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Biomedical subjects

S Jayabose

Publications and source records attributed to S Jayabose.

At least 37 records · Page 2Linked to original sources

Gastric ganglioneuroblastoma: a rare finding in an infant with multifocal ganglioneuroblastoma.

PURPOSE: This report describes a female infant with stage 4 multifocal ganglioneuroblastoma with gastric involvement. PATIENT: The patient had a right cervical tumor, a left posterior mediastinal tumor, bilateral adrenal tumors, and bony and bone marrow metastases. The tumor cells were diploid and lacked N-myc gene amplification. The gastric involvement, which did not produce clinical symptoms, was only detected by meticulous exploration during laparotomy. RESULTS: Our patient achieved only a partial response to alternating cycles of cyclophosphamide, vincristine, and adriamycin; and etoposide and cisplatin. She currently has stable, unresectable disease with elevated catecholamines. CONCLUSIONS: Multifocal ganglioneuroblastomas may arise from either neuroblastic rests or aberrant deposits of neuroblasts. The latter mechanism may have accounted for our patient's gastric tumor. Patients with multifocal ganglioneuroblastomas warrant meticulous radiographic and surgical evaluation to completely document the full extent of disease, and to ensure appropriate staging and therapy.

Female↗

Recurrent benign intracranial hypertension due to iron deficiency anemia. Case report and review of the literature.

PURPOSE: Iron deficiency anemia (IDA) causes benign intracranial hypertension (BIH). PATIENTS AND METHODS: A case of an 11-year-old girl with severe IDA and benign intracranial hypertension is presented. RESULTS: Treatment of iron deficiency resulted in the resolution of BIH. Recurrence of BIH is observed with the recurrence of IDA. Chronic intermittent blood loss from the intestinal polyps was the etiology of recurrent IDA. CONCLUSIONS: A thorough search to determine the etiology of IDA is essential for proper treatment. Although BIH is a rare complication of IDA, its association with IDA should be recognized for prompt treatment before an extensive and costly workup for BIH is done.

Adolescent↗

Recombinant human granulocyte colony stimulating factor in cyclic neutropenia: use of a new 3-day-a-week regimen.

BACKGROUND: G-CSF has been shown to be beneficial in cyclic neutropenia when given as a daily subcutaneous injection. We investigated the usefulness of a new three-day-a-week regimen. METHODS: A ten year old boy with cyclic neutropenia was initially treated with G-CSF 7 micrograms/kg given on alternate days for seven months. He was then placed on the same dose, given three days a week. The effectiveness of these regimens were assessed by serial CBCs and by the frequency and duration of the symptoms. RESULTS: The mean absolute neutrophil count (ANC) increased from 1282 before therapy to 11,718/microliters on alternate day regimen and 7716/microliters on three-day-a-week regimen. The nadir ANC improved from 30/microliters before therapy to 546/microliters and 198/microliters on treatment. The duration and frequency of mouth sores were significantly less on therapy, and there was an estimated cost savings of $23,826/year on three-day-a-week regimen compared to a daily regimen. CONCLUSION: The three-day-a-week G-CSF regimen is clinically effective and cost saving in the treatment of cyclic neutropenia and should be studied in a larger cohort of patients.

Child↗

Transient erythroblastopenia of the newborn caused by human parvovirus.

PURPOSE: We describe a newborn baby boy with anemia in whom human parvovirus (HPV) antibodies were detected in his serum. PATIENTS AND METHODS: Severe reticulocytopenia and anemia lasted until day 11 after his birth. Thereafter reticulocytosis was observed. Work-ups for immune hemolytic anemia, fetomaternal hemorrhage, placental hemorrhage, intrauterine infections were negative by clinical and laboratory analysis. HPV-specific IgM and IgG were positive on both the baby's and the maternal serum. RESULTS: Despite severe anemia, the baby remained clinically stable. His hemoglobin levels steadily increased along with ongoing reticulocytosis. He is now 14 months old with normal hemoglobin. CONCLUSIONS: Detection of HPV antibodies indicated its causal relation to the development of congenital pure red cell aplasia. The possibility of HPV infection should be considered in the assessment of anemia of the newborn.

