■神经节减少症与两种疾病类似,患者可能出现严重便秘或假性梗阻。迄今为止,由于在诊断标准方面缺乏国际共识,目前仍很难对神经节减少症进行诊断.本研究旨在评估免疫组织化学的使用,为我们对神经节减少症的最初主观印象提供客观支持,并描述本研究的形态特征。
■这是一项横断面研究。九州大学医院切除了三例节节病患者的肠道样本,福冈,日本被纳入本研究。一个健康的肠样品用作对照。所有标本用抗S-100蛋白进行免疫组织化学染色,抗α-平滑肌肌动蛋白(α-SMA),和抗c-kit蛋白抗体.
■(I)S-100免疫染色:在肠的几个节段中观察到肌间神经节发育不全和肌内神经纤维明显减少。(II)α-SMA免疫染色:所有节段的肌肉层模式几乎正常;然而,某些区域显示圆形肌肉(CM)层肥大和纵向肌肉(LM)层肥大。(III)C-kit免疫染色:在几乎所有切除的肠段中观察到Cajal间质细胞(ICCs)的数量减少,甚至在肌间神经丛周围.
■神经节减少症的每个肠段都有不同数量的ICC,尺寸,和神经节的分布,以及肌肉组织的模式,可能从严重异常到接近正常。关于定义的进一步调查,病因学,诊断,应进行这种疾病的治疗,以改善这种疾病的预后。
UNASSIGNED: Hypoganglionosis resembles Hirschsprung\'s disease as in both diseases, patients may present with severe constipation or pseudo-obstruction. To date, diagnosis of
hypoganglionosis is still difficult to be established due to lack of international consensus regarding diagnostic criteria. This study aims to evaluate the use of immunohistochemistry to provide objective support for our initial subjective impression of
hypoganglionosis as well as to describe the morphological features of this study.
UNASSIGNED: This is a cross-sectional study. Three resected intestinal samples from patients with
hypoganglionosis at Kyushu University Hospital, Fukuoka, Japan were included in this study. One healthy intestinal sample was used as control. All specimens were immunohistochemically stained with anti-S-100 protein, anti-α-smooth muscle actin (α-SMA), and anti-c-kit protein antibodies.
UNASSIGNED: (I) S-100 immunostaining: hypoplasia of the myenteric ganglia and marked reduction of intramuscular nerve fibers were observed in several segments of the intestine. (II) α-SMA immunostaining: the pattern of the muscular layers was almost normal in all segments; however, some areas showed hypotrophy of the circular muscle (CM) layers and hypertrophy of the longitudinal muscle (LM) layers. (III) C-kit immunostaining: a decreased in the number of interstitial cells of Cajal (ICCs) was observed in almost all segments of the resected intestine, even around the myenteric plexus.
UNASSIGNED: Each segment of intestine in
hypoganglionosis had different numbers of ICCs, sizes, and distributions of ganglions, as well as patterns of musculature, which may range from severely abnormal to nearly normal. Further investigations regarding the definition, etiology, diagnosis, and treatment of this disease should be performed to improve the prognosis of this disease.