Shear-wave elastography for monitoring Fontan-associated liver disease: A prospective cohort study.

The spectrum of Fontan-associated liver disease (FALD) varies from abnormal liver function tests to fibrosis and even cirrhosis. In this prospective study, we evaluated the role of shear-wave elastography (SWE) in predicting the presence of advanced FALD. Forty-eight patients (30 males, 13.9 [6-21] years) with a Fontan circulation were evaluated at 8.3 (2.1-18.7) years since the Fontan surgery. The median liver stiffness measurement (LSM) value was higher than values in normal children at 15.4 (9.5-38.7) kPa. The LSMs had a weak but significant correlation with age at the time of LSM (r = 0.25, p = 0.01) and duration post-Fontan surgery (r = 0.31, p = 0.02). It had a poor correlation with the concomitant aspartate transaminase-to-platelet ratio index (r = 0.1, p = 0.39). No difference in the elastography values between children with and without ultrasound evidence of advanced liver disease (17.7 [interquartile range, IQR: 4] vs. 16.1 [IQR: 6], p = 0.62] was observed. Further studies are required to determine the precise role of SWE as a noninvasive marker of liver fibrosis in FALD.

Celiac Disease in Children: A 2023 Update.

Celiac disease (CeD) is a chronic immune-mediated enteropathy, which occurs in genetically predisposed individuals by the ingestion of gluten proteins present in wheat, barley and rye. The global pooled prevalence of CeD is 0.7% and it has been reported from nations all around the globe and can affect individuals of any age. It has a wide clinical spectrum ranging from being asymptomatic to being symptomatic with severe manifestations. Though initial descriptions of CeD focused on the classical presentation with gastrointestinal manifestations, in recent years it has been found that more patients have non-classical manifestations such as anemia, osteoporosis, increased transaminases, failure to thrive or short stature. The definitive diagnosis of CeD is based on a combination of clinical history, serologic testing with/without examination of duodenal biopsies. The preferred initial serologic test regardless of age for the detection of CeD is the tissue transglutaminase (IgA anti-tTG). Children with a high tTG-IgA (≥10 ULN) AND a positive anti-endomysial IgA antibody (EMA) can be diagnosed to have CeD without the need for duodenal biopsies. The rest should undergo biopsies with at least 4 biopsies from the distal duodenum and at least 1 from the bulb. A correctly orientated biopsy showing increased intraepithelial cells and a villous to crypt ratio of <2 is suggestive of CeD. The management of CeD is a lifelong complete dietary avoidance of gluten. IgA-TGA acts as a surrogate marker for healing of the small-bowel mucosa and should be performed every 6 mo until normalization and then every 12-24 mo thereafter.

Characterization of Colonoscopies in Preschool Children.

OBJECTIVES: The indications, diagnostic yield, complications, and cecal and ileal intubation rates (CIR and IIR) for colonoscopies in children aged <6 years, denoted preschoolers, is unclear since there is limited information for this group. We aimed to describe the above parameters in our cohort of preschoolers undergoing a colonoscopy. METHODS: Retrospective review of all colonoscopies in a tertiary pediatric hospital between December 1, 2014 to December 31, 2020 was undertaken. Demographic factors, indication for colonoscopy, extent of colonoscopy, CIR, IIR, and histologic findings were noted. Preschoolers were further subdivided into those aged <2 years, and those aged 2 to <6 years. RESULTS: One thousand six hundred seventy-one total colonoscopies were performed, of which 13% (n = 219) were in preschoolers with median age 3.9 (range 0.3-5.9) years. Most common indications in preschoolers were rectal bleeding 35% (n = 78), inflammatory bowel disease 24% (n = 53), diarrhea 13% (n = 30), iron-deficiency anemia 11% (n = 25), and abdominal pain 7% (n = 16). IIR and CIR were lower in preschoolers compared to older children, 81% vs 92% ( P = 0.0001), and 93% vs 96.4% ( P = 0.02), respectively, and even lower in those aged <2 years, 48.1% IIR ( P = 0.0001) and 85.1% CIR. Juvenile polyps, 31% (n = 27), were the most common positive finding in preschool children. CONCLUSION: Rectal bleeding was the most common indication and juvenile polyps the most common finding at colonoscopy in preschoolers. A high IIR is achievable in young children but rates are increasingly lower the younger the child.

