OUTCOME OF EWING SARCOMA IN CHILDREN AND ADOLESCENTS: A FIVE-YEAR SURVIVAL FROM A SINGLE INSTITUTION

Ewing sarcoma, together with neuroblastoma and central nervous system tumors, has got the lowest percentage of complete cure among all malignant diseases in children. From 1993 to 2003, 26 patients with Ewing sarcoma were treated in Pediatric Hematology and Oncology Department, Medical University in Lublin, Poland. In this study the character and time of the clinical symptoms to making the diagnosis, the abnormalities of laboratory tests, and the primary site of tumor were analyzed. The prognosis and results of treatment in children and adolescents were evaluated.     

慢性心力衰竭时β受体密度变化及药物干预的研究

报告３５例慢性心力衰竭患儿血浆与对照组比较去甲肾上腺素、肾上腺素浓度明显升高（Ｐ分别＜０．０１和＜０．０５）；β受体密度显著降低（Ｐ＜０．０１）。其中３０例用β受体阻滞剂治疗后，心功能改善，心胸比率缩小（Ｐ均＜０．０５）；去甲肾上腺素、肾上腺素浓度降低，β受体密度上调，治疗前后差异有非常显著意义（Ｐ分别＜０．０１，０．０５和０．０１）。结果提示β受体阻滞剂治疗慢性心力衰竭安全有效，可明显改善其预后。     

TREATMENT RESULTS OF OSTEOSARCOMA OF THE EXTREMITY IN CHILDREN AND ADOLESCENTS AT EGE UNIVERSITY HOSPITAL

Twenty-five patients were treated for osteosarcoma of the extremity at Ege University Hospital. Eight of them were metastatic. All patients received cisplatin, doxorubicin, ifosfamide, and methotrexate preoperatively. Twenty-three patients underwent surgery at around week 15 (11-18 weeks). All but one underwent limb-sparing surgery. While good responders continued to receive the same drugs, poor responders were given the same regimen before 1996, but high-dose ifosfamide alone after 1996. For all patients the projected event-free survival (EFS) rates were 63.5% at 2 years and 53% at 5 years. The projected overall survival (OS) rates were 72% at 2 years and 62% at 5 years. For nonmetastatic patients, 5-year EFS and OS rates were 67% as compared with metastatic patients (25 and 50%)( p = .01 for EFS; p > .05 for OS). The results show that nonmetastatic patients with osteosarcoma of the extremity have favorable prognosis on this therapy regimen, allowing a high rate of limb-sparing surgery.     

CONGENITAL HYPOTHYROIDISM: AGE AT START OF TREATMENT VERSUS OUTCOME

ABSTRACT. We studied 27 patients with congenital hypothyroidism by neurological and psychometric methods. 7 healthy siblings served as a control group for the psychometric evaluation. In 7 patients treatment had been started before the age of 1 month and in 10 patients after the age of 3 months. Our findings suggest that the progressive loss of intelligence potential starts from birth but if treatment is begun before the age of 1 month, then intelligence remains within normal range. The neurological damage seems to originate partly before birth, but more serious injuries arise if treatment is delayed beyond the age of 3 months.     

TREATMENT OF CYSTINOSIS WITH A DIET POOR IN CYSTINE AND METHIONINE

Two siblings with cystinosis are presented. Case 1, a 16-month-old boy, presented with a severe renal tubular insufficiency. Case 2, a 7-year-old girl, was a dwarf with both glomerular and tubular renal insufficiency. Case 1 was initially treated with high doses of vitamin D₂ and electrolyte supplements for more than 2 months without significant alteration of the condition. Thereafter he was treated for 23 days with 150 mg penicillamine per day again without any significant clinical or biochemical improvement. Both patients were then followed through 1 year on treatment with a diet poor in cystine and methionine supplemented with cholinechloride, an anabolic steroid, high doses of vitamin D₂, electrolytes, oral iron and a combined vitamin preparation. After some time there was considerable difficulty in giving the patients sufficient amounts of the diet, consequently the diet had to be modified with supplement of cow's milk. On this treatment case 1 attained a distinct clinical improvement with healed rickets and normal growth. There was no evidence of mobilisation of the stored cystine. Case 2 obtained a healing of the rickets and some gain in height during the treatment, but otherwise the general condition was unaltered, and she continued to have increasing renal glomerular insufficiency.     

BEHAVIOURAL METHODS IN THE TREATMENT OF SLEEP DISORDERS—A PILOT STUDY

The efficacy of behavioural methods of treatment for severe sleep disorders was examined in a pilot study involving 35 children aged 1-5 years. Improvement occurred in 77%. Methodological issues concerning the selection of children for treatment, selecting adequate controls and outcome measures, and using parents as therapists, are discussed.     

