Inherited disorders of 3-methylcrotonyl CoA carboxylation

The clinical course of 4 patients who had reduced activities of 3-methylcrotonyl CoA carboxylase (also called 3-methylcrotonylglycinuria) is described. Two children presented with a metabolic acidosis, one in the neonatal period and the other with episodes of acidosis that started in the second year of life. In the other 2 children neurological symptoms were prominent, one having infantile spasms and the other developmental regression with a skin rash and alopecia. Three of the children responded well to oral biotin and dietary protein restriction but the fourth, despite a biochemical response to biotin, has a severe neurological handicap. The clinical presentation of inborn errors of 3-methylcrotonyl CoA carboxylase is variable. Metabolic acidosis may not be conspicuous and instead neurological features may predominate.     

Suspected poisoning in children. Study of the incidence of true poisoning and poisoning scare in a defined population in North East Bristol

The distinction between true and suspected poisoning in children has not been made clear in previous work on childhood poisoning. A study of suspected poisoning in children under 15 years of age in a defined population of North East Bristol from November 1970 to July 1973 carried out by the Health Education Council Medical Research Division included 53,000 child-years at risk. The number of suspected poisonings was 3-4/1000 population aged under 15 years per year, with a higher incidence in younger age groups. Detailed investigation of the circumstances of the accidents carried out by a multidisciplinary team showed that at least 65%, and possibly as many as 78% were poisoning scares and not true poisoning. The evidence used by the casualty doctor and by the parents to diagnose poisoning was explored, and in many cases was circumstantial. Children with fathers in nonmanual occupations were over-represented. This may reflect differences in patterns of utilization behaviour rather than true differences in incidence.     

Exogenous lung surfactant: effect on radiographic appearance in premature infants

At birth, premature infants of 25-29 weeks gestation, at high risk for development of neonatal respiratory distress syndrome (RDS), were given a single dose (90 mg) of calf lung surfactant extract (CLSE) by intratracheal instillation. The frequency and severity of RDS were assessed with use of a simple radiographic scoring system in which pulmonary parenchymal densities and the prominence of the air-bronchogram effect were used as indicators of widespread atelectasis. Radiographs were obtained in surfactant-treated and control infants within the first 90 minutes of life as part of an initial evaluation of their pulmonary status. Subsequent examinations were performed at less than 24 hours and less than 48 hours of age. Radiographic assessment of lung disease compared consistently with coordinated data on oxygen and mean airway pressure requirements of the infants. Both indicated a significantly decreased frequency and severity of RDS in the infants treated with surfactant. The results provide supporting evidence of the effectiveness of exogenous lung surfactant replacement in mitigating RDS in very premature infants.     

Interpeduncular fossa sign: CT criterion of subarachnoid hemorrhage

A retrospective study of 100 cerebral computed tomography scans interpreted as displaying subarachnoid hemorrhage was undertaken to determine the frequency of blood in the interpeduncular fossa as a useful radiographic sign. The results indicate that the interpeduncular fossa sign is second only to blood in the Sylvian fissure-circular sulcus area as a reliable indicator of subarachnoid hemorrhage on CT scans.     

Myometrial invasion by endometrial carcinoma: sonographic assessment

The depth of myometrial invasion by endometrial carcinoma was evaluated using real-time sonography (US) in 20 patients with histologically proved adenocarcinoma of the endometrium. In 14 of 20 (70%) cases, US-based estimation of the depth of myometrial invasion was within 10% of the actual measurement in the gross specimen. The US-based estimation of tumor invasion was low in seven patients, high in four patients, and agreed with pathologic findings (+/- 5%) in nine patients. In four patients with polypoid intraluminal extension of tumor, a deeply invasive tumor was suspected on US but was not found on pathologic examination. In 12 superficially invasive tumors, the continuity of the demarcating subendometrial halo was intact in nine and incomplete in three. In six patients with deeply invasive tumors, this zone was partially disrupted in four, totally disrupted in one, and intact in one. Errors of estimation of the depth of myometrial invasion on US most frequently occurred when a tumor had a significant intraluminal polypoid extension. Demonstration of a subendometrial halo usually indicated superficial invasion, whereas the absence of a halo was frequently associated with deep invasion.     

US-guided percutaneous biopsy: use of a screw biopsy stylet to aid needle detection

Insertion of a screw biopsy stylet into a thin-walled biopsy needle greatly enhances detection of the needle during ultrasound-guided percutaneous biopsy. This technique is helpful when precise needle-tip localization is needed for biopsies of small lesions.     

Hemisphere preference, phobia, and depression

The present study was undertaken in order to examine whether depression and anxiety are related to preference for a right hemisphere mode of thinking. On the basis of a paper-and-pencil test presumably measuring hemisphere preference (Preference Test; Zenhausern, 1978), normal subjects (N = 75) were assigned to a group characterized by a left hemisphere thinking style, a group characterized by a right hemisphere thinking style and a group with mixed tendencies (left and right hemisphere tendencies). Additionally, subjects completed the Fear Questionnaire and the Depression Symptom Inventory. It was found that subjects with a right hemisphere thinking style reported more depressive and fear symptoms than subjects with a left hemisphere thinking style. This finding is in line with theories which assume that the right hemisphere is characterized by a negative, dysphoric emotional tendency.     

Pulmonary cystic disease: comparison of Pneumocystis carinii pneumatoceles and bullous emphysema due to intravenous drug abuse

A rare pulmonary manifestation of the acquired immunodeficiency syndrome or intravenous (IV) drug abuse is upper lobe cystic disease--pneumatoceles in Pneumocystis carinii pneumonia (PCP) and bullous emphysema in IV drug abuse. Because these disorders overlap, the radiographic findings in 56 patients were compared. During a 12-month period, 16 patients less than 40 years of age were found to have bullous emphysema; the 10 who were IV drug abusers constituted group 1. In the same time period, 40 patients with PCP were encountered; the eight (20%) who had or developed pneumatoceles constituted group 2. In both groups, the conventional radiographic manifestations of upper lobe cystic disease were similar. Eight patients underwent computed tomography of the chest. In five patients with bullous disease, the distribution of the bullous lesions was peripheral, with sparing of the central portions of the lungs. In contrast, PCP pneumatoceles in three patients were dispersed throughout the lung parenchyma.