Acute Complications of Sickle Cell Disease in Children Under 5 Years at a Level II Hospital.

The aim of this study was to characterize the acute complications that required hospitalization in children under 5 years with Sickle Cell Disease. The population included 70 patients, 64% of African ascendency and 95.7% homozygotic for the HbS. We analyzed data from 337 hospitalizations. The first hospitalization occurred before 12 months in 38.6% and the average hospital admission per child was 3.92. The mean duration per hospitalization was 5.36 days. The most common diagnosis was vaso-occlusive crisis (36.8%) followed by fever, upper airway infections, bacterial pneumonia and splenic sequestration. Hemoglobin values at admission were between 6 and 7 g/dL in 22.3%, with 44.2% requiring at least 1 blood transfusion. There are several acute complications of SCD, being vaso-occlusive crisis the most common. Splenic sequestration generally occurs during the first 2 years and is associated with the need of transfusion. They represent a significant burden, with each child spending approximately 3 weeks hospitalized.

Influence of SARS-CoV-2 pandemic on sleep habits in a pediatric population.

Objective: Assessment of changes in sleep habits at home in children during COVID-19 lockdown. Methods: Retrospective, transversal study in a pediatric ward of a level II hospital. Questionnaires concerning sleep quality, patterns and its modifications during lockdown were distributed from June to August 2020. Comparison with a control sample from previous study (2019). Statistical analysis on SPSS Statistics23. Results: Two groups were compared: during lockdown (n=36, mean age 9.3 years-old) and before lockdown (n=48, mean age 8.8 years-old). 55.6% stated changes in sleep patterns. There was an increase in sleep hours, specifically in school-aged children (p=0.05) and adolescents (p=0.03), with no impact in global subjective sleep quality. Significative increase in screen hours (p=0.02) and its use after dinner (p=0.04). Discussion: Changes in sleep patterns during lockdown were frequent, alongside a higher use of screens. However, these did not affect the subjective sleep quality nor increased the occurrence of sleep disturbances.

Predictors of OSA following adenotonsillectomy in children with trisomy 21.

OBJECTIVES: Given that 30%-50% of children with trisomy 21 have persistent obstructive sleep apnoea (OSA) after adenotonsillectomy, we evaluated whether demographic, clinical and polysomnographic factors predicted persistent OSA and OSA severity after adenotonsillectomy. DESIGN: Retrospective study. SETTING: Secondary care hospital. PARTICIPANTS: Retrospective review of 32 children with the diagnosis of trisomy 21 and OSA by polysomnography who underwent adenotonsillectomy, from January 2010 to December 2018. MAIN OUTCOME AND MEASURE: Non-parametric analysis was used to compare pre- and postoperative factors, and regression was used to model persistent OSA and OSA severity. RESULTS: Thirty-two children were included (17 males, median age 10.00 ± 8.00 years, median body mass index z-score 0.89 ± 1.25). Overall, adenotonsillectomy resulted in a significant improvement in median obstructive apnoea-hypopnoea index (oAHI) from 7.5 ± 8.95 to 4.40 ± 4.38 events per hour (P < .001) and in median OSA-18 score from 85.00 ± 12.00 to 61.00 ± 37.75 (P < .001). Persistent OSA was found in 56.25% of the children. Univariate regression suggests that postoperative OSA-18 score was associated with persistent OSA after adenotonsillectomy. Preoperative oAHI, preoperative oxygen desaturation index, pre- and postoperative OSA-18 scores correlated with OSA severity after adenotonsillectomy. However, in a multivariate model only the postoperative OSA-18 score correlated with OSA severity after adenotonsillectomy. CONCLUSIONS: Although adenotonsillectomy results in a significant improvement of OSA in children with trisomy 21, more than half of the children had persistent OSA. The postoperative OSA-18 score was associated both with persistent OSA and OSA severity after adenotonsillectomy.

Drug-Induced Sleep Endoscopy in Pediatric Obstructive Sleep Apnea.

