Health-related quality of life in pediatric ulcerative colitis patients on conventional medical treatment compared to those after restorative proctocolectomy.

PURPOSE: Health-related quality of life (HRQL) is not well studied in proctocolectomy patients with pediatric onset of ulcerative colitis (UC). We aimed to (1) compare the HRQL of proctocolectomy patients with those treated with conventional therapy and (2) determine factors that influence HRQL in UC patients < 18 years. METHODS: Chart review was done on patients diagnosed with pediatric onset of UC (<18) at the Stollery Children's Hospital. HRQL was evaluated in 88 patients using disease- and age-specific questionnaires; IMPACT III (<18) and Inflammatory Bowel Disease Questionnaire (IBDQ; ≥18). Demographics, disease characteristics, disease index (PUCAI), HRQL EuroQoL visual analog scale (EQ-5D/VAS) were collected and analyzed from all patients. RESULTS: Sixty-five respondents completed the IMPACT III (74 %) and 23 patients completed the IBDQ (26 %). Thirty-three surgical patients (34 %) responded (mean IMPACT III score = 148.9 ± 12.7; mean IBDQ = 171.2 ± 40.1). There was no significant difference in IMPACT III scores of surgical patients vs. medically treated patients (148.9 ± 12.7 vs. 140.6 ± 19.4, p = 0.09). Patients with high IMPACT scores (>143 points) were most likely to be in remission (p = 0.05), they were less likely to be on medication (p < 0.05), have parent/guardian with postsecondary education (p = 0.01), did not suffer from fatigue (p < 0.01), and did not report depression (p < 0.02). The IMPACT correlation with PUCAI (adjusted r (2) = 0.33) and EQ-VAS (adjusted r (2) = 0.45) was strong. CONCLUSIONS: Surgical patients reported to have a HRQL comparable to or better than the nonsurgical patients. Depression, fatigue, parent/guardian education, and drugs influence HRQL.

Let's unite in valuing the contribution we all make.

I have just read Aldo Picek's envious rant about NHS staff pensions ( letters August 17 ). He writes: 'NHS nurses do not know how privileged they are.'

Gangliosides protect bowel in an infant model of necrotizing enterocolitis by suppressing proinflammatory signals.

OBJECTIVES: Necrotizing enterocolitis (NEC) has high morbidity in premature infants. Hypoxia-ischemia, infection, and enteral feeding are risk factors associated with NEC, whereas feeding human milk is protective. Vasoactive and inflammatory mediators in NEC remain elusive. Gangliosides are found in human milk and enterocyte membranes. An infant bowel model of NEC was developed to test the hypothesis that gangliosides modulate the inflammatory response to infection and hypoxia. PATIENTS AND METHODS: Viable, noninflamed bowel was obtained from 9 infants between 26 and 40 weeks' gestational age. Infant bowel was treated in culture with Escherichia coli lipopolysaccharide (LPS) and hypoxia in the presence or absence of preexposure to gangliosides. Bowel necrosis and production of nitric oxide, endothelin-1, serotonin, eicosanoids, hydrogen peroxide, and proinflammatory cytokines were measured. RESULTS: Ganglioside preexposure reduced bowel necrosis and endothelin-1 production in response to LPS. Gangliosides suppressed infant bowel production of nitric oxide, leukotriene B4, prostaglandin E2, hydrogen peroxide, interleukin-1beta, interleukin-6, and interleukin-8 in response to LPS exposure and hypoxia. CONCLUSIONS: A bowel protective effect of gangliosides is indicated by modulation of vasoactive mediators and proinflammatory signal suppression.

A mobile Meckel!

A technetium-99m pertechnetate Meckel scan is the standard diagnostic test to diagnose Meckel diverticulum. Although a negative scan does not exclude Meckel diverticulum, it should be kept in mind that the diagnosis can be missed on the basis of a single negative scan. Another important point is the typical position of this anatomical abnormality in the scan. We report a peculiar anatomical variation of Meckel diverticulum as seen in the technetium-99m pertechnetate scan. Recognizing this variant of Meckel diverticulum is important to avoid missing such a potentially life-threatening congenital anomaly.

