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Children with severe physical disabilities frequently have respiratory problems which affect their quality of life (QOL). They commonly stem from central nervous system dysfunctions and/or severe motor disabilities, and consist of various impairments deriving primarily from central and motor dysfunctions, such as dysmyotonia, deformation, dysphagia, and gastro-esophageal reflux (GER), which often influence each other and result in respiratory insufficiency without adequate interventions. Aging is also an important factor to worsen respiratory involvements and to change their underlying pathophysiology gradually, even if the primary cause of the disability is non-progressive. A key to effective treatment and improvement of their QOL is to understand the pathophysiology. Evaluation is primarily based on regular physical examination. Other examinations include blood sampling to examine inflammation, nutritional state and blood gas analysis, round-the-clock SpO2 and TcPCO2 or EtCO2 monitoring, chest X ray and computed tomography, polysomnography, laryngoscopy, bronchoscopy, evaluation of swallowing function, and evaluation of GER. Postural control, relaxation, respiratory physiotherapy, and treatment of dysphagia are the most important and common therapeutic procedures. Treatment of GER, treatment of upper airway obstruction, oxygen therapy, tracheostomy, or mechanical ventilation may be needed. It is also important to take into account the possibility that the treatment procedures themselves could affect the patient's QOL. Counseling should be performed before and throughout the treatment process about how patient's and family's everyday life will be influenced positively and negatively by the treatment, and a multidisciplinary team should support all aspects of their needs.
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PMID:[Treatment of respiratory disturbance in children with severe physical disabilities to improve their quality of life]. 1275 51

The purpose of this study is to review our initial experience with a minimally invasive approach to manage symptomatic epiphrenic esophageal diverticula. Five patients with symptomatic epiphrenic esophageal diverticula underwent surgical management between August 1997 and December 2002. All patients complained of dysphagia; had experienced symptoms for at least 12 months; and were evaluated preoperatively by a barium esophagram, esophagogastroduodenoscopy, and esophageal manometry. The epiphrenic esophageal diverticula measured 5 cm or less in all patients. Manometry demonstrated esophageal dysmotility in three patients. A minimally invasive technique was completed in all five patients. Four patients underwent laparoscopic diverticulectomy and myotomy including a concomitant Toupet fundoplication, and one patient underwent thoracoscopic diverticulectomy and myotomy. The mean operative time was 245 minutes (range 175-334). The longest operative time was for the thoracoscopic procedure. The estimated blood loss was minimal (range 30-100 cm3). The laparoscopic patients had a mean postoperative length of stay of 2.75 days (range 2-4) and the patient undergoing a thoracoscopic approach was discharged on postoperative day 6 due to a history of lung disease and home oxygen requirements. There were no other postoperative complications. After a mean follow-up of 16.2 months (range 3-36) all patients are asymptomatic. Short-term follow-up after our initial experience with minimally invasive approaches for epiphrenic esophageal diverticula demonstrates that thoracoscopic and laparoscopic approaches are feasible; safe; and effectively alleviate dysphagia, regurgitation, and other associated symptoms. Long-term outcomes should be monitored during the evolution of these novel minimally invasive techniques to ensure outcomes comparable to those of a transthoracic open approach.
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PMID:Minimally invasive management of epiphrenic esophageal diverticula. 1285 2

Botulism has been recognized as a clinical entity in foals since the 1960s. Also known as "Shaker foal" disease, the toxicoinfectious form of botulism affects foals, with the highest incidence in the United States seen in Kentucky and the mid-Atlantic region. The disease is characterized by progressive muscular weakness caused by the action of botulism neurotoxin at cholinergic neuromuscular junctions. Increased number of episodes and duration of recumbency, muscular trembling, and dysphagia are seen in affected foals. Left untreated, the disease can be rapidly fatal, with death occuring secondary to respiratory muscle paralysis within 24 to 72 hours of the onset of clinical signs. Very mildly affected foals can survive with minimal treatment Despite advances made in treatment of these foals, including administration of botulism antitoxin early in the course of the disease, there is still an impression that the disease carries a high mortality rate. The purpose of this study was to evaluate outcome in 30 foals <6 months of age diagnosed with botulism between 1989 and 2002 at the George D. Widener Large Animal Hospital, New Bolton Center. Two foals were euthanized for economic reasons early in the disease course, and I died while being treated. Survival of treated cases was greater than 96%. Approximately 50% of the cases required oxygen therapy, whereas 30% required mechanical ventilation. All foals, excepting 1 mildly affected foal, received botulism antitoxin. Mean duration of hospitalization was 14 days. With appropriate treatment, foals with botulism have a high survival rate.
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PMID:Botulism in foals less than 6 months of age: 30 cases (1989-2002). 1452 38

