Effect of preoperative stabilization on respiratory system compliance and outcome in newborn infants with congenital diaphragmatic hernia

To determine whether preoperative stabilization and delay of operative repair of congenital diaphragmatic hernia (CDH) may decrease operative risk, we performed serial pulmonary function tests on 22 newborn infants with CDH and on four infants without pulmonary hypoplasia (two with ileal atresia and two with tracheoesophageal anomalies) who served as control subjects. We used 2 passive respiratory mechanics technique to measure respiratory system compliance. All patients with CDH had respiratory distress immediately after birth, and required mechanical ventilation. Thirteen babies underwent emergency repair (six survived, seven died); nine of them received extracorporeal membrane oxygenation (ECMO) after the operation (two survived, seven died). Operative repair was delayed deliberately for 2 to 11 days in nine infants with severe hypoxemia. Six immediately received ECMO for 4 to 10 days; one died of intraventricular hemorrhage, and five survived and later underwent surgical repair. The seventh patient did not receive ECMO but appeared to have respiratory distress syndrome of infancy and improved after administration of synthetic surfactant. Improvement was seen in two additional infants who received conventional assisted ventilation during a 48-hour delay before surgery, and survived. In all, eight of nine infants who underwent preoperative stabilization survived (p less than 0.05 compared with survival after emergency surgery). Following surgical repair immediately after birth, respiratory system compliance improved only slightly during the first week of life, a time when control infants had a rapid increase in respiratory system compliance (p less than 0.001). In contrast, respiratory system compliance increased nearly twofold in the nine patients undergoing preoperative stabilization (p less than 0.02). Preoperative ECMO was associated with an increase in respiratory system compliance of more than 60% for 1 week, a significant difference from respiratory system compliance among patients undergoing emergency CDH repair (p less than 0.05). These observations provide physiologic evidence of possible benefits of preoperative stabilization before repair of CDH.     

Adjuvant six cycles of high-dose adriamycin,cyclophosphamide, methotrexate, 5-fluorouracil (ACMF) vs. 12 cycles of low-dose acmf with tamoxifen for premenopausal,node-positive breast cancer patients: Results of a prospective randomized study

A prospective randomized study was conducted to compare the adjuvant efficacy of six cycles of high-dose ACMF (Adriamycin, ADM; cyclophosphamide, CPA; methotrexate, MTX; 5-fluorouracil, 5-FU) with that of 12 cycles of low-dose ACMF in premenopausal, node-positive breast cancer patients. The six-cycle ACMF group (93 patients) received, intravenously (iv), 130 mg/m² CPA, 26 mg/m² MTX, and 600 mg/m² 5-FU on days 1 and 8, and 26 mg/m² ADM on day 1 of each cycle. The 12-cycle ACMF group (97 patients) received, iv, 65 mg/m² CPA, 13 mg/m² MTX, and 300 mg/m² 5-FU on days 1 and 8, and 13 mg/m² ADM on day 1 of each cycle. These treatments were repeated every 4 weeks, and all the patients took tamoxifen (30 mg/day) for 2 years. The background factors of the two groups were comparable. There were non-significant trends toward better disease-free and overall survival rates in the high-dose, six-cycle ACMF group. Both treatments were well tolerated, but more patients in the low-dose, 12-cycle group refused to continue to receive chemotherapy. These data suggest that escalating doses of ACMF over a shorter period, even with doses within the conventional range, are superior to low-dose, prolonged therapy. © 1995 Wiley-Liss, Inc.     

Pontine glioblastoma multiforme initially presenting with leptomeningeal gliomatosis

A 49-year-old female presented with diffuse leptomeningeal gliomatosis as the initial manifestation of pontine glioblastoma. Magnetic resonance imaging initially revealed diffuse leptomeningeal enhancement caused by metastatic deposits, predominantly along the basal cistern and bilateral sylvian fissures. The primary pontine lesion appeared as hypointense on T1-weighted imaging and hyperintense on T2-weighted imaging, but with no enhancement by gadolinium-diethylenetriaminepenta-acetic acid. There was no diffuse enlargement of the pons. The patient died 11 months after the initial presentation. The primary lesion in the pons was histologically confirmed at autopsy. Diffuse enhancement of leptomeningeal dissemination may occur as the initial manifestation of non-enhanced pontine glioblastoma.     

