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Therapy for pediatric aplastic anemia; 7 years experience at the Puerto Rico Pediatric Oncology Program.

Nine pediatric patients with aplastic or hypoplastic anemia were seen at the Pediatric Hematology-Oncology service of the University of Puerto Rico, from 1980 to first trimester of 1987. Included were eight males and one female ranging from twenty months to sixteen 8/12 years (mean 13 years). Hematomas and fever were the most common complaints. The interval since the first symptoms and the diagnosis varied from one day to one week. The peripheral cell count was abnormal in all patients; pancytopenia being the most common finding. Five of seven who received antithymocyte globulin (ATG-AM), showed improvement in their peripheral count. The median time interval from the end of ATG-AM and response was two months (range 2 weeks to 15 months).

Adolescent↗

[Cooperation between pediatrics and child psychiatry in a pediatric hospital; possibilities and limits].

Cooperation between pediatrics and child psychiatry in a children's hospital is becoming more and more important and necessary in dealing with many common problems such as psychological reactions to physical illness, psychosomatic disturbances, physical examination of psychiatric patients, and disorders and retardation of growth and development. The authors describe a cooperative model which has been developed and proved its usefulness during a period of 10 years. This method is an alternative to the traditional referral or consulting system, which involves little real cooperation between the two specialties, and also to excessive penetration of nonmedical psychology into pediatrics.

Child Psychiatry↗

Declaring pediatric brain death: current practice in a Canadian pediatric critical care unit.

OBJECTIVE: To document the criteria used to declare brain death in a pediatric critical care unit (PCCU). DESIGN: Retrospective chart review. SETTING: Regional PCCU in southwestern Ontario. PATIENTS: Sixty patients 16 years of age or less declared brain dead from January 1987 through December 1992. OUTCOME MEASURES: Presence or absence of documentation of irreversible deep coma, nonresponsive cranial nerves, absent brain-stem reflexes, persistent apnea after removal from ventilator, presence or absence of blood flow detected by radioisotope scanning, presence or absence of electroencephalographic evidence of electrocerebral activity. RESULTS: The 60 patients accounted for 1.5% of all PCCU admissions; 17 were under 1 year of age. In 39 cases brain death was diagnosed using clinical criteria ("certified brain death"), which could not be fully applied in the remaining 21 cases ("uncertifiable but suspected brain death"). Electroencephalography and cerebral blood-flow studies with technetium-99m hexamethyl-propyleneamine oxime were used as ancillary tests in 16 patients with certified brain death and in 17 with uncertifiable but suspected brain death who survived long enough to be tested. Electrocerebral silence was demonstrated in all nine patients who underwent electroencephalography. Cerebral blood flow was undetectable in 26 of the 30 patients tested, and an abnormal pattern of blood flow was seen in the remaining 4, all of whom received a diagnosis of certified brain death. CONCLUSIONS: Pediatricians in this large tertiary care referral centre are using clinical criteria based on the 1987 guidelines of the CMA to diagnose brain death in pediatric patients, including neonates. When clinical criteria cannot be fully applied, ancillary methods of investigation are consistently used. Although the soundness of this pattern of practice is established for adults and older children, its applicability to neonates and infants still needs to be validated.

Adolescent↗

Medication dispensing in pediatric office practice. Committee on Practice and Ambulatory Medicine, American Academy of Pediatrics.

The changing practice of dispensing medication in pediatric offices needs ongoing evaluation to determine the extent to which patients' and practice's needs are being met. Research should assess the extent to which in-office dispensing enhances patient compliance and patient education or whether it increases or decreases patient medication errors. Pediatricians choosing to dispense medication should monitor such research carefully, review state and federal laws periodically, and evaluate recommendations from professional organizations. The American Academy of Pediatrics holds that the practice of medication dispensing is acceptable and appropriate provided that it is legally permissible and that it is structured primarily to serve the best interests of the patient.

Drug Prescriptions↗

Pediatric rheumatology clinic populations in the United States: results of a 3 year survey. Pediatric Rheumatology Database Research Group.

