The congenital renal cystic disease encompasses a complex group of pathologic and clinical entities. We retrospectively reviewed 42 cases of congenital renal cystic lesions classified into four Potter types in a series of 2,063 consecutive autopsies from 1981 to 1996. According to our study based on morphologic, clinical, genetic features and associated anomalies, type I and III are relatively compatible with Potter's original definition. However, it was reasonable that type II and IV are classified to the same group because of: 1) very similar histologic findings representing dysplastic kidney, 2) many associated anomalies, 3) no evidence of inheritance, and 4) presence of a combined type. Syndrome associated cysts, such as Meckel-Gruber syndrome, were also separately classified. If the dysplastic evidence was insufficient for diagnosis to the dysplastic kidney in type II and IV, then these cases would be better classified into a cystic disease associated with congenital hydronephrosis. We propose a classification of the congenital cystic disease of the kidney to be: 1) dysplastic kidney, 2) cystic disease associated with congenital hydronephrosis, 3) polycystic kidney, and 4) syndromic cystic disease.
Autopsy ; Classification* ; Diagnosis ; Hydronephrosis ; Kidney* ; Polycystic Kidney Diseases ; Retrospective Studies ; Wills

For the evaluation of the kidney impairment, serum creatinine concentrations or glomerular filtration rates are mainly used, and the conditions of solitary or transplanted kidney and chronic dialysis are also taken into the considerations. Some symptoms and signs of the chronic renal disability in spite of adequate treatment add one additional grade. For evaluating bladder and urethral impairment, the criteria include voiding symptoms and signs. The patients with urinary diversions have impairment grades depending on the alteration of upper urinary tract function. For penile impairment, the degrees are evaluated using the international index of erectile function, nocturnal penile tumescence and color doppler ultrasonography. For evaluating impairment of other male reproductive organs, functional and anatomical changes of these organs, analysis of the semen or hormones and the state of solitary testis are used as the criteria. For evaluating impairment of female reproductive organs, pregnancy potential, requirement of continuous treatment and the ability of sexual intercourse are used. Also, degree of impairment is modified according to the ages in evaluating female reproductive systems. We have tried to make this evaluation system objective, scientific, and convenient, but still find it leaving much to be desired.
*Disability Evaluation ; Female ; Genitalia, Female/physiopathology ; Genitalia, Male/physiopathology ; Humans ; Kidney Diseases/classification/*diagnosis ; Korea ; Male ; Program Development ; Severity of Illness Index ; Urethral Diseases/classification/*diagnosis ; Urinary Bladder Diseases/classification/*diagnosis ; Urologic Diseases/classification/*diagnosis

OBJECTIVETo analyze the effect of special staining and immunohistochemical staining in distinguishing the types of renal amyloidosis to improve the diagnosis accuracy.

METHODSCongo red staining with different methods, and immunohistochemical staining of Kappa, Lambda and Amyloid A with different antigen retrieval methods were used for staining frozen and paraffin-embedded renal tissue sections.

RESULTSWright's Congo red staining produced a better contrast and a higher resolution and showed a greater stability than the other 2 methods after repeated use for staining the renal tissue sections (P<0.05). Immunofluorescent staining produced better results in frozen renal tissue sections than in paraffin-embedded tissues. Immunofluorescent staining produced had better performance than immunohistochemical staining in paraffin-embedded tissues. The retrieval effect with protein kinase K was the best among the antigen retrieval methods in paraffin-embedded tissues.

CONCLUSIONWright's Congo red staining is better than the other 2 methods in diagnosing renal amyloidosis. Immunohistochemical staining of Kappa, Lambda and Amyloid A in frozen renal tissue sections is necessary to discriminate the types of renal amyloidosis. For paraffin-embedded renal tissues, antigen retrieval using protein kinase K is better than the other 2 methods.

