Evaluation of Baseline Characteristics and Prognostic Factors in Multisystemic Inflammatory Syndrome in Children: Is It Possible to Foresee the Prognosis in the First Step?

Background: Two years after the first cases, critical gaps remain in identifying prognostic factors in multisystem inflammatory syndrome in children (MIS-C). Methods: This retrospective study included 99 patients with MIS-C hospitalized between August 2020 and March 2022 in a pediatric tertiary center. The patients were divided into two groups according to clinical severity (low- and high-risk). Prognostic values of baseline clinical and laboratory characteristics were evaluated with advanced statistical analysis, including machine learning. Results: Sixty-three patients were male, and the median age was 83 (3−205) months. Fifty-nine patients (59.6%) were low-risk cases. Patients aged six years and over tended to be at higher risk. Involvement of aortic or tricuspid valve or >1 valve was more frequent in the high-risk group. Mortality in previously healthy children was 3.2%. Intensive care unit admission and mortality rate in the high-risk group were 37.5% and 7.5%, respectively. At admission, high-risk patients were more likely to have reduced lymphocyte count and total protein level and increased brain natriuretic peptide (BNP), ferritin, D-dimer, and troponin concentrations. The multiple logistic regression model showed that BNP, total protein, and troponin were associated with higher risk. When the laboratory parameters were used together, BNP, total protein, ferritin, and D-dimer provided the highest contribution to the discrimination of the risk groups (100%, 89.6%, 85.6%, and 55.8%, respectively). Conclusions: Our study widely evaluates and points to some clinical and laboratory parameters that, at admission, may indicate a more severe course. Modeling studies with larger sample groups are strongly needed.

Comparison of the value of immature retyculocyte and immature platelet in the diagnosis of sepsis.

BACKGROUND: Sepsis is one of the causes of pre-treatment morbidity and mortality in the pediatric age group. In the present study, we investigated the place of the immature granulocyte percentage, (IG) immature reticulocyte fraction (IRF), and immature platelet fraction (IPF) in the diagnosis of sepsis. METHODS: Complete blood count, C-reactive protein, (CRP) procalcitonin (PCT) and blood cultures were measured in 125 critical patients who were followed-up in the intensive care unit with the suspicion of sepsis and 65 healthy children between 2017 and 2019. In addition to the complete blood counts and routine parameters, IG, IRF, and IPF were examined in the patients. RESULTS: When the critical patient group and the healthy control group were compared, it was found that the total number of leukocytes (white blood cells), neutrophil count, platelet count, CRP, PCT, IG, IRF, and IPF values were higher at statistically significant levels. When septic and non-septic patients were compared, it was found that the CRP, PCT,IGP, and IPF were higher at statistically significant levels in the septic patients. CONCLUSIONS: It was concluded that CRP, PCT, IG, and IPF were significant in determining sepsis and that PCT was the most sensitive and specific biomarker in these parameters. We believe that these parameters may be suitable for practical use in determining sepsis because they give faster results and suggest the diagnosis of sepsis.

A multicentered study on efficiency of noninvasive ventilation procedures (SAFE-NIV)

