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The acquired form of pulmonary alveolar proteinosis was determined in 3 patients, a woman of 31 and 2 men of48 and 38 years, respectively. Their symptoms consisted of progressive dyspnoea, with or without coughing and a tight feeling in the chest. Bronchoscopy with bronchoalveolar lavage yielded milky white, frothy material, and high resolution CT revealed parenchymal densification. All 3 patients were successfully treated with recombinant granulocyte-macrophage colony-stimulating factor (GM-CSF; sargramostim); in addition, the first and last patient underwent total pulmonary lavage. During the pregnancy of the woman, the GM-CSF treatment was suspended; this was resumed after parturition, which took place via caesarean section. Pulmonary alveolar proteinosis is a rare disease characterised by accumulation of surfactant in the alveoli. Until recently, the treatment consisted only of total lung lavage under general anaesthesia. It has recently been discovered that IgG autoantibodies play an important role in the development ofthe disease, namely in the accumulation of surfactant in the alveoli. IgG autoantibodies appear to neutralise the biological activity of natural GM-CSF, which leads to accumulation of used surfactant in the alveoli and a decrease of the pulmonary diffusion capacity. These cases and other publications from the past few years underline the important role of GM-CSF, in addition to a total lung lavage, in the treatment of pulmonary alveolar proteinosis.

In certain centres, following the practice in liver and kidney transplantation, lung transplantation programmes have been set up in which two healthy living donors undergo removal of the right lower lobe and the left lower lobe, respectively, which are then implanted bilaterally in the recipient in place of the usual whole right and left post-mortem lungs: 'living donor lobar lung transplantation'. The relatively high morbidity in the donors and the complicated procedure constitute obstacles to worldwide application. Besides medical problems, the procedure also raises ethical questions. Whether living donor lobar lung transplantation should also be applied in the Netherlands can only be answered following a broad public, ethical and medical discussion. In the meantime, the lung transplant centres may analyse the prerequisites and make the necessary preparations.

Three patients, two Moroccan men aged 27 and 25 and a Turkish man aged 25, presented with haemoptysis caused by pulmonary aneurysm. The aneurysms had formed as a complication of Behçet's disease. Two of them were treated with high doses of corticosteroids. One man recovered and another died as a consequence of massive haemoptysis. The third man underwent emergency thoracotomy and pneumectomy due to massive haemoptysis. Postoperatively he was treated with cyclosporine resulting in full recovery. Behçet's disease is a multisystem vasculitis characterised by orogenital ulcerations and uveitis. In a minority of cases pulmonary aneurysms develop, often causing massive haemoptysis. Aneurysms are often accompanied by venous thrombosis. Treatment consists of immunosuppressive therapy. Nevertheless a considerable number of patients die following massive haemoptysis.

Objective: Investigating the frequency and nature of sub-standard care factors in non-complicated pregnancies in primary obstetric care., Design: Retrospective investigation of medical files., Method: Data concerning obstetric care in 3 midwifery practices in the Delft area (Pijnacker, Nootdorp, Den Hoorn and Schipluiden), the Netherlands, from 1989-1999 were gathered from the primary National Obstetrics Register. Of the 8362 pregnancy records, 72 were selected at random. Using a checklist containing criteria based on the Obstetrics Indication List, the Cochrane Pregnancy and Childbirth Database, and from an expert panel, the records were analysed for the frequency of occurrence of sub-standard factors in perinatal care., Results: Of the 72 pregnancy records, only 1 was found to contain no sub-standard factors. On average 1.7 sub-standard factors were seen with a maximum of 7. Most frequently found were: too few check-ups during the first trimester (39%), no testing for proteinuria at the first visit (26%) and no administration of prophylactic vitamin K1 (43%). Less frequently found sub-standard care factors were: no ultrasound despite indication (11%), no referral to secondary care in the event of foetal distress (6%), no consultation with secondary care in the event of hypertension (3%), or in the case of membranes ruptured for more than 24 hours (1%). Frequently the circumstances surrounding the departure from the main checklist criteria were found to justify the action., Conclusion: Sub-standard care factors were demonstrated in many of the pregnancies investigated. A limited number of these factors gave reason to question whether guidelines for good quality perinatal care are being properly applied.

