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.

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.

Plasma zinc levels inversely correlate with vascular cell adhesion molecule-1 concentration in children with sickle cell disease.

Zinc deficiency has been implicated in impaired cell-mediated immunity of children with sickle cell disease (SCD). However, its influence on the expression of vascular cell-adhesion molecule-1 (VCAM-1) on endothelial cells, a protein involved in vasoocclusion, has not been previously investigated. We therefore measured (soluble) sVCAM-1 and zinc in 76 SCD children and 96 non-SCD children, mean age 7.73 years and 11.24 years, respectively. Although mean zinc levels of both groups were within the normal range (approximately 14.5 micromol/l), 14.5 % of SCD and 11% of non-SCD children (without inflammation) had levels below normal (10.7 micromol/L). Mean sVCAM-1 concentrations of SCD children (837 microg/l) were significantly higher than those of controls (627 microg/l) (p < 0.001). Differences persisted after taking into account age, hemoglobin phenotype, and inflammation (alpha-l acid glycoprotein >l g/l and C-reactive protein >10 mg/I). sVCAM-1 negatively correlated with serum (r = -0.444) and red blood cells zinc (r = -0.242, p < 0.05) but not with acute-phase proteins. Mean sVCAM-1 tended to be higher in SCD children with than in those without a history of a health problem (infection, pain crisis or were transfused; not significant). Data suggest that zinc may modulate the clinical status of SCD children through VCAM-1 expression, and zinc supplementation may be beneficial in these patients.

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.

Younger women are affected by breast cancer in South India - a hospital-based descriptive study.

INTRODUCTION: Breast cancer is one of the most common cancers in the world among women and its effective control depends upon sufficient knowledge about its epidemiology at the regional as well as global levels. MATERIALS AND METHODS: A record based descriptive study was conducted in a tertiary care hospital situated in coastal Karnataka in South India. Cases included were all histopathologically diagnosed cases of breast cancer not otherwise specified among females that were treated in the hospital in the 3 years period of 2005-2007. A total of 112 cases were found and their data retrieved and analyzed using SPSS. RESULTS: Mean age of the subjects was 45 years (SD=10.4) (range 23 -70). Age distribution showed two peaks at 35-39 years and 50-54 years. Some 12 (11%) patients had a previous history of breast cancer and 9 (8%) had a family history. Among the patients who had history of breast cancer in the past, 61.5% were during the pre-menopausal age. CONCLUSION: The age affected by breast cancer was lower than usually seen in other parts of the world and there was more of premenopausal occurrence which has implications on their own and their relatives' chances of acquiring breast cancer later. The findings show that breast cancer control efforts and recommendations in different parts of the world have to be tailored to the regional epidemiological patterns.

Carney Complex: case report and review.

Carney complex is a very rare multiple neoplasia syndrome with cardiac, cutaneous, and neural tumours with a variety of pigmented lesion of skin. We are reporting a rare case of carney complex in which left atrial myxoma with superficial angiomyxoma, giant cell tumour of bone and lentigines showed a unique association. This patient underwent successful surgical excision of left atrial myxoma under cardiopulmonary bypass.

Effective management of pain in pediatric hematology and oncology.

In the last several decades, there have been major advances in the treatment of pediatric cancers. 5 year survival of children with acute lymphoblastic leukemia has increased from 25% to 80%. Early stages of non -Hodgkin's, Hodgkin's and Wilms tumors all have more than 90% long term survival. In addition to improving survival, the comprehensive care of children with cancer must offer total care including special emphasis on pain management and psychosocial support by a multidisciplinary team. Pain considerations in children are unique and differ from those in adults. For example, bone pain is often one of the presenting symptoms of leukemia in children, but can be mistaken for growing pain or labeled psychological. Bone pain is also a prominent symptom in late stage neuroblastoma, and of course in bone tumors. The American Medical Association and National Cancer Institute promote the absence of pain as a patient right and a marker of good clinical care and a quality of care issue. Pain due to disease burden responds dramatically to chemotherapy and the uninitiated are often surprised by the sudden increase in activity and playfulness of children undergoing induction chemotherapy. History and physical data, with special assessment of pain should be part of the medical record of all children.

Tonsillectomy and adenoidectomy for obstructive sleep apnea in sickle cell anemia.

OBJECTIVE: To evaluate the effects of Tonsillectomy and Adenoidectomy (T&A) on Obstructive Sleep Apnea (OSA) secondary to Upper Airway Obstruction (UAO) in children with Sickle Cell Disease (SCD). METHODS: Twenty eight children with SCD and history of snoring, with or without adeno tonsillar hypertrophy, were evaluated for UAO and OSA. RESULTS: Eight children were confirmed to have OSA by multichannel polysomnography and underwent T&A. Follow up examination showed symptomatic clinical and objective improvement in sleep study parameters in OSA. CONCLUSIONS: T&A appears to be a safe, effective option to treat selected patients with OSA due to UAO in SCD children.

A rare case of congenital diaphragmatic hernia with ectopic liver and absent pericardium.

We present a 37-year-old woman with a rare combination of absent pericardium with congenital diaphragmatic hernia. Only 3 patients with congenital diaphragmatic hernia with complete absence of pericardium and ectopic liver have been described, and all of them were neonates. This interesting case gives us an opportunity to study the natural history of this rare combination of anatomic defect and consolidate the existing scarce data on this condition.