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During six consecutive months, seven patients admitted to our ICU (15 beds, general ICU, approximately 300 intubated patients per year) for acute respiratory failure requiring intubation and mechanical ventilation presented with a peculiar neuromuscular disorder. After the occurrence of this cluster group of patients, we detected two more similar but isolated cases in the following 18 months, ie , altogether 9 patients in 2 years of observation, or 1.55 percent of all intubated patients in our ICU. Sedation was achieved using midazolam, curarization was effected with the neuromuscular nondepolarizing agent pancuronium bromide (PB), and corticosteroids were administered to eight patients. Shortly after discontinuation of sedation and curarization, we observed a persistent tetraparetic syndrome and/or peroneal palsy with a concomitant increase of serum creatine kinase (CK). None of the patients was septic or had the multisystem organ failure. A strong association between CK increase and PB administration was found, whereas no patient suffered severe liver or kidney failure. The duration of the neurologic deficit ranged from 4 to 52 weeks, with only partial recovery for some patients; the duration of dysfunction was apparently related to the total dose of corticosteroids received. Two patients had difficulty being weaned from the respirator and required tracheostomy. Electrophysiologic studies showed signs of axonal neuropathy and myopathic changes, ie , motor units of brief duration, small amplitude, overly abundant for the voluntary effort being exerted. Muscle biopsies showed significant myopathic alterations, with foci of muscle necrosis in most patients and minimal lymphocytic inflammation in one patient. The neurologic complication described differs from the polyneuropathy in critically ill patients. Furthermore, PB or corticosteroids or both appear to be the causal agents. The duration of the neuromuscular dysfunction may be related to concomitant steroid therapy. The CK enzyme seems to be a marker of the disorder. This disorder is associated with myopathic alterations and axonal degeneration in some patients. Pancuronium bromide should be used with caution, particularly when associated with steroids therapy, and it may cause difficulty in weaning patients from the respirator.

It has recently been emphasised that a subset of patients with type 2 Gaucher disease die in the neonatal period. This report describes an Afghani family with two conceptuses having severe, prenatally detected Gaucher disease. Mutational analysis showed that the family carried a known complex allele which included mutations at amino acids L444P, A456P, and V460V. Although glucocerebrosidase RNA was present, an affected fetus had virtually no glucocerebrosidase cross reactive material on western analyses. The severe clinical course and pathology observed in these patients resemble that of the null allele Gaucher mouse, and suggest that the absence of glucocerebrosidase activity results in early death.

Chorionic villus sampling performed for advanced maternal age revealed trisomy 3 in 20% of mitoses studied after a semi-direct chromosomal harvest. Amniocytes and cord blood showed a non-mosaic 46,XY karyotype. The birthweight of the normal newborn was at the tenth percentile. Analysis of term placenta by cytogenetics and by fluorescent in situ hybridization (FISH) confirmed the presence of the trisomy 3 in 20% and 12%, respectively, of cells from two peripheral placental biopsies. Placental histology was heterogeneous, some portions showing immature, edematous and undervascularized villi. DNA analysis confirmed the biparental origin of the chromosomes 3 in the child, whose development is normal at 36 months.

Cardiac pacing has become a major therapeutic tool in the treatment of a wide variety of conduction disturbances of the heart, many of which are life-threatening. The sophistication of modern designs, together with the introduction of the automatic implantable cardioverter defibrillator, has broadened the indications for the implantation of cardiac pacemakers, making them serious competitors to drug therapies for most of the aforementioned conditions. Both systems, alone or combined, are now widely used in various pathological heart conditions, and have also been adapted for use in infants and children.

The optical properties of both electrochemically anodized and chemically stain-etched porous silicon are presented. Fourier transform infrared (FTIR) spectroscopy showed that absorbance in stain-etched samples was 3x and 1.7x greater than in anodized samples for the SiH/SiH2 stretch and scissors-bending modes, respectively. Also, oxygen is detected in stain-etched samples immediately after formation, unlike anodized samples. Photoluminescence measurements showed different steady state characteristics. Electrochemical-etched silicon samples stored in air increased in photoluminescent intensity over time, unlike the stain-etched samples. A photoluminescent device made by anodization on epitaxial p-type material (0.4 Ωm) on n-type substrate (0.1 Ω-cm) did not exhibit electroluminescence.