Background/aim: To characterize the clinical course of noninvasive positive pressure ventilation (NIPPV) and high flow humidified nasal cannula ventilation (HFNC) procedures; perform risk analysis for ventilation failure. Material and methods: This prospective, multi-centered, observational study was conducted in 352 PICU admissions (1 month-18 years) between 2016 and 2017. SPSS-22 was used to assess clinical data, define thresholds for ventilation parameters and perform risk analysis. Results: Patient age, onset of disease, previous intubation and hypoxia influenced the choice of therapy mode: NIPPV was preferred in older children (p = 0.002) with longer intubation (p < 0.001), ARDS (p = 0.001), lower respiratory tract infections (p < 0.001), chronic respiratory disease, (p = 0.005), malignancy (p = 0.048) and immune deficiency (p = 0.026). The failure rate was 13.4%. sepsis, ARDS, prolonged intubation, and use of nasal masks were associated with NIV failure (p = 0.001, p < 0.001, p < 0.001, p = 0.025). The call of intubation or re-intubation was given due to respiratory failure in twenty-seven (57.5%), hemodynamic instability in eight (17%), bulbar dysfunction or aspiration in 5 (10.6%), neurological deterioration in 4 (8.5%) and developing ARDS in 3 (6.4%) children. A reduction of less than 10% in the respiration within an hour increased the odds of failure by 9.841 times (OR: 9.841, 95% CI: 2.0021­48.3742). FiO2 > 55% at 6th hours and PRISM-3 >8 were other failure predictors. Of the 9.9% complication rate, the most common complication was pressure ulcerations (4.8%) and mainly observed when using full-face masks (p = 0.047). Fifteen (4.3%) patients died of miscellaneous causes. Tracheostomy cannulation was performed on 16 children due to prolonged mechanical ventilation (8% in NIPPV, 2.6% in HFNC) Conclusion: Absence of reduction in the respiration rate within an hour, FiO2 requirement >55% at 6th hours and PRISM-3 score >8 predict NIV failure.

Outcomes and prognostic factors for pediatric cancer patients admitted to an intensive care unit in a university hospital.

BACKGROUND AND OBJECTIVES: The aim of this study was to investigate the factors predicting Pediatric Intensive Care Unit (PICU) mortality and the outcomes in cancer patients admitted to PICU. METHODS: We conducted a retrospective study in 48 consecutive cancer patients admitted to the PICU between January 1, 2015 and January 1, 2018. A total of 48 patients (21 males and 27 females) were enrolled in this study. RESULTS: The median age was 77 (33,5-149) months. The median duration of PICU stay was 5 (2-9) days. Patients were classified according to their stage of disease. Ten (20.8%) patients were in the remission group, 9 (18.8%) patients were in the induction period and 29 (60.5%) patients were in the progressive diseasegruops. Thirtynine patients (81.2%) had hematological malignancies, 6 (12.5%) had extracranial solid tumors and 3 (6.3%) had intracranial solid tumors. Thirty-seven patients died and the mortality rate was found to be 77.1%. mortality rates were 11%, 88% and 93% for patients in remission,during induction period and in the progressive disease group, respectively (p < 0.01).The most frequent reasons of PICU admission were respiratory failure in 29 (60.4%), sepsis in 12 (25%), circulatory collaps in 2 (4.2%), and other reasons in 5 patients (10.4%). The median PRISM III among survivors was significantly lower than non-survivors (13.1 ± 6.4; vs. 20.7 ± 5.2; p < 0.001). At a cut-off value of 13, the sensitivity of the PRISM III was 94.4% and the specificity was 58.3% (AUC: 0.821). OSDwas present in 41 (85%) patients, 82% of them died (34/41). The presence of MOF, the use of mechanical ventilation and inotrop support were significantly related with mortality. Univariate logistic regression analysis showed that male gender [odds ratio (OR)=5.588, P= 0.041, 95% confidence interval (95%CI) 1.070-29.191], presence of organ system dysfunction[OR=12.143, P= 0.008, 95%CI 1.947- 75.736], need for mechanical ventilation[OR=34.000, P= 0.001, 95%CI 5.272-219.262], IS [OR=8.5, P= 0.001, 95%CI 1.318-54.817]were the predictors ofhigh mortality in pediatric cancer patients. PRISM III score ≥ 13 was a predictive criteria of PICU mortality. CONCLUSION: We conclude that the key to improving survival rates is to pick up on this group of patients as soon as possible.We, believe that cancer patients could be saved by earlier evaluation and intervention by the PICU team when they have a less severe disease.

Could you say that was an atrial flutter or not?

