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Papers by Ewa Dzienis-Koronkiewicz

Research paper thumbnail of Gastric mucosal exposure to histamine in rats is followed by absorption of histamine but not mucosal hyperemia

Hepato-gastroenterology, 2002

BACKGROUND/AIMS Superficial mucosal damage is associated with mucosal hyperemia and release of hi... more BACKGROUND/AIMS Superficial mucosal damage is associated with mucosal hyperemia and release of histamine into venous blood and gastric lumen. This study was designed to examine if histamine administrated directly to gastric mucosa becomes absorbed and transported away with blood, and if so whether it is capable of increasing gastric mucosal blood flow. METHODOLOGY Superficial mucosal injury was induced by exposing chambered rat stomachs to 2 M NaCl for 10 min, and 45 min later 4.5 x 10(-2) or 4.5 x 10(-3) M histamine was instilled into the chamber. Mucosal blood flow was determined by radioactive microspheres and continuously by Laser Doppler flowmetry. RESULTS Histamine concentration in arterial blood was markedly increased 15 min after administration of histamine. The mucosal blood flow did not increase after application of histamine. Significant increase in heart rate and fall in arterial blood pressure were observed. Similar results were obtained after application of histamine t...

Research paper thumbnail of Hirschsprung disease and other intestinal neuropathies in children

Proper intestinal motility depends on interaction between muscles, nerve cells, and tendinous con... more Proper intestinal motility depends on interaction between muscles, nerve cells, and tendinous connective tissue of muscularis propria. Intestinal motility disorders refer to varied intestinal neuromuscular pathologies, including enteric neuropathies. The most common symptoms of these diseases are delayed passage of meconium in newborns and chronic constipation in infants and older children. If organic causes of clinical features are detected, a further multidisciplinary team approach for the management of these patients is recommended. Entities discussed in this review include Hirschsprung disease, hypoganglionosis, intestinal neuronal dysplasia, ganglioneuromatosis, and chronic intestinal pseudo-obstruction. Emphasis is given to the clinical symptoms and diagnostic features that distinguish these conditions enabling faster diagnosis and appropriate treatment.

Research paper thumbnail of Urazy okołoporodowe ośrodkowego układu nerwowego w materiale Kliniki Chirurgii Dziecięcej Uniwersytetu Medycznego w Białymstoku

STRESZCZENIE Wstęp. Urazem okołoporodowym określamy nieprawidłowy stan noworodka będący wynikiem ... more STRESZCZENIE Wstęp. Urazem okołoporodowym określamy nieprawidłowy stan noworodka będący wynikiem porodu. Najczęściej mamy do czynienia z obrażeniami skóry, układu kostno-stawowego, nerwowo-mięśniowego i urazami poszczególnych narządów. ...

Research paper thumbnail of Late-presenting congenital diaphragmatic hernia in an infant with tuberous sclerosis – case report and a review of the literature

Pediatria Polska

Tuberous sclerosis complex (TSC) is a rare, genetic syndrome, which is characterized by the occur... more Tuberous sclerosis complex (TSC) is a rare, genetic syndrome, which is characterized by the occurrence of small, benign multilocalised hamartomas. The clinical manifestation of the disease is variable, from mild to life threating. This report presents a 9-month-old male baby suffering from TSC, which was diagnosed prenatally. The child was under constant medical, multidisciplinary monitoring. This boy presented skin lesions, hamartomas in the brain and heart, and observation toward hamartoma of the right retina. The infant was admitted to the hospital because of vomiting, fever and cough. Chest X-ray showed left diaphragmatic hernia with mediastinum shift. He underwent thoracoscopic hernia repair. The postoperative period was complicated by a left pneumothorax, atelectasis and pneumonia, but finally the child recovered and remains under ambulatory monitoring. Every pathological symptom must be imaged and diagnosed, despite good general condition.

Research paper thumbnail of Gastric mucosal exposure to histamine in rats is followed by absorption of histamine but not mucosal hyperemia

Hepato-gastroenterology

Superficial mucosal damage is associated with mucosal hyperemia and release of histamine into ven... more Superficial mucosal damage is associated with mucosal hyperemia and release of histamine into venous blood and gastric lumen. This study was designed to examine if histamine administrated directly to gastric mucosa becomes absorbed and transported away with blood, and if so whether it is capable of increasing gastric mucosal blood flow. Superficial mucosal injury was induced by exposing chambered rat stomachs to 2 M NaCl for 10 min, and 45 min later 4.5 x 10(-2) or 4.5 x 10(-3) M histamine was instilled into the chamber. Mucosal blood flow was determined by radioactive microspheres and continuously by Laser Doppler flowmetry. Histamine concentration in arterial blood was markedly increased 15 min after administration of histamine. The mucosal blood flow did not increase after application of histamine. Significant increase in heart rate and fall in arterial blood pressure were observed. Similar results were obtained after application of histamine to normal mucosa. Histamine applied t...

Research paper thumbnail of Successful exclusive propranolol therapy in an infant with life-threatening Kasabach–Merritt syndrome

Journal of Pediatric Surgery Case Reports, 2013

We describe the successful treatment of an infant with life-threatening KasabacheMerritt syndrome... more We describe the successful treatment of an infant with life-threatening KasabacheMerritt syndrome (KMS) who received propranolol therapy. A full-term, 6-week-old female infant, in poor general condition, was admitted with a large vascular tumor of right buttock, perineal region, extending to a lumbar region and the right thigh. There was a gray-blue, tense, shiny, warm swelling over the site of vascular tumor. The child had platelet count of 20,000 cells/mm. D-dimer was positive and suggestive of consumptive coagulopathy. A diagnosis of KMS was made. Computer tomography (CT) imaging was suggestive of a vascular tumor e Kaposiform hemangioendothelioma. The baby was initially treated with platelet, fresh frozen plasma and blood transfusions. There was temporary rise in platelets to 118,000 cells/mm but after transfusions the platelets dropped to 20,000 cells/mm and the baby developed purpuric swelling over right thigh and right buttock. Platelet transfusions were discontinued and propranolol treatment was started. From the 4th day, the swelling started regressing and the platelet count rose to 73,000 cells/mm. At the age of 2 years, the tumor has nearly completely involuted. In our case propranolol proved to be effective first-line treatment for KasabacheMerritt syndrome associated with a vascular tumor e Kaposiform hemangioendothelioma. Minor side effect was poor weight gain after prolonged treatment.

