Biomedical subjects

B H Landing

Publications and source records attributed to B H Landing.

At least 55 records · Page 3Linked to original sources

PubMed1986

Abnormal Auerbach plexus in the esophagus and stomach of patients with esophageal atresia and tracheoesophageal fistula.

The Auerbach plexus of the upper gastrointestinal tract of five patients with esophageal atresia and tracheoesophageal fistula (EA-TEF) who had not had surgical reconstruction of the esophagus was studied by a microdissection technique to investigate the etiology of dysphagia, gastroesophageal reflux, and delayed gastric emptying; common findings in patients with repaired EA-TEF. All five patients showed a looser than normal Auerbach plexus in the distal esophagus, and to a lesser degree in the proximal esophagus, confirmed by point-count morphometric studies. The Auerbach plexus of the gastric fundus of all the patients was also abnormal. These findings suggest the existence of congenital functional impairment of the upper gastrointestinal tract in patients with EA-TEF, due to abnormal development of the myenteric plexus.

Esophageal Atresia↗

PubMed1986

Abnormal tracheal innervation in patients with esophageal atresia and tracheoesophageal fistula: study of the intrinsic tracheal nerve plexuses by a microdissection technique.

The automatic nervous plexus of the trachea of patients with esophageal atresia and tracheoesophageal fistula (EA-TEF) was examined by a microdissection method. Of 28 autopsied patients with EA-TEF, four patients who had not had esophageal anastomosis were selected, to exclude any changes secondary to anastomosis. The plexus of the pars membranacea of the trachea was hypoplastic in all four patients, and severely so in one. An extra plexus, similar to the myenteric plexus of the esophagus, was present in three of the patients, and an abnormal muscle bundle was seen in the fibroelastic tissue dorsal to the pars membranacea of the trachea in two patients. These findings are suggestive of abnormal separation of the trachea from the esophagus. An abnormal extrachondral plexus was also observed in two patients. These congenital abnormalities of the intrinsic tracheal nervous plexus may be related to tracheal dysfunction and respiratory symptoms in patients who survive EA-TEF.

Adult↗

PubMed1986

Pulmonary calcinosis after renal transplantation in pediatric patients.

Pulmonary calcinosis is a recognized complication of renal failure. The resulting pulmonary compromise may be severe or even fatal. The potential contribution of hypercalcemia, hyperphosphatemia, and increased calcium-phosphorus product to the development of pulmonary calcinosis has been controversial. We describe four patients (ages 2 1/4 to 18 years) who had severe pulmonary calcinosis and respiratory failure within three to five days after renal transplantation. Initial clinical and roentgenographic findings suggested noncardiogenic pulmonary edema. Marked pulmonary hypertension was present in the two patients in whom pulmonary artery pressure data were available. Other clinical features in common included poor allograft function with persistent uremia requiring dialysis and evidence of moderate to severe secondary hyperparathyroidism. In three of the patients, the calcium-phosphorus product increased markedly after transplantation, to peak values of 122 to 147. This increase occurred at the same time as the onset of respiratory failure. Peak serum calcium levels were 10.0 to 11.0 mg/dL and peak serum phosphorus levels were 9.2 to 13.5 mg/dL. All patients died of respiratory failure five to 58 days after transplantation. The posttransplantation period may be a time of increased risk of potentially fatal pulmonary calcinosis in pediatric renal transplant recipients. The diagnosis should be considered in any patient with respiratory failure of unknown cause following renal transplantation.

Adolescent↗

PubMed1986

Ossification centre of the hyoid bone in DiGeorge syndrome and tetralogy of Fallot.

The incidence of radiographic visibility of the ossification centre of the body of the hyoid bone in radiographs taken during the first month of life was analysed for 34 autopsied infants: 16 with DiGeorge syndrome (DGS), 14 with tetralogy of Fallot (TOF), four with interrupted aortic arch (IAA) and a further 13, surviving infants with non-DGS TOF or non-DGS IAA. The incidence of visible hyoid ossification centre (HOC) was 75.7% in a control series of infants with neither congenital heart disease (CHD) nor DGS. Autopsied patients with DGS, TOF without DGS, and IAA without DGS showed a significantly low incidence of visible HOC. Infants with TOF (and possibly those with IAA) who did not have DGS and who did not die during infancy showed a normal incidence of visible HOC in radiographs taken during the first post-natal month. Radiological visibility of the HOC in the first post-natal month appears useful in the diagnosis of DGS and forms of CHD often seen in association with DGS and in assessing prognosis of neonates with certain types of CHD.

