Final height in children with steroid-sensitive nephrotic syndrome

BACKGROUND: Growth retardation following steroid treatment in children is a major problem. Reduction of steroid dose has been tried using immunosuppressive agents such as cyclosporine A or mizoribine in children with frequently relapsing nephrotic syndrome. Few reports concerning final height in steroid-sensitive nephrotic syndrome (SSNS) are available. METHOD: Patients who developed SSNS before 15 years of age and reached their final height were retrospectively studied by standard deviation score (SDS) of height and target height calculated by their parental height. RESULTS: A total of 34 patients were evaluated for their final height. The mean age at onset of SSNS was 8.0 years and the mean age at last follow up was 21.6 years. In total, 22 patients had frequent relapses and were treated with cyclophosphamide, mizoribine or cyclosporin A. All patients had normal renal function at the last evaluation. The mean final height was 168 cm in males and 155 cm in females. The mean height SDS was 0.37 at the time of onset and was -0.43 when they reached their final height (P = 0.0001). The final height was a mean of 2.5 cm below target height and was significantly lower than their siblings (P = 0.007). Final height of two boys who continued to have frequent relapses during puberty and were not treated with cyclosporin A was 146 and 150 cm. CONCLUSION: Final height in children with SSNS was slightly affected by steroid treatment and two patients had severe growth retardation.     

Stromal cell-derived factor-1α improves infarcted heart function through angiogenesis in mice

BACKGROUND: Local delivery of stromal cell-derived factor-1alpha (SDF-1) has been demonstrated to improve hind limb ischemia through enhanced neovascularization in animals. It was hypothesized that local administration of SDF-1 also contributes to neovascularization of ischemic heart. METHOD: Acute myocardial infarction was created by left coronary artery ligation in C57BL/6J mice. Immediately after infarction induction, mice were treated by injection directly into the center of ischemic myocardium either with saline (control group) or SDF-1 (SDF-1 group). Cardiac function was measured on echocardiogram 2 and 4 weeks after infarction. On week 4 mice were killed to evaluate infarction size and capillary vessel density. To determine the contribution of bone marrow cells to angiogenesis, the same procedures were performed on C57BL/6J chimeric mice reconstituted with green fluorescent protein-positive bone marrow cells. RESULTS: Fractional shortening was greater in the SDF-1 group at 4 weeks (0.31 +/- 0.06% vs 0.23 +/- 0.03%, P = 0.037). The infarct area was smaller in the SDF-1 group compared to the control group (9.31 +/- 2.76% vs 18.07 +/- 5.69%, P = 0.028). Green fluorescent protein-positive cells accumulated predominantly at the peri-infarction site, and were located with the capillary vessels. Capillary vessel density was significantly increased in the SDF-1 group (13.08 +/- 4.11 vessels/mm(2) vs 34.50 +/- 7.59 vessels/mm(2), P = 0.014). CONCLUSIONS: SDF-1 protects against deterioration of cardiac function after acute myocardial infarction by promoting angiogenesis. The safety and long-term prognosis of this treatment remains to be determined.     

Reduced shear stress and disturbed flow may lead to coronary aneurysm and thrombus formations

BACKGROUND: With Kawasaki disease it is important to clarify the mechanisms of coronary artery aneurysm and thrombus to avoid acute myocardial infarction. The authors tested the hypothesis that shear stress is reduced at coronary branching sites and in coronary artery aneurysms, and that this reduction of shear stress can promote formation of coronary artery aneurysms and thrombus. METHODS: The subjects were 111 children with Kawasaki disease with left coronary artery aneurysms, classified into three groups: giant coronary artery aneurysm (n= 28, diameter of coronary artery >8 mm), aneurysm (n= 44, diameter of coronary artery =8 mm), and normal-appearing coronary (n= 39). Averaged peak flow velocity (APV), flow patterns and shear stress were measured and calculated at normal-appearing coronary vessels, left coronary artery branching sites and intra-coronary aneurysm using flow wire, and coronary angiography. Also, presence and appearance of thrombus were detected by intravascular ultrasonography. RESULTS: The authors found that 90.3% of the coronary artery aneurysms occurred at major left coronary branching sites. APV and shear stress were significantly decreased in giant coronary artery aneurysms (APV, 7.1 +/- 2.1 cm/s; shear stress, 3.8 +/- 2.1 dyne/cm(2)) and at the left coronary artery branching site (APV, 9.1 +/- 1.2; shear stress, 1+/-+/-.2 3.0). In total, 20 of 24 thrombi were detected only in giant aneurysm, and all patients exhibited disturbed flow pattern in their giant coronary artery aneurysms. CONCLUSIONS: Reduced shear stress and disturbed flow pattern may lead to coronary artery aneurysm and thrombus formation.     

Sympathetic skin response in diabetic children: Do diabetic children have diabetic neuropathy?

