A rare complication of embolization in juvenile nasopharyngeal angiofibroma

Juvenile nasopharyngeal angiofibroma (JNA) is one of the most common benign nasal cavity tumors of adolescence. It exhibits a strong tendency to bleed and despite being microscopically benign, its behavior is locally aggressive. Preoperative embolization is helpful to minimize intraoperative bleeding. However, embolization procedure may have some important thromboembolic complications such as central retinal artery occlusion. In this article, a young male with juvenile nasopharyngeal angiofibroma, who lost his vision in the left eye following embolization was presented and the possible therapeutic options in such a complication were discussed.     

An unusual case of monosomy 18p: minor malformations with speech delay

An unusual case of monosomy 18p with molecular cytogenetic characterization of 18;21 whole arm translocation who had mild speech delay and normal motor development is presented. A 3.5-year-old boy with complaints of speech delay, open mouth and drooling saliva was the child of a 33-year-old healthy mother and 35-year-old nonconsangineous father with unremarkable prenatal history. Beside delayed speech, hyperactive movements, flat nasal bridge, prominent ears, micrognathia, hypotonia, and overriding of left 3rd the on 2nd toe were present. Cytogenetic studies revealed de novo 45,XY del (18) t(18;21)-21 karyotype, which was confirmed by fluorescence in situ hybridization (FISH).     

Prognostic factors and treatment outcome in childhood Hodgkin disease

BACKGROUND: The goals of this study included: (1) Identification of factors prognostic for event-free survival (EFS) and overall survival (OS), and (2) Definition of risk groups for risk adapted therapy in children with Hodgkin disease (HD). PROCEDURE: From 1991 to 2003, 69 children with newly diagnosed, untreated biopsy-proven stage I-IV HD were treated with chemotherapy (CT) and low-dose involved field radiotherapy (LD-IFRT). The relationship of pretreatment factors to EFS and OS was analyzed by univariate and multivariate analysis. RESULTS: The 5-year EFS and OS for all patients were 90.77% and 96.22%, respectively with a median follow-up of 73 months (3-137 months). Male to female ratio was 3:1 and 21 children (32.3%) were less than 7 years of age. Mixed cellularity was the predominant histologic subtype (38.5%). Factors associated with inferior EFS by univariate analysis were extranodal disease, hemoglobin level <11 g/dl, number of involved lymph node regions and stage. By multivariate analysis only stage IV disease was significant. CONCLUSION: Our study confirms that excellent results are achievable with combined modality therapy in childhood HD. In order to use risk-adapted therapy in children with HD, clinical prognostic factors should be validated with large, multicentered prospective clinical studies.     

Deletion analysis and clinical correlations in patients with Xp21 linked muscular dystrophy

We carried out molecular deletion analysis on 142 patients with Duchenne/Becker muscular dystrophy which covered 25 exons of the dystrophin gene. We also evaluated the results by comparing with the clinical findings and examples in the literature. A deletion ratio of 63.7% was achieved. Exon 46 was the most frequently affected region. Interestingly we also observed four cases with muscle promoter (Mp) region deletions which have been rarely reported in the literature.     

Resistive index in febrile urinary tract infections: predictive value of renal outcome

In the absence of specific symptomatology in children, the early diagnosis of acute pyelonephritis is a challenge, particularly during infancy. In an attempt to differentiate acute pyelonephritis from lower urinary tract infection (UTI), we measured intrarenal resistive index (RI). We evaluated its ability to predict renal involvement as assessed by dimercaptosuccinic acid (DMSA) scintigraphy. In total 157 patients admitted to the pediatric department of the ili Etfal Hospital with clinical signs of febrile UTI were included in the study. The children were divided into groups according to their age at the time of ultrasonography (US). RI was measured from the renal arteries with Doppler US in the first 72 h in all 157 children. Renal involvement was assessed by ^99mTc-DMSA scintigraphy in the first 7 days after admission. The examination was repeated at least 6 months later if the first result was abnormal. All available patients with an abnormal scintigraphy underwent voiding cystourethrography 4–6 weeks after the acute infection. All patients with vesicoureteral reflux and scarred kidneys were excluded from the study. DMSA scintigraphy demonstrated abnormal changes in 114 of 157 children and was normal in the remaining 43 children. Of these 114 children, 104 underwent repeat scintigraphy, of whom 77 showed partially or totally reversible lesion(s). Of these 77 children, 17 children (22%) with vesicoureteral reflux were excluded. Thus, we compared the 43 children with lower UTI with the 60 children with definite acute pyelonephritis at admission. Kidneys with changes of acute pyelonephritis had a mean RI of 0.744±0.06 in infants, 0.745±0.03 in preschool children, and 0.733±0.09 in patients of school age with upper UTI. However, the mean RI was 0.703±0.06 in infants, 0.696±0.1 in preschool children, and 0.671±0.09 in school-aged patients with lower UTI. The mean RI values were significantly higher in patients with upper UTI (P<0.001). There was a highly significant correlation between RI values and the severity of the renal lesion as ranked by DMSA scintigraphy (P<0.001). When the cut-off RI value was 0.715, there was an 80% sensitivity and a 89% specificity for diagnosing upper UTI. Refluxing kidneys and scarred kidneys also had higher RI values. In conclusion, RI values were increased significantly in children with febrile UTI when renal parenchymal involvement (assessed by DMSA scintigraphy) was present. Our results also support the view that the children with high RI values are at a high risk of reflux, scarring, or both, which was frequently observed in febrile UTI. This might allow identification of patients at risk for severe renal lesions that require more aggressive therapy, investigation, and follow-up than those with lower UTI.An erratum to this article can be found at     

