DATE OF SERVICE: MM/DD/YYYY
HISTORY OF PRESENT ILLNESS: The patient was evaluated in the pediatric nephrology clinic for ongoing care of her history of multicystic dysplastic kidney, UTIs, and metabolic syndrome. This is a patient with Turner syndrome. Today, she is accompanied by her grandmother. The history was obtained from the patient and her grandmother.
The patient is a (XX)-year-old young lady with a diagnosis of Turner syndrome who was born with a right multicystic dysplastic kidney and a normal left kidney that has developed compensatory hypertrophy. She also has a history of urinary tract infections in the past but has had a negative VCUG.
In her adolescence, she developed obesity and metabolic syndrome with some insulin resistance and hyperlipidemia. She has also had some intermittent elevated high blood pressures. She has no history of structural cardiac disease. She has also had some urine microalbumin measurements that have been elevated, and we have been following her closely for early signs of hyperfiltration injury.
Today, she returns for her routine followup. She has been generally healthy since her last visit. She has had no significant intercurrent illnesses and has no complaints today. She still has not made any significant lifestyle modifications and has gained weight. She is not exercising and does not make the best food choices. She does complain of some back pain related to her scoliosis and is looking for a new orthopedic surgeon. She says she has some intermittent mild headaches that do not require any medications and self-resolves.
She is also having some irregular menstrual spotting due to starting new hormone replacement in the form of birth control pills. She otherwise is feeling well. She has had no chest pain, palpitations, lightheadedness, dizziness, dyspnea and no urinary symptoms.
In addition to what is mentioned above, all other systems reviewed were negative.
SOCIAL HISTORY: The patient lives at home with her grandmother. She does admit to being depressed intermittently because of her grandfather’s death and this makes her eat more.
MEDICATIONS: Oral contraceptive pill and vitamin D 2000 units daily.
PHYSICAL EXAMINATION: The patient’s height is 160 cm and weight is 94 kg, which is at the 98th percentile. Her blood pressure was initially 132/86; on repeat, 126/70. On examination, she is a well-appearing, obese, adolescent girl, in no distress. She does have short stature. She has no other obvious dysmorphic features. Oropharynx is clear. She has no tonsillar hypertrophy. Her TMs are clear. Her neck is supple. She has no cervical lymphadenopathy. Her chest is clear to auscultation bilaterally. Her heart has regular rate and rhythm. No murmurs, rubs or gallops. Her abdomen is soft, nontender, and nondistended. She has no hepatosplenomegaly. No joint swelling or tenderness. No pallor or rash. She has some striae on her abdomen and no peripheral edema. Her neuro exam is nonfocal.
LABORATORY DATA: The patient’s urine dipstick today has specific gravity greater than 1.030, pH of 5.6. There was large blood, 1+ protein and 1+ ketones; it was otherwise negative. A urine microalbumin to creatinine ratio was sent to the lab and is elevated at 68. In reviewing her previous ratios; in October, it was 60; in May, it was 110; and even back six years ago, it was slightly abnormal at 32.
Blood work was done today as well. Her serum creatinine concentration is normal at 0.64 mg/dL. Electrolyte panel including potassium of 4.2 and her serum glucose was 98, which was not fasting.
IMPRESSION: The patient is a (XX)-year-old young woman with Turner syndrome who was born with a right-sided multicystic dysplastic kidney with a normal left kidney that has demonstrated compensatory hypertrophy. She also has a history of UTIs, but none recently, and has had a negative VCUG in the past.
Currently, she is obese and has a metabolic syndrome with insulin resistance and hyperlipidemia. She has had some intermittent high blood pressures, and most concerning, she has now had sequential urine microalbumin to creatinine ratios that have been elevated in the microalbuminuria range, which is consistent with early hyperfiltration.
Given that the patient has a single kidney, she is at risk for this hyperfiltration area injury and chronic kidney disease, and certainly, her obesity and metabolic syndrome are significant risk factors for the progression of CKD.
RECOMMENDATIONS:
1. Given that the patient has had sustained microalbuminuria over the last year, we do think she meets indications for ACE inhibitor therapy, particularly given the fact that she has decreased nephron mass from her single kidney. We have prescribed lisinopril 5 mg daily; this will also help with lowering her blood pressure. We have gone over how this medication is dosed and potential side effects, including cough, lightheadedness, and dizziness. We did not go over the risks for pregnancy given that it is our understanding that she has had an oophorectomy.
2. We have again encouraged the patient to make therapeutic lifestyle modifications to optimize her weight. We emphasized the importance of weight loss and regular exercise in her cardiovascular and renal health. We warned her that her progression of chronic kidney disease will certainly be faster if she remains obese and she will be at greater risk of developing significant hypertension and requirement for more medication.
3. We have asked the patient to call us if she has any trouble taking her medication on a daily basis. She is scheduled to have lab work done in one to two weeks in preparation for her endocrinology visit; therefore, we have given her a lab slip to have a repeat renal function panel to monitor her creatinine and potassium while taking an ACE inhibitor. We asked her to give the phlebotomist this requisition when she goes for her endocrine labs.
4. We would like to evaluate the patient again in nephrology clinic in one month’s time to see how she is tolerating her medication and check her blood pressure and urinalysis.
