Clinical Determination of Brain Death in Children Supported by Extracorporeal Membrane Oxygenation.

BACKGROUND/OBJECTIVE: Children supported by extracorporeal membrane oxygenation (ECMO) are at risk of catastrophic neurologic injury and brain death. Timely determination of brain death is important for minimizing psychological distress for families, resource allocation, and organ donation. Reports of successful determination of brain death in pediatric patients supported by ECMO are limited. The determination of brain death by clinical criteria requires apnea testing, which has historically been viewed as challenging in patients supported by ECMO. We report eight pediatric patients who underwent a total of 14 brain death examinations, including apnea testing, while supported by veno-arterial ECMO (VA-ECMO), resulting in six cases of clinical determination of brain death. METHODS: We performed a retrospective review of the medical records of pediatric patients who underwent brain death examination while supported by VA-ECMO between 2010 and 2018 at a single tertiary care children's hospital. RESULTS: Eight patients underwent brain death examination, including apnea testing, while supported by VA-ECMO. Six patients met criteria for brain death, while two had withdrawal of technical support after the first examination. During the majority of apnea tests (n = 13/14), the ECMO circuit was modified to achieve hypercarbia while maintaining oxygenation and hemodynamic stability. The sweep flow was decreased prior to apnea testing in ten brain death examinations, carbon dioxide was added to the circuit during three examinations, and ECMO pump flows were increased in response to hypotension during two examinations. CONCLUSIONS: Clinical determination of brain death, including apnea testing, can be performed in pediatric patients supported by ECMO. The ECMO circuit can be effectively modified during apnea testing to achieve a timely rise in carbon dioxide while maintaining oxygenation and hemodynamic stability.

Septic Shock Caused by Rocky Mountain Spotted Fever in a Suburban Texas Patient with Pet Dog Exposure: A Case Report.

BACKGROUND Rocky Mountain spotted fever (RMSF) is associated with high mortality and requires prompt identification and treatment to ensure better outcomes. CASE REPORT We describe an advanced case of RMSF in a 45-year-old female patient with pet dog exposure who presented with altered mental status, dyspnea, and ataxia progressing to septic shock and acute hypoxic respiratory failure requiring intubation and mechanical ventilation. CONCLUSIONS This case illustrates the importance of keeping RMSF in the differential diagnosis in patient populations outside of the usual geographic areas of incidence in the appropriate clinical setting.

Retrospective Study of Rocky Mountain Spotted Fever in Children.

BACKGROUND/OBJECTIVES: Rocky Mountain spotted fever (RMSF), a lethal tick-borne illness, is prevalent in the south central United States. Children younger than 10 years old have the greatest risk of fatal outcome from RMSF. The objective of the current study was to review pediatric cases of RMSF seen in the dermatology consult service and to evaluate dermatology's role in the diagnosis and management of this disease. METHODS: A retrospective review was performed of inpatient dermatology consultations at a tertiary care center in North Carolina from 2001 to 2011. Data collected included patient demographic characteristics, symptoms, pre- and postconsultation diagnoses, diagnostic procedures, length of hospital stay, and outcome. RESULTS: A total of 3,912 consultations were conducted in the dermatology service over 10 years. Six patients with RMSF, ranging in age from 22 months to 10 years (mean 5.1 years), were evaluated during April, May, and June. All preconsultation diagnoses included RMSF in the differential diagnosis. All patients underwent skin biopsies, and a culture was obtained in one case. Fifty percent of patients died within 4 days of hospitalization. CONCLUSIONS: Variables associated with mortality from RMSF are delayed diagnosis and initiation of antirickettsial therapy. Physicians should consider RMSF in children presenting with fever and rash during the summer months. Dermatology consultation is useful in evaluating patients with suspicious clinical features of RMSF with skin findings.

Diagnosis and Management of Tickborne Rickettsial Diseases: Rocky Mountain Spotted Fever and Other Spotted Fever Group Rickettsioses, Ehrlichioses, and Anaplasmosis - United States.

