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Biomedical subjects

B H Landing

Publications and source records attributed to B H Landing.

At least 37 records · Page 2Linked to original sources
PubMed1989

Infantile hepatic hemangiomas. Clinical features, radiologic investigations, and treatment of 20 patients.

The clinical features, radiologic investigation, and treatment of 20 infants with hepatic hemangiomas are presented. Palpable abdominal mass (n = 18) and cardiac failure (n = 11) were the common presenting features. Nine patients had hyperconsumptive coagulopathy. Seven patients had other hemangiomas. Ultrasound (n = 15) showed the number and distribution of the hemangiomas within the liver. Hypoechoic and hyperechoic elements were present in addition to prominent vascular channels and diminished caliber of the distal aorta. Radionuclide sulfur colloid (n = 12) and labeled red blood cell (n = 7) studies showed the distribution and vascularity of the hemangiomas. Computed tomography (n = 8) revealed central hypointensity with marked peripheral enhancement after contrast. Arteriography now performed only as a prelude to therapeutic embolization demonstrated hypervascularity in each patient, contrast pooling in six and early draining veins in five. Magnetic resonance scanning (n = 3) showed decreased signal intensity on T1 images and high intensity signal on T2. In two patients, there was resolution or improvement of the hemangiomas without therapy. Four patients had surgery (lobectomy [2], trisegmentectomy [1], and surgical evacuation of a central hematoma [1]). Steroids and radiation were given to seven patients, and one patient also required therapeutic embolization. Steroids were the initial therapy in five patients, one of whom later required therapeutic embolization and another cyclophosphamide. Two patients were treated initially with radiation therapy, one of whom also needed emergency hepatic artery ligation. Seventeen of the 20 patients are alive and well from 6 months to 14 years after diagnosis.

Angiography↗
PubMed1989

Immature teratomas: identification of patients at risk for malignant recurrence.

We investigated the significance of immature elements in an otherwise benign teratoma in 28 patients with immature teratomas diagnosed and treated at the Childrens Hospital of Los Angeles from 1941 to 1986. Different characteristics, including age, sex, primary tumor site, type of surgery (complete resection vs. partial resection or biopsy), and preoperative levels of alpha-fetoprotein (AFP) were analyzed to evaluate their association with risk of subsequent local malignant recurrence. After a median follow-up of 6 years, 21 patients are alive with no recurrence of the tumor (72% event-free survival). One patient died from infection after surgery and six patients had local malignant tumor recurrence within 1 year from diagnosis. Of the 28 patients, 12 had AFP levels measured at diagnosis. Eight patients had normal levels with no further evidence of tumor recurrence, and four had elevated levels with three tumor recurrences. Our experience demonstrates that only at the time of diagnosis do AFP levels correlate with a subsequent malignant behavior of these tumors (P = .004). Those patients with immature teratomas and elevated AFP levels at diagnosis should receive adjuvant chemotherapy after the initial surgical resection.

Adolescent↗
PubMed1989

Short trachea, a hazard in tracheal intubation of neonates and infants: syndromal associations.

Short trachea results from reduction in number of tracheal cartilage rings to 15 or fewer from normal mean of 17 rings in infants. In a review of radiologic and pathologic data, the thoracic vertebral level of tracheal bifurcation as seen in anteroposterior chest radiographs of infants with congenital malformations, cardiovascular anomalies, and skeletal dysplasias, was compared with numbers of tracheal cartilage rings demonstrated in postmortem specimens. Increased frequency of short trachea was seen in patients with DiGeorge anomaly (77%), skeletal dysplasias (55%), brevicollis (57%), diaplacental rubella (40%), and patients with congenital heart disease who did not have DiGeorge anomaly (36%, with range 25-83% for different types, the highest, 83%, being interrupted aortic arch). Preintubation high kilovoltage chest radiographs to establish the level of tracheal bifurcation in patients with increased risk of short trachea can be helpful in avoiding bronchial intubation and its complications. Postintubation chest films to assure the level of the endotracheal tube tip should be considered for such patients. Growth in length of the trachea with age is accomplished both by increase in size of tracheal cartilage rings and interring membranes, and by increase in ring number.

Bronchi↗
PubMed1989

Renal lesions and clinical findings in thalassemia major and other chronic anemias with hemosiderosis.

Renal lesions found in 21 autopsied patients with hemosiderosis, 18 with beta-thalassemia, two with Blackfan-Diamond anemia, and one with aplastic anemia included: cellular glomeruli with increased mesangial matrix; hemosiderin deposit in visceral and parietal glomerular epithelial cells; greater hemosiderin deposit in terminal straight portions of proximal convoluted tubules and distal convoluted tubules than in connecting segments, or collecting tubules, connective tissue ferrugination; lipofuscin in tubular epithelium and vascular smooth muscle; infrequently, intimal or medial arterial thickening, and, in one patient with thalassemia, an infarct resulting from arterial thrombus. The progression of these lesions over the course of disease, and possible effects on the various lesions of high transfusion regimen, oral pancreatin, vitamin E supplementation, or treatment with intramuscular, subcutaneous, or intravenous desferrioxamine were evaluated. The results of urine and renal function studies of 4 of the autopsied patients (3 thalassemia, 1 Blackfan-Diamond anemia), and 14 patients with thalassemia and 4 with Blackfan-Diamond anemia who were not autopsied, are presented. Rarely significant until preterminal stages, the renal functional changes reflect distal more than proximal tubule dysfunction.

