Notes

Tracheobronchopathia osteochondroplastica-stalactite regarding air passage.
To the authors' knowledge, this is the first report of morbillivirus disease in killer whales.Polysaccharide storage myopathies have been described in several animal species and are characterized by periodic acid-Schiff (PAS)-positive, diastase-resistant intrasarcoplasmic inclusions in myocytes. Skeletal and cardiac muscle samples from a subset of a single pod of stranded long-finned pilot whales (Globicephala melas) were evaluated by light and transmission electron microscopy. Twelve individuals demonstrated sporadic basophilic packets of PAS-positive, diastase-resistant complex polysaccharide material, either centrally or peripherally, in skeletal and cardiac myocytes. Few microscopic myopathic changes were found but included focal inflammation and internalized nuclei. Ultrastructurally, the inclusions consisted of loosely arranged, tangled filaments and were not membrane-bound, which is consistent with polyglucosan inclusions. Within skeletal muscle, the number of inclusions had a marginal statistically significant (P = 0.0536) correlation with length, as a proxy for age, suggesting that such inclusions in skeletal muscles may be age-related, although the cause remains unclear.A Himalayan Rex guinea pig was presented with a history of nodular, partially ulcerated masses in the subcutis of the left shoulder. Histological examination revealed a garland-like to nodular, infiltrative neoplastic mass of the epidermis and hair follicle epithelium, which obscured the dermoepidermal junction. Neoplastic cells were immunopositive for S100, PNL-2, vimentin and melan-A antigens. No immunolabelling of CD3, CD79, Iba-1 or pancytokeratin was observed. This is the first detailed description of spontaneous amelanotic malignant melanoma in this species.We describe a fatal kidney disease in green iguanas (Iguana iguana), associated with severe nephromegaly. Affected animals have enlarged kidneys, which fill the pelvic cavity, leading to compression of adjacent organs, obstipation and, ultimately, death. The pathological features of this disease have been poorly described and its aetiology is unknown. We performed detailed gross and histological analyses of 17 green iguanas with a clinical diagnosis of nephromegaly, and compared the results with those of eight healthy controls. Grossly, the kidneys of all 17 individuals were markedly enlarged and the distal colons were distended and overfilled with faecal contents. Histopathological examination revealed that these enlarged kidneys consisted mainly of tubular hyperplasia, resembling poorly developed proximal segments. The nephrogenic zones were either poorly developed or absent. There was a reduction in the density of glomeruli and the distal segments were reduced in number. There was no histological evidence of an aetiology for the nephron disruption and nephromegaly.Anorectal malformations (ARM) are well recognized to be associated with anomalies in other organ systems. The introduction of screening protocols has increased the diagnosis of these anomalies and greater collaboration with other specialties has influenced the treatment and follow-up of patients with ARMs. Much of the medical literature regarding the treatment of anorectal malformations has focused on technical details of operations and early post-surgical outcomes. Recently, an increase in published data regarding the long-term sequelae of an ARM diagnosis has resulted in an emphasis extended follow up in this population. Patient support groups have highlighted complex issues in ARM patients persist into adulthood have advocated for improved transitional care. This article describes the benefits of long-term follow-up and identifies key issues in ARM patients with respect to urologic and gynecologic health. A collaborative model of care is outlined and suggested timings of screening for potential problems is described.In this review, the care of children with a previously repaired anorectal malformation is explored. We know that the surgical care of children with anorectal malformations is complex; however, despite an increased understanding of the congenital anomaly and significant technical advances in the operative repair, many of these children continue to have poor functional outcomes. In this article we focus on the common surgical complications, discuss typical presentations, consider appropriate investigations, and review the risks and benefits of revisional surgery in those patients that are 'not doing well' following their primary reconstruction.Caring for children with colorectal and pelvic reconstructive needs is complex and requires a lifelong commitment from clinicians devoted to the field. There has been a myriad of advances that have improved care and it has become clear that an integrated, multi-disciplinary approach maximizes the goal of improving the quality of life of children afflicted with these conditions. The purpose of this review is to briefly discuss the history of this field and to describe the key advances that have improved patients' lives.During this early part of the 21st century online technology has prompted many major advances in medical care. In this section we argue that this is particularly evident in the treatment and care of patients born with Anorectal Malformation (ARM) and Hirschsprung's Disease (HD). Our stories show that anyone born with these complex colorectal conditions in the 20th century was destined to a life of isolation and stigma. Here we explore the lack of understanding and recognition of the psychological effects on children and families which has characterised this period. We show that advances in clinical practice has been supported by developing social media platforms. There has been a rapid creation of online support groups for patients and families which has enabled survivors' greater access to patient and parent organizations