Radiation Emergencies: Evaluation,Management, and Transplantation

Radiation or marrow toxic emergencies can lead to severe pancytopenia along with other multiorgan injury. Experience in managing severe myelosuppression suggests that hematology, oncology and transplantation physicians should participate in preparedness planning for such events. Evaluation and management of marrow injured patients requires their expertise. Understanding of the biology of radiation injury, clinical dosimetry to estimate exposure and defined elements of supportive care are essential for appropriate emergency and follow-up treatment. Some patients with expected radiation exposure >4Gy may have extended myelosuppression and be candidates for consideration of allogeneic hematopoietic stem cell transplantation (HSCT). Issues related to patient screening, supportive care, and planning for transplantation are best addressed ahead of time to enable readily available information and guidelines for patient management. National and international contingency planning for such urgencies is underway as effective emergency mobilization requires forethought, education, and pre-established protocols for treatment. Radiation and marrow toxic emergencies may seem unlikely, but the best approach to appropriate medical support is preparedness, contingency planning, and planned research to improve guidelines for the future.     

Recent Update in Food Protein-Induced Enterocolitis Syndrome: Pathophysiology,Diagnosis, and Management

Food protein-induced enterocolitis syndrome (FPIES), though first reported in the 1970s, remains poorly understood and likely underdiagnosed. It is a non-immunoglobulin E (IgE)-mediated food allergy syndrome, most commonly identified in infancy and childhood. It can manifest as a constellation of symptoms following food ingestion, including repetitive and projectile emesis (1–4 hours), accompanied by pallor, lethargy, muscular hypotonia, and diarrhea (5–10 hours). In more severe reactions, significant leukocytosis with neutrophilia, thrombocytosis, metabolic derangements, methemoglobinemia, anemia, low albumin, and total protein may be present. Hypotension and ultimately hypovolemic distributive shock may occur in up to 15%–20% of cases. The diagnosis of FPIES is challenging and providers continue to face difficulties in management. This review article aims to highlight the most recent updates in epidemiology, natural history, pathophysiology, potential diagnostic markers, and guidelines for the management of FPIES.     

Bordetella pertussis Infection: Pathogenesis, Diagnosis, Management, and the Role of Protective Immunity

 Whooping cough is presently one of the ten most common causes of death from infectious disease worldwide. Despite a high vaccine uptake, resurgences of this disease have been observed in several countries. Virulence factors of Bordetella pertussis include agglutinogens, fimbriae, P.69/pertactin, pertussis toxin, filamentous haemagglutinin, adenylate cyclase, tracheal cytotoxin, dermonecrotic toxin, lipopolysaccharide, tracheal colonisation factor, serum resistance factor, and type III secretion. Virulence factor expression is regulated by the bvgAS locus, a two-component signal transduction system. The pathophysiologic sequence consists of attachment (fimbriae, P.69/pertactin, tracheal colonisation factor, pertussis toxin, filamentous haemagglutinin), evasion of host defence (adenylate cyclase, pertussis toxin, serum resistance factor), local effects (tracheal cytotoxin), and systemic effects (pertussis toxin). Bordetella pertussis is transmitted by respiratory droplets and causes disease only in humans. Various diagnostic methods are available, including culture, serological methods, and the polymerase chain reaction. Serotyping of isolates to detect agglutinogens 2 and 3 is useful because serotype 1,2 may be associated with higher mortality, and antibodies to these antigens (agglutinins) may be protective in both animals and humans. Immunisation using whole-cell vaccine is effective but is reactogenic. Acellular vaccines containing one to five components are being used increasingly in various countries. Protective immunity to pertussis correlates with high levels of antibody to each of pertactin, fimbriae, and pertussis toxin; however, doubt remains as to the relationship between agglutinogen 3 and fimbria 3, making results of trials investigating these virulence factors difficult to interpret.     

