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Biomedical subjects

R C Shamberger

Publications and source records attributed to R C Shamberger.

At least 73 records · Page 4Linked to original sources
PubMed1993

Stage III neuroblastoma over 1 year of age at diagnosis: improved survival with intensive multimodality therapy including multiple alkylating agents.

PURPOSE: A nonrandomized, single-arm trial was conducted to assess the efficacy of multimodality therapy including intensive chemotherapy with multiple alkylating agents in the treatment of children with Evans stage III neuroblastoma older than 1 year at diagnosis. PATIENTS AND METHODS: Twenty-five patients with a median age of 18 months at diagnosis were treated with multimodality therapy including surgery and chemotherapy using either nitrogen mustard (mechlorethamine), doxorubicin, cisplatin, dacarbazine (DTIC), vincristine, and cyclophosphamide (MADDOC) or cisplatin and cyclophosphamide induction followed by maintenance MADDOC (induction MADDOC) protocols. Sixteen of 25 patients also received radiotherapy to the tumor bed and primary lymph nodes. Event-free survival (EFS) was compared with that reported previously in the literature. N-myc amplification was evaluated prospectively and the Shimada classification was evaluated retrospectively as potential prognostic factors. RESULTS: We report a 72% EFS (95% confidence interval +/- 18%) with a median follow-up of 85 months. EFS was significantly worse for patients with tumors demonstrating N-myc amplification (P = .018). Patients classified as favorable according to the Shimada system experienced a significantly better EFS (P = .04), but unfavorable patients still maintained a 60% EFS. CONCLUSION: Intensive multimodality treatment including MADDOC and induction MADDOC chemotherapy provides a very good EFS for children older than 1 year who have stage III neuroblastoma. Children classified as favorable according to the Shimada system have a better prognosis. Patients whose tumors demonstrate N-myc amplification have a poor prognosis despite therapy.

Alkylating Agents↗
PubMed1993

Prenatal detection of neuroblastoma: a ten-year experience from the Dana-Farber Cancer Institute and Children's Hospital.

OBJECTIVES: To assess the relative frequency of, the clinical and pathological correlates in, and the prognosis of the subset of infants with neuroblastoma who were identified initially by prenatal ultrasonography. DESIGN: Retrospective review of all patients with neuroblastoma evaluated between 1982 and 1992. SETTING: Large, urban, tertiary care children's hospital in Boston, Massachusetts. PATIENTS: Eleven infants with neuroblastoma initially detected with prenatal sonograms were identified. RESULTS: Nine patients had adrenal tumors; two had thoracic paraspinal tumors. Typical diagnostic evidence for neuroblastoma including a palpable abdominal mass and elevations in urinary catecholamines were not commonly seen postnatally. These patients had multiple favorable prognostic indicators including low stage of disease (10/11), favorable biological markers including cellular DNA content (5/5) and N-myc oncogene copy number (5/5), and histopathology suggestive for neuroblastoma in situ (7/11). All patients were treated by surgical resection. One patient exhibited progression of disease postoperatively, but demonstrated a complete clinical response to multiagent chemotherapy. Overall survival in our population was excellent with no deaths seen at a mean follow-up of 37 months (range 3 to 120 months). CONCLUSIONS: Patients with neuroblastoma identified by prenatal ultrasonography generally, although not exclusively, follow a clinically favorable course in which surgical resection is curative. Chemotherapy is not indicated unless substantial progression of disease occurs.

Adrenal Gland Neoplasms↗
PubMed1992

Pneumatocele complicating hyperimmunoglobulin E syndrome (Job's Syndrome).

The case of a boy with hyperimmunoglobulin E syndrome or Job's syndrome is presented to demonstrate the occurrence of pneumatoceles in this syndrome as well as their unusual natural history and failure to spontaneously resolve. Surgical resection was required for two complications in this patient, persistent bronchopleural fistula and a pulmonary abscess that destroyed one lung and required pneumonectomy. Pathologic examination of the specimens demonstrated the wall of the cysts consisted of granulation tissue with chronic active inflammation surrounded by infarcted pulmonary parenchyma with coagulative necrosis. The mechanism responsible for increased immunoglobulin E production in this syndrome is unknown, as is the manner in which elevated immunoglobulin E levels impair normal immune function.

