A Correlation of the Platelet Count with D-Dimer Levels as an Indicator for Component Therapy in Children with Dengue Hemorrhagic Fever.

Dengue Fever (DF) may evolve into two life threatening forms-Dengue Hemorrhagic Fever (DHF) and Dengue Shock Syndrome (DSS). DHF is associated with increased vascular permeability and plasma leakage causing thrombocytopenia and loss of clotting factors into the third space and may result in bleeding initially due to thrombocytopenia and later due to disseminated intravascular coagulation (DIC), often as a terminal event. Prompt recognition and treatment of minor bleeds in DF children with incipient DIC with component therapy may be associated with improved survival while failure to do so is usually catastrophic. A sensitive marker for early DIC is the presence of D-dimer (DD) in the blood. To determine the correlation between the severity of thrombocytopenia and early DIC in children with DHF. The impact of additional factors like age and shock will also be evaluated. Case control prospective study of 60 DHF sero -positive children (1-15 years) with thrombocytopenia. After clinical evaluation they were divided into two equal groups based on the degree of thrombocytopenia (more than/less than 30,000/mm3). PT/APTT and DD levels were estimated in all children of both groups and statistical correlation was done. There was no significant difference in the DD levels between the two groups. However, children in either group, presenting with clinical features of shock and thrombocytopenia had significantly higher DD levels. Empirical component therapy in children with DHF based purely on their low platelet counts may not be justified. However, in DHF children with thrombocytopenia and features of shock, aggressive component therapy may prevent subsequent bleeding and may be justified.

Looking beyond the obvious: an unusual cause of iron deficiency anaemia.

Iron deficiency anaemia (IDA) is the most common haematological disorder seen in childhood. While poor intake of iron is the most common cause of IDA, a diligent search must be made to exclude other causes. We present a 5 year old girl who had recurrent hospital admissions for chest infection, radiographic evidence of bilateral pulmonary infiltrates and severe IDA. When she failed to respond to repeated courses of antibiotics, anti tubercular and parenteral and oral iron therapy, a lung biopsy was done which revealed blood and hemosiderophages within the alveoli. Further investigations were suggestive of SLE (dsDNA positivity). A diagnosis of SLE induced alveolar haemorrhage was made and therapy with glucocorticoids was initiated. She has responded well to therapy.

Cytopenia and Bone Marrow Dysplasia in a Case of Wilson's Disease.

We describe a sixteen year old with Wilson's disease on copper chelation and subsequent high dose oral zinc who developed severe anemia and neutropenia. Bone marrow aspirate done to evaluate the cause of bicytopenia revealed trilineage dysplasia. Correlating the clinical context with bone marrow and biochemical parameters, copper deficiency was suspected and he was given a trial of therapy, following which the hematological parameters improved. This case highlights hypocupremia as a reversible cause of bone marrow dysplasia in patients with Wilson's disease on chelation, where serum copper levels are not useful in the diagnosis. We also believe that monitoring of the blood counts in patients on copper chelation may provide a clue to impending copper deficiency.

Nutritional assessment of children with hematological malignancies and their subsequent tolerance to chemotherapy.

BACKGROUND: Our research goals were to assess the prevalence of malnutrition in children with cancer, observe malnutrition's effect on tolerance to chemotherapy, and establish malnutrition at onset as one of the prognostic factors in children with hematological malignancies. METHODS: This prospective study examined children ages 1-15 years with a confirmed diagnosis of acute lymphoblastic leukemia (ALL) and non-Hodgkin lymphoma. Each child was subjected to a detailed history, anthropometric examination, and laboratory investigations. Based on the anthropometric measurements that used weight-for-age Z scores, we divided the children into 4 groups: group 1, without malnutrition; group 2, mild malnutrition; group 3, moderate malnutrition; and group 4, severe malnutrition. We analyzed data for each group regarding the behavior of blood indices, the quantum of hematological support, bone marrow remission status on day 28, adherence to protocol schedules, and complications in the first 4 months of intensive chemotherapy. RESULTS: Of the 34 patients in the study (mean age, 7.1 years; male:female ratio, 1.6:1), 79% had deficient calorie intake and 74% had deficient protein intake. Packed cell requirements and complications were significantly higher in malnourished children, whereas the requirement for platelet transfusions was statistically insignificant. Also, 50%, 40%, 38%, and 44% of children in groups 1, 2, 3, and 4, respectively, completed chemotherapy within the specified time period. At the end of the induction phase, 92%, 60%, 87%, and 77% of the patients in groups 1, 2, 3, and 4, respectively, achieved bone marrow remission. No deaths occurred in group 1; 1 death each occurred in groups 3 and 4, and 2 in group 2. When these deaths were extrapolated to the weight/height ratio (acute malnutrition), we found that all occurred in children with malnutrition, a statistically significant result. CONCLUSIONS: Malnutrition is widely prevalent in children with ALL in India and has a significant bearing on the occurrence of life-threatening complications and short-term outcomes in these children. Malnutrition is also a significant factor influencing treatment planning and therapeutic decisions.

