Real vs simulated umbilical cords for emergency umbilical catheterization training: a randomized crossover study.

OBJECTIVE: To measure performance, fidelity and preference of two emergency umbilical vessel catheter (eUVC) simulation models. STUDY DESIGN: A randomized crossover trial of senior pediatric residents randomized to place an eUVC first using a real cord (RC) or simulated cord (SC), and then place an eUVC using the other model. The eUVC placement times were recorded and analyzed. Subjects rated physical and functional fidelity and preference for each model. RESULTS: The eUVC placement time (mean±s.d. s) was slower in RC vs SC (153 s ±71 vs 88 s ±35, P<0.001), however, there was no difference in eUVC placement time in the group that worked with SC first (115 s ±36 vs 97 s ±35, P=0.161). Physical and functional fidelity of RC were rated higher than SC (P<0.001), and RC were preferred. CONCLUSION: RC has higher physical and functional fidelity, and are preferred for training by pediatric residents, despite longer placement times.

Establishing the Feasibility of Face Transplantation in Granulomatosis With Polyangiitis.

Granulomatosis with polyangiitis (GPA; formerly Wegener's granulomatosis) is a rare vasculitis that commonly starts in the craniofacial region. We report a case that was masked by prior facial trauma and associated with pyoderma gangrenosum (PG). Disease progression and aggressive debridements led to severe facial tissue loss. The decision to perform a face transplant was controversial because of the risk of disease relapse on the facial allograft. We reviewed renal transplant outcomes in GPA for possible relevance. A PubMed search retrieved 29 studies. Patient and graft survival, relapse, morbidity, mortality, rejection and immunosuppression were assessed. Ten-year patient survival and graft survival were 84.4% and 72.6%, respectively. GPA relapse occurred in 31.5%, and upper airway/ocular relapse occurred in 17.8% (resolved in 76.9%). Mortality was 12.3%. Acute and chronic rejection rates were 14.9% and 6.8%, respectively. Traditional posttransplant immunosuppression was effective. Our review suggests that GPA renal transplant outcomes are comparable to general renal transplant cohorts. Furthermore, transplanted GPA patients exhibit lower disease relapse secondary to lifelong immunosuppression. This supported our decision to perform a face transplant in this patient, which has been successful up to the present time (1-year posttransplantation). Untreated GPA and PG are potential causes of worse surgical outcomes in the craniofacial region.

Uterine papillary serous and clear cell carcinomas predict for poorer survival compared to grade 3 endometrioid corpus cancers.

To compare the survival of women with uterine papillary serous carcinoma (UPSC) and clear cell carcinoma (CC) to those with grade 3 endometrioid uterine carcinoma (G3EC). Demographic, pathologic, treatment, and survival information were obtained from the Surveillance, Epidemiology, and End Results Program from 1988 to 2001. Data were analysed using Kaplan-Meier and Cox proportional hazards regression methods. Of 4180 women, 1473 had UPSC, 391 had CC, and 2316 had G3EC cancers. Uterine papillary serous carcinoma and CC patients were older (median age: 70 years and 68 vs 66 years, respectively; P<0.0001) and more likely to be black compared to G3EC (15 and 12% vs 7%; P<0.0001). A higher proportion of UPSC and CC patients had stage III-IV disease compared to G3EC patients (52 and 36% vs 29%; P<0.0001). Uterine papillary serous carcinoma, CC and G3EC patients represent 10, 3, and 15% of endometrial cancers but account for 39, 8, and 27% of cancer deaths, respectively. The 5-year disease-specific survivals for women with UPSC, CC and G3EC were 55, 68, and 77%, respectively (P<0.0001). The survival differences between UPSC, CC and G3EC persist after controlling for stage I-II (74, 82, and 86%; P<0.0001) and stage III-IV disease (33, 40, and 54; P<0.0001). On multivariate analysis, more favourable histology (G3EC), younger age, and earlier stage were independent predictors of improved survival. Women with UPSC and CC of the uterus have a significantly poorer prognosis compared to those with G3EC. These findings should be considered in the counselling, treating and designing of future trials for these high-risk patients.

Experimental model of pyridoxine (B6) deficiency-induced neuropathy.

