Objective To summarize the clinical features of primary nephrotic
syndrome (PNS) complicated by plastic bronchitis (PB) in children to provide
guidance for treatment.
Methods We conducted a retrospective review of the clinical data of
25 children hospitalized with NS complicated by PB in our Hospital between
10/2016 and 03/2019, and summarized the clinical
manifestations, imaging and fiberoptic bronchoscopy (FOB) examinations,
treatment course and outcome of them.
Results 1). The 25 children, with a nephrotic syndrome (NS) course of
one to 36 months, were all diagnosed with PB after FOB, among which 8 cases
(32%) had respiratory failure and required ventilatory support. All
of them started with respiratory symptoms such as fever and cough, and then
suffered from dyspnea and progressive aggravation after 1–3 day(s)
of onset, with rapid occurrence of bidirectional dyspnea and even
respiratory failure in severe cases. 2). Laboratory test for pathogens:
influenza A virus H1N1 (11 cases), influenza B virus (9 cases), adenovirus
(3 cases) and mycoplasma pneumoniae (2 cases). There was no statistically
significant difference (P>0.05) between children with common NS
complicated by influenza virus (IV) infection (not accompanied by dyspnea)
and those with kidney disease who developed PB in the white blood cell
count, lymphocyte count, the inflammatory biomarkers C-reactive protein
(CRP), procalcitonin (PCT) and humoral immunity (IgG level), yet the total
IgG level was found significantly higher and the blood albumin level lower
in the latter (P<0.05). 3). The 25 children were all examined with
the FOB and treated with lavage, 15 of which had typical bronchial tree-like
casts and 10 broken and stringy casts. Based on histopathological
classification, all children were of Type I. 4). Twenty children
(80%) with influenza were administered the antiviral drug
Oseltamivir, 20 (80%) were treated with antibiotics, oral hormones
were replaced with the same dosage of intravenous Methylprednisolone for 5
cases (20%), and 20 (80%) were intravenously administered
gamma globulins (400–500 mg/kg x 3 days). These children
showed a remarkable improvement after treatment and there were no
deaths.
Conclusion NS children are at high risk of influenza virus infection.
Children with a severe case of NS are more susceptible to PB. If symptoms
like shortness of breath, wheezing and progressive bidirectional dyspnea
occur, FOB examination and lavage treatment should be performed as early as
possible. Hyper-IgE-emia and hypoproteinemia may be the high risk factors
for PNS complicated by PB in children.