The aim of this retrospective study was to evaluate the efficacy and safety of weekly high-dose 5-fluorouracil (5-FU)/folinic acid (FA) as 24-h infusion (AIO regimen) plus irinotecan in patients with histologically proven metastatic gastroesophageal adenocarcinoma (UICC stage IV).
From 08/1999 to 12/2008, 76 registered, previously untreated patients were evaluable. Treatment regimen: irinotecan (80 mg/m2) as 1-h infusion followed by 5-FU (2000 mg/m2) combined with FA (500 mg/m2) as 24-h infusion (d1, 8, 15, 22, 29, 36, qd 57).
Median age: 59 years; male/female: 74%/26%; ECOG ≤1: 83%; response: CR: 1%, PR: 16%, SD: 61%, PD: 17%, not evaluable in terms of response: 5%; tumor control: 78%; median OS: 11.2 months; median time-to-progression: 5.3 months; 1-year survival rate: 49%; 2-year survival rate: 17%; no evidence of disease: 6.6%; higher grade toxicities (grade 3/4): anemia: 7%, leucopenia: 1%, ascites: 3%, nausea: 3%, infections: 12%, vomiting: 9%, GI bleeding of the primary tumor: 4%, diarrhea: 17%, thromboembolic events: 4%; secondary metastatic resection after downsizing: 16 patients (21%), R-classification of secondary resections: R0/R1/R2: 81%/6%/13%, median survival of the 16 patients with secondary resection: 23.7 months.
Combined 5-FU/FA as 24-h infusion plus irinotecan may be considered as an active palliative first-line treatment accompanied by tolerable toxicity; thus offering an alternative to cisplatin-based treatment regimens. Thanks to efficient interdisciplinary teamwork, secondary metastatic resections could be performed in 16 patients. In total, the patients who had undergone secondary resection had a median survival of 23.7 months, whereas the median survival of patients without secondary resection was 10.1 months (p≤0.001).
gastroesophageal cancer; palliative chemotherapy; irinotecan; 5-fluorouracil
Community-acquired pneumonia (CAP) is a common cause of pediatric admission to hospital. The objectives of this study were twofold: 1) to describe the clinical characteristics of CAP in children admitted to a tertiary care pediatric hospital in the pneumococcal vaccination era and, 2) to examine the antimicrobial selection in hospital and on discharge.
A retrospective review of healthy immunocompetent children admitted to a tertiary pediatric hospital from January 2007 to December 2008 with clinical features consistent with pneumonia and a radiographically-confirmed consolidation was performed. Clinical, microbiological and antimicrobial data were collected.
One hundred and thirty-five hospitalized children with pneumonia were evaluated. Mean age at admission was 4.8 years (range 0–17 years). Two thirds of patients had been seen by a physician in the 24 hours prior to presentation; 56 (41.5%) were on antimicrobials at admission. 52 (38.5%) of patients developed an effusion, and 22/52 (42.3%) had pleural fluid sampled. Of 117 children who had specimens (blood/pleural fluid) cultured, 9 (7.7%) had pathogens identified (7 Streptococcus pneumoniae, 1 Group A Streptococcus, and 1 Rhodococcus). 55% of patients received 2 or more antimicrobials in hospital. Cephalosporins were given to 130 patients (96.1%) in hospital. Only 21/126 patients (16.7%) were discharged on amoxicillin. The median length of stay was 3 days (IQR 2–4) for those without effusion and 9 (IQR 5–13) for those with effusion. No deaths were related to pneumonia.
This study provides comprehensive data on the clinical characteristics of hospitalized children with CAP in the pneumococcal 7-valent vaccine era. Empiric antimicrobial choice at our institution is variable, highlighting a need for heightened antimicrobial stewardship.
The availability of a simple, sensitive, and rapid test using whole blood to facilitate processing and to
reduce the turnaround time could improve the management of patients presenting with
chest pain. The aim of this study was an evaluation of the Innotrac Aio! second-generation
cardiac troponin I (cTnI) assay. The Innotrac Aio! second-generation cTnI assay was
compared with the Abbott AxSYM first-generation cTnI, Beckman Access AccuTnI, and Innotrac
Aio! first-generation cTnI assays. We studied serum samples from 15 patients with positive
rheumatoid factor but with no indication of myocardial infarction (MI). Additionally, the stability
of the sample with different matrices and the influence of hemodialysis on the cTnI
concentration were evaluated. Within-assay CVs were 3.2%–10.9%, and
between-assay precision ranged from 4.0% to 17.2% for cTnI. The functional sensitivity
(CV = 20 %) and the concentration giving CV of 10% were approximated to be 0.02 and 0.04,
respectively. The assay was found to be linear within the tested range of 0.063–111.6
μ g/L. The correlations between the second-generation Innotrac Aio!, Access,
and AxSYM cTnI assays were good (r coefficients 0.947–0.966), but
involved differences in the measured
concentrations, and the biases were highest with cTnI at low concentrations. The
second-generation Innotrac Aio! cTnI assay was found to be superior to the first-generation assay
with regard to precision in the low concentration range. The stability of the cTnI level was best in the
serum, lithium-heparin plasma, and lithium-heparin whole blood samples (n = 10 , decrease
< 10 % in 24 hours at +20°C and at +4°C.
