Clinical spectrum & changing presentation of celiac disease in Indian children.

Background & objectives: Celiac disease (CD) is a genetic immune mediated disorder characterised by gluten intolerance. This single centre study, from north India was aimed to assess the clinical, serological and histological profile of CD in a large cohort of children and the changing trends in its presentation. Methods: A review of clinical details of CD children diagnosed between 2000 and 2019 and currently on follow up was performed. Information on demography, symptoms, associated conditions, serology, biopsy findings and gluten-free diet were analyzed. Results: The mean age (±standard deviation) of 891 children included in the study, at onset and at diagnosis was 4.0±2.7 and 6.2±3.1 yr, respectively. Growth faltering, abdominal pain, abdominal distension and diarrhoea were presenting symptoms in 70, 64.2, 61.2 and 58.2 per cent, respectively. A positive family history of CD was present in 14 per cent and autoimmune conditions in 12.3 per cent of children. Thyroid disorders were seen in 8.5 per cent of children and Type 1 diabetes mellitus (T1DM) in 5.7 per cent. The duration of breastfeeding had a weak positive correlation with age at onset and diagnosis of CD (P<0.001). Non-classical CD was significantly more common in children aged >10 yr and in those presenting after 2010 (P<0.01). T1DM and hypothyroidism occurred more frequently in non-compliant children. Interpretation & conclusions: This was the largest single centre study, pertaining to the presentation and follow up of CD in children. Infants and young children were more likely to present with classical symptoms of diarrhoea, abdominal distension and growth failure while older children presented with non-classical CD. There was a trend towards non-classical forms of CD in recent years.

Hepatitis A-induced acute liver failure with glucose 6 phosphate dehydrogenase deficiency induced hemolysis and renal failure.

Hepatitis A is the most prevalent viral hepatitis in India and rarely can lead to life-threatening complications such as acute liver failure (ALF). Glucose 6 phosphate dehydrogenase (G6PD) deficiency is the most common enzyme deficiency in the world, and in the setting of acute viral hepatitis, it can cause massive intravascular hemolysis, resulting in acute kidney injury. Here, we report a case of a 12-year-old male child who had hepatitis A-associated ALF, which was complicated by massive hemolysis due to underlying G6PD deficiency, manifesting as acute renal failure requiring renal replacement therapy with other supportive management. He had a prolonged, protracted stormy clinical course, which was further complicated by dialysis disequilibrium syndrome, posterior reversible encephalopathy syndrome, and nosocomial sepsis, which improved over 4 weeks. Our case highlights the importance of having high index of clinical suspicion for G6PD deficiency in a child with acute viral hepatitis with complications.

Pancreatic ascites and Pleural Effusion in Children: Clinical Profile, Management and Outcomes.

BACKGROUND: Pancreatic ascites (PA) and pleural effusion (PPE) are rarely encountered in children. They develop due to disruption of the pancreatic duct (PD) or leakage from an associated pancreatic fluid collection (PFC). The literature on childhood PA/PPE and its management is scarce. METHODS: A retrospective review of children with PA/PPE diagnosed and managed at our center over the last 4 years was performed. The clinical, biochemical, radiological and management profiles were analyzed. Conservative management included nil per oral, octreotide and drainage using either percutaneous catheter or repeated paracentesis. Endotherapy included endoscopic retrograde cholangiopancreatography (ERCP) and transpapillary stenting. RESULTS: Of the 214 children with pancreatitis, 15 (7%) had PA/PPE. Median age was 9 years with a third under 2 years. Median ascitic fluid amylase was 8840 U/L and all had elevated protein (>2.5 g/dl) and low serum ascites-albumin gradient ascites (<1.1). While PA/PPE was the first manifestation of underlying chronic pancreatitis (CP) in 10 children (67%), trauma was seen in 4 (26%) and hypertriglyceridemia in 1 (7%). On imaging, PD disruption could be identified in 10 (67%) children. ERCP and stenting was done in 10 children. Conservative management alone (n = 4) and endotherapy (n = 10) was successful in 93% with only one requiring surgery. The younger children (n = 4), were managed conservatively and only 1 of them required surgery. Resolution of PA/PPE was achieved in all with no recurrences. CONCLUSIONS: Conservative management and ERCP plus transpapillary stenting results in resolution of majority of pediatric PA/PPE. Children presenting with PA/PPE needs to be evaluated for CP.

Advances in management of end stage liver disease in children.

