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)

The first girl of an unrelated couple was noted to have failure to thrive since age 3 months, generalized hypotonia and weakness, hepatomegaly, hypoglycemia, and lactic acidosis at 4 months. She was found to have severe mitochondrial DNA (mtDNA) depletion and respiratory chain complex IV deficiency in both skeletal muscle and liver but without other common mtDNA mutations. Her younger brother developed vomiting at age 3 weeks and was diagnosed as having pyloric stenosis. His skeletal muscle and liver also showed severe mtDNA depletion. He developed generalized weakness and hypotonia, hepatomegaly, and lactic acidosis at age 3 months. Both siblings died of hepatic failure and hemorrhagic complication before 6 months of age. The brother also had chemical pancreatitis, which had not been reported before in mtDNA depletion in children. Severe mtDNA depletion may present with nonspecific symptoms such as vomiting, failure to thrive, and developmental delay; multiorgan involvement such as hepatomegaly, pancreatitis, and myopathy occurs later. Mitochondrial DNA depletion should be considered in the differential diagnosis in children with developmental delay or failure to thrive of unknown etiology.
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PMID:Mitochondrial DNA depletion in children. 1119 1

Coxsackieviral infections have been linked etiologically to multiple diseases. The serotype CB4 is associated with acute pancreatitis and autoimmune type 1 diabetes. To delineate the mechanisms of host survival after an acute infection with CB4 (strain E2), we have investigated the role of nitric oxide (NO), generated by the inducible form of nitric oxide synthase (NOS2), in viral clearance and pancreatic beta-cell maintenance. Mice deficient in NOS2 (NOS2-/- mice) and their wild-type (wt) counterparts were injected with CB4, after which both groups developed severe pancreatitis, hepatitis, and hypoglycemia within 3 days. Within 4 to 7 days postinfection (p.i.), most of the NOS2-/- mice died and at a strikingly higher mortality rate than wt mice. Histological examination of pancreata from both infected NOS2-/- and infected wt mice revealed early and complete destruction of the pancreatic acinar tissue, but intact, insulin-stained islets. When examined up to 8 weeks p.i., neither surviving NOS2-/-mice nor surviving wt mice developed hyperglycemia. However, the clearance of infectious CB4 was different between the mice. The spleens of NOS2-/- survivors were cleared of infectious virus with kinetics similar to that of wt mice, but the livers, pancreata, kidneys, and hearts of the NOS2-/- groups cleared virus more slowly than those of the wt group. This delayed clearance was particularly prominent in the livers of infected NOS2-/- mice, which also showed prolonged histopathological features of viral hepatitis. Taken together, this outcome suggests that NOS2 (and NO) is not required for the prevention of pancreatic beta-cell depletion after CB4 infection. Instead the critical actions of NOS2 apparently occur early in the host immune response, allowing mice to survive and clear virus. Moreover, the data support the existence of an organ-specific dependency on NO for a rapid clearance of CB4.
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PMID:A critical role for inducible nitric oxide synthase in host survival following coxsackievirus B4 infection. 1127 93

Octreotide is a somatostatin analogue that has been suggested as a therapeutic agent in various diverse disease processes including gastrointestinal bleeding, pancreatitis, hypoglycemia related to hyperinsulin states, and chylous peritoneum/thorax. Despite successful use in the adult population, there is limited information concerning its use in pediatric patients. The authors retrospectively review their experience with octreotide in 10 infants and children ranging in age from 14 days to 17 years. Octreotide, administered by continuous intravenous infusion or intermittent bolus dosing, was used in the treatment of gastrointestinal bleeding in four patients, pancreatitis in three patients, chylous leaks in two patients, and hypoglycemia related to nesidioblastosis in one patient. The clinical course of these patients and the potential therapeutic impact of octreotide are evaluated. Additionally, previous experiences with octreotide in pediatric patients, dosing regimens, and the potential role of the drug in other disease processes are discussed.
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PMID:Initial experience with octreotide in the pediatric population. 1170 79

