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Context: The prevalence of unilateral primary aldosteronism (UPA) with cortisol co-secretion varies geographically. Objective: To investigate the prevalence and clinical characteristics of UPA with cortisol co-secretion in a Chinese population. Design: Retrospective cohort study. Methods: We recruited 580 patients with UPA who underwent cosyntropin stimulation test (CST) after the 1-mg dexamethasone suppression test (DST) and retrospectively analyzed the clinical characteristics and postoperative outcomes of UPA with and without cortisol co-secretion. Results: UPA with cortisol co-secretion (1 mg DST>1.8 ug/dL) was identified in 65 of 580 (11.2%) patients. These patients were characterized by older age, longer duration of hypertension, higher concentration of plasma aldosterone and midnight cortisol, lower adrenocorticotropic hormone (ACTH) and dehydroepiandrosterone sulfate (DHEAS), larger tumor diameter, and more history of diabetes mellitus. Cortisol and aldosterone levels were higher and DHEAS level was lower in UPA with cortisol co-secretion at 0--120 min after CST. Among 342 UPA patients with KCNJ5 gene sequencing and follow-up results, the complete clinical success rate was lower in UPA with cortisol co-secretion (33.3% vs. 56.4%, P<0.05); the complete biochemical success rate and KCNJ5 mutation did not differ between the two groups. Age, tumor size, and ACTH were independent predictors of UPA with cortisol co-secretion. Sex, BMI, duration of hypertension, KCNJ5 mutation, and cortisol co-secretion were independent predictors for complete clinical success in UPA after surgery. Conclusions: UPA with cortisol co-secretion is not uncommon in China, but the clinical features were distinctly different from those without co-secretion. Cortisol co-secretion is an independent risk factor for incomplete clinical success after surgery in UPA. [ABSTRACT FROM AUTHOR]

Familial glucocorticoid deficiency (FGD) is a rare autosomal recessive disease characterized by single cortisol deficiency but normal aldosterone and renin levels. Beginning from the discovery of the disease to that of the pathogenic genes over a period of 30 years, the development of gene detection technology has identified a large number of FGD-related genes. Despite the fact that the genetic defect underlying this disease is known for approximately 70% of the patients diagnosed with FGD, there are still several unknown factors causing it. FGD is divided into type 1, type 2 and non-classical type according to the mutant gene. The case described in the present study reported two patients, who were siblings, having skin hyperpigmentation and undergone treatment in adulthood. The gonadal development was normal and the proband had a 10-year-old son. Laboratory tests suggested glucocorticoid deficiency and a mild lack of mineralocorticoid, indicating hyponatremia and hypotension in the proband. In addition, cortisol deficiency was not affected by adrenocorticotropic hormone treatment, while the adrenal glands in the two patients did not show any hyperplasia. Gene analysis revealed two compound heterozygote mutations c.533T>A (p. Leu178Gln) and c.737A>G (p. Asp246Gly) in the steroid hormone acute regulatory protein (STAR) gene in both patients, which may have been obtained from their parents and the proband passed one of the mutations to her son. The present study results revealed that STAR mutations cause non-classic congenital lipoid adrenal hyperplasia in China. [ABSTRACT FROM AUTHOR]

Familial glucocorticoid deficiency type 1 (FGD1) is an autosomal recessive disorder caused by mutations in the melanocortin 2 receptor (MC2R) gene, characterized by a low or undetectable serum cortisol level and a high adrenocorticotropic hormone (ACTH) level. Clinical manifestations include hypoglycemia, seizure, skin hyperpigmentation, hyperbilirubinemia, cholestasis, and a tall stature. Some dysmorphic features such as, a prominent forehead, hypertelorism, a broad nasal bridge, and small tapering fingers, have been reported. Children with FGD1 may have other isolated endocrine abnormalities. To date, no patient with FGD1 has been reported in mainland China. Here we report on a Chinese patient with FGD1 having a novel MC2R gene variant, a mild transverse palm crease, hypertelorism, and subtle/transient endocrine abnormalities relating to all three zones of the adrenal cortex and thyroid gland. We also reviewed cases with dysmorphic features or additional endocrine abnormalities. [ABSTRACT FROM AUTHOR]

Simple Summary: China produces the largest number of meat-producing geese in the world. However, few studies have investigated the effects of electrical stunning parameters on stunning stress or meat lipid oxidation in geese. We aimed to evaluate the stunning stress and meat lipid oxidation in geese stunned at a high electrical frequency level with different electrical current intensities in a water bath. Stunning the geese at 40 mA resulted in the minimum stunning stress based on the gene expression and hormones released in the hypothalamus–pituitary–adrenal axis. Stunning geese with 40 mA resulted in low stunning stress and lipid oxidation in breast meat (d 2), with moderate antioxidant capacity in the meat of the breast (d 2) and thigh (d 0) as compared with 100 mA. A combination of 40 mA, 500 Hz, 10 s per bird is suitable for the electrical stunning of geese and for the alleviation of stunning stress and meat lipid oxidation. This study may help to alleviate stunning stress and improve meat quality in the geese production industry. Intensive slaughtering with electrical stunning (ES) is replacing traditional manual slaughtering of geese in China. This study aimed to assess stunning stress and meat lipid oxidation in geese stunned by high-frequency current intensities. Forty male Yangzhou geese, 92 days old, were randomly allocated into five treatments with eight replicates per treatment. The geese in the control group were not stunned, while the other birds were stunned by alternating current (AC) in a water bath. Each bird received a current intensity of 20 mA (E20mA), 40 mA (E40mA), 70 mA (E70mA), or 100 mA (E100mA) for 10 s at 500 Hz. The gene expression of c-jun N-terminal kinase 1 tended to decrease in the E40mA birds (p = 0.08). Stunning with 40 mA resulted in the maximum serum uric acid and urea among the ES groups and decreased serum adrenocorticotropin and creatine kinase (p < 0.01) compared with 70 mA and 100 mA. Increasing the current intensity reduced the diphenylpicrylhydrazyl free radical elimination ability and total-superoxide dismutase linearly in goose breast meat at d 2 and in thigh meat at d 0 (all p < 0.01). Stunning geese with 40 mA at 500 Hz for 10 s could alleviate stunning stress and meat lipid oxidation. [ABSTRACT FROM AUTHOR]