Immunoadsorption therapy for a meningococcemia patient with myocarditis, adrenal hemorrhage, and purpura fulminans: a case report

Abstract Neisseria meningitidis, also known as meningococcus, is a relatively uncommon cause of invasive infection, but when it occurs, it is frequently severe and potentially life-threatening. A ten-year-old female patient developed a purpuric rash with fever. Upon arrival to the pediatric intensive care department, she was unconscious and in a poor general condition. We combined treatment with antibiotics, volume resuscitation, hydrocortisone, and CytoSorb® therapy resulted in a stabilization of hemodynamics, as well as control of hyperinflammation. We observed a significant decrease in vasopressor dosage in this patient.

BIG score is a strong predictor of mortality and morbidity for high-energy traumas in pediatric intensive care unit.

BACKGROUND: Severe traumatic injuries not only constitute an important population of pediatric intensive care unit (PICU) but they also play a major role in mortality and morbidity. Mortality risk assessment of traumatic injuries in the PICU is a delicate issue as it influences the treatment decisions. BIG score (Base Deficit +[2.5 × INR] + [15-GCS]) and the Pediatric Trauma Score (PTS) are utilized in pediatric trauma centers for the assessment of trauma severity. In this research, we aimed to elucidate the predictivity of trauma severity scores, the PRISM-3 (pediatric risk of mortality), and admission laboratory parameters in pediatric patients with high-energy traumas. METHODS: Children who had been exposed to high-energy polytraumas between 2018 and 2020 and treated in a tertiary care PICU were included in this retrospective analysis. Newly developed mental or motor disabilities, post-traumatic acquired epilepsy, requirement for tracheostomy, and/or extremity loss at PICU discharge were defined as morbidity. The PTS, the BIG score, PRISM-3 score, and admission laboratory parameters were utilized for mortality and morbidity prediction. RESULTS: A total of 155 patients were included in the study. The median age of the participants were 66 months (25-134). The origin of trauma was fall from height in 45.2% (n=70) of the subjects and traffic accident 54.8% (n=85) of the cases. New morbidities had occurred in 8.7% (n=13) and 3.2% (n=5) of the patients deceased in the ICU. The results of logistic regression analysis indicated that BIG score (p=0.01), PTS (p=0.003), PRISM-3 (p=0.02), admission D-dimer (p=0.01), and albumin levels (p=0.001) were significantly associated with mortality. The receiver operating characteristics curve analysis denoted that BIG score (cutoff >21.5, area under the curve [AUC]: 0.984 95% CI: 0.943-0.988), PRISM-3 score (cutoff >18, AUC: 0.997 95% CI: 0.970-1), the PTS (cutoff ≤3, AUC: 0.969 95% CI: 0.928-0.990), admission albumin level (cutoff ≤3 g/dL, AUC: 0.987 95% CI: 0.953-0.998), and D-dimer level (cutoff >13,100 mcg/L, AUC: 0.776 95% CI: 0.689-0.849) all had high predictive values for mortality. CONCLUSION: Regarding the results of this research, one can conclude that BIG score is a strong predictor of mortality and morbidity in high-energy pediatric traumas. Although PRISM-3 score has a similar predictive capability, the earlier and easier calculation as-sets of BIG score positions itself as a more useful and powerful predictor for mortality and morbidity in pediatric high-energy traumas.

Mortality Risk Factors Among Critically Ill Children With Acute COVID-19 in PICUs: A Multicenter Study From Turkish Pediatric Critical COVID-19 and MIS-C Study Group.