There was no remarkable influence of hemodialysis on the cTnI release. False-positive
cTnI values occurred in the presence of very high rheumatoid factor values, that is, over 3000 U/L.
The 99th percentile of the apparently healthy reference group was ≤ 0.03
μ g/L. The results demonstrate the very good analytical performance of the second-generation
Innotrac Aio! cTnI assay.
To assess inter-observer reliability of the identification of episodes of the systemic inflammatory response syndrome (SIRS) in critically ill hospitalized infants and children.
Retrospective cross-sectional study of the application of the 2005 consensus definition of SIRS in infants and children by two independent trained reviewers using information in the electronic medical record.
18-bed pediatric multidisciplinary medical/surgical pediatric intensive care unit (PICU).
A randomly selected sample of children admitted consecutively to the PICU between May 1 and September 30, 2009.
Measurements and Main Results
60 infants and children were selected from a total of 343 admitted patients. Their median age was 3.9 years (interquartile range [IQR], 1.5–12.7), 57% were female, and 68% were Caucasian. 19 (32%) children were identified by both reviewers as having an episode of SIRS (88% agreement, 95% confidence interval [CI] 78–94; κ = 0.75, 95% CI, 0.59–0.92). Amongst those 19 children, agreement between the reviewers for individual SIRS criteria was: temperature (84%, 95% CI 60–97); white blood cell count (89%, 95% CI 67–99); respiratory rate (84%, 95% CI 60–97); heart rate (68%, 95% CI 33–87).
Episodes of SIRS in critically ill infants and children can be identified reproducibly using the consensus definition.
systemic inflammatory response syndrome; inflammation; sepsis; intensive care units; pediatric; reproducibility; reliability
Starship Children's Hospital in Auckland, New Zealand, serves a population of 1.2 million people and is a tertiary institution for pediatric trauma. This study is designed to review all cases of abdominal injury (blunt and penetrating) that resulted in injury of a hollow abdominal viscus including the stomach, duodenum, small intestine, large intestine and urinary bladder. The mechanism of injury; diagnosis and outcome were studied. This was done by retrospective chart review of patients admitted from January 1995 to December 2001. Thirty two injuries were found in 29 children. The age ranged from 7 months to 15 years with boys represented more commonly. Small bowel was the most frequently injured hollow viscus. Computerized Tomography (CT scan) is an extremely useful tool for the diagnosis of HVI.
There is an ongoing debate regarding the optimal surgical management for pilonidal disease in the pediatric population. The purpose of this study was to evaluate a pediatric surgeon’s experience at a Canadian children’s hospital over 35 years.
We performed a retrospective review of the charts of patients seen and treated from July 1969 to December 2003, inclusive. All patients were evaluated for age, sex, clinical diagnosis, infection, treatment, healing time, complications and results.
In all, 121 adolescents with pilonidal disease (64 boys, 57 girls) with a mean age of 15 (range 12–19) years were evaluated at the same children’s hospital. The 107 (88%) patients with infection (46% acute) underwent surgery. At operation, all 107 pilonidal cysts were either excised and packed open, marsupialized or excised and closed primarily without drainage under general anesthesia; the operation performed was arbitrarily chosen. Vacuum-assisted closure was not used. All patients received antibiotics. The time for healing after the initial operation in the group whose cysts were excised and packed open was at least twice as long (75 d) as in the other 2 groups (p = 0.031). Disease recurred in 24 (22%) patients, 6 (25%) of whom experienced 2 recurrences. Among the 90 patients in the excised and packed open group, 20 (22%) experienced recurrences and 5 (25%) experienced 2 recurrences. Among the 13 patients in the marsupialized group, 3 (23%) experienced recurrences and 1 (33%) experienced 2 recurrences. Among the 4 patients in the excised and closed primarily without drainage group, 1 (25%) experienced a recurrence and none experienced 2 recurrences (p = 0.12). Each recurrence was smaller than the original. All wounds eventually healed. There were no other complications and no deaths. A multivariable logistic regression analysis revealed that the type of surgical approach was not predictive of recurrence after controlling for age and sex.
