Case Report: Hemophagocytic Lymphocytosis in a Patient With Glutaric Aciduria Type IIC

Hemophagocytic lymphocytosis (HLH) is a rare disease caused by inborn errors of immunity (IEI), secondary to infection, lymphoma or autoimmune disorders, but we often overlook the fact that HLH can be secondary to inborn errors of metabolism (IEM). Here, we describe a patient who was diagnosed with glutaric aciduria type IIC complicated by features suggestive of possible HLH. The diagnosis of glutaric aciduria type IIC, a IEM, was confirmed by whole exome sequencing. The patient was treated with coenzyme Q10 and riboflavin which effectively improved her liver function. During treatment, the patient developed severe anemia and thrombocytopenia. Persistent fever, splenomegaly, cytopenias, increased ferritin, hypertriglyceridemia, hypofibrinogenemia, and hemophagocytosis in the bone marrow pointed to the diagnosis of HLH; however, the patient eventually died of gastrointestinal bleeding. After other potential causes were ruled out, the patient was diagnosed with glutaric aciduria type IIC complicated by features suggestive of possible HLH. When cytopenias occurs in IEM patients, HLH is a possible complication that cannot be ignored. This case suggests a possible relationship between IEM and risk for immune dysregulation.

Bartonellosi e D-dimero

The paper reports the case of a 17-year-old immunocompetent boy with persistent fever and elevated D-dimer. The family referred contact with kitten and ingestion of homemade deer salami. Abdominal ultrasound scan and CT showed multiple hepatic and splenic abscesses. High levels of antibody title for Bartonella henselae confirmed the diagnosis of hepatosplenic bartonellosis.

Bacterial shedding and serologic responses following an outbreak of Salmonella Typhi in an endemic cohort

Background: Salmonella enterica serovar Typhi (S. Typhi), the causative agent of Typhoid fever, is transmitted faecal-orally. Some typhoid sufferers shed S. Typhi beyond convalescence, but culturing stool following every case is impractical. Here we hypothesised that serology might direct testing and identify shedding after a typhoid outbreak. Methodology/Principle Findings: In 2016 there was a typhoid outbreak in a Nursing School in Malosa, Malawi. We collected serum three and six-months post-outbreak. We measured IgG antibody titres against Vi capsular polysaccharide (anti-Vi IgG) and IgM / IgG antibodies against H:d flagellin (anti-H:d). We screened faecal samples from participants with high and low anti-Vi IgG (measured at visit one) by culture and PCR. Participants reported whether they had persistent fever for ≥ three days (in keeping with World Health Organization definitions for typhoid) during the outbreak. We tested for environmental S. Typhi. 368 people provided serum at 3-months, of whom 320 provided serum at 6-months; 49 participants provided a faecal sample (25 from the highest and 24 from the lowest deciles for anti-Vi IgG titre). We did not grow S. Typhi from faeces, but one sample produced a positive PCR amplification for S. Typhi. Median anti-Vi IgG titre fell amongst participants with persistent fever (8.08 to 3.7 EU/ml, <0.000001, Wilcoxon signed rank). Median anti-H:d IgG titres fell in those with and without persistent fever (87.8 to 77.4 EU/, p = <0.000001 and 82.4 to 79.2 EU/ml, p = 0.0002, Wilcoxon signed rank, respectively). Anti-H:d IgM titres did not change significantly. Non-Typhoidal Salmonellae were identified in water sampled at source and a kitchen tap. Conclusions / Significance: We did not identify culture-confirmed shedding through sero-surveillance. Serologic trends signify a fall from an outbreak-associated peak. Despite effective vaccines, identifying ways to detect and treat shedding remain vital to break transmission and eliminate typhoid.

Right costophrenic angle abscess due to Salmonella Dublin infection in combination with type 2 diabetes mellitus

The main manifestations of type 2 diabetes mellitus are excessive drinking, polyphagia, polyuria and wasting or weight loss in a short period of time, but it is rare to have persistent fever of unknown origin as the main manifestation. This case report describes a 68-year-old male patient with type 2 diabetes mellitus presenting with unexplained fever with persistent exacerbation and a cystic lesion in the right costophrenic horn on abdominal computed tomography (CT). A cytoculture examination of the puncture fluid suggested that the infection was due to Salmonella Dublin. The patient was treated with drainage of the abscess in the right costophrenic angle area, which then healed successfully. These findings suggest that Salmonella Dublin infection should be considered when a patient with type 2 diabetes mellitus presents with an unexplained persistent fever. At the same time, CT-guided abscess puncture can be performed to improve the patient's symptoms, aid diagnosis and improve the quality of life.

Heparin-Induced Fever in Neurointensive Care Unit: A Rarity Yet a Possibility

Fever is considered a protective response having multitude of benefits in terms of enhancing resistance to infection, recruiting cytokines to the injured tissue, and promoting healing. In terms of an injured brain, this becomes a double-edged sword triggering an inflammatory cascade resulting in secondary brain injury. It is important to identify the etiology so that corrective measures can be taken. Here we report a case of persistent fever in a patient with Guillain-Barré syndrome, which was probably due to heparin. This is the first report of heparin-induced fever in a neurocritical care setting and third report overall.

Hematuria as an Early Sign of Multisystem Inflammatory Syndrome in Children: A Case Report of a Boy With Multiple Comorbidities and Review of Literature

Introduction: While the clinical course of SARS-CoV-2 infection seems to be milder or asymptomatic within the pediatric population, growing attention has been laid to the rare complication elicited by virus, multisystem inflammatory syndrome in children temporarily associated with COVID-19 (MIS-C). Published definition and criteria of MIS-C include persistent fever, multisystem involvement, and elevated markers of inflammation, without obvious microbial inflammation or other plausible diagnosis. However, the aim of this case report is to emphasize the diversity of symptoms of MIS-C, beyond the defined criteria.Case Presentation: We present a 10-year-old boy with 8p23.1 microdeletion syndrome and multiple comorbidities who initially came to our attention due to hematuria, persistent fever, rash, and elevated markers of inflammation. Within the next 2 days, his condition worsened despite the broad-spectrum antibiotic therapy. Assuming his past history of SARS-CoV-2 exposure, MIS-C was suspected. A high level of clinical suspicion was further supported by significant clinical features (vomiting, abdominal pain, conjunctivitis, arrhythmia, and mild left ventricular systolic dysfunction with pleural effusion) along with laboratory findings (elevated ESR, CRP, proBNP, D-dimers and fibrinogen, positive IgG SARS-CoV-2 antibodies, and negative microbiological cultures). The patient was given intravenous immunoglobulin (IVIG) and began to show instantaneous clinical and laboratory improvement.Conclusion: Despite numerous reports of MIS-C cases in children, there are still many uncertainties regarding the clinical presentation and laboratory findings, as well as mechanisms beyond this intriguing disorder. In our case, for the first time hematuria is reported as an early symptom of MIS-C. We strongly believe that reporting various manifestations and outcomes in MIS-C patients will lead to improved diagnosis, treatment, and overall understanding of this novel inflammatory condition.