A rare complication of leukostasis with AML M4 patient: Microcirculatory dysfunction of upper extremities.

BACKGROUND: In children with acute myeloid leukemia, the incidence of hyperleukocytosis is 5-33%. Patients with AML and hyperleukocytosis have a higher early mortality rate than patients with nonhyperleukocytic AML because of the increased risk of severe pulmonary and neurologic complications. Leukapheresis provides rapid cytoreduction and reduces early mortality rates. CASE PRESENTATION: In this report, we present a case with microcirculatory failure of upper extremities as a rare symptom of hyperleukocytic AML M4 at initial presentation. CONCLUSIONS: Early diagnosis and treatment of patients with AML admitted to emergency services with these symptoms is too important to prevent from loss of extremities. Most of the complications of hyperleukocytosis can be reversible with early treatment.

Severe influenza virus infection in children admitted to the PICU: Comparison of influenza A and influenza B virus infection.

Although the influenza virus usually causes a self-limiting disease, deaths are reported even in children without risk factors. We aimed to identify the clinical features, mortality associated with severe influenza A and B virus infections of children admitted to the pediatric intensive care unit (PICU). We conducted a retrospective study of children with confirmed influenza infection between 2012 and 2019 who were admitted to the PICU. Demographic features, risk factors, clinical data, microbiological data, complications, and outcomes were collected. Over seven influenza seasons (2012-2011 to 2015-2016), 713 children diagnosed with laboratory-confirmed influenza-related LRTI, and PICU admission was needed in 6% (46/713) of the patients. Thirty-one patients (67.4%) were diagnosed with influenza A and 15 patients were diagnosed with influenza B. Epidemiologic and clinical characteristics were similar in both influenza types, lactate dehydrogenase levels were significantly higher for influenza A than for influenza B infections. Although the influenza A to B ratio among the patients admitted to the PICU was 2.06, the percentage of cases requiring PICU admission was nearly two times higher in influenza B cases. There was no statistically significant difference in disease severity and complications in patients with influenza A and influenza B.

Multisystem inflammatory syndrome in children associated with COVID-19 in 101 cases from Turkey (Turk-MISC study).

AIM: Multisystem inflammatory syndrome in children (MIS-C) may cause shock and even death in children. The aim of this study is to describe the clinical features, laboratory characteristics and outcome of children diagnosed with MIS-C in 25 different hospitals in Turkey. METHODS: The retrospective study was conducted between 8 April and 28 October 2020 in 25 different hospitals from 17 cities. Data were collected from patients' medical records using a standardised form. Clinical and laboratory characteristics and outcomes according to different age groups, gender and body mass index percentiles were compared using multivariate logistic regression analysis. RESULTS: The study comprised 101 patients, median age 7 years (interquartile range (IQR) 4.6-9.3); 51 (50.5%) were boys. Reverse-transcriptase polymerase chain reaction (PCR) assay was positive in 21/100 (21%) patients; 62/83 (74.6%) patients had positive serology for SARS-CoV-2. The predominant complaints were fever (100%), fatigue (n = 90, 89.1%), and gastrointestinal symptoms (n = 81, 80.2%). Serum C-reactive protein (in 101 patients, median 165 mg/L; range 112-228), erythrocyte sedimentation rate (73/84, median 53 mm/s; IQR 30-84) and procalcitonin levels (86/89, median 5 µg/L; IQR 0.58-20.2) were elevated. Thirty-eight patients (37.6%) required admission to intensive care. Kawasaki disease (KD) was diagnosed in 70 (69.3%) patients, 40 of whom had classical KD. Most patients were treated with intravenous immunoglobulin (n = 92, 91%) and glucocorticoids (n = 59, 58.4%). Seven patients (6.9%) died. CONCLUSION: The clinical spectrum of MIS-C is broad, but clinicians should consider MIS-C in the differential diagnosis when persistent fever, fatigue and gastrointestinal symptoms are prominent. Most patients diagnosed with MIS-C were previously healthy. Immunomodulatory treatment and supportive intensive care are important in the management of cases with MIS-C. Glucocorticoids and intravenous immunoglobulins are the most common immunomodulatory treatment options for MIS-C. Prompt diagnosis and prompt treatment are essential for optimal management.

