Evaluation of Neurosurgical Emergencies in the Pediatric Emergency Department: Clinical Warning Signs.

BACKGROUND: We aimed to evaluate the patients who underwent neuroimaging with suspicion of neurosurgery pathology and identify the clinical warning signs for the early recognition of neurosurgical emergencies. METHODS: Patients aged one month to 18 years who underwent neuroimaging with a preliminary diagnosis of intracranial pathology requiring emergency surgery and symptom duration less than one month were included in the study. Patients were divided into three groups according to their definitive diagnosis as neurosurgical emergencies, neurological emergencies, and nonurgents. RESULTS: A total of 140 patients were included in the study (the median age was 8 [interquartile range IQR 3 to 13] years and 52.8% were male). Neurosurgery emergency group and neurological emergency group were significantly younger than the nonurgent group (P < 0.001). Vomiting, meningeal irritation findings, and papilledema (grade 2 and above) were more common in the neurosurgical emergency group (P 0.029, 0.023, and < 0.001, respectively). For neurosurgical emergencies, in the presence of papilledema (grade 2 and above) and focal neurological deficit, the specificity was 99.2%, positive predictive value (PPV) 83.3%, negative predictive value (NPV) 88.1%, and odds ratio (OR) 36.8 (P < 0.001, confidence interval [CI] 4.04 to 336.0); in the presence of altered consciousness and focal neurological deficit, the specificity was 97.5%, PPV 50%, NPV 86.6%, and OR 6.4 (P = 0.014, CI 1.20 to 34.4). CONCLUSIONS: Younger age, presence of vomiting, signs of meningeal irritation, papilledema grade 2 and above, and altered consciousness are the crucial "warning signs" of a potential neurosurgical emergency.

Utility of biomarkers in predicting complicated appendicitis: can immature granulocyte percentage and C-reactive protein be used?

BACKGROUND: Acute appendicitis is the most common reason for abdominal surgery in children. The aim of this study was to evaluate the utility of biomarkers in predicting complicated appendicitis (CA). METHODS: Patients having a diagnosis of acute appendicitis who underwent operations were retrospectively determined, and the utility of biomarkers in predicting CA was evaluated. RESULTS: A total of 251 patients were included in the study. The mean age was 130.9 ± 48.8 months, 148 of the cases (59%) were simple appendicitis, and 103 (41%) were CA. The C-reactive protein (CRP) levels, immature granulocyte (IG) percentage, white blood cell counts, and absolute neutrophil counts were significantly higher in the CA patients. The bilirubin levels and neutrophil to lymphocyte ratios were not useful for predicting CA. The best area under the curve (AUC) values to predict CA were with the IG percentage and CRP level (0.82), the IG percentage [odds ratio (OR) 9.36, 95% CI (4.94-17.75), p < 0.001] and CRP [OR 8.42, 95% CI (4.72-15.02), p < 0.001] were the best marker in predicting CA. The sensitivity of the IG percentage and CRP level were higher than other markers. CONCLUSION: To predict CA, the best AUC values were associated with the IG percentage and the CRP level. Because it is easy, fast to measure, does not require taking extra blood, and does not lead to additional costs, IG percentage may be preferred in the diagnosis of patients with CA.

Minimum ingested iron cut-off triggering serious iron toxicity in children.

BACKGROUND: Iron intoxication can occur accidentally in children or intentionally by adolescents as a suicide attempt. They usually present with various symptoms including vomiting and diarrhea. Clinical studies in this field has been reported different doses of ingested elemental iron that caused serious toxicity, but none of these studies determined the minimum cut-off of ingested iron that triggered the risk of severe toxicity. The aim of this study was therefore to investigate the demographic features of iron intoxication in Turkish children and to determine the lowest cut-off of ingested elemental iron triggering serious intoxication and the need for prompt management. METHODS: This retrospective study investigated 83 Turkish patients with accidental and intentional iron poisoning. RESULTS: Of the 83 cases of acute iron intoxication, accidental iron consumption was more common than intentional use. Fifty-three patients ingested a median toxic dose of elemental iron of 40.0 mg/kg (IQR, 33.5 mg/kg). The median serum iron concentration in the first 6 h of ingestion was 150 µg/dL (IQR, 282 µg/dL). Twenty patients were given deferoxamine, whereas 63 patients were given supportive treatment. CONCLUSION: The cut-off of ingested elemental iron that triggered serious toxicity and the need for deferoxamine in children <18 years of age was 28 mg/kg.

Evaluation of the Colchicine Poisoning Cases in a Pediatric Intensive Care Unit: Five Year Study.

BACKGROUND: Colchicine poisoning is an uncommon but serious form of drug intoxication. It may produce life-threatening systemic effects. In toxic doses it produces nausea and vomiting and bone marrow suppression, often leading to sepsis, hypocalcemia, adult respiratory distress syndrome, and direct cardiotoxic effects. OBJECTIVE: The aim of this study was to describe demographic features and the outcome of patients poisoned with colchicine. METHODS: A retrospective study of the pediatric intensive care unit database was performed for patients ≤18 years of age who had colchicine poisoning between July 2008 and July 2013. RESULTS: The total number of patients with drug poisoning in the study period was 144. Nine of 144 were related to colchicine poisoning. The median age was 4 years (range 20 months to 16 years) and the number of females was five. Six of the nine cases presented after ingesting <0.5 mg/kg, whereas two patients had consumed 0.5 to 0.8 mg/kg. One patient had received colchicine >0.8 mg/kg. Three patients died. CONCLUSIONS: Among drug intoxications, colchicines can lead to severe clinical conditions. All patients suspected of having colchicine intoxication should be managed in the pediatric intensive care unit regardless of the actual degree of poisoning.

