Higher prevalence of vitamin D deficiency in mothers of rachitic than nonrachitic children

Vitamin D deficiency [serum 25-hydroxyvitamin D <25 nmol/L (<10 ng/mL)] was identified in 92% of rachitic Arab children and 97% of their mothers compared with 22% of nonrachitic children and 52% of their mothers. There was a positive correlation between maternal and child vitamin D levels. We conclude that mothers of rachitic children should be investigated and treated for vitamin D deficiency.     

Cholelithiasis in children with sickle cell disease

Abdominal ultrasonography was performed on 305 children with sickle cell disease (SCD) (285 SS and 20 S-beta-thalassemia) to establish the prevalence of cholelithiasis in Saudi children with SCD. Their ages ranged from 1 to 18 years (mean 10.45 years). Gallstones were demonstrated in 60 children, giving a prevalence of 19.7%. An additional 50 patients (16.4%) had only biliary sludge. The youngest patient with gallstones was 3 years old. There was a correlation between the presence of gallstones and increasing age. Patients with gallstones were also found to have higher serum bilirubin levels, but their hemoglobin, hematocrit, reticulocyte count, hemoglobin S, and hemoglobin F levels were not significantly different from those of patients without gallstones.     

Early response to vitamin D2 in children with calcium deficiency rickets

OBJECTIVE: To assess the effect of vitamin D(2) administration on serum vitamin D metabolite concentrations in calcium deficiency rickets. STUDY DESIGN: We administered vitamin D(2), 50,000 IU orally to 16 Nigerian children 15 to 48 months of age with radiographically active rickets. We measured calcium and vitamin D metabolites at baseline and at 1, 3, 7, and 14 days. RESULTS: At baseline, ranges of serum 25-hydroxyvitamin D (25(OH)D) concentrations were 18 to 40 nmol/L (7-16 ng/mL), and 1,25-dihydroxyvitamin D (1,25-(OH)(2)D) concentrations were 290 to 790 pmol/L (120-330 pg/mL). After vitamin D administration, serum 25(OH)D and 1,25(OH)(2)D concentrations rapidly rose and peaked at 2.8 and 1.9 times the baseline values (P < .001), respectively, at 3 days. Positive correlations between 1,25(OH)(2)D and 25(OH)D were strongest at day 3 (r = 0.84, P < .001) and weakest at day 14 (r = 0.41, P = .11). The relationship of 1,25(OH)(2)D with 25(OH)D at baseline and the increase in 1,25(OH)(2)D in response to vitamin D were similar to those described in children with vitamin D deficiency. However, unlike the pattern in vitamin D deficiency, 1,25(OH)(2)D remained positively correlated with 25(OH)D after administration of vitamin D. CONCLUSION: Dietary calcium deficiency increases the demand for 25(OH)D above that required in vitamin D deficiency to optimize 1,25(OH)(2)D concentrations. Assessment of vitamin D sufficiency in persons or communities may need to be adjusted for habitual dietary calcium intake.     

新生儿维生素D营养状况调查

目的通过测定新生儿血清25-羟维生素D[25（OH）D]、甲状旁腺激素（PTH）、钙、磷浓度,了解兰州市新生儿维生素D营养状况,指导临床补充维生素D。方法选取2012年9～12月在兰州大学第一医院住院的90例新生儿为研究对象,早产儿30例,足月儿60例,采用酶联免疫法检测两组患儿生后10天内血清25（OH）D、PTH浓度,并分析两者之间的相关性;血清标本的钙、磷水平由贝克曼AU2700全自动生化分析仪测定。结果早产儿血清25（OH）D浓度为（21．9±2．5）nmol／L,显著低于足月儿（34．9±10．8）nmol／L,差异有统计学意义（P〈0．05）;早产儿血清町H浓度为（94．7±40．2）pg／ml,显著高于足月儿（56．1±30．0）pg／ml,差异有统计学意义（P〈0．05）,新生儿血清25（oH）D水平与明H呈负相关;旱产儿血钙浓度为（2．1±0．3）mmob／L,低于足月儿（2．3±0．3）mmol／L,差异有统计学意义（P〈0．05）;早产儿血磷浓度为（1．8±0．7）mmoL／L,足月儿为（1．6±0．5）mmol／L,差异无统计学意义（P〉0．05）。结论兰州市新生儿普遍存在维生素D缺乏或不足的现象,应加强孕母和新生儿的维生素D补充。     

Vitamin A status, hospitalizations, and other outcomes in young children with sickle cell disease

