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Wstęp Wprowadzenie nowej farmakologicznej metody doustnej terapii systemowej z użyciem (3-blokerów zrewolucjonizowało leczenie naczyniaków wczesnodziecięcych. Od marca 2009 roku, gdy w naszej Klinice z takich wskazań po raz pierwszy w Polsce zastosowano propranolol, minęło ponad sześć lat. W ciągu 77 miesięcy terapię wdrożono u 268 dzieci, 212 dziewczynek (79,1%) i 56 chłopców (20,9%) w wieku od 4 tygodni do 5,5 roku. Zdecydowana większość leczonych zmian była w fazie proliferacji - 256 (95,5%); w 12 przypadkach (4,5%) leczenie włączono u starszych dzieci w celu przyspieszenia zaniku naczyniaków. U 3 pacjentów zakwalifikowane zmiany nie były naczyniakami. Odpowiedź na leczenie (zahamowanie wzrostu, zmniejszenie zmiany, wygojenie owrzodzeń) zaobserwowano u 252 pacjentów. Oceniono ostateczny wygląd zmian, podkreślając istniejące wskazania do korekcji chirurgicznej i/lub laseroterapii. Cel pracy Prezentacja pierwszych sześciu lat doświadczenia w leczeniu naczyniaków wczesnodziecięcych propranololem ze zwróceniem szczególnej uwagi na potrzebę leczenia chirurgicznego i uzupełniającej laseroterapii. Wnioski Wprawdzie leczenie propranololem wydaje się być postępowaniem z wyboru w przypadku większości naczyniaków wczesnodziecięcych wymagających leczenia, jednakże zdarzają się zmiany oporne na lek, a znaczny odsetek (41%) nadal wymaga procedur inwazyjnych pod postacią redukcji nadmiaru tkanek lub laseroterapii. Najlepsze wyniki uzyskuje się przy wczesnym wdrożeniu leczenia. Nasze 6,5-let-nie doświadczenie potwierdza wysoki współczynnik bezpieczeństwa terapii. [ABSTRACT FROM AUTHOR]

Wstęp Wrodzone malformacje tętniczo-żylne (AVM) to jedna z rzadziej występujących form anomalii naczyniowych skóry i tkanek miękkich. Początkowo o niewinnym wyglądzie różowej lub czerwonej płaskiej „plamy", stopniowo ulegają one progresji wynikającej ze zwiększającego się przepływu przez nieprawidłowe połączenia tętniczo-żylne, przybierając postać guzowatą. Uwypuklaniu towarzyszą nasilające się dolegliwości bólowe, zmiany troficzne skóry z nawracającymi, trudnymi do opanowania krwawieniami, a przy większych zmianach również objawy krążenia hip er kinetycznego z postępującą niewydolnością serca. Tylko postawienie właściwego rozpoznania i podjęcie wczesnego skojarzonego leczenia dają szansę na uzyskanie kontroli nad tą wadą, a w części przypadków trwałego wyleczenia. Cel pracy Przedstawienie na przykładach wybranych pacjentów specyfiki malformacji tętniczo-żylnych, będących wyzwaniem zarówno diagnostycznym w momencie stawiania rozpoznania, jak również terapeutycznym - w czasie nasilających się objawów. Leczenie pacjentów z wrodzonymi anomaliami naczyniowymi wymaga znajomości i zrozumienia aktualnej klasyfikacji ISSVA, ale również umiejętności przewidywania dalszej dynamiki w rozwoju malformacji i wynikających z tego następstw. Z racji zajmowania niejednokrotnie większych obszarów skóry i tkanek miękkich, nierzadko w okolicach o dużym znaczeniu czynnościowym i estetycznym; w leczeniu chirurgicznym AVM niezbędna okazuje się znajomość technik chirurgii plastycznej i rekonstrukcyjnej. Należy podkreślić konieczność łączenia wiedzy i doświadczenia przedstawicieli różnych specjalności wchodzących w skład wielo-specjalistycznego zespołu zajmującego się leczeniem wad naczyniowych powłok. Wnioski 1. Wrodzone malformacje tętniczo-żylne stanowią wyzwanie diagnostyczne i terapeutyczne. 2. W leczeniu AVM łączy się techniki radiologii interwencyjnej z postępowaniem chirurgicznym. 3. Tylko radykalne usunięcie malformacji tętniczo-żylnej daje szansę na całkowite wyleczenie. 4. Leczenie wymaga niejednokrotnie łączenia różnych technik chirurgii plastycznej i rekonstrukcyjnej, takich jak: przeszczepy skóry, ekspansja tkankowa i techniki płatowe. [ABSTRACT FROM AUTHOR]

