21 research outputs found
Clinical characteristics of 320 pediatric Crohn's disease patients registered in the nationwide Crohn's disease registry in Poland
Wstęp: Nieswoiste choroby zapalne jelit (inflammatory bowel
diseases - IBD), zwłaszcza choroba Leśniowskiego-Crohna
(Crohn’s disease - CD), są narastającym problemem w gastroenterologii pediatrycznej. Dostępne dane dotyczące klinicznych i demograficznych aspektów choroby w Polsce są ograniczone.
Cel: Zebranie rzetelnych danych o klinicznych i demograficznych aspektach choroby Leśniowskiego-Crohna u dzieci w Polsce na podstawie utworzonego internetowo prospektywnego
rejestru choroby mających pomóc w opracowaniu najbardziej
optymalnych strategii terapeutycznych dla tej grupy pacjentów.
Materiał i metody: We wrześniu 2005 roku został utworzony
w Internecie ogólnopolski rejestr pacjentów z chorobą
Leśniowskiego-Crohna. Do projektu włączono 10 jednostek
szpitalnych (9 szpitali akademickich, 1 rejonowy szpital referencyjny). W celu zebrania danych demograficznych i klinicznych zastosowano dostępny internetowo kwestionariusz,
który następnie przesyłano do centralnego rejestru do prospektywnej analizy. Ocenie poddano następujące dane:
demografia, historia rodzinna, lokalizacja i postać choroby,
objawy pozajelitowe, choroby współistniejące, diagnostyka
oraz leczenie (włączając w to interwencje chirurgiczne).
Wyniki: Przez 4 lata 320 pacjentów (płeć męska : płeć żeńska -
191 : 129) w wieku poniżej 16 lat ze zdiagnozowaną CD (średni wiek w momencie postawienia diagnozy: 9,2 ±6,8 roku)
zostało zarejestrowanych w bazie danych. Tak zwany wczesny początek choroby (wiek przy rozpoznaniu poniżej 5 lat)
stwierdzono u 68 dzieci (21,25%). Rodzinne występowanie
(obciążony wywiad rodzinny) odnotowano u 16 pacjentów
(5%). Główne miejsce zmian chorobowych (według Klasyfikacji montrealskiej: L1 - jelito cienkie, L2 - jelito grube, L3 - ileocolon, L4 - górny odcinek przewodu pokarmowego) stanowiła lokalizacja krętniczo-kątnicza (L3) - 217 (67,8,%). Postać
niepenetrująca bez zwężeń była przeważającą postacią choroby - 225 (70,32%) pacjentów. Objawy pozajelitowe zaobserwowano u 20 chorych (6,25%).
Wnioski: Badanie dostarcza pełnych informacji dotyczących
aspektów demograficznych i klinicznych choroby Leśniowskiego-Crohna w Polsce. Uzyskane dane są zgodne z doniesieniami z innych krajów. Wnioski z badania są następujące:
zbierane informacje muszą być dobrze zdefiniowane i określone już na samym początku badania, weryfikowane oraz
aktualizowane systematycznie w trakcie jego trwania, aby
usprawnić pracę i uzyskać jak najbardziej wiarygodne wyniki.Introduction: Inflammatory bowel disease, particularly
Crohn’s disease (CD), is a rising problem in pediatric gastroenterology. Limited information is available on demographic and clinical aspects of pediatric CD in Poland.
Aim: Preliminary data on demographic and clinical characteristic of pediatric CD in Poland based on the web-based
prospective registry in order to gather reliable information to
identify appropriate treatment strategies.
Material and methods: In September 2005 a web-based
prospective registry of CD patients was initiated in Poland.
Ten institutes (9 academic centers, 1 referred regional hospital) took part in the project with the object of obtaining
the demographic and clinical data of pediatric CD patients
across the country. With this end in view, a computerized
questionnaire was used and the collected data were sent prospectively to a central registry for analysis. The following
data were analyzed: demographics, family history, location
and behavior of disease, extraintestinal manifestation, coexisting diseases, diagnostic work-up, and medical treatment
including surgical intervention.
Results: Through the period of 4 years, 320 patients (male :
female - 191 : 129) aged below 16 years with CD diagnosed at
the mean age of 9.2 ±6.8 years were incorporated in the registry. Early onset of disease (age at diagnosis below 5 years)
was recorded in 68 children (21.25%). Positive family history
was reported for 16 patients (5%). The predominant localization of lesions described using the Montreal classification
(L1 for small intestine, L2 for colon, L3 for ileocolon, and L4
for the upper gastrointestinal tract) was ileocolon (L3) - 217
patients (67.8%). The predominant behavior of disease was
non-stricturing and non-penetrating - 225 patients (70.32%).
