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1.

Objectives

To determine the effect of association of dysembryogenesis (manifested by presence of dysmorphic markers) on the developmental profile of autistic children.

Methods

26 autistic children were classified into complex autism (if they had specific dysmorphic markers) or essential autism (in the absence of dysmorphic markers) using the Miles Autism Dysmorphology Measure (ADM). The developmental abilities (Griffith’s Mental Development Scales) and the clinical severity (Childhood Autism Rating Scale) of both groups were compared. The prevalence of dysmorphic markers was also determined in 140 non-autistic controls.

Results

Children with complex autism had poorer development (General Quotient 29.4 vs 34.0, P=0.06) and earlier onset of autistic symptoms (18 vs 24 mo, P=0.05). Dysmorphic markers were significantly more in autistic children compared to normal children (27% vs 10%, P=0.002).

Conclusions

Dysembryogenesis may contribute to the clinical heterogeneity of autistic children.
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2.

Objectives

To compare antenatal, natal and postnatal factors among children between 2–6 y of age with autism and a control group of normal children.

Methods

One hundred and forty three confirmed cases of 2–6 y-old children with autism, attending autism clinic of Child Development Centre, who had a CARS score of ≥?30 were included in the study. Two hundred normal children in the same age group were recruited from the well-baby/immunization clinic of SAT Hospital, Thiruvananthapuram. Data was collected using a structured pre-piloted questionnaire consisting of 21 antenatal, 8 natal and 6 postnatal risk factors.

Results

The multivariate analysis on antenatal, natal and postnatal possible risk factors for autism showed statistically significant high odds ratios for (i) excess fetal movement (OR?=?11.44; 95 % CI: 2.85 – 45.98); (ii) maternal respiratory infection/asthma (OR?=?6.11; 95 % CI: 1.56–24.02; (iii) maternal vaginal infection (OR?=?5.20; 95 % CI: 1.72 – 15.73); (iv) maternal hypothyroidism (OR?=?4.25; 95 % CI: 1.38–13.07) and (v) family history of neuro-developmental disorders (OR?=?2.90; 95 % CI: 1.72 – 4.88).

Conclusions

This case control study involving 143 children between 2 and 6 y with autism as per CARS criteria and a control group of 200 normal children has shown that excess fetal movement, maternal respiratory infection/asthma, maternal vaginal infection, maternal hypothyroidism and family history of neuro-developmental disorders are possible risk factors for autism.
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3.

Objective

To compare parent reported feeding difficulties and nutritional adequacy of children with Autism Spectrum Disorders (ASD) to an age and socio-economically matched group of typically developing children.

Methods

The scores on Children’s Eating Behavior Inventory (CEBI), three-day food records, anthropometric measures and adequacy of micro- and macro- nutrients were compared for 63 children diagnosed with ASD and 50 typically developing children enrolled from the department of pediatrics of a tertiary care teaching hospital from North India.

Results

The majority (79%) of the parents of ASD children reported some concern regarding their feeding behavior as compared to 64% of the parents of typically developing children. As compared to controls, ASD children had significantly higher CEBI scores (97.28 vs. 89.48, t = 3.15, P = 0.002) and more feeding problems (6.42 vs. 2.70, t = 3.74, P = 0.001). Relative to controls, ASD children consumed fewer number of food items (P = 0.022), particularly fruits (P = 0.004), vegetables (P = 0.011), and proteins (P = 0.015); had significantly lower daily intake of potassium (P = 0.001), copper (P = 0.007), and folate (P = 0.001). Although children with autism did not differ significantly from controls on intake of calories, height, weight, or body mass index, significantly greater proportion of ASD children failed to meet the estimated average requirement of thiamine (P = 0.039), vitamin C (P = 0.013), and copper (P = 0.005).

Conclusions

The findings underscore the need for comprehensive assessment and empirically-supported interventions for eating problems and dietary deficiencies found in ASD children.
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4.

Purpose

Simulation-based training has the potential to improve team-based care. We hypothesized that implementation of an in situ multidisciplinary simulation-based training program would improve provider confidence in team-based management of severely injured pediatric trauma patients.

Methods

An in situ multidisciplinary pediatric trauma simulation-based training program with structured debriefing was implemented at a free-standing children’s hospital. Trauma providers were anonymously surveyed 1 month before (pre-), 1 month after (post-), and 2 years after implementation.

