Congenital high airway obstruction syndrome (CHAOS): Natural history,prenatal management strategies,and outcomes at a single comprehensive fetal center

PurposeCongenital high airway obstruction syndrome (CHAOS) is a devastating fetal condition of complete airway discontinuity resulting in significant hydrops and extreme lung hyperplasia. It is universally fatal with survival reported only in the rare spontaneous fistulization or EXIT intervention (Ex Utero Intrapartum Treatment). Even in these cases, mortality remains high, and current investigations are targeting prenatal interventions. This report describes our experience with management and fetal interventions for CHAOS, including laser laryngotomy.MethodsWe retrospectively reviewed all patients diagnosed with CHAOS at a single academic institution between 2006 and 2017.ResultsFifteen patients were identified. Eight had obstruction at the trachea and seven at the larynx. In the laryngeal obstructions, three expired shortly after birth, and one survived after spontaneous fistulization and subsequent EXIT to tracheostomy. The remaining three underwent in-utero treatment with laser laryngotomy. One had preterm premature rupture of membranes (PPROM), delivered 3 days post-operatively, and died. Two underwent EXIT to tracheostomy with one surviving to discharge and is currently 2 years old.ConclusionOur study demonstrates the outcomes of a large series of patients diagnosed with CHAOS. While mortality remains high, options for fetal intervention are being explored to allow alterations in the prenatal natural history and improve postnatal outcomes.Type of StudyRetrospective Treatment Study.Level of EvidenceLevel IV.     

Endosonographic characteristics of perigastrointestinal lymphnodes studied ex-corpore.

OBJECTIVES: Assessment of morphological characteristics of lymphnodes studied ex-corpore by echoendoscopy--shape, sharpness of limits, echogenicity and dimensions--to categorize them either as metastatic or non-metastatic. MATERIAL AND METHODS: Fifty-four lymphnodes were studied. They were identified and studied by echoendoscopy in fresh surgical specimens of oesophagus, stomach and rectum. Eight nodal characteristics were evaluated. The data obtained were studied by multivariate analysis using the logistical estimate method in two different statistical models. RESULTS: In model 1, logistical estimate demonstrated that well-defined limits (WDL) of the lymphnodes and the association of hypoechogenicity and round shape were the most significant variables suggesting the presence of invasion. In model 2, the most significant variables were WDL, round shape and association of round shape with WDL. The first model had a high sensitivity of 93.1% and specificity of 68% whereas the second model had a greater specificity of 84%, with a slight fall in sensitivity. CONCLUSION: These models might have an application to clinical practice particularly in the pre-operative assessment of patients with oesophageal carcinoma, gastric carcinoma or rectal carcinoma.     

Orbicularis oculi responses to stimulation of nerve afferents from upper and lower limbs in normal humans

A brief mechanical or electrical stimulus to peripheral nerve afferents from the upper and lower limbs elicited a small and inconsistent EMG response of the orbicularis oculi muscles. This response was facilitated when the stimuli were delivered at fixed leading time intervals, of 45–300 ms, with respect to a supraorbital nerve electrical stimulus. Also, the peripheral nerve stimulus modified the conventional blink reflex responses, inducing facilitation of R1 and inhibition of R2. These results suggest a complex processing of sensory inputs from the face and the limbs at the brainstem, where they are probably integrated in a network of interneurons influencing the excitability of facial motoneurons.     

Influence of bradykinin B1 and B2 receptors in the immune response triggered by renal ischemia–reperfusion injury

Bradykinin B1 receptors are exclusively expressed in inflamed tissues. For this reason, they have been related with the outcomes of several pathologies. Ischemia–reperfusion injury is caused by the activation of inflammatory and cytoprotective genes, such as macrophage chemoattractant protein-1 and heme oxygenase-1, respectively. This study was aimed to analyze the involvement of bradykinin B1 and B2 receptors (B1R and B2R) in tissue response after renal ischemia–reperfusion injury. For that, B1R (B1−/−), B2R (B2−/−) knockout animals and its control (wild-type mice, B1B2+/+) were subjected to renal bilateral ischemia, followed by 24, 48 and 120 h of reperfusion. At these time points, blood serum samples were collected for creatinine and urea dosages. Kidneys were harvested for histology and molecular analyses by real-time PCR. At 24 and 48 h of reperfusion, B1−/− group resulted in the lowest serum creatinine and urea levels, indicating less renal damage, which was proved by renal histology. Renal protection associated with B1−/− mice was also related with higher expression of HO-1 and lower expression of MCP-1. In conclusion, the absence of B1R had a protective role against inflammatory responses developed after renal ischemia–reperfusion injury.     

Deposition and passage of transthyretin through the blood-nerve barrier in recipients of familial amyloid polyneuropathy livers

Familial amyloid polyneuropathy (FAP) is characterized by deposition of mutated transthyretin (TTR) in the peripheral nervous system. Prior to amyloid fibrils, nonfibrillar TTR aggregates are deposited inducing oxidative stress with increased nitration (3-NT). As the major source of TTR is the liver, liver transplantation (LT) is used to halt FAP. Given the shortage of liver donors, domino LT (DLT) using FAP livers is performed. The correlation between TTR deposition in the skin and nerve was tested in biopsies from normal individuals, asymptomatic carriers (FAP 0) and FAP patients; in FAP 0, nonfibrillar TTR was observed both in the skin and nerve in the same individuals; in patients, amyloid was detected in both tissues. The occurrence of amyloidosis in recipients of FAP livers was evaluated 1-7 years after DLT: TTR deposition occurred in the skin 3 years after transplantation either as amyloid or aggregates; in one of the recipients, fibrillar TTR was present in the epineurium 6 years after DLT. Deposits were scarce and 3-NT immunostaining was irrelevant. Nerve biopsies from DLT recipients had no FAP-related neuropathy. Our findings suggest that TTR amyloid formation occurs faster than predicted and that TTR of liver origin can cross the blood-nerve barrier. Recipients of FAP livers should be under surveillance for TTR deposition and tissue damage.     

A new nematode, Ansiruptodera scapteromi sp. nov. (Nematoda: Aspidoderidae), recovered from the Argentinean water rat Scapteromys tumidis (Waterhouse, 1837) in Uruguay

This report describes a new species of aspidoderid nematode, Ansiruptodera scapteromi sp. nov., the second species to be reported in the genus Ansiruptodera Skrjabin and Shikhobalova, 1947. The A. scapteromi sp. nov. is the first species of the genus to be recorded from a rodent host. The new species is clearly different from the only other species, A. ansirupta (Proença, 1937) Skrjabin and Shikhobalova, 1947, in that it possesses short lateral alae that terminate before the midbody; a smaller cephalic extremity; a shorter esophagus, pharynx, tail, and tail appendage; a smaller sucker; and longer spicules. The two species also differ in the numbers and arrangements of caudal papillae. A. scapteromi appears to be a parasite of capture and the water rats seem to have been infected from Edentata in Uruguay.