SYSTEMATIC REVIEW AND META - ANALYSIS OF THE FREQUENCY AND RE-CLASSIFICATION TRENDS OF PEDIATRIC INFLAMMATORY BOWEL DISEASE - UNCLASSIFIED.

BACKGROUND: The term inflammatory bowel disease-unclassified (IBDU) is used when an individual has chronic colitis but cannot be sub-typed into ulcerative colitis (UC) or Crohn's disease (CD) on the basis of the clinical, endoscopic, imaging and histopathological features. On follow-up a proportion of patients with IBDU are re-classified as CD or UC. There has been considerable variability in the frequency and reclassification rates of pediatric IBDU in published literature. METHODS: PubMed and Scopus and were searched for publications related to Pediatric Inflammatory Bowel Disease (PIBD) published between Jan,2014 and July,2021. Two reviewers independently searched and selected studies reporting the frequency of IBDU and/or their re-classification. The pooled prevalence was expressed as proportion and 95%CI. Meta-analysis was performed using the inverse variance heterogeneity model. RESULTS: A total of 2750 studies were identified through a systematic search of which 27 studies were included in this systematic review. The overall pooled frequency of IBDU (n=16064) was found to be 7.1% (95%CI 5.8-8.5%). There was no variation in IBDU frequency by geographical location. Seven studies (n=5880) were included in the IBDU re-classification analysis. Overall, 50% (95%CI 41-60%) children with IBDU were re-classified on follow-up. Amongst these 32.7% (95% 21-44%) were re-classified to UC and 17% (95%CI 12-22%) were re-classified to CD. CONCLUSION: IBDU comprises 7.1% of PIBD at initial diagnosis. Half of these children are re-classified into UC or CD on follow-up with a higher likelihood of re-classification to UC as compared to CD.

Revisão sistemática e meta-análise das tendências de frequência e reclassificação da doença inflamatória pediátrica intestinal - não classificadas / Systematic review and meta - analysis of the frequency and re-classification trends of pediatric inflammatory bowel disease - unclassified

ABSTRACT Background: The term inflammatory bowel disease-unclassified (IBDU) is used when an individual has chronic colitis but cannot be sub-typed into ulcerative colitis (UC) or Crohn's disease (CD) on the basis of the clinical, endoscopic, imaging and histopathological features. On follow-up a proportion of patients with IBDU are re-classified as CD or UC. There has been considerable variability in the frequency and reclassification rates of pediatric IBDU in published literature. Methods: PubMed and Scopus and were searched for publications related to Pediatric Inflammatory Bowel Disease (PIBD) published between Jan,2014 and July,2021. Two reviewers independently searched and selected studies reporting the frequency of IBDU and/or their re-classification. The pooled prevalence was expressed as proportion and 95%CI. Meta-analysis was performed using the inverse variance heterogeneity model. Results: A total of 2750 studies were identified through a systematic search of which 27 studies were included in this systematic review. The overall pooled frequency of IBDU (n=16064) was found to be 7.1% (95%CI 5.8-8.5%). There was no variation in IBDU frequency by geographical location. Seven studies (n=5880) were included in the IBDU re-classification analysis. Overall, 50% (95%CI 41-60%) children with IBDU were re-classified on follow-up. Amongst these 32.7% (95% 21-44%) were re-classified to UC and 17% (95%CI 12-22%) were re-classified to CD. Conclusion: IBDU comprises 7.1% of PIBD at initial diagnosis. Half of these children are re-classified into UC or CD on follow-up with a higher likelihood of re-classification to UC as compared to CD.

