Etiology, Characteristics, and Outcomes of Neonatal Liver Failure: Lessons Learned Over the Last 3 Decades.

OBJECTIVE: To evaluate trends in etiology and outcomes of neonatal liver failure (NLF) over 30 years retrospectively at a single institution. STUDY DESIGN: Inclusion criteria for this retrospective cohort study were babies presenting at a chronological age of ≤28 days between 1991 and 2020 with prothrombin time ≥20 seconds and biochemical liver injury. Demographics, etiology, laboratory investigations, need for extrahepatic organ support, acute kidney injury, and intervention with liver transplant (LT) were recorded. Survival outcomes were measured as discharge from the hospital alive with native liver or LT. The study period was stratified into 3 10-year blocks. Trends were analyzed for hospital admissions, etiology, and survival outcomes. RESULTS: One hundred twenty-six babies met the NLF criteria. Admissions to the hospital increased from 21 in 1991-2000 to 65 in 2011-2020. An increasing trend in infectious and metabolic causes, while a decreasing trend in indeterminate etiology, was noted. Survival with native liver improved from 23.8% in 1991-2000 to 55.4% in 2011-20 (P = .021), and mortality reduced from 52.4% to 35.4% during the same periods (P = .213). Twenty-three (18.2%) neonates received LT. Post-LT survival outcomes were 100% for gestational alloimmune liver disease, 66.6% in the indeterminate group, and 25% for herpes simplex virus. Specific etiologies (gestational alloimmune liver disease, OR = 0.07 [0-0.77, P = .048]), presence of acute kidney injury (OR = 6.22 [1.45, 29.38, P = .015]) and need for inotropes (OR = 6.22 [1.45, 29.38, P = .028]) influenced mortality in multivariable logistic regression analysis. CONCLUSIONS: In the last 30 years, advances in diagnosis, treatment, and increasing experience with LT have improved survival in NLF.

Association of Helicobacter pylori in Children With Self-Hand Hygiene, Maternal Hand Hygiene, Cooking, and Feeding Practices.

Background and objective Helicobacter pylori infection is widely prevalent, but its route of transmission is not clear. Person-to-person transmission seems plausible, with hand hygiene being one of the many factors that play a role. The objective of this study was to study the effect of the children's and their mother's hand hygiene and feeding practices on the prevalence of H. pylori in children. Methodology This cross-sectional study involved 475 children and their mothers. A questionnaire was administered to mothers to gather information about maternal hygiene practices, specifically handwashing before food handling and after using the toilet. Additionally, both mothers and children underwent assessments for nail length (whether cut or uncut) and the presence or absence of dirt under their nails, if nails were uncut. The association of these parameters with H. pylori seropositivity in children was comprehensively examined. Furthermore, children were divided into two distinct groups: a younger age group (one month to two years and 11 months) and an older age group (three years to 15 years). For one specific parameter - the presence of dirt under mothers' nails (i.e., if nails were uncut) - the association was further analyzed separately within these age groups. The chi-square test was applied to all variables. P < 0.05 was considered significant Results The association of all variables with H. pylori seropositivity in children was tested. Association with H. pylori seropositivity was not present in mothers with uncut nails (P = 0.050315), mothers with uncut nails harboring dirt under their nails within the entire sample of 475 mothers (P = 0.39476), and mothers with uncut nails harboring dirt under their nails in the older age group (three years to 15 years) of children (P = 0.760071). Association with H. pylori seropositivity was present in mothers with dirt under their uncut nails belonging to the younger age group of children (one month to two years and 11 months (P = 0.014127) and mothers who did not wash their hands before food handling (P = 0.003032) and after using the toilet (P = 0.003082). In all 475 children, association with H. pylori seropositivity was significant with dirt under the uncut nails of children (P = 0.015194) and was not significant for children with merely grown nails but not harboring dirt under them (P = 0.355967).  Conclusions Mother-to-child transmission is one of the likely routes of transmission of H. pylori, and poor hand hygiene seems to play a major role in this process.

Pediatric living donor intestine transplant following an atypical complication of COVID-19: A unique case report from India.

