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Extraadrenal paragangliomas are rare pediatric tumors with variable symptoms that cause difficultly in diagnosis and delayed treatment. We report the case of a 10-year-old boy who presented to the pediatric emergency department with acute chest pain and dyspnea mimicking a non-ST-segment elevation acute myocardial infarction. He was subsequently found to have an extraadrenal paraganglioma after a series of imaging and laboratory examinations. The mass was subsequently removed, and the diagnosis of extraadrenal paraganglioma was confirmed histologically. Acute coronary syndrome as the presenting feature of extraadrenal paragangliomas in pediatric population is an even rarer clinical entity.

To examine differences in clinical characteristics among children with norovirus, rotavirus, and bacterial gastroenteritis and investigate the outcomes in children with sporadic norovirus gastroenteritis. The study included patients aged 4 mo to 14 y who had acute gastroenteritis and were admitted to a tertiary care center between April 2008 and July 2009. The clinical features and laboratory findings of acute gastroenteritis were recorded. Fecal specimens were collected and tested for viruses, bacteria, and parasites. A total of 198 children (median age, 2.1 y) with acute gastroenteritis were studied. The pathogens identified included norovirus (n = 38), rotavirus (n = 47), adenovirus (n = 5), astrovirus (n = 1), bacteria (n = 43), and mixed infections (n = 7). No causative organisms were identified in 57 patients. The norovirus-infected group had a significantly higher proportion of those still vomiting 1 d after the onset of vomiting (p

OBJECTIVE: The aim of this article was to assess the usefulness of procalcitonin (PCT) as a marker for predicting dilating (grades III-V) vesicoureteral reflux (VUR) in young children with a first febrile urinary tract infection. METHODS: Children ≤2 years of age with a first febrile urinary tract infection were prospectively evaluated. Serum samples were tested for PCT at the time of admission to a tertiary hospital. All children underwent renal ultrasonography (US), Tc-dimercaptosuccinic acid renal scan, and voiding cystourethrography. The diagnostic characteristics of PCT test for acute pyelonephritis and dilating VUR were calculated. RESULTS: Of 272 children analyzed (168 boys and 104 girls; median age, 5 months), 169 (62.1%) had acute pyelonephritis. There was VUR demonstrated in 97 (35.7%), including 70 (25.7%) with dilating VUR. The median PCT value was significantly higher in children with VUR than in those without (P < 0.001). Using a PCT cutoff value of ≥1.0 ng/mL, the sensitivity and negative predictive value for predicting dilating VUR were 94.3% and 95.4%, respectively, for PCT, and 97.1% and 97.8%, respectively, for the combined PCT and US studies, whereas the positive and negative likelihood ratios were 2.03 and 0.107, respectively, for PCT, and 1.72 and 0.067, respectively, for the combined studies. By multivariate analysis, high PCT values and abnormalities on US were independent predictors of dilating VUR. CONCLUSIONS: PCT is useful for diagnosing acute pyelonephritis and predicting dilating VUR in young children with a first febrile urinary tract infection. A voiding cystourethrography is indicated only in children with high PCT values (≥1.0 ng/mL) and/or abnormalities found on a US.

BACKGROUND Delayed hematopoietic reconstitution after cord blood transplantation (CBT) may lead to increased risk of complications and longer hospitalization. Bone marrow-derived mesenchymal stem cells (MSCs) have been found to promote engraftment after hematopoietic stem cell transplantation. However, harvesting MSCs from bone marrow involves an invasive procedure. Then again, MSCs can be easily obtained from umbilical cords without harm to the donors. METHODS Umbilical cord-derived MSCs (UCMSCs) were isolated from Wharton's jelly and then ex vivo cultured. After showing normal karyotype and negative for infectious contamination, culture-expanded UCMSCs were intravenously infused into the recipients on the day of CBT. The control patients were those receiving CBT alone. Adverse effects and efficacy of intravenous UCMSCs were evaluated. RESULTS A total of five patients received cotransplantation of UCMSCs at the time of CBT. No serious adverse events were observed. The time to achieve neutrophil engraftment ranged from 7 to 13 days (median, 11 days) and platelet engraftment ranged from 22 to 41 days (median, 32 days). Compared with the nine patients receiving CBT alone, patients receiving cotransplantation of UCMSCs had significantly faster hematopoietic recovery of neutrophils and platelets (P=0.02 and 0.01, respectively). CONCLUSIONS This pilot study is the first report of cotransplantation of UCMSCs in CBT. Intravenous infusion of UCMSCs appeared to be a feasible and safe modality to enhance hematopoietic engraftment in patients receiving CBT. Further studies were warranted.

Ectopic kidney is a rare condition. Giant hydronephrosis is also an uncommon lesion in children and is usually due to ureteropelvic junction obstruction. We report a case of 7-year-old girl presenting with abdominal fullness. The imaging characteristics of magnetic resonance urography of the lesions are reported. The findings from the radiologic investigations were suggestive of a dysplastic ectopic pelvic kidney with giant hydronephrosis. Subsequent surgery confirmed the diagnosis. An ectopic kidney with giant hydronehrosis is an extremely rare condition in children and can present as an asymptomatic abdominal mass. A careful survey for other structural anomalies is mandatory in such cases.

