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Biomedical subjects

A Fretzayas

Publications and source records attributed to A Fretzayas.

At least 19 recordsLinked to original sources

Clinical expression of co-inherited Dubin-Johnson and thalassaemic heterozygous states.

Dubin-Johnson syndrome is a worldwide prevalent familial conjugated hyperbilirubinaemia. The identification of multidrug resistance-associated protein 2 provided an understanding of the complex metabolic impairment involved in this syndrome. We report the first family with modified clinical expression of Dubin-Johnson heterozygous state due to genotypic interaction with co-inherited beta and deltabeta thalassaemia, an interaction that has never been described.

Adult↗

The effect of inhaled budesonide on adrenal and growth suppression in asthmatic children.

The present authors evaluated adrenal reserve in asthmatic children on long-term inhaled corticosteroids and whether possible adrenal suppression could be predicted by growth retardation. Low-dose synacthen test (0.5 microg x 1.73 m(-2)) was performed in 72 asthmatic children with a median age of 9.4 (range 4.2-15.7) yrs on long-term treatment (median 18 (range 6-84) months) with low-to-moderate doses (median 363 (range 127-1012) microg x m(-2)) of inhaled budesonide, as well as in 30 controls. Adrenal suppression was considered as a peak serum cortisol <495 nmol x L(-1). The current authors calculated height standard deviation score (HSDS) at the time of testing and height velocity SDS (HVSDS) in the preceding year. Mean HSDS was 0.06+/-1.3 and HVSDS was -0.9+/-2.3. Adrenal suppression was disclosed in 15 asthmatic children (20.8%). There were no differences in HSDS and HVSDS between children with and without adrenal suppression. There was no correlation between peak cortisol response and dose or duration of treatment. However, a positive relationship between HVSDS and duration of treatment was noted. These data suggest that long-term treatment of asthmatic children with low and moderate doses of inhaled budesonide may result in mild adrenal suppression that cannot be predicted by growth deceleration. The negative influence of inhaled corticosteroids on growth becomes less the longer the duration of treatment.

Administration, Inhalation↗

Invasive meningococcal disease presenting as Henoch-Schonlein purpura.

Henoch-Schonlein purpura (HSP) is an acute systemic form of vasculitis that has been associated with a number of viral and bacterial infections. Described here are the cases of two children with invasive meningococcal disease who presented with clinical and laboratory findings typical of HSP. Meningococcal infection may have been the trigger for the manifestation of HSP in these patients.

Bacteremia↗

Circulating cytokines in patients with cat scratch disease.

Levels of circulating interleukin (IL)-2, IL-6, and IL-10, measured by enzyme-linked immunosorbent assay, were significantly higher in patients with cat scratch disease (CSD) than in healthy control subjects; no induction of IL-12 was observed, and levels of interferon-gamma and IL-4 were generally not detectable. This is the first report showing increased circulating cytokine levels in patients with CSD. The induction of these mediators can partly explain some clinical and pathological features of the disease.

Antibodies, Bacterial↗

Unsuspected extralymphocutaneous dissemination in febrile cat scratch disease.

Cat scratch disease (CSD) commonly manifests as regional self-limited lymphadenitis. However, dissemination of the infection to distant multiple sites may occur even in immunocompetent patients. We report a series of 11 children with fever and extralymphocutaneous manifestations of CSD, in order to highlight potential multiorgan involvement in patients with febrile CSD. To be eligible for enrollment, patients had to present with involvement of sites other than regional lymph nodes. The diagnosis was based on suggestive clinical criteria, histological findings and positive serology. The utilization of ultrasound imaging revealed hepatic lesions in 3 children and splenic lesions in 8 children, whereas osteolytic lesions were observed in 4 children by bone scan. Hepatic or splenic involvement was not suggested by clinical signs or biochemical investigation in 2/3 and 6/8 children, respectively. Bone involvement was supported either by relative symptoms or signs. Our findings indicate that, in the presence of fever, extralymphocutaneous manifestations have to be anticipated in patients with clinically suspected CSD. The systematic use of imaging modalities in patients with serologically documented Bartonella henselae infection could contribute to a better understanding of the clinical spectrum of CSD.

Adolescent↗

Splenic granulomatous lesions in immunocompetent pediatric patients with visceral leishmaniasis.

