Migratory foreign body of neck in a battered baby: A case report

Only a small number of ingested foreign bodies perforate the esophagus and even smaller fraction migrate extramucosally with no symptoms. Both of these events are even rarer after marble ingestion. We report here a case of battered baby with homicidal marble ingestion which perforated the esophagus and migrated into the soft tissue of neck. It was diagnosed after 21 days asymptomatic period. The foreign body migrated into the tracheo-esophageal groove, lying deep to strap muscles, which was removed by neck exploration.     

Malignant peripheral nerve sheath tumors of the head and neck: A clinicopathological study

Twenty-seven cases of malignant peripheral nerve sheath tumors involving the head and neck region over a period of 7 years were reviewed. They were graded from 1–3 based on necrosis, mitosis, cellularity, and pleomor-phism. Mean age of occurrence was 42 years, with a range of 12–70 years. Male preponderance was noted (M:F = 3.5:1). The most common site of involvement was the neck (44.6%). The main presenting symptom was an enlarging mass. The nerve of origin could be identified in 33% of patients. Treatment consisted of wide excision. The 5-year observed survival was 33%. Fifty-two percent of patients developed local recurrence of disease. Fifteen percent of patients died due to advanced local disease within 18 months of treatment. Distant metastasis was seen in 18.5% of patients. Lymph node metastasis was not seen. At the end of 5 years 15% of patients remained disease free. Large tumor size (>5 cm) adversely affected the prognosis (P = < 0.1). No significant correlation was noted between the grade of tumor and survival. © Wiley-Liss, Inc.     

Synthesis of two-component injectable polyurethanes for bone tissue engineering

The advent of injectable polymer technologies has increased the prospect of developing novel, minimally invasive arthroscopic techniques to treat a wide variety of ailments. In this study, we have synthesised and evaluated a novel polyurethane-based injectable, in situ curable, polymer platform to determine its potential uses as a tissue engineered implant. Films of the polymers were prepared by reacting two pentaerythritol-based prepolymers, and characterised for mechanical and surface properties, and cytocompatibility. This polymer platform displayed mechanical strength and elasticity superior to many injectable bone cements and grafts. Cytotoxicity tests using primary human osteoblasts, revealed positive cell viability and increased proliferation over a period of 7 days in culture. This favourable cell environment was attributed to the hydrophilic nature of the films, as assessed by dynamic contact angle (DCA) analysis of the sample surfaces. The incorporation of beta-TCP was shown to improve mechanical properties, surface wettability, and cell viability and proliferation, compared to the other sample types. SEM/EDX analysis of these surfaces also revealed physicochemical surface heterogeneity in the presence of beta-TCP. Based on preliminary mechanical analysis and cytotoxicity results, these injectable polymers may have a number or potential orthopaedic applications; ranging from bone glues to scaffolds for bone regeneration.     

Childhood Sex-Typed Behavior and Gender Change in Individuals with 46,XY and 46,XX Disorders of Sex Development: An Iranian Multicenter Study

Disorders of sex development (DSD) are congenital conditions in which the typical genetic and hormonal profiles are affected and thereby the usual process of sexual differentiation. Most of these studies, however, have been conducted in Western countries. In the present study, preschool sex-typed activities of Iranian individuals with DSD and their age-matched non-affected male and female relatives were assessed using the Pre-School Activities Inventory (PSAI) modified for retrospective self-report. A total of 192 individuals participated in our study, including 33 46,XX individuals with congenital adrenal hyperplasia (CAH; M age?=?10.36, SD?=?5.52), 15 46,XY individuals with complete androgen insensitivity syndrome (CAIS; M age?=?19.8, SD?=?7.14), and 16 46,XY individuals with 5-alpha reductase deficiency type-2 (5α-RD-2; M age?=?17.31, SD?=?7.28), as well as one age-matched non-affected male and female relative for each patient. With regard to PSAI scores, male-identifying participants with 5α-RD-2 and male controls reported similar levels of male-typical childhood play. Female-identifying participants with 5α-RD-2 and CAH showed comparable scores: significantly less masculine and more feminine than male controls, but significantly more masculine and less feminine than females with CAIS and female controls. These findings support the role of androgens in the development of sex-typical childhood play behavior, with those being exposed to higher levels of fetal functional androgens expressing more masculine behavior at preschool ages.     

