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Biomedical subjects

D T Purtilo

Publications and source records attributed to D T Purtilo.

At least 199 records · Page 11Linked to original sources
PubMed1976

Pathogenesis and phenotypes of an X-linked recessive lymphoproliferative syndrome.

A new X-linked recessive lymphoproliferative syndrome has variable phenotypes: fatal infectious mononucleosis (I.M.), agammaglobulinaemia after I.M., American Burkitt's lymphoma, histiocytic lymphoma, immunoblastic sarcoma of B cells, or plasmacytoma. An immunodeficiency to rubeola and the Epstein-Barr virus probably ensues from the mutant gene. The phenotypes (spectrum of B-cell disorders) have a common inheritance and the aetiology is similar.

Adolescent↗
PubMed1976

Lobomycosis. An electronmicroscopic, histochemical and immunologic study.

A 73-year-old woman from Guyana had nodular keloidal lesions on her face which had persisted for 56 years. A biopsy of the lesion revealed lobomycosis, a rare fungal infection. Microscopically, numerous fungi were seen in giant cells and macrophages. The macrophages had abundant pink reticulated cytoplasm. These macrophages in lobomycosis, considered by some in the past to be granular myoblastoma cells, were rich in glycoproteins and appeared to be "Gaucher-like cells" in electronmicrographs. Further, a transition of macrophages containing organisms to Gaucher-like cells was noted. Antibodies to the fungus were demonstrated in the patient's serum by counter immunoelectrophoresis and indirect immunofluorescence studies. We postulate that the Gaucher-like cells formed because of the inability of host macrophages to digest glycoproteins in the capsule of this unusual fungus.

Aged↗
PubMed1976

Giant and "granular melanosomes" in Leopard syndrome: an ultrastructural study.

Electron microscopy of lentigines was performed to study the pigmentation abnormality in two children with LEOPARD syndrome. Giant melanosomes similar to those seen in café-au-lait spots of neurofibromatosis and nevus spilus were found in a lentigine from one of our cases. Our results show that "spherical granular melanosomes" described in neurofibromatosis, are lysosomal-like structures associated with the development of complex melanin granules. Our study also demonstrates that immature melanosomes are present in some keratinocytes of LEOPARD syndrome. This finding is in contrast to the prevailing concept that only mature melanosomes are transferred to keratinocytes. The occurrence of individual melanosomes of normal size and shape in keratinocytes of skin in whites with LEOPARD syndrome, suggests that neither the size of melanosomes, nor the racial differences are the factors determining the distribution of melanosomes in keratinocytes.

Abnormalities, Multiple↗
PubMed1976

Humoral immunity of parasitized, malnourished children.

The nutrition, intensity of parasitism, complete blood count, and the serum immunoglobulin concentration of 63 children were studied. Hyperimmunoglobulinemia was present in the vast majority of subjects regardless of their nutritional status. The IgA and IgM concentrations were moderately increased, the IgG was increased threefold, and the IgE was markedly increased in concentration. The concentrations of the immunoglobulins were related significantly to the intensity of parasitism.

Ascariasis↗
PubMed1976

Clonorchiasis and hepatic neoplasms.

Thirty-eight subjects from Hong Kong with chronic infestation by Clonorchis sinensis were studied. Ten of the patients died of hepatocellular carcinoma, seven of cholangiocarcinoma, and one each of carcinoma of the common bile duct and lymphoma. The major difference between the patients having cholangiocarcinoma and hepatocellular carcinoma was cirrhosis. Only one patient with cholangiocarcinoma had cirrhosis; whereas all but one patient with hepatocellular carcinoma had cirrhosis. The etiopathogenesis of these two tumors is substantially different.

Adenoma, Bile Duct↗
PubMed1975

Opportunistic mycotic infections in pregnant women.

Immunosuppression was the major factor associated with the deaths of 44 women who died from opportunistic mycotic infections. The patients were immunosuppressed by malignancies, irradiation, cytotoxic drugs, and metabolic diseases. An additional four patients may have developed candidiasis as a result of antibiotic therapy. Coccidoidomycosis was the only mycosis solely associated with pregnancies not altered by exogenous immunosuppression.

