Mortality risk factors among critically ill children with MIS-C in PICUs: a multicenter study.

BACKGROUND: This study evaluated of clinical characteristics, outcomes, and mortality risk factors of a severe multisystem inflammatory syndrome in children admitted to a the pediatric intensive care unit. METHODS: A retrospective multicenter cohort study was conducted between March 2020 and April 2021 at 41 PICUs in Turkey. The study population comprised 322 children diagnosed with multisystem inflammatory syndrome. RESULTS: The organ systems most commonly involved were the cardiovascular and hematological systems. Intravenous immunoglobulin was used in 294 (91.3%) patients and corticosteroids in 266 (82.6%). Seventy-five (23.3%) children received therapeutic plasma exchange treatment. Patients with a longer duration of the PICU stay had more frequent respiratory, hematological, or renal involvement, and also had higher D-dimer, CK-MB, and procalcitonin levels. A total of 16 patients died, with mortality higher in patients with renal, respiratory, or neurological involvement, with severe cardiac impairment or shock. The non-surviving group also had higher leukocyte counts, lactate and ferritin levels, and a need for mechanical ventilation. CONCLUSIONS: In cases of MIS-C, high levels of D-dimer and CK-MB are associated with a longer duration of PICU stay. Non-survival correlates with elevated leukocyte counts and lactate and ferritin levels. We were unable to show any positive effect of therapeutic plasma exchange therapy on mortality. IMPACT: MIS-C is a life-threatening condition. Patients need to be followed up in the intensive care unit. Early detection of factors associated with mortality can improve outcomes. Determining the factors associated with mortality and length of stay will help clinicians in patient management. High D-dimer and CK-MB levels were associated with longer PICU stay, and higher leukocyte counts, ferritin and lactate levels, and mechanical ventilation were associated with mortality in MIS-C patients. We were unable to show any positive effect of therapeutic plasma exchange therapy on mortality.

Severe Rhabdomyolysis and Acute Renal Failure Treated by Continuous Venovenous Hemodiafiltration in a Child with Diabetic Ketoacidosis.

Diabetic ketoacidosis (DKA) is the most serious hyperglycemic emergency in patients with type I diabetes mellitus and is associated with significant morbidity and mortality. DKA may be a life-threatening condition due to severe clinical and biological impairments and treatment-associated complications [cerebral edema, acute respiratory failure, acute renal failure (ARF), hypokalemia, hypophosphatemia]. The development of ARF with rhabdomyolysis is a rare but potentially lethal disorder in children with DKA with an estimated mortality of about 50%. Continuous renal replacement therapy is commonly used in intensive care units to provide renal replacement and fluid management. We successfully treated a 13-year-old boy with continuous venovenous hemodiafiltration, who had been diagnosed with severe DKA, complicated with hypophosphatemia-induced acute respiratory failure, rhabdomyolysis and ARF, persistent acidosis, and coma. How to cite this article: Yaman A. Severe Rhabdomyolysis and Acute Renal Failure Treated by Continuous Venovenous Hemodiafiltration in a Child with Diabetic Ketoacidosis. Indian J Crit Care Med 2022;26(1):136-138.

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.

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.

Predictors of Early Extubation After Pediatric Cardiac Surgery: A Single-Center Prospective Observational Study.

This prospective, observational, single-center study aimed to determine the perioperative predictors of early extubation (<24 h after cardiac surgery) in a cohort of children undergoing cardiac surgery. Children aged between 1 month and 18 years who were consecutively admitted to pediatric intensive care unit after cardiac surgery for congenital heart disease between January 2012 and June 2014. Ninety-nine patients were qualified for inclusion during the study period. The median duration of mechanical ventilation was 20 h (range 1-480), and 64 patients were extubated within 24 h. Four of them failed the initial attempt at extubation, and the success rate of early extubation was 60.6 %. Older patient age (p = .009), greater body weight (p = .009), absence of preoperative pulmonary hypertension (p = .044), lower RACHS-1 category (OR, 3.8; 95 % CI 1.35-10.7; p < .05), shorter cardiopulmonary bypass (p = .008) and cross-clamp (p = .022) times, lower PRISM III-24 (p < .05) and PELOD (p < .05) scores, lower inotropic score (p < .05) and vasoactive-inotropic score (p < .05), and lower number of organ failures (OR, 2.26; 95 % CI 1.30-3.92; p < .05) were associated with early extubation. Our study establishes that early extubation can be accomplished within the first 24 h after surgery in low- to medium-risk pediatric cardiac surgery patients, especially in older ones undergoing low-complexity procedures. A large prospective multiple institution trial is necessary to identify the predictors and benefits of early extubation and to facilitate defined guidelines for early extubation.