OBJECTIVE: To describe drug-induced sleep endoscopy (DISE) findings in children with obstructive sleep apnea and to differentiate them between surgically naïve children and children who had adenotonsillectomy performed. STUDY DESIGN: Retrospective case series with chart review. SETTING: Secondary care hospital. SUBJECTS AND METHODS: A cohort of 56 children with the diagnosis of obstructive sleep apnea was submitted to DISE and subsequent upper airway surgery: 23 were surgically naïve, and 33 had persistent obstructive sleep apnea after adenotonsillectomy. Comparisons between groups were calculated with chi-square test and Student's t test. Simple linear regression was used to model polysomnographic indices. RESULTS: In surgically naïve children, the most common sites of obstruction were the adenoids (78.2%) and the lateral pharyngeal walls/tonsils (82.6%). In children with persistent obstructive sleep apnea after adenotonsillectomy, the most common sites of obstruction were the adenoids (54.5%), followed by the supraglottis (48.5%) and the tongue base (45.5%). No correlation was found between obstructive apnea-hypopnea index and DISE findings. Simple linear regression revealed that the degree of obstruction at the tongue base (ß = -0.73; 95% CI, -1.22 to -0.25; P = .004) and the presence of multilevel obstruction (ß = -1.75; 95% CI, -3.20 to -0.30; P = .02) predicted saturation nadir in children with persistent obstructive sleep apnea after adenotonsillectomy. CONCLUSION: DISE findings differed between surgically naïve children and children with persistent obstructive sleep apnea after adenotonsillectomy. Increased obstruction at the level of the tongue base and the presence of multilevel obstruction predicted a lower saturation nadir in children with persistent obstructive sleep apnea after adenotonsillectomy.

Overweight in youth and sleep quality: is there a link?

ABSTRACT Objective Overweight seems to be related to a higher prevalence of sleep disturbances. Decreased sleep duration and altered sleep quality are risk factors for obesity. Our aim was to compare the sleep pattern of overweight children with that of a matched control group and assess the relationship between sleep quality and obesity. Materials and methods Retrospective cohort study comparing 41 overweight children with a normal-weight control group, both submitted to polysomnography. The samples were matched for age, sex, and apnea-hypopnea index. Body mass index (BMI) z-scores were calculated using World Health Organization (WHO) growth charts. Insulin resistance in the study group was determined using the homeostatic model assessment for insulin resistance (HOMA-IR). Sleep patterns were compared. The statistical analysis was performed using SPSS® version 21. Results The mean age (± standard deviation) of the population was 10 ± 3.4 years (min. 5 years; max. 17 years). Fifty-six percent of the participants in both groups were girls. N3% was lower in the study group (18.95 ± 6.18%) compared with the control group (21.61 ± 7.39%; t (40) = 2.156, p = 0.037). We found a correlation in the study group between HOMA-IR and N3% (Rs = -0.434, p = 0.008). Conclusion The present study suggests a link between overweight/obesity and altered sleep quality due to compromised non-rapid eye movement sleep, an indirect marker of sleep quality. There was also a link between slow-wave sleep duration and insulin resistance. We must find a strategy to provide adequate slow-wave sleep duration to reduce the obesity epidemic at young ages. Further research is needed.

Overweight in youth and sleep quality: is there a link?

