Transient low level of IgG3 induced by sepsis

Staphylococcus aureus sepsis developed in a 14 year old girl. Immunological evaluation revealed low level of IgG3, although total IgG level was normal. The level of IgG3 increased gradually along with the recovery from sepsis. Immunoglobulin replacement therapy might have been useful in this patient, even though the total immunoglobulin level was within normal limits.     

Diffuse fibromatosis on the scalp in infancy: A variant of juvenile hyaline fibromatosis

Various types of fibromatosis have been reported in infancy and early childhood. We describe an infant with diffuse fibromatosis on the scalp. A one year and five months-old girl showed a diffuse and hard mass 3 × 5 cm in diameter and no tenderness on the scalp. Two months later, the size of the mass had increased and several other tumors appeared on the lateral head. The magnetic resonance imaging (MRI) disclosed that a large and diffuse tumor had spread from the frontal to occipital head; a ‘helmet-like’ configuration of the tumor was exhibited on sagittal MR images. The tumor showed high signal intensity on T2-weighted images and was enhanced with Gd-DTPA. Histological examination showed a fibroblastic proliferation with intervening thick collagen bundles. The patient was diagnosed as having diffuse fibromatosis. The tumor at the resection site immediately recurred, whereas the tumor in the frontal head showed marked regression. Three months after the resection, new tumors appeared in the occipital head. The size and number of these tumors have remained unchanged for more than 18 months. The sites and appearance of the tumors were identical to that of juvenile hyaline fibromatosis (JHF) in this patient. However, JHF usually includes fibroblasts associated with large amounts of hyalinized collagen-like material, which were not present in our patient. The different histology of JHF comparing our case and other reported cases may depend on the different phase of the disease progression at resection. Long-term observation is necessary for the appropriate diagnosis and evaluation of prognosis in this patient.     

Lennox-Gastaut syndrome: A new vista

Abstract Lennox-Gastaut syndrome (LGS) is regarded as a model of the epileptic syndrome because of its specific clinicoelectrical manifestation. However, a close investigation reveals that its outline is somewhat vague, having the borderland around it. Precise diagnosis in an individual case is not always easy. In this paper, the diagnostic criteria of LGS are described. According to these criteria, cases with LGS were subclassified into the typical and the atypical cases, and also cases in the borderland of LGS were reviewed. On the other hand, our prospective long-term follow-up study revealed that cortical mechanisms played an important role in the pathophysiology, clinical features and refractoriness of LGS. Secondary bilateral synchrony (SBS) is supposed to be a mode of expression of cortical mechanisms of LGS. A newly developed method with coherence and phase analysis demonstrated that the pathophysiology was based on SBS in 33% of the typical LGS cases. This finding is not only crucial for the choice of rational treatment including epilepsy surgery, such as callosotomy, but also contributes to a more refined subclassification of LGS.     

Moyamoya disease associated with bilateral renal artery stenosis

Recent research has suggested that an association exists between moyamoya disease and fibromuscular dysplasia which involves systemic vessels, including renal arteries. We report a 3 year old girl with moyamoya disease associated with bilateral renal artery stenosis. This case may support the common etiology of these two clinical conditions. To our knowledge this is the youngest case of moyamoya disease associated with bilateral renal artery stenosis.     

Galanin-like immunoreactive endocrine cells in bovine pancreas

Pancreata of fetal, neonatal and adult cattle were studied immunohistochemically for galanin. The results revealed galanin-like immunoreactivity both in the endocrine cells and in the neural elements. The galanin-like immunoreactive endocrine cells (Gal-LIEC) were confined to the large islets, and were not observed in the islets of Langerhans and exocrine pancreas. They were first detected at the third prenatal month. Their developmental profile showed an increase from fetal to early neonatal stage with a subsequent decrease towards adulthood. The considerable number of Gal-LIEC from late prepartum to early postpartum stage may imply functional significance of galanin during the perinatal development of cattle. Coexistence of galanin and insulin was also observed which may suggest autocrine interaction between the 2 hormones.     

线分法与行为偏侧忽略的相关性研究

目的 研究线分法与行为偏侧忽略的相关性，探讨线分法能否预测行为偏侧忽略。方法 根据行为偏侧忽略的评估量表——凯瑟林一波哥量表将30例脑卒中致左侧偏瘫的患者分为4组：严重偏侧忽略组，中度偏侧忽略组，轻度偏侧忽略组和无偏侧忽略组。11例年龄匹配的健康成人作为对照组。进行线分法检查时，在A4纸的左侧、中央和右侧分别画6条水平线段，让患者用右手持圆珠笔在线的中央划一垂直分隔线。计算分隔线右侧长度占该线段全长的百分比。结果 除轻度偏侧忽略组外，被分割线段空间的位置对其它各组人员的线分结果有明显影响。严重偏侧忽略组、无偏侧忽略组、对照组出现线分法中的位置“反转效应（crossovereffect）”。严重偏侧忽略组的患者在分隔A4纸左侧及中央的线段时出现向右的偏差，而在分隔A4纸右侧的线段时出现向左的偏差。在无偏侧忽略组及对照组，被检人员在分隔A4纸左侧线段时出现向左的偏差，而在分隔中央及右侧线段时出现向右的偏差。结论 本研究表明线分法中的位置“反转效应”若出现在位于患者右侧的线段，则强烈提示患者有重度行为偏侧忽略。     

