Chronic calcific pancreatitis (C.C.P.) in western Maharashtra (experience of 55 cases).
Chronic calcifying/calcific pancreatitis (CCP) is a special form of chronic pancreatitis that tends to calcify or is associated with pancreatic lithiasis. Pancreatic lithiasis refers either to true stones in the duct system of the pancreas called pancreatic calculi or to false stones due to calcification of the parenchyma. The term tropical calcific pancreatitis (TCP) refers to the disease which occurs in children and young adults in tropics and has features that distinguish it from alcoholic calcific pancreatitis (ACP) seen in developed countries of the subtropics.
In India TCP has been mainly reported from Kerala, and Madras. This paper describes our experience with 55 cases with chronic calcific pancreatitis collected over 14 years chiefly from Western Maharashtra and adjoining areas.
This is a retrospective analysis of the case records of 55 (43 males and 12 females) patients with chronic calcific pancreatitis collected over 14 years (1973-1986) from the endocrinology (diabetic clinic), surgery and gastroenterology departments of KEM Hospital, Bombay. The basic diagnosis was made on demonstration of calculi/calcification in the region of pancreas on plain X-ray abdomen in 54 cases and on endoscopic retrograde pancreatogram (ERP) in 1 case.
Available clinical, biochemical and radiological features of each case were noted. Twenty three patients were followed up in the outpatient departments for periods between 1 to 13 years.
Age and sex
The median age of our patients was 33 years (range 7 to 60 years). The peak age of presentation amongst males (31-35 years) was higher than that of females (16 to 20 years).
The alcoholic patients (median 35 years, range 30 to 63) were over a decade older than non-alcoholic patients (median 22.5 years, range, 7 to 63 years). There was a preponderance of males with a male: female ratio of about 4:1.
Clinical features [Table 1]
Presenting symptoms: Of the 55 patients, 38 presented with symptoms of diabetes mellitus viz. polyuria, polydypsia and remaining 17 patients present with abdominal pain. Fifteen of the 38 diabetics also had abdominal pain and 3 of the 17 presenting with abdominal pain also had diabetes mellitus on investigations. The median duration of onset of presenting symptoms was 1 year (range 1 week to 15 years). Weight loss was present in all 55 cases.
Abdominal pain: Twenty eight of 32 patients with abdominal pain had pain typical of pancreatitis viz. epigastric pain radiating to back associated with a characteristic forward posture. The remaining four had non radiating epigastric pain.
Diabetes mellitus: Of the 41 patients with diabetes mellitus, 37 were insulin dependant and in 4 the diabetes mellitus was controlled by diet and chlorpropamide.
Alcoholism: Nineteen of our 55 patients had a history of significant alcohol intake i.e. steady drinking of moderate to large (>50 g of absolute alcohol/day) amounts for at least 5 years.
Steatorrhoea: Eight of 23 patients had steatorrhoea ranging from 6.3 to 17.0 g/day.
Family history: Of the 12 patients in whom detailed enquiries were made, a family history of pancreatitis or DM was not present in any.
Ethnic and geographical features
Forty six patients were Hindus, 8 Christians and 1 Muslim. Most patients came from Western Maharashtra and Goa (49) and the remaining from other regions-Kerala (2), Gujarat (2), Karnataka (1) and Tamil Nadu (1).
The nutritional status was analysed on the basis of available details of height and weight. Of the 34 patients with such data, 33 were below 50th percentile of normal.
Associated diseases and complications [Table 2]
Almost all complications were probably related to diabetes mellitus.
Lundh meal test was performed in 7 patients without steatorrhoea which revealed pancreatic insufficiency in all 7. Abdominal ultrasonogram revealed pancreatic calcification in 8 patients studied. ERPs were done in 7 patients, which showed typical duct dilatation with calcification in all of them; one of these 7 patients did not have calcification on plain X-ray of adbomen. A computerised tomogram of abdomen obtained in one patient revealed calcification and ductal irregularity.
Seven of our patients with severe abdominal pain were treated with pancreatic enzymes (3.6 to 4.8 g/day of pancreatin). A good response was observed in 6 as demonstrated by significant decrease in severity and frequency of pain. The 41 diabetics were controlled with insulin (37 patients) and diet and oral anti-diabetic (4 patients).
