Hereditary pancreatitis: An updated review in pediatrics

Panchoo, Arvind V; VanNess, Grant H; Rivera-Rivera, Edgardo; Laborda, Trevor J.

doi:10.5409/wjcp.v11.i1.27

Cited by 12 publications

(6 citation statements)

References 51 publications

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“…Both the CTRC and the R75Q mutations are autosomal recessive and are less commonly identified. 4 In the pediatric study, approximately 27% of the patients in this study were found to have the same mutation as our patient, the N34S mutation in SPINK1. 6 While the exact prevalence is unknown, chronic pancreatitis is rare in children.…”

Section: In Summarysupporting

confidence: 60%

“…This has led others to consider the mutation to be more of a disease modifier. 4 However, our patient had a homozygous mutation, which could contribute to the presentation. Several studies have reported a tendency for earlier onset of symptoms with the homozygous mutation, but due to the decreased incidence, it is not statistically significant.…”

Section: In Summarymentioning

confidence: 65%

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Persistent Eosinophilia and Fever in Pancreatitis: A Clinical Conundrum

Noel

Fields

Rami

et al. 2022

Journal of Investigative Medicine High Impact Case Reports

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A healthy 11-year-old girl presents with epigastric abdominal pain, fever, weight loss, and decreased appetite for 1 month. On physical examination, she appears ill, dehydrated, and cachectic. Her abdominal examination is significant for large ascites with a fluid wave and is nontender to palpation. Her labs show leukocytosis with an eosinophilic-predominant granulocytosis and an absolute eosinophil count of 6800/mm3. She has elevated serum inflammatory markers, hypoalbuminemia, and lipase is 5000 U/L. Magnetic resonance cholangiopancreatography (MRCP) shows an irregular and dilated pancreatic duct, so she had an endoscopic retrograde cholangiopancreatography with pancreatic stent placement, paracentesis, and colonoscopy. Her peritoneal fluid was significant for an eosinophilic-predominant granulocytosis with no evidence of malignancy on flow cytometry. All other studies and cultures did not reveal an etiology. She initially showed improvement, 18 days later she developed a fever, night sweats, tachycardia, and abdominal distention. Empiric antibiotics were initiated due to concern for infected pancreatic necrosis versus spontaneous bacterial peritonitis. Repeat MRCP showed interval development of 2 peripancreatic fluid collections and re-accumulation of ascites. She continued to have daily fever ranging from 39°C to 40°C. Repeat paracentesis and evaluation of her peritoneal fluid showed resolution of eosinophilia with an elevated neutrophil count, negative Gram stain, and no growth on culture. She completed a 10-day course of antibiotics, however, remained febrile with elevated inflammatory markers and leukocytosis throughout her hospitalization. A genetic panel to evaluate for a hereditary cause of chronic pancreatitis was sent and returned positive for a mutation of the serine protease inhibitor Kazal type 1.

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Section: In Summarysupporting

confidence: 60%

Section: In Summarymentioning

confidence: 65%

Persistent Eosinophilia and Fever in Pancreatitis: A Clinical Conundrum

Noel

Fields

Rami

et al. 2022

Journal of Investigative Medicine High Impact Case Reports

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“…Наследственный панкреатит (HХП) -очень редкое заболевание, которое характеризуется проявлением хронического панкреатита в раннем детском возрасте [20][21][22][23][24]. Это аутосомно-доминантное заболевание с пенетрантностью генов примерно 80% [20].…”

Section: наследственный панкреатитunclassified

“…Это аутосомно-доминантное заболевание с пенетрантностью генов примерно 80% [20]. Истинную распространённость наследственного панкреатита трудно определить, учитывая нечастое генетическое тестирование, по оценкам мировых исследований, она составляет 0,3 на 100 000 человек [21,23]. На сегодняшний день в мире ведётся активная работа по изучению генетических аспектов развития панкреатита.…”

