Dr. Alfred E. Slonim M.D
Clinical Professor of Pediatrics
Division of Molecular Biology
Columbia University Medical College
Attending physician
Clinical Genetics
New York Presbyterian Hospital
Regulation of Metabolic fuels in diseased states
My primary research interest is the investigation of the disturbances in metabolic fuels that takes place in various disease states. Specifically, this interest encompasses the disturbances in glucose, lipid and protein/amino acid fuels brought about by genetically inherited enzyme deficiencies. We have studied the metabolic disturbances brought about by these enzyme deficiencies and have devised therapies that modify the severity of these disturbances. These therapies consist of nutritional, exercise and hormonal means of compensating for these fuel disturbances. This method has led to compensatory therapies for a number of these enzyme deficiency diseases specifically involving the liver, muscle and gastrointestinal tract, and more recently the inflammatory bowel disease group of diseases. This form of therapy has led to a dramatic improvement in the natural history of a number of these diseases.
Representative Publications:
Greene, H.L., Slonim, A.E., Burr, I.M., O’Neill, J.A.: Nocturnal intragastric feeding for type I glycogen storage disease. N. Engl. J. Med. 1976, 294: 423-425.
Slonim, A.E., Terry, A.B., Greene, H.L., Lacy, W.W., Burr, I.M.: Nocturnal intragastric therapy in type I glycogen storage disease. Effect on hormonal and amino acid metabolism. Metabolism. 1979, 28: 707-715.
Slonim, A.E., Weisberg, C., Benke, P., Evans, O.B., Burr, I.M.: Reversal of debrancher deficiency myopathy by the use of high protein nutrition. Annals. Neurol., 1982, 11:420-422.
Parker, PH., Slonim, A.E., Burr, I.M., Greene, H.L.: Regression of hepatic adenomas in Type I glycogen storage disease with dietary therapy. Gastroenterology, 1981, 81:534-536.
Slonim, A.E., Coleman, R.A., McElligot, M.A., Najjar, J., Hirschhorn, K., Labadie, G.U., Mrak, R., Evans, O.B., Shipp, E., Presson, R.: Improvement of muscle function in acid maltase deficiency by high-protein therapy. Neurology, 1983, 33:24-38.
Slonim, A.E., Coleman, R., Moses, S., Weisberg, C., Shipp, E., Mushlin, P.: Amino acid disturbances in type III glycogenosis: Differences from type I glycogenosis. Metabolism, 1983, 32:70-74.
Slonim, A.E., Coleman, R.A., Moses, W.S.: Myopathy and growth failure in debrancher enzyme deficiency: Improvement with high protein nocturnal enteral therapy. J. Pediat., 1984, 105: 906-911.
Slonim, A.E., Goans, P.J.: Myopathy in McArdle’s syndrome: Improvement with high protein diet therapy. N. Engl. J. Med., 1985, 312:355-359.
Cinnamon, J., Slonim, A.E., Black, K.S., Gorey, M.T., Scuderi, D.M., Hyman, R.A.: Evaluation of the lumbar glycogen storage disease: CT demonstration of paraspinal muscle atrophy. J. Neuroradiology, 1991, 12:1099-1103.
Goldberg, T., Slonim, A.E.: Nutritional therapy for hepatic glycogen storage diseases. J. Amer. Diet. Assoc., 1993. 93:1423-1430.
Vlachopapadopoulo, E., Zachweija, J.J., Gertner, J.M., Manzione, D., Bier, D.M., Matthews, D.E., Slonim, A.E.: Metabolic and clinical response to recombinant human insulin-like growth factor-I (rhIGF-I) in myotonic dystrophy. J. Clin. End. Metab., 1995, 80:3715-3723.
Alemzadeh, R., Langley, G., Upchurch, L., Smith, P., Slonim, A.E.: Beneficial effect of diazoxide in obese hyperinsulinemic adults. J. Clin. Endoc. Metab., 1998, 1911-1915.
Slonim, A.E., Bulone, L., Ritz, S., Goldberg, T., Chen, A., Martinuik, F.: Identificationof two subtypes of infantile acid maltase deficiency. J. Pediatrics. 2000, 137:283-285.
Slonim, A.E., Bulone, L., Damore, M.B., Goldberg, T., Wingertzahn, M.A., McKinley,M.J.: A preliminary study of growth hormone therapy for Crohn’s disease. N. Engl. J. Med. 2000, 342: 1633-1637.
Vladutiu, GD, Slonim AE. Combined biochemical and molecular diagnosis in blood of a common lipid myopathy. Muscle Nerve. 2000 Nov; 23(11)):1779-84.
Slonim, A.E., Bulone, L., Minikes, J., Hays, A.P., Shanske, S., Tsujino, S, DiMauro, S.Benign course of Glycogen Storage Disease Type IIb in two brothers: Nature or Nurture? Muscle Nerve 2006; 33: 571- 4
Slonim AE, Bulone L, Slonim E, Goldberg T, MinikesJ, Galanko J, Martiniuk F. AdultAcid Maltase Deficiency: Modification of natural history by Nutrition & Exercise Therapy. Muscle and Nerve 2007; 35: 70-77.
Alfred E. Slonim, MD, Melvyn Grovit, DPM, MS, CNS and Linda Bulone, RN. “Effect of exclusion diet with nutraceutical therapy in juvenile Crohn’s disease” published June 2009, vol.28, no.3, pp.277-285 in The Journal of the American College of Nutrition