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BACKGROUND AND OBJECTIVES: The impact of chronic pancreatitis (CP) on quality of life (QOL) of children is not well established. Our objective was to evaluate the QOL, identify contributing factors, and determine the prevalence of anxiety and depression in children with CP in India. METHODS: Children (8-18y old) with CP were prospectively enrolled across three pediatric gastroenterology centres in India. QOL was assessed using the pediatric QOL inventory (PedsQL 4.0) scale, administered to both children and their parents. Anxiety and depression was studied using the Revised Children's Anxiety and Depression Scale (RCADS 25). Contributing factors were identified using binary logistic regression analysis. The data was compared against published QOL data in healthy Indian children. RESULTS: 121 children with CP (boys-57.9 %, age at QOL-14 ± 3.2years) were enrolled. A majority (82.7 %) had pain and advanced disease (Cambridge grade IV- 63.6 %). Children with CP had poorer QOL compared to controls (total score 74.6 ± 16 vs. 87.5 ± 11.1, p < 0.0001). QOL scores were similar across centres. Older children were similar to younger ones, except for a poorer emotional QOL. Taking QOL < -2 standard deviation (SD) of controls, â¼35 % had poor physical (50.9 ± 11.9) and 20 % had poor psychosocial (PS) QOL score (52.1 ± 7.2). On analysis, presence of pain and lower socio-economic status (SES) adversely affected both physical and PS-QOL. Additionally, girls had poorer PS-QOL than boys (Odds ratio 3.1, 95%CI:1.23-7.31). Anxiety and depression were uncommon (2,1.6 %). CONCLUSIONS: Patients with CP had impaired physical and psycho-social QOL. Presence of pain and lower SES adversely affected QOL. Psychiatric comorbidities were uncommon.
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Significance of autoantibodies in pediatric metabolic dysfunction-associated steatotic liver disease (MASLD) is unknown. Our aim was to determine the prevalence and significance of autoantibodies in MASLD. PubMed and Scopus were searched and six articles (689 [487 males] MASLD patients) were identified. Antinuclear antibodies (ANA) was positive in 28% (95% confidence interval [CI]: 17%-39%, n = 6 studies), Antismooth muscle antibodies (ASMA) in 28% (95% CI: 8%-50%, n = 5 studies), Actin-positive in 15% (95% CI: 10%-20%, n = 2 studies) and elevated immunoglobulin G in 17% (95% CI: 1%-39%, n = 4 studies). Anti-liver-kidney-microsomal antibody was not present in any patient. There was no significant association of ANA positivity with degree of liver steatosis, liver fibrosis or nonalcoholic fatty liver disease activity score (NAS) but patients with ASMA positivity had advanced fibrosis (pooled risk ratio [RR] 1.77; 95% CI 1.16-2.71) and higher risk of NAS ≥5 (pooled RR 1.21; 95% CI: 1.01-1.44, n = 2 studies, 243 patients). To conclude, non-organ specific autoantibodies are present in over one-fourth of children with MASLD and the presence of ASMA maybe associated with increased disease severity.
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OBJECTIVES: Radiological intervention (RI) is the preferred treatment in children with Budd-Chiari syndrome (BCS). We studied the comparative long-term outcome of BCS children, with and without RI and utility of liver and splenic stiffness measurement (LSM, SSM) by 2-dimensional shear wave elastography (2D-SWE) in assessing response. METHODS: Sixty children (40 boys, median age 10.5 [6.5-15.25] years) with BCS (29 newly diagnosed, 31 follow-up) were evaluated. LSM and SSM by 2D-SWE and vascular patency were monitored pre- and postprocedure (≥ 6 months postprocedure) in those undergoing RI. Medical therapy without anticoagulation and monitoring was done in subjects without RI. The RI and no-RI groups were compared. RESULTS: Ascites (54,90%), hepatomegaly (56,93%) and prominent abdominal-veins (42,70%), were the commonest features. The majority (46,78%) had isolated hepatic vein block. 44 (73%) cases underwent RI, while 16 (27%) were managed conservatively. Both groups were similar at baseline. Post-RI subjects showed significant improvement in clinical findings, liver functions and portal hypertension. LSM [33 (32-34.5) to 19.2 (18-20.67) kPa] and SSM [54.5 (52.3-57.6) to 28.9 (27.6-30.25) kPa] showed a significant decline from baseline value over a follow-up of 12 (6-13) months. Gradual reduction occurred in the LSM and SSM over 1-5 years, with near-normal LSM [10.2 (9.2-11.5) kPa] and SSM [22.3 (20.5-24.3) kPa] values in patients (n-16) with > 5 years follow-up. Patients without RI showed worsening in LSM and SSM. Hepatopulmonary syndrome and hepatocellular carcinoma developed in 4 (8%) and 1 (1.7%) cases respectively. CONCLUSION: RI leads to clinical recovery and reduction with near normalization of LSM and SSM over long-term follow-up in children with BCS. 2D-SWE is a promising tool to monitor outcomes.
