Diverse manifestations of acute sickle cell hepatopathy in pediatric patients with sickle cell disease: A case series

The hepatic complications of sickle cell disease (SCD) are associated with increased morbidity and mortality in adults; children usually survive but may suffer significant sequelae. Few diagnostic tools differentiate the various hepatic manifestations of SCD. Why patients exhibit one hepatic pathology versus another is unclear. We report four pediatric patients with hemoglobin SS disease with diverse manifestations of acute hepatic involvement including acute sickle hepatic crisis, hepatic sequestration, sickle cell intrahepatic cholestasis, and a non‐SCD cause of hepatopathy in a patient with viral hepatitis. These complications require a systematic approach to extensive evaluation and coordinated multidisciplinary care.     

Effects of adenotonsillectomy on polysomnographic parameters in children with sickle cell disease

Obstructive sleep apnea (OSA) in the pediatric sickle cell disease (SCD) population can promote nightly hemoglobin oxygen desaturation, which increases the risk of central nervous system insult and may impair cognitive function. Adenotonsillectomy can ameliorate OSA symptoms, but its effect in children with SCD has not been fully investigated. We reviewed the effects of adenotonsillectomy in thirteen children with SCD by comparison of pre and post‐adenotonsillectomy polysomnography (PSG) parameters. Significant reduction in hemoglobin oxygen desaturation, decreased apnea‐hypopnea index, and increased rapid eye movement sleep occurred after adenotonsillectomy. Adenotonsillectomy promotes improvement in sleep quality in children with SCD and PSG‐confirmed OSA. Pediatr Blood Cancer 2013; 60: E26–E28. © 2013 Wiley Periodicals, Inc.     

Acute silent cerebral infarction in children with sickle cell anemia

Silent cerebral infarctions (SCI) occur in up to 35% of children with sickle cell anemia (HbSS) but are rarely recognized during the initial 10–14 days when diffusion weighted magnetic resonance imaging (MRI) can differentiate acute infarctions from remote events. We report acute SCI in seven children with HbSS who had areas of restricted diffusion on MRI without persistent focal neurologic deficits. Four had acute SCI identified following acute anemic events. Our observations suggest that SCI are detectible in the acute phase, present with subtle neurologic symptoms, result in permanent neurologic injury, and may be caused by acute anemic events. Pediatr Blood Cancer 2010;54:461–464. © 2009 Wiley‐Liss, Inc.     

Variation in hospitalizations and hospital length of stay in children with vaso-occlusive crises in sickle cell disease

OBJECTIVE: As a measure of morbidity of vaso-occlusive crises (VOC) in sickle cell disease, we used a national hospital discharge database, the Kids' Inpatient Database (KID), to determine the number of hospitalizations and hospital length of stay (LOS) by age. PROCEDURE: Nationally weighted hospital discharges for VOC in children with sickle cell disease were analyzed using data from 2,500 hospitals in the Healthcare Cost and Utilization Project (HCUP) KID. Number of discharges and hospital LOS by age group were analyzed. Multiple linear regression was performed to analyze the effect of age on hospital LOS. RESULTS: There were 20,271 hospital discharges for children with sickle cell disease and VOC. Mean age (SD) at time of admission was 10.6 (5.38) years with 49% being males. The overall average LOS was 4.4 days. There were differences in the number of hospital discharges by age group (P < 0.001). In addition, there was a difference in LOS by age group (P < 0.0001). After controlling for potential confounders, older age was associated with a longer LOS (P < 0.0001). CONCLUSIONS: Older children with sickle cell disease and VOC have increased hospitalizations and longer LOS. This age effect should be considered when measuring the effect of an intervention on hospital utilization in these children.     

Prospective evaluation for respiratory pathogens in children with sickle cell disease and acute respiratory illness

Background

Human rhinovirus (HRV), human coronavirus (hCoV), human bocavirus (hBoV), and human metapneumovirus (hMPV) infections in children with sickle cell disease have not been well studied.

Procedure

Nasopharyngeal wash specimens were prospectively collected from 60 children with sickle cell disease and acute respiratory illness, over a 1‐year period. Samples were tested with multiplexed‐PCR, using an automated system for nine respiratory viruses, Chlamydophila pneumoniae, Mycoplasma pneumoniae, and Bordetella pertussis. Clinical characteristics and distribution of respiratory viruses in patients with and without acute chest syndrome (ACS) were evaluated.

Results

A respiratory virus was detected in 47 (78%) patients. Nine (15%) patients had ACS; a respiratory virus was detected in all of them. The demographic characteristics of patients with and without ACS were similar. HRV was the most common virus, detected in 29 of 47 (62%) patients. Logistic regression showed no association between ACS and detection of HRV, hCoV, hBoV, hMPV, and other respiratory pathogens. Co‐infection with at least one additional respiratory virus was seen in 14 (30%) infected patients, and was not significantly higher in patients with ACS (P = 0.10). Co‐infections with more than two respiratory viruses were seen in seven patients, all in patients without ACS. Bacterial pathogens were not detected.

Conclusion

HRV was the most common virus detected in children with sickle cell disease and acute respiratory illness, and was not associated with increased morbidity. Larger prospective studies with asymptomatic controls are needed to study the association of these emerging respiratory viruses with ACS in children with sickle cell disease. Pediatr Blood Cancer 2014;61:507–511. © 2013 Wiley Periodicals, Inc.

     

Neurocognitive deficits in children with sickle cell disease: a comprehensive profile

Background

Sickle cell disease (SCD) can lead to profound cerebral damage, associated with neurocognitive deficits. The aim of the current study was to evaluate a broad range of neurocognitive functions in children with SCD compared to a SES‐matched control group, in order to gain more insight into the specific deficits of these patients.

Methods

Forty‐one children with homozygous SCD (HbSS or HbS‐β0‐thalassemia) and 38 controls were assessed on a comprehensive set of well‐defined and validated measures of neurocognitive functioning. Besides general intelligence, we evaluated executive functioning extensively (including response inhibition, sustained attention, planning, visuo‐spatial working memory, and verbal working memory) as well as visuo‐motor functioning.

Results

SCD was clearly associated with lower IQ scores. More than one in three children with SCD had a Full‐scale IQ below 75. Furthermore, children with SCD showed deficits in visuo‐motor functioning. Some evidence was found for executive dysfunction: Children with SCD displayed poor visuo‐spatial working memory, as well as subtle deficits in sustained attention and planning. No significant differences were found between children with SCD and controls in terms of response inhibition and verbal working memory.

Conclusions

Children with SCD are at increased risk of lower intelligence, visuo‐motor impairments, and executive dysfunction. These neurocognitive deficits may underlie high rates of scholastic impairments in these children. The present findings further illuminate the importance of regular neurocognitive evaluations and future neurocognitive rehabilitation programs for children with SCD. Pediatr Blood Cancer 2011;56:783–788. © 2010 Wiley‐Liss, Inc.     

The effect of acute pain crisis on exhaled nitric oxide levels in children with sickle cell disease

Exhaled nitric oxide (FE(NO)) has been shown to be decreased in children with sickle cell disease. We sought to evaluate the effect of sickle cell vaso-occlusive crisis (VOC) on FE(NO) levels. We measured FE(NO) levels in 42 children with sickle cell disease, 29 in their baseline health and 13 during an acute VOC. There was no difference in FE(NO) levels between children at baseline (15.12 +/- 9.32 ppb) and those during an acute VOC (15.68 +/- 7.26 ppb; P = 0.794). FE(NO) is not a useful marker of acute VOC in children with sickle cell disease.