Evaluation and Treatment of Children with Primary Immune Deficiency in the Emergency Department

The potential for morbidity and mortality in patients who have PID with febrile and nonfebrile illness is extremely high. Familiarity with the clinical manifestations of PID and collaboration with a pediatric immunologist are prerequisites for optimal short-term care of these complex patients. Conservative management with empiric broad-spectrum antimicrobials, early and aggressive surgical debridement of abscesses, and admission at a tertiary pediatric care center are often indicated.     

Conditions of Alzheimer-type dementia in the old-old and oldest-old patients with special reference to extreme conditions of brain aging

A neuropathological study on 1540 consecutive autopsy brains ranging from 60 to 107 years of age revealed the following points. (1) Of the of the demented cases of the plaque-predominant type, 93% were complicated with multiple tiny cortical infarcts. They showed a tendency for dementia to develop before or after the appearance or worsening of a systemic disorder such as cardiovascular disease, respiratory infection and cancer. However, there was no case showing Alzheimer-type dementia (ATD). (2) The plaque-predominant type might be an extreme condition of brain aging in terms of senile plaques (SP). It is likely that although the pathological appearance of SP alone is not responsible for dementia, its coexistence with multiple cortical infarcts could be the cause of dementia. Therefore, this type should be distinguished from ATD. (3) Primary hippocampal degeneration could also be an extreme condition of brain aging in terms of neurofibrillary tangles. This condition was different pathologically from the hippocampal lesion in ATD. (4) Several characteristics of old-old and oldest-old patients were clarified.     

Open reduction and varus-detorsion osteotomy with femoral shortening in treatment of congenital dislocation of the hip

In this study, we clinically and radiographically evaluated open reduction with shortening of the femur in children more than 1 year old with refractory congenital dislocation of the hip. In 19 children (aged 1–4 years), 22 joints were operated on. The patients were followed-up for an average of 8.7 years (range, 2–13 years). Functional results were satisfactory in all joints, and differences in limb length were not significant. Radiographically, good results (grades I and II) were obtained in 16 of the 22 joints, according to Severin's criteria. This surgical procedure may be indispensable for treating refractory congenital dislocation of the hip in children over 1 year old. Received for publication on May 2, 1997; accepted June 3, 1998     

Secondary hyperparathyroidism and acute tubular necrosis following renal transplantation

In the present study we investigated the relationship betweensecondary hyperparathyroidism in renal graft recipients andpost-transplantation acute tubular necrosis (ATN). Patientswere divided into two groups according to graft function: groupA consisted of 28 patients who had an uneventful postoperativeperiod and did not require haemodialysis. Group B comprised26 patients with primary non-function of the graft due to biopsy-provenATN who required continued haemodialysis for the first postoperativeweek or longer (mean 14.2 ±8.7 days). Both groups hadcomparable donor characteristics, HLA-matching and ischaemiatimes. All patients were given cyclospo-rin and low-dose prednisolonefor immunosuppression. Pretransplant levels of intact PTH weresignificantly greater in group B than in group A (203.5 ±193.1pg/ml versus 81.7±45.2 pg/ml, P<0.01). Group B patientshad more transplant biopsies (50 versus 7) and a longer hospitalizationtime (33.4 ± 10.9 days versus 21.9 ± 11.9 days,P<0.01), although serum creatinine on the day of dischargewas higher in group B (1.77 ± 0.51 mg/dl versus 1.5±0.45mg/dl, P<0.05). We conclude that patients with secondaryhyperparathyroidism as assessed by measuring circulating levelsof intact PTH have an increased incidence of ATN.     

THE CASE AGAINST OPEN NECK BIOPSY

Many patients present with lateral neck lumps due to benign or malignant conditions, and they may be difficult to differentiate clinically. It is detrimental to perform an open neck biopsy on a patient with a cancer originating from the head and neck region (upper aerodigestive tract or skin) prior to definitive treatment. The biopsy interferes with the assessment and management of the neck, increasing morbidity. It may also decrease curability and perhaps induce fungation. A protocol to avoid the need for an open biopsy, using fine needle aspiration cytology and a thorough examination of the upper aerodigestive tract is recommended. The authors also recommend combined radical radiotherapy and surgery for the patient who has had an open biopsy. A thoughtless biopsy is both needless and harmful.     

