Psychedelics: Threshold of a Therapeutic Revolution.

This Special Issue of Neuropharmacology on psychedelics provides a timely and comprehensive update on progress following the previous Neuropharmacology Special Issue "Psychedelics: New Doors, Altered Perceptions". Remarkable advances have been made in basic and clinical research on psychedelics in the five years since 2018. It is partly based on the seminar series focused on psilocybin organized by the National Institutes of Health (NIH), USA from April to June 2021, the "NIH Psilocybin Research Speaker Series".

National Institutes of Health psilocybin research speaker series: State of the science, regulatory and policy landscape, research gaps, and opportunities.

The U.S. National Institutes of Health (NIH) convened a seminal first ever psychedelic drug substance-focused speaker series, from April 22 to June 10, 2021, titled the "NIH Psilocybin Research Speaker Series.

Policy considerations that support equitable access to responsible, accountable, safe, and ethical uses of psychedelic medicines.

There is mounting evidence suggesting psychedelic and entactogen medicines (namely psilocybin and 3,4-methylenedioxymethamphetamine [MDMA]), in conjunction with proper psychosocial support, hold the potential to provide safe, rapid acting, and robust clinical improvements with durable effects. In the US, both psilocybin and MDMA have been granted Breakthrough Therapy designations by the US Food and Drug Administration and may potentially receive full FDA approval with similar regulatory considerations occurring in multiple countries. At the same time, regulatory changes are poised to increase access to legal or decriminalized psychedelic use in various non-medical settings.

Predicting the risk of cardiac myxoma in Carney complex.

Purpose: Carney complex (CNC), is an autosomal dominant multiple neoplasia and lentiginosis syndrome. We aimed to identify risk factors associated with the occurrence and recurrence of cardiac myxomas, the predominant cause of death in CNC patients.

Methods: Patients with CNC were monitored prospectively between 1995 and 2020 for the development of cardiac myxomas.

PRKAR1A deficiency impedes hypertrophy and reduces heart size.

Protein kinase A (PKA) activity is pivotal for proper functioning of the human heart, and its dysregulation has been implicated in a variety of cardiac pathologies. PKA regulatory subunit 1α (R1α, encoded by the PRKAR1A gene) is highly expressed in the heart, and controls PKA kinase activity by sequestering PKA catalytic subunits. Patients with PRKAR1A mutations are often diagnosed with Carney complex (CNC) in early adulthood, and may die later in life from cardiac complications such as heart failure.

Aggressive pituitary tumors in the young and elderly.

Aggressive pituitary tumors (APTs) represent rare pituitary adenomas (PAs) with local invasion of surrounding tissues, increased risk for multiple recurrence, rapid tumor growth, or resistance to standard therapies. The most common APTs in children and adolescents are giant prolactinomas and somatotropinomas. Few cases of Crooke's cell adenomas, silent corticotroph adenomas and pituitary carcinomas have also been reported in the literature.

Pediatric Cushing Syndrome; an Overview.

Endogenous Cushing syndrome (CS) is a rare entity in the pediatric population. Patients usually present with height deceleration and continuous weight gain, along with other classic stigmata of CS, such as acne, striae, hirsutism and others. The diagnosis of CS involves documentation of abnormal cortisol secretion with measurement of urinary free cortisol and midnight serum or salivary cortisol, and the use of the 1mg dexamethasone suppression test.

An update on adrenal endocrinology: significant discoveries in the last 10 years and where the field is heading in the next decade.

The last 10 years have produced an amazing number of significant discoveries in the field of adrenal endocrinology. The development of the adrenal gland was linked to specific molecules. Cortisol-producing lesions were associated mostly with defects of the cyclic AMP (cAMP) signaling pathway, whereas aldosterone-producing lesions were found to be the result of defects in aldosterone biosynthesis or the potassium channel KCNJ5 and related molecules.

Exploring Expressions of Depression in Black Single Mothers.

Problem: This study investigates the experience of depression from the perspective of Black single mothers, an understudied diverse-sub-group who consistently report high levels of depressive symptoms that go undetected and untreated.