BACKGROUND: Abnormal sympathetic skin response (SSR) has been reported in adult patients with diabetic neuropathy. In addition, other studies have revealed abnormal SSR in diabetic patients not having autonomic symptoms and autonomic dysfunctions. These findings have been only obtained from adult patients. There have been few reports on the autonomic functions in diabetic children. Accordingly, it is not clear whether the autonomic neuropathy occurs in diabetic children. The aim of the present study is to clear autonomic function in children with insulin-dependent diabetes mellitus by SSR. METHODS: The SSR was measured in 28 normal healthy children and in eight patients with IDDM not having symptoms of dysautonomia. The SSR was elicited using 10 stimuli on programmed Nihonkoden Neuropack Sigma model machine. Following a single electrical stimulation, four SSR were recorded in both the palms and the soles simultaneously. RESULTS: The SSR were simultaneously obtained in 100% of the two groups. The amplitudes in the palms and soles were not significantly different between the two groups. The mean and shortest latency in the soles were significantly longer in the IDDM group than in the control group (P < 0.01). None of the measurements of SSR revealed correlation with duration of diabetes and onset of illness. CONCLUSIONS: Diabetic neuropathy may not have occurred in young patients having shorter duration of illness. Conversely, assuming that prolonged latency is abnormal, it may even have occurred in them. Follow up on these patients with prolonged latencies would be required.     

A Study of Malignant Bile Duct Stenosis Using Percutaneous Transhepatic Cholangioscopy

Abstract: From June, 1987 to November, 1989, 11 patients with malignant bile duct stenosis, which was later confirmed by surgery or autopsy, were examined by percutaneous transhepatic cholangioscopy (PTCS) and by an endoscopic biopsy. The endoscopic findings obtained with the usual observation methods and methylene blue staining and the histological findings of the biopsy specimens were compared. A fine vascular proliferation was seen in all of the patients and a granular appearance was noted in patients with carcinoma of the major papilla. The papillary appearance was noted in one case each of pancreatic carcinoma, bile duct carcinoma and carcinoma of the major papilla. Marginal protrusion was not noted in patients with pancreatic carcinoma. A distorted narrow segment was seen only in cases of pancreatic carcinoma. The presence of a granular appearance indicated that the carcinoma was exposed on the surface. A high degree of fine vascular proliferation and a papillary appearance tended to indicate a carcinoma which invaded mainly into the fibromuscular layer without invasion of the mucosa. The methylene blue staining method was simple and effective for better visualization of the surface structure of the abnormal area, normal mucosa and the border zone.     

Pacing Failure Due to Markedly Increased Stimulation Threshold 2 Years after Implantation: Successful Management with Oral Prednisolone: A Case Report

In a 53-year-old male who had undergone transvenous permanent pacing, infracardiac potential reduced progressively over a 2-year period and exit block developed, while the stimulation threshold was markedly increased. After oral prednisolone for 5 months, the stimulation threshold reduced gradually along with an increase in intracardiac potential, culminating in no recurrence of exit block for the ensuing 2 years. The clinical course suggests that progressive reduction in intracardiac potential may have presaged the late development of exit block, and that oral prednisolone may be a therapeutic approach for the restoration of ventricular capture even 2 years after implantation.     

Delta infection in Japan: Immunohistological and immuno-electron microscopic study of delta antigen in liver tissue

Abstract Although the vast majority of hepatitis B surface antigen (HBsAg) carriers of the world inhabit South-east Asia, very little is known about delta infection in this area. Therefore, a serological and immunohistological study was made in the Tokyo-Chiba area. One of 58 (1.7%) HBsAg carriers had anti-delta antibody in a high titre in serum. Delta antigen was immuno-histologically localized in the liver in two of 146 (1.4%) HBsAg carriers studied. The antigen was strongly stained in the nuclei, and positive cells were diffusely scattered throughout the liver in both cases. Neither subject was an illicit drug user: one had travelled to Italy 10 years earlier and the other had a blood transfusion during a 5-year residence in Bangkok in the past.
Thus, there is delta infection among non-intravenous drug users in Japan. Delta infection has been linked to severe liver damage, occasionally fatal. Once introduced, it could become epidemic in a country where hepatitis B virus infection is endemic, and might spread among non-drug users.     

Practical Aspects of Rate Adaptive Atrial (AAI,R) Pacing: Clinical Experiences in 44 Patients

Forty-four patients with sinus node disease and chronotropic incompetence but no evidence of AV conduction disturbances were treated with rate adaptive atriul (AAI,R) pacemakers. Medtronic Activitrax and Siemens Sensolog activity sensing single chamber pulse generators were used. Twentyfour patients (55%) had the bradycardia-tachycardia syndrome. The mean folloiw-up time is 20 ± 14 months (range 1–48, median 17 months). All patients remain alive. Two patients were reoperated upon for lead problems without change of pacing mode. One patient developed symptomatic: srecond-degree Wenckebach block during follow-up, and received a DDD,R system. Although 22 of the patients were treated with antiarrhythmic drugs postoperatively, no further cases of significant AV conduction disturbances were seen. During rapid atrial pacing, exercise-induced enhancement of AV conduction was a consistent finding, although less pronounced in patients treated with beta-blocking drugs. One patient developed permanent atrial fibrillation with an adequate ventricular rate. By systematic reprogramming procedures, QRS complex sensing through the atrial electrode could be demonstrated in 25 patients (23/28 with unipolar and 2/16 with bipolar leads). it could be counteracted effectively by pulse generator program selection in all cases. Forty-two of 44 patients (95%) remain in AAI,R pacing with normal function, Rate adaptive atrial pacing can be successfully applied in this patient group.     

Acute myocardial infarction due to thrombus formation in congenital coronary artery fistula

A young female patient developed acute myocardial infarctionduring physical exercise. Coronary arteriography in the acutephase demonstrated a coronary fistula originating from the leftcoronary main trunk and communicating with the right atrium.Several branches of the left coronary artery were obstructedby thrombotic material originating from the fistula, causingan acute myocardial infarction.