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We describe 16 infants born preterm with birth weights <1500 g and transient hyperinsulinism. The onset of hyperinsulinism was delayed and often coincident with clinical stabilization. We hypothesize that postnatal stress caused by prematurity and associated problems may contribute to development of delayed-onset transient hyperinsulinism., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Background: The aim of this meta-analysis and systematic review was to evaluate the association between intraoperative bile cultures and postoperative complications of patients undergoing pancreaticoduodenectomy., Methods: A detailed literature search was performed from January 2015 to July 2022 in PubMed, Web of Science, Google Scholar, and EMBASE for related research publications. The data were extracted, screened, and graded independently. An analysis of pooled data was performed, and a risk ratio with corresponding confidence intervals was calculated and summarized., Results: A total of 8 articles were included with 1,778 pancreaticoduodenectomy patients who had an intraoperative bile culture performed. A systematic review demonstrated that some of the most common organisms isolated in a positive intraoperative bile culture were Enterococcus species, Klebsiella species, and E. coli. Four studies also showed that specific microorganisms were associated with specific postoperative complications (surgical site infection and intra-abdominal abscess). The postoperative complications that were evaluated for an association with a positive intraoperative bile culture were surgical site infections (risk ratio = 2.33, 95% confidence interval [1.47-3.69], P < .01), delayed gastric emptying (risk ratio = 1.23, 95% confidence interval [0.63-2.38], P = n.s.), 90-day mortality (risk ratio = 0.68, 95% confidence interval [0.01-52.76], P = n.s.), postoperative pancreatic hemorrhage (risk ratio = 1.70, 95% confidence interval [0.33-8.74], P = n.s.), intra-abdominal abscess (risk ratio = 1.70, 95% confidence interval [0.38-7.56], P = n.s.), and postoperative pancreatic fistula (risk ratio = 0.97, 95% confidence interval [0.72-1.32], P = n.s.)., Conclusion: The cumulative data suggest that a positive intraoperative bile culture has no association with predicting the postoperative complications of delayed gastric emptying, 90-day mortality, postoperative pancreatic hemorrhage, intra-abdominal abscess, or postoperative pancreatic fistula. However, the data also suggest that a positive intraoperative bile culture was associated with a patient developing a surgical site infection., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Background: Policies concerning the management of operatively placed drains after pancreatic surgery are still under debate. Open passive drains and closed-suction drains are both used currently in clinical practice worldwide, but there are no reliable data regarding potential differences in the postoperative outcomes associated with each drain type. The aim of the present study was to compare open passive drains and closed-suction drains with regard to postoperative contamination of the drainage fluid and overall morbidity and mortality., Methods: This study was a prospective, observational analysis of 320 consecutive, standard, partial resections (pancreaticoduodenectomy and distal pancreatectomy at a single institution from April 2016 to April 2017. Either open passive drains (n = 189, 51%) or closed-suction drains (n = 131) were used according to the operating surgeon's choice. Postoperative outcomes, including samples of drainage fluid collected on postoperative day V and sent for microbiologic analysis, were registered., Results: The open passive drain and closed-suction drain cohorts did not differ in terms of their clinical features, use of neoadjuvant chemotherapy or preoperative biliary drainage, fistula risk zone, and type of operative procedure. The overall rate of postoperative day V drainage fluid contamination (27.5% vs. 20.6%, P = .1) was similar between the groups. The same results were obtained for each specific procedure. The postoperative outcomes, namely, overall 30-day morbidity, postoperative pancreatic fistula, intra-abdominal fluid collections, percutaneous drainage, wound infections, reintervention, mean duration of hospital stay, and mortality did not differ between the 2 groups. Qualitative microbiologic analysis revealed that after pancreaticoduodenectomy, 61.5% of the bacteria contaminating the drainage fluid were attributable to human gut flora, while after distal pancreatectomy, 84.8% of the bacteria belonged to skin and mucous flora (P < .01), however, the spectrum of bacterial contamination did not significantly differ between the open passive drain and closed-suction drain cohorts., Conclusion: The use of open passive drains and closed-suction drains for major pancreatic resection does not significantly impact the postoperative outcome. The spectrum of drain contamination depends on the specific operative procedure rather than on the type of drain used., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Background: Chyle leak is an uncommon complication after pancreatic surgery. The chyle leak incidence, definition, diagnosis, and treatment had been reported heterogeneously so far. Recently a consensus definition and grading system was published by the International Study Group for Pancreatic Surgery. This study aims to evaluate the differences in the clinical and economic burden of chyle leak applying the new definition., Methods: All data from patients who underwent pancreatic surgery for any disease from January 2014 to December 2016 were retrieved from the institutional prospective database. The 2017 International Study Group for Pancreatic Surgery definition and classification were applied. The classification was validated analyzing the differences in major complications, length of stay, and hospitalization costs., Results: A total of 945 patients was the final population. A chyle leak was reported in 43 patients (4.5%). Grade A chyle leak occurred in 10 patients (23.3%), Grade B chyle leak in 31 patients (72.1%), and Grade C chyle leak in 2 patients (4.6%). Chyle leak occurred as unique postoperative complication in 29 cases (67.4%). The economic analysis showed that the average costs of the 3 grades were 2,806, 7,150 and 15,684 euros respectively (P < .001). Furthermore, the length of stay, the rates of septic events, and major complications were significantly different among the 3 grades (P = .008, P = .004, and P < .001, respectively). Of note, we did not find any intraoperative factor associated with chyle leak., Conclusion: The present study confirms the validity of the International Study Group for Pancreatic Surgery classification of chyle leak. The 3 grades of chyle leak proposed identify reliably clinical and economical differences among the chyle leak cases., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Background: Pancreatoduodenectomy sometimes causes late benign biliary complications, such as biliary stricture and/or hepaticolithiasis, which require intervention. The risk factors and timing of late biliary complications remain unclear. The purpose of this study was to clarify the incidence, timing of occurrence, and risk factors for late biliary complications after pancreatoduodenectomy., Methods: A total of 732 patients who underwent pancreatoduodenectomy between 2002 and 2016 were included in this retrospective study. Postoperative late biliary