Primary pulmonary hemangiopericytoma: a case report

Primary pulmonary hemangiopericytoma is a very rare tumor. A case of the disease is reported, together with a review of the literature. The patient was a 78-year-old male, who was admitted to hospital with an abnormal shadow on his chest x-ray. A primary tumor, located in the left S4, was resected, and a final diagnosis of hemangiopericytoma of pulmonary origin was made. The disease recurred in the left lower lobe 14 months postoperatively. The patient received radiotherapy and is alive to date, 23 months after the operation.     

Systemic lupus erythematosus and pulmonary hypertension during pregnancy: report of a case fatality

The case of a 25-year-old pregnant woman with systemic lupus erythematosus and severe pulmonary hypertension is presented. The pregnancy was complicated by worsening right heart failure and pre-eclampsia, requiring a caesarian section at 29 weeks' gestation. On the fourth day postpartum, the patient's respiratory status worsened and she was transferred to the coronary care unit where she soon died from combined right heart failure and respiratory arrest. The presumed pathogenesis and etiology of lupus-related pulmonary hypertension are discussed, in addition to noninvasive tests and proposed management. Given that the mortality rate is very high during pregnancy and therapy is of limited value, women with lupus-associated pulmonary hypertension should avoid conceiving. Those who choose to become pregnant must be carefully managed by a multidisciplinary team.     

Primary pulmonary botryomycosis: case report and review

Botryomycosis is an uncommon bacterial disease characterized by the microscopic formation of eosinophilic granules that resemble those of infection by Actinomyces species. The diagnosis of botryomycosis can be made when microscopic inspection and culture of the granules reveal gram-positive cocci or gram-negative bacilli. Botryomycosis is caused by common bacterial pathogens including Staphylococcus aureus, Escherichia coli, and Pseudomonas aeruginosa, yet the host and microbial factors that contribute to the pathobiology remain unknown. Pulmonary botryomycosis can resemble actinomycosis, tuberculosis, or invasive carcinoma by causing a mass lesion with constitutional symptoms. Radiographically, it invades bone and disrupts tissue planes. Successful treatment often requires a combination of both surgical debridement and long-term antimicrobial therapy. We report a case of primary pulmonary botryomycosis and review the literature on this unusual infectious process.     

Primary congenital pulmonary lymphangiectasia--a case report

We report on a 22 year-old women with recurrent pleural effusions and shadowing of the right lower lobe, which was refractory to antibiotic treatment. Histologic examination (open lung biopsy) was interpretated as indicating an early stage of lymphangioleiomyomatosis. Because of progression of the pulmonary changes and development of a pericardial effusion in spite of antiestrogen treatment to achieve pharmacological castration, and in view of the atypical findings in high resolution computed tomography (lack of cysts) a second open lung biopsy was performed, confirming the diagnosis of pulmonary lymphangiectasia. The patient was given oral corticosteroids postoperatively and showed almost complete resolution of the pleural and pericardial effusions.     

Primary pulmonary lymphoma: report of a case diagnosed by transbronchial biopsy

Primary pulmonary lymphomas are uncommon tumours arising from mucosa associated lymphoid tissue (MALT). The authors report a case diagnosed by transbronchial biopsy and treated by surgery only. They discuss the distinct clinicalpathological features and debate the different ways of treating this condition.     

Primary pulmonary hypertension

Justification of early treatment of nocturnal enuresis is founded in the negative psychological impact on the child. In fact nocturnal enuresis delays early autonomy and socialisation by decreasing in self-esteem and self-confidence. Nocturnal enuresis classification is the preliminary step to correct therapy. Enuresis must be classified as primary (never acquired nocturnal control) or secondary (at least 6 months of dry nights). A child is also classified as having monosymptomatic enuresis if she/he experienced only night wetting and symptomatic enuresis if she/he experienced night wetting associated with diurnal voiding symptoms (urinated > or = 7 times a day, urgency, damp pants, squatting, holding the perineum, sitting on one heel). Monosymptomatic patients must be treated with desmopressin nasal spray at the daily dose of 20 micrograms at bed time. If the reduction of at least the 50% of the basal number of the wet nights is not achieved, the dosage must be increased until 40 micrograms. For patients affected by rhinitis or asthma, desmopressin is now available in tablets. In symptomatic patients desmopressin therapy must be associated to oxybutinin (5 mg x 2). Therapy interruption must be gradual with desmopressin reduction of 10 micrograms every 30 days. In symptomatic patients oxybutinin must be introduced only at bed time. The efficacy of the drugs depends on the therapy length. The highest percentage of success is obtained if the treatment is protracted for at least six months. Antidepressants are also used for nocturnal enuresis especially imipramine. The dosage varies between 0.5-1.5 mg/ kg/daily. As plasmatic levels are achieved only in 30% of treated patients, a 3-5 fold increase in suggested. Nevertheless these levels result in near toxic threshold concentration. Sporadic treatment purposes include amytriptiline, diclofenac sodicum, viloxsazine and methilphenidate if giggle incontinence is present. Non responders may be treated with alarm. If after 16 weeks of treatment no success is obtained alarm use must be interrupted.     