A novel G6PC3 gene mutation in a patient with severe congenital neutropenia.

Glucose-6-phosphatase catalytic subunit 3 (G6PC3) deficiency is a newly described syndromic type of severe congenital neutropenia, associated with multiple organ abnormalities including facial, cardiac, and urogenital abnormalities, and increased visibility of superficial veins. The molecular pathophysiology of G6PC3 deficiency is associated with the disturbed glucose homeostasis, increased endoplasmic reticulum stress, and apoptosis in neutrophils. We report a new case of G6PC3 deficiency caused by a novel homozygous G6PC3 gene mutation (p.Leu154Pro). Most remarkable is that the chronic neutropenia that originated from this novel G6PC3 genetic defect is also accompanied by some other unusual manifestations in this patient: myelokathexis and hypercholesterolemia.

Clinical and epidemiological features of Turkish children with 2009 pandemic influenza A (H1N1) infection: experience from multiple tertiary paediatric centres in Turkey.

BACKGROUND: In April 2009 a novel strain of human influenza A, identified as H1N1 virus, rapidly spread worldwide, and in early June 2009 the World Health Organization raised the pandemic alert level to phase 6. Herein we present the largest series of children who were hospitalized due to pandemic H1N1 infection in Turkey. METHODS: We conducted a retrospective multicentre analysis of case records involving children hospitalized with influenza-like illness, in whom 2009 H1N1 influenza was diagnosed by reverse-transcriptase polymerase chain reaction assay, at 17 different tertiary hospitals. RESULTS: A total of 821 children with 2009 pandemic H1N1 were hospitalized. The majority of admitted children (56.9%) were younger than 5 y of age. Three hundred and seventy-six children (45.8%) had 1 or more pre-existing conditions. Respiratory complications including wheezing, pneumonia, pneumothorax, pneumomediastinum, and hypoxemia were seen in 272 (33.2%) children. Ninety of the patients (11.0%) were admitted or transferred to the paediatric intensive care units (PICU) and 52 (6.3%) received mechanical ventilation. Thirty-five children (4.3%) died. The mortality rate did not differ between age groups. Of the patients who died, 25.7% were healthy before the H1N1 virus infection. However, the death rate was significantly higher in patients with malignancy, chronic neurological disease, immunosuppressive therapy, at least 1 pre-existing condition, and respiratory complications. The most common causes of mortality were pneumonia and sepsis. CONCLUSIONS: In Turkey, 2009 H1N1 infection caused high mortality and PICU admission due to severe respiratory illness and complications, especially in children with an underlying condition.

Eponym. Kostmann disease.

Rolf Kostmann (1909-1982) was a Swedish pediatrician and army doctor. He was the first to describe an inherited form of chronic neutropenia in childhood. In 1956, Kostmann published his article "Infantile genetic agranulocytosis" in Acta Paediatrica. "Infantile agranulocytosis," as Rolf Kostmann named this hereditary syndrome, has been known for more than half a century, yet the underlying genetic mutations have remained unknown for many decades. Fifty years later, homozygous mutations in the gene encoding the mitochondrial protein HCLS1-associated X1 were found in affected members of the original Kostmann pedigree. Therefore, the eponym "Kostmann disease" best fits this specific mutation and mode of inheritance. The identification of genetic cause now allows the analysis of genotype-phenotype correlations. After the development of recombinant human granulocyte colony-stimulating factor (G-CSF), the prognosis and quality of life improved dramatically. Hematopoietic stem cell transplantation remains the only currently available treatment for refractory cases to G-CSF and patients who have transformed into leukemia.

Measles, rubella, mumps, and varicella seroprevalence among health care workers in Turkey: is prevaccination screening cost-effective?

BACKGROUND: To investigate the immune status of health care workers (HCWs) against measles, rubella, mumps, and varicella zoster (MMRV) in Turkey and to define an appropriate vaccination program among HCWs. METHODS: Voluntary HCWs from a children's hospital and a general hospital were included in the study between March and May 2005. The specific IgG antibodies against MMRV viruses were screened by ELISA. RESULTS: Three hundred sixty-three HCWs participated in the study; 186 (51%) were physicians, 118 (33%) were nurses, 36 (10%) were housekeeping staff, and 23 (6%) were medical technicians. The proportion of HCWs who had antibodies against measles was 98.6%; rubella, 98.3%; mumps, 92.2%; and varicella, 98%. No association was found between the susceptibility to at least 1 of MMRV virus infections and gender, age, duration of work, profession, and department of work in analysis either among the whole study group, or each hospital. The positive predictive value for the history of varicella was 100%, whereas it was 92% for MMR. The cost of vaccination for varicella was significantly expensive without screening before vaccination. However, there was not much difference for MMR infections. CONCLUSION: A policy based on obtaining the history of varicella infection from the staff and then screening the ones with negative history and vaccination of only seronegative HCWs was found to be appropriate.

Immune thrombocytopenic purpura as sole manifestation in a case of acute hepatitis A.

Acute hepatitis due to hepatitis A virus is usually a benign selflimiting disease during childhood. Although many viral infections such as hepatitis B virus, Parvovirus, and Epstein-Barr virus are associated with extrahepatic autoimmune phenomena, such manifestations are rare in patients with acute hepatitis A infection. Immune thrombocytopenia is a benign, self-limiting disease in children, responding well to treatment and generally associated with viral infections. Immune thrombocytopenic purpura is rarely reported as a manifestation of acute hepatitis A. We report a five-year-old boy with immune thrombocytopenic purpura as the sole manifestation of anicteric acute hepatitis A infection. Acute hepatitis A should be included in the differential diagnosis of immune thrombocytopenic purpura.