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Biomedical subjects

Claire Langston

Publications and source records attributed to Claire Langston.

16 recordsLinked to original sources
PubMed2006

Spontaneous pulmonary interstitial emphysema in a term unventilated infant.

Pulmonary interstitial emphysema (PIE) is a form of air block most frequently seen in ventilated preterm infants with severe lung disease; it is rarely reported in spontaneously breathing term infants. We report on an infant previously diagnosed with laryngomalacia and congestive heart failure and with evidence of antenatal stroke before the onset of pulmonary disease. He presented at 6 weeks of age with spontaneous pneumothorax. Focal cystic changes were seen on imaging studies of the lungs. There was no prior history of mechanical ventilation. Prior chest X-rays did not show cystic changes. He subsequently underwent resection of the affected lung areas. Pathologic examination revealed persistent PIE with cystic expansion, pleural blebs, and reactive pleuritis, as well as subpleural air-space enlargement. The patient did well postoperatively and was discharged home without further problems. This case demonstrates that PIE can occur in an infant without any history of mechanical ventilation, suggesting the need for a high index of suspicion for PIE, even in nonventilated and spontaneously breathing term neonates. PIE should be included in the differential diagnosis of cystic lung lesions in all young infants.

Heart Failure↗
PubMed2006

Prenatal presentation and outcome of children with pleuropulmonary blastoma.

BACKGROUND/PURPOSE: Pleuropulmonary blastoma (PPB) is a rare primary neoplasm of pleuropulmonary mesenchyme. Fewer than 170 children have been reported, and few single institutions have reported more than several cases. Treatment for this condition is primarily surgical resection; however, increasing experience suggests that adjuvant chemotherapy may decrease recurrence and improve outcome. METHODS: We reviewed the charts of all children with PPB treated at our institution since 1960. We reviewed the prenatal features, clinical presentation, operation, pathological findings, adjuvant treatment, and outcome. RESULTS: Ten children (6 boys and 4 girls) were treated for PPB at a mean age of 3.2 +/- 4.3 years. In 2, a cystic lung mass was diagnosed prenatally, and in 8, a cystic or solid and cystic lung mass was diagnosed postnatally (right lung, 3; left lung, 4; and bilateral, 3). In no patient was PPB considered preoperatively. Surgical resection was performed at 1 day to 11 years (median, 23 months) of age. Seven children had complete resection; 1 had microscopic residual disease, and 2 had gross residual disease. Pathology showed type I PPB in 7, type II in 1, and type III in 2. Five patients received adjuvant chemotherapy with vincristine, actinomycin, and cyclophosphamide-based regimens. At follow-up (mean, 7.7 +/- 11.5 years; range, 1-456 months), children with type I PPB have no evidence of disease (n = 6) or are lost to follow-up (n = 1), whereas all those with type II/III PPB have died of the disease. CONCLUSIONS: PPB must be included in the differential diagnosis of a fetus, neonate, or child with a cystic lung mass. This finding supports early resection of these lesions rather than observation or treatment with nonoperative strategies.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed2005

Interstitial lung disease in patients with ataxia-telangiectasia.

Ataxia-telangiectasia (A-T) is an autosomal-recessive multiorgan disease characterized by progressive neurologic deterioration in which the most common causes of death are diseases of the respiratory system and cancers. The aim of this retrospective study was to delineate the clinical, radiographic, and pathologic manifestations of the chronic progressive interstitial lung disease seen in patients with A-T. The medical records of 97 patients with A-T and chronic lung disease were reviewed. Interstitial lung disease (ILD) was specifically diagnosed in 25 of 97 patients. None of these patients had evidence of an infectious process preceding the onset of their lung disease, and none had lasting clinical improvement after treatment with antibiotics. Although many medications were used to treat their ILD, only treatment with systemic corticosteroids early in the course of their illness was associated with clinical and radiographic improvement. Nineteen of these 25 patients with ILD died within 24 months of the onset of their ILD, and of 7 patients treated with corticosteroids, 5 are still alive. Recognition of interstitial lung disease in patients with A-T and its early treatment could reduce or eliminate pulmonary disease as a leading cause of death for these patients.