Wstęp Dziecięca chirurgia plastyczna zajmuje się szerokim spektrum schorzeń, na które składają się wady wrodzone, zmiany pourazowe i następstwa działań leczniczych, a także skutki infekcji itd. Rosnące wymagania co do efektów leczenia prowadzą do wzbogacania konwencjonalnego postępowania chirurgicznego o nowe techniki. Jedną z najbardziej obiecujących i dynamicznie rozwijających się dziedzin jest laseroterapia, będąca w niektórych przypadkach podstawową metodą leczniczą. W październiku 2013 roku zostało otwarte w Gdańsku pierwsze centrum terapii laserowej dla dzieci (w ramach klinicznego ośrodka chirurgii dziecięcej). Cel pracy Celem pracy jest przedstawienie doświadczeń jednego ośrodka w zastosowaniu różnych rodzajów wysokoenergetycznej laseroterapii u dzieci. W pracy uwzględniono pacjentów leczonych w ciągu pierwszych 2 lat funkcjonowania ośrodka. Omówiono główne grupy schorzeń stanowiących wskazania do zastosowania poszczególnych rodzajów laserów. Wnioski Pomimo krótkiego okresu funkcjonowania centrum, zaobserwowano rosnącą liczbę pacjentów wymagających terapii laserowej. Największą grupę tworzą dzieci z wrodzonymi anomaliami naczyniowymi. Dotyczy to w szczególności malformacji kapilarnych, dla których jest to metoda z wyboru. Drugą liczną grupę stanowią pacjenci z bliznami, głównie pooparzeniowymi. Dotychczasowe wyniki zachęcają do dalszych działań na rzecz optymalizacji i rozwoju techniki laserowej. [ABSTRACT FROM AUTHOR]

Background: Infantile hemangioma (IH) is the most common vascular tumor of childhood and infancy. It is distinguished by rapid proliferation of endothelial cells during the first year of life followed by spontaneous regression thereafter. One of the possible factors responsible for the IH development is vascular endothelial grow factor (VEGF). The purpose of this study was to evaluate the influence of selected polymorphisms in the genes coding for VEGF-A (+405 G/C, rs2010963; +936 C/T, rs3025039) and its receptor VEGFR-2 (+1416 T/A, rs1870377; -271 G/A, rs7667298) on the susceptibility to infantile hemangioma., Methods: We performed a case-control study of 99 Polish children hospitalized due to IH and compared them with matched healthy control subjects. The polymorphisms were ascertained through genotyping by PCR-RFLP assay, PCR-HRM, or the allelic discrimination method., Results: The study revealed a lower odds of infantile hemangioma in individuals with GG genotype or G allele for +405 G/C VEGF-A polymorphism (OR dis = 0.52, P = 0.023 and OR dis  = 0.63, P = 0.025, respectively). No association was observed for the remaining VEGF and VEGFR-2 polymorphisms and IH risk., Conclusions: In our study, none of the investigated VEGF-A and VEGFR-2 genes polymorphisms was found to be an independent prognostic marker for infantile hemangioma. However, there is evidence that individuals carrying at least one G allele of +405 G/C VEGF-A polymorphism have significantly lower risk of IH., (© 2018 The International Society of Dermatology.)

