Objective: To determine if Lactobacillus GG (LGG) is an effective adjunct to lactulose for treating constipation in children. Study design: Eighty- four chi ldren (2- 16 years of age) with constipation (< 3 spontan...Objective: To determine if Lactobacillus GG (LGG) is an effective adjunct to lactulose for treating constipation in children. Study design: Eighty- four chi ldren (2- 16 years of age) with constipation (< 3 spontaneous bowel movements [ BMs] per week for at least 12 weeks) were enrolled in a double- blind, randomiz ed placebo- controlled trial in which they received 1 mL/kg/day of 70% lactul ose plus 109 colony- forming units (CFU) of LGG (experimental group, n = 43) or a placebo (control group, n = 41) orally twice daily for 12 weeks. The primary outcome measure was treatment success, and analyses were performed on an intenti on- to- treat basis. Results: Treatment success, defined as ≥ 3 spontaneous B Ms per week with no fecal soiling, was similar in the control and experimental g roups at 12 weeks (28/41 [68% ] vs 31/43 [72% ], respectively; P =. 7) and at 24 weeks (27/41 [65% ] vs 27/42 [64% ], respectively; P = 1.0]. Groups also di d not differ in their mean number of spontaneous BMs per week or episodes of fec al soiling per week at 4, 8, and 12 weeks. Adverse events and overall tolerance did not differ between groups. Conclusion: LGG, as dosed in this study, was not an effective adjunct to lactulose in treating constipation in children.展开更多
Background: Recently, polyethylene glycol (PEG 3350) has been suggested as a good alternative laxative to lactulose as a treatment option in paediatric constipation. However, no large randomised controlled trials exis...Background: Recently, polyethylene glycol (PEG 3350) has been suggested as a good alternative laxative to lactulose as a treatment option in paediatric constipation. However, no large randomised controlled trials exist evaluating the efficacy of either laxative. Aims: To compare PEG 3350 (Transipeg: polyethylene glycol with electrolytes) with lactulose in paediatric constipation and evaluate clinical efficacy/side effects. Patients: One hundred patients (aged 6 months-15 years) with paediatric constipation were included in an eight week double blinded , randomised, controlled trial. Methods: After faecal disimpaction, patients < 6 years of age received PEG 3350 (2.95 g/sachet) or lactulose (6 g/sachet) while children ≥6 years started with 2 sachets/day. Primary outcome measures were: de fecation and encopresis frequency/week and successful treatment after eight weeks. Success was defined as a defecation frequency ≥3/week and encopresis ≤1 every two weeks. Secondary outcome measures were side effects after eight weeks of treatment. Results: A total of 91 patients (49 male) completed the study. A significant increase in defeca tion frequency (PEG 3350: 3 prev 7 post treatment/week; lactulose: 3 prev 6 post/week) and a significant decrease in encopresis frequency (PEG 3350: 10 prev 3 post/week; lactulose: 8 prev 3 post/week) was found in both groups (NS). However, success was significantly higher in the PEG group (56%) compared with the lactulose group (29%). PEG 3350 patients reported less abdominal pain, straining, and pain at defecation than children using lactulose. However, bad taste was reported significantly more often in the PEG group. Conclusions: PEG 3350 (0.26 (0.11) g/kg), compared with lactulose (0.66 (0.32) g/kg), provided a higher success rate with fewer side effects. PEG 3350 should be the laxative of first choice in childhood constipation.展开更多
Purpose Fundus autofluorescence is due to accumulation of lipofuscin in the retinal pigment ep ithelium(RPE)resulting from incomplete digestion of N-retinylidene-phosphatidyl-ethanolamine from shed photorecep tor oute...Purpose Fundus autofluorescence is due to accumulation of lipofuscin in the retinal pigment ep ithelium(RPE)resulting from incomplete digestion of N-retinylidene-phosphatidyl-ethanolamine from shed photorecep tor outer segment discs.Alteration in autofluorescence reflects changes in lipofuscin content of the RPE.Mutations on both alleles of RPE65result in absent or largely decrease d formation of rhodopsin,due to a defect in alltrans retinol is omerization in the RPE.Autofluorescence could therefore b e altered.This study was conducted to evaluate fundus autofl uorescence in patients with early-onset severe retinal dystrophy(EOSRD,or ear-ly-onset rod-cone dystrophy)associated with mutations on both alleles of RPE65.Design Case se