Prediction of therapeutic strategy and outcome for antenatally diagnosed pulmonary atresia/stenosis with intact ventricular septum

Background The therapeutic strategy for pulmonary atresia (PA) and severe pulmonary stenosis (PS) with intact ventricular septum is controversial. Recent improvements in prenatal detection necessitate the identification of predictors of outcome for appropriate counseling and prenatal management planning. Methods and Results Echocardiograms of 18 fetuses antenatally diagnosed with PA (n=14) and PS (n=4) were reviewed and the total cardiac dimension (TCD) and tricuspid valve diameter (TVD) were measured. The right ventricular end-diastolic volume (RVEDV) was calculated from the right ventriculogram of the neonatal period by the percentage of the predicted normal value (%RVEDV). There was a positive correlation between TVD/TCD and %RVEDV (p<0.001). As the initial treatment, balloon atrioseptostomy was performed in 13 cases of TVD/TCD <0.26. As the final treatment, patients with TVD/TCD <0.17 underwent or were planned for the Fontan procedure. Patients with TVD/TCD >0.21 underwent or were planned for biventricular repair. Patients whose TVD/TCD was between 0.17and 0.21 underwent or were planned for 1.5 ventricular repairs. Conclusion TVD/TCD is a useful index for selecting the postnatal initial treatment for PA/PS and to predict the final status of the fetus. Prenatal detection and prediction of the future status is helpful for family counseling. Furthermore, it will help to decide the postnatal management prenatally. (Circ J 2008; 72: 1471 - 1475).     

Efficacy of the circulatory management of an antenatally diagnosed congenital diaphragmatic hernia: outcomes of the proposed strategy

Object

The purpose of this study is to evaluate the outcome of our therapeutic strategy for antenatally diagnosed congenital diaphragmatic hernia (ADCDH).

Methods

We treated 61 cases of ADCDH according to our strategy. Prostaglandin E1 was required to be maintained the patency of the ductus arteriosus (PDA) in 39 cases (Group I) while it was not administered in 22 cases (Group II). Left ventricular end-diastolic dimension (LVDD) and Tei index were measured with echocardiography on days 0, 2, and 7 after birth. Radical surgery was performed on all cases by day 2.

Results

On day 0, Group I showed smaller LVDD and Tei index than those in Group II. Between day 0 and day 2, these parameters increased significantly in Group I, but not in Group II. On day 7, no significant difference in these parameters was observed between the two groups. Five patients died of cardiac and respiratory failure, resulting in a survival rate of 92 %.

Conclusion

Our therapeutic strategy improves the clinical outcome of ADCDH. This can be attributed to two factors: earlier surgery resulting in improved LV function. The latter attenuates pulmonary hypertension and maintains PDA with a consequent decrease in right ventricular afterload to compensate for the low cardiac output resulting from PDA.     

Severe Hepatic Injury Caused by Imatinib Mesylate Administered for the Treatment of Chronic Myeloid Leukemia and the Efficacy of Prednisolone for its Management

Imatinib mesylate is a specific inhibitor of BCR-ABL tyrosine kinase, which is now widely used for the treatment of chronic myeloid leukemia (CML) with a high efficacy. Although severe hepatic injury caused by imatinib mesylate is rare, such a side effect may force patients to discontinue taking imatinib mesylate. In the present paper, we report on the case of a 51-year-old woman with CML who experienced hepatic injury with severe hyperbilirubinemia caused by imatinib mesylate. The findings from a liver biopsy specimen and her clinical course suggested the hepatic injury to presumably have been caused by an allergic mechanism. The co-administration of prednisolone was thus tried, and she has been able to continue imatinib mesylate administration without any liver dysfunction and finally was able to obtain a complete cytogenetic response.We therefore recommend that prednisolone should be tried when severe hepatic injury caused by imatinib mesylate is observed, since it might enable such patients to continue imatinib mesylate treatment and thereby improve the prognosis in such cases.     