Adolescent↗
PubMed1988

Reconsideration of the anatomy of sling left pulmonary artery: the association of one form with bridging bronchus and imperforate anus. Anatomic and diagnostic aspects.

In sling (retrotracheal) left pulmonary artery (SLPA), the tracheobronchial pattern is generally considered basically normal. Analysis of dissected specimens and/or bronchograms and other preparations from five studied and 32 reported patients suggests that there are two different forms of SLPA: (1) types 1A and B, with normal TB pattern [with (A) or without (B) a right pre-eparterial (tracheal bronchus)], and the aberrant left pulmonary artery causing TB compression. The tracheal bifurcation in type 1 SLPA is usually demonstrable at the fourth to fifth thoracic vertebral level; (2A) SLPA type 2A, with bridging bronchus (BB), in which condition the right main bronchus supplies the right upper lobe, but the bronchus supplying the right middle and lower lobes (the bridging bronchus) arises from the left main bronchus (LMB), posterior to which the SLPA courses; (2B) SLPA type 2B, with absence of the right bronchial tree, and the right lung (usually hypoplastic) supplied by a BB from the LMB, posterior to which the SLPA courses. SLPA types 2A and B have in common varying degrees of tracheal stenosis with abnormal cartilage rings and absent tracheal pars membranacea, abnormally low tracheal "bifurcation" (pseudocarina) at average level T6, increased bronchial angles with "inverted T" pattern, and lower level of anterior esophageal indentation by the SLPA than SLPA type 1. Imperforate anus occurred in 8/58 (14%) of patients with SLPA types 2A or B, but possibly in none with SLPA type 1. SLPA type 2 is the predominant form of SLPA, with the incidence of type 2B being twice that of type 2A.(ABSTRACT TRUNCATED AT 250 WORDS)

Abnormalities, Multiple↗
PubMed1987

Embryologic and other developmental considerations of thirty-eight possible variants of the DiGeorge anomaly.

The DiGeorge anomaly (DGA) represents a polytopic developmental field defect that can be caused by a number of different chromosomal, mendelian, toxic, or metabolic factors operating in early embryonic life. If the affected field is thought to be focused on either the fourth branchial arch or the third branchial pouch, with variable cephalad or caudad extension, 38 different combinations of malformations can include DiGeorge anomalies, 24 complete and 14 partial DGA, with the constraints that the field defect must be contiguous (involvement of more than one branchial arch or pouch requires involvement of all intervening arches or pouches) and complete (all derivatives of an affected branchial arch or pouch are deficient). The types and relative frequencies of abnormalities of structures other than the thymus and parathyroid glands in these possible "subsets" of DGA are discussed, and the need for more data on their occurrence in patients diagnosed as having DGA is emphasized.

Branchial Region↗
PubMed1987

Demonstration of myenteric plexus abnormalities in genetic diseases by a microdissection technique: preliminary studies.

Eighty-eight specimens of esophagus, small intestine, or colon from 45 patients, predominantly infants and children, with 30 different genetic diseases were analyzed by a microdissection technique for the following abnormalities of the Auerbach (myenteric) plexus: (1) abnormality of the pattern of the nervous network of the plexus, (2) abnormal fraction of neural tissue in the plane of the plexus, (3) abnormal size or appearance of the cytoplasm of the neurons of the plexus, and (4) abnormal number of neurons in the ganglia of the plexus. Seven of 8 specimens of esophagus from patients with neuronal storage diseases (infantile Niemann-Pick disease, Jansky-Bielschowsky disease, etc.) showed an increased fraction of neural tissue in the plane of the plexus, whereas 2 of 3 patients with Cockayne syndrome showed a reduced fraction, with abnormally slender interganglionic fibers. The fraction of neural tissue in the plane of the plexus was also abnormal at one or more levels in patients with adrenoleukodystrophy, ataxia telangiectasia, Krabbe disease, and juvenile metachromatic leukodystrophy. Abnormality of neuron size and cytology was seen in several neuronal lipidoses, including Jansky-Bielschowsky and Sandhoff diseases and juvenile GM2 gangliosidosis, with the most striking neuronal enlargement noted in infantile Niemann-Pick disease. Abnormalities of plexus mass or pattern, as well as those of neuronal cytoplasm and neuron number, offer improved insight into possible mechanisms producing gastrointestinal tract dysfunction (swallowing difficulty, gastroesophageal reflux, constipation, etc) in patients with genetic disorders.

Child↗
PubMed1987

Multicentric osteolysis: report of the second successful renal transplant.