RESUMO Contexto: O termo doença inflamatória intestinal não classificada (DIINC) é usado quando um indivíduo tem colite crônica, mas não pode ser sub tipificado em colite ulcerativa (UC) ou doença de Crohn (DC) com base nas características clínicas, endoscópicas, de imagem e histopatológicas. No acompanhamento, uma proporção de pacientes com DIINC são reclassificadas como DC ou UC. Houve considerável variabilidade nas taxas de frequência e reclassificação de DIINC pediátrico na literatura publicada. Métodos: Foram procuradas publicações no PubMed e Scopus relacionadas à doença inflamatória pediátrica intestinal publicadas entre janeiro de 2014 e julho de 2021. Dois revisores pesquisaram e selecionaram estudos independentemente relatando a frequência da DIINC e/ou sua reclassificação. A prevalência agrupada foi expressa em proporção e para IC95%. A meta-análise foi realizada utilizando o modelo de heterogeneidade de variância inversa. Resultados: Foram identificados 2.750 estudos por meio de uma busca sistemática, dos quais 27 estudos foram incluídos nesta revisão sistemática. A frequência total agrupada da DIINC (n=16064) foi de 7,1% (IC95% 5,8-8,5%). Não houve variação na frequência da DIINC por localização geográfica. Sete estudos (n=5880) foram incluídos na análise de reclassificação da DIINC. No geral, 50% (IC95% 41-60%) foram reclassificadas no seguimento. Entre esses 32,7% (95% 21-44%) foram reclassificados para UC e 17% (IC95%12-22%) foram reclassificados para DC. Conclusão: DIINC compreende 7,1% da doença inflamatória pediátrica intestinal no diagnóstico inicial. Metade dessas crianças são reclassificados em UC ou DC no seguimento com maior probabilidade de reclassificação para UC em comparação com o DC.

Current practices in the management of corrosive ingestion in children: A questionnaire-based survey and recommendations.

BACKGROUND AND AIM: Corrosive ingestion causes significant morbidity in children with no standard guidelines regarding management. This survey aimed to understand practices adopted by gastroenterologists, identify lacunae in evaluation and management and suggest a practical algorithm. METHODS: Indian gastroenterologists participated in an online survey (65 questions) on managing corrosive ingestion. When ≥ 50% of respondents agreed on a management option, it was considered as 'agreement'. RESULTS: Ninety-eight gastroenterologists (72 pediatric) who had managed a total of ~ 2600 corrosive ingestions in the last 5 years responded. The commonest age group affected was 2-5 years (61%). Majority of ingestion was accidental (89%) with 80% due to improper corrosive storage. Ingestion of alkali and acid was equally common (alkali 41%, acid 39%, unknown 20%). History of inducing-vomiting after ingestion by community physicians was present in 57%. There was an agreement on 77% of questions. The respondents agreed on endoscopy (70%) and chest X-ray (67%) in all, irrespective of symptoms. Endoscopy was considered safe on days 1-5 after ingestion (91%) and relatively contraindicated thereafter. The consensus was to use acid suppression, always (59%); steroids, never (68%) and antibiotics, if indicated (59%). Feeding was based on endoscopic findings: oral in mild injuries and nasogastric (NG) in others. Eighty percent placed a NG tube under endoscopic guidance. Stricture dilatation was considered safe after 4 weeks of ingestion. Agreement on duration of acid suppression and stricture management (dilatation protocol and refractory strictures) was lacking. CONCLUSION: Corrosive ingestion mostly affects 2-5-year olds and is accidental in majority. It can be potentially prevented by proper storage and labelling of corrosives. An algorithm for management is proposed.

Unusual cause of severe iron deficiency anaemia in a child: paraoesophageal hernia.

Iron deficiency anaemia is a known complication of a large hiatal hernia in adults. It occurs as a result of erosions on the gastric mucosa secondary to traction at the hiatus during respiration and/or gastric acid-related injuries to the mucosa. Even though anaemia occurs as a result of chronic gastrointestinal blood loss, testing for faecal occult blood is often negative and upper gastrointestinal endoscopy normal as the bleeding is intermittent. In children, a hiatus hernia as a rare cause of iron deficiency anaemia and has been described only in case reports. Here, we describe a 5-year-old boy who presented with severe transfusion-dependent iron deficiency anaemia caused by a paraoesophageal hernia. Surgical repair of the hiatus hernia led to complete resolution of anaemia. One should consider a hiatus hernia as a diagnostic possibility when evaluating a child with refractory iron deficiency anaemia.

Leptospira Triggered Anti-N-Methyl-d-Aspartate Receptor Encephalitis.

Primary neuroleptospirosis although rare but has been reported in the literature in the form of case reports and case series. However, there are no reports of autoimmune encephalitis triggered by leptospirosis in the literature, although four cases of acute disseminated encephalomyelitis, which is also considered to have autoimmune etiology have been reported. We are reporting an adolescent girl, who developed anti-N-methyl-d-aspartate receptor encephalitis after the resolution of systemic symptoms of leptospirosis. Her symptoms including neuropsychiatric and extrapyramidal features and sleep disturbances resolved completely after immunotherapy. As recently autoimmune encephalitis triggered by various infections are getting reported more frequently around the world, the clinicians need to consider this clinical possibility, even in patients with leptospirosis, who develop neurological symptoms while systemic clinical features are subsiding. Early recognition and timely administration of immunotherapy have the potential to completely reverse the neurological symptoms.

