Rigid spine syndrome with chronic respiratory failure.

Rigid spine syndrome (RSS) is a rare myopathic ailment characterized by mild axial and proximal muscle weakness. Muscle contraction in these patients causes limitation of neck and trunk flexion, scoliosis, and mild joint deformity. We report the case of a 30-year-old man with RSS who presented with severe restrictive ventilatory defect (forced vital capacity, 1.53 L, 39% of predicted), mild scoliosis (Cobb's angle 12), proximal muscle weakness and stiff back. Creatine phosphokinase was 986 IU/L. Muscle biopsy of the right vastus lateralis revealed increased variation in muscle fiber diameter, proliferation of endomysium, and type I fiber atrophy. Round and whorled fibers indicating myopathic change were found on sections stained with nicotinamide adenine dinucleotide dehydrogenase and succinate dehydrogenase. RSS was diagnosed based on myopathic findings and clinical presentation. Nocturnal chronic respiratory failure and fragmented sleep developed. He was treated with continuous positive airway pressure and had recovered his normal daily activity by 1-year follow-up. We suggest that patients with RSS should be assessed for possible ventilatory failure; treatment with nasal continuous positive airway pressure or bilevel continuous positive airway pressure therapy should be considered.     

Role of open lung biopsy in patients with diffuse lung infiltrates and acute respiratory failure.

BACKGROUND AND PURPOSE: Open lung biopsy (OLB) is the standard procedure for the diagnosis of specific parenchymal lung diseases. The purpose of this study was to investigate the influence of OLB on subsequent treatment strategy and outcome in patients with diffuse lung infiltrates and acute respiratory failure. METHODS: This retrospective review included 32 patients (aged 50.6 +/- 21.7 years) with acute respiratory failure and diffuse pulmonary infiltrates who underwent OLB from 1990-2002. Data analyzed included diagnoses, treatment alterations, 30-day survival, oxygenation status, and histologic results. RESULTS: Specific diagnoses were made in 53.1% of patients (17/32), 23 (71.9%) of whom had acute respiratory distress syndrome (ARDS). Diagnostic yields did not differ with immunity status or ARDS. OLB led to specific decisions of treatment in 46.9% of patients (15/32), and only 7 of these 32 patients (21.8%) survived. Overall mortality was 56.2% (18/32) and was not influenced by pre-OLB oxygenation or histologic results. Although perioperative complications affected 40.6% of patients (13/32), none of the deaths were surgery-related. Complication rates were significantly higher in patients with ARDS (p = 0.04). CONCLUSIONS: OLB is associated with a low perioperative mortality rate and acceptable morbidity rate in patients with diffuse lung infiltrates and acute respiratory failure, including those patients with ARDS. In this study, a specific diagnosis was obtained by OLB in more than half of patients with diffuse pulmonary infiltrates and ARDS. In addition, OLB resulted in either use of a new therapeutic strategy or elimination of unnecessary treatment in nearly one-half of patients (46.9%).     

Acute renal failure in patients with severe acute respiratory syndrome.

BACKGROUND AND PURPOSE: Severe acute respiratory syndrome (SARS) is caused by a new coronavirus, and results in respiratory failure. Acute renal failure (ARF) may also occur and/or complicate the disease course, however, its incidence, causes and impact in SARS patients are not known. METHODS: ARF patients were identified from a total of 78 (33 men and 45 women) probable SARS cases admitted to a single hospital. The clinical features of patients with ARF were characterized, and the etiologies analyzed. RESULTS: Patients were assigned to ARF (n = 13; 17%) and non-ARF groups (n = 65). Patients with ARF were older than their non-ARF counterparts. ARF developed 7.2 +/- 4.3 days after admission. The incidence of ARF was higher in males (77% vs 35%; p < 0.05). Comorbidities of diabetes and heart failure were more common in patients who developed ARF (38% vs 6%, p < 0.01 and 38% vs 2%, p < 0.001, respectively) and the incidence of respiratory failure (85% vs 26%, p < 0.001) and mortality (77% vs 8%, p < 0.001) were also higher. Multiple organ system failure usually accompanied ARF. Hypotension (77%) caused by nosocomial infections, gastrointestinal bleeding, or SARS per se, and rhabdomyolysis (43%) was associated with ARF in addition to pre-renal factors. Five patients in the ARF group and 1 female patient with end-stage renal failure underwent renal replacement therapy during hospitalization; however, both eventually died. Of the 16 medical staff performing renal replacement therapy, none was subsequently infected with SARS coronavirus. CONCLUSIONS: Development of ARF during the disease course in SARS patients is associated with catastrophic outcome. The cause of ARF in SARS patients is often associated with pre-renal factors, hypotension, rhabdomyolysis, and previous comorbidities including diabetes and old age. Universal precautions to prevent viral transmission are mandatory for medical staff performing renal replacement therapy.     