Dysphagia is a common finding in infants and children with neuromuscular disabilities. Dysphagia may be developmental, as in the preterm infant, transient, chronic, or progressive. The evaluation of dysphagia must take into account the age of the patient and typical development of feeding and swallowing for that age. The typical abilities seen in neonatal, early infancy, later infancy and early childhood periods vary in sensorimotor skills and feeding efficiency. In addition to knowing the substrate of expected skills by age, knowledge of the neurophysiology of feeding and swallowing is essential to diagnosis. Each physiologic phase of deglutition: oral, pharyngeal, and esophageal can present with symptoms of dysphagia that can guide investigation. Common symptoms of dysphagia include generalized feeding difficulty such as poor efficiency, food refusal and failure to thrive. Specific symptoms include tongue thrust, choking, cough, and oxygen desaturation. The possibility of dysphagia can be identified through a thorough feeding history. Examination initially includes the infant's muscle tone and posture in the head, neck and body. Anomalies of structures of the head and neck must be identified and examined for their effect on function. Next, examination of oral structures for reflexes, tongue movements, and symmetry will identify neurologic abnormalities. Observation of feeding is essential and will reveal signs of dysphagia. Aspiration in the infant can present without specific signs. Respiratory abnormalities or Gastroesophageal reflux can be identified during history or examination. Investigation of dysphagia most commonly includes videofluoroscopy, endoscopy, and ultrasonography. The management of dysphagia requires an individualized approach and will include neurologic, respiratory, nutritional and possibly gastrointestinal management. Six broad areas are identified that must be considered in the management of dysphagia in infants and children. They include: normalization of posture and positioning, adaptation of foods and feeding equipment, oromotor therapy, feeding therapy, nutritional support and management of associated disorders. A team of professionals will assist the parent and child in achieving pleasant feedings to foster appropriate growth and development.
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PMID:Investigation and management of dysphagia. 1499 57

Mitochondriopathies (MCPs) are either due to sporadic or inherited mutations in nuclear or mitochondrial DNA located genes (primary MCPs), or due to exogenous factors (secondary MCPs). MCPs usually show a chronic, slowly progressive course and present with multiorgan involvement with varying onset between birth and late adulthood. Although several proteins with signalling, assembling, transport, enzymatic function can be impaired in MCP, most frequently the activity of the respiratory chain (RC) protein complexes is primarily or secondarily affected, leading to impaired oxygen utilization and reduced energy production. MCPs represent a diagnostic challenge because of their wide variation in presentation and course. Systems frequently affected in MCP are the peripheral nervous system (myopathy, polyneuropathy, lactacidosis), brain (leucencephalopathy, calcifications, stroke-like episodes, atrophy with dementia, epilepsy, upper motor neuron signs, ataxia, extrapyramidal manifestations, fatigue), endocrinium (short stature, hyperhidrosis, diabetes, hyperlipidaemia, hypogonadism, amenorrhoea, delayed puberty), heart (impulse generation or conduction defects, cardiomyopathy, left ventricular non-compaction heart failure), eyes (cataract, glaucoma, pigmentary retinopathy, optic atrophy), ears (deafness, tinnitus, peripheral vertigo), guts (dysphagia, vomiting, diarrhoea, hepatopathy, pseudo-obstruction, pancreatitis, pancreas insufficiency), kidney (renal failure, cysts) and bone marrow (sideroblastic anaemia). Apart from well-recognized syndromes, MCP should be considered in any patient with unexplained progressive multisystem disorder. Although there is actually no specific therapy and cure for MCP, many secondary problems require specific treatment. The rapidly increasing understanding of the pathophysiological background of MCPs may further facilitate the diagnostic approach and open perspectives to future, possibly causative therapies.
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PMID:Mitochondriopathies. 1500 63