Bancroftian filariasis in Bagdogra town, district Darjeeling (West Bengal)

Filaria endemicity is probably gradually increasing over the years in Bagdogra town, district Darjeeling (West Bengal). Of 1511 night blood smears examined, 35 were found positive for Wuchereria bancrofti (mf rate 2.32%). The microfilaria (mf) rates for males and females were 2.84% and 1.79 % respectively. The age of these positives ranged from 5-45 years. The mf rate was highest (4.46%) in the age group of 20-29 years. All mf carriers were local residents of Bagdogra and Wuchereria bancrofti was only the infection encountered. Mean microfilaria density was 7.71 per 20 cmm. of blood, whereas Median microfilaria density (MFD50) was 21 per 20 cmm. of blood. Disease and filarial endemicity rates were 0.33% and 2.65% respectively. Ten man hour vector mosquito density ranged from 30 to 65. Out of 49 female Culex quinquefasciatus mosquitoes dissected for the presence of filaria infection, none was found positive for the infection. During 1976 survey in the same town, mf rate was 1.6% and disease rate was nil.     

Angiographic assessment of sequential and individual coronary artery bypass grafting

OBJECTIVE: In trying to answer the question about the controversial use of sequential grafts, we determined the mid-term angiographic outcome of patients in whom coronary artery bypass was performed with different types of vein grafts. METHODS: A total of 1034 coronary anastomoses on 724 saphenous vein grafts (SVGs) (apart from 497 left internal mammarian artery (LIMA) anastomoses) were assessed in 509 patients in an average of 55.4 +/- 17.6 months after coronary artery bypass grafting. RESULTS: The patency rates of sequential conduits were markedly higher than those of individual ones (86.6% vs 69.6%, p = 0.0001). Also, the anastomoses on the sequential conduits had better patency rates (80.6% vs 69.6%, p = 0.0001). This difference was even more pronounced in coronary arteries of poor quality/small (< 1.5 mm) diameter (68.9% vs 51.6%) for the sequential and individual grafts, respectively (p = 0.03). Also, the patency of the entire sequential conduit was lower when most distally located anastomosis was of poor runoff (45.2%). CONCLUSIONS: The patency of a sequential vein conduit is generally superior than that of an individual one, especially for poor runoff coronary vessels, provided that the most distally located anastomosis is performed on a good coronary artery in terms of quality and diameter. Using a minimal length of SVG is another advantage. However, failure of a single sequential conduit jeopardizes all of the anastomoses along that graft segment. Besides, being technically more demanding, technical expertise in performing a sequential anastomosis is probably among the important predictors of patency.     

Management of stiff hypertrophic nonunions by distraction osteogenesis: a report of 16 cases

OBJECTIVE: Hypertrophic nonunions can be managed successfully with distraction. Hypertrophic changes indicate that the tissue at the nonunion site has a biologic healing potential. The missing component is an appropriate mechanical environment to transform a hypertrophic nonunion into solid bone. DESIGN: At our institution, the records of 10 male and 6 female patients treated for stiff hypertrophic nonunion with the Ilizarov distraction method were retrospectively analyzed. The average age of the patients was 42.3 years (range 15-69 years). The nonunion time ranged from 8-48 months. All patients had at least 1 cm shortening, 3 patients had a deformity in one plane, and 13 had a deformity in two planes. The pathology was localized to the upper extremity in 5 patients, to the lower extremity in 11 patients, with a periarticular localization in 11 patients. An Ilizarov-type circular external fixator was applied in all patients to correct shortening, to correct deformity, and to achieve a solid union. RESULTS: All nonunions healed at an average follow-up of 38.1 months (range 24-95 months). The average time spent in the external fixator was 7.1 months (range 5-10 months). The average preoperative length discrepancy was 2.25 cm (range 1-8 cm), which was eliminated in all patients at the time of frame removal. The average coronal plane angulation of 19.7 degrees (range 15-37 degrees) and sagittal plane angulation of 20.8 degrees (range 5-45 degrees), together with translation in one patient, also were corrected to normal anatomic alignment. Complications included minor pin tract infections and hardware problems; recurrence of deformity was observed in one patient who refused to wear a protective brace after frame removal. CONCLUSIONS: Hypertrophic nonunions can be managed successfully with distraction. The Ilizarov device can address every aspect of a stiff hypertrophic nonunion, including shortening and deformity.