Research paper thumbnail of Treatment of problematic infantile hemangiomas with propranolol: a series of 40 cases and review of the literature

Postępy Higieny i Medycyny Doświadczalnej, 2014

Infantile hemangiomas (IH) are neoplastic proliferations of endothelial cells which occur with an... more Infantile hemangiomas (IH) are neoplastic proliferations of endothelial cells which occur with an incidence of 10 to 12% within the first year of life. IH grow after birth and usually regress spontaneously, but still can lead to deformities when they are located in the facial areas of the lip, eyelid, nasal tip or the ear. We wanted to share our experience in the treatment of problematic IH with propranolol. A retrospective review of medical charts was performed for 40 consecutive children treated with propranolol because of problematic IH between 2009 and 2012. 40 patients (33 girls, 7 boys) with a median age of 4.2 months (aged 1 to 11 months) were treated because of problematic IH. Rapid improvement was reported in the first days of treatment in 38 patients. In one case we had to terminate the treatment because serious tachycardia developed within the first 48 hours after propranolol was started. In this case the patient benefited from alternative treatment with timolol maleate gel. 35 patients (87%) showed an excellent response with complete resolution of the lesion. 4 patients (10%) showed a good result with >50% reduction in the size of the hemangioma. Also a patient with residual IH after terminating oral propranolol benefited from topical treatment with timolol maleate gel. A minor side effect was poor weight gain during prolonged treatment in one patient and tachycardia in another patient in which case we had to terminate the treatment. Conclusions: Our observations show that gradually increasing the dosage of propranolol up to 3 mg/kg and gradually weaning the dosage is safe and effective in treatment of problematic IH. Timolol maleate gel should be considered as a complementary treatment for residual hemangiomas after terminating propranolol treatment or as an alternative treatment in patients who do not tolerate oral propranolol well. hemangioma • children • timolol • propranolol • residual hemangioma Treatment of problematic infantile hemangiomas with propranolol: a series of 40 cases and review of the literature

Research paper thumbnail of Treatment of rapidly proliferating haemangiomas in newborns with propranolol and review of the literature

Journal of Maternal-Fetal and Neonatal Medicine, 2014

Treatment of rapidly proliferating haemangiomas in newborns with propranolol and review of the li... more Treatment of rapidly proliferating haemangiomas in newborns with propranolol and review of the literature Article no: DJMF_A_986650 Enclosures: 1) Query sheet 2) Article proofs Dear Author, Please check these proofs carefully. It is the responsibility of the corresponding author to check against the original manuscript and approve or amend these proofs. A second proof is not normally provided. Informa Healthcare cannot be held responsible for uncorrected errors, even if introduced during the composition process. The journal reserves the right to charge for excessive author alterations, or for changes requested after the proofing stage has concluded. The following queries have arisen during the editing of your manuscript and are marked in the margins of the proofs. Unless advised otherwise, submit all corrections using the CATS online correction form. Once you have added all your corrections, please ensure you press the ''Submit All Corrections'' button. Please review the table of contributors below and confirm that the first and last names are structured correctly and that the authors are listed in the correct order of contribution. Contrib. No.

Research paper thumbnail of Anal atresia and embryonic malignant teratoma

Journal of Pediatric Surgery Case Reports, 2013

We report a unique coincidence of malignant tumor projected via anus in an infant girl operated o... more We report a unique coincidence of malignant tumor projected via anus in an infant girl operated on for congenital anal atresia without fistula. The case seems to be different from Currarino syndrome. After five month of uneventful follow-up after posterior sagittal anorectoplasty (PSARP), a rapidly enlarging polyplike bulge protruded from the anus. Blood tumor markers were negative. X-ray computed tomography (CT scan) revealed a tumor located subcutaneously between the coccygeal bone and rectum. The intraoperative biopsy showed malignancy. By perineal approach, the tumor was radically resected enbloc with anus, part of neorectum, and distal fragment of coccygeal bone. Mature descending colostomy was performed. The postoperative course was uneventful. The final histological diagnosis revealed embryonic malignant teratoma. The bone scintigraphy was negative. Additional chemotherapy was applied without complication. After one year follow-up free of tumor recurrence or metastasis, the perineal anus reconstruction was performed by combined abdominal and posterior sagittal approach. Anal dilatation program was started on 10th postoperative day when the stoma was closed. Five months after the last surgery, the girl presents bowel control and defecates regularly 2e3 times a day without complaints, constipation or cicatricial anal stenosis. Oncologic surveillance is negative.

Research paper thumbnail of Topical timolol gel for the treatment of residual facial hemangioma previously treated with propranolol

Research paper thumbnail of Epidemiology, risk factors, management of cholelithiasis in children and review of the literature

Research paper thumbnail of Ocena rozwoju motorycznego dzieci urodzonych z przepukliną oponowo-rdzeniową

Pediatria Polska, 2011

ABSTRACT

Research paper thumbnail of Analiza etiologii i epidemiologii oraz ocena wyników leczenia oparzeń u dzieci

