A Case of IgM Deficiency with B Cell Deficiency Detected by ABO Discrepancy in a Patient with Acute Osteomyelitis

ABO discrepancy refers to an inconsistency between red cell and serum typings and has various causes, including hypogammaglobulinemia. IgM deficiency is a rare disorder that may accompany several conditions such as infection and autoimmune disorders. Here, we describe a case of IgM deficiency discovered during the evaluation of an ABO discrepancy in a 16-yr-old Korean boy. ABO blood grouping showed that while his cell type was O+, serum typing detected only anti-A (3+). Anti-B was not detectable at room temperature but was graded at 1+ at 4degrees C. ABO genotyping revealed an O/O genotype. His serum IgG, IgA, and IgM concentrations were 770 mg/dL (reference range: 800-1,700 mg/dL), 244 mg/dL (reference range: 100-490 mg/dL), and 13.5 mg/dL (reference range: 50-320 mg/dL), respectively. He was diagnosed with acute osteomyelitis on the basis of clinical presentation and imaging studies. The symptoms gradually improved within 3 weeks of treatment. However, the ABO discrepancy and IgM deficiency persisted even 6 months after recovery and lymphocyte subset analysis revealed CD19+ B cell deficiency. To the best of our knowledge, IgM deficiency detected by ABO discrepancy in a patient with acute osteomyelitis has not been reported before.

A Case of IgM Deficiency with B Cell Deficiency Detected by ABO Discrepancy in a Patient with Acute Osteomyelitis

ABO discrepancy refers to an inconsistency between red cell and serum typings and has various causes, including hypogammaglobulinemia. IgM deficiency is a rare disorder that may accompany several conditions such as infection and autoimmune disorders. Here, we describe a case of IgM deficiency discovered during the evaluation of an ABO discrepancy in a 16-yr-old Korean boy. ABO blood grouping showed that while his cell type was O+, serum typing detected only anti-A (3+). Anti-B was not detectable at room temperature but was graded at 1+ at 4degrees C. ABO genotyping revealed an O/O genotype. His serum IgG, IgA, and IgM concentrations were 770 mg/dL (reference range: 800-1,700 mg/dL), 244 mg/dL (reference range: 100-490 mg/dL), and 13.5 mg/dL (reference range: 50-320 mg/dL), respectively. He was diagnosed with acute osteomyelitis on the basis of clinical presentation and imaging studies. The symptoms gradually improved within 3 weeks of treatment. However, the ABO discrepancy and IgM deficiency persisted even 6 months after recovery and lymphocyte subset analysis revealed CD19+ B cell deficiency. To the best of our knowledge, IgM deficiency detected by ABO discrepancy in a patient with acute osteomyelitis has not been reported before.

Effects of Osmolality and Osmotic Agents on Viability and Proliferation of Human Peritoneal Mesothelial Cells / 대한신장학회잡지

High glucose activates protein kinase C, induces reactive oxygen species generation, and upregulates expression of transforming growth factor-beta1(TGF-beta1) and fibronectin by human peritoneal mesothelial cells(HPMC). High glucose also induces premature senescence in mesothelial cells. Mesothelial cells shrink after exposure to hypertonic medium and intracellular uptake of amino acids increase to ensure subsequent volume increase. Based on these observations, new and more biocompatible peritoneal dialysis solutions that are glucose free and/or iso-osmolar have been developed. We investigated the effects of different osmolality and different osmotic agents including glucose, mannitol, and icodextrin on viability and proliferation of HPMC. HPMC were obtained from the omental tissues of consenting patients undergoing Cesarean section or elective abdominal surgery. All experiments were performed using cells in the 2nd or 3rd passage. Near-confluent HPMC grown in culture dishes were incubated with serum-free medium for 48 hours to arrest and synchronize cell growth. Lactate dehydrogenase(LDH) release was measured for cell viability and [3H]-thymidine incorporation for proliferation of cultured HPMC, after exposing HPMC to different concentrations of glucose, mannitol, and icodextrin for up to 96 hours. High glucose and mannitol at concentrations up to 100 mM(375 mOsm) did not increase LDH release up to 96 hours compared to control M199. When HPMC were exposed to 2, 4, 7.5, and 9% of icodextrin for 24-96 hours, LDH release did not increase. Glucose at 30, 50, and 100 mM significantly inhibited [3H]-thymidine incorporation by HPMC at 24 and 48 hours. Mannitol at 30, 50, and 100 mM for 24 hours and at only 100 mM for 48 hours also significantly inhibited cell proliferation. Icodextrin 9% (305 mOsm) inhibited cell proliferation compared with control M-199 at 24 hours. In conclusion, high osmolality per se dose not appear to increase HPMC death. However, high osmolality appears to inhibit HPMC proliferation at early stage. In addition, high glucose appears to inhibit HPMC proliferation independent of osmolality since high glucose continues to inhibit cell proliferation at 48 and 72 hours when mannitol at the same concentration did not. Icodextrin 9% of which osmolality is 305 mOsm inhibits HPMC proliferation at early stage but does not appear to increase HPMC death.