OBJECTIVE: Overweight seems to be related to a higher prevalence of sleep disturbances. Decreased sleep duration and altered sleep quality are risk factors for obesity. Our aim was to compare the sleep pattern of overweight children with that of a matched control group and assess the relationship between sleep quality and obesity. MATERIALS AND METHODS: Retrospective cohort study comparing 41 overweight children with a normal-weight control group, both submitted to polysomnography. The samples were matched for age, sex, and apnea-hypopnea index. Body mass index (BMI) z-scores were calculated using World Health Organization (WHO) growth charts. Insulin resistance in the study group was determined using the homeostatic model assessment for insulin resistance (HOMA-IR). Sleep patterns were compared. The statistical analysis was performed using SPSS® version 21. RESULTS: The mean age (± standard deviation) of the population was 10 ± 3.4 years (min. 5 years; max. 17 years). Fifty-six percent of the participants in both groups were girls. N3% was lower in the study group (18.95 ± 6.18%) compared with the control group (21.61 ± 7.39%; t (40) = 2.156, p = 0.037). We found a correlation in the study group between HOMA-IR and N3% (Rs = -0.434, p = 0.008). CONCLUSION: The present study suggests a link between overweight/obesity and altered sleep quality due to compromised non-rapid eye movement sleep, an indirect marker of sleep quality. There was also a link between slow-wave sleep duration and insulin resistance. We must find a strategy to provide adequate slow-wave sleep duration to reduce the obesity epidemic at young ages. Further research is needed.

Hipercalcemia grave como forma de apresentação de leucemia linfoblástica aguda em crianças / Severe hypercalcemia as a form of acute lymphoblastic leukemia presentation in children

RESUMO A hipercalcemia é um distúrbio metabólico raro em pediatria, potencialmente fatal, apresentando um vasto diagnóstico diferencial, incluindo neoplasias. Relatamos aqui o caso de uma criança de 3 anos, previamente saudável, admitida no serviço de urgência por fadiga, hiporreatividade, febre e claudicação da marcha com 5 dias de evolução, de agravamento progressivo. À observação, apresentava-se inconsciente (escore de coma Glasgow: 8). Laboratorialmente, apresentava hipercalcemia grave (cálcio total 21,39mg/dL, ionizado 2,93mmol/L) e anemia microcítica. Iniciou hiper-hidratação e foi transferido para a unidade de cuidados intensivos pediátricos. Instituiu-se hemodiafiltração venovenosa contínua com soluto livre de cálcio, ocorrendo a progressiva normalização da calcemia, com melhoria do estado de consciência. Administrou-se zolendronato. Excluíram-se causas metabólicas, infecciosas e intoxicação. O mielograma permitiu o diagnóstico de leucemia linfoblástica aguda. A hipercalcemia associada à malignidade em pediatria é rara, ocorrendo como forma de apresentação da neoplasia ou na recorrência desta. Em situações com risco de vida iminente, deve se considerar hemodiafiltração venovenosa contínua.

ABSTRACT Hypercalcemia is a rare metabolic disorder in children and is potentially fatal. It has a wide differential diagnosis, including cancer. Here, we report the case of a previously healthy 3-year-old who was admitted to the emergency room with fatigue, hyporeactivity, fever and limping gait that had evolved over 5 days and that was progressively worsening. On examination the patient was unconscious (Glasgow coma score: 8). Laboratory tests indicated severe hypercalcemia (total calcium 21.39mg/dL, ionized calcium 2.93mmol/L) and microcytic anemia. Hyperhydration was initiated, and the child was transferred to the pediatric intensive care unit. Continuous venovenous hemodiafiltration with calcium-free solution was instituted, which brought progressive normalization of serum calcium and an improved state of consciousness. Zoledronate was administered, and metabolic and infectious causes and poisoning were excluded. The bone marrow smear revealed a diagnosis of acute lymphoblastic leukemia. Hypercalcemia associated with malignancy in children is rare and occurs as a form of cancer presentation or recurrence. Continuous venovenous hemodiafiltration should be considered in situations where there is imminent risk to life.

The effect of NaCl 0.9% and NaCl 0.45% on sodium, chloride, and acid-base balance in a PICU population / Efeito do NaCl a 0,9% e do NaCl a 0,45% sobre o sódio, cloreto e equilíbrio ácido-base em uma população de UTIP

