Previously diagnosed cases of hepatosinusoidal T-cell lymphoma and malignant histiocytosis (MH) may include lymphoid neoplasms of natural killer (NK) cell lineage associated with Epstein-Barr virus (EBV). Such hepatosinusoidal neoplasms were found to demonstrate hepatomegaly but not lymphadenopathy, and all were diagnosed by a liver biopsy. Sixteen adult patients diagnosed with hepatosinusoidal leukaemia/lymphoma (six NK-cell leukaemia/lymphomas [NKLLs], five instances of MH, three T-cell malignant lymphomas [T-MLs], and two adult T-cell leukaemia/lymphomas [ATLLs] were examined for EBV by in situ hybridization, then were studied immunohistochemically and subjected to a DNA analysis. Among our five patients with MH, neoplastic cells showed T-cells, but no histiocytic markers, and they were considered to have either a T-cell or NK-cell lineage. All NKLLs, MHs and T-MLs, except for ATLLs accompanied by reactive hemophagocytic histiocytes, varied in number in each case. In situ hybridization revealed the presence of EBV in the nuclei of atypical cells in all of the six lymphoid neoplasms of NK-cell lineage. Each case of MH and each T-ML which represented EBV demonstrated no definite T-cell or histiocytic markers. Patients with ATLL did not reveal EBV. In all patients with hemophagocytosis, EBV was present in the nuclei of the neoplastic lymphocytes, but not in the hemophagocytic cells. Finally, the 16 cases were reclassified into eight cases with EBV-containing NKLLs, six T-MLs, and two ATLLs. In addition, no true histiocytic neoplasms were observed. The mechanism of hemophagocytosis may be therefore the production of lymphokines (macrophage-activating factors) by neoplastic lymphocytes. EBV-associated hepatosinusoidal leukaemia/lymphoma may thus contain a lymphoid neoplasm of NK-cell lineage, which made it difficult to be distinguished from the previously designated malignant histiocytosis.

Adult↗

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Japanese psychiatry.

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Ethics, Medical↗

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State of Japanese psychiatry.

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Ethics, Medical↗

PubMedDate not supplied

Treatment of disseminated herpes zoster in six severely immunocompromised patients: acyclovir and vidarabine.

We treated 6 patients with disseminated herpes zoster (D-H-Z) and in a severely immunocompromised condition. Three were effectively treated with 15 mg/kg/day of acyclovir. Ten mg/kg/day of vidarabine alone was given to two patients, but without positive effects. In two patients, acyclovir was effective in limiting the progression of the lesions. The skin lesions looked like a "stitch" on vesicles, thereby suggesting that the "stitch like appearance" may be a useful marker to identify the resistance to acyclovir treatment. In one patient, acyclovir was given for 58 days, but with limited effectiveness. When vidarabine was given in addition to acyclovir, the lesions dramatically disappeared. These findings suggest that acyclovir is superior to vidarabine for treating D-H-Z and the combination therapy with acyclovir and vidarabine should be considered for prescription for patients with D-H-Z, if acyclovir alone is not completely effective.

Acyclovir↗

PubMedDate not supplied

A case of Japanese cleidocranial dysplasia with a CBFA1 frameshift mutation.

Cleidocranial dysplasia (CCD), which is caused by mutations of the core binding factor alpha 1 (CBFA1)/runt-related gene 2 (Runx2), is an autosomal, dominantly inherited disorder of high penetrance affecting skeletal ossification and tooth development. Recently, we found a novel frameshift mutation 383-T-insertion (S128F) in exon 3 in the CBFA1 gene of a Japanese classic CCD patient. We describe our detailed investigation of the patient with CCD associated with the CBFA1 mutation. The patient showed the characteristic expression of CCD, such as dysplasia of the clavicles, patent fontanelles, short stature, impacted supernumerary teeth, and delayed eruption of the permanent teeth. In addition to these characteristics, orthopantomography delayed ossification of the mandibular symphysis and a three-dimensional computed tomograph (3D-CT) analysis showed hypoplasia of the zygomatic arch. Furthermore, the acellular cementum of an impacted supernumerary tooth was absent in this patient. Thus, the CBFA1 mutation was critical for the pathogenesis of CCD in this patient.

Amino Acid Sequence↗

PubMedDate not supplied

Influences of sodium on the contractile action of vanadate in depolarized vas deferens and ureter of the guinea-pig.

