N Kurtulmus, S Yarman, R Tanakol, F Alagol
Division of Endocrinology, Metabolism and Nutrition, Departmant of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University, Capa, Istanbul, Turkey
Correspondence to: Neslihan Kurtulmus, Cevahir Koru Sitesi A Blok D : 21, 34010, Merter , Istanbul – Turkey Tel : 0 90 212 482 82 38 Fax : 0 90 212 311 23 67 Email : neslihandr@hotmail.com
SMJ 2005 50(4): 172-173
Abstract
We report a 30-year-old woman who was confined to a wheelchair because of severe myopathy. She was first seen by a neurologist because of a convulsive syndrome of unknown etiology when she was nine. She was started on anticonvulsive drugs but the drug was stopped when her serum calcium level was found to be very low. She had a history from childhood of steatorrhea and abdominal pain after a fatty meal and became vegetarian at age five years. She worked in a hospital as a nurse and at home her living room received no direct sunlight. As a result of these conditions osteomalacia progressed. We believe an awareness of chronic pancreatitis (CP) during childhood could have prevented the consequences of the disease in this case.
Key words : Chronic pancreatitis complications , osteomalacia , malabsorbtion syndromes
Introduction
Most reports about vitamin D deficiency in adults cite restricted exposure to sunlight as a cause.1,2 However, strict vegeterians, dark skinned individuals, patients with gastrointestinal and chronic liver diseases, alcoholics and those taking anticonvulsive drugs are also prone to osteomalacia.1,3,4 Among patients with gastrointestinal disease, vitamin D deficiency was significantly more common than chronic pancreatitis following gastroenterostomy.
Major predisposing risk factors to chronic pancreatitis may be categorised as either toxic-metabolic, idiopathic, genetic, autoimmune, recurrent and severe acute or obstructive pancreatitis. Chronic pancreatitis is typically characterised by clinical, (steatorrhea, abdominal pain, loss of body weight) morphological, (calcifications, dilated ductus pancreaticus) and functional (malabsorbtion, diabetes mellitus) parameters. Approximately 30% of patients with chronic pancreatitis have no identifiable cause for their disease.3,5 In this study, we report a case of severe osteomalacia in patient with idiopathic chronic pancreatitis.
Case report
A 30-year-old Turkish woman presented to our department with fatigue, marked pains in the wrist and leg and an inability to ambulate. As a result of this she was confined to a wheelchair. There was no family history of any chronic or pancreatic diseases. She had a history since childhood of steatorrhea and abdominal pain which began after consuming a fatty meal or dairy produce and for this reason she became vegetarian from age five years. At age nine years she was seen by a neurologist because of a convulsive syndrome of unknown etiology. She was started on an anticonvulsive drug but when her serum calcium level was found to be very low the drug was stopped. No further convulsions were reported.
She worked in a hospital as a nurse and at home in day time her living room had no direct sunlight. For this reason she was taking vitamin D and oral calcium but only during winter time. Her symptoms worsened. When admitted to our clinic physical examination of her locomotor system was not possible because of marked pain and weakness in her trunk and wrist combined with bone tenderness. She was under-weight, her body mass index was eighteen. On routine analysis, serum levels of calcium (1.57mmol/L, corrected calcium:1.77mmol/L, normal(nl): 2.2-2.6mmol/L), phosphate (0.83mmol/L, nl=1.0-1.4mmol/L), albumin (31g/L,nl:35-55g/L) and 25(OH) vitamin D (17.47nmol/L, nl :24.96-99.84nmol/L,) were low, while serum alkaline phosphatase (6.27µKat/L, nl:1.53–4.42µKat/L) and intact PTH(565ng/L, nl:10- 60ng/L) levels were high. Her fasting glucose level and oral glucose tolerance test were normal. Serum amylase level (0.74 µKat/L, nl:0.42- 3.4µKat/L)was normal. Serum iron, vitamin B12 and vitamin A levels were also low. Antibodies to gliadin and endomysium were negative. Urinary of calcium and phosphore were low (0.995mmol/d, nl: 2.5- 7.5mmol/d; 6.458mmol/d, nl:16.14–48.43mmol/d,respectively). Sweat test was normal. Fecal elastase concentration was significantly decreased in this patient. ERCP showed abnormal duct changes( irregularity, stricture and dilatation) in the main pancreatic duct,but the spincter of oddy was normal.