A case of glomerulocystic disease of the kidneys in a newborn is described. The kidneys were very large and diffusely cystic. Histologically, all portions of the nephron were involved and some of the cysts were lined by a prominent hyperplastic eosinophilic epithelium. This, together with the presence of numerous cardiac rhabdomyomas, a nodular haemangiomatous harmartoma and histiocytoid cell nests in the spleen were strongly suggestive of tuberous sclerosis. The relation between glomerulocystic kidney disease and tuberous sclerosis is discussed.

Biopsy of the triceps surae muscle was carried out in 11 patients who had sustained a spontaneous rupture of the Achilles tendon during sports. All the specimens showed important histological and ultrastructural changes, the most striking being the presence of large quantities of lipid droplets within the muscle fibres. A factor common to every patient was a period of inactivity before resuming sports. Preliminary training may be valuable in producing positive adaptive changes in the muscles, and the consequent improvement in function may reduce the risk of rupture of the Achilles tendon.

We report the post-mortem findings of the case of a 31-year-old male who, through sexual contacts with a female drug addict, was found to be HIV-positive and developed the acquired immunodeficiency syndrome (AIDS) 2 years later. He was treated for various opportunistic infections over the next 7 years when he presented with cardiac abnormalities and multiple cerebral lesions which were responsible for his death. The results revealed Aspergillus fumigatus endocarditis and myocarditis with mycotic thromboembolic extension to the brain, spleen, kidney and pancreas. We review the literature of Aspergillus infection in patients with AIDS and more specifically cardiac involvement with this pathogen.

We report the clinical and pathological findings in a young female patient who was a chronic drug addict for over eight years. In 1984, she presented with tricuspid endocarditis and three years later, was found to have irreversible pulmonary hypertension. Post-mortem examination revealed not only persistent tricuspid endocarditis and evidence of repeated pulmonary thromboembolism explaining her pulmonary hypertension but also a diffuse eosinophilic myocarditis, with myocardial fibre degeneration. The combined pulmonary and myocardial lesions have rarely been documented in the same drug addict. The relationship of these various pathological findings are discussed in the light of the existing literature.

Infrared absorption spectra of 108 borophosphosilicate glass (BPSG) thin films produced in a multiple-wafer low-pressure chemical vapor deposition (LPCVD) reactor were collected to enable the development and testing of a rapid and inexpensive method for determining if films are within the desired specifications. Classification of samples into good and bad product categories was made by applying principal component analysis to the spectra. Mahalanobis distances were used as the classification metric. The highest overall percentage of correct classification of samples based upon their spectra with two-step classification was 95%. The misclassified samples were, however, within the error of the reference methods that were used in making the original classification against which the infrared (IR) classification methods were tested. The classification errors are thus just as likely to be a result of misclassification by the reference method rather than errors by the IR classification. Although reference measurements were used in this article for the original classification of the samples, these expensive and time-consuming reference methods can be eliminated simply by building classification models on samples determined to produce a product within the correct device specifications. The IR classification methods presented here hold great promise as a tool for rapid quality control of BPSG deposition.

The pathological features of 14 cases of human African trypanosomiasis (HAT) occurring in Uganda over an 8 year period are described. Three cases were clinically proven and in all 14 cases a chronic meningoencephalitis was found. In 2 cases there was histological evidence of ganglion radiculitis and in one of these chronic choroiditis and peripheral neuritis associated with chronic myositis were present. The cardiac lesions consisted of a chronic pancarditis of varying degree in 8 cases and in 3 a generalized valvulitis was observed. In 2 cases, specially investigated, generalized lesions of the conducting system were noticed. Previous histopathological descriptions of HAT are briefly reviewed. The present findings are compared with some of those recorded in human american trypanosomiasis and experimental African trypanosomiasis.

