Autologous platelet scintigraphy and clinical outcome of splenectomy in immune thrombocytopenia: A systematic review and meta-analysis.

Autologous platelet sequestration pattern is associated with post-splenectomy platelet response in patients with immune thrombocytopenia (ITP). However, published results are contradictory, and have not been systematically reviewed. Our aim is to systematically review and meta-analyse the association between sequestration pattern and post-splenectomy platelet response. Articles were selected from MEDLINE when they a) included ITP patients, b) performed scintigraphy, and c) included post-splenectomy platelet response. The 23 included studies (published between 1969-2018) represented 2966 ITP-patients. Response to splenectomy occurred most frequently in patients with a splenic pattern (87.1 % in splenic versus 47.1 % in mixed and 25.5 % in hepatic patterns). A pooled analysis of 8 studies showed an odds ratio of 14.21 (95 % CI: 3.65-55.37) for platelet response in the splenic versus the hepatic group. Our findings indicate that a splenic sequestration pattern is associated with better response after splenectomy. Platelet sequestration patterns may be useful in the clinical decision-making regarding splenectomy.

The practice of physical medicine and rehabilitation in subSaharan Africa and Antarctica: a white paper or a black mark?

AIM: The medical specialty of physical medicine and rehabilitation (PM&R) has had a proven impact on persons with disability and on healthcare systems. Documents such as The White Book on Physical and Rehabilitation Medicine in Europe have been important in defining the scope of practice within various regions. However on some continents the practice has not been well defined. The aim of this paper was to explore the practice of PM&R in subSaharan Africa and Antarctica. METHODS: Medline searches, membership data searches, fax survey of medical schools, Internet searches, and interviews with experts. RESULTS: The continents are dissimilar in terms of climate and government; However, both Antarctica and subSaharan Africa have no PM&R training programs, no professional organizations, no specialty board requirements, and no practicing physicians in the field. Since there are no known disabled children on Antarctica and adults are airlifted to world-class health care, the consequences of this deficit are minimal there. However the 788,000,000 permanent residents of subSaharan Africa including approximately 78 million persons with disability are left unserved. CONCLUSIONS: Antarctica is doing fine. Africa is in a crisis. Local medical schools, hospitals doctors, and persons with disability; along with foreign volunteers, aid groups, and policymakers can impact the crisis. However government specifically national ministries of health is ultimately responsible for the health and wellbeing of citizens.

The quality of life of ventilator-assisted children.

Although an increasing number of ventilator-assisted children are being cared for in the home, few studies in the literature evaluate the resulting quality of life from multiple informant perspectives. A questionnaire was used to gather data on 38 ventilator-assisted children from the children themselves and their caregivers about activities of daily living, measures of independence, and psychological state. The majority (79.4%) of ventilator-assisted children were perceived as either satisfied or very satisfied with how they spent their time, and most (77.1%) were rated as having an excellent or good emotional adjustment to the need for mechanical ventilation. Children older than 12 years were reported to be significantly less satisfied with their daily activities than the younger children, but greater activity levels correlated with greater levels of total satisfaction. A higher total satisfaction did not correlate with the age at onset of assisted ventilation, or the amount of time spent in other activities. Adolescents and primary caregivers had similar responses to most activity and independence parameters on the survey. There were no significant differences in parents' perceptions of the adolescents' emotional status as compared to the adolescents' self- assessment of their emotional status in any parameter.

Recurrent and migratory reflex sympathetic dystrophy in children.

Reflex sympathetic dystrophy is a syndrome characterized by superficial pain and tenderness associated with swelling, vasomotor instability, and dystrophic changes of the skin. In children, it is rarely reported and is felt to have a more benign and self-limited course. This case illustrates that, in children, reflex sympathetic dystrophy can occur without any previous history of trauma, and may be recurrent and migratory. A review of the literature is included. An 11-year-old girl, with no history of trauma, presented in 1992 with spontaneous onset of right leg pain. She was diagnosed with reflex sympathetic dystrophy, and she was treated unsuccessfully with oral medications. Her symptoms then resolved in 2 weeks after receiving epidural anaesthesia and aggressive physical therapy. Over the next 5 years, she presented to the paediatric rehabilitation clinic three times with recurrent RSD in her bilateral arms. The first two times were refractory to conservative management and resolved with four stellate ganglion blocks. The third recurrence persisted with three stellate ganglion blocks and resolved with gabapentin.

