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Biomedical subjects

P Mustajoki

Publications and source records attributed to P Mustajoki.

At least 91 records · Page 5Linked to original sources
PubMed1987

Psychosocial factors in obesity.

Obese people as a group have similar mental health as normal weight people, and there are no psychiatric features characteristic of obesity in general. However, small subgroups of obese individuals may have psychiatric abnormalities which are specific for obesity, such as body image disturbance or periodic compulsive overeating (bulimia). Obesity is strongly related to sociocultural factors. In western countries obesity is commoner in lower than in higher social classes. Thus, the development of obesity is influenced by social status. However, also the converse is true: recent observations suggest that obese people lose social status. This is probably due to prejudice and discrimination against obese persons in the modern western society.

Body Image↗
PubMed1986

Pharmacokinetics of intravenously administered haem arginate.

The pharmacokinetics of haem were investigated after intravenous administration of a therapeutic dose of haem arginate (3 mg haem kg-1) to four healthy volunteers and four symptomless porphyric patients. Plasma haem concentrations were measured also during a treatment course of four infusions in six patients with porphyria. Plasma haem concentrations declined monoexponentially over 48 h in both healthy volunteers and porphyric patients, with a mean +/- s.e. mean elimination half-life of 10.8 +/- 0.6 h. Other kinetic parameters were also similar in the two groups, total plasma clearance was 3.7 +/- 0.4 ml min-1 and volume of distribution was 3.37 +/- 0.34 l. In the multiple dose study the elimination half-life increased significantly, from 11.3 +/- 0.4 h to 18.1 +/- 1.4 h over 4 consecutive days. Plasma haemopexin values decreased with time after a single haem arginate dose. The infusion of haem arginate did not cause thrombophlebitis.

Adult↗
PubMed1985

Genetic heterogeneity in acute intermittent porphyria: characterisation and frequency of porphobilinogen deaminase mutations in Finland.

The occurrence of different porphobilinogen deaminase mutant types in 68 patients with acute intermittent porphyria from 33 unrelated families in Finland was studied with biochemical and immunological techniques. In this fairly homogenous population four different porphobilinogen deaminase mutant types were identified and their frequencies determined. Most (about 80%) of the mutations were cross reacting immunological material (CRIM) negative, including a large kindred with normal erythrocyte porphobilinogen deaminase activities. The remainder of the families had CRIM positive mutations, including an unusual type (type 2) that had an immunoreactive, non-catalytic porphobilinogen deaminase level considerably greater than the maximal theoretical ratio of CRIM to activity of 2.0 for a single mutant allele. Correlations of the amount of residual porphobilinogen deaminase activity and the occurrence of acute clinical manifestations in each mutant type suggested that CRIM positive type 2 patients may have fewer acute symptoms.

Ammonia-Lyases↗
PubMed1985

Ketanserin without effects on basal anterior pituitary hormone secretion in healthy subjects.

The effect of ketanserin, a selective serotonin-2 (5-HT2) receptor blocking agent, on the secretion of anterior pituitary hormones was studied in 4 healthy volunteers. Ketanserin (10 mg) was administered as a slow iv injection and its effect was compared with that of saline. Ketanserin influenced neither the basal plasma levels of HGH, ACTH, TSH, LH or prolactin, nor the plasma levels of T4, T3, cortisol or glucose. Even if a single dose of ketanserin had no hormonal effects, this must also be studied after long term use.

Adrenocorticotropic Hormone↗
PubMed1985

Morbid obesity, gastric plication and a severe neurological deficit.

A 39-year-old man had protracted vomiting after gastric plication for morbid obesity. Within three months he lost 53 kg in weight and developed neuromuscular weakness, especially in the lower extremities. Clinical and laboratory studies suggested both radicular and peripheral neuropathy. One year later the condition was only marginally improved: he took only few steps unsupported. The apparent etiology is malnutrition but the primary cause remained unknown.

Adult↗
PubMed1985

Variant of acute intermittent porphyria with normal erythrocyte uroporphyrinogen-I-synthase activity.

