Pancreatic cancer is one of the most lethal malignancies. Despite the availability of newer and better antineoplastic combinations, the 5-year survival rate for all stages of pancreatic cancer remains only 6%. Palliative care represents an important aspect of care in patient with pancreatic cancer. Thus, optimal palliation of symptoms to maximize remaining quality of life is of primary importance to most patients. This review discusses highlights the most significant problems faced when caring for patients with advanced pancreatic cancer. Common problems include pain, pancreatic insufficiency, anorexia-cachexia and depression. To provide optimal and meaningful supportive care for patients with advanced pancreatic cancer is complex and requires ongoing close monitoring of the physical and emotional aspects of the patient. Prompt management of the many symptoms and problems associated with pancreatic cancer is essential to minimize distress and improve quality of life for patients with this fatal disease. Recognizing end-of-life concerns and patient preferences during the dying process helps clinicians find ways to alleviate suffering for patients and families.
Depression ; Exocrine Pancreatic Insufficiency ; Humans ; Palliative Care ; Pancreatic Neoplasms ; Patient Preference ; Quality of Life ; Survival Rate

The digestive enzyme preparations are one of the most widely used drugs in Korea. However, it is rather misused or abused. Pancreatic enzyme supplement is only indicated for pancreatic insufficiency, such as cystic fibrosis, chronic pancreatitis, pancreatectomized patients. Pancreatic insufficiency causes malabsorption, most commonly manifested by steatorrhea. However, malabsorption symptoms are not apparent until the pancreas secretes less than 5% of its maximal secretory capacity, Which means that the use of enzyme preparations are very limited and should be prescribed after careful functional study of the pancreas. In Korea, however, digestive enzyme preparations are OTC drugs and furthermore, laymen's organizations insist that the digestive enzymes be sold freely in supermarkets. The concept about the use of digestive enzymes should be changed, and their proper use by physicians as well as laymen must be emphasized.
Cystic Fibrosis ; Exocrine Pancreatic Insufficiency ; Humans ; Korea ; Nonprescription Drugs ; Pancreas ; Pancreatitis, Chronic ; Steatorrhea

A 1. 8-year-old intact female Maltese dog was presented because of a history of chronic diarrhea, polyphagia, weight loss, and coprophagia. The patient was severely emaciated and evacuated very moist and four-smelling, yellow feces. Fecal stain with Sudan III revealed numerous lipid droplets. Result of fat absorption test showed aldigestion. A definite diagnosis was made based on trypsin-like immunoreactivity assay in serum which was low enough to be diagnosed as an exocrine pancreatic insufficiency. After pancreatic enzyme supplement with porcine pancreatin powder, the clinical signs were disappeared. This case report documents clinical manifestations, diagnostic tools, treatment and efficiency of oral pancreatic enzyme replacement therapy of exocrine pancreatic insufficiency in a Maltese dog.
Animals ; Dog Diseases/*drug therapy ; Dogs ; Exocrine Pancreatic Insufficiency/drug therapy/*veterinary ; Female ; Pancreatin/*therapeutic use

In order to clearly define the features of Shwachman-Diamond syndrome (SDS) in Chinese children, this article analyzes and summarizes the epidemiology, clinical features, and key points in the diagnosis and treatment of SDS in Chinese children with review of the clinical data of 27 children with SDS from related articles published previously. A comparative analysis was made between the Chinese and international data related to childhood SDS. The results showed a male/female ratio of about 2:1 in the Chinese children with SDS, with an age of onset of <1 month to 5 years (median 1 month) and an age of 3 months to 12 years (median 12 months) at the time of confirmed diagnosis. Reductions in peripheral blood cells due to myelopoiesis inhibition were observed in all 27 children with SDS, among whom 93% had neutropenia. Chronic diarrhea (85%), liver damage (78%), and short stature (83%) were the three main clinical features of SDS. Supplementation of pancreatin and component blood transfusion may temporarily alleviate the disease, while allogeneic hematopoietic stem cell transplantation is still an effective radical treatment. The comparative analysis of the Chinese and oversea data showed that compared with those in the European and American countries, the children with SDS in China had significantly higher incidence rates of chronic diarrhea, reductions in peripheral blood cells (three lineages), and liver damage, and there were also differences in the type of mutant genes.
Child ; China ; Exocrine Pancreatic Insufficiency ; Female ; Humans ; Male ; Neutropenia ; Shwachman-Diamond Syndrome ; Treatment Outcome

