[Ross-Konnno Procedure for the Patient of Intractable Subaortic Stenosis after Operation of Double Outlet Right Ventricle with Coarctation of the Aorta and Subaortic Stenosis:Report of a Case].

Recurrent left ventricular outflow obstruction (LVOTO) after intraventricular rerouting (IVR) with ventricular septal defect (VSD) enlargement and aortic arch repair for double-outlet right ventricle (DORV), non-committed VSD and coarctation of the aorta was successfully performed by a Ross-Konno procedure at 1 year 6 months as the second reoperation. Ross-Konno procedure could be an effective option in treatment of recurrent LVOTO after IVR for DORV.

Megalin-Mediated Tubuloglomerular Alterations in High-Fat Diet-Induced Kidney Disease.

Obesity, an important risk factor for metabolic syndrome (MetS) and cardiovascular disease, is often complicated by CKD, which further increases cardiovascular risk and causes ESRD. To elucidate the mechanism underlying this relationship, we investigated the role of the endocytic receptor megalin in proximal tubule epithelial cells (PTECs). We studied a high-fat diet (HFD)-induced obesity/MetS model using kidney-specific mosaic megalin knockout (KO) mice. Compared with control littermates fed a normal-fat diet, control littermates fed an HFD for 12 weeks showed autolysosomal dysfunction with autophagy impairment and increased expression of hypertrophy, lipid peroxidation, and senescence markers in PTECs of the S2 segment, peritubular capillary rarefaction with localized interstitial fibrosis, and glomerular hypertrophy with mesangial expansion. These were ameliorated in HFD-fed megalin KO mice, even though these mice had the same levels of obesity, dyslipidemia, and hyperglycemia as HFD-fed control mice. Intravital renal imaging of HFD-fed wild-type mice also demonstrated the accumulation of autofluorescent lipofuscin-like substances in PTECs of the S2 segment, accompanied by focal narrowing of tubular lumens and peritubular capillaries. In cultured PTECs, fatty acid-rich albumin induced the increased expression of genes encoding PDGF-B and monocyte chemoattractant protein-1 via megalin, with large (auto)lysosome formation, compared with fatty acid-depleted albumin. Collectively, the megalin-mediated endocytic handling of glomerular-filtered (lipo)toxic substances appears to be involved primarily in hypertrophic and senescent PTEC injury with autophagy impairment, causing peritubular capillary damage and retrograde glomerular alterations in HFD-induced kidney disease. Megalin could be a therapeutic target for obesity/MetS-related CKD, independently of weight, dyslipidemia, and hyperglycemia modification.

Compression brace for secondary pectus carinatum in infants and toddlers undergoing cardiac surgery with midline sternotomy.

PURPOSES: This study aimed to retrospectively assess the response to a newly developed compression brace for improving the deformity of the secondary pectus carinatum in infants and toddlers undergoing cardiac surgery with midline sternotomy. Factors affecting the response to the brace were identified. METHODS: Fifty-one children were enrolled. Severity was expressed as the protrusion angle of the sternum obtained from chest X-ray. The patients were divided into two groups by positive or negative binary residuals of the relationship between the angle at the beginning and its percentage change after wearing the brace. Logistic regression analysis was used to identify the influencing factors. RESULTS: Thirty patients (58.8%) showed zero and positive residuals to the relationship (good responders, Group G), whereas 21 patients showed negative residuals (poor responders, Group P). Male sex, severe cardiac anomaly, complex surgical procedure, multiple sternotomy, total duration, and self-discontinuation were associated with poor response to the brace by univariate analysis. The first three factors remained with high odds ratio for poor response by multivariate analysis. No adverse events occurred with the brace. CONCLUSION: Our newly developed compression brace contributed, at least in part, to improve the deformity of the secondary pectus carinatum. Further studies are required to clarify the therapeutic efficacy of anterior chest compression for secondary pectus carinatum.

Isolated left hepatic vein draining into the coronary sinus: a rare anomaly encountered during mitral valve surgery.

A left hepatic vein draining into the coronary sinus is an extremely rare congenital abnormality as a solitary cardiovascular malformation. We encountered the anomaly during the mitral valve surgery in a 72-year-old woman. The operation was successfully carried out using an additional suction directly in the left hepatic vein.

