Background. The short bowel syndrome (SBS) is a malabsorptionsyndrome usually caused by small bowelresection. The extent of resection or the length ofremaining intestine determines the degree of malabsorptionand the need for parenteral nutrition.Objective. The aim of this study was to analyze thecases of intestinal failure resulting from short bowelsyndrome arising as a result of surgical treatment.Methods. The medical records of children with shortbowel syndrome resulting from surgical treatment requiringparenteral nutrition and remaining under thecare of the Children’s Memorial Health Institute in September2010, were retrospectively analyzedResults. Among 84 children with SBS in 77 cases itresulted from bowel resection. The main reasons forresection were: congenital atresia of the intestine, intestinaltorsion, gastroschisis and necrotizing enterocolitis(NEC). Among the 25 patients studied in 15 casesSBS resulted from NEC treatment and in 8 cases SBSwas caused by gastroschisis. In patients with NEC anaverage age at birth was 31 Hbd, the first resection wasmade on average 29th day of life, at the time of primarysurgery in 15 patients (88%) stoma was formated,the average time from primary operation to stoma closurewas 197 days. Congenital eventration caused SBSin patients born at 35 week, the first resection wasmade on average 16th day of life. In 5 children stoma was performed and continuity of gastrointestinal tractwas reconstituted on average 163 days after surgery.Conclusions. The short bowel syndrome (SBS) is apotential complication of gastrointestinal surgery inchildren with subsequent awaiting for closing of stomaand restoring continuity of the gut for many months.

Currently, more and more centers advocate to postponefirst operation for about 2–3 weeks or even 2–3 monthsand closed the bladder together with iliac osteotomy.It was also confirmed by author’s study of 100 caseswith bladder exstrophy operated with and without osteotomy.Bladder prolaps was observed more oftenin patients underwent primary closure without osteotomy.

Introduction. Infantile haemangiomas are the mostcommon skin and soft tissue lesions in children. Dueto a well known fact of an inevitable spontaneous involution,it usually requires only a regular follow-up. However,at least in 15% of all cases, the lesion interfereswith developing vital functions, causes complications orprogressing deformity and therefore needs treatment;often systemically. Systemic forms of treatment likesteroids, interferon or chemotherapy are all connectedwith a wide spectrum of possible adverse reactions.Aim. Presentation of our own experience in the treatmentof infantile haemangiomas with propranolol.Material and methods. Systemic therapy with propranololin children with infantile haemangiomas wasintroduced at the Department of Surgery and Urologyfor Children and Adolescents, Medical Universityof Gdansk, in March 2009. So far the treatment hasbeen used in 64 patients (57 girls and 7 boys), agedfrom 6 weeks to just over 3 years; mainly to stop theproliferation of extensive, segmental lesions, but alsoin a few cases requiring bulk reduction prior to planedsurgical excision and one patient with a disseminatedhaemangiomatosis with liver involvement. There wasa visible response to therapy in all cases. The treatmentwas discontinued in 21 patients, with only two recurrencesrequiring its restoration. No significant adverse reactions were noted. There was no response to treatmentin only one case – a girl with a vascular tumour(tufted angioma).Conclusions. The presence of an infantile haemangiomain a proliferative phase; especially when complicated,interfering with vital functions or causing deformity;might require a systemic therapy. Recently introducedpropranolol treatment seams to be a very promisingalternative; especially due to a high rate of clinical responsein all phases of haemangioma developmentwithout any visible, significant adverse effects. Earlyintroduction of treatment enables the prevention of anemerging dysfunction or deformity.

