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Biomedical subjects

P Wasi

Publications and source records attributed to P Wasi.

At least 91 records · Page 5Linked to original sources
PubMed1983

Different patterns of intraerythrocytic inclusion body distribution in the two types of haemoglobin H disease. An ultrastructural study.

Electron microscopic examination of intraerythrocytic inclusion bodies induced by methylene blue was carried out in 7 non-splenectomized patients with haemoglobin (Hb) H disease. In classical Hb H disease with the genotype of alpha-thalassaemia 1/alpha-thalassaemia 2 the inclusion bodies were mostly membrane-associated. In contrast, in Hb H disease of alpha-thalassaemia 1/Hb Constant Spring genotype the majority of inclusion bodies were in a floating stage. Possible mechanisms for the unexpected difference are discussed.

Erythrocyte Inclusions↗
PubMed1982

Zinc and copper status in hemoglobin H disease and beta-thalassemia/hemoglobin E disease.

The plasma zinc and copper and urinary zinc levels were determined by an atomic absorption spectrophotometer technic in 14 patients with hemoglobin (Hb) H disease, 34 patients with beta-thalassemia/Hb E disease and 30 control subjects. Plasma zinc levels were lower and the plasma copper levels were higher in the patients than in controls. The plasma Cu:Zn ratios, a more valuable indicator of body zinc status, increased in the diseased subjects, indicating zinc deficiency. Higher urinary zinc levels in the both Hb H disease and 30 control subjects. Plasma zinc levels were lower and the plasma copper levels were higher in the patients than in controls. The plasma Cu:Zn ratios, a more valuable indicator of body zinc status, increased in the diseased subjects, indicating zinc deficiency. Higher urinary zinc levels in the both Hb H disease and 30 control subjects. Plasma zinc levels were lower and the plasma copper levels were higher in the patients than in controls. The plasma Cu:Zn ratios, a more valuable indicator of body zinc status, increased in the diseased subjects, indicating zinc deficiency. Higher urinary zinc levels in the both Hb H disease and beta-thalassemia/Hb E disease suggest that chronic hemolysis is responsible for hyperzincuria leading to zinc deficiency. The thalassemic patients with undergrowth had higher plasma Cu:Zn ratios than the patients with normal development, indicating a more severe zinc deficiency in the former.

Adolescent↗
PubMed1982

Heterogeneity of beta thalassaemia in Thailand.

Beta thalassaemia in Thailand is heterogeneous. Clinical, genetical, haematological and globin chain biosynthetic studies were performed in seven beta-thalassaemia families. The results showed different gene combinations. These were alpha-thalassaemia/homozygous beta 0-thalassaemia, questionable double heterozygosity between a beta-thalassaemia and a silent beta-thalassaemia genes with low Hb F of unexplained cause, silent beta-thalassaemia/beta+ - or beta 0-thalassaemia, high Hb A2 high Hb F-beta-thalassaemia - a new mutant, mild beta+-thalassaemia/Hb E, and beta 0-thalassaemia/heterocellular HPFH or delta beta-thalassaemia associated with alpha-thalassaemia. Most of variability of clinical and haematological findings in these families is due to heterogeneity of the beta-thalassaemia and related genes.

Adolescent↗
PubMed1981

Spinal cord compression in thalassemia. Report of 12 cases and recommendations for treatment.

Twelve patients with beta-thalassemia/hemoglobin E disease had spinal cord compression. Ten were made and two female, aged 17 to 40 years. The causes of spinal cord compression presumably were extramedullary hematopoietic masses. This was proved by surgery in two cases. In six cases, myelography demonstrated extradural blockade. In the others, the recurrent nature of the paraparesis and the prompt response to deep x-ray therapy were compatible with cord compression by extramedullary hematopoietic masses. Although spontaneous recovery and disappearance of the neurological signs after blood transfusions were observed, these were slow and uncertain. Deep x-ray therapy led to prompt response with more lasting benefit in all cases and is thus recommended as standard treatment for this complication.

Adolescent↗
PubMed1981

Homozygous haemoglobin Constant Spring: a need for revision of concept.

Twenty-two patients with mild haemolytic anaemia and haemoglobin (Hb) Constant Spring (CS) of around 6% were studied because they were suspected of having homozygous Hb CS. Family studies revealed Hb CS trait in both parents of eight patients, supporting that they were homozygous for Hb CS. The other patients were included because they had clinical and haematological features similar to the diagnosed cases of homozygous Hb CS. Heterozygosity and homozygosity for Hb CS are clearly distinguishable in that the former is asymptomatic but the latter is associated with overt haemolytic anaemia, and the levels of Hb CS in the two conditions of less than 1% and around 6%, respectively, do not overlap. The findings in homozygous Hb CS contradict prediction. There are four alpha-structural genes per normal human diploid genome. Hb CS trait is believed to be almost equivalent to alpha-thalassaemia 2 or a loss of one alpha-gene because HB CS, and alpha-variant, is barely or not detectable. Homozygosity for Hb CS has thus been predicted to be equivalent to alpha-thalassaemia 1 or a loss of two genes. The latter is asymptomatic and associated with microcytic-hypochromic red cells. However, Hb CS homozygosity presents with mild overt haemolytic anaemia and normal sized red cells. Pathogenesis associated with Hb CS inheritance is more complex than originally believed. There is a possibility that the unstable alpha CS mRNAs precipitate and aggregate leading to pathology of red cells and to the basophilic stippling appearance, so striking in this syndrome.

Adolescent↗
PubMed1981

Ineffective erythropoiesis in haemoglobin E beta -thalassaemia: an electron microscope study.

