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L Portmann

Publications and source records attributed to L Portmann.

44 records · Page 3Linked to original sources

Distal renal tubular acidosis and lymphocytic thyroiditis with spontaneously resolving hyperthyroidism. Report of 1 case without nephrocalcinosis.

In a 33-year-old woman concurrence of a complete distal renal tubular acidosis (RTA) and lymphocytic thyroiditis with spontaneously resolving hyperthyroidism was observed. Until recently, the rare association of RTA and hyperthyroidism had been thought to be governed by nephrocalcinosis, via hypercalcemia and hypercalciuria. However, in this case, nephrocalcinosis was not present, but there were histological signs of renal interstitial mononuclear cell infiltration, and the RTA persisted despite the resolution of the hyperthyroidism. This observation supports the idea that immunological mechanisms may relate RTA and hyperthyroidism when the latter has an autoimmune origin.

Acidosis, Renal Tubular↗

Anti-thyroid peroxidase antibody in patients with autoimmune thyroid disease: possible identity with anti-microsomal antibody.

Thyroid microsomal antigen and peroxidase (TPO) have a close intracellular anatomical relationship, especially in exocytotic vesicles. We considered that antibodies to microsomal antigen might react with TPO and therefore looked for the presence of antibodies against TPO in the serum of patients with autoimmune thyroid disease (AITD). TPO was prepared from Graves' thyroid glands, solubilized by n-octyl glucoside, and its activity was assayed by the guaiacol method. Control sera and sera with a positive microsomal hemagglutination test (MCHA(+) ) were assayed for their ability to precipitate TPO activity by incubation of sera with TPO and protein A. We identified MCHA(+) sera which caused precipitation of TPO activity, and the extent of precipitation was related to the amount of serum added. A significant correlation was present between this anti-peroxidase activity and microsomal antibodies titers, measured by a micro-ELISA method. Affinity columns prepared from immunoglobulins of MCHA(+) sera, coupled to Reacti-Gel (6X), bound TPO activity, whereas using control IgG the recovery in the unbound fraction was high. These data provide evidence of antibodies against thyroid peroxidase in the serum of patients with AITD and suggest a close link between microsomal antigen and thyroid peroxidase.

Antibodies↗

Drinking water for stone formers: is the calcium content relevant?

Stone formers are often told to select a drinking water with low Ca content. To see whether this measure has a rational biochemical background, 4 Ca hyperabsorbers were asked to drink, first tap water ad libitum, then 2 liters/day of tap water, then 2 liters/day of a low Ca water (A) and finally 2 liters/day of a high Ca water (B). On A, subjects were normocalciuric but hyperoxaluric; whereas on B, they were markedly hypercalciuric but normooxaluric. Therefore, Ca . Ox concentration products were similar on B and A. However, on A as well as on B, Ca . Ox products were much lower than on tap water ad libitum due to the high fluid intake which had thus been imposed. It is concluded that, in the prevention of the recurrence of nephrolithiasis, 'have a high fluid intake' is probably a more relevant advice than 'select a water with a particularly low Ca content'.

Calcium↗

[Simple treatment of tumor-induced hypercalcemia: i.v. aminohydroxypropylidene biphosphonate].

The treatment of tumour-induced hypercalcemia (HCa) by diphosphonates appears to be safe and reliable with very mild side effects. We have tested APD as sole treatment in 14 patients with cancer and symptomatic HCa. APD was given i.v. in a dose of 0.38 +/- 0.03 mg/kg BW/day until two subsequent normal plasma calcium (Ca) levels were obtained (Ca determined daily). The mean duration of treatment was 5.56 +/- 1.9 (4 to 11 days). As expected, there was a significant correlation between initial Ca and duration of therapy (p less than 0.001). Mean Ca fell from 3.38 +/- 0.19 mmol/l before APD to 2.43 +/- 0.09 after 4 days of treatment and remained normal four weeks after the start of treatment. Four patients relapsed 11 to 31 days after the end of treatment during a one-month follow-up. This recurrence did not depend on initial Ca, length of treatment and duration of remission. Two of these patients were treated successfully with a second course of APD, while in the other two no further treatment was undertaken. Both mean UCa and UHydroxyproline decreased significantly when measured seven days after the beginning of the treatment. Plasma PTH values remained in the normal range, while NcAMP rose significantly after a week of treatment from low to normal values. Plasma phosphorus decreased significantly (p less than 0.005) during the treatment while TRP % increased, suggesting a positive phosphorus balance. We conclude that short treatments with APD of tumour-induced HCa are effective and sufficiently sustained, even if they are interrupted as soon as normal Ca is obtained.

Adenosine Diphosphate↗

[Idiopathic calcium nephrolithiasis: therapeutic aspects].

