Pediatr Nephrol (2017) 32:773–775

DOI 10.1007/s00467-016-3453-y

CLINICAL QUIZ

The ‘hole’ story of a lung: Questions

Aadil Kakajiwala 1 & Priyanka Fernandes 2 & Bruce R. Pawel 3,4 & Sandra Amaral 1,4

Received: 23 June 2016 / Revised: 24 June 2016 / Accepted: 27 June 2016 / Published online: 18 July 2016
# IPNA 2016

Keywords Renal transplantation . Immunosuppression .
Fevers . Pulmonary cavitary lesion
Background
A 14 year-old male patient with prune belly syndrome and
chronic kidney disease (CKD) who underwent his second renal
transplant 2 years earlier was admitted with a worsening cough.
Transplant and immunosuppression
The patient underwent his first living-related donor renal
transplant at the age of 3 years from his mother. Eight years
later, the transplant failed secondary to recurrent urinary tract
infections (UTI) and chronic allograft nephropathy. The first
transplant had been managed with thymoglobulin induction,
The answer to these questions can be found at http://dx.doi.org/10.1007
/s00467-016-3454-x.
* Aadil Kakajiwala
kakajiwalaa@icloud.com
1
Division of Nephrology, The Children’s Hospital of Philadelphia,
34th Street and Civic Center Blvd, Philadelphia, PA 19104, USA
2
Division of Infectious Disease, Department of Pediatrics, Boston
Children’s Hospital, Boston, MA, USA
3
Department of Pathology, The Children’s Hospital of Philadelphia,
Philadelphia, PA, USA
4
Perlman School of Medicine, The University of Pennsylvania,
Philadelphia, PA, USA
prednisone, tacrolimus, mycophenolate mofetil (MMF), and
sirolimus. He received a second living-related donor renal
transplant from his father at the age of 12 years. He was in-
duced with thymoglobulin and maintained on prednisone,
MMF, and tacrolimus. One month after his second transplant,
he was noted to have BK viremia, with titers of 5,236,070
copies/ml. In response, MMF was discontinued, and he was
started on leflunomide. Four months later, he experienced a
gradual increase in creatinine, with renal biopsy findings of
mild chronic allograft nephropathy, focal interstitial inflam-
mation, tubular atrophy, and focal vascular hyalinosis, sugges-
tive of calcineurin toxicity. He was started on sirolimus for
immunosuppression (with a goal sirolimus level of 5–8 ng/ml)
and continued on a very low dose of tacrolimus of 1 mg twice
daily, prednisone 5 mg (initially daily and switched to every
other day 8 months after the transplant), and leflunomide
15 mg daily. He developed significant Epstein Barr virus
(EBV) viremia 14 months after transplantation (titers up to
903,013 copies/ml). The EBV viremia improved by 18 months
posttransplantation (52,498 copies/ml). BK viremia also re-
solved and leflunomide was discontinued. Twenty months
after transplantation, his tacrolimus was discontinued due to
another rise in EBV titers (up to 645,692 copies/ml).
Past medical history
Following his second transplant, he had multiple admissions
for UTIs and fevers of unknown origin. About a year after the
transplant, he presented with fevers and abdominal pain. His
white cell count was 31,000/μl and absolute neutrophil count
23,636/μl. Blood and urine cultures at the time were negative.
Within this context, his EBV DNA titers were found to be
elevated to 903,013 copies/ml. Computed tomography (CT)
774 Pediatr Nephrol (2017) 32:773–775

