Proposal of an algorithm for the management of rectally inserted foreign bodies: a surgical single-center experience with review of the literature.

BACKGROUND: Retained rectal foreign bodies (RFBs) are uncommon clinical findings. Although the management of RFBs is rarely reported in the literature, clinicians regularly face this issue. To date, there is no standardized management of RFBs. The aim of the present study was to evaluate our own data and subsequently develop a treatment algorithm. METHODS: All consecutive patients who presented between January 2006 and December 2019 with rectally inserted RFBs at the emergency department of the Klinikum Stuttgart, Germany, were retrospectively identified. Clinicopathologic features, management, complications, and outcomes were assessed. Based on this experience, a treatment algorithm was developed. RESULTS: A total of 69 presentations with rectally inserted RFBs were documented in 57 patients. In 23/69 cases (33.3%), the RFB was removed transanally by the emergency physician either digitally (n = 14) or with the help of a rigid rectoscope (n = 8) or a colonoscope (n = 1). In 46/69 cases (66.7%), the RFB was removed in the operation theater under general anesthesia with muscle relaxation. Among these, 11/46 patients (23.9%) underwent abdominal surgery, either for manual extraction of the RFB (n = 9) or to exclude a bowel perforation (n = 2). Surgical complications occurred in 3/11 patients. One patient with rectal perforation developed pelvic sepsis and underwent abdominoperineal extirpation in the further clinical course. CONCLUSION: The management of RFBs can be challenging and includes a wide range of options from removal without further intervention to abdominoperineal extirpation in cases of pelvic sepsis. Whenever possible, RFBs should obligatorily be managed in specialized colorectal centers following a clear treatment algorithm.

Low-grade fibromyxoid sarcoma: A report of the Cooperative Weichteilsarkom Studiengruppe (CWS).

BACKGROUND: Low-grade fibromyxoid sarcoma (LGFMS) is a rare soft-tissue tumor with benign histologic appearance, though fully malignant behavior is possible. METHODS: Patients with LGFMS <21 years registered in Cooperative Weichteilsarkom Studiengruppe trials until 2017 were analyzed. Firstline treatment consisted of complete surgical resection whenever possible. RESULTS: Median age of 31 patients was 10.9 years (first month to 17.1 years). Twenty-six tumors were confirmed to the tissue of origin (T1), four invaded contiguous structures (T2), one was TX. Eight were >5 cm. The best surgical result was resection with free margins (R0) in 24 and microscopic residuals (R1) in seven. Five-year event-free (EFS), 5-year local-relapse-free (LRFS), and 5-year overall-survival were 71 ± 18.6% confidence interval (CI) 95%, 76 ± 17.6% CI 95%, and 100%, respectively. Six patients suffered local relapse in a median of 1 year, one combined within 1.3 year and one metastatic relapse with lesions in the lung, back muscles, and thigh discovered in whole-body imaging 6 years after the first diagnosis. In univariate analysis, T status correlated with EFS (T1 79.6 ± 18.6%, T2 50.0 ± 49.0%, P = .038). Resection with free margins tends to be associated with better LRFS (R0 82.4 ± 18.6%, R1 53.6 ± 39.4%, P = .053). Among 24 patients with R0 resection, five (21%) suffered relapse, thereof three local, one metastatic, and one combined. Among seven patients with R1-resection, three (43%) suffered local relapse. CONCLUSION: Special caution is advisable in T2 tumors. The metastatic potential with lesions in unusual sites indicates that affected patients need to be informed. If long-term follow-up with whole-body imaging is beneficial, it may be addressed in larger intergroup analyses. Further research in disease biology is essential for optimal treatment and follow-up care.

Localized synovial sarcoma of the foot or ankle: A series of 32 Cooperative Weichteilsarkom Study Group patients.