ries.Participants and controls Ten 10-to 55-year-old p atients with EOSRD and compound heterozygous or homozy gous mutations in RPE65.For comparison,6heterozygous parents and 2patients with other forms of EOSRD we re examined.Methods Participants underwent,in addition to standard clinical and electrophysiological examination,autofluores-cence imaging using a confocal scanning laser ophthalmo-scope.Three of the patients were als o examined by optical coherence tomography(OCT)to evaluate the status of retinal degeneration.Mutations in7patients have been reported previously;the other pati ents were investigated by polymerase chain reaction-single-strand conformation poly-morphism and direct sequencing for mutations in RPE65and lecithin retinol acyltransfera se(LRAT).Main outcome measures Fundus autofluorescence a nd OCT.Results Ab-sent or minimal autofluorescence wa s found in all patients with compound heterozygous or homozygous RPE65muta-tions.Autofluorescence was normal in the heterozygous parents.Autofluorescence was present in 2children with EOSRD not associated with mutations in RPE65or LRAT,another gene involved in retinol recycling.Optical coher-ence tomography in younger patients revealed an intraretinal appearance similar to that of their h ealthy,heterozygous parents.Conclusions Lack of autofl uorescence in patients with EOSRD associated with mutation s in RPE65is in ac-cordance with the biochemical defect and can be used as a clinical marker of this genotype.Optical coherence tomog-raphy results in younger patients wo uld indicate still viable photoreceptors despite the absence of autofluorescence.展开更多
Background: Constipation and encopresis frequently cause problems with respect to emotional wellbeing, and social and family life.Instruments to measure Health Related Quality of Life (HRQoL) in these disorders ill ar...Background: Constipation and encopresis frequently cause problems with respect to emotional wellbeing, and social and family life.Instruments to measure Health Related Quality of Life (HRQoL) in these disorders ill are not available.Methods: Adisease specific HRQoL instrument, the "Defecation Disorder List"(DDL) for children with constipation or functional non-retentive faecal soiling (FNRFS) was developed using accepted guidelines.For each phase of the process, different samples of patients were used.The final phase of development included 27 children.Reliability was assessed in two ways: internal consistency of domains with Cronbach’s alpha, and test-retest reliability with intra-class correlation coefficients (ICC).To assess validity, comparable items and domains were correlated with Tacqol, a generic HRQoL instrument for children (TNOAZL).Results: In the final phase of the development, 27 children completed the instrument.It consisted of 37 items in four domains.The response rate was 96%.Reliability was good for all domains, with Cronbach’s alpha values ranging from 0.61 to 0.76.Measures of test-retest stability were good for all four domains with ICCs ranging from 0.82 to 0.92.Validity based on comparison with the Tacqol instrument was moderate.Conclusion: The DDL is promising as a measure of HRQoL in childhood defecation disorders.展开更多
Familial adenomatous polyposis (FAP) is an autosomal dominant disorder that ch aracteristically presents with colon cancer in early adult life. We describe a P akistani FAP family in which two sons had an unusually ea...Familial adenomatous polyposis (FAP) is an autosomal dominant disorder that ch aracteristically presents with colon cancer in early adult life. We describe a P akistani FAP family in which two sons had an unusually early manifestation of co lorectal cancer. The index patient presented at 11 years of age with abdominal p ain, rectal bleeding and iron deficiency anaemia. Colonoscopy showed that the co lon was carpeted with a myriad of polyps. Oesophago-gastric and duodenal endosc opy revealed that polyps had also developed in the duodenum. Multiple biopsies i ndicated neoplastic lesions. The patient underwent a proctocolectomy and endosco pic duodenal mucosectomy. The diagnosis of an adenocarcinoma of the colon and fu rther adenomatous polyps with low-grade and high-grade dysplasia was confirmed by histology. Family screening including a blood test for anaemia and bowel exa mination revealed that his 12-year-old brother was also affected. Conclusion: Children with familial adenomatous polyposis are at risk for colon cancer and em phasise the need for early tumour recognition. Gastrointestinal symptoms in chil dren should be thoroughly evaluated and standard screening for colonic polyposis should be performed in all individuals with a positive family history and/or kn own mutations in cancer-associated genes, particularly in children who are unde r 10 years of age.展开更多
