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Previous studies suggest that patients with bilateral diaphragm paralysis are at high risk of developing chronic respiratory failure due to alveolar hypoventilation. However such studies mostly describe patients with generalised neuromuscular disorders in which other respiratory muscles are also weak. We therefore studied three patients with diaphragm paralysis (two due to neuralgic amyotrophy, one following traumatic diaphragm rupture) but no weakness of the other respiratory muscles. All were breathless on exertion (MRC Grade 4) with orthopnoea and abdominal paradox but with no symptoms of nocturnal hypoventilation. Maximum expiratory mouth pressure was >100% pred. consistent with normal intercostal and abdominal muscle strength. Vital capacity was 45–65% pred., falling by 36–55% when supine. Transdiaphragmatic pressure (Pdi) was zero during (a) tidal breathing, (b) inspiration to TLC, and (c) supermaximal stimulation of both phrenic nerves. It was also very low during a maximum sniff (10, 7.5 and 12.5 cm H2O respectively, normal >100 cm H2O). Maximum inspiratory mouth pressure was 35–70% pred. Arterial blood samples sitting at rest showed a pO2 of 10.6–12.3 kPa and normal pCO2 of 4.7–5.5 kPa. Continuous overnight monitoring on two consecutive nights, in normal sleeping positions (one on his side, two propped up), using an ear oximeter (Biox 3) and a Hewlett Packard capnometer, showed no significant pO2 desaturation nor any increase in pCO2. These patients have now remained stable for one to four years without developing any signs of nocturnal hypoventilation or respiratory failure, nor have daytime blood gases deteriorated. We conclude that in the absence of generalised respiratory muscle weakness, severe diaphragm weakness does not lead to respiratory failure.

A particularly high hypercalcemia (141 mg/ml) was observed in a man with Graves' disease. An intense muscle asthenia, with lack of dynamism and vomiting which may cause dehydration, are the most suggestive signs of hypercalcemia. Bone biopsy and above all parathormone estimations permit one to eliminate associated hyperparathyroidism. The efficacy of mithramycin used alone, without any other hypocalcemic drug, was remarkable. The direct responsibility of thyrotoxicosis as a cause of the calcium disorder seems undoubted but the precise mechanism of the hypercalcemia remains unexplained.

Fibrosis of the intestinal lymphatic vessels, produced in one case by tuberculosis and, in the other, by appendicitis and peritonitis, caused blockage of the main lymphatic vessels causing, clinically, a protein-losing enteropathy similar to that noted in congenital lymphatic diseases of childhood. In the laboratory, there was noted a fall in serum protein, lipid and cholesterol. A fat absorption test was very abnormal showing a flat curve. During laparotomy, there was discovered on the small intestine, the same layout of lymph vessels, resembling a lace network, as that observed in congenital malformations. Intestinal lymphography showed considerable stasis of the opaque substance and absence of injection of the lymph vessels in the mesentery.

Hyperosmolar diabetic coma revealed the presence of a pancreatic cancer in two patients. The first case was a 59-year-old man, without a history of diabetes, treated with prednisone for jaundice and marked weight loss over the last month, and admitted in hyperosmolar coma (346 m0sm/l). After recovery from the acute episode, a diagnosis of adenocarcinoma of the head of the pancreas was established following operation. The patient died six months later. The second case, a 71-year-old man also without a history of diabetes, was admitted in hyperosmolar coma (315 m0sm/l) during the course of a pulmonary infection. Rapidly fatal cholostatic jaundice appeared one year later. An adenocarcinoma of the head of the pancreas was demonstrated at autopsy. The diagnostic criteria in both cases were those of hyperosmolar diabetic coma. Though cases of combined diabetes and pancreatic cancer are well documented, only one case of hyperosmolar coma and cancer of the pancreas has been reported in the published literature. The pathogenesis of hyperosmolar diabetic coma is discussed. The fact that it developed during the course of a pancreatic affection could be explained by a functional reduction in insulin secretion, associated with a triggering factor such as dehydration, infection, hypoglycemic agent administration, etc... The onset of hyperosmolar diabetic coma in an elderly patient without a history of diabetes, especially with associated marked weight loss, should lead to investigation for a possible pancreatic cancer.

