Clinical characteristics and outcomes of continuous renal replacement therapy performed on younger children weighing up to 10 kg.

BACKGROUND: This study aimed to investigate the clinical features, modality, complications, and effecting factors on the survival of children weighing up to 10 kg who received continuous renal replacement therapy (CRRT). METHODS: This study was a retrospective observational study conducted in five pediatric intensive care units in tertiary hospitals in Turkey between January 2015 and December 2019. RESULTS: One hundred and forty-one children who underwent CRRT were enrolled in the study. The median age was 6 (range, 2-12)months, and 74 (52.5%) were male. The median weight of the patients was 6 (range, 4-8.35) kg and 52 (36.9%) weighed less than 5 kg. The most common indication for CRRT was fluid overload in 75 (53.2%) patients, and sepsis together with multiorgan failure in 62 (44%). The overall mortality was 48.2%. DISCUSSION: Despite its complexity, CRRT in children weighing less than 10 kg is a beneficial, lifesaving extracorporeal treatment modality.

Epidemiology of pediatric severe sepsis and septic shock in Turkey: Prevalence, Results and Treatments Study.

AIM: This study was aimed at characterizing the prevalence, management, and outcomes of pediatric severe sepsis and septic shock in tertiary pediatric intensive care units (PICUs) in Turkey. METHODS: A point prevalence study was conducted on five days over the course of one year in 29 PICUs in Turkey. Outcomes included severe sepsis and septic shock point prevalence, therapies used, duration of PICU stay, and mortality at day 28. RESULTS: Of the 1757 children who were admitted to the PICU during the study period, 141 (8.0%) children met the consensus criteria for severe sepsis and 23 (1.3%) children met the criteria for septic shock. Pediatric severe sepsis and septic shock accounted for 8% and 1.3% of all PICU admissions, respectively. The median age of the patients was 2.6 years (interquartile range (IQR), 0.7-8.6 years). Enteral nutrition (79.3%) was preferred compared to parenteral nutrition (31.1%) for the first 3 days after PICU admission. A total of 39 patients died while in the PICU, for a 23.8% mortality rate, which did not vary by age. CONCLUSION: The mortality rate was similar to that in other studies. Hematologic-immunologic comorbidity, parenteral nutrition and the use of vasoactive drugs were independently associated with mortality.

Early Neurologic Complications and Long-term Neurologic Outcomes of Extracorporeal Membrane Oxygenation Performed in Children.

BACKGROUND: We aimed at evaluating acute neurologic complications (ANC) and clinical outcome at a 2-year follow-up in children after extracorporeal membrane oxygenation (ECMO). METHODS: We conducted a single-center, retrospective review of our patient cohort aged between 1 month and 18 years at the time of ECMO support (between June 2014 to January 2017). Outcome analysis included ANC and their clinical consequences.The Pediatric Overall Performance Category (POPC) and Pediatric Cerebral Performance Category (PCPC) were used for neurologic assessment performed at discharge from the hospital and at 2nd year follow-up. RESULTS: There were 35 children who required ECMO. The median ECMO time was 9 days (range 2-32 days). Decannulation from ECMO was achieved in 68.6% of patients, and overall, 42.8% survived (15 patients), The incidence of ANC in the surviving patients was 40% (6 children). ANC were intracranial hemorrhage, seizures, cerebral infarction, which occurred in one, two and three of the 15 surviving patients respectively (6.6, 13.3 and 20%). A higher rate of organ failure was related to death (p=0.043), whereas duration on ECMO was a risk factor for the development of ANC (p<0.05). At hospital discharge, the 14 patients evaluated had normal development or -mild disability in 73.2%, and at the 2-year follow-up, 93.4% had these scores. CONCLUSION: Children who receive ECMO have a risk to develop ANC, which was related to the length of ECMO treatment, while survival was related to less organ failure, Long-term neurological outcome was good in our patient cohort.

Clinically mild encephalitis/encephalopathy with a reversible splenial lesion of the corpus callosum in childhood: a single-center experience.

