Tacrolimus-Eluting Suture Inhibits Neointimal Hyperplasia: An Experimental In Vivo Study in Rats.

OBJECTIVE/BACKGROUND: Neointimal hyperplasia (NIH) remains one of the leading causes of graft failure after vascular anastomoses. Cytotoxic drugs, such as rapamycin and tacrolimus, have been shown to inhibit the development of NIH. In this study, the aim was to test the impact of a sustained releasing tacrolimus-chitosan-eluting suture on the development of NIH in a rat model. METHODS: After tacrolimus-chitosan coating of a 7/0 polyvinylidene difluoride (PVDF) Trofilen® suture, the tacrolimus concentration on the coated suture and in vitro release trials were performed spectrophotometrically. Twelve Wistar rats were included. After midline laparotomy, a 7-8 mm longitudinal aortotomy in the infrarenal aorta was made and then closed by a bare 7/0 PVDF (group C, n = 6) and a 7/0 tacrolimus-chitosan coated PVDF suture (0.65 µg/cm tacrolimus [0.9 wt%] + 1.82 µg/cm chitosan [2.28 wt%]) (group T, n = 6). After 1 month, rats were sacrificed and aortotomy sites were examined histologically by ratio of intimal area (including neointima) and immunohistochemically by α-smooth muscle actin (ASMA) and proliferating cell nuclear antigen (PCNA) immunostaining. The PCNA positive cells were indexed to total cell number and expressed as percentage. RESULTS: In vitro tacrolimus release tests for a 7/0 tacrolimus-chitosan coated PVDF suture were confirmed for 1 month without an initial burst release. Endothelialisation over the aortotomy line occurred in both groups. The area of neointima was significantly reduced in group T compared with group C (ratio 0.22 ± 0.12 vs. 0.42 ± 0.11; p = .017) 1 month post-operatively. Likewise, the percentage of PCNA immunostaining significantly decreased in group C compared with group T (3.83 ± 2.85% vs. 11.17 ± 7.78%; p = .026). The cells constituting NIH were positive for ASMA immunostaining. CONCLUSIONS: Tacrolimus-chitosan-eluting suture is shown to be an effective way to reduce NIH without interfering with normal endothelialisation.

Results of pericardiectomy for constrictive pericarditis : Single-center experience.

BACKGROUND: We evaluated our early and late outcomes after pericardiectomy in patients with constrictive pericarditis (CP). PATIENTS AND METHODS: We retrospectively reviewed 31 patients who underwent pericardiectomy for CP from 1997 to 2015. Their mean age was 49.2 ± 18.5 years and 74.2 % of them were male. The vast majority had severe functional impairment (NYHA class III-IV) with a mean duration of symptoms of 14.2 ± 10.1 months. RESULTS: Early mortality was 9.7 %: n = 3; multiorgan failure (MOF) in 1, respiratory failure in 1, and left heart failure in 1. Preoperative systolic pulmonary artery pressure over 60 mmHg (p = 0.038, odds ratio [OR] = 0.12) and postoperative low cardiac output syndrome (p = 0.005, OR = 13.5) were significant predictors of early mortality in univariate analysis. Mean follow-up time was 57.8 ± 61.9 months (4-216 months). Late mortality was 6.8 % (2/28 patients) and the cause was MOF secondary to end-stage right heart failure. In Kaplan-Meier analyses, actuarial (including early mortality) and event-free survival rates were 83.9 and 51.1 % at 216 months, respectively. At the end of follow-up, the majority of patients (23/26, 92.9 %) were in good functional status (NYHA class I-II). There were fewer patients under diuretic therapy in the postoperative than in the preoperative period; however, the difference was not statistically significant (12/31 vs. 4/26, p = 0.76). There was no significant difference between the preoperative and follow-up tricuspid annular plane systolic excursion values (15.5 ± 2.2 and 16.6 ± 2.2 mm, respectively, p = 0.088). Left ventricular systolic function was preserved in all patients postoperatively. CONCLUSION: Although early mortality after pericardiectomy remains high, the procedure provides significant improvement in functional status in the long term.

A NEONATE PRESENTING WITH GRACILE SYNDROME AND BJORNSTAD PHENOTYPE ASSOCIATED WITH BCS1L MUTATION.

