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Pemphigoid vegetans is a very rare type of bullous pemphigoid which usually affects the elderly and has not been reported in children. It shows a clinical resemblance to pemphigus vegetans but has distinct histological and immunopathological features of bullous pemphigoid. A 9-year-old girl presented with recurrent purulent and verrucous vegetating lesions on her forehead, groin and vulva along with scaling, crusted, bullous and purulent lesions on the eyelids, periorbital, periauricular, perioral region and lips. She had oral lesions and a cerebriform tongue. Though she showed clinical features of pemphigus vegetans, histology revealed a subepidermal blister with the absence of acantholysis. Direct immunofluorescence studies were suggestive of bullous pemphigoid. On clinicopathological correlation, a diagnosis of pemphigoid vegetans was made. She responded well to oral corticosteroids and dapsone therapy with complete resolution of the lesions.

Background: Renal biopsy is a well-established diagnostic modality for the assessment of kidney diseases in children. It can provide diagnostic precision and prognostic value and guide in therapeutic options for many renal diseases., Objectives: This report describes the indication, histopathological patterns, and epidemiology of renal diseases in children in India., Patients and Methods: This is a single-center study on renal biopsies performed between January 2008 and December 2013 in 346 children (age ≤ 14 years)., Results: Eleven (3.17%) biopsies were inadequate, and 335 biopsies were considered for analysis. The mean age was 7.91 ± 3.04 years with a predominance of males (68.1%). Nephrotic syndrome (46.2%) was the most common indication, followed by urinary abnormality (41.19%), acute nephritic syndrome (10.74%), and chronic renal failure (1.79 %). Primary glomerulonephritis (GN) was predominant (81.79%), and secondary GN constituted 16.12% of the biopsies. Primary GN included mesangial proliferative GN (MePGN), IgM nephropathy, focal segmental glomerulosclerosis, minimal change disease, IgA nephropathy, membranoproliferative GN, membranous nephropathy, crescentic GN, and post-infectious GN. Secondary GN revealed lupus nephritis, hemolytic uremic syndrome, amyloidosis, and hypertensive nephropathy. Tubulointerstitial nephritis was observed in 2.08%. The most common histological pattern of primary GN was MePGN (20%) and in secondary GN it was lupus nephritis (7.76%)., Conclusions: The present study provides data on the epidemiology of renal diseases in children in India and will be helpful for developing a national registry and devising therapeutic guidelines.

Isolated thoracic spinous process fractures involving multiple adjacent vertebral segments are a rare occurrence in the setting of high-energy trauma. These findings should prompt further investigation to exclude other concomitant osseous or ligamentous injuries. Evaluation by computed tomography is often most useful to detect these fractures. Proper treatment of extensive multilevel injury is poorly defined in the literature. In our experience, conservative management consisting of initial bracing with graduated lifting restrictions has produced excellent functional results.

Dysphagia after anterior cervical discectomy and fusion (ACDF) is common, with a prevalence ranging between 28% and 57% of cases. However, nearly all cases resolve spontaneously within 2 years, thus identifying patients who require more detailed or invasive work-up is a challenging task for clinicians. A review of literature reveals a paucity of case reports detailing work-up and successful management options. The authors performed a clinical and radiographic review of a case of a 47-year-old female who presented with persistent dysphagia 3 years following anterior cervical spine surgery and was found to have an erosive pharyngeal defect with exposed spinal hardware. The diagnosis was made with direct laryngoscopy and treatment consisted of plate removal and pharyngeal repair, followed by revision fusion with deformity correction. This case and the accompanying pertinent review of the literature highlight the importance of a thorough evaluation of dysphagia, especially in the mid- and late-term postoperative period following ACDF, when most cases of dysphagia should have been resolved. Correctly identifying the underlying etiology of dysphagia may lead to improved revision of ACDF outcomes. Unresolved dysphagia should be a red flag for surgeons as it may be the presentation of erosive esophageal/pharyngeal damage, a rare but serious complication following ACDF.

