End-stage disease due to liver cirrhosis is an important cause of death worldwide. Cirrhosis results from progressive, extensive fibrosis and impaired hepatocyte regeneration. The only curative treatment is liver tran...End-stage disease due to liver cirrhosis is an important cause of death worldwide. Cirrhosis results from progressive, extensive fibrosis and impaired hepatocyte regeneration. The only curative treatment is liver transplantation, but due to the several limitations of this procedure, the interest in alternative therapeutic strategies is increasing. In particular, the potential of bone marrow stem cell(BMSC) therapy in cirrhosis has been explored in different trials. In this article, we evaluate the results of 18 prospective clinical trials, and we provide a descriptive overview of recent advances in the research on hepatic regenerative medicine. The main message from the currently available data in the literature is that BMSC therapy is extremely promising in the context of liver cirrhosis. However, its application should be further explored in randomized, controlled trials with large cohorts and long follow-ups.展开更多
Recurrence of hepatitis C virus(HCV)infection following liver transplantation(LT)is almost universal and can accelerate graft cirrhosis in up to 30%of patients.The development of effective strategies to treat or preve...Recurrence of hepatitis C virus(HCV)infection following liver transplantation(LT)is almost universal and can accelerate graft cirrhosis in up to 30%of patients.The development of effective strategies to treat or prevent HCV recurrence after LT remains a major challenge,considering the shortage of donor organs and the accelerated progression of HCV in LT recipients.Standard antiviral therapy with pegylated-interferon plus ribavirin is the current treatment of choice for HCV LT recipients,even though the combination is not as effective as it is in immunocompetent patients.A sustained virological response in the setting of LT improves patient and graft survival,but this is only achieved in 30%-45%of patients and the treatment is poorly tolerated.To improve the efficacy of pre-and post-transplant antiviral therapy,a new class of potent direct-acting antiviral agents (DAAs)has been developed.The aim of this review is to summarize the use of DAAs in LT HCV patients.PubMed,Cochrane Library,MEDLINE,EMBASE,Web of Science and clinical trial databases were searched for this purpose.To date,only three clinical studies on the topic have been published and most of the available data are in abstract form.Although a moderately successful early virological response has been reported,DAA treatment regimens were associated with severe toxicity mitigating their potential usefulness.Moreover,the ongoing nature of data,the lack of randomized studies,the small number of enrolled patients and the heterogeneity of these studies make the results largely anecdotal and questionable.In conclusion,large welldesigned clinical studies on DAAs in HCV LT patients are required before these drugs can be recommended after transplantation.展开更多
AIM: To evaluate the role of genetic factors in the pathogenesis of Crohn's disease (CD) and ulcerative colitis (UC), we investigated the single nucleotide poly- morphisms (SNPs) of NOD2/CARD15 (R702W, G908R and L...AIM: To evaluate the role of genetic factors in the pathogenesis of Crohn's disease (CD) and ulcerative colitis (UC), we investigated the single nucleotide poly- morphisms (SNPs) of NOD2/CARD15 (R702W, G908R and L1007fi nsC), and Toll-like receptor 4 (TLR4) genes (D299G and T399I) in a selected inflammatory bowel disease (IBD) population coming from Southern Italy. METHODS: Allele and genotype frequencies of NOD2/ CARD15 (R702W, G908R and L1007finsC) and TLR4 (D299G and T399I) SNPs were examined in 133 CD pa-tients, in 45 UC patients, and in 103 healthy controls. A genotype-phenotype correlation was performed. RESULTS: NOD2/CARD15 R702W mutation was sig-nificantly more frequent in CD (9.8%) than in controls (2.4%, P = 0.001) and in UC (2.3%, P = 0.03). No sig-nificant difference was found between UC patients and control group (P > 0.05). In CD and UC patients, no signifi cant association with G908R variant was found. L1007f insC SNP showed an association with CD (9.8%) compared with controls (2.9%, P = 0.002) and UC patients (2.3%, P = 0.01). Moreover, in CD patients, G908R and L1007finsC mutations were significantly associated with different phenotypes compared to CD wild-type patients. No association of IBD with the TLR4 SNPs was found in either cohort (allele frequencies: D299G-controls 3.9%, CD 3.7%, UC 3.4%, P > 0.05; T399I-controls 2.9%, CD 3.0%, UC 3.4%, P > 0.05). CONCLUSION: These findings confirm that, in our IBD patients selected from Southern Italy, the NOD2/ CARD15, but not TLR4 SNPs, are associated with in-creased risk of CD.展开更多
To evaluate the effects of inflammatory bowel diseases(IBDs)on human reproduction,we reviewed the current literature using a systematic search for published studies(articles and/or abstracts)without limits for English...To evaluate the effects of inflammatory bowel diseases(IBDs)on human reproduction,we reviewed the current literature using a systematic search for published studies(articles and/or abstracts)without limits for English language.We searched on Medline(through PubMed),the Institute for Scientific Information,the Web of Science and the websites for the registration of controlled trials(http://controlled-trials.com/).Bibliographies of retrieved articles,books,expert opinion review articles and reviewed bibliographies from subject experts were manually searched.Titles and abstracts were screened initially,and potential relevant articles were identified and reviewed.Whenever possible,data were analyzed by comparing IBD patients vs healthy controls,and patients with active IBDs vs those with disease in remission.The effects of IBDs on female fertility,fertility in infertile couples,pregnancy and male infertility were examined separately.Patients with IBDs in remission have normal fertility.At the moment,there is no established guideline for the preservation of fertility in women with IBD undergoing surgery.Further data are needed regarding guidelines for the management of these patients.Data regarding IBDs and infertility are currently completely lacking.Considering the prevalence of intestinal pathology in young adults of childbearing age,this field is of great scientific and clinical interest,opening up important future perspectives.Another important and as yet unexplored point is the response to treatments for infertility in patients with IBDs.In particular,the question is whether the reproductive outcomes(clinical and biological)can be influenced by the IBD of one of the partners.The goals for successful reproductive outcomes in IBD population are correct counseling and disease remission.IBDs significantly affect several reproductive aspects of human(female,male,couple)reproduction.Further data are needed to develop guidelines for the clinical management of subjects of reproductive age with IBDs.展开更多
Short bowel syndrome(SBS)with intestinal failure(IF)is a rare but severe complication of Crohn’s disease(CD),which is the most frequent benign condition that leads to SBS after repeated surgical resections,even in th...Short bowel syndrome(SBS)with intestinal failure(IF)is a rare but severe complication of Crohn’s disease(CD),which is the most frequent benign condition that leads to SBS after repeated surgical resections,even in the era of biologics and small molecules.Glucagon-like peptide-2 analogues have been deeply studied recently for the treatment of SBS-IF.These drugs have a significant intestinotrophic effect and the potential to reduce the chronic dependence of SBSIF patients on parenteral support or nutrition.Teduglutide has been approved for the treatment of SBS-IF,and apraglutide is currently in clinical development.The use of these drugs was examined with a focus on their use in CD patients.展开更多
