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Biomedical subjects

J Westin

Publications and source records attributed to J Westin.

At least 91 records · Page 5Linked to original sources

Trisomy 1q in polycythemia vera and its relation to disease transition.

Clinical and cytogenetic details of 12 patients with polycythemia vera and complete or partial trisomy of the long arm of chromosome 1 are reported. All patients had trisomy for at least the segments 1q22 to 1qter. The 1q or material from 1q was translocated to another chromosome in eight patients. This was chromosome 9 in four patients, and those cases all had trisomy also for 9p. The trisomy 1q was found at the time of diagnosis in three patients, later during the polycythemic phase in five, and in four patients when they were first examined during a late stage of the disease. Acute leukemia or a myelodysplastic syndrome developed in eight of the 12 patients. Signs of advanced disease, eg, myeloid metaplasia or myelofibrosis, preceded the leukemia in four cases and was noted in one more patient. Trisomy 1q was the most frequent structural chromosome abnormality in patients with polycythemia vera. It is thus one of several nonrandom abnormalities that can appear at any stage of the disease. It seems to occur with higher frequency in patients with myelofibrosis and/or leukemia, but it is not a specific characteristic of these complications.

Adult↗

Primary gastrointestinal lymphoma incidence, clinical presentation, and surgical approach.

During a 5-year period, 15 cases of primary gastrointestinal lymphoma, corresponding to 14% of the non-Hodgkin's lymphomas, were diagnosed in a population of 200,000. Most patients had a short duration of symptoms--less than 3 months--and a clinical picture dominated by abdominal pain. All patients were laparotomized and six patients presented with an acute abdomen and were subjected to emergency operations. In seven cases, gastrointestinal malignancy was not suspected preoperatively. The patients were treated with a combination of surgery, cytostatic therapy, and/or radiotherapy. Cases with localized disease had a favorable prognosis, seven of nine patients being alive 31 to 70 months after diagnosis. A statistically significant difference in median survival was also noted between patients with lymphomas of low-grade and high-grade malignancy (70+ vs. 10.5 months).

Abdomen, Acute↗

Malabsorption of vitamin B12 in dermatitis herpetiformis and its association with pernicious anaemia.

Serum vitamin B12 concentrations were determined in 129 patients and a pentagastrin test performed in 116 patients with dermatitis herpetiformis (DH). Low serum vitamin B12 levels were found in 27 patients (21%) and achlorhydria in 30. Forty-two patients with achlorhydria and/or vitamin B12 deficiency were subjected to further haematological and gastrointestinal investigations. Functional or morphological involvement of the small intestine was seen in almost all patients with vitamin B12 deficiency but, in addition, atrophic gastritis resulting in defective secretion of intrinsic factor (pernicious anaemia) was found in 8 cases (7% of all DH patients). Thus, a low vitamin B12 level in DH patients should result in a careful gastrointestinal examination as both an atrophic gastritis and a coeliac-like enteropathy have further clinical implications.

Achlorhydria↗

Cyanocobalamin absorption in the elderly: results for healthy subjects and for subjects with low serum cobalamin concentration.

We examined the intestinal absorption of cyano[57Co]cobalamin from a non-protein-bound test dose given to 38 subjects from a population of elderly. The subjects were 76 years old and were apparently free from conditions known to affect cyanocobalamin absorption. Their gastric mucosal function was normal, as judged from determinations of serum gastrin and pepsinogen I. The urinary excretion of radioactivity during the first 24 h was 24(SD 7)%, range 8.6 to 45.2%, corresponding to a health-associated reference interval of 10 to 38%. The results indicate that cyanocobalamin absorption does not decline during normal aging. Duplicate studies were performed in another 20 subjects (70-81 years old) from the same population study; these subjects had a serum cobalamin concentration less than 130 pmol/L. The imprecision (CV) was 23%.

Aged↗

Karyotypic evolution in Ph-positive chronic myeloid leukemia in relation to management and disease progression.

In a prospective study of 32 patients with chronic myeloid leukemia the frequency of chromosome abnormalities in addition to the Philadelphia chromosome (Ph) increased when the disease progressed. Before metamorphosis, 10 patients (31%) had developed additional abnormalities. Such abnormalities were present in three of them at the time of diagnosis; in the other seven, they were detected late in the chronic phase. New clonal abnormalities heralded or accompanied a more malignant phase of the disorder, usually a blastic leukemia. During metamorphosis, 78% of the patients had additional abnormalities, which in 68% of these cases comprised at least one of +8, +22q- or i(17q). Clones with additional abnormalities disappeared in eight cases, either spontaneously or in association with cytostatic therapy during the chronic or blastic phase. Involvement of chromosome #8, usually in the form of a trisomy, was found in 7 of 12 patients treated with busulfan, but was not found in any of the 10 hydroxyurea-treated patients, of whom 8 were splenectomized early during the chronic phase. Cells from the spleen, obtained by fine needle aspiration or splenectomy were cytogenetically examined in 18 cases during the chronic phase, but abnormalities in addition to the Ph were noted in only one patient, who was examined in the late chronic phase. The same abnormalities were present in bone marrow cells of this patient.

