Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: EC:3.4.23.15 (renin)
 35,795 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A case of polyarteritis nodosa (PN) in childhood involving various organs such as the gastrointestinal tract, skin, CNS, kidneys and liver with hypogammaglobulinemia is reported. This 6 month old girl was admitted to our hospital with vomiting, diarrhea, bloody stools with mucous and weight loss. For the past 5 months she had these abdominal symptoms. She was diagnosed as having PN of the Kussmaul-Maier variety on the grounds of the biopsy of skin lesion where a necrotizing vasculitis was found. Prednisolone and methylprednisolone pulse treatment were not effective in suppressing the progress of the disease. At the age of 1 year 7 month a combination therapy of prednisolone and immunosuppressants (cyclophosphamide) was started and this was found to be effective. She was discharged when she was 2 year and 2 month. The dosage of prednisolone was tapered as the activity of the PN decreased and she did well with a maintainance dosage of 9.5 mg/day. At 3 year 6 month of age she suddenly developed hypertension (the plasma renin activity was found to be 16.6 ng/m/hr. and the aldosterone 220 ng/dl). CNS involvement such as spinal cord dysfunction, left sided convulsions, cerebral hemorrhage developed 5 months later. Methylprednisolone pulse therapy was performed 3 times and 2 mg/kg/day of prednisolone was administered. In spite of this therapy she passed away with a massive cerebral hemorrhage at the age of 4 year 8 month. Unfortunately an autopsy was not performed. Results of the immunological tests proved that the hypogammaglobulinemia was a common variable immunodeficiency (CVI). It has been reported that primary immuno-deficiency syndrome is often associated with collagen disease and auto-immune disease. This lack of the defense mechanism against the virus or extra antigen could be related to the onset of collagen and auto-immune disease. As the correlation between CVI and PN has not been clarified this case is of interest as concerns the cause of PN.
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PMID:[A case of hypogammaglobulinemia associated with polyarteritis nodosa presenting a variety of symptoms in childhood]. 197 16

The present case is a 5-month-old female with atopic dermatitis who was brought to hospital for growth failure noted upon regular health examination. Laboratory examinations revealed hyponatremia, hyperkalemia, hypoproteinemia, hypogammaglobulinemia, elevated plasma renin activity and hyperaldosteronemia. Immune function was normal. Composition of the exudate collected from the skin lesions of atopic dermatitis was similar to that of plasma. Application of a steroid ointment improved the lesions as well as all laboratory values. These findings indicate that voluminous exudation caused by extensive atopic dermatitis can lead to hypotonic dehydration, electrolyte abnormalities, hypoproteinemia, hypogammaglobulinemia and, finally, to growth failure in infants. We conclude that intensive treatment is important for severe atopic dermatitis in infants to prevent serious complications.
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PMID:Hyperaldosteronemia and hypogammaglobulinemia secondary to atopic dermatitis-induced exudation in an infant presenting with growth failure. 2131 51