Ethanol concentrations were measured in femoral venous blood in deaths attributed to acute alcohol poisoning (N = 693) or chronic alcoholism (N = 825), according to the forensic pathology report. Among acute alcohol poisonings were 529 men (76%) with mean age 53 years and 164 women (24%) with mean age 53 years. In the chronic alcoholism deaths were 705 men (85%) with mean age 55 years and 120 women (15%) with mean age 57 years. The blood-ethanol concentrations were not related to the person's age (r = -0.17 in acute poisonings and r = -0.09 in chronic alcoholism). The distribution of blood-ethanol concentrations in acute poisoning cases agreed with a normal or Gaussian curve with mean, median, standard deviation, coefficient of variation, and spread of 0.36 g/100 mL, 0.36 g/100 mL, 0.086 g/100 mL, 24% and 0.074 to 0.68 g/100 mL, respectively. The corresponding concentrations of ethanol in chronic alcoholism deaths were not normally distributed and showed a mode between 0.01 and 0.05 g/100 mL and mean, median, and spread of 0.172 g/100 mL, 0.150 g/100 mL, and 0.01 to 0.56 g/100 mL, respectively. The 5th and 95th percentiles for blood-ethanol concentration in acute poisoning deaths were 0.22 and 0.50 g/100 mL, respectively. However, these values are probably conservative estimates of the highest blood-ethanol concentrations before death owing to metabolism of ethanol until the time of death. In 98 chronic alcoholism deaths (12%) there was an elevated concentration of acetone in the blood (>0.01 g/100 mL), and 50 of these (6%) also had elevated isopropanol (>0.01 g/100 mL). This compares with 28 cases (4%) with elevated blood-acetone in the acute poisoning deaths and 22 (3%) with elevated blood-isopropanol. We offer various explanations for the differences in blood-ethanol and blood-acetone in acute poisoning and alcoholism deaths such as chronic tolerance, alcohol-related organ and tissue damage (cirrhosis, pancreatitis), positional asphyxia or suffocation by inhalation of vomit, exposure to cold coupled with alcohol-induced hypothermia, as well as various metabolic disturbances such as hypoglycemia and ketoacidosis.
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PMID:Comparison of blood-ethanol concentration in deaths attributed to acute alcohol poisoning and chronic alcoholism. 1287 10

Diabetes mellitus (DM) in chronic pancreatitis (ChP) is considered a unique clinical and metabolic unit. Compared to type I DM it has many different properties: glycemic lability, more frequent hypoglycaemic episodes, and minimum incidence of ketoacidosis. The need of insulin administration to achieve satisfying diabetes mellitus compensation is significantly lower and response of peripheral tissues to endogenous and exogenous insulin significantly higher compared to type I diabetics. These clinical differences result from decreased but always preserved insulin secretion, decreased glucagon production, impaired external pancreatic secretion, and also excessive alcohol use or insufficient or irregular food intake of the patients. Secondary DM in ChP is accompanied by chronic, microangiopathic and neuropathic complications analogous to other DM types. Nonpharmacological treatment measurements of the first choice are elimination of alcohol, sufficient and adequate nutrition, and simultaneous treatment of impaired exocrinal secretion. A pharmacology treatment is insulin therapy! It is a substitution treatment for insulin deficiency. Insulin doses must be chosen very carefully because of the risk of hypoglycaemia. The most frequent cause of secondary diabetes mellitus in patients with pancreatic diseases in Europe is chronic alcoholic pancreatitis and in tropical countries and India non-alcoholic tropical calcific pancreatitis (TCP).
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PMID:[Diabetes mellitus in chronic pancreatitis]. 1530 35

Chronic pancreatitis is mostly caused by heavy alcohol consumption and is characterized by the onset of symptoms in the fourth and fifth decade. Beginning in patients older than 65 years of age is rare. Leading symptom is recurrent or persisting abdominal pain which is missed only in approximately 5% of the cases. Chronic pancreatitis is classified as idiopathic if there is no anamnesis of alcohol abuse or some rare specific causes. The Idiopathic Chronic Senile Pancreatitis (ICSP) is a subset of the non-alcoholic pancreatitis and is characterized by advanced age at the time of first manifestation. Although life expectancy especially in chronic alcoholic pancreatitis is reduced, there are many patients who reach older age. The natural history in all forms of chronic pancreatitis shows a decrease in pain and the manifestation of exocrine and endocrine insufficiency as late complications. Especially in the elderly loss of weight may occur with steatorrhea and pancreatic diabetes mellitus as the dominating clinical problem of chronic pancreatitis. If pain persists treatment is symptomatically with analgesics. The possibility of causal surgery or the indication for endoscopic treatment of painful chronic pancreatitis should be proven in every single case. Standard pancreatin treatment consisting of large amounts of enzymes will abolish maldigestion. Pancreatic diabetes requires often insulin, there is a tendency to hypoglycaemia. In contrast to chronic pancreatitis cancer of the pancreas is a typical and frequent disease of the elderly. The prognosis is bad and one year life expectancy is just about 11%. One of the reasons is, that the diagnosis is found lately because early symptoms are missing. Specific symptoms like pain, weight loss or jaundice occur lately. In suspicion of pancreatic cancer a lot of methods of morphological diagnostic are available such as CT, MRCP, ultrasound, ERCP and PET, in addition the specific tumor markers CA 19-9 and CEA. After diagnostic is completed, curative resection is possible in only a low percentage of all cases. Old age is no contraindication for surgery, prognosis and the risk of surgery don't differ to other age groups. In most cases palliative therapy is the only possible option because of an advanced tumor stage. Sufficient pain therapy, endoscopic stenting in case of obstructive jaundice or gastroenterostomy in case of duodenal are useful interventions.
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PMID:[Chronic pancreatis and pancreatic carcinoma in the elderly]. 1598 40

