[Diagnosis criteria in diabetes mellitus].
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Diabetes Mellitus↗
Biomedical subjects
D Damiani
Publications and source records attributed to D Damiani.
[Thyroid carcinoma in children and adolescents - review of six cases]
OBJECTIVE: Childhood thyroid carcinoma is a rare entity whose management is controversial. The objective of the present study was to evaluate the characteristics of these patients in terms of diagnosis and evolution. PATIENT AND METHODS: The evolution of six patients with thyroid cancer, followed at a Pediatric Endocrinology Unit during the past 17 years, was retrospectively reviewed. RESULTS: Six female patients with age ranging from 4.5 to 12 years were studied. In all 6 cases, thyroid nodules were present on the initial evaluation. Ultrasonography and 131I scintigraphy showed solid and cold nodules in four patients. Histologic findings indicated four papillary and two follicular carcinomas. All patients were submitted to total thyroidectomy; four were subsequently submitted to radiodine therapy due to the presence metastases and/or residual thyroid tissue. CONCLUSION: Our findings support the notion that children and adolescents with thyroid carcinoma have a positive prognosis; no cases of death occurred after 17 years of follow-up. Our data are in agreement with the literature, which describes low mortality rates for these cases.
Journal Article↗
[Childhood hypoglycemia - still a challenge].
OBJECTIVE: Hypoglycemia in the infant is potentially hazardous to the central nervous system(CNS), and any delay in solving the problem may impose irreversible sequelae. The aim of this review is to provide basic knowledge to the pediatrician to adequately deal with a hypoglycemic patient. METHODS: Bibliographic review was performed in the Medline for the last 10 years, and the most pertinent papers were selected. RESULTS: Even today, there are discrepancies as for the best conduct in the hyperinsulinemic patients. The surgical or clinical approaches have had the support of very important authors. Some state that the best choice is the total pancreatectomy, in which 95% of the pancreas is removed, with all its long-term sequelae, while others emphasize the preservation of the pancreas with drug therapy instead of pancreatectomy. CONCLUSIONS: The doctor who takes care of a hypoglycemic child has to remember that the biggest task is to preserve the CNS integrity, and all the efforts must be made to provide an adequate glucose supply to the brain, otherwise irreversible consequences will ensue.
English Abstract↗
[Persistent hyperinsulinemic hypoglycemia of infancy: case report]
OBJECTIVE: To report a case of Persistent Hyperinsulinemic Hypoglycemia in twins which is a situation not yet reported in the literature. METHODS: Report of seizures in identical twins, from consanguineous parents, with persistent hypoglycemia as cause of the seizures. Laboratory tests, performed for etiological investigation of the hypoglycemia, included thyroid hormones (T4/TSH), insulin, cortisol, growth hormone, stimulation test with glucagon (to evaluate the insulin/glucose relation), and histopathological study of the pancreas. RESULTS: Laboratorial investigation revealed a persistent hypoglycemia with hyperinsulinism which were confirmed with the stimulation test with glucagon. The histopathological exam showed a persistence of first generation pancreatic islet, confirming the diagnosis of Persistent Hyperinsulinemic Hypoglycemia in Infancy (the new denomination of Nesidioblastosis). CONCLUSION: Although rare, this condition must be early suspected early in the evaluation of hypoglycemia of the young infant, even out of the neonatal period, specially if the parents are consanguineous. The adequate therapy must be quickly initiated in order to prevent neurological damage.
Journal Article↗
[True precocious puberty in girls].
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Comment↗
[Diabetic ketoacidosis - revisiting the basic concepts]
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Editorial↗
Castleman's disease: an unusual cause of mediastinal mass and anemia.
We reported a case of thoracic localized, mixed variant, Castleman's Disease (angiofollicular lymph node hyperplasia) with a favourable clinical course presenting with mediastinal mass, microcytic anemia, mild thrombocytosis, polyclonal hyper-gammaglobulinaemia and without symptoms. Castleman's Disease must be always considered in the differential diagnosis of solitary mediastinal masses. Transthoracic fine needle aspiration cytology is usually nondiagnostic; in fact, cytologically, Castleman's Disease can be confused with other mediastinal lymphoproliferative disorders (such as thymomas or lymphomas). The optimal therapeutic approach is unknown but a complete surgical resection is treatment of choice for localized disease.
