Biomedical subjects

D Zurakowski

Publications and source records attributed to D Zurakowski.

At least 91 records · Page 5Linked to original sources

PubMed1999

Infection with adeno-associated virus may protect against excitotoxicity.

Gene therapy has developed as a promising approach for therapy in a broad variety of conditions. Viral vectors have been developed that may replace a defective gene, prevent expression of a mutant gene, or deliver a protective gene and thereby delay cellular loss. Using adeno-associated virus containing green fluorescent protein (AAV-GFP) we were able to specifically transduce cells located in the inner retina and induce over-expression of GFP in adult rat retinae. The delivery and expression of GFP had no influence themselves on retinal ganglion cell survival. Administration of the reporter vector AAV-GFP provided retinal ganglion cells with slight but significant protection from intravitreal NMDA. This was a locally mediated phenomenon; greater protection was seen in regions with more transduced cells. Any evaluation of the efficacy of a putative viral vector should consider the possible protective or toxic effect of the native virus.

Animals↗

PubMed1999

bcl-2 gene therapy exacerbates excitotoxicity.

The protooncogene bcl-2 can block neuronal death from both naturally occurring apoptosis and exogenous insults. bcl-2 is therefore a promising candidate for the prevention of excitotoxic neuronal death. Using an adeno-associated viral vector, we delivered the bcl-2 gene to the ganglion cell layer of the rat eye. We hypothesized that infection with bcl-2 would protect ganglion cells against excitotoxic cell death. However, retinal infection with bcl-2 increased ganglion cell susceptibility to both axonal injury and intravitreal NMDA. Our study--intended to explore the possibility of bcl-2 transduction as an in vivo therapeutic approach--revealed a deleterious effect of bcl-2 transduction.

Animals↗

PubMed1999

Reticulocyte hemoglobin content to diagnose iron deficiency in children.

CONTEXT: Early identification of iron deficiency in children is essential to prevent the damaging long-term consequences of this disease. However, it is not clear which indices should be included in a diagnostic panel for iron deficiency and iron deficiency anemia in children. OBJECTIVE: To develop an effective approach for the diagnosis of iron deficiency and iron deficiency anemia in young children. DESIGN AND SETTING: Retrospective laboratory analysis, carried out over 7 weeks in 1996, using blood samples ordered by pediatricians and sent to a large metropolitan hospital for analysis. PATIENTS: A total of 210 children (mean [SD] age, 2.9 [2.0] years; 120 were male) who had a lead screening test (complete blood cell count and plasma lead level) ordered by a primary care pediatrician. MAIN OUTCOME MEASURES: Levels of hemoglobin (Hb), iron, transferrin, transferrin saturation (Tfsat), ferritin, and circulating transferrin receptor and reticulocyte Hb content (CHr) among patients with and without iron deficiency, defined as Tfsat of less than 20%, and iron deficiency anemia, defined as Tfsat of less than 20% and Hb level of less than 110 g/L. RESULTS: Of the 210 subjects, 43 (20.5%) were iron deficient; 24 of these had iron deficiency anemia. Reticulocyte Hb content and Hb levels were the only significant predictors of iron deficiency (likelihood ratio test [LRT] = 15.96; P<.001 for CHr, and LRT = 6.59; P = .01 for Hb), and CHr was the only significant multivariate predictor of iron deficiency anemia (LRT = 30.43; P<.001). Plasma ferritin level had no predictive value (P = .97). Subjects with CHr of less than 26 pg (optimal cutoff value based on sensitivity/specificity analysis) had lower Hb level, mean corpuscular volume, mean corpuscular Hb level, serum iron level, and Tfsat, and increased red blood cell distribution width vs those with CHr of 26 pg or more (P<.001 for all). CONCLUSIONS: Reticulocyte Hb content level was the strongest predictor of iron deficiency and iron deficiency anemia in children. It holds promise as an alternative to biochemical iron studies in diagnosis.

Anemia, Iron-Deficiency↗

PubMed1999

Cation channel control of neurite morphology.