BACKGROUND: During the coronavirus disease 2019 (COVID-19) pandemic, the world has a large number of reported COVID-19 cases and deaths. Information on characteristics and mortality rate of pediatric intensive care unit (PICU) cases with COVID-19 remains limited. This study aims to identify the risk factors for mortality related to COVID-19 in children admitted to PICU. METHODS: A retrospective multicenter cohort study was conducted between March 2020 and April 2021 at 44 PICUs in Turkey. Children who were 1 month-18-year of age with confirmed COVID-19 admitted to PICU were included in the study. Children with multisystem inflammatory syndrome and asymptomatic for COVID-19 were excluded. RESULTS: Of 335 patients with COVID-19, the median age was 6.8 years (IQR: 1.2-14) and 180 (53.7 %) were male, 215 (64.2 %) had at least one comorbidity. Age and gender were not related to mortality. Among 335 patients, 166 (49.5%) received mechanical ventilation, 17 (5.1%) received renal replacement therapy and 44 (13.1 %) died. Children with medical complexity, congenital heart disease, immunosuppression and malignancy had significantly higher mortality. On multivariable logistic regression analysis, organ failure index [odds ratio (OR): 2.1, 95 confidence interval (CI): 1.55-2.85], and having congenital heart disease (OR: 2.65, 95 CI: 1.03-6.80), were associated with mortality. CONCLUSIONS: This study presents detailed data on clinical characteristics and outcomes of patients with COVID-19 admitted to PICU in the first pandemic year in Turkey. Our study shows that having congenital heart disease is associated with mortality. In addition, the high organ failure score in follow-up predict mortality.

A rare structural myopathy: Nemaline myopathy.

Nemaline myopathy, which is characterized by the accumulation of ''rod'' bodies in muscle fibers is a very rare inherited muscle disease. According to the underlying mutation, the disease has varying severity of clinical outcomes. Patients with severe forms of the disease die because of hypotonia, feeding difficulties, aspiration pneumonia, and respiratory failure in the neonatal or infancy period. Mild forms of the disease present with walking-swallowing difficulties and respiratory distress in late childhood or adulthood. A two-and-a-half-month-old boy was monitored in our Pediatric Intensive Care Unit with hypotonia, pneumonia, and respiratory distress. Nemaline myopathy was diagnosed as the result of a muscle biopsy. An advanced molecular examination revealed heterozygous mutations in the skeletal muscle α-actin (ACTA1) gene, which is the second most common cause of this disease. Nemaline myopathy should be kept in mind in patients of all age groups with respiratory failure and walking difficulty secondary to muscle weakness.

Nemalin miyopatisi oldukça nadir görülen kalitimsal bir kas hastaligi olup kas liflerinde ''rod''(nemalin) cisimcigi birikimi ile tanimlanmaktadir. Hastalik altta yatan mutasyona ve mutasyonun kalitim biçimine göre degisen agirlikta klinik gidise sahiptir. Agir sekillerinde olgular yutma ve solunum kaslarinin etkilenmesi sonucu beslenme yetersizligi, aspirasyon pnömonisi ve solunum yetmezligi nedeni ile yenidogan ya da süt çocuklugu döneminde kaybedilmektedir. Geç baslangiçli hafif olgular yasam kalitesini bozan yürüme-yutma zorlugu ve solunum sikintisi ile geç çocukluk ya da eriskin yasta bulgu verebilmektedir. Hipotoni, pnömoni ve solunum sikintisi ile Çocuk Yogun Bakim Birimi'nde izlenen iki buçuk aylik erkek bebege kas biyopsisi sonucu nemalin miyopatisi tanisi koyuldu. Ileri moleküler inceleme sonucu hastaligin ikinci en sik nedeni olan "Skeletal Muscle α-Actin" (ACTA1) geninde heterozigot mutasyon saptandi. Yenidogan döneminden eriskin döneme kadar kas güçsüzlügüne bagli solunum yetmezligi ve yutma-yürüme güçlügü varliginda yapisal miyopatiler içinde nemalin miyopatisi akilda bulundurulmali, süphenilen olgulara kas biyopsisi ya/ya da genetik inceleme yapilmalidir.

Hemolytic uremic syndrome with dual caution in an infant: cobalamin C defect and complement dysregulation successfully treated with eculizumab.