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.

Daily Practice of Mechanical Ventilation and Weaning in Turkish PICUs: A Multicenter Prospective Survey.

OBJECTIVES: To investigate conventional mechanical ventilation weaning characteristics of patients requiring conventional mechanical ventilation support for greater than 48 hours within the PICU. DESIGN: The prospective observational multicenter cohort study was conducted at 15 hospitals. Data were being collected from November 2013 to June 2014, with two designated researchers from each center responsible for follow-up and data entry. SETTING: Fifteen tertiary PICUs in Turkey. PATIENTS: Patients between 1 month and 18 years old requiring conventional mechanical ventilation for greater than 48 hours were included. A single-center was not permitted to surpass 20% of the total sample size. Patients with no plans for conventional mechanical ventilation weaning were excluded. INTERVENTIONS: Conventional mechanical ventilation MEASUREMENTS AND MAIN RESULTS:: Pertinent variables included PICU and patient demographics, including clinical data, chronic diseases, comorbid conditions, and reasons for intubation. Conventional mechanical ventilation mode and weaning data were characterized by daily ventilator parameters and blood gases. Patients were monitored until hospital discharge. Of the 410 recruited patients, 320 were included for analyses. A diagnosis of sepsis requiring intubation and high initial peak inspiratory pressures correlated with a longer weaning period (mean, 3.65 vs 1.05-2.17 d; p < 0.001). Conversely, age, admission Pediatric Risk of Mortality III scores, days of conventional mechanical ventilation before weaning, ventilator mode, and chronic disease were not related to weaning duration. CONCLUSIONS: Pediatric patients requiring conventional mechanical ventilation with a diagnosis of sepsis and high initial peak inspiratory pressures may require longer conventional mechanical ventilation weaning prior to extubation. Causative factors and optimal weaning for this cohort needs further consideration.

Comprehensive Analysis of Severe Viral Infections of Respiratory Tract admitted to PICUs during the Winter Season in Turkey.

OBJECTIVES: To analyze the course of seasonal viral infections of respiratory tract in patients hospitalized in pediatric intensive care units (PICU) of 16 centers in Turkey. MATERIALS AND METHODS: It is a retrospective, observational, and multicenter study conducted in 16 tertiary PICUs in Turkey includes a total of 302 children with viral cause in the nasal swab which required PICU admission with no interventions. RESULTS: Median age of patients was 12 months. Respiratory syncytial virus (RSV) was more common in patients over one year of age whereas influenza, human Bocavirus in patients above a year of age was more common (p <0.05). Clinical presentations influencing mortality were neurologic symptoms, tachycardia, hypoxia, hypotension, elevated lactate, and acidosis. The critical pH value related with mortality was ≤7.10, and critical PCO2 ≥60 mm Hg. CONCLUSION: Our findings demonstrate that patients with neurological symptoms, tachycardia, hypoxia, hypotension, acidosis, impaired liver, and renal function at the time of admission exhibit more severe mortal progressions. Presence of acidosis and multiorgan failure was found to be predictor for mortality. Knowledge of clinical presentation and age-related variations among seasonal viruses may give a clue about severe course and prognosis. By presenting the analyzed data of 302 PICU admissions, current study reveals severity of viral respiratory tract infections and release tips for handling them. HOW TO CITE THIS ARTICLE: Kockuzu E, Bayrakci B, Kesici S, Citak A, Karapinar K, Emeksiz S, et al. Comprehensive Analysis of Severe Viral Infections of Respiratory Tract admitted to PICUs During the Winter Season in Turkey. Indian J Crit Care Med 2019;23(6):263-269.

