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.     

A cholesterol embolism following coronary angioplasty. A case report

Cholesterol embolism is a rare but serious complication of heart catheterization. We report a patient in whom cholesterol embolization syndrome developed after coronary angioplasty complicated by an acute myocardial infarction which was treated with streptokinase and heparin. The clinical outcome was satisfactory. Cholesterol embolism occurrence might have been precipitated in this patient by thrombolytic and anticoagulant therapy.     

Structure-activity relationships among di- and tetramine disulfides related to benextramine

The synthesis and irreversible alpha-blocking activity in the rat vas deferens of a series of tetra- and diamine disulfides 2-38, structural analogues of benextramine (BHC), are described. All compounds containing a central cystamine moiety displayed an irreversible alpha-adrenergic blockade at concentrations ranging from 10(-4) to 6 X 10(-6)M. Potency was increased in cystamines N,N'-disubstituted with 6-aminohexyl groups, especially when the outer nitrogen atoms bear arylalkyl substituents or are enclosed in a ring. However, N,N,N',N'-tetrasubstituted cystamines were poor blockers. Structural specificity in the outer portion of the tetramine disulfide is low, since many types of substituents gave rise to potent alpha-blockers. Even replacement of the outer amines with nonbasic ethers or amides was observed to maintain irreversible alpha-blockade.     

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.     

Visceral leishmaniasis: another HIV-associated opportunistic infection? Report of eight cases and review of the literature

Visceral leishmaniasis (VL) is considered an opportunistic infection in immunocompromised patients. We review the clinical, laboratory, and therapeutic data in 63 patients (eight new cases and 55 cases reported in the literature) with Mediterranean VL (kala azar) and HIV-1 infection to determine whether VL should be considered an opportunistic infection in HIV-infected adults. We conclude that: (1) in areas where both leishmaniasis and HIV-1 infection are endemic, VL may be more frequent among HIV-infected adults; (2) in HIV-infected patients, the clinical picture did not differ significantly from classical kala azar, although it often ran a recurrent course, with resistance to antimonial therapy. We propose the inclusion of VL in the IVC-2 subgroup of the Centers for Disease Control (CDC) clinical classification of HIV-1 infection while prospective and larger studies further define whether there are clinical presentations that could justify adding VL to the list of opportunistic infections indicative of AIDS.     

Bilateral abscessed orchiepididymitis associated with sepsis caused by Veillonella parvula and Clostridium perfringens: case report and review of the literature.

Veillonella species is a gram-negative coccus which is part of the anaerobic normal flora in the oral cavity, small intestine, upper respiratory tract, vagina, and urinary tract. The role that this organism plays in infection is not well known, and it is generally associated with other bacteria. We present a case of bilateral abscessed orchiepididymitis associated with septicemia due to Veillonella parvula and, later, to Clostridium perfringens, with the development of severe renal insufficiency and septic shock, which resolved favorably with antibiotic therapy, treatment of shock, and hyperbaric oxygen therapy. In reviewing the literature, we have not found any other case of sepsis due to Veillonella sp. associated with urological disorders.