تصفح
دخول
تسجيل
نسيتها؟

end-stage renal disease, painful skin lesions, diffuse pulmonary infiltrates, and respiratory failure


end-stage renal disease, painful skin lesions, diffuse pulmonary infiltrates, and respiratory failure


حالة سريرية

الوصف الكامل Background

HISTORY — A 20-year-old man with end-stage renal disease and a painful, violaceous, plaque-like skin eruption over the lower extremities was admitted to the hospital. His past medical history was remarkable for a rapidly progressive glomerulonephritis, an unsuccessful cadaveric renal transplant, and poorly controlled hypertension. Shortly after admission, he developed respiratory distress requiring mechanical ventilation. Over the next few weeks, his skin lesions spread to the abdomen and back and became ulcerative and necrotic, forming eschars. The patient eventually required tracheotomy for chronic ventilator support.

HOSPITAL COURSE — Later in the hospitalization, the serum calcium increased to 14 mg/dL, and a serum mid-chain parathyroid hormone level was increased at 246 pg/mL. A skin biopsy revealed diffuse subcutaneous and vascular calcification with in situ thrombosis. A bone scan is shown

الفحص السريري Clinical Exam

PHYSICAL EXAMINATION — Afebrile; respirations 24 on the ventilator; blood pressure 100/90. General: chronically ill appearing. Chest: scattered rhonchi. Cardiac examination: II/VI systolic ejection murmur at the left sternal border. Skin: multiple plaque-like lesions with areas of necrosis, ulceration, and eschars.

التشخيص التفريقي DD

???

الاستقصاءات Investigations

LABORATORY FINDINGS — Hct 19.7 percent; WBC 14,500/mm3. Na+ 125 mEq/L; K+ 3.6 mEq/L; Cl - 89 mEq/L; HCO3 -16 mEq/L; BUN 95 mg/dL; creatinine 10.3 mg/dL; phosphate 6.5 mg/dL; calcium 7.2 mg/dL. Chest radiograph : diffuse infiltrates. Right heart catheterization: PAOP 12 mmHg; CI 3.7 L/min/m2; SVR 1170 dyne sec cm -5.

التدبير Managment

???

كتابة حرة وطرح موضوع النقاش!

QUESTION — What is the cause of the patient's respiratory failure?

by
السنة الخامسة

Note:
the 1st pic is normal CXR
the 2nd 20_yo_with_ESRD_bone_scan
the 3rd 20_yo_with_ESRD_PA
lifespring
السنة الخامسة


Discussion Diffuse tissue calcification occurs in two distinct clinical presentations: calciphylaxis and systemic calcinosis. In 1962, Seyle coined the term calciphylaxis tissue calcification to describe a process of soft tissue calcification and necrosis produced in laboratory animals when an appropriate biochemical milieu was present with "sensitizing" and "precipitating" influences. Sensitizing factors, which include azotemia, elevated parathyroid hormone, increased calcium and phosphate product, and vitamin D compounds, create an environment for tissue calcification. Precipitating influences are thought to initiate the process and include elevated parathyroid hormone, ionized calcium, intravenous albumin, warfarin, corticosteroids and immunosuppressants.

Calcification occurs in subcutaneous connective tissue, including small and intermediate blood vessels. Subsequent tissue ischemia occurs, and patients develop painful violaceous mottling of the skin (livedo reticularis) that can progress to well-demarcated plaques, ulcers, and eschar formation. Mortality is high, with uncontrollable sepsis being the usual lethal event. Systemic calcinosis refers to deposition of calcium in the connective tissue of various organs, including the lungs, kidney, stomach, heart, and skin.

In contrast to calciphylaxis, patients with systemic calcinosis are usually asymptomatic. The lack of an inflammatory reaction to calcium deposition may be in part due to its smaller crystal decomposition when compared to calcification in calciphylaxis. Pulmonary calcinosis, at least on a microscopic level, appears common in chronic renal failure. Calcification occurs in the alveolar septa, alveolar wall, bronchial submucosa, and the pulmonary arterioles. Most cases are clinically silent, with patients being asymptomatic and having normal chest radiographs. With extensive calcification, the chest radiograph may show a persistent, dense alveolar infiltrate. Pulmonary function may be reduced, and symptoms may progress from long periods of relative quiescence to respiratory failure.

The differential diagnosis of pulmonary calcinosis includes pulmonary alveolar microlithiasis, granulomatous disease, mitral stenosis, ectopic parathyroid hormone-producing tumors, bone-destroying tumors, and other conditions associated with hypercalcemia (milk-alkali, vitamin D intoxication, and primary, secondary and tertiary hyperparathyroidism). Computerized tomography, gallium scan and technetium-99 methylene diphosphonate bone scan have used to screen patients in whom systemic calcinosis is suspected. Lung biopsy may be required for a definitive diagnosis. Elimination of any known precipitating or sensitizing influences is the cornerstone of therapy for calciphylaxis and systemic calcinosis.

The present patient had clinical and laboratory features of pulmonary calcinosis and calciphylaxis from secondary and tertiary hyperparathyroidism. After parathyroidectomy, the patient' s calcium and phosphate product was lowered, and he underwent successful weaning from the ventilator.

lifespring
السنة الخامسة


Shocked Evil Evil Twisted Evil Crying or Very sad Question Exclamation Mad confused Sad Cool Very Happy

lifespring
السنة الخامسة
ابق على تواصل مع حكيم!
Google+