Correspondence Address: Arvind Bagga Department of Pediatrics, Division of Pediatric Nephrology, All India Institute of Medical Sciences, New Delhi India
Source of Support: None, Conflict of Interest: None
How to cite this article: Rajeev N, Mathew G, Sinha A, Hari P, Bagga A. Atypical hemolytic uremic syndrome with critical illness neuropathy. Asian J Pediatr Nephrol 2019;2:48-9
How to cite this URL: Rajeev N, Mathew G, Sinha A, Hari P, Bagga A. Atypical hemolytic uremic syndrome with critical illness neuropathy. Asian J Pediatr Nephrol [serial online] 2019 [cited 2021 Jul 25];2:48-9. Available from: https://www.ajpn-online.org/text.asp?2019/2/1/48/258557
We report a patient with atypical hemolytic uremic syndrome who presented with diaphragmatic paralysis during the acute illness. She had a satisfactory recovery of diaphragmatic paralysis with conservative management.
A 7-year-old girl presented with anasarca for 5 weeks and progressive pallor and decreased urine output for 4 weeks. There was no history of fever, bleeding manifestations, jaundice, dark urine, headache, altered sensorium, seizures, fever, diarrhea, cough, coryza, or breathlessness. At presentation to another center, she was noted to have severe anemia, respiratory distress, congestive cardiac failure, and hypertension. Investigations showed anemia, thrombocytopenia, and deranged renal function, without evidence of microangiopathic hemolysis. She required mechanical ventilation and multiple episodes of peritoneal dialysis over 15 days. Three episodes of extubation failures were associated with subglottic stenosis.
At presentation to this center, the child was undernourished and had tachypnea with respiratory distress. She had paradoxical breathing; blood saturation ranged from 88% to 92% in room air that required noninvasive ventilation. Investigations revealed severe anemia (hemoglobin level 5.6 g/dL), thrombocytopenia (66,000/mm3), microangiopathic hemolysis (4%–5% schistocytes), and deranged kidney functions (urea 250 mg/dl, creatinine 6.2 mg/dL). Urinalysis showed 3+ proteinuria and microscopic hematuria (25–30 red cells/high-power field). Renal ultrasound showed normal-sized kidneys with increased echogenicity. A diagnosis of anti-factor H (FH)-associated hemolytic uremic syndrome (HUS) was made in the presence of low levels of complement (C3 62 mg/dL) and elevated anti-FH antibody titer (2100 AU/ml; normal <150 AU/ml). Blood level of B12 level was low (19 pg/ml, normal 25–150); levels of folate and transaminases were normal. The child attained hematological remission promptly following the initiation of double-volume plasma exchanges and corticosteroids. The patient required hemodialysis for 6 weeks.
During the hospital stay, there were frequent and intermittent episodes of respiratory distress along with paradoxical breathing, hypoxia, and hypercarbia. Chest X-ray showed reduced lung volume without features of collapse, fluid overload, effusion, or asymmetry of diaphragmatic domes. Ultrasound of the thorax suggested bilateral diaphragmatic palsy, which was confirmed on videofluoroscopy [Video 1].
While muscle tone, power, and deep-tendon reflexes were normal, nerve conduction study showed diffuse axonal polyneuropathy. Bilateral phrenic nerve palsy was attributed to critical illness neuropathy, compounded by severe malnutrition and B12 deficiency. Therapy included 1 g/kg of intravenous immunoglobulin (IVIG), noninvasive positive pressure ventilation, nutrition rehabilitation by nasogastric feeding, and B12 supplements.
At the time of discharge, the child was maintaining saturation in room air and was off dialysis. At 4 months follow-up, she was well with normal renal functions and normal pulmonary function on spirometry. There was no evidence of diaphragmatic palsy; nerve conduction study did not show features of neuropathy.
The central nervous system is affected in nearly 30% of patients with atypical HUS in the acute phase of the illness, usually in the form of altered sensorium, seizures, visual loss, and basal ganglia involvement.,, Peripheral neuropathy and myelopathy were reported to coexist with thrombotic microangiopathy in patients with cblC mutations. Critical illness neuropathy is observed in 33%–100% of patients in intensive care units (ICUs). Risk factors include multiorgan dysfunction, female sex, acute kidney injury, and prolonged ICU stay. Diaphragmatic dysfunction, seen in up to 64% of patients, is usually transient and recovers rapidly with conservative management.,
Diaphragmatic weakness and generalized neuropathy might be a consequence of any of multiple etiologies simultaneously observed in the present patient. Severe malnutrition along with Vitamin B12 deficiency put her at high risk of neuropathy. The occurrence of HUS along with multiorgan dysfunction, uremia, and prolonged mechanical ventilation during ICU stay might have lead to generalized motor axonopathy and bilateral diaphragmatic paralysis. Diaphragmatic palsy was confirmed on video fluoroscopy and resolved following nutrition rehabilitation, renal replacement therapy, B12 supplements, IVIG, plasma exchanges, and noninvasive mechanical ventilation. Tracheostomy was avoided, and the patient showed recovery from HUS and the bilateral diaphragmatic paralysis.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
We would like to acknowledge the support of the Indian Council of Medical Research towards evaluation for antibodies to complement FH assay in a research grant (5/7/1090/2013-RHN).
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