Corrigendum: Sacral Curvature in Addition to Sacral Ratio to Assess Sacral Development and the Association With the Type of Anorectal Malformations.

[This corrects the article DOI: 10.3389/fped.2021.732524.].

Sacral Curvature in Addition to Sacral Ratio to Assess Sacral Development and the Association With the Type of Anorectal Malformations.

Introduction: Sacral ratio (SR) is currently the only measurement to quantitatively evaluate sacral development in patients with anorectal malformations (ARM). This study proposes sacral curvature (SC) as a new indicator to qualitatively assess the sacrum and hypothesizes that sacral development, both quantitatively and qualitatively, can be an indicator to predict the type of ARM. The study aims to investigate the difference of SR and SC between ARM types and the association with the type of ARM. Methods and Materials: This study was retrospectively conducted between August 2008 and April 2019. Male patients with ARMs were enrolled and divided into three groups based on the types of ARM: (1) rectoperineal fistulae, (2) rectourethral-bulbar fistulae, and (3) rectourethral-prostatic or rectobladder-neck fistulae. SC was measured in the sagittal views of an MRI or a lateral radiograph of the sacrum. Results: Included in the study were 316 male patients with ARMs. SRs were 0.73 ± 0.12, 0.65 ± 0.12, and 0.57 ± 0.12 in perineal, bulbar, and prostatic/bladderneck fistula, respectively (p < 0.01). The SCs in perineal fistulae and bulbar fistulae were significantly higher than that in prostatic/bladderneck fistulae (0.25 ± 0.04, 0.22 ± 0.14, and 0.14 ± 0.18, p < 0.01). When SR ≥ 0.779, there was an 89.9% of possibility that the child has a perineal fistula. When SR ≤ 0.490 and SC ≤ 0, the possibilities of the child having prostatic/bladderneck fistulae were 91.6 and 89.5%, respectively. SC < 0 was also noted in 27 (27.8%), 19 (10.5%), and no (0%) patients of prostatic/bladderneck, bulbar, and perineal fistulae (p < 0.01), respectively. Sacral defect was noted in 63% of patients with SC ≤ 0, compared to none with SC > 0 (p < 0.01). Conclusions: The higher the rectal level is in an ARM, the lower are the objective measurements of the sacrum. SC ≤ 0 is associated with sacral defects and implies a high likelihood of prostatic/bladderneck fistulae.

Primary liver actinomycosis in a pediatric patient: A case report and literature review.

BACKGROUND: Primary hepatic actinomycosis is a rare infection that can be clinically confused with hepatic pyogenic abscesses or neoproliferative processes. Only a few cases of primary hepatic actinomycosis in children have been reported in the English literature. CASE SUMMARY: We describe a pediatric patient with primary hepatic actinomycosis that involved the base of the right lung and anterior abdominal wall and skin. The patient was diagnosed via histological examination of spontaneously drained material. The patient was successfully treated with an exploratory laparotomy and right posterior segmentectomy of the liver, combined with antibiotic treatment. Following surgery, the patient remains in excellent condition, without evidence of recurrence at the time of drafting this report. To summarize the clinical manifestations, diagnosis, treatment, and outcomes of primary hepatic actinomycosis, 18 case reports in English were reviewed. CONCLUSION: We conclude that actinomycosis clinically features a chronic onset, nonspecific symptoms, and a primarily histologic diagnosis. Prolonged antibiotic treatment combined with invasive intervention provides a good prognosis.

Middle-term bowel function and quality of life in low-type anorectal malformation.

BACKGROUND: Low-type anorectal malformations (ARMs) are considered benign; however, in China, data regarding such conditions are limited. Thus, this study aimed to assess the middle-term bowel functions and quality of life (QOL) among children with low-type ARM. METHODS: Children > 3 years of age who underwent treatment for low-type ARM (vestibular fistula [VF] and perineal fistula [PF]) during 2013 and healthy children were included. The children were interviewed during their outpatient visits. The primary outcome measures were bowel function, as assessed using the Baylor Continence Scale (BCS), and QOL, as measured using the Pediatric Quality of Life Inventory (PedsQL 4.0). RESULTS: A total of 82 patients responded; mean patient age was 6.8 (range, 3-12) years. Mean BCS score in the control group (7.94 ± 4.74) was significantly lower than that in the VF (18.69 ± 11.11, P < 0.001) and PF (15.47 ± 6.50, P < 0.001) groups. However, there were no differences in PedsQL 4.0 scores among the groups. The patients scored the lowest for emotional function and the highest for physical function. Nearly all measurements of QOL significantly decreased with increased BCS score. CONCLUSIONS: Children with low-type ARM can achieve good bowel control and QOL. However, although ARMs are benign, several children with this condition suffer from anal function problems that affect QOL. Redo operations, mislocated anus, and incorrect constipation treatment are the iatrogenic causes of fecal incontinence.

Establishment of an induced pluripotent stem cell model of Hirschsrpung disease, a congenital condition of the enteric nervous system, from a patient carrying a novel RET mutation.

Hirschsprung disease (HSCR) is a complex genetic disorder of the enteric nervous system that is characterized by a complete loss of the neuronal ganglion cells in the intestinal tract. It is one of the most frequent causes of congenital intestinal obstruction and more than 80% of the causative mutations are in RET. Here, we identified a new RET mutation in a patient and established a cell model that can be used to elucidate the pathogenesis of HSCR. Peripheral blood was collected from a patient who was clinically and pathologically diagnosed with HSCR with a heterozygous deletion mutation (c.180delT; p.Glu61ArgfsX163) in exon 2 of RET. Patient-derived induced pluripotent stem cell (iPSC) lines were generated from dermal fibroblasts. Using immunofluorescence staining and RT-PCR, we showed that the generated iPSCs expressed the pluripotency markers OCT4, SSEA4, SOX2, TRA-1-60, and NANOG. We also showed that the HSCR-iPSCs could differentiate into cells from all three germ layers by spontaneous in-vitro differentiation. In addition, 3 months after the administration of a subcutaneous injection of these iPSCs into nude mice, teratomas with all three germ layers were observed. We identified a new RET gene mutation causing HSCR and successfully established a human iPSC line from an HSCR patient carrying this novel RET mutation, which could be useful in pathogenesis studies of HSCR.