Alper Ozcan

Comparing the Efficacy of 7%, 3% and 0.9% Saline in Moderate to Severe Bronchiolitis in Infants

BACKGROUND There is no standard treatment option in acute bronchiolitis. 3-7% hypertonic saline (HS) seems to be the effective treatment choice for reducing the hospitalization day. AIMS To compare the effect of nebulized 7% HS/salbutamol and 3% HS/salbutamol to 0.9% saline/salbutamol. The primary outcome measure was the effect of study drugs on the length of hospital stay (LOS). Secondary outcome measures were safety and efficacy in reducing the clinical severity score (CSS) at the 24 hours of the study. STUDY DESIGN Prospective, double-blinded randomized clinical study. METHODS The study consists of 104 infants. Groups were constituted according to the treatment they received: These are, group A - 0.9% saline/salbutamol, group B -3% HS/salbutamol and group C-7% HS/salbutamol. Heart beat, Bronchiolitis CSS and oxygen saturation of the patients were determined before and after nebulization. The patients were monitored for adverse reactions. RESULTS Length of hospital stay in group A, B and C were as follows; 72.0 (20-288) hours in group A, 64.0 (12-168) hours in group B and 60.0 (12-264) hours in group C. No significant differences was observed among three groups (p>0.05). CONCLUSION 7% HS and 3% HS does not have any effect to decrease LOS for infants with bronchiolitis.

Evaluation of Microorganisms Causing Ventilator-Associated Pneumonia in a Pediatric Intensive Care Unit

OBJECTIVE The aim of this study was to identify microorganisms causing ventilator-associated pneumonia (VAP) and also study the antibiotic resistance/susceptibility. MATERIALS AND METHODS We retrospectively assessed microorganisms isolated from patients diagnosed with VAP in a pediatric intensive care unit between January 1, 2014, and June 30, 2016. RESULTS We included 44 patients diagnosed with VAP. The prevalence thereof was 8.6 patients per 1,000 ventilator days. Mechanical ventilation was required for 56.5% of patients. Thirty-three patients (75%) died. An underlying chronic disease was detected in 75% of patients (n=33). Fifty microorganisms were isolated from 44 patients. Single microorganisms were isolated from 86.4% (n=38) and two from 13.6% (n=6) of patients. Of all the isolated bacteria, 96% (n=48) were gram-negative; the most common was Pseudomonas aeruginosa (32%), followed by Klebsiella pneumoniae (24%) and Acinetobacter baumannii (22%). The isolates were most susceptible to colistin (92.6%), followed by piperacillin-tazobactam (71.4%), amikacin (65.2%), and gentamicin (52.2%). No enterobacterium or Acinetobacter strain was resistant to colistin; however, 13% of P. aeruginosa isolates were resistant. CONCLUSION In VAP, it is essential to catalog antibiotic resistance patterns of bacteria present in the unit to ensure that empirical antibiotic therapy is effective.

Neuroblastoma in a Child With Wolf-Hirschhorn Syndrome.

Neuroblastoma is the most common extracranial solid tumor of childhood originating from sympathetic nervous system cells. Neuroblastoma has also been diagnosed in conjunction with other congenital conditions such as Hirschsprungs disease, congenital hypoventilation disorder, and neurofibromatosis type 1. Wolf-Hirschhorn syndrome is a congenital disorder caused by microdeletion of short arm of chromosome 4 encoding MSX1 gene with characteristic facial features. We describe a child with dysmorphic features, developmental delay, mental retardation who developed neuroblastoma at 2 years of age and cytogenetic analysis of blood lymphocytes revealed an interstitial deletion of 4p(15,2). To best our knowledge, this report is the first report of neuroblastoma in a child with Wolf-Hirschhorn syndrome; and the reported association may be an important clue for oncological follow-up of patients with Wolf-Hirschhorn syndrome.

