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Biomedical subjects

F Magee

Publications and source records attributed to F Magee.

16 recordsLinked to original sources

Bronchial casts in children: a proposed classification based on nine cases and a review of the literature.

Bronchial casts are characterized by the formation of obstructive airway plugs that may be large enough to fill the branching pattern of an entire lung. The condition is rare but can occur at any age. Casts may be secondary to underlying diseases such as asthma and cystic fibrosis, but there are often no predisposing factors. There is no accepted classification system for bronchial casts; but only a confusion of descriptive terms such as mucoid impaction, fibrinous bronchitis, and pseudomembranous bronchitis. Based on a review of nine well-documented cases and the available literature, we have separated bronchial casts into two well-defined groups: Type 1 (inflammatory), consisting of casts composed mainly of fibrin with a dense eosinophilic inflammatory infiltrate; and Type 2 (acellular), consisting of casts composed mainly of mucin with little or no cellular infiltrate and occurring only in children with congenital cyanotic heart disease. Acute mortality was high in both groups. Survivors of Type 1 casts seem to be well controlled with inhaled steroids. Optimal therapy for patients with Type 2 casts is not clear; the prognosis probably depends on underlying cardiac status. We hope that this simple classification will provide a framework for further study of this obscure condition.

Adolescent↗

Histochemical studies of intestinal epithelial goblet cell glycoproteins during the development of the human foetus.

Histochemical studies performed on specimens of intestine from 12 to 37-week human foetuses showed that the epithelial glycoproteins of the goblet cells of the small intestine are non-sulphated sialoglycoproteins containing neutral sugar (hexose, 6-deoxy hexose or N-acetyl hexosamine residues with Periodic acid-Schiff (PAS) reactive vicinal diols), sialic acids without O-acyl substituents, smaller and variable quantities of sialic acids with O-acyl substituents at positions C8 or C9 (or with two or three side chain substituents) and O-acyl sugars (neutral sugars with an ester substituent blocking PAS reactivity). In the lower small intestine glycoproteins containing 8 (or 9)-O-acyl sialic acids are first observed in goblet cells at the tips of the villi. As the foetus matures their quantity increases and they are found in goblet cells located along the length of the villi. Smaller quantities of O-acyl sialic acids and traces of O-acyl sugars occur in the goblet cells of the upper small intestine. The colonic goblet cells contain sulphosialoglycoproteins of two types. The first type, found in the majority of specimens, contains O-sulphate ester, neutral sugar, O-acyl sugars and 8 (or 9)-O-acyl sialic acids. The second type contains O-sulphate ester, neutral sugars, and sialic acids which are either without side chain O-acyl substituents or are a mixture of such acids and 8 (or 9)-O-acyl sialic acids; O-acyl sugars are reduced or absent.(ABSTRACT TRUNCATED AT 250 WORDS)

Animals↗

Pulmonary vascular structure and function in chronic obstructive pulmonary disease.

Cardiac catheterization data from eight patients with severe chronic obstructive lung disease and pulmonary hypertension at rest (greater than 25 mm Hg) were compared with those obtained from 14 patients with mild to moderate disease whose pulmonary artery pressure was within the normal range at rest (mean 15 (SEM 1) mm Hg), but increased with exercise (30 (2) mm Hg). We obtained lung sections from necropsy material from the group with severe disease, and from surgical specimens in the group with mild to moderate disease, and compared the structure of the vasculature in these groups with that obtained from surgical specimens in a non-smoking control group of seven patients. Oxygen administration either at rest or during exercise did not greatly affect the pulmonary arterial pressures. When cardiac index was plotted against pulmonary artery pressure at rest and during exercise and extrapolated to the axis there was no evidence for a critical closing pressure in either group. The vessels in the groups with mild to moderate and severe chronic obstructive lung disease showed intimal thickening (each 19% (SD 0.5%)) by comparison with the non-smoking group (16% (0.5%]. The group with severe disease, in addition, had medial hypertrophy (27% (0.5%) versus 24% (SD 1%) in the non-smoking group). These data are consistent with the idea that the diseased vessels are distorted and rigid. The lack of effect of breathing oxygen on the vascular response at rest and during exercise suggests that hypoxic vasoconstriction has a minimal role in the pulmonary hypertension of chronic obstructive lung disease. The data suggest that the intimal changes could narrow the vessel calibre in those patients with mild to moderate disease, and that the thickened media present in the vessels from patients with severe disease may act in concert with the enlarged intima to produce more severe vascular obstruction.

Blood Pressure↗

Neural differentiation in Wilms' tumor.

Immunoperoxidase studies using antibodies to neuron-specific enolase (NSE), glial fibrillary acidic protein (GFAP), and S100 were used to confirm the presence of neural differentiation identified histologically in five cases of Wilms' Tumor. These observations invite reappraisal of Masson's proposed neuroectodermal histogenesis of Wilms' tumor because they are difficult to reconcile with the currently accepted metanephrogenic blastematous origin. They also provide a stimulus to examine the expression of neural markers during normal renal development and differentiation.

