Bilateral bartholin's gland abscesses in a 4-year-old girl with vitamin a deficiency: a case report.

BACKGROUND: A Bartholin's gland abscess is one of the most common infections in women of reproductive age. Although Bartholin's gland abscesses have been reported in prepubertal children, they are rarer in prepubertal children than in adults. Herein, we report a case of bilateral Bartholin's gland abscesses in a 4-year-old girl with vitamin A deficiency. CASE PRESENTATION: A 4-year-old girl diagnosed with autism spectrum disorder was admitted to the hospital for close examination and treatment because of persistent fever and malaise. The child was a marked fussy eater and was diagnosed with corneal ulceration and night blindness secondary to vitamin A deficiency. Both of the patient's labia were swollen, and a diagnosis of a bilateral Bartholin's gland abscess was made using computed tomography. Incisional drainage was performed under general anesthesia. The patient's postoperative course was uneventful, and she was discharged from the hospital on day 8 after the surgery. During hospitalization, attempts were made to correct the vitamin deficiency by adding nutritional supplements to the diet. Three months after the surgery, no recurrence of abscesses was noted. CONCLUSIONS: Decreased immunocompetence and mucosal barrier function due to vitamin A deficiency is thought to be the underlying cause of Bartholin's gland abscesses. Although prepubertal Bartholin's gland abscesses have been reported, they are rare. To the best of our knowledge, no reports of bilateral Bartholin's gland abscesses potentially caused by vitamin A deficiency have been reported. When prepubertal girls present with Bartholin's gland abscesses, the presence of immunodeficiency due to vitamin or trace element deficiency should also be considered.

Genetic autopsy and genetic counseling for a case of fatal oligohydramnios due to de novo 17q12 deletion syndrome.

We report here a fatal oligohydramnios case, which was suspected due to autosomal recessive polycystic kidney disease at first, but genetic analysis using chorionic tissue and umbilical cord after stillbirth led to the diagnosis of 17q12 deletion syndrome. Subsequent genetic analysis of the parents showed no 17q12 deletion. In this case, if the fetus had autosomal recessive polycystic kidney disease, the recurrence rate in the next pregnancy was suspected to be 25%, but since it was a de novo autosomal dominant disorder, the recurrence rate is extremely low. When a fetal dysmorphic abnormality is detected, a genetic autopsy not only helps to understand the cause but also provides information about the recurrence rate. This information is important for the next pregnancy. A genetic autopsy is useful in cases of fetal deaths or abortions resulting from fetal dysmorphic abnormalities.

A cohort study of the universal neonatal urine screening for congenital cytomegalovirus infection.

OBJECTIVES: This prospective cohort study aimed to evaluate the efficacy of the universal neonatal urine screening, followed by diagnosis, workup and antiviral therapy for symptomatic congenital cytomegalovirus (CMV) infection to reduce neurological impairments and sequelae. METHODS: Neonates born in three facilities underwent the universal urine screening of PCR analyses for CMV-DNA. Neonates with symptomatic congenital CMV infection (cCMV) received oral valganciclovir (VGCV) of 32 mg/kg/day for six weeks or six months, and were evaluated for neurological outcomes including developmental quotient (DQ) and hearing function at around 18 months of corrected age. RESULTS: cCMV was diagnosed in 56 (0.48%) of 11,736 neonates, consisting of 23 neonates with symptomatic and 33 with asymptomatic cCMV. The incidence of cCMV in the general perinatal medical center (0.69%) was higher than that in the primary maternity hospital (0.23%, p<0.01%). Twenty of the 23 infants with symptomatic cCMV received VGCV therapy, and 19 underwent neurological assessment. Eight neonates (42%) had severe sequelae of DQ < 70, bilateral hearing dysfunction, and/or epilepsy. Four neonates (21%) had mild sequelae of DQ 70-79 or unilateral hearing dysfunction only, and seven (37%) showed normal development without any impairment. CONCLUSIONS: This study on a large scale demonstrated that a series of universal neonatal urine screening, diagnosis, workup, and VGCV therapy for neonates with symptomatic cCMV may decrease neurological impairments, because 58% of the treated infants had normal development or mild sequelae. The universal urine screening likely identifies subclinical symptomatic cCMV. Mothers with fetuses of cCMV seem to be selectively transferred to perinatal medical centers before deliveries.

