DES daughters in France: experts' points of view on the various genital, uterine and obstetric pathologies, and in utero DES exposure.

BACKGROUND: Compensation of diethylstilbestrol exposure depends on the judicial system. In France, girls having been exposed to diethylstilbestrol are currently being compensated, and each exposure victim is being evaluated. Fifty-nine expert evaluations were studied to determine the causal relation between exposure to diethylstilbestrol and the pathologies attributable to diethylstilbestrol. METHODS: The following were taken into consideration: age at the first signs of the pathology; age of the sufferer at the time of evaluation; the pathologies grouped into five categories: fertility disorders - cancers - mishaps during pregnancy - psychosomatic complaints - pathologies of "3rd generation DES victims"; submission of proof of DES exposure; the degree of causality determined (direct, indirect, ruled out). RESULTS: 61% of the cases related to fertility disorders, 28.8% to cancer pathologies (clear-cell adenocarcinoma), 18.6% to mishaps during pregnancy, 8.5% to disorders resulting from preterm delivery, and 3.4% to psychosomatic disorders. Some cases involved a combination of two types of complaints. Indirect causality was determined in 47.1% of the cases involving primary sterility, in 66.7% involving secondary sterility, and in 5 out of 6 cases of total sterility. There is direct causality between in utero diethylstilbestrol exposure and vaginal or cervical clear cell adenocarcinoma. Causality is indirect in the case of disorders linked to prematurity in third generation victims. CONCLUSION: Causality was determined by the experts on the basis of scientific criteria which attribute the presenting pathologies to diethylstilbestrol exposure. When other risk factors come into play, or when exposure is indirect (third generation), this causality is diminished.

Spontaneous oesophageal perforation.

An autopsy was performed on a young adult, who apparently died during his sleep. Mediastinitis was established and empyema was also found in left pleural cavity. The oesophagus examination showed a tear in left side. The lesion occurred in the distal oesophagus and showed the leak communicating freely with the left pleural space. Oesophageal perforation was the source of empyema, resulted from barotrauma to the lower oesophagus during the effort of vomiting. Death caused by septic shock. Boerhaave syndrome is a serious and rapidly fatal spontaneous oesophagus rupture. Forceful ejection of gastric contents in an unrelaxed oesophagus against a closed glottis is the mechanism described. The tear thus produced is vertical. The case report discusses the historical, statistical, pathophysiological, diagnostic and therapeutic aspects of Boerhaave syndrome. The syndrome is a cause of sudden death, which be known by forensic pathologists.

Macrophage activation syndrome: an autopsy case of sudden death.

In a context of viral gastroenteritis, we report an unusual case of sudden death in an 8-year-old child. The only macroscopic abnormality observed in the autopsy was a diffuse mesenteric adenitis. Organ samples were taken for histopathological examination and a diffuse lymphocytic infiltration was observed. A sinusoidal histiocytic hyperplasia was found in the lymph nodes. Microscopic examination of the lungs and the lymph nodes revealed haemophagocytic lesions (lymphocytes within the cytoplasm of histiocytic macrophages). Immunohistochemical studies demonstrated that the histiocytes were CD68+ PS100- CD1A-. Following this microscopic examination macrophage activation or haemophagocytic syndrome was diagnosed. The syndrome is a distinct clinical entity characterised by fever, pancytopaenia, splenomegaly, and haemophagocytosis in the bone marrow, liver and lymph nodes. It is a clinical entity that is very difficult to diagnose due to the lack of specific clinical signs. It is generally a complication of an infectious process, an aggravation of an auto-immune disease or a complication of a neoplastic process. The physiopathology involves a disregulation of T lymphocytes and particularly T helper lymphocytes. To make this diagnosis the anatomopathological examination must be performed by an experienced practitioner. The presence of a lymphocyte infiltrate of macrophagic histiocytes in myeloid organs and especially positive CD68+ immune markers are the anatomopathological proofs of diagnosis. The autopsy examination must be carefully performed and include systematic sample harvesting for anatomopathological examination. The results of all these examinations taken together allow the diagnosis of haemophagocytic syndrome to be finally made.

Cerebellar-pulmonary embolism, cause of death in the newborn.

A 28-year-old woman delivered twin girls. The first twin was delivered without any difficulty. The head of the second twin failed to descend with pushing. A special kind of obstetrical forceps, Thierry's spatulas, were used to extract the second twin in the occipito-posterior vertex position. She was declared dead after recording Apgar scores of 0 and 0 and after 35 min of resuscitation. An autopsy was performed for medico-legal reasons. Macroscopic examination of the brain showed a small area of leptomeningeal haemorrhage in the left sylvian fossa and the base of the brain. Histopathological studies demonstrated cerebellar tissue emboli in meningeal and pulmonary arteries. Excessive pressure on the suboccipital region during delivery can cause traumatic separation of the occipital chondral junctions, which may lead to separation of the occipital squama from lateral parts of the occipital bones. The inferior part of the occipital squama is displaced forward and upward into the posterior fossa. This produces tearing of the duramater and occipital sinuses leading to leptomeningeal haemorrhage in the posterior cranial fossa, often associated with cerebellar lesions. Major stretching and tearing of the posterior aspect of tentorium cerebelli in contact with the sinuses and the cerebellar cortex may also occur, inducing slight movement of the occipital bones and subsequent emboli. This case study is that of a newborn death due to pulmonary cerebellar tissue embolism occurring during delivery with Thierry's forceps, which are considered less traumatic to the foetal cranium. A review of the literature identified 17 other published cases. In difficult deliveries this pathology should sought carefully. Brain, lung and placenta tissue sections must be studied.

