Case report

We report the case of a 17-year-old, 19-kg male who presented with the chief complaint of a right lower extremity animal wound complicated by the development of lower right extremity cellulitis. Our patient has a complex past medical history including a cardiac diagnosis of hypoplastic left heart syndrome. After successful Norwood/Sano and Glenn procedures, he underwent a low-risk fenestrated Fontan procedure at age four. Extracardiac history is significant for hypogammaglobulinaemia with severe T-cell deficiency, osteoporosis, congenital hypothyroidism, and heterozygous pathogenic deletion of chromosome 2q13.

He was initially diagnosed with protein-losing enteropathy approximately three months after his Fontan procedure in 2011. Cardiac catheterisation at that time demonstrated acceptable haemodynamics and no important anatomic obstruction or valve regurgitation. He was managed with standard of care heart failure medications and traditional protein-losing enteropathy therapies for many years, with only occasional need for supplemental albumin. He was subsequently referred for cardiac transplant; however, he was denied secondary to his immunologic disorder.

In 2023, he progressed to severe protein-losing enteropathy recalcitrant to standard therapy requiring weekly outpatient albumin infusions and frequent admissions for intensive albumin repletion. Catheterisation and imaging were performed in August 2023, which was significant for a hypoplastic innominate vein with a large decompressing vein to the superior vena cava, Dori type 3 lymphatics, and acceptable haemodynamics. Cardiac catheterisation was repeated in April 2024, at which time the innominate vein was successfully dilated without evidence of remaining decompression channels.

Immediately preceding his admission, he failed intravenous dopamine therapy, which was complicated by occlusion and thrombosis of the central line, necessitating line removal, dopamine discontinuation, and thrombosis treatment with low-molecular-weight heparin. He was maintained with close follow-up and outpatient albumin infusions until February 2024. Over the following five months, he required nine admissions and received a monthly average of 184 g (9.7 g/kg) albumin to maintain a monthly average serum level of 2.2 g/dL. In the few weeks prior to admission, his average albumin serum level was 1.45 g/dL despite 150 g repletion.

Our patient was most recently admitted for cellulitis treatment with intravenous ampicillin/sulbactam and subsequently transitioned to enteral amoxicillin/clavulanate. After clinical resolution of cellulitis, his course was further complicated by difficulty maintaining albumin serum concentrations within patient-specific goals as well as resulting fluid overload requiring close titration of diuretics. His albumin on admission was 1.1 g/dL, and he progressively required 1.5–3 g/kg albumin repletion one to three times per week while admitted. He continued to be unresponsive to traditional supportive measures consistent with his history.

He developed persistently increased stool output not felt to be related to his recent antibiotic therapy for cellulitis and was diagnosed with Cryptosporidium gastroenteritis. His stool output remained significantly elevated at a 7-day average of 39 cc/kg/day with continued detection of Cryptosporidium in the stool despite a 22-day course of nitazoxanide (500 mg twice daily) and high-dose loperamide. He was converted to salvage therapy with a 2-week course of azithromycin (250 mg once daily) and paromomycin (250 mg twice daily) combination therapy. ^{Reference Sparks, Nair, Castellanos-Gonzalez and White1}

After starting this therapy, his serum albumin levels began to rise without the need for repletion. Simultaneously, his stool output improved as depicted in Figure 1. Six weeks into admission and two weeks without albumin repletion, combination antimicrobial therapy was discontinued. He was then discharged with an albumin level of 3.3 g/dL without changes to his long-standing home medications.

Figure 1.

Trends in serum albumin, supplemental albumin requirements, and select antimicrobial exposure prior to, during, and after admission.

To date, our patient has been without readmission, maintains a normal serum albumin level of 3.7 g/dL and remains clinically well. It has been over 11 months since his last albumin infusion and he remains asymptomatic of his disease.