Glomerulomegaly and proteinuria in a patient with idiopathic pulmonary hypertension

In an attempt to identify genes involved in neuroblastoma, we scanned neuroblastoma tumour DNAs for homozygous deletions by representational difference analysis (RDA). The RDA produced several difference products, nine of which represented hemizygous deletions located on chromosome 1 or 3. In order to detect deletions, a genomewide loss of heterozygosity (LOH) screening with polymorphic markers was performed. Allelic losses on a number of different chromosomes were detected, mainly in favourable neuroblastomas (stage 1, 2 and 4S). The most frequently deleted region, apart from 1p, was chromosomal region 3p. A more detailed study was made in this region, which showed that 9 out of 58 (16%) tested neuroblastoma tumours showed allelic loss in the same region on chromosome 3p, i.e. 3pter-14.2. Thus, both RDA and LOH studies showed chromosome region 3p as being frequently involved in deletions and/or rearrangements in neuroblastoma tumours. Therefore, it is possible that one or more of the 3p genes implicated in the development of other cancers also play a role in neuroblastoma development and/or progression.     

Severe pulmonary hypertension: a feature of Swyer-James syndrome?

Swyer-James syndrome, unilateral hyperlucent lung with air entrapment, generally occurs after severe infections during childhood. It is usually diagnosed by its characteristic chest radiographic image film or computed tomography, in patients who are almost asymptomatic. We report a case of Swyer-James syndrome, diagnosed from the study of severe pulmonary hypertension and with a fatal outcome.     

Long-term inhalation of nitric oxide for a patient with primary pulmonary hypertension

Primary pulmonary hypertension is a disease with a high mortality rate and for which there is no satisfactory medical treatment. The safety of long-term inhalation of nitric oxide (NO) as a treatment is described. A 9-year-old girl inhaled NO for 32 weeks, accompanied with oral administration of beraprost sodium. Although NO did not improve her long-term prognosis, it eased the patient's dyspnea and increased her blood oxygenation. At doses of 20 ppm or more, attempts to withdraw from inhaled NO seemed to lead to an immediate elevation of the pulmonary artery pressure. This rebound phenomenon did not happen at doses under 5 ppm. This case study suggests that long-term inhalation of NO is safe and effective, but that pulmonary hypertension may rebound following withdrawal at higher doses of NO.     

Agrobacterium radiobacter bacteremia in a patient with chronic obstructive pulmonary disease

Agrobacterium radiobacter is a gram-negative bacillus, which is recognized as an emerging opportunistic human pathogen. To our knowledge, there have been only 25 cases of A. radiobacter bacteremia reported. In most of these, A. radiobacter was associated with long-term indwelling plastic central venous catheters. We describe a 78-year-old man who had a history of chronic obstructive pulmonary disease with long-term use of a corticosteroid. He was admitted to the China Medical College Hospital with pneumonia caused by Serratia marcescens. His general condition gradually improved after initiation of appropriate treatment. Unfortunately, he developed A. radiobacter bacteremia while hospitalized in the medical intensive care unit. With the onset of this infection, the patient had a high fever, leukocytosis, raised C-reactive protein level, and positive blood cultures for A. radiobacter. A central venous catheter-related infection was suspected because of redness and localized tenderness at the catheter site. The patient gradually recovered after removal of the catheter and appropriate antimicrobial treatment with latamoxef 1.5 g intravenously every 8 hours for 10 days.     

PCR analysis of T. whippelii DNA in a case of Whipple's disease: effect of antibiotics and correlation with histology

A 58-yr-old man developed severe weight loss, arthralgias, and diarrhea. Endoscopic examination of the stomach and duodenum revealed thickened folds of duodenal mucosa. Biopsy of the gastric mucosa was negative, whereas duodenal biopsy revealed blunted epithelial villi and PAS-positive foamy macrophages within the lamina propria. Bacilli typical of those associated with Whipple's disease were found by electron microscopy. The diagnosis was confirmed by polymerase chain reaction (PCR) assay, which detected a portion of the 16S ribosomal RNA gene sequence corresponding to the Whipple bacillus (Tropheryma whippelii) in duodenum, stomach, and liver biopsies before therapy. T. whippelii DNA was eliminated from all tissues tested within 3 months of starting antibiotic treatment, but the histological improvement lagged behind the clinical and molecular evidence of improvement.     

