Allogeneic bone marrow transplantation for fucosidosis.

Bone marrow transplantation was performed on an 8-month-old boy who was diagnosed as having fucosidosis following the diagnosis of the disease in his older brother. Although he was asymptomatic and his development was normal, abnomalities were found on an MRI scan prior to transplant. In the absence of a suitable related donor, an unrelated volunteer donor was used. Conditioning for the transplant consisted of busulphan and cyclophosphamide. Graft-versus-host disease prophylaxis consisted of in vitro T cell-depletion of the bone marrow and in vivo administration of cyclosporin. The post-transplant period was complicated by moderately severe graft-versus-host disease. Engraftment was documented by the presence of donor levels of alpha-fucosidase, donor blood group and tissue type (difference in the DQ antigen), and chromosomal polymorphism pattern of donor origin. Eighteen months after transplant, there is evidence of mild neurodevelopmental delay. By contrast, his elder sibling showed far greater developmental delay at the same age. The patient's MRI scan shows improvement. We believe this to be the first case of human fucosidosis treated by bone marrow transplantation.

The predictive value of serum interleukins in recurrent respiratory papillomatosis: a preliminary study.

OBJECTIVE: IL-2 is the primary interleukin responsible for activation of the cell-mediated (Th1) arm of the immune response. Our objective was to determine whether a correlation existed between circulating levels of interleukin-2 as well as its soluble receptor (sIL-2R) and the clinical course of recurrent respiratory papillomatosis. METHODS AND MATERIALS: Fifteen children with a histological diagnosis of RRP were recruited. Age at the time of study, time since first diagnosis, and number of surgical interventions were recorded. The number of surgically treated recurrences per year was then calculated. We obtained serum samples from each of these 15 children and from 10 normal control subjects. We then performed in vitro determination of serum IL-2 and soluble IL-2 receptor levels using enzyme-linked immunosorbent assay (ELISA) techniques. RESULTS: IL-2 was significantly lower (136.6 vs. 199.9 pg/mL, P =.035) in papilloma patients than in control subjects. IL-2R was also lower in papilloma patients (531.7 vs. 785.8 U/mL, P =.025). There was no statistical age difference between the papilloma and control groups. Among patients with papillomatosis, IL-2 and sIL-2R levels were significantly higher in those with aggressive disease (>4 surgically treated recurrences per year) versus non-aggressive disease (179.2 vs. 99.2 pg/mL, P =.024; and 697 vs. 387 U/mL, P =.022). Age was also significantly lower in the aggressive papilloma group (P =.002). CONCLUSIONS: Levels of interleukin-2 and IL-2 receptor were significantly lower in patients with recurrent respiratory papillomatosis compared with normal children. These data support the presence of an aberrant cell-mediated immune response in children with recurrent respiratory papillomatosis.

Brief bilateral vocal cord paralysis after insecticide poisoning. A new variant of toxicity syndrome.

We describe a new variant of life-threatening organophosphate toxicity syndrome that produces a brief bilateral vocal cord paralysis. There are 3 recognized types of toxicity syndrome: acute (instantaneous), intermediate (slightly delayed, i.e., hours to days), and delayed (weeks to months). Ingestions of large doses of insecticides lead to a cholinergic crisis and possible death (acute-type syndrome). Exposures to lower doses may cause the intermediate- or delayed-type syndrome. The intermediate-type syndrome is characterized by slightly delayed polyneuropathy and generalized weakness. Transient vocal cord paralysis has also been reported in association with other neurologically profound findings. The delayed-type syndrome can produce muscle weakness for months. Our patient, a 2-year-old boy, was found eating a substance later found to be contaminated with insecticide. Within minutes, fever and somnolence developed, followed by progressive respiratory distress and stridor, without generalized weakness. The child's condition progressed to complete airway obstruction, and intubation was necessary. Emergency laryngoscopy and bronchoscopy were performed to rule out epiglottitis or a foreign body. Instead, a bilateral vocal cord paralysis was found. The paralysis lasted 2 days before completely resolving. Insecticide poisoning was suspected. We theorize that our patient manifested a combination of the acute-type syndrome, because of the immediacy of the onset of the symptoms (i.e., fever and somnolence), and the intermediate-type syndrome, because of the transient vocal cord paralysis. Because of the potential dangers involved, we wish to familiarize physicians concerning organophosphate poisoning; to alert the medical community to this variant of toxicity syndrome, which involves transient bilateral vocal cord paralysis; and to demonstrate the benefit of early otolaryngological consultation for the prevention and treatment of airway obstruction in patients with suspected organophosphate poisoning, be it from insecticides or poison gas.

