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Biomedical subjects

R M Filler

Publications and source records attributed to R M Filler.

At least 19 recordsLinked to original sources

Parenteral feeding in the management of children with cancer.

Total parenteral nutrition TPN has been used to treat or prevent malnutrition in 65 children with a variety of solid tumors and leukemia in the past 7 years. TPN was used in 58 patients with gastrointestinal complications of surgery, chemotherapy, or radiation; in 2 patients for preoperative correction of malnutrition; and in 8 who were entered into a prospective study. During TPN, general nutrition and appearance improved in all patients. Weight gain was noted in most. Despite gastrointestinal complications, which usually require the interruption of chemotherapy and radiation, treatment could be continued at full dose in 31 children with nutritional support by TPN. TPN was discontinued in 6 patients when blood cultures became positive. Sepsis was treated successfully by removal of the central venous catheter in all 6 and administration of antibiotics in 3. TPN appears to be safe and effective means of combating malnutrition which may occur with cancer and its therapy.

Adolescent

Prognostic factors in omphalocele and gastroschisis.

Size of the abdominal wall defect, viscera exposed or herniated, birth weight, associated medical conditions and congenital anomalies, mode and complications of treatment, and the use of total parenteral alimentation was reviewed in 79 cases of omphalocele and 44 cases of gastroschisis treated in the past 10 yr. Sixty-seven percent of infants with omphalocele and 73% of those with gastroschisis survived. In omphalocele, the most important factors affecting mortality were the presence of other associated abnormalities and low birth weight. The size of the abdominal wall defect, the viscera herniated, and the mode of treatment did not appear to affect mortality. In gastroschisis, the size of the abdominal defect, birth weight, viscera exposed or herniated, and associated anomalies were not significant factors affecting mortality. Mortality was usually secondary to intestinal or wound complications. Statistical analysis could not prove that primary repair resulted in greater survival than the use of a silon pouch, but analysis of complications clearly indicates that the former method is preferable and that silon pouch should be reserved for cases in which primary repair is not possible.

Abdominal Muscles

The role of radiation therapy in the treatment of soft tissue sarcomas of childhood.

The treatment of soft tissue sarcomas in children at the Joint Center for Radiation Therapy, Children's Hospital Medical Center, and the Sidney Farber Cancer Institute from 1970 to 1976 has been reviewed. Twenty-seven patients were diagnosed with rhabdomyosarcoma, and twenty patients were diagnosed with soft tissue sarcomas of other histologies. An aggressive, combined modality therapeutic approach was applied in the treatment of all patients with emphasis placed on conservation of function. Of irradiated patients, local control was achieved in 96% of those with rhabdomyosarcoma and 85% in other sarcomas. Cumulative relapse-free survival (actuarial) at 5 years is projected at 65% for the rhabdomyosarcoma patients and at 63% for the other sarcoma patients. Although there were differences in chemotherapy regimens (vincristine, actinomycin-D and cyclophosphamide for rhabdomyosarcoma and adriamycin and DTIC for other soft tissue sarcomas), the surgical and radiation therapeutic approaches are similar for both groups. The high probability of local control using function-conserving surgery and high dose radiation therapy supports this emerging approach. Improvements in survival will require better control of metastatic disease.

Adolescent

Plication of the diaphragm for symptomatic phrenic nerve paralysis.

Paralysis of the diaphragm in infants may produce severe respiratory difficulty because of the paradoxic motion of the affected diaphragm and shift of a mobile mediastinum to the contralateral side. Six infants with diaphragmatic paralysis and severe respiratory distress underwent plication of the diaphragm by a simple technique. Five of the six infants had significant improvement in respiratory effort and were ultimately weaned from ventilatory support. One patient with bilateral paralysis had only minimal improvement. Diaphragmatic function returned in two patients after plication. Plication of the diaphragm is a safe and useful procedure to improve ventilation in infants with a paralyzed diaphragm. Since this technique does not prevent return of diaphragmatic function, it should be employed prior to the development of sequelae of prolonged assisted ventilation and sooner if the phrenic nerve is permanently injured.

