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Biomedical subjects

Z Farfel

Publications and source records attributed to Z Farfel.

At least 55 records · Page 3Linked to original sources
PubMed1995

Prolonged therapy by the combination of furosemide and thiazides in refractory heart failure and other fluid retaining conditions.

The efficacy and side effects of the combination therapy of thiazide and furosemide administered to patients with refractory heart failure, for a prolonged period of time, were assessed. Thirty-two patients were hospitalized during the years 1985-1991. Left heart failure (left ventricular ejection fraction (LVEF = 22.4% +/- 6.6%) was present in 26 patients, right heart failure in 3 patients, chronic renal failure, cirrhosis and bilateral pleural effusion were present each in one patient. Chlorothiazide 0.5 g daily was added to conventional therapy. Patients were monitored closely during hospitalization and later as outpatients. During hospitalization, addition of chlorothiazide caused a reduction of 4.8 +/- 4.0 kg in patients' weight, serum potassium decreased from 4.4 +/- 0.6 to 4.0 +/- 0.5 mmol/l (P < 0.005) and serum sodium from 139.0 +/- 4.7 to 136.8 +/- 5.5 mmol/l (P < 0.05). The duration of the combined therapy was 17.2 +/- 19.1 months. Thirteen patients had short treatment (1.6 +/- 0.8 months) and 19 patients had prolonged treatment (26.5 +/- 19.0 months). No specific characteristics distinguished patients in both groups. Thiazides were discontinued in 19 patients, 10 of which had side effects. In only 5 of the 19 patients treated for the prolonged period had thiazides to be discontinued because of side effects. Addition of thiazides to furosemide is efficacious in severe heart failure. The combination should be started during hospitalization. Many patients can be maintained on this combination for a prolonged period of time on an ambulatory basis.

Aged↗
PubMed1995

[Oculopharyngeal muscular dystrophy with dysphagia in a Jewess from Uzbekistan].

An 82-year-old Jewish woman from Tashkent, Uzbekistan was hospitalized because of dysphagia and weight loss. Physical examination revealed bilateral ptosis. The diagnosis of the rare inherited disease oculopharyngeal muscular dystrophy was confirmed by electron microscopy of biopsied muscle. It has recently been found that the prevalence of the disease among Jews from Uzbekistan is very high. It should therefore be considered when such patients present with dysphagia, so invasive imaging procedures can be avoided.

Aged↗
PubMed1994

Exacerbation of Wegener's granulomatosis during pregnancy: report of a case with tracheal stenosis and literature review.

Wegener's granulomatosis (WG) is a rare multisystem disease of unknown etiology. We describe a woman with WG who relapsed twice during 2 pregnancies with appearance of tracheal stenosis. During the first pregnancy which ended with a delivery of a healthy baby, the subglottic stenosis responded to laser radiation and glucocorticosteroid therapy. An uneventful therapeutic abortion was performed during the 2nd pregnancy. Our case brings to 15 the number of pregnancies which were recorded in 10 women with WG. Diagnosis of WG was done during pregnancy in 4 cases, and at the postpartum period in 3 cases. Eight pregnancies occurred in women with known WG, relapse occurred in 5 pregnancies. Two cases ended with maternal death. It seems therefore that pregnancy may have a significant contribution both to the relapse of WG and to its appearance.

Adult↗
PubMed1994

Cryptococcus neoformans vertebral osteomyelitis.

A 67-year-old previously healthy woman presented with low back pain of 2 months duration and daily fever of 39 degrees C for 3 weeks. CT scan showed a lytic lesion in the third lumbar vertebra and a small right lower lobe lung infiltrate with mediastinal lymphadenopathy. Culture of material obtained from open biopsy of the vertebra grew Cryptococcus neoformans var. neoformans, which was also demonstrated on histology. Cryptococcal antigen was detected in the patient's serum. Treatment with amphotericin B (1000 mg total dose) and oral 5-fluorocytosine, resulted in complete recovery and resolution of the chest X-ray findings with a follow-up of 2 years. Since this case, as well as most of the previously described cases of cryptococcal osteomyelitis, were in normal hosts, cryptococcal osteomyelitis should be considered in the differential diagnosis even in a normal host, and therefore, prior to possible invasive diagnostic procedures, cryptococcal antigen in the serum should be determined.

