Ankara Üniversitesi Tıp Fakültesi Mecmuası 0365-8104 1307-5608 Ankara University Medical Biochemistry - Lipids Medical Biochemistry - Proteins, Peptides and Proteomics Tıbbi Biyokimya - Lipidler Tıbbi biyokimya - Proteinler, Peptitler ve Proteomik Primary Hyperlipoproteinemia: Case Report of a Boy with Familial Hypertrialyceridemia and Review of the Literature Primer Hiperlipoproteinemi Bir Hipertrigliseride Mİ Vakasının Takdimi ve Literatürün Gözden Geçirilmesi Danışoğlu Necip ANKARA ÜNİVERSİTESİ, TIP FAKÜLTESİ, DAHİLİ TIP BİLİMLERİ BÖLÜMÜ, İÇ HASTALIKLARI ANABİLİM DALI, GASTROENTEROLOJİ BİLİM DALI 10 31 1972 25 5 1030 1043 09 01 1972 10 31 1972 Copyright © 1947, Ankara Üniversitesi Tıp Fakültesi Mecmuası 1947 Ankara Üniversitesi Tıp Fakültesi Mecmuası

In this paper we discuss a case of hypertrigiyceridemia. In view of the recent developments in serum lipidemias, we are trying to give an insight to the better diâgnosis of primary hyperlipoproteinemias, according to our countriğş vossihilities. When necessary methods for correct laboratory diagnosis are lacking, clinical experience may be helpful. Thus, we present a patient of ours who turns out to bea familial Type: 1 primary hyperlipoproteinemia more because of his clinical impression than laboratory results.

Bu yazımızda bir hipertrigliseridemi vakasını takdim etmekte ve son yıllarda primer hiperlipoproucinemiler alanında görülen değişikliklerden bahseumekteyiz. Memleketimiz şartlarının gereklilaboratuvar tetkiklerinin temin edemediği zamanlarda klinik bulguların değerli olduğu inancındayız. Bu yönden laboratuvar bulgularından ziyade klinik olarak Tip : 1 hiperlipoproetinemi olmasımuhtemel bir vakamızı takdim etmeyi faydalı bulduk.

