Assessment of exocrine pancreatic function in children and adolescents with direct and indirect testing

Hopson, Puanani; Smadi, Yamen; Mehta, Vijay; Patel, Samit; Mehta, Devendra I.; Horváth, Károly

doi:10.3389/fped.2022.908542

Cited by 6 publications

(6 citation statements)

References 151 publications

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“…Somatostatin, in addition to atropine, is a potent inhibitor of cholecystokinin (CCK)-mediated release of pancreatic enzyme secretion. CCK usually acts via the CCK receptor on pancreatic acinar cells, intrapancreatic neurons, and afferent cholinergic neurons ( 6 ).…”

Section: Discussionmentioning

confidence: 99%

Acute Interstitial Nephritis and Oxalate Nephropathy After Rapid Pasireotide Response in Treatment-resistant Acromegaly

Hayes,

Penny,

Aivazian

et al. 2024

JCEM Case Reports

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We report a case of interstitial nephritis, likely secondary to oxalate nephropathy, due to the development of pancreatic exocrine dysfunction after commencement of pasireotide for acromegaly. Pasireotide is known to impair insulin secretion but can also impair pancreatic exocrine function, hypothezised to result from high-affinity binding of somatostatin receptors 1, 2, 3, and 5. This has been an advantage in postoperative tissue anastomoses after pancreatic surgery, but exocrine insufficiency has not been reported when used for the treatment of acromegaly. A 73-year-old woman, diagnosed with acromegaly, was unable to achieve biochemical control despite 2 surgical resections of an invasive mammosomatotroph pituitary tumor and treatment with cabergoline and maximal-dose lanreotide. The tumor expressed somatostatin receptor type 5 but not somatostatin receptor type 2, predicting good response from pasireotide, which was commenced at 40 mg every 4 weeks. IGF-1 rapidly normalized, but the patient presented with nausea, anorexia, and acute kidney injury. Renal biopsy revealed acute-on-chronic interstitial nephritis, with numerous oxalate crystals. Increased fecal fat globules were noted on fat stain (3+), supporting malabsorption as an etiology of secondary enteric hyperoxaluria. Renal function recovered to near baseline over months following pasireotide withdrawal and high-dose glucocorticoids.

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Section: Discussionmentioning

confidence: 99%

Acute Interstitial Nephritis and Oxalate Nephropathy After Rapid Pasireotide Response in Treatment-resistant Acromegaly

Hayes,

Penny,

Aivazian

et al. 2024

JCEM Case Reports

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“…Ultrasound is often the first radiological test used to evaluate the pancreatic anatomy but computer tomography (CT), or magnetic resonance cholangiopancreatography (MRCP), specifically secretin-enhanced MRCP, are more helpful to better delineate the pancreatic anatomy such as pancreatic atrophy, pancreatic lipomatosis and evidence of chronic pancreatitis such as pancreatic ductal disruptions, parenchymal calcifications 106 , 107 ( Figure 1A-D ). In young children, the CT or MRCP testing may require deep sedation or anesthesia.…”

Section: Diagnosis Of Epimentioning

confidence: 99%

Exocrine Pancreatic Insufficiency in Children – Challenges in Management

Sankararaman,

Schindler

2023

PHMT

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Cystic fibrosis (CF) is the leading etiology for exocrine pancreatic insufficiency (EPI) in children, followed by chronic pancreatitis, Shwachman-Diamond syndrome, and other genetic disorders. Management of EPI in children poses several unique challenges such as difficulties in early recognition, lack of widespread availability of diagnostic tests and limited number of pediatricspecific pancreatic centers. Pancreatic enzyme replacement therapy is the cornerstone of EPI management and in young children difficulties in administering pancreatic enzymes are frequently encountered. Patients with EPI also should be screened for fat-soluble vitamin deficiencies and receive appropriate supplementation. Among disorders with EPI in children, CF is the relatively well-studied condition, and most management recommendations for EPI in children come from expert consensus and conventional practice guidelines. The impact of EPI can be greater in children given their high metabolic demands and rapid growth. Early diagnosis and aggressive management of EPI prevent consequences of complications such as malnutrition, fat-soluble vitamin deficiencies, and poor bone health and improve outcomes. Management by multi-disciplinary team is the key to success.

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“…[9][10][11] Several studies have described the AAP phenotype and suggested underlying risk factors, which include older age at ALL diagnosis, higher cumulative dose of asparaginase, and certain host genome variants. [12][13][14][15][16] Potential long-term sequelae (e.g., organrelated symptoms, exocrine deficiency, and diabetes mellitus [DM]) could have a negative impact on growth, overall health, and quality of life, [17][18][19][20][21] however, this has not previously been systematically explored. Symptoms of pancreatic dysfunction may be unspecific and thus misinterpreted unless targeted screening is performed.…”

Section: Introductionmentioning

confidence: 99%

Pancreas‐related persisting sequelae in ALL survivors with a history of asparaginase‐associated pancreatitis: A part of the ALL‐STAR study

Skipper,

Birkebæk,

Jensen

et al. 2024

European J of Haematology

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ObjectivesAsparaginase‐associated pancreatitis (AAP) occurs in up to 18% of patients treated for acute lymphoblastic leukemia (ALL); however, long‐term sequelae are largely unexplored. We aimed to explore pancreatic sequelae among ALL survivors with and without AAP.MethodsWe investigated pancreatic sequelae in a national cohort of ALL survivors, aged 1–45 years at ALL diagnosis treated according to the NOPHO‐ALL2008 protocol and included sex‐ and age‐matched community controls.ResultsWe included 368 survivors (median follow‐up 6.9 years), including 47 survivors with AAP and 369 controls. The p‐lipase and p‐pancreas‐type amylase levels were lower in AAP survivors compared with both non‐AAP survivors (Medians: 23 U/L [IQR 14–32] and 18 U/L [IQR 10–25] versus 29 [IQR 24–35] and 22 [17–28], p < .001 and p = .002) and community controls (28 U/L [IQR 22–33] and 21 U/L [IQR 17–26], both p < .006). Fecal‐elastase was more frequently reduced in AAP survivors compared with non‐AAP survivors (7/31 vs. 4/144, p = .001). Persisting pancreatic sequelae were found in 15/47 of AAP survivors and 20/323 of non‐AAP survivors (p < .001), including diabetes mellitus in 2/39 of AAP survivors and 2/273 of non‐AAP survivors.ConclusionsALL survivors with AAP are at increased risk of persisting pancreatic dysfunction and require special attention during follow‐up.

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Assessment of exocrine pancreatic function in children and adolescents with direct and indirect testing

Cited by 6 publications

References 151 publications

Acute Interstitial Nephritis and Oxalate Nephropathy After Rapid Pasireotide Response in Treatment-resistant Acromegaly

Acute Interstitial Nephritis and Oxalate Nephropathy After Rapid Pasireotide Response in Treatment-resistant Acromegaly

Exocrine Pancreatic Insufficiency in Children – Challenges in Management

Pancreas‐related persisting sequelae in ALL survivors with a history of asparaginase‐associated pancreatitis: A part of the ALL‐STAR study

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