Electron microscope studies have been performed on the bone marrow cells of two non-splenectomized patients and the circulating erythroblasts and reticulocytes of three splenectomized patients with HbE/beta-thalassaemia. Some intracellular precipitates (probably consisting of alpha-chains) and mild dyserythropoietic changes were found in the early polychromatic erythroblasts within the bone marrow. Larger quantities of precipitate and more marked dyserythropoietic changes were found in the late polychromatic erythroblasts and reticulocytes both within the marrow and within the circulation. The bone marrow macrophages contained phagocytosed erythroblasts within their cytoplasm. These data indicate that the anaemia in HbE/beta-thalassaemia results largely from dyserythropoiesis and ineffective erythropoiesis. The ultrastructural abnormalities encountered in the cases of HbE/beta-thalassaemia were qualitatively and quantitatively similar to those seen in homozygous beta-thalassaemia.

Adult↗
PubMed1981

Morphology and kinetics of erythropoiesis in haemoglobin H disease.

Some aspects of erythroblast function have been investigated in two Thai patients with haemoglobin H disease. Electron microscope and high resolution autoradiographic studies revealed that (1) some erythroblasts and marrow reticulocytes contained branching intracytoplasmic inclusions and (2) the presence of such inclusions did not necessarily prevent the entry of cells either from the G1 phase into the S phase or from the G2 phase into mitosis. The distribution of early polychromatic erythroblasts in the different stages of the cell cycle was determined by the technique of combined Feulgen microspectrophotometry and 3H-thymidine autoradiography and found to be essentially normal. It is concluded that although the presence of an excess of alpha-chains in the beta-thalassaemia syndromes has been previously shown to be associated with a gross impairment of erythroblast proliferation, the presence of an excess beta-chains in HbH disease is not.

Adult↗
PubMed1981

Endocrine function in thalassaemia.

Twenty-one Thai patients with beta-thalassemia/haemoglobin E and haemoglobin H diseases, 8-20-years-old, were studied. These patients had receive none or minimal blood transfusion. The important clinical endocrine abnormalities were growth retardation and sexual immaturity. GH secretion was found to be impaired in the majority of patients. Oral GTT showed chemical diabetes in one out of sixteen tests, a much lower incidence than in thalassaemic patients treated by hypertransfusion in the West. The mean insulin levels basally and after glucose loading were lower than those of the normal controls. Thyroid function was normal in all of the patients. Serum cortisol and 24-h urinary oxogenic steroids 917 OGS) levels were normal, as was adrenal cortical reserve in all the patients. The literature on endocrine function in in thalassaemia is reviewed.

Adolescent↗
PubMed1981

Serum ferritin levels in thalassemias and the effect of splenectomy.

Iron overload is a constant and the more important complication in thalassemia. Serum ferritin concentration accurately reflects body iron stores. A total of 245 thalassemic patients aged 12-55 years were examined, 71 having Hb H disease and 174 beta-thalassemia/Hb E disease. The patients received minimal or no blood transfusions. 73 patients with beta-thalassemia/Hb E were studied 1-28 years after splenectomy. The serum ferritin levels in both Hb H and beta-thalassemia/Hb E patients were higher than normal. They were higher in beta-thalassemia/Hb E than Hb H disease. Most striking was the significantly higher serum ferritin levels in splenectomized patients with beta-thalassemia/Hb E disease than in the nonsplenectomized ones. The observation is compatible with previous observations that splenectomy in thalassemia is associated with increased iron deposition and increased transferrin iron saturation. The further increase in iron overload after splenectomy in thalassemia should be borne in considering removal of this organ.

Adolescent↗
PubMed1981

Hypoxaemia and the effect of aspirin in thalassaemia.

Blood gas analysis of arterial blood was performed in 34 patients with beta o-thalassaemia/haemoglobin E disease and homozygous beta o-thalassaemia. Among the 22 splenectomised patients 19 had PaO2 lower than the normal expected value, and 18 of these had PaO2 lower than 80 mmHg. Of the 12 non-splenectomised patients 5 had PaO2 lower than the expected normal value but only in one case it was lower than 80 mmHg. After aspirin or Persantin administration there was a definite rise in the PaO2 in 10 out of 12 patients. The hypoxaemia is believed to occur from increased platelet aggregation leading to pulmonary artery occlusion. The rise of the arterial PaO2 after aspirin administration indicates that the observed hypoxaemia is due to reversible platelet aggregation in the majority of cases.

Adolescent↗
PubMed1981

Increased circulating platelet aggregates in thalassaemia.

Examination for circulating platelet aggregates according to Wu and Hoak revealed increased circulating platelet aggregates in 71% of splenectomized and 35% of nonsplenectomized patients with beta (0)-thalassaemia/Hb E disease. This may be causally related to the newly observed high incidences of pulmonary artery thrombosis and hypoxaemia in splenectomized thalassaemic patients. It is recommended that anti-platelet aggregation drugs such as aspirin and/or dipyridamole are given to thalassaemic patients after splenectomy.

Humans↗
PubMed1980

Haemoglobin Bart's hydrops syndrome in Greece.

A case of haemoglobin Bart's hydrops syndrome was characterised in a Greek family with a history of three other fetuses with hydrops. Family studies showed that both the mother and father carried alpha-thalassaemia genes, and globin-chain synthesis analysis of the present fetus showed a total absence of alpha-chain production. The haemoglobin composition of the fetus was similar to that seen in cases in south-east Asia, and analysis of DNA from the Greek case confirmed the total deletion of the alpha-chain genes. The extent of the deletion, however, differed from that seen in south-east Asian cases and included the loss of one of the embryonic zeta-chain genes. Thus the severe form of alpha-thalassaemia occurs in Greece but has arisen independently from the similar condition in south-east Asia. The condition must be considered in any woman of this racial background who gives a history of unexplained stillbirths.

Adult↗