分级诊疗背景下多病共存患者就医机构选择行为及其影响因素研究

背景 近年来,国内针对多病共存患者在分级诊疗背景下就医机构选择行为的研究较少,研究多病共存患者的就医机构选择行为有重要的现实意义。目的 以分级诊疗为视角,分析多病共存患者就医机构选择行为及其影响因素,为进一步落实分级诊疗制度和合理配置医疗资源提供研究参考。方法 采用随机抽样法,选取2019-06-01至2020-01-01在上海市杨浦区中心医院医疗联合体（上海市杨浦区中心医院和上海市杨浦区定海、延吉、长白社区卫生服务中心）就诊的多病共存患者为研究对象。采用自设问卷对其进行调查,收集多病共存患者的一般资料,了解其对分级诊疗政策的认知情况及实际遵守情况、在不同疾病控制状况下至社区卫生服务中心首诊的意愿,以及其选择就诊医疗机构时考虑的因素等。采用二分类Logistic回归分析多病共存患者此次因病就医是否选择至社区卫生服务中心首诊的影响因素。结果 共发放1 100份问卷,回收有效问卷1 072份,有效问卷回收率为97.45%。1 072例多病共存患者中,老年（≥60岁）多病共存患者占85.07%(912/1 072)。624例（58.21%）患者表示知晓分级诊疗制度;940例（87.69%）患...     

Primary breast sarcoma: A review of 33 cases with immunohistochemistry and prognostic factors

The clinical and pathological features of 33 previously untreated patients with primary breast sarcoma were retrospectively analysed to evaluate the prognostic significance of histologic variables on survival. The series comprised 17 cystosarcomas phyllodes and 16 stromal sarcomas (excluding angiosarcomas).All tumors were reviewed and classified in similar fashion to extramammary soft tissue sarcomas. In addition, immunohistochemical studies were performed on paraffin sections with a panel of several antibodies directed against cytoskeletal filaments and cellular enzymes; five cases were also examined by electron microscopy.Most tumors were malignant fibrous histiocytoma (21 cases) and fibrosarcoma (6 cases) types. Surgery was the main therapy. Metastasis-free survival rate was significantly correlated only with histological grade, consisting of tumor differentiation, tumor necrosis, and mitotic activity. Courses and survivals of the cystosarcoma and stromal groups were identical, questioning the clinical value of this pathologic distinction. All local recurrence, metastasis, or death occurred within 30 months, though follow-up was much longer.Immunohistochemistry was disappointing for identification of specific histologic sub-types.     

Primary cerebral neuroblastoma (neurocytoma) in adults

Summary We report a case of a third ventricular neuroblastoma (neurocytoma) in a 66 year old man. A stereotactic needly biopsy was performed to obtain a tissue diagnosis and was followed by total resection. We elected not to give radiation or chemotherapy and to follow the patient closely with serial CT scans. Presently, 48 months postoperatively, the patient is free of tumor by head CT scan and able to live independently. We reviewed the literature of primary cerebral neuroblastomas/neurocytomas occurring in adults (15 years of age) and found 32 cases. Our patient is the oldest of this group with a mean age of 32 ± 14 years (S.D.). The location of the 33 neoplasms was intraventricular in 17 cases (52%) and intraparenchymal in 16 cases. The male to female ratio was 2: 1. Of the 17 patients having a minimal follow-up period of 5 months (mean 51 months), five developed recurrences after 5 to 144 months (mean 50 months) compared to 12 patients without recurrence after a 6- to 72-month follow-up period (mean 52 months). Recurrences occurred statistically significantly more often in parenchymal neuroblastomas/neurocytomas than in intraventricular tumor locations.     

Alpha-SM actin expression as prognostic indicator in IgA nephropathy (Berger's disease)

Recent studies have demonstrated that α-Smooth Muscle actin expression in glomerular and tubulointerstitial compartments of renal tissue could represent a prognostic marker in several renal diseases. Our objective was to identify the prognostic value of α-SM actin actin expression on the evolution of renal damage in Primary IgA nephropathy (Berger’s Disease). 43 patients followed up from 1988 to 1999 at the University Hospital, Faculty of Medicine of Ribeirão Preto, University of São Paulo, Brazil, was studied. Clinical-laboratory data were obtained from the medical records of the patients using a protocol containing name, race, gender, origin, profession, age at clinical presentation of the disease and personal and family history. The parameters assessed in the approach to IgA nephropathy were serum creatinine, creatinine clearance, serum albumin, total serum protein, 24 hours proteinuria, glycaemia, serum sodium, potassium, calcium and phosphorus ions, analysis of urinary sediment, serum complement profile, blood count, and renal biopsy. Morphological evaluation was performed by renal biopsy using common light and immunofluorescence microscopy. Immunohistochemical studies were performed using a murine monoclonal antibody to α-SM actin. Our data showed that α-SM actin expression in the glomerular and tubulointerstitial compartments are not correlated with unfavorable clinical course of primary IgA nephropathy.     

Comparison of the biomechanical effect of posterior condylar offset and kinematics between posterior cruciate-retaining and posterior-stabilized total knee arthroplasty

Background

The effect of the changes in the femoral posterior condylar offset (PCO) on anterior–posterior (AP) translation and internal–external (IE) rotation in cruciate-retaining (CR) and posterior-stabilized (PS) total knee arthroplasty (TKA) remains unknown. The purpose of this study was to compare the kinematics in CR and PS TKA with respect to the difference in prosthetic design and PCO change through a computational simulation.