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Biomedical subjects

C Hanley

Publications and source records attributed to C Hanley.

6 recordsLinked to original sources
PubMed2004

Successful long-term treatment of hepatic carnitine palmitoyltransferase I deficiency and a novel mutation.

Individuals with carnitine palmitoyltransferase I (CPT-I) deficiency cannot metabolize long-chain fatty acids and can develop life-threatening hypoglycaemia. We present a boy with CPT-I deficiency maintained on a very low-fat diet with nighttime uncooked cornstarch feedings for 5(1/2) years with good success. He has had normal growth and no episodes of hypoglycaemia or adverse side-effects. We found that he was homozygous for a previously undescribed mutation, T314I, in the CPT1A protein.

Carnitine O-Palmitoyltransferase↗
PubMed1989

Characterization of a partly folded protein by NMR methods: studies on the molten globule state of guinea pig alpha-lactalbumin.

NMR spectroscopy has been used to investigate the structure of a partially folded state of a protein, the molten globule or A-state of alpha-lactalbumin. The 1H NMR spectrum of this species differs substantially from those of both the native and fully unfolded states, reflecting the intermediate level of order. The resolution in the spectrum is limited by the widespread overlap and substantial line widths of many of the resonances. Methods have therefore been developed that exploit the well-resolved spectrum of the native protein to probe indirectly the A-state. A number of resonances of the A-state have been found to be substantially shifted from their positions in the spectrum of the unfolded state and have been identified through magnetization transfer with the native state, under conditions where the two states are interconverting. The most strongly perturbed residues in the A-state were found to be among those that form a hydrophobic core to the native structure. A number of amides were found to be highly protected from solvent exchange in the A-state. These have been identified through pH-jump experiments, which label them in the spectrum of the native protein. They were found to occur mainly in segments that are helical in the native structure. These results enable a model of the A-state to be proposed in which significant conformational freedom exists but where specific elements of native-like structure are preserved.

Animals↗
PubMed1978

Noncompliance in an exercise rehabilitation program for men who have suffered a myocardial infarction.

A group of 163 men in the Hamilton, Ont. region who had suffered a myocardial infarction were enrolled in a rehabilitation program of physical activity. These men constituted one cohort of a multicentre collaborative study designed to determine the effects of regular exercise of differing intensity on morbidity and mortality over a 4-year period.The noncompliance rate was 43% for the men who could have participated for 1 year, 46% of whom were classified as noncompliers within 1 month of entry into the program. There was no difference in overall compliance between the men exercising at high intensity and those exercising at low intensity; lack of motivation or interest was the most common reason for their leaving the program. Those leaving the program early tended to have a type A behaviour pattern (they were aggressive, ambitious and competitive, with a chronic sense of time urgency), were inactive during their leisure time, had had at least two previous infarctions and smoked. These characteristics suggest that the men leaving the program early may have been those at greatest risk for a further myocardial infarction.

Adult↗
PubMedDate not supplied

Results of early excision and full-thickness grafting of deep palm burns in children.

The timing and method of treatment of deep palm and finger burns varies widely. Our protocol involves performing full-thickness skin grafts (FTSG) in nonhealing palm burns. We reviewed the functional and cosmetic results after FTSG to the palm. From August 1997 to April 1999, 11 patients (12 palms) underwent excision and FTSG within 2 weeks of injury. A panel of medical and nonmedical professionals evaluated follow-up pictures of the grafts at 1, 2 to 4, and beyond 4 months. Parameters used for evaluation were color match with the unburned skin (1 = no difference to 4 = large difference), graft thickness (1 = flat to 4 = markedly raised), overall appearance (1 = poor to 100 = excellent), and time to maturity. Hand function, as assessed by occupational therapy notes, was also recorded. All 12 grafted palms had a 100% take and healed with minimal scarring. Mean color match scores were 2.4 at 1 month, 1.8 at 2 to 4 months, and 1.6 beyond 4 months. On the graft thickness scale, grafts were given scores of 2.6, 2.2, and 1.9 during the same intervals. The overall appearance was 71 at 1 month, 81 at 2 to 4 months, and 85 beyond 4 months. All grafts except 1 were judged to be mature at 2 to 4 months follow-up. Full passive range of motion was attained in all grafted hands within the period of 2 to 4 months after operation. Early excision and FTSG of deep pediatric palm burns can be performed in the outpatient setting. The grafts have an acceptable color match, are minimally raised, and achieve excellent cosmetic result. The grafts mature within a few months after surgery to allow for rapid return to normal range of motion. FTSG should be considered as a first choice for deep palm burns.

Burns↗