Adolescent↗
PubMed1975

X-linked recessive progressive combined variable immunodeficiency (Duncan's disease).

Of 18 boys in Duncan kindred, 6 died of a lymphoproliferative disease. They exhibited a subtle, progressive combined variable immunodeficiency disease characterised by benign or malignant proliferation of lymphocytes, histiocytosis, and alterations in concentrations of serum-immunoglobulins. Infectious mononucleosis occurred during or preceding terminal events in at least 3 of the cousins. Fever, pharyngitis, lymphadenomegaly, hepatosplenomegaly, atypical lymphocytosis, and a spectrum ranging from agammaglobulinaemia to polyclonal hyper-gammaglobulinaemia occurred. At necropsy, the thymus gland and thymic-dependent areas in the lymph-nodes and spleen were depleted of lymphocytes. Diffuse infiltrates composed of lymphocytes, plasma cells, and histiocytes, some containing erythrocytes, invaded the haematopoietic organs, viscera, and central nervous system. In addition, 2 half-brothers had lymphomas of the ileum and central nervous system. Approximately half the boys, including the half-brothers, were affected, and girls were spared, implying sex-linked recessive inheritance. Various lymphohistiocytoses resemble Duncan's disease, but it is distinctive from them in the mode of inheritance or by histiological characteristics. This study suggests that the Epstein-Barr virus or other viruses triggered the fatal proliferation of lymphocytes and that progressive attrition of T-cell functions allowed uncontrolled lymphoproliferation.

Adult↗
PubMed1975

Primary hepatic malignancy in pregnant women.

Three women dying from hepatic carcinoma during pregnancy are presented. One of these women with a hepatocellular carcinoma and alpha fetoprotein in the serum and antibody to hepatitis B antigen. A fourth patient died 2 months post partum with a cholangiocarcinoma. A false positive pregnancy test suggested that she had metastatic choriocarcinoma in the liver, and a panhysterectomy was performed. The clinical diagnosis with the use of alpha fetoprotein and chorionic gonadotropin for detection of hepatoma and the etiopathogenesis of primary hepatic malignancy in pregnancy are discussed.

Adenoma, Bile Duct↗
PubMed1975

Incidence of cancer in patients with leprosy.

Individuals with congenital immunodeficiences and patients who are immunosuppressed for maintenance of organ allografts experience a marked increase in the occurrence of malignancy. Patients with lepromatous leprosy also have depressed cellular immunity, but or study of 195 autopsied subjects with leprosy did not reveal an increase in the occurrence of cancer. Thirty-three of the 195 subjects with leprosy or 16.9% died of cancer, which is comparable to an age-matched group of individuals.

Adult↗
PubMed1975

The immune system of the nine-banded armadillo (Dasypus novemcinctus, Linn).

The armadillo is rapidly emerging as an animal for biomedical research. A morphological study of the immune system of the armadillo was undertaken to try to demonstrate a possible defect in the morphogenesis of the lymphoid organs that could account for their susceptibility to leprosy. Blood from 20 domesticated adult armadillos revealed lymphocytes predominated among circulating leukocytes in the majority of animals, whereas in wild animals neutrophils predominated. Necropsies revealed well-developed thymus glands and the thymus-dependent areas in lymph nodes and spleens contained abundant lymphoid cells. Hence, we conclude that the susceptibility of the armadillo to leprosy cannot be explained on a morphological basis.

Age Factors↗
PubMed1975

Fatal infections in protein-calorie malnourished children with thymolymphatic atrophy.

The clinicopathological features of 25 children who died with protein-calorie malnutrition were studied. All but four subjects were found at necropsy to have nutritional thymectomy and all but 3 died of infectious diseases. The infectious agents were chiefly intracellular micro-organisms including miliary tuberculosis, Herpes simplex, varicella, measles, Pneumocystis carinii, and Plasmodium falciparum. Staphylococcal infections, salmonellosis, shigellosis, strongyloidiasis, and hookworm were other significant infectious agents. Nutritionally acquired defective immunity, especially cell-mediated immunity, probably permitted these infectious agents to multiply and to disseminate widely.

Atrophy↗