OBJECTIVE: To determine the relation of serum vitamin A status to growth, nutritional and hematologic status, and to the number of hospitalizations in children with sickle cell disease-SS (homozygous for the S allele, SCD-SS). STUDY DESIGN: Children (2-9.9 years of age) with SCD-SS were assessed for serum retinol, hemoglobin, hematocrit, reticulocyte count, height, weight, body mass index, and recalled dietary intake. Vitamin A status was defined on the basis of serum retinol concentration as suboptimal (<30 microg/dL) and normal (> or =30 microg/dL). Hospitalizations were determined for 1 year after vitamin A assessment. RESULTS: Mean serum retinol was 26.7 +/- 6.8 microg/dL in 66 subjects (39 girls) and was suboptimal in 66% of children. Compared with those with normal status, children with suboptimal vitamin A had significantly lower body mass index z score (-0.7 +/- 1.0 vs -0.1 +/- 0.6) and hemoglobin (7.9 +/- 1.1 vs 8.5 +/- 1.1), and hematocrit (23.3 +/- 3.0 vs 25.1 +/- 3.8) and significantly more hospitalizations (2.8 +/- 2.0 vs 0.7 +/- 0.8). After adjusting for age and sex, suboptimal vitamin A status was associated with a 10-fold increased risk for hospitalization (OR, 10.5; 95% CI, 2.3, 48.6) and with increased pain (OR,5.3; 95% CI, 1.3, 21.6) and fever episodes (OR, 6.4; 95% CI, 1.7, 24.9) requiring hospitalization. CONCLUSIONS: Suboptimal vitamin A status was prevalent in US children with SCD-SS and was associated with increased hospitalizations and poor growth and hematologic status.     

Acute chest syndrome in children with sickle cell disease

Acute chest syndrome (ACS) is an acute pulmonic process in patients with sickle cell disease. We prospectively studied 50 patients with ACS admitted to the Pediatric Medical Ward during one year period (Jan. 1993 through Dec: 1993). Twenty eight of them were males and twenty two were females giving a male: female ratio of 1.2: 1. The age ranged between one and 12 years. Twelve (24%) of the patients had chest pain on presentation. Twenty seven (54%) patients had significant temperature (>38°C). The x-ray findings showed that the right lung was involved in 30 patients, the left in 10 patients and both lungs in 10 patlents. Three patients had pleural effusion that required chest tube insertion. Laboratory profiles showed that the erythrocyte sedimentation rate ranged between 15 and 90mm/h, and their hemoglobin ranged between 4.2 gm and 12 gm/dl. Seven (14%) patients had significantly positive mycoplasma pneumoniae titer. None of the blood cultures was positive. All of our patient received antibiotic, usually either Cefuroxime or Ceftriaxone with Erythromycin in addition to other supportive measures such as blood transfusion, oxygen therapy and hydration therapy.     

Pneumonia in young children with homozygous sickle cell disease: risk and clinical features

The incidence and clinical features of pneumonia have been examined in children with homozygous sickle cell (SS) disease and in age/sex matched control children with a normal haemoglobin (AA) genotype followed in a cohort study of sickle cell disease from birth.Survival curve analysis indicated a similar incidence of pneumonia in the two genotypes up to the ages of 8 months after which pneumonia became significantly more prevalent in SS disease, the relative risk exceeding a factor of four by 4 years of age. Children with SS disease were also more prone to multiple episodes. Comparison of clinical features in the two genotypes yielded no difference in sex or seasonal involvement, or in the results of bacteriological and radiological investigations. Children with SS disease and pneumonia had an increased frequency and increased duration of hospital admission, and mortality was confined to this group.It is concluded that children with SS disease have an increased prevalence of single and multiple attacks of pneumonia and that these events run a more serous clinical course than in control children.Abbreviations SS sickle cell disease - AA normal haemoglobin genotype     

Splenectomy in children with sickle cell disease and thalassemia

A number of Saudi children (31) with sickle cell disease and thalassemia underwent splenectomy : 12 for frequent blood transfusions, 15 for chronic hypersplenism (most of whom were also the recipients of periodic blood transfusion) and 4 for splenic abscess. The mean age of splenectomy was 8.8 years (8 months 3–18 years). Eight patients had sickle cell disease, 14 betathalassemia and 9 had sickle cell thalassemia. All patients received prophylaxis against pneumococcal infection. There was one postoperative death most probably due to sepsis. Sixteen of those who required frequent preoperative blood transfusions needed no more transfusions, while in 7 the need for transfusions decreased significantly (p < 0.05). For those with hypersplenism, there was a significant postoperative increase in total hemoglobin (P < 0.001), RBC (P < 0.001) and platelet counts (p < 0.02); and a substantial decrease in reticulocyte counts (p < 0.05). The common post splenectomy complications were chest infection and a brief episode of pyrexia, but without undue morbidity. The study establishes a definite place for splenectomy in a selected population of children with sickle cell disease and thalassemia. Presented at the 9th Congress of the Asian Association of Pediatric Surgeons, April 6–10, 1988, Singapore.     