Cardiopulmonary Resuscitation in Children With In-Hospital and Out-of-Hospital Cardiopulmonary Arrest: Multicenter Study From Turkey.

OBJECTIVES: The objectives of this study were to determine the causes, location of cardiopulmonary arrest (CPA) in children, and demographics of cardiopulmonary resuscitation (CPR) in Turkish pediatric emergency departments and pediatric intensive care units (PICUs) and to determine survival rates and morbidities for both in-hospital and out-of-hospital CPA. METHODS: This multicenter descriptive study was conducted prospectively between January 15 and July 15, 2011, at 18 centers (15 PICUs, 3 pediatric emergency departments) in Turkey. RESULTS: During the study period, 239 children had received CPR. Patients' average age was 42.4 (SD, 58.1) months. The most common cause of CPA was respiratory failure (119 patients [49.8%]). The location of CPA was the PICU in 168 (68.6%), hospital wards in 43 (18%), out-of-hospital in 24 (10%), and pediatric emergency department in 8 patients (3.3%). The CPR duration was 30.7 (SD, 23.6) minutes (range, 1-175 minutes) and return of spontaneous circulation was achieved in 107 patients (44.8%) after the first CPR. Finally, 58 patients (24.2%) were discharged from hospital; survival rates were 26% and 8% for in-hospital and out-of-hospital CPA, respectively (P = 0.001). Surviving patients' average length of hospital stay was 27.4 (SD, 39.2) days. In surviving patients, 19 (32.1%) had neurologic disability. CONCLUSION: Pediatric CPA in both the in-hospital and out-of-hospital setting has a poor outcome.

Patients with primary immunodeficiencies in pediatric intensive care unit: outcomes and mortality-related risk factors.

PURPOSES: The aims of this study were to review the frequency, characteristics, and the clinical course of primary immunodeficiency (PID) patients admitted to pediatric intensive care unit (PICU) and attempt to identify factors related with mortality that might predict a poor outcome. METHODS: We performed a retrospective review of children with PID aged 1 month to 18 years and admitted to PICU from January 2002 to January 2012 in our tertiary teaching children's hospital. RESULTS: There were a total of 51 patients accounting for 71 admissions to the PICU. The most common diagnosis was severe combined immunodeficiency. Respiratory problems were the leading cause for admission. A total of 20 patients received hematopoietic stem cell transplantation. Immune reconstitution was achieved in 9 (45 %) patients and eight of them did survive. In all 56 % of all admission episodes resulted in survival. Risk factors for mortality included requirement of mechanical ventilation (P < .001), number of organ system failure (P = .013), need for renal replacement therapy (P < .001), use of inotropes (P < .001), higher Pediatric Logistic Organ Dysfunction (PELOD) score (P = .005), and length of PICU stay (P < .001). CONCLUSIONS: This is the first study regarding the outcome and mortality-related risk factors for PID patients requiring PICU admission. We suggest that PICU management is as important as early diagnosis and treatment for these patients. Prediction of those at risk for poorer outcome might be beneficial for accurate intensive care management and survival.

Cyclosporine-associated thrombotic microangiopathy and thrombocytopenia-associated multiple organ failure: a case successfully treated with therapeutic plasma exchange.

