|Year : 2019 | Volume
| Issue : 3 | Page : 140-142
Fibrocalculous pancreatic diabetes in a young female: A case report from South Gujarat
Vineeta Singh, Mohit Singh Tandon
Department of General Medicine, Shrimad Rajchandra Hospital, Dharampur, Gujarat, India
|Date of Web Publication||27-Aug-2019|
Dr. Vineeta Singh
Department of General Medicine, Shrimad Rajchandra Hospital, Valsad, Dharampur, Gujarat - 396 050.
Source of Support: None, Conflict of Interest: None
Fibrocalculous pancreatic diabetes (FCPD) is a rare form of diabetes secondary to chronic calcific pancreatitis. The patients generally have clinical triad of pain abdomen, steatorrhoea and diabetes. The peculiar features are presence in a tropical country, younger age of onset, presence of pancreatic calculi, ketosis resistance and chances of progression to malignancy. We present a case of a young female presenting with recurrent abdominal pain, diabetes and chronic calcific pancreatitis. She was diagnosed to have FCPD based on the clinical, biochemical and radiological findings. She was managed with insulin, pancreatic enzyme supplementation and analgesics for pain relief. This is the first case reported from south Gujarat as per our knowledge. FCPD is quite rare. This report signifies the importance of keeping high index of suspicion in patients presenting with high sugars and abdominal pain. Early management with sugar control, pain relief and supplementing enzymes may prevent progression to complications.
Keywords: Fibrocalculous pancreatic diabetes, south Gujarat, young female
|How to cite this article:|
Singh V, Tandon MS. Fibrocalculous pancreatic diabetes in a young female: A case report from South Gujarat. J Diabetol 2019;10:140-2
| Introduction|| |
Fibrocalculous pancreatic diabetes (FCPD) is a rare form of secondary diabetes which occurs due to chronic calcific pancreatitis in the absence of alcohol abuse and is mainly restricted to the tropical regions of the world. There occurs exocrine and endocrine pancreatic failure due to the chronic pancreatic inflammation and calcification associated with dilatation of the pancreatic duct with intraductal calculi. In India, most cases are reported from southern and eastern parts of India such as Kerala, Orissa and Chennai. Only one study on the prevalence of tropical chronic pancreatitis has been reported by Balaji where a population of 28,507 in Kerala was studied and 1 in 1020 participants had chronic calcific pancreatitis (0.09%). The first case was reported in 1937 from India by Kini and only one case has been reported from Vadodara, Gujarat, as per our knowledge. Here, we report the first case of FCPD from south Gujarat diagnosed based on the clinical, biochemical and radiological findings.
| Case Report|| |
A 17-year-old female admitted to the hospital with recurrent pain abdomen since 6 years and is aggravated for 1 day. She described a moderate-to-severe intensity pain, located in the epigastric region and radiating to the back. The pain used to get aggravated by meals and lying down position and relieved by stooping forward. She also gave a history of polyuria, polydipsia and polyphagia for the past 5 months and was diagnosed to have diabetes 5 months back. She was treated with oral medicines, but her sugars were not controlled by this, and hence, she came with high sugars and severe pain abdomen. There was no history of diabetes in the parents. The history of diabetes in any other family members could not be obtained. There was no history of consumption of alcohol and tobacco. She consumes a non-vegetarian diet and belongs to low socioeconomic status.
On examination, she was afebrile, conscious alert, responsive to commands and pale but no icterus, cyanosis, no clubbing, lymphadenopathy or parotid gland enlargement. Her blood pressure was 110/70 mmHg, pulse 78/min, respiratory rate was 18/min and SpO2 was 100%. Her body mass index was 17.72kg/m2. On systemic examination, her neurological, cardiorespiratory and abdomen examination were normal.
She had high sugars 593mg/dl. Her urine analysis showed glycosuria with negative urine ketones. Her complete blood count revealed haemoglobin 13.7 gm/dl, total leucocyte count 10400/cumm, platelet count 261000/cumm, haemoglobin A1c (HbA1c) 13.3%, and serum protein and lipid profile were normal. Serum calcium was 9.3mg/dl. Her kidney functions and liver functions were normal. Her C-peptide level was 0.57ng/ml (0.9–7.1 normal range). Serum amylase was 215 IU/L and serum lipase was 112 IU/L.
