Diagnostic Dilemma in a Case of Necrotizing Lymphadenitis With Macrophage Activation Syndrome.

Necrotizing lymphadenitis is a histological diagnosis that can arise from various conditions, including lupus lymphadenitis (LL), Kikuchi disease (KD), and infectious causes. Distinguishing between Kikuchi disease and lupus lymphadenitis can be challenging in clinical practice. In this report, we present the clinical scenario of a young female patient with lymphadenopathy and elucidate the process through which we ultimately arrived at a diagnosis of systemic lupus erythematosus (SLE) with macrophage activation syndrome. This case underscores the significance of recognizing Kikuchi disease as a condition that can mimic lupus and sheds light on the distinguishing features of necrotizing lymphadenitis, with a particular focus on Kikuchi disease and lupus lymphadenitis.

Typical evanescent and atypical persistent polymorphic cutaneous rash in an adult Brazilian with Still's disease: a case report and review of the literature.

Adult onset Still's disease (AOSD) is a systemic auto-inflammatory condition of unknown etiology, characterized by high fever, an evanescent, salmon-pink maculopapular skin rash, arthralgia or arthritis and leukocytosis. AOSD can also present with atypical cutaneous manifestations, such as persistent pruritic coalescent papules or plaques and linear lesions that have highly distinctive pathological features and are usually associated with severe disease. Herein, we present a 31-year-old Brazilian man with both typical Still's rash and atypical persistent polymorphic cutaneous manifestations associated with severe systemic inflammatory response syndrome. Eosinophils that are consistently lacking in the AOSD-associated skin lesions were evident in the skin biopsy of the persistent atypical cutaneous manifestations and were either drug-related or AOSD-associated.

Acute porphyria in a patient with Arnold Chiari malformation.

BACKGROUND: Acute porphyria and Arnold Chiari malformation are both uncommon genetic disorders without known association. The insidious onset, non-specific clinical manifestations, and precipitating factors often cause diagnosis of acute porphyria to be missed, particularly in patients with comorbidities. CASE REPORT: A women with Arnold Chiari malformation type II who was treated with oxybutynin and antibiotics, including Bactrim for neurogenic bladder and recurrent urinary tract infection, presented with non-specific abdominal pain, constipation, and diarrhea. After receiving Flagyl for C. difficile colitis, the patient developed psychosis, ascending paralysis, and metabolic derangements. She underwent extensive neurological workup due to her congenital neurological abnormalities, most of which were unremarkable. As a differential diagnosis of Guillain Barré syndrome, acute porphyria was then considered and ultimately proved to be the diagnosis. After hematin administration and intense rehabilitation, the patient slowly recovered from the full-blown acute porphyria attack. CONCLUSIONS: This case report, for the first time, documents acute porphyria attack as a result of a sequential combination of 3 common medications. This is the first case report of the concomitant presence of both acute porphyria and Arnold Chiari malformation, 2 genetic disorders with unclear association.

If asked, hospitalized patients will choose whether to receive life-sustaining therapies.

BACKGROUND: No national policy requires health care providers to discuss with hospitalized patients whether the latter would want cardiopulmonary resuscitation (CPR) or mechanical ventilation (MV) in the event of cardiopulmonary failure. OBJECTIVE: To determine whether hospitalized patients are willing to discuss end-of-life issues and choose whether to receive CPR and MV. DESIGN: Prospective randomized trial. PARTICIPANTS: 297 patients admitted to the medicine service of a 350-bed community teaching hospital. INTERVENTION: Patients were randomized to receive routine care or a scripted intervention, delivered by research physicians, that included detailed information about CPR, MV, and advance directives. MEASUREMENTS: Number of patients who welcomed the scripted intervention, number who chose to receive or reject CPR/MV, and number of advance directives created during hospitalization. RESULTS: Of the 297 patients studied, 136 were in the intervention group and 161 were in the control group. Baseline characteristics and severity of illness were similar in the 2 groups. Of the 136 patients in the intervention group, 133 (98%) willingly discussed CPR and mechanical ventilation, and 112 (82%) found the information useful. One hundred and twenty-five (92%) clarified their preferences regarding CPR and MV after receiving the intervention; of the 48 patients who were initially documented as wanting CPR/MV, 3 requested no CPR/MV after the intervention. Of the 87 patients in the intervention group who had no documentation of code status on admission, 5 asked for no CPR/MV. Of the 161 patients in the control group, 55 had documentation of their code status on admission. Of the 106 patients without documentation, 6 were later documented to receive no CPR/MV. Thirteen of the 102 patients who had no advance directive on admission created one after the intervention, whereas only 1 of the 128 patients in the control group did so (P < .001). CONCLUSIONS: Patients are willing to discuss and give informed consent for CPR and mechanical ventilation early in hospitalization. Only a minority drafted advance directives during hospitalization. Larger studies that include patients at other centers are required to determine whether these findings are reproducible and whether this approach is clinically feasible.

