Mucinous Cystadenocarcinoma of the Pancreas in an Adolescent with Cystic Fibrosis

2005 ◽  
Vol 8 (3) ◽  
pp. 391-396 ◽  
Author(s):  
Christopher M. Oermann ◽  
Qasem Al-Salmi ◽  
Dan K. Seilheimer ◽  
Milton Finegold ◽  
Nina Tatevian
Keyword(s):  
Cystic Fibrosis ◽  
Case Reports ◽  
Epidemiologic Studies ◽  
Therapeutic Interventions ◽  
Mucinous Cystadenocarcinoma ◽  
Pancreatic Cysts ◽  
Pancreatic Cancers ◽  
Increased Risk ◽  
Risk Of Malignancy ◽  
Biliary Tracts

Epidemiologic studies indicate that the overall risk of malignancy among patients with cystic fibrosis (CF) is similar to that of the general population. However, these studies and a number of case reports suggest that patients with CF may be at increased risk for the development of specific gastrointestinal cancers. Tumors of the esophagus, stomach, small and large bowels, liver and biliary tracts, and pancreas have been described. Previous reports of pancreatic cancers among patients with CF have included only adenocarcinoma in adults. This is the first description of a mucinous cystadenocarcinoma of the pancreas in an adolescent with CF. The tumor developed within a pancreatic cyst that had been initially identified 13 years before resection. Our report highlights the increased risk of pancreatic malignancy among patients who have CF and illustrates the premalignant potential of pancreatic cysts in this at-risk population. Further, it reinforces the need for careful surveillance and suggests a role for more aggressive diagnostic and therapeutic interventions for patients with atypical findings on pancreatic imaging studies.

scholarly journals Radiation dose from common radiological investigations and cumulative exposure in children with cystic fibrosis: an observational study from a single UK centre

BMJ Open ◽  
2017 ◽  
Vol 7 (8) ◽  
pp. e017548 ◽  
Author(s):  
Rebecca Ward ◽  
William D Carroll ◽  
Paula Cunningham ◽  
Sheng-Ang Ho ◽  
Mary Jones ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Radiation Dose ◽  
Radiation Exposure ◽  
Cumulative Exposure ◽  
X Rays ◽  
Modern Equipment ◽  
Increased Risk ◽  
Risk Of Malignancy ◽  
The Mean ◽  
Cumulative Radiation Exposure

ObjectivesCumulative radiation exposure is associated with increased risk of malignancy. This is important in cystic fibrosis (CF) as frequent imaging is required to monitor disease progression and diagnose complications. Previous estimates of cumulative radiation are outdated as the imaging was performed on older equipment likely to deliver higher radiation. Our objectives were to determine the radiation dose delivered to children during common radiological investigations using modern equipment and to identify the number of such investigations performed in a cohort of children with CF to calculate their cumulative radiation exposure.Design, setting and participantsData including age at investigation and radiation exposure measured as estimated effective dose (EED) were collected on 2827 radiological studies performed on children at one UK paediatric centre. These were combined with the details of all radiological investigations performed on 65 children with CF attending the same centre to enable calculation of each child’s cumulative radiation exposure.ResultsThe mean EED for the common radiological investigations varied according to age. The range was 0.01–0.02 mSv for chest X-rays, 0.03–0.11 mSv for abdominal X-rays, 0.57–1.69 mSv for CT chest, 2.9–3.9 mSv for abdominal and pelvic CT, 0.20–0.21 mSv for sinus CT and 0.15–0.52 mSv for fluoroscopy-guided procedures. The mean EED was three to five times higher for helical compared with axial chest CT scans. The mean annual cumulative EED for our cohort of children with CF was 0.15 mSv/year with an estimated cumulative paediatric lifetime EED (0–18 years) of 3.5 mSv.ConclusionsThis study provides up-to-date estimations of the radiation exposure when using common radiological investigations. These doses and the estimates of cumulative radiation exposure in children with CF are lower than previously reported. This reflects the reduced EED associated with modern equipment and the use of age-specific scanning protocols.

scholarly journals Colorectal Microcarcinoids in Association with Long-Term Exposure to Urinary Content: A Case Report and Review of the Literature