Unlabelled: In the last few years propranolol has revolutionized infantile hemangioma therapy. This nonselective β bloker has been proven to be safe and effective but the molecular bases of its actions remain unclear. One of debated theories holds that propranolol may inhibit angiogenesis and induce apoptosis. To investigate this claim, this study aims to analyze the serum and tissue profiles of VEGF and VEGRR1/2 in patients treated with propranolol., Materials and Methods: To assess the expression if VEGF and VEGRR1/2 we used three independent methods. First we analyzed serum VEGF levels in 50 children with IH before and 3 months after the therapy using ELISA test (I.). Then we used immunohistochemistry to evaluate tissue expression of VEGF and VEGFR1/2 in IH treated (n=27) and not treated (n=45) with propranolol (II.). Finally we assessed mRNA of VEGF and VEGFR1/2 in the same patients as in part II (III.)., Results: (I) There was no distinct decrease of VEGF level in children with IH after propranolol treatment. (II) We found no significant difference in VEGFR1 and VEGFR2 expression in hemangiomas from the study and control group. The expression of VEGF was even higher than before therapy. (III) VEGF and VEGFR1 mRNA expression was significantly lower in IH tissue after propranolol treatment compared to those without treatment. VEGFR2 demonstrated no differences in expression between the two groups., Conclusions: The obtained results show distinct discrepancies between in vitro and clinical studies as well as among different methods used for analyzing the same phenomenon. Only VEGF and VEGFR1 expression in mRNA studies may prove the proposed theory of antiangiogenic properties of propranolol. Other results do not confirm it and remain inconsistent with the fantastic clinical response to this medication., (Copyright © 2016 European Federation of Immunological Societies. Published by Elsevier B.V. All rights reserved.)

Purpose: morphobiometric evaluation of macula and peripapillary retinal nerve fiber layer thickness with the use of high-resolution optical coherence tomography in children with a history of regressed retinopathy or prematurity., Materials and Methods: 18 patients at the age of 8 to 14 years with a history of spontaneously regressed retinopathy of prematurity were studied prospectively. For statistical purposes a control group of 21 matched subjects at the age of 8 to 15 years was used. Ophthalmic examination and optical coherence tomography were performed in each patient. Peripapillary nerve fibre layer thickness, foveal and parafoveal thickness ratio, total macular volume and subfoveal choroidal thickness were measured in both groups., Results: in the optical coherence tomography, the foveal thickness in children with retinopathy of prematurity was significantly higher [269.5 μm (232-321)] compared to the controls [224.5 μm (207-267)]. The macular volume in the study group was also higher (8.68 mm³). The subfoveal choroidal thickness was reduced in study group [321 μm (112-365)] compared to the control group [337 μm (294-358)]. There was no statistical significant difference in total peripapillary nerve fibre layer thickness between the two groups., Conclusion: The morphobiometric macular changes in eyes with a history of regressed retinopathy of prematurity are possibly related to the developmental abnormalities, which retinopathy of prematurity is due to the presence of the abnormal foveal structure across all retinal layers.

Purpose: To evaluate the effect of optic nerve head drusen on retinal nerve fiber layer (RNFL) thickness in children, with the use of optical coherence tomography (OCT)., Material and Methods: 21 patients (mean age 13.9 years) with optic nerve head drusen were studied prospectively. For statistic comparison a group of 15 control subjects (mean age 14.1 years) participated. Ophthalmologic examination, automated visual field testing and optical coherence tomography were performed in each patient. For statistical analysis we used Shapiro-Wilk and U Mann-Whitney tests., Results: In the OCT measurements the nasal RNFL was significantly thinner as compared to the reference group. Neither statistically significant thinning of global RNFL between groups nor relation between subjects age and RNFL thickness was found., Conclusions: Optic nerve head drusen can lead to visual function defects, therefore early and correct diagnosis is mandatory. Optical coherence tomography is a usefull diagnostic tool used to determine the cause of disc elevation, which is especially important in childhood, when drusen can cause disc to appear papilledema-like. Overall OCT appears to be a non-invasive, sensitive and early-indicating method of RNFL thinning.