Visceral focal lesions are rarely encountered in patients with leishmaniasis despite the fact that splenomegaly, and to a lesser extent hepatomegaly, predominate among the clinical features of the disease. We present, using high resolution ultrasonography, the first 3 reported cases of focal splenic involvement in immunocompetent children with visceral leishmaniasis. All patients showed a prompt response to the conventional anti-leishmanial treatment administered. This favorable outcome indicates that localized splenic lesions should not be considered as a poor prognostic factor and that alteration of the standard anti-leishmanial regimens is not justified.

Antiprotozoal Agents↗

Polymorphonuclear elastase as a diagnostic marker of acute pyelonephritis in children.

OBJECTIVE: Experimental evidence suggests that neutrophils and their metabolites play an important role in the pathogenesis of pyelonephritis. The aim of this study was to investigate the diagnostic value of polymorphonuclear elastase-a(1)-antitrypsin complex (E-a(1)-Pi) for the detection of acute pyelonephritis in children. METHODS: Eighty-three patients, 29 boys and 54 girls, 25 days to 14 years of age, with first-time symptomatic urinary tract infection were prospectively studied. Fifty-seven healthy children served as controls. Dimercaptosuccinic acid (DMSA) scan and voiding cystourethrography were performed in all patients. Plasma and urinary E-a(1)-Pi, C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), neutrophil count, urinary N-acetyl-beta-glucosaminidase (NAG), N-acetyl-beta-glucosaminidase b (NAG b), and creatinine levels were measured in all patients on admission and 3 days after the introduction of antibiotics. The same markers were also measured in the control subjects. RESULTS: Planar DMSA scintigraphy demonstrated changes of acute pyelonephritis in 30 of 83 children (group A). It was normal in the remaining 53 children (group B). The sex and age distributions were not significantly different between the 2 groups, as well as between the patients and the control subjects (group C). Nineteen of the 53 children with a normal DMSA had body temperature >/=38 degrees C, whereas all but 4 children with abnormal DMSA had temperature >/=38 degrees C. Therefore, the temperature was significantly different between these 2 groups. The sensitivity and specificity of fever (>/=38 degrees C) as an indicator of renal involvement based on isotopic findings were 86% and 64%, respectively. Given the significant number of the febrile children with normal DMSA scintiscans, group B was subdivided into B(1) with 19 febrile children (14 boys and 5 girls) and B(2) with 34 children whose body temperature was below 38 degrees C (8 boys and 26 girls). The sex and age distribution was significantly different between groups B(1) and B(2). The mean age of group B(1) was.78 years (range: 28 days to 9 years; median:.25 years; standard deviation: 2.1). All but 1 child in this group were younger than 1 year of age. In contrast, in group B(2), there were only 4 infants, the remaining 30 children were older than 2.5 years (mean age: 6 years; median: 7 years; standard deviation: 3.5; range: 34 days to 12 years). The mean duration of fever before hospital admission was 2.8 days for group A and 1.8 days for group B(1). This difference was not statistically significant. Similarly, body temperature was not significantly different between these 2 groups. The distribution of plasma E-a(1)-Pi values was normal in the control subjects. The sensitivity and specificity of plasma E-a(1)-Pi, as an indicator of renal involvement, were 96% and 50%, respectively, taking the 95th percentile of the reference range as a cutoff value. However, considering as a cutoff value the level of 72 microg/dL (95th percentile of group B(2)), its sensitivity and specificity were 74% and 86%, respectively. Plasma E-a(1)-Pi levels were significantly elevated in group A compared with group B and in both groups, the plasma E-a(1)-Pi values were significantly higher than in the control subjects. A significant difference also was noticed between group A and each of the subgroups B(1) and B(2) and also between the subgroups themselves. Plasma E-a(1)-Pi concentrations correlated significantly with neutrophil count in groups A (r =.3), B (r =.4), and B(2) (r =.46), but the correlation was not significant in group B(1.) ESR levels showed, among the different groups, similar differences with those of E-a(1)-Pi values. Unlike E-a(1)-Pi, CRP levels were comparable between groups A and B(1), which both consisted of febrile children. Neutrophil count was not significantly different between subgroups B(1) and B(2). (ABSTRACT TRUNCATED)

Acute Disease↗

Multiorgan involvement in systemic cat-scratch disease.

Cat-scratch disease is considered in the differential diagnosis of benign regional lymphadenopathy. We describe a case of cat-scratch disease in a 12-year-old boy with multiple bony, hepatic and splenic lesions which resolved with chemotherapy. The present case with simultaneous multiorgan involvement supports the view of a systemic nature of the disease.

