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Biomedical subjects

J P Pracros

Publications and source records attributed to J P Pracros.

At least 19 recordsLinked to original sources

Complications of Ilizarov leg lengthening: a comparative study between patients with leg length discrepancy and short stature.

The Ilizarov technique has been used to treat severe limb length discrepancy and short stature. However, complications of this treatment are frequent. Between 1984 and 2001, 57 patients (94 tibias) had an Ilizarov procedure for limb lengthening. Twenty patients had limb discrepancy and 37 had short stature. Their mean age was 20.2 years (range 15-34). The average limb lengthening was 8.37 cm (range 3.2-14.7), which was equivalent to 26% (range 9.2-60%) average tibial lengthening. A total of 90 complications were observed. Thirty-three unplanned procedures were required during the lengthening programme. Two patients stopped the lengthening programme. There was no difference in the complications in leg lengthening using Ilizarov technique between the group of patients with leg length discrepancy and the group with short stature. A good knowledge of the Ilizarov technique is necessary to perform a lengthening programme with a low rate of complications.

Adolescent↗

[Value of contrast-enhanced MR enterography in pediatric Crohn's disease: preliminary study].

OBJECTIVE: Feasibility study of contrast enhanced MR enterography without enteroclysis as a new diagnostic tool for children with known or suspected Crohn's disease. METHODS: We prospectively included 15 children, 8-18 years old, with clinical suspicion of Crohn's disease. MR enterography without enteroclysis was performed on a 1,5 T clinical MR system. A total of 1000 ml of mannitol 5% was orally administered 60 minutes prior to MRI. Coronal and axial breath-hold sequences were acquired. The following sequences were obtained: True-FISP, FLASH T1 2D/3D with Fat saturation before and after gadolinium injection. Two radiologists, blinded to patient information, independently reviewed all examinations to record image quality, the degree of distension of the distal ileum, the presence of abnormal bowel segments and the presence of extra-intestinal complications. MRI findings were correlated to sonographic, endoscopic and biological results (sensitivity, specificity, Kappa test). RESULTS: The examinations were considered of satisfactory diagnostic quality in 93.3% of patients. Respiratory artifacts were present in one case. The entire GI tract could be identified on all sequences. Distention of the distal ileum was recorded as good to excellent in 89% of healthy subjects. Five MR examinations were considered abnormal with isolated ileal involvement in 2 cases, ileocolic involvement in 2 cases, and isolated colonic involvement in 1 case. The sensitivity and specificity of MR for the positive diagnosis of Crohn's disease were 100% and 83% respectively. Three extra-intestinal complications were detected: one case of ileo-ileal fistula, not identified on ultrasonography, an asymptomatic anal fistula and a symptomatic inflammatory stricture. CONCLUSION: MR enterography without enteroclysis is a well tolerated, effective non invasive method in the evaluation of known or suspected Crohn's disease. Because of the absence of ionizing radiation, MR enterography should become the gold standard in pediatric patients.

Administration, Oral↗

[Gastric trichobezoar in an adolescent presenting with anemia: ultrasound and MRI findings].

Trichobezoars are rare and composed of ingested hair or fibers. Diagnosis is usually made by plain radiographs and barium meal. The usefulness of computed tomography has been reported in the preoperative period. In the pediatric population the authors propose, based on a recent case report, a non invasive and non irradiating protocol using sonography and T1W and T2W MR imaging.

Adolescent↗

Prenatal presentation of cervical congenital neuroblastoma.

OBJECTIVE: To describe a cervical neuroblastoma revealed in the prenatal period. METHOD: We were recently confronted with a case of fetal solid neck mass suggestive of teratoma, which proved postnatally to be a neuroblastoma. Combining this case with a review of recent literature, we have evaluated the diagnostic tools required for increased precision in the prenatal diagnosis and perinatal management of fetal solid neck masses. RESULTS: Detailed ultrasound and MRI of fetal neck and liver, in conjunction with amniocentesis for measurement of homovanillic acid levels, should enable fetal medical practitioners to consider the diagnosis of fetal cervical neuroblastoma. CONCLUSION: Fetal cervical neuroblastoma is an extremely rare condition that has not been previously reported, but should be considered in the presentation of fetal solid neck masses.