Aspergillosis↗
PubMed1992

The role of surgery in abdominal Burkitt's lymphoma.

The abdomen is the most frequent site of involvement in nonendemic Burkitt's lymphoma (small noncleaved cell). Some authors have proposed a role for extensive surgical resection or "second look" laparotomy in these patients. We retrospectively reviewed our series of 53 patients with Burkitt's lymphoma (1977 to 1990) to assess the role of surgery in their treatment. Patients were 2.5 to 21 years of age (median, 9.5 years) and 44 were males. The primary site of disease was the abdomen (38), head and neck (12), axilla (1), and bone marrow (2). Twenty-four of the 38 patients with abdominal primaries underwent laparotomy. Twelve of these patients presented with acute abdominal symptoms (right lower quadrant pain or intestinal obstruction) and at exploration underwent resection of the primary tumor. Ten of these 12 patients achieved grossly complete excision of tumor (9 had disease limited to the ileocecal area and adjacent mesentery and one had exophytic tumor adherent to the liver, which was excised). Of note, only 1 of these 12 patients had metastatic disease outside of the abdomen. The remaining 12 patients who underwent laparotomy had an incisional biopsy performed. Of the 14 patients who did not have a laparotomy, the diagnosis was made by bone marrow biopsy (6), and/or cytology of pleural fluid or ascites (6), lymph node biopsy (1), testicular biopsy (1), tibial biopsy (1), and percutaneous biopsy (1). Murphy staging for these 38 patients was: stage II (10), stage III (19), stage IV (5), and B cell acute lymphoblastic leukemia (ALL) (4). All patients received cyclophosphamide-containing combination chemotherapy regimens and stage III/IV/B cell ALL patients received central nervous system (CNS) prophylaxis.(ABSTRACT TRUNCATED AT 250 WORDS)

Abdominal Neoplasms↗
PubMed1992

Experience with a nurse practitioner program in the surgical department of a children's hospital.

Manpower limitations are occurring in residency programs because of fewer residency positions and increasing governmental regulations. This limitation prompted the development of a program to utilize pediatric nurse practitioners (PNPs) in our Department of Surgery. Two began in September 1989. Their role was similar to that of the junior resident and included collaborative management of patients, admission evaluations, discharges, and a variety of procedures. The impact of this program was evaluated by questionnaire to attending surgeons, house staff, and parents. There were 123 surveys mailed (12 attending surgeons, 46 residents, 65 parents). The overall response rate was 51% (100% attending surgeons, 57% residents, and 38% parents). Only two of the attending surgeons had previous experience working with a PNP. All attending surgeons were enthusiastic about the PNP role in managing their patients and felt the PNP had decreased the workload of the resident. Eighty-three percent did not feel the PNP interfered with resident learning; 17% were undecided. Eleven of 12 felt the PNP had helped communication between them and their patients; one was undecided. The resident responses were assessed by level of training. Among senior residents, 82% felt the PNP had decreased the workload of the junior resident and 91% viewed the PNP as a valuable resource, whereas 87% of the junior residents felt the PNP decreased their workload and 100% felt the PNP was a valuable resource. None of the residents felt the PNP interfered with their learning and 73% of the junior residents and 55% of the senior residents felt the PNP contributed to their learning.(ABSTRACT TRUNCATED AT 250 WORDS)

Boston↗
PubMed1992

Response to Haemophilus influenzae type B conjugate vaccine in children undergoing splenectomy.

