Hepatitis B surface antigen in urine of renal transplant recipients.
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Biomedical subjects
Publications and source records attributed to R S Shapiro.
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Tracheomalacia occasionally occurs after repair of esophageal atresia and distal tracheoesophageal fistula. It is usually mild, and tracheostomy is rarely necessary. In severe cases of tracheomalacia after repair of esophageal atresia and distal tracheoesophageal fistula, the tracheomalacia may extend below the tip of a regular plastic tracheostomy tube. A patient with just such a problem is presented. A custom-made long plastic tracheostomy tube was designed. A method of gluing the upper portion of a standard plastic tracheostomy tube to an uncuffed thin-walled polyvinyl chloride endotracheal tube was discovered and the technique is described in detail. The literature is reviewed concerning custom-made long plastic tracheostomy tubes and tracheomalacia in relation to esophageal atresia and distal tracheoesophageal fistula.
At the Cleft Palate Unit of The Montreal Children's Hospital a number of patients are seen each year who developed velopharyngeal insufficiency after adenoidectomy. Each time one of these patients is seen, the question is asked: "What would have happened to the speech if adenoidectomy had not been done, and the adenoid had been allowed to undergo the normal process of atrophy at puberty?" We had not seen a patient develop velopharyngeal insufficiency at puberty without adenoidectomy until recently, when an 11.5-year-old boy presented with a history of hypernasality for 1.5 years. Physical examination revealed the soft palate to be slightly short but with good mobility. The voice was mildly hypernasal but there was no nasal escape. Radiologic investigation revealed a very deep nasopharynx with virtual absence of adenoid tissue and a slight shortness of the palate. The child had been on speech therapy and the recommendation was that the therapy be stopped. When the child was seen 2.5 months after cessation of speech therapy, the hypernasality had cleared completely. Speech Pathologists at other Cleft Palate Units were contacted and no similar cases were known. The literature also did not reveal any similar cases of velopharyngeal insufficiency developing after puberty in the absence of adenoidectomy and without a cleft palate.
Three patients developed massive proteinuria, hypoalbuminemia, and edema after transplantation. These findings occurred in the immediate post-transplant period in two patients, and renal failure developed. The third patient developed proteinuria 4 months post-transplantation. There was complete remission of proteinuria in two patients and recovery of renal function in one. Renal histology was similar in all. Light microscopy demonstrated that the glomeruli contained a mild increase in mesangial matrix, but were otherwise normal. No significant interstitial cellular infiltration or fibrosis was present. Immunofluorescence microscopy revealed no staining for immunoglobulins or complement. Electron microscopy demonstrated fusion of foot processes, but the glomerular basement membrane was normal. No electron-dense deposits were found. The usual causes of post-transplant nephrotic syndrome were ruled out. We believe these three patients represent a unique disorder of the transplanted kidney.
Platelet aggregation studies were performed on 10 pediatric patients with acute lymphoblastic leukemia (ALL) receiving induction therapy with vincristine, prednisone, and L-asparaginase. An isolated abnormality in platelet aggregation in response to collagen was found in all patients during the course of therapy. Platelet aggregation in response to collagen normalized following the discontinuation of L-asparaginase, while patients were still on vincristine and prednisone. In contrast to the abnormal collagen response, platelet aggregation induced by epinephrine, arachidonic acid, adenosine diphosphate (ADP), and thrombin were normal both during and following therapy. In the one patient with a normal platelet count before therapy, aggregation induced by all agents was normal. This selective abnormality in collagen aggregation therefore appears to result from therapy, with the use of L-asparaginase in particular being implicated.
