INVITED REVIEW GASTROINTESTINAL MANIFESTATIONS IN PEDIATRIC AIDS

The pathologic changes in the gastrointestinal tract of children with AIDS are variable, clinically significant, and reflect multisystemic disease processes. Inflammation, changes in the lymphoid tissue, miscellaneous lesions, and tumors are documented in 58 patients in addition to cases reported in the literature. Cytomegalovirus infection of the gastrointestinal tract, associated with ulcerations, hemorrhage, perforation, and intestinal obstruction, carries a high morbidity and mortality, whereas the remaining infections are not life threatening. Special stains and electron micrographic examination are important to identify correctlycertain microorganisms such asmycobacterium avium intracellulare, cryptosporidia, and microsporidia. Lymphoproliferative changes of the gastrointestinal tract, a component of the generalized lymphoproliferative process, need to be characterized by tumor markers and cytogenetic studies. Within the miscellaneous lesions, AIDS associated arteriopathy can be complicated by intestinal ulceration and perforation. Both lymphomas and smooth muscle tumor in children with AIDS are related to Epstein-Barr virus infection. The smooth muscle tumors are frequently malignant and multiple.     

PRIMARY INTRACRANIAL GERM CELL TUMORS: Clinicopathologic Review of 32 Cases

Primary intracranial germ cell neoplasms are rare tumors and constitute a heterogeneous group. We have reviewed 32 cases, over a 21-year period, from the University of Florida. The cases include 22 germinomas, 6 mixed germ cell tumors, and 4 teratomas. The clinical presentations in these cases were more closely related to the location of the tumor, that is, pineal or suprasellar, rather than the histologic subtype. Neuroimaging evaluation was useful in distinguishing between germinomas, teratomas, and other mixed germ cell tumors (MGCTs), primarily by evaluation of cystic versus solid lesions (teratoma versus germinoma), contents of cysts (teratoma versus MGCT), and infiltrative nature of the tumors (MGCT), although cytologic-histopathologic confirmation remains necessary. Germinomas responded favorably to radiation therapy with survival periods of over 16 years; MGCTs were treated with combination chemotherapy and radiation, with a markedly poorer prognosis. This study underlines the critical significance of histopathologic evaluation of the tumor in determining therapeutic interventions as well as prognosis.     

Fat gram target to achieve high energy intake in cystic fibrosis

Higher fat and energy intakes confer a survival advantage in cystic fibrosis (CF). There is a need to develop effective nutrition programmes that ensure optimal energy intake in CF.

Methodology:

A cross-sectional measurement of clinical characteristics and energy and fat intakes in patients attending the CF outpatients clinic of the John Hunter Hospital, Newcastle was undertaken. Twenty-nine subjects, mean age 12 years (range 4.3–20.2), completed weighed food records to determine the contribution of fat to the percentage of the recommended energy intake obtained and to document use of pancreatic enzyme replacement therapy.

Results:

Diets with a high percentage of energy derived from fat did not guarantee that individuals with CF met their energy requirements. Subjects with total fat intakes of 100 g per day or greater, however, achieved in excess of 110% recommended daily intake (RDI) for energy. Up to 47% of subjects consumed more pancreatic enzyme replacement capsules than shown to give maximum effectiveness.

Conclusion:

Setting a 100 g daily fat target is a realistic way of ensuring high energy intakes in CF. Fat ready reckoners would identify the fat content of food and prescribe specific numbers of pancreatic enzyme replacement capsules to be consumed with each meal or food item.     

外耳道腺样囊性癌(附11例报告)

外耳道晚样囊性癌较为少见，从1978年至l993年我院共收治11例，其中男4例，女7例，平均45．6岁。本文对其临床特点、诊断和治疗方法进行了分析。指出首次根治性手术非常重要。术后放疗可降低其复发率，放疗还可缓解由肿瘤引起的疼痛。     

Permanent low-activity iodine-125 implants for cerebral metastases

Beginning in 1987, selected patients with metastatic braintumors were treated with permanent implants of low-activityradioactive iodine-125 (¹²⁵I) seeds. These patients underwent craniotomy,gross total resection of the metastatic lesion, andplacement of the seeds. In general, criteria fortreatment included the presence of a recurrent tumorwith a volume too large to permit radiosurgery,and a Karnofsky Performance Score of 70 orhigher. Thirteen patients underwent 14 implant procedures; allreceived external whole-brain radiotherapy. Implant dose ranged from43 Gy to 132 Gy, with a meanof 83 Gy. Survival after implantation ranged from2 weeks to almost 9 years, with amedian of 9 months. Clinical and radiographic localcontrol was obtained in 9 patients. Two patientsdied of acute, postoperative complications within a monthof implantation, so no information regarding tumor controlis available for them. Late complications included abone flap infection in one patient and aCSF leak in another; both were treated withoutfurther sequelae.These results demonstrate that permanent ¹²⁵I implants canresult in good survival and quality of life,and occasionally can yield long-term survival. Potentially, itis a cost-effective treatment in that a separateprocedure for stereotactic implantation or radiosurgery is notneeded, as is the case with the useof temporary high-activity seeds. The permanent implantation itselfadds less than 10 minutes to the craniotomy,and the risk of symptomatic radiation necrosis islow. We recommend consideration of this procedure inpatients harboring large, recurrent metastatic tumors that requirefurther surgery.     