Age, sex and surgical approach were not predictive of recurrence. From our experience, excision and packing open the wound produced a longer morbidity but offered the same results compared with marsupialization or excision and primary closure without drainage.
In a 20-year period at the Los Angeles Children's Hospital, 46 infants and children have had operation for cysts within the abdomen. The age range of patients was from newborn to 13 years. Most of them were under four years old. There were four general groups of these cysts. (1) About one-half were cysts of the ovary, some of them serous and some dermoid. These cysts are attached by a stalk that often twists, causing gangrene or rupture with acute symptoms simulating appendicitis. (2) Next in frequency were cysts arising in the mesentery of the intestine. They usually caused little trouble until by their size (up to a 2-quart capacity) they created pressure and obstruction in the intestine. (3) Enteric cysts were found in four patients. (4) Cysts of the pancreas were present in three of the children.
X-ray examination was helpful in diagnosis. Usually the type of cyst was not determined until operation was done. Transection of the intestinal tract sometimes was necessary for removal of the cyst. Surgical correction was satisfactory in 44 of the 46 cases.
The arsenite oxidase (Aio) from the facultative autotrophic Alphaproteobacterium Rhizobium sp. NT-26 is a bioenergetic enzyme involved in the oxidation of arsenite to arsenate. The enzyme from the distantly related heterotroph, Alcaligenes faecalis, which is thought to oxidise arsenite for detoxification, consists of a large α subunit (AioA) with bis-molybdopterin guanine dinucleotide at its active site and a 3Fe-4S cluster, and a small β subunit (AioB) which contains a Rieske 2Fe-2S cluster. The successful heterologous expression of the NT-26 Aio in Escherichia coli has resulted in the solution of its crystal structure. The NT-26 Aio, a heterotetramer, shares high overall similarity to the heterodimeric arsenite oxidase from A. faecalis but there are striking differences in the structure surrounding the Rieske 2Fe-2S cluster which we demonstrate explains the difference in the observed redox potentials (+225 mV vs. +130/160 mV, respectively). A combination of site-directed mutagenesis and electron paramagnetic resonance was used to explore the differences observed in the structure and redox properties of the Rieske cluster. In the NT-26 AioB the substitution of a serine (S126 in NT-26) for a threonine as in the A. faecalis AioB explains a −20 mV decrease in redox potential. The disulphide bridge in the A. faecalis AioB which is conserved in other betaproteobacterial AioB subunits and the Rieske subunit of the cytochrome bc1 complex is absent in the NT-26 AioB subunit. The introduction of a disulphide bridge had no effect on Aio activity or protein stability but resulted in a decrease in the redox potential of the cluster. These results are in conflict with previous data on the betaproteobacterial AioB subunit and the Rieske of the bc1 complex where removal of the disulphide bridge had no effect on the redox potential of the former but a decrease in cluster stability was observed in the latter.
Background: Obstructive sleep apnea syndrome (OSA) is a frequent disorder in children. The clinical characteristics of OSA in very young children under 2 years of age, and more particularly, in those born prematurely, and who have respiratory complications such as bronchopulmonary dysplasia (BPD), are not well defined. We therefore retrospectively reviewed our experience in a group of preterm infants with OSAS. Methods: The records of premature infants with BPD followed in the Pediatric Pulmonary Clinic at the University of Chicago who were diagnosed with OSA from 2004 to 2009 were reviewed and analyzed. Results: Twelve children, eight males, and four females with a mean gestational age of 27 weeks were found to have OSA. Mean age at diagnosis was 19 months. Inability to wean nighttime oxygen, the need to resume oxygen after intercurrent respiratory illness, and snoring were the most common presenting symptoms. The apnea–hypopnea index ranged from 1 to 120/h total sleep time (TST; mean: 29). SpO2 nadir ranged from 50 to 91%. Despite adenotonsillectomy (AT), all children had persistent sleep disordered breathing. Conclusion: In preterm infants, while snoring is a frequent symptom, poor weight gain, and inability to wean nighttime oxygen may indicate the need for further investigation for OSA. In the former preterm infant structural changes in the airway may play an important role along with adenotonsillar hypertrophy. A high level of clinical awareness is required to identify OSA in the formerly preterm infant.