A patient with end stage renal disease due to sporadic idiopathic multicentric osteolysis (type 3 multicentric osteolysis) is described. His pre-transplant course was similar to those of previously described patients, while his post-transplant course has been uncomplicated. The pathology and pathogenesis of the nephropathy of sporadic idiopathic multicentric osteolysis is not well characterized. The short term outcome of renal transplantation is excellent in our patient and in the other similar case known to have been transplanted.

Adolescent↗
PubMed1987

Pulmonary lesions in childhood onset systemic lupus erythematosus: analysis of 26 cases, and summary of literature.

The pathological findings in the lungs and related organs of 26 patients (21 female, 5 male) with systemic lupus erythematosus (SLE), with onset of disease before age 20 years, were reviewed. Several categories of lung lesions were found. Chronic interstitial pneumonitis was present in all 26 patients and was severe in 5. Acute pneumonia was present in 20, mild in 13, moderate in 2, and severe in 5. Alveolar hemorrhage, massive enough to cause death in 5, was seen in 18 patients, and pulmonary edema was found in 13. Fourteen patients had hyaline membranes indicative of acute alveolar damage (DAD), 12 had alveolitis obliterans, indicative of prior episodes of DAD, and 9 had bronchiolitis obliterans. Other parenchymal lesions were mild interstitial fibrosis in 12, alveolar hemosiderosis and alveolar overinflation in 10 each, and alveolar septal calcinosis with chronic renal insufficiency in 3. Pleural effusion, pleuritis, or pleural thickening were noted in 15 of 26, 6 of 23, and 7 of 23 evaluable patients, respectively. Vascular lesions were present in 16 as intimal thickening (9), thromboemboli (8), medial hypertrophy (6), calcinosis (3), and vasculitis (2). A previously unreported lesion was chronic (proliferative) peribronchitis, noted in 11 patients. Diaphragmatic lesions included mild variation in fiber size in 7, mild fibrosis in 2, and calcinosis in 1 of 13 evaluable patients. Correlation of the above lesions with previously described lung syndromes in SLE such as lupus pneumonitis, hemorrhagic lung disease, chronic interstitial fibrosis, lupus cor pulmonale, pleurisy, and "shrinking lung syndrome" are discussed.

Adolescent↗
PubMed1987

Pathologic features of gastrointestinal tract lesions in childhood-onset systemic lupus erythematosus: study of 26 patients, with review of the literature.

A review of the gastrointestinal tracts of 26 autopsied cases of childhood-onset systemic lupus erythematosis (SLE) showed the single most common pathologic finding to be chronic nonspecific mucosal infiltration (96%). The most frequent general category was ischemic bowel lesions, attributable to vascular lesions of SLE (60%) or to nonocclusive causes of circulatory insufficiency such as congestive heart failure, uremia, or shock (40%). Complications of ischemic bowel lesions included secondary invasion by opportunistic organisms such as Candida (9 patients), pneumatosis intestinalis (1), and perforation of a gastroesophageal ulcer (1). Colonic ulcers, clearly ischemic in etiology, were noted in 6 patients, none of whom had other bowel lesions resembling those of Crohn's disease or ulcerative colitis. Other relevant findings included ascites (88%), peritoneal inflammation or fibrosis (42%), upper esophageal skeletal muscle fiber atrophy (8%), heterotopic calcification of gastric mucosa (12%), and severe intestinal mucus inspissation (4%). Correlations between the various lesions and clinical manifestations, and possible etiologic and pathogenetic mechanisms of these lesions, are discussed.

Adolescent↗
PubMed1987

Pathological features of Kawasaki disease (mucocutaneous lymph node syndrome).

Kawasaki disease (mucocutaneous lymph node syndrome) (MCLS) is an apparently infectious disease, an etiological agent of which has not been established, with peak age incidence at about 1 year, but with progressively fewer cases occurring into the fourth decade. Early clinical features include fever, rash, conjunctival injection, dry reddened lips, oropharyngeal reddening, enlarged cervical nodes, and swelling and redness of hands and feet. Peeling of skin of fingers and toes, arthralgia, and marked thrombocytosis are frequent 1-2 weeks after onset. Myocarditis, cardiac valvulitis, and lymphocytic or mixed interstitial infiltration of pancreas, renal, splenic, and hepatic hilar regions are seen in the early phase, but arteritis, typically of extraparenchymal arteries, is the most important aspect of MCLS, hence the term infantile periarteritis nodosa, formerly applied to fatal cases of MCLS. Thrombosis of coronary artery aneurysms is the most common cause of death (rate about 0.5%). The peak time of death is 3-4 weeks from onset, but death from coronary occlusion has been seen as late as 14 years after the acute phase. Aneurysmal rupture with hemopericardium or retroperitoneal hemorrhage is rare, as are late brachial, iliac, or other arterial aneurysms. Pathological features of MCLS in the early and later stages are described and illustrated, and the epidemiologic, etiologic, forensic, and other aspects of the disease are discussed.

Adolescent↗