Pediatria Polska, 2011

STRESZCZENIE Wst p. Oparzenia nale do najbardziej dramatycznych, a jednocze nie najcz stszych ura... more STRESZCZENIE Wst p. Oparzenia nale do najbardziej dramatycznych, a jednocze nie najcz stszych urazów wieku dzieci cego. Cel. Celem pracy jest analiza etiologii, rozleg o ci i stopnia oraz wyników leczenia oparze u dzieci. Materia. Materia do bada stanowi a dokumentacja 357 pacjentów leczonych z powodu oparze w Dzieci cym Szpitalu Klinicznym w Bia ymstoku, w Klinice Chirurgii Dzieci cej i w Poradni Chirurgicznej, w latach 2004-2009. Wyniki. Dzieci najcz ciej ulega y oparzeniu gor cymi p ynami-w ponad 90% przypadków. Najwi cej dzieci by o leczonych zachowawczo. Pi tna cioro dzieci wymaga o leczenia operacyjnego. Czas leczenia-zarówno zachowawczego, jak i operacyjnego by uzale niony od procentu poparzonej powierzchni cia a. Im wi ksza by a powierzchnia oparzenia-tym d u ej trwa o leczenie zachowawcze i operacyjne. Oparzenia Iº i IIº goi y si bez pozostawienia blizn. Oparzenia IIIº i IVº pozostawia y po sobie blizny pooparzeniowe. Wnioski. Wyniki leczenia oparze u dzieci zale przede wszystkim od g boko ci urazu i powierzchni oparzonej. W a ciwe post powanie jest bardzo wa ne w celu osi gni cia dobrego efektu kosmetycznego oraz zapobiegania wtórnym deformacjom w obr bie stawów.

Research paper thumbnail of Ocena odległych wyników leczenia noworodków z martwiczym zapaleniem jelit leczonych w Klinice Chirurgii Dziecięcej UMB w latach 2003–2006

Pediatria Polska, 2010

STRESZCZENIE Cel. Celem pracy jest ocena rozwoju psychomotorycznego dzieci, które były leczone z ... more STRESZCZENIE Cel. Celem pracy jest ocena rozwoju psychomotorycznego dzieci, które były leczone z powodu martwiczego zapalenia jelit i które pozostawały w opiece ambulatoryjnej w Klinice Chirurgii Dziecięcej w czasie 3-letniej obserwacji. Materiał. W Klinice Chirurgii Dziecięcej Uniwersytetu Medycznego w Białymstoku w latach 2003-2006 leczono 19 noworodków z martwiczym zapaleniem jelit. Wyniki. Sześcioro dzieci zmarło. Znacznego stopnia uszkodzenie funkcji psychomotorycznych stwierdzono u 5 pacjentów po leczeniu NEC. Rozwój 5 pacjentów oceniono jako prawidłowy. Gorszy wynik odległy stwierdzono u pacjentów po chirurgicznym leczeniu NEC. Wnioski. Na gorszy rozwój psychomotoryczny dzieci po martwiczym zapaleniu jelit wpływa wiele czynników-czynniki działające jeszcze w okresie prenatalnym, będące przyczyną przedwczesnego porodu, ciężkość przebiegu NEC, deficyty odżywcze, interwencje chirurgiczne i ich powikłania. Najczęstszym odległym powikłaniem martwiczego zapalenia jelit w naszym materiale była niedrożność na tle zwężenia jelit.

Research paper thumbnail of Expression of estrogen receptors α and β in paratesticular tissues in boys operated on for unilateral cryptorchidism between the 1st and 4th years of life

Medical Science Monitor, 2012

The aim of this study was to assess the expression of estrogen receptors a and b in paratesticula... more The aim of this study was to assess the expression of estrogen receptors a and b in paratesticular tissues in a group of boys with and without cryptorchidism, and evaluation of karyotypes, localization, morphology and the major length of the undescended testes. Material/Methods: Fifty boys (1-4 years old) with unilateral cryptorchidism were evaluated. Fifty healthy boys within the same age range, with inguinal hernia, served as a control group. Measurements concerning expression of ERa ERb receptors were preformed using monoclonal mouse antibodies against human receptor a and b. Results: In the mesothelial layer, the expression of ERa was higher in the patients group with undescended testes and it was statistically significant (p=0.04). There was no difference in the expression of ERb in this layer between groups. In the stromal cell layer there was statistically significant higher expression of ERb (p<0.05) in the group of patients with undescended testes. Conclusions: There was no difference between expressions of ERa in stromal cell layer. In the endothelial layer there was no difference in expression of ERa and ERb. In the smooth muscle layer there was no expression of ERa in either group. The expression of ERb in the smooth muscle layer was nearly identical in both groups. Undescended testes were generally found in the superficial inguinal pouch (n=46). The major lengths of the undescended testes were smaller in comparison to the testes positioned normally. In 9 of the cases the testes had different shape, and turgor deficit, and epididymides were smaller, dysplastic and separated from the testis.

Research paper thumbnail of Anti-Müllerian hormone levels in serum 1 year after unilateral orchiopexy

Journal of Pediatric Endocrinology and Metabolism, 2000

The aim of this study was to determine the serum AMH levels in a group of boys with 11 cryptorchi... more The aim of this study was to determine the serum AMH levels in a group of boys with 11 cryptorchidism before and one year after operation. Additionaly the effect of orchiopexy 12 based on the testicular position and morphology was evaluated. Methods: Fifty boys who 13 were 1-4 years old (median = 2.4 years) with unilateral cryptorchidism were evaluated before 14 and one year after they underwent orchiopexy.

Research paper thumbnail of Peritoneal Cell Response during Adhesion Formation

Journal of Investigative Surgery, 2010

We estimated a number of cell populations of peritoneal fluid in rats with experimentally develop... more We estimated a number of cell populations of peritoneal fluid in rats with experimentally developed peritoneal adhesions. The fluid was taken in standarized conditions at the first operation and during reoperation. Animals were divided into four groups. The first group was the control group (n = 20) on which the abdomen was opened and closed without any manipulations (group 1a), and the reoperation was done after 72 hr (group 1b). The other groups (2, 3, 4; n = 20 for each group) were operated, and scarification of the parietal peritoneum and serosa of the bowel was performed. The rats were reoperated after 24, 72, and 168 hr, respectively, after the first surgery. The peritoneal cavity was washed with the 0.9% saline solution. The number of the peritoneal adhesions and populations of the cells were counted. The highest number of peritoneal adhesions was observed in the group of animals reoperated after 72 hr. After 72 and 168 hr, the higher number of mast cells (MC) and neutrophils was observed. The difference was statistically significant. The percentage of mast cells (MC) increased during the experiment. It was different from other cell populations which decreased after 168 hr. The highest change in number was observed for MC. The highest number of MC was observed on the seventh day after the laparotomy. MC influence peritoneal adhesion formation and probably take part in adhesion remodeling. MC and neutrophils changed significantly after manipulations in the peritoneal cavity. Probably, they play an important role in peritoneal adhesion formation.

