Acute encephalopathy with parvovirus B19 infection in sickle cell disease.

A 13 year old girl with haemoglobin Sbeta(+)thalassaemia developed simultaneous aplastic crisis and encephalopathy associated with parvovirus B19 (PB19) infection. Brain magnetic resonance imaging findings were consistent with central nervous system (CNS) vasculitis and her symptoms resolved with steroid therapy. Thus, PB19 induced CNS hypersensitivity vasculitis must be considered in the differential diagnosis of encephalopathy.

Characterization of pica prevalence among patients with sickle cell disease.

OBJECTIVE: To determine the prevalence of pica and its characteristics among children with sickle cell disease. DESIGN: Retrospective, observational study. SETTING: An urban, ambulatory care, interdisciplinary center. PATIENTS: The medical records of all 480 patients who visited the center from March 1, 1998, to June 30, 1999, were reviewed. Patients were excluded for history of stroke, long-term transfusions, pregnancy, acute illness, or age younger than 3 years. MAIN OUTCOME MEASURES: Sex, age, weight, height, Tanner stage, complete blood cell count, sickle cell genotype, pica history, and levels of iron, zinc, lead, and fetal hemoglobin (Hb). RESULTS: Of 395 study patients, 134 (33.9%) reported pica. Ingested items included paper, foam, and powders. There was a significantly higher prevalence of pica among patients homozygous for Hb S (Hb SS, sickle cell anemia) compared with the combined group of double heterozygous patients with Hb SC, Hb SD, and Hb Sbeta thallasemia (Sbeta(+)or Sbeta(0)) (35.6% vs 25.5%; P =.03). Within genotype, mean Hb levels were significantly lower and reticulocyte counts were significantly higher in the patients with pica. Overall, the mean age of patients with pica was significantly lower; however, the prevalence was 23.3% (27/116) among those aged 10.0 to 14.9 years and 14.8% (8/54) among those aged 15.0 to 19.0 years. Within age groups, patients with pica weighed significantly less. CONCLUSIONS: Pica appeared to have an unusually high prevalence in patients with sickle cell disease and a correlation with lower Hb levels. It is unclear whether pica is a specific marker of disease severity, because our review did not show a relationship to increased number and duration of hospitalizations. The association between pica and low body weight suggests a nutritional effect on its prevalence.

Sickle cell.

The initiation of newborn screening and its virtually universal implementation will eventually yield a population in which sickle cell disease has been identified and comprehensive care is provided for children. The situation with SCT is different; there will continue to be the identification of parents who have the potential for having a child with a sickle cell disease but because they will not be tested or counseled, there will continue to be a population of children with a sickle cell disease whose parents have not been enabled to make informed decisions that they believe are in their best interest relative to family planning. Also, we will continue to have a population of pregnant women with a fetus with sickle cell disease who will not be given an opportunity to decide whether they wish to continue or terminate the pregnancy. They all will give birth to a child with a lifetime of chronic illness with its associated psychological, social, and financial burdens for the individual and his or her parents. The failure to implement prenatal diagnosis is an abridgment of two fundamental rights: the right to know and the right to decide. In this case it is the right to know about the potential health status of their children if that is possible, and the right to decide about the actual health care status of their children if options are available.

Prevalence of penicillin-nonsusceptible Streptococcus pneumoniae in nasopharyngeal cultures from patients with sickle cell disease.

BACKGROUND: Prevention of pneumococcal sepsis in children with sickle cell disease (SCD) is threatened by the emergence of penicillin-nonsusceptible pneumococci. METHODS: In this study, nasopharyngeal colonization with Streptococcus pneumoniae and penicillin susceptibility were compared in children with SCD and a control group. Nasopharyngeal cultures were obtained from 130 children with SCD and 123 control children. Penicillin susceptibility was determined by Epsilometer test. Compliance with penicillin prophylaxis in SCD patients was determined by parent interviews and review of patients' medical and pharmacy records. RESULTS: Streptococcus pneumoniae was isolated from 8 (6%) of 130 SCD patients, and 21 (17%) of 123 control patients. Of the 29 S pneumoniae isolates, 6 (21%) were nonsusceptible to penicillin; 4 of 8 (50%) were from the SCD group and 2 of 21 (10%) from the control group. CONCLUSIONS: Penicillin prophylaxis decreased the rate of S pneumoniae colonization in SCD patients; however, it also increased the risk of selective colonization with penicillin-nonsusceptible S pneumoniae.

Maxillary sinus marrow hyperplasia in sickle cell anemia.

Marrow hyperplasia is a sequela of sickle cell anemia (SCA) and may be seen in the skull in children after 5 years of age [1]. The facial bones, except for the mandible and orbits, are usually not involved [1-3]. We report an unusual case of a 28-month-old black boy with SCA who presented with extensive marrow hyperplasia of the maxillary sinuses in addition to severe calvarial and mandibular changes. The imaging characteristics on CT (similar to other sites of marrow hyperplasia) and MR (low signal on both T1 and T2 sequences) should aid in making the correct diagnosis.