Inflammatory bowel disease(IBD)is a chronic condition that requires continuous medical treatment.To date,the medical management of patients with moderatelyto-severely active IBD who develop dependence or resistance to...Inflammatory bowel disease(IBD)is a chronic condition that requires continuous medical treatment.To date,the medical management of patients with moderatelyto-severely active IBD who develop dependence or resistance to corticosteroids is based on immunomodulator drugs.Such therapies are licenced after passing through three phases of randomized controlled trials(RCTs),and are subsequently adopted in clinical practice.However,the real-life population of IBD patients who require these therapies can significantly differ from those included in RCTs.As a matter of fact,there is a number of exclusion criteria–nearly ubiquitous in all RCTs–that prevent the enrolment of specific patients:Chronic refractory pouchitis or isolated proctitis in ulcerative colitis,short-bowel syndrome and stomas in Crohn’s disease,ileorectal anastomosis in both ulcerative colitis and Crohn’s disease,and elderly age are some representative examples.In this frontier article,we aim to give an overview of current literature on this topic,in order to address the main knowledge gaps that need to be filled in the upcoming years.展开更多
Infantile pyknocytosis(IP) is a rare, self-limited neonatal haemolytic anaemia that may require multiple blood transfusions. Only a little more than 50 cases have been reported in the medical literature, and the great...Infantile pyknocytosis(IP) is a rare, self-limited neonatal haemolytic anaemia that may require multiple blood transfusions. Only a little more than 50 cases have been reported in the medical literature, and the great majority of them concerns term infants. The etiology of IP is not well understood; most likely it results from a transient extra-corpuscular factor, whose nature is unknown, transmitted from mother to child or, alternatively, from a deficiency of an anti-oxidative agent. We report the case of two preterm twins, one of which suffered from IP and developed severe anaemia at age 2 wk, while the other was unaffected. Although no specific agent was identified as the cause of anaemia and IP, we speculate that the transmission of an agent from mother to child was unlikely, as only twin one suffered from IP. Smelly greenish diarrhoea occurred just before the presentation of IP, suggesting that the same agent led to both the diarrhoea and the oxidative injury. Because IP may remain underdiagnosed, it should be considered in cases of early unexplained severe hemolytic anemia.展开更多
BACKGROUND: The use of laparoscopic distal pancreatectomy(LDP) increased in the past twenty years but the real diffusion of this technique is still unknown as well as the type of centers(high or low volume) in wh...BACKGROUND: The use of laparoscopic distal pancreatectomy(LDP) increased in the past twenty years but the real diffusion of this technique is still unknown as well as the type of centers(high or low volume) in which this procedure is more frequently performed.DATA SOURCE: A systematic review was performed to evaluate the frequency of LDP in Italy and to compare indications and results in high volume centers(HVCs) and in low volume centers(LVCs).RESULTS: From 95 potentially relevant citations identified, only5 studies were included. A total of 125 subjects were analyzed, of whom 95(76.0%) were from HVCs and 30(24.0%) from LVCs.The mean number of LDPs performed per year was 6.5. The mean number of patients who underwent LDP per year was 8.8in HVCs and 3.0 in LVCs(P0.001). The most frequent lesions operated on in HVCs were cystic tumors(62.1%, P0.001) while,in LVCs, solid neoplasms(76.7%, P0.001). In HVCs, malignant neoplasms were treated with LDP less frequently than in LVCs(17.9% vs 50.0%, P〈0.001). Splenectomy was performed for non-oncologic reason frequenter in HVCs than in LVCs(70.2%vs 25.0%, P0.004). The length of stay was shorter in HVCs than in LVCs(7.5 vs 11.3, P0.001). No differences were found regarding age, gender, ductal adenocarcinoma treated, operative time, conversion, morbidity, postoperative pancreatic fistula,reoperation and margin status.CONCLUSIONS: LDPs were frequently performed in Italy.The "HVC approach" is characterized by a careful selection of patients undergoing LDP. The "LVC approach" is based on the hypothesis that LDPs are equivalent both in short-term and long-term results to laparotomic approach. These data are not conclusive and they point out the need for a national register of laparoscopic pancreatectomy.展开更多
In the two past decades, a number of communications, case-control studies, and retrospective reports have appeared in the literature with concerns about the development of a complex set of clinical, laboratory and his...In the two past decades, a number of communications, case-control studies, and retrospective reports have appeared in the literature with concerns about the development of a complex set of clinical, laboratory and histological characteristics of a liver graft dysfunction that is compatible with autoimmune hepatitis. The de novo prefix was added to distinguish this entity from a pre-transplant primary autoimmune hepatitis, but the globally accepted criteria for the diagnosis of autoimmune hepatitis have been adopted in the diagnostic algorithm. Indeed, de novo autoimmune hepatitis is characterized by the typical liver necroinflammation that is rich in plasma cells, the presence of interface hepatitis and the consequent laboratory findings of elevations in liver enzymes, increases in serum gamma globulin and the appearance of nonorgan specific auto-antibodies. Still, the overall features of de novo autoimmune hepatitis appear not to be attributable to a univocal patho-physiological pathway because they can develop in the patients who have undergone liver transplantation due to different etiologies. Specifically, in subjects with hepatitis C virus recurrence, an interferon-containing antiviral treatment has been indicated as a potential inception of immune system derangement. Herein, we attempt to review the currently available knowledge about de novo liver autoimmunity and its clinical management.展开更多
The scarcity of available organs and the gap between supply and demand continue to be the main limitations of liver transplantation. To relieve the organ shortage, current transplant strategies have implemented extend...The scarcity of available organs and the gap between supply and demand continue to be the main limitations of liver transplantation. To relieve the organ shortage, current transplant strategies have implemented extended criteria, which include the use of liver from patients with signs of past or present hepatitis B virus(HBV) infection. While the use of liver grafts from donors with evidence of past HBV infection is quite limited, some data have been collected regarding the feasibility of transplanting a liver graft from a hepatitis B surface antigen(HBs Ag) positive donor. The aim of the present work was to review the literature regarding liver transplants from HBs Ag-positive donors. A total of 17 studies were identified by a search in Medline. To date, HBs Ag positive grafts have preferentially been allocated to HBs Ag positive recipients. The large majority of these patients continue to be HBs Ag positive despite the use of immunoglobulin, and infection prevention can only be guaranteed by using antiviral prophylaxis. Although serological persistence is evident, no significant HBV-related disease has been observed, except in patients coinfected with delta virus. Consistently less data are available for HBs Ag negative recipients, although they are mostly promising. HBs Agpositive grafts could be an additional organ source for liver transplantation, provided that the risk of reinfection/reactivation is properly prevented.展开更多