across the globe and thereby stimulated a sense of belonging and solidarity. Selleck TL12-186 Online technology and social media platforms have also opened up the opportunity for pediatric medical professionals to provide a greater level of patient education. There is no doubt families have become much more aware of the complexities of ARM & HD and achieved greater comfort and understanding of their needs. We have generated "lightbulb moments" for pediatric providers with adult ARM & HD patients, enabling them to share their lived experiences in a therapeutic exchange. In the past survivors felt they were abandoned by the adult healthcare system. We are seeing evidence-based research of major psychosocial issues experienced by adult patients and, as a result, improved understanding of how to treat ARM & HD survivors across their whole of life journey. The winds of change continue to direct our cohorts to a mature approach based on improving levels of interactive communication and education. We argue that this maturity has mostly been facilitated by the use of online technology and the ensuing collaboration between providers and patient and parent organizations.Constipation and fecal incontinence are common problems in children after repair of an anorectal malformation (ARM). While many children can be effectively managed with an oral laxative regimen, others require a mechanical colonic washout to achieve social continence. Appendicostomy and cecostomy are two techniques which permit antegrade access to the colon for the purpose of enema delivery, which improves compliance and quality of life for patients and families. The purpose of this article is to review, using a case-based approach, the indications for placement of a channel for antegrade enema access, clinical scenarios in which one technique would be preferred over another, common complications following each procedure.Anorectal malformations affect 1 in 3000-5000 children, with varied incidences dependent upon geographical location. Accurate assessment, and subsequent targeted management in the newborn is critical to reducing potential morbidity and mortality. We have focused in this review upon the management of newborns with anorectal malformations, and the evaluation of the potential for long-term fecal continence.Planned health care transition can improve the ability of young adults to manage their own health care to effecively use health services and ultimately maximize life-long functioning and well-being. Transitional care is a purposeful, planned process that addresses the medical, psychosocial and educational needs of adolescents and young adults with chronic physical and medical conditions as they move from child-centered to adult-oriented healthcare systems. Unsuccessful surgical transtion may result in physical and mental health implications for young patients, negative long-term outcomes and suboptimal use of health care resources. Anorectal malformation and Hirschsprung patients are an especially vulnerable patient population with ongoing surgical, physiologic and pyschosocial challenges.The treatment of patients with colorectal disorders and their associated urologic, gynecologic, gastrointestinal, spinal, and orthopedic anomalies requires care from various medical and surgical specialties over the course of their lifetime. This is ideally handled by a collaborative center which facilitates the assessment and development of a long-term patient care plan among multiple specialties which can enhance the quality of care, improve communication among different specialties, and improve patient satisfaction and outcomes. We describe the process, as well as lessons learned in developing such a center.Optimal outcomes in the management of children with Anorectal Malformation (ARM) require careful surgical preparation and detailed understanding of the anatomic principles and operative setup. A clear understanding of operative anatomy and surgical principals guides decision making. Adherence to the principles of ARM repair, as well as the application of operative and imaging adjuncts, will yield the safest and most successful approach to ARM. In this review, we detail the surgical preparation, anatomic principles, and surgical management issues unique to ARM.Imaging is extremely important throughout all phases of care provided to children with anorectal malformations (ARM). A preoperative determination of the patient specific malformation will help establish the operative plan. Moreover, the majority of ARM patients will have an associated anomaly that will require imaging workup for full understanding of those abnormalities prior to addressing the ARM. The complexity of ARM care will mandate continued imaging throughout the post-operative period even in those with straight forward malformations.Colorectal disease profiles for children in low- and middle-income settings (LMIC) are characterized by late presentation, increased complications and limited follow-up in many cases. There is a high prevalence of infectious conditions causing secondary colorectal disease such as Mycobacterium Tuberculosis(TB), Human Immunodeficiency Virus(HIV) and Human Papilloma Virus(HPV), which also impact the management of other primary colorectal conditions, such as wound-healing and intestinal anastomosis. Perineal trauma from sexual assault, motor vehicle or pedestrian accidents, burns, and traditional enemas are commonly encountered and may require adaptation of principles used in treatment of congenital anomalies such as Hirschsprung's disease and Anorectal Malformations for reconstruction. Endemic conditions in certain LMIC require further research to delineate underlying causes and optimize management, such as "African" degenerative visceral leiomyopathy, congenital pouch colon in the Indian subcontinent, and congenital H-type rectal fistulae prevalent in Asia.
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