An infantile case of cytomegalovirus induced idiopathic thrombocytopenic purpura with predominant proliferation of CD10 positive lymphoblast in bone marrow

An infant with cytomegalovirus infection (CMV) developed idiopathic thrombocytopenic purpura (ITP) at 4 months of age. A bone marrow (BM) aspiration showed a remarkable increase of immature megakaryocytes and prominent proliferation of lymphoblasts. Flow cytometric analysis of the bone marrow cells showed that the predominant cells in the lymphocyte cluster were of B-lineage (CD19) with CD10 (common acute lymphoblastic leukemia antigen) positive. Virus study showed a high titer of CMV antibody. Cytomegalovirus DNA was detected by the polymerase chain reaction (PCR) method in urine, peripheral cells and marrow cells. Low-grade fever, diarrhea and petechiae were accompanied by mild liver dysfunction. Complete remission was made with intravenous high-dose immunoglobulin (IVIg) without progression to overt acute leukemia. The percentage of CD10⁺/CD19⁺ lymphocytes in bone marrow also diminished. We postulated that the proliferation of immature lymphocytes and megakaryocytes in bone marrow was caused by maturation arrest that might result from CMV infection.     

The Characteristics and Distribution of the Scar Tissue Predict Ventricular Tachycardia in Patients with Advanced Heart Failure

Background: Contrast‐enhanced magnetic resonance imaging (CMR) identifies scar tissue as hyperenhanced areas. We sought to clarify the relationship between the scar characteristics and occurrence of sustained ventricular tachycardia (VT) in patients with advanced heart failure. Methods: CMR was performed in 29 patients with dilated cardiomyopathy (DCM group) and 18 patients with ischemic cardiomyopathy (ICM group). The characteristics, volume, and distribution of the hyperenhanced areas were analyzed by CMR. The CMR parameters and clinical arrhythmic events were compared between the two groups. Results: In the DCM group, almost all hyperenhanced areas were nontransmural, and presented frequently in the midwall layer. The volume of the hyperenhanced areas and total number of hyperenhanced segments were greater in patients with sustained VT than in those without. On the other hand, in the ICM group, transmural or subendocardial hyperenhanced areas were detected in the territory of the coronary arteries. The volume of the hyperenhanced areas and total number of transmural hyperenhanced segments in patients with sustained VT were unexpectedly smaller than in those without. However, the percentage of nontransmural hyperenhanced segments was greater in patients with sustained VT than in those without. Conclusions: The presence and magnitude of the nontransmural scar tissue may predict sustained VT in patients with advanced heart failure. There was the possibility that a recruitment bias was responsible for the finding of the smaller scars in the ICM patients with sustained VT.     

The clinical features of Tourette's disorder with obsessive-compulsive symptoms

Twenty-three patients with Tourette's disorder (13 with obsessive-compulsive symptoms [OCS] and 10 without) were comparatively investigated. In contrast to OCS-free Tourette's disorder patients, those with OCS were found to be characterized by (i) a higher incidence of volatile temper, (ii) a higher incidence of compulsive tics, (iii) a higher incidence of perinatal disorders and brain wave abnormalities, (iv) a higher severity as rated using the Seventy Scale, and (v) a higher prevalence of complications, especially of developmental disorders. Of the subjects with OCS-accompanied Tourette's disorder, approximately half had developed OCS by the onset of tics. These findings suggest the likelihood that OCS-accompanied Tourette's disorder is more strongly associated with organic cerebral disorders, independently of sites of tic disorders, than is OCS-free Tourette's disorder.     

A study of cognitive development and behavior problems in mentally retarded children

Abstract Cognitive development in seventy-one mentally retarded children (19 autistic, 52 non-autistic; aged 7–19 years) from a school for handicapped children was studied, using Ohta's scale for evaluating cognitive development level based on language comprehension (Ohta's stage), and other developmental scales. Behavior problems were also examined. The present study reports on the utility of Ohta's stage in non-autistic children, and the relationship between cognitive development level and behavior problems in mentally retarded children. In non-autistic children, there were temporal correlations between Ohta's stage and other development scales (a standard developmental test, speech development, symbolic play development, imitation development), suggesting that in non-autistic children as well, Ohta's stage may serve well as a scale for cognitive development, and reflect symbolic representational functioning. In non-autistic children, most behavior problems in feeding, elimination and sleeping, hyperkinesis, hypokinesis, stereotyped behaviors, self-injurious behavior and licking were closely associated with cognitive development level, and were more often noted in children of lower cognitive development level rather than only in the severely mentally retarded children. Some behavior problems may often occur in the sensorimotor period and hardly occur in the symbolic representational period.     