Effects of extracellular Na+ on the contractile action of vanadate were studied using depolarized smooth muscles of the guinea-pig vas deferens and ureter. Both sodium vanadate and ammonium vanadate induced tension development of the preparations in K-Tyrode's solution containing 12.3 mM Na+. The tension developments were enhanced greatly in K-60 mM solution with 89.3 mM Na+ and diminished in Na+-free K-Tyrode's or Na+-free K-60 mM Tyrode's solution, and slight relaxations were observed in the vas deferens soaked in the latter two Na+-free solutions. The addition of Na+ to Na+-free solution in the presence of vanadate induced a marked increase in tension. Vanadate induced slight tension development of depolarized preparations in the absence of Ca which was less affected by Na+. Ca-induced contraction of depolarized preparations was also markedly potentiated by vanadate. The potentiation was more prominent in the presence of Na+ and less prominent in the absence of Na+; the maximum tension was less affected by Na+ than the vanadate-induced contraction of depolarized preparation, but the tension decay was greatly accelerated in the absence of Na+. It was suggested that Na+ enhances the contractile action of vanadate through Ca extrusion mechanism of cell membrane.

Animals↗

PubMedDate not supplied

[Contrast echocardiographic detection of pulmonary arteriovenous shunt in a hypoxemic patient with liver cirrhosis].

A 62-year-old man with liver cirrhosis and hypoxemia was admitted to evaluate the etiology of hypoxemia. The patient had noticed exertional dyspnea for three years. Physical examination, laboratory tests, ultrasonography and liver scintigraphy revealed liver cirrhosis. The arterial blood gas test showed hypoxemia (PO2 46.3 mmHg). Chest roentgenogram showed old pulmonary tuberculosis and the pulmonary function test demonstrated decreased FEV 1.0% and %DLco, which did not account for the marked hypoxemia. Contrast echocardiography was performed by injecting hand-agitated saline into the antecubital vein. Three seconds after the right ventricle was opacified, the contrast echoes appeared in the left atrium and then the left ventricle. Pulmonary arteriography revealed no pulmonary arteriovenous fistula. Hemodynamic data showed low pulmonary vascular resistance. Contrast echocardiography by injecting hand-agitated saline from the catheter tip was performed at both pulmonary arteries and the left atrium was opacified by each injection. The hypoxemia was mainly attributed to a intrapulmonary arteriovenous shunt. Although the etiology of hypoxemia in liver cirrhosis seems to be multifactorial, the intrapulmonary arteriovenous shunt is the most important factor. Contrast echocardiography was useful for detecting this shunt.

Arteriovenous Fistula↗

PubMedDate not supplied

[Changes in gadolinium-DTPA enhanced magnetic resonance signal intensity ratio in hypertrophic cardiomyopathy].

Serial gadolinium-diethylene-triamine-pentaacetic acid (Gd-DTPA) enhanced magnetic resonance (MR) signal intensity ratios were measured in 6 normal subjects and 20 hypertrophic cardiomyopathy (HCM) patients to try to differentiate normal from disorganized myocardial tissue. Images were obtained at 10-minute intervals 5-60 minutes after Gd-DTPA (0.1 mmol/kg) injection. The signal intensity ratio (myocardial signal intensity/skeletal muscle signal intensity) was measured at both hypertropic and nonhypertrophic regions in each image at the apex and mid-ventricular levels. The signal intensity ratio was standardized to compare each case. Hypertrophic myocardium was classified into two types. Type I in 11 of 20 patients was visualized as a homogeneous image, while type II in the other 9 patients was revealed as a mixed isointensity and high intensity area. The peak value of the standardized signal intensity ratio at the apex level was 1.28 +/- 0.09 in HCM patients and 1.23 +/- 0.06 in normal subjects, and at the mid ventricular level was 1.26 +/- 0.07 in hypertrophic regions, 1.17 +/- 0.12 in non-hypertrophic regions, and 1.16 +/- 0.07 in normal subjects. Thirty minutes after Gd injection, the standardized signal intensity ratio at the apex level was 1.21 +/- 0.08 in HCM patients and 1.07 +/- 0.08 in normal subjects, and those at the mid ventricular level was 1.20 +/- 0.09 in hypertrophic regions, 1.11 +/- 0.11 in non-hypertrophic regions, and 1.04 +/- 0.06 in normal subjects. The delayed decay of the signal intensity ratio and high signal intensity ratio in Gd-DTPA enhanced MR images are useful in myocardial tissue characterization in hypertrophic cardiomyopathy.

Cardiomyopathy, Hypertrophic↗