Informed consent for invasive procedures in a community hospital medical intensive care unit.

OBJECTIVE: To describe the informed consent practices in the medical intensive care unit (MICU) of a university-affiliated, community teaching hospital. DESIGN: Prospective, observational study. SUBJECTS: 177 critically ill patients. MEASUREMENTS: Study personnel identified all critically ill patients who had an invasive medical procedure during the study period (120 days). It was first determined whether written informed consent was obtained for the procedure. If it was, standardized questionnaires were used to determine whether consent-givers recalled the indications, complications, and alternatives of invasive medical procedures. Documentation of consent and of the performance of invasive procedures in the medical records was also examined. An educational in-service was created to help improve the informed consent process. After a 45-day lead-in (control) period, the in-service was administered and IC was again studied for 75 days. RESULTS: A total of 181 procedures were performed on 112 patients over 120 days in the MICU. The rates of written consent for invasive procedures averaged 89%; rates of consent were not affected by the educational in-service. Following administration of the in-service, consent-givers recalled a greater number of complications (2.5 to 4.1, P=0.01) and documentation of consent improved. CONCLUSIONS: These results suggest that informed consent can be obtained procedure-by-procedure, as needed, at a high frequency, and with reasonable consenter comprehension.

Informed consent for medical procedures: local and national practices.

BACKGROUND: No studies have assessed whether clinicians obtain informed consent for invasive medical procedures, and there are no explicit national standards to guide the process. HYPOTHESIS: Informed consent practices are inconsistent for commonly performed invasive medical procedures. METHODS: A simple questionnaire was electronically mailed and/or faxed to training program directors of critical care medicine and internal medicine departments, and to ICU directors in the state of Connecticut. The questionnaire listed common invasive medical procedures and asked the respondents to check those for which practitioners routinely obtain informed consent in their hospital. RESULTS: The three samples, national intensivists (88 respondents), Connecticut intensivists (29 respondents), and national internists (56 respondents) demonstrated heterogeneity of consenting practices. The rate of obtaining consent for common vascular access procedures ranged from 20 to 90%. The rates of obtaining consent for Foley catheterization and nasogastric intubation were uniformly < 10%, and those for endoscopic procedures were > 90%. Separate consent (beyond the general consent to treat) was not uniformly obtained for the transfusion of blood products (range, 74 to 93%) and common diagnostic medical procedures (eg, thoracentesis, paracentesis, or lumbar puncture; range, 77 to 96%). Surgical intensivists reported that the obtaining of consent for invasive procedures was less routine compared to medical intensivists. Lower rates of consent were noted by those respondents who used a "blanket" consent form, which was signed at the time of hospital admission, to cover subsequent procedures. CONCLUSIONS: In this relatively small sample, there was no uniform practice of informed consent for commonly performed invasive medical procedures. Consent was routinely obtained for GI endoscopy, bronchoscopy, and medical research, and was not obtained for Foley catheterization and nasogastric intubation. The obtaining of consent for vascular cannulation and diagnostic procedures was not routine in the ICUs of a substantial number of respondents. Explicit standards that delineate specifically which procedures require consent may be required to assure more uniform practices.