Child↗

Malignant mesothelioma caused by childhood exposure to long-fiber low aspect ratio tremolite.

A 41-year-old man was found to have a malignant mesothelioma of the pleura. During childhood in Corsica, he had been exposed at home to chrysotile ore from the Canari mine. Analysis of lung mineral content revealed background levels of chrysotile but an elevated level of tremolite and actinolite asbestos. The latter had a geometric mean length of 3.7 microns, a value considerably longer than we have found for tremolite and actinolite from Quebec chrysotile miners but roughly the same as the mean length of amosite and crocidolite in workers with occupational amphibole exposure. No tremolite or actinolite fibers of length greater than 8 microns microns and width less than 0.25 micron were observed. The mean aspect ratio of the tremolite and actinolite fibers was 7, a value similar to that found in chrysotile miners with mesothelioma but considerably less than the mean aspect ratio of amosite and crocidolite from those with occupational exposure. These data suggest that long-fiber tremolite is a potential mesothelial carcinogen in humans, and that fiber length is more important than fiber aspect ratio in this regard.

Adult↗

Pulmonary capillary hemangiomatosis.

Pulmonary capillary hemangiomatosis (PCH) is a rare cause of pulmonary hypertension; only three cases have been reported. Four additional cases are described in this report. All of the patients in this study presented with signs and symptoms of pulmonary hypertension, and in none was a correct morphologic diagnosis made during life. Histologically, the most striking feature was the presence of numerous cytologically benign thin-walled capillary-sized blood vessels proliferating diffusely through alveolar walls and in and around larger vessels and airways. Venous infiltration was associated with intimal fibrosis and secondary veno-occlusive disease (VOD). Because of the presence of hemorrhage, the apparent interstitial widening and inflammation, and the venous changes, the condition in these cases was initially misdiagnosed as interstitial fibrosis or VOD. However, the identification of proliferating and invasive capillaries, which are unique to PCH, led to the correct diagnosis. Although the nature of PCH is unknown, it behaves like a low-grade vascular neoplasm.

Adolescent↗

Two unusual pathological reactions to nitrofurantoin: case reports.

Idiosyncratic pulmonary reactions to nitrofurantoin are not unusual, often presenting as eosinophilic pneumonia. We report two cases of uncommon pathological reactions, one resembling a hypersensitivity pneumonitis and the other mimicking a giant cell interstitial pneumonia.

Aged↗

Pulmonary capillary hemangiomatosis.

We report a case of pulmonary capillary hemangiomatosis affecting a 35-yr-old Caucasian woman. Progressive pulmonary hypertension was documented by serial cardiac catheterizations, and pulmonary function studies showed changes suggestive of chronic pulmonary congestion. Although a difficult condition to diagnose during life as it may mimic veno-occlusive disease, capillary hemangiomatosis has a distinct histopathologic picture.

Adult↗

Prolactin in hirsute women: possible roles for androgens in suppressing basal levels, and for oestrogens in enhancing TRH-induced responses.

Hyperprolactinaemic patients occasionally demonstrate hirsutism and elevated levels of DHA-S, a weak androgen of adrenal origin. Abnormal adrenal function is frequently observed in hirsute patients. These observations prompted speculation that prolactin may modulate normal adrenal secretion and that derangements of adrenal androgen secretion may be due to abnormalities in prolactin. In this study we examined the possibility that elevated prolactin levels may be involved in the pathogenesis of hyperandrogenaemia in hirsute patients. However, basal prolactin levels in hirsute women, with or without menstrual disturbances, 201 +/- 24.3 mU/l (mean +/- SE) and 192 +/- 24.3 mU/l respectively, were significantly suppressed below levels in normal women, 289 +/- 12.2 mU/l. The prolactin responses to stimulation with TRH and to suppression with L-dopa were also studied in hirsute patients. The prolactin response to TRH (maximum increment or integrated response) was exaggerated significantly in hirsute women with menstrual disturbances when compared to normal women, to hirsute women with normal menses or to normal men. This abnormal response may have been due to elevated oestrone levels present in patients with oligomenorrhoea (318 +/- 49.5 pmol/l compared to 191 +/- 12.1 pmol/l in normal women and 161 +/- 15.5 pmol/l in hirsute women with normal menses, P less than 0.05). There were no abnormalities detected in the suppression of prolactin in response to L-dopa in any of these groups. These findings do not support a role for prolactin in the pathogenesis of hyperandrogenaemia in hirsute patients. However, elevated androgen levels in women may bring about suppression of basal prolactin levels to values seen in normal men. (ABSTRACT TRUNCATED AT 250 WORDS)

Adrenal Glands↗

Adrenal abnormalities in idiopathic hirsutism.