BACKGROUND: Mild encephalitis/encephalopathy with a reversible splenial lesion (MERS) is a rare clinicoradiological syndrome that typically presents with central nervous system symptoms such as loss of consciousness, seizure, headache, and ophthalmoparesis. METHODS: Here, we highlight the characteristics of this syndrome together with the clinical and MRI findings of 6 pediatric patients with MERS. RESULTS: Between January 2017 and October 2020, 6 patients with MERS (3 boys and 3 girls) presented to our center. The mean age was 122 ± 54.6 (min-max: 44-180) months. None of the patients had a chronic disease. In our study, infectious agents were detected in 4 patients (66.6%), while noninfectious causes (one seizure and the other hyponatremia) were detected in two patients. All of our cases were discharged without any sequelae after an average of 12.1 ± 7 (min-max: 4-20) days of hospitalization. In 1 patient (case 6), control MRI could not be performed, and the radiological recovery of our other patients was shown to be between 14 days and 2 months. DISCUSSION: MERS is an acute encephalopathy with good prognosis and should be considered by neurologists in differential diagnosis due to its variable clinical presentation and specific MRI findings.

A novel therapeutic approach using the Zipper method to treat chorea in a pediatric-onset systemic lupus erythematosus patient.

Pediatric-onset systemic lupus erythematosus is among the prototypic systemic autoimmune diseases seen in children. Although the neuropsychiatric involvement rate varies during the course of the disease, it is an important cause of morbidity and mortality. The clinical picture of neuropsychiatric SLE (NPSLE) is highly variable, and neurological features can precede systemic findings, leading to some diagnostic difficulties. NPSLE requires early and aggressive immunosuppressive therapy. Some patients can be resistant to immunosuppressive therapy. Chorea is a rare manifestation that occurs in 1.2%-2% of SLE patients and can result from an immunologically mediated mechanism, antiphospholipid autoantibodies or ischemia. Herein we present the first case of pediatric-onset SLE diagnosed with central nervous system involvement and treated with Zipper method. The Zipper method is a new immunomodulation treatment. The clinical findings of the patient, which were resistant to corticosteroids and cyclophosphamide, resolved by this novel treatment.

Successful Treatment With Bortezomib for Refractory and Complicated Acquired Thrombotic Thrombocytopenic Purpura in an Adolescent Girl.

Thrombotic thrombocytopenic purpura (TTP) is a rare, dangerous, life-threatening disease characterized by microangiopathic hemolytic anemia and thrombocytopenia, along with organ dysfunction due to microangiopathy-related ischemia. Plasma exchange and steroids are used for initial treatment, and rituximab is often used in refractive patients. Caplacizumab, cyclophosphamide, and splenectomy are among other treatment options. It has been reported that bortezomib, a proteasome inhibitor, can be used in the management of refractory acquired TTP. Herein, we present a 16-year-old female patient who was monitored for acquired TTP and treated with high-dose steroids, plasma exchange, rituximab, cyclophosphamide, and N-acetylcysteine but developed renal, cardiac, gastrointestinal, and neurologic complications. The girl was then successfully treated with bortezomib, and she has been monitored in remission for 6 months. We consider that bortezomib is a beneficial treatment, especially in patients with refractory TTP.

Overlap Stevens Johnson Syndrome/Toxic Epidermal Necrolysis developed due to the use of toxic-dose vinblastine in case of Langerhans Cell Histiocytosis(Letterer-Siwe).

Except for side effects expected standart dose use of the chemotherapeutics agents, toxic effects (poisoning) may occur if high doses of are mistakenly used in the treatment of haemato-oncological diseases and these toxic doses are usually fatal. Here, we report a case of Stevens Johnson Syndrome (SJS) / Toxic Epidermal Necrolysis (TEN) following administration of toxic dose of vinblastine by mistake. A 20-month-old male patient with a diagnosis of Langerhans Cell Histiocytosis (Letterer-Siwe) at the pediatric oncology department was admitted to intensive care unit, after having received treatment protocol consisting of vinblastine, etoposide and prednisolone, with fever, altered consciousness and decompensated shock findings. Skin biopsy which performed from bullous lesions in the perianal, neck and axillary regions was resulted compatible with SJS / TEN in the patient with multiple organ failure, at 48 h of admission. It was later determined that the patient has been mistakenly given 10 times the normal dose of vinblastine he needed (60 mg/m2), which was 6 mg/m2. Plasma exchange was performed 3 times for vinblastine toxicity, intravenous immunoglobulin was administered for SJS / TEN therapy and phenobarbital was initiated to increase drug metabolism. The patient whose clinical picture fully improved, was transferred to the oncology department on the 30th day of intensive care hospitalization. Vinblastine toxicity is a life-threatening condition that can cause multiple organ failure, SJS / TEN. Plasma exchange is an effective treatment method for the removal of vinblastine from the body and in these cases of toxicity.

