Tests for pernicious anemia: serum intrinsic factor blocking antibody.
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Biomedical subjects
Publications and source records attributed to F M Howard.
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We evaluated a 2-year experience involving 22 patients who required prolonged mechanical ventilation for respiratory failure associated with myasthenia gravis. The most frequent cause of respiratory failure was operation, and the most common type of procedure was thymectomy. Next in frequency as exacerbating factors were myasthenic crisis and cholinergic crisis. Of the 22 patients, 21 survived and were weaned from the ventilator after 1 to 32 days of respiratory support.
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Seventy-nine patients with acute Guillain-Barré syndrome were seen during a 6-year period. Twenty-one were admitted to a respiratory intensive care unit, where they remained for 58 +/- 26 days (range 14 to 105 days). Thirteen patients required nasotracheal intubation followed by tracheostomy and mechanical ventilation. The tracheostomy tube was in place for an average of 50 +/- 27 days (range 10 to 104 days). Four patients had complications of tracheostomy; two of these were significant, and one of them led directly to the patient's death. There were no complications due to mechanical ventilation, from which 11 patients were successfully weaned after a mean period of 37 +/- 29 days (range 7 to 93 days). Three of the 79 patients (3.8%) died of complications of their disease or its treatment. Respiratory failure in this condition is protracted and its complications are mainly those of prolonged endotracheal intubation with a tracheostomy tube.
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A family study of undiagnosed protracted diarrhoea in infancy was undertaken, based on 67 such patients, seen at The Hospital for Sick Children, London, over a 6-year period. All were fully investigated with the exclusion of those with known cause, such as coeliac disease, cow's milk intolerance, or enteric infections. The families were traced and visited. The material is certainly heterogeneous. In the case of five patients the condition was associated with a syndrome. The remaining 62 index patients could be divided into a group of 15 with severe illness and 47 with a milder illness. Six had onset before one month of age, four died, and in five the diarrhoea lasted more than 12 months and the children persistently failed to thrive. These 15 severe cases had six affected sibs out of 22 (Weinberg proband method) and in all but one of these affected sibs the condition was also severe. Two further sibs had had protracted diarrhoea, but this had not been fully investigated. The 47 index patients with milder disease had 68 sibs of whom only one was affected (this boy was one of the severely affected index patients), and one other sib had protracted diarrhoea which was not fully investigated. It is proposed that the severe group includes one or more autosomal recessive entities, in which the basic defects are not yet known, but are likely to be inborn metabolic errors.
Stenosis of the aqueduct of Sylvius accounts for about one third of cases of congenital hydrocephalus. At least 32 families have been reported in which the aqueduct stenosis is inherited in an X linked fashion. In half of these families, flexed adducted thumbs were noted in some affected family members. Occasionally other male members were mentally retarded, suggesting limited expression of the gene. The problem of giving genetic advice to an isolated, clinically unremarkable, case of aqueduct stenosis remains, so a family study was undertaken based on 24 such cases seen at The Hospital for Sick Children over a 19-year period. There were 15 male and nine female index patients. The diagnosis was confirmed in all cases by air encephalogram. One boy had a radial club hand and another developed clasped thumbs secondary to spasticity. No cases had hyaloidoretinal dysplasia. The 15 boys had 18 brothers and 19 sisters, of whom one sister was similarly affected. The nine girls had 12 sibs, none of whom was affected. This study, combined with a similar study in the USA, suggests that the empirical risk of recurrence of a sporadic case of aqueduct stenosis is about 4.5%.
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The terminal and lytic complement component (C9) was localized at the motor end-plate in acquired autoimmune myasthenia gravis (MG) by the immunoperoxidase method, with adequate preservation of fine structure and negligible background staining. C9 was localized on short segments of the postsynaptic membrane on degenerated fragments of the junctional folds shed into the synaptic space, and on disintegrating junctional folds. An inverse relationship was noted between the structural integrity of the junctional folds and the abundance of C9 at a given end-plate region. Destruction of junctional folds by complement may induce relocation of the nerve terminal and increased spatial separation of end-plate regions on the muscle fiber. Destruction of junctional folds by the complement membrane attack complex is a cause of the acetylcholine receptor deficiency at the MG end-plate, but antibody-dependent modulation of the receptor may also contribute to deficiency of the receptor. In certain disorders other than autoimmune MG, pathological mechanisms other than complement-mediated lysis may affect the structural integrity of the postsynaptic region.
Of the babies admitted to the Special Care Baby Unit of the Royal Free Hospital over 20 months, 10.2% were infected or colonised by klebsiella. The fluorescent antibody technique was used to identify epidemics caused by three strains: capsular type 8 K. aerogenes, type 68 K. oxytoca, or type 13 K. aerogenes, each of which was predominant at a different time, exhibited a difference in virulence, and showed a predilection for different sites of infection. Intestinal colonisation was frequently followed by the presence of sepsis in other sites by the same capsular type. Antibiotic administration led to a higher incidence of klebsiella infection, while the widespread use of compounds containing hexachlorophane could have contributed to skin colonisation and infection by klebsiella. An environmental survey indicated that 1% Hycolin failed to disinfect the incubators, that the babies were the reservoirs of the organisms, and that transmission was due to inadequate hand-washing of nurses and mothers. The mothers were found to have been uninformed of hygienic techniques. They were observed in various practices which could have contributed to the spread of the organism, including contaminating communal areas and handling babies other than their own. It has been recommended that the mothers of premature infants be instructed in the hygienic measures required in dealing with this susceptible population and that the nursing and medical staff be more strict in their own observance of these procedures.
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Four babies with complete heart block associated with maternal systemic lupus erythematosus (SLE) are described, together with a 5th baby whose mother had serological abnormalities only. One baby had a rapidly fatal outcome, one has required digoxin for heart failure, and the remaining 3 are asymptomatic but remain in complete heart block. Additional manifestations were present in 2 of them. The spectrum of neonatal abnormalities that may occur in association with maternal SLE and related connective tissue disorders is discussed, together with the possible causes and the prognosis. We conclude that congenital heart block is more common than had previously been appreciated.
Myasthenia gravis is a neuromuscular disease of insidious onset, characterized by weakness and fatigability of voluntary muscles. Most patients present with symptoms relating to the head and neck and thus may be seen first by the otolaryngologist. Predominant symptoms may be ocular (ptosis or diplopia) or related to fatigue of the oropharyngeal or laryngeal musculature (dysarthria, dysphonia, or dysphagia). Alleviation of muscular weakness and fatigability after administration of anticholinesterase drugs is pathognomonic of myasthenia gravis.
Compression osteotomy of the tibia is to allow knee motion postoperatively without case immobilization. Rigid internal stabilization by axial compression and tension band wiring enhances union. The rigid internal stabilization prevents loss of position of the osteotomy, often seen in other techniques. The benchmark for this osteotomy operation is no fractures of the osteotomy fragments, no position loss, no thromboemboli, and no deep wound infections.
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