BACKGROUND: Synovial sarcoma of the foot/ankle is rare. Mutilating surgery is often discussed. METHODS: Patients registered from 1981 to 2013 were analyzed. Cooperative Weichteilsarkom Studiengruppe (CWS) protocols recommend chemotherapy for all synovial sarcoma patients. RESULTS: Thirty-two of 330 patients with localized synovial sarcoma had their tumor at the foot/ankle. Eleven of thirty-two tumors were >5 cm. Twenty were T1, 11 T2, and one TX, respectively. Eight (25%) patients underwent primary complete resection with free margins (Intergroup Rhabdomyosarcoma Study [IRS] I), 12 of 32 (38%) primary complete resection with positive margins (IRS II), and 12 of 32 (38%) had macroscopic residuals (IRS III). The best surgical result at any time was R0 in 19, R1 in 10 and R2 in one patient, and missing in two. Mutilation was documented in 14 of 32 (44%). Radiotherapy was conducted in 20 patients. All patients achieved a first complete remission. Five-year-event-free survival and overall survival rates were 80% and 86%, respectively. Four patients suffered local and four other metastatic recurrences. IRS and the best surgical result at any time did not correlate with survival. There was no prognostic difference between R0- and R1-resection. CONCLUSION: Survival expectancies for patients with localized synovial sarcomas of the foot/ankle compare favorably to that of those with other affected sites. DISCUSSION: Further studies are needed to set the limits of minimally required aggressiveness of local therapies.

Di (2-Ethylhexyl) Phthalate and Its Role in Developing Cholestasis: An In Vitro Study on Different Liver Cell Types.

OBJECTIVES: Di(2-ethylhexyl) phthalate (DEHP) is a plasticizer used in many polyvinylchloride medical devices and is washed out easily. Thereby critically ill infants can become exposed to DEHP concentrations significantly exceeding the recommended threshold. We suspect DEHP to play an important role in the development of intestinal failure-associated liver disease. The aim of this study was therefore to determine the direct influence of DEHP on different liver cell types. METHODS: HepG2, human upcyte hepatocytes, primary murine hepatocytes, LX-2, human upcyte hepatic stellate cells, and liver organoids were cultured with DEHP (0.5-500 µmol/L) and parameters including cytotoxicity, cell-cell interactions, and expression of metabolizing enzymes were investigated. RESULTS: DEHP modulated the expression of xenobiotic metabolizing enzymes, reduced the formation of bile canaliculi and cell polarity, and inhibited Cyp-activity in hepatocytes. DEHP had a toxic effect on LX-2 and induced the fibrogenic activation of hepatic stellate cells. The mode of action of DEHP was different in monolayer cultures compared to 3D-liver organoids, which were more sensitive to DEHP. CONCLUSIONS: This study suggests that DEHP modulates expression and activity of drug-detoxifying liver enzymes in humans at a clinically relevant concentration. Furthermore, it may contribute to the development of cholestasis and fibrosis. These findings strongly support the opinion, that there is a significant potential for serious adverse effects of DEHP derived from medical devices on human health, especially in very young infants with immature livers.

The Use of Fish Skin Grafts in Children as a New Treatment of Deep Dermal Burns-Case Series with Follow-Up after 2 Years and Measurement of Elasticity as an Objective Scar Evaluation.

Background: Wound healing in deep dermal burn injuries continues to be a challenge in paediatrics. In the absence quick and spontaneous wound closure, split-thickness skin grafting is often necessary. Since the development of a new type of acellular fish matrix, which is very similar to the human skin matrix, skin closure and wound conditioning can be achieved without split-thickness skin grafting. Methods: The following study shows a case series of 20 children in whom a fish skin graft was used. The aim was to develop an algorithm for selecting and using fish skin and its long-term results after one and two years. Acellular fish skin worked as a granulation base for wound healing and also as a substitute for split-thickness skin grafts. Results: There was no evidence of infection. Skin transplants and, thus, additional operations could be avoided. The follow-up examinations showed an excellent result, both objectively by means of elasticity measurements (DermalabCombo®) and in the subjective assessment of the skin as part of the Patient and Observer Scar Assessment Scale (POSAS). Conclusion: Fish skin grafts are a good alternative to split-thickness skin grafts for deep dermal wounds. These results should be further analysed with a larger number of patients in future publications.

Recurrent Sigmoid Volvulus in Children-Our Experience and Systematic Review of the Current Literature.

