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Trauma Case Presentation

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Clinical approach to renal tubular acidosis in children

*Corresponding author: Dr. Nivedita Kamath, Department of Pediatric Nephrology, St John’s Medical College Hospital, Bengaluru, Karnataka, India. [email protected]

How to cite this article: Reddy S, Kamath N. Clinical approach to renal tubular acidosis in children. Karnataka Paediatr J 2020;35(2):88-94.

Renal tubular acidosis (RTA) is a common inherited tubulopathy in children. Proximal RTA, usually secondary to a systemic metabolic disease, is characterized by a generalized dysfunction of the proximal tubule resulting in Fanconi syndrome. Distal RTA occurs due to mutation in the transporters of the distal tubule resulting in acidification defects. Hyperchloremic metabolic acidosis with normal anion gap is the characteristic feature of RTA. In addition to supportive therapy, specific treatment for the underlying etiology and regular monitoring of growth and laboratory parameters are of utmost importance.

Renal tubular acidosis

Fanconi syndrome, distal renal tubular acidosis.

• INTRODUCTION

The kidneys play a key role in preserving the acid–base homeostasis in the body by excretion of acid and regeneration of bicarbonate. Renal tubular acidosis (RTA) arises from the kidney’s inability to excrete enough acid or retain enough bicarbonate, in the presence of normal renal function, resulting in a clinical syndrome characterized by persistent normal anion gap hyperchloremic metabolic acidosis. [ 1 , 2 ]

• ACID–BASE HOMEOSTASIS: ROLE OF THE KIDNEY
• Bicarbonate reabsorption in the proximal tubule

The proximal tubule is responsible for reabsorption of 85–90% of filtered bicarbonate through secretion of protons (H + ) through the sodium hydrogen exchangers and proton pumps (H + ATPase). [ 1 , 3 ] In the lumen, the secreted hydrogen ions combine with HCO 3 - to form carbonic acid which rapidly dissociates into carbon dioxide and water, a reaction catalyzed by carbonic anhydrase. The carbon dioxide generated diffuses freely into the proximal tubule cell and reacts with water to form carbonic acid, a reaction catalyzed by carbonic anhydrase (CA II). Bicarbonate ions from the dissociated carbonic acid exit through the basolateral membrane by the sodium bicarbonate exchanger (NBCE1) [ 4 , 5 ] [ Figure 1 ].

• Urinary acidification in the distal tubule

The acidification of urine occurs in the distal collecting tubule and collecting duct. The intercalated cells A (secrete H + ), B (secrete HCO 3 - ) and principal cells (reabsorb sodium, water, and secrete K + ) of the distal nephron play a pivotal role in fine tuning acid and base excretion. The generated HCO 3 - is transported to the blood in exchange for chloride by the basolateral Cl - /HCO 3 - exchanger. The hydrogen ions secreted into the lumen by intercalated cells A are buffered by titratable acids (phosphate) and ammonia and excreted in the urine [ 6 ] [ Figure 2 ].

• TYPES OF RTA
• Type 1 RTA – distal RTA (dRTA)

dRTA, characterized by impaired hydrogen ion secretion in the distal tubules, is commonly seen due to inherited mutations of transporters in the distal tubule.

The hallmark of distal RTA is the inability to lower urine pH maximally in the presence of moderate-to-severe metabolic acidosis. Failure of the distal tubules to excrete the acid load generated by metabolism and in the growing bone in children leads to accumulation of acid and worsening base deficit. Extracellular bicarbonate and hydroxyapatite in the bones serve as buffers to neutralize the accumulated acid. Hypokalemia seen in RTA is due to proximal and distal wasting of sodium, leading to volume contraction and secondary hyperaldosteronism. It is more commonly associated with dRTA as potassium is the only available cation for exchange with sodium and distal secretion of H + is impaired. Hypercalciuria and hypocitraturia are seen in dRTA which make the children prone for early-onset nephrocalcinosis and nephrolithiasis. [ 7 ]

Inherited distal RTA [ 8 ]

Refer to [ Table 1 ].

Acquired dRTA

Autoimmune – Sjogren’s syndrome, systemic lupus erythematosus, and Graves’ disease

Medications – Amphotericin, lithium, and aminoglycosides.

RTA: Renal tubular acidosis, CNS: Central nervous system

• Type II RTA – proximal RTA

Proximal RTA is caused by an impairment of bicarbonate reabsorption with intact distal acidification mechanisms and is characterized by decreased renal bicarbonate threshold (14–18 mEq/L), defect in ammonia generation, and ability to lower urine pH <5.5. Hypokalemia is commonly seen in proximal RTA. [ 9 ]

Proximal RTA usually occurs in association with other tubular defects as a part of the generalized proximal tubular defect – Fanconi syndrome (inherited or acquired). Isolated proximal RTA is rare.

Isolated proximal RTA

Autosomal dominant

Autosomal recessive – with ocular abnormalities (NBCE1), CA II mutation.

Inherited – CA II inhibitors

Fanconi syndrome [ 10 ]

Fanconi syndrome is a generalized dysfunction of the proximal tubule resulting in hypokalemia, polyuria, bicarbonate wasting, glycosuria, low-molecular-weight proteinuria, generalized aminoaciduria, and phosphaturia resulting in hypophosphatemia.

Acquired causes

Autoimmune conditions – Sjogren’s syndrome

Malignancy – Acute lymphatic leukemia, multiple myeloma

Medications – Valproic acid, acetazolamide, cisplatin, lamivudine, tenofovir, outdated tetracyclines, aminoglycosides, Chinese herbs, heavy metals (lead, cadmium, mercury, and copper).

