Pulmonary Sarcoidosis.

BACKGROUND: A 34-year-old male teacher was referred to the hospital with a persisting dry cough and dyspnea on exercise since eight weeks. He had no fever, neither complaints of ear, nose or throat. There were no complaints during the night. He had been a smoker until four months before presentation (12 pack years). At work a student was diagnosed with pulmonary tuberculosis, but the Mantoux and Quantiferon tests were negative. Physical examination was normal, without fever, lymphadenopathy or auscultation abnormalities. Laboratory investigation revealed a C-reactive protein of 2 mg/L. Pulmonary function testing showed a slight restriction. Immunological bronchial alveolar lavage (BAL) was rich of cells, especially T-lymphocytes of the CD4 type. CD4+/CD8+ ratio of the BAL was raised to 4.2, compared to a ratio of 2.4 in blood. There were no eosinophils found in the BAL. Conventional chest radiographs were performed, and showed multiple areas of consolidation in the bilateral lung fields, predominantly on the right side.

Jeune syndrome: description of 13 cases and a proposal for follow-up protocol.

Jeune syndrome (asphyxiating thoracic dystrophy, ATD) is a rare autosomal recessive skeletal dysplasia characterized by a small, narrow chest and variable limb shortness with a considerable neonatal mortality as a result of respiratory distress. Renal, hepatic, pancreatic and ocular complications may occur later in life. We describe 13 cases with ages ranging from 9 months to 22 years. Most patients experienced respiratory problems in the first years of their life, three died, one experienced renal complications, and one had hepatic problems. With age, the thoracic malformation tends to become less pronounced and the respiratory problems decrease. The prognosis of ATD seems better than described in literature and in our opinion this justifies long term intensive treatment in the first years. We also propose a follow-up protocol for patients with ATD.

Oesophageal dilatation on high-resolution computed tomography scan of the lungs as a sign of scleroderma.

BACKGROUND: Systemic sclerosis (SSc) is a generalised autoimmune disease that causes morbidity and reduced life expectancy. Recently, evidence has been accumulating that immunosuppressive treatment in an early stage of the disease could improve survival, enhancing the need for early diagnosis and regular evaluation of organ involvement. Among others, a high-resolution computer tomography (HRCT) scan of the chest is performed for the assessment of pulmonary involvement in SSc. The objective of this study is to evaluate the predictive value of oesophageal dilatation on the HRCT scan for the diagnosis of SSc. METHODS: In total, 105 consecutive patients with scleroderma and 107 consecutive controls were included in this study. The first available scan for each patient and control was evaluated in random order and blinded for the diagnosis, by two independent radiologists, for oesophageal dilatation and interstitial lung disease. RESULTS: The positive predictive value of oesophageal dilatation for the diagnosis of SSc was 83%. No significant correlation of oesophageal dilatation and interstitial lung disease was found in the patients with scleroderma or controls. CONCLUSION: Oesophageal dilatation as visible on an HRCT scan of the chest may alert doctors to look for other signs or symptoms of SSc in these patients, enabling early diagnosis and specific treatment.

Prophylactic G-CSF and antibiotics enable a significant dose-escalation of triplet-chemotherapy in non-small cell lung cancer.

In advanced non-small cell lung cancer (NSCLC) the clinical benefit of a platinum-based doublet is only modest, therefore, attenuated dosed three-drug combinations are investigated. We hypothesized that with adequate support a full dosed chemotherapy triplet is feasible. The study was designed as a dose finding study of paclitaxel in chemotherapy-naive patients. Paclitaxel was given as a 3-h infusion on day 1, followed by fixed doses of teniposide (or etoposide) 100mg/m(2) days 1, 3, 5 and cisplatin 80 mg/m(2) day 1 every 3 weeks. As myelotoxicity was expected to be the dose-limiting toxicity, prophylactic G-CSF and antibiotic support was evaluated. Indeed, paclitaxel 120 mg/m(2) resulted in dose-limiting neutropenia, despite G-CSF support. Teniposide/etoposide day 1, 3, 5 was less myelotoxic compared to day 1, 2, 3. G-CSF support allowed paclitaxel dose-escalation to 250 mg/m(2). The addition of prophylactic antibiotics enabled dose-escalation to 275 mg/m(2) without reaching MTD. In conclusion, G-CSF and antibiotics prophylaxis enables the delivery of a full dosed chemotherapy triplet in previously untreated NSCLC patients.

Determining mucosal barrier injury to the oesophagus using CT scan.