In the case of a 52-year-old man, who was in a vegetative state after resuscitation following an unwitnessed cardiac arrest, the nursing-home physician assumed responsibility for the total treatment in a transfer unit of the hospital. His systematic, multidisciplinary approach enabled direction of the complex situation in which many medical and paramedical personnel were involved. When an airway infection occurred as a complication shortly after responsibility for the patient had been assumed, the nursing-home physician could take a well-considered decision to withdraw medical treatment on the basis of the treatment plan which had been formulated in a short time and contact with the family. In clinical practice it is difficult to determine the most appropriate moment to withdraw all medical therapy, including artificial nutrition and hydration, in order to prevent a hopeless vegetative state. This case illustrates how hospital physicians and nursing-home physicians may cooperate during the 'waiting phase' of the clinical course of a vegetative state. A hopeless vegetative state can be prevented by using these transmural possibilities for cooperation, including an early input of knowledge and experience about the long-term course of a vegetative state.

Objective: To evaluate a perinatal audit procedure by communicating the results to the caregivers (midwives and obstetricians) involved, in order to determine whether the audit led to specific suggestions for improving practice and whether evaluation of the panel assessments by caregivers leads to a different evaluation of the audit process., Design: Descriptive evaluation study., Method: Because of privacy regulations, the results of a recently published audit concerning perinatal mortality were reported at an aggregated level. At their own request, two participating hospitals received panel assessment reports of their own cases. The audit procedure, the 77 panel assessments and the care provided were then evaluated during closed meetings with the caregivers affiliated to the respective hospitals., Results: In two audited cases of mortality the caregivers judged the panel's assessments as being too light and as too severe in one other case (Cohen's kappa: 0.98). Detailed case description was considered essential to the audit procedure. While aggregated reporting of audit results provides a general understanding of substandard factors in the care provided, feedback of results on an individual practice level led to specific suggestions for improvement (in relation to medical aspects, patient-caregiver relationship and collaboration between caregivers). Lack of anonymity appeared not to be an issue for the caregivers., Conclusion: The feedback of perinatal audit results to the caregivers involved as well as discussion of these results led to specific starting points in the areas of collaboration, documentation and policymaking at both individual and institutional level.

The term 'vegetative state' is most appropriate for the state which develops when patients open their eyes after a comatose phase, without regaining consciousness. The definition and the diagnostic criteria from the Multi Society Task Force on Persistent Vegetative State are usable for the clinical practice in the Netherlands. The vegetative state must be differentiated from coma, locked-in syndrome and minimally conscious state. A systematic, multidisciplinary approach under the direction of a physician is key to diagnosing vegetative state. To this end, a clinical assessment is recommended with reassessment and verification of the diagnosis at appropriate moments. Careful observation remains the fundamental to the diagnosis.

Objective: To assess the level of suboptimal care prior to cases of perinatal death and the extent to which perinatal mortality can be reduced by further improvements in care., Design: Retrospective panel audit investigation., Method: Cases of perinatal death occurring in 1996 and 1997 among women living in the region Zuid-Holland-Noord, the Netherlands, were identified by approaching midwives, obstetricians/gynaecologists and paediatricians/neonatologists. The medical records of the cases were studied by an expert panel using a checklist of evidence-based criteria for standard care in order to determine circumstances and actions that did not comply with professional protocols, or that indicated either low compliance of the mother or an inadequate healthcare infrastructure (so-called sub-standard factors). The panel also assessed whether the perinatal death could have been prevented., Results: A total of 342 perinatal deaths were found. For 332 cases sufficient information was available for a panel assessment and for 318 cases the panel reached a consensus on the assessment. One or more sub-standard care factors were identified in more than half of the cases. In 19% of the cases the panel agreed that the sub-standard factor had 'possibly' contributed to the death, and in 6% they agreed that the sub-standard factor had 'probably' contributed to the death. In the last group the main problems involved were antenatal care (particularly a failure to detect or inadequate management of intrauterine growth retardation) and intrapartum care (too much of a 'wait and see' approach)., Conclusions: This regional audit revealed that further quality improvement of obstetric care is possible if clinical practice guidelines for effective and safe care are better implemented. It is expected that these improvements could reduce the perinatal mortality rate by between 6% and 25%.

