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

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*

Bone Marrow Diseases ; diagnosis ; genetics ; therapy ; Child, Preschool ; Exocrine Pancreatic Insufficiency ; diagnosis ; genetics ; therapy ; Female ; Humans ; Infant ; Lipomatosis ; diagnosis ; genetics ; therapy ; Male ; Mutation

Chronic pancreatitis is an inflammatory disease characterized by the progressive destruction of pancreatic tissue and resulting in pancreatic exocrine and endocrine insufficiency. Although a lot of efforts have been made in managing chronic pancreatitis, treatment of chronic pancreatitis is still challenging and remains mostly empirical. Medical treatment of chronic pancreatitis focuses on chronic abdominal pain, pancreatic exocrine insufficiency and also pancreatic endocrine insufficiency. Chronic disabling abdominal pain is the main symptom of chronic pancreatitis, which could be managed by analgesics and non-enteric coated pancreatic enzymes as well as adjunctive agents such as antidepressants, duolexitine and pregabalin. Pancreatic exocrine insufficiency is commonly associated with weight loss, steatorrhea and malnutrition. Adequate pancreatic enzyme replacement therapy can significantly improve not only exocrine insufficiency related symptoms but also nutritional status. Several problems affecting the efficacy of pancreatic enzyme replacement can be avoided by the use of enteric-coated enzyme microspheres with or without proton pump inhibitors.
Abdominal Pain ; Analgesics ; Antidepressive Agents ; Enzyme Replacement Therapy ; Exocrine Pancreatic Insufficiency ; gamma-Aminobutyric Acid ; Malnutrition ; Microspheres ; Nutritional Status ; Pancreatitis, Chronic ; Proton Pump Inhibitors ; Steatorrhea ; Weight Loss ; Pregabalin

Abnormalities, Multiple ; genetics ; pathology ; Child, Preschool ; Chromosome Aberrations ; Diagnosis, Differential ; Diarrhea ; diagnosis ; etiology ; therapy ; Exocrine Pancreatic Insufficiency ; complications ; Fever ; diagnosis ; etiology ; Fingers ; abnormalities ; Humans ; Male ; Pancreatic Diseases ; etiology ; Syndrome