[Asthma mortality in children].
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Biomedical subjects
Publications and source records attributed to R Mariani.
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BACKGROUND: Neonatal bacterial infections are potentially lethal. The infant must be started on an antibiotic regimen to cover the organisms most frequently implicated. Since the introduction of gentamicin therapy for neonatal infections, attention has focused on aminoglycoside pharmacokinetics in these very young patients. PATIENTS AND METHODS: The pharmacokinetics parameters of netilmicin during its first administration were analysed in 22 newborn infants with a gestational age over 34 weeks, aged 1 to 3 days, in whom a maternofetal infection was suspected. Netilmicin was given intravenously at a dose of 6 mg/kg/day in two daily injections for 35 minutes. Blood concentrations of netilmicin were measured from samples taken 5, 15, 30, 60 minutes and 2 1/2, 5 1/5 and 11 1/2 hours after injection. The patients were also given cefotaxime plus ampicillin. RESULTS: The kinetics were bicompartimental: prematurity, proven infections and other perinatal factors influenced the pharmacologic parameters and it was not possible to define a predictive formula for antibiotic administration. CONCLUSION: The blood levels of netilmicin must be monitored even in infants who were not born prematurely. Because of the large distribution volume and the long half-life, we propose a dose of 6-7.5 mg/kg given once daily.
Here, we present an analysis of clinical and biological data from 15 infantile visceral leishmaniasis recorded between 1985 and 1992 in the French department of Alpes-Maritimes. The association of fever, splenomegalia, tricytopenia remains the most constant and predictive of diagnosis (90% of cases). Bone marrow smear affirms diagnosis in 93% of cases. Leishmania infantum MON-1 is the causative agent. Except for one case of advanced stade with severe hepatic lesions and one case of clinical resistance to Glucantime, prognosis is favourable under treatment with antimony derivatives. The long term results for children considered cured after initial treatment is satisfactory. In the department, the number of cases of combined ages has increased regularly since 1985. The number of infantile forms has increased but now represents nevertheless no more than 28% of total cases. On the other hand, cases are increasing in adults and 40% of those adult cases are in HIV positive patients.
BACKGROUND--Acute dehydration with hypochloronatremic metabolic alkalosis is a classical complication of cystic fibrosis of the pancreas. Its progressive development as a revealing manifestation of the disease is rare as is its appearance in newborns. Case n. 1.--A 13 month-old girl was admitted because of status epilepticus. She was severely dehydrated (20% weight loss) but had normal diuresis. Investigations showed metabolic alkalosis, hypochloronatremia and hypokalemia. All the manifestations disappeared within 5 days with treatment but three sweat tests were abnormal. Case n. 2.--A 7 month-old girl was admitted because she suffered from progressive loss of weight (10%); she was dehydrated and had metabolic alkalosis plus hypochloronatremia, but her diuresis was normal. Two sweat tests were abnormal. Case n. 3.--A 4.5 month-old boy was admitted because he suffered from severe (12%) weight loss. His diuresis was normal despite dehydration; metabolic alkalosis and hypochloronatremia were found. Two subsequent sweat tests were abnormal. Case n. 4.--A 3 day-old girl was admitted suffering from meconium ileus. Two initial mechanism analysis for protein were abnormal. She had a cardiorespiratory arrest on the 13th day of life, when she had lost 14% of her birth weight; investigations showed metabolic alkalosis, hyponatremia (83 mEq/l), hypochloremia (45 mEq/l); kalemia was 5.9 mEq/l. Peritoneal dialysis was needed to correct hydroelectrolytic changes. A sweat test performed on the 26th day of life was also abnormal. Investigations performed during the periods of dehydration, and repeated later, showed transient functional kidney failure. None of the four patients had any respiratory or gastrointestinal clinical manifestations of cystic fibrosis. CONCLUSION--Metabolic alkalosis with hypochloronatremia plus progressive, severe dehydration in infants whose diuresis is paradoxically normal must be followed by examination for cystic fibrosis.
While blood norepinephrine and epinephrine levels are well established in adults, literature data concerning concentrations in young children are conflicting. This situation prompted us to assay plasma catecholamines in 86 healthy subjects aged two days to fifteen years using a sensitive high-performance liquid chromatography technique with electrochemical detection. Norepinephrine and epinephrine concentrations remained elevated up until 24 months of age, then dropped progressively to adult levels between 24 and 36 months, with fluctuations. These fluctuations, which reflect the sensitivity of children to environmental factors, prevented demonstration of any catecholaminergic secretory abnormality by blood assays in subjects under two years of age. Urinary assays, for which established values exist, are the only applicable procedure in such patients.