A pyridoxine (B6) dietary deficiency was studied in female adult Sprague-Dawley rats by hind-limb walking-track analysis. Serum levels of pyridoxine and three metabolites were quantified by high-pressure liquid chromatography with fluorescence measurement. Morphometric analysis of the sciatic and posterior tibial nerves (from within the tarsal tunnel) was performed after 1 year on a diet deficient in vitamin B6. The B6-deficient rats developed abnormal walking-track patterns by 8 months, and these track parameters were different from age- and sex-matched normal diet control rats at the p < 0.05 level. Adding B6 at 10 parts per million to the diet then partially corrected these parameters, whereas the addition of 30 parts per million B6 corrected the abnormal pattern completely. Serum pyridoxal concentration correlated with the functional parameters, dropping from a mean of 115 mg per liter to 39.5 mg per liter (p < 0.05), and correcting with the B6 additive. Morphometric analysis demonstrated that the B6-deficient nerve from the tarsal tunnel had a decreased nerve fiber density (p < 0.001), with a normal total myelinated nerve fiber number, and an increased axon-to-myelin ratio (p < 0.003). It is concluded that a diet totally deficient in vitamin B6 results in a peripheral neuropathy.

Characterization and management of paraesophageal hernias in children after antireflux operation.

PURPOSE: The aim of this study was to determine the important factors in the development and subsequent treatment of postoperative paraesophageal hernia (PPEH). METHODS: A retrospective analysis was performed in 464 consecutive children (ages 3 days to 18 years) for PPEH after a primary antireflux operation performed at a Children's Hospital and University Hospital between 1985 and 1997. All operations included a crural repair, but the Nissen fundoplication was performed with (n = 162) and without (n = 70) plication of the esophagus to the crus at 3 points. Patients with and without PPEH were compared with respect to the type of antireflux operation, the patient's age at operation, and the preoperative and postoperative clinical courses. A preoperative corrected gastric emptying value was obtained from a radionuclide gastric emptying study in 289 patients. The treatment of PPEH also was examined. RESULTS: The incidence of PPEH in our patients was 4.5% (21 of 464). Although there was a lower incidence of PPEH in patients with crural plication compared with patients without crural plication during Nissen fundoplication (5 of 162, 3% v 7 of 70, 10%; P = .035), 2 patients with crural plication had a postoperative esophageal leak. Patients with PPEH had a significantly increased prevalence of gagging before the initial antireflux operation compared with patients without PPEH (3 of 21, 14.3% v 7 of 443, 1.6%; P = .007). A higher prevalence of slow corrected gastric emptying preoperatively also was seen in patients with PPEH compared with patients without PPEH (8 of 15, 53% v 79 of 274, 29%; P = .046). The prevalences of central nervous system disease, young age (<6 months) at initial operation, and a particular type of antireflux operation were not higher in patients with PPEH. Nine patients with a small PPEH treated by simple observation alone subsequently had resolution of symptoms. CONCLUSIONS: Patients who have gagging or slow corrected gastric emptying before an antireflux operation are at higher risk for a postoperative paraesophageal hernia. Patients with a small postoperative paraesophageal hernia can be treated nonoperatively. Crural plication of the esophagus during Nissen fundoplication reduces the occurrence of postoperative paraesophageal hernia, but also may result in significant morbidity.

Esophageal pH monitoring abnormalities and gastroesophageal reflux disease in infants with intestinal malrotation.