The development of neuronal polarity and morphology is essential for a functioning nervous system. The present study was undertaken to explore whether blockade of specific channels alter neuronal morphology. Retinal ganglion cells were cultured in the presence of antagonists to NMDA, AMPA/kainate, L-, N-, P-, and Q-type voltage-dependent calcium channels (VDCCs). Five parameters were measured under these conditions: the number of neurites at the cell body, total neurite length, the length of the longest neurite, the number of branch points per neurite, and the diameter of the cell soma. Antagonists to NMDA and L-type VDCCs reduce the number of neurites at the cell body; antagonists to P- and Q-type VDCCs increase the number of neurites. Antagonists to the N-type VDCCs increase total neurite outgrowth, while antagonists to the NMDA and P-type channels reduce total neurite length. Antagonists to the NMDA and L-type channels increase the length of a single neurite, while decreasing the number of branch points; antagonists to the P- and Q-type VDCCs do essentially the opposite-increase the number of neurites, while decreasing the length of each. Blockade of one or more cation channels in developing retinal ganglion cells significantly perturbs neurite morphology. This study may help elucidate part of the role that cation channel signaling plays in neuritic development.

2-Amino-5-phosphonovalerate↗

PubMed1999

Lung growth after reduced size transplantation in a sheep model.

BACKGROUND: The growth of mature allografts is a critical issue in pediatric lung transplantation. This study explores the architectural changes of mature sheep lung when submitted to two different compensatory growth forces: either transplantation into a neonatal host or expansion in an otherwise empty adult hemithorax. METHODS: Right upper lobes (RUL) (mean+/-SEM, 66.7+/-1.9 kg) from 4- to 5-year old (adult sheep) were transplanted into newborn (n=6) lambs (5.4+/-0.3 kg, 5+/-2 days old) that were then allowed to survive for 45 days. Changes in pulmonary volume and architecture were measured before and after transplantation. Allografts were compared with both normal adult RUL (n=10) and adult (65.8+/-2.2 kg and 4 to 5 year old) RUL that remained in situ for 45 days after resection of the corresponding middle and lower lobes (n=6). Statistical differences were analyzed using two-sample and paired t tests. RESULTS: In adult animals, RUL remaining in the otherwise empty hemithorax compensated by an 85% increase in volume (251.5+/-18.7 ml vs. 466+/-32.8 ml) (P<0.0001). Concomitant increases in total internal alveolar surface area (48%) and alveolar size were prominent. The number of alveoli per volume decreased proportionately to the increases in volume (P<0.0001). There was no significant change in the calculated number of alveoli (345.6+/-40.5 x 10(6)) compared with the normal adult RUL (402.4+/-40.7x10(6)) (P=0.37). Transplant recipients received a reduced-size normal adult RUL (49%) in volume (125.3+/-21.5 ml). Allografts 45 days after transplantation showed a 73% increase in volume (216.4+/-21.3 ml) (P<0.0001) with a parallel (83%) increase in total internal alveolar surface area (P=0.008). The number of alveoli per volume remained constant (P=0.21) despite the increase in volume. There was therefore a significant increase in the calculated number of alveoli from before transplantation (172.5+/-35.9x 106) compared with that observed 45 days after transplantation (389.7+/-77.7x10(6)) (P=0.012). CONCLUSIONS: We conclude that mature sheep RUL parenchyma compensates with dilation of the respiratory structures in the adult animal, whereas there is alveolar multiplication when transplanted into newborn recipients.

Analysis of Variance↗

PubMed1999

Cervicomedullary astrocytomas of childhood: clinical and imaging follow-up.

UNLABELLED: BACKGROUND. Cervicomedullary astrocytomas are a unique subset of brainstem tumors in children because they have a good prognosis when compared to the pontine subset of brainstem gliomas. Objective. To review the clinical and imaging findings in a series of children with cervicomedullary astrocytomas as to diagnosis and management. MATERIALS AND METHODS: A retrospective review of eleven children (six females, five males, age range: 10 days-18 years; mean = 7 years) with cervicomedullary tumors was done including the clinical presentation, imaging studies (MR: eleven, CT and MR: four), surgical findings, pathological results, and follow-up clinical and imaging findings (range: 0.2-11 years; mean = 5.2 years). RESULTS: Symptoms and signs were delayed and protracted, often occurring over months to years (mean = 2.3 years, range 0.5-7 years). The tumors expanded the dorsal medulla and involved the upper cervical spinal cord (mean maximum tumor diameter = 4.4 cm). Only three patients had hydrocephalus. In three of four cases the tumor was not seen on CT. On MR, the majority of the tumors were T1 hypointense and T2 hyperintense. Treatment consisted of surgery only in six patients, surgery and radiation therapy in four, and surgery, chemotherapy, and radiation in one. There was recurrent local disease in four patients and on follow-up metastatic disease in the brain in one. On follow-up the majority of the patients are alive and stable (mean = 5.2 years, range 0.2-11 years). There has been one death. The majority of tumors were pilocytic astrocytomas. CONCLUSION: Cervicomedullary tumors are a unique subset of brainstem gliomas in childhood that present with a long duration of symptoms and a greater long-term survival than pontine gliomas.

