Impaired growth hormone secretion in patients operated for pituitary adenomas.

The frequency of growth hormone (GH) deficiency in patients operated for pituitary neoplasms of various size and type was investigated using the insulin tolerance test. 45 patients were included in the study. 20 of the patients had a non-hormone secreting pituitary neoplasm, 9 had GH-, 6 ACTH-, 7 prolactin secreting adenomas and 3 had a craniopharyngeoma. Complete endocrinological examination was obtained in all patients after pituitary surgery. Apart from patients operated for GH secreting adenomas, GH deficiency was very common after pituitary surgery (92%), even in patients operated for small lesions. Among the 45 patients LH/FSH deficiency was found in 33%, ACTH in 33%, TSH in 18% and ADH deficiency in 9% of the patients. In this study, impaired GH secretion was found to be independent of the size of the tumors and was present in nearly all patients after pituitary surgery (with exception of GH secreting adenomas). Deficiencies of other pituitary hormones were predominantly observed after surgery for large tumors.

Abnormal essential fatty acid metabolism in Darier's disease.

Fatty acid levels in plasma and erythrocyte cell membranes were determined in 13 Danish patients with Darier's disease and 21 Danish controls. Concentrations of the main dietary essential fatty acids, linoleic acid (18:2n-6) and alpha-linolenic acid (18:3n-3), were consistently modestly above normal; concentrations of the delta 6-desaturase metabolites of both linoleic and alpha-linolenic acids, however, were consistently and often significantly below normal. These results suggest that the capacity of the enzyme delta 6-desaturase activity is inadequate in patients with Darier's disease.

The clinical significance of straight-leg raising (Lasègue's sign) in the diagnosis of prolapsed lumbar disc. Interobserver variation and correlation with surgical finding.

Limitation of straight-leg raising (SLR) (Lasègue's sign) is considered an important test in the diagnosis of herniated lumbar disc disease. In a prospective study of 55 patients suffering from unilateral sciatica this sign was evaluated. Two aspects were investigated: 1) the interobserver variation, and 2) the correlation between the result of the test and the surgical finding. There was considerable interobserver variation among three observers concerning the measured angle at which pain was elicited. However, in 2/3 to 3/4 of the cases the variation amounted to 10 degrees or less. There was also some discordance in the classification of the type of pain that was elicited. Fifty-two patients underwent surgery; 45 had a prolapsed disc. SLR was "positive" in 49 cases, 43 of whom harbored a prolapsed disc. Crossed SLR was noted in 20, 19 of whom had a prolapsed disc at surgery. Absence of SLR limitation does not preclude the presence of a herniated lumbar disc.

Increased number of circulating suppressor T-lymphocytes in sun-induced multiple skin cancers.

The distribution of peripheral lymphocyte subsets was studied in fifteen patients with multiple nonmelanoma skin cancers, selected according to history of ultraviolet (UV) or X-ray exposure. The skin cancer was associated with previous heavy exposure to UV light in seven patients, and past exposure to x-rays in eight patients. In the UV group, the helper T-lymphocytes/suppressor T-lymphocytes (Th/Ts) ratio was abnormally low (P less than 0.01) compared with the ratios of the x-ray and control groups. The low Th/Ts ratio was associated with an absolute increase in the number of Ts. This suggests that heavy sun exposure may cause a permanent increase in the number of Ts in certain persons. These extra T-lymphocytes may in turn prevent immune rejection of transformed keratinocytes.

The nevoid basal cell carcinoma syndrome: sensitivity to ultraviolet and x-ray irradiation.

Demographic studies in patients with skin cancer have demonstrated the importance of exposure to ultraviolet and x-ray irradiation. This paper describes in vitro studies in peripheral lymphocytes from three patients with the nevoid basal cell carcinoma syndrome. Particular stress was placed on the following factors: (1) the distribution of the lymphocyte subsets, (2) the frequency of spontaneous sister chromatid exchange, (3) the effect of ultraviolet C (UVC) (254 nm) on deoxyribonucleic acid (DNA) synthesis, (4) the effect of UVC on the phytohemagglutinin-stimulated lymphocyte proliferation, and (5) the capacity to repair x-ray-induced DNA damage. Our data indicate that the distribution of the peripheral lymphocytes was normal, while the frequency of spontaneous sister chromatid exchange was high. The capacity of the lymphocytes to repair x-ray-induced DNA damage was low in all three patients. In two patients the UVC-induced DNA synthesis was reduced, while an increased UVC-induced inhibition of lymphocyte proliferation was observed. These cellular responses in vitro to ultraviolet and x-ray irradiation correspond to the clinical features of the nevoid basal cell carcinoma syndrome. A clearly defective in vitro cellular response to x-ray irradiation, reflecting the clinically evident x-ray sensitivity in the nevoid basal cell carcinoma syndrome, has not been reported previously.

