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.

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.

Severe postnatal cytomegalovirus infection with multisystem involvement in an extremely low birth weight infant.

Cytomegalovirus (CMV) infection is the most common intrauterine and perinatal viral infection. Postnatal CMV infection is acquired mainly from breast milk and may cause severe illness in preterm infants. We report an extremely low birth weight infant who presented with a sepsis-like syndrome and multiple organ involvement, notably hepatitis and pneumonitis, and treated with ganciclovir without adverse effect or relapse.

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.

Tricuspid valve replacement: an analysis of risk factors and outcomes.

BACKGROUND: Tricuspid valve replacement (TVR) is rarely performed and is associated with a high morbidity and mortality. We report our experience with TVR and related adverse events. METHODS: Between January 1996 and December 2007, 35 patients underwent TVR with mechanical (n = 33) or bioprosthetic (n = 2) valves. Twenty-nine patients underwent concomitant cardiac procedures. RESULTS: All patients completed follow-up (mean 47 months). Thirty-day mortality was 20 % (n = 7). Risk factors included perioperative low arterial blood pressure ( P = 0.000), New York Heart Association (NYHA) functional class III or IV ( P = 0.001), severe pulmonary hypertension (pulmonary arterial pressure greater than 60 mmHg) ( P = 0.000), hepatic dysfunction ( P = 0.000), ascites ( P = 0.003), and reoperation ( P = 0.015). Late mortality occurred in five patients. Valve-related complications included bleeding (n = 1) and stroke (n = 1). Kaplan-Meier estimates of 1-, 5- and 10-year survival (including early mortality) and event-free survival were 77.1 %, 60 %, and 54.3 % and 91.1 %, 80.6 %, and 55.9 %, respectively. Severe pulmonary hypertension was the only predictor of late mortality ( P = 0.001). Among survivors, the mean NYHA class improved from 2.8 to 1.1 ( P = 0.000). CONCLUSIONS: Although early outcome after TVR is suboptimal, long-term survival and functional improvement is satisfactory.

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.

The impact of individual room on rehospitalization and health service utilization in preterms after discharge.

AIM: To compare individual room implemented family-centred care to classical designed neonatal intensive care unit and find out its effect on rehospitalization and application to health services in preterm infants after discharge. METHODS: Mothers whose infants were born before 34 gestational weeks and hospitalized for at least one week in the NICU were enrolled in the study. Mothers who were hospitalized with their preterm infants in individual rooms (Group I) were compared with mothers who were not hospitalized with their preterm infants (Group II). After the third postdischarge month, groups were compared for their rates of phone consultations to physician/hospital, acute care applications, rehospitalization and parent's perception of child's vulnerability. RESULTS: Although demographic and medical information did not indicate any differences between the groups, the mean number of acute care visits (p = 0.046), the median number of phone consultations (p = 0.001) and rehospitalization rate (12.9% vs. 34.5%, p < 0.05) were significantly higher in Group II. The anatomical problems, such as inguinal hernia and retinopathy of prematurity, were the main recorded causes in Group I whereas problems related to prematurity like feeding difficulties were dominating in Group II. CONCLUSION: The availability of individual rooms that allows maternal presence and participation during the hospitalization of the mother's preterm infant, is correlated with lower rates of rehospitalization and healthcare applications.

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.

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.

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.