Post-Perruche: what responsibilities for professionals?

Damage compensation relating to handicapped new-borns, the wrongful life law, was put in place after a great controversy in France by the Perruche ruling of November 13th 2000. The French parliaments have voted a special law against wrongful life action. But questions about medical liability are not answered by the new law. For wrongful life, what are now the medical errors likely to directly provoke a handicap or aggravate it or obstruct measures to alleviate it? For a wrongful birth claim it limits parental damages to psychological damages only, due to a lack of mental preparation, whilst denying economic losses, thus preventing complete compensation arising from medical malpractice for all the other types of difficulty suffered by the parents. Why does it give a new definition of medical malpractice as a "characterized fault" and insist upon the qualification 'blatant" in the field of medical liability? For health professionals, it is a question of knowing and being acquainted with the errors that fall under their liability with regard to handicapped persons and a "characterized fault" with regard to parents. It is through a review of past and present case law that we will try to answer these medical questions. All court rulings that awarded monetary damages to the child were reviewed. We include only three decisions after the new law, which do not allow damage compensation in respect of a handicapped new-born. The rulings incorporate decisions taken by the French Supreme Court, of the council of state as well as by courts of appeal. Failure of voluntary termination of pregnancy, missing rubella immune antibody, omission of mandatory tests during pregnancy, typical medical fetal malpractice, were and are professional faults.

[Driving and the elderly: aspects of aging and handicap]. / La conduite automobile chez les sujets âgés: implications liées au vieillissement et au handicap.

Impairment of cognitive performance is associated with an excess risk of accidents. Adaptation of driving behavior in subjects with benign cognitive disorders reduces risk of automobile accidents. Cessation of driving or at least not driving alone limits the excess accident risk for drivers with dementia. Alterations in visual field and acuity increase risk of traffic accidents. Drugs affecting vigilance and neurological, cardiovascular and osteoarticular disorders increase accident risk. Screening for these disorders in the elderly is a necessary public safety measure.

["Medicolegal" bone fluorosis. Apropos of 2 cases of possible criminal poisoning]. / Fluorose osseuse "médico-légale". A propos de deux cas de probable intoxication criminelle.

We report two cases of skeletal fluorosis in two men, 55 and 69 year-old. The physical findings and the roentgenograms were typical and chemical analysis confirmed the diagnosis. Surprisingly, fluoride concentrations in blood and urine were increased at the beginning of the hospitalizations and lower during the hospitalizations. The origin of fluor intake remained unknown, leading to consider a possible criminal poisoning. Then, we discuss the attitude of physician in a case a possible criminal poisoning: denunce the crime to the police or not, as regards french law.

[Bronchopulmonary manifestations of Horton's disease. Apropos of a case]. / Manifestations broncho-pulmonaires de la maladie de Horton. A propos d'une observation.

A 78 years old woman developed a pulmonary recurrence four years after the diagnosis of temporal arteritis. The lung biopsy showed the same giant-cells granulomatous lesions with central vessels as the initial biopsy. The recurrence in giant cells-arteritis has already been published but the pulmonary involvement had never been illustrated before by histological data. The difficulties of diagnosis in case of granulomatous angiitis on lung samples is emphasized.

[Clavicle osteolysis in MacDuffie's syndrome]. / Ostéolyse claviculaire au cours d'un syndrome de MacDuffie.

MacDuffie's syndrome emerges from systemic vasculitis as including recurrent urticaria-like skin lesions, episodic angio-oedemas, articular manifestations and abdominal pains, with a tendency to affect women. Renal involvement may burden the prognosis. Hypocomplementemia is usual by deficiency of the classical pathway. Corticosteroid therapy is habitual. The case reported, fitting to this nosological entity offers two new features: in the first place, the osteolysis of the distal part of the clavicle whereas the joints are not affected by destroying lesions; then the non steroidal anti-inflammatory drugs was effective within a period of one year without complication.

[Horton's disease in elderly patients aged over 75: clinical course, complications of corticotherapy. Comparative study of 164 patients. Towards a reduced initial dose]. / Maladie de Horton du sujet âgé de plus de 75 ans: particularites évolutives, complications de la corticothérapie. Etude comparative sur une série de 164 patients. Vers une dose d'attaque réduite.