obstructive sleep apnea; premature infant; lung disease; tonsillectomy
Although significant advances have been made in the treatment of advanced/metastatic colorectal cancer, 5-fluorouracil (5-FU) still forms the basis of chemotherapy. Recently, new 5-FU schedules and novel fluoropyrimidines have been developed, but there are no trials directly comparing these regimens. The current review describes the mechanisms of action, pre-clinical and phase I/II studies of two oral fluoropyrimidine therapies, capecitabine and uracil with tegafur plus leucovorin. It also compares the phase III studies of these agents with those of the two most popular infusional 5-FU-based regimens: de Gramont and German AIO (The Association of Medical Oncology (AIO) of the German Cancer Society). Both oral and infusional regimens demonstrated similar survival to the Mayo Clinic regimen, a standard treatment for colorectal cancer. Therefore, other endpoints must be examined to decide optimum therapy, including response rates, time to disease progression, tolerability and patients' convenience. All four new therapies demonstrated superior safety profiles compared with the Mayo Clinic regimen. However the uracil with tegafur plus leucovorin regimen was associated with severe diarrhoea and capecitabine with hand–foot syndrome. Patients will not sacrifice efficacy for the convenience of oral therapy alone, therefore the fact that capecitabine achieved superior response rates and equivalent time to disease progression compared with the Mayo Clinic regimen, while uracil with tegafur plus leucovorin produced lower response rates and significantly inferior time to disease progression, is highly relevant in choosing treatment.
British Journal of Cancer (2002) 86, 1670–1676. doi:10.1038/sj.bjc.6600341 www.bjcancer.com
© 2002 Cancer Research UK
colorectal cancer; 5-fluoropyrimidine; capecitabine; chemotherapy; oral; intravenous infusions; UFT; tegafur
Several randomized trials have indicated that combination chemotherapy applied in metastatic colorectal cancer (mCRC) does not significantly improve overall survival when compared to the sequential use of cytotoxic agents (CAIRO, MRC Focus, FFCD 2000-05). The present study investigates the question whether this statement holds true also for bevacizumab-based first-line treatment including escalation- and de-escalation strategies.
The AIO KRK 0110/ML22011 trial is a two-arm, multicenter, open-label randomized phase III trial comparing the efficacy and safety of capecitabine plus bevacizumab (Cape-Bev) versus capecitabine plus irinotecan plus bevacizumab (CAPIRI-Bev) in the first-line treatment of metastatic colorectal cancer. Patients with unresectable metastatic colorectal cancer, Eastern Cooperative Oncology Group (ECOG) performance status 0-1, will be assigned in a 1:1 ratio to receive either capecitabine 1250 mg/m2 bid for 14d (d1-14) plus bevacizumab 7.5 mg/kg (d1) q3w (Arm A) or capecitabine 800 mg/m2 BID for 14d (d1-14), irinotecan 200 mg/m2 (d1) and bevacizumab 7.5 mg/kg (d1) q3w (Arm B). Patients included into this trial are required to consent to the analysis of tumour tissue and blood for translational investigations. In Arm A, treatment escalation from Cape-Bev to CAPIRI-Bev is recommended in case of progressive disease (PD). In Arm B, de-escalation from CAPIRI-Bev to Cape-Bev is possible after 6 months of treatment or in case of irinotecan-associated toxicity. Re-escalation to CAPIRI-Bev after PD is possible. The primary endpoint is time to failure of strategy (TFS). Secondary endpoints are overall response rate (ORR), overall survival, progression-free survival, safety and quality of life.
The AIO KRK 0110 trial is designed for patients with disseminated, but asymptomatic mCRC who are not potential candidates for surgical resection of metastasis. Two bevacizumab-based strategies are compared: one starting as single-agent chemotherapy (Cape-Bev) allowing escalation to CAPIRI-Bev and another starting with combination chemotherapy (CAPIRI-Bev) and allowing de-escalation to Cape-Bev and subsequent re-escalation if necessary.
ClinicalTrials.gov Identifier NCT01249638
Although trauma is the primary cause of death in children, few reports or series exist regarding the management of traumatic aortic disruption in the pediatric age group. The clinical outcome in children diagnosed with acute aortic disruption may be directly influenced by diagnostic and therapeutic management decisions.
We reviewed the clinical course of 3 consecutive pediatric patients (mean age, 10.0 years; range, 4–16 years) admitted to our institution from January 2002 through May 2003 with the diagnosis of acute aortic disruption due to blunt trauma. In each case, the cause was a motor vehicle accident. Major, concomitant injuries involving other organ systems were present in each patient. Our operative goals were to use primary repair techniques, avoid the use of endovascular stent grafts, and use partial left heart bypass during aortic cross-clamping whenever possible.