Experimental model of respiratory distress syndrome by lung lavage with fluorocarbon

The purpose of this study was to determine how much alveolar surfactant is washed out by lung lavage with fluorocarbon and also to find out whether or not instillation of artificial surfactant can restore pulmonary function after lung lavage in rats. The lung lavage was performed manually, administering a tidal volume 5 mg (2 mg/100g body weight) of fluorocarbon for about eight seconds. This process was repeated more than 40 times. In the study group, 4 ml/kg (120 mg/kg) of artificial surfactant was instilled into the trachea, and the same amount of normal saline was instilled in the control group. The amount of phospholipids extracted in lung lavage was 16.6 +/- 3.6 mg/kg body weight in the control group and 18.9 +/- 3.7 mg/kg body weight in the experimental group. Immediately after instillation of the surfactant, arterial oxygen pressure increased from 84 +/- 17 mmHg to 195.1 +/- 26.7 mmHg, and remained high, at about 170-260 mmHg. In contrast the physiological saline treated group did not show any change. We conclude that lung lavage with fluorocarbon is an adequate ideal experimental model of respiratory distress syndrome and suggests that combination of artificial surfactant treatment after lung lavage with fluorocarbon might be a new pulmonary washing method for severe lung disease.     

Acute respiratory distress syndrome caused by leukemic infiltration of the lung.

Respiratory distress syndrome resulting from leukemic pulmonary infiltrates is seldom diagnosed antemortem. Two 60- and 80-year-old women presented with general malaise, progressive shortness of breath, and hyperleukocytosis, which progressed to acute respiratory distress syndrome (ARDS) after admission. Acute leukemia with pulmonary infection was initially diagnosed, but subsequent examinations including open lung biopsy revealed leukemic pulmonary infiltrates without infection. In one case, the clinical condition and chest radiography improved initially after combination therapy with chemotherapy for leukemia and aggressive pulmonary support. However, new pulmonary infiltration on chest radiography and hypoxemia recurred, which was consistent with acute lysis pneumopathy. Despite aggressive treatment, both patients died due to rapidly deteriorating condition. Leukemic pulmonary involvement should be considered in acute leukemia patients with non-infectious diffusive lung infiltration, especially in acute leukemia with a high blast count.     

ABCA3 deficiency: neonatal respiratory failure and interstitial lung disease

ABCA3 is a member of the ATP Binding Cassette family of proteins, transporters that hydrolyze ATP in order to move substrates across biological membranes. Mutations in the gene encoding ABCA3 have been found in children with severe neonatal respiratory disease and older children with some forms of interstitial lung disease. This review summarizes current knowledge concerning clinical, genetic, and pathologic features of the lung disease associated with mutations in the ABCA3 gene, and also briefly reviews some other forms of childhood interstitial lung diseases that have their antecedents in the neonatal period and may also have a genetic basis.     

Langerhans' cell histiocytosis presenting with a para-aortic lesion and heart failure.

