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We report the case of a 4-month-old boy diagnosed with DiGeorge syndrome with novel ocular features. The patient was diagnosed through genetic testing, with a noted 22q11.2 deletion, and had the additional clinical findings of cardiac anomalies, Hirschsprung's disease, and intracranial microhemorrhages. Eye findings included bilateral microphthalmia, persistent fetal vasculature, chorioretinal coloboma, and a unilateral orbital cyst. Given no known additional inciting exposures, a dysgenic mechanism resulting in failed closure of developmental fissures associated with the chromosomal deletion likely gave rise to these combined pathologies.

Purpose To report the clinical features, antibiotic susceptibility profiles, treatment, and visual acuity (VA) outcomes of endophthalmitis caused by Corynebacterium species. Design Retrospective case series. Participants Patients with endophthalmitis caused by Corynebacterium species. Methods Microbiology database records were retrospectively reviewed for all patients with endophthalmitis caused by Corynebacterium species from January 1, 1990, to December 31, 2012, at a large university referral center. The corresponding clinical records were then reviewed to evaluate endophthalmitis clinical features and treatment outcomes. Main Outcome Measures Presenting clinical features, visual acuity outcomes, and antibiotic susceptibility patterns. Results For the 10 patients identified, clinical settings included post–cataract surgery (n = 6), post–penetrating keratoplasty (n = 2), and posttrabeculectomy (n = 2). The mean time from surgical procedure to presentation with endophthalmitis was 6.8 months (range: 1 day to 28 months). All isolates were susceptible to vancomycin. Presenting VA ranged from 7/200 to no light perception. Initial treatment strategies were vitreous tap and intravitreal antibiotic injection (n = 5) and pars plana vitrectomy with intravitreal antibiotic injection (n = 5). VA outcomes were ≥20/60 in 5 of 10 patients (50%) and ≤20/400 in 5 of 10 patients (50%). Conclusions The most common clinical setting was post–cataract surgery. All isolates were susceptible to vancomycin. Despite prompt treatment with appropriate antibiotics, visual outcomes varied.

To report the clinical settings, bacterial isolates, antibiotic sensitivities, and visual acuity outcomes of patients with persistently positive vitreous cultures after intravitreal antibiotics.Consecutive, noncomparative case series.setting: Tertiary care center.Thirty-six eyes of 36 patients with exogenous endophthalmitis with the same bacterial organism identified on at least 2 consecutive vitreous cultures from 1981 to 2015.Vitreous cultures with intravitreal injections of antibiotics and pars plana vitrectomies with intravitreal antibiotics.Bacterial isolates, antibiotic sensitivities, visual outcomes.Thirty-six eyes of 36 patients met the study criteria. The mean follow-up was 26.5 months. The most common clinical settings were after cataract extraction (18/36, 50%) and glaucoma surgery (11/36, 31%). The mean initial visual acuity was 2.16 ± 0.77 logMAR (Snellen equivalent ≈20/2900), and there was no statistically significant change at the final evaluation (2.08 ± 0.97 logMAR, ≈20/1900, P = .72). The most common bacteria were Staphylococcus (11/36, 31%) and Streptococcus (9/36, 25%). Gram-positive bacteria were sensitive to vancomycin (27/27, 100%); gram-negative bacteria were sensitive to amikacin (5/5, 100%). The antibiotic sensitivities were the same on repeat cultures in 34 of 36 patients (94%). The initial treatment was a vitreous culture and intravitreal injection of antibiotics in 28 of 36 patients (78%). The vision at the last follow-up was 20/200 or better in 12 patients (33%) and no light perception in 11 of 36 patients (31%).The most commonly identified organisms were gram-positive bacteria. There was good concordance in the antibiotic sensitivities between initial and subsequent cultures. Patients with persistently vitreous culture-positive endophthalmitis had poor visual outcomes.

We report here the in vivo conversion of [3H]myoinositol to [3H]chiroinositol. After labeling intraperitoneally with [3H]myoinositol for 3 days to reach radioisotope equilibrium in urine, [3H]chiroinositol was isolated from tissues and purified after 6 N HCl hydrolysis by two sequential paper chromatographies and high performance liquid chromatography (HPLC). Percent conversion of [3H]myoinositol to [3H]chiroinositol was highest in urine (36%), liver (8.8%), muscle (8.8%), and blood (7.6%) with intestine, brain, kidney, spleen, and heart decreasing in percentage from 2.8 to 0.7%. Labeling of other inositol isomers including scyllo-, neo-, and epi-, and mucoinositol was minimal, approximately 0.06% of [3H]myoinositol. Glucose was unlabeled, but glucuronate, the product of myoinositol oxidation, was labeled up to 1.5% of the [3H] myoinositol. Acid hydrolysates of combined inositol-containing phospholipids contain significant labeled chiroinositol. [3H]Phosphatidylinositols and [3H]glycosylphosphatidylinositols were extracted from liver, muscle, and blood, isolated by thin layer chromatography, and inositols purified by HPLC after acid hydrolysis. Percent conversion of [3H]myoinositol to [3H] chiroinositol was highest in blood (60.4%) followed by muscle (7.7%) and liver (2.2%).

