Iron, genes, and viruses: the porphyria cutanea tarda triple threat.

Porphyria cutanea tarda (PCT) is a vesiculobulIous disorder often associated with estrogens, hepatitis C virus (HCV), alcoholism, hereditary hemochromatosis (HH), and human immunodeficiency virus. Hepcidin, a peptide hormone produced by the liver, has been associated with iron metabolism in 3 common precipitating factors for PCT: HCV, HH, and alcohol consumption. We present the case of a patient with erosions and noninflammatory bullae on his hands and forearms who received a diagnosis of PCT. On further examination, the patient was found to be positive for 3 precipitating factors: HCV, an HH gene mutation, and alcohol use. For patients with PCT, it is important to perform phenotypic screening for HCV and HH. Targeting hepcidin with replacement therapy to decrease iron may be a treatment of not only HCV, HH, and alcoholic cirrhosis, but also PCT.

Thyroid function and autoantibody status among women who spontaneously deliver under 35 weeks of gestation.

OBJECTIVE: Recent studies report an association between maternal thyroid auto-antibodies and preterm birth. None have made the important distinction between spontaneous and iatrogenic preterm birth. We investigated the association between spontaneous preterm birth before 35-week gestation and both thyroid function and auto-antibody status. DESIGN: Retrospective nested case-control study, samples retrieved from a biobank of around 8000 samples. PATIENTS: Forty-six cases of spontaneous preterm birth <35-weeks gestation compared with 124 cases who delivered at 40 weeks gestation, matched for maternal age and duration of serum storage. All resulted in a singleton liveborn. MEASUREMENTS: First trimester serum levels of TSH, free T4, free T3, anti-thyroid peroxidase (anti-TPO) and anti-thyroglobulin (anti-Tg) antibodies. RESULTS: Women who had a preterm birth delivered at a median gestational age of 33 (+1 day) weeks gestation (controls 40 + 3 weeks) and had significantly lower birthweight infants [mean (range) weight 1965 (581-3117) grams compared with controls 3526 (2685-4760) grams]. Median levels of anti-thyroglobulin and anti-TPO antibodies were similar in the two groups (Anti-Tg: 3 IU/ml (Range 1-316) v 3 IU/ml (1-691); Anti-TPO 8 IU/ml (3-73) v 9 IU/ml (3-499); P >/= 0.18 for both comparisons, compared using three statistical models). In the preterm group, the median TSH level was 0.70 mIU/l (range 0.04-3.84), similar to controls (0.88 (0.01-2.87), P = 0.1). Free T3 and T4 levels were also similar in both groups. CONCLUSIONS: Thyroid function and thyroid autoantibody status among women who have a spontaneous preterm delivery before 35 weeks gestation are not different from women delivering at term.

Disease knowledge, perceived risk, and health behavior engagement among adolescents and adults with congenital heart disease.

OBJECTIVE: Survivors of congenital heart disease (CHD) are at risk for life-threatening complications as they age. This study aimed to examine the association of knowledge of future health risks, perceived risk, and health behaviors among adolescents and adults with CHD. METHODS: CHD survivors (N = 200, ages 15-39; 23% simple, 44% moderate, 33% complex lesions) completed measures of risk knowledge accuracy and perceived risk for developing complications, and reported physical activity and saturated fat intake. RESULTS: CHD survivors reported poor risk knowledge and consuming high-fat diets. Adolescents reported more physical activity than young adults. Greater risk knowledge was associated with lower fat intake, and participants who exercised more expected fewer future complications, and this difference remained statistically significant when accounting for education and age. CONCLUSIONS: CHD survivors, regardless of age, have poor risk knowledge and diets. Survivors may benefit from emphasis on future health risks and health behaviors from both pediatric and adult providers.