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Congenital defects of bile acid synthesis are rare disorders that cause progressive liver dysfunction. Prolonged neonatal hyperinsulism (PNH) is a separate entity that leads to persistent hypoglycemia secondary to stress. We present a 4-month-old infant who presented with liver failure secondary to a bile acid synthesis defect. The patient’s liver failure resolved with oral cholic acid therapy. This patient also developed PNH, which slowly resolved over time. This case illustrates a possible relationship between cholestatic liver failure and PNH. This relationship may help define specific stressors that increase the likelihood of developing PNH.

Please see related articles and author responses: http://www.nutritionandmetabolism.com/content/9/1/50http://www.nutritionandmetabolism.com/content/10/1/10http://www.nutritionandmetabolism.com/content/10/1/13 ABSTRACT: The article entitled "Monosodium glutamate (MSG) intake is associated with the prevalence of metabolic syndrome in a rural Thai population", concluded that higher amounts of individual's MSG consumption are associated with the risk of having the metabolic syndrome and being overweight independent of other major determinants. However, this epidemiological study is the only study indicating such a relationship between MSG intake and the prevalence of metabolic syndrome and there is no direct supporting evidence for a causal relationship between MSG intake and prevalence of metabolic syndrome. This study does not indicate that MSG causes metabolic syndrome. Furthermore, there are several questionable points concerning study methods. Further carefully designed studies taking into account all glutamate sources are necessary to demonstrate the relationship between overweight, metabolic syndrome, MSG intake and umami sensitivity.

We measured the effect of 3 d of creatine (Cr) supplementation on repeated sprint performance and thigh muscle volume in elite power athletes.Ten male (mean +/- standard deviation of body mass and percentage of fat (81.1 +/- 10.5 kg and 9.8 +/- 3.5) and ten female (58.4 +/- 5.3 kg and 15.0 +/- 3.4) athletes were matched for sex and 10-s cycle sprint scores, paired by rank, and randomly assigned to the Cr or placebo (P) group. Subjects completed six maximal 10-s cycle sprints interspersed with 60 s of recovery before and after 3 d of Cr (0.35 g/kg of fat-free mass) or P (maltodextrin) ingestion. Before and after supplementation, 10 contiguous transaxial images of both thighs were obtained with magnetic resonance imaging.Cr supplementation resulted in statistically significant increases in body mass (0.9 +/- 0.1 kg, P0.03), total work during the first sprint (P0.04), and peak power during sprints 2 to 6 (P0.10). As expected, total work and peak power values for males were greater than those for their female counterparts during the initial sprint (P0.02); however, the reverse was true during the last three sprints (P0.01). Imaging data showed a 6.6% increase in thigh volume in five of six Cr subjects (P = 0.05).These data indicate that 3 d of Cr supplementation can increase thigh muscle volume and may enhance cycle sprint performance in elite power athletes; moreover, this effect is greater in females as sprints are repeated.

ContextManagement of antiretroviral treatment failure in patients receiving protease inhibitor (PI)–containing regimens is a therapeutic challenge.ObjectiveTo assess whether adding a second PI improves antiviral efficacy of a 4-drug combination in patients with virologic failure while taking a PI-containing regimen.DesignMulticenter, randomized, 4-arm trial, double-blind and placebo-controlled for second PI, conducted between October 1998 and April 2000, for which there was a 24-week primary analysis with extension to 48 weeks.SettingThirty-one participating AIDS (acquired immunodeficiency syndrome) Clinical Trials Units in the United States.ParticipantsA total of 481 human immunodeficiency virus (HIV)–infected persons with prior exposure to a maximum of 3 PIs and viral load above 1000 copies/mL.InterventionSelectively randomized assignment (per prior PI exposure) to saquinavir (n = 116); indinavir (n = 69); nelfinavir (n = 139); or placebo twice per day (n = 157); in combination with amprenavir, abacavir, efavirenz, and adefovir dipivoxil.Main Outcome MeasuresPrimary efficacy analysis involved the proportion with viral load below 200 copies/mL at 24 weeks. Other measures were changes in viral load and CD4 cell count from baseline, adverse events, and HIV drug susceptibility.ResultsOf 481 patients, 148 (31%) had a viral load below 200 copies/mL at week 24. The proportions of patients with a viral load below 200 copies/mL in the saquinavir, indinavir, nelfinavir, and placebo arms were 34% (40/116), 36% (25/69), 34% (47/139), and 23% (36/157), respectively. The proportion in the combined dual-PI arms was higher than in the amprenavir-plus-placebo arm (35% [112/324] vs 23% [36/157], respectively; P = .002). Overall, a higher proportion of nonnucleoside reverse transcriptase inhibitor (NNRTI)–naive patients had a viral load below 200 copies/mL compared with NNRTI-experienced patients (43% [115/270] vs 16% [33/211], respectively; P

Eighteen patients received 1,250 mg of allopurinol riboside (AR) four times daily for 28 d. Nine of the patients concurrently received 500 mg probenecid (PB) four times daily. Cure was assessed clinically and parasitologically. Patients who had culture-positive and nonhealing lesions 3 mo after therapy received pentavalent antimony. Of the nine patients who received AR alone, four (44%) had clinical improvement at the end of therapy and two (22%) were culture-negative. A third patient became culture negative at 2 mo after therapy. The culture-negative patients were completely healed at 1 mo and remained so at 1 y after therapy. Of the nine patients who received AR plus PB, four had complete healing and two had clinical improvement at the end of therapy; however, all patients remained culture-positive. At 2-3 mo after therapy, six (67%) of the patients were completely healed, and of these, five (56%) were culture-negative. The drug was well-tolerated.