Özsoylu S, Akyildiz BN, Dursun A, Pamukçu Ö. Could you say that was an atrial flutter or not? Turk J Pediatr 2019; 61: 608-610. Muscle-tremor artefact is a potential cause of misdiagnosis of atrial arrhythmias on electrocardiography (ECG) monitoring. Such errors may lead to inappropriate and potentially dangerous therapies in some patients. We present a case of a patient with uncontrolled seizures whose bedside electrocardiogram monitor analysis appeared to demonstrate atrial flutter with 4:1 conduction through the AV node. The ECG monitor and ECG rhythm strip additionally showed a regular ventricular rate of 94 bpm with an underlying regular `saw-tooth` baseline. We applied cardioversion to convert to sinus rhythm. Amiodarone was loaded and added to the patients therapy who had atrial flutter after cardioversion. Echocardiogram was performed by a pediatric cardiologist and they noted that the atrial rate and ventricular rate were equal. After this, we began to suspect this situation might be a pseudoflutter due to his muscle contractions. We applied rocuronium to the patient to understand whether this was a pseudo-flutter or not. We saw that the ECG returned to normal sinus rhythm. Physicians especially working in intensive care units should be aware of artifact to avoid unnecessary therapeutic procedures. As Hippocrates said centuries ago `First, do no harm.`.

Ogilvie syndrome presenting with septic shock.

Özsoylu S, Akyildiz BN, Dursun A. Ogilvie syndrome presenting with septic shock. Turk J Pediatr 2018; 60: 225-227. Acute colonic pseudo-obstruction (ACPO) is also known as Ogilvie`s. We report a 10-year-old child with an unremarkable past history who presented with septic shock including hypotension, prolonged capillary refill time, decreased urine output ( < 0.5 ml/kg/h), metabolic acidosis, liver failure, respiratory failure. The symptoms resolved with supportive therapy. In our patient septic shock contributed to Ogilvie syndrome. Although it is a rare condition in pediatric population, pediatricians should be aware of children with abdominal distention; supportive management is successful and morbidity/mortality is minimal.

Neutrophil-to-lymphocyte ratio and mean platelet volume can be useful markers to predict sepsis in children.

OBJECTIVES: Neutrophil-to-Lymphocyte Ratio (NLR) and Mean Platelet Volume (MPV) have been found to be useful indexes for the diagnosis of sepsis in adults. However, the knowledge of their roles and cut-off values in pediatric patients is limited. The primary objective of this study was to assess the ability of NLR and MPV to predict sepsis in children. A secondary aim was to evaluate the comparison of these parameters with C-reactive Protein (CRP). METHODS: The study was conducted on pediatric patients, who had two or more of the following criteria were included in the study: tachycardia, tachypnea, temperature change, leukocytosis, or leukopenia for age. Patients were classified into sepsis and non-sepsis groups. The sepsis group was defined as the presence of two or more age specific Systemic Inflammatory Response Syndrome (SIRS) criteria and increased Procalcitonin (PCT) level (>0.5 ng/ml). RESULT: The median age of the study population was 18 (6-169) months. Two hundred-sixty four episodes of sepsis were recorded in 125 patients. Eighty two were classified as sepsis and 182 as non-sepsis. CRP level and MPV value were significantly higher in the sepsis group compared to non-sepsis group. The median CRP level was 47.8 mg/dl (10.2-119.5) in the sepsis group and 18.6 mg/dl (4.9-66.1) in the non-sepsis group (p=0.006). In the sepsis group, the median MPV value was 8.4 (7.6-9.5) and it was 7.8 (7.1-8.5) in the non-sepsis group (p=0.001). Significant correlations were found between the procalcitonin (PCT) and CRP level (p<0.001; r = 0.279), NLR (p=0.02; r = 0.186) and MPV (p<0.001; r = 0.243). MPV had the highest specificity for predicting sepsis (75.8%). The largest AUC was 0.629 with a cut-off value 8.5 for MPV, while the AUC was 0.557 with a cut-off value 1.97 for NLR and 0.606 with a cut-off value 38.9 for CRP. CONCLUSIONS: NLR and MPV values should alert clinicians to the possibility of sepsis and to initiate or change antibiotic treatment.

A case of DRESS syndrome associated with carbamazepine treatment.

Fever and rash associated in a wide clinical spectrum, drug rash with eosiophilia and systemic symptoms syndrome (DRESS) is a potentially life-threatening hypersensitivity reaction. Early diagnosis and treatment and removal of the offending agent can be life-saving. Physicians should be aware of DRESS syndrome, particularly in patients receiving antiepileptic medication and admitted with a symptoms of fever and skin rash. In this study, a girl aged three years who had been under carbamazepine therapy for one month was admitted to our hospital with symptoms of fever and rash and was diagnosed as having DRESS syndrome, is presented to increase awareness of DRESS syndrome among physicians.

