Case Report: Giant choledochal cyst

We report an adult female with a rare giant choledochal cyst. The patient presented following a normal pregnancy with the classical triad of an abdominal mass associated with jaundice and right upper quadrant abdominal pain. The cyst was excised using an intramural technique and biliary reconstruction achieved with a Roux-en-Y hepaticojejunostomy. Our patient has remained well with no evidence of malignancy over a 12 year review period. The aetiology and current management of this condition are discussed.     

Growth hormone benefits children with 18q deletions

Most individuals with constitutional deletions of chromosome 18q have developmental delays, dysmyelination of the brain, and growth failure due to growth hormone deficiency. We monitored the effects of growth hormone treatment by evaluating 23 individuals for changes in growth, nonverbal intelligence quotient (nIQ), and quantitative brain MRI changes. Over an average of 37 months, the treated group of 13 children had an average nIQ increase of 17 points, an increase in height standard deviation score of 1.7, and significant change in T1 relaxation times in the caudate and frontal white matter. Cognitive changes of this magnitude are clinically significant and are anticipated to have an effect on the long-term outcomes for the treated individuals.     

Determination of optimal cryoprotectants and procedures for their addition and removal from human spermatozoa

The objective was to test the hypothesis that the optimal cryoprotective agent for cryopreservation of human spermatozoa would be a solute for which cells have the highest plasma membrane permeability, resulting in the least amount of volume excursion during its addition and removal. To test this hypothesis, theoretical simulations were performed using membrane permeability coefficients to predict optimal procedures for the addition and removal of a cryoprotectant. Simulations were performed using data from four different cryoprotectants: (i) glycerol, (ii) dimethyl sulphoxide, (iii) propylene glycol and (iv) ethylene glycol. Thermodynamic formulations were applied to determine approaches for the addition and removal of 1 M and 2 M final concentrations of cryoprotectant, allowing the spermatozoa to maintain a cell volume within their osmotic tolerance limits. Based on these data, ethylene glycol was predicted to be optimal for minimizing volume excursions among the solutes evaluated. These predictions were then experimentally tested using glycerol as the control cryoprotectant and ethylene glycol as the experimental cryoprotectant. The results indicate that there was a higher (P < 0.05) recovery of motile spermatozoa after cryopreservation when using 1 M ethylene glycol than with 1 M glycerol, supporting the hypothesis that use of the cryoprotectant for which the cell has the highest permeability will result in higher cell survival.      

Comparison of isolation of Haemophilus vaginalis (Corynebacterium vaginale) from peptone-starch-dextrose agar and Columbia colistin-nalidoxic acid agar.

A total of 447 cervical or vaginal specimens were inoculated in parallel onto peptone-starch-dextrose (PSD) and Columbia colistin (10 mg/ml)-nalidixic acid (15 mug/ml) (CNA) agar and were incubated for 48 h at 35 degrees C in an atmosphere with 2 to 10% CO2. One hundred (22.4%) of the cultures were positive for Haemophilus vaginalis. Forty-eight of the isolates were recovered from both PSD and Columbia CNA agar, five from PSD only, and 47 from Columbia CNA agar only (P less than 0.001). On Columbia CNA agar, 76 of the isolates were detected after 24 h of incubation, and the remainder were detected within 4 days of incubation.     

Inhibition of axoplasmic transport in the developing visual system of the rat—I. Structural changes in the retina and optic nerve with graded doses of intraocular colchicine

In order to study the role of axonal transport in the mediation of transneuronal metabolic stimulation upon a population of differentiating neurons, colchicine, a potent inhibitor of rapid and slow phases of axonal transport, was injected into the eye of albino rats at 1,3,5, 10, 15 and 20 days postnatal in concentrations ranging from 10^?5 M to 2 × 10²M and in quantities of 0.3 to 0.5μl. Quantitative light and electron microscopy were subsequently employed to assess reactive alterations in the developing retina and optic nerve. Application of colchicine severely retarded the development of the sensory elements, with disappearance of synaptic ribbons of sensory cell axons, a significant reduction in the thickness of the inner plexiform layer, due to the presence of numerous shrunken synaptic elements and the appearance of rosettes of sensory cells displaced to the inner nuclear layer. These alterations were found to be dose-dependent. Counts of ganglion cell populations at various times after application of colchicine demonstrated optimal concentrations which could be injected at each postnatal age without causing ganglion cell degeneration. Ultrastructural examination of such cells revealed varying degrees of disorganization and dissolution of the endoplasmic reticulum with the formation of occasional small cytoplasmic vacuoles. Higher concentrations of colchicine caused extensive vacuole formation in all classes of retinal neurons, scattered hyperchromic cells and widespread degeneration and autolysis.The diameter of the optic nerve was reduced to 60–95% of normal following intraocular colchicine. depending on the concentration employed, but electron microscopy revealed normal patterns of distribution of axoplasmic microtubules and filaments in control and experimental animals and quantitative analysis revealed no significant loss of axons. While no reactive changes took place in individual elements, the periphery of the nerve was often indented by a highly-folded glia limitans.Maximal doses of intraocular colchicine for each age level were established by this study. These were: 1 day, 10^?3 M: 5 days, 5 × 10^?3M; 10 days, 5 × 10^?3M; 15 and 20 days, 10^?2 M. The information derived from this morphological analysis provides the foundation for subsequent measurements of axonal transport inhibition in the developing visual system to be reported in the second article of this series.     

C1-esterase inhibitor blocks T lymphocyte proliferation and cytotoxic T lymphocyte generation in vitro

We have previously shown that activated C1s complement and activated T cells cleave beta2-microglobulin (beta2m) in vitro leading to the formation of desLys58 beta2m. This process can specifically be inhibited by C1-esterase inhibitor (C1-inh). Furthermore we showed that exogenously added desLys58 beta2m in nanomolar amounts to a one-way allogenic mixed lymphocyte culture (MLC) increased the endogenous production of IL-2 and the generation of allo-specific cytotoxic T lymphocytes. C1-inh was purified from fresh human plasma and added to human or murine MLC and mitogen-stimulated lymphocyte cultures grown in the presence of complement-inactivated serum. Read-outs were cell proliferation, lymphokine production and development of T cell-mediated cytotoxicity. We found that addition of C1-inh to MLC and mitogen- exposed murine and human lymphocyte cultures inhibited proliferation, the development of allospecific cytotoxic activity, and changed the endogenous production of IL-2, IL-4, IL-10, IL-12 and IFN-gamma. These data clearly demonstrate a regulatory function of C1-inh on T cell- mediated immune functions.