Paediatric inflammatory bowel disease in India: a prospective multicentre study.

BACKGROUND: Paediatric inflammatory bowel disease (PIBD) is increasing across the world. However, information from India is sparse. This multicentre study evaluated the demographics, clinical phenotype and outcome of PIBD from India. METHODS: Data of children (≤18 years) with PIBD were collected using a proforma containing details of demographics, clinical profile, extraintestinal manifestations (EIM), investigations, disease extent and treatment. RESULTS: Three hundred twenty-five children [Crohn's disease: 65.2%, ulcerative colitis: 28.0%, IBD unclassified (IBDU): 6.7%, median age at diagnosis: 11 (interquartile range 6.3) years] were enrolled. 6.9% children had family history of IBD. Pancolitis (E4) was predominant in ulcerative colitis (57.8%) and ileocolonic (L3, 55.7%) in Crohn's disease. Perianal disease was present in 10.9% and growth failure in 20.9% of Crohn's disease cases. Steroids were the initial therapy in 84.2%, 5-amino salicylic acid in 67.3% and exclusive enteral nutrition (EEN) in 1.3% cases. Overall, immunomodulators and biologics were given to 84.3 and 17.9% cases, respectively, and 2.9% cases underwent surgery. Very early onset IBD (VEOIBD) was seen in 60 (19.2%) children. IBDU was commoner in the VEOIBD than the older-PIBD (18/60 vs 4/253; P < 0.001). VEOIBD-Crohn's disease patients more often had isolated colonic disease than the older Crohn's disease (45.4% vs 11.8%; P < 0.001). Prevalence of perianal disease, EIM, therapeutic requirements and outcome were not different between VEOIBD and older-PIBD. CONCLUSION: Disease location and phenotype of PIBD in Indian children is similar to the children from the west. However, the therapeutic options of EEN, biologics and surgery are underutilized. VEOIBD accounted for 19.2% of PIBD.

Consumptive Hypothyroidism Due to Diffuse Hepatic Hemangiomas Treated With Propranolol Therapy.

Infantile hepatic hemangioma (IHH)-related consumptive hypothyroidism is rare and occurs as a result of excess thyroid hormone inactivating enzyme, type-3 iodothyronine deiodinase. We present an infant with IHH-related hypothyroidism, in whom treatment with propranolol led to regression of tumor and subsequent euthyroid status.

Normal Gamma Glutamyl Transferase Levels at Presentation Predict Poor Outcome in Biliary Atresia.

OBJECTIVES: Gamma-glutamyl transferase levels (GGT) are typically elevated in biliary atresia (BA), but normal GGT levels have been observed. This cohort of "normal GGT" BA has neither been described nor has the prognostic value of GGT level on outcomes in BA. We aimed to describe outcomes of a single-centre Australian cohort of infants with BA and assess the impact of GGT level at presentation on outcomes in BA. METHODS: Infants diagnosed with BA between 1991 and 2017 were retrospectively analysed. Outcomes were defined as survival with native liver, liver transplantation (LT), and death. Patients were categorized into normal (<200I U/L) or high GGT groups based on a mean of 3 consecutive GGT values done before Kasai portoenterostomy (KPE). Baseline parameters, age at surgery, clearance of jaundice (COJ), and outcomes were compared between the 2 groups. RESULTS: One hundred thirteen infants underwent KPE at median 61 (30-149) days. At a median follow-up of 14.2 (0.9-26.3) years, 35% (39/113) patients were surviving with native liver, 55% (62/113) underwent LT and 11% (12/113) died pretransplant. 12.3% (14/113) patients had normal GGT. Age at KPE and time to COJ were similar between normal and high GGT groups. Normal GGT group had shorter time from KPE to LT (11 vs 18 months, P = 0.02), underwent LT at a younger age (14 vs 20 months, P = 0.04), and had poorer transplant-free survival (P = 0.04) than high GGT group. CONCLUSIONS: 12.3% of infants with BA had normal GGT levels at diagnosis. Low GGT levels at presentation in BA was associated with a poorer outcome.