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At least 19 recordsLinked to original sources
PubMed2025

Treatment response variations to a single large bolus of enteral cholecalciferol in vitamin D deficient critically Ill children: Metabolomic insights for precision nutrition.

Vitamin D deficiency (VDD) is prevalent globally and in pediatric intensive care units, where it represents a modifiable risk factor that may impact patient recovery during hospitalization. Herein, we performed a retrospective analysis of serum samples from a phase-II randomized placebo-controlled trial involving a single large bolus of 10,000 IU/kg vitamin D3 ingested by critically ill children with VDD (25-OH-D < 50 nmol/L). Targeted and untargeted methods were used to comprehensively measure 6 vitamin D metabolites, 239 lipids, 68 polar metabolites, and 4 electrolytes using a multi-step data workflow for compound authentication. Complementary statistical methods classified circulating metabolites/lipids associated with vitamin D repletion following high-dose vitamin D3 intake (n&#x202f;=&#x202f;20) versus placebo (n&#x202f;=&#x202f;11) comprising an optional standard of care maintenance dose (< 1000 IU/day). There was a striking increase in median serum concentrations of 25-OH-D3 (4.7-fold), 3-epi-25-OH-D3 (24-fold) and their C3-epimer ratio (6.7-fold) in treated patients on day 3, whereas serum vitamin D3 peaked on day 1 (128-fold) unlike placebo. Treatment response differences were attributed to D3 bioavailability and C3-epimerase activity without evidence of hypercalcemia. For the first time, we report the detection of circulating 3-epi-D3 that was strongly correlated with vitamin D3 uptake (r&#x202f;=&#x202f;0.898). Metabolomic studies revealed that vitamin D sufficiency (serum 25-OH-D >75 nmol/L) coincided with lower circulating levels of 3-methylhistidine, cystine, S-methylcysteine, uric acid, and two lysophosphatidylcholines 7 days after treatment. Rapid correction of VDD was associated with indicators of lower oxidative stress, inflammation, and muscle protein turn-over that may contribute clinical benefits in high-risk critically ill children.

Humans↗
PubMed1977

Accuracy of blood volume estimations in critically ill children using 125I-labelled albumin and 51Cr-labelled red cells.

Blood volume was estimated using 51chromium labelled red cells and 125iodinated human serum albumin in 5 children with sepsis, in 6 burned children and 7 children with acute lymphoblastic leukaemia. Studies of the equilibration pattern demonstrated that the mixing time of labelled red cells was prolonged to 40 minutes or more in 5 children, indicating the existence of slowly circulating red cells. Mixing of labelled albumin was complete within 10 minutes in 15 patients and within 20 minutes in all the children studied. In a burned patient with severe sepsis, exchange transfusion improved the clinical state and normalized the equilibration pattern of labelled red cells. The mean body/venous haematocrit ratio was 0.893+/-0.018 (SD) in the children with sepsis, 0.859+/-0.052 in the burned patients, and 0.916+/-0.078 in the children with acute lymphoblastic leukaemia, increasing with spleen size in the latter group.

Adolescent↗
PubMed1979

Thoracoscopy. Early diagnosis of interstitial pneumonitis in the immunologically suppressed child.

Interstitial pneumonitis in immunosuppressed patients demands prompt diagnosis and treatment. In an effort to achieve a simple yet highly accurate method of diagnosis, we have evaluated the usefulness of thoracoscopic examination. Twenty-seven procedures have been performed in 24 patients between the ages of 17 months and 18 years. All patients were immunosuppressed, most for treatment of malignant processes. All procedures have been performed under anesthesia with intravenously administered ketamine, without endotracheal intubation. A definitive diagnosis has been made in every case, with pneumonia due to Pneumocystis carinii being identified in 18 instances. Complications have been minimal and include four minor pneumothoraces, two instances of bleeding, and two instances of prolonged air leak. Mortality attributable to the procedure has been nil, although five patients have died due to their underlying diseases within 30 days of the thoracoscopic procedure. Thoracoscopy has proven to be a rapid and safe technique for providing accurate histologic and bacteriologic diagnoses in these critically ill children.

