Multiple minute carcinoids in type A gastritis: attempt at 3-D reconstruction

In type A gastritis, the numbers of endocrine cell micronests (ECM) and carcinoids increase through the trophic action of gastrin. This study examined the characteristics and growth of carcinoids in type A gastritis. A total of 395 lesions in five surgically removed stomachs with type A gastritis were investigated, in terms of number, size, distribution and histological appearance, to clarify the tumorigenesis and progression of carcinoids. 3-D reconstruction using serial paraffin sections was used to study carcinoid progression. Our findings suggest that in type A gastritis, carcinoids arise in areas where minute carcinoids are present at a high density. They also suggest that early stage carcinoids not only become large expansively, but also develop in a very complex manner, by maintaining contact with surrounding minute carcinoids.     

Left ventricular assist system with a magnetically levitated impeller technology

After the accumulation of clinical experience with the current generation of pulsatile implantable left ventricular assist systems (LVAS), these devices have demonstrated major limitations: high incidence of thromboembolic complications; large size; high infection rate; and limited long-term durability. To address the limitations of current-generation LVAS, second- and third-generation LVAS utilizing rotary blood pump technology are currently undergoing clinical trials and the final stage of product development. Among them, the rotary blood pump with a magnetically levitated impeller is one of the most promising pumps for long-term circulatory assist. The Terumo DuraHeart LVAS is one of the third-generation LVAS using a centrifugal pump with a magnetically levitated impeller. This article describes the characteristics of the DuraHeart LVAS and its development status.     

A Case of Adult-onset Pompe Disease with Cerebral Stroke and Left Ventricular Hypertrophy

Background

Pompe disease is an autosomal recessive glycogen storage disorder caused by a deficiency of the lysosomal glycogen-hydrolyzing enzyme acid α-glucosidase. The adult-onset form, late-onset Pompe disease, has been characterized by glycogen accumulation, primarily in skeletal and smooth muscles, causing weakness of the proximal limb girdle and respiratory compromises.

Ultrasonographic diaphragm thickness correlates with compound muscle action potential amplitude and forced vital capacity

Introduction: Noninvasive evaluation of respiratory function in patients with various neuromuscular disorders is important for predicting life expectancy. Methods: We performed B‐mode ultrasonography (USG) and nerve conduction studies in 37 patients (16 had amyotrophic lateral sclerosis; 11, myopathy; and 10, neuropathy) and 10 controls. USG of the diaphragm was performed in the supine position using a linear probe over the intercostal space at the anterior axillary line. Diaphragm muscle thickness was measured at end‐expiration. The amplitude of diaphragm compound muscle action potentials (CMAP) was obtained by phrenic nerve stimulation with a surface electrode. Respiratory function was measured with standard pulmonary function tests including forced vital capacity (FVC). Results: Diaphragm thickness was significantly correlated with FVC (r = 0.74) and CMAP amplitude (r = 0.53). Conclusions: Diaphragm USG is useful for objective evaluation of pulmonary function in neuromuscular disorders without requiring undue patient effort or cooperation. Muscle Nerve 53 : 522–527, 2016     

Human herpesvirus 6 encephalopathy predominantly affecting the frontal lobes

Isolated cases of human herpesvirus 6 encephalopathy have recently been reported, although the pathophysiology remains largely unknown. To elucidate the changes specific to human herpesvirus 6 encephalopathy on diagnostic images, this study investigated magnetic resonance imaging findings in 10 patients with a diagnosis of human herpesvirus 6 encephalopathy including diffusion-weighted imaging in 6 of 10, and findings of cerebral blood flow imaging by single-photon emission computed tomography in 9 of 10 patients. No abnormalities were evident on T(1)-weighted, T(2)-weighted, or fluid-attenuated inversion-recovery magnetic resonance imaging during acute phases; however, diffusion-weighted imaging indicated abnormal hyperintensity in the subcortical white matter of the frontal lobes in all six patients during the acute phase. Cerebral blood flow single-photon emission computed tomography revealed decreased perfusion, predominantly in the frontal region of all nine patients during their clinical course. Disturbances predominantly affecting the frontal lobes (region) on magnetic resonance imaging and cerebral blood flow single-photon emission computed tomography were common in all patients, suggesting that the findings may be characteristic of human herpesvirus 6 encephalopathy.     