The insulin-like growth factors (IGF-I and IGF-II) may play an important role in postpneumonectomy compensatory lung growth by translating hormonal inputs and mechanical forces into cellular proliferation signals. We examined the mRNA abundance of IGF-I, IGF-II, and IGF binding proteins (IGFBPs) in lungs of rats on postoperative days 1, 2, 3, 5, and 7 following left pneumonectomy (PNX) or shamoperation (SC) and in normal animals (CON). There was no difference in the abundance of lung IGF-I mRNA (measured by Northern analysis) or serum IGF-I (measured by radioimmunoassay (RIA)) between SC and PNX animals. IGF-II mRNA abundance was initially decreased following PNX (73% decrease compared to SC animals on day 1, p < .05) and then rose to approach SC group values on subsequent days. Transcripts for IGFBP-2, -3, -4, -5, and -6 were decreased in both the SC and PNX groups compared to CON animals on the day following pneumonectomy, then rose back to baseline by postoperative day 2-3. Tissue IGFBPs, measured by ligand blot analyses, were not different in either the SC or PNX groups. In contrast, all serum IGFBP bands were increased on postoperative day 1 following either sham or PNX surgery. In addition, serum IGFBP-4 was increased in PNX animals compared to the SC group on days 1 and 2 (increase of 38% and 78%, respectively, p < .05). We conclude that the changes observed in lung IGF and IGFBP expression following pneumonectomy do not represent major.

A case of a patient 62 years old with a severe congestive heart failure in the course of aortic stenosis and after myocardial infarction was presented. A concomitant mitral insufficiency, tricuspid insufficiency and pulmonary hypertension was also observed. An excellent improvement after aortic valve replacement was reached.

Ectodermal anhydrotic dysplasia is a rare, usually X-linked recessive malformation of ectodermal tissues and organs. The case of a 17-year-old boy with ectodermal anhydrotic dysplasia and concomitant combined mitral valve disease and aortic insufficiency is described. The surgical risk was considered to be high due to severe atrophia of the laryngo-pharyngeal mucosa complicating endotracheal intubation and the possibility of postoperative temperature control disturbances. Double valve replacement was performed late in the autumn, after laryngo-pharyngeal pharmacological treatment preparing for endotracheal intubation. The peri-operative course was managed without any complication related to the hereditary malformation. Our experience suggests that patients suffering from ectodermal anhydrotic dysplasia may undergo open heart surgery after appropriate preparation. Potential postoperative problems related to the hypohydrosis and impaired spontaneous temperature control could be prevented in our patient.

The aim of this study was to estimate coincidence of coronary artery disease (CAD) and rheumatic mitral valve disease in 264 patients treated in the National Institute of Cardiology (1976-1990). Severity of stenoses on coronary angiography with respect to age, sex, symptoms and risk factors were also estimated. Stenoses over 70% of artery lumen in relation to artery diameter before lesion and over 50% for left main coronary artery were stated as severe. There were 180 (68%) females and 84 (32%) males in the studied group; mean age was 52.5 year. Patients were divided into two groups: with angina--126 pts and without CAD symptoms--138 pts. 8 females (4%) had severe stenoses and 45 (25%) non-severe. Respectively 14 males (16.7%) had severe stenoses and 14 non-severe. Severe lesions were present in a group of females older than 50 years and in a group of males older than 45 years. Both in group with or without angina prevalence of coronary artery lesions was similar. Sensitivity and specificity of CAD clinical symptoms was low (less than 50%). Significantly more risk factors were present in pts with coronary stenoses than in pts free of CAD. No correlation between high pulmonary artery pressure and angina in patients without coronary stenoses occurred.

We present a rare case of bacterial endocarditis of tricuspid valve caused by temporary intracardiac pacing. The 48-year old male patient developed complete a-v block during the 1st day of acute inferior myocardial infarction. Intracardiac electrode was inserted for temporary pacing. After 4 days signs of bacterial endocarditis developed. Patient was markedly febrile, moderate tachycardia with gallop rhythm and systolic murmur of tricuspid valve insufficiency were present. Dullness to percussion was audible at the base of right lung. Hepato- and splenomegaly appeared during the second month of hospitalization. Laboratory tests revealed: elevated ESR, leukocytosis with a shift to the left, several blood cultures were positive to Staphylococcus aureus. On repeated chest X-ray patchy infiltrates with thin-walled translucent pools were visible. Transthoracic and transoesophageal++ echocardiography provided more precise informations. Bacterial vegetations were visualised on the tricuspid valve. Coronary angiography revealed proximal occlusion of the right coronary artery and 75-80% stenosis of the left circumflex artery. Antibacterial treatment guided by blood cultures was begun: vancomycin combined with netilmycin, then tienamycin and diflucan--after 10 weeks treatment was decided to be unsuccessful and the decision about surgical treatment was made. In extracorporeal circulation posterior left leaflet together with granular bacterial growths was excised. Septal and anterior leaflets were found normal. Cultures made of excised tissue were positive for Staphylococcus aureus and subsequent treatment with fluoroquinolones gave satisfactory result. Postoperative echocardiography revealed only small tricuspid valve insufficiency. Coronary by-pass surgery was performed later because of the high risk of simultaneous operation.(ABSTRACT TRUNCATED AT 250 WORDS)

