Kawasaki Shock Syndrome with Initial Presentation as Neck lymphadenitis: A Case Report.

Kawasaki disease (KD) is an acute systemic vasculitis of unknown cause that mainly affects infants and children and can result in coronary artery complications if left untreated. A small subset of KD patients with fever and cervical lymphadenitis has been reported as node-first-presenting KD (NFKD). This type of KD commonly affects the older pediatric population with a more intense inflammatory process. Considering its unusual initial presentation, a delay in diagnosis and treatment increases the risk of coronary artery complications. Herein, we report the case of a 9-year-old female with fever and neck mass that rapidly deteriorated to shock status. A diagnosis of KD was made after the signs and symptoms fulfilled the principal diagnostic criteria. The patient's heart failure and blood pressure improved dramatically after a single dose of intravenous immunoglobulin. This case reminds us that NFKD could be the initial manifestation of KDSS, which is a potentially fatal condition. We review the literature to identify the overlapping characteristics of NFKD and KDSS, and to highlight the importance of early recognition of atypical KD regardless of age. We conclude that unusually high C-reactive protein, neutrophilia, and thrombocytopenia serve as supplemental laboratory indicators for early identification of KDSS in patients with NFKD.

Characteristics of children with Kawasaki disease requiring intensive care: 10 years' experience at a tertiary pediatric hospital.

BACKGROUND/PURPOSE: Kawasaki disease (KD) is a febrile systemic vasculitis, and some patients may develop serious complications requiring intensive care. We aim to ascertain the clinical presentations and outcomes of these patients. METHODS: From October 2004 to October 2014, children with KD who had stayed in the pediatric intensive care unit (ICU) for acute stage treatment were defined as case patients; for each case, three age/sex-matched patients with KD but without ICU stay, if identified, were selected as control patients. Clinical data were retrospectively collected and analyzed. RESULTS: Among the total of 1065 KD patients, we identified 26 case patients and 71 controls for statistical analysis. ICU patients had a longer fever duration, and tended to have hemoglobin level < 10 g/dL, platelet count < 150 × 109/L, band cell percentage > 10%, peak serum C-reactive protein level > 200 mg/L, serum albumin value < 3 g/dL, and often presented with multiorgan system involvement. Time from symptom onset to the diagnosis of KD was similar between the two groups, but ICU patients were less likely to have KD as a leading admission diagnosis. Shock (73.1%, n = 19) was the most common reason for ICU admission. ICU patients were more likely to receive antibiotics, albumin infusion, and require a second dose of intravenous immunoglobulin or steroid therapy. No in-hospital mortality was observed. CONCLUSION: Patients with KD requiring ICU admission are significantly associated with multiorgan involvement, abnormal hematological and biochemistry biomarkers, KD recognition difficulty at the time of admission, and intravenous immunoglobulin-refractory KD.

Mitochondrial Sirt3 supports cell proliferation by regulating glutamine-dependent oxidation in renal cell carcinoma.

Clear cell renal carcinoma (RCC), the most common malignancy arising in the adult kidney, exhibits increased aerobic glycolysis and low mitochondrial respiration due to von Hippel-Lindau gene defects and constitutive hypoxia-inducible factor-α expression. Sirt3 is a major mitochondrial deacetylase that mediates various types of energy metabolism. However, the role of Sirt3 as a tumor suppressor or oncogene in cancer depends on cell types. We show increased Sirt3 expression in the mitochondrial fraction of human RCC tissues. Sirt3 depletion by lentiviral short-hairpin RNA, as well as the stable expression of the inactive mutant of Sirt3, inhibited cell proliferation and tumor growth in xenograft nude mice, respectively. Furthermore, mitochondrial pyruvate, which was used for oxidation in RCC, might be derived from glutamine, but not from glucose and cytosolic pyruvate, due to depletion of mitochondrial pyruvate carrier and the relatively high expression of malic enzyme 2. Depletion of Sirt3 suppressed glutamate dehydrogenase activity, leading to impaired mitochondrial oxygen consumption. Our findings suggest that Sirt3 plays a tumor-progressive role in human RCC by regulating glutamine-derived mitochondrial respiration, particularly in cells where mitochondrial usage of cytosolic pyruvate is severely compromised.