The authors report 4 cases of acute gout in the lower limb occurring soon after starting heparin therapy for venous thromboembolic disease. As none of the usual factors which induce gout were present, the possible roles of the venous thrombosis and the heparin therapy were examined. Intra-articular thermic and acid-base changes secondary to the thrombosis could have explained the site of the attacks of gout in 3 of our 4 patients; the fourth patient had normal phlebography; the relationship between the attack and the start of heparin therapy suggested that the heparin may have induced the gout, possibly by an interaction with the synovial proteoglycans leading to the precipitation of urate crystals in the joint. In the absence of references in the literature, these hypotheses need further confirmation.

The authors report a case of Waldenstrom's disease, the first sign of which, remaining for a long time the only sign, was a pseudo-carcinomatous infiltration of the stomach. Later, the patient developed a pleural localisation. These two lesions, gastric and pleural, are known but rare. The authors recall the main signs. They emphasize the long duration of the disease when treated by chlorambucil. A pathological IgM was found, not only in the serum of this patient, but also in the jejunal fluid aspirated during small intestinal biopsy.

A 82 years old woman with no past history of cardiac or pulmonary disease or asbestos exposure, but with chronic administration of paraffin oil as laxative, had lipid pneumonia and a primary neoplasic pleural effusion. From the reported case, the authors discuss the possible part of mineral oil as pleural carcinogenic factor (mesothelioma).

The goals of osteoporosis treatment are not only to decrease bone resorption, but also and mainly to increase bone formation. Present research is based on bone histomorphometry and prospective double blind therapeutic studies. Physical exercise, adequate calcium intake oestrogen therapy in postmenopausal or oophorectomized women have been shown to be effective in prevention of the disease. A moderate beneficial effect of the association of synthetic anabolic steroids with oral calcium has been recognized. Better results are expected from combined treatments such as low doses of calcitonin and oral calcium, or vitamin D metabolites and sodium fluoride, and from stimulating bone formation by low doses of parat-hormone.

The authors report the first two cases of lethal AIDS in haemophilia A in France. One French case of AIDS in haemophilia B has already been reported. The diagnosis was based on the observation of opportunist infections associated with severe depression of cell-mediated immunity in both cases. The possibility of active transmission of the LAV retrovirus by factor VII concentrate is discussed. This raises the problem of the signification of anti-LAV antibodies in haemophiliac patients and the control of products used for the treatment of haemophilia.

The cause of the reduced lung volume in the 'shrinking lung' syndrome of systemic lupus erythematosus (SLE) was investigated in 12 patients with the condition. Nine patients described persistent episodes of pleuritic chest pain. Narrow section (3 mm) computed tomography of the thorax revealed no interstitial fibrosis or significant pleural disease. Assessment of diaphragmatic function using manoeuvres more reliable than the maximal occluded efforts previously used alone to assess respiratory muscle strength, showed that diaphragm strength was unequivocally normal in nine of 12 patients. In three, maximum transdiaphragmatic pressure was moderately reduced, but phrenic nerve stimulation demonstrated that this was due to incomplete activation of the diaphragm during a maximal voluntary effort, rather than to a primary abnormality of the diaphragm. Results of maximum lung recoil pressures and dynamic compliance, and analysis of the 12-s maximum voluntary ventilation, suggested a restriction in chest-wall expansion, although it was not possible to identify the underlying cause of this on the basis of our results. We conclude that the 'shrinking lung' syndrome of SLE is not explained by a primary abnormality of the diaphragm.