Adult↗

Prenatal diagnosis of congenital genu recurvatum following amniocentesis complicated by leakage.

Congenital genu recurvatum (CGR) is an extremely rare condition observed at birth. It is associated with, among other malformations, genetic entities such as the Larsen syndrome. When CGR is isolated, orthopedic treatment will usually lead to a good functional prognosis. We report the first case of isolated CGR diagnosed prenatally and suspected to be a consequence of reduced amniotic fluid volume due to leakage following amniocentesis. The etiology and management options for CGR are briefly discussed.

Adult↗

[Rectal localization of Burkitt's lymphoma].

BACKGROUND: Rectal tumors are rare in childhood and among them malignant tumors are even less common. Only eight cases of primary rectal lymphomas were reported in children, with various presenting signs and histology. Burkitt's lymphomas are among these cases. CASE REPORT: A five-year-old child presented with hematochezia and unusual constipation. The rectal examination showed a voluminous intra rectal mass. Radiographic and pathologic examinations led to the diagnostic of Burkitt's lymphoma with medullary involvement. Complete remission was obtained after initial chemotherapy but a local relapse occurred and the child died eight months later. CONCLUSION: Hematochezia associated with unusual constipation impose a rectal examination. Early diagnosis of a rectal tumor may allow patients survival.

Antineoplastic Combined Chemotherapy Protocols↗

[Dual energy x-ray absorptiometry assessment of the bone mineral content and body composition during growth].

PURPOSE: To propose a simple method of analysis of whole body DXA scan results, for the bone mineral content of the entire skeleton (BMCt) and the arms and legs (BMCa and BMCl), as well as the analysis of the body composition in lean (Lt, La and Ll) and fat (Ft, Fa and Fl) in children and adolescents with normal growth. Materials and methods. A whole body DXA scan (Norland XR36(TM) DXA system) was performed in 90 control females and 76 control males aged from 2.3 y to 21.0 y, and from 2.3 y to 20.1 y, respectively. BMC values, as well as L and F values were correlated with the body weight (BW) of the measured subjects. BMCt, Lt and Ft values corrected for the body surface area (BS), and the ratio BMCl/BMCa, were also calculated. RESULTS: High linear correlations (r>0.9) were found between BMCt, Lt or Ft values and BW, as well as between BMC (a & l) and BMCt, L (a & l) and Lt, F (a & l) and Ft. The ratios BMCt/BS(1.5), Lt/BS(1.5), and Ft/BS(1.5) had constant values of 1.2+/-0.1, 17.5+/-1.9, and 8.7+/-2.0, respectively, in females, and 1.2+/-0.1, 18.9+/-1.3, and 7.3+/-1.4, in males. The ratio BMCl/BMCa had a constant value of 2.8+/-0.4 in both sexes. CONCLUSION: The reference values obtained by DXA for the bone mineral content, lean and fat masses of the entire body and limbs are of interest, in our experience, to follow-up the growth in patients with long-term treatments, and to quantify orthopedic disorders and treatments.

Absorptiometry, Photon↗

Bilateral internal carotid agenesis: value of CT angiography and correlation to embryogenesis.

Bilateral internal carotid artery agenesis is an uncommon disease, difficult to differentiate from bilateral carotid artery thrombosis. A few case reports have described the contribution of conventional angiogram to make the diagnosis and recognize the anatomic details of this rare malformation. Advantages of CT angiography as a non-invasive radiologic tool are discussed in this case report.

Adolescent↗

Pathophysiology, diagnosis and management of prenatal upper tract dilatation.

Upper tract dilatation presenting as renal pelvis dilatation with or without ureteric dilatation is one of the more common abnormalities detected on prenatal ultrasound scanning. The majority of cases are due to pelvi-ureteric and vesico-ureteric junction anomalies, and vesico-ureteric reflux. Prenatal diagnosis with ultrasound may be aided by biochemical analysis of fetal blood and urine. The natural history of prenatally detected abnormalities remains under review. Precise diagnosis, and therefore prognosis, may not be made until after birth.

