An unusual presentation of a tracheo-oesophageal fistula.
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Biomedical subjects
Publications and source records attributed to J Bruce.
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This report describes a 1-day-old term boy with sigmoid volvulus secondary to Hirschsprung's disease. The presentation and treatment are outlined.
We compared quantitative urinary cystine values in stone-forming family members and unaffected relatives based on an index stone-forming patient. A family pedigree of 39 members was identified. Quantitative urinalyses and serum biochemical profiles were collected with the subjects on similar diets over 72 hours. Detailed medical histories were obtained. Three family members are stone-forming cystinuric patients with a mean urinary cystine excretion of 496 mg/g of creatinine per day. Six persons without a history of stones (mean age 50) had a mean urinary cystine excretion of 364 mg/g of creatinine per day. Thirty-four family members had elevated urinary cystine concentrations (mean 151 mg/g of creatinine per day), and only two family members had normal urinary cystine excretion. Fifteen subjects with elevated urinary cystine values (> 150 mg/g of creatinine per day) had no history of urinary stones despite a mean age of 44 years. Urinary electrolytes and volumes were similar in the stone-forming and non-stone-forming groups. Thirty-nine per cent of the persons from this family pedigree had elevated quantitative urinary cystine concentrations and no history of urinary stones despite a mean age of 44 years. Acalculous cystinuria is an elevated urinary cystine concentration without evidence of urinary stone disease. Despite abnormal urinary cystine excretion, acalculous cystinuria does not necessarily culminate in clinical urinary stone disease.
Like many organizations, Continental Rehabilitation Hospital of San Diego designs and communicates templates and targets for desired changes in behaviors and tracks results. It recognizes and celebrates successes and struggles with its failures. A recent case example demonstrates the importance of generating immediate feedback and consequences as a strategy for increasing effectiveness in changing behavioral patterns.
The role of cellular and humoral immunity coeliac disease was investigated by the measurement of serum levels of interleukin-10 (IL-10), interferon-gamma (IFN-gamma) and soluble CD23 (sCD23). Coeliac disease was diagnosed by duodenal biopsy and response to a gluten-free diet (GFD). The results were compared with age and sex-matched patients with non-specific upper gastrointestinal symptoms and normal duodenal histology. While the levels of serum IL-10 were significantly elevated (P < 0.01) in patients with coeliac disease taken as a whole, the levels of serum IFN-gamma were normal and sCD23 significantly decreased (P < 0.002). The median serum sCD23 was significantly lower in the coeliac disease patients not on a GFD compared with those asymptomatic on a GFD (P < 0.03) and the control group (P < 0.0004). The coeliac disease patients on a GFD also had significantly lower serum sCD23 and higher IL-10 compared with the control group (P < 0.01 and P < 0.015). There was no significant difference in the serum IL-10 between the coeliac disease patients on a GFD and those not on a GFD and between the latter and the control group. The low levels of serum sCD23 in coeliac disease suggest diminished humoral immunity and, conversely, exaggerated cellular immunity. The aetiology of the raised levels of IL-10 in coeliac disease is unclear and similar to that observed in patients with inflammatory bowel disease. However, this may represent a regulatory response to the elevated levels of proinflammatory cytokines described in coeliac disease. A combination of diminished sCD23 and raised IL-10 is clearly unusual as both are associated with Th2-type functions. The possible causes of this finding are discussed.
Management of unilateral multicystic dysplastic kidneys (MCDK) presents physicians and surgeons with a significant dilemma. Recent studies have indicated that the incidence of short term complications of MCDK is low and many authors have recommended conservative non-operative treatment. Surgery has been proposed by some because of the potential complications of hypertension, infection, and malignant change. Three children with hypertension secondary to MCDK seen at this institution in the past four years, one of whom had been discharged from follow up as a result of 'disappearance' of the cystic kidney on ultrasound examination, are reported. We believe that the risks of hypertension secondary to MCDK have been understated, and that based on the conclusions of these studies, many children may be receiving suboptimal follow up. We currently favour elective nephrectomy as the treatment of choice for this lesion.
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PURPOSE: We developed criteria for nonoperative treatment of penetrating renal injuries and predicting which patients will benefit from immediate surgery. MATERIALS AND METHODS: We reviewed the records of 120 patients with grades 2 to 4 renal lacerations from gunshot and stab wounds, of whom 41 were treated nonoperatively (group 1) and 79 underwent immediate renal exploration (group 2). RESULTS: Group 1 had significantly lower incidences of gunshot wounds, shock, associated injuries, need for transfusion and high grade renal injuries. In patients with grade 2 lacerations no complications resulted from nonoperative treatment. However, 23.5% of patients in group 1 with grades 3 and 4 injuries had delayed renal bleeding versus none in group 2. CONCLUSIONS: Nonoperative treatment of penetrating renal lacerations is appropriate in hemodynamically stable patients without associated injuries. Grade 2 injuries can be treated nonoperatively but grades 3 and 4 injuries are associated with a significant risk of delayed bleeding if treated expectantly. Exploration should be considered if laparotomy is indicated for other injuries.
This report describes three children, age range 7 weeks to 5 months, who presented with obstructive jaundice secondary to gallstones. Previous Escherichia coli septicaemia and frusemide therapy were predisposing risk factors in two of the patients. All three were successfully treated with cholecystectomy and exploration of the common bile duct.
A 7-week-old boy born at 32 weeks' gestation developed massive upper gastrointestinal bleeding from a duodenal ulcer. The base of the ulcer had eroded into the hepatic artery. Haemorrhage was only controlled following operation.