Results

Survey response rate was 49% (n?=?93/190) pre-simulation, 22% (n?=?42/190) post-simulation, and 79% (n?=?150/190) at 2-year follow-up. These providers reported more anxiety (p?=?0.01) and less confidence (p?=?0.02) 1-month post-simulation. At 2-year follow-up, trained providers reported less anxiety (p?=?0.02) and greater confidence (p?=?0.01), compared to untrained providers.

Conclusions

Implementation of an in situ multidisciplinary pediatric trauma simulation-based training program may initially lead to increased anxiety, but long-term exposure may lead to greater confidence.

Level of evidence

II, Prospective cohort.
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5.

Objective

To investigate the changes in lymphocyte subsets that are caused by infection with different pathogens in children with hand, foot, and mouth disease.

Methods

T lymphocyte subsets were measured in the patients’ peripheral blood, and serum, throat swab, and fecal samples were tested for enterovirus.

Results

Fecal and throat swab samples exhibited similar positive detection rates, and were significantly more likely to be positive, compared to serum samples (P < 0.01). The EV71-positive group exhibited significantly lower CD4 + TM cell counts (QR: 1.058), compared to the CD4 + TM cell counts in the CoxA16-positive group (QR: 1.391; P < 0.05).

Conclusions

Throat swab and fecal samples exhibited significantly higher positive detection rates, compared to serum samples. In addition, EV71-infected children exhibited significantly lower CD4+ T-cell counts, compared to CoxA16-infected children, which suggests that EV71 infection may be associated with a poorer prognosis.
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6.

Purpose

To assess the indications, safety and outcomes of tunneled central venous catheters (CVCs) placed via a cutdown approach into the axillary vein in children, an approach not well described in this population.

Methods

A retrospective cohort study was performed on pediatric patients who received CVCs via open cannulation of the axillary vein or one of its tributaries between January 2006 and October 2016 at two hospitals.

Results

A total of 24 axillary CVCs were placed in 20 patients [10 male (42%); mean weight 7.0 kg (SD 2.9); mean age 10 months (SD 6)]. The most common indications for axillary vein access included neck or chest wall challenges (tracheostomies or chest wall wounds) (n = 18). The median duration of line placement was 140 days (IQR 146). The most common indications for removal were completion of therapy (n = 7, 39%) and infection (n = 5, 28%). There were no early complications. Long-term complications included infection (n = 5) or catheter malfunction (n = 3).

Conclusions

Tunneled CVC placement via a cutdown approach into the axillary vein or its tributary can be an effective alternative approach to obtain long-term vascular access in children. Outcomes may be comparable to lines placed in traditional internal jugular and subclavian vein locations.
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7.

Background

Functional constipation is a clinical problem with an incompletely understood etiology. Functional bowel diseases have been shown to be related to inflammation in many studies in adults. In this study, we aimed to evaluate leukocytes, C-reactive protein, proinflammatory and anti-inflammatory cytokines, and neopterin levels in children with functional constipation.

Methods

Seventy-six children with constipation and 71 healthy controls (mean age 7.12 ± 3.46 years and 7.32 ± 4.33 years, respectively, P = 0.991) were included in the study. Leukocytes, C-reactive protein, interleukin (IL)-1β, IL-6, IL-10, IL-12, tumor necrosis factor-alpha (TNF-α) and neopterin levels were assessed in patients and healthy controls. Parameters were measured in the serum using enzyme-linked immunosorbent assay methods.

Results

Mean IL-6 (20.31 ± 12.05 vs. 16.2 ± 10.25 pg/mL, respectively, P = 0.003), IL-12 (181.42 ± 133.45 vs. 135.6 ± 83.67 pg/mL, respectively, P = 0.018) and neopterin levels (2.08 ± 1.12 vs. 1.52 ± 1.02 pg/mL, respectively, P = 0.001) were significantly higher in constipated children than healthy controls. Leukocyte and thrombocyte counts, C-reactive protein, and IL-1β, IL-10 and TNF-α levels did not show any difference between the two groups.

Conclusions

In this study, IL-6, IL-12 and neopterin levels of constipated patients were found to be higher than those of controls. These results indicate the presence of subclinical inflammation in children with functional constipation.
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8.