HYPOTHESIS: Infants with rotational abnormalities of the midgut mesentery are at high risk for gastroesophageal reflux disease (GERD) and for sudden infant death (SID) from GERD. DESIGN: A survey of the prevalence of GERD and the risk factor for SID from GERD in a case series of infants treated for congenital anomalies that include intestinal malrotation. Eighty-one (89%) of the infants studied for GERD had a mean follow-up of 23.2 months (median, 12 months). SETTING: Patients treated in 2 tertiary care centers consisting of a children's hospital and a university medical center. PATIENTS: Two hundred eighty-six consecutive infants were treated for congenital anomalies from September 1, 1985, through May 31, 1998. The patients selected for study were 91 infants who had 18- to 24-hour esophageal pH monitoring performed and no prior operation on the stomach or esophagus. The studied infants had intestinal malrotation either alone (n = 55) or associated with a repaired abdominal wall defect (n = 23) or congenital diaphragmatic hernia (n = 13). Of the 91 infants, 34 were asymptomatic at the time of esophageal pH monitoring. INTERVENTIONS: Eighteen- to 24-hour esophageal pH monitoring was used to determine the presence of GERD (abnormal pH score >2 hours postcibal) and the risk factor for SID from GERD (type I or III reflux pattern in combination with a prolonged mean duration of sleep reflux). MAIN OUTCOME MEASURES: The prevalence of GERD and the risk factor for SID from GERD. The follow-up of GERD was reported as a combination of clinical outcome and subsequent extended esophageal pH monitoring. RESULTS: Of the 91 infants studied, 80 (88%) had GERD and 26 (29%) had the risk factor for SID from GERD. Of 55 infants with intestinal malrotation alone, 52 (95%) had GERD, and 20 (36%) had the risk factor for SID from GERD. Although GERD was found in 19 (83%) of 23 patients with repaired abdominal wall defects, the prevalence of the risk factor for SID from GERD was significantly lower (13% [3 patients]; P = .03) than in patients with intestinal malrotation alone. The prevalence of GERD in infants with repaired congenital diaphragmatic hernia was significantly lower (69% [9/13]; P = .02) than in infants with intestinal malrotation alone but not for the prevalence of the risk factor for SID from GERD (23% [3/13]; P = .19). Both symptomatic and asymptomatic patients had similar prevalences of GERD (91% [52/57] vs. 82% [28/34], P = .17) and for the risk factor for SID from GERD (31% [18/57] vs. 24% [8/34]; P = .28). On follow-up, the prognosis for GERD in infants with intestinal malrotation was better in the infants who were asymptomatic than in those who were symptomatic at the initial extended esophageal pH monitoring. CONCLUSIONS: The prevalence of GERD in infants with intestinal malrotation is high, and the prevalence of the risk factor for SID from GERD is a significant concern. The prevalence of GERD is lower in infants with congenital diaphragmatic hernia. Infants with repaired abdominal wall defects have a lower prevalence of the risk factor for SID from GERD. We recommend careful evaluation and follow-up of infants with intestinal malrotation for problems, such as SID, from GERD.

Inhibin and CD99 (MIC2) expression in uterine stromal neoplasms with sex-cord-like elements.

Uterine mesenchymal neoplasms with sex-cord-like elements are designated as endometrial stromal tumor with sex-cord-like elements (ESTSCLE) or uterine tumor resembling ovarian sex-cord tumor (UTROSCT), depending on the extent of sex-cord-like differentiation. Occasionally, sex-cord elements similar to those in ESTSCLE and UTROSCT occur in uterine adenosarcomas. To determine whether the sex-cord-like elements in these tumors show immunohistological evidence of sex-cord differentiation, we studied a series of uterine neoplasms for expression of inhibin, a peptide hormone expressed by normal ovarian granulosa cells and ovarian sex-cord neoplasms, and CD99, a protein also expressed by granulosa cells, Sertoli cells, and some ovarian sex-cord tumors. Thirty uterine mesenchymal neoplasms (five epithelioid or plexiform smooth muscle tumors, three endometrial stromal tumors, two mixed endometrial stromal and smooth muscle tumors, 10 ESTSCLE, five UTROSCT, and five miscellaneous stromal processes) and five epithelial neoplasms were evaluated for expression of CD99 (clone 12E7) and inhibin (clone R1) in formalin-fixed, paraffin-embedded tissue. Three of 10 (30%) ESTSCLE and five of five (100%) UTROSCT were inhibin and CD99 immunoreactive. Inhibin staining was confined to the areas with sex-cord-like differentiation, and staining was generally much stronger and more extensive in areas featuring prominent foam cells. There were no differences in the degree or intensity of staining for inhibin in premenopausal and postmenopausal women. CD99 expression tended to correlate with inhibin and was typically confined to similar cell types in the individual neoplasms. Weak CD99 immunoreactivity was seen in one additional epithelioid smooth muscle tumor, whereas all other mesenchymal and epithelial neoplasms studied for inhibin and CD99 were negative. These results provide further immunohistological support for true sex-cord differentiation within uterine mesenchymal proliferations and suggest that the degree of sex-cord differentiation may correlate with the expression of these markers.

Diffusely infiltrative endometrial adenocarcinoma: an adenoma malignum pattern of myoinvasion.