PURPOSE: Our study compares clinical and therapeutic courses (corticosteroid response, corticosteroid amount, complications) in people with giant cell arteritis before and over 75 years, during the first year of treatment. METHODS: A series of 164 patients was retrospectively analysed (mean age: 73.3 years) among the two subgroups: before 75 and over 75 years. Patient received (monitoring of reduction in the corticosteroid dosage) a 240 mg intravenous bolus of methylprednisolone followed by 0.5 or 0.7 mg/kg/d of prednisone, or 0.7 mg/kg/d of prednisone without the bolus. RESULTS: Corticosteroid response was identical for the two groups, before and over 75 (patients with corticoresistance: 15% vs 11.4%; NS) and giant cell arteritis-related complications were equivalent (n = 2 vs n = 2; NS). Corticosteroid load was slightly lower in the elderly group (cumulative dose of corticosteroids during the first year of treatment 5.2 g vs 5.8 g; P = 0.03). Patients with rheumatic side effects (collapses of vertebral bodies, mainly) were more frequent in the elderly group (15.5% vs 4.3%; P = 0.01), in spite of a limited mean follow-up period (10.7 months). CONCLUSION: Even if steroid response was identical in the therapeutic course of giant cell arteritis, rheumatic side effects appeared more frequent in the elderly group (over 75 years). In order to obtain a corticosteroid-sparing effect, new studies are necessary to evaluate a reduced initial dosage of corticosteroids.

[Shulman's syndrome. A variant of scleroderma? Two cases (author's transl)]. / Syndrome de Shulman: une forme clinique des sclérodermie? A propos de deux cas.

Observations of two patients illustrating the problem of Shulman syndroma. The first had presented an oedema of the four limbs, without injury of the trunk, but with myalgias, histological fasciitis, eosinophilia and hypergammaglobulinemia. In spite of corticotherapy, evolution toward a generalized morphea. The second has presented the same oedema of the four limbs, with myalgias, but also Raynaud syndroma, histological fasciitis and eosinophilia. Evolution toward acrosclerosis in spite of corticotherapy. For the authors, Shulman's syndroma is apprehended as a particular clinical form of scleroderma.

[The concept of characterized malpractice in wrongful birth]. / La faute médicale caractérisée dans le champ du diagnostic anténatal.

OBJECTIVES: The law of March2002 was introducing a new concept of liability in the field of prenatal diagnosis, survey and investigations. For wrongful birth, medical physicians are responsible for newborn infants with congenital or hereditary disorder only in a case of characterized malpractices. MATERIALS AND METHODS: We performed a study about decisions that awarded or refused monetary damages specifically to the parents in the light of this new law. This research was performed by using official court rulings website, crossed in the search engine two expression-keys that were characterized: fault and antenatal diagnosis. Fifteen rulings were reviewed and studied. The matter of our research is to understand how the judges characterize the medical malpractice and insist upon the qualification "Blatant" in the field of prenatal medical liability? RESULTS: In fact, judicial decisions explicit more what it is not a characterized fault than what it is. Such judicial rejections stated that the medical practices were based on to established medical references and so couldn't be characterized. The taking care, the survey and the biological and radiological investigations of the pregnancy were evaluated in accordance to the knowledge of science although they didn't diagnose fetal malformations. Only four rulings accepted this kind of medical fault, focused the notion of medical neglect accompanied with hurriedness for some judges when physicians performed in particular antenatal echography. In these circumstances, the physician performed quickly and without interest the medical investigations which missed to detect fetal malformations. Two rulings of them categorized the malpractice by the inversion of results, and by the no information about unusual margin of error for cellular culture. The judge argued that negligence of health-care-providers denied to the parents the psychological preparation of hosting disabilities newborn. In this point of view, the judges want to damage parent's injuries.

[Etiologic diagnosis of a digital necrosis: essential thrombocythemia. Review of the literature]. / Diagnostic d'une nécrose digitale: la thrombocytémie essentielle. Revue de la littérature.

The author relates the disease of a man with a digital necrotizing in keeping with an excess of platelets. Clinical, biological and histological study may evolve diagnosis of idiopathic thrombocythemia. The check of concerned literature may specify profile of this disease which agree with platelet side of a myeloproliferative syndrome. Coagulation study is often disappointing but may involve interference of prostaglandins as an intimate mechanism for aggregation and adhesivity of platelets. Clinical course may very easily be supervised with standard medical treatment.

[Tuberculous abscess of the central nervous system (author's transl)]. / Abcès tuberculeux du névraxe. Revue de la littérature sur dix ans.

A case of tuberculous abscess of the cerebellum is reported by the authors. A check-up of concerned literature of ten-past years is drawn up. Diagnosis is seldom raised before surgery in spite of former or evolved tuberculosis. Histology and bacteriology are essential for a supportive therapy. The prognosis is favorable in seventy three per cent of the cases. This pathology must not be forgotten in patients who imigrate from countries of high endemic level of tuberculosis.