Objectives

To assess and compare the Quality of Life (QOL) of children with beta-thalassemia major on regular transfusion therapy with normal children, and of the caregivers of children with beta-thalassemia major to that of caregivers of normal children.

Methods

A cross-sectional comparison of QOL in 75 thalassemic and 80 non-thalassemic children was conducted using the PedsQL? 4.0 generic core scale. Also self-rated health was assessed in their caregivers using Short Form-36 Health Survey.

Results

The total QOL score according to child-self report [83.7 (10.8) vs. 97.6 (3.3); p < 0.001] and parent-proxy report [84.2 (11.9) vs. 96.7 (3.5); p < 0.001] was significantly lower in cases as compared with controls. It was found that a significantly higher proportion of caregivers of cases reported poor health compared with caregivers of controls (29.2% vs. 2.5%, p < 0.001). Even after adjusting for age, sex, socio-economic status, and total QOL score by the parent, it was found that caregivers of thalassemic children were significantly more likely to report poor health compared with those of controls (odds ratio: 15.8, 95% confidence intervals: 2.8–89.9).

Conclusions

Health Related QOL is significantly affected in children with beta-thalassemia major on regular transfusion across all age groups, gender and socio-economic classes and also in their caregivers.
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9.

Objective

To identify risk factors for microbiologically confirmed intrathoracic tuberculosis in children.

Methods

Children, 6 mo to 15 y of age, attending the out-patient department of a tertiary care centre in India, with probable intrathoracic tuberculosis were enrolled. Microbiological confirmation of tuberculosis was defined as positivity on smear (Ziehl-Neelsen staining) and/or Xpert MTB/RIF and/or MGIT-960 culture. Association of various factors with microbiological confirmation were assessed by univariate and multivariate analysis.

Results

Microbiologic confirmation was documented in 39 (25%) of 153 patients enrolled. On univariate analysis, microbiological positivity was associated with female gender, higher mean (SD) age [136.6 (31.8) vs. 117.3 (41.4) mo], parenchymal lesion on chest radiograph, low body mass index for age, having symptoms of cough and weight loss, lower mean (SD) hemoglobin [10.4 (1.37) g/dl vs. 11(1.52) g/dl; p = 0.04], and higher mean (SD) monocyte: lymphocyte ratio [0.38 (0.30) vs. 0.24 (0.02); p = 0.37]. Higher proportion of microbiologically negative children were BCG vaccinated (95% vs. 79%; p = 0.002). On multivariate analysis, microbiological positivity showed significant association with low body mass index for age (p = 0.033) and higher monocyte: lymphocyte ratio (p = 0.037).

Conclusions

Low body mass index for age and higher monocyte: lymphocyte ratios were associated with microbiological confirmation in children with intrathoracic tuberculosis.
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10.

Objective

To assess the effect of monotherapy with Carbamazepine (CBZ) and Sodium valproate (VPA) on serum 25-OH vitamin D levels in children with epilepsy compared to controls.

Design

Cross-sectional study.

Setting

Outpatient department of a tertiary-care Pediatric Neurology centre, and a nearby day-care centre and school.

Study period

June 2012 to May 2013

Participants

Children with epilepsy aged 2 to 13 years on monotherapy with CBZ (n=28) or VPA (n=28) for at least 6 months; 109 age-matched controls from a nearby day-care centre and school.

Results

The median (IQR) values of 25 (OH) vitamin D was 18.0 ng/mL (13.7-27.3), 21.35 ng/mL (16.4 -25.2) and 30.5 ng/mL (19.1-43.7) in CBZ, VPA and control group, respectively (P=0.008). 60.7% of patients in CBZ group and 35.7 % in VPA group had low 25 (OH) D levels (<20 ng/mL) compared to 27.8% in controls (P=0.001).The serum alkaline phosphatase level was higher in children on carbamazepine therapy (P=0.001) than controls.

Conclusion

This study identifies significant risk of vitamin D deficiency in ambulant children with epilepsy on monotherapy with CBZ or VPA.
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11.

Objective

To investigate vitamin D levels in patients with recurrent wheeze at early ages of childhood.

Methods

In the present cross-sectional study, serum 25-hydroxy vitamin D [25 (OH)D], levels which is known as an indicator of vitamin D adequacy, was examined in infants with three or more wheezing attacks.