A case of successful ablation of a gastrophrenic fistula with n-Butyl-2-Cyanocrylate.

A 79-year-old woman with a fistula between a subphrenic abscess and the fundus of the stomach was successfully treated with n-Butyl-2-Cyanoacrylate. Conservative management had failed. Clinical presentation, treatment progress and imaging findings by computed tomography scan, ultrasound, gastroscopy and fluoroscopy are presented, along with a brief review of the relevant literature.

Esophageal mucoceles causing airway obstruction as a complication of esophageal diversion: two rare pediatric cases and a review of the literature.

An esophageal mucocele causing airway obstruction is an exceptionally rare complication of esophageal diversion in children. In this instance, they are fluid-filled dilatations of the esophageal remnant following bipolar exclusion of the thoracic esophagus. Only six pediatric cases have been reported previously in the literature. We present two consecutive cases of esophageal mucoceles causing respiratory distress in children following surgical exclusion of the esophagus. Bronchoscopy followed by imaging (computerized tomography or magnetic resonance imaging) was used to reach the diagnosis. Complete resection of the thoracic esophagus was required in both patients. Esophageal mucoceles can occur many years after esophageal exclusion, and the clinical features are often non-specific. Furthermore, complex co-morbidities may mask the underlying etiology of the respiratory distress, thus the diagnosis may be difficult to delineate. A high degree of suspicion, clinical awareness, and the use of the proper diagnostic tools, are essential for a diagnosis of mucoceles in children with a past history of esophageal exclusion.

Produce a positive strategy for whistleblowing.

A day in the life of UK healthcare can make sad reading. Complaints made to the ombudsman over care funding have doubled in 5 years. Plans to encourage people to save to meet their own social care costs in old age are being discussed. The majority of specialist nurses believe that the care provided to young people with mental health problems is inadequate and getting worse.

Increased catabolism and decreased unsaturation of ganglioside in patients with inflammatory bowel disease.

AIM: To investigate whether accelerated catabolism of ganglioside and decreased ganglioside content contribute to the etiology of pro-inflammatory intestinal disease. METHODS: Intestinal mucosa from terminal ileum or colon was obtained from patients with ulcerative colitis or inflammatory Crohn's disease (n = 11) undergoing bowel resection and compared to control samples of normal intestine from patients with benign colon polyps (n = 6) and colorectal cancer (n = 12) in this observational case-control study. Gangliosides and phospholipids of intestinal mucosa were characterized by class and ceramide or fatty acid composition using liquid chromatography triple-quad mass spectrometry. Content and composition of ganglioside classes GM1, GM3, GD3, GD1a, GT1 and GT3 were compared among subject groups. Content and composition of phospholipid classes phosphatidylcholine (PC) and phosphatidylethanolamine were compared among subject groups. Unsaturation index of individual ganglioside and phospholipid classes was computed and compared among subject groups. Ganglioside catabolism enzymes beta-hexosaminidase A (HEXA) and sialidase-3 (NEU3) were measured in intestinal mucosa using western blot and compared among subject groups. RESULTS: Relative GM3 ganglioside content was 2-fold higher (P < 0.05) in intestine from patients with inflammatory bowel disease (IBD) compared to control intestine. The quantity of GM3 and ratio of GM3/GD3 was also higher in IBD intestine than control tissue (P < 0.05). Control intestine exhibited 3-fold higher (P < 0.01) relative GD1a ganglioside content than IBD intestine. GD3 and GD1a species of ganglioside containing three unsaturated bonds were present in control intestine, but were not detected in IBD intestine. The relative content of PC containing more than two unsaturated bonds was 30% lower in IBD intestine than control intestine (P < 0.05). The relative content of HEXA in IBD intestine was increased 1.7-fold (P < 0.05) and NEU3 was increased 8.3-fold (P < 0.01) compared to normal intestine. Intestinal mucosa in IBD is characterized by increased GM3 content, decreased GD1a, and a reduction in polyunsaturated fatty acid constituents in GD3, GD1a and PC. CONCLUSION: This study suggests a new paradigm by proposing that IBD occurs as a consequence of increased metabolism of specific gangliosides.