Clinical and Histopathological Analysis of 494 Cases of Percutaneous Renal Biopsies / 대한신장학회잡지

Percutaneous renal biopsy is an essential component for diagnosis and management of glomerular diseases. In order to elucidate clinical and pathological features, 494 renal patients who had undergone renal biopsies at Gil Medical Center from January 1989 to June 1999 were studied retrospectively. The male to female ratio was 1.3 : 1 and average age was 33.2 years. There were 370(83.5%) cases of primary glomerular disease and 58(13.1%) cases of secondary glomerular disease. Among primary glomerular disease, IgA nephropathy was the most common(175 cases), followed by minimal change disease(84 cases), membranous glomerulonephritis(34 cases), and focal segmental glomerulosclerosis(33 cases). In secondary glomerular disease, lupus nephritis was the most common(21 cases), followed by 11 cases of hepatitis B associated glomerulonephritis, 9 cases of Henoch-Sch nlein purpura, and 4 cases of diabetic nephropathy. Among 99 cases of asymptomatic urinary abnormalities, IgA nephropathy was most common(69 cases) followed by 12 cases of thin basement membrane disease, 4 cases of minimal change disease, and 3 cases of focal segmental glomerulosclerosis, membranous glomerulonephritis, nonspecific glomerulonephritis. Among 159 cases of nephrotic syndrome, minimal change disease was most common(60 cases) followed by 25 cases of IgA nephropathy, 23 cases of focal segmental glomerulosclerosis, 21 cases of membranous glomerulonephritis, and 13 cases of lupus nephritis. Documented complication of renal biopsies included 23 cases of gross hematuria, 6 cases of perirenal hematoma, and 4 cases of infection. Death, AV fistula, aneurysm or serious compications that required surgical intervention were not reported. In conclusion, the percutaneous renal biopsy is relatively safe, and useful for diagnosis and management of glomerular diseases. The most common type of primary glomerular disease was IgA nephropathy.

Catheter Ultrasonography as a Tool in the Diagnosis of Tunnel Infection in CAPD Patients / 대한신장학회잡지

In CAPD patients, exit site infection and peritonitis are occasionally not improved due to undetected tunnel infection. The diagnosis of tunnel infection is mainly based on the clinical symptoms and signs which seem insensitive for early diagnosis and timely intervention. We examined usefulness of catheter ultrasonography(USG) as a tool in the diagnosis of tunnel infection in various kinds of infectious complications in CAPD patients. Positive result was defined by the presence of hypoechoic area more than 2mm in diameter along the subcutaneous catheter tunnel. 26 USG were performed in 23 patients who represented normal in 4 cases, exit site infections in 6 cases, tunnel infections in 2 cases, peritonitis in 10 cases and 4 cases of combined infections. The mean age was 53.6 years and mean duration of CAPD and break-in period were 16.7 months and 24.7 days respectively. Positive USG findings were noted in 13 cases(50%). High rate of positive finding was observed in clinically suspected exit site infection (80.3%), tunnel infection(100%) and combined infections (75%). Positive results were most commonly observed near the external cuff area. The most common causative organism was S. aureus followed by Pseudomonas and CNS infections. S. aureus infection most likely resulted in positive sonographic findings and catheter loss despite of antibiotic treatment. We conclude that USG is a sensitive tool for the diagnosis of tunnel infections and tunnel infection caused by S. aureus is at high risk of catheter loss.

