Metyrapone as treatment in the neonatal McCune-Albright syndrome.

Objectives To present a case report of succesfully metyrapone treatment of a neonatal patient with McCune-Albrigth syndrome (MAS), a rare disease caused by a genetically mosaic disorder and is characterized by variable hyperfunctional endocrinopathies, bone dysplasia, and café-au-lait spots. Case presentation A preterm newborn was admitted to hospital and she presented difficulty controlling hypertension, café-au-lait spots, and failure to thrive. An abdominal ultrasound and a magnetic resonance showed a high volume of both suprarenal glands. Therefore, MAS was suspected. Laboratory data confirmed adrenocorticotropic hormone-independent Cushing's syndrome with hepatic dysfunction and metyrapone treatment was initiated. A progressive normalization of cortisol levels was achieved despite poor oral tolerance. Conclusion Our case shows that metyrapone is useful in the management of neonatal Cushing's syndrome due to McCune-Albright syndrome.

Pre and post intervention study of antiblastic drugs contamination surface levels at a Pharmacy Department Compounding Area using a closed system drug transfer device and a decontamination process.

Assuring healthcare workers security on Hazardous Drugs (HD) compounding is critical in healthcare settings. Our study aims to demonstrate that the use of a Close System drug Transfer Device (CSTD) PhaSeal™ added to a decontamination process reduces antiblastic surface contamination levels in the Compounding Area (CA) of our Pharmacy Department (PD). We selected cyclophosphamide, 5-fluorouracil and iphosphamide to be evaluated. Testing was carried out with a wipe kit and quantified by an independent laboratory. We defined four sampling times: baseline; just after a decontamination procedure, which was repeated weekly during the study; four months after introduction of CSTD PhaSeal™ for cyclophosphamide and 5-fluorouracil compounding; and after eight months using CSTD PhaSeal™ for cyclophosphamide and 5-fluorouracil and one month for iphosphamide compounding. There was a decrease at the number of positive samples at the beginning/end of the study for all the drugs tested: 28/15 for cyclophosphide, 29/23 for iphosphamide and 7/1 for 5-fluorouracile. Comparing to the baseline, median cyclophosphamide levels significantly decreased (p-value <0.001) at 4 and 8 months sampling time (baseline: 1.01 ng/cm2 to 0.06 ng/cm2 and 0.01 ng/cm2), and median iphosphamide levels significantly decreased (p < 0.001) at 8 months sampling time (baseline: 3.02 ng/cm2 to 0.06 ng/cm2). 5-Fluorouracil did not show significant differences between the sampling times (baseline: 0.09 ng/cm2 to 0.09 ng/cm2). We saw a significant increase at iphosphamide levels at 4 months sampling point, contrary to cyclophosphamide, which levels had decreased. The use of CSTD PhaSeal™ for iphosphamide compounding the last month was implemented for ethical reasons after this intermediate results review. Our study suggests that the use of CSTD PhaSeal™, adding to decontaminating procedures, significantly reduces antiblastic drug surface levels at the CA of our PD.

Amethocaine-lidocaine cream, a new topical formulation for preventing venopuncture-induced pain in children.

BACKGROUND AND OBJECTIVES: The aim of this study was to compare the effectiveness of 3 anesthetic creams in relieving venous puncture-related pain in children. METHODS: We performed a double-blind, randomized, prospective study in 300 patients, American Society of Anesthesiologists (ASA) I-II, aged 3 months to 10 years, scheduled for minor elective surgical procedures. These children were distributed into 3 groups, with 100 patients each, according to each type of cream applied to the back of the children's hands before performing the venous puncture: Group I was given EMLA (eutectic 2.5% lidocaine and 2.5% prilocaine mixture), group II received 4% amethocaine (4% AMET), and group III was treated with AMLI (2.5% amethocaine and 2.5% lidocaine), a new anesthetic cream developed at our hospital. Each group was further classified into 5 subgroups, with 20 patients each, according to the time length of the application of the individual cream (group A, < or = 30 minutes; B, 30 minutes to 60 minutes; C, 60 minutes to 90 minutes; D, 90 minutes to 120 minutes; E, > or = 120 minutes). We evaluated pain, movement, and verbalization to puncture, as well as the appearance of adverse effects. RESULTS: Group I (EMLA), subgroup A (< or = 30 minutes), experienced significantly more pain, movement, and verbalization than groups II and III. Patients in group II (4% AMET) exhibited a higher frequency of untoward effects. CONCLUSIONS: All 3 creams proved to be effective in relieving venous puncture pain in children. EMLA presented a longer latency period, 4% amethocaine showed the highest percentage of untoward effects, and AMLI exhibited a shorter latency period than EMLA, while having fewer adverse effects than 4% AMET and EMLA.