Inhibitors of an enkephalin degrading membrane-bound metalloendopeptidase: analgesic properties and effects on striatal enkephalin levels

Intraperitoneal administration of N-[1-(R,S)-carboxy-2-phenylethyl-Phe-p-aminobenzoate, synthesized in this laboratory as a potent inhibitor of membrane-bound metalloendopeptidase (EC 3.4.24.11) caused a prolonged but weak analgesic effect on rats as measured by the tail flick test. It also caused a transitory but significant increase in striatal [Leu5]- and [Met5]enkephalin levels 3 h, after administration. Analogs of the inhibitor in which the phenylalanyl residue was replaced by an alanyl or glycyl residue also elicited prolonged analgesic responses although their inhibitory potencies were 75 and more than 1500 times lower respectively. The glycine containing derivative did not alter striatal enkephalin levels 3 h, after administration. The data suggest that inhibition of the metalloendopeptidase decreases the rate of degradation of endogenous enkephalins, however the analgesic properties of the inhibitors do not seem to be related to their inhibitory potencies. Factors other than changes in striatal enkephalin levels may contribute to the analgesic effect of the three N-carboxyphenylethyl derivatives.     

Early Outcomes With HeartWare HVAD as Bridge to Transplant in Children: A Single Institution Experience

The HeartWare HVAD has been used as a bridge to cardiac transplantation in the pediatric population. We describe outcomes following HeartWare HVAD implantation at a single center. A retrospective chart review was performed of all HeartWare HVAD implants performed at our institution between May 2013 and March 2015. Eight children between the ages of 9 and 17 years underwent HVAD implantation as a bridge to transplant (N = 7 cardiomyopathy, N = 1 complex single ventricle). There was one operative death in the complex single ventricle patient. Seven patients (87%) were successfully bridged to transplant. Median time of support was 24.5 days (range, 6–91 days). All transplanted patients are alive and well at a median follow‐up of 448 days. Our results demonstrated that mechanical support with HeartWare HVAD is feasible in patients of varying sizes (from older children to adolescents).     

A case of cleft lip and palate associated with Seckel syndrome.

OBJECTIVE: We report an unusual association of complete cleft of the primary and secondary palate with Seckel syndrome. Seckel syndrome is a very rare syndrome, with only 60 reported cases in the medical literature. It is an autosomal recessive disorder characterized by birdlike face, intrauterine growth retardation, dwarfism, and microcephaly. This young child of 5 years had a successful cleft lip repair under general anesthesia. The main features of the syndrome and the technical problems of anesthesia and surgery are discussed.     

Outcomes in pharyngoplasty: a 10-year experience.

OBJECTIVE: The outcomes of 61 patients who underwent a pharyngoplasty for velopharyngeal insufficiency were reviewed to determine potential risk factors for reoperation. DESIGN: This was a retrospective chart review of 61 consecutive patients over approximately 10 years (1993 to 2003). Variables analyzed included gender, cleft type, age at the time of pharyngoplasty, length of time between palate repair and pharyngoplasty, and associated syndromes. PARTICIPANTS: Of the 61 patients, 20 (34%) had a unilateral cleft lip and palate, 5 (8%) had a bilateral cleft lip and palate, 13 (21%) had an isolated cleft palate, 7 (11%) had a submucous cleft palate, and 16 (26%) were diagnosed with noncleft velopharyngeal insufficiency. RESULTS: Of the 61 patients, 10 (16%) required surgical revision. No statistically significant difference was found among gender, cleft type, age at the time of pharyngoplasty, the length of time between palate repair and pharyngoplasty, and associated congenital syndromes, with respect to the need for surgical revision (p > .05). Of the surgical revisions, 50% (5) were performed for a pharyngoplasty that was placed too low. CONCLUSIONS: Because 50% of the pharyngoplasty revisions had evidence of poor velopharyngeal closure and associated hypernasality resulting from low placement of the sphincter, the pharyngoplasty needs to be placed at a high level to reduce the risk for revisional surgery. The pharyngoplasty is a good operation for velopharyngeal insufficiency with an overall success rate of 84% (51 of 61) after one operation and greater than 98% (60 of 61) after two operations.     

Isolated intracranial Rosai Dorfman disease masquerading as meningioma: a case report

Isolated intracranial Rosai Dorfman disease (sinus histiocytosis with massive lymphadenopathy) is rare. We present a 26-year-old male who presented with left focal motor seizures becoming secondarily generalized of one-year duration. Clinically and radiologically patient was diagnosed to have a right parietal convexity meningioma. However on histopathological examination a final diagnosis of intracranial Rosai Dorfman disease was rendered.