Abscess↗

High mebendazole doses in pulmonary and hepatic hydatid disease.

Thirty nine children with 71 hydatid cysts were given mebendazole orally in a dose of 100-200 mg/kg/day for 12 weeks and were followed up for a mean (SD) of 63 (24) months. Twenty children (three of them after a second course) were cured and another two avoided at least one operation. No serious side effects of the drug were observed.

Administration, Oral↗

[Cat-scratch disease associated with osteomyelitis].

A case of cat-scratch disease in an 8 year-old girl is reported. The inoculation injury followed by regional lymphadenopathy was typical of Parinaud syndrome. This patient also had vertebral lesions on scintigraphy, x-rays and CT scan. While the oculoglandular localization spontaneously improved within a few weeks, osteomyelitic lesions remained grossly unchanged after almost 2 years of follow-up.

Cat-Scratch Disease↗

Radiation dose to the bladder wall and the gonads from direct radionuclide cystography in children.

A dynamic spherical bladder model, previously developed in our laboratory, was implemented to evaluate the absorbed dose to the bladder wall from 99mTc-pertechnetate and direct radionuclide cystography (DRC). This model takes into account the flow rate of normal saline into the bladder and the bladder volume as a function of the filling time. The DRC examinations of 36 children were analysed and calculations were carried out in order to estimate (a) the absorbed dose to the bladder during and following the DRC examination and (b) the gonadal dose. It is shown that the estimated dose to the bladder wall is much greater than that estimated by static models. It is also shown that the absorbed dose to the bladder due to the activity in the residual urine following the examination is even greater than the dose received during the DRC procedure. Our results indicate that in order to reduce the radiation burden, besides the so far proposed precautions, concerning the shortening of the DRC procedure, the patient should also be well hydrated and encouraged to void frequently after the examination.

Child↗

RES phagocytosis in children with homozygous beta-thalassemia in relation to blood transfusion.

In order to investigate the effect of blood transfusion on the RE cell function in thalassemia major, 14 children with homozygous beta thalassemia were studied prior to and 7 to 10 d following blood transfusion, when, according to previous studies, spleen reaches its minimum size. The denatured human albumin (DHA) low and large dose clearance techniques were used in order to estimate the maximum RES phagocytic capacity. It is shown that, despite the confirmed post-transfusion high hepatosplenic circulation, the RE cell phagocytic capacity is not significantly affected. Furthermore the RES phagocytic capacity of beta-thalassemics is shown to be significantly higher than that of normal controls, and this can probably be attributed to the RES hyperplasia accompanying the disease.

Adult↗

Statistical aspects of hydatid disease in Greek adults.

A retrospective study was undertaken to estimate the incidence of hydatidosis among Greeks over 18 years of age. Discharge and operating room records in all community and private hospitals during the period 1 January 1969 to 31 December 1975 were reviewed, and new hydatid patients were registered. Hydatid disease was diagnosed in 4,202 adults. The average annual incidence of all hospital cases was 9.77 per 100,000 population. The diagnosis was confirmed at surgery in 3,397 patients (80.84%). The average annual surgical incidence was 7.89 per 100,000 inhabitants. The incidence of the disease varied among different geographic areas in the country; its percentage was highest in rural areas. In this series, both sexes were equally involved (P greater than 0.1). Solitary organ involvement occurred in 95.88% of cases. Cysts were localized in the liver in 69.64% and in the lungs of 23.43% of the cases with single organ involvement. Liver involvement was more frequent in women, while lung involvement was more frequent in men (P less than 0.001).

Adult↗

Grading of vesicoureteral reflux by radionuclide cystography.

Thirty-six children with urinary tract infection aged 6 months to 14 years (mean age 4 1/2 years) were studied sequentially using direct radionuclide (RNC) and conventional voiding cystourethrography (VCU). Vesicoureteral reflux (VUR) was detected equally well by both methods. Twenty-seven refluxing ureters were found by RNC, 23 by VCU and 22 by both methods. Radiologic grade of reflux may be determined approximately with the isotope technique from the volume of regurgitating urine and duration of reflux, at a much decreased radiation exposure. Residual urine was also measured by RNC and found to be higher in children with VUR. RNC is a reliable method for detecting and grading VUR and should effectively replace VCU as the follow-up examination of choice.

Adolescent↗