Pancreas lipomatosis is definde as fatty replacement of pancreatic acinar cells. Of the nine cases evaluated, seven cases (77.8%) of lipomatosis were limited inbody and tail, one case (11.1%) showed total lipomatosis excluding uncinate process and the remaining one case (11.1%) only in tail. As to the severity of lipomatosis, complete fat replacement in body and tail was found in four cases (44.4%), incomplete body and complete tail involvement in two (22.2%), incomplete body and tail, complete all except uncinate process, and complete tail involvements were found in one case (11.1%) each. Associated or predisposing factors included three diabetes mellitus (33.3%) combined with pancreas divisum, pancreas lithiasis and cholelithiasis respectively, hepatitis (22.2%) in two, and pseudocyst (11.1%) in one case, but in three cases (33.3%) nothing was found. In conclusion, pancreas lipomatosis was easily diagnosed by the abdominal CT and it was associated or predisposed by several e tities but had no major clinical symptoms, such as pancreatic insufficiency.
Acinar Cells ; Causality ; Cholelithiasis ; Diabetes Mellitus ; Exocrine Pancreatic Insufficiency ; Hepatitis ; Lipomatosis* ; Lithiasis ; Pancreas* ; Tail ; Tomography, X-Ray Computed

Shwachman-Diamond syndrome(SD syndrome) is a rare genetic disorder chracterized by pancreatic insufficiency, short stature, skeletal abnormalities and bone marrow dysfunction. Exocrine pancreatic insufficiency and neutropenia are the main components of the syndrome. A hallmark of SD syndrome is varying severity of pancreatic dysfunction due to acinar maldevelopment. The hematologic abnormalities associated with SD syndrome include varying cytopenias, marrow aplasia, myelodysplasia and a high risk of development of leukemia. We report a case of SD syndrome in a 10-year-old boy who presented with failure to thrive and myelodysplastic syndrome.
Bone Marrow ; Bone Marrow Diseases ; Child ; Exocrine Pancreatic Insufficiency ; Failure to Thrive ; Humans ; Leukemia ; Male ; Myelodysplastic Syndromes ; Neutropenia

Shwachman-Diamond syndrome (SDS) is an autosomal recessive genetic disorder, consisting of exocrine pancreatic insufficiency, chronic neutropenia, neutrophil chemotaxis defects, metaphyseal dysostosis, short stature, dental caries, and multiple organ involvements. Although SDS is the second most common hereditary abnormality of exocrine pancreas following cystic fibrosis in the Western countries, it has rarely been reported in Asia. We diagnosed a case of SDS in a 42-month-old girl, and genetic analysis including the relatives of the patient confirmed the diagnosis for the first time in Korea. She had short stature, steatorrhea, dental caries, and recurrent prulent otitis media and pneumonias. Laboratory studies revealed cyclic neutropenia, and serum levels of trypsin, amylase, and lipase were decreased. Simple radiography revealed metaphyseal sclerotic changes at the distal femur. A CT scan demonstrated a fatty infiltration and atrophy of the pancreas. On direct sequencing analysis of Shwachman-Bodian-Diamond Syndrome gene exon 2 region, the patient was homozygous for the c.258+2T>C mutation and heterozygous for the c.183_184TA>CT mutation and c.201A>G single nucleotide polymorphism. Treatment with pancreatic enzyme replacement, multivitamin supplementation, and regular to high fat diet improved her weight gain and steatorrhea.
Child, Preschool ; Dental Caries/*genetics ; Dysostoses/*genetics ; Exocrine Pancreatic Insufficiency/*genetics ; Female ; Humans ; *Mutation ; Neutropenia/*genetics ; Pedigree ; Syndrome