The indication for surgery in all 14 patients, who underwent surgery was relentless pain. Puestow's pancreato-jejunostomy was done in 6, partial pancreatectomy with lateral pancreatico-jejunostomy in 5, cystoduodenostomy in 1 and 2 patients were operated elsewhere, the nature of which not known. Of 11 patients who survived following surgery, pain was relieved in 9 patients.
A follow-up of 1 to 13 years is available in 23 cases who are alive and well. Four of our patients died-3 in post-operative period and 1 due to unrelated cause.
Our analysis shows that CCP occurs in our area not infrequently and the proportion of alcoholic and non-alcoholic cases is 1 : 2. It occurs predominantly in young adult males. The main presenting symptoms are diabetes mellitus and abdominal pain.
The median age of 33 years in our patients is similar to a mean age of 38.4 years at the time of first painful crisis among 93 French patients. In contrast, in Kerala patients the average age of onset was 12.5 years. The alcoholic patients in our series were over a decade older than non-alcoholic patients (median 22.5 years). It is obvious therefore that even if the alcoholic patients are excluded from our series, our patients are older than Kerala patients at the time of presentation by at least one decade.
Abdominal pain was present in about three fifths (32/55) of our patients, while in French series it was present in 93%. The type and character of pain experienced by our patients was similar to that of French series.
In our series about one third (19/55) patients had a history of significant alcohol intake, in comparison to 90% of French patients. Contrary to this, 95% of Kerala patients had no history of alcohol intake. Alcohol intake in a population has been shown to correlate well with the frequency of CCP in certain other western countries,,, while it is less common in those countries where alcohol consumption is comparatively low as in Great Britain, West Germany, and Northern part of India.
Overt diabetes mellitus was present in approximately four fifths (41/55) of our patients, majority of these being from diabetic clinic. Of 1700 patients in the Kerala series 90% were overtly diabetic, while in the French series, 23% of 100 patients had overt diabetes and 24% had an abnormal glucose tolerance. Diabetes mellitus is not as common in our patients as in Kerala, but it is more common than in France (23%), Howard and Jordan's series (34%) and South Africa (45%). The presence of manifest diabetes at the time of diagnosis, therefore, appears to be linked to the age of onset of pancreatitis, earlier the onset, more is the frequency of diabetes.
Fat balance studies revealed steatorrhoea (6.3 to 17.0 g/day) in one third of patients (8 of 23) studied, which is comparable to frequency of steatorrhoea in French patients (17/48) and in Dublin series (32%). However, degree of steatorrhoea in some French patients was much higher (upto 50 g/day). In contrast, patients from Kerala had a much lower incidence (5-10%) of steatorrhoea. Notably, only two of eight had symptoms of diarrhoea suggesting that chronic subclinical steatorrhoea is common and may contribute to these patients' poor nutrition. An abnormal lundh meal test indicated pancreatic insufficiency in all 7 patients who were subjected to this test, despite absence of steatorrhoea. Secretin/pancreozymin test was positive in 95% (38/40) of Dublin series patients. Almost all (33/34) our patients were significantly undernourished, which is comparable to constant finding of weight loss in 100% of French and Dublin series patients.
In our series, family history of pancreatitis/diabetes mellitus was not present in any of the 12 patients, while it was 8% in Kerala patients. The precise nature of inheritance of this disease is not clear and yet genetic basis for the disease cannot be accepted without reservation. Environmental factors such as diet, infection etc. may still be important. Our analysis of small number of cases indicates lack of genetic basis for CCP.
Pulmonary tuberculosis was present in about one fifth (9/55) of patients, all of whom were diabetic. Presence of other conditions were not directly related to pancreatitis. This may be related to poor nutrition and immuno-suppression due to diabetes. Most of the complications in our patients viz. nephropathy, neuropathy and keto-acidosis were related to DM.
Abdominal ultrasonogram, ERP and CT scan abdomen also demonstrated pancreatic calculi, which were visualised on plain X-ray abdomen except in one patient in whom plain X-ray abdomen did not show calcification but ERP showed calculi. ERPs were not obtained in all patients prior to surgery as facilities were not available about 3 years ago. The indication for surgery in our patients was relentless pain.
In conclusion, CCP in our area appears to occupy an intermediate position between the TCP of Kerala and ACP of the West with reference to the age of onset (young adults), mixed alcoholic and non-alcoholic etiology and the intermediate frequency of diabetes and steatorrhoea.