Acute necrotizing encephalopathy of childhood: a single-center experience

Background/aim: Acute necrotizing encephalopathy is a rare type of acute encephalopathy characterized by multi-ocal brain lesions and associated severe neurological findings and various organ dysfunctions may accompany it. Materials and Methods: Patients with acute necrotizing encephalopathy of childhood diagnosed by pediatric neurology and pediatric intensive care at Sami Ulus Maternity, Child Health and Diseases Training and Research Hospital between 2007 and 2020 were included in this study. Results: Nine patients (six females, three males) with a mean age of 4.05 ± 1.94 years (age range 1­6.5) were included in this study. The interval range between fever and encephalopathy in patients was 1­4 days. Influenza A (3H1N1, one untyped) was detected in four patients, influenza B in three patients, and no cause was found in two patients. Major clinical findings other than febrile encephalopathy in all patients were a hemodynamic shock in seven patients, seizures in six patients, vomiting in five patients, dystonia in three patients, and flaccid paralysis in the upper extremity in one patient. Despite all our treatment approaches, including plasmapheresis, moderate to severe neurological sequelae was observed in all of our patients, who survived even with significant radiological improvement. Three patients for whom we could not perform plasmapheresis died. Conclusion: Our study revealed that thalamic involvement increased as the interval shortened, and brainstem involvement increased in patients over four years of age. The presence of persistent vomiting accompanying encephalopathy during the parainfectious period and plasmapheresis treatment being a treatment option that could prevent mortality were cautionary for our study.

Continuous venovenous hemodiafiltration in the treatment of newborns with an inborn metabolic disease: a single center experience

Background/aim: Most inborn metabolic diseases are diagnosed during the neonatal period. The accumulation of toxic metabolites may cause acute metabolic crisis with long-term neurological dysfunction and death. Renal replacement therapy (RRT) modalities allow the efficient removal of toxic metabolites. In this study, we reviewed our experience with continuous venovenous hemodiafiltration (CVVHDF) as RRT for newborns with an inborn metabolic disease. Materials and methods: Patients diagnosed with an inborn metabolic disease and who received CVVHDF treatment at our neonatal intensive care unit between January 2014 and December 2017 were included in this study. Their demographic and clinical data were collected, and the efficacy and safety of CVVHDF was evaluated. Results: A total of nine continuous RRT (CRRT) sessions as CVVHDF were performed in eight newborns with a diagnosis of urea cycle defect (n = 5), maple syrup urine disease (n = 2), or methylmalonic acidemia (n = 1). The mean age at admission was 10 ± 8.6 days (range: 3­28 days). The mean plasma levels of ammonium were 1120 ± 512.6 mg/dL and 227.5 ± 141.6 mg/dL before and at the end of the treatment, respectively. Plasma levels of leucine were 2053.5 ± 1282 µmol/L and 473.5 ± 7.8 µmol/L before and at the end of the treatment, respectively. The CVVHDF duration was 32.3 ± 11.1 h (median: 37 h; range: 16­44 h), and the mean length of hospitalization was 14.6 ± 12.9 days. The mean duration of CVVHDF was 32.3 ± 11.1 h (range: 16­44 h). Circuit clotting was the most common observed complication (37.5%) and the survival rate was 50%. Among surviving patients, two developed severe and two developed mild mental and motor retardation. Conclusion: CVVHDF is a CRRT modality that can be used to treat newborns with an inborn metabolic disease. Early diagnosis, commencement of specific medical therapy, diet, and extracorporeal support, if needed, are likely to result in improved short and long- term outcomes.

Fatal Refractory Ventricular Fibrillation Due to Ingestion of Hydrofluoric Acid.

