Clinical problem-solving. Testing, testing, testing....
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Adrenal Cortex Hormones↗
Biomedical subjects
E Ben-Chetrit
Publications and source records attributed to E Ben-Chetrit.
Familial Mediterranean fever in the "Chuetas" of Mallorca--origin in inquisition?
The aim of our study was to compare the features of familial Mediterranean fever (FMF) in Mallorcan "Chuetas" with those in non-Ashkenazi Jews in Israel. The clinical and laboratory data of FMF were evaluated in a recently identified cluster of 50 FMF patients from Mallorca (the Chuetas) and 45 patients from Israel. We found that the prevalence and clinical manifestations of FMF were similar among the Chuetas and the Israeli group. Furthermore, in contrast to other ethnic groups with FMF, joint involvement was quite common in both the Chuetas (70%) and the Israeli group (75%). The Chuetas are descendants of Mallorcan Jews who emigrated from Spain to the island in the 12th century. The non-Ashkenazi Jews originated mainly in North Africa and are descendants of refugees who escaped from Spain as a result of the Inquisition in the 15th century. We suggest that the non-Ashkenazi Jews and the Chuetas may have a common gene defect for FMF.
Adolescent↗
Clinical problem-solving. Still hazy after all these years.
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Abdominal Pain↗
Pulmonary embolism as the presenting feature of hepatocellular carcinoma.
Primary hepatocellular carcinoma can be revealed by recurrent pulmonary embolism as observed in this case of a 63-year-old woman initially hospitalized for abdominal pain and shortness of breath. The clinical diagnosis was confirmed by laboratory findings, a ventilation perfusion scan and pulmonary angiography which demonstrated peripheral basal artery cut-off and slow filling with delayed washout. The patient was treated with heparin then with nicoumarol and responded well. One month after discharge the patient again complained of shortness of breath and was readmitted. Anticoagulation was adequate as evidenced by a prothrombin time of 1.39 INR and the physical examination and laboratory tests again suggested pulmonary emboli, confirmed by a ventilation perfusion scan. Computed tomography of the chest and abdomen revealed multiple hypodense masses filling half of the liver volume and needle biopsy led to the diagnosis of hepatocellular carcinoma. Hypercoagulability in malignancy is well-known although cases of migratory thrombophlebitis are extremely rare. Pulmonary embolism has not been described as a presenting feature of hepatocellular carcinoma. In this case, there was no evidence of hepatic dysfunction and the pulmonary embolism occurred despite adequate anticoagulation. Clinicians should include occult carcinoma among the possible causes of recurrent pulmonary embolism and when searching for malignancy can include hepatocellular carcinoma among the causes of hypercoagulation.
Carcinoma, Hepatocellular↗
Systemic lupus erythematosus induced by ovulation induction treatment.
Infertile women are treated with various regimens for ovulation induction. The ultimate end-result of these treatments is a significant rise in levels of serum gonadotropins and estradiol--the most potent natural estrogen. Estrogens may affect diverse biologic functions, including immune and inflammatory reactions. A role for estrogens in the development or exacerbation of systemic lupus erythematosus (SLE) has been suggested by many studies. In this report, we present 3 cases of otherwise healthy women who received ovulation induction agents and subsequently developed full-blown SLE. The possible association between this treatment and SLE is discussed.
Adult↗
Colchicine disposition in patients with familial Mediterranean fever with renal impairment.
OBJECTIVE: To assess the pharmacokinetics of serum colchicine in patients with familial Mediterranean fever (FMF) with renal impairment. METHODS: Using a specific radioimmunoassay we determined serum colchicine concentration at various time points following oral administration of a single dose of the drug. RESULTS: Patients with renal insufficiency had a mean +/- SD apparent total colchicine clearance of 0.168 +/- 0.063 l/h/kg, apparent volume of distribution of 4.56 +/- 1.64 l/kg and elimination half-life (t1/2) of 18.8 +/- 1.2 h. Patients with FMF with normal kidney function had a mean clearance of 0.726 +/- 0.110 l/h/kg, volume of distribution of 4.87 +/- 2.05 l/kg and terminal t1/2 of 4.4 +/- 1.0 h. CONCLUSION: The kidneys have an important role in the clearance of colchicine. Caution should be exercised in the use of colchicine in patients with renal insufficiency.
