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Fryns Syndrome - Case Report. Documentation of Anomalies not Described Previously and Review of the Literature

100%
Bacewicz L., Polnik D., Ganowicz J., Apanasiewicz A., Kaliciński P.
Polish Journal of Surgery
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2009
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vol. 81
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issue 3
149-155
EN
Fryns syndrome is a rare and most often lethal autosomal recessive complex congenital malformation. We report on a case of Fryns syndrome with "coarse facies", broad nasal bridge, dysmorphic low-appearing ears, left cleft lip and palate, left congenital diaphragmatic hernia with lung hypoplasia, distal digital limb hypoplasia of fifth's finger and thumbs, ureter ectopia to the persistent urogenital sinus, bilateral megaureters and hydronephrosis, agenesia of corpus callosum, hernia of the umbilical cord, Meckel's diverticulum, malrotation, choledochal cyst and bilateral inguinal hernia. The persistent urogenital sinus with ectopic ureters and choledochal cyst were not previously described in the literature in association with other features of Fryns syndrome. These malformations add to our knowledge of the phenotype of Fryns syndrome.
2
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Immaturity of Ganglion Cells - A Study of Our Own Material

86%
Markiewicz-Kijewska M., Kowalski A., Bacewicz L., Drewniak T., Ismail H., Kluge P., Kaliciński P.
Polish Journal of Surgery
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2009
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vol. 81
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issue 2
95-102
EN
The most common causes of clinical symptoms of bowel obstruction in newborn are the various dysmotylity disorders.The aim of the study was a retrospective analysis of the newborn's case history with the diagnosis of ganglion cells immaturity, their clinical course, diagnostic imagine and treatment in our own material.Material and methods. In the years 1981-2007 we treated in the Department of Pediatric Surgery in Children's Memorial Health Institute 420 children with various dysmotylity disorders so-called dysganglionosis. Among them were 15 infants, who finally were diagnosed ganglion cells immaturity. In all clinical symptoms associated with impaired motoric function of large bowel occurred in the first week of life. We performed a retrospective analysis of the case history of these children's including: demographic data, perinatal anamnesis, the dominant clinical symptoms, how to conduct an emergency and a final treatment, and long term follow-up.Results. The children were born at 28-40 week of pregnancy (mean 35,9 weeks) with the body mass between 0.95 and 4.15 kg (mean 2,72 kg), 7 of them were premature infant in 28-36 week of pregnancy and body mass 950-2900 g (mean 1970 g). All infants after birth were evaluated by the Apgar's scale of the 1-10 point (mean 8.5). The first meconium in the first 24 hours of life passed only 1 infant, the remaining 12 meconium passed delayed from 2 to 4 day of life. In 5 children were present concomitant diseases and malformations making it difficult to establish early correct diagnosis: congenital gastroschisis (1 child), intestinal volvulus (1 child) and necrotizing enterocolitis (NEC) (3 children). Diagnosis of ganglion cells immaturity was found at age from 1 to 365 day on the basis of the clinical course, radiological imagine (11 children) and histopathological test of rectal biopsy specimens (9 children) or surgical biopsy specimens (6 children). Four children were treated medically; the others require the temporary emergence colostomy or ileostomy. Functional maturity of ganglion cells identified between 2 and 16.5 month on the basis of electromanometric study (12 children), and/or rectal biopsy specimens (7 children) and in 3 only on clinical course. GI tract reconstruction was performed in the other 11 children at aged from 4.2 to 20.3 month of life, mean 10.6 month. After GI tract reconstruction in 1 child it was mechanical adhesive ileus and malabsorption of unknown origin requiring partial parenteral nutrition. In another child remained ileus and chronic cholestasis, this patient died after next laparotomy because of bleeding and failure liver function. Other patients live in a good general condition with a normal motoric function of the digestive tract from long term follow-up 1.8 to 17.8 years (mean 6.7 years).Conclusions. Early differential diagnosis of dysganglionosis is difficult, but possible subject to the full panel of diagnostic (radiological imagine, electromanometric and histopathological study) already in newborn with impaired bowel motoric function. Only in such cases with correct diagnosis patient can be adequately treated and to guarantee good initial and definitive treatment.
3
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Radiofrequency Assisted Partial Resection of Parenchymal Organs in Children

