Ergin Çiftçi

Underlying causes of recurrent pneumonia in Turkish children in a university hospital.

The aim of this study was to determine the relative frequency of underlying illnesses for recurrent pneumonia in children. Children who had two or more episodes of pneumonia per year, or three or more episodes in a lifetime were investigated retrospectively at Ankara University Medical School, Department of Pediatric Infectious Diseases, between January 1997 and October 2002. Out of 788 children hospitalized for pneumonia, 71 (9 per cent) met the criteria for recurrent pneumonia. An underlying illness was demonstrated in 60 patients (85 per cent). In this group, the underlying illness was diagnosed prior to pneumonia in 11 patients (18.3 per cent), during the first episode in 12 patients (20 per cent), and during recurrence in 37 patients (61.7 per cent). Underlying diseases were bronchial asthma (32 per cent), gastroesophageal reflux (15 per cent), immune disorders (10 per cent), congenital heart defects (9 per cent), anomalies of the chest and lung (6 per cent), bronchopulmonary dysplasia (4 per cent), cystic fibrosis (3 per cent), tuberculosis (3 per cent), and aspiration syndrome (3 per cent). No predisposing illness could be demonstrated in 11 patients (15 per cent). In conclusion, approximately one-tenth of hospitalized children with pneumonia in our hospital had recurrent pneumonia. Most of these children had an underlying illness, which was demonstrated by intensive investigation. Bronchial asthma in children aged more than 2 years and gastroesophageal reflux in children aged less than 1 year were the most common underlying illnesses for recurrent pneumonia.

Familial neonatal Marfan syndrome due to parental mosaicism of a missense mutation in the FBN1 gene

We present a family in which three siblings were born with neonatal Marfan syndrome (MFS) to unaffected parents. The clinical findings included joint contractures, large ears, loose skin, ectopia lentis, muscular hypoplasia, aortic root dilatation, mitral and tricuspid valve insufficiency, and pulmonary emphysema. All three siblings died due to cardiorespiratory insufficiency by 2–4 months of age. Screening of the FBN1 gene showed the heterozygous c.3257G > A (p.Cys1086Tyr) mutation in the proband. Mosaicism of the mutation was demonstrated in the somatic cells and in the germ line of the father. Although three examples of parental mosaicism for classical MFS were demonstrated previously, this is the first report of familial occurrence of neonatal MFS due to a heterozygous mutation in FBN1. In conclusion, the p.Cys1086Tyr mutation in FBN1 is consistently associated with neonatal MFS. Parental mosaicism should always be kept in mind when counseling families with MFS.

Pyrexia of unknown origin in children: a review of 102 patients from Turkey

Abstract Pyrexia of unknown origin (PUO) has not been appropriately investigated in Turkish children and therefore a study was undertaken to determine the causes of PUO and to evaluate which clinical procedures are useful in establishing a diagnosis. A total of 102 children fitting the classical PUO criteria seen in our clinic between 1995 and 2002 were investigated retrospectively. Infections, collagen vascular disorders, malignancy and miscellaneous conditions constituted 44.2%, 6.8%, 11.7% and 24.5% of cases, respectively, while 12.8% of the cases remained undiagnosed. Enteric fever, brucellosis and respiratory tract infections were the most commonly encountered infections, whereas familial Mediterranean fever was the commonest non-infectious disorder. Biopsy, aspiration, serology, bacteriology, radiology and observation of the clinical course were the most useful diagnostic procedures.

Successful treatment of central venous catheter infection due to Candida lipolytica by caspofungin-lock therapy.

