Comparison of totally implanted and external catheters in paediatric oncology patients.

From June 1982 until December 1989, 93 permanent central venous catheters [59 external catheters (ECs) and 34 implanted catheters (ICs)] were placed in 69 patients. The median age of these patients at placement was 5.6 years for ECs and 8.8 years for ICs (P less than 0.05). Follow-up evaluation was possible on 86 catheters (58 ECs and 28 ICs). The median time of insertion was 236 days and 316 days for ECs and ICs, respectively (P less than 0.05). The median number of open days was 58 for ECs and 66 for ICs (not significant). 17 catheters (6 ECs and 11 ICs) were transiently obstructed (P less than 0.005). 30 episodes of bacteraemia were documented in 20 patients. The incidence of catheter sepsis and bacteraemia of unknown source was one in 278 and 283 open days for ECs and ICs, respectively (not significant). In this retrospective study, ECs appeared to be as safe as ICs when infection was correlated with use of the catheter, but this finding should be confirmed in a randomised design.

Regression of cardiac insufficiency after ambulatory intravenous deferoxamine in thalassemia major.

A case of severe cardiac failure due to iron overload in a patient with beta-thalassemia major is reported. The patient was successfully treated with high-dose ambulatory intravenous deferoxamine (desferrioxamine). This type of chelation appears to be a valuable alternative to subcutaneous deferoxamine administration in the presence of severe iron overload.

Intrathoracic neuroblastoma presenting with neonatal cardiorespiratory distress.

We report an unusual presentation of congenital intrathoracic neuroblastoma. Severe respiratory distress was present at birth and several salient clinical signs were suggestive of neonatal myocarditis. Excessive catecholamine secretions may have caused the observed cardiac dysfunction. Peculiar previously unreported cutaneous lesions were also noted in this case.

Cefetamet pivoxil in the treatment of pharyngotonsillitis due to group A beta-hemolytic streptococci: preliminary report.

Penicillin therapy has considerably reduced the occurrence of serious late complications of streptococcal pharyngotonsillitis. However, treatment failure with this antibiotic is currently reported in up to 30% of the cases. Beta-lactamase production by the commensal flora of the tonsils, and poor compliance of patients have been implicated as the main causes of treatment failure. Cefetamet pivoxil is a new oral cephalosporin with a twice daily dosage and striking stability against beta-lactamases. We are conducting a prospective, randomized study in 120 children, comparing the efficacy of cefetamet pivoxil 10 mg/kg bid for 5 and 10 days and phenoxymethyl penicillin 25,000 U/kg tid for 10 days in the treatment of group A beta-hemolytic streptococcus (GABHS) pharyngotonsillitis. Children are enrolled after the positive culture of a throat swab and randomly assigned to one of the three groups. A follow-up check-up is performed at the end of the therapy (clinical check-up) and 3 to 5 days later (bacteriological check-up). The preliminary analysis of 55 cases shows that in 88.9%, 100% and 87.5% of children GABHS was eradicated by 5 days or 10 days of cefetamet pivoxil, or 10 days of penicillin respectively (p = NS). Side effect were mild and transient in cefetamet pivoxil-treated patients, but required cessation of treatment in 2 children treated with penicillin. These results suggest that cefetamet pivoxil therapy for 5 days eradicates GABHS from the throat in streptococcal pharyngotonsillitis as efficiently as cefetamet pivoxil or phenoxymethyl penicillin for 10 days.

[Gamma-glutamyltransferase in maternal serum and prenatal screening for the fetal alcohol syndrome]. / Gamma-glutamyltransférase sérique maternelle et dépistage prénatal du syndrome de l'alcoolisme foetal.

In order to investigate prospectively in pregnant women a correlation between serum levels of a biological marker of alcohol and the clinical status of the newborn, we measured the gamma-glutamyltransferase (GGT) in 630 women between 14 and 20 weeks of pregnancy. In 7% of the cases, an elevated value was observed while history confirmed alcohol consumption in only 1%. Preliminary statistical analysis established upon the blind examination of 308 newborns show a correlation between maternal GGT levels and birthweight as well as the pre- and perinatal complications. However, the sensitivity of this test is weak.

