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To link to full-text access for this article, visit this link: http://dx.doi.org/10.1016/j.forsciint.2009.12.056 Byline: Valerio Causin (a), Carla Marega (a), Antonio Marigo (a), Rosario Casamassima (b), Giuseppe Peluso (b), Luigi Ripani (b) Keywords: Forensic science; Questioned documents; Paper; Cellulose; Polymers; IR spectroscopy; X-ray diffraction Abstract: The possibility to discriminate between sheets of paper can be of considerable importance in questioned document examinations. 19 similar types of office paper were characterized by infrared spectroscopy and X-ray diffraction to individuate the most discriminating features that could be measured by these techniques. The discriminating value associated to them was also assessed. By using a sequence of these two techniques, all the samples could be differentiated. Author Affiliation: (a) Dipartimento di Scienze Chimiche dell'Universita, via Marzolo 1, 35131 Padova, Italy (b) Sezione di Chimica, Esplosivi ed Infiammabili, Reparto Carabinieri Investigazioni Scientifiche, Viale Tor di Quinto 151, 00191 Roma, Italy Article History: Received 10 July 2009; Revised 16 December 2009; Accepted 17 December 2009

Cancer still represents a “nightmare” worldwide, causing annually millions of victims. Several antiproliferative molecules are currently used as drugs market and offer a pharmaceutical opportunity for attenuating and treating tumor manifestations. In this context, natural sources have a relevant role, since they provide the 60% of currently-used anticancer agents. Among the numerous natural products, acting via different mechanisms of action, microtubule-targeting agents (MTAs) have a high therapeutic potential, since they disrupt the abnormal cancer cell growth, interfering with the continuous mitotic division. Vinca alkaloids (VAs) are the earliest developed MTAs and approved for clinical use (Vincristine, Vinblastine, Vinorelbine, Vindesine, and Vinflunine) as agents in the treatment of hematological and lymphatic neoplasms. Here, we review the state-of-art of VAs, discussing their mechanism of action and pharmacokinetic properties and highlighting their therapeutic relevance and toxicological profile. Additionally, we briefly disclosed the technological approaches faced so far to ameliorate the pharmacological properties, as well as to avoid the drug resistance. Lastly, we introduced the recent advances in the discovery of new derivatives. [ABSTRACT FROM AUTHOR]

Background Extensive literature has proved that the Nuss procedure leads to permanent remodeling of the chest wall in pediatric patients with pectus excavatum (PE). However, limited long-term follow-up data are available for adults. Herein, we report a single-institution experience in the management of adult PE with the Nuss procedure, evaluating long-term outcomes and overall patient satisfaction after bar removal. Methods Adult patients who underwent PE repair with a modified Nuss procedure between January 1998 and June 2011 were retrospectively identified. Outcomes of interest were postoperative pain, recurrence, and patient satisfaction. A modified single-step Nuss questionnaire was administered to evaluate patient satisfaction and quality-of-life improvement after PE repair. Results Ninety-eight patients with a median age of 30.9 years (range, 21.8 to 55.1 years) at the time of repair were identified. One bar was placed in most patients (89.7%). Four patients (4.1%) required reoperation for bar displacement. Results after bar removal were overall satisfactory in 94.4% of patients; 2 patients required reoperation for recurrence. Thirty-nine patients participated in the survey. Satisfaction with chest appearance was reported by 89.7% of responders. Seven patients reported dissatisfaction with the overall results; the most common complaints were severe postoperative chest pain and dissatisfaction with surgical scars. Conclusions Favorable long-term results can be achieved with the Nuss procedure in adults. However, postoperative pain may require a more aggressive analgesic regimen, and it may be the overriding factor in the patient’s perception of the quality of the postoperative course. [ABSTRACT FROM AUTHOR]

Summary In animals, the protein kinase C (PKC) family has expanded into diversely regulated subgroups, including the Rho family-responsive PKN kinases. Here, we describe knockouts of all three mouse PKN isoforms and reveal that PKN2 loss results in lethality at embryonic day 10 (E10), with associated cardiovascular and morphogenetic defects. The cardiovascular phenotype was not recapitulated by conditional deletion of PKN2 in endothelial cells or the developing heart. In contrast, inducible systemic deletion of PKN2 after E7 provoked collapse of the embryonic mesoderm. Furthermore, mouse embryonic fibroblasts, which arise from the embryonic mesoderm, depend on PKN2 for proliferation and motility. These cellular defects are reflected in vivo as dependence on PKN2 for mesoderm proliferation and neural crest migration. We conclude that failure of the mesoderm to expand in the absence of PKN2 compromises cardiovascular integrity and development, resulting in lethality. [ABSTRACT FROM AUTHOR]

