Incorporation of Integra<Superscript>®</Superscript> in tissue defects: a pilot study in the rat model

Integra has been shown to be very useful in accelerating the growth of neodermis. It has found extensive use in case of burns as a primary dressing immediately after a burn, after release of contractures and following scar revision. It has been used to achieve cover after the debridement of extensive infective processes involving the skin. Encouraged by these results we have assessed the application of Integra to augment and/or patch defects of the urinary bladder, diaphragm and the abdominal wall in the rat model. This was a pilot study and involved the incorporation of Integra in the diaphragm, the urinary bladder (extramucosal) and the muscle layer of the abdominal wall. Eight adult Wistar rats were given general anaesthesia and Integra was implanted with absorbable sutures at the sites mentioned. The omentum was hitched to the collagen matrix surface to revascularise the graft. The silicone was left in situ. The operative period was covered with antibiotics. The anaesthesia was then reversed. Postoperatively the rats were given analgesia and feeds started immediately. The rats were sacrificed after 3 weeks. The abdominal cavity was examined for adhesions. The Integra implant along with adjacent tissue was harvested and examined histologically. There were no visible intra-abdominal adhesions. The histology revealed good degree of neovascularisation and fibrosis in and adjacent to the implant. This was comparable to the changes seen in the skin. This pilot study has shown that implanting Integra invokes a similar response in deeper tissues and it can develop neovascularisation from the omentum. Hence, this could find some application in treating congenital conditions such as diaphragmatic hernias, abdominal wall defects and for bladders requiring augmentation. Our initial results are quite encouraging and we feel that this field should be further explored.     

Matriderm<Superscript>®</Superscript> 1 mm versus Integra<Superscript>®</Superscript> Single Layer 1.3 mm for one-step closure of full thickness skin defects: a comparative experimental study in rats

Purpose

Dermal templates, such as Matriderm^® and Integra^®, are widely used in plastic and reconstructive surgery, often as two-step procedures. A recent development is the application of thin dermal templates covered with split thickness skin grafts in one-step procedures. In this experimental study, we compare the two thin matrices Matriderm^® 1 mm and Integra^® Single Layer in a one-step procedure with particular focus on neodermis formation.

Methods

Matriderm^® 1 mm and Integra^® Dermal Regeneration Template—Single Layer (1.3 mm) were compared in a rat model. In three groups of five animals each, a full thickness wound was covered with (a) Matriderm^® 1 mm and neonatal rat epidermis, (b) Integra^® Single Layer and neonatal rat epidermis, or, (c) neonatal rat epidermis only (control). Histological sections 2 weeks post transplantation were analyzed with regard to take of template and epidermis, neodermal thickness, collagen deposition, vascularization, and inflammatory response.

Results

Take of both templates was complete in all animals. The Matriderm^®-based neodermis was thinner but showed a higher cell density than the Integra^®-based neodermis. The other parameters were similar in both matrices.

Conclusion

The two templates demonstrate a comparable biological behavior early after transplantation. The only difference was found regarding neodermal thickness, probably resulting from faster degradation of Matriderm^®. These preliminary data suggest that both dermal templates appear similarly suitable for transplantation in a one-step procedure.

     

Selective deficiency of naïve CD4<Superscript>+</Superscript> T-lymphocytes in a child with congenital lymphoedema

We describe the first known case of congenital lymphoedema associated with selective deficit of naïve CD4⁺ T-lymphocytes. A high proportion of naïve CD4⁺ T-lymphocytes was found in the ascitic fluid, supporting the hypothesis of extra-vascular sequestration of these cells into lymphoedematous tissue.Abbreviation ICL idiopathic CD4⁺ lymphocytopenia     

A unique service in UK delivering Plastibell<Superscript>®</Superscript> circumcision: review of 9-year results

Muslim infants undergo circumcision for religious reasons and Bradford has a high Muslim population. The National Health Service in UK does not provide religious circumcision, so in 1996 a nurse-delivered circumcision service led by consultant urologists was set up at a no-profit and cost-only basis. Plastibell circumcision was offered to all infants between 6 and 14 weeks old and performed under local anaesthesia. Information leaflets and videotapes about the procedure were available to parents prior to the procedure. A three monthly audit of the service was undertaken. Between July 1996 and June 2005 (9 years) 1,129 circumcisions were performed. The common complications were problems with the ring (3.6%) and bleeding (3%). Overall, there was 96% satisfaction rate among the service users. The Plastibell technique for circumcision is a simple method and can be safely performed by trained nurses with acceptable complication rates.     

