ABSTRACT
Autoinflammatory disorders are characterized by spontaneous episodes of systemic inflammation deriving from inherited defects of the innate immune system. Childhood is usually the lifetime involved in most inherited autoinflammatory disorders, but a moderate number of patients may experience disease onset during adulthood. Herein we report our experience in the clinical and genetic approach to the diagnosis of autoinflammatory disorders in regard of the first 500 pediatric and adult patients evaluated during the period 2007-2012 in our Center, due to histories of periodically-recurring inflammatory attacks, giving emphasis to the differences observed according to patients'age and to the most relevant data differentiating child and adult-onset autoinflammatory disorders in the medical literature.
Subject(s)
Hereditary Autoinflammatory Diseases , Adolescent , Adult , Age of Onset , Child , Hereditary Autoinflammatory Diseases/diagnosis , Humans , Young AdultSubject(s)
Crohn Disease/diagnosis , Magnetic Resonance Imaging/methods , Contrast Media , Female , Humans , Male , Prospective StudiesABSTRACT
PURPOSE: To evaluate intraluminal brachytherapy (ILBT) in patients with extrahepatic bile duct or pancreatic cancers. MATERIALS AND METHODS: Thirty-one patients (aged 33-87 years) with unresectable extrahepatic bile duct (n = 18) or pancreatic (n = 13) cancer received ILBT exclusively or as part of a definitive treatment regimen. ILBT was performed with transhepatic percutaneous drainage in four patients and with endoscopic retrograde cholangiopancreatography in 27. Fourteen patients with no metastases, an Eastern Cooperative Oncology Group performance score of < or = 2, and good hematologic parameters received combined modality treatment: 30-Gy ILBT and 45-Gy external-beam radiation therapy with continuous infusion of fluorouracil. Seventeen patients underwent 50-Gy ILBT alone for palliation. RESULTS: No direct treatment-related acute toxic reactions were seen. Three patients had cholangitis early in the study. Three patients had late gastrointestinal bleeding. Jaundice was palliated in all patients (n = 29); pain, in 11 of 13 patients. The survival rate in patients with extrahepatic bile duct cancer was 62% (five of eight) at 2 years for combined modality treatment. No patient with pancreatic cancer lived for longer than 2 years. CONCLUSION: ILBT is an effective palliative treatment of unresectable extrahepatic bile duct and pancreatic cancers. Results suggest a possible "curative" role in specific clinical settings when properly integrated with other treatments.
Subject(s)
Bile Duct Neoplasms/radiotherapy , Bile Ducts, Extrahepatic , Brachytherapy/methods , Palliative Care/methods , Pancreatic Neoplasms/radiotherapy , Adult , Aged , Aged, 80 and over , Bile Duct Neoplasms/diagnosis , Bile Duct Neoplasms/mortality , Brachytherapy/adverse effects , Feasibility Studies , Female , Follow-Up Studies , Humans , Male , Middle Aged , Pancreatic Neoplasms/diagnosis , Pancreatic Neoplasms/mortality , Radiotherapy Dosage , Survival AnalysisABSTRACT
PURPOSE: A new method of palliation of malignant obstructive jaundice is presented. METHODS AND MATERIALS: Twelve patients with carcinoma of the extrahepatic bile ducts (EHBD-five patients) or pancreatic head (PH-seven patients) received radiation therapy between 1988 and 1991. Percutaneous transhepatic biliary drainage was performed in four EHBD patients and an endoprosthesis was placed during endoscopic retrograde cholangiopancreatography (ERCP) in the other eight patients. All 12 received intraluminal brachytherapy (ILBT): 20-50 Gy calculated at 1 cm from the Iridium-192 (192Ir) wire. In four PH patients the source was placed in the duct of Wirsung; in the other eight patients ILBT was performed via the common bile duct. Five of the seven PH patients and one of the five EHBD patients received External Beam Radiation Therapy (EBRT): 26-50 Gy, alone or with concomitant 5-Fluorouracil (5-FU). RESULTS: Cholangitis occurred in six patients. Three PH patients treated with EBRT+ILBT developed gastrointestinal toxicities. With a minimum follow-up of 18 months, median survival times were 14 months (EHBD) and 11.5 months (PH); one of the seven PH patients is alive (29 months) and two of the EHBD patients are alive (18 and 43 months). All patients had satisfactory control of jaundice. CONCLUSIONS: The results in the EHBD patients suggest that the addition of ILBT after biliary drainage prolongs survival. Further experience is necessary to determine whether ILBT in the common bile duct and/or in the duct of Wirsung may be, in PH patients, an alternative boost technique to Interstitial Brachy-therapy (IBT) or Intraoperative Electron Beam Radiation Therapy (IOEBRT).
