Liver Abscess in Chronic Granulomatous Disease—Two Decades of Experience from a Tertiary Care Centre in North-West India

Pilania, Rakesh Kumar; Rawat, Amit; Vignesh, Pandiarajan; Guleria, Sandesh; Jindal, Ankur Kumar; Das, Gargi; Gupta, Anju; Gupta, Kirti; Chan, Koon-Wing; Lau, Yu Lung; Imai, Kohsuke; Singh, Surjit

doi:10.1007/s10875-020-00938-9

Cited by 10 publications

(8 citation statements)

References 34 publications

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“…Seventeen patients presented with liver abscesses (7.2%), and one among these had a recurrent liver abscess (19). Four patients with liver abscess had concurrent other site abscesses (staphylococcal skin pustules in one patient, Pseudomonas aeruginosa cervical abscess in one patient, and multiple deepseated abscesses noted in two other patients).…”

Section: Liver Abscessmentioning

confidence: 99%

“…Site of involvement was variable: ribs-five; lower extremities [tibia (n = 2), fibula (n= 1)]; vertebrae-one; radius-one; skull-one; and small bone osteomyelitis of the hands in two patients. Aspergillus fumigatus was isolated from four patients; Enterobacter sp., Chromobacterium violaceum, A. terreus, and Serratia marcescens were isolated in one patient each (16,19,20). Mycobacterial infection was responsible for two cases of osteomyelitis (one with M. tuberculosis, and another with atypical mycobacterial disease).…”

Section: Bonementioning

confidence: 99%

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Clinical, Immunological, and Molecular Profile of Chronic Granulomatous Disease: A Multi-Centric Study of 236 Patients From India

et al. 2021

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BackgroundChronic granulomatous disease (CGD) is an inherited defect in phagocytic respiratory burst that results in severe and life-threatening infections in affected children. Single center studies from India have shown that proportion of autosomal recessive (AR) CGD is more than that reported from the West. Further, affected patients have high mortality rates due to late referrals and difficulties in accessing appropriate treatment. However, there is lack of multicentric collaborative data on CGD from India.ObjectiveTo describe infection patterns, immunological, and molecular features of CGD from multiple centers in India.MethodsA detailed proforma that included clinical and laboratory details was prepared and sent to multiple centers in India that are involved in the care and management of patients with inborn errors of immunity. Twelve centers have provided data which were later pooled together and analyzed.ResultsOf the 236 patients analyzed in our study, X-linked and AR-CGD was seen in 77 and 97, respectively. Male female ratio was 172:64. Median age at onset of symptoms and diagnosis was 8 and 24 months, respectively. Common infections documented include pneumonia (71.6%), lymphadenitis (31.6%), skin and subcutaneous abscess (23.7%), blood-stream infection (13.6%), osteomyelitis (8.6%), liver abscess (7.2%), lung abscess (2.9%), meningoencephalitis (2.5%), splenic abscess (1.7%), and brain abscess (0.9%). Forty-four patients (18.6%) had evidence of mycobacterial infection. Results of molecular assay were available for 141 patients (59.7%)—CYBB (44.7%) gene defect was most common, followed by NCF1 (31.9%), NCF2 (14.9%), and CYBA (8.5%). While CYBA variants were documented only in Southern and Western parts of India, a common dinucleotide deletion in NCF2 (c.835_836delAC) was noted only in North Indian population. Of the 174 patients with available outcome data, 67 (38.5%) had expired. Hematopoietic stem cell transplantation was carried out in 23 patients, and 12 are doing well on follow-up.ConclusionsIn India, proportion of patients with AR-CGD is higher as compared to Western cohorts, though regional differences in types of AR-CGD exist. Clinical profile and mortality rates are similar in both X-linked and AR-CGD. However, this may be a reflection of the fact that milder forms of AR-CGD are probably being missed.

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Section: Liver Abscessmentioning

confidence: 99%

Section: Bonementioning

confidence: 99%

Clinical, Immunological, and Molecular Profile of Chronic Granulomatous Disease: A Multi-Centric Study of 236 Patients From India

et al. 2021

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“…Histopathological examination of the abscess has shown chronic granulomatous inflammation around the lesion similar to the histopathology seen in patients with CGD ( 8 ). This could be because of the impaired neutrophil function in patients with PLS similar to the neutrophil defect seen in patients with CGD, resulting in inflammatory granuloma ( 9 ). In immunocompetent patients, liver abscess usually has a thin rim with central, liquefied, non-enhancing contents, and responds to antibiotic therapy ( 9 ).…”

Section: Discussionmentioning

confidence: 99%

“…This could be because of the impaired neutrophil function in patients with PLS similar to the neutrophil defect seen in patients with CGD, resulting in inflammatory granuloma ( 9 ). In immunocompetent patients, liver abscess usually has a thin rim with central, liquefied, non-enhancing contents, and responds to antibiotic therapy ( 9 ). Patients with neutrophil-killing defect and hyperinflammation may have atypical features such as dense, homogeneous with thick inspissated fluid in the liver abscess ( 9 ).…”

Section: Discussionmentioning

confidence: 99%

“…In immunocompetent patients, liver abscess usually has a thin rim with central, liquefied, non-enhancing contents, and responds to antibiotic therapy ( 9 ). Patients with neutrophil-killing defect and hyperinflammation may have atypical features such as dense, homogeneous with thick inspissated fluid in the liver abscess ( 9 ). Index cases had dense organized abscess with calcification.…”

Section: Discussionmentioning

confidence: 99%

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Case report: Corticosteroids as an adjunct treatment for the management of liver abscess in Papillon–Lefèvre syndrome: A report on two cases

et al. 2022

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Papillon–Lefèvre syndrome (PLS) is a rare autosomal recessive disorder characterized clinically by palmoplantar keratoderma, periodontitis, and recurrent pyogenic infections. Liver abscess is rarely reported in patients. The use of corticosteroids for the treatment of liver abscess akin to chronic granulomatous disease (CGD) has not been reported previously. Here, we report 2 cases of liver abscess in PLS that responded to corticosteroids.

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Liver Abscesses in Tropics

et al. 2021

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Liver Abscess in Chronic Granulomatous Disease—Two Decades of Experience from a Tertiary Care Centre in North-West India

Cited by 10 publications

References 34 publications

Clinical, Immunological, and Molecular Profile of Chronic Granulomatous Disease: A Multi-Centric Study of 236 Patients From India

Clinical, Immunological, and Molecular Profile of Chronic Granulomatous Disease: A Multi-Centric Study of 236 Patients From India

Case report: Corticosteroids as an adjunct treatment for the management of liver abscess in Papillon–Lefèvre syndrome: A report on two cases

Liver Abscesses in Tropics

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