Dilatation, Pathologic↗

Multicenter prospective study of children with sickle cell disease: radiographic and psychometric correlation.

After obtaining familial informed consent, between January 1996 and July 1997, 173 children (5 to 15 years old) with sickle cell disease were enrolled in a prospective multicenter study using blood screening, transcranial Doppler ultrasonography (n = 143), cerebral magnetic resonance imaging (n = 144), and neuropsychologic performance evaluation (n = 156) (Wechsler Intelligence tests WISC-III, WIPPSI-R), which were also performed in 76 sibling controls (5 to 15 years old). Among the 173 patients with sickle cell disease (155 homozygous for hemoglobin SS, 8 sickle cell beta0 thalassemia, 3 sickle cell beta+ thalassemia, 7 sickle cell hemoglobin C disease SC), 12 (6.9%) had a history of overt stroke, and the incidence of abnormal transcranial Doppler ultrasonography (defined as mean middle cerebral artery velocity > 200 cm/sec or absent) was 8.4% in the overall study population and 9.6% in patients with homozygous sickle cell anemia The silent stroke rate was 15%. Significantly impaired cognitive functioning was observed in sickle cell disease patients with a history of stroke (Performance IQ and Full Scale IQ), but also in patients with silent strokes (Similarities, Vocabulary, and Verbal Comprehension). However, infarcts on magnetic resonance imaging were not the only factors of cognitive deficit: Verbal IQ, Performance IQ, and Full Scale IQ were strongly impaired in patients with severe chronic anemia (hematocrit < or = 20%) and in those with thrombocytosis (platelets > 500 x 10(9)/L). Multivariate logistic regression analysis showed that abnormal magnetic resonance imaging (odds ratio [OR] = 2.76) (P = .047), hematocrit < or =20% (OR = 5.85) (P = .005), and platelets > 500 x 10(9)/L (OR = 3.99) (P = .004) were independent factors of cognitive deficiency (Full Scale IQ < 75) in sickle cell disease patients. The unfavorable effect of low hematocrit has already been suggested, but this is the first report concerning an effect of thrombocytosis and showing that silent stroke alone is not a factor of cognitive deficit when not associated with low hematocrit or thrombocytosis. The effect of hydroxyurea, which is known to increase hematocrit and decrease platelet count, on cognitive functioning of sickle cell patients should be evaluated prospectively.

Adolescent↗

Dual energy X-ray absorptiometry of the hand and wrist--a possible technique to assess skeletal maturation: methodology and data in normal youths.

Ninety five normal Caucasian subjects (51F, 44M) aged from 2 to 25 y were measured at the hand and wrist level with a small DXA system (pDEXA) in order to obtain the normal values of the bone mineral content (BMC), density (BMD) and projected area (A) of carpal (c) and metacarpal (m) bones. BMDc ranged from 0.065 +/- 0.007 g/cm2 to 0.365 +/- 0.035 g/cm2 in females and 0.425 +/- 0.040 g/cm2 in males. It presented a sharp change of increase rate at 15.5 and 17 y of age in girls and boys, respectively. Ac presented the same kind of evolution as BMDc, but had a larger value dispersion. The second metacarpal bone had the highest BMCm value in 85% of females and 90% of males. The sum of BMCmi or Ami values (i = 1-5) and the projected mean density of the 5 metacarpal bones was well correlated with BMCc, Ac and BMDc, respectively (r > 0.90). A volumetric mineral density, dmi, calculated for each of these bones, approximated to a cylinder, was correlated with age (r > 0.85).

Absorptiometry, Photon↗

Aneurysmal bone cysts: percutaneous embolization with an alcoholic solution of zein--series of 18 cases.