Atypical hemolytic uremic syndrome (aHUS) although rare is the commonest cause of acute renal failure (ARF) in children and has poor prognosis. We present single centre experience of aHUS. Thirty six children (29 males, 7 females) with mean age, 7.9 years presented with ARF, 2 children also had tonic-clonic type convulsions. Their hematology examination revealed hemolytic anemia with s. creatinine (SCr), 5.54 mg/dl. Acute HUS was observed in 75 %, acute on chronic HUS in 19.4 % and patchy cortical necrosis (PCN) in 5.6 % biopsies. Mean 5.4 plasma exchanges (PE) were carried out. Supportive management of anti-hypertensives and prednisone was also given. Recovery end points were establishment of urine output, improvement of SCr and hematological profile. Hematology and renal function profile improved variably in all children, 5.6 % died, relapse was observed in 80.5 % over mean 70 days; 13.9 % children are doing well over mean follow-up of 268.8 days. Thus poor prognosis was observed in 86.1 % children. Children with acute on chronic HUS and PCN did not recover. Six children who recovered had acute HUS. aHUS in Indian children occurs at an older age of around 8 years and chronic/irreversible changes on histopathology examination are harbingers of poor prognosis. PE is life-saving however further research for developing strategies to improve long-term survival is needed.

Tumors of the parapharyngeal space (PPS) are uncommon, comprising less than 1 % of all head and neck neoplasm's. Neurogenic lesions are the most common tumors of the poststyloid PPS accounting for nearly 25-30 % of all the PPS lesions. Schwannomas are uncommon nerve sheath tumors that may originate from any peripheral, cranial or autonomic nerve of the body, about one-third of them are known to arise in the head and neck region. Trigeminal schwannomas are known to arise from the root, ganglion, or intracranial portion of the three peripheral divisions of the nerve - ophthalmic (CN V1), maxillary (CN V2) and mandibular (CN V3). Schwannomas involving the mandibular division of the trigeminal nerve and localizing exclusively in the parapharyngeal space (PPS) is extremely rare and a surgical approach to such tumors has not been well established. The management of a PPS schwannoma is thus a clinical challenge, an adequate preoperative imaging with the identification of the possible nerve of origin allows for making informed decisions regarding the various approaches of management.

Myoepithelial carcinoma (MC) is a rare neoplasm of the salivary gland generally occurring in the parotid gland and rarely in the minor salivary glands. It poses a diagnostic challenge on fine-needle aspiration (FNA) cytology because it can show different cell types and lack clear features of malignancy. This can lead to a range of differential diagnosis on cytology. The diagnostic difficulty can be compounded if the lesion is present at an unusual site. A 41-year-old male presented with a recurrent swelling on the check since 2 years with a prior history of pleomorphic adenoma (PA) at the same site 8 years back. FNA was performed and a diagnosis of recurrent PA or myoepithelial cell neoplasm was given. Final diagnosis was made on histology and immunohistochemistry studies and reported as MC of minor salivary gland originating within PA. Pathologist should be aware of the occurrence of MC at the sites of the minor salivary glands in the oral cavity and its wide morphologic spectrum to make a confident diagnosis of MC preoperatively.

In the USA, obesity rates have significantly increased in the last 15 years. Mirroring this trend, a large proportion of patients undergoing spinal surgery are obese. Concern exists for increased complications due to surgical challenges posed by obese patients and their often-prevalent comorbidities. Studies have shown associations between body mass index (BMI) and perioperative complications in lumbar and thoracolumbar fusion surgeries; however, few studies have evaluated the impact of obesity on anterior cervical fusion surgery. As such, this study aimed to evaluate complications and perioperative characteristics in obese patients undergoing anterior cervical fusion. We queried medical records to identify patients with BMI >30 who underwent anterior cervical fusion surgery. A total of 69 patients were included and subdivided based on obesity class: Class 1 (BMI 30-35), Class 2 (BMI 35-40), and Class 3 (BMI >40). Subgroup analysis included comorbidities, diagnosis, procedure, levels treated, and length of hospital stay. Overall mean BMI was 35.1, mean age was 54.3 years, and 43 (63.3%) were men. Disc herniation was the most common diagnosis. Length of stay differed significantly among obesity subgroups (p=0.02). Mean length of stay was 2.8, 3.5, and 4.0 days for Classes 1, 2, and 3, respectively. Three (4.3%) complications were observed, comprising of urinary tract infection, wound dehiscence, and neck hematoma. Complication rates by class were 5.5%, 0%, and 16.6% for Classes 1, 2, and 3, respectively (p=0.17). We found that obese patients undergoing anterior cervical spine surgery experience relatively few complications. Hospital stay, however, appears to lengthen with increased BMI., (Copyright © 2013 Elsevier Ltd. All rights reserved.)

Forced degradation is a degradation of new drug substance and drug product at conditions more severe than accelerated conditions. It is required to demonstrate specificity of stability indicating methods and also provides an insight into degradation pathways and degradation products of the drug substance and helps in elucidation of the structure of the degradation products. Forced degradation studies show the chemical behavior of the molecule which in turn helps in the development of formulation and package. In addition, the regulatory guidance is very general and does not explain about the performance of forced degradation studies. Thus, this review discusses the current trends in performance of forced degradation studies by providing a strategy for conducting studies on degradation mechanisms and also describes the analytical methods helpful for development of stability indicating method.