The prognostic value of nucleated red blood cell count at birth in relation t o neonatal outcome has been established. However, reference values were needed t o usefully interpret this variable. The normal range of re...The prognostic value of nucleated red blood cell count at birth in relation t o neonatal outcome has been established. However, reference values were needed t o usefully interpret this variable. The normal range of reference values for abs olute nucleated red blood cell count in 695 preterm and term newborns is reporte d.展开更多
<strong>Background:</strong> In Nigeria Pentavalent vaccine had replaced Diphtheria-Pertussis- Tetanus [DPT] vaccine in the prevention of pertussis since 2012. <strong>Aims and Objectives:</strong...<strong>Background:</strong> In Nigeria Pentavalent vaccine had replaced Diphtheria-Pertussis- Tetanus [DPT] vaccine in the prevention of pertussis since 2012. <strong>Aims and Objectives:</strong> The aim of this study was to compare the anti-pertussis immunoglobin G (IgG) response of children who received DPT with those who received the pentavalent vaccine. <strong>Subjects and Methods:</strong> This study was carried out in Akpabuyo LGA of Cross River State from April to June 2016. It was a cross-sectional survey of anti-pertussis IgG levels in children aged 6 months to 5 years who received DPT and those who received pentavalent vaccine. IgG antibody levels were determined using enzyme-linked immunosorbent assay. The protective level was set at >11 DU according to manufacturer’s cut off point. <strong>Results:</strong> Seventy eight out of 230 children [33.9%] who had received DPT had protective levels of anti-pertussis IgG compared to 74 out of 192 children [38.5%] who had received pentavalent vaccine. The difference was not statistically significant [<em>p</em> = 0.61]. The median IgG antibody level in those who received DPT was 8.0 DU (interquartile range (IQR) 4.0 - 13.0) compared with 9.0 DU (IQR) 4.0 - 15.0 in those who received pentavalent vaccine [<em>p</em> = 0.18]. No single factor investigated predicted the development of protective levels of antibody in the multivariate analysis. <strong>Conclusion/Recommendation:</strong> There was no difference in the antipertussis antibody response between DPT and pentavalent vaccines recipients. Further study is needed to elucidate factors that could be responsible for low anti-pertussis antibody response in this population.展开更多
Fifteen patients with intestinal villous atrophy, but simultaneously negative results of antiendomysial antibodies (EMA) were studied. Two patients were final ly diagnosed as having coeliac disease. The predictive val...Fifteen patients with intestinal villous atrophy, but simultaneously negative results of antiendomysial antibodies (EMA) were studied. Two patients were final ly diagnosed as having coeliac disease. The predictive value of negative results of EMA ass essment in children suspected of coeliac disease is high, approaching 86.7%.展开更多
文摘Objective: To determine if Lactobacillus GG (LGG) is an effective adjunct to lactulose for treating constipation in children. Study design: Eighty- four chi ldren (2- 16 years of age) with constipation (< 3 spontaneous bowel movements [ BMs] per week for at least 12 weeks) were enrolled in a double- blind, randomiz ed placebo- controlled trial in which they received 1 mL/kg/day of 70% lactul ose plus 109 colony- forming units (CFU) of LGG (experimental group, n = 43) or a placebo (control group, n = 41) orally twice daily for 12 weeks. The primary outcome measure was treatment success, and analyses were performed on an intenti on- to- treat basis. Results: Treatment success, defined as ≥ 3 spontaneous B Ms per week with no fecal soiling, was similar in the control and experimental g roups at 12 weeks (28/41 [68% ] vs 31/43 [72% ], respectively; P =. 7) and at 24 weeks (27/41 [65% ] vs 27/42 [64% ], respectively; P = 1.0]. Groups also di d not differ in their mean number of spontaneous BMs per week or episodes of fec al soiling per week at 4, 8, and 12 weeks. Adverse events and overall tolerance did not differ between groups. Conclusion: LGG, as dosed in this study, was not an effective adjunct to lactulose in treating constipation in children.