complications were defined as symptomatic benign biliary stricture and hepaticolithiasis, requiring radiologic or endoscopic intervention. Perioperative variables were collected to analyze the risk factors for late biliary complications. The treatment of late biliary complications was then evaluated., Results: A total of 28 patients (3.8%) developed late biliary complications with a median interval of 23.4 (0.7-98.9) months. Late biliary complications consisted of hepaticolithiasis (n = 11) and stricture (n = 27) (including overlap). The 5-year cumulative incidence of late biliary complications was 7.3%. A multivariate analysis showed that a common hepatic duct diameter of < 4 mm was an independent risk factor for late biliary complications. The 5-year cumulative incidence of late biliary complications in patients with a common hepatic duct diameter of < 4 mm was significantly higher than that of patients with a common hepatic duct diameter of ≥ 4 mm (27.6% vs 1.3%, P < .001). For initial treatment, endoscopic intervention (balloon dilation, lithotomy, and stenting) was successfully performed in 21 patients (75.0%), and percutaneous transhepatic intervention was performed in 7 (25.0%). No patients underwent surgery., Conclusion: The occurrence of late biliary complications was associated with a common hepatic duct diameter of < 4 mm. At the end of follow-up, high-risk patients should be adequately informed about late biliary complications and their symptoms., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Background: Despite improvements in perioperative care, mortality and morbidity rates associated with pancreatic operation still reach 2% and >50%, respectively. Infectious complications after pancreaticoduodenectomy occur in about one-third of the cases. The aim of the study is to define the real burden of infectious complications after pancreaticoduodenectomy and to analyze the risk factors associated with their onset., Methods: Data of consecutive pancreaticoduodenectomies performed at the authors' institution from January 2011 to June 2016 were retrieved from a prospectively maintained database. Based on the presence of infectious complications, the population was separated into 2 groups (infection group positive [IG+] and infection group negative [IG-]) and then compared., Results: During the study period 893 pancreaticoduodenectomies were performed. Overall, infectious complications were detected in 409 out of 893 patients (45.8%). Preoperative biliary drain was the only independent preoperative risk factor for the development of infectious complications (P < .001, odds ratio 3.8). Each complication was found to be statistically more frequent in IG+. In addition, IG+ also had a prolonged hospital stay (P < .020, odds ratio 1.1) and all deaths occurred in this group. The overall multisite infection rate was 41.6%. Multidrug-resistant bacteria were detected in 78.5% of patients. The development of multisite infection was the best predictor of outcome after pancreaticoduodenectomy., Conclusion: The development of postoperative infectious complications is a major determinant of outcome after pancreaticoduodenectomy. In this setting, the reliable negative predictors of the outcome include preoperative biliary drain, site of infection, multidrug-resistant bacteria infections and type of bacteria. Multisite infection was found to be the best predictor of a worse postoperative course after pancreaticoduodenectomy., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Background: The manifestations of non-alcoholic fatty liver disease in patients who have undergone pancreatoduodenectomy differ from those associated with obesity-related non-alcoholic fatty liver disease. This study aimed to identify factors that predicted the occurrence of and recovery from non-alcoholic fatty liver disease after pancreatoduodenectomy., Methods: This retrospective study included 120 patients who underwent pancreatoduodenectomy between April 2004 and December 2013. Non-alcoholic fatty liver disease was diagnosed using unenhanced computed tomography as a value of <40 Hounsfield units. Recovery from non-alcoholic fatty liver disease was based on increases in liver computed tomographic attenuation values. Pre-, intra-, and postoperative factors were analyzed using univariate analysis and multivariable logistic regression models., Results: Non-alcoholic fatty liver disease occurred after pancreatoduodenectomy in 45 patients (38%), and in 11 of 41 patients (27%) who received prophylactic pancreatic enzyme supplementation therapy and in 34 of 79 patients (43%) who did not (P = .082). Six patients received therapeutic supplementation after diagnosis. The non-alcoholic fatty liver disease recovery rates in patients who did and did not receive pancreatic enzyme supplementation therapy were 100% and 58%, respectively (P = .069). Multivariable analysis identified a high body mass index, small pancreatic volume, long operative time, and a high aspartate aminotransferase/alanine aminotransferase ratio 1 month after pancreatoduodenectomy as independent risk factors. A small diameter main pancreatic duct, a low serum amylase level at postoperative day 28, and a high minimum liver computed tomographic value predicted recovery from non-alcoholic fatty liver disease., Conclusion: The non-alcoholic fatty liver disease occurrence rate in patients undergoing pancreatoduodenectomy is high, but in about half of these patients, non-alcoholic fatty liver disease will resolve without any enzyme supplementation. Prophylactic supplementation in the postoperative management of pancreatoduodenectomy patients should be based on risk factors, and therapeutic supplementation should be based on recovery factors., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Background: The hospital volume-outcome relationship for complex procedures has led to the suggestion that care should be centralized. This study was performed to investigate whether centralization is occurring for pancreatoduodenectomy (PD) and to examine its effect on short-term postoperative outcomes., Methods: We queried the New York State Statewide Planning and Research Cooperative System database (n = 6,185, 2002-2011) and the California and Florida State Inpatient Databases (n = 6,766 and 4,810, respectively, 2002-2011) for PD. Hospitals were divided into low (≤10), medium (11-25), high (25-60), and very high (≥61) groups depending on annual volume. Hierarchical logistic modeling accounted for patient clustering within hospitals., Results: A migration of cases from low-volume to medium, high, and very high-volume (MHVH) hospitals occurred in these 3 states (P < .01). There was an increase in the number of MHVH hospitals and a decrease in the number of low-volume hospitals performing PD across all states over time, with a large number of hospitals ceasing to perform PD cases entirely. Comorbidities such as congestive heart failure and diabetes were more prevalent in low-volume hospitals. After we adjusted for all predictors, MHVH hospitals had less rates of mortality and morbidity and shorter durations of stay than low-volume hospitals (P < .05); 30-day readmission rates were similar across all volume groups., Conclusion: Centralization of PD is occurring in these 3 states and probably across the nation. After PD, MHVH hospitals had statistically better outcomes (mortality, morbidity, and duration of stay) than low-volume hospitals. Readmission rates were not affected by volume., (Copyright © 2016 Elsevier Inc. All rights reserved.)

