Herbicides and teratogenesis.
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Biomedical subjects
Publications and source records attributed to A Lipson.
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A 29-year-old previously healthy man had a Pseudomonas aeruginosa pneumonia and bacteremia. Early recognition of the organism and institution of appropriate antibiotic therapy led to rapid clinical response and recovery. Although Pseudomonas is rarely the cause of pneumonia in a healthy host, it should be considered in the differential diagnosis.
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The association of the Albright syndrome (polyostotic fibrous dysplasia of bone, hyperpigmented skin macules, and endocrine disorders) with acromegaly has been infrequently substantiated. The case of an 18-year-old girl with the classic Albright syndrome and acromegaly is described. The patient had a history of coarsening of acral and facial features, an insulin-resistant form of diabetes mellitus and elevated fasting growth hormone values. Neuro-endocrine studies demonstrated failure of growth hormone to suppress to less than 5 ng/ml during an oral glucose tolerance test, and the abnormal release of growth hormone upon injection of thyrotropin-releasing hormone. Although L-dopa failed to decrease growth hormone levels, bromocriptine produced a modest decline in growth hormone within two hours of ingestion. The patient had also experienced secondary amenorrhea with sub-normal follicle-stimulating-hormone (FSH) and luteinizing hormone (LH) levels, both of which demonstrated a prolonged sluggish response to an injection of gonadotropin-releasing hormone (GnRH); this response suggested hypogonadotropic hypogonadism, possibly on the basis of a tumor involving both pituitary and hypothalamus. Sellar polytomography demonstrated an enlarged sella with dorsal erosion and an asymmetric floor. Computerized tomography of the brain visualized a suprasellar mass extending into the hypothalamus. These findings suggest a hypersecretion of hypothalamic releasing factors, pituitary hormones, or both as an etiology for the endocrinopathy in this patient, and lend support to the theory that the endocrinopathies associated with the Albright syndrome result from over-production of hypothalamic-releasing hormones or autonomous secretion of pituitary hormones from an adenoma.
Serum gastrin concentrations were measured in 22 untreated and 10 treated thyrotoxic patients. In contrast to similar studies carried out in Japan, serum gastrin levels were normal in hyperthyroid subjects. The mean value in hyperthyroid subjects (94.8 +/- 34.5 pg/ml) was higher than that in the treated patients (73.6 +/- 33.2 pg/ml), but the difference was not significant (P greater than 0.05).
The quantitative and qualitative distribution of bile salts in the duodenal juice of 13 patients with cystic fibrosis (CF) was studied after a test meal. The effects of triolein (TO), bovine serum albumin (BSA), and ricinoleic acid (RA) on the absorption of taurocholate (TCA) in the distal ileum of the rat in vivo was also studied. The mean (and ranges) of total bile salt concentrations, glycine: taurine conjugate ratios, and percentage of dihydroxy bile salts in the patients with CF and pancreatic insufficiency were 3.5 (1.3--6.6) mmol/l, 8.6 (greater than 10-3.1), and 37 (10--60) compared with control values of 7.4 (3.0--16.0) mmol/l, 3.0 (1.3--4.5), and 61 (52--70) respectively. The differences between the control and CF values were statistically significant (P less than 0.01--P less than 0.001). Three of the 13 CF patients had total bile salt concentrations less than 2 mmol/l, 8 had much higher glycine: taurine ratios, and 8 had a reduced percentage of dihydroxy bile salts. In 2 patients with normal pancreatic enzyme activities, duodenal bile salts were both quantitatively and qualitatively normal. TO (10 and 30 mmol/l), BSA (3%), and RA (5 mmol/l) had no inhibitory effect on the ileal absorption of TCA. These results show pronounced abnormalities of duodenal juice bile salts in CF with pancreatic insufficiency consistent with a broken enterohepatic circulation (EHC); such abnormalities may contribute to defective lipid absorption in CF. The data in the experimental animal do not support the suggestion that unhydrolysed dietary substrates play a role in the pathophysiology of the broken EHC.
