scholarly journals Idiopathic Pancreatitis in a Patient with a STAT3 Mutation

2016 ◽  
Vol 7 (1) ◽  
pp. ar.2016.7.0148 ◽  
Author(s):  
Chelsea Michaud ◽  
Brian Peppers ◽  
John Frith ◽  
Haig Tcheurekdjian ◽  
Robert Hostoffer

Background Hyperimmunoglobulin E syndrome (HIES) is a rare primary immunodeficiency characterized by recurrent skin infections with abscesses, recurrent pneumonias with pneumatoceles, and immunoglobulin E levels of >10 times the upper limit of normal. Case The patient described herein had a classic case of signal transducer and activator of transcription S (STAT3) deficiency associated with HIES diagnosed several years before this particular presentation. He demonstrated extraimmune manifestations of the disease as well, including characteristic facies and a history of skeletal fractures. In addition, the patient had several distinct episodes of idiopathic pancreatitis for which a full gastrointestinal workup had been performed. STAT3 mutation was confirmed by genotyping at the time of diagnosis of HIES. Conclusions STAT3, a mammalian protein that regulates cell growth, survival, and differentiation, has been linked to human pancreatic carcinogenesis as well as the above-mentioned immune deficiency. Mouse studies demonstrated that genetic ablation of STAT3 exacerbates the course of acute pancreatitis, whereas normal pancreatic STAT3 seems to have a protective effect against necrotizing pancreatitis. An association between STAT3 mutations and pancreatitis has not yet been revealed in humans. Here we describe a case of acute pancreatitis that presented in a patient with STAT3 mutation.

Author(s):  
Julia Cristina Coronado Arroyo ◽  
Marcio José Concepción Zavaleta ◽  
Eilhart Jorge García Villasante ◽  
Mikaela Kcomt Lam ◽  
Luis Alberto Concepción Urteaga ◽  
...  

AbstractAcute pancreatitis is a rare condition in pregnancy, associated with a high mortality rate. Hypertriglyceridemia represents its second most common cause. We present the case of a 38-year-old woman in the 24th week of gestation with a history of hypertriglyceridemia and recurrent episodes of pancreatitis. She was admitted to our hospital with acute pancreatitis due to severe hypertriglyceridemia. She was stabilized and treated with fibrates. Despite her favorable clinical course, she developed a second episode of acute pancreatitis complicated by multi-organ dysfunction and pancreatic necrosis, requiring a necrosectomy. The pregnancy was ended by cesarean section, after which three plasmapheresis sessions were performed. She is currently asymptomatic with stable triglyceride levels. Acute pancreatitis due to hypertriglyceridemia represents a diagnostic and therapeutic challenge in pregnant women, associated with serious maternal and fetal complications. When primary hypertriglyceridemia is suspected, such as familial chylomicronemia syndrome, the most important objective is preventing the onset of pancreatitis.


2017 ◽  
Vol 4 (2) ◽  
pp. 534
Author(s):  
MD. Shakeel ◽  
Syed Shahid Irfan

Background: Pancreatitis is an inflammatory process leading to abdominal pain, progressive destruction of the exocrine tissue and in some patients a loss of endocrine tissue as well. However owing to the tremendous reserve of pancreatic function, insufficiency may be at subclinical at least in the beginning of the disease.Methods: A cross sectional study was conducted which included the patients admitted with pancreatitis. For all the patients, meticulous records will be maintained regarding clinical features, family history of pancreatitis, alcohol intake, dietary habits, and stigmata of alcoholic liver diseases and by performing various investigations. 200 cases admitted, diagnosed to have pancreatitis.Results: The most common diagnosis was acute pancreatitis (48%) followed by chronic pancreatitis (12%), acute pancreatitis and pseudocyst (12%), acute on chronic pancreatitis (12%), necrotizing pancreatitis (8%) and acute pancreatitis with others (8%).Conclusions: Overall acute pancreatitis constituted 76%, chronic pancreatitis 12% and acute on chronic pancreatitis 12%.


2017 ◽  
Vol 11 (1) ◽  
pp. 120-126 ◽  
Author(s):  
Jennifer Reppucci ◽  
Michael Chang ◽  
Steven Hughes ◽  
Xiuli Liu

Eosinophilic pancreatitis is a rare form of recurrent acute pancreatitis that demonstrates distinct histologic features, including diffuse, periductal, acinar, and septal inflammatory infiltrates comprised of a pure or predominant population of eosinophils, eosinophilic phlebitis and arteritis, and localized eosinophilic infiltrates with pseudocyst formation. It is associated with elevated serum immunoglobulin E levels, an elevated eosinophil count with systemic manifestations, and eosinophilic infiltrates in other organs of the gastrointestinal tract. We present a case of eosinophilic pancreatitis in a 44-year-old man who was diagnosed after pancreatic resection for recurrent bouts of acute pancreatitis. While the gross and histologic evaluations matched other reported cases of eosinophilic pancreatitis, our patient had only minimal peripheral eosinophilia, no reported history of symptoms related to elevated eosinophilia or immunoglobulin E, and only mild eosinophilic infiltrates in his gallbladder.


