Sebnem Calkavur

Successful caspofungin treatment of multidrug resistant Candida parapsilosis septicaemia in an extremely low birth weight neonate.

Candida septicaemia with multidrug resistance is an uncommon event in preterm neonates. We present an extremely low birth weight infant (gestational age of 27 weeks, birth weight of 980 g) who developed congenital Candida parapsilosis septicaemia. Because the fungus was resistant both to amphotericin B and fluconazole, caspofungin was chosen for therapy. The fungus was successfully eradicated without any clinical or laboratory adverse effects.

A novel missense mutation of the bilirubin UDP-glucuronosyltransferase gene in a Turkish patient with Crigler-Najjar syndrome type 1.

Crigler-Najjar syndrome type 1 is a severe form of hereditary unconjugated hyperbilirubinemia caused by mutations in the bilirubin UDP-glucuronosyltransferase gene (UGT1A1) (1). Persons with this syndrome have homozygous or compound heterozygous nonsense mutations, frame shift mutations, or splicing site mutations (2,3); some have missense mutations (4). Here we report a novel missense mutation of UGT1A1 in a Turkish infant with Crigler-Najjar syndrome type 1.

Epidemic Adenoviral Keratoconjunctivitis Possibly Related to Ophthalmological Procedures in a Neonatal Intensive Care Unit: Lessons from an Outbreak

Purpose: Epidemic adenoviral keratoconjunctivitis can spread rapidly among preterm infants who frequently undergo ophthalmological examination. Here we present our experience on a nosocomial outbreak that affected 8 nursery staff members and 26 premature infants. We focus on the presentation and progress of the outbreak, the diagnosis of the disease and the measures taken for its control. Methods: Data were collected from patients’ files and records of the infection control team. Conjunctival swabs were collected to perform direct fluorescent assay (DFA) and viral culture. Diagnosis was made according to clinical evidence and/or detection of the virus. Statistical analysis was performed using SPSS 15.0 statistical software. Results: Infection was introduced to our unit after a laser photocoagulation procedure of a 28-week gestational infant and circulated rapidly within the unit due to direct transmission through contaminated medical equipment, fomites and hands of nursery staff members. Neither the patients, nor the nursery staff members developed systemic symptoms. While DFA tests were positive in seven infants, culture positivity could be demonstrated in only three infants. Contact and droplet precautions were implemented with the recommendation of the infection control team. No recurrence occurred after definition of the last case on the 32nd day. Conclusion: Ophthalmologic procedures continue to be a potential source of adenovirus outbreaks. However, negligence of contact measures during routine daily nursing care seems to be a more important contributing factor for rapid spread. Strict adherence to appropriate aseptic procedures is required to prevent this potentially hazardous infection in preterm infants.

Impact of haemodialysis on QTc dispersion in children

Background:  The purpose of the present paper was to investigate the corrected QT (QTc) interval and QTc dispersion value, and the impact of haemodialysis on these parameters in children with chronic renal failure.

Association between bronchopulmonary dysplasia and MBL2 and IL1-RN polymorphisms.

The imbalance between pro‐inflammatory and anti‐inflammatory cytokines may play a role in the development of bronchopulmonary dysplasia (BPD) in preterm infants. Mannose binding lectin (MBL) codon 54 and interleukin 1 receptor antagonist gene (IL1‐RN) polymorphisms cause genetic predisposition to increased risk of infection and inflammation, therefore may increase the risk of BPD. The aim of this study was to investigate the relationship between MBL, IL1‐RN gene polymorphisms and BPD development in preterm infants.

Role of Digoxin-Like Immunoreactive Substance in the Pathogenesis of Transient Tachypnea of Newborn

Background. Transient tachypnea of newborn (TTN) is usually observed in term or near-term infants. It constitutes an important part of the respiratory distress cases observed in the neonatal intensive care unit (NICU). Aim. This paper examines the effects of digoxin-like immunoreactive substance (DLIS) on fluid and ion balance, hemodynamic and echocardiographic parameters of neonates with TTN. Methods. Plasma DLIS, Na+, K+, urea, creatinine, serum and urine osmolarity, urine FeNa+, 24-hour urine output, echocardiographic investigation and mean blood pressure, and clinical parameters of disease severity were recorded in TTN group and compared with control on the 1st and 7th days of their lives. Results. Plasma DLIS levels were statistically higher in TTN group (0.66 ± 0.37 ng/mL) compared to control group (0.24 ± 0.20 ng/mL) both on the 1st day (P < 0.01) and the 7th day (P < 0.05). For TTN group, significant correlation was found between plasma DLIS levels and maximum respiratory rate, duration of tachypnea, and length of hospitalization on the 1st day. Plasma DLIS levels were correlated negatively with serum osmolarity levels. Plasma DLIS levels were positively correlated with urine output, urinary FeNa+ levels, cardiac output, left ventricles end diastolic diameters, and right ventricles end diastolic diameters. Conclusions. Increased DLIS levels were correlated with disease severity in cases with TTN. This increase may be a primary or secondary event in the disease progress. It may help reduce the fluid overload due to already disturbed cardiac functions in patients by increasing urine output and natriuresis; however it may also contribute to disease pathogenesis, by inhibiting alveolar Na+-K+-ATPase which further decreases fetal alveolar fluid resorption.

