Vastasyntyneen keltaisuus ei ole aina harmitonta

Vasta­syn­tyneen kel­taisuus on yleistä ensim­mäi­sinä elin­vuo­ro­kau­sina, ja hyper­bi­li­ru­bi­nemiaan liit­tyvän ­aivo­sai­rauden estä­mi­seksi sen hoi­tona käy­tetään sini­valoa. Keltai­suuden pitkit­ty­minen yli kah­den vii­kon, konju­goi­tuneen bili­ru­biinin yli 20 %:n osuus koko­nais­bi­li­ru­bii­nista tai vaa­leat ulosteet voi­vat vii­tata koles­taasiin. Vasta­syn­tyneen koles­taasi edel­lyttää ri­peää diagnos­tiikkaa ja hoi­toa. Sappi­tieat­resia on yleisin ime­väisen koles­taasin aiheut­taja, ja sen hoi­to on mahdol­li­simman varhai­sessa vai­heessa suori­tettu leik­kaus. Suo­messa leikkaus­hoito on keski­tetty HYKS:n Lastenk­li­ni­kalle. Koles­taat­tisen ime­väisen ravit­se­mus­hoito aloi­tetaan viivy­tyk­settä seu­raten myös mak­san toi­mintaa ja luus­ton aineen­vaih­duntaa.Peer reviewe

Serum fasting GLP-1 and GLP-2 associate with intestinal adaptation in pediatric onset intestinal failure

Aim: Glukagon-like-peptide-1 (GLP-1) and -2 (GLP-2), produced by intestinal L-cells, are key hormones regulating intestinal transit, secretion, absorption, and mucosal growth. We evaluated nai ve fasting serum GLP-1 and GLP-2 levels in pediatric intestinal failure (IF). Methods: Fifty-five IF patients with median age 4.2 (IQR 1.3-12) years and 47 matched healthy controls underwent measurement of fasting serum GLP-1 and GLP-2. Results: Serum GLP-2 [19.9 (13.8-27.9) vs 11.6 (7.0-18.6) ng/mL, P <0.001], but not GLP-1 [6.1 (4.0-15.7) vs 6.4 (3.9-10.7) ng/mL, P = 0.976], levels were increased in IF patients. Serum GLP-2 concentrations were higher in patients with small bowel -colic continuity [21.1 (15.0-30.7) ng/mL] compared to patients with an endostomy [10.4 (6.6-17.9) ng/mL, P = 0.028], whereas no association with preservation of ileum or ileocecal valve was observed. During PN delivery, GLP-2 inversely associated with remaining small bowel length (r = -0.500, P = 0.041) and frequency of PN infusions (r = -0.549, P = 0.042). Serum GLP-1 levels were lower in patients receiving PN currently [4.1 (2.8-5.1)] compared to patients, who had weaned off PN [6.5 (5.1-21.1), P = 0.005],. and correlated positively with duration of PN (r = -0.763, P = 0.002) and negatively with percentage parenteral energy requirement (r = -0.716, P = 0.006). Conclusions: In pediatric IF, serum GLP-2 levels increase in patients with small bowel -colic continuity proportionally to the length of resected small intestine. Increase in serum GLP-1 and GLP-2 levels paralleled reducing requirement for parenteral support. These findings support regulation of intestinal adaption by GLP-2 and GLP-1 in children with IF. (C) 2016 Elsevier Ltd and European Society for Clinical Nutrition and Metabolism. All rights reserved.Peer reviewe

Infection Prevention and Management in Pediatric Short Bowel Syndrome

Short bowel syndrome (SBS) is a rare disease with potentially life-threatening consequences. In addition to intestinal failure-associated liver disease, infections and other complications related to central venous catheters (CVCs) cause a significant burden to patients with SBS and may even necessitate an intestinal transplant eventually. The need for long-term central venous access and the intestinal dysfunction associated with SBS drive the need for intestinal failure-specific approach to prevent and treat infections in patients with SBS. In bacterial infections, the line can often be salvaged with proficient antibiotic therapy. Repeated catheter replacements are predisposed to recurrent infections and thrombotic complications, which may limit the long-term survival of patients with SBS. Protocol-based CVC access procedures and daily care including taurolidine and ethanol catheter locks have been shown to reduce infection rates substantially. Compromised intestinal function in SBS predisposes to small bowel bacterial overgrowth, mucosal injury, and increased permeability. These pathophysiological changes are concentrated in a subset of patients with excessive bowel dilatation and frequent bowel-derived infections. In such patients, reconstructive intestinal surgery may be indicated. Probiotics have not been effective in infection prevention in SBS and carry a significant risk of complications. While more studies focusing on the prevention of infections and their complications are needed, protocol-based approach and multidisciplinary teams in the care of patients with SBS have been shown to reduce complications and improve outcomes.Peer reviewe

GLP-2 cures the gut - What about the liver?