Results

A total of 186 infants with recurrent wheezing were included in the study along with 118 healthy control peers. The recurrent wheezing study participants were classified into two groups according to Asthma Predictive Index (API) positivity and compared to control subjects regarding their serum vitamin D status. The API negative group had the lowest mean serum 25 (OH)D level (n = 121; 22.71 ± 10.76 ng/ml) followed by API positive group (n = 65; 24.08 ± 9.02 ng/ml) compared to healthy group (26.24 ± 11.88 ng/ml) (p < 0.05). In addition, higher vitamin D deficiency was observed in infants in API negative group (52.1 %; p < 0.01) and API positive group (38.5 %; p < 0.05) than control group (31.4 %).

Conclusions

Low levels of 25 (OH)D were detected in infants with two different phenotypes of recurrent wheeze. Vitamin D deficiency may play a role in the pathogenesis of infants with recurrent wheezing.
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12.

Purpose

Recently, genetic markers within a locus on 7q21.11 containing the SEMA3A, SEMA3C, and SEMA3D genes were reported to be associated with Hirschsprung disease (HSCR). Here, we investigated three polymorphisms, rs1583147, rs12707682, and rs11766001, at this locus to determine their potential contributions to the susceptibility of Indonesian HSCR patients.

Methods

Three variants were analyzed in 60 non-syndromic HSCR patients and 118 ethnicity-matched controls for association studies by genotyping.

Results

The risk allele frequencies of SEMA3 rs12707682 (allele C) and rs1583147 (allele T) is higher in cases, 53 and 23 %, than in controls, at 42 and 13 %, respectively. However, these frequency differences were not statistically significant with p value of 0.06 and 0.023, respectively. These findings were consistent with transmission disequilibrium test results with p values of 0.041 and 0.11 for rs12707682 and rs1583147, respectively. Furthermore, the frequencies of SEMA3 rs11766001 risk allele in HSCR cases and controls were 1.7 and 0.8 %, respectively.

Conclusions

SEMA3 rs12707682 and rs1583147 variants are not common risk factors for HSCR in Indonesia. The rarity of the SEMA3 rs11766001 polymorphism in Indonesian population might be due to a founder effect.
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13.

Purpose

Pediatric surgeons often care for children with ovarian tumors. Few studies report long-term outcomes for these patients. This study characterizes intermediate-term results for patients who underwent surgical resection of ovarian neoplasms as children.

Methods

Patients who underwent surgery for ovarian neoplasms at a children’s hospital were identified. They were invited to participate in a telephone-based survey assessing post-surgical recurrence, dysmenorrhea, quality of life, and fertility.

Results

188 patients were identified; 79 met criteria. 31 patients had ovarian-sparing tumor resection; 48 had oophorectomy; five had recurrences. 56 were successfully interviewed at a median follow-up of 4.6 years. Dysmenorrhea rates of 52 and 78 % were reported (p = 0.07), respectively. Two patients suffered from infertility. Quality of life was generally reported as good.

Conclusion

Intermediate outcomes are good for patients who underwent ovarian-sparing tumor resection or oophorectomy for pediatric ovarian tumors. Additional long-term monitoring would be beneficial to better assess fertility and dysmenorrhea outcomes.
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14.

Purpose

Despite significant radiation exposure involved with computed tomography (CT) in evaluation of pediatric appendicitis, its use is still widespread. The goal of this study was to assess the effect of a staged imaging pathway for appendicitis to significantly decrease CT use while maintaining diagnostic accuracy.

Methods

Chart review was performed for patients evaluated for appendicitis over a 12-month period prior to and after pathway implementation.

Results

There was a significant decrease in CT use as initial imaging after implementation of the pathway; 87.1–13.4 % for evaluations positive for appendicitis (decrease 84.6 %, p < 0.0001) and 82.6–9.2 % for evaluations negative for appendicitis (decrease 88.9 %, p < 0.0001). Use of CT during any point in the evaluation decreased from 91.7 to 25.1 % (decrease 72.6 %, p < 0.0001). The negative appendectomy rate was similar; 5.4 % prior, 4.9 % post (p = 0.955). The missed appendicitis rate did not statistically change; 1.1 % prior, 3.7 % post (p = 0.523). The perforation rate was not statistically altered; 6.5 % prior; 9.8 % post (p = 0.421). 350 less patients underwent CT during the year following the pathway.

