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Case presentation
Speaker:
Supervisor:
C1 李玟軒
R2 張喬翔
Vs 許育瑞
General Data
• Name: 汪XX
• Male
• 21 y/o
• ID : 25XXXX8
2
Chief complaint
• Soreness over the bilateral lower back for one week and nausea with fever up to 38.5 for three days.
3
4
Present illness: The patient with history of brown stone passage since he was a 6-
month-old boy. He was admitted to 林口長庚 hospital for therapy.
But no significant metabolism disorder or other disease was noted. 20000613: Ca 9.7 mg/dL K 4.7 mmol/L P 4.4 mg/dL Mg 2.0 mg/dL
20000623: Ca 10.4 mg/dL K 3.8 mmol/L P 4.4 mg/dL Mg 2.0 mg/dL
1998
0.3 0.6 0.6 0.6
13 13
890613 921124 930323 931101
Creatinine BUN (mg/dL)
5
Present illness: he visited the 中山hospital for further management of "renal stone".
He claimed he had normal thyroid and parathyroid function and the
stone analysis reported calcium oxalate.
• 20160720: Ca 9.7(mg/dL) Uca(24hrs 900ml) 18(mg/day)
P 3.9(mg/dL) Up(24hrs 900ml) 540(mg/day)
Cre 1.7(mg/dL) Ucre(24hrs 900ml) 100.8(mg/day)
Free T4 1.45(ng/dL) TSH 0.66(uIU/ml)
2005
1.2 1.2 1.4 1.4 1.6 2.3 1.8 1.7
1040212 1040528 1040716 1050225 1050816 1060107 1060208 1060720
Creatinine
Creatinine
51.2 25.8 16.9
940326 1050217 1060720
Intact PTH
Intact PTH
(mg/dL)
(pg/mL)
6
Present illness:
He was referred to another urologist at 郵政醫院 and
urethroscopy was done about twice per year.
2018
1.62 1.88 1.73 1.86 2.34 1.99 2.28
20.51
15
20
58.6
49.4
53.8
49.5
38
45.8
38.7
3.1
44.6
1070124 1070309 1070716 1070805 1070905 1080130 1080623 1081218
Creatinine BUN eGFR Intact PTH(mg/dL) (mg/dL) (mg/dL) (pg/mL)
7
Present illness:
His girlfriend found him nausea and vomiting with
normal appetite. But he denied progressive decreased
urine output.
20191204
He felt soreness over the bilateral lower back and he arranged the
GU OPD follow-up at 郵政醫院.
He also experienced dyspnea on exertion in recent half month and
condition became much worse before OPD visiting.
Additionally, he took the temperature and fever was noticed once
up to 38.5'C.
20191211
On visiting the GU OPD on 12/18, acute kidney injury with
creatininie up to 20 mg/dl and anemia (Hb dropped from 13 to 7.9
mg/dl) was noted. He was referred to our ED for help.
20191218
Past History
• Systemic disease: • Bilateral nephrocalcinosis post extracorporeal shock
wave lithotripsy since 6-month-old
• Hypertension for 1 year
• Operation history: • extracorporeal shock wave lithotripsy (now twice per
year) since 6-month-old
• No known allergies
• Medication: • denied recent NSAID use
Personal History
• Habit of cigarette smoking: denied
• Habit of alcoholic drinking: social drinking
• Social relationship: Well
• Psychologic status: normal
• Occupation: student
• Contact history: denied
• Cluster history: denied
• History of recent traveling: denied
• Full-term birth, normal weight
Review of Systems
• Positive findings:
Nausea(+),
Vomiting(+),
Fever(+),
Dyspnea on exertion(+),
Soreness over the bilateral lower back(+)
Conscious: alert
GCS: E4V5M6
Extremities:
No petechiae, no
ecchymosis, ROM
normal
Lung:
Regular breathing
pattern, no
wheezing, no
crackle, no rhonchi
Heart:
Regular heartbeat,
no murmur was noted
Abdomen: Impalpable liver and
spleen, no tenderness, no
rebounding pain,
normal bowel sound,
Vital sign:
BT: 36.7 ℃
HR: 81 b.p.m
RR: 20 /min
BP: 153 / 86 mmHg
Physical examinations
HEENT:
pale conjunctiva
2019.12.18
2019.12.18
20191218-CHEST, P-A VIEW
20191218-ABDOMEN C.T.
20191218-ABDOMEN C.T.
5.21:1
54
192
21.19%
1.99
The Lancet 2005 365, 417-430
DOI: (10.1016/S0140-6736(05)17831-3)
Tentative diagnosis
*. Urinary tract infection with sepsis
syndrome complicated with acute on
chronic kidney disease and uremia and high
anion gap metabolic acidosis
*. Bilateral nephrocalcinosis post annual
extracorporeal shock wave lithotripsy
*. Hypertensive cardiovascular disorder
Hospital course
22
12/18 22:16 Admitted to ED.