Nodular hidradenomas are benign adenomatous tumours of sweat gland origin, which commonly arise from the skin of the head, neck and extremities. Some of these benign appearing lesions exhibit aggressive clinical behaviour with rapid growth, pigmentation and ulceration. These tumors are designated as "atypical nodular hidradenomas" and are known to histologically possess some focal atypical features i.e. nuclear pleomorphism, focal necrosis or prominent mitotic activity. Nodular hidradenomas involving the pinna are rare and to the best of our knowledge there have been no prior reports in the English language literature. We describe a unique case of a 45-year-old woman with an atypical nodular hidradenoma of the left pinna and discuss the aesthetic and the oncological issues pertaining to the reconstruction of the pinna.

Mucinous cystadenocarcinoma of renal pelvis is a rare epithelial tumor with poor prognosis. It is postulated to arise from metaplastic glandular mucosa in response to chronic irritation, and comprises less than 0.3% of total renal pelvic tumors. We present this case of a tumor noted in a 45-year-old lady that was diagnosed as mucinous cystadenocarcinoma on histological examination after radical nephrectomy. The patient is remaining well over a follow-up of three months.

Background: The management of esophageal cancer continues to be riddled with controversies, even as more and more clinical trials are being conducted amid a remarkable change in histology and epidemiology. Significant variations exist in the surgical treatment of esophageal cancer, and there is no consensus on the best surgical approach or the extent of lymphadenectomy. Interestingly, extended esophagectomy (three-field lymphadenectomy) has not been compared with transhiatal esophagectomy in a head-to-head fashion., Methods: We did a retrospective comparison of 111 consecutive patients who underwent curative resection for carcinoma of the esophagus, via either a transthoracic esophagectomy with three-field dissection (3F TTE) or transhiatal esophagectomy ("THE") at a regional cancer center in South India over a period of 5 years from 2002 to 2006. The primary outcome measure was 5-year disease-free (DFS) and the overall survival (OS). An exhaustive analysis of the short-term outcomes was also made., Results: The 5-year overall survival and disease-free survival were 52 and 49% in the 3F TTE group and 37 and 37%, respectively, in the "THE" group, which were not statistically significant. The short and the long-term outcomes in both the groups compared favorably with the other published series., Conclusions: Our study possibly for the first time compares 3F TTE and "THE" in the management of resectable carcinoma of the esophagus. Although the survival outcomes of both the groups were not statistically different, 3F TTE did show a trend towards improved DFS and OS when compared to "THE" group. However, this being a retrospective study, the results of this analysis need to be verified in an adequately sized prospective randomized study.

Magnetic resonance imaging (MRI) has been shown to be sensitive in identifying ligamentous injury to the cervical spine. The major drawbacks to its routine use are cost and availability. The purpose of this study was to compare the cost of using MRI to rule out ligamentous injury of the cervical spine with the cost of immobilization in a cervical collar and outpatient follow-up. Neurologically intact and nonobtunded patients with neck pain and normal findings on radiographs evaluated for ligamentous injury of the cervical spine were studied. Patients were either evaluated with MRI or immobilized in a cervical collar and followed up for repeat clinical and radiographic evaluation as outpatients. The authors gathered year 2011 fees from their institution and 2011 Medicare reimbursement data and compared the costs of MRI with the costs of cervical collar and outpatient follow-up. In addition, the median income of the local community was used to estimate opportunity costs associated with cervical collar immobilization. After 7 days of lost wages at the median local income, MRI became a less costly option when comparing hospital fees. Alternatively, when considering Medicare reimbursement, MRI became less costly after only 2 days of lost wages at the median local income. On the basis of these findings, MRI of the cervical spine is less costly than other current management strategies when opportunity costs are considered., (Copyright 2014, SLACK Incorporated.)

