Dyskeratosis congenita (DC) is a rare hereditary disorder characterized by bone marrow failure, cancer predisposition (11-fold increase compared to general population), ectodermal dysplasia (nail dystrophy, oral leukoplakia, and abnormal skin pigmentation) and other additional somatic abnormalities. A 22-year-old man presented with fever, chills, and a painful throat. Leukoplakia was noted on his tongue and some of his fingers and toe nails were markedly dystrophic. His skin seemed spotted with pigmentation on the anterior chest and neck. Patient reported family history of “blood disease” and leukemia. He was admitted for the management of neutropenic fever (102.9 °F, WBC: 940, ANC: 404, platelets: 21,000 and Hb: 9.2), and was put on broad spectrum antibiotics. A bone marrow biopsy revealed normocellular marrow with erythroid predominance and mild dyserythropoiesis. Chromosomal analysis indicated no numerical or structural chromosomal abnormalities. The fluorescence in situ hybridization report did not reveal any assay specific abnormalities. Viral marker for hepatitis and studies of autoimmune antibodies showed negative results. CT scan had shown splenomegaly. Patient was discharged after he has been fever and symptoms free, with a follow-up at hematology clinic. Individuals with DC most commonly present with skin pigmentation, dystrophic nails and leukoplakia, as presented in this case. Genetic abnormality was not confirmed in our patient, but it is suggested that X-linked recessive pattern would be significant because of greater prevalence in men than in women (10:1). The distribution of blood counts and bone marrow biopsy categorizes our patient in the moderate aplastic anemia class which is the most prevalent pattern. When fever in young patients with a failing bone marrow is diagnosed, it is important that physicians rule out the possible underlying causes. DC is a rare disease, but can be diagnosed by simple inspection of the mucocutaneous abnormalities. DC is a severe multisystem disorder associated with premature morbidity and mortality. We believe that reporting this case would add more information to the existing fund of knowledge.
Acremonium is a saprophytic fungus mostly causing superficial skin, nail, or ocular infections after traumatic inoculation. However, it is being recently recognized as one of the opportunistic infections in immunocompromised patients including neutropenia, malignancies, chronic granulomatous disease (CGD) and transplant recipients. To our knowledge there have been no reported cases of Acremonium infection, related to HIV or AIDS. We present a case of Acremonium pneumonia in a patient with no past medical history who was found to have AIDS.
S. pyogenes is the cause of many important human diseases, ranging from mild superficial skin infections to life-threatening systemic diseases. The post streptococcal syndromes are immune mediated phenomena including Immunoglobulin A Vasculitis (Henoch-Schönlein purpura).HSP is more common in children and usually self limited but it can cause skin, joint, renal, gastrointestinal and rarely respiratory involvement. We present a case with Streptococcus pyogenes pneumonia that presented with respiratory failure, pulmonary hemorrhage, extensive rash and renal failure.
Aerococcus urinae, a previously misidentified pathogen, has become increasingly recognized to cause severe and even fatal infections. Aerococcus-related perineal abscess infections have not previously been reported in the literature. Most reported cases of infections caused by Aerococcus are urinary tract infections, bacteremia, and even rare cases of endocarditis. We report an unusual case of a perineal abscess caused by Aerococcus urinae.
Background The spleen removes microorganisms from the bloodstream and produces antibodies for enhanced immune response. Patients with asplenia are at increased risk for infections caused by encapsulated organisms and have a 6-fold increased risk of sepsis compared to the general population. Those that acquire an infection have a mortality rate of 80%. The CDC recommends administering the meningococcal, influenza, pneumococcal, and Haemophilus influenzae type B (Hib) vaccine at least 2 weeks before or after splenectomy. Methods This retrospective chart review evaluated the appropriateness of vaccine administration for patients undergoing splenectomy at Long Island Jewish Medical Center (LIJMC) from January 2016 to June 2020. Patients were included if they were admitted to LIJMC, 18 years or older, and had splenectomy at LIJMC within previous 7 years. Patients were excluded if they were pregnant, admitted to an outside hospital for splenectomy, or had an unknown time of splenectomy. The primary objective was appropriateness of vaccine administration. Inappropriateness was defined as having at least 1 error with administration, including vaccine omissions, timing of vaccine, sequencing (Pneumovax®23 given before Prevnar13®), vaccine interaction (Menactra® given with Prevnar13®), or duplicated vaccines. Results Of the 174 patients screened, 29 patients were included in analysis, with a mean age of 58.2 ± 20.6 years. The splenectomy was elective in 69% of patients and emergent in 31% of patients. The vaccine regimen was given inappropriately in 96.6% of patients. The reasons for inappropriateness were vaccine selection (100%), timing (39.3%), vaccine sequence (39.3%), vaccine interaction (14.3%), and duplicate vaccines (7.1%). Excluding emergent splenectomies, 100% of patients received an inappropriate vaccine regimen. The vaccines were given at least 2 weeks before elective splenectomies in 40% of patients, peri-operatively in 30% of patients, and not given in 25% of patients. Conclusion Patients undergoing splenectomy received inappropriate vaccine regimens mainly due to omissions, timing, and sequencing. Prevnar13® should be given before Pneumovax®23 when both are indicated and vaccines should be administered at least 2 weeks before splenectomy. An orderset was created to mitigate errors with vaccine selection for patients undergoing splenectomy. Disclosures All Authors: No reported disclosures.
