Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0030305 (pancreatitis)
 16,014 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Little is known about octreotide therapy in asparaginase-associated pancreatitis (AAP) in children. Of the 59 children with acute lymphoblastic leukemia (ALL) receiving E. coli L-asparaginase, 5 patients (8.5%) developed AAP. Octreotide was administered to four patients. Clinical and laboratory improvement were evident after octreotide therapy. There were no deaths and no severe adverse side effects were noted. No pseudocysts were detected; however, two of the four patients developed diabetes. One child without octreotide treatment developed chronic pancreatitis and pseudocyst. We conclude that octreotide therapy appears to be safe and potentially beneficial in the management of AAP in children.
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PMID:Octreotide therapy in asparaginase-associated pancreatitis in childhood acute lymphoblastic leukemia. 1872 19

Plasmapheresis for the treatment of hypertriglyceridemia has previously been performed in patients with sudden onset severe hypertriglyceridemia and acute pancreatitis; however, only a few reports of this procedure have been published. We report here on a case showing severe hypertriglyceridemia during asparaginase (Asp) treatment for acute lymphocytic leukemia (ALL), and give an overview of a lipid-lowering apheresis therapy. To prevent the complication of pancreatitis due to hypertriglyceridemia, we performed plasma exchange (PE) three times using fresh frozen plasma. PE remarkably reduced both serum triglyceride and total cholesterol levels from 5430 mg/dL to 403 mg/dL and from 623 mg/dL to 204 mg/dL, respectively. The causes of severe hyperlipidemia in this patient were considered to include: the Asp treatment for ALL, and a genetic background with a heterozygote of familial lipoprotein lipase (LPL) defect syndrome, because the patient's plasma LPL level after intravenous heparin injection was low at 137 ng/mL. Hence, PE using fresh frozen plasma may be useful not only to remove lipoproteins, but also to supply defective factors, such as LPL, in similar cases.
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PMID:A case report of an adult with severe hyperlipidemia during acute lymphocytic leukemia induction therapy successfully treated with plasmapheresis. 1914 Aug 51

Hyperglycemia has been described as a common event occurring during acute lymphocytic leukemia chemotherapy. It is associated with the synergistic effect of L-asparaginase and glucocorticoids, and related to poor outcome. Our goal was to compare clinical and laboratory findings between hyperglycemic episodes occurring during childhood acute lymphocytic leukemia induction chemotherapy. Here we describe 12 (3.8%) high-risk patients of 311 total patients, 9 (75%) of who are female. The 12 patients presented with 16 hyperglycemic episodes classified into adverse or satisfactory categories. There were no differences in clinical or laboratory variables among groups, although the majority of episodes occurred in pubescents, regardless of the type of glucocorticoid employed. Despite the fact that only 1 patient was overweight, pancreatitis was not diagnosed. Although we could not determine whether hyperglycemia predicts an adverse outcome, glucose evaluation played an important role during induction chemotherapy. To date, recognized risk factors for hyperglycemia no longer explain our findings, thus other mechanisms related to insulin secretion and action should be further studied.
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PMID:Transient hyperglycemia during childhood acute lymphocytic leukemia chemotherapy: an old event revisited. 1970 Nov 54

Treatment with asparaginase for acute lymphoblastic leukemia can cause acute pancreatitis. Complication of pancreatitis by pancreatic pseudocyst formation can prolong the hospital stay, delay chemotherapy, and necessitate long-term parenteral nutrition. We report 5 children with acute lymphoblastic leukemia who developed acute pancreatitis complicated by pancreatic pseudocysts. They required modifications to their chemotherapy regimen and prolonged parenteral nutrition but no surgical intervention. All 5 patients survive in first remission and their pseudocysts resolved after 3 to 37 months or continued to decrease in size at last follow-up. These cases illustrate that nonsurgical management of pancreatic pseudocyst is safe, though pseudocyst resolution may require many months. In addition, these patients demonstrate that oral feeding can be initiated after the acute episode of pancreatitis resolves even if a pseudocyst is present.
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PMID:Conservative management of pancreatic pseudocysts in children with acute lymphoblastic leukemia. 1995 23

Over the past several decades, L-asparaginase, an important component of therapy for acute lymphoblastic leukemia (ALL), has typically been administered intramuscularly rather than intravenously in North America because of concerns regarding anaphylaxis. We evaluated the feasibility of giving polyethylene glycosylated (PEG)-asparaginase, the polyethylene glycol conjugate of Escherichia coli L-asparaginase, by intravenous infusion in children with ALL. Between 2005 and 2007, 197 patients (age, 1-17 years) were enrolled on Dana-Farber Cancer Institute ALL Consortium Protocol 05-01 and received a single dose of intravenous PEG-asparaginase (2500 IU/m(2)) over 1 hour during remission induction. Serum asparaginase activity more than 0.1 IU/mL was detected in 95%, 88%, and 7% of patients at 11, 18, and 25 days after dosing, respectively. Toxicities included allergy (1.5%), venous thrombosis (2%), and pancreatitis (4.6%). We conclude that intravenous administration of PEG-asparaginase is tolerable in children with ALL, and potentially therapeutic enzyme activity is maintained for at least 2 weeks after a single dose in most patients. This trial was registered at www.clinicaltrials.gov as #NCT00400946.
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PMID:Intravenous PEG-asparaginase during remission induction in children and adolescents with newly diagnosed acute lymphoblastic leukemia. 2000 9

