Day 8: Pediatric Nephrology

Patient 1: 20 yr kidney transplant pateint with aspergers /autism
Kidney transplant from father, function not great

• Creatanin 1.8, double creatanine means the kidney function is half of what it should be, even if they only have one kidney

Given long acting erythropoietin -methoxypolyethylene glycol epoetin beta- for his anemia because his kidney is not functioning and producing epo normally. Injected with IV and flushed with saline.
What things are regulated by the kidney that need to be monitored?

• Electrolytes, creatanine, urea, rbc, signs of infection, urine culture, protein, glucose ect
• BP is a problem with liver disease, but was ok.
• Triple immunosuppression; microfinulate, low dose prednisone, techrilamus (like cyclosporine but better)

Patient 2: Uretur reflux, UTI, hydronephrosis

Normally shows black on US with white fatty capsule surrounding, but with hydronephrosis, the black center is more shriveled and decreased in size as the edematous cortex is increased in size.

Patient 3: 15 year old boy with VATER association syndrome

VATER is an acronym or abbreviation representing the first letter of each feature in the association: Vertebral (spine) abnormalities, Anal atresia (partial absence of the anus or unusual connection between anus and rectum),Tracheo-Esophageal fistula (connection between the windpipe and the tube carrying food from mouth to stomach), and Radial (bone of the forearm) or Renal (kidney) differences.

• X-Ray of hand and elbow to see the growth, bone age and if the epiphysis has fused or not. In these kids the growth is stunted and delayed. His bone age was of a 12 yr old boy which means he still has the potential to grow. 
• US-child has horse shoe shaped kidney
• PE-scoliosis and scars from lumbar spinal surgery for a tethered spinal cord which gave him weakness and other neurological symptoms from the motor neuron like weakness or incontinence.  
• Blood exam-PTH & Vitamin D test should be packed with ice and sent to lab.
• Renal function is borderline

Patient 4: 7 year old boy with kidney transplant from mom 3 years ago, in hospital for pneumonia and has pleuritic pain. 

Patient was born with dysplastic kidneys-End Stage Renal Disease. Was able to get Palestine to pay for dialysis, but not for kidney transplant, so patient had a hook up in AMMAN, Jordan, so had a kidney transplant there. 

• X-ray- Pneumonia in upper part of lower lobe, we know this because we can see the border of the heart still.
• Antibiotics: Pressolat 10 (Nifedapine 10mg) 
• BP-138/75 high, 117/70, 95/40
• give Paracetamol for pleuritic pain (steroids have nephrotic effect)
• Creatinine 0.7-0.8- normal. 

(note: Doc told me prisoners are killed and china and organs used for transplants)

Patient 5: Boy born with posterior urethral valve-doing well now

• had a past inguinal hernial repair.
• creatinine 0.19 (good) 
• HG-10.9
• HCT-32.6 
• X-ray with dye-contur of bladder misformed
• prenatal US showed hydronephrosis due to post urethral obstruction
• VCUG- cystogram scintography-functional exam shows dialations below and above the second valve. 
• kidneys show a large parenchyma which is good end of the spectrum
• bladder is damaged due to backed up pressure, could cause filling and emptying defects, but in this case is normal. 

Patient 6: kidney transplant patient with pneumonia follow up 

•  external jugular vein was used to take blood due to bad veins. 

Patient 7: Minimal Change Nephrotic Syndrome- 3 yr old girl diagnosed 1 yr ago. Was given steroids and went into remission. Doctors assume it is Minimal Change NS because the child is too young for a renal biopsy and the fact that she responds to treatment. 

• blood test-protein negative
• BP-86/50 good

parent must check the urine daily with a dipstick to check for positive signs for remission. like swollen puffy eyes and face ext

treat with steroids-does not cause kidney damage. 

Patient 7: 6.5 yr old boy with autosomal recessive nephrotic syndrome that is not treatable/not responsive to medication due to the genetic defect in a glomerular protein. 

He will ultimately be left to face end stage renal disease where he will need dialysis or transplant. 

Medications mom brought in for boy:

• Ventoline (flixotide) inhaler for asthma (no relation to renal disease)
• furosamide (lasix)
• Vitamin D
• Eltoxime thyroid replacement- Has hyperthyroidism due to massive loss of protein. Thyroid binding protein binds thyroid hormone and is excreted and the body looses thyroid hormone. 
• calcium channel blocker-Norvasc-amylodipine  
• enaladux
• enoxaparin sodium-heparin-needed due to chronicity of kidney disease, highly nephrotic and at risk for thrombosis and clotting problems

PE

• look for edema on tibia-was normal
• belly has ascites 
• felt edge of liver at a lower level than normal, percussion showed that liver was close to normal if not just a bit enlarged. 
• could not feel spleen 

Treat

• give diuretics for edema
• vitamin D (Vit D low for nephrotic patients) this boy is at 7
• he will need a transplant around the age of 10-20 yrs old
• follow his weight-this will tell you if he is retaining fluid-last week he weight 25 kilo, now 19 kilo due to fluid retention. 

Patient 8: Pylonephritis and multiple abscess disease, hydronephrosis

treated with IV antibiotics and home IV of Tazocine (broad spectrum antibiotic) for a few weeks.