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Síndrome de Budd-Chiari , Diagnóstico por Imagen de Elasticidad , Hipertensión Portal , Neoplasias Hepáticas , Masculino , Niño , Humanos , Síndrome de Budd-Chiari/diagnóstico por imagen , Síndrome de Budd-Chiari/terapia , Diagnóstico por Imagen de Elasticidad/métodos , Hígado/patología , Hipertensión Portal/patología , Neoplasias Hepáticas/patología , Cirrosis Hepática/patologíaRESUMEN
BACKGROUND: There are limited treatment options for children with steroid-refractory or dependent ulcerative colitis (UC). A few observational studies suggest efficacy of oral tacrolimus. We performed a systematic review and meta-analysis to assess the efficacy of tacrolimus in pediatric UC. METHODS: PubMed and Scopus were searched for publications related to the use of oral tacrolimus in pediatric UC. Data regarding the clinical response and colectomy-free survival were extracted from studies that met the selection criteria. RESULTS: The search strategy yielded 492 articles of which 7 studies were included in the final review. They included 166 children (111 steroid-refractory, 52 steroid-dependent, 3 no steroids). Majority of cases (150/166 [90%]) were naïve to biologics. An initial response to tacrolimus therapy was seen in 84% (95% CI: 73%-93%) (n = 7 studies). No difference was observed between children with high (>10 ng/mL) or low tacrolimus levels (127/150 [85%] vs 12/16 [75%], P = 0.3). No difference in initial response between the children who were steroid refractory or dependent (92/111 [83%] vs 46/52 [88%], P = 0.36). The response in the biologic-exposed group (n = 10) was 70%. At 1-year follow-up, 15.2% (95% CI: 7%-21%) (n = 2 studies, 85 patients) had a sustained response on only tacrolimus. The pooled frequency of 1-year colectomy-free survival in children treated with initial oral tacrolimus was 64% (95% CI: 53%-75%). Twelve (7.2%) patients required cessation of therapy because of side effects. CONCLUSION: Tacrolimus has a high initial response in biologic naïve UC children. It can be effectively used as a bridge to other therapies with a 1-year colectomy-free survival of 64%.
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Colitis Ulcerosa , Tacrolimus , Niño , Humanos , Colitis Ulcerosa/tratamiento farmacológico , Colitis Ulcerosa/cirugía , Inmunosupresores , Resultado del Tratamiento , Esteroides/uso terapéuticoRESUMEN
BACKGROUND AND AIM: Portal hypertension determines the outcome of children with biliary atresia (BA) and is common even after a successful Kasai portoenterostomy (KPE). However, there are no clear-cut guidelines on the age of starting surveillance and the modality (endoscopy vs non-invasive tests [NITs]). In this cohort study, we analyzed our database to find out the utility of NITs in detecting high-risk esophageal varices in BA. METHODS: From June 2010 to May 2022, consecutive children of BA who underwent upper gastrointestinal (UGI) endoscopy were included. Esophageal varices were classified as high-risk (grade II with red-color signs or grade III or IV irrespective of red-color signs. NITs such as splenomegaly (clinical and USG), platelet count, aspartate transaminase to platelet ratio index (APRI), and platelet-to-spleen diameter ratio were compared between cases with high-risk and low-risk varices. RESULTS: A total of 110 children, 75 boys (66 successful KPE and 44 failed/KPE not performed) were enrolled. The median age at KPE was 85 days (IQR 63-98). Thirteen (11.8%) children presented with UGI bleeding. The first endoscopy revealed gastroesophageal varices in 75.4% of cases, and 32% of them had high-risk varices. Multivariate analysis revealed failed KPE, history of UGI bleeding, bigger spleen size (> 3.5 cm), lower platelet count (< 150 000), and higher APRI (> 2) are independent predictors of the presence of high-risk esophageal varices. CONCLUSION: Endoscopy is the best in predicting the presence of high-risk varices that might bleed; hence, early surveillance endoscopy should be started in children with splenomegaly, thrombocytopenia, and high APRI score to prevent variceal bleeding.
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Atresia Biliar , Várices Esofágicas y Gástricas , Várices , Masculino , Niño , Humanos , Lactante , Várices Esofágicas y Gástricas/diagnóstico , Várices Esofágicas y Gástricas/etiología , Atresia Biliar/complicaciones , Atresia Biliar/diagnóstico , Atresia Biliar/cirugía , Esplenomegalia/diagnóstico por imagen , Esplenomegalia/etiología , Estudios de Cohortes , Hemorragia Gastrointestinal/diagnóstico , Hemorragia Gastrointestinal/etiología , Hemorragia Gastrointestinal/prevención & control , Endoscopía Gastrointestinal , Cirrosis HepáticaRESUMEN
Disease phenotype of pediatric inflammatory bowel disease (PIBD) in children from the Asia-Pacific region differs from that of children from the West. Many parts of Asia are endemic for tuberculosis, making diagnosis and management of pediatric Crohn's disease a challenge. Current available guidelines, mainly from Europe and North America, may not be completely applicable to clinicians caring for children with PIBD in Asia due to differences in disease characteristics and regional resource constraints. This position paper is an initiative from the Asian Pan-Pacific Society for Pediatric Gastroenterology, Hepatology and Nutrition (APPSPGHAN) that aims to provide an up-to-date, evidence-based approach to PIBD in the Asia-Pacific region. A group of pediatric gastroenterologists with a special interest in PIBD performed an extensive literature search covering epidemiology, disease characteristics and natural history, management, and monitoring. Attention was paid to publications from the region with special consideration to a resource-limited setting. This current position paper deals with surgical management, disease monitoring, immunization, bone health, and nutritional issues of PIBD in Asia. A special section on differentiating pediatric Crohn's disease from tuberculosis in children is included. This position paper provides a useful guide to clinicians in the surgical management, disease monitoring, and various health issues in children with IBD in Asia-Pacific region.