INTRODUCTION: Thrombotic microangiopathy (TMA) is characterized by microvascular thrombosis, thrombocytopenia, and microangiopathic hemolytic anemia. Previous studies have shown that cyclosporine (CsA) is associated with TMA but the number of reported cases is very limited. We describe a 13-year-old girl with CsA-associated TMA and thrombocytopenia-associated multiple organ failure (TAMOF). CASE REPORT: The patient was diagnosed with polyglandular deficiency syndrome and had a history of celiac disease, autoimmune thyroiditis, and diabetes mellitus type I. CsA was started 7 months before her admission to our pediatric intensive care unit for persistent diarrhea associated with celiac disease. At the time of her admission to our pediatric intensive care unit, she was thrombocytopenic and anemic with multiple organ failure. Laboratory and clinical findings were consistent with TMA and TAMOF. CsA was discontinued and therapeutic plasma exchange was performed daily for 5 days. The patient improved clinically, laboratory findings normalized, and TMA and multiple organ failure dissolved. CONCLUSION: This case report indicates that therapeutic plasma exchange may be effective in the treatment of CsA-associated TMA and TAMOF, especially in the presence of systemic findings.

Advanced sclerosis of the chest wall skin secondary to chronic graft-versus-host disease: a case with severe restrictive lung defect.

Pulmonary chronic graft-versus-host disease (cGvHD) is one of the most common causes of morbidity and mortality after allogeneic hematopoietic stem cell transplantation (aHSCT). Herein, we describe a patient with severe restrictive lung defect secondary to cGvHD. A 21-year-old male patient was admitted to our pediatric intensive care unit (PICU) with pneumonia and respiratory distress. He had a history of aHSCT for chronic myelogeneous leukemia at the age of 17 years. Six months after undergoing aHSCT, he had developed cGvHD involving skin, mouth, eye, lung, liver, and gastrointestinal tract. At the time of PICU admission he had respiratory distress and required ventilation support. Thorax high-resolution computed tomography was consistent with bronchiolitis obliterans. Although bronchiolitis obliterans is an obstructive lung defect, a restrictive pattern became prominent in the clinical course because of the sclerotic chest wall skin. The activity of cGvHD kept increasing despite the therapy and we lost the patient because of severe respiratory distress and massive hemoptysis secondary to bronchiectasis. In conclusion, pulmonary cGvHD can present with restrictive changes related with the advanced sclerosis of the chest wall skin. Performing a fasciotomy or a scar revision for the rigid chest wall in selected patients may improve the patients ventilation.

Valproic acid-induced acute pancreatitis and multiorgan failure in a child.

Valproic acid (VPA) is still an important antiepileptic drug, with the broadest spectrum used in all types of seizures and syndromes. It has serious adverse effects such as hepatotoxicity, hyperammonemic encephalopathy, coagulation disorders, and pancreatitis. The incidence of VPA-associated pancreatitis has been estimated to be 1:40,000. We present a 6-year-old boy who developed acute pancreatitis (AP) and multiple-organ failure after 3 months of VPA therapy. The patient's laboratory values showed that his kidney and hepatic function had impaired and thrombocytopenia, and coagulopathy had developed. The patient's abdominal tomography showed a suspected appearance, which was consistent with pancreatitis. Because amylase and lipase levels were found to be high, AP was considered. The patient improved after cessation of VPA treatment. Ten days later, the patient recovered both clinically and laboratorial. Consequently, the patient was discharged with cure. In conclusion, AP is a rare, severe adverse reaction to VPA treatment. If a child, who is receiving VPA, develops abdominal pain and vomits, VPA-associated pancreatitis must be considered.

Immediate Extubation in the Operating Room Following Pediatric Liver Transplantation: A Retrospective Cohort Study.

