La Pediatria medica e chirurgica : Medical and surgical pediatrics
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Review Case Reports
[A not very essential obesity: the Rohhad syndrome. Description of two cases and review of the literature].
Rapid-onset Obesity with Hypothalamic Dysfunction, Hypoventilation, and Autonomic Dysregulation (ROHHAD) is a rare and complex pediatric disorder. Children typically show ROHHAD after the first years of life with rapid weight gain and subsequently autonomic nervous system dysregulation (altered pain perception, pupillary dysfunction, hypothermia and bradycardia); alveolar hypoventilation with risk of cardiorespiratory arrest and hypothalamic dysfunction (central diabetes insipidus, hypothyroidism, growth hormone and corticotrophin deficiency). Tumours of neural crest origin, such as ganglioneuroblastoma and ganglioneuronoma, are reported in 33% of the patients and may be found in the chest or abdomen. Here we describe two girls who presented with rapid weight gain, at the age of 5 and 9 years respectively. The first was admitted due to obesity and central hypothyroidism. After two months she rapidly developed a clinical picture characterized by thermal dysregulation, hypodipsia and severe hypernatriemia, hypertrigliceridemia, alveolar hypoventilation supported by mechanical ventilation. The second presented with rapid-onset obesity and a mild hyperprolactinemia. After three months of follow-up she was admitted due to a clinical picture of hypothermia, seizures and hyponatremia. Subsequentely she developed altered water balance (severe hypernatremia) and severe hypoventilation. Chest CT and MR imaging showed a posterior mediastinal mass. Endocrinological investigation showed corticotrophin deficiency and central hypothyroidism treated with specific replacement therapies. ⋯ On the basis of our experiences we can infer that it is necessary perform specific further investigations of hypothalamic function in all the children with rapid onset obesity in order to early prevent the catastrophic consequences that may occur in this syndrome.
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Review
[Nurse's role in the processes of hospital humanization and procedural pain relief in children].
In childhood, hospital admission is frequently seen as something unexplanable and close to a "punishment". Involving parents/caretakers in the process of care is critical for the child to cope with the distress arising from hospital contacts and medical procedures. However, some grey zone remains for common procedures as venipuncture in hospital and ambulatory settings. ⋯ However, children attitude toward communication of health information may not be so linear, because they tend to continuously shift from a position of "passive bystander" to a "active participant" one, and viceversa. Nurse's role is crucial for the reduction of procedural distress because of her/his frequent contact with the young patients, but often she/he does not have the skills to help children to cope with procedural distress. Several approaches to this issue are discussed.
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In some premature newborns, 7 months old and with less than 1 kg of body wheight, total parenteral nutrition is used for weeks, so that good antiseptics can cooperate to the prevention of nosocomial infections, associating the best biocide effects to the best topical tolerability. Details are reported on the biocide cutaneous properties of some chloro-derivates, as sodium hypochlorite and NaDCC, of ethyl and propyl alcohols, of chlorhexidine, of iodophors and also of triclosan and octenidine (even if these latter biocids are not normally available in Italy as cutaneous antiseptics).
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Many paediatric cardiac patients now survive to adulthood following early surgery. This population of adult patients with a congenital heart disease offer distinct challenges such as unusual anatomy and demands such as pregnancy and exercise tolerability not found in conventional paediatric or traditional adult interventional patients. ⋯ There has been a rapid growth in interventional cardiology techniques to treat adults with congenital heart disease, mirroring the rise of interventional cardiology as a cardiology subspecialty and the increasing population of adults with congenital abnormalities. We review current indications for adult congenital heart disease intervention and best practice, reviewing the patient spectrum commonly treated, devices used, and emerging treatments.