• Type III RTA – mixed RTA

Type III RTA, an outdated terminology, has combined features of proximal and distal RTA. Association of Type III RTA with osteopetrosis has been commonly described with loss of function mutation of CA II. [ 11 ]

• Type IV RTA – hyperkalemic RTA

In hyperkalemic RTA, the primary defect lies in the regeneration of bicarbonate secondary to lack of adequate urinary ammonia. This is seen in conditions with aldosterone deficiency or resistance. [ 12 ] The acquired causes of aldosterone resistance are the most common causes encountered in clinical practice.

Congenital hypoaldosteronism

Pseudohypoaldosteronism.

Urinary tract – posterior urethral valve, reflux nephropathy, pyelonephritis

Autoimmune conditions – interstitial nephritis

Drugs – trimethoprim, nonsteroidal anti-inflammatory drugs, calcineurin inhibitors, angiotensin-converting enzyme inhibitors

• CLINICAL CLUES TO SUSPECT RTA IN A CHILD

RTA must be suspected in any child who presents with

Failure to thrive

Resistant rickets

Polyuria, polydipsia

Salt craving – preference for salty foods

Symptoms of hypokalemia

Acidotic breathing

Nephrocalcinosis/nephrolithiasis.

In younger children, polyuria may manifest as recurrent episodes of dehydration in the absence of diarrheal losses, normal urine output in the presence of dehydration, or preference for water over other liquids. Hypokalemia may manifest as abdominal distension, muscle weakness – head lag, or a sudden onset of hypotonia/paralysis usually following an acute illness.

EVALUATION OF RTA [ 9 , 13 ]

The first step in the evaluation of RTA is the identification of hyperchloremic normal anion gap metabolic acidosis in the presence of normal renal function. [ 9 ]

Anion gap: The anion gap should be calculated by a simultaneous measurement of serum electrolytes and bicarbonate.

Anion gap = [Na + - (Cl - + HCO 3 - ) Normal anion gap is 8–12 meq/L.

Anion gap may be affected by the serum albumin levels.

Corrected anion gap = calculated anion gap+ 2.5 (Normal albumin – measured albumin).

The two common conditions causing normal anion gap are diarrhea and RTA.

Urine anion gap

The urine anion gap is an indirect measurement of the urinary ammonia excretion in response to metabolic acidosis.

Urinary anion gap = (Na + +K + )-Cl - (measured in the urine).

Normally, the urine anion gap is positive. In the presence of metabolic acidosis, the normal kidney is able to generate ammonium (NH 4 + ) and excrete it along with Cl - , making the urine anion gap negative. A persistent positive anion gap in the presence of metabolic acidosis is suggestive of a renal acidification defect.

Caveats: Urine anion gap estimates ammonium excretion only in chronic metabolic acidosis. If the urine pH>6.5, bicarbonate is excreted, hence, urinary anion gap does not reflect urinary ammonium levels.

Urine osmolal gap: This is used in situations where the urine anion gap is not reliable.

Urine osmolal gap = measured osmolality – calculated osmolality.

Calculated urine osmolality= 2[Na + +K + ] + urea + glucose 6 18

Urinary ammonium excretion is estimated to be half the urinary osmolal gap and is considered to be increased if it >100 mosm/kg.

Once, the diagnosis of RTA is confirmed, the next step is to differentiate between the different types of RTA [ 13 ]

Urine analysis: A routine analysis of urine for the presence of proteinuria (usually non-nephrotic range, i.e., urine albumin 1+–2+), glycosuria indicates the presence of generalized tubular wasting seen with Fanconi syndrome

Urine pH: A urine pH >5.5 in the presence of metabolic acidosis is suggestive of distal acidification defect seen with dRTA. If systemic acidosis is mild, it can be induced using the ammonium chloride loading test. Ammonium chloride is given at 0.1 mg/kg followed by measurement of urine pH every hour for the next 2–8 h. The total plasma CO 2 should fall by 3–5 meq/L and urine pH should be <5.5. Ammonium chloride loading test should not be performed in children with moderate-to-severe acidosis (serum bicarbonate <17 meq/L)

Caveat: The urine pH should be measured in a freshly voided sample of urine using a pH meter. The urine pH must always be interpreted with the urine sodium. Low urine sodium will result in low H + secretion

Fractional excretion of bicarbonate: Following a bicarbonate loading test (sodium bicarbonate 0.5 meq/ ml is administered at 3 ml/min), measure the urine pH every 30–60 min till 3 consecutive samples show a pH >7.5.

The fractional excretion of bicarbonate is calculated

The normal fractional excretion of bicarbonate is <5%. A fractional excretion > 15% suggests bicarbonate wasting

Urine-blood PCO 2 : After a load of sodium bicarbonate, the secreted H + reacts with the luminal HCO 3 - in the distal lumen forming carbonic acid. In the absence of CA II, this slowly gets converted to CO 2 which remains trapped in the lumen. At a urine pH>7.5 and serum bicarbonate 23–25 meq/L, the urine PCO 2 should be >70 mmHg. The urine-blood PCO 2 > 20 mmHg is seen with normal acid secretion. Urine-blood PCO 2 <20 mmHg indicates a defect in distal acid secretion

Caveats: Urine for PCO 2 should be collected from a freshly voided sample in a sealed syringe using mineral oil and measured by a blood gas analyzer

Furosemide fludrocortisone test: The furosemide fludrocortisone test assesses the ability of the distal tubule to secrete acid. Furosemide increases the distal sodium delivery, which, in turn, enhances distal H + secretion. Fludrocortisone also increases H + secretion. After an overnight fasting, early morning pH is checked, if urine pH >5.5, oral furosemide is administered at 1 mg/kg and fludrocortisone at 0.025 mg/kg. Urine pH is measured hourly for 4–6 h. A urine pH <5.5 indicates an intact distal acidification mechanism.

Caveat: Ensure normal serum potassium before the test

Fractional excretion of phosphate: Fractional excretion of phosphate can be measured using a spot or timed urine sample along with simultaneously measured serum values. The normal fractional excretion of phosphate is 8–15%. Fractional excretion >15% suggests phosphate wasting.