The association between neonatal jaundice and childhood asthma is a new finding of two reports. The purpose of the study was to verify their results.Data from 11,321 children were collected from the National Health Insurance Research Database. Their claims data were evaluated from birth to 10 yr old. Children were analyzed as case (those with neonatal jaundice) and controls (those without neonatal jaundice). The diagnostic criteria for asthma were as follows: at least four asthma diagnoses at outpatient services and emergency department (ED), or one asthma diagnosis during an admission. In children fitting the asthma criteria, those with no asthma diagnosis after 1 yr of age were excluded. Mantel-Haenszel's odds ratios were calculated after adjustment for the following confounders: preterm/low birth weight, neonatal infection, other respiratory conditions, other birth conditions, and gender. Asthma rate, onset time, the use of drugs, upper respiratory infection and lower respiratory infection (LRI) rates, hospital admission/ED visit rates, and the effect of phototherapy were evaluated.After adjustment for the confounding factors, the rate of asthma was higher in icteric children (OR: 1.64, 95% CI 1.36-1.98, p0.001), and the influence in females was stronger. There still was an association between neonatal jaundice and late onset asthma (asthma onset after 3 yr of age). In asthmatic children, those with neonatal jaundice have increased asthma onset rate before age 6, increased use of inhalant steroids, LRI rates, and ED visits for respiratory disease.Neonatal jaundice increased the rate and severity of childhood asthma in subjects aged up to 10 yr and may be a risk factor for childhood asthma.

Acute bacterial meningitis (ABM) is a serious disease with severe neurological sequelae. The intense calcium-mediated microglial activation and subsequently pro-inflammatory cytokine release plays an important role in eliciting ABM-related oxidative damage. Considering resveratrol possesses significant anti-inflammatory and anti-oxidative properties, the present study aims to determine whether resveratrol would exert beneficial effects on hippocampal neurons following ABM. ABM was induced by inoculating Klebsiella pneumoniae into adult rats intraventricularly. The time-of-flight secondary ion mass spectrometry (TOF-SIMS), Griffonia simplicifolia isolectin-B4 (GSA-IB4) and ionized calcium binding adaptor molecule 1 (Iba1) immunohistochemistry, enzyme-linked immunosorbent assay as well as malondialdehyde (MDA) measurement were used to examine the calcium expression, microglial activation, pro-inflammatory cytokine level, and extent of oxidative stress, respectively. In ABM rats, strong calcium signaling associated with enhanced microglial activation was observed in hippocampus. Increased microglial expression was coincided with intense production of pro-inflammatory cytokines and oxidative damage. However, in rats receiving resveratrol after ABM, the calcium intensity, microglial activation, pro-inflammatory cytokine and MDA levels were all significantly decreased. Quantitative data showed that much more hippocampal neurons were survived in resveratrol-treated rats following ABM. As resveratrol successfully rescues hippocampal neurons from ABM by suppressing the calcium-mediated microglial activation, therapeutic use of resveratrol may act as a promising strategy to counteract the ABM-induced neurological damage.

An ectopic ureter draining into the seminal vesicle or vas deferens in males is a very rare anomaly and is usually associated with renal dysplasia or agenesis. An ectopic ureter associated with a dysplastic kidney is not usually a suspected cause during clinical evaluation of children with abdominal pain. This report presents a rare case of an ectopic ureter associated with a dysplastic kidney with an acute infection in a previously healthy 12-year-old boy, demonstrated by magnetic resonance imaging. He presented with abdominal pain that mimicked acute appendicitis-like symptoms which was subsequently complicated by epididymitis manifesting as an acute scrotum. Clinicians should consider including an ectopic ureter in the differential diagnosis of children presenting with acute abdomen.

Supplementary consumption of probiotics may temporarily alter the intestinal microflora of infants and children, thereby preventing and treating allergic disorders.To compare the clinical efficacy of levocetirizine with that of levocetirizine plus Lactobacillus johnsonii EM1 (Lj EM1) for treating perennial allergic rhinitis (PAR) in children.Sixty-three children aged 7-12 years fulfilled the entry criteria for the study and had moderate to severe PAR of at least 1 year's duration. The treatment followed a randomized, open-label crossover design: all subjects were randomized to 2 crossover treatment regimens of levocetirizine with Lj EM1 (group 1) or levocetirizine alone (group 2) for 12 weeks; subsequently, treatments were reversed for a further 12 weeks. The effects of the 2 regimens were compared using the Pediatric Rhinoconjunctivitis Quality of Life Questionnaire (PRQLQ) and the total symptom score (TSS) from diary cards. The parameters evaluated were nasal peak expiratory flow rate (nPEFR), FVC, FEV1, serum immunoglobulin E (IgE), mite-specific IgE, eosinophilic cationic protein (ECP), resistin, blood eosinophils, eosinophil percentage in nasal smears, IL-4, IL-10, interferon-γ (IFN-γ), and transforming growth factor-β (TGF-β).After the first 12 weeks of treatment, TSS in both groups had improved progressively compared with that in the run-in period. Both groups had improved TSS at weeks 4, 8, and 12 (P0.05), and group 1 was more efficacious than group 2 at week 4 (P=0.014), week 8 (P=0.011), and week 12 (P0.009). During the second 12-week period, group 2 showed continual and progressive improvement, while group 1 did not. The PRQLQ scores were significantly decreased in both groups (P0.05), but there was no statistically significant difference between the 2 groups (P=0.446). The eosinophil percentage in nasal smears decreased in both groups compared with that in the run-in period, and significant differences were detected in groups 2 and 1at 16 and 24 weeks of treatment, respectively (P0.05). Both groups showed significant improvement in nPEFR at weeks 4, 8, 12, 16, and 24 (P0.01), and the treatment for group 1 appeared to be more efficacious than that for group 2 at weeks 12, 16, and 20 (P0.05). FVC and FEV1 were improved in both groups at weeks 8 through 24 (P0.05), but there was no significant difference between the 2 groups. In cytokine measurements, IFN-γ and IL-10 increased significantly and IL-4 decreased significantly in both groups, while elevation of TGF-β was seen only in group 1 at 12 weeks (P0.001). However, the difference in TGF-β disappeared after 24 weeks treatment. There was no difference in serum resistin levels. No serious adverse events were recorded in either treatment group.The 24-week, 2-phase, crossover treatment program showed that levocetirizine plus Lj EM1 was more effective for PAR than levocetirizine and that this difference persisted for at least 3 months after discontinuation of Lj EM1.