scan of the abdomen with contrast showed perinephric inflam-

mation with peripheral enhancement defects, consistent with
pyelonephritis. There were no enlarged lymph nodes to sug-
gest posttransplant lymphoproliferative disease (PTLD).
Incidentally, in the sub-pleural airspace of the right lower
lobe of the lung, there was a nonenhancing opacity prompting
further imaging. Chest CT with intravenous contrast demon-
strated a single subpleural focus of wedge-shaped airspace
consolidation, measuring ∼3.6 × 1.8 cm, with septal thicken-
ing and contiguous linear stranding in the right lower lobe,
without surrounding ground-glass attenuation. The focus was
reported as likely being due to atelectasis.
Three months later, due to recurrent episodes of fever, he
had a repeat chest CT, again with intravenous contrast, which
showed persistent subpleural airspace opacity within the right
lower lobe without any significant interval change. A magnet- Fig. 1 Computed tomography (CT) scan of chest (axial) showing 2.8-cm
cavitary lesion in the right lower lobe, with increased soft tissue
ic resonance imaging (MRI) study of the brain was performed component and increased cavitary component of the lesion (red arrow)
a month later for evaluation of chronic headaches. This study
showed left maxillary sinusitis. The patient was then evaluat-
ed by an otolaryngologist and was diagnosed with a mucus 5390/μl, and C-reactive protein <0.5 mg/dl. His creatinine
retention cyst of the left maxillary sinus. Following this, the was at his baseline of 1.1 mg/dl. His urine appeared clear
patient continued to have intermittent episodes of low-grade and was negative for leukocytes, nitrites, and protein but
fever, cough, and post nasal drip, leading to his recent showed trace blood. Chest X-ray showed a small opacity
admission. silhouetting the right ventricle border, likely representing at-
Additionally, as a child, he had respiratory syncytial virus electasis. Additionally, in the right lung base, a rounded opac-
(RSV) bronchiolitis, which required hospitalization, and ity was present and was more prominent than at the prior X-
would wheeze due to upper respiratory infections. He had a rays. He was admitted for further workup and management.
history of multiple episodes of pneumonia. Due to chronic
allergies, he was maintained on intranasal fluticasone spray.

Current admission

One month prior to admission, he developed a dry, hacking,

nonproductive cough. Two weeks into his presentation, he
was prescribed a 10-day course of amoxicillin by his primary
care physician. In addition, levalbuterol and over-the-counter
cough syrup was given, with no relief. Over the next 5 days,
the cough became expectorant with greenish-yellow sputum. a b
He was exposed to sick contacts at home and school who also
had upper respiratory tract infections. He had no known other c
exposures, including compost, flower arrangements, or con-
struction within household, hospital, or school. He had no
fevers, sinus tenderness, or emesis. He complained of inter-
mittent diffuse headache. Review of systems was otherwise
negative. He had no recent travel history. Prior purified protein
derivative (PPD) test was negative, and there was no exposure
to tuberculosis.
On presentation to the emergency department (ED), his
heart rate was 116/min and blood pressure 112/66 mmHg.
Significant findings on examination included occasional rhon- Fig. 2 a Gross pathology, b histology, hematoxylin and eosin (H&E)
chi and wheezes. Laboratory tests showed hemoglobin 11 g/ (×20 magnification), c special staining with Gomori methenamine silver
dl, white cell count 9800/μl, absolute neutrophil count (×600 magnification)
Pediatr Nephrol (2017) 32:773–775 775

Summary opacification of the paranasal sinuses, with an air–fluid level

in the right maxillary sinus suggesting acuity. Hyperdense
In consultation with the pulmonology service, inhaled secretions were noted in the ethmoid air cells, with lacy
fluticasone was initiated because of a history of asthma and septations and mucosal thickening of the sphenoid sinuses.
obstructive lung disease on spirometry. Mycoplasma and per- A biopsy of the right nasal turbinate was performed. Nasal
tussis polymerase chain reaction (PCR) were negative. Two mucosal cultures were negative. Nasal mucosal pathology
days after admission, a CT scan without contrast of the si- showed nonkeratinized squamous mucosa with focal mild in-
nuses, chest, abdomen, and pelvis was obtained due to elevat- flammation, fibrovascular tissue with numerous mucous
ed EBV DNA titers (3,186,970 copies/ml). CT of the lungs glands, and mild chronic inflammation.
showed a nonspecific 2.8-cm cavitary lesion in the right lower
lung [Fig. 1]. The soft tissue density portion of the lesion had
Questions
some punctate areas of increased density representing inspis-
sated mucus or calcification. There were patchy opacities in
1. Based on the images in Fig. 2, what is the most likely
the right middle and left lower lobes most suggestive of atel-
diagnosis?
ectasis. There were no pathologically enlarged lymph nodes in
2. What is the differential diagnosis for a patient with a
the chest, abdomen, or pelvis. The patient underwent right
pulmonary cavitary lesion in an immunosuppressed host?
thoracotomy and open lung-wedge resection. Tissue from
3. What are the characteristic findings of the diagnosis on
the lung biopsy specimen was sent for staining and cultures
CT scan?
for aerobic and anaerobic bacteria, fungi, and mycobacteria, as
4. What changes must be considered to the immunosuppres-
per recommendation from the immunocompromised infec-
sive medication regimen when initiating therapy?
tious diseases service.
Biopsy images and histology are displayed in Fig. 2. Based Compliance with ethical standards
on the results, our patient was started on appropriate therapy.
The CT scan of the sinuses showed near complete Conflict of interest The authors have no conflict of interest to disclose
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