The evaluation of safety and efficacy of colistin use in pediatric intensive care unit: Results from two reference hospitals and review of literature.

Colistin, an old cationic polypeptide antibiotic, have been reused due to rising incidence of infections caused by multi-drug resistant (MDR) Gram-negative microorganisms and the lack of new antibiotics. Therefore, we evaluated safety and efficacy of colistin in treatment of these infections. This study included 104 critically ill children with a median age of 55,9 months between January 2011 and January 2016. Nephrotoxicity occurred in 11 (10.5%) patients. Nephrotoxicity occurred between the third and seventh day of treatment in 63% of colistin induced nephrotoxicity episodes. The subgroup analysis between the patients who developed nephrotoxicity during colistin treatment and those that did not, showed no significant difference in terms of age, underlying disease, cause for PICU admission and type of infection required colistin treatment, P values were 0.615, 0.762, 0.621, 0.803, respectively. All patients were receiving a concomitant nephrotoxic agent (P = 0,355). The majority of the patients (52%) were having primary or secondary immune deficiency in treatment failure group and the most common cause of PICU admission was sepsis in treatment failure group, P values were 0.007 and 0.045, respectively. Mortality attributed to colistin failure and crude mortality were 14.4% and 29.8%, respectively. In conclusion, colistin may have a role in the treatment of infections caused by multidrug-resistant Gram-negative bacteria in critically ill children. However, the patients have to be followed for side effects throughout colistin treatment, not for only early stage. And the clinicians should be aware of increase in the rate of nephrotoxicity in patients those have been receiving a concomitant nephrotoxic agent.

Inferior Petrosal Sinus Thrombosis in a Child due to Malposition of Central Venous Catheter: A Case Report.

OBJECTIVE: To report a case of inferior petrosal sinus thrombosis associated with malposition of central venous catheterization (CVC). CLINICAL PRESENTATION AND INTERVENTION: A 5-month-old boy was admitted to Sifa University Hospital because of pneumonia. When exophthalmos occurred in the right eye, he was referred to Ege University Hospital. Cranial magnetic resonance imaging and magnetic resonance venography confirmed that the catheter in the right inferior petrosal sinus caused the thrombosis. The catheter was extracted and anticoagulant treatment was started. CONCLUSION: In this case report, malpositioning of the CVC was the cause of the thrombosis. To minimize such complications, catheterization should be done with the supervision of an expert and postprocedure radiography should also be performed.

Trichosporon asahii, sepsis, and secondary hemophagocytic lymphohistiocytosis in children with hematologic malignancy.

Trichosporon asahii (T. asahii) is an uncommon fungal pathogen rarely seen in patients with hematologic malignancies. Although appropriate therapy is started, infection with T. asahii usually leads to mortality. Here, we describe two patients developed severe T. asahii infection and secondary HLH. Despite rapid identification of T. asahii and negative blood cultures achieved by prompt initiation of treatment with voriconazole, fever and pancytopenia, persisted and both developed hepatosplenomegaly, and their clinical state worsened. Bone marrow aspiraton revealed hemophagocytosis. Elevated ferritin, triglyceride levels were seen. The first patient did not receive HLH directed therapy and died with multiple organ dysfunctions. Prompt diagnosis and treatment of secondary HLH led to rapid improvement in clinical and laboratory abnormalities in the second patient and kept her alive. We suggest that HLH may present as a secondary condition, accompanying a severe infection with T. asahii may, at least in part, contribute to high mortality rates in these cases.

Leukapheresis in childhood acute leukemias: single-center experience.