Functional outcome of paediatric stroke survivors.

OBJECTIVE: To examine the medical and functional outcome of paediatric stroke survivors. PATIENTS: Patients aged 1 month to 18 years diagnosed with stroke over a 10 year period. MAIN OUTCOME MEASURES: Discharge functional outcome data were collected by reviewing therapy, nursing, and other chart notes relating to specific functional tasks. Current functional information, living situation, school placement, and medical outcome data were obtained in the telephone survey. RESULTS: Fifty patients responded. The mean age at event was 8.0 years (range: 7 months to 17 years, 7 months). The mean follow-up time was 70 months. Diagnoses included: haemorrhagic (30%), thrombotic/embolic (46%), and undiagnosed (24%). At follow-up, 76% of the patients were independent in all activities of daily living (ADL), compared to 64% at hospital discharge. Younger age at onset, female gender, history of cardiac disease, and presentation with hemiparesis were significant risk factors for dependence in ADL (p < 0.05), while thrombotic/embolic aetiology demonstrated a trend (p = 0.06). Eighty-four per cent were independent in mobility, compared to 74% at discharge. Forty per cent of the patients had speech and language deficits. Of the school age children, only 50% were in a regular classroom. CONCLUSIONS: Children and adolescents who survive stroke have good outcome for mobility and ADL skills, but more difficulty with language and cognitive recovery. Functional recovery is maintained after discharge, and functional gains occur over time with very little evidence of functional regression. Comorbidities are relatively low. All children in the group returned to a home setting.

Perinatal spinal cord injury requiring long-term home mechanical ventilation: a report of four cases.

Although perinatal spinal cord injury has been discussed in detail in the literature with respect to aetiology and diagnosis, few studies address long-term outcome, and none address the outcome of long-term home mechanical ventilation in this population. The four patients reported here with perinatal spinal cord injury have used home mechanical ventilation for time periods ranging from 6 to 17 years, with varying results. Their courses with respect to respiratory, neuromuscular, neuropsychological, and nutritional issues are described with reference to the current literature. It is believed that, with the advent of organized home mechanical ventilation programmes and increased acceptance of this technology by the lay public and medical community, the prognosis of the child with perinatal spinal cord injury requiring assisted ventilation is much improved, and may be more positive than the literature currently suggests.

Parental report of pediatric tracheostomy care.

OBJECTIVE: There are little data on the actual care given pediatric tracheostomy patients in their homes. Information on the use of supplies and on techniques and frequency of care is valuable for a better understanding of the needs of this population. DESIGN: Questionnaires were distributed by mail or at clinic visits from May 1995 to June 1996 to a convenience sample of tracheotomized patients at the University of Michigan Pediatric Physical Medicine and Rehabilitation clinic. SETTING: Tertiary care clinic. RESULTS: Clean technique for suctioning was reported by 96.7% of subjects and the rest reported sterile technique. Fifty percent of subjects reported reusing suction catheters. Cleaning solutions used to clean suction catheters for reuse varied. Tracheostomy tube reuse was reported by 55% of subjects. Sixty percent of those who reused tracheostomy tubes had had pneumonia within the previous year, whereas only 25% of those who never reused the tracheostomy tube had pneumonia in the same time period. CONCLUSIONS: Suctioning frequency, suction catheter, and tracheostomy tube reuse and cleaning methods are variables that warrant further investigation of safety and efficacy.

Bowel management in children and adolescents with spinal cord injury.