Sigmoid volvulus (SV) occurs rarely in children. After encountering two cases of recurrent SV, we reviewed the literature to define the recurrence risk, identify outcome predictors and to give treatment recommendations. We found 256 cases reported in children (mean age 10.2 years, gender ratio (m:f) 2.3:1). Associations exist with Hirschsprung disease (HD) in 10%, neurodevelopmental disorders in 10.9% and chronic constipation in 10.2%. Common symptoms and clinical signs were abdominal pain (85%), distension (85%), tenderness (54%) and vomiting (59%). Signs of peritonitis were present in 14% and indicated a gangrenous sigmoid (X2 = 45.33; p < 0.001). A total of 183 had abdominal radiographs, and 65% showed a positive 'coffee-bean-sign'. Contrast enemas were positive in 90%. A total of 124 patients underwent laparotomy; in 41 cases, the sigmoid was gangrenous and associated with more complications (X2 = 15.68; p < 0.001). Non-operative treatment (NOT) like endoscopic, fluoroscopic or rectal tube decompression was performed in 135 patients and successful in 79% with a 38-57% recurrence rate. A total of 73 patients subjected to elective surgery: 50 underwent sigmoid resection; 17 had surgery for HD. Clinicians should consider SV in all children with abdominal pain, distension and vomiting. Gangrene carries a higher morbidity. After successful NOT we recommend counselling about the recurrence risk and definitive surgery should be advised. HD is frequent in newborns but sometimes found in older children.

A Single-Institution Experience About 10 Years With Children Undergoing Fractional Ablative Carbon Dioxide Laser Treatment After Burns: Measurement of Air Pressure-Induced Skin Elevation and Retraction Time (Dermalab) Including Standardized Subjective and Objective Scar Evaluation.

Laser treatment has gained more and more importance in the therapy of scars during the last years. Scientific work, especially in children, is scarce on this topic and mostly with an insufficient number of cases or only subjective descriptions. Our study included 77 children from 2012 to 2022 with scars after thermal injury. These were treated at least three times or more by CO2 laser or in combination with pulsed dyed laser (PDL). Beforehand, scar texture and elasticity were determined by a skin elasticity analysis system. Regarding the subjective evaluation, a questionnaire was given to the patients or their parents. Further criteria were the Patient and Observer Scar Assessment Scale (POSAS) and Vancouver Scar Scale (VSS). A statistically significant improvement in elasticity was demonstrated in all scars of any age after each laser treatment. In addition, a significant correlation was found between the number of laser treatments and an increase in elasticity. The assessments of scars after one or more laser sessions by the observer as well as the patient showed a decreasing score in all categories with an increase in the number of laser therapies. The VSS score also improved significantly after each laser session. The mean score before treatment was about 7, after the first laser session, the mean score was already below 6 with a statistical significance. Ninety-six percent of the patients or their parents were satisfied with the laser therapy, and 90% wished to repeat the procedure. This work confirms, by means of the objectification by the scar measurement, the previous scientific works concerning a scar therapy by laser and emphasizes particularly that this can also be used without problems with children with symptomatic scars, contractures, or with cosmetically relevant scars.

A multi-step approach to the treatment of giant scalp congenital hemangiomas: a report of two cases.

This is a review of two cases of neonatal giant scalp congenital hemangioma. Both patients were treated with propranolol using a similar multi-step approach that included transarterial embolization of the supplying arteries followed by surgical resection of the lesion. In this report, we discuss the treatments, complications, and clinical outcomes of interventions and surgical procedures.

Esophageal Diameter as a Function of Weight in Neonates, Children and Adolescents: Reference Values for Dilatation of Esophageal Stenoses.

Introduction: Esophageal stenoses are frequent complications after esophageal atresia surgery as well as after acid, alkali and battery ingestion. Worldwide, repeated balloon dilatations are the most frequently performed procedures for these stenoses. In most cases the stenoses can be dilated sufficiently to allow adequate enteral nutrition. Until recently, age dependent esophageal lumen size has not been established; which was aim of the current study. Methods: All children in whom an esophageal contrast imaging was performed between 1/2011 and 5/2021 were included. The width was measured by two investigators at two measuring points in two planes, the diameter was calculated and plotted against the respective weight of the child. Bland-Altmann plots have confirmed the validity of the measurements of both investigators. Results: Esophagus diameter was measured in more than 100 children. The resulting curves show a very good correlation with weight (upper measuring point: r = 0.86743, p < 0.0001; lower measuring point: r = 0.80593, p < 0.0001). Conclusion: These results are the first to define the esophageal diameter in children. The results of this study may guide physicians performing esophageal interventions including dilatations in future.

