PubMed Health⌕ Search

Biomedical subjects

B Knobel

Publications and source records attributed to B Knobel.

At least 19 recordsLinked to original sources

[Cholesterol pericarditis associated with rheumatoid arthritis].

Cholesterol pericarditis (CP) is a rare and unusual disease characterized by chronic pericardial effusion with high cholesterol concentration. Precipitation of cholesterol crystals may occur and induce inflammation and constrictive pericarditis. CP may be idiopathic, but is usually associated with a systemic disease, such as tuberculosis, myxedema, or as in our case, rheumatoid arthritis (RA). We present a 78-year-old woman with RA, typical deformities of the metacarpo- and metatarso-phalangeal joints and subcutaneous rheumatoid nodules. She was hospitalized with increasing dyspnea and weakness and a 2-dimensional transthoracic echocardiogram showed a large pericardial effusion, without tamponade. Blood cholesterol was 208 mg/dl, triglycerides 169 mg/dl, LDH 37 u/L and rheumatoid factor 2560 u; glucose, kidney, and thyroid function tests were normal and PPD test negative. Pericardiocentesis yielded 800 ml of opaque, cloudy fluid, with glucose 19 mg/dl, cholesterol 264 mg/dl (normal 20-40 mg/dl), triglycerides 169 mg/dl, LDH 5820 u/L and rheumatoid factor 40 u; viral titers and cultures for bacterial, mycobacterial and fungal infections were negative. The pericardial fluid had a distinctive scintillating, gold-paint appearance and many cholesterol crystals were evident microscopically. The patient responded to treatment with methotrexate and steroids. Factors responsible for increase in pericardial fluid cholesterol may be its liberation from injured pericardial cells and rheumatoid nodules, lysis of red cells, or lymphatic obstruction and impairment of the absorptive capacity of the pericardium.

Aged↗

[Malignant humoral hypercalcemia associated with angiotropic large B cell lymphoma].

Angiotropic large B cell lymphoma (angiotropic LCL) or intravascular large cell lymphoma (IVLCL) was diagnosed by liver and bone marrow biopsies and immunohistochemical studies in a 52 year old Caucasian male. IVLCL is a very rare disease characterized by widespread intravascular proliferation of lymphoma cells. Although it most commonly affects the central nervous system or skin and occasionally bone marrow, angiotropic LCL may be present without evidence of localized disease, as seen initially in our patient. To date, only a few cases of intravascular malignant lymphomatosis associated with parathyroid hormone related protein (PTH-rP) induced humoral hypercalcemia have been published. Our extraordinary case was diagnosed mainly by liver biopsy. The neoplastic lymphoid cells stained diffusely and strongly positive with CD-20 (Pan B) and were negative for CD-3 (Pan T) immunostain. The most significant, initial clinical finding was severe, unexplained hypercalcemia (until 18.6 mg/dl). Plasma PTH-rP showed a ten-fold increase at 8 pmol/L (normal value less than 0.8 pmol/L). Very unusual cytogenic abnormalities were found. The patient received the massive third generation combination chemotherapy comprising of Methotrexate, Doxorubicine, Cyclophosphamide, Vincristine, Prednisone and Bleomycin and developed, complete although temporary, clinical, humoral and cytogenetic remission.

Antineoplastic Combined Chemotherapy Protocols↗

[Severe accidental hypothermia in an elderly woman].

Profound hypothermia (core temperature of less than 28 degrees C) is a life threatening state and a medical emergency associated with a high mortality rate. The prognosis depends on underlying diseases, advanced or very early age, the duration prior to treatment, the degree of hemodynamic deterioration, and especially, the methods of treatment, including active external or internal rewarming. This is a case study of an 80-year-old female patient with severe accidental hypothermia (core temperature 27 degrees C). She was found in her home lying immobile on the cold floor after a fall. The patient was in a profound coma with cardiocirculatory collapse, and the medical staff treating her was inclined to pronounce her deceased. On her arrival at the hospital, she was resuscitated, put on a respirator and actively warmed. Very severe metabolic disorders were found, including a marked metabolic acidosis composed of diabetic ketoacidosis (she had suffered from insulin treated type 2 diabetes mellitus) and lactic acidosis with a very high anion gap (42) and a hyperosmotic state (blood glucose 1202 mg/dl). There were pathognomonic electrocardiographic abnormalities, J-wave of Osborn and prolonged repolarization. Slow atrial fibrillation with a ventricular response of 30 bpm followed by a nodal rhythm of 12 bpm and reversible cardiac arrest were recorded. The pulse and blood pressure were unobtainable. Despite the successful resuscitation and hemodynamic and cognitive improvement, rhabdomyolysis (CKP 6580 u/L), renal failure and hepatic damage developed. She was extubated and treated with intravenous fluids containing dopamine, bicarbonate, insulin and antibiotics. Her medical condition gradually improved, and she was discharged clear minded, functioning very well and independent. Renal and liver tests returned eventually to normal limits. Progressive bradycardia, hypotension and death due to ventricular fibrillation or asystole commonly occur during severe hypothermia. Respiratory and metabolic, sometimes lactic, acidosis, lethargy and coma, hypercoagulopathy, hyperosmolar state, acute pancreatitis and renal and hepatic failure are frequent complications of hypothermia. Underlying predisposing causes of hypothermia are diabetic ketoacidosis, cerebrovascular disease, mental retardation, hypothyroidism, pituitary and adrenal insufficiency, malnutrition, acute alcoholism, liver damage, hypoglycemia, sepsis, hypothalamic dysfunction, sepsis and polypharmacy, and especially, the use of sedative and narcotic drugs. Our case demonstrates once again that CPR once begun should continue until the successful rewarming because "no one is dead until warm and dead".