Amyloidosis ; classification ; diagnosis ; pathology ; Coloring Agents ; Congo Red ; Fluorescent Antibody Technique ; methods ; Frozen Sections ; Humans ; Immunohistochemistry ; Kidney ; pathology ; Kidney Diseases ; classification ; diagnosis ; pathology ; Paraffin Embedding ; Staining and Labeling ; methods

Objective: To investigate the correlation between the syndrome types of traditional Chinese medicine (TCM) and clinical symptoms of benign prostatic hyperplasia (BPH) with chronic prostatitis (BPH-CP). METHODS: We selected 150 cases of BPH-CP in this study and divided them into 7 TCM syndrome types. Using univariate and multivariate logistic regression analyses, we studied the correlation of each TCM syndrome type with the age, disease course, prostate volume, postvoid residual urine volume (PVR), prostate-specific antigen (PSA) level, maximum urinary flow rate (Qmax), and International Prostate Symptoms Score (IPSS). RESULTS: Kidney-yin deficiency was correlated positively with the prostate volume but negatively with Qmax and IPSS; kidney-yang deficiency positively with the age and prostate volume but negatively with IPSS; the damp heat syndrome positively with the PSA level but negatively with the disease course, prostate volume and Qmax; the spleen-qi deficiency syndrome positively with the prostate volume but negatively with the disease course; liver-qi stagnation positively with the disease course but negatively with the age, prostate volume and PVR; the syndrome of qi stagnation and blood stasis positively with the disease course and IPSS but negatively with PVR; the syndrome of lung-heat and qi blockage positively with the age, Qmax and IPSS but negatively with the disease course. CONCLUSIONS The TCM syndrome types of BPH-CP are closely correlated to their clinical symptoms. The analysis of the clinical objective indexes of BPH-CP can provide some reliable evidence for accurate identification of the TCM syndrome type of the disease.
Age Factors ; Chronic Disease ; Disease Progression ; Humans ; Kidney Diseases ; diagnosis ; Liver Diseases ; diagnosis ; Male ; Medicine, Chinese Traditional ; Organ Size ; Prostate-Specific Antigen ; blood ; Prostatic Hyperplasia ; classification ; diagnosis ; Prostatitis ; classification ; diagnosis ; Qi ; Regression Analysis ; Splenic Diseases ; diagnosis ; Symptom Assessment ; classification ; methods ; Urination ; Yang Deficiency ; diagnosis ; Yin Deficiency ; diagnosis

Smooth muscle antibodies (SMAs) are diagnostic markers for the serological diagnosis of type 1 autoimmune hepatitis. SMA that is restricted to staining of the stomach muscle and blood vessel walls was referred to as "SMA-V". In addition, SMAs are classified into the peritubular (SMA-T) and glomerular (SMA-G) patterns. SMAs are occasionally present in patients with malignancies, but have not yet been reported in thyroid cancer. We came across the first case of SMA positivity in a patient with papillary thyroid carcinoma (PTC). A 31-yr-old male was admitted to our hospital for evaluation of incidentally detected thyroid cancer. He had been diagnosed with PTC based on pathological results following fine-needle aspiration biopsy. The patient underwent total thyroidectomy followed by radio-iodine treatment. The serum levels of AST and ALT were increased before radiotherapy. Tests were conducted for the evaluation of liver disease. SMA was positive at a titer of 1:320, showing positive results for the vessel walls but negative results for the glomerulus and tubules in the kidney (SMA-V pattern). The association of SMA with malignancies and the classification of SMA immunofluorescent subtypes have been previously reported. However, these studies have not clearly established the ability of SMA subtype to predict a specific disease. Therefore, evaluation of an association of SMA pattern with specific diseases in SMA-positive patients may provide additional and useful information for the rapid diagnosis and accurate treatment of patients with autoimmune diseases or malignancies. This case report could serve as a great resource for further studies on SMA.
Antibodies ; Autoimmune Diseases ; Biopsy, Fine-Needle ; Blood Vessels ; Classification ; Diagnosis ; Hepatitis, Autoimmune ; Humans ; Kidney ; Liver Diseases ; Male ; Muscle, Smooth* ; Radiotherapy ; Stomach ; Thyroid Gland* ; Thyroid Neoplasms* ; Thyroidectomy

Sarcopenia was listed in the International Classification of Diseases, Tenth Revision, Clinical Modification (ICD-10-CM) as M62.84, on October 1, 2016. Sarcopenia is primarily associated with metabolic diseases, such as diabetes, obesity, and cachexia, as well as chronic renal failure, congestive heart failure, and chronic obstructive pulmonary disease. Sarcopenia is also significantly associated with osteoporosis in elderly populations and the combined disease is defined as osteosarcopenia. Several studies have confirmed that sarcopenia and osteoporosis (osteosarcopenia) share common risk factors and biological pathways. Osteosarcopenia is associated with significant physical disability, representing a significant threat to the loss of independence in later life. However, the pathophysiology and diagnosis of osteosarcopenia are not fully defined. Additionally, pharmacologic and hormonal treatments for sarcopenia are undergoing clinical trials. This review summarizes the epidemiology, pathophysiology, diagnosis, and treatment of osteosarcopenia, and includes Korean data.
Aged ; Cachexia ; Diagnosis ; Epidemiology ; Heart Failure ; Humans ; International Classification of Diseases ; Kidney Failure, Chronic ; Korea ; Metabolic Diseases ; Obesity ; Osteoporosis ; Pulmonary Disease, Chronic Obstructive ; Risk Factors ; Sarcopenia

