It is not uncommon for research and quality control samples, including carbonated beverage samples, to be refrigerated or frozen during peak periods of production and/or sampling, when analytical demand exceeds instrumental capacity. However, the effect of sub-ambient temperatures on carbonated beverage composition during storage has not been well characterized. Mid-infrared (MIR) spectroscopy combined with principal component analysis (PCA) and traditional chemical analyses were used to evaluate the effects of refrigeration (for 1 week) and freezing (for 1 or 6 weeks) on the composition of carbonated beverages, including sparkling water, sparkling wine, beer, and cider. Carbonated beverages were generally resistant to changes in pH, titratable acidity, alcohol, total phenolics, sugar, and color, during short-term (1 week) storage. However, long-term (6 week) freezing resulted in decreased total phenolics, with acidity also affected, albeit to a lesser extent. MIR spectroscopy combined with PCA enabled discrimination of carbonated beverages based on composition, with alcohol content having a significant influence. Examination of the MIR 'fingerprint' region indicated subtle compositional changes occurred in carbonated beverages following prolonged freezing.Several studies have considered the quality of beer [2], wine [3,4], and soft drinks [5] stored at ambient or slightly elevated temperatures, in the context of shelf life. Additionally, the effects of refrigeration on wine [3] and beer [6] composition have previously been examined, by gas chromatography-mass spectroscopy (GC-MS) or ultra-performance liquid chromatography-mass spectrometry (UPLC-MS); albeit these methods are expensive, time consuming, and typically require trained staff to interpret the data. However, there is a lack of information concerning the effect of freezing on carbonated beverage composition, particularly using cost effective methods of analysis.Infrared (IR) spectroscopy is a rapid analytical technique that exploits the absorbance of light by different molecules, depending on their structural characteristics [7], which can then be used to identify components within a given matrix. Spectroscopic techniques in the mid-infrared (MIR) region have enabled characterization of many beverage constituents, including acids (malic, tartaric, and acetic acids), sugars, and alcohol [8]; with the minimal sample preparation requirements facilitating high sample throughput [9], compared to conventional GC-MS or UPLC-MS [6].Wine, beer, and cider are highly complex substrates, comprising an array of constituents at different concentrations, many at trace levels. A number of studies have been undertaken to investigate the applications of MIR spectroscopy as non-destructive, cost-effective methods of beverage analysis. In wine, MIR analysis has been used for authentication [10] and varietal identification [11], as well as for determinations of alcohol, volatile acidity, titratable acidity, pH, sulphur dioxide, sugars, esters, and/or phenolic compounds, in ...

A 52-year-old female presented with Philadelphia chromosome-positive acute nonlymphocytic leukemia and a morphologically benign-appearing histiocytosis with intramedullary cytophagocytosis of formed blood elements. No cause of the reactive histiocytosis could be found. Despite initial successful therapy of the acute nonlymphocytic leukemia with induction of a cytological remission, pancytopenia with marked cytophagocytosis persisted. Therapy aimed at reducing the degree of cytophagocytosis by the histiocytes, in the form of vinblastine-treated platelets and, subsequently, prednisone, was instituted. There was no significant clinical response to either therapeutic maneuver. Cytophagocytosis persisted until leukemic relapse and death ensued.

Plethyle~ietetral~ydrofolate red&tase-@KtlFR) catirlyzes the formation of 5-met.l1yltetral1ydrofolate, the m i n tissue and serum f o n of folic acid, and methyl donor for conversion of homocysteine to methionine. Four patients, 2 of whom are sibs, have been identified. All 4 have neurologic abnormalities and one of the sisters has a folate-responsive, schizophrenialike disorder (Freeman et al. N. h g l . J. &fed. 292:491, 1975). hmFR activity is present at comparable levels i n normal skin fibroblasts, anniotic fluid cells and lymphoblasts.MlTFR activities in extracts of both n o m l and reductasedeficient fibroblasts were low and quite variable during log growth, and were therefore studied at conflutncy. PmIFR activity in the patients' fibroblasts was 14-20% of normal. Activities in the parents of a patient with 20% of normal activity were 40% and 359 of n o m l suggesting autosomal recessive inheritance. When extracts were incubated at 55'~~ residual MI)lFR activity in the sibs slmwed normal t h e m 1 stability, decreasing to 22% and 38% of the initial values in 30 min. In contrast hfElFR from a 3rd patient was exponentially inactivated in 70 min, while that from a 4th unrelated patient was also completely inactivated but somwhat less rapidly.These results suggest that the reductase deficiency i n these unrelated families results frm at least 3 distinct mutant alleles. COMPARISON OF COLLAGEN FROM CULTURED HUMAN FIBRO- BLASTS DERIVED FROM NORMAL INDIVIDUALS AND THOSE WITHCONNECTIVE TISSUE DISORDERS. E. Feng. O.M. Rennert. Dept. Pediatr. and Biochern., Univ. Fla., Gainesville. Florida.In 1973 Priest st. indicated that collagen from cultured fibroblasts from patients with Marfan Syndrome contained more soluble collagen than that from normals. In vivo studies of patients with Ehlers-Danlos Syndrome, specifically types V,VI and VII, indicated abnormally soluble collagen. The copper cofactor requirement of lysyl oxidase, abnormal copper transport in Menkes Kinky Hair Syndrome (MKHS) and abnormal vascular collagen and elastic fibers prompted the study of collagen in MKHS fibroblasts.Acid-soluble collagen was extracted from the cell layer of cultured human fibroblasts and estimated by measuring hydroxyprolina The amount of hydroxyproline in the soluble fraction of fibroblast cultures from normal individuals, Ehlers-Danlos Syndrome (EDS). Marfan Syndrome (M) and Cutis Laxa (CL) were 68% + 3%. 70% + 7%. 77% + 10% and 65% + 10%. respectively. Fibroblasts from 2 -MKHS patients gave values of 76% and 87%. There was no correlation between the percentage of soluble collagen and the passage number of the cultures.Total collagen, combined soluble and insoluble fractions, expressed with respect to DNA content indicated 113 -112 the total collagen in EDS, CL and M fibroblasts as contrasted to normals. Studies of 14~-proline incorporation into collagen in these fibroblasts as a function of passage number will be presented. ARC IN RANDOM X-INACTIVATION IN A FEMALE WITH AN INTERSTI-502 TIAL SHORT ARM DELETION OF THE X CHROMOSOME....