Gene symbol: IDS. Disease: mucopolysaccharidosis type II (Hunter syndrome).
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Child↗
Biomedical subjects
M Sibilio
Publications and source records attributed to M Sibilio.
[Percutaneous radio frequency thermal ablation of non-resectable lung cancer: state of the art].
BACKGROUND: Unfortunately, as of yet, most lung cancers are not operable as soon as diagnosis is available; in these situations chemo- and radio-therapy still play a key role, albeit palliative, improving survival rate moderately, but are not lacking in toxic effects, especially in case of concurrent pathology, reduced cardio-respiratory functionality or being advanced in years. Therefore thermal ablation mini-invasive techniques, already employed as ancillary treatments of hepatic cancer or in place of surgery, have been performed for these pathologies. AIM: Aim of this work is to define the current state of the art for Radio-Frequency Ablation (RFA) to be performed on non-resectable lung cancer, also by means of a thorough review of international literature, from which to infer purposes, suggestions, methodologies, effectiveness, safety, complications and achievements, also in terms of the possible improvement of life quality and/or survival expectancy. PATIENTS AND METHODS: Patients have been carefully selected. Pulmonary nodules have been treated with TC or echo-guided percutaneous thermal ablation and, afterwards, evaluated by radiological and clinical (sometimes histopathological) follow-up. RESULTS: The size of the RFA-treated nodules is necessary in order to evaluate full or partial necrosis extent and, therefore, average survival rate. CONCLUSIONS: Availability of more extensive and homogeneous case histories, as well as standard follow-up (TC and/or histopathological sampling) methodologies, is required. Nevertheless several authors agree that RFA is a safe and effective technique within the framework of a substitutive or complementary treatment of non-operable lung cancer. The best results can be achieved for cancers less than 3 cm wide; RFA, performed before chemo- and/or radio-therapy, plays a neoadjuvant role for larger cancers, decreasing cancer volume and weakening the symptoms.
Catheter Ablation↗
Crohn's-like ileo-colitis in patients affected by glycogen storage disease Ib: two years' follow-up of patients with a wide spectrum of gastrointestinal signs.
AIM: To investigate the presence of inflammatory bowel disease (IBD) and to evaluate the progression of bowel involvement after two years' follow-up in seven patients affected by glycogen storage disease type Ib (GSDIb). METHODS: Seven patients (5F, 2M, aged 4.5-20.6 y) entered the study. Bowel involvement was evaluated by ileocolonoscopy and specific IBD serologic markers. To evaluate disease activity, Paediatric Crohn's Disease Activity Index (PCDAI), terminal ileum wall thickness detected at ultrasonography (US), 99mTechnetium labelled autologous White Cell Scan (Tc-WCS) and barium meal with follow-through were investigated. RESULTS: Ileocolonoscopy and histology examination revealed variable degrees of bowel involvement in all patients. The results of serologic markers were indicative of a Crohn's-like ileocolitis. US and Tc-WCS, could clearly define patients with severe inflammatory involvement, but failed to identify all patients with mild to moderate disease. For the most severely affected patients, anti-inflammatory agents and steroids were prescribed, whereas nutritional therapy with polymeric formula and antibiotics were assumed by two other patients and antibiotics only by one patient. Granulocyte colony-stimulating factor (G-CSF) was prescribed to all patients. Ileocolonoscopy and histology data improved in all patients. The assumption of G-CSF and/or gastric drip feeding (g.d.f.) was inversely associated with the PCDAI results (p < 0.05). CONCLUSION: IBD is common in patients affected by GSDIb independently of the severity of gastrointestinal signs and symptoms. Different therapeutic approaches can be used according to the severity of IBD. G-CSF treatment and g.d.f. can be protective factors for IBD.
Adolescent↗
[Pulmonary hernia].
Chest-wall hernia is a very unusual pathological event. The Authors report a singular diagnostic case of chest-wall hernia, miming rib tumor. Instrumental diagnostic tools and surgical treatment is reported. Finally patient's follow-up is discussed.
Hernia↗