Impact of tocolysis-intent magnesium sulfate and beta-adrenergic agonists on perinatal brain damage in infants born between 28-36 weeks' gestation.

AIMS: Magnesium sulfate has neuroprotective effects in preterm infants. Whether other antepartum treatments interfere with the neuroprotective actions is not well known. This study aims to explore the impacts of antenatal administration of Magnesium sulfate or beta-2 adrenergic agonists as tocolytic agents on the developing brain in premature infants. METHODS: This is a retrospective cohort study in four tertiary perinatal centers in Japan. We collected data of pregnant women and infants born between 28 and 36 weeks for tocolytic agents, gestational age, sex, antenatal corticosteroid, fetal growth restriction, pathological chorioamnionitis, low umbilical arterial pH values (<7.1), multiple pregnancy, mode of delivery and institutions after excluding clinical chorioamnionitis, non-reassuring fetal status or major anomalies. Tocolytic agents were categorized into four groups: no-tocolysis, magnesium sulfate, beta-2 adrenergic agonists and the combination of them. We conducted multiple comparisons with multivariate analyses using generalized linear regression models to compare the prevalence of a poor perinatal outcome defined as infant's death, brain damage, particularly cerebral palsy and developmental delay. RESULTS: Among 1083 infants, 39% were no-tocolysis, 47% were magnesium sulfate, 41% were beta-2 adrenergic agonists and 27% were combination group, including the duplication. The incidence of poor perinatal outcome was decreased by magnesium sulfate (OR 0.27, 95% CI 0.10-0.72), but not changed significantly by beta-2 adrenergic agonists (OR 1.28, 95% CI 0.63-2.59) or the combination group (OR 2.24, 95% CI 0.67-7.54), compared with the no-tocolysis. CONCLUSION: The combination therapy for tocolysis with beta-2 adrenergic agonists diminished the magnesium sulfate neuroprotective action after adjusting for covariables.

[Laparoscopic Gastrectomy for Gastric Adenocarcinoma of the Fundic Gland Type].

Case 1: A 66-year-old man underwent esophagogastroduodenoscopy(EGD), which showed a slightly elevated lesion at the greater curvature of the cardia. We diagnosed gastric adenocarcinoma(tub1, 2)as a result of the biopsy. Endoscopic submucosal dissection(ESD)was performed. The pathological examination revealed a gastric adenocarcinoma of the fundic type(GA-FG), with a tumor depth of SM2. Consequently, laparoscopic gastrectomy was additionally performed. Case 2: A 65-year-old woman underwent EGD, which revealed a slightly elevated lesion at the posterior wall of the upper body. We made a diagnosis of GA-FG as on the basis of biopsy resuit. ESD was performed. A pathological examination revealed that the tumor depth was SM2. Consequently, laparoscopic gastrectomy was additionally performed. GA-FG rarely demonstrates metastasis and recurrence. Most cases undergo ESD, few reports of surgical resection exist. We report our experience of laparoscopic gastrectomy for GA-FG.

[A Case of Rectal Gastrointestinal Stromal Tumor(GIST)Treated with Imatinib Mesylate Neoadjuvant Therapy to Preserve the Anus].

A woman in her 40's who initially presented with anal pain was diagnosed with rectal GIST. A 9 cm tumor extended to near the anus, and curative abdominoperineal tumor resection was required. The patient initially received neoadjuvant therapy with imatinib mesylate. Neoadjuvant chemotherapy for 6 months reduced the tumor to approximately 47% of its original size and permitted anus-preserving surgery. The present case suggests that neoadjuvant therapy with imatinib mesylate is useful for large rectal GISTs, from the standpoint of anal function preservation.