Antibodies, Viral↗

Transfusion therapy for severe anemia.

PURPOSE: We evaluated the safety and efficacy of a new transfusion regimen for children with severe anemia. PATIENTS AND METHODS: Twenty-two consecutive patients with severe anemia (hemoglobin < 5 g/dl) of gradual onset requiring transfusion of packed red blood cells (PRBC) were studied. The transfusion regimen consisted of continuous infusion of PRBC at the rate of 2 cc/kg/h until the desired volume was given. Throughout the transfusion, the patients were closely monitored for any clinical signs of heart failure. The rise in hematocrit per 1 cc of PRBC/kg transfused was computed for each patient. RESULTS: No patient developed any signs of cardiac failure or increase in the heart rate during or after the completion of transfusion. All patients had a decrease in the heart rate by the completion of transfusion. The mean decrease in the heart rate was 28% of the pretransfusion heart rate (range 12-44%). Excluding the four patients with sickle cell anemia, the remaining 18 patients had a mean increase in the hematocrit of 1.04% per 1 cc/kg of PRBC (range 0.85-1.28). CONCLUSION: We conclude that for children with severe anemia of gradual onset requiring transfusion therapy, continuous transfusion of PRBC at the rate of 2 cc/kg/h is a safe and effective regimen resulting in an increase in the hematocrit of approximately 1% for each 1 cc/kg of PRBC transfused in all patients, except patients with sickle cell anemia.

Acute Disease↗

Home chemotherapy for children with cancer.

A program was established in which the parents of children with cancer were trained to administer intravenous chemotherapy to the children in their homes. The main objectives of this program (Home Intravenous Chemotherapy by Parents [HICP]) were to improve the quality of life for children with cancer and their parents and to decrease the cost of medical care for these children by decreasing the number of clinic visits and hospital stays intended solely for the administration of chemotherapy. Twenty-four months of experience with this program indicates that with a close communication network and a good working relationship among a home care organization (HCO), the parents of the patient, a pediatric oncology nurse, and an attending pediatric oncologist, many chemotherapeutic agents can be safely administered to these children by their parents in their homes. The patients and their parents are enthusiastic about this program because of the valuable time saved and the increased participation in care. In addition, this program greatly decreased the cost of medical care for these children.

Adolescent↗

Coombs-positive hemolytic anemia preceding ulcerative proctitis.

A 12-year-old girl is described who developed rectal bleeding 5 months after being diagnosed as having a Coombs-positive hemolytic anemia. Colonoscopy showed that the rectal bleeding was due to ulcerative proctitis. This is the first case report of Coombs-positive hemolytic anemia preceding the onset of ulcerative proctitis in a child.

Anemia, Hemolytic, Autoimmune↗

Combined occurrence of chyloperitoneum and chylothorax after surgery and chemotherapy for Wilms' tumor.

Chyloperitoneum is an extremely rare complication of abdominal surgery in children and a combined occurrence of chylothorax and chyloperitoneum after abdominal surgery has never been reported in children. Chylous ascites usually occurs as a result of operative trauma to the thoracic duct, cisterna chyli, or its tributaries. About one third of all patients with chylous ascites after retroperitoneal lymph node dissection also develop secondary chylothorax. Diaphragmatic defects have been shown to be responsible for the occurrence of chylothorax secondary to chyloperitoneum. Congenital diaphragmatic weakness may result in evagination of the peritoneum causing diaphragmatic blebs, the rupture of which results in the movement of the peritoneal fluid into the pleural cavity. In the authors' patient, the rent in the diaphragm that occurred during surgery was probably responsible for the chylothorax. The role of chemotherapy, if any, in the pathophysiology of this complication is unknown. Total parenteral nutrition (TPN) is a simple and effective treatment for postoperative chylous effusions. Surgical treatments such as abdominal exploration for the repair of leaking lymphatics and peritoneovenous shunt should be reserved for patients who fail TPN.

Chylothorax↗

Hypercalcemia in childhood renal tumors.