The central scar is a relatively uncommon radiologic finding. However, when present, it is a highly useful imaging feature which may suggest a specific diagnosis in patients with renal, hepatic, or pancreatic masses of unknown etiology. This pictorial review will serve to illustrate the computed tomography, magnetic resonance, and ultrasonographic imaging features of abdominal neoplasms associated with a central scar.

Background: The aim of this study was to establish an optimal management strategy for pancreatic disorders associated with anomalous pancreaticobiliary junction (APBJ)., Methods: In 64 adult surgical cases of APBJ (common channel 15 mm or longer) (43 with and 21 without choledochal cyst), associated pancreatic disorders, pancreatographic results, and treatment outcomes were analyzed., Results: Of 64 patients, 56 had pancreatobiliary symptoms. Twenty-four patients (38%) had pancreatic disorders: acute pancreatitis (n = 20), chronic calcifying pancreatitis (n = 2), and pancreatic carcinoma (n = 2). Twenty patients (31%) had abnormal pancreatograms. The incidence of acute pancreatitis was significantly higher in patients with an abnormal pancreatogram, particularly dilatation, protein plugs or stones of the common channel or main pancreatic duct, and coexisting pancreatic ductal anomaly. All patients with choledochal cyst underwent cyst excision and hepaticojejunostomy. Eleven patients without choledochal cyst or pancreatobiliary carcinoma underwent cholecystectomy alone. Protein plugs and pancreatic stones were extracted through the bile duct stump or by sphincterotomy. No patients experienced pancreatitis during a mean postoperative follow-up of 6.7 years., Conclusions: In managing APBJ, attention should be paid to the possibility of associated pancreatic disorders and an abnormal pancreatogram. APBJ with choledochal cyst requires cyst excision. Cholecystectomy alone may be adequate for APBJ without cyst.