OBJECTIVE: To describe the patient population referred to pediatric rheumatology centers (PRC) across the US 1992-95; and to compare these results to data on regional PRC populations. METHODS: A Pediatric Rheumatology Disease Registry was established in 1992. Data on new patients seen at 25 PRC across the US were submitted to the registry for a 36 month period from 1992 through 1995. RESULTS: A total of 12,939 patients were submitted to the registry. Of these patients, 5245 (40.5%) had rheumatological diagnoses. Patients with juvenile rheumatoid arthritis were the largest group of the patient population (2071 patients-16% of total diagnoses). There were 1568 patients with other forms of childhood arthritis (12%), 1172 with collagen vascular diseases (9%), and 434 with vasculitis (3.3%). Over 50% of the patients had nonrheumatologic diagnoses, including 1577 with idiopathic pain syndromes (12%). Other diagnoses included infections, orthopedic conditions, and malignancies. Fourteen percent of the patients were not given a diagnosis at the time of the initial visit. CONCLUSION: PRC see a wide variety of patients. Although the majority do have rheumatologic conditions, over 50% of new patients have conditions not autoimmune in origin. Fourteen percent of the patients cannot be diagnosed at the time of their first clinic visit, requiring time to see the evolution of their symptoms before a definitive diagnosis can be assigned.

Adolescent↗

Pediatric rheumatology in the United Kingdom: data from the British Pediatric Rheumatology Group National Diagnostic Register.

OBJECTIVE: To establish a computerized national diagnostic register for pediatric rheumatology in the UK; to describe the demography and diagnostic classification of children referred to pediatric rheumatology clinics; to estimate the current incidence of juvenile arthritis (JA) in the UK. METHODS: A diagnostic register was established in 1989: 23 centers have contributed data on all new cases seen since they joined the register; 18 centers have also contributed data on all current attenders. For 2 centers with well defined catchment areas, the incidence of JA was estimated. RESULTS: A total of 4948 cases were registered, of whom 2962 (60%) were female. 1991 (40%) had a diagnosis of JA. The 2nd largest category was mechanical/orthopedic problems (24%). Pauciarticular juvenile chronic arthritis was the most common subtype of JA. Seropositive RA was rare. Tertiary referral centers saw proportionately more JA and district general hospitals saw proportionately more mechanical problems. The annual incidence rate for JA from 2 centers was 10/100,000, and for all rheumatic disorders was 32-42/100,000 children under age 16. CONCLUSION: The relative proportion of patient diagnoses varies between centers. Nevertheless, the incidence of hospital referred JA seems very uniform.

Adolescent↗

The role of the pediatrician in rural EMSC. American Academy of Pediatrics. Committee on Pediatric Emergency Medicine.

In rural America pediatricians can play a key role in the development, implementation, and ongoing supervision of emergency medical services for children (EMSC). Often the only pediatric resource for a large region, rural access pediatricians are more likely to treat pediatric emergencies in their own offices, and are a vital resource for rural physicians, or other rural health care professionals (physician assistants, nurse practitioners), and emergency medical technicians (EMTs) to improve system-wide EMSC by providing education about issues from prevention to rehabilitation, technical assistance in protocol writing, hospital care, and dat accumulation, and as advocates for community and state legislation to support the goals of EMSC.

Child↗

Phase I study of a 120-hour continuous intravenous infusion of 5-fluorouracil in pediatric patients with recurrent solid tumors: a Pediatric Oncology Group study.

To determine the maximum tolerated dose of 5-fluorouracil administered as a 120-hour continuous intravenous infusion to pediatric patients, we performed a phase I study using a starting dosage of 900 mg/m2/day. The maximum tolerated dosage (MTD) was 1,100 mg/m2/day. At this dosage level 40% of courses were complicated by grade 3 mucositis. Three additional patients were treated at the dosage level of 1,000 mg/m2/day after the MTD was determined. We recommend the dosage level of 1,000 mg/m2/day for phase II studies of 5-fluorouracil administered as a 120-hour continuous intravenous infusion to pediatric patients.

Adolescent↗

Rationale and methodology of the multicenter pediatric cerebrospinal fluid shunt design trial. Pediatric Hydrocephalus Treatment Evaluation Group.