Synchronous colorectal carcinoma(SCRC) indicates more than one primary colorectal carcinoma(CRC) discovered at the time of initial presentation, accounts for 3.1%-3.9% of CRC, and may occur either in the same or in di...Synchronous colorectal carcinoma(SCRC) indicates more than one primary colorectal carcinoma(CRC) discovered at the time of initial presentation, accounts for 3.1%-3.9% of CRC, and may occur either in the same or in different colorectal segments. The accurate preoperative diagnosis of SCRC is difficult and diagnostic failures may lead to inappropriate treatment and poorer prognosis. SCRC requires colorectal resections tailored to individual patients, based on the number, location, and stage of the tumours, from conventional or extended hemicolectomies to total colectomy or proctocolectomy, when established predisposing conditions exist. The overall perioperative risks of surgery for SCRC seem to be higher than for solitary CRC. Simultaneous colorectal and liver resection represents an appealing surgical strategy in selected patients with CRC and synchronous liver metastases(CRLM), even though the cumulative risks of the two procedures need to be adequately evaluated. Simultaneous resections have the noticeable advantage of avoiding a second laparotomy, give the opportunity of an earlier initiation of adjuvant therapy, and may significantly reduce the hospital costs. Because an increasing number of recent studies have shown goodresults, with morbidity, perioperative hospitalization, and mortality rates comparable to staged resections, simultaneous procedures can be selectively proposed even in case of complex colorectal resections, including those for SCRC and rectal cancer. However, in patients with multiple bilobar CRLM, major hepatectomies performed simultaneously with colorectal resection have been associated with significant perioperative risks. Conservative or parenchymal-sparing hepatectomies reduce the extent of hepatectomy while preserving oncological radicality, and may represent the best option for selected patients with multiple CRLM involving both liver lobes. Parenchymal-sparing liver resection, instead of major or two-stage hepatectomy for bilobar disease, seemingly reduces the overall operative risk of candidates to simultaneous colorectal and liver resection, and may represent the most appropriate surgical strategy whenever possible, also for patients with advanced SCRC and multiple bilobar liver metastases.展开更多
AIM:To evaluate if 3 mo oral supplementation with Eviendep was able to reduce the number of duodenal polyps in familial adenomatous polyposis(FAP)patients with ileal pouch-anal anastomosis(IPAA).METHODS:Eleven FAP pat...AIM:To evaluate if 3 mo oral supplementation with Eviendep was able to reduce the number of duodenal polyps in familial adenomatous polyposis(FAP)patients with ileal pouch-anal anastomosis(IPAA).METHODS:Eleven FAP patients with IPAA and duodenal polyps were enrolled.They underwent upper gastrointestinal(GI)endoscopy at the baseline and after 3 mo of treatment.Each patient received 5 mg Eviendep twice a day,at breakfast and dinner time,for3 mo.Two endoscopists evaluated in a blinded manner the number and size of duodenal polyps.Upper GI endoscopies with biopsies were performed at the baseline(T0)with the assessment of the Spigelman score.Polyps>10 mm were removed during endoscopy and at the end of the procedure a new Spigelman score was determined(T1).The procedure was repeated 3 mo after the baseline(T2).Four photograms were examined for each patient,at T1 and T2.The examined area was divided into 3 segments:duodenal bulb,second and third portion duodenum.Biopsy specimens were taken from all polyps>10 mm and from all suspicious ones,defined by the presence of a central depression,irregular surface,or irregular vascular pattern.Histology was classified according to the updated Vienna criteria.RESULTS:At baseline the mean number of duodenal detected polyps was 27.7 and mean sizes were 15.8mm;the mean Spigelman score was 7.1.After polypectomy the mean number of duodenal detected polyps was 25.7 and mean sizes were 7.6 mm;the mean Spigelman score was 6.4.After 3 mo of Eviendep bid,all patients showed a reduction of number and size of duodenal polyps.The mean number of duodenal polyps was 8(P=0.021)and mean size was 4.4 mm;the mean Spigelman score was 6.6.Interrater agreement was measured.Lesions>1 cm found a very good degree of concordance(kappa 0.851)and a good concordance was as well encountered for smaller lesions(kappa 0.641).CONCLUSION:Our study demonstrated that shortterm(90 d)supplementation with Eviendep in FAP patients with IPAA and with recurrent adenomas in the duodenal mucosa,resulted effective in reducing polyps number of 32%and size of 51%.展开更多
AIM To assess vitamin E efficacy,defined as its ability to induce hepatitis B e antigen(HBeAg) seroconversion,in children with HBeAg-positive persistent hepatitis.METHODS In July 2016,we extracted articles published i...AIM To assess vitamin E efficacy,defined as its ability to induce hepatitis B e antigen(HBeAg) seroconversion,in children with HBeAg-positive persistent hepatitis.METHODS In July 2016,we extracted articles published in MEDLINE and the Cochrane Library using the following search terms:"chronic hepatitis B","children","childhood","therapy","treatment","vitamin E","tocopherols","tocotrienols".Only randomized controlled trials(RCTs) published in English language were collected.RESULTS Three RCTs met inclusion criteria and were considered in the present meta-analysis.Overall,23/122 children in the treatment group underwent HBeAg seroconversion vs 3/74 in the control group(OR=3.96,95%CI:1.18-13.25,P=0.025).CONCLUSION Although our meta-analysis has several limits,including the very small number of available studies and enrolled children with HBeAg positivity-related hepatitis,it suggests that vitamin E use may enhance the probability to induce HBeAg seroconversion in these patients.Further well designed and adequately sized trials are required to confirm or deny these very preliminary results.展开更多
BACKGROUND The therapeutic approach of metastatic renal cell carcinoma(RCC)represents a real challenge for clinicians,because of the variable clinical course;the recent availability of numerous targeted therapies that...BACKGROUND The therapeutic approach of metastatic renal cell carcinoma(RCC)represents a real challenge for clinicians,because of the variable clinical course;the recent availability of numerous targeted therapies that have significantly improved overall oncological results,but still with a low percentage of complete responses;and the increasing role of metastasectomy(MSX)as an effective strategy to achieve a durable cure,or at least defer initiation of systemic therapies,in selected patients and in the context of multimodality treatment strategies.CA^E SUMMARY We report here the case of a 40-year-old man who was referred to our unit in November 2004 with lung and mediastinal lymph nodes metastases identified during periodic surveillance 6 years after a radical nephrectomy for RCC;he underwent MSX of multiple lung nodules and mediastinal lymphadenectomy,with subsequent systemic therapy with Fluorouracil,Interferon-alpha and Interleukin 2.The subsequent clinical course was characterized by multiple sequential abdominal and thoracic recurrences,successfully treated with multiple systemic treatments,repeated local treatments,including two pancreatic resections,conservative resection and ablation of multiple bilobar liver metastases,resection and stereotactic body radiotherapy of multiple lung metastases.He is alive without evidence of recurrence 20 years after initial nephrectomy and sequential treatment of recurrences in multiple sites,including resection of more than 38 metastases,and 5 years after his last MSX.CONCLUSION This case highlights that effective multimodality therapeutic strategies,including multiple systemic treatments and iterative aggressive surgical resection,can be safely performed with long-term survival in selected patients with multiple metachronous sequential metastases from RCC.展开更多
