycemia due to noninsulinoma pancreatogenous hypoglycemia syndrome (NIPHS) may ensue. Management of refractory NIPHS is challenging. We report a case that was successfully treated with RYGB reversal. Case Report. A 58-year-old male with history of RYGB nine months earlier for morbid obesity presented for evaluation of postprandial, hypoglycemic seizures. Testing for insulin level, insulin antibodies, oral hypoglycemic agents, pituitary axis hormone levels, and cortisol stimulation was unrevealing. Computed tomography (CT) scan of the abdomen was unremarkable. A 72-hour fast was completed without hypoglycemia. Mixed meal testing demonstrated endogenous hyperinsulinemic hypoglycemia (EHH) and selective arterial calcium stimulation testing (SACST) was positive. Strict dietary modifications, maximal medical therapy, gastrostomy tube feeding, and stomal reduction failed to alleviate symptoms. Ultimately, he underwent laparoscopic reversal of RYGB. Now, 9 months after reversal, he has markedly reduced hypoglycemia burden. Discussion. Hyperfunctioning islets secondary to exaggerated incretin response and altered intestinal nutrient delivery are hypothesized to be causative in NIPHS. For refractory cases, there is increasing skepticism about the safety and efficacy of pancreatic resection. RYGB reversal may be successful. Bhavana B. Rao, Benjamin Click, George Eid, and Ronald A. Codario Copyright © 2015 Bhavana B. Rao et al. All rights reserved.