Pancreatic exocrine insufficiency (PEI) can be induced by various kinds of diseases, including chronic pancreatitis, acute pancreatitis, and post-pancreatectomy. The main pathogenetic mechanism of PEI involves the decline of trypsin synthesis, disorder of pancreatic fluid flow, and imbalance of secretion feedback. Animal studies have shown that PEI could induce gut bacterial overgrowth and dysbiosis, with the abundance of Lactobacillus and Bifidobacterium increasing the most, which could be partially reversed by pancreatic enzyme replacement therapy. Clinical studies have also confirmed the association between PEI and the dysbiosis of gut microbiota. Pancreatic exocrine secretions and changes in duodenal pH as well as bile salt malabsorption brought about by PEI may affect and shape the abundance and composition of gut microbiota. In turn, the gut microbiota may impact the pancreatic exocrine acinus through potential bidirectional crosstalk. Going forward, more and higher-quality studies are needed that focus on the mechanism underlying the impact of PEI on the gut microbiota.
胰腺外分泌功能不全（PEI）可由多种疾病引起，包括慢性胰腺炎、急性胰腺炎、胰腺切除术后等。PEI其主要发病机制与胰蛋白酶合成下降、胰液流动紊乱、分泌反馈失衡有关。动物研究表明：PEI可诱导肠道细菌过度生长和生态失调，其中乳杆菌和双歧杆菌的丰度增加最为多见；肠道细菌过度生长和生态失调可通过胰酶替代治疗得到部分逆转。临床研究也证实PEI与肠道菌群失调之间存在关联。胰脏外分泌水平的下降，伴随PEI产生的十二指肠pH值的改变，以及PEI引起的胆盐吸收不良，这可能是PEI导致肠道菌群丰度和组成发生改变的潜在作用机制。反之，肠道微生物群也可能通过潜在的双向调节影响胰腺外分泌腺泡的功能。展望未来，仍需要更多的高质量研究来揭示胰腺外分泌不足对肠道微生物群影响的机制。.

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Pancreatic enzyme replacement therapy (PERT) has been recommended as the preferred method for pancreatic exocrine insufficiency caused by chronic pancreatitis (CP). However, at present, the patient-related factors for the poor PERT management are not clear, and there are no studies on the adherence to PERT in patients with CP in East China. This was a mixed-method study following the principle of sequential explanatory design and included two parts: a quantitative and qualitative study. A cross-sectional survey of medication adherence (MA) was first carried out, followed by a semi-structured interview to further explore and explain the influencing factors of adherence to PERT. Of the 148 patients included in this study, 48.0% had poor MA and only 12.8% had good MA. Multivariate logistic regression showed that lower levels of education and income were contributing factors for non-adherence to PERT. Semi-structured interviews with 24 patients revealed that the reasons for non-adherence also included lack of knowledge, self-adjustment of PERT, lifetime of medication, side effects of PERT, forgetfulness, financial burdens, and accessibility issues. The adherence to PERT was poor among patients with CP in East China. Healthcare providers should personalize medication strategies to improve patients\' MA.

Shwachman-Diamond syndrome (SDS) is an autosomal recessive disease which results in inherited bone marrow failure (IBMF) and is characterized by exocrine pancreatic dysfunction and diverse clinical phenotypes. In the present study, we reviewed the internationally published reports on SDS patients, in order to summarize the clinical features, epidemiology, and treatment of SDS.
We searched the WangFang and China National Knowledge Infrastructure databases with the keywords \"Shwachman-Diamond syndrome,\" \"SDS,\" \"SBDS gene\" and \"inherited bone marrow failure\" for relevant articles published from January 2002 to October 2022. In addition, studies published from January 2002 to October 2022 were searched from the Web of Science, PubMed, and MEDLINE databases, using \"Shwachman-diamond syndrome\" as the keyword. Finally, one child with SDS treated in Tongji Hospital was also included.
The clinical features of 156 patients with SDS were summarized. The three major clinical features of SDS were found to be peripheral blood cytopenia (96.8%), exocrine pancreatic dysfunction (83.3%), and failure to thrive (83.3%). The detection rate of SDS mutations was 94.6% (125/132). Mutations in SBDS, DNAJC21, SRP54, ELF6, and ELF1 have been reported. The male-to-female ratio was approximately 1.3/1. The median age of onset was 0.16 years, but the diagnostic age lagged by a median age of 1.3 years.
Pancreatic exocrine insufficiency and growth failure were common initial symptoms. SDS onset occurred early in childhood, and individual differences were obvious. Comprehensive collection and analysis of case-related data can help clinicians understand the clinical characteristics of SDS, which may improve early diagnosis and promote effective clinical intervention.