Primary pulmonary hypertension

Primary pulmonary hypertension (PPH) is a progressive disease characterised by raised pulmonary vascular resistance, which results in diminished right-heart function due to increased right ventricular afterload. PPH occurs most commonly in young and middle-aged women; mean survival from onset of symptoms is 2-3 years. The aetiology of PPH is unknown, although familial disease accounts for roughly 10% of cases, which suggests a genetic predisposition. Current theories on pathogenesis focus on abnormalities in interaction between endothelial and smooth-muscle cells. Endothelia-cell injury may result in an imbalance in endothelium-derived mediators, favouring vasoconstriction. Defects in ion-channel activity in smooth-muscle cells in the pulmonary artery may contribute to vasoconstriction and vascular proliferation. Diagnostic testing primarily excludes secondary causes. Catheterisation is necessary to assess haemodynamics and to evaluate vasoreactivity during acute drug challenge. Decrease in pulmonary vascular resistance in response to acute vasodilator challenge occurs in about 30% of patients, and predicts a good response to chronic therapy with oral calcium-channel blockers. For patients unresponsive during acute testing, continuous intravenous epoprostenol (prostacyclin, PGI2) improves haemodynamics and exercise tolerance, and prolongs survival in severe PPH (NYHA functional class III-IV). Thoracic transplantation is reserved for patients who fail medical therapy. We review the progress made in diagnosis and treatment of PPH over the past 20 years.     

Methylmalonic aciduria in pregnancy: a case report

Methylmalonic aciduria is a rare metabolic disorder of amino acid metabolism that is characterized by accumulation of large amounts of methylmalonic acid in the blood and urine. To our knowledge this is the first case report of a patient with methylmalonic aciduria who carried a pregnancy to term; the outcome was favorable despite high levels of methylmalonic acid in the serum and urine.     

Phyllodes tumour in pregnancy: a case report

An 18 1/2-year-old castrated male donkey with progressively worsening right forelimb lameness presented with a mass on the distal dorsal aspect of its P3 bone. Grossly, the firm, gritty mass was infiltrative, disrupted the contours of the overlying hoof wall, and had mottled and cavitated areas on cut surface. Histologically, the growth was composed of densely cellular sheets of mildly pleomorphic mesenchymal cells forming irregularly shaped islands of poorly mineralized osteoid. The neoplastic mass had patchy areas of necrosis. The diagnostic possibilities considered for this donkey's mass include osteosarcoma, osteoma, ossifying fibroma, and fibrous dysplasia. Careful consideration of the gross and histological characteristics of this donkey's mass support a diagnosis of osteosarcoma.     

Primary pulmonary arterial hypertension

This study describes the results of a survey undertaken to assess the management of potentially malignant oral mucosal lesions by oral medicine practitioners and compares their approach with that of oral & maxillofacial surgeons that we have previously described. Significant differences were noted between the two groups in the use of photography to document the lesions and in the use of certain special investigations, which included measurement of serum iron, serum ferritin, serum Vit B12, red cell folate and candidal isolation. The groups also varied in the perceived importance of the age of the patient and anatomical site of the lesion when deciding on the need for further biopsy. There was also significant variation in the use of certain treatment modalities, including excising non-dysplastic and severely dysplastic/carcinoma in-situ lesions and eliminating trauma when treating mild/moderately dysplastic and severely dysplastic/carcinoma in-situ lesions. Significant differences in the frequency and duration of follow-up were noted for non-dysplastic lesions. Finally, the two groups differed significantly when asked to rank the perceived importance of certain factors (the histopathology of the most recent biopsy and the anatomical site of the lesion) when deciding the need to follow-up. Possible reasons for the variation are discussed.     