Diaphragm

The management of testicular tumors in children.

Of 43 testicular tumors in children seen at the Children's Hospital Medical Center and Sidney Farber Cancer Institute 33 were malignant. These tumors occurred primarily in young children. Any solid scrotal mass in an infant must be considered malignant until proved otherwise. Primary emphasis is placed on the 20 cases of embryonal cell carcinoma. Of 9 patients who underwent only orchiectomy 5 have died, whereas all 11 patients who received combination therapy have survived. We recommend radical inguinal orchiectomy, extended unilateral retroperitoneal lymphadenectomy and adjuvant chemotherapy in repeated courses for 2 years. Radiotherapy is withheld if the lymphadenectomy is negative.

Castration

Hypoglycemia of infancy and nesidioblastosis. Studies with somatostatin.

We treated a two-month-old infant with servere intractable hypoglycemia and nesidioblastosis with continuous glucose infusions (0.75 g per kilogram per hour) via a central venous catheter. Preprandial glucose levels on this regimen were 37+/-2 mg per deciliter (+/-S.E.M.). Basal serum insulin levels were within normal fasting levels for this age group but inappropriately elevated for the blood glucose levels. The beta cells were exquisitely sensitive to infusions of synthetic cyclic somatostatin, with a dose-dependent rise in blood glucose and concomitant suppression of serum insulin levels. There was only minimal suppression of plasma glucagon levels. Single subcutaneous injections of 50 microng of protamine zinc somatostatin raised preprandial blood glucose levels to 83+/-3 mg per deciliter for four to five days although preprandial hormone levels were unchanged. These findings indicate that hypoglycemia of infancy is a hyperinsulin state with abnormal basal regulation of insulin secretion.

Adenoma, Islet Cell

The role of radiation therapy in localized resectable intestinal non-Hodgkin's lymphoma in children.

Forty children with localized resectable intestinal non-Hodgkin's lymphoma were seen between 1948 and 1974. Survival was related to the extent of disease at presentation and to therapy. No deaths occurred after 15 months and no recurrences occurred after 13 months after diagnosis. Six of eight stage IE patients (75%) and nine of 29 stage IIE patients (31%) have survived a minimum of 2 years; one of the three stage IVE patients has survived 17 years. Unfavorable prognostic findings at surgery were serosal involvement, presence of tumor at the surgical margins, mesenteric nodal involvement, and the presence of abdominal fluid or blood. Paraortic nodal involvement or multiple primary foci were universally fatal. Eight of 11 patients (73%) treated with surgery and whole abdominal irradiation (greater than or equal 2000 rad) with or without chemotherapy have survived. Seven of 18 patients (39%) treated with surgery and low dose chemotherapy have survived. One of seven patients treated with surgery and localized or low dose radiation therapy with or without chemotherapy has survived. Four patients treated with surgery alone died. Bone marrow and central nervous system involvement occurred after previous disease relapse elsewhere.

Adolescent

The increasing importance of radiation therapy in the improved prognosis of children with Wilms' tumor.

Treatment results for 32 children with Wilms' tumor seen at the JCRT, CHMC and SFCI from January 1, 1971, through December 31, 1975, are presented. Indications for and technical details of irridation are discussed. The uniform efficacy of irradiation in preventing local relapse in all patients presenting with unilateral disease despite tumor spill in nine is stressed. Actuarial survival of nearly 80% of all patients seen and followed for more than 2 years has been achieved. Eight of 14 patients with documented pulmonary relapse have been controlled with irradiation, chemotherapy and surgery. The importance of an aggressive multidisciplinary approach to this tumor is stressed.

Child

Parenteral nutritional support in children with cancer.

