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PubMed1975

Calculi complicating a renal transplant.

Four months after a cadaver kidney transplant, kidney stones were found in the renal allograft. Three major predisposing causes of nephrolithiasis were found in the patient, including hyperparathyroidism, renal tubular acidosis, and urinary tract infection. Hypercalcemia was corrected by parathyroidectomy. During the subsequent three years there was no enlargement of the renal stones and adequate kidney function was maintained. Renal tubular acidosis was not severe and seemed to be related to chronic rejection. Urinary tract infection was readily corrected with antibiotics and did not recur after the immediate post-transplant period. Surgical therapy for nephrolithiasis involving a kidney allograft was defferred since urinary flow was not obstructed. This course of management is recommended for use in patients with calculi complicating renal transplantation.

Acidosis, Renal Tubular↗
PubMed1979

Lithium and the kidney.

Three middle-aged women treated with lithium carbonate for a manic-depressive illness have had complicating nephrogenic diabetes insipidus, renal tubular acidosis, acute reversible renal functional impairment in association with hypercalcaemia, or irreversible chronic renal damage. Renal toxicity developed in the presence of normal levels of lithium in the serum. The possibility of permanent renal damage as a result of long-term lithium therapy is of major concern.

Acidosis, Renal Tubular↗
PubMed2005

Marble brain disease in two Saudi Arabian siblings.

Marble brain disease, also known as Guibaud-Vainsel syndrome, is a syndrome consisting primarily of renal tubular acidosis, cerebral calcification and osteopetrosis. The majority of reports originate from the Middle East. It is an autosomal recessive condition owing to carbonic anhydrase type II deficiency in renal and brain cells with a variant form of osteopetrosis. We report two siblings with this condition from Saudi Arabia. Both cases improved in both somatic growth and mental development after commencing treatment for renal tubular acidosis in the form of alkaline therapy and potassium supplementation.

Acidosis, Renal Tubular↗
PubMed1986

Renal involvement in Sjögren's syndrome.

Renal involvement is uncommon in Sjögren's syndrome. The case is reported of a woman with Sjögren's syndrome who developed renal failure, hypergammaglobulinaemic renal tubular acidosis and a renal tubular concentrating defect due to a severe tubulointerstitial nephritis. There was an impressive response to corticosteroid therapy.

Acidosis, Renal Tubular↗
PubMed1995

[Renal acidification mechanism disorders in patients with osteoporosis].

Eight patients (6 women and 2 men) with osteoporosis caused or aggravated by renal acidification defects are presented. Three of the female patients were premenopausal; the others were 9, 20 and 22 years postmenopausal, and two of them were on hormonal replacement therapy. Two patients had nephrolithiasis: one male with recurrent calcium phosphate stones and a left sided staghorn calculus, and one female with nephrocalcinosis due to medullary sponge kidney and hypercalciuria (patients No. 1 and 2, respectively, Table 1). In the remaining subjects, clinical suspicion was based on: a) Hip fracture in a 44-yr-old premenopausal female without any risk factor (No. 3, Table 2). b) Several vertebral compression fractures in a 45-yr-old male without hypogonadism or other predisposing factors (No. 7, Table 2). c) Lack of response to antiosteoporotic therapy in 3 women (patients No. 4, 6 and 8, Table 2). Serum bicarbonate levels and urine acidification capacity were studied in all patients. Three had low serum bicarbonate (two of whom showed high fractional excretion of bicarbonate), four had a distal defect, and one had a mixed form. Serum creatinine and potassium, and venous blood pH were normal in all cases, suggesting incomplete renal tubular acidosis. Bone mineral density in Z-score (means +/- s.e.m.) was - 1.75 +/- 0.08 in the lumbar spine (n = 8), and - 1.57 +/- 0.09 in the femoral neck (n = 4) [Tables 1 and 2; Figs 1 and 2]. Following one year treatment with oral sodium bicarbonate and potassium citrate, total skeletal calcium increased by 3-10% in five of the patients. Whereas the high prevalence of renal acidification defects among renal stone formers with or without hypercalciuria is well acknowledged, renal tubular acidosis is not included in the list of entities causing secondary osteoporosis. As shown in 6 patients of this series, incomplete RTA should be considered as another disease capable of causing osteoporosis or worsening involutional bone loss.