The prognostic significance of a diffusely infiltrative intramyometrial growth pattern was evaluated in 110 cases of low-stage (stages I and II) endometrial adenocarcinoma. Fifty cases were associated with diffuse infiltration (DI group), and 50 cases had more conventional granulation tissue type intramyometrial infiltration (GTT group). Ten cases with carcinomatous involvement of deeply situated adenomyosis (ADMY group) were also studied. The diffusely infiltrative "adenoma malignum" growth pattern featured typically round, regular individual glands, clearly within myometrium but with minimal or absent stromal or inflammatory cell response. Myoinvasion of the conventional sort was characterized by irregular, sharply angulated abnormal glands within myometrium without interposed normal glands or endometrial stroma. The abnormal glands were surrounded, at least focally, by edematous stroma with granulation tissue type reaction and/or an inflammatory cell infiltrate. Mean follow-up was 77.8 months (range 3-219 months) for the patients with diffusely infiltrative myoinvasion and deep adenomyosis and 86.9 months (range 1-206 months) for the patients with conventional myoinvasion. Recurrence-free survival for patients with stage I disease and conventional myoinvasion (94%) was similar to that of patients with diffuse adenoma malignum infiltration (98%; p = 0.13). Survival rates for both groups were also similar. Two (4%) of the 50 patients with diffusely infiltrative adenoma malignum pattern of myoinvasion died of endometrial carcinoma 36 and 72 months after hysterectomy, and 2 (4%) of the 50 patients with conventional myoinvasion died 34 and 67 months after hysterectomy (p = 0.41). Survival in these patients correlated with depth of myometrial invasion and stage. There were no recurrences in the patients with deep adenomyosis. These results suggest that although endometrial carcinomas with diffuse myometrial infiltration are fully capable of aggressive clinical behavior, they do not appear to behave any more aggressively than those with conventional myometrial invasion. Prognostic indicators of clinically aggressive disease are similar to those that have been previously identified for endometrial carcinomas with the more conventional pattern of myometrial infiltration. They include cervical involvement, deep myometrial invasion, higher histologic grade, and lymph-vascular space invasion. Endometrial carcinomas with extensive involvement of adenomyosis and adjacent foci of minimal myometrial infiltration appear to have very low malignant potential, but the number of cases with this finding and adequate clinical follow-up is limited. This finding needs to be confirmed in a much larger series of cases.

Persistent gastroesophageal reflux disease after antireflux surgery in children: I. immediate postoperative evaluation using extended esophageal pH monitoring.

PURPOSE: There is a paucity of quantitative and reproducible follow-up data on childhood operations for gastroesophageal reflux disease (GERD). With the development of minimally invasive techniques for antireflux operations in children, there is a need to quantitatively determine immediate outcomes for such operations performed by laparotomy for comparison. METHODS: A retrospective review of 385 children (age range, 1 week to 15 years) who had a primary antireflux operation in a Children's or University Hospital performed by laparotomy between 1983 and 1997, and who also had an extended esophageal pH study performed within the first 12 postoperative weeks, was conducted. The operations performed included Nissen fundoplication (n = 135), Thal fundoplication (n = 195), and Boerema gastropexy (n = 55). An immediate postoperative failure of the operation to control GERD was defined as an abnormal esophageal pH score persisting up to the twelfth postoperative week. RESULTS: Eleven patients (2.9%) were classified as having an immediate postoperative failure of their operation to control GERD. An additional three patients had an abnormal esophageal pH score 2 weeks postoperatively, which subsequently reverted to a normal esophageal pH score by 12 weeks. The immediate postoperative failure rate was 1.5% (2 of 135) for the Nissen fundoplication, 1.5% (3 of 195) for the Thal fundoplication, and 10.9% (6 of 55) for the Boerema gastropexy. A higher failure rate (five patients, 36%) was seen for the first 14 patients who underwent a Boerema gastropexy during the learning curve period for this operation before 1985, and by excluding these patients the failure rate was 2.4% (1 of 41) after 1985. There was no significantly increased probability of immediate postoperative failure in patients with central nervous system disorders, prematurity, repaired esophageal atresia, or gastric emptying abnormalities. Only 5 (36%) of the 14 children with persisting symptoms suggestive of GERD had immediate postoperative failure of their operation. CONCLUSIONS: Extended esophageal pH monitoring during the first 12 postoperative weeks is a helpful tool to assess the immediate outcome of antireflux operations in children because clinical symptoms alone may be unreliable. The immediate failure rate for an antireflux operation performed in children by laparotomy is very low and seems to be unaffected by comorbid factors.