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Gastroenterología , Enfermedades Inflamatorias del Intestino , Tuberculosis , Humanos , Enfermedades Inflamatorias del Intestino/diagnóstico , Enfermedades Inflamatorias del Intestino/epidemiología , Enfermedades Inflamatorias del Intestino/cirugía , Asia/epidemiología , Fenotipo , Manejo de la EnfermedadRESUMEN
BACKGROUND: Pain is a major problem in 90% of patients with chronic pancreatitis (CP). Studies evaluating response to antioxidants (AO) are conflicting and no pediatric studies are available. AIMS: To evaluate markers of oxidative stress (OS), and efficacy and predictors of response to AO in improving pain in children with CP. METHODS: Antioxidants were given to CP children for 6 months. Subjects were assessed at baseline and post-therapy for pain and markers of OS [serum thiobarbituric acid reactive substances (TBARS), superoxide dismutase (S-SOD)] and antioxidant levels [vitamin C, selenium, total antioxidant capacity-ferric reducing ability of plasma (FRAP)]. Matched healthy controls were assessed for OS and antioxidant levels. Good response was defined as ≥ 50% reduction in number of painful days/month. RESULTS: 48 CP children (25 boys, age 13 years) and 14 controls were enrolled. 38/48 cases completed 6 months of therapy. CP cases had higher OS [TBARS (7.8 vs 5.2 nmol/mL; p < 0.001)] and lower antioxidant levels [FRAP (231 vs. 381.3 µmol/L; p = 0.003), vitamin C (0.646 vs. 0.780 mg/dL; p < 0.001)] than controls. Significant reduction in TBARS and S-SOD and increase in FRAP, vitamin C, and selenium occurred after 6 months. 10.5% cases had minor side effects. 26(68%) cases had a good response, with 9(24%) becoming pain-free. Subjects with severe ductal changes had lower median BMI (- 0.73 vs 0.10; p = 0.04) and responded less often than those with mild changes (17/29 vs 9/9; p = 0.036). CONCLUSION: CP children have higher OS than healthy controls. Antioxidant therapy is safe. Pain response is seen in 68% cases, less often in patients with severe ductal changes.
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Pancreatitis Crónica , Selenio , Masculino , Humanos , Niño , Adolescente , Antioxidantes/uso terapéutico , Antioxidantes/metabolismo , Selenio/uso terapéutico , Sustancias Reactivas al Ácido Tiobarbitúrico , Estrés Oxidativo/fisiología , Pancreatitis Crónica/complicaciones , Pancreatitis Crónica/tratamiento farmacológico , Ácido Ascórbico , Dolor/tratamiento farmacológico , Superóxido Dismutasa , Vitaminas/uso terapéuticoRESUMEN
INTRODUCTION: Relative adrenal insufficiency (RAI) is associated with poor outcome in adult cirrhotics. So far, pediatric studies are not available on the same. We aimed to prospectively study the presence and outcome of RAI in children with decompensated cirrhosis over 180 days. METHODS: Hemodynamically stable children with decompensated cirrhosis were sampled for serum basal cortisol and peak cortisol (after 30 minutes of 1-µg intravenous Synacthen) at day 1 and day 21. RAI was diagnosed as peak cortisol <500 nmol/L. Serum cytokines (interleukin-6 and tumor necrosis factor-α) and lipid profile were correlated with RAI. Cohort was followed up for outcomes over 180 days for complications and survival. With the identified risk factors, prognostic models were derived and compared with pediatric end-stage liver disease (PELD) and Child-Turcotte-Pugh scores. RESULTS: Prevalence of RAI was 54% at baseline and 61% at day 21 in the enrolled patients (n = 63, aged 128 ± 48 months, male 78%). No significant differences in cytokines and serum lipid levels were seen between RAI and normal adrenal function groups. Patients with RAI at baseline (D1-RAI) developed higher complications at follow-up as compared to the normal adrenal function group (53% vs 24%, P = 0.02). The PELD score (odds ratio 1.08, confidence interval 1.05-1.12, P < 0.01) and D1-RAI (odds ratio 3.19, confidence interval 1.32-7.73, P = 0.01) were independent predictors of follow-up complications. The PELD-delta cortisol model (area under the receiver operating curve 0.84, P < 0.001, 92% sensitivity; 60% specificity) predicted morbidity better than isolated PELD or Child-Turcotte-Pugh scores. DISCUSSION: RAI is a risk factor for development of complications in pediatric cirrhosis over short-term follow-up. The PELD-delta cortisol score is a promising prognostic model for predicting follow-up complications.
