A 38y/o woman with periodic weakness & vomiting Dr.Etminan Dr.Saeedi
Case Peresentation CC:periodic weakness & vometing PI:She presented with complaining periodic weakness & vometing of from 6months before. Approximately 6month ago she complains N/V without headache,vertigo,dysphagia,chest pain,abdominaln pain & dysuria .but she complains of anorexia ,flunk pain,constipation ,bloating,no related with feeding . She has 4kg Wt loss in duration 6month ago. Her weakness was related with vomiting,periodic & in proximal muscle.weakness no progress at afternoon
PMH: Due to new symptoms she had history of several admission ,surgical consult&endoscopy but she had not any improvement & referred to this center,,, In past history she had renal colic & nephrolithasis & ESWL she has not history of other problem or surgical in past medical
Drug history • PPI • Anti emetic
Familial history • There is not history of disease or cancer in her family
Social & habitual history • No smoking or using opium • She distributed her social communication because this problem
R .O.S: • NO headache,vertigo or hearing loss • NO hair loss,ulcer in mouth or photo sen • NO dry mouth or eye • NO dysphagia or odinoohagia • NO chest pain or dyspnea • NO abd pain but constipation & bloating &flunk pain • NO problem in mens or dysuria but frequency &nacturia • NO sweeling of joint or stiffness
She complains muscle pain but no bone pain& unable to perform specific tasks, such as climbing stairs or combing hair. • NO complain of changing color hands in cold waret or skin problems
Physical Exam She was ill but not toxic & not in acute distress T:37.1 Ax at night BP: 110/80, PR: 89, RR :21 O2 sat: 95% pale no ichteric no tenderness on PNs No lymphadenopathy Temporal wasting No Hoarseness No up jvp &no thyromegaly
Chest examination • Revealed equal bilateral breath sounds, • with no wheezes & bilateral clear • She had a normal S1&S2 sound and no murmurs.
Abdomen • was soft and nontender, with no masses or hepatosplenomegaly. • no CVA tenderness Ext • no peripheral edema • and no rash, telangiectasia, or other skin changes • no clubbing. • joints revealed no warmth, swelling, erythematic, tender prox muscle
Nourologic exam: • Cranial & prieferal ner NL --- No ptosis,gag nl • DTR +2 • Muscle force: upper(prox4/5 dist5/5) • lower(prox3/5 dis5/5)
Problem list:A 38y/o woman with periodic weakness & vomiting about 6 month before that vometing no related with feeding but related with weakness constipation bloating. • Weakness is in prox muscle,tender.
WBC:7300 (PMN;70) • Hb:10.8 • MCV:84 • MCH:26 • MCHC:31 • PIT:420000
Lab Data BS:105 AsT:66 AlT:11 Na:135 Bill(t):0.4 K:2.7 – 2.4 Bill(d):0.2 alkp:135 Ca:8.1 P: 2 .3 -- 1.8 Alb:3.7 Urea:44 Cr:1.1
ESR:33 CRP:+1 • CPK:278 LDH:471 TSH:3.5 BHCG:- • U/A SG:1018 PH:8 BLOOD:- RBC:- WBC:3-5 Bact:- • CAST:- CRYSTAL:OX-Ca few
PH:7.25 serum CL:117 AG:8 • PCO2:26 • BE:-13 • HCO3:11
ferritin:141 Iron:58 TIBC:235 ANA,Anti ds DNA:- RF:- Lipase,amylase: nl
ENDOSCOPY:NL • Abd CT: nephrocalcinosis both kidny • Abd sono:diffuse nephrocalcinosis in both kidney • (Nephrocalcinosis is characterized by the deposition of both calcium oxalate and calcium phosphate paranchyma and tubules).
Problem list:A 38y/o woman with periodic weakness & vomiting 6 month before, that no related with feeding but related with weakness constipation bloating. • Weakness is in prox muscle,tender. • K:2/4 metabolic acidosis AG:nl urine PH:8 ca:8/1 p:1/8 nephrocalcinosis CXR
Urine 24h: 1700cc • P:100 mg (300 -900) • Ca:280mg(50-300) • Pr:100mg(25-100) • K:80mmol(25-150)
PTH:19.3 pg/ml(9-90) • Cortizol 8am:86(55-287) • 25(Oh)vitD:37ng/ml(30-50) • Mg:2.1(1.6 -2.5) • AFP,CA125:NL
Severe muscle weakness or rhabdomyolysis . • The pattern of weakness in hypokalemia is similar to that associated with hyperkalemia. Weakness usually begins in the lower extremities, progresses to the trunk and upper extremities, and can worsen to the point of paralysis. • In addition to causing muscle weakness, severe potassium depletion (serum potassium less than 2.5 meq/L) can lead to muscle cramps, rhabdomyolysis, and myoglobinuria . Potassium release from muscle cells during exercise normally mediates vasodilation and an appropriate increase in muscle blood flow . Decreased potassium release due to profound hypokalemia can diminish blood flow to muscles during exertion, leading to ischemic rhabdomyolysis . A potential diagnostic problem is that the release of potassium from the cells with rhabdomyolysis can mask the severity of the underlying hypokalemia or even lead to normal or high values.
