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R3 case conference. 報告者 : 楊仁星 指導老師 : 方基存醫師 報告日期 :2011-11-2. Index. Case presentation Discussion Back to the case. Case Presentation. Patient ’ s Profiles. Name: 陳 x 羽 Gender: female Age: 58 years Ethnic: Taiwanese Marriage: married Occupation: Waitress, School janitor, 有線電視服務員
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R3 case conference 報告者:楊仁星 指導老師:方基存醫師 報告日期:2011-11-2
Index • Case presentation • Discussion • Back to the case
Patient’s Profiles • Name: 陳x羽 • Gender: female • Age: 58 years • Ethnic: Taiwanese • Marriage: married • Occupation: Waitress, School janitor, 有線電視服務員 • Chart number: 39037xxx • Date of admission: 2011-9-26
Chief complaint • General weakness for 4 days
Present Illness-I • The patient had weakness of bilateral lower limbs for several days • 4 days before admission, the weakness was progressed to general weakness. • She received injection of unknown substance (may be analgestic) at LMD.
Present Illness-II • After injection, the weakness became worsen and then the patient couldn’t walk and stand. • The associated symptoms included palpitation. • She came to our ER for help
Present Illness-III • She denied fever, chest pain, chest tightness, cold sweating, dyspnea, dizzness, nausea, vomiting, diarrhea, anorexia, tarry or bloody stool, frequency, urgency and flank pain.
Past History: • Diabetes mellitus for half year without control • Denied hypertension, heart disease, HBV, HCV and operation history
Personal history: • Allergy: no known allergy • Alcohol: denied • Smoking: denid • Betelnut: denied • Medication history: Chinese Medicine (大豆類黃酮,保護關節藥)
Physical Examination-I • Vital sign: BT: 36℃, PR:62/min, RR: 18/min, BP: 121/80 mmHg • General appearance: fair looking • Consciousness: alert, E4V5M6 • HEENT: sclera: not icteric, conjunctiva: not pale • Neck: supple, JVE (-/-), LAP (-/-) • Chest: bilateral symmetric expansion bilateral breathing sound: clear • Heart: regular heart beat without murmur
Physical Examination-II • Abdomen: flat no superficial vein engorgement no spider angioma normoactive bowel sound no tenderness, no rebound pain no Murphy’s sign liver and spleen not palpable no shifting dullness • Back: no knocking pain over bilateral flank pain • Extremities: no pitting edema at bilateral lower limbs • Skin: no petechiae or ecchymoses no skin rash
Impression • Hypokalemia periodic paralysis • Diabetes mellitus
Clinical course-II • Other blood tests during hospitalization
Clinical course-III • Urine anion gap: 56+14.1-62 = 7.9
Clinical course-Vkidney echo on 9/30 • Left Kidney Length: 10.7 cm • Right Kidney Length: 11.8 cm • Impression: • Right renal stone (0.7cm) • Bilateral renal calcification spots • Parenchymal renal disease
Final diagnosis • Type I renal tubular acidosis, suspect autoimmune disease related • Renal stones • Type II diabetes mellitus, under diet control
Renal tubular acidosis • A systemic hyperchloremic and normal anion gap acidosis with relatively normal glomerular filtration rate • Results from either the net retention of hydrogen chloride or net loss of sodium bicarbonate • Three major subgroups of RTA • Distal or type 1 RTA • Proximal or type 2 RTA • Hypoaldosteronism or type 4 RTA
Renal tubular acidosis-distal RTA (type 1) • Failure of distal nephron and collecting duct to secret hydrogen ion • Failure to reabsorb filtered bicarbonate that was not reabsorbed in proximal tubule • Failure of titration of phosphate buffer and decreased exc • retion of NH4+ • Precise nature of defect is not known.
dRTA (Type 1)- Clinical features • muscle weakness • Hyperventilation • Acute acidosis • Hypokalemia • Inability to acidify the urine to a pH of less than 5.3 • 70% have either nephrocalcinosis (very rarely a feature of other types) or calcium containing renal calculi • Rickets and growth stunting are frequent features in childhood cases. ± osteomalacia in adults
Renal tubular acidosis-proximal RTA (type 2) • Proximal tubule reabsorption of approximaly 80~90% • Type 2 RTA: • an impariment of HCO3- reabsorption in the proximal tubule • Decreased renal HCO3- threshold • Distal acidifcation mechanisms are intact • May lower urine pH below 5.5
pRTA ( Type 2): Clinical feature • General weakness • Metabolic acidosis • Hypokalemia • proximal myopathy, osteomalacia or rickets • Normal urinary acidification • Nephrocalcinosis and renal calculi are virtually never present
Renal tubular acidosis-type 4 • Type 4 RTA is not actually a tubular disorder • reduction in proximal tubular ammonium excretion, which is secondary to hypoaldosteronism or pseudohypoaldosteronism, and results in a decrease in urine buffering capacity. • Presentation: hyperkalemia and normal urinary acidification • Nephrocalcinosis and Urolithiasis are absent in type 4
Sjögren's syndrome • A chronic autoimmune disease, affecting mainly the exocrine glands. • Prevalence: 0.3%~0.6% • Female:male: 9:1 • Can affect extraglandular, such as involvement of the musculoskeletal, pulmonary, GI, hepatobiliary, hematologic, vascular, dermatologic, renal and nervous systems.
Sjögren's syndrome-Classification • Primary SS: occur alone • Secondary SS: association with another defined autoimmune disease (eg, SLE, RA, or scleroderma)
Sjögren's syndrome-Symptoms • Two main symptoms: dry eye and dry mouth • Other: • Joint pain, swelling and stiffness • Swollen salivary gland • Skin rashes or dry skin • Vaginal dryness • Persistent dry cough • Prolonged fatigue
Diagnosis Criteria • 2002 American-European consensus Classification Criteria, required four of the following
Sjogren’s syndrome is an autoimmune disorder and the target organs are the exocrine glands, especialy the lacrimal and salivary • Kidney involvement is a frequent extraglandular manifestation of primary Sjogren’s syndrome • 30~40% of Sjogren’s syndrome patients have symptomatic and asymptomatic renal involvment. • The understanding of the clinical presentation of renal involvement in primary Sjogren’s syndrome is based on case reports and small retrospective cohorts.
Clinical and laboratory features • Patient with Sjogren’s syndrome and RTA tend to present hypokalemic periodic paralysis (HPP)