2015 ◽  
Vol 2015 ◽  
pp. 1-4
Author(s):  
Grace W. Weyant ◽  
Dipti M. Karamchandani ◽  
Negar Rassaei
Keyword(s):  
Case Report ◽  
Urinary Tract ◽  
Colonic Mucosa ◽  
Case Reports ◽  
Urothelial Cell Carcinoma ◽  
Cell Hyperplasia ◽  
Increased Risk ◽  
Urinary Content ◽  
Risk Of Malignancy

Long-term exposure of colonic mucosa to urinary content and its association with increased risk of infection, mechanical and biochemical irritation, and malignancy have been described in the literature. Existing case reports and studies depict the low but distinct risk of malignancy in gastrointestinal segments which come in contact with urinary content as a result of surgical correction of urinary tract abnormalities. However, these reports are largely limited to colonic adenocarcinoma and urothelial cell carcinoma. Late urointestinal carcinoma in patients with ileal incorporation into the urinary tract has also been reported. To the best of our knowledge, however, there is only one case report documenting neuroendocrine (NE) cell hyperplasia in colonic mucosa after long-term cystoplasty. Our case is the first to describe microcarcinoids and diffuse NE hyperplasia occurring in a patient with congenital anorectal anomalies, resulting in long-term exposure of colonic mucosa to fecal stream and urinary content. This case, in conjunction with the reported cases in the literature, raises the distinct possibility of an association between exposure of colonic mucosa to urine and long-term development of malignancy, including NE neoplasms.

scholarly journals Branch Duct Intraductal Papillary Mucinous Neoplasms: Recommendations for Follow-Up and Surgery

2020 ◽  
Vol 109 (1) ◽  
pp. 34-41
Author(s):  
A. Caravati ◽  
S. Andrianello ◽  
T. Pollini ◽  
M. Biancotto ◽  
A. Balduzzi ◽  
...  
Keyword(s):  
High Volume ◽  
Cystic Neoplasm ◽  
Pancreatic Cysts ◽  
Intraductal Papillary Mucinous Neoplasms ◽  
Branch Duct ◽  
Cystic Neoplasms ◽  
Increased Risk ◽  
Mucinous Neoplasms ◽  
Risk Of Malignancy

Background and Aims: Pancreatic cysts are increasingly diagnosed, mainly during abdominal imaging performed for other reasons. Between pancreatic cystic neoplasm, intraductal papillary mucinous neoplasms are the most common pre-malignant entities. Intraductal papillary mucinous neoplasms involving side branches overall harbor a low risk of malignancy, and in the recent past, a progressively more conservative approach has been consolidated. Purpose of this report is to summarize the evidence supporting the current practice for the management of branch duct intraductal papillary mucinous neoplasm and to offer a useful practical guide from first observation to post-operative follow-up. Materials and Methods: Review of the most important scientific literature on intraductal papillary mucinous neoplasms was made. In this review article, we also report the experience of a high volume center in managing Pancreatic cystic neoplasms. Results: The correct management during surveillance still is a matter of debate, since many guidelines have been published suggesting different clinical approaches. Recently, follow-up discontinuation has also been proposed in selected cases. Conclusion: Despite significant improvements made by the increase of evidence, selecting surgical candidates because of an increased risk of malignant progression remains an unsolved issue and a hot topic for pancreatologists.

Association of HMG-CoA Reductase Inhibitors with Neuropathy

2003 ◽  
Vol 37 (2) ◽  
pp. 274-278 ◽  
Author(s):  
James M Backes ◽  
Patricia A Howard
Keyword(s):  
Peripheral Neuropathy ◽  
Case Reports ◽  
Epidemiologic Studies ◽  
Term Therapy ◽  
Hmg Coa Reductase ◽  
Reductase Inhibitors ◽  
Increased Risk ◽  
Hmg Coa Reductase Inhibitors ◽  
Coa Reductase ◽  
Long Term Therapy

