The use of recombinant activated factor VII in the circumcision operation in the case of a congenital factor VII deficiency.

Congenital factor VII deficiency is a rare autosomal recessive hemorrhagic disorder and surgery is normally the cause of excessive bleeding. In this report, we describe the first case with congenital factor VII deficiency admitted to our clinics for the sunnet operation (circumcision), in which recombinant activated factor VII (rFVIIa; NovoSeven) was used to manage the bleeding. The patient was an 8-year old boy with moderate factor VII deficiency (factor VII level, 4%), and rFVIIa was administered at a dose of 20 microg/kg per dose during the circumcision operation. The same dose was repeated at 2, 4, 6, 9, 12, 15, 18, 21 and 24 h post operation. The circumcision operation could therefore be safely performed in patients with congenital factor VII using rFVIIa.

An unusual association of TAR syndrome with esophageal atresia: a variant?

Thrombocytopenia and absent radii (TAR) syndrome is a rare disorder. It may be associated with, for example, as cardiovascular, genitourinary, gastrointestinal, skeletal, neurological, ophthalmic, and facial anomalies. Esophageal atresia is also a rare anomaly associated with genitourinary, skeletal, and cardiovascular anomalies, among others. Here, the authors present a child with TAR syndrome and esophageal atresia with tracheoesophageal fistula. This association has been reported in only one other case in literature.

Intestinal atresia with segmental musculature and neural defect.

BACKGROUND/PURPOSE: The aim of this study was to investigate the possible etiologic factors of small bowel atresia and to detect the prognostic role of adequate resection and tapering in postoperative morbidity and mortality. METHODS: Intestinal resection specimens were obtained from 10 patients with jejunoileal atresia and 3 control subjects without any gastrointestinal disease. Intestinal specimens taken from 2-cm and 4-cm proximal sides of atresia, atretic segment, and 1-cm and 2-cm distal sides of atresia were stained with Masson trichrome and H&E. Immunohistochemical staining of the biopsy specimens with synaptophysin was also performed to ascertain the number, the intensity, and the morphology of ganglia. RESULTS: At the blind proximal end, there was segmental absence of muscular layers, presence of neural defects, and replacement of the muscular layers with fibrous tissue beside the relatively intact mucosa. CONCLUSIONS: Segmental defects in muscular and neural structures of the intestinal wall observed in both the antimesenteric and mesenteric sides of the atretic small bowel were considered to support the vascular insult theory as an etiologic factor. Adequate resection rather than tapering the dilated proximal atretic intestinal segment should be included in the surgical treatment of this pathology to avoid the intestinal dysmotility, which may result in gut-related sepsis and death in the postoperative period.

Ileoileal invagination without obstruction in a four-year-old boy.

Intussusception is one of the most common causes of acute abdomen in the first year of life. Its clinical presentation is vomiting, bloody stools, severe colicky abdominal pain, and mass. The authors reported a case of intussusception with an inappropriate clinical picture, but both characteristic ultrasonography and computed tomography findings led to the diagnosis of ileo-ileal intussusception.

An unusual type of severe barotrauma owing to automobile tire biting in a child.

The authors report a rare case of a barotraumatic pneumothorax and pneumomediastinum associated with esophageal perforation and orbital emphysema. A 4-year-old boy presented with sudden respiratory distress after blowout of a defective tire that he bit. Computed tomography scan showed right pneumothorax and bilateral orbital emphysema. A linear rupture has been detected in the cervical esophagus in esophagoscopy. Stamm gastrostomy and tube thoracostomy were performed, and broad-spectrum antibiotics have been introduced. Oral feeding started 23 days after blowout of tire, and the patient was discharged 50 days after injury.

Clinical experience of removing aerodigestive tract foreign bodies with rigid endoscopy in children.

OBJECTIVES: This study was undertaken to document the aerodigestive tract foreign body accidents among children, and to investigate the circumstances surrounding these events. METHODS: A review of the charts of pediatric patients admitted with the definitive or suspicious diagnosis of aerodigestive tract foreign bodies was carried out in the period between January 1, 1998 to December 31, 2002. RESULTS: There were 53 eligible children; 39 boys and 14 girls, with an age range of 7 months to 14 years. Food items were the most common airway foreign bodies and coins were the most common esophageal foreign bodies. Among the 32 patients who underwent bronchoscopy, no foreign body was identified in 9 patients. Among the 21 patients who underwent esophagoscopy, foreign body was removed in 19 patients. In 2 cases, large foreign bodies which we could not extract with forceps were pushed into the stomach. CONCLUSIONS: Foreign bodies in the airway and esophagus constitute a constant hazard in all age groups, which demands immediate approach and management. Although the rigid endoscopic removal of aerodigestive foreign bodies was successful in this series, the most effective treatment of foreign body accidents is their prevention.

Lung injury after aortic occlusion-reperfusion in rats: the role of gadolinium chloride.

