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J R Patsch

Publications and source records attributed to J R Patsch.

155 records · Page 9Linked to original sources
PubMed1976

Electron microscopic characterization of lipoproteins from patients with familial type III hyperlipoproteinaemia.

The lipoproteins of very low density, low density and intermediate density were isolated by zonal ultracentrifugation from the plasma of subjects with familial type III hyperlipoproteinaemia and studied by electron microscopy. Each of these lipoproteins exhibited a spherical shape when free-standing. The very low density, low density and intermediate density lipoproteins ranged in diameter from 31 - 80, 17 - 23, .and 21 - 35 nm, respectively. These results are consistent with our previous findings that the intermediate density lipoprotein particles characteristic of plasma from type III subjects exhibit properties which are intermediate between those of very low density and low density lipoproteins.

Centrifugation, Zonal↗
PubMed1976

Isolation and partial characterization of two abnormal human plasma lipoproteins: LP-X1 and LP-X2.

Two abnormal lipoproteins (LP-X1 and LP-X2) rich in free cholesterol and phospholipid have been isolated from the plasma of nine different patients with obstructive jaundice. As isolated, both lipoproteins were devoid of low density lipoproteins as judged by agar electrophoresis. In addition, they were free of apoB as judged by double immunodiffusion. However, LP-X1 exhibited a higher phospholipid to protein ratio and a lower hydrated density than LP-X2. The levels of LP-X1 and LP-X2 in plasma were measured in three patients studied for 14 days after corrective surgery for obstructive liver disease. The level of both these abnormal lipoproteins decreased after surgery until the twelfth day when they were completely absent from the plasma of these patients. The rates of this decrease for the two particles are significantly different. These differences in metabolic, chemical, and physical properties of LP-X1 and LP-X2, establish their identity as two distinct and unique lipoprotein particles.

Adult↗
PubMed1975

Lipoprotein of the density 1.006-1.020 in the plasma of patients with type III hyperlipoproteinaemia in the postabsorptive state.

Using a rate zonal ultracentrifugal technique in 10 patients with type III hyperlipoproteinaemia, very low density lipoproteins (VLDL) (d less than 1.006, S(f) greater than 20), an intermediate lipoprotein density class with a S(f) range of 15 - 20 (Lp III), and low density lipoproteins (LDL) (S(f) 6 - 8) could be isolated from the plasma. The Lp III was found as a symmetrical peak between VLDL and LDL; its plasma concentration was 248 mg/100 ml plasma (mean), 92 - 469 mg/100 ml plasma (range); the Lp III/LDL ratio was 0.65 (mean), 0.25 - 2.3 (range); the chemical composition w/w was 35.8 percent total cholesterol, 19.3 percent triglycerides, 24.3 percent phospholipids, and 20.4 percent protein (mean). In agarose gel electrophoresis, the Lp III migrated faster than LDL, but slower than VLDL. By means of a double immunodiffusion technique and immunoelectrophoresis, apo-Lp B and apo-Lp C were detectable. In an angle head rotor at 1.4 x 10-8 g. min. Lp III can be separated from VLDL at a solvent density of 1.006, and from LDL at a density of 1.025. In the postabsorptive state in one patient with type I hyperlipoproteinaemia, five with type II and 20 with type IV, and in 50 healthy persons of both sexes, this Lp III peak was not found. The finding of a high concentration of Lp III and a rather low concentration of LDL in the plasma of patients with type III hyperlipoproteinaemia can be regarded as further evidence for an impaired catabolism of VLDL to LDL at Lp III ("intermediate" lipoprotein).

Blood Protein Electrophoresis↗
PubMed1974

Separation of the main lipoprotein density classes from human plasma by rate-zonal ultracentrifugation.

The major lipoprotein density classes (chylomicrons-VLDL, LDL, HDL(2) and HDL(3)) were isolated from human plasma in a two-step ultracentrifugal procedure using the Ti-14 zonal rotor. The isolation of the two major high density lipoprotein subclasses (HDL(2) and HDL(3)) was achieved in a 24-hr run using a nonlinear NaBr gradient in the density range of 1.00-1.40. The lipoproteins with a density < 1.063 found in the rotor's center were isolated in a second run of 140 min duration using a continuous linear NaBr gradient in the density range of 1.00-1.30. The isolated lipoproteins were analyzed for chemical composition and for electrophoretic mobility; purity of isolated fractions was checked by immunochemistry. The lipoproteins exhibited flotation rates, chemical compositions, and molecular weights similar to those found with the common sequential procedures in angle-head rotors. The amount of lipoprotein lipids in the bottom fraction of the zonal rotor was comparable to that of the angle-head rotor. The described method yields the main lipoprotein density classes free from albumin in a very short running time; compared with the rate-zonal techniques already in use, this method allows the quantitative separation of an additional lipoprotein density class (HDL(2)) without increasing the running time. Furthermore, this procedure proved to be suitable for isolation of plasma lipoproteins from subjects with various types and varying degrees of hyperlipoproteinemia.

Adolescent↗
PubMedDate not supplied

Anti-thymocyte globulin treatment of a patient for paroxysmal nocturnal haemoglobinuria-aplastic anaemia syndrome: complement activation and transient decrease of the PNH clone.