Lactated Ringer's B 500 ml as a source of water and
electrolytes and as an alkalinizing agent.
Admitted to NEP ward for for further management
12/19 Lactated Ringer's B 1000 ml as a source of water and
electrolytes and as an alkalinizing agent.
Rolikan 2.8 g in H/S 500 ml to prevent hyperchloremic
acidosis.
13:38
12/19 Consult INF for evulation of antibiotic for his UTI.
INF: Sintrix
H/S 1000ml(each bottle+Rolikan 2.8g total 5.6g)+
Sintrix 2000mg
Consult CVS for PERM catheter placement.
12/20
12/21 H/S 1000ml(each bottle+Rolikan 2.8g total 5.6g)+
Sintrix 2000mg
Hydralazine+Doxazosin for hypertension control.
On R'T PERM cath
Start hemodialysis
12/22 H/S 1000ml(each bottle+Rolikan 2.8g total 5.6g)+
Sintrix 2000mg
Hemodialysis
PRBC for relief of anemia.
12/24
12/25 H/S 1000ml(each bottle+Rolikan 2.8g total 5.6g)
Hemodialysis
Due to suspected systemic oxalosis, we consulted
OPH for exclusion of oxalate deposition. →excluded
12/26 H/S 1000ml(each bottle+Rolikan 2.8g total 5.6g)
Start regular hemodialysis (W2.4.6)
Due to suspected renal tubular acidosis, we
consulted ENT for exclusion of abnormal hearing.
→excluded
12/28
12/31
Regular hemodialysis (W2.4.6)
01/01
Hold regular hemodialysis (W2.4.6)
Nausea and vomiting
01/02 Regular hemodialysis
Consult RAD for renal biopsy.
Symptomatic treatment include: • Acetaminophen for fever and pain relief.
• Nexium for gastroesophageal reflux disease.
• Talex for relief of vomiting.
• Sevikar, Hydralazine, Doxazosin for hypertension control.
• Carvedilol for reduced mortality in patients with
congestive heart failure.
01/12
2019.12.19
2019.12.19
2019.12.19
2019.12.23
Na
K
Free Calcium
Magnesium
2019.12.19
2019.12.19
2019.12.23
2019.12.25
2019.12.26
Before HD
The Lancet 2005 365, 417-430
DOI: (10.1016/S0140-6736(05)17831-3)
Summary
• Full-term birth, normal weight
• Bilateral nephrocalcinosis post extracorporeal shock
wave lithotripsy since 6-month-old
• 2 years old: serum P 4.4(mg/dL) (Hypophosphatemia)
• 21 years old: CKD, HTN
Nephrocalcinosis
Hypercalcemia and
hypercalciuria Hyperphosphaturia
Hypercalciuria without
hypercalcemia Hyperoxaluria
Urinary excretion of oxalate
Genetic testing
Liver biopsy
Hyperphosphaturia
hyperphosphatemia tumor lysis syndrome
oral sodium phosphate
bowel preparations
kidney transplant
Inherited tubulopathies
Dent's disease
Low-molecular-weight proteinuria
At least one of the
following
Nephrocalcinosis
Nephrolithiasis
Hematuria
Hypophosphatemia
Chronic kidney disease
Lowe's syndrome
Dent's disease
Plus
renal tubular acidosis
congenital cataracts
mental retardation
X-linked hypophosphatemic
rickets
hyperphosphaturia but not
hypercalciuria
autosomal dominant and
autosomal recessive
hypophosphatemic rickets
hyperphosphaturia but not
hypercalciuria
Hypercalciuria without
hypercalcemia
Distal renal tubular acidosis
Metabolic acidosis
subsequent release of bone
calcium and phosphate
hypocitraturia
Medullary sponge kidney
Hypocitraturia
observed radiographically
Neonatal nephrocalcinosis
birth weight below 1500 g
prolonged administration of a
loop diuretic
loop diuretics Inherited
tubulopathies
Gene test
Chronic hypokalemia
primary aldosteronism
Liddle's syndrome
Beta thalassemia
Inherited tubulopathies
Bartter syndrome
Hypokalemia
metabolic alkalosis
Autosomal dominant
hypocalcemia
Normal or low serum calcium
Normal serum PTH
Recurrent nephrolithiasis
and nephrocalcinosis
Genetic testing
Hypomagnesemic hypercalciuric
nephrocalcinosis
Dent disease Lowe
syndrome
Differential diagnosis
• Hyperoxaluria(Primary hyperoxaluria) • Urinary excretion of oxalate
• Genetic testing
• Liver biopsy
• Dent's disease • Urine test
• Low-molecular-weight proteinuria
• Genetic testing
• Autosomal dominant hypocalcemia • Genetic testing
Thanks for attention