Current experience in interventional cardiology and radiology,progress in technology and miniaturization ofequipment, allow successful percutaneous treatmentof newborns, including low weight newborns. Decisionabout time and type of procedures should betaken by the different specialties doctors, involved inthe diagnostic and therapeutic protocol. Interventionaltherapy in newborns with selected, duct-dependentcongenital heart defects and extracardiac malformationscausing cardiac failure due to severe left to rightshunt (for example aneurysm of the vein of Galen, hepaticarterio-venous malformations) is widely recognized.In such defect as critical, duct-dependent arterialvalves stenosis, balloon valvuloplasty is procedure fromchoice in newborns. In, for example, severe innate orpostsurgical arterial narrowings, balloon angioplasty isone of therapeutic option that should be considered.In some complex, congenital heart defects interventionalprocedures, such as for example balloon atrioseptostomy,can be the first stage treatment. In themost complex cases, the decision about interventionaltreatment should be the result of individual analysisof all available datas and local possibilities. Majorityof interventional procedures performed in newbornsare life-saving. Most of them are palliative allowing toimprove patient condition and delay the surgery.

The author discusses the most important aspects of legalresponsibility of medical staff for pregnant womanand her newborn, during labor and after delivery. Thisresponsibility results from patient’s laws in hospital aswell as human rights, civil rights, penal law and corporativelaws.

Sudden Infant Death Syndrome (SIDS) is poorly definedsyndrom of the sudden death of a child between1 month and 1 year old without any known reason.Several disorders were disscused as a cause of SIDS,also some surgically treatable like gastroesophagealreflux and hyperinsulinemic hypoglycemia.

The authors present analysis of clinical data and treatmenteffect in 39 infants with perianal abscess or/andfistula that were treated in the Children’s MemorialHealth Institute between 2000 and 2010. Surgical procedurewas fistulotomy. More than 50% of our patientshad medical history of non-effective conservative treatmentor puncture/drainage of the abscess. In our group69% of patients were cured without complications,12,8% had recurrence and required second surgery. Themean hospitalization was 4,6 days and antibiotics weregiven from 1 to 10 days. Our treatment policy is effective,pointing out the number of previously pretreatedpatients in our group. The time to cure is relatively shortcomparing to that published by authors who advocateconservative treatment policy.

The author describes the most important and commonproblems related to skin and soft tissue infections innewborns and infants. The paper covers the treatmentguidelines based on literature review and own experience.

The paper presents personal experience and recentchanges introduced to surgical proceeding in thebiggest Polish center specialized in the treatment ofepidermolysis bullosa. The majority of operations isperformed on dystrophic form of the disease. Latestobservations prove that treatment results may dependon limitating of operative iatrogenic trauma as wellas of type and number of dressings performed undergeneral anesthesia. It seems plausible that resignationfrom full finger extension lessens operative trauma andleads to improved treatment results.

Cerebral palsy (CP) is a group of disorders of movementand posture, resulting from nonprogressivedisturbances of the fetal or neonatal brain. The prevalenceof CP is about 2/1000 live births. Etiologicalagents involve: prematurity and intrauterine growthrate restriction, birth asphyxia or trauma, congenitalinfections, chromosomal abnormalities, inborn errorsof metabolism and other. Congenital infections, includingcytomegalovirus infection (CMV), are known causeof neurodevelopmental disabilities include CP. Up to7% of all cases of CP may be attributable to congenitalCMV infection. Children with congenital CMV infectiontransmitted during first trimester of gestation, aremore likely to have neurodevelopmental disabilities asa result of malformations of the cerebral cortex. Magneticresonance imaging (MRI) is the most useful inthe diagnostic of the brain abnormalities, which havethe prognostic value.

A review of orthopedic and physiotherapeutic problemsconcerning cerebral palsy patients was presented. Generalguidelines of orthopedic management (indications,goals, methods) are discussed.

Children with cerebral palsy (CP) require a coordinatedand multidisciplinary treatment due to complicationsaccompanying this disease. Most of these patientspresent symptoms associated with impaired motilityof the gastrointestinal tract. The role of the pediatricsurgeon is associated mostly with solving the problemsrelated to swallowing disorders, gastro-esophageal reflux,gastric emptying disorders. The aim of this studywas to analyze indications, methods and results ofsurgical treatment of children with cerebral palsy anddisorders of the gastrointestinal tract on the basis ofreports from the literature and own material. The needof placing gastrostomy, performing fundoplication andimproving gastric emptying was discussed.