Nodular mucinosis of the breast: A case report with clinical and imaging findings

We present a very rare case of nodular mucinosis of the breast. A 30-year-old woman noticed a right breast lump and consulted at our hospital because it gradually increased in size. On physical examination, the lump was 30 x 25 mm in size, and was located in the upper outer quadrant close to the nipple of the right breast. It was well-demarcated, mobile and hard. Ultrasonography (US) showed a clearly circumscribed, lobulated, and homogeneous hypoechoic lesion. Mammography (MMG) showed a round-lobular-shaped radiopaque mass without microcalcifications or spicula formation. Fine needle aspiration cytology (FNA) revealed no malignancy and mucin. Histologically, the excised tumor consisted of an abundant myxoid substance with scattered spindle cells without epithelial elements in the mucous lake. The mucinous substance stained positively with Alcian blue. Nodular mucinosis, simulating mucinous carcinoma or phyllodes tumor on clinical and imaging examinations, should be included in the differential diagnosis in cases of mucinous lesions occurring near the nipple in a young woman.     

Prognostic and predictive factors in the adjuvant treatment of breast cancer

The selection of systemic adjuvant therapy should be based on the appropriate prognostic and predictive factors. The established prognostic factors currently used in cases of primary breast cancer include axillary lymph node involvement, histologic subtype, tumor size, nuclear or histologic grade, estrogen (ER) and progesterone receptor (PR) status and proliferative index. Adjuvant chemotherapy has had an impact on the management of node-positive breast cancer, while the St. Gallen recommendations were established for postoperative adjuvant therapy for node-negative breast cancer. However, there is some contention regarding the histological (or nuclear) grading systems among different pathologists. With regard to biological measurements, the most useful prognostic/predictive factors are hormone receptor status and HER-2 overexpression. ER and PR status can be used to establish the necessity of hormone therapy in the adjuvant setting. If the anti-HER-2 antibody and/or antiangiogenic agents are introduced into the adjuvant setting in the near future, determination of these factors is also recommended.     

Hypermethylation of the hMLH1 gene promoter in solitary and multiple gastric cancers with microsatellite instability

Human cancers with a high frequency microsatellite instability phenotype develop due to defects in DNA mismatch repair genes. Silencing of a DNA mismatch repair gene, hMLH1 gene, by promoter hypermethylation is a frequent cause of the microsatellite instability-H phenotype. Using methylation specific PCR we investigated the methylation status of the hMLH1 gene promoter in 17 solitary gastric cancers (12 microsatellite instability-H and five microsatellite stable tumours from 17 patients), and 13 multiple gastric cancers (eight microsatellite instability-H, one low frequency microsatellite instability-L and four microsatellite stable tumours from five patients) and also examined non-cancerous gastric mucosa both adjacent to and distant from each tumour. Expression of hMLH1 protein was evaluated by immunohistochemistry. All microsatellite instability-H tumours (20 out of 20) had evidence of methylation of hMLH1 promoter, whereas only one out of 10 microsatellite instability-L and microsatellite stable tumours did (P<0.0000005), and the methylation status correlated with hMLH1 protein expression (P<0.000003). Furthermore, methylation of the hMLH1 promoter was detected in 50% (6 out of 12) and 63% (5 out of 8) of non-cancerous gastric mucosa samples adjacent to, and in 33% (4 out of 12) and 40% (2 out of 5) of those obtained from distant portion of, solitary and multiple cancers with microsatellite instability-H. Thus both solitary and multiple gastric cancers with microsatellite instability-H have evidence of similar high levels of hMLH1 promoter hypermethylation in the surrounding non-cancerous tissue. Hypermethylation of the hMLH1 promoter occurs in non-cancerous gastric mucosa of microsatellite instability-H tumours and may increase the risk of subsequent neoplasia.     