Extraintestinal manifestation was reported in 20 patients
(6.25%). Coexisting diseases occurred in 35 patients
(10.93%). The predominant initial therapy was mesalazine
(227 patients - 70.1%). Seventeen patients (5.31%) required
a surgical intervention.
Conclusions: This study provides comprehensive information
on demographic and clinical aspects of pediatric CD in
Poland. Our results are consistent with the previously published reports from other countries in terms of age of onset
and male predominance in pediatric CD patients. Our conclusions are as follows: information needs to be well defined,
validated at entry, and updated at every visit, which facilitates our work and makes the data more reliable
Związek pomiędzy surowiczym stężeniem osteokalcyny, otłuszczeniem ciała i zaburzeniami metabolicznymi u dzieci i młodzieży z otyłością
Introduction: Childhood obesity has been associated with the development of insulin resistance, potentially leading to several metabolicdisorders. Osteocalcin has been reported to contribute to the regulation of glucose tolerance and insulin sensitivity.The purpose of this study was to examine the relationship between serum osteocalcin and metabolic risk factors in obese children andadolescents.Material and methods: Age, gender, pubertal stage, adiposity markers (standard deviation score of body mass index: BMI-SDS, percentageof body fat, waist circumference), blood pressure, serum osteocalcin (OC), fasting plasma glucose and insulin, glycated haemoglobin level(HbA1c), insulin resistance estimated by homeostasis model assessment (HOMA-IR), lipid profile, C-reactive protein (CRP), fibrinogen(FB), white blood cell count (WBC) and 25-hydroxyvitamin D (25-OH-D) were evaluated in 142 obese children and adolescents. Stepwisemultiple regression analysis was used to determine the relationship between serum osteocalcin and metabolic risk parameters.Results: Mean serum osteocalcin level was 72.0 ± 20.5 μg/L (range: 16.8–181.5 µg/L). After adjustment for multiple potential confounders,serum osteocalcin concentration was inversely associated with adiposity markers as well as HOMA-IR, HbA1c, triglycerides, CRP, FBand positively with 25-OH-D and HDL-cholesterol. In stepwise multiple linear regression analysis adjusted for age, gender and pubertalstage, osteocalcin was significantly negatively related to HOMA-IR, triglycerides and waist circumference.Conclusions: Serum osteocalcin concentration is associated with blood markers of dysmetabolic phenotype and measures of adiposity,suggesting that osteocalcin is important not only for bones but also for glucose and fat metabolism as early as during childhood.(Endokrynol Pol 2013; 64 (5): 346–352)Wstęp: Otyłość u dzieci wiąże się z rozwojem insulinooporności i ryzykiem wielu zaburzeń metabolicznych. Osteokalcyna odgrywa rolęw kształtowaniu tolerancji glukozy oraz wrażliwości na insulinę. Celem pracy było zbadanie zależności pomiędzy stężeniem osteokalcynyw surowicy a czynnikami ryzyka zaburzeń metabolicznych u dzieci i młodzieży.Materiał i metody: Wśród czynników ryzyka zaburzeń metabolicznych, których związek ze stężeniem osteokalcyny w surowicy analizowanow modelu wielokrotnej regresji krokowej w grupie 142 otyłych dzieci i młodzieży, znalazły się: płeć, stopień dojrzałości płciowej,markery otłuszczenia (odchylenie standardowe wskaźnika masy ciała: BMI-SDS, odsetek tkanki tłuszczowej, obwód talii), ciśnienie tętniczekrwi, stężęnie osteokalcyny w surowicy, stężenie glukozy i insuliny na czczo, zawartość hemoglobiny glikowanej (HbA1c), stopieńoporności na insulinę oceniany na podstawie modelu homeostatycznego (HOMA-IR), profil lipidowy, stężenie białka C-reaktywnego(CRP) i fibrynogenu (FB), liczba krwinek białych oraz stężenie 25-hydroksywitaminy D (25-OH-D).Wyniki: Średnie stężenie osteokalcyny w surowicy wyniósł 72,0 ± 20,5 μg/l (zakres: 16,8–181,5 µg/L). Po uwzględnieniu poprawki napotencjalne czynniki zakłócające, stężenie osteokalcyny okazało się być odwrotnie skorelowane z poziomem markerów otłuszczenia,a także z HOMA-IR, HbA1c, stężeniem triglicerydów, CRP i FB; stwierdzono też dodatnią korelację ze stężeniem 25-OH-D i HDL. W modeluwielokrotnej regresji krokowej, uwzględniającym poprawkę na wiek, płeć oraz stopień dojrzałości płciowej, stężenie osteokalcynyokazało się być odwrotnie skorelowane z HOMA-IR, stężeniem triglicerydów i obwodem talii.Wnioski: Stężenie osteokalcyny w surowicy wykazuje związek z wykładnikami zaburzeń metabolicznych oraz miernikami otłuszczenia;obok udziału w metabolizmie tkanki kostnej, osteokalcyna wydaje się więc odgrywać istotną rolę w przemianach glukozy i lipidówu dzieci. (Endokrynol Pol 2013; 64 (5): 346–352