Acidosis, Renal Tubular↗
PubMedDate not supplied

Interstitial nephritis in patients with systemic lupus erythematosus: a manifestation of concomitant Sjögren's syndrome?

In order to estimate the frequency of functionally relevant tubular damage in SLE patients we used the presence of overt renal tubular acidosis as an indicative parameter of interstitio-tubular damage in a cohort of 109 SLE patients who were well characterized for potential Sjögren's syndrome. Only two patients had overt renal tubular acidosis and interstitial nephritis without major glomerular involvement. Both patients, aside from having systemic lupus erythematosus, had a number of clinical features of concomitant primary Sjögren's syndrome. Based on the results obtained and the analyses of previously published cases, we put forward the hypothesis of simultaneous primary Sjögren's syndrome as the prevailing associative factor for the rare occurrence of isolated interstitial nephritis in SLE.

Acidosis, Renal Tubular↗
PubMed1980

Complete proximal tubular acidosis (Type 2, RTA) in chronic active hepatitis.

Fifteen patients with chronic active hepatitis (CAH) were tested in order to ascertain the site of tubular dysfunction in renal tubular acidosis (RTA) associated with CAH. Renal plasma flow and GFR were reduced in the patients compared to controls (P < 0.005). Underbasal and acidification conditions, twelve patients showed normal acid-base balance and net acid excretion, while three patients had basal hyperchloremic metabolic acidosis while passing alkaline urine and showing bicarbonaturia. A sustained acidification test showed adequate urinary acidification in these three patients. Bicarbonate loading carried out in two of the three patients showed a proximal tubular acidifying defect (Type 2 RTA), Distal RTA (Type 1 RTA) complicating CAH is widely known, but these data suggest that CAH can also involve the proximal convoluted tubule in isolation.

Acid-Base Equilibrium↗
PubMed1995

Ambulatory evaluation of nephrolithiasis: an update of a 1980 protocol.

Evaluations of 1,270 patients with recurrent nephrolithiasis in an outpatient setting were analyzed for the purpose of updating the classification of nephrolithiasis. All but 4% had abnormal urinary biochemistry that placed them into one or more of 20 etiologic categories. A single diagnosis was documented in 41.3% of patients. The remaining 58.7% had more than one diagnosis. Hypercalciuric calcium (Ca) nephrolithiasis, encountered in 60.9% of patients, comprised six variants--absorptive hypercalciuria Type I and II, renal hypercalciuria, primary hyperparathyroidism, and unclassified hypercalciuria (renal phosphate leak and fasting hypercalciuria). Hyperuricosuria Ca nephrolithiasis (HUCN) and gouty diathesis (GD) accounted for 35.8% and 10.0% of patients, respectively. Distinguishing features were hyperuricosuria and normal urinary pH in HUCN, and normal urinary uric acid and low urinary pH (< 5.5) in GD. Hyperoxaluric Ca nephrolithiasis, occurring in 8.1% of patients, was subdivided into enteric, primary, and dietary variants. Hypocitraturic Ca nephrolithiasis affected 28% of patients in its idiopathic variant. Many of these patients' problems were probably dietary in origin, while some could have had incomplete renal tubular acidosis. Hypocitraturia due to renal tubular acidosis or chronic diarrheal syndrome affected only 3.3% of patients. Hypomagnesiuric Ca nephrolithiasis, infection stones, and cystinuria were uncommon, accounting for 6.8%, 5.9%, and 0.9% of patients, respectively. The acquired problem of low urine volume (< 1 L/d) was found in 15.3% of patients. The remaining 3.5% of patients were difficult to classify despite the presence of abnormal urinary biochemistry.

