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This reprinted article originally appeared in American Journal of Orthopsychiatry, 1979, Vol. 49, No. 2, 240-245. (The following abstract of the original article appeared in record 2013-43131-006.) The fourth follow-up study of adolescent delinquent boys treated in a community-based program that combined job placement, remedial education, and psychotherapy indicates that the better overall adjustment of the treated group, compared to untreated controls, is maintained well into adulthood. It reaffirms the importance of developing sound, innovative means of reaching adolescents in crisis, and suggests the value of a fullscale replication of the original program.

The relationships between large and frequent transfusions and erythropoiesis have been studied with special regard to thalassemia major in 8 children (some of them splenectomized).With large t

Strategies for working with community groups must be developed if the principles of community mental health are to be put into practice. An experimental outpatient treatment program for adolescent delinquents that required the cooperation of two community groups, the school and businessmen, necessitated the development of certain procedures for mobilizing the groups around the implementation of treatment. The success of the program serves to highlight certain principles of working with community members.

Since 1989, through the work of SPEAR, considerable experience has been accumulated on the technique of organising evaluation panels and of supporting their activity with a methodological input and with external consultants. SPEAR has played a major role in creating a true community of European R&D evaluators, and the discipline is now more active in Europe than its birthplace in the USA. Copyright , Beech Tree Publishing.

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, nonessential, 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.

We report an 8-year-old patient with clinical features suggesting Leigh's syndrome and with a decreased activity of the E1 component of the pyruvate dehydrogenase complex in cultured skin fibroblasts. A nerve biopsy showed the presence of severe peripheral neuropathy, rarely described in the literature. The partial correction of lactic acidosis with oral sodium bicarbonate chronic therapy may result in a slow evolution of the clinical symptoms.

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.

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.

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.

This report looks at evaluation studies of a number of cooperative academic-industrfy research programmes in Europe, and suggests that indirect economic benefits should be taken into account as well as motives and goals of participating firms.

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.

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.

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.

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.

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).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).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.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.

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.

Under-Aged Donor Informed Consent for Allogeneic Bone Marrow Transplantation in The European Union (E.U.) Countries. Legal Aspects

Adolescence is the time of life in which the most important transformations take place. There is also an evolutionary “ break down”, a period in which the somatic and the psychic evolution run along very different paths and at different speeds. Both contracting a potentially lethal disease and facing such hard treatment as bone marrow transplantation create strong interference of the growth process. In our Bone Marrow Transplantation-Unit over the past year we began observing 7 patients who were between 12 and 17 years of age, starting from when diagnosis was communicated and for the whole time they were under treatment. Simultaneously we studied 7 adolescents who had undergone transplantation and were off treatment for at least two years. We preferred to carry out psychodynamic interviews rather than psychometric tests because we realized that the latter were not well accepted by the adolescents. The data obtained from the observation and interviews reveal that the most disturbing facts regard the “ fragmentation of the ego”, fantasies about death and resurrection and above all about their sexual identities when the donor is of opposite gender. We saw two adolescents who developed psychotic access. In one case this took place during hospitalisation and in the other at release. Some very meaningful data regard the high frequency of “ panic attacks”. In our country this disturbance is at present more frequent than it ever was in the past, yet however is lower than in adolescents who have undergone transplantation.

Two siblings affected with congenital dyserythropoietic anemia type I are described. They are the sixth familial occurrence reported. Particularly interesting is the comparison between the course and laboratory data of our cases. An unusual finding is the presence of the antigen 'i' on the erythrocytes of both patients.

In high temperature gas-cooled pebble bed reactors a meaningful way of describing the fuel element temperature is a statistical distribution function. Such a function gives information from which fuel element damage and fission product release can be assessed. A numerical method for the calculation of this statistical temperature distribution has been developed taking into account the flow properties of the pebble bed. Results are given for the 300 MWel THTR reactor. For this reactor statistical distributions have been calculated for the temperature throughout the fuel, for the surface temperature of the fuel compact, and for the temperature difference from the center to the surface of the fuel compact.

Analyses of thematic stories applied to selected pictures by delinquent and nondelinquent adolescent boys reveal that the delinquents apparently are more concerned with interpersonal relationships. This concern is primarily hostile (either moving against or protecting oneself from people). Delinquents also rarely see authority figures as passively cooperative. This suggests that delinquents may develop a cognitive awareness and sensi tivity to people with many adaptive aspects. This may reflect the object hunger and desire for relationships, hostility and fear of such relationships, and the intense need to manipulate others in order to obtain gratification, all of which have been found to be delinquent characteristics.

SUMMARYTwo DZ monoplacental twins are reported, one affected by mongolism and the other normal. Chromosome examination in the former indicated trisomy 21, while the latter presented a mosaicism consisting, at the age of 13 days, in 70% normal and 30% 21-trisomic cells; at the age of 5 months, in 95% normal and 5% trisomic cells; while, at the age of one year, all the cells appeared to be normal.The possibility of mosaicism, and, according to clinical and hematological findings, that of feto-fetal blood transfusion are excluded.The hypothesis is suggested of a progressive loss of the reproductive capacity of « chimaera » of abnormal lymphocytes.