Acute and chronic starvation is often associated with childhood cancer. Total parenteral nutrition (TPN) with 20% glucose and 3.0% amino acids, and minerals and vitamins was instituted to treat or prevent malnutrition in 41 children with cancer, ages three months to 18 years. TPN was required for anorexia, vomiting and diarrhea associated with anti-cancer therapy in 33 patients for intestinal complications or surgery in nine, and for preoperative correction of malnutrition in two. During TPN, general nutrition and appearance improved in all patients. Weight gain was noted in most. Despite gastrointestinal complications which usually require the interruption of chemotherapy and irradiation, in 21 children treatment could be continued at full dose with nutritional support by TPN. TPN was discontinued in six patients when blood cultures became positive. Sepsis was treated successfully by removal of the central venous catheter in all six and administration of antibiotics in three. No metabolic complications were noted. TPN appears to be a safe and effective means of combating the malnutrition which may occur with cancer and its therapy.

Adolescent

Preliminary results of aggressive multimodality therapy for metastatic osteosarcoma.

Ten patients with metastatic osteosarcoma were treated at the Joint Center for Radiation Therapy, the Sidney Farber Cancer Institute, and the Children's Hospital Medical Center from 1973 to 1976. Patients were treated with an aggressive multimodality approach with included surgery, chemotherapy, and radiation therapy. Three of 10 patients are alive with no evidence of disease, five are alive, with disease, and two are dead of disease. The median survival is 12+ months. Local control data for radiation combined with high dose methotrexate in metastatic osteosarcoma is shown.

Adolescent

Multidisciplinary treatment for childhood sarcoma.

Surgical extirpation of the primary tumor has traditionally been utilized as initial treatment for sarcomas in children. The present report, however, demonstrates that sarcomas are optimally treated by means of a coordianted multidisciplinary approach. The latter offers the potential for achieving improved survival and preservation of organs and limbs, particularly for structures of the head and neck, for extremities, and in the genitourinary system.

Adolescent

Prognosis for children with neuroblastoma presenting with paralysis.

Since 1947, we have treated 19 children with neuroblastoma whose first symptoms were paralysis or weakness of an extremity, and/or incontinence due to tumor in the spinal canal. In 18 patients, the spine tumor was part of a dumbbell tumor which was present in the adjacent paravertebral area and in one, no extraspinal tumor was found. Aggressive treatment was employed for all. In 17 children, the intraspinal tumor was treated by laminectomy and irradiation with and without chemotherapy. Radiation and chemotherapy were used for two. The extraspinal tumor was excised totally in six and partially in six. All 12 children received postoperative radiation and chemotherapy. In 6 children, the extraspinal tumor was treated only with radiation and chemotherapy. Nine of 19 children are alive without evidence of neuroblastoma. Thirteen patients showed either partial (6) or full (7) neurologic recovery. Survival was related to the child's age at diagnosis and the extent of disease. While 8 of 9 children under 1 yr of age survived, only 1 of 10 children over 1 yr survived. None of the 5 children with Stage IV disease at diagnosis could be saved. The degree and frequency of neurologic recovery were greatest in children whose neurologic symptoms had been present the shortest times and were equal among those who survived and those who died. The outlook for children who became paralyzed by neuroblastoma is not hopeless; therapy aimed at saving life or neurologic function is both worthwhile and rewarding.

Age Factors

High-dose methotrexate with citrovorum factor in osteogenic sarcoma--progress report II.

Vincristine (VCR), high-dose methotrexate (MTX), and citrovorum factor (CF) were administered to 12 patients with classic osteogenic sarcoma with local control. Seven patients (58%) are free of pulmonary metastases for 2+-31/2+ years. With a second adjuvant program incorporating adriamycin (VCR, MTX, CF, and adriamycin), 16 of 20 patients are free of pulmonary metastases for 6+-18+ months. Five patients who developed pulmonary metastases were rendered free of disease by surgical resection. The VCR, MTX, and CF program was also administered at weekly intervals to eight patients with pulmonary metastases or unresected primary lesions. Two complete and one partial response were obtained in four patients with pulmonary metastases and three complete and one partial response were obtained in patients with primary lesions. This program was also administered in combination with radiation therapy to four patients who relapsed on conventional VCR, MTX, CF, and adriamycin therapy following surgical resection of pulmonary metastases. They remain free of recurrent disease for 2+-14+ months. There was no alteration in the incidence of toxicity when compared to earlier investigations. The results indicate that the VCR, MTX, and CF program has had a major impact on the management of osteogenic sarcoma.

