Transgastric drainage for subdiaphragmatic abscess secondary to perforation of the sigmoid colon after cytoreductive surgery for advanced ovarian cancer.

Drainage of subdiaphragmatic abscesses is difficult due to its anatomical location and it can result in adverse events, including organ damage and the spread of infection. In recent years, endoscopic ultrasonography (EUS) guided drainage for upper abdominal abscesses has become available. We report a case of successful infection control using this procedure for a subdiaphragmatic cyst secondary to perforation of the sigmoid colon after cytoreductive surgery for advanced ovarian cancer. A Japanese woman in her 60s underwent laparotomy for ovarian cancer, and then developed sigmoid colon perforation 6 days after surgery. The emergency reoperation was performed, and a cyst suspected to be an antibiotic-resistant fungal abscess appeared under the left diaphragm in the postoperative period. We adopted an EUS-guided route for diagnostic and therapeutic drainage method, which enabled shrinkage of the cyst and did not concur further adverse events. This procedure was effective as a minimally invasive drainage route for subdiaphragmatic cysts.

Successful pneumovaginoscopic surgery for recurrent abscess of OHVIRA syndrome, preventing further recurrence and subsequent pregnancy.

INTRODUCTION: OHVIRA syndrome is a rare congenital anomaly of Müllerian duct development characterized by uterine didelphys, obstructed hemivagina, and ipsilateral renal agenesis. The primary treatment is surgical excision of the obstructed hemivaginal septum and hematometrial drainage. In recent years, minimally invasive approaches such as hysteroscopic or vaginoscopic septum resection have been reported. Furthermore, we originally developed some novel pneumovaginoscopic gynecologic surgeries for years using a device that consists of a cylinder that fits into the vagina and a lid that mounts multiple ports, allowing the vagina to be dilated with carbon dioxide gas, similar to a single-port laparoscope. MATERIALS AND SURGICAL TECHNIQUE: We report a successful pneumovaginoscopic surgery for a complicated recurrent abscess in a patient with OHVIRA syndrome. Conventional surgery was performed with a single forceps in a liquid, as in cystoscopy or hysteroscopy. However, this new surgery allowed multiple forceps in a gas, as in laparoscopy. So pus and blood were aspirated and washed away without leaking into the abdominal cavity via fallopian tubes. The surgical smoke generated by thermal coagulation also aspirated to clean the field of vision immediately. And thick, complicated abscesses were drained successfully. The patient conceived through IVF with ICSI and delivered safely at full term. DISCUSSION: Pneumovaginoscopy could benefit complex vaginal surgery cases, such as abscess formation in patients with OHVIRA syndrome.

Different pharmacoresistance of focal epileptic spasms, generalized epileptic spasms, and generalized epileptic spasms combined with focal seizures.

OBJECTIVE: Among standard treatments for infantile spasms, adrenocorticotropic hormone (ACTH) is reported as the best treatment, but ACTH is ineffective in one-half of the patients. To establish precision medicine, we examined pharmacoresistance of focal epileptic spasms (ES), generalized ES, and generalized ES combined with focal seizures, diagnosed based on the revised seizure classification of ILAE in 2017. METHODS: We conducted a retrospective nationwide study in Japan on the long-term seizure outcome of ES. Long-term seizure outcome was evaluated by seizure-free rate, seizure-free period, and Kaplan-Meier curve. Seizure-free was defined as seizure control for longer than 2 months. RESULTS: From the medical history of 501 patients, 325 patients had generalized ES only (GES group) at the start of the first treatment, 125 patients had generalized ES after focal seizure onset (FS-GES group), seven patients had focal ES after focal seizure onset (FS-FES group), and 24 patients had generalized ES combined with focal seizures after focal seizure onset (FS-GES + FS group). Seizure-free period of ES (generalized ES and focal ES) [mean (95% confidence interval)] was 2.7 (0.0-5.4) months in GES group, 1.1 (0.1-2.2) months in FS-GES group, 1.0 (0.2-1.9) months in FS-GES + FS group, and 0.1 (-0.2-0.5) months in FS-FES group. Seizure-free rate, seizure-free period, and Kaplan-Meier curve of generalized ES were almost the same in GES group and FS-GES group, with characteristics of superior response to ACTH. Mean seizure-free period of generalized ES combined with focal seizures was significantly shorter in FS-GES + FS group than in GES group. Mean seizure-free period of focal ES in FS-FES group was extremely short with exceedingly early relapse. SIGNIFICANCE: Pharmacoresistance was different in generalized ES, focal ES, and generalized ES combined with focal seizures. ES with focal features or with focal seizures may have focal lesions, thus consider surgical options earlier in the course.