Adult↗

Plasma levels of luteinizing hormone and steroid hormones in free-living winter groups of willow tits (Parus montanus).

Plasma levels of LH, DHT, testosterone, and corticosterone were measured for all members in free-living winter flocks of willow tits, Parus montanus. Hormonal data were related to (1) flock size and (2) age/sex differences. The winter flock defends a large winter territory and shows a well-established social hierarchy in which adults consistently dominate first-year birds. One winter group normally consists of four individuals, two adults and two juveniles. In flocks containing four or five members juvenile willow tits had significantly higher corticosterone values than adults. In small-sized groups, containing three members, all individuals had high plasma levels of corticosterone. No other effects of flock size was found. When data were treated on an age/sex basis, i.e., flock size was not considered, juvenile females were found to have significantly higher plasma levels of testosterone than adult birds, and also significantly higher levels of DHT than juvenile males and adult females. Also, juvenile willow tits had significantly higher plasma levels of corticosterone than adult birds.

Age Factors↗

Histamine symptoms and histamine metabolism in chronic granulocytic leukaemia.

Histamine metabolism was investigated in three patients with chronic granulocytic leukaemia (CGL) during symptoms suspected to be caused by histamine release. Plasma histamine levels were excessively high compared with periods when the patients were symptomless or with CGL patients without such symptoms. The patients seemed to be adapted to high plasma histamine levels as the symptoms were not typically systemic in nature, but rather localized phenomena like oedema and pruritus in the extremities. Also CGL patients without symptoms showed abnormally high plasma histamine concentrations, which were significantly related to the whole blood histamine concentration. The possibility must be considered that part of the plasma histamine increase is artificial due to rupture of the basophils during the blood collection procedure.

Basophils↗

Cytogenetic studies of bone marrow and extramedullary tissues and clinical course during metamorphosis of chronic myelocytic leukemia.

Of 33 consecutive patients with chronic myelocytic leukemia, examined during metamorphosis, 82% showed chromosome abnormalities in addition to the Ph1. Aberrations most frequently encountered were +8 (39%), +22q - (30%), and i(17q) (18%). Translocations other than the Ph1 were observed in four cases and - Y clones in four cases. Discrepancies in the cytogenetic pattern between bone marrow and extramedullary tissues or blood were noted in a total of 15 patients. In six cases, transformation occurred in extramedullary organs at a time when it was not present in the marrow. In three cases the bone marrow transformation was preceded by a lymph node blastic infiltrate; in one case, by a skin infiltrate; and in one case, by a subdural blastoma. Clonal abnormalities additional to the Ph1 were identified in the tumor tissue from all these cases. Patients with primary extramedullary transformation tended to have a lower median age at onset of metamorphosis, shorter survival, and higher incidence of chromosome abnormalities than the cases without extramedullary involvement. Patients with only Ph1-positive cells and no other anomalies had a slightly longer duration of metamorphosis and longer total survival. Basophilia and thrombocytopenia were more marked in cases with i(17q) than in the rest of the series.

Adult↗

Renal function in patients treated with tobramycin-cefuroxime or tobramycin-penicillin G.

In order to evaluate the potentiating effect of cefuroxime on tobramycin nephrotoxicity 21 immunocompromised patients with suspected septicaemia were randomized to treatment with either tobramycin + cefuroxime or tobramycin + penicillin G. 51Chromium-EDTA clearance, serum creatinine, serum beta 2-microglobulin, urinary, alanine aminopeptidase, urinary N-acetyl-beta-D-glucosaminidase and urinary beta 2-microglobulin were measured during a nine day course of the antibiotic combinations. Enzymuria and a small but significant decrease of 51Chromium-EDTA clearance of equal magnitude in both treatment groups occurred. However the results cannot be extrapolated to other doses and treatment times than used in the study.

Cefuroxime↗

Hereditary ring sideroblastic anaemia and Christmas disease in a Swedish family.