Effects of global competition on health care administration in Finland and the United States. What you can do to save your practice.

Global competition, a faltering Finnish economy, and deep recession are forcing difficult decisions on the health care administrators of this Nordic country known for its extensive social welfare safety net and socialized health care system. In the first part of this article the Finnish health care system and its problems, as well as possible solutions, are presented. Similarities to our own system are noted and discussed. In an interview, the director general for the Minister of Social Affairs and Health of Finland, Kimmo Leppo, expressed concern about future funding sources in his country for the same levels of social welfare and health care programs that presently exist. The Finns are considering managed competition among providers, increasing copayments, excess hospital beds, anticipated hospital closures in their country, block grants, and how to deal with rising physician unemployment. He was interested in the proposed US government health care plans, and especially the state of Tennessee's TennCare plan, a state plan that closely resembled the failed Clinton health care reform package. The second part of this article explores how global competition has brought the pressure of modern competitive economics into the US medical arena.

Malignant infantile osteopetrosis: otolaryngological complications and management.

OBJECTIVES: To inform otolaryngologists about upper airway obstruction requiring tracheotomy and other otolaryngological manifestations of malignant infantile osteopetrosis (MIOP) and to discuss pathophysiological features, management, and new treatment strategies in MIOP. DESIGN: Ongoing case series combined with a retrospective chart review. SETTING: International tertiary pediatric hospital. INTERVENTIONS: Patients with MIOP were initially referred for treatment and routine follow-up. Tracheotomy was performed to manage obstructive sleep apnea. Audiograms were also performed at regular intervals. RESULTS: The records of 9 patients were examined. The otolaryngological findings of hearing loss, obstructive sleep apnea (sometimes requiring tracheotomy), otitis media, and chronic osteomyelitis with facial fistulas were identified. CONCLUSIONS: Osteopetrosis is a rare condition caused by a failure of the osteoclast to resorb bone. This results in thickened dense, deformed, and easily fractured bone. As a result, growth failure, anemia, hypoplastic dentition, chronic infections, facial fistulas, blindness, hearing loss, nasal congestion, and upper airway obstruction may occur. The management of otolaryngological problems in a child with osteopetrosis is an important component in comprehensive care. To our knowledge, this study represents the largest case series of MIOP in the otolaryngology literature.

Airway management of neonates with antenatally detected head and neck anomalies.

Five cases of prenatally detected neck masses that had a potential for airway obstruction at birth are described. The various options for management of the airway are discussed, including using maternal-fetal circulation until intubation, rigid bronchoscopy, tracheotomy, cyst aspiration, or extracorporeal membrane oxygen support. Congenital abnormalities involving the fetal face or neck are extremely rare. With technical advances in ultrasonography, these masses were first noted on prenatal ultrasound in the late 1970s. Before that period, they were detected at delivery. These masses are solid or cystic and may cause asphyxia because of airway obstruction at the time of delivery. The survivability of these neonates without immediate intervention at birth is 0% to 20%. If a neck mass is detected in the fetus by prenatal ultrasonography, then a strategic plan for these types of cases should be developed early in the prenatal period. The airway management plan should be tailored for each individual case. Coordination and the expertise of an obstetrician, neonatologist, anesthesiologist, and pediatric otolaryngologist are needed to manage these complex situations.

A simple method to improve the energy value of bank human milk.

Drip milk can be collected in large amounts, hence its potential value for human milk banks. This advantage is however offset by its low energy value. For those milk banks which depend mostly on drip milk, the energy value of its donor milk can be improved to a more acceptable level, and total volumes of donations increased, by encouraging mothers to combine collections of both drip and expressed milk.

Unusual neck masses secondary to jugular venous abnormalities: case report and discussion.

Thrombosis of the lateral sinus/proximal jugular vein and jugular venous ectasia are infrequent but not uncommon neck masses. Lateral sinus thrombosis was well described in the preantibiotic era, but modern-day surgeons may not be familiar with this entity. Failure to diagnose a neck mass due to jugular vein thrombosis may have deadly consequences. Jugular vein ectasia, which is innocuous, has been mistaken for laryngocele, cystic hygroma, as well as other vascular lesions. However, the management of jugular vein ectasia, which is thought to be caused by incompetence of the bicuspid jugular venous valve, is conservative. We present a case illustrating a neck mass arising from the internal jugular vein with a discussion of jugular venous pathology.

Severe combined immunodeficiency: otolaryngological presentation and management.