End stage liver disease (ESLD) is an irreversible condition that is a management challenge to the paediatrician. The aetiology and natural history of ESLD in children is not only distinct from adults but also variable depending upon the age of presentation. Children are especially vulnerable to developmental delay, frailty and malnutrition. Nutritional support is the cornerstone of management of these children as it has a significant impact on the clinical course and survival, both before and after transplantation. Further, the complications of ESLD in children including but not limited to, ascites, portal hypertension, spontaneous bacterial peritonitis and encephalopathy raise unique management challenges. In this review we provide a concise review of and highlight recent advances in the management of paediatric ESLD.

Challenges in handling a civilian mass pediatric disaster during flood relief in a partially inundated armed forces medical facility.

BACKGROUND: Armed forces hospitals are often called upon to provide medical aid to civilians during natural calamities. Though children are often the most vulnerable segment of population in these events, research that addresses their unique needs and the role of armed forces hospitals remains sparse. OBJECTIVES: We examined pediatric morbidity and mortality at a flooded armed forces hospital. Factors that affected outcomes were identified. METHODS: 158 patients were evacuated en masse from a children's hospital in northern India that was submerged by flood to an adjacent partially inundated armed forces hospital specializing in military medicine and adult trauma. The children were provided case-based clinical care as per existing disaster management protocol. Geoclimatic vulnerability factors, morbidity/mortality, and medical and logistical challenges for future intervention were investigated. RESULTS: One pediatrician who provided initial triage was joined by two others after 48 hours. A limited load of adult patients permitted more resources for the children, majority (49 percent) of whom were neonates. Intensive care was necessitated for 32 (20.2 percent) cases, with half managed in adult ICU. Overall in-hospital mortality was 5.7 percent. Experienced staff, cross-specialty multitasking, and innovative and noncensorious leadership were identified as assets amidst resources compromised by flooding. Clear delineation of primary caregiver role of pediatrician at outset, pediatric emergency care training, pediatric triage, resource allocation for thermoregulation, oxygen therapy and ventilation, earmarking centers for transfer of cases, and safe transportation to the centers were identified as areas meriting further attention. CONCLUSION: Armed forces hospitals in vulnerable geoclimatic zones must address pediatric concerns in disaster management plans.

Challenges in handling a civilian mass pediatric disaster during flood relief in a partially inundated armed forces medical facility.

BACKGROUND: Armed forces hospitals are often called upon to provide medical aid to civilians during natural calamities. Though children are often the most vulnerable segment of population in these events, research that addresses their unique needs and the role of armed forces hospitals remains sparse. OBJECTIVES: We examined pediatric morbidity and mortality at a flooded armed forces hospital. Factors that affected outcomes were identified. METHODS: 158 patients were evacuated en masse from a children's hospital in northern India that was submerged by flood to an adjacent partially inundated armed forces hospital specializing in military medicine and adult trauma. The children were provided case-based clinical care as per existing disaster management protocol. Geoclimatic vulnerability factors, morbidity/mortality, and medical and logistical challenges for future intervention were investigated. RESULTS: One pediatrician who provided initial triage was joined by two others after 48 hours. A limited load of adult patients permitted more resources for the children, majority (49 percent) of whom were neonates. Intensive care was necessitated for 32 (20.2 percent) cases, with half managed in adult ICU. Overall in-hospital mortality was 5.7 percent. Experienced staff, cross-specialty multitasking, and innovative and noncensorious leadership were identified as assets amidst resources compromised by flooding. Clear delineation of primary caregiver role of pediatrician at outset, pediatric emergency care training, pediatric triage, resource allocation for thermoregulation, oxygen therapy and ventilation, earmarking centers for transfer of cases, and safe transportation to the centers were identified as areas meriting further attention. CONCLUSION: Armed forces hospitals in vulnerable geoclimatic zones must address pediatric concerns in disaster management plans.

Pulmonary thromboembolism: A rare complication of amoebic liver abscess in a child.

Amoebic liver abscess is common in children in developing countries due to lack of hygiene and sanitary conditions. Inferior vena cava thrombosis is a rare complication of this disease, with only a few cases reported in the literature, where this thrombus led to pulmonary thromboembolism. We report the case of a 7-year-old child with amoebic liver abscess who developed pulmonary thromboembolism and was promptly diagnosed and managed.

Clinical Characteristics of Children With Celiac Disease Not Responding to Hepatitis B Vaccination in India.