Demographic characteristics of children with early clinical manifestation of inflammatory bowel disease
Abstract Introduction: Inflammatory bowel disease (IBD), which includes Crohn's disease (CD) and ulcerative colitis (UC), is a chronic condition of the colon and small intestine. The disease is common in young people (children and young adults), but it is rare in children younger than five years of age. Therefore, IBD developing during the first years of life (under the age of 5) is known as an early-onset IBD (EO-IBD), and it is considered to be a specific entity with a distinct phenotype. However, the available data on that issue are still insufficient. Aim: To determine the characteristics and clinical course of children with early-onset IBD. Material and methods: We performed a retrospective database analysis of 47 infants younger than 5 years old diagnosed with IBD. Patient's demographic data, including age, sex, and age at disease onset, were collected in 6 paediatric hospitals in Poland. Disease location was established on the basis of the review of all endoscopic, colonoscopic, histopathological, and radiological records. All possible complications were reported, as well as any treatment and its efficacy. Since the diagnosis was established all patients have been on follow up. Results: Among 47 children registered in the database, 23 (49%) had a diagnosis of CD, 16 (34%) had UC, and 8 (17%) had IC (indeterminate colitis). The mean age at diagnosis was 28.5 ±27.5 months; 57.4% were male. The most common location/type of disease was ileocolonic disease (L3). The most common complication of IBD was anaemia, found in 30 (63.8%) children. The observed course of the disease was either severe or moderate. In 4 children younger than 2 years old, surgery was performed. Conclusions: Inflammatory bowel disease in children younger than 5 years old includes UC, CD, and a relatively high proportion of IC. In early-onset IBD severe and moderate course of the disease is usually observed. Disease manifestation in these patients is predominantly ileocolonic
Evaluation of the infliximab therapy of severe form of pediatric Crohn's disease in Poland : retrospective, multicenter studies
Polish statement on food allergy in children and adolescents
An adverse food reaction is defined as clinical symptoms occurring in children, adolescents or adults after ingestion of a food or chemical food additives. This reaction does not occur in healthy subjects. In certain individuals is
a manifestation of the body hypersensitivity, i.e. qualitatively altered response to the consumed food. The disease
symptoms observed after ingestion of the food can be triggered by two pathogenetic mechanisms; this allows
adverse food reactions to be divided into allergic and non-allergic food hypersensitivity (food intolerance). Food
allergy is defined as an abnormal immune response to ingested food (humoral, cellular or mixed). Non-immunological mechanisms (metabolic, pharmacological, microbiological or other) are responsible for clinical symptoms
after food ingestion which occur in non-allergic hypersensitivity (food intolerance).
Food allergy is considered a serious health problem in modern society. The prevalence of this disorder is varied
and depends, among other factors, on the study population, its age, dietary habits, ethnic differences, and the
degree of economic development of a given country. It is estimated that food allergy occurs most often among
the youngest children (about 6-8% in infancy); the prevalence is lower among adolescents (approximately 3-4%)
and adults (about 1-3%). The most common, age-dependent cause of hypersensitivity, expressed as sensitization or allergic disease (food
allergy), are food allergens (trophoallergens). These are glycoproteins of animal or plant origine contained in: cow's
milk, chicken egg, soybean, cereals, meat and fish, nuts, fruits, vegetables, molluscs, shellfish and other food products. Some of these allergens can cause cross-reactions, occurring as a result of concurrent hypersensitivity to food,
inhaled or contact allergens.
