Humans ; Immunoglobulin A ; blood ; Immunoglobulin Light Chains ; blood ; Leukemia ; blood ; Multiple Myeloma ; blood

Objective:To investigate the clinical characteristics of patients with lymphoplasmacytic lymphoma/Waldenstr?m macroglobulinemia (LPL/WM), and the diagnosis and optimal treatment of LPL/WM.Methods:The clinical data of 13 LPL/WM patients treated in Zhongnan Hospital of Wuhan University from January 2013 to June 2018 were retrospectively analyzed, and the literature was reviewed.Results:The median age of 13 patients was 60 years old (35-79 years old). There were 12 males and 1 female. Initial symptom was fatigue or edema of both lower limbs for majority of patients. All patients had immunoglobulin M (IgM) monoclonal, 3 of them had elevated immunoglobulin G (IgG) level, including 1 patient with monoclonal IgG. LDH was increased in 2 patients. Coombs test was positive in 5 patients. MyD88 gene mutation status was detected in 8 patients, of which gene mutation in 5 patients was positive. Among 13 patients, 1 patient lost follow-up, 3 patients died, 9 patients were alive with the median survival of 36 months (19-81 months).Conclusions:Incidence of LPL/WM is relatively low with a generally indolent evolution, but heterogeneity is not negligible. Few patients have poor treatment response with a quick disease progress. The high-risk patients undergoing hematopoietic stem cell transplantation after remission-induction chemotherapy may improve the prognosis.

BRCA2 Protein ; Humans ; Leukemia, Myeloid, Acute

【Objective】 To investigate the influencing factors behind the follow-up compliance of patients with low/no phenylketonuria (PKU) for special medical use, in order to provide a basis for regulating the follow-up of PKU patients and ensuring the effectiveness of special diet treatment. 【Methods】 A survey was conducted on PKU patients treated in Urumqi Maternal and Child Health Hospital for over 1 year, from January 2010 to December 2020. Interviews and questionnaires were conducted with their caregivers to collect and analyze the current status of PKU patients undergoing special diet treatment, and to identify the influencing factors behind their compliance with follow-up treatment. 【Results】 Patients who had received neonatal disease screening, neonatal gene diagnosis, and maternal Down′s screening during pregnancy had better compliance, with statistically significant differences (χ²=5.753, 10.993, 9.189, P<0.05). PKU children with parents who had a college education or above showed significantly higher adherence to special diet treatment (χ²=8.321, 7.415, P<0.05). PKU children with parents having a fixed occupation also showed higher compliance, with a statistically significant difference (χ²=20.626, 7.895, P<0.05). Patient age, interval of buying special diet, number of blood samples sent and enrollment of normal age, all had a significant impact on the follow-up compliance of PKU patients with special diet (χ²=19.443, 8.090, 69.482, 12.001, P<0.05). 【Conclusions】 PKU is a treatable genetic metabolic disease. Strengthening health education, formulating standardized follow-up plans and procedures, and improving follow-up treatment compliance are crucial in enhancing the treatment and follow-up effectiveness of PKU patients.

OBJECTIVETo explore the relationship between gastric retention and full enteral feeding during the course of feeding in extremely low birth weight (ELBW, birth weight <1 000 g) infants.

METHODA total of 43 ELBW infants were fed with formula according to the strategy for premature infants feeding of Canadian Society of Neonatology. The information such as gastric retention, the time they finish full enteral feeding and sucking spontaneously and complication were recorded. These infants had transition to full enteral feeding step by step since initiating formula feeding on the second day of life. The volume of gastric retention and the duration of gastric retention was analyzed with the time of attaining full enteral feeding and sucking spontaneously by linear regression.

RESULTForty-one infants finished the course, the remaining 2 infants got necrotizing enterocolitis (NEC) and were rescued by surgery. The incidence was 4.6%. In 18 infants full enteral feeding could not be initiated successfully on the second day of life, the incidence was 43.9%. The peak duration of gastric retention was the first week of feeding. The average time of attaining enteral feeding was (26.71 ± 12.24) days. The proportion of different residual contents was simlar, the major content was milky content after 3 weeks of feeding. The gastric retention time had a significant effect of on the time of attaining full enteral feeding (β = 1.045, P = 0.001) and sucking well (β = 0.787, P = 0.034) .

CONCLUSIONThe course of formula feeding ELBW infants to attaining full enteral feeding was a long period, in the early stage of formula feeding the occurrence of gastric retention was high; the amount of formula during the first week of feeding should be slowly increased as compared to the second week; the present strategy and aggressive strategy should be done 2 weeks later. The time of attaining full enteral feeding can be predicted by the duration of gastric retention.

Enteral Nutrition ; methods ; Enterocolitis, Necrotizing ; epidemiology ; Female ; Gestational Age ; Humans ; Infant ; Infant Formula ; Infant, Extremely Low Birth Weight ; growth & development ; Infant, Newborn ; Infant, Premature ; Infant, Premature, Diseases ; epidemiology ; Length of Stay ; Male ; Parenteral Nutrition ; Time Factors ; Weight Gain