Yoshihide Uraoka

Plasma Factor XIII Levels in Children with Renal Disease

Plasma levels of coagulation factor XIII determined quantitatively were found to have a tendency to be decreased in more severely affected cases of acute poststreptococcal glomerulonephritis and Henoch-Schönlein nephritis, and found to be decreased in patients with rapidly progressive glomerulonephritis and chronic renal failure in comparison with normal controls. It seems that the degree of decrease in factor XIII reflects the intensity of glomerular damage. In contrast, the patients with the nephrotic syndrome showed significant elevation of plasma factor XIII levels and it was closely correlated with the increase in serum triglycerides. The elevation of factor XIII levels in the nephrotic syndrome is suggested to be mainly attributed to enhanced protein synthesis in connection with urinary loss of proteins.

Urinary Fibrinogen and Fibrin Fragments in Children with Renal Disease

Fibrinogen fragments (X, Y, D and E) and fibrin fragments (D-dimer and E) were examined in the urine of 52 patients with various types of renal diseases. One or more of the fibrinogen fragments were detectable in all the urine specimens. D-Dimer together with fibrinogen fragments was found in 38 of the 52 patients. The clearance ratio of D-dimer to IgG, which indicates D-dimer generated in the kidney, was lower than 1 in all the patients with the minimal changes nephrotic syndrome, and was greater than 1 in the majority of patients with acute glomerulonephritis, rapidly progressive glomerulonephritis, mesangial proliferative glomerulonephritis and membranoproliferative glomerulonephritis. Our results suggest that urinary fibrin/fibrinogen degradation products (FDP) in renal diseases are derived primarily from increased filtration of FDP from the plasma through a damaged glomerular basement membrane, and that the mechanism of lysis of cross-linked fibrin deposited in the glomeruli occurs simultaneously in some types of glomerulonephritis. It seems that the determination of the clearance ratio of D-dimer to IgG may be useful in assessing the activation of the coagulation and fibrinolysis systems in the kidney in patients with renal diseases.

Coagulation and Fibrinolytic System in Various Glomerular Diseases in Childhood 3rd Report–Plasma Factor XIII

The purpose of this study was to clarify the role of plasma factor XI11 (F.XII1) in various glomerular diseases. F. XI11 was determined with the monodansyl cadaverine incorporation method in 107 children with renal disease [normal range: 16 f 4 (SD) U/ml]. There was significant decrease of plasma F.XII1 in the acute phase of acute glomerulonephritis (12 f 3 U/ml), which did not correlate with proteinuria, ASO, BUN or creatinine clearance (FIG. I). F.XIII was also low in chronic glomerulonephritis (1 1 k 3 U/ml) and in rapidly progressive glomerulonephritis (9 f 1 U/ml), yet in the normal limits in persistent glomerulonephritis (16 f 4 U/ml). Plasma F.XIII was found to be significantly higher in patients with nephrotic syndrome (18 k 6 U/ml), and correlated with serum triglyceride. No correlation was observed between plasma F.XII1 level and serum albumin, cholesterol, plasma fibrinogen, serum or urine FDP, platelet count, proteinuria, renal histology or corticosteroid treatment. Such elevation is assumed to be mainly due to enhanced hepatic synthesis of the coagulation factor, resulted from protein loss into the urine. Plasma F.XII1 level was found to be significantly lower in the acute stage of HenochSchonlein purpura nephritis (12 k 5 U/ml), and there was also a decrease of F.XII1 in Henoch-Schonlein purpura without renal involvement (1 1 & 4 U/ml). In the chronic stage of purpura nephritis, F.XII1 was observed to be in the normal range (14 f 2 U/ml). In the acute stage of purpura nephritis, the group with a decreased level of F.XII1 showed significantly heavier proteinuria during the clinical course than the group with the normal F.XIII. Diffuse intensive lesions and a fairly high incidence of crescent formation, though less than 50%, were observed in three of four patients in the decreased group, and the crescent was, in contrast, found in none of two patients in the normal group. Outcome was seemed to be more favourable in the latter group. These findings suggest that intra-renal coagulation, which decreases plasma F.XIII secondarily, may play a role in the pathogenesis of HenochSchonlein purpura.

