Efficacy of the Andresen activator before peak growth in class II patients.

Andresen activator (AA) is a functional appliance used to correct Class II malocclusion in growing patients. It corrects the malocclusion stimulating mandibular growth and determining a palatoversion of the upper incisors and a vestibularization of the lower incisors. The aim of this study was to analyze the treatment efficacy of class II malocclusion due to mandibular hypodevelopment before peak growth. Fourteen subjects with class II relationship of the skeletal bases and cervical vertebrae maturation stage 1 or 2 were enrolled in the study. Cephalometric analyses were carried out using landmarks derived from the analyses of Pancherz, Ricketts, Tweed and Steiner. A significant decrease (P less than 0.05) in ANB angle was found (-2.29±3.05°) after treatment, which was expression of an improvement in maxillo-mandibular sagittal skeletal relationships. There was also a significant reduction of OJ after treatment (-4.44±2.36 mm; P less than 0.001), indicating a vestibularization of the mandibular incisors and a palatoversion of the maxillary incisors, and a correction of the molar relationship. The favorable effects of the Andresen activator for the correction of the mandibular defect can be found even prior to peak growth; the achieved class I relationship maintains a correct mandible position in time, ensuring a proper skeletal growth. .

Abnormalities of acid-base balance and predisposition to metabolic acidosis in Metachromatic Leukodystrophy patients.

Metachromatic Leukodystrophy (MLD; MIM# 250100) is a rare inherited lysosomal storage disorder caused by the deficiency of Arylsulfatase A (ARSA). The enzymatic defect results in the accumulation of the ARSA substrate that is particularly relevant in myelin forming cells and leads to progressive dysmyelination and dysfunction of the central and peripheral nervous system. Sulfatide accumulation has also been reported in various visceral organs, although little is known about the potential clinical consequences of such accumulation. Different forms of MLD-associated gallbladder disease have been described, and there is one reported case of an MLD patient presenting with functional consequences of sulfatide accumulation in the kidney. Here we describe a wide cohort of MLD patients in whom a tendency to sub-clinical metabolic acidosis was observed. Furthermore in some of them we report episodes of metabolic acidosis of different grades of severity developed in acute clinical conditions of various origin. Importantly, we finally show how a careful acid-base balance monitoring and prompt correction of imbalances might prevent severe consequences of acidosis.

Guidelines for the use of long-term central venous catheter in children with hemato-oncological disorders. On behalf of supportive therapy working group of Italian Association of Pediatric Hematology and Oncology (AIEOP).

In the last 30 years, the use of long-term central venous catheters (CVC) is increased especially for children with hemato-oncological disorders. However, the use of CVC is associated to complications, as mechanical accidents, thrombosis, and infections that can determine a prolongation of hospital stay, an increase of costs, and sometimes life-threatening conditions that require urgent systemic treatment or CVC removal. CVC removal may be troublesome especially in neonates, infants, or any other "highly needed CVC patients"; in these selected cases, the prevention and treatment of CVC-related complications play a pivotal role and specific surveillance programs are crucial. While extensive literature is focused on CVC management in adults, no guidelines are available for children. To this aim, the first recommendations for the management of CVC infectious complication in pediatric age have been written after pediatric and adult literature review and collegial discussion among members of Supportive Therapy working group of Italian Association of Pediatric Hematology and Oncology. Compared to the adult age, the necessity of peripheral vein cultures for the diagnosis of CVC-related infection remains controversial in children because of the poorer venous asset and a conservative, pharmacologically focused management through CVC remains mandatory, with CVC removal to be performed only in selected cases.

Requesting iodine supplementation in children on parenteral nutrition.

BACKGROUND & AIMS: Iodine supplementation of parenterally fed infants recommended by ESPGHAN is 1 microg/kg/day. To assess nutritional and thyroid status of children on parenteral nutrition (PN) through urinary iodine concentration (UIC). PATIENTS AND METHODS: Children (1-17 yrs), undergoing PN and receiving an iodine supply of 1 microg/kg/day, were enrolled from 2000 to 2007. RESULTS: We observed 15 children (10 males, mean age 76.53+/-60.4 months) on PN from 14 to 84 weeks (mean 38.5+/-21.4). Ten were on TPN and five on PPN; nine had short bowel syndrome (SBS) and six had other intestinal diseases requiring PN. Iodine supply in TPN ranged between 1 and 1.6 microg/kg/day (mean 1.1+/-0.3 microg/kg/day), while in PPN it ranged from 2.3 to 2.8 microg/kg/day (mean 2.6+/-0.7 microg/kg/day). We found an inverse correlation between duration of PN in months and UIC (P=0.05). Four weeks after PN onset, UIC<100 microg/L was found in all SBS patients and 3/6 non-SBS patients (P<0.05). After 12 weeks, 8/15 (53%) patients had UIC<50 microg/L, but thyroxine, TSH and thyroid volume remained unchanged. CONCLUSIONS: A PN iodine supply of 1 microg/kg/day may be suboptimal. Higher supplies should be evaluated in controlled trials.