Congenital radial clubhand is a rare deformity, which is characterized by total or partial absence of the radial ray of forearm and hand. We experienced 9 radial clubhands in 7 patients during the last 12 years. Excluding 3 clubhands in 2 patients which were very mild in their deformity, 6 clubhands in 5 patients were operated. Radialization was done in 5 clubhands, and centralization in one. Concomitant corrective osteotomy of ulna was combined in all. The results of the surgical treatment were evaluated by ; radiological findings including Hand-forearm angle(HFA), Hand-forearm position(HFP) and Ulnar bowing(UB) range of motion of the wrist ; and parents' satisfaction. Patients were followed up for average 2 years and 6 months, ranging from 1 to 6 years. All the radiological findings were improved. Although their range of wrist motion was not increased, this was changed into more functional position, that is the radial deviation decreased but ulnar deviation increased. Most parents were satisfied with the surgical results.
Congenital Abnormalities ; Forearm ; Hand ; Humans ; Osteotomy ; Parents ; Range of Motion, Articular ; Ulna ; Wrist

OBJECT: Pre-ALL is a very rare preteukemic state, which percedes acute lymphocytic leukemia, while MDS(pre-ANLL), usually the well-known type of preleukemic state, precedes acute non-lymphocytic leukemia. Initially it shows transient pancytopenia without any evidence of leukemia in bone marrow findings, followed by acute lymphocytic leukemia after recovery from pancytopenia of a short period within weeks or months. We report a case with pre-ALL in childhood. CASE: A 15-month-old male baby was admitted with the complaints of fever and cough for 5 days and pallor for 2 weeks prior to admission. On admission, CBC showed pancytopenia without any evidence of leukemia, which was recovered spontaneously in a short period, and then was followed by acute lymphocytic leukemia of CALLA negative, early pre-B cell type. During antileukemic chemotherapy, he had suffered from severe bacterial infections and was finally died of sepsis 8 months after first admission. CONCLUSION: We report a case of pre-ALL in childhood, which was preceded by CALLA negative, early pre-B cell ALL, with a review of the literatures, briefly.
Bacterial Infections ; Bone Marrow ; Cough ; Drug Therapy ; Fever ; Humans ; Infant ; Leukemia ; Male ; Pallor ; Pancytopenia ; Precursor Cell Lymphoblastic Leukemia-Lymphoma* ; Precursor Cells, B-Lymphoid ; Sepsis

Seven patients with average age of 15years and 6 months (range: 8 years and 11 months 25 years and 6 months) underwent forearm lengthening by callotasis. The indications for lengthening were shortening and/or deformity of the forearm due to exostosis of the distal ulna in three cases, growth disturbance due to physeal injury of the distal radius in three, congenital radial dislocation in one. Three had lengthening of the radius, three of the ulna and one of both the radius and the ulna. The average lengthening achieved was 3.8 cm (3.5 - 4.0) in radius, 2.7 cm (2.3 - 3.0) in ulna. Complications encountered were pin tract infection in two cases, nonunion in one and temporary nerve palsy in one. All of these complications were recovered completely without any residua. Retrospective review after average 41 months of follow-up (range: 36 to 78) showed satisfactory improvement in appearance and function. Callotasis was considered as one of the safe and reliable treatment methods for bone lengthening and deformity correction of the forearm.
Bone Lengthening ; Congenital Abnormalities* ; Dislocations ; Exostoses ; Follow-Up Studies ; Forearm* ; Humans ; Osteogenesis, Distraction* ; Paralysis ; Radius ; Retrospective Studies ; Ulna

There has been a few articles dealing with the incidence of congenital anomalies of the hand in Korea. In this paper, we have analyzed patients who have visited the hand clinic of Seoul National University Children s Hospital from March 1993 to February 1995 to study the relative incidence of congenital anomalies of the hand. We regarded more than two visits of the same patients as one. They were categorized into two groups by our classification system. First group consisted of the patients who showed hand anomaly only. The other group consisted of the patients who had anomalies in the other sites as well as in the hand. The first group was divided into two subgroups; the patients who had single entity of hand anomaly, and those who had more than two entities of hand anomalies. From the clinical analysis based on above criteria, following results were obtained. There were 626 anomalies in 547 patients, 319 (58.3%) males and 228 (41.7%) females (1.4: 1). Unilateral involvements of hand anomaly were noted in 385 (70.3%) patients (229 in right, 156 in left) and bilateral in 162 (29.7%). As a whole, the most common anomaly was trigger thumb (16.5%) and the second was polydactyly (16.1%). Other anomalies, in order of frequency, were syndactyly, camptodactyly, hypoplasia, brachydactyly, cleft hand and so on. In the first group who had hand anomaly only, there were 407 patients who had single entity of hand anomaly. Among these, trigger thumb were 25.3%, and polydactyly 16.2%. There were 68 patients who had multiple entities of anomalies. Among these, the syndactyly was the most common anomaly (23.9%) and the polydactyly was the next (16.2%). Fifty eight (10.6%) patients had anomalies in the hand as well as in the other site. Among these, camptodactyly was the most commonly found (36.5%) and polydactyly was the next common variety (19.0%) in the hand. In the anomalies which occurred in other than hand, arthrogryposis multiplex congenita was the most common (30.0%). Syndactyly of the foot (22.9%) and polydactyly of the foot (18.6%) were the next common anomalies.
Arthrogryposis ; Brachydactyly ; Child ; Classification ; Female ; Foot ; Hand* ; Humans ; Incidence* ; Korea ; Male ; Polydactyly ; Seoul ; Syndactyly ; Trigger Finger Disorder