Malleolar ankle fractures have been classified using plain radiographs,and there is no consensus regarding the role of computed tomography(CT)scans in preoperative planning.We analyzed critical aspects,such as limits ...Malleolar ankle fractures have been classified using plain radiographs,and there is no consensus regarding the role of computed tomography(CT)scans in preoperative planning.We analyzed critical aspects,such as limits of standard radiographs,types of injury,classification methods and cost/benefit evaluations.CT scans allow a 3 D analysis of the fracture to be obtained and consequently assess the indication for surgical procedure,surgical access and the type of fixation devices required.This exam is useful for detecting lesions that may go unnoticed on radiographs and will help surgeons to clarify the pathoanatomy of ankle fractures.According to Arbeitsgemeinschaft fur Osteosynthesefragen/Orthopaedic Trauma Association(AO/OTA)classification,CT scan is recommended in medial malleolar fractures with vertical rim,type 44 B fractures with posterior malleolar involvement and all type 44 C fractures(according to AO/OTA).Also Tillaux-Chaput fractures(43-B1 according to AO/OTA),malleolar fractures in the presence of distal tibial fractures(43 according to AO/OTA)and distal tibia fractures in adolescents should be studied with CT scans.展开更多
In December 2019 a novel coronavirus disease 2019(COVID-19),caused by the severe acute respiratory syndrome coronavirus-2(SARS-CoV-2),started spreading from Wuhan city of Chinese Hubei province and rapidly became a gl...In December 2019 a novel coronavirus disease 2019(COVID-19),caused by the severe acute respiratory syndrome coronavirus-2(SARS-CoV-2),started spreading from Wuhan city of Chinese Hubei province and rapidly became a global pandemic.Clinical symptoms of the disease range from paucisymptomatic disease to a much more severe disease.Typical symptoms of the initial phase include fever and cough,with possible progression to acute respiratory distress syndrome.Gastrointestinal manifestations such as diarrhoea,vomiting and abdominal pain are reported in a considerable number of affected individuals and may be due to the SARS-CoV-2 tropism for the peptidase angiotensin receptor 2.The intestinal homeostasis and microenvironment appear to play a major role in the pathogenesis of COVID-19 and in the enhancement of the systemic inflammatory responses.Long-term consequences of COVID-19 include respiratory disturbances and other disabling manifestations,such as fatigue and psychological impairment.To date,there is a paucity of data on the gastrointestinal sequelae of SARS-CoV-2 infection.Since COVID-19 can directly or indirectly affect the gut physiology in different ways,it is plausible that functional bowel diseases may occur after the recovery because of potential pathophysiological alterations(dysbiosis,disruption of the intestinal barrier,mucosal microinflammation,post-infectious states,immune dysregulation and psychological stress).In this review we speculate that COVID-19 can trigger irritable bowel syndrome and we discuss the potential mechanisms.展开更多
Cystic fibrosis, a common autosomal recessive genetic disorder among Caucasians, is caused by defects in the transmembrane conductance regulatory(CFTR) gene. The analysis of CFTR gene mutations is useful to better cha...Cystic fibrosis, a common autosomal recessive genetic disorder among Caucasians, is caused by defects in the transmembrane conductance regulatory(CFTR) gene. The analysis of CFTR gene mutations is useful to better characterize the disease, and for preconceptional screening, prenatal and preimplantation genetic diagnosis. Here we report the results of a genetic analysis in a 16-year-old boy from southwestern Iran diagnosed as having cystic fibrosis in infancy based on gastrointestinal and pulmonary manifestations, with positive sweat chloride tests. He lacked both normal and mutant forms of the fragment corresponding to the F508 allele in initial genetic studies. Multiplex ligationdependent probe amplification-based testing revealed a homozygous deletion spanning exons 4 to 10 of the CFTR gene. We predict an in-frame deletion removing 373 amino acids based on our sequencing results. Determining CFTR gene mutations in patients and their family members would be helpful to prevent the occurrence of new cases, especially in populations in which consanguinity is common.展开更多
Patients affected by pancreatic ductal adenocarcinoma(PDAC)frequently present with advanced disease at the time of diagnosis,limiting an upfront surgical approach.Neoadjuvant treatment(NAT)has become the standard of c...Patients affected by pancreatic ductal adenocarcinoma(PDAC)frequently present with advanced disease at the time of diagnosis,limiting an upfront surgical approach.Neoadjuvant treatment(NAT)has become the standard of care to downstage non-metastatic locally advanced PDAC.However,this treatment increases the risk of a nutritional status decline,which in turn,may impact therapeutic tolerance,postoperative outcomes,or even prevent the possibility of surgery.Literature on prehabilitation programs on surgical PDAC patients show a reduction of postoperative complications,length of hospital stay,and readmission rate,while data on prehabilitation in NAT patients are scarce and randomized controlled trials are still missing.Particularly,appropriate nutritional management represents an important therapeutic strategy to promote tissue healing and to enhance patient recovery after surgical trauma.In this regard,NAT may represent a new interesting window of opportunity to implement a nutritional prehabilitation program,aiming to increase the PDAC patient’s capacity to complete the planned therapy and potentially improve clinical and survival outcomes.Given these perspectives,this review attempts to provide an in-depth view of the nutritional derangements during NAT and nutritional prehabilitation program as well as their impact on PDAC patient outcomes.展开更多
The objective of this study was to evaluate the association between HIV infection and hypertensive disorders of pregnancy, comparing the rates of preeclampsia and gestational hypertension in a HIV-infected pregnant gr...The objective of this study was to evaluate the association between HIV infection and hypertensive disorders of pregnancy, comparing the rates of preeclampsia and gestational hypertension in a HIV-infected pregnant group and in a HIV-negative control pregnant group matched for age and parity. Furthermore, we aimed to compare the rates of hypertensive disorders in a subgroup of HIV-positive and HIV-negative African-American Black women. Patients and Methods: This was a prospective observational cohort study conducted at two University Departments of Obstetrics and Gynecology, Salesi Hospital, Ancona, and Sant’Orsola Hospital, Bologna. The HIV-infected patients’ group consisted of 126 pregnant women;140 HIV-negative pregnant women matched for age and parity served as controls. Gestational hypertension and preeclampsia were diagnosed according to NHBPEP-ISSHP criteria. Categorical data were analyzed using the Fisher exact test. Statistical significance was set at a p value < 0.05. Results: Gestational hypertension and preeclampsia were diagnosed in 3 of 126 HIV-positive patients (2.38%) and in 14 of 140 HIV-negative patients (10%), with a relative risk of 0.24 (p = 0.0112). In the subgroup of African-American Black women, gestational hypertension and preeclampsia were diagnosed in 2 out of 43 HIV-positive (4.7%) and in 3 out of 18 HIV-negative patients (16.7%) with a relative risk of 0.28, not statistically significant (p = 0.1887). Conclusion: Pregnant women with HIV infection seem to be protected against gestational hypertension and preeclampsia and this protective effect remains also in a high risk population, such as African-American Black ethnic group. The effect is present independently from treatment received and virus copies. The lack of immune response present since the conception period should account for unopposed trophoblast invasion resulting in a better placentation.展开更多