In order to increase the awareness of otolaryngologists of severe combined immunodeficiency syndrome (SCIDS) so they may contribute to an earlier diagnosis of this disorder, we performed a retrospective chart review of a multicenter series from 2 children's hospital medical centers. Eighteen cases were identified, and 14 had an otolaryngological presentation. The average age of presentation was 3.3 months, and 72% were males. Most cases were inherited in an X-linked fashion. Five patients had thrush; 4 had recurrent otitis media. Other otolaryngological presentations included cough, mouth ulcers, pharyngitis, mastoiditis, and bilateral neck abscess. The most severe form of immunodeficiency, SCIDS is a rare condition that involves a disorder in both T and B cell functions. The manifestations involving the head and neck include recurrent upper respiratory tract infections, otitis media, thrush, oral ulcers, and abscesses. It is important that SCIDS be considered in any infant with recurrences of these common infections.

Evaluation and management of hemoptysis in infants and children. A report of nine cases.

Hemoptysis is an occasional complication of adult pulmonary disease and is rare in children. The most common causes in adults are infection, bronchiectasis, pulmonary neoplasm, cystic fibrosis, pulmonary infarct, and trauma; in children the most common causes are infection and congenital abnormalities of the cardiopulmonary vasculature. Nine cases of hemoptysis in seven infants and two children will be presented. Two cases were fatal, thus illustrating the importance of rapid and definitive therapy early in the course. One of the fatal cases is the first case report in the otolaryngological literature of fatal hemoptysis in the newborn as a result of vascular anomalies associated with an absent left pulmonary artery. Eight cases of various causes, including small vessel vascular abnormality, trauma, recurrent pulmonary infection, and laryngotracheal papilloma, are discussed. The literature is reviewed, embryology is discussed, and a mechanism of death is theorized for the patient with the congenital vessel anomaly. Management considerations for hemoptysis in infants and children are discussed.

Acquisition of hearing aids and assistive listening devices among the pediatric hearing-impaired population.

Sufficient access to health care is of concern to the indigent population in the US and to their health care providers. This study was undertaken to elucidate the rate of the follow-up among lower socioeconomic hearing-impaired pediatric patients who had received a recommendation for hearing aids and/or assistive listening devices. Our question was, would the families' financial situation have a negative effect on the acquisition of hearing aids and assistive listening devices? Fifty patients, age 0-18 years, who had been seen in our clinic over 2 years were evaluated via a telephone survey. The survey consisted of seven questions, including whether or not the devices or aids were obtained, what type was purchased, where the device was being used, and the child's apparent performance with the device. Eighty-two percent of our patients were on TennCare, a state mandated Medicaid HMO system. Two-thirds of these TennCare patients are at or below the poverty level and the remaining one-third is either disabled or uninsurable according to the Aid for Dependent Children (AFDC) with indeterminate income. In addition the TennCare organization did not cover hearing amplification equipment for these children. The study showed that the majority of the patients did follow-up as recommended. Furthermore, this equipment is easily obtainable for the pediatric indigent population due to financial resources available in the community outside the mandated Medicaid system.

Combinations of alfalfa hay and wet corn gluten feed in limit-fed growing diets for beef cattle.

Two experiments were conducted to evaluate the effects of alfalfa hay (AH) and wet corn gluten feed (WCGF) combinations on ADG and gain efficiency of cattle limit-fed growing diets. In Exp. 1, crossbred beef steers (n = 220; initial BW = 262 kg) were limit-fed diets consisting of steam-flaked corn and 40% WCGF (DM basis) with 0, 10, or 20% ground AH (0AH, 10AH, and 20AH, respectively). A fourth diet containing 20% ground AH and steam-flaked corn served as a control. All diets were fed once daily at 1.8% of BW (DM basis). Growing period ADG, gain efficiency, and dietary NE calculated from performance data decreased linearly (P < 0.01) with addition of AH to diets containing WCGF. Rate of DMI increased linearly (P < 0.05) with AH addition to diets containing WCGF. Following the growing period, steers were finished on a common diet offered ad libitum. Gain efficiencies during the finishing period were higher (P < 0.05) for steers fed the 20AH diet than for steers fed the control diet. In Exp. 2, crossbred beef heifers (n = 339; initial BW = 277 kg) were limit-fed diets containing steam-flaked corn with 10, 20, or 30% ground AH and 0, 40, or 68% WCGF in a 3 x 3 factorial arrangement, fed once daily at 1.6% of BW (DM basis). An AH x WCGF interaction occurred (P < 0.05) for growing period ADG and gain efficiency. Increasing AH or WCGF decreased cattle ADG, gain efficiency, and dietary NE with the exception of heifers fed 30AH/40WCGF, which had ADG that did not differ (P > 0.10) from that of heifers fed 20AH/0WCGF or 30AH/0WCGF, and which had greater gain efficiencies (P < 0.05) than heifers fed 30AH/0WCGF. Rate of DMI increased linearly (P < 0.01) with increasing AH and decreased linearly (P < 0.01) with increasing WCGF. Heifers were finished on diets containing 33% WCGF with 0 or 0.5% added urea (DM basis) offered ad libitum. Increasing WCGF in growing diets tended (linear, P < 0.10) to increase finishing ADG and gain efficiency, whereas increasing AH decreased (linear, P < 0.05) kidney, pelvic, and heart fat, and the percentage of carcasses grading USDA Prime. Urea tended to increase ADG (P < 0.10), but decreased (P < 0.04) the percentage of carcasses grading USDA Choice. Results suggest that the value of WCGF relative to steam-flaked corn in limit-fed growing diets might be improved in diets containing 30% AH relative to diets containing 10 or 20% AH.

