Acute splenic sequestration in female children with sickle cell disease in the North of Jordan

The objective of this study was to evaluate the rate of acute splenic sequestration (ASSC) in patients with sickle beta-thalassaemia and sickle cell anaemia, the risk of recurrence in those who survive the first episode, and the relationship between ASSC episodes and subsequent hypersplenism. All patients with confirmed diagnosis of sickle cell disease at a tertiary referral teaching hospital, between January 1994 and December 2002 were interviewed and had their medical records reviewed. Seventy-seven patients with sickle cell disease were identified. Their ages ranged between 2 and 18 years (mean, 10.1 years). There were 35 females and 38 males. Thirty-seven (50.6%) had sickle beta-thalassaemia, and 36 (49.4%) had homozygous sickle cell anaemia. Of these, 26 had high level of Hb F and 11 had normal level of fetal haemoglobin (Hb F). Twenty-one patients (28%) had 63 episodes of acute splenic sequestration. Thirty-seven episodes were experienced by 12 patients with sickle beta-thalassaemia; of these 11 were major attacks with one fatality. Twenty-six episodes were experienced by nine patients with sickle cell anaemia. Splenomegaly and hypersplenism were greater in the acute splenic sequestration group than in the rest of the sickle cell anaemia patients, and the differences were extremely significant. ASSC was found in nine siblings of sickle beta-thalassaemia group, while none were found in the sickle cell anaemia group. The mean age of the first episode was significantly higher in sickle beta-thalassaemia, with significant differences in the levels of Hb F, Hb S, size of spleen and severity of crisis between both groups. In the sickle cell anaemia group the only significant difference between patients with and these without acute splenic sequestration was the difference in the size of spleen. In this study, the rate of ASSC in the sickle beta-thalassaemia patients was 32%, in contrast to 25% in the sickle cell anaemia patients. The risk of recurrence was about 70% in those who survived their first episodes. There was a close relationship between ASSC and subsequent hypersplenism. Important predictable factors for ASSC in sickle beta-thalassaemia patients were the presence of splenomegaly of more than 5 cm below the costal margin, history of acute splenic sequestration in siblings and high Hb F. Most of first episodes in sickle cell anaemia occur under the age of 2 years, while in sickle beta-thalassaemia the majority of patients have their first crisis at the age of > or =3.5 years.     

Splenectomy and acute splenic sequestration crises in sickle cell disease

Acute splenic sequestration crises (ASSC) is one of the complications of sickle cell disease (SCD) that can be life-threatening due to loss of blood volume. Over a 5-year period, we have treated 19 patients ranging in age from 4 to 32 years with ASSC. There were 14 males and 5 females; 17 had homozygous SCD and the other 2 had sickle thalassemia. Two patients presented with severe anemia and acute circulatory collapse; 1 of them developed residual weakness of his limbs and decreased visual acuity. Nine patients underwent splenectomy after major episodes of sequestration while the remaining 10 had recurrent minor episodes of sequestration. The clinical features and the role of splenectomy are discussed.     

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.     

Human parvovirus B19-induced aplastic crisis in iron deficiency anemia

Human parvovirus B19 (HPVB19) infects and replicates in erythroid progenitor cells. Its specific cytotoxic effect on these cells results in aplastic crises in patients with congenital hemolytic anemias. Aplastic crisis due to HPVB19 infection in a healthy girl revealed occult iron deficiency anemia. The condition is characterized by a high serum iron level in the aplastic phase and rapid recovery after administration of iron. Temporary HPVB19-induced red blood cell aplasia could occur in patients with other anemias, particularly those with non-inherited form of hemolysis.     

Parvovirus B19-associated transient red cell aplasia in children: the role of bone marrow examination in unusual presentations

Human parvovirus B19 (PV B19) infection in children commonly presents as fifth disease. Transient red cell crisis, the other manifestation of PV B19 infection, is usually reported in children with chronic hemolytic anemia, with a worsening of the anemia. However, this condition may pass unrecognized in children without an underlying hemolytic disorder, since the anemia may be of a short duration and self-limiting. The authors report 3 cases of PV B19-induced transient aplastic in different clinical settings--pancytopenia in one child, during induction phase for acute lymphoblastic leukemia in the second, and fever with joint pains in the third. Treatment for PV B19-induced transient aplastic crisis is essentially supportive. There may be a dilemma in patients on immunosuppressive therapy, since initially it is difficult to distinguish between chronic pure red cell aplasia (a condition where intravenous immunoglobulin therapy is beneficial) and transient aplastic crisis, where supportive red cell transfusions suffice. The patient with leukemia also recovered spontaneously despite being on steroids. In all the 3 patients, the pure red cell aplasia recovered spontaneously without administration of intravenous gammaglobulins.     

Life threatening parvovirus B19 and herpes simplex virus associated acute myocardial dysfunction in a child with homozygous sickle cell disease

Human parvovirus (HPV) B19, a common infection, frequently causes transient red cell aplasia in children with hemolytic anemia, such as sickle cell disease (SCD). It was considered to be a self-limited condition, easily treated with blood transfusion. However, acute splenic sequestration, acute chest syndrome, nephrotic syndrome, and stroke have been reported in SCD patients following HPV B19 infection. We report a 3-year-old child with SCD who developed fulminant myocarditis following HPV B19-related aplastic crisis. The diagnosis of myocarditis should be considered in a patient with hemolytic anemia with an infection with HPV B19 who develops signs of cardiopulmonary failure despite correction of anemia.     

