Thoraco-abdominal duplication cyst of the jejunum presenting as respiratory distress in a young infant-a case report.

Thoraco-abdominal duplication cysts are rare congenital anomalies that can accompany vertebral and spinal cord pathologies and occur most frequently in the small intestines. Symptoms such as respiratory distress, dyspnea, tachypnea, cough, hemoptysis, cyanosis, vomiting and dysphagia may develop depending on the location. The cyst has several clinical and radiological dilemmas. We present a rare case of thoraco-abdominal duplication cyst in a 3-month-old male patient presenting with respiratory distress in the neonatal period. Thoraco-abdominal duplications require a high index of suspicion and meticulous clinical management. Thus, patients can be successfully managed without any unnecessary interventions, complications and loss of time.

Burn-associated bloodstream infections in pediatric burn patients: Time distribution of etiologic agents.

BACKGROUND: Infections are the leading cause of morbidity and mortality in patients with burns in burn units. Bloodstream infections (BSIs) in patients with burns may result from burn wound infection, use of invasive devices such as central venous catheters, and translocation of the gastrointestinal flora. OBJECTIVE: In this study, we investigated the distribution and antimicrobial drug resistance of causative pathogens in children with burns and the durational changes of microorganisms in the distribution of BSIs in children. METHODS: This study was conducted at the Pediatric Burn Unit (PBU) of Dr. Behçet Uz Children Research and Training Hospital during the period of November 2008-April 2015. The study subjects were all the patients admitted to the PBU, in whom microorganisms were isolated at least from one of the cultures, including blood and catheter cultures. RESULTS: Gram-positive bacteria were the most common causative agents of BSI in patients with burns (66.4%), followed by gram-negative bacteria (22.1%) and fungi (11.5%). The median duration of development of BSIs caused by gram-positive bacteria from the time of burn was 5 days (ranging from 2 to 54 days of burn), which was significantly shorter than that of BSIs caused by gram-negative bacteria (12 days) and fungal pathogens (13 days). CONCLUSION: The etiologic agents of BSIs in children may differ from those in adults. Gram-negative drug-resistant bacteria such as multidrug-resistant Pseudomonas aeruginosa and Acinetobacter baumannii were important agents of BSI in patients with burns, especially in the long term; however, gram-positive bacteria should also be considered while deciding the antimicrobial therapy, especially in the early periods of burn.

[Angiomyxoma: always myxoid, sometimes aggressive]. / Anjiomiksom: Her Zaman Miksoid, Bazen Agresif.

Angiomyxoma is a distinct soft tissue tumor characterized by the presence of prominent myxoid matrix and numerous thin-walled blood vessels. This tumor has a predilection for the trunk, head and neck, extremities, and genitalia. It is a benign tumor and total excision is curative. Recurrence is rare except for aggressive angiomyxomas. A 12-year-old girl with a 10-year history of a subcutaneous mass on the left gluteus measuring 4.5x4x3 cm had been referred. The tumor was encapsulated and was located in the reticular dermis and subcutaneous tissue, composed of stellate cells with mucinous stroma. Thin-walled blood vessels were prominent. Immunohistochemically, tumor cells were immunoreactive for vimentin. No immunoreactivity was present for estrogen receptor, CD34, smooth muscle actin, S-100 protein and desmin. The purpose of this report is to present a classical example of an isolated superficial angiomyxoma and discuss the differential diagnosis, because of its relatively infrequent occurence.

Telomerase reverse transcriptase catalytic subunit expression and proliferation index in Wilms tumor.

