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Biomedical subjects

J J Murphy

Publications and source records attributed to J J Murphy.

At least 19 recordsLinked to original sources

Eosinophilia-myalgia syndrome in L-tryptophan-exposed patients.

OBJECTIVES: To study the incidence of eosinophilia-myalgia syndrome, the risk factors associated with the syndrome, and the clinical spectrum of illness associated with L-tryptophan use in an exposed population. DESIGN: Retrospective cohort and nested case-control studies of risk factors for eosinophilia-myalgia syndrome using inpatient and outpatient chart reviews, telephone interviews, and in-person patient interviews. Descriptive study of clinical course of L-tryptophan users. SETTING: Office practice of one psychiatrist based in a small city (population 43,467) in South Carolina. PATIENTS: Eligible subjects were all patients from the practice who used L-tryptophan during the 1989 study interval. Of these, 418 (87%) were interviewed. MAIN OUTCOME MEASURES: Clinical spectrum of illness associated with L-tryptophan use, including definite and possible cases of eosinophilia-myalgia syndrome. RESULTS: Among the 418 interviewed L-tryptophan users, we identified 47 definite cases (11%) and 68 possible cases (16%) of eosinophilia-myalgia syndrome, most of which involved patients who were using one retail brand of L-tryptophan (brand A). Among the 157 brand A users, we identified 45 definite cases (29%) and 36 possible cases (23%) of eosinophilia-myalgia syndrome, and the risk for the syndrome increased as the brand A dose increased. Fifty percent (19 of 38) of those using more than 4000 mg/day developed definite eosinophilia-myalgia syndrome, and 84% (32 of 38) developed either definite or possible eosinophilia-myalgia syndrome. On multivariate analysis, risk for definite eosinophilia-myalgia syndrome was associated with brand A dose and age of the patient; however, gender, race, and use of other medications were not associated with the syndrome. CONCLUSIONS: These results suggest that many people exposed to the agent causing eosinophilia-myalgia syndrome may develop illness, and dose of presumably contaminated L-tryptophan is the single most important predictor of eosinophilia-myalgia syndrome. The broad range of signs and symptoms reported by patients using L-tryptophan illustrates that a strict case definition may identify only about half of those affected.

Adult

Sirenomelia: case of the surviving mermaid.

This report describes a neonate with sirenomelia who was recently treated at British Columbia Children's Hospital. Prenatal diagnosis was made at 29 weeks' gestation. Survival was not anticipated. Cesarean-section at term showed a live 2,375-g infant with excellent Apgar scores. Anomalies noted were fused lower extremities, imperforate anus, colon atresia, bilateral fused pelvic kidneys with renal dysplasia, pelvic and sacral dysplasia, and genital abnormalities. Laparotomy and colostomy were performed. All other anomalies are compatible with life and she is neurologically normal. Eventual separation of the lower extremities is planned. This is the second reported case of survival in a patient born with sirenomelia.

Abnormalities, Multiple

The bowel management tube: an effective means for controlling fecal incontinence.

Regular bowel washout enemas have been used as a method of management of fecal incontinence. The effective administration of a washout enema to a child with weak anal sphincters is often a problem. Using a new silastic balloon-tipped enema catheter (bowel management tube [BMT]) of our design, we prospectively studied its effectiveness in a group of children who suffered fecal incontinence. Thirty-one children were studied over a 1-year period. Their diagnoses included meningomyelocele (19), postoperative Hirschsprung's disease or imperforate anus (10), and other (2). Before and after starting the BMT enema system, clinical assessment and a diary, which graded the degree of fecal incontinence and satisfaction with the system, were completed. Five patients failed to benefit because of noncompliance (3) or balloon extrusion (2). Three more patients discontinued the use of the tube system. Twenty-three patients achieved successful results with this system as evidenced by a significant amelioration in their fecal incontinence and their unwillingness to give up the use of the BMT. We conclude that the use of a regular washout enemas with BMT can be an effective method for control of fecal incontinence in children.

Adolescent

Coagulopathy associated with large sacrococcygeal teratomas.

