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Octreotide is recommended as a second-line treatment for patients with congenital hyperinsulinism (CHI), especially for those who do not respond to diazoxide or surgical intervention. Studies on the adverse effects of octreotide in large cohorts are still scarce. We evaluated the safety of octreotide in CHI patients by reviewing cases from the two largest centres in China that specialize in the management of this condition. Our study analysed adverse events in 122 CHI patients on octreotide, with a 93% success rate for the therapy alone. The mean maximum dose of octreotide was 13.1 ± 6.5 µg/kg/day, with no difference in required doses between diffuse and focal lesion patients. Common side effects were hepatobiliary injuries (20.5%), gastrointestinal symptoms (31.1%), and transient hyperglycaemia (49.2%), with one case of necrotizing enterocolitis. Adverse event rates increased in patients treated with intermediate octreotide dose to those treated with higher doses, rising from 58% at doses of 5-10 µg/kg/day to 100% at doses exceeding 20 µg/kg/day. Patients experiencing adverse events received significantly higher doses., Conclusion: Octreotide effectively maintained blood glucose levels in diazoxide-unresponsive CHI patients without serious adverse effects, across all subtypes. Our study suggests that intermediate octreotide dosing is associated with a lower incidence of adverse events. We recommend close monitoring of patients receiving octreotide, especially during the initiation phase and when higher doses are administered., What Is Known: • Octreotide is a commonly used second line treatment for patients with congenital hyperinsulinism. • The administration of octreotide is frequently associated with gastrointestinal adverse events., What Is New: • Our study indicates that octreotide is used without serious adverse effects to maintain blood glucose levels in infants with congenital hyperinsulinism who are otherwise in good health. • The incidence of adverse events in patients treated with octreotide is dose-dependent., Competing Interests: Declarations. The studies involving humans were approved by Children’s hospital of Fudan University (2017-130). The studies were conducted in accordance with the local legislation and institutional requirements. The participants provided their written informed consent to participate in this study. Competing interests: The authors declare no competing interests., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: ABCC8 variants can cause hyperinsulinemia by activating or deactivating gene expression. This study used targeted exon sequencing to investigate genetic variants of ABCC8 and the associated phenotypic features in Chinese patients with hyperinsulinemic hypoglycemia (HH)., Methods: We enrolled eight Chinese children with HH and analyzed their clinical characteristics, laboratory results, and genetic variations., Results: The age at presentation among the patients ranged from neonates to 0.6 years old, and the age at diagnosis ranged from 1 month to 5 years, with an average of 1.3 ± 0.7 years. Among these patients, three presented with seizures, and five with hypoglycemia. One patient (Patient 7) also had microcephaly. All eight patients exhibited ABCC8 abnormalities, including six missense mutations (c. 2521 C > G, c. 3784G > A, c. 4478G > A, c. 4532T > C, c. 2669T > C, and c. 331G > A), two deletion-insertion mutations (c. 3126_3129delinsTC and c. 3124_3126delins13), and one splicing mutation (c. 1332 + 2T > C). Two of these mutations (c. 3126_3129delinsTC and c. 4532T > C) are novel. Six variations were paternal, two were maternal, and one was de novo. Three patients responded to diazoxide and one patient responded to octreotide treatment. All there patients had diazoxide withdrawal with age. Two patients (patients 3 and 7) were unresponsive to both diazoxide and octreotide and had mental retardation., Conclusions: Gene analysis can aid in the classification, treatment, and prognosis of children with HH. In this study, the identification of seven known and two novel variants in the ABCC8 gene further enriched the variation spectrum of the gene., (© 2024. The Author(s).)

Most of the neuroendocrine tumors(NETs) overexpress the somatostatin receptor(SSTR),which provides a reliable target for SSTR-targeted peptide receptor radionuclide therapy(PRRT).Compared with drug therapy,PRRT has high objective response rate and significantly prolongs patients' survival.Moreover,the patients have good tolerance to this therapy.Considering that PRRT is in clinical trial phase in China,this article elaborates on the selection and preparation of patients,pre-treatment medications,administration methods,treatment cycles,side effects,follow-up plan,and the combination of PRRT with other drugs based on the published international guidelines in this field and our experience from clinical practice.Hoping that relevant professionals can well understand the principle of PRRT and apply it in clinical practice,we write this article to provide a basis for serving real-world patients and carrying out clinical trials.