A number of investigators have shown that 5-hydroxytryptamine (5-HT) administration to pregnant animals produces changes in the placenta and leads to death of the fetuses. It has been well known that 5-HT is the most potent umbilical-placental vasoconstrictor and that its administration affects the nutritional function of the placenta. In view of these results it seemed desirable to determine 5-HT concentration in the placenta in experimental intrauterine growth retardation. IUGR was induced on the 17th day of gestation in pregnant rats by ligating the uterine artery of one horn as described by Wigglesworth with the opposite horn left untouched (control). On day 22, fetuses were delivered by C-section. 5-HT was determined by a fluorometric method. Statistical analysis employed the paired Student t-test. Average weight of IUGR fetuses was 3,05 g, whereas control fetuses 4,48. The mean concentration of 5-HT was 11% higher in IUGR fetal placentas. The mean placental concentration in IUGR was 247 micrograms/g of tissue, whereas in control the mean concentration was 222 micrograms/g of tissue. The difference between these placental concentrations was significant (p less than 0,01). We conclude that decreased blood supply to the pregnant rat uterus results in increased concentration in the placenta of 5-HT.

The concentration of serotonin (5-hydroxytryptamine, 5-HT) was determined in the brain of fetal rats with experimental intrauterine growth retardation (IUGR). IUGR was induced using the procedure of Wigglesworth. On the 17th day of gestation, the uterine artery supplying one uterine horn was ligated. The artery of the opposite uterine horn was left untouched (control). On the 22nd day the fetuses were delivered by C-section. 5-HT was determined by fluorometric method. Statistical analysis employed the paired Student t-test. The average body weight of the IUGR fetuses was 3.15 g, whereas control fetuses 4,50 g. The mean concentration of 5-HT was 10,4% lower in IUGR fetal brains. The mean 5-HT brain concentration in IUGR was 155 micrograms/g of tissue, whereas in control the mean concentration was 173 micrograms/g of tissue. The difference between these brain concentrations was significant (p less than 0,005). We conclude that reduction in maternofetal blood flow in the 3rd trimester of gestation results in decreased concentration in the brain of 5-HT.

Hypophysectomized rats were injected intraperitoneally for 4 days with various doses of homocysteic acid or growth hormone. The effects of these compounds on epiphyseal cartilage thickness and circulating somatomedin activity levels were evaluated in an attempt to repeat the results of Clopath, Smith, and McCully, who reported that this compound had growth hormone-like activity. DNA polymerase activity in livers of animals treated with growth hormone or with 10 mg/day of homocysteic acid was also measured. Using larger number of animals and including higher doses of homocysteic acid than those previously employed, we did not observe an increase of epiphyseal cartilage thickness in homocysteic acid treated hypophysectomized rats. Growth hormone significantly increased cartilage thickness. DNA polymerase levels in homocysteic acid treated hypophysectomized rats were not substantially increased although a larger, dose-dependent increase was observed with pGH and hGH. Neither homocysteic acid nor GH increased circulating somatomedin activity under the conditions used in this investigation. These observations demonstrate that homocysteic acid was not a substance with growth hormone-like activity in our hands and cast doubt on its possible future usefullness as a substitute for GH in clinical situations.

Experiments described here with partially nephrectomized pregnant rats with pair-fed controls and controls fed at will indicate that decreased maternal food intake is a major factor in the intrauterine growth retardation associated with moderate renal insufficiency during the last trimester of gestation. Although renal disease in human pregnancy is often associated with vascular insufficiency, the possibility that maternal undernutrition may also play a contributory role in the fetal growth failure associated with certain cases of human renal compromise merits further study.