Symptomatic cardiomyopathy as a presentation in Whipple's disease

A patient presenting with congestive cardiac failure and anaemia underwent investigation which led to the diagnosis of Whipple's disease, associated with dilated cardiomyopathy. Conventional antibiotic therapy for Whipple's disease resulted in resolution of the traditional features of Whipple's disease and a marked improvement in the patient's heart failure.     

Severe pulmonary manifestation in adult-onset Still's disease

Adult onset Still's disease may sometimes be complicated by severe manifestations. We report here a case of adult Still's disease with acute respiratory failure requiring mechanical ventilation.     

Approach to the patient with pulmonary disease

The approach to the HIV-infected patient with pulmonary disease is summarized by the algorithms in Figures 3 and 4. These are not intended to be followed in a rigid step-wise fashion. Rather, the practitioner's knowledge of the patient with his or her accompanying medical risks influences the path taken, including the depth and the speed of the evaluation. For example, the patient with cough who is afebrile and breathing at 18 breaths a minute, with a normal chest radiograph and a CD4 count of 350 cells/mm3, is reasonably treated with a macrolide or cephalosporin for bacterial bronchitis and clinical follow-up while awaiting cultures (see Fig. 4). A febrile patient with a cough productive of thin mucus, but known to have a CD4 count of 60 cells/mm3 should be started on anti-PCP therapy while being evaluated for PCP with an induced sputum and if nondiagnostic, a bronchoscope despite a normal chest radiograph. Screening can be as simple as placing an oximeter on the patient's finger in the clinic. If the oxygen saturation of a patient with a normal chest radiograph is low, then the patient should be hospitalized and begun on treatment for PCP while diagnostic evaluation is initiated. If the oxygen saturation is normal, the patient can be exercised to elicit desaturation. If there is no desaturation, PCP is unlikely. If the results are equivocal (i.e., a decrease in saturation, but less than 3%), rest and exercise arterial blood gases can be performed, along with a Dlco-Gallium scanning can be done in patients known to have abnormal Dlco or those who cannot exercise. Patients with focal infiltrates who have acute onset of symptoms (see Fig. 4) commonly have bacterial infections, but the possibility of PCP or TB should not be dismissed. Induced sputum should be examined if TB or PCP is suspected. Patients who are severely ill might go quickly to bronchoscopy without awaiting improvement on empiric therapy. The patient with diffuse infiltrates (see Fig. 4) needs no screening because the presence of disease is apparent from the radiograph. The diagnostic part quickly leads to bronchoscopy for these patients and the initiation of therapy for PCP when suspected. In patients with known pulmonary KS, gallium scanning can be helpful to rule out acute infection, but bronchoscopy is warranted if the patient is severely ill, or at high risk for PCP. This approach should avoid unnecessary procedures in patients with simple bacterial infections, without missing opportunistic infections and tumors.     

Nocturnal pulmonary hypertension in an elderly patient with sleep apnea syndrome

We report a case of sleep apnea syndrome (SAS) with nocturnal pulmonary hypertension (NPH) in a 71-year-old man suffering from dyspnea during sleep. Severe snoring at night and daytime sleepiness were noticed before admission by his wife. Nocturnal oxygen desaturation (NOD) was documented with a pulse oximeter and severe sleep apnea syndrome was diagnosed on the basis of results of respiratory inductive plethysmography, an apnea index (AI) > 20, minimum SpO2 56%. NPH was diagnosed by Swan-Ganz catheter. The levels of NPH were severe. Elevation of systolic pulmonary arterial pressure (PAP) above 40 mmHg was observed 137 episodes at night. Both NPH and NOD were improved by 1 L/min of nasal oxygen therapy. A number of episodes of systolic PAP above 40 mmHg with oxygen therapy was 55 episodes. Peak mean PAP was 36 mmHg in room air vs 33 mmHg in oxygen therapy. Minimum SpO2 with oxygen therapy was improved to 69%. Total time of SpO2 < 90% at night was 153 minutes in room air vs 37 minutes in oxygen therapy. In this case, NPH and NOD due to severe SAS were remarkably improved by oxygen therapy.     