PCR was used to clone isolates of the human immunodeficiency virus type 1 (HIV-1) nef gene directly from peripheral blood leukocytes of HIV-1-infected individuals. A transient expression system with human CEM T cells was used to assess the effect of nef on CD4 antigen expression on the cell surface. We show that CD4 down-regulation is a frequent property of primary HIV-1 nef alleles. Mutations in conserved amino acid motifs of Nef disrupted CD4 down-regulation. Our observations strongly suggest that CD4 down-regulation reflects a conserved function of nef, which is selected in vivo in human HIV-1 infection. Methodology described here provides quantitative assays to establish whether alterations in nef correlate with the dynamics of disease progression in human AIDS.
The nef gene, which encodes related cytoplasmic proteins in both human (HIV) and simian (SIV) immunodeficiency viruses is dispensable for viral replication in vitro. In contrast, in vivo experiments have revealed that SIV nef is required for efficient viral replication and development of AIDS in SIV infected rhesus monkeys, thus indicating that nef plays an essential role in the natural infection. We show that expression of the Nef protein from the HIV-1 NL43 isolate in transgenic mice perturbs development of CD4+ T cells in the thymus and elicits depletion of peripheral CD4+ T cells. Thymic T cells expressing NL43 Nef show altered activation responses. In contrast, Nef protein of the HIV-1 HxB3 isolate does not have an overt effect on T cells when expressed in transgenic animals. The differential effects of the two HIV-1 nef alleles in transgenic mice correlate with down-regulation of CD4 antigen expression on thymic T cells. The differential interactions of the NL43 and HxB3 nef alleles with CD4 were reproduced in a transient assay in human CD4+ CEM T cells. Down-regulation of CD4 by nef in both human and transgenic murine T cells indicates that the relevant interactions are conserved in these two systems and suggests that the consequences of Nef expression on the host cell function can be analyzed in vivo in the murine system. Our observations from transgenic mice suggest that nef-elicited perturbations in T cell signalling play an important role in the viral life cycle in vivo, perhaps resulting in elimination of infected CD4+ T cells.
A prospective registration of incident cancers in childhood in two south-east regions of France since 1 January 1984 allows us to collect pertinent data on 875 cases throughout a period of 8 years. World age-standardised overall incidence rate is 137.63 cases/million/year. It is close to that reported in other white European. North American and Oceanian populations. The age-adjusted (age-standardised) relative frequency of each pathological group is: leukaemias 29.71%; central nervous system tumours 20.61%; lymphomas 12.75%; sympathetic tumours 9.03%; soft tissues tumours 7.37%; bone tumours 5.89%; kidney tumours 4.82%; epithelial tumours 3.83%; germinal and gonadal tumours 3.24%; retinoblastomas 2.11%; liver tumours 0.45% and others 0.14%. The comparison of these results with international available data shows that we record the world highest adjusted incidence rates for neuroblastomas (15.46) and rhabdomyosarcomas (7.04) and a high rate for Ewing's sarcomas (3.30); this fact will need to be confirmed by a longer period of observation, but even now the total number of cases (particularly for neuroblastoma) is high when compared with the data of other children registries which give rates for longer periods and for similar or larger populations.
The case of a 13 year old boy with a severe form of visceral leishmaniasis (VL) mimicking portal hypertension and of unfavorable outcome is presented. The authors stress the usefulness of investigating humoral immune response by using Western Blot assay in order to confirm the diagnosis when the parasite cannot be isolated. Since VL may be responsible for death if the treatment is delayed, screening of the disease in children presenting with chronic liver disease is important in endemic area.
Sertoli-Leydig tumors stem from the mesenchyma and sexual cords of the embryonic gonad. Two cases are reported. One manifested as symptoms of virilization in a 12 year old girl. The other patient developed adnexal torsion at the age of five years. Pelvic ultrasonography visualized the tumor in both cases. Increased production of ovarian androgens suggested the diagnosis in the first case. Histological studies disclosed intermediate differentiation in the first case and tubular differentiation in the second. These tumors usually exhibit low-grade malignancy and unilateral salpingo-oophorectomy ensures recovery in most instances.