An epidemiological study of children with status epilepticus in Okayama, Japan: incidence, etiologies, and outcomes.

To clarify the incidence of first-ever episodes of status epilepticus (SE), their etiologies and outcomes among Japanese children, we performed an epidemiological study in Okayama City. One hundred and twenty patients (69 males, 51 females) experienced first-ever SE episodes between 2003 and 2005. Overall, the annual incidence of SE was 42.0 per 100,000 population (95% CI: 34.5-49.5). The highest incidence was seen in patients aged <2years, especially in the second year of life. Febrile SE accounted for 59 (49.2%) cases, and acute-symptomatic etiologies accounted for 21. Ten were considered to have remote-symptomatic etiologies, and eight to have acute-on-remote-symptomatic etiologies. Ten were classified as cryptogenic/idiopathic epilepsy-related, and 12 were unclassified. Nineteen (15.8%) patients were diagnosed with exanthema subitum, including three with encephalitis/encephalopathy, and 17 (14.2%) patients with influenza, including four with encephalitis/encephalopathy. After SE, eight (6.7%) patients suffered from motor disturbance with or without mental disturbance. One of these died during the follow-up period. Ultimately 34 (28.3%) of the 120 patients had been diagnosed with epilepsy by the end of the follow-up. We conclude that the incidence of SE among Japanese children is higher than the reported incidence among Caucasian children. Febrile SE accounted for approximately half of the cases. Among the etiologies, exanthema subitum was the most important infectious disease, followed by influenza. Both types of infection caused encephalitis/encephalopathy associated with SE as well as febrile SE.

Evaluation of partial body composition using bioelectrical impedance in Japanese children.

To clarify the growth pattern of body composition by body part for the management of childhood obesity, we measured body fat and muscle using BIA (bioelectrical impedance analysis) in 685 Japanese elementary schoolchildren (aged 6-11 years). The growth patterns of percentage body fat (%BF), fat mass (FM), and muscle mass (MM) were examined throughout the whole body and in various body parts. The %BF of the whole body was greater in females than in males, and this difference widened with age. The %BF, FM, and MM in each body part showed similar growth patterns and gender differences to those of the whole body. The mean %BF of the left limbs was higher than that of the right limbs at all age groups. BMI was strongly correlated with %BF in both sexes. In conclusion, the compositions of all body parts change similarly with age, and gender differences are also similar in childhood. The effect of one's dominant arm on body composition is seen at a young age. The accumulation of body composition data according to body part is indispensable for understanding childhood body composition and managing obesity.

[A case of acute encephalitis with refractory, repetitive partial seizures requiring a high concentration of isoflurane to suppress the seizures and showing abnormal findings in the bilateral thalamus on MRI during the convalescent period].

We report a patient with acute encephalitis with refractory, repetitive partial seizures requiring a high concentration of isoflurane to suppress the seizures and showing rare abnormal findings in the bilateral thalamus on MRI during the convalescent period. A five-year-old boy was admitted to our hospital because of fever and convulsions. Shortly after admission, convulsions became frequent and disturbance of consciousness became apparent. Abnormal findings on electroencephalogram indicated encephalitis. Convulsions could not be suppressed by any intravenous anticonvulsants including barbiturates. Inhalational anticonvulsant treatment with isoflurane was performed, but it was ineffective at a low concentration. A high concentration of isoflurane was needed to suppress convulsions. Since a high concentration of isoflurane induces hypotension, various drugs to increase blood pressure were also administered. Convulsions reappeared during withdrawal of isoflurane. Finally isoflurane was withdrawn without inducing status epilepticus by administration of intravenous and oral anticonvulsants during the withdrawal process. Neurological sequelae were very severe and there are no anticonvulsants that have effectively suppressed the convulsions to date. MRI during the convalescent period showed high-intensity signal changes on fluid-attenuated inversion recovery, diffusion-weighted image and T2-weighted image in the bilateral thalamus. But it is uncertain whether these changes were the cause of this disorder.

