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Biomedical subjects

L Massimo

Publications and source records attributed to L Massimo.

At least 19 recordsLinked to original sources

Psychosocial aspects of survivors of childhood cancer or leukemia.

The majority of childhood cancer patients can expect nowadays to be cured and the percentage is now between 70% and 80%. The number of long-term survivors, off- threatment for at least 5 years, is rising rapidly and is becoming a new population, which needs a special care. It is becoming increasingly important to know how to prevent and treat the physical late effects as well as the psychosocial ones. The oldest among these patients are now in their 40's. How will their old age be like? Are they really cured? The aim of this study is to present a detailed survey of the literature on this topic as well as the authors' personal experience. Several techniques of psychological investigation for this population are highlighted. The semistructured interviews are mostly used for mono-institutional research, while the narrative dialogues are useful for small groups of patients. Questionnaires are usually conducted by epidemiologists for large groups of survivors. Tests are used for specific items such as defense mechanisms, self-esteem, relationships within the family, fear, and panic. The evaluation of the post-traumatic stress disorder is considered and the most important literature data are reported. It is also stressed the need of prevention of any type of psychosocial distress. In conclusion, most of the survivors appear to lead normal adult lives, to have obtained high school degrees, good jobs, and several have families and children. Nevertheless, a small percentage show some psychological or social problems, such as anxiety, depression, fear over the future or over relapse, a second primary, or sterility. The most vulnerable among them are females, people in poor financial conditions, the unemployed and those with poor educations.

Child↗

[Legal regulations to protect under-aged donors issued by E.U. Member Countries. Analysis of the present situation and proposals].

Allogeneic bone marrow transplantation in the E.U. is a routine treatment which requires specific legal procedures to protect under-aged donors. The European Council (5/29/1978) decreed the guidelines concerning organ transplantation for the Member Countries. These guidelines included obtaining written consent from the donor or his/her guardian. The International Convention on children s rights (New York 11/20/1989, art. 12) stated that the minor has the right to give his/her opinion, which must be taken into consideration. Currently, though legal guidelines vary among Member Countries, all require the parents or guardian s written consent. In France, an ethics committee (L. 76-1181/1976) must inform the minor about the consequences of the procedure, respect his/her will and obtain consent. In Luxembourg, the Department of Health requires written consent as well as authorization by three experts, including two physicians. In Spain an ethics committee must obtain the minor s consent which is then approved by a government authority and counter-signed by a physician. In UK an N.H.S. directive (1st Aug. 1993) states that as of 16 years of age donors must give consent. In Germany minors over 14 are allowed to give consent. A legal guarantee is required when the recipient is a parent to avoid conflict of interest. In Belgium minors between 15 and 18 require witnessed written consent, counter-signed by the guardian and approved by a physician. Married donors below 21 need the consenting adult s approval. In Portugal verbal consent by the minor and the guardian is sufficient. In Denmark the guardian s written consent is needed. In Italy a law is being prepared to best protect minors. In conclusion it would be useful for the European Council to decree the detailed legal guidelines and require greater uniformity among the E.U. Countries.

Adolescent↗

[Home care services and the role of "caregivers"].

The care of children and adolescents with cancer continues to grow in complexity. While in most cases we are winning the fight, now quality of life (QoL) is becoming a problem to face and a challenge. Pediatric total care policy includes also home care to deliver in any stage of the disease. This can become very useful in the terminal stage, when QoL is the primary goal. The WHO has defined palliative care as integrating the psychologic and spiritual aspects of patient care; affirming life, and regarding dying as a normal process; neither hastening nor postponing death, offering a support system to help patients live as actively as possible until death; and offering a support system to help the family cope during the patient's illness and their own bereavement&. Recently this aim is considered also for children. The home care team is usually composed by a physician responsible, few physicians, several nurses, social workers, psychologists plus the family/home caregiver. In most countries health professionals now rely on family/home caregivers, who can play an important role in the team. The American College of Physicians has recently edited useful Guidelines. The Penn State Milton S. Hershey Medical Center suggests four key ideas which make the word COPE in order to succeed in solving problems: C for Creativity, O for Optimism, P for Planning, E for Expert information. Not everywhere in Europe home care is included as a part of the Health Care System, even if there are increasing pressures from shrinking budgets. As hospitals can be upsetting for the child, is hospital care always necessary during the long course of the disease? Home care, when feasible, can be an alternative approach. Strong motivations support pediatric home care. The life rhythms are better preserved if the whole family is at home. Parents must be taught how to cope and how to talk with their children, the sick one and his/her siblings. The dialogue is easier at home. Home care respects the needs of siblings. Adjustment to living is easier at home, taking into consideration the active help of relatives and close friends. Children need stability and honesty. Pain is a source of great distress for children. When possible pain control must be obtained or at least tried at home. The child may find a better comfort when in his own bedroom and, if he feels better, he can play at home. When the child is dying the treatment is focused for a good QoL and no more on cure: children can feel the change. The difference is impressive if the child is followed at home and not only in hospital. Volunteer Groups can help the child's family better at home then in hospital. Also in Italy Scientific Pediatric Societies, health care professionals together with Parents' Associations could push politicians and implement the integration of several types of services for treatment of children, including home care, offering their collaboration in the global therapy design.