Prevalence of child sexual abuse among adolescents in Geneva: results of a cross sectional survey.

OBJECTIVE: To measure the cumulative prevalence of child sexual abuse in a representative sample of the adolescent population of Geneva. DESIGN: Cross sectional survey with an anonymous self administered questionnaire centred on a factual description of sexual activities. SETTING: 68 classes (17 schools) randomly selected from the 201 ninth grade classes of the public school system in Geneva. SUBJECTS: 1193 adolescents aged 13-17 years, of whom 1116 (93.5%; 568 girls, 548 boys) consented to the study and returned completed questionnaires. RESULTS: 192 (33.8%) girls and 60 (10.9%) boys reported having experienced at least one sexually abusive event. The prevalence of abuse involving physical contact was 20.4% (116 cases) among girls and 3.3% (18) among boys. The prevalence of abuse involving some form of penetration was 5.6% (32 cases) among girls and 1.1% (six) among boys. One third of the abused adolescents had experienced more than one abusive event and 46.5% (92/198) had experienced the first event before age 12. Abuse by a family member was reported by 20.5% (36/176) of abused girls and 6.3% (3/48) of abused boys. Abusers were known to victims in two thirds of cases. Ninety per cent of abusers were male and 35.3% (71/201) came from the victim's peer group. Over 80% of participants found the questionnaire interesting, clearly formulated, and useful. CONCLUSIONS: Child sexual abuse is a universal social phenomenon. Adolescents themselves can contribute to research and so help in the search for more efficient prevention and intervention strategies.

[Maternal serum gamma-glutamyltransferase and prenatal diagnosis of the fetal alcohol syndrome]. / Gamma-glutamyltransférase sérique maternelle et dépistage prénatal du syndrome de l'alcoolisme foetal.

In order to investigate prospectively the relationship between maternal serum levels of a biologic marker of alcohol and the outcome of pregnancy, we measured serum gamma-glutamyltransferase in 541 women between 14 and 20 weeks of pregnancy. An abnormally elevated value was observed in 6.8% of the cases but only 16.2% of these suspected alcohol abusers admitted drinking practices during pregnancy. Analysis of obstetrical issue and blind examination of the newborns showed a significant correlation between raised gamma-glutamyltransferase levels and an increased incidence of pre-/perinatal complications, congenital anomalies and intrauterine growth retardation. However, the sensitivity of this test is weak, limiting its use in the early recognition and prevention of fetal alcohol effects.

Use of recombinant erythropoietin in treatment of the anemia of prematurity.

The anemia of prematurity is defined by a progressive decline in hemoglobin level occurring over the first 2 months of life. Unlike term newborns whose "physiologic anemia" rarely if ever necessitates any treatment, preterm infants may become anemic enough to have clinical symptoms that indicate a need for red blood cell transfusions. Various factors contribute to the development of this anemia. Some of these factors, such as the short life span of erythrocytes in preterm infants, increased sensitivity of the erythrocytes to oxidative injury, and the blood losses caused by repeated phlebotomies, would normally be expected to induce corrective reticulocytosis. Characteristically, however, this anemia is hyporegenerative. Thus, it is associated with relative reticulocytopenia, low serum erythropoietin levels, and bone marrow erythroid hypoplasia. The recent availability of recombinant human erythropoietin has opened new perspectives in the management of a variety of anemias. Based on current knowledge of the regulation and pathophysiology of fetal and neonatal erythropoiesis, recombinant erythropoietin may represent a logical and efficient alternative to giving red blood cell transfusions in the treatment of the anemia of prematurity. Clinical trials have been initiated in several countries using different approaches and methodology. At this early stage these trials do not yet fully affirm that recombinant erythropoietin can be used as the first-line therapy in infants with the anemia of prematurity. Our own observations, however, suggest that this agent is well tolerated by preterm infants and may exert a corrective effect on the anemia of prematurity.

Is bone marrow examination justified in idiopathic thrombocytopenic purpura?

