PURPOSE: While treating 14 phenylketonuria(PKU) patients, we evaluated bone density, changes in bone age, andbony changes such as spiculation or metaphyseal widening. MATERIALS AND METHODS: A total of 14 PKU patients agedbetween 1 month and 14 years(mean, 6.4 years) were under dietary treatment. Eight and eleven patients underwentradiography of the left hand and wrist and bone densitometry(BMD) of the lumbar spine, respectively. The resultswere reviewed with regard to abnormal bony changes, delayed bone age, and osteopenia. Patients were assigned toeither the early or late treatment group, depending on whether or not dietary therapy was started before 3 monthsof age. Those in whom a blood phenylalanine level of under 10 mg/dl was maintained were assigned to the 'goodcontrol' group; others were classified as 'variable control'. The findings of radiographs of the left hand andlumbar BMD were evaluated in relation to the time of dietary therapy, and adequacy of treatment. RESULTS: Onlumbar BMD, four of 11 patients (36%) showed reduced bone density of more than 1 S.D. None of the 11 who underwentradiography of the left hand showed bony abnormalities such as spiculation or metaphyseal widening. In four of the11, bone age was less than chronological age by at least one year. According to Fisher's exact test there was norelation between delayed bone age , osteoporosis and the time and adequacy of dietary therapy (p >0.05). CONCLUSION: None of the 14 PKU patients who underwent dietary therapy had bony abnormalities such as spiculationor metaphyseal widening. In four of the 11, bone age was at least one year less than chronological age, and onlumbar BMD, osteoporosis was seen. For the evaluation of bone change in PKU patients, plain radiography and BMDare thus complementary.
Bone Density ; Bone Diseases, Metabolic ; Hand ; Humans ; Metabolism ; Osteoporosis ; Phenylalanine ; Phenylketonurias* ; Radiography ; Spine ; Wrist

The development of leukemia during pregnancy is rare and difficult to diagnosis and treatment. Acute leukemias are among the most common malignant neoplasms of young women, but paradoxically, their incidence complicating pregnancy is cited to be 0.9 to 1.2 cases per 100,000. l'he signs and symptoms of acut:e leukemia may mask the signs and symptoms of early pregnancy. 'I'he occurrence of acute leukemia during pregnancy raises many therapeut,ic and ethical dilemmas because of the potential tetatogenic effects of chemotherapy and the danger of fetal wastage, in addition to the well-known problem of marrow suppression in the mother. We experienced a case where diagnosis of a woman at the 15 gestational weeks revealed acute lymphocytic leukemia and immediate treatment. as well as termination of pregnancy was made to prevent abnormal neonatal birth. We present this case with review of related literatures.
Bone Marrow ; Diagnosis ; Drug Therapy ; Female ; Humans ; Incidence ; Leukemia ; Masks ; Mothers ; Parturition ; Precursor Cell Lymphoblastic Leukemia-Lymphoma* ; Pregnancy*

An acardiac twin is one of the very rare anomalies that occurs in monochorionic twins and the incidence of this is about one out of 35,000 births. We present the serial prenatal ultrasound findings, along with the postnatal histologic correlation, of an acardiac twin that manifested as a single lower extremity.
Fetus ; Humans ; Incidence ; Lower Extremity ; Parturition ; Ultrasonography, Prenatal

The congenital partial anonychia is known to be very rare developmental anomalies of the nail. Recently, we experienced a case of congenital partial anonychiain male newborn infant due to autosomal dominance. In this condition there complete absence of nails on the both thumb, index, middle fingers and toes were affected in a similar way to the fingers. But there were no other associated abnormalities. We present a case with a brief review of literature.
Fingers ; Humans ; Infant, Newborn ; Male ; Thumb ; Toes

Invasive micropapillary carcinoma (IMPCa) is a rare variant of invasive ductal carcinoma of the breast. This variant is associated with a set of peculiar cytological findings and aggressive biological behaviors. In most reported cases, IMPCa has involved massive axillary lymph node metastases at the time of diagnosis. We experienced four cases of cytological features of IMPCa, all of which were verified by histological examination. Fine needle aspiration cytology (FNAC) revealed malignant epithelial cells, which formed small, oval to angulated papillary clusters, which lacked central fibrovascular cores. The histological findings of the four cases revealed both pure and mixed forms of IMPCa, composed of cohesive malignant epithelial cells, surrounded by distinctive clear spaces and separated by thin fibrous septa. All patients evidenced axillary lymph node metastases at the time of diagnosis. It is important to identify the peculiar cytological findings which would differentiate IMPCa from other diseases.
Biopsy, Fine-Needle* ; Breast* ; Carcinoma, Ductal ; Diagnosis ; Epithelial Cells ; Humans ; Lymph Nodes ; Neoplasm Metastasis