Aged↗

Bilateral hydronephrosis due to fecaloma in an elderly woman.

By computer search of the literature, we found few cases of hydronephrosis due to fecal impaction. Because such a complication is extremely rare, we describe an 81-year-old woman with dementia, gallstones, arterial hypertension, and diverticulosis of the sigma who was hospitalized for severe constipation, fecaloma, and bilateral hydronephrosis. Through simultaneous lavage by two rectal tubes and manual disimpaction of fecaloma, bilateral hydronephrosis was resolved. We also briefly review the appropriate literature.

Aged↗

[Follicular splenic lymphoid hyperplasia associated with EBV infection].

Massive splenomegaly is defined as a spleen weighing about 10 times normal weight. We describe a 36-year-old man who had huge splenomegaly and secondary pancytopenia simulating malignant lymphoma for about 3 months. Splenectomy was necessary because of the suspicion of hematologic malignancy, especially isolated lymphoma of the spleen, and pain and mechanical abdominal disturbances. On operation, the spleen was 25 cm long and weighted 250 g. There was florid, reactive follicular lymphoid hyperplasia. Immunohistochemical staining with CD-20(L26), CD-45Ro(UCHL), bcl-2 oncoprotein (Dakopatts), EBV (anti-EBV mol weight 60 KD, Dakopatts) was consistent with reaction to EBV infection and not with follicular lymphoma. Lack of PCR amplification using DNA extracted from paraffin-embedded splenic tissue indicated absence of a monoclonal B cell population carrying rearranged immunoglobulin genes. The lymphocytic population was proven polyclonal by the negative results of PCR for the bcl-2 gene rearrangement. EBV seroconversion from high titer antibodies of anti-EBV-VCA-IgM to negative, and from negative EBNA to positive was consistent with an apparent primary EBV infection. We have not found on computerized search a previous report of reactive follicular splenic hyperplasia to EBV infection causing huge splenomegaly, with or without EBV-induced infectious mononucleosis.

Adult↗

Sudden death and cerebral anoxia in a young woman with congenital ostial stenosis of the left main coronary artery.

We report a 36-year-old woman with ventricular fibrillation, subsequent sudden clinical cardiac death, and a prolonged brain anoxia. After a successful resuscitation coronary angiography revealed congenital ostial left main coronary artery (LMCA) stenosis. Surgical anastomosis of the left internal mammary artery (LIMA) to LAD led to a complete recovery. Postoperative electrophysiological examination, mainly programmed ventricular stimulation, failed to excite any rhythm disturbances. Cathet. Cardiovasc. Intervent. 48:67-70, 1999.

Adult↗

Pyrazinamide-induced granulomatous hepatitis.

Noncaseating granulomatous hepatitis may be caused by a variety of drugs, but we have not found, by computer search of the literature, a previous describe of granulomatous hepatitis associated with pyrazinamide. We describe a 52-year-old man with hectic fever, chills, extreme fatigue, liver damage, and hyperuricemia about 4 weeks after commencing pyrazinamide therapy. A liver biopsy specimen showed noncaseating epithelioid granulomas. The patient recovered soon after the interruption of tuberculostatic treatment.

Antitubercular Agents↗

Isolated splenic infarction following left cardiac catheterization: case report and a review of the literature.

A cardiac catheterization was performed in a 57-year-old man for post-infarction angina. A severe left flank pain developed following the angiography. Ultrasonography, computed tomography, and radionuclear scanning of the abdomen showed splenic infarction. An isolated cholesterol atheroembolism of spleen from disrupted atheromatous plaques so far has not been reported.

Cardiac Catheterization↗

Hyperphosphatemic hypocalcemic coma caused by hypertonic sodium phosphate (fleet) enema intoxication.

We describe an elderly woman with a deep hyperphosphatemic hypocalcemic coma, hypernatremia, hypokalemia, metabolic acidosis, pancytopenia and respiratory and circulatory failure secondary to phosphate intoxication following the overdose administration of hypertonic sodium phosphate enema. The causes of increased colonic retention and absorption and decreased renal excretion are discussed. We recommend the use of the safer and less toxic cathartic medications or at least a very cautions use of such enemas in anyone with renal failure.

Aged↗

Myocardial bridging: surgical technique and operative results.