Analysis of digital pulse-volume recordings with radial and ulnar artery compression.

The vascular noninvasive studies of 289 consecutive cardiac surgery patients were reviewed to better understand hand blood-flow physiology in an older population with vascular disease. The radial artery was found to be more important to pulsatile digital blood flow than the ulnar artery. In more than 20 percent of hands, the thumb and the index and fifth fingers lost pulsatile blood flow with radial artery compression at the wrist compared with only 5 percent with ulnar artery compression. The maintenance of pulsatile digital blood flow did not follow anatomic patterns of blood vessels previously presumed to be of paramount importance. The hand acts more like a single vascular bed than it does like two separate systems with a connecting arch.

Congenital thymic cysts in children--mostly misdiagnosed.

BACKGROUND/PURPOSE: Thymic cysts are rare lesions of the neck and mediastinum that are difficult to diagnose. Often considered inconsequential, these lesions can frequently be symptomatic. In this report the authors contrast their experience with that of the literature. METHODS: From 1984 through 1997, the authors encountered 14 patients with this lesion. All cysts were completely excised. Patients that had an acquired cyst of the thymus were excluded from this series. RESULTS: Of the 14 patients ranging in age from 2 weeks to 16 years, seven patients had cervical masses, five had mediastinal masses, and two children had both sites involved. Seven children were symptomatic with wheezing and upper respiratory infection, with cough and fever being the most common clinical features. Investigations included chest radiograph, contrast esophagram, sonography (US) and computerized tomography (CT). Displacement of vital mediastinal or neck structures was observed in eight patients. Only two patients received correct diagnosis before surgery. Successful and complete excision of all cysts was achieved. The cysts were benign and ranged in size from 2 to 22 cm in diameter. CONCLUSION: Often forgotten, thymic cysts are rare benign lesions that should be considered in the differential diagnosis of cervical and mediastinal masses in children.

Radial artery use in bypass grafting does not change digital blood flow or hand function.

BACKGROUND: Patient selection criteria have not been clearly established for use of the radial artery as a bypass conduit. To help establish such criteria, we measured changes in digital blood flow and hand function after radial artery removal. METHODS: Ninety-eight patients of the first 122 consecutive patients considered for radial artery harvest met predetermined criteria by vascular noninvasive studies to undergo removal of the radial artery. In 42 of these 98 patients, the radial artery was actually used as a bypass conduit; 28 of these 42 patients returned for noninvasive vascular studies, a critical review of hand function, and a hand symptom questionnaire. RESULTS: There were no significant differences between the operated and nonoperated hands for digital-brachial indices, cold response, grip or pinch strength, digital two-point discrimination, or nine-hole peg tests. The patients had an increased incidence of a small amount of forearm numbness and tingling, but no increase of pain or cold intolerance. CONCLUSIONS: For properly selected patients, there are minimal changes in hand function after radial artery removal.

Developmental regulation of MCM replication factors in Xenopus laevis.

At the midblastula transition (MBT) during Xenopus laevis development, zygotic transcription begins [1], and the rapid, early cleavage cycles are replaced by cell-division cycles that lengthen and acquire G (gap) phases [2] and checkpoints [3-5]. This cell-cycle remodeling may result from either a loss of maternal products, the transcription of zygotic genes, or the replacement of maternal proteins by zygotic gene products. We have identified an example of the third possibility: distinct maternal and zygotic genes encoding a member of the minichromosome maintenance (MCM) protein family. The mcm genes were identified in yeast by mutations that blocked replication of artificial chromosomes or perturbed the G1/S transition in the cell cycle [6,7]. In Xenopus eggs, the MCM2-MCM7 proteins assemble as multimeric complexes at chromosomal origins of replication [8-14]. The sequential, cell-cycle-dependent assembly of the origin replication complex (ORC), CDC6 protein and the MCM complex at origins of replication ensures that DNA replicates only once per cell cycle [15,16]. The periodic association of the MCM complex with chromatin may be regulated via phosphorylation by cyclin-dependent kinases (Cdks) [11]. We have cloned the first example of a developmentally regulated mcm gene, zygotic mcm6 (zmcm6), expressed only after gastrulation when the cell cycle is remodeled. The zMCM6 protein assembles into MCM complexes and differs from maternal MCM6 (mMCM6) in having a carboxy-terminal extension and a consensus cyclin-Cdk phosphorylation site. There may also be maternal-zygotic pairs of other MCMs. These data suggest that MCMs are critical for cell-cycle remodeling during early Xenopus development.