Total serum thyroxine (T4), triiodothyronine (T3), T3 resin uptake (T3U), thyrotrophin (TSH), and reverse T3 (rT3) were measured in 209 healthy adults 20--89 yr old. Mean T4 values for men were stable throughout life, but in females under age 60, T4 values were significantly higher than in older women. Values for T3U in males were significantly higher than in females throughout all decades, although females had a significant increase in T3U after age 60. TSH values increased significantly in females over age 60. Throughout all decades, males had stable TSH levels that were slightly higher than the female results before age 60 and lower thereafter. Mean serum T3 declined similarly for both sexes with increasing age, although not to the extent previously reported. Men had significantly higher mean rT3 values over all decades than females, although female rT3 levels decreased after age 50 whereas males maintained stable values. The physiologic reasons for these findings may be due to sex-related changes in binding proteins and alterations in metabolic clearance rates, production, and degradation of these hormones with increasing age.
High-density lipoproteins (d=1.095--1.21) (H.D.L.( were isolated from six healthy men and women who added 4 to 6 eggs per day to their diet for 4 weeks and from five individuals who gradually increased their egg consumption to 3 per day over an 18-week period. Pre-diet and post-diet H.D.L.-binding activities for the cell-surface receptors of fibroblasts were compared by determining the quantity of 125I-labelled low-density lipoprotein which was competitively displaced by H.D.L. in binding, internalisation, and degradation assays. Irrespective of whether plasmacholesterol changed during the course of the diet, the binding activity of the post-diet H.D.L. was enhanced 2.6-fold to 4-fold compared with pre-diet activity. Furthermore, the increased binding activity in the H.D.L. could be accounted for by a minor, but potent, H.D.L. subfraction precipitated by heparin/manganese. Both binding activity and heparin precipitability appeared to correlate with an increase in arginine-rich apoprotein (apo-E) in the active H.D.L. subfraction. These data show that consumption of large numbers of eggs, whether or not it leads to an increase in plasma-cholesterol, does alter the properties of human H.D.L.
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Eighteen children with pneumocystis carinii pneumonia diagnosed over a period of 16 years at a children's hospital are reviewed. All had an underlying disease, either congenital immunodeficiency or a malignancy. 15 patients were treated, 10 with pentamidine isethionate alone, 2 with both pentamidine and co-trimoxazole, and 3 with co-trimoxazole alone. 12 of the treated group recovered and the 3 untreated patients died. The 3 deaths after treatment occurred in children receiving pentamidine alone, and in whom secondary factors contributed. The side effects of treatment with pentamidine were high, and included local reactions, hypoglycaemia, and uraemia. However, our results confirm that pentamidine is an effective treatment for pneumocystis carinii pneumonia in childhood. Co-trimoxazole may be an effective and relatively nontoxic alternative treatment.
Contamination of a powdered preparation of pancreatin with Salmonella schwarzengrund and S. eimsbuettel resulted in the infection of at least 31% of one group of paediatric patients with cystic fibrosis. The pancreatin contained very small numbers of Salmonellae, the infecting dose in at least one child being less than 44 organisms. More stringent bacteriological standards are needed for pharmaceuticals and foods used by paediatric patients.
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PURPOSE: To review the clinical features and possible aetiology of all cases of Möbius' syndrome presented at the Royal Alexandra Hospital for Children in Sydney, Australia, from 1963 through 1995. METHODS: All charts of patients suffering from Möbius' syndrome were reviewed, and each patient was thoroughly assessed by a geneticist and a pediatric ophthalmologist. RESULTS: Patterns of systemic and ocular clinical features became evident. Of 23 patients in the series, 10 (43%) had a history significant events in utero. CONCLUSIONS: All patients suffering from Möbius' syndrome require thorough assessment by a multidisciplinary team including a geneticist or pediatrician and a pediatric ophthalmologist. Significant vascular events of pregnancy may explain some cases of fetal vascular disruption to the brainstem.
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