Respiratory muscle weakness • Involvement of gastrointestinal muscles, resulting in ileus and its associated symptoms of distension, anorexia, nausea, and vomiting.
Cardiac arrhythmias and ECG abnormalities— A variety of arrhythmias may be seen in patients with hypokalemia. These include premature atrial and ventricular beats, sinus bradycardia, paroxysmal atrial or junctional tachycardia, atrioventricular block, and ventricular tachycardia or fibrillation . Hypokalemia produces characteristic changes on the ECG although they are not seen in all patients. There is depression of the ST segment, decrease in the amplitude of the T wave, and an increase in the amplitude of U waves which occur at the end of the T wave . U waves are often seen in the lateral precordial leads V4 to V6.
Renal abnormalities — Prolonged hypokalemia can cause multiple structural and functional changes in the kidney . These include: • Impaired concentrating ability • Increased ammonia production • Increased bicarbonate reabsorption • Altered sodium reabsorption • Hypokalemic nephropathy • Elevation in blood pressure • Glucose intolerance — Hypokalemia reduces insulin secretion, which may play an important role in thiazide-associated diabetes. However, worsening glucose tolerance is much less common in the era of low-dose thiazide therapy (eg, 12.5 to 25 mg of hydrochlorothiazide)
Nephrocalcinosis • is associated with conditions that cause hypercalcemia, hyperphosphatemia, and the increased excretion of calcium, phosphate, and/or oxalate in the urine. Hypocitraturia • Hypercalciuria with hypercalcemia • Hypercalciuria without hypercalcemia • Hyperphosphaturia
Hypercalcemia and hypercalciuria : • Primary hyperparathyroidism • Sarcoidosis • Vitamin D therapy • Milk alkali syndrome • Williams' syndrome • Congenital hypothyroidism
Hypercalciuria without hypercalcemia : • Distal (type 1) renal tubular acidosis — Distal (type 1) renal tubular acidosis (RTA) is the most common cause of nephrocalcinosis (particularly in children) due to hypercalciuria without hypercalcemia . Distal RTA results in a systemic acidosis that requires increased buffering of acid by bone, with the subsequent release of bone calcium and phosphate. Metabolic acidosis is also associated with hypocitraturia, which can promote calcium precipitation in the tubules. • The reported prevalence of nephrocalcinosis in patients with distal RTA ranges from 60 to 80 percent . • Medullary sponge kidney
Loop diuretics • Bartter syndrome • Chronic hypokalemia — Hypercalciuria and nephrocalcinosis have been observed in chronic hypokalemic states including primary aldosteronism and Liddle's syndrome . Nephrocalcinosis and chronic hypokalemia are also seen in distal (type 1) renal tubular acidosis and Bartter syndrome but, as noted above, hypercalciuria is thought to be primarily responsible. Support for this hypothesis comes from the observation that chronic hypokalemia in Gitelman syndrome, which involves a mutation in the thiazide-sensitive sodium-chloride cotransporter in the distal tubule, is associated with hypocalciuria and the absence of nephrocalcinosis .
Hyperphosphaturia— Hyperphosphaturia with or without hypercalciuria is a risk factor for nephrocalcinosis. Hyperphosphaturia may occur with or without hyperphosphatemia. Hyperphosphaturia and hyperphosphatemia plus acute renal failure are observed in tumor lysis syndrome and after ingestion of oral sodium phosphate bowel preparations. These acute disorders are typically characterized by microscopic nephrocalcinosis, and are discussed elsewhere. • Hyperphosphaturia in the absence of hyperphosphatemia (ie, phosphate wasting), usually results from inherited tubulopathies, although acquired forms may be observed in the setting of malignancy or renal transplant.
Hyperoxaluria — • Hyperoxaluria may also be secondary to the increased enteric absorption of oxalate . Fat malabsorption is the most common cause of increased oxalate absorption . • Secondary hyperoxaluria may also be due to the chronic ingestion of excessive amounts of oxalate precursors, such as vitamin C, or of foods rich in oxalic acid such as rhubarb, parsley, cocoa, nuts, or star fruit