Background Childhood obesity is a worsening epidemic. Little is known about the impact of elevated BMI on perioperative and postoperative complications in children who undergo laparoscopic surgery. The purpose of this study was to examine the effects of obesity on surgical outcomes in children using laparoscopic appendectomy as a model for the broader field of laparoscopic surgery. Study design Using the Pediatric National Surgical Quality Improvement Program (NSQIP) data from 2012, patients aged 2–18 years old with acute uncomplicated and complicated appendicitis who underwent laparoscopic appendectomy were identified. Children with a body mass index (BMI) ≥ 95th percentile for their age and gender were considered obese. Primary outcomes, including overall morbidity and wound complications, were compared between nonobese and obese children. Multivariate regression analysis was conducted to identify the impact of obesity on outcome. Results A total of 2812 children with acute appendicitis who underwent appendectomy were included in the analysis; 22% were obese. Obese children had longer operative times but did not suffer increased postoperative complications when controlling for confounders (OR 1.3, 95% CI: 0.83–0.072 for overall complications, OR 1.3, 95% CI: 0.84–1.95 for wound complications). Conclusions Obesity is not an independent risk factor for postoperative complications following laparoscopic appendectomy. Although operative times are increased in obese children, obesity does not increase the likelihood of 30-day postoperative complications. [ABSTRACT FROM AUTHOR]

Background Optimal management of recurrent pectus excavatum (PE) has not been established. Here, we review our institutional experience in managing recurrent PE to evaluate long-term outcomes and propose an anatomic classification of recurrences, and a decision-making algorithm. Methods Clinical records of patients undergoing repair of recurrent PE (1996–2011) were reviewed. Univariate and multivariate logistic regression analyses were employed to examine patient characteristics as potential predictors for re-recurrence. Results Eighty-five patients with recurrent PE were identified during the study period. The initial operation was a Ravitch procedure in 85% of cases. Revision procedures were most frequently Nuss repairs ( N = 73, 86%), with remaining cases managed via open approach. Overall cosmetic and functional results were satisfactory in 67 patients (91.8%) managed with Nuss and in 7 (58%) patients managed with other techniques. Seven (8%) patients required additional surgical revision. Multivariate analysis identified no statistically significant patient or procedural factors predictive of re-recurrence. Conclusion This study demonstrates that the Nuss procedure can be an effective intervention for recurrent pectus excavatum, regardless of the initial repair technique. However, open repair remains valuable when managing severe cases with abnormalities of the sternocostal junction and cartilage regrowth under the sternum. [ABSTRACT FROM AUTHOR]

Abstract: Background: For a number of pediatric and adult conditions, morbidity and mortality are increased when patients present to the hospital on a weekend compared to weekdays. The objective of this study was to compare pediatric surgical outcomes following weekend versus weekday procedures. Methods: Using the Nationwide Inpatient Sample and the Kids’ Inpatient Database, we identified 439,457 pediatric (<18years old) admissions from 1988 to 2010 that required a selected index surgical procedure (abscess drainage, appendectomy, inguinal hernia repair, open fracture reduction with internal fixation, or placement/revision of ventricular shunt) on the same day of admission. Outcome metrics were compared using logistic regression models that adjusted for patient and hospital characteristics as well as procedure performed. Results: Patient characteristics of those admitted on the weekend (n=112,064) and weekday (n=327,393) were similar, though patients admitted on the weekend were more likely to be coded as emergent (61% versus 53%). After multivariate adjustment and regression, patients undergoing a weekend procedure were more likely to die (OR 1.63, 95% CI 1.21–2.20), receive a blood transfusion despite similar rates of intraoperative hemorrhage (OR 1.15, 95% CI 1.01–1.26), and suffer from procedural complications (OR 1.40, 95% CI 1.14–1.74). Conclusion: Pediatric patients undergoing common urgent surgical procedures during a weekend admission have a higher adjusted risk of death, blood transfusion, and procedural complications. While the exact etiology of these findings is not clear, the timing of surgical procedures should be considered in the context of systems-based deficiencies that may be detrimental to pediatric surgical care. [Copyright &y& Elsevier]