Adolescent↗
PubMed2005

Persistent tachypnea of infancy is associated with neuroendocrine cell hyperplasia.

We sought to determine the clinical course and histologic findings in lung biopsies from a group of children who presented with signs and symptoms of interstitial lung disease (ILD) without identified etiology. Patients were identified from the pathology files at the Texas Children's Hospital who presented below age 2 years with persistent tachypnea, hypoxia, retractions, or respiratory crackles, and with nonspecific and nondiagnostic lung biopsy findings. Age-matched lung biopsy controls were also identified. Their clinical courses were retrospectively reviewed. Biopsies were reviewed, and immunostaining with antibodies to neuroendocrine cells was done. Fifteen pediatric ILD patients and four control patients were identified for inclusion in the study. Clinically, the mean onset of symptoms was 3.8 months (range, 0-11 months). Radiographs demonstrated hyperinflation, interstitial markings, and ground-glass densities. Oxygen was initially required for prolonged periods, and medication trials did not eliminate symptoms. After a mean of 5 years, no deaths had occurred, and patients had improved. On review of the lung biopsies, all had a similar appearance, with few abnormalities noted. Immunostaining with antibodies to neuroendocrine cell products showed consistently increased bombesin staining. Subsequent morphometric analysis showed that immunoreactivity for bombesin and serotonin was significantly increased over age-matched controls. In conclusion, we believe this may represent a distinct group of pediatric patients defined by the absence of known lung diseases, clinical signs and symptoms of ILD, and idiopathic neuroendocrine cell hyperplasia of infancy. These findings may be important for the evaluation of ILD in young children.

Age of Onset↗
PubMed2005

Pulmonary capillaritis in children: a review of eight cases with comparison to other alveolar hemorrhage syndromes.

OBJECTIVE: To review clinical, laboratory, and outcome characteristics of children diagnosed with pulmonary capillaritis (PC), a small-vessel vasculitis, presenting as diffuse alveolar hemorrhage (DAH), and to compare these findings with those for children with other alveolar hemorrhage syndromes. STUDY DESIGN: A retrospective chart review of patients who underwent a lung biopsy because of a clinical suggestion of pulmonary hemorrhage. RESULTS: PC was identified in 8 of 23 patients. In these patients, cough, crackles, and hypoxia were common. Alveolar infiltrates on radiography and anemia were present in 7 of 8 cases. Serologic evidence of a systemic vasculitis was present in 50% of patients. High-dose corticosteroids proved effective in controlling alveolar hemorrhage in all cases. There were no presenting signs or symptoms that could differentiate patients with PC from those with non-immune-mediated alveolar hemorrhage. In general, patients with PC had a lower hematocrit and higher erythrocyte sedimentation rate (ESR). CONCLUSION: Children presenting with lower respiratory tract symptoms, chest x-ray abnormalities, and anemia should undergo evaluation for PC, as early initiation of immunosuppression can be lifesaving and organ sparing. No clinical signs to differentiate immune and non-immune-mediated alveolar hemorrhage were evident in this study.

Biopsy↗
PubMed2004

Pediatric interstitial lung disease revisited.

The spectrum of pediatric interstitial lung disease (PILD) includes a diverse group of rare disorders characterized by diffuse infiltrates and disordered gas exchange. Children with these conditions typically present with tachypnea, crackles, and hypoxemia. Recent advances have been made in the identification of different types of PILD that are unique to infancy. More exciting has been the discovery of genetic abnormalities of surfactant function, now described in both children and adults. A systematic evaluation of the child presenting with diffuse infiltrates of unknown etiology is essential to the diagnosis. Most often, lung biopsy is required. Current treatment options remain less than satisfactory, and morbidity and mortality remain considerable.

Biopsy↗
PubMed2004

Expanding the phenotype of alveolar capillary dysplasia (ACD).