A 57-year-old male patient, recently known with an anal carcinoma with inguinal lymph node involvement, was admitted because of anorexia, nausea, vomiting and constipation. On physical examination the patient was dehydrated, and a systolic murmur, grade III/VI, punctum maximum apex cordis, was heard. Serum calcium was raised (4.50 mmol/l), as was the serum creatinine (328 mumol/l). Both values had been normal 14 days before admission. Serum parathormone was suppressed. A bone scan did not reveal evident lesions in the skeleton. FDG-PET scan showed uptake of the tracer into the bone marrow. A bone biopsy showed metastasis of a squamous cell carcinoma. Shortly after that the patient died. Hypercalcaemia is associated with cancer. Colorectal/anal carcinomas have a low incidence of hypercalcaemia. The prognosis of patients with cancer associated with hypercalcaemia is poor.

A 36-year-old man was admitted because of haemoptysis and weight loss. Despite elaborate investigations, including multiple biopsies of affected organs (pleura, lung, kidney and liver) no diagnosis was established. The patient refused further diagnostic procedures and left hospital for winti treatment in Surinam. He did not take the prescribed pheneticillin and returned after seven weeks in a very poor condition. The second admission was complicated by septic shock. Despite intensive treatment he died. On autopsy actinomycosis abscesses were found in lung, liver and kidney. Even when suspected, an infection with Actinomyces is difficult to diagnose. Without adequate treatment this infection can lead to life-threatening complications.

Objective: To estimate the numbers of hip fractures among Dutch women caused by senescence in contrast to decreasing postmenopausal bone loss., Design: A Markov chain model in a multi-state life table., Setting: Department of Public Health, Erasmus University of Rotterdam, the Netherlands., Method: Cross-sectional data of prevalences of femoral neck bone mineral density, measured in an epidemiological survey (1993), were related to numbers of hospital admissions for hip fractures among women aged 55 and older, using a (published) relative risk for hip fracture of 2.6 (95% confidence limits: 1.9-3.6) per decrease by I SD of bone mineral density. We varied the prevalences of bone mineral density in the multi state life table and looked at changes in the expected number of fractures., Results: If bone loss were to cease after the age of 55, this would decrease the number of fractures by 39.4% (30.4%-45.2% in sensitivity analysis). If bone loss were delayed by 7.5 years, the number of hip fractures would decrease by 14.8% (10.0%-19.9% after 5 to 10 years' delay). If compliance decreases by age (3% per year), then the number of hip fractures would decrease by 5.6% (3.4%-11.0% after 1%-5% decrease of compliance per year)., Conclusion: Bone loss is only one of the many age related causes of hip fractures. Population based interventions targeting only bone mineral density, while ignoring comorbidity, have only a limited potential for preventing hip fractures.

Objective: To analyse patient characteristics and the results of rehabilitation among patients admitted for rehabilitation to a nursing-home., Design: Retrospective study., Setting: St. Jozefsheil in Bakel, part of the Stichting Verpleeghuizen Gewest Helmond Nursing Homes Foundation, District Helmond, SVGH)., Method: On all 60 patients admitted to the SVGH homes in 1987, information was obtained from medical, paramedical and nursing records. The Barthel index was determined on admission and at discharge and the differences were tested statistically using a t-test., Results: 37 female and 23 male patients were admitted, with a median age of 75.5 years. 62% had had a cerebrovascular accident and 90% were referred from the hospital, mainly by the neurologist. The median hospitalization time was 9 weeks and the waiting list for a nursing home 2.9 weeks. In 1990, 47% of the patients were discharged, after a median rehabilitation time of 15 weeks; 23% had died and 30% remained in the nursing home. Among the discharged patients the increase in mean Barthel index from admission to discharge was 17.9 points. The difference in mean Barthel index at admission compared with non-discharged patients was 35.4 points and that compared with the decreased patients 37.7 points. For the 23 patients discharged within one year compared with the 5 patients discharged after more then one year the difference was 13.6 points. The mean Barthel index at admission of the 14 discharged patients living alone was 21 points more then that of the 10 discharged patients living with a partner. At discharge this difference was 9.9 points., Conclusion: The Barthel index appears useful for evaluation and comparison of results of (geriatric) rehabilitation.