Research paper thumbnail of Topography and morphometry of intestinal mast cells in children with Hirschsprung's disease

Folia Histochemica et Cytobiologica, 2008

Mast cells (MC) are source of many biological active compounds like cytokines, arachidonic acid d... more Mast cells (MC) are source of many biological active compounds like cytokines, arachidonic acid derivates, proteoglicanes, prostaglandins, proteases, free oxygen radials, NGF, PAF and many more. The role of MC in pathogenesis of Hirschsprung's disease (HD) is not clear. Substances produced by MC may exert an important effect on embryology, growth, differentiation and regeneration of intestinal nervous system. Additionally, MC products modulate inflammation processes thus influencing on the clinical course of HD. Present study was established to evaluate the morphologic MC examination as a support of making diagnosis in HD. The MC topography and morphometry were evaluated in specimens collected from aganglionic colon of patients with diagnosed HD. The results were compared with corresponding data from normally innervated colon of patients suffering from constipation, and normal colon of children not presenting defecation problems. MC were visualized using indirect immunohistochemical method LSAB with mouse antibody against human tryptase. The MC visualized in submucosa and muscular layer in Hirschsprung's disease were significantly larger in comparison with control group (p<0.05). Also the number of MC/mm 2 in mucosa and lamina propria in HD was significantly elevated (p<0.05). However, the MC density in submucosa was also higher but it was not high statistically significant. In muscular layer and in serosa density of MC/mm 2 was not statistically significant. In the intestinal wall MC in aganglionic segment in Hirschsprung's disease are significantly activated comparing with normally innervated colon segments taken from the patients from other groups. This may confirm the role of MC both in pathogenesis of HD and in the reparation processes of bowel nervous system.

Research paper thumbnail of Hemorrhagic Shock-Induced Changes in the Cathepsin D Activity in the Intestinal Wall and Blood Serum in Rats

European Journal of Pediatric Surgery, 1998

Hemorrhagic shock causes release of lysosomal proteolytic enzymes which contribute to intestinal ... more Hemorrhagic shock causes release of lysosomal proteolytic enzymes which contribute to intestinal wall destruction and can be moved into the circulation as well as into the gut lumen. The aim of the study was to examine the activity of cathepsin D in relation to the intestinal wall injury after 60 minutes of untreated hemorrhagic shock in rats. The total cathepsin D activity was investigated in duodenum, jejunum, ileum, cecum and colon, as well as in systemic and mesenteric blood serum, and the biochemical results were compared with morphological changes in the respective segments including immunohistochemical expression of cathepsin D. We found an increase in cathepsin D activity in duodenum and significant decrease in other parts of the gut in shocked rats. The enzyme activity increased also in blood serum, especially systemic (p &lt; 0.05) and insignificantly in mesenteric blood. However, sham-operated animals (one-side carotid artery occlusion) revealed a significant increase in cathepsin D activity measured in mesenteric blood. The shock resulted in lowering protein concentration in the intestinal wall and its increase in mesenteric blood. The contents of peptides and amino nitrogen, as potential proteolytic reaction products, changed in different ways in various segments of intestine. Morphologically, the most intensive destruction was observed in ileum, duodenum and jejunum. Lifting of epithelial layer from lamina propria was the most frequently observed lesion of the intestinal wall after 60 minutes of shock. More advanced lesions, such as denuded mucosa with disintegration of lamina propria, occurred rarely and were not observed in colon and rectum. By means of polyclonal antibodies against cathepsin D, we found that the strong expression of this enzyme was in epithelial layer--the part of intestinal wall which was partially detached into gut lumen due to hemorrhagic shock. The changes of cathepsin D activity after 60 minutes of hemorrhagic shock were correlated with signs of morphological damage to the intestinal wall. Cathepsin D liberation in the intestinal wall during shock indicates the lysosomal membranes impairment and can confirm involvement of proteases in the damage to the intestinal tissue. We conclude that liberation of intestinal cathepsin D is an early phenomenon during hemorrhagic shock which may contribute to the local wall disintegration and activation of systemic inflammatory response.

Research paper thumbnail of Serum AMH concentration as a marker evaluating gonadal function in boys operated on for unilateral cryptorchidism between 1st and 4th year of life—why patients with inguinal hernia served as controls

Endocrine, 2012

The aim of this study was to measure the serum AMH (anti-Mullerian hormone) concentrations in a g... more The aim of this study was to measure the serum AMH (anti-Mullerian hormone) concentrations in a group of boys with or without cryptorchidism, evaluation of karyotypes, testicular position, morphology, and major length of the undescended testes. Fifty boys who were 1-4 years old (median = 2.4 years) with unilateral cryptorchidism were evaluated. All of them underwent orchidopexy in 2010. Prior to the procedure, all of the subjects had undergone karyotyping to exclude chromosomal abnormalities. Fifty healthy boys within the same age range (median = 2.1 years) admitted for planned inguinal hernia repair in 2010, served as controls. Blood samples were collected, while obtaining blood for standard laboratory tests routinely performed before the surgeries. Medians of AMH in boys with cryptorchidism were lower than in boys with inguinal hernia and differed significantly between two groups. Undescended testes were generally found in superficial inguinal pouch (n = 46), in two cases were noted to be in the external ring of the inguinal canal, and in another two instances, in the abdominal cavity. The major lengths of the undescended testes were smaller in comparison to the testes positioned normally (mean of 1 cm vs. a mean of 1.5 cm, respectively). In nine of the cases, the testes had turgor deficit, a drop shape, with epididymides that were small, dysplastic, and separated from the testis. The authors found that AMH was lower in boys with unilateral cryptorchidism (also found to have smaller testis) when compared with the control group.

