Puberty in girls with Prader-Willi syndrome: cohort evaluation and clinical recommendations in a Latin American tertiary center.

Introduction: Prader-Willi syndrome (PWS) is a genetic disorder characterized by hypothalamic-pituitary deficiencies including hypogonadism. In girls with PWS, hypogonadism can present early in childhood, leading to genital hypoplasia, delayed puberty, incomplete pubertal development, and infertility. In contrast, girls can present with premature activation of the adrenal axis leading to early pubarche and advanced bone age.

SGA-born adults with postnatal catch-up have a persistently unfavourable metabolic health profile and increased adiposity at age 32 years.

Background: Catch-up in weight-for-length in the first year of life results in more insulin resistance, an adverse lipid profile and more fat mass (FM) in 21-year-old adults born small for gestational age (SGA-CU) compared to peers born SGA without catch-up and those born appropriate for gestational age (AGA). The aim of present study was to investigate if the adverse metabolic health profile in the SGA-CU group would worsen or remain stable over the years and to determine the cardiometabolic health at 32 years between the SGA and AGA groups.

Methods: We longitudinally investigated 287 adults, 170 SGA with catch-up growth (SGA-CU) or persistent short stature (SGA-S) and 117 AGA at ages 21 and 32 years.

A 12-Day-Old Boy with Methemoglobinemia After Circumcision with Local Anesthesia (Lidocaine/Prilocaine).

A 12-day-old boy presented with duskiness 4 h after circumcision with local anesthesia: infiltration with lidocaine (6 mL 1 %) and topical EMLA cream (2.5 % lidocaine/2.5 % prilocaine).