Unusual Thyroid Mass in an Adolescent Patient.

Hurthle cell tumors are rare follicular-derived thyroid neoplasms. Hurthle cell tumors may be benign or malignant. Workup includes imaging, fine needle aspiration, and treatment usually consists of observation versus thyroidectomy. We describe a case of Hurthle cell adenoma in an adolescent; to the best of our knowledge, this represents only the third case described in the English literature of adolescent Hurthle cell adenoma.

Group VIA Phospholipase A2 (iPLA2ß) Modulates Bcl-x 5'-Splice Site Selection and Suppresses Anti-apoptotic Bcl-x(L) in ß-Cells.

Diabetes is a consequence of reduced ß-cell function and mass, due to ß-cell apoptosis. Endoplasmic reticulum (ER) stress is induced during ß-cell apoptosis due to various stimuli, and our work indicates that group VIA phospholipase A2ß (iPLA2ß) participates in this process. Delineation of underlying mechanism(s) reveals that ER stress reduces the anti-apoptotic Bcl-x(L) protein in INS-1 cells. The Bcl-x pre-mRNA undergoes alternative pre-mRNA splicing to generate Bcl-x(L) or Bcl-x(S) mature mRNA. We show that both thapsigargin-induced and spontaneous ER stress are associated with reductions in the ratio of Bcl-x(L)/Bcl-x(S) mRNA in INS-1 and islet ß-cells. However, chemical inactivation or knockdown of iPLA2ß augments the Bcl-x(L)/Bcl-x(S) ratio. Furthermore, the ratio is lower in islets from islet-specific RIP-iPLA2ß transgenic mice, whereas islets from global iPLA2ß(-/-) mice exhibit the opposite phenotype. In view of our earlier reports that iPLA2ß induces ceramide accumulation through neutral sphingomyelinase 2 and that ceramides shift the Bcl-x 5'-splice site (5'SS) selection in favor of Bcl-x(S), we investigated the potential link between Bcl-x splicing and the iPLA2ß/ceramide axis. Exogenous C6-ceramide did not alter Bcl-x 5'SS selection in INS-1 cells, and neutral sphingomyelinase 2 inactivation only partially prevented the ER stress-induced shift in Bcl-x splicing. In contrast, 5(S)-hydroxytetraenoic acid augmented the ratio of Bcl-x(L)/Bcl-x(S) by 15.5-fold. Taken together, these data indicate that ß-cell apoptosis is, in part, attributable to the modulation of 5'SS selection in the Bcl-x pre-mRNA by bioactive lipids modulated by iPLA2ß.

Ultrasound characteristics of the thyroid in children and adolescents with goiter: a single center experience.

INTRODUCTION: Autoimmune thyroiditis (AIT) is a common cause of goiter in children, and sonographic changes have been reported in more than one-third at presentation. The aim of this study was to evaluate the ultrasound (US) characteristics of the thyroid and the prevalence of thyroid nodules in children and adolescents presenting with goiter in the presence or absence of AIT. METHODS: A retrospective review was conducted of the US characteristics of 154 children and adolescents aged <18 years of age with goiter from July 2008 to December 2010. US characteristics were analyzed according to each patient's age, sex, thyrotropin (TSH) levels, and thyroid peroxidase antibody titer (TPOAb). Heterogeneity and nodule prevalence were compared between antibody-positive and -negative goiter. RESULTS: Heterogeneity was more common in TPOAb-positive (59/71, 83%) compared to TPOAb-negative goiter (24/46, 52%; p<0.001), but there was no correlation between the presence of heterogeneity and TPOAb titer within the antibody-positive group. Nodules were equally prevalent in children with (17%) and without (17.4%) TPOAb, and there was no correlation between the serum TSH level or TPOAb titer and the presence of nodules. Papillary thyroid cancer (PTC) was diagnosed in 3/71 with positive TPOAb compared to 1/46 with negative antibodies. Pseudonodules were identified in 11/71 antibody-positive and none of the antibody-negative patients. However, during follow-up, two of these were later identified as nodules and one was PTC. CONCLUSION: The majority of children and adolescents with goiter had positive TPOAb (71/117). Sonographic heterogeneity was more common among TPOAb-positive patients. However, thyroid nodules and PTC were equally common in both groups. Only 15% of the nodules and none of the PTC were palpable. These data support the utility of thyroid US to detect unsuspected thyroid nodules and PTC in children with goiter. Prospective follow-up studies of children with goiter are needed to formulate recommendations for evaluation with US and fine-needle aspiration.

Fetal right-ventricular hypertrophy with pericardial effusion and maternal untreated hyperthyroidism.

Uncontrolled/untreated maternal hyperthyroidism has been associated with fetal tachycardia. We report a case of right-ventricular (RV) hypertrophy with pericardial effusion related to untreated maternal Graves' disease. A 33-year-old G4P1021 woman with uncontrolled Graves' disease presented at 29 weeks gestation with abdominal pain and vaginal bleeding. Fetal echocardiogram showed severe RV hypertrophy and a pericardial effusion. The infant was born prematurely, and initial transthoracic echocardiogram showed severe RV hypertrophy and a small pericardial effusion. The infant had clinical findings consistent with congenital thyrotoxicosis and was treated for this. Follow-up imaging at 4 weeks showed improvement of the cardiac hypertrophy and pericardial effusion. This article describes the presentation of fetal RV hypertrophy with congenital thyrotoxicosis and underscores the importance of screening for this prenatally in mothers with uncontrolled or untreated hyperthyroidism.

Staphylococcus epidermidis Urinary Tract Infection in an Infant.

We describe the case of a previously healthy 7-month-old male infant with urinary tract infection due to Staphylococcus epidermidis grown from two separate urine cultures. Further evaluation showed severe bilateral vesicoureteral reflux. Physicians should not assume that S. epidermidis is always a contaminant in urine cultures.

Safety of intravenous terbutaline in acute severe asthma: a retrospective study.

OBJECTIVE: (1) To determine the effect of intravenous terbutaline in children with acute severe asthma on parameters like heart rate, blood pressure, electrocardiogram and serum electrolytes; (2) to assess the safety profile and to evaluate the outcome of children treated with intravenous terbutaline for acute severe asthma. DESIGN: Retrospective study of admission records of children admitted with acute severe asthma who needed intravenous terbutaline. SETTING: Children's Hospital at the Leicester Royal Infirmary, UK. PATIENTS: 77 children with acute severe asthma admitted between April 1999 and October 2002. RESULTS: There was a significant increase in heart rate and significant fall in diastolic blood pressure in this cohort. Four patients required inotropic support. None of the patients had cardiac arrhythmias. Potassium supplements were required in 10 patients due to hypokalaemia. All patients improved and none required initiation of ventilation after commencing terbutaline. There was no mortality in this cohort. CONCLUSIONS: Terbutaline was found to be safe for use in this patient group in doses ranging between 1 and 5 microg/kg/min. Intravenous terbutaline was found to be a useful adjunct in those who failed to respond to standard initial therapy.