The use of Surgisis<Superscript><Emphasis Type="Bold">®</Emphasis></Superscript> for abdominal wall reconstruction in the separation of omphalopagus conjoined twins

Abdominal wall reconstruction in omphalopagus twins poses a difficult reconstructive challenge, as separation often results in a large abdominal wall defect. A number of options are available for closure, including tissue flaps, expanders and patches made of foreign material. Surgisis is a new biodegradable small intestine scaffolding substrate that permits tissue in-growth and results in a permanent durable scar. We describe its use in abdominal wall reconstruction after separation of a set of conjoined twins. A set of omphalopagus conjoined twins shared liver and abdominal wall. After separation at 6 months of age, Twin A's abdomen could be closed primarily, but Twin B could not. A 4-ply Surgisis mesh was used in the upper abdominal closure, and a skin flap was created, to completely cover the patch. Both twins survived the operation. A small portion of the skin flap over the Surgisis broke down, healing by secondary intention. In follow up of over 18 months post procedure, there have been no wound infections and the abdominal wall is intact with no evidence of a hernia. Surgisis can be successfully used for the reconstruction of complex abdominal wall defects in the pediatric patient, including reconstruction after separation of conjoined twins.     

Mechanism of Rho-kinase-mediated Ca<Superscript>2+</Superscript>-independent contraction in aganglionic smooth muscle in a rat model of Hirschsprung’s disease

Purpose  

Lack of ganglion cells is the main cause of bowel movement disorder in Hirschsprung’s disease. Because smooth muscle is the primary organ, the properties of intestinal smooth muscle need to be investigated. We therefore investigated the reactivity of the contractile system and the mechanism of contraction in aganglionic intestinal smooth muscle.     

Disseminated Bacillus Calmette-Guérin lymphadenitis in a patient with gp91<Emphasis Type="Italic">phox</Emphasis><Superscript>−</Superscript> chronic granulomatous disease 25 years after vaccination

A boy developed ipsilateral axillary lymphadenitis after Bacillus Calmette-Guérin (BCG) inoculation at the age of 5 months. Subsequently, he was diagnosed with X-linked chronic granulomatous disease (CGD) by the nitroblue tetrazolium assay when he was 4 years old. Body computerized tomography (CT) performed at the age of 25 years showed enlarged lymph nodes in the left periclavicular and axillary regions, and was confirmed by gallium scintigraphy. Mycobacterial culture, smear, and polymerase chain reaction (PCR) of the sputum and gastric fluid were negative. Whole-blood IFN-γ assay was negative as well. Mycobacterium bovis BCG was isolated from the lymph node biopsy by PCR amplification and culture. No mutation of the IFN-γ receptor 1 could be identified. In conclusion, CGD can be the underlying condition for BCG-itis; whole-blood IFN-γ assay might be useful in differentiating BCG infection and tuberculosis in CGD patients; BCG vaccination is contraindicated in X-linked CGD.     

Arrhythmia Detection in Pediatric Patients: ECG Quality and Diagnostic Yield of a Patient-Triggered Einthoven Lead-I Event Recorder (Zenicor <Emphasis Type="Italic">EKG</Emphasis>-<Emphasis Type="Italic">2™</Emphasis>)

Symptoms that may be caused by arrhythmia are common in pediatric outpatient departments, though it remains challenging to reveal paroxysmal tachycardia. This investigation evaluated prospectively the quality and diagnostic yield of a newly available handheld patient-activated event recorder (ER) in children. In 226 children (pts) aged 0–17 years with or without congenital heart defects, pacemaker/ICDs or arrhythmia, a lead-I ER ECG was created. ER ECGs were recorded by pressing the patients’ thumbs on the device and were analyzed in comparison with a lead-12 ECG, as gold standard. Event recording and data transmission were possible in all cases. ECG quality of the ER showed a high accordance in measuring heart rate (ICC = 0.962), duration of QRS complexes (κ = 0.686), and PR interval (ICC = 0.750) (p < 0.001) although P wave detection remained challenging (p = 0.120). 36 % (n = 82) of the pts had heart rhythm disturbances. The ER yielded 92 % sensitivity in diagnosing supraventricular tachycardia plus 77 % sensitivity and 92 % specificity in identifying abnormal ECGs. In children, the application of the tested ER was suitable. ECGs of good quality could be performed and transmitted easily, and also complex arrhythmia analysis was possible. This ER is an excellent diagnostic device for the detection and exclusion of tachycardia in children.     