Mental Health and Disorders of Sex Development/Intersex Conditions in Iranian Culture: Congenital Adrenal Hyperplasia, 5-α Reductase Deficiency-Type 2, and Complete Androgen Insensitivity Syndrome

Sixty-one patients (22 patients with congenital adrenal hyperplasia [CAH] with a mean age of 14.86 years [range, 5–23], 20 patients with 5-α reductase deficiency type 2 [5α-RD-2] with a mean age of 19.5 years [range, 5–29], and 19 patients with complete androgen insensitivity syndrome [CAIS] with a mean age of 18.26 years [range, 5–28]) were evaluated using the Kiddie Schedule for Affective Disorders and Schizophrenia, the Structured Clinical Interview for DSM-IV Axis I, Axis II, and the Global Assessment Functioning Scale. All participants were female-assigned at birth. Ten patients (16.4%) transitioned to the male gender. Overall, 68% of patients had one or more lifetime Axis I disorders, including 63.6% of the CAH participants, 90% of 5α-RD-2 participants, and 52.6% of the CAIS participants. The most commonly observed were affective disorders (27.9%), gender identity disorder (27.9%), and anxiety (16.4%). Our study demonstrates that mental health of Iranian patients with DSD is at risk. This might be due to the fact that patients with DSD conditions are mostly treated medically and their mental health is often superficially addressed in developing countries such as Iran, at least in the past. We argue that it is important to pay attention to the mental health issues of patients with DSD and focus on specific issues, which may vary cross-culturally.     

Uterine artery pulsatility index and serum BMP-9 predict resistance to methotrexate therapy in gestational trophoblastic neoplasia: A cohort study

Background: Methotrexate is the most common first-line chemotherapy for low-risk gestational trophoblastic neoplasia (GTN). Uterine artery pulsatility index (UAPI) is an ultrasound marker for tumor vascularity that has been associated with an increased risk of methotrexate resistance. The combination of circulating angiogenic factor levels with UAPI data may improve the capacity of this model to predict chemoresistance. Methods: This was a single-center cohort study of women newly diagnosed between January 2008 and June 2012 with low-risk GTN during postmolar surveillance and treated with single-agent methotrexate at Charing Cross Hospital, a UK national center for treatment of gestational trophoblastic disease. Two hundred seventeen women underwent an ultrasound for UAPI measurement prior to initiation of chemotherapy. To examine serologic markers of methotrexate resistance among this cohort, we performed a case-control study using archived serum from 76 patients who could be matched based on prognostic risk score. Serum samples were examined by immunoassay to measure 8 different angiogenic factors (VEGF-A, FGF-basic, PLGF-1, PDGF-BB, EGF, ANGPT2, BMP-9, and ENG). Receiver-operator characteristic area under the curve (AUC) values were calculated for the ability of each analyte to correctly classify patients as methotrexate sensitive (MTX-S) or resistant (MTX-R). Results: Total human chorionic gonadotropin levels were similar between the MTX-S and MTX-R groups. UAPI values were significantly higher in MTX-S (median 1.30 [interquartile range {IQR} = 0.80-1.90]) compared to MTX-R patients (median 0.875 [IQR = 0.60-1.30]; P < 0.0001) with AUC 0.68 (95% confidence interval 0.61-0.76; P < 0.0001). In univariate analysis, only BMP-9 concentrations were significantly different between groups, lower among MTX-S (median of 225 ng/L, IQR = 170-287) compared to MTX-R patients (median 280 ng/L [IQR = 200-339]; P= 0.03). Combining UAPI with BMP-9 concentration improved prediction for chemoresistance with AUC 0.77 (95% confidence interval 0.66-0.88; P < 0.0001). Conclusion: Circulating levels of BMP-9 are elevated in newly diagnosed women with low-risk GTN destined to fail primary methotrexate therapy. A combined test using serum BMP-9 plus UAPI might improve prediction of MTX-R in low-risk GTN.     

Genetic Variations in Bilirubin Metabolism Genes and Their Association with Unconjugated Hyperbilirubinemia in Adults

Objective : The present study was undertaken to investigate the genotype and allele frequencies of the variants in the four bilirubin metabolism genes (UGT1A1, OATP2, HMOX1, and BLVRA) and their association with hyperbilirubinemia. Material and Methods : Genotyping of 17 genetic variants was performed in 115 adults with hyperbilirubinemia and 150 controls by PCR‐RFLP, GeneScan analysis, and direct DNA sequencing. Results : Genetic polymorphisms of the UGT1A1 promoter, specifically the T‐3279G phenobarbital‐responsive enhancer module and (TA)7 dinucleotide repeat, as well as the intron and coding region variants of the OATP2, HMOX1, and BLVRA genes, were significantly higher among the cases than the controls. Further, nearly 82% of the cases showed the presence of significantly four or more variants as compared to 37% of the controls (P < 0.0001) and the mean total serum bilirubin levels also increased according to the number of variants co‐expressed. Conclusions : This study demonstrates that polymorphisms in the bilirubin metabolism genes had a significant effect on bilirubin levels and could be genetic risk factors for hyperbilirubinemia.