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Biomedical subjects

P Hathaway

Publications and source records attributed to P Hathaway.

16 recordsLinked to original sources

Failure to thrive: knowledge for social workers.

Growth failure in young children no longer is classified simply as organic or nonorganic failure to thrive. The symptom of failure to thrive encompasses a variety of complex disorders, and each requires a specific treatment approach. Various forms of nonorganic growth failure and the social work role in intervention are reviewed. The diagnosis and treatment of attachment disorder, a potentially dangerous form of nonorganic failure to thrive that is considered child neglect, are discussed in detail.

Child↗

Hb Potomac (101 Glu replaced by Asp): speculations on placental oxygen transport in carriers of high-affinity hemoglobins.

Blood from a woman with unexplained erythrocytosis had increased oxygen affinity, but no abnormality could be detected by electrophoresis or chromatography of her hemolysate. Separation of the tryptic peptides of her beta chains disclosed two half-sized peaks in the regions of beta T-11. The faster of these was abnormal, with the structure beta 101 Glu replaced by Asp. The new hemoglobin was called "Potomac." Three of the proband's four surviving siblings and both of her children were carriers. Differences in the ratio of carrier: normal children born to male of female carriers of 23 other high-affinity hemoglobins were not significant. The high proportion of carriers in this kindred was probably due to chance alone, and not because high maternal oxygen affinity interfered with oxygen transport to fetuses with normal hemoglobin.

Adult↗

Postsynthetic deamidation of hemoglobin Providence (beta 82 Lys replaced by Asn, Asp) and its effect on oxygen transport.

Carriers of hemoglobin Providence have three types of beta chain in their hemolysates. The two abnormal chains have asparagine (Providence N, Prov N) or aspartic acid (Providence D) at position beta 82, instead of lysine. In vitro, only two beta chains are synthesized by reticulocytes of carriers, betaA and betaProv N. In vivo studies showed that the specific activity of Providence N was initially 10-fold higher than that of Providence D; the specific activities of the two labeled hemoglobins were approximately equal 5 wk after injection of isotope. Oxygen affinity of carriers' blood was somewhat increased, but they were not polycythemic. The affinity of the purified hemoglobins Providence was decreased. Addition of 2, 3 diphosphoglycerate had little effect on the affinity of either hemoglobin component, and addition of inositol hexaphosphate produced no change in the affinity of Providence D. These studies demonstrate that Providence N is deamidated to Providence D during the life span of the erythrocyte, and suggest this finding may represent only an easily observed prototype of posttranslational modification of proteins in general. Despite and abnormal P50 of the blood, oxygen transport is probably normal in carriers of the abnormal hemoglobins.

Adolescent↗

Improved growth of in vitro colonies in human acute leukemia with the feeding culture method.

Bone marrow cells freshly aspirated from the 10 consecutive untreated adult patients with acute nonlymphocytic leukemia were cultured by 2 different methods: the conventional agar culture method for myeloid colony formation and its modification by daily feeding with culture medium. In 5 patients, colonies grew in much higher numbers (4.7-to 330-fold) with feeding than without. Three patients grew colonies only with feeding. Two of these 3 patients required L-ascorbic acid in the fed medium for colony growth. Colonies did not grow from the remaining 2 patients by any method. In 7 patients the number of colonies grown with feeding were much higher, up to 170 times higher, than were those from normal control marrows, which grew the same number of colonies regardless of feeding or L-ascorbic acid. Peroxidase and Wright's stains indicated the myeloid differentiation of the cells in the leukemic marrow colonies. The leukemic origin of the colonies was proven by chromosomal analysis. The wide range of linearity between the number of cells plated and the number of colonies grown permits quantitative assay of colony-forming leukemic cells. This assay should be valuable for studies of chemotherapy, growth regulation, and differentiation of leukemic cells.

Animals↗

A patient with the Larsen syndrome.

A child with the Larsen syndrome is described. His multiple malformations included a flattened nasal bridge and other unusual facial features, a cleft palate, a poorly developed larynx and dislocations involving several joints.

Abnormalities, Multiple↗

Hemoglobins in sheep: multiple differences in amino acid sequences of three beta-chains and possible origins.

Among the three adult sheep hemoglobins (A, B, and C), two (A and B) are reportedly products of alleles. The beta-chains of A and B differ by at least seven scattered amino acid residues whereas the beta-sequence of C differs from A by at least 16 residues and from B by at least 21 residues. These changes suggest that the origin of C-beta antedated the divergence of A and B. Five shared differences between A-beta and C-beta with respect to B-beta can be interpreted as the result of selective advantage in favor of B. A complex of additional mechanisms has possibly been involved in maintaining the A-B- C porymorphism.

Amino Acid Sequence↗