Acute scrotum caused by Henoch-Schönlein purpura

Abstract A boy aged 3 years and 11 months with arthralgia and purpuric skin rash was diagnosed with Henoch‐Schönlein purpura (HSP) following an acute occurrence of his scrotal pain and swelling 17 days after the appearance of arthralgia. Immediate scrotal exploration was performed to confirm vasculitis of the left epididymis compatible with a scrotal manifestation of HSP. Postoperative course was uneventful. Twenty‐five cases of HSP with acute scrotum reported in Japan, including this case, are calculated and discussed.     

Interleukin-6 inhibits the chemotaxis of human malignant plasma cell lines

The chemotaxis of human malignant plasma cells is promoted by two extracellular matrix proteins (ECMs): fibronectin (FN) and laminin (LN). We examined the effect of the supernatant from a bone marrow stroma cell line, KM-101, on the chemotaxis of human malignant plasma cell lines to assess the chemotaxis-regulatory roles of the bone marrow microenvironment. Five human malignant plasma cell lines, FR4ds, OPM-1ds, U266/B1, RPMI-8226 and ARH-77 showed different profiles of the expression of β1 integrins of FN and LN receptors. FR4ds, OPM-1ds and U266/B1 cells showed chemotaxis promoted by FN (Ch^FN) and LN (Ch^LN). ARH-77 cells showed Ch^FN but not Ch^LN. RPMI-8226 cells did not show either Ch^FN or Ch^LN. The supernatant from KM-101 cells inhibited the chemotaxis of each of these cell lines regardless of whether the chemotaxis was promoted by FN or LN. Among the cytokines produced by KM-101 cells, it was postulated that IL-6 mediated this inhibitory effect because anti-IL-6 monoclonal antibody (MoAb) and anti-IL-6 receptor MoAb significantly reversed the inhibition. Recombinant IL-6 (rIL-6) also exhibited a similar inhibitory effect. Because anti-gp130 MoAb significantly reversed the chemotaxis inhibitory effect of rIL-6, the inhibitory signal is probably transduced via the signal transducing receptor component, gp130. The chemotaxis-regulatory effect is another previously unrecognized function of this pleiotropic cytokine, IL-6. High levels of IL-6 in the bone marrow microenvironment of patients with multiple myeloma appears to be favourable for the localization of myeloma cells there.     

Prenatal and postnatal histories of very low birthweight infants who developed hepatoblastoma*

BACKGROUND: Hepatoblastoma in children of very low birthweight (< 1500 g) is increasing in Japan and this has suggested the presence of either a genetic or environmental etiology. This study was aimed at revealing common prenatal and postnatal histories, including family history of hepatoblastomas in children of very low birthweight. METHODS AND RESULTS: The medical records of 15 patients, nine boys and six girls, were reviewed. The patients were diagnosed at the age of 6-77 months (median 16 months). Their birthweight ranged from 560 to 1380 g (median 826 g) and the gestational age was 23-33 weeks (median 25 weeks). No parents were exposed to any occupational risk factors and there were no characteristic features in the parents' history or the maternal reproductive history, although one patient was born to a mother who had taken a contraceptive before she got pregnant with the patient as a result of in vitro fertilization. A ventricular septal defect and an atresia of the external auditory canal were congenital anomalies seen in the patients, but congenital anomalies associated with hepatoblastoma were not seen. Early postnatal illnesses included respiratory distress syndrome in six patients, symptomatic patent ductus arteriosus in three patients, chronic lung disease in seven patients, cytomegalovirus hepatitis in one patient and cholelithiasis in one patient. Oxygen therapy was given to 13 patients for a period of 4-508 days (median 112 days) and lengths of oxygen therapy and assisted ventilation were significantly longer in patients with a stage IIIB or IV tumor than those with a stage II or IIIA tumor (P = 0.0040 and 0.0190, respectively). Furosemide was used in 13 patients for a period of 6-460 days (median 88 days) and the length of the treatment was also significantly longer in patients with advanced tumors (P = 0.0420). Among the patients at 23-25 weeks of gestation, these treatments tended to be longer in patients with a stage IIIB or IV tumor than those with a stage II or IIIA tumor. CONCLUSIONS: These results suggest the presence of an environmental etiology, rather than a genetic one, which is responsible for the development of hepatoblastoma in children of very low birthweight. Close monitoring of the children after being discharged from the neonatal intensive care unit is essential and a case-control study is necessary to identify risk factors for hepatoblastoma in children of very low birthweight.     