Palatal fractures: classification, patterns, and treatment with rigid internal fixation.

A classification of palatal fracture types is developed from patterns observed on CT scans, and success with open reduction techniques is correlated with fracture pattern. The six palatal fracture types are as follows: I, anterior and posterolateral alveolar; II, sagittal; III, parasagittal; IV, para-alveolar; V, complex; and VI, transverse. Associated fractures were LeFort I (100 percent), LeFort II and III (55 percent), mandible (48 percent), and dental (55 percent). Large segment, sagittally oriented palatal fractures could be stabilized with rigid internal fixation. Complete rigid fixation of the palate consists of (1) roof of mouth, (2) pyriform or alveolar, and (3) four LeFort I buttress stabilization. Comminuted palatal fractures were managed by standard LeFort I and alveolar buttress fixation, palatal splinting, and intermaxillary, fixation. If complete rigid fixation was employed in the palate in type II, III, and IV fractures, a palatal splint was avoided in 60 percent of these cases. Rigid internal fixation is therefore concluded to facilitate the treatment of certain types of palatal fractures by reduced length of intermaxillary fixation and avoidance of palatal splinting.

Value of skin biopsies in assessing prognosis and progression of acute graft-versus-host disease.

Skin biopsies are commonly performed after allogeneic bone marrow transplantation (BMT) to help establish the origin of a new skin rash in a transplant recipient. Histologic criteria and a grading system for acute graft-versus-host reaction of the skin are well established. Histologic diagnosis, however, can be difficult and is based on interpretation of subtle changes that show significant overlap with features seen in other entities that can be responsible for a skin rash in the posttransplantation period such as drug reactions, viral exanthems, and the effects of chemotherapy. We retrospectively reviewed 179 skin biopsies from 137 patients who had undergone allogeneic BMT. We compared 98 skin biopsies from 71 patients with acute graft-versus-host disease (GvHD) with 81 biopsies from 66 patients who underwent biopsy to exclude GvHD but did not go on to develop the disease on clinical grounds. Two observers reviewed each slide without knowledge of the clinical situation and graded 16 histologic parameters. No single parameter (e.g., dyskeratotic keratinocytes, basal vacuolization, satellitosis, necrotic cells in appendages) achieved statistical significance on univariate analysis. A search for factors to separate GvHD biopsies from non-GvHD biopsies using logistic regression failed to reveal a single best predictor or a combination of predictors. We conclude that skin biopsies after allogeneic BMT are of limited use in predicting the progression of a skin rash to clinical grade II or higher GvHD.

Lymphangioleiomyomatosis of the uterus simulating high-stage endometrial stromal sarcoma.

Symptomatic uterine lymphangioleiomyomatosis (LAM) simulating high-stage uterine sarcoma in a patient with tuberous sclerosis complex is reported. A 49-year-old female presented with abdominal pain and anemia. Preoperative workup revealed a uterine mass and a large amount of peritoneal free fluid and possible metastatic implant along the lateral edge of the liver. The patient also had a large right pleural effusion. A fungating anterior uterine fundal mass with apparent perforation and intraabdominal hemorrhage was found on laparotomy. A portion of the mass was excised and initially interpreted as an endometrial stromal sarcoma. Microscopic examination revealed multiple vascular epithelioid smooth muscle proliferations in the uterus and serosal surface of the fallopian tube and periaortic lymph node lymphangioleiomyomas. The uterine, fallopian tube, and nodal lesions were positive for smooth muscle actin, desmin, and HMB-45, findings characteristic of LAM. Additional examination of the patient revealed stigmata of tuberous sclerosis complex. Although uterine LAM is uncommon, it may be associated with pelvic and/or abdominal symptoms and may simulate a primary uterine mesenchymal neoplasm.