The tubular resorption of phosphate corrected for glomerular filtration rate (TmP/GFR) is a better indicator and can be calculated using the Bijvoet’s nomogram or using the formula. Normal TmP/GFR is 2.8–4.4 mg/dL, which will be reduced in Fanconi syndrome.

Generalized aminoaciduria: Generalized aminoaciduria> 5% is suggestive of proximal tubular wasting

Low-molecular-weight proteinuria: Elevated levels of β2 microglobulinuria is suggestive of Fanconi syndrome

Urinary calcium creatinine ratio: The spot urinary calcium to creatinine ratio is increased (>0.2 in children >2 years of age) in distal RTA and some forms of proximal RTA like Dent’s disease. A 24 h urinary calcium excretion >4 mg/kg/day is considered as hypercalciuria

Hypocitraturia – 24 h urine citrate is estimated using colorimetric methods. 24 h urine citrate levels of <2 mg/kg is termed as hypocitraturia

Ultrasound examination of the kidney – for nephrocalcinosis and nephrolithiasis – seen in distal RTA

Evaluation for extra-renal involvement – Eye examination could reveal cystine crystals in children with cystinosis. Assessment of hearing must be done in all children with dRTA.

The clinical and laboratory presentation of proximal and distal RTA is compared in [ Table 2 ].

RTA: Renal tubular acidosis

• TREATMENT FOR RTA

The goal of treatment is to correct metabolic acidosis, prevent bony deformity, and improve growth. Treatment for dRTA consists of alkali therapy to correct metabolic acidosis. Bicarbonate supplements (4–6 mEq/kg/day in infants to 2–4 mEq/kg/day in children), in the form of potassium citrate formulations, are recommended to ensure normal growth. [ 14 ] Hypokalemia improves with correction of acidosis, but some children require long-term supplements (1–2 mEq/kg/day). Potassium citrate in addition to correcting metabolic acidosis and hypokalemia provides citrate which reduces hypercalciuria. It must be remembered that these measures will not retard the progression of nephrocalcinosis or hearing impairment. Regular screening for nephrocalcinosis and hearing impairment must be done and targeted therapy instituted.

• Type II RTA

Syrups – Potrate (Bicarbonate and potassium 2 mEq/ml), Nodosis (0.8 mEq/ml)

Tablets – Sodamint (3.6 mEq/300 mg, 6 mEq/500 mg, 7.8/650 mg), Acidose 500 mg.

Phosphate supplements (Addphos sachet 500 mg, K-Phos tablet 500 mg, and Joules solution 30 mg/ml) are administered at 20–40 mg/kg/day in 3–4 divided doses. The common side effects include diarrhea and abdominal pain. Active Vitamin D supplementation (calcitriol 20–40 ng/kg/day) with regular assessment of urine calcium and annual renal scan to monitor for nephrocalcinosis is recommended. [ 15 ] Nutrition in children with RTA is compromised due to polydipsia, polyuria with increased losses of sodium and other nutrients, and poor intake. Intake of calorie dense foods rich in potassium and phosphorus with adequate fluids is encouraged.

• Hyperkalemic RTA

The primary treatment is to stabilize serum potassium concentration by stopping all potassium containing medications, restricting dietary sources, and addition of potassium-binding resins (calcium polystyrene sulfonate K-Bind 15 g sachets – 1 g/kg/day in 2–3 divided doses). [ 12 ] Bicarbonate supplement should be administered to correct acidosis.

• SPECIFIC DISORDERS CAUSING RTA
• Proximal RTA

Cystinosis is the most common hereditary cause of Fanconi syndrome. It is an autosomal recessive lysosomal storage disorder resulting from a defect in the gene CTNS (SLC3A1, SLC7A9) encoding for cystinosin, a lysosomal cystine-proton cotransporter. The commonly affected organs include kidney, eyes, thyroid, pancreas, gonads, and central nervous system. The infantile nephropathic form is seen in 95% of all cases of cystinosis. [ 16 ] There is early onset of renal involvement with rapid progression to end-stage renal disease by the end of the first decade. Ocular (corneal deposits in 100% by 18 months), endocrine (50–70% hypothyroidism by the 2 nd decade), gonadal (70% have primary hypogonadism), and central nervous system (encephalopathy in 45% by late second decade) involvement are common extrarenal manifestations. [ 17 ] Cystine depletion therapy with cysteamine delays end-stage renal disease but does not stop its progression. [ 18 ]

Tyrosinemia

Hereditary tyrosinemia Type I is an autosomal recessive inborn error of metabolism, due to deficiency of fumarylacetoacetate hydrolase, mainly affecting the liver and kidney during early infancy. Hepatic manifestations include abnormal synthetic function with coagulopathy, transaminitis, hypoglycemia, acute liver failure in the first few weeks to life, and progression to cirrhosis in early childhood. The spectrum of renal involvement varies in severity from hypophosphatemic rickets, generalized aminoaciduria, and proteinuria with occasional glucosuria (lower incidence in view of decreased plasma glucose levels) to long-term complications of glomerulosclerosis, nephrocalcinosis, and chronic kidney disease. Treatment with low phenylalanine and low tyrosine diet improves renal tubular dysfunction. An effective medical treatment with nitisinone (2-[2-nitro-4-trifluoromethylbenzoyl]-1,3-cyclohexanedione) has optimal long-term preventive effects if initiated early and when plasma levels are maintained at 40–60 μmol/L. [ 19 ]

Dent’s disease

Dent’s disease is an X-linked hereditary form of Fanconi syndrome characterized by proximal tubular dysfunction – low-molecular-weight proteinuria, hypercalciuria, hypophosphatemic rickets, nephrolithiasis, nephrocalcinosis, and progression to end-stage renal disease. More than 50–60% of cases are attributed to mutations in CLCN5 gene (Dent I), 15% have mutations involving the OCRL1 gene (Dent 2). Extrarenal symptoms are rare in Dent’s disease. [ 20 ] There is no specific treatment available to retard the progression to end-stage renal disease, which commonly occurs by 30–50 years of age.