Factors Affecting Length of Postoperative Hospitalization for Pediatric Cardiac Operations in a Large North American Registry (1982–2007)

Surgical treatment of congenital heart disease represents a major cause of pediatric hospitalization and healthcare resource use. Larger centers may provide more efficient care with resulting shorter length of postoperative hospitalization (LOH). Data from 46 centers over 25 years were used to evaluate whether surgical volume was an important determinant of LOH using a competing risk regression strategy that concurrently accounted for deaths, transfers, and discharges with some time interactions. Earlier discharge was more likely for infants and older children compared to neonates [subhazard ratios at postoperative day 6 of 1.64 (99 % confidence interval (CI) 1.57, 1.72) and 2.67 (99 % CI 2.53, 2.80), respectively], but less likely for patients undergoing operations in Risk Adjustment for Congenital Heart Surgery categories 2, 3, 4, and 5/6 compared to category 1 [subhazard ratios at postoperative day 6 of 0.66 (99 % CI 0.64, 0.68), 0.34 (95 % CI 0.33, 0.35), 0.28 (99 % CI 0.27, 0.30), and 0.10 (99 % CI 0.09, 0.11), respectively]. There was no difference by sex [non-time-dependent subhazard ratio 1.019 (99 % CI 0.995, 1.040)]. For every 100-operation increase in center annual surgical volume, the non-time-dependent subhazard for discharge was 1.035 (99 % CI 1.006, 1.064) times greater, and center-specific exponentiated random effects ranged from 0.70 to 1.42 with a variance of 0.023. The conditional discharge rate increased with increasing age and later era. No sex-specific difference was found. Centers performing more operations discharged patients sooner than lower volume centers, but this difference appears to be too small to be of clinical significance. Interestingly, unmeasured institutional characteristics estimated by the center random effects were variable, suggesting that these played an important role in LOH and merit further investigation.     

Novel <Emphasis Type="Italic">UBR1</Emphasis> gene mutation in a patient with typical phenotype of Johanson–Blizzard syndrome

Johanson–Blizzard syndrome is a rare autosomal recessive disorder, characterized by exocrine pancreatic deficiency and a wide range of other abnormalities. We present here an infant with failure to thrive, exocrine pancreatic deficiency, short stature and developmental delay, cutis aplasia on the scalp, aplasia of alae nasi, hypospadias, hypothyroidism, myxomatous mitral valve, and patent ductus arteriosus. Molecular studies revealed a novel homozygous nonsense mutation in exon 38 of the UBR1 gene, which confirmed the diagnosis of Johanson–Blizzard syndrome. It should be acknowledged that the combination of exocrine pancreatic insufficiency and nasal wing hypo-aplasia is pathognomonic for this syndrome. Prompt diagnosis and exact monitoring of the patients with JBS are required to avoid further complications.     

<Emphasis Type="Italic">PHOX2B</Emphasis> mutations in patients with Ondine–Hirschsprung disease and a review of the literature

Congenital central hypoventilation syndrome (CCHS), also known as Ondine's curse, is characterized by idiopathic failure of autonomic breathing and is often associated with neurocristopathies such as Hirschsprung disease (HSCR). CCHS is caused by mutations in the paired-like homeobox 2B (PHOX2B) gene, often manifest as polyalanine repeat expansions. Herein, we report the cases of two unrelated Korean patients with Ondine–Hirschsprung disease. The patient's clinical manifestations were apnea and cyanosis requiring immediate endotracheal intubation, recurrent hypoventilation with hypercapnia, hypoxia after ventilator removal, and abdominal distension since birth. Intestinal biopsies were performed and the absence of ganglion cells in the colon was consistent with HSCR. We performed direct sequencing analysis in the PHOX2B and RET genes and fluorescence polymerase chain reaction in order to determine the polyalanine tract expansion in exon 3 of the PHOX2B gene. Expansion mutations were detected in both patients; one had 20/24 repeats and the other had 20/27 repeats. The 20/24 genotype has not been previously described in severe CCHS phenotypes and associated HSCR. We believe that the information in this report will improve our understanding of the phenotypic and genotypic heterogeneities of CCHS and HSCR.     

Solitary ecthyma gangrenosum (EG) — like lesion consequent to <Emphasis Type="Italic">Candida albicans</Emphasis> in a neonate

Ecthyma gangrenosum (EG) is a well recognized cutaneous lesion most commonly associated with Pseudomonas aeruginosa bacteremia. It usually occurs in immunocompromised and critically ill patients. Uncommonly, EG like lesions have also been observed in patients with fungal infections, like Fusarium Candida, Mucor and Aspergillus. We present a case of EG-Like lesion due to Candida albicans, in a 12 day old neonate.     