Age Factors↗

PubMed1986

Ossification centre of the hyoid bone in complete transposition of great vessels, Ivemark asplenia syndrome, and Down's syndrome with congenital heart disease: correlation with the humeral capital epiphysis.

The incidence of radiographic visibility of the ossification centres of the body of the hyoid bone and of the humeral capital epiphysis in antero-posterior or lateral chest radiographs taken during the first month of life of 63 autopsied infants were analysed. The group comprised patients with Down's syndrome (DS) with congenital heart disease, 15; complete transposition of the great vessels (TGV), 10; Ivemark asplenia syndrome (IS), 17; and a control group of infants with congenital heart disease (CHD) who had none of the above conditions, nor tetralogy of Fallot, interrupted aortic arch, DiGeorge syndrome or hypoplastic left-heart complex, 31. The incidence of radiographically visible hyoid ossification centre (HOC) in the control group was 71% and of humeral capital epiphysis (HE), 16.1%. Autopsied infants with TGV, IS or DS with CHD showed increased visibility of HOC (100%); the incidence of visible HE was increased in neonates with IS (71.4%) and with TGV (50%). The differences in the incidence of radiographic visibility of HOC and HE in neonates with CHD, in this study and in others in the literature, appear to have diagnostic value.

Age Factors↗

PubMed1986

Pancreatic islets in older patients with cystic fibrosis with and without diabetes mellitus: morphometric and immunocytologic studies.

Forty patients with cystic fibrosis (CF), including 34 who died above age 10 years without having developed clinical diabetes mellitus and 6 who died with both cystic fibrosis and diabetes mellitus, were studied. The mean age of the female patients with CF but not diabetes was 15.8 +/- 5.6 years; of males without diabetes, 17.2 +/- 6.4 years; of female patients with CF and diabetes mellitus, 20.2 +/- 6.9 years; and of males with CF and diabetes, 21.3 +/- 6.6 years. The mean number of pancreatic islets in microscopic sections for patients with cystic fibrosis but not diabetes was 4.18 +/- 2.76/mm2, and the value for patients with both cystic fibrosis and diabetes mellitus was 2.61 +/- 2.07/mm2. The lowest density of pancreatic islets (1.69 +/- 0.48/mm2) for cystic fibrosis was found in patients with the latest-stage pathologic lesion. Nesidioblastosis (presence of ductuloinsular complexes) was identified in 14 of 38 cystic fibrosis patients, both with and without diabetes mellitus. The pancreatic islets of both diabetic and nondiabetic patients with CF showed hypertrophy; the mean volume of the three largest pancreatic islets for CF only was 0.0117 +/- 0.00657 mm3 and that for cystic fibrosis and diabetes was 0.00795 +/- 0.00599 mm3, both values being larger than normal. Ratios of the amounts of islet endocrine cells, A cells, B cells, and D cells, were determined by peroxidase--anti-peroxidase labeled antibody staining. The B cells composed 43.0% of endocrine cell mass in cystic fibrosis alone and 30.1% in cystic fibrosis with diabetes mellitus, which were lower than normal proportions. The D cell values, 11.9% in cystic fibrosis and 15.1% in cystic fibrosis with diabetes mellitus, on the other hand, were greater than normal ratios.

Adolescent↗

PubMed1986

Increased cardiac muscle fiber size and reduced cell number in Down syndrome: heart muscle cell number in Down syndrome.