Stools examination revealed no fat globules, ova and cyst. Electrocardiography, chest X-ray and fundus examination were normal. Her X-ray abdomen was also normal. Ultrasonography (USG) abdomen showed chronic pancreatitis with the presence of pancreatic calculi [Figure 1]. Computed tomography (CT) abdomen showed multiple calcifications scattered throughout the body, head and tail with dilated tortuous main pancreatic duct in the head, body and tail region with intraductal calculi in the head of the pancreas [Figure 2]. Hence, on the basis of clinical features and investigations, a diagnosis of FCPD was made. The patient was given fixed-dose combination insulin of 35 units in the morning before breakfast and 24 units before dinner. On the 8th day of her admission, her fasting was 130mg/dl and post-prandial (PP) was 200mg/dl. She was also given pancreatic enzyme supplementation for malabsorption together with calcium supplementation. She was counselled about symptoms of both hypo and hyperglycaemia and also was given dietary advice on discharge. However, following this admission, the patient stopped insulin on her own and also lost to follow-up for a prolonged period. During her recent visit, her blood sugars were uncontrolled due to poor compliance with fasting blood sugar 220mg/dl, PP blood sugar 340mg/dl and HbA1C 11%. At present, she is advised to be compliant on insulin and keep a regular follow-up for dose optimisation.
|Figure 1: Ultrasound abdomen showing chronic pancreatitis with pancreatic calculi and dilated main pancreatic duct|
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|Figure 2: Computed tomography abdomen showing multiple calcifications scattered throughout the body, head and tail with dilated tortuous main pancreatic duct with intraductal calculi in the head of pancreas|
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| Discussion|| |
FCPD is a rare form of diabetes secondary to pancreatic calcification in non-alcoholics, mostly seen in young population between the age of 10 and 40 with male preponderance though here the patient is 17-year young female. The factors which are responsible for the pathogenesis of FCPD are genetic association, malnutrition, toxic effects of cyanide due to frequent cassava consumption and increased oxidant stress from Vitamin C and A deficiencies. However, the cassava hypothesis has been discarded because cassava consumption was not found to be a risk factor in the case–control studies.
Recently, a link between the serine protease inhibitor, Kazal type 1 gene and tropical calcific pancreatitis is confirmed.
Abdominal pain, steatorrhoea and diabetes are the three important features of FCPD. Abdominal pain is the most predominant symptom which is severe in nature and epigastric in location and radiating. There are intermittent episodes of remissions and exacerbations. About one-third of the patient complaints of passing large bulky and oily stools while stool examination in our patient showed the absence of fat globules. The absence of steatorrhoea was attributed to the low fat and high carbohydrate diet. Pancreatic calculi are commonly seen on abdominal X-ray. USG and CT scan aid in localising pancreatic calculi and delineating other features such as atrophy, fibrosis, ductal dilatation and finally, ‘Bag of stones’ appearance in extreme cases.
Although high sugars, ketosis is absent possibly due to the residual beta-cell function, reflected by the intermediate levels of C-peptide. Majority of the patients require insulin for hyperglycaemia control.
In patients who have negligible C-peptide, it was proposed by Yajnik that the mechanisms other than beta-cell function are also involved. The key factors are as follows:
- Residual B-cell function adequate to prevent ketosis
- Concomitant destruction of A-cells and thus loss of glucagon, a major ketogenic hormone
- Subcutaneous fat loss and therefore reduced supply of non-esterified fatty acids (NEFAs) – the ‘fuel’ for ketogenesis
- The resistance of subcutaneous adipose tissue lipolysis to adrenaline
- Carnitine deficiency affecting the transfer of NEFA across the mitochondrial membrane.
Diagnostic criteria for FCPD:
- Occurrence in a ‘tropical’ country
- Diabetes by the WHO study group criteria
- Evidence of chronic pancreatitis: pancreatic calculi on X-ray or at least three of the following:
- a. Abnormal pancreatic morphology by USG
- b. Chronic abdominal pain since childhood
- c. Steatorrhoea
- d. Abnormal pancreatic function test.
- The absence of other causes of chronic pancreatitis i.e., alcoholism, hepatobiliary disease or primary hyperparathyroidism, etc.
In our patient, recurrent episodes of abdominal pain, diabetes with absent ketosis and evidence of pancreatic calculi on USG and abdominal CT helped us making a diagnosis of FCPD.
These patients may develop microvascular complications such as retinopathy, neuropathy and nephropathy while macrovascular complications are less common possible due to young age, their lean body mass and the low cholesterol levels.
However, a case of the severe peripheral vascular disease with gangrene was reported in the absence of other risk factors of atherosclerotic vascular disease, suggesting complete foot examination of such patients at least once a year. A study of 30 FCPD patients demonstrated the prevalence of cardiac autonomic neuropathy as 63.3%, thus warranting frequent screening for autonomic dysfunction. Although being rare, a high index of suspicion is required while managing diabetes. Adequate glycaemic control, pancreatic enzyme for malabsorption and nutrient supplementation is the mainstay of treatment to prevent complications. Misdiagnosed cases or delayed diagnosis may result in fatal complications like pancreatic carcinoma and hence periodic screening is also recommended.
Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the editor-in-chief of this journal.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understand that names and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]