The allergy is dependent on the balance between Th1 and Th2. The fungal immunodulatory protein (FIP- fve ) was isolated from Flammulina velutipes. FIP- fve has been demonstrated to skew the response to Th1 cytokine production. We investigated whether oral administrations of FIP- fve inhibited allergen (OVA)-induced chronic airway inflammation in the mouse asthma model. After intranasal challenge with OVA, the airway inflammation and hyperresponsiveness were determined by bronchoalveolar lavage fluid (BALF) analysis and ELISA assay. Both pre-treated and post-treated with FIP- fve suppressed the airway hyperresponsiveness by methacholine challenge and significantly decreased the number of infiltrating inflammatory cells and Th2 cytokines in bronchoalveolar lavage fluid (BALF) and serum compared with the OVA sensitized mice. In addition, FIP- fve reduced OVA-specific IgE levels in serum. FIP- fve markedly alleviated the OVA-induced airway hyperresponsiveness (AHR) to inhaled methacholine. Based on lung histopathological studies using hematoxylin and Liu’s staining, FIP- fve inhibited inflammatory cell infiltration compared with the OVA-sensitized mice. Oral FIP- fve had an anti-inflammatory effect on OVA-induced airway inflammations and might posses the potential for alternative therapy for allergic airway diseases.

Purulent pericarditis is an extremely rare complication of invasive Streptococcus pneumoniae infection among children in the antibiotic era, and its mortality remains high if left untreated. This report involves a 4½-year-old girl who presented to our emergency department with productive cough, shortness of breath, and left-sided chest pain with a diagnosis of pneumococcal pneumonia. She subsequently developed life-threatening conditions including bilateral empyema with respiratory failure, purulent pericarditis, and multiple organ failure leading to death. The case highlights that purulent pericarditis is a rare yet possible disorder complicating pneumococcal disease in the antibiotic era. The increase in strains resistant to penicillin should alert emergency physicians to the potential for reemergence of pneumococcal pericarditis in children.

Objectives Rotavirus and norovirus are the two most common causes of acute viral gastroenteritis in children. This study aimed to explore the association of serum interleukin-6 (IL-6) and interleukin-10 (IL-10) levels and the clinical features in children with rotavirus and norovirus gastroenteritis. Methods This prospective study enrolled 168 acute gastroenteritis patients admitted to a tertiary care center. Peripheral blood samples were collected for IL-6 and IL-10 assays within the first 72 h of illness. The diagnostic performance of clinical tests was estimated using the receiver operating characteristic (ROC) analysis. Binary logistic regression modeling was performed to examine the predictive variables. Results Serum IL-6 and IL-10 were measured in children with rotavirus infection ( n = 30), norovirus infection ( n = 25), Salmonella infection ( n = 26), and in 11 healthy controls. There were significant higher degrees of severity of illness and levels of IL-10 in the rotavirus group as compared to the norovirus group. The binary logistic regression analysis revealed that both the ANC and maximum body temperature (BT) were significant clinical predictors for discriminating rotavirus and norovirus gastroenteritis. The ROC curve to evaluate the accuracy of logistic regression model had an AUC of 0.847 (95% CI: 0.741–0.952, p Conclusions IL-10 shows a significant discriminating ability between rotavirus and norovirus infection. A model incorporating maximum BT and ANC can help pediatricians to distinguish between rotavirus and norovirus in children with a suspected viral gastroenteritis.

Acute bacterial meningitis caused by Klebsiella pneumoniae (K. pneumoniae) is a major health threat with a high mortality rate and severe neuro-cognitive sequelae. The intense pro-inflammatory cytokine released from calcium-mediated microglial activation plays an important role in eliciting neuronal damage in the hippocampal region. Considering melatonin possesses anti-inflammatory and immuno-modulatory properties, the present study determined whether melatonin can effectively decrease inflammatory responses and prevent hippocampal damage in animals subjected to K. pneumoniae. Adult rats inoculated with K. pneumoniae received a melatonin injection immediately thereafter at doses of 5, 25, 50, or 100 mg/kg. Following 24 h of survival, all experimental animals were processed for time-of-flight secondary ion mass spectrometry (for detecting glial calcium intensity), isolectin-B4 histochemistry (reliable marker for microglial activation), pro-inflammatory cytokine measurement as well as cytochrome oxidase and in situ dUTP end-labeling (representing neuronal bio-energetic status and apoptotic changes, respectively). Results indicate that in K. pneumoniae-infected rats, numerous calcium-enriched microglia, enhanced pro-inflammatory cytokine, and various apoptotic neurons with low bio-energetic activity were detected in hippocampus. Following melatonin administration, however, all parameters including glial calcium intensity, microglial activation, pro-inflammatory cytokine levels, and number of apoptotic neurons were successfully decreased with maximal change observed at a melatonin dose of 100 mg/kg. Enzymatic data corresponded well with above findings in which all surviving neurons displayed high bio-energetic activity. As effectively reducing glia-mediated inflammatory response is neuro-protective to hippocampal neurons, the present study supports the clinical use of melatonin as a potential therapeutic agent to counteract K. pneumoniae meningitis-induced neuro-cognitive damage.