Each patient underwent successful operative repair. Aortic reconstruction techniques included primary suture repair in the 4-year-old patient, patch angioplasty in the 16-year-old, and placement of an interposition conduit in the 10-year-old for a blow-out type aortic injury. All patients received partial left heart bypass during aortic cross-clamping (mean, 36.6 min; range, 27–50 min), via a centrifugal pump, and anticoagulation. All patients recovered without evidence of adverse neurologic sequelae.
Operative repair of acute aortic disruption in pediatric patients using circulatory support can provide good outcomes. Although not always feasible, the preferential use of primary aortic repair techniques in lieu of interposition conduits and endovascular aortic stents may decrease the potential for late pseudocoarctation.
Accidents, traffic; aorta, thoracic/injuries; aortic rupture/surgery; child; multiple trauma/therapy; thoracic injuries; tomography, X-ray computed; vascular surgical procedure/methods; wounds, nonpenetrating
Inhaled nitric oxide (iNO) reduces death or need for extracorporeal membrane oxygenation (ECMO) in infants with persistent pulmonary hypertension of the newborn (PPHN). However, the response to iNO is variable and only 50–60% of infants demonstrate a response to iNO. It is not known why only some infants respond to iNO. Adults and children with blood groups B or AB do not respond as well to iNO as those with blood groups O/A.
To determine if blood group was associated with iNO response in newborn infants, a retrospective medical record review was done of infants admitted to a regional NICU from 2002-9 with a diagnosis of PPHN. Data were collected during the first twelve hours post-initiation of treatment. Of 86 infants diagnosed with PPHN, 23 infants had blood group A [18 received iNO], 21 had group B [18 with iNO], 40 had group O [36 with iNO], and 2 had group AB [both received iNO]. Change in PaO2/FiO2 was less in infants with blood group A, of whom less than half were responders (ΔPaO2/FiO2>20%) at 12 h versus 90% of infants with either O or B. Race, sex, birth weight, gestational age, Apgar scores at 1 and 5 minutes, and baseline PaO2/FiO2 were similar among groups. Outcomes including need for ECMO, death, length of ventilatory support, length of iNO use, and hospital stay were statistically not different by blood groups.
Our results indicate that blood group influences iNO response in neonates. We hypothesize that either there is genetic linkage of the ABO gene locus with vasoregulatory genes, or that blood group antigens directly affect vascular reactivity.
To assess the continuity of care and outcome of pediatric HIV prevention, testing, and treatment services, focusing on early infant diagnosis with DNA PCR.
A retrospective observational cohort.
Maternal HIV antibody, infant HIV DNA PCR test results, and outcome data from HIV-infected infants from the prevention of mother-to-child transmission, early infant diagnosis, and pediatric HIV treatment programs operating in Lilongwe, Malawi between 2004 and 2008 were collected, merged and analyzed.
Of the 14,669 pregnant women who tested HIV antibody positive, 7,875 infants (53.7%) received HIV DNA PCR testing. One thousand eighty-four infants (13.8%) were HIV-infected. 320 (29.5%) children enrolled into pediatric HIV care, with 202 (63.1%) at the Baylor Center of Excellence. Among these, antiretroviral therapy was initiated on 110 infants (54.5%) whose median age was 9.1 months (interquartile range, 5.4 to 13.8) and a median of 2.5 months (interquartile range, 1.4 to 9.2) after HIV clinic registration. Sixty-nine HIV-infected infants (34.2%) died or were lost by December 2008. Initiation of antiretroviral therapy increased the likelihood of survival seven-fold (odds ratio, 7.1; 95% confidence interval, 3.68 -13.70).
Separate programs for maternal and infant HIV prevention and care services demonstrated high attrition rates of HIV-exposed and HIV-infected infants, elevated levels of mother-to-child transmission, late infant diagnosis, delayed pediatric antiretroviral therapy initiation, and high HIV-infected infant mortality. Antiretroviral therapy increased HIV-infected infant survival, emphasizing the urgent need for improved service coordination and strategies that increase access to infant HIV diagnosis, improve patient retention, and reduce antiretroviral therapy initiation delays.
Africa; antiretroviral therapy; early infant diagnosis; HIV testing; pediatric HIV; prevention of mother-to-child transmission; Malawi
The contemplation for the salvage operations and the nonoperative treatment for the pediatric splenic injuries had increasingly been suggested as the standard case management.
The study was carried out to identify the risk factors, the presentations, the severities and outcome of the interventions of blunt splenic injuries in the children and adolescents.