Section: наследственный панкреатитunclassified

Surgical treatment of chronic pancreatitis in children

Kislenko,

Razumovsky,

Kholostova

et al. 2024

jour

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Chronic pancreatitis in children is a continuous or recurrent disease characterized by irreversible inflammatory and destructive changes in the pancreas (PG), progressive fibrosis, accompanied by a gradual decrease in its exocrine and endocrine functions. In recent decades, there has been a significant increase in patients with CP; the incidence in children is estimated at 2 cases per 100,000 people. According to the literature, pancreatic cancer in the outcome of chronic pancreatitis in childhood is 6.2%. Mortality rates for CP vary in different studies: 15-20% of children with CP die from complications and secondary disorders. The purpose of the study is to study current problems and determine the prospects for the treatment of chronic pancreatitis in children based on a review of modern literature. Materials and methods. As part of the preparation, a systematic review of the world and domestic literature on the surgical treatment of chronic pancreatitis in children was carried out. Results. A literature search confirmed the lack of consensus regarding the surgical treatment of CP in children. The small number of observations does not allow us to analyze the long-term results of surgical treatment of children with CP. Patients with hereditary pancreatitis pose a particular challenge; treatment algorithms for this group of patients are not described in the literature. Also in the literature there are isolated descriptions of complications with CP in children; there are no reliable results of the effectiveness of surgical intervention for endocrine and exocrine insufficiency in children. It is necessary to conduct scientific and practical research to develop indications for surgical treatment of children with CP and to verify specific complications.

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“…Mutation in the PRSS1 is a genetic risk factor for pancreatitis. [7] However, taking into consideration laboratory tests on cancer cells, it would be necessary to repeat the test after treatment in order to confirm mutation in cells. The most reliable test would be the 1 carried before the disease.…”

Section: Introductionmentioning

confidence: 99%

Severe complications in the induction phase of therapy in a pediatric patient with T-cell acute lymphoblastic leukemia: A case report

Rocka,

Woźniak,

Lejman

et al. 2023

Medicine

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Rationale: Acute lymphoblastic leukemia (ALL) represents approximately 1-quarter of all new cases of childhood cancer. Although overall survival following diagnosis has improved in recent years, the toxicity of chemotherapy remains a concern. Patient concerns: We describe an 11-year-old male patient diagnosed with T-cell precursor ALL who developed compounded complications during the induction phase of chemotherapy. Patient was hospitalized in the Department of Pediatric Hematology, Oncology, and Transplantology of the Medical University of Lublin, Poland. The patient’s induction therapy was started according to the AIEOP-BFM ALL 2017 protocol IAp (International Collaborative Treatment Protocol for Children and Adolescents with Acute Lymphoblastic Leukemia). Diagnoses: Patient developed compounded complications such as cholecystitis, hepatotoxicity, pancreatitis and myelosuppression. Interventions: The patient was treated with leukapheresis, received a broad-spectrum antibiotic, potassium supplementation and hepatoprotective treatment and laparotomy cholecystectomy. Outcomes: In the available literature, there is a limited amount of similar clinical cases with multiple complications in pediatric patients with ALL. Toxicities cause delays in the treatment of the underlying disease. Lessons: In children with acute lymphoblastic leukemia, there are side effects during the treatment such as cholecystitis and pancreatitis. Complications during treatment require a quick response and modification of disease management. Abdominal ultrasound performed before treatment makes it possible to observe the dynamics of lesions. Genetic mutation analysis could allow us to more precisely respond to the possible susceptibility to and appearance of complications after the use of a given chemotherapeutic agent.

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Hereditary pancreatitis: An updated review in pediatrics

Cited by 12 publications

References 51 publications

Persistent Eosinophilia and Fever in Pancreatitis: A Clinical Conundrum

Persistent Eosinophilia and Fever in Pancreatitis: A Clinical Conundrum

Surgical treatment of chronic pancreatitis in children

Severe complications in the induction phase of therapy in a pediatric patient with T-cell acute lymphoblastic leukemia: A case report

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