Adolescent↗

PubMed1999

Sedation for pediatric radiological procedures: analysis of potential causes of sedation failure and paradoxical reactions.

BACKGROUND: Sedation for diagnostic imaging and interventional radiologic procedures in pediatrics has greatly increased over the past decade. With appropriate patient selection and monitoring, serious adverse effects are infrequent, but failure to sedate and paradoxical reactions do occur. OBJECTIVE: The purpose of this study was to determine, among patients undergoing sedation for radiologic procedures, the incidence of sedation failure and paradoxical reaction to pentobarbital and to identify potentially correctable causes. MATERIALS AND METHODS: Records of 1665 patients who were sedated in the radiology department from 1 November 1997 to 1 July 1998 were reviewed. Patients failing sedation or experiencing paradoxical reaction were compared with respect to sex, age group, diagnosis, scan type, time of day, NPO status, use of IV contrast and type of sedation agent using the Fisher exact test, Pearson chi-square, analysis of variance (ANOVA), the Student t-test, and logistic regression. RESULTS: Data analysis revealed a sedation failure rate of 1 % and paradoxical reaction rate of 1.2 %. Stepwise multiple logistic regression revealed that the only significant independent multivariate predictor of failure was the need for the administration of a combination of pentobarbital, fentanyl, and midazolam IV. CONCLUSION: The low rate of sedation failure and paradoxical reactions to pentobarbital was near optimal and probably cannot be improved with the currently available sedatives.

Child↗

PubMed1999

Forty-one years of surgical experience with congenital supravalvular aortic stenosis.

OBJECTIVE: Several techniques for symmetric reconstruction of the aortic root in congenital supravalvular aortic stenosis have been developed, but it remains unclear whether these prove superior to patch enlargement of the noncoronary sinus alone. We reviewed our experience with surgical treatment of supravalvular aortic stenosis and investigated the impact of the surgical technique on long-term results. METHODS AND RESULTS: Seventy-five patients underwent operations to treat congenital supravalvular aortic stenosis at our institution between 1957 and 1998. Surgical procedures included patch enlargement of the noncoronary sinus only (n = 34), inverted bifurcated patch plasty (n = 35), and 3-sinus reconstruction of the aortic root (n = 6). There were 7 early deaths. Among those who survived the operation, 100% were alive at 5 years, 96% were alive at 10 years, and 77% were alive at 20 years. According to time-related analysis diffuse stenosis of the ascending aorta proved a risk factor for both survival and reoperation (P <.01 for each). Patients with multiple-sinus reconstructions of the aortic root accounted for only 2 of the 14 reoperations and none of the late deaths (both P <.001). Residual gradients were lower after multiple-sinus reconstruction of the aortic root (median 10 mm Hg vs 20 mm Hg for patch enlargement of the noncoronary sinus only, P =. 008), as was the prevalence of moderate aortic regurgitation at follow-up (3% vs 22%, P =.05). CONCLUSIONS: Results of operations for supravalvular aortic stenosis improved greatly after the introduction of more symmetric reconstructions of the aortic root. Multiple-sinus reconstructions (inverted bifurcated patch plasty and 3-sinus reconstruction) resulted in superior hemodynamics and were associated with reductions in both mortality rate and need for reoperation.

Aortic Valve Stenosis↗

PubMed1999

Improved results with selective management in pulmonary atresia with intact ventricular septum.

