Management considerations in hypokalemic periodic paralysis include accurate Hypokalemic periodic paralysis is a disorder of muscle whereby .. Nutritive Value in aracer.mobi://aracer.mobi pdf. CLINICAL CHARACTERISTICS: Hypokalemic periodic paralysis (hypoPP) is a condition in which affected individuals experience paralytic episodes with. Hypokalemic periodic paralysis. Children with permanent myopathic weakness. Mark Dyken, M.D., Wolfgang Zeman, M.D., and Thomas Rusche, M.D.
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Hypokalemic periodic paralysis. (HPP) were diagnosed if there was spot/24 hour urine potassium excretion. hypokalemia and flaccid. What is Hypokalemic Periodic Paralysis? • Rare disorder of muscle. One of , individuals. • Channelopathy – disorder of ion channel. • Ion channels are. particular emphasis on thyrotoxic periodic paralysis. .. variation for thyrotoxic hypokalemic periodic paralysis. J Hum Genet.
Sporadic periodic paralysis Those with normal acid—base balance with no documented renal loss of potassium in the presence of normal thyroid function.
Thyrotoxic periodic paralysis Those with normal acid—base balance with no documented renal loss of potassium in the presence of thyrotoxicosis.
Hyperaldosteronism Patients with hypertension, hypokalemia, and metabolic alkalosis with high aldosterone and low renin levels. Liddle's syndrome Patients with hypertension, hypokalemia, and metabolic alkalosis with low aldosterone and renin levels.
Differences in categorical variables were compared using Fisher's exact test. Results Two hundred and six patients with a mean age of 37. Male: female ratio was 2.
Patients were grouped as periodic and non-periodic paralysis based on acid—base status as shown in [Table 1]. The mean potassium at presentation, rate of recurrence, incidence of respiratory paralysis, mean potassium requirement, and recovery time are tabulated in [Table 2].
Table 2: Differentiation of various subgroups of HKP Click here to view Hypokalemic non-periodic paralysis Patients in this cohort had acid—base disorder, and they were classified into two groups, namely, metabolic acidosis and metabolic alkalosis. Diuretic use was excluded by careful scrutiny. They were diagnosed to have Gitelman syndrome. Two among them had adrenal adenoma.
One patient who had hypertension with low plasma renin activity and aldosterone was diagnosed as Liddle's syndrome.
There was no significant difference in serum potassium level at the time of presentation between the periodic and non-periodic groups as shown in [Table 3]. However, there was a significant difference in urinary potassium loss, potassium requirement, and recovery time between the periodic and non-periodic forms of paralysis. There was a significant incidence of rebound hyperkalemia in patients with periodic paralysis.
Patients of Gitelman syndrome with concurrent hypomagnesemia also received magnesium supplements. Table 3: Clinical presentation and treatment response between periodic and non-periodic paralysis Discussion Hypokalemia and paralysis can be divided into two types, hypokalemic periodic paralysis where there is redistribution of potassium into cells and hypokalemic non-periodic paralysis resulting from a large deficit of potassium due to various etiologies.
The differential diagnosis in a patient with hypokalemic paralysis can be challenging due to the heterogeneity of its etiologies, but it is important to make the diagnosis promptly because different therapies are required for each type and identifying causes that are reversible is important.
The presence of a positive family history and recurrent episodes in a patient can be helpful in making a diagnosis of periodic paralysis, but periodic and non-periodic forms cannot be distinguished on clinical features alone. The most common cause of dRTA in our series was Sjogren's syndrome. Ram et al. This study validates the observation that hypokalemic paralysis in Sjogren's syndrome may precede classical clinical description and can serve as the clinical marker for diagnosis.
As Gitelman syndrome is characterized by high phenotypic variability and clinical manifestations may vary from nonspecific symptoms to severe disability, there has been significant underdiagnosis of Gitelman syndrome.
Genetic studies and long-term follow-up will be required to map the higher prevalence of Gitelman syndrome in our population. Among the various etiologies of hypokalemic paralysis in our series, Gitelman syndrome has less recurrence, implying good response to treatment. In the present study, we had seven patients 3. Two had adrenal adenoma and underwent adrenalectomy, while the rest were treated with spironolactone in maximum tolerated doses.
This cohort required more potassium replacement and needed more time for recovery when compared to the other cohorts. However, that series involved only 31 patients from a tertiary endocrine center. This cohort required less potassium replacement, less recovery time, and more chances of rebound hyperkalemia.
Treatment was started with intravenous potassium 100mEq during the first 24h. As a result, the patient had lost more than 20kg initial weight 143kg, BMI 46. Persistent constipation had occurred as a consequence, and had led the patient to use a stimulating laxative Cassia angustifolia.
In addition, 15 days before the onset of the clinical picture the patient had been advised to drink daily herbal teas of hawthorn HT [Crataegus monogyna] and olive leaves to improve HBP control.
No changes were initially found on examination in the ward. Normal thyroid function, basal cortisol, creatinine 0. Difficult to control HBP was also seen, and primary hyperaldosteronism was suspected.
Drugs interfering with diagnosis were discontinued, and doxazosin only was used. The clinical signs and symptoms resolved and his potassium level normalized four days after admission.
ECG normalized in 48h. A probable multifactorial, secondary hypokalemic paralysis was suspected due to the use of diuretics, laxatives, and HT tea. The patient was discharged home with no symptoms, with doxazosin prescribed as the only antihypertensive, in order to rule out primary hyperaldosteronism. Primary hyperaldosteronism was therefore ruled out, and the suspected diagnosis was confirmed.
HP is an uncommon condition characterized by acute flaccid paralysis associated with hypokalemia. The causes of HP may be primary or secondary.
Primary HP, 1 such as familial hypokalemic periodic paralysis FHP , is autosomal dominant and occurs most often in Caucasians, 2,3 usually before 25 years of age. A family history is usually found. According to the Burcet 3 series, this is the most common cause of HP. Secondary forms are more prevalent in elderly Asian patients with lower potassium levels and more marked clinical signs and symptoms.
Other causes reported include thyrotoxicosis, renal tubular acidosis RTA , primary hyperaldosteronism Conn's syndrome , Gitelman's syndrome, and viral infections such as dengue. Less common causes include Cushing's syndrome, Liddle's syndrome, massive liquorice intake, or some forms of congenital adrenal hyperplasia. In the Ravindra et al. This is a rare complication of hyperthyroidism which occurs more commonly in Asians 2,4 and is attributed to a dysfunction of the transmembrane Na—K—ATPase pump.
In other series, the most common etiology was RTA and primary hyperaldosteronism. Genetic predisposition could possibly play a role. If the condition is severe, rhabdomyolysis may eventually occur.