The effect of adjuvant therapies on long-term outcome for primary resected synovial sarcoma in a series of mainly children and adolescents.

BACKGROUND: The benefit of adjuvant therapy in synovial sarcoma (SS) treatment is under debate. Long-term follow-up data are missing. METHODS: SS patients treated in the consecutive trials CWS-81, CWS-86, CWS-91, CWS-96, CWS-2002-P, and the SoTiSaR-registry till 2013 were analyzed. RESULTS: Median age of 185 patients was 13.9 years (0.1-56)-with median follow-up of 7.4 years for 163 survivors. Most tumors (76%) were located in extremities. Size was < 3 cm in 58 (31%), 3-5 cm in 59 (32%), 5-10 cm in 42 (23%), and > 10 cm in 13 (7%) (13 missing). In 84 (45%) tumors, first excision was complete (R0 corresponding to IRS-I-group) and in 101 (55%) marginal (R1 corresponding to IRS-II-group). In a subsequent surgical intervention during chemotherapy, R0-status was accomplished in 23 additional IRS-II-group patients with secondary surgery. Radiotherapy was administered to 135 (73%), thereof 62 with R0-status and 67 R1-status (6 missing information). Adjuvant chemotherapy was administered to all but six patients. 5-year event-free (EFS) and overall survival (OS) was 82.9% ± 5.7 (95%CI) and 92.5% ± 3.9. Local and metastatic relapse-free survival was 91.3% ± 4.3 and 92.3% ± 4.1 at 5 years, respectively. In the multivariate analysis, tumor size and no chemotherapy were independently associated with EFS. Size and site were associated with OS. In a detailed analysis of local and metastatic events, tumor size was associated with an independent risk for developing metastases. No independent factor for suffering local recurrence could be identified. DISCUSSION: Omission of chemotherapy in a non-stratified way seems not justified. Size governs survival due to high linear association with risk of suffering metastatic recurrence in a granular classification.

Esophageal Diameters in Children Correlated to Body Weight.

INTRODUCTION: The development of stenoses after correction of an esophageal atresia or acid and lye burn of the esophagus are well-known problems in pediatric surgery. Currently, stenoses are treated in the majority of cases by repeated balloon dilatations. The diameter of the balloons used is not standardized; standard curves do not exist. The aim of this study was to evaluate the diameter of the esophagus correlated to the body weight of the children as measured in upper gastrointestinal (GI) studies to answer the important question to what extent a stenosis should be dilated. MATERIALS AND METHODS: Within the time period from 2011 through 2016, 60 patients with upper GI studies were selected. Evaluations were blinded to two different examiners. The diameters were measured under maximum contrast filling between the second and third rib (cranial point of measurement) and between the seventh and eighth rib (caudal point of measurement). For both, the anteroposterior and lateral aspect was examined. The diameter was calculated as the arithmetic average of both measurements within one level. The diameters were correlated to the weight of the children. RESULTS: All children (n = 38) within the 3rd to 97th weight percentile were analyzed. Linear correlation and coefficients of 0.67 at the cranial point and 0.70 at the caudal point were found. Mean diameter at the cranial point of measurement was 6.75 mm at the lowest weight (2.6 kg) and 14 mm at 74 kg. Mean weight of these children (standard deviation [SD]) was 25.3 (18.8) kg and median age was 7 years. Within weight groups (0-10 kg; 10-20 kg; 20-35 kg; 35-50 kg; >50 kg), we calculated SD and two side tested critical 95% confidence interval for all measurements (n = 74). CONCLUSION: Although the variation in measurements is considerable, this evaluation gives a reliable hint to which extent esophageal stenoses should be dilated in relation to the body weight. To the best of our knowledge, this is the first investigation to evaluate the diameter of the esophagus in children in relation to the body weight.

Extraction of diethylhexylphthalate by home total parenteral nutrition from polyvinyl chloride infusion lines commonly used in the home.