OBJECTIVE: To review the possible association between statins and peripheral neuropathy. DATA SOURCES: Literature was obtained from MEDLINE (1984–September 2002) and International Pharmaceutical Abstracts (1970–June 2002). Key search terms included statin, neuropathy, and HMG-CoA reductase inhibitor. DATA SYNTHESIS: Epidemiologic studies and case reports suggest an increased risk of peripheral neuropathy with statin drugs. Most patients were receiving long-term therapy, although the onset was highly variable. The majority of cases were at least partially reversible with drug cessation. Specific risk factors or mechanisms have not been identified. CONCLUSIONS: Observational data suggest a link between chronic statin use and increased risk of peripheral neuropathy. However, the risk appears to be small relative to the significant cardioprotective benefits.

scholarly journals SPLUNC1: A Novel Marker of Cystic Fibrosis Exacerbations

2020 ◽  
Author(s):  
Sara Khanal ◽  
Megan Webster ◽  
Naiqian Niu ◽  
Myra Nunez ◽  
Geoffrey Chupp ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Lung Function ◽  
Standard Error ◽  
Hazard Ratio ◽  
Therapeutic Interventions ◽  
Lung Function Decline ◽  
Markers Of Inflammation ◽  
Increased Risk ◽  
Healthy Control ◽  
One Year

Acute pulmonary Exacerbations (AE) are episodes of clinical worsening in cystic fibrosis (CF), often precipitated by infection. Timely detection is critical to minimize the morbidity and lung function decline associated with acute inflammation during AE. We previously demonstrated that the airway protein Short Palate Lung Nasal epithelium Clone 1 (SPLUNC1) is regulated by inflammatory signals. Here, we investigated the use of SPLUNC1 fluctuations to diagnose and predict AE in CF. We enrolled adult CF subjects from two independent cohorts to measure AE markers of inflammation in sputum and recorded clinical outcomes for a 1-year follow-up period. SPLUNC1 levels were high in healthy control sputum (n=9, 10.7µg/mL), and significantly decreased in CF subjects without AE (n=30, 5.7µg/mL, p=0.016). SPLUNC1 levels were 71.9% lower during AE (n=14, 1.6µg/mL, p=0.0034) regardless of age, sex, CF-causing mutation, or microbiology findings. Cytokines Il-1β and TNFα were also increased in AE, whereas lung function did not consistently decrease. Stable CF subjects with lower SPLUNC1 levels were much more likely to have an AE at 60 days (Hazard Ratio: 11.49, Standard Error: 0.83, p=0.0033). Low-SPLUNC1 stable subjects remained at higher AE risk even one year after sputum collection (Hazard Ratio: 3.21, Standard Error: 0.47, p=0.0125). SPLUNC1 was transcriptionally downregulated by inflammatory cytokines and degraded by proteases increased in sputum during AE. Our findings suggest that low sputum SPLUNC1 levels could detect subjects at increased risk of AE in order to guide early therapeutic interventions in CF.

Bowel ischaemia after endovascular aneurysm repair

VASA ◽  
2018 ◽  
Vol 47 (4) ◽  
pp. 273-277
Author(s):  
Christopher Lowe ◽  
Oussama El Bakbachi ◽  
Damian Kelleher ◽  
Imran Asghar ◽  
Francesco Torella ◽  
...  
Keyword(s):  
Case Reports ◽  
Endovascular Aneurysm Repair ◽  
Artery Occlusion ◽  
Bibliographic Databases ◽  
Bowel Ischaemia ◽  
Prophylactic Measures ◽  
Increased Risk ◽  
Prisma Statement ◽  
Meta Analyses ◽  
Aneurysm Repair