Adolescent↗
PubMed1978

Management of severe cerebral edema in the metabolic encephalopathy of Reye-Johnson syndrome.

Fifteen critically ill children with the diagnosis of Reye-Johnson syndrome were treated with techniques developed to maintain adequate cerebral perfusion pressure and levels of circulating blood glucose. One child died, three sustained neurological deficit, and nine children (70%) recovered without significant neurological dysfunction. The technique developed during the period these children were treated, the indications for their use, and factors that can interfere with maintaining adequate cerebral perfusion in patients with increased intracranial pressure from metabolic encephalopathy are described. The results suggest that neurological damage in this syndrome results from neuronal injury secondary to inadequate cerebral perfusion and/or hypoglycemia, and that neurological dysfunction like hepatic dysfunction should produce minimal mortality and morbidity if cerebral perfusion and adequate levels of circulating blood glucose are sustained during the period of increased intracranial pressure and liver failure.

Adolescent↗
PubMed2026

Rapid genome sequencing identifies treatable conditions in non-intensive care unit hospitalized children.

PURPOSE: The utility of rapid genome sequencing (RGS) has been evaluated in pediatric intensive care unit (ICU) settings, but few studies have investigated its use in non-critically ill hospitalized children. Our study assesses the impact of RGS use in the non-ICU setting. METHODS: We analyzed RGS results obtained for hospitalized children from 2019 to 2023 and evaluated the impact on non-ICU patient care. Changes in management were determined via chart review of the first 30 days after testing. RESULTS: RGS was performed on 422 individuals: 339 ICU and 83 non-ICU. The diagnostic rate was 39% (32 of 83) in non-ICU and 35% (120 of 339) in ICU patients. Eighty-one percent of diagnostic RGS results in non-ICU patients had a management change within 30 days, and 56% (18 of 32) received a disease-targeted intervention, including medication or diet change, listing for transplant, or connection with a clinical trial. Of the children who received these intervention changes, the most common disease categories were metabolic (61%, 11 of 18) and epilepsy (22%, 4 of 18). CONCLUSION: RGS is effective at identifying treatable diagnoses in the non-ICU setting, with most patients experiencing a change in their care, and over half receiving disease-focused interventions. Our results support the utility of RGS in non-ICU hospitalized children and can impact providers' decision-making and payer coverage.

Genome sequencing↗
PubMed2024

Critically unwell infants and children with mitochondrial disorders diagnosed by ultrarapid genomic sequencing.

PURPOSE: To characterize the diagnostic and clinical outcomes of a cohort of critically ill infants and children with suspected mitochondrial disorders (MD) undergoing ultrarapid genomic testing as part of a national program. METHODS: Ultrarapid genomic sequencing was performed in 454 families (genome sequencing: n&#xa0;= 290, exome sequencing&#xa0;+/- mitochondrial DNA sequencing: n&#xa0;= 164). In 91 individuals, MD was considered, prompting analysis using an MD virtual gene panel. These individuals were reviewed retrospectively and scored according to modified Nijmegen Mitochondrial Disease Criteria. RESULTS: A diagnosis was achieved in 47% (43/91) of individuals, 40% (17/43) of whom had an MD. Seven additional individuals in whom an MD was not suspected were diagnosed with an MD after broader analysis. Gene-agnostic analysis led to the discovery of 2 novel disease genes, with pathogenicity validated through targeted functional studies (CRLS1 and MRPL39). Functional studies enabled diagnosis in another 4 individuals. Of the 24 individuals ultimately diagnosed with an MD, 79% had a change in management, which included 53% whose care was redirected to palliation. CONCLUSION: Ultrarapid genetic diagnosis of MD in acutely unwell infants and children is critical for guiding decisions about the need for additional investigations and clinical management.

Humans↗
PubMed1978

Cardiac output measured by thermodilution in infants and children.

