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At least 127 records · Page 7Linked to original sources
PubMed2004

Non-Hodgkin lymphoma in a child with Williams syndrome.

Non-Hodgkin lymphoma in an 8-year-old boy with Williams syndrome is reported. Molecular DNA analysis showed a maternal deletion at 7q11.23, the locus of elastin and several other genes, including the BCL7B gene, involved in early development. To our knowledge, this is the second reported case of a lymphoma in a Williams syndrome patient and the first in a child.

Burkitt Lymphoma↗
PubMed1994

Long-term prognosis in primary hyperoxaluria type II (L-glyceric aciduria).

Primary hyperoxaluria type II (PH-II) or L-glyceric aciduria was first reported by Williams and Smith in 1968 (N Engl J Med 278:233-239, 1968). Deficiencies of D-glycerate dehydrogenase and glyoxylate reductase activity in patients with this disorder leads to increases in urinary oxalate and glycerate excretion. Clinically, PH-II presents in a similar fashion to the more common variant of the disorder, PH-I, with symptoms of calcium oxalate nephrolithiasis. To date, 16 patients with PH-II have been described, and information regarding follow-up is available in only three. We review these reports and present five additional patients from three families, four of whom have been followed closely for more than 20 years. The favorable long-term outcome of our patients over 110 patient-years, as determined by renal function testing and radiographic evaluation, suggests that PH-II may have a more favorable prognosis when compared with the natural history of PH-I. Systematic screening of all PH patients in our practice led to the discovery of five of 30 with PH-II, thus suggesting that this subset population may be more common than originally suspected.

Adolescent↗
PubMed2000

[Two cases of primary carcinoma developed in a vesical diverticulum treated with ileal neobladder for total bladder replacement].

Two cases of carcinoma developed in vesical diverticulum were encountered. Both these males, the first, 66-year-old and the second case, 68-year-old gentleman, visited our clinic with a complaint of asymptomatic gross hematuria. Total cystectomy was performed, and an ileal neobladder was constructed for total bladder replacement in both two cases. Pathological examination of the first case revealed transitional cell carcinoma, and the second case showed adenocarcinoma mixed with transitional cell carcinoma. Although primary carcinoma developed in vesical diverticulum was initially reported by Williams in 1883, adenocarcinoma is very rare. The diagnosis is often challenging and the prognosis is dire. Both our cases were successfully treated with total cystectomy and an ileal neobladder was constructed for total bladder replacement. Careful management and aggressive therapy for such patients may make even cure possible.

Adenocarcinoma↗
PubMed1990

A rare case of the inferior mesenteric artery and some colic arteries in man.

The case in which the inferior mesenteric artery (MI) arose from superior one (MS), the common mesenteric artery (MC) of Kitamura et al. (1987), was observed in a 61-year-old female cadaver. This is the 6th report on this anomaly and the only one on the female. The MS arising normally from abdominal aorta sent the MI as the first colic artery, this condition being common in 4 among 5 cases of this anomaly. The MI, however, did not send left colic artery (CS) which arose from a common trunk with right (CD) and middle colic (CM) arteries, which pattern has not been reported. 3 cases of this anomaly may be led from either of 3 kinds of intermesenteric artery (IM) reported by Williams and Klop (1957); the actual IM, an anastomotic artery between CM and CS and the one between MS and CS. The present case is extremely rare, being a compound type led from the first 2 IMs and another case with the common trunk composed of CM and CD, the last of which is collected in Lippert and Pabst (1985). From these examination, formation of MC is discussed with special reference to the inferior pancreatico-duodenal artery.

Arteries↗
PubMed1999

Gas phase activation energy for unimolecular dissociation of biomolecular ions determined by focused RAdiation for gaseous multiphoton ENergy transfer (FRAGMENT)

We present a novel approach for the determination of activation energy for the unimolecular dissociation of a large (>50 atoms) ion, based on measurement of the unimolecular dissociation rate constant as a function of continuous-wave CO(2) laser intensity. Following a short ( approximately 1 s) induction period, CO(2) laser irradiation produces an essentially blackbody internal energy distribution, whose 'temperature' varies inversely with laser intensity. The only currently available method for measuring such activation energies is blackbody infrared radiative dissociation (BIRD). Compared with BIRD, FRAGMENT: (a) eliminates the need to heat the surrounding ion trap and vacuum chamber to each of several temperatures (each requiring hours for temperature equilibration); (b) offers a three-fold wider range of effective blackbody temperature; and (c) extends the range of applications to include initially cold ions (e.g., gas-phase H/D exchange). Our FRAGMENT-determined activation energy for dissociation of protonated bradykinin, 1.2 +/- 0.1 eV, agrees within experimental error to the value, 1.3 +/- 0.1 eV, previously reported by Williams et al. from BIRD experiments. Copyright 1999 John Wiley & Sons, Ltd.

Journal Article↗
PubMed2000

Stability constants of metal-humic acid complexes and its role in environmental detoxification.