OBJECTIVE: To explore the characteristics of Shwachman-Diamond syndrome (SDS) in Chinese children in order to provide a reference for early diagnosis.
METHODS: With Shwachman-Diamond syndrome, SDS, SBDS gene and inherited bone marrow failure as the keywords, the search period was set from January 2002 to October 2022. Relevant literature was retrieved from the Wanfang Database and China National Knowledge Infrastructure (CNKI) database. In addition, by using Shwachman-diamond syndrome as a keyword, the search period was also retrieved from the Web of Science, PubMed, and MEDLINE databases from January 2002 to October 2022. A child with SDS treated at the Tongji Hospital was also included. A total of 44 cases with complete clinical data were analyzed with reference to the International Standard for SDS Diagnosis. Chi-square test and t test were used for statistical analysis. Evidence-based research was carried out in the form of systematic review. The epidemiology, clinical characteristics and key points of early diagnosis of the Chinese SDS children were summarized and compared with the international data.
RESULTS: The main characteristics of SDS in Chinese children were summarized as follows: The ratio of males to females was about 1.3 : 1, the median age of onset was 3 months, and the median age of diagnosis was 14 months. The first symptoms were often exocrine pancreatic insufficiency (31.8%) and granulocytopenia with infection (31.8%). According to the international consensus, the incidence rates of the three major diseases of SDS were hemocytopenia (95.4%), pancreatic disease (72.7%), and bone abnormality (40.9%). The common factors underlying SDS disease were variants of the SBDS gene (c.258+2T>C and c.183_184TA>CT), albeit there was no significant correlation between genotype and phenotype (P > 0.05). Compared with international reports, the clinical manifestations and genotypes of Chinese SDS children are different (P < 0.05).
CONCLUSIONS: The SDS children have an early age of onset and significant individual difference. It is necessary to analyze the case-related data to facilitate early recognition, diagnosis and clinical intervention.

BACKGROUND: Pancreatic endocrine insufficiency is more likely to occur after acute pancreatitis (AP), but the risk factors affecting pancreatic endocrine function remain controversial. Therefore, exploring the incidence and risk factors of fasting hyperglycaemia following first-attack AP is important.
METHODS: Data were collected from 311 individuals with first-attack AP without previous diabetes mellitus (DM) or impaired fasting glucose (IFG) history treated in the Renmin Hospital of Wuhan University. Relevant statistical tests were performed. A two-sided p-value < 0.05 was considered statistically significant.
RESULTS: The incidence of fasting hyperglycaemia in individuals with first-attack AP was 45.3%. Univariate analysis showed that age (χ2 = 6.27, P = 0.012), aetiology (χ2 = 11.184, P = 0.004), serum total cholesterol (TC) (χ2 = 14.622, P < 0.001), and serum triglyceride (TG) (χ2 = 15.006, P < 0.001) were significantly different between the hyperglycaemia and non-hyperglycaemia groups (P < 0.05). The serum calcium concentration (Z=-2.480, P = 0.013) was significantly different between the two groups (P < 0.05). Multiple logistic regression analysis showed that age- ≥60 years (P < 0.001, OR = 2.631, 95%Cl = 1.529-4.527) and TG ≥ 5.65 mmol/L (P < 0.001, OR = 3.964, 95%Cl = 1.990-7.895) were independent risk factors for fasting hyperglycaemia in individuals with first-attack AP (P < 0.05).
CONCLUSIONS: Old age, serum triglycerides, serum total cholesterol, hypocalcaemia, and aetiology are associated with fasting hyperglycaemia following first-attack AP. Age ≥ 60 years and TG ≥ 5.65 mmol/L are independent risk factors for fasting hyperglycaemia following first-attack AP.

