Abstract: BACKGROUND/AIM: In congenital diaphragmatic hernia (CDH), there is pulmonary neuroendocrine cell (PNEC) hyperplasia and Clara (nonendocrine) cell hypoplasia, the meaning of which remains unknown. In embryonic/fetal lung, an intricate cross talk between Notch pathway and basic helix-loop-helix transcription factors Mash1 and Hes1 determines the balance between endocrine and nonendocrine epithelial cell fate. Differences at the molecular level in pulmonary epithelial cell differentiation between control and CDH hypoplastic lungs were investigated. MATERIAL AND METHODS: The nitrofen-induced CDH rat model was used. At 15.5 days postconception (dpc), fetuses were assigned to 2 experimental groups: control and nitrofen (exposed to nitrofen, without CDH), whereas at 17.5, 19.5, and 21.5 dpc, fetuses were assigned to 3 experimental groups: control, nitrofen, and CDH (exposed to nitrofen, with CDH). The fetal lungs were processed for expression quantification of CC10, Hes1, Mash1, and Dll1 by real-time polymerase chain reaction. RESULTS: In control fetuses, expression of all studied genes increased with gestational age. In nitrofen-exposed fetal lungs, endocrine cell marker Mash1 was downregulated only at the earliest studied gestational age, whereas Dll1 expression levels were significantly increased in the CDH group at 19.5 and 21.5 dpc. Regarding nonendocrine markers, Hes1 presented increased expression at 15.5 and 19.5 dpc, whereas CC10 was downregulated at 17.5 and 19.5 dpc but not at term. CONCLUSIONS: This study suggests that PNEC hyperplasia in CDH fetal lung is likely because of Notch signaling deregulation, whereas Clara cell hypoplasia in CDH lungs could be a consequence of protein synthesis delay, reflecting a functional maturation hindrance and not a cell fate commitment problem.
Abstract: BACKGROUND: Previous morphological studies had produced controversial results with regard to heart development in congenital diaphragmatic hernia (CDH), whereas a few publications investigated cardiac function and myocardial maturation. Myocardium maturation is associated with age-dependent increasing of gene expression of gap junction protein connexin 43 (Cx43), adenosine triphosphatase of the sarcoplasmic reticulum (SERCA2a), as well as switching of myosin heavy chains (MHCs) from beta to alpha isoforms. Our aim was to evaluate myocardium maturity in nitrofen-induced CDH rat model. METHODS: Fetuses from dated pregnant Sprague-Dawley rats were assigned to 3 experimental groups: control, nitrofen (exposed to nitrofen, without CDH), and CDH (exposed to nitrofen, with CDH). Myocardial samples collected from left ventricle free wall were processed to (i) quantification of messenger RNA (mRNA) of Cx43, SERCA2a, alpha and beta MHC isoforms, as well as beta-actin (housekeeping gene); and (ii) separation of MHC isoforms (alpha and beta isoforms) by sodium dodecyl sulfate polyacrylamide gel electrophoresis silver stained. RESULTS: We demonstrated that there is no difference in myocardial gene expression of Cx43 (control, 1.0 +/- 0.1; nitrofen, 1.1 +/- 0.2; CDH, 1.3 +/- 0.2) and SERCA2a (control, 1.0 +/- 0.1; nitrofen, 0.9 +/- 0.1; CDH, 1.0 +/- 0.2). Myocardial gene expressions of alpha and beta mRNA of MHC isoforms were slightly decreased both in nitrofen and CDH fetuses when compared with control fetuses, but evaluation of the alpha-to-beta ratios of MHC isoforms at protein level revealed no significant differences between CDH and control (control, 16.9 +/- 2.5; CDH, 17.0 +/- 2.0). CONCLUSIONS: Myocardial quantification of Cx43 and SERCA2a mRNA, as well as the expression pattern of MHC isoforms at protein levels, was similar in all studied groups. We predict, therefore, that acute heart failure commonly observed in CDH infants might be attributed predominantly to cardiac overload secondary to severe pulmonary hypertension rather than to myocardial immaturity.
Abstract: A case of isolated velopalatine paralysis in an 8-year-old boy is presented. The symptoms were sudden-onset of nasal speech, regurgitation of liquids into the nose and dysphagia. Brain MRI and cerebrospinal fluid examination were normal. Infectious serologies disclosed an antibody arrangement towards parvovirus B19 that was typical of recent infection. In the absence of other positive data, the possibility of a correlation between the tenth nerve palsy and parvovirus infection is discussed.
Abstract: Wallerian degeneration (WD) after ischemic stroke has been associated to persistent motor impairment, but signal intensity changes on conventional magnetic resonance imaging (MRI) are generally not detected until four weeks after the event. We report a 54 year old male patient, referred to our hospital for sudden-onset left hemiparesis. Cerebral CT showed right fronto-parietal infarct (middle cerebral artery stroke). We performed two CT control, which revealed no haemorrhagic transformation. MRI, obtained 13 days after the onset, demonstrated the infarct, mainly subcortical, extending throughout fronto-temporo-parietal areas and restricted diffusion in the ipsilateral corticospinal tract. In conclusion, WD is apparent on diffusion-weighted imaging within two weeks of stroke, allowing a better prognostic evaluation of recovery. The abnormal signal should not be misinterpreted as new ischaemic lesions.