Humic acid with hydroxyl-, phenoxyl-, and carboxyl-reactive groups can form coordination compounds with metals. The ion-exchange equilibrium method using Dowex AG 50W-X8, 20-25 mesh Na(+) form was used to determine stability constants of complexes formed between humic acid (isolated from the soil) 50-250 microg ( approximately 3x10(-5)-15x10(-5) mol/liter) and metal salts solution 200 microg at pH 3.5. The stability constant (log K) for different metal-humic acid complexes indicated the following order of the stabilities of complexes formed between humic acid and metal ions: Cu>Fe>Pb>Ni>Co>Ca>Cd>Zn>Mn>Mg. The data on stability constants demonstrated substantial deviation from Irwing-Williams series reported for divalent ions. The molar humic acid/metal ratios were also calculated. Some of the factors affecting the stability constants such as cation exchange capacity of humus soil, molecular radius, and molecular surface area of humic molecules were also estimated. The significance of the data to predict the behavior of these complexes in the environment is discussed.

Algorithms↗
PubMed1992

The human visual system averages speed information.

It has been known for many years that human observers are unable to detect modest accelerations and decelerations in moving visual stimuli. We find that human observers can integrate speeds over many dots, moving at different speeds, producing a global speed percept analogous to the global direction percept first reported by Williams, D. W. and Sekuler, R. (1984, Vision Research, 24, 55-62). We measured speed discrimination for random dot stimuli which contained many different speeds. Our results show that observers always base their discrimination on the mean speed of the stimulus; changes in other stimulus characteristics (e.g. mode) are not detected. Moreover, discrimination thresholds for the global mean speed derived from many different speeds are comparable to those obtained with stimuli in which all dots move at the same speed suggesting that the internal noise associated with the encoding of speed information is quite high.

Differential Threshold↗
PubMed2004

Involvement of cytochrome P450 1A in sanguinarine detoxication.

Sanguinarine (SA), a member of the benzo[c]phenanthridine alkaloids, is a potent anti-microbial agent with anti-inflammatory and anti-neoplastic properties. However, toxicity of the alkaloid severely limits its medical applications. Recent report by Williams et al. implicated rat hepatic cytochrome P450 (CYP) 1A2 as a likely modulator of SA toxicity. Indeed, the in vitro toxicity of SA in primary culture of rat hepatocytes and human hepatic cell line HepG2, demonstrated as lactate dehydrogenase leakage and metabolic capability (MTT assay), was diminished following induction of CYP1A by 2,3,7,8-tetrachlorodibenzo-p-dioxin, 3-methylcholanthrene, and beta-naphtoflavone. Using microsomes containing recombinant CYP1A1 or CYP1A2 we show that SA causes non-competitive inhibition of the former and competitive inhibition of the latter as assessed by ethoxyresorufin de-ethylation (EROD). In human hepatic microsomes SA exhibits competitive inhibition of EROD activity with apparent K(i) of 2 microM, a value identical to that observed for CYP1A2 inhibition in recombinant system. Pre-incubation of SA with human liver microsomes resulted in time-dependent, but not dose-dependent decline in EROD activity suggesting CYP1A2 inhibition is not mechanism based. SA also inhibits activity of NADPH:CYP reductase, an enzyme required for CYP activity, with IC(50) very similar to that observed for EROD inhibition. Tentative mechanism for CYP1A involvement in decreased in vitro SA toxicity is discussed.

Alkaloids↗
PubMed2001

Ancient chemistry fuels new biology.

An enormous new greenhouse project in southern Britain aims to heighten awareness of the human relationship with plants and the growing potential of plant-derived compounds to find new uses, reports Nigel Williams

Biological Factors↗
PubMed2001

Butterfly effects.

Concerns about the potential toxicity of pollen from genetically modified maize on neighbouring butterflies prompted urgent studies, reports Nigel Williams.

Animals↗
PubMed2006

Is there a need to optimize glycemic control in hemodialyzed diabetic patients?

The report of Williams et al. gives rise to at least two important questions regarding diabetic patients on maintenance hemodialysis: (1) Does glycemic control play a significant role? (2) Is HbA1c a reliable measure of glycemic control? These questions are discussed. It is recommended that you treat ESRD patients with diabetes according to guidelines given for patients without ESRD.

Blood Glucose↗
PubMed1988

Effect of growth hormone treatment on sleep EEGs in growth hormone-deficient children.

Total sleep time, sleep stages 1-4, REM, REM latency, and sleep efficiency were analyzed in seven children with growth hormone deficiency (GHD) before and after growth hormone (GH) therapy. Before GH therapy, GHD children spent 19.5% of their total sleep time in REM sleep, 9.7% in stage 1, 41.0% in stage 2, 10.0% in stage 3, and 19.7% in stage 4. GHD children had more stage 1 and 3 sleep and less REM as compared with age-matched normal children reported by Williams et al. After GH therapy was initiated, six of the seven patients had decreases in the duration of stage 3 sleep, with an average decrease of 21.8 min. The difference between stage 3 sleep before and during GH treatment was significant, with a p value of less than 0.025. When the results were expressed as the percentage of the total sleep period, the difference was also significant, (10.0 +/- 2.0 to 7.5 +/- 3.1%, mean +/- SD; p less than 0.05). No other sleep parameters were significantly affected by GH therapy. The changes observed in stage 3 sleep, non-REM sleep, and the lack of any other changes in sleep before and after GH therapy have not been described before in GH-deficient children. They differ from studies in normal humans and animals which showed that REM sleep increased with administration of growth hormone. These differences suggest that GH deficiency is associated with a specific sleep EEG anomaly that is corrected in part by GH therapy.

Child↗