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Insuficiencia Suprarrenal/etiología , Cirrosis Hepática/complicaciones , Adolescente , Insuficiencia Suprarrenal/epidemiología , Niño , Preescolar , Femenino , Estudios de Seguimiento , Humanos , India/epidemiología , Masculino , Prevalencia , Pronóstico , Estudios Prospectivos , Factores de Riesgo , Índice de Severidad de la Enfermedad , Factores de TiempoRESUMEN
BACKGROUND AND AIM: Amitriptyline improves symptoms in functional abdominal pain disorders (FAPD) in adults with variable results in pediatric studies. The study aims to evaluate the efficacy of amitriptyline in pediatric FAPD. METHODS: In this open-label trial, children (≤ 18 years) diagnosed as FAPD based on ROME IV criteria were randomized to amitriptyline or placebo for 12 weeks. Post-treatment improvement of pain and quality of life (QOL) from the baseline were compared between the two groups. RESULTS: The mean age of 149 children (amitriptyline 75, placebo 74) was 11.3 ± 3.5 years (79 boys). There was a significant difference in pain improvement in terms of reduction in scores for intensity (3.4 vs 0.9), frequency (3.6 vs 0.6), duration (3.5 vs 0.9), and QOL (2.3 vs 0.9) between amitriptyline and placebo group (P < 0.001 in all). Responders (> 50% reduction) in pain was seen in 76% in amitriptyline compared with 14.9% in the placebo group (P < 0.001). On multivariate analysis, the use of amitriptyline was the only factor predictive of response (odds ratio 24.1, 95% confidence interval: 9.1-64.6, P < 0.001). Minor adverse events were comparable between the groups (25.3% vs 13.5%, respectively, P = 0.07). Eighty-nine percent of children (24/27) who had extended treatment duration (6.8 ± 1.8 months) had pain improvement. After discontinuation of amitriptyline, 70% had sustained response over a mean follow up of 15.84 ± 5.6 months. CONCLUSIONS: A 3-month trial of amitriptyline gives sustained relief of pain in two-thirds of children with FAPD. The safety profile of the drug and its efficacy necessitate more frequent use in the clinical settings.
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Amitriptilina , Síndrome del Colon Irritable , Dolor Abdominal/inducido químicamente , Dolor Abdominal/etiología , Adulto , Anciano de 80 o más Años , Amitriptilina/efectos adversos , Niño , Método Doble Ciego , Humanos , Síndrome del Colon Irritable/tratamiento farmacológico , Masculino , Calidad de Vida , Resultado del TratamientoRESUMEN
Pediatric inflammatory bowel disease (PIBD) is rising rapidly in many industrialised and affluent areas in the Asia Pacific region. Current available guidelines, mainly from Europe and North America, may not be completely applicable to clinicians caring for children with PIBD in this region due to differences in disease characteristics and regional resources constraints. This position paper is an initiative from the Asian Pan-Pacific Society for Pediatric Gastroenterology, Hepatology and Nutrition (APPSPGHAN) with the aim of providing an up-to-date, evidence-based approach to PIBD in the Asia Pacific region, taking into consideration the unique disease characteristics and financial resources available in this region. A group of pediatric gastroenterologists with special interest in PIBD performed an extensive literature search covering epidemiology, disease characteristics and natural history, management and monitoring. Gastrointestinal infections, including tuberculosis, need to be excluded before diagnosing IBD. In some populations in Asia, the Nudix Hydrolase 15 (NUD15) gene is a better predictor of leukopenia induced by azathioprine than thiopurine-S-methyltransferase (TPMT). The main considerations in the use of biologics in the Asia Pacific region are high cost, ease of access, and potential infectious risk, especially tuberculosis. Conclusion: This position paper provides a useful guide to clinicians in the medical management of children with PIBD in the Asia Pacific region.