New Biomarkers to Diagnose Ventilator Associated Pneumonia: Pentraxin 3 and Surfactant Protein D.

OBJECTIVE: To detect the most effective biomarker to confirm ventilator associated pneumonia (VAP). METHODS: Fifty patients with VAP suspicious diagnosis and 30 healthy patients were recruited. Suspicion of VAP was established if patients met the modified CPIS score ≥ 6 points. The confirmation of VAP was defined by the quantitative culture of nonbronchoscopic bronchoalveolar lavage (BAL) >105 CFU/ml of pathogenic microorganism. Serum samples for determination of C-reactive protein (CRP), procalcitonin (PCT), pentraxin 3 (PTX3), surfactant protein D (SPD) were collected on suspected VAP. RESULTS: Twenty seven of 50 patients were accepted as confirmed VAP group whose nonbronchoscopic BAL cultures were positive and rest of them were accepted as unconfirmed VAP group. PTX3, PCT and SPD levels were significantly higher in confirmed VAP group, (P = 0.021, P = 0.007, P < 0.001 respectively). There were no significant differences in CRP levels between the two groups (P = 0.062). The most sensitive marker for diagnosing VAP was SPD (P < 0.001). Receiver operating characteristic (ROC) curve for modified clinical pulmonary infection score (CPIS) to confirm VAP was evaluated (AUC 0.741 ± 0.07, P < 0.001) and the optimal cutoff value was >7 with a sensitivity of 51.85% and a specificity of 91.3%. SPD levels were significantly higher in Acinetobacter baumannii and Pseudomonas aeruginosa infected patients than culture negative patients (P < 0.001). CONCLUSIONS: The index findings suggest that serum SPD is the most sensitive biomarker in diagnosis of VAP and it can be used as an early and organism specific marker for Acinetobacter baumannii and Pseudomonas aeruginosa.

Evaluating the basic life support knowledge among schoolteachers: A cross-sectional survey in Kayseri, Turkey.

Dursun A, Özsoylu S, Emeklioglu B, Akyildiz BN. Evaluating the basic life support knowledge among schoolteachers: A cross-sectional survey in Kayseri, Turkey. Turk J Pediatr 2018; 60: 702-708. Children spend a significant proportion of their day in school when they are not with their families.Therefore they might experience medical emergency situations due to injuries, complications of chronic health conditions, or unexpected major illnesses that occur in school. In cases of emergencies, school teachers are expected to play a key role in performing basic life support (BLS) on school children. Very limited data are present in the literature that address the knowledge of the schoolteacher regarding BLS. The primary objective of this study was to asses the BLS knowledge, training status and attitude towards pediatric BLS among schoolteachers. The study had a cross-sectional research design and was conducted between January and March 2017. A self administered questionnaire was used for data collection to assess the knowledge of teachers. The questionnaires were filled in by 541 teachers (243 male and 298 women). The median age of the study population was 39 (34-45) years. One-third of the respondents reported having taken a BLS class in the past (33.1%). The mean for the correct answers for the study population was 5/14 (4/14-7/14). For trained teachers, it was 6/14 (4/14-8/14) and for untrained teachers, it was 5/14 (3/14-7/14)(P < 0.001). There were no differences between teachers who had attended different BLS courses. Significant differences between teachers were observed: 62% of teachers with previous BLS training felt capable of providing cardio pulmonary resusitation (CPR) to their students compared to 48% in the group without previous training (P =0.001). Ninety- five percent of the teachers were eager to attend a BLS course and 92% reported that BLS training should be mandatorily given for teacher certification. Teachers are aware of the importance of BLS and they are willing to attend BLS training programs and improve their knowledge. Despite the fact that the knowledge of trained teachers was found to be better than those of untrained teachers, school teachers in Turkey have a low level of knowledge and skills regarding BLS.