Telomerase activity provides telomere maintenance in chromosomes. It prevents cells from entering senescence. Telomerase activity is one of the crucial steps in various cancers. Wilms tumor (nephroblastoma) is one of the most common solid tumors of childhood. Hitherto, telomerase reverse transcriptase (TERT) catalytic subunit expression in Wilms tumor has not been investigated widely. The aim of this study was to explore the expression level of human TERT in Wilms tumor and to correlate with some clinical prognosis factors such as tumor weight, stage, histology, and Ki67 expression. This study included 41 nephroblastoma cases of childhood. The telomerase catalytic subunit expression and proliferation index was determined using an immunohistochemical method on archival paraffin-embedded tissue sections. Statistical analysis was done on SPSS 9.05 by Mann-Whitney U test and Spearman's correlation analysis. TERT expression was negative in 11 cases (26.8%), weakly positive in 14 cases (34.1%), and strongly positive in 16 cases (39%). The proliferation index was found to be 20 to 90 (mean 58.9 ± 26.8). Using Spearman correlation analysis, both the TERT expression (p=0.032) and Ki67 index (p=0.048) were found to be correlated with survival rate. Similarly, both the telomerase expression (p=0.011) and the Ki67 index (0.040) were correlated with the weight and dimension of the tumor. But there was no relationship between telomerase expression and Ki67 index (p=0.429). The mean survival time for telomerase negative cases was 56.6 ± 27.3 months, while it was 34.67 ± 28.36 months for positive cases. The Mann-Whitney U test revealed that levels of telomerase (p=0.040) significantly affected the survival rate. In the present study, we showed that the presence of TERT expression correlated with both tumor size and survival time. These findings suggest that senescence may play an important role in WT evolution, and determination of telomere maintenance will be useful to predict survival and follow-up of patients with Wilms tumor.

Erythropoietin restores bowel damage and hypoperistalsis in gastroschisis.

BACKGROUND AND PURPOSE: Despite the decreased mortality in gastroschisis (Gx), patients experience postoperative intestinal hypoperistalsis, malabsorption, and shortened bowel length. The trophic effects of recombinant human erythropoietin (rEpo) in the developing small bowel have been reported, increasing the length and height of the villi, and villous surface area. This study investigated the effects of rEpo on intestinal malfunction in the chick embryos with Gx. METHODS: Thirteen-day-old fertilized chicken eggs were used to create Gx model. Study groups included the following: group 1, control; group 2, Gx-only; group 3, Gx + 0.075% saline exchange; group 4, Gx + 10 IU rEpo exchange; group 5, Gx + 20 IU rEpo exchange. The bowels were evaluated by in vitro muscle strip technique, and the response was expressed as a percentage of the maximum carbachol-evoked contraction (Emax). In addition, parasympathetic ganglion cells per 10 plexuses and villi height were determined by light microscopy. Results were evaluated statistically by Mann-Whitney U, chi2, and Fisher's Exact test tests. RESULTS: Saline exchange had no effect on ganglion cell number (P = .63) and villi height (P = .10). In group 4, ganglion cell number was not increased (P = .82), but villi height increase was significant (P = .03). In Gx + 20 IU rEpo group, both the number of ganglia (P = .0001) and villi height (P = .002) were significantly increased. The decrease in contractility in group 2 (P = .0121) was significantly reversed by rEpo 20 IU treatment (P = .0216), no significant difference was obtained in groups 3 (P = .0809) and 4 (P = .1516) compared with group 2. CONCLUSION: These data suggest that rEpo has prokinetic effects on hypoperistalsis and restores bowel damage in Gx.

Structural changes of smooth muscle in congenital ureteropelvic junction obstruction.

BACKGROUND/PURPOSE: Ureteropelvic junction (UPJ) obstruction is the most common cause of congenital hydronephrosis. Previous studies have reported that the excess amount of collagen restricting mobility and resiliency of the UPJ is the result of an impaired collagen production by anomalous smooth muscle cells (SMCs). Our purpose was to evaluate the role of SMC differentiation in the pathogenesis of UPJ obstruction. METHODS: Surgical specimens of UPJ from 21 patients (8 girls/13 boys) who were subjected to dismembered pyeloplasty were examined immunohistochemically using monoclonal antibodies against smooth muscle (SM) myosin heavy chain isoforms including SM1, SM2, and SMemb. The age ranged from 1 month to 13 years. Ureteropelvic walls taken from 14 forensic autopsy cases, with no urological abnormalities, served as age-matched control group. RESULTS: The immunohistochemical expression of SM1 and SM2 in UPJ obstruction was significantly increased when compared with controls (P < .05). In contrast, there was no statistical difference of expression of SMemb. CONCLUSION: Our findings supported the hypothesis that the primary anomaly in UPJ obstruction may be attributed to a malfunction of SMCs in the ureter.

Atresia of the colon.