Background: This is the first study on phenotype frequencies of various blood group systems in blood donors of south Gujarat, India using conventional tube technique., Material and Methods: A total of 115 "O" blood group donors from three different blood banks of south Gujarat were typed for D, C, c, E, e, K, Jk(a), Le(a), Le(b), P1, M, and N antigens using monoclonal antisera and k, Kp(a), Kp(b), Fy(a),Fy(b), Jk(b), S,s, Lu(a), and Lu(b) antigens were typed using polyclonal antisera employing Indirect Antiglobulin Test. Antigens and phenotype frequencies were expressed as percentages., Results: From the 115 blood donor samples used for extended antigen typing in the Rh system, e antigen was found in 100% donors, followed by D [84.35%], C [81.74%], c [56.32%], and E [21.74%] with DCe/DCe (R1 R1, 40.87%) as the most common phenotype. k was found to be positive in 100% of donors and no K+k- phenotype was found in Kell system. For Kidd and Duffy blood group system, Jk(a+b+) and Fy(a-b-) were the most common phenotypes with frequency of 52.17% and 48.69%, respectively. In the MNS system, 39.13% donors were typed as M+N+, 37.39% as M+N-, and 23.48% as M-N+. S+s+ was found in 24.35% of donors, S+s- in 8.69%, and S-s+ as the commonest amongst donors with 66.96%. No Lu(a+b+) or Lu(a+b-) phenotypes were detected in 115 donors typed for Lutheran antigens. A rare Lu(a-b-) phenotype was found in 2.61% donors., Conclusion: Data base for antigen frequency of various blood group systems in local donors help provide antigen negative compatible blood units to patients with multiple antibodies in order to formulate in-house red cells for antibody detection and identification and for preparing donor registry for rare blood groups.

Background Context: The objective of the North American Spine Society's (NASS) Evidence-Based Clinical Guideline for the Diagnosis and Treatment of Lumbar Disc Herniation with Radiculopathy is to provide evidence-based recommendations to address key clinical questions surrounding the diagnosis and treatment of lumbar disc herniation with radiculopathy. The guideline is intended to reflect contemporary treatment concepts for symptomatic lumbar disc herniation with radiculopathy as reflected in the highest quality clinical literature available on this subject as of July 2011. The goals of the guideline recommendations are to assist in delivering optimum efficacious treatment and functional recovery from this spinal disorder., Purpose: To provide an evidence-based educational tool to assist spine specialists in the diagnosis and treatment of lumbar disc herniation with radiculopathy., Study Design: Systematic review and evidence-based clinical guideline., Methods: This guideline is a product of the Lumbar Disc Herniation with Radiculopathy Work Group of NASS' Evidence-Based Guideline Development Committee. The work group consisted of multidisciplinary spine care specialists trained in the principles of evidence-based analysis. A literature search addressing each question and using a specific search protocol was performed on English-language references found in Medline, Embase (Drugs and Pharmacology), and four additional evidence-based databases to identify articles. The relevant literature was then independently rated using the NASS-adopted standardized levels of evidence. An evidentiary table was created for each of the questions. Final recommendations to answer each clinical question were developed via work group discussion, and grades were assigned to the recommendations using standardized grades of recommendation. In the absence of Level I to IV evidence, work group consensus statements have been developed using a modified nominal group technique, and these statements are clearly identified as such in the guideline., Results: Twenty-nine clinical questions were formulated and addressed, and the answers are summarized in this article. The respective recommendations were graded by strength of the supporting literature, which was stratified by levels of evidence., Conclusions: The clinical guideline has been created using the techniques of evidence-based medicine and best available evidence to aid practitioners in the care of patients with symptomatic lumbar disc herniation with radiculopathy. The entire guideline document, including the evidentiary tables, suggestions for future research, and all the references, is available electronically on the NASS Web site at http://www.spine.org/Pages/PracticePolicy/ClinicalCare/ClinicalGuidlines/Default.aspx and will remain updated on a timely schedule., (Copyright © 2014 Elsevier Inc. All rights reserved.)

There is considerable interest in transplanting stem cells or progenitors into the injured nervous system and enhancing their differentiation into mature, integrated, functional neurons. Little is known, however, about what intrinsic or extrinsic signals control the integration of differentiated neurons, either during development or in the adult. Here we ask whether purified, postmitotic, differentiated retinal ganglion cells (RGCs) directly isolated from rat retina or derived from in vitro-differentiated retinal progenitor cells can survive, migrate, extend neurites, and form morphologic synapses in a host retina, in vivo and ex vivo. We found that acutely purified primary and in vitro-differentiated RGCs survive transplantation and migrate into deeper retinal layers, including into their normal environment, the ganglion cell layer (GCL). Transplanted RGCs from a wide range of developmental ages, but not from adults, were capable of extending lengthy neurites in the normal and injured adult rat retina ex vivo and to a lesser degree after transplantation in vivo. We have also demonstrated that RGCs may be differentiated and purified from retinal precursor cultures and that they share many of the same cell biological properties as primary RGCs. We have established that progenitor-derived RGCs have similar capacity for integration as developing primary RGCs but appear to form a lower number of presynaptic punctae. This work provides insight for further understanding of the integration of developing RGCs into their normal environment and following injury.