A DOUBLE-BLIND DRUG TRIAL OF TREATMENT IN YOUNG CHILDREN WITH WAKING PROBLEMS

A double-blind trial using trimeprazine tartrate was carried out in 22 children with severe waking problems. On parental verbal reports sleep was significantly improved on the drug compared with the original baseline and the placebo, but diaries kept by the parents showed that this improvement was clinically only moderate, with many wakeful nights still occurring. Taking the drug produced no permanent effect on sleep patterns and a follow-up of 14 children 6 months later showed persisting sleep problems in the majority.     

COMPLETE PLATELET RECOVERY AFTER TREATMENT OF HELICOBACTER PYLORI INFECTION IN A CHILD WITH CHRONIC IMMUNE THROMBOCYTOPENIC PURPURA: A Case Report

Helicobacter pylori gastritis has been associated with autoimmune disease, including immune thrombocytopenic purpura (ITP). The most recent reports also have supported this association in adults. ITP in children differs from that in adults in terms of clinical picture and mechanisms of thrombocytopenia. The authors report a case of a 12-year-old boy with chronic ITP, in whom they detected H. pylori infection and observed a complete platelet recovery after the eradication of H. pylori.     

TREATMENT OF RECURRENT URINARY TRACT INFECTION IN CHILDREN

Abstract. Daschner, F. and Marget, W. (Division for Antimicrobial Therapy, Children's Hospital, University of Munich, BRD). Treatment of recurrent urinary tract infection in children. Acta Paediatr Scand, 64:105, 1975.–Since none of the studies of long-term management of recurrent urinary tract infections considered the possibility of patients' not taking medication, compliance with long-term antibiotic therapy was tested by urine check in 93 children with recurrent urinary tract infection. Only 30 children (32.2%) took the prescribed drugs at regular intervals, 27 children (19.1%) did not take the antibiotics at all and 36 patients took the drugs irregularly, skipping one or two doses a day. The difference in infection rate between regular takers (3.9/year) and non-takers (7.2/year) and irregular takers (4.8/year) and non-takers was statistically highly significant. Forgetfulness and negligence of the parents was found to be the main reason for not giving the child the medication. Treatment studies which show the advantage of one regimen over another or which try to define optimal duration of therapy should take particular care in evaluation compliance.     

COMPLIANCE, ''NEGATIVISM'', AND THE EFFECTS OF TREATMENT STRUCTURE IN AUTISM: A NATURALISTIC, BEHAVIORAL STUDY

Nineteen autistic individuals were observed in their familiar residential environment using a time-sample technique. Rates of stereotyped and self-injurious behaviors, the direction of the subject's gaze (at staff, task or elsewhere) and the number of requests made to a subject by staff were a function of treatment structure, as defined by the staff: child ratio. While patterns of compliance in response to different types of requests were observed, subjects were generally compliant. The data do not support the notion that autistic children are unusually 'negativistic'. The utility of ecologically valid observations and the need for normative data are discussed.     

CLINICOLABORATORY FINDINGS AND TREATMENT OF IRON-DEFICIENCY ANEMIA IN CHILDHOOD

One of the major causes of anemia in childhood worldwide is iron deficiency. Its prevalence depends mainly on age, being higher in infancy and adolescence. Its etiology varies, but poor iron diet is considered the commonest causative factor. Better tactics may be needed, like the targeted screening of children who belong to high-risk groups, to eradicate childhood iron deficiency. The amount of the body iron regulates its absorption from the gut through mechanisms that are still poorly understood. Early identification of iron deficiency is essential for the prevention not only of anemia but also the numerous and long-term consequences caused by the lack of iron. Many tests are available for the diagnosis of the disease. Some of them seem very promising for the early detection of iron deficiency, but further research is needed before they become widely acceptable in clinical practice. Treatment is based on oral iron salts, which do not have any serious side effects.     

PREVENTION OF CEREBRAL PALSY IN MOTOR RISK INFANTS BY TREATMENT AD MODUM VOJTA: A Controlled Study

Abstract. Brandt, S., Lønstrup, H., Marner, T., Rump, K. J., Selmar, P., Schack, L. K. (Clinic for Cerebral Palsy and Child Neurology, Orthopedic Hospital, Rigshospitalet, Copenhagen, Denmark) and d'Avignon, M., Norén, L. and Årman, T. (The Paediatric Clinics of St. Göran's and Danderyd Hospitals, Stockholm, Sweden). Prevention of cerebral palsy in motor risk infants by treatment ad modum Vojta. Acta Paediatr Scand, 69: 283, 1980.—The proposal by V. Vojta in 1974 to prevent development of cerebral palsy in 'motor risk' infants by special treatment has been investigated in 11 Danish and 10 Swedish babies and compared with 30 control infants with a similar risk, who were not given Vojta treatment. We found a tendency for 'uncomplicated' cerebral palsy cases to accumulate in the control group, although the difference was non-significant on a 5% level. Further con-trolled studies must be completed before it is possible to accept the prophylactive treatment of cerebral palsy recommended by Vojta.     