文摘Background: Recently, polyethylene glycol (PEG 3350) has been suggested as a good alternative laxative to lactulose as a treatment option in paediatric constipation. However, no large randomised controlled trials exist evaluating the efficacy of either laxative. Aims: To compare PEG 3350 (Transipeg: polyethylene glycol with electrolytes) with lactulose in paediatric constipation and evaluate clinical efficacy/side effects. Patients: One hundred patients (aged 6 months-15 years) with paediatric constipation were included in an eight week double blinded , randomised, controlled trial. Methods: After faecal disimpaction, patients < 6 years of age received PEG 3350 (2.95 g/sachet) or lactulose (6 g/sachet) while children ≥6 years started with 2 sachets/day. Primary outcome measures were: de fecation and encopresis frequency/week and successful treatment after eight weeks. Success was defined as a defecation frequency ≥3/week and encopresis ≤1 every two weeks. Secondary outcome measures were side effects after eight weeks of treatment. Results: A total of 91 patients (49 male) completed the study. A significant increase in defeca tion frequency (PEG 3350: 3 prev 7 post treatment/week; lactulose: 3 prev 6 post/week) and a significant decrease in encopresis frequency (PEG 3350: 10 prev 3 post/week; lactulose: 8 prev 3 post/week) was found in both groups (NS). However, success was significantly higher in the PEG group (56%) compared with the lactulose group (29%). PEG 3350 patients reported less abdominal pain, straining, and pain at defecation than children using lactulose. However, bad taste was reported significantly more often in the PEG group. Conclusions: PEG 3350 (0.26 (0.11) g/kg), compared with lactulose (0.66 (0.32) g/kg), provided a higher success rate with fewer side effects. PEG 3350 should be the laxative of first choice in childhood constipation.
文摘Purpose Fundus autofluorescence is due to accumulation of lipofuscin in the retinal pigment ep ithelium(RPE)resulting from incomplete digestion of N-retinylidene-phosphatidyl-ethanolamine from shed photorecep tor outer segment discs.Alteration in autofluorescence reflects changes in lipofuscin content of the RPE.Mutations on both alleles of RPE65result in absent or largely decrease d formation of rhodopsin,due to a defect in alltrans retinol is omerization in the RPE.Autofluorescence could therefore b e altered.This study was conducted to evaluate fundus autofl uorescence in patients with early-onset severe retinal dystrophy(EOSRD,or ear-ly-onset rod-cone dystrophy)associated with mutations on both alleles of RPE65.Design Case se ries.Participants and controls Ten 10-to 55-year-old p atients with EOSRD and compound heterozygous or homozy gous mutations in RPE65.For comparison,6heterozygous parents and 2patients with other forms of EOSRD we re examined.Methods Participants underwent,in addition to standard clinical and electrophysiological examination,autofluores-cence imaging using a confocal scanning laser ophthalmo-scope.Three of the patients were als o examined by optical coherence tomography(OCT)to evaluate the status of retinal degeneration.Mutations in7patients have been reported previously;the other pati ents were investigated by polymerase chain reaction-single-strand conformation poly-morphism and direct sequencing for mutations in RPE65and lecithin retinol acyltransfera se(LRAT).Main outcome measures Fundus autofluorescence a nd OCT.Results Ab-sent or minimal autofluorescence wa s found in all patients with compound heterozygous or homozygous RPE65muta-tions.Autofluorescence was normal in the heterozygous parents.Autofluorescence was present in 2children with EOSRD not associated with mutations in RPE65or LRAT,another gene involved in retinol recycling.Optical coher-ence tomography in younger patients revealed an intraretinal appearance similar to that of their h ealthy,heterozygous parents.Conclusions Lack of autofl uorescence in patients with EOSRD associated with mutation s in RPE65is in ac-cordance with the biochemical defect and can be used as a clinical marker of this genotype.Optical coherence tomog-raphy results in younger patients wo uld indicate still viable photoreceptors despite the absence of autofluorescence.