Criticism of nurses by nurses is unwelcome and unjustified.

Your letters page ( February 15 ) gave startlingly contrasting opinions on the Confessions of a Nurse series on More 4 - from inspirational to disreputable. I see it as refreshing.

Trade unions have long since dropped divisive attitudes.

The letter by Bríd Hehir from the Do Good Charity (September 26) will no doubt stir up a lot of reaction by her provocative views on trade union values. Well Ms Hehir, if you think there is too much 'chaff' in the NHS that needs chucking out then please bring your 'do good' skills to the table.

Colocolic intussusception in a preterm infant with intestinal malrotation.

The association of intussusception and intestinal malrotation is referred to as Waugh syndrome. A prospective study among 49 children with intussusception found a 40% incidence of malrotation. We describe the rare occurrence of colocolic intussusception in a neonate with malrotated intestine. We believe this is the first reported case in the neonatal period where the pathogenic lead point was an intestinal lymphangioma.

Fetus-in-fetu: confirmation of prenatal diagnosis with MRI.

Fetus-in-fetu is a rare form of monozygotic diamniotic twin pregnancy in which one fetus is enclosed within its twin (Khadaroo et al., 2000). This case report highlights the use of prenatal MRI to confirm prenatal diagnosis of an intra-abdominal FIF to aid obstetricians, neonatologists, and pediatric general surgeons in perinatal and surgical management.

Autonomous thyroid nodule in an adolescent 10 years after total body irradiation for bone marrow transplant.

A male patient with B-cell lymphoma was treated with chemotherapy and allogeneic bone marrow transplant, including preparatory total body irradiation. Ten years later, at age 15 years, the patient developed an autonomous thyroid nodule and an incidental papillary microcarcinoma. This is the first report of an autonomous thyroid nodule after total body irradiation for bone marrow transplant. The case is presented and the literature is reviewed.

Association between surgical margins and long-term outcome in advanced hepatoblastoma.

BACKGROUND: Many patients with hepatoblastoma present with unresectable disease. Neoadjuvant therapy has improved resectability rates to as high as 70% to 90%. Despite this improvement, many patients will be left with tumors that are of borderline resectability. The authors hypothesize that favorable outcomes may be achieved even with resection margins less than 1 cm thus sparing the need for liver transplantation. METHODS: Between January 1981 and March 2003, 23 patients age less than 16 years with a diagnosis of hepatoblastoma undergoing surgical resection were identified. The clinical characteristics, pathologic resection margins, and survival status were reviewed. RESULTS: Eighteen (78%) of the patients were alive with no evidence of recurrence at last follow-up. Thirteen (56.5%) had > or =1 cm resection margins, whereas 10 (43.5%) had resection margins less than 1 cm. Eleven (47.8%) presented with PRETEXT III tumors. There was no significant difference in survival rate between resection margins less than 1 cm and > or =1 cm (P =.13; 95% CI 0.91 to 2.61). Thirteen patients (56.5%) presented with synchronous pulmonary metastatic disease, where survival was significantly worse (P =.04; 95% CI 1.10 to 2.50). Subgroup analysis confirmed that margins less than 1 cm did not significantly affect survival after controlling for pulmonary metastatic disease (P =.56; 95% CI 0.71 to 3.61). CONCLUSIONS: Surgical resection with margins less than 1 cm are associated with survival that is equivalent to resection with margins > or =1 cm. Our findings suggest it is preferable to preserve key structures with a small resection margin and therefore spare the need for liver transplantation in patients with advanced hepatoblastoma.