GRACILE Syndrome, is an autosomal recessive disease presenting with growth retardation, severe lactic acidosis, Fanconi type tubulopathy, cholestasis, iron overload and early death without any dysmorphological or neurological features. The BCSIL gene mutation is responsible for GRACILE syndrome, Bjornstad syndrome and complex III deficiency. Bjomstad syndrome is characterized by sensorineural hearing loss and abnormal flat twisted hair shafts. The case is GRACILE syndrome with Bjomstad phenotype in neonatal period due to BCSL1 gene mutation.

Crigler-Najjar syndrome type I in a Turkish newborn caused by a novel mutation and Gilbert type genetic defect.

Crigler-Najjar syndrome (CNS), caused by deficiency of bilirubin uridine diphosphate glucuronosyltransferase (UGT) 1A1, is a rare and autosomal recessive inherited disorder characterized by severe unconjugated nonhemolytic hyperbilirubinemia since birth. We present a girl with CNS type I caused by a novel mutation and Gilbert type genetic defect. Gilbert's Syndrome (GS) and CNS type I both involve abnormalities in bilirubin conjugation secondary to deficiency of bilirubin UGT. The combined defects even in benign genetic forms were shown to cause more serious clinical disease. The patient has been treated with daily home-based phototherapy for more than nine months and considered as a candidate for liver transplantation.

Assessment of novel biomarkers: sTREM-1, pentraxin-3 and pro-adrenomedullin in the early diagnosis of neonatal early onset sepsis.

BACKGROUND: Early onset bacterial sepsis in neonates (EOS) is recognized as an important health condition. Early diagnosis is crucial. However, blood culture results are released in 48-72 hours. Many biomarkers have been investigated but none have been accepted as the gold standard. This study aimed to investigate the diagnostic value of the molecules: soluble form of triggering receptor expressed on myeloid cells-1 (sTREM-1), pentraxin-3 (PTX-3) and pro adrenomedullin (pro-ADM) in EOS and compare with currently used biomarkers. METHODS: In this multicenter prospective study, patients were enrolled from different NICUs around the Turkey. Patient data were collected via web-based registry system from attending centers. Neonates, hospitalized with a suspicion of EOS were enrolled. Blood culture and routine blood tests were collected and a serum sample was obtained and kept in - 80°C for studying the molecules. According to laboratory results, patients were divided into three groups as; proven sepsis, clinical sepsis and control group. Groups were compared in terms of demographic, clinical and laboratory findings. The primary outcome of the study was to assess any difference between groups in terms of the diagnostic value of the markers aforementioned. RESULTS: A total of 130 patients were enrolled; proven sepsis (n = 36), clinical sepsis (n = 53) and control (n = 41) groups. Groups were similar in terms of demographic findings; mean WBC (P = 0.445), procalcitonin (PCT) (P = 0.083) and IL-6 (P = 0.814) levels. Mean C-reactive protein (CRP) level was significantly higher in clinical sepsis and proven sepsis groups compared to control group (P < 0.001). Mean PTX-3 (P = 0.547), pro-ADM (P = 0.766) and sTREM-1 (P = 0.838) levels were similar between groups. CONCLUSION: These promising molecules failed to help in early diagnosis of EOS. Their relation to correlation with disease progression may make more sense as they seem to be expressed in higher amounts with the progression of the disease in previous studies. CRP was the most frequently used biomarker for detecting the sepsis in our study population.

Hyperekplexia in a neonate: a novel finding in Fukuyama type congenital muscular dystrophy.

The Fukuyama type congenital muscular dystrophy (FCMD) is a rare autosomal recessive disorder characterized by cranial, cerebellar and ocular malformations and congenital muscular dystrophy. Hyperekplexia is characterized by transient, generalized rigidity in response to unexpected loud noises or sudden tactile stimulation. Herein, we report an infant who had typical clinical features of FCMD with hyperekplexia. Our purpose is to draw attention to this first report of concomitant FCMD and hyperekplexia.

Median sternotomy for an unexpected complication of permanent hemodialysis catheters: "stuck catheter".

Cuffed tunneled venous access catheters are commonly used for temporary and permanent access in patients undergoing hemodialysis. These catheters play an essential role in providing permanent access in patients in whom all other access options have been exhausted. However, they are prone to several complications like catheter thrombosis, catheter fibrin sheating and infection. Herein, we report two uncommon cases of stuck hemodialysis cuffed tunneled catheters causing stenosis and thrombosis in central veins which needed to be removed by median sternotomy.

Ophthalmo-acromelic syndrome: report and review.