Hypercalcemia is an uncommon complication of childhood renal tumors. It is exclusively seen in infants 6 months of age or younger with malignant rhabdoid tumor of the kidney (MRTK) or congenital mesoblastic nephroma (CMN). Secretion of parathormone or prostaglandin E2 by the tumor cells is responsible for the hypercalcemia in most of these patients. Bone metastasis has been notably absent in these patients, and the hypercalcemia completely resolves with the removal of the tumor. Hypercalcemia in itself probably does not have any prognostic significance; however, it may serve as a tumor marker in some patients. Early recognition and effective management of this complication may prevent the acute life-threatening as well as the longstanding complications of this serious metabolic disorder.

Female↗

Presacral mesenchymoma: a case report.

A 4-year-old girl with a presacral benign mesenchymoma is reported. The nature of the tumor was not recognized preoperatively or intraoperatively. There was evidence of pericapsular infiltration of the soft tissues of the pelvis but not signs of malignancy. A follow-up examination after 3 months indicates that the patient has no clinical evidence of recurrence. Review of the English literature shows the rarity of benign mesenchymoma in childhood and the lack of reports of its occurrence in the pelvis.

Child, Preschool↗

Use of intravenous methylprednisolone in acute idiopathic thrombocytopenic purpura.

Twenty newly diagnosed children with acute idiopathic thrombocytopenic purpura (ITP) with a platelet count (PC) of less than 20,000 were treated with intravenous methyl prednisolone (IVMP) 5 mg/kg/day in four divided doses until the platelet count increased to 50,000/mm3. They were then placed on oral prednisone at conventional doses for a total of three weeks of steroid therapy. The platelet counts increased by an average of 55,000/mm3 within 48 h of treatment. Within 72 h (day 3) of treatment all patients achieved a PC of greater than 20,000/mm3. By day 5, all 20 patients achieved a PC of greater than 50,000. IVMP is highly effective in rapidly raising the PC in acute ITP above the critical level of 20,000, thereby reducing the duration of risk for intracranial hemorrhage (ICH).

Adolescent↗

Intravenous anesthesia with propofol for painful procedures in children with cancer.

OBJECTIVE: To study the safety and efficacy of propofol-based intravenous anesthesia in children with cancer undergoing painful procedures. METHODS: This study is a retrospective analysis of data collected from 52 consecutive children who underwent 335 procedures using propofol anesthesia. These data were routinely collected in all patients: time to induction, duration of the procedure, time to recover, and the doses of the drugs used. Monitoring with electrocardiography and pulse oximetry was continuous during the procedure; blood pressures were recorded before and after the procedure and every 5 to 10 minutes during the procedure. The patients received one of these four propofol-based intravenous regimens according to the anesthesiologist's preference: propofol only; propofol plus fentanyl; propofol plus midazolam; or propofol, fentanyl, and midazolam. The efficacy of sedation was rated by this scoring system: 3 = no movement during procedure; 2 = minimal movement that did not interfere with the procedure; 1 = moderate movement requiring physical restraint to complete the procedure. RESULTS: There were six episodes of mild hypoxia (oxygen saturation 85%-94%) and one episode of laryngospasm. None required intubation. Two patients had agitation and one patient had emesis during the postrecovery phase. There was no difference in the efficacy of sedation between the four regimens. Patients receiving the combination of propofol, fentanyl, and midazolam received the least amount of propofol and required the least time to recover. There were no life-threatening complications. CONCLUSIONS: Propofol-based anesthesia, when administered by an anesthesiologist in a controlled setting, is safe and effective for performing painful procedures in children with cancer.

Ambulatory Care↗

Solid variant of aneurysmal bone cyst in a patient with cyclic neutropenia.

A 16-year-old female with cyclic neutropenia (CN) was incidentally found to have a thoracic vertebral mass during the preoperative work-up for maxillary sinus surgery. An exploratory thoracotomy revealed a very vascular tumor involving T-10 and T-11 vertebral bodies. Gross total resection of the tumor was achieved. Pathology revealed a solid variant of aneurysmal bone cyst. This is a rare benign neoplasm of bone more commonly seen in the mandible and facial bones as well as involving the small tubular bones of the hands and feet. Because of its rarity, location, and an unknown association with CN, we found this case worthwhile to publish.

Adolescent↗