Study Design: Retrospective review., Objective: To describe a novel application of rotational sternocleidomastoid (SCM) muscle flap in management of ventral cervical durotomy., Summary of Background Data: Even for the most experienced surgeons, incidental durotomy is a common occurrence in spine surgery. Primary direct suture repair is indicated to avoid possible complications such as pseudomeningocele or spinocutaneous fistula formation. Significant secondary effects of these complications have been described, including airway compromise, radiculopathy, myelopathy, and infection. When primary repair is not feasible, surgeons have used alternative management techniques based on their clinical judgment. In the setting of persistent symptomatic cerebrospinal fluid leak after repair, reoperation is warranted., Methods: A retrospective review was conducted of clinical records and radiographic data for 2 patients who underwent reoperation for management of ventral cervical durotomy encountered during anterior cervical spine surgery. SCM muscular flap was used to augment durotomy repair., Results: Both patients did not have any persistent cerebrospinal fluid leak after repair with pedicled SCM muscle flap, and did not require any further surgical procedures related to the cervical spine., Conclusions: The use of a rotational SCM muscular flap may be useful in cases of ventral cervical durotomy refractory to conventional management.

Purpose: To explore the association of angiographic nonperfusion in focal and diffuse recalcitrant diabetic macular edema (DME) in diabetic retinopathy (DR)., Design: A retrospective, observational case series of patients with the diagnosis of recalcitrant DME for at least 2 years placed into 1 of 4 cohorts based on the degree of DR., Methods: A total of 148 eyes of 76 patients met the inclusion criteria at 1 academic institution. Ultra-widefield fluorescein angiography (FA) images and spectral-domain optical coherence tomography (SD OCT) images were obtained on all patients. Ultra-widefield FA images were graded for quantity of nonperfusion, which was used to calculate ischemic index. Main outcome measures were mean ischemic index, mean change in central macular thickness (CMT), and mean number of macular photocoagulation treatments over the 2-year study period., Results: The mean ischemic index was 47% (SD 25%; range 0%-99%). The mean ischemic index of eyes within Cohorts 1, 2, 3, and 4 was 0%, 34% (range 16%-51%), 53% (range 32%-89%), and 65% (range 47%-99%), respectively. The mean percentage decrease in CMT in Cohorts 1, 2, 3, and 4 were 25.2%, 19.1%, 11.6%, and 7.2%, respectively. The mean number of macular photocoagulation treatments in Cohorts 1, 2, 3, and 4 was 2.3, 4.8, 5.3, and 5.7, respectively., Conclusions: Eyes with larger areas of retinal nonperfusion and greater severity of DR were found to have the most recalcitrant DME, as evidenced by a greater number of macular photocoagulation treatments and less reduction in SD OCT CMT compared with eyes without retinal nonperfusion. Areas of untreated retinal nonperfusion may generate biochemical mediators that promote ischemia and recalcitrant DME., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Background: Cancer patients who continue smoking are at increased risk for adverse outcomes including reduced treatment efficacy and poorer survival rates. Many patients spontaneously quit smoking after diagnosis; however, relapse is understudied. The goal of this study was to evaluate smoking-related, affective, cognitive, and physical variables as predictors of smoking after surgical treatment among patients with lung cancer and head and neck cancer., Methods: A longitudinal study was conducted with 154 patients (57% male) who recently quit smoking. Predictor variables were measured at baseline (ie, time of surgery); smoking behavior was assessed at 2, 4, 6, and 12 months after surgery. Analyses of 7-day point prevalence were performed using a Generalized Estimating Equations approach., Results: Relapse rates varied significantly depending on presurgery smoking status. At 12 months after surgery, 60% of patients who smoked during the week prior to surgery had resumed smoking versus only 13% who were abstinent prior to surgery. Smoking rates among both groups were relatively stable across the 4 follow-ups. For patients smoking before surgery (N = 101), predictors of smoking relapse included lower quitting self-efficacy, higher depression proneness, and greater fears about cancer recurrence. For patients abstinent before surgery (N = 53), higher perceived difficulty quitting and lower cancer-related risk perceptions predicted smoking relapse., Conclusions: Efforts to encourage early cessation at diagnosis, and increased smoking relapse-prevention efforts in the acute period following surgery, may promote long-term abstinence. Several modifiable variables are identified to target in future smoking relapse-prevention interventions for cancer patients., (Copyright © 2012 American Cancer Society.)