BACKGROUND: About 60-70% of cases of anaphylactoid purpura suffer from abdominal colic, which may be quite severe. Because severe abdominal pain may be difficult to differentiate from a surgical abdomen, repeated examination and radiological studies are often necessary. CASE REPORT: A 4 year 7 month-old girl suffered from abdominal pain, vomiting and the skin rash characteristic of anaphylactoid purpura. Because of worsening of the abdominal manifestations, a Gastrografin enema was given. The result was normal but 48 hours later, the child suffered from acute abdominal colics plus symptoms of shock. X-ray examination revealed a large distension of colon and persistence of the contrast material. Exploratory laparotomy failed to find any perforation or necrosis. The colon was drained and the child was given parenteral nutrition and antibiotics. Blood culture showed Klebsiella pneumoniae. An upper gastrointestinal roentgenogram using meglumine ioxitalamate was performed 19 days later because of recurrence of the abdominal pain. This showed a submucosal hemorrhage in the first small bowel loop. Increased abdominal distension occurred 3 days later and the results of X-rays were similar to those seen the Gastrografin enema. A second enema using meglumine ioxitalamate was performed 3 days later. It showed sigmoid constriction but a second laparotomy failed to confirm this obstruction. The child died a few hours later despite ileostomy and antibiotics. CONCLUSIONS: The severe colectasy seen in this case of anaphylactoid purpura indicates that caution is required in performing enemas in acute digestive complications of this disease. Such X-ray studies should not be repeated and all water-soluble contrast material should be voided as soon as possible.
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Assays of urinary catecholamines and their metabolites (HVA, VMA, dopamine) permit biochemical diagnosis of neuroblastoma in approximately 80% of patients. The urinary methylated catecholamine metabolites normetanephrine (NMN), metanephrine (MN), and 3-methoxytyramine (3-MT) were analyzed in 18 patients with neuroblastoma and compared with reference values established for 69 healthy pediatric controls. All 18 neuroblastoma patients had raised urinary excretion of at least one of the three commonly assayed metabolites (HVA, VMA, dopamine). Similarly, raised urinary excretion of a methylated metabolite was noted in all but one of the neuroblastoma patients. The 3-MT level was pathologic in 16 of the 18 patients (89%). In this series, 3-MT assay sensitivity was sufficient to warrant trials on a larger population including comparison with patients considered nonsecretors by routine assay procedures.
A five-year-old child presented with a large undifferentiated sarcoma in the right lung. Urinary catecholamines were assayed as tumor markers. A short-lived but significant rise in urinary norepinephrine and dopamine followed by rises in urinary levels of corresponding breakdown products were evidenced.
Traumatic lung pseudocyst is a lung injury due to closed chest trauma. The authors report one case in a 20 month-old boy. This cavitary lesion is underestimated since associated lesions often mask the pneumatocele. Its frequency is higher in children and young adults. Diagnosis can be assessed early when chest film shows the characteristic cavitary lesion. Computed tomography may be helpful in the recognition of paramediastinal traumatic lung pseudocyst. Since the lung pseudocyst usually involves, no aggressive therapy is justified.
Twenty-six cases of congenital toxoplasmosis observed in the department of Alpes Maritimes (Chief Town: Nice) between 1984 and 1990 are reported. All affected children were treated by pyrimethamine and sulfonamide as soon as the diagnosis was established. None of them exhibited serious sequellae. In most cases (58%) there were no clinical manifestations in agreement with other reports. Benign manifestations were observed in 10 cases (38%): 4 chorioretinitis; 4 intracranial calcifications: 1 febrile seizure; 7 hyperproteinorachias: 3 isolated and 4 associated with other signs. In 3 cases, secondary lesions appeared during the treatment period. This confirms the need for active therapeutic research in order to decrease the risk of late ocular complication which is the major problem of the disease.
Acute leukemia in the newborn child is a rare event. The clinical and biological characteristics differ from those normally encountered in the older child. Tumoral syndrome and extra-medullar locations are frequently described in the literature. Many authors have noted the difficulty of diagnosis due to the immaturity of the malignant proliferation. While it is generally agreed that therapeutic abstention is justified in the leukemoid reaction in Down's syndrome, the choice is debatable in the phenotypically intact newborn. For this reason, blastic karyotype analysis is essential and may provide guidelines when considering treatment. We report on a case history of acute monoblastic leukemia with translocation 9;11 that was diagnosed at birth in a normal newborn infant. The juxtaposition of c-ets 1 protooncogene and the beta-interferon gene has been associated with this kind of cytogenetic disease and probably constitutes a model for human leukemogenesis.