PURPOSE: To assess the efficacy of percutaneous embolization with an alcoholic solution of zein in the treatment of aneurysmal bone cysts. MATERIALS AND METHODS: Eighteen patients with aneurysmal bone cysts were treated percutaneously with alcoholic zein. The cysts were in the lower limb (n = 7), upper limb (n = 5), pelvis (n = 3), cervical spine (n = 2), and mandible (n = 1). All patients were symptomatic, three had previously undergone surgery. Percutaneous embolization was performed with fluoroscopic or computed tomographic guidance with the patient under general anesthesia. Clinical and imaging follow-up lasted 18 months to 4 years. RESULTS: Percutaneous embolization was performed in 16 cases. In two cases, cystograms showed marked venous drainage and thus embolization was not attempted. Six patients underwent repeat embolization. Complications consisted of a local transitory inflammatory reaction (n = 5), aseptic osteitis (n = 1), and a small pulmonary infarct without sequelae (n = 1). Relief of symptoms was achieved in all patients except one, who underwent surgery. At imaging, improvement was total in 13 cases (87%) and partial in two cases (13%). No recurrence was noted during follow-up. CONCLUSION: Percutaneous embolization of aneurysmal bone cysts with alcoholic zein should be considered a reliable alternative to surgery, especially in cases with a difficult surgical approach or cases of postsurgical recurrence.

Adolescent↗

MR cholangiography in neonates and infants: feasibility and preliminary applications.

OBJECTIVE: The objective of the study was to evaluate the accuracy of MR cholangiography in showing the biliary tree in neonates and infants and to show preliminary applications in a pediatric population. SUBJECTS AND METHODS: MR cholangiography was performed with T2-weighted turbo spin-echo sequences (8000/300 [TR/TE], six or eight excitations). The study included seven patients with suspected bile duct disorders and 10 control individuals. RESULTS: In the control group, the extrahepatic bile ducts (EHBDs) were revealed by MR cholangiography in all patients. In one of the five patients with neonatal cholestasis, biliary atresia was excluded by identifying the entire EHBD on MR cholangiograms. In the four other patients with neonatal cholestasis, biliary atresia was suspected because the EHBD could not be seen on MR cholangiography. The diagnosis was confirmed in three of these four patients, in whom the MR finding was associated with significant periportal thickening. In the remaining patient, in whom periportal thickening was not seen, sclerosing cholangitis was diagnosed on biopsy. In the sixth patient with neonatal cholestasis, who had received a liver transplant, MR cholangiography showed biliary dilatation above the biliary-digestive anastomosis. In the seventh patient, a neonate with a prenatal diagnosis of hepatic cyst, MR cholangiography showed a choledochal cyst. CONCLUSION: MR cholangiography may become an important diagnostic tool in the detection of biliary atresia, congenital choledochal dilatation, and biliary complications in hepatic transplantation for pediatric patients.

Bile Duct Diseases↗

Disappearing suprarenal masses in fetuses and infants.

This paper presents 12 infants (9 boys, 3 girls) in whom the diagnosis of a suprarenal mass (10 left, 2 bilateral) was made on antenatal sonography. All were otherwise healthy neonates who were born at term after a normal pregnancy, labor and delivery. The masses ranged from 1 to 3.5 cm in diameter on initial scans at gestational ages of 19-35 weeks. Eleven masses were hyperechoic and 4 of these contained small, well-defined cysts. The 12th was hypoechoic. Follow-up sonography showed complete disappearance of the mass antenatally in 1 case and postnatally by 4-6 months in 5 cases; there was marked diminution in the size of the mass by 2 months of age in 4 infants, by 4 months in 1 case and by 15 months in 1 case. Eleven were managed nonoperatively. Laparotomy (after disappearance of the mass) in the 12th case revealed only some fibrous tissue. The 11 echogenic masses resemble previously reported imaging findings in infants with histologically proven intra-abdominal sequestrated lung. Conservative management with careful sonographic follow-up should, therefore, be considered in otherwise healthy fetuses or neonates with these imaging findings. We are less certain what the hypoechoic mass represented.

Bronchopulmonary Sequestration↗