OBJECTIVE: To study the clinical features and genetic mutations of children with Shwachman-Diamond syndrome (SDS) and malignant myeloid transformation. METHODS: Next-generation sequencing was used to analyze the gene mutations in 11 SDS children with malignant myeloid transformation, and their clinical features and genetic mutations were analyzed. RESULTS: Of the 11 children with SDS, 9 (82%) presented with refractory cytopenia of childhood (RCC), 1 (9%) had myelodysplastic syndrome with excess blasts (MDS-EB), and 1 (9%) had acute myeloid leukemia with myelodysplasia-related changes (AML-MRC). The median age of onset of malignant myeloid transformation was 48 months (ranged 7 months to 14 years). Of the 11 children, 45% had abnormalities in the hematological system alone. Mutations of the SBDS gene were detected in all 11 children, among whom 5 (45%) had c.258+2T>C homozygous mutation and 3 (27%) had c.184A>T+c.258+2T>C compound heterozygous mutation. The new mutations of the SBDS gene, c.634_635insAACATACCTGT+c.637_638delGA and c.8T>C, were rated as "pathogenic" and "possibly pathogenic" respectively. The 3-year predicted overall survival rates of children transformed to RCC and MDS-EB/AML-MRC were 100% and 0% respectively (P=0.001). CONCLUSIONS SDS children may have hematological system symptoms as the only manifestation, which needs to be taken seriously in clinical practice. The type of malignant transformation is associated with prognosis.
Adolescent ; Child ; Child, Preschool ; Exocrine Pancreatic Insufficiency ; Humans ; Infant ; Leukemia, Myeloid, Acute ; Mutation ; Myelodysplastic Syndromes ; Shwachman-Diamond Syndrome

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). CONCLUSION 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.
Female ; Humans ; Male ; Bone Marrow Diseases/therapy* ; China ; East Asian People ; Exocrine Pancreatic Insufficiency/therapy* ; Shwachman-Diamond Syndrome/therapy*

BACKGROUND: Since the introduction of the Whipple procedure, it has been the standard treatment method for periampullary carcinomas. However, since the pancreatoduodenectomy has high operative morbidity and mortality, numerous modifications of the pancreatoduodenectomy have been developed to reduce the operative risk. Among the modifications of the pancreatoduodenectomy, the pancreatogastros tomy was developed to reduce pancreatic leakage, which is the most serious complication after a pancreatoduodenectomy. Many favorable data about the pancreatogastrostomy have been published recently. However, some surgeons are reluctant to do a pancreatogastrostomy for fear of early remnant pancreatic insufficiency due to reflux of gastric juice. For that reason, we compare the functional aspects of a pancreatoduodenectomy between a pancreatojejunostomy (P-J) and a pancreatogastrostomy (P-G). METHODS: We studied 35 patients who underwent a pylorus-preserving pancreatoduodenectomy (PPPD) with a diagnosis of a periampullary carcinoma (n=34) or pancreatitis (n=1) at Seoul National University Hospital between 1994 and 1997 and who lived without recurrence for over 1 year. The mean age was 57 year, and the sex ratio was 20:15. Among them, 20 patients received a pancreatojejunostomy; the others received a pancreatogastrostomy. To compare the two groups, we analyzed (1) general nutritional status, (2) gastrointestinal (GI) symptoms and pancreatic exocrine function by measuring fecal elastase, and (3) pancreatic endocrine function by using the oral glucose tolerance test (GTT). RESULTS: After a PPPD, the body weight was decreased in both groups compared to that of the preoperative healthy state, but there was no difference between two groups. Also, no statistical difference could be found in triceps skin-fold thickness and serum protein and albumin and postoperative gastrointestinal symptoms except steatorrhea. There were 4 mild and 15 severe pancreatic exocrine insufficiencies in P-J patients, but all P-G patients showed severe pancreatic insufficiency on the stool elastase test. Excluding preoperative diabetes patients, 44% (7/16) of the P-J patients had an abnormal GTT after the operation, but 75% (9/12) of the P-G patients had an abnormal GTT (p=0.114). CONCLUSION: Exocrine and endocrine pancreatic insufficiencies developed after a PPPD, but did not induce the general malnutrition. A P-G had more deterioration of the pancreatic function than a P-J did. Thus, we must consider the general nutritional status, as well as the risk of pancreatic leakage, in the determination of pancreatoenteric anastomosis.
Body Weight ; Diagnosis ; Exocrine Pancreatic Insufficiency ; Gastric Juice ; Glucose Tolerance Test ; Humans ; Malnutrition ; Mortality ; Nutritional Status ; Pancreatic Elastase ; Pancreaticoduodenectomy* ; Pancreaticojejunostomy ; Pancreatitis ; Pylorus* ; Recurrence ; Seoul ; Sex Ratio ; Steatorrhea