Saturday, August 24, 2013

1. LVAD increases overall survival as well as quality of life in comparison to medical management (MM) in patients with NYHA class IV heart failure (REMATCH trial, n=129). LVAD was associated with a 48% reduction in risk of death from any cause; survival at 1 year was 52% in LVAD vs 25% in MM, at 2 years was 23% and 8%. (NEJM)  Another paper doing sub-group analysis of REMATCH trial data showed clear survival benefit in patients receiving inotropic therapy. In patients not receiving inotropic therapy (n=38), survival for LVAD/MM were 57%/40% at 1 year and 22%/16% at 2 years, but the survival differences were not statistically significant (probably underpowered). (circulation) My interpretation is that the benefits of LVAD are clear among people who are very sick (i.e. NYHA IV and inotrope dependent). Among those who are less sick (NYHA class IV not inotrope dependent), LVAD probably still offers a survival benefit, but it is less clear, and must be weighed against the costs (emotional and financial) of going through a big, painful open heart surgery. Note: all-cause mortality includes mortality from complications of LVAD.
2. ECMO use in neonates is associated with sensorineural hearing loss, with a mean incidence of 7.5% (range 3-21%), compared to an overall rate of 1-3% in NICU survivors. This hearing loss is frequently progressive (70%), and delayed-onset (50%). One study (n=111) found the factors associated with an increased risk of developing SNHL after ECMO are diagnosis of congenital diaphragmatic hernia, extended time on ECMO, and extended use of aminoglycosides. (Pediatrics) My interpretation is that ECMO is probably associated with an overall hypoxia, relative to not being on ECMO (ie. having heart and lungs that work), and perhaps the nerve cells of the cochlea are more sensitive to hypoxia than other cells. CDH is associated with lung hypoplasia and sometimes severe hypoxia, so that fits in well, and aminoglycosides are known ototoxins, so that also fits.
3. Factors associated with congenital/neonatal SNHL: 
-Infection: prenatal CMV infection (most common overall cause of congenital SNHL), toxo, rubella, syphillis, bacterial meningitis (s.pneumo, hib).
-trauma: head trauma, temporal bone fracture
-toxin: aminoglycoside, loop diuretic, lead, arsenic, radiation.
-low birth weight (<1500g), apgar scores less than 4 at 1 min and 6 at 5 min, hyperbilirubinemia requiring exchange transfusion
-hypoxia: mechanical ventilation >5 days, ECMO
-stigmata of conditions associated with SNHL: renal abnormalities, craniofacial abnormalities
4. Waardenburg syndrome: partial albinism (white forelock), SNHL, broad mandible.
5. Sandifer syndrome: GERD, hiatal hernia, hypotonia, spasmodic torticollis and dystonia: spasms last 1-3 minutes and occur multiple times a day, sometimes associated with feeding. Can be mistaken for infantile spasms or epilepsy, esp if the GI symptoms are not clear.
6. Mucopruluent drainage from one eye in a neonate, in the absence of conjuncitivitis, with a history of increased tears in one eye: think nasolacrimal duct obstruction, rather than GC/chlamydia (must have conjunctivitis). These things can also progress to infected cysts that require IV antibiotics.
7. Isolated throbocytopenia in a child with a prodome of viral illness (esp VZV, EBV, CMV), think ITP. CBC with diff will reveal normal morphology of platelets and RBCs, and normal levels of other blood cells. If there is a decrease in WBC/RBCs as well, or LAD/splenomegaly, do a BM biopsy to r/o aplastic anemia or cancer. Among people with ITP, 50% resolve in 1 month, another 30% in 6 months. Treatment is to watch them carefully to prevent injury. Pharmacologic treatment is not shown to improve outcomes, and is only indicated if platelets are <20,000, there are severe bleeding symptoms (intracranial- occurs in 1% of ITP, massive GI bleed) or if a safe home environment cannot be guaranteed. Tx options: IVIg, systemic steroids, splenectomy (need lifelong vaccines against strep pneumo). Platelet transfusion only if there is life-threatening bleeding.
8. Drugs that cause decreased platelets: Heparin (HIT), bactrim, quinine, quinidine, cimetidine, benzos, penicillin.
9. Deep brain stimulators are now incorporating recording as well as stimulating functionality, moving closer to the ultimate goal of a closed-loop, self-adjusting stimulator system. (medgadget)
10. Closed-loop insulin management is finally here! A team at the University of Virginia have linked a blood glucose monitor to an insulin pump via smartphone (bluetooth). The first trial enrolled 20 people with type 1 diabetes for 42 hours, and found the system had a 97% uptime. Paving the way for new trials. (c/o medgadget) (diabetes care) 

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