Lowe syndrome

Lowe syndrome (oculocerebrorenal syndrome) is an X-linked disorder resulting from mutation in OCRL gene (encoding α-phosphatidylinositol 4, 5-biphosphate phosphatase). Bilateral cataract, generalized hypotonia at birth, and proximal RTA with progression to chronic kidney disease by the second decade are characteristic of Lowe syndrome. Treatment is primarily supportive and involves cataract removal, glaucoma control, targeted rehabilitation therapy, and correction of tubular dysfunction with supplements. [ 21 ]

Glycogen storage disorder (GSD)

GSD Type Ia is an autosomal recessive disorder with deficiency of glucose-6-phosphatase-α. Children with GSD I have failure to thrive, hypoglycemia with hepatorenomegaly, hyperuricemia, hyperlipidemia, and proximal RTA.

The other GSD with Fanconi phenotype is Fanconi Bickel syndrome, also referred to as GSD XI, which is caused by a deficiency of GLUT2 transporter, secondary to mutations in SLC2A2 and is characterized by failure to thrive, chronic diarrhea, fasting hypoglycemia, postprandial hyperglycemia, hepatomegaly, and proximal tubular dysfunction typically manifesting in infancy. Dietary modification with restriction of glucose and galactose with incorporation of cornstarch is the main treatment. [ 22 ]

Autosomal dominant distal RTA occurs due to heterozygous mutation of anion exchanger (AE1) in the basolateral surface of the Type A intercalated cells. The usual presentation is during adolescence with features of nephrocalcinosis, nephrolithiasis, occasional rachitic changes, and mild or compensated hyperchloremic metabolic acidosis. Hereditary spherocytosis and ovalocytosis can occur secondary to involvement of AE1 on the erythrocyte membrane. [ 23 , 24 ]

Autosomal recessive

Mutations in ATP6V1B1, ATP6V0A4, and SLC4A1 present in early infancy or childhood with failure to thrive, episodes of vomiting or dehydration, rickets, and nephrocalcinosis with severe metabolic acidosis. These autosomal recessive distal renal tubular disorders are associated with early-onset bilateral sensorineural hearing loss in those with ATP6V1B1 mutations. [ 25 ]

Proximal RTA secondary to cystinosis, Dent’s disease, etc., have a relentless progression to chronic kidney disease. Although the clinical presentation of proximal tubular dysfunction may be variable, most causes of pRTA are associated with severe growth failure and bony deformities despite adequate treatment. The metabolic abnormalities are often hard to correct despite large supplementary doses.

Distal renal tubular dysfunction is often limited to the kidney alone. They rarely progress to chronic kidney disease. Growth impairment, bony deformities, and metabolic parameters are correctable with adequate therapy.

Children with renal tubular acidosis require a thorough evaluation to ascertain the etiology of RTA. Regular long-term follow-up and monitoring of growth, bony deformities and renal function is required.

Declaration of patient consent

Patient’s consent not required as there are no patients in this study.

Financial support and sponsorship

Conflicts of interest.

There are no conflicts of interest.

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• EVALUATION OF RTA[9,13]

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The Case for Free Trade and the Role of RTAs

Then p will change as tariff rates are adjusted from pre-rta mfn levels to ... each spoke enjoys preferential access only to the hub ... – powerpoint ppt presentation.