Twelve neonates with sacrococcygeal teratoma (SCT) have been treated at British Columbia Children's Hospital over the past 5 years. Clinically significant coagulopathy developed in four of these neonates and two died, one before surgical intervention could be undertaken. Disseminated intravascular coagulation (DIC) was found in one patient and thrombocytopenia in another on preoperative laboratory studies. Etiology of the coagulopathy is unclear, but appears to be multifactorial. Although several clinical reviews have noted mortalities due to exsanguinating hemorrhage, no study has focused solely on this issue. The diagnosis of SCT in the neonate at high risk for development of coagulopathy is usually made prenatally. Premature labor is often precipitated by associated polyhydramnios and large tumor size. Fetal distress, prematurity, and low birth weight are common. Presence of placentamegaly, hydrops fetalis, and congestive heart failure are ominous prognostic signs. Early identification of patients at increased risk for development of hemorrhagic complications may allow optimization of their management. Cesarean section should minimize trauma to the SCT during delivery. Expeditious resection of the lesion may improve survival.

Blood Coagulation Tests

The treatment of simultaneous choriocarcinoma in mother and baby.

Simultaneous occurrence of choriocarcinoma in mother and child is rare. Such a case is described in which a massive intrahepatic tumor in the infant led to its treatment and that of the mother who showed evidence of the same tumor. Both are alive and well 1 year after treatment, the longest recorded infant survival.

Angiography

Rhabdomyosarcoma arising within congenital pulmonary cysts: report of three cases.

Over the past 9 months, three cases of primary pulmonary rhabdomyosarcoma have been treated at British Columbia Children's Hospital. Two patients (aged 24 and 37 months) presented with spontaneous pneumothoraces and had cystic changes in the affected lung on chest radiograph. The third patient (aged 42 months) was evaluated for chronic cough, fever, and failure to thrive. Chest x-ray showed a large mass in the left lower lobe as well as mediastinal adenopathy. All three of these lesions originated within congenital lung cysts, one a peripheral bronchogenic cyst and the others cystic adenomatoid malformations. This report suggests that there is a significant risk for the development of rhabdomyosarcoma within malformed pulmonary tissue.

Cell Transformation, Neoplastic

Prodromal chest pains: clues to the pathogenesis of non-Q wave acute myocardial infarction?

Prodromal chest pains were recorded from consecutive admissions to a single coronary care unit and the symptoms of those with Q wave and non-Q wave acute myocardial infarction were compared. Of 809 admissions there were 201 with Q wave ad 88 with non-Q wave infarction. The non-Q wave infarction group was older, included more women and a greater proportion had suffered a previous myocardial infarct. Effort angina was equally common in both groups, but in the Q wave infarction group, angina was more often of recent onset, within the previous 4 weeks. Symptoms to indicate worsening angina, more prolonged, intense or frequent pain, were equally common in both groups. These findings suggest that although the extent of coronary artery disease may differ, Q wave and non-Q wave myocardial infarction share a common pathogenic mechanism.

Adult

Predictors of risk in patients with unstable angina admitted to a district general hospital.

OBJECTIVE: To observe the long-term prognosis of patients with unstable angina and select simple criteria to identify high and low risk subgroups. DESIGN: A six month prospective survey with three year follow up. SETTING: One eleven bed coronary care unit. PATIENTS: All patients admitted with chest pain in whom no infarct was confirmed by subsequent electrocardiographic or enzyme changes and for whom no alternative cause of chest pain was found were studied. Unstable angina was also diagnosed if there was evidence of myocardial ischaemia in the form of previous effort angina, previous myocardial infarction, or if transient electrocardiographic changes accompanied the pain. When none of the above were present, chest pain without a known cause, was diagnosed. INTERVENTIONS: No routine intervention. Angiography and revascularisation for persistent symptoms despite medical treatment. OUTCOME MEASURES: Death or non-fatal infarction. RESULTS: In the 141 patients with unstable angina there were eight deaths and five non-fatal infarctions during the first eight weeks. Symptoms of increasing angina before admission were similar in all three groups and did not help predict early complications. Recurrence of pain in hospital, a rise in cardiac enzymes to less than twice the upper limit of normal, and transient electrocardiographic changes were all associated with an increased risk of early events. The presence of either abnormal enzyme activity or more than five episodes of pain in hospital identified a group of 49 in whom 11 of the 13 early events occurred. After three years, 29 of the 141 patients had died and eight had had infarctions (overall event rate 26%). Seventeen had undergone revascularisation (12%) and 51 (36%) were on antianginal treatment. Thirty six (26%) were still alive, without new myocardial infarction, and were free of angina. In the 29 patients with chest pain without a known cause there were no early events and only one non-fatal infarction during the three year follow up. CONCLUSION: When patients are admitted to the coronary care unit with chest pain not due to myocardial infarction, the history, electrocardiography and measurement of cardiac enzymes are sufficient to identify high and low risk subgroups.