The association of hereditary ring sideroblastic anaemia with Christmas disease in a Swedish family is described. We have studied the transmission of the sideroblastic trait, in relation to HLA groups and Christmas disease, and also evaluated the erythrocyte morphology, uroporphyrinogen-I-synthetase activity and S-ferritin for the detection of latent cases of ring sideroblastic anaemia. The proband had ring sideroblastic anaemia, Christmas disease and haemochromatosis. 3 cases of ring sideroblastic anaemia were found among the 12 family members studied. Using the factor IX deficiency as a marker of the X chromosome, it appeared that autosomal transmission of the sideroblastic trait was most likely. The sideroblastic trait did not seem to be linked to HLA-A3-alloantigen. Erythrocyte morphology was normal in all non-anaemic subjects. S-ferritin was found to be increased in all 3 cases of sideroblastic anaemia as well as in 1 non-anaemic relative. Erythrocyte uroporphyrinogen-I-synthetase was elevated in 10 of the 12 family members; those with sideroblastic anaemia had the highest values indicating that uroporphyrinogen-I-synthetase is of importance in the disturbed haem-synthesis of ring sideroblastic anaemia. This interpretation is supported by the positive correlation between S-ferritin values and the uroporphyrinogen-I-synthetase activity.

Anemia, Sideroblastic↗

Monoclonal gammopathy--a diagnostic challenge. Illustrated by four cases of multiple myeloma with an unusually indolent (smoldering) course.

Four patients with IgG multiple myeloma (MM) and an unusually indolent course are presented. They had received no cytostatic therapy for 12--23 years. The M-component was initially 25--33 g/l, the bone marrow plasma cell percentage was 4--6%, but bone lesions were not demonstrated. A marked increase in size of the M-component and in the plasma cell percentage was noted in all patients, but a definitely neoplastic proliferation in only one patient. Spontaneous regression of the M-component was observed in two patients. In the light of the present cases we believe that neither a high level of the M-component nor an increase in its size should be used alone as indicators of a malignant course in MM patients. An indolent, non-progressive variant obviously occurs as one extreme in the clinical spectrum of this disorder with a course sometimes extended over 10--20 years.

Aged↗

The effect of single giant interneuron lesions on wind-evoked motor responses in the cockroach, Periplaneta americana.

In order to determine the contribution of individual giant interneurons (GIs) to wind-evoked motor outputs, responses were recorded from depressor and levator motor neurons in the cockroach Periplaneta americana to wind puffs of different directions. The depressor response was generally stronger to wind from the ipsilateral rear than to wind from the contralateral rear. The levator response was more variable but was more often stronger to wind from the contralateral rear than to wind from the ipsilateral rear. These results are as expected based on behavioral responses to wind puffs. (Camhi and Tom, 1978). The depressor response was nearly or totally abolished by severing the ipsilateral connective of the nerve cord but was little affected by severing the contralateral connective. The levator response was greatly reduced for some angles by severing the ipsilateral connective and for other angles by severing the contralateral connective. Blocking conduction in the ipsilateral GI 5 nearly or completely abolished the depressor response to wind from the ipsilateral rear. This is the first time that a GI has been shown to be necessary, not just sufficient, for a given motor output.

Animals↗

Rearrangement of chromosome no. 3 in a case of preleukemia with thrombocytosis.

The clinical and cytogenetic findings of a patient with the preleukemic syndrome and a structural rearrangement involving both chromosomes No. 3 are described. The karyotypic abnormality consisted of an insertion of a part of the long arm of one chromosome No. 3 into the other, i.e., ins(3;3)(q27;q21q27). A prominent feature of the bone marrow was a marked megakaryocytic hyperplasia. The platelet count temporarily exceeded 1000 x 10(9)/liter. The findings of the present case, together with similar observations by others, suggest that the long arm of chromosome No. 3 may contain a region involved in the regulation of megakaryopoiesis.

Bone Marrow Cells↗

Bone scintigraphy in the diagnosis of skeletal involvement and metastatic calcification in multiple myeloma.

The contribution of bone scintigraphy to the diagnosis of skeletal involvement in multiple myeloma was evaluated in a consecutive, unselected series of 25 previously untreated patients. Definite scintigraphic abnormalities were found in 11 patients (localized in 4, generalized in 7) (44%). In the majority of patients the clinical value of the information gained from scintigraphy was roughly equal to that found by radiography. However, 2 patients with normal and 2 with questionable bone scans had X-ray evidence of skeletal involvement, and the extent of bone destruction in 4 cases was seriously underestimated in the bone scans. Small osteolytic lesions were as a rule not detected by scintigraphy. On the other hand, in 8 patients the bone scan added information of involvement of ribs, pelvis and vertebrae, not clearly visualized by X-ray. In 2 patients, both with IgA myeloma, hypercalcemia and uremia, a massive extraskeletal uptake of the bone-seeking isotope was observed. Autopsy verified in one case the presence of a disseminated metastatic calcification.

Adult↗