The impact of severe late-effects after 12 Gy fractionated total body irradiation and allogeneic stem cell transplantation for childhood leukemia (1988-2010).

This study consists of a retrospective study including 71 childhood leukemia survivors (36 females) treated with allo-HSCT 12 Gy fractionated total body irradiation (fTBI) conditioning, with a median age of 25.0 y at time of follow-up and a median delay of 14.8 y since the graft.

Second malignant neoplasm following childhood cancer: A nested case-control study of a recent cohort (1987-2004) from the Childhood Cancer Registry of the Rhône-Alpes region in France.

From a population-based cohort of cases of first cancers diagnosed between 1987 and 2004, before the patient's age of 15 years, the authors conducted a nested case-control study, matching 64 patients who experienced a second malignant neoplasm (SMN) with 190 controls. SMNs comprised 10 leukemia or myelodysplastic syndromes, 5 lymphomas induced by Epstein-Barr virus after allograft, and 49 solid tumors, including mainly 25 carcinomas (17 of the thyroid), 9 bone sarcomas, and 7 central nervous system (CNS) tumors. The median latency occurrence was 6.

Age at Birth of First Child and Fecundity of Women Survivors of Childhood Acute Lymphoblastic Leukemia (1987-2007): A Study of the Childhood Cancer Registry of the Rhône-Alpes Region in France (ARCERRA).

We studied the fecundity of 174 successive ALL (1987-2007) in females of the Childhood Cancer Registry of the Rhône-Alpes Region (ARCERRA) with a median age at follow-up of 25.6 years (18.0-37.

Academic difficulties and occupational outcomes of adult survivors of childhood leukemia who have undergone allogeneic hematopoietic stem cell transplantation and fractionated total body irradiation conditioning.

We studied academic and employment outcomes in 59 subjects who underwent allogeneic hematopoietic stem cell transplantation (a-HSCT) with fractionated total body irradiation (fTBI) for childhood leukemia, comparing them with, first, the general French population and, second, findings in 19 who underwent a-HSCT with chemotherapy conditioning. We observed an average academic delay of 0.98 years among the 59 subjects by Year 10 of secondary school (French class Troisième), which was higher than the 0.

Final height and body mass index after fractionated total body irradiation and allogeneic stem cell transplantation in childhood leukemia.

Impaired linear growth has been reported in patients treated during childhood with allogeneic stem cell transplantation and fractionated total body irradiation (fTBI). The objective of this study was to determine the final height and body mass index (BMI) achieved. Forty-nine patients with leukemia were included and surveyed for more than 5 years.

Second malignant neoplasms following childhood cancer: a study of a recent cohort (1987-2004) from the childhood cancer registry of the Rhône-Alpes region (ARCERRA) in France.

Studies of second malignant neoplasms (SMNs) in childhood are generally conducted in old cohorts. The aim of this study was to determine the actual incidence of all SMNs in a recent cohort. The authors studied a cohort of 2907 children included in the population-based Childhood Cancer Registry of the Rhône-Alpes Region for a first cancer diagnosed between 1987 and 2004.

[Long term mortality of five-year survivors of childhood cancer in Rhône-Alpes region].

Background: The population of survivors of childhood cancer is currently growing. Studies from other countries have shown an increased risk of late mortality. In order to measure this risk within a French cohort, the mortality of children who had survived five years from a cancer diagnosis were compared to the mortality of the general population, according to follow-up interval and cancer and treatment characteristics.

Trends in treatment-related deaths (TRDs) in childhood cancer and leukemia over time: a follow-up of patients included in the childhood cancer registry of the Rhône-Alpes region in France (ARCERRA).

Background: We assessed the number and causes of treatment-related deaths (TRDs) in childhood cancer over time and correlated them with adherence to therapeutic guidelines.

Procedure: We compared two cohorts of children of the Childhood Cancer Registry of the Rhône-Alpes Region: Cohort I (1987-1992, 909 patients) and Cohort II (1996-1999, 648 patients).

Results: In all cancers together, 75 TRDs were reported in Cohort I and 24 in Cohort II (P = 0.

Childhood cancer survival in France, 1990-1999.

The aim of this study was to describe the overall survival after childhood cancer in France using follow-up data from regional population-based registries. The survival of children (aged under 15 years) diagnosed with a cancer during 1990-1999 was analysed. For all cancers, the survivals were, respectively, 90.

[Childhood cancer incidence and survival rates in the Rhône-Alpes regional paediatric registry 1987-1999].

Unlabelled: Cancer is rare in children, and pediatric malignancies represent only 1% of all cancers.

Objectives: The cure rate is high and increasing, and ongoing data collection is therefore warranted.

Materials And Methods: Here we report the incidence and survival rates of childhood cancers between 1987 and 1999 in the Rhône-Alpes region of France.

Cancer incidence among children in France, 1990-1999.

Background: Cancer is the second most important cause of death for children aged less than 15 years in France, unintentional injuries being the leading cause. The aim of the present study was to estimate the incidence of childhood cancer from six Childhood Cancer Registries covering 32% of France.

Procedure: Incident cancer cases diagnosed between 1990 and 1999 in children (0-14 years) resident in the administrative areas covered by each Registry were included.

[Symptomatic rickets in adolescents].

Although systematic vitamin D supplementation in adolescents remains debated, rickets is nevertheless a well recognized pathology in this age group. Adolescence is an at-risk period because of rapid growth, insufficient calcium intake and/or vitamin D status. Surveys have shown that calcium intake is insufficient (< 1000 mg a day) in 45% of boys and 71% of girls and that vitamin D status is deficient (25-OH-D < 10 ng/ml).