Materials and Methods:
This retrospective review was carried out in a tertiary care hospital in Sikkim on the children and adolescents admitted with splenic injury from January 2005 to December 2009. Splenic injuries were graded with the American Association for the Surgery of Trauma Splenic Injury Scale followed by the operative and nonoperative managements (NOM).
Overall 147 cases with the abdominal trauma were diagnosed with splenic injury. Of them, males reported in higher numbers; three-fourths were adolescents with preponderance above 16 years of age. Majority of the cases [n=91(61.90%)] were due to fall from heights and others from road traffic accidents. Immediate surgical interventions was instituted in the hemodynamically unstable cases (n=87) NOM failed in 27 patients; of them eight cases underwent splenectomy, and 19 underwent surgical salvage; 33 were closely followed up by conservative approach with both clinical and CT criteria. Total number of cases in grade III and above was significantly higher than with lower grades of injury.
In total 95(64.63%) of the cases were managed with total splenectomy; 19 cases in the initial nonsurgical group underwent salvage operation and 33 cases received NOM.
Splenectomy; splenic injury; nonoperative management
Two of 15 children who survived neonatal meconium ileus had “meconium ileus equivalent.” They were treated with hydration, pancreatic enzyme therapy and antibiotics. One of these children died from pulmonary disease at the time of the bowel obstruction.
The survival rate of infants with meconium ileus is steadily improving because of prompt operative intervention, better preoperative and postoperative care and long-term treatment with enzyme supplements and antibiotics. Late intestinal obstruction due to adhesive bands, volvulus, intussusception or “meconium ileus equivalent” may occur in children previously treated for meconium ileus of infancy.
The omission of pancreatic enzyme supplementation and the occurrence of respiratory infections are frequently associated with “meconium ileus equivalent.”
In this series of patients four of the infants treated surgically for neonatal meconium ileus died in the early postoperative period.
Oral amiodarone was administered to 30 children (aged one week to 14 years) for treatment of resistant or life threatening tachycardias. Five children received initial intravenous medication. The mean duration of oral treatment ranged from two weeks to 64 months (mean 23 months). Infants required a higher oral dose than older children when this was calculated on the basis of body weight but not when it was calculated on the basis of body surface area, indicating that the prescribed dose of amiodarone for infants should be calculated on the basis of body surface area. Although plasma concentrations of amiodarone were similar in infants and children, the plasma concentration of the metabolite desethylamiodarone was lower in infants. The arrhythmias were effectively controlled, by amiodarone alone in 19 and by amiodarone in combination with other drugs in nine children; amiodarone was ineffective in the remaining two children. Unwanted effects were common but were not significantly related to the dose, duration of treatment, or plasma concentration of amiodarone when group results were analysed. Grey facial skin pigmentation developed in two patients who received high cumulative doses of amiodarone and in whom plasma concentrations of amiodarone were high. Four children with biochemical hepatic dysfunction had high plasma concentrations of amiodarone and a further four children who experienced sleep disturbance had required high doses of amiodarone.
Focal intestinal perforation (FIP) has long been described in the pediatric literature. Peritoneal drainage (PD) is widely used as treatment for focal intestinal perforation. Here we report a premature infant that underwent PD on day of life 9 for a FIP. The infant recovered well from this episode and was discharged home without known sequelae. Subsequently, the same patient presented 16 months later with peritonitis. A perforation was discovered at laparotomy without evidence of surrounding necrosis. Given this finding, we believe this second episode of perforation was at the same site as the initial episode of FIP. The finding of FIP has been described without findings of surrounding necrosis. However, we believe this to be the first report of delayed perforation greater than 1 year from initial presentation after FIP treated definitively with peritoneal drain.
In this study with the model organism Agrobacterium tumefaciens, we used a combination of lacZ gene fusions, reverse transcriptase PCR (RT-PCR), and deletion and insertional inactivation mutations to show unambiguously that the alternative sigma factor RpoN participates in the regulation of AsIII oxidation. A deletion mutation that removed the RpoN binding site from the aioBA promoter and an aacC3 (gentamicin resistance) cassette insertional inactivation of the rpoN coding region eliminated aioBA expression and AsIII oxidation, although rpoN expression was not related to cell exposure to AsIII. Putative RpoN binding sites were identified throughout the genome and, as examples, included promoters for aioB, phoB1, pstS1, dctA, glnA, glnB, and flgB that were examined by using qualitative RT-PCR and lacZ reporter fusions to assess the relative contribution of RpoN to their transcription. The expressions of aioB and dctA in the wild-type strain were considerably enhanced in cells exposed to AsIII, and both genes were silent in the rpoN::aacC3 mutant regardless of AsIII. The expression level of glnA was not influenced by AsIII but was reduced (but not silent) in the rpoN::aacC3 mutant and further reduced in the mutant under N starvation conditions. The rpoN::aacC3 mutation had no obvious effect on the expression of glnB, pstS1, phoB1, or flgB. These experiments provide definitive evidence to document the requirement of RpoN for AsIII oxidation but also illustrate that the presence of a consensus RpoN binding site does not necessarily link the associated gene with regulation by AsIII or by this sigma factor.