OBJECTIVE: Late outcome of neonatal pulmonary atresia with intact ventricular septum remains poor in most reported series. We have followed a selective approach toward either single ventricle repair versus complete or partial biventricular repair based on the presence of right ventricle-dependent coronary circulation and growth of the right ventricle. METHODS: A retrospective chart review was conducted of 47 patients who underwent surgery between January 1991 and September 1998. RESULTS: Sixteen (34%) patients had a right ventricle-dependent coronary circulation, with a tricuspid valve Z-score of -3.0 +/- 0.66 versus -2.0 +/- 0.95 (P =.002) for those without a right ventricle-dependent coronary circulation. A systemic-pulmonary artery shunt only was performed in all patients with a right ventricle-dependent coronary circulation, with 1 death. Fourteen of 16 patients with a right ventricle-dependent coronary circulation underwent a bidirectional Glenn shunt at a median of 9 months after their first operation, 9 of whom have had a Fontan procedure (no deaths). In the 31 (66%) patients without a right ventricle-dependent coronary circulation, 6 patients underwent only a systemic-pulmonary artery shunt, 23 had a shunt and right ventricular decompression, and 2 had only a transannular patch. In this group, 10 patients received a 2-ventricle repair, 6 a 1. 5-ventricle repair, and 8 patients had a Fontan procedure. There was 1 early death and the overall survival was 98% at 1 year, 5 years, and 7 years. CONCLUSIONS: If patients are stratified well, excellent survival can be achieved in the treatment of pulmonary atresia with intact ventricular septum. This result may be at the price of achieving a 1-ventricle as opposed to a 2-ventricle repair.

Actuarial Analysis↗

PubMed1999

Plasma levels of vascular endothelial growth factor are increased in patients with metastatic prostate cancer.

OBJECTIVES: Vascular endothelial growth factor (VEGF) is a cytokine that plays an important role in tumor angiogenesis. VEGF is overexpressed in many human cancers, including prostate cancer, but circulating levels of VEGF in patients with prostate cancer have not been reported. In this study, we analyzed plasma concentrations of VEGF in a cohort of patients with prostate cancer and compared them with a normal population. METHODS: Twenty-six healthy, cancer-free individuals and 80 patients with prostate cancer (54 patients with localized prostate cancer and 26 patients with metastatic prostate cancer [bone or lymph node positive]) were analyzed in this study. Blood was drawn in the same fashion from all individuals and deposited in tubes containing ethylenediaminetetraacetic acid as anticoagulant. Plasma was extracted and VEGF concentrations were determined using a quantitative sandwich enzyme immunoassay technique. RESULTS: Median plasma VEGF was 28.5 pg/mL (interquartile range 19.3 to 57.0) in patients with metastases; 7.0 pg/mL (interquartile range 0 to 26.5) in patients with localized disease, and 0 pg/mL (interquartile range 0 to 24) in controls. These differences were statistically significant (P <0.001). When compared group by group, the metastatic group had significantly higher plasma VEGF than the localized disease group and the control group (P = 0.003 and P <0.001, respectively). There was a tendency for plasma VEGF to be higher in the localized disease group than in the control group, a trend that almost reached statistical significance (P = 0.038). Using a cutoff of 18 pg/mL, the sensitivity and specificity of the test in differentiating between patients with and without metastatic disease was 81% and 71%, respectively. The odds of metastatic disease were almost 10 times greater for patients with VEGF values greater than 18 pg/mL than for those with values less than 18 pg/mL. There was no correlation between age and plasma VEGF values or between plasma VEGF and serum prostate-specific antigen (PSA). However, patients with serum PSA greater than 20 ng/mL had significantly higher plasma VEGF values than patients with serum PSA less than 20 ng/mL (P <0.001). No direct relation was found between Gleason sum and plasma VEGF, although VEGF levels were higher in patients with Gleason sums of 8 to 10 than in patients with lower Gleason sums. CONCLUSIONS: Our study indicates that patients with metastatic prostate cancer have higher plasma VEGF levels than patients with localized disease or healthy controls. A larger prospective study is needed to confirm the predictive utility of VEGF.

Aged↗

PubMed1999

Relationship between birthweight and blood pressure in childhood.