Objective: To determine the prevalence and the burden of diseases of the nervous system in the Netherlands., Design: Descriptive., Setting: Institute of Public Health Care, Rotterdam, the Netherlands., Method: Data from medical registrations and statistics concerning diseases of the nervous system from different tiers of health care (general practitioners, hospital, nursing homes) and effects on patient and community (mortality, sickness absenteeism, occupational disablement and costs of illness) were analysed., Results: In general practice, 3-5% of total consultation consisted of neurological diseases (450,000-750,000 cases per year), of which migraine and epilepsy instituted a relatively large group. Of all the hospital admissions, 7% were because of a neurological disease. Neurological disease accounted for 70% of all nursing home admissions. Cerebrovascular accident and hernia nuclei pulposi instituted the largest groups in all sectors. Diseases of the nervous system accounted for 14% of total mortality, 6% of total sickness absenteeism and 14% of total health care costs (f 3.8 billion)., Conclusion: Estimating the burden of diseases is difficult because of imperfect medical registration, variations in structure of patient referral and influence of secondary diagnoses. Taking into account the current aging of the population, the prevalence of some neurological diseases, such as CVA, Parkinson's disease and dementia will rise. Priority setting for care and treatment is hampered by differences in burden of illness on the individual and on the community level.

Two patients presented with severe side effects of theophylline. Due to the theophylline intoxication patient A developed ischaemic hepatitis, which made haemodialysis treatment necessary to accelerate the elimination of the drug from the body. The intoxication caused immobilisation and total dependence with respect to the daily activities of patient B. In neither case was the association between mood disorders--like depression, agitation and anxiety--and an overdose of theophylline recognised. Administration of theophylline should be monitored with regular measurements of the serum theophylline concentration, because of its narrow therapeutic margin and the serious toxic side effects.

Diaphragmatic rupture is commonly of traumatic origin and can complicate thoracoabdominal injuries. Some cases are diagnosed after a delay of years. We present three patients with a rupture of the right hemi-diaphragm with herniation of liver fragments, and discuss the diagnostic and therapeutic challenge.

In order to assess the effect of rehabilitation of lower-extremity amputees in a nursing home, a retrospective study was conducted. The results were compared with existing Dutch data. Moreover, the influence of several selected factors on the results of rehabilitation was assessed. The study covered a period of five years and concerned 61 patients admitted with a lower-extremity amputation. Their median age was 73 (range 38-91) years. After rehabilitation two-thirds of the patients were able to walk with an ambulatory aid and five without; a quarter came to depend on a wheelchair. Return to home or sheltered housing was possible for almost three-quarters of the patients. Only one out of eight selected factors was in this study of influence on the results of rehabilitation i.e. motivation.

Bilateral lung transplantation in patients with cystic fibrosis is reported. Five were operated upon: one died six days after operation; during the operation extracorporeal circulation had been used in view of severe haemodynamic instability. One other patient died after 96 days due to invasive ulcerative aspergillosis. Three patients are alive and well. Selection of patients is shown to be the most important determinant of successful outcome.

The localized fibrous tumour of the pleura, also named localized fibrous mesothelioma or fibroma of the pleura, is a very rare tumour with a benign behaviour. Its origin, clinical behaviour and prognosis are very different from malign mesothelioma. In a 33-year period 22 patients with this diagnosis were surgically treated. The prognosis was excellent. In this article the clinical and radiological signs, as well as the diagnosis and therapy are discussed.

In September 1989 the first unilateral lung transplantation in The Netherlands was performed on a woman with chronic total respiratory failure due to a concentric constrictive form of obliterating bronchiolitis. The transplantation was right-sided. Total duration of operation was 7 hours; the ischaemia time of the donor lung amounted to 108 minutes. Postoperatively artificial respiration was applied for a long time. No severe infections or rejection signs occurred. The transplanted lung fairly rapidly took over perfusion and ventilation.

In this review the histological and clinical features of 12 endobronchial hamartomas seen between 1960 and 1987 are presented. All patients were men; the mean age was 54.0 years. Nine patients presented with pulmonary symptoms, most frequently cough. All lobes were affected, with a slight preponderance in the left upper lobe. Surgical treatment was given to 10 patients. In the other 2 cases bronchoscopic resection was preferable. There were no complications due to therapy. No recurrences were noted. This lesion should be regarded as a true neoplasm, in view of the clinical and histopathological characteristics.