We report a case of a 37-year-old symptomatic male with anterior myocardial ischemia. Coronary angiography demonstrated systolic obstruction of the midportion of the left anterior descending coronary artery due to myocardial bridging. The patient was treated with a supra-arterial myotomy. Two years postoperatively, the patient is without evidence of myocardial ischemia. Angiography, 2 years postoperatively, shows no evidence of systolic narrowing of the left anterior descending coronary artery.

Adult↗

[Acute renal failure as a complication of cholera].

We present a 72-year-old man who had episodes of severe, acute renal failure during severe attacks of diarrhea caused by Vibrio cholerae. Patterns of acute tubular necrosis and tubulointerstitial nephritis developed following hypotension and decrease in renal blood flow, causing secondary renal ischemia. There was severe dehydration with profound hypovolemia and infection. The clinical picture included fever, weakness, arthralgia, pedal edema, mild bilateral pleural effusions, anemia, leukocytosis, azotemia with a maximum of 330 mg/dl of urea, creatine to a maximum of 9.8 mg/dl, hypoproteinemia, severe metabolic acidosis, marked increase in lactate dehydrogenase (LDH) and creatine phosphokinase (CPK), microscopic hematuria, sterile leukocyturia, normoglycemic glucosuria and phosphaturia with diminished tubular reabsorption of phosphorus. A short oliguric phase was followed by a polyuric phase lasting about 10 days, and glomerular and tubular function became normal after about 3 weeks. Treatment was by intensive infusions of fluids, electrolytes, sodium bicarbonate, salt-free albumin and antibiotics. To the best of our knowledge, this renal complication of cholera has not yet been described in Israel.

Acute Kidney Injury↗

Ectopic medullary hematopoiesis as a cause of ascites in agnogenic myeloid metaplasia. Case report and review of the literature.

We report an unusual case of a 44-year-old female patient with 'malignant' ascites caused by ectopic foci of extramedullary hematopoiesis in the course of agnogenic myeloid metaplasia. The patient had suffered also from severe Coombs-positive acquired hemolytic anemia and had been splenectomized. Two years after splenectomy, ascites caused by peritoneal implants of hematopoietic tissue appeared. The ascites responded promptly to treatment with busulfan and hydroxyurea. The clinical picture, treatment and a review of the literature concerning the mechanisms of this uncommon evolution are discussed.

Anemia, Hemolytic↗

[Remission of acute promyelocytic leukemia after all-trans-retinoic acid].

This is the first report in Israel of the successful treatment of acute promyelocytic leukemia (APL; M3) with an active metabolite of vitamin A. In a 42-year-old woman with APL all-trans-retinoic acid (ATRA; tretinoin), 45 mg/m2/day was given per os for 90 days. APL is associated with a distinct cytogenetic abnormality: translocation of a portion of the long arm chromosome 17 onto the long arm chromosome 15t (15; 17) with a breakpoint on chromosome 17 in the region of the retinoic acid receptor-alpha (RAR-alpha), playing a crucial role in the leukemogenesis of APL. In man, the drug induces myeloid and mainly promyelocytic leukemic cells to differentiate, without the development of bone marrow hypoplasia. In our patient it caused complete remission and the disappearance of intravascular disseminated coagulation. The only side-effects were a transient macular rash, gastrointestinal symptoms and mild hypertriglyceridemia. Other principal adverse effects reported in the literature are relatively not very serious and consist of dryness of the skin, occasional headaches and intracranial hypertension, nasal congestion, lymphadenopathy, respiratory distress with infiltrates in the lung, bone pain and increased hepatic aminotransferase. A hyperleukocytosis syndrome seems to be more problematic. ATRA appears to be superior to conventional chemotherapeutic regimens. It is safe and highly effective in inducing clinical, morphologic and karyotypic remission with a marked decrease in the expression of the abnormal RAR-message in APL. There is a possible molecular link between the pathogenesis and treatment of this severe and often fatal coagulopathic disease. This therapy of course does not eradicate the leukemic clone, and consolidation chemotherapy or bone marrow transplantation is necessary.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

Intracardiac primary fibrosarcoma. Case report and literature review.

A 58-year-old woman was hospitalized because of shortness of breath, cough, weakness, and physical signs suggestive of mitral stenosis. Echo-Doppler examination revealed a left atrial mass. This was removed and turned out to be a fibrosarcoma. Recurrence of the tumor with metastases into the pericardium, thyroid goiter, and left kidney led to the patient's death 6 months later. The clinical and pathological features of our rare case are compared with those in the literature.

Female↗

Danazol in non-splenectomized patients with refractory idiopathic thrombocytopenic purpura.

Seven non-splenectomized patients with chronic refractory idiopathic thrombocytopenic purpura were treated with danazol 800 mg daily. All were glucocorticoid failures and four were refractory to all additional previous therapy. Five patients benefited from danazol and in two sustained normal platelet counts, for over 44 and 51 months, were observed. We conclude that danazol is useful for long term management of otherwise refractory idiopathic thrombocytopenic purpura. The advantage of danazol over splenectomy as a first line treatment in steroid failure is suggested.

Aged↗