ABSTRACT OBJECTIVES: To study the effect of two intravenous maintenance fluids on plasma sodium (Na), and acid-base balance in pediatric intensive care patients during the first 24 h of hospitalization. METHODS: A prospective randomized controlled study was performed, which allocated 233 patients to groups: (A) NaCl 0.9% or (B) NaCl 0.45%. Patients were aged 1 day to 18 years, had normal electrolyte concentrations, and suffered an acute insult (medical/surgical). Main outcome measured: change in plasma sodium. Parametric tests: t-tests, ANOVA, X 2 statistical significance level was set at a = 0.05. RESULTS: Group A (n = 130): serum Na increased by 2.91 (±3.9) mmol/L at 24 h (p < 0.01); 2% patients had Na higher than 150 mmol/L. Mean urinary Na: 106.6 (±56.8) mmol/L. No change in pH at 0 and 24 h. Group B (n = 103): serum Na did not display statistically significant changes. Fifteen percent of the patients had Na < 135 mmol/L at 24 h. The two fluids had different effects on respiratory and post-operative situations. CONCLUSIONS: The use of saline 0.9% was associated with a lower incidence of electrolyte disturbances.

RESUMO OBJETIVO: Estudar o efeito de dois fluidos de manutenção intravenosos sobre o sódio (Na) plasmático e o equilíbrio ácido-base em pacientes de terapia intensiva pediátrica durante as primeiras 24 horas de internação. MÉTODOS: Foi feito um estudo controlado randomizado prospectivo. Alocamos aleatoriamente 233 pacientes para os grupos: (A) NaCl a 0,9% e (B) NaCl a 0,45%. Os pacientes com um dia a 18 anos apresentavam concentrações normais de eletrólitos e sofriam de insulto agudo (médico/cirúrgico). Principal resultado: variação no sódio plasmático. Testes paramétricos: teste t, Anova, qui-quadrado. O nível de relevância estatística foi estabelecido em a = 0,05. RESULTADOS: Grupo A (n = 130): o Na sérico aumentou 2,91 (± 3,9) mmol L-1 em 24 h (p < 0,01); 2% dos pacientes apresentaram Na acima de 150 mmol L-1. Concentração média de Na na urina: 106,6 (± 56,8) mmol L-1. Sem alteração no pH em 0 e 24 horas. Grupo B (n = 103): o Na sérico não apresentou alterações estatisticamente significativas; 15% dos pacientes apresentaram Na < 135 mmol L-1 em 24 h. Os dois fluidos tiveram efeitos diferentes sobre as situações respiratória e pós-operatória. CONCLUSÃO: O uso de solução fisiológica a 0,9% foi associado à menor incidência de distúrbios eletrolíticos.

The effect of NaCl 0.9% and NaCl 0.45% on sodium, chloride, and acid-base balance in a PICU population.

OBJECTIVES: To study the effect of two intravenous maintenance fluids on plasma sodium (Na), and acid-base balance in pediatric intensive care patients during the first 24h of hospitalization. METHODS: A prospective randomized controlled study was performed, which allocated 233 patients to groups: (A) NaCl 0.9% or (B) NaCl 0.45%. Patients were aged 1 day to 18 years, had normal electrolyte concentrations, and suffered an acute insult (medical/surgical). MAIN OUTCOME MEASURED: change in plasma sodium. Parametric tests: t-tests, ANOVA, X(2) statistical significance level was set at α=0.05. RESULTS: Group A (n=130): serum Na increased by 2.91 (±3.9)mmol/L at 24h (p<0.01); 2% patients had Na higher than 150 mmol/L. Mean urinary Na: 106.6 (±56.8)mmol/L. No change in pH at 0 and 24h. Group B (n=103): serum Na did not display statistically significant changes. Fifteen percent of the patients had Na<135 mmol/L at 24h. The two fluids had different effects on respiratory and post-operative situations. CONCLUSIONS: The use of saline 0.9% was associated with a lower incidence of electrolyte disturbances.

Sleep pathology characterization in sickle cell disease: case-control study.