BACKGROUND/PURPOSE: Colonic atresia (CA) is one of the rarest causes of neonatal intestinal obstructions, and no large series can be reported. Therefore, we did perform a retrospective clinical trial to delineate our CA cases and carry out a literature survey. METHODS: We reviewed the charts of CA cases treated in our center between 1992 and 2002. We aimed to collect all reported cases in Medline, and personal communications with the authors of published series were used to reach the missing data. RESULTS: The chart review revealed 9 newborns with CA treated in our center (6 cases of type III, 2 cases of type II, and 1 case of type IV). These accounted for 3.7% of all gastrointestinal atresias managed in our center. Of the CA cases, 3 were isolated and 6 had at least one or more associated congenital anomalies. The preferred surgical technique at the initial treatment of CA was performing a proximal stoma and distal mucous fistula in an average of postnatal 59.4 hours. The literature survey enabled us to reach 224 cases of CA, including our cases. CONCLUSIONS: Because of the low incidence of CA, delay in diagnosis and treatment may occur. The mortality is statistically higher when the surgical management is performed after 72 hours of age. However, the prognosis of CA is satisfactory if diagnosis and surgical management could be made promptly and properly.

The efficacy of non-operative management in childhood blunt hepatic trauma.

BACKGROUND: In this study the results of non-operative management of pediatric hepatic injury after blunt abdominal trauma were evaluated. METHODS: Multitrauma patients (n = 498) admitted between 1998 and 2002 were analysed as for mechanism of trauma retrospectively. Liver injuries were classified according to the American Association for the Surgery of Trauma's Organ Injury Scaling System. Liver function tests, transfusion status, duration of hospital stay, complications, and operative intervention needed were recorded. RESULTS: Seventy-five patients had liver injuries. The mean age was 6.7 years and male/female was 2.5/1. Number of patients in terms of injury grade: I: 15, II: 26, III: 29, IV: 4, V: 1. There was no significant difference between mean blood pressures, heart rates, and hematocrite values on admission and post-stabilisation. In three of 21 transfused patients, it exceeded 40 ml/kg and two of them were operated. The mean pediatric trauma score was +2. As a complication biloma was encountered in one patient. One patient with grade V hepatic injury died in the operating room because of heavy bleeding. The average hospital stay was 8,6 days. CONCLUSION: Our results support the efficacy of non-operative management of any grade hepatic injuries due to blunt abdominal trauma, with resultant low complication and mortality rates and shorter hospital stays.

Associated malformations in delayed presentation of congenital diaphragmatic hernia.

BACKGROUND/PURPOSE: Delayed presentation of congenital diaphragmatic hernia (CDH) has been considered rare, and clinical manifestations differ from the more common newborn entity. Associated malformations in late-appearing CDH have been reported in a few patients. The authors reviewed their clinical experience to catalogue the frequency and clinical importance of associated malformations in patients with late-presenting CDH. METHODS: The records of patients greater than 1 month of age with Bochdalek type CDH treated in the authors' clinic, from 1991 to 2001, were retrospectively reviewed. Twenty patients (age range, 1 month to 10 years) were included in the study. RESULTS: Associated malformations were documented in 16 of the patients (80%) and included malrotation in 12 patients, umbilical hernia in 2, pulmonary hypoplasia in 4, pulmonary sequestration in 1, gastroesophageal duplication cyst coexisting with polysplenia in 1, atrial septal defect and ventricular septal defect in 1, hydronephrosis in 1, wandering spleen in 1, talipes equinovarus in 1, and type I diabetes mellitus in 1. CONCLUSIONS: The results of this study show a significant incidence and a wide spectrum of associated malformations. These findings suggest that patients with late CDH should be evaluated carefully for additional anomalies that may help to establish correct diagnosis and treatment.

Ceacal perforation in a premature newborn infant complicating milk curd syndrome: case report.

A 34-weeks-old female baby having a closed ceacal perforation due to milk curd syndrome is reported. The obstructing milk curd was located in the transvers colon and the perforation was in the ceacum. Primary repair of the ceacal perforation, aspiration of the material from an enterotomy and a protective ileostomy were performed. The patient died in the second postoperative day as a result of cardiopulmonary arrest and septic shock.