agammaglobulinemia/phosphatase

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Changes in biochemical, hematological and immunological profiles after low-dose intravenous immunoglobulin administration in patients with hypogammaglobulinemia.

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We assessed the immediate effect of intravenous immunoglobulins (IVIG) on the biochemical, immunological and hematological profiles in patients with hypogammaglobulinemia. Over a period of three months, patients with antibody deficiencies, who had been established on stable IVIG treatment as

Needle-shaped inclusions in plasma cells in a patient with hypogammaglobulinemia.

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A case of a patient with needle-shaped inclusions in plasma cells was reported. Some of the inclusions were positively stained for acid phosphatase and beta-glucuronidase. Ultrastructurally, each inclusion was surrounded by a single limiting membrane without any relation to rough-surfaced

Lymphocyte ecto-5'-nucleotidase deficiency in agammaglobulinemia.

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Fresh peripheral blood lymphocytes from eight patients with congenital agammaglobulinemia demonstrate reduced ecto-5'-nucleotidase activity when compared to the mean activity of normal subjects and patients with other forms of immunoglobulin deficiency. A specific defect of ecto-5'-nucleotidase is

Association of primary sclerosing cholangitis, thymoma and hypogammaglobulinemia.

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A 64-year-old Japanese woman with thymoma has been suffering from diarrhea and increased alkaline phosphatase levels without jaundice. Her serum immunoglobulin levels of IgM and IgG were less than half of the normal levels, with an increase in CD8 (suppressor/cytotoxic) T cell percentage and a

[Intestinal disaccharidase and alkaline phosphatase activities of jejunal biopsies in small bowel diseases of children (author's transl)].

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Intestinal disaccharidase activities were determined in 294 jejunal biopsies obtained from 254 children with various disorders of the small bowel, and alkaline phosphatase activity was measured in 251 biopsies. In normal mucosa a broad range of enzyme activity was found corresponding with the data

Intravenous immunoglobulin prophylaxis causing liver damage in 16 of 77 patients with hypogammaglobulinemia or IgG subclass deficiency.

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Sixteen of 77 patients (21 percent) with common variable immunodeficiency or IgG subclass deficiency contracted non-A, non-B hepatitis in association with intravenous infusions of immunoglobulin. The hepatitis seemed to run a more severe course in these patients than in non-immunodeficient patients.

Comparison of Bone Mineral Density in Common Variable Immunodeficiency and X-Linked Agammaglobulinaemia Patients.

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BACKGROUND Primary antibody deficiency (PAD) is the most common group of primary immunodeficiency disorders, resulting from different defects in the development and function of B cell lineage. Common variable immunodeficiency (CVID) and X-linked agammaglobulinemia (XLA) are two of the major types of

Congenital disorders of the function of polymorphonuclear neutrophils.

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This review has concentrated on clinical syndromes for which a congenital basis of polymorphonuclear neutrophil dysfunction has been identified. The first clinical syndrome found to be associated with dysfunctional polymorphs was chronic granulomatous disease of childhood. Identification of a

WHI-131 Promotes Osteoblast Differentiation and Prevents Osteoclast Formation and Resorption in Mice.

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The small molecule WHI-131 is a potent therapeutic agent with anti-inflammatory, antiallergic, and antileukemic potential. However, the regulatory effects of WHI-131 on osteoblast and osteoclast activity are unclear. We examined the effects of WHI-131 on osteoblast and osteoclast differentiation

Biological secondary contributors to osteoporosis in fractured patients, is an early systematic assay relevant?

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To evaluate the prevalence of biological abnormalities leading to secondary osteoporosis in recently fractured patients.Adults older than 50, hospitalized for a non-vertebral fracture from July 2015 to October 2016, were assessed for bone fragility

Myeloid, erythroid, and immune system defects in a family. A new stem-cell disorder?

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Four of six siblings, offspring of Sicillian first cousins, developed a clinical disorder in early adulthood affecting the hematopoietic and immunoglobulin-producing systems. A female sibling died at age 21 with myeloid aplasia and agranulocytosis. A male sibling, at age 17, presented with erythroid

[Suspected inherited granulocytopathy in four Holstein Friesian calves].

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Five Holstein Friesian calves varying in age from 7 to 9 weeks old, were suspected of suffering from an inherited granulocytopathy known as bovine leucocyte adhesion deficiency (BLAD). Four of them were examined clinically and at necropsy. The most significant clinical findings were fever,

Osteopetrosis mimicking juvenile myelomonocytic leukemia.

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A 5-month-old boy developed splenomegaly, anemia, thrombocytopenia with elevated white cells, monocytosis and immature granulocytes in the peripheral blood. Bone marrow showed dysplasia without blastosis. Increased colony-forming unit-granulocyte-macrophage was found in the peripheral blood,

Hepatic amyloidosis (primary [AL], immunoglobulin light chain): the natural history in 80 patients.

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OBJECTIVE We wished to study patients with a premortem diagnosis of primary hepatic amyloidosis to determine what clinical and laboratory features might assist in recognizing the disease and assessing prognosis. METHODS A group of 80 patients with liver biopsy proven primary hepatic amyloidosis was

Sarcoidosis and common variable immunodeficiency. Report of 8 cases and review of the literature.

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The true incidence of sarcoidosis in common variable immunodeficiency (CVID) is unknown. We report here 8 cases of sarcoidosis among 80 patients with CVID followed in our clinics, along with 22 well-documented cases reported in the literature. Sarcoidosis, therefore, represents an important entity

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