Background: Microcirculatory changes and leukocyte-endothelial interaction are both central to the pathogenesis of acute pancreatitis. We studied the effects of nitric oxide (NO) donors (intravenous or inhaled) and NO inhibitors, which affect each of these processes, on markers of experimental mild (edematous) and severe (necrotizing) pancreatitis in rats., Methods: Mild pancreatitis was induced with intravenous cerulein (n = 100) and severe pancreatitis with intravenous cerulein and intraductal glycodeoxycholic acid (n = 100). Each group was randomly divided into five equal treatment subgroups: control, NO-synthase substrate L-arginine, NO donor sodium nitroprusside, NO-synthase inhibitor N-nitro-L-arginine methyl ester (L-NAME), and NO-inhalation. After 6 hours edema was measured by a wet/dry weight ratio, and pancreatic injury was quantified by tissue levels of trypsinogen activation peptides (TAPs) and by histologic analysis of inflammation and necrosis., Results: In mild pancreatitis (1) both NO donors reduced edema formation (p < 0.001) and also reduced intrapancreatic TAPs (p < 0.03); (2) L-NAME significantly increased tissue TAPs (p < 0.03); and (3) inhaled NO had no effect. In severe pancreatitis (1) both intravenous NO donors reduced edema formation (p < 0.005) and both markedly reduced intrapancreatic TAPs (p < 0.001); (2) L-NAME did not further increase the already high tissue TAPs; and (3) inhaled NO decreased tissue TAPs (p = 0.01). Evaluation of inflammation and necrosis by histologic scoring confirmed the reduction of pancreatic injury by NO donors and worsening with NO-synthase inhibitor., Conclusions: NO donors have a beneficial effect on edema formation in acute pancreatitis but confer more important protection against ectopic trypsinogen activation, which correlates with mortality, inflammation, and necrosis. Although direct microcirculatory action is likely, the salutary effect of inhaled NO in severe pancreatitis may suggest indirect action on circulating leukocytes, which are thought to potentiate tissue injury.