• P. J. Lloyd and Donald MacLaren
• University of Melbourne
• Regional Trade Agreements and the WTO
• Geneva, 14th November 2003
• Introduction
• 1. Predictions of trade theory
• 2. Evidence from CGE models
• 3. Hub and spokes arrangements
• 4. Effects of RTAs on multilateral trade negotiations
• 5. Conclusions
• The theory of RTAs on which GATT Article XXIV could have been based
• Viners contribution
• trade creation and trade diversion
• ... where the trade-diverting effect is predominant, one at least of the member countries is bound to be injured, the two combined will suffer a net injury, and there will be injury to the outside world and to the world at large.
• During the 1950s and 1960s it was demonstrated that parts of Viners predictions may not hold, e.g., trade diversion did not necessarily imply a lowering of welfare
• Modern analytics for a single, open economy with perfect competition and constant returns to scale (first-generation assumptions), can use a compensation function
• B e ( p, u ) g ( p, v ) ( p p ) m (1)
• where e is national expenditure, g is national product and ( p p ) m is trade tax revenue
• B measures the compensation needed to allow households in the economy to reach a given level of utility
• Suppose this economy joins an RTA
• Then p will change as tariff rates are adjusted from pre-RTA MFN levels to preferential RTA levels
• ? B ? e ? ? g ? ? R
• which can be expanded, after some algebra, to give
• ? B ?trade volume ?intra-union terms of trade ?extra-union terms of trade (2)
• The sign of ?B is ambiguous
• If ?B 0, it loses
• However, there is a presumption that members gain from the RTA
• This expression for ?B also shows clearly that measuring changes in trade volumes alone will not predict whether a country gains or loses
• Other assumptions
• imperfect competition (love of variety and economies of scale) second generation models
• investment, factor accumulation and growth third generation models
• Second and third generation models tend to strengthen the presumption that members gain
• For second-generation models, equation (2) becomes
• ? B ?trade volume ?intra-union terms of trade ?extra-union terms of trade ?output ?average cost ?varieties
• What are the welfare effects for excluded countries which, pre-RTA, traded with the members?
• In first generation models, theory predicts that excluded members, as a group, lose, although some individual countries may gain
• In second generation models, theory predicts that these losses may be greater than in first-generation models
• What does the evidence from computable general equilibrium (CGE) models suggest?
• data base of bilateral trade flows
• data base of transportation costs and tariff rates
• tariff on good i from region r to region s
• behavioural equations
• accounting identities
• design of the experiment
• values of the elasticities
• the Armington assumption and CES functions biases
• Summary of Results
• Formation of NAFTA (Brown et al. (1992))
• using a second generation model they found that
• each member gained
• 31 excluded countries lost
• using a third generation model they found that
• the gains to Mexico were three times greater
• the excluded countries lost
• EU Single market (Haaland and Norman (1992))
• using a second generation model and representing deeper integration by a 2.5 reduction in intra-EU trade costs, they found that
• EU gained 0.64 of GDP (internal markets integrated) and 0.40 (internal market segregated)
• EFTA lost 0.22 and 0.15 of GDP, respectively
• conclusion excluded members lose but the size depends on assumptions about market structure in the RTA
• Asia-Pacific Region
• Conclusions about gainers and losers
• global, multilateral trade liberalisation generates the greatest gains to the world economy
• the size of the gains is dependent upon the underlying theory, the base period and the design of the experiment
• countries, in aggregate, lose from being excluded from an RTA
• the larger the RTA, the larger are these losses
• in principle, a Pareto improvement is possible but in the absence of inter-regional transfers, RTAs are undesirable for outside countries
• The configuration of RTAs has changed
• from a given country being a member of one and only one RTA, if a member at all
• to the same country being a member of more than one RTA
• The configuration today has been described as hub and spokes
• The hub may be
• a single country, e.g., the U.S. or Singapore
• a group of countries (plurilateral hub), i.e., an RTA itself, e.g., ASEAN
• The spoke may be
• a single country
• an RTA (plurilateral spoke)
• Hubs and spokes create
• two layers of discrimination
• the hub enjoys access to each and every spoke on a preferential basis as most hubs are developed countries, it is to them that the bulk of the gains have gone
• each spoke enjoys preferential access only to the hub
• each hub and each spoke discriminates against non-members
• complex rules of origin
• forces for RTAs to coalesce into regional blocs
• increased discrimination against excluded countries, particularly developing and least-developed countries
• The growth of RTAs may affect the rate of multilateral liberalisation by
• affecting the pace of liberalisation from MTNs the building block or stumbling block debate
• affecting the pace of unilateral liberalisations
• Evidence on the first
• if based on theory, is ambiguous, depending on whether the objective is welfare maximising or political self-interest
• if based on empirical evidence pre-Cancún, RTAs have not slowed the pace
• post-Cancún, the EU and the U.S. have signalled a passive role in MTN but a pro-active role in RTAs
• Evidence on the second is mixed
• countries in ASEAN, CER and Latin America have continued to liberalise unilaterally within their RTAs
• within NAFTA, Mexico and Canada have done likewise
• neither the EU nor the U.S. has engaged in significant unilateral liberalisation over two decades
• the growth in RTAs has substantially increased discrimination in world trade
• with the creation of plurilateral hubs and spokes, that discrimination has become multi-layered
• rules of origin have become more complex
• Article XXIV of GATT has failed to protect the interests of outside countries because
• it was based on inadequate trade theory
• it is inconsistent with the elimination of discriminatory treatment in international commerce (preamble to GATT 1947, see WTO (1995))
• the elimination of discriminatory treatment in international trade relations appears in the preamble to the Marrakesh Agreement establishing the WTO (WTO (1995)) and yet it, too, is being ignored by those Members who are forming RTAs
• Brown, D. K., A. V. Deardorff and Stern, R. M. (1992), A North American Free Trade Agreement Analytical Issues and Computational Assessment, The World Economy, 15, 11-29
• Haaland, J. and V. Norman (1992), Global production effects of European integration, in L. A. Winters (ed.) Trade Flows and Trade Policy after 1992, Cambridge University Press, Cambridge
• Scollay, R. and J. P. Gilbert (2001), New Regional Trading Arrangements in the Asia Pacific?, Institute for International Economics, Washington, D. C.
• Viner, J. (1950), The Customs Union Issue, Carnegie Endowment, New York
• WTO (1995), The Results of the Uruguay Round of Multilateral Trade Negotiations The Legal Texts, Geneva

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Distal Renal Tubular Acidosis in Sjögren's Syndrome: A Case Report

Scarlet louis-jean.

1 Department of Medicine, American University of Antigua, Saint John's, ATG

Patrick R Ching

2 Department of Medicine, University of Maryland Medical Center Midtown Campus, Baltimore, USA

Allison Wallingford

Sjögren’s syndrome is an autoimmune lymphocytic infiltrative disease that leads to chronic inflammatory and degradatory changes to exocrine glands and extra-glandular systemic organs. It rarely affects children and adolescents. In cases where adolescents are affected, a paucity of sicca symptoms, xerostomia, and xerophthalmia often leads to a missed diagnosis. Consequently, the first presenting sign of Sjögren’s syndrome in adolescents may be heterogeneous, with varying clinical symptoms related to parotitis or systemic organ involvement. In this case report, we discuss a 19-year-old girl with distal renal tubular acidosis (RTA), who had experienced severe hypokalemic episodes since the age of 14 years; the patient was eventually diagnosed with Sjögren’s syndrome. She was managed and maintained on potassium and alkali repletion therapy.

Introduction

Renal tubular acidosis (RTA) is characterized by renal tubular impairment in balancing physiologic acid-base. It often results from a defect in tubular transporters, which participate in the secretion or uptake of specific ions, due to congenital causes, exposure to nephrotoxic drugs, diuretic abuse, autoimmune disease, or malignancy (e.g., multiple myeloma). There are three major types of RTA: distal or type 1, proximal or type 2, and hyperkalemic or type 4. All three types of RTA are characterized by a positive urine anion gap, hyperchloremic non-anion gap metabolic acidosis, alkalotic or acidotic urine pH, and serum potassium derangements (hypo- or hyperkalemia). 