Pregnancy, labour and the neonatal period are predictorsof neuro-developmental outcome. Genetic andenvironmental factors also seem to play a crucial rolein this process. Developmental disorders present witha wide spectrum of clinical syndromes, out of whichcerebral palsy (CP) is the most common. CP is a multifactorialdisease, which presents typically with spasticor flaccid palsy. Etiological factors of CP are very complex,but the most frequent intra uterine predictorsare as follows:
-intrauterine asphyxia (cord blood pH < 7.10; BE< -12 mmol/L);
-prematurity – neonates born with low or extremelylow birth weight;nn multiple pregnancies;
-hypoglycemia;
-hypercapnia and hypocapnia.

Aim: We report here the clinical characteristics and frequencyanalysis of subtelomeric aberrations in patientswith combination of mental retardation with dysmorphyand/or congenital anomalies.Material and methods: 149 cases with normal karyotyperesults were screened using the subtelomeric FISHprobes and MLPA kits SALSA P036b and P070.Results: Subtelomeric aberrations were identified in 19cases, what gives the frequency of 12.7%.Conclusions: Presented results confirm the significantrole of subtelomeric rearrangements as a causeof unclassified intellectual disability in patients withdysmorphic features. We believe that our study givesinsight into the etiology and diagnostics of mental retardationby clinical delineation of identified subtelomeric aberrations. Above that, careful clinical examinationmay improve a detection rate of subtelomericrearrangements.

The ketogenic diet has been used to treat patientswith drug-resistant epilepsy since 1920’s. Besides sideeffects and some difficulties in preparing meals, it iseffective, non-pharmacological method of reductionof the seizure frequency and severity by more than50%. Traditionally used ketogenic diet is based on foodproducts of animal origin, so it is rich in saturated fatsand contributes to lipid metabolism disorders.We performed a prospective observational study in15 patients with drug-resistant epilepsy in the Children’sMemorial Health Institute in Warsaw treatedwith ketogenic diet. Contrary to previous studies weused mostly unsaturated fats in the diet to improvefatty acid profile. Our objective was to determine theeffect of high-fat diet on plasma levels of the majorindicators of lipid metabolism. After 1 year of treatment14 patients presented >50% reduction in epilepsysymptoms and 2 patients >90%. All but one (with familial hypercholesterolemia) patient presented lipidmetabolism parameters within normal range. We wereable to confirm the clinical efficacy of ketogenic dietwhich was effective in controlling seizures. Moreover,it did not produce significant adverse effects on plasmalipid levels, except for decreasing lipoprotein HDLcholesterol,which can be attributed to the quality offat used in the diet.

Objectives. Development of blood pressure referenceranges for school-aged children and adolescents.Materials and methods. Oscillometric blood pressuredata from 17 093 randomly selected participants of currenthealth survey ‘Elaboration of the reference rangeof arterial blood pressure for the population of childrenand adolescents in Poland – PL0080 OLAF’ were used.Data were analyzed with SAS 9.2 software. Centilecharts of blood pressure were constructed using theLMS method with LMS Chart Maker Pro 2.42 package.Results. Current and representative for population ofchildren and adolescents centile charts of blood pressureaccording to age and height.Conclusions. Presented blood pressure centile chartsby age, gender and height for children and adolescentsenable early detection of increased blood pressure.

Giant cell hepatitis (GCH – Giant Cell Hepatitis) associatedto autoimmune hemolytic anemia (AHA – AutoimmuneHemolytic Anemia) is a very rare disease ofearly childhood with unknown pathogenesis, probablyrelated to immune dysregulation usually leading tohepatic failure and death. We present a case reportof a boy with giant cell hepatitis associated to autoimmunehemolytic anemia, with no effect of conventionalimmunosuppresive therapy and successful rituximabtreatment. Rituximab is currently regarded as effectivetreatment and should be considered early in patientswho didn’t respond to the first line therapy.