INTRODUCTION: Oral mucositis is recognised as one of the most debilitating complications of high-dose cytostatic chemotherapy used to prepare for haematopoietic stem cell transplantation (HSCT), but very little is known about oesophageal mucositis, as endoscopy is not routinely performed. MATERIALS AND METHODS: We incorporate the computed tomography (CT) scan in the diagnostic workup of fever during neutropenia to detect evidence of pulmonary complications. This allowed us to evaluate whether mucosal barrier injury to the oesophagus can be determined. We selected 46 patients without oesophageal cancer or immune suppression (controls), who had a normal oesophagus, and measured the mucosal thickness at the upper part (UP), middle part (MP) and lower part (LP) of the oesophagus. Next, we selected 30 patients having a CT scan done for diagnostic purposes within 14 days after HSCT and measured mucosal thickness at the same levels. We also scored oral mucositis and gut toxicity. RESULTS: The mucosal thickness of the UP, MP and LP, respectively, for the controls (mean +/- SD) was 4.1 mm (+/-1.1), 4.2 mm (+/-1.2) and 4.8 mm (+/-1.3), and the corresponding values for the subjects were 5.9 mm (+/-2.2), 5.9 mm (+/-2.0) and 7.7 mm (+/-3.0). Analysis of variance showed statistically significant differences between subjects and controls at all oesophageal levels. All patients suffered from severe oral mucositis at the time. CONCLUSION: Hence, mucosal barrier injury to the oesophagus can be objectively measured using CT scan.

Critical aneurysmal dilatation of the thoracic aorta in young adolescents with variant hyperimmunoglobulin E syndrome.

The autosomal-dominant (AD) form of the hyperimmunoglobulin E syndrome (HIES) has been described as a multisystem disorder including immune, skeletal and dental abnormalities. Recently, the evaluation of patients from families in which HIES was inherited in a manner more consistent with autosomal-recessive (AR) inheritance, showed that AR-HIES is a clinically distinct disease entity. In addition to classical immunologic findings of AD-HIES, the AR form presents with severe recurrent fungal and viral infections with herpes zoster, herpes simplex and characteristic mollusca contagiosa. Furthermore, cerebral vascular sequelae, including vasculitis, infarction and haemorrhage were noted. In this report, we describe the clinical picture of two patients who showed remarkable resemblance to the description of AR-HIES, but also developed fatal aneurysmal dilatation of the thoracic aorta in adolescence. This finding may further consummate the clinical picture of AR-HIES and emphasize the possibility to develop early aortitis, most likely preceding the critical aneurysm formation at older age. This process should be anticipated during childhood in cases with AR-HIES.

[Therapeutic dilemmas in patients with a centrally-located pulmonary embolism confirmed by spiral CT-scan but with no cardiogenic shock]. / Dilemma's rond de behandeling van patiënten met een centrale longembolie op de spiraal-cT-scan zonder tekenen van cardiogene shock.

3 patients, 2 women aged 64 and 44 and 1 man aged 67, had severe dyspnoea and a large centrally-located pulmonary embolism (PE) without any accompanying arterial hypotension. They were all given conventional anticoagulation therapy, although thrombolytic therapy was also considered. The women recovered but the man eventually died of a second massive embolism. PE is a disease with a potentially high mortality. Patients with cardiogenic shock due to PE are candidates for thrombolytic therapy. A subset of patients with right-ventricular dysfunction (submassive PE) also have a poorer prognosis despite the absence ofarterial hypotension or shock. Spiral CT-scan is becoming the first-line imaging test of preference in patients with suspected PE. Spiral CT enables the accurate visualization ofthrombi. The value of risk management using cardial biomarkers, spiral CT and echocardiography is not yet clear. There is no evidence that thrombolytic therapy is beneficial in patients with acute PE and right-ventricular dysfunction without overt shock.

[Improved diagnostics in invasive aspergillosis and systematic monitoring in patients at high risk of infection]. / Verbeterde diagnostiek van invasieve aspergillose en systematische controle bij patiënten met een verhoogd risico.

Invasive aspergillosis remains an important cause of morbidity and mortality in patients with prolonged and severe immune suppression such as following haematopoietic stem-cell transplantation. Consensus definitions, which allow categorisation of patients based on diagnostic criteria, are an important improvement in uniform registration of invasive mycoses in clinical trials. Prospective monitoring of high-risk patients for the circulating aspergillus cell-wall component galactomannan, results in earlier diagnosis in two-thirds of patients when compared with conventional diagnostic methods. High-resolution CT (HRCT) enables the lesions characteristic of invasive mycoses to be detected earlier and better than by chest radiograph. In addition, invasive mycoses cause characteristic lesions on the HRCT scan including the halo-sign and the air-crescent sign. The pre-emptive management strategy which combines monitoring of patients for surrogate markers with a HRCT scan appears to be a promising approach to the early identification and treatment of patients with invasive aspergillosis.

