Tumor de células yuxtaglomerulares (reninoma) como causa de hipertensión arterial en la adolescencia. A propósito de un caso / Juxtaglomerular cell tumor (reninoma) as a cause of arterial hypertension in adolescents. A case report

La hipertensión arterial (HTA) grave en pediatría responde fundamentalmente a causas secundarias. Presentamos una paciente adolescente de 14 años con HTA grave, alcalosis metabólica e hipopotasemia, secundaria a un tumor de células yuxtaglomerulares productor de renina, diagnosticado luego de dos años de evolución de HTA.

Severe arterial hypertension (HTN) in pediatrics is mainly due to secondary causes. Here we describe the case of a 14-year-old female adolescent with severe HTN, metabolic alkalosis, and hypokalemia, secondary to a renin-secreting juxtaglomerular cell tumor diagnosed after 2 years of HTN progression.

Intraoperative management of potentially fatal arrhythmias after anesthesia induced by severe hypokalemia: A case report / 北京大学学报(医学版)

Severe hypokalemia is defined as the concentration of serum potassium lower than 2.5 mmol/L, which may lead to serious arrhythmias and cause mortality. We report an unusual case of potentially fatal ventricular arrhythmias induced by severe hypokalemia in a patient undergoing laparoscopic partial nephrectomy in Peking University Third Hospital due to irregular use of indapamide before operation. Indapamide is a sulfonamide diuretic with vasodilative and calcium antagonistic effects, which enhances sodium delivery to the renal distal tubules resulting in a dose-related increase in urinary potassium excretion and decreases serum potassium concentrations. The electrolyte disorder caused by the diuretic is more likely to occur in the elderly patients, especially those with malnutrition or long-term fasting. Hence, the serum potassium concentration of the patients under indapamide therapy, especially elderly patients, should be monitored carefully. Meanwhile, the potassium concentration measured by arterial blood gas analysis is different from that measured by venous blood or laboratory test. According to the previous research, the concentration of potassium in venous blood was slightly higher than that in arterial blood, and the difference value was 0.1-0.5 mmol/L. This error should be taken into account when rapid intravenous potassium supplementation or reduction of blood potassium level was carried out clinically. In the correction of severe hypokalemia, the standard approach often did not work well for treating severe hypokalemia. The tailored rapid potassium supplementation strategy shortened the time of hypokalemia and was a safe and better treatment option to remedy life-threatening arrhythmias caused by severe hypokalemia with a high success rate. Through the anesthesia management of this case, we conclude that for the elderly patients who take indapamide or other potassium excretion diuretics, the electrolyte concentration and the general volume state of the patients should be comprehensively measured and fully evaluated before operation. It may be necessary for us to reexamine the serum electrolyte concentration before anesthesia induction on the morning of surgery in patients with the history of hypokalemia. For severe hypokalemia detected after anesthesia, central venous cannulation access for individualized rapid potassium supplementation is an effective approach to reverse the life-threatening arrhythmias caused by severe hypokalemia and ensure the safety of the patients.

Caso Clínico: Parálisis Periódica Hipopotasémica / Case Report: Hypokalemic Periodic Paralysis

INTRODUCCIÓN: La parálisis periódica hipopotasémica es una enfermedad poco frecuente. Se caracteriza por episodios de debilidad muscular o plejia, reversible con la normalización de los niveles de potasio. Al ser una entidad poco común, el reporte del presente caso será de utilidad para el diagnóstico diferencial de la debilidad muscular aguda. CASO CLÍNICO: Paciente masculino de 22 años de edad, que posterior a ejercicio físico extenuante e ingesta moderada de hidratos de carbono y alcohol, presenta debilidad muscular aguda de miembros superiores e inferiores. EVOLUCIÓN: Al ingreso se realizó el diagnóstico de polirradiculoneuropatía desmielinizante aguda, administrándose una dosis de inmunoglobulina humana. Sin embargo, una vez obtenidos los resultados de laboratorio, se evidenció un potasio sérico de 2.4 mEq/L. Se inició la reposición con cloruro potásico en infusión. El paciente fue valorado por neurología y genética, con base en la anamnesis, examen físico, laboratorios y cuadro clínico del paciente, se realizó el diagnóstico de parálisis periódica hipopotasémica. Paciente presentó una evolución favorable, recibiendo el alta al cuarto día de hospitalización. CONCLUSIONES: La parálisis periódica hipopotasémica es una entidad poco frecuente, raramente incluida en el diagnóstico diferencial de la debilidad muscular aguda. La identificación oportuna y la consejería apropiada son esenciales para la prevención de complicaciones potencialmente mortales para el paciente.