Background: Pancreatic panniculitis is a rare disease in which necrosis of fat in the panniculus and other distant foci occurs in the setting of pancreatic disease., Objective: Our purpose was to study the clinical and histopathologic features of this disease., Methods: We conducted a retrospective review of 11 patients., Results: In five patients subcutaneous fat necrosis preceded the diagnosis of pancreatic disease by an average of 13 weeks. Five of our patients had underlying pancreatitis, and the remainder had carcinoma of the pancreas. Joint manifestations were prominent in six patients. Intestinal submucosal fat necrosis developed in two cases and caused massive gastrointestinal bleeding in one. Acute lesions demonstrated focal fat necrosis with "ghost cells," pathognomonic of this condition. Chronic lesions showed granulomatous and lipophagic panniculitis, with rare areas of basophilic fat necrosis. Cholecystectomy for gallstone pancreatitis was curative in two patients., Conclusion: Pancreatic panniculitis has distinctive clinical and pathognomonic histopathologic findings that can be the presenting features of pancreatic disease.

Data from 17,857 patients with cystic fibrosis submitted in 1990 to the registry maintained by the Cystic Fibrosis Foundation were used to described their demographic characteristics, survival rates, pulmonary function, anthropometry, microbiologic data, complication rates, and health care utilization. Comparisons with similar data collected in 1969, 1972, and 1978 demonstrated a significant shift in the age distribution of patients with cystic fibrosis. The proportion of adult patients increased fourfold between 1969 (8%) and 1990 (33%). In 1990 the median age of all patients in the cystic fibrosis registry was 12.5 years; the median age at diagnosis was 7 months; cystic fibrosis was diagnosed in 90% of all patients by age 12 years. Meconium ileus at birth was reported for 16% of all patients with a new diagnosis in 1990. Median survival age doubled between 1969 and 1990, from 14 to 28 years. Female patients consistently had a lower median survival age than male patients (25 vs 30 years in 1990). The most frequently reported respiratory pathogen was Pseudomonas aeruginosa, cultured in specimens from 61% of all patients, ranging from 21% of those less than 1 year of age to more than 80% of those aged 26 years or older. Overall, patients with cystic fibrosis are living much longer than in the past but still have chronic pulmonary infections and other medical complications related to their disease, including diabetes, intestinal obstruction, cirrhosis, hemoptysis, and pneumothorax.

An infant with persistent hyperinsulinism had hypertrophic cardiomyopathy that progressed until near-total pancreatectomy was performed. After pancreatectomy the condition resolved. Hyperinsulinism may be a cause of treatable cardiomyopathy.

Severe neonatal hypoglycemia with pathologic findings of diffuse nesidoblastosis of the pancreas is described in five children of both sexes from two families with unaffected parents. This appears to represent an autosomal recessive disorder of pancreatic development. Despite extensive testing, the diagnosis of hyperinsulinism was difficult in the index case of each family and delayed definitive treatment. Medical therapy with steroids and diazoxide was unsuccessful; pancreatectomy was required to treat persistent hypoglycemia. An abnormality of circulating glucagon found in one child with this disorder suggested that hyperinsulinism may not be the sole hormonal imbalance present, but rather that this disease is one of generalized disturbance of islet cell function. The history of severe, persistent neonatal hypoglycemia in an older sibling should lead the physician to investigate subsequent children for the presence of asymptomatic hypoglycemia.