Acute bacterial meningitis caused by Klebsiella pneumoniae (K. pneumoniae) is a major health threat with a high mortality rate and severe neuro-cognitive sequelae. The intense pro-inflammatory cytokine released from calcium-mediated microglial activation plays an important role in eliciting neuronal damage in the hippocampal region. Considering melatonin possesses anti-inflammatory and immuno-modulatory properties, the present study determined whether melatonin can effectively decrease inflammatory responses and prevent hippocampal damage in animals subjected to K. pneumoniae. Adult rats inoculated with K. pneumoniae received a melatonin injection immediately thereafter at doses of 5, 25, 50, or 100 mg/kg. Following 24 h of survival, all experimental animals were processed for time-of-flight secondary ion mass spectrometry (for detecting glial calcium intensity), isolectin-B4 histochemistry (reliable marker for microglial activation), pro-inflammatory cytokine measurement as well as cytochrome oxidase and in situ dUTP end-labeling (representing neuronal bio-energetic status and apoptotic changes, respectively). Results indicate that in K. pneumoniae-infected rats, numerous calcium-enriched microglia, enhanced pro-inflammatory cytokine, and various apoptotic neurons with low bio-energetic activity were detected in hippocampus. Following melatonin administration, however, all parameters including glial calcium intensity, microglial activation, pro-inflammatory cytokine levels, and number of apoptotic neurons were successfully decreased with maximal change observed at a melatonin dose of 100 mg/kg. Enzymatic data corresponded well with above findings in which all surviving neurons displayed high bio-energetic activity. As effectively reducing glia-mediated inflammatory response is neuro-protective to hippocampal neurons, the present study supports the clinical use of melatonin as a potential therapeutic agent to counteract K. pneumoniae meningitis-induced neuro-cognitive damage.

Background: Interleukin (IL)-8 acts as a potent neutrophils chemoattractant responsible for the migration of neutrophils into the infected renal tissue to protect against invading pathogens. The aim of this study was to assess the role of IL-8 on acute-phase pyelonephritis and later renal scarring in children. Patients and Methods: A total of 124 children with a first-time febrile urinary tract infection (UTI) were studied. The diagnosis of acute pyelonephritis was confirmed by 99m Tc-dimercaptosuccinic acid (DMSA) renal scan. Serum and urine samples were obtained from 124 children with UTI and 20 healthy children for IL-8 measurement. Results: The 124 children were divided into acute pyelonephritis (n = 70) and lower UTI (n = 54) groups according to the results of DMSA scans. The initial serum and urine IL-8 values of children with acute pyelonephritis were significantly higher when compared with lower UTI and healthy controls (all P < 0.001). Renal scarring was seen in 26 (38.8%) of these 67 children with acute pyelonephritis at follow-up DMSA scans. Both the initial serum and urine IL-8 concentrations were significantly higher in children with renal scarring than in those without (both P < 0.001). The mean age of children with renal scarring was also significantly lower than those without scarring (P = 0.004). Multivariate analysis showed that the highest initial IL-8 values, age

Background 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.

Acute pyelonephritis is a common infectious disease in children and can result in permanent renal damage. Interleukin-6 (IL-6) is an important mediator of inflammation in response to bacterial infection. This study investigated the potential relationship between acute-phase IL-6 and subsequent renal scarring in children with a first time febrile acute pyelonephritis.In total, 79 children (age range 1-120 months) with a first time febrile urinary tract infection (UTI) were included. The diagnosis of acute pyelonephritis was confirmed by (99m)Tc-dimercaptosuccinic acid (DMSA) renal scan. Serum and urine samples were collected for IL-6 measurement by enzyme-linked immunosorbent assay before antibiotic treatment for the infection.The 79 children were divided into acute pyelonephritis (n=45) and lower UTI (n=34) groups according to the findings of DMSA scans. The initial serum and urine IL-6 levels of children with acute pyelonephritis were significantly higher compared with lower UTI (p0.001). Renal scarring was detected at the follow-up DMSA scans in 15 (34.1%) of the 44 children with acute pyelonephritis. Both serum and urine IL-6 levels during the acute phase of pyelonephritis were significantly higher in children with renal scarring than in those without (p=0.005 and p = 0.002). The median age of children with renal scarring was significantly lower than those without (p=0.034). Multiple regression analysis showed that higher initial serum and urine IL-6 levels and a younger age were associated with renal scarring.These results demonstrate that in younger children with a first time febrile acute pyelonephritis, elevations of the acute-phase serum and urine IL-6 levels were correlated with an increased risk of subsequent renal scarring.