A combination of polyglycolic acid fabric and fibrin glue prevents air leakage from a lung defect.

OBJECTIVES: Air leakage after lung resection is a common morbidity that may lengthen hospital stay. Applying sealant to a lesion is an effective prophylaxis in clinical practice. This study aimed to examine the effect of a combination of a bioabsorbable polyglycolic acid (PGA) fabric and fibrin glue (FG) on air sealing by measuring the in vitro mechanical strength and degradation of the fabric, and in vivo histological changes after implantation. METHODS: A defect was created in the canine left upper lung lobe, and then filled with a fibrinogen solution and covered with a PGA sheet spray-coated with fibrinogen and thrombin. After 1 and 4 weeks, air leakage from the lesion was examined in vivo under airway pressure. Tissue samples were harvested for histological assessment. RESULTS: The mechanical strength of the PGA fabric remained at 80-90% of the baseline level for 1 week in phosphate-buffered saline, and then rapidly decreased to zero thereafter. Air leakage from the lung defect was prevented by the combination of PGA fabric and FG at 1 and 4 weeks. Histological examinations showed that PGA bundles persisted with a non-specific inflammatory response for 2 weeks and then gradually broke into sparse yarns surrounded by collagen fibres and capillaries by 8 weeks. The lung defect was filled with FG at 1 week and by granulation tissue thereafter. CONCLUSIONS: These results provide evidence for the efficacy of a combination of PGA fabric and FG for the prevention of air leakage in the critical period after lung surgery.

Megalin is downregulated via LPS-TNF-α-ERK1/2 signaling pathway in proximal tubule cells.

Expression and function of megalin, an endocytic receptor in proximal tubule cells (PTCs), are reduced in diabetic nephropathy, involved in the development of proteinuria/albuminuria. Lipopolysaccharide (LPS) is chronically increased in diabetic sera, by the mechanism called metabolic endotoxemia. We investigated low-level LPS-mediated signaling that regulates megalin expression in immortalized rat PTCs (IRPTCs). Incubation of the cells with LPS (10 ng/ml) for 48 h suppressed megalin protein expression and its endocytic function. TNF-α mRNA expression was increased by LPS treatment, and knockdown of the mRNA with siRNA inhibited LPS-mediated downregulation of megalin mRNA expression at the 24-h time point. Incubation of IRPTCs with exogenous TNF-α also suppressed megalin mRNA and protein expression at the 24- and 48-h time points, respectively. MEK1 inhibitor PD98059 competed partially but significantly TNF-α-mediated downregulation of megalin mRNA expression. Collectively, low-level LPS-mediated TNF-α-ERK1/2 signaling pathway is involved in downregulation of megalin expression in IRPTCs.

Neonatal repair of total anomalous pulmonary venous connection accompanied by unilateral lung agenesis and Goldenhar syndrome: a case report.

BACKGROUND: Total anomalous pulmonary venous connection accompanied by unilateral lung agenesis and Goldenhar syndrome is extremely rare. CASE PRESENTATION: We present a case of total anomalous pulmonary venous connection accompanied by unilateral lung agenesis and Goldenhar syndrome in a patient who was diagnosed based on transthoracic echocardiography and computed tomography. We observed complete absence of the lung, the bronchial tree, and vascular structures on the right side, with abnormal drainage of the left pulmonary veins into the innominate vein. The patient showed clear clinical evidence of pulmonary venous obstruction and underwent surgery 3 days after birth. The pulmonary venous chamber containing the vertical vein was anastomosed to the left atrium using 7-0 PDS running sutures via a median sternotomy. Echocardiography and computed tomography performed 1 year postoperatively revealed no pulmonary venous obstruction. CONCLUSION: We report a rare case of total anomalous pulmonary venous connection accompanied by unilateral lung agenesis and Goldenhar syndrome, which was successfully repaired 3 days after birth. A median sternotomy is a safe and effective approach for surgical repair of congenital heart disease with unilateral lung agenesis. Repair of the supra cardiac total anomalous pulmonary connection using the vertical vein is feasible in patients with a small pulmonary venous chamber.