Pediatric hospitals' and physician strategies for the 21st century.

Changes in market-driven health care economics are rapid and of great magnitude. This report describes a study of some of these changes in regard to children's health issues. We used a survey tool to assess long-range plans (next 10 years) and marketing strategies for major free-standing children's hospitals in different regions of the United States. We then used these assessments to draw conclusions about the impact of the plans and strategies on the practice of pediatric physicians and their workforce requirements. This may allow pediatric specialists and their programs to develop strategic plans and to take actions to contend with these market-driven economic changes. The tool was a questionnaire mailed to chief executive officers of 30 randomly chosen but geographically well-distributed children's hospitals. Seventeen children's hospitals responded (57%), providing information concerning each hospital and its current medical economic environment. The data were analyzed and trends were then identified from their responses. All institutions in this study expected to have fewer physicians on staff in the future. These institutions plan: (1) to improve quality and (2) to reduce costs. Quality will be improved by utilizing Continuous Quality Improvement (CQI) and/or Benchmarking to Best Practices, both of which encourage physicians to follow standardized treatment protocols. Costs will be reduced by decreasing hospital staff size. Some children's hospitals have merged or will merge with larger, full-service adult hospitals, but most plan to remain autonomous. Many expect a continued decrease in revenues, and almost all expect to downsize both bed number and staff. Restructuring will reduce the number of specialists, particularly in the fields of hematology-oncology, psychiatry, endocrinology, nephrology, and cardiology, and will also reduce the number of surgical specialists. The administrators predicted that more nurse practitioners will be employed at these hospitals in the 21st century, serving the role of physician extenders, if not physician substitutes. To ensure their own survival, physicians must develop their own strategic plans as have the hospitals in which they practice. Optimally they should embrace those activities that parallel their hospital's efforts such as Quality Improvement (CQI, Benchmarking), cost containment, and practice mergers.

Eagle's syndrome: a reappraisal.

W.W. Eagle in 1937 described two patients with elongated styloid processes, cervico-facial pain and a history of pharyngeal trauma. Selected case reports are used to illustrate the spectrum of Eagle's Syndrome. An analysis of the prevailing theories of etiology and causation is undertaken correlating anatomy, embryology, and physiology to derive a clearer understanding of Eagle's Syndrome.

Head and neck manifestations of langerhan's cell histiocytosis in children: a 46-year experience.

OBJECTIVE: Langerhan's cell histiocytosis (LCH) is an uncommon disease characterized by abnormal proliferation of polyclonal Langerhan's cells, most commonly presenting with head and neck manifestations. This is a report of a series of patients with LCH at St. Jude Children's Research Hospital over a 46-year period. The purpose was to examine the head and neck presentations of LCH, their treatments and outcomes, and to compare with other previously reported series. METHODS: This was a retrospective study of all patients with a diagnosis of LCH who presented to St. Jude Children's Research Hospital, Memphis, TN between 1962 and 2008. Patients who presented with an initial diagnosis of LCH but were later determined to not fit the diagnostic criteria were excluded from the study. IRB approval was obtained and a chart review was conducted to collect data regarding demographics, tumor site(s) and manifestations, pathology, treatment, surgical procedures, and outcomes. This data was compiled and compared to previously published results. RESULTS: Eighty-eight cases of LCH with at least one head and neck manifestation were diagnosed during the period studied. There were 54 males and 34 females, with an average age of onset of 4.1 years. The most common lesions were those involving the skull (44.3%) and cervical lymph nodes (40.9%). The most common treatment was chemotherapy (80%) and corticosteroids (64%), with vinblastine being the most common chemotherapeutic agent. Surgical intervention occurred in 39% of cases. Total mortality due to LCH was 9.1%. The results were found to generally concur with other previously published studies. CONCLUSION: This study represents the largest series of head and neck manifestations of LCH reported to date. Although LCH is a rare disease, it often mimics other common head and neck pathologies and therefore requires a high index of suspicion. Biopsy is required for definitive diagnosis and the mainstay of treatment is chemotherapy.