Clinical effects and pharmacokinetics of the fusion protein PIXY321 in children receiving myelosuppressive chemotherapy

A hemopoietin with the ability to accelerate both platelet and granulocyte recovery after intensive chemotherapy would have great clinical utility. The recombinant fusion protein composed of human granulocyte-macrophage colony-stimulating factor and interleukin-3 (PIXY321), showed some promise in early adult trials. However, studies for pediatric patients are limited, and there are no systematic data on the pharmacokinetics of PIXY321 given over prolonged periods at current dosage levels. Purpose: To determine the safety, clinical effects and plasma concentrations of increasing doses of PIXY321 in children treated with myelosuppressive chemotherapy. Methods: A total of 39 children with relapsed or high-risk solid tumors were enrolled in this phase I/II study. PIXY321 was administered once or twice daily by subcutaneous injection in total doses of 500 to 1000 μg/m² per day for 14 days after each course of chemotherapy with ifosfamide, carboplatin, and etoposide (ICE). Pharmacokinetic studies were performed on day 1 of the first course in 33 patients and repeated on day 14 in 13 patients (once-daily schedule only). Results: Although mild local skin reactions and fever were frequent, no dose-limiting toxicity was identified at the maximum dose studied (1000 μg/m² per day). There were no statistically significant differences in chemotherapy-induced hematologic toxicity with increasing doses of PIXY321 or with twice-daily vs once-daily dosing. On day 1, the median PIXY321 clearance was 657 ml/min per m² (range 77–1804 ml/min per m²) and the median half-life was 3.7 h (range 2.1–20.8 h). On day 14, clearance increased in all patients studied (median increase 63%), with a corresponding decrease in the median 12-h concentration (from 1.2 to 0.25 ng/ml). Maximum concentrations were <1 ng/ml in 81% of patients, and only two patients had maximum plasma concentrations equivalent to those required for consistent activity in vitro. Conclusions: The recombinant fusion protein PIXY321 proved safe in children treated with myelosuppressive ICE chemotherapy but had no demonstrable clinical benefits. The pharmacokinetic studies suggest that the observed lack of hematologic benefit may be explained by low plasma concentrations resulting from increased clearance with prolonged administration. Moreover, the significant increase in PIXY321 systemic clearance in the absence of increased circulating myeloid cells suggests that the upregulation of either extravascular compartment hematopoietic progenitor cells or nonhematopoietic cells may play an important role in controlling circulating concentrations of this unique cytokine. These findings highlight the importance of a thorough assessment of the systemic disposition of cytokines when determining the dose and schedule necessary to achieve clinical activity in patients. Received: 29 January 1997 / Accepted: 9 May 1997     

Assessment of proliferating cell nuclear antigen immunostaining in parotid tumors

We evaluated the prognostic value of immunostaining proliferating cell nuclear antigen (PCNA) by using a monoclonal antibody (PC10) in patients with parotid tumors. Twenty-seven cases were studied. Immunohistochemical studies were carried out on paraffin-embedded tissues from the patients, and the PCNA index was calculated as the percentage of positively staining tumor cells. The PCNA index ranged from 0.1 to 65.3%. We divided the 27 lesions into three groups histologically: group A with benign pleomorphic tumors (11 cases), group B with low-grade malignant tumors (5 cases), and group C with high-grade malignant tumors (11 cases). The mean PCNA index was 0.7% in group A, 2.0% in group B, and 23.1% in group C. The clinical data revealed a significantly higher local tumor recurrence and mortality rate in group C than in groups A and B. We conclude that PCNA may be used as an important indicator for determining clinical prognosis in parotid tumors. Received: 26 July 1997 / Accepted: 28 October 1997     

Facial nerve outcome in lateral skull base surgery for benign lesions

Objective: To statistically identify factors most important in affecting CN7 outcome in lateral skull base surgery for benign lesions. Study Design: A retrospective review of 217 nonmalignancy lateral skull base procedures from 1970 to 1995 at the Otology Group in Nashville. Methods: Charts were reviewed for epidemiology, histopathology, staging, type of CN7 mobilization (none, short, long, severance with reanastomosis, and resection), preoperative and postoperative CN7 function, surgery performed, and survival. Results: Average House-Brackman (HB) scores for mobilizations were as follows: short, 1.65: long, 2.74: and grafting, 4.33. Factors found to affect outcome in a statistically significant fashion were preoperative HB score, staging, type of CN7 manipulation, and surgical approach. Meningiomas were found to have a worse outcome than glomus tumors. Conclusions: Complete resection of tumors should be performed with minimal manipulation of the facial nerve based on regional anatomy and tumor anatomy. Laryngoscope, 108:1480–1484, 1998