The possibility that abnormal adrenal androgen production may be present in patients with idiopathic hirsutism was examined. Plasma testosterone, dihydrotestosterone and androstenedione levels were elevated in hirsute patients. In response to exogenous alpha 1-24 ACTH the increments in plasma androstenedione, dehydroepiandrosterone (DHA) and cortisol were significantly greater in hirsute patients than in normal subjects. The testosterone response was exaggerated following endogenous stimulation induced by metyrapone. Treatment with dexamethasone, 0.5 mg each night for 3 months, corrected both the androgen excess and the exaggerated androgen responses but not the excessive cortisol response to stimulation. These observations indicate adrenal abnormalities in idiopathic hirsutism. The dissociation of cortisol and adrenal androgen responsiveness following dexamethasone suggests that the abnormalities observed may be due to excessive adrenal androgen production stimulated by a dexamethasone-suppressible factor other than ACTH. Excess adrenal androgen production may be the primary disorder leading to the development of idiopathic hirsutism.

17-Hydroxycorticosteroids↗

Routine heparin therapy inhibits adrenal aldosterone production.

While heparin-induced aldosterone deficiency has been sporadically reported, it is not known whether heparin always inhibits aldosterone production to a variable extent or if this is an idiosyncratic effect, nor is the mechanism underlying the phenomenon known. We have examined plasma aldosterone, PRA, and aldosterone to renin activity ratios in 20 patients before, during and after treatment with heparin. Aldosterone cell during heparin treatment from 73.5 +/- 20.5 to 36.8 +/- 11.2 pg/ml (P less than 0.05) and rose after its withdrawal to 94.8 +/- 37.1 p/ml (P less than 0.05). PRA rose with heparin treatment from 2.8 +/- 1.0 to 6.1 +/- 1.6 ng/ml . h (P less than 0.05) and fell to 2.4 +/- 0.5 ng/ml . h (P less than 0.05) when the drug was withdrawn. Aldosterone to renin activity ratios, which are indices of aldosterone responsiveness to angiotensin, fell from 59.5 +/- 1.7 to 25 +/- 14.9 (P less than 0.01) with heparin treatment and rose after withdrawal of the drug to 58.5 +/- 24.9 (P less than 0.01). There was a significant small fall in serum sodium levels with the introduction of heparin, but none of the patients developed clinical mineralocorticoid deficiency. Although heparin consistently perturbs aldosterone production in the glomerulosa cell, this effect is not clinically significant when normal adjustments can be made in the generation of angiotensin. However, where limitations in the renin-angiotensin-aldosterone axis exist, e.g. in diabetes mellitus, mineralocorticoid insufficiency may be precipitated by heparin.

Adrenal Cortex↗

Amenorrhea with cryptic hyperandrogenemia.

It is current practice to assume that when menstrual disturbances are associated with androgen excess there will be additional clinical evidence of this. We have recently seen three women with secondary amenorrhea who did not have any other clinical features of androgen excess, i.e. hirsutism, acne, etc., but who had elevated plasma testosterone and androstenedione levels in addition to increased estrone values. Correction of hypertestosteronemia and elevated estrone levels was followed by ovulation, regular menstruation, and pregnancy. Variable tissue sensitivity to androgens probably accounts for these observations. If measurement of androgen levels is omitted in the evaluation of patients with amenorrhea without hirsutism, cryptic hyperandrogenemia will remain undetected. Plasma testosterone levels should be measured in all patients with amenorrhea of unknown etiology, and only if these are normal should a diagnosis of functional amenorrhea be assigned.

Adolescent↗

Multiple pulmonary chemodectomas in a child: results of four different therapeutic regimens.

PURPOSE: Chemodectomas (or paragangliomas) are rare tumors of neuroendocrine chemoreceptors, such as the carotid body. This report describes a case of multiple pulmonary chemodectomas in an adolescent and discusses the results of four therapeutic regimens. PATIENTS: At 15 years of age, the patient had cough and fatigue. Investigation revealed numerous 1- to 2-cm diameter nodules throughout both lungs. Biopsy revealed multiple pulmonary chemodectomas of uncertain malignant potential. No extrapulmonary primary site could be found. RESULTS: Because of deteriorating pulmonary function, she was treated with courses of etoposide-cisplatin and subsequently somatostatin without effect. She finally responded to a course of doxorubicin and streptozocin. She is currently maintained on interferon-alpha 2B but her measured vital capacity continues to fall slowly, reflecting increased tumor growth. Because there is still no evidence of extrapulmonary spread, she is considered to be a candidate for lung transplantation. CONCLUSIONS: A doxorubicin-streptozocin combination produced a temporary remission of this patient's multiple pulmonary chemodectomas.

Adolescent↗