Researchers are finding limitations of currently available disease-focused questionnaire tools for outcome studies in complementary and alternative medicine/integrative medicine (CAM/IM).
Three substudies investigated the new one-item visual analogue Arizona Integrative Outcomes Scale (AIOS), which assesses self-rated global sense of spiritual, social, mental, emotional, and physical well-being over the past 24 hours and the past month. The first study tested the scale's ability to discriminate unhealthy individuals (n = 50) from healthy individuals (n = 50) in a rehabilitation outpatient clinic sample. The second study examined the concurrent validity of the AIOS by comparing ratings of global well-being to degree of psychological distress as measured by the Brief Symptom Inventory (BSI) in undergraduate college students (N = 458). The third study evaluated the relationships between the AIOS and positively- and negatively-valenced tools (Positive and Negative Affect Scale and the Positive States of Mind Scale) in a different sample of undergraduate students (N = 62).
Substudy (i) Rehabilitation patients scored significantly lower than the healthy controls on both forms of the AIOS and a current global health rating. The AIOS 24-hours correlated moderately and significantly with global health (patients r = 0.50; controls r = 0.45). AIOS 1-month correlations with global health were stronger within the controls (patients r = 0.36; controls r = 0.50). Controls (r = 0.64) had a higher correlation between the AIOS 24-hour and 1-month forms than did the patients (r = 0.33), which is consistent with the presumptive improvement in the patients' condition over the previous 30 days in rehabilitation. Substudy (ii) In undergraduate students, AIOS scores were inversely related to distress ratings, as measured by the global severity index on the BSI (rAIOS24h = -0.42, rAIOS1month = -0.40). Substudy (iii) AIOS scores were significantly correlated with positive affect (rAIOS24h = 0.56, rAIOS1month = 0.57) and positive states of mind (rAIOS24h = 0.42, rAIOS1month = 0.45), and inversely correlated with negative affect (rAIOS24h = -0.41, rAIOS1month = -0.59).
The AIOS is able to distinguish relatively sicker from relatively healthier individuals; and correlates in expected directions with a measure of distress and indicators of positive and negative affect and positive states of mind. The AIOS offers a tool for CAM/IM research that extends beyond a disease emphasis.
complementary and alternative medicine; well-being; global outcomes; questionnaire; validation; rehabilitation
AIMS—To present our experience of
severe upper airway obstruction caused by ulcerative laryngitis in children.
METHODS—Retrospective case note
review of 263 children with severe upper airway obstruction and a
clinical diagnosis of croup admitted to a paediatric intensive care
unit (PICU) over a five year period.
RESULTS—A total of 148 children
(56%) underwent microlaryngoscopy (Storz 3.0 rigid telescope).
Laryngeal ulceration with oedema was documented in 15 of these children
(10%), median age 14 months (range 10-36) and median weight 10 kg
(range 6-12). Twenty seven of the children who underwent
microlaryngoscopy (18%) also had ulcerative gingivostomatitis
consistent with herpes simplex virus infection. Ulcerative laryngitis
was documented in nine of 27(33%) children with, and in six of 121 (5%) children without, coexistent ulcerative gingivostomatitis. One of
the 15 children did not require airway intervention. Nine children
required nasotracheal intubation for a median of 4 days (range 3-11)
and median PICU stay of 6 days (range 4-14). Five children required
tracheostomy ab initio, with a median PICU stay of 30 days (range
20-36), and duration of tracheostomy in situ for a median of 19 days
(range 15-253). All 15 children survived.
CONCLUSION—Ulcerative laryngitis is
more common in our patient population than the few reports suggest.
Early diagnostic microlaryngoscopy is recommended in children with
severe croup who follow an atypical course.
Coronary artery involvement is the most dreaded long-term complication of Kawasaki disease. Our aim was to look at the pattern of cardiovascular involvement in Pakistani children admitted with Kawasaki disease.
This study included children admitted with Kawasaki disease at the Aga Khan University Hospital Karachi over a period of 14 years from January 1997 to December 2010. Information gathered included patient demographics, clinical features, investigations, echocardiographic findings, treatment and follow-up. Those with coronary artery involvement on initial echocardiogram remained on long-term follow-up with clinical examination and echocardiogram.