The histological and ultrastructural aspects of three cases representing different types of compartment syndromes are analyzed. In the acute syndrome edema is the prominent feature. The two chronic cases are characterized by an accumulation of intermyofibrillar lipid globules. All three have in common mitochondrial enlargement, disorganization of the cristae and paracrystalline inclusions. These pathological findings are discussed in the light of lesions seen in striated muscle ischaemia or in certain metabolic myopathies.

A case of double inlet right ventricle in which both atrioventricular valves opened completely into the right ventricle is described. The left atrium communicated with the right ventricle by way of an opening low in the atrial septum and there was a large ventricular septal defect. The pulmonary trunk and aorta took their origin from the right and hypoplastic left ventricles respectively. The literature on this rare cardiac anomaly is reviewed and the morphological findings in this case are compared with those of the recorded cases. A short discussion of the possible embryogenesis is given.

The case of a 14-year old girl presenting with headaches, severe progressive hypertension and high plasma renin levels, in whom a voluminous epithelial liver hamartoma or adenoma was discovered at surgery is documented. The morphological characteristics of the hamartomatous abnormality are described and evidences are put forward which would suggest that the liver lesion might have been the site of the abnormal renin production which was responsible for the systemic arterial hypertension.

The clinical manifestations and pathological features of an extremely rare cardiac tumour, a mature (benign) teratoma, in a 6 year-old girl are described. These are compared with those reported in the literature.

The case of a calcified left ventricular aneurysm in a 6-year-old asymptomatic boy is presented. The anomaly was discovered on a routine chest X-ray. The etiology of this condition is discussed. A review of the literature reveals that this is a rare occurrence in children and that the origin is often unknown. Selective left coronary arteriography showed no arterial anomalies but early filling of the right coronary veins, apparently through small AV fistulas. This might have caused some peripheral myocardial underperfusion, followed by myocardial infarction and aneurysm formation. Aneurysmectomy was carried out uneventfully.

The case of a patient suffering from liver schistosomiasis diagnosed by liver biopsies is presented. There were also lesions in the liver suggestive of non-suppurative destructive cholangitis and the serum-immunological studies strengthened the diagnosis of primary biliary cirrhosis. The eo-existence of these two affections and their possible interrelationship are discussed. The authors further discussed some clinical, biochemical, immunological and pathological aspects of these two conditions and evoked the eventuality of an auto-immune type of hepatic affection triggered by the presence of the schistosome eggs.

A fatal case of diffuse systemic necrotizing angiitis is described in a 14 year old boy presenting with Schonlein-Henoch purpura and developing renal failure. It is proposed that a long-acting sulfonamide (sulfamidethoxine) caused the disease, thus serving to reinforce previously published warnings against the indiscriminate use of long-acting sulfonamides.

Anatomo-radiological study of a case of osteolysis of the femoral head and neck in a 56-year old male who had recieved, 22 years earlier, an intense radiotherapeutic treatment for a soft tissue tumour of the corresponding hip region. The treatment is considered as being the main etiological factor responsible for the bone lesions. The latter seems to have been favored by an indolent joint due to a thoracic cordotomy for post-irradiation pains; it may have been prepared by a secondary osteodystrophy which preceded the radiotherapy and seems due to two previous surgical excisions.

The study compares the histotopographical lesions of two cases of endomyocardial fibrosis with those of two hearts in sleeping sickness, all of them from Uganda. There was considerable overlapping of the fibrosed zones in endomyocardial fibrosis and the corresponding sites occupied by the chronic inflammatory process in trypanosomiasis. There was a tendency towards fibrosis in the hearts of trypanosomiasis while, in endomyocardial fibrosis, there were mild focal chronic cellular infiltrations. This overlapping and to some extent similarities would suggest that the two conditions may only differ in evolution in time and intensity and that they may be the result of the same inflammatory process. Stipulating a cardiac tropism for African trypanosomes, it is suggested that some African cases of endomyocardial fibrosis may be merely burnt-out lesions of trypanosomal pancarditis. Previous descriptions of carditis in human African and American trypanosomiasis are briefly reviewed, and the present findings are discussed in view of a possible trypanosomal aetiology in some African cardiomyopathies.