Langerhans' cell histiocytosis (LCH) is an uncommon disease with variable manifestations. We report a case of LCH with the unusual initial presentations of chest pain and progressive heart failure in a 5-year-old boy. Chest radiography revealed a wide mediastinum with cardiomegaly. Electrocardiography showed first-degree atrioventricular block and an inverted T wave over V4-V6. Echocardiography, computed tomography, and magnetic resonance imaging of the chest all showed an infiltrating lesion that enveloped the entire heart, great vessels, and coronary arteries. Pathologic examination of the biopsy specimen revealed LCH. Chemotherapy, which included prednisolone, vincristine, methotrexate, and 6-mercaptopurine, had only a minimal effect on the tumor. After the addition of etoposide, the lesion decreased in size, and the symptoms and signs of heart failure and chest pain were ameliorated.     

Small cell anaplastic lung cancer presenting as an ovarian metastasis

An unusual case of small cell anaplastic carcinoma of the lung, presenting as a pelvic mass, is presented. Histochemical and electron microscopic findings confirmed the diagnosis. The pathologic features and unique clinical characteristics are discussed with comments on the topic of metastatic ovarian tumors.     

Recurrent small cell lung cancer presenting as bilateral adnexal masses

BACKGROUND: Small cell lung cancer (SCLC) carries a very poor prognosis, although recent advances in chemotherapy have produced some long-term survivors. Despite this, recurrences are common, and these patients require a high level of surveillance. Given that the female gender is a favorable prognostic factor in patients with SCLC and that more women are becoming lung cancer survivors, it is important to consider sites of metastasis that are restricted to women. CASE: This is a presentation of a 42-year old previously diagnosed with SCLC who presented with an isolated recurrence of SCLC in the ovaries. This is a case report of an isolated recurrence of SCLC in the ovaries with a review of the literature. CONCLUSION: Although systemic relapses and recurrences often occur in these patients, isolated metastasis such as this can occur and should be considered in female patients with a history of small cell lung cancer.     

Acute respiratory distress syndrome and lung fibrosis after ingestion of a high dose of ortho-phenylphenol.

Ortho-phenylphenol (OPP) and its sodium salt are used as fungicides and antibacterial agents, ingestion of which has been found to cause liver toxicity, renal toxicity and carcinomas in the urinary tract of rats. Lung damage due to OPP ingestion has not been reported in humans. We report a suicidal 39-year-old woman with stage II cervical cancer who drank a potentially lethal dose of OPP in the form of a commercial antiseptic, which led to the complication of liver and renal function impairment, severe lung damage with acute respiratory distress syndrome and subsequent severe lung fibrosis. Open lung biopsy showed diffuse alveolar damage. She was discharged after 34 days of hospitalization with continuing domiciliary oxygen therapy.     

Electrolyte disorders associated with respiratory distress syndrome and bronchopulmonary dysplasia.

In understanding the various electrolyte disorders associated with respiratory failure, one has to appreciate how the otherwise healthy premature infant maintains extracellular fluid homeostasis. The same physiologic processes that regulate body fluids are affected to varying degrees in the infant with respiratory failure. Maintenance of sodium conservation by the kidney is of critical importance for survival and growth. The immature kidney tends to exhibit excessive sodium wasting especially early on, but appropriate positive sodium balance is achieved generally within the first week. Appropriate reduction in extracellular fluid occurring during the first week or so of life is expected and if it is not allowed to occur may result in dilutional hyponatremia and an increase in pulmonary interstitial water. Diuretics, when used to promote an increase in lung compliance, can themselves generate significant morbidity. Sodium depletion, hypokalemia, alkalosis, and calcium wasting all can either be caused by or exaggerated by the loop diuretics. Thus, one must consider the maturational state of the infant, the infant's response to pulmonary failure, and iatrogenic factors influencing the outcome.     

Familial respiratory distress syndrome in three consecutive full-term infants. Case reports and documentation of lung enzyme activities.

Familial respiratory distress syndrome in full-term newborn infants is a rare occurrence. Our patient delivered three consecutive full-term infants who developed findings consistent with respiratory distress syndrome. All three died from autopsy-proven hyaline membrane disease. Analysis of the activities of four enzymes that play an important role in the biosynthesis of lecithin (choline kinase, choline phosphotransferase, phospholipase A and lysolecithin acyltransferase) failed to disclose an abnormality in lung samples in our patient with familial respiratory distress syndrome.