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CHRONIC PANCREATITIS IN CHILDREN
Deepak Aamarapurkar
Senior Consultant Cardiologist, Kovai Heart Centre, Coimbatore.
Pancreatic diseases in children are acute Pancreatitis, congenital anomalies that may involve pancreas, diseases causing chronic pancreatic insufficiency and chronic pancreatitis. Common causes of acute pancreatitis in children are trauma, viral infections (mumps, viral hepatitis), ascariasis, pancreas divisum, choledochal cysts, hyperlipidaemia and hereditary pancreatitis. [1-3] Chronic pancreatic insufficiency without pancreatitis is seen in children suffering from cystic fibrosis, Shwachman syndrome, congenital enzyme deficiencies, enterokinase and lipase, Co-lipase and trypsin. [1] Chronic pancreatitis in children can be due to hereditary pancreatitis; idiopathic chronic pancreatitis, tropical chronic pancreatitis hypertriglyceridaemia, hyperparathyroidism. [1] , [3-5] All these types of pancreatitis can present with repeated acute attacks and progress to chronic calcific pancreatitis.
HEREDITARY PANCREATITIS
Comfort and Steinberg [6] gave the first description of hereditary pancreatitis in 1952. Since then a number of reports and hereditary pancreatitis have been reported in various countries including India. [3] , [5] In hereditary pancreatitis inheritance pattern was strongly suggesting of autosomal dominant trait. The classical description of clinical picture includes recurrent episodes of pancreatitis since childhood equals sex distribution, family history of at least two other affected members, calcific pancreatitis absence of known precipitating factors. [7] Recent developments in genetics have shown that hereditary pancreatitis usually associated with two specific mutations in the cationic trypsinogen. [8] These two mutations in the cationic trypsinogen gene appeared to allow prematurely activated trypsinogen to cause acinar cell auto digestion and acute pancreatitis. Chronic pancreatitis in these patients certainly arises from recurrent acute pancreatitis.
Clinical features of hereditary pancreatitis include recurrent episodes of acute pancreatitis in 80% of the family members and eventual chronic pancreatitis in about 20 to 30% family members. [7] , [8] Patients with hereditary pancreatitis usually begin with recurrent episodes of acute pancreatitis in the childhood. The attacks of acute pancreatitis are similar to other cause of pancreatitis in the childhood. The attacks of acute pancreatitis are similar to other cause of pancreatitis in severity and complications. The frequency of attacks is quite variable. Severe attacks may occur 3 to 4 times a year, followed by year without attacks. Hereditary pancreatitis can lead to chronic pancreatitis with all of the typical complications including fibrosis, dilated ducts, pseudocyst calcification, diabetes, and malabsorption. There is fifty-fold rise in risk of pancreatic cancer in patients with hereditary pancreatitis when compared with general population. [8]
Management of acute pancreatitis is similar to other patients with acute pancreatitis. In chronic pancreatitis, pancreatic enzymes supplements, antioxidants have been used to reduce the frequency and severity of the attacks. [9] Surgical intervention for pain relief and complications like pseudocyst, ascites and obstruction has been used in similar ways as in patients other causes of chronic pancreatitis. Immediate and long-term results from the surgical procedures have shown encouraging results and have confirmed the beneficial effect of surgery in well selected cases with a goal of avoiding total pancreatectomy. [10] Current knowledge about the biochemistry and molecular biology have shown a ray of hope to gene therapy. Those several features of the disease make gene therapy unlikely in near future. Early detection of disease may lead to the early treatment with effective measures.
