Cases reported "Chronic Disease"

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1/118. Aortic dissection in young patients with chronic hypertension.

    We describe four patients aged 14 to 21 years who developed acute aortic dissection. In three of the four patients, the course was fatal, despite aggressive medical and surgical intervention. All four patients had sustained systemic hypertension related to chronic renal insufficiency. The patients had no other identifiable risk factors for aortic dissection, including congenital cardiovascular disease, advanced atherosclerosis, vasculitis, trauma, pregnancy, or family history of aortic dissection. Although aortic dissection is rare in individuals younger than 40 years of age, young patients with sustained systemic hypertension are at increased risk for this serious and often fatal condition. physicians must be aware of this rare complication of hypertension and consider aortic dissection in the differential diagnosis of unusual chest, abdominal, and back pain in hypertensive children, adolescents, and young adults.
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2/118. Renal granulomatous sarcoidosis in childhood: a report of 11 cases and a review of the literature.

    We analysed retrospectively 11 children with renal granulomatous sarcoidosis confirmed by renal histology in order to describe the course and prognosis of the disease. Symptomatic sarcoidosis was diagnosed at a mean age of 10.1 years. Nine children had renal involvement at the time of diagnosis. In the course of the disease, nine patients developed renal failure and mild proteinuria, seven had transient sterile leukocyturia, four showed microscopic haematuria, seven had a urinary concentrating defect, and enlarged kidneys were seen in three patients. One child had hypercalcaemia and hypercalciuria, none had hypertension. light microscopy of the kidney showed interstitial infiltration by mononuclear cells in all children, interstitial fibrosis in nine patients, epithelioid granulomas in seven, tubular involvement in eight, and mild glomerular involvement in seven patients. Renal immunofluorescence was negative. Ten children received prednisone for 1-11 years. After a mean follow up of 5.5 years, three patients had entered end-stage renal failure and one had chronic insufficiency after interruption of medical supervision and prednisone therapy. CONCLUSION: Renal failure, proteinuria, leukocyturia, haematuria, and concentration defect are the prominent features of renal granulomatous sarcoidosis in children. Steroid therapy, adjusted according to disease activity, may prevent end-stage renal failure.
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3/118. The development of a pancreatic abscess, suppurative pylethrombosis, and multiple hepatic abscesses after a pancreatojejunostomy for chronic pancreatitis: report of a case.

    We present herein an autopsy case of 63-year-old Japanese man who died as a result of pancreatic abscess, suppurative pylethrombosis, and multiple liver abscesses that had developed 10 years after a pancreato- and cystojejunostomy with side-to-side anastomosis for chronic pancreatitis. Even after this operation, the patient had continued to consume excessive amounts of alcohol. He had first experienced back pain with leukocytosis 9 years after the operation, which relapsed the following year. Despite percutaneous transhepatic gallbladder drainage, his icterus had deteriorated into hepatic insufficiency. Computed tomographic scans of the abdomen had disclosed multiple liver abscesses. At autopsy, a pancreatic abscess and suppurative pylethrombosis as well as multiple liver abscesses were found. There have been few reported cases of such lethal complications developing after a pancreato- and cystojejunostomy for chronic pancreatitis. As the consumption of alcohol would have exacerbated the chronic pancreatitis, such patients should be strongly advised to abstain from drinking alcohol.
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4/118. Acute and chronic venous insufficiency in the finger.

    Venous hypertension with subsequent chronic venous insufficiency and its sequelae in the hand is reported as an uncommon complication of arteriovenous fistulae for hemodialysis.
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ranking = 3377.4346178497
keywords = venous insufficiency, insufficiency
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5/118. Maxillofacial osteonecrosis in a patient with multiple "idiopathic" facial pains.

    Previous investigations have identified focal areas of alveolar bone tenderness, increased mucosal temperature, abnormal anesthetic response, radiographic abnormality, increased radioisotope uptake on bone scans, and abnormal marrow within the quadrant of pain in patients with chronic, idiopathic facial pain. The present case reports a 53-year-old man with multiple debilitating, "idiopathic" chronic facial pains, including trigeminal neuralgia and atypical facial neuralgia. At necropsy he was found to have numerous separate and distinct areas of ischemic osteonecrosis on the side affected by the pains, one immediately beneath the major trigger point for the lancinating pain of the trigeminal neuralgia. This disease, called NICO (neuralgia-inducing cavitational osteonecrosis) when the jaws are involved, is a variation of the osteonecrosis that occurs in other bones, especially the femur. The underlying problem is vascular insufficiency, with intramedullary hypertension and multiple intraosseous infarctions occurring over time. The present case report illustrates the extreme difficulties involved in the diagnosis and treatment of this disease.
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6/118. Approaching the patient with chronic malabsorption syndrome.