Tickborne rickettsial diseases continue to cause severe illness and death in otherwise healthy adults and children, despite the availability of low-cost, effective antibacterial therapy. Recognition early in the clinical course is critical because this is the period when antibacterial therapy is most effective. Early signs and symptoms of these illnesses are nonspecific or mimic other illnesses, which can make diagnosis challenging. Previously undescribed tickborne rickettsial diseases continue to be recognized, and since 2004, three additional agents have been described as causes of human disease in the United States: Rickettsia parkeri, Ehrlichia muris-like agent, and Rickettsia species 364D. This report updates the 2006 CDC recommendations on the diagnosis and management of tickborne rickettsial diseases in the United States and includes information on the practical aspects of epidemiology, clinical assessment, treatment, laboratory diagnosis, and prevention of tickborne rickettsial diseases. The CDC Rickettsial Zoonoses Branch, in consultation with external clinical and academic specialists and public health professionals, developed this report to assist health care providers and public health professionals to 1) recognize key epidemiologic features and clinical manifestations of tickborne rickettsial diseases, 2) recognize that doxycycline is the treatment of choice for suspected tickborne rickettsial diseases in adults and children, 3) understand that early empiric antibacterial therapy can prevent severe disease and death, 4) request the appropriate confirmatory diagnostic tests and understand their usefulness and limitations, and 5) report probable and confirmed cases of tickborne rickettsial diseases to public health authorities.

Tick-Borne Illnesses.

Tick-borne diseases are prevalent throughout the United States. These illnesses are caused by a variety of different pathogens that use ticks as vectors, including bacteria, viruses, rickettsia, and protozoa. Some of the most common illnesses caused by ticks are Lyme disease, Rocky Mountain spotted fever, babesiosis, ehrlichiosis, anaplasmosis, tularemia, Colorado tick fever, tick-borne relapsing fever, and Powassan disease. Unique skin changes, fever, and influenza-like symptoms may indicate tick-borne disease. Early diagnosis can be difficult as well as nonspecific and can resemble overtraining syndrome. Diagnosis is important to facilitate early treatment to decrease morbidity and mortality and should often be initiated before a definitive diagnosis is made. Treatment guidelines are published by the Centers for Disease Control and Prevention. As tick-borne diseases increase and their geographic regions expand, it is important for providers to distinguish the often overlapping and diverse presentations of these diseases.

Recognition of and Prompt Treatment for Tick-Borne Infections in Children.

Tick-borne infections create diagnostic challenges because they tend to present with nonspecific findings. Because clinicians often fail to recognize tick-borne illnesses in early stages, therapy is frequently delayed or omitted. This is especially problematic for rickettsial infections (Rocky Mountain spotted fever, ehrlichiosis, and anaplasmosis), because the risk of long-term morbidity and mortality increases with delayed treatment. We emphasize the need for clinicians to maintain a high index of suspicion for tick-borne infections; to diagnose these illnesses presumptively, without waiting for confirmatory laboratory test results; and to promptly start therapy with doxycycline, even in young children, when rickettsial infections are suspected.

Doxicilina injetável e o cloranfenicol suspensão para terapêutica da febre maculosa brasileira e outras riquetsioses / Doxycycline and chloramphenicol suspension for therapy of Brazilian spot fever and other rickettsial

A febre maculosa brasileira (FMB) é uma doença infecciosa febril aguda, de transmissão vetorial exclusiva, de gravidade variável, cuja apresentação clínica pode variar desde formas leves e atípicas até formas graves, com elevada taxa de letalidade, sendo por isso considerada um problema de saúde pública e doença de notificação compulsória no Brasil. Tendo em vista a limitação das técnicas laboratoriais atuais em possibilitar o diagnóstico específico imediato e a rápida progressão para formas graves da doença, a recomendação formal é de que frente a casos suspeitos da doença, que apresentem os critérios clínicos e laboratoriais compatíveis com os critérios de definição vigentes, a terapêutica específica (doxiciclina ou cloranfenicol), seja em âmbito ambulatorial ou hospitalar, deve ser prontamente instituída. O sucesso do tratamento está relacionado à precocidade e à especificidade de sua instalação. Os únicos fármacos com comprovada ação e eficácia são as tetraciclinas e o cloranfenicol. A escolha entre a tetraciclina (doxiciclina) e o cloranfenicol relaciona-se à gravidade da doença, devendo-se priorizar a tetracilina em função de sua superioridade. Considerando o alto potencial de letalidade da FMB e frente às evidências científicas sob a eficácia do uso da doxiciclina e do cloranfenicol, o Ministério da Saúde, em seu material instrucional (Guia de Vigilância Epidemiológica) da Secretaria de Vigilância em Saúde, recomenda estes medicamentos para o tratamento de pacientes com sinais e sintomas clínicos da febre maculosa brasileira. No entanto, não estão disponíveis a doxiciclina injetável e o cloranfenicol solução, por desinteresse comercial de laboratórios produtores e indisponibilidade de registro dos medicamentos no Brasil, o que motiva a aquisição destas drogas via \r\nimportação e disponibilização como medicamentos do componente estratégico do Ministério da Saúde para o tratamento da FMB e outras riquetsioses. Conforme avaliação realizada pela área técnica de vigilância epidemiológica da FMB e outras riquetsioses e Departamento de Assistência Farmacêutica do MS, estes \r\nmedicamentos poderão ser adquiridos de forma centralizada pelo Ministério da Saúde com recurso próprio e destinados às áreas endêmicas do país. Nesta avaliação, considerando as opções terapêuticas existentes no Brasil (doxiciclina comprimidos e cloranfenicol injetável), será necessária para atender a demanda anual a aquisição do quantitativo de 24.000 mil ampolas de doxiciclina injetável (100 mg) e 4.000 frascos de cloranfenicol solução (125 mg), o que promoverá um impacto orçamentário anual na ordem de US$ 126.000,00 mil dólares, estimativa anual baseada nas notificações de casos da FMB, que deverão ser tratados com os \r\nrespectivos medicamentos. Diante do exposto, os membros da CONITEC, presentes na reunião ordinária do \r\ndia 1º de agosto de 2013, decidiram, por unanimidade, pela incorporação da doxiciclina injetável e cloranfenicol solução para terapêutica antimicrobiana em pacientes com febre maculosa brasileira e outras riquetsioses.