文摘Background: Constipation and encopresis frequently cause problems with respect to emotional wellbeing, and social and family life.Instruments to measure Health Related Quality of Life (HRQoL) in these disorders ill are not available.Methods: Adisease specific HRQoL instrument, the "Defecation Disorder List"(DDL) for children with constipation or functional non-retentive faecal soiling (FNRFS) was developed using accepted guidelines.For each phase of the process, different samples of patients were used.The final phase of development included 27 children.Reliability was assessed in two ways: internal consistency of domains with Cronbach’s alpha, and test-retest reliability with intra-class correlation coefficients (ICC).To assess validity, comparable items and domains were correlated with Tacqol, a generic HRQoL instrument for children (TNOAZL).Results: In the final phase of the development, 27 children completed the instrument.It consisted of 37 items in four domains.The response rate was 96%.Reliability was good for all domains, with Cronbach’s alpha values ranging from 0.61 to 0.76.Measures of test-retest stability were good for all four domains with ICCs ranging from 0.82 to 0.92.Validity based on comparison with the Tacqol instrument was moderate.Conclusion: The DDL is promising as a measure of HRQoL in childhood defecation disorders.
文摘Familial adenomatous polyposis (FAP) is an autosomal dominant disorder that ch aracteristically presents with colon cancer in early adult life. We describe a P akistani FAP family in which two sons had an unusually early manifestation of co lorectal cancer. The index patient presented at 11 years of age with abdominal p ain, rectal bleeding and iron deficiency anaemia. Colonoscopy showed that the co lon was carpeted with a myriad of polyps. Oesophago-gastric and duodenal endosc opy revealed that polyps had also developed in the duodenum. Multiple biopsies i ndicated neoplastic lesions. The patient underwent a proctocolectomy and endosco pic duodenal mucosectomy. The diagnosis of an adenocarcinoma of the colon and fu rther adenomatous polyps with low-grade and high-grade dysplasia was confirmed by histology. Family screening including a blood test for anaemia and bowel exa mination revealed that his 12-year-old brother was also affected. Conclusion: Children with familial adenomatous polyposis are at risk for colon cancer and em phasise the need for early tumour recognition. Gastrointestinal symptoms in chil dren should be thoroughly evaluated and standard screening for colonic polyposis should be performed in all individuals with a positive family history and/or kn own mutations in cancer-associated genes, particularly in children who are unde r 10 years of age.
文摘The prognostic value of nucleated red blood cell count at birth in relation t o neonatal outcome has been established. However, reference values were needed t o usefully interpret this variable. The normal range of reference values for abs olute nucleated red blood cell count in 695 preterm and term newborns is reporte d.
文摘<strong>Background:</strong> In Nigeria Pentavalent vaccine had replaced Diphtheria-Pertussis- Tetanus [DPT] vaccine in the prevention of pertussis since 2012. <strong>Aims and Objectives:</strong> The aim of this study was to compare the anti-pertussis immunoglobin G (IgG) response of children who received DPT with those who received the pentavalent vaccine. <strong>Subjects and Methods:</strong> This study was carried out in Akpabuyo LGA of Cross River State from April to June 2016. It was a cross-sectional survey of anti-pertussis IgG levels in children aged 6 months to 5 years who received DPT and those who received pentavalent vaccine. IgG antibody levels were determined using enzyme-linked immunosorbent assay. The protective level was set at >11 DU according to manufacturer’s cut off point. <strong>Results:</strong> Seventy eight out of 230 children [33.9%] who had received DPT had protective levels of anti-pertussis IgG compared to 74 out of 192 children [38.5%] who had received pentavalent vaccine. The difference was not statistically significant [<em>p</em> = 0.61]. The median IgG antibody level in those who received DPT was 8.0 DU (interquartile range (IQR) 4.0 - 13.0) compared with 9.0 DU (IQR) 4.0 - 15.0 in those who received pentavalent vaccine [<em>p</em> = 0.18]. No single factor investigated predicted the development of protective levels of antibody in the multivariate analysis. <strong>Conclusion/Recommendation:</strong> There was no difference in the antipertussis antibody response between DPT and pentavalent vaccines recipients. Further study is needed to elucidate factors that could be responsible for low anti-pertussis antibody response in this population.
文摘Fifteen patients with intestinal villous atrophy, but simultaneously negative results of antiendomysial antibodies (EMA) were studied. Two patients were final ly diagnosed as having coeliac disease. The predictive value of negative results of EMA ass essment in children suspected of coeliac disease is high, approaching 86.7%.
