Ali Ertug Arslankoylu

Critically ill children with pandemic influenza (H1N1) in pediatric intensive care units in Turkey.

Objectives: To outline the epidemiologic features, clinical presentation, clinical courses, and outcomes in critically ill children with pandemic influenza in pediatric intensive care units. Design: Retrospective, observational, multicenter study. Setting: Thirteen tertiary pediatric intensive care units in Turkey. Patients: Eighty-three children with confirmed infection attributable to pandemic influenza detected by reverse-transcriptase polymerase chain reaction assay between November 1 and December 31, 2009 who were admitted to critical care units. Interventions: None. Measurements and Main Results: During a 2-month period, 532 children were hospitalized with pandemic influenza and 83 (15.6%) needed critical care. For the 83 patients requiring critical care, the median age was 42 (range, 2–204) months, with 24 (28.9%) and 48 (57.8%) of patients younger than 2 and 5 yrs, respectively. Twenty (24.1%) patients had no underlying illness, but 63 (75.9%) children had an underlying chronic illness. Indications for admission to the pediatric intensive care unit were respiratory failure in 66 (79.5%), neurologic deterioration in six (7.2%), and gastrointestinal symptoms in five (6.0%) patients. Acute lung injury was diagnosed in 23 (27.7%), acute respiratory distress syndrome was diagnosed in 34 (41%), and 51 (61.4%) patients were mechanically ventilated. Oseltamivir was used in 80 (96%) patients. The mortality rate for children with pandemic influenza 2009 was 30.1% compared to an overall mortality rate of 13.7% (p = .0016) among pediatric intensive care unit patients without pandemic influenza during the study period. Also, the mortality rate was 31.7% in patients with comorbidities and 25.0% in previously healthy children (p = .567). The cause of death was primary pandemic influenza infection in 16 (64%), nosocomial infection in four (16%), and primary disease progression in five (20%) patients. The odds ratio for respiratory failure was 14.7 (95% confidence interval, 1.85–111.11), and odds ratio for mechanical ventilation was 27.7 (95% confidence interval, 0.003–200). Conclusions: Severe disease and high mortality rates were seen in children with pandemic influenza. Death attributable to pandemic influenza occurred in all age groups of children with or without underlying illness. Multiple organ dysfunction syndrome is associated with increased mortality, and death is frequently secondary to severe lung infection caused by pandemic influenza.

Bilateral adrenal cystic neuroblastoma with massive hepatomegaly and intracystic hemorrhage