Rocky Mountain spotted fever, Panama.

We describe a fatal pediatric case of Rocky Mountain spotted fever in Panama, the first, to our knowledge, since the 1950s. Diagnosis was established by immunohistochemistry, PCR, and isolation of Rickettsia rickettsii from postmortem tissues. Molecular typing demonstrated strong relatedness of the isolate to strains of R. rickettsii from Central and South America.

Dermatologic emergencies.

Primary care physicians are the gatekeepers of the medical community. They are the physicians to whom patients first present, and they are often the physicians with whom patients have the longest lasting relationships. Primary care physicians, as a result of these long-term relationships, have been endowed with a unique responsibility to the health of their patients. By the very nature of their practice, primary care physicians do not have the resources to treat emergent life-threatening conditions. They must, however, be able to diagnose these potentially life-threatening conditions and be able to stabilize and appropriately refer a patient for urgent evaluation by specialists or emergency physicians. There are many types of emergencies encountered in the outpatient setting, ranging from cardiac to toxicologic. As important as recognizing signs and symptoms of cardiac ischemia is the ability to recognize potentially life-threatening dermatologic disorders or dermatologic manifestations of life-threatening systemic diseases.

Rocky mountain spotted fever hospitalizations among American Indians.

To describe the epidemiology of Rocky Mountain spotted fever (RMSF) among American Indians/Alaska Natives (AI/ANs), we conducted a retrospective analysis of hospitalization records with an RMSF diagnosis using Indian Health Service (IHS) hospital discharge data for calendar years 1980-2003. A total of 261 RMSF hospitalizations were reported among AIs, for an average annual hospitalization rate of 1.21 per 100,000 persons; two deaths were reported (0.8%). Most hospitalizations (88.5%) occurred in the Southern Plains region, where the rate was 4.23 per 100,000 persons. Children 1-4 years of age had the highest age-specific hospitalization rate of 2.50 per 100,000 persons. The overall annual RMSF hospitalization rate declined during the study period. Understanding the epidemiology of RMSF among AI/ANs and educating IHS/tribal physicians on the diagnosis of tick-borne diseases remain important for the prompt treatment of RMSF and the reduction of the disease occurrence among AI/ANs, particularly in high-risk areas.

Diagnosis and management of tickborne rickettsial diseases: Rocky Mountain spotted fever, ehrlichioses, and anaplasmosis--United States: a practical guide for physicians and other health-care and public health professionals.

Tickborne rickettsial diseases (TBRD) continue to cause severe illness and death in otherwise healthy adults and children, despite the availability of low cost, effective antimicrobial therapy. The greatest challenge to clinicians is the difficult diagnostic dilemma posed by these infections early in their clinical course, when antibiotic therapy is most effective. Early signs and symptoms of these illnesses are notoriously nonspecific or mimic benign viral illnesses, making diagnosis difficult. In October 2004, CDC's Viral and Rickettsial Zoonoses Branch, in consultation with 11 clinical and academic specialists of Rocky Mountain spotted fever, human granulocytotropic anaplasmosis, and human monocytotropic ehrlichiosis, developed guidelines to address the need for a consolidated source for the diagnosis and management of TBRD. The preparers focused on the practical aspects of epidemiology, clinical assessment, treatment, and laboratory diagnosis of TBRD. This report will assist clinicians and other health-care and public health professionals to 1) recognize epidemiologic features and clinical manifestations of TBRD, 2) develop a differential diagnosis that includes and ranks TBRD, 3) understand that the recommendations for doxycycline are the treatment of choice for both adults and children, 4) understand that early empiric antibiotic therapy can prevent severe morbidity and death, and 5) report suspect or confirmed cases of TBRD to local public health authorities to assist them with control measures and public health education efforts.