Cerebrospinal fluid shunt failure remains a common and at times overwhelming problem in pediatric patients with hydrocephalus. Two new shunt valve designs, the Orbis-Sigma (Cordis Corporation, Miami, Florida) and the Delta valve (PS Medical, Goletta, California), have flow/pressure characteristics dramatically different from those of standard differential pressure valves which have been used for over three decades. Both new designs reduce the siphoning effect in the upright position, and have been reported to reduce shunt failure rates in uncontrolled series, allegedly due to reduction in shunt overdrainage. Most mechanical shunt failure in the first 2 years after implantation is due to proximal shunt obstruction, overdrainage, and loculated ventricles. By reducing the incidence of slit ventricles associated with standard valves, both new designs could be envisioned as reducing the early mechanical complications. The improved results with both new valves could, however, also be to a large extent due to other confounding effects of shunt surgery, including patient selection, surgical technique, and specific configuration of the components of the shunt other than the valve. There are also theoretical reasons why these valve designs might be worse than their predecessors, including the narrow orifice and high resistance of the Orbis-Sigma, and the flexible membrane of the siphon control portion of the Delta valve, which may increase the ventricular pressure in the upright position or become blocked by encasing scar tissue. For this reason a randomized trial is required to determine efficacy, and a standard differential pressure valve is required as the control design. A significant reduction in early shunt failure would dramatically improve the morbidity and mortality of pediatric hydrocephalic patients, as well as providing substantial savings to the health care system. Failure to determine any difference would focus attention on other issues surrounding shunt surgery, such as patient characteristics or surgical technique.

Cerebrospinal Fluid Shunts↗

Ventriculoperitoneal shunts for hydrocephalus: a focus group discussion on the selection of shunt systems in pediatrics. A report of the Pediatric Neurosurgery Research Group meeting, December 1992.

As part of the Continuing Quality Improvement Study, phase I, 12 pediatric neurosurgeons participated in a discussion group to respond to the various issues related to selection of ventriculoperitoneal shunt systems in their practice. It was estimated that between then all the 12 participants performed approximately 1200 shunt procedures per year in the USA. The data were analyzed by grid and fish-bone charts as part of the data collection. All 12 participants indicated that they preferred a reservoir in the shunt system, 8 of them indicating that they preferred this for a manual test of the system. Three indicated that they did not routinely manipulate the system for testing. One participant stated that he placed a reservoir within the shunt system primarily to prevent migration of the ventricular catheter. Nine neurosurgeons indicated that they employed cranial valves in their shunt systems, and three indicated that they employed slit-end peritoneal catheters only. In reference to Delta valves, nine participants indicated that they employed them on occasions. In reference to pressure in the valve system, one participant employed high-pressure valves at all times, five employed only medium-pressure systems, and the remaining six always employed low-pressure valves. All 12 neurosurgeons stated that the incidence of symptomatic slit ventricle in their practices was between 1% and 5%, in their mind irrespective of the system used. It was concluded that any form of ventriculoperitoneal shunt, with or without valves, with slit-end valves or cranial valves seemed to work equally well in the hands of a dedicated pediatric neurological surgeon.(ABSTRACT TRUNCATED AT 250 WORDS)

Cerebrospinal Fluid Pressure↗

A phase II study of every other day high-dose ifosfamide in pediatric brain tumors: a Pediatric Oncology Group Study.

Despite reported activity in many other solid tumors, high-dose ifosfamide produces few objective responses in recurrent pediatric brain tumors. Alkylating agents such as cyclophosphamide (CYCLO) possess good activity in many of solid tumors, including brain tumors. Although Ifosfamide (IFOS), a structural congener of CYCLO, has been suggested to have greater activity in several tumors, its activity in brain tumors is uncertain. We conducted a phase II trial of every-other day IFOS (3 gm/M2/qod x 3) in 87 recurrent pediatric brain tumors. Responses were evaluable in 71 patients. Partial responses occurred in 1/6 patients with low grade astrocytoma, 1/16 with malignant glioma, 1/16 with medulloblastoma, 1/3 with pineoblastoma and 1/12 patients with ependymoma. No responses occurred among 10 patients with brain stem gliomas or 8 patients with other brain tumors. Despite the poor objective response rate, 23/71 patients were clinically and imaging stable for periods of 8-62 weeks. There was no relationship between prior CYCLO treatment and subsequent response or failure with IFOS. The predominant toxicity was myelosuppression. Although generally reversible, prolonged suppression and sepsis were responsible for the deaths of 3 heavily pretreated patients. Renal toxicity was uncommon; 2 patients had grade III, and one grade IV renal tubular dysfunction. One patient had grade IV hematuria. Neurotoxicity was less common than in studies of daily ifosfamide; only 1 patient had grade IV neurotoxicity. Three patients had grade III or IV IFOS related hyponatremia. Despite the good stable disease rate, the poor rate of objective response suggests that IFOS monotherapy possesses little clinically meaningful activity in brain tumors.

Adolescent↗

An evaluation of pediatric in-hospital advanced life support interventions using the pediatric Utstein guidelines: a review of 203 cardiorespiratory arrests.