The significance of biliary sludge in children with sickle cell disease

The prevalence of cholelithiasis was studied prospectively by abdominal ultrasound (US) examination in 305 children with sickle cell disease aged 1–18 years (mean 10.45). Gallstones were present in 60 children (19.7%); an additional 50 had biliary sludge only (16.4%). On follow-up of 35 of the 50 children with sludge, 23 (65.7%) had developed gallstones and 5 had already had a cholecystectomy. Five continued to have sludge on follow-up while 7 were reported to have no sludge. Children with US evidence of sludge should be followed up regularly by US, and those who develop gallstones should undergo elective cholecystectomy. For those with biliary sludge only, we recommend elective cholecystectomy if there are hepatobiliary symptoms. Accepted: 16 December 1996     

Laparoscopic splenectomy and/or cholecystectomy for children with sickle cell disease

Background  In 1991, Delaitre reported the first laparoscopic splenectomy (LS). Since then LS has become the procedure of choice to treat hematological diseases requiring splenectomy. The Eastern province of Saudi Arabia is known to have a high incidence of hemoglobinopathies including sickle cell disease (SCD), which is known to be associated with complications necessitating splenectomy and/or cholecystectomy. This report describes our experience with LS and/or laparoscopic cholecystectomy (LC) for children with SCD. Patients and methods  The medical records of all children with SCD who had LS and/or LC were retrospectively reviewed for age, sex, indication for splenectomy, operative time, hospital stay, and post-operative complications. The results were compared to a similar group of children with SCD who had open splenectomy (OS) and/or open cholecystectomy (OC). Results  Over a period of 3.5 years (January 2005 and June 2008), a total of 45 children had LS with or without LC, 30 (66.7%) of them had SCD. Their age ranged from 2 to 12 years (mean 7 years). There were 16 males and 14 females. In all, LS was done because of recurrent splenic sequestration crisis except one who had a large spleen with multiple infarcts that was causing abdominal pain. The operative time ranged from 1.5 to 9 h (mean 2.75 h). Their hospital stay ranged from 3 to 9 days (mean 4.5 days). There was no mortality. Two patients (6.7%) required conversion to OS due to a large-sized spleen and severe adhesions in one and uncontrolled intra-operative bleeding in the other. The results were compared to a group of 120 children with SCD who had OS only (88) and OS with OC (32). From 1994 to 2006, a total of 55 children had LC only, 47 (26 M:21 F) of them (85.5%) had SCD. Their age ranged from 4 to 15 years (mean 11.4 years). The indications for cholecystectomy were: biliary dyspepsia (20), biliary colic (35), acute cholecystitis (5), obstructive jaundice (5), asymptomatic (6), and biliary pancreatitis (1). There was no mortality, but one (2.1%) required conversion to OC because of severe adhesions and another underwent postoperative exploration because of bleeding from an accessory cystic artery. The results were compared to a similar group of 27 children with SCD who underwent OC. Conclusions  With good peri-operative management, LS is feasible and safe in children with SCD and can be done concomitantly with cholecystectomy. Currently, it requires more operative time than the open approach. This is specially so for children with SCD who are known to have a large spleen with severe adhesions. It is, however, superior to OS with regard to duration of hospital stay, cosmetic appearance, post-operative complications, and post-operative recovery. LC is also safe in children with SCD. When compared with OC, it is associated with less post-operative complications, a shorter hospital stay, better cosmetic appearance and a faster recovery.     

Outcomes for children with gallbladder abnormalities and sickle cell disease

OBJECTIVE: To determine whether elective cholecystectomy is justifiable in children with sickle cell disease (SCD), gallbladder abnormalities, and minimal clinical symptoms. STUDY DESIGN: A retrospective review comparing clinical presentations and abdominal ultrasound results with outcomes in 146 children with SCD. RESULTS: Ultrasound examination showed sludge or stones in 83 of 146 children (57%). This was found during a diagnostic ultrasound in 59 patients (71%) and during a screening ultrasound in 24 asymptomatic patients (29%). Fifty-four (65%) children with a positive ultrasound underwent cholecystectomy; 13 of these were initially asymptomatic patients who had subsequent development of clinical symptoms. Of the patients with cholecystectomy, 93% had histopathologic evidence of cholecystitis. Perioperative complications were rare, and there were no episodes of postoperative acute chest syndrome. Children who underwent elective surgery had an average 12-days-shorter overall hospital stay than those who underwent emergent surgery (4 vs 16 days, P <.001). CONCLUSIONS: Elective laparoscopic cholecystectomy may be safely performed in children with SCD. Surgery should be strongly considered at the time of gallstone diagnosis before symptoms or complications develop. Histopathologic chronic cholecystitis does not correlate with clinical symptoms.     