The biology and clinical behavior of Head and Neck Squamous Cell Carcinomas (HNSCCs) is very distinct within different subgroups due to the distinct molecular profiles for the HPV positive versus HPV negative tumors. HPV status is the most important independent prognostic variable in multivariate analysis taking into account all other prognostic factors like tumour stage, smoking status, age and performance status. The debate today is whether the intense therapy is too aggressive in this group of patients since they show a superior survival regardless of treatment strategies. A highly divergent prognosis and distinct biology of HPV positive and HPV negative HNSCCs underlines the fact that treating them as distinct diseases is the need of the hour. Infection with HPV is associated with less aggressive disease, better loco regional control and lower rates of second primary cancers. An important caveat that remains is the emergence of intermediate prognosis of HPV positive smokers and HPV negative non smokers. Though molecular biology has provided important data on the interaction of the HPV onco proteins with genes important in cell cycle control, also speculated to be involved in pathogenesis of HNSCC, more basic research is needed to describe the differential mechanisms of tumorigenesis among the HNSCCs that show presence and absence of HPV. This is clinically relevant to reduce morbidity without compromising tumour control in HPV positive patients and improving tumour control and co-morbid illness that could be pre-existing or treatment related in HPV negative patients. There may be a need for treatment intensification and incorporation of newer agents into induction chemotherapy protocols for the HPV negative patients and so HPV detection is important to aid in this selection. HPV tumour status is therefore more important than just providing the prognostic information in these classes of tumours. This article discusses the role and clinical relevance of HPV in HNSCCs.

Recurrence of primary focal segmental glomerulosclerosis (FSGS) is an important cause of graft loss after renal transplantation (RTx). We report our experience in 34 patients with primary FSGS who underwent RTx between April 1999 and June 2009, using hematopoietic stem cell transplantation (HSCT). They belonged to four groups: group 1 (n = 12) received high-dose HSCT in periphery, thymus, bone-marrow, and portal circulation with low-dose non-myeloablative conditioning; group 2 (n = 7) was modified with HSCT without marrow/thymic infusion; and group 3 (n = 3) received HSCT and proteasome inhibitor Bortezomib replacing conditioning. Group 4 (n = 12), were controls who opted for RTx under standard triple-drug immunosuppression. Patient/donor demographics were comparable in all. No recurrence was noted in group 1 with mean follow-up of 8.1 years, whereas 28.6% of group 2, 33.3% of group 3, and 36.4% of group 4 had recurrence over mean follow-up of 2.6, 1.1, and 6.5 years, respectively. Mean serum creatinine was 1.62, 1.69, 1.41, and 1.73 mg%, respectively. Rejections were noted in 41.7%, 28.6%, 0%, and 45.5% grafts, respectively. Groups 1 and 4 had 25% patient loss each, group 2 had 28.6% loss, and no loss was observed in group 3. Graft loss was noted in 33.3% in group 1, 14.3% in group 2, nil in group 3, and 16.7% in the last group. Recurrent FSGS was prevented in RTx with HSCT in thymic, marrow infusion under low-dose non-myeloablative conditioning compared to controls and Bortezomib group, thus suggesting potential role of central tolerance in FSGS.

Background: Atypical hemolytic uremic syndrome (aHUS), although rare, is the most common cause of acute renal failure (ARF) in children and has poor prognosis. We present a single centre experience of aHUS., Methods: Thirty six children (29 males, 7 females), with mean age 7.9 years, presented with ARF, 2 children also had tonic-clonic type convulsions. Their hematology examination revealed hemolytic anemia with serum create-nine (SCr) of 5.54 mg/dL. Acute HUS was observed in 75%, acute-on-chronic HUS in 19.4%, and patchy cortical necrosis (PCN) in 5.6% biopsies. A mean of 5.4 plasma exchanges (PE) were carried out. Supportive management of anti-hypertensives and prednisone was also given. Recovery end points were establishment of urine output and improvement of SCr and hematological profile., Results: Hematology and renal function profile improved variably in all children, 5.6% died, relapse was observed in 80.5% over a mean of 70 days; 13.9% children are doing well over a mean follow-up of 268.8 days. Thus, poor prognosis was observed in 86.1% children. Children with acute or chronic HUS and PCN did not recover. Six children who recovered had acute HUS., Conclusions: aHUS in Indian children occurs at an older age of around 8 years and chronic/irreversible changes on histopathology examination are harbingers of poor prognosis. PE is life-saving; however, further research for developing strategies to improve long-term survival is needed.