PURPOSE: Evaluate the efficacy of advanced life support interventions using the pediatric Utstein guidelines. METHODS: Charts from all patients for whom a cardiorespiratory arrest code was called during a six-year period in a university affiliated centre were reviewed. Data were recorded according to the pediatric Utstein guidelines and a P < 0.05 was considered significant. RESULTS: Of the 234 calls, 203 were retained for analysis. The overall survival rate at one year was 26.0% of which 10% had deterioration of their neurologic status compared to the pre-cardiorespiratory arrest evaluation. Time to achieve sustained return of spontaneous circulation (ROSC; P < 0.0001) and sustained measurable blood pressure (P = 0.002), to perform endotracheal intubation (P = 0.04) and the dose of sodium bicarbonate (P < 0.0001) were indicators of long-term survival. Two patients were alive at one year with unchanged neurologic status despite a time to achieve sustained ROSC longer than 30 min (38 and 44 min). The mean first epinephrine dose of patients for whom ROSC was achieved but unsustained was higher than those for whom ROSC was achieved and sustained (0.038 +/- 0.069 mg*kg(-1) vs 0.011 +/- 0.006 mg*kg(-1); P = 0.004). Survival rate and mean first epinephrine dose of patients who received their first epinephrine dose endotracheally (13.3%; 0.011 +/- 0.004 mg*kg(-1)) were comparable to those of patients who received their first epinephrine dose intravenously (7%; 0.015 +/- 0.027 mg*kg(-1)). CONCLUSIONS: For intravenously administered epinephrine, a dose of 0.01 mg*kg(-1) seems appropriate as the first dose. The endotracheal route is a valuable alternative for epinephrine administration and, for infants, the dose does not need to be increased. A minimal resuscitation duration time of 30 min can be misleading if ROSC is used as the indicator.

Academic Medical Centers↗

Three dimensional finite element analysis of the pediatric lumbar spine. Part II: biomechanical change as the initiating factor for pediatric isthmic spondylolisthesis at the growth plate.

A non-linear 3-dimensional finite element pediatric lumbar spine model with vertebral growth plate and apophyseal bony ring was developed. Lumbar spondylolysis was simulated in the model. The Von Mises stresses in the structures surrounding the vertebral growth plate, including apophyseal bony ring and osseous endplate were calculated in various loading modes. Instantaneous axis of rotation (IAR) path from flexion to extension was also analyzed. The results were compared with those of the intact model and the literature. The IAR path was at the posterior disc-endplate space of the lower vertebra in the intact spine, and moved cranially towards the upper-posterior disc space in the lytic spine. This was in agreement with in vivo radiological data by Sakamaki et al. [19]. During various loading modes, stresses in the spondylolytic pediatric model were higher than that of the intact model; ranging from 1.1 to 6.0 times, with the highest value in extension at the growth plate. In conclusion, FE models indicate that stress concentrations in the lytic model increase at the growth plate which may lead to physis stress fracture leading to spondylolisthesis.

Biomechanical Phenomena↗

Transesophageal echocardiography and the intraoperative management of pediatric congenital heart disease: initial experience with a pediatric esophageal 2D color flow echocardiographic probe.

Intraoperative color Doppler transesophageal echocardiography (TEE) was performed in 26 patients undergoing corrective or palliative surgery for congenital heart disease. Age ranged from 1 day to 15 years, and body weight ranged from 2.9 to 42 kg. Objectives of the study were to determine the smallest infant in whom the pediatric probe could be used safely, additional diagnostic value, and it role in the intraoperative assessment of the surgical repair. The insertion of the pediatric probe was possible in all 26 patients. The smallest infant in this series was a newborn weighing 2.9 kg. Excellent correlation was obtained with preoperative transthoracic echocardiographic findings and operative findings. Assessment of the surgical repair was obtained in the immediate postcardiopulmonary bypass period. No short-term complications occurred in this series. Intraoperative color Doppler TEE provided a detailed and accurate assessment of the morphology, the function of the heart, and altered the management of at least two patients.

Adolescent↗

Pediatric Voice Handicap Index (pVHI): a new tool for evaluating pediatric dysphonia.

