Parental opinions on treatment decisions for myelomeningocele infants: a descriptive study.
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Biomedical subjects
Publications and source records attributed to R A Jacobs.
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Parents of MMC patients have strong attitudes and beliefs on specific issues. They believed in the need to be involved in decisions regarding their children and felt strongly that their role was central and was important as that of the physician. They believed also in the importance of providing both medical and nutritional care to handicapped infants with a minimum of government intrusion.
A retrospective chart review of 138 subjects with myelomeningocele was performed to determine factors affecting upper extremity function in this population. Upper extremity dysfunction was found in 62 subjects (46.6%). A relationship was found between upper extremity dysfunction and hydrocephalus. Subjects with hydrocephalus had a significantly greater incidence of upper extremity dysfunction than subjects without hydrocephalus. No significant relationship was found between surgically treated versus spontaneously arrested hydrocephalus or number of shunt revisions and upper extremity dysfunction. A relationship was found between level of lesion of the spinal cord and upper extremity dysfunction. High level lesions had a significantly higher incidence of upper extremity dysfunction. Of 62 patients with upper extremity dysfunction only 3 (3.2%) had normal development. This was statistically significant. Little attention has been given to general developmental status in myelomeningocele children with upper extremity dysfunction. This study suggests a strong correlation between these two variables. In conclusion, hydrocephalus, level of spinal cord lesion, and developmental status, appear to be significant factors affecting upper extremity dysfunction in children with myelomeningocele.
Some infants with myelomeningocele, hydrocephalus, and Arnold-Chiari malformation have symptomatic apnea or hypoventilation. The incidence of abnormalities of the ventilatory pattern during sleep in asymptomatic infants with myelomeningocele is not known. Therefore we performed overnight pneumograms (recordings of ventilatory pattern and electrocardiogram) in 18 asymptomatic infants with myelomeningocele and compared them with pneumograms from 64 control infants. Infants with myelomeningocele had longer total sleep time (596 +/- 16 minutes vs 536 +/- 10 minutes, P less than 0.005), longer episodes of longest apnea (12.6 +/- 0.8 seconds vs 8.1 +/- 0.3 seconds, P less than 0.001), greater total duration of apnea greater than or equal to 6 seconds as percent total sleep time (1.02% +/- 0.18% vs 0.23% +/- 0.03%, P less than 0.001), and lower mean heart rates (120 +/- 5 vs 145 +/- 5, P less than 0.001) than did control infants. No abnormal bradycardia was observed in either group. Thirteen (72%) of 18 infants with myelomeningocele had abnormal pneumograms, compared with 4 (6%) of 64 control infants (P less than 0.0005). We conclude that asymptomatic infants with myelomeningocele have a high incidence of ventilatory pattern abnormalities during sleep.
Hypoxic and hypercapneic arousal responses from quiet sleep were tested in seven infants with myelomeningocele and Arnold-Chiari malformation who were symptomatic with apnea and/or hypoventilation. All infants with myelomeningocele required tracheostomy and posterior fossa decompression. Responses were compared with those of nine healthy control infants. To assess hypoxic arousal, inspired PO2 was decreased until the end-tidal (alveolar) PO2 reached 45 mm Hg for a maximum of three minutes. Eleven studies were performed in seven infants with myelomeningocele, and arousal occurred in only two studies (18.2%). Eight of nine control infants aroused to hypoxia (89%). To test hypercapneic arousal, inspired PCO2 was increased until end-tidal PCO2 reached 60 mm Hg for a maximum of three minutes. Eight studies were performed on six infants with myelomeningocele, and arousal occurred in three studies (37.5%). All seven control infants studied aroused to hypercapnea (100%). Three infants with myelomeningocele subsequently died. Infants with myelomeningocele, Arnold-Chiari malformation, and apnea or hypoventilation have arousal deficits to respiratory stimuli.
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Although clinical disease due to enterococcus is common, there has been only limited experience in the treatment of central nervous system infections by this pathogen. In particular, there have been few reports regarding the treatment of such infections in the penicillin-allergic individual. We present two cases of meningitis due to enterococci, including one case with a brain abscess, in patients with strong histories of penicillin sensitivity. We treated these patients with vancomycin hydrochloride and an aminoglycoside. Vancomycin with an aminoglycoside seems to be a reasonable treatment for enterococcal central nervous system infections.
