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Biomedical subjects

B H Landing

Publications and source records attributed to B H Landing.

At least 163 records · Page 9Linked to original sources
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Solitary plasmacytoma of the larynx and upper trachea associated with systemic lupus erythematosus.

Solitary plasmacytoma rarely involves the larynx and trachea. This tumor caused severe laryngeal and tracheal obstruction in an adolescent female with systemic lupus erythematosus of nine years' duration. Her systemic disease had been in remission at the time she developed symptoms of laryngeal and tracheal obstruction by plasmacytoma invasion. A complete survey failed to reveal any other focus of tumor. Because steroid therapy failed to clear the plasma cell infiltrate, she was treated with irradiation after the tumor was surgically debulked endoscopically. Postirradiation biopsy showed no evidence of tumor, but the patient continued to experience moderate airway obstruction from fibrosis in the irradiated trachea. The patient has been followed, and is free of disease nine years after establishing the diagnosis.

Airway Obstruction↗
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Wegener's granulomatosis causing laryngeal and tracheobronchial obstruction in an adolescent girl.

A 12-year-old girl presenting with a painful neck mass and ulcerated gingival lesions was found to have Wegener's granulomatosis involving the gingivae, paranasal sinuses, ears, mastoids, larynx, trachea, bronchial tree, lungs, kidneys and joints. The initial illness and two subsequent exacerbations were treated with intravenous and oral cyclophosphamide and prednisone. Secondary infections have been managed with establishment of good drainage and use of appropriate antibiotics. The patient is well with a normal sedimentation rate 4 1/2 years after the onset of her disease.

Airway Obstruction↗
PubMedDate not supplied

Primary lymphosarcoma of the larynx in a child.

A 4 7/12-year-old Caucasian female with a history of "croup-like symptoms" and persistent airway obstruction, was found to have a primary lymphosarcoma by biopsy at the time of laryngoscopy and bronchoscopy. No metastatic disease was found. After an induction course of vincristine, prednisone and local irradiation, she received CNS prophylaxis with intrathecal methotrexate and cranial irradiation. Maintenance therapy, administered over a 2 3/4 year period, consisted of cyclophosphamide, methotrexate, and 6-mercaptopurine. Excluding the diagnostic evaluation, she was hospitalized only once for the management of suspected sepsis, gastrointestinal ulceration and severe bone marrow depression. Since discontinuing treatment 27 months ago, she has remained free of disease.

Child, Preschool↗
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Epidermolysis bullosa associated with laryngeal stenosis.

A boy with epidermolysis bullosa developed progressive laryngeal obstruction after age six months, with tracheotomy necessary at age three years. By eight years, the larynx was completely occluded by web and scar. Stenosis recurred repeatedly over the next seven years, three to six months after each corrective procedure, but laryngeal patency has been better-maintained since age 15. Laryngeal stenosis, presumably secondary to epithelial detachment, although a rare complication of epidermolysis bullosa, can be serious and life-threatening.

Child↗
PubMedDate not supplied

Neurofibroma of the larynx in a child.

Laryngeal obstruction due to neurofibromatosis in a girl with von Recklinghausen's disease first occurred in the neonatal period, and recurred by age seven years. She has done well for four years following laryngofissure surgery. Pharyngeal and laryngeal neurofibromatosis are rare manifestations of von Recklinghausen's disease. The dominant hereditary pattern of this disease requires that the condition be considered when respiratory distress occurs in the child of an affected parent, whether or not skin tumors or other features are present.

Child, Preschool↗
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Myopathic skeletal muscle fiber abnormalities in cardiomyopathies of childhood.

Skeletal muscle fibers isolated from 50 muscle specimens from 10 children with cardiomyopathy of unknown cause are compared to those from 18 specimens from 5 patients with skeletal muscle myopathies, 45 specimens from 18 patients with congenital heart disease, and 15 specimens from 7 patients with no genetic, chromosomal, or cardiac disease. Muscle fibers from the myopathy specimens show increased nuclei/mm of fiber and increased nuclei/mm/micron of diameter (R value), as well as reduced surface area and volume of cytoplasm per nucleus, compared to control values. The values for cardiomyopathy deviate from normal in the same way as, but to a lesser degree than, those for myopathy--namely, in this material, diseases with cardiomyopathy tend also to produce mild myopathy. Since cardiac and skeletal muscle pathologic findings have not been adequately studied for the majority of the approximately 50 genetic disorders causing cardiomyopathy or otherwise affecting cardiac function described to date, the data indicate primarily that skeletal muscle biopsy will undoubtedly be more useful in cardiomyopathic disorders when the appropriate correlative studies of cardiac and skeletal muscle in such diseases have been done. Because larger biopsy specimens can be obtained, skeletal muscle merits further exploitation in biochemical research on basic mechanisms of disorders causing cardiomyopathy.