Hyperleukocytosis in patients with leukemia is associated with early mortality, especially due to the pulmonary and neurological complications of leukostasis. The prompt use of leukapheresis may improve patients' survival in the initial treatment period. The medical records of all previously untreated acute leukemia patients were reviewed to determine whether there was hyperleukocytosis at presentation. This study summarizes a single-center experience of leukapheresis that was applied to 12 children with acute leukemia and hyperleukocytosis. The median leukocyte count at diagnosis was 589,000/mm(3) (range: 389,000-942,000/mm(3)) for ALL patients and 232,000/mm(3) (range: 200,000-282,000/mm(3)) for AML patients. A central venous catheter (CVC) was inserted, and leukapheresis procedures were repeated at 12-hour intervals. A total of 29 leukapheresis cycles were performed on 12 children. The median number of cycles of leukapheresis required by each patient was two (range: 1-4). The median absolute and percentage reductions in white blood cell (WBC) count after the first cycle of leukapheresis were 113,000/mm(3) (range: 55,000-442,000/mm(3)) and 36% (range: 16-57.4%), respectively. As a laboratory finding, mild hypocalcemia was the most frequently observed complication. No patients developed any other problem related to the procedure. Our results showed that leukapheresis is a safe and effective procedure if performed by experienced staff.

Therapeutic plasma exchange in critically ill children: A single center experience.

INTRODUCTION: Therapeutic plasma exchange (TPE) is used in a wide spectrum of diseases in critically ill pediatric patients. We aim to review the indications, complications, safety, and outcomes of critically ill children who received TPE. METHODS: All of the TPE procedures performed in a pediatric intensive care unit providing tertiary care during 19 years (January 2013-January 2023) were evaluated retrospectively. A total of 154 patients underwent 486 TPE sessions. RESULTS: Median age was 6 years (2-12.5) and 35 children had a body weight of <10 kg (22.7%). Number of organ failure was 4 (2-6). Liver diseases were the most common indication for TPE (31.2%) followed by sepsis with multiorgan dysfunction syndrome (27.3%). Overall survival rate was 72.7%. The highest mortality was observed in hemophagocytic lymphohistiocytosis group. Non-survivors had significantly higher number of organ failure (p < 0.001), higher PRISM score (p < 0.001), and higher PELOD score on admission (p < 0.001). Adverse events were observed in 68 (13.9%) sessions. Hypotension (7.8%) and hypocalcemia (5.1%) were the most frequent adverse events. CONCLUSION: TPE is safe for critically ill pediatric patients with experienced staff. Survival rate may vary depending on the underlying disease. Survival decreases with the increase in the number of failed organs.

Sepsis associated acute kidney injury in pediatric intensive care unit.

BACKGROUND: We aimed to compare the acute kidney injury (AKI) incidence in pediatric septic shock patients according to the three different classifications. METHODS: We analyzed retrospectively 52 patients with severe sepsis between January 2019 and December 2019. RESULTS: While 21 patients have been diagnosed with SA-AKI according to the pRIFLE criteria, 20 children have been diagnosed according to the AKIN criteria, and 21 children have been diagnosed according to the KDIGO criteria. Older age, lower platelet count were determined as independently risk factor for SA-AKI. Older age and higher PRISM score were associated with mortality. According to Canonical correlation coefficients, pRIFLE is the most successful classification to distinguish AKI state. The canonical correlation coefficients for pRIFLE, KDIGO, and AKIN were 0.817, 0.648, and 0.615, respectively. CONCLUSION: Although AKI incidence was similar between the three classifications, pRIFLE was the most successful classification to distinguish AKI state.

Hyperferritinemia in the critically ill child with secondary hemophagocytic lymphohistiocytosis/sepsis/multiple organ dysfunction syndrome/macrophage activation syndrome: what is the treatment?