Coronavirus disease-19 (COVID-19) infection causing severe gastrointestinal complications is rare. A 9-year-old child after recovering from mild COVID-19 infection developed small bowel gangrene due to superior mesenteric artery thrombosis. He required resection of entire necrotic small bowel along with caecum causing ultra-short bowel syndrome. Reverse transcriptase-polymerase chain reaction (RT-PCR) done on the resected specimen was positive for COVID-19. He was maintained on individualized parenteral nutrition for 3 months. A living donor intestinal transplant was performed using 200 cm of ileum donated by the patient's father. The graft function was satisfactory and was not complicated with thrombosis, infection, reactivation of latent COVID-19 or rejection. He could be weaned off completely from parenteral nutrition by postoperative day 21. The donor had an uneventful recovery. Six month follow-up was satisfactory with the child achieving complete enteral autonomy as well as target goal nutrition. Thrombotic phenomena associated with COVID-19 infection can affect larger vessel-like superior mesenteric artery leading to small bowel gangrene. Intestine transplant could be done safely after 3 months of recovery from COVID-19 without any adverse outcomes. Further studies are required to establish optimal timing and safety of small bowel transplant in this situation.

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.

Low Osmolarity Oral Rehydration Salt Solution (LORS) in Management of Dehydration in Children.

JUSTIFICATION: The IAP last published the guidelines "Comprehensive Management of Diarrhea" in 2006 and a review in 2016. The WHO in 2002 and the Government of India in 2004 recommended low osmolarity rehydration solution (LORS) as the universal rehydration solution for all ages and all forms of dehydration. However, the use of LORS in India continues to be unacceptably low at 51%, although awareness about ORS has increased from a mere 14% in 2005 to 69% in 2015. Availability of different compositions of ORS and brands in market added to the confusion. PROCESS: The Indian Academy of Pediatrics constituted a panel of experts from the fields of pediatrics, pediatric gastroenterology and nutrition to update on management of dehydration in children with particular reference to LORS and issue a current practice guideline. The committee met twice at CIAP HQ to review all published literature on the aspect. Brief presentations were made, followed by discussions. The draft paper was circulated by email. All relevant inputs and suggestions were incorporated to arrive at a consensus on this practice guideline. OBJECTIVES: To summarize latest literature on ORT and empower pediatricians, particularly those practicing in rural areas, on management of dehydration by augmenting LORS use. RECOMMENDATIONS: It was stressed that advantages of LORS far out-weigh its limitations. Increased use of LORS can only be achieved by promoting better awareness among public and health-care providers across all systems of medicine. LORS can also be useful in managing dehydration in non-diarrheal illness. More research is required to modify ORS further to make it safe and effective in neonates, severe acute malnutrition, renal failure, cardiac and other co-morbidities. There is an urgent need to discourage production and marketing all forms of ORS not in conformity with WHO approved LORS, under a slogan "One India, one ORS".

Monosegment Liver Allografts for Liver Transplantation in Infants Weighing Less Than 6 kg: An Initial Indian Experience.

BACKGROUND: Living donor liver transplantation in small infants is a significant challenge. Liver allografts from adults may be large in size. This is accompanied by problems of graft perfusion, dysfunction, and the inability to achieve primary closure of the abdomen. Monosegment grafts are a way to address these issues. METHODS: Two recipients in our cohort weighed less then 6 kg. The prospective left lateral segments from their donors were large for size. Therefore, monosegment 2 liver grafts were harvested. Data regarding the preoperative, intraoperative, and postoperative events in the donor and the recipient were recorded. RESULTS: We were able to achieve significant reduction in the sizes of the grafts harvested. The donors underwent surgery and hospital stay uneventfully. The recipients had normal graft perfusion and no graft dysfunction, and we could achieve primary abdominal closure. One recipient had self-limiting bile leak postoperatively. CONCLUSIONS: Monosegment 2 liver allografts are safe and effective for use in living donor liver transplantation in small infants weighing less than 6 kg.

Initial experience with per-rectal endoscopic myotomy for Hirschsprung's disease: medium and long term outcomes of the first case series of a novel third-space endoscopy procedure.

