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Biomedical subjects

A Pagliuca

Publications and source records attributed to A Pagliuca.

69 records · Page 4Linked to original sources
PubMed1990

Lead poisoning: clinical, biochemical, and haematological aspects of a recent outbreak.

The clinical, biochemical, and haematological aspects of a recent outbreak of lead poisoning, in which exposure was related to the oxyacetylene cutting of red lead painted ironwork, were investigated. Initial suspicion was raised when a blood film showed punctate basophilia which remains a simple and useful method of picking up lead toxicity. Estimations of blood lead concentration and conventional laboratory data confirmed the diagnosis. Although there was prominent punctate basophilia, spectrophotometric analysis showed only negligible accumulation of pyrimidine-5'-nucleotides despite severe suppression of pyrimidine-5'-nucleotidase activity. The pattern of the red cell glycolytic intermediates, investigated for the first time, suggested that lead may also affect glycolysis at the hexokinase step. Once the diagnosis was made intravenous chelation treatment was begun with a rapid improvement in symptoms. Long term follow up is required to assess any sequelae of intoxication. These cases emphasise the classic features of lead poisoning, and despite the currently available diagnostic tests, lead intoxication may still go unrecognised unless a thorough occupational history is taken.

5'-Nucleotidase↗
PubMed1990

Palmar-plantar erythema associated with combination chemotherapy.

We report three cases of a distinctive palmar-plantar erythema associated with the treatment of non-Hodgkin's lymphoma and acute myeloid leukaemia. The rash is characterized by a painful, sharply demarcated, intense erythema of the palms and/or soles followed by bulla formation, desquamation and healing. The eruption is self limiting in nature and did not adversely affect prognosis. Treatment need be neither altered nor stopped; only symptomatic measures are required.

Adult↗
PubMed1990

Mutation of the human FMS gene (M-CSF receptor) in myelodysplastic syndromes and acute myeloid leukemia.

We studied 41 patients with myelodysplastic syndromes or acute myeloid leukemia to assess the presence of point mutations in the human FMS gene (M-CSF receptor). Using the polymerase chain reaction and hybridization of oligonucleotide probes to the amplified sequences, we have detected mutations in eight of 41 patients, at codons 301 and 969. In vitro work has highlighted mutations at these codons as being oncogenic. We now report the detection of potentially activating mutations of the human FMS gene in vivo. The consequence of these mutations in the multistep pathogenesis of myeloid malignancy and their relevance to prognosis remains to be determined.

Alleles↗
PubMed1990

In vitro colony culture and chromosomal studies in hepatic and portal vein thrombosis--possible evidence of an occult myeloproliferative state.

We have studied the prevalence of an underlying myeloproliferative state in 20 patients with either hepatic or portal vein thrombosis. Using conventional clinical and laboratory criteria, an underlying myeloproliferative state was identified as the cause of the thrombosis in five patients (25%). A further 10 of the remaining 15 cases were found to have characteristic in vitro bone marrow culture studies and cytogenetic abnormalities suggestive of an underlying myeloproliferative disorder. Although none of these 10 cases have developed overt clinical and laboratory features of such a myeloproliferative disorder after a median observation period of two years, the presence of clonal karyotypic abnormalities in three cases, increased megakaryocyte colony growth in three cases and endogenous erythropoietin independent colony growth of the marrow erythroid progenitors in seven cases, argues strongly in favour of a primary haematological disorder. This has important therapeutic implications, particularly in cases being considered for orthotopic liver transplantation.

Adolescent↗
PubMed1990

Fulminant fungal sinusitis following intensive chemotherapy.

Three cases of fulminant fungal sinusitis in patients undergoing intensive chemotherapy for haematological malignancies are described. Predisposing factors included courses of broad-spectrum antibiotics for febrile episodes. The presentation of sinusitis was acute, with facial pain and swelling, progressing to proptosis and visual disturbance. CT scanning is the investigation of choice to assess the extent of disease and subsequent response to treatment. Management requires urgent drainage, systemic and local antifungal therapy and decompressive procedures if necessary. Aggressive management can achieve good local control, but the long-term outlook remains poor.

Acute Disease↗
PubMed1989

Coulter S Plus STKR histograms detect spurious elevation of leucocyte and platelet counts associated with cryoglobulinaemia.