Aortic ischemia-reperfusion (AIR) induced lung injury has already been documented. Kupffer cell blockage (KCB) with gadolinium chloride (GdCl3) has also been shown to attenuate remote organ damage caused by ischemia reperfusion. The present study was designed to examine the effect of GdCl3 in lung ischemia-reperfusion injury induced by aortic occlusion. Thirty-two rats were randomly allocated to four groups as follows: SHAM (Sham Laparotomy), SHAM+KCB, AIR, and AIR+KCB. An atraumatic microvascular clamp was placed across the infrarenal abdominal aorta just after its origin from the aorta for 30 minutes. The microvascular clamp on the infrarenal abdominal aorta was removed and reperfused for 60 minutes. GdCl3 was given 24 hours prior to the experiment. Malondialdehyde (MDA) level and myeloperoxidase (MPO) activity were assayed in lung tissues. MDA level and MPO activity in the AIR group were significantly higher than those in the other groups. When compared to AIR group, KCB with GdCl3 significantly decreased MDA level and MPO activity in the AIR+KCB group. These results suggest that GdCl3 attenuates the lung injury caused by AIR. The effects of GdCl3 on reduced lung damage may be mediated through significant decreases in both MDA level and MPO activity.

Splenectomy attenuates intestinal ischemia-reperfusion-induced acute lung injury.

BACKGROUND/PURPOSE: Intestinal ischemia-reperfusion (IIR) induced acute lung injury (ALI) has been documented. Kupffer cell blockage with gadolinium chloride (GdCl(3)) has been shown to attenuate IIR-induced ALI. However, the effects of splenic monocytes/macrophages on IIR-induced ALI has not been studied previously. In the current study, the authors aimed to investigate the role of splenectomy in IIR-induced ALI. METHODS: Forty-eight rats were divided randomly into 6 groups as follows: SHAM, SHAM + KCB, SHAM + SPLN, IIR, IIR + KCB, IIR + SPLN. Two hours of ischemia and 1 hour of reperfusion has been applied by clipping the SMA. GdCl3 was given 24 hours before experiment. Splenectomy was done just before SMA clipping. Lung levels of tumor necrosis-factor (TNF), interleukin (IL)-6, myeloperoxidase (MPO), and malondialdehyde (MDA) were assayed biochemically. Lung leukosequestration was determined by counting PMNLs histologically. Kruskal-Wallis and Mann-Whitney U tests were done; P values less than.003 were considered significant. RESULTS: Polimorphonuclear leukocyte (PMNL) counts and biochemical parameters in the IIR group were significantly higher than the other groups (P <.003). When compared with IIR group, PMNL counts and biochemical parameters were significantly decreased in the IIR+KCB and IIR+SPLN groups, respectively (P <.003). However, they were still significantly higher than their sham-operated controls (P <.003). CONCLUSIONS: This study documents that splenectomy attenuates ALI as well as Kupffer cell blockage. Spleen, an important component of mononuclear phagocytic system as liver Kupffer cells, might play an important role in the IIR-induced ALI.

The effects of unilateral testicular ischemia and hemicastration on contralateral testicular IGF-1 level, histology and lipid peroxidation.

Hemicastration is followed by compansatory hypertrophy whereas unilateral testicular torsion is followed by atrophy in contralateral testicle in rats. Insulin-like growth factor (IGF-1) has important roles in testicular paracrine and autocrine functions. In this study it was aimed to compare ischemic parameters and IGF-1 levels in the contralateral testicle in unilateral spermatic cord ligation, testicular torsion, and hemicastratron. 32 wistar rats were equally altocated into sham, ligation, torsion, and hemicastration groups. In ligation group, right spermatic cord was ligated with 3/0 silk suture. In the torsion group, right testis was tcrsed for 720 degrees. In hemicastration group, right orchiectomy was done. 48 hours later left orchiectomy was done in all groups. Malondialdehyde (MDA) and IGF-1 levels were determined in the testicle. Average values of the groups were compared with Anova followed by Dunnett T3 multiple comparison tests. MDA levels were significantly reduced in ligation and torsion groups (p < 0.05). This reduction was more prominent in hemicastration group (p < 0.05). Contralateral testicular IGF-1 levels in ligation and torsion groups were not different compared with the sham group. Left testicular IGF-1 level in the hemicastration group was decreased significantly compared with other groups (p < 0.05). Histological. changes evaluated. Contralateral Johnsen's testicular biopsy scores were significantly decreased in all experimental groups but mean tubular diameter was not changed in all groups.

The effect of electromagnetic field on undescended testis after orchiopexy.

Undescended testis is a common problem leading to infertility. After orchiopexy some studies support the necessity of hormonal therapy. Electromagnetic field stimulation on living tissues increase cell proliferation, protein and DNA synthesis. Sixteen prepubertal rats was objected to the fixation of left testes to the anterior abdominal wall for 30 days, right testes were removed. Another group of sixteen rats objected only to the right orchiectomy and a manipulation simulating study group without fixation. After orchiopexy, animals were divided into two groups. Both groups had eight rats. Electromagnetic field (EMF) stimulation group had the stimulation for two hours every day for ten days, while the second group did not. The sham group also divided into two groups. The first one applied EMF and name as Group CEM, the second one was sham. Weight of removed testes were measured and fixed in 10% formaldehyde for histopathological evaluation. At the creating of undescended testis and right orchiectomies a blood sample was obtained for testosterone level of prepubertal rats. After finishing EMF stimulation the rats were mated with females for 17 days. After fertility study a blood sample was obtained for testosterone assay and body weight were measured and fixed in formaline for histopathologic evaluation. All the rats were killed with overdose ether anesthesia and number of fetuses were recorded. Histopathological evaluation was based on Johnsen criteria and seminiferous tubule diameter measurements. We conclude that EMF stimulation resulted in Leydig cell proliferation, increase in testosterone level, testis weight, but decrease in germ cell population.