Purpose This study aim to assess the potential of prenatal predictors of postnatal severe pulmonary artery hypertension (PAH) in isolated left congenital diaphragmatic hernia (CDH) and to define a new prenatal pulmonary hypertension index (PPHI). Methods A retrospective chart review of CDH patients between May 2005 and October 2008 was conducted. Ten patients with systemic/suprasystemic and 9 patients with subsystemic pulmonary hypertension at 3 weeks of age were identified. Diameters of the right pulmonary artery, left pulmonary artery (LPAd), aorta, and the length of vermis of the cerebellum were obtained from prenatal magnetic resonance imaging to calculate the PPHI [=(LPAd/length of vermis of the cerebellum) × 10] and the modified McGoon index (MGI) [=(diameter of the right pulmonary artery + LPAd)/diameter of aorta]. Prenatal pulmonary hypertension index and MGI were compared with lung-to-head ratio, percent predicted lung volume, and total lung volume for pulmonary hypertension and survival. Results The PPHI and MGI had a significant, negative correlation with pulmonary hypertension (r = –0.61, P = .005, and r = –0.72, P < .005, respectively). The PPHI and MGI are significantly lower in the systemic/suprasystemic PAH group compared with the subsystemic PAH group (1.11 ± 0.32 versus 1.63 ± 0.28, P = .004, and 0.71 ± 0.15 versus 1.05 ± 0.11, P < .001, respectively). There were no significant differences between the groups comparing the lung-to-head ratio, percent predicted lung volume, and total lung volume. Conclusion Both PPHI and MGI accurately predict the severity of postnatal PAH in isolated left CDH.

Bone marrow derived endothelial progenitor cells (EPCs) are known to play an important role in neovascularization and wound healing. We investigated the temporal effects of cutaneous wounding on EPC surface markers within the peripheral blood (PB) and bone marrow (BM), and better understand the role of the SDF-1α/CXCR4 axis on EPC mobilization after wounding. FVB/NJ mice were administered bilateral 8mm circular full thickness skin wounds. PB and BM were isolated at daily intervals post wounding through day 7 for EPC mobilization characteristics and levels of SDF-1α. Cutaneous wounding was found to cause a transient increase in EPC mobilization that peaked on day 3. In contrast, SDF-1α protein within blood plasma was observed to significantly decrease on days 3, 4, and 7 following cutaneous wounding. BM levels of SDF-1α protein decreased to a nadir on day 3, the same day as peak mobilization was observed to occur. The decrease in BM SDF-1α protein levels was also associated with a decrease in SDF-1α mRNA suggesting transcriptional down regulation as a contributing factor. This study for the first time characterizes EPC mobilization following cutaneous wounding in mice and supports a major role for the SDF-1α/CXCR4 axis in regulating mobilization within the BM, without evidence for systemic increases in SDF-1α.

Abstract-This study was performed to test the hypothesis that long-term nitric oxide synthase (NOS) inhibition during pregnancy may alter the predominance of the vasodilator kallikrein system. Sprague-Dawley rats were treated with the competitive inhibitor of NOS N -nitro-L-arginine (L-NNA, 50 mg ⅐ kg Ϫ1 ⅐ d Ϫ1, dissolved in water) from days 7 to 21 of pregnancy. Rats were studied before treatment (day 5), at days 11, 17, and 21 of pregnancy (during treatment), and at postpartum days 7 and 21 (after the drug was withdrawn at delivery). Each group (nϭ5 to 8) had its corresponding control group (C) that received only vehicle. Additional rats were treated with N G -nitro-L-arginine methyl ester (L-NAME) alone or with an excess of L-arginine. At each study day, we measured blood pressure, collected urine overnight, obtained blood samples, and processed the kidneys for conventional histology and immunohistochemistry. In L-NNA rats, fetal and placental weights were reduced at days 17 and 21. Blood pressure was higher at days 17 and 21, returning to normal after L-NNA was removed. Urinary kallikrein activity was lower at days 11 and 17 (L-NNAϭ1147Ϯ213 and Cϭ2317Ϯ146 nmol/16 h, PϽ0.001). Plasma renin activity was reduced at day 21 (L-NNAϭ9.6Ϯ2.1 and Cϭ25.9Ϯ5 ng ⅐ mL Ϫ1 ⅐ h Ϫ1 , PϽ0.05) and remained lower at postpartum day 7. L-NNA rats exhibited glomerular lesions and tubular atrophy, particularly of connecting tubules that displayed reduced kallikrein staining. Tubulointerstitial infiltrating macrophages (ED1ϩ) were also observed. Renal lesions were present as early as day 11 and persisted at day 7 postpartum. L-NAME rats exhibited similar alterations that were attenuated with an excess of L-arginine. We postulate that the reduction in renal kallikrein may contribute to the hemodynamic alterations described in this model. Key Words: pregnancy Ⅲ nitric oxide Ⅲ plasma renin activity Ⅲ renal kallikrein Ⅲ renal lesions Ⅲ macrophages N ormal pregnancy is characterized by a marked stimulation of the renin-angiotensin system (RAS), which causes renal water and sodium retention and increases plasma volume. 1,2 In turn, plasma volume expansion allows a sustained elevation in cardiac output and, indirectly, in uterine blood flow, conditions necessary for normal fetal growth. Despite the rise in blood volume and the activation of the vasoconstrictor RAS, blood pressure decreases during pregnancy 2 because of a marked reduction in peripheral vascular resistance associated with a predominance of vasodilator, such as kallikrein and nitric oxide (NO), over vasoconstrictor substances. 3 Thus, systemic vasodilatation would initiate the series of hemodynamic changes that are important for fetal growth. Consistent with such a possibility, in normal pregnant rats we have demonstrated a rise in urinary kallikrein activity that precedes the increase in plasma renin activity (PRA). 4 In addition, in conditions associated with a limited plasma volume expansion, such as idiopathic fetal growth restriction 5 and preeclampsia, 6 and in underweig...

Introducción: La acalasia es el trastorno motor primario más frecuente del esófago. Su incidencia reportada es baja, aún más en pacientes pediátricos. La miotomía de Heller laparoscópica corresponde al estándar actual de tratamiento. Durante los últimos años la miotomía endoscópica por vía oral (POEM) se ha posicionado como una alternativa terapéutica segura y tan efectiva como el Heller para la acalasia esofágica. Objetivo: Describir la técnica de POEM y reportar el primer caso pediátrico en nuestro país. Caso clínico: Paciente de 11 años, previamente sano, que se presentó con disfagia ilógica progresiva y baja de peso. El estudio concluyó una acalasia tipo II. Fue sometido a POEM y cursó un postoperatorio sin incidentes. A un año de la intervención se ha documentado resolución de la sintomatología, seguimiento endoscópico y manométrico sin complicaciones. Conclusiones: El caso descrito corresponde al primer POEM en un paciente pediátrico en nuestro país. La acalasia esofágica es infrecuente en pediatría y el POEM ha demostrado éxito clínico y seguridad comparables a la miotomía de Heller laparoscópica en el corto y mediano plazo. El seguimiento a largo plazo permitirá determinar su rol definitivo en el tratamiento de pacientes pediátricos con acalasia esofágica.