A stress test has been designed which shows a consistent abnormality in platelets from carriers of the Wiskott-Aldrich-syndrome (W.A.S.) gene. 2-deoxy-D-glucose (D.D.G.), an inhibitor of glycolysis, completely inhibited second-wave adrenaline (epinephrine)-induced aggregation of platelets from 10 W.A.S. carriers, whereas it had no effect on the response of control platelets. Antimycin A (Ant A), an inhibitor of oxidative phosphorylation, had no effect on adrenaline-induced platelet aggregation of either carriers or controls. Incubation of control platelets with a combination of Ant A and D.D.G. inhibited aggregation in a way comparable to the effect of D.D.G. alone on carrier cells. Thus, W.A.S. carriers have a defect in platelet metabolism similar to that produced in normal platelets with Ant A. The D.D.G. stress test is a simple reproducible assay for detection of W.A.S. carriers.
The role of renal biopsy in 46 patients with end stage renal failure was assessed. Renal tissue was obtained by open renal biopsy in 24 patients, by needle biopsy in 5, and at bilateral nephrectomy in 17. In 4 patients the renal biopsy specimens showed advanced changes that could only be diagnosed as "end stage kidney". In 42 patients (91 per cent), a precise renal diagnosis was made. In 20 patients (43 per cent) the prebiopsy clinical impression differed from the histologic diagnosis. In the 29 patients undergoing renal biopsy, minor postoperative morbidity occurred in 5. We conclude that a renal biopsy in patients with end stage renal failure will result in a change in clinical diagnosis in a significant number of patients and can be performed with low morbidity.
Platelet function was evaluated in eight patients with chronic granulocytic leukaemia (CGL), seven Ph1 positive and one Ph1 negative. Seven of the eight patients' platelets had an absence of the second wave of adrenaline induced aggregation on at least one occasion, while five had impaired collagen aggregation. The platelets of all seven patients with abnormal responses to adrenaline, aggregated with arachidonic acid, thus ruling out a cyclo-oxygenase deficiency. A marked decrease in the ADP, serotonin, and dense body content of platelets was found in all five patients evaluated. Mixtures of CGL patient platelets with platelets from normal donors who had ingested aspirin gave a normal biphasic response to adrenaline. Normal release of the storage pool contents from aspirin treated platelets was shown by stirring a mixture of CGL platelets and 14C-serotonin labelled aspirin treated platelets with adrenaline. The CGL platelets alone or in the mixture produced malondialdehyde in response to adrenaline. These experimental results suggest that CGL platelets have a storage pool deficiency but can synthesize prostaglandins and thromboxanes in response to arachidonic acid and adrenaline.
Two cases of complete subglottic stenosis, with absence of any subglottic airway, are presented. Both patients were treated via an anterior tracheo-crico-thyrotomy with excision of scar tissue. In both patients the newly created lumen was stented with Silastic covered with a buccal graft. One of these patients also had a hyoid bone autograft inserted between the cut edges of the cricoid ring. Both patients were successfully decannulated. The literature is reviewed in order to trace the history of the development of the procedures used.
Seventeen cases of intranasal epithelial tumors are reviewed. Thirteen were epithelial papillomas and four were squamous cell carcinomas associated with epithelial papillomas. In two cases followed over a 10 year period, transformation from an initial papilloma into a carcinoma was demonstrated. The histopathologic caracteristics of these lesions are described including the presence of prominent palisading and intraepithelial nesting in some tumors. The literature was reviewed for reports of carcinoma arising in previously benign epithelial papillomas. We suggest that squamous cell carcinoma may occasionally arise from a previously benign epithelial papilloma.
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A case of massive proteinuria following an A-match living donor transplantation is described. All attempts to define the cause of the proteinuria failed. The proteinuria resolved while the patient was on triple-drug therapy. Presently, the patient remains well with minimal proteinuria and excellent renal function.
Rhabdomyoma is a benign tumor of striated muscle origin that is very rare in young children. Review of the world literature revealed 20 cases of extracardiac adult-type rhabdomyoma in the head and neck area. All reported cases have been encapsulated and easily excised. A 22 1/2-month-old girl with a parapharyngeal rhabdomyoma is unique in that her tumor had atypical histology and lacked a capsule; it could not be "shelled-out".
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