Asparaginase is an enzyme that breaks down extracellular asparagine into aspartic acid and ammonia. Depletion of extracellular asparagine inhibits the growth of lymphocytic leukemic cells. Unlike normal cells, lymphoblasts lack the enzyme to synthesize asparagine and therefore rely on an exogenous source of this amino acid to maintain cellular protein synthesis. Asparagine depletion results in nutritional deprivation, inhibition of protein synthesis, and subsequent apoptotic cell death in lymphoblasts. Asparaginase therapy is an essential component of the treatment protocol for acute lymphoblastic leukemia. The effect of asparaginase on protein synthesis may result in a number of toxicities, including thrombosis, pancreatitis, hyperglycemia, and hepatotoxicity. This review discusses the incidence of asparaginase-related adverse events, compares available asparaginase formulations with respect to the emergence of certain toxicities, and considers management strategies for these toxicities in patients with acute lymphoblastic leukemia.
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PMID:Incidence and management of asparaginase-associated adverse events in patients with acute lymphoblastic leukemia. 2002 Jun 72

L-asparaginase (L-ASNase) has been an essential component of multiagent chemotherapy for acute lymphoblastic leukemia in childhood for over 3 decades. There are currently 2 Food and Drug Administration (FDA)-approved formulations of L-ASNase derived from Escherichia coli and 1 non-FDA approved formulation derived from Erwinia chrysanthemi. Modifications in L-ASNase have included pegylation, which decreases drug immunogenicity and increases the half-life, allowing less frequent administration. Although L-ASNase is well-tolerated in most patients and causes little myelosuppression, significant toxicities occur in up to 30% of patients. Hypersensitivity is the most common toxicity of L-ASNase therapy and limits the further use of the drug. Other significant toxicities relate to a reduction in protein synthesis and include pancreatitis, thrombosis, central nervous system complications, and liver dysfunction. The spectrum of common toxicities and the efficacy of different formulations of L-ASNase are presented in this review.
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PMID:Tolerability and efficacy of L-asparaginase therapy in pediatric patients with acute lymphoblastic leukemia. 2072 51

Pancreatic infiltration of leukemic cells is a very rare manifestation at the onset of acute lymphoblastic leukemia (ALL) in childhood. Pancreatic enlargement in this situation is unusual and pancreatic involvement is often associated with biliary obstruction, cholestasis and pancreatitis. We report a 3-month-old girl who presented with asymptomatic leukemic infiltration of the pancreas, demonstrated by US with heterogeneous pancreatic enlargement associated with multiple hypoechogenic lesions, without cholestasis. Although these manifestations are rare, ALL should be considered a cause of pancreatic enlargement.
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PMID:Asymptomatic leukemic-cell infiltration of the pancreas: US findings. 2107 24

Duplication 22q11.2 syndrome is the result of a microduplication of the same chromosomal region that is deleted in DiGeorge and Velocardiofacial syndromes. We describe a patient with dysmorphic features who was diagnosed with pre-B acute lymphoblastic leukemia, and developed cystinuria and pancreatitis during treatment. Duplication 22q11.2 has not been previously described in association with hematologic abnormalities. Chromosomal microarray technology was used to diagnose duplication 22q11.2 syndrome. In this era of advanced genomics, this technology has become an important method for helping to determine the molecular basis of diseases, best treatments and ultimately patient outcomes.
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PMID:B-acute lymphoblastic leukemia and cystinuria in a patient with duplication 22q11.21 detected by chromosomal microarray analysis. 2122 31

L-Asparaginase is an effective drug in childhood acute lymphoblastic leukemia (ALL) and it has become an important component of most childhood ALL regimens with administration in induction, intensification, and maintenance phases of treatment. L-Asparaginase is associated with side effects occurring either in a dose or time-dependent fashion or as hypersensitivity reactions. Some well-known toxicities in asparaginase-containing regimens are hypersensitivity/allergy and thromboembolic events. When asparaginase and steroids are used together, mild hyperlipemia is reasonably common. As some published studies show, this abnormality is often underdiagnosed. Hyperlipemia rarely constitutes a clinical problem; however, when triglyceride elevation is greater than 1000 mg/dL, the risk of pancreatitis is increased. We report the case of a young female presenting with acute severe hypertriglyceridemia (9250 mg/dL) during intensification phase of ALL, with neurologic symptoms but without the development of pancreatitis. She was successfully managed with 1 single run of plasmapheresis.
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PMID:L-Asparaginase and steroids-associated hypertriglyceridemia successfully treated with plasmapheresis in a child with acute lymphoblastic leukemia. 2139 28


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