Aged↗
PubMed1994

Wernicke's encephalopathy in hyperemesis gravidarum: association with abnormal liver function.

A 27-year-old woman developed Wernicke's encephalopathy in the 18th week of her pregnancy after 11 weeks of vomiting accompanied by weight loss of 21 kg and moderately abnormal liver function tests. The patient recovered after thiamine therapy but the fetus was lost. Review of the literature published during the last 25 years revealed an additional 14 cases of Wernicke's encephalopathy complicating hyperemesis gravidarum. All patients vomited for at least 4 weeks. Six of the 15 patients (40%) had aspartate aminotransferase values > 100 U/l, much higher than the rate reported in previous series of patients with hyperemesis gravidarum (7%). This suggests the need for parenteral thiamine supplementation in patients with severe hyperemesis gravidarum lasting more than 3 weeks, especially those with abnormal liver function, and supports the hypothesis that the hepatic abnormality plays a pathogenetic role in the development of Wernicke's encephalopathy in hyperemesis gravidarum.

Adult↗
PubMed1993

Substitution of three amino acids switches receptor specificity of Gq alpha to that of Gi alpha.

Agonist-bound receptors activate heterotrimeric (alpha beta gamma) G proteins by catalysing replacement of GDP bound to the alpha-subunit by GTP. mutations in the C terminus of the alpha-subunit, its covalent modification by pertussis toxin-catalysed ribosylation of ADP, peptide-specific antibodies directed against it, and peptides mimicking C-terminal sequences, all inhibit receptor-mediated activation of G proteins. The logical prediction--that specific amino-acid residues at the C-termini of alpha-subunits can determine the abilities of individual G proteins to discriminate among specific subsets of receptors--has so far not been tested experimentally. Different hormone receptors specifically activate Gq or Gi, whose alpha-subunits (alpha q or alpha i) stimulate phosphatidylinositol-specific phospholipase C or inhibit adenylyl cyclase, respectively. Here we replace C-terminal amino acids of alpha q with the corresponding residues of alpha i2 to create alpha q/alpha i2 chimaeras that can mediate stimulation of phospholipase C by receptors otherwise coupled exclusively to Gi. A minimum of three alpha i2 amino acids, including a glycine three residues from the C terminus, suffices to switch the receptor specificity of the alpha q/alpha i2 chimaeras. We propose that a C-terminal turn, centered on this glycine, plays an important part in specifying receptor interactions of G proteins in the Gi/Go/Gz family.

Adenylate Cyclase Toxin↗
PubMed1993

[Actinobacillus actinomycetemcomitans endocarditis].

Bacterial endocarditis caused by Actinobacillus actinomycetemcomitans (AA) is an extremely rare disease. AA, a gram negative cocco-bacillus, normally resides in the oral cavity. It is involved mostly in local oral cavity infections, but severe systemic infections caused by it have been reported. We describe a 63-year-old man with endocarditis caused by AA which probably originated from a dental abscess. The course of the disease was complicated by acute myocardial ischemia, apparently caused by coronary artery embolism. To enable growth of this bacterium, blood cultures should be maintained for 2-3 weeks in a CO2-rich atmosphere.

Actinobacillus Infections↗
PubMed1992

Albright hereditary osteodystrophy with hypothyroidism, normocalcemia, and normal Gs protein activity: a family presenting with congenital osteoma cutis.

The syndrome of Albright hereditary osteodystrophy (AHO), pseudohypoparathyroidism (PHP) and pseudopseudohypoparathyroidism (PPHP) is clinically and genetically heterogeneous. Classically, patients with PHP have the skeletal features of AHO, resistance to multiple hormones that work via cAMP such as parathyroid hormone and thyroid stimulating hormone, and deficient activity of Gs protein, the guanine nucleotide-binding protein that stimulates adenylate cyclase. However, patients without hormone resistance but with AHO and Gs deficiency were described (PPHP), as well as patients with multiple hormone resistance but without AHO or Gs deficiency. In a few patients with deficient Gs activity, hypothyroidism rather than hypocalcemia was the initial presentation of the disorder. We describe here a new variant of the syndrome, affecting 5 individuals in a 3 generation family with AHO, normal Gs activity and hypothyroidism. In the first 2 generations, mild features of AHO were present. The 2 sibs in the third generation had severe manifestations of AHO, including mild mental retardation as well as hypothyroidism. Diagnosis of congenital osteoma cutis at birth of the proband led to the diagnosis of the family. Elucidation of the molecular defect will shed light on the relationship between hormone resistance and AHO, as well as on the physiological mechanism of hormonal signal transduction.