BACKGROUND: Hypokalemic periodic paralysis is a rare disease, characterized by episodes of limb muscle weakness, reversible with the normalization of potassium levels. Being a rare entity, this report will be useful for the differential diagnosis of acute muscle weakness. CASE REPORT: A 22-year-old male patient, who after strenuous physical activity and a moderate intake of carbohydrates and alcohol, presented acute muscle weakness of the upper and lower limbs. EVOLUTION: On admission, the patient was diagnosed of acute demyelinating polyradiculoneuropathy, administering a dose of human immunoglobulin. However, once the laboratory results were available, a serum potassium of 2.4 mEq/L was evidenced. The replacement was started with potassium chloride in infusion. The patient was evaluated by neurology and genetics. Based on the anamnesis, physical examination, laboratories and clinical picture of the patient, the diagnosis of hypokalemic periodic paralysis was made. Patient presented a favorable evolution, receiving discharge on the fourth day of hospitalization. CONCLUSIONS: Hypokalemic periodic paralysis is an uncommon disease, rarely included in the differential diagnosis of acute muscle weakness. Timely identification and appropriate counseling are essential for the prevention of life-threatening complications.

Cambios electrocardiográficos asociados a hipopotasemia: alteraciones del segmento ST imitando isquemia miocárdica de múltiples territorios / Electrocardiographic changes associated with hypokalemia: ST-segment abnormalities mimicking ischemia of multiple territories

Resumen: Se presenta el caso de un paciente de sexo femenino de 42 años sin antecedentes clínicos de relevancia, con hipopotasemia severa y cambios electrocardiográficos imitando isquemia miocárdica con enfermedad corona-ria de múltiples vasos.

Abstracts: A 42-year-old woman with no previous medical history developed severe hypokalemia. The ECG changes suggested multivessel coronary artery disease with ischemia in several territories.

Adult presentation of Bartter syndrome type IV with erythrocytosis / Apresentação tardia de síndrome de Bartter tipo IV com eritrocitose

Abstract Bartter syndrome comprises a group of rare autosomal-recessive salt-losing disorders with distinct phenotypes, but one unifying pathophysiology consisting of severe reductions of sodium reabsorption caused by mutations in five genes expressed in the thick ascending limb of Henle, coupled with increased urinary excretion of potassium and hydrogen, which leads to hypokalemic alkalosis. Bartter syndrome type IV, caused by loss-of-function mutations in barttin, a subunit of chloride channel CLC-Kb expressed in the kidney and inner ear, usually occurs in the antenatal-neonatal period. We report an unusual case of late onset presentation of Bartter syndrome IV and mild phenotype in a 20 years-old man who had hypokalemia, deafness, secondary hyperparathyroidism and erythrocytosis.

Resumo A síndrome de Bartter compreende um grupo raro de doenças autossômicas recessivas perdedoras de sal, decorrentes de mutações em genes expressos na porção ascendente espessa da alça de Henle, com fenótipos distintos, porém fisiopatogenia única, que consiste em redução severa da reabsorção de sódio, e aumento da excreção urinária de hidrogênio e potássio, levando à alcalose hipocalêmica. A síndrome de Bartter tipo IV, causada por mutações com perda de função da bartina, uma subunidade do canal de cloro CLC-Kb expressa no rim e ouvido interno, geralmente se apresenta nos períodos ante e neonatal. No presente relato, descreve-se um caso não usual de síndrome de Bartter tipo IV com apresentação tardia e fenótipo atenuado, diagnosticado por análise molecular, em um homem adulto de 20 anos que se apresentava com hipocalemia, surdez, hiperparatireoidismo secundário e eritrocitose.