Low total IgM values and high cytomegalovirus loads in the blood of newborns with symptomatic congenital cytomegalovirus infection.

AIMS: Neurological outcomes differ considerably between symptomatic and asymptomatic infants with congenital cytomegalovirus (CMV) infection. Our objective was to characterize laboratory markers in symptomatic newborns in comparison with asymptomatic newborns with congenital CMV infection. METHODS: Ten newborns with symptomatic and 13 newborns with asymptomatic congenital CMV infection were included in this 3-year prospective cohort study. Total immunoglobulin M (IgM), CMV-IgM, CMV antigenemia, and CMV-DNA in blood and urine were measured and their positive rates and quantitative values compared between the symptomatic and asymptomatic groups. RESULTS: Fifty percent of newborns in the symptomatic group were positive based on total IgM; this was significantly lower than in the asymptomatic group (100%). Quantitative total IgM values were significantly lower, and there were significantly more copies of CMV-DNA in the blood of symptomatic newborns than in asymptomatic newborns (median values for total IgM: 14 vs. 43 mg/dL and blood CMV-DNA: 3.2×102 vs. 3.5×101 copies/106 white blood cells). CMV-IgM, CMV antigenemia, and urine CMV-DNA did not differ significantly between groups. CONCLUSION: Low total IgM values and high blood CMV loads were associated with the presence of symptoms in newborns with congenital CMV infection.

Factors affecting maternal serum magnesium levels during long-term magnesium sulfate tocolysis in singleton and twin pregnancy.

AIM: Our aim was to determine factors that affect maternal serum magnesium (Mg) levels, to help ensure the safety and efficacy of long-term magnesium sulfate (MgSO4 ) therapy for threatened preterm labor in singleton and twin pregnancies. MATERIAL AND METHODS: We retrospectively and arbitrarily studied 100 patients (singleton pregnancy, n = 65; twin pregnancy, n = 35) who received i.v. MgSO4 for >48 h for tocolysis of threatened preterm labor. We used multiple regression analysis to investigate the functional relations between the candidate factors and maternal serum Mg levels. RESULTS: MgSO4 was administered as a loading dose of 3 g for 1 h followed by a maintenance dose of 1.0-2.0 g/h. There were no maternal severe adverse events related to the elevated Mg levels in any of the subjects. The results of multiple regression analysis revealed that total dose of MgSO4 for 24 h before blood collection (g/day), total serum protein level (g/dL), serum total calcium level (mg/dL), serum creatinine level (mg/dL) and maternal bodyweight (kg) significantly affected maternal serum Mg levels in both singleton and twin pregnancies (all P-values were < 0.001). Gestational age (weeks) and period of MgSO4 administration (days) at blood collection had no significant effect in singleton or twin pregnancies. CONCLUSION: Our study statistically shows that dose of MgSO4 , total serum protein level, serum total calcium level, serum creatinine level and maternal bodyweight are key factors to achieving safe and effective long-term tocolysis with MgSO4 in not only singleton but also twin pregnancies.

The Association between the First Cry and Clinical Outcomes in CDH Neonates: A Retrospective Study.

