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