Xanthogranulomatous pyelonephritis: A rare case report of a 54 year old female (a potentially fatal infection).

INTRODUCTION AND IMPORTANCE: Xanthogranulomatous pyelonephritis (XPGN) is a rare pathology of the kidneys occurring in 0.6 to 1% of all cases of renal infections, in both men and women. It is characterized by severe inflammation of the renal parenchyma leading to formation of granulomatous tissue containing lipid-laden macrophages. This condition may mimic less aggressive or benign conditions but may worsen or be fatal if not treated aggressively. CASE PRESENTATION: Our patient is a 54 year old Caucasian female who presented with five days of left flank pain, hematuria, chills, nausea and vomiting. Imaging and biopsy results showed that the patient had XPGN. CLINICAL DISCUSSION: XPGN is a difficult condition to diagnose as the symptoms are non-specific relative to renal cell carcinoma or other common renal infections. Definitive diagnosis is made with a biopsy; however, clues in various imaging modalities are used to make a tentative diagnosis. It is unclear whether earlier surgical intervention would have improved overall patient outcomes. Currently, a partial or complete nephrectomy is the only effective treatment. CONCLUSION: Aggressive management including early diagnosis, antibiotics and nephrectomy appears to be critical in preventing progression and complications of XPNG.

Acute myocardial infarction in the setting of left bundle branch block: Chapman's sign.

Acute myocardial infarction (AMI) diagnosis in patients with pre-existing left bundle branch block (LBBB) can be difficult. Undiagnosed or delayed diagnosis of AMI in these patients can put them at risk of having shock, mechanical complications, and death. We present a case of 77-year-old Caucasian male with a known LBBB and coronary artery bypass surgery for coronary artery disease who presented to the emergency department with a chief complaint of chest pain and shortness of breath. The patient had recurrent chest pain despite using aspirin, nitroglycerine, and morphine. An electrocardiogram (ECG) showed a new notch in the upslope of the R wave in leads I, AVL that indicated a positive Chapman's sign. Troponin levels were initially normal, but serial troponin showed elevated enzyme giving evidence of acute coronary syndrome (ACS). The patient was started on heparin drip and underwent subsequent coronary catheterization. Physicians should be aware of Chapman's sign on ECG in patients presenting with chest pain who have baseline LBBB as it might represent myocardial ischemia and warrant emergent treatment for ACS.

Multivariate analysis of hypertension in general US adults based on the 2017 ACC/AHA guideline: data from the national health and nutrition examination survey 1999 to 2016.

PURPOSE: Since the publication of the 2017 ACC/AHA hypertension guideline, few studies have analyzed the epidemiology and management of hypertension across many heterogeneous subgroups in the US. MATERIALS AND METHODS: We analyzed the data collected by the National Health and Nutrition Examination Survey (NHANES) from 1999 to 2016. Participants greater than 20 years of age, who had valid blood pressure measurements, were included in the study. A comprehensive analysis of 14 population subgroups was done to systematically examine how hypertension awareness, treatment, and control varied by subpopulations. RESULTS: Our study included 45,557 participants with a mean age of 47.1 years, 48.3% were male and 51.7% were female. 47.8% participants had hypertension, of which 59.9% were aware of their diagnosis. Among the participants who were aware of their hypertension, 90.3% were prescribed medication(s) to lower blood pressure, of which 39.2% had achieved control. Multivariate analysis showed age, gender, Race/ethnicity, annual family income, education level, BMI, waist to height ratio, diabetes, and previous cardiovascular events to be independent risk factors for hypertension. Lower likelihoods of awareness and treatment were associated with male sex, younger age, Mexican ethnicity, participants without health insurance, absence of previous cardiovascular events, diabetes, obesity, or smoking. Overall, hypertension control rate was suboptimal across most of subgroups (<40%). CONCLUSION: Based on the 2017 ACC/AHA guideline, almost half of the US adult population has hypertension. The suboptimal number of people with controlled blood pressure is pervasive in all subgroups and warrants greater efforts in prevention, as well as more effective treatment.

The Clinical Significance of Relative Bradycardia.

INTRODUCTION: Relative bradycardia is a poorly understood paradoxical phenomenon that refers to a clinical sign whereby the pulse rate is lower than expected for a given body temperature. OBJECTIVE: To provide an overview and describe infectious and noninfectious causes of relative bradycardia. METHODS: PubMed and Medline databases were searched using individual and Medical Subject Headings terms including relative bradycardia, fever, pulse-temperature dissociation and pulsetemperature deficit in human studies published from inception to October 2, 2016. The causes and incidence of relative bradycardia were reviewed. RESULTS: Relative bradycardia is found in a wide variety of infectious and noninfectious diseases. The pathogenesis remains poorly understood with proposed mechanisms including release of inflammatory cytokines, increased vagal tone, direct pathogenic effect on the myocardium, and electrolyte abnormalities. The incidence of this sign varies widely, which may be attributable to multiple factors, including population size, time course for measuring pulse and temperature, and lack of a consistent definition used. The fact that this sign is not consistently identified in case series suggests that relative bradycardia is caused by mechanisms presumably involving or influenced by pathogen and host factors. CONCLUSIONS: Relative bradycardia is a sensitive but nonspecific clinical sign that may be an important bedside tool for narrowing the differential diagnosis of potential infectious and noninfectious etiologies. Recognizing this relationship may assist the clinician by providing bedside clinical clues into potential etiologies of disease, particularly in the setting of infectious diseases and in circumstances when other stigma of disease is absent.