Myelodysplastic syndromes in children: where are we today?

Myelodysplastic syndromes (MDSs) and other myeloproliferative disorders (MPDs) are rare entities, particularly in children. The latest classification of MPDs separates them into 3 major groups: MDS, juvenile myelomonocytic leukemia, and Down syndrome-associated myeloid leukemia.Although the blast count plays a central role in differentiating leukemias from MDSs, it is by no means sacrosanct; biological features as well as disease characteristics are also important considerations for diagnosis. Genetic alterations in MDSs have increasingly been noted in a majority of patients and when detected make diagnosis easy. Hematopoietic stem cell transplant is the treatment of choice, while newer agents have shown promise in adults and are presently in the advanced stages of clinical trials.

Palmar dermatoglyphics in children with acute lymphoblastic leukemia--a preliminary investigation.

BACKGROUND: Acute lymphoblastic leukemia (ALL) is a malignant disease of as yet an unknown origin. Researchers in the past have studied the association between dermatoglyphic features in the hand and congenital diseases and diseases with genetic influences. OBJECTIVE: The present research is intended to study the association between acute lymphoblastic leukemia (ALL) and palmar dermatoglyphic characteristics to assess the value of dermatoglyphics as a screening tool to detect leukemia in high risk groups. METHODS: Case-control study conducted at the department of Pediatrics, KMC Attavara, a tertiary care teaching hospital of Kasturba Medical College, Mangalore (Manipal University), India during 2006. Twenty-four children suffering from acute lymphoblastic leukemia were included in the study. The dermatoglyhic features (ab- ridge count, atd, tda and dat angles) of their palm prints were compared with the age and sex matched controls. RESULTS: The present research indicates an association between dermatoglyphic features and ALL. The mean ab-ridge count, and the mean atd - angle were observed to be higher in cases while the mean tda - angle was found to be lower in cases than controls. CONCLUSIONS: The findings of the present research are suggestive of a possible trend and an association of dermatoglyphic features with children suffering from ALL. Similar studies can be useful in rare forensic case work where the association of dermatoglyphic features with certain diseases is to be explored.

Study of finger print patterns in children with acute lymphoblastic leukemia.

Acute lymphoblastic leukemia (ALL) is a malignant disease of an unknown origin. The present case control study was intended to compare the finger print patterns between the children diagnosed with ALL and a control group. A total of 24 children with established ALL were included in the study. Fingerprints of the affected children were analyzed in both hands and compared with the finger print patterns of 24 age and sex matched controls. The study indicated an increase in frequency of whorls and a decreased frequency of loops in children suffering from ALL. Radial loops, double loops, central pocket loops and tented arches are found to be proportionately less frequent in cases than controls. On quantitative analysis of patterns, mean pattern intensity index (PII) was found to be higher in cases than controls; however, a significant overlapping was evident. Most of the cases (n = 10, 41.7%) had a PII of 16-20, while a PII of 11-15 is reported among most children in the control group (n = 16, 66.7%). The findings of the study are suggestive of a possible trend and an association of finger print patterns with children suffering from ALL. The present research emphasizes the significance of the application of forensic science in medicine and pathology.

Significance of C-reactive protein during febrile neutropenia in pediatric malignancies.

Fifty episodes of febrile neutropenia (FN) in 33 children with malignancies were studied to evaluate the usefulness of C-reactive protein (CRP) levels as an indicator of infection, and the efficacy of antibiotic therapy. Nineteen FN episodes occurred in children with documented infection whereas, 9 and 22 episodes occurred with probable infection and fever of unknown origin, respectively. CRP positivity during episodes of documented and probable infection was significantly higher than with febrile episodes of unknown origin. Blood culture was positive in 15 episodes; of these, CRP was positive in 11. CRP declined to normal on 7th day of antibiotic therapy. CRP is a useful indicator of infection in neutropenic children and also in determining the efficacy of antibiotic therapy.