Adult↗
[Comparison between biopsy data and the resistive index obtained by Doppler echography in the evaluation of the transplanted kidney].
Doppler sonography is nowadays considered as a "first step" tool for diagnosis of vascular complications in kidney transplantation. Quite recently, it has been sometimes considered useful and effective investigation in order to obtain information about parenchymal dysfunctional pathologies, particularly about acute rejection. This has been obtained by studying the variation of resistive indexes. The goal of the following investigation was compare Doppler sonography data and histological examination in 50 kidney transplanted recipients in whom the renal biopsy was performed on a clinical basis. In the Authors' experience. Doppler sonography and study of the resistive index does not offer any reliable help in differentiating acute rejection from cyclosporine A toxicity.
Biopsy↗
Cancer chemotherapy does not enhance MDR-associated 170 kd glycoprotein expression in normal blood mononuclear cells.
BACKGROUND: The multidrug resistance (MDR) associated 170kd glycoprotein (P170) is expressed at a low level in normal and malignant cells, but in the latter it becomes frequently overexpressed after chemotherapy. This study evaluated P170 expression in normal blood mononuclear cells after and during cancer chemotherapy. METHODS: P170 was detected by immunocytochemistry with two monoclonal antibodies (MRK-16 and JSB-1). RESULTS: P170 expression was low in all samples before, during and after chemotherapy. CONCLUSIONS: Cancer chemotherapy did not enhance P170 expression in normal peripheral blood mononuclear cells. The mechanisms enhancing P170 expression are likely to be more operative in tumor cells than in normal cells.
ATP Binding Cassette Transporter, Subfamily B, Mem↗
Proliferation and maturation effects of in-vivo granulocyte-macrophage colony stimulating factor in acute non-lymphocytic leukaemia.
BACKGROUND: Granulocyte-macrophage colony stimulating factor (GM-CSF) can affect the treatment of acute non-lymphocytic leukaemia (ANLL) by supporting normal haemopoiesis and by enhancing the proliferation and the maturation of leukaemic cells. METHODS: A human recombinant E. Coli synthesized GM-CSF was administered to 7 patients with ANLL at a dose of 5 or 10 micrograms/kg/day for 7 days, prior to antileukaemic treatment. Peripheral blood neutrophil and blast cell count was monitored daily. Changes in marrow blast cell morphologic features, mitotic index, and the reactivity to a panel of monoclonal antibodies were assessed after 3 and 7 days of treatment. RESULTS: Peripheral blood neutrophil and blast cell count increased in 6/7 cases. The mitotic index of marrow cells increased in 6/7 cases. A significant maturation occurred along the granulocytic line (2 cases) and the monocytic line (1 case). Mild to moderate eosinophilia developed in 3/7 cases. Early stem cell markers (CD 34 and HLA-DR) were not lost, or actually increased. Myelomonocytic markers (CD 33, CD 13, and CD 14) rose and fell. Expression of the multidrug resistance-associated 170 kd glycoprotein remained stable. CONCLUSIONS: These data showed that in vivo GM-CSF more consistently enhanced the proliferation than the maturation of leukaemic cells.
Adult↗
[Substitutive therapies in sepsis and acute renale failure].
Substitutive treatment of sepsis associated acute renal failure is an emergent challenge in the intensive care unit due to the number of cases and to the high mortality rate. Standard hemofiltration is unable to improve survival, since a high mortality rate is sustained by the septic process. New therapeutic approaches currently available are based on the increased clearance of molecules ranging 10-30 kDa considered important in the physiopathology of sepsis and multiorgan failure. Clinical experiences in progress are: (1) adsorption resins able to bind bacterial products, cytokines, anaphylotoxins and several inflammation mediators; (2) the bioartificial kidney, that is the addition to hemofilter of human tubular cell culture grown in devices in order to mimic metabolic tubular function to a traditional hemofilter; (3) increased exchange volumes (high volume hemofiltration), up to 0-100 L/24 hr and; (4) increased membrane permeability associated with either discarded ultrafiltrate (high cut-off membranes) or plasma substitution plasmapheresis with regeneration by sorbents technology (C FA). Generally, by applying these new technologies to septic shock patients, the observed survival was higher than that predicted by the gravity score. While these results are encouraging, they are not conclusive and need further study.