There is little in the literature regarding bowel management in children and adolescents with spinal cord injuries (SCI). This study was undertaken to examine specific patterns of bowel care, individual levels of satisfaction with bowel management, the incidence of incontinence in this population, and effects on lifestyle because of time commitment and dependence in bowel management. Surveys were sent to all persons (n = 45) under age 19 with a diagnosis of SCI who had received care at our medical center since 1985. Thirty-one subjects (69 percent) returned the surveys. The average age at injury was 8.1 years, with an average follow-up period of 3.9 years. Fifty-five percent were individuals with tetraplegia and 77 percent had a complete injury (ASIA Class A). A bowel management program, including medications or manual manipulation, was required for 81 percent of the subjects; only two were independent in their bowel management. Over half of the subjects performed evening bowel care and over half performed their care daily. Digital stimulation tended to be used more commonly by younger children. Medications, either oral, rectal, or both, were used by 88 percent. Sixty percent of the subjects reported they were completely or very satisfied with their bowel management. About half the subjects had limited freedom because of their bowel programs, which caused some dissatisfaction. Sixty-eight percent reported occasional or frequent interference with school activities because of their bowel programs. No correlation was found between bowel accidents and satisfaction with bowel management, despite the fact that almost 84 percent of the children reported at least rare accidents. Lifestyle limitations, bowel accidents, dependence in bowel management, and subject and family dissatisfaction continue to be significant problems for children and adolescents with SCI.

Bladder management in patients with pediatric onset neurogenic bladders.

Our objective was to determine which clean intermittent catheterization (CIC) methods and supplies were used by patients with pediatric onset neurogenic bladders and to relate methodology and materials to reported urinary tract infections. Data were collected via questionnaires distributed by mail and at clinic visits at our university tertiary care outpatient pediatric rehabilitation clinic. Questionnaires were given to 165 patients. Fifty-nine percent were returned (68 patients with myelomeningocele, 27 with pediatric onset spinal cord injury (SCI) and two with other diagnoses). Mean age was 12 years (range 1-27). Fifty-four percent of patients participated in their own CIC. Only two percent used sterile catheterization technique, whereas 98 percent used CIC. A sterile catheter was employed with clean technique by 22 percent. Catheters were reused by 76 percent. Subjects used a wide ranging number of catheters per month, with a median of 5.3. There was no correlation between the number of urinary tract infections (UTIs) per year and the type of catheter used or the use of prophylactic antibiotics. Compared with patients with myelomeningocele, subjects with SCI were significantly more likely to use sterile catheters (p = 0.04), > 10 catheters per month (p = 0.01) and gloves (p < 0.001). Subjects who used gloves or more catheters were more likely to experience UTI. These data suggest that clean reused supplies are not related to an increased likelihood of UTI and should be considered a way to lower costs in these populations.

The use of methylphenidate in paediatric traumatic brain injury.

Methylphenidate (MPH) has been used safely and effectively for many years in children for the treatment of attention deficit disorder with hyperactivity (ADHD). Behavioural and cognitive sequelae to traumatic brain injury (TBI) have features in common with ADHD, and MPH has been reported to be an effective treatment in adults with TBI. There is little literature documenting the efficacy of MPH in children with TBI. A chart review of 10 children who had sustained TBI and were subsequently treated with MPH was performed. Nine of the cases resulted from motor-vehicle accidents, and one from a fall. The severity of TBI ranged from mild to severe. In eight of the cases, MPH was used for cognitive and behavioural problems, and in two cases it was used to stimulate minimally responsive patients. The introduction of MPH improved cognitive function, behaviour and arousal as measured by parental and teacher reports, evaluation by in-/outpatient rehabilitation team, and/or neuropsychometric testing. MPH appears to be an effective treatment for post-TBI cognitive and behavioural sequelae in children, as well as improving arousal in the minimally responsive brain-injured child. Further prospective research is necessary to clarify the role of MPH in children with TBI.

Spondyloepiphyseal dysplasia congenita with ventilator dependence: two case reports.