The size of cardiac muscle fibers was determined for 15 patients with Down syndrome who did not have congenital heart disease and for 15 age- and sex-matched control patients by counting the number of cardiac muscle cells in specified areas of microscopic sections (method of Black-Schaffer and Turner). Mean ratio of muscle cells per unit area for the Down patients versus controls was 84.9%, with mean cross-sectional area of Down fibers 117.8% of control value and calculated mean volume of Down cells 127.7% of control value. Mean weights of Down hearts related to normal for age, versus control hearts, was 79.8% of control values. Calculated ratio of total cardiac muscle cell number in Down hearts was 62.5% of the value for controls. This value corresponds closely to the 62.4%, which can be calculated from Naeye's data on cardiac muscle fiber size in Down syndrome based on a point-count method, and is also close to published values for liver cell size and number and skeletal muscle nuclear number in Down syndrome. This study demonstrates that the Black-Schaffer and Turner method gives results equivalent to those of the point-count method for studies of cardiac muscle fiber size, and further supports the suggestion that chromosome 21 controls the size and number of at least certain cell types.

Cardiomegaly↗

PubMed1986

Infantile systemic hyalinosis: report of four cases of a disease, fatal in infancy, apparently different from juvenile systemic hyalinosis.

Four female Mexican-American infants, two siblings, had widespread deposit of hyaline material in skin, gastrointestinal tract, adrenals, urinary bladder, ovaries, skeletal muscles, thymus, parathyroids, and other loci. Clinical features included thickness and focal nodularity of skin, relatively short limbs and neck, gum hypertrophy, hypotonia and reduced movement, joint contractures, osteoporosis, growth failure, diarrhea, and recurrent infections. Clinical onset was in the first week, and all 4 patients died by age 20 months. Infantile systemic hyalinosis appears to be a specific, presumably autosomal recessive, genetic disease, differing from the disorder called systemic hyalinosis, juvenile hyaline fibromatosis, or Puretic syndrome. The biochemical defect and the pathogenetic mechanisms responsible for the pathologic and clinical features of this condition remain to be established.

Chromosome Aberrations↗

PubMed1986

Abnormal growth of the thyroid cartilage in the DiGeorge syndrome.

Larynges from 17 patients with DiGeorge syndrome (DGS) and from 14 patients with tetralogy of Fallot (TOF) (11 non-DGS and 3 possible but unproven DGS) were dissected, measured, and compared to a control population of comparable body length. The patients with DGS and the 3 patients with TOF suspected of having DGS showed the following: small thyroid cartilages with increased anterior angle, abnormally short superior cornua, low ratio of mean superior cornual length to distance between superior cornual tips, and delayed time of maximal rate of increase in superior cornual length relative to increase in body length. The hypoplasia, delayed maximal growth rate and persistent fetal shape of the thyroid cartilage (predominantly a derivative of the fourth branchial arch) in DGS, indicates that the causative process in the syndrome affects not only the third and fourth branchial pouches, but also, by a contiguous field defect, other derivatives than great vessels of the fourth-sixth branchial arches.

DiGeorge Syndrome↗

PubMed1986

Intestinal plexuses in Crohn's disease and ulcerative colitis in children: pathologic and microdissection studies.

The relative fraction of neural tissue in the plane of the myenteric (Auerbach) plexus (MEP) of ileum and colon of patients with Crohn's disease and ulcerative colitis was analyzed by point-count morphometric analysis of microdissected flat-mount ("circuit diagram") preparations of the plexus. Increase in the relative fraction of neural tissue (myenteric plexus hyperplasia) was seen in the MEP of some patients in both ileum and colon in both diseases. The frequency of ulceration or inflammation extending into the bowel wall deep enough to injure the various levels of the intestinal plexus system (mucosal plexus of Cajal; upper submucosal plexus of Meissner; deeper submucosal plexus of Henle; intermuscular plexus of Auerbach; outer muscular/serosal plexus of Stöhr) was analyzed for small intestine, appendix, and colon for patients with both diseases. As expected, Crohn's disease tends to produce deeper intestinal wall damage in ileum versus colon, and ulcerative colitis the reverse. The effect of such injury on local and more distal motor control of the intestine and on integrative functions of the intestinal plexus system appears not to have been considered previously. Since injury deep enough to damage any given plexus necessarily damages all overlying plexus levels, analysis of such effects appears a fruitful field for study.