PURPOSE: The Voice Handicap Index (VHI) is widely used and accepted into adult clinical practice. The present study was initiated to adapt the VHI to the pediatric population and to validate it in the form of a parental proxy. METHODS: The initial modification of the adult VHI involved changing the language of the statements to reflect a parent's responses about their child and eliminating questions that would not relate to children. It was administered in conjunction with 10 open-ended questions regarding the impact of the child's voice quality on overall communication, development, education, social and family life. The pVHI was then modified in content and language, and the final 23-item parental proxy product was used for the validation process. The modified pVHI was administered to two groups of patients following IRB approval from Cincinnati Children's Hospital Medical Center. RESULTS: Normative data was obtained from 45 parents of healthy children. The group consisted of 21 males, age ranges 3-12 years old. The mean scores of the total pVHI and its subscales are: functional (F) 1.47, physical (P) 0.20, emotional (E) 0.18 and total (T) 1.84. The test group consisted of 33 guardians of children presenting for a voice evaluation pre- or post-laryngotracheal reconstruction. This group differed greatly from the control group on each subscale and total score. The mean scores of the airway group were as follows: F 13.94, P 15.48, E 12.15 and T 41.58. Test-retest reliability of the total pVHI score was measured using Pearson's correlation coefficient. The scores were 0.95, 0.77, 0.79 and 0.82, respectively. A correlation matrix for pVHI subscore and total score showed significance, with results similar to those reported for the original adult VHI. CONCLUSIONS: The aim of the present study was to modify the VHI to serve a similar role in the evaluation of the effects of dysphonia on the pediatric population. The statistical results reveal a high correlation between the VHI and the pVHI. The pVHI provides a high internal consistency and test-retest reliability. This tool will be utilized to follow a child's development following surgical, medical and behavioral interventions.

Adolescent↗

Radiotherapy in pediatric medulloblastoma: quality assessment of Pediatric Oncology Group Trial 9031.

PURPOSE: To evaluate the potential influence of radiotherapy quality on survival in high-risk pediatric medulloblastoma patients. METHODS AND MATERIALS: Trial 9031 of the Pediatric Oncology Group (POG) aimed to study the relative benefit of cisplatin and etoposide randomization of high-risk patients with medulloblastoma to preradiotherapy vs. postradiotherapy treatment. Two-hundred and ten patients were treated according to protocol guidelines and were eligible for the present analysis. Treatment volume (whole brain, spine, posterior fossa, and primary tumor bed) and dose prescription deviations were assessed for each patient. An analysis of first site of failure was undertaken. Event-free and overall survival rates were calculated. A log-rank test was used to determine the significance of potential survival differences between patients with and without major deviations in the radiotherapy procedure. RESULTS: Of 160 patients who were fully evaluable for all treatment quality parameters, 91 (57%) had 1 or more major deviations in their treatment schedule. Major deviations by treatment site were brain (26%), spinal (7%), posterior fossa (40%), and primary tumor bed (17%). Major treatment volume or total dose deviations did not significantly influence overall and event-free survival. CONCLUSIONS: Despite major treatment deviations in more than half of fully evaluable patients, underdosage or treatment volume misses were not associated with a worse event-free or overall survival.

Antineoplastic Combined Chemotherapy Protocols↗

Mortality following radiofrequency catheter ablation (from the Pediatric Radiofrequency Ablation Registry). Participating members of the Pediatric Electrophysiology Society.

Deaths have been reported following radiofrequency catheter ablation (RFCA), but the mortality rate in children has not been defined. This study sought to analyze the incidence and the factors associated with mortality related to RFCA. Ten of 4,651 cases (0.22%) reported to the Pediatric RFCA Registry resulting in death were reviewed and compared with a matched control group (n = 18). Death occurred in 5 of 4,092 children (0.12%, ages 0.1 to 13.3 years) with structurally normal hearts. Death was related to traumatic injury, myocardial perforation and hemopericardium, coronary or cerebral thromboembolism, and ventricular arrhythmia. All cases were left-sided (p = 0.019 vs right or septal) supraventricular arrhythmias with radiofrequency applications in the systemic atrium and/or ventricle, and all procedures were successful. Mortality occurred in 5 of 559 children (0.89%, p = 0.001 vs normals, ages 1.5 to 17.4 years) with structural heart disease. No new pathology except the mural radiofrequency lesions was seen at autopsy. Those with structurally normal hearts who died were smaller (32.7 vs 55.6 kg, p = 0.023) and had more radiofrequency applications (26.3 vs 8.7, p = 0.019) than those who survived. No differences were demonstrated for those with abnormal hearts. Operator experience was not different (deaths 103 +/- 106 vs controls 117 +/- 125, p = 0.41). Mortality associated with pediatric RFCA is rare, but is more frequent when there is underlying heart disease, lower patient weight, greater number of radiofrequency energy applications, and left-sided procedures. Operator experience does not appear to be a factor leading to mortality.

Adolescent↗