Predicting obesity in early adulthood in Japanese women.

AIM: We analysed the body measurements of Japanese women to determine which factors may forecast adult obesity and also performed a comparative study of the utility of body mass index (BMI), which is used widely in Western Europe, and percentage of overweight, which is used in Japan. METHODS: Subjects included 244 Japanese women who were born between 1983 and 1986. Using a questionnaire, we investigated anthropometric values from birth to present and parents' present anthropometric data, and analysed factors that correlate with current BMI data. RESULTS: (i) BMI after 10 years of age and BMI increase between ages 7 and 8 years correlated with BMI in adulthood. The carrying over rate of overweight increased with age. Meanwhile, percentage of overweight after 13 years onwards correlated with BMI in adulthood. (ii) Adult BMI positively correlated to both parents' BMI. CONCLUSIONS: (i) For a Japanese woman, BMI in childhood is a good indicator of young adult BMI, and has the possibility of becoming an important parameter to monitor obesity progression. (ii) Therefore, attempts to control obesity in elementary school girls are necessary. (iii) Parents' weights may potentially influence obesity in adulthood; however, further examination of other confounding factors is necessary.

Epstein-Barr virus DNA load and seroconversion in pediatric renal transplantation with tacrolimus immunosuppression.

EBV infection is one of major complications arising in pediatric patients who have undergone renal transplantation. A strong correlation between the grade of immunosuppression and the development of PTLD, one of the most severe EBV-associated diseases, has been recognized. In this study, we monitored the serologic profile in conjunction with peripheral blood EBV-DNA load of 32 children who underwent renal transplantation with tacrolimus as an immunosuppressant. Six patients were EBV-seronegative (EBV-) before the transplantation, and the mean DNA load in the EBV- group was significantly higher than that in the EBV-seropositive (EBV+) group. Seroconversion occurred in five of these patients in a mean period of 22 weeks after the transplantation. The EBV-DNA load in the EBV+ group was maintained at a low level for a year, whereas it increased rapidly to over 1 x 10(5) copies/mL in two patients in the EBV- group three to seven months after the transplantation, which corresponds to the timing of seroconversion, and one of them developed PTLD. These observations suggest that the close monitoring of the EBV-DNA load, along with longitudinal observation of seroconversion, is essential in pediatric renal transplantation, particularly for younger children who are more likely to be EVB-.

An infant case of macroprolactinemia with transient idiopathic central precocious puberty.

Macroprolactinemia was recognized more than a decade ago as a cause of hyperprolactinemia and the prevalence of macroprolactinemia is thought to be 10%-26% of patients with hyperprolactinemia. However, there are few published reports about macroprolactinemia in childhood. We report a 7-year-and-1-month-old girl with hyperprolactinemia due to macroprolactinemia with the complication of transient idiopathic central precocious puberty (ICPP). At the age of 6 years and 9 months, she was diagnosed with ICPP at another clinic, on the basis of isolated mammary development and increased height velocity with slightly advanced bone age. At that time, the unexpected finding of high PRL level was also observed. Four months later, she was referred to our clinic for persistently high PRL level. At this time, other endocrinological data showed prepubertal stage and we demonstrated macroprolactinemia and the presence of anti-PRL autoantibody. After other causes of hyperprolactinemia such as prolactinoma and stress were ruled out, we finally diagnosed her with hyperprolactinemia due to macroprolactinemia. Because most patients with macroprolactinemia are symptom-free despite hyperprolactinemia and drug therapy would not be indicated, macroprolactinemia should be suspected even in children to avoid unnecessary examinations and treatments.

An epidemiological study of children with status epilepticus in Okayama, Japan.