OBJECTIVES: To define the phenotype of congenital alveolar capillary dysplasia (ACD) as a first step toward mapping the responsible gene(s). STUDY DESIGN: Analysis of pathology reports and microscopic slides of 23 subjects with ACD and sequence analysis of two candidate genes. RESULTS: Our review of the pre- and postmortem records delineates both the natural history of this condition and the associated anomalies. Our collection of families corroborates the likely autosomal recessive nature of this condition in some families and provides additional data for genetic and prenatal counseling. Anomalies of many organ systems were detected either in the prenatal period or during the hospital course. However, some major anomalies were not detected until postmortem examination. Left-right asymmetry and gastrointestinal malrotation emerge as important, previously recognized but underappreciated phenotypic features of ACD. Finally, we used sequence analysis to exclude mutations in the coding region of two candidate genes, bone morphogenetic protein type II receptor (BMPR2) and endothelial monocyte-activating polypeptide II (EMAP II), as candidates for ACD. CONCLUSIONS: Understanding the clinical spectrum of ACD and the cloning of an "ACD gene" both have implications for counseling, for prenatal testing, and for understanding the molecular pathophysiology of ACD and other organ malformations that are associated with this condition.

Abnormalities, Multiple↗
PubMed2004

Pulmonary capillaritis and hemorrhage in neonatal lupus erythematosus (NLE).

We describe the case of an infant who presented to our institution with tachypnea, cough, and hypoxemia. The baby had been diagnosed with complete heart block (heart rate 50-60 beats/min) in utero and had a pacemaker placed at birth. The mother was found to have both anti-Ro and anti-La antibodies. At age 3 months, the chest radiograph showed diffuse bilateral interstitial infiltrates. A lung biopsy confirmed the presence of necrotizing capillaritis and alveolar hemorrhage, which is an infrequent complication in pediatric patients with systemic lupus erythematosus. The patient did well after recognition and aggressive treatment of this previously unrecognized complication of neonatal lupus.

Journal Article↗
PubMed2003

New concepts in the pathology of congenital lung malformations.

Focal developmental malformations of the lung are a heterogeneous group of lesions that display a variety of features. They often are thought to be quite distinct entities, but there is considerable overlap among these lesions. Both standard nomenclature and common usage do not identify clearly the components of these lesions in a distinctive fashion. A classification based on pathologic features is presented. A malformation sequence based on airway obstruction during development is proposed as a unifying pathogenetic mechanism for some seemingly unrelated lesions.

Bronchi↗
PubMed2002

Medial smooth muscle thickness in small pulmonary arteries in sudden infant death syndrome revisited.

Increased relative medial thickness (RMT) of smooth muscle in small pulmonary arteries, peripheral extension of smooth muscle into the alveolar wall arteries, and right ventricular hypertrophy (RVH), in response to purported prolonged hypoxia, have been reported in sudden infant death syndrome (SIDS). Prone sleep position, an important risk factor for SIDS, predisposes infants to hypoxia from airway obstruction or rebreathing. Since publication of the earlier pulmonary artery studies, the SIDS definition has been expanded, and sudden infant death investigational protocols have been implemented. Our aims in this study were to (1) compare RMT in preacinar arteries (PA), intra-acinar arteries accompanying small airways (SIA), and alveolar wall arteries (AW) in SIDS infants and controls; (2) correlate RMT with postmortem variables; (3) determine if peripheral extension occurred more often in SIDS infants than in controls; and (4) determine if RVH occurred in SIDS. Movat-stained sections from standardized tissue blocks taken prospectively from the apex of the right upper lobe from 88 SIDS cases and 17 controls were evaluated using a computer-assisted digitizing system with images obtained from a microscope with an attached video camera. When adjusted for age, the RMT values for the SIA arteries were significantly greater in controls, while the PA and AW arteries were not statistically different between the SIDS cases and controls. Peripheral medial smooth muscle extension did not differ between the groups, and RVH was not seen in SIDS cases. Given the recent identification of brain stem abnormalities interfering with protective cardiorespiratory responses against acute life-threatening hypoxia perhaps precipitated by prone sleeping, our data suggest that SIDS is an acute event not preceded by recurrent or prolonged apnea and hypoxia.

Age Factors↗
PubMed2002

Lethal toxicity, severe endothelial injury, and a threshold effect with high doses of an adenoviral vector in baboons.