Caregivers↗

Neuroblastoma in two siblings supports the role of 1p36 deletion in tumor development.

Familial neuroblastoma occurs rarely. We studied a family with three children; one of them has a disseminated (stage 4) and another has a localized (stage 2) neuroblastoma. We observed subtelomeric locus D1Z2 (1p36) deletion in both tumors by using double-color fluorescence in situ hybridization. The MYNC gene was found in single copy in both tumors. Loss of heterozygosity (LOH) and restriction fragment length polymorphism analyses were performed by using DNA from frozen tumor cells and from microdissected tumor areas excised from paraffin-embedded sections. We detected somatic LOH at locus D1S468 (1p36) in a tumor-cell population with a trisomy 1 of the stage-2 patient. Neuroblastoma cells of the stage-4 patient were diploid and showed allelic loss at the following loci: D1S172, D1S80, D1S94, D1S243, D1S468, D1S214, D1S241, and D1S164. Haplotype study showed that the siblings inherited the same paternal 1p36-->pter chromosome region by homologous recombination and that, in the two tumors, arm 1p of different chromosomes of maternal origin was damaged. Our results suggest that the siblings inherited the predisposition to neuroblastoma associated with paternal 1p36 region and that tumors developed as a consequence of somatic loss of the maternal 1p36 allele.

Abdominal Neoplasms↗

Ethical problems in bone marrow transplantation in children.

The medical staff caring for children with hematological or oncological diseases is often faced with delicate ethical and, at times, legal problems. Although many of these are common to other branches of pediatrics where patients are children lacking decision-making capacity, others, such as bone marrow transplantation (BMT), are peculiar to this specialty. This is a vanguard treatment for a wide range of diseases that are either incurable or resistant to conventional therapies. If the patient does not have a related donor, ethical and legal problems can occur: profound emotional dilemmas arise both because of the fretful search for a donor and because of the limited period the patient can be transplanted with good expectations of success. National and International Registries are linked to all BMT Units for the identification of compatible donors with the recipient. Hospitals hosting a BMT Unit must fulfill several requirements which are crucial to guaranteeing the best results when faced with the complexity and aggressiveness of transplantation and possible complications, such as a blood bank, an infectious diseases department, a rehabilitation unit, the availability of consultants, as well as playworkers, teachers, psychologists and social workers. Any trial carried out in a Center without sufficient experience, simply pleading the concept of "last hope" is not ethical, even considering possible late effects and sequelae. For allogeneic bone marrow transplantation it is necessary to obtain both the Consent of the donor and the Consent of parents of the recipient. Often the donor is a child sibling of the patient, lacking decision-making capacity as well. Information to parents of both children must be detailed. In reality, there are practically no risks for the child donor and, if any, they are linked to the general anaesthesia. The text of the Informed Consent is usually deliberated and accepted in advance by the Ethics Committee of the Institution where the patient is followed. Voluntary donor's Consent is required at different steps: at the entry in the Registry, when typing of major histocompatibility complex, at bone marrow harvesting under general anaesthesia. In Italy, Article No. 3, Law No. 107 of May 4, 1990 allows parents or legal guardians to consent to the bone marrow donation of a minor.

Bone Marrow Transplantation↗

The central venous catheter for young patients: an "umbilical cord" fantasy.

