Pancreatitis in pediatrics

Pancreatitis is an inflammatory condition of the pancreas. Two major forms : acute pancreatitis (is reversible) and chronic pancreatitis(is irreversible).

1. PANCR EATITIS By Dr NDAYISABA CORNEILLE

2. INTRODUCTION Pancreatitis is an inflammatory condition of the pancreas. Two major forms : acute pancreatitis (is reversible) and chronic pancreatitis ( is irreversible ) . Acute pancreatitis is more prevalent, and most patients have a single episode of pancreatitis. A small number of patients have recurrent episodes of acute pancreatitis and are at risk of developing Chronic Pancreatitis. Research demonstrated differences between pancreatitis in children and adults, particularly in presentation, etiology, prognosis, and nature of acute recurrent pancreatitis (ARP ). 1/18/2020 Dr NDAYISABA CORNEILLE

3. P ancreas The healthy pancreas is protected by : Pancreatic proteases that are synthesized as inactive proenzymes; Digestive enzymes that are segregated into secretory granules at pH 6.2 with low calcium concentration, which minimizes trypsin activity; The presence of protease inhibitors both in the cytoplasm and zymogen granules; enzymes that are secreted directly into the ducts. 1/18/2020 Dr NDAYISABA CORNEILLE

4. ACUTE PANCREATITIS Acute pancreatitis, the most common pancreatic disease in children .Acute pancreatitis can occur in mild and severe forms. in general, mild pancreatitis is limited to the pancreas and the peripancreatic fat, whereas severe disease includes pancreatic necrosis, involvement of other organs , cardiovascular collapse, infection, or fluid collections 1/18/2020 Dr NDAYISABA CORNEILLE

5. Acute Pancreatitis Pathophysiology Acute pancreatitis results from injury of the pancreas and a subsequent inflammatory response that may involve adjacent and distant tissues and organs. an event that initiates a process of acinar cell injury such as Pancreatic duct obstruction(Due to the effect of lipase, any obstruction will cause fat necrosis and produce an inflammatory response). Primary acinar cell injury (it can be damaged by ischemia , viral infections (e.g., mumps) , Drugs , Direct trauma to the pancreas.). Defective intracellular transport of proenzymes within acinar cells. The cell injury produces pancreatic edema and a local inflammatory response, with release of inflammatory mediators. The production of cytokines and chemokines provoke a systemic inflammatory response. The magnitude of this inflammatory response determines the clinical severity of acute pancreatitis and can lead to complications such as pancreatic necrosis, shock, and distant organ failure . 1/18/2020 Dr NDAYISABA CORNEILLE

6. C alcium signals initiate the premature intracellular activation of trypsinogen to trypsin . Trypsin, in turn, activates other digestive proenzymes( zymogens most notably trypsinogen ) Normally, trypsinogen is converted into trypsin (in duodenum ) where it assists in the digestion of proteins . Then trypsinogen meet with lysosomal enzymes (specifically  cathepsin ), which activate trypsinogen to trypsin. This lead to inflammation, edema, vascular injury, and even cellular death 1/18/2020 Dr NDAYISABA CORNEILLE

7. etiology of acute pancreatitis in children 1.Common : a. Biliary disorders such as sepsis, shock (alone or with sepsis), hemolytic uremic syndrome, and systemic lupus erythematosus b. Systemic conditions such as Brain tumor, DM, Malnutrition, Peptic ulcer, Renal failure c. Medications such as valproic acid,  Acetaminophen (overdose), Cimetidine, Erythromycin, Methyldopa, Tetracycline,   Metronidazole, Isoniazid,… d. Traumatic such as Blunt injury, Burns, Hypothermia, Surgical trauma,….. e. Idiopathic 2.Less common : a. Infection such as   Hepatitis A, B; Influenza A, B; Malaria, Measles,Rubelle,mumps b. Genetic/hereditary disorders such as Cationic trypsinogen gene, Cystic fibrosis gene, SPINK-1 gene( serine protease inhibitor Kazal type 1) 3.Rare : a. Autoimmune pancreatitis occurs in two forms (types 1 and 2) type 2 associated with Inflammatory bowel disease,type 1 no elevation of immunoglobulin G4 (IgG4) this respond to corticosteroid therapy b. Anatomic pancreaticobiliary abnormalities such as pancreas divisum , abnormal junction of the common bile duct and main pancreatic duct (common channel syndrome), choledochal cysts, … 1/18/2020 Dr NDAYISABA CORNEILLE

8. CLINICAL MANIFESTATIONS. severe abdominal pain (87%) persistent vomiting (64%) and fever. The pain is epigastric or in either upper quadrant and steady . The abdomen may be distended and tender(77%)[lead to abdominal guarding,rebound tenderness]. A mass may be palpable. The pain increases in intensity for 24–48 hr , during which time vomiting may increase the patient may require hospitalization for dehydration and may need fluid and electrolyte therapy. The prognosis for complete recovery in the acute uncomplicated case is excellent Mild acute pancreatitis 1/18/2020 Dr NDAYISABA CORNEILLE

9. Severe acute pancreatitis Severe acute pancreatitis is rare in children . P atient present with : severe nausea, vomiting, and abdominal pain. Shock , high fever, jaundice, ascites, hypocalcemia, and pleural effusions can occur. A bluish discoloration may be seen around the umbilicus ( Cullen sign) or in the flanks ( Grey Turner sign). The pancreas is necrotic and can be transformed into an inflammatory hemorrhagic mass. The mortality rate, which is ≈25%, is related to the systemic inflammatory response syndrome with multiple organ dysfunction (MOD): shock, renal failure, acute respiratory distress syndrome, disseminated intravascular coagulation, massive gastrointestinal bleeding, and systemic or intra-abdominal infection. Has a poor prognosis. 1/18/2020 Dr NDAYISABA CORNEILLE