Studies have shown an inverse relationship between birthweight and blood pressure in later life. The objective of this study is to analyze the relationship between birthweight and blood pressure in childhood in a North American-based population. Data on 2,958 births with follow-up at 7 years of age from the Providence, RI, cohort of the Collaborative Perinatal Project of the National Institute of Neurological Diseases and Stroke were retrospectively analyzed using univariate and multivariate analytic methods. Bivariate analysis of the total cohort showed a direct relationship between follow-up weight at age 7 years and birthweight (r = 0.24; P < 0.001) and follow-up weight with systolic (SBP) and diastolic blood pressure (DBP; r = 0.33; P < 0.001 and r = 0.22; P < 0.001, respectively). On multivariate analysis, follow-up weight and height were the strongest predictors of SBP and DBP. There was also a significant inverse relationship between birthweight and SBP. A cohort of term infants (n = 2,561) was subdivided into birthweight-for-gestational-age groupings to further evaluate the effects of birthweight on blood pressure. Small-for-gestational-age (SGA) infants were markedly smaller at age 7 years than those large-for-gestational-age (LGA; 21 +/- 4 kg v 26 +/- 4 kg; P < 0.01). Despite the direct association between follow-up weight and blood pressure, the mean blood pressure did not differ between SGA (103/58 mm Hg) and LGA patients (103/59 mm Hg). To assess whether birthweight was an independent predictor of blood pressure, blood pressures were predicted using linear regression equations. For every 1-kg decrease in birthweight in term infants, SBP at 7 years increased by 1.3 mm Hg and DBP by 0.6 mm Hg. In conclusion, controlling for weight and height in term infants at 7 years of age has an inverse linear effect on blood pressure. This suggests that birthweight in relation to gestation may be a contributor to the multifactorial cause of essential hypertension.

Birth Weight↗

PubMed1999

Elevated serum vascular endothelial growth factor in children and young adults with Crohn's disease.

Vascular endothelial growth factor (VEGF) is a cytokine released by fibroblasts, epithelial cells, and leukocytes that potentiates vascular permeability and growth of new capillaries. Because of these multiple effects, VEGF has been postulated to play a role in the pathogenesis of autoimmune disease, as well as in wound healing. We hypothesized that VEGF was potentially important in mediating the vascular permeability and angiogenesis seen in Crohn's disease, and therefore that VEGF would be increased in the serum of children with Crohn's disease. Serum was obtained from 73 children and young adults with Crohn's disease, 47 with ulcerative colitis, and 29 controls. VEGF levels were measured by enzyme-linked immunosorbent assay. Mean VEGF levels were significantly higher in patients with Crohn's disease (436.4 +/- 37.2 pg/ml) than in ulcerative colitis (306 +/- 41.1 pg/ml) or control (167.8 +/- 29.6 pg/ml) patients. Serum VEGF also correlated significantly with disease activity, being elevated in patients with moderate/severe Crohn's disease and ulcerative colitis. We conclude that serum VEGF is released by inflamed tissues in children with Crohn's disease. This multifunctional cytokine could promote inflammation by increasing vascular permeability or promote wound healing by mediating capillary growth.

Adolescent↗

PubMed1999

The effect of hematocrit on cerebral blood flow velocity in neonates and infants undergoing deep hypothermic cardiopulmonary bypass.

UNLABELLED: Varying degrees of hemodilution are used during deep hypothermic cardiopulmonary bypass. However, the optimal hematocrit (Hct) level to ensure adequate oxygen delivery without impairing microcirculatory flow is not known. In this prospective, randomized study, cerebral blood flow velocity in the middle cerebral artery was measured using transcranial Doppler sonography in 35 neonates and infants undergoing surgery with deep hypothermic cardiopulmonary bypass. Patients were randomized to low Hct (aiming for 20%) or high Hct (aiming for 30%) during cooling on cardiopulmonary bypass (CPB). Systolic (V(s)), mean (Vm), and diastolic (Vd) cerebral blood flow velocity, as well as pulsatility index (PI = [V(s) - Vd]/Vm) and resistance index (RI = [V(s) - Vd]/V(s)) were recorded at six time points: postinduction, at cannulation, after 10 min cooling on CPB, rewarmed to 35 degrees C on CPB, immediately off CPB, and at skin closure. Vm was significantly lower in the high Hct group compared with that in the low Hct group during cooling (P < 0.01). Postinduction, the high Hct group demonstrated significantly lower Vd immediately off CPB (P < 0.01) and significantly lower Vm and V(s) at skin closure (P < 0.001). We conclude that there is an inverse relation between hematocrit and cerebral blood flow velocity during deep hypothermic cardiopulmonary bypass in neonates and infants. IMPLICATIONS: There is an inverse relation between hematocrit and cerebral blood flow velocity during deep hypothermic cardiopulmonary bypass in neonates and infants. Further studies correlating Hct and cerebral blood flow velocity with cerebral metabolic rate and neurologic outcome are necessary to determine the optimal Hct during deep hypothermic cardiopulmonary bypass.