A kindred in which several members have otherwise typical acute intermittent porphyria but normal erythrocyte uroporphyrinogen-I-synthase activity has been described from Finland. We studied two porphyric members of this kindred, two patients with typical acute intermittent porphyria, and two healthy controls using the delta-aminolaevulinic acid loading test and by measuring the erythrocyte enzymes of haem biosynthesis. The excretion pattern of haem precursors after the delta-aminolaevulinic loading test in the members of the kindred studied, was similar to that in typical acute intermittent porphyria suggesting an identical enzyme defect in the liver. The activity of all red cell enzymes studied was normal in the members of the kindred. The results suggest that porphyria in the kindred studied is a variant of acute intermittent porphyria, where the uroporphyrinogen-I-synthase defect is manifested in the liver but not in red cells.

Acute Disease↗
PubMed1985

Acute intermittent porphyria: characterization of a novel mutation in the structural gene for porphobilinogen deaminase. Demonstration of noncatalytic enzyme intermediates stabilized by bound substrate.

To investigate the molecular pathology in acute intermittent porphyria (AIP), the nature of the defective porphobilinogen (PBG)-deaminase was determined in erythrocyte lysates from 165 AIP heterozygotes from 92 unrelated families representing 20 different ethnic or demographic groups. Immunologic and physicokinetic studies revealed the occurrence of four classes of PBG-deaminase mutations. In the majority of families studied, the amount of immunoreactive enzyme protein corresponded to the amount of enzymatic activity, indicating the absence of cross-reacting immunologic material (CRIM) produced by the mutant allele. In 78 of these CRIM-negative families (designated type 1), the affected heterozygotes had half-normal PBG-deaminase activity. In three families (designated CRIM-negative type 2), symptomatic patients had increased urinary excretion of delta-aminolevulinic acid and PBG, and normal levels of erythrocyte PBG-deaminase activity. In contrast, noncatalytic, immunoreactive protein was expressed in heterozygotes from 11 families, about one-eighth of those studied, consistent with mutations in the structural gene for PBG-deaminase. Two types of CRIM-positive mutations were identified: the type 1 mutation had a CRIM/activity ratio of approximately 1.7 and a crossed-immunoelectrophoretic profile in which all the enzyme intermediates were increased, with the B or monopyrrole-enzyme intermediate predominant (B greater than A much greater than C congruent to D greater than E). The mutation altered both the kinetic and stability properties of the noncatalytic immunoreactive enzyme protein. The second CRIM-positive mutation, type 2, had markedly increased levels of noncatalytic immunoreactive protein (CRIM/activity ratio approximately 5.7). Crossed-immunoelectrophoresis revealed markedly increased amounts of the substrate-bound intermediates, B, C, D, and E (B greater than C greater than D greater than E much greater than A). The accumulation of these noncatalytic enzyme intermediates presumably resulted from the enhanced binding and/or defective release of substrate molecules. The conformation of these enzyme-substrate intermediates apparently rendered the complexes more resistant to intraerythrocyte proteolysis. These findings provide evidence for the presence of different allelic mutations in the structural gene for PBG-deaminase and document molecular genetic heterogeneity in AIP.

Acute Disease↗
PubMed1985

Prolactinoma of the pituitary containing amyloid.

A transsphenoidal operation was performed in a 27 year old man because of a large intrasellar expansion and marked hyperprolactinaemia (33 000 mU/l). The tumour found in the operation bulged into the sphenoidal sinus and was extirpated. On histopathological examination the tumour consisted mainly of congophilic amyloid spherules with intermingled sparse adenoma cells. The adenoma cells were strongly positive for prolactin in immunoperoxidase staining, and in electron microscopy they contained secretory granules. Ultrastructurally the amyloid spherules consisted of masses of concentrical extracellular amyloid filament bundles.

Adenoma↗
PubMed1985

Comparison of insulin regimens in the therapy of type 1 diabetes.

