Clinical Observations, Plasma Retinol Concentrations, and In Vitro Lymphocyte Functions in Children With Sickle Cell Disease.

BACKGROUND: Children with sickle cell disease (SCD) often have infections, growth deficits, and impaired immunity, problems that also are observed in individuals with a vitamin A deficiency (plasma retinol concentration <20 µg/dL). The goal of this study was to investigate the association between vitamin A, health status, and the in vitro immune function of children with SCD. METHODS: Fifty-nine children (40 SS, 11 SC, and 8 Sßthalassemia [Sßthal] hemoglobin genotypes) 9 months to 18 years old were investigated for plasma levels of retinol, retinol binding protein, C-reactive protein, alpha-1-acid glycoprotein, lymphocyte proliferation, and interleukin (IL)-2 activity in supernatant of phytohemagglutinin-treated lymphocytes. RESULTS: The plasma retinol concentrations of children with SCD (mean 57.6 µg/dL, range 4.6-116 µg/dL) were not different from those of 21 normal individuals (mean 62 µg/dL, range 28.7-162 µg/dL). Plasma retinol concentrations did not vary by hemoglobin genotype but were lower in boys than in girls (P < 0.05) and were also lower in children with inflammation (P = 0.1). Seven children (11.9%) (6 HbSS, 1 HbSß0thal) were vitamin A-deficient, and 9 children (15.3%) had suboptimal vitamin A status (plasma retinol concentration of 20-29 µg/dL). Children with vitamin A deficiency had slightly lower height (P = 0.09) and weight mean percentiles, lymphocyte proliferative responses, and IL-2 activity (P > 0.1), but higher means of C-reactive protein (P = 0.05), pain crisis episodes and inflammation (P = 0.1), and health scores (P > 0.1) than children who were not vitamin A-deficient. Lymphocyte proliferative responses negatively correlated with health score, pain crisis episodes, and blood units received, but positively correlated with retinol binding protein (P < 0.05 to P = 0.1). CONCLUSION: Identification and correction of suboptimal vitamin A status in children with SCD may improve immunity and attenuate certain health complications associated with this disease.

Pesticide (endosulfan) levels in the bone marrow of children with hematological malignancies.

OBJECTIVES: (1) To confirm the presence of Pesticide (Endosulfan) residues in the bone marrow (BM) of children with acute hematological malignancies and compare them with controls. (2) To ascertain if children with Endosulfan in their marrow reside in areas sprayed with Endosulfan. STUDY DESIGN: Case control study SETTING: Pediatric oncology unit of a medical college teaching hospital in Dakshina Kannada district of Karnataka. SUBJECTS: 26 patients with proven hematological malignancy and 26 age matched controls suffering from benign hematological disease. METHODS: Endosulfan residues in the BM were estimated by gas chromatography mass spectrometry (Minimum detection limit 10ng/mL). The subjects geographical area of location (residence) was determined to see whether they belong to sprayed area or not. The Chi-square test was applied to see an association between exposure status and hematological malignancy. RESULTS: A total of 52 children were enrolled of which 26 were study cases and 26 were controls. Of the study and control groups, 84.7% ;and 73.1%, respectively were from exposed areas. The major (88.4%) illness in the study group was ALL, while ITP (50%) occurred most frequently in the control group. Six out of 26 study cases tested positive for endosulfan in the BM, against 1 out of 26 controls (P = 0.042). The Odds ratio was 7.5. All children who had endosulfan in the bone marrow originated from areas, where endosulfan is still being used. CONCLUSIONS: Children with hematological malignancy had raised levels of endosulfan in the bone marrow compared to those without. All the children with raised bone marrow Endosulfan levels were found to be from areas exposed to the pesticide.

Carcinoid tumor with bilateral renal involvement in a child.

Carcinoid tumors are uncommon in children. Kidneys are rarely involved as they do not possess neuro-endocrine cells. Work up of painless hematuria after abdominal trauma in a 10-year-old boy revealed primary carcinoid tumors with metastasis to both kidneys. We were unable to find any previous reports of renal involvement by carcinoid tumor in children.