Blood Flow Velocity↗

PubMed1999

Anterior cruciate ligament reconstructive surgery in adolescent soccer and basketball players.

OBJECTIVE: To evaluate trends in the frequency of anterior cruciate ligament (ACL) tears among adolescent soccer and basketball players resulting in reconstructive surgery, and to assess differences between female and male adolescent athletes. DESIGN: A retrospective review of ACL reconstructive surgeries performed from 1992 through 1997 and annual statewide participation rates obtained from the National Federation of State High School Associations. SETTING: Children's Hospital, Boston, Massachusetts, U.S.A. PATIENTS: Adolescent athletes 13 to 19 years of age (78 girls, 31 boys) admitted for ACL reconstruction due to soccer or basketball injuries. MAIN OUTCOME MEASURES: Number of ACL reconstructive surgeries and number of Massachusetts participants in high school soccer and basketball. RESULTS: A total of 69 soccer (49 girls, 20 males) and 40 basketball (29 girls, 11 males) players underwent ACL reconstruction. A greater proportion of girls underwent ACL surgery in both sports. The frequency increased over time for both sexes in both sports. Soccer-related surgeries increased at a faster rate among girls than boys. Basketball-related surgeries increased at a similar rate for both sexes, although in any given year more surgeries were performed on girls. CONCLUSION: The frequency of ACL surgery at our institution has increased among high school soccer and basketball players in a manner consistent with trends in sports participation. In our study, ACL surgery was more common among girls than boys.

Adolescent↗

PubMed1999

Pulmonary outcome in cystic fibrosis is influenced primarily by mucoid Pseudomonas aeruginosa infection and immune status and only modestly by genotype.

Whether allelic variants of the cystic fibrosis (CF) transmembrane conductance regulator (CFTR) independently contribute to pulmonary outcome in CF patients has not been resolved. We used both cross-sectional and mixed-model longitudinal analyses of data from CF patients that were at least 12 years old to determine the influence on pulmonary function (percent predicted forced expiratory volume [FEV(1)]) of the CFTR gene genotype, gender, mucoid Pseudomonas aeruginosa (MPA) infection status, presence of total opsonic antibody to MPA, and, separately, the opsonic antibody activity specific to the mucoid exopolysaccharide (MEP) surface antigen. Two different factors were independently associated with the lack of MPA infection: a high level of MEP-specific opsonic activity (MSOA), implicating an immunologically based mechanism of resistance to infection, and a lack of any type of opsonic antibody to MPA, indicative of no significant exposure or infection. This latter phenotype was found in a subset of CF patients who carried at least one uncommon CFTR gene allele suggestive of a genetic basis for resistance to infection in this group of older CF patients. For CF patients in whom both CFTR gene alleles were identified by screening for the 12 most common variants (75% of alleles), cross-sectional analysis showed that MPA infection was best correlated with lower percent predicted FEV(1), while genotype (two versus one DeltaF508 CFTR gene allele) and a low level of MSOA were associated with increased risk of infection. A mixed-model analysis of longitudinal spirometric measurements that considered multiple risk factors to derive regression equations was used to determine which clinical parameters had the greatest effect on the annual rate of decline in percent predicted FEV(1). This analysis showed that the CFTR gene genotype only modestly modified the constant (y intercept) of the derived equations, while gender and MPA infection status had the largest effects on annual rates of decline in percent predicted FEV(1). These results indicate that the CFTR genotype is usually not a primary determinant of pulmonary function in most CF patients, but gender and MPA infection status are. Infection status is potentially influenced by both immunologic (a high level of MSOA) and genetic factors, such as carriage of a CFTR gene allele that leads to a diagnosis of CF but still confers resistance to infection that is comparable to that of the wild-type CFTR gene.

Adolescent↗

PubMed1999

Occult lumbosacral dysraphism in children and young adults: diagnostic performance of fast screening and conventional MR imaging.