Serologic monitoring of disease and treatment in a patient with pulmonary aspergilloma

Disease progression and efficacy of fungistatic treatment in pulmonary aspergilloma (PA) are difficult to monitor. The usefulness of chest tomography, IgG-ELISA serology, double immunodiffusion, and IgG-immunoblotting was assessed in monitoring disease progression and efficacy of itraconazole treatment during a 9-yr follow-up of a patient with two exacerbations of PA. A rise in IgG-ELISA titer coincided with a recrudescence of clinical symptoms, whereas a decrease after treatment paralleled clinical improvement. IgG-binding to a 32-kD serine protease and to 60- and 94-kD proteins produced with collagen-containing culture medium closely corresponded with IgG-ELISA titers. IgG-binding to a 40-kD metalloprotease remained at very low levels until symptoms and fungal growth became well advanced, when a sharp rise was seen. Responses to all antigens rapidly diminished after the start of successful treatment with itraconazole. Serology may be a useful adjunct in the monitoring of disease progression and the efficacy of itraconazole treatment in patients with PA. IgG-binding to individual fungal proteins shows subtle differences in kinetics. Immunologic responses to fungal proteases raised with collagen-containing culture media may reflect fungal proteolytic involvement during disease progression and treatment more closely than responses to proteins raised with conventional media.     

Secondary pulmonary hypertension in a patient with severe chronic restrictive pulmonary insufficiency--normalized during treatment with non-invasive intranasal ventilation and oxygen

The present case report describes a young woman with severe restrictive lung failure, who developed pulmonary hypertension. She was treated successfully with nocturnal noninvasive nasal ventilation and oxygen, which removed symptoms and signs of cardiac incompensation. The improvement has persisted for five years.     

Primary pulmonary hypertension in a patient with CD8/T-cell large granulocyte leukemia: amelioration by cladribine therapy

We report a case of primary pulmonary hypertension in an adult man with CD8/T-cell large granulocyte leukemia. Successful treatment of his leukemia with cladribine resulted in dramatic and sustained improvement of his pulmonary hypertension.     

Ureteral obstruction in a patient with chronic granulomatous disease, receiving combined prophylaxis with IFN-gamma and antibiotics

For a successful take, skin grafts must be immobilized when applying dressings. Surgical beds requiring a stent to maintain contact between the graft and wound bed can be treated with Biobrane (Dow B. Hickam, Inc., Sugarland, Texas), which is held in place with skin staples at the periphery. Moistened gauze is placed over the grafted area, the redundant Biobrane stretched over the gauze, and this top layer again stapled at the periphery. When the stent is no longer needed, the top layer of Biobrane and gauze are removed, leaving a graft adherent to the bed and covered with Biobrane. This allows the surgeon to quickly construct an effective stent.     

Non-invasive estimation of pulmonary arterial hypertension in chronic obstructive pulmonary disease

The feasibility and reliability of the combination of several noninvasive methods using a multivariate method of analysis to predict pulmonary artery hypertension (PAH) is evaluated in 20 patients with chronic obstructive pulmonary disease. These methods comprised arterial blood gases (Pao2, Paco2), pulmonary functional parameters (FEV1), echo-Doppler parameters (tricuspid regurgitation jets, acceleration time on pulmonary valve), computed tomography measurements (transhilar distance, hilar thoracic index, and measurement of the descending branch of the right pulmonary artery to the lower lobe). A multiple stepwise regression analysis (including one Doppler parameter, two parameters of arterial blood gases, and one functional parameter) revealed a coefficient of determination (R2) equal to 0.954 for mean pulmonary artery pressure (MPAP) with a standard error of estimate (S.E.E.) of 5.25 mmHg. A stepwise regression analysis including computed tomography and radiographic parameters revealed an R2 equal to 0.970 for PAP with a S.E.E. of 4.26 mmHg. Logistical regression analysis classified correctly 80% of patients with PAH using noninvasive methods such as the diameter of the main pulmonary artery and the diameter of the left pulmonary arterial branch calculated by computed tomography. Not only the presence of PAH but also the level of MPAP can be estimated by the combination of multiple stepwise and logistical regression analyses.     

Fatal interstitial pulmonary disease in a patient with dyskeratosis congenita after allogeneic bone marrow transplantation

Local control rate by radical radiation therapy was analyzed in 33 patients with a piriform sinus cancer. Twenty-five patients (76%) were in stage T3 + T4. Local recurrence-free survival at 3 years was 49% in T1 + T2 and 25% in T3 + T4 (p = 0.01). In T1 + T2 lesions, a biologically effective dose for an acute reaction over 80 Gy and total treatment time less than 70 days appeared to improve local control. In T3 + T4 lesions, good radiation response assessed by the regaining of laryngeal mobility affected local control favorably. An esophageal involvement and destruction of the laryngeal cartilage as well as soft tissue extension precluded the possibility of local control by radiation therapy alone. In addition to the T-stage, other tumor factors should also be considered for predicting local control with radiation therapy.