Neuroinfluenza: evaluation of seasonal influenza associated severe neurological complications in children (a multicenter study).

PURPOSE: Although influenza primarily affects the respiratory system, in some cases, it can cause severe neurological complications. Younger children are especially at risk. Pediatric literature is limited on the diagnosis, treatment, and prognosis of influenza-related neurological complications. The aim of the study was to evaluate children who suffered severe neurological manifestation as a result of seasonal influenza infection. METHODS: The medical records of 14 patients from six hospitals in different regions of the country were evaluated. All of the children had a severe neurological manifestations related to laboratory-confirmed influenza infection. RESULTS: Median age of the patients was 59 months (6 months-15.5 years) and nine (64.3%) were male. Only 4 (28.6%) of the 14 patients had a comorbid disease. Two patients were admitted to hospital with influenza-related late complications, and the remainder had acute complication. The most frequent complaints at admission were fever, altered mental status, vomiting, and seizure, respectively. Cerebrospinal fluid (CSF) analysis was performed in 11 cases, and pleocytosis was found in only two cases. Neuroradiological imaging was performed in 13 patients. The most frequent affected regions of nervous system were as follows: cerebellum, brainstem, thalamus, basal ganglions, periventricular white matter, and spinal cords. Nine (64.3%) patients suffered epileptic seizures. Two patients had focal seizure, and the rest had generalized seizures. Two patients developed status epilepticus. Most frequent diagnoses of patients were encephalopathy (n = 4), encephalitis (n = 3), and meningitis (n = 3), respectively. The rate of recovery without sequelae from was found to be 50%. At discharge, three (21.4%) patients had mild symptoms, another three (21.4%) had severe neurological sequelae. One (7.1%) patient died. The clinical findings were more severe and outcome was worse in patients <5 years old than patients >5 years old and in patients with comorbid disease than previously healthy group. CONCLUSION: Seasonal influenza infection may cause severe neurological complications, especially in children. Healthy children are also at risk such as patients with comorbid conditions. All children who are admitted with neurological findings, especially during the influenza season, should be evaluated for influenza-related neurological complications even if their respiratory complaints are mild or nonexistent.

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.

Childhood stroke: results of 130 children from a reference center in Central Anatolia, Turkey.

BACKGROUND: Although stroke among children is rare, it can cause significant morbidity and mortality. We aim to share our experience of children with arterial ischemic stroke. METHODS: The initial symptoms, demographical features, risk factors, neurological examination, neuroradiological findings, and clinical follow-up data of 130 Turkish children seen between 2002 and 2013 were retrospectively analyzed. RESULTS: Sixty-eight patients were male. Thirty of the children were aged from 1 to 12 months (seven of them died in this period). Focal neurological signs were the most common presentation, and hemiplegia or hemiparesis were the most common focal signs. Underlying risk factors were detected in 103 patients. Infections and congenital heart disease were the most common risk factors. Seven of the nine children with recurrent arterial ischemic strokes had one or more underlying diseases (moyamoya disease in two children along with factor V Leiden mutation, tuberculous meningitis, congenital heart disease, homocystinuria, and hemiconvulsion-hemiplegia-epilepsy syndrome). The arterial ischemic stroke was located in the middle cerebral circulation in 68 (36 left and 32 right) and in the posterior cerebral artery in 41. Eighteen children died. The neurological outcome was assessed in 98 children. Of these children, 66 children have neurological deficits and 52 children have seizures. Stroke in the first year of life is more often fatal. Recurrent stroke is associated with poor prognosis. CONCLUSION: Tuberculous meningitis is still a risk factor for arterial ischemic stroke in Turkey. Arterial ischemic stroke in the first year of life and recurrent arterial ischemic stroke represent poor prognostic features.

Familial clustering of HBV in families with children who are diagnosed as chronic hepatitis B or inactive carriers of HBV.