Bone marrow examination is widely accepted among pediatric hematologists as a mandatory investigation in childhood idiopathic thrombocytopenic purpura (ITP). The aim of this procedure is to confirm the presence of megakaryocytes and to exclude other conditions, such as leukemia and aplastic anemia. To assess the need for bone marrow examination, we reviewed the charts of 127 children with presumed ITP and found that bone marrow examination led to a different diagnosis in five (3.9%) of them. All five patients had presented with clinical and/or laboratory features atypical of acute ITP; none had leukemia. The initial clinical and laboratory findings of 50 patients with aplastic anemia also were reviewed; all had features atypical of acute ITP. Proper history and physical examination as well as a complete blood cell count are reliable means of recognizing patients with typical vs atypical features of ITP. Bone marrow aspiration could be limited safely to those patients with atypical features of ITP or to patients being treated with corticosteroids.

Diamond-blackfan anemia: etiology, pathophysiology, and treatment.

Diamond-Blackfan anemia (DBA) is manifested by a wide variety of clinical and in vitro abnormalities. Despite this biological diversity, the hematological phenotype is remarkably similar for all patients and consists of a normochromic-macrocytic anemia in early childhood, reticulocytopenia, and a normocellular marrow with a selective deficiency of red cell precursors. Fetal hemoglobin is usually increased, distributed heterogeneously, has a fetal G gamma/A gamma pattern, and is associated with increased expression of red cell i antigen. Although most cases are sporadic, there are examples of autosomal recessive and autosomal dominant inheritance patterns. Approximately 70% of patients with DBA respond to prednisone, and many can be maintained on tapered doses. Those who are steroid-dependent at high dosage as well as those who do not respond are managed on a transfusion and iron chelation program. Claims of efficacy for other therapies, such as cyclosporine or high-dose intravenous methylprednisolone, require substantiation. Bone marrow transplantation has been successfully performed in patients who have tissue-matched donors, and the procedure cures the anemia. Recombinant growth factors may be a therapy of the future. Regarding pathophysiology, initial reports of humoral or cellular inhibitors of erythropoiesis were not confirmed in all laboratories. However, some patients have lymphocyte dysfunction with decreased T cells, decreased T4/T8 ratios, and defective lymphocyte-mediated suppression of lymphoproliferation. A large body of data indicates that the erythroid stem cells are intrinsically defective in DBA, and they are partly or completely refractory to erythropoietin. The role of elevated red cell adenosine deaminase activity in the pathogenesis of this abnormal erythropoiesis is not clear, but this finding is characteristic of the syndrome in most patients. Present studies using recombinant growth factors have demonstrated a diversity of defects in erythropoiesis in patients with DBA. Blocks in red cell production and red cell maturation were seen at various levels along the differentiation pathway. Of clinical interest, interleukin-3 has a corrective effect in vitro on the aberrant marrow erythropoiesis of steroid-refractory patients, and, hence, it may have therapeutic application.

Prepubertal gynecomastia following topical inunction of estrogen containing ointment.

A six and a half-year-old boy was investigated for prepubertal gynecomastia and hyperpigmentation of the skin. The biological, clinical and radiological work-up did not reveal any endocrinological or tumoral cause. It was incidentally noticed that the patient's sister presented with similar clinical signs which could be related to an estrogen containing ointment. Long-term follow-up of the boy showed complete but delayed regression of the gynecomastia and persistence of hyperpigmentation. His sister developed later on a normal puberty.

[The adolescent girl and contraception. Notes on the psychodynamics of pregnancy and motherhood desires (author's transl)]. / L'adolescente et la contraception. Reflexions sur la psychodynamique des desirs de grossesse et de maternite.

PIP: The availability of efficient birth control methods does not guarantee efficient contraception. Many adolescent girls are insufficiently informed about the mechanisms of reproduction or the techniques of birth control and first come to medical attention or consult a family planning center when already pregnant. However, among those teenagers who do become pregnant or even experience motherhood at an age when society would rather see them on school benches, some are neither ignorant about sexuality nor the victims of an unpredictable failure. For those, contraception and the desire for pregnancy or motherhood are in conflict with one another and is only further aggravated by various sociocultural and psychosocial factors. All too often, such a conflict results in a voluntarily ignored or unconsciously rejected contraception option, with an unplanned pregnancy as the outcome.^ieng

Transient hyperphosphatasemia of infancy.