Background and Purpose: Constipation is one of the major sequelae in patients after correction of anorectal anomalies (ARAs). The aim of the present work has been to assess the colonic transit time,using radioisotope ...Background and Purpose: Constipation is one of the major sequelae in patients after correction of anorectal anomalies (ARAs). The aim of the present work has been to assess the colonic transit time,using radioisotope scintigraphy,in patients operated for ARA and experiencing constipation in the follow-up. The results were compared with transit time from children with true functional constipation. Methods: Twelve or 32 patients operated for ARA during the period 1994-2003 experienced mild or severe constipation (6 with high or intermediate form of ARA and 6 with low type) at follow-up. The mean age of this group was 5.8 years. Eighteen patients,mean age 6.7 years,with true functional constipation were studied as well. Colonic transit times were investigated using radioisotope scintigraphy. Normal values for colonic transit time were derived from historical controls. Radioisotope diethylenetriamine pentaacetic acid labelled with indium 111 was administered orally to determine a segmental colonic transit. Images of the abdomen have been taken at 6,24,48,and again at 72 hours,if radioactivity was not cleared from the colon. To quantify colonic transit,we calculated the geometric centre (GC) dividing the colon into anatomic regions. Results: According to normal controls,2 different type of delayed transit can be observed: (a) slow-transit constipation if GC at 48 hours is less than 4.1; (b) functional rectosigmoid obstruction (FRSO) if GC at 48 hours is 4.1 or more but less than 6.1 at 72 hours. Patients with functional constipation were divided into 2 groups: (a)-slow-transit constipation in 12 patients with a GC at 48 hours of 3.7 ± 0.5; (b) FRSO in 6 patients with a GC of 4.7 ± 0.04 and 5.02 at 48 and 72 hours,respectively. Patients operated for high ARA had values characteristic of FRSO with GC at 48 hours of 5.1 ± 0.8 and 4.75 ± 0.5 at 72 hours. In low ARA,the transit times were similar to the ones observed in patients with high ARA at 48 hours with a GC of 4.9 ± 0.5. Conclusions: Patients with ARA frequently have functional sequelae in the postoperative period such as constipation. According to our results,constipation seems to be secondary to segmental motility disorders limited to the rectosigmoid area,similar to constipated children with FRSO. No evidence of more gener-alised motility disturbance,as previously postulated,could be recorded.展开更多
文摘End-stage disease due to liver cirrhosis is an important cause of death worldwide. Cirrhosis results from progressive, extensive fibrosis and impaired hepatocyte regeneration. The only curative treatment is liver transplantation, but due to the several limitations of this procedure, the interest in alternative therapeutic strategies is increasing. In particular, the potential of bone marrow stem cell(BMSC) therapy in cirrhosis has been explored in different trials. In this article, we evaluate the results of 18 prospective clinical trials, and we provide a descriptive overview of recent advances in the research on hepatic regenerative medicine. The main message from the currently available data in the literature is that BMSC therapy is extremely promising in the context of liver cirrhosis. However, its application should be further explored in randomized, controlled trials with large cohorts and long follow-ups.
文摘Recurrence of hepatitis C virus(HCV)infection following liver transplantation(LT)is almost universal and can accelerate graft cirrhosis in up to 30%of patients.The development of effective strategies to treat or prevent HCV recurrence after LT remains a major challenge,considering the shortage of donor organs and the accelerated progression of HCV in LT recipients.Standard antiviral therapy with pegylated-interferon plus ribavirin is the current treatment of choice for HCV LT recipients,even though the combination is not as effective as it is in immunocompetent patients.A sustained virological response in the setting of LT improves patient and graft survival,but this is only achieved in 30%-45%of patients and the treatment is poorly tolerated.To improve the efficacy of pre-and post-transplant antiviral therapy,a new class of potent direct-acting antiviral agents (DAAs)has been developed.The aim of this review is to summarize the use of DAAs in LT HCV patients.PubMed,Cochrane Library,MEDLINE,EMBASE,Web of Science and clinical trial databases were searched for this purpose.To date,only three clinical studies on the topic have been published and most of the available data are in abstract form.Although a moderately successful early virological response has been reported,DAA treatment regimens were associated with severe toxicity mitigating their potential usefulness.Moreover,the ongoing nature of data,the lack of randomized studies,the small number of enrolled patients and the heterogeneity of these studies make the results largely anecdotal and questionable.In conclusion,large welldesigned clinical studies on DAAs in HCV LT patients are required before these drugs can be recommended after transplantation.
文摘AIM: To evaluate the role of genetic factors in the pathogenesis of Crohn's disease (CD) and ulcerative colitis (UC), we investigated the single nucleotide poly- morphisms (SNPs) of NOD2/CARD15 (R702W, G908R and L1007fi nsC), and Toll-like receptor 4 (TLR4) genes (D299G and T399I) in a selected inflammatory bowel disease (IBD) population coming from Southern Italy. METHODS: Allele and genotype frequencies of NOD2/ CARD15 (R702W, G908R and L1007finsC) and TLR4 (D299G and T399I) SNPs were examined in 133 CD pa-tients, in 45 UC patients, and in 103 healthy controls. A genotype-phenotype correlation was performed. RESULTS: NOD2/CARD15 R702W mutation was sig-nificantly more frequent in CD (9.8%) than in controls (2.4%, P = 0.001) and in UC (2.3%, P = 0.03). No sig-nificant difference was found between UC patients and control group (P > 0.05). In CD and UC patients, no signifi cant association with G908R variant was found. L1007f insC SNP showed an association with CD (9.8%) compared with controls (2.9%, P = 0.002) and UC patients (2.3%, P = 0.01). Moreover, in CD patients, G908R and L1007finsC mutations were significantly associated with different phenotypes compared to CD wild-type patients. No association of IBD with the TLR4 SNPs was found in either cohort (allele frequencies: D299G-controls 3.9%, CD 3.7%, UC 3.4%, P > 0.05; T399I-controls 2.9%, CD 3.0%, UC 3.4%, P > 0.05). CONCLUSION: These findings confirm that, in our IBD patients selected from Southern Italy, the NOD2/ CARD15, but not TLR4 SNPs, are associated with in-creased risk of CD.
文摘To evaluate the effects of inflammatory bowel diseases(IBDs)on human reproduction,we reviewed the current literature using a systematic search for published studies(articles and/or abstracts)without limits for English language.We searched on Medline(through PubMed),the Institute for Scientific Information,the Web of Science and the websites for the registration of controlled trials(http://controlled-trials.com/).Bibliographies of retrieved articles,books,expert opinion review articles and reviewed bibliographies from subject experts were manually searched.Titles and abstracts were screened initially,and potential relevant articles were identified and reviewed.Whenever possible,data were analyzed by comparing IBD patients vs healthy controls,and patients with active IBDs vs those with disease in remission.The effects of IBDs on female fertility,fertility in infertile couples,pregnancy and male infertility were examined separately.Patients with IBDs in remission have normal fertility.At the moment,there is no established guideline for the preservation of fertility in women with IBD undergoing surgery.Further data are needed regarding guidelines for the management of these patients.Data regarding IBDs and infertility are currently completely lacking.Considering the prevalence of intestinal pathology in young adults of childbearing age,this field is of great scientific and clinical interest,opening up important future perspectives.Another important and as yet unexplored point is the response to treatments for infertility in patients with IBDs.In particular,the question is whether the reproductive outcomes(clinical and biological)can be influenced by the IBD of one of the partners.The goals for successful reproductive outcomes in IBD population are correct counseling and disease remission.IBDs significantly affect several reproductive aspects of human(female,male,couple)reproduction.Further data are needed to develop guidelines for the clinical management of subjects of reproductive age with IBDs.