[The association of polyarteritis nodosa and familial Mediterranean fever].

Introduction: Some necrotizing vasculitis may be associated with familial Mediterranean fever (FMF). We report a new case of polyarteritis nodosa (PAN) that preceded the diagnosis of FMF.

Observation: A young woman of Turkish origin had a long childhood history of inflammatory arthralgia and myalgia, leading to the provisional diagnosis of chronic juvenile arthritis, then, after a confirmative muscle biopsy, to the diagnosis of PAN, whose outcome remained benign.

Myelofibrosis: an unusual presentation of vitamin D-deficient rickets.

Unlabelled: We present the case of a breast-fed 5-month-old infant who presented with pancytopenia, secondary to intense myelofibrosis during the winter months because of undiagnosed rickets. The patient responded to oral vitamin D with rapid resolution of symptoms. Secondary hyperparathyroidism was the probable cause of the myelofibrosis.

Is there IgA from gut mucosal origin in the serum of children with Henoch-Schönlein purpura?

Thirty-two children with Henoch-Schönlein purpura with or without renal symptoms were studied to characterize the IgA hyperglobulinemia observed in the serum of most patients. It was shown that only the IgA1 subclass concentration was increased. Secretory IgA and IgA to gliadin levels were frequently increased in serum, with a good correlation between them.

[A familial case with generalized resistance to thyroid hormones].

Background: The syndrome of generalized resistance to thyroid hormones is more frequent than was thought.

Case Reports: A 13-year old girl was examined for her short stature. Evaluation of her thyroid function showed increased levels of IT3 and IT4 and normal value of TSH; she also had mosaic Turner's syndrome.

Fulminant liver failure in a 12-year-old girl with sickle cell anaemia: favourable outcome after exchange transfusions.

Unlabelled: Acute liver failure is unusual in sickle cell anaemia. We describe a child with homozygous sickle cell anaemia who developed acute liver disease of abrupt onset during an episode of limb pain. She presented with sudden onset of persistent vomiting, headache, lethargy, epistaxis, and painful liver enlargement.

[Follow-up of allogenic bone marrow graft with DNA polymorphisms. Use of two minisatellite MS31 and MS43 probes. Comparison with data of chromosomal polymorphisms].

Objectives: Allogenic bone marrow transplantation is widely used to treat many diseases of the haemopoietic system as well as metabolic disorders. Follow-up is essential to assess acceptance, rejection or post-graft relapse. This study was undertaken to evaluate the usefulness of the minisatellite probes MS31 and MS43 used as a routine follow-up test after bone marrow transplantation.

A mutation located at the 5' splice junction sequence of intron 3 in the p67phox gene causes the lack of p67phox mRNA in a patient with chronic granulomatous disease.

Chronic granulomatous disease (CGD) is due to a functional defect of the O2(-)-generating NADPH oxidase of neutrophils. Mutations resulting in CGD have been shown to occur in only four genes, thus identifying the main components of the oxidase complex, namely the two subunits of a membrane-bound cytochrome b and two cytosolic factors of activation of 67 kD (p67phox) and 47 kD (p47phox). The present study deals with the biochemical and genetic analysis of the defect in a patient suffering from a p67phox-deficient form of CGD.

[Klinefelter syndrome in 1993. Results of a multicenter study on 58 cases and review of the literature].

A retrospective multicenter study found 58 cases of Klinefelter syndrome of which 23 (39%) were diagnosed before puberty. Although as common as Down syndrome, Klinefelter syndrome is underdiagnosed and often recognized only in adulthood. Suggestive manifestations in infants, children, and teenagers include facial dysmorphism, micropenis, and delayed speech and should lead to examination of the karyotype.

Transient erythroblastopenia of childhood presenting with echovirus 11 infection.

We report a case of transient erythroblastopenia in a three-year-old girl presenting with echovirus 11 infection. Viral infection was demonstrated by isolation of echovirus 11 in stool cultures and the presence of echovirus 11-specific IgM antibody in serum. We suggest that echovirus may have played a role in the pathogenesis of transient erythroblastopenia of childhood in this patient.

Bone marrow transplantation for acute myeloblastic leukaemia: an EBMT Leukaemia Working Party prospective analysis from HLA-typing.

The optimal post-remission therapy for patients with acute myeloblastic leukaemia remains controversial. Allogeneic bone marrow transplantation, autologous bone marrow transplantation, and consolidation chemotherapy are the major options. In order to evaluate their respective value the European Group for Bone Marrow Transplantation conducted a prospective registration study.

[Citrullinemia: management and clinical course. Apropos of a familial case].

The authors report two cases of citrullinemia in siblings which add to 68 observations from the literature. They overview the clinical presentation, diagnosis and therapeutic management of the disease. The prognosis of severe neonatal form remains poor but an early adequate management may contribute to an acceptable outcome.

[Study of serum kinetics of vancomycin during the "antibiotic-lock" technique].

The serum kinetics of vancomycin was studied in two patients aged 3 and 15 years during antibiotic therapy for catheter related sepsis associated with Staphylococcus epidermidis. Vancomycin was administered, simultaneously, by parenteral conventional doses (30 mg/kg/day div q 8 h) and using the antibiotic-lock technique in the infected catheter at a high concentration (150 mg/ml) during one hour, 3 hours after each infusion. Pharmacokinetics data did not show any significant change in the serum kinetics of the antibiotic.