SUMMARY: Aim: Acute pyelonephritis is a common infectious disease in children and can result in permanent renal damage. Interleukin (IL)-1β is an important inflammatory mediator that appears early during bacterial infection. This prospective study examined urine IL-1β levels in children with acute pyelonephritis documented by 99mTc-dimercaptosuccinic acid (DMSA) scan, and also evaluated whether this cytokine correlated with renal scarring. Methods: A total of 75 children aged 1–121 months with a diagnosis of first-time febrile urinary tract infection (UTI) were studied. The following inflammatory markers were assessed: fever, white blood cell (WBC), neutrophil, C-reactive protein (CRP) and urine IL-1β. Urine samples were collected for IL-1β measurement by enzyme-linked immunosorbent assay before and after antibiotic treatment of the infection. Follow-up DMSA scan was performed at 6–12 months after the acute pyelonephritis to detect renal scarring. Twenty children with other febrile illnesses served as non-renal febrile controls. Results: The 75 children were divided into acute pyelonephritis (n = 41) and lower UTI (n = 34) groups according to the findings of DMSA scans. Fever, WBC count, neutrophil count and CRP were significantly higher in the children with acute pyelonephritis than in those with lower UTI (all P

Neonatal startle disease or stiff baby syndrome is a rare hereditary disease. It is often misdiagnosed as either neonatal seizure or cerebral palsy because of its characteristic exaggerated fits, which can be activated by external voice or touch. In fact, the exact diagnosis can be made easily according to the patient's family history and clinical examination at bedside. Glabellar tapping is a method to elicit the exaggerated responses, and the events can be relieved by forced knee-chest position. Herein, we report one case with hereditary startle disease accompanied with cyanosis and severe apneas, which could be relieved by forced knee-chest position and oral clonazepam. In conclusion, we suggest the diagnosis of the startle disease should be considered when newborns present the symptoms of neonatal seizure. Once the disease is noticed, we can pursue a simple method to confirm the diagnosis at bedside and unnecessary examination like magnetic resonance imaging can be avoided.

Streptococcus pneumoniae is an uncommon etiologic organism in children with hemolytic uremic syndrome (HUS). Historically, severe S. pneumoniae -associated HUS usually has a poor clinical outcome. The clinical manifestations of marked jaundice and hepatic dysfunction in this form of HUS are extremely rare. We report a 10-month-old female infant with S. pneumoniae -associated HUS who had the unusual manifestation of severely elevated conjugated bilirubin and hepatic transaminases. Screening for viral hepatitis was negative, and evidence of biliary obstruction and hepatotoxic drug exposure was also absent. The patient was treated with antihypertensive agents for 2.5 months and required peritoneal dialysis for a period of 26 days. Hepatic function returned to normal on the 8 th day of hospitalization. Renal function was mildly impaired at 1-year follow-up. Our report suggests that severe conjugated hyperbilirubinemia is a rare manifestation of S. pneumoniae -associated HUS in children. It is important for pediatricians that pneumococcal infection with severe hematologic and renal disorders should be investigated for evidence of S. pneumoniae -associated HUS. [ J Formos Med Assoc 2007;106(2 Suppl):S17-S22]

Congenital mesoblastic nephroma (CMN) is a rare benign tumor that occurs during the neonatal period and early infancy. The vast majority of these tumors present as asymptomatic palpable abdominal masses. We describe an unusual presentation of a CMN in a 10-month-old male infant who presented with massive hematuria and the development of hemorrhagic shock. Abdominal ultrasound showed a heterogeneous solid complex mass measuring 4.8 x 3.5 cm arising from the upper pole of the left kidney. The patient was resuscitated using intravenous fluids and blood transfusions because persistent massive bloody urine leading to progressive shock occurred the night of the admission day. Preoperative diagnosis was possible Wilms tumor of the left kidney. The histopathological findings were consistent with the character of a cellular variant of CMN. The patient was free of recurrence and metastasis at the 2-year follow-up examination. Our case report suggests that CMN is a rare benign renal tumor during infancy and may present with unusual massive hematuria and shock.

Urinary tract infection (UTI) is a common clinical disorder in younger infants and children and may result in permanent renal damage. The inflammatory cytokines interleukin (IL)-6 and IL-8 play an important role in response to bacterial infection. This prospective study investigated the association between serum and urine IL-6 and IL-8 levels and acute pyelonephritis confirmed by (99m)Tc-dimercaptosuccinic acid (DMSA) scan. A total of 78 children aged 1-121 months with a diagnosis of first-time febrile UTI were included. The following inflammatory markers were assessed: fever; white blood cells count (WBC); C-reactive protein (CRP); and serum and urine IL-6 and IL-8. The patients were divided into the acute pyelonephritis group (n=42) and the lower UTI group (n=36) according to the results of DMSA scan. Fever, WBC and CRP levels were significantly higher in children with acute pyelonephritis than in those with lower UTI (all p0.001). Significantly, higher initial serum and urine IL-6 and IL-8 levels were found in children with acute pyelonephritis than in those with lower UTI (all p0.001). Serum and urine IL-6 in children with acute pyelonephritis were positively correlated with fever, CRP and leucocyturia. These results indicate that both serum and urine IL-6 and IL-8 levels, particularly IL-6, are useful diagnostic tools for early recognition of acute pyelonephritis in febrile children.

Intrapleural instillation of fibrinolytic agent such as urokinase has been shown to be effective as an adjunctive therapy for children with complicated parapneumonic effusion and empyema. In this study, we described our experience with the use of intrapleural urokinase in the management of complicated parapneumonic effusion in children. We collected 13 patients with a mean age of 50.8 months with parapneumonic pleural effusion or empyema; all were treated with intrapleural urokinase after poor response to appropriate antibiotics and simple tube drainage. We also reviewed another 13 patients with a mean age of 45.8 months from the clinical records of children hospitalized with the same conditions prior to urokinase introduction as a control group. The mean fluid drained during the first 24 hours and the first 72 hours after urokinase instillation were significantly greater than those during 24 hours before instillation, p=0.002 and p0.001, respectively. The total volume of fluid drained was also greater in the urokinase group than that in the control group (p0.001). The mean duration of chest tube drainage was significantly shorter in the urokinase group (8.7 +/- 2.8 days vs. 14.7 +/- 6.1 days, p0.02). The mean length of hospitalization was also significantly shorter in the urokinase group (15.5 +/- 5.3 days vs. 24.4 +/- 6.9 days, p=0.002). All 13 patients were managed successfully with urokinase treatment without further surgical procedures. None of the patients experienced any side effect or adverse event after urokinase instillation. Two patients of the control group finally underwent surgical debridement. In conclusion, the use of intrapleural urokinase treatment in children with complicated parapneumonic effusion is an effective and safe therapy.