Broad and unexpected phenotypic expression in Greek children with steroid-resistant nephrotic syndrome due to mutations in the Wilms’ tumor 1 (<Emphasis Type="Italic">WT1</Emphasis>) gene

Mutations in the Wilms’ tumor suppressor gene 1 (WT1), most commonly within exons 8 or 9 or intron 9, are found in cases with the overlapping conditions of Denys–Drash and Frasier syndromes, as well as in patients with steroid-resistant nephrotic syndrome (SRNS). This study investigated the presence of WT1 gene mutations in cases with childhood SRNS, along with an evaluation of their clinical outcome. Twenty-seven Greek children with sporadic (19 cases) and familial (8 cases) SRNS were tested. Four phenotypically female patients with sporadic SRNS were found to carry de novo WT1 mutations, including two cases with p.R394W, and one case each with p.R366H, or n.1228+5G>A. Karyotype analysis found 46XX in three cases, but 46XY in one. No phenotype–genotype correlations were apparent in the WT1 gene positive cases since their clinical presentation varied broadly. Interestingly, one patient with a pathological WT1 nucleotide variation responded fully to combined therapy with cyclosporine A and corticosteroids. This study further illustrates that investigation of WT1 gene mutations is clinically useful to support definitive diagnosis in children presenting with SRNS in order to direct the most appropriate clinical management.     

Familiarity with current practices of granting and maintaining privileges in pediatric interventional radiology—a worldwide survey of the members of the Society for Pediatric Interventional Radiology (SPIR)

Background

Physician credentialing is a complex process driven by the demand for quality improvement in health care. In the U.S., the Joint Commission Standard of 2007 has tied hospital accreditation to credentialing through mandated use of the Focused Professional Practice Evaluation (FPPE) and Ongoing Professional Practice Evaluation (OPPE).

Objective

To assess pediatric interventional radiologists’ knowledge of how institutions grant them privileges.

Materials and methods

Members of the Society for Pediatric Interventional Radiology (SPIR) were sent a web-based survey regarding credentialing.

Results

Of 122 members from 19 countries, 81 (66%) responded, and of these 81, 59 (73%) were familiar with their hospital’s privileging process. Of 49 U.S. respondents and 32 non-U.S. respondents, 37 (76%) and 17 (53%), respectively, stated that interventional radiology credentialing was different from diagnostic radiology credentialing. Of the 49 U.S. respondents, 24 (49%) reported an OPPE, and of the 32 non-U.S. respondents, 8 (25%) reported an ongoing evaluation. The U.S. OPPE is performed at shorter intervals than its international equivalent.

Conclusion

Four years after the Joint Commission defined the FPPE and OPPE, separate credentialing of pediatric interventional radiology from pediatric diagnostic radiology is more likely in the U.S. than internationally, and U.S. pediatric interventional radiologists are more likely to have a defined ongoing professional evaluation and to be evaluated every 6?months or more frequently. There are many SPIR members who do not know how they obtain privileges and/or are not knowingly subject to an OPPE. This lack of knowledge may affect future education of interventional radiologists as well as the definition of pediatric interventional radiology practices within individual institutions.     

Sudden and unexpected deaths after the administration of hexavalent vaccines (diphtheria,tetanus, pertussis,poliomyelitis, hepatitis B,<Emphasis Type="Italic"> Haemophilius influenzae</Emphasis> type b): is there a signal?

Deaths in temporal association with vaccination of hexavalent vaccines have been recently reported. The objective of this paper is to assess whether these temporal associations can be attributed to chance. Standardised mortality ratios (SMR) for deaths within 1 to 28 days after administration of either of the two hexavalent vaccines in the 1st and 2nd year of life were determined using the respective annual rates for sudden unexpected deaths (SUDs) from the national vital statistics. The distribution of SUD cases and the vaccination uptake by month were estimated from surveys and sales figures for the individual vaccines. Sensitivity analyses were performed to account for limitations in the data sources. For one of the vaccines, Vaccine B, all SMRs were well below one. For the other, Vaccine A, SMRs exceeded one insignificantly on the 1st day after vaccination in the 1st year of life. In the 2nd year of life, however, the SMRs for SUD cases within 1 day of vaccination with vaccine A were 31.3 (95% CI 3.8–113.1; two cases observed; 0.06 cases expected) and 23.5 (95% CI 4.8–68,6) for within 2 days after vaccination (three cases observed; 0.13 cases expected). Extensive sensitivity analyses could not attribute these findings to limitations of the data sources. Conclusion: These findings based on spontaneous reporting do not prove a causal relationship between vaccination and sudden unexpected deaths. However, they constitute a signal for one of the two hexavalent vaccines which should prompt intensified surveillance for unexpected deaths after vaccination.In this paper the two vaccines have been labelled Vaccine A and Vaccine B. The brand names are not conveyed since the European licensing agency has not recommended regulatory action against either vaccine.