ABSTRACT
Severe hyperthyroidism can cause the injuries of multiple organs including heart and liver and ultimately be fatal. A 26-year-old young woman admitted to Peking Union Medical College Hospital for severe hyperthyroidism due to irregular use of anti-thyroid drugs. She had heart failure,atrial fibrillation,and severe liver damage at admission. Anti-thyroid drugs were then actively used to treat the primary disease,along with interventions to correct heart failure and control atrial fibrillation. Severe total bilirubin elevation was found during the treatment, which was resolved after the use of glucocorticoid and liver-protective therapy. The patient was regularly followed up after discharge,and the clinical manifestations were good.
Subject(s)
Atrial Fibrillation , Heart Failure , Hyperthyroidism , Liver Diseases , Adult , Female , HumansABSTRACT
OBJECTIVE: Wolfram syndrome (WS) is a rare, degenerative, and hereditary disorder characterized by ear diabetes mellitus (DM) and optic atrophy (OA). We aim to characterize clinical features in Chinese patients who had been poorly studied until now. METHODS: We performed a retrospective review of patients with WS seen in the Peking Union Medical College Hospital from 2002 to 2017. Data including demographic data, clinical presentations, examination results, family history, and genetic analysis were described. RESULTS: Six patients with WS were identified, meeting the diagnostic criteria of the coincidence of DM and OA before 15 years old or the existence of two WFS1 mutations. All were male, with the median age of 14.5 years (range 10-19 years). Blood glucose impairment, OA, and diabetes insipidus were present in all (100%), hearing impairment in four (66.7%), urological abnormalities in four (66.7%), neurological abnormalities in one (16.7%), and endocrine disorder in one (16.7%). Rare presentation includes cataract, glaucoma, and spina bifida occulta. Diabetes was insulin-dependent and not ketosis onset, with antibody to glutamic acid decarboxylase and islet cell negative. Genetic analysis revealed mutations in WFS1 in three patients. A novel frameshift mutation (p.Asp151Glufs*93) was identified in exon 4 of WFS1. CONCLUSION: Our series of WS patients indicated that WS is a degenerative disease with a wide and variable spectrum, characterized by ear non-autoimmune DM and bilateral OA. Genetic analysis is recommended when suspected of WS.
ABSTRACT
OBJECTIVE: To explore the relationship between maternal milk and serum thyroid hormones in patients with thyroid-related diseases. METHODS: Serum and breast milk samples were collected from 56 breastfeeding mothers. Milk and serum free triiodothyronine (FT3), free thyroxine (FT4), triiodothyronine(T3), thyroxine (T4), and thyrotrophin (TSH) were determined, and T3/T4 was calculated. Using the serum thyroid hormones as the independent variables and milk thyroid hormones as the dependent variables, we performed linear regression analysis. RESULTS: The milk FT3, FT4, T3, T4, TSH, and T3/T4 were (2.30 ± 0.82) pg/ml ,(0.45 ± 0.26) ng/dl, (0.35 ± 0.20) ng/ml, (2.96 ± 1.55) Μg/dl, (0.12 ± 0.08) ΜU/ml, and 0.12 ± 0.04, respectively. Milk FT3 (r = 0.778, P = 0.000), T3 (r = 0.603, P = 0.000), T4 (r = 0.485, P = 0.004), and TSH (r = 0.605, P = 0.000) concentrations were positively correlated with those in serum. CONCLUSION: Thyroid hormones are present in human milk and are positively correlated with those in serum.