Clinical and Epidemiological studies in Acute Febrile Mucoutaneous Lymph Node Syndrome in Osaka Prefecture (Kawasaki Disease)

The acute febrile mucocutaneous lymph node syndrome (MCLS) is a newly recognized disease and its cause and pathogenesis are not yet defined. We experienced 48 patients with MCLS between May 1975 and August 1979, and studied these patients clinically and epidemiologically. The results of epidemiological studies are as follows. Patients with MCLS increased yearly in the last five years, and more were experienced in July, August, November and December, and fewer in April and October. Endemic outbreaks were noticed in a few regions, such as the T area in Sakai City (FIG. I). The age distributions of the patients were 2.1% in 0-6 months of age, 25.0% in 6-12 m.o.a., 8.3% in 12-18m.o.a., 14.6% in 12-18m.o.a., 6.3% in 2-3 years of age, 12.5% in 3-4 y.o.a., 14.6% in 4-5 Y.o.s., 8.8% in 5-6 y.0.a. and 8.4% over 6 years of age. The sex distribution disclosed 35 males and 13 females (the mole to female ratio was 2.7:l). Clinically, the symptoms varied. Aneurysm of the coronary artery was noticed in five patients, aseptic meningitis-encephalopathy in three, jaundice in six, abnormal liver functions in 18, bronchopneumonic changes in chest Xray photographs in 15, and transient renal damage (proteinuria and hematuria) in five. In laboratory examinations, positive C-reactive protein, increased erythrocyte sedimentation rate, leukocytosis with nuclear shift to the left, and a slight degree of anemia were found in the same way as described by other authors. FIG. I . The position of outbreaks of the MCLS patients (0) and Kinki University (X) .

Four Cases of Hemolytic Anemias: Clinical Courses of 4 Patients of Different Causes and Their Lipids Composition of Red Cells

Four cases of hemolytic anemia were subject to a special investigation on their lipid constitution of erythrocytes, and the results are described in this report. The first case was of GGPD-deficient disease carried by his mother, having repeated the hemolytic fit on suffering some infectious disease. A prudent selection of medicines for the therapy had brought marked improvement without such a symptom during the past three years. The second case was of autoimmune hemolytic anemia caused by IgG warm type of autoantibody, and the patient had been a carrier of HB virus. He healed of the disease by himself within two months. The third case was of hereditary spherocytic hemolytic anemia. We assumed his father also had suffered from the disease. Because of a failure in obtaining the const of his family, his spleen was not removed. The fourth case was of sporadic spherocytic hemolytic anemia which had been found on the onset of aplastic crisis derived from urinary tract infection. Before removal of her spleen, the anemia of Dacie I1 type was detected by autohemolysis test, and after the removal, she was much improved and that of Dacie I. With respect to the lipid constituents of the four patients on admission, the cholesterol level was comparatively low in the two cases of spherocytic hemolytic anemia. In all the four cases, the level of phosphatidyl choline increased, while the levels of fractions of phosphatidyl serine and phosphatidyl inositol decreased.

Coagulation and Fibrinolytic System in Various Glomerular Diseases in Childhood: 2nd report A Study on the Origin of Urinary Fibrin/Fibrinogen Degradation Products (FDP)

The present study was undertaken to clarify the origin of urinary FDP in various glomerular diseases in childhood. Urinary FDP were measured by hemagglutination inhibition (HI) test. The relationships of urinary FDP with urinary protein and histological findings were studied in 71 patients of various glomerular diseases. Furthermore, the molecular distribution of urinary FDP was analysed before and after treatment of thrombin, using Sephadex G-200 gel filtration from ten patients among them. The amount of FDP in each eluate was measured by HI test and the filtration pattern of FDP was compared with that of an eluate obtained after gel filtration of a solution of human fibrinogen digested by plasmin.