Research paper thumbnail of Gastric mucosal exposure to histamine in rats is followed by absorption of histamine but not mucosal hyperemia

Hepato-gastroenterology, 2002

BACKGROUND/AIMS Superficial mucosal damage is associated with mucosal hyperemia and release of hi... more BACKGROUND/AIMS Superficial mucosal damage is associated with mucosal hyperemia and release of histamine into venous blood and gastric lumen. This study was designed to examine if histamine administrated directly to gastric mucosa becomes absorbed and transported away with blood, and if so whether it is capable of increasing gastric mucosal blood flow. METHODOLOGY Superficial mucosal injury was induced by exposing chambered rat stomachs to 2 M NaCl for 10 min, and 45 min later 4.5 x 10(-2) or 4.5 x 10(-3) M histamine was instilled into the chamber. Mucosal blood flow was determined by radioactive microspheres and continuously by Laser Doppler flowmetry. RESULTS Histamine concentration in arterial blood was markedly increased 15 min after administration of histamine. The mucosal blood flow did not increase after application of histamine. Significant increase in heart rate and fall in arterial blood pressure were observed. Similar results were obtained after application of histamine t...

Research paper thumbnail of Hirschsprung disease and other intestinal neuropathies in children

Proper intestinal motility depends on interaction between muscles, nerve cells, and tendinous con... more Proper intestinal motility depends on interaction between muscles, nerve cells, and tendinous connective tissue of muscularis propria. Intestinal motility disorders refer to varied intestinal neuromuscular pathologies, including enteric neuropathies. The most common symptoms of these diseases are delayed passage of meconium in newborns and chronic constipation in infants and older children. If organic causes of clinical features are detected, a further multidisciplinary team approach for the management of these patients is recommended. Entities discussed in this review include Hirschsprung disease, hypoganglionosis, intestinal neuronal dysplasia, ganglioneuromatosis, and chronic intestinal pseudo-obstruction. Emphasis is given to the clinical symptoms and diagnostic features that distinguish these conditions enabling faster diagnosis and appropriate treatment.

Research paper thumbnail of Urazy okołoporodowe ośrodkowego układu nerwowego w materiale Kliniki Chirurgii Dziecięcej Uniwersytetu Medycznego w Białymstoku

STRESZCZENIE Wstęp. Urazem okołoporodowym określamy nieprawidłowy stan noworodka będący wynikiem ... more STRESZCZENIE Wstęp. Urazem okołoporodowym określamy nieprawidłowy stan noworodka będący wynikiem porodu. Najczęściej mamy do czynienia z obrażeniami skóry, układu kostno-stawowego, nerwowo-mięśniowego i urazami poszczególnych narządów. ...

Research paper thumbnail of Late-presenting congenital diaphragmatic hernia in an infant with tuberous sclerosis – case report and a review of the literature

Pediatria Polska

Tuberous sclerosis complex (TSC) is a rare, genetic syndrome, which is characterized by the occur... more Tuberous sclerosis complex (TSC) is a rare, genetic syndrome, which is characterized by the occurrence of small, benign multilocalised hamartomas. The clinical manifestation of the disease is variable, from mild to life threating. This report presents a 9-month-old male baby suffering from TSC, which was diagnosed prenatally. The child was under constant medical, multidisciplinary monitoring. This boy presented skin lesions, hamartomas in the brain and heart, and observation toward hamartoma of the right retina. The infant was admitted to the hospital because of vomiting, fever and cough. Chest X-ray showed left diaphragmatic hernia with mediastinum shift. He underwent thoracoscopic hernia repair. The postoperative period was complicated by a left pneumothorax, atelectasis and pneumonia, but finally the child recovered and remains under ambulatory monitoring. Every pathological symptom must be imaged and diagnosed, despite good general condition.

Research paper thumbnail of Gastric mucosal exposure to histamine in rats is followed by absorption of histamine but not mucosal hyperemia

Hepato-gastroenterology

Superficial mucosal damage is associated with mucosal hyperemia and release of histamine into ven... more Superficial mucosal damage is associated with mucosal hyperemia and release of histamine into venous blood and gastric lumen. This study was designed to examine if histamine administrated directly to gastric mucosa becomes absorbed and transported away with blood, and if so whether it is capable of increasing gastric mucosal blood flow. Superficial mucosal injury was induced by exposing chambered rat stomachs to 2 M NaCl for 10 min, and 45 min later 4.5 x 10(-2) or 4.5 x 10(-3) M histamine was instilled into the chamber. Mucosal blood flow was determined by radioactive microspheres and continuously by Laser Doppler flowmetry. Histamine concentration in arterial blood was markedly increased 15 min after administration of histamine. The mucosal blood flow did not increase after application of histamine. Significant increase in heart rate and fall in arterial blood pressure were observed. Similar results were obtained after application of histamine to normal mucosa. Histamine applied t...

Research paper thumbnail of Successful exclusive propranolol therapy in an infant with life-threatening Kasabach–Merritt syndrome

Journal of Pediatric Surgery Case Reports, 2013

We describe the successful treatment of an infant with life-threatening KasabacheMerritt syndrome... more We describe the successful treatment of an infant with life-threatening KasabacheMerritt syndrome (KMS) who received propranolol therapy. A full-term, 6-week-old female infant, in poor general condition, was admitted with a large vascular tumor of right buttock, perineal region, extending to a lumbar region and the right thigh. There was a gray-blue, tense, shiny, warm swelling over the site of vascular tumor. The child had platelet count of 20,000 cells/mm. D-dimer was positive and suggestive of consumptive coagulopathy. A diagnosis of KMS was made. Computer tomography (CT) imaging was suggestive of a vascular tumor e Kaposiform hemangioendothelioma. The baby was initially treated with platelet, fresh frozen plasma and blood transfusions. There was temporary rise in platelets to 118,000 cells/mm but after transfusions the platelets dropped to 20,000 cells/mm and the baby developed purpuric swelling over right thigh and right buttock. Platelet transfusions were discontinued and propranolol treatment was started. From the 4th day, the swelling started regressing and the platelet count rose to 73,000 cells/mm. At the age of 2 years, the tumor has nearly completely involuted. In our case propranolol proved to be effective first-line treatment for KasabacheMerritt syndrome associated with a vascular tumor e Kaposiform hemangioendothelioma. Minor side effect was poor weight gain after prolonged treatment.