Distal RTA (type 1 or classic RTA), which is the focus of this case report, can be further defined by an alkalotic urinary pH (>5.5) and profound hypokalemia (<3 mmol/L). It is often caused by an impairment in hydrogen ion secretion in the distal renal alpha-intercalated cells. Consequently, due to an impaired luminal gradient, ionic wasting occurs, leading to the possible development of nephrocalcinosis, nephrolithiasis, rickets/osteomalacia, muscle weakness, and respiratory failure. Distal RTA can be a rare complication of Sjögren’s syndrome in adolescents, although studies have demonstrated that approximately a greater proportion of adult patients develop defects in distal tubular acidification due to tubulointerstitial nephritis [ 1 ]. In this report, we discuss a case of distal RTA secondary to Sjögren’s syndrome in an adolescent patient and her management with potassium and alkali repletion.

Case presentation

A 19-year-old girl with a remote history of bipolar disorder and a five-year history of multiple hospitalizations for hypokalemic paralysis was brought to the emergency room for sudden-onset bilateral thigh cramping progressing to the shoulder and biceps after awakening from a nap. The patient reported that her symptoms were similar to her previous episodes and were exacerbated by her menses, which she had recently completed four days prior to symptom onset. A review of systems did not reveal syncope, gastrointestinal disturbances, urinary symptoms, joint pain, rashes, diuretic/laxative abuse, or suicidal or homicidal ideation. There was no family history of autoimmune diseases. She endorsed smoking cannabis occasionally and denied tobacco or alcohol use. Aside from her potassium chloride and sodium bicarbonate regimen, the patient was not on any psychotropic or other medications.

Except for bradycardia (heart rate of 50 bpm), her vital signs were within normal limits and physical examination was unremarkable. Results of initial lab tests showed sodium of 143 mmol/L, potassium of 2.1 mmol/L, chloride of 110 mmol/L, and bicarbonate of 17 mmol/L. Venous blood gas pH was 7.21. Urine sodium was 74 mmol/L, urine potassium was 18.4 mmol/L, and urine chloride was 68 mmol/L. Urinalysis showed a urinary pH of 7.0 without blood or protein (Table ​ (Table1). 1 ). The renal sonogram did not show nephrolithiasis. Electrocardiogram showed sinus bradycardia, delayed intraventricular conduction, and U waves. With a positive urine anion gap of 24 and hyperchloremic non-anion gap metabolic acidosis, she was diagnosed with distal RTA. She was promptly hydrated with intravenous normal saline and was given potassium chloride and sodium bicarbonate, which corrected both her hypokalemia and hyperchloremic non-anion gap metabolic acidosis. She was later discharged on oral potassium chloride and sodium bicarbonate.

ANA: antinuclear antibody; anti-dsDNA: anti-double-stranded DNA antibody; anti-SSA: anti-Sjögren’s syndrome-related antigen A; anti-SSB: anti-Sjögren’s syndrome-related antigen B; anti-U1-RNP: anti-U1 ribonucleoprotein antibody

Over the next few months, the patient had several similar presentations at hospitals for upper and lower extremity weakness and paralysis secondary to hypokalemia. Initially, exposure to synthetic cannabinoids was believed to have induced the patient’s severe hypokalemia and acid-base disturbances in the absence of known nephrotoxic drug exposure and autoimmune history. Additionally, suspicion of disordered eating/exercise or diuretic/laxative abuse as a contributor to her metabolic derangements was low based on patient report and chart review. On further probing, she admitted to infrequent, intermittent dry mouth and dry eyes with a sand-like sensation associated with ocular pruritus for several months, but denied any history of eye inflammation, use of artificial teardrops, or increased occurrence of cavities.

Her autoimmune panel was positive for antinuclear antibodies (ANA) (1:2560) and anti-Ro/SSA antibody (SSA-52: 261 AU/mL and SSA-60: 130 AU/mL) and negative for anti-double-stranded DNA (dsDNA), anti-Smith, anti-La/SSB, and anti-U1-ribonucleoprotein (RNP) antibodies (Table ​ (Table1). 1 ). Diagnostic tests for SCN4A deletion/duplication, urine organic acids, plasma amino acids, carnitine (total and free), acylcarnitine, and urine porphyrins were all within normal reference values. Given the patient did not have other derangements of urine and serum electrolytes, other causes of renal tubulopathies, such as Bartter, Gitelman, and Fanconi syndromes were subsequently ruled out. Based on sicca symptoms and positive anti-Ro/SSA antibodies, a presumptive diagnosis of Sjögren’s syndrome was made. She was advised to follow up with nephrology and rheumatology in the outpatient setting; however, the patient never attended her appointments.

Sjögren’s syndrome is an autoimmune exocrinopathy with variable systemic involvement caused by monolymphocytic infiltration. Systemic associations can include cardiac involvement leading to prolonged QT, pulmonary disease, neurologic involvement, and tubulointerstitial disease (e.g., tubulointerstitial nephritis, RTA, Fanconi syndrome, and glomerulonephritis). However, the true mechanism behind RTA is not well known. Available data point to reduced hydrogen electrolyte secretion consequently due to immune-mediated injury of the hydrogen-ATPase pump of the alpha-intercalated cells or autoantibody directed against carbonic anhydrase II [ 2 ].

There are two classifications of the disease: primary and secondary Sjögren’s syndrome. Primary Sjögren’s syndrome is thought to be an isolated condition not often caused by another pathological process, whereas secondary Sjögren’s syndrome is a sequela of other rheumatological diseases like systemic lupus erythematosus, rheumatoid arthritis, or scleroderma. Sjögren’s syndrome often affects middle-aged women, with a sex-adjusted prevalence of 2.2-10.3 per 10,000 individuals [ 3 ]. It is often diagnosed based on the American-European Consensus Group’s (AECG) criteria for Sjögren’s syndrome, which details ocular symptom/sign(s) of three months' duration, oral symptom/sign(s), lymphocytic sialadenitis on minor salivary gland biopsy, and auto-antibodies (anti-Ro/SSA and -La/SSB) as the basis for a diagnosis [ 4 , 5 ]. The diagnosis of primary Sjögren’s syndrome requires patients to meet four of the six criteria, which must include histopathology or autoantibodies, or any three of four objective criteria [ 4 ]. However, only 22% of patients have been reported to meet the AECG or American College of Rheumatology's (ACR) criteria for diagnosis as the required tests are not often performed in clinical practice [ 3 ]. Moreover, the current diagnostic criteria have been shown to have limited scope in juvenile cases, thereby posing a diagnostic challenge in adolescents who present heterogeneously and do not have classical sicca symptoms [ 6 , 7 ]. 