Tropical Calcific Pancreatitis
Tropical calcific pancreatitis though initially described from southern India; is seen in all parts of our country. [4] , [5] This type of pancreatitis is seen exclusively in tropics. It is also characeritized by recurrent episodes of abdominal pain, protracted malnutrition, ketosis resistant diabetes. Ten per cent patients with tropical calcific pancreatitis develop pancreatic cancer. Almost one third of patients with tropical calcific pancreatitis get recurrent episodes of pain in childhood can develop diabetes in childhood. Dense calcification is seen in pancreatic parenchyma. Though exact aetiology of tropical chronic pancreatitis is known, malnutrition with protein deficiency, cassava toxicity, impaired immune response, viral infection and genetic susceptibility have been considered as various factors in the aetiopathogenesis. [4] , [5] As is with other forms of chronic pancreatitis treatment of chronic calcific pancreatitis includes control of diabetes, relief of pain with analgesics, pancreatic enzyme replacements, endoscopic or surgical decompression of dilated ducts and removal of pancreatic calculi. [5]
IDIOPATHIC RECURRENT PANCREATITIS
Symptoms of abdominal pain, back pain vomiting are similar to those of acute episodes in hereditary pancreatitis, complications like pancreatic ascites pseudocyst, portal vein thrombosis are uncommon as compared to hereditary pancreatitis. [9] Calcification diabetes and steatorrhoea is remarkably less as compared to tropical pancreatitis.
Hypertriglyceridaemia and hyper parathyroidism though rare reported to have chronic pancreatitis in children. [1]
In our experience of 20 cases of chronic pancreatitis in children, four patients had hereditary pancreatitis, six had idiopathic, eight had tropical pancreatitis while hypertriglyceridaemia and hyperparathyroidism in one case each. Unusual case of chronic pancreatitis was eight years old child presenting with progressive distension of abdomen without pain. This patient had haemorrhagic ascites with markedly elevated ascitic fluid amylase. [1] His ERCP (Fig. 1) showed markedly dimg%, chylomicrons present, apolipoprotein A was 1.84 mg% while Apolipoprotein B 0.76 mg%, CT scan of abdomen showed mildly dilated pancreatic duct with few speckled calcification. Patient was started on lipid lowering agent and remarkable improvement in the symptoms. A nine year old boy presented with recurrent abdominal pain, diagnosed to have calcific pancreatitis. His serum calcium was found to be 12 mg%. Serum parathormone was markedly elevated. Sonography of neck showed parathyroid adenoma at left lowerpole of thyroid. Parathyroid adenoma was resected with improvement in the symptoms.
Fig 1: ERCP showing dilated pancreatic duct with pseudocyst rupture. Normal biliary system. In child of eight years with chronic pancreatitis.
Chronic pancreatitis is common in children, but when chronic pancreatitis is diagnosed efforts should be made to identify treatable causes like hypertriglyceridaemia, hyper parathyroidism, pancreas divisum and other congenital anomaly. Hereditary pancreatitis can be suspected with careful family history and genetic diagnosis of hereditary pancreatitis should be routine soon and will identify the patients at early stage.
REFERENCES
1.Roberts IM. Disorders of the pancreas in children. Gastroenterol Clinic 1990; 963-73.
2.Amarapurkar DN, Begani MM, Mirchandani K. Acute pancreatitis in hepatitis A infection. Tropical Gastroenterol 1996; 17 : 30-4.
3.Shah S, Amarapurkar D, Pitchumoni CS. Hereditary pancreatitis. Am J Gastroenterol 1994; 928-30.
4.Tandon RK. Tropical Calcific pancreatitis. J Gastroenterol and Hepatol 1998; 13 (Suppl) : S284-88.
5.Balkrishnan V. Chronic calcific pancreatitis in the tropics. Ind J Gastroenterol 1984; 3 : 65-7.
6.Comfort MU, Steinberg AG. Pedigree of family with hereditary chronic relapsing pancreatitis. Gastroenterology 1952; 21 : 54-63.
7.Perrault J, Burtholomew LG. Hereditary and familial pancreatitis in Gastroenterology Berk E Eds Philadelphia WB Saunders. 1985; 4050-4.
8.Whitcomb DC. The spectrum of complication of hereditary pancreatitis; is this model for future gene therapy. Gastroenterol Clinic 1999; 28 : 525-41.
9.Konzen KM, Perraulf J, Moir C, et al. Long-term followup of young patients with chronic hereditary or idiopathic pancreatitis. Mayo Clin Proc 1993; 68 : 450-57.
10.Perrault J. Hereditary Pancreatitis. Gastroentero Clin 1994; 23 : 743-52.
11.Dhawan P, Amarapurkar D, Bhandarkar P, Kalro RH. Pancreatic ascites. J Assoc Phys India 1993; 41 : 582-83.
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