Rabdomiólisis por hipopotasemia severa: Report of six cases / Severe hypokalemic rhabdomyolysis

Rhabdomyolysis results from acute necrosis of skeletal muscle fibers and consequent leakage of muscle constituents into the circulation. It ranges from an asymptomatic state to a severe condition associated with extreme elevations in creatine kinase and acute renal failure. Reported etiologies of rhabdomyolysis include alcohol abuse, drugs, muscle trauma and muscle overexertion. Less common causes include muscle enzyme deficiencies, electrolyte abnormalities, infectious causes, toxins and endocrine disorders. Hypokalemia is a rare cause of rhabdomyolysis. We report six patients aged 31 to 57 years (three women) with a severe hypokalemic rhabdomyolysis, secondary to chronic diarrhea in two patients, treatment with loop diuretics in one and Gitelman syndrome in three. Rhabdomyolysis may be underdiagnosed in the context of hypokalemia, because the neuromuscular symptoms can be attributed solely to the electrolyte disorder.

Utilidad de la relación aldosterona y actividad renina plasmática en el diagnóstico de hiperaldosteronismo primario / Aldosterone/renin ratio in the diagnosis of primary aldosteronism

El hiperaldosteronismo primario (HP) es la forma más común de hipertensión arterial (HTA) secundaria. Recientemente varios estudios sugieren que la prevalencia de esta enfermedad varía entre 5% y 15% entre la población hipertensa, y 20% para hipertensión arterial refractaria. El objetivo de este estudio fue, ante la gran variabilidad de prevalencias sobre HP según las publicaciones internacionales y pocos datos en la Argentina, aportar la prevalencia de HP en un hospital general del interior del país, relacionándolo con el grado de HTA y presencia de normokalemia. En este estudio transversal se realizó dosaje de A/ARP en 123 pacientes hipertensos, suspendiendo toda medicación que interfiriera en los dosajes hormonales; se utilizó como método confirmatorio el test de solución salina (SS) para el diagnóstico de HP y la tomografía computarizada (TC) de abdomen para el diagnóstico etiológico del HP. Se detectó una relación A/ARP elevada en 20 (16.4%) pacientes. En 18 se realizó el test de SS, confirmando el diagnóstico de aldosteronismo en 8 (6.5% del total). En la TC, dos presentaron adenomas, y seis glándulas suprarrenales normales. Todos los pacientes con HP pertenecían al grupo II y III de HTA según el VI Joint National Committee (VI JNC) y el 50% fue normokalémico. Encontramos una prevalencia de 6.5% de HP, asociado a grado II y III de hipertensión, y valores de potasio normal en la mitad de los pacientes.

Primary aldosteronism (PA) is a possible cause of endocrine hypertension. Recent studies have suggested a prevalence ranging between 5% and 15% of all hypertensive patients, and 20% in patients with refractory hypertension.The objective of this transversal study was to establish the prevalence of PA in a hypertensive population using the aldosterone / plasma renin ratio (ARR) as a screening method, considering that the prevalence rates for PA among hypertensive people present a wide range and that there are only few reports in Argentina. This ratio was then related with the degree of hypertension and with the presence or absence of hypokalemia. Serum aldosterone and plasma renin activity levels were measured in 123 hypertensive patients after discontinuing all medications that could interfere with the hormonal tests. Patients with an aldosterone/plasma renin activity ratio > 25 were submitted to the saline suppression test (SST) to confirm the diagnosis of PA, followed by computed tomography (CT) of the abdomen. Twenty patients presented an ARR > 25 (16.4%). Eighteen were submitted to the SST, eight had a diagnosis of PA confirmed with positive SST (6.5%). Of 8 patients who underwent an abdominal CT, two showed adenoma, and six normal adrenal anatomy. All the eight patients with a PA diagnosis belonged to group II and III of hypertension according to Joint National Committee VI (JNC VI), and only 4 (50%) were normokalemic. We found a 6.5% prevalence of PA, associated with grade II and III hypertension, and normal potassium values in half of the patients with PA.