We have reviewed 74 cases of patients with permanent pacing, using different types of pacing leads in order to determine what morphological changes are produced by this therapy. Macroscopic examination of the heart was performed and slides at the implantation site reviewed when available. Severe chronic inflammation, scarring and a myocardial response were the prominent findings. From a strictly morphological aspect, the initial trauma at implantation time, the chronic foreign body reaction and myocardial response to chronic trauma, are both much less significant in endovenous than in epimyocardial pacing.

Full-contact, a sport which is becoming more and more popular in our society as a means of self-defence, is not without its hazards. We describe the case of a 32-year-old man who, shortly after an intensive practice match, presented symptoms consistent with cerebral damage which resulted in his death within three days. The main pathological findings were confined to the left internal carotid artery, which presented a dissecting aneurysm as a result of rupture of its intima with thrombosis of its lumen. The thrombus extended as far as the intracranial portion of the artery, resulting in massive cerebral infarction as cause of death. Furthermore, there were histological modifications of both main carotid arteries and their branches which suggested repeated microtraumatic events. The causal relation between the lesions observed and the practice of full-contact are discussed.

A case in which there was Ebstein's anomaly of both the tricuspid and mitral valve is described: the structure of the h eart was otherwise normal.

The tracheal and bronchial lesions observed are described in seven patients, presenting with respiratory distress syndrome and receiving both conventional and high frequency jet ventilation for various periods. The histological findings are related to the duration of the exposure as well as the number of pulsations administered to the tracheobronchial tree. Severe damage to the mucosa leading to acute tracheobronchitis, hyperplasia and hypersecretion of the mucosal glands may explain some of the clinical symptoms observed, especially the upper respiratory obstruction. Care should be taken to limit these changes which may lead to various degrees of stenosis in survivors receiving this mode of therapy.

Four instances of sudden death in childhood and adolescence with autopsy findings are presented. In the four cases unsuspected cystic medionecrosis of the aorta was discovered. In three of the four patients it was associated with a cardiovascular malformation, viz (1) double coarctation of the aorta, (2) coarctation of the aorta associated with Ebstein anomaly, and (3) subvalvular aortic stenosis. In three patients ocular abnormalities were demonstrated as an additional autopsy finding. Only one child could be said to have the Marfan habitus. We suggest that until new biological criteria emerge such observations should remain classified under the eponym of Marfan syndrome.

The case of a calcified left ventricular aneurysm in a 6-year-old asymptomatic boy is presented. The anomaly was discovered on a routine chest X-ray. The etiology of this condition is discussed. A review of the literature reveals that this is a rare occurrence in children and that the origin is often unknown. Selective left coronary arteriography showed no arterial anomalies but early filling of the right coronary veins, apparently through small AV fistulas. This might have caused some peripheral myocardial underperfusion, followed by myocardial infarction and aneurysm formation. Aneurysmectomy was carried out uneventfully.

The authors describe a rare 'pseudo-tumour' in the right heart, discovered after a cerebrovascular accident (paradoxal embolus probably). The mass occupied the anterior leaflet of the tricuspid valve. Its mobility and numerous calcifications facilitated diagnosis, determination of its exact position, and evaluation of haemodynamic consequences. The histopathologic features are reported, and its aetiology is discussed.