Administration, Oral↗
Transient neutropenia induced by intravenous immune globulin.
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Adult↗
Poliovaccines and the course of systemic lupus erythematosus--a retrospective study of 73 patients.
Flare in disease activity occurred in four out of 73 patients (5%) under the age of 45 with systemic lupus erythematosus (SLE) who were immunized against poliomyelitis in a nationwide campaign following the Israeli outbreak of 1988. The flare appeared within 3 months of the immunization, whereas in 37 matched SLE patients who were not immunized, the disease remained stable in the same period. Both oral live-attenuated (OPV) and injected killed poliovaccines (IPV) were implicated in the flares (1/24 and 3/49 patients, respectively). The study was based on detailed questionnaires and confirmed by telephone calls and examination of the patients' hospital medical records. Poliovaccines, including IPV, should not be administered to SLE patients without careful consideration and a further prospective study should be undertaken to determine their safety in SLE.
Adult↗
Palmar rheumatoid nodulosis associated with local pressure.
Rheumatoid nodulosis is a term used to describe adult patients with rheumatoid arthritis with little or no clinical joint inflammation who have numerous subcutaneous nodules indistinguishable from those of patients with active rheumatoid arthritis. This paper reports the case of a woman with quiescent rheumatoid arthritis who developed palmar nodulosis three weeks after the strenuous activity of painting her apartment. This case illustrates the direct association between the appearance of nodulosis and physical pressure despite inactive disease.
Adult↗
Thrombosed hemorrhoid mimicking rectal carcinoma at CT.
A 46-year-old man with cirrhosis and portal hypertension complained of lower pelvic pain. CT of the rectum raised a strong suspicion of a rectal tumor. However, rectal examination, anoscopy, direct rectoscopy, and, unfortunately, post-mortem dissection, failed to confirm its existence. Nevertheless, large flat hemorrhoids were evident. Review of the patient's chart disclosed the presence of large thrombosed hemorrhoids detected by rectal examination prior to the CT examination. It is suggested that rectal hemorrhoids be included in the differential diagnosis of rectal tumor shown by CT in patients with portal hypertension.
Diagnosis, Differential↗
Acute colchicine intoxication--possible role of erythromycin administration.
A 29-year-old patient with familial Mediterranean fever and amyloidosis involving the kidney, liver, and gastrointestinal tract received longterm colchicine, 1 mg daily. In the last year she developed diarrhea and abdominal pain, that coincided with toxic colchicine blood levels. After 2 weeks of oral erythromycin therapy she was hospitalized for acute, life threatening colchicine toxicity, with fever, diarrhea, abdominal pain, myalgia and lower extremity parasthesias and later convulsions and alopecia. Pancytopenia evolved into rebound leukocytosis, disturbed liver function and hypoglycemia. After a long stormy course she improved. Colchicine toxicity with combined liver and renal impairment and the role of erythromycin in her colchicine toxicity are discussed.
Acute Disease↗
Familial erythema nodosum.
Erythema nodosum (EN) is a hypersensitivity reaction associated with many diseases. We describe a family in which 4 sisters had acute or recurrent EN. HLA typing showed a common haplotype in the affected members of the family. A review of familial EN and HLA distribution in EN is presented.
Acute Disease↗
Inappropriate secretion of antidiuretic hormone in Sheehan's syndrome: a rare cause of postpartum hyponatremia.
