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Query: UMLS:C0000737 (abdominal pain)
 31,184 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Fibrosing colonopathy, characterized by dense submucosal fibrosis in the large bowel, is a disorder associated with bowel dysfunction in patients with cystic fibrosis who receive pancreatic enzyme supplementation. Most commonly, patients present with a distended abdomen and abdominal pain. Radiographs frequently demonstrate colonic wall thickening and luminal narrowing. Here I describe a neonate with cystic fibrosis who presented with both clinical and histological features of fibrosing colonopathy who had not received pancreatic enzymes. This report expands our understanding of the pathogenesis of fibrosing colonopathy.
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PMID:Cystic fibrosis with fibrosing colonopathy in the absence of pancreatic enzymes. 1046 74

Hereditary pancreatitis (HP) is an autosomal dominant disorder characterized by recurrent acute attacks of severe abdominal pain with an onset in early childhood. Many HP patients progress to complicated chronic pancreatitis and/or pancreatic cancer. Initially, a single mutation R117H in the cationic trypsinogen gene was detected in all affected members of five unrelated HP families. Further studies identified a second mutation (N21L) in two HP families without the R117H mutation. Before the association between cationic trypsinogen and HP was found, we detected a cystic fibrosis transmembrane conductance regulator (CFTR) gene mutation (L327R) in all affected individuals of a family with HP. We therefore performed a mutational analysis for R117H and N21L in cationic trypsinogen in this and three additional unrelated families with HP. The R117H mutation was detected in all 9 affected members of three HP families and in 3 asymptomatic but at-risk relatives. However, neither the R117H nor the N21L mutation in the cationic trypsinogen were found in the HP family with the L327R alteration in CFTR. The L327R allele segregates with the disease within this HP family and was not detected on 360 unrelated Caucasian non-CF chromosomes. Although close to 800 different mutations have been detected in the CF gene of cystic fibrosis patients, L327R is a new alteration, not yet reported in connection with CF. The results of this study indicate that the CFTR gene may play a role in the etiology of minority of cases with HP and suggest that hereditary pancreatitis is genetically heterogeneous disease.
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PMID:Evidence that hereditary pancreatitis is genetically heterogeneous disorder. 1065 40

With the exception of cystic fibrosis and hereditary pancreatitis, case reports about pancreatitis in children have rarely been mentioned. We report here an 11-year-old boy with type V hyperlipidemia, who suffered from two episodes of acute pancreatitis. Sudden onset of severe upper abdominal pain, fever, and hypertriglyceridemia were the common presentations. Initial treatments including analgesics, fasting, parenteral nutrition support and following diet control with medium-chain triglycerides seem to be successful in our case.
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PMID:Recurrent pancreatitis secondary to type V hyperlipidemia: report of one case. 1110 May 29

The incidence of acute and chronic pancreatitis in childhood is unknown. Both are associated with significant morbidity and mortality. The role of clinical suspicion is vital as these disorders can be misdiagnosed easily. Molecular basis of several disorders are being elicited and promising new diagnostic tests are being developed, including tests to assess fat malabsorption by non-invasive methods. Etiological spectrum of acute pancreatitis ranges from congenital, structural or inherited disorders to trauma, infections, drug toxicity and interventions such as endoscopic retrograde cholangiopancreatography and organ transplantation. Chronic tropical calculus pancreatitis is a progressive disorder that presents in childhood with recurrent abdominal pain, progressing to diabetes by puberty. Idiopathic recurrent pancreatitis has recently been associated with higher frequency of cystic fibrosis gene mutations. Therapeutic use of lexipafant opens the field to new powerful therapies designed to reduce the systemic inflammatory response syndrome and thus reduce the morbidity and mortality significantly.
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PMID:Acute and chronic pancreatitis in childhood. 1113 75

We report on the case of a 35 year-old woman who was initially admitted for acute pancreatitis in october 1995. The patient was suffering from asthma (since childhood) and diffuse abdominal pain (since adolescence). The diagnosis of cystic fibrosis was made fortuitously during a sterility evaluation. After extensive etiological screening the acute pancreatitis was considered to be a manifestation of the cystic fibrosis. Despite therapy with pancreatic enzymes, the patient continued to suffer from chronic abdominal pain. High intake of analgesics was required. Until December 1995, the patient was repeatedly admitted for episodes of acute pancreatitis. In January 1996, we initiated a preventive treatment with subcutaneous octreotide between 100 and 200 microgram, three time a day. Thereafter, there were fewer episodes of pancreatitis and the consumption of analgesics decreased. Side effects of octreotide were intermittent diarrhea and development of cholelithiasis that was complicated by biliary migration in November 1998. In June 1999, the prolonged-release form of the molecule was given without modification of the efficacy.
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PMID:[Repetitive acute pancreatitis in a late-diagnosed cystic fibrosis: prevention of relapses by octreotide in the long term]. 1152 Nov 9