Congenital diaphragmatic hernia (CDH) is a life-threatening condition characterized by the herniation of abdominal organs into the thorax, resulting in hypoplastic lungs and pulmonary hypertension. The impact of the first cry, a crucial event for lung transition during birth, on CDH patients remains unclear. This study investigated the impact of the first cry during birth on CDH patient survival, along with other prognosis factors. A multi-institutional retrospective study assessed CDH patient characteristics and survival rates by analyzing factors including the first cry, disease severity, birth weight, Apgar scores, oxygenation index (OI) and surgical closure. Among the CDH patients in the study, a positive first cry was linked to 100% survival, regardless of disease severity (p < 0.001). Notably, the presence of a positive first cry did not significantly affect survival rates in patients with worse prognostic factors, such as low birth weight (<2500 g), high CDH severity, low Apgar scores (1 min ≤ 4), high best OI within 24 h after birth (≥8), or those who underwent patch closure. Furthermore, no significant association was found between the first cry and the use of inhaled nitric oxide (iNO) or extracorporeal membrane oxygenation (ECMO). In conclusion, this study suggests that the first cry may not have a negative impact on the prognosis of CDH patients and could potentially have a positive effect.

A new approach to risk stratification using fetal MRI to predict outcomes in congenital diaphragmatic hernia: the preliminary retrospective single institutional study.

BACKGROUND: Congenital diaphragmatic hernia (CDH) is a condition with a wide range of severity. Prenatal diagnosis is essential to optimize postnatal management, especially for severe cases. The lung to head ratio (LHR) and liver herniation estimated by prenatal ultrasound has been used as prenatal predictors in CDH. However, reliability of these factors remains to be proven and prediction of outcome from prenatal imaging is still challenging. We propose our new stratification system using lung to liver signal intensity ratio (LLSIR) in fetal MRI, which has been shown to be related to pulmonary maturation. METHODS: Retrospective chart review was conducted on 25 infants with CDH treated from 2009 through 2016 in our hospital. We stratified patients according to fetal T2-weighted MRI as Grade I, detectable ipsilateral lung at the apex; Grade II, undetectable ipsilateral lung at the apex and contralateral LLSR ≥2.0; Grade III, undetectable ipsilateral lung at the apex and contralateral LLSR <2.0. To evaluate this stratification system, we analyzed survival, severity [inhaled nitric oxide (iNO) usage with or without extracorporeal membrane oxygenation (ECMO)], and requirement of patch closure. RESULTS: All 15 patients survived in Grade I, while 2 out of 6 died in Grade II, and 3 out of 4 died in Grade III (P=0.003). Four were severe in Grade I, and all in Grade II and III who survived (P=0.007). One needed patch in Grade I, and all in Grade II and III (OR: 414,238,332; 95% CI, 0-∞). Liver herniation was noted in five patients, and significantly associated with survival (P=0.04), however, neither with severity (P=1.00) nor with the requirement of patch closure (P=0.52). CONCLUSIONS: The risk stratification algorithm using contralateral LLSIR in fetal MRI could be useful and more reliable than liver herniation to predict survival, severity, and need of patch closure. Further investigation is warranted.

Neurological outcomes in symptomatic congenital cytomegalovirus-infected infants after introduction of newborn urine screening and antiviral treatment.

BACKGROUND: Newborn screening for urinary cytomegalovirus (CMV) and early introduction of antiviral treatment are expected to improve neurological outcomes in symptomatic congenital CMV-infected infants. This cohort study prospectively evaluated neurological outcomes in symptomatic congenital CMV-infected infants following the introduction of hospital-based newborn urinary CMV screening and antiviral treatment. SUBJECTS/METHODS: Following institutional review board approval and written informed consent from their parents, newborns were prospectively screened from 2009 to 2014 for urinary CMV-DNA by PCR within 1 week after birth at Kobe University Hospital and affiliated hospitals. CMV-positive newborns were further examined at Kobe University Hospital, and those diagnosed as symptomatic were treated with valganciclovir for 6 weeks plus immunoglobulin. Clinical neurological outcomes were evaluated at age ⩾12 months and categorized by the presence and severity of neurologic sequelae. RESULTS: Urine samples of 6348 newborns were screened, with 32 (0.50%) positive for CMV. Of these, 16 were diagnosed with symptomatic infection and 12 received antiviral treatment. Four infants developed severe impairment (33%), three developed mild impairment (25%), and five developed normally (42%). CONCLUSIONS: This is the first Japanese report of neurological assessments in infants with symptomatic congenital CMV infection who received early diagnosis and antiviral treatment. Urinary screening, resulting in early diagnosis and treatment, may yield better neurological outcomes in symptomatic congenital CMV-infected infants.