    The causes of chronic malabsorption may be categorized as decreased intestinal absorption, most commonly caused by celiac sprue; or maldigestion caused by pancreatic insufficiency. The initial step in the evaluation of these patients should include stool studies to confirm fat malabsorption. If fat malabsorption is confirmed, endoscopy with small-bowel biopsies and aspirates for bacterial culture usually follows. A normal endoscopic examination should lead to assessment of pancreatic function. In the setting of normal pancreatic function and the absence of bile acid deficiency, a barium radiograph of the small bowel should be made, looking for anatomical abnormalities. Celiac sprue is an intolerance to gluten caused by a combination of genetic, environmental, and immunologic factors. It classically causes malabsorption. However, it is likely that many patients who exhibit only minor manifestations of the disease go unrecognized and untreated. A presumed diagnosis of celiac sprue is confirmed after a clinical and endoscopic response to a gluten-free diet. Serological markers are available with high degrees of sensitivity and specificity, but duodenal biopsy remains the gold standard for diagnosis. A minority of patients are unresponsive to a gluten-free diet, and intestinal lymphoma should be suspected in these cases.
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7/118. Treatment of giant aortic aneurysm with tracheal compression and sternal erosion without circulatory arrest.

    Treatment of huge aneurysms involving the ascending aorta and the aortic arch with compression of the surrounding structures represents a surgical challenge. The case of a patient affected by respiratory insufficiency and sternal erosion caused by chronic giant aortic aneurysm is reported. The use of a stepwise approach and selective cerebral arterial perfusion ensured successful operative management, avoiding circulatory arrest and enabling an expeditious postoperative recovery.
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keywords = insufficiency
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8/118. serum itraconazole and hydroxyitraconazole concentrations and interaction with digoxin in a case of chronic hypertrophic pachymenigitis caused by aspergillus flavus.

    A patient treated with itraconazole (ITCZ) under the diagnosis of aspergillus flavus-induced chronic hypertrophic pachymeningitis is presented. The reason for the successful cure of this patient was investigated by the pharmacokinetic analysis of serum levels of ITCZ. Concurrently administered digoxin was also investigated for its drug-drug interaction. The patient (a 75-year-old male) developed ophthalmopathy, and was diagnosed as having A. flavus hypertrophic pachymeningitis by pachymeninx biopsy. After admission, he was treated with FLCZ, AMPH, 5-FC and MCZ. The infection tended to subside with the AMPH administration. Since renal insufficiency was induced by AMPH and the other antifungal drugs were ineffective, daily administration of 200 mg of ITCZ was initiated, and the inflammatory signs and symptoms gradually subsided. The symptoms did not recur during the 36 months of itraconazole treatment after discharge, and it was concluded that ITCZ was effective for A. flavus hypertrophic pachymeningitis. Pharmacokinetic parameters of ITCZ and OH-ITCZ as follows: ITCZ: Cmax 93.2 ng/ml, T1/2 beta 11 hours, AUC0-24 999 ng.h/ml, OH-ITCZ: Cmax 159.4 ng/ml, T1/2 beta 16. 2 hours, AUC0-24 of 1391 ng.h/ml. Both ITCZ and OH-ITCZ reached steady states seven days after administration began. The ITCZ and OH-ITCZ levels in serum collected 36 months after the initiation of administration were 452.9 ng/ml and 1233.6 ng/ml, respectively. Cmax and AUC0-24 of ITCZ and OH-ITCZ on the second day were markedly lower than those in healthy adults reported by Oguchi et al., and hypoalbuminemia observed at administration on that day was considered the most probable cause. It was assumed that the most plausible reason for a successful cure even at a low dose of ITCZ was the increase of distribution to tissue by the increase of the unbound form. digoxin was concurrently given to this patient at 0. 125 mg/day, but the blood digoxin level was not elevated. Consideration of the blood level of albumin is believed to be important for evaluating the blood concentration of ITCZ.
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keywords = insufficiency
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9/118. Acquired segmental megacolon in an adult patient with cystic fibrosis.

    cystic fibrosis (CF) is characterized by symptoms related to pulmonary dysfunction and pancreatic insufficiency. constipation, though a frequent complaint of patients with CF, receives less attention. We report a case of acquired segmental megacolon and constipation necessitating surgical colonic resection in an adult patient with CF. The differential diagnosis and possible causes of megacolon in this setting are discussed.
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ranking = 1
keywords = insufficiency
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10/118. Acute adrenal insufficiency after cardiac surgery.

    adrenal insufficiency after cardiac surgery can easily be confused during the course of an immediate unstable postoperative period. If unrecognized, this condition may cause serious morbidity and can be fatal. We report on a 43-yr-old female patient with chronic known adrenal insufficiency, who, despite her adequate preoperative replacement therapy, presented with one episode of acute hypoadrenal crisis after elective open heart surgery, which could serve as a model to illustrate the salient clinical features and possible problems in this setting for diagnosing this problem to patients in whom chronic adrenal insufficiency remains unknown.
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ranking = 7
keywords = insufficiency
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