Clinical analysis of 24 cases of pediatric acute lymphoblastic leukemia with renal involvement as the initial manifestation / 中华实用儿科临床杂志

Objective:To investigate the clinical features and long-term prognosis of pediatric acute lymphoblastic leukemia (ALL) with renal involvement as the initial manifestation, thus enhancing the diagnostic and therapeutic efficacy.Methods:Twenty-four cases of pediatric ALL with renal involvement as the initial manifestation treated in the First Affiliated Hospital of Zhengzhou University from March 2013 to March 2019 were analyzed retrospectively, and their clinical characteristics were analyzed.According to renal imaging examination findings, they were divided into abnormal group and normal group.The differences in clinical features between the two groups were compared, and the cumulative survival rate was evaluated by Kaplan-Meier method.Results:Among 1 030 newly treated cases of pediatric ALL, 24 cases(2.33%) had renal involvement as the initial manifestation, involving 20 males and 4 females, with a male/female ratio of 5∶1 and the median age of 4.3 years (1.3-14.0 years). There were 16 cases of superficial lymph node enlargement and 21 cases of hepatosplenomegaly.Immature cells in peripheral blood were found in 15 cases.Nine cases were examined with abnormal renal imaging, involving 8 cases returned normal after chemotherapy, and 1 died of renal failure.At the end of follow-up on August 1, 2020, there were 9 cases of bone marrow relapse, 11 survival cases, 10 death cases and 3 cases of loss to follow-up.There were no significant differences in the sex, age, immunophenotype, organ infiltration and urinary protein between the two groups (all P>0.05). The proportion of high creatinine level and intramedullary recurrence rate in the abnormal group were significantly higher than those in the normal group [55.6%(5/9 cases) vs.0(0/15 cases), P=0.003; 66.7%(6/9 cases) vs.20.0%(3/15 cases), P=0.036]. The survival analysis indicated that the 3-year cumulative survival in the abnormal group was significantly lower than that of normal group (17.3% vs.72.7%, χ2=4.047, P< 0.05). Conclusions:For children with unexplained renal involvement as the initial manifestation, clinicians should consider the possibility of leukemic renal infiltration or nephrogenic lymphoma.Physical examinations of the liver, spleen and lymph nodes, morphological analysis of peripheral blood cells, bone marrow examination and renal biopsy are important to make a definite diagnosis in time.Children with imaging abnormalities caused by leukemic renal infiltration are more likely to relapse and have a lower survival rate, which may be a poor prognostic factor for ALL.

Spine Metastases as the Initial Sign of Differentiated Thyroid Carcinoma: Two Case Reports / 대한내분비외과학회지

Bone metastases as the first manifestation of thyroid carcinoma are extremely rare. Interestingly, evaluation at appropriate initials and proper treatment will lead to satisfactory long-term survival. We report here on two such cases; the patients presented with back pain and fine needle aspiration cytology of spine lesion revealed a metastatic carcinoma. A wide excision of the bone lesion was carried out and the histopathology was consistent with features of metastatic carcinoma of the thyroid. The management of thyroid carcinoma and the subsequent bone metastases is reviewed and the controversial points are highlighted.

H+ -ATPase Pump Defects of Distal Renal Tubular Acidosis as Initial Manifestation of Systemic Lupus Erythematosus / 대한신장학회잡지

Systemic lupus erythematosus (SLE) is a multisystem disease with marked variability in its manifestation. Tubulointerstitial involvement is well recognized in SLE. But usually the tubular dysfunction is latent and usually presents after diagnosis of SLE. We report a 20 years old female whose initial symptom of SLE was distal renal tubular acidosis (RTA). She presented with severe muscle weakness at emergency room with laboratory fingding consistent with distal RTA. After several months she developed fever, arthritis, serologic fingding which was compatible to diagnose SLE. We report a case whose initial symptom of SLE had been distal RTA.

The Analysis of Primary Origin in Spinal Metastasis Occurring as the Initial Manifestation of Malignancy

OBJECTIVE: The objective of this study is to delineate clinical features and specific diagnostic and therapeutic implications of spinal metastasis occurring as the initial manifestation of malignancy(SM-IMM)-a less common event than spinal metastasis in the setting of previously established malignancy(SM-PEM). METHODS: The authors reviewed retrospectively the clinical records of 19 patients in SM-IMM group at Korean Cancer Center Hospital between January 1, 2000 and December 31, 2001 and analyzed symptom duration, primary origin, treatment modality and survival time. Then 287 cases of SM-PEM group were searched for primary origin. RESULTS: Thyroid cancer(36.8%), metastasis of unknown origin(31%), liver cancer(10.5%) and stomach cancer (10.5%) were found as primary tumor in SM-IMM group. But in SM-PEM group lung cancer was most frequent primary tumor(22%), breast cancer(17%), stomach cancer(7.3%) and liver cancer (7.3%) followed. Primary pathology was confirmed with spinal decompressive surgery(7 cases), biopsy of spinal lesion(5 cases), and biopsy of other sites except spine(7 cases). Patients in SM-IMM group showed short symptom duration and multi-segmental involvement at diagnosis. And post-treatment survival time was short except thyroid cancer in spite of aggressive treatment(mean survival time, 2.7 months). CONCLUSION: SM-IMM group showed different profile from SM-PEM group on primary origin. And in the diagnosis of primary origin in SM-IMM group, important clues were provided with history taking, physical examination and PET.

Adult-onset Moyamoya Disease: Clinical Features and Prognosis

BACKGROUND: The age distribution of patients with moyamoya disease (MMD) forms two characteristic peaks in children and adults. It is well known that hemorrhagic presentation is more frequent in adults, while hemorrhage is rare in children. There is controversy in the management of adult-onset MMD and its natural course is not well defined. We investigated the clinical features and outcomes of adult onset MMD to clarify its characteristics. METHODS: Fifty patients with MMD whose first manifestation appeared later than 15 years of age were studied retrospectively. The first manifestations were divided into ischemic and hemorrhagic categories. The types of reattack and their frequencies according to treatment type, as well as the differences between probable (unilateral) and definite (bilateral) MMD, and current clinical outcomes by the Modified Rankin Scale were investigated. RESULTS: The mean follow-up duration was 24.4+/-25.9 months. The female/male ratio was 1.5. The most frequent age of onset was in the 4th decade. Ischemia was more frequent (58%) than hemorrhage (40%) at the initial presentation. Reattack occurred in 20 (41%) patients and more frequently in the ischemic type (55%) than the hemorrhagic (20%). Patients with probable MMD had a later onset age (39 vs. 31.5 years old) and more frequent reattacks (50%) than patients with definite MMD (38%). Reattack occurred in 2 patients (9.5%) among 21 patients who received surgical revascularization during mean follow-up of 15.1+/-21.7 months. One patient had ischemic reattack and the other had hemorrhagic reattack after operation. Thirty five (71%) of 50 patients had good outcomes but 3 patients with hemorrhagic reattacks had poor outcomes (6%). CONCLUSIONS: Probable and definite MMD have some differences in clinical features such as onset age and reattack. Regardless of initial manifestations, most of the adult MMD patients had good outcomes. However, patients with recur-rent attacks had worse outcomes than those without reattacks, especially of the hemorrhagic type. Therefore, revention of rebleeding should receive more attention in the management of adult MMD.