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Biomedical subjects

A Dees

Publications and source records attributed to A Dees.

At least 19 recordsLinked to original sources

[Progressive multifocal leukoencephalopathy in a patient following long-term immunosuppressive therapy for systemic lupus erythematosus].

A 41-year-old woman who had suffered from systemic lupus erythematosus (SLE) for 22 years presented with signs of neurological deficits. CT-scanning of the brain revealed hypodense lesions that suggested cerebral infarction due to vasculitis in SLE. However, in spite of intensified immunosuppressive therapy, she showed rapid neurological deterioration. After extensive, additional examinations and tests, the diagnosis was finally changed to progressive multifocal leukoencephalopathy, caused by an opportunistic infection by the JC polyomavirus. Neurological and psychiatric symptoms frequently occur in patients with SLE. The differential diagnosis of these symptoms in SLE is extensive and includes, on the one hand, primary neurological and psychiatric diseases related to direct involvement of the nervous system by SLE, and on the other hand, secondary syndromes arising as a result of complications of the SLE or the immunosuppressive treatment. Opportunistic infections are often an important secondary cause of neurological and psychiatric syndromes in patients with SLE. The clinical symptoms and radiological cerebral signs are non-specific and usually do not suffice to differentiate between the various syndromes. Since each syndrome requires its own specific clinical approach and treatment, extensive diagnostics are mandatory before the diagnosis 'cerebral lupus' can be made and immunosuppressive therapy can be started or intensified.

Adult↗

Sporadic porphyria cutanea tarda due to haemochromatosis.

Haemochromatosis is a hereditary iron-overload syndrome caused by increased intestinal iron absorption and characterised by accumulation of potentially toxic iron in the tissues. Sometimes this disease presents as a cutanea porphyria. We describe a patient with joint complaints and blistering skin lesions on sun-exposed skin. After identifying the porphyria cutanea tarda by urine analysis we found that the serum activity of uroporphyrinogen decarboxylase (UROD) was normal, meaning a partial inactivation of UROD in liver tissue due to external factors. Further investigation showed the homozygous Cys282Tyr missense mutation and high levels of serum ferritin. It is important to recognise the symptoms of iron overloading at an early stage because hereditary haemochromatosis needs to be treated immediately. We therefore advocate routine sampling of ferritin levels in patients with unexplained joint complaints.

DNA↗

[Abnormalities in tattooed skin: sometimes sarcoidosis].

A 43-year-old man presented with a nodular tattoo lesion on his right upperarm. Histologically it resembled the granulomatous reaction seen in systemic sarcoidosis. Further evaluation revealed asymmetrical hilar lymphadenopathy with no interstitial lung disease. Since the patient was a heavy smoker, bronchus carcinoma could not be excluded and cervical mediastinoscopy was performed in order to obtain a lymph-node biopsy. This confirmed the diagnosis of systemic sarcoidosis. The patient was treated by local application of corticosteroids, but with little result. Skin lesions in scars or tattoos may be the first symptom of systemic sarcoidosis. Skin biopsy for histological confirmation of the diagnosis is recommended, as is further investigation to evaluate other organ systems which may be affected.

Administration, Topical↗

Severe hypokalaemic paralysis and rhabdomyolysis due to ingestion of liquorice.

Chronic ingestion of liquorice induces a syndrome with findings similar to those in primary hyperaldosteronism. We describe a patient who, with a plasma K+ of 1.8 mmol/l, showed a paralysis and severe rhabdomyolysis after the habitual consumption of natural liquorice. Liquorice has become widely available as a flavouring agent in foods and drugs. It is important for physicians to keep liquorice consumption in mind as a cause for hypokalaemic paralysis and rhabdomyolysis.

Candy↗

[A man from Surinam with haemorrhagic diarrhoea after long-standing schistosomiasis].

A 35-year-old man presented with a brief history of haemorrhagic diarrhoea. He had moved to The Netherlands 24 years before and had been in Surinam 12 years ago for the last time. Physical examination and routine laboratory tests revealed no abnormalities. Colonoscopy showed a striking congestion of the small blood vessels; histological examination of a sigmoid biopsy revealed Schistosoma eggs and microbiological investigation of the feces revealed ova of Schistosoma mansoni. The schistosomiasis was thus diagnosed more than 10 years after the last possible exposure to schistosomal cercariae. The patient was treated with a single dose of praziquantel.

Adult↗

Fluid homeostasis in chronic obstructive lung disease.