OBJECTIVES: Recently, our group detected that polyvinyl chloride (PVC) perfusion lines leach large amounts of the toxic plasticizer diethylhexylphthalate (DEHP) under conditions typical of intensive care units. In the present study, we investigated the extraction of DEHP from PVC connecting tubes that are commonly used for total parenteral nutrition (TPN) solutions. The aim of the study was to estimate the amount of DEHP to which children receiving home TPN are exposed for months and years. MATERIALS AND METHODS: 1000 mL of TPN, identical in constitution and amount to the home TPN of 1 of our patients, were perfused through 5 different connecting tube systems and collected in hexane-rinsed glass bottles. The concentration of DEHP in the TPN was analyzed before and after perfusion. RESULTS: Before perfusion of the lines, the solution had a DEHP concentration of 0.05 to 0.69 microg/mL (baseline value). After perfusion of the lines, the load of DEHP in the solution varied between 1.41 and 2.07 microg/mL. This TPN was established for children weighing 20 kg. The daily dosage is between 71 and 104 microg x kg(-1) x day(-1). TPN is administered at home for many months and years. The monthly charge of DEHP is between 42.3 and 62.1 mg. Children weighing 20 kg therefore receive a dosage between 2.1 and 3.1 mg x kg(-1) x month(-1). CONCLUSIONS: Diethylhexylphthalate and its metabolite monoethylhexylphthalate have been demonstrated to be carcinogenic, embryotoxic, hepatotoxic, pneumotoxic, and cardiotoxic and are known to disrupt endocrine pathways and liver detoxifying capacity in animals. They are suspected of having multiple effects in humans as well. The doses presented above should therefore be avoided in children receiving home TPN by the use of tubing systems that are completely free of DEHP. Such systems are available.

Congenital diaphragmatic hernia: a modern day approach.

Centralization of all complicated congenital diaphragmatic hernias (CDH) was organized in Germany from 1998, collecting 325 consecutive patients with striking increasing survival rates. This series report 244 patients from 2002 to 2007. Today, large defects are detected early in pregnancy by ultrasound and magnetic resonance imaging (MRI). In extracorporeal membrane oxygenation (ECMO) patients, prenatal lung head ratio (LHR) was 1.2 (median) at the 34th week of gestation or less than 25 ml lung tissue in MRI. This means that all patients below LHR of 1.4 should be transferred prenatally in a tertiary center. High risk group for survival was defined as LHR below 0.9, ie, 10 ml in MRI planimetry. Inborn patients show better results than outborns. In algorithm therapy, gentle ventilation plays an important role in preventing damage to the lung tissue and avoiding long term ventilation. When PaCO(2) was more than 75 mmHg, ventilation was changed to high frequency oscillatory ventilation (HFOV). Indication for ECMO was seen in preductal PaO(2) less than 50 mmHg over 2-4 h or less than 40 mmHg over 2 h. ECMO related risks included intracerebral bleeding (9%), intrapulmonary bleeding (14%), and convulsions (16%). Surgically, a longitudinal midline incision for exposure of the defect, the duodenal kinking, and probably for abdominal patching was perfect. A cone formed goretex patch provided more abdominal space and reduced abundant intrathoracical cavity. No drain was used. Postoperative complications were described. Overall survival in 244 consecutive patients was 86.5% for all patients born alive. All those who needed ECMO survived in 71%, underlining ECMO as a treatment of last choice. Follow-up for quality of life after CDH is described.

Comparison of two silver dressings for wound management in pediatric burns.

UNLABELLED: Purpose. Silver wound dressings are widely used in the treatment of burns. Dressings differ in material characteristics, various antimicrobial activities, and ease of use. The purpose of this study was to evaluate both dressing performance and amount of pain during the dressing changes of 2 silver dressings Urgotul SSD® (Laboratoires Urgo, Chenove, France), and Contreet Ag® (Coloplast, Minneapolis, MN) in children. METHODS: A retrospective cohort study was performed with 2 groups of 20 burns treated with Urgotul SSD and Contreet Ag until the wounds were healed or grafted. Seventy dressing changes in the Contreet Ag group and 67 dressing changes in the Urgotul group were evaluated. Every dressing change was assessed regarding the dressing performance (exudate, adherence, bleeding, and dressing application/removal), and pain. RESULTS: Pain was "absent or slight" in 61 (92%) dressing changes with Urgotul SSD, and in 60 (85%) of the dressing changes with Contreet Ag. Dressing application in the Urgotul group was more often "very easy" (n = 33; 49%) or "easy" (n = 32; 48%) than in the Contreet Ag group, "very easy" (n = 25; 35%), and "easy" (n = 42; 60%). Contreet Ag had a greater ability to absorb exudate ("very good" n = 60; 85%, and "good" n = 11; 15%) than Urgotul SSD ("very good" n = 34; 51%, and "good" n = 13; 19%). CONCLUSION: Urgotul SSD and Contreet Ag are comparable regarding pain during dressing change. The dressings differ in their ability to absorb exudate and ease of application. Both dressings provided nearly painless wound management, and therefore were highly accepted by the nurses and especially the children being treated.