Ideal esthetic and functional full-thickness lower eyelid "like with like" reconstruction using a combined Hughes flap and swing skin flap technique.

BACKGROUND: A goal in ideal lower eyelid reconstruction is to achieve not only an esthetic recovery of the shape of the eyelid but also to obtain satisfactory eyelid function without damage to the corneal surface. In this report, we introduce our "Like with Like" reconstruction technique using a combined Hughes flap and swing skin flap. The Hughes flap is a tarsoconjunctival pedicled flap from the upper eyelid, and the swing skin flap is a kind of transposition flap based on the orbicularis muscle pedicle. PATIENTS AND METHODS: Eight patients who had more than 25% of the lower eyelid affected by a full-thickness defect were included. There were five men and three women, and the average age was 68.4years old. All patients had undergone wide tumor excision of the lower eyelid due to a malignant tumor and had also had primary two-stage eyelid reconstruction with a combined Hughes flap and swing skin flap. RESULTS: The average reconstructive time was 1:20, and no postoperative severe complications such as flap necrosis or wound dehiscence were observed. The follow-up period was an average of one year, and there were no functional problems such as ectropion, lower eyelid retraction, lid margin hypertrophy, lacrimation disorder, or corneal problems observed in any cases. Esthetically, no reconstructed eyelid bulging or asymmetry in the lid margin was observed in any of the cases. CONCLUSIONS: Combining the Hughes flap and skin swing flap technique might be the best choice for a full-thickness lower eyelid defect with regard to the esthetic and functional results.

Reconstruction of the Congenital Divided Nevus of the Eyelids and Proposal of New Classification.

BACKGROUND: Divided nevus is a rare form of congenital nevus that usually occurs on both upper and lower eyelids of one eye, forming one large nevus when the eyelids are closed. Typically, congenital melanocytic nevi of the body are classified according to their size (small < 1.5 cm, medium 1.5-20 cm, and large > 20 cm). However, because divided nevus of the eyelids is usually relatively small, this classification may be too coarse and not adequate. Furthermore, there is no practical classification for divided nevus of eyelids yet. METHODS: We treated 3 cases of medium and large congenital divided nevus of the eyelids using local flaps based on the idea of esthetic subunits. Also, we proposed a new classification especially for congenital divided nevus of the eyelids based on the idea of esthetic subunits. RESULTS: All patients were treated successfully with the reconstructive idea of esthetic subunits for the eyelids. Case 1 (medium size) was reconstructed by orbicularis musculocutaneous flap and case 2 (large size) was reconstructed by expanded flap, and case 3 (medium size) was treated by modified V-Y orbicularis oculi musculocutaneous flap. In all cases, the flap color and texture were well matched and satisfactory results were obtained. Also, by using the new classification, we were able to plan practical scheme for divided nevus reconstruction. CONCLUSIONS: The use of our proposed classification and esthetic subunits for the divided nevus of the eyelids allows systematic surgical planning, leading to improved reconstructive outcomes.

Establishment of a Standardized Technique for Concha-type Microtia-How to Incorporate the Cartilage Frame into the Remnant Ear.

We have already reported surgical procedures for lobule-type microtia that provide an excellent contour and shape of the ear with minimum sacrifice of the donor. We have succeeded in establishing a standard surgical technique for almost all types of concha-type microtia that effectively uses the remnant ear and can use a unified costal cartilage frame. METHODS AND RESULTS: The concept of our technique is that remnant cartilage should be used maximally but that the deformed area should be completely replaced by the costal cartilage frame. The differences between the cartilage frame for lobule-type microtia and that for concha-type microtia are that the lower half beneath the antihelical area and the concha cymba in the base frame are omitted in concha-type microtia. The area from the tragus to the incisura of the tragus in the antihelical-tragal frame is also omitted. The area of the helical crus in the helical frame and the lower half in the antihelix are not immobilized in the base frame and are free edges. On the other hand, the remnant cartilage outside the concha is removed, but the antitragus is preserved. When the cartilage frame and the remnant are incorporated, all of the components of the ear can be provided. CONCLUSION: The ears created by our technique have a natural appearance and clear contour.