Depletion of MHC class II invariant chain peptide or γ-δ T-cells ameliorates experimental preeclampsia.

Excessive innate immune system activation and inflammation during pregnancy can lead to organ injury and dysfunction and preeclampsia (PE); however, the molecular mechanisms involved are unknown. We tested the hypothesis that Toll-like receptor (TLR) activation induces major histocompatibility complex (MHC) class II invariant chain peptide (CLIP) expression on immune cells, makes them pro-inflammatory, and are necessary to cause PE-like features in mice. Treatment with VG1177, a competitive antagonist peptide for CLIP in the groove of MHC class II, was able to both prevent and treat PE-like features in mice. We then determined that γ-δ T cells are critical for the development of PE-like features in mice since γ-δ T-cell knockout mice, like CLIP deficient mice, are resistant to developing PE-like features. Placentas from women with PE exhibit significantly increased levels of γ-δ T cells. These preclinical data demonstrate that CLIP expression and activated γ-δ T cells are responsible for the development of immunologic PE-like features and that temporarily antagonizing CLIP and/or γ-δ T cells may be a therapeutic strategy for PE.

Secondary malignant neoplasms after high-dose chemotherapy and autologous stem cell rescue for high-risk neuroblastoma.

BACKGROUND: Outcomes for high-risk neuroblastoma remain poor. Modern treatment protocols utilizing intense induction followed by myeloablative consolidation chemotherapy with autologous stem cell rescue (ASCR) have improved survival rates, but the long-term sequelae, including development of secondary malignant neoplasms (SMN), are just now surfacing. METHODS: We retrospectively reviewed data from 87 patients with high-risk neuroblastoma who were treated with intensive induction chemotherapy followed by ASCR between January 1991 and July 2011 following one of two institutional protocols: Chicago Pilot 1 (CP1; n = 12) and Chicago Pilot 2 (CP2; n = 75). RESULTS: The 15-year overall survival rate for all 87 patients was 33.9% (95% confidence interval [CI], 23.1-45.0%). The 10- and 15-year cumulative incidence of SMN was 16.5% (95%CI, 7.2-38.0%) and 34.2% (95%CI, 18.6-63.1%), respectively, without evidence of a plateau at 15 years. Six of the 10 patients (n = 2 in CP1 and n = 8 in CP2) who developed SMN had hematologic malignancies including acute myeloid leukemia (AML)/myelodysplastic syndrome (MDS). Solid tumors included thyroid papillary carcinoma, chondrosarcoma, hepatocellular carcinoma, and biliary adenocarcinoma. CONCLUSION: A significantly higher incidence of SMN, especially hematological malignancies, was observed in this cohort compared to older neuroblastoma studies, potentially due to exposure to epipodophyllotoxins and a high cumulative dose of alkylating agents these patients received. The risk of developing an SMN continued to increase with survival time and did not reach the plateau at 15 years. Although the number of the patients is relatively small, our study emphasizes the need for life-long follow-up of survivors who were treated using modern therapy.

Disseminated mucormycosis in a patient with recent kidney transplantation: a case report and review of the literature.

Mucormycosis is an invasive fungal infection commonly seen in diabetics and immunocompromised patients. We report a case of disseminated mucormycosis in a 47-year-old diabetic male who underwent deceased donor renal allograft transplantation about 5 weeks prior to presentation. Our patient presented with increasing fatigue, diarrhea and oligoanuria and was found to have significant acute kidney injury. Doppler ultrasound of the allograft revealed segmental decreased renal perfusion in the upper pole of the allograft with moderate hydronephrosis. Nephrostomy tube placement yielded minimal urine output. An allograft biopsy showed diffuse C4d-positive staining and fungal hyphae suggestive of Mucor infection. Computed tomography (CT) imaging revealed a right upper lobe mass, a small hypodensity in the liver and normal findings in the head. Despite prompt management including discontinuation of immunosuppression, amphotericin B and allograft nephrectomy, the patient had a rapid decompensation, developed respiratory failure requiring intubation, hypotension and supraventricular tachycardia with multiple new areas of hypoattenuation on head CT - all of which ultimately resulted in his death. A review of the literature revealed that mucormycosis is a relatively rare disease with a cumulative 12-month incidence rate of 0.07% in solid organ transplant recipients. Disseminated disease was found in about 23% of cases, with a mortality rate of 96%.