Conclusions

The staged imaging pathway resulted in a marked decrease in children exposed to CT without compromising diagnostic accuracy.
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15.

Purpose

The diagnosis of pediatric appendicitis is still a challenge, resulting in perforation and negative appendectomies. The aim of this study was to evaluate novel biomarkers in urine and to use the most promising biomarkers in conjunction with the Pediatric Appendicitis Score (PAS), to see whether this could improve the accuracy of diagnosing appendicitis.

Methods

A prospective study of children with suspected appendicitis was conducted with assessment of PAS, routine blood tests, and measurements of four novel urinary biomarkers: leucine-rich α-2-glycoprotein (LRG), calprotectin, interleukin 6 (IL-6), and substance P. The biomarkers were blindly determined with commercial ELISAs. Urine creatinine was used to adjust for dehydration. The diagnosis of appendicitis was based on histopathological analysis.

Results

Forty-four children with suspected appendicitis were included, of which twenty-two (50 %) had confirmed appendicitis. LRG in urine was elevated in children with appendicitis compared to children without (p < 0.001), and was higher in children with gangrenous and perforated appendicitis compared to those with phlegmonous appendicitis (p = 0.003). No statistical significances between groups were found for calprotectin, IL-6 or substance P. LRG had a receiver operating characteristic area under the curve of 0.86 (95 % CI 0.79–0.99), and a better diagnostic performance than all routine blood tests. LRG in conjunction with PAS showed 95 % sensitivity, 90 % specificity, 91 % positive predictive value, and 95 % negative predictive value.

Conclusion

LRG, adjusted for dehydration, is a promising novel urinary biomarker for appendicitis in children. LRG in combination with PAS has a high diagnostic performance.
  相似文献   

16.

Objective

To document the diagnostic accuracy of the Childhood Autism Scale (CARS) thresholds to identify mild, moderate and severe autism in India.

Methods

The CARS scores of 623 children, with and without autism were compared against the Diagnostic and Statistical Manual for Mental Disorders 4th edition (DSM-IV-TR) for ASD diagnosis and clinical consensus between two developmental paediatricians as the reference standard for autism severity using the Receiver operating characteristics (ROC) curve analyses and contingency tables.

Results

The CARS total score for mild, moderate and severe autism ranged from 30.5 to 35, 35.5–40 and ≥40.5 respectively in this study. The overall diagnostic accuracy of CARS total score in the mild range was moderate [AUC?=?0.68 (95%CI?=?0.62–0.88), z?=?1.34; P?=?0.18], moderate range was high [AUC?=?0.90 (95%CI?=?0.77–0.97), z?=?8.62; P?=?0.0001] and severe range was also high [AUC?=?0.85 (95%CI?=?0.77–0.90), z?=?7.09; P?=?0.0001].

Conclusions

There are validated severity scores for Childhood Autism Rating Scale for clinical and research use in India.
  相似文献   

17.

Purpose

Hirschsprung’s disease-associated enterocolitis (HAEC) is the most common cause of morbidity and mortality in Hirschsprung’s disease (HSCR). Altered intestinal epithelial barrier function is implicated in the pathogenesis of HAEC. IL-17 is a proinflammatory cytokine that plays a crucial role in host defense against microbial organisms in the development of inflammatory diseases. Act1 is an essential adaptor molecule required for the IL-17-mediated inflammatory responses via interaction with IL-17 receptor (IL-17R). We designed this study to investigate the hypothesis that Act1/Il-17R expression is upregulated in HSCR.

Methods

We investigated Act1 and IL17R expression in ganglionic andaganglionic bowel of HD patients (n = 10) and controls (n = 10). qPCR, Western blotting and confocal immunofluorescence were performed.

Main results

qPCR and Western blot analysis revealed that Act1 and IL17R are strongly expressed in the aganglionic and ganglionic colon of patients with HSCR. Act1 and IL17R expression was significantly increased in HSCR specimens compared to controls (p < 0.05). Confocal microscopy revealed a markedly increased expression of Act1 and IL17R in the colonic epithelium of patients with HSCR compared to controls.

Conclusion

To our knowledge, we report, for the first time, the expression of Act1 in the human colon. The increased expression of Act1 and Il-17 in the aganglionic and ganglionic bowel in HSCR may result in IL-17-mediated increased inflammatory response leading to the development of HAEC
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18.