Adult

Patient recovery following cholecystectomy through a 6 cm or 15 cm transverse subcostal incision: a prospective randomized clinical trial.

The effect of incision length on patient recovery following cholecystectomy has not been investigated previously. In this study, 30 patients with symptomatic gallstones were randomized to cholecystectomy through a 6 cm or 15 cm transverse subcostal incision. Postoperative hospital stay was significantly shorter in the 6 cm incision group (median 3 days vs 5 days; P = 0.0069 Mann-Whitney U-test). In the 6 cm group analgesic requirements were reduced (median 2.5 vs 4.5 intramuscular opiate injections per patient) and recovery of depressed postoperative pulmonary function (FVC and FEV1) was faster (3% difference between groups on day 1 and 7% on day 3), although these differences did not achieve statistical significance. These results suggest that the length of incision may influence patient recovery following elective cholecystectomy. This has important implications as surgery carried out through shorter and less traumatic incisions may offer a cost-effective alternative to laparoscopic cholecystectomy. Moreover, some surgeons may find mini-laparotomy cholecystectomy easier to adopt than laparoscopic techniques.

Adult

Eosinophilic gastroenteritis presenting as ascites. Case report.

A 46 year old man presented with a four week history of ascites and loss of weight. The diagnosis of eosinophilic gastroenteritis was made only after histological examination of a biopsy specimen taken at laparotomy. All other investigations had been unhelpful. He made a good recovery and was given no treatment.

Ascites

Familial juvenile polyposis coli and colorectal cancer.

A 40-year-old mother and her 7-year-old son underwent colectomy for polyposis coli. Both colectomy specimens contained predominantly juvenile polyps. The mother's specimen also had numerous tubular adenomas and one 5-cm severely dysplastic villous adenoma without invasive carcinoma. Foci of adenomatous change in juvenile polyps were present in the son's specimen. The histologic features of both cases suggest a sequence of change from juvenile polyps to adenomatous polyps to cancer.

Adenomatous Polyposis Coli

Evidence for protein kinase C--independent pathways mediating phorbol ester induced plasmacytoid differentiation of B chronic lymphocytic leukemia cells.

The effects of phorbol esters on many cell types are known to be mediated through activation of the protein kinase C (PKC) signal transduction pathway. By using the specific inhibitor of this enzyme 1-(5-isoquinolinylsulfonyl)-2-methyl-piperazine dihydrochloride (H7) we have assessed the role of PKC activation in phorbol ester (phorbol 12-myristate 13-acetate, PMA)-induced plasmacytoid differentiation of B chronic lymphocytic leukemia cells (B-CLL) as a model of terminal differentiation of human B lymphocytes. H7 affected a dose-dependent inhibition of PMA-induced thymidine and uridine uptake with ID50 values of 41 microM and 32 microM, respectively. A comparable ID50 value (34 microM) was obtained for H7 inhibition of B-CLL PKC activity in a cell-free system. PMA-induced changes in cell morphology, expression of CD20, CD37 and FMC7 surface antigens together with increased secretion of immunoglobulin were variably abrogated by H7 suggesting that PKC activation is more important in B cell activation/DNA synthesis than in the differentiative response. Consistent with this, expression of a sizable proportion of PMA-inducible genes was not significantly affected by H7. These data are consistent with the existence of a PMA-activated, PKC-independent signal transduction pathway which may be important, though by itself apparently insufficient, for eliciting full terminal differentiation in B lymphocytes.

1-(5-Isoquinolinesulfonyl)-2-Methylpiperazine

The management of some special problems with renal neoplasm.

Simultaneous bilateral renal cell carcinoma has been treated in 5 patients at our hospital during the last 6 years. Improvements in radiologic diagnostic techniques are responsible for the increased detection of these lesions. Results of surgical management are reported. Angiographic embolization of renal cell carcinoma has proved to be a useful method to manage patients with solitary kidneys or those with severe medical problems contraindicating an operation, and our results with this approach are described.

Adenocarcinoma