Recurrent parotid swelling has been documented as the most common presenting feature in the pediatric population, occurring more frequently than sicca symptoms [ 8 ]. The most common renal manifestation of Sjögren’s syndrome is tubulointerstitial nephritis, which may present as RTA in adult populations; however, RTA is a rare occurrence in the pediatric population as it accounts for 7.1-19.2% of cases who present with renal potassium wasting or hypokalemic paralysis [ 9 ]. Our patient first presented with an episode of muscle weakness and syncope in the setting of severe hypokalemia at the age of 14 years, often exacerbated by menses. In a study by Sandhya et al., adequate levels of estrogen and androgens were associated with increased protection of glandular epithelial cells from apoptosis and lymphocytic infiltration [ 10 , 11 ]. Therefore, the mechanism whereby decreased levels of sex hormones lead to lymphocytic tubulopathy may explain an association with the patient’s hypokalemic exacerbation during menses especially since she endorsed increased intake of her potassium pills during her menstrual cycles.

Over a period of five years, our patient was hospitalized for several episodes of symptoms related to severe hypokalemia secondary to distal RTA. With every hospitalization, the opportunity to assess the patient for Sjögren’s syndrome was missed, likely due to a lower index of suspicion based on age and absence of classical signs and symptoms at the time. Pediatric patients with Sjögren’s syndrome have been observed to develop sicca symptoms with increasing age, which may explain why our patient endorsed infrequent intermittent pruritic dry eyes and difficulty chewing crackers during a later hospitalization [ 12 ]. Nonetheless, once obtained, autoimmune antibodies for Sjögren’s syndrome demonstrated high serum ANA and anti-Ro/SSA-52 and -60, suggesting a higher suspicion for the disease, especially as SSA-52 has a prevalence rate of 63.2% in Sjögren’s syndrome [ 13 ]. The presence of high anti-Ro/SSA titers has also been associated with a greater likelihood of earlier disease onset and extra-glandular involvement [ 10 , 14 , 15 ].

Due to a confluence of social issues that involved a contentious relationship with her parents, the patient did not follow up on her scheduled outpatient appointments. Attempts by social workers to improve the patient’s access to medical appointments were to no avail. If the patient had undergone additional diagnostic studies, glucocorticoid therapy could have been initiated. However, while glucocorticoid therapy is the universal treatment modality for patients with Sjögren’s-induced renal involvement, a review of the literature has demonstrated the recurrence of hypokalemia and acidosis with glucocorticoid tapering, especially in cases where early treatment was not started [ 6 , 1 , 16 ]. As curative treatments remain to be elucidated, monitoring for complications such as nephrocalcinosis, nephrolithiasis, and rickets/osteomalacia should also take place along with ongoing treatment of metabolic derangements and symptoms.

Conclusions

The early identification and diagnosis of Sjögren’s syndrome can be a difficult challenge as current diagnostic guidelines contribute to a higher likelihood of missed diagnosis in pediatric cases. Juvenile patients may present with heterogeneous findings inconsistent with the classic presentation of the disease. Thus, adolescent patients presenting with evidence of renal pathologies should undergo further diagnostic investigations to rule out Sjögren’s syndrome while simultaneously receiving prompt management of metabolic derangements with potassium and alkali treatment to prevent potentially fatal sequelae.

The content published in Cureus is the result of clinical experience and/or research by independent individuals or organizations. Cureus is not responsible for the scientific accuracy or reliability of data or conclusions published herein. All content published within Cureus is intended only for educational, research and reference purposes. Additionally, articles published within Cureus should not be deemed a suitable substitute for the advice of a qualified health care professional. Do not disregard or avoid professional medical advice due to content published within Cureus.

The authors have declared that no competing interests exist.

Human Ethics

Consent was obtained by all participants in this study

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Khanty-Mansi Autonomous Okrug - Yugra, Russia

The capital city of Khanty-Mansi okrug: Khanty-Mansiysk .

Khanty-Mansi Autonomous Okrug - Yugra - Overview

Khanty-Mansi Autonomous Okrug - Yugra is a federal subject of Russia, part of the Urals Federal District. Khanty-Mansiysk is the capital city of the region.

The population of Khanty-Mansi Autonomous Okrug - Yugra is about 1,702,200 (2022), the area - 534,801 sq. km.

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Khanty-mansi okrug coat of arms.

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Khanty-mansi okrug latest news and posts from our blog:.

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History of Khanty-Mansi Autonomous Okrug - Yugra

Yugra is the historical homeland of the Ob-Ugric peoples: Khanty, Mansi, Nenets, and Selkup. They were engaged in hunting, fishing, cattle breeding. After the Turkic peoples pushed them from south to north, these peoples had to apply their skills in more severe conditions. It is at this new location Ugrians began to domesticate deer.

In the first half of the second millennium AD, the main features of the material and spiritual culture of Khanty, Mansi and forest Nenets were formed. It is believed that since then they have not undergone major changes. Since the second half of the 13th century, a new factor in the development of the region was its entry into the Golden Horde.

At the end of the 14th century, the collapse of the Golden Horde led to the emergence of a separate Tyumen Khanate. In 1495, the Siberian Khanate appeared. At that time the basic principles of political, administrative and socio-economic organization of this territory were developed. The region was called Ugra or Yugra.