Distal renal tubular acidosis, hypokalemic paralysis, nephrocalcinosis, primary hypothyroidism, growth retardation, osteomalacia and osteoporosis leading to pathological fracture: a case report

Renal tubular acidosis [RTA] is a constellation of syndromes arising from different derangements of tubular acid transport. Recent advances in the biology of urinary acidification have allowed us to discern various molecular mechanisms responsible for these syndromes. RTA often presents as renal stone disease with nephrocalcinosis, ricket/osteomalacia and growth retardation in children with ultimate short stature in adulthood. The case reported here has features of distal renal tubular acidosis [dRTA], hypokalemic paralysis, primary hypothyroidism, growth retardation, osteomalacia and osteopenia leading to stress fracture. All these features presenting in a single case [as in our case] is a rare occurrence, so far other cases of distal renal tubular acidosis [dRTA] have been reported

Hiperaldosteronismo primário / Primary aldosteronism

O hiperaldosteronismo primário (HAP) é uma síndrome decorrente do aumento da secreção autônoma de aldosterona pela glândula adrenal, independente do controle da renina. O rastreamento do HAP está indicado em indivíduos com hipertensão arterial sistêmica (HAS) e hipocalemia espontânea ou grave com doses moderadas de diuréticos ou HAS refratária ao tratamento (%3 agentes anti-hipertensivos). No presente relato, paciente masculino apresentava diabete melito (DM) de início recente e HAS, emagrecimento, poliúria, polidipsia, e cansaço aos médios esforços. Apresentava hipocalemia grave e investigação laboratorial confirmou a suspeita de HAP, com medida de aldosterona sérica e urinária elevadas com diminuição da atividade da renina plasmática e aumento da razão aldosterona sérica/renina. Tomografia computadorizada de adrenais mostrou adenoma na adrenal esquerda. Após cirurgia, o paciente evoluiu com melhora dos níveis tensionais e normalização do metabolismo da glicose. Embora a prevalência de HAP em pacientes com DM não seja diferente da população de hipertensos não-diabéticos, a sua presença deve ser investigada nos casos de HAS refratária ou quando há hipocalemia. Em pacientes com DM, o metabolismo do cortisol também deve ser investigado para afastar a concomitância de hipercortisolismo decorrente de adenoma misto.

Primary aldosteronism (PA) is a syndrome that results from adrenal autonomous secretion of aldosterone. The screening for this syndrome is indicated for individuals with arterial hypertension (AH) and spontaneous or severe hypocalemia after diuretics, and refractory AH (%3 antihypertensive agents). In this report, a male patient presented recent-onset diabetes mellitus (DM) and AH, weight lost, poliuria, polidipsia, and tiredness. Severe hypocalemia was present, and the laboratory workup confirmed the hypothesis of PA, with increased plasmatic and urinary aldosterone levels, low plasma renin activity and increased aldosterone/renin ratio. Adrenal computerized tomography showed a left adrenal adenoma. After the surgical procedure, blood pressure levels and glycemia were brought to normal. Though the prevalence of PA is not increased in patients with DM, it should be screened in patients with refractory AH or persistent hypocalemia. In patients with DM, cortisol metabolism should also be evaluated to rule the presence of hypercortisolism in a mixed adenoma.

A case report of familial periodic paralysis.

A 20-year-old male was brought to the hospital with the complaints of severe weakness and inability to move the limbs of 12 hours duration. For the last 2 years he had the same episodes with spontaneous recovery. Family history strongly suggested involvement of other members of the family. Physical examination did not suggest any neurological deficit. All investigations were normal except serum potassium level being 2.2 meq/l during attack and 3.4 meq/l after the attack. He was treated with oral acetazolamide and potassium chloride. The case was diagnosed to be familial periodic paralysis belonged to the group 'episodic myasthenia'.

Primary Sjö-gren's syndrome presenting with distal, renal tubular acidosis and rhabdomyolysis.