Participants: The sample consisted of 210 Black single mothers aged 18-45 who reside in urban communities.

Methods: A descriptive, cross-sectional design was used to categorize the responses of Black single mothers to an open-ended question that asks about feelings of depression according to the four domains of the Centers for Epidemiologic Studies Depression Scale.

Cushing's Syndrome in Pediatrics: An Update.

Cushing syndrome (CS) is a multisystem disorder resulting from the prolonged exposure to excess glucocorticoids. In children, CS most commonly results from the exogenous administration of steroids and the typical presentation is height deceleration concomitant with weight gain. Endogenous and ectopic causes are rare.

Neonatal Cushing Syndrome: A Rare but Potentially Devastating Disease.

Neonatal Cushing syndrome (CS) is most commonly caused by exogenous administration of glucocorticoids and rarely by endogenous hypercortisolemia. CS owing to adrenal lesions is the most common cause of endogenous CS in neonates and infants, and adrenocortical tumors (ACTs) represent most cases. Many ACTs develop in the context of a TP53 gene mutation, which causes Li-Fraumeni syndrome.

The ARMC5 gene shows extensive genetic variance in primary macronodular adrenocortical hyperplasia.

Objective: Primary macronodular adrenal hyperplasia (PMAH) is a rare type of Cushing's syndrome (CS) that results in increased cortisol production and bilateral enlargement of the adrenal glands. Recent work showed that the disease may be caused by germline and somatic mutations in the ARMC5 gene, a likely tumor suppressor gene (TSG). We investigated 20 different adrenal nodules from one patient with PMAH for ARMC5 somatic sequence changes.

Carney complex: an update.

Carney complex (CNC) is a rare autosomal dominant syndrome, characterized by pigmented lesions of the skin and mucosa, cardiac, cutaneous and other myxomas and multiple endocrine tumors. The disease is caused by inactivating mutations or large deletions of the PRKAR1A gene located at 17q22-24 coding for the regulatory subunit type I alpha of protein kinase A (PKA) gene. Most recently, components of the complex have been associated with defects of other PKA subunits, such as the catalytic subunits PRKACA (adrenal hyperplasia) and PRKACB (pigmented spots, myxomas, pituitary adenomas).

Phosphodiesterase 8B and cyclic AMP signaling in the adrenal cortex.

Bilateral adrenocortical hyperplasia (BAH) in humans and mice has been recently linked to phosphodiesterase (PDE) 8B (PDE8B) and 11 (PDE11A) defects. These findings have followed the discovery that defects of primary genes of the cyclic monophosphatase (cAMP) signaling pathway, such as guanine nucleotide binding alpha subunit and PRKAR1A, are involved in the pathogenesis of BAH in humans; complete absence of Prkar1a in the adrenal cortex of mice also led to pathology that mimicked the human disease. Here, we review the most recent findings in human and mouse studies on PDE8B, a cAMP-specific PDE that appears to be highly expressed in the adrenal cortex and whose deficiency may underlie predisposition to BAH and possibly other human diseases.

X-linked acrogigantism syndrome: clinical profile and therapeutic responses.

X-linked acrogigantism (X-LAG) is a new syndrome of pituitary gigantism, caused by microduplications on chromosome Xq26.3, encompassing the gene GPR101, which is highly upregulated in pituitary tumors. We conducted this study to explore the clinical, radiological, and hormonal phenotype and responses to therapy in patients with X-LAG syndrome.

Is IGSF1 involved in human pituitary tumor formation?

IGSF1 is a membrane glycoprotein highly expressed in the anterior pituitary. Pathogenic mutations in the IGSF1 gene (on Xq26.2) are associated with X-linked central hypothyroidism and testicular enlargement in males.

The Clinical Center's blood glucose management service: a story in quality integrated care.

Purpose: Blood glucose management in the inpatient setting has been shown to be crucial to patient outcomes. As the evidence develops to determine best clinical practices for achieving inpatient glycemic goals, the Clinical Center at the National Institutes of Health has implemented a streamlined multidisciplinary approach to managing blood glucose levels for hospitalized patients. The purpose of this article is to describe the blood glucose management service at the Clinical Center.