Hydrogen peroxide is an oxidising agent that is used in a number of household products, including general-purpose disinfectants, chlorine-free bleaches, fabric stain removers, contact lens disinfectants and hair dyes, and it is a component of some tooth whitening products. In industry, the principal use of hydrogen peroxide is as a bleaching agent in the manufacture of paper and pulp. Hydrogen peroxide has been employed medicinally for wound irrigation and for the sterilisation of ophthalmic and endoscopic instruments. Hydrogen peroxide causes toxicity via three main mechanisms: corrosive damage, oxygen gas formation and lipid peroxidation. Concentrated hydrogen peroxide is caustic and exposure may result in local tissue damage. Ingestion of concentrated (>35%) hydrogen peroxide can also result in the generation of substantial volumes of oxygen. Where the amount of oxygen evolved exceeds its maximum solubility in blood, venous or arterial gas embolism may occur. The mechanism of CNS damage is thought to be arterial gas embolisation with subsequent brain infarction. Rapid generation of oxygen in closed body cavities can also cause mechanical distension and there is potential for the rupture of the hollow viscus secondary to oxygen liberation. In addition, intravascular foaming following absorption can seriously impede right ventricular output and produce complete loss of cardiac output. Hydrogen peroxide can also exert a direct cytotoxic effect via lipid peroxidation. Ingestion of hydrogen peroxide may cause irritation of the gastrointestinal tract with nausea, vomiting, haematemesis and foaming at the mouth; the foam may obstruct the respiratory tract or result in pulmonary aspiration. Painful gastric distension and belching may be caused by the liberation of large volumes of oxygen in the stomach. Blistering of the mucosae and oropharyngeal burns are common following ingestion of concentrated solutions, and laryngospasm and haemorrhagic gastritis have been reported. Sinus tachycardia, lethargy, confusion, coma, convulsions, stridor, sub-epiglottic narrowing, apnoea, cyanosis and cardiorespiratory arrest may ensue within minutes of ingestion. Oxygen gas embolism may produce multiple cerebral infarctions. Although most inhalational exposures cause little more than coughing and transient dyspnoea, inhalation of highly concentrated solutions of hydrogen peroxide can cause severe irritation and inflammation of mucous membranes, with coughing and dyspnoea. Shock, coma and convulsions may ensue and pulmonary oedema may occur up to 24-72 hours post exposure. Severe toxicity has resulted from the use of hydrogen peroxide solutions to irrigate wounds within closed body cavities or under pressure as oxygen gas embolism has resulted. Inflammation, blistering and severe skin damage may follow dermal contact. Ocular exposure to 3% solutions may cause immediate stinging, irritation, lacrimation and blurred vision, but severe injury is unlikely. Exposure to more concentrated hydrogen peroxide solutions (>10%) may result in ulceration or perforation of the cornea. Gut decontamination is not indicated following ingestion, due to the rapid decomposition of hydrogen peroxide by catalase to oxygen and water. If gastric distension is painful, a gastric tube should be passed to release gas. Early aggressive airway management is critical in patients who have ingested concentrated hydrogen peroxide, as respiratory failure and arrest appear to be the proximate cause of death. Endoscopy should be considered if there is persistent vomiting, haematemesis, significant oral burns, severe abdominal pain, dysphagia or stridor. Corticosteroids in high dosage have been recommended if laryngeal and pulmonary oedema supervene, but their value is unproven. Endotracheal intubation, or rarely, tracheostomy may be required for life-threatening laryngeal oedema. Contaminated skin should be washed with copious amounts of water. Skin lesions should be treated as thermal burns; surgery may be required for deep burns. In the case of eye exposure, the affected eye(s) shod eye(s) should be irrigated immediately and thoroughly with water or 0.9% saline for at least 10-15 minutes. Instillation of a local anaesthetic may reduce discomfort and assist more thorough decontamination.
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PMID:Hydrogen peroxide poisoning. 1529 93