Acquired hemophilia A associated with Epstein–Barr-virus-associated T/natural killer-cell lymphoproliferative disease: A case report

Introduction:Acquired hemophilia A (AHA) is a rare bleeding disorder caused by autoantibodies against factor VIII (FVIII). Hematological malignancies, especially lymphoid malignancies, are known to be underlying causes of AHA; however, thus far, there is no report of AHA associated with Epstein–Barr-virus-associated T/natural killer-cell lymphoproliferative disease (EBV-T/NK-LPD). Here, we present a case of AHA that developed during treatment for EBV-T/NK-LPD.History:A 69-year-old man visited our hospital because of general fatigue. Blood examination showed pancytopenia, and computed tomography revealed whole-body lymphadenopathy, but there were no findings indicating hematological malignancy from bone marrow aspiration and cervical lymph node biopsy. The level of EBV DNA in peripheral blood was extremely high, and he was diagnosed with EBV-T/NK-LPD. EBV-T/NK-LPD improved with prednisolone (PSL) administration. Seventeen months after starting treatment, the patient complained of back and right leg pain. At that time, he had been treated with low-dose PSL, and EBV-T/NK-LPD was well controlled. Imaging revealed hematoma of the right iliopsoas muscle. Prolonged activated partial thromboplastin time (APTT) was the only abnormal finding in a screening coagulation test. FVIII coagulant activity was below detection limit, and FVIII inhibitor level was increased. From these results, he was diagnosed with AHA.A higher dose of PSL was administered, and, after 1 month of treatment, FVIII activity gradually increased, and FVIII inhibitor level became undetectable. APTT also normalized, and complete remission was achieved and maintained for 13 months with low-dose PSL. During treatment, EBV-T/NK-LPD was well controlled.Conclusion:It is speculated that proliferating lymphocytes interfere with normal immune functions and that abnormal autoantibodies are produced from those lymphocytes in patients with LPD. Therefore, we speculate that EBV-infected and proliferating monoclonal NK cells might have modulated the immune system and produced autoantibodies against FVIII, thus causing AHA in this patient with EBV-T/NK-LPD.     

A lesion diagnosed by MRI in a case of transient global amnesia]

Transient global amnesia(TGA) had been recognized as a disease without abnormal findings on neuroimaging before magnetic resonance(MRI) imaging was practicable. Recently, abnormal findings on MRI reported in cases of TGA. We here reported a case of TGA showing a transient abnormal intensity in left hippocampus on MRI diffusion-weighted image. A 52-year-old man suddenly became to unable to keep his recent memory without histories of trauma or epilepsy. He showed no abnormal neurologic findings excepting for the recent memory. MRI performed within an hour after onset demonstrated no abnormality on T1- and T2-weighted images. Diffusion-weighted image (b = 1,000) showed hyperintensity in the left medial temporal lobe, and the apparent diffusion coefficient(ADC) in this region was lower(72.8 cm2/s) than that in the contralateral region(94.4 cm2/s). TGA was completely resolved 17 hours after onset.     

Case of intraspinal epidural tumor developing after systemic mastocytosis with marked osteosclerosis and myelofibrosis

A 64-year-old man was diagnosed as having urticaria pigmentosa in 1998, and treated with PUVA therapy. In January 2002, X-ray imaging revealed osteosclerosis was detected in the systemic bone and bone scintigraphy. A bone marrow aspiration sample was not obtained due to a dry tap. CT scans showed hepatosplenomegaly and mesenteric lymphadenopathy. Myelofibrosis and diffuse mast cell infiltration were revealed by a bone marrow biopsy, and a diagnosis of systemic mastocytosis with severe osteosclerosis and myelofibrosis was made. In October 2003, he was admitted to our hospital because of mid back pain. A neurological examination showed muscle weakness in the upper and lower limbs, sensory disturbance below the level of Th4 and urinary obstruction. T1 and T2 weighted images of MRI demonstrated a high intensity epidural mass lesion extending from the vertebral level of C5 to Th2 and severely compressing the spinal cord. We considered the possibility of the invasion of the spinal canal by the mastocytosis. The patient was treated with interferon alpha-2b (IFN-alpha2b) and prednisolone. Subsequently, the motor and sensory disturbances were gradually alleviated, and spinal MRI confirmed a marked reduction in the size of the epidural tumor. However, the patient became resistant to interferon, and died of multiple organ failure in spite of steroid pulse and cladribine therapies. Multiple organ infiltration by mast cells was revealed at autopsy.