To determine the accuracy and reproducibility of cardiac output determination by thermodilution (COT) in children, simultaneous outputs were measured by the Fick technique (COF) (using measured oxygen consumption) and thermodilution in 26 children, ranging in age from 8 to 86 months, who were undergoing cardiac catheterization. There was excellent correlation between mean output by thermodilution and by the Fick technique: COT = 1.10 COF -- 0.2 l/minute, R = 0.91. In three-quarters of the patients with COT differed by 15% or less and in none differed by more than 25%. Serial values of thermodilution outputs were reproducible in each patient with a SD of 5.5%. Our observations indicate that COT is accurate, reproducible, and valuable in the care of critically ill infants and children.

Cardiac Catheterization↗
PubMed1978

Importance of mixed venous oxygen saturation in the care of critically ill patients.

The relation between mixed venous oxygen saturation and cardiac index was determined in 11 children who underwent surgical treatment for congenital heart disease. The correlation between these two variables was found to be reliable (r = 0.78, P = 0.001). The simple determination of mixed venous oxygen saturation performance, particularly when sophisticated equipment for measuring cardiac output is not available.

Cardiac Output↗
PubMedDate not supplied

Counseling the parents of a critically ill newborn.

Nurses caring for critically ill infants should be reminded of their responsibilities to the parents of these children. The parents' needs often go either unrecognized or unmet. Nurses in a neonatal intensive care unit should have a working knowledge of their additional responsibility of serving in the role of counselor. Knowing sufficiently what emotional factors should be considered, one can effectively implement this counseling.

Counseling↗
PubMed1979

Clinical course of whooping cough in children younger than six months.

The patient records of 59 children aged 2--26 weeks with culture-verified pertussis were analysed. Twenty-four of them were hospitalized, in most cases for social reasons. Only one child with hypothyroidism and a complicating pneumonia was critically ill. Seventeen of the 35 non-hospitalized patients had a mild disease without developing typical whooping attacks. Thirteen children were treated with erythromycin in the catarrhal stage. There was a tendency towards milder disease in this group but the differences compared to untreated children were not statistically significant.

Age Factors↗
PubMed2025

Long-term follow-up of children who received rapid genomic sequencing.

PURPOSE: To explore long-term trajectories of children who received rapid genome sequencing (RGS) in intensive care settings. METHODS: We examined the electronic health records of 67 critically ill pediatric patients who received RGS 6 to 8 years ago with a collective initial diagnostic yield of 46%. RESULTS: The median length of follow-up was 6.2 years (interquartile range 4.0-7.2 years). RGS-diagnosed patients had a longer average follow-up time compared with undiagnosed patients (5.9 years vs 4.8 years, P = .026) and more subspecialty appointments per follow-up year (9.4 vs 6.9, P = .036). Mortality during the follow-up period was 9%. Patients averaged 2.1 hospital readmissions per follow-up year and 28.1 hospitalized days per follow-up year. Forty-four patients (66%) had a documented new phenotype in the electronic health records during their follow-up period. Seven patients received clinician-driven reanalysis during the follow-up period, yielding 1 new diagnosis. Systematic reanalysis of RGS performed as part of this study identified 4 new candidate diagnoses. CONCLUSION: Pediatric patients who receive RGS during intensive care unit hospitalizations continue to be high health care utilizers in subsequent years, regardless of whether RGS identified a diagnosis. Additionally, two-thirds of this cohort had a documented phenotypic change over the follow-up period, indicating dynamic clinical evolution in the years after RGS.

Humans↗
PubMed1978

Civilian ground and air transport of adults with acute respiratory failure.

The intermountain Respiratory Intensive Care Unit (IRICU), established in 1973 at the LDS Hospital in Salt Lake City, provides specialized care for adults and older children with severe respiratory failure in the intermountain West. Because of the large area serviced by the IRICU, a transportation system for the critically ill was developed. Our report describes the transport system, including team organization, techniques, and experiences. Of 44 attempted transports during the last three years, 43 (98%) were safely accomplished without obvious ill effect to the patient. Patients with severe respiratory failure, if their conditions are adequately stabilized, can be safely transported hundreds of kilometers by ground and air by a well-equipped and well-trained team of physicians and nurses.

Acute Disease↗
PubMed1977

Hemolytic uremic syndrome. Results of treatment with hemodialysis.