Tick-borne infections in children: epidemiology, clinical manifestations, and optimal management strategies.

Ticks can transmit bacterial, protozoal, and viral infections to humans. Specific therapy is available for several of these infections. Doxycycline is the antimicrobial treatment of choice for all patients, regardless of age, with Rocky Mountain spotted fever, human monocytic ehrlichiosis, or human granulocytic ehrlichiosis. Chloramphenicol has been used to treat these infections in children but is demonstrably inferior to doxycycline. In patients with Mediterranean spotted fever, doxycycline, chloramphenicol, and newer macrolides all appear to be effective therapies. Therapy of Lyme disease depends on the age of the child and stage of the disease. For early localized disease, amoxicillin (for those aged <8 years) or doxycycline (for those aged >/=8 years) is effective. Doxycycline, penicillin V (phenoxymethylpenicillin) or penicillin G (benzylpenicillin) preparations, and erythromycin are all effective treatments for tick-borne relapsing fever. Hospitalized patients with tularemia should receive gentamicin or streptomycin. Doxycycline and ciprofloxacin have each been investigated for the treatment of tularemia in outpatients; however, these agents do not yet have established roles in the treatment of this disease in children. Combination therapy with clindamycin and quinine is preferred for children with babesiosis; the combination of azithromycin and atovaquone also appears promising. Ribavirin has been recently shown to markedly improve survival in patients with Crimean-Congo hemorrhagic fever. The role of antiviral therapy in the treatment of other tick-borne viral infections, including other hemorrhagic fevers and tick-borne encephalitis, is not yet defined.

Life-threatening eruptions due to infectious agents.

Some infectious diseases may cause rapidly fatal eruptions that need to be diagnosed and treated in an early phase for patient survival. The main life-threatening eruptions of infectious etiology include Rocky Mountain spotted fever, meningococcemia, toxic shock syndrome, streptococcal toxic shock syndrome, and staphylococcal scalded skin syndrome.

Deadly viral syndrome mimics.

Upper respiratory tract infections (ie, "the common cold") have several hundred causes, the most common of which include rhino-virus, coronavirus, and respiratory syncytial virus. The clinical presentation varies with symptoms. Every emergency department, no matter what the demographics, cares for patients with this constellation of symptoms. Emergency physicians examine, diagnose, and treat these disorders frequently. With increasing burdens being placed on emergency physicians, it is possible to assume a diagnosis of upper respiratory tract infection without generating a complete differential diagnosis. The challenge is to identify and recognize the distinctions between an innocuous upper respiratory tract infection and a life-threatening disease "mimic" or entities. This article discusses some of these life-threatening mimics.

Rocky Mountain spotted fever at Koair Children's Hospital, 1990-2002.

The reported average annual incidence of Rocky Mountain spotted fever (RMSF) in Kentucky is less than 5 per million population, although seroprevalence studies suggest that exposure to Rickettsia riskettsii, the causative agent, is relatively common among children. The experience with RMSF at Kosair Children's Hospital over a 12-year period was reviewed. Fifteen cases were identified (5 boys and 10 girls). Illness onset ranged from April to October, and 4 patients resided in Jefferson County. The classic triad of fever, rash, and headache was present in only 60% of cases, and tick attachment was reported in only 40%. On average, 6 days elapsed from onset of symptoms to initiation of appropriate antibiotic therapy. One patient suffered splenic infarction and necrosis of the digits due to shock and disseminated intravascular coagulopathy, and 2 patients died. RMSF is a significant cause of pediatric morbidity and mortality in this region of Kentucky. Affected children may reside in relatively urban parts of the state. Initial clinical features may be nonspecific. This, as well as decreased awareness of disease and (unjustified) reluctance to use doxycycline may contribute to delays in initiating therapy.

An update on Lyme disease and other tick-borne illnesses.

Lyme disease, the most common vector-borne illness in North America, is a multisystem, multistage infectious disease caused by the tick-transmitted spirochete Borrelia burgdorferi. Although Lyme disease is not fatal, it can cause musculoskeletal, neurological, and cardiovascular manifestations that may be difficult to treat. Clinicians must also be aware of other potentially fatal tick-borne diseases such as babesiosis, ehrlichiosis, and Rocky Mountain spotted fever. Early identification and treatment of tick-borne diseases are crucial to preventing devastating sequelae.