PEDIATRICS ◽  
1963 ◽  
Vol 32 (1) ◽  
pp. 93-102
Author(s):  
Charles Frey ◽  
S. Frank Redo

Eighteen children with acute pancreatitis, not including those with cystic fibrosis of the pancreas, were encountered at The New York Hospital from 1932 to 1962. Steroid administration, congenital anomalies, mumps, operative trauma, periarteritis nodosa, intestinal obstruction, and malnutrition and infection were thought to be factors in the etiology of acute pancreatitis in these patients. The ages of the children ranged from 1 day to 10 years. Fourteen of the children were male and four female. Fourteen patients died. Significant postmortem findings included kidney and renal lesions in addition to pancreatitis. Survivors were children 5 to 10 years of age whose pancreatitis was attributable to mumps, operative trauma, and annular pancreas. Liver and renal dysfunction in patients with pancreatitis appeared to augur a poor prognosis. All patients thought to have "idiopathic" pancreatitis had a history of poor nutrition and/or infection, which probably are etiologic factors in the development of pancreatitis in these children.


1995 ◽  
Vol 31 (5-6) ◽  
pp. 11-17 ◽  
Author(s):  
N. Charoenca ◽  
R. S. Fujioka

An association between using coastal waters for recreation and staphylococcal skin infections has been reported by canoe paddlers and several physicians in Hawaii. A retrospective epidemiological/microbiological monitoring study was undertaken to determine the association between S aureus skin infections in youngsters (4 months to 16 years of age) and their exposure to recreational use of coastal waters. Telephone interviews were conducted of 53 patients with such skin infections and 53 similar (controlled for age and sex) patients with no infection. A significant association between skin infection and water exposure was found, the odds showing that those developing skin infection caused by S aureus were 4 times more likely to have had a history of seawater contact than the control group. Moreover, the antibiotic sensitivity patterns and phage types of S aureus isolated from patients were similar to those isolated from seawater at bathing beaches.


2021 ◽  
Vol 20 (1) ◽  
Author(s):  
Robert J. Sanchez ◽  
Wenzhen Ge ◽  
Wenhui Wei ◽  
Manish P. Ponda ◽  
Robert S. Rosenson

Abstract Background This retrospective cohort study assessed the annualized incidence rate (IR) of acute pancreatitis (AP) in a nationally representative US adult population, as well as the variation in the risk of AP events across strata of triglyceride (TG) levels. Methods Data were obtained from IQVIA’s US Ambulatory Electronic Medical Records (EMR) database linked with its LRxDx Open Claims database. Inclusion criteria included ≥1 serum TG value during the overlapping study period of the EMR and claims databases, ≥1 claim in the 12-month baseline period, and ≥ 1 claim in the 12 months post index. All TG measurements were assigned to the highest category reached: < 2.26, ≥2.26 to ≤5.65, > 5.65 to ≤9.94, > 9.94, and > 11.29 mmol/L (< 200, ≥200 to ≤500, > 500 to ≤880, > 880, and > 1000 mg/dL, respectively). The outcome of interest was AP, defined as a hospitalization event with AP as the principal diagnosis. Results In total, 7,119,195 patients met the inclusion/exclusion criteria, of whom 4158 (0.058%) had ≥1 AP events in the prior 12 months. Most patients (83%) had TGs < 2.26 mmol/L (< 200 mg/dL), while < 1% had TGs > 9.94 mmol/L (> 880 mg/dL). Overall, the IR of AP was low (0.08%; 95% confidence internal [CI], 0.08–0.08%), but increased with increasing TGs (0.08% in TGs < 2.26 mmol/L [< 200 mg/dL] to 1.21% in TGs > 11.29 mmol/L [> 1000 mg/dL]). In patients with a prior history of AP, the IR of AP increased dramatically; patients with ≥2 AP events at baseline had an IR of 29.98% (95% CI, 25.1–34.9%). Conclusion The risk of AP increases with increasing TG strata; however, the risk increases dramatically among patients with a recent history of AP.


2021 ◽  
Vol 12 (6) ◽  
Author(s):  
Michittra Boonchan ◽  
Hideki Arimochi ◽  
Kunihiro Otsuka ◽  
Tomoko Kobayashi ◽  
Hisanori Uehara ◽  
...  