BACKGROUND: Hemolytic uremic syndrome (HUS) is a clinical syndrome characterized by hemolytic anemia, thrombocytopenia, and acute kidney injury. Atypical hemolytic uremic syndrome (aHUS) is a devastating disease with significant mortality and high risk of progression to end-stage kidney disease. It is mostly caused by dysregulation of the alternative complement pathway. Cobalamin C (Cbl C) defect is a genetic disorder of cobalamin metabolism and is a rare cause of HUS. CASE-DIAGNOSIS/TREATMENT: We present a 6-month-old male infant who was admitted to the pediatric intensive care unit (PICU) due to restlessness, severe hypertension, anemia, respiratory distress, and acute kidney injury. Metabolic screening revealed elevated plasma homocysteine levels, low methionine levels, and methylmalonic aciduria, and the patient was diagnosed as having HUS secondary to Cbl C defect. Additionally, complement factor H (CFH) and complement C3 levels were decreased. The infant was treated with betaine, hydroxycobalamin, and folic acid. After treatment, the homocysteine and methylmalonic acid levels were normalized but hemolysis and acute kidney failure persisted. He required continued renal replacement treatment (CRRT) and plasma exchange due to thrombotic microangiopathy (TMA). Therefore, we considered a second mechanism in the pathogenesis as complement dysregulation and gave eculizumab to the patient. After eculizumab treatment, the renal and hematologic indices improved and he was free of dialysis. CONCLUSIONS: To the best of our knowledge, our patient is the first to have Cbl C defect-HUS accompanied by complement dysregulation, who responded well to eculizumab therapy.

[A case of bronchiolitis obliterans secondary to human metapneumovirus bronchiolitis]. / Insan metapnömovirus bronsiyolitine ikincil gelisen bronsiyolitis obliterans olgusu.

Human metapneumovirus (hMPV), formerly classified in Paramyxoviridae family is now moved into Pneumoviridae, which was described as a novel family. It causes upper and lower respiratory tract infections (LRTIs) usually in children younger than five years old. The recent epidemiological studies indicated that hMPV is the second most frequently detected virus in LRTIs of young children, following the respiratory syncytial virus (RSV). Bronchiolitis obliterans (BO) is a chronic obstructive lung disease characterized by fibrosis of the distal respiratory airways. It is usually a result of an inflammatory process triggered by a LRTI related to adenovirus, RSV, Mycoplasma pneumoniae, measles virus, Legionella pneumophila, influenza virus or Bordetella pertussis as a causative agent. In this report, a case of hMPV bronchiolitis complicated with BO has been reported to point out the complications and severity of the clinical progress belongs to this virus. A three-month-old female patient has admitted to our pediatric intensive care unit with the diagnosis of acute bronchiolitis and respiratory failure. She was born at term, weighing 2950 gram and had been hospitalized in newborn intensive care unit for 11 days with the diagnosis of transient tachypnea of the newborn and neonatal sepsis. On auscultation, there were bilateral crepitant rales, wheezing and prolonged expirium. Her oxygen saturation was 97-98% while respiratory support was given with a non-rebreathing reservoir mask. Complete blood count, procalcitonin and C-reactive protein levels were in normal ranges. The chest radiography yielded right middle lobe atalectasia, left paracardiac infiltration and bilateral air trapping. A nasopharyngeal swab sample was analyzed by a commercial multiplex real-time reverse transcriptase-polymerase chain reaction (Thermo Fisher Scientific®, USA) developed for the detection of 15 respiratory viruses. Her sample yielded positive result for only hMPV. On the 4th day of hospitalization, the patient was intubated because of respiratory failure and carbon dioxide retention. She was extubated on the 19th day but could not tolerate. In the thorax computed tomography (CT), bilateral hyperinflation, patchy infiltration, mosaic perfusion and atelectasis especially bilateral posterior areas were detected. Bronchoscopy was normal except mild bronchomalacia in right middle lobe bronchus. The patient was diagnosed as BO secondary to hMPV bronchiolitis, according to the clinical, virological, bronchoscopic and thorax CT results. On the 76th day of admission, she was discharged with respiratory support with home ventilation via a tracheostomy cannula and medical treatments of oral metilprednisolone, nebulized salbutamol and budesonide. In conclusion, hMPV should not be undervalued especially in infants with severe LRTI that can be complicated with BO.