Adolescent↗

PubMed1986

Osteoporosis in juvenile-onset diabetes mellitus: morphometric and comparative studies.

Ribs and vertebrae of 8 children and young adults aged from 17 months to 24 years with juvenile-onset diabetes mellitus, 4 with diabetes secondary to cystic fibrosis and 2 with diabetes secondary to thalassemia major, were analyzed for osteoporosis by a point-count morphometric method. The mean ratio of bone spicule to marrow space in cancellous bone of ribs of patients with juvenile-onset diabetes mellitus or with diabetes mellitus secondary to cystic fibrosis or thalassemia was 55% that of 10 control patients. The lengths of the zones of proliferating and mature cartilage cells in costal epiphyses of patients with juvenile-onset diabetes mellitus were also below normal. The ratio of bone spicule to marrow space of vertebrae of the diabetic patients was not significantly different from control values. The data confirm clinical reports that osteoporosis is a regular feature of juvenile-onset diabetes mellitus and suggest that the degree of bone matrix and mineral deficiency in such patients is greater than is usually considered.

Adolescent↗

PubMed1986

Microdissection study of the incidence of branched eccrine sweat glands and the number of eccrine glands per unit area of infants' and childrens' skin.

That branched eccrine sweat glands occur has rarely been mentioned in the literature, and their incidence has never been determined. All eccrine glands in 0.5-cm2 pieces of anterior trunk skin of 59 children were microdissected, and the total number of glands and the number of branched eccrine glands determined. One-hundred fourteen of 17,539 (0.65%) were branched, 90% in the middermis and 10% at the epidermis. The apparently normal anatomic property that almost 1% of eccrine sweat glands are branched has not hitherto been appreciated. One doubly branched gland was found. Patients with leukemia (11 in the study) possibly had more branched glands than nonleukemic patients of the same body size. The best statistical relation of the number of sweat glands per unit area of skin (GUA) to surface area (SA) or age in children was the natural logarithm of GUA versus the reciprocal of surface area: LnGUA = (0.2205 X 1/SA) + 5.42. This result is consistent with the classical proposition that there is no important degree of formation of new eccrine glands, nor of loss, after birth, the density of glands per unit area of skin reducing as SA rises with growth during childhood.

Child↗

PubMed1986

Pulmonary alveolar septal calcinosis causing progressive respiratory failure in acute lymphoblastic leukemia in childhood.

A syndrome of pulmonary alveolar septal calcinosis, pneumothorax, and pneumomediastinum, leading to rapidly progressive acute respiratory insufficiency and death was observed in 2 children with acute lymphoblastic leukemia (ALL). Primary clinical and radiological considerations in these patients were pulmonary edema and infection, and the diagnosis of pulmonary alveolar septal calcification was established only at autopsy. One patient, a 15-year-old girl, was found also to have parathyroid hyperplasia typical of familial hyperparathyroidism. The other, a 16-month-old girl, showed osteitis fibrosa of the bones and parathyroid hyperplasia of secondary type, suggesting that the pulmonary calcinosis resulted from hypercalcemia caused by a parathormone or prostaglandin-secreting tumor. The cause of pneumothorax and pneumomediastinum may have been rupture of calcified alveolar septa induced by high PEEP during ventilation of these patients. Other possible mechanisms contributing to hypercalcemia and pulmonary calcinosis in children with acute leukemia include bone resorption due to marrow infiltration, immobilization syndrome, renal failure, and administration of calcium, phosphate, or bicarbonate. This complication of acute leukemia in childhood is rare (2 patients in 430 autopsied over the period 1961-1982 at Childrens Hospital of Los Angeles). How often the process can be reversed if diagnosed before severe respiratory insufficiency is present is not known.

Adolescent↗

PubMed1986

Microdissection demonstration of the lesion of the endocrine kidney in children.