A total of 56 patients were admitted. (Mean age at diagnosis 33 ± 30 months, age range 2 months to 9 years). 18% of patients had incomplete features. Twenty-five percent (14/56) patients presented after 10 days of fever. Cardiac examination was normal except for tachycardia. Abnormal coronary arteries were seen in 23 patients (41%) - left main coronary artery in 23 (41%), left anterior descending and right main coronary artery in 20 (36%), circumflex branch in 17 (30%). Risk factors for cardiac involvement were male sex, fever >10 days duration at the time of initial presentation and neutrophil percentage >75% in the initial white blood cell counts. Fifty four of 56 cases received intravenous immunoglobulin (IVIG), Seventy-five percent of the patients received IVIG within 10 days of illness. Mean duration of follow-up was 2.5 years. Eight percent of the patients still continue to have abnormal coronaries. There was no mortality.
A higher incidence of coronary artery involvement was found in our study. Presentation after 10 days of illness increases the risk of coronary artery involvement. High index of suspicion among the general pediatricians about the disease can possibly be helpful for early referral and treatment.
Coronary aneurysms; coronary ectasia; Kawasaki disease
Childhood achalasia is an uncommon condition characterised by the absence of oesophageal peristalsis together with increased resting pressure and failure of relaxation of the lower oesophageal sphincter. The currently accepted management is a modified Heller's cardiomyotomy with Nissen fundoplication; however, the long-term results are uncertain. A retrospective review of the notes of 10 children undergoing surgical treatment of achalasia at our institution over a 23-year period were reviewed. There were six boys and four girls. The median age at onset of symptoms was 123 months and at diagnosis was 133 months. Five children were below average weight at the time of presentation. All underwent a modified Heller's operation and Nissen fundoplication. The follow-up ranged from 12 to 277 months with a mean of nearly 10 years. The results were excellent in terms of symptomatic improvement in eight of ten with one good and one moderate outcome. All children below average weight have shown improvement postoperatively. We would conclude that the management of achalasia in childhood should consist of an extended Heller's cardiomyotomy performed by the abdominal approach with the addition of an antireflux procedure such as Nissen fundoplication.
A retrospective review was carried out of 11 consecutive
patients with the Pierre Robin sequence referred to a tertiary
paediatric referral centre over a five year period from 1993 to 1998. Ten patients were diagnosed with significant upper airway obstruction; seven of these presented late at between 24 and 51 days of age. Failure
to thrive occured in six of these seven infants at the time of
presentation, and was a strong indicator of the severity of upper
airway obstruction. Growth normalised on treatment of the upper airway
obstruction with nasopharyngeal tube placement. All children had been
reviewed by either an experienced general paediatrician or a
neonatologist in the first week of life, suggesting that clinical signs
alone are insufficent to alert the physician to the degree of upper
airway obstruction or that obstruction developed gradually after
discharge home. The use of polysomnography greatly improved the
diagnostic accuracy in assesssing the severity of upper airway
obstruction and monitoring the response to treatment. This report
highlights the prevalence of late presentation of upper airway
obstruction in the Pierre Robin sequence and emphasises the need for
close prospective respiratory monitoring in this condition. Objective
measures such as polysomnography should be used, as clinical signs
alone may be an inadequate guide to the degree of upper airway obstruction.
In 1998, the one-year experience in minimally invasive abdominal surgery in children at a pediatric training center was assessed. Seven years later, we determined the current status of pediatric minimally invasive surgery in daily practice and surgical training.
A retrospective review was undertaken of all children with intra-abdominal operations performed between 1 January 2005 and 31 December 2005.
The type of operations performed ranged from common interventions to demanding laparoscopic procedures. 81% of all abdominal procedures were performed laparoscopically, with a complication rate stable at 6.9%, and conversion rate decreasing from 10% to 7.4%, compared to 1998. There were six new advanced laparoscopic procedures performed in 2005 as compared to 1998. The children in the open operated group were significantly smaller and younger than in the laparoscopic group (p < 0.001 and p = 0.001, respectively). The majority (64.2%) of the laparoscopic procedures were performed by a trainee. There was no difference in the operating times of open versus laparoscopic surgery, or of procedures performed by trainees versus staff surgeons. Laparoscopy by trainees did not have a negative impact on complication or conversion rates.
Laparoscopy is an established approach in abdominal procedures in children, and does not hamper surgical training.
Pediatric surgery; Minimally invasive surgery; Training