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Radiological embolization is the treatment of choice in adults with visceral artery pseudoaneurysm (PSA) and gastrointestinal bleeding, but pediatric data is scanty. We analyzed the etiology, clinical presentation, and outcome of radiological intervention in children with PSA of celiac (CA) or superior mesenteric artery (SMA) branches. Electronic records of children with PSA of CA or SMA branches were reviewed and data on clinical and laboratory profile, radiological intervention, and outcome was recorded. Eleven children with PSA (5 boys, 11 [7-17] years) were studied. Etiology was liver abscess (n 4), abdominal trauma (n 3), pancreatitis (n 3), and indeterminate in 1 case. Ten (91%) patients were symptomatic: abdominal pain (10, 91%), hematemesis/melena (9, 81%), and Quincke's triad (1, 9%). One child with pancreatic pseudocyst was diagnosed incidentally on imaging. Doppler ultrasound identified PSA only in 3 cases, while computed tomography angiography (CTA) picked all cases. Children with liver abscess, trauma, and unknown etiology had PSA from CA (right hepatic artery 7, left hepatic artery 1). Of the 3 pancreatitis cases, 2 had PSA from SMA (inferior pancreatico-duodenal artery and ileal branch) and 1 from CA (left gastric artery). Radiological embolization was done in 9 (81%) cases (coil 6, glue 2, both 1), without any complications or failure. One case resolved spontaneously and 1 died pre-intervention. Nine intervened cases were asymptomatic in follow-up [6 (1-24) months].Conclusion: Liver abscess, trauma, and pancreatitis are causes of PSA of CA and SMA branches in children. A majority present with gastrointestinal bleeding and are identified on CTA. Radiological embolization was safe with 100% success. What is Known: ⢠Pseudoaneurysm of visceral artery is an uncommon cause of gastrointestinal bleeding. ⢠Endoluminal intervention is an established and efficacious treatment modality in adults and preferred over surgery. What is New: ⢠Liver abscess, abdominal trauma and pancreatitis are common causes of celiac artery and superior mesenteric artery branch pseudoaneurysm in children and computed tomography angiography has high sensitivity in identifying these pseudoaneurysms. ⢠Minimally invasive radiological angio-embolization, in the hands of trained radiologists, is a safe and successful modality of treatment in children.
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Aneurisma Falso , Embolización Terapéutica , Adulto , Aneurisma Falso/diagnóstico , Aneurisma Falso/diagnóstico por imagen , Angiografía , Niño , Hemorragia Gastrointestinal/diagnóstico , Hemorragia Gastrointestinal/etiología , Hemorragia Gastrointestinal/terapia , Arteria Hepática , Humanos , MasculinoRESUMEN
BACKGROUND/OBJECTIVES: Endoscopic transmural drainage is the preferred method of drainage of pancreatic fluid collections (PFCs) in adults; however, there is scant literature in children. We analyzed our experience of 33 endoscopic cystogastrostomies done in 29 children to find its efficacy and safety. METHODS: We retrospectively analyzed the prospectively collected database of 31 consecutive children (<18 years) who underwent endoscopic cystogastrostomy from June 2013 to December 2017. The procedure was done using the standard technique with an adult duodenoscope. Data related to clinical details, technical success, complications and follow-up were collected. RESULTS: The median age was 14 (3-17) years (22 males). Indications were early satiety in 28 (90%), vomiting in 15 (48%), and duodenal obstruction and infected pseudocyst in 2 children each. Etiology includes acute pancreatitis 22, post-traumatic 4 and chronic pancreatitis 5. The procedure was successful in 29 of 31 (93.5%) children with no mortality. Adverse events happened in four cases (12.9%); two infections, another with bleeding and another with pneumoperitonium, both of which resolved spontaneously. Incidents (minor bleeding) were noted in 6 (19%). Stents were removed in 26 (90%) after 12 (7-20) weeks and got spontaneously migrated out in 3 (10%) cases. Over a median follow-up of 26 (5-48) months, 26 (90%) had no recurrence of pseudocyst and 3 (10%) had recurrence of a small, asymptomatic pseudocyst. CONCLUSIONS: Endoscopic cystogastrostomy is a safe and effective method of draining bulging PFCs in children. The procedure carries acceptable morbidity with minimal recurrence. In younger children it may be the preferred method of drainage of PFCs.
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Seudoquiste Pancreático , Pancreatitis , Enfermedad Aguda , Adolescente , Adulto , Niño , Drenaje , Humanos , Masculino , Seudoquiste Pancreático/cirugía , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
BACKGROUND: Malnutrition and bone disease are common in adults with chronic pancreatitis (CP). We studied the nutritional status and bone mineral density (BMD) of children with CP and the factors predicting them. METHODS: CP children were prospectively evaluated with a detailed questionnaire, anthropometry, 25-hydroxy vitamin D, fecal elastase and BMD [total body less head (TBLH), spine and hip] by dual energy x-ray absorptiometry. Body mass index (BMI) Z score of -1 to -1.9, -2 to -2.9 and <-3 was taken as mild, moderate and severe malnutrition respectively. Low BMD and osteoporosis were defined as per International Society for Clinical Densitometry. RESULTS: 83 children (46 boys, 14[4.3-21]years) with CP were enrolled. Majority had Cambridge IV (51,62.2%) or III (15,18.3%) changes. 34(41%) had undernutrition (mild-37.3%, moderate-2.4%, severe-1.2%). Overweight and obesity were present in 3.6% and 1.2% cases. BMI had a significant correlation with haemoglobin, serum albumin, percentage body fat and BMD. A majority had low fecal elastase (69 [84.1%], <100 µg/g) and vitamin D deficiency (70[84.3%],<20 ng/ml). 9 cases had a history of fractures. 14/75(18.6%) cases had low TBLH-BMD and this group had a lower BMI (-1.3[-1.9 to 0.34] vs 0.8 [-2.1 to 5.50; p = 0.03) than patients with normal BMD. There was no difference in age, disease duration, vitamin D, fecal elastase and Cambridge grade between normal and low BMD. CONCLUSIONS: 41% CP children have undernutrition with a majority having mild undernutrition. Nearly 20% have low BMD, with osteoporosis in none. Subjects with low BMI have lower BMD and percentage body fat.
