Human sex ratios and sex distribution in sibships of size 2

We previously analyzed data from the U.S. National Health Interview Survey (NHIS, 1998 to 2002) on families with two biological children (10 years of age and younger) and found that the distribution of families with two boys, two girls, and one boy + one girl did not statistically conform to a binomial distribution regardless of the boy/girl sex ratio used. Using the best estimate of the sex ratio from the data, we found that there were significantly more families with opposite-sex siblings than families with same-sex siblings. No biological mechanism could explain these results at the time. In the present study we conducted an analysis of the first two children in sibships of size 3 from the same data source and found that there are significantly more same-sex sibships than unlike-sex sibships. Combining the two sets of data for the first two children produced observed numbers in close agreement with the expected numbers. A hypothesis of parental choice (family planning) appears to be strongly supported as an explanation for the discrepancies in the two sets of data individually. For example, parents who have a boy and a girl (either order) as their first two children are more likely to stop having children ("stopping rule") than are parents whose first two children are of the same sex.     

Successful Ablation of Antero-septal Accessory Pathway in the Non-Coronary Cusp in a Child

A 15-year-old boy with Wolff-Parkinson-White syndrome underwent an electrophysiology study for symptoms of palpitations and persistence of pre-excitation during peak exercise. He was detected to have right antero-septal accessory pathway with relatively long effective refractory period and no inducible tachycardia. He had only transient normalization with cryoablation. Eight months later, he presented again with two episodes of