Adult

Case Study #633N

Initial Patient Presentation: A 35-year-old female presents to the clinic with reports of fever, chills, and left side of flank pain, which has steadily become more severe over the past 24 hours. A review of systems is unremarkable, aside from these acute symptoms.

Past Medical History:
G3P3
Hypertension (diagnosed 10 years ago)
Pyelonephritis x 3 within the past three years

Medications:
Fosinopril 40 mg po daily

Family History:
Her father died of an MI at the age of 58; he had just been diagnosed with diabetes mellitus and had “bad” kidneys.

Social History:
• Non-smoker
• No illicit drug use
• 1-2 glasses of wine per week
• Married x 15 years

Physical Exam:
Vital Signs: temp 1020F, BP 140/96, Pulse 96.
General: Appears anxious and younger than stated age. Intermittent bouts of shaking chills.
HEENT: Normocephalic. Atraumatic. Pupils are equal, round, and reactive to light. Mucous membranes of the oral cavity are pink and moist, no ulcerations, lesions, or evidence of infection.
Neck: Supple, no lymphadenopathy.
Chest: Lungs clear to auscultation and percussion.
Heart: Rapid regular rhythm, no murmur.
Abdomen: Pain on deep palpation more on left, no true guarding. Positive Murphy’s punch on left.
Pelvic: Unremarkable except for vague discomfort on deep bimanual palpation, no drainage from cervix.
Extremities: No edema. Pulses equal bilaterally.
Skin: Warm and dry.

Section One: Initial Information
Patient Case Question 1. Create a problem list based upon this patient’s initial presentation and exam findings (before obtaining diagnostics).

Problem list
 Inflammation/infection
 Gallbladder
 Liver
 Kidney Cancer
 Virous infection
 Renal artery disease
 Kidney stone
 UTI
 Kidney infection
 Hypertension
Diagnostics

Lab Data:
Complete Blood Count (CBC) Basic Metabolic Panel (BMP)
Hgb-10gm/dL BUN: 28 mg/dL
MCV- 65um3 Creatinine: 1.2 mg/dL
WBC- 16.5×103/ul Glucose: 112 mg/dL
Platelets: 210,000×103/ul Na: 138 mmol/dL
Differential: K: 4 mmol/dL
Neutrophils- 10×103/ul Cl: 103 mmol/dL
Bands- 3.0×103/ul CO2: 23 mmol/dL
Lymph-2.0x103ul Calculated GFR: 54.3 mL/min
Mono-0.8×103/ul

Urinalysis: Slightly red appearance, specific gravity 1.010, glucose negative, hemoglobin +, nitrate +
**Culture of the urine grew E. coli.

Urine Sediment examination: 4+ RBC, 4+ WBC, occasional WBC cast

Urine Pregnancy Test: Negative

Cervix Culture: Negative for gonorrhea and chlamydia.

Ultrasound Abdomen/Pelvis: Bilateral enlarged multicystic kidneys. Several cysts were also noted in the liver and the pancreas.

Section Two: Differential Diagnosis & Definitive Diagnosis
Patient Case Question 2. Create a differential diagnosis list that is supported by problem-based rationale.

Differential diagnosis list
Kidney disease-BUN (23) higher than normal and GFR (54.3) low than normal
Infection and inflammation -WBC and Temperature elevation
UTI – Nitrate + culture
ACE inhibiter (Fosinopril) serious side effect of ACE inhibitors causes Kidney failure/injury.

Patient Case Question 3: What is your final diagnosis?

Diagnosis

 Acute kidney injury
 History of pyelonephritis
 Positive Murphy punch test
 UA analysis shows positive nitrite and hematuria

Section Three: Explanation of Results
Patient Case Question 4. Why does this patient have a fever? Discuss the mechanisms involved in the development of fever.

Inflammation and infection, when any strange thing happens to the body like injury, viruses, bacteria or toxic chemicals will trigger your immune system. The immune system will try to attack the object and the reaction will cause rise in temperature, fever, swelling and pain.

Patient Case Question 5: What is a “Murphy’s punch”?
Murphy’s punch is a test performed to diagnosis Kidney disorder mimic muscle skeleton pain. It is also call fist percussion test. If pain is present upon test, it is an indication of kidney disease.

Patient Case Question 6: How would you make a clinical (not pathological) diagnosis of polycystic kidney disease?
The clinical diagnosis for polycystic kidney disease.
 High blood pressure
 Flank pain
 Hematuria
 Kidney failure
 Positive Murphy’s punch test

The patient was treated with IV antibiotics and recovered. Over the next several years she had numerous bouts of pyelonephritis and hematuria. Hemorrhaging became so severe that a nephrectomy was performed.

Surface of the Kidney

Cut Surface of the Kidney

Section Four: Pathophysiology of Disease Process and Symptoms
Patient Case Question 7: What is adult polycystic kidney disease (PKD)? Your response needs to include the following (at minimum): the genes involved, the frequency of disease occurrence, the gross pathological findings, and the tissues where cysts or other abnormalities can be found.
Adult polycystic kidney disease is genetic disorder in which kidney is filled with cysts larger than normal which fails over time
The genetic involved is autonomic dominate gene.
According epidemiologic study report a point prevalence of three to five cases per 10000 in the general population.

Other tissues where cysts or other abnormalities can be found includes, the liver, pancreas, spleen, or epididymis.

Polycystic kidneys have a striking appearance (Fig. 1). Typically, both kidneys
are massively enlarged as a consequence
of the growth of hundreds or thousands
of cysts scattered throughout the renal
cortex and medulla. The gross anatomical distortion caused by these cysts is
thought to be responsible for the progressive loss of kidney function associated
with this disease and the renal failure experienced by approximately 50% of affected
individuals.

kidney manifestations that can occur include hypertension, urinary tract infection, concentrating defect, hematuria, nephrolithiasis, and acute or chronic flank and abdominal pain; protein excretion is generally not a prominent feature but when present is associated with increased risk

Patient Case Question 8: Explain the possible mechanism of cyst formation in polycystic kidney disease? In your response, include information about the significance of polycystins.

Section Five: Disease Course, Management, & Outcome
Patient Case Question 9. How would you treat this patient? Your response should include more than a referral.

Patient with polycystic kidney disease treatment target symptoms. Symptoms can include hypertension, cholestasis, Renal failure, Portal hypertension.
Hypertension can be treated with Angiotensin converting enzyme inhibitors and Angiotensin receptors blockers.
Renal Failure: Treatment includes dialysis and transplant.
Portal Hypertension: Treatments include portocaval shunt-bypasses liver transplant

Patient Case Question 10: What are the common causes of death in patients with polycystic kidney disease? Why do approximately 10% of patients die of subarachnoid hemorrhage?
The common cause of death includes ruptured intracranial aneurysm, HTN, ischemic stroke ESRD, infection.

Patient Case Question 11: The patient is worried that her children may be affected. How would you explain the risk? Should her children be tested for the gene or the disease?