A 27-year-old woman experienced hemorrhagic shock after delivery. One week later she was seen in an obtunded state of consciousness. The results of laboratory evaluation were consistent with the syndrome of inappropriate antidiuretic hormone secretion caused by hypopituitarism. Hydrocortisone rapidly corrected sodium levels. Syndrome of inappropriate secretion of antidiuretic hormone caused by Sheehan's syndrome should be considered in the differential diagnosis of postpartum hyponatremia.
Adult↗
Colchicine intoxication: clinical pharmacology, risk factors, features, and management.
The use of colchicine for acute gouty arthritis dates to ancient times. In recent years, colchicine also has been used successfully for various other rheumatic and nonrheumatic conditions. Colchicine is a safe drug when used according to established therapeutic guidelines. However, toxicity can be considerable if ingested intentionally or if the recommended doses are exceeded. Colchicine intoxication is characterized by multi-organ involvement and by the poor prognosis associated with administration of large amounts of the drug. Therapy is basically supportive and symptomatic because of the rapid distribution and binding of colchicine to the affected tissues. Use of anticolchicine antibodies is a novel approach that has shown promise in experimental models. Important research questions pertain to the effect of liver and kidney disease on colchicine metabolism, use of colchicine levels in the diagnosis of intoxication and for prognostication, and application of immunotoxicotherapy for colchicine poisoning in humans.
Animals↗
Colchicine prophylaxis in familial Mediterranean fever: reappraisal after 15 years.
As determined in this study of 45 patients, the prolonged use of colchicine therapy in familial Mediterranean fever (FMF) is safe and effective in preventing flares of FMF and amyloidosis. It has acceptable adverse effect profile and can be used in children and pregnant women. Its discontinuation predisposes patients to acute FMF attacks and the development of amyloidosis. Articular involvement is less responsive to colchicine and may require therapy with nonsteroidal antiinflammatory drugs.
Adolescent↗
Etiology, treatment, and prognosis of large pericardial effusions. A study of 34 patients.
During the last 20 years, only a few studies have been published concerning large pericardial effusion. We recently reviewed 34 patients who presented with large pericardial effusion not associated with trauma. Our analysis revealed that half of the patients (52 percent) had pericardial effusion of unknown origin. Four patients had postmyocardial infarction pericardial effusion, three had associated malignant neoplasms, three suffered from collagen diseases, and two had infectious agents. Uremia and irradiation accounted for a single case each. Twenty-seven (79 percent) of the patients underwent pericardiocentesis and two (5.8 percent) had a pericardial window operation. The overall prognosis of the patients was excellent.
Adult↗
Acute pericarditis: etiology, treatment and prognosis. A study of 115 patients.
One hundred and 15 hospitalized patients with acute pericarditis were analyzed retrospectively for their etiology, management and long-term prognosis. It was found that most of the patients had either idiopathic or viral etiologies (60%), collagen disease (16.4%) or malignancy (6.9%). Most of the patients were treated with non-steroidal anti-inflammatory drugs (NSAID). Twenty-six patients (22%) required corticosteroids following NSAID treatment failure. Only one patient underwent pericardiocentesis for tuberculous pericarditis. The long-term prognosis was good, although 21.9% of the patients suffered from recurrent episodes of pericarditis. It is concluded that in hospitalized patients with pericarditis, an extensive workup may not reveal the major etiologies, and the disease may be more complicated than previously thought.
Acute Disease↗
Lupus refractory pleural effusion: transient response to intravenous immunoglobulins.
A patient with systemic lupus erythematosus complicated by refractory bilateral pleural effusions is described. High dose corticosteroids with azathioprine, as well as intrapleural instillation of corticosteroids, proved ineffective in management. As our patient remained severely symptomatic and required repeated thoracocentesis, a therapeutic trial of intravenous immunoglobulins (IVIG) was attempted. IVIG had a beneficial effect, although of a transient and partial nature. Despite the results achieved, it seems that IVIG has limited value in treating lupus pleural effusion.
Adult↗