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Densidad Ósea , Trastornos de la Nutrición del Niño/complicaciones , Estado Nutricional , Pancreatitis Crónica/complicaciones , Adolescente , Niño , Preescolar , Femenino , Humanos , Masculino , Prevalencia , Estudios Prospectivos , Deficiencia de Vitamina D , Adulto JovenRESUMEN
BACKGROUND: The utility of measuring thiopurine metabolites (TM) to individualize therapy in autoimmune hepatitis (AIH) has not been defined, and the evidence regarding its use in clinical practice is heterogeneous. This systematic review and meta-analysis aimed to compare the mean concentration of TM between patients in biochemical remission and those not in remission. METHODS: A systematic literature search was conducted using PubMed, Scopus, the Cochrane Library, and Google Scholar for keywords related to TM and AIH. Two reviewers independently searched and selected studies comparing the levels of 6-methyl mercaptopurine (6-MMP) and 6-thioguanine nucleotide (6-TGN) and their ratio in cases of AIH in remission and otherwise. Meta-analysis was performed by calculating the weighted mean difference using the inverse variance heterogeneity model. RESULTS: A total of 1066 records were identified through systematic search; of which, 7 (n = 3 pediatric, n = 4 adults) were considered for inclusion, and 442 TM measurements (n = 128 in children) were analyzed. Mean 6-TGN levels were significantly higher among patients in remission than in those who were not, with a pooled weighted mean difference (WMD) of 15.67 [95% confidence interval (CI), 6.68-24.66] pmol/8 × 108 red blood cells (RBC). The difference was higher in the pediatric age group (WMD, 56.11; 95% CI, 13.60-98.62) than in adults (WMD, 13.77; 95% CI, 4.58-22.97). There was no significant difference in the 6-MMP levels (WMD, -431.7; 95% CI, -1237.4 to 373.9 pmol/8 × 108 RBC; I2 = 82%; n = 3 studies) or 6-MMP/6-TGN ratio among the patients who were in biochemical remission and those who were not (WMD, -0.97; 95% CI, -5.77 to 3.84; I2 = 82%; n = 3 studies). CONCLUSIONS: This meta-analysis suggests a link between 6-TGN levels and biochemical remission in AIH. Further high-quality studies are required to determine the therapeutic cutoff of 6-TGN.
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Hepatitis Autoinmune , Tioguanina , Adulto , Azatioprina/farmacocinética , Azatioprina/uso terapéutico , Niño , Hepatitis Autoinmune/tratamiento farmacológico , Humanos , Inmunosupresores/farmacocinética , Inmunosupresores/uso terapéutico , Mercaptopurina/farmacocinética , Tioguanina/farmacocinéticaRESUMEN
INTRODUCTION: There are gaps in the literature regarding outcome of multiple polyps and dilemmas in the management issues in polyposis syndromes in children. OBJECTIVE: We aimed to study the clinical behaviour of gastrointestinal (GI) polyps with emphasis on therapeutic outcomes of polyposis syndrome. METHODS: Proven cases of GI polyp(s) on endoscopy were classified as single polyp, multiple polyps, and polyposis syndrome. Complex presentation was defined as 1 or more of the following: severe anaemia, anasarca, intussusception, rectal mucosal prolapse, and diarrhoea. A clinico-endoscopic criterion was applied in polyposis syndrome patients for the decision of surgery versus endoscopic therapy with surveillance. RESULTS: Of total 240 patients, there were no significant differences between single (52.5%, n = 126) versus multiple polyps (27.5%, n = 66) with respect to age, symptoms, histology, and recurrence. Polyposis syndrome (20%, n = 48) presented with complex symptoms (50%), higher family history, significantly lower haemoglobin, total protein, and albumin as compared to single and multiple polyps (p < 0.01). Nineteen polyposis patients with favourable clinico-endoscopic criteria were endoscopically eradicated for polyps in 3 (1-4) sessions with sustenance of laboratory parameters at 1 year and 30% symptomatic recurrence at follow-up of 23.5 (7-40) months. There were no major endoscopic complications. Nineteen patients required proctocolectomy with improvement in laboratory parameters 6 months post-surgery. CONCLUSIONS: Multiple polyps behave similar to single polyps in children. A clinico-endoscopic criterion may guide for optimal management of polyposis syndrome. Colectomy may be effectively deferred in a large proportion of polyposis syndrome patients if maintained on an endoscopic protocol.