The development of an allergic process is a consequence of adverse health effects on the human body of different
factors: genetic, environmental and supportive. In people predisposed (genetically) to atopy or allergy, the development of food allergy is determined by four allergic-immunological mechanisms, which were classified and described
by Gell-Coombs. It is estimated that in approximately 48-50% of patients, allergic symptoms are caused only by
type I reaction, the IgEmediated (immediate) mechanism. In the remaining patients, symptoms of food hypersensitivity are the result of other pathogenetic mechanisms, non-IgE mediated (delayed, late) or mixed (IgE mediated,
non-IgE mediated). Clinical symptomatology of food allergy varies individually and depends on the type of food induced pathogenetic
mechanism responsible for their occurrence. They relate to the organ or system in which the allergic reaction has
occurred (the effector organ). Most commonly the symptoms involve many systems (gastrointestinal tract, skin,
respiratory system, other organs), and approximately 10% of patients have isolated symptoms. The time of symptoms onset after eating the causative food is varied and determined by the pathogenetic mechanism of the allergic immune reaction (immediate, delayed or late symptoms). In the youngest patients, the main cause of food reactions is allergy to cow’s milk. In developmental age, the clinical picture of food allergy can change, as reflected in the so-called allergic march, which is the result of anatomical and functional maturation of the effector organs, affected by various harmful allergens (ingested, inhaled, contact allergens and allergic cross-reactions). The diagnosis of food allergy is a complex, long-term and time-consuming process, involving analysis of the allergic history (personal and in the family), a thorough evaluation of clinical signs, as well as correctly planned allergic
and immune tests. The underlying cause of diagnostic difficulties in food allergy is the lack of a single universal laboratory test to identify both IgE-mediated and non-IgE mediated as well as mixed pathogenetic mechanisms of
allergic reactions triggered by harmful food allergens. In food allergy diagnostics is only possible to identify an
IgE-mediated allergic process (skin prick tests with food allergens, levels of specific IgE antibodies to food allergens). This allows one to confirm the diagnosis in patients whose symptoms are triggered in this pathogenetic
mechanism (about 50% of patients). The method allowing one to conclude on the presence or absence of food
hypersensitivity and its cause is a food challenge test (open, blinded, placebo-controlled). The occurrence of clinical symptoms after the administration of food allergen confirms the cause of food allergy (positive test) whereas
the time elapsing between the triggering dose ingestion and the occurrence of clinical symptoms indicate the pathogenetic mechanisms of food allergy (immediate, delayed, late). The mainstay of causal treatment is temporary removal of harmful food from the patient’s diet, with the introduction of substitute ingredients with the nutritional value equivalent to the eliminated food. The duration of dietary
treatment should be determined individually, and the measures of the effectiveness of the therapeutic elimination
diet should include the absence or relief of allergic symptoms as well as normal physical and psychomotor development of the treated child. A variant alternative for dietary treatment of food allergy is specific induction of food tolerance by intended contact of the patient with the native or thermally processed harmful allergen (oral immunotherapy). This method has
been used in the treatment of IgE-mediated allergy (to cow's milk protein, egg protein, peanut allergens).
The obtained effect of tolerance is usually temporary. In order to avoid unnecessary prolongation of treatment in a child treated with an elimination diet, it is recommended to perform a food challenge test at least once a year. This test allows one to assess the body's current ability to acquire immune or clinical tolerance. A negative result of the test makes it possible to return to a normal diet,
whereas a positive test is an indication for continued dietary treatment (persistent food allergy). Approximately 80% of children diagnosed with food allergy in infancy "grow out" of the disease before the age of
4-5 years. In children with non-IgE mediated food allergy the acquisition of food tolerance is faster and occurs in
a higher percentage of treated patients compared to children with IgE-mediated food allergy.
Pharmacological treatment is a necessary adjunct to dietary treatment in food allergy. It is used to control the rapidly increasing allergic symptoms (temporarily) or to achieve remission and to prevent relapses (long-term treatment).