PURPOSE: Rapidly progressive glomerulonephritis (RPGN) is a clinicopathologic entity characterized by extensive crescent formation and rapid deterioration of renal function within few months. For better understanding of its clinical course and designing better treatment strategies, a clinicopathological study of childhood RPGN was performed. METHODS: The clinical manifestations and pathological findings were reviewed retro spectively in 12 children who were diagnosed as having RPGN by clinical manifestations and renal biopsy during a period from 1991 to 2003. Several clinicopathological parameters were analyzed as prognostic factors. RESULTS: Among a total of 12 patients, 4 were male and 8 were female. The median onset age was 11.5 years(range 5.5-14.6 years), and the median period of follow-up was 25 months(range 7 months-6.6 years). According to the pathological classification, 10 patients (83%) were type II RPGN(immune-complex mediated glomerulonephritis), 2 patients were type III RPGN(pauci-immune glomerulonephritis), and none was type I RPGN(anti-glome rular basement membrane nephritis). All patients were treated with oral steroid in various combinations with methylprednisolone pulse therapy(10 patients, 83%), cyclophosphamide(8 patients, 67%), or plasmapheresis(4 patients, 33%). Clinical outcomes of 12 patients were complete remission in 1(8%), end-stage renal disease in 2(17%), chronic renal insufficiency with persistent proteinuria in 2(17%), and normal renal function with persistent proteinuria in 7(58%) at the last follow-up. Poor prognosis is associated with increased serum creatinine level, severe anemia and younger age at the time of diagnosis. CONCLUSION: Immune-complex mediated glomerulonephritis is the major cause RPGN in children and most cases showed improvement of renal function with aggressive management. For better understanding of this rare disease, a prospective multicenter study should be done.
Age of Onset ; Anemia ; Basement Membrane ; Biopsy ; Child* ; Classification ; Creatinine ; Diagnosis ; Female ; Follow-Up Studies ; Glomerulonephritis* ; Humans ; Kidney Failure, Chronic ; Male ; Methylprednisolone ; Prognosis ; Proteinuria ; Rare Diseases ; Renal Insufficiency, Chronic

OBJECTIVETo explore the relationship between CD8+ T-cell CD28 molecular expression in peripheral blood and TCM type in patients with chronic aplastic anemia (CAA).

METHODSUsing flow cytometry to detect the CD28 expression in 45 in-patients or out-patients and 24 healthy subjects for control. And the relation with TCM type was analyzed from the immunological aspect.

RESULTS(1) The levels of CD8, CD28, CD8+ CD28+ expression and CD8+ CD28+/CD8+ CD28- were all higher in the CAA patients than those in the healthy subjects (P < 0.05 or P < 0.01). (2) The levels of CD28, CD8+ CD28+ expression and CD8+ CD28+/CD8+ CD28- were all higher in the CAA patients of Shen-Yin deficiency type than those in the CAA patients of Shen-Yang deficiency type (P < 0.05 or P < 0.01).

CONCLUSION(1) The abnormal high expression of peripheral blood co-stimulatory molecules CD28 suggested CD28 disorder may play an important role in immuno-pathogenesis of CAA. (2) The levels of peripheral CD28, CD8+ CD28+ expression and CD8+ CD28+/CD8+ CD28- can be taken as an objective indexes for TCM typing of CAA, which was disordered more severe in patients of Shen-Yin deficiency type than in those of Shen-Yang deficiency type.