Effect of calcium antagonist,nicardipine, on cerebral blood flow in postasphyxial newborn piglets

An experiment was carried out in nine piglets within 24 h after birth (control group: four, nicardipine group: five) for the purpose of evaluating the effects of a calcium antagonist, nicardipine, on cerebral blood flow changes induced by asphyxia neonatorum. Under respiratory control with a mechanical ventilator, the animals were exposed to hypoxia. The inspiratory oxygen level was lowered at 15 min intervals from 0.08 to 0.06 and then to 0.05. When bradycardia (heart rate: 60/min or less) was observed, 100% oxygen, adrenaline, and sodium bicarbonate were administered for resuscitation. Nicardipine was administered at a dosage of 10μg/kg via bolus injection 30 min after the resuscitation. It was administered thereafter at a rate of 10μg/kg per h. The cerebral blood flow was measured using a laser Doppler velocimeter. The cerebral blood flow, electroencephalograph (EEG), blood pressure, and heart rate were continuously measured for 120min after the resuscitation. In the control group, the mean arterial pressure 35 min after the resuscitation was 60 mmHg or more. However, the cerebral blood flow was lower than the prehypoxia value in the animals with a mean arterial pressure of 75mmHg or less. In the nicardipine group, the mean arterial pressure was lower, but the cerebral blood flow was higher than the prehypoxia value and cerebral ischemia was not induced. The mean arterial pressure 120 min after the resuscitation was 72.0 ± 8.2 mmHg in the control group, while it was 56.7 ± 7.5 mmHg in the nicardipine group. It was significantly lower in the latter. The cerebral blood flow as compared to that before the initiation of exposure to hypoxia (this being considered 100) was 83.1 ± 22.0% in the control group, while it was 125.1 ± 26.2% in the nicardipine group. It was significantly higher in the latter. A suppression burst-like abnormal EEG finding occurred in two of four animals from the control group and was noted in one of five animals from the nicardipine group. In the nicardipine group, the mean arterial pressure was lowered, but a decrease in the cerebral blood flow after asphyxia was totally prevented and the incidence of EEG abnormality was low. It seems possible that this drug protects the brain from asphyxia-induced changes.     

Long-Term Clinical Follow-up after Sirolimus-Eluting Stent versus Bare Metal Stent Implantation in Patients with Acute Coronary Syndrome

Background and Objective: Drug-eluting stents have been shown to reduce the incidence of restenosis and target vessel revascularization (TVR) compared with bare metal stents (BMSs); however, the long-term efficacy of sirolimus-eluting stent (SES) implantation in patients with acute coronary syndrome (ACS) has not been well established. We have investigated the long-term clinical outcome of SES in patients with ACS.
Methods: Consecutive 245 patients with ACS treated by primary stenting within 24 hours after onset were enrolled. There were 128 patients treated with SES and 117 patients were treated with BMS. We evaluated the incidence of major cardiac events (MACE; total death, nonfatal myocardial infarction, TVR) at 3 years, comparing with 8-month clinical outcome.
Results: Eight-month clinical follow-up shows a significantly lower incidence of TVR in the SES group, 3.1% in the SES group versus 9.4% in the BMS group (P = 0.04). At 3-year clinical follow-up, there was no significant difference in the rate of TVR between the two groups, 8.4% versus 12.4% (P = 0.37). Cumulative incidence of total MACE was 9.2% in the SES group compared with 15.9% in the BMS group (P = 0.18). Only one case of stent thrombosis was observed in the SES (late thrombosis), while two cases of stent thrombosis occurred in the BMS group (late and very late thrombosis; P = 0.55).
Conclusion: SES implantation in patients with ACS is associated with favorable long-term clinical outcome with no excess of late stent thrombosis. Further long-term clinical follow-up will be warranted to confirm the safety and efficacy of SES.