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.

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.

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.

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.

[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.

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.

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).

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.

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.

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.

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.

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.

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.

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.

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.