Hydrofluoric acid (HF) is a colorless and odorless solution of the hydrogen fluoride in water. It is used in some household products. The rapid onset of severe toxicity and death after the ingestion of HF is not reported often. Also, there is no reported fatal pediatric case after HF ingestion. In this case report, we present a 3.5-year-old girls who unintentionally drunk a rust remover that contained 8% HF. She died in a short period as a result of refractory ventricular fibrillation, which was developed due to fluoride intoxication.

Attention to diseases that re-emerge due to refusal of vitamin K: an infant case.

Intramuscular vitamin K injection is recommended for all newborns to prevent bleeding. However, the number of parents who reject vitamin K is at an increase. We present a 1-month girl who presented with haemorrhagic shock due to extraordinary intra-thoracic bleeding. The patient was treated with thoracentesis and blood transfusion. Parents were informed the about the benefits of Vitamin K and they were convinced to continue a routine immunization programme.

Analysis of Intestinal and Nasopharyngeal Microbiota of Children with Meningococcemia in Pediatric Intensive Care Unit: INMACS-PICU Study.

Microbiota composition might play a role in the pathophysiology and course of sepsis, and understanding its dynamics is of clinical interest. Invasive meningococcal disease (IMD) is an important cause of community-acquired serious infection, and there is no information regarding microbiota composition in children with meningococcemia. In this study, we aimed to evaluate the intestinal and nasopharyngeal microbiota composition of children with IMD. Materials and Methods: In this prospective, multi-center study, 10 children with meningococcemia and 10 age-matched healthy controls were included. Nasopharyngeal and fecal samples were obtained at admission to the intensive care unit and on the tenth day of their hospital stay. The V3 and V4 regions of the 16S rRNA gene were amplified following the 16S Metagenomic Sequencing Library Preparation. Results: Regarding the alpha diversity on the day of admission and on the tenth day at the PICU, the Shannon index was significantly lower in the IMD group compared to the control group (p = 0.002 at admission and p = 0.001, on the tenth day of PICU). A statistical difference in the stool samples was found between the IMD group at Day 0 vs. the controls in the results of the Bray-Curtis and Jaccard analyses (p = 0.005 and p = 0.001, respectively). There were differences in the intestinal microbiota composition between the children with IMD at admission and Day 10 and the healthy controls. Regarding the nasopharyngeal microbiota analysis, in the children with IMD at admission, at the genus level, Neisseria was significantly more abundant compared to the healthy children (p < 0.001). In the children with IMD at Day 10, genera Moraxella and Neisseria were decreased compared to the healthy children. In the children with IMD on Day 0, for paired samples, Moraxella, Neisseria, and Haemophilus were significantly more abundant compared to the children with IMD at Day 10. In the children with IMD at Day 10, the Moraxella and Neisseria genera were decreased, and 20 different genera were more abundant compared to Day 0. Conclusions: We first found alterations in the intestinal and nasopharyngeal microbiota composition in the children with IMD. The infection itself or the other care interventions also caused changes to the microbiota composition during the follow-up period. Understanding the interaction of microbiota with pathogens, e.g., N. meningitidis, could give us the opportunity to understand the disease's dynamics.

Serum concentrations of neuron-specific enolase in pediatric migraine.

Recent studies suggest that migraine might be a progressive disease that causes neuronal damage, rather than being a benign headache disorder. The objective of the present study was to investigate the concentrations of neuron-specific enolase (NSE) in pediatric migraineurs in order to identify possible neuronal damage. Forty-one children and adolescents with migraine (mean age: 14.58 +/- 2.35 years, range: 7-17 years, 12 with aura) and 30 control subjects were included. Serum NSE levels were measured during the attack and repeated at least 7 days thereafter in the patients, and measurements were obtained once in the control group. There were no significant differences in NSE concentrations with respect to values during the attack versus pain-free period or between the patient and control groups. NSE levels did not differ according to the clinical variables, including the presence of aura, severity and duration of headaches, nor with the length of migraine. In conclusion, our study showed that NSE levels did not change during migraine attack in pediatric patients. Further studies with different markers are warranted to assess possible neuronal injury in pediatric migraine.