Can neonatal hepatitis be more fatal than biliary atresia

Background/Aims: The basic problem in diagnosis of neonatal cholestasis (NC) is to differentiate biliary atresia (BA) from other non-obstructive disorders. Because if bile flow cannot be provided by surgery, BA leads to cirrhosis and death within the first year of life. The aim of the present study is to determine histopathological features that may help to differentiate BA from neonatal hepatitis (NH). Material and Methods: This retrospective study was carried out on 105 liver biopsy specimens of 74 infants with NC who were diagnosed between 2003 and 2012. Results: The mean age was 76.5 ± 40.64 days. The most valuable biopsy findings for the discrimination between NH and BA, in decreasing order of importance, were ductular proliferation (p < 0.001), cholestasis in neoductuli (p < 0.001), fibrosis (p = 0.002), and extramedullar hematopoiesis (p = 0.02). While Kasai operations were performed in 19 cases, liver transplantation was performed in 10 cases. Survival rate among the death cases with BA was longer than the survival time of the death cases with NH (p = 0.023). Currently more children live with a close to normal quality of life with portoenterostomy and/or liver transplantation. On the contrary, NH can be more fatal with associated disorders such as growth retardation, specific infections, respiratory distress, and metabolic or endocrine diseases.

Is there a relationship between maternal periodontitis and pre-term birth? A prospective hospital-based case-control study

Abstract Objective. The aim of this study is to verify the existence of an association between maternal periodontal disease and pre-term delivery in an unselected population of post-partum Turkish women. Materials and methods. This case-control study was conducted on 100 women who gave birth in either a special or a government maternity hospital. The case group consisted of 50 mothers who had delivered an infant before 37 weeks’ gestation and weighed under 2500 g. The control group included 50 mothers who had given birth to an infant with a birth weight of more than 2500 g and a gestational age of ≥37 weeks. Data of mothers and infants were collected using medical registers and questionnaires. Clinical periodontal examinations were carried out in six sites on every tooth in the mother’s mouth. A participant who presented at least four teeth with one or more sites with a PPD ≥4 mm and CAL ≥3 mm at the same site was considered to have periodontal disease. Statistical methods included parametric and non-parametric tests and multiple logistic regression analysis. Results. There were no statistically significant differences between the cases and controls with regard to periodontal disease and pre-term delivery (OR = 1.48; 95% CI = 0.54–4.06). Conclusion. The findings indicated that maternal periodontitis was not a possible risk factor for pre-term delivery. Further studies with additional clinical trials are needed to explore the possible relationship between periodontal disease and pre-term birth.

Effect of adventitial dissection in brachiobasilic arteriovenous fistulae opened in children as vascular access for hemodialysis on patency and maturation of fistulae.

Background Recently, therapy of pediatric patients with chronic renal failure has been carried out using hemodialysis, peritoneal dialysis, or renal transplant. In this study, we prospectively investigated the role of adventitial dissection (performed on brachiobasilic arteriovenous fistulae in the antecubital area) in the patency rate, maturation, and early initiation of dialysis among pediatric cases undergoing chronic hemodialysis. Methods Thirty children (7 male and 23 females) were included in this study. They were operated on at the Department of Cardiovascular Surgery of Alsancak State Hospital and Dokuz Eylul University between March 2001 and June 2003. Their mean age was 12.3 ± 2.9 years (range 7-17 years), and their mean weight was 25.5 ± 7.9 kg (range 16-44 kg). Children who underwent only brachiobasilic arteriovenous fistula operation were included. Group 1 (n = 15) consisted of those who underwent adventitial dissection, and group 2 (n = 15) consisted of those not receiving the adventitial dissection procedure. Results The procedure was conducted by the same surgeon, and follow-up examinations were done at the Department of Pediatric Nephrology by investigators masked to treatment groups. Conclusion There were no significant differences in age, sex, existence of preoperative hypertension, diameter of vein measured preoperatively, and first day of dialysis between the groups. In 29 cases, a palpable thrill was noted at the operation site. Hemodialysis had been initiated after the fourth week, when the fistulae had matured. The mean duration of follow-up was 12.53 ± 8.98 months in group 1 and 11.85 ± 7.55 months in group 2 (p = .880). In group 1, one case developed fistula infection in the second month and one case developed lymphatic drainage. In group 2, one case developed early thrombosis, one case developed hematoma, and one case developed transient ischemia owing to arterial spasm in the ipsilateral limb. One case in each group developed minimal edema in the forearm, which was treated conservatively. The primary patency rate was 93.3% in both groups, whereas the secondary patency rate was 100% in group 1. Adventitial dissection performed on the vein during arteriovenous fistula formation does not result in any additional benefit with respect to fistula maturation, early initiation of dialysis, and patency. Among pediatric patients with chronic renal failure, in cases of inappropriate forearm veins, brachial arteriovenous fistulae performed with a meticulous surgical technique should be the procedure of choice because it is associated with minimal complications and a high patency rate.

A Histologically Diagnosed Case with Infantile Osteopetrosis Complicated by Hypopituitarism

Malignant infantile osteopetrosis is a rarely seen severe disorder which appears early in life with general sclerosis of the skeleton. It is caused by functionally defective osteoclasts which fail to resorb bone. Affected infants can exhibit a wide spectrum of clinical manifestations including impaired hematopoiesis, hepatosplenomegaly, visual impairment, and hypocalcemia. With the exception of secondary hyperparathyroidism, involvement of the endocrine system seems to be quite rare. Hypopituitarism is defined as underproduction of the growth hormone in combination with deficiencies of other pituitary hormones. Any lesion that damages hypothalamus, pituitary stalk, or anterior pituitary can cause secondary hypopituitarism. In this report, we presented a rare combination of malignant infantile osteopetrosis and secondary hypopituitarism in a newborn who presented predominantly with endocrinological symptoms. This is the first case report of malignant infantile osteopetrosis accompanied by hypopituitarism secondary to sclerosis of the sella turcica. On the other hand, this is a very interesting case which was diagnosed based on histological examination of bone marrow biopsy specimens despite lack of any clinical suspicion.