Abstract: Background: The safety and efficacy of minimally invasive pectus excavatum repair have been demonstrated over the last twenty years. However, technical details and perioperative management strategies continue to be debated. The aim of the present study is to review a large single-institution experience with the modified Nuss procedure. Methods: A retrospective review was performed of patients who underwent primary pectus excavatum repair at a single tertiary hospital via a modified Nuss procedure that included: no thoracoscopy, retrosternal dissection achieved via a left-to-right thoracic approach, four-point stabilization of the bar, and no routine epidural analgesia. Data collected included demographics, preoperative symptoms, operative characteristics, hospital charges and postoperative outcomes. Results: A total of 336 pediatric patients were identified. No cardiac perforations occurred and the rate of pericarditis was 0.6%. Contemporary rates of bar displacement have fallen to 1.2%. Routine use of chlorhexidine scrub reduced superficial site infections to 0.7%. Two patients (0.6%) with severe recurrence required reoperation. Bars were removed after an average period of 31.7(SD 13.2) months, with satisfactory cosmetic and functional results in 94.9% of cases. Conclusions: We report here a single-institution large volume experience, including modifications to the Nuss procedure that make the technique simpler and safer, improve results, and minimize hospital charges. [Copyright &y& Elsevier]

Abstract: Background: Acquired Jeune's syndrome is a severe iatrogenic deformity of the thoracic wall following a premature and aggressive open pectus excavatum repair. We report herein our technique and experience with this rare condition. Methods: From 1996 to 2011, nineteen patients with acquired Jeune's syndrome were retrospectively identified in a tertiary referral center. The technique used to expand and reconstruct the thoracic wall consisted of 1) release of the sternum from fibrous scar tissue, 2) multiple osteotomies along the lateral aspect of the ribs with anterior advancement of costal-cartilages to protect the heart, 3) stabilization of the thorax by placing a curved bar for retrosternal support and, 4) restoration of the sterno-costal junction by wiring the lower cartilages to the edge of the sternum. Results: Major complications observed in this series were: bar displacement (seven cases), postoperative death from cardiac arrest following bronchoscopy (one case), late cardiac tamponade from migration of wire suture fragment (one case), and need for multiple reoperations (one case). Long-term cosmetic results and improvement in daily quality of life were reported as positive in the majority of cases. Conclusions: Anterior chest wall reconstruction successfully treated our series of patients with acquired Jeune's syndrome. This multifaceted technique is an effective procedure that allows expansion of the thoracic cavity and improvement of aerobic activity. [Copyright &y& Elsevier]

Background: Pectus excavatum (PE) can be associated with congenital and acquired cardiac disorders that also require surgical repair. The timing and specific surgical technique for repair of PE remains controversial. The present study reports the experience of combined repair of PE and open heart surgery at Johns Hopkins Hospital. Methods: A retrospective case review was conducted of all patients who presented for repair of PE deformity while undergoing concurrent open heart surgery from 1998 through 2011. Results: A total of 9 patients met inclusion criteria. All patients had a connective tissue disorder. Repair of PE was performed by modified Nuss technique after completion of the cardiac procedure, performed through a median sternotomy. Open heart procedures were either aortic root replacement or mitral valvuloplasty. Eight patients had bar removal after an average period of 30.3 months. No PE recurrence, bar displacement, or upper sternal depression was reported in 7 patients. Postoperatively, 1 patient exhibited pectus carinatum after a separate spinal fusion surgery for scoliosis. One patient died of unrelated cardiac complications before bar removal. Conclusions: Simultaneous repair of PE and open heart surgery is safe and effective. We recommend that the decision to perform a single-stage versus a multistage procedure should be reserved until after the cardiac procedure has been completed. In such cases, the Nuss technique allows for correction of the pectus deformity with good long-term cosmetic and functional results. [ABSTRACT FROM AUTHOR]

