Intussusception in celiac disease: Is it a common feature in children?

BACKGROUND AND AIM: There are sporadic reports of occurrence of intussusception in celiac disease (CD) but no systematic study yet. We prospectively studied the prevalence and natural history of intussusception in newly diagnosed CD. METHODS: From January 2010 to October 2013, 150 children, diagnosed to have CD on the basis of positive serology and duodenal biopsy, were recruited in this study. Abdominal ultrasonography was carried out before starting gluten-free diet (GFD) and repeated in those who had intussusception, on day 3 and then weekly till the resolution of intussusception. RESULTS: The median age was 72 (range, 16-204) months, and 79 (53%) were boys. Diarrhea was the presenting symptom in 119 (79%) cases. A total of 45 intussusceptions were detected in 37 (25%) children. Out of 45 intussusceptions, 95% involved small bowel. All but one had asymptomatic intussusception. On GFD, intussusception resolved spontaneously within 7 days in 65%, within 14 days in 84%, and within 28 days in 92% of cases, and none required surgical or hydrostatic reduction. On univariate analysis, younger age, low weight z score, presentation with diarrhea, abdominal distension, rickets, lower serum albumin, more severe villous atrophy, and refeeding syndrome were more common in children with intussusception. On multivariate analysis, abdominal distension and hypoalbuminemia were found to be significantly associated with intussusception. CONCLUSIONS: Intussusception is frequently (25%) seen in children with newly diagnosed CD, generally asymptomatic and resolves spontaneously on GFD. It is often associated with more severe disease. Children with CD and intussusception should not be subjected to surgical/radiological intervention.

Pediatric hepatocellular carcinoma in a developing country: Is the etiology changing?

HCC is the second most common malignant liver tumor of childhood. It typically affects children with a median age of 10-14 yr on background hepatitis B-related liver disease and is often metastatic or locally advanced at diagnosis. Children below the age of five yr typically constitute <10% of all children with HCC. In these children, it occurs on a background of congenital or metabolic liver disease. The records of all children with HCC who presented to our department over a six-yr study period were reviewed. Twelve patients with a median age of 5.9 yr (range 1.6-15.4) were diagnosed to have HCC. All patients underwent liver transplantation, and none were resected. Eleven patients had background congenital or metabolic liver disease. All five of those with hereditary tyrosinemia type 1 who presented to us were found to have HCC. No patient had hepatitis B-related liver (HBV) disease. Eight (66.7%) patients had incidentally discovered HCC on examination of the explant. Incidentally discovered HCC were smaller, well differentiated, and did not show microvascular invasion compared to those diagnosed preoperatively. There was no recurrence with a median follow-up of five months. The patient demographic for pediatric HCC is changing probably as a consequence of successful immunization against HBV. Younger patients with congenital and metabolic liver disease in whom liver transplantation is the ideal treatment are likely to constitute an ever-increasing proportion of patients with pediatric HCC as HBV disease is controlled or eradicated.

Estimation of liver volume in the western Indian population.

BACKGROUND: A number of formulae to estimate standard liver volume (SLV) exist. However, studies have shown that only certain formulae are applicable to a particular patient population, whereas the other formulae have not been accurate in estimating the SLV. Aim of this study was to assess which formula is most accurate in estimating SLV in the western Indian population. METHODS: Data for donors of living donor liver transplantation from September 2014 to July 2015 was analyzed. Liver volumes were measured using computed tomography volumetry (CTV). SLV was calculated using formulae by the currently existing formulae. The mean SLV and CTV, percentage error in the SLV, and the correlation between SLV and CTV were calculated. RESULTS: Fifty-nine healthy subjects underwent donor hepatectomy [28 (47.5 %) males]. The mean age, mean body mass index (BMI), and mean body surface area (BSA) were 31.8 ± 8.8 years, 23.8 ± 3.7 kg/m(2), and 1.6 ± 0.4, respectively. Mean CTV was 1178 ± 246.8 mL. Difference between mean SLV and mean CTV ranged from -133.5 (±189) mL to 632.2 (±190.2) mL. Mean SLV was significantly different from CTV by all the formulae except Urata. Percentage of population whose SLV was within 15 % of the mean CTV ranged from 1.7 % to 67.8 %, with the highest percentage obtained by using Fu-Gui's formula. However, there was wide inter-individual variation on scatter plots between SLV and CTV by both these formulae. CONCLUSION: Currently existing formulae were not accurate in estimating SLV in our population.

Hepatopulmonary Syndrome in children: a comparative study of non-cirrhotic vs. cirrhotic portal hypertension.

BACKGROUND & AIMS: Hepatopulmonary syndrome in children has not been extensively studied particularly in different subsets of portal hypertension. We prospectively studied hepatopulmonary syndrome in children with cirrhosis and extrahepatic portal venous obstruction. Their comparison might shed light on the pathogenesis of hepatopulmonary syndrome. METHODS: Between 1 to 18 years of age, 135 children with portal hypertension (cirrhosis 35, extrahepatic portal venous obstruction 100) were studied. Hepatopulmonary syndrome was diagnosed by the presence intrapulmonary shunt using transthoracic contrast echocardiography and hypoxia/orthodeoxia (in arterial blood gas examination). Comparative analyses were done for both the groups, and also intra- and intergroups with and without hepatopulmonary syndrome. RESULT: Of 135 children, hepatopulmonary syndrome occurred more frequently in cirrhosis (40%) than extrahepatic portal venous obstruction (13%) (P = 0.01). Cirrhotics with hepatopulmonary syndrome were symptomatic (85% vs. 14%, P < 0.001) and had longer disease duration [16(1-120) months vs. 28 (1-168) months, P = 0.01] as compared to cirrhotics without hepatopulmonary syndrome. Prevalence of hepatopulmonary syndrome among cirrhotics did not correlate with the severity of liver disease as assessed by both paediatric end-stage liver disease score and Child-Turcotte-Pugh score. Patients with extrahepatic portal venous obstruction and hepatopulmonary syndrome remain asymptomatic manifesting with clubbing alone (38%). CONCLUSIONS: Hepatopulmonary syndrome occurs more frequently in cirrhosis, a majority being symptomatic with more severity in comparison to extrahepatic portal venous obstruction having no symptoms and less severity. There seems to be a role of liver dysfunction besides portal hypertension as an added factor in the pathogenesis of hepatopulmonary syndrome.