Asplenic children are at increased risk for serious infection with polysaccharide encapsulated bacteria including Haemophilus influenzae type b (HIB), Streptococcus pneumoniae, and Neisseria meningitidis. Immunization with polysaccharide vaccines is recommended for children undergoing splenectomy. In 1987 a new more immunogenic HIB vaccine was licensed in the US to replace the pure HIB polysaccharide vaccine that was licensed in 1985. This polysaccharide-conjugate vaccine consists of the HIB polysaccharide linked to a protein carrier, diphtheria toxoid. Therefore, we evaluated the immune response of children undergoing splenectomy to HIB-conjugate vaccine. Thirteen children (7 with Hodgkin's disease, 4 with idiopathic thrombocytopenia, 2 with hereditary spherocytosis) aged 3 to 19 years were immunized with HIB-conjugate vaccine prior to splenectomy and serum was obtained following splenectomy. In addition, 15 healthy control children aged 2 to 14 years were immunized with the pure polysaccharide HIB vaccine for comparison. The patients undergoing splenectomy who received the HIB-conjugate vaccine had a geometric mean IgG anti-HIB antibody concentration of 48,106 ng/mL versus 10,786 ng/mL for the control patients who received the pure polysaccharide vaccine (P = .01). The presumed protective level of antibody is 1,000 ng/mL and all children were well above this concentration. Therefore, we propose that children undergoing splenectomy be immunized with an HIB-conjugate vaccine.

Adolescent↗
PubMed1992

Reduced-size lung transplantation from adult to neonatal sheep.

Lung transplantation continues to be limited by donor availability. This shortage is particularly acute in the pediatric population. A model was developed in sheep to simulate human pulmonary transplantation from adults into newborns. Pulmonary allografts were performed in 8 neonatal lambs (less than 10 days old) weighing between 2.5 and 5 kg. Unrelated adult sheep donors were used (weighing 55 to 82 kg). The recipient body weight was approximately 6% that of the donor, but ranged from 4% to 8%. Ipsilateral or contralateral upper lobe allografts were used, although the latter required inversion prior to implantation. All venous anastomoses utilized left atrial cuffs. The pulmonary arterial and bronchial anastomoses were constructed end-to-end. Following implantation the lobes were further contoured to fit within the recipient hemithorax using a linear surgical stapler. Animals were immunosuppressed with cyclosporine. Two animals died 1 week postoperatively but the remaining six survived 1 to 5 months. Postoperative lung scans at 10 to 14 days confirmed satisfactory ventilation and perfusion of the allografts. All vascular anastomoses were intact at postmortem examination. Primary bronchial healing accomplished without an omental wrap. We conclude that successful lung transplantation can be performed from adult donors into neonatal recipients. Preservation of the maximal arterial and venous length during native pneumonectomy is essential to allow matching of the vascular cuffs. Short donor lobar bronchi may be responsible for the reliable primary healing. Improved immunosuppression will be required to assess long-term function and growth of these reduced-size allografts.

Animals↗
PubMed1991

Consistent cytogenetic aberrations in hepatoblastoma: a common pathway of genetic alterations in embryonal liver and skeletal muscle malignancies?

Cytogenetic analyses of four consecutive hepatoblastomas revealed near-diploid stemline karyotypes with relatively simple chromosome aberrations. Cytogenetic abnormalities shared by each tumor included trisomy for all of part of chromosome 2 and trisomy for chromosome 20. In two cases, partial trisomy for chromosome 2 resulted from direct duplication of long arm material with the shortest region of overlap being 2q23-2q35. In one tumor, each metaphase also contained a variable number of double minute chromosomes found not to derive from NMYC amplification. Interestingly, trisomy for 2q and trisomy 20 are also characteristic events in pediatric embryonal rhabdomyosarcomas. Furthermore, others have reported loss of heterozygosity for the short arm of chromosome 11 in both hepatoblastoma and childhood embryonal rhabdomyosarcoma, and both these malignant diseases are associated with Beckwith-Wiedemann syndrome. These cytogenetic and molecular findings suggest a parallel pathway of genetic steps in the initiation and/or progression of tumors of embryonal liver and skeletal muscle.

Carcinoma, Hepatocellular↗
PubMed1991

Surgical management of stage III and IV neuroblastoma: resection before or after chemotherapy?