Abstract: Background: Advances in surgical technique for pectus excavatum repair continue to change practice patterns. The present study examines trends in operative age in a nationwide administrative database. Methods: A cross-sectional descriptive analysis was performed using the Nationwide Inpatient Sample (NIS) and Kids’ Inpatient Database (KID) data from 1998 to 2009. Pediatric discharges involving surgical repair of pectus excavatum were selected. Patients were sub-grouped by age at operation and calendar year of repair for further comparison. Results: A total of 5830 elective admissions were identified that met inclusion criteria. Mean age at operation was 13.5 years, and this increased from 11.8 years to 14.4 years over the period studied and was accompanied by narrowing of the interquartile range. Examined over groups of four calendar years, patient age at the time of repair was significantly higher in more recent years in both unadjusted and multivariate analyses (P < .001). Conclusions: The age at operation in this sample has steadily increased, with an accompanying decrease in variability. This is consistent with previous findings and with overall trends in patient selection reported in the literature. This selection pattern may reflect evolving consensus regarding optimal management of pectus excavatum and provide clinical guidance regarding appropriate referral and intervention. [Copyright &y& Elsevier]

Abstract: This study examined the effect of administration of verbascoside on plasma oxidative status and on specific blood and milk production parameters in Lacaune ewes during the peripartum period. The trial, lasting 60d, ran from 20d before lambing until 40d after and was conducted on 40 ewes in their 5th month of gestation. The animals were divided into two equal groups of 20: a control group CON, which did not receive food supplement, and the experimental group V, which received a supplement of verbascoside in their feed. Consumption of the supplement, which was 2g/ewe/d, was assured by individual and daily administration of feed. The animals received a pellet feed concentrate at a rate of 400g/ewe/d prepartum and 700g/ewe/d postpartum. The ration was completed with mixed hay ad libitum. The experimental measures included body weight; blood samples 20d before lambing and 0d, 20d and 40d after lambing; body condition score; individual yield and quality of milk. The administration of verbascoside significantly improved milk yield by 9.3% (P <0.01), significantly decreased triglycerides, total cholesterol, low-density lipoprotein cholesterol, alanine transferase (P <0.01) and bilirubin (P <0.05), and increased high density lipoprotein cholesterol (P <0.01). Furthermore, the parameters of oxidative status in plasma were positively influenced by the use of dietary verbascoside, which resulted in significant decreases in reactive oxygen metabolites (P <0.01) and thiobarbituric acid-reactive substances (P <0.05) and increases in vitamins A and E (P <0.01).The use of verbascoside during the peripartum period provided benefits on some blood parameters, oxidative status and milk production, particularly in the immediate postpartum period (0–20d). [Copyright &y& Elsevier]

Abstract: The application of diffuse reflectance UV–VIS–NIR spectroscopy is proposed to differentiate 20 office paper samples, which had been deemed similar by a preliminary visual examination under several different lighting sources. The samples were firstly screened on the basis of the qualitative appearance of their spectra. A further discrimination was obtained by taking into account three parameters: the average reflectivity of the paper samples in the range 680–900nm, and the integrated intensity of the absorption peak in the UV range at 272nm and at 360nm. The homogeneity of these parameters on both sides of the paper sheets was assessed, detecting a very uniform distribution of the optical brighteners. A special focus was posed on the determination of the discriminative power, in order to give a quantitative parameter on the proposed procedure, which is important when reporting results to the Court. The remarkable achievement of differentiating all the examined samples was obtained by UV–VIS spectroscopy, a very less expensive technique which is readily available in practically all forensic laboratories. [Copyright &y& Elsevier]

Purpose: To evaluate a retrospective single-institution outcome after hypofractionated stereotactic body radiotherapy (SBRT) for adrenal metastases. Methods and Materials: Between February 2002 and December 2009, we treated 48 patients with SBRT for adrenal metastases. The median age of the patient population was 62.7 years (range, 43–77 years). In the majority of patients, the prescription dose was 36 Gy in 3 fractions (70% isodose, 17.14 Gy per fraction at the isocenter). Eight patients were treated with single-fraction stereotactic radiosurgery and forty patients with multi-fraction stereotactic radiotherapy. Results: Overall, the series of patients was followed up for a median of 16.2 months (range, 3–63 months). At the time of analysis, 20 patients were alive and 28 patients were dead. The 1- and 2-year actuarial overall survival rates were 39.7% and 14.5%, respectively. We recorded 48 distant failures and 2 local failures, with a median interval to local failure of 4.9 months. The actuarial 1-year disease control rate was 9%; the actuarial 1- and 2-year local control rate was 90%. Conclusion: Our retrospective study indicated that SBRT for the treatment of adrenal metastases represents a safe and effective option with a control rate of 90% at 2 years. [ABSTRACT FROM AUTHOR]