Streptococcus pneumoniae is an uncommon etiological organism in children with hemolytic uremic syndrome (HUS). Patients with S. pneumoniae-associated HUS commonly have a pneumonia or meningitis. Historically, S. pneumoniae-associated HUS usually has a poor clinical outcome. We report 3 pediatric cases of pneumococcal pneumonia-induced HUS. All 3 patients were2 years old, had an empyema complicating pneumococcal pneumonia, and developed renal failure with oliguria and required peritoneal dialysis for a period of 9 to 26 days. All children received several transfusions of unwashed packed red cells and platelets. All of the patients survived. Of the 3 cases, 2 had a normal renal function at discharge, and 1 had a mild renal impairment at 16-month follow-up. Our report suggests S. pneumoniae-associated HUS remains a rare but severe complication of invasive pneumococcal infection in children. It is important for pediatricians to note that children with pneumococcal pneumonia with severe hematologic and renal dysfunction should be investigated for evidence of S. pneumoniae-associated HUS.

Systemic lupus erythematosus (SLE) rarely presents with massive ascites secondary to severe peritonitis before the occurrence of major diagnostic features. We described a 13-year-old girl who developed four episodes of severe abdominal pain with massive ascites. During the first episode, the patient experienced deterioration with massive ascites over a 3-day period, after a 3-month history of intermittent abdominal pain. This episode resolved under conservative treatment. During the second episode, her acute abdominal findings mimicked appendicitis or gynecologic emergency. An exploratory laparotomy was initially planned, but not performed. Thrombocytopenia postponed the surgical intervention and led to further investigation. Autoimmune studies confirmed SLE and prompted steroid therapy, which rapidly improved her painful ascites and spared an unnecessary operation. After improvement of peritonitis with ascites, and following discharge, other major clinical features of SLE ensued. The third episode of peritonitis with massive ascites responded well to steroid pulse therapy. The fourth episode of ascites was accompanied by intracranial hemorrhage that led to her death. To the best of our knowledge, this is the first case report of recurrent peritonitis with massive ascites as the initial and major manifestation of SLE in a pediatric patient.

Post-infectious acute glomerulonephritis with hypertensive encephalopathy is characterized by episodic hypertension with headache, vomiting, and hematuria. The association between hypertensive encephalopathy and cortical blindness in children with acute glomerulonephritis is extremely rare. We report the case of a 10-year-old boy with acute glomerulonephritis who presented with gross hematuria, headache, vomiting, and oliguria, and developed transient cortical blindness as a complication of hypertensive encephalopathy. No occurrence of seizure was observed during the clinical course. T2-weighted cranial magnetic resonance imaging showed a high-intensity signal over cortical and subcortical areas of bilateral occipital regions. His vision recovered fully, 2 days after receiving antihypertensive therapy. The patient was well without complaint at 1-year follow-up. This case highlights the possibility that cortical blindness may develop as a complication of acute glomerulonephritis in children. Prevention of the occurrence of neurological deficits in children with acute glomerulonephritis and hypertensive encephalopathy requires careful evaluation and appropriate management of hypertension.

Pseudomonas aeruginosa is widely prevalent in the hospital environment, especially in intensive care units. Selective IgA deficiency is characterized by a serum IgA level less than 5 mg/dl with no deficiency of other immunoglobulins. The occurrence of community-acquired P. aeruginosa pneumonia with empyema is rare in pediatric patients. We present a 10-month-old male infant who was referred due to persistent fever and progressive respiratory distress for 1 week. A chest radiograph revealed a right lobar pneumonia with pleural effusion. P. aeruginosa that was subsequently isolated from both blood and pleural effusion cultures. The patient received treatment with ceftazidime and intrapleural instillation of urokinase to promote drainage of empyema. Subsequent immunological screening revealed a very low serum IgA level (5 mg/dl). We present our experience in successfully treating a loculated empyema with intrapleural instillation of urokinase in an infant. It is also important for pediatricians to be aware that they should be alert for the patient who present with respiratory infections due to unusual organisms. An advanced immunological study to investigate the underlying disorders in these patients is mandatory.

BACKGROUND: A nationwide asthma survey on the effects of air pollution is lacking in Taiwan. The purpose of this study was to evaluate the time trend and the relationship between air pollution and health care services for asthma in Taiwan. METHODS: Health care services for asthma and ambient air pollution data were obtained from the National Health Insurance Research database and Environmental Protection Administration from 2000 through 2009, respectively. Health care services, including those related to the outpatient and inpatient visits were compared according to the concentration of air pollutants. RESULTS: The number of asthma-patient visits to health-care facilities continue to increase in Taiwan. Relative to the respective lowest quartile of air pollutants, the adjusted relative risks (RRs) of the outpatient visits in the highest quartile were 1.10 (P-trend = 0.013) for carbon monoxide (CO), 1.10 (P-trend = 0.015) for nitrogen dioxide (NO2), and 1.20 (P-trend