Acute Kidney Injury↗
HTLV-I positive adult T-cell leukaemia-lymphoma: report of a typical case from Italy.
A case of acute adult T-cell leukemia-lymphoma (ATLL) was observed in northeast Italy, presenting with fever, lymphadenomegaly, splenomegaly, hypercalcemia and renal failure. Leukaemic cells were morphologically typical, expressed a T-cell CD4+ phenotype, did not display any helper functions, and grew in vitro under supply of exogenous interleukin-2. Antibodies to human T-cell lymphotropic virus (HTLV-I) were found in the serum, and the virus was isolated from leukaemic cells. The family members who could be tested were seronegative. The patient had never travelled outside Italy, had never received blood transfusions and did not belong to any known categories at risk of viral disease transmission. Present knowledge of the epidemiology of HTLV-I infection warns that other cases of HTLV-I induced disease are expected to occur outside already recognised endemic areas. This case suggests that untraceable, presumably short-term exposures can also account for HTLV-I transmission.
Adult↗
Sequential treatment of Ph-positive chronic myeloid leukemia with interferon gamma and interferon alpha.
Several clinical observations have shown that alpha-IFN is presently the most interesting investigational agent for the treatment of Ph+ chronic myeloid leukemia (CML). Gamma-IFN is also effective, and experimental data as well as preliminary clinical observations suggest that the combination of the two IFNs is worth investigating. No comparative data are available on the effects of the two IFNs, given alone, in the same patients. In this study 11 patients with PH+ CML were first treated with gamma-IFN, up to a maximum period of 35 weeks, and after a short rest period were retreated with alpha-IFN. Both IFNs were ineffective in 3 patients in accelerated or instable chronic phase. Both IFNs were equally effective in 8 patients in stable chronic phase, but none of these patients achieved a karyotypic conversion with either IFN. This study did not show any measurable differences in the therapeutic response to gamma-IFN and alpha-IFN given consecutively to the same patients.
Adolescent↗
Serum lactate dehydrogenase is an important risk determinant in acute lymphocytic leukemia.
Pretreatment serum total lactate dehydrogenase (LDH) activity was measured in 341 adult patients (greater than 15 years old) with acute lymphocytic leukemia (ALL) in order to assess its prognostic value. Failure, death during induction and remission were not related to LDH. In contrast, a negative and continuous relationship was found between LDH and relapse-free survival. Though LDH activity was significantly higher in cases with already established risk factors such as high WBC count, FAB L3 cytotype, mature B-cell phenotype, and central nervous system involvement, LDH was confirmed to be the strongest predictor of remission duration by multivariate analysis. This study provides a definitive confirmation of the negative prognostic value of LDH in adult ALL.
Adolescent↗
Restoring uptake and retention of daunorubicin and idarubicin in P170-related multidrug resistance cells by low concentration D-verapamil, cyclosporin-A and SDZ PSC 833.
BACKGROUND: Overexpression of the mdr-1 gene that codes for a 170 Kd transmembrane glycoprotein (P170) is a factor responsible for decreased cell sensitivity to anthracyclines and other drugs, and is related to treatment failure in acute leukemia and other tumors. Several agents, including verapamil and cyclosporine derivatives, can modify P170-related resistance in vitro and can be proposed as adjuvant treatment for leukemia and cancer. MATERIALS AND METHODS: To investigate the optimal conditions for adjuvant treatment, D-verapamil (D-VRP), cyclosporin-A (CyA) and the cyclosporine derivative SDZ PSC 833 (PSC) were used alone and in combination at drug concentrations that can be achieved and maintained in vivo. The drugs were tested for their capacity to restore intracellular daunorubicin (DNR) and idarubicin (IDA) accumulation in high-resistance (CEM VLB 300) and intermediate-resistance (CEM VLB 100) cells to levels found in the non-resistant parental cell line (CCRF CEM). RESULTS: In intermediate-resistance cells, IDA alone was more easily restored than DNR plus modifiers, and intracellular IDA concentration was returned to the level of non-resistant cells with low-dose D-VRP (1 microM), CyA (0.8 microM) and PSC (0.4 microM). In high-resistance cells no modifier or modifier combination was able to restore intracellular DNR concentration to the value of non-resistant cells. Intracellular IDA concentration was almost completely restored only with D-VRP (2-3 microM) and CyA (0.8-1.6 microM) in combination or with PSC alone (0.4 microM). CONCLUSIONS: These data suggest that as far as P170-related resistance is concerned, IDA alone is as efficient as or even more efficient than DNR plus modifiers, and that residual resistance to IDA can be overcome with a combination of D-VRP and Cy-A at a clinically achievable concentration, or with a more powerful modifier like SDZ PSC 833.