Correction of Lobule-type Microtia: Part 2: The Stage of Ear Elevation.

BACKGROUND: Here, we introduce our recent operative technique for ear elevation that results in (1) minimal morbidity for patients, (2) symmetric appearance, (3) clearer 3-dimensional structure with a deep concha, (4) good aesthetic appearance by hiding the grafted area behind the ear, and (5) maintenance of deep temporoauricular sulcus and angle. METHODS: After a skin incision, the ear is elevated with temporoparietal fascia underlying the cartilage. On the conchal area, undermining is performed just below the skin so that the deep concavity can be maintained. Scalp and neck skin behind the ear is undermined subcutaneously and lifted up cranially to hide the entire area of grafted skin behind the ear. The postauricular surface is covered by full-thickness skin from the lower abdomen. A protective splint is applied for 3 months while sleeping. RESULTS: A total of 137 ears in 121 patients were corrected with our technique and followed up for at least 3 years. All of the scar tissue could be hidden behind the ear, an aesthetically excellent result. CONCLUSIONS: Our technique made it possible to acquire an excellent and symmetrical shape of the ear. The important points in our procedure are as follows: (1) subcutaneous posterior undermining to enlarge the conchal cavity, (2) careful arrangement of the temporoauricular angle and auriculo-earlobe angle, (3) reduction in the area of temporally grafted skin to hide all scars behind the ear, and (4) protection of the ear to maintain the shape using a postoperative splint.

Regulation of megalin expression in cultured proximal tubule cells by angiotensin II type 1A receptor- and insulin-mediated signaling cross talk.

Impairment of proximal tubular endocytosis of glomerular-filtered proteins including albumin results in the development of proteinuria/albuminuria in patients with chronic kidney disease. However, the mechanisms regulating the proximal tubular function are largely unknown. This study aimed to investigate the role of angiotensin II type 1A receptor (AT(1A)R)- and insulin-mediated signaling pathways in regulating the expression of megalin, a multiligand endocytic receptor in proximal tubule cells (PTCs). Opossum kidney PTC-derived OK cells that stably express rat AT(1A)R but are deficient in endogenous angiotensin II receptors (AT(1A)R-OK cells) were used for this study. Treatment of the cells with angiotensin II suppressed mRNA and protein expression of megalin at 3- and 24-h incubation time points, respectively. Cellular uptake and degradation of albumin and receptor-associated protein, megalin's endocytic ligands were suppressed 24 h after angiotensin II treatment. The AT(1A)R-mediated decrease in megalin expression was partially prevented by ERK inhibitors. Insulin competed with the AT(1A)R-mediated ERK activation and decrease in megalin expression. Inhibitors of phosphatidylinositol 3-kinase (PI3K), a major component of insulin signaling, also suppressed megalin expression, and activation of the insulin receptor substrate (IRS)/PI3K system was prevented by angiotensin II. Collectively the AT(1A)R-mediated ERK signaling is involved in suppressing megalin expression in the OK cell line, and insulin competes with this pathway. Conversely, the insulin-IRS/PI3K signaling, with which angiotensin II competes, tends to stimulate megalin expression. In conclusion, there is AT(1A)R- and insulin-mediated competitive signaling cross talk to regulate megalin expression in cultured PTCs.

Megalin-mediated endocytosis of cystatin C in proximal tubule cells.

Serum levels of cystatin C, an endogenous cysteine proteinase inhibitor, are often used as an indicator of glomerular filtration rate. Although it is known that cystatin C is filtered by glomeruli and metabolized in proximal tubule cells (PTC), the precise molecular mechanism underlying this process is undetermined. Using quartz-crystal microbalance analyses, we demonstrate that cystatin C binds directly to megalin, an endocytic receptor in PTC, in a Ca(+)-dependent manner. We also find that cystatin C is endocytosed specifically via megalin in rat yolk sac epithelium-derived L2 cells which share a variety of characteristics with PTC. Finally, in vivo studies using kidney-specific megalin knockout mice provide evidence that megalin mediates proximal tubular uptake of cystatin C. We conclude that megalin is an endocytic receptor of cystatin C in PTC.