Infant with a hereditary blistering disorder: an interesting case in the NICU.

This is a case of hereditary skin disorder in a full-term female newborn, with family history of epidermolysis bullosa (EB), who developed skin vesicles on the first day of life (DOL) without mucosal or ocular involvement. A multidisciplinary approach involving dermatology, wound care, and occupational therapy led to full recovery in our patient within six days of life. Special precautions were taken to prevent complications. Upon genetic testing, the patient was found to have a genetic variant of unknown significance (VUS). The goal of this case report is to give a detailed account of the patient's course, provide management recommendations which could be applied to similar cases and settings in the newborn period.

Acute Presentation of Tuberculosis Empyema in a Healthy Adolescent.

BACKGROUND Tuberculosis (TB) was the leading cause of infectious death worldwide until the COVID-19 pandemic, which reduced case reporting and disrupted TB diagnosis and services. While Mycobacterium tuberculosis remains a leading cause of morbidity and mortality globally, the disease burden within developed nations remains relatively rare. Although the many complications of TB are well known, no current data exists on those infected with TB who subsequently developed recurrent TB empyema, as it is such a rare complication, especially in pediatric and adolescent populations. CASE REPORT A previously healthy 15-year-old male patient presented with 5-day duration of cough, congestion, intermittent fever, and post-tussive emesis. Although born in the United States, 3 months before presentation, he returned from Senegal, where he had lived for 4 years. Imaging demonstrated consolidation with loculated effusion. Patient underwent video-assisted thoracoscopy and chest tube placement, draining 750 mL of purulent fluid testing positive for rare acid-fast bacilli. Rifampin, isoniazid, pyrazinamide, and ethambutol were administered, with discharge medication compliance ensured by daily videos surveillance through the Department of Health. Although compliant with medications, patient presented to the Emergency Department 2 months later with a multi-loculated fluid recollection and fistula formation requiring chest tube placement. After this discharge, patient experienced resolution of disease following completion of therapy. CONCLUSIONS TB complication should be considered as a differential diagnosis for pleural effusion in the appropriate clinical setting. Providers should not only consider the diagnosis but pursue appropriate testing and management early, particularly in those with risk factors, including travel to an endemic location.

Case Series of Clinical Findings of Multi-System Inflammatory Syndrome in Children in Contrast to Kawasaki Disease.

Several months into the coronavirus disease 2019 (COVID-19) pandemic, there is growing concern over an increase in the incidence of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2)-linked Kawasaki-like disease in the pediatric population. The pediatric patients presented to the emergency room with impending shock in the setting of an atypical Kawasaki picture. On May 14, 2020, the CDC Health Alert Network released a case definition for this evolving syndrome and named it multi-system inflammatory syndrome in children (MIS-C). We report three cases of MIS-C associated with SAR-COV2 who presented to our emergency room. Persistent fever was present in all three patients and mucocutaneous and gastrointestinal symptoms were the most common associations. All three patients were found to have antibodies to COVID-19. MIS-C is a similar but distinct entity as compared to Kawasaki disease. High inflammatory markers are supportive of the diagnosis, and cardiac evaluation is crucial in MIS-C. High suspicion for the diagnosis and low threshold for workup will prevent delayed treatment.

Complicated Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS) Syndrome History in a 14-Year-Old.

BACKGROUND Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is a drug-induced hypersensitivity reaction that can result in a severe cutaneous adverse drug reaction (SCAR). It is a rare and potentially life-threatening condition that occurs after exposure to sulfonamides, antibiotics, or antiepileptics. Its incidence in children is not established; however, the mortality rate is documented at approximately 10%. DRESS syndrome is believed to result from an interaction between multiple factors, including genetics, abnormalities of metabolism, and reactivation of certain herpes family viruses including EBV and HHV-6. The classic presentation includes fever, rash, and lymphadenopathy. Symptoms begin approximately 3 to 8 weeks after exposure to the offending agent. CASE REPORT We present a unique case of DRESS syndrome in a 14-year-old girl occurring after the ingestion of minocycline and amoxicillin-clavulanic acid (amoxicillin). Identification of the offending agent was complicated by the patient having been on multiple antibiotics within a short timeframe of the initial presentation of symptoms. In addition to swelling and pruritus, the patient experienced vision problems due to papilledema with bilateral hemorrhage. The treatment course was further complicated by a decrease in kidney function, requiring the patient's medication regimen to be adjusted accordingly. CONCLUSIONS This is a unique case of DRESS syndrome demonstrating the potential influence of certain viruses on the severity of its presentation. This case also highlights the need to adjust the steroid regimen to reduce the potentially harmful effects on various organ systems.

Case Report: Invasive Non Type b Haemophilus influenzae in Immunocompromised Children.

BACKGROUND Implementation of the Haemophilus influenzae type b (Hib) conjugate vaccine brought about a reduction in the number of cases and morbidity from type B but increase in nontypeable strain infections. CASE REPORT We had 3 cases of invasive non type Hemophilus influenzae (NTBHI) in immunocompromised children. The first was a fully vaccinated 2-year-old male with a history of pseudomonas sepsis who presented with 1 day of lethargy, fever, vomiting, and diarrhea. Blood culture was positive for Haemophilus influenzae e and cerebrospinal fluid (CSF) confirmed meningitis. Immune deficiency and genetic testing revealed X-linked agammaglobulinemia. The second case was a 4-year-old male, status post liver transplantation, who presented with pneumonia, with positive blood culture for H. influenzae. The last case was of a 2-year-old male with H. influenzae biotype VI in both blood and CSF cultures, who on follow-up was confirmed to have hypogammaglobulinemia. CONCLUSIONS For children diagnosed with an invasive disease caused by NTBHI, a workup for immunodeficiency could be warranted. With the appearance of nontype b serotypes, more studies are needed to determine epidemiology and virulence of these types, and their clinical relevance - perhaps developing a new vaccine to cover nontype b stereotypes, especially for immunodeficient patients.

The Association Between Alcohol Use and the Progression of Alzheimer's Disease.

OBJECTIVE: To examine the relationship between alcohol, both the amount and type, and cognitive decline in a cohort of Alzheimer's disease (AD) patients. METHODS: A cohort of 360 patients with early AD in New York, Boston, Baltimore and Paris were followed-up biannually for up to 19.28 years. At each visit, the cognitive profile of the patients was assessed using the modified Mini-Mental State Examination (mMMSE), and patients' alcohol intake, including beverage type, was reported by patients' primary caregivers. General estimating equation analysis was used to determine whether baseline alcohol use was associated with the rate of cognitive decline. RESULTS: Heavy drinkers (8 or more alcoholic drinks/week) had a faster cognitive decline, deteriorating 1.849 more points on their mMMSE score annually compared to abstainers (P = 0.001), or 2.444 more points compared to mild-moderate drinkers (1-7 alcoholic drinks/week) (P = 0.008). There was no significant difference when comparing mild-moderate drinkers to abstainers. Increasing standard drinks of hard liquor, but not beer or wine, was also associated with a faster rate of cognitive decline (ß = -0.117 P = 0.001). CONCLUSION: Heavy alcohol consumption and more hard liquor are associated with a faster rate of cognitive decline in AD patients, suggesting that they may hasten progression of AD. Our results suggest that alcohol drinking habits might alter the course of AD.