The 75% of the renal stone formers have a so-called idiopathic calcium urolithiasis. The majority of these patients, however, do have a detectable biochemical disorder such as hypercalciuria, hyperuricosuria or hyperoxaluria. A high fluid intake unequivocally represents the first step in the therapeutic approach to these patients. Nevertheless, the detection of any type of biochemical disturbance is of great importance since the addition of a specific therapy will then become possible. Patients with absorptive idiopathic hypercalciuria will be advised to decrease their intake of dairy products as a function of the degree of calcium hyperabsorption, and simultaneously the major dietary sources of oxalate such as chocolate, spinach, rhubarb and asparagus will be eliminated; neutral orthophosphates (3-4 times 500 mg/d) or a thiazide, resp. an analogue as chlorthalidone (50 mg/d) are reasonable alternatives. Renal idiopathic hypercalciuria should be treated, according to the authors, with chlorthalidone (50 mg/d), with or without allopurinol (300 mg/d) depending on the presence of concomitant hyperuricosuria. Patients with dietary idiopathic hypercalciuria should be advised to better equilibrate the various components of their dietary intake. Finally, patients with isolated idiopathic hyperuricosuria whose disease would remain active despite a high fluid intake should receive allopurinol (300 mg/d). The treatment of isolated idiopathic hyperoxaluria is not yet well established. Two main arguments favor this so to say "tailored" approach to the idiopathic stone former: first, some metabolic disturbances are causally related to a particularly active and severe urolithiasis, whereas others are less so; second, the lack of efficacy of some types of treatment appears more and more to be due to insufficient screening of the patients before starting a given treatment.

Calcium↗

[Incidence of hyperoxaluria in idiopathic calcium nephrolithiasis].

Urinary excretion rate of oxalate was measured in 79 patients with idiopathic calcium (Ca) nephrolithiasis and the results were compared with those obtained in 28 healthy volunteers. The group of stone formers consisted of 20 patients with idiopathic hypercalciuria (IHC) of the absorptive type, 23 patients with IHC of the renal type, 11 patients with hypercalciuria secondary to dietary factors, 1 patient with hyperuricosuria (as an isolated finding) and 24 patients without hypercalciuria nor hyperuricosuria. Classification was based upon the urinary excretion rate of uric acid, as well as that of calcium measured under 3 different dietary conditions (i.e. free diet, free diet supplemented with 3 g Ca/day for 3 days, and diet free of dairy products for 5 days). On a free diet, normal values of oxaluria ranged from 125 to 435 mumol/24 h; an elevated value was observed in 11 (14%) patients, 5 of whom belonged to the subgroup without hypercalciuria nor hyperuricosuria. On a low Ca diet, mild hyperoxaluria occurred in 3 controls and in 19 patients, the tendency to develop hyperoxaluria being particularly marked in the subgroup with absorptive-IHC. Moreover, there was a positive correlation between oxaluria on a low Ca diet and the estimated degree of intestinal absorption of Ca. This study confirms the finding that on a free diet, the incidence of mild hyperoxaluria amongst idiopathic stone formers is rather low. It shows, however, that a significant percentage of patients classically referred to as "without metabolic disorder" have in fact slight hyperoxaluria, an observation with a potential therapeutic impact. Finally, it shows that on a low Ca diet, patients with absorptive-IHC are particularly prone to develop hyperoxaluria: the latter observation renders questionable the relevance of a low Ca diet for patients with absorptive IHC, unless their intake of oxalate is simultaneously reduced.

Adolescent↗

Medullary sponge kidney on axial computed tomography: comparison with excretory urography.

To evaluate features of medullary sponge kidney (MSK) on computed tomography (CT), 4-mm-thick axial slices without intravenous contrast material were first made in 13 patients through 24 kidneys which showed images of MSK on excretory urograms. On CT, papillary calcifications were found in eleven kidneys. In five of these kidneys, the calcifications were not detectable on plain films. Some hyperdense papillae (attenuation value 55-70 Hounsfield units) without calcification were found in four other kidneys. Nine kidneys appeared normal. Ten of these 24 kidneys were reexamined by a second series of 4-mm-thick axial slices, 5 min after intravenous injections of 50 ml of Urografin. Images suggesting possible ectasia of precaliceal tubules were found in only four kidneys. These images appear much less obvious and characteristic on CT than on excretory urogram and do nothing more than suggest the possibility of MSK. In conclusion, the sensitivity of CT in the detection of MSK is markedly lower than that of excretory urography. In the most florid cases of the disease, CT can only show images suggesting the possibility of MSK. On the other hand, CT appears much more sensitive than plain films and tomograms of excretory urography in the detection of papillary calcifications, the most frequent complication of MSK.

Female↗