Adult↗
PubMed2004

Osteal complications as first manifestation in a patient with primary Sjögren's Syndrome and with associated distal tubular acidosis (type 1) and chronic renal insufficiency.

Renal affection is among the complications associated with the Sjögren's Syndrome. Tubulo-interstitial nephritis constitutes the most frequent renal lesion and distal tubular acidosis (Type 1) is the most important clinical manifestation of this tubular dysfunction, although the occurrence of chronic renal insufficiency is not an uncommon finding in the presence of distal renal tubular acidosis. Osteomalacia is a clinical consequence of tubular acidosis caused by buffering of H+ in the bone. We present the case of a woman with osteal complication a year before being diagnosed with primary Sjögren's Syndrome and with distal tubular acidosis and renal insufficiency associated at diagnosis.

Acidosis, Renal Tubular↗
PubMed1980

Nephrotic syndrome and multiple tubular defects in children: an early sign of focal segmental glomerulosclerosis.

The nephrotic syndrome is rarely associated with renal tubular defects, and the combination has been reported only in association with advanced renal insufficiency. We report here five children with nephrotic syndrome and multiple tubular defects which evolved when glomular filtration rate ranged between 56 and 90 ml/minute/1.73 m2. The tubular defects were first noted at 3, 4, 4, 7, and 22 months after the onset of the nephrotic syndrome, and renal glycosuria was the first sign in all five children. Glycosuria was intermittent in three patients, constant in two, and ceased with loss of kidney function. Four patients had hyperaminoaciduria and renal tubular acidosis (two of four tested had distal renal tubular acidosis). Three patients had decreased tubular reabsorption of phosphorus and defective maximum concentrating capacity. All five had focal segmental glomerulosclerosis proven by renal biopsy. Over a follow-up period of seven years, all of the children have developed advanced renal insufficiency, four of the five have required dialysis or transplantation within 21 to 72 months after onset, and one has stabilized renal function at 35 ml/minute/1.73 m2. The one patient receiving a kidney transplant has had recurrence of focal segmental glomerulosclerosis in the transplanted kidney and became nephrotic with three subsequent transplants. Our experience suggests that the nephrotic syndrome associated with tubular defects in children forms a subgroup of focal segmental glomerulosclerosis, with rapid progression to renal insufficiency and the potential for recurrence of the lesion in the transplanted kidney.

Acidosis, Renal Tubular↗
PubMed1982

Disorders of distal nephron function.

In this review, the distal nephron is considered to be that portion of the renal tubule commencing with the thick ascending limb of the loop of Henle and ending with the papillary collecting duct. The collecting duct, including its subdivisions in the cortex and medulla, originates from a different embryologic anlage than more proximal nephron segments, which may explain its morphologic and functional dissimilarities from the thick ascending limb and the distal convoluted tubule. This review summarizes selected aspects of the physiology of the distal nephron, with particular emphasis on the physiology of distal nephron transport of sodium, potassium, chloride and hydrogen ion. The pathophysiologic features of the following disorders of distal nephron function are reviewed: (1) pseudohypoaldosteronism, a heterogenous group of disorders in which the signs and symptoms are suggestive of aldosterone deficiency, but in which aldosterone levels are supernormal and administration of exogenous mineralocorticoid is not ameliorative; (2) pseudohyperaldosteronism (Liddle syndrome), a familial disorder in which the clinical manifestations closely resemble those resulting from an aldosterone-producing adenoma of the adrenal gland (primary aldosteronism), but in which the measured rate of aldosterone secretion and excretion is greatly subnormal; (3) Bartter syndrome and related syndromes of renal potassium wasting; (4) type 1 renal tubular acidosis (classic, distal); (5) type 4 renal tubular acidosis (hyperkalemic). Reference citations are generally to articles reporting recent advances in these areas and to review articles that contain comprehensive bibliographies.