INTRODUCTION: Hyperferritinemia is associated with increased mortality in pediatric sepsis, multiple organ dysfunction syndrome (MODS), and critical illness. The International Histiocyte Society has recommended that children with hyperferritinemia and secondary hemophagocytic lymphohistiocytosis (HLH) or macrophage activation syndrome (MAS) should be treated with the same immunosuppressant/cytotoxic therapies used to treat primary HLH. We hypothesized that patients with hyperferritinemia associated secondary HLH/sepsis/MODS/MAS can be successfully treated with a less immunosuppressant approach than is recommended for primary HLH. METHODS: We conducted a multi-center cohort study of children in Turkish Pediatric Intensive Care units with hyperferritinemia associated secondary HLH/sepsis/MODS/MAS treated with less immunosuppression (plasma exchange and intravenous immunoglobulin or methyl prednisolone) or with the primary HLH protocol (plasma exchange and dexamethasone or cyclosporine A and/or etoposide). The primary outcome assessed was hospital survival. RESULTS: Twenty-three children with hyperferritinemia and secondary HLH/sepsis/MODS/MAS were enrolled (median ferritin = 6341 µg/dL, median number of organ failures = 5). Univariate and multivariate analyses demonstrated that use of plasma exchange and methyl prednisolone or intravenous immunoglobulin (n = 17, survival 100%) was associated with improved survival compared to plasma exchange and dexamethasone and/or cyclosporine and/or etoposide (n = 6, survival 50%) (P = 0.002). CONCLUSIONS: Children with hyperferritinemia and secondary HLH/sepsis/MODS/MAS can be successfully treated with plasma exchange, intravenous immunoglobulin, and methylprednisone. Randomized trials are required to evaluate if the HLH-94 protocol is helpful or harmful compared to this less immune suppressive and cytotoxic approach in this specific population.

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.

Cardiac failure in a child with tuberculous meningitis as a complication of Paroxysmal sympathetic hyperactivity.

BACKGROUND: Paroxysmal sympathetic hyperactivity (PSH) is a disorder due to the loss of regulation of autonomic activity. The most common condition predisposing to the development of PSH is traumatic brain injury (TBI), followed by anoxic brain injury, stroke, tumors, and infections. Awareness about the condition and early recognition is important to avoid life threatening complications. CASE: We report a 4-year-old child with tuberculous meningitis with symptoms of PSH who developed cardiac failure. PSH episodes were treated with beta blocker, benzodiazepine, morphine, dexmedetomidine, baclofen, and tizanidine. Three weeks after readmission PSH episodes decreased and the patient was transferred to the general ward. CONCLUSIONS: PSH assessment tool has benefits such as monitoring the patient, evaluating response to treatment and early diagnosing PSH patients.

First Successful Organ Procurement From a Pediatric Patient With a Nonpulsatile Ventricular Assist Device.

Left ventricular assist devices have become an important therapeutic option as a mechanical circulatory support system in the treatment of end-stage heart failure. Organ transplants from brain dead donors on mechanical circulatory support are rare. In the literature, many successful solid-organ transplants have been reported using these donors. However, to our knowledge, this is the first report of successful solid-organ transplant from a child donor with a nonpulsatile ventricular assist device.

A rescue therapy with a combination of caspofungin and liposomal amphotericin B or voriconazole in children with haematological malignancy and refractory invasive fungal infections.

Combination treatment of paediatric invasive fungal infections (IFIs) has rarely been reported. A total of 17 children with 19 IFI episodes were enrolled in the study. The median age of the patients was 5.3 (range 0.5-17) years. IFI was classified as proven in 4, probable in 12 and possible in 3 episodes. These patients received empiric antifungal treatment, which consisted of liposomal amphotericin B (LAmB) monotherapy for a median duration of 12 days (range 3-69 days). All patients were refractory to LAmB; therefore, caspofungin was added to the therapy in 11 patients. In the remaining six patients, LAmB was ceased and a combination of caspofungin and voriconazole was started. Among the patients who received caspofungin + LAmB, four did not show favourable response and the combination was switched to caspofungin + voriconazole. The median (range) and total duration of the therapy were 7 (3-14) days and 91 patient days for LAmB + caspofungin combination and 49 (7-126) days and 516 patient days for caspofungin + voriconazole combination. We found a favourable response rate of 68.4% in 16 proven or probable IFI episodes. Twelve-week survival rate of these patients was 75%. No serious side effect was observed among the patients. Our data suggest that combination antifungal therapy is safe and effective in children with haematological malignancies.

