Nezrele ganglijske stanice kolona kao uzrok megakolona u dojenačkoj dobi: prikaz bolesnika

IImmaturity of ganglion cells is a rare form of dysganglionosis that belongs to the group of dysmorphic types, characterized by a normal or mildly decreased number of ganglion cells, with the cells and their nuclei being considerably smaller in size. The disorder usually manifests clinically early after birth with abdominal distension, vomiting, feeding intolerance, and delayed passing of meconium. Diagnostic evaluation may include radiological studies; however, biopsy of colonic mucosa with histochemical analysis is considered the most reliable method. Manometry is a reliable noninvasive diagnostic study to confi rm motility disorder. Treatment may be medical in mild cases and more commonly surgical due to complications of the disorder. We present a case of immature colonic ganglion cells as a cause of megacolon in infancy. In our case, medicamentous treatment was eff ective and led to normalization of bowel emptying and stool consistency. Considering that follow up rectosigmoidoscopy was refused by the patient’s parents, we were not able to compare the histopathologic fi ndings before and after the medicamentous treatment; however, the favorable clinical course made us assume that the ganglion cells had probably fully matured.Nezrelost ganglijskih stanica je rijedak oblik disganglionoze koji spada u skupinu dismorfi čnih tipova, a obilježen je normalnim ili blago povišenim brojem ganglijskih stanica, pri čemu su stanice i njihove jezgre znatno manje veličine. Klinički se bolest obično očituje rano poslije rođenja uz nadutost trbuha, povraćanje, nepodnošenje hranjenja i odgođen prolazak mekonija. Dijagnostička procjena može obuhvatiti radiološka ispitivanja, međutim, biopsija sluznice kolona uz histokemijsku analizu smatra se najpouzdanijom metodom. Manometrija je pouzdana neinvazivna dijagnostička pretraga za potvrdu poremećaja pokretljivosti. Liječenje može biti medikamentno u blažim slučajevima, ali je češće kirurško zbog komplikacija bolesti. Prikazuje se slučaj gdje su nezrele ganglijske stanice kolona uzrokovale megakolon kod dojenčeta. U našem slučaju se medikamentno liječenje pokazalo učinkovitim i dovelo je do normalizacije pražnjenja crijeva i konzistencije stolice. Kako su bolesnikovi roditelji odbili kontrolnu rektosigmoidoskopiju, nismo mogli usporediti histopatološke nalaze prije i poslije medikamentnog liječenja, ali povoljan klinički tijek ukazuje na to da su ganglijske stanice vjerojatno sazrele

Nutritional considerations in pediatric pancreatitis : a position paper from the NASPHAN Pancreas Committee and ESPHAN Cystic Fibrosis/Pancreas Working Group

Objectives: Wide variations exist in how physicians manage the nutritional aspects of children affected by acute pancreatitis (AP), acute recurrent pancreatitis (ARP), and chronic (CP) pancreatitis. Better consensus for optimal management is needed. Methods: This consensus statement on nutrition in pediatric pancreatic diseases was developed through a joint ESPGHAN-NASPGHAN working group that performed an evidence-based search of the literature on nutrition in AP, ARP, and CP with a focus on pediatrics. The literature was summarized, quality of evidence reviewed, and expert recommendations developed. The authorship met to discuss the evidence and statements. Voting on recommendations occurred over 2 rounds based on feedback. A consensus of at least 75% was required to approve a recommendation. Areas requiring further research were identified. Results and Discussion: The literature on nutrition in pediatric pancreatitis is limited. Children with mild AP benefit from starting an early nutritional regimen in the course of the attack. Early nutrition should be attempted in severe AP when possible; enteral nutrition is preferred over parenteral nutrition. Children with ARP are likely to tolerate and benefit from a regular diet. Children with CP need ongoing assessment for growth and nutritional deficiencies, exocrine and endocrine insufficiencies. Conclusions: This document presents the first authoritative recommendations on nutritional considerations in pediatric pancreatitis. Future research should address the gaps in knowledge particularly relating to optimal nutrition for AP in children, role of diet or dietary supplements on recurrent attacks of pancreatitis and pain episodes, monitoring practices to detect early growth and nutritional deficiencies in CP and identifying risk factors that predispose children to these deficiencies