慢性肾脏疾病患儿维生素D水平研究进展

儿童慢性肾脏疾病(chronic kidney disease,CKD)是威胁儿童正常生长发育的主要疾病之一,在我国主要病因以肾小球疾病为主,常并发心血管疾病、肾性贫血、肾性骨病等,严重影响儿童生活质量.CKD患儿由于肾脏病变25-羟维生素D[25-(OH) Vit D]羟化受到影响、尿毒症导致的皮肤变化使骨化三醇合成减少等因素常引起25-(OH) Vit D水平低下.该文就CKD患儿25-(OH) Vit D基础水平和影响因素作一综述.     

25 Hydroxyvitamin D and vitamin E absorption in healthy children and children with chronic intrahepatic cholestasis

Patients with chronic cholestasis have reduced 25-hydroxyvitamin D (25 OHD) and vitamin E levels. We determined serum concentrations of 25 OHD, 1,25-dihydroxyvitamin D [1,25(OH)₂D] and vitamin E before and after oral administration of 10 g/kg body weight 25-hydroxyvitamin D₃ (25 OHD₃) and 100 IU/kg body weight vitamin E, respectively, in 4 patients with intrahepatic cholestasis and 6 healthy children. Vitamin E increased in all controls but in only one of the four patients. In contrast, oral 25 OHD₃ induced a normal rise in circulating 25 OHD and 1,25(OH)₂D. The low serum levels of 25 OHD in the patients before the oral bolus may have been due to inadequate parenteral vitamin D administration and/or to the simultaneous phenobarbital treatment. The latter possibility is supported by the increase of serum 25 OHD into the normal range after withdrawal of phenobarbital in one of the four patients.We conclude that vitamin E has to be supplemented parenterally or in water-soluble oral form. Further studies are necessary to clarify whether high-dose long-term oral 25 OHD₃ supplementation is sufficient to prevent vitamin D deficiency in patients with chronic cholestasis.Abbreviations 25 OHD 25-hydroxyvitamin D - 25 OHD₃ 25-hydroxyvitamin D₃ - 24,25(OH)₂D 24,25-dihydroxyvitamin D - DBP vitamin D binding protein This report was presented in part at the XIX European Symposium on Calcified Tissues in Stockholm, June 1986. This study was supported by the Deutsche Forschungsgemeinschaft Bu-199-9-1     

Clinical profile of sickle cell disease in Orissa

Children comprised 52% of patients with Sickle Cell Disease (SCD). Types of Sickle Cell Disease encountered were SS (92.7%). SB thalassaemia (6.7%) and SD disease (0.7%). The disease was widespread in almost all castes and communities in the society; largest number of patients (20%) belonging to scheduled castes and only 1.4% were from scheduled tribes. Maximum number of cases were in the age group 2–4 and 4–6 years, many of whom died around this age. Besides attacks of pain, jaundice and anemia, frequent attacks of fever with anemia or only anemia in childhood were a predominant presenting feature. Splenic sequestration was frequent (10.1%). The patients usually had a steady state hemoglobin level of 6–10 g/dl, with which they thrived well. Fetal hemoglobin was 5–30%. Blood transfusion was not a frequent requirement, but prophylactic long acting penicillin was helpful in preventing frequency of crisis.     

Acute chest syndrome in sickle cell disease: etiology and clinical correlates

Acute chest syndrome (ACS) is a new pulmonic process in a clinically ill patient with sickle cell disease. We prospectively analyzed 102 episodes of ACS in patients in our hospital during a 2-year period to study cause and clinical correlates. In 12% of the episodes, ACS was judged to be secondary to bacterial pneumonia (including only 3% secondary to Streptococcus pneumoniae), 8% was associated with uncomplicated viral pneumonias, and 16% with mycoplasmal pneumonias. The clinical course and seasonal variations in these groups were compared with those in the remaining 64% of episodes. In comparison with episodes of ACS of undetermined origin (presumably secondary to pulmonary infarct, atelectasis, or missed infections), patients with bacterial pneumonia were sicker, as shown by fever and hospitalization of longer duration, the percent of those requiring red blood cell transfusion, and the presence of pleural effusions. The lower incidence of bacterial pneumonias among our patients compared with that previously reported may reflect our use of penicillin prophylaxis and pneumococcal immunization to prevent S. pneumoniae infections.