The purpose of this study was to evaluate the results of various types of opponensplasties which have been performed for the last 15 years. For the patients who had median nerve injury with or without ulnar nerve injury, we usually transferred the ECRL or ECRB to restore thumb opposition and Huber s procedure were usually done for thumb hypoplasia. With the criteria outlined by Sundararaj and Mani, postoperative function of 27 hands from 23 patients who underwent the opponensplasty from March 1981 to March 1995 were evaluated. The results from each etiology group were analyzed by the same criteria. Also subjective opinion of the patients about the scar were investigated by our own criteria. The analysis from overall 27 hands yielded 19 excellent (70.4%), 4 good (14.8%), 3 fair (11.1%), and 1 poor (3.7%) results with Sundararaj and Mani s criteria and 1 excellent (3.7%), 14 good (81.5%), 3 fair (11.1%), and 1 poor (3.7%) results about the scar. In 11 patients with nerve injury (median nerve with or without ulnar nerve injury), 7 excellent and 2 fair results were yielded by the ECRL or ECRB transfer, 1 excellent by Phalen-Miller's procedure, and 1 excellent by Burkhalter's procedure. In 8 patients with congenital hypoplastic thumb, 7 excellent results were yielded by Huber's procedure and 1 excellent by the ECRB transfer. In 8 patients with residual poliomyelitis or brachial plexus injury, the ECRB transfer yielded 3 excellent results. However, 2 good and 1 fair results were obtained by Bunnell's procedure; and l good, l poor by Camitz's procedure. In summary, the ECRL or ECRB transfer in the patients who had neuropathy, such as median with or without ulnar nerve injury, brachial plexus injury or residual poliomyelitis; and Huber's procedure in congenital hypoplastic thumb were thought to be one of the useful methods for the restoration of the thumb opposition.
Brachial Plexus ; Cicatrix ; Hand ; Humans ; Median Nerve ; Poliomyelitis ; Thumb* ; Ulnar Nerve

No abstract available.
Metacarpophalangeal Joint* ; Palmar Plate*

No abstract available.
Coronary Vessels*

No abstract available.
Subarachnoid Hemorrhage, Traumatic*

Dystrophic calcification can be defined as a calcification that occurs in degenerated or necrotic tissue. It is associated with multiple clinical conditions, such as collagen vascular diseases. It involves the deposition of calcium in soft tissues despite no generalized disturbance in the calcium or phosphorus metabolism, and this is often seen at sites of previous inflammation or damage. Potassium-titanyl phosphate (KTP) laser vaporization of the prostate is safe and relatively bloodless procedure that results in a shorter catheterization, immediate symptomatic improvement, and less severe postoperative irritative symptoms. However, longer follow-up studies or reports about complications are lacking. Here in we report a case of dystrophic calcification and stone formation on the entire bladder neck after performing KTP laser vaporization of benign prostate hyperplasia. That was treated by lithotripsy and transurethral resection.
Aged ; Calcinosis/*diagnosis/pathology ; Humans ; Lasers, Solid-State/*adverse effects ; Lithotripsy ; Male ; Prostatic Hyperplasia/*surgery ; Urinary Bladder Calculi/*diagnosis/etiology/ultrasonography ; Urinary Bladder Diseases/*diagnosis/etiology/ultrasonography

Congenital dyserythropoietic anemia Type II (herditary erythroblatic multinuclearity with positive acidified serum test; HEMPAS) is characterized by binuclearity, multinuclearity, pluripolar mitoses, karyorrhexis of normoblasts, and the presence of abnormal antigens on the red cells. We experienced a case of HEMPAS in a 2 month old girl patient who had an intermittent fever, abdominal distention with palpable liver & spleen, and generalized jaundice. The blood analysis revealed anemia, and thrombocytopenia. Peripheral blood smear showed an averge of 6 mature normoblast per 100 leukocyte count. The red cells showed moderate anisocytosis, poikilocytosis, irregularly crenated, contracted cells and occasional spherocytes. The leukocytes showed relative lymphocytosis, and there were occasional villous lymphocytes. The marrow smear showed abnormality in erythroid series. About ten percent of the erythroblasts showed 2~7 nuclei or lobulated nuclei. The mitotic forms of the erythroid precursors were also increased in frequency. The M:E ratio was 1:3.2. Blood culture on this patient showed a pure growth of Coxiella burnetti. Medical treatment with Doxycycline and Rifampin was performed. After treatment, she became afebrile showing improved general condition with decreased size of liver & spleen. In spite of clinical improvement, she died suddenly 3 weeks after initial treatment.
Anemia ; Anemia, Dyserythropoietic, Congenital* ; Bone Marrow ; Coxiella ; Doxycycline ; Erythroblasts ; Female ; Fever ; Hempa ; Humans ; Infant ; Jaundice ; Leukocyte Count ; Leukocytes ; Liver ; Lymphocytes ; Lymphocytosis ; Mitosis ; Rifampin ; Spherocytes ; Spleen ; Thrombocytopenia