文摘Short bowel syndrome(SBS)with intestinal failure(IF)is a rare but severe complication of Crohn’s disease(CD),which is the most frequent benign condition that leads to SBS after repeated surgical resections,even in the era of biologics and small molecules.Glucagon-like peptide-2 analogues have been deeply studied recently for the treatment of SBS-IF.These drugs have a significant intestinotrophic effect and the potential to reduce the chronic dependence of SBSIF patients on parenteral support or nutrition.Teduglutide has been approved for the treatment of SBS-IF,and apraglutide is currently in clinical development.The use of these drugs was examined with a focus on their use in CD patients.
文摘Inflammatory bowel disease(IBD)is a chronic condition that requires continuous medical treatment.To date,the medical management of patients with moderatelyto-severely active IBD who develop dependence or resistance to corticosteroids is based on immunomodulator drugs.Such therapies are licenced after passing through three phases of randomized controlled trials(RCTs),and are subsequently adopted in clinical practice.However,the real-life population of IBD patients who require these therapies can significantly differ from those included in RCTs.As a matter of fact,there is a number of exclusion criteria–nearly ubiquitous in all RCTs–that prevent the enrolment of specific patients:Chronic refractory pouchitis or isolated proctitis in ulcerative colitis,short-bowel syndrome and stomas in Crohn’s disease,ileorectal anastomosis in both ulcerative colitis and Crohn’s disease,and elderly age are some representative examples.In this frontier article,we aim to give an overview of current literature on this topic,in order to address the main knowledge gaps that need to be filled in the upcoming years.
文摘Infantile pyknocytosis(IP) is a rare, self-limited neonatal haemolytic anaemia that may require multiple blood transfusions. Only a little more than 50 cases have been reported in the medical literature, and the great majority of them concerns term infants. The etiology of IP is not well understood; most likely it results from a transient extra-corpuscular factor, whose nature is unknown, transmitted from mother to child or, alternatively, from a deficiency of an anti-oxidative agent. We report the case of two preterm twins, one of which suffered from IP and developed severe anaemia at age 2 wk, while the other was unaffected. Although no specific agent was identified as the cause of anaemia and IP, we speculate that the transmission of an agent from mother to child was unlikely, as only twin one suffered from IP. Smelly greenish diarrhoea occurred just before the presentation of IP, suggesting that the same agent led to both the diarrhoea and the oxidative injury. Because IP may remain underdiagnosed, it should be considered in cases of early unexplained severe hemolytic anemia.
文摘BACKGROUND: The use of laparoscopic distal pancreatectomy(LDP) increased in the past twenty years but the real diffusion of this technique is still unknown as well as the type of centers(high or low volume) in which this procedure is more frequently performed.DATA SOURCE: A systematic review was performed to evaluate the frequency of LDP in Italy and to compare indications and results in high volume centers(HVCs) and in low volume centers(LVCs).RESULTS: From 95 potentially relevant citations identified, only5 studies were included. A total of 125 subjects were analyzed, of whom 95(76.0%) were from HVCs and 30(24.0%) from LVCs.The mean number of LDPs performed per year was 6.5. The mean number of patients who underwent LDP per year was 8.8in HVCs and 3.0 in LVCs(P0.001). The most frequent lesions operated on in HVCs were cystic tumors(62.1%, P0.001) while,in LVCs, solid neoplasms(76.7%, P0.001). In HVCs, malignant neoplasms were treated with LDP less frequently than in LVCs(17.9% vs 50.0%, P〈0.001). Splenectomy was performed for non-oncologic reason frequenter in HVCs than in LVCs(70.2%vs 25.0%, P0.004). The length of stay was shorter in HVCs than in LVCs(7.5 vs 11.3, P0.001). No differences were found regarding age, gender, ductal adenocarcinoma treated, operative time, conversion, morbidity, postoperative pancreatic fistula,reoperation and margin status.CONCLUSIONS: LDPs were frequently performed in Italy.The "HVC approach" is characterized by a careful selection of patients undergoing LDP. The "LVC approach" is based on the hypothesis that LDPs are equivalent both in short-term and long-term results to laparotomic approach. These data are not conclusive and they point out the need for a national register of laparoscopic pancreatectomy.
文摘In the two past decades, a number of communications, case-control studies, and retrospective reports have appeared in the literature with concerns about the development of a complex set of clinical, laboratory and histological characteristics of a liver graft dysfunction that is compatible with autoimmune hepatitis. The de novo prefix was added to distinguish this entity from a pre-transplant primary autoimmune hepatitis, but the globally accepted criteria for the diagnosis of autoimmune hepatitis have been adopted in the diagnostic algorithm. Indeed, de novo autoimmune hepatitis is characterized by the typical liver necroinflammation that is rich in plasma cells, the presence of interface hepatitis and the consequent laboratory findings of elevations in liver enzymes, increases in serum gamma globulin and the appearance of nonorgan specific auto-antibodies. Still, the overall features of de novo autoimmune hepatitis appear not to be attributable to a univocal patho-physiological pathway because they can develop in the patients who have undergone liver transplantation due to different etiologies. Specifically, in subjects with hepatitis C virus recurrence, an interferon-containing antiviral treatment has been indicated as a potential inception of immune system derangement. Herein, we attempt to review the currently available knowledge about de novo liver autoimmunity and its clinical management.
文摘The scarcity of available organs and the gap between supply and demand continue to be the main limitations of liver transplantation. To relieve the organ shortage, current transplant strategies have implemented extended criteria, which include the use of liver from patients with signs of past or present hepatitis B virus(HBV) infection. While the use of liver grafts from donors with evidence of past HBV infection is quite limited, some data have been collected regarding the feasibility of transplanting a liver graft from a hepatitis B surface antigen(HBs Ag) positive donor. The aim of the present work was to review the literature regarding liver transplants from HBs Ag-positive donors. A total of 17 studies were identified by a search in Medline. To date, HBs Ag positive grafts have preferentially been allocated to HBs Ag positive recipients. The large majority of these patients continue to be HBs Ag positive despite the use of immunoglobulin, and infection prevention can only be guaranteed by using antiviral prophylaxis. Although serological persistence is evident, no significant HBV-related disease has been observed, except in patients coinfected with delta virus. Consistently less data are available for HBs Ag negative recipients, although they are mostly promising. HBs Agpositive grafts could be an additional organ source for liver transplantation, provided that the risk of reinfection/reactivation is properly prevented.