Varied clinical course of aplastic crisis in hereditary spherocytosis

This study is the first to report a familial case involving differing clinical courses of aplastic crisis triggered by parvovirus B19 in two patients with HS, although similar eosin‐5‐maleimide‐binding test and sodium dodecylsulfate–polyacrylamide gel electrophoresis results had been obtained for both. One patient had short‐term mild symptoms, whereas the other patient developed severe anemia that required blood transfusion, experienced fever for 13 days, and did not have any rash. The severity of aplastic crisis is reported to be correlated with the severity of the underlying hemolytic anemia; the present findings show that the severity of infection should also be considered as an important predictive factor of the severity of aplastic crisis.     

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.     

An analysis of inpatient pediatric sickle cell disease: Incidence,costs, and outcomes

1 Objective

To identify characteristics of pediatric sickle cell disease (SCD) hospitalizations and to examine admission demographics and medical expenditures.

2 Methods

Admissions with SCD were identified from the 2009 and 2012 releases of the Healthcare and Cost Utilization Project's Kids Inpatient Database. Disease‐specific secondary diagnoses including acute chest syndrome (ACS), vaso‐occlusive pain crisis (VOC), splenic sequestration, and stroke/transient ischemic attack were analyzed for patient and hospital demographics. Analytical endpoints included total healthcare expenditures and mortality.

3 Results

We reviewed 75,234 inpatient hospitalizations with a diagnosis of SCD. Over $900,000,000 was spent annually in associated healthcare expenditure. The median length of hospitalization stay (LOS) for all admissions was 3 days (interquartile range [IQR] 2–5 days). VOC was the most frequent secondary diagnosis, recording 48,698 total hospitalizations and a median LOS of 3 days (IQR 2–6 days). Of the 8,490 hospitalizations with ACS, the infant population had a significantly higher mortality rate compared to other age groups (2% vs. 0.3%, P < 0.001). Cerebral vascular accidents incurred the second highest median hospitalization cost ($18,956), behind ACS ($22,631). A high proportion of Caucasian patients died during hospitalization for VOC (0.4% vs. 0.1%, P = 0.014) and ACS (4% vs. 0.2%, P < 0.001) when compared to non‐Caucasians.

4 Conclusion

Inpatient hospitalizations for secondary manifestations of pediatric SCD were associated with significant healthcare expenditures. Patients with an increased statistical risk for death during hospitalization included Caucasians with SCD complications of ACS and VOC, and patients <1‐year‐old with ACS. Further research is needed to substantiate the associated clinical significance of these findings.     

Management of sickle cell disease: management of acute episodes in the community and in hospital

This review discusses the presentation and management of acute sickle crises, highlighting which aspects of diagnosis and management can be undertaken in the community and which require urgent referral to hospital. GP's, community nurse specialists, and community paediatricians should be aware of the different acute presentations in order to provide effective and safe care, and should understand warning symptoms and signs which indicate the need for assessment in hospital. It is also important that the parents have a good awareness of these symptoms and know when and how to seek help. The common complications which may be encountered in an acute hospital setting are described together with recommendations for management based on published evidence and the author's experience.     

Impact of hospitalization for vaso-occlusive crisis on health-related quality of life in children with sickle cell disease

Background

Sickle cell disease (SCD) is characterized by vaso-occlusive crises (VOCs) that impair the health-related quality of life (HRQoL). The aim of this study is to evaluate the impact of hospitalization for VOCs on HRQoL in children with SCD over time.

Methods

In this longitudinal cohort study, children aged 8–18 years diagnosed with SCD at the Amsterdam UMC were included between 2012 and 2021. HRQoL was annually measured as part of standard care using the Pediatric Quality of Life Inventory. The impact of hospitalization for VOC on HRQoL was evaluated using linear mixed models 3, 6, 9, and 12 months after hospitalization. The effect of frequency of hospitalization for VOC on HRQoL was evaluated over the last 12 months.

Results

In total, 94 children with SCD were included with a median age of 11.8 years (interquartile range [IQR]: 9–14). Thirty-seven patients (39%) had been hospitalized for a VOC. Hospitalization for VOC led to a decrease of 3.2–4.8 points in total HRQoL compared to patients without hospitalization, most pronounced 3 months after hospitalization. Recurrent admission for VOC in the last 12 months was associated with a decrease of 2.3 points in total HRQoL (p = .04). The most affected subscale was physical functioning.

Conclusion

The adverse effects of hospitalization for VOC in children with SCD persist up to 12 months after hospitalization. After hospitalization for VOC, extra attention and support for its negative impact on HRQoL are recommended. This study also underlines the importance of systematically measuring HRQoL, allowing clinicians to intervene accordingly.     

Reversible posterior leuko‐encephalopathy in children with sickle cell disease

Children with sickle cell disease (SCD) have high risk of neurologic morbidity and mortality, such as strokes, silent infarcts and TIA's. A retrospective review of magnetic resonance imaging and magnetic resonance angiography identified eight children with radiological and clinical characteristics of reversible posterior encephalopathy (RPLS). These patients had no evidence of previous cerebral infarcts or vasculopathy. Three have died during the 5‐year follow up; one developed a stroke and one a conditional TCD. RPLS needs to be considered in the differential diagnosis of children with SCD that present with acute neurological changes, especially if they are already been hospitalized. Pediatr Blood Cancer 2009;52:373–375. © 2008 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.     

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.