Itoh K, Itoh H
Department of Pediatrics, Takatsuki General Hospital, Osaka, Japan.
Pediatr Pathol. 1988;8(1):65-81. doi: 10.3109/15513818809022280.
To discriminate between different forms of pulmonary hypoplasia (PH), 24 hypoplastic lungs were studied for their development of bronchial cartilage plates and peripheral air spaces. In 6 lungs from premature infants with oligohydramnios, normal amounts of immature and irregularly shaped cartilages were distributed with mitoses concentrated toward the periphery of the bronchi. Pulmonary acini appeared markedly immature. In 5 lungs from infants with diaphragmatic hernia, large numbers of cartilage bars were clustered around the proximal bronchi, whose branching was much reduced. Peripheral air spaces were small but structurally mature. In Potter syndrome, small amounts of tiny, mature cartilages were observed irregularly around the proximal bronchi and poorly distributed into the peripheral bronchi. The acinar structure was very immature. In 4 anencephalic infants, a marked decrease in the volume of mature cartilage was present, with cartilage seen only around proximal bronchi; the acini were atelectatic and less well developed. It is suggested that the earlier the action of a teratogen, the greater the abnormality of bronchial branching, cartilage distribution, and later lung development.
为鉴别不同形式的肺发育不全（PH），对24个发育不全的肺进行了支气管软骨板和外周气腔发育情况的研究。在6例羊水过少的早产儿肺中，有正常数量的未成熟且形状不规则的软骨，有丝分裂集中在支气管周围，肺腺泡明显未成熟。在5例膈疝婴儿的肺中，大量软骨条聚集在近端支气管周围，支气管分支明显减少，外周气腔小但结构成熟。在波特综合征中，在近端支气管周围不规则地观察到少量微小、成熟的软骨，且很少分布到外周支气管，腺泡结构非常不成熟。在4例无脑儿中，成熟软骨体积明显减少，仅在近端支气管周围可见软骨，腺泡肺不张且发育较差。提示致畸剂作用越早，支气管分支、软骨分布及后期肺发育的异常就越严重。