PRESENTING FEATURES: A 53-year-old man who had human immunodeficiency virus (HIV) presented to the Johns Hopkins Hospital with a 3-month history of increasing dysphagia, cough, dyspnea, chest pain, and an episode of syncope. His past medical history was notable for oral and presumptive esophageal candidiasis that was treated with fluconazole 6 months prior to presentation. Three months prior to presentation, he discontinued his medications, and his symptoms of dysphagia recurred. During that time he developed intermittent fevers and chills, progressively worsening dyspnea on exertion, and a cough productive of white sputum. He also reported a 40-lb weight loss over the past 3 months. On the day prior to presentation, he had chest pain and shortness of breath followed by weakness, dizziness, and a brief syncopal episode. He denied orthopnea, paroxysmal nocturnal dyspnea, lower extremity edema, jaundice, hemoptysis, hematemesis, melena, hematochezia, or diarrhea. There was no history of alcohol use, and he stopped smoking tobacco approximately 1 month previously. He smoked cocaine but denied injection drug use. The patient had never been on antiretroviral therapy and had never had his CD4 count or viral load measured. On physical examination, the patient was a thin, cachectic man who appeared older than his stated age. His vital signs were notable for blood pressure of 102/69 mm Hg, resting tachycardia of 102 beats per minute, resting oxygen saturation of 92% on room air, normal resting respiratory rate, and a temperature of 38.1 degrees C. His oropharynx was clear, with no signs of thrush or mucosal ulcers. His pulmonary examination was notable for diminished breath sounds in the lower lung fields bilaterally. Cardiac, abdominal, and neurologic examinations were normal. His skin was intact, with no visible petechiae, rashes, nodules, or ulcers. Laboratory studies showed a total white blood cell count of 3.2 x 10(3)/microL, with a total lymphocyte count of 330/microL, hematocrit of 30.2%, a serum sodium level of 129 mEq/L, and a serum lactate dehydrogenase level of 219 IU/L. The patient had an absolute CD4 count of 8 cells/mm3 and a HIV viral load of 86,457 copies/mL. His arterial blood gas on room air had a pH of 7.51, a PCO2 of 33 mm Hg, and a PO2 of 55 mm Hg. Electrocardiogram and serial serum cardiac enzymes were normal. A chest radiograph showed bilateral upper lobe patchy infiltrates with left upper lobe consolidation. Computed tomographic (CT) scan of the chest with contrast showed bilateral ground glass infiltrates with focal consolidation (Figure 1) and no evidence of pulmonary embolism. Induced sputum was negative for Pneumocystis carinii, fungi, or acid-fast bacilli. A bronchoalveolar lavage was performed. What is the diagnosis?
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PMID:Cases from the Osler Medical Service at Johns Hopkins University. Diagnosis: P. carinii pneumonia and primary pulmonary sporotrichosis. 1533 85

Photodynamic therapy (PDT) is a form of photochemotherapy requiring the simultaneous presence of a photosensitiser, activating light of the proper wavelength and molecular oxygen in order to produce a localised therapeutic effect thought to be due to high-energy singlet oxygen generation. Neither drug nor light alone are effective as therapeutic agents and thus PDT treatment methods should be looked upon as true, necessary, drug and device combinations ('systems'). Selectivity of treatment is imparted by a combination of factors, including accumulation of photosensitiser by the target lesion and targeted application of activating light. The most common systemic side effect of systemically administered photosensitisers is cutaneous photosensitivity of varying periods of time. Local toxicities depend on the area of treatment. Sources of light which have been used in PDT include lasers, arc lamps, light-emitting diodes and fluorescent lamps. PDT has been used for a wide variety of clinical applications. In 1995, the first PDT system, using porfimer sodium (Photofrin, Axcan Pharma, Inc.), lasers and fibre optic light delivery methods, developed by QuadraLogic Technologies, was approved in the US for endoscopic palliation of malignant dysphagia caused by oesophageal cancer. A topical PDT system, aminolevulinic acid HCL (Levulan Kerastick) and the large-area BLU-U PDT Illuminator, was developed by DUSA Pharmaceuticals, Inc. for the treatment of actinic keratoses of the face and scalp and approved in the US in 2000. Topical PDT has applicability to a wide variety of skin cancers and precancerous conditions. In 2001, Novartis launched the systemically administered verteporfin (Visudyne) laser-based PDT system in the US as the first pharmacologic treatment for age-related macular degeneration. Development programmes are continuing to investigate PDT for the potential treatment of a variety of diseases, yielding therapeutic results with minimal toxicity.
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PMID:Photodynamic therapy systems and applications. 1598 54