Local control is vital for long-term survival for patients with stage III neuroblastoma, and although cure is difficult, ultimate success in stage IV neuroblastoma will necessitate control of the primary tumor as well as effective therapy of the metastases. The proper timing of surgical resection of the primary tumor is uncertain. Patients with stage III and IV neuroblastoma treated from 1977 to 1988 were retrospectively reviewed as to whether the resection was performed before or after chemotherapy. Complications assessed include significant blood loss, damage to adjacent organs, and delays before postsurgical chemotherapy could be given. Sixty patients were treated primarily at the authors' institution: 18 with stage III and 42 with stage IV disease. Chemotherapy consisted of combinations of nitrogen mustard, adriamycin, dacarbazine (DTIC), cisplatin, vincristine, and cyclophosphamide (MADDOC). Nine patients with stage III neuroblastoma underwent initial resection of the primary tumor before receiving chemotherapy. Three had complications, all with excessive blood loss (0.57, 2.0, and 3.0 times the estimated total blood volume [TBV]). One patient had renal infarction, and another had regrowth of the tumor before chemotherapy could be administered 35 days after surgery. There were no complications in the eight secondary explorations, four of which were complete resections. All had viable tumor in the resected specimen. Eleven of the 42 stage IV patients had primary resections, 5 of whom had complications: colocutaneous fistula, unilateral renal necrosis, chylothorax, and excessive blood loss (1.3 and 2 TBV). None of the 18 patients with delayed resection after 3 to 12 courses of chemotherapy had surgical complications with complete (14 patients), near complete (2 patients), or subtotal resections (2 patients).(ABSTRACT TRUNCATED AT 250 WORDS)

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1991

CT quantitation of tracheal cross-sectional area as a guide to the surgical and anesthetic management of children with anterior mediastinal masses.

Pediatric patients presenting with anterior mediastinal masses between January 1980 and November 1988 were reviewed to assess the correlation between tracheal cross-sectional area and anesthetic risks. Forty-two patients had evaluable computed tomography (CT) scans and underwent a surgical procedure. Thirty-four patients had Hodgkin's disease, six had non-Hodgkin's lymphoma, and two had mediastinal teratoma. Tracheal cross-sectional areas were greater than 75% of expected in 19 cases, greater than 50% to 75% in 16 cases, greater than 25% to 50% in five cases, and less than or equal to 25% in two cases. The presence or extent of symptoms did not correlate well with the degree of tracheal narrowing shown by CT scan except for orthopnea. Local anesthesia was used primarily in patients with significant tracheal narrowing (tracheal size was less than or equal to 56% in 5 of 6 patients). General anesthesia with spontaneous ventilation by mask was performed in four patients with tracheal areas of 33%, 73%, 76%, and 98% of expected. General endotracheal anesthesia was utilized in the remaining 32 patients, only three of whom had tracheal areas of less than 50% of expected (down to 30%, 26%, and 24% of expected) and one received preoperative radiotherapy (26%). None of these 32 patients had symptoms of orthopnea or dyspnea at rest, and only one had dyspnea on exertion. All tolerated anesthesia without difficulty. No patient in this series suffered respiratory or cardiovascular collapse during surgery. Adequate biopsy material was obtained in all cases.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1991

Immune thrombocytopenia: surgical therapy and predictors of response.

We have reviewed 40 patients with immune thrombocytopenia purpura (ITP) to assess current methods of preparation for surgery and to evaluate perioperative complications and response to splenectomy. Twenty-one patients had chronic ITP (greater than 1 year duration) and 19 patients had severe acute thrombocytopenia (platelet counts less than 10,000). A progression of methods of pretreatment was seen in the 10-year period reviewed. Seventeen patients received no treatment before admission for surgery, and 10 of these received platelet transfusions. Seventeen patients received steroids immediately preceding surgery; 16 of these responded and 1 received a platelet transfusion. Recently, 5 patients received intravenous gamma globulin (IgG) preceding surgery with all patients responding and none receiving platelet transfusions. One patient received a combination of steroids and IgG with good response and did not require platelet transfusion. No major postoperative complications occurred (ie, pancreatitis, small bowel obstruction, or sepsis) except for one patient requiring a secondary exploration for an accessory spleen and recurrent thrombocytopenia. Eight patients (20%), 6 with severe ITP and 2 with chronic ITP (5 males and 3 females) developed recurrence of thrombocytopenia following surgery up to 1 1/2 years after splenectomy. These patients all required further medical therapy. Three additional patients (2 chronic and 1 severe) developed thrombocytopenia following viral illnesses, but required no further therapy. Of the 8 surgical failures, 4 failed to respond to prior treatment with steroids, 1 to IgG, and 2 failed to respond to combination therapy, while one surgical failure responded to both steroid and combination therapy. Of the responders to splenectomy (32 patients), only 3 failed to respond to prior treatment with steroids.(ABSTRACT TRUNCATED AT 250 WORDS)

Acute Disease↗
PubMed1990

Human polysaccharide-specific B cells are responsive to pokeweed mitogen and IL-6.