Research paper thumbnail of Treatment of problematic infantile hemangiomas with propranolol: a series of 40 cases and review of the literature

Postępy Higieny i Medycyny Doświadczalnej, 2014

Infantile hemangiomas (IH) are neoplastic proliferations of endothelial cells which occur with an... more Infantile hemangiomas (IH) are neoplastic proliferations of endothelial cells which occur with an incidence of 10 to 12% within the first year of life. IH grow after birth and usually regress spontaneously, but still can lead to deformities when they are located in the facial areas of the lip, eyelid, nasal tip or the ear. We wanted to share our experience in the treatment of problematic IH with propranolol. A retrospective review of medical charts was performed for 40 consecutive children treated with propranolol because of problematic IH between 2009 and 2012. 40 patients (33 girls, 7 boys) with a median age of 4.2 months (aged 1 to 11 months) were treated because of problematic IH. Rapid improvement was reported in the first days of treatment in 38 patients. In one case we had to terminate the treatment because serious tachycardia developed within the first 48 hours after propranolol was started. In this case the patient benefited from alternative treatment with timolol maleate gel. 35 patients (87%) showed an excellent response with complete resolution of the lesion. 4 patients (10%) showed a good result with >50% reduction in the size of the hemangioma. Also a patient with residual IH after terminating oral propranolol benefited from topical treatment with timolol maleate gel. A minor side effect was poor weight gain during prolonged treatment in one patient and tachycardia in another patient in which case we had to terminate the treatment. Conclusions: Our observations show that gradually increasing the dosage of propranolol up to 3 mg/kg and gradually weaning the dosage is safe and effective in treatment of problematic IH. Timolol maleate gel should be considered as a complementary treatment for residual hemangiomas after terminating propranolol treatment or as an alternative treatment in patients who do not tolerate oral propranolol well. hemangioma • children • timolol • propranolol • residual hemangioma Treatment of problematic infantile hemangiomas with propranolol: a series of 40 cases and review of the literature

Research paper thumbnail of Treatment of rapidly proliferating haemangiomas in newborns with propranolol and review of the literature

Journal of Maternal-Fetal and Neonatal Medicine, 2014

Treatment of rapidly proliferating haemangiomas in newborns with propranolol and review of the li... more Treatment of rapidly proliferating haemangiomas in newborns with propranolol and review of the literature Article no: DJMF_A_986650 Enclosures: 1) Query sheet 2) Article proofs Dear Author, Please check these proofs carefully. It is the responsibility of the corresponding author to check against the original manuscript and approve or amend these proofs. A second proof is not normally provided. Informa Healthcare cannot be held responsible for uncorrected errors, even if introduced during the composition process. The journal reserves the right to charge for excessive author alterations, or for changes requested after the proofing stage has concluded. The following queries have arisen during the editing of your manuscript and are marked in the margins of the proofs. Unless advised otherwise, submit all corrections using the CATS online correction form. Once you have added all your corrections, please ensure you press the ''Submit All Corrections'' button. Please review the table of contributors below and confirm that the first and last names are structured correctly and that the authors are listed in the correct order of contribution. Contrib. No.

Research paper thumbnail of Anal atresia and embryonic malignant teratoma

Journal of Pediatric Surgery Case Reports, 2013

We report a unique coincidence of malignant tumor projected via anus in an infant girl operated o... more We report a unique coincidence of malignant tumor projected via anus in an infant girl operated on for congenital anal atresia without fistula. The case seems to be different from Currarino syndrome. After five month of uneventful follow-up after posterior sagittal anorectoplasty (PSARP), a rapidly enlarging polyplike bulge protruded from the anus. Blood tumor markers were negative. X-ray computed tomography (CT scan) revealed a tumor located subcutaneously between the coccygeal bone and rectum. The intraoperative biopsy showed malignancy. By perineal approach, the tumor was radically resected enbloc with anus, part of neorectum, and distal fragment of coccygeal bone. Mature descending colostomy was performed. The postoperative course was uneventful. The final histological diagnosis revealed embryonic malignant teratoma. The bone scintigraphy was negative. Additional chemotherapy was applied without complication. After one year follow-up free of tumor recurrence or metastasis, the perineal anus reconstruction was performed by combined abdominal and posterior sagittal approach. Anal dilatation program was started on 10th postoperative day when the stoma was closed. Five months after the last surgery, the girl presents bowel control and defecates regularly 2e3 times a day without complaints, constipation or cicatricial anal stenosis. Oncologic surveillance is negative.

Research paper thumbnail of Topical timolol gel for the treatment of residual facial hemangioma previously treated with propranolol

Research paper thumbnail of Epidemiology, risk factors, management of cholelithiasis in children and review of the literature

Research paper thumbnail of Ocena rozwoju motorycznego dzieci urodzonych z przepukliną oponowo-rdzeniową

Pediatria Polska, 2011

ABSTRACT

Research paper thumbnail of Analiza etiologii i epidemiologii oraz ocena wyników leczenia oparzeń u dzieci