To The Editor: Cystic neuroblastoma (CNB) is a rare form of this tumor which is characterized by a large cystic lesion and microscopic cysts, and is frequently located in the adrenal gland. It has a little tendency to metastasis and it has an excellent prognosis if early diagnosis can be made [1–10]. Furthermore, bilateral adrenal CNB is an extremely unusual presentation of NB which may represent a second primary tumor or a contralateral metastasis [4]. Most of the patients with CNB are under 1 year of age. Moreover, massive intratumoral hemorrhage, hepatic metastases, and advanced clinical stage are also rare clinical findings. The differential diagnosis of a cystic suprarenal mass is difficult since it occurs not only in CNB, but also in adrenal hemorrhage, enteric cyst, extralobar sequestration, dilatation of upper-pole renal calyces, congenital adrenal cyst, cystic Wilms tumor, and adrenal abscess [2–4]. A 19-day-old girl, being born in another hospital, was admitted to our hospital with the symptoms of paleness, respiratory distress, and abdominal distention. Physical examination revealed tachycardia, tachypnea, hepatomegaly, and a large left-sided abdominal mass. Laboratory findings were as follows: hemoglobin 5.6 g/dl, serum lactate dehydrogenase 1,367 U/L, neuron specific enolase 56 ng/ml, ferritin 220 ng/ml, and urine vanillylmandelic acid 29.5 mg/day. Bone marrow infiltration with tumor cells (8%) was also found. Thorax CT revealed geometric shaped subpleural densities in the lungs consistent with atelectasis. Furthermore, CT scan of the abdomen showed bilateral adrenal cystic mass lesions with intracystic echogenities and fluid levels, suggesting intracystic hemorrhage. Besides hepatosplenomegaly, multiple hypodense lesions conforming to metastases in the liver were also observed (Fig. 1). Radionuclide bone scan was negative. Clinical diagnosis was congenital bilateral CNBwith liver metastases. However, lung metastases were also suspected. Since primary surgery could not be performed, chemotherapy was initiated. On the 8th day of chemotherapy, the patient died of extensive hepatic involvement, which caused respiratory and inferior vena cava compromise. Histopathologic examination revealed neuroblastoma cells at the wall of the almost entirely hemorrhagic cystic masses with favorable histology according to the Shimada classification, and lung, liver, and spleen involvement (Fig. 2). DNAcontent, N-myc expression, and allelic loss of chromosome 1p could not be obtained. Differentiation between adrenal hemorrhage and adrenal CNB is especially important because the treatment of hemorrhage is generally conservative. Although intratumoral hemorrhage is common in CNB, massive symptomatic hemorrhage is a rare finding [1,4,6]. To our knowledge, there have been only three reported cases with bilateral adrenalCNBwith intracystic hemorrhage [4,6,8]. In the newborn, reported by Lee et al. [4] the presence of the liver metastases made differential diagnosis easier as in our case. Since the other two reported cases with bilateral CNB which were diagnosed after the first month of life did not have liver involvement, the differential diagnosis of adrenal hemorrhagewas difficult [6,8]. MR imaging seems to be a good alternative radiological method in this differential diagnosis [6,9]. Most patientswithCNBare diagnosed in the early stage of disease with an excellent long-term prognosis, except patients with stage IV disease, hydrops, or massive hepatomegaly [2,3,10]. Only 9.7% of cases with CNB have metastatic disease at diagnosis [7]. When present, as in our case, the fetal liver is themost common site [5]. Two of three reported children with bilateral CNB had stage IVS, but one patient had stage IV disease. In children with CNB, less aggressive management may be warranted in the absence of stage IV disease. Some authors recommend that prenatally suspected non-metastatic CNB should undergo surgical intervention, unless tumor size decreases within about 1 month after birth [2,9]. While chemotherapy is necessary for advanced disease, as in our case, radiotherapy may be used in patients with unresectable or incompletely resected tumors [4]. One of two reported cases with bilateral CNB of stage IVS, to whom only surgical resection was performed, was lost to follow up,

Procalcitonin is more useful than C-reactive protein in differentiation of fever in patients with sickle cell disease.

This study aimed at evaluating the value of C-reactive protein (CRP) and procalcitonin (PCT) levels in the differential diagnosis of fever in patients with sickle cell disease (SCD). The study included 86 children with SCD (group 1) and 49 controls (group 2). During the study, the patients had 114 acute episodes or routine visits to the units. They were classified as having vasoocclusive crisis with fever (group 1A), vasoocclusive crisis without fever (group 1B), and no crisis or fever (steady state, group 1C). Only patients with crises were admitted to the hospital. Patients admitted to the hospital with various clinical signs and symptoms each and every time were included in groups 1A, 1B, and 1C. Thus, a total of 114 clinical episodes were analyzed. The mean CRP levels in the 3 patient groups were significantly higher than that in the group 2, and among the patient groups, the mean CRP was significantly higher in group 1A than the other groups. The mean CRP level in group 1A and group 1B was significantly higher than that in group 1C. There were no significant differences among the 3 SCD groups in terms of the median serum PCT level; however, the median PCT level in group 1A, group 1B, and group 1C patients was significantly higher than that in group 2 patients. These data indicate that vasoocclusive disease with or without fever apparently does not significantly increase PCT levels in relation to the baseline status of children with SCD, which in turn are clearly more elevated than PCT levels of control children.