Central Venous Catheter (CVC) allows the administration of drugs, parenteral nutrition and blood sample taking for laboratory tests, avoiding continuous damage to the peripheral veins. Most of the psychological traumas caused by crude treatments also decrease and therefore CVC may initially be experienced as the end of continuous physical injuries. Nevertheless, it often happens that other sorts of psychological trauma, due to fantasies and fears elicited by the CVC, arise in children and their families. The CVC is often felt as a dangerous and invasive "foreign body". Moreover after it has been used for a long time it may create a psychological state of dependency and it frequently becomes the only means of salvation. This makes removal particularly difficult and it is felt as a "cutting" of the CVC at the end of the therapy. Young patients elaborate complex fantasies about the starting point, the route and the end point of the catheter. Their parents are often unknowing accomplices of these fears, partially because they project their own experience and also because they are conditioned by the way they have elaborated the information provided by the assistance staff. Since the handling of CVC is under the parent's responsibility when the child is not in Hospital, we should be able to help the patients and their families to cope with this experience in the most suitable way. This makes it possible to avoid both excessive worries and the tendency to underestimate the risk of infection. This is why we consider that submitting children and their family members to interviews, surveys and spontaneous drawing is wise in order to evaluate the existence, nature and extent of the fantasies. This helps to modify possible distortions both of perception and behavior.

Adolescent↗

Gene expression and protein localisation of calcyclin, a calcium-binding protein of the S-100 family in fresh neuroblastomas.

Calcyclin gene, a Ca(2+)-binding protein with homology to S-100, has been found to be expressed at different levels in leukaemic cells and in other tumour cells. We recently reported the expression of the gene in human neuroblastoma (NB) cell lines, and suggested a possible role of calcyclin in cell differentiation. To extend our findings, we investigated the expression of the gene in NB cells induced to differentiate by retinoic acid (RA), using the reverse transcriptase-polymerase chain reaction (RT-PCR) technique. Time-course experiments employing LA-N-5 cells showed that calcyclin mRNA appeared 2 h after RA treatment, long before the cells were blocked in the G1 cell-cycle phase and before the neurite-like structures outgrew from the cell bodies. This suggests the involvement of the gene in the early phase of cell differentiation. Furthermore, we investigated mRNA expression in a series of fresh neuroblastomas. NB tumours showed a heterogeneous pattern of calcyclin expression, although calcyclin seemed to be expressed more frequently in cases with a favourable Shimada histology. We also studied the expression of the protein in formalin fixed and paraffin embedded tissues, by using a specific anticalcyclin antibody. The protein was detected in stromal cells which characterise a more mature histological type, and in nerve sheaths, whereas neuroblasts were negative. The tissue that expressed calcyclin protein showed a Schwann-like differentiation and, unlike S-100 protein, calcyclin was expressed in the perineurium.

Base Sequence↗

Risk-directed therapy for childhood acute lymphoblastic leukemia. Results of the Associazione Italiana Ematologia Oncologia Pediatrica '82 studies.