Pediatria Polska, 2011

STRESZCZENIE Wst p. Oparzenia nale do najbardziej dramatycznych, a jednocze nie najcz stszych ura... more STRESZCZENIE Wst p. Oparzenia nale do najbardziej dramatycznych, a jednocze nie najcz stszych urazów wieku dzieci cego. Cel. Celem pracy jest analiza etiologii, rozleg o ci i stopnia oraz wyników leczenia oparze u dzieci. Materia. Materia do bada stanowi a dokumentacja 357 pacjentów leczonych z powodu oparze w Dzieci cym Szpitalu Klinicznym w Bia ymstoku, w Klinice Chirurgii Dzieci cej i w Poradni Chirurgicznej, w latach 2004-2009. Wyniki. Dzieci najcz ciej ulega y oparzeniu gor cymi p ynami-w ponad 90% przypadków. Najwi cej dzieci by o leczonych zachowawczo. Pi tna cioro dzieci wymaga o leczenia operacyjnego. Czas leczenia-zarówno zachowawczego, jak i operacyjnego by uzale niony od procentu poparzonej powierzchni cia a. Im wi ksza by a powierzchnia oparzenia-tym d u ej trwa o leczenie zachowawcze i operacyjne. Oparzenia Iº i IIº goi y si bez pozostawienia blizn. Oparzenia IIIº i IVº pozostawia y po sobie blizny pooparzeniowe. Wnioski. Wyniki leczenia oparze u dzieci zale przede wszystkim od g boko ci urazu i powierzchni oparzonej. W a ciwe post powanie jest bardzo wa ne w celu osi gni cia dobrego efektu kosmetycznego oraz zapobiegania wtórnym deformacjom w obr bie stawów.

Research paper thumbnail of Ocena odległych wyników leczenia noworodków z martwiczym zapaleniem jelit leczonych w Klinice Chirurgii Dziecięcej UMB w latach 2003–2006

Pediatria Polska, 2010

STRESZCZENIE Cel. Celem pracy jest ocena rozwoju psychomotorycznego dzieci, które były leczone z ... more STRESZCZENIE Cel. Celem pracy jest ocena rozwoju psychomotorycznego dzieci, które były leczone z powodu martwiczego zapalenia jelit i które pozostawały w opiece ambulatoryjnej w Klinice Chirurgii Dziecięcej w czasie 3-letniej obserwacji. Materiał. W Klinice Chirurgii Dziecięcej Uniwersytetu Medycznego w Białymstoku w latach 2003-2006 leczono 19 noworodków z martwiczym zapaleniem jelit. Wyniki. Sześcioro dzieci zmarło. Znacznego stopnia uszkodzenie funkcji psychomotorycznych stwierdzono u 5 pacjentów po leczeniu NEC. Rozwój 5 pacjentów oceniono jako prawidłowy. Gorszy wynik odległy stwierdzono u pacjentów po chirurgicznym leczeniu NEC. Wnioski. Na gorszy rozwój psychomotoryczny dzieci po martwiczym zapaleniu jelit wpływa wiele czynników-czynniki działające jeszcze w okresie prenatalnym, będące przyczyną przedwczesnego porodu, ciężkość przebiegu NEC, deficyty odżywcze, interwencje chirurgiczne i ich powikłania. Najczęstszym odległym powikłaniem martwiczego zapalenia jelit w naszym materiale była niedrożność na tle zwężenia jelit.

Research paper thumbnail of Expression of estrogen receptors α and β in paratesticular tissues in boys operated on for unilateral cryptorchidism between the 1st and 4th years of life

Medical Science Monitor, 2012

The aim of this study was to assess the expression of estrogen receptors a and b in paratesticula... more The aim of this study was to assess the expression of estrogen receptors a and b in paratesticular tissues in a group of boys with and without cryptorchidism, and evaluation of karyotypes, localization, morphology and the major length of the undescended testes. Material/Methods: Fifty boys (1-4 years old) with unilateral cryptorchidism were evaluated. Fifty healthy boys within the same age range, with inguinal hernia, served as a control group. Measurements concerning expression of ERa ERb receptors were preformed using monoclonal mouse antibodies against human receptor a and b. Results: In the mesothelial layer, the expression of ERa was higher in the patients group with undescended testes and it was statistically significant (p=0.04). There was no difference in the expression of ERb in this layer between groups. In the stromal cell layer there was statistically significant higher expression of ERb (p<0.05) in the group of patients with undescended testes. Conclusions: There was no difference between expressions of ERa in stromal cell layer. In the endothelial layer there was no difference in expression of ERa and ERb. In the smooth muscle layer there was no expression of ERa in either group. The expression of ERb in the smooth muscle layer was nearly identical in both groups. Undescended testes were generally found in the superficial inguinal pouch (n=46). The major lengths of the undescended testes were smaller in comparison to the testes positioned normally. In 9 of the cases the testes had different shape, and turgor deficit, and epididymides were smaller, dysplastic and separated from the testis.

Research paper thumbnail of Anti-Müllerian hormone levels in serum 1 year after unilateral orchiopexy

Journal of Pediatric Endocrinology and Metabolism, 2000

The aim of this study was to determine the serum AMH levels in a group of boys with 11 cryptorchi... more The aim of this study was to determine the serum AMH levels in a group of boys with 11 cryptorchidism before and one year after operation. Additionaly the effect of orchiopexy 12 based on the testicular position and morphology was evaluated. Methods: Fifty boys who 13 were 1-4 years old (median = 2.4 years) with unilateral cryptorchidism were evaluated before 14 and one year after they underwent orchiopexy.

Research paper thumbnail of Peritoneal Cell Response during Adhesion Formation

Journal of Investigative Surgery, 2010

We estimated a number of cell populations of peritoneal fluid in rats with experimentally develop... more We estimated a number of cell populations of peritoneal fluid in rats with experimentally developed peritoneal adhesions. The fluid was taken in standarized conditions at the first operation and during reoperation. Animals were divided into four groups. The first group was the control group (n = 20) on which the abdomen was opened and closed without any manipulations (group 1a), and the reoperation was done after 72 hr (group 1b). The other groups (2, 3, 4; n = 20 for each group) were operated, and scarification of the parietal peritoneum and serosa of the bowel was performed. The rats were reoperated after 24, 72, and 168 hr, respectively, after the first surgery. The peritoneal cavity was washed with the 0.9% saline solution. The number of the peritoneal adhesions and populations of the cells were counted. The highest number of peritoneal adhesions was observed in the group of animals reoperated after 72 hr. After 72 and 168 hr, the higher number of mast cells (MC) and neutrophils was observed. The difference was statistically significant. The percentage of mast cells (MC) increased during the experiment. It was different from other cell populations which decreased after 168 hr. The highest change in number was observed for MC. The highest number of MC was observed on the seventh day after the laparotomy. MC influence peritoneal adhesion formation and probably take part in adhesion remodeling. MC and neutrophils changed significantly after manipulations in the peritoneal cavity. Probably, they play an important role in peritoneal adhesion formation.