OBJECTIVE: To develop and validate a radiomics nomogram based on a fully automated pancreas segmentation to assess pancreatic exocrine function. Furthermore, we aimed to compare the performance of the radiomics nomogram with the pancreatic flow output rate (PFR) and conclude on the replacement of secretin-enhanced magnetic resonance cholangiopancreatography (S-MRCP) by the radiomics nomogram for pancreatic exocrine function assessment.
METHODS: All participants underwent S-MRCP between April 2011 and December 2014 in this retrospective study. PFR was quantified using S-MRCP. Participants were divided into normal and pancreatic exocrine insufficiency (PEI) groups using the cut-off of 200 µg/L of fecal elastase-1. Two prediction models were developed including the clinical and non-enhanced T1-weighted imaging radiomics model. A multivariate logistic regression analysis was conducted to develop the prediction models. The models\' performances were determined based on their discrimination, calibration, and clinical utility.
RESULTS: A total of 159 participants (mean age [Formula: see text] standard deviation, 45 years [Formula: see text] 14;119 men) included 85 normal and 74 PEI. All the participants were divided into a training set comprising 119 consecutive patients and an independent validation set comprising 40 consecutive patients. The radiomics score was an independent risk factor for PEI (odds ratio = 11.69; p < 0.001). In the validation set, the radiomics nomogram exhibited the highest performance (AUC, 0.92) in PEI prediction, whereas the clinical nomogram and PFR had AUCs of 0.79 and 0.78, respectively.
CONCLUSIONS: The radiomics nomogram accurately predicted pancreatic exocrine function and outperformed pancreatic flow output rate on S-MRCP in patients with chronic pancreatitis.
CONCLUSIONS: • The clinical nomogram exhibited moderate performance in diagnosing pancreatic exocrine insufficiency. • The radiomics score was an independent risk factor for pancreatic exocrine insufficiency, and every point rise in the rad-score was associated with an 11.69-fold increase in pancreatic exocrine insufficiency risk. • The radiomics nomogram accurately predicted pancreatic exocrine function and outperformed the clinical model and pancreatic flow output rate quantified by secretin-enhanced magnetic resonance cholangiopancreatography on MRI in patients with chronic pancreatitis.

BACKGROUND: Nowadays, minimally invasive intervention (MII) has largely replaced delayed open surgery in acute necrotizing pancreatitis (ANP). However, the timing of MII remains unclear. The present study investigated the effect of early versus delayed MII on complications in ANP.
METHODS: Studies evaluating the impact of the timing of MII on complications in ANP patients were thoroughly searched on PubMed, Embase, Cochrane Library, and Web of Science from inception to June 2022. The primary outcome of interest was mortality. Secondary outcomes were the incidence of complications.
RESULTS: Nine studies reporting 870 patients undergoing MII for ANP were included. No significant difference was found in mortality between the early and delayed intervention groups. In addition, the timing of MII was not associated with the incidence of new-onset respiratory failure, new-onset cardiovascular failure, new-onset renal failure, new-onset multiple organ failure, gastrointestinal fistula or perforation, pancreatic fistula, stent migration, bleeding, venous thrombosis, and new-onset pancreatic endocrine insufficiency. Notably, in the subgroup analysis of biliary and Asian ANP patients, early intervention was associated with a significantly higher risk of new-onset renal failure than delayed intervention.
CONCLUSIONS: Early intervention is safe and recommended only for patients with indications for intervention, such as infection.