Treatment of arterial stenoses by the Simpson atherectomy catheter is carried out by resecting the atheroma. A rotating cutter housed in a cylindrical chamber with a lateral opening is applied against the arterial wall by a low-pressure balloon. Several cuts are made until a large enough diameter of arterial lumen is obtained. We report the results in the first 6 patients treated by this technique using 7F and 9F catheters. All had short (less than 2 cm) and narrow (greater than or equal to 70%) stenoses of iliac, superficial femoral or popliteal arteries. To reduce the stenoses to 30% or less, 4 to 11 cuts were necessary. Histological study of the fragments was performed. The result was inadequate in the only case of stenotic iliac artery and we proceeded with balloon dilatation. The small fragments obtained in this particular case were very hard and calcified. 4 patients with claudication (superficial femoral or popliteal stenosis) were asymptomatic after treatment, whereas one patient suffering from Burger's disease with acral necrotic lesions did not benefit from the procedure in spite of a good morphological result. No complication was encountered. This efficient technique is especially indicated in short segmental stenoses of femoro-popliteal or possibly iliac arteries. It avoids the usual intimal cracks associated with balloon angioplasty and produces a smooth surface whose long term benefit has still to be demonstrated.

A nine-year-old boy from the Central African Republic presented with an enlarged cervical lymph node. Biopsy revealed Kaposi's sarcoma. Lymphocyte subsets and reactivity to all antigens tested were normal, and the patient's serum contained no antibodies against LAV or HTLV3, the viruses presumably responsible for AIDS. We conclude that in this case, the lymphadenopathic form of Kaposi's sarcoma was not related to AIDS.

The case of an extraskeletal myxoid chondrosarcoma in a 65-year-old male, localized in the lower right thigh, is presented. The histological, histochemical, and ultrastructural features of this rare tumour are described and compared with those of the pertinent literature. The value of histochemical methods as an aid in differentiating this tumour from other malignant myxoid tumours of soft tissue is emphasized.

This paper describes the pathological features of 10 cases of human African trypanosomiasis occurring in Uganda over an 8-year period. Three cases were clinically proved, and in all 10 cases chronic meningoencephalitis and myocarditis were present. There was chronic pancarditis in 6 cases, and in 3 of these there was a generalized focal valvulitis and in 2 lesions of the conducting system. Previous descriptions of carditis in human African trypanosomiasis are briefly reviewed, and the present findings are compared with some cardiomyopathies as well as American human and experimental trypanosomiasis.

Three cases of hypertrophic cardiomyopathy are described. The first case, a man aged 17 years, died suddenly, while the second case was diagnosed in a 40-year-old male who presented with auricular fibrillation; this patient died two years later from a cerebral tumour. The third patient, who died at the age of 72 years from cerebral haemorrhage, had had a pacemaker inserted five years previously because of atrioventricular block. The cardiomyopathy was diagnosed only at autopsy. The morphological features of the heart in these cases are documented. The characteristic histological aspect of the myocardial fibres, commonly referred to as disarray, is described and our findings are compared with those in the literature. The relationship between the various morphological presentations and their clinical manifestations is discussed.

The VATER association is the sporadic non-random association of Vertebral anomalies, Anal atresia, Tracheo-oesophageal fistula with Esophageal atresia, Renal defects, and Radial limb dysplasia. Cardiac defects are common, as are other limb malformations. The present report describes a premature infant with most of the known major and minor defects of the association as well as agenesis of the bladder and penis and an askeletal rudimentary tail. The latter have not previously been described.

After reporting the case of a child suffering from pseudomembranous colitis (PMC) rapidly cured by vancomycin, the authors present current knowledge which had led to this new mode of therapy. A patient of 2 years 7 months was treated with amoxycillin per os for 8 days for otitis media. A week after this treatment, diffuse abdominal pains appeared, with 8-12 diarrhoeic stools per day containing blood and mucus and recurrent episodes of rectal prolapsus. PMC was diagnosed after rectoscopy and rectal biopsy. The symptomatology persisted for 4 weeks at which time a treatment was begun with vancomycin 4 x 125 mg/day per os. Improvement of the gastrointestinal symptoms occurred 36 hours later, and a new rectal biopsy demonstrated recovery from PMC. Clinical and experimental studies have demonstrated that PMC is due to the proliferation of a toxin producing anaerobic bacterium, clostridium difficile. This organism is highly sensitive to vancomycin. Treatment with vancomycin eliminates clostridium difficile and its toxin from the stools of patients suffering from PMC and permits clinical and histological recovery.