INTRODUCTION: Hirschsprung's disease (HSCR) is congenital aganglionosis affecting the hindgut and presents with constipation. Surgical pull-through is the current standard treatment but causes morbidity. Per-rectal endoscopic myotomy is a novel third-space endoscopy technique for treating short-segment (SS)-HSCR. METHODS: Retrospective study of SS-HSCR patients diagnosed on history, contrast enema, rectal biopsies, and anorectal manometry, and treated by PREM. The aganglionic segment was mapped before PREM was performed using third-space endoscopy principles. Stool frequency and laxative usage before and after PREM were compared. RESULTS: Nine patients (age 7.5 [±â€Š5.2] years; 7 male) underwent PREM during a 4-year period. Mean aganglionic segment length was 6.3 cm, mean procedure time 96.1 minutes, and mean length of hospital stay 2.5 days. Median follow-up was 17 months (range 9-58 months). Stool frequency was 1/4.4 days before vs. 1/1.2 days after PREM (P = 0.0004). Mean laxative usage was 5.4 units of laxative (UL) before vs. 0.4 UL after PREM (P = 0.0002). No laxatives were used by 6/9 patients after PREM. The single adverse effect seen (anal stenosis) was treated with dilatation. CONCLUSIONS: PREM is a safe and effective minimally invasive procedure to treat SS-HSCR and results in long-term response.

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.

Therapeutic coil embolization of dominant shunt in hepatopulmonary syndrome enhances post-liver transplant respiratory recovery.

Coil embolization of the atypical enlarged pulmonary artery/arteriole with visible shunting may improve hypoxemia in patients with hepatopulmonary syndrome (HPS). When used selectively in cases with large shunts, either pre- or post-liver transplantation (LT), it can aid an early recovery and reduce morbidity. We present a case where a large intrapulmonary shunt was embolized preoperatively to improve hypoxemia associated with HPS and enhance post-operative recovery.

Pancreatitis in Children.

Pancreatic disease in children has a wide clinical spectrum and may present as Acute pancreatitis (AP), Acute recurrent pancreatitis (ARP), Chronic pancreatitis (CP) and Pancreatic disease without pancreatitis. This article highlights the etiopathogenesis and management of pancreatitis in children along with clinical data from five tertiary care hospitals in south India [Chennai (3), Cochin and Pune].

A questionnaire survey of pediatric inflammatory bowel disease in India.

BACKGROUND: Inflammatory bowel disease (IBD) is not uncommon in children and is an important cause of morbidity. Since information on IBD in Indian children is sparse, the study aimed at highlighting the salient features in them. MATERIALS AND METHODS: A questionnaire survey was done among 221 children and adolescents with IBD [ulcerative colitis (UC) 93 (42.1 %); Crohn's disease (CD) 122 (55.2 %); unclassified (IBD-U) 6 (2.7 %)] across seven centers in India. The cut-off age was 18 years and below. RESULTS: The mean age of presentation for UC and CD was 10.2 ± 4.4 and 11.0 ± 4.5 years, respectively, with no gender difference. Diarrhea (69.9 %, p = 0.001) and blood in the stools (90.3 %, p = 0.0001) were common in UC, whereas abdominal pain (73.8 %, p = 0.01), fever (39.3 %, p = 0.0001), anemia (64.7 %, p = 0.001), and growth failure (76.2 %, p = 0.0001) were common in CD. Extraintestinal manifestations (EIM) were a feature in 23.6 % and 36.1 % of UC and CD, respectively. Pancolitis (E3) was predominant in UC (70.9 %) and 88 % required steroids. Ileocolonic CD (L3) was common in 72.9 %; 76.2 % required azathioprine for maintenance. Of the children with UC, 11.8 % had complications like massive hemorrhage and toxic megacolon, while 27 % of CD had fistulae, perianal abscess, stricture, and perforation. Biologicals were used in 0.8 % of severe UC and in 12.2 % of CD. In UC, 4.3 % required surgical intervention. CONCLUSION: Pediatric inflammatory bowel disease (P-IBD) in India shares similarities with adult-onset IBD. Distinctive features were growth failure and more severe forms of the disease necessitating immunomodulators.