The naturally-occurring polyamines were found to stimulate glycogen synthase phosphatase from rat skeletal muscles. The sequence of effectiveness in the stimulation was spermine greater than spermidine greater than putrescine. It was shown that the spermine-sensitive phosphatase was present primarily in the soluble fraction of the muscle extract. In the presence of spermine, the phosphatase activity can be further stimulated by Mn2+; however, a lower Mn2+ concentration is required for the activation of the enzyme in comparison with that in the absence of spermine. Kinetic studies indicated that activation of glycogen synthase phosphatase by spermine was achieved by an increase in its V without significant alteration in the Km, suggesting that spermine directly stimulated the catalytic efficiencly of the phosphatase enzyme.

Substantial evidence suggests that insulin receptor-associated protein kinase may play a pivotal role in the expression of the intracellular effects of insulin. This study was undertaken to determine whether insulin receptor kinase contributes to the generation of putative insulin mediators. The effect of ATP and divalent cation addition on the production of insulin mediators from liver plasma membranes was investigated. ATP (1 mM) added to liver plasma membranes in the absence of divalent cations enhanced insulin-stimulated release/generation of mediator slightly (approximately 3-fold). ATP in the presence of Mn2+ further increased release/generation of mediator markedly (approximately 100-fold). In contrast, ATP in the presence of Mg2+ had no stimulatory effect. Mn2+ and Mg2+ alone were ineffective. Addition of EDTA completely diminished the stimulatory effects of insulin, ATP, and Mn2+. The stimulation was ATP-specific since other nucleotides and nonhydrolyzable analogues of ATP had no or very weak activity. ATP-Mn2+ stimulated insulin-dependent mediator release/generation in a dose-dependent manner. These results suggest that insulin mediator release/generation is markedly stimulated by an ATP-Mn2+-dependent phosphorylation reaction, similar to insulin-stimulated receptor tyrosine kinase phosphorylation.

Two fractions of protein kinase(s) from rabbit skeletal muscle have been shown to catalyze the phosphorylation of endogenous protein, histone, and glycogen synthase I utilizing UTP, GTP, CTP, and ITP as phosphoryl donors. With one protein kinase fraction, it was found that the enzymatic activity was enhanced by Mn2+ when CTP served as the phosphoryl donor. This is in contrast to the inhibitory effect of Mn2+ on the kinase activity with ATP as the phosphoryl donor. With the second protein kinase fraction, the UTP, GTP, CTP and ITP associated phosphorylation reactions were found to be inhibited by cyclic AMP, whereas the phosphorylation reaction in which ATP served as the phosphoryl donor was found to be stimulated by cyclic AMP. These results suggest that different forms of the protein kinase(s) may exist which have different nucleoside triphosphate donor specificity and metal ion requirement. The serine residues of endogenous protein have been demonstrated to be the sites of phosphorylation when UTP, GTP, CTP and ITP served as the phosphoryl donors.

1. 1. The regulatory subunit of Type I cAMP-dependent protein kinase from rabbit skeletal muscle can bind [ 3 H]cAMP to form the R-[ 3 H]cAMP complex, and the slow phase of the enhanced exchange of free cAMP with [ 3 H]cAMP from the R-[ 3 H]cAMP complexes was studied under various conditions using the equilibrium isotope exchange technique. 2. 2. Results indicate that Mg-ATP and the catalytic subunit are absolutely required for the enhanced exchange reaction to occur, but phosphorylation of the regulatory subunit by Mg-ATP does not play a determining role in the slow rate of the dissociation/association of the Type I protein-kinase in the presence of cAMP and the catalytic subunit. 3. 3. We interpret the role of Mg-ATP as being one in which it may provide the structural attributes required for formation of a stabilized transient state of the cAMP-regulatory subunit-catalytic subunit ternary complex, an obligatory intermediate involved in the dissociation/association of Type I cAMP-dependent protein kinase.

Monoclonal antibodies with an apparent specificity for human small-cell carcinoma, adenocarcinoma, and squamous carcinoma of the lung are produced by some hybridomas obtained from mice and rats immunized with an established line of human small cell lung cancer. Out of 85 of these antibodies produced by independently isolated hybridomas from 15 different fusions, 21 are directed against the sugar sequence which occurs in lacto- N -fucopentaose III ceramide, in several higher glycolipids and in glycoproteins. Specificity was determined by autoradiography of thin-layer chromatograms of glycolipids, by solid-phase radioimmunoassays, and by hapten inhibition studies. All 21 antibodies are of the immunoglobulin M type.