Biomedical subjects
Publications and source records attributed to S Ozen.
A seven-year-old girl is presented, who was previously diagnosed as having Henoch-Schönlein vasculitis, and was further evaluated for hypertension. Urograms showed bilateral strictures of the ureter, causing obstruction on the left side. Tuberculosis, our preoperative diagnosis, evidenced by her sterile pyuria and PPD conversion was confirmed by the surgical specimen and treatment was begun.
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Systemic lupus erythematosus (SLE) is a rare disease in childhood. Here two cases with SLE are presented, both with C4 null alleles yielding a functional C4 deficiency. The first case, a 14-year-old girl with a C4A null allele only, had a mild disease course, whereas the second child, a seven-year-old boy with both C4A0 and C4B0, had a more relentless course leading to death in five years. We conclude that complement activation by the classical pathway might be an essential mechanism that protects against the emergence of autoimmune or immune-complex diseases, and that the deficient state in our patients predisposed them to the early development of SLE.
A four-year-old girl presenting with fever and purpuric lesions was diagnosed with systemic vasculitis based on her clinical and laboratory findings. She also had homozygote beta thalassemia. Oral steroids were administered and during the course of her treatment she developed necrotizing lesions on her extremities along with severe myalgia and an autoimmune refractory anemia. Autoantibodies against the Rh antigen causing a persistent hemolysis of her erythrocytes were detected in her serum. Since no improvement in her skin lesions and autoimmune hemolytic anemia was achieved with bolus methylprednisolone therapy and cyclophosphamide, plasma exchange was performed. After three sessions of plasma exchange, her immune complex and autoantibody levels gradually declined and a remission in her clinical and laboratory findings was achieved. We suggest the use of plasma exchange along with conventional therapy for similar cases with ongoing immunologic injury.
Cardiovascular complications are an important cause of morbidity and mortality in chronic hemodialysis patients. In order to examine the effect of parathyroid hormone (PTH) and vitamin D on left ventricular functions, 11 patients between the ages of 13 and 18 years on regular hemodialysis were investigated using M-mode echocardiography and systolic time intervals. The ratio of the pre-ejection period to the left ventricular ejection time was found to be 0.38 +/- 0.02 (range (0.25 +/- )>50) and was elevated above normal in five of the 11 patients examined. Four of these patients has hypertension and one had severe anemia. The left ventricular ejection fraction was 55 +/- 2.54% and fractional fiber shortening was 31.55 +/- 2.26%, both of which were within normal limits for age. Although the velocity of circumferential fiber shortening was within normal limits in the majority of cases, the mean value was 1.437 +/- 0.11 circ/s, which is above normal for this age period. PTH levels were between one and 4.70 ng/ml. All of the hemodialysis patients had been receiving 1 alpha hydroxy-cholecalciferol and had normal calcium levels. Although they had high PTH levels, most of these patients displayed normal myocardial contractility. No significant correlation was obtained between increment in PTH levels and myocardial function indices. These results imply that PTH is not the only factor affecting myocardial functions. Since all of these patients have received vitamin D therapy for long periods, we suggest that vitamin D may have prevented the deleterious effect of PTH on myocardial function.
The efficacy of oxybutynin, pseudoephedrine and indomethacin treatment was investigated in 29 patients with primary nocturnal enuresis. Patients were randomly assigned to either oxybutynin (1st group, n = 9), pseudoephedrine (2nd group, n = 11) or indomethacin (3rd group, n = 9) treatments. Oxybutynin and indomethacin did not cause a statistically significant difference in the number of dry nights (p > 0.05), but patients treated with pseudoephedrine had a significant increase in the number of dry nights (p < 0.05). Five patients in the oxybutynin and one patient in the indomethacin group experienced side effects. None of the patients in the pseudoephedrine group had any complaints with the drug. We therefore conclude that pseudoephedrine can be an alternative in the treatment of primary nocturnal enuresis.
We report a case of systemic lupus erythematosus initially presenting with thrombocytopenia and diagnosed as immune thrombocytopenic purpura. The patient subsequently developed lymphadenopathy, arthritis and cardiac involvement along with anticardiolipin antibodies. We would like to emphasize the fact that these autoantibodies have a role in the pathogenesis of thrombocyte destruction, and that patients with immune thrombocytopenic purpura should be followed for signs of systemic lupus erythematosus.
OBJECTIVE: To evaluate the changes in the plasma levels of thrombomodulin (TM), tissue-type plasminogen activator (t-PA) and plasminogen activator inhibitor-1 (PA1-1) as indices of endothelial injury/activation and fibrinolysis in childhood Henoch-Schönlein purpura (HSP). METHODS: Twenty-six children with HSP aged between 4-15 years and 10 healthy controls were included in the study. Blood samples were taken from these patients at admission and 6-12 weeks after healing of skin rash and arthritis. Plasma levels of TM, t-PA and PAI-1 activities and t-PA and PAI-1 antigen (Ag) levels were measured. RESULTS: The plasma levels of TM, t-PA Ag and PAI-1 Ag in patients during the acute phase were significantly different from the controls. The difference in TM between the acute phase and recovery in patients was also significant. The decrease in plasma levels of t-PA Ag and PAI-1 Ag in patients between the acute and recovery phases was not significant. t-PA activity was significantly higher in the acute phase than in the recovery phase. CONCLUSION: We suggest that increased levels of TM, t-PA, and PAI-1 activity may reflect the presence of endothelial injury and/or activation and fibrinolytic activation in patients with HSP.
Autosomal recessive polycystic kidney disease (ARPCD) is a congenital kidney disease with severe prognosis. We present a male infant who was diagnosed prenatally by ultrasonography. He died at two months of age in a septic stage. The genetic defect for ARPCD has been mapped to chromosomal region of 6p21-cen. This represents the first study from this region of the world. The linkage studies up to this date fall to show genetic heterogeneity.