Citrin, encoded by SLC25A13, is a liver-type mitochondrial aspartate-glutamate carrier (AGC), of which deficiency, in autosomal recessive trait, causes neonatal intrahepatic cholestasis (NICCD) and adult-onset type II citrullinemia (CTLN2). NICCD patients have jaundice, hypoproteinemia, hypoglycemia, galactosemia, growth retardation, fatty liver and multiple aminoacidemia including citrulline, methionine, threonine and tyrosine. Some of the neonates who have experienced NICCD suffer from severe CTLN2 more than 10 years or several decades later. In CTLN2, neuropsychotic symptoms such as disorientation, aberrant behavior, coma and death are observed. Laboratory findings reveal hyperammonemia, citrullinemia, fatty liver and liver-specific decrease in a urea cycle enzyme, argininosuccinate synthetase (ASS). In some cases, hyperlipidemia, pancreatitis and hepatoma are accompanied with CTLN2. Citrin as a liver-type AGC plays a role in supplying aspartate to the cytosol for urea, protein and nucleotide synthesis by exchanging mitochondrial aspartate for cytosolic glutamate and proton, and transporting cytosolic NADH reducing equivalent to mitochondria as a member of malate aspartate shuttle essential for aerobic glycolysis. AGC is also important for gluconeogenesis from lactate. Although it is difficult to explain pathogenesis of the symptoms such as cholestasis in NICCD and liver-specific decrease of ASS protein in CTLN2 from the functions of the AGC, some are understandable by the loss of citrin functions. Many CTLN2 patients have been treated with a low protein and high carbohydrate diet and glycerol at the hyperammonemic coma. We argue that those treatments may result in fatty liver, hyperlipidemia, hyperammonemia and even death due to loss of the citrin functions. Loss of citrin first cause deficiency of aspartate in the cytosol, which results in an increase in cytosolic NADH/NAD(+) ratio and then activation of fatty acid synthesis pathway to compensate the aberrant ratio. This follows inhibition of fatty acid oxidation. The peculiar fondness for food of CTLN2 patients who like protein and dislike carbohydrate and sweets may be related to their metabolic requirements.
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PMID:Metabolic derangements in deficiency of citrin, a liver-type mitochondrial aspartate-glutamate carrier. 1619 99

Glycogen storage disorder type 1A (GSD 1A) is an inherited disorder of glycogen metabolism characterized by fasting hypoglycemia, lactic acidosis, hyperuricemia, and hyperlipidemia. These children have a higher risk of developing pancreatitis because of hypertriglyceridemia. Drug-induced pancreatitis accounts for a small proportion of cases of pancreatitis. The mechanism of drug-induced pancreatitis include hypersensitivity, direct toxic injury or indirectly by inducing hypertriglyceridemia. Propofol is often the drug of choice for induction of anesthesia in ambulatory surgical procedures. There are various reports in the literature describing pancreatitis induced by propofol. We present a 4-year-old girl with GSD 1A, who required tonsillectomy and adenoidectomy under general anesthesia. She developed acute pancreatitis in the postoperative period. Propofol was used as a general anesthetic and the postoperative incidence of pancreatitis is discussed.
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PMID:Acute pancreatitis after anesthesia with propofol in a child with glycogen storage disease type IA. 1671 86

A 7-year-old girl with severe hereditary pancreatitis underwent total pancreatectomy. A total of 160,000 islet equivalents (6400 islet/kg) were transplanted to the brachioradialis muscle of the right forearm. Her plasma C-peptide level was undetectable after pancreatectomy but increased to 1.37 ng/mL after 17 days; at this time point, her insulin requirement was 0.75 units of insulin/kg/day. At 5- and 27-months, her hemoglobin A1c (HbA1c) and insulin requirements were 4.5 and 5.3% and 0.3 and 0.18 units/kg/day, respectively. Basal and stimulated C-peptide levels were 0.67 +/- 0.07 and 3.36 +/- 1.37 ng/mL, respectively. Stimulated insulin levels were 30% higher in the islet-bearing arm compared to the contralateral arm after glucagon stimulation. After surgery and islet transplantation, the quality of life improved dramatically and she gained 8 kg of weight. In summary, a normal HbA1c, a low insulin requirement and the absence of recurrent hypoglycemia and the gradient of insulin between the arms indicate that the intramuscularly transplanted islets contribute to a long-term clinically significant metabolic control.
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PMID:Intramuscular autotransplantation of pancreatic islets in a 7-year-old child: a 2-year follow-up. 1809 67

Pancreatic infiltration with leukemic cells is a rare manifestation of acute lymphoblastic leukemia. There are only a few reported cases. We report the clinical and radiologic findings of a 4-year-old boy with mature B-cell acute lymphoblastic leukemia and pancreatic involvement. A computed tomography scan of his abdomen demonstrated diffuse hypodense lesions in the pancreas. Plasma amylase and lipase levels at that time were high, but no signs of hypoglycemia or hyperglycemia were observed. After 2 cycles of chemotherapy, the lesions in his pancreas, liver, and kidney disappeared, and his pancreatitis resolved as well. At 11 months' follow-up, after completion of therapy, the patient continues to be in remission.
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PMID:A case with mature B-cell acute lymphoblastic leukemia and pancreatic involvement at the time of diagnosis. 1817 91


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