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Biomedical subjects

P Lambert

Publications and source records attributed to P Lambert.

At least 109 records · Page 6Linked to original sources
PubMed1986

[Refsum's disease. Epidemiologic, clinical and biological correlation. 6 cases].

Nine patients with symptoms and signs of Refsum's disease are reported. In 6 a systemic accumulation of phytanic acid was demonstrated, together with low phytanic acid oxidase activity in skin fibroblasts in 5 of them. In 3, no disorder of phytanic acid metabolism was demonstrated. In 3, the diagnosis was made during the pre-clinical period. The disease seems more frequent in Northern France, which agrees with the hypothesis of a genetic mutation which would have taken place in Scandinavia some centuries ago and was subsequently spread by the Vikings. The effects of a dietary treatment on serum phytanic acid levels and clinical disorders are reported. The general condition of the patients improved remarkably but only partially. The diet is unpalatable and in some patients the level of serum phytanic acid increased, due to the mobilization of body fat. Patients with very high levels of phytanic acid might be initially treated by plasmapheresis. For the same reason, the diet should supply enough calories to keep body weight unchanged, and body weight loss whatever its cause should be avoided.

Adolescent↗
PubMed1985

Malignant hypertension in general practice.

A diagnosis of malignant hypertension was recorded for 165 patients in the national morbidity study between 1970 and 1973. Three patients with benign hypertension were selected as age- and sex-matched controls for each case. The general practitioners in the study were asked to complete a further questionnaire about the patients and 66% of the practices agreed to take part. Information about the retinal findings for the patients was requested and less than half of those in the national morbidity study proved to have a strict diagnosis of accelerated or malignant hypertension although they were originally recorded as patients with malignant hypertension. Of those patients originally classified as having benign hypertension 5% had the retinal appearance of accelerated or malignant hypertension.Patients had been diagnosed as having hypertension for a mean of more than five years prior to entry into the national morbidity study and the survival of patients with both benign and accelerated or malignant hypertension was good. Thirtyfour per cent of those with confirmed benign hypertension and 62% of those with definite accelerated or malignant hypertension died in the follow-up period which was on average 10 years from entry into the national morbidity study.The survival of patients registered with doctors who did not collaborate and of patients whose clinical details were missing was similar to the survival of patients for whom full details were provided.Blood pressure control was only fair with a mean of 172/101 mmHg for the group with benign hypertension and 177/107 mmHg for the group with accelerated or malignant hypertension. Blood pressure control was the poorest for those who died from a stroke. A high proportion (78%) of deaths in association with accelerated or malignant hypertension were from cardiovascular or renal causes.

Aged↗
PubMed1983

Calcium regulates troponin-tropomyosin binding in the reconstituted thin filament.

Steady-state fluorescence anisotropy technique was used to determine the binding constant of troponin for IAEDANS-labeled tropomyosin under various conditions. In the absence of actin, Ca does not affect the binding between troponin and tropomyosin. The presence of actin greatly strengthens troponin-tropomyosin binding in the absence of Ca. However, Ca weakens troponin-tropomyosin binding by about 2.5-fold in the reconstituted filament. It is suggested that the Ca-regulated binding may serve as a molecular switch for the troponin molecule to get "on" and "off" the actin-myosin interaction site regulating muscle contraction-relaxation cycles.

Animals↗
PubMed1983

Primary and secondary MLR characterization of three allelic variants of HLA-Dw7 segregating in a single family.

Three of four parental haplotypes of a kindred from the Old Order Amish religious isolate carried HLA-DR7 and specificities of the HLA-Dw7 "cluster." Intrafamily primary and secondary mixed lymphocyte responses clearly distinguished the three Dw7-related allelic specificities. Two of the specificities fall within the Dw11 crossreacting group, designated here as Dw11 "short" (Dw11S) and Dw11 "long" (Dw11L), while the third is more closely related to Dw7. Reaction patterns in this family illustrate the complexity of antigen recognition in primary and secondary mixed lymphocyte responses and the important role played by the responder cell in generating discriminatory primed lymphocyte typing reagents.

Alleles↗
PubMed1982

[Primary neuromeningeal manifestation, isolated for a long period, of acute lymphoblastic leukemia].

The course of acute leukaemias, particularly of the lymphoblastic type, is very frequently complicated by the development of neuromeningeal localisations of the disease. They may, more rarely, be presenting initial lesions, their origin being confirmed clinically and/or biologically, but it is exceptional that they remain a strictly isolated expression of the affection. A case is reported of a 16-year-old adolescent with leukaemic meningitis, in whom haematological tests (blood counts, bone marrow smear, iliac medullary biopsy, lymph node biopsy) gave negative results over a period of at least two months. Based on the findings in this case, the significance of early neuromeningeal localisations of acute leukaemias is discussed.

Adolescent↗
PubMed1981

20 years of hospital admissions for peptic ulcer in England and Wales.

Mortality and hospital admission rates for peptic ulcer in England and Wales have fallen in the past twenty years. Within this pattern there has been a wide range of variation. Perforated ulcers have become less common in young adults, and perforated gastric ulcers in young men now occur with between a quarter and a tenth of their previous frequency. Perforated duodenal ulcer has become more common in middle-aged and elderly women. The reasons for these changes are not understood, but they are likely to reflect the impact of environmental influences on people of different ages.

Adolescent↗