Biopsy↗
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Eccrine sweat gland anatomy in cockayne syndrome: a possible diagnostic aid.

Cockayne syndrome is an autosomal recessive disease, which includes as major features motor and mental retardation (beginning in the second year), microcephaly, ataxia, retinal degeneration and pigmentation, cataracts, progeroid features, intracranial calcification, hypogonadism, and growth retardation. Many other diseases have some of these features, so that diagnosis of Cockayne syndrome can be difficult, especially in younger children. Eccrine sweat glands were microdissected from autopsy or biopsy specimens from patients with Cockayne syndrome, and mean values for duct length, secretory coil volume, ratio of coil volume to duct length, and axis ratio of the secretory coil were determined. In comparison with values for eccrine glands of patients with no known genetic or chromosomal disease, eccrine glands in Cockayne syndrome are abnormally small for age. Whether other diseases with various similarities to Cockayne syndrome produce similar growth abnormality of eccrine sweat glands is not known, but determination of sweat gland size may provide data suggesting or supporting the diagnosis of Cockayne syndrome.

Child↗
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Papillary pore counts: a method of studying developmental aberrations in diseased juvenile kidneys.

Counts of the number of pores of primary collecting tubules (ducts of Bellini) on renal papillae, and values calculated by adjusting the counts of compound papillae to those of "virtual" single papillae, were determined for kidneys of patients with end-stage renal failure. Values for chronic glomerulonephritis, Alport's disease, infantile polycystic disease, trisomy 18, and trisomy 13 were not abnormal. Kidneys of patients with CUTO showed significantly low pore counts, indicating that this process in some cases is a true hypoplasia, with mean reduction of number of ducts of Bellini of 26%. FGS showed a high proportion of single papillae (80% vs. normal 60%) with high virtual pore counts, suggesting that a developmental abnormality underlies this disorder (or this outcome of nephrotic syndrome). Cystinosis showed a high proportion of compound papillae (80% vs. 40%) but low virtual pore counts, implying that this genetic disorder causes both maldevelopment and postnatal functional abnormality of the kidneys. A Jeune syndrome kidney produced very low pore counts (mean 8 vs. 16.6 for virtual pore counts), and Down's syndrome also showed low pore counts (mean VPC 15.1 vs. normal 16.6), indicating that the low kidney weights demonstrated by others with Down's syndrome reflect a true hypoplasia.

Child↗
PubMedDate not supplied

Postnatal development of the cystic lung lesion of Down syndrome: suggestion that the cause is reduced formation of peripheral air spaces.

The incidence of small subpleural lung cysts was analyzed for autopsies of 98 patients with Down syndrome (DS): 9 fetuses or stillborns and 89 liveborns. Such cysts were not seen in any of the fetuses or stillborn infants; they were identified in 18 (20%) liveborn DS patients but were present in only 1 of 11 DS patients who survived less than 4 weeks. Such cysts were not found in lungs of 61 non-Down patients with ostium atrioventricular commune (AVC), the most frequent congenital cardiac lesion of DS, or in those of 60 non-DS patients with bronchopulmonary dysplasia. Although subpleural lung cysts were more frequent in DS patients with than in those without congenital heart disease, the difference in incidence of such cysts between 25 liveborn DS patients with AVC and 61 non-DS patients with AVC was highly significant (chi 2 - 19.46, P less than .001). The data suggest that the lung cysts in DS are an intrinsic, albeit not invariant, feature of the disease and not necessarily secondary to congenital heart disease or sequelae thereof. We suggest that they may result from reduced postnatal production of peripheral small air passages and alveoli, which in turn may reflect the slow rate of cell proliferation that appears to be a general feature of DS.

Age Factors↗
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The urinary system in Down syndrome: a study of 124 autopsy cases.