BACKGROUND: In 1982, the Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP) started its third-generation study, aiming to improve previous results obtained by AIEOP '79 study and to deliver a standardized treatment to most Italian children with acute lymphoblastic leukemia (ALL). METHODS: We treated 902 children (older than 1 year and younger than 15 years of age) with newly diagnosed ALL in multicenter studies of risk-directed therapy (111 low risk [LR] from Study 8201; 570 average risk [AR] from Study 8202; and 117 and 104 high risk [HR] from Studies 8303 and 8503, respectively). Induction therapy was composed of vincristine, prednisone, and asparaginase for LR or AR patients and these agents plus daunorubicin, (Study 8503) or vincristine, prednisone, cytarabine, and intermediate-dose methotrexate (Study 8303) for HR patients. Central nervous system (CNS) preventive therapy consisted of intrathecal methotrexate only (LR), intrathecal methotrexate plus 18 Gy cranial irradiation (AR and HR Study 8503), or high-dose (HD) cytarabine (HR Study 8303). Reinduction therapy was vincristine/prednisone/daunorubicin for AR patients with cyclophosphamide added for HR patients in Study 8303 and HD asparaginase in Study 8503. LR patients did not receive intensification therapy. Continuation therapy comprised 6-mercaptopurine plus methotrexate and monthly pulses with vincristine plus prednisone for all patients, except for HR patients in Study 8303 who also received teniposide plus cytarabine. Weekly HD asparaginase was also given in Study 8503. Duration of treatment was 24 months for Studies 8201 and 8202, 15 months for Study 8303, and 22 months for Study 8503. The overall complete remission (CR) rate was 94.7% (97.3% for LR, 94.9% for AR, and 93.2% for HR). RESULTS: Overall 7-year event-free survival (EFS) was 53.6% (standard error [SE], 1.8). EFS was 60.8% in LR (SE, 4.7), 60.6% in AR at 7 years (SE, 4.7), and 18.5% in Study 8303 (HR) at 5 years (SE, 3.8). Because of the poor result in HR patients, a successor study (8503) was developed that yielded a 5-year EFS of 46.1% (SE, 5.1). Site-specific relapse rates were 18.5% (LR), 13.4% (AR), 35.1% (HR on 8303), and 18.3% (HR in Study 8503) for bone marrow and 9.2%, 7.9%, 17.5%, and 19.3%, respectively, for the CNS (isolated). Isolated testicular relapse was observed in 3.9% of male patients. CONCLUSIONS: This risk-directed therapy cured at least 50% of patients with ALL with relatively nonintensive therapy. The 80% overall survival rate for LR and AR patients at 7 years suggested that the total cure rate may be higher than 50% because of the significant salvage rate for patients who received antimetabolite-based therapy initially.

Adolescent↗

Predicted creatinine clearance to assess glomerular filtration rate in chronic renal disease in humans.

The work was designed to assess the suitability of both measured endogenous creatinine clearance (CCR) and predicted creatinine clearance (P-CCR) to evaluate GFR in chronic renal disease (CRD) by utilizing the renal clearance of inulin (CIN) as gold standard. A total of 124 subjects were studied (62 healthy, 62 with CRF). CCR significantly overestimated GFR in healthy subjects as well as in CRF, whereas P-CCR was identical to GFR. The CCR/CIN ratio which calculates the fractional creatinine clearance and provides a rough estimation of the contribution of creatinine secretion in explaining the differences between CCR and GFR was increased in CRD and especially in CRD of glomerular origin. The ration P-CCR/CIN was significantly lower than CCR/CIN in healthy subjects and in patients with CRD of glomerular origin. The data are against the use of CCR in assessing GFR in healthy subjects and in patients with CRD.

Creatinine↗

Renal functional reserve.

The available data on renal reserve and on the fraction of filtration capacity utilized at rest in childhood are analyzed both in healthy state and in renal disease. The methodological problems which might have biased available information are also discussed.

Adult↗

MYCN amplification does not affect survival of neuroblastoma patients treated with autologous bone marrow transplantation.

From an extended series neuroblastoma cases evaluated for MYCN amplification (MNA) at the "G. Gaslini" Hospital 15 (4 with and 11 without NMA) underwent myeloablative therapy and bone marrow transplantation (MAT-ABMT). Such cases ranged in age at diagnosis from 13 months to 7 years and were followed up at least 8 months after MAT-ABMT. MNA was present in 2/10 cases dead for disease, in 0/1 cases alive with disease, and in 2/4 cases presently in complete clinical remission. This preliminary evidence would discourage to consider MNA as a marker capable of predicting the final outcome of patients with metastatic Nb.

Bone Marrow Transplantation↗

Renal hemodynamics, plasma amino acids and hormones after a meat meal in progressive nephron loss.

Ten patients with chronic renal failure (GFR 29-97 ml/min), on free diets providing 1 g/kg B.W. of proteins, ingested an oral protein load (meat meal, 2 g/kg B.W.). GFR and RPF increased significantly over baseline with no change in filtration fraction. Within 30 min of the meal and for the next 3 h a statistically significant increase was observed in the plasma concentrations of the following amino acid groups: essential, non-essential, total, branched-chain, ketogenic, glycogenic, glycogenic and ketogenic, basic, acid, polar and non-polar. At 30 min the smallest increase was seen in acid and polar amino acids (6.7% and 7.6%, respectively). At 180 min the largest increase (78.8%) was seen for glycogenic and ketogenic amino acids and total plasma amino acids were 1.58 times baseline. After the meat meal plasma glucagon and insulin rose significantly, while growth hormone, plasma renin activity and aldosterone did not vary.

Adult↗