PURPOSE: To compare fast screening and conventional magnetic resonance (MR) imaging for the detection of occult dysraphic myelodysplasias in children and young adults. MATERIALS AND METHODS: A retrospective case-control study included 101 patients (mean age, 4.9 years; range, 1 day to 26 years) suspected of having occult lumbosacral dysraphism. Sixty case patients had myelodysplastic lesions (19 filar lipoma, 14 syringomyelia, 10 intradural lipoma, eight dermal sinus, five diastematomyelia, five lipomyelomeningocele, two caudal regression syndrome); 41 control patients had no dysraphic lesions; 17 patients had associated renal anomalies. Two neuroradiologists reviewed MR images from conventional and fast screening protocols. Diagnostic performance parameters included sensitivity, specificity, and area under the receiver operating characteristic curve (Az value). RESULTS: The sensitivity of conventional and fast screening MR studies was 97.1% and 98.5%, respectively, the specificity was 90.9% and 84.8%, respectively. The Az value was 0.973 for the fast screening and 0.976 for the conventional MR studies (P = .83). Interobserver agreement was very good for fast screening images (kappa = 0.68) and excellent for conventional images (kappa = 0.75). For renal anomalies, the Az value was 0.786 and 0.853 for fast screening and conventional MR imaging, respectively (P = .28). CONCLUSION: Conventional three-plane lumbosacral MR imaging in children and young adults suspected of having occult dysraphism provides better diagnostic information than does fast screening two-plane MR imaging because of its higher specificity and interobserver agreement.

Adolescent↗

PubMed1999

Legg-Calvé-Perthes disease: MR imaging evaluation during manual positioning of the hip--comparison with conventional arthrography.

PURPOSE: To evaluate the use of magnetic resonance (MR) imaging during manual positioning of the hip, or multipositional MR imaging, in an open-magnet configuration to study femoral head containment, articular congruency, and femoral head deformity in Legg-Calvé-Perthes disease. MATERIALS AND METHODS: In 12 children with advanced Legg-Calvé-Perthes disease, multipositional MR imaging and conventional arthrography were compared in the assessment of containment, femoroacetabular congruency, and femoral head deformity. Images of the hips in several positions were compared subjectively and objectively. RESULTS: MR imging correlated well with arthrography for overall subjective assessment of severity of disease (r = 0.71, P = .01), with good interobserver agreement (kappa = 0.65, P < .001). MR images demonstrated all cases of hinge abduction shown arthrographically. However, MR imaging failed to depict one case of femoral head flattening. MR imaging correlated well with arthrography in the objective evaluation of joint fluid and lateral subluxation (r = 0.80, P < .01). MR imaging correlated poorly with arthrography in the measurement of sphericity of the femoral head. CONCLUSION: Multipositional MR imaging with an open-magnet configuration was comparable to arthrography for demonstration of femoral head containment and congruency of the articular surfaces of the hip. In the evaluation of deformity, it performed less well.

Arthrography↗

PubMed1999

Mannose-binding protein B allele confers protection against tuberculous meningitis.

Inhalation is the principal mode of entry for Mycobacterium tuberculosis in humans. Primary infection is usually restricted to the lungs and contiguous lymph nodes. In a subset of infected individuals, predominantly children, the infection is spread hematogenously to the meninges. The host factors that influence the development of tuberculous meningitis have not been well elucidated. The mannose-binding protein (MBP), a serum protein, is considered as an "ante-antibody." MBP has been shown to bind mycobacteria and acts as an opsonin in vitro. Although MBP plays a role in first-line host defense, it may under certain circumstances be deleterious to the host. In tuberculosis (TB), MBP may assist the spread of this intracellular pathogen. Therefore, we hypothesized that MBP genotypes that result in a phenotype of low MBP levels might be protective. We studied a well-defined South African population in which TB has reached epidemic levels. We found that the MBP B allele (G54D), which disrupts the collagen region of the protein and results in low MBP levels, was found in 22 of 79 (28%) of the TB-negative controls from the same community, compared with 12 of 91 (13%) of the patients with pulmonary TB (p < 0.017), and 5 of 64 (8%) of patients with tuberculous meningitis (p < 0.002). In addition, we found significantly lower serum MBP concentrations in TB-negative controls compared with postacute phase, fully recovered TB patients (p < 0.004). These findings suggest that the MBP B allele affords protection against tuberculous meningitis.

Adult↗