Six patients with transient hyperphosphatasemia of infancy (THI) are described and compared to similar cases reported in the literature. THI appears to be more common than usually thought and may occur in healthy children as well as in patients with various clinical disorders. The evolution of such children seems uniformly normal. Invasive diagnostic procedures should therefore be avoided in this benign condition.

Early hospital discharge of children with fever and neutropenia: a prospective study.

PURPOSE: We report a prospective study on brief IV antibiotic therapy in selected children with cancer experiencing fever and neutropenia (FN) after chemotherapy. PATIENTS AND METHODS: All children with FN (T degree > or = 38 degrees C; ANC < 0.5 x 10(9)/L) were hospitalized for treatment with broad spectrum IV antibiotics. They were divided into three groups: group A (no infection), group B (clinically documented infection), and group C (bacteremia). Children in group A (and some children in group B) were discharged before recovery of neutropenia, if afebrile and in good condition. RESULTS: Eighty-eight consecutive episodes of FN occurred in 30 children. Children in group A (44 episodes; 50%) received IV antibiotics for a median of 3 days; on 25 occasions (57%), IV antibiotics were stopped before recovery of neutropenia. In children in group B (30 episodes; 34%), early discharge was allowed in eight cases of minor infections (27%); six received oral antibiotics. Two children (group A) were rehospitalized for recurrent FN but recovered without complications. CONCLUSION: In chemotherapy-induced neutropenia, children hospitalized for fever but without documented infections and some children with minor infections can cautiously be discharged before evidence of bone marrow recovery if afebrile and in good general condition.

Diamond-Blackfan anemia: promotion of marrow erythropoiesis in vitro by recombinant interleukin-3.

To clarify the defective erythropoiesis in eight patients with Diamond-Blackfan anemia, we studied their bone marrow response in vitro to recombinant human interleukin-3 (IL-3) and recombinant granulocyte-macrophage colony-stimulating factor (GM-CSF). In an erythropoietin-containing assay system, specimens from six of the eight patients yielded low numbers of erythroid colonies compared to control values, and in five of these no erythropoietin dose-response could be elicited. Addition of IL-3, GM-CSF or both to cultures from the six patients had no effect on CFU-E-derived colonies. In contrast, IL-3 but not GM-CSF induced a marked increase in the number (183%) and size of the BFU-E-derived colonies in five of the six cases and partially corrected the impaired dose-response to erythropoietin in four. Bone marrow from the other two patients yielded numbers of CFU-E and BFU-E colonies comparable to controls and manifested similar increments in colonies with increasing concentrations of erythropoietin. When IL-3 was added to these cultures, further increments were observed in the number and size of BFU-E colonies. We conclude that IL-3 enhanced the marrow erythropoiesis in most of the patients and exerted a corrective effect on the aberrant colony formation in the presence of erythropoietin. The data raise the possibility of IL-3 as a therapeutic agent in Diamond-Blackfan anemia.

Modulation of human marrow haematopoiesis by leucotrienes in vitro.

The influence of products derived from the arachidonic acid 5-lipoxygenase (5-LO) pathway on normal human marrow haematopoiesis was studied using clonogenic assays. The leucotrienes (LT) B4 and C4 caused a reduction in granulocyte-macrophage (CFU-GM) as well as erythroid (CFU-E and BFU-E) colony numbers in a dose-dependent manner. Conversely, nordihydroguaiaretic acid (NDGA), a LT synthesis inhibitor, promoted proliferation of these colonies. The inhibitory effect of LTC4 but not that of LTB4 was abolished by FPL-55712, a LT antagonist; the enhanced colony growth induced by NDGA was reversed by both LTB4 and LTC4. LTD4 had no significant effect on colony growth despite its structural similarity to LTC4, which implies that the haematopoietic suppression by LTB4 and LTC4 is specific for these compounds. Depletion of marrow T-cells or removal of adherent cells, or both, did not alter the response to LTB4 and LTC4, suggesting that LT action is exerted directly on progenitor cells and probably is not mediated by other cell populations. Our studies show that the 5-LO pathway is functional in these culture systems and yields products with inhibitory properties. The 5-LO pathway may have an important regulatory function in normal marrow haematopoiesis.

