Rickets in adult cystic fibrosis with myopathy, pancreatic insufficiency and proximal renal tubular dysfunction

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      Rickets is reported in a 19 year old white man with cystic fibrosis in whom pancreatic and hepatic involvement was advanced. There was evidence of secondary hyperparathyroidism with proximal renal tubular acidosis, aminoaciduria, phosphaturia and hypophosphatemia. Treatment with oral pancreatic and parenteral vitamin D supplements led to full recovery of the rachitic syndrome and the proximal renal tubular dysfunction.
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        • Crozier DN
        Cystic fibrosis. A not-so-fatal disease.
        Pediatr Clin North Am. 1974; 21: 935
        • Lapey A
        • Kattwinkel J
        • Di Sant'Agnese PA
        • et al.
        Steatorrhoea and azotorrhoea and their relation to growth and nutrition in adolescents and young adults with cystic fibrosis.
        J Pediatr. 1974; 84: 328
        • Di Sant'Agnese PA
        • Andersen DH
        Cystic fibrosis of the pancreas in young adults.
        Ann Intern Med. 1959; 50: 1321
        • Coates EO
        Characteristics of cystic fibrosis in adults. A report of seven patients.
        Dis Chest. 1966; 49: 195
        • Hunton DB
        • Long WK
        • Tsnmagari HY
        Meconium ileus equivalent. An adult complication of fibrocystic disease.
        Gastroenterology. 1966; 50: 99
        • Tyson KRT
        • Schuster SR
        • Shwachman H
        Portal hypertension in cystic fibrosis.
        J Pediatr Surg. 1968; 3: 271
        • Harris JT
        • Muller DPR
        Absorption of different doses of fat soluble and water miscible preparations of vitamin E in children with cystic fibrosis.
        Arch Dis Child. 1971; 46: 341
        • Underwood BA
        • Denning CR
        Blood and liver concentrations of vitamins A and E in children with cystic fibrosis of the pancreas.
        Pediatr Res. 1972; 6: 26
        • James O
        The Lundh test. Progress report.
        Gut. 1973; 14: 582
        • Sproul A
        • Huang N
        Growth pattern in children with cystic fibrosis.
        J Pediatr. 1964; 65: 664
        • Simopolulos AD
        • Pak CYC
        • Kattwinkel J
        Calcium metabolism and parathyroid function in cystic fibrosis.
        in: Cystic Fibrosis Club Conference, Atlantic City, New JerseyApril 23, 1971: 13
        • Oppenheimer EH
        Focal necrosis of striated muscle in an infant with cystic fibrosis and evidence of lack of absorption of fat-soluble vitamins.
        Bull Johns Hopkins Hosp. 1956; 98: 353
        • Siwe SA
        Uber die exokrine function des pankreas and die folgen ihres wagfalles.
        Deutsches Arch Klin Med. 1932; 173: 339
        • Shwachman H
        • Dooley RR
        • Guilmetts F
        • et al.
        Cystic fibrosis of the pancreas with varying degrees of pancreatic insufficiency.
        Am J Dis Child. 1956; 92: 347
        • Clark ML
        • Harris JT
        Abosrption of Lipids.
        (chap 5)in: McCholl I Sladen GEG Intestinal Absorption in Man. Academic Press, New York1975: 209
        • Weber AM
        • Roy CC
        • Chartrand L
        • et al.
        Relationship between bile acid malabsorption and pancreatic insufficiency in cystic fibrosis.
        Gut. 1976; 17: 295
        • Stern RC
        • Stevens DP
        • Boat TF
        • et al.
        Symptomatic hepatic disease in cystic fibrosis: incidence, course, and outcome of portal systemic shunting.
        Gastroenterology. 1976; 70: 645
        • Muldowney FP
        • Freany R
        • McGeeney D
        Renal tubular acidosis and aminoaciduria in osteomalacia of dietary or intestinal origin.
        Q J Med. 1968; 37: 517
        • Muldowney FP
        • Carrol DV
        • Donohoe JF
        • et al.
        Correction of renal bicarbonate wastage.
        Q J Med. 1971; 40: 487
        • Schott GD
        • Wills MR
        Muscle weakness in osteomalacia.
        Lancet. 1976; 1: 626
        • Patten BM
        • Bilezikian JP
        • Mallate LE
        • et al.
        Neuromuscular disease in primary hyperparathyroidism.
        Ann Intern Med. 1974; 80: 182
        • Sung JH
        Neuroaxonal dystrophy in mucoviscidosis.
        J Neuropathol Exp Neurol. 1963; 23: 567
        • Malamud N
        • Nelson MM
        • Evans HM
        Effects of chronic vitamin E deficiency on the nervous system in rats.
        Ann NY Acad Sci. 1949; 52: 135