BACKGROUND: The incidence of status epilepticus (SE) in Asian children, including Japanese, has not been reported. METHODS: In 2003, we performed an epidemiological study of SE on Japanese children (31 days or older to <15 years of age) in Okayama City by ascertaining all lifetime first episodes of SE. RESULTS: Thirty-seven patients (22 males and 15 females) were identified. The annual incidence of SE was 38.8 per 100,000 population (95% CI: 24.5-49.5). Febrile SE in the absence of CNS infection accounted for 17. Acute symptomatic etiologies other than febrile SE were observed in eight patients, including three cases of influenza encephalitis/encephalopathy. Five were classified as remote symptomatic and the remaining seven as cryptogenic. The highest incidence (155.1/100,000) was seen in the age range of 31 days or older to <1 year, followed by 101.5/100,000 in the age range of one year, and the incidence decreased after eight years. In 26 of the 37 patients, SE was their first seizure. As for seizure types, 32 had convulsive SE, including tonic status in one. Five others showed nonconvulsive SE, including complex partial SE in four and absence status in one. No one died of SE. Two patients who brought on SE because of influenza encephalitis/encephalopathy suffered from motor disturbance with or without mental disturbance after SE. CONCLUSIONS: The incidence of SE tended to be higher in Japanese children than reported in Caucasians. The Japanese had an age-specific incidence pattern similar to that of Caucasians.

An infant case of Graves' disease with ophthalmopathy.

Graves' disease is a rare disorder in children, particularly in infants. Ocular manifestations of Graves' disease in children are even more rare and are mild compared to adults. We report a 3-year-old girl with Graves' ophthalmopathy who visited our clinic because of lacrimation. Her family had also noticed exophthalmos, goiter, irritability and increased appetite for more than 3 months. The ophthalmologist noted bilateral proptosis, eyelid erythema, lacrimation, entropion of the lower eyelid, and superficial keratitis. Her serum concentrations of free thyroxine and free triiodothyronine were high, and thyroid-stimulating hormone (TSH) was low. Serum samples were markedly positive for antibodies to TSH receptor (TRAb) and thyroid-stimulating antibody (TSAb). Although hyperthyroidism was controlled with propylthiouracil within 3 weeks, her eye signs did not improve. We administered methylprednisolone pulse therapy for ophthalmopathy, but the effect was limited and the lacrimation due to entropion and superficial keratitis persisted. Titers of both TRAb and TSAb decreased slightly and transiently with the pulse therapy. One year later, both titers remained high and eye signs did not improve any more though she was clinically euthyroid. This might indicate that both TRAb and TSAb levels correlate with the clinical course. Therefore, TRAb or TSAb might be good indicators of progress of Graves' ophthalmopathy. Ocular manifestations of Graves' disease should be followed closely with measurements of both TRAb and TSAb even in infant cases.

Age-dependent phosphate homeostasis is regulated by a circulating factor.

To evaluate the age-dependent phosphate homeostasis, we studied the serum inorganic phosphate (P(i)) concentration in 78 recipients, aged 5-25 years, a year after renal transplantation (RT). The significant age-dependent decline of the serum P(i) concentration was observed in recipients (p < 0.0001) as well as in normal children. Our study revealed that a circulating factor may play a central role in the age-dependent change of phosphate regulation in human.

A case of post-transplant hyperparathyroidism treated with ethanol injection.

A 15-year-old boy with chronic renal failure secondary to Alport's syndrome underwent living-related renal transplantation from his 48-year-old father. His primary immunosuppressive regimen was composed of tacrolimus, mizolibine, and methylprednisolone. The postoperative course was satisfactory with one episode of mild acute rejection, treated successfully with methylprednisolone pulse therapy. Two months later, hypercalcemia (11.8-13.2 mg/dl) and hypophosphatemia (2.5-3.0 mg/dl) were noted without any bone symptoms. The serum intact-parathyroid hormone (PTH) and serum alkaline phosphatase levels were 240 pg/ml and 2483 IU/l, respectively. Ultrasound studies revealed enlargement of the two parathyroid glands. Under the diagnosis of tertiary hyperparathyroidism, he underwent percutaneous ethanol injection (PEIT) into the left parathyroid gland. Although levels of serum calcium and phosphorus returned to normal ranges and the intact PTH level decreased to 95 pg/ml with the three injections, another injection was needed to normalize recurrent hypercalcemia 2 months later. The patient experienced only transient mild dysphonia and local pain after PEIT. Although PEIT is believed less effective than parathyroidectomy, it has some advantages such as applicability to high-risk patients, repeatability of treatment, low incidence and severity of side effects.