Fungal infections, mainly represented by bloodstream infections (BSI) due to Candida spp., have maintained a constant incidence rate over the past 10 years, representing around 10% of catheter-related BSI (Kojic EM et al., Clin Microbial Rev 2004; 17: 255–67). The Infectious Diseases Society of America (IDSA) guidelines on Candida catheter-related BSI recommend the systemic antifungal therapy and catheter removal (Pappas PG et al., Clin Infect Dis 2009; 48: 503–35). This is warranted both by the ability of Candida spp. to form biofilms that greatly reduce antifungal activity and by the extremely high risk of metastatic infections, particularly endocarditis and retinitis (Viale P et al., J Chemother 2006; 18: 235–49). In clinical practice, catheter removal is not always easily performed, depending both on type of device (for tunnelled or totally implanted catheters, a surgical procedure is needed) and on the patient s condition (e.g. patients with severe platelet or coagulation factor deficit). For these reasons, antifungal-lock technique (ALT) alternative to the device removal is proposed (Mermel LA et al., Clin Infect Dis 2001; 32: 1249–72). ALT consists of catheter lumen replenishment by a selected antimicrobial agent and then locking it for a time to eradicate the microbes embedded in endoluminal biofilms (Angel-Moreno A et al., J Infect 2005; 51: e85–87). Treatment of catheter-related BSI due to Candida spp. with ALT has been tried in few cases (Mermel LA et al., Clin Infect Dis 2001; 32: 1249–72; Buckler BS et al., Pediatr Infect Dis J 2008; 27: 762–4; Arnow P et al., Am J Med 1991; 90: 128–30; Viale P et al., Clin Infect Dis 2001; 33: 1947–8; Benoit JL et al., Clin Infect Dis 1995; 21: 1286–8). Herein, to our knowledge, we report the first case of non-complicated Candida lipolytica fungaemia related to a Hickman catheter cured with intraluminal caspofungin in addition to systemic caspofungin therapy.

Nasopharyngeal colonization with penicillin-resistant Streptococcus pneumoniae in Turkish children.

Abstract Background: Streptococcus pneumoniae is one of the major infectious agents observed in children. In spite of the fact that penicillin is preferred in the treatment of infections caused by S. pneumoniae, there has been a world‐wide increase in the frequency of penicillin‐resistant S. pneumoniae.

Old agent, new experience: colistin use in the paediatric Intensive Care Unit—a multicentre study

Nosocomial infections caused by multidrug-resistant (MDR) microorganisms are a common problem around the world, especially in Intensive Care Units. The aim of this study was to investigate the efficacy and safety of colistin therapy in paediatric patients with severe nosocomial infections caused by MDR Gram-negative bacteria. There were 87 episodes in 79 paediatric Intensive Care Unit patients in five different hospitals; each patient was treated intravenously with colistin and evaluated. Of the 79 patients, 54.4% were male and the median age was 30 months. The most commonly isolated microorganism was Acinetobacter baumannii, the most common isolation site was tracheal aspirate fluid and the most common type of infection was ventilator-associated pneumonia. The mean colistin dose in patients without renal failure was 5.4 ± 0.6 mg/kg/day, the mean therapy duration was 17.2 ± 8.4 days and the favourable outcome rate was 83.9%. Serious side effects were seen in four patient episodes (4.6%) during therapy; two patients suffered renal failure and the others had convulsive seizures. Other patients tolerated the drug well. The infection-related mortality rate was 11.5% and the probability of death within the first 9 days of treatment was 10 times higher than after the first 9 days. In conclusion, this study suggests that colistin is effective in the treatment of severe nosocomial infections caused by MDR Gram-negative bacteria and is generally well tolerated by patients, even after relatively long-term use.

Clinical and Epidemiological Characteristics of Children with Kawasaki Disease in Turkey

BACKGROUND Kawasaki disease (KD) is the leading cause of acquired heart disease in childhood in the developed countries. The objective of this study is to describe the clinical and epidemiological characteristics of children with KD in Turkey. METHODS The medical records of 24 patients treated for KD between January 1994 and June 2009 at Ankara University Medical School, Turkey were reviewed. RESULTS The male-to-female ratio was 1.4 : 1. The median age at diagnosis was 2 years (range: 6.5 months to 11 years). Conjunctivitis and changes in the lips and oral cavity were seen in 21/24 (87.5%), cervical lymphadenopathy 17/24 (70.8%), polymorphous rash 16/24 (66.7%) and peripheral changes in 12/24 (50%). Coronary artery abnormality (CAA) was observed in 8/24 (33.3%) cases. CAA was seen in both the complete and incomplete groups with similar frequency (31.3% vs. 37.5%, respectively). CONCLUSIONS KD must be kept in mind in the differential diagnosis of infants with prolonged fever.