Scurvy: A rare cause of arthritis in a child with neurologic disorder

A 7-year-old boy presented with swelling in the knees, walking difficulty, petechial rashes on the lower extremity, and gum swelling and bleeding. His medical history was remarkable for mental retardation and autism. He was referred to our clinic with a differential diagnosis of bleeding disorder. His dietary history was positive for unbalanced nutrition (yogurt soup, chocolate, and wheat bread). On admission, his weight and height were normal, looked very ill, and had a body temperature of 38.3°C. His physical examination was remarkable for swollen and bleeding gum, follicular hyperkeratosis with perifollicular purpura at the lower extremities, and soft tissue swelling of both knees, which were painful during passive motion with bilateral 30° flexion contracture of the knees (Figure 1a and b). He had persistent and severe self-injurious behavior. Blood investigations showed anemia of chronic disease, elevated CRP level, and prolonged in vitro bleeding time. Bilateral knee diagraph showed a radio-dense band at the chondro-osseous junction Frankel’s line (Figure 2a). Magnetic resonance imaging demonstrated bright signal intensity on the meZeynep Küçükaydın1 , İsmail Dursun1 , Burcu Daldaban1 , Alper Özcan2, Ekrem Ünal2 Images in Rheumatology

The relationship between hematological parameters and prognosis of children with acute ischemic stroke

BackgroundStroke is rarely seen in children, but it is a major cause of morbidity and mortality. Therefore, there is a need for inexpensive and noninvasive diagnostic methods for estimating the prognosis. Although the prognostic importance of hematological parameters in acute ischemic stroke were reported in adult studies, there is a lack in pediatric ages. The aim of the study is to investigate the relationship between hematological parameters and prognosis of acute ischemic stroke in children.MethodsRetrospectively scanned in the study were 106 pediatric patients with acute ischemic stroke who managed at the Medical Faculty of Erciyes University, Kayseri, between the years of 2000 and 2014. White blood count (WBC); neutrophil, lymphocyte, and platelet count; mean platelet volume (MPV); platelet distribution width (PDW); neutrophil count/lymphocyte count (N/L) ratio values obtained from the measurements and initial symptoms; demographical features; risk factors; neurological examination; and clinical follow-up were recorded. Their hematological parameters were compared with those of 106 age and sex-matched healthy individuals.ResultsMPV and PDW values were found similar in patient and control groups, and the platelet count was found significantly low in the control group (p = 0,028). WBC, neutrophil count, and N/L ratio were found considerably high in the patient group (p < 0.001). Lymphocyte count, however, was found significantly low in the control group (p < 0.001). No statistically significant difference was detected in WBC, neutrophil count, lymphocyte count, platelet count, N/L ratio, and MPV and PDW values between the group with sequelae and the one without sequelae. In addition, it was determined that WBC, neutrophil count, lymphocyte count, platelet count, N/L ratio, and MPV and PDW values in the univariate Cox-regression analysis of the patient group had no effect on survival and disease-free survival. When receiver operating characteristic curve was applied, it was observed that the area below WBC, N/L ratio curve was important in the patient group in terms of predicting acute ischemic stroke.ConclusionThe values of WBC, neutrophil count, and N/L ratio differ significantly from those of the control group. The WBC and N/L ratio may help for an earlier diagnosis in children with acute ischemic stroke. WBC, thrombocyte count, MPV, PDW, and N/L ratio do not constitute a risk in overall survival, disease-free survival, and sequelae development.

Succesful treatment of extravasation injury with topical heparinoid in a pediatric patient

Extravasation is unintentional drug administration or leakage to perivascular space, subcutaneous tissues during intravenous drug administration. It is one of the common complications, which is seen in many patients admitted to pediatric intensive care unit for long-term, and received multiple drug support. Pain, erythema and swelling can be observed when vesicant agents leak to extra-vascular space. Irritant agents can cause burning sensation and pain when extravasation occurs or by leading irritation at vessel wall before or at onset of extravasation. It may lead inflammation which can progress to phlebitis without necrosis (1-6). Here, we presented extravasation from total parenteral nutrition solution in an infant with mitochondrial disorder with poor general status who had been followed in pediatric intensive care unit. We achieved recovery without sequel in this patient by local heparinoid treatment.