Abstract: The effect of water restriction on milk yield and composition, feed intake, body weight and blood parameters was evaluated in Comisana sheep reared under intensive condition. The experiment, lasted 40 days, was performed on 26 lactating ewes subdivided into three treatment groups; the control T group received water ad libitum (W-100) and Group 1 (W-80) and Group 2 (W-60) were watered, respectively, with 80 and 60% of the water consumed by the control T group. Water was supplied in the morning once a day while feed twice. Milk quality analysis was performed every 10 days for pH, fat, total protein, lactose and somatic cell content. Serum samples, performed at days 0, 13, 26 and 40, were analyzed for glucose, cholesterol, triglycerides, creatinine, total proteins, albumin, sodium, potassium, calcium and chloride. Compared to W-100, W-60 was significantly different (P <0.05) for body weight and serum potassium levels and showed an increase (P <0.01) in serum concentrations of triglycerides, albumin, total proteins and cholesterol (P <0.05). Total proteins, triglycerides and sodium levels increased (P <0.05) in W-80. Body weight showed a significant difference (P <0.05) only in W-60 if compared to W-100. No significant differences were observed in milk yield, milk composition and feed intake across the treatment groups. [Copyright &y& Elsevier]

Purpose: An analysis is performed of the setup errors measured by a kV cone beam computed tomography (CBCT) for intracranial stereotactic radiotherapy (SRT) patients immobilized by a thermoplastic mask and a bite-block and positioned using stereotactic coordinates. We evaluated the overall positioning precision and accuracy of the immobilizing and localizing systems. The potential of image-guided radiotherapy to replace stereotactic methods is discussed. Methods and Materials: Fifty-seven patients received brain SRT. After a frame-guided setup, before each fraction (131 fractions), a CBCT was acquired and the detected displacements corrected online. Translational and rotational errors were analyzed calculating overall mean and standard deviation. A separate analysis was performed for bite-block (in conjunction with mask) and for simple thermoplastic mask. Interobserver variability for CBCT three-dimensional registration was assessed. The residual error after correction and intrafractional motion were calculated. Results: The mean module of the three-dimensional displacement vector was 3.0 ± 1.4 mm. Setup errors for bite block and mask were smaller (2.9 ± 1.3 mm) than those for thermoplastic mask alone (3.2 ± 1.5 mm), but statistical significance was not reached (p = 0.15). Interobserver variability was negligible. The maximum margin calculated for residual errors and intra fraction motion was small but not negligible (1.57 mm). Conclusions: Considering the detected setup errors, daily image guidance is essential for the efficacy of SRT treatments when mask immobilization is used, and even when a bite-block is used in conjunction. The frame setup is still used as a starting point for the opportunity of rotational corrections. Residual margins after on-line corrections must be evaluated. [Copyright &y& Elsevier]

Purpose: Different biologically equivalent dose (BED) values associated with stereotactic radiotherapy (SRT) of patients with primary and metastatic pulmonary nodules were studied. The BED values were calculated for tumoral tissue and low α/β ratio, assuming that better local response could be obtained by using stereotactic high-BED treatment. Methods and Materials: Fifty-eight patients with T1–T3 N0 non–small-cell lung cancer and 46 patients with metastatic lung nodules were treated with SRT. The BED was calculated for α/β ratios of 3 and 10. Overall survival (OS) was assessed according to Kaplan-Meier and appraised as a function of three BED levels: low (30–50 Gy), medium (50–70 Gy), and high (70–98 Gy; α/β = 10). Results: The OS rates for all 104 patients at 12, 24, and 36 months were 73%, 48.3%, and 35.8%, respectively. Local response greater than 50% for low, medium, and high BED values was observed in 54%, 47%, and 73%, respectively. In the high-BED treated group, OS rates at 12, 24, and 36 months (80.9%, 70%, and 53.6%, respectively) were significantly improved compared with low- (69%, 46.1%, and 30.7%, respectively) and medium-BED (67%, 28%, and 21%, respectively) treated patients. Results are also discussed in terms of BED calculated on α/β 3 Gy characteristic of the microcapillary bed. No acute toxicity higher than Grade 1 was observed. Conclusions: Radioablation of pulmonary neoplastic nodules may be achieved with SRT delivered by using a high-dose fraction with high BED value. [Copyright &y& Elsevier]

