Biomedical subjects
C Micalizzi
Publications and source records attributed to C Micalizzi.
Mucin deposits in morphea and systemic scleroderma.
BACKGROUND: Though rarely reported, mucin deposition may be observed in scleroderma. OBJECTIVE: To verify the frequency of significant amounts of mucin in the biopsy specimens. METHODS: Biopsies from 20 patients with scleroderma were reviewed and stained to verify the presence of mucin. RESULTS: Mucin deposits were found in all of the 20 specimens. CONCLUSION: Mucin deposition is probably a constant feature in both morphea and systemic scleroderma. Its relevance in differential diagnosis between scleredema and scleroderma is debatable.
Heterogeneity of polymorphous light eruption: a study of 105 patients.
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Chronic childhood neutropenia: studies on the in vitro clonogenicity of bone marrow myeloid progenitors.
Bone marrow mononuclear cells (MNC) from 6 pediatric patients with chronic neutropenia were tested for myeloid colony formation upon stimulation with the supernatant of the 5637 cell line or with recombinant granulocyte-macrophage colony-stimulating factor or interleukin 3. Heterogeneous patterns of response of myeloid progenitors were observed in the individual patients, with no colony growth in 2 cases and abnormalities of colony formation or composition in two additional cases. Morphologic and surface marker analyses showed that the bone marrow of some patients contained an excess of lymphocytes with an altered subset distribution. In order to investigate whether or not there was a relationship between the latter abnormality and the observed clonogenic defects, marrow MNC were tested for myeloid colony formation before and after lymphocyte depletion. No evidence for a cell-mediated suppression of colony growth was obtained; likewise, patient sera failed to inhibit colony formation by normal bone marrow myeloid progenitors. Taken together, these data make it unlikely that, in our cases, immunologic mechanisms were responsible for the pathogenesis of chronic neutropenia.
Incidence in Italy, genetic heterogeneity, and segregation analysis of cystic fibrosis.
Taking advantage of the availability of an archive of consanguineous marriages that gives accurate estimates of consanguinity in Italy, it has been possible to calculate the increase of first- and second-cousin marriages among 624 couples of cystic fibrosis (CF) parents over the general population. From these estimates, the incidence of CF in Italy has been found to correspond approximately to 1/2,000. In turn, the same data have been used to test the hypothesis of genetic heterogeneity of CF, recently proposed, which is based on the presence of two distinct genetic disorders having similar frequencies. If such a hypothesis were true, the number of first-cousin marriages among CF parents should be significantly higher than that observed in our present study. Finally, the segregation analysis of 624 CF sibships has yielded under multiple selection a segregation ratio of 0.252, confirming the recessive mode of inheritance.
[The significance of 2-thiol-propionamido-acetic acid in the treatment of cystic fibrosis].
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Treatment with liposomal amphotericin B of a child affected with drug-resistant visceral leishmaniasis.
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Childhood systemic lupus erythematosus: a review of 30 cases.
We review 30 cases of pediatric systemic lupus erythematosus followed over an 8-year period at our institution. The female to male ratio was 3.3:1; the age at diagnosis ranged between 3.5 and 16 years. On first admission, renal involvement was detected in the majority of the patients, as assessed by laboratory findings and/or clinical manifestations. Other frequently observed symptoms were fever, skin rashes, arthralgias and/or arthritis and serositis. All of the patients were treated with corticosteroids and most of them also received immunosuppressive drugs in order to control disease activity. Two patients were lost to the follow-up, five died and only one of the 23 evaluable patients is off therapy after a median follow-up of 5 years. This study confirms that pediatric systemic lupus erythematosus is a very aggressive disease.
New clinical aspects of Pearson's syndrome. Report of three cases.
We describe 3 patients affected by Pearson's syndrome, presenting anemia, exocrine pancreas failure, and skeletal abnormalities; insulin-dependent diabetes mellitus arose in two cases during the course of the disease. Bone marrow dysplasia and exocrine pancreas failure are also reported in Shwachman's syndrome; the two forms differ in bone marrow morphology. The clinical pattern of Pearson's syndrome can be so polymorphic as to increase the difficulties of differential diagnosis with Shwachman's syndrome.
[Human monocomponent insulin in the metabolic control of diabetes mellitus in pediatric patients].
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[Sepsis related to a permanent central venous catheter in children with neoplastic disease: practical implications of a continuous surveillance of the etiology].
Indwelling central venous catheter-related bacteremias are an important complication in patients with cancer. In general they are due to Staphylococcus aureus and Candida, while bacteremias caused by Gram-negatives are less common and often related to infusate contaminans. We describe a survey of etiological surveillance of Broviac catheter-related infections at G. Gaslini Children's Hospital of Genoa, Italy. In the period 1989-1992 an increase of Broviac catheter-related bacteremias due to Gram-negatives was demonstrated as compared with previous years (1985-1988). At home parental management was suspected as an important risk factor, since this complication was frequently due to infusate contaminants and no epidemic cluster or positive surveillance culture was observed in the Hospital. Therefore at home management was changed, especially regarding heparin storage. The subsequent, prospective follow-up from July 1993 to December 1995 showed a significant decrease in catheter-related bacteremias due to Gram-negatives (P = 0.003, chi-square test). In conclusion, a strict control on at home catheter management procedures must be maintained in order to reduce the risk of indwelling central venous catheter-related infections in children with cancer.
[Leukemia in pediatric age].
The prognosis for children with leukemia has improved dramatically in the last 20 years based on prospective clinical investigations of best choice and scheduling of combinations of several agents, considering the risk factors of each patient. The authors review childhood leukemia with respect to epidemiology, classification, laboratory findings (including studies of morphology, cytochemistry, immunologic surface markers, biochemical cytoplasmic markers, cytogenetics with the most frequent chromosome anomalies, molecular biology, immunoglobulin gene and T cell receptor gene rearrangements), clinical manifestations, differential diagnosis, prognostic factors treatment. In respect of patients, it is actually followed the concept of a "total therapy", which includes the purpose the eradicate the invading leukemic cells preserving the expression of normal progenitors, and the psychological and social aspects. The authors consider the clinical trials, the role of bone marrow transplantation (BMT), the supportive care, the evaluation of the minimal residual disease, the study of sequelae and late effects in the survivors, the ethical aspects.