Three cases of autoimmune hemolytic anemia in infants are described. This disease is very rare in the first few months of life. One case (No. 3) was complicated with a rare hemoglobinopathy, which may have been coincidental. Corticosteroid treatment in larges doses was partially and temporarily effective in controlling the disease. Immunosuppressive therapy (azathioprine = Imuran, 2-5 mg./Kg./day) produced a complete cure in Case 1 and allowed a marked decrease in steroid doses in the other two cases. Most probably, a vicious circle of autoimmunity was broken by this drug. Failure to gain weight on steroids was more than compensated for by the clear-cut "catch-up growth" in Case 1, even though Imuran was being given. Indications regarding precautions and dosage of azathioprine are stated. The exact mode of action of these drugs is discussed in an attempt to explain the nature of immunosuppression.

SUMMARYThe Authors have observed a high rate of chromosomal morphologic alterations in leukocyte cultures in 10 children who had previously undergone Roentgen irradiation for diagnostic purposes. Temporary alterations have been observed, such as interruptions of chromatine on the proteic thread, defined as achromatic lesions of one or more chromatids; fragmentations of chromatids; fragments without centromere; deletions; ring-chromosomes; dicentric and tricentric chromosomes and, in certain cases, increase of polyploid mitoses, both on account of endo-reduplications or of a lacking cytoplasm division.

The PHA-responsiveness in cell-culture of mononuclear cells obtained in acute leukemia from different sources, such as blood, bone marrow, lymph-nodes, leukemic pleural effusion and spinal fluid were investigated. The peripheral blood lymphocytes of all but 2 of 28 untreated patients showed extents of blastogenesis from 48 to 90%. The bone-marrow cells of 18 of the 28 patients gave high extents of blast-like cells (52 to 88%) in 5. In fact, the marrow cells of 9 patients showed blast extents only from 1 to 14%, whereas the remaining 4 patients had no marrow-cell transformation at all. Also the lymphocytes from lymph-nodes of 2 patients show low extents of blastogenesis (8.5–12.3%). Again, the mononuclear cells obtained from the spinal fluid in 7 patients affected by meningeal leukemia, and from the pleural effusion in 1 patient did not undergo any blastic transformation. The question arises whether the PHA-responsive cells from the acute leukemia peripheral blood are normal immuno-competent lymphocytes circulating in the blood, independent from the cell type of leukemia. As a consequence, it comes up whether the bone-marrow cells are PHA-responsive in the lymphocytic type of acute leukemia and PHA-nonresponsive in other cell-types of leukemia (myelo-monocytic). Thus, the PHA-cell culture of both the peripheral blood and the bone-marrow cells could aid in recognizing the cell types of leukemia one is dealing with.

A follow-up study of the adolescent delinquent boys participating in a new comprehensive, vocationally-oriented psychotherapy program reported earlier revealed that two to three years following termination the treated boys continued to show major improvement in ego functioning, though at a slower rate than during treatment. An untreated group deteriorated during that time. The results are of significance in planning programs of intervention, especially with lower socioeconomic groups.

SUMMARYA case of a female newborn, followed up to the 9th month of life, presenting a clinical picture of multiple malformations, typical of trisomy-18, is reported by the AA.: microcephaly, low set of ears, micrognaty, ogival palate, superabunding skin in the neck, simian crease, typical hands attitude, luxation of the right hip, severe neurological symptomatology with the characteristic rigidity and, finally, an extremely severe mental defect.Growth has always been very poor; death occurred at the 9th month of life.Chromosome research, done with two cultures of capillary blood, revealed the presence of two lines of normal and trisomy-18 cells in similar percentage.The literature concerning the subject is examined and discussed.

In the present study, 15 children with non-Hodgkin lymphoma have been immunologically evaluated by the following parameters on peripheral blood (PB) lymphocytes: phytohemagglutinin responsiveness (PHA-r); non-immune rosette formation with sheep red blood cells (RF-L) as T-cell marker; presence of surface immunoglobulins (sIg-L) as B-cell marker; serum immunoglobulins levels (IgA, IgM, IgG). Our patients (pts) have been divided in two groups: the first one includes 10 children without PB involvement; the second one includes 5 pts with bone-marrow and PB invasion. From our data it appears that: 1) the majority of pts of the first group presented normal values of membrane markers; PHA-r was impaired in 4/8 pts; 2) in pts with PB invasion absolute number of B and "null" cells was always abnormal and PHA-r altered; 3) in the second group of pts, a "null" cell origin can be suggested by the high percentage and absolute number of cells without surface markers. In our opinion, the high incidence of "null" cells represents the most relevant question: whether they are cells deprived of specific markers, or endowed with markers not identifiable by our current techniques, remains to be established.

The spleen is the least common intra-abdominal site for the development of cystic disease. A lymphoangioma of the spleen was first reported in 1885 by Fink; since that time 47 cases of lymphoangioma or lymphoangectasia have been reported. This report concern a case of cystic lymphoangioma of the spleen consisting of a large splenic cyst with associated multiple small subcapsular cysts and lymphangectasia. The diagnosis and treatment of splenic cysts are discussed and a new classification of splenic cysts is proposed.