Autoimmune hepatitis presenting as severe anemia.

Autoimmune hepatitis (AIH) is relatively rare in children. Herein, our case demonstrates a unique presentation of AIH in a previously healthy 18-year-old female presenting with a mild cough, fatigue, and severe anemia (hemoglobin 2.9 g/dL). Initial evaluation revealed jaundice and scleral icterus, prompting transfer of care and further testing, which demonstrated severe microcytic anemia, pancytopenia, elevated liver enzymes, direct hyperbilirubinemia, and marked splenomegaly. Concern for autoimmune hemolytic anemia resulted in a delayed diagnosis. The combination of triple antibody positivity (anti-nuclear antibodies, anti-actin, and anti-liver-kidney microsomal-1) and liver histology findings confirmed the diagnosis of AIH. Intravenous methylprednisolone was initiated to induce remission. Due to pancytopenia and persistently elevated international normalized ratio, tacrolimus was chosen as the maintenance immunosuppression instead of azathioprine. This case highlights several significant considerations for clinicians, including the importance of a timely clinicopathologic diagnosis, the severe anemia presentation secondary to hypersplenism, and the rare finding of triple autoantibody-positive AIH.

Severe Thrombocytopenia in Decompensated Liver Disease: An Example of Accelerated Intravascular Coagulation and Fibrinolysis.

INTRODUCTION: Advanced liver disease can present with severe thrombocytopenia that can be difficult to delineate and manage. Here we describe a unique entity of accelerated intravascular coagulation and fibrinolysis (AICF) in a patient with decompensated liver disease. CASE PRESENTATION: A 56-year-old male with a history of alcoholic cirrhosis was admitted for weakness, nausea, metabolic derangement, and acute kidney injury determined to be secondary to decompensated liver disease. During admission, his platelet count declined to <10 000/µL requiring 8 total platelet transfusions. Laboratory and clinical evaluation supported a diagnosis of AICF, and the patient gradually improved with supportive management. DISCUSSION: AICF can present similarly to disseminated intravascular coagulation, and careful evaluation of specific laboratory values is required for accurate diagnosis. Appropriate management minimizes the associated increased risk of bleeding and prevents delay in procedural intervention. CONCLUSIONS: This case highlights the importance of early clinical and laboratory correlation, multidisciplinary care, and supportive treatment in the management of AICF.

Topical Treatment of Cutaneous Squamous Cell Carcinoma in Situ and the Impact of Clinical Risk Factors and Positive Histologic Margins at the Time of Diagnosis.

BACKGROUND: Limited data exist for the efficacy of topical 5-fluorouracil (5-FU) and imiquimod for cutaneous squamous cell carcinoma (cSCC) in situ (cSCCis) with positive histologic margins at the time of diagnosis. OBJECTIVE: Identify the efficacy of topical 5-FU and imiquimod in the treatment of cSCCis with positive histologic margins at the time of diagnosis in relation to clinical risk factors. MATERIALS AND METHODS: Pathology records were screened at a single institution from 2014 to 2021 for cSCCis with positive histologic margins. Patients were included if they were treated with curative intent with topical 5-FU or imiquimod. Recurrences were evaluated in relation to multiple clinical risk factors. RESULTS: Of 215 patients treated with 5-FU or imiquimod after biopsy-proven cSCCis, 19 patients had recurrent cSCCis and 1 patient had upstaging to invasive cSCC. Recurrence was more likely in larger lesions at the time of initial biopsy ( p = .033) and in patients treated with topical imiquimod compared with topical 5-FU ( p < .01). CONCLUSION: Topical 5-FU is an appropriate therapy for cSCCis in the correct clinical scenario. Extra consideration should be taken for use of 5-FU in larger diameter cSCCis lesions. Although limited by sample size, our study does not support the use of imiquimod for cSCCis.

Respiratory viral infections are prevalent but uncomplicated in single ventricle CHD.

BACKGROUND: Patients with single ventricle CHD have significant morbidity and healthcare utilisation throughout their lifetime, including non-cardiac hospital admissions. Respiratory viral infections are the main cause of hospitalisation in children, but few data exist for single ventricle patients. We sought to identify how respiratory viral infections impact patients with single ventricle CHD and potential differences between Glenn and Fontan circulation. METHODS: We conducted a retrospective study of patients seen from 01/01/2011-12/31/2020. We identified patients with a history of single ventricle CHD and Glenn palliation, and a normoxic control group with isolated atrial septal defect requiring surgical closure. We compared viral-related clinical presentations, admissions, and admission characteristics. RESULTS: A total of 312 patients were included (182 single ventricle, 130 atrial septal defect). Single ventricle patients were more likely than children with isolated atrial septal defect to be admitted with a respiratory virus (odds ratio 4.15 [2.30-7.46]), but there was no difference in mechanical ventilation or hospital length of stay (p = 0.4709). Single ventricle patients with Glenn circulation were more likely than those with Fontan circulation to present and be admitted (odds ratio 3.25 [1.62-6.52]), but there was no difference in ICU admission, mechanical ventilation, or hospital length of stay (p = 0.1516). CONCLUSIONS: Respiratory viral infections are prevalent but uncomplicated in patients with single ventricle CHD. Viral-related presentations and admissions are more prevalent during the period of Glenn circulation compared to Fontan circulation; however, rate of mechanical ventilation and hospital length of stay are similar.