The responsiveness of polysaccharide-specific B cells to PWM was examined in vitro. Spleen cells from six patients immunized with Haemophilus influenzae type b-diphtheria toxoid, pneumococcal and meningococcal vaccines were T cell-depleted and separated by Percoll density gradient centrifugation. In each B cell fraction, spontaneous antibody production was demonstrated to capsular polysaccharides as well as diphtheria toxoid. The peak of spontaneous antibody production was demonstrated to be five to seven days after immunization. When T cells and PWM were added, the total Ig secretion increased in all B cell fractions. PWM also enhanced IgG antibody directed to each of three polysaccharide Ag measured. This enhancement was most noticeable for nonresting B cells. The PWM effect was not confined to IgG, as IgM and IgA to Neisseria meningitidis type C were measured and also enhanced. The kinetics of the PWM response demonstrated the most IgG antibody to polysaccharide Ag from spleens immunized five to seven days before splenectomy. When the patients were immunized either 2 days or 4 mo before splenectomy, no spontaneous IgG antibody to polysaccharides was detected although PWM induced small amounts of antibody. Finally, anti-IL-6 antibody blocked PWM-induced total and polysaccharide-specific antibody production. We conclude that human polysaccharide-specific B cells are responsive to PWM and IL-6. We suggest that polysaccharide B cells are not truly "T cell-independent" and may respond to T cell lymphokines and thus are similar to protein-specific B cells.

Antibodies, Bacterial↗
PubMed1990

A proposed cause for the hepatic dysfunction associated with parenteral nutrition.

Total parenteral nutrition (TPN) is associated with cholestasis and hepatic steatosis, which can be lethal in infants who cannot be fed orally. It was determined that route of administration was not the critical variable in the development of hepatic steatosis. Two groups of young rats received equivalent amounts of a standard TPN solution either orally or intravenously for an 8- to 10-day period during which they received no other nutrition. Both groups gained equivalent weight and developed marked hepatic steatosis. To test whether the solution was toxic or deficient, three groups of rats were given TPN solution orally and a fraction of their usual daily intake of rat chow. Rats receiving less than 10% of their usual chow intake developed steatosis; rats receiving more than that did not. To determine the solubility of the protective material in chow, two groups of rats were given TPN solution orally and chow that been extracted with either water or the organic solvent chloroform. Rats eating the water-extracted chow developed steatosis, rats eating chloroform-extracted chow did not. Although the protective component in chow was apparently water soluble, addition of a water soluble extract of chow to the TPN solution fed another two groups of rats did not prevent steatosis at 0.1 mg/mL and only partially, if at all, at 10 mg/mL. TPN-related hepatic dysfunction, as measured by the development of hepatic steatosis in this model, may be due to a deficiency in the TPN solution. The missing constituent(s) appears to be present in rat chow and can be extracted with water, but not with an organic solvent.

Animals↗
PubMed1990

The therapy of biliary atresia combining the Kasai portoenterostomy with liver transplantation: a single center experience.

Survival has improved dramatically for children with extrahepatic biliary atresia (EHBA), but optimal surgical management remains controversial. We have studied 28 infants born between June 1981, and April 1988, who underwent Kasai's portoenterostomy as primary surgical treatment. Those with evidence of subsequent hepatic decompensation were evaluated for liver transplantation (LT). All were cared for by surgeons who perform both the Kasai portoenterostomy and LT. Following the Kasai operation, 16 of 28 patients (57.1%) have achieved total biliary drainage, while 5 of 28 (17.9%) achieved partial drainage, and 7 of 28 (25%) achieved no drainage. Nine of 28 (32.1%) have undergone LT, 4 of whom were transplanted at greater than 2 years of age. To date, 25 of 28 (89.3%) are alive. Twenty three of 25 (92%) living are jaundice-free, 65% of whom have undergone the Kasai operation only. We project that 5 of 28 (17.8%) will come to transplantation, and 12 of 28 (43%) have no current indication that they are likely to come to transplantation. We conclude that combining Kasai's portoenterostomy with LT as needed is an effective therapy for children with EHBA, that the Kasai operation offers significant benefit by delaying LT in many, and that the long-term results of management remain to be determined.