Home mechanical ventilation has recently become feasible in a number of conditions. Several small series and case reports mention respiratory insufficiency or failure in a subset of patients with spondyloepiphyseal dysplasia congenita. Two cases of successful home mechanical ventilation in children, now 7 years of age, who have spondyloepiphyseal dysplasia congenita and respiratory insufficiency, are reported. Novel rehabilitation planning has facilitated the growth and development of these children.

Home mechanical ventilation of children.

The medical records of 89 ventilator-assisted children followed at the University of Michigan Medical Center from 1978 to 1993 were reviewed. The status of these children was remarkably stable. Parameters of communication, nutrition, education, and mobility changed very little over time, and fewer than half had to be re-admitted. Children aged 9 to 12 years had the most nursing hours; in terms of diagnosis, those with spinal cord injury and bronchopulmonary dysplasia had the most. The younger children had the longest initial hospital stay and the most re-admissions. The authors conclude that appropriate rehabilitation during the initial hospitalization can minimize later changes, instability and rehospitalizations, and that careful follow-up and periodic evaluation can improve the patients' health and function.

Incidence of maxillofacial involvement in arthrogryposis multiplex congenita.

PURPOSE: This study determined the incidence of maxillofacial involvement in patients diagnosed with arthrogryposis multiplex congenita (AMC). PATIENTS AND METHODS: Twenty-three patients were evaluated by the pediatric physical medicine and rehabilitation, orthopedic surgery, and pediatric oral and maxillofacial surgery departments. Any patient in whom the diagnosis of AMC was in doubt was excluded from the study. All patients with limited mandibular function underwent computed tomography (CT) examination of their temporomandibular joints (TMJ). The results of physical therapy were followed. RESULTS: Five of the 23 patients diagnosed with AMC were found to have maxillofacial involvement, eg, presence of cleft palate, Robin-like sequence, high-arched palate, open-bite deformity, facial muscle weakness, esophageal dysfunction, and limited mandibular opening. No TMJ abnormalities were found by CT scan. Physical therapy was used for treatment of the limited opening, but relapse occurred quicky after therapy was discontinued. CONCLUSION: The incidence of maxillofacial findings is similar to that of most other reports. Treatment involves surgical correction of abnormal anatomy when possible (ie, cleft repair), symptomatic management (ie, esophageal dysfunction), and physical therapy.

Percutaneous feeding tube placement in pediatric patients: immediate and 30-day results.

PURPOSE: To evaluate fluoroscopically guided percutaneous feeding tube placement in pediatric patients. MATERIALS AND METHODS: Sixty-one procedures were performed. Periprocedural care protocol was changed after patient nine. Forty-eight-hour and 30-day outcomes were assessed. RESULTS: Almost 97% of procedures were successful. The 48-hour major and minor complication rates were 1.9% and 9.6%, respectively, after the initial nine procedures. Risk factors for early complications were the use of the initial care protocol (P < .01) and patient weight below the 50th percentile (P < .05). Major and minor 30-day complication rates were 8.3% and 12.0%, respectively. Risk factors for delayed complications were placement of a gastrojejunostomy tube rather than a gastrostomy tube (P < .05) and immunosuppression (P < .05). CONCLUSION: Percutaneous feeding tubes can be placed in children with a high rate of technical success. Optimal results require attention to periprocedural care. Morbidity is common during the first month of tube use.

Home mechanical ventilation in mitochondrial encephalomyopathy syndrome.

Long-term home mechanical ventilation of children has only recently become more practically feasible and ethically acceptable by the medical community. It has been particularly controversial in cases of degenerative myopathies in which quality of life has been questioned. There are no reports in the literature of long-term home mechanical ventilation of a child with mitochondrial encephalomyopathy (MELAS) syndrome despite the many descriptions of possible etiologies of the concomitant respiratory failure. The patient reported here has used home mechanical ventilation for 6 years with few medical complications, no hospitalizations in the past 3 years, and increased function in activities of daily living. Despite the ill-defined nature of the disease and uncertain prognosis, we believe that long-term home mechanical ventilation of children with early onset MELAS syndrome is a viable option for both patients and their families and results in overall improvement in quality of life for the patient.