Chronic obstructive pulmonary disease (COPD) often leads to massive oedema and the development of what is usually called cor pulmonale. The mechanisms by which patients with COPD retain salt and water are not completely understood. Several abnormalities have been found including reduced renal blood flow with relatively preserved glomerular filtration rate and elevated levels of renin, aldosterone, arginine vasopressin and atrial natriuretic peptide. Generally, these abnormalities worsen with the severity of COPD and are most marked during the oedematous phases. Cardiac output is remarkably normal, suggesting that "cor pulmonale" is not primarily a cardiac disorder but rather a condition of volume overload due to activation of sodium-retaining mechanisms. The stimulus for this activation could be underfilling of the arterial system (reduced effective circulating volume) secondary to a fall in total peripheral vascular resistance. The latter is caused by hypercapnia-induced dilation of the precapillary sphincters. Apparently, the massive sodium retention by the kidney is not able to restore the circulating volume and a vicious cycle ensues ultimately leading to a clinical picture which resembles right-sided heart failure. Predictably, only blockade of the effects of carbon dioxide at the level of the precapillary sphincters would be able to halt this process.

Edema↗

Life-threatening hypokalaemia and quadriparesis in a patient with ureterosigmoidostomy.

We report quadriparesis as a result of severe hypokalaemia and acidosis in a 50-year-old man who had undergone ureterosigmoidostomy for bladder extrophy 48 years earlier. Aggressive suppletion with intravenous potassium and bicarbonate combined with potassium-sparing diuretics and ACE inhibitors resulted in complete restoration of the serum potassium and resolution of the neurological symptoms. The underlying mechanism as well as the treatment of hypokalaemia and hyperchloraemic metabolic acidosis after ureterosigmoidostomy are briefly discussed.

Acidosis, Renal Tubular↗

Extramedullary plasmacytoma of the breast simulating breast cancer.

A patient with a palpable mass in the breast suggestive of carcinoma underwent radical modified mastectomy. Surprisingly, histology of the tumor revealed an extramedullary plasmacytoma. Further diagnostic work up showed no evidence of underlying multiple myeloma. Among neoplastic lesions of the breast, although rare, malignancy of mesenchymal or lymphoproliferative origin should always be considered.

Aged↗

[Two patients with emphysematous pyelonephritis].

A 55-year-old man with diabetes mellitus was sick with shivering, abdominal pain and Escherichia coli in blood and urine cultures; a 40-year-old woman with asthmatic bronchitis had abdominal pain and anaemia. Both had renal dysfunction and the CT scan showed hydronephrosis with a perirenal inflammatory infiltrate and gas in the right and left renal pelvis, respectively. The man had a difficult recovery after treatment with antibiotics and percutaneous drainage. In the woman, a calculus obstructed the pyelo-ureteral passage; the resected kidney contained a squamous cell carcinoma. She was operated, received chemotherapy and recovered. Emphysematous pyelonephritis is a rare, severe disease. Percutaneous drainage and, if necessary, nephrectomy are paramount in the treatment. The condition is observed mostly in patients with diabetes mellitus or obstruction of the urinary tract.

Adult↗

The effect of balloon angioplasty on hypertension in atherosclerotic renal-artery stenosis. Dutch Renal Artery Stenosis Intervention Cooperative Study Group.

BACKGROUND: Patients with hypertension and renal-artery stenosis are often treated with percutaneous transluminal renal angioplasty. However, the long-term effects of this procedure on blood pressure are not well understood. METHODS: We randomly assigned 106 patients with hypertension who had atherosclerotic renal-artery stenosis (defined as a decrease in luminal diameter of 50 percent or more) and a serum creatinine concentration of 2.3 mg per deciliter (200 micromol per liter) or less to undergo percutaneous transluminal renal angioplasty or to receive drug therapy. To be included, patients also had to have a diastolic blood pressure of 95 mm Hg or higher despite treatment with two antihypertensive drugs or an increase of at least 0.2 mg per deciliter (20 micromol per liter) in the serum creatinine concentration during treatment with an angiotensin-converting-enzyme inhibitor. Blood pressure, doses of antihypertensive drugs, and renal function were assessed at 3 and 12 months, and patency of the renal artery was assessed at 12 months. RESULTS: At base line, the mean (+/-SD) systolic and diastolic blood pressures were 179+/-25 and 104+/-10 mm Hg, respectively, in the angioplasty group and 180+/-23 and 103+/-8 mm Hg, respectively, in the drug-therapy group. At three months, the blood pressures were similar in the two groups (169+/-28 and 99+/-12 mm Hg, respectively, in the 56 patients in the angioplasty group and 176+/-31 and 101+/-14 mm Hg, respectively, in the 50 patients in the drug-therapy group; P=0.25 for the comparison of systolic pressure and P=0.36 for the comparison of diastolic pressure between the two groups); at the time, patients in the angioplasty group were taking 2.1+/-1.3 defined daily doses of medication and those in the drug-therapy group were taking 3.2+/-1.5 daily doses (P<0.001). In the drug-therapy group, 22 patients underwent balloon angioplasty after three months because of persistent hypertension despite treatment with three or more drugs or because of a deterioration in renal function. According to intention-to-treat analysis, at 12 months, there were no significant differences between the angioplasty and drug-therapy groups in systolic and diastolic blood pressures, daily drug doses, or renal function. CONCLUSIONS: In the treatment of patients with hypertension and renal-artery stenosis, angioplasty has little advantage over antihypertensive-drug therapy.