Doxorubicin

Life-threatening anoxic spells caused by tracheal compression after repair of esophageal atresia: correction by surgery.

Three infants who developed anoxic spells 2, 5, and 20 mo following repair of esophageal atresia developed apneic spells during or within a few minutes of feeding. These episodes began with stridor and cyanosis; when severe, they progressed to apnea and loss of consciousness. Mouth-to-mouth resuscitation was often necessary. Investigations failed to detect esophageal obstruction and/or a recurrent tracheoesophageal fistula. No neurologic or cardiac abnormalities were found. The cause was compression of a 1- to 3-cm segment of trachea anteriorly by a vascular structure and posteriorly by a dilated esophagus that emptied slowly because of poor motility. Endoscopy confirmed the x-ray findings. The aortic arch and innominate artery were suspended to the sternum anteriorly, which relieved the apneic spells in all patients.

Apnea

Liver tumors in children in the particular reference to hepatoblastoma and hepatocellular carcinoma: American Academy of Pediatrics Surgical Section Survey--1974.

A review of 227 cases of hepatoblastoma, hepatic cell carcinoma in children seen in the United States over a 10-yr period is presented. Both tumors were seen most commonly in infancy, but the hepatocellular carcinoma shows a second peak of incidence around puberty. Males predominated in both diseases more so in hepatoblastoma. Presenting symptoms in both diseases were very similar, most commonly an upper abdominal mass or abdominal enlargement associated with anorexia and weight loss. In the preoperative evaluation the presence of alpha-feto protein was one of the most helpful diagnostic tests. Disturbances of liver function were usually mild but were more marked in those children with hepatocellular carcinoma. Preoperative x-rays were abnormal in a large percentage of cases with the hepatic arteriogram and vena cavagram being the most useful diagnostic x-rays for liver tumors. Liver scans were positive for liver tumor in 95% of the children when this test was carried out. The follow-up for these patients ranged from 2 to 10 yr. The size of the primary tumor did not appear to correlate with survival but bilateral location of the tumor, 33% in hepatoblastoma and 45% in hepatocellular carcinoma, made many of these tumors inoperable. Multicentric tumors were also found in a large number of patients, being more common in hepatocellular carcinoma. There was a high rate of local recurrence or local extension after operation in both diseases, and metastatic spread was similar being most common to the lungs and abdomen. A wide variety of surgical procedures were carried out in these patients from biopsy only to extended hepatic lobectomy. When incomplete excision or biopsy only was carried out no patient survived in either group. Among the hepatoblastoma patients, 45 of 78 patients who had complete excision are surviving. In the hepatocellular carcinoma patients where the operability rate was much lower 12 of 33 patients are surviving when tumor was completely excised. Complications were frequent, the most common being excessive blood loss at operation. There were eight operative deaths and 17 postoperative deaths in the combined group. There was no evidence that radiation therapy or chemotherapy controlled disease which could not be completely excised surgically. The only direct evidence of a favorable effect of radiation and chemotherapy were three cases of hepatoblastoma in which the tumor changed from inoperable to operable by a combination of radiation therapy and multiple drug chemotherapy. Both tumors are highly malignant, and 90% of the children who died of hepatoblastoma died within 12 mo of diagnosis. In the hepatocellular carcinoma 80% of the deaths occurred within 1 yr of diagnosis. At this time it seems that operative excision offers the only chance of cure in children with these tumors and cure rates of 60% can be expected with hepatoblastoma and 33% in hepatocellular carcinoma if the tumor can be completely excised.

Adolescent