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Biomedical subjects

B H Landing

Publications and source records attributed to B H Landing.

At least 73 records · Page 4Linked to original sources
PubMed1985

Splenic growth rates in cirrhotic and other splenomegalic diseases of childhood.

The weights of the spleens of series of patients with various disorders of children dating from birth or early infancy and causing splenomegaly, with or without cirrhosis of the liver, were analyzed. The linear regression equation for spleen weight versus age in months for each disease was derived, and the rate constants from these equations were adjusted for the age range of the patients in each group. The original data of Coppoletta and Wolbach were used for normal values. The rates of splenic growth of appropriate entities for which the regression equation could be computed fell into three groups, with adjusted rate constants (growth of spleen in grams per month) of 6.53-6.95 (biliary atresia, thalassemia, and cirrhosis following neonatal hepatitis), 2.30-2.62 (cirrhosis of alpha-1-antitrypsin deficiency, infantile polycystic disease, and spherocytosis), and 1.06-1.11 (cystic fibrosis and idiopathic thrombocytopenic purpura). These classes of splenic growth rates are approximately 10, 3.7, and 1.6 times the normal growth rate (0.67 g/mo). Rate constants could not be computed for the categories cirrhosis following viral hepatitis and hemolytic anemia other than spherocytosis and sickle cell anemia, and the numbers of patients with splenic vein obstruction, cirrhosis with the cholestatic syndrome of parenteral alimentation, hypoplastic anemia with hemosiderosis, tyrosinemia, Byler's disease, congenital hepatic fibrosis, and Wilson's disease were too few for analysis. The significance of the finding of classes or "quantum groups" of splenic growth rates in disorders of children, dating from birth or early infancy and causing splenomegaly, is uncertain. Comparable data on adequate series of patients with other appropriate disorders will be necessary.

Adolescent↗
PubMed1985

Time course of the intrahepatic lesion of extrahepatic biliary atresia: a morphometric study.

The time course of the intrahepatic lesions of untreated extrahepatic biliary atresia was evaluated by morphometric analysis of 49 specimens from 27 patients. The data show an early phase of rapid bile ductular proliferation, with peak in this material at 205 days, followed by rapid duct regression to approximately 400 days, and slower progressive intrahepatic duct loss thereafter. The ratio of ducts to connective tissue in portal tracts follows a similar course. Connective tissue in portal tracts rises on a slower course and continues to increase after maximum duct regression is reached, so the ratio of parenchyma to fibrous septa falls over the later course of the process. Although duct and connective tissue proliferation in hepatic portal tracts is associated in many liver diseases, the data of this study demonstrate dissociability of the relationships, with the fibrosis in the later stages of the intrahepatic process in extrahepatic biliary atresia apparently neither responsive to nor inducing biliary ductular proliferation.

Bile Ducts↗
PubMed1985

Preferentially longer survival of males than of females with untreated extrahepatic biliary atresia: correlations with time course of intrahepatic lesions and suggestion of possible ethnic influences.

Males with untreated or unsuccessfully treated extrahepatic biliary atresia show statistically significantly longer survival than females. Females show a greater degree of intrahepatic biliary ductular proliferation than males in the early phase of the process (to age approximately 200 days) and greater duct regression and more rapid connective tissue proliferation than males from approximately 200 to 400 days. Females also show greater frequency of statistically significant hepatomegaly than males over the first year. The black and Hispanic patients in this series tended to show a less marked degree of bile ductule proliferation in the early phase of the process than other patients, but the numbers of such patients available for study were not enough for statistical significance. This point, and its possible clinical correlates, will require further analysis, as will possible biochemical explanations for the differences between males and females in the time course of the intrahepatic lesions of extrahepatic biliary atresia.

Black or African American↗
PubMed1985

A possibly distinctive vacuolar change of the amniotic epithelium associated with gastroschisis.

Histological examination of the placental membranes of 3 of 4 infants with gastroschisis revealed peculiar vacuolar change of the cytoplasm of the amniotic epithelial cells. No storage material was demonstrated by special stains or electron microscopic study. This vacuolar change is different from other amniotic cell abnormalities, such as those related to meconium staining.

Abdominal Muscles↗
PubMed1985

Pathologic studies of the osteoporosis of Von Gierke's disease (glycogenosis 1a).

Pathologic and point count-morphometric studies of ribs, vertebrae, and iliac crests of 7 patients with Von Gierke's glycogenesis type Ia aged 5 months to 30 years were performed. The bone lesion is a pure osteoporosis (reduction in mass of bone matrix) with no evidences of significant physeal cartilage abnormality or of osteitis fibrosa or osteomalacia (reduced mineralization of bone matrix). The osteoporosis was already marked in the youngest patient studied (5 months). The discrepancy between normal and glycogen storage disease (GSD) bones increased progressively with age for ribs and was less severe for vertebrae. Available biochemical data give no indication of primary disturbance of calcium or phosphate metabolism, of parathyroid activity, or of vitamin D metabolism. Clinical data suggest that the osteoporosis of Von Gierke's disease is due to hypoglycemia or a metabolic sequela thereof, such as insulinopenia, but pathologic study of patients treated by newer techniques of maintaining euglycemia in GSD is needed.