Metanephric adenomas are a rare neoplasm of the kidney with less than 200 cases reported. We report a case of a metanephric adenoma incidentally found on imaging in a 52-year-old Hispanic female and treated with robot-assisted laparoscopic partial nephrectomy. A brief review of the literature is also included.

The aim of the study was to formulate and evaluate nanoparticles based on albumin to deliver 5-fluorouracil. The nanoparticles were prepared by coacervation method. The nanoparticles were characterized for particle size, surface charge, size distribution and drug loading capacity. The drug loading capacity varied from 4.22% to 19.8% (w/w). The mean particle size was 141.9 nm and surface charge was -30.3 mV. The drug loaded particles exerted a bi-phasic release pattern with an initial burst effect followed by a sustained release in pH 7.4 phosphate buffer. The drug release was first order diffusion controlled and the mechanism was Fickian. The drug loaded nanoparticles showed superior cytotoxicity when compared to the free drug., (Copyright © 2012 Elsevier B.V. All rights reserved.)

Object: The theoretical advantage of pars interarticularis repair over spinal fusion to correct pars defects is that the treatment is a direct osteosynthesis that preserves motion at the involved functional spinal unit. Several techniques and constructs have been used to achieve greater rigidity, but these techniques may risk entry into the spinal canal, and adverse events are common. A pedicle and laminar screw construct placed entirely outside the spinal canal may offer greater stiffness and achieve higher pars defect healing rates. The purpose of this study was to biomechanically assess an intralaminar screw construct in cadaveric lumbar spines in comparison with other types of constructs typically used in pars repair and to quantify the sizes of screws that can be placed safely in both normal and spondylolytic vertebrae., Methods: The L-4 and L-5 laminae in patients with spondylolysis and in controls who underwent CT (n = 41, each group) were measured by analysis of conventional axial CT images and multiplanar reformations constructed on a Vitrea workstation to determine the feasibility of translaminar fixation with a 4.5-mm-diameter screw. Biomechanical tests for torsion and flexion-extension were performed on 8 fresh human cadaveric lumbar spines before and after modeling for bilateral spondylolytic defects. Three pars repair techniques were tested at each level and in the following sequence: pedicle screw-cable, pedicle screw-rod-hook, and pedicle screw-intralaminar screw., Results: The majority of laminae can accept 4.5 × 25-mm screws. The cable construct allowed the greatest motion and least stability across the defect in all biomechanical tests. The hook and laminar screw constructs performed similarly in all tests and exhibited no significant difference in stiffness., Conclusions: A surgically placed intralaminar screw construct may be a safe and effective alternative to current pars repair methods.

Introduction: Superporous hydrogel (SPH) swells very rapidly in a shorter period of time to an equilibrium size and contains highly porous structure., Aim: The synthesis of SPH of poly (acrylamide-co-acrylic acid) and its composites viz. Ac-Di-Sol and polyvinylpyrollidone (PVP) was carried out by solution polymerization., Materials and Methods: The SPH and SPH composites (SPHCs) were characterized by measurement of apparent density, porosity, swelling, mechanical strength, and scanning electron microscopy (SEM) studies., Results: FTIR studies confirmed the existence of acrylamide and acrylic acid in SPH. In distilled water SPH showed tremendous increase in equilibrium swelling capacity with conventional SPH as compared to its SPHCs of Ac-Di-Sol and PVP due to the increased in physical cross-linking network, respectively. The presence of Ac-Di-Sol and PVP in SPHCs increased the mechanical strength as compared to conventional SPH which is suitable for gastric retention. SEM pictures clearly indicated the formation of interconnected pores and capillary channels., Conclusion: The amount and type of polymers used affect almost all the characterization parameters of SPHs, and hence, depending upon the applications perspective such polymers could be used in drug delivery systems, successfully.