OBJECTIVE: To determine the effectiveness of a breastfeeding support service attached to a general practice. DESIGN: A lactation consultant (LC) was employed in a general practice in Happy Valley. Adelaide over 12 months to provide an appropriate intervention program of education and support for mothers experiencing problems with breastfeeding. The mothers were asked to evaluate the service via a postal questionnaire. The baseline rates of women in this practice who were solely breastfeeding were determined by a retrospective questionnaire sent to all mothers of the practice whose children were 18 months to 2 1/2 years of age (168 mothers formed this baseline group). There were 119 mothers in the intervention group. RESULTS: There was a high, breastfeeding initiation rate for both baseline (94.6%) and intervention (93.4%) groups. There were significantly higher breastfeeding rates in the intervention group at 24 and 26 weeks (63.3% vs 51.2% at 24 weeks [p = 0.015] and 64.7% vs 50.6% at 26 weeks [p = 0.018]). While there was no significant difference in the total number of breastfeeding problems encountered by either group significantly more mothers from the baseline group suffered from engorgement and/or too much milk. Evaluation of the service indicated a high degree of satisfaction. Over 94% of the mothers found the service friendly, supportive, and useful. CONCLUSIONS: This service provides an effective method for the support and protection of breastfeeding.
INTRODUCTION AND OBJECTIVES: The pathological findings in surgical material from children with refractory epilepsy has not offered yet a clear understanding of its role in this condition. The objective of this paper is to report our findings to further expand our knowledge about refractory epilepsy in children. MATERIAL AND METHODS: Results of microscopic examination of the surgical specimen obtained from 80 children, ages 12 or younger, who had surgery for intractable epilepsy at Miami Children's Hospital between 1990 and 1996 were reviewed. RESULTS: Examination was normal only in one. The rest revealed ectopic neurons (1), dysplastic cells with ectopic neurons (2), dyslamination with large neurons (7), dyslamination with ectopic neurons (18), dyslamination with dysplastic cells (10), pachygyria (2), encephalomalacia (9), gliosis with ectopic neurons (10), gliosis without ectopic neurons (3), developmental ectodermal tumor (6), ganglioglioma (2), tumors (3), and Rasmussen encephalitis (4). Lesions were located to the temporal lobe in 34 children. CONCLUSIONS. Extratemporal lesions are more frequent than temporal one, including hyppocampal sclerosis. Ectopic neurons, the most frequent pathological findings, rather than a cause of seizure may be a marker other highly epileptogenic cortical malformations.
We compared the serum levels of several cytokines with established tumour markers in 24 patients with non-small cell lung cancer (NSCLC) and 31 patients with benign lung disease (BLD). Cytokine levels were measured using in-house double determinant sandwich ELISAs and tumour markers by a variety of established techniques. There was no correlation between serum cytokines and expression of cytokeratins 18 and 19, MUCI and carcinoembryonic antigen. While no significant difference was observed in any of the cytokines between patients with NSCLC and BLD, patients with metastatic tumour had a significantly higher level of serum tumour necrosis factor alpha and interleukin 10 than those with localised disease (P < 0.015 and P < 0.05 respectively). The serum levels of granulocyte macrophage colony stimulating factor and interferon gamma were no different between these groups. These results suggest immunological effects of NSCLC which tends towards impaired cell mediated immunity in patients with metastatic disease.
OBJECTIVE: To assess the efficacy of the Whitaker diathermy hook in the treatment of posterior urethral valves (PUV). PATIENTS AND METHODS: Seventeen patients with PUV underwent primary treatment under general anaesthesia using a diathermy hook. The results were assessed by the clinical course of the patient, serial measurements of serum creatinine level and repeat cystography 6 weeks and 6 months after treatment. RESULTS: There were no complications in 12 patients and they required no further treatment of the valves. Three patients required a repeat procedure which resolved the urethral obstruction. Two patients continued to show incomplete disruption of the valves and required endoscopic treatment. Two patients developed ureteric obstruction after disruption of the valves, which was treated by supravesical diversion and later reversed with no further treatment of the valves. CONCLUSIONS: The Whitaker diathermy hook is a safe and effective instrument which has not gained its rightful place in the treatment of patients with PUV.
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To determine if resistance/tolerance to the antischistosomal drug praziquantel (PZQ) is appearing in Egyptian villages within the Nile delta region, where it has been used extensively, we treated 1,607 infected villagers and observed that 321 required one additional treatment while 89 villagers required two additional treatments; 24 of the 89 were still not cured after a third dose of this drug. Eggs were isolated from fecal samples and serum was isolated from blood taken from seven villagers successfully treated after a single dose and from 14 villagers not successfully treated after two or three doses of PZQ. The eggs were used to establish infections in mice (isolates), which were then treated six weeks after infection with three different doses of PZQ. Serum was used to determine the concentration of PZQ in the infected humans. Three of the egg isolates from the 14 villagers that could not be treated with three doses of PZQ produced infections in mice that were statistically less responsive to PZQ when compared with isolates obtained from patients that were cured after a single dose of this drug. Pharmacokinetic parameters were the same in patients treated successfully after a single dose versus those not treated successfully following two or three doses, thus eliminating the possibility that poor cure rates among infected villagers was due to a decrease in PZQ bioavailability. From our data, approximately 1-2.4% of the villagers treated with PZQ could not be completely cured of their infection and three of every 1,000 treated villagers may harbor parasites that can tolerate high doses of PZQ. These results indicate that the extensive use of PZQ in the Nile delta region of Egypt has not resulted in a dramatic change in the efficacy of this drug. The isolation of schistosomes that are less susceptible to PZQ may be a warning signal that will require establishment of a monitoring system, similar to the one we have developed, to determine if the percentage of patients that cannot be cured by PZQ is increasing. Furthermore, if that percentage begins to increase over time, it will be critical to determine, by pharmacologic methods reported in this study, whether isolates obtained from uncured patients are becoming increasingly resistant to PZQ.
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