[Pneumatosis intestinalis in 9 children with an oncologic disease]. / Pneumatosis intestinalis bij 9 kinderen met een oncologische aandoening.

OBJECTIVE: To acquire knowledge regarding the rare condition pneumatosis intestinalis (PI) in children treated for malignant disease. DESIGN: Retrospective. METHOD: In 1998-1999 PI was diagnosed in 9 of the 140 children with malignant disease in the department of Paediatric Oncology of the UMC St Radboud, Nijmegen, the Netherlands. By examination of the records of these 9 children, data were collected on the symptomatology, diagnostics, treatment and prognosis of PI. RESULTS: The 9 children included 7 boys and 2 girls, varying in age from 2 to 12 years. In 7 patients the underlying disease was acute lymphocytic leukaemia and in 2 it was a stage IV neuroblastoma. The presenting symptoms were nonspecific and included: a distended abdomen, abdominal pain, diarrhoea and constipation. In all children, PI was located in the colon. Supplemental blood and microbiological analysis did not reveal any typical abnormalities. 8 children were treated with lactitol because of constipation. A laparotomy was performed in the first patient, while the other 8 were treated with gastric suctioning, parenteral nutrition and antibiotics. All 9 children recovered within a few weeks. CONCLUSION: With supportive care, PI in children with malignant disease is mostly a self-limiting condition. A pneumoperitoneum in PI is no indication for surgery, except in the presence of an acute abdomen. Chemotherapy can be continued.

Current imaging concepts in pediatric osteomyelitis.

The diagnosis of osteomyelitis remains a difficult diagnostic dilemma. In this article, which is particularly aimed at those whose practice does not include a large paediatric population, we review the pathophysiology of paediatric osteomyelitis and contrast it with the available imaging modalities. We examine the role of the radiologist as well as the usefulness of each modality. Secondly, we review the different clinical scenarios such as acute, subacute and chronic, as well as specific forms of osteomyelitis; the latter includes subacute chronic ostemyelitis, toxic synovitis, spondylodiscitis as well as the congenital inflammatory disorders such as rubella and syphylis. The most useful imaging findings to look for and their significance are assessed and we evaluate their usefulness in each case. Close cooperation between clinicians and imagers remains the key to early and adequate diagnosis of paediatric osteomyelitis.

[Haemangiomas and congenital vascular malformations: their classification and diagnosis]. / Hemangiomen en congenitale vasculaire malformaties; classificatie en diagnostiek.

Mulliken and Glowacki's classification of peripheral blood- and lymph-vessel abnormalities is based on their clinical course and cellular characteristics, and is therefore clear to and readily usable by the practising physician. In order to make the diagnostic process more accessible, the Haemangiomas and Congenital Vascular Malformations Nijmegen working group has developed a system of diagnostic guidelines on the basis of this classification. The anamnesis should be directed at the following six distinguishing characteristics: presence of the anomaly at birth, growth, involution, change in volume, pain and outflow. The physical examination is directed at the following five characteristics: the possibility of emptying or pushing aside the anomaly, changes in volume during engorgement, murmur/'thrill'/pulsation, phleboliths, and hyper- or hypotrophy. If a diagnosis still cannot be made, then additional investigations may be carried out. Duplex scanning is usually sufficient for this purpose, after which the nature and extent of the malformation can be determined with MRI. On the basis of the results, the persons involved can be informed as to the prognosis of the malformation and a plan of treatment can be proposed.

Testicular simple cyst and teratoma: asynchronous bilateral occurrence within the first year of life.

Benign and malignant testicular tumors are rare in infancy. Moreover, only a few cases of bilateral testicular tumors in children have been reported to date. To our knowledge, we report the first case of an asynchronous bilateral simple testicular cyst and testicular teratoma in an infant. This case demonstrates that although both lesions are benign in the prepubertal child, treatment decisions should be made carefully.

The use of X-ray for lymph node determination in the axillary dissection specimen.

A new and simple method by X-ray is described for lymph node determination in the axillary specimen of breast cancer patients. X-rays were performed of the axillary specimens of 49 women with breast cancer. The number of lymph nodes visible on the X-rays were assessed by two radiologists (A and B). The number of nodes identified in the axillary specimens was reported by the pathologist independently. The method described shows a clear correlation between the mean numbers of nodes counted on the X-rays of the specimens (radiologist A 18.3, B 16.1 nodes) and the mean numbers of nodes recovered by the pathologist (18.4). No intra-observer variation was observed and only a small inter-observer variation (2.2 nodes). This method of X-ray determination of lymph nodes can be used in auditing the surgeon's accuracy in performing complete axillary dissection as well as in auditing the number of lymph nodes found by the pathologist.