Background: X-linked hyper-immunoglobulin M (IgM) syndrome (XHIGM) is a rare immunodeficiency disease caused by mutations of the CD40 ligand gene. Patients are subject to recurrent infections and have normal or elevated levels of IgM but markedly decreased serum IgG. Objective: We describe molecular genetic studies and clinical manifestations in three generations of one family, as well as results of long-term treatment of 2 young men with the disorder. Methods: Of 37 living family members, mutational analysis of the CD40 ligand gene was performed in 36 members. Laboratory data for patients and carriers were reviewed. Results: Four male family members had died of unexplained causes. The 3 patients with XHIGM syndrome and the 5 carriers all had a novel mutation located at Tyr 169 Asn (T526A) in exon 5, the tumor necrosis factor domain of the CD40 ligand gene. In the 3 patients, CD40 ligand expression in activated CD4+ T cells was below 1%. In the carriers, about half of activated CD4+ cells expressed CD40 ligand. One carrier had malignant lymphoma. Long-term (>20 years) intravenous immunoglobulin therapy in 2 patients improved IgG levels but did not fully suppress the high levels of IgM, nor did it prevent late complications (bronchiectasis and sclerosing cholangitis). Conclusions: Diagnosis of a genetic immunodeficiency, especially an X-linked disease such as XHIGM syndrome, should prompt a survey of the entire family.

BackgroundAcupuncture is applied for treating numerous conditions in children, but few studies have examined the safe needling depth of acupoints in the pediatric population. In this study, we investigated the depths to which acupuncture needles can be inserted safely in the upper back acupoints of children and the variations in safe depth according to sex, age, weight, and body mass index (BMI).MethodsWe retrospectively studied computed tomography (CT) images of patients aged 4 to 18 years who underwent chest CT at China Medical University Hospital between December 2004 and May 2013. The safe depths of 23 upper back acupoints in the Governor Vessel (GV), Bladder Meridian (BL), Small Intestine Meridian (SI), Gallbladder Meridian (GB) and Spleen Meridian (SP) were measured directly from the CT images. The relationships between the safe depths of these acupoints and sex, age, body weight, and BMI were analyzed.ResultsThe results indicated significant differences in safe needling depth between boys and girls in most upper back acupoints, except at BL42, BL44, BL45, BL46, GB21 and SP21. Safe depths differed significantly depending on age (p < 0.001), weight (p ≤ 0.01), and BMI (p < 0.05). Multiple regression analysis revealed that weight was the most crucial factor in determining the safe depth.ConclusionsSex, age, weight, and BMI are relevant factors in determining the safe needling depths of upper back acupoints in children. Physicians should pay attention to wide variations in needle depth when performing acupuncture.

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Background. Acupuncture is applied to treat numerous diseases in pediatric patients. Few reports have been published on the depth to which it is safe to insert needle acupoints in pediatric patients. We evaluated the depths to which acupuncture needles can be inserted safely in chest acupoints in pediatric patients and the variations in safe depth according to sex, age, body weight, and body mass index (BMI). Methods. We retrospectively studied computed tomography (CT) images of pediatric patients aged 4 to 18 years who had undergone chest CT at China Medical University Hospital from December 2004 to May 2013. The safe depth of chest acupoints was directly measured from the CT images. The relationships between the safe depth of these acupoints and sex, age, body weight, and BMI were analyzed. Results. The results demonstrated significant differences in depth among boys and girls at KI25 (kidney meridian), ST16 (stomach meridian), ST18, SP17 (spleen meridian), SP19, SP20, PC1 (pericardium meridian), LU2 (lung meridian), and GB22 (gallbladder meridian). Safe depth significantly differed among the age groups (P < 0.001), weight groups (P < 0.05), and BMI groups (P < 0.05). Conclusion. Physicians should focus on large variations in needle depth during acupuncture for achieving optimal therapeutic effect and preventing complications.

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