Danish experience with the one-piece shunt. A long-term follow-up.

A review of the efficacy and complications of the simple one-piece ventriculoperitoneal shunt is given, based on a consecutive series of 61 children with nontumorous hydrocephalus. Control of the hydrocephalic state was satisfactory but was, however, complicated by a rather high frequency of slit ventricles and subdural effusions. No significant increase in the complication rate was observed among premature babies. The infection rate was low (3%) and no visceral perforations were observed. The one-piece shunt can be recommended, especially for smaller children with hydrocephalus.

Erythema elevatum diutinum and pre-AIDS.

Erythema elevatum diutinum (EED) is a chronic disease with symmetrical persistent erythematous nodules and plaques primarily in an acral distribution. EED is often associated with infections, especially of streptococcals. An immunological reaction has been proposed as pathogenetic mechanism. We describe a patient, who developed EED secondary to a LAV/HTLV III positive lymphadenopathy syndrome. Immunological investigation of a skin lesion and a lymph node biopsy is described.

Raynaud's phenomenon with oral manifestation in systemic lupus erythematosus.

A 24-year-old woman with discoid lupus erythematosus developed systemic lupus erythematosus after 6 years. One of the clinical features was Raynaud's phenomenon in the fingers and toes, and furthermore Raynaud's phenomenon appeared in the tongue when exposed to cold and windy weather.

Mechanism of slowing of mucociliary transport induced by SO2 exposure.

Responses of nasal mucociliary transport mechanisms to exposure to 6 ppm SO2 were studied in chickens in vivo. This model takes advantage of the natural cleft palate which exposes the mucociliated base of the nasal septum. Exposure to 6 ppm SO2 decreased the mucociliary transport rate along the base of the nasal septum. The minimum force required to move an iron particle along this area of mucous membrane by use of a magnetic field in vivo increased significantly after SO2 exposure, while the minimum force required to move an iron particle on a pool of mucus collected from the same chicken and tested in vitro showed no change after SO2 exposure. The elastic recoil distance of mucus was measured both in vivo and in vitro. The in vivo recoil distance decreased significantly after SO2 exposure, while SO2 exposure did not change recoil distance in vitro. It is proposed that exposure of chickens to SO2 results in the formation of multiple points of adhesion of strands of mucus between the acinar gland cells and the emergent extracellular mucus or adhesion of a mucous blanket to the cilia, causing mucociliary transport to be retarded or static.

Effect of mechanical stimulation on mucociliary clearance of chicken sinus.

The hypothesis that homeostatic control mechanisms control mucociliary function in ciliated mucous membranes was induced artificially by means of mechanical stimulation. The edge of right palatine cleft was stimulated mechanically by gentle touching with a dissecting needle, and sinus clearance time was recorded as soon as mechanical stimulation was initiated. Mechanical stimulation caused acceleration of mucociliary flow of the sinus; sinus clearance time was accelerated on the side adjacent to the mechanically stimulated side of the palatine cleft, but not on the opposite side. Therefore, the reflex may be effective only on the stimulated side. We investigated the effect of nerve blockers on mechanical stimulation. Mucociliary clearance in the chicken sinus was not affected by parasympatholytic agents, but was decelerated by the beta-adrenergic blocker. The effect of nerve blockers on the mechanical stimulation showed that parasympatholytic agents blocked mechanical stimulation, while sympatholytic agents did not completely block the response. These data suggest that mucociliary clearance may be regulated by the reflex of parasympathetic and partially sympathetic nerve fibers.

The carpal tunnel syndrome and amyloidosis. A clinical and histological study.

Twenty-six non-randomized patients with carpal tunnel syndrome are presented. It is documented that three out of four patients may be diagnosed pre-operatively by five or more clinical parameters. All patients were screened for amyloidosis in biopsies from the carpal tunnel. One patient presented amyloid deposits in the transversal carpal ligament. The importance of macro- and microscopic findings in the carpal tunnel inclusive local amyloidosis for the pathogenesis of the carpal tunnel is discussed. It is concluded that provided systemic amyloidosis is not suspected, screening for amyloidosis may have diagnostic interest, however without therapeutic consequences and therefore unnecessary.