HABIT REVERSAL AND DIFFERENTIAL REINFORCEMENT OF OTHER BEHAVIOUR IN THE TREATMENT OF THUMB-SUCKING: AN ANALYSIS OF GENERALIZATION AND SIDE-EFFECTS

This study evaluated the generalization, maintenance, and negative side-effects of Habit Reversal (HR) and Differential Reinforcement of Other Behaviour (DRO) in the elimination of thumb-sucking. Thirty children who sucked their thumbs or fingers excessively were randomly assigned to a HR, DRO or wait-list control group. Observation data were collected in three observation settings. Both procedures effectively reduced thumb-sucking in both a training setting and in two generalization settings, and initial generalization effects maintained over time. However, both procedures resulted in some temporary increases in oppositional behaviour and produced low elimination rates. Differences between HR and DRO were not statistically significant; however parents evaluated HR somewhat more favourably than DRO.     

SUCCESSFUL TREATMENT OF ASPERGILLUS BRAIN ABSCESS IN A CHILD WITH ACUTE LYMPHOBLASTIC LEUKEMIA AND LIVER FAILURE

Invasive fungal infection continues to pose a significant threat to immunocompromised patients, with cerebral aspergillosis being among the most feared ones. The authors describe an adolescent girl with acute lymphoblastic leukemia (ALL) with subsequent acute liver failure, who developed an aspergillus brain abscess. The patient was treated with combined antifungal therapy using amphotericin B local instillation, prolonged systemic amphotericin B colloidal dispersion along with vinca alkaloids-containing chemotherapy, followed by neurosurgical débridement and oral voriconazole in the setting of ongoing antileukemic maintenance chemotherapy. Her ALL remains now in complete remission 30 months from diagnosis, with no evidence of fungal infection.     

CYSTIC FIBROSIS IN DENMARK IN THE PERIOD 1945-1981 EVALUATION OF CENTRALIZED TREATMENT

Nielsen, O. H. and Schiøtz, P. O. (Paediatric Department TG, Rigshospitalet, Copenhagen, Denmark). Cystic fibrosis in Denmark in the period 1945-1981. Acta Paediatr Scand 1982; suppl 301: 107-119. — The present study gives a re-evaluation of the course of the prognosis for cystic fibrosis (CF) in Denmark in the period January 1, 1945 — September 15, 1981. A total of 490 patients fulfilled the criteria of entry.
Despite a rather constant annual incidence, the number of surviving CF patients is highly increasing: thus 31 patients were alive as on December 31, 1960, 119 as on December 31, 1970, and 223 as on December 31, 1980. The survival was further evaluated using the decrement method, where the patients are entered by time of birth or time of diagnosis, and the Warwick & Monson method, where the patients are entered by time of diagnosis. It applies to both methods that the underlying patient populations did not include patients with meconium ileus and patients with a retrospective or autopsy diagnosis. The probability of reaching the age of 10 years was 52% for CF patients treated exclusively in departments other than the CF centre as compared to 84% for the CF centre patients.
We conclude that the centralized treatment should be preserved for a so relatively uncommon disease as CF, both because our survival curves demonstrated the prognosis to be by far the better in the patients treated at the national CF centre and because centralized treatment alone can offer appropriate research conditions.     

AN INVESTIGATION OF TWO SELF-REPORT MEASURES OF OBSESSIONAL PHENOMENA IN OBSESSIVE-COMPULSIVE ADOLESCENTS: RESEARCH NOTE

A number of studies have recently demonstrated the clinical utility of objective self-report inventories in the assessment of obsessive-compulsive disorders in adults. To date, the usefulness of these instruments has not been demonstrated with obsessive-compulsive adolescents. In the present study the Leyton Obsessional Inventory and the Maudsley Obsessional-Compulsive Inventory were administered to 11 obsessive-compulsive adolescents and a group of 10 anxious non-obsessional patients. The obsessional adolescents scored significantly higher than the controls on the Maudsley total score and checking factors alone. Possible explanations of the results are discussed.