Microdissection of nephrons of kidneys of children showing advanced ischemic tubular atrophy, and removed for control of hypertension, demonstrates marked proximal convoluted tubular atrophy, with formation of multiple small proximal tubular diverticula. These diverticula presumably contribute to the microscopic appearance of large numbers of small tubules lined by low epithelial cells with pale or clear cytoplasm, adjacent to glomeruli in the cortices of kidneys showing ischemic tubular atrophy (endocrine kidney). Segmentation of such atrophic tubules leads to formation of blind segments (microcysts), as demonstrated in this study and by Oliver. The distinctive microscopic appearance of the endocrine kidney, a not infrequent finding in kidneys of children with chronic renal insufficiency who require nephrectomy for control of hypertension, has not hitherto been emphasized in the literature on pediatric renal disease.

Adolescent↗

PubMed1986

Reduced number of neurons in esophageal plexus ganglia in Down syndrome: additional evidence for reduced cell number as a basic feature of the disorder.

Four patients with Down syndrome and 3 age-matched control patients were studied by a microdissection technique to investigate the number of neurons in the ganglia of the deep submucous and Auerbach plexuses of the esophagus. The mean number of neurons per ganglion of the deep submucous plexus in Down patients was 69% of the control value, and the value for the Auerbach plexus was 75% of control number. These findings may explain esophageal dysfunction, common in Down syndrome, and support a proposal that a biochemical "signal" relating to chromosome 21 determines the number and sizes of certain cell types.

Cell Count↗

PubMed1985

Sacrococcygeal teratomas in infants and children.

From 1941 through 1983, a total of 66 patients with sacrococcygeal teratoma were seen, representing 41 percent of the total of 162 patients with teratomas from all anatomic sites seen over this period. Forty-six (70 percent) of the patients with sacrococcygeal teratomas were female and 34 (52 percent) were neonates. Younger patients had a significantly better prognosis. Approximately half (48 percent) of the patients had benign tumors. Of the other 34 patients, 19 (29 percent) had frank malignancy, being either a purely malignant tumor or a tumor with malignant mixed with benign elements, and 15 (23 percent) patients had tumors containing embryonic but no malignant components. Patients with tumors containing mature adult or embryonic tissues have a good prognosis. However, malignant sacrococcygeal teratoma portends a bleak prognosis irrespective of therapy (in this series only one patient survived among 19 patients with an average survival of 16 months after diagnosis).

Antineoplastic Combined Chemotherapy Protocols↗

PubMed1985

Association of common origin of the carotid arteries with anomalous origin of the left coronary artery from the pulmonary artery.

Common origin of the carotid arteries (COCA) is a common pattern of aortic arch vessels and is the single most common cause of tracheobronchial compression by a congenital cardiovascular anomaly. By no means all affected patients are symptomatic. Symptoms may range from recurrent pulmonary infections and "noisy respiration" to stridor and apneic spells. In our study of patients with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) we found a highly significant association of COCA with ALCAPA (85%), although no patient with ALCAPA in this study had evidence of tracheal stenosis documented in the hospital chart. As COCA is easily correctable, we suggest consideration of COCA during evaluation and surgery of patients with ALCAPA so that, if the patient also has symptoms possibly related to COCA, the artery can be suspended from the posterior wall of the sternum.

Aorta, Thoracic↗

PubMed1985

Short trachea, with reduced number of cartilage rings--a hitherto unrecognized feature of DiGeorge syndrome.

Adequate tracheas from 6 of 14 patients with DiGeorge syndrome (2 complete and 4 partial forms) were available for tracheal ring study. The number of tracheal rings in these patients was significantly low (mean +/- SD 14.7 +/- 1.4). This is consistent with an effect of deficient blood supply to the fetal cervical region, a mechanism that has been proposed to explain the defective development of thymus and parathyroid glands in DiGeorge syndrome. To our knowledge this lesion has not been described as one of the malformations found in the syndrome. In 8 of 14 (57%) patients with DiGeorge syndrome, common origin of the carotid arteries was found, and 4 of these 8 patients showed minor tracheal compression. These findings support the proposal that abnormal blood supply to the region of the third and fourth pharyngeal arches may play a role in the pathogenesis of DiGeorge syndrome.

Adolescent↗