Assessment of Cardiac Functions in Sickle Cell Anemia with Doppler Myocardial Performance Index

BACKGROUND The purpose of this study was to evaluate left and right ventricular functions by using the Doppler myocardial performance index (MPI), in children with sickle cell anemia (SCA). METHODS We examined 32 patients with SCA and 30 age-matched healthy children. Echocardiography and Doppler examinations were completed for each of the subjects. MPI was calculated from the Doppler tracings. RESULTS Compared with controls, left ventricle (LV) end-diastolic diameter, end-systolic diameter, early-diastolic mitral flow velocity and late-diastolic mitral flow velocity were significantly higher in the patients with SCA. Although, the LV ejection fractions were in the normal ranges in patients and controls, both LV and right ventricle (RV) MPI were significantly higher in patients than those in normal children. CONCLUSION MPI may be a useful noninvasive and sensitive tool for assessing the sub-clinical cardiac LV and RV dysfunctions in patients with SCA.

Admission time and mortality rates

ObjectiveThe objective of this study was to determine whether there there are any associations between time of admission and mortality rates in the pediatric intensive care unit.MethodsWe analyzed retrospectively 210 consecutive admissions to the PICU from November 2005 to April 2006 for patients aged 1 mth to 18 yr.ResultsThere was no significant difference for overall mortality rates between weekend and weekday admissions (12.2% vs 17.4%, p=0.245), and daytime and evening admissions (11.3 % vs 15.4%, p=0.254). There was also no significant difference between different admission times for within 24 hours, 48 hours and 72 hours mortality rates.ConclusionsIn respect of the overall mortality rates, it can be said that in a closed system PICU management under the control of a pediatric intensivist there is no association between time of admission and mortality rates.

Management of Patients With Status Epilepticus Treated at a Pediatric Intensive Care Unit in Turkey

We investigated the etiology, treatment, and prognosis of patients treated for status epilepticus at a pediatric intensive care unit. Medical records of 89 patients admitted to a pediatric intensive care unit with status epilepticus were reviewed retrospectively. Patients ranged in age from 2 months to 18 years (mean age ± S.D., 4.7 ± 3.8 years). Seizure etiologies comprised remote symptomatic in 47 (52.7%), febrile in 15 (16.9%), acute symptomatic in 12 (13.5%), and unknown in 15 (16.9%). Seizure durations ranged from 30-60 minutes in 58 patients, whereas 31 manifested refractory seizures longer than 60 minutes. Seizure control was achieved within 30 minutes in 55 patients, from 30-60 minutes in 19, and after 60 minutes in 15. Rectal diazepam was administered to 38 (42.7%) patients before admission to the hospital. Length of intensive care unit stay increased with increasing seizure duration (P < 0.05). The total mortality rate was 3.4%. This lower mortality rate may be considered evidence of the effectiveness and reliability of the status epilepticus treatment protocol in our pediatric intensive care unit. Prehospital rectal diazepam administration and the treatment of brain edema in the intensive care unit may be useful in the management of patients with status epilepticus.

Recurrent and atypical posterior reversible encephalopathy syndrome in a child with hypertension

Posterior reversible encephalopathy syndrome (PRES) is a clinical and radiologic entity with typical symptoms including headache, seizures, visual disturbance, altered mental status, vomiting, nausea and focal neurologic signs. In this article, we report recurrent and atypical PRES in a child with hypertension due to end-stage renal disease (ESRD) who was on a peritoneal dialysis program for 6 months. After the second hypertension attack, PRES findings did not recover and persisted as encephalomalacia. As far as we know, this case is the first child with ESRD who developed encephalomalacia after recurrent episodes of PRES. When a patient with a history of PRES presented with new clinical and neuroradiological findings, recurrent PRES should be considered.