Recombinant human erythropoietin in the treatment of infants with anaemia of prematurity.

Recombinant human erythropoietin (rHuEPO) was administered subcutaneously three times a week to 18 infants with the anaemia of prematurity at doses of 75, 150, 300, or 600 units/kg per week for 4 weeks, starting at 3-4 weeks of postnatal age. A significant and dose-dependent increase in reticulocyte count was observed from a mean baseline value of 71 x 10(9)/l to 200 x 10(9)/l after 3 weeks of therapy, compared with a change from 69 to 97 x 10(9)/l in 66 historical controls. The haematocrit value remained unchanged during rHuEPO treatment, whereas it steadily declined until 9 weeks of postnatal age in the controls. These effects were accompanied by a marked reduction in serum iron concentration and transferrin saturation in patients receiving standard-dose iron supplements, but not in those given larger doses. Only 3 of 18 patients required a red blood cell transfusion. These infants were among the most anaemic at entry into the study and 2 of them were unable to complete rHuEPO therapy, while the third developed iron deficiency anaemia. These data indicate that rHuEPO with appropriate iron supplementation may accelerate the recovery from anaemia of prematurity. Larger scale placebo-controlled studies are now needed to confirm these findings and verify their impact on transfusion requirements of premature infants.

Severe acquired aplastic anemia in children: 11-year experience with bone marrow transplantation and immunosuppressive therapy.

We reviewed the management and outcome of severe acquired aplastic anemia in 36 patients younger than 18 years old between March 1977 and June 1987. In most patients, no cause could be found. The best therapeutic results were achieved with allogeneic bone marrow transplantation from histocompatible related donors (14 patients), with a cure rate of 79%. Bone marrow transplantation from mismatched related donors was attempted in two patients and failed in both. Immunosuppression using antithymocyte globulin and/or high-dose methylprednisolone represented an alternative treatment in the absence of a suitable bone marrow donor (12 patients) and induced complete remission in 25% of the patients. Children treated with supportive care only (eight patients) had a 75% mortality with a very short survival time. These data confirm the superiority of allogeneic bone marrow transplantation over immunosuppressive therapy in children with severe aplastic anemia.

Effects of recombinant human erythropoietin in infants with the anemia of prematurity: a pilot study.

In an attempt to stimulate endogenous erythrocyte production and thereby provide an alternative to erythrocyte transfusions, we administered recombinant human erythropoietin (rHuEpo) in doses of 75 to 300 units/kg/wk to seven infants with the anemia of prematurity. Treatment was started between 21 and 33 days of life, maintained for 4 weeks, and was well tolerated. All the patients had low baseline serum erythropoietin levels. After rHuEpo therapy, the number of reticulocytes increased from a mean baseline count of 75 x 10(9)/L to 95, 141, and 165 x 10(9)/L on days 7, 10, and 14 of therapy, respectively. Correction or stabilization of the anemia was observed in six of seven patients, whose estimated total erythrocyte volume increased by 49% during therapy (vs a predicted increment of 18% in the absence of rHuEpo). In one patient, however, the hematocrit declined during the treatment, and in three of the responders a secondary fall in hematocrit was noted either during therapy or after its discontinuation. Serum iron and ferritin levels rapidly decreased after the initiation of rHuEpo therapy, and in most patients transient early thrombocytosis and late neutropenia were observed. These data suggest that rHuEpo may correct or stabilize the anemia of prematurity. Its effects, however, may be limited by a variety of factors, among which iron availability probably plays an important role. Controlled studies will be needed to confirm these preliminary observations.