Unlabelled: The aryl hydrocarbon receptor (AhR) is a ligand-activated transcription factor. Activation of AhR mediates the expression of target genes (e.g., CYP1A1) by binding to dioxin response element (DRE) sequences in their promoter region. To understand the multiple mechanisms of AhR-mediated gene regulation, a microarray analysis on liver isolated from ligand-treated transgenic mice expressing a wild-type (WT) Ahr or a DRE-binding mutant Ahr (A78D) on an ahr-null background was performed. Results revealed that AhR DRE binding is not required for the suppression of genes involved in cholesterol synthesis. Quantitative reverse-transcription polymerase chain reaction performed on both mouse liver and primary human hepatocyte RNA demonstrated a coordinated repression of genes involved in cholesterol biosynthesis, namely, HMGCR, FDFT1, SQLE, and LSS after receptor activation. An additional transgenic mouse line was established expressing a liver-specific Ahr-A78D on a Cre(Alb)/Ahr(flox/flox) background. These mice displayed a similar repression of cholesterol biosynthetic genes, compared to Ahr(flox/flox) mice, further indicating that the observed modulation is AhR specific and occurs in a DRE-independent manner. Elevated hepatic transcriptional levels of the genes of interest were noted in congenic C57BL/6J-Ah(d) allele mice, when compared to the WT C57BL/6J mice, which carry the Ah(b) allele. Down-regulation of AhR nuclear translocator levels using short interfering RNA in a human cell line revealed no effect on the expression of cholesterol biosynthetic genes. Finally, cholesterol secretion was shown to be significantly decreased in human cells after AhR activation., Conclusion: These data firmly establish an endogenous role for AhR as a regulator of the cholesterol biosynthesis pathway independent of its DRE-binding ability, and suggest that AhR may be a previously unrecognized therapeutic target., (Copyright © 2012 American Association for the Study of Liver Diseases.)

Oral streptococci have been associated with systemic diseases, including infective endocarditis and neutropenic bacteremia. We analyzed 58 recent oral streptococcal bloodstream isolates, and we obtained clinical and demographic data for source patients. The sodA gene was found to be a better target than the 16S-23S rRNA internal transcribed spacer for DNA sequence-based species identification. Together, Streptococcus mitis and Streptococcus oralis were significantly more likely than the 12 combined remaining species to be isolated from neutropenic patients.

Background: Pemphigus vulgaris (PV), an autoimmune disorder characterized by blistering skin/mucus membrane lesions, is mediated by desmoglein-3 autoantibodies. We carried out a prospective clinical trial of hematopoietic stem cell transplantation (HSCT) in thymus, bone marrow (BM) and periphery to reconstitute central and peripheral arms of self-tolerance., Materials and Methods: Eleven (M:F=5:6) patients with mean age 33.5 years and mean duration of disease 22.8 months, having painful pruritic blisters and ulcers resistant to corticosteroids, were treated with cytokine-stimulated allogeneic HSCT (mean dose: 21.8 × 10(8) cells/kg BW) from blood group-matched related donors. BM with mean CD34+ count 1.1% was inoculated into thymus, marrow and periphery, followed by two peripheral blood stem cell (PBSC) infusions., Results: Recovery began within 24 hours of HSCT and new lesions stopped after 6 months. No graft versus host disease (GvHD)/adverse effect was observed in any patient/donor. Over a mean follow-up of 8.02 years, all patients were well without recurrence/new lesions., Conclusion: Drug-resistant PV can be successfully and safely treated by allogeneic HSCT.

Ranibizumab, a recombinant, humanized, monoclonal antibody antigen-binding fragment that neutralizes all VEGF-A isoforms, is the first US FDA-approved therapy for neovascular age-related macular degeneration (AMD) to result in improvement in visual acuity. The benefit of intravitreal ranibizumab applies to all angiographic subtypes of neovascular AMD and across all lesion sizes. The two original phase III studies (ANCHOR and MARINA) demonstrated sustained visual acuity (VA) gains over a two-year monthly dosing schedule. Following these trials, several studies looked at ways to decrease the treatment burden while maintaining similar visual gains. These trials included PIER, PrONTO, EXCITE, SUSTAIN, HORIZON, and CATT. Visual acuity data shows that monthly dosing of ranibizumab produces superior vision outcomes compared to a less-frequent, fixed-dosing schedule. Intravitreal ranibizumab is well tolerated and shown to have a very low rate of adverse ocular or systemic side-effects.

The silent sinus syndrome is characterized by atelectasis of the maxillary sinus in the setting of subclinical maxillary sinusitis and obstruction of the osteomeatal unit. The resultant expansion of orbital volume causes enophthalmos and hypoglobus. A review of the literature reveals only unilateral cases. The authors present a case of bilateral silent sinus syndrome presenting with chronic ocular surface disease. A 56-year-old man was referred for tarsorrhaphy to treat a chronic recurrent corneal ulcer. Exam revealed an area of corneal thinning in the left eye. The patient appeared cachectic and there was enophthalmos, hypoglobus, and lagophthalmos on both sides, left worse than right. Due to the patient's appearance and a history of sinonasal malignancy, recurrent malignancy was suspected. A systemic workup was unrevealing, and radiographic studies showed the features of silent sinus syndrome bilaterally. The patient underwent sinus surgery and placement of a left orbital floor implant.