A 27-year-old male intravenous drug user presented to the Emergency Department of St James's Hospital with a 1-week history of progressive dysphasia, dysphagia and difficulty 'holding his head up' and 'keeping his eyes open'. He also complained of increasing weakness in his upper limbs, as a result of which he kept dropping things. He was on a methadone program but was using both intravenous heroin and cocaine at the time of presentation. Examination of his motor function revealed generalized hypotonia, hyporeflexia and reduced power in both upper limbs. No sensory loss was observed. Co-ordination was intact. The clinical picture of a proximal symmetrical descending weakness and an absence of sensory loss was suggestive of botulism. Clostridium botulinum is a spore-forming, obligate anaerobe. The three forms of human botulism are food-borne, wound and intestinal. A fourth man-made form is produced from aerosolized botulinum toxin and results in inhalational botulism. A little as 1 g of aerosolized botulinum toxin has the potential to kill 1.5 million people. Toxin is detected in serum or stool specimens in only approximately 46% of clinically diagnosed cases. Treatment involves supportive care and early passive immunization with equine antitoxin. Patients should be regularly assessed for loss of gag and cough reflex, control of oropharyngeal secretions, oxygen saturation, vital capacity and inspiratory force. When respiratory function begins to deteriorate, anticipatory intubation is indicated. Early symptom recognition and early treatment with antitoxin are essential in order to prevent mortality, and to prevent additional cases, it is important to ascertain the presence of similar symptoms in contacts of the patient and local public health officials must be notified as one case may herald an outbreak. Given the continued threat of bioterrorism, the Centre for Disease Control Surveillance System in the United States must also be notified of any cases of botulism.
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PMID:Descending polyneuropathy in an intravenous drug user. 1617 64

Osteoradionecrosis is a process of dysvascular bone necrosis and fibrous replacement following exposure to high doses of radiation. The poorly vascularized necrotic tissue may cause pain and/or instability, and it cannot resist infection well, which may result in secondary osteomyelitis. When these processes affect the cervical spine, the resulting instability and neurological deficits can be devastating, and immediate reestablishment of spinal stability is paramount. Reconstruction of the cervical spine can be particularly challenging in this subgroup of patients in whom the spine is poorly vascularized after radical surgery, high-dose irradiation, and infection. The authors report three cases of cervical spine osteoradionecrosis following radiotherapy for primary head and neck malignancies. Two patients suffered secondary osteomyelitis, severe spinal deformity, and spinal cord compression. These patients underwent surgery in which a vascularized fibular graft and instrumentation were used to reconstruct the cervical spine; subsequently hyperbaric oxygen (HBO) therapy was instituted. Fusion occurred, spinal stability was restored, and neurological dysfunction resolved at the 2- and 4-year follow-up examinations, respectively. The third patient experienced pain and dysphagia but did not have osteomyelitis, spinal instability, or neurological deficits. He underwent HBO therapy alone, with improved symptoms and imaging findings. Hyperbaric oxygen is an essential part of treatment for osteoradionecrosis and may be sufficient by itself for uncomplicated cases, but surgery is required for patients with spinal instability, spinal cord compression, and/or infection. A vascularized fibular bone graft is a very helpful adjunct in these patients because it adds little morbidity and may increase the rate of spinal fusion.
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PMID:Osteoradionecrosis of the cervical spine resulting from radiotherapy for primary head and neck malignancies: operative and nonoperative management. Case report. 1637 Mar 6


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