Fifteen cases of postoperative Candida parapsilosis endophthalmitis occurring secondary to a contaminated lot of an irrigating solution were studied. All patients underwent a vitreous tap or diagnostic and therapeutic vitrectomy. Eleven of the 15 specimens were positive for the organism. Fourteen patients were treated with pars plana vitrectomy surgery. All patients were treated with intravitreal amphotericin B and systemic amphotericin B and 5-fluorocytosine. Two clinical recurrences were successfully treated with intravitreal amphotericin B, removal of the pseudophakos, and oral ketoconazole. The intraocular lens was retained in 11 of the 14 pseudophakic patients. Final visual acuities ranged from 20/25 to no light perception with eight of 15 patients having 20/60 or better visual acuities. Measurable levels of intraocular amphotericin B were found after systemic amphotericin B administration. Two patients with totals of 20 and 30 micrograms of intravitreal amphotericin B over 48 and 96 hours, respectively, had near normal ERGs one year later. Posterior capsulotomy and vitrectomy appear to decrease amphotericin B toxicity and allow sequential intraocular injection of this drug within a short time period.
Aortoesophageal fistula is a rare cause of massive gastrointestinal hemorrhage, and may occur as a sequela to prosthetic replacement of the thoracic aorta. Esophageal compression necrosis with leakage of microorganisms into the proximal suture line is probably central to pathogenesis. Like the more common aortoduodenal fistula, diagnosis by traditional radiographic and endoscopic methods is difficult. We report here such a fistula, in which computed tomographic soft tissue abnormalities were characteristic of perigraft abscess; fistulization occurred subsequently. CT holds potential for being a sensitive study to show localized perivascular infection, an important precursor to aortoenteric fistula, and as such should be positive early in the development of a fistula.
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We reviewed the records of 32 patients with acute leukemia and proved invasive fungal infections to determine the clinical and pathologic characteristics of systemic mycosis in patients undergoing intensive induction chemotherapy. The incidence of invasive fungal infections among our patients was at least 27 percent, and Candida and Aspergillus accounted for the majority of these infections. Patients with systemic candidiasis generally had prolonged severe neutropenia, fever refractory to antibiotics, and evidence of mucosal colonization by fungi. At autopsy, Candida was always widely disseminated. Patients with aspergillosis generally had neutropenia, fever, and pulmonary infiltrates at the time of admission to the hospital and, at autopsy, their infections were primarily confined to the lungs. Patients infected with both Candida and Aspergillus had clinical and pathologic findings that were a combination of the features of each type of infection. A diagnosis of invasive fungal infection was established before death in only nine of the patients, all of whom had systemic candidiasis. Four of these patients were successfully treated and survived their hospitalization. The reasons for frequently misdiagnosing and unsuccessfully treating systemic mycosis in patients with acute leukemia are examined, and suggestions are made for improved management of patients at high risk for these infections. These suggestions are based upon recognition of the clinical settings in which fungal infections occur, the aggressive use of invasive diagnostic procedures, and the early empiric use of amphotericin B.
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Short trachea, typically due to reduced tracheal cartilage ring number, produces high tracheal bifurcation which can be demonstrated in radiographs with evaluable air bronchograms. Accidental bronchial intubation with sequelae has been reported in short trachea patients who have tracheal intubation. Short trachea is associated with a number of syndromes, including DiGeorge anomaly and several types of congenital heart disease and skeletal dysplasias. Review of chest radiographs of 87 patients with myelomeningocele revealed that 31 (36%) had short tracheas. Two patients had a total of 3 episodes of bronchial intubation. This degree of association of short trachea with myelomeningocele, the frequency of myelomeningocele, and the number of surgical procedures performed on many such patients suggest that special attention to the short trachea is warranted in myelomeningocele patients because of the risk of accidental bronchial intubation and subsequent sequelae. Twelve (14%) of the myelomeningocele patients lacked radiographic evidence of the presence of twelfth ribs and 11 (13%) had hypoplasia of the twelfth ribs.