By 1996, the median survival of patients with cystic fibrosis (CF) in North America had increased to 31 years. With the markedly improved life expectancy, many CF patients are now adults. There is an associated increased risk of certain colonic disorders, and the emergence of other previously unrecognized disorders, in adult CF patients. The distal intestinal obstruction syndrome (DIOS), which is more common in older patients, is a frequent cause of abdominal pain. Intussusception may complicate DIOS; other differential diagnoses include appendiceal disease, volvolus, Crohn's disease, fibrosing colonopathy and colonic carcinoma. The diagnosis of acute appendicitis, although uncommon in patients with CF, is often delayed, and appendiceal abscess is a frequent complication. The prevalence of Crohn's disease in CF has been shown to be 17 times that of the general population. Right-sided microscopic colitis is a recently recognized entity in CF of uncertain clinical significance. Fibrosing colonopathy has been confined mostly to children with CF, attributed to the use of high strength pancreatic enzyme supplements, but it has been reported in three adults. Nine cases of carcinoma of the large intestine have been reported worldwide, associated with an apparent excess risk of digestive tract cancers in CF. Despite high carrier rates of Clostridium difficile in patients with CF, pseudomembranous colitis is distinctly rare, but severe cases complicated by toxic megacolon have been reported. In these patients, watery diarrhea is often absent. Adult CF patients with refractory or unexplained intestinal symptoms merit thorough investigations.
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PMID:Colonic disorders in adult cystic fibrosis. 1157 1

We describe a 19-year-old cystic fibrosis patient, with pancreatic insufficiency since the age of 4 who presented at the age of 13 with postprandial abdominal pain. Ultrasonography and computed tomography showed several pancreatic cysts that progressively increased in diameter over 6 years. The lack of association with clinical and biochemical signs of acute pancreatitis is highlighted.
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PMID:Pancreatic cystosis in cystic fibrosis: case report. 1190 32

Chronic pancreatitis is a rare differential diagnosis of obstructive jaundice and/or recurrent abdominal pain in childhood and adolescence. The hereditary calcifying and the noncalcifying obstructive form are the two major forms of juvenile chronic pancreatitis. Other causes include cystic fibrosis, hyperparathyroidism, hyperlipoproteinemia and ascariasis. Even less common is the so called idiopathic or fibrosing pancreatitis. Since the first description by Comfort in 1946 only 41 further cases of juvenile idiopathic fibrosing pancreatitis have been published. An association with gene mutations (PRSS1, SPINK1, CTFR-5T genotype) is suspected. We report the cases of a 17-year-old male patient who presented with painless obstructive jaundice and a 16-year-old female patient who presented with abdominal pain and obstructive jaundice. Both patients underwent surgical treatment with duodenum-preserving pancreatic head resection. The relevant literature with special regard to modern pancreatic surgery is reviewed to give an overview about this rare but surgically treatable pediatric condition, which merits the attention of pediatricians and gastroenterologists in cases of children and adolescents suffering from unexplained abdominal pain.
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PMID:Juvenile idiopathic fibrosing pancreatitis. 1206 96

Cystic fibrosis is rare in the Asian population, and is often associated with consanguinity and rare genotypes. We report on a 23-year-old Asian man from a consanguineous pedigree referred to the regional cystic fibrosis unit after a diagnosis of congenital bilateral absence of the vas deferens during investigations for infertility. A detailed history revealed several previous episodes of acute pancreatitis. Full diagnostic appraisal showed homozygosity for a novel cystic fibrosis transmembrane conductance regulator (CFTR) gene mutation, but normal sweat test and nasal potential difference studies. An endoscopic retrograde cholangiopancreatogram (ERCP) showed chronic pancreatitis with bulky side branches. The vas deferens and the pancreas appeared exquisitely sensitive to mild CFTR dysfunction. Patients with cystic fibrosis and unexplained upper abdominal pain should be screened for pancreatitis, and consideration should be given to screening patients with idiopathic pancreatitis for mutations in the CFTR gene.
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PMID:Cystic fibrosis presenting as acute pancreatitis and obstructive azoospermia in a young adult male with a novel mutation in the CFTR gene. 1242 49

The authors report the case of a 26-year-old woman with hepatomegaly, who had recurrent and progressive nausea and abdominal pain. Computed tomography and magnetic resonance imaging demonstrated fatty replacement of the entire pancreas resulting from cystic fibrosis. The pulmonary disease was totally asymptomatic. Fatty replacement of the pancreas is the most frequent pattern in older patients with cystic fibrosis. This pattern correlates with pancreatic exocrine dysfunction.
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PMID:[Fatty replacement of the pancreas in a patient with asymptomatic pulmonary cystic fibrosis]. 1275 56


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