Prenatal diagnosis of persistent cloaca

We report four cases of persistent cloaca diagnosed at 32–33 weeks of gestation. In cases of persistent cloaca, serial prenatal ultrasonography shows transient fetal ascites, enlarged cystic structures arising from the fetal pelvis. Our four cases of persistent cloaca were diagnosed prenatally. Persistent cloaca should be considered in any female fetus presenting with hydronephrosis and a large cystic lesion arising from the pelvis as assessed by ultrasound and magnetic resonance imaging. Neither pulmonary hypoplasia nor severe oligohydramnios were found in any of our four cases, and they each had a good prognosis. Prenatal diagnosis allows time for parental counseling and delivery planning at a tertiary care center for neonatal intensive care and pediatric surgery.     

Predominance of genotype A HBV in an HBV-HIV-1 dually positive population compared with an HIV-1-negative counterpart in Japan

Hepatitis B virus (HBV) has seven genotypes, A to G. Previous studies have shown that genotype C is the most prevalent strain in chronic HBV carriers in East Asia. This study was undertaken to investigate the epidemiology of HBV genotypes among Japanese patients who are coinfected with human immunodeficiency virus type 1 (HIV-1). The sequences of the complete hepatitis B surface antifen (HBsAg) genes were obtained from 18 coinfected Japanese patients. Among the 18 patients, 12 of 13 men who had sex with men (MSM) had genotype A (92%), whereas only one of five heterosexual or hemophiliac patients had genotype A. The predominance of genotype A HBV in MSM showed a striking contrast to the current genotype prevalence in the Japanese population. Owing to the recent decrease in the rate of vertical transmission in Japan, the role of sexual behavior in the transmission of HBV cannot be overestimated. Thus, the relative proportion of genotype A may gradually increase in Japan.     

Impairment of Sulfatide, Acid Mucopolysaccharides and Cholesterol Sulfate Degradation in Cultured Skin Fibroblasts of Patients with Multiple Sulfatase Deficiency (Mukosulfatidosis)

A new case of Japanese patient with Multiple Sulfatase Deficiency (MSD) was subjected for the study of metabolism of various sulfated compounds in vivo using cultured skin fibroblasts. Several sulfatase activities (arylsulfatase A, B and C, cholesterol sulfatase) were deficient in MSD cultured fibroblasts under F-10-CO₂ medium. Incorporation and degradation of ³⁵S-sulfatide, ³⁵S-mucopolysaccharides and ¹⁴C-cholesterol sulfate by MSD cells were also studied, comparing to those of control, Hunter and Metachromatic leukodytrophy's cells. MSD fibroblasts accumulated and failed to degrade these compounds in vivo. Cholesterol sulfate also incorporated into control and pathological cells, and MSD cells were not able to hydrolyze cholesterol sulfate, though cholesterol sulfate is known to be hydrolyzed in non-lysosomal subfraction. These data are consistent with the foct that multiple enzyme deficiencies in MSD fibroblasts were also demonstrated in vivo.     

In vitro and ex vivo platelet interactions with hydrophilic-hydrophobic poly(ethylene oxide)-polystyrene multiblock copolymers

Hydrophilic-hydrophobic multiblock copolymers synthesized from telechelic oligomers of poly(ethylene oxide) (PEO) and polystyrene (PS) have been used to study the influence of hydrophilic and hydrophobic balance on interfacial interactions of these surfaces with blood components. In vitro coagulation assays show no inherent ability of these amphiphilic surfaces to affect contact activation or coagulation factors. In vitro platelet adhesion and release reactions from rabbit platelet-rich plasma were shown to be greatest on Biomer and PS homopolymer surfaces and least on cross-linked PEO surfaces, with the PEO-PS block copolymers demonstrating intermediate responses. These same substrates were tested in a new low-flow, low-shear arterio-artery shunt system in rabbits. Whole blood occlusion times were not a direct function of hydrophilic content as both PEO and PS homopolymers and Biomer showed short occlusion times, while PEO-PS block copolymers prolonged occlusion times considerably, depending on composition. Overall, results suggest that PEO-PS block copolymers promote unique whole blood responses in contrast to homopolymer and Biomer controls which are more complex than direct correlations to bulk hydrophilic and hydrophobic contents.