Guideline implementation for the treatment of undescended testes: Still room for improvement.

BACKGROUND: Early orchidopexy (OP) around the age of 1 year is recommended in boys with congenital undescended testis (UDT) worldwide since decades. Former retrospectives studies did not distinguish congenital from acquired UDT with a consecutive negative bias concerning the age at surgery. METHODS: In a retrospective analysis, data of all boys who underwent OP in eight pediatric surgery institutions from 2009 to 2015 were analyzed. Congenital or acquired UDT were differentiated. Patients were categorized into 3 groups of age at surgery: (1) <12 months, (2) 12-24 months, (3) >24 months. Data of one institution were analyzed in detail: exact age of first referral, exact age at surgery, intraoperative findings. RESULTS: Out of 4448 boys, 3270 boys had congenital UDT. In 81% (2656 cases) surgery was performed beyond the age of 1 year, in 54.4% (1780) beyond the age of 2 years. chi-Square statistics showed a higher rate of early operations in hospitals compared to outpatient services and in Germany compared to Switzerland. In 694 congenital detailed cases, median age at referral was 13 months [range 0-196], median age at surgery was 15 months [range 0-202]. CONCLUSION: Delayed referral is the main reason for guideline non-conform delayed surgery in UDT. TYPE OF STUDY: Clinical Research paper. LEVEL OF EVIDENCE: Level III: Treatment Study.

Extracorporeal membrane oxygenation in infants with congenital diaphragmatic hernia: follow-up MRI evaluating carotid artery reocclusion and neurologic outcome.

OBJECTIVE: The purpose of our study was to prospectively assess, using MRI and MR angiography, the cerebral and vascular status of 2-year-old children with congenital diaphragmatic hernia (CDH) in whom carotid artery reconstruction was performed after neonatal extra-corporeal membrane oxygenation (ECMO) therapy and to compare the neurologic development of children with vascular reocclusion with that of CDH children with successful repair and with non-ECMO controls. SUBJECTS AND METHODS: A total of 30 infants (17 boys, 13 girls; 2 +/- 0.26 years) were included. Of these, 18 (60%) infants received arteriovenous ECMO therapy with subsequent reconstruction of the right common carotid artery (RCCA). Two years postoperatively, the children were examined with cerebral MRI, including 3D time-of-flight and contrast-enhanced 3D MR angiography of the intra- and extracranial brain-supplying arteries. The pathologic findings were analyzed for the ability to predict impaired neurologic development. RESULTS: The RCCA was occluded or highly stenotic in 13 (72%) of 18 children. All infants showed intra- and extracranial collaterals and a patent internal carotid artery. The average duration of ECMO was not longer than in cases of successful reconstruction (p = 1). The ECMO group showed a significantly greater incidence of cerebral injuries (p = 0.007) but no relevant impairment in neurologic development compared with controls (p = 0.26). Unsuccessful RCCA repair had no predictive value for a poor neurologic outcome (p = 1). CONCLUSION: The outcome of RCCA repair after ECMO is possibly poorer than expected, with vascular occlusion or high-grade stenosis occurring in almost three quarters of patients. Although reocclusion of the RCCA does not increase the risk for cerebral lesions or an impaired neurologic development during the first 2 years postoperatively, the overall benefit of RCCA repair remains doubtful, and the potential long-term risk arising from these plaques has yet to be assessed.

Extraction of the plasticizers diethylhexylphthalate and polyadipate from polyvinylchloride nasogastric tubes through gastric juice and feeding solution.

