Cap polyposis is a rare and under-recognised cause of rectal bleeding in children. Our study has characterized the disease phenotype and treatment outcomes in a pediatric cohort.
In PCD, rapid food reintroduction following 6-week EEN is safe and equally effective as longer food reintroduction. We propose that a rapid food reintroduction schedule be recommended as the most tolerable approach for food reintroduction. Relapse rate and duration of remission are uninfluenced by the type of food reintroduction.
BackgroundExclusive enteral nutrition (EEN) is as effective as corticosteroids in inducing remission in children with Crohn’s disease (CD). However, over 50% of these children relapse by 12 months of diagnosis. Thiopurines are commonly prescribed as maintenance therapy for CD, but evidence for its efficacy is controversial. Data on the effectiveness of EEN in Southeast Asian (SEA) children with CD is scarce. This study aims to evaluate the efficacy of EEN induction therapy in a cohort of SEA children with newly diagnosed CD. The secondary aim was to evaluate concomitant early azathioprine (EAZ) use in determining remission rate at 6 and 12 months.MethodsCase records of all children with newly diagnosed CD from 2011 to 2014 were reviewed and relevant demographic as well as clinical data were extracted. The primary outcome measure was the number of patients who completed EEN induction therapy and achieved remission (Paediatric Crohn’s Disease Activity Index; PCDAI≤10). Factors influencing duration of remission were evaluated in particular early azathioprine (EAZ) defined as starting azathioprine within one month of diagnosis versus late azathioprine (LAZ) use.ResultsForty children with newly diagnosed CD were identified. Thirty-three children: 67% boys, median age 13y (range 3–17) completed 8 weeks of EEN induction therapy and 91% achieved remission. Significant improvements were seen in PCDAI scores (32.7 ± 9.2 to 4.2 ± 5.1; p < 0.001), mean BMI z-score (− 1.38 ± 1.57 to − 0.82 ± 1.27; p = 0.004) and baseline inflammatory markers: Erythrocyte Sedimentation Rate (51.6 ± 30.1 mm/h to 13.3 ± 7.1 mm/h; p < 0.0001) C-Reactive Protein (44.6 ± 51.0 mg/L to 5.2 ± 7.6 mg/L; p = 0.001), Albumin (30.7 ± 7.5 g/L to 38.7 ± 3.9 g/L; p < 0.0001), Platelets (464 ± 161 × 109 to 370 ± 111 × 109; p < 0.0001),. Early azathioprine initiation was associated with a remission rate of 80 and 73% at 6 and 12 months respectively. Remission was also maintained for longer duration in EAZ vs LAZ groups (p = 0.048).ConclusionEEN effectively induces remission in this cohort of SEA children with newly diagnosed CD. Early initiation of thiopurine with EEN induction therapy is effective in maintaining steroid-free remission for at least one year.
In all children with fulminant hepatic failure, the differential diagnosis of leukaemia should be considered, particularly if there is hepatosplenomegaly, pancytopenia and a high lactate dehydrogenase. Early bone marrow aspiration should be performed to confirm diagnosis, as peripheral blood smears may often be normal.Hepatomegly is a common feature of leukaemia but acute liver failure as the first presentation is extremely uncommon [1,2].We report a 4-year-old girl with a 2-week history of lethargy, vomiting and fluctuating sensorium for 2 days. There was no history of drug or toxin ingestion. She was icteric with liver 15 cm and spleen 9 cm below the costal margin respectively. Due to grade 3 encephalopathy, she was mechanically ventilated on the liver intensive care unit.Laboratory investigations revealed the following: haemoglobin 91 g/l (reference range 115-155 g/l), WBC 0.85·10 9 /l (reference range 5.5-15.5·10 9 /l), neutrophil count 0.5·10 9 /l (reference range 3-5.8·10 9 /l), platelet count 29·10 9 /l (reference range 150-400·10 9 /l) and no blasts were seen in the peripheral smear. Furthermore, INR 2.6 (reference range 0.8-1.2), D-dimer 1925 ng/ml, AST 714 IU/l (reference range 15-55 IU/l), total bilirubin 6 mg/dl (103 lmol/l; reference range 3.4-17 lmol/ l), LDH 4289 IU/l (reference range 150-500 IU/l), ammonia 102 lmol/l (reference range 34-47 lmol/l), lactate 9 mmol/l (reference range 0.9-1.7 mmol/l) and creatinine 94 lmol/l (reference range 27-62 lmol/l).Ultrasonography revealed a heterogeneous liver with multiple hypoechoic areas. Viral screen was negative. Bcell ALL, type L3 (Burkitt) was diagnosed from bone marrow examination done at 12 h.Early haemofiltration was started because of fulminant hepatic failure (FHF) and to prevent tumour lysis syndrome. Following pre-hydration and rasburicase, intravenous vincristine (1 mg/m 2 ) on days 1 and 8; and oral prednisolone (60 mg/m 2 ) daily (day 2 onward) was started. On day 4, after correction of her coagulopathy, a first dose of triple intrathecal chemotherapy (methotrexate 15 mg, cytosine arabinoside 30 mg and hydrocortisone 15 mg) was administered. The cytospin of the CSF was clear of leukaemic cells.
How Often Do You Fail to Take All of Your Medication?T o the Editor: We read with interest the article by Prieto-Jimenez et al (1); however, the conclusion overlooks the problem of adherence to treatment. A success rate of 44% has never been reported since dual therapies were introduced or in more recent pediatric trials. Compliance is the most important factor predicting treatment success (2), and eradication rates of 20% were reported in those taking <60% of pills (3).In the El Paso children, several indicators of poor adherence are present: treatment of asymptomatic disease, side effects/ bad taste of medication, patient's lack of belief in the benefits of treatment and of insight into the illness, complexity of treatment and possibility of receiving placebo, patients not asking for medical advice, and low income (4).That compliance was suboptimal is confirmed by a second article on the same cohort aiming at assessing iron stores after Helicobacter pylori treatment (5). How do the authors explain that after 6 months of iron administration, ferritin levels only showed a minimal increase after H pylori eradication? A similar experience revealed that in 2 months, children who received iron plus eradication doubled their ferritin content. ( 6) Perhaps, as in the case of antibiotics, they were poorly adherent to iron therapy. We have employed the sequential regimen to treat >200 children with an eradication rate >85% (7). Our data have been confirmed by a recent meta-analysis (eradication rate of 90.7%; 95% confidence interval 83.8%-94.9%) (8).Adherence is a primary determinant of treatment efficacy. When treatment outcome is markedly lower than expected, the key to the reading may well be the question ''how often do you fail to take all your medication?''
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