The immunological response to hepatitis B virus (HBV) vaccine may be suboptimal in children with celiac disease (CD), but the reasons for this are not well defined. Objectives: This study was undertaken to assess the immune response to HBV vaccine in CD children and to explore the possible factors affecting the immune response. Methods: The study population consisted of 3 groups-50 newly diagnosed CD children (group 1), 50 previously diagnosed CD children who were on gluten free diet (GFD) >3 months (group 2), and 100 age and gender matched healthy controls (group 3). The patient characteristics were recorded, and the blood samples were analyzed for HBsAg and anti-HBs titers. The nonresponders were given a booster dose of HBV vaccine and reevaluated after 6 weeks. Results: Positive anti-HBs response was found in 46% in newly diagnosed CD children, 60% in CD children on GFD, and 83% in healthy controls (P < 0.001). The immune response to HBV vaccine in CD children was inferior to that in healthy children (53% vs 83%, P < 0.001). The immune response was found to be significantly affected by age at diagnosis, delay in diagnosis, type of presentation, and compliance to GFD. 44 out of 45 (97.77%) nonresponders from CD group seroconverted after a single booster dose. Conclusion: Early diagnosis and good compliance to GFD may improve the immune response to HBV vaccine in CD children. Single additional booster dose is sufficient to attain optimal immune response.

Liver Abscess in Children-experience From a Single Tertiary Care Center of North India: Etiology, Clinical Profile and Predictors of Complications.

BACKGROUND: Liver abscess (LA), a common problem in children in the tropics, is believed to be mostly pyogenic (PLA), sometimes amebic (ALA). We aimed to analyze the clinical profile, etiology, risk factors for complications, management and outcomes of LA in children. METHODS: The details of 81 children with LA managed in a tertiary set up over a period of 3 years were analyzed. A comparison of different parameters was performed with respect to etiology and complications. RESULTS: ALA, PLA and mixed infection LA were diagnosed in 40 (49.4%), 32 (39.5%) and 9 (11.1%) children. The triad of fever, hepatomegaly and right upper quadrant tenderness was seen in 65 (80.2%). Coagulopathy was observed in 60 (77%) and jaundice in 12 (14.8%). Majority (71.6%) had a single LA in the right lobe (69%). Conservative, percutaneous needle aspiration, percutaneous catheter drainage and surgical drainage were done in 11.1%, 3.7%, 82.7% and 2.5%, respectively. Forty-three (53.1%) had complicated LA with rupture in 55.8% and vascular thrombosis in 16.2%. Children with complicated LA had higher alanine transaminase, prolonged prothrombin time/international normalized ratio, low serum protein and albumin levels (P < 0.05). Median duration of follow-up was 2 months and mean time to resolution of LA was 48.5 ± 18 days. CONCLUSIONS: ALA is the commonest cause of pediatric LA in endemic regions and is difficult to differentiate from PLA clinically. Percutaneous catheter drainage is safe and effective modality for the management of LA in children. A higher alanine transaminase, prolonged prothrombin time/international normalized ratio and low serum albumin levels (<3 g/dL) at presentation identify complicated LA.

Life-threatening Upper Gastrointestinal Bleeding Due to Ruptured Gastroduodenal Artery Aneurysm in a Child.

Gastroduodenal artery (GDA) aneurysm is a rare cause of massive life-threatening upper gastrointestinal (UGI) bleeding in children. Prompt resuscitation with fluids, administration of large amount of blood products (massive transfusion), prompt diagnostic evaluation using computed tomography (CT) angiography or digital subtraction angiography (DSA), and therapeutic endovascular or catheter-based interventions are life-saving. In cases with failed endovascular interventions, open surgical approach to ligate aneurysm is required. We report a 10-year-male with life-threatening UGI bleed due to ruptured GDA aneurysm possibly secondary to sepsis requiring resuscitation, massive transfusion, CT angiography and DSA, endovascular intervention, and ultimately surgical management with good outcome.

Distal and proximal resource influences on economic dependency among the oldest old.

BACKGROUND: As exceptional survivors, centenarians may have characteristics that reduce their dependency on family and community support systems despite the expectation that their extreme age creates a burden on those systems. The Georgia Centenarian Study obtained information about assistance for income, medical care, and caregiving of all types for a sample of centenarians and octogenarians. Previous studies have not established which characteristics may contribute to economic dependency among the oldest old. OBJECTIVE: To identify distal and proximal resource influences on economic dependency, considering past lifestyle, proximal health, economic resources, personality, and coping behavior. METHODS: Analysis sample sizes ranged from 109 to 138 octogenarians and centenarians. Blockwise multiple regressions predicted whether they received income assistance, number of medical care events, number of caregiving types, and total caregiving hours. RESULTS: Past life style, gender, ethnicity, socioeconomic status, functional health, and coping were not related to economic dependency. With the exception of the number of types of care, centenarians were not more dependent than octogenarians. Cognitive ability had the strongest effects for medical care and caregiving services. 'Extraversion', 'ideas', 'neuroticism', and 'competence' personality factors had significant effects for caregiving types and total hours of care received. CONCLUSION: Monitoring and intervention to maintain cognitive ability are critical practices for autonomy and reduced economic dependency among the oldest old. Psychological resources are more important influences on social support than functional health and other proximal economic resources.