More historical facts…

The region became part of Russia in the end of the 16th century. From the middle of the 18th century, this region became a place of exile for criminals. December 10, 1930, Ostyako-Vogul national okrug was formed with the center in the settlement of Samarovo. Construction of a new center began 5 km away from it. In February 1932, the new center of the region was named Ostyako-Vogulsk.

In 1934, the first steps to find oil and natural gas in the region were taken. October 23, 1940, Ostyko-Vogul national okrug was renamed Khanty-Mansi national okrug and Ostyko-Vogulsk was renamed Khanty-Mansiysk. August 14, 1944, the region became part of Tyumen Oblast. On January 27, 1950, Khanty-Mansiysk became a city.

On September 21, 1953, in Berezovo, the first natural gas in Western Siberia was produced. On June 23, 1960, the first oil in Western Siberia was discovered near Shaim. This was followed by the discovery of many other oil and natural gas fields. Along with the industrial exploitation of oil and gas fields, the timber industry developed rapidly.

By the end of the 20th century, under the influence of demographic and socio-economic developments the Khanty-Mansi region in fact lost its national basis. On July 25, 2003, Khanty-Mansi Autonomous Okrug was renamed Khanty-Mansi Autonomous Okrug - Yugra.

Khanty-Mansi Autonomous Okrug - Yugra views

Rest on the lake in Yugra

Author: O.Frolov

Golden autumn in the Khanty-Mansy region

Author: Leonid Karpushin

Beautiful nature of Yugra

Khanty-Mansi Autonomous Okrug - Yugra - Features

The name of the region is associated with the self-names of the two main groups of northern peoples - Khanty and Mansi. In the Middle Ages, the word “Yugra” was used to refer to peoples and lands beyond the Northern Urals.

This region, located in the middle of Russia, occupies the central part of the West Siberian Plain. Its territory stretches from west to east for almost 1,400 km, from north to south for 900 km. The area of the region is comparable to France or Ukraine.

The climate is temperate continental characterized by rapid change of weather especially in spring and autumn. Winters are long, snowy and cold with frosts below minus 30 degrees Celsius. Summers are short and warm. From the west this region is protected by the Ural mountains, from the north it is open to cold arctic air.

The highest points of the region are Mount Narodnaya (1,895 m) in the Polar Urals and Mount Pedy (1,010 m) in the Northern Urals. Two major rivers flow in Khanty-Mansi autonomous okrug: the Ob (3,650 km) and its tributary the Irtysh (3,580 km). About 30% of the territory is covered by swamps. There are more than 300,000 lakes surrounded by marshes and forests.

The largest cities of Khanty-Mansi Autonomous Okrug - Yugra are Surgut (396,000), Nizhnevartovsk (280,800), Nefteyugansk (128,700), Khanty-Mansiysk (106,000), Kogalym (69,200), Nyagan (58,500). Today, only about 32,000 people are representatives of indigenous peoples: Khanty, Mansi and Nenets. Half of them live in the traditional way.

This region is very rich in oil and natural gas. The largest oil and natural gas fields are Samotlorskoye, Fedorovskoye, Mamontovskoye, Priobskoye. There are also deposits of gold, coal, iron ore, copper, zinc, lead and other mineral resources.

The climate is not favorable for agriculture. Most of the agricultural products and foodstuffs is brought from other Russian regions. Waterways and railways are the main shipping ways. The total length of the pipeline network is 107,000 km.

About 60% of Russian oil is produced in Khanty-Mansi Autonomous Okrug - Yugra. In total, more than 10 billion tons of oil were produced here. The total number of oil and natural gas fields discovered is 475. In the coming decades, the Khanty-Mansi region will remain the main resource base of hydrocarbons in Russia.

Tourism in Khanty-Mansi Autonomous Okrug - Yugra

Yugra has unique natural, cultural and historical resources for the development of recreation and tourism. On the territory of the region there are historical and cultural monuments, as well as modern infrastructure for lovers of cultural, educational, recreational tourism, and outdoor activities. International events (sports competitions, festivals and forums) help to open this place to foreigners as an amazing corner of the globe.

Khanty-Mansi Autonomous Okrug - Yugra has a number of wonderful natural sites worthy of attention: two nature reserves (“Malaya Sosva” and “Yugansky”), four nature parks (“Samarovsky Chugas”, “Siberian ridges”, “Numto”, “Kondinskie lakes”), ten monuments of nature, archeological complexes (“Barsova Mountain”, “Saygatino”, Sherkaly settlement).

Holidays of the northern peoples are also popular among tourists: Reindeer Herder’s Day, Day of indigenous Peoples of the North “Crow day”, Fisherman’s Day, Bear holiday and others.

Active and extreme types of tourism (skiing, snowboarding, kiting) are gaining in popularity. There are seven ski resorts in the region. In summer, travelers can go rafting on mountain rivers of Siberia. Tourists can also go on a special oil tour that includes a visit to the oil-producing companies. They learn about the oil industry and the history of oil exploration in Siberia.

Khanty-Mansi Autonomous Okrug - Yugra is a region of endless charm of the beautiful nature and modern tourist facilities. True lovers of northern landscapes and local cultures will be able to fully enjoy the incomparable scenery and generous hospitality in Ugra.

Khanty-Mansi okrug of Russia photos

Khanty-mansi autonomous okrug scenery.

Road through autumn forest in Khanty-Mansi Autonomous Okrug

Deep winter snow is not a problem in Khanty-Mansi Autonomous Okrug

Author: Chernenko

Yugra scenery

Author: Sergej Fedotov

Pictures of Khanty-Mansi Autonomous Okrug - Yugra

Winter in Khanty-Mansi Autonomous Okrug

Orthodox church in Khanty-Mansi Autonomous Okrug

Author: Alexey Borodko

Church in Khanty-Mansi Autonomous Okrug

Author: Peter Sobolev

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