Aged↗

[Hypertensive crisis: definition, pathophysiology and treatment].

Hypertensive crises are currently subdivided into hypertensive emergencies and urgencies depending on the acuteness with which the elevated blood pressure has to be lowered. Malignant hypertension, defined as severe hypertension and a hypertensive fundus grade III or IV, can present itself as an emergency or an urgency. For a hypertensive emergency intravenously acting blood pressure lowering agents are almost always required, whereas an urgency can usually be treated with oral agents. In view of the danger of cerebral hypoperfusion, blood pressure reduction during the initial treatment phase of a hypertensive crisis should not be more than 20 to 25%. Agents that exert a controllable blood pressure lowering action are preferred. Controllable blood pressure lowering cannot be achieved with nifedipine capsules. The practice of biting and swallowing a nifedipine capsule for the treatment of a hypertensive crisis therefore is to be discouraged.

Acute Disease↗

Late recurrence of liposarcoma simulating adenoma of the duodenum.

A large pedunculated, polypoid mass in the duodenum of a patient with asymptomatic anaemia, with mucosal biopsies indicating a villous adenoma, turned out to be a liposarcoma during laparotomy. The patient had had a completely resected retroperitoneal liposarcoma 8 years before. Liposarcoma recurrence should be highly suspected even in case of atypical presentation and long disease free interval.

Adenocarcinoma↗

[Hypertension with neurologic manifestations caused by an enlarged prostate].

A 74-year-old man suffering from hypertension had transient loss of strength in his left arm and leg. His severe hypertensive spells were caused by high-pressure chronic bladder retention. The patient had benign hyperplasia of the prostate for which transvesical enucleation was performed. The blood pressure then returned to normal. The transient neurological deficits had probably been caused by the abrupt blood pressure changes. In older male patients the possibility of this reversible cause of hypertension should be kept in mind.

Aged↗

Jaundice associated with acenocoumarol exposure.

A 69-year-old man developed cholestatic liver enzyme disturbances three and a half weeks after starting treatment with acenocoumarol because of a deep venous thrombosis in his leg. Serological testing showed no signs of recent viral infections. A presumptive diagnosis of hepatotoxicity caused by the use of acenocoumarol was made and the anticoagulant was replaced by low molecular weight heparin. Three weeks after withdrawal of the acenocoumarol, the enzymes had improved. The patient made a full recovery within two months. This case suggests a causal relationship between acenocoumarol exposure and liver damage.

Acenocoumarol↗

The Dutch Renal Artery Stenosis Intervention Cooperative (DRASTIC) Study: rationale, design and inclusion data.

RATIONALE: Renal artery stenosis may lead to renovascular hypertension, risking multiple organ damage including damage to the contralateral kidney. Progression of stenosis may impair the function of the affected kidney. It is important to identify individuals with this disease among hypertensive patients. The first aim of the Dutch Renal Artery Stenosis Intervention Cooperative (DRASTIC) study is to assess the prevalence of renal artery stenosis in patients with well-defined forms of drug-resistant hypertension, and to determine the predictive value of clinical characteristics and diagnostic tests in these pre-selected patients. With regard to treatment, the effect of renal angioplasty on hypertension is disappointing in atherosclerotic stenosis and technical failure frequently occurs. Therefore, the second aim is to compare the effects of balloon angioplasty and antihypertensive medication on blood pressure in patients with atherosclerotic renal artery stenosis. DESIGN HYPERTENSIVE: patients receiving standard antihypertensive medication in whom diastolic blood pressure remained > or =95 mmHg during three consecutive visits to the outpatient clinic underwent full diagnostic work-up, including renal arteriography. The prevalence of renal artery stenosis in this well-defined patient group was then established, and the predictive value of the various diagnostic tests was assessed. Patients with an atherosclerotic renal artery stenosis of > or =50% were then randomly assigned to balloon angioplasty or to treatment with antihypertensive drugs. After 1 year of intensive follow-up of blood pressure and renal function, re-arteriography was performed. CONCLUSION: In total, 1205 patients have been included in the study, about 500 have received diagnostic work-up, and it is expected that 100 patients will be randomly assigned for renal angioplasty or medical treatment.

Adolescent↗