Symptomatic and asymptomatic candidiasis in a pediatric intensive care unit

IntroductionThis study aimed to examine the incidence, epidemiology, and clinical characteristics of symptomatic and asymptomatic candidiasis in a pediatric intensive care unit (PICU), and to determine the risk factors associated with symptomatic candidiasis.MethodsThis retrospective study included 67 patients from a 7-bed PICU in a tertiary care hospital that had Candida-positive cultures between April 2007 and July 2009. Demographic and clinical characteristics of the patients, Candida isolates, antimicrobial and antifungal treatments, and previously identified risk factors for symptomatic candidiasis were recorded, and symptomatic and asymptomatic patients were compared.ResultsIn all, 36 (53.7%) of the patients with Candida-positive cultures had asymptomatic candidiasis and 31 (46.3%) had symptomatic candidiasis. Candida albicans was the most common Candida sp. in the asymptomatic patients (n = 20, 55.6%), versus Candida parapsilosis in the symptomatic patients (n = 15, 48.4%). The incidence of central venous catheter indwelling, blood transfusion, parenteral nutrition, and surgery was higher in the symptomatic patient group than in the asymptomatic patient group (P < 0.5). Surgery was the only independent predictor of symptomatic candidiasis according to forward stepwise multivariate logistic regression analysis (OR: 6.1; 95% CI: 1.798-20.692).ConclusionSurgery was the only risk factor significantly associated with symptomatic candidiasis and non-albicans Candida species were more common among the patients with symptomatic candidiasis. While treating symptomatic candidiasis in any PICU an increase in the incidence of non-albicans candidiasis should be considered.

The prevalance of and factors associated with intra-abdominal hypertension on admission Day in critically Ill pediatric patients: A multicenter study

PURPOSE To investigate admission prevalence of intraabdominal hypertension (IAH) and to determine clinical and laboratory characteristics on admission day associated with IAH in critically ill pediatric patients. MATERIALS AND METHODS One hundred thirty newly admitted critically ill pediatric patients were included. Intra-abdominal pressure (IAP) was measured 4 times (every 6 hours) with the bladder pressure method. Data included the demographics, diagnostic category, pediatric logistic organ dysfunction score and pediatric risk of mortality score II, clinical concomitant factors, and conditions potentially associated with increased intra-abdominal pressure. RESULTS Seventy patients (56.1%) had a normal IAP (≤10 mmHg, mean IAP [mmHg] 7.18 ± 1.85), while 60 patients (43.9%) had IAP >10 mmHg (mean IAP [mmHg] 15.46 ± 5.21). Hypothermia frequency, lactate levels, number of patients with oligo-anuria, and mechanical ventilation requirement were higher among patients with IAH compared to patients without IAH (both, P< .05). Hypothermia (OR, 3.899; 95% CI, 1.305-11.655; P< .03) and lactate levels (OR, 1.283 for each mmol/L increase; 95% CI, 1.138-1.447; P< .001) were only significantly associated with IAH. CONCLUSIONS Intra-abdominal hypertension seems to affect nearly half of newly admitted critically ill pediatric patients. Lactate level and the presence of hypothermia seem to be the independent predictors of the presence of IAH.

Lethal neonatal rigidity and multifocal seizure syndrome with a new mutation in BRAT1

Rigidity and Multifocal Seizure Syndrome, Lethal Neonatal (RMFSL) (OMIM# 614498) is a rare and recently characterized epileptic encephalopathy that is related to variants in the BRAT1 gene (Breast Cancer 1-associated ataxia telangiectasia mutated activation-1 protein). In this report, an RMFSL case, who died in the 10th month of the life, with rigidity, drug-resistant myoclonic seizures in the face and extremities, with, significant motor delays is presented. The exon sequence was determined and a new homozygous variant (C.2230_2237dupAACATGC) was detected. This RMFSL case with a homozygous variant in the BRAT1 gene, is the fourth one in the literature and the first one being reported from a Turkish family.