Methylmalonic acidemia (MMA) is a severe, heterogeneous disorder of methylmalonate and cobalamin (cbl; vitamin B12) metabolism with a poor prognosis that can cause brain damage. Identifying the magnetic resonance imaging (MRI) findings of MMA might help to make accurate diagnoses earlier in the disease course and exploring the relationship between neuropsychological scores and MRI findings, when therapy is more effective and to improve therapeutic efficacy. Cerebral MRI studies from 37 children with MMA were evaluated by a neuroradiologist. Clinical and imaging data were collected from each patient. All tests were performed during routine investigations and in accordance with the ethical principles of the Declaration of Helsinki. Informed consent was obtained from the guardians of all patients for inclusion in the study. The most common and significant findings were periventricular white matter changes (78.4%), ventricular dilation (29.7%) and cerebral atrophy (40.5%). According to the developmental quotient, the 37 patients were divided into the normal intelligence subgroup (NI, developmental quotient ≥ 85) and the low intelligence subgroup (LI, developmental quotient < 85). The incidence of corpus callosal thinning, cortical atrophy, subcortical white matter changes, and ventricular dilation (grades 0–3) was significantly higher in the LI subgroup than in the NI subgroup (P < 0.05). The incidence of no-mild and moderate-severe ventricular dilation was significantly higher in the LI subgroup than in the NI subgroup (P < 0.05). Ventricular dilatation, cerebral atrophy, white matter changes, and corpus callosal thinning are the main MRI abnormalities in MMA patients, and these manifestations are significantly correlated with delayed development in children.

Objective This study aims to evaluate the accuracy of magnetic resonance imaging (MRI) in diagnosing orofacial clefts and proposes specific signs for determining the type of cleft. Methods Pregnant women in whom fetal facial malformations are suspected by ultrasonography (US) underwent 1.5 T MRI. The accuracy of the prenatal US and MRI diagnosis was compared with the postnatal findings. Results A total of 71 fetuses were included in the final analysis, which comprised of 35 cases of isolated cleft lip, six cases of cleft lip and alveolus, 21 cases of cleft lip and palate, four cases of isolated cleft palate, and five normal fetuses. MRI was more sensitive than US (MRI 100%, US 77.5%; Fisher's exact test: MRI vs US P=0.000). The accuracy of MRI for all types was 100%. Specific signs (Inverted T, L/anti‐L, U, and “Notch” signs) were proposed, indicating the normal hard, unilateral cleft, bilateral cleft, and cleft soft palates, respectively, which can facilitate the identification of different types of cleft palates. Conclusion MRI can accurately diagnose the type of cleft based on typical signs, which can serve as a definite diagnostic modality and an effective supplement of US.

This study is to investigate the role of cervical morphology evaluated by magnetic resonance imaging (MRI) in predicting invasive placenta previa (IPP).Totally 105 cases of pregnant women underwent prenatal placental MRI at 32 to 36 weeks of gestation for suspected IPP were included in this study. Cervical morphology (cervical length and placental protrusion) was evaluated independently by 2 radiologists. The association between the cervical morphology and surgery findings was analyzed.Totally, 57 pregnant women were confirmed as IPP. For invasion degree, there were 17 cases with placenta percreta and 40 with accreta. For invasion topography, there were 27 cases with S1 invasion and 30 with S2 invasion. The sensitivity and specificity for the MRI sign of placental protrusion in evaluating cervical invasion of IPP were 100% and 100%, respectively. All the 7 IPP patents with cervical invasion received total hysterectomy and had severe blood loss (3500–6000 mL). The IPP patients with S2 invasion had shorter cervical length than those with S1 invasion (2.66 ± 0.66 cm vs 3.24 ± 0.64 cm, P <.001). However, cervical lengths did not correlate with the degree of invasion (placenta percreta vs placenta accreta: 2.82 ± 0.86 vs 2.99 ± 0.60, P = .21).Placental protrusion is a reliable MRI sign for cervical invasion of IPP, and cervical lengths correlate with the invasion topography.

Short-term efficacy and safety of magnetic resonance-guided focused ultrasound surgery (MRgFUS) for analgesia in children with metastatic bone tumors were studied. Thirty children with pediatric bone metastases admitted to Jinan Maternity and Child Care Hospital from March 2015 to March 2018 and who received MRgFUS treatment of the bone metastasis lesions were collected. The postoperative efficacy was assessed. The observation indicators included the Numerical Rating Scale (NRS) scores, European Organization for Research and Treatment of Cancer (EORTC) and Quality of Life Questionnaire (QLQ) scores before surgery, and 1 week, 1, 2, and 3 months after surgery and the Karnofsky Performance Status (KPS) scores before surgery, and 1 week and 3 months after surgery. The adverse reactions and medications of the children after the surgery were also under observation. NRS and VAS scores of all different observation time-points after surgery were statistically lower than those before surgery (P<0.05), and the NRS scores and Visual Analog Scale (VAS) scores 3 months after surgery were lower than those 1 week after surgery (P<0.05). Compared with the preoperative conditions, the QLQ-C30 scores of terms like physical function, cognitive function, nausea and vomiting, and degree of pain 1 week, 1, 2, and 3 months after surgery were decreased, and the clinical symptoms of the children were relieved (P<0.05). There was a statistical difference between the total QLQ-C30 scores 3 months after operation and the total QLQ-C30 scores 3 months before operation (P<0.05). No serious adverse reactions related to treatment were reported, and the application of analgesics was reduced. MRgFUS relieves bone metastasis pain in children with good curative effect and high safety.

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Background To meet the needs of foetal growth and development, marked changes in lipid profiles occur during pregnancy. Abnormal lipid metabolism is often accompanied by adverse pregnancy outcomes, which seriously affect maternal and infant health. Further understanding of the mechanism of lipid metabolism during pregnancy would be helpful to reduce the incidence of adverse pregnancy outcomes. Methods Pregnant mice were euthanized in the virgin (V) state, on day 5 of pregnancy (P5), on day 12 of pregnancy (P12), on day 19 of pregnancy (P19) and on lactation day 2 (L2). Body weight and energy expenditure were assessed to evaluate the general condition of the mice. Triglyceride (TG) levels, the cholesterol content in the liver, liver histopathology, serum lipid profiles, serum β-hydroxybutyrate levels, fibroblast growth factor-21 (FGF21) levels and the levels of relevant target genes were analysed. Results During early pregnancy, anabolism was found to play a major role in liver lipid deposition. In contrast, advanced pregnancy is an overall catabolic condition associated with both increased energy expenditure and reduced lipogenesis. Moreover, the accumulation of hepatic TG did not appear until P12, after the onset of endoplasmic reticulum (ER) stress on P5. Then, catabolism was enhanced, and FGF21 secretion was increased in the livers of female mice in late pregnancy. We further found that the expression of sec23a, which as the coat protein complex II (COPII) vesicle coat proteins regulates the secretion of FGF21, in the liver was decreased on P19. Conclusion With the activation of ER stress and increased FGF21 secretion during pregnancy, the hepatic TG content changes, suggesting that ER stress and FGF21 may play an important role in balancing lipid homeostasis and meeting maternal and infant energy requirements in late pregnancy.

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