It has been well established that intrauterine growth retardation (IUGR) is associated with greater morbidity and mortality rates during perinatal and adult life. The aim of this study is to clarify whether IUGR, defined as a birth weight less than the 10th percentile for gestational age, influences the clinical course of minimal change nephrotic syndrome (MCNS) in children. The study included 50 children aged 1 to 13 years at the onset of MCNS. The diagnosis of MCNS was confirmed by renal biopsy in 25 children (50%). Eight children (16%) had IUGR at birth. Comparisons between children with and without IUGR showed significant differences in mean number of relapses (13.0 +/- 3.5 versus 3.4 +/- 3.0 relapses; P < 0.0001) and relapse rates (relapses per year, 1.6 +/- 0.3 versus 0.5 +/- 0.4; P < 0.0001). We also observed a greater incidence of steroid dependence (100% versus 21.4%; P < 0.001) in children with than without IUGR. In addition, children with IUGR underwent treatment with cytotoxic agents and cyclosporine more frequently than those without IUGR. There was a significantly greater incidence of complications and concomitant diseases of nephrotic syndrome in children with IUGR; results show that MCNS in children with IUGR had a more unfavorable course and outcome. It is important for clinicians to be aware that IUGR may help in the early identification of children at greater risk for frequent relapses and the development of steroid dependence and/or steroid resistance. More aggressive therapy may be indicated for these children.

Chloride transport in the rabbit proximal convoluted tubule (PCT) has components of active, transcellular, and passive, paracellular transport. The preferential reabsorption of bicarbonate and organic solutes by the early proximal tubule leaves the luminal fluid with a higher chloride concentration than that in the peritubular capillaries. Previous studies have suggested that solute permeability of the paracellular pathway may be higher in the neonatal PCT and that the neonatal proximal tubule reabsorbs solutes by passive mechanisms to a greater extent than the adult segment. A higher chloride permeability would provide a mechanism for the greater rate of passive NaCl transport by the neonatal proximal tubule. The purpose of the present in vitro microperfusion study was to directly examine the chloride permeability of neonatal and adult PCT. Superficial and juxtamedullary, neonatal and adult PCT were perfused with a high chloride perfusate without organic solutes, simulating late proximal tubular fluid, at 20 degrees C, and bathed in a serum-like albumin solution. Chloride concentrations in the perfusate and the collected fluid were measured by electrometric titration. Neonatal juxtamedullary PCT chloride permeability (PCl) was significantly lower than adult juxtamedullary PCT PCl (0.15 +/- 0.25 x 10(-5) cm/s versus 5.23 +/- 0.57 x 10(-5) cm/s, p0.001). The PCl of neonatal superficial PCT was not different from that of adult superficial PCT (0.81 +/- 0.48 x 10(-5) cm/s versus 0.05 +/- 0.62 x 10(-5) cm/s). Thus, there is a maturational increase in juxtamedullary PCT PCl, whereas superficial PCT PCl remains very low. The passive diffusion of chloride in neonatal PCT is extremely low and is not a mechanism to explain a higher rate of passive NaCl transport in this segment.

To verify some animal experimental results in humans, we have studied urinary epidermal growth factor (EGF) excretion in normal children as well as children with acute renal failure (ARF). Urinary EGF excretion was expressed as a ratio of urinary EGF to urinary creatinine concentration (EGF/Cr) for random and 24-h urine, and a daily total urinary EGF for 24-h urine. The highest urinary EGF/Cr in children was found at 1 mo to 3 y of age. There was a highly significant correlation between random urine EGF/Cr and 24-h urine EGF/Cr (r = 0.92, p < 0.001), whereas no correlation of urinary EGF/Cr with daily total urine EGF was found. During the course of ARF, a decline in urinary EGF/Cr from the period before peak serum creatinine to the period after the declination of serum creatinine was noted (p = 0.013, n = 13, by repeated measure analysis), with a constant low daily total urine EGF (p value not significant). However, a rise in both urinary EGF/Cr and daily total urine EGF was found between the period of serum creatinine decline and the period of completely normal serum creatinine (p < 0.001). Serum EGF remained unchanged throughout the course of ARF. These results suggest 1) the possible role of EGF in renal growth or maturation during the first 2 or 3 y of life, 2) the possible renal origin of human urinary EGF, and 3) decreased urinary EGF excretion in children with ARF. In particular, EGF/Cr is not a reliable indicator for the expression of actual urinary EGF excretion in ARF. Instead of urinary EGF/Cr, urinary EGF concentration may be used to predict the daily total urinary EGF excretion during ARF. These results provide the pattern of urinary EGF excretion during ARF in children and may be of help for further clinical studies.

The present study investigated the signal-transduction pathway responsible for the epidermal growth factor (EGF) stimulation of phosphate transport (JPhos) in the rabbit proximal convoluted tubule (PCT). Genistein, 10(-4) M, bath and lumen, an inhibitor of EGF receptor tyrosine kinase activity, blocked the EGF effect on JPhos, consistent with a role for tyrosine kinase in the signal-transduction pathway. Both staurosporine (5 x 10(-8) M) and calphostin C (10(-8) M), inhibitors of protein kinase C, blocked the EGF stimulation of JPhos, indicating that protein kinase C is involved in EGF signaling. Intracellular calcium (Ca2+i) concentrations were measured in perfused tubules using fura PE3 to determine whether changes in Ca2+i were also part of the signaling pathway. After addition of 3 nM EGF, there was no change in Ca2+i, suggesting that stimulation of protein kinase C is not from phosphatidylinositol hydrolysis by phospholipase C-gamma. To determine whether phospholipase A2 (PLA2) is involved, the inhibitor mepacrine was used. Mepacrine (5 x 10(-5) M) had no direct effect on PCT transport but blocked the stimulatory effect of EGF on JPhos. PLA2 activity, assessed as free arachidonic acid release from proximal tubules in suspension, increased by 18.8% with 3 nM EGF. Thus the stimulation of JPhos by EGF is mediated via a signal-transduction pathway involving tyrosine kinase, protein kinase C, and PLA2.