STATISTICAL DEFINITION OF NAILFOLD CAPILLARY PATTERN IN PATIENTS WITH PSORIASIS

Background. The microvasculature plays an important role in the pathogenesis of psoriatic skin lesions. Our purpose was to try to define a psoriatic pattern in the nailfold capillary, to clarify the relationship between nailfold capillary microscopic changes and nail involvement and to note the general clinical features of psoriasis. Methods. Image analysis of nailfold capillaries was performed in 62 patients with psoriasis. The capillary pattern was defined statistically comparing it with that of 51 healthy volunteers. We attempted to differentiate the psoriatic pattern from normal controls with “canonical discriminant analysis.” Results. Forty-nine of 62 patients with psoriasis could be differentiated from normal controls by our definition of psoriatic pattern that was significantly correlated with periungual psoriatic plaque, nail pitting, onycholysis, and the extent of the involved area. Conclusions. Our data suggest that nailfold capillary changes reflect microvascular changes of psoriasis and that the nailfold capillary pattern is a useful tool in evaluating nail involvement and the severity of psoriasis.     

Frequency Dependent Shortening of Conduction Time through the Reentrant Pathway during Transient Entrainment of Ventricular Tachycardia

In a patient with nonischemic ventricular tachycardia (VT), VT was entrained and the conduction time from the pacing site to the entrained local electrogram showed a rate dependent shortening and its degree affected by the pacing site. The QRS complex, which was entrained by the last pacing stimulus, was constant and identical to that of VT and no rate dependent facilitated conduction was observed when the heart was paced at similar paced cycle lengths during sinus rhythm. As the mechanism of the shortening of the conduction time through the reentrant circuit, a shift of the entrance seems most likely.     

The regulation of intestinal hypersensitivity reactions to ovalbumin by ω-3 fatty acid enriched diet: Studies of IEL and LPL in mucosal damage

In order to clarify the mechanisms of food-sensitive enteropathy, a food hypersensitive model was generated by feeding ovalbumin to female BALB/c mice after intraperitoneal injection of cyclophosphamide and morphological and immunological changes in the gut mucosa were investigated. Villus atrophy, crypt hyperplasia and increased numbers of intra-epithelial lymphocytes (IEL) were confirmed in this model, as seen in food-sensitive enteropathy in humans. Subpopulations of IEL and lamina propria lymphocytes were enumerated by immunohistochemical observation. CD8-positive cells were increased both in epithelium and lamina propria, whereas CD4-positive cells were decreased in lamina propria. We document here that orally administered food antigen actually induces food-sensitive enteropathy and mucosal damage is generated by lymphocytes that infiltrate the intestinal mucosa. We also investigated the effect of feeding an ω-3 fatty acid-enriched diet in this model and found that it was efficient in attenuating mucosal damage.     

Attenuating effect of castration or oestrogen administration on glomerular injury in adriamycin-induced nephropathy of rats

Summary: To clarify the role of sex-related factors in the development of focal segmental glomerulosclerosis (FSGS) we investigated the effect of castration or oestrogen administration in adriamycin (ADR)-induced nephropathy of Sprague-Dawley rats. At 6 weeks of age, group 1 (control group) and group 3 were shamoperated and group 2 was castrated. Adriamycin 2 mg/kg was administered intravenously to all rats at 8 weeks of age twice at a 20-day interval. Group 3 was administered 0.2 mg oestrogen subcutaneously once a month. Bodyweight (BW) and urinary protein were measured every 4 weeks (mm 15 to 23 weeks of age. Blood pressure and serum constituents were measured at 15 and 23 weeks of age. Each group was studied morphologically at 23 weeks of age. Adriamycin induced massive proteinuria in group 1, whereas castration or oestrogen significantly attenuated proteinuria, accompanied by a significant reduction of urinary sex-related low molecular weight (LMW) protein. the glomerulosclerosis index was significantly higher in the control group than in the castrated group or oestrogen-treated group. Attenuation in glomerular injury was more associated with reduction of urinary sex-related LMW protein than the reduction of serum testosterone. These observations suggest that sex-related factors, such as a sex-related LMW protein, influenced by castration or oestrogen administration may play a contributory role in the development of glomerulosclerosis in ADR-induced nephropathy.