Effect of infection and SO2 exposure on nasal and paranasal mucociliary clearance in intact chickens.

The hypothesis that homeostatic control mechanisms control mucociliary function in ciliated mucous membrane was tested. Nasal mucociliary transport rates were recorded in chickens in vivo at successive intervals during exposure to SO2 or after inoculation with Newcastle disease virus (NDV), or both. Either agent alone caused deceleration of the turbinate clearance. However, when SO2 exposure was limited to one nasal fossa and turbinate mucociliary rates were determined in the unexposed and infected side, the two acted antagonistically and yielded approximately normal rates. Exposure of the nasal mucosae to SO2 caused decreased rates of sinus clearance, while NDV infection of nasal membranes induced increased rates of sinus clearance. Exposure of nasal mucosae to both agents acted antagonistically to effect rates of sinus clearance in normal ranges. These data support the idea of homeostasis.

Differences in mucus-stimulating serum fractions of cystic fibrosis patients and controls.

In nature, the urn cell complexes which swim in the coelomic fluid of the marine invertebrate, Sipunculus nudus, produce "tails" of mucus in response to bacterial pathogens. Since they produce measurable tails of mucus in vitro, suspensions of urn cell complexes provide a bioassay for mucus-stimulating substances (MSS) in biological fluids, including several human body fluids. Heat-activated seawater dilutions of human serum contain MSS. Serum from 87 cystic fibrosis (CF) homozygotes, 60 obligate heterozygotes, and 45 controls were fractionated on a Sephadex G-200 gel filtration column. After subsequent heating for 4 min at 85 degrees C, the fractions of all normal sera showed two characteristic peaks of MSS activity. The pattern differed in heated serum fractions of CF patients, in that the second peak was lacking in 59% of individual tests. The pattern was intermediate in heterozygote sera. Of the 36 CF serum fractions which did have two peaks of activity, 89% had the predominant activity in peak 1. The frequency of single peaks of activity increased with patient age, from 33% in those under 10 years to 75% in those over 16. The molecular weight of peak 1 is about 75,000 daltons, of peak 2 about 30,000. One may speculate that the frequent lack of peak 2 serum components may be associated with the inability of most CF patients to produce normal mucus following respiratory infection.

Effect of SO2 exposure on nasal mucociliary clearance in intact chickens.

The time required for mucociliary clearance from the chicken nasal turbinate and from the maxillary sinus was investigated in individual animals by using a newly designed plastic holder for the experimental animals. Determined in this way were: 1) the effect of SO2 exposure on sinus and turbinate clearance time, 2) the effect of the nerve blocking drugs atropine, scopolamine, reserpine, and propranolol on turbinate clearance time, and 3) the effect of these nerve blockers on clearance rates in chickens exposed to SO2. Turbinate mucociliary clearance was measured at 5 intervals per day, during 1 to 7 hr after exposure, for 7 consecutive days. Sinus clearance time was measured twice daily 1 to 4 hr after exposure. Turbinate clearance time in birds exposed to 6 ppm, and sinus clearance time in birds exposed to 40 ppm intermittently for 2 consecutive days both increased strikingly as a direct effect of SO2 exposure. However, continuous exposure to 6 ppm of SO2 during 16 hr per day for 7 consecutive days produced double peaks of increased turbinate clearance time with intervening recovery periods, suggesting an intranasal mucociliary homeostatic response. In individual animals, 26 of 35 animals (75%) exposed to 5 ppm, and 5 of 10 animals (50%) exposed to 20 ppm continuously during 16 hr per day for 7 consecutive days showed the same patterns. Reserpine and propranolol, which are sympatholytic agents, produced decelerated intranasal transport rates. Atropine and scopolamine, which are parasympatholytic agents, did not affect clearance rates. These nerve blockers, however, blocked the biphasic recovery pattern due to SO2 exposure. This blocking effect was statistically significant for atropine and reserpine 1 hr after injection.

Mucus secretion-stimulating activity in human lymphoblastoid cells.

Two fractions isolated from cultured lymphoblastoid cells stimulated mucus secretion from the urn cell complex of the marine invertebrate Sipunculus nudus. The activity detected in the nuclear fraction was trypsin-sensitive, and it increased in response to specific nucleotides.