The effects of intravenous administration of a first-generation adenoviral vector expressing beta-galactosidase were compared in two baboons receiving a high dose or lower dose of vector, 1.2 x 10(13) or 1.2 x 10(12) particles/kg, respectively. The high-dose baboon developed acute symptoms, decreased platelet counts, and increased liver enzymes, and became moribund at 48 hr after injection, while the lower-dose baboon developed no symptoms. Expression of the beta-galactosidase transgene was prominent in liver, spleen, and endothelium of the arterial vasculature in the high-dose baboon, but was much more limited and spared the endothelium in the lower-dose baboon. Injury to the vascular endothelium was the most prominent abnormality in the high-dose baboon. Extensive histological studies provide a detailed picture of the pathology associated with a lethal dose of first-generation adenoviral vector in a primate.

Adenoviridae↗
PubMed2002

Pulmonary lipoblastoma in an 18-month-old child: a unique tumor in children.

Lipoblastoma, a rare benign tumor of adipose tissue, typically occurs in the superficial or deep layers of soft tissue on the trunk or extremities. Other sites of occurrence include the head, neck, and retroperitoneum. Lipoblastoma of the chest wall and parietal pleural have been reported, but occurrence within the lung has not been previously described. We report a case of pulmonary lipoblastoma in a young child presenting with complete opacification of the left hemithorax and mediastinal shift on chest radiograph. A lobectomy was performed, and the diagnosis was made by histological examination.

Humans↗
PubMed2002

Epstein-Barr virus-associated smooth muscle tumors in ataxia-telangiectasia: a case report and review.

Chromosomal breakage syndromes, including ataxia-telangiectasia (AT), are autosomal recessive disorders in which DNA repair mechanisms are defective resulting in chromosomal instability. Affected individuals are at high risk for developing malignancy because of the widespread resulting cellular effects. One such effect, severe immunosuppression, can permit virally mediated neoplasms to manifest, similar to those seen in acquired immunodeficiency syndrome (AIDS), congenital immune deficiency syndromes, and posttransplant populations. Epstein-Barr virus (EBV) is a common viral agent known to be associated with lymphoid, epithelial, and smooth muscle malignancies in such patients. Although smooth muscle tumors have been reported in patients with AT, their association with EBV has not been evaluated. We present a case of EBV-associated laryngeal leiomyosarcoma and jejunal cellular leiomyoma in a child with AT. This case suggests that the development of neoplasia in patients with chromosomal breakage syndromes may be related to the immunosuppressive consequences of these diseases, and searching for infectious causes (such as EBV) is important.

Ataxia Telangiectasia↗
PubMedDate not supplied

A protocol for the handling of tissue obtained by operative lung biopsy: recommendations of the chILD pathology co-operative group.

This is the first of a series on pediatric pulmonary disease that will appear as Perspectives in Pediatric Pathology over the coming months. The series will include practical issues, such as this protocol for handling lung biopsies and another on bronchoalveolar lavage in childhood, as well as reviews of advances in various areas in pediatric pulmonary pathology. It has been 11 years since the last Perspectives on pulmonary disease. Much has happened since then in this area, and this collection will highlight some emerging and rapidly advancing areas in pediatric lung disease. These will include a review of molecular mechanisms of lung development, and another of mechanisms of pulmonary vascular development. The surfactant system and its disorders, as well as recent advances in the biology of the pulmonary neuroendocrine system and mechanisms of respiratory viral disease, will be addressed. Articles on pulmonary hypertension, pulmonary neoplasia, and pediatric lung transplantation, with their implications for the pediatric pathologist, are also planned. The contributors to this series are a diverse group with special interests and expertise in these areas. As Dr. William Thurlbeck noted in his foreword to the previous volume, Pulmonary Disease, volume 18 of Perspectives in Pediatric Pathology, pediatric pathology had been largely concerned with phenomenology, rather than with mechanisms, model systems, and experimental investigation. I think he would have been pleased to see the changes that have occurred over the past 10 years in pediatric lung biology and pathology in particular, because these were particularly favored interests of his later years.

Child↗