BACKGROUND: Children and adolescents with sickle cell disease (SCD) have a higher incidence of sleep pathology and obstructive sleep apnea syndrome (OSAS). The nocturnal hypoxemia is a risk to vaso-occlusive crisis among other SCD morbidities. Our aim was to compare polysomnography (PSG) results in a sample of children with SCD with a sample of children with suspected OSAS without SCD. DESIGN AND METHODS: A retrospective study compared clinical and PSG parameters. A descriptive analysis and t-test were done considering P < 0.05 as significant. RESULTS: PSG was done in 65 children with SCD and 65 control-children. Control sample was selected to be equal to SCD sample considering gender (53.8% were male), age (mean age was 9.4 years (SD ± 4.6) and AHI (mean 3.57 events/hr). Mean efficiency, latency and percentage of sleep phases in both groups showed no statistically significant differences. Mean SpO2 and minimum SpO2 were lower in SCD group and it was statistically significant (P < 0.01). Enuresis was more frequent in the SCD children group (35.4% vs. 6.2%, P < 0.01). CONCLUSION: Comparing children with and without SCD, sleep architecture was similar in both groups and minimum SpO2 was significantly lower in SCD children although both groups had a similar AHI. This is an important issue in these children, so it is essential to have a sleep evaluation in order to prevent complications and co-morbidities.

The family role in children׳s sleep disturbances: Results from a cross-sectional study in a Portuguese Urban pediatric population.

BACKGROUND: Sleep Disorders (SlD) are frequently undervalued complaints in childhood. Several factors influence sleep, particularly socio-cultural environment and medical conditions such as breathing disorders. Poor sleep hygiene has physical, educational and social consequences. In Portugal, there are few published studies about children׳s sleep habits and rarely based on validated questionnaires. AIM: To study the prevalence of SlD and associated factors, in an outpatient pediatric population of a Primary Health Care Center (PHCC). METHODS: Cross-sectional study of children admitted to a PHCC on a suburban area of Lisbon. Children Sleep Habits Questionnaire, validated for the Portuguese population (CSHQ-PT) for the screening of SlD (cut-off=44), was applied to parents, as well as a demographic inquiry. Body mass index z-score was evaluated. Children scoring 44 or above were sent to Pediatric Sleep Disorders consultation (PSDC). Parametric and non-parametric tests were used whenever appropriate. RESULTS: From 128 children, 57.8% were male; the median age was 6.0 years (P 25=5.0; P 75=8.0). The median of cohabitants per family was 4.0 (P 25=3.0; P 75=5.0); 21.1% lived in a single-parent family. From CSHQ-PT, 59.4% (76) scored above the cut-off. Data showed that children from a single-parent family have more SlD (p=0.048), particularly parasomnia (p=0.019). Children with sleep disordered breathing (SDB) suffer more daytime sleepiness (p=0.034). From 63 children sent to PSDC, 33 attended. Regarding these children, a difference was found between BMI z-scores of those with and without SDB (p=0.06). CONCLUSION: Family structure plays a non-negligible role in children's sleep habits. Daily performance of children with SDB may become compromised.

Severe hypercalcemia as a form of acute lymphoblastic leukemia presentation in children.

Hypercalcemia is a rare metabolic disorder in children and is potentially fatal. It has a wide differential diagnosis, including cancer. Here, we report the case of a previously healthy 3-year-old who was admitted to the emergency room with fatigue, hyporeactivity, fever and limping gait that had evolved over 5 days and that was progressively worsening. On examination the patient was unconscious (Glasgow coma score: 8). Laboratory tests indicated severe hypercalcemia (total calcium 21.39mg/dL, ionized calcium 2.93mmol/L) and microcytic anemia. Hyperhydration was initiated, and the child was transferred to the pediatric intensive care unit. Continuous venovenous hemodiafiltration with calcium-free solution was instituted, which brought progressive normalization of serum calcium and an improved state of consciousness. Zoledronate was administered, and metabolic and infectious causes and poisoning were excluded. The bone marrow smear revealed a diagnosis of acute lymphoblastic leukemia. Hypercalcemia associated with malignancy in children is rare and occurs as a form of cancer presentation or recurrence. Continuous venovenous hemodiafiltration should be considered in situations where there is imminent risk to life.