Enthesitis: an obscured extraintestinal manifestation in pediatric inflammatory bowel disease.

BACKGROUND: Enthesitis is an extra-intestinal manifestation of inflammatory bowel disease (IBD) which often remains underdiagnosed in children. We aimed to evaluate the frequency of enthesitis in pediatric IBD patients using physical examination and ultrasound (US) assessment as the reference standard. METHODS: 31 children, 7 -18 years of age, diagnosed with IBD were recruited for a cross-sectional study. All subjects completed a study questionnaire and underwent both physical and US examination for the presence of the enthesitis. RESULTS: Of 31 subjects (17girls; median age 14(6) years) enrolled, 17 (55%) had ulcerative colitis, 11 (35%) had Crohn`s disease, and 3 (10%) had indeterminate colitis. The median time from IBD diagnosis was 1.2 years. At least one enthesitis (range 1-4) was identified in 14 (45%) patients of whom nine had more than one enthesitis with symmetric involvement in eight. The quadriceps femoris insertion at the superior portion of the patella was the frequently involved site (32%, 9 of 28 sites), followed by patellar ligament insertion at tibial tuberositas. The presence of enthesitis was associated with a higher intensity of the musculoskeletal pain (p=0.018), but physical activity remained unaffected (p=0.056). CONCLUSIONS: Enthesitis is a common underestimated extra-intestinal manifestation of IBD that may impact the musculoskeletal health of children. Future studies with more extensive cohorts are needed to evaluate enthesial involvement both with physical examination and US in order to predict the long-term outcomes of the enthesitis on children with IBD.

Anesthetic Management in Pediatric Patient for Percutaneous Endoscopic Gastrostomy with Mitochondrial Myopathy: Leigh Syndrome.

Leigh syndrome (LS) is a rare disease mainly affecting the central nervous system due to the abnormalities of mitochondrial energy generation and seen in early childhood with progressive loss of movement, mental abilities, seizures, nystagmus, ophthalmoparesis, optic atrophy, ataxia, dystonia, or respiratory failure. Anesthesia and surgery exacerbate the risks of aspiration, wheezing, and breathing difficulties. Tracheal irritability can be stimulated with the efforts of intubation. We report the anesthetic management of a rare case of an 11-year-old boy with a severe form of LS for percutaneous endoscopic gastrostomy insertion. The patient was closely monitored during the procedure and the postoperative period. Carefully chosen anesthetic agents, good pain control, and close monitoring are essential.

Cholestasis and hypercalcemia secondary to panhypopituitarism in a newborn.

Dursun F, Gerenli N, Kirmizibekmez H. Cholestasis and hypercalcemia secondary to panhypopituitarism in a newborn. Turk J Pediatr 2017; 59: 100-103. Cholestatic hepatitis and hypercalcemia are rare features of hypopituitarism in newborns. So diagnosis of hypopituitarism is frequently delayed. The most frequent symptoms of congenital hypopituitarism are hypoglycemia, prolonged jaundince and micropenis. A patient with congenital hypopituitarism associated with cholestasis and hypercalcemia is reported here. Newborns with hypercalcemia and cholestasis should alert pediatricians to the possibility of congenital hypopituitarism and prompt endocrinological investigation.

Hepatoportal sclerosis in childhood: descriptive analysis of 12 patients.

Hepatoportal sclerosis (HPS) is defined as sclerosis of portal areas in the absence of cirrhosis. There is little information about HPS in children in the literature. The aim of this study was to describe the clinical presentation, associated disorders, laboratory characteristics and outcome of children who were diagnosed as HPS. This study included 12 children diagnosed as HPS by the Pathology Department between 2005 and 2011. Data were collected from the gastroenterology clinic charts retrospectively, including demographics, presentation characteristics, laboratory data and recent status of patients. Twelve patients were enrolled (6 girls, 6 boys). The median age of patients was 13.5 yr. Median age at the time of biopsy was 11 yr. Four patients had splenomegaly, 3 had esophageal varices, one had hepatopulmonary syndrome and had been transplanted. Smooth muscle antibody was found positive in 4 patients, without autoimmune hepatitis findings in liver biopsy. One patient had celiac disease and another patient had positive celiac disease serology but pathology findings. Another patient had Turner's syndrome. Mean follow-up time was 39 months (3.3 yr) after biopsy. Hepatoportal sclerosis does not necessarily present with portal hypertension in children.