AbstractBackground: Cases of unethical clinical trials and negligencein new drug’s development have led to the implementationof international and local law regulations,which have changed the way of planning, conductingand reporting clinical studies.Aim of the study: The aim of this paper is to present thereview of the international regulations in clinical trialsincluding Declaration of Helsinki, Good Clinical Practice,European Union and US regulationsDiscussion: During past decades many legislative effortshave been made to ensure that clinical trials areethical and their results are reliable source for marketingnew drugs. Declaration of Helsinki, the InternationalConference on Harmonization and the developmentof Good Clinical Practice were the milestones. On thebasis of these regulations, European Union has passedseveral directives, which markedly impacted thelocal requirements regarding clinical studies in the EUcountries. Similarly, IFDA regulations should be alsoimplemented in the clinical trials to register a new drugin the USA.Conclusions: Knowledge about international, EU andUS law regulations is very import_ant for investigatorsconducting global clinical studies in accordance withinternational standards. Initiatives undertaken for theharmonization of international requirements regardingclinical studies have not achieved all planned resultsyet.

Stem cells brought the promise of breakthrought therapiesfor many neurological conditions. This review focuseson clinical data on stem cell therapy in centralnervous system disorders.

In this article the mechanism of growth disturbances inchildren with chronic inflammatory diseases has beenpresented. The role of pro-inflammatory cytokines andcorticosteroids in this process has been explained aswell as the possibility of treatment with human recombinantgrowth hormone.

Changes in the cardiovascular system may occur inthe course of systemic inflammatory connective tissuediseases, whose common feature in the histopathologicalpicture is fibrous tissue necrosis. Changes incardiovascular system occur most frequently in childrenwith juvenile chronic arthritis, systemic lupus erythematosus,the systemic scleroderma, neonatal lupuserythematosus.

Metabolism and activity of vitamin D is tightly connectedwith skin – the one of major sources of vitamin D. Hormonalform of vitamin D – 1,25(OH)2 D is well knownbasic factor of Ca-P homeostasis. Classical mechanisms of1,25(OH)2 D activity in kidney, bone and intestine are wellrecognized. Extra-renal activity of 1,25(OH)2 D is linkedwith cellural growth, differentiation and function.During last few years unique pathway of vitamin D metabolismlocalized only in epidermal keratinocytes wasreported. These cells can synthesize their own 1,25(OH)2D from 7-dehydrocholesterol through 25 OH D form.Epidermal keratinocytes also contain vitamin D receptor(VDR) – target to 1,25(OH)2 D activity. Associationbetween 1,25(OH)2 D extra-renal activity and skin inhealth and disease is documented. Wide spectrum ofactivity of 1,25(OH)2 D and VDR receptor in the skinwas be discussed concerning treatment and prophylaxisskin disorders, especially hyperproliferative skindisorders, like psoriasis.

Drug-induced liver injury (DILI) is a common liver disease,but diagnosis of DILI is not easy. Although manyreports indicate increase of DILI, many of them arenot recognized. The clinical picture of DILI is variable,ranging from transient and very often mild elevation ofliver enzymes to fulminant liver failure or even death.Authors present practical guidelines for diagnosis andmanagement with suggestions when to withdraw ofthe drug suspected of being responsible for drug-inducedliver injury.

The paper reviews current data about epidemiology ofHelicobacter pylori (H. pylori) infection in children andadolescents in Poland and in Europe. Special attentionwas paid to decreasing prevalence of this infectionamong children in Europe, as well as to reasons andconsequences of this phenomenon. The author suggeststhat the current prevalence of H. pylori infection inPolish children may be much lower than previously reported.Two studies performed in Upper Silesia duringthe period 2005–2010 allow to estimate this prevalenceat no more than 15% of children. Improvement in meanincomes and living conditions seem to be the main factorsassociated with lower percentage of children beeinginfected during their first years of life.

Currently, there is a number of scientific studies pointingout the crucial role of natural feeding in strengtheningthe health of a child and a mother in additionto its further beneficial effects on the adult populations’health. Despite the advantages, the health consequencesof natural feeding are still not exploited at thesatisfactory level, i.a. due to routine and often unjustifiedablactation during pharmacotherapy of a mother.In this paper, the authors have performed the shortanalysis of safety of pharmacotherapy during breastfeedingperiod. Strategies of risk reduction for a childand supporting natural feeding during pharmacotherapyof a nursing mother have been introduced.

