Elevated serum 1,25-dihydroxyvitamin D concentrations in siblings with primary Fanconi's syndrome.
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Adolescent↗
Biomedical subjects
D Modai
Publications and source records attributed to D Modai.
[Renal phosphate leak, rickets and hypercalciuria--a new hereditary syndrome].
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Calcitriol↗
The renotrophic factor, a persistent stimulus that crosses the placenta in mice.
1. Twenty 7-week female mice underwent right nephrectomy and twenty others were sham operated. A week later all animals were made pregnant. Pregnancy was repeated five more times consecutively and various renal parameters were assessed in the pups. 2. Fractional fresh kidney weight (relative to body weight) was significantly increased in the pups of nephrectomized mothers while percentage renal water and protein content expressed as mg/g kidney weight were not statistically different in the two groups of pups. Thus dry kidney weight and amount of protein per kidney were increased in the experimental group. This was true for the newborns of all six pregnancies. 3. Renal morphometric studies performed in newborns of first pregnancies showed that the mean number of glomeruli per microscopic field, mean fractional cumulative glomerular area (relative to microscopic field area) and the mean number of cells per glomerulus were significantly greater in the experimental group. Mean glomerular radius was not statistically different in the two groups. 4. The results indicate that: (1) the renotrophic factor(s) crosses the placenta in mice; (2) its activity in maternal circulation following uninephrectomy is sustained for a relatively long period; and (3) fetal response to enhanced maternal renotrophin stimulation consists of increased renal dry weight and renal protein, formation of super-physiological numbers of glomeruli and cellular hyperplasia of the glomeruli.
Alanine Transaminase↗
Effect of levamisole on chemotaxis of granulocytes from uremic patients.
Uremic granulocyte chemotaxis was assessed in the presence of four different concentrations of levamisole. Chemotactic responsiveness of uremic granulocytes was significantly decreased compared to normal, both in the absence of levamisole and with all levamisole concentrations tested. However, with 10(-3) and 10(-4) M levamisole concentrations, uremic granulocyte chemotaxis was similar to that of normal granulocytes without levamisole. Defective chemotactic activity of granulocytes may play a role in the increased susceptibility of uremic patients to infections. Pharmacological correction of this defect may improve the patients' ability to cope with infections.
Adult↗
1,25-Dihydroxyvitamin D-induced suppressor cells in uremic versus normal lymphocytes.
1,25-Dihydroxyvitamin D3 (DHD) has been shown to suppress mitogen-induced blast transformation. This inhibition is abolished by prior elimination of adherent cells. Chronic renal failure is an immunodeficiency state on the one hand and is associated with abnormalities in vitamin D metabolism on the other. The effect of DHD on the induction of suppressor cells in uremic vs. normal peripheral blood mononuclear cells was investigated. Study groups included 16 chronically uremic patients and 16 age- and sex-matched controls. DHD induced suppressor cell activity in normal lymphocytes. However, no suppressor cell activity was observed in lymphocytes from the uremic patients preincubated with DHD. The origin of the responder cells (normal or uremic) did not affect the outcome. The results would suggest that monocyte-adherent suppressor cells from uremic subjects are either incapable of binding DHD or fail to mount a normal post-receptor intracellular chain of events culminating in suppressor activity.
Adult↗
Autosomal dominant carpal tunnel syndrome in a Karaite family.
A clinical examination and a history of 64 members belonging to four generations of a Karaite kindred revealed 11 patients with carpal tunnel syndrome (CTS). Bilateral involvement and early age of onset are salient features. The mutation may be traced in four generations. Hereditary primary CTS may be more prevalent than hitherto suspected.
Adult↗
Frozen saphenous vein allografts for constructing vascular access for hemodialysis.
Frozen saphenous vein allografts were used in seven patients to construct vascular access for hemodialysis. In five patients the allografts connected the brachial artery to the axillary vein, and in two, the radial artery to the basilic vein. Five allografts functioned for periods varying from 6 to 20 months, one is still functioning after 3 years, and one graft occluded immediately following surgery. The availability of this biological material, the ease of its preparation, the very low cost, and the satisfactory graft survival, call for further evaluation of this method for complex vascular access problems in patients on chronic hemodialysis.
Adult↗
"Idiopathic" hypercalciuria and hereditary hypophosphatemic rickets. Two phenotypical expressions of a common genetic defect.