Electroconvulsive therapy in a twin pregnancy: a case report

We present a case of twin gestation complicated by severe depression and psychotic behavior; the mother was treated with electroconvulsive therapy (ECT). She had received multiple medications for treatment of her depression earlier during the first part of the pregnancy. However, frequent use of ECT later in the course of pregnancy did not result in adverse fetal outcome as is evident from normal fetal surveillance tests. We conclude that, when indicated, ECT during pregnancy improves maternal condition and does not adversely affect fetal well-being.     

Primary pulmonary hypertension in adults]

Primary pulmonary hypertension is a rare disease of unknown etiology which relates to the precapillary form of the hypertension. The review of the literature and authors' data on the changes of pulmonary vessels and the heart weight are presented. So-called plexiform structure characteristics for this pathology are considered by the authors as glomus anastomoses developing as a compensation of pulmonary circulation disturbances.     

Primary bronchopulmonary fibrosarcoma: report of a case

We herein report a case of primary bronchopulmonary fibrosarcoma in a 70-year-old man. The patient was referred to our hospital for investigation of hemosputum and an abnormal shadow. On admission, chest radiograph and computed tomography scan showed a mass lesion in right S3 and an infiltrative shadow in the right upper lobe. Transbronchial biopsy specimens showed findings of malignancy, and adenocarcinoma was suspected. A right pneumonectomy was performed, and pathologic examination confirmed a diagnosis of fibrosarcoma. The patient had an uneventful recovery and no sign of recurrence has been found in the year since his operation, although strict follow-up is essential.     

Rupture of rudimentary horn pregnancy: a case report

OBJECTIVE: The aim of this study was to develop a method for measuring renal function with 99mTc-MAG3 gamma-camera renography without blood or urine sampling and evaluate its feasibility. PATIENTS, MATERIAL AND METHODS: Twelve patients with nephrological disorders underwent 99mTc-MAG3 renography and para-aminohippurate clearance measurement. Plasma clearance of 99mTc-MAG3 (ClMAG) was calculated through early renal uptake of 99mTc-MAG3 after appropriate correction of parameters (background, measured attenuation coefficient of 99mTc, and the actual depth of kidneys measured with computed tomography), and on one-compartment assumption of the kinetics of 99mTc-MAG3. We compared the resultant ClMAG with standard effective renal plasma flow (ERPF), using the para-aminohippurate clearance method and with simulated ClMAG derived from the two-compartment model. RESULTS: ClMAG calculated by the one-compartment model (283+/-131 ml/min, mean +/- SD) correlated with ERPF (r = 0.94, p <0.001), and was similar to the simulated ClMAG estimated from the two-compartment model in all patients (283+/-139 ml/min). CONCLUSION: This alternative method, which employs theoretical modeling of the pharmacokinetics of 99mTc-MAG3, may provide easy, noninvasive measurement of individual renal function without blood sampling or in vitro equipment. Further studies should be warranted.     

Marked pulmonary function abnormalities in a case of HIV-associated pulmonary hypertension

Recent reports have suggested an association between primary pulmonary hypertension and human immunodeficiency virus (HIV) infection. This appears to be an accelerated syndrome, associated with a relatively brief duration of symptoms, yet prominent right ventricular failure and severe pulmonary hypertension on presentation. We present a case of a primary pulmonary hypertension in a 35-year-old HIV-seropositive hemophiliac. His accelerated clinical course is consistent with previously reported cases of HIV-related pulmonary hypertension. However, this patient's pulmonary function tests revealed marked hyperinflation, a decreased diffusing capacity, and no airflow obstruction. To our knowledge, this very usual constellation of pulmonary function changes has not been described previously in this syndrome.     

Primary leiomyoma of the ovary: a case report

The imaging features of primary ovarian leiomyoma have seldom been described because of the very low prevalence of the tumor. Herein we present a case report on its magnetic resonance imaging features. Both T1- and T2-weighted images revealed a low-signal-intensity mass, which was well circumscribed and sharply demarcated from the uterus. Magnetic resonance imaging was useful in distinguishing it from a malignant ovarian tumor and uterine leiomyoma.