Adult ; Anemia, Aplastic ; classification ; diagnosis ; immunology ; CD28 Antigens ; biosynthesis ; CD8-Positive T-Lymphocytes ; immunology ; Chronic Disease ; Diagnosis, Differential ; Humans ; Kidney Diseases ; immunology ; Male ; Medicine, Chinese Traditional ; Yang Deficiency ; immunology ; Yin Deficiency ; immunology

Congenital nephrotic syndrome(CNS) is a rare disease defined by nephrotic syndrome at birth or within the 1st year of life. This study is performed to investigate a classification based on clinicopathology and to evaluate the clinical course and prognosis according to types of CNS. We performed retrospective clinical study with chart review in 8 patients who were diagnosed as CNS from 1980 to 1995. The results were as follows: Their ages at the onset of illness ranged from birth to 7 months (median 2.2 months) and there were 7 males and 1 female. There were proteinuria, hypoalbuminemia and edema in all cases, accompanied with ascites(7cases), hematuria(5cases), hepatosplenomegaly(2cases), umbilical hernia(1case) and inguinal hernia(1case). A classification of these based on clinicopathology showed CNS of Finnish type in 2 patients, congenital syphilitic nephrotic syndrome in 2, mesangial glomerulosclerosis, minimal change disease, Drash syndrome and undefined CNS in 1 each. Of the 8 patients with CNS, 3 died of sepsis and renal failure, 1 responded to steroid and cyclosporin therapy and is alive at 20 months, 1 responded to penicillin, 1 discharged voluntarily, and 2 were lost to follow-up. In conclusion, it had been considered that all forms of CNS except the secondary ones have a very poor prognosis. But if the appropriate management including early renal transplantation is established under the definite diagnosis which is based on clinicopathology, we can expect long term survival, normal growth and development for the child of CNS.
Child ; Classification ; Cyclosporine ; Denys-Drash Syndrome ; Diagnosis ; Edema ; Female ; Growth and Development ; Humans ; Hypoalbuminemia ; Kidney Transplantation ; Lost to Follow-Up ; Male ; Nephrosis, Lipoid ; Nephrotic Syndrome* ; Parturition ; Penicillins ; Prognosis ; Proteinuria ; Rare Diseases ; Renal Insufficiency ; Retrospective Studies ; Sepsis

PURPOSE: Systemic lupus erythematosus(SLE) is an autoimmune disease with multi-system involvement and renal damage is a major cause of morbidity and mortality in children. Renal involvement is more common and severe in children than in adults. Therefore, renal biopsy plays a crucial role in planning effective therapy. In this study, we investigated the clinical and pathological findings of lupus nephritis in children to aid clinical care of the disease. METHODS: The clinical and pathological data of 40 patients who were diagnosed as SLE with renal involvement in Shinchon Severance Hospital from Jan. 1990 to Sep. 2002 were analyzed retrospectively. RESULTS: The ratio of male to female patients was 1:3 and the median age at diagnosis was 12.1 (2-18) years old. FANA(95.0%), anti-ds DNA antibody (87.5%), malar rash (80.0%) were the most common findings among the classification criteria by ARA. Microscopic hematuria with proteinuria (75.0%), nephrotic syndrome (55.0%), and microscopic hematuria alone (15.0%) were the most common renal presentations in the respective order at diagnosis. There were 27 cases with WHO class IV lupus nephritis confirmed by renal biopsy and 3 cases with pathological changes of WHO class type. Different treatment modalities were carried out : prednisolone only in 5 cases, prednisol-one+azat-hioprine in 9 cases, prednisolone+azathioprine+intravenous cyclophosphamide in 14 cases, prednisolone+cyclosporine A+intravenous cyclophosphamide in 12 cases, plasma exchange in 9 cases and intravenous gamma-globulin in 2 cases. The average follow-up period was 51.8 40.5 months. During 51.8+/-40.5 months. During follow-up, 4 patients expired. The risk factors associated with mortality were male, WHO class IV and acute renal failure at diagnosis. CONCLUSION: Renal involvement was noted in 63.5% of childhood SLE, and 67.5% of renal lesion was WHO class IV lupus nephritis which is known to be associated with a poor prognosis. Therefore aggressive treatment employing immunosuppressant during the early stages of disease could be helpful in improving long-term prognosis. But careful attention should be given to optimize the treatment due to unique problems associated with growth, psychosocial development and gonadal toxicity, especially in children.
Acute Kidney Injury ; Adult ; Autoimmune Diseases ; Biopsy ; Child* ; Classification ; Cyclophosphamide ; Diagnosis ; DNA ; Exanthema ; Female ; Follow-Up Studies ; gamma-Globulins ; Gonads ; Hematuria ; Humans ; Lupus Nephritis* ; Male ; Mortality ; Nephrotic Syndrome ; Plasma Exchange ; Prednisolone ; Prognosis ; Proteinuria ; Retrospective Studies ; Risk Factors ; Treatment Outcome*