Pathogenesis,Diagnosis, and Management of Primary Antibody Deficiencies and Infections

Summary: Primary antibody deficiencies are the most common primary immunodeficiency diseases. They are a heterogeneous group of disorders with various degrees of dysfunctional antibody production resulting from a disruption of B-cell differentiation at different stages. While there has been tremendous recent progress in the understanding of some of these disorders, the etiology remains unknown for the majority of patients. As there is a large spectrum of underlying defects, the age at presentation varies widely, and the clinical manifestations range from an almost complete absence of B cells and serum immunoglobulins to selectively impaired antibody responses to specific antigens with normal total serum immunoglobulin concentrations. However, all of these disorders share an increased susceptibility to infections, affecting predominantly the respiratory tract. A delay of appropriate treatment for some diseases can result in serious complications related to infections, while timely diagnosis and adequate therapy can significantly decrease morbidity and increase life expectancy and quality of life.     

Proximal Rectus Femoris Avulsion: Ultrasonic Diagnosis and Nonoperative Management

Objective

To present a case of ultrasonic diagnosis and nonoperative management of a complete proximal rectus femoris avulsion in a National Collegiate Athletic Association Division 1 soccer goalkeeper.

Background

While delivering a goal kick, a previously uninjured 24-year-old collegiate soccer goalkeeper had the sudden onset of right anterior thigh pain. He underwent rehabilitation with rapid resolution of his presenting pain but frequent intermittent recurrence of anterior thigh pain. After he was provided a definitive diagnosis with musculoskeletal ultrasound, he underwent an extended period of rehabilitation and eventually experienced complete recovery without recurrence.

Differential Diagnosis

Rectus femoris avulsion, rectus femoris strain or partial tear, inguinal hernia, or acetabular labral tear.

Treatment

Operative and nonoperative options were discussed. In view of the player''s recovery, nonoperative options were pursued with a good result.

Uniqueness

Complete proximal rectus femoris avulsions are rare. Our case contributes to the debate on whether elite-level kicking and running athletes can return to full on-field performance without surgery.

Conclusions

Complete proximal rectus femoris avulsions can be treated effectively using nonoperative measures with good preservation of function even in the elite-level athlete. In addition, musculoskeletal ultrasound is an excellent tool for on-site evaluation and may help guide prognosis and management.Key Words: athletic injuries, rehabilitation, soccerComplete proximal rectus femoris avulsions are infrequent. On-field examination can be challenging and may lead to inexact recommendations for rehabilitation and return to play. In addition to history and physical examination, musculoskeletal ultrasound can improve diagnostic accuracy and help direct management.     

Ultrastructural Immunolabeling in the Evaluation, Diagnosis, and Characterization of Neuroendocrine Neoplasms

Neuroendocrine neoplasia represents a heterogenous entity with variable morphologic light microscopic expressions. In many cases a definite diagnosis is easily made by light microscopic examination, but in some cases this does not suffice. In the latter instances, immunocytochemistry, ultrastructural examination, or both are required to diagnose a neuroendocrine neoplasm. However, basing a diagnosis of neuroendocrine neoplasia exclusively on the results obtained from immunocytochemical or ultrastructural evaluation of these tumors may not be entirely accurate in some instances. Ultrastructural immunolabeling plays a key role in accurately defining localization of immunoreactive substances in well-characterized neuroendocrine neoplasms, can assess colocalization of antigenic epitopes, helps define specificity and significance of immunocytochemistry results obtained at the light microscopic level, and is more sensitive than light microscopic immunocytochemistry. Some evolving diagnostic entities can be further characterized by utilization of ultrastructural labeling techniques. Controversies concerning the neuroendocrine nature of electron-dense structures identifiable at the ultrastructural level can be readily and accurately resolved. By providing a way to evaluate combined immunomorphologic parameters, ultrastructural immunogold labeling can settle important questions pertaining to neuroendocrine neoplasia. The present article illustrates a series of cases where the above-mentioned applications were tested.