In a personal series of 188 patients with pancreatic disease referred for endoscopic retrograde cholangiography (ERCP), one or more ducts were cannulated in 168 patients; of the 168 patients; of the 168 patients, 19 had carcinoma and 149 had chronic pancreatitis. Abnormalities were found in 71 of the 149 patients with pancreatitis, either in the pancreatic duct, the bile duct, or both ducts. The demonstration of these abnormalities was of critical importance in planning the management of these patients. Ducts were normal in 78 patients. No benefit could be expected from operating upon these patients. It is concluded that approximately one half of the patients with chronic pancreatitis will have demonstrable lesions amenable to operation, and one half will not. ERCP is essential in the investigation and management of patients suspected of having chronic pancreatitis.

The rare instance of a gastrointestinal hemorrhage caused by a lithiasis of the duct of Wirsung is described. The world literature records only 13 other cases. Pancreatic calculi cause hemorrhage by either migrating through the pancreatic parenchyma to perforate the duodenum, ulcerating the vessels of the periductal parenchyma, or rupturing the pancreas. The usual sign of this complication of chronic calcifying pancreatitis, difficult to diagnose, is repeated gastrointestinal hemorrhages and sometimes pain, as in epigastric colitis. The immediate therapy is control of the hemorrhage. With transcatheter occlusive techniques, radical surgery can be postponed until it is more tolerable.

Massive hemorrhage associated with pancreatitis is a rare but frequently lethal complication. Fifteen patients with this complication are presented. Bleeding occurred in four patients with necrotizing pancreatitis, in three patients with pancreatic abscesses, in seven patients with pseudocysts, and in one patient with chronic relapsing pancreatitis following longitudinal pancreaticojejunostomy. The initial presentation of hemorrhage was gastrointestinal in eight patients and retroperitoneal or intraperitoneal in seven. Abdominal pain with associated nausea and vomiting was present in all patients on admission. Duration of symptoms prior to hospitalization averaged 6 days. During hospitalization the 15 patients received a total of 512 units of blood for transfusions ranging from 8 to 177 units. Admission amylase values were of no benefit in assessing severity of the disease, but application of Ranson's criteria accurately predicted both severity and prognosis. The common denominator in all cases of bleeding appeared to be the presence of an overwhelming or continuing inflammatory process with necrosis and erosion of adjacent vascular and visceral structures. The overall mortality rate in the series was 53.3%. Those patients with hemorrhage associated with pseudocyst formation had the highest survival rates, whereas those with necrotizing pancreatitis and hemorrhage had an extremely poor response to aggressive medical and/or surgical management.

In the past decade, we have studied four unrelated children with what we believe is a previously unreported disorder affecting the bone marrow and exocrine pancreas. During infancy these patients had the onset of severe, transfusion-dependent, macrocytic anemia plus a variable degree of neutropenia and thrombocytopenia. Their bone marrows had normal cellularity but were characterized by remarkable vacuolization of erythroid and myeloid precursors, hemosiderosis, and ringed sideroblasts. The vacuoles probably represented manifestations of cellular degeneration and death. In two patients, in vitro bone marrow cultures showed abnormal erythroid and myeloid progenitor cell growth and, in one child, abnormal vacuolated erythroid colonies. Family histories were unrevealing, parents were hematologically normal, and both sexes were involved. There was no evidence of specific nutritional deficiencies or exposure to agents associated with marrow vacuolization. A number of therapeutic interventions produced no effect. One child had clinical malabsorption. This child and one other had extensive pancreatic fibrosis at autopsy. The other two patients had findings indicating exocrine pancreatic dysfunction. Two children had splenic atrophy. This new syndrome, with associated bone marrow and exocrine pancreatic dysfunctions, differs in several respects from the syndrome of pancreatic liposis and neutropenia described by Shwachman et all and Bodian et al, and from other conditions with vacuolization of the marrow or sideroblastosis.