Raj P. Warrier

The Immune Response in Protein-Energy Malnutrition and Single Nutrient Deficiencies

Protein-energy malnutrition (PEM) (undernutrition or overnutrition) is one of the nutritional problems recognized by the WHO as public health problems in different parts of the world. The others are nutritional anemias, vitamin A deficiency, and iodine deficiency. Primary PEM (undernutrition) has long been regarded as a problem of underprivileged, low-socioeconomic groups, especially those from less developed countries. Although severe malnutrition (kwashiorkor and/or marasmus) are rare except in countries hit by natural disasters such as drought and war, mild and moderate malnutrition are quite common. The prevalence of this form of malnutrition varies from 21 to 70% depending on the continent and/or country (Rao and Naider, 1977; Suskind et al., 1990). This form of malnutrition may not be any less deleterious on the immune response than severe malnutrition, especially when it occurs in the context of poor sanitation, infections, and multiple nutritional deficiencies.

Successful treatment of multisystem Langerhans cell histiocytosis (histiocytosis X) with etoposide.

Purpose Langerhans cell histiocytosis (LCH) in its disseminated form usually occurs in the very young, and has a fulminant, rapidly progressive, and fatal course despite different forms of therapy. Patients and Methods We treated two patients, who had failed on vinblastine treatment, with i.v. etoposide (VP-16) at a dose of 150 mg/kg/day for 3 days. Patient I, 8 months of age, presented with failure to thrive and huge bilateral granulomatous lesions of the external auditory canal with erosion and extensive destruction of the petrous pyramids and mastoid area. Patient II, 20 months of age, presented with widespread purpuric skin rash, hepatosplenomegaly, and bone marrow involvement. Results Both patients sustained complete remission (CR) following three to six courses of VP-16 and continued to be in unmaintained CR for >48 months from diagnosis. No major toxicity was noted. Conclusions Etoposide (VP-16), an epipodophyllotoxin known for its usefulness in the treatment of malignancies of the monocyte/macrophage lineage, appears to be an effective treatment for the severe multisystem (disseminated) LCH of childhood and should be strongly considered as front-line therapy for this subgroup of patients with poor prognostic factors.

Differences in iron requirements by concanavalin A-treated and anti- CD3-treated murine splenic lymphocytes

Fe availability is critical for optimal lymphocyte proliferation; however, the minimum required levels are unknown. Such information is valuable when assessing in vitro immune responses in Fe-deficient subjects, because serum (Fe) added to the culture medium may replete lymphocytes. To address this issue, splenic lymphocytes obtained from seventeen 3-month-old C57BL/6 mice were incubated without and with 1 mg/l concanavalin A or 50 microg/l anti-CD3 antibody in media that contained between 0.113 and 9.74 micromol Fe/l. Fe was provided by either fetal calf serum (FCS, 0-100 ml/l), newborn calf serum (NBCS, 0-100 ml/l), or NBCS (10 ml/l) plus ferric ammonium citrate. As expected, the rate of DNA synthesis increased with Fe levels (P<0.01). Maximum DNA synthesis was obtained with 2.26 micromol Fe/l (50 ml FCS/l) for concanavalin A and 0.895 micromol/l (20 ml FCS/l) for anti-CD3-treated cells. In serum-free media (0.113 micromol Fe/l), the proliferative responses to concanavalin A were below the background, while they rose 5.5-fold in anti-CD3-treated cells (P<0.05). In apotransferrin-supplemented media (0.13 micromol Fe/l), the proliferative responses to concanavalin A and anti-CD3 antibody were 18.6 and 71 %, respectively, of that obtained with 4.66 micromol Fe/l (100 ml FCS/l). Interleukin 2 secretion also followed the same trend as lymphocyte proliferation. Since differences between both mitogens persisted after FCS was substituted with NBCS, we can rule out an effect on ribonucleotide reductase activity, or by other serum growth factors. We speculate an Fe effect at an early step of T-cell activation. Data suggest that the minimum Fe concentration required for lymphocyte proliferation varies with the mitogen.

Retinoblastoma: A review

Retinoblastoma is the most common primary ocular malignancy in childhood, usually occurring below the age of five. Recent advances in molecular biology have enabled understanding of retinoblastoma tumorigenesis as well as detecting carriers of the mutant retinoblastoma allele. It is possible now to predict prenatally whether a child carries the retinoblastoma genome. This has enabled early detection of retinoblastoma and improved outcome. Also these children are at risk for mesenchymal tumors in early adult life necessitating life long vigilance. Great strides have been made in the management of retinoblastoma. Introduction of a new pathological staging system and addition of combination chemotherapy for extraocular disease has led to more than 80% long term disease free survivors in this group of patients, who earlier had a very poor outcome. For localized intraocular disease, local treatment seems to be all that is necessary. Newer radiation techniques have helped preserve useful vision and reduced radiation related side effects.

Systemic Lupus Erythematosus and Sickle Cell Disease

A case of 10-year-old girl with an unusual association of sickle cell disease (SCO) with systemic lupus erythematosus (SLE) is presented. The report discusses the clues to the diagnosis of this rare combination with review of relevant literature and highlights the diagnostic dilemma that may arise because of the similarity of symptoms.

Neuroblastoma with opsomyoclonus and Turner syndrome

A neuroblastoma developed in a child with Turner’s syndrome (45, X), the second reported case of such an association. The initial symptom, opsomyoclonus, is associated with a better prognosis, as was evident in our case. The use of computed tomography is valuable in the diagnosis of occult neuroblastoma.

Iron deficiency in lead poisoning

The anemia of lead poisoning is multifactorial. Of 44 hematologically evaluated children with class III or IV lead intoxication, 50 per cent were anemic and 39 per cent were iron deficient. Iron deficiency may play an important role in the anemia of lead poisoning. Early diagnosis and correction of nutritional deficiencies may decrease the metabolic toxicity of lead poisoning.

Hemoglobin CE Disease: A Rare Hemoglobinopathy Detected By Capillary Isoelectric Focusing Techniques (CIE)

Hemoglobin CE Disease: A Rare Hemoglobinopathy Detected By Capillary Isoelectric Focusing Techniques (CIE)

Interleukin-17 Regulates the Recruitment of Stromal Progenitor Cells by Increasing Their Responsiveness To Growth Factors under Serum Deprived Growth Conditions

Interleukin-17 Regulates the Recruitment of Stromal Progenitor Cells by Increasing Their Responsiveness To Growth Factors under Serum Deprived Growth Conditions

Interleukin 17 (IL-17) Regulates the Recruitment of Early Stromal Progenitors into Colony Formation

Interleukin 17 (IL-17) Regulates the Recruitment of Early Stromal Progenitors into Colony Formation