Case report: Aggressive progression of acute heart failure due to juvenile tuberculosis-associated Takayasu arteritis with aortic stenosis and thrombosis.

Background: Takayasu arteritis (TA) is a chronic granulomatous vasculitis with unknown pathophysiology. TA with severe aortic obstruction has a poor prognosis. However, the efficacy of biologics and appropriate timing of surgical intervention remain controversial. We report a case of tuberculosis (TB)-associated TA with aggressive acute heart failure (AHF), pulmonary hypertension (PH), thrombosis, and seizure, who failed to survive after surgery. Case presentation: A 10-year-old boy who developed a cough with chest tightness, shortness of breath, hemoptysis with reduced left ventricular ejection fraction, PH, and increased C-reactive protein and erythrocyte sedimentation rate was hospitalized at the pediatric intensive care unit of our hospital. He had strongly positive purified protein derivative skin test and interferon-gamma release assay result. Computed tomography angiography (CTA) showed occlusion of proximal left subclavian artery and stenosis of descending aorta and upper abdominal aorta. His condition did not improve after administration of milrinone, diuretics, antihypertensive agents, and intravenous methylprednisolone pulse followed by oral prednisone. Intravenous tocilizumab was administered for five doses, followed by two doses of infliximab, but his HF worsened, and CTA on day 77 showed complete occlusion of the descending aorta with large thrombus. He had a seizure on day 99 with deterioration of renal function. Balloon angioplasty and catheter-directed thrombolysis were performed on day 127. Unfortunately, the child's heart function continued to deteriorate and died on day 133. Conclusion: TB infection may be related to juvenile TA. The biologics, thrombolysis, and surgical intervention failed to achieve the anticipated effect in our case with aggressive AHF due to severe aortic stenosis and thrombosis. More studies are needed to determine the role of biologics and surgery in such dire cases.

Case Report: Macrophage Activation Syndrome and Widespread Neuroimaging Abnormality in Childhood-Onset Systemic Lupus Erythematosus.

Macrophage activation syndrome (MAS) and widespread brain lesions are rare and severe complications of childhood-onset systemic lupus erythematosus (SLE). We report an 11-year-old girl who presented with recurrent rashes for half a year and fever for 2 weeks. Clinical and laboratory features at admission pointed to the diagnosis of SLE and SLE-associated MAS. Cerebral magnetic resonance imaging taken on day 4 after admission showed abnormal signals. Glucocorticoid therapy was started on day 5. Two days later, the patient appeared weak and ill, then the next day she exhibited dizziness, drowsiness, apathia, and dysarthria. High-dose methylprednisolone, cyclophosphamide, and intravenous immunoglobulin were used to treat the patient, and intrathecal dexamethasone was given. The patient was discharged on day 30 after admission and showed complete clinical resolution and improved magnetic resonance imaging resolution.

Juvenile-onset multifocal osteonecrosis in systemic lupus erythematosus: A case report.

RATIONALE: Osteonecrosis (ON) is a devastating illness that leads to bone ischemia and potential joint destruction. Systemic lupus erythematosus (SLE) is a chronic, autoimmune disease, with multi-system involvement which is closely associated with occurrence of ON. Multifocal ON, with an estimated morbidity of 3% in SLE patients, is extremely rare in juvenile subjects. PATIENT CONCERNS: A 13.3-year-old female SLE patient was admitted to hospital 20 months following the SLE diagnosis because of a sudden aggravation of sore knees. She suffered from double knee joint pain and her left knee joint showed typical signs of inflammation including redness, swelling, heat, and pain. DIAGNOSES: The SLE patient was diagnosed with multifocal ON of her knee joint based on magnetic resonance imaging findings of bone destruction and osteoproliferation at the bilateral distal femur and proximal tibia. INTERVENTIONS: The patient received high-dose methylprednisolone and intravenous cyclophosphamide pulse therapies for controlling active lupus and nephritis. Oral calcitriol and dipyridamole were administered to alleviate knee pain and inhibit thrombi formation, thereby suppressing ON progress. OUTCOMES: Three weeks following the treatment, the swelling in patient's left knee subsided. Her self-reporting pain score decreased from 9 to 4 and walking time increased from 45minutes to 90minutes per day. Nearly 5 weeks later, the pain in bilateral knee joints disappeared and the patient could walk without difficulties. LESSONS: This patient is the youngest SLE patient who developed multifocal ON based on the reported literature. It suggests that ON can occur in young SLE patients. A combination of internal and external risk factors can promote the development of ON. The balanced approach to the application of corticosteroids and immunosuppressors in the treatment of SLE and prevention of ON is a challenging problem that deserves further exploration.

Acute Flaccid Myelitis in Children in Zhejiang Province, China.