We studied six patients with isolated bilateral paralysis or severe weakness of the diaphragm, present for 2 to 60 months (mean = 25), to document the clinical and respiratory sequelae of the condition. Severe diaphragm dysfunction was confirmed by the demonstration of the very low maximal transdiaphragmatic pressure (Pdi) generated by either a sniff (13 +/- 6 cm H2O, normal 148 +/- 24) or a static inspiration (11 +/- 8, normal 108 +/- 30) and during bilateral phrenic nerve stimulation (0.8 +/- 2.0, normal 22 +/- 4). Resting arterial blood gases were normal (SaO2 = 95 to 97%) and no oxygen desaturation occurred during maximal exercise on a treadmill. Maximum voluntary ventilation was low and related to PImax (r = 0.89). Overnight sleep monitoring showed that time spent in rapid eye movement sleep was normal (mean 55 +/- 36 min, range 26 to 117 min). Mean maximum increment in transcutaneous CO2 was within normal limits (6 +/- 2 mm Hg, range 3 to 9 mm Hg). Three patients had occasional brief episodes of oxygen desaturation (mean maximal decrease 13 +/- 10%, range 2 to 27%); however, only two of these spent a measurable proportion of total sleep time (TST) with an SaO2 of less than 80% (1% and 3% TST, respectively). No patient has developed any symptoms of nocturnal hypoventilation or chronic respiratory failure during periods of observation of up to five yr. We conclude that bilateral paralysis or very severe weakness of the diaphragm does not of itself lead to respiratory failure unless weakness of other respiratory muscles is present.

The authors report a new case of Behcet's disease in association with ankylosing spondylitis. A study of the HLA system in this patient, showed the presence of an HLA 5 (Behcet's disease) and the absence of HLA W 27 (ankylosing spondylitis). The authors recall the frequency of HLA W 27 during ankylosing spondylitis, whether primary or associated with psoriasis, or enteric disease. Desensitisation to autologous saliva proved a failure. A trial of D-Penicillamine produced no beneficial effect on the course of the mucosal ulcers and the inflammatory joints.

We report the fifth case of carcinoid tumour of Vater ampulla with Von Recklinghausen's neurofibromatosis. We have reviewed the literature and discussed the significance of such an association which seems fortuitous.

Two cases of familial hypocalciuric hypercalcemia (FHH) are reported with a review of the literature. Both cases had hypercalcemia, hypophosphatemia, variable parathormone (PTH) levels and hypocalciuria. The parathyroid glands were only slightly hyperplastic and subtotal parathyroidectomy did not reduce the hypercalcemia. FHH is an autosomal dominant congenital disease with high penetrance. It is characterised by hypocalcemia. This contradictory biological finding should alert the physician to the diagnosis and initiate a familial enquiry. The serum PTH and urinary cyclical-AMP levels do not distinguish FHH from primary hyperparathyroidism. Surgery is usually contraindicated because it is ineffective and because the disease is usually benign. Semi-quantitative bone histology in a patient with a high PTH level was normal. The value of bone biopsy in these cases is discussed.

The authors report a personal case of mitral incompetence, due to rupture of the chordae tendineae and note the signs of this disease. The patient first developed pulmonary oedema with thoracic pain, a mitral systolic murmur and a presystolic gallop rhythm, which suggested the diagnosis in a patient in sinus rhythm with a normal size heart, the left atrium was expansive and there were obvious signs of pulmonary congestion. The rapidly fatal course may be explained by the large number of ruptured chordae tendineae. Autopsy showed that the mitral valve was normal in texture. Histological study of the ruptured cord, showed lesions of dense hyaline fibrosis and mucoid infiltration of the basic substance.

An analysis of 80 cases of sarcoidosis admitted to the departments of respiratory medicine (36), rheumatology (19) and internal medicine (25) over a 7 year period, revealed a wide range of clinical presentations; there was a higher incidence of associated disease and a greater number of localisations of the disease in patients admitted to the department of internal medicine than in those admitted to the other two departments. On the other hand, bronchial biopsy was more commonly positive in patients admitted to the department of respiratory medicine whose respiratory function was more disturbed than the patients in the other two departments. The patients referred to the departments of rheumatology and internal medicine without radiological respiratory involvement had respiratory function tests and positive alveolar lavages. The prognosis was the same in all three departments; 50 p. 100 were treated with steroids. The recruitment of the patients in this series allows a different evaluation of the disease compared to series reported from more specialised departments.

The association of hyperthyroidism and thyroid cancer is rare. The commonest finding is multilobular goitres. When a toxic adenoma is associated with a thyroid cancer they are usually clearly separate lesions. A thyroid cancer lying within a toxic adenoma, as in this case, is a much rarer occurrence, and a review of the published literature suggests that the relatively frequent association of the two lesions is fortuitous. The possibility of a cancer occurring near, or within a toxic adenoma, is an argument in of surgical treatment of these formations.