ABSTRACT
PURPOSE: Congenital limb anomalies are manifested in various degree of severity and complexity bearing confusion for description and nomenclature of each anomaly. We retrospectively analyzed the roentgenograms of congenital limb anomalies for the purpose of further understanding of radiologic manifestations based on the embryonal defect and also to find the incidence of each anomaly. MATERIALS AND METHODS: Total number of the patients was 89 with 137 anomalies. Recently the uniform system of classification for congenital anomalies of the upper limb was adopted by International Federation of Societies for Surgery of the Hand (IFSSH), which were categorized as 7 classifications. We used the IFSSH classification with some modification as 5 classifications;failure of formation of parts, failure of differentiation of parts, duplications, overgrowth, and undergrowth. RESULTS: The patients with upper limb anomalies were 65 out of 89(73%), lower limb were 21(24%), and both upper and lower limb anomalies were 3(4%). Failure of formation was seen in 18%, failure of differentiation 39%, duplications 39%, overgrowth 8%, and undergrowth in 12%. Thirty-five patients had more than one anomaly, and 14 patients had intergroup anomalies. CONCLUSION: The upper limb anomalies were more common than lower limb. Among the anomalies, failure of differentiation and duplications were the most common types of congenital limb anomalies. Patients with failure of formation, failure of differentiation, and undergrowth had intergroup association of anomalies, but duplication and overgrowth tended to be isolated anomalies.
Subject(s)
Humans , Classification , Extremities , Hand , Incidence , Lower Extremity , Retrospective Studies , Upper ExtremityABSTRACT
The functional immaturity of PMNs is one of the major causes of overwhelming sepsis in newborns. In this study, we observed functions and surface markers of PMNs to investigate what causes the functional immaturity of PMNs in newborns. As results, the percentage of EA rosette forming PMNs (58.5 +/- 15.5%) and the chemotactic movement (0.14 +/- 0.09 mm) of cord blood PMNs were significantly lower than those of adult peripheral blood PMNs (70.8 +/- 9.9%, 0.60 +/- 0.34 mm). Cord blood PMNs showed decreased glass adherence and ADCC activity. The expression of Fc gamma RII or Fc gamma RIII was a little lower than those of adult peripheral blood PMNs, but the expression of Fc gamma RI (43.1 +/- 26.8%) was significantly higher than that of adult peripheral blood PMNs (3.2 +/- 1.8%). There was a significant difference in LFA-1 expression between EA rosette forming PMNs (92.9 +/- 9.1%) and EA rosette non-forming PMNs (25.6 +/- 22.6%). From these results, it is assumed that neonatal PMNs may consist of heterogeneous populations. And the relatively high percentage of EA rosette non-forming PMNs which express a low level of LFA-1 may be responsible for the functional immaturity of cord blood PMNs.
Subject(s)
Humans , Antibody-Dependent Cell Cytotoxicity , Cell Adhesion , Chemotaxis, Leukocyte , Fetal Blood/cytology , Lymphocyte Function-Associated Antigen-1/physiology , Neutrophils/physiology , Receptors, IgG/physiology , Rosette FormationABSTRACT
Hereditary spastic paraplegia is a familial disorder which is inherited by autosomal dominant, autosomal recessive or sex linked pattern. We experienced a family who has hereditary spastic paraplegia with mental retardation and extrapyramidal symptom that is thought inherited by autosomal dominant inheritance pattern. A review of literatures was made briefly.
Subject(s)
Humans , Inheritance Patterns , Intellectual Disability , Spastic Paraplegia, HereditaryABSTRACT
Hereditary spastic paraplegia is a familial disorder which is inherited by autosomal dominant, autosomal recessive or sex linked pattern. We experienced a family who has hereditary spastic paraplegia with mental retardation and extrapyramidal symptom that is thought inherited by autosomal dominant inheritance pattern. A review of literatures was made briefly.
Subject(s)
Humans , Inheritance Patterns , Intellectual Disability , Spastic Paraplegia, HereditaryABSTRACT
Serum immunoglobulin concenatrations were measured in 156 patients with various types of glomerulonephritis such as acute poststreptococcal glomerulonephritis, minimal lesion nephrotic syndrome and Henoch-Sch?lein purpura nephritis. 1) Serum IgG level was above normal mean in acute poststreptococcal glomerulonephritis, and normal range in Henoch-Scholein purpura nephritis. Serum IgG concentrations were significantly reduced in minimal lesion nephrotic syndrome. 2) Serum IgA concentrations were normal limit in minimal lesion nephrotic syndrome. In one-forth of acute poststreptococcal glomerulonephritis and one-half of Henoch-Sch?lein purpura nephritis, serum IgA concentrations were elevated. 3) Serum IgM concentrations were normal in acute poststreptococcal glomerulonephritis, minimal lesion nephrotic syndrome and Henoch-Sch?lein purpura nephritis. We concluded that serum immunoglobulin concentrations were not useful as an aid in diagnosing glomerulonephritis.
Subject(s)
Humans , Glomerulonephritis , Immunoglobulin A , Immunoglobulin G , Immunoglobulin M , Immunoglobulins , Nephritis , Nephrotic Syndrome , Purpura , Reference ValuesABSTRACT
A 4 day old male neonate with severe dehydration, uremia, and hyperbilirubinemia presented with bilateral renal vein thrombosis. Following exchange transfusion and peritoneal dialysis renal function returned to normal without nephrectomy. The importance of adequate and prompt management of renal failure to expect the spontaneous recanalization is stressed. Because of the high mortality rate associated with the nephrectomy in neonates, adequate and prompt medical management including peritoneal dialysis or exchange transfusion, if indicated, with expectation of spontaneous recovery is considered the treatment of choice in this age group.