Abnormalities in the size frequency histogram generated by the Coulter S Plus STKR at room temperature raised the possibility of the presence of a cryoglobulinaemia in a man admitted to the hospital with pneumonia. The leucocyte and platelet counts generated by the Coulter S Plus STKR were higher when counts were done at room temperature than counts done at 37 degrees C. Further investigation of the patient revealed the presence of Non-Hodgkin's Lymphoma which had not been suspected on clinical grounds. The importance of testing samples at 37 degrees C, where an abnormality in the Coulter S Plus STKR histogram has been detected at room temperature, is emphasized.

Aged↗
PubMed1989

Myelofibrosis in primary myelodysplastic syndromes: a clinico-morphological study of 10 cases.

We describe 10 cases of primary myelodysplastic syndrome in which marrow fibrosis was striking at presentation. All the cases showed trilineage dysplasia with increased megakaryopoiesis and marked reticulin fibrosis. Significant organomegaly was notably absent. This association has hitherto not been highlighted and it is important to distinguish these cases from those of idiopathic myelofibrosis with which they may be confused. Furthermore, their comparatively long survival distinguishes these cases from those previously described as acute myelodysplasia with myelofibrosis and malignant myelosclerosis. The pathogenesis of fibrosis in these cases may be related to disordered megakaryopoiesis and the platelet-derived cytokines that may be released. The treatment of these fibrotic cases remains problematical and further investigation is required.

Adult↗
PubMed1989

Development of polycythaemia vera in a patient with myelofibrosis.

In March 1981, a 53-year-old man presented with itching and was diagnosed as having myelofibrosis. There was gradual enlargement of the spleen over the following 5 yr. His spleen had to be removed in February 1986 because of physical discomfort. 3 months post-splenectomy he became polycythaemic. Bone marrow examination was consistent with severe myelofibrosis. It was possible to demonstrate erythropoietin-independent BFU-E from peripheral blood, and ferrokinetic studies showed that erythropoiesis was localised to the liver with little bone marrow activity. Thus, despite severe marrow fibrosis, liver erythropoiesis was now polycythaemic, suggesting the coexistence of myelofibrosis and polycythaemia vera.

Bone Marrow↗
PubMed1989

Bilateral adrenal lymphoma presenting as Addison's disease.

We describe an unusual case of non-Hodgkin's lymphoma of the adrenals which presented as Addison's disease. Examination of tissue taken by computed tomography guided biopsy revealed a high grade B cell centroblastic lymphoma. The patient was treated with chemotherapy but died from invasive aspergillosis. Autopsy confirmed bilateral adrenal involvement by lymphoma.

Addison Disease↗
PubMed1985

Recurrent infections in sickle cell disease: haematological and immune studies.

Some haematological and immunological indices were compared in 19 children with sickle cell disease and a history of recurrent infections and in 16 children with sickle cell disease without any known infections. The recurrent infection group had significantly greater pitted red cell counts and greater absolute monocyte counts. No differences were apparent in routine haematological indices, foetal haemoglobin, immunoglobulin, or complement levels between the groups. The interpretation of these results is discussed.

Anemia, Sickle Cell↗
PubMedDate not supplied

Evaluation of goiter endemia by ultrasound in schoolchildren in Val Sarmento (Italy).

Ultrasonography is an excellent and objective method for assessing thyroid volume, especially in children where clinical evaluation is inaccurate. The aim of this study was to evaluate the presence of goiter by thyroid ultrasound and palpation in 244 schoolchildren, 6 to 14 years old, living in some rural villages of Val Sarmento, a mountain area of Basilicata, Italy. In 1996 we revealed the presence of endemic goiter in 25% of the schoolchildren evaluated by palpation, according to World Health Organization (WHO) criteria, and in 15.9% of the schoolchildren evaluated by ultrasonography (7.5 MHz linear probe). The median urinary iodine excretion, taken from an extemporaneous sample of the first urines in the morning, was 62.2 microg/l. This study includes Val Sarmento, an area with mild-moderate grade (Grade I) of iodine deficiency, suggesting the need for iodine prophylaxis. Furthermore, it proves that the measurement of thyroid volume by ultrasonography is an essential instrumental method for a correct epidemiological study of endemic goiter, particularly in areas where there is mild iodine deficiency.

Adolescent↗