Catheter Ultrasonography as a Tool in the Diagnosis of Tunnel Infection in CAPD Patients / 대한신장학회잡지

In CAPD patients, exit site infection and peritonitis are occasionally not improved due to undetected tunnel infection. The diagnosis of tunnel infection is mainly based on the clinical symptoms and signs which seem insensitive for early diagnosis and timely intervention. We examined usefulness of catheter ultrasonography(USG) as a tool in the diagnosis of tunnel infection in various kinds of infectious complications in CAPD patients. Positive result was defined by the presence of hypoechoic area more than 2mm in diameter along the subcutaneous catheter tunnel. 26 USG were performed in 23 patients who represented normal in 4 cases, exit site infections in 6 cases, tunnel infections in 2 cases, peritonitis in 10 cases and 4 cases of combined infections. The mean age was 53.6 years and mean duration of CAPD and break-in period were 16.7 months and 24.7 days respectively. Positive USG findings were noted in 13 cases(50%). High rate of positive finding was observed in clinically suspected exit site infection (80.3%), tunnel infection(100%) and combined infections (75%). Positive results were most commonly observed near the external cuff area. The most common causative organism was S. aureus followed by Pseudomonas and CNS infections. S. aureus infection most likely resulted in positive sonographic findings and catheter loss despite of antibiotic treatment. We conclude that USG is a sensitive tool for the diagnosis of tunnel infections and tunnel infection caused by S. aureus is at high risk of catheter loss.

A Case of Antinuclear Antibody(ANA) Negative Lupus Nephritis / 대한신장학회잡지

Systemic lupus erythematosus is a multisystemic autoimmune disease in which the kidneys are frequently involved. Clinical diagnosis of SLE is based on the criteria of American Rheumatism Association (ARA). A few cases who were classified as SLE by the ARA criteria but were antinuclear antibody (ANA)-negative have been reported. It was reported that critical factor in ANA positivity is the choice of substrate. It is generally accepted that the cultured cell of human origin, especially HEP-2 cell, is better than tissue section or animal cells. Thus, the ANA test is negative only in approximately 2M of SLE patients when human tissue culture cells are used as substrate. We report a 25-year-old man admitted to our hospital because of generalized edema. He was found to have active lupus nephritis(WHO class IV), photosensitivity and pancytopenia. The result of FANA test which used HEP-2 cell as substrate was repeatedly negative, but anti-ds DNA and anti-Ro antibody were positive.

Successful Hemodialysis in Acute Phenobarbital Intoxication / 대한신장학회잡지

Phenobarbital is one of long-acting barbiturate with low lipid solubility and used frequently as an anticonvulsant. However, in severe intoxication, hypotension and respiratory arrest are the major causes of prehospital mortality. Mortality is 3M for blood levels over HO pg/mL and estimated lethal adult dose is 6-10g. No effective antidotes are available. We report a case of phenobarbital intoxication in a 29-year-old female, treated successfully with hemodialysis. She was found corhatose on the day of admission and was alleged to have taken 30g of phenobarbital. On physical examination, the blood pressure was 80/60mmHg, and pulse, 97/min. There was no respiration. Pupil was dilated fully. Corneal and deep tendon reflexes were absent. There was no response to painful stimuli. She was treated conservatively with mechanical ventilation, gut decontamination and forced diuresis. Hemodialysis was tried to remove excess phenobarbital for 13 hours. The blood phenobarbital level at admission was 162.2 pg/ mL, which was decreased to 114.4pg/mL after first session of hemodialysis. On the fifth hospital day, blood level decreased to 41.8 pg/mL and she regained her consciousness. She was discharged on the 10th hospital day without major sequelae.

A Case of Overlapping Syndrome of Primary Membranous Nephropathy and IgA Nephropathy / 대한신장학회잡지

We report a rare case of primary glomerular disease with both features of IgA nephropathy and membranous glomerulonephritis in a 27 year-old woman with nephrotic syndrome. Histologically, glomeruli showed slight mesangial expansion, proliferation of mesangial cells, and short subepithelial spikes on capillary wall. Direct immunofluorescence demonstrated granular IgG and C3 deposits along the capillary walls, and IgA with C3 deposits in mesangium. Granular subepithelial and rnesangial deposits were observed by electron microscopy. The patient showed complete remission after 6 months therapy with steroid and ACE inhibitor.