We examined 124 autopsy cases of Down syndrome for the presence of renal and urinary tract abnormalities. The cases were divided into three groups: (I) fetuses of 16-22 weeks gestation (n = 18), (II) stillborns or newborns who died on the first day of life (n = 9), and (III) Down patients 1 day to 25 years of age (n = 97). Kidney weight was reduced by a mean of 14.4% compared with expected values. Renal hypoplasia, defined as kidney weight less than two-thirds expected, was found in 18 cases. Glomerular microcysts were found in 23 of 97 cases in group III. Focal dilatation of tubules was found in 10, simple cysts in 7, and immature glomeruli deep in the renal cortex in 18 cases. Obstructive uropathy occurred in 2 of 18 (11.1%) in group I, 2 of 9 (22.2%) in group II, and 4 of 97 (4.1%) in group III. Obstructive uropathy with bilateral cystic dysplastic kidneys resulted in Potter's sequence. We suggest that obstructive uropathy is associated with Down syndrome. When severe, it results in Potter's sequence and an early perinatal death. A chromosomal analysis is recommended in any case of obstructive uropathy in the fetal or neonatal period.

Adolescent↗
PubMedDate not supplied

Microdissection study of the myenteric plexus in acardia, ataxia-telangiectasia, cystic fibrosis, extrahepatic biliary atresia, pediatric AIDS and Werdnig-Hoffmann disease.

Microdissection-point count morphometric study of the myenteric (Auerbach) plexus or esophagus, small intestine, and colon was done for infants and children with acardia (2), ataxia-telangiectasia (5), cystic fibrosis of the pancreas (CFP) (25), extrahepatic biliary atresia (EBA) (17), pediatric AIDS (10), and Werdnig-Hoffmann disease (WHD) (8). Values for fractional area of neural tissue in the plane of the plexus were compared to those of control patients in same age range as those in each disease category by t-test. Statistically abnormal values included low values for small intestine and colon in Werdnig-Hoffmann disease, high values for small intestine and colon in biliary atresia, and high value for colon but a low value for small intestine in cystic fibrosis. Values for all three loci were within the normal range for ataxia telangiectasia and pediatric AIDS. The mechanisms of the low value for small and large intestines in WHD, which causes chronic constipation as a result of skeletal muscle weakness, and of the high values for colon in CFP and EBA, both causing malabsorption with bulky stools, are unclear. The value for small intestine in acardia was normal for term but lower than expected for fetal bowel of the same size, possibly because of reduced neural crest inflow to the fetal bowel.

Acquired Immunodeficiency Syndrome↗
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Studies of vertebral coronal cleft in rhizomelic chondrodysplasia punctata.

Dissection and serial section-reconstruction preparations from vertebrae with coronal cleft of a 2 5/12-year-old girl with rhizomelic chondrodysplasia punctata are described. The cartilage plate between the dorsal and ventral vertebral ossification centers, abnormal thickness of which is the explanation of the radiologic coronal cleft, shows differences in thickness, with dorsal and ventral midline extensions and with small foci of communication of the cancellous bone of the dorsal and ventral ossification centers of the vertebral body bilaterally. Although the process in coronal cleft is in some ways similar to that of radiologic "retarded epiphyseal closure," the explanation of the coronal clefts in rhizomelic chondrodysplasia punctata appears to be overproduction of cartilage in the zone between the dorsal and ventral vertebral body ossification centers, plus reduced removal of cartilage cells from this zone. No evidences of persistence of notochord cells were seen microscopically in sections of the coronal clefts.

Child, Preschool↗
PubMedDate not supplied

Association of Down syndrome and segmental tracheal stenosis with ring tracheal cartilages: a review of nine cases.

Four patients with Down syndrome and midtracheal stenosis, three with proven absence of the midtracheal pars membranacea ("hourglass trachea"), are reported. Five previously reported patients who had Down syndrome and tracheal stenosis of this type are summarized. Respiratory difficulty and stridor were the reported clinical features of all but one of the patients whose clinical story is available. That approximately half the patients with tracheal stenosis with hourglass trachea and midtracheal absence of the tracheal pars membranacea reported had Down syndrome suggests that the association of this pattern of congenital tracheal stenosis with Down syndrome is, although infrequent, significant.

Abnormalities, Multiple↗
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Syndromal associations of common origin of the carotid arteries.