Primary Sjögren's syndrome (PSS) is rare in India. Clinically manifest renal disease in PSS is uncommon and is usually an autoimmune tubulointerstitial nephritis presenting with distal renal tubular acidosis (dRTA) or a urinary concentrating defect. Hypokalemic paralysis due to dRTA in PSS is rare but well documented in medical literature. Rhabdomyolysis as a consequence of hypokalemia in PSS is exceptional. We report a case of PSS with dRTA and rhabdomyolysis causing prolonged respiratory failure and quadriparesis.

Diagnosis and management of metabolic alkalosis.

Elevated pH and elevated plasma bicarbonate level above normal characterise metabolic alkalosis. When bicarbonate is elevated pCO2 must also be elevated to maintain pH to its normal range. Therefore with metabolic alkalosis, the compensation is to decrease alveolar ventilation, and increase pCO2. The causes of metabolic alkalosis are gastro-intestinal hydrogen and chloride loss and due to renal cause. For metabolic alkalosis to continue both generation and maintenance of high levels of bicarbonate are necessary. The diagnosis of metabolic alkalosis is established by noting pH, serum bicarbonate (elevated) and pCO2 (compensatory) elevation. To establish the causes it is necessary to determine intravascular volume, supine and standing blood pressure and renin angiotension alolosterone axis. In chloride responsive alkalosis in which the conditions are extracellular volume depletion, hypokalaemia and hypochloraemia correction of intravascular volume with sodium chloride is needed. In severe metabolic alkalosis of any cause dilute hydrochloric acid (0.1 N HCl) may be infused intravenously but haemolysis may be a complication. In emergency situation with severe hypokalaemia dialysis with higher K+, Cl- and low HCO3- bath will be appropriate.

The prevalence of hypokalemia in hospitalized patients with infectious diseases problem at Cipto Mangunkusumo Hospital, Jakarta.

AIM: to know the prevalence of hypokalemia that occurs in hospitalized patients with infectious diseases. METHODS: a cross sectional study was carried out in the internal ward Cipto Mangunkusumo General Hospital in Jakarta from December 2005 until June 2006. All hospitalized patients with infectious diseases receiving "replacement solution" were included in this study. We collected the blood sample to perform the serum potassium level at the time of admission and discharge. RESULTS: one hundred and five patients were enrolled in this study; consisting of 44 males and 61 females. The age ranged from 14 to 70 years old. The most common infectious diseases were dengue fever, while the underlying diseases were hepatobiliary disorders. "Replacement solutions" which were given, were ringer's lactate 91%, normal saline 8%, and ringer's acetate 1%. Prevalence of hypokalemia among the hospitalized patients, on admission was 24 patients (23%) and during hospitalization was 39 patients (37%). The mean level of hypokalemia on admission was 3.11 + SD 0.37 mEq/L (range from 1.7 to 3.4 mEq/L) and during hospitalization was 3.13 + SD 0.25 mEq/L (range from 2.5 to 3.4 mEq/L). On admission, the ratio of mild : moderate : severe hypokalemia is 22 : 2 : 1. And at discharge the ratio of mild to moderate hypokalemia becomes 19 : 6. CONCLUSION: the prevalence of hypokalemia in hospitalized patients with infectious disease in Cipto Mangkunkusumo's Hospital, Jakarta is 23%. Further studies are needed to know the contributing factors including the usage of 'intravenous fluid' in relations to hypokalemic state during hospitalization.

Acute ascending hypokalaemic paralysis

A fifty-year-old male presented with weakness, difficulty in talking and breathing along with ascending paralysis. He gave prior history of excessive sweating in hot weather and had been administered many intravenous drips of dextrose solutions over the last three days. On clinical examination he was fully conscious, but was restless and tachycardiac. Motor power was 1/5, there was no obvious muscle wasting or fasciculation and the tendon reflexes along with abdominals and plantars were absent. Fundoscopy was unremarkable. Blood biochemistry revealed marked hypokalemia of 2.2 mmol along with an elevated blood and CSF sugar. Electrocardiography revealed flattened T waves and non-specific ST changes. His ventilation was supported and he was given I/V fluids with potassium supplementation and hyperglycaemia was managed with appropriate doses of insulin. The patient rapidly improved over the following few days with serum levels of potassium and ECG changes reverting to normal. The patient was discharged home in an ambulant state and he did not report back with any recurrence of symptoms