Bilateral renal cortical necrosis in a child with acute pancreatitis.

Bilateral renal cortical necrosis (RCN) as a cause of acute kidney injury is very rare in the pediatric population. Progression to end-stage renal disease is seen virtually in every patient with RCN. There are many causes for the occurrence of cortical necrosis in children, with severe pancreatitis being a rarity. In this report, we describe a child with severe acute pancreatitis complicated by bilateral RCN.

Coinfection with Hymenolepis nana and Hymenolepis diminuta infection in a child from North India: A rare case report.

Hymenolepiasis is considered the most common tapeworm infection throughout the world infecting 50-75 million people. Hymenolepis diminuta infection is not commonly reported in human beings as compared to Hymenolepis nana because it is primarily a parasite of rats and mice. There are few case reports of H. diminuta in the Indian population. To the best of our knowledge, not a single case of coinfection with H. nana and H. diminuta has been reported from India. We present here a rare case report of coinfection of H. nana and H. diminuta in a 4-year-old male child from a semirural area of India who presented with acute and severe colitis.

Assessment of quality of life before and after successful percutaneous transvenous mitral commissurotomy in patients with severe mitral stenosis.

BACKGROUND: Rheumatic mitral stenosis (MS) is a significant cause of morbidity and mortality in India. Percutaneous transvenous mitral commissurotomy (PTMC) has become the procedure of choice for severe MS with pliable leaflets. Despite a wealth of literature on the technical aspects of PTMC, there is a dearth of literature addressing the impact of PTMC on the quality of life (QOL). AIM: The aim of the study is to assess the impact of PTMC on clinical status and QOL of patients with severe MS. MATERIALS AND METHODS: Twenty-five consecutive patients with severe MS undergoing PTMC were included in the study with their informed consent. All patients were subjected to routine blood tests, electrocardiogram, chest X-ray, two-dimensional and color echocardiography, treadmill test (TMT), and World Health Organization (WHO) QOL scale. The echocardiography, TMT, and WHOQOL scale were repeated after the procedure. RESULTS: The procedural success was 98%. The mean (range) mitral valve area preprocedure was 0.82 (0.59-0.92) cm2 and postprocedure was 1.61 (1.51-1.76) cm2. The difference was statistically significant (t = 5.02; P < 0.01). The mean (range) of TMT preprocedure was 4.05 (3.0-7.0) METS and postprocedure was 8.52 (6-12) METS. The difference was statistically significant (T = 3.08; P < 0.01). The mean (range) of QOL assessment pre- and post-procedure on physical domain was 8.83 (8.3-10.1) which increased to 11.11 (10-12.7); on social relationship domain from 9.17 (7.5-12.4) to 11.37 (9.4-12.0); on personal relationship from 11.6 (11-13) to 12.52 (12-13); on environment domain from 10.78 (10.2-11.7) to 11.56 (10.8-12); and on level of independence from 9.02 (8-10) to 12.29 (11.0-13.6). All the differences were statistically significant (Wilcoxon signed-rank test z = -4.376; -4.379; -4.234; -4.200; -4.375; respectively, all P < 0.001 highly significant). CONCLUSIONS: PTMC resulted in a significant improvement in the QOL of patients with severe MS. The significant improvement in QOL post-PTMC may be an indication for offering PTMC at an earlier stage to those patients whose QOL is severely compromised.

R542X mutation in SMPD1 gene: genetically novel mutation with phenotypic features intermediate between type A and type B Niemann-Pick disease.

Niemann-Pick disease (NPD) is a heterogenous group of progressive neurovisceral disorder characterised by lysosomal accumulation of sphingomyelin. NPD types A and B are caused by mutations involving sphingomyelin-phosphodiesterase-1 (SMPD1) gene and are characterised by deficiency of acid sphingomyelinase activity. We present a case of a 9-month infant with clinical manifestations intermediate between types A and B NPD and genetically illustrating a novel R542X mutation in the exon 6 of SMPD1.