Caspofungin treatment in two infants with persistent fungaemia due to Candida lipolytica

Candida lipolytica has infrequently been identified as a cause of infection and is associated mostly with vascular catheter-related fungaemia. Patients reported in the literature have been successfully treated with catheter removal or amphotericin B treatment. We report 2 infants with C. lipolytica fungaemia unresponsive to catheter removal and amphotericin B therapy and treated successfully with the addition of caspofungin to amphotericin B.

Ligneous conjunctivitis, hydrocephalus, hydrocele, and pulmonary involvement in a child with homozygous type I plasminogen deficiency

Ligneous conjunctivitis is a rare and unusual form of chronic pseudomembranous conjunctivitis which usually starts in early infancy. Plasminogen deficiency has recently been associated with ligneous conjunctivitis. The disease may be associated with pseudomembranous lesions of other mucous membranes in the mouth, nasopharynx, trachea, and female genital tract and also with congenital hydrocephalus. In this report, a 1-month-old Turkish boy who had pseudomembranous conjunctivitis, occlusive hydrocephalus, and hydrocele is presented. After surgery for ventriculo-peritoneal shunt establishment, he developed inspiratory stridor, respiratory distress, and pulmonary atelectasis. Tracheal pseudomembranes were also demonstrated by bronchoscopy. Plasminogen antigen level and plasminogen activity were very low. Genomic DNA from the patient was screened for mutations in the plasminogen gene and a homozygous L650fsX652 mutation (deletion of 2081C) was detected. Both of his parents were heterozygous for this mutation. He died due to respiratory failure during follow-up. Conclusion: ligneous conjunctivitis related to type I plasminogen deficiency is relatively common in the Turkish population, however, mutations are heterogeneous and a common founder is unlikely.

The use of colistin in critically ill children in a pediatric intensive care unit.

Background: Colistin is active against most multidrug-resistant, aerobic Gram-negative bacteria. Because of the reported nephrotoxicity during the first years of use of colistin, there were concerns of its use in pediatrics where there was limited experience The aim of this study is to document the clinical characteristics and outcomes of use of colistin in pediatric patients at a pediatric intensive care unit in Turkey. Methods: We reviewed the medical and laboratory records of 29 critically ill children who were treated with colistin for 38 courses between January 2011 and December 2011 at the Department of Pediatric Intensive Care Unit in Ankara University Medical School, Turkey. Results: The median age was 17 months (range 3–217 months). Male-to-female ratio was 1:1.37. Ventilator-associated pneumonia (21 courses) was the leading diagnosis followed by catheter-related blood stream infection (6 courses), bacteremia (4 courses), ventriculoperitoneal shunt infection, peritonitis and pneumonia (1 course). The most commonly isolated microorganisms were Acinetobacter baumanni, Pseudomonas aeruginosa, Klebsiella pneumoniae, Serratia marcescens, Stenotrophomonas maltophilia, and Enterobacter cloacae. Two colistin formulations were used. Colimycin (Kocak Farma) was used in 21 colistin treatment episodes. The median dosage of colistin in this group was 5.0 mg/kg/d (2.3–5.6 mg/kg/d). Colomycin (Forest Laboratories) was used in 17 colistin treatment episodes. The median dosage of colistin in the second group was 75,000 International Unit/kg/d (50,000–80,000 International Unit/kg/d). Thirty colistin treatment episodes (79%) had a good or partial clinical response and 8 (21%) had a poor clinical response. Of the 8 colistin treatment episodes with poor clinical response, 3 were in the Colimycin group and 5 were in the Colomycin group. Ten patients died. There was no evidence of neurotoxicity in this study. Nephrotoxicity was observed in 1 patient but was not attributed to colistin because the patient had multiorgan failure at the same time. Conclusions: This study in a small cohort of patients suggests that the use of colistin in severe nosocomial infections caused by multidrug-resistant Gram-negative bacteria is well-tolerated and efficacious.