hypergammaglobulinemia/atrophy

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An autosomal recessive syndrome of joint contractures, muscular atrophy, microcytic anemia, and panniculitis-associated lipodystrophy.

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BACKGROUND Genetic lipodystrophies are rare disorders characterized by partial or complete loss of adipose tissue and predisposition to insulin resistance and its complications such as diabetes mellitus, hypertriglyceridemia, hepatic steatosis, acanthosis nigricans, and polycystic ovarian

Hyperimmunoglobulinemia E syndrome of neonatal onset.

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A case of hyperimmunoglobulinemia IgE with recurrent infections of neonatal onset and unfavourable evolution is described. The first clinical finding was the appearance of maculopapular rash in the skin when the child was fifteen days old. From this period onwards, respiratory and cutaneous

Autoimmune manifestations in acquired idiopathic splenic atrophy: A puzzling association.

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Splenic atrophy is an uncommon diagnosis associated with celiac sprue or other well-characterized connective tissue diseases, drepanocytosis, or amyloidosis. We report two patients with splenic atrophy revealed by thrombocytosis. Both patients had anti-nuclear antibodies. Patient 1 also had a grade

Sinus histiocytosis with massive lymphadenopathy (Rosai and Dorfman) and significant skin involvement.

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Clinicopathological features of this female patient described here, showing unusual and extremely marked sinus histiocytosis of lymph nodes with considerable systemic lymphadenopathy, accompanying fever as well as acceleration of erythrocyte sedimentation rate fairly well coincided with the symptoms

A child with primary Sjögren syndrome and a review of the literature.

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Primary Sjögren syndrome (pSS) is an uncommon disease in childhood. Childhood pSS might have different clinical manifestations than adult pSS. We describe a 13-year-old girl with multiple episodes of bilateral parotid swelling lasting 2 years. Her history included severe arthralgia, local edema, and

Waldenström hypergammaglobulinemic purpura and pregnancy.

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BACKGROUND Waldenström hypergammaglobulinemic purpura is characterized by hypergammaglobulinemia, recurring purpura, and an elevated erythrocyte sedimentation rate. It is a rare disease and, to our knowledge, there have been no previous reports of its presence during pregnancy. We report a patient

Visceral leishmaniasis in a patient with sicca syndrome and nephropathy.

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A 63-year-old woman presented with severe volume depletion and pre-renal azotemia. She had xerostomia, xerophthalmia and cervical lymhadenopathy. Urine examination revealed proteinuria, hematuria and glycosuria. Laboratory studies, after volume repletion, revealed hyper-gammaglobulinemia. Renal

Immunological and histological disorders in cats experimentally infected with feline immunodeficiency virus subtype B (TM2 stain).

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Three conventional cats were experimentally infected with subtype B (TM2 strain) of FIV, and two conventional cats served as controls. The infected cats were examined immunologically 99-176 weeks post FIV inoculation (wpi) and histologically at 130 wpi. Two of the three infected cats exhibited lower

Acute liver biopsy lesions in early autoimmune ("lupoid") chronic active hepatitis.

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We studied two female patients with autoimmune ("lupoid") chronic active hepatitis whose liver biopsies at initial presentation showed the unusual features of an acute hepatitis. Centrilobular hepatocyte swelling and multinucleation, acidophilic degeneration, cholestasis, mild fatty change and bile

[Two cases of Kimura's disease associated with bronchial asthma].

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We encountered two rare cases of Kimura's disease associated with bronchial asthma presenting eosinophilia and hyperimmunoglobulinemia E. Patient 1 was a 26-year-old man who had been admitted to our hospital with recurrent increase in left parotid mass in May 1997. He had previously undergone

Liver Cirrhosis in a Child Associated With Castleman's Disease: A Case Report

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Background: Castleman's disease (CD) is a lymphoproliferative disorder. TAFRO syndrome is classified as a variant of CD based on its key clinical manifestations of thrombocytopenia, anasarca (generalized edema and pleural effusion), fever (pyrexia), reticulin

Beneficial effect of a human monoclonal IgM cryoglobulin on the autoimmune disease of New Zealand black mice.

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NZB mice spontaneously develop an autoimmune disease characterized by autoimmune hemolytic anemia, thymic atrophy, lymphoid hyperplasia, and hypergammaglobulinemia. The aim of this study was to examine the hypothesis that cryoglobulins may have an immunoregulatory effect on the autoimmune process.

Extensive anterior cranial fossa idiopathic hypertrophic pachymeningitis: a case report and review of the literature.

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Idiopathic hypertrophic cranial pachymeningitis is a rare chronic inflammatory and fibrosing process of unknown etiology. This entity is characterized by fibrosis and thickening of the dura mater and resulting in neurological syndrome. The authors report a 72 year-old woman who presented with

[Clinical features of the peripheral nerve involvement in necrotizing angitis--characteristics in polyarteritis nodosa and allergic granulomatous angitis].

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Thirteen patients with peripheral neuropathy caused by necrotizing vasculitis were clinico-pathologically analyzed. These patients consisted of nine classical periarteritis nodosa (PN), four allergic granulomatous angitis (Churg-Strauss syndrome, AGA). All of them were proven to have a necrotizing

[A case of motor neuron disease with IgM gammopathy which showed anti-cerebrum antibody].

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A patient, 50-year-old female, developed progressive weakness of lower extremities, and gait disturbance for 2 years. Neurological examination revealed hyperreflexia with pathological reflex, fasciculation in the limbs and tongue, muscle weakness and atrophy in distal limbs, but no sensory

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