Adult↗
PubMed1984

The renal lesion of congenital hepatic fibrosis: pathologic and morphometric analysis, with comparison to the renal lesion of infantile polycystic disease.

The renal lesion of congenital hepatic fibrosis (CHF = Blyth and Ockenden's juvenile polycystic disease of liver and kidneys) was analyzed from 6 specimens from patients aged 3 3/12 to 19 3/12 years and compared with that of 5 patients with infantile polycystic disease (IPCD) aged 6 months to 14 4/12 years. Pathologic, microdissection, injection, and morphometric studies show that the predominantly medullary cystic lesion of CHF shows different distribution in medullary, cortico-medullary, and cortical zones of kidney from the lesion of IPCD, and shows a different time course, from early life to renal insufficiency, from that of IPCD. The renal cysts in CHF affect deep or central collecting tubules, in contrast to the involvement of more peripheral orders of collecting tubules in IPCD. Papillary pore counts, performed for 1 patient, gave significantly low values, in contrast to normal values reported for IPCD. The findings support the previously published conclusion, based on differences in the hepatic lesions of the two conditions, that CHF and IPCD are difference diseases, rather than different permissible manifestations of a single disease.

Adolescent↗
PubMed1984

Tubulointerstitial renal diseases of children: pathologic features and pathogenetic mechanisms in Fanconi's familial nephronophthisis, antitubular basement membrane antibody disease, and medullary cyst disease.

The renal lesions of 15 children with tubulointerstitial diseases (TID), originally diagnosed as Fanconi's familial nephronophthisis (FFN), medullary cyst disease (MCD), or antitubular basement membrane (TBM) antibody disease were studied by light, electron, and immunofluorescence microscopy and by peroxidase-antiperoxidase stain for IgG. Kidneys originally considered in all three categories showed similar pathologic findings, with periglomerular fibrosis, interstitial fibrosis and infiltration, TBM thickening, and glomerulopathy. The data suggest that MCD and FFN are not synonyms, but that FFN can lead to medullary cystic disease, as can other tubulointerstitial diseases. Anti-TBM disease appears to be a more frequent cause of TID than is usually recognized. Glomerular involvement in TID may be due to deposition of anti-TBM antibody/TBM antigen complexes.

Autoimmune Diseases↗
PubMed1984

Lipid-laden macrophages in tracheal aspirates of newborn infants receiving intravenous lipid infusions: a cytologic study.

Seventy-four neonates with respiratory distress syndrome had cytologic study of tracheal aspirates. Thirteen, all of whom were receiving intravenous lipid and nothing orally had abundant macrophages with foamy cytoplasm, positive for intracytoplasmic lipid by Sudan black stain, in the tracheal aspiration specimens. Cytologic surveillance of tracheal aspiration specimens may be a useful method of monitoring infants receiving intravenous lipid infusions.

Fat Emulsions, Intravenous↗
PubMed1982

Comparisons of eccrine sweat gland anatomy in genetic, chromosomal, and other diseases, and a suggested procedure for use of sweat gland measurements in differential diagnosis.

Statistical analysis of the dimensions of microdissected eccrine sweat glands (duct length, coil volume, ratio of coil volume to duct length, and axis ratio of coil) was performed for several diseases (cystic fibrosis of the pancreas, Werdnig-Hoffmann disease, tetralogy of Fallot, chronic renal disease, and trisomies 13, 18, and 21) using both individual and grouped age-matched control patients. Duct length, coil volume, and the ratio of the two all rise with age. Eccrine gland duct length was found to be significantly large in tetralogy of Fallot and Werdnig-Hoffmann disease and small in chronic renal disease (less so in males than in females, trisomy 13 and trisomy 18). Secretory coil volume was significantly smaller than normal in trisomy 21 (Down syndrome) and in chronic renal disease, and the ratio of coil volume to duct length was low in trisomy 21 and chronic renal disease. The shape of the secretory coil (axis ratio) was possibly abnormal in trisomy 13. Gland dimensions were normal for cystic fibrosis. Using the multivariate procedure of discriminant analysis, it was found that sweat gland measures significantly contributed to the differentiation of diseases, after adjustments were made for variations in age-at-death. This suggested the possibility that criteria for distinction of clinically similar genetic, metabolic, or chromosomal diseases by study of the anatomic properties of eccrine glands obtained by skin biopsy could be developed. A procedure of analysis comparing the "percentage of normal" of gland dimensions for each disease to control values, and thereby differentiating disease categories on the basis of the "percentage of normal" values, is presented.

Age Factors↗
PubMed1982

Triosephosphate isomerase deficiency. A case report with neuropathological findings.

A 12-year-old girl had chronic nonspherocytic hemolytic anemia due to triosephosphate isomerase deficiency. Developmental and motor delay and muscular weakness were followed by cerebellar dysfunction and finally spasticity with hyperreflexia. Abnormal histopathological findings were hyaline cell bodies and axonal "spheroids" in the hypothalamus and cerebellar cortex, severe neuronal loss in the dentate and olivary nuclei, and partial loss of cerebellar Purkinje's and granular layer cells (olivocerebellar atrophy).

Brain↗