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1.
Orphanet J Rare Dis ; 18(1): 339, 2023 Oct 27.
Article in English | MEDLINE | ID: mdl-37891621

ABSTRACT

BACKGROUND: Inflammatory Bowel Diseases (IBD) are known to occur in association with Hirschsprung disease (HSCR). Most of cases are represented by Crohn Disease (CD) occurring in patients with Total Colonic Aganglionosis (TCSA) with an estimated prevalence of around 2%. Based on these considerations and on a number of provisional data belonging to our Center for Digestive Diseases, we developed a unicentric cross-sectional observational study aimed at describing phenotype, genotype, pathology and metagenomics of all patients with TCSA and Crohn-like lesions. RESULTS: Out of a series of 62 eligible TCSA patients, 48 fulfilled inclusion criteria and were enrolled in the study. Ten patients did not complete the study due to non-compliance or withdrawal of consent and were subsequently dropped out. A total of 38 patients completed the study. All patients were tested for chronic intestinal inflammation by a combination of fecal calprotectine (FC) or occult fecal blood (OFB) and underwent fecal metagenomics. Nineteen (50%) tested positive for FC, OFB, or both and subsequently underwent retrograde ileoscopy. Fourteen patients (36.8%) presented Crohn-like lesions, occurring after a median of 11.5 years after surgery (range 8 months - 21.5 years). No statistically significant differences regarding demographic, phenotype and genotype were observed comparing patients with and without lesions, except for need for blood transfusion that was more frequent in those with lesions. Faecal microbiome of patients with lesions (not that of caregivers) was less biodiverse and characterized by a reduction of Bacteroidetes, and an overabundance of Proteobacteria. FC tested negative in 3/14 patients with lesions (21%). CONCLUSIONS: Our study demonstrated an impressive 10-folds higher incidence of chronic inflammation in TCSA. Up to 50% of patients may develop IBD-like lesions postoperatively. Nonetheless, we failed in identifying specific risk factors to be used to implement prevention strategies. Based on the results of our study, we suggest screening all TCSA patients with retrograde ileoscopy regardless of FC/OFB values. The frequency of endoscopic assessments and the role of FC/OFB screening in prompting endoscopy is yet to be determined.


Subject(s)
Hirschsprung Disease , Inflammatory Bowel Diseases , Humans , Hirschsprung Disease/genetics , Hirschsprung Disease/pathology , Cross-Sectional Studies , Inflammatory Bowel Diseases/complications , Inflammatory Bowel Diseases/pathology , Inflammation
2.
Med Oral Patol Oral Cir Bucal ; 23(6): e707-e715, 2018 Nov 01.
Article in English | MEDLINE | ID: mdl-30341259

ABSTRACT

BACKGROUND: To establish whether telephone follow-up is really able to intercept post-extraction complications and to evaluate the degree of patient satisfaction with this kind of post-surgical monitoring. MATERIAL AND METHODS: six hundred and thirty-eight patients were enrolled and randomly assigned to a test or control group. Test group patients were monitored by telephone follow-up 24 and 72 hours after surgery to investigate the presence of local symptoms that are frequently associated with surgical wound infection and inflammation. Both test and control group patients were examined 7 days at suture removal. Patients with systemic diseases, those in which intra-operative accidents occurred during surgery and those for whom extraction suture was not required, were excluded. RESULTS: At least one complication among alveolar osteitis, alveolar inflammation, alveolar infection and dehiscence involved 15.70% of the patients in the test group and 30.70% of the patients in the control group and telephone follow-up proved to be useful in early identification of anomalies in the post-extraction wound healing process. Comparable results were recorded in all extraction subgroups divided according to the type (surgical and non-surgical) and the number (single and multiple) of extractions performed in the same session. Telephone follow-up showed an 8.60 ± 1.17 (0 to 10 score scale) average acceptance. All cases of alveolar osteitis and infection occurred in patients who underwent antibiotic prophylaxis. CONCLUSIONS: Telephone follow-up seems to allow early detection of any possible wound healing complications, it is widely accepted by patients and it could therefore be considered a valid method for wound healing monitoring after tooth extractions, due to its effectiveness, feasibility and low costs.


Subject(s)
Inflammation/diagnosis , Postoperative Complications/diagnosis , Surgical Wound Infection/diagnosis , Telephone , Tooth Extraction , Adult , Early Diagnosis , Female , Follow-Up Studies , Humans , Male , Patient Satisfaction
3.
Minerva Med ; 81(7-8): 517-22, 1990.
Article in Italian | MEDLINE | ID: mdl-2381552

ABSTRACT

Essential mixed cryoglobulinemia (EMC) is a syndrome characterized by cryoglobulinemia and clinical features including purpura, arthralgia, asthenia (Meltzer-Franklin syndrome) without evidence of any systemic disease Liver involvement in the course of EMC is described in 50-84% of patients. It consists of mild silent hepatosplenomegaly and slightly rise of serum amino transferase. Eleven patients with clinical and laboratory findings suggestive for EMC (five type II and six type III) underwent percutaneous liver biopsy to evaluate the degree of liver involvement. Two liver cirrhosis, two chronic active hepatitis, one chronic persistent hepatitis and a case of hepatic steatosis were found. A type III cryoglobulinemia was present in four of the six patients with liver involvement. All the patients were Hbs Ag negative but three of them were Hbs Ab positive. The pathogenesis of liver involvement in the course of EMC is still now uncertain. The authors believe that a previous HBV infection plays no role in the pathogenesis of EMC syndrome. This syndrome must be considered different from mixed cryoglobulinemia secondary to chronic liver disease. They suggest that liver biopsy is mandatory during the course of EMC even when clinical and laboratory data are silent.


Subject(s)
Cryoglobulinemia/diagnosis , Liver Diseases/diagnosis , Aged , Asthenia/diagnosis , Biopsy, Needle , Cryoglobulins/analysis , Female , Hepatitis B Antibodies/analysis , Hepatitis B Surface Antigens/analysis , Hepatitis, Chronic/diagnosis , Humans , Liver/pathology , Male , Middle Aged , Purpura/diagnosis , Raynaud Disease/diagnosis , Syndrome
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