ABSTRACT
BACKGROUND: Protocols for transfusion therapy in transfusion-dependent thalassemia (TDT) children differ among various medical centers. In India, most centers consider only the patient's weight while calculating the volume of packed red blood cells (PRBCs) to be transfused. This study aimed to compare the efficacy of PRBC transfusions of different volumes calculated either by weight or by a formula using weight and pretransfusion hemoglobin of patient and hematocrit of PRBC. STUDY DESIGN AND METHODS: Sixty TDT patients in the age group of 3-9 years were enrolled and randomly allocated to two groups. Group A received PRBC transfusion volume based on the patient's weight, and Group B received PRBC volume calculated using a formula for 6 months. RESULTS: Average pretransfusion hemoglobin in Group A and Group B (9 ± 0.4 vs. 8.9 ± 0.4 g/dl) was not significantly different (p = .353). Although the average number of visits in 6 months was less for Group A compared to Group B (7 ± 1 vs. 8 ± 1; p = .001); the average volume transfused per visit was more (351 ± 78 vs. 287 ± 68 ml; p = .003). The calculated average annual pure red cell requirement of the patients was 178 ml/kg/year for Group A and 154 ml/kg/year for Group B (p = .000). Total donor exposures were significantly lower in Group B than Group A (11 ± 3 vs. 14 ± 3; p = .006). CONCLUSION: The number of donor exposures and annual pure red cell requirement was significantly lower in the formula-based group. Transfusions based on formula are recommended in TDT patients.
Subject(s)
Erythrocyte Transfusion , Hemoglobins , Blood Transfusion/methods , Child , Child, Preschool , Erythrocyte Transfusion/methods , Erythrocytes/chemistry , Hematocrit , Hemoglobins/analysis , HumansABSTRACT
Tropical pulmonary eosinophilia (TPE) is an exaggerated immunological response to filarial antigens mainly seen in adults in endemic areas and is uncommonly reported in children. The diagnosis is frequently delayed as it mimics asthma clinically, while the chest radiograph might show miliary mottling which is often confused with that in pulmonary tuberculosis. A 5-year-old girl presented with episodes of nocturnal cough, wheeze, low grade fever and breathlessness for the previous 4 months and was being treated for asthma. The absolute eosinophil count was markedly raised (53 × 109/L) with prominent bilateral infiltrates on the chest radiograph. Serology for microfilaria and a test for microfilaria antigens were positive and the patient showed a dramatic response to diethylcarbamazine, which confirmed the diagnosis of TPE. TPE is an important differential diagnosis of asthma especially in endemic areas.
Subject(s)
Asthma/pathology , Pulmonary Eosinophilia/diagnosis , Pulmonary Eosinophilia/pathology , Child, Preschool , Diagnosis, Differential , Diethylcarbamazine/administration & dosage , Female , Filaricides/administration & dosage , Humans , Pulmonary Eosinophilia/diagnostic imaging , Radiography, Thoracic , Treatment OutcomeABSTRACT
BACKGROUND: The aim of this study is to understand the epidemiology of tumors in children in our region due to a paucity of studies on the histologic review of the childhood tumors in general and benign tumors in particular. MATERIALS AND METHODS: The records of all the tumors diagnosed histopathologically in children <14 years of age during a period of 8-year (2005-2012) were reviewed. RESULTS: A total of 385 tumors were seen in the age range of 1 month-14 years with 231 (60%) in boys and 154 (40%) in girls. Highest number of cases, 224 (58.18%) were in the age group of 10-14 years. Benign tumors comprised 275 (71.43%) cases while the malignant tumors accounted for 110 (28.57%) cases. In benign tumors, vascular tumors were in majority with 68 cases, while in malignant category bone tumors were most common with 36 cases. CONCLUSIONS: Although the exact incidence rate cannot be provided by this hospital-based study, the information is useful in showing patterns of childhood tumors. We included both benign and malignant tumors, while most of the studies in the past have focused mainly on malignant tumors in children.
Subject(s)
Dermatomyositis/complications , Tachycardia, Ventricular/complications , Amiodarone/therapeutic use , Anti-Arrhythmia Agents/therapeutic use , Child , Dermatomyositis/drug therapy , Dermatomyositis/physiopathology , Fatal Outcome , Humans , Immunosuppressive Agents/therapeutic use , Male , Methotrexate/therapeutic use , Prednisolone/therapeutic use , Recurrence , Tachycardia, Ventricular/drug therapy , Tachycardia, Ventricular/physiopathologyABSTRACT
Microcytic anemia refractory to usual supplementation is an important clue to an alternative diagnosis. Accompanying pulmonary and renal involvement during the disease course suggests a multisystem disease. Small-vessel vasculitis should be suspected in any patient who presents with a multisystem disease that is not caused by an infectious or malignant process. Among these, antineutrophilic cytoplasmic antibodies (ANCA)-associated vasculitis is a distinct subclass involving anti-neutrophil cytoplasmic antibody (ANCA) as the common pathogenesis. Microscopic polyangiitis is a rare form of such vasculitis in children characterized by pulmonary-renal syndrome with pauci-imune rapidly progressive glomerulonephritis and a poor outcome.
Subject(s)
Anemia, Refractory/etiology , Microscopic Polyangiitis/diagnosis , Child , Female , HumansABSTRACT
There is no report of the use of antisnake venom (ASV) in the neonatal age group in literature. We report a 27 days old female neonate who presented with neuroparalytic manifestations of snake bite and was treated successfully with ASV. A total of 50 vials (500 mL) of polyvalent antisnake venom were given as infusion in hourly aliquots of 50 mL, over 72 hours.
Subject(s)
Antivenins/administration & dosage , Snake Bites/drug therapy , Female , Humans , Infant, NewbornABSTRACT
Anti-GQ1b IgG antibody syndrome comprises a wide range of diseases presenting with ophthalmoplegia and ataxia. Anti-GQ1b antibodies have been strongly associated in the literature with Miller Fisher Syndrome, with acute ophthalmoplegia associated with Guillain-Barré syndrome, and with isolated ophthalmoplegia. Acute ophthalmoplegia presents as various combinations of external and internal ophthalmoplegia. Reported here is a novel case of isolated ptosis as a manifestation of ophthalmoplegia. The present finding of bilateral ptosis and areflexia with anti-GQ1b IgG antibody positivity helps confirm the existence of the syndrome. Further research is needed on diagnosis and treatment.
Subject(s)
Autoantibodies/blood , Autoimmune Diseases of the Nervous System/diagnosis , Blepharoptosis/diagnosis , Blepharoptosis/immunology , Gangliosides/immunology , Immunoglobulin G/blood , Ophthalmoplegia/diagnosis , Acute Disease , Ataxia/diagnosis , Autoimmune Diseases of the Nervous System/immunology , Autoimmune Diseases of the Nervous System/therapy , Blepharoptosis/therapy , Child, Preschool , Humans , Immunoglobulins, Intravenous/therapeutic use , Immunologic Factors/therapeutic use , Male , Ophthalmoplegia/immunology , Ophthalmoplegia/therapy , Reflex , Syndrome , Treatment OutcomeABSTRACT
Split-hand/split-foot malformation is a rare limb malformation with median clefts of the hands and feet and aplasia/hypoplasia of the phalanges, metacarpals and metatarsals. When present as an isolated anomaly, it is usually inherited as an autosomal dominant form. We report a case of autosomal recessive inheritance and discuss the antenatal diagnosis, genetic counseling and treatment for the malformation.