Non peer reviewe

Fragmented management of long-term parenteral support for adult intestinal failure in Finland

Objectives: Parenteral support (PS) is the first-line therapy for intestinal failure (IF). Optimal patient outcomes require experienced multidisciplinary teams adhering to structured protocols. As practices to provide long-term PS for adult IF patients in Finland are unknown, this cross-sectional nationwide study aimed to evaluate current management of PS for adult IF across the country.Materials and methods: An internet-based survey was emailed to all Finnish hospitals and hospital-at-home services with the potential to provide PS for adult IF. The survey included 20 items addressing the provision of long-term PS for adult IF patients (aged >= 18years). Data were analysed using descriptive statistics.Results: Overall, 52 (47%) of the 111 identified units responded. Of responding units, 38 (73%) had at some point provided long-term (>= 120days) PS for adult IF, and 23 (44%) had done so during the preceding year. Only three units currently managed >= 3 adult patients. Most (65%) of the respondents worked in a hospital and were either physicians (38%) or dietitians (39%). Only 65% of respondents reported that their unit had an assigned physician responsible for PS provision, and 28% reported that a team was responsible for long-term PS. Only 26% of respondents reported having a written protocol to guide PS management.Conclusions: Health care providers with very limited experience and a fragmented approach manage most Finnish adult IF patients. Evidence-based protocols and multidisciplinary teams are scarce. The care for adult IF patients on long-term PS needs to be improved in Finland.Peer reviewe

Long-term Outcomes and Health Perceptions in Pediatric-onset Portal Hypertension Complicated by Varices

Objectives: Outcomes of pediatric-onset portal hypertension are poorly defined. We aimed to assess population-based long-term outcomes of pediatric-onset portal hypertension complicated by varices. Methods: All children with esophageal varices (n = 126) were identified from 14,144 single nationwide referral center endoscopy reports during 1987 to 2013, and followed up through national health care and death registers. A questionnaire was sent to survivors (n = 94) of whom 65 (69%) responded. Results: Nineteen underlying disorders included biliary atresia (35%), extrahepatic portal vein obstruction (35%), autosomal recessive polycystic kidney disease (7%), and other disorders (23%). During median follow-up of 15.2 (range 0.5-43.1) years patients underwent median 9 (1-74) upper gastrointestinal endoscopies. Esophageal varices were first observed at a median age of 4.0 (0.3-18.2) years, 112 (89%) patients underwent median 6 (1-56) sclerotherapy/banding sessions, and 61 (48%) experienced median 2 (range 1-20) variceal bleeding episodes. Forty-eight surgical shunt procedures were performed to 41 (36%) patients and 38% underwent liver transplantation. Portal hypertensive biliopathy was diagnosed in 4 patients. Hepatopulmonary syndrome necessitated liver transplantation in 2 patients, hepatic encephalopathy in 2, and hepatorenal syndrome in 1. No patient died of variceal bleeding. Patient-reported perception of health on a scale of 1 to 10 was 9 (range 4-10), and 86% reported no current symptoms attributable to esophageal varices. Conclusions: Pediatric-onset portal hypertension is a heterogeneous disease with significant long-term morbidity, requiring multimodal approach with considerable resources and continuation of follow-up in adulthood. Although mortality to variceal bleeding was avoided, bleeding episodes recurred also in adulthood, while patient-reported health of long-term survivors was encouraging.Peer reviewe

Anorektumin epämuodostumien hoito ja myöhäisongelmat

Vertaisarvioitu. English summaryAnorektumin epämuodostumat ovat tavallisimpia suoliston synnynnäisiä epämuodostumia, jotka vaihtelevat peräaukon lievästä virhesijainnista vaikeisiin monianomalioihin. Niiden yleisyys Suomessa on 1/2 500 elävänä syntynyttä, ja 65 %:lla potilaista todetaan liitännäisanomalioita. Lievät anorektumin kehityshäiriöt hoidetaan ensisijaisesti laajentamalla tai yksinkertaisella anoplastialla, mutta vaikeimmissa epämuodostumissa potilaille joudutaan tekemään monimutkaisia rekonstruktioita avannesuojassa. Lievissä tapauksissa suolen toiminnallinen ennuste on erinomainen. Vaikeammissa tapauksissa merkittävälle osalle potilaista jää suolen pysyvä toimintahäiriö, johon voi liittyä virtsateiden ja sukuelinten toiminnallisia ongelmia sekä heikentynyt elämänlaatu. Potilaiden onnistunut hoito edellyttää asiantuntevaa seurantaa ja tukitoimia läpi koko lapsuusiän, ja niiden tulisi jatkua aikuislääketieteen piiriin siirtymisen jälkeenkin.Peer reviewe

Manometric findings in relation to functional outcomes in different types of anorectal malformations

Aims: To compare anorectal manometry (AM) in patients with different types of anorectal malformations (ARMs) in relation to functional outcomes. Methods: A single-institution, cross-sectional study. After ethical approval, all patients >= 7 years old treated for anterior anus (AA), perineal fistula (PF), vestibular fistula (VF), or rectourethral fistula (RUF) from 1983 onwards were invited to answer the Rintala bowel function score (BFS) questionnaire and to attend anorectal manometry (AM). Patients with mild ARMs (AA females and PF males) had been treated with minimally invasive perineal procedures. Females with VF/PF and males with RUF had undergone internal-sphincter saving sagittal repairs. Results: 55 of 132 respondents (42%; median age 12 (7-29) years; 42% male) underwent AM. Patients with mild ARMs displayed good anorectal function after minimally invasive treatments. The median anal resting and squeeze pressures among patients with mild ARMs(60 cm H2O and 116 cm H2O respectively) were significantly higher than among patients with more severe ARMs (50 cm H2O, and 80 cm H2O respectively; p Conclusions: Our findings support the appropriateness of our minimally invasive approaches to the management of mild ARMs, and IAS-saving anatomical repairs for patients with more severe malformations. (C) 2017 Elsevier Inc. All rights reserved.Peer reviewe

Ruokatorviatresia - hoito, komplikaatiot ja pitkäaikaisongelmat

English summaryPeer reviewe