PDF(Pubmed)
Abstract：
The Interstitial Cells of Cajal (ICC) are responsible for rhythmic electrical activity. A paralytic ileus is present in gastroschisis (GS), a malformation due to a defective closure of the abdominal wall through which part of the intestine herniates during pregnancy. In experimental GS, ICC morphological immaturity was shown in the rat foetus at-term but it could not be demonstrated whether differentiation is accomplished post-natally. For this purpose we morphologically investigated ICC, as well as enteric neurons and smooth muscle cells, in a case of human GS at birth and 1 month later when peristaltic activity had initiated. A 36 weeks gestation female was born by c/section with prenatal diagnosis of GS and possible volvulus of the herniated intestine. At birth, the necrotic intestine was resected and both ileostomy and colostomy were performed. The intestine continuity was restored after 4 weeks. Intestinal specimens, taken during both operations at the level of the proximal stoma, were immunostained with c-kit, neuron-specific-enolase and alpha-smooth-muscle-actin antibodies and some processed for electron microscopy. ICC were present at the myenteric plexus only. At birth, these cells were rare and ultrastructurally immature; 1 month later, when partial enteral feeding was tolerated, they formed rows or groups and many of them were ultrastructurally differentiated. Neurons and smooth muscle cells, immature at birth, had developed after 1 month. Therefore, ICC differentiation, as well as that of neurons and smooth muscle cells, is delayed at birth and this might explain the paralytic ileus in GS. One month later, differentiation quickly proceeded at all cellular levels paralleling the increasing tolerance of enteral nutrition.
摘要：
Cajal间质细胞(ICC)负责节律性电活动。腹裂（GS）中存在麻痹性肠梗阻，由于腹壁闭合有缺陷而导致的畸形，在怀孕期间部分肠道突出。在实验GS中，在足月的大鼠胎儿中显示ICC形态不成熟，但无法证明分化是否在出生后完成。为此，我们对ICC进行了形态学研究，以及肠神经元和平滑肌细胞，在出生时和1个月后蠕动活动开始时的人类GS病例中。一名妊娠36周的女性通过剖腹产出生，产前诊断为GS，可能是疝肠扭转。出生时,切除坏死的肠，并进行回肠造口术和结肠造口术。4周后恢复肠连续性。肠标本,在近端造口水平的两次手术中，用c-kit免疫染色,神经元特异性烯醇化酶和α-平滑肌肌动蛋白抗体，一些经过电子显微镜处理。ICC仅存在于肌间神经丛。出生时,这些细胞是罕见的和超微结构上不成熟的;1个月后,当部分肠内喂养耐受时，它们形成行或组，其中许多是超微结构分化的。神经元和平滑肌细胞，出生时不成熟,在1个月后发展。因此,ICC差异化，以及神经元和平滑肌细胞，出生时延迟，这可能解释了GS的麻痹性肠梗阻。一个月后,随着肠内营养耐受性的增加，分化在所有细胞水平上迅速进行。