Rachitic syndrome after disodium etidronate therapy in an adolescent.

A 12-year-old patient with a severe traumatic brain injury developed heterotopic ossification (HO) with rapidly decreasing range of motion in multiple joints despite intensive passive range of motion exercises and the use of nonsteroidal antiinflammatory drugs (NSAIDs). His alkaline phosphatase was markedly elevated. Etidronate, 20mg/kg/d was used to control the ossification. After 7 months of continual etidronate use, the patient developed periarticular pain with widened growth plates, suggesting a rachitic syndrome. Serum laboratory panel including calcium, phosphorous, alkaline phosphatase, and vitamin D were normal. After 3 months off etidronate, radiological studies showed growth plate calcification, though not before development of bilateral slipped femoral capital epiphyses. Further improvement and resolution of all complications occurred 5 months after discontinuation of etidronate. Rachitic syndromes secondary to didronel use in a clinical setting has not been previously reported. Other possible causes for a rachitic syndromes were not present. Alternate treatments for children with or at risk for HO should be considered.

Hyperthermia associated with baclofen withdrawal and increased spasticity.

Rapid baclofen withdrawal is known to cause markedly increased spasticity, but high fever associated with this complication has not been reported. We describe a 13-year-old boy with sensory incomplete C1 quadriplegia two years after injury who was on 200mg of baclofen per day for spasticity. Concerns about adverse side effects prompted tapering of his baclofen. Severely increased spasticity was noted with associated hyperthermia to 107 degrees F after the dosage was gradually decreased. Sepsis work-up was negative, head computed tomography scan was unchanged, and electroencephalogram showed no epileptiform activity. Cooling blankets, intravenous diazepam, and return of baclofen to 160mg per day decreased spasticity and normalized body temperature without recurrence of hyperthermia. Possible fever etiology is the hypermetabolic state associated with the acute return of spasticity.

Risk factors for heterotopic ossification in children and adolescents with severe traumatic brain injury.

Previous reports on the incidence of heterotopic ossification (HO) in children and adolescents with traumatic brain injury (TBI) have ranged from 3% to 20%. These studies mention HO briefly or address patient cohorts with coma more than three months. We reviewed the medical records of 90 children younger than 19 years old, who were comatose more than 24 hours after TBI. Mean followup was 33.1 months (range = 2 to 128 months). Incidence of HO was 14.4% (n = 13). One to five sites were involved, with hip and knee most frequent, followed by shoulder, elbow, and nonjoint sites. Children with HO were older than 11 years (relative risk [RR] = 18.85, confidence interval [CI] = 3.7, 95.7), had a greater length of coma (RR = 7.22, CI = 1.4, 37.1), and had a greater associated risk for poor functional outcome (RR = 2.89, CI = 1.02, 7.9) compared to those without RO. Multiple limb fractures showed a trend toward association with increased incidence of HO. Other factors, including gender, history of skull fracture, brain CAT scan findings, and presence of spasticity, were not predictive of HO. Eleven children were treated with etidronate, four with nonsteroidal antiinflammatory drugs, and one with surgery. Only three children had residual functional impairments attributed to the presence of HO.

Characteristics of pediatric rehabilitation training offered by physical medicine and rehabilitation residencies.

Pediatric physiatry is a growing subspecialty. A survey was designed to determine the nature of pediatric rehabilitation training in physical medicine and rehabilitation residency programs in the United States. Sixty-five programs (93%) responded. More than three quarters of the programs required three months or more in pediatric rehabilitation, usually in the HO-III or HO-IV year. Forty-two percent of the programs had an even balance of inpatient and outpatient clinical opportunities. Approximately half offered exposure to a separate pediatric rehabilitation ward, and 82% placed residents in a general pediatric rehabilitation clinic. Advanced training has become more widespread in pediatric rehabilitation, with 46% of the residency programs having preceptors with board certification in pediatrics and physiatry or fellowship training, and 42% of the programs responding offering advanced training opportunities in pediatric rehabilitation.