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Biomedical subjects

L Breysem

Publications and source records attributed to L Breysem.

14 recordsLinked to original sources

Multicystic kidney dysplasia: a prospective study on the natural history of the affected and the contralateral kidney.

UNLABELLED: In a 6-year period, 41 young infants with multicystic kidney dysplasia were seen in our department. In 30 cases, the diagnosis had already been suspected by prenatal ultrasonography. A prospective protocol was proposed to the parents which comprised ultrasound evaluation every 3 months until the age of 24 months and renal function assessment at the age of 18 months. In 33 patients, the study was completed as scheduled. At the start of the study, the maximal diameter of the multicystic kidney was above the mean length of normal kidneys in all cases where precise measurement was possible. Within 24 months, 7 of the dysplastic kidneys disappeared, 20 regressed in size, 1 remained unchanged and only 5 increased in size. Between the age of 0 to 3 months, renal length of the contralateral kidney was within the normal range in 19 infants and above +2SD in 14 cases. At the age of 18 to 24 months, renal length was, with few exceptions, between 0 and +4SD. Inulin clearance was normal in all 33 individuals with a median value of 112 ml/min per 1.73 m2. CONCLUSION: As a rule, multicystic kidneys shrink in the first 2 years of life. In most cases the contralateral kidney maintains a normal renal function as a consequence of progressive compensatory hypertrophy.

Child↗

Treatment of Pseudomonas aeruginosa lung infection in cystic fibrosis with high or conventional doses of ceftazidime.

In cystic fibrosis patients with Pseudomonas aeruginosa colonization and increasing pulmonary infection, ceftazidime 150 mg/kg/day was compared with 320 mg/kg/day. Changes in clinical findings, laboratory tests, pulmonary function and chest radiographs were evaluated after 14 days of treatment in hospital. Both treatments were associated with a significant improvement, but the higher dose did not offer an additional benefit. An increase in alanine aminotransferase (ALT) occurred after both treatments; with a significantly greater increase after the high-dose therapy (mean increase +/- S.E.M. 8% +/- 2% vs 2% +/- 1 %; P < 0.01). All but one of the ALT values after treatment were within normal limits.

Adolescent↗

Caroli's syndrome.

We report the case of a two-year-old girl presenting with recurrent episodes of fever and hepatomegaly. High resolution ultrasound (US) and computed tomography (CT) of the liver show dilated intrahepatic bile ducts with bridge formation and intraluminal protrusions. The extrahepatic bile duct is normal. The diagnosis of irregular dilated bile ducts with signs of cholangitis and cholangiolitis is proved by liver biopsy. The histologically associated congenital liver fibrosis in our patient enter into the Caroli's syndrome. We illustrate the high resolution US and the CT findings in this case.

Bile Ducts, Intrahepatic↗

Bronchopulmonary dysplasia: correlation of radiographic and clinical findings.

BACKGROUND AND PURPOSE: Abnormalities of the chest wall have been described in bronchopulmonary dysplasia (BPD). Clinical, radiographic and pulmonary function variables were evaluated in 1-year-old children ventilated because of neonatal lung disease in order to quantify these thoracic changes and to evaluate the lung disease. METHODS: The pulmonary status of 51 infants with neonatal lung disease requiring artificial ventilation was reevaluated clinically and radiographically at the age of 1 year. Twenty-two of these infants had developed BPD. Thoracic depth and width were measured clinically and on chest X-ray. The Toce score evaluated the presence of cardiomegaly, hyperinflation, emphysema and interstitial lung disease. Lung function was measured after sedation using previously reported methods. In BPD patients, Toce score and lung function were determined and compared at 1 month and at 1 year of age. RESULTS: In BPD patients, chest depth was significantly smaller when measured clinically as well as on chest radiograph (P < 0.05; Mann-Whitney U-test). There was a statistically significant correlation between chest depth measured clinically and on chest X-ray. Toce score was significantly higher in BPD patients (P < 0.05). In BPD patients intersitial abnormalities and decreased lung compliance were more frequent at the age of 1 month than at the age of 1 year. At the age of 1 year, hyperinflation was more frequent and at that time increased airway resistance was still noted. Thus the type of X-ray abnormality reflects the type of lung function disturbance. CONCLUSION: The flatness of the chest is most likely a consequence of the long-standing lung function abnormalities.