OBJECTIVES: Except for polyadipate, which is used as an alternative in polyvinylchloride (PVC) feeding tubes, diethylhexylphthalate (DEHP) is the plasticizer used almost exclusively in PVC medical products. A clear response to the chronic intake of DEHP has been shown in several organ systems from different species. In the present study, we compared the extraction of DEHP and polyadipate from PVC gastric tubes. METHODS: An in vivo setting was simulated. We used 5 cm of PVC nasogastric tubes containing DEHP or polyadipate, which were incubated with gastric juice for 1 week and a feeding solution for 4 weeks. The leakage of these plasticizers was measured daily in the gastric juice group and weekly in the feeding solution group. The amount of plasticizer extracted was compared between groups and days. RESULTS: In the feeding solution group, the extraction of DEHP ranged from 200 to 542 microg after 1 week and from 660 to 1700 microg after 4 weeks. The extraction of polyadipate was 10 times lower than that of DEHP. In the gastric juice group, extracted DEHP ranged from 635 to 1043 microg, whereas the extraction of polyadipate was 100 times lower. CONCLUSIONS: Within 1 week, the extraction of DEHP from a 5-cm PVC tube reaches up to 1 mg. Extrapolated, this represents an in vivo load of up to 4 mg. The load accumulated by a newborn in an intensive care unit can therefore easily reach several milligrams of DEHP per day. Polyadipate nasogastric tubes may therefore be an alternative and help to reduce the daily load of DEHP.

Influence of different intravenous lipid emulsions on hepatobiliary dysfunction in a rabbit model.

OBJECTIVES: Long-term total parenteral nutrition (TPN) in children is often complicated by the development of cholestasis, liver fibrosis, and liver failure. High doses of intravenous lipids may be involved in the pathogenesis of hepatobiliary dysfunction. The purpose of this study was to determine whether the use of 2 newly developed lipid emulsions could reduce liver damage. MATERIALS AND METHODS: Three groups of prepubescent rabbits received TPN including a lipid emulsion either based on soybean oil, olive oil, or soybean oil with n-3 fatty acids added. Enterally fed animals served as controls. After 21 d the animals were killed. Serum samples were obtained at the beginning and end of the study period. Specimens were processed for histological evaluation using a specific score to assess the severity of liver damage. RESULTS: Biochemical parameters did not predict the extent of liver damage. Hydropic degeneration as an indicator of toxic liver injury was the predominant histological alteration regardless of the type of lipids infused. The extent of fibrosis did not significantly differ among treatment groups except for animals infused with n-3 fatty acids exhibiting increased fibrotic transformation as compared with controls. CONCLUSION: In our animal model, the use of a lipid emulsion with a reduced amount of polyunsaturated fatty acids was not superior to a lipid emulsion based on soybean oil. Long-term application of n-3 fatty acids was associated with more extensive fibrosis. Therefore, intravenous n-3 fatty acids containing lipid preparations (fish oil) should not be used in patients for long-term TPN.

High proportion of large genomic STK11 deletions in Peutz-Jeghers syndrome.

Germline mutations in the STK11 gene have been identified in 10-70% of patients with Peutz-Jeghers syndrome (PJS), an autosomal-dominant hamartomatous polyposis syndrome. A second locus was assumed in a large proportion of PJS patients. To date, STK11 alterations comprise mainly point mutations; only a small number of large deletions have been reported. We performed a mutation analysis for the STK11 gene in 71 patients. Of these, 56 met the clinical criteria for PJS and 12 were presumed to have PJS because of mucocutaneous pigmentation only or bowel problems due to isolated PJS polyps. No clinical information was available for the remaining three patients. By direct sequencing of the coding region of the STK11 gene, we identified point mutations in 37 of 71 patients (52%). We examined the remaining 34 patients by means of the multiplex ligation-dependent probe amplification (MLPA) method, and detected deletions in 17 patients. In four patients the deletion extended over all 10 exons, and in eight patients only the promoter region and exon 1 were deleted. The remaining deletions encompassed exons 2-10 (in two patients), exons 2-3, exons 4-5, or exon 8. When only patients who met the clinical criteria for PJS are considered, the overall mutation detection rate increases to 94% (64% point mutations and 30% large deletions). No mutation was identified in any of the 12 presumed cases. In conclusion, we found that approximately one-third of the patients who met the clinical PJS criteria exhibited large genomic deletions that were readily detectable by MLPA. Screening for point mutations and large deletions by direct sequencing or MLPA, respectively, increased the mutation detection rate in the STK11 gene up to 94%. There may be still other mutations in the STK11 gene that are not detectable by the methods applied here. Therefore, it is questionable whether a second PJS locus exists at all.