Active transcellular NaCl transport in the proximal convoluted tubule (PCT) is via apical parallel Na/H and Cl/base exchange. The mechanism of Cl/base exchange remains unclear. The present in vitro microperfusion study examined the mechanism of Cl/base exchange in superficial and juxtamedullary PCT by examining the rate of change in intracellular pH in response to luminal Cl removal. In superficial PCT the rate of Cl/base exchange was 24.0 +/- 2.3 without formate, 36.4 +/- 6.6 with 10 microM formate (P < 0.05), and 43.6 +/- 2.8 pmol.mm-1.min-1 (P < 0.001) with 1 mM luminal formate. Cl/base exchange was inhibited by luminal 4,4'-diisothiocyanostilbene-2,2'-disulfonic acid (DIDS) in the presence and absence of formate. In juxtamedullary PCT, Cl/base exchange was 22.2 +/- 3.8 without formate and 25.0 +/- 5.4 pmol.mm-1.min-1 in the presence of 1 mM luminal formate [P = not significant (NS)]. Cl/base exchange was inhibited by luminal DIDS in juxtamedullary PCT. The rates of Cl/base exchange in both superficial and juxtamedullary PCT were not affected by 0.1 mM acetazolamide and 2 mM cyanide and were the same in the presence and absence of HCO3/CO2, consistent with Cl/OH rather than Cl/HCO3 exchange. To examine the effect of formate on PCT transport, tubules were perfused with a high-Cl solution without organics simulating late proximal tubular fluid. In superficial PCT net volume absorption (JV) was 0.00 +/- 0.05 in the absence of formate and 0.14 +/- 0.06 nl.mm-1.min-1 in the presence of 1 mM formate (P < 0.05).(ABSTRACT TRUNCATED AT 250 WORDS)

A total of 75 jaundiced infants with gestational ages ranging from 37 to 42 weeks were studied during the first 10 days of age to evaluate renal function by measuring endogenous creatinine clearance (Ccr), fractional excretion of N-acetyl-beta-D-glucosaminidase (NAG) to creatinine, fractional excretion of sodium (FENa) and urine osmolality. All jaundiced infants were divided into two groups. Group 1 infants (n = 35) had total serum bilirubin levels ranging between 21 and 39.6 mg/dl (mean 27.2). Exchange transfusions were performed in all group 1 infants at the time of the initial study. Group 2 infants (n = 40), whose total serum bilirubin levels ranged between 12.3 and 20 mg/dl (mean 16.4), received phototherapy, except for 2. Conjugated bilirubin levels were less than 1.0 mg/dl in all these infants. Results were compared with 25 untreated control infants with corresponding gestational and postnatal ages. Follow-up studies were done in 27 of the 35 group 1 infants and in 32 of the 40 group 2 infants prior to hospital discharge, when total serum bilirubin levels had decreased to less than 10 mg/dl. Ccr, fractional excretion of NAG to creatinine, FENa and urine osmolality of group 1 infants were statistically significantly different when compared to those of group 2 and the control infants. The measured parameters in the post-treatment follow-up study of group 1 infants returned to near-normal levels when total serum bilirubin levels became normal. However, no significant differences were seen between group 2 and the control infants in any of the measured parameters.(ABSTRACT TRUNCATED AT 250 WORDS)

Between January 1990 and December 1991, serial real-time ultrasound examinations and analyses of urine were performed on a total of 50 infants with birth weights less than 1,500 g to assess the incidence of renal calcification. Five infants (10%) developed renal calcification at a mean age of 48.8 +/- 14.1 days. These 5 infants with renal calcification had significantly shorter gestations (28.2 +/- 0.8 vs. 30.1 +/- 1.7 weeks, p0.0005) and lower birth weights (934 +/- 45 vs. 1,311 +/- 188 g, p0.0005) when compared with infants without renal calcification. None of the affected infants were treated with furosemide. Affected infants had a mean urine volume of 85.8 +/- 11.3 ml/kg/24 h, mean urine calcium level of 5.07 +/- 1.18 mg/kg/24 h, mean urine calcium to creatinine (mg/mg) ratio of 0.67 +/- 0.09, and a mean urine N-acetyl-beta-D-glucosaminidase (NAG) to creatinine (U/g) ratio of 259 +/- 133. Urinalyses showed that affected infants had significantly higher urine pH values and hematuria. Alkaline phosphatase concentrations and initial parathyroid hormone levels were not different among the two groups. In summary, renal calcification occurred in 10% of very low birth weight infants and multiple risk factors seem to be contributory. The smaller, sicker and more immature infants appear to have increased risk for developing renal calcification.(ABSTRACT TRUNCATED AT 250 WORDS)

The onset of autosomal dominant polycystic kidney disease in infants and children is unusual, and renal involvement is typically bilateral. The presentation of a unilateral renal mass in such a disorder is extremely rare. We report a 2-month-old infant with autosomal dominant polycystic kidney disease presenting with unilateral renal involvement; the literature concerning this entity is reviewed.