Analysis of Variance↗
PubMed1989

Genitourinary rhabdomyosarcoma in children.

Although rhabdomyosarcoma of the genitourinary tract occurs infrequently in children, it presents a challenging problem to the surgeon, radiotherapist, and oncologist. Thirty-six children with genitourinary rhabdomyosarcoma were treated at our institutions from 1957 to 1985. The primary site of tumor involved the bladder, prostate, or both in 15 patients, the paratesticular structures in 12, and female reproductive organs in nine. Although treatment modalities and philosophies of care varied over the past 27 years, the majority of patients were treated with a combination of surgery, chemotherapy, and radiation therapy. The event-free survival for all patients is 74%. Patients with paratesticular tumors had the best prognosis (88% event-free survival). Regional nodal radiation therapy is not necessary in this favorable group if the retroperitoneal lymph node dissection is negative. Of 15 children with bladder/prostate tumors nine had initial conservative surgery. Of these nine six are long-term survivors, but only three patients have intact bladders. The girls with tumors of the reproductive organs had an event-free survival of 63%; all patients in this group had hysterectomies. Chemotherapy and radiotherapy have not substituted for radical surgical procedures in most children with bladder or prostate rhabdomyosarcomas. Innovative therapeutic approaches are necessary for this group of patients.

Adolescent↗
PubMed1989

Chest wall tumors in infancy and childhood.

The chest wall is an infrequent site of malignancy in infancy and childhood. Management of these tumors, however, is of particular concern because of their aggressive behavior and the functional impairment which may result from local treatment. From 1976 to 1987 we have treated seventeen infants and children with tumors of the chest wall. Askin's tumors and Ewing's sarcoma were considered as a single entity, malignant small round cell tumor (MSRCT), and account for the majority (11 of the 17 patients). Other tumors represented were infantile fibrosarcoma (1), undifferentiated spindle cell sarcomas (2), osteogenic sarcoma (1), large cell lymphoma (1), and synovial sarcoma (1). Nine of 17 patients have survived (median follow-up of survivors 5 years); six patients died of disease and two from complications of therapy. All four patients with MSRCT and metastasis at diagnosis died of disease despite chemotherapy and radiotherapy. Four of the seven patients with localized MSRCT, who received combined modality therapy including resection (two after initial chemotherapy), radiotherapy, and chemotherapy, were continuously disease-free 16 months to 10 years following diagnosis. One of the three patients who failed died of complications of surgery to her extensive primary. A second patient had a relapse of disease in a hilar node four years after finishing vincristine, actinomycin, and cytoxan (VAC) chemotherapy; she was retreated with Adriamycin (doxorubicin, Adria Laboratories, Columbus, Ohio), vincristine, and cyclophosphamide as well as radiotherapy to her hilum and remains in second remission 56 months following her recurrence. The third patient suffered a distant relapse in bone and, before succumbing to his MSRCT, developed acute monocytic leukemia and died during a remission induction attempt. Mixed results were obtained for the patients with other tumor types.

Adolescent↗
PubMed1989

Surgical treatment of pancreas divisum causing pancreatitis in children.

Although controversial, pancreas divisum has been implicated as a cause of acute pancreatitis when there is stenosis of the accessory papilla that drains the duct of Santorini. Over the past 5 years, four children with pancreas divisum and recurrent pancreatitis were successfully treated surgically. The diagnosis was made by endoscopic retrograde cholangiopancreatography (ERCP) in each case. Surgical treatment included sphincteroplasty to the accessory papilla to improve drainage of the duct of Santorini, opening the ampulla of Vater to expose the ostium of the duct of Wirsung to enlarge it, and cholecystectomy.

Acute Disease↗