BACKGROUND: Although immediate extubation in the operating room following pediatric liver transplantation can be safe and beneficial for select patients, many surgeons and anesthesiologists are still cautious. The study aimed to evaluate the safety and efficiency of immediate extubation in the operating room following pediatric liver transplantation. METHODS: Sixty-four pediatric liver transplant recipients were included in this retrospective study. Patients were divided into 2 groups: immediate extubation (IE) (those who were extubated in the operating room) and delayed extubation (DE) (those who were extubated in the intensive care unit). Preoperative, intraoperative, and postoperative variables were recorded. RESULTS: Although a total of 19 (29.7%) patients were extubated in the pediatric intensive care unit (group DE), 45 (70.3%) were extubated in the operating room at the end of surgery (group IE). The use of fresh frozen plasma and platelets was statistically higher in group DE (P = .017 and P = .002, respectively). Duration of anesthesia and length of stay in the pediatric intensive care unit was statistically longer in group DE (P = .020 and P = .0001, respectively). Three (15.8%) patients required reintubation in group DE and 2 (4.4%) in group IE. Hospital stay was statistically longer in group DE (P = .012). CONCLUSIONS: The current study demonstrated that immediate extubation in the operating room after surgery for pediatric patients who have undergone liver transplantation was safe. The duration of anesthesia and the intraoperative use of blood products such as platelet and fresh frozen plasma can effectively decide immediate extubation.

Prolonged respiratory failure responds to conventional therapy in isolated homocysteine remethylation defects.

Isolated remethylation defects are rare inherited diseases caused by a defective remethylation of homocysteine to methionine, preventing various essential methylation reactions to occur. Patients present with a systemic phenotype, which can especially affect the central and peripheral nervous systems leading to epileptic encephalopathy, developmental delay and peripheral neuropathy. Respiratory failure has been described in some cases, caused by both central and peripheral neurological involvement. In published cases, the genetic diagnosis and initiation of appropriate therapy were rapidly performed following respiratory failure and led to a rapid recovery of respiratory insufficiency within days. Here, we present two infantile-onset cases of isolated remethylation defects, cobalamine (Cbl)G and methylenetetrahydrofolate reductase (MTHFR) deficiencies, which were diagnosed after several months of respiratory failure. Disease modifying therapy based on hydroxocobalamin and betaine was initiated and shows a progressive improvement and enabled weaning off respiratory support after 21 and 17 months in CblG and MTHFR patients respectively. We show that prolonged respiratory failure responds to conventional therapy in isolated remethylation defects, but can require a sustained period of time before observing a full response to therapy.

Assessment of the Clinical Course of Human Rhinovirus/Enterovirus Infections in Pediatric Intensive Care.

BACKGROUND: This study aims to evaluate the clinical course of human rhinovirus/enterovirus (HRV/EV) infections in the pediatric intensive care unit. METHODS: The study was conducted as a multicenter, prospective observational study from September 2022 to December 2022. Cases with positive polymerase chain reaction testing for HRV/EV of nasopharyngeal swab samples within the first 24 hours of pediatric intensive care unit admission were recorded. There were 2 groups: 1-24 months and >24 months. RESULTS: A total of 75 cases (39 male) were included in the study. The median age for all cases was 21 months. The highest polymerase chain reaction positivity rates were observed in October (37.33%). Among the cases, 32 (42.67%) presented with bronchopneumonia/pneumonia, 24 (32%) presented with acute bronchiolitis/bronchitis and 7 (9.33%) presented with sepsis/septic shock. The frequency of pediatric acute respiratory distress syndrome was found to be 6.67%. In the age group of 1-24 months, mean lymphocyte and liver enzyme levels were higher, while in the age group of >24 months, mean hemoglobin and mean kidney function test levels were higher ( P ≤ 0.05). Continuous oxygen therapy was provided to 65.3% of the cases, noninvasive ventilation to 33.3%, high-flow nasal cannula-oxygen therapy to 32% and invasive mechanical ventilation to 16%. CONCLUSIONS: HRV/EV infections primarily affect the respiratory system and generally exhibit a clinical course with low mortality rates (1, 1.3%). In cases with underlying chronic diseases, more severe clinical conditions such as pediatric acute respiratory distress syndrome and septic shock may occur.

COVID-19 Transmission and Clinical Features in Pediatric Intensive Care Health Care Workers.