The aim of this study was to assess and compare the spread of HBV infection in families with children who are diagnosed as chronic hepatitis B or are inactive carriers of HBV. A total of 570 patients aged 2-16 years and 2358 family members were included in the study. Patients were classified as inactive carriers (Group 1, 350 patients) or patients diagnosed as chronic active hepatitis B (Group 2, 220 patients). Demographic features of the families, HBV serological markers and routes of transmission were evaluated. Parental transmission was lower compared to nonparental transmission (34.8% and 65.1%, respectively). HBsAg positivity rate was found to be highest among mothers in both of the groups. HBeAg positivity and anti-HBs positivity were significantly higher in family members of Group 2. Transmission rates were significantly higher in families consisting of five or more members compared to families consisting of less than five members. HBsAg positivity of siblings was lowest when both parents were HbsAg negative and highest when both parents were HBsAg positive in both groups. A high risk of HBV transmission among mothers of HBsAg carriers and patients with chronic hepatitis B was demonstrated.

Anomalous left coronary artery arising from the pulmonary artery.

A 13-year-old boy was found unconscious. Ventricular fibrillation was seen in his admission to the emergency room. Dilated right coronary artery and increased coronary arterial flow were seen in echocardiography. In the angiography, left coronary artery abnormally arising from pulmonary artery and aneursymatic dilatation in the right coronary artery was seen. .

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.

Case series of mercury toxicity among children in a hot, closed environment.

Mercury poisoning is much more prevalent in the general population than possibly many physicians realize. We present data on 26 pediatric cases with mercury intoxication from exposure to mercury by inhalation or skin contact as a result of a broken thermometer in a school laboratory. This is the largest pediatric series in Turkey. During a 3-month period, the study team observed the children for clinical symptoms, physical findings, and blood and mercury levels. Of all patients, 21 inhaled, 3 inhaled and touched the element, and 2 took the mercury home. Sixteen children were symptomatic at admission, although blood mercury levels in the symptomatic children were higher than those in asymptomatic children (P = 0.003). The urine mercury levels were not statistically different between the groups at the admission (P > 0.05). The exposure times were 3.5 and 2 hours for symptomatic and asymptomatic children, respectively (P = 0.003). The 2 children who took the mercury home had the highest blood mercury levels and the most prolonged exposure time. N-acetylcysteine and chelation treatments were started in 21 children who had symptoms of mercury intoxication and high mercury levels in their blood or urine. No adverse effects were observed during chelation therapy. Prompt removal of children from contaminated environments and proper decontamination or elimination of devices containing large amounts of mercury from schools are necessary to prevent serious complications caused by exposure to mercury.

Treatment of pediatric refractory status epilepticus with topiramate.

OBJECTIVE: We evaluated a topiramate (TPM) regimen for treating refractory status epilepticus in the largest pediatric series, reported to date. METHODS: Fourteen patients received TPM via the nasogastric route. Initially, all patients received TPM as a 5 mg/kg loading dose followed by 5 mg/kg/day in two doses as maintenance. Thereafter, patients were divided into three groups based on the response to TPM therapy and seizure cessation time (full responder, partial responder, and nonresponder). Four patients received only thiopental, two received thiopental, and high-dose midazolam, one received thiopental, high-dose midazolam, and propofol, two received only propofol, one received propofol, and high-dose midazolam and four patients were on a high-dose midazolam infusion. RESULTS: The median time to seizure cessation was 5.5 h (range 2-48 h). Nine patients were full responders, three were partial responders, and two were nonresponders At follow-up, six patients were weaned successfully from thiopental, two patients from high-dose midazolam and three patients from propofol. Three patients developed mild metabolic acidosis during TPM theraphy. CONCLUSIONS: Most of the patients responded to this treatment which was well tolerated. So we recommended its use for terminating refractory status epilepticus in children.

Cytomegalovirus infection and haemophagocytosis in a patient with congenital nephrotic syndrome.

Congenital nephrotic syndrome is a rare clinical entity defined as massive proteinuria leading to symptoms within the infant's first 3 months of life. Although the association between congenital nephrotic syndrome and cytomegalovirus infection has been identified, association with haemophagocytosis has not been reported in the literature. In this case report we describe concomitant cytomegalovirus infection and haemophagocytosis in a 3-month-old girl with congenital nephrotic syndrome.