Hydatidiform moles are generally separated into two classifications. Complete hydatidiform moles are characterized by cystic swelling of all villi, often pronounced diffuse trophoblastic hyperplasia, lack of fetal parts, all 46 chromosomes of paternal origin (androgenesis). Partial hydatidiform moles appear to be focal trophoblastic hyperplasia, a fetus or indications of previous fetal existence, 69 chromosomes. Hydatidiform mole with coexistent fetus is a very rare phenomenon, with an estimated incidence of 0.005 to 0.01 percent of all pregnancies and associated with a risk of persistent gestational trophoblastic tumor. Recently, hydatidiform moles with a fetus have become more common due to use of assisted reproductive technology. Most patients with hydatidiform mole and coexisting normal fetus must cautioned about the potential for risks of malignant change and severe medical complications, such as preeclampsia, hyperthyroidism and antepartum hemorrhage, that may necessitate prompt pregnancy termination. We experienced a case of partial hydatidiform mole with coexistent live fetus, which was diagnosed by ultrasonography at 15 gestational weeks. A brief reviews of related literature was done.
Classification ; Female ; Fetus* ; Hemorrhage ; Humans ; Hydatidiform Mole* ; Hyperplasia ; Hyperthyroidism ; Incidence ; Pre-Eclampsia ; Pregnancy ; Reproductive Techniques, Assisted ; Trophoblastic Neoplasms ; Trophoblasts ; Ultrasonography

PURPOSE: To evaluate the radioloic findings of malignant retroperitoneal fibrosis. MATERIALS AND METHODS: Post-contrast CT (n=9) and urographic (n=7) findings of nine patients with malignant retroperitoneal fibrosis were retrospectively analyzed. Primary tumors were found to be advanced gastric cancer (n=6), early gastric cancer (n=1), breast cancer (n=1), and cervical cancer (n=1). We analyzed CT findings with regard to the site of soft tissue lesion, ureteral involvement, the presence or absence of hydronephrosis, and distant metastasis. The level and length of ureteral involvement, presence or abscence of ureteral stenosis, and ureteral displacement as seen on urography, were analyzed. RESULTS: On CT scans, enhanced soft tissue lesions (mass, 5 cases; plaque, 4 cases) encircling the abdominal aorta and IVC were noted in all cases. Thickening of the ureteral wall (n=8), hydronephrosis (n=9), and enlarged lymph node (n=5) were also seen. On urography, irregular stenosis and medial displacement of ureters from level L2 to S2 were noted in all cases. The length of ureteral involvement was 4-6.5cm. CONCLUSION: The common CT findings of malignant retroperitoneal fibrosis were enhanced soft tissue lesion encircling the abdominal aorta and IVC, hydronephrosis, and thickening of the ureteral wall. On urography, ureteral stenosis and medial displacement were seen.
Aorta, Abdominal ; Breast Neoplasms ; Constriction, Pathologic ; Humans ; Hydronephrosis ; Lymph Nodes ; Neoplasm Metastasis ; Retroperitoneal Fibrosis* ; Retrospective Studies ; Stomach Neoplasms ; Tomography, X-Ray Computed ; Ureter ; Urography ; Uterine Cervical Neoplasms

Multiple large bowel polyps are the hallmark of familial adenomatous polyposis (FAP), and many progress to colorectal cancer. Desmoid tumors are more common in patients with FAP than in other people, occurring, particularly, in those who have previously undergone prophylatic total colectomy. In such patients, desmoid tumors are a common cause of death. In an FAP patient without extracolic manifestation, who has undergone prophylatic surgery, multifocal desmoid tumors occur periodically. We report the serial radiologic findings of progressive desmoid tumors in FAP, drawing attention to the related findings of previous research.
Adenomatous Polyposis Coli ; Cause of Death ; Colectomy ; Colorectal Neoplasms ; Fibromatosis, Aggressive* ; Humans ; Polyps

The subjects of the study were the 120 mothers who had children hospitalized in 6 hospitals in P city during the period from Feb. 1. to May 30. 1996. The purpose of the study are (1) the problem encountered when they carried out Home Care after discharge and (2) the degrees of demand that they had against Home Care Services. In order to investigate the purpose above-mentioned the researchers used the 55 items of Home Care services belonging to 10 areas selected by Korea Home-Care Association in 1994. The items were modified and supplemented to 4-point scale. The selected data were analyzed by SPSS:PC+ and the following results were derived. 1. The subjects payed great attention to 5 items such as "pain control, OPD visits, diet-control problems" among the 15 sources of anxiety that they had against Home Care services after discharge. Especially, "OPD visits and knowledge deficit" would to be overcome by the nursing activities of the Home Care Nurses. 2. The acknowledge level of the subjects to the Home Care Project that governmental plans to carry out was surveyed and found as under : 50.0% responded they heard about Home Care Nursing System. 60.8% said that they thought the system was "very necessary" and "somewhat necessary". 65.0% said they would like to use the system. However, the acknowledge level of 50.0% is still low and is thought to need further national promotion and propagation. 3. The demand of the subjects against 55 Home Care Services was investigated and found as

. "Assessment, Lab, basic nursing technology, relay & transfer, training & education and curative nursing" showed comparatively high scores. However, the items belonging to pediatric Home Care Services such as "umbilical cord care, breast=feeding, infant care, and post-parum care & loan of breast-pump" showed relatively low scores.
Anxiety ; Child ; Child, Hospitalized* ; Education ; Home Care Services* ; Humans ; Infant ; Infant Care ; Korea ; Mothers ; Nursing ; Child Health