Abstract: Objectives: To evaluate the cystographic follow-up of patients with multicystic dysplastic kidney (MCDK), renal agenesis, and renal ectopia with associated primary vesicoureteral reflux (VUR). Methods: Patients with primary associated VUR (grade 2 or more) and with a minimal follow-up of 24 months were included in this study. Results: Of the children with renal agenesis, 24% had VUR. The median grade of VUR was significantly greater in the boys than in the girls (4 versus 2, respectively; P &#60;0.05). All girls and 34% of the boys experienced spontaneous resolution 1 year after diagnosis; 66% of the boys required operative treatment. Of the patients with MCDK, 16% had VUR. The median grade of VUR in the kidney contralateral to the MCDK was greater in the boys than in the girls (3.5 versus 2, respectively; P = 0.06). All girls and 60% of the boys had spontaneous resolution 1 year after diagnosis; 40% of the boys underwent operative treatment. Of the children with renal ectopia, 30% had VUR. The median grade of VUR for the refluxing unit was significantly greater in the girls than in the boys (3 versus 2, respectively; P &#60;0.05). Spontaneous resolution of VUR was observed in all boys (66% at 1 year) and in 40% of the girls; 60% of the girls required operative treatment. Conclusions: Spontaneous resolution of VUR can be anticipated in girls with MCDK or renal agenesis and in boys with renal ectopia. These data can be used in planning the proper follow-up schedule for babies with a kidney anomaly detected by ultrasonography. [Copyright &y& Elsevier]

Heteromultimerization between different potassium channel subunits can generate channels with novel functional properties and thus contributes to the rich functional diversity of this gene family. The inwardly rectifying potassium channel subunit Kir5.1 exhibits highly selective heteromultimerization with Kir4.1 to generate heteromeric Kir4.1/Kir5.1 channels with unique rectification and kinetic properties. These novel channels are also inhibited by intracellular pH within the physiological range and are thought to play a key role in linking K[sup +] and H[sup +] homeostasis by the kidney. However, the mechanisms that control heteromeric K[sup +] channel assembly and the structural elements that generate their unique functional properties are poorly understood. In this study we identify residues at an intersubunit interface between the cytoplasmic domains of Kir5.1 and Kir4.1 that influence the novel rectification and gating properties of heteromeric Kir4.1/Kir5.1 channels and that also contribute to their pH sensitivity. Furthermore, this interaction presents a structural mechanism for the functional coupling of these properties and explains how specific heteromeric interactions can contribute to the novel functional properties observed in heteromeric Kir channels. The highly conserved nature of this structural association between Kir subunits also has implications for understanding the general mechanisms of Kir channel gating and their regulation by intracellular pH. [ABSTRACT FROM AUTHOR]

Protein kinase N 1 (PKN1), which in part resembles yeast protein kinase C, has been shown to be under the control of Rho GTPases and 3-phosphoinositide-dependent kinase 1 (PDK1). We show here that green fluorescent protein-tagged PKN1 has the ability to translocate in a reversible manner to a vesicular compartment following hyperosmotic stress. PKN1 kinase activity is not necessary for this translocation, and in fact the PKN inhibitor HA1077 is also shown to induce PKN1 vesicle accumulation. PKN1 translocation is dependent on Rac1 activation, although the GTPase binding HR1abc domain is not sufficient for this recruitment. The PKN1 kinase domain, however, localizes constitutively to this compartment, and we demonstrate that this behavior is selective for PKNs. Associated with vesicle recruitment, PKN1 is shown to undergo activation loop phosphorylation and activation. It is established that this activation pathway involves PDK1, which is shown to be recruited to this PKN1-positive compartment upon hyperosmotic stress. Taken together, our findings present a pathway for the selective hyperosmotic-induced Rac1-dependent PKN1 translocation and PDK1-dependent activation. [ABSTRACT FROM AUTHOR]

Noonan syndrome is a genetic condition that can present with complex thoracic defects, the management of which often presents a surgical challenge. We present the surgical approach applied to a severe combined excavatum/carinatum deformity that had resulted in a Z-type configuration of the chest in a 9-year-old girl with Noonan syndrome. [ABSTRACT FROM AUTHOR]