Acidosis, Renal Tubular↗
PubMedDate not supplied

Aetiological, clinical and metabolic profile of hypokalaemic periodic paralysis in adults: a single-centre experience.

BACKGROUND: Hypokalaemic periodic paralysis constitutes a heterogeneous group of disorders that present with acute muscular weakness. In this analysis, we discuss the aetiological factors that appear to be more common in the Indian population. METHODS: From 1995 to 2001, 31 patients presented with periodic paralysis (mean age 34.5 years, range 11-68 years). Of the 31 patients, 19 were men. The clinical and laboratory data of these patients were analysed. Patients were investigated for possible secondary causes of hypokalaemla. RESULTS: There were 13 patients (42%) with renal tubular acidosis, 13 with primary hyperaldosteronism (42%), 2 each with thyrotoxic periodic paralysis and sporadic periodic paralysis, and I with Gitelman syndrome. Of the 13 patients with renal tubular acidosis, 10 had proximal and 3 distal renal tubular acidosis. Three of these patients with renal tubular acidosis had Sjogren syndrome. The patients diagnosed to have renal tubular acidosis had significantly lower serum bicarbonate (18.7 [14.6] v. 29.6 [5.0] mEq/L; p < 0.05) and higher levels of chloride (107.5 [6.0] v. 99.5 [3.4] mEq/L; p < 0.05) compared with those who had primary hyperaldosteronism, although the potassium values were similar (2.4 [0.65] v. 2.26 [0.48] mEq/L; p = 0.43). All patients with primary hyperaldosteronism had hypertension at presentation and were proven to have adrenal adenomas. CONCLUSION: A significant number of patients in this study had secondary and potentially reversible causes of hypokalaemic periodic paralysis. The common causes were renal tubular acidosis and primary hyperaldosteronism. A detailed work-up for secondary causes should be undertaken in Indian patients with hypokalaemic periodic paralysis.

Acidosis, Renal Tubular↗
PubMed1979

Pyruvate carboxylase and phosphoenolpyruvate carboxykinase activity in leukocytes and fibroblasts from a patient with pyruvate carboxylase deficiency.

Normal values are given for the activities of pyruvate carboxylase (E.C.6.4.1.1), mitochondrial phosphoenolpyruvate carboxykinase (E.C. 4.1.1.32, PEPCK), and citrate synthase (E.C. 4.1.3.7) in fibroblasts, lymphocytes, and leukocytes. Also given are values for these enzymes in the leukocytes and fibroblasts from a severely mentally and developmentally retarded patient with proximal renal tubular acidosis and hepatic, cerebral, and renal cortical pyruvate carboxylase deficiency. In normals, virtually all of the mitochondrial PEPCK and pyruvate carboxylase activity was present in the mononuclear leukocyte fraction of whole venous blood. Cellular fractionation studies with human lymphocytes and fibroblasts demonstrated that all of the PEPCK activity in these cells is mitochondrial. Normal values for pyruvate carboxylase in leukocytes were 0.092 (0.070--0.208) mU/mg protein (n=5), in lymphocytes 0.154 (0.092--0.262) mU/mg protein (n=5), and in fibroblasts 1.36 (0.778--2.19) mU/mg protein (n=5). The patient with hepatic, renal, and cerebral pyruvate carboxylase deficiency had no detectable activity (less than 0.009 mU/mg protein) in his leukocytes and 0.018 mU/mg protein in his fibroblasts. Data from an assay for pyruvate carboxylase activity in the patient's fibroblasts show that the activity observed is significant but very close to the lower limits of the assay. Values for PEPCK in normal lymphocytes were 1.42 (0.824--1.88) mU/mg protein (n=5), in leukocytes 1.68 (1.64--1.72) mU/mg protein (n=2), and in fibroblasts 5.49 (3.94--6.33) mU/mg protein (n=6).