 Hipertrigliseridemi Hiperlipoproteinemi Klinik 1 — Cecil - Loeb Textbook of Medicine, Beeson and MeDermott, Saunders, s : 1196,1967 2 — Harrison's Principles of Internal Medicine MeGraw.-Hill s: 629, 1970. 3 — FREDERİCKSON, D.S., LEVY, R.JI., LEES, R.S.: Fat Transport in lipoprotelns: An Integrated Approach to Mechanisms and Disorders, New Eng. J. Med., 276 : 34, 94, 142, 215, 278, 1967 4 — Classificatlon of Hyperlipidemias and Hyperlipoproteinemias, Bulletin of the World Health Organisation, 43, 891, 1970 and by permisslon Circulation 45 : 274, 1972 5 — STEİNBERG, D.: Some Observations on Hyperlipoproteinemias and their Classitication, 45 : 247, 1972 6 — ADLERSBERG, D., FELDMAN, E.B.: lipids in Pacreatitis, Gastroenterology 36 : 832, 1959 7 — ADLERSBERG, D.: Hormonal Influences on the Serum Lipids, Am. J Med., 28 : 169, 1957. 8 — Cecil - Loeb Textbook of Medicine, Beeson and McDermott, Saunders, s : 1665, 1971 9 — ALPTUNA, E.: Safra asitleri ve Klinik Önemi, Ankara Ü. Tıp Fak, Mec, 24 : 1024, 19T1 10 — TYTGAT, G.N., RUBİN, C.E., SAUNDERS, D.R.: Synthesis and Transport of Lipoprotein Particles by Intestinal Absorbtive Cells in Man, J. Clin. Invest., 50 : 2065, 1971 11 — EATON, R.P., KIPNİS, D.M.: Physiologic Studies of Plasma pre - beta Apo-protein in Man, Diabetes, 20: suppl. I, 329, 1971. 12 — ARKY, R.A.: Post- Heparin Lipolytic Activity in Diabetic Patients with a History of Mixed Hyperlipemia: Relative Rates Against Artificial Substrates and Human Chylomicrons, Diabetes 18 : 562, 1969 13 — BARRY, W.S., BRAY, G.A.: Plasma Trigiycerides in Genetically Obese Rats, Metabolism, 18 : 833, 1969 14 — CHAZAN, B.I., FERGUSON, B.D. CASTELLİ, W.P., TOUBORG, N.F,, BLADİMUS, M.C., RUTSTAN, D.: Lipemia Retinalis: Microcirculatory Changes and Lipid Studies in a Family, Metabolism, 18: 978, 1969. 15 — CAMİTTA, F.D. GRAY, T.K.: A Study in the Pathogenesis of Hyperlipemia in a Patient with Acromegaly and Diabetes Mellitus, Diabetes: 18: 51, 1969 16 — BAGDEDE, J.D., BİERMAN, E.L.,, PORTE, D.; Influence of Obesity on the Relatlonship Between Insulin and Trigiyceride Levels in Endogenous Hypertrigiyceridemis, Diabetes, 20: 664, 1971. 17 — HARPER, L., DAVİDSON, P., ALBRİNK, M,, WALKER, J.: Hypertrigiyceridemia: An Indication of Latent Diabetes, Diabetes, 20 : Suppl. I, 359, 1971 18 — PARK, B.N., LEVY, R.I, SOELDNER, J.T., GLASON, R.E., FREDERİCKSON, D.S.: Hormone substrate Relationships to Plasma Lipid Levels in Normal and Prediabetic Males, Dlabetes 20: Suppl, I, 371, 1971. 19 — BANES, P.A., JANOWİTZ, H.: Some Metabolic Aspects of Exocrine Pancreatic Disease, Gastroenterology, 56: 601, 1969. 20 — CAREN, R., CORBO, L.: Depression of Plasma Pipid Fractions and Inhibitlon of Platelet Aggregation by Action of Glucagon, Metabolism, 20 : 1507,1971 21 — JEYNER, C.R., Jr: Essential Hyperlipemia, Ann, İnt. Med. 88: 759, 1953. 1953 22 — BANKS, S., MAKS, I.N.: Hyperlipemlae, pancreatitis and the PIN, Postgrad. Med. J., 46: 576, 1970. 23 — KEKKİ, M., NİKKİLA, E. AL.: Plasma Trigiyceride Turnover During Use of Oral Contraseptives, Metabolism, 20 : 878, 1972 24 — PİCARD, J., VEİSSİERE, D., VOYER, F.: Les lipoprotines anorales de la Cholestase, La Presse Med., 78: 2319, 1970. 25 — LOEPER, J., ROUFFY, J.: Hiperlipoprot&in&mies Atherogenes İntéret d3 Videntification chez Venfant, La Presse Med., 78: 1219, 1970. 26 — GROSS, J.B., COMFORT, M.W.: Chronic Pacreatitis, Am. J. Med, 21: 596, 1956 27 — KLATSKİN, G., GORDON, M.: Relationship between Relapsing Pancreatitis and Essential Hyperlipemia, Amn. İnt. Med. 12: 1952. 28 — CHİN, H.P.: Clinical Felectrophoresis, Plasma Lipoproteıns: and Giycoproteins, Gelman Instrument Company Pamphlet, s: 3-21, 1969, 29 — FREDRİCKSON, D.S., LEES, R.S.: A system for phenotyping Heyperlipoproteinemia, Circulation, $1: 331, 1965. 30 — MOREAU, P.G, PİCHON, P. RİFLE, G.: Manifestatlons Choriorétiniennes des Hyperlipidémies. La Rétinopathie Hyperlipidémique. A propos de 44 observatlons., Sem. Höp, Paris, 46 : 3467, 1970 31 — SMELE, L.S., ELLİOTT, H.C.: Lipoprotein patterns in Diabetes in Diabetes Mellitus and Their Pelations to it's Complicatjons, Diabetes 20; Sayı: 1: I, 378, 1971. 32 — LOBPER, JI., TRİCOVT, R., LOEPER, J., ROUFFY, J.: Valeur du Lipidogramme, Joint au dosage des Fractions Lipidiques du sérum pour le Dépistage et la classification des hyperlipoprotéinémiques, Lu Presse Med. 71: 171, 1969. 33 — WOOD, P.D.S.. STERN, M.P., SİLVEN, A, REAVEN, G.M.: Von der Groeben, J,, Prevalence of Plasma Lipoprotein Abnormalities in a Free - Living Population of the Central Valley, Califormia, Circulation, 45: 114, 1972. 34 — AKTAN, H., ONARAN, L, PAYKOÇ, 2.: Hiperlpemia ve Pankreatitis, Mavi Bülten, I: 34, 1071.