First cycle dosimetry calculation of 177 Lu-DOTATOC (single activity:1.59-3.49 GBq) was carried out in eight patients with advanced neuroendocrine tumors (NETs) who underwent whole-body planar (0.5, 24, 48, 72 h) and SPECT/CT scans (24 h). Focal uptake of 177 Lu-DOTATOC was found in primary and metastatic tumors. Organs with the highest absorbed doses per injected activity were tumors (1.293 ± 0.862 mGy/MBq) and spleen (0.461 ± 0.408 mGy/MBq), while low absorbed doses were observed in kidneys (0.384 ± 0.112 mGy/MBq) and bone marrow (0.0297 ± 0.0123 mGy/MBq). 177 Lu-DOTATOC is safe, well-tolerated and appropriate in Chinese NETs patients for PRRT., (Copyright © 2021. Published by Elsevier Ltd.)

Background: Efficacy of pancreatic enzyme inhibitors in acute pancreatitis (AP) is unclear in China., Aims: We aimed to present the current status of AP and evaluate the efficacy of pancreatic enzyme inhibitors in a larger population in China., Method: A retrospective, cross-sectional, real-world, multicenter analysis of a large dataset of patients presenting with AP from four hospitals of China over a two-year period was performed. Data were collected from the existing clinical records and the patients were grouped into medication group (somatostatin or octreotide or somatostatin and octreotide) and no medication group. Pair wise propensity score matching was performed for comparing somatostatin, octreotide and somatostatin/octreotide. The end points were incidence of disease complications, organ failure, hospitalization duration, and recovery time taken (hours) for serum amylase/serum lipase to normalcy., Results: A total of 3900 patients were recruited and 2775 patients were included for analysis. A total of 1100, 661, 676 and 338 patients received either somatostatin or octreotide or somatostatin and octreotide or no medication, respectively. The incidence of complications (7.6% vs 13.6%), organ failure (4.5% vs 7.4%), and the instances of entering ICU (9.3% vs 13.3%) were higher in unmedicated group. Complications at discharge (2.91 times), organ failure (2.53 times), and hospitalization stay were higher in octreotide-treated patients compared with somatostatin-treated patients. In comparison to the octreotide group, the serum amylase/lipase recovery time was shorter in the somatostatin group., Conclusion: This real-world study suggested that the use of pancreatic enzyme inhibitors was positively associated with greater clinical efficacy in AP patients and somatostatin might be more effective than octreotide in real-world settings in China.

Primary nephrotic syndrome (PNS) is one of the most common primary glomerular diseases in children. Patients complicated nephrotic syndrome with pancreatic lesions are rarely reported, and the clinical manifestations in children are atypical. This study has observed the incidence, clinical types, and prognosis of acute pancreatitis (AP) in children with primary nephrotic syndrome, and analyzed its related factors, early diagnosis, and treatment.Seven children with PNS and AP in Shanghai Children's Hospital from January 2015 to December 2017 were reviewed. The clinical data including age, height, weight, body mass index (BMI), diet, biliary tract disease, PNS durations, drugs, proteinuria, creatinine, glucose, glycated hemoglobin, amylase and lipase, albumin, cholesterol, triglyceride, ultrasound, computerized tomography (CT), renal pathology and estimated glomerular filtration rate (eGFR) were retrospectively analyzed. All patients were followed for >2 years.Ten in 589 patients with PNS were detected pancreatic lesions by abdominal ultrasound. Seven were diagnosed as AP, which the incidence was 1.2%. Only 1 of 7 patients had elevated serum amylase. Lesions of pancreas were found by ultrasound and/or enhanced CT. Four of 7 patients had been treated with tacrolimus. All patients with AP were improved after octreotide acetate injection and supportive treatment. Only 1 patient suffered recurrent AP during the relapse of PNS 10 months later.AP in children with PNS is not common, and the clinical manifestations are not typical. Abdominal ultrasound and enhanced CT are of high value in diagnosis. The adverse effects of tacrolimus should be concerned. Early diagnosis and timely treatment can be helpful for a prognosis.

Objective: To describe our clinical experience with surgical treatments for sinonasal phosphaturic mesenchymal tumors diagnosed at our institution., Study Design: Retrospective case series., Setting: Affiliated Sixth People's Hospital, Shanghai Jiao Tong University., Subjects and Methods: We retrospectively reviewed the medical records of 10 patients diagnosed with phosphaturic mesenchymal tumors associated with tumor-induced osteomalacia between December 2014 and October 2019., Results: There were 4 men and 6 women with a disease course of 1 to 19 years. All patients exhibited hypophosphatemia and tumor-induced osteomalacia. The tumor was located in the sinonasal region, frontal bone, and temporal bone in 8 patients, 1 patient, and 1 patient, respectively. Technetium-99m octreotide scintigraphy was used for tumor localization in 4 cases. Six patients underwent endoscopic resection; the remaining 4 underwent unilateral transorbital anterior and posterior ethmoid artery ligation + endoscopic resection, endoscopic resection + skull base repair, internal carotid artery stenting + transcatheter arterial embolization + temporal bone tumor excision + adipose tissue plugging, and endoscopic resection + transfrontal craniotomy (n = 1 each). Two patients had a history of incomplete endoscopic resection. All patients achieved clinical remission and normalized biochemical indices after surgery. Only 1 patient developed recurrence and died of a brain hernia., Conclusions: A diagnosis of sinonasal phosphaturic mesenchymal tumors should be based on a combination of clinical, imaging, and pathological findings. Technetium-99m octreotide scintigraphy helps in locating the tumor. Complete surgical excision guarantees clinical remission, and preoperative transcatheter arterial embolization or feeding artery ligation may reduce intraoperative bleeding in cases of highly vascularized tumors.

