Manifestations of pseudoxanthoma elasticum in childhood.

BACKGROUND: Pseudoxanthoma elasticum (PXE) affects the skin, retina and cardiovascular system. Most cases are related to mutations in the ABCC6 gene. The diagnosis is most often made late in the second or third decade of life. OBJECTIVES: To describe the manifestations of PXE before the age of 15 years. METHODS: Children under age 15 years with definite PXE were evaluated at a PXE referral centre, as were adult patients in whom serious manifestations of PXE had occurred before the age of 15 years. RESULTS: Our series included 96 patients; 15 (16%) had paediatric onset of the disease. Nine children were diagnosed at a mean age of 10 years, a mean of 2.5 years after the presenting symptoms. Cutaneous lesions were the presenting symptoms in eight. None had cardiovascular or ophthalmological symptoms. Six adult patients had had severe cutaneous and/or cardiovascular manifestations before the age of 15 years. Both adult patients with early extensive skin lesions had the PXE-like condition related to the GGCX gene. No ocular symptoms were recorded during childhood. CONCLUSIONS: Cutaneous manifestations of PXE are the same in children as in young adults. Absence of complications is common in childhood, but severe complications are unpredictable. The frequency of complications was retrospectively estimated to be 7% in the adults of our series, although this figure was probably an overestimate because of the recruitment bias in a referral centre. It is, however, important to consider PXE in the paediatric setting, as early diagnosis may be important to provide accurate information and discuss lifestyle adjustments in order to improve the prognosis of the disease.

Early and late hemodynamic evaluation after cardiac transplantation: a study of 28 cases.

Right heart catheterization was performed in 28 patients 1 week and 6 to 24 months after orthotopic cardiac transplantation. All patients were receiving cyclosporine and methylprednisolone orally. At early catheterization, right heart pressures as well as pulmonary capillary wedge pressure still remained above normal values in the majority of patients. Systemic arterial hypertension was already present in 29% of the patients and cardiac index was usually in the normal range, without any inotropic support. Results of late catheterization showed continuing improvement with return of right heart pressures to normal values in most but not all patients. Systemic arterial hypertension was noted in nearly all patients and is likely to be the result of hypervolemia secondary to cyclosporine-induced sodium retention. The increase in cardiac index, which was above normal values in 39% of the patients, was also consistent with hypervolemia in the setting of cardiac denervation. Thus, cardiac function at rest is satisfactory at short- and long-term assessment after cardiac transplantation, but the development and persistence of systemic arterial hypertension associated with cyclosporine use are a matter of concern in such patients.

[Operative risk in coronary bypass. A multivariate analysis of prognostic factors]. / Le risque opératoire du pontage coronaire. Analyse multivariée des facteurs pronostiques.

In order to determine the predictors of operative risk in coronary bypass surgery, we have studied 2112 consecutive patients who underwent isolated coronary bypass surgery between January 1979 and September 1984. The overall operative mortality (OM) was 4.4 percent (3.5 percent during the last 3 years). OM increases significantly with age (from O before the age of 30 to 12.3 percent after 70), the functional class (FC) of angina, the FC of dyspnea (NYHA), the creatinine blood level (23.5 percent if greater than 200 mumol/l), the left ventricular end-diastolic pressure and in case of reoperation (16.7 percent), as well as in women (11.6 percent). There is a trend toward higher OM in case of past history of ventricular tachycardia or arterial hypertension, atherosclerotic disease of the lower extremities, left ventricular dysfunction or severe stenosis of the left main coronary artery. OM is not increased in patients with multivessel disease, diabetes or with a past history of myocardial infarction, and is even decreased in obese patients. The variables selected by multivariate analysis were: creatinine blood level, then angina FC, sex, dyspnea FC, age, the absence os obesity, left ventricular dysfunction, the year of surgery and finally reoperation. These results, mainly based on simple clinical variables, should facilitate the therapeutic decisions in borderline indications of coronary bypass surgery.

Prevention of atrial fibrillation or flutter by acebutolol after coronary bypass grafting.

Supraventricular tachyarrhythmias are common after coronary artery bypass graft surgery (CABG) and may have deleterious hemodynamic consequences. To determine if acebutolol, a cardioselective beta-blocking drug, prevents such tachyarrhythmias after CABG, 100 consecutive patients, aged 30 to 77 years (mean +/- standard deviation 53 +/- 9), were entered into a randomized, controlled study. Exclusion criteria were: contraindications to beta-blocking drugs, left ventricular aneurysm, major renal failure, history of cardiac arrhythmia and cardiac arrhythmia during the immediate postoperative period. From 36 hours after surgery until discharge (usually on the seventh day), 50 patients were given 200 mg of acebutolol (or 400 mg if weight was more than 80 kg) orally twice a day (dosage than modified to maintain a heart rate at rest between 60 and 90 beats/min). The 50 patients in the control group did not receive beta-blocking drugs after CABG. The 2 groups were comparable in angina functional class, ejection fraction, number of diseased vessels, antianginal therapy before CABG, number of bypassed vessels and duration of cardiopulmonary bypass All patients were clinically evaluated twice daily and had continuous electrocardiographic monitoring and daily electrocardiograms. A 24-hour continuous electrocardiogram was recorded in the last 20 patients.(ABSTRACT TRUNCATED AT 250 WORDS)

[Long-term angiographic surveillance of operated ascending aorta dissections]. / Contrôle angiographique à long terme des dissections de l'aorte ascendante opérées.

Systematic angiographic surveillance of ascending aorta dissections demonstrates the frequency and diversity of often asymptomatic anatomical lesions. A study in 10 previously operated cases enabled differentiation with the prosthetic tube, usually implanted in the ascending aorta, of proximal postoperative lesions (residual dissection, aortic incompetence, aneurysm) which may require further surgery in the more or less long term, and distal lesions (the usual persistence of a false channel in type I dissections) which are generally left untouched.

[Diffuse interstitial pneumopathy associated with limited Sjögren's syndrome (author's transl)]. / Pneumopathies interstitielles diffuses au cours du syndrome de Sjögren limité (sans connectivite associée).

Diffuse interstitial pneumopathy was found to be associated with limited Sjögren's syndrome (i.e. without connective tissue involvement) in 5 women aged from 48 to 83 years. The respiratory symptoms (dyspnoea, unproductive cough and crepitations) appeared before the dry-eye-and-mouth syndrome was diagnosed in two patients and several years afterwards in three. Respiratory function studies showed a mixed restrictive and obstructive syndrome, perturbed Co transfer and increased total expiratory airways resistance. These changes reflected the underlying pathology, as revealed by bronchial and lung biopsies, which consisted of lymphocyte and plasmocyte infiltration and fibrosis of the interalveolar septa, peribronchial spaces and bronchial glands. Abnormal respiratory function tests in 5 other patients without any respiratory symptom suggest that subclinical and subradiological lesions of the lungs and bronchi are not uncommon in Sjögren's syndrome.