Duodenal morphometry and small bowel permeability in children with portal hypertension.

OBJECTIVE: We prospectively studied children with portal hypertension (PHT) for portal hypertensive duodenopathy (PHTD) and small bowel intestinal permeability (SIP) with the objectives of defining histopathological parameters for PHTD and to find out whether any association existed among structural changes, SIP, and nutritional status. METHOD: SIP was assessed by using lactulose and mannitol sugar probes in 31 children with PHT (cirrhosis n = 15 and extrahepatic portal venous obstruction n = 16) and 15 healthy children as controls. Morphometric assessment from duodenal biopsies was done in children with PHT. SIP and morphometric parameters were correlated with nutritional status and dietary intake. RESULTS: Among children with PHT, 48% had PHTD defined as presence of villous atrophy (villous to crypt ratio < 2.5:1), dilated capillaries (capillary diameter > 16.8 µm, capillary area > 151 µm, capillary perimeter > 56 µm), and thickened muscularis mucosae (>22.2 µm). Lactulose excretion alone was increased in children with PHT as compared with healthy children (median %: 0.03, 0.02, and 0.01 for cirrhosis, extrahepatic portal venous obstruction, and controls, respectively [P < 0.01]) signifying increased paracellular permeability in PHT. Children with PHT had significantly lower z scores for height, weight, and triceps skin-fold thickness (<-2SD), whereas no differences were found in dietary intake between patients and controls. Increased SIP, nutritional compromise, and PHTD in our patients had no correlation. CONCLUSIONS: PHT is often associated with duodenopathy. SIP does occur as a result of increased paracellular permeability. Factors of increased SIP, undernutrition, and PHTD do not have correlation in childhood PHT.

Serial evaluation of children with ALF with advanced MRI, serum proinflammatory cytokines, thiamine, and cognition assessment.

OBJECTIVES: This prospective, sequential study was done to understand changes in cerebral edema (CE) on magnetic resonance imaging and magnetic resonance spectroscopy, liver functions, and neurocognitive testing (NCT) in children with acute liver failure (ALF). METHODS: A total of 11 ALF and 8 healthy controls were evaluated with advanced magnetic resonance (MR) imaging, blood proinflammatory cytokines (PCs), thiamine levels, liver functions, and NCT. Reevaluation was done at 43.5 ±â€Š26.9 days (first follow-up, n = 8) and 157.3 ±â€Š52.3 days (second follow-up, n = 6) after discharge. RESULTS: At diagnosis, patients with ALF had vasogenic and cytotoxic CE, raised brain glutamine (23.2 ±â€Š3.4 vs. 15.3 ±â€Š2.7), and serum PCs (tumor necrosis factor [TNF]-α 40.1 ±â€Š8.9 vs. 7.2 ±â€Š2.7  pg/mL, interleukin [IL]-6 29.2 ±â€Š14.4 vs. 4.7 ±â€Š1.2  pg/mL). The mammillary bodies (MBs) were smaller, and brain choline (1.9 ±â€Š0.36 vs. 2.6 ±â€Š0.6) and blood thiamine (55.2 ±â€Š6.7 vs. 81.8 ±â€Š10.2  nmol/L) were lower than controls. At first follow-up, the brain glutamine and CE recovered. Brain choline and MBs volume showed improvement and thiamine levels normalized. Significant reduction in TNF-α and IL-6 was seen. The patients performed poorly on NCT, which normalized at second follow-up. Liver biochemistry and thiamine levels were normal and TNF-α and IL-6 showed further reduction at second follow-up. CONCLUSIONS: Patients with ALF have CE contributed by raised brain glutamine and PCs. MBs are small because of thiamine deficiency and show recovery in follow-up. CE and brain glutamine recover earlier than normalization of NCT and liver functions. Persistence of raised cytokines up to 6 months after insult suggests possible contribution from liver regeneration.

Low levels of vitamin D in North Indian children with newly diagnosed type 1 diabetes.

BACKGROUND: To find out whether vitamin D levels are lower in children with newly diagnosed type 1 diabetes (T1D) as compared to non-diabetic subjects. METHODS: Plasma levels of vitamin D (25-OHD) were measured by high performance liquid chromatography (HPLC) in 50 children aged between 6 and 12 yr within a week of diagnosis of T1D, and in 50 healthy children. RESULTS: The mean levels of vitamin D were significantly lower in patients as compared to their controls [20.02 +/- 10.63 ng/mL (50.05 +/- 26.57 mmol/L) vs. 26.16 +/- 12.28 ng/mL (65.4 +/- 30.7 mmol/L), p-value 0.009]. Twenty-nine (58%) children in the study group were vitamin D deficient (25-OHD level < 20 ng/mL or < 50 mmol/L) as compared to only 16 (32%) in the control group. Overall, 43 (86%) diabetic and 38 (76%) healthy children were either vitamin D deficient or insufficient. CONCLUSION: These results suggest that vitamin D levels are low at the onset of T1D, and they strongly support the need for further clinical studies to prospectively evaluate the effect of vitamin D supplementation on T1D rates in this patient population.