Introduction We present a real-world experience of a U.S. Navy Hospital Ship deployed amid a global Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) surge and the challenges of navigating policy while maintaining a mission-focused itinerary in an operational environment. Materials and Methods We performed a chart review of SARS-CoV-2 cases from April 18 to September 20, 2022, within a closed population of fully vaccinated adults onboard the USNS Mercy (T-AH 19) during the 5-month 2022 Pacific Partnership mission to Guam, Vietnam, Palau, Philippines, and the Solomon Islands. Results There were 123 total SARS-CoV-2 cases over the course of the mission, constituting 16.6% of the total crew (123/741). No more than 14 service members were actively infected at a given time (1.9%, 14/741). The average number of active cases at any given time was 0.8 (1.9 SD, 0.1% [0.8/741]), and just 14 of these were shipboard secondary cases. No significant operational requirements of the ship were impacted by infection-related manning shortages, there were no hospitalizations, and all infected members experienced full recovery. Conclusions Despite ongoing cases throughout the majority of the mission, a healthy immunized crew experienced no serious cases and minimal impact on operational effectiveness.
BackgroundLatent tuberculosis infection (LTBI) treatment is essential in preventing the reactivation of tuberculosis. We compared the clinical and demographic characteristics of patients that have completed traditional therapy with 9 months of isoniazid (9H) with those that have completed 3 months of rifapentine plus isoniazid using directly observed therapy (3HP), focusing on adverse effects, a barrier to completion that may contribute to discontinuation of therapy.MethodsWe conducted a retrospective chart review (July 2013-March 2017) to compare the 9H group and 3HP group. Demographic and clinical variables were described by therapy type and groups were compared using Fisher’s exact test or t-test, as appropriate.ResultsPatients in the study sample (n = 124) had a mean age of 49.8 (SD=14.8) years old. Approximately half received 3HP (n = 64, 51.6%). Demographics in the 3HP and 9H groups were similar. Significantly more patients in the 3HP group completed treatment (81.3% vs. 61.7%, P < 0.0001). No patients were lost to follow-up in the 3HP group, 14 (23.33%) were lost in the 9H group. Gastrointestinal (GI) upset (n = 16), elevated liver function tests (LFTs) (n = 11), and headaches (n = 9) were the most frequent side effects. Except for neuropathy and pancreatitis, all other adverse side effects had higher incidence in the 3HP group. Specifically, the incidence of GI symptoms (23.4% vs. 1.7%, P = 0.0003), weakness (9.4% vs. 0%, P = 0.028), and headache (14.1% vs. 0%, P = 0.003) were significantly higher in the 3HP group. Of the observed patients with adverse reactions that received 3HP, 88.24% (n = 30) had them resolved within the first two weeks.ConclusionThe 3HP group had a higher completion rate and no loss to follow-up compared with 23% loss to follow-up in the 9H group, however, adverse reactions were significantly higher in the 3HP group. Closer weekly monitoring of the 3HP group could lend itself to capturing more adverse reactions, however, 88% of those adverse reactions resolved within the first two weeks of therapy. Liver function tests were not significantly different (P = 0.2079) between the two groups, and were mildly elevated. We conclude that three months of rifapentine plus isoniazid for the treatment of LTBI may be a favorable option over the traditional 9 months of isoniazid in certain populations.Disclosures All authors: No reported disclosures.
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