Research paper thumbnail of Topography and morphometry of intestinal mast cells in children with Hirschsprung's disease

Folia Histochemica et Cytobiologica, 2008

Mast cells (MC) are source of many biological active compounds like cytokines, arachidonic acid d... more Mast cells (MC) are source of many biological active compounds like cytokines, arachidonic acid derivates, proteoglicanes, prostaglandins, proteases, free oxygen radials, NGF, PAF and many more. The role of MC in pathogenesis of Hirschsprung's disease (HD) is not clear. Substances produced by MC may exert an important effect on embryology, growth, differentiation and regeneration of intestinal nervous system. Additionally, MC products modulate inflammation processes thus influencing on the clinical course of HD. Present study was established to evaluate the morphologic MC examination as a support of making diagnosis in HD. The MC topography and morphometry were evaluated in specimens collected from aganglionic colon of patients with diagnosed HD. The results were compared with corresponding data from normally innervated colon of patients suffering from constipation, and normal colon of children not presenting defecation problems. MC were visualized using indirect immunohistochemical method LSAB with mouse antibody against human tryptase. The MC visualized in submucosa and muscular layer in Hirschsprung's disease were significantly larger in comparison with control group (p<0.05). Also the number of MC/mm 2 in mucosa and lamina propria in HD was significantly elevated (p<0.05). However, the MC density in submucosa was also higher but it was not high statistically significant. In muscular layer and in serosa density of MC/mm 2 was not statistically significant. In the intestinal wall MC in aganglionic segment in Hirschsprung's disease are significantly activated comparing with normally innervated colon segments taken from the patients from other groups. This may confirm the role of MC both in pathogenesis of HD and in the reparation processes of bowel nervous system.

Research paper thumbnail of Hemorrhagic Shock-Induced Changes in the Cathepsin D Activity in the Intestinal Wall and Blood Serum in Rats

European Journal of Pediatric Surgery, 1998

Hemorrhagic shock causes release of lysosomal proteolytic enzymes which contribute to intestinal ... more Hemorrhagic shock causes release of lysosomal proteolytic enzymes which contribute to intestinal wall destruction and can be moved into the circulation as well as into the gut lumen. The aim of the study was to examine the activity of cathepsin D in relation to the intestinal wall injury after 60 minutes of untreated hemorrhagic shock in rats. The total cathepsin D activity was investigated in duodenum, jejunum, ileum, cecum and colon, as well as in systemic and mesenteric blood serum, and the biochemical results were compared with morphological changes in the respective segments including immunohistochemical expression of cathepsin D. We found an increase in cathepsin D activity in duodenum and significant decrease in other parts of the gut in shocked rats. The enzyme activity increased also in blood serum, especially systemic (p &lt; 0.05) and insignificantly in mesenteric blood. However, sham-operated animals (one-side carotid artery occlusion) revealed a significant increase in cathepsin D activity measured in mesenteric blood. The shock resulted in lowering protein concentration in the intestinal wall and its increase in mesenteric blood. The contents of peptides and amino nitrogen, as potential proteolytic reaction products, changed in different ways in various segments of intestine. Morphologically, the most intensive destruction was observed in ileum, duodenum and jejunum. Lifting of epithelial layer from lamina propria was the most frequently observed lesion of the intestinal wall after 60 minutes of shock. More advanced lesions, such as denuded mucosa with disintegration of lamina propria, occurred rarely and were not observed in colon and rectum. By means of polyclonal antibodies against cathepsin D, we found that the strong expression of this enzyme was in epithelial layer--the part of intestinal wall which was partially detached into gut lumen due to hemorrhagic shock. The changes of cathepsin D activity after 60 minutes of hemorrhagic shock were correlated with signs of morphological damage to the intestinal wall. Cathepsin D liberation in the intestinal wall during shock indicates the lysosomal membranes impairment and can confirm involvement of proteases in the damage to the intestinal tissue. We conclude that liberation of intestinal cathepsin D is an early phenomenon during hemorrhagic shock which may contribute to the local wall disintegration and activation of systemic inflammatory response.

Research paper thumbnail of Serum AMH concentration as a marker evaluating gonadal function in boys operated on for unilateral cryptorchidism between 1st and 4th year of life—why patients with inguinal hernia served as controls

Endocrine, 2012

The aim of this study was to measure the serum AMH (anti-Mullerian hormone) concentrations in a g... more The aim of this study was to measure the serum AMH (anti-Mullerian hormone) concentrations in a group of boys with or without cryptorchidism, evaluation of karyotypes, testicular position, morphology, and major length of the undescended testes. Fifty boys who were 1-4 years old (median = 2.4 years) with unilateral cryptorchidism were evaluated. All of them underwent orchidopexy in 2010. Prior to the procedure, all of the subjects had undergone karyotyping to exclude chromosomal abnormalities. Fifty healthy boys within the same age range (median = 2.1 years) admitted for planned inguinal hernia repair in 2010, served as controls. Blood samples were collected, while obtaining blood for standard laboratory tests routinely performed before the surgeries. Medians of AMH in boys with cryptorchidism were lower than in boys with inguinal hernia and differed significantly between two groups. Undescended testes were generally found in superficial inguinal pouch (n = 46), in two cases were noted to be in the external ring of the inguinal canal, and in another two instances, in the abdominal cavity. The major lengths of the undescended testes were smaller in comparison to the testes positioned normally (mean of 1 cm vs. a mean of 1.5 cm, respectively). In nine of the cases, the testes had turgor deficit, a drop shape, with epididymides that were small, dysplastic, and separated from the testis. The authors found that AMH was lower in boys with unilateral cryptorchidism (also found to have smaller testis) when compared with the control group.