Adolescent↗
PubMed1992

EDTA-induced pseudothrombocytopenia.

A 45-year-old man was admitted to the hospital because of unstable angina pectoris. During the wait for coronary artery bypass surgery, the platelet count gradually decreased from 154,000 to 20,000/mm3, without hemorrhagic manifestations. Medications were discontinued and surgery was postponed. Direct blood smear revealed a normal number of platelets, establishing the diagnosis of pseudothrombocytopenia. The mechanism of the development of pseudothrombocytopenia is not clear.

Angina, Unstable↗
PubMed1992

Acute onset of celiac disease in the puerperium.

An acute, severe onset of celiac disease is very unusual in adults. We describe its occurrence postpartum in two patients. These, together with two similar cases previously reported, suggest a causal association with the puerperium. Possible causes include potentiation of a pathogenetic immune reaction and hormonal mechanisms.

Adult↗
PubMed1991

Cardiac conduction defects associated with hyponatremia.

Cardiac conduction defects have not been previously described in association with hyponatremia, although in patients with congestive heart failure the frequency of ventricular premature beats was found to correlate to the severity of hyponatremia. We describe three patients with second-degree or complete atrioventricular (AV) block which occurred during or shortly after an episode of severe hyponatremia. The first had thiazide-induced hyponatremia while on amiodarone. In the second, definite etiology for hyponatremia which was associated with longstanding polydipsia could not be established. The third had ischemic heart disease and intermittent conversion of his first-degree to second-degree AV block while hyponatremic after diuretics use. Although it is usually difficult to single out hyponatremia as the cause of conduction defects which usually occur in the presence of cardiac disease, potent medications or other electrolyte abnormalities, we suggest that hyponatremia may play a role in the pathogenesis of conduction defects in the diseased heart.

Adult↗
PubMed1991

Mitochondrial abnormalities in oculopharyngeal muscular dystrophy.

A family with oculopharyngeal muscular dystrophy (OPMD) is described. Histological and histochemical studies of muscle biopsy showed nonspecific myopathic changes; no "ragged-red" fibers were seen. Electron microscopy demonstrated bizarre large mitochondria with abnormal cristae, but no intranuclear inclusion bodies. Our findings are compatible with the possibility that OPMD is a heterogeneous syndrome, and may be a manifestation of mitochondrial myopathy.

Aged↗
PubMed1990

[Vitamin B12 deficiency: neurological disease with minimal or no anemia].

3 patients with vitamin B12 deficiency are described (men aged 64 and 67 and a women of 75). All presented with neurological disease; 2 had had severe weight loss. In 1 neither anemia nor macrocytosis was found, while in 2 there was only mild macrocytic anemia, so that the diagnosis was established quite late during the disease. These patients demonstrate that a high index of suspicion is required to diagnose vitamin B12 deficiency when there are only mild or no hematological abnormalities. This is especially important because of the direct relation between time elapsed before therapy and the irreversibility of neurological damage. Of note is the severe weight loss simulating malignant disease that can be the presenting symptom or appear during the course of the disease.

Aged↗
PubMed1990

Antibodies to Bordetella pertussis adenylate cyclase are produced in man during pertussis infection and after vaccination.

Bordetella pertussis produces several potential virulence factors. One of these is an adenylate cyclase which penetrates eukaryotic cells, is activated by calmodulin and generates high levels of intracellular cAMP. We have found that pertussis infection in man leads to production of high titres (2000-8000) of anti-B. pertussis adenylate cyclase antibodies. Such antibodies also are produced after pertussis vaccination. They persist into adulthood, cross the placenta and disappear a few months after birth. The anti-adenylate cyclase antibodies found in human serum during pertussis infection do not neutralise the catalytic and penetrative activities of the enzyme.

Adenylyl Cyclases↗