The presence of serum tissue transglutaminase and endomysial autoantibodies is predictive of small-bowel abnormalities indicative of celiac disease. There is a good correlation between autoantibody positivity and specific HLA haplotypes. We estimate that the prevalence of celiac disease among Finnish schoolchildren is at least 1 case in 99 children.

©М.О. Гончарь СТАН ФУНКЦІОНАЛЬНОЇ АДАПТАЦІЇ СЕРЦЕВО-СУДИННОЇ СИСТЕМИ У ДІТЕЙ ПІСЛЯ ХІРУРГІЧНОЇ КОРЕКЦІЇ ВРОДЖЕНИХ ВАД СЕРЦЯ Харківський національний медичний університет СТАН ФУНКЦІОНАЛЬНОЇ АДАПТАЦІЇ СЕРЦЕВО-СУДИННОЇ СИСТЕМИ У ДІТЕЙ ПІСЛЯ ХІРУРГІЧНОЇ КОРЕКЦІЇ ВРОДЖЕНИХ ВАД СЕРЦЯ. В результаті обстеження 148 школярів у 73,7% пацієнтів, які були прооперовані з приводу вроджених вад серця, у віддалені терміни спостереження виявлено зниження рівня фізичноїпрацездатності за даними велоергометрі!. У 22,1% дітей встановлено добру толерантність до фізичного навантаження, у 4,2%-задовільну, у 70,7%-знижену, низьку-у 3,0% хворих. Незадовільну толерантність до фізичного навантаження мають діти з комбінованими вадами серця, що мали ускладнення до оперативної корекції. СОСТОЯНИЕ ФУНКЦИОНАЛЬНОЙ АДАПТАЦИИ СЕРДЕЧНО-СОСУДИСТОЙ СИСТЕМЫ У ДЕТЕЙ ПОСЛЕ ХИРУРГИЧЕСКОЙ КОРРЕКЦИИ ВРОЖДЕННЫХ ПОРОКОВ СЕРДЦА. В результате обследования 148 пациентов у 73,7% детей, прооперированных по поводу врожденных пороков сердца, в отдаленные сроки наблюдения по данным велоэргометрии выявлено снижение уровня физической работоспособности. У 22,1% детей выявлена хорошая толерантность к физической нагрузке, у 4,2%-удовлетворительная, у 70,7 %-сниженная, низкая-у 3,0% больных. Неудовлетворительную толерантность кфизической нагрузке имеют дети с комбинированными пороками сердца, имевшие осложнения при оперативной коррекции. CONDITION OF CARDIOVASCULAR FUNCTIONAL ADAPTATION IN CHILDREN AFTER SURGICAL TREATMENT OF CONGENITAL HEART DISEASE. 148 patients after surgical treatment of congenital heart disease have been observed. 73,7% children had impaired tolerance to physical exertion during bicycle ergometry, among them 70,7% patients with decreased and 3,0% with low level. 22,1% children have shown good and 4,2%-satisfactory exercise tolerance. Poor tolerance to physical exertion was typical in children with combined congenital heart disease who had complications during surgery. Ключові слова: вроджені вади серця, міокардиальна дисфункція, велоергометрія. Ключевые слова: врожденные пороки сердца, миокардиальная дисфункция, велоэргометрия.

Background-This study was designed to assess the epidemiology, characteristics, and outcome of cardiac sarcoidosis (CS) in Finland. Methods and Results-We identified in retrospect all adult (>18 years of age) patients diagnosed with histologically confirmed CS in Finland between 1988 and 2012. A total of 110 patients (71 women) 51±9 years of age (mean±SD) were found and followed up for outcome events to the end of 2013. The annual detection rate of CS increased >20-fold during the 25-year period, reaching 0.31 in 1×10 5 adults between 2008 and 2012. The 2012 prevalence of CS was 2.2 in 1×10 5 . Nearly two thirds of patients had clinically isolated CS. Altogether, 102 of the 110 patients received immunosuppressive therapy, and 56 received an intracardiac defibrillator. Left ventricular function was impaired (ejection fraction <50%) in 65 patients (59%) at diagnosis and showed no overall change over 12 months of steroid therapy. During follow-up (median, 6.6 years), 10 patients died of a cardiac cause, 11 patients underwent transplantation, and another 11 patients suffered an aborted sudden cardiac death. The Kaplan-Meier estimates for 1-, 5-, and 10-year transplantation-free cardiac survival were 97%, 90%, and 83%, respectively. Heart failure at presentation predicted poor outcome (log-rank P=0.0001) with a 10-year transplantation-free cardiac survival of only 53%. Conclusions-The detection rate of CS has increased markedly in Finland over the last 25 years. With current therapy, the prognosis of CS appears better than generally considered, but patients presenting with heart failure still have poor longterm outcome. (Circulation. 2015;131:624-632.

