Congenital Anomalies of the Kidney
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Transcript of Congenital Anomalies of the Kidney
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Morning Report
Friday March 22nd 2013
Dana Boucek MD, PGY-2
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Case Presentation
10 day old term infant female
Chief complaint of poor feeding.
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HPI
Feeding well breast and bottle in the hospital
then mostly bottle feeding at home.
Presented with poor feeding X 1 week (since
home). Initially taking 1.5 ounces then
decreased to 1 oz every 3ish hours.
Taken to PCP, still gaining weight ok. Parents
told to feed with syringe when things looked
like they were worsening in terms of intake
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HPI
Parents then noted complete refusal of feedsX about 1 day prior to presentation
Took her to PCP again who sent them to ED in
Idaho Falls. Sent to PCMC for further evaluation of poor
feeding/ weight loss.
Noted to be febrile on arrival to PCMC at 38.2and on further questioning parents reportfever at outside hospital ED
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HPI
Infant had also had what parents describe assome loose stools
They estimate 1-2 wet diapers over the last
day but difficult due to the loose stools. Mother also describes weird breathing X 1-2
days where she would breathe fast then pause
for a little while Had been up to 7 lb 10 oz (3.47 kg) at PCP 2
days PTA
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PMHx
37 weeker born by repeat C section with BW
7lb 6 oz. No complications in hospital. Home
after 4 days.
Maternal labs all normal by report but records
not available.
Got a frenulectomy in the hospital
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Family History
Some asthma on the fathers side
No other significant medical history on either
side
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Social History
Lives in with mother, father, 2 older full
siblings and 1 half sister
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Meds and Allergies and Imms
None
And None
Up to date (got Hep B per parental report)
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Physical Exam
Vitals: Weight 3.3 kg; T 38.2; P 142; R 46; BP 80/41; O299% on RA
Gen: Awake, alert somewhat pale appearing infant inno acute distress in parents lap
HEENT: NCAT, fontanelle soft and mildly depressed, RR+ bilat, conjunctiva clear, OP clear, ear canals patentand external ears normal appearing
Neck: supple with full ROM
RESP: CTAB with no retractions, wheezes, crackles CV: Grade 2 systolic murmur at the LUSB that radiates
into the axillae. Femoral, brachial distal pulses 2+, caprefill < 3 seconds.
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Exam contd
Abd: soft, somewhat full, apparently TTP on the right sidewith significant guarding. No appreciable mass. Does notguard on the left side. Normal BS present. Right flankappears prominent and somewhat dusky and appears TTP
Back: No appreciable abnormalities, no sacral dimple ortuft
Ext: warm and well perfused, no edema, no cyanosis
Skin: slightly mottled but with cap refill < 3 seconds,erythematous rash in the diaper area
NEURO: appropriately awake, appropriate withinterventions, grossly normal strength and tone, patellarDTRs intact
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Summary
10 day old female infant with 1 week of
progressive difficulty feeding, 1 day of feeding
refusal, fever, and weight loss with abdominal
tenderness and flank swelling.
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Differential Diagnosis
Viral infection, bacterial infection/sepsis,
severe dehydration, pneumonia, congenital
heart disease, arrhythmia, myocarditis,
malrotation/volvulus, NEC, NAT, inborn errorof metabolism (urea cycle defects, organic
acidemias, aminoacidopathies, galactosemia),
hypoglycemia, botulism, CAH, toxic exposure,seizures, NAS,
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Labs
Urine: large hgb, trace protein, small LE, 10
WBC, 25 RBC, 2+ bact.
CSF: 3 WBC,
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More labs:
CBC: WBC 10.6, hct 46, plt 77 (34B, 48N, 14L,
3M)
CMP: na 134, k 8.4, cl 102, bic 16, BUN 75, Cr
3.91, ca 7.9, prot 5.3, alb 2.9, bili 3.7, alk phos
143, alt 24, ast 25.
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ED interventions
40 ml/kg of NS
Blood, urine, CSF obtained
Amp, cefotax started CXR, EKG
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Imaging:
CXR: normal
Abdominal CT:1. No left kidney identified.
2. Large right kidney is consistent compensatory hypertrophy..Rounded low-attenuation lesions in the right kidney mayrepresent areas of infarction, focal pyelonephritis, abscess or
may be cysts.3. Moderate amount of ascites.
4. Soft tissue swelling in the right lateral abdominal wall isprobably cellulitis.
Renal US:1. Solitary right kidney with compensatory hypertrophy and
multiple simple cysts within the parenchyma of the kidney. Nohydronephrosis.
2. Fluid lateral to the right kidney of uncertain etiology.
3. Moderate ascites.
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Congenital anomalies of kidney
Hypoplasia (small kidney < 2 std. dev below nlsize)
Aplasia (what this patient had)
Dysplasia (also what this patient had) Multicystic dysplasia
Renal tubular dysgenesis (rare)
Genetic cystic diseases Anomalies of migration (horshoe kidney)
Anomalies of collecting system
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Renal Agenesis
Unilateral accounts for 5% of renal malformations
Incidence approx 1:2900 births
Male:female of 1.7:1
Multiple factors implicated (genes, teratogens,environment)
Majority asymptomatic
Other urological abnormalities in up to 33-65% VUR= most common in up to 37% (also in this
patient)
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Renal Agenesis
Other assoc. anomalies
Cardiac (septal defects most common)
Genital tract
Gastrointestinal Respiratory
Skeletal
Females at risk for Mullerian anomalies Vaginal duplication
Uterine didelphys
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Multicystic dysplastic kidney
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Renal Dysplasia
Unilateral or bilateral
2-4 per 1000 births
M:F 1.3:1 bilateral, 1.9:1 unilateral Infants with bilateral dysplasia may have renal
dysfunction and may have subsequent renal
failure