The characteristics of the hemolytic-uremic syndrome in 7 children living in a well defined area in the south of Sweden are described. All the patients had a severe form of the disease and were critically ill. The clinical activity could best be followed by measuring blood platelets and urinary FDP. Early institution of hemodialysis treatment, given almost daily until normalisation of platelet count and urinary output, is the most important live-saving measure. Full dosage heparin seems not to be necessary. Six patients survived and were followed-up for 1-7 years. When last seen they all had normal renal function and blood pressure.

Blood Cell Count↗
PubMed1979

Sequelae of prolonged ventilatory support for pediatric surgical patients.

The evolution of intensive prolonged respiratory support has been a major development in improving survival in the critically-ill child. That intensive respiratory support can be maintained for prolonged periods of time even in the home with survival of good babies is the subject of this report. In a 3-yr period, 2112 surgical patients were admitted to intensive care facilities with an over-all survival of 95%. Ventilatory support was required in 368 (17.4%) of these children, and survival in this group was 75.3%. Prolonged mechanical ventilation was necessary in 13 of the 368 children (3.5%) for a mean support time of 359 days (range 101 to 1095). Of these 13 children, 4 died while hospitalized (30.8%), and 3 died subsequently after being discharged (23.1%) for a total mortality of 53.8%. However, 6 children (46.2%) survive, 3 free of ventilatory support and 3 being weaned from their machine at home. The greatest cost in this expensive program was delivered to the survivors and psycho-social and developmental data confirm that these children are good babies with favorable long-term prognoses.

Child↗
PubMedDate not supplied

Family members' perceptions of communications in late stage cancer.

A study of patterns of communications in families with a terminal cancer patient being treated at three urban institutions revealed that may first-order relatives (spouses, children, siblings) of patients did not have a communication link to the physician, especially if direct communication was not established at the time of diagnosis. Family members were frequently critical of the way information was communicated, yet relied upon the physician's interpretation of the patient's status to form their own opinion of the patient's future. Intra-familial communications regarding illness and dying were frequently discordance and guarded, leading to preceptions that the patient was withdrawing, and fostering a reliance upon the hospital for terminal care. More than half of the family members were uncomfortable visiting the patient in the hospital, experiencing feelings of helplessness, or sensing helplessness in the patients. Bearing the patient's pain was seen as particularly difficult.

Adaptation, Psychological↗
PubMed1979

Factors contributing to the mortality associated with open-heart surgery in infants.

About 40% of children born with congenital heart disease die within the first year of life unless treated surgically. The results of surgery have improved with increasing experience. Currently, the mortality rate of open-heart procedures performed during the first year of life have decreased to about 25%. The present study analyses the causes of death in 53 infants who underwent open heart surgery at the Hospital for Sick Children, Great Ormond Street, London between 1973--1977. Fifty-three deaths represented 25% of the total 212 operated infants. Causes of death and possible contributing factors are discussed under the following headings: Inoperable lesions (18), poor preoperative condition (8), mistake in pre-, intra- and postoperative management (15), infection (2) and unclear (10). It is suggested that earlier diagnosis and operation may improve the results. The purpose of this study was to identify problems in the management of critically ill infants with congenital heart disease and thus improve their prognosis in future.

Age Factors↗
PubMed1979

A year's experience of the rotavirus syndrome and its association with respiratory illness.

In a hospital study rotavirus was identified in 51% of 152 children with diarrhoea. These patients showed a clinical pattern that was distinct from patients in whom the diarrhoea was associated with bacteria, other viruses, or no pathogens. A respiratory illness was described in 66% of rotavirus patients and usually preceded the gastrointestinal symptoms. Vomiting lasted between one and 3 days and was curtailed by substituting the normal diet with clear fluids. Watery diarrhoes continued for 4 or 5 days, even when rehydration was by the intravenous rather than the oral route. Prolonged diarrhoea was rare. Most children infected with rotavirus were under 2 years of age, but dehydration was most severe in infants aged between 12 and 18 months. A clinician can thus recognise the rotavirus syndrome and expect spontaneous recovery if adequate rehydration is maintained for a critical few days.

Adolescent↗