Assessment of clinical impact and predisposing factors for neonatal thrombocytopenia

Thrombocytopenia is a common hemostatic abnormality in the newborn infant. The early diagnosis of thrombocytopenia and the underlying primary pathology process play an important role in reducing the risk of severe complications and mortality. We performed a 2-year prospective study of 643 neonates admitted to our neonatology unit to determine the frequency, predisposing factors, and clinical impact of thrombocytopenia. Thrombocytopenia developed in 18.2% of the preterm neonates and 0.8% of the term neonates. Prematurity, sepsis, hypoxia, intrauterine growth retardation, and disseminated intravascular coagulation were identified as predisposing factors for thrombocytopenia. The incidence of complications and mortality were higher in thrombocytopenic infants. Especially the prognosis was worse in cases who had mucosal hemorrhage, without a relation with the degree of thrombocytopenia. The thrombocytopenia occurred by day 2 in 43% of the infants, and resolved by day 8 in 61%. The platelet count nadir occurred by day 2. Since thrombocytopenic infants are at greater risk for bleeding, and the thrombocytopenia itself may have contributed to the high mortality, predisposing factors such as prematurity, infections, hypoxia must be eliminated by prividing better care, giving adequate hygiene of both mother and the baby during the prenatal, natal, and neonatal period.     

Serum hyaluronic acid concentrations in children with cirrhosis.

BACKGROUND: Liver disease is associated with increased levels of hyaluronic acid (HA). AIM: To evaluate serum HA concentrations in children with cirrhosis and its relation with liver function tests and Child-Pugh score. METHODS: Twenty-two children with biopsy-proven liver cirrhosis were studied. All were assessed for the presence of ascites or encephalopathy and liver function tests were performed. Patients were categorized according to Child-Pugh criteria. Serum HA was measured using microELISA (normal 0-100 ng/mL). Twenty-two children with chronic hepatitis B and no cirrhosis were studied as controls. RESULTS: Serum HA level in the cirrhotic children was 85.2 (72.8) ng/mL; levels were high (166.0 [46.3] ng/mL; range 115-246) in 8 (36.4%) patients. Three of 11 (27.2%) Child-Pugh class A patients, 3 of 8 (37.5%) class B patients, and 2 of 3 (66.7%) class C patients had elevated serum HA values (p=ns). Serum HA levels correlated with direct bilirubin level. The control group had lower levels (4.8 [2.3] ng/mL; p< 0.05), which were in the normal range. CONCLUSION: Serum HA level may be useful as a diagnostic tool in children with cirrhosis.     

Can E-selectin be a reliable marker of inflammation in lumbar disc disease?

The cause of sciatica and low back pain associating with lumbar disc herniation has not been clearly identified until now. Inflammation has been shown to occur via immunohistochemical and biochemical methods in herniated disc tissues. The important prognostic role of E-selectin has recently been substantiated by other studies in early rheumatoid arthritis (RA) and juvenile rheumatoid arthritis (JRA). The important role of adhesion molecules in the initiation and progression of the inflammatory response is well known for infectious diseases and autoimmune disorders. In our study, we aimed to show the role of E-selectin as an inflammatory marker and the correlation of inflammation with straight-leg raise (SLR) test findings and subtype of disc herniation. We found that the cases with positive SLR test had higher rates of immunostaining with E-selectin. This led us to think that E-selectin might play an important role in the activity status of the disease, meaning patients with more limited movement capacity might benefit from E-selectin antagonist therapy. Among the many studies performed to identify the relationship between the inflammation markers and activity of lumbar disc herniation, this is the first investigation held with E-selectin.     

In Vivo Study to Assess the Viability and Mass Effect of Fascia Injection in Comparison with Block Fascia Graft

Recently, fat injection has gained favor among plastic surgeons for soft tissue augmentation. However, fat injections lose 50% of their volume after 1 year. The profession is in need of an injectable bulking material that gives a long-lasting improvement. Using 30 male rats, this study investigated the stability of the size and structure of the injected fascia autograft and compared it with surgically transplanted fascia. The fascia graft was harvested from the back of the rat, big, and divided into two equal pieces. The first piece was minced into a paste and injected subcutaneously on the anterior surface of the right ear. The other piece was transplanted subcutaneously on the anterior surface of the left ear. The grafts were observed for any sign of resorption over 1 to 6 months.Grossly, injection and transplantation sites were palpable at the end of the observation periods. Microscopic examination showed that injected fascia maintains its histomorphologic structure.These findings indicate that the injected fascia graft is well tolerated, and the size of the graft remained stable. According to this study, fascia injection can result in bulking material that gives a long-lasting improvement, and can be a viable alternative to other methods.     

Inflammatory Myofibroblastic Tumor of the Ileocecal Mesentery Mimicking Abdominal Lymphoma in Childhood: Report of Two Cases

An inflammatory myofibroblastic tumor is an uncommon benign tumor located in various organs that can be misdiagnosed as a malignant neoplasm. We herein present two patients with ileocecal inflammatory myofibroblastic tumors. An abdominal mass was detected in a 13-year-old girl and a 15-year-old boy who presented with paleness, fatigue, intermittent fever, and night sweating. The radiological findings confirmed a mass originating from the ileocecal region. The presumptive diagnosis was Burkitt’s lymphoma. The histopathological diagnosis was inflammatory myofibroblastic tumor. After a surgical resection, all systemic symptoms rapidly resolved. Inflammatory myofibroblastic tumor is a rare pseudosarcomatous clinical and pathological entity. Although this tumor is more commonly reported in the lung, it can be detected in extrapulmonary sites, including the mesentery. Because the choice of treatment for this tumor is conservative surgery, an accurate preoperative analysis is important to avoid any unnecessary aggressive surgical intervention or other therapeutic approaches.     