Paroxysmal nocturnal haemoglobinuria (PNH) is an acquired clonal stem cell disorder resulting in insufficient and defective haematopoesis associated frequently with aplastic anaemia (AA). A deficiency of the glycosyl phosphatidylinositol (GPI)-anchored complement activation regulatory proteins CD55 and CD59 is responsible for an increased sensitivity of erythrocytes to complement attack leading to chronic intravascular haemolysis with haemoglobinuria. In this study we investigated the effects of complement activation caused by anti-thymocyte globulin (ATG) treatment on the PNH clone in a patient affected with the PNH/AA-syndrome. Fluid phase complement components C3, C4, C6 and terminal complement complex (TCC) were assayed by ELISA. CD55, CD59 and cell-associated TCC were monitored by flow cytometry. ATG treatment resulted in profound systemic complement activation which led to a decrease in the levels of native C3 and C4 to 65% and 40%, respectively, of the original levels on day 5 and of C6 and TCC to 61% and 23%, respectively, on day 10. A return to pre-treatment levels was observed for C3 by day 15, for C6 by day 30 and for C4 by day 90. Flow cytometry revealed that the deficiency in the GPI-anchored protein was restricted to granulocytes, while lymphocytes remained unaffected. Cell-bound TCC increased by 1.67-fold and 2.37-fold on day 5 and day 10, respectively, decreasing to 1.40-fold and 1.30-fold on day 15 and day 30, respectively. The percentage of PNH granulocytes as identified by the absence of the CD55- and CD59-antigens exhibited a temporary decrease from 72% on day 0 to 65% on day 5 and 59% on day 10 and returned thereafter to the original percentage of 70% by day 15 and exceeding this level to 76% on day 30 and 79% on day 90. We report profound activation of the classical pathway of the complement cascade and the terminal complement complex by the globulin leading to a transient decrease of the PNH clone, presumably due to subsequent lysis of the PNH cells devoid of complement regulatory proteins.

Anemia, Aplastic↗
PubMedDate not supplied

Genetic susceptibility and resistance to diet-induced atherosclerosis and hyperlipoproteinemia.

To test the hypothesis that genetic susceptibility or resistance to diet-induced atherosclerosis is correlated with serum levels of specific lipids, lipoproteins, or apoproteins, male mice of a genetically susceptible and a genetically resistant strain were fed either a normal or an atherogenic diet. After 20 weeks on a normal diet, neither the resistant nor the susceptible strain mice had atherosclerosis; resistant strain mice had serum cholesterol of 66 +/- 11 while the susceptible strain mice had 90 +/- 1 mg/dl serum cholesterol, and lipoproteins were dominated by a single alpha-migrating HDL. After 20 weeks on an atherogenic diet, resistant strain mice had 185 +/- 55 mg/dl cholesterol, their lipoproteins remained dominated by alpha-migrating HDL, and two of eight mice had mild atherosclerotic lesions; susceptible strain mice had 510 +/- 94 mg/dl cholesterol, multiple alpha- and pre-beta-migrating lipoprotein species, and all 13 had advanced aortic atherosclerosis. The resistant strain mice had an apolipoprotein E/total lipoprotein protein ratio of 0.42 on the normal diet and 0.53 on the atherogenic diet, while the susceptible strain mice had the significantly lower ratios of 0.07 and 0.31, respectively. These data indicate that genetic resistance to diet-induced aortic atherosclerosis in mice is correlated with capacity to prevent large increases in serum cholesterol, to suppress abnormal alpha- and pre-beta-migrating lipoproteins, and to maintain elevated serum apolipoprotein E/total lipoprotein protein ratios. Our data do not preclude the possibility of additional gene control at the level of arterial end organ response.

Animals↗
PubMedDate not supplied

Prevention of perinatal morbidity by tight metabolic control in gestational diabetes mellitus.

In a prospective controlled trial, we studied the effect of tight metabolic control on the outcomes of 102 gestational diabetes mellitus (GDM) pregnancies compared with outcomes of 102 matched nondiabetic control pregnancies. Women with GDM were treated to achieve and maintain a blood glucose concentration of less than 130 mg/dl at 1 h after breakfast. Treatment consisted of a diet low in oligosaccharides and fat and, if necessary, once daily insulin. By the end of gestation, 88 of the 102 women with GDM received insulin at a mean dose of 18 U/day. Duration of insulin therapy ranged from 3 to 32 wk with a median of 11 wk. Perinatal outcome of GDM pregnancies under this management equaled that of control pregnancies. The full spectrum of excess morbidity from GDM was prevented, and normal distribution of birth weight and normal rates of macrosomia, dystrophy, hypoglycemia, hypocalcemia, hyperbilirubinemia, fetal acidosis, and low Apgar scores were achieved. No mortality was observed. In addition to the two main study groups, we also studied a third group of 24 women with GDM whose treatment lasted less than or equal to 5 wk due to late diagnosis. This suboptimally treated group demonstrated a significant (P less than .05) increase of macrosomia and umbilical artery acidosis compared with the well-treated GDM group. The study reported herein demonstrates that excess mortality and morbidity typically observed in GDM can be prevented by early institution of tight metabolic control, which required insulin in 86% of our patients.

Birth Weight↗