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Пульмонология, гематология (англ,рус)

·        Diagnosis is clinical in children but usually warrants biopsy in adults. Disease is usually self-limited.

·        Corticosteroids can relieve arthralgias and GI symptoms but do not alter the course of the disease. Progressive glomerulonephritis may require high-dose corticosteroids and cyclophosphamide.

 

Symptoms and Signs of HSP

·        The disease begins with a sudden palpable purpuric rash typically occurring on the feet, legs, and arms and as a strip across the buttocks. The purpura may start as small areas of urticaria that become indurated and palpable. Crops of new lesions may appear over days to several weeks. Many patients also have fever and polyarthralgia with periarticular tenderness and swelling of the ankles, knees, hips, wrists, and elbows.

·        GI symptoms are common and include colicky abdominal pain, abdominal tenderness, and melena.

·        Symptoms usually remit after about 4 wk. In most patients, the disorder subsides without serious sequelae; however, some patients develop chronic renal failure.

 

Diagnosis of HSP

·        Biopsy of skin lesions

·        The diagnosis is suspected in patients, particularly children, with typical skin findings. It is confirmed by biopsy of skin lesions when leukocytoclastic vasculitis with IgA in the vessel walls is identified.

·        Urinalysis is done; hematuria, proteinuria, and RBC casts indicate renal involvement.

·        If renal function is deteriorating, renal biopsy may help define the prognosis. Diffuse glomerular involvement or crescent formation in most glomeruli predicts progressive renal failure.

 

Treatment of HSP

treatment is symptomatic. Corticosteroids (prednisone 2 mg/kg up to a total of 50 mg po once/day) may help control abdominal pain and are occasionally needed to treat severe joint pain or renal disease. Pulse IV methylprednisolone followed by oral and cyclophosphamide can be given to attempt to control inflammation when the kidneys are severely affected. However, the effects of corticosteroids on renal manifestations are not clear.

 

 

 

Autoerythrocyte Sensitization (Gardner-Diamond Syndrome)

·        Autoerythrocyte sensitization is a rare disorder affecting women. It is characterized by local pain and burning preceding painful ecchymoses that occur primarily on the extremities.

·        In women with autoerythrocyte sensitization, intradermal injection of 0.1 mL of autologous RBCs or RBC stroma may result in pain, swelling, and induration at the injection site.

·        Diagnosis is based on examination of the site of intradermal injection of autologous RBCs and of a separate control injection site (without RBCs) 24 to 48 h after injection.

 

Dysproteinemias Causing Vascular Purpura

Amyloidosis causes amyloid deposition within vessels in the skin and subcutaneous tissues, which may increase vascular fragility, producing purpura. In some patients, coagulation factor X is adsorbed by amyloid and becomes deficient, but this usually is not the cause of bleeding. Periorbital purpura or a purpuric rash that develops in a nonthrombocytopenic patient after gentle stroking of the skin suggests amyloidosis.

 

Hereditary Hemorrhagic Telangiectasia   (Rendu-Osler-Weber Syndrome)

·        Hereditary hemorrhagic telangiectasia is a hereditary disease of vascular malformation transmitted as an autosomal dominant trait affecting men and women.

·        telangiectatic lesions on the face, lips, oral and nasal mucosa, and tips of the     fingers and toes.

·        Some patients have pulmonary arteriovenous fistulas. These fistulas may produce significant right-to-left shunts, which can result in dyspnea, fatigue, cyanosis, or polycythemia.

·        However, the first sign of their presence may be a brain abscess, transient ischemic attack, or stroke as a result of infected or noninfected emboli. Cerebral or spinal arteriovenous malformations occur in some families and may cause subarachnoid hemorrhage, seizures, or paraplegia.

Treatment

·        Treatment for most patients is supportive, but accessible telangiectases (in the nose or GI tract via endoscopy) may be treated with laser ablation.

·        Arteriovenous fistulas may be treated by surgical resection or embolotherapy.

·        Iron therapy

 

Prepared by Orlovsky A. V., assistant, D.Ph.,

                    Murenets N. A., postgraduate

 

 

 

"Пульмонология, гематология"

 

1.                  

 

 

Короткий теоретико материал

 

Хроническая обструктивная болезнь легких (ХОБЛ)

 

Хроническая обструктивная болезнь легких является международно предпочел термин, охватывающий хронический бронхит и эмфизему.

По ХОБЛ определение является хроническим, медленно прогрессирующее заболевание характеризуется обструкцией дыхательных путей (ОФВ1 <80% от должного и FEV 1 / VC соотношение <70%), которые заметно не изменится в течение нескольких месяцев. Нарушения функций легких в значительной степени фиксированной, но может быть частично обратимым по бронхолитической терапии.

 

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