Among 59 closely related members of one Bedouin tribe, we identified 9 who had the characteristic features of hereditary hypophosphatemic rickets with hypercalciuria (HHRH). We found "idiopathic" hypercalciuria in 21 of the 50 asymptomatic members. The biochemical abnormalities observed in these 21 subjects were qualitatively similar to those in the 9 with HHRH, but were quantitatively milder. The urinary calcium concentration was 0.43 +/- 0.14 mg per milligram of creatinine (mean +/- SD) in the patients with HHRH, 0.34 +/- 0.07 in the subjects with idiopathic hypercalciuria, and 0.14 +/- 0.05 in normal subjects from the same tribe. Tubular reabsorption of phosphorus and serum phosphorus concentrations were 3.0 and 4.3 SD units below the age-related mean, respectively, in HHRH, and 1.1 SD units below the normal mean for both variables in idiopathic hypercalciuria. Mean serum levels of 1,25-dihydroxyvitamin D (1,25-(OH)2D) were 303 pg per milliliter in HHRH and 145 pg per milliliter in idiopathic hypercalciuria (upper normal limit, 110). We conclude that the subjects with hypercalciuria and the patients with HHRH shared a hereditary renal phosphate leak that led to hypophosphatemia, elevated serum concentrations of 1,25-(OH)2D, increased intestinal calcium absorption, and hypercalciuria. The magnitude of the hypophosphatemia, which regulates 1,25-(OH)2D levels, appears to determine which subjects will have hypercalciuria alone and which will also have bone disease.
Adolescent↗
Hydrochlorothiazide-amiloride causes excessive urinary zinc excretion.
Serum zinc levels and urinary zinc excretion were compared in 15 patients with essential hypertension taking chronically a combination of hydrochlorothiazide and amiloride as monotherapy, eight patients maintained with hydrochlorothiazide alone, and eight control subjects. Serum zinc values were statistically comparable in all three groups. However, urinary zinc excretion was abnormally elevated in the two patient groups. In the dosage used, amiloride did not have a zinc-sparing effect.
Adult↗
Adult Still's disease presenting as PUO; a not uncommon disorder.
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Adolescent↗
Barium appendicitis: fact or fancy? Report of a case and review of the literature.
Whether barium retained in the appendix can be a cause of acute appendicitis is debatable. We describe a 40-year-old man who developed nonspecific right abdominal pain 7 weeks after a barium enema, which proved to be normal. On abdominal film a distended appendix containing barium was seen, and at laparotomy acute appendicitis was present. Thus far, 26 cases of "barium appendicitis" have been reported. On the basis of the relevant literature and the cases collected, it is appropriate to draw the following conclusions: 1) With present knowledge it is not possible to state whether retained barium plays any etiologic role in the development of subsequent uncomplicated acute appendicitis. 2) If a later appendicitis does supervene, it carries a high risk of being complicated; barium seems to be responsible for the complication. 3) The longer the interval between the barium study and the subsequent appearance of acute appendicitis, the higher will be the risk of complications.
Acute Disease↗
Liver dysfunction in measles, liver biopsy findings.
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Adult↗
Sarcoid nephrocalcinotic renal failure reversed by sodium cellulose phosphate.
A case of renal failure due to sarcoidosis with hypercalciuria and nephrocalcinosis is described. Prolonged treatment with inorganic absorbable phosphate significantly deteriorated the patient's renal function. After a sodium cellulose phosphate treatment, renal failure was completely reversed. We suggest that sodium cellulose phosphate is the treatment of choice in sarcoidotic renal failure induced by nephrocalcinosis.
Calcium↗
Primary pulmonary Hodgkin's disease with air bronchogram.
A 30-year-old woman presented with a large pulmonary mass and air bronchogram. Hodgkin's disease of the nodular sclerosing type was diagnosed. Survival was short despite aggressive treatment. Primary pulmonary Hodgkin's disease is extremely rare. This is the first reported case of its presentation with an air bronchogram.
Adult↗
Induction of suppressor cells in normal lymphocytes by uremic serum.
Sera of patients on chronic hemodialysis induced suppressor cell activity (SCA) in normal peripheral blood mononuclear cells, which significantly impaired blastogenic response to PHA. This SCA is statistically not different from Con A induced SCA. Both SCAs are however additive. Speculations concerning the modes of action of this induced SCA are discussed.
Adult↗
Serum beta-N-acetyl hexosaminidase levels in chronic renal failure.
Serum beta-N-acetyl hexosaminidase (beta-NAH) levels, the indirect indicators of hepatic endothelial and Kupffer cell function, were examined in 16 anuric chronic hemodialysis patients, and in 11 patients in different stages of chronic renal failure (serum creatinine 2-8.8 mg/dl). They were found to be lower than those of the healthy controls, contrary to expectation. It might be concluded that nonparenchymal liver cells are functioning well in chronic renal failure. However, the possibility that production of beta-NAH in these patients is abnormally reduced cannot be excluded.
Adult↗
Replacement of ruptured aneurysm of arteriovenous fistula by segmental frozen venous allograft: a case report.
A ruptured aneurysm of an arteriovenous fistula created for chronic hemodialysis was replaced by a segment of frozen saphenous vein allograft. Fifteen months following the procedure the graft is patent. Frozen vein allografts should be considered as an appropriate option for the reconstruction of disrupted A-V fistulae.
Adult↗
An esoteric occupational hazard for lead poisoning.
A case of life threatening lead poisoning was diagnosed clinically in a Jewish scribe and verified by appropriate laboratory studies. The special ink used by the scribe was found to contain lead in appreciable amounts. Eleven more asymptomatic subjects, both scribes and manufacturers of the ink, were studied and five were found to have subclinical lead overload. Handling or production of this ink is a potential hazard for lead intoxication.
Adult↗