Probiotics are live microorganisms that, when ingestedin sufficient quantities, confer benefits to the host’shealth. Saccharomyces boulardii (SB) is a yeast usedin Europe since 1950. Since 1982, there have been 338publications on animal and human studies and 53randomized controlled clinical trials that include 8475subjects, investigating the safety and efficacy of SBin pediatric and adult patients with several diseaseindications. SB has proven to have multiple beneficialeffects in children and adults in prevention and treatmentof antibiotic associated diarrhea, diarrhea dueto Clostridium difficile, acute diarrhea, traveler’s diarrhea,tube feeding associated diarrhea, diarrhea in HIVinfected individuals, inflammatory bowel disease, irritablebowel syndrome, giardiasis, as well as benefitsin the treatment for Helicobacter pylori infection. It isa safe probiotic that does not suffer genetic changesleading to antibiotic resistance and is resistant to antibacterials.Its cost is lower than other probiotics ofsimilar proven effects.

Mucus is the most important defensive substance ofthe mucous membranes. In the respiratory system, inthe conjunction with ciliary cleaning mechanism, coughreflex is a prerequisite for the maintenance of drainingnon-infected airways. Mucus creates two layers,sol and gel. A therapeutic effect is aimed by removingexcess mucus from the airways. Drugs, which actthrough direct stimulation of bronchial glands andincrease the amount and liquidity of secretions arecalled mucokinetics. This is working mechanism of expectorantssuch as: essential oils, creosote derivatives,expectorant drugs of plant origin. Mucolytic agents like:n-acetylcysteine, carbocysteine, erdosteine, disrupt disulfidebonds and form a hydrophilic binding. Ambroxoland bromhexin have both effects: mucokinetic andmucolytic, they reduce the amount of mucopolysaccharidesand albumins in the mucus, and they directlystimulate the secretion of mucus-enriched substanceswhich increase the local resistance. Mucolytic drugsare most commonly used drugs in acute and chronicrespiratory diseases presenting with cough and secretiondefaulting.

Bronchiolitis is the most common lower respiratorytract infection in infants and children up to the age of2, mainly caused by respiratory syncytial virus (RSV)1, 2.In 2–3% of cases a course of the disease is severe2. Fewmanagement methods have proven to be effective inrandomised trials. The article presents recommendationsof management of bronchiolitis according to theguidelines of American Academy of Pediatrics and ScottishIntercollegiate Guidelines Network.

Bronchial asthma is an inflammatory disease of therespiratory system. Despite the fact that every fifthchild in Poland suffers from asthma, the diagnosis andmanagement of the disease remain a challenge for pediatricians.This article is a review of the changes inthe approach to diagnosis, monitoring and treatmentof asthma in children, according to a revision of GINAguidelines 2009, which summarize current knowledgeand guidelines in this issue.

Vitamin D is necessary for maintaining appropriate calciumand phosphate homeostasis in the body (classicalfunction) and for ensuring appropriate functioningof many tissues, organs, cells not related to mineraleconomy (nonclassical function). Vitamin D deficiencycauses in adults osteomalacia, increases the risk offractures in osteoporosis, conduces the development ofcardiovascular diseases, diabetes type 1 and 2, multiplesclerosis, Lesniowski‑Crohn disease and cancer, amongthem colon, breast, and prostate cancer.The causes for vitamin D deficiency in healthy populationare: decreased level of cutaneus synthesis as wellas inadequate intake of Vitamin D in food and in supplements.Vitamin D deficiency (level 25(OH) D below20 ng/ml) is common. It occurs in several dozen percentof healthy persons in many regions of the world, independentof race, gender and age. The daily dosage ofVitamin D consumption as set by Food and NutritionBoard in 1997 is insufficient. The biggest obstacle isthat the dosage of 50 μg/day is the highest level infood supplements. Nowadays, it is recommended thatadults require a minimum of 800–1000 j.m./day when the exposition to the sun is inadequate (in Poland fromOctober to April). This dosage should be given to allpersons who avoid being exposed to sunlight and topersons older than 65 years because of decreased skinsynthesis of Vitamin D and proven antifracture andantifalls effects.