Purpose

To investigate and compare the outcomes after tubularized incised plate (TIP) urethroplasty in mid-shaft and proximal hypospadias using a standard and a modified technique.

Methods

We conducted a retrospective study in 104 consecutive children who underwent mid-shaft or proximal TIP repairs from Jan 2007 to Sept 2015. Patients in Cohort One had dorsal dartos (DD) neourethral coverage while patients in Cohort Two had either de-epithelialized split preputial (DESP) or tunica vaginalis (TV) flap coverage. TV flap was used only when DESP flap was not sufficient to cover the neourethra.

Results

There were 52 patients each in Cohort One (DD, n = 52) and Cohort Two (DESP, n = 38; TV, n = 14) with no difference in ratio of mid-shaft/proximal between the two cohorts. At a median follow-up of 28 months, 36 patients (34.6 %) developed 47 complications including fistula (n = 19; 18.3 %) and neourethral dehiscence (n = 4; 3.8 %). Cohort One patients had significantly more fistula (28.8 vs 7.7 %; p = 0.005) and neourethral dehiscence (7.7 vs 0 %; p = 0.04) than Cohort Two. There was no difference between the two cohorts in the complication rates of meatal stenosis, recurrent ventral curvature and neourethral stricture.

Conclusions

Both DESP and TV flap appear to be superior to DD in preventing fistula and neourethral dehiscence in non-distal TIP repairs.
  相似文献   

19.

Purpose

Pancreatic neoplasms are uncommon in children. This study sought to analyze the clinical and pathological features of surgically resected pancreatic tumors in children and discuss management strategies.

Methods

We conducted a retrospective review of patients ≤21 years with pancreatic neoplasms who underwent surgery at a single institution between 1995 and 2015.

Results

Nineteen patients were identified with a median age at operation of 16.6 years (IQR 13.5–18.9). The most common histology was solid pseudopapillary neoplasm (SPN) (n = 13), followed by pancreatic neuroendocrine tumor (n = 3), serous cystadenoma (n = 2) and pancreatoblastoma (n = 1). Operative procedures included formal pancreatectomy (n = 17), enucleation (n = 1) and central pancreatectomy (n = 1). SPNs were noninvasive in all but one case with perineural, vascular and lymph node involvement. Seventeen patients (89.5 %) are currently alive and disease free at a median follow-up of 5.7 (IQR 3.7–10.9) years. Two patients died: one with metastatic insulinoma and another with SPN who developed peritoneal carcinomatosis secondary to a concurrent rectal adenocarcinoma.

Conclusions

Pediatric pancreatic tumors are a heterogeneous group of neoplastic lesions for which surgery can be curative. SPN is the most common histology, is characterized by low malignant potential and in selected cases can be safely and effectively treated with a tissue-sparing resection and minimally invasive approach.
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20.

Objective

To assess the dynamics of the onset of antipyretic efficacy of intravenous (IV) acetaminophen vs. oral (PO) acetaminophen in the management of fever in children.

Methods

This observational single-dose study was conducted at Department of Pedriatrics, Army Hospital (Research and Referral), a multispecialty tertiary care center in New Delhi in fever patients to assess the antipyretic efficacy of IV acetaminophen 15 mg/kg/dose vs. PO acetaminophen 15 mg/kg/dose over 6 h. Subjects were randomly assigned to receive either IV acetaminophen (n = 200) or PO acetaminophen (n = 200).

Results

Demographics and baseline characteristics were similar between the two groups and were normally distributed. Allergic reaction was found in 7 (3.5%) patients in IV acetaminophen group and was absent in PO acetaminophen group. Onset of constipation and dry mouth was found in 8 patients (4%) in IV acetaminophen group and was absent in PO acetaminophen group. Additional dose was required in 6 patients (3%) in intravenous acetaminophen group and 10 patients (5%) in oral acetaminophen group respectively. Statistically significant differences in the rate of fall in temperature through 180 min were observed in favor of the IV acetaminophen group when compared to those receiving PO acetaminophen.

Conclusions

A single dose of intravenous acetaminophen is safe and effective in reducing fever where patients are unable to tolerate oral administration or when rapid reduction of temperature is desirable.
  相似文献   

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