Preventive measures (primary prevention of allergies) are recommended for children born in a "high risk" group for
the disease. These are comprehensive measures aimed at preventing sensitization of the body (an appropriate way
of feeding the child, avoiding exposure to some allergens and adverse environmental factors). First of all, the infants
should be breast-fed during the first 4-6 months of life, and solid foods (non milk products, including those containing gluten) should be introduced no earlier than 4 months of age, but no later than 6 months of age. An elimination diet is not recommended for pregnant women (prevention of intrauterine sensitization of the fetus and
unborn child). The merits of introducing an elimination diet in mothers of exclusively breast-fed infants, when the
child responds with allergic symptoms to the specific diet of the mother, are disputable. Secondary prevention focuses on preventing the recurrence of already diagnosed allergic disease; tertiary prevention is the fight against organ
disability resulting from the chronicity and recurrences of an allergic disease process. Food allergy can adversely affect the physical development and the psycho-emotional condition of a sick child, and
significantly interfere with his social contacts with peers. A long-term disease process, recurrence of clinical symptoms, and difficult course of elimination diet therapy are factors that impair the quality of life of a sick child and
his family. The economic costs generated by food allergies affect both the patient's family budget (in the household), and the overall financial resources allocated to health care (at the state level). The adverse socio-economic
effects of food allergy can be reduced by educational activities in the patient’s environment and dissemination of
knowledge about the disease in the society
The natural history of IgE mediated wheat allergy in children with dominant gastrointestinal symptoms
Adherence to Gluten-Free Diet in Children with Celiac Disease
Celiac disease (CD) can only be treated by rigorous life-long gluten-free diet (GFD). The study included 102 mothers and their CD children treated with GFD for at least two years. Frequency and cause of diet failure in children treated at present (54 children) and 10 years ago (48 children) were compared. Dietary adherence was evaluated serologically (tTG), while diet management difficulties were examined by means of a questionnaire. The study shows that one-third of patients fail to follow GFD, more often 10 years ago than now (40% vs. 26%; p < 0.05), mainly children aged 13–18 (54% vs. 40% now; p < 0.05). Younger children (up to 12) are less likely to abandon the diet (27% vs. 8%; p < 0.05). In this age group non-intentional diet failure prevails, while teenagers interrupt their diet intentionally (45% vs. 33%; p = ns (small population of children in this groups)). Currently, the most common causes of teenage diet failure are the absence of symptoms after consuming a small amount of gluten and, even more often, troublesome diet administration. Previously, the absence of peer acceptance prevailed. With this study we found that: 1. In West Pomerania, every fourth CD child does not follow GFD. 2. For years, teenagers have failed to follow GFD due to the absence of symptoms after consuming small amounts of gluten. 3. The incidence of non-intentional failure to follow GFD has significantly decreased over years, which indicates better dietary care
What Do We Know Now about IgE-Mediated Wheat Allergy in Children?
IgE-mediated wheat allergy is a gluten-related disorder. Wheat is one of the five most common food allergens in children. However, the natural history of IgE-mediated wheat allergy has seldom been described in the research literature. This study presents the current state of knowledge about the IgE-mediated wheat allergy in children
An irreducible left scrotal hernia containing a sigmoid colon tumor (adenocarcinoma) – Case report
AbstractINTRODUCTIONIn relation to all inguinal hernias, large irreducible scrotal hernias are quite rare, while such hernias containing colon tumors in the sac have so far been described in fewer than 30 cases.PRESENTATION OF CASEA 61-year-old patient was admitted for a planned surgery because of a large irreducible left-sided scrotal hernia. Intraoperatively, a large tumor of the sigmoid colon was found in the hernial sac. In a histopathological examination it was diagnosed as adenocarcinoma. A palliative operation was performed and he was referred to further systemic and palliative treatment (because of numerous coexisting liver metastases).DISCUSSIONUntil now, only about 30 cases of colon tumor in inguinal hernia sac have been reported.CONCLUSIONIt should be remembered that even the most obvious preoperative diagnosis may be verified intraoperatively
Chronic Milk-Dependent Food Protein-Induced Enterocolitis Syndrome in Children from West Pomerania Region
Characteristics of chronic milk-dependent food protein-induced enterocolitis syndrome (FPIES) in children from the region of Western Pomerania were studied. Prospectively, 55 children were diagnosed at a median of 2.2 months. The open food challenges (OFC), morphologies, milk-specific IgE (sIgE) (FEIA method, CAP system), and skin prick tests (SPTs) were examined. Vomiting and diarrhea escalated gradually but quickly led to growth retardation. Of the infants, 49% had BMI < 10 c, 20% BMI < 3 c; 25% had anemia, and 15% had hypoalbuminemia. During the OFCs we observed acute symptoms that appeared after 2–3 h: vomiting diarrhea and pallor. A total of 42% children required intravenous hydration. Casein hydrolysates or amino acids formulae (20%) were used in treatment. In 25% of children, SPT and milk sIgE were found, in 18%—other food SPTs, and in 14% allergy to other foods. A transition to IgE-dependent milk allergy was seen in 3 children. In the twelfth month of life, 62% of children had tolerance to milk, and in the twenty-fifth month—87%. Conclusions. Chronic milk-dependent FPIES resolves in most children. By the age of 2 children are at risk of multiple food sensitization, and those who have milk sIgE are at risk to transition to IgE-mediated milk allergy. Every OFC needs to be supervised due to possible severe reactions