The term "common origin of the carotid arteries" (COCA) has been proposed to replace the older terms "origin of the left carotid artery from the innominate stem" and "bicarotid trunk with anomalous right or left subclavian artery." These anatomic patterns are usually reported to occur in about 11% of whites and 20-25% of blacks and have been reported to have increased frequency in patients with esophageal atresia-tracheoesophageal fistula, DiGeorge anomaly, and anomalous origin of the left coronary artery from the pulmonary artery. COCA is a significant, if not invariant, feature of the great arteries in the condition usually called in the more recent literature "anomalous origin of the innominate artery," the most frequent cause of symptomatic tracheal compression by anomalous systemic arteries. Analysis of associations of COCA with various other congenital cardiovascular lesions showed, in addition, significant association with congenital polyvalvular disease, truncus arteriosus, aorticopulmonary window, trisomy 13, 18, and 21 syndromes, acrocephalosyndactyly (especially Apert syndrome), tetralogy of Fallot not associated with DiGeorge anomaly, and clinical Noonan phenotype. Pentalogy of Cantrell was associated with no increase in incidence of COCA.

Carotid Artery, Common↗
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Branching patterns of right pulmonary artery in cardiovascular anomalies.

The branch of the right pulmonary artery (RPA) to the upper lobe of the right lung (RUL), the truncus anterior of the RPA, and the pars anterior of the left pulmonary artery, which supplies the left upper lung lobe (LUL), were demonstrated by both dissection of postmortem specimens and angiography for 20 infants and children, by angiography only for 57, and by specimen dissection only for 59 (total 136). In posteroanterior angiograms, the RUL artery branches from the RPA near the right lateral border of the vertebral column, while the LUL artery or arteries arise more laterally, near the left midclavicular plane. This pattern is reversed in situs inversus (eight patients studied). Thirty-nine patients in other categories of congenital cardiovascular disease showed an abnormal RUL or LUL arterial pattern, including pulmonary isomerism, right lung type (RUL artery pattern present bilaterally, 12 patients); pulmonary isomerism, left lung type (RUL artery pattern absent bilaterally, 11 patients); scimitar syndrome (RUL artery pattern normal, 1 patient; absent bilaterally, 4 patients); and left pulmonary artery sling (RUL artery normal, one; hypoplastic, one; absent, two patients). Five patients with tetralogy of Fallot (TOF) with right aortic arch (RAA) and 1 of 15 with RAA not TOF or situs inversus showed a relatively large RUL artery arising more laterally than usual. Three of six patients with double outlet right ventricle had the LUL artery larger than usual plus two accessory RUL arteries, and one patient with crossed pulmonary arteries showed a similar pattern. Two patients with single ventricle had an RUL artery of normal pattern although the RUL bronchus was absent, and one patient with single ventricle and situs inversus had a comparable pattern in the left lung. The ease of demonstration of the right and left upper lobe branches of the pulmonary artery by dissection or angiocardiography warrants greater attention to their patterns in patients with congenital cardiovascular disease. Dissociation of upper lobe bronchial and vascular patterns is unusual and may also be of diagnostic value.

Angiocardiography↗
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Labeled lectin studies of renal tubular dysgenesis and renal tubular atrophy of postnatal renal ischemia and end-stage kidney disease.

Renal tubular dysgenesis (RTD), with hypoplasia especially of renal proximal convoluted tubules and clinical neonatal anuria or oliguria, has been reported as a congenital familial (autosomal recessive) disease, variably with features of oligohydramnios, Potter syndrome, or pulmonary hypoplasia. A similar tubular lesion due to antenatal tubular atrophy has been reported for conjoined twins with twin-twin transfusion syndrome or acardia and in infants of mothers given antihypertensive agents, including angiotensin-converting enzyme (ACE) inhibitors, during pregnancy, and it has been seen as a unilateral lesion in young infants with renal artery stenosis due to arteritis or medial arterial calcinosis. The renal tubular changes in RTD are very like those of the "endocrine kidney" in experimental animals and resemble those of the renal tubular atrophy of end-stage kidney diseases such as glomerulonephritis, tubulointerstitial kidney disease, obstructive uropathy/pyelonephritis, graft rejection of transplanted kidneys, or the renal parenchymal changes seen with protracted dialysis therapy. Labeled lectins that differentially mark proximal convoluted, distal convoluted and connecting, and collecting tubules showed no distinctive differences in staining patterns of the hypoplastic renal tubules of infants and children with RTD, postnatal renal artery obstruction, or the various types of end-stage renal disease with the lectins used (PNA, GSLI, UEA, and LTA). The findings suggest that the renal tubular changes in some if not all the conditions studied are the result of renal ischemia. The reported familial RTD with hypernephronic nephromegaly may be a specific disorder, but other forms could reflect renal ischemia acquired in utero or in early or later postnatal life.

Adolescent↗