The findings can be utilized in developing supportive activities for families with disabled children. By recognizing the coping strategies used by the family, professionals and service providers can find the right ways to support their adaptation. As the role of physicians, nursing staff and other professionals in this process is very important, more attention should be attached to the collaboration between these groups, to enable them to view the situation from the perspective of the whole family.

Aim: To study the macronutrient and energy contents of human milk fractions during the first 6 mo of lactation. Study design: A total of 483 milk samples, including 52 pairs of fore‐ and hindmilk samples from 20 mothers, 253 samples from 53 donor mothers and 126 samples from 36 mothers of preterm infants, were collected longitudinally, starting at 1 wk postpartum and continuing monthly up to 6 mo. Protein, lactose and fat contents were measured and energy density estimated. Results: The protein content was significantly lower in fore‐ and hindmilk than in donor or preterm milk during the first months of lactation. In donor and preterm milk, the protein content declined consistently from 2.0 g/100 ml at 1 wk to half of that at 6 mo, and a similar trend was observed in fore‐ and hindmilk. Lactose content showed no significant changes between the groups or in the course of lactation. The fat content was highest in hindmilk, being approximately two‐ to threefold that of foremilk. Accordingly, hindmilk included 25–35 kcal/100 ml more energy on average than foremilk. Conclusions: The fat content of human milk increases in relation to breast emptying, while the other macronutritients of milk show only slight changes. When enteral feeding with high‐energy human milk is preferred, as in the case of very preterm infants, hind milk, with its higher fat content, would be a natural choice.

As a source of transforming growth factor beta1 (TGF-beta1), mast cells have been implicated as potential effector cells in many pathological processes. However, the mechanisms by which mast cells express, secrete, and activate TGF-beta1 have remained vague. We show here by means of RT-PCR, immunoblotting, and immunocytochemistry that isolated rat peritoneal mast cells synthesize and store large latent TGF-beta1 in their chymase 1-containing secretory granules. Mast cell stimulation and degranulation results in rapid secretion of the latent TGF-beta1, which is converted by chymase 1 into an active form recognized by the type II TGF-beta serine/threonine kinase receptor (TbetaRII). Thus, mast cells secrete active TGF-beta1 by a unique secretory mechanism in which latent TGF-beta1 and the activating enzyme chymase 1 are coreleased. The activation of latent TGF-beta1 specifically by chymase was verified using recombinant human latent TGF-beta1 and recombinant human chymase. In isolated TbetaRI- and TbetaRII-expressing peritoneal macrophages, the activated TGF-beta1 induces the expression of the plasminogen activator inhibitor 1 (PAI-1), whereas in the mast cells, the levels of TbetaRI, TbetaRII, and PAI-1 expression were below detection. Selective stimulation of mast cells in vivo in the rat peritoneal cavity leads to rapid overexpression of TGF-beta1 in peritoneal mast cells and of TbetaRs in peritoneal macrophages. These data strongly suggest that mast cells can act as potent paracrine effector cells both by secreting active TGF-beta1 and by enhancing its response in target cells.

When early diagnosed and adequately treated, the long-term prognosis of CLD is favorable. A putative role of a primary anion exchange defect of SLC26A3 in male subfertility and the decline of renal function due to chronic dehydration deserve further characterization.

Objective-To evaluate social maturation and psychiatric morbidity in young adults treated for epilepsy during their childhood. Methods-Eighty one young adults (43 women, 38 men, mean age 22-3 years) with epilepsy during their childhood were interviewed about their social development and psychiatric symptoms. The results were compared with those from 211 randomly selected controls (106 women, 105 men, mean age 23-2 years). Results-Compared with the controls the patients had more often not succeeded in passing the normal comprehensive school (20% v 2%) or had left school at the secondary level (53% v 46%) and remained without any vocational education (27% v 11 %). There was no significant difference in the employment status of those with work between the patients and the controls. The patients were significantly more often labelled with poor social maturation and dependent lifestyle factors such as living with their parents. However, risk analysis showed that neither the disease itself nor antiepileptic medication were significant predisposing factors for poor social adjustment but low or borderline mental capacity or learning disabilities relating to epilepsy were. Psychiatric morbidity was similar in both groups. Conclusions-The social handicap found in a certain group of young adults with epilepsy during childhood is largely associated with neurological and cognitive impairments other than epilepsy itself. With the present mode of treatment epilepsy itself does not seem to disturb adolescent social and psychological development.