Surface TREM-1 as a Prognostic Biomarker in Pediatric Sepsis.

OBJECTIVES: To investigate the association between the triggering receptor expressed on myeloid cells-1 (TREM-1) levels and prognosis in septic children. METHODS: Patients admitted to pediatric intensive care units (PICU) of three tertiary centers were included in this prospective observational study. Serum samples were taken at admission from patients who were hospitalized with sepsis. RESULTS: Of the 87 patients included, 34 (39.1%) had severe sepsis and 53 (60.9%) had septic shock. The median age was 2 y (2 mo to 16 y). TREM-1 values were found to be significantly higher in septic shock patients 129 pg/ml (min 9.85- max 494.90) compared to severe sepsis 105 pg/ml (min 8.21- max 289.17) (p = 0.048). Despite higher TREM-1 levels been measured in non-survivors compared to survivors, it was not statistically significant [168.98 pg/ml (min 9.85- max 494.90) vs. 110.79 pg/ml (min 8.21- max 408.90), (p = 0.075)]. CONCLUSIONS: Admission TREM-1 levels were higher in septic shock compared to severe sepsis patients. There was no association between mortality and TREM-1 levels in sepsis. TREM-1 measurements should be used carefully in pediatric sepsis prognosis.

An unusual cause of multiple organ dysfunction syndrome in the pediatric intensive care unit: hemophagocytic lymphohistiocytosis.

OBJECTIVE: To report our experience in children with primary or secondary hemophagocytic lymphohistiocytosis (HLH) presented with multiple organ dysfunction syndrome (MODS) in pediatric intensive care unit (PICU). DESIGN: The records of patients with a diagnosis of HLH and MODS between January 2005 and January 2008 were reviewed. The patients' characteristics, treatment modalities, and outcomes were assessed. SETTING: PICU of Ege University Hospital. PATIENTS/SUBJECTS: Twelve children who were hospitalized in the PICU met the diagnostic criteria for HLH, and presented with MODS were entered into the study. RESULTS: The median age of the patients was 3 years (range, 2 months-15.5 years). Six patients had a history of parental consanguinity and two had an affected sibling. Five of the patients were classified as primary HLH. All of the patients had hepatosplenomegaly, elevated ferritin levels, hypofibrinogenemia, anemia, thrombocytopenia, and hemophagocytosis in bone marrow examination at presentation. The median Pediatric Logistic Organ Dysfunction score of the patients at onset was 51 (range, 12-62). Four patients had six, four had five, two had four, and the remaining two had three organ dysfunctions. Organ dysfunction, other than hematologic dysfunction which was present in all patients, was most commonly seen in hepatic (n = 11, 91.7%), respiratory (n = 11, 91.7%), and cardiovascular systems (n = 10, 83.3%). Although nine patients showed neurologic dysfunction including convulsion and coma, renal failure was detected in five patients. Eleven patients were supported with mechanical ventilation and four patients required hemodialysis. Eight patients were treated according to the HLH 2004 treatment protocol, consisting of cyclosporine A, etoposide, and dexamethasone. The remaining four patients received only intravenous immunoglobulin and supportive treatment. Seven of the patients died. CONCLUSION: HLH is a frequently lethal disease and with a clinical presentation similar to severe sepsis, MODS, disseminated intravascular coagulation, or septic shock, which are frequent diagnoses in the PICU. In the PICU, HLH should be considered in the case of prolonged fever, splenomegaly, cytopenia, and MODS. It is important for pediatricians and particularly pediatric intensivists to know the diagnostic criteria and possible clinical presentations of HLH so treatment is initiated promptly.