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Poliposis Adenomatosa del Colon/cirugía , Endoscopía , Pólipos/cirugía , Poliposis Adenomatosa del Colon/patología , Adolescente , Niño , Preescolar , Femenino , Humanos , Lactante , Masculino , Pólipos/patología , Resultado del TratamientoRESUMEN
BACKGROUND AND AIM: Functional dyspepsia (FD) is common in children, and treatment targeted towards the altered pathophysiology can improve outcome. We evaluated FD children for abnormality of gastric accommodation and emptying, psychological stressors (PS), Helicobacter pylori (HP) infection, and post-infectious FD. METHODS: Diagnosis of FD was based on ROME III criteria. Clinical evaluation including dyspeptic symptom scoring and assessment for PS was performed. Satiety drink test for gastric accommodation, gastroscopy with biopsy for HP infection, and solid meal gastric emptying were performed. Sixty-seven healthy children were enrolled for assessing PS and satiety drink test. RESULTS: Fifty-five FD children (33 boys, age 12 [6-18] years) with symptoms for 4 (2-48) months and dyspeptic score of 5 (1-13) were enrolled. PS were more common in FD than in controls (46/55 vs 9/67; P < 0.001). Median satiety drink volume was 360 mL (180-1320 mL); no patients had satiety drink volume of < 5th centile of healthy children. The frequency (98% vs 85%; P = 0.01) and severity (65 [10-175] vs 50 [5-130]; P < 0.001) of postprandial symptoms were higher in FD than in controls. Of the postprandial symptoms, pain (20.3% vs 0%; P = 0.000) was present only in FD. Delayed gastric emptying was present in 6.5%, HP infection in 11%, and post-infectious FD in 13% cases. Etiological factor was identified in 87% children, with 20% having multiple factors. CONCLUSIONS: Abnormality of gastric sensorimotor function is seen in one-fourth of FD cases. HP infection and post-infectious FD are present in 11% and 13% cases, respectively.
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Dispepsia/diagnóstico , Dispepsia/etiología , Adolescente , Niño , Técnicas de Diagnóstico del Sistema Digestivo , Dispepsia/fisiopatología , Femenino , Vaciamiento Gástrico , Gastritis/complicaciones , Gastritis/microbiología , Infecciones por Helicobacter , Humanos , Masculino , Índice de Severidad de la Enfermedad , Estrés Psicológico/complicacionesRESUMEN
OBJECTIVES: To study the presentation, management strategies and long-term natural history of children with pancreatic trauma. METHODS: Children admitted with pancreatic trauma were analyzed for their presentation, management and outcome. Management included nasojejunal feeds, total parenteral nutrition (TPN), octreotide, drainage (radiological and endoscopic), endoscopic retrograde cholangiopancreatography (ERCP) and surgery. Patients were assessed in follow-up for development of chronic pancreatitis (CP). RESULTS: 36 children [29 boys, age 144 (13-194) months] presented at 30 (3-210) days after trauma. Most common cause of trauma was bicycle handle bar injury [nâ¯=â¯18,50%]. Presenting features were abdominal pain [nâ¯=â¯26,72%], lump [nâ¯=â¯16, 44.4%], ascites [nâ¯=â¯13,36%], pleural effusion [nâ¯=â¯9,25%] and anasarca [nâ¯=â¯3,8.3%]. All presented with sequelae of ductal disruption with pseudocyst, ascites or pleural effusion. Fifteen (41.6%) patients each had Grade III and IV injury, 4 (11%) had grade V, and grading was unavailable in 2. Other organs were injured in 4 (11%) cases. Management consisted of various combinations of nasojejunal feeds [nâ¯=â¯17,47.2%], TPN [nâ¯=â¯5,13.8%], octreotide [nâ¯=â¯13,36%], pseudocyst drainage [radiological (nâ¯=â¯18,50%), endoscopic (nâ¯=â¯3,8.3%)] and ERCP [nâ¯=â¯12,33.3%]. Surgical intervention was done in 2 (5.5%) cases [cystojejunostomy and peritoneal lavage in 1 each]. Two (5.5%) patients died due to sepsis. Of the 32 cases in follow-up, 19 (59.3%) recovered and 13 (40.6%) developed CP, with half (6/13) of them being symptomatic with recurrent pain. CONCLUSION: Multi-disciplinary non-operative management is effective for managing pancreatic trauma in 94.4% of children, with 75% requiring radiological or endoscopic intervention. 40% developed structural changes later but only half were symptomatic.