BACKGROUND The aim of this study was to explore the risk factors for post-endoscopic retrograde cholangiopancreatography (ERCP) pancreatitis and investigate the effect of octreotide combined with nonsteroidal anti-inflammatory drugs on preventing its occurrence. MATERIAL AND METHODS A total of 139 patients undergoing ERCP in our hospital from May 2016 to April 2017 were retrospectively analyzed, and divided into an observation group (n=67) (octreotide + indomethacin) and a control group (n=72) (no preventive drugs). The preoperative and postoperative inflammatory cytokines such as tumor necrosis factor-α (TNF)-α, interleukin-6 (IL-6) and IL-8, and serum amylase levels were measured, and the incidence of pancreatitis and hyper amylasemia were monitored. RESULTS Serum amylase level was increased significantly 3 hours after operation in both groups with significantly higher level in the control group compared to the observation group. After 24 hours, serum amylase in the observation group was decreased to preoperative level, whereas it was still higher than preoperative in the control group (P<0.05). Regarding the levels of TNF-α, IL-6, IL-8, and visual analogue scale, they were significantly increased in both groups after operation with significantly higher levels in the control group compared to the observation group (P<0.05). Furthermore, logistic regression analysis showed that difficult intubation, pancreatic duct angiography, surgery for a long time, and the history of previous pancreatitis were risk factors for post-ERCP pancreatitis (P<0.05). CONCLUSIONS Difficult intubation, pancreatic duct angiography, surgery for a long time, and the history of previous pancreatitis were risk factors for post-ERCP pancreatitis. Octreotide combined with non-steroidal anti-inflammatory drugs can reduce the pain of patients with abdominal pain as well as the incidence of postoperative pancreatitis, indicating that they might be effective preventative approaches for pancreatitis.

Congenital chylous ascites in the neonatal period is a rare entity. Total parenteral nutrition (TPN), medium chain triglyceride (MCT)-based diet, octreotide and repeated paracentesis are regarded as appropriate medical treatment for congenital chylous ascites, and surgery is recommended when conservative therapy has failed. We present two cases in which ascites were confirmed via an abdominal sonogram and diagnostic paracentesis. In our clinical experience, rice soup combined with PN can be a safe and effective intervention.

Objectives: Since the introduction of endoscopic retrograde cholangiopancreatology (ERCP) in clinical use, pancreatitis has become a common complication of ERCP. Octreotide is an inhibitor of pancreatic enzyme secretions. Several studies have evaluated the effect of octreotide on the incidence of clinical pancreatitis after ERCP, but with different results. The aim was to determine the efficacy of prophylactic administration of octreotide for the prevention of post-ERCP pancreatitis (PEP) and hyperamylasemia., Methods: In this study, patients with scheduled ERCP were randomized to receive either octreotide (0.3 mg) via intramuscular injection or a placebo. The study was conducted in 12 digestive endoscopic units in China. Patients were randomized into two groups: an octreotide group (N = 414) and a control group (N = 418). In the octreotide group, octreotide (0.3 mg) was dissolved in 500 mL of 0.9% saline solution and administered by continuous intravenous infusion, beginning 1 h before endoscopic examination and continued for 6 h thereafter; 0.1 mg of octreotide was injected subcutaneously at 6 and 12 h after the intravenous injection was stopped. The control group was given a placebo intravenously. The end point was the development of acute pancreatitis., Results: The overall incidence of acute pancreatitis was 3.85%; this included 2.42% (10/414) in the octreotide group and 5.26% (22/418) in the control group (P = 0.046). The overall incidence of hyperamylasemia was 14.9%; 12.32% (51/414) in the octreotide group and 17.46% (73/418) in the control group (P = 0.041). No side effects were found., Conclusion: The results indicate that octreotide can prevent PEP and hyperamylasemia.