Objective: To investigate the clinical phenotypes and genotypic spectrum of exocrine pancreatic insufficiency in children with cystic fibrosis. Methods: This was a retrospective analysis of 12 children with cystic fibrosis who presented to Children\'s Hospital of Fudan University from December 2017 to December 2021. Clinical features, fecal elastase-1 level, genotype, diagnosis and treatment were systematically reviewed. Results: A total of 12 children, 7 males and 5 females, diagnosis aged 5.4 (2.0, 10.6) years, were recruited. Common clinical features included chronic cough in 12 cases, malnutrition in 7 cases, steatorrhea in 7 cases, bronchiectasis in 5 cases and electrolyte disturbance in 4 cases. Exocrine pancreatic insufficiency were diagnosed in 8 cases,the main clinical manifestations were steatorrhea in 7 cases, of which 5 cases started in infancy; 6 cases were complicated with malnutrition, including mild in 1 case, moderate in 2 cases and severe in 3 cases; 3 cases had abdominal distension; 2 cases had intermittent abdominal pain; 4 cases showed fatty infiltration or atrophy of pancreas and 3 cases showed no obvious abnormality by pancreatic magnetic resonance imaging or B-ultrasound. All 8 children were given pancreatic enzyme replacement therapy, follow-up visit of 2.3 (1.2,3.2) years. Diarrhea significantly improved in 6 cases, and 1 case was added omeprazole due to poor efficacy. A total of 20 variations of CFTR were detected in this study, of which 7 were novel (c.1373G>A,c.1810A>C,c.270delA,c.2475_2478dupCGAA,c.2489_c.2490insA, c.884delT and exon 1 deletion). Conclusions: There is a high proportion of exocrine pancreatic insufficiency in Chinese patients with cystic fibrosis. The main clinical manifestations are steatorrhea and malnutrition. Steatorrhea has often started from infancy. Pancreatic enzyme replacement therapy can significantly improve the symptoms of diarrhea and malnutrition.
目的： 探讨囊性纤维化患儿中胰腺外分泌功能不全的临床及基因型特征。 方法： 回顾性分析复旦大学附属儿科医院2017年12月至2021年12月经汗液氯离子浓度及全外显子测序确诊的12例囊性纤维化患儿的临床表型、粪便弹性蛋白酶1水平、基因型、诊断及治疗情况等信息，对其临床表型和基因型进行总结。 结果： 12例患儿中男7例、女5例，确诊年龄5.4（2.0，10.6）岁，常见临床表现包括慢性咳嗽12例、营养不良7例、脂肪泻7例、支气管扩张5例、低钾低氯性碱中毒4例。8例患儿合并胰腺外分泌功能不全，主要的临床表现为脂肪泻7例，其中5例起病于婴儿期；营养不良6例，包括轻度1例、中度2例、重度3例；3例患儿有腹胀；2例患儿有间断腹痛；7例患儿完善胰腺磁共振成像或B超，4例提示胰腺脂肪浸润或体积缩小，3例未见明显异常；8例患儿均给予胰酶替代治疗，随访2.3（1.2，3.2）年，6例患儿脂肪泻症状明显改善，1例因疗效不佳而加用奥美拉唑。共检测出20个CFTR基因变异位点，其中7个为新发现变异（c.1373G>A、c.1810A>C、c.270delA、c.2475_2478dupCGAA、c.2489_c.2490insA、c.884delT、1号外显子缺失）。 结论： 我国囊性纤维化患儿多存在胰腺外分泌功能不全，临床以婴儿期起病的脂肪泻及营养不良为主要表现。胰酶替代治疗能显著改善腹泻及营养不良症状。.

OBJECTIVE: Steatorrhea, a sign of severe pancreatic exocrine insufficiency (PEI), is related to consequences caused by pancreatitis. This study aimed to identify predictors and to construct a nomogram for steatorrhea in idiopathic chronic pancreatitis (ICP).
METHODS: ICP patients admitted to our hospital from January 2000 to December 2013 were enrolled in this retrospective-prospective cohort study and randomly assigned to the training and validation cohorts. The cumulative rate of steatorrhea was calculated. A Cox proportional hazard regression model was used to identify predictors for steatorrhea and construct the nomogram. Internal and external validation of the nomogram was then performed.
RESULTS: There were 1633 ICP patients enrolled, with a median follow-up duration of 9.8 years and 20.8% (339/1633) of patients developed steatorrhea following onset of ICP. Steatorrhea was observed in 93, 115, and 133 patients at 1, 3, and 5 years following diagnosis of CP, with a cumulative rate of 6.5% (95% confidence interval [CI] 5.1%-7.9%), 8.0% (95% CI 6.2%-9.8%), and 9.3% (95% CI 6.6%-12.0%), respectively. Male sex (hazard ratio [HR] 2.479, P < 0.001), diabetes mellitus at/before diagnosis of ICP (HR 2.274, P = 0.003), and aged less than 18 years at onset of ICP (HR 0.095, P < 0.001) were identified risk factors for steatorrhea. Initial manifestations were associated with development of steatorrhea. The nomogram was proven to have good concordance indexes.
CONCLUSIONS: We identified predictors and developed a nomogram for predicting steatorrhea in ICP. It was recommended that high-risk populations be followed up closely, which might contribute to the early diagnosis and treatment of PEI.