A Case of Anti-glomerular Basement Membrane Antibody mediated Rapidly Progressive Glomerulonephritis / 대한신장학회잡지

Anti-glomerular basement membrane antibody mediated rapidly progressive glomerulonephritis(anti- GBM antibody mediated RPGN) is defined by the clinical picture of renal failure developing over days or weeks and the histological appearance of crescents and linear immune deposits mediated by the circulating autoantibodies. We report a case of anti-GBM antibody mediated RPGN with review of literature. A 59-year-old female patient was admitted to the Chungang Gil Hospital because of fever and acute deterioration of renal function. On admission, hemoglobin was 7.39g/dL, hematocrit was 20.9%, and BUN/Cr were 39.7 and 5.23mg/dL respectively. Urinalysis showed albumin (1+) with many RBCs. Renal biopsy revealed the presence of segmental or circumferential cellular crescents associated with smooth linear staining of glomerular basement membrane with antibody to IgG. High titer of circulating antibody to glomerular basement membrane antigen was demonstrated by the ELISA. High doses of corticosteroid with plasmapheresis were administered, but her renal function was progressively deteriorated.

Incidence of Hypercalcemia in Dialysis Patients: HD vs. CAPD / 대한신장학회잡지

Oral phosphate binders and high calcium dialysate have been used as standard therapies for dialysis patients to prevent renal osteodystrophy. Calcium containing phosphate binders are used to prevent intestinal absorption of dietary phosphate and to avoid aluminum loading by using aluminum containing phosphate binders. The use of calcium products resulted in hypercalcemia in a substantial portion of dialysis population. Calcium carbonate as a phophate binder is widely used in Korea. However, the incidence of hypercalcemia in Korean dialysis patients has not been reported to date. In this study we evaluated the incidence of hypercalcemia in dialysis patients. Patients with associated diseases that may influence serum calcium level were excluded from the study. A total of 180dialysis patients (116 HD patients and 64 CAPD patients) maintained at Soon Chun Hyang University Hospital were included. Three consecutive 2 monthly measurements of serum calcium, phosphate, albumin, alkaline phosphatase, bicarbonate in HD and two consecutive measurements in 3 month interval in CAPD patients were retrospectively evaluated. Ionized calcium and intact parathyroid hormone (N-terminal) were measured every 6 months. Serum total calcium level was corrected by serum albumin level. Three HD patients(2.5%) were hypercalcemic pre-HD while 50(43.1%) hypercalcemic postdialysis. 5 CAPD patients(7.8%) were hypercalcemic. Pre-HD calcium level did not differ from the value in CAPD patients. An average value of pre-and post-HD calcium, and serum albumin levels were significantly higher in HD patients than those values in CAPD patients(p<0.01). Ionized calcium (p<0.01) and serum PTH(p<0.05) levels were significantly higher, while serum bicarbonate level (p<0.01) was significantly lower in HD patients than in CAPD patients. The amount of calcium carbonate used were 2.2g in HD and 2.8g in CAPD. In conclusion, the incidence of hypercalcemia is low in pre-HD (2.5%) and in CAPD patients(7.8%). However, the high incidence of post-HD hypercalcemia observed in this study advocates a future study to evaluate the effect of low calcium dialyste on calcium-phosphate metabolism.

A Case of Primary Aldosteronism, Nephrotic Syndrome and Chronic Renal Failure: A Diagnostic Dilemma / 대한신장학회잡지

Primary aldosteronism is characterized by hypertension, hypokalemia, low plasma renin activity (PRA) and elevated plama aldosterone (PA) level. Primary aldosteronism is suspected in patients with hypertension and unexplained hypokalemia. In chronic renal failure(CRF), however, renin-angiotensin-aldosterone axis is altered by renal disease per se, antihyppertensive drugs used and volume status. Therefore, it is difficult to diagnose primary aldosteronism in CRF on the basis of serum potassium, PRA and PA level. Recently, we experienced a case of primary aldosteronism associated with nephrotic syndrome and CRF. The patient was a 49 years old woman who presented with 10 year old history of high blood pressure and general weakness of one year's duration. Her initial serum creatinine was 7.3mg/dL and serum potassium 2.6mEq/L. PRA was decreased and PA was markedly increased. Persistent hypokalemia urged to evaluate adrenal gland in this case. The round mass was found in left adrenal gland and it was surgically removed. CRF and nephrotic syndrome can alter serum potassium and PRA and there lies the diagnostic dilemma for primary aldosteronsim. It will be well to consider associated primary alodsteronism in a patient with CRF and persistent hypokalemia.