Abstract. The aim of this review was to investigate presentation, aetiology, management, and outcomes of bowel ischaemia following EVAR. We present a case report and searched electronic bibliographic databases to identify published reports of bowel ischaemia following elective infra-renal EVAR not involving hypogastric artery coverage or iliac branch devices. We conducted our review according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) statement standards. In total, five cohort studies and three case reports were included. These studies detailed some 6,184 infra-renal elective EVARs, without procedure-related occlusion of the hypogastric arteries, performed between 1996 and 2014. Bowel ischaemia in this setting is uncommon with an incidence ranging from 0.5 to 2.8 % and includes a spectrum of severity from mucosal to transmural ischaemia. Due to varying reporting standards, an overall proportion of patients requiring bowel resection could not be ascertained. In the larger series, mortality ranged from 35 to 80 %. Atheroembolization, hypotension, and inferior mesenteric artery occlusion were reported as potential causative factors. Elderly patients and those undergoing prolonged procedures appear at higher risk. Bowel ischaemia is a rare but potentially devastating complication following elective infra-renal EVAR and can occur in the setting of patent mesenteric vessels and hypogastric arteries. Mortality ranges from 35 to 80 %. Further research is required to identify risk factors and establish prophylactic measures in patients that have an increased risk of developing bowel ischaemia after standard infra-renal EVAR.

The Need for Personalized Risk-Stratified Approaches to Treatment for Gestational Diabetes: A Narrative Review

2021 ◽  
Author(s):  
Shamil D. Cooray ◽  
Jacqueline A. Boyle ◽  
Georgia Soldatos ◽  
Shakila Thangaratinam ◽  
Helena J. Teede
Keyword(s):  
Gestational Diabetes ◽  
Pregnancy Outcomes ◽  
Population Based ◽  
Adverse Pregnancy Outcomes ◽  
Therapeutic Interventions ◽  
Clinical Prediction Model ◽  
Increased Risk ◽  
Adverse Pregnancy ◽  
The Individual ◽  
Personalized Risk

AbstractGestational diabetes mellitus (GDM) is common and is associated with an increased risk of adverse pregnancy outcomes. However, the prevailing one-size-fits-all approach that treats all women with GDM as having equivalent risk needs revision, given the clinical heterogeneity of GDM, the limitations of a population-based approach to risk, and the need to move beyond a glucocentric focus to address other intersecting risk factors. To address these challenges, we propose using a clinical prediction model for adverse pregnancy outcomes to guide risk-stratified approaches to treatment tailored to the individual needs of women with GDM. This will allow preventative and therapeutic interventions to be delivered to those who will maximally benefit, sparing expense, and harm for those at a lower risk.

scholarly journals Haemostatic and thrombo-embolic complications in pregnant women with COVID-19: a systematic review and critical analysis

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Juliette Servante ◽  
Gill Swallow ◽  
Jim G. Thornton ◽  
Bethan Myers ◽  
Sandhya Munireddy ◽  
...  
Keyword(s):  
Venous Thrombosis ◽  
Pregnant Women ◽  
Clinical Evidence ◽  
Case Reports ◽  
Standard Form ◽  
Test Results ◽  
Maternal Deaths ◽  
High Clinical Suspicion ◽  
Increased Risk ◽  
Definition Of

Abstract Background As pregnancy is a physiological prothrombotic state, pregnant women may be at increased risk of developing coagulopathic and/or thromboembolic complications associated with COVID-19. Methods Two biomedical databases were searched between September 2019 and June 2020 for case reports and series of pregnant women with a diagnosis of COVID-19 based either on a positive swab or high clinical suspicion where no swab had been performed. Additional registry cases known to the authors were included. Steps were taken to minimise duplicate patients. Information on coagulopathy based on abnormal coagulation test results or clinical evidence of disseminated intravascular coagulation (DIC), and on arterial or venous thrombosis, were extracted using a standard form. If available, detailed laboratory results and information on maternal outcomes were analysed. Results One thousand sixty-three women met the inclusion criteria, of which three (0.28, 95% CI 0.0 to 0.6) had arterial and/or venous thrombosis, seven (0.66, 95% CI 0.17 to 1.1) had DIC, and a further three (0.28, 95% CI 0.0 to 0.6) had coagulopathy without meeting the definition of DIC. Five hundred and thirty-seven women (56%) had been reported as having given birth and 426 (40%) as having an ongoing pregnancy. There were 17 (1.6, 95% CI 0.85 to 2.3) maternal deaths in which DIC was reported as a factor in two. Conclusions Our data suggests that coagulopathy and thromboembolism are both increased in pregnancies affected by COVID-19. Detection of the former may be useful in the identification of women at risk of deterioration.

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