Intraoperative red cell salvage during obstetric surgery in 50 Japanese women.

OBJECTIVE: To determine the clinical usefulness of intraoperative cell salvage (ICS) in obstetrics. METHODS: A retrospective analysis was performed using data for 50 patients who had received ICS blood during obstetric surgery at 13 Japanese facilities between January 1, 2007 and December 31, 2013. The frequencies of ICS-associated adverse events, allogeneic blood transfusion (ABT), and preoperative autologous donation (PAD) were assessed. RESULTS: Placenta previa was the indication for ICS in 42 (84%) women. The ICS blood was reinfused in all women (median 366 mL; range 80 to at least 3715). No ICS-associated adverse events occurred. The median estimated blood loss (EBL) was 2171 mL (range 574-47 000); 27 (54%) women lost at least 2000 mL. ABT was not used in 33 (66%) women. Among 26 women who lost at least 2000 mL of blood and were included in analyses, 12 (44%) did not receive ABT. EBL was linearly correlated with the total volume of transfused blood (P<0.001). CONCLUSION: ICS caused no adverse events among women at elevated risk of peripartum hemorrhage and might be safe for use in obstetrics.

Bioelectrical impedance analysis in the clinical management of preeclamptic women with edema.

OBJECTIVE: The aim of this study was to correlate bioelectrical impedance analysis (BIA) with the clinical course of preeclampsia with edema. DESIGN: 440 pregnant women with apparently normal, single pregnancy participated in this longitudinal study. Anthropometric measurements and BIA were performed during pregnancy and postpartum period. RESULTS: All of the measurements were completed in 333 of the women; 279 of the women had a normal pregnancy (control group). The remaining 54 women developed edema during the third trimester of pregnancy. Of these, 40 women had only edema, and 14 women had edema followed by hypertension and/or proteinuria (preeclampsia group). The BIA index (the height squared divided by resistance) in the control group increased significantly towards late pregnancy, compared to that in early pregnancy. The indexes in the edema group were significantly higher during the third trimester compared to those of the control group at the same gestational week. The index in the preeclampsia group was higher relative to that in the control group. Moreover, a substantial increase in the index preceded the development of edema in the cases in which pregnancy was terminated due to deterioration of preeclampsia. CONCLUSIONS: Our results show that BIA is a useful method for monitoring longitudinal changes in total body water in pregnant women, and that BIA may be a powerful predictor of deterioration of preeclampsia preceded by edema.

Noncompaction of the left ventricular myocardium diagnosed in pregnant woman and neonate.

Noncompaction of the left ventricular myocardium (NCLV) is an uncommon congenital cardiomyopathy with poor prognosis. We describe a case of NCLV that developed in a pregnant woman and her neonate. A nulliparous woman was referred at 24 weeks' gestation due to dyspnea and fetal hydrops. Maternal echocardiography demonstrated NCLV with characteristic findings of prominent and excessive ventricular trabeculations and deep intertrabecular recesses in the left ventricle. An M-mode echocardiography suggested depressed left ventricular systolic function. A fetal echocardiography at 24 weeks' gestation demonstrated cardiomegaly, but morphologic findings were not definitive for NCLV. An emergency cesarean section was performed due to maternal heart failure. A neonatal echocardiography diagnosed NCLV with depressed left ventricular systolic function. The neonate died of cardiac failure on the second day of life. Autopsy confirmed the echocardiographic findings. Since patients with NCLV may develop heart failure, multidisciplinary management is mandatory. In addition, awareness of familial occurrence of NCLV should be kept in mind for early diagnosis in the fetus and neonate.