Cephalopod tropomyosins: Identification as major allergens and molecular cloning

Heated extracts prepared from the mantle muscles (for decapods) or leg muscles (for octapods) of nine species of cephalopods were shown to be all reactive with serum IgE in crustacean-allergic patients. No marked difference in the reactivity with IgE was recognized among the cephalopods, suggesting that they are almost equally allergenic. Immunoblotting and inhibition immunoblotting data revealed that the major allergen is tropomyosin in common with the nine species of cephalopods and that the cephalopod tropomyosins are cross-reactive with one another and also with crustacean tropomyosins. Molecular cloning experiments first elucidated the primary structures of tropomyosins from five species of cephalopods. The cephalopod tropomyosins show high sequence identity (more than 92% identity) with one another, being the molecular basis for their cross-reactivity. Although the sequence identity between cephalopod and crustacean topomyosins is only about 63–64%, some of the IgE-binding epitopes proposed for brown shrimp Penaeus aztecus tropomyosin (Pen a 1) are well conserved in the cephalopod tropomyosins, supporting the cross-reactivity between cephalopod and crustacean tropomyosins.     

Henoch-Schönlein purpura with hypocomplementemia in children

BACKGROUND: The clinical course and prognosis of Henoch-Schonlein purpura (HSP) associated with hypocomplementemia are not clear. METHODS: The clinical findings of 10 children with HSP and hypocomplementemia were studied. RESULTS: Purpuric rash in all patients, abdominal pain in five, and arthralgia in nine were noted. The findings in HSP were not different from others with HSP. In eight patients, infection preceded hypocomplementemia. Serum levels of CH50, C3 or C4 were depressed variously. Complement levels returned to normal within 5 weeks in all patients. Antistreptolysin-O (ASO) titer was elevated in all patients and nephritis occurred in eight patients. Six patients had generalized edema and hypertension. Macroscopic hematuria occurred in two patients and heavy proteinuria in five patients. One patient was diagnosed as having poststreptococcal acute glomerulonephritis (PSAGN) combined with HSP nephritis according to renal biopsy findings. In three of eight patients with nephritis, abnormal urinary findings continued for more than 1 year. CONCLUSIONS: Hypocomplementemia in children with HSP was transient and was not related to severity of HSP. Incidences of elevated ASO titer and nephritis were high. The nephritis resembled PSAGN during the acute stage and long-term clinical courses varied. These findings suggest PSAGN may be associated with HSP nephritis.     

Familial cases of Henoch-Schönlein purpura in eight families

Abstract Background : Familial cases of Henoch‐Schönlein purpura (HSP) have rarely been reported. Methods : Familial cases of HSP were reviewed by medical records of 418 children with HSP. Results : Two members developed HSP in eight families. HSP occurred in a mother and her daughter in one family and in siblings, including one pair of twin sisters, in seven other families. Four pairs of patients developed HSP at the same age. Three pairs presented HSP within 1 month of each other and the other pairs presented HSP between 9 months and 5 years. Seven patients had a history of allergic diseases. The clinical courses of 12 patients were reviewed. Upper respiratory tract infection preceded HSP in 10 patients, two of whom had elevated antistreptolysin‐O titers. No pairs of patients in a family received the same drugs before the onset of HSP. Abdominal pain was noted in eight patients, arthralgia in six and nephritis in four. Severity of skin lesions, presence of abdominal pain and nephritis, and serum IgA levels at the acute stage varied among family members of HSP. Conclusions : The incidence of HSP in family members of children with HSP seems to be high. Onset at the same age and onset of HSP within 1 month in siblings have not previously been reported. There were no characteristic or similar findings between two patients of the same family. No trigger or genetic factor causing HSP was identified.