文摘Synchronous colorectal carcinoma(SCRC) indicates more than one primary colorectal carcinoma(CRC) discovered at the time of initial presentation, accounts for 3.1%-3.9% of CRC, and may occur either in the same or in different colorectal segments. The accurate preoperative diagnosis of SCRC is difficult and diagnostic failures may lead to inappropriate treatment and poorer prognosis. SCRC requires colorectal resections tailored to individual patients, based on the number, location, and stage of the tumours, from conventional or extended hemicolectomies to total colectomy or proctocolectomy, when established predisposing conditions exist. The overall perioperative risks of surgery for SCRC seem to be higher than for solitary CRC. Simultaneous colorectal and liver resection represents an appealing surgical strategy in selected patients with CRC and synchronous liver metastases(CRLM), even though the cumulative risks of the two procedures need to be adequately evaluated. Simultaneous resections have the noticeable advantage of avoiding a second laparotomy, give the opportunity of an earlier initiation of adjuvant therapy, and may significantly reduce the hospital costs. Because an increasing number of recent studies have shown goodresults, with morbidity, perioperative hospitalization, and mortality rates comparable to staged resections, simultaneous procedures can be selectively proposed even in case of complex colorectal resections, including those for SCRC and rectal cancer. However, in patients with multiple bilobar CRLM, major hepatectomies performed simultaneously with colorectal resection have been associated with significant perioperative risks. Conservative or parenchymal-sparing hepatectomies reduce the extent of hepatectomy while preserving oncological radicality, and may represent the best option for selected patients with multiple CRLM involving both liver lobes. Parenchymal-sparing liver resection, instead of major or two-stage hepatectomy for bilobar disease, seemingly reduces the overall operative risk of candidates to simultaneous colorectal and liver resection, and may represent the most appropriate surgical strategy whenever possible, also for patients with advanced SCRC and multiple bilobar liver metastases.
文摘AIM:To evaluate if 3 mo oral supplementation with Eviendep was able to reduce the number of duodenal polyps in familial adenomatous polyposis(FAP)patients with ileal pouch-anal anastomosis(IPAA).METHODS:Eleven FAP patients with IPAA and duodenal polyps were enrolled.They underwent upper gastrointestinal(GI)endoscopy at the baseline and after 3 mo of treatment.Each patient received 5 mg Eviendep twice a day,at breakfast and dinner time,for3 mo.Two endoscopists evaluated in a blinded manner the number and size of duodenal polyps.Upper GI endoscopies with biopsies were performed at the baseline(T0)with the assessment of the Spigelman score.Polyps>10 mm were removed during endoscopy and at the end of the procedure a new Spigelman score was determined(T1).The procedure was repeated 3 mo after the baseline(T2).Four photograms were examined for each patient,at T1 and T2.The examined area was divided into 3 segments:duodenal bulb,second and third portion duodenum.Biopsy specimens were taken from all polyps>10 mm and from all suspicious ones,defined by the presence of a central depression,irregular surface,or irregular vascular pattern.Histology was classified according to the updated Vienna criteria.RESULTS:At baseline the mean number of duodenal detected polyps was 27.7 and mean sizes were 15.8mm;the mean Spigelman score was 7.1.After polypectomy the mean number of duodenal detected polyps was 25.7 and mean sizes were 7.6 mm;the mean Spigelman score was 6.4.After 3 mo of Eviendep bid,all patients showed a reduction of number and size of duodenal polyps.The mean number of duodenal polyps was 8(P=0.021)and mean size was 4.4 mm;the mean Spigelman score was 6.6.Interrater agreement was measured.Lesions>1 cm found a very good degree of concordance(kappa 0.851)and a good concordance was as well encountered for smaller lesions(kappa 0.641).CONCLUSION:Our study demonstrated that shortterm(90 d)supplementation with Eviendep in FAP patients with IPAA and with recurrent adenomas in the duodenal mucosa,resulted effective in reducing polyps number of 32%and size of 51%.
文摘AIM To assess vitamin E efficacy,defined as its ability to induce hepatitis B e antigen(HBeAg) seroconversion,in children with HBeAg-positive persistent hepatitis.METHODS In July 2016,we extracted articles published in MEDLINE and the Cochrane Library using the following search terms:"chronic hepatitis B","children","childhood","therapy","treatment","vitamin E","tocopherols","tocotrienols".Only randomized controlled trials(RCTs) published in English language were collected.RESULTS Three RCTs met inclusion criteria and were considered in the present meta-analysis.Overall,23/122 children in the treatment group underwent HBeAg seroconversion vs 3/74 in the control group(OR=3.96,95%CI:1.18-13.25,P=0.025).CONCLUSION Although our meta-analysis has several limits,including the very small number of available studies and enrolled children with HBeAg positivity-related hepatitis,it suggests that vitamin E use may enhance the probability to induce HBeAg seroconversion in these patients.Further well designed and adequately sized trials are required to confirm or deny these very preliminary results.
文摘BACKGROUND The therapeutic approach of metastatic renal cell carcinoma(RCC)represents a real challenge for clinicians,because of the variable clinical course;the recent availability of numerous targeted therapies that have significantly improved overall oncological results,but still with a low percentage of complete responses;and the increasing role of metastasectomy(MSX)as an effective strategy to achieve a durable cure,or at least defer initiation of systemic therapies,in selected patients and in the context of multimodality treatment strategies.CA^E SUMMARY We report here the case of a 40-year-old man who was referred to our unit in November 2004 with lung and mediastinal lymph nodes metastases identified during periodic surveillance 6 years after a radical nephrectomy for RCC;he underwent MSX of multiple lung nodules and mediastinal lymphadenectomy,with subsequent systemic therapy with Fluorouracil,Interferon-alpha and Interleukin 2.The subsequent clinical course was characterized by multiple sequential abdominal and thoracic recurrences,successfully treated with multiple systemic treatments,repeated local treatments,including two pancreatic resections,conservative resection and ablation of multiple bilobar liver metastases,resection and stereotactic body radiotherapy of multiple lung metastases.He is alive without evidence of recurrence 20 years after initial nephrectomy and sequential treatment of recurrences in multiple sites,including resection of more than 38 metastases,and 5 years after his last MSX.CONCLUSION This case highlights that effective multimodality therapeutic strategies,including multiple systemic treatments and iterative aggressive surgical resection,can be safely performed with long-term survival in selected patients with multiple metachronous sequential metastases from RCC.
文摘Malleolar ankle fractures have been classified using plain radiographs,and there is no consensus regarding the role of computed tomography(CT)scans in preoperative planning.We analyzed critical aspects,such as limits of standard radiographs,types of injury,classification methods and cost/benefit evaluations.CT scans allow a 3 D analysis of the fracture to be obtained and consequently assess the indication for surgical procedure,surgical access and the type of fixation devices required.This exam is useful for detecting lesions that may go unnoticed on radiographs and will help surgeons to clarify the pathoanatomy of ankle fractures.According to Arbeitsgemeinschaft fur Osteosynthesefragen/Orthopaedic Trauma Association(AO/OTA)classification,CT scan is recommended in medial malleolar fractures with vertical rim,type 44 B fractures with posterior malleolar involvement and all type 44 C fractures(according to AO/OTA).Also Tillaux-Chaput fractures(43-B1 according to AO/OTA),malleolar fractures in the presence of distal tibial fractures(43 according to AO/OTA)and distal tibia fractures in adolescents should be studied with CT scans.