Successful treatment of Aspergillus peritonitis in a peritoneal dialysis patient.

Aspergillus peritonitis is a rare, potentially fatal complication of continuous ambulatory peritoneal dialysis (CAPD). We report the successful treatment of refractory fungal peritonitis in an 8-year-old girl treated by peritoneal dialysis for 3.3 years. This is the second report of Aspergillus thermomutatus(telemorph: Neosartorya pseudofischeri) in humans. Comprehensive treatment included early removal of the CAPD catheter, the use of liposomal amphotericin B, and the use of itraconazole.

Kidney transplantation after liver transplantation from the same donor: four cases of successful steroid withdrawal.

BACKGROUND: Administration of corticosteroids to kidney recipients has hampered the complete clinical success of kidney transplantation. Because most organ transplantation in Japan is living-related, we had the experience of performing kidney transplantation (KT) after liver transplantation (LT) from the same donor in four patients and successfully withdrew corticosteroid administration. METHODS: Three pediatric and one adult patient received kidney allografts from 3 to 10 months after LT from the same donor. The immunosuppressive regimen consisted of a corticosteroid and tacrolimus. The steroid was withdrawn after KT in all four patients. After complete withdrawal of the steroid, DNA was extracted from two recipients and examined by polymerase chain reaction to detect microchimerism. A mixed lymphocyte reaction (MLR) and cell-mediated lymphocytotoxicity assay (CML) were performed to test for donor-specific hyporesponsiveness. RESULTS: Steroid withdrawal was successfully accomplished after KT in every patient. No steroid-withdrawal-associated complications were observed. In the three pediatric patients, remarkable catch-up growth was observed after steroid withdrawal. In the two patients tested, donor DNA was not detected by polymerase chain reaction, suggesting the absence of microchimerism. MLR and CML showed that recipient lymphocytes reacted against donor lymphocytes at the same level as against the third-party lymphocytes. CONCLUSION: Steroid withdrawal was successfully achieved in four kidney recipients who had received a liver allograft from the same donor. The MLR and CML findings indicated the absence of donor-specific hyporesponsiveness in vitro. Although the precise mechanism is not yet clear, KT after LT from the same donor should be considered as a method that allows steroids to be withdrawn from the immunosuppressive regimen of KT.

Urinary megalin deficiency implicates abnormal tubular endocytic function in Fanconi syndrome.

Normal reabsorption of glomerular filtrate proteins probably requires recycling of the endocytic receptors megalin (gp330) and cubilin. Both receptors are located on the luminal surface of the renal proximal tubule epithelium. Whether abnormal amounts of receptor are present in the urine of patients with Dent's disease, Lowe's syndrome, or autosomal dominant idiopathic Fanconi syndrome was explored. They are all forms of the renal Fanconi syndrome and are associated with tubular proteinuria. Urine samples of equal creatinine contents were dialyzed, lyophilized, and subjected to electrophoresis on nonreducing sodium dodecyl sulfate-5% polyacrylamide gels. Proteins were blotted and probed with anti-megalin IgG, anti-cubilin IgG, or receptor-associated protein. Megalin and cubilin levels detected by immunochemiluminescence were measured as integrated pixels and expressed as percentages of the normal mean values. A striking deficiency of urinary megalin, compared with normal individuals (n = 42), was observed for eight of nine families with Dent's disease (n = 10) and for the two families with Lowe's syndrome (n = 3). The family with autosomal dominant idiopathic Fanconi syndrome (n = 2) exhibited megalin levels within the normal range. The measured levels of cubilin were normal for all patients. These results are consistent with defective recycling of megalin to the apical cell surface of the proximal tubules and thus decreased loss into urine in Dent's disease and Lowe's syndrome. This defect would interfere with the normal endocytic function of megalin, result in losses of potential ligands into the urine, and produce tubular proteinuria.