ATP Binding Cassette Transporter, Subfamily B, Mem↗
The expression of the multidrug resistance related glycoprotein in adult acute lymphoblastic leukemia.
BACKGROUND: More than 50% of adults with acute lymphoblastic leukemia (ALL) actually die with resistant leukemia. The multidrug resistance (MDR) related P170 glycoprotein may be involved in treatment failure. METHODS: P170 content of leukemic cells was assayed by immunocytochemistry with the MRK-16 monoclonal antibody in 41 consecutive cases of adult ALL (27 at onset and 14 at relapse). RESULTS: Positive cells were found in 11/14 cases at relapse and in 18/27 cases at onset. All cases with a high WBC, FAB L3, B-mature phenotype, and t(9; 22) were positive. In the 27 patients who were studied at onset and were treated with the same protocol, the overall failure rate was 44% in negative cases and 83% in positive cases. CONCLUSION: P170 overexpression may contribute to drug resistance and to chemotherapy failure in adult ALL.
ATP Binding Cassette Transporter, Subfamily B, Mem↗
p170-dependent multidrug resistance. Restoring full sensitivity to idarubicin with verapamil and cyclosporin A derivatives.
BACKGROUND: Cell sensitivity to anthracyclines and other drugs depends on several factors, including overexpression of a 170Kd transmembrane glycoprotein (P170) that enhances drug efflux from the cells. Since the result of treatment is negatively related to the expression of P170 in leukemia, malignant lymphoma and other tumors, it is important to investigate drugs and methods that can modify multidrug resistance (MDR). MATERIALS AND METHODS: Using an MTT-microcultured tetrazolium colorimetric method, we assayed sensitivity to daunorubicin (DNR) and to its 4-demethoxy derivative idarubicin (IDA) in two MDR cell lines (CEM VLB and LOVO DX) and in their respective non-MDR parental lines (CEM and LOVO 109), with and without three MDR modifiers, namely the D-isomer of verapamil (DVRP), cyclosporin A (CyA) and the new CyA derivative SDZ PSC 833. RESULTS: We showed that down-modulation of resistance with MDR modifiers was greater for DNR than for IDA in MDR cells. However, we also demonstrated that restoration of full sensitivity could only be achieved for IDA, not for DNR. DVRP and CyA in combination were more effective than either compound alone and could abolish P170-related resistance to IDA at concentrations of 1-2 microM and 1.6 microM, respectively. SDZ PSC 833 alone was even more effective and set MDR to zero at a concentration ranging between 0.8 and 1.6 microM. CONCLUSIONS: These data suggest that combinations of IDA and MDR modifiers may improve the results of cancer and leukemia treatment and that they are worth investigating in vivo, with attention to possible effects on drug pharmacokinetics and on normal tissue damage.
ATP Binding Cassette Transporter, Subfamily B, Mem↗
Overexpression of MDR-related p170 glycoprotein in chronic myeloid leukemia.
BACKGROUND AND METHODS: Philadelphia (Ph) positive chronic myeloid leukemia (CML) cannot be induced into a true remission with conventional chemotherapy. Blast cells and precursors obtained from 51 Ph+ CML cases were assayed for expression of the multidrug resistance (MDR)-associated glycoprotein (p170) by immunocytochemistry (APAAP) with the MRK-16 monoclonal antibody. RESULTS AND CONCLUSIONS: Positive cells were found in 11/17 cases in chronic phase (65%), in 4/8 cases in accelerated phase, and in 23/26 cases in blastic phase (89%). The proportion of positive cells, which ranged between less than 1% and 95%, was higher in blastic phase (mean 32 +/- 29.9) than in chronic phase (mean 3 +/- 5.3) (p = 0.006). These findings show that p170 overexpression is common in Ph+ CML, especially after progression to blastic phase, and suggest that p170-related MDR may contribute significantly to treatment failure.
ATP Binding Cassette Transporter, Subfamily B, Mem↗