A Case of Alopecia Universalis Associated with Autoimmune Poly Glandular Syndrome type II / 대한피부과학회지

Autoimmune polyglandular syndrome (APGS) is characterized by the association of two or more endocrine disorders that are mediated by autoimmune mechanism. We report herein a case of alopecia universalis associated with APGS manifesting autoimmune Addison's disease, insulin-dependent diabetes mellitus, Hashimoto's thyroiditis, primary gonadal failure, which met the criteria of APGS type II. Alopecia is less commonly associated PGA type II.

Pseudoxanthomatous Mastocytosis(xanthelasmoidea) Treated with PUVA Therapy

We herein report a case of mastocytosis with unusual cutaneous manifestations resembling pseudoxanthoma elasticum or xanthoma which were exaggerated on the intertriginous areas including axillae, groin and neck in a 39-year-old woman. It was characterized by multiple yellowish papules and plaques with a cobblestone appearance. In addition, there were diffuse red-dish macules and papules of varying sizes mainly on the trunk and extremities. Histopathologic findings showed a very dense infiltrate of large numbers of round to oval cells in the upper and mid dermis. These cells revealed metachromasia on the toluidine blue stain. She also had hepatosplenomegaly and some evidence of bone marrow involvement. There was significant improvement in,the skin lesions with psoralen plus ultraviolet A(PUVA) therapy and potent topical corticosteroid.

The Protective Effect of Marly SkinR on Housewife' s Eczema / 대한피부과학회지

BACKGROUND: It is important to avoid the direct contact with water of detergents as possible for the treatment and prevention of hand eczema. OBJECTIVE: The purpose of this study is to evaluate the protective effect of skin protector, Marly skin on housewifes eczema, METHODS: The patients with hand eczema apply skin protector, Maly skin, four times a day on their both hands. They are evaluated with the scores of subjective symptor is(itch, dryness, smarting) and objective signs(erythema, scaling, fissures), and skin replica which are taken from the specified site are analysed by using an image analyser and, scanning electron micrascipe. RESULTS: 4 weeks after application of skin protector, there are statstically significant decrease of total clinical symptom scores. Scanning electron microscopic findings of the skin replicas show marked changes between the beginning and the end of the study. On quantive image analysis of the skin replicas, the length of fissure is a little decreased and the depth of rrows is slightly increased than before application of skin protector, but these are not statistically significant. CONCLUSION: Skin protector, Marly skin, has a protective effect on the development of hand eczema.

A Case of Dirty Neck' of Atopics / 대한피부과학회지

We report a case of Dirty Neck in a 27-year-old male suffering from severe atopic dermatitis. 'Dirty Neck', a reticulate pigmentation of the anterior and anterolateral aspects of the neck, is a specific finding in some subjects with atopic dermatitis. Two groups have been indentified. One is found in older patients with severe atopic dermatitis whose pigmentary lesions develop after puberty and show little seasonal variation. The other is found in younger patients with mild eczema who have a increase in pigment during the summer. Our patient showed a dark brown colored, linear, reticulate pigmentation of the neck, especially anterolateral aspeet regarded as the former group. More comprehensive and detailed clinical study is needed to evaluate the importanee of this entity in regard to as a minor clinical feature of Korean atopic dermatitis.

A Case of Sarcoidosis

We report a case of sarcoidosis which developed in the skin, lung and eyes in a 50-year-old woman. The skin lesions showed two types of cutaneous manifestations which were subcutaneous nodules on her back and erytrematous papules on the face for several months. Our patient's seurm angiotensin converting enzyme (ACE) level was elevated at 33.5 IU/L(reference range, 8.3-21.4 IU/L) and the purified protein derivative(PPD) skin test had a negative result. On her roentgenographic examinations, we could find multiple nodular densities with hazziness on both her lungs and radionuclides(Gallium 67) uptakes in right lower lung field. The ratio of helper to suppressor T cells was 9 in bronchial lavage. Diagnosis of sarcoidosis was made on the basis of histologic findings of non-caseating granulomas from skin and lung tissue. Treatment was initiated with oral alternate-day prednisone 40mg. The cutaneous lesions and dyspnea improved in 2 and 4 weeks respectively, and serum ACE level returned to normal in 2 months.

Idiopathic cyclic edema : clinical evaluation / 대한신장학회잡지

No abstract available.

A case of chylopericardium associated with tuberculous mediastinal lymphadenopathy / 대한내과학회지

No abstract available.

A comparison of the acute antiemetic effect of ondansetron with combination of metoclopramide, dexamethasone, lorazepam in patients receiving cisplatin / 대한암학회지

No abstract available.