Symptomatic and asymptomatic hypohidrosis in children under topiramate treatment

Topiramate (TPM) has peculiar side effects such as speech difficulties, weight loss, oligohidrosis and hyperthermia. We present the frequency and severity of hypohidrosis in our patients under TPM treatment. One hundred and two patients treated with TPM were evaluated retrospectively. Five (8 months-15 years of age) of them experienced symptomatic hypohidrosis manifested with prolonged or intermittent fever. Pilocarpine iontophoresis sweat test had been performed on the five patients before they were managed, and no sweat had been collected in 4/5 cases. Of the 102 patients, 42 who started TPM treatment when the study was established were evaluated prospectively regarding oligohidrosis. First, they were questioned about whether hypohidrosis occurred after TPM. Of 42, 11 patients complained of hypohidrosis. A sweat test was then performed on these 11 patients. Sweat could not be obtained in 5/11, and increased chloride concentration was found in 4/11. However, sweat could be obtained in the patients who had no complaint of hypohidrosis. Pediatricians should be aware of this side effect of TPM to prevent nonrelevant and cumbersome investigations in patients with prolonged or intermittent fever onset during TPM treatment. Our findings suggest that 5% of patients would experience hyperthermia during TPM treatment. Hypohidrosis without hyperthermia would be more frequent. If it is possible to collect sweat in patients who get fever during TPM treatment, it would be highly probable that the fever is not due to hypohidrosis. Increased chloride concentration alone does not seem to be significant for the hyperthermia risk.     

Fanconi-Bickel syndrome in three Turkish patients with different homozygous mutations

Three Turkish patients with Fanconi-Bickel syndrome are presented. Prominent clinical findings of patients included hepatomegaly, growth retardation, hypoglycemia, characteristic tubular nephropathy, and rickets. Each patient had a different homozygous mutation of glucose transporter 2 (GLUT2) gene.     

Inflammatory bowel disease-like colitis in a young Turkish child with glycogen storage disease type 1b and elevated platelet count

Inflammatory bowel disease (IBD)-like colitis is a known entity in glycogen storage disease (GSD) type 1b patients. The mean age of the reported cases with IBD-like colitis was 12 +/- 5 years, and all had absolute neutrophil count (ANC) less than 1,000 cells/microl. We report a three-year-old girl with GSD type 1b that was dignosed by mutation analysis. The patient was hospitalized with fever, diarrhea, and perioral and anal ulcers. Colonoscopy was performed and IBD-like colitis was diagnosed. The patient had elevated platelet count beyond the age of three months, but IBD-like colitis was diagnosed at three years of age. An elevated platelet count may be a warning sign for the IBD-like colitis in young patients with GSD type 1b.     

Etiologic evaluation in 247 children with global developmental delay at Istanbul, Turkey

OBJECTIVE: Developmental delay is a common pediatric problem, having a great number of underlying causal factors. Etiologic diagnosis is important for providing information about pathogenesis, prognosis, recurrence risk and specific medical interventions. The aim of this study was to determine the etiologic yield and spectrum of a consecutive cohort of global developmentally delayed children. METHODS: This retrospective study included all children younger than 5 years of age with global developmental delay referred to a single university-based ambulatory pediatric neurology clinic for initial evaluation over a 14-month period from January 1997. Diagnostic studies consisted of history, physical examination, electroencephalography and selected investigations including neuroimaging, screening for metabolic disease, karyotype and fragile X testing. RESULTS: In the study 247 patients (136 males) with a mean age of 24.2+/-20.3 months were evaluated. Etiologic diagnosis was determined in 64 per cent of the patients classified under the following categories: perinatal complications (21 per cent), cerebral dysgenesis (18 per cent), chromosomal abnormalities (9 per cent), genetic/dysmorphic syndromes (3 per cent), metabolic disorders (4 per cent), hypothyroidism (4 per cent), neurocutaneous syndromes (3 per cent), intrauterine infection (2 per cent). Etiology was unknown in 36 per cent of the patients. Two laboratory tests (neuroimaging and cytogenetic analysis) together with the history and physical examination were most helpful in determining the etiologic diagnosis. CONCLUSION: This study suggests that optimal management of global developmentally delayed children and their family should involve a comprehensive evaluation.     

Constitutional delay of growth and puberty: from presentation to final height

This retrospective study evaluated clinical characteristics of patients with constitutional delay of growth and puberty (CDGP) at presentation, during puberty and at final height. The records of 151 children (105 boys, 46 girls) with CDGP were reviewed and the results were evaluated with respect to findings in healthy Turkish schoolchildren. CDGP was twice as frequent in boys as in girls. Height and weight deficit and short sitting height of the children were evident at presentation and continued up to final height. Mean age of onset of puberty was retarded by 2.5 years in girls and by 3 years in boys. The time between onset of puberty and pubertal growth spurt was shorter in both girls and boys than in the controls. Peak growth velocity was compromised in both girls and boys. Forty-one patients (30 boys, 11 girls) reached final height (FH). Mean FH was shorter than both target height and predicted adult height. The Bayley-Pinneau method was found to be a better predictor of FH than either the Tanner-Whitehouse method or target height. FH also showed correlation with the father's height. There was no effect of testosterone treatment on final height. Height deficit at onset of puberty, shorter duration between onset of puberty and pubertal growth spurt, compromised peak growth velocity and short upper segment due to delayed puberty, are findings which may explain the decreased final height of children with CDGP.