Ultrasonographic appearance of cervical pregnancy following successful treatment with methotrexate.

We report a case of cervical ectopic pregnancy successfully treated with systemic methotrexate. Conservative management with single-dose methotrexate was undertaken, but owing to the failure of human chorionic gonadotropin (hCG) levels to fall by 15% by day 7 and the persistence of fetal cardiac activity, two further doses of methotrexate were required. The patient's hCG levels were monitored, and repeat transvaginal ultrasonography was performed until complete resolution of the pregnancy by spontaneous miscarriage. We describe the ultrasonographic findings, which showed that the sac size increased despite treatment.     

Postnatal assessment of growth,nutrition, and urinary tract infections of infants with antenatally detected hydronephrosis

The widespread utilization of prenatal ultrasonography and the detection of antenatal hydronephrosis (AH) have raised the importance of postnatal follow-up of these infants. In this study, we aimed to determine the importance of an early diagnosis for the treatment of urinary tract malformations (UTM) as well as the postnatal evaluation of growth and nutrition status and the frequency of urinary tract infection (UTI) in infants with AH. We evaluated 246 infants (183 boys, 63 girls) whose routine antenatal scans showed an anterior-posterior pelvic diameter (APPD) ≥5 mm. Of the 246 patients, 175 (71.1%) were found to be pathological and 71 were evaluated as normal after the follow-up period. The median follow-up periods of normal and abnormal cases were 45.7 and 43.4 months, respectively. All cases with or without UTM were evaluated in terms of UTI, scars on DMSA, growth [Height Z score (HZ), Weight Z score (WZ)] and nutrition [Weight height index (WHI)] status. The annual UTI frequency was higher in cases with UTM (1.32 ± 1.66 episode/year) than in cases without abnormality (0.27 ± 0.67 episode/year) (P < 0.001). The postnatal evaluation of growth and nutritional status in children with UTM (mean WHI, HZ, and WZ scores: 96.82 ± 10.21, 0.03 ± 0.54 and 0.04 ± 0.61, respectively) was found to be significantly worse than in cases without abnormality (102.25 ± 9.84, 0.14 ± 0.64 and 0.24 ± 0.76, respectively), (P < 0.05). In abnormal patients, the mean WHI, HZ, and WZ were significantly improved to 101.63 ± 9.75, 0.26 ± 1.07, and 0.28 ± 0.98, respectively, and HZ or WZ scores were found to be similar when compared to normals. In conclusion, postnatal early management of infants with AH seems to prevent frequent UTIs and nutritional disturbances enabling normal growth.     

Protracted febrile myalgia syndrome in familial Mediterranean fever

Familial Mediterranean fever (FMF) is an autosomal, recessively inherited multisystem disease that affects various groups of people originating from the Mediterranean Sea region, most specifically those of Jewish, Turkish, Armenian, and Arabic ethnicity. Recurrent attacks of fever and sterile polyserositis of the peritoneum, synovial membranes, and pleura are the main clinical features, although the clinical features of FMF have been expanded in recent years to also include severe myalgia, scrotal swelling, cardiac involvement, and protracted febrile myalgia syndrome (PFMS). PFMS is seen in only a small percentage of FMF patients and is characterized by severe debilitating myalgia of the upper and lower extremities and high fever, occasionally accompanied by abdominal pain, diarrhea, arthritis/arthralgia, and transient vasculitic purpura mimicking Henoch-Schönlein purpura (HSP). Here, we report on a patient with FMF who also presents with PFMS, which is an uncommon and severe manifestation of the disease.     

Transient gestational diabetes insipidus diagnosed in successive pregnancies: review of pathophysiology,diagnosis, treatment,and management of delivery

Gestational diabetes insipidus (GDI) is a rare disorder characterised by polyuria, polydypsia, and excessive thirst usually manifesting in the third trimester of pregnancy. The etiology is thought to depend on excessive vasopressinase activity, a placental enzyme that degrades arginine-vasopressin (AVP), but not 1-deamino-8-d-arginine vasopressin (dDAVP), which is a synthetic form. This is a transient syndrome and may be associated with acute fatty liver of pregnancy and preeclampsia. The use of dDAVP in symptomatic cases has been proven as a safe method for both the mother and the fetus during the pregnancy. We report a case of recurrent gestational diabetes insipidus in successive pregnancies, which responded to dDAVP and subsided after delivery. This report was presented at 8th European Congress of Endocrinology held in Goteborg, SWEDEN in 3–7 September 2005.     

Merosin-negative congenital muscular dystrophy: diffusion-weighted imaging findings of brain

Merosin-negative congenital muscular dystrophy is a rare genetic disease of childhood involving the central and peripheral nervous system. There were high signal intensities throughout the centrum semiovale, periventricular, and sub-cortical white matters on T2-weighted images in a 4-year-old girl with merosin-negative congenital muscular dystrophy. An apparent diffusion coefficient map revealed increased signal intensity and apparent diffusion coefficient values in the periventricular and deep white matters. It may be attributable to increased water content in the white matter because of an abnormal blood-brain barrier rather than to decreased or abnormal myelination.     

Executive and verbal working memory dysfunction in first-degree relatives of patients with bipolar disorder

The authors aimed to investigate cognitive performance of first-degree relatives of probands with bipolar disorder (BD). They hypothesized that the relatives of BD patients would have impaired performance on cognitive tests of frontal-executive functions. A neuropsychological battery was administered to 34 first-degree relatives of BD probands and 25 control subjects. Relatives showed significant impairment in verbal working memory and executive function. Verbal memory and psychomotor performances of relatives were not different from control subjects. One particular component of executive function, cognitive flexibility, was associated with family history of mood episodes with psychotic features. Verbal working memory and executive function deficits may be useful endophenotypic markers of genetic vulnerability to BD.