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This study was designed to examine the capability of renal ultrasonography (US) for predicting vesicoureteral reflux (VUR) and renal scarring (RS), and to assess, using initial US, the significant urologic abnormalities that impact on management of children hospitalized with a first febrile urinary tract infection (UTI). Methods: Hospitalized children aged ≤ 2 years with a first febrile UTI were prospectively evaluated using imaging studies, including 99mTc dimercaptosuccinic acid (DMSA) scan, US, and voiding cystourethrography. Results: Of the 310 children analyzed (195 boys and 115 girls), 105 (33.9%) had abnormal US. Acute DMSA scans were abnormal in 194 children (62.6%), including 89 (45.9%) with concomitant abnormal US. There was VUR in 107 children (34.5%), including 79 (25.5%) with Grades III–V VUR. The sensitivity and negative predictive values of US were 52.3% and 75.1%, respectively, for Grades I–V VUR and 68.4% and 87.8%, respectively, for Grades III–V VUR. Eighty-five children (27.4%) had RS, including 55 (64.7%) with abnormal US. Of the 105 children with abnormal US, 33 (31.4%) needed subsequent management (surgical intervention, parental counseling, or follow up of renal function). Nephromegaly on initial US and Grades III–V VUR were risk factors of RS. Conclusion: Abnormal US may carry a higher probability of Grades III–V VUR and RS, and can affect subsequent management in a significant number of children. Nephromegaly on initial US and Grades III–V VUR are strongly associated with an increased risk for RS. Thus, US should be performed on children after a first febrile UTI and children with normal US may not require voiding cystourethrography.

Deficiency of hypoxanthine phosphoribosyltransferase activity is a rare inborn error of purine metabolism with subsequent uric acid overproduction and neurologic presentations. The diagnosis of Lesch–Nyhan syndrome (LNS) is frequently delayed until self-mutilation becomes evident. We report the case of a boy aged 1 year and 10 months who was diagnosed with profound global developmental delay, persistent chorea, and compulsive self-mutilation since the age of 1 year. Serial serum uric acid levels showed normal uric acid level, and the spot urine uric acid/creatinine ratio was >2. The hypoxanthine phosphoribosyltransferase cDNA showed the deletion of exon 6, and the boy was subsequently diagnosed to have LNS. He also had respiratory distress due to pulmonary embolism documented by chest computed tomography scan. This report highlights the need to determine the uric acid/creatinine ratio caused by increased renal clearance in LNS in young children. The presence of pulmonary embolism is unusual and may be the consequence of prolonged immobilization.

Rotavirus and norovirus are the most common known causes of viral gastroenteritis in children. This study examined the association between serum interleukin 6 (IL-6) and interleukin 8 (IL-8) levels and disease severity in the acute phase of rotavirus and norovirus gastroenteritis in children, and it also explored the role of fecal cytokine levels in children with viral and bacterial gastroenteritis. Methods: This prospective study enrolled patients aged 4 months to 14 years admitted with acute gastroenteritis in a tertiary care center. Peripheral blood samples were collected for IL-6 and IL-8 assays within the first 3 days of diarrhea. Stool samples were obtained from the patients in the first 24 hours after admission. Results: Serum IL-6 and IL-8 were measured in children with viral (n = 66) and bacterial (n = 23) infections, and in healthy controls (n = 10). In the acute phase of gastroenteritis, a moderately positive correlation was found between serum IL-6 levels and disease severity (rs = 0.41, p

Multicystic dysplastic kidney (MCDK) is a congenital maldevelopment in which the renal cortex is characteristically replaced by numerous cysts of multiple sizes. MCDK presenting as a single predominant large cyst in morphology is less common. We report on the prenatal imaging findings and perinatal management of a fetus with MCDK unusually presenting as a single predominant large cyst, erroneously interpreted as a severe fetal hydronephrosis. Details of the perinatal history, radiological evaluation, morphological characteristic, and clinical aspect of this case are presented. We also discuss a few studies addressing the sensitivity of magnetic resonance urography for the prenatal diagnosis of MCDK.

Tuberous sclerosis complex (TSC) is a multisystem disorder resulting in hamartomatous lesions. Despite diverse manifestations, skeletal muscular comorbidity is rarely reported in TSC. We report a 2-year-old girl with TSC who suffered from multiple subcutaneous masses over the paraspinal and bilateral scapular areas, which caused disabling pain on any passive movement. Three-dimensional computed tomography scanning revealed multiple calcifications that were consequently diagnosed as fibrodysplasia ossificans progressiva. Such imaging features should be evaluated cautiously to avoid unnecessary surgical intervention and biopsy that may worsen the condition.

Cordyceps militaris is a well-known fungus with immunomodulatory activity. It is generally used in traditional Chinese medicine to treat hemoptysis, bronchial or lung inflammation, and urogenital disorders. The purpose of our study was to evaluate the effect of cultivated C. militaris on airway inflammation in a mouse asthma model. Methods: BALB/c mice were sensitized with intraperitoneal ovalbumin (OVA) on Days 0 and 14, and were then given intranasal OVA on Day 14 and Days 25-27. Randomized treatment groups of sensitized mice were administered C. militaris, prednisolone, montelukast, or placebo by gavage from Days 15-27. Airway hyperreactivity to aerosolized methacholine was determined. Bronchoalveolar lavage fluid and serum were analyzed to assess airway inflammation. Results: OVA-sensitized mice developed a significant airway inflammatory response that was inhibited by prednisolone and montelukast, whilst C. militaris reduced airway inflammation less effectively. Airway hyperresponsiveness to methacholine was observed in OVA-sensitized mice and was reversed by both prednisolone and montelukast. C. militaris initially reversed airway hyperreactivity, but this effect disappeared at higher methacholine doses. Conclusion: C. militaris can modulate airway inflammation in asthma, but it is less effective than prednisolone or montelukast. These results demonstrate that C. militaris is unable to adequately block the potent mediators of asthmatic airway inflammation.

Leigh syndrome is an early-onset progressive neurodegenerative disorder with a characteristic neuropathology consisting of focal, bilateral lesions in one or more areas of the central nervous system. The brain images of Leigh syndrome are characterized by markedly symmetrical involvement, most frequently of the putamen. We report a 2-year-old girl with Leigh syndrome manifested as acute onset of altered level of consciousness. Brain magnetic resonance images showed abnormal signal intensity over the bilateral basal ganglia and cerebellar dentate nuclei. Despite normal biochemical studies, in particular serum lactate levels, magnetic resonance spectroscopy demonstrated a downward doublet lactate peak. The diagnosis of Leigh syndrome was subsequently confirmed by genetic study which showed a novel mutation at 8597T>C of the mitochondrial ATPase6 gene.