10. DIAGNOSIS Most children (≥90 %) have mild disease . Acute pancreatitis in pediatric patients requires at least two of three criteria : (1) abdominal pain ( ie , abdominal pain of acute onset, especially in the epigastric region ); (2) serum amylase or lipase activity at least three times greater than the upper limit of normal; (3) imaging findings compatible with acute pancreatitis . use computed tomography (CT) of the abdomen gives information about the presence or absence of pancreatic necrosis, pancreatic enlargement, a pancreatic masses, fluid collections, and abscesses Use of Endoscopic ultrasonography (EUS ) and magnetic resonance cholangiopancreatography (MRCP) magnetic resonance imaging of the biliary tree and surrounding structures (pancreas and liver ) but are very expensive . Other laboratory abnormalities that may be present in acute pancreatitis include hemoconcentration( increased concebtration of cell and solids in the blood usually result from loss of fluid to tissue ), coagulopathy,hypocalcemia,hyperglycemia 1/18/2020 Dr NDAYISABA CORNEILLE

11. TREATMENT Mild acute pancreatitis Fluid replacement ( first 12-24h ) Aggressive hydration at a rate of 5 to 10 mL/kg per hour of isotonic crystalloid solution (e.g., normal saline or lactated Ringer’s solution)  In severe volume depletion that manifests as hypotension and tachycardia, more rapid repletion with 20 mL/kg of intravenous fluid given over 30 minutes followed by 3 mL/kg/hour for 8 to 12 hours Pain control such as Acetamophin Nutritional support ( within 48h of admission ): oral intake in mild disease and in absence of nausea/vomiting and abdominal pain The treatment of severe acute pancreatitis may involve : enteral or total parenteral nutrition, antibiotics, gastric acid suppression and peritoneal lavage to reduce the risk of secondary infection . Endoscopic therapy may be of benefit when pancreatitis is caused by anatomic abnormalities, such as strictures or stones . Surgical therapy of acute pancreatitis is rarely required, but may include drainage of necrotic material or abscesses. management of a patient with acute pancreatitis consists of supportive care with fluid resuscitation, antiemetics , pain control , nothing by mouth, and nutritional support . (basic RX) { Mild ACUTE PANCREATITIS (use basic RX) Then on Severe acute pancreatitis(use Basic Rx +ICU)} 1/18/2020 Dr NDAYISABA CORNEILLE

12. Clinical manifestation Biochemical examination Imaging studies Diagnosis Physical findings .abdominal pain,vomiting , Cullen sign, Grey Turner sign. Chest & abdominal x-ray Ultrasonography,CT,MRCP Serum amylase,lipase leval Other pancreatic enzyme Laboratory findings coagulopathy,hyperglycemia Severity assesment severe <basic RX+ICU> Mgt of multiorgan failure Prevention & RX of infection Surgical therapy Non severe <Basic RX> fluid resuscitation, antiemetics , pain control nutritional support Recovery period Reduction of drug,Oral intake 1/18/2020 Dr NDAYISABA CORNEILLE

13. Acute Recurrent Pancreatitis Acute Recurrent Pancreatitis defined as at least two episodes of acute pancreatitis per year, or more than three episodes over a lifetime, in a patient without CP or a pancreatic pseudocyst . Etiology : including biliary disease, anatomic pancreaticobiliary abnormalities, inflammatory bowel disease, and autoimmune pancreatitis,.. 1/18/2020 Dr NDAYISABA CORNEILLE

14. Chronic Pancreatitis Chronic Pancreatitis is defined as a process leading to irreversible destruction of the pancreatic parenchyma and ducts and loss of exocrine function. Many of these patients have a history of ARP before the irreversible changes in pancreatic anatomy and function become apparent. The cause of Chronic Pancreatitis is usually idiopathic or associated with mutations in PRSS-1, SPINK-1, CFTR, or CTRC genes, alone or in combination . 1/18/2020 Dr NDAYISABA CORNEILLE

15. DIAGNOSIS The diagnosis of CP is clinical and based on a combination of symptoms , imaging studies , and functional insufficiency . It is important to consider all of these parameters when CP is suspected in a patient, because diagnosis often is delayed. With advanced disease, amylase and lipase levels will not be elevated, even in the presence of disabling pain . imaging such as CT,.. functional insufficiency :this identify pancreatic insufficiency and support the diagnosis of CP 1/18/2020 Dr NDAYISABA CORNEILLE

16. TREATMENT pain control :acetaminophen may be effective Endoscopic treatment for CP should be considered only when ductal strictures or pancreatic duct stones are present or for symptomatic pseudocysts . Total pancreatectomy with islet cell autotransplant is currently offered to patients who have genetic causes of pancreatitis Pancreatic insufficiency is treated with pancreatic enzyme replacement therapy. The goal is to restore digestive function and maintain weight gain and growth 1/18/2020 Dr NDAYISABA CORNEILLE

17. REFERENCE 1. .2 American academy of peadiatric accessed on http ://pedsinreview.aappublications.org/ Kliegman : Nelson Textbook of Pediatrics, 18th ed. 1/18/2020 Dr NDAYISABA CORNEILLE