OBJECTIVE: This study aims to investigate modes of transmission and clinical features of coronavirus disease 2019 in healthcare workers in pediatric intensive care units. MATERIALS AND METHODS: This multicenter descriptive study was conducted between March and November 2020. Patient demographics, clinical characteristics, origin of coronavirus disease 2019, treatment modalities, and loss of workdays were recorded. RESULTS: Seven hundred and sixty-eight healthcare workers from 16 pediatric intensive care units were enrolled and 114 (14.8%) healthcare workers with a mean age of 29.7 ± 6.7 years became coronavirus disease 2019 patients. Seventy-six (66.7%) patients were female. Approximately half (54.3%) of the patients were physicians, 34.2% were nurses, and 11.4% were ancillary staff. Transmission was deemed to occur through patient contact in 54.3% of the patients. Comorbid illness was present 10.5% of the patients. Transmission occurred during endotracheal intubation in 21%, cardiopulmonary resuscitation in 9.6%, and non-invasive ventilation in 12.2% of patients, while transmission was a result of multiple possible procedures in 43.8%. Intensive care admission was needed for 13.1% of the patients. Five patients needed oxygen by cannula, 7 needed oxygen with a non-rebreathing mask, 5 needed high-flow nasal cannula support, 5 needed non-invasive ventilation, and 3 needed invasive mechanical ventilation. Fortunately, no infected healthcare workers died. CONCLUSION: Coronavirus disease 2019 in healthcare workers is a significant problem in pediatric intensive care units. Transmission seems to occur particularly frequently during patient care procedures such as intubation, ventilation and aerosol therapy, which highlights the importance of proper use of full sets of personal protective equipment during all procedures during care of coronavirus disease 2019 patients.

Evaluation of Forensic Cases in the Pediatric Intensive Care Unit.

OBJECTIVES: Traffic accidents, falls, assaults, occupational accidents, intoxications, burns, electric shock, lightning strike, all cases of asphyxia, penetrating and firearm injuries, suspected or definite cases of sexual abuse, and suicide attempts should be evaluated in the forensic category. In this study, we aimed to present our intensive care experiences in forensic cases. METHODS: We retrospectively evaluated forensic cases admitted to our Pediatric Intensive Care Unit between 1 February 2017 and 1 September 2018. RESULTS: This study included 153 children, 65 (42.5%) boys and 88 (57.5%) girls. The forensic causes of hospitalizations in the intensive care unit included drug intoxication with a rate of 54.9%, followed by suicide attempts with 24.2%, falling from a high place with 5.2%, child abuse with 5.2%, pedestrian (out-of-vehicle) traffic accidents with 2.6%, drowning in water with 2.6%, road (in-vehicle) traffic accidents with 2%, electric shocks with 2%, and CO (carbon monoxide) poisoning with 1.3%. The drug intoxication was caused by drugs prescribed to the mother and the child with a rate of 40.6% and 27.1%, respectively. Analgesic anti-inflammatory drugs (33.1%) and antidepressant drugs (22.3%) were identified as major causes of intoxication. In addition, paracetamol was the most common cause of intoxication, with a rate of 21.9% among all intoxication cases and 72.5% in the analgesic group. Amitriptyline was the most common agent in the antidepressant group (59.2%). The admission rate to the intensive care unit between 08:00 and 14:00 was 35.1% for suicide attempts and 16.4% for non-suicide attempts, with a statistically significant difference (p=0.025; p<0.05, respectively). CONCLUSION: Drug intoxications had the highest rate of forensic cases followed in our pediatric intensive care unit. The majority of these intoxications (69.4%) arose from accidental drug ingestion. Therefore, we believe that there may be a significant decrease in the number of hospitalizations of forensic cases associated with drug intoxications in pediatric intensive care units by preventing children's access to drugs.

Perioperative factors associated with hyperglycemia after pediatric cardiac surgery and impact of hyperglycemia on morbidity and mortality.