A Case of Familial Hemophagocytic Lymphohistiocytosis Type 4 With Involvement of the Central Nervous System Complicated With Infarct

Background: Familial hemophagocytic lymphohistiocytosis (HLH) is a fatal disease affecting infants and very young children. Central nervous system involvement of HLH can cause catastrophic results. Method: We present a case with cranial involvement of familial HLH type 4 who showed diffuse infiltration of white matter complicated with intracranial thrombosis. A 5-year-old girl from a consanguineous couple presented with fever and pancytopenia, and was referred to our hematology unit. Examination revealed fever, lymphadenopathy, and hepatosplenomegaly. Ultrasound examination revealed hepatosplenomegaly and free intra-abdominal fluid. HLH was revealed on bone marrow aspiration biopsy. Defective natural killer and T lymphocyte cytotoxicity using degranulation tests was determined. In the genetic analysis, syntaxin gene mutation was found. On T2-weighted and T2-fluid-attenuated inversion recovery magnetic resonance imaging (MRI), diffuse hyperintense signal changes of cerebral white matter, indicating white matter demyelination, were observed. A second brain MRI showed an acute infarct involving the left temporooccipital region. Immunosuppressive therapy according to the HLH 2004 protocol was started. The infarct resolved but white matter lesions were stable on the brain MRI that was performed 1 month later. Brain MRI taken 4 months after the first examination showed stable cerebral white matter lesions, but hyperintense signal changes appeared in the cerebellar white matter and were regarded as progression. The patient died because of infection despite immunosuppressive therapy. Conclusions: Physicians managing patients with HLH must be vigilant about the possibility of central nervous system involvement including stroke.

Interstitial deletion 5p14.1-p15.2 and 5q14.3-q23.2 in a patient with clubfoot, blepharophimosis, arthrogryposis, and multiple congenital abnormalities

Interstitial deletions of the short and long arms of chromosome 5 are rare cytogenetic abnormalities. The 5p distal deletion is a genetic disorder characterized by a high‐pitched cat‐like cry, microcephaly, epicanthal folds, micrognathia, severe intellectual disability and motor delays. Previously, more than 46 patients with the 5q deletion have been reported. Here, we report de novo interstitial deletions involving 5p14.1–p15.2 and 5q14.3–q23.2 in a patient with multiple congenital abnormalities, including blepharophimosis, arthrogryposis, short neck, round face, pelvic kidney, agenesis of the corpus callosum, and clubfoot. The deletions were characterized using GTG banding and aCGH microarray analysis. Concurrent 5p and 5q interstitial deletions in humans have not been previously reported. We also discussed the relationship between the 5q deleted region and clubfeet.

Combination of two different homozygote mutations in Pompe disease

Pompe disease (OMIM no 232300) is an autosomal recessive inherited metabolic disorder, caused by glycogen accumulation in the lysosome due to deficiency of the lysosomal acid 03B1‐glucosidase enzyme. Here we report the case of an 8‐month‐old girl of consanguineous Turkish parents, who was diagnosed with the infantile form of Pompe disease. Two different uncommon homozygote mutations (c.32‐13 T > G homozygote and c.1856G > A homozygote) were detected. The patient had a more progressive clinical course than expected. We emphasize the rare combination of genetic mutations in this Turkish family with Pompe disease.

Fibromuscular Dysplasia Complicated With Cerebral Stroke in a Child With Congenital Dyserythropoietic Anemia Type II.

Congenital dyserythropoietic anemia type II belongs to a subtype of bone marrow failure syndrome, which is characterized by monolineage involvement and typical morphologic abnormalities in erythroid precursor cells resulting in different degrees of hyporegenerative anemia. Moreover, reticulocytosis, which is not corresponding to the degree of anemia, with jaundice and splenomegaly are major diagnostic criteria. Causative gene is located at SEC23B. Although stroke among children is rare, it can cause significant morbidity and mortality. Herein we present a 3-year-old male with congenital dyserythropoietic anemia type II who presented with stroke-like symptoms, and was diagnosed with fibromuscular dysplasia.