73%
Nachulewicz P., Kaliciński P., Kasprzyk W., Kowalski A., Szymczak M., Drewniak T., Ismail H., Stefanowicz M., Bacewicz L.
Polish Journal of Surgery
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2009
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vol. 81
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issue 3
135-141
EN
The aim of the study was the analysis of radiofrequency assisted partial resection of parenchymal organs in children performed in our center.Material and methods. First operation with use of thermoresection was performed in February 2005 and since than we performed 19 such procedures. In 9 patients partial resection of spleen (hemisplenectomy), in 8 patients partial resection of liver were performed. In 2 cases we performed "in situ" reduction of liver during cadaveric multiorgan procurement (left hepatic lobe was procured).Age of the patients ranged from 19 days to 18 years, medium - 4.5 years. Indication for partial resection of splen was cyst in all cases. In 6 patients it was located in superior pole, in 3 in inferior pole of spleen. Laparoscopic procedure was performed in 5 patients, open surgery was performed in 4 children.Indication for partial resection of liver was neoplasm in 4 cases (3 hepatoblastoma, 1 undiferentiated sarcoma). In 2 patients indication for operation was giant liver hemangioma, in 1 - focal nodular hyperplasia (FNH), in 1 - ischemia of the right lobe of the liver after transplantation.Results. Postoperative period was uncomplicated in all cases except three of them. In 1 patient with acute liver failure, after transplantation ischemia of the liver revealed which was caused by compression of the costal archs. Right hemihepatectomy was performed in the 2nd day after transplantation. This patient died in the 3rd day after transplantation due to multiorgan failure. In newborn patient with giant liver hemangioma which occupied right lobe and IV segment, stricture of biliary duct occured. The most possible cause of the stricture was the result of the radiofrequency coagulation impact on the left biliary duct lying to close to the hemagioma. In 1 patient 2 months after resection of the superior spleen pole, perforation of the diaphragm and diaphragmatic hernia occured which required operation. Follow up of the patients after thermoresection is 5 to 36 months.Conclusions. Monopolar radiofrquency coagulation is very useful technique in partial resection of solid organs in children however there are limitations due to risk of the use of the coagulation too close to the vessels or biliary ducts.
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Liver Transplantation in Infants with Body Mass Less than 6 KG

59%
Ismail H., Broniszczak D., Apanasiewicz A., Kasprzyk W., Teisserye J., Bacewicz L., Teisserye M., Markiewicz-Kijewska M., Szymczak M., Jezierska E., Kaliciński P.
Polish Journal of Surgery
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2009
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vol. 81
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issue 2
75-80
EN
Liver transplantation (LTx) is the only treatment of fulminant liver failure and end stage liver disease (ESLD).The aim of the study was to assess indications, status at transplantation, surgical techniques, early and late complications and outcome of liver transplantation in children less than 6 kilograms of body weight.Material and methods. Between 1990 and 2008 in the Department of Pediatric Surgery and Organ Transplantation, Children's Memorial Health Institute, there were performed 350 LTx. Within them there were 13 (3.7%) newborn and infants with body weight below 6 kilogram. Retrospective analysis was carried out regarding indications for LTx, status at LTx, surgical techniques, early and late follow-up and outcome.Results. Follow-up of 11 living patients ranges from 5 to 111 months (mean 31.5 months). Patients and graft survival is 84.5%. In all patients liver function is very good or good. Advancement in liver surgery and perioperative care caused that LTx in small recipients became possible and are carried out with good outcome.Conclusion. Encouraging results of liver transplantation in newborn and infants caused that lower age and weight limits for liver recipients are no more significant.
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