Percutaneous coronary angioplasty (PCA) is sometimes complicated by bradyarrhythmias necessitating emergency temporary cardiac pacing. This is usually performed by the classical transvenous endocardial approach. This experimental study investigated the possibility of using the metallic guide wire used during PCA as a monopolar electrode. Systematic electrical stimulation at different levels of the coronary arteries in 6 anaesthetised pigs showed threshold levels in the distal segments of 3 to 15 mA, close to the values observed in the right ventricule. Short periods of pacing were well tolerated. On the other hand, prolonged pacing (2 to 5 days) invariably led to the formation of a thrombus in the coronary segment occupied by the metallic guide wire and so should be avoided. This study shows that the metallic guide wire can be used as an emergency pacing electrode during PCA but this should be limited to a short period. This technique could replace the systematic introduction of classical transvenous pacing catheters.

The clinical symptoms, x-ray appearances, and anatomical description of a large gastric hamartoma in an 8-year-old girl are presented. The literature of this condition in childhood is reviewed. The differentiation between true genuine hamartomas of the gastric wall and heterotopic (ectopic) tissues is stressed.

An association was noticed between ischaemic lesions of the gut and perivascular and entero-mesenteric smooth muscle bundles in the intestinal wall which has not been reported in the literature. Having observed these abnormalities in two ischaemic intestinal specimens, a retrospective study was undertaken to determine their frequency. 148 specimens from 137 patients with ischaemic lesions, covering a period of five years, were examined. 33 specimens were devoid of abnormal muscular structures. Among the remaining 115 specimens, 66 (57,4%) presented perivascular bundles at different levels of the thickness of the wall and/or mesentery. Some of these bundles contained nerve plexuses. 25 specimens had entero-mesenteric bundles. Of a control group of 67 surgical specimens devoid of ischaemic lesions, 17 had perivascular bundles and 8 entero-mesenteric structures. It is postulated that the abnormal perivascular structures may contribute to the genesis of the ischaemic gut lesions. The entero-mesenteric bundles may serve simply as anchors in the mesentery.

A 13-year-old girl suffering from rhumatoid arthritis developed a myasthenia gravis. Her circulating anti-acetylcholine receptor antibodies were observed before and after thymectomy. Surgical removal of the thymus was followed by complete clinical remission associated with a progressive decrease of the antibody level; these antibodies did not disappear completely even as late as 2 years after the operation.

A case is described of a newborn infant, small for gestational age, with the features of the "fetal alcohol syndrome", who presented a murmur within the first few days of life, and died at age 7 days in heart failure. Autopsy revealed interruption of the aortic arch, type A, an aortopulmonary window and intact interventricular septum. The association of "fetal alcohol syndrome" and cardiac malformation is discussed, with reference to other cases described in the literature.

A 28-week male fetus with the cardinal signs of the lethal multiple pterygium syndrome (multiple pterygia, congenital joint contractures, lung hypoplasia, facial abnormalities, and hydrops) is reported here. In addition, he had hydranencephaly, an anomaly not yet reported in this group of conditions. This potentially new form of the lethal multiple pterygium was detected prenatally by ultrasound examination. In discussing the case, we consider the probable autosomal recessive inheritance pattern and raise questions about the etiology and heterogeneity of this syndrome.

Eight cases of pseudomalignant myositis ossificans are presented. There was a male predominance and four patients were under 20 years of age. There was no history of trauma, but there was evidence of mild local or general inflammation with positive roentgenograms in two cases. Histologically there was active fibroblastic proliferation, appearing to develop essentially in fibrin deposits with metaplasia leading to the formation of an unusual osteoid. The newly formed mass was surrounded by bone and compressed the surrounding muscle fibers, which were either atrophic or trapped therein. The histological criteria distinguishing this disorder from other forms of myositis ossificans are discussed, and comparisons are drawn with nodular fascitis and proliferative myositis. An association with the latter was observed in one case. This nontumourous growth must be distinguished from osteogenic sarcoma of soft tissue. The differential diagnosis is based on careful histological criteria, which must be supported by clinical and radiological data.