Ödek Ç, Kendirli T, Yildirim-Yildiz N, Yaman A, Uçar T, Eyileten Z, Ates C, Uysalel A, Tutar E, Atalay S. Perioperative factors associated with hyperglycemia after pediatric cardiac surgery and impact of hyperglycemia on morbidity and mortality Turk J Pediatr 2018; 60 497-505. This retrospective, observational, single-center study aimed to determine the perioperative factors associated with postoperative hyperglycemia (blood glucose level ≥126 mg/dl) and the impact of hyperglycemia on morbidity and mortality in a cohort of children undergoing cardiac surgery. Non-diabetic children aged between 1 month to 18 years who were consecutively admitted to pediatric intensive care unit (PICU) after cardiac surgery for congenital heart disease between January 2008 and December 2013 were included. One hundred and twenty-six patients were qualified for inclusion during the study period. Seventy-four (57.8%) of the patients had at least one glucose measurement ≥ 126 mg/dl. Higher PRISM III-24 (OR 1.1, 95% CI 1.02-1.18, p= 0.004) and PELOD (p=0.006) scores, higher Wernovsky inotropic score (p=0.027) and vasoactive-inotropic score (p=0.029) were associated with hyperglycemia. Postoperative hyperglycemia was not associated with duration of mechanical ventilation), length of PICU stay, healthcare associated infections, or mortality. Our study establishes that hyperglycemia is common after pediatric cardiac surgery but not associated with short-term morbidity and mortality. Insulin therapy can be accomplished without hypoglycemia when a permissive glycemic target is used. A large prospective multiple institution trial is necessary to facilitate defined guidelines for postoperative hyperglycemia after pediatric cardiac surgery.

An infant with glutaric aciduria type IIc diagnosed with a novel mutation.

Isikay S, Yaman A, Ceylaner S. An infant with glutaric aciduria type IIc diagnosed with a novel mutation. Turk J Pediatr 2017; 59: 315-317. Glutaric aciduria type II is a rare inborn error of metabolism. The clinical picture is highly variable with symptoms ranging from acute metabolic decompensations to chronic, mainly muscular problems or even asymptomatic cases. Herein we described a 7-month-old female patient presented with respiratory failure and diagnosed with glutaric aciduria type II via whole exome sequencing that exhibited one known and a novel mutation. Her blood and urine analyses were all normal. After the diagnosis, dramatic and sustained improvement on a low-fat, low-protein, and high-carbohydrate diet supplemented with oral riboflavin and carnitine was determined. In especially hypotonic patients with unknown etiologies, though the blood and urine analyses are normal, glutaric aciduria type II should also be kept in mind and genetic tests may be required for the diagnosis.

Severe hyperphosphatemia after oral laxative administration in a 7-year-old patient.

Sodium phosphate based laxatives are commonly used for constipation and pre-procedural bowel cleansing. Phosphate intoxication related with these preparations is well recognized. Herein, we present a case of severe hyperphosphatemia and seizure in a 7-year-old male patient after administration of an oral sodium phosphate based laxative. At the time of admission, serum phosphorus level was 25.6 mg/dl. Aggressive fluid therapy was started. Although serum phosphorus level decreased to 20.9 mg/dl eight hours after admission, hemodialysis was performed because of the preexisting renal disease and declined glomerular filtration rate. Serum phosphorus level and blood gas analysis returned to normal after hemodialysis and the patient was discharged on hospital day two. In conclusion, sodium phosphate based laxatives should be used carefully in patients with preexisting renal diseases. Intravenous hydration and correction of hypocalcemia are important components of treatment. Hemodialysis is indicated in patients with renal failure.

Paracetamol infusion-related severe hypotension and cardiac arrest in a child.

Paracetamol (also known as Acetaminophen) is an antipyretic, non-opioid analgesic, and non-steroidal anti-inflammatory drug (NSAID), and is one of the most commonly used medications worldwide. In recent years, IV paracetamol has been frequently used in hospitalized patients to reduce fever and pain. Significant adverse reactions associated with intravenous paracetamol are extremely rare. Typically reported adverse events include hypotension, malaise, hypersensitivity reaction, liver enzyme elevation, and thrombocytopenia. We present herein a case of IV paracetamol infusion-related severe hypotension and cardiac arrest.