On x-ray it was noted that she had a milkman fracture(Fig.1)and pancreatic calcification.(Fig. 2) During hospitalisation, she reported severe abdominal pain with steatorrhea after eating a fatty meal(>40g fat). A 24hr stool specimen contained 38g fat (nl:below 6g/24hr). No specific cause for the chronic pancreatitis was found and a diagnosis of chronic pancreatitis was made from her history and clinical, (abdominal pain, steatorrhea)morphological,(pancreatic calcifications, irregular ductus pancreaticus) and functional (maldigestion) findings. Pancreatic enzymes preparates that containing 25000 units of lipase (enteric–coated microencapsulated preparation)was given per meal. In additional, supplementation with vitamin D (1mcg of calcitriol daily) and calcium carbonate (1.5gr elemental calcium daily) were started. Over the next 4- 6 weeks there was an improvement in muscle pain and muscle strength and in eight weeks she had become fully mobile. Physical examination of her locomotor system was normal and her muscle strength(5/5) was perfect. Serum levels of calcium(2.25mmol/L), phosphate(1.03mmol/ L), albumin (41g/L), 25(OH) vitamin D (69.88nmol/L), alkaline phosphatase (2.46µKat/L) and intact PTH (44ng/L) were normal after drug therapy.
Discussion
Chronic pancreatitis is a slowly progressive disease. The diagnosis of chronic pancreatitis is difficult in the early stages of the disease. It is based on clinical history and functional and morphologic parameters. Pancreatic function tests, imaging procedures are essential in patients with suggestive clinical history for CP.6 Fecal elastase test is a simple and accurate functional test for CP, although sometimes its value for diagnosis of chronic pancreatitis is limited.6,7 Patients with idiopathic pancreatitis may have genetic disorders like mutations of cationic trypsinogen gene(PRSS 1) or pancreatic secretory trypsin gene(SPINK1).8 Gene mutations of the cystic fibrosis transmembrane conductance regulator(CFTR), cationic trypsinogen, and pancreatic secretory trypsin inhibitor may investigate in idiopathic chronic pancreatitis if the patient developed pancreatic insufficiency in childhood.9 There is genetic heterogeneity in patients with hereditary and idiopathic chronic pancreatitis.10,11 Impaired glucose tolerance and insülin-dependent diabetes are common findings in chronic pancreatitis.12 If exocrine pancreatic insufficiency is mild in chronic pancreatitis, glucose tolerance may not impaired.13 In our patient, fasting glucose level and glucose tolerance test were normal.
Osteomalacia may occur as a complication of steatorrhea secondary to chronic pancreatitis. Pancreatic insufficiency induces malabsorption of fat and fat-soluble vitamins including vitamins A and D and as a result such patients usually prefer a vegeterian diet because of gastrointestinal complaints.1,14,15 In this case,malabsorption due to pancreatic insufficiency was the main cause of vitamin D deficiency. Nutritional deficiency and inadequate sunlight exposure were additional causes of vitamin D deficiency. Nutrition has an important role in the management of chronic pancreatitis and the first aim is to provide optimal nutrition support.16 Pancreatic enzymes and antioxidants are important for treatment of chronic pancreatitis.14,16,17 The measurements of serum 25(OH) vitamin, parathyroid hormone, serum alkaline phosphatase, and urinary calcium levels should be checked annually in these patients .
In conclusion, an awereness of chronic pancreatitis during childhood could have prevented all the consequences of this uncommon disease.
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