Acidosis, Renal Tubular↗
PubMedDate not supplied

Life threatening hyperkalemia and acidosis secondary to trimethoprim-sulfamethoxazole treatment.

We present a 77-year-old male with moderate chronic renal insufficiency from diabetic nephropathy who developed severe metabolic acidosis and life threatening hyperkalemia on treatment with regular dose of trimethoprim-sulfamethoxazole (TMP-SMZ) for urinary tract infection. The metabolic acidosis and hyperkalemia resolved upon appropriate medical intervention and discontinuation of TMP-SMZ. While hyperkalemia has commonly been reported with high dose of TMP-SMZ, severe metabolic acidosis is quite uncommon with regular dose TMP-SMZ. We emphasize that patients with renal tubular acidosis (RTA), renal insufficiency, aldosterone deficiency, old age with reduced renal mass and function, and angiotensin converting enzyme (ACE)-inhibitor therapy are at high risk of developing these severe and potentially life threatening complications.

Acidosis, Renal Tubular↗
PubMed1991

Renal tubular dysfunction in methylmalonic acidaemia.

Renal tubular function was assessed in seven patients with methylmalonic acidaemia not responsive to vitamin B12. Five patients failed to concentrate their urine normally and in these patients the glomerular filtration rate was also reduced. Fractional excretion of sodium was increased in four patients, fractional excretion of potassium in one patient and in three there was a decreased tubular reabsorption of phosphate. Although possibly representing primary tubular damage these findings were thought to be consistent with adaptive changes secondary to the reduced glomerular filtration rate. Two patients had evidence of a defect of urinary acidification and several had a degree of hyporeninaemic hypoaldosteronism suggesting type 4 renal tubular acidosis. In one patient with a mild variant no renal disease was detected. Decreased renal function and tubular abnormalities were common in patients with methylmalonic acidaemia. It is likely that they are linked and essentially secondary to the tubulo-interstitial nephritis that is histologically demonstrable on renal biopsy. The failure of urinary concentrating ability and the disturbed urine acidification will contribute to the metabolic derangement during episodes of decompensation.

Acidosis, Renal Tubular↗
PubMed1983

The influence of indomethacin on renal acidification in normal subjects and in patients with sickle cell anemia.

Patients with sickle cell nephropathy have been shown to have a distal type of incomplete renal tubular acidosis. We evaluated renal tubular acidification before and after indomethacin administration because prostaglandins have been shown to inhibit the transepithelial potential difference in the collecting tubule and since we previously found indirect evidence of increased prostaglandin synthesis in patients with sickle cell nephropathy. Indomethacin did not change urine pH in the sickle cell anemia (SCA) patients or in the control subjects. It induced, however, an increase in titratable acid excretion particularly in the SCA patients. Ammonium excretion decreased after indomethacin in the SCA patients while it did not change significantly in the control subjects. In the SCA patients, net acid excretion did not rise after indomethacin. In contrast there was an increase in net acid excretion after indomethacin administration in the control subjects. We conclude from our study that the inability of patients with SCA to lower urine pH to a normal extent after ammonium chloride loading is not improved by indomethacin. The decrease in ammonium excretion after indomethacin administration in SCA might be due to an effect of the prostaglandin synthesis inhibitor indomethacin on ammoniagenesis.

Acidosis↗
PubMed1991

Renal failure in adult onset hypophosphatemic osteomalacia with Fanconi syndrome: a family study and review of the literature.

Follow-up of a previously reported family with dominantly inherited adult onset hypophosphatemic osteomalacia with Fanconi syndrome and diabetes mellitus has shown that both the proposita and her affected sister have developed renal glomerular failure. We describe the evolution of renal failure in this family and discuss the possible mechanisms involved. The development of renal tubular acidosis in this condition further impairs renal function and we suggest that correction of systemic acidosis might improve renal function and prevent further decline in these patients.

Acidosis, Renal Tubular↗