What is the appropriate aganglionic bowel length on contrast enema for attempting single stage transanal endorectal pull-through in Hirschsprung disease?

PurposeTo identify influence of different values of age and abnormal bowel length in HD patients selected for single stage TERPT which affects the technique of surgery.MethodsThis observational study was carried out for over 2.5 years. All children younger than 14 years old with clinical suspicion for HD, typical transitional zone (TZ) on contrast enema (CE) distal to splenic flexure, preoperative diagnosis approved by full thickness biopsy, no previous surgical history and no urgency were included. The distance between the anus and TZ was considered as aganglionic length on CE. Biopsy was taken from distal to proximal of resected bowel to reach circumferentially normal innervated bowel. Paired sample Student's t-test, Pearson correlation test, receiver operating characteristic (ROC) analysis were performed.ResultsForty-eight patients were enrolled in this study. Measured mean for aganglionic bowel length on CE and pathology were 33.5 ± 17.1 cm and 56.8 ± 33.5 cm, respectively (p < 0.01). Correlation coefficient (R) and coefficient of determination (R2) were 0.632 and 40%, respectively (p < 0.01). The difference between radiologic and pathologic measurements in females was higher than males (mean: 29.3 vs 21.9 cm) but was not statistically significant (p = 0.75). There was statistically significant difference between CE and pathologic results in the infants younger than 10 months (p = .004). Abnormal bowel length equal to 52 cm predicted requirement of laparoscopy assistance/laparotomy with 75% sensitivity and 85% specificity.ConclusionOur investigation showed it is safe to attempt for single stage TERPT when aganglionic length on CE is less than 52 cm and the child with HD is older than 10 months. Chance of requiring additional laparotomy or laparoscopy assistance is low in these patients.Type of studyStudy of diagnostic test.Level of evidenceLevel II.     

Rosai–Dorfman Disease: A Rare Clinicopathological Presentation and Review of the Literature

Rosai–Dorfman disease (RDD) is a rare and self-limiting disease process that presents most commonly in young patients as massive, painless, cervical lymphadenopathy. Extranodal involvement may also occur. Histopathologic evaluation is the main diagnostic modality. We report an unusual presentation of RDD with cervical lymphadenopathy and an incidentally discovered sinonasal mass, clinically worrisome for malignancy. We emphasize that a high index of clinical suspicion is critical for accurate diagnosis of RDD. Clinicians and pathologists should consider RDD in a differential diagnosis of cervical lymphadenopathy, especially in young patients.     

IMMUNOHISTOCHEMISTRY OF MEDULLOEPITHELIOMA AND NEURAL TUBE

Immunohistochemistry profiles of medulloepithelioma (from two 2 1-year-old girls who had 2 cerebral medulloepitheliomas and a 35-week postconceptional female infant with congenital posterior fossa tumor) and neural tube are compared. Microscopically, the tumors contained a medulloepitheliomatous component, manifested as tubular epithelial structures lined by pseudostratified columnar epithelium delineated by well-defined basement membranes. In all cases, glial and neuronal differentiation were noted to differing extents. The medulloepitheliomatous components did not exhibit glial fibrillary acidic protein, neuron-specific enolase, or S-100 protein reactivity. Neurofilament, cytokeratin, and epithelial membrane antigen were focally present in one case. Extensive nestin immunopositivity was confined to the basal cell layer of the epithelium, leaving the luminal surface unreactive or slightly reactive. These cells also displayed a reactivity to vimentin and to microtubuleassociated protein type 5 similar to that of cells of the primitive neural tube. The similarity between the immunohistochemical profile of medulloepithelioma and that of neural tube epithelium suggests a possible reexpression of that component of the genome responsible for neural tube growth and differentiation in medulloepithelioma.     

Efficacy and safety of rituximab therapy in patients with systemic sclerosis disease (SSc): systematic review and meta-analysis

Clinical Rheumatology - The clinical benefits of rituximab in systemic sclerosis (SSc) are still contentious. The present meta-analysis aimed to systematically assess rituximab’s safety and...     

Interactions between angiotensin-I converting enzyme insertion/deletion polymorphism and response of plasma lipids and coronary atherosclerosis to treatment with fluvastatin: the lipoprotein and coronary atherosclerosis study

OBJECTIVES: Our objectives were to determine whether angiotensin-1 converting enzyme (ACE) insertion/deletion (I/D) polymorphism was associated with the severity of coronary artery disease (CAD) and its progression/regression in response to fluvastatin therapy in the Lipoprotein and Coronary Atherosclerosis Study (LCAS) population. BACKGROUND: Genetic factors are involved in susceptibility to CAD. Angiotensin-1 converting enzyme I/D polymorphism, which accounts for half of the variance of plasma and tissue levels of ACE, has been implicated in susceptibility to CAD and myocardial infarction (MI). METHODS: Angiotensin-1 converting enzyme genotypes were determined by polymerase chain reaction (PCR). Fasting plasma lipids were measured and quantitative coronary angiograms were obtained at baseline and 2.5 years following randomization to fluvastatin or placebo. RESULTS: Ninety-one subjects had DD, 198 ID and 75 II genotypes. The mean blood pressure, minimum lumen diameter (MLD), number of coronary lesions and total occlusions were not significantly different at baseline or follow-up among the genotypes. There was a significant genotype-by-treatment interaction for total cholesterol (p = 0.018), low-density lipoprotein cholesterol (LDL-C) (p = 0.005) and apolipoprotein (apo) B (p = 0.045). In response to fluvastatin therapy, subjects with DD, compared with those with ID and II genotypes, had a greater reduction in total cholesterol (19% vs. 15% vs. 13%), LDL-C (31% vs. 25% vs. 21%) and apo B (23% vs. 15% vs. 12%). Definite progression was less (14%) and regression was more common (24%) in DD as compared with those with ID (32% and 17%) and II (33% and 3%) genotypes (p = 0.023). Changes in the mean MLD and lesion-specific MLD also followed the same trend. CONCLUSIONS: Angiotensin-1 converting enzyme I/D polymorphism is associated with the response of plasma lipids and coronary atherosclerosis to treatment with fluvastatin. Subjects with DD genotype had a greater reduction in LDL-C, a higher rate of regression and a lower rate of progression of CAD.     

Analysis of six heavy metals in Ortho mineral trioxide aggregate and ProRoot mineral trioxide aggregate by inductively coupled plasma–optical emission spectrometry

Ortho mineral trioxide aggregate (MTA) is a mineral aggregate newly developed for perforation repair, root end filling and pulp capping. The aim of this study was to investigate the levels of cadmium (Cd), copper (Cu), iron (Fe), manganese (Mn), nickel (Ni) and zinc (Zn) in Ortho MTA and ProRoot MTA. A total of 0.2 g of each MTA was digested using a mixture of hydrochloric and nitric acids and filtered. Six heavy metals in the resulting filtrates were analyzed by inductively coupled plasma–optical emission spectrometry (n = 5). The results were statistically analyzed using the Mann–Whitney U‐test. The concentrations of Cd, Cu, Fe, Mn, Ni and Zn in Ortho MTA were 0.10, 7.73, 49.51, 2.58, 0.82 and 10.09 p.p.m., respectively. The concentrations of Cd, Cu, Fe, Mn, Ni and Zn in ProRoot MTA were 0.16, 9.38, 1438.11, 74.51, 18.98 and 4.05 p.p.m., respectively. In conclusion, Ortho MTA had lower levels of Cd, Cu, Fe, Mn and Ni than ProRoot MTA.