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Páncreas/lesiones , Heridas y Lesiones/terapia , Niño , Femenino , Humanos , Masculino , Pancreatitis Crónica/diagnóstico por imagen , Pancreatitis Crónica/etiología , Resultado del TratamientoRESUMEN
BACKGROUND AND AIMS: The medical literature on colonic changes in children with extrahepatic portal venous obstruction (EHPVO) is limited. We evaluated EHPVO children for prevalence, nature, and relation of colonic changes with disease duration, extent of splenoportal axis (SPA) thrombosis, portal hypertensive gastropathy (PHG), and esophageal varices (EVs). The correlation between histologic and endoscopic changes was studied. METHODS: Subjects were evaluated by colonoscopy with ileoscopy and biopsy sampling, clinico-laboratory profiles, and SPA imaging. Colonic changes were classified as varices (rectal/colonic) and portal hypertensive colopathy (PHC; colitis-like or vascular lesions). Morphometric analysis of colonic biopsy specimens was performed. RESULTS: Fifty-four children (median age, 12 years [range, 8-15]; hematochezia in 9 [16.6%]) were evaluated. Rectal and colonic varices were seen in 51 (94%) and 2 (3.7%) cases, respectively. Seventy-five percent of patients had PHC, and colitis-like lesions were more common than vascular lesions (36/40 vs 23/40; P = .001). Colopathy changes were pancolonic in 52.5%, left-sided in 42.5%, and right-sided in 5% of cases. Sixteen percent of patients (8/49) had ileal changes. Children with PHC had PHG more often (90% vs 57%; P = .01), more endotherapy sessions (mean 6 [range, 4-8] vs 2 [range, 1-4]; P = .03), and large EVs less often (12.5% vs 43%; P = .02) than those without colopathy. The extent of SPA thrombosis was similar in patients with and without PHC. The number of capillaries per crypt was higher in EHPVO than in control subjects. Morphometric changes had no correlation with endoscopy. CONCLUSIONS: Most EHPVO children had colonic changes, and 16% had ileopathy. "Colitis-like" changes and left-side involvement were more common. Patients with PHG and eradicated EVs had a higher risk of PHC.
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Vena Porta , Adolescente , Niño , Várices Esofágicas y Gástricas , Hemorragia Gastrointestinal/epidemiología , Hemorragia Gastrointestinal/etiología , Humanos , Hipertensión Portal/complicaciones , Hipertensión Portal/epidemiología , Vena Porta/diagnóstico por imagen , PrevalenciaRESUMEN
BACKGROUND & AIMS: Early detection of raised intracranial pressure (ICP) improves outcome in acute liver failure (ALF). We evaluated the feasibility of bedside, ultrasound-guided measurement of optic nerve sheath diameter (ONSD) in normal and ALF children and correlation of ONSD with grade of hepatic encephalopathy (HE), international normalized ratio (INR) and blood ammonia (BA). METHODS: Forty-one ALF and 47 healthy children (5-18 years) were prospectively enrolled and 12 hourly clinical evaluation was done. Laboratory parameters including INR and BA were recorded. ONSD was measured at admission, change in HE grade and at recovery in ALF, and once in controls. RESULTS: One hundred and twenty-one ONSD measurements (ALF-74, controls-47) were taken without complications. ONSD was 4.2 (3.9-4.3), 4.4 (4.0-4.6), 5.2 (4.8-5.8) and 3.9 (3.3-4.1) mm in controls, ALF without HE, with HE and at recovery respectively. ONSD was significantly higher in ALF with HE than those without HE. ALF without HE and at recovery had ONSD similar to controls. ONSD was higher in ALF with the clinical signs of raised ICP than those without (5.4 [4.9-5.7] vs 4.6 [4.1-5.3] mm; P = .01). ONSD of 4.6 mm differentiated ALF with HE vs without HE and 5.1 mm between poor vs good non-transplant outcome with ≥80% sensitivity and specificity. ONSD positively correlated with INR (r = .53, P < .001) and BA (r = .42, P = .002). CONCLUSIONS: ONSD can be safely and easily measured in ALF children and correlates with HE grade, INR and BA. Normal ONSD in children (>4 years) is <4.5 mm and value of >5.1 mm in ALF requires urgent attention.
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Presión Intracraneal , Fallo Hepático Agudo , Adolescente , Niño , Preescolar , Humanos , Nervio Óptico/diagnóstico por imagen , Proyectos Piloto , Estudios Prospectivos , UltrasonografíaRESUMEN
BACKGROUND: Late-onset hepatic failure (LOHF) is a distinct entity of intractable liver diseases with limited pediatric experience. We aimed to identify the etiology and risk factors that determine the poor outcome (PO) of pediatric LOHF. METHODS: LOHF was defined as liver failure occurring 5-24 weeks after onset of jaundice and without any evidence of underlying chronic liver disease. PO (death or liver transplantation within 160 days) was compared with spontaneous recovery (SR; complete normalization of liver functions in the native liver). Pediatric end-stage liver disease (PELD) score and King's College Criteria (KCC) were applied to investigate their prognostic value. RESULTS: We enrolled 47 children (6 [2-16] years) with LOHF. Hepatitis A was the most common etiology (15, 32%) and 64% complicated with infections. Twelve children (25%) had SR over 6 (1-24) months, while 28 (60%) children had PO. Univariate analysis showed indeterminate etiology, hepatic encephalopathy (HE), infection, acute kidney injury, and high PELD score determined PO. On multivariate regression analysis, only PELD score with a cutoff 32 (area under curve 0.833, sensitivity 68%, specificity 92%) predicted PO. KCC showed a sensitivity of 85.7%, specificity of 41.7% to determine PO in our cohort. CONCLUSION: Indeterminate etiology, presence of HE, occurrence of infection at any site, and acute kidney injury lead to the PO. PELD score ≥32 can be utilized to optimize the listing for liver transplantation. A significant proportion survives with the native liver.