What happens to patients who frequently harm themselves? A retrospective one-year outcome study.

Between 1982 and 1987, 42 patients were selected who had been admitted three times in one week to the Regional Poisoning Treatment Centre in Edinburgh for the treatment of deliberate self-harm. Most (87%) of these admissions related to drug overdose. Most of the patients were young, unemployed, lacking a partner and from the low social classes. Three-quarters of the sample had at least one known conviction and more than a third were known to have served a prison sentence. There was only one patient in whom mental illness was thought to be relevant to the episode of self-harm, although five patients were mildly mentally handicapped. Case notes were reviewed to assess the outcome one year after the index admission. None of the patients had committed suicide, although 36 repeated self-harm during the follow-up year.

Accidental modification of Attwood's stain on decalcified preparations, yielding improved differentiation between woven and lamellar bone.

In an earlier report, a modification of the Attwood's stain was used to study sections of decalcified bone and the procedure aided in the distinction between lamellar and woven bone. Lamellar bone showed preferential affinity for phloxine (red) yet resisted differentiation with tartrazine (yellow), while woven bone stained well with tartrazine. In addition, osteoid seams were identified by their affinity for tartrazine. Differential staining was abolished by pretreatment with periodic acid. Recently, accidental use of potassium permanganate in an acidified medium of pH 1.94, instead of pH 6.44, yielded unexpected results with a much sharper, consistent distinction between woven and lamellar bone. This probably reflects changes in the noncollagenous, interfibrillary matrix proteins of bone, but the exact reason(s) is not known. In our view, this serendipitous modification represents an advance over the previous method, and has allowed us to study the structure of bone in some of the systemic disorders, e.g., osteogenesis imperfecta, with greater ease than before. This method does, however, suffer the disadvantage of interfering with the intensity of the staining of any cartilaginous matrix, and the original application is still of value.

Is old-fashioned electroconvulsive therapy more efficacious? A randomised comparative study of bilateral brief-pulse and bilateral sine-wave treatments.

In-patients suffering from major depressive disorder (endogenous subtype) were randomly allocated to treatment by either traditional ECT with constant-voltage modified sine-wave stimuli (n = 17) or modern, constant-current brief-pulse ECT (n = 14). All treatments were bilateral and monitored by simultaneous recording by EEG. The severity of depressive illness was assessed the day before treatment, after three treatments, and seven days after the last treatment. The improvement and final depression rating scores, the likelihood of recovery, and the average number of treatments received were virtually identical in the two groups. We concluded that the policy of bilateral suprathreshold modern ECT monitored by EEG is as efficacious as traditional ECT.

Loss of breast milk nutrients during tube feeding.

Nutrient losses from banked breast milk during tube feeding were determined from laboratory models. Large fat losses occurred, continuous feeding giving rise to significantly greater losses than bolus feeding. More fat loss occurred with low flow rates and there were also significant protein losses.

Fire in the operating room during tracheostomy.

BACKGROUND: Fire in the modern operating room is still a constant danger today despite the usual absence of the historically explosive anesthetic gases, cyclopropane, and ether. During a tracheostomy, three conditions are present that will support an explosive or combustive event: heat, fuel and oxygen. METHODS: We report three routine tracheostomy cases during which a fire was ignited. One patient had a minor burn during the cauterizing of a bleeding vessel. There was a visible flame in all cases and, in one case, the cloth drapes ignited. There were no serious airway injuries to any patients and all had a complete and uneventful recovery. We duplicated the same conditions in our laboratory. RESULTS: We discuss each case and give effective techniques to prevent ignition during surgery in the future. For our study, we reproduced flames in a cadaver trachea using halothane and the electrocautery in an oxygen-rich environment. We describe a protocol that has effectively prevented tracheostomy fires in our institution and may decrease the risk during other procedures as well. Also, we reviewed the literature to provide insight into the magnitude of the problem. CONCLUSIONS: High-oxygen concentration, the presence of fuels such as suture and tissue, and an igniting spark from the electrocautery device produce the proper conditions for a fire during tracheostomy. Taking the proper precautions can minimize if not eliminate this risk.