AbstractThe sensing of various extrinsic stimuli triggers the receptor-interacting protein kinase-3 (RIPK3)-mediated signaling pathway, which leads to mixed-lineage kinase-like (MLKL) phosphorylation followed by necroptosis. Although necroptosis is a form of cell death and is involved in inflammatory conditions, the roles of necroptosis in acute pancreatitis (AP) remain unclear. In the current study, we administered caerulein to Ripk3- or Mlkl-deficient mice (Ripk3−/− or Mlkl−/− mice, respectively) and assessed the roles of necroptosis in AP. We found that Ripk3−/− mice had significantly more severe pancreatic edema and inflammation associated with macrophage and neutrophil infiltration than control mice. Consistently, Mlkl−/− mice were more susceptible to caerulein-induced AP, which occurred in a time- and dose-dependent manner, than control mice. Mlkl−/− mice exhibit weight loss, edematous pancreatitis, necrotizing pancreatitis, and acinar cell dedifferentiation in response to tissue damage. Genetic deletion of Mlkl resulted in downregulation of the antiapoptotic genes Bclxl and Cflar in association with increases in the numbers of apoptotic cells, as detected by TUNEL assay. These findings suggest that RIPK3 and MLKL-mediated necroptosis exerts protective effects in AP and caution against the use of necroptosis inhibitors for AP treatment.


PEDIATRICS ◽  
1994 ◽  
Vol 94 (6) ◽  
pp. 895-901
Author(s):  
Anne L. Wright ◽  
Catharine J. Holberg ◽  
Marilyn Halonen ◽  
Fernando D. Martinez ◽  
Wayne Morgan ◽  
...  

Objective. To investigate the natural history of and risk factors for allergic rhinitis in the first 6 years of life. Methods. Parents of 747 healthy children followed from birth completed a questionnaire when the child was 6 years old. Data were obtained regarding physician-diagnosed allergic rhinitis (PDAR), associated symptoms, and age at onset. Risk-factor data were taken from earlier questionnaires, and data regarding immunoglobulin E (IgE) and skin-test reactivity were obtained at age 6. Results. By the age of 6, 42% of children had PDAR. Children whose rhinitis began in the first year of life had more respiratory symptoms at age 6 and were more likely to have a diagnosis of asthma. Early introduction of foods or formula, heavy maternal cigarette smoking in the first year of life, and higher IgE, as well as parental allergic disorders, were associated with early development of rhinitis. Risk factors for PDAR that remained significant in a multivanate model included maternal history of physician-diagnosed allergy (odds ratio: 2.2, 95% confidence interval: 1.35-3.54), asthma in the child (4.06, 2.06-7.99), and IgE greater than 100 IU/mL at age 6 (1.93, 1.18-3.17). The odds for atopic as opposed to nonatopic PDAR were significantly higher only among those with high IgE and those who had dogs. Conclusion. Allergic rhinitis developing in the first years of life is an early manifestation of an atopic predisposition, which may be triggered by early environmental exposures.


1993 ◽  
Vol 27 (1) ◽  
pp. 36-37 ◽  
Author(s):  
Anthony E. Zimmermann ◽  
Brian G. Katona ◽  
Joginder S. Jodhka ◽  
Richard B. Williams

OBJECTIVE: To report a case of probable ceftriaxone-induced acute pancreatitis. CASE SUMMARY: A patient with a history of short-bowel syndrome on home total parenteral nutrition developed fever, chills, and right flank pain. She was diagnosed with gram-negative catheter sepsis and prescribed antibiotic therapy to be administered for four weeks. After completion of the first week of therapy, the antibiotic regimen was changed to intravenous injections of ceftriaxone to be given daily at home. Prior to discharge the patient developed acute abdominal pain, leukocytosis, jaundice, and markedly elevated lipase and amylase concentrations consistent with acute pancreatitis. The patient's condition improved upon discontinuation of the ceftriaxone and the remainder of her stay was uneventful. DISCUSSION: There is only one other case report in the literature of probable ceftriaxone-induced pancreatitis. Multiple other medications have been implicated in causing acute pancreatitis. The exact mechanism of this uncommon adverse effect of ceftriaxone is unknown. CONCLUSIONS: There was a temporal relationship between the development of this patient's signs and symptoms and the administration of ceftriaxone. We could not identify any other factors that may have been responsible for the development of her acute pancreatitis. Ceftriaxone should be considered as a possible etiologic agent in patients who present with acute abdominal pain and elevated lipase and amylase concentrations.


Sign in / Sign up

Export Citation Format

Share Document