The major problems with one or two daily subcutaneous injections of fast and intermediate acting insulins are morning hyperglycaemia and nocturnal hypoglycaemia. These problems can be avoided to a great extent by giving a third injection at bedtime. However, the kinetics of plasma free insulin during these insulin regimens is unphysiological and appropriate meal related plasma insulin peaks cannot be achieved. The new intensified methods of insulin delivery, multiple daily injections (MDI) and continuous subcutaneous insulin infusion (CSII) are more physiological. Consequently, a near normal glycaemic control can be achieved with these regimens; more often with CSII than with MDI. The risk of complications of CSII is on the other hand slightly greater. The importance and need of intensified insulin therapy in the treatment of insulin dependent diabetes is not yet fully settled. At the present it is not a primary form of treatment and indicated only if the conservative insulin regimens fail.

Blood Glucose↗
PubMed1984

Remissions in newly diagnosed type 1 (insulin-dependent) diabetes: influence of interferon as an adjunct to insulin therapy.

We studied the effect of interferon as an adjunct to conventional insulin therapy on the early course of Type 1 diabetes in 43 newly diagnosed patients. Compared with conventional therapy, interferon administration slightly delayed the improvement of glucose homeostasis and the rise of high density lipoprotein cholesterol, while C-peptide secretion was unaffected. Independent of the type of therapy, 18 patients (42%) entered partial remission. The remission began 2.0 +/- 0.6 months (mean +/- SEM) from the start of therapy and lasted for 4.1 +/- 1.1 months. Seven patients (16%) were still in remission 1 year after diagnosis. The patients who entered remission had higher initial C-peptide secretion, lower glycosylated haemoglobin levels and better initial control than patients without remission. Thus, interferon provided no benefits as an adjunct to conventional insulin therapy in unselected patients with newly diagnosed Type 1 diabetes. An important factor for the development of remission was the presence of C-peptide secretion at the time of diagnosis.

Adolescent↗
PubMed1984

Development of Cushing's disease in a patient with anorexia nervosa.

A young woman developed clinical features of anorexia nervosa with a maximal loss of ideal body weight of 54 per cent at the age of 16 yr. A partial recovery from the anorectic symptoms was encountered six years later. At the age of 27 yr pituitary Cushing's disease was diagnosed and corticotroph cell pituitary adenoma subsequently removed by transsphenoidal surgery. Within two years after the operation the clinical and biochemical signs of hypercortisolism had disappeared but some of the anorectic features reappeared. Without neglecting the likelihood of a simple coincidence, the possibility of a common pathogenetic mechanism for anorexia nervosa and Cushing's disease, either affecting the function of the hypothalamic serotonergic pathways or concentration of glucocorticoid receptors, is discussed.

Adenoma↗
PubMed1984

Serum lipoproteins in asymptomatic acute porphyria: no evidence for hyperbetalipoproteinemia.

Hypercholesterolemia and elevated LDL cholesterol have previously been reported to be associated with acute porphyria. We therefore carried out a systematic study on serum lipoprotein levels in asymptomatic patients with acute porphyria. In 15 patients with acute intermittent porphyria and in seven with variegate porphyria, the total serum cholesterol as well as VLDL- and LDL-cholesterol concentrations did not differ from those of matched controls. On the other hand, the mean HDL-cholesterol concentration was significantly higher in patients with porphyria than in controls. Total serum triglycerides and triglycerides in various lipoproteins were normal in patients with porphyria. In contrast to earlier reports, we could not find increase of LDL cholesterol in asymptomatic acute porphyria. The results suggest that elevated HDL-cholesterol levels might occur in this group of porphyrias.

Acute Disease↗
PubMed1984

Long-term outcome after jejunoileal bypass for morbid obesity.

Long-term results after jejunoileal bypass operation for morbid obesity in 47 patients are presented. Two patients died postoperatively and a reanastomosis was made in three. Forty-one patients were re-examined on average 4.7 years after operation. Mean weight loss was 43 kg or 31 per cent of original weight. Increased stool frequency and abdominal discomfort were common postoperative symptoms. Abnormalities in serum electrolytes, serum vitamin levels and in liver function were relatively uncommon and in none serious. The net effect of the operation on joint and back symptoms was positive. In most patients the quality of life was clearly improved and all except two were satisfied with the results. Our experience suggests that jejunoileal bypass is still an alternative in the treatment of morbid obesity.

Adolescent↗