In July-December 2018, an outbreak of polio-like acute flaccid myelitis (AFM) occurred in Zhejiang province, China. Enterovirus (EV)-D68 infection has been reported to be associated with AFM. This study aimed to investigate the clinical presentation, laboratory findings, and outcomes of AFM patients. We investigated the clinical and virologic information regarding the AFM patients, and real-time PCR, sequencing, and phylogenetic analysis were used to investigate the cause of AFM. Eighteen cases met the definition of AFM, with a median age of 4.05 years (range, 0.9-9 years), and nine (50%) were EV-D68 positive. Symptoms included acute flaccid limb weakness and cranial nerve dysfunction. On magnetic resonance imaging, 11 (61.1%) patients had spinal gray matter abnormalities. Electromyography results of 16 out of 17 patients (94.1%) were abnormal. Cerebrospinal fluid (CSF) pleocytosis was common (94.4%), while CSF protein concentration was normal in all patients. There was little improvement after early aggressive therapy. Phylogenetic analysis revealed that EV-D68 subclade B3 was the predominant lineage circulating in Zhejiang province in 2018.

Phalangeal microgeodic syndrome in childhood.

BACKGROUND: Phalangeal microgeodic syndrome is an uncommon benign self-limiting condition that often occurs during cold weather. The etiology and the pathogenesis of the disease remain unclear. OBJECTIVE: To report a series of children with phalangeal microgeodic syndrome. MATERIALS AND METHODS: Twenty children with phalangeal microgeodic syndrome were retrospectively identified at our hospital after 2007. The clinical data, radiologic manifestation and pathologic appearance were analyzed. RESULTS: The average age was 10.3 years (range: 6.5-14.6 years). Twelve patients were boys. Twenty-five phalanges were affected radiographically (23 middle phalanges [92%] and 2 proximal phalanges [8%]). On radiographs, there were multiple small phalangeal lacunae in all cases. Metaphyseal rarefaction was seen in 15 phalanges, and metaphyseal transverse lucent bands were found in 7 phalanges. Epiphyseal rarefaction was seen in three phalanges. On magnetic resonance imaging (MRI), diffuse signal abnormalities of affected phalanges were observed in all cases. Multiple other phalanges and metacarpals also showed marrow edema in three cases. CONCLUSION: Phalangeal microgeodes may represent bone absorption and destruction in response to exaggerated peripheral circulatory impairment following chilblain, and mainly occur in bone growth spurts.

Identification and characterization of DNAzymes targeting DNA methyltransferase I for suppressing bladder cancer proliferation.

Epigenetic inactivation of genes plays a critical role in many important human diseases, especially in cancer. A core mechanism for epigenetic inactivation of the genes is methylation of CpG islands in genome DNA, which is catalyzed by DNA methyltransferases (DNMTs). The inhibition of DNMTs may lead to demethylation and expression of the silenced tumor suppressor genes. Although DNMT inhibitors are currently being developed as potential anticancer agents, only limited success is achieved due to substantial toxicity. Here, we utilized a multiplex selection system to generate efficient RNA-cleaving DNAzymes targeting DNMT1. The lead molecule from the selection was shown to possess efficient kinetic profiles and high efficiency in inhibiting the enzyme activity. Transfection of the DNAzyme caused significant down-regulation of DNMT1 expression and reactivation of p16 gene, resulting in reduced cell proliferation of bladder cancers. This study provides an alternative for targeting DNMTs for potential cancer therapy.

Imaging findings of pancreatoblastoma in 4 children including a case of ectopic pancreatoblastoma.

BACKGROUND: Pancreatoblastoma is a rare primary pancreatic neoplasm of children. The tumour may arise from any portion of the pancreas, but no tumour originating from ectopic pancreatic tissue has been reported in the English medical literature. OBJECTIVE: We report the radiological findings of pancreatoblastoma in four children, including one tumour originating from the mesentery, to our knowledge, reported for the first time. MATERIALS AND METHODS: Four children with pancreatoblastoma were identified at our hospital between 2000 and 2007. US and CT were performed in all children, with MR performed in two. Various CT and MRI features were evaluated including size, organ of origin, definition and quality of tumour margins, heterogeneity and attenuation of the mass, calcification, presence of ascites, adenopathy, metastases, and signal intensity on MR images. Complete excision was performed in all children. The diagnosis of pancreatoblastoma was confirmed with histopathological results. RESULTS: Our cases displayed some typical and atypical features of pancreatoblastoma. The findings of our series suggest that the majority of these tumours are large, heterogeneous and of low to intermediate signal intensity on T1-W images and high signal intensity on T2-W images. Calcifications were common on CT. All of the tumours had heterogeneous enhancement on CT and MR images. CONCLUSION: Pancreatoblastoma is a rare primary tumour of the pancreas. When a large mass is identified in the pancreas in children that is heterogeneous in nature with internal calcifications, the diagnosis should be considered.

[Cetyltrimethylammonium bromide for fluorescence enhancement of anhydrotetracycline hydrochloride and iso-tetracycline].

Fluorescence enhancement of anhydrotetracycline hydrochloride and iso-tetracycline has been described. The fluorescence intensities of anhydrotetracycline hydrochloride and iso-tetracycline with cetyltrimethylammonium bromide (CTMAB) enhanced by micellar solution have been examined. It is found that fluorescence enhancement of anhydrotetracycline hydrochloride and iso-tetracycline depends on the concentration of CTMAB and pH of the solution. It can be used to develop sensitive methods for the determination of tetracycline hydrochloride and its decomposition product.