Bronchopulmonary Dysplasia↗

The Holt-Oram syndrome: radiological approach.

The clinical and radiological findings in a female child with Holt-Oram syndrome are reported. The most important features are cardiac anomalies associated with typical anomalies of the skeleton of the forearms.

Child, Preschool↗

High-dose growth hormone treatment of short children born small for gestational age.

The effect of GH administration was evaluated over 2 yr in 50 short, prepubertal, non-GH deficient children born small for gestational age, who had been randomly allocated to a group receiving no treatment or daily sc GH treatment at a dose of 0.2 or 0.3 IU/kg. At the start of the study, mean age was 5.2 yr, bone age was 4.0 yr, height SDS was -3.5, height velocity SDS was -0.8, weight SDS was -2.7, and body mass index SDS was -1.9. Catch-up growth was observed in none of the untreated and all of the treated children. The response to GH treatment included a near doubling of growth velocity and of weight gain and a mean height increment of more than 2 SDS. GH treatment was associated with a distinct acceleration of bone maturation. The differences between the growth responses evoked by the two GH doses were minor. The prepubertal GH-induced catch-up growth was associated with elevated serum concentrations of insulin, insulin-like growth factor-I, insulin-like growth factor binding protein-3, and osteocalcin, whereas insulin-like growth factor-II levels remained unaltered. GH treatment was well tolerated. In conclusion, high-dose GH administration over 2 yr is emerging as a potential therapy to increase the short stature that results from insufficient catch-up growth in young children born small for gestational age. The long-term impact of this approach remains to be delineated.

Age Determination by Skeleton↗

Localized brain proton NMR spectroscopy in young adult phenylketonuria patients.

Localized proton magnetic resonance spectroscopy with short echo time (TE = 20 ms) was used to investigate biochemical changes in the cerebral white matter of 20 young adult patients (median 19 years) with phenylketonuria (PKU). Results were compared with those of a group of 12 age-matched healthy volunteers (median 25 years). Concentrations of N-acetyl-aspartate (NAA) and choline (Cho) relative to creatine (Cr) were unchanged. However, concentrations of inositol (Ins) relative to creatine were found to be significantly lower (P < 0.001) in the PKU patients (0.30 +/- 0.09 versus 0.57 +/- 0.17). Individual inositol concentrations did not correlate with age, diet, serum phenylalanine (Phe) levels or extent of pathological regions in the T2-weighted images. The lack of correlation with individual data suggests that the decreased inositol concentration could be related to a metabolic deficiency during fetal development. No signal from the phenyl ring protons of phenylalanine was detected in the PKU patients (phenylalanine serum concentration < or = 1.27 mM), which suggests that concentration of phenylalanine may be lower in brain than in serum.

Adolescent↗

Late-onset Bochdalek hernia with a rare postoperative complication: case report.

We report a case of a 7-year-old-girl with a late-presenting Bochdalek hernia. The postoperative course was complicated by splenic torsion and subsequent total splenic infarction. Total splenic infarction due to splenic torsion has been reported in children with "wandering spleen" and in incarcerated Bochdalek hernias, but to our knowledge, not as a postoperative complication of Bochdalek hernia repair.

Child↗

Stenosing hemorrhagic ureteritis in a child with Henoch-Schönlein purpura: CT appearance.

Henoch-Schönlein purpura is rarely considered in the differential diagnosis of ureteral obstruction in children. Renal ultrasound and intravenous urography may reveal hydroureteronephrosis but usually fail to yield a specific diagnosis. In this child CT differentiated between an intrinsic ureteral stenosis (hemorrhagic ureteritis) and an extrinsic ureteral stenosis (retroperitoneal fibrosis), two rare complications of Henoch-Schönlein purpura. The CT findings of hemorrhagic ureteritis are described.

Child↗