文摘In December 2019 a novel coronavirus disease 2019(COVID-19),caused by the severe acute respiratory syndrome coronavirus-2(SARS-CoV-2),started spreading from Wuhan city of Chinese Hubei province and rapidly became a global pandemic.Clinical symptoms of the disease range from paucisymptomatic disease to a much more severe disease.Typical symptoms of the initial phase include fever and cough,with possible progression to acute respiratory distress syndrome.Gastrointestinal manifestations such as diarrhoea,vomiting and abdominal pain are reported in a considerable number of affected individuals and may be due to the SARS-CoV-2 tropism for the peptidase angiotensin receptor 2.The intestinal homeostasis and microenvironment appear to play a major role in the pathogenesis of COVID-19 and in the enhancement of the systemic inflammatory responses.Long-term consequences of COVID-19 include respiratory disturbances and other disabling manifestations,such as fatigue and psychological impairment.To date,there is a paucity of data on the gastrointestinal sequelae of SARS-CoV-2 infection.Since COVID-19 can directly or indirectly affect the gut physiology in different ways,it is plausible that functional bowel diseases may occur after the recovery because of potential pathophysiological alterations(dysbiosis,disruption of the intestinal barrier,mucosal microinflammation,post-infectious states,immune dysregulation and psychological stress).In this review we speculate that COVID-19 can trigger irritable bowel syndrome and we discuss the potential mechanisms.
基金Supported by Shiraz University of Medical Sciences,Shiraz,Iran and Bologna University,Bologna,Italy
文摘Cystic fibrosis, a common autosomal recessive genetic disorder among Caucasians, is caused by defects in the transmembrane conductance regulatory(CFTR) gene. The analysis of CFTR gene mutations is useful to better characterize the disease, and for preconceptional screening, prenatal and preimplantation genetic diagnosis. Here we report the results of a genetic analysis in a 16-year-old boy from southwestern Iran diagnosed as having cystic fibrosis in infancy based on gastrointestinal and pulmonary manifestations, with positive sweat chloride tests. He lacked both normal and mutant forms of the fragment corresponding to the F508 allele in initial genetic studies. Multiplex ligationdependent probe amplification-based testing revealed a homozygous deletion spanning exons 4 to 10 of the CFTR gene. We predict an in-frame deletion removing 373 amino acids based on our sequencing results. Determining CFTR gene mutations in patients and their family members would be helpful to prevent the occurrence of new cases, especially in populations in which consanguinity is common.
文摘Patients affected by pancreatic ductal adenocarcinoma(PDAC)frequently present with advanced disease at the time of diagnosis,limiting an upfront surgical approach.Neoadjuvant treatment(NAT)has become the standard of care to downstage non-metastatic locally advanced PDAC.However,this treatment increases the risk of a nutritional status decline,which in turn,may impact therapeutic tolerance,postoperative outcomes,or even prevent the possibility of surgery.Literature on prehabilitation programs on surgical PDAC patients show a reduction of postoperative complications,length of hospital stay,and readmission rate,while data on prehabilitation in NAT patients are scarce and randomized controlled trials are still missing.Particularly,appropriate nutritional management represents an important therapeutic strategy to promote tissue healing and to enhance patient recovery after surgical trauma.In this regard,NAT may represent a new interesting window of opportunity to implement a nutritional prehabilitation program,aiming to increase the PDAC patient’s capacity to complete the planned therapy and potentially improve clinical and survival outcomes.Given these perspectives,this review attempts to provide an in-depth view of the nutritional derangements during NAT and nutritional prehabilitation program as well as their impact on PDAC patient outcomes.
文摘The objective of this study was to evaluate the association between HIV infection and hypertensive disorders of pregnancy, comparing the rates of preeclampsia and gestational hypertension in a HIV-infected pregnant group and in a HIV-negative control pregnant group matched for age and parity. Furthermore, we aimed to compare the rates of hypertensive disorders in a subgroup of HIV-positive and HIV-negative African-American Black women. Patients and Methods: This was a prospective observational cohort study conducted at two University Departments of Obstetrics and Gynecology, Salesi Hospital, Ancona, and Sant’Orsola Hospital, Bologna. The HIV-infected patients’ group consisted of 126 pregnant women;140 HIV-negative pregnant women matched for age and parity served as controls. Gestational hypertension and preeclampsia were diagnosed according to NHBPEP-ISSHP criteria. Categorical data were analyzed using the Fisher exact test. Statistical significance was set at a p value < 0.05. Results: Gestational hypertension and preeclampsia were diagnosed in 3 of 126 HIV-positive patients (2.38%) and in 14 of 140 HIV-negative patients (10%), with a relative risk of 0.24 (p = 0.0112). In the subgroup of African-American Black women, gestational hypertension and preeclampsia were diagnosed in 2 out of 43 HIV-positive (4.7%) and in 3 out of 18 HIV-negative patients (16.7%) with a relative risk of 0.28, not statistically significant (p = 0.1887). Conclusion: Pregnant women with HIV infection seem to be protected against gestational hypertension and preeclampsia and this protective effect remains also in a high risk population, such as African-American Black ethnic group. The effect is present independently from treatment received and virus copies. The lack of immune response present since the conception period should account for unopposed trophoblast invasion resulting in a better placentation.
文摘Background and Purpose: Constipation is one of the major sequelae in patients after correction of anorectal anomalies (ARAs). The aim of the present work has been to assess the colonic transit time,using radioisotope scintigraphy,in patients operated for ARA and experiencing constipation in the follow-up. The results were compared with transit time from children with true functional constipation. Methods: Twelve or 32 patients operated for ARA during the period 1994-2003 experienced mild or severe constipation (6 with high or intermediate form of ARA and 6 with low type) at follow-up. The mean age of this group was 5.8 years. Eighteen patients,mean age 6.7 years,with true functional constipation were studied as well. Colonic transit times were investigated using radioisotope scintigraphy. Normal values for colonic transit time were derived from historical controls. Radioisotope diethylenetriamine pentaacetic acid labelled with indium 111 was administered orally to determine a segmental colonic transit. Images of the abdomen have been taken at 6,24,48,and again at 72 hours,if radioactivity was not cleared from the colon. To quantify colonic transit,we calculated the geometric centre (GC) dividing the colon into anatomic regions. Results: According to normal controls,2 different type of delayed transit can be observed: (a) slow-transit constipation if GC at 48 hours is less than 4.1; (b) functional rectosigmoid obstruction (FRSO) if GC at 48 hours is 4.1 or more but less than 6.1 at 72 hours. Patients with functional constipation were divided into 2 groups: (a)-slow-transit constipation in 12 patients with a GC at 48 hours of 3.7 ± 0.5; (b) FRSO in 6 patients with a GC of 4.7 ± 0.04 and 5.02 at 48 and 72 hours,respectively. Patients operated for high ARA had values characteristic of FRSO with GC at 48 hours of 5.1 ± 0.8 and 4.75 ± 0.5 at 72 hours. In low ARA,the transit times were similar to the ones observed in patients with high ARA at 48 hours with a GC of 4.9 ± 0.5. Conclusions: Patients with ARA frequently have functional sequelae in the postoperative period such as constipation. According to our results,constipation seems to be secondary to segmental motility disorders limited to the rectosigmoid area,similar to constipated children with FRSO. No evidence of more gener-alised motility disturbance,as previously postulated,could be recorded.