The ophthalmo-acromelic syndrome of Waardenburg is an autosomal recessive trait comprising eye malformations ranging from true anophthalmia to mild microphthalmia with acromelic malformations. Some 29 affected individuals have been reported since Waardenburg's first report in 1935 [Waardenburg et al., 1961]. We report on a new case with bilateral anophthalmia and typical limb malformations. The patient also was found to have interruption of the inferior vena cava with azygos continuation as an additional finding. The previous reports are reviewed to elucidate the spectrum of the syndrome.

An adult case of Bland White Garland syndrome with huge right coronary aneurysm.

We report the case of a 62-year-old male patient with left main coronary artery originating from the pulmonary trunk, severe mitral insufficiency, and huge right coronary artery aneurysm. He is the oldest such patient among those reported in the literature, surviving to the sixth decade without any anginal symptoms. He is also the first such case with such a huge and calcified right coronary artery aneurysm and a prominent collateral from the noncoronary circulation.

Management of central diabetes insipidus with oral desmopressin in a premature neonate.

The effect of oral administration of desmopressin (DDAVP) solution was investigated in a very low birth weight premature infant with central diabetes insipidus that was associated with grade four germinal matrix hemorrhage. As an alternative to the nasal route, long-term successful management resulting in favorable growth and development during infancy was achieved using the oral route.

Congenital early onset isolated adrenocorticotropin deficiency associated with a TPIT gene mutation.

TPIT is a highly cell-restricted transcription factor that is required for the expression of the propiomelanocortin (POMC) gene and for terminal differentiation of the pituitary corticotroph lineage. Its exclusive expression in pituitary POMC-expressing cells has suggested that its mutation may cause isolated deficiency of pituitary ACTH. We present a neonate with the diagnosis of congenital early onset isolated ACTH deficiency (IAD) associated with a loss of POMC function as a result of a missense mutation in the TPIT gene. A 5 day-old male infant was admitted for hypoglycemia, limpness and conjugated hyperbilirubinemia. Laboratory investigations indicated low plasma cortisol concentration (0.1 microg/dl) accompanying a very low ACTH (<5 pg/ml) concentration. An increase in plasma cortisol concentration following stimulation with low dose exogenous ACTH was observed. On replacement therapy with hydrocortisone (15 mg/m2/day orally), cholestatic jaundice and hypoglycemia resolved and subsequent normal growth (weight, height and head circumference, 25th, 10th and 50th percentile, respectively) and development was achieved without recurrence of hypoglycemic episodes.

Coarctation of the aorta in infants and neonates: results and assessment of prognostic variables.

From 1984 until 1994, 75 consecutive patients younger than 12 months of age were operated on for coarctation of the aorta. We retrospectively analyzed predictive factors for morbidity and mortality, and also interaction between surgical procedures and recoarctation. Surgical procedures were as follows: resection with a traditional end-to-end (E-E) anastomosis in 55 patients (73.3%), prosthetic patch aortoplasty (PPA) in 12 patients (16%) and subclavian flap aortoplasty (SFA) in 8 patients (10.7%). Early mortality was 9.33% (7 patients). Logistic regression analysis proved that age at operation, associated anomalies of heart, type of coarctation, aortic arch hypoplasia and pulmonary banding were independent predictors of hospital death. Late mortality occurred in 7 patients (10.3%). Associated anomalies of heart were an independent prognostic factor for late mortality. Actuarial freedom from recoarctation at 1 year was 91% [confidence limits (CL): 82% to 97%] and 5 years were 74% (CL: 67% to 86%). Immediate postrepair gradient was equal after E-E anastomosis and other procedures. We conclude that the treatment of first choice in the management of coarctation of the aorta in infants is E-E anastomosis.

Pulmonary vascular sling responsible for esophageal and tracheal obstruction. A case report.

The disease of pulmonary vascular sling is a rare congenital condition. When it does occur it is often in conjunction with tracheal compression. The authors report herein a newborn with this anomaly presenting with a tracheal and esophageal compression.

A simple management of mediastinitis.

Between January 1, 1986 and December 31, 1993, 6940 open heart procedures were performed via median sternotomy at Hacettepe University Medical Center. Mediastinitis developed in 55 patients (0.79%). Twenty-four were children. All were reoperated for treatment of mediastinitis. Following radical debridment 17 were irrigated with dilute povidone-iodine solution after primary reclosure (Group A), the remaining 38 were simply reclosed while leaving just drains in the mediastinum (Group B). Hospital mortality was 17.65% in Group A and 15.79% in Group B. Time of hospitalization after treatment in living patients was 17.6 days in Group A and 16.3 days in Group B. A mortality of 15.79% is an acceptable result which shows early radical debridment and drainage of the mediastinum is the most important cornerstone of the treatment of mediastinitis and is mostly enough with proper systemic antibiotic therapy.

Effects of oral and intramuscular vitamin K prophylaxis on PIVKA-II assay parameters in breastfed infants in Turkey.

Protein induced by vitamin K absence (PIVKA-II) has been used for the evaluation of vitamin K deficiency in the newborn. Differing PIVKA-II detection rates in various studies on hemorrhagic disease of the newborn have not been explained satisfactorily. In this study we investigated the PIVKA-II values of 44 healthy breastfed infants, of whom 29 received vitamin K1 either orally (N = 13) or intramuscularly (n = 16), and the remaining 15 constituted the control group. PIVKA-II was detected in 15.3 percent (2/13) of the oral and 25 percent of the (4/16) intramuscular group on the third day of life. The detection rate was 93.3 percent (14/15) in the control group. However, at the one-month follow-up, there were no PIVKA-II positive infants. In conclusion, PIVKA-II positivity among breastfed Turkish infants on the third day of life was high compared to that in other studies, perhaps due to a delay in enzyme maturation related to racial, environmental and nutritional factors.

The influence of pulmonary insufficiency on ventricular function following total correction of Fallot's tetralogy: evaluation using radionuclide ventriculography and clinical findings.

Long-standing pulmonary insufficiency after repair of tetralogy of Fallot may adversely affect ventricular function. We evaluated 20 patients postoperatively by radionuclide ventriculography and clinical findings after total correction of tetralogy of Fallot. Patients were divided into two groups as follows: Group I patients (10) had no pulmonary insufficiency; Group II patients (10) had moderate or severe pulmonary insufficiency. Preoperatively, there was no difference between groups in terms of age, functional capacity according to the New York Heart Association criteria, hemoglobin and hematocrit level, cardiothoracic ratio, McGoon ratio, left and right ventricular ejection fraction, cardiac output or cardiac index. Postoperatively, right ventricular ejection fraction was 40.10 +/- 2.28 in Group I and 29.5 +/- 2.86 in Group II, p < 0.01. Left ventricular ejection fraction was 59.3 +/- 2.90 in Group I and 50.9 +/- 4.19 in Group II, p < 0.01. Radionuclide ventriculography is a useful means of identifying right ventricular dysfunction following repair of tetralogy of Fallot. The dysfunction appears significantly worse in patients with pulmonary insufficiency.

Total correction for tetralogy of Fallot after Brock-type operation.

We present a case of tetralogy of Fallot who had previously undergone a graded Brock-type operation. The post-operative development of pulmonary arteries and pulmonary trunk after the Brock operation were very suitable for total correction, which was then performed successfully. In contrast to shunt, in Brock-type operations enlargement of the outflow tract induces symmetrical growth of hypoplastic pulmonary arteries without the risk of acquired pulmonary obstruction or peripheral stenosis at the site of anastomosis. After palliative operations, the repair can be deferred until the pulmonary arteries are of a suitable size.

Congenital double-orifice mitral valve. A case report.

A three-year-old girl was admitted to Hacettepe Children's Hospital due to sweating and poor feeding. Echocardiography demonstrated ostium primum atrial septal defect with a cleft mitral valve. After two months of medical follow-up with digitalis, she was subjected to open-heart surgery, and a primum-type atrial septal defect with double mitral orifice was found. To avoid creating acute mitral stenosis, the cleft was only partially sutured and the septal defect was closed with a patch. Her postoperative recovery was uneventful. It is emphasized that surgical intervention should be individualized in each case.

The effect of asphyxia on gut blood flow in term neonates.

Transcutaneous Doppler ultrasound measurements were made of the superior mesenteric artery of 25 term infants to correlate the intestinal blood flow with neonatal acid-base status as measured in umbilical artery blood and newborn condition as reflected by Apgar score. Compared with the babies whose umbilical artery pH was higher than 7.20 (peak systolic velocity: 84.1 cn/sn, mean velocity 48.6 cm/sn), the blood flow of superior mesenteric artery was significantly decreased in the newborn babies whose umbilical artery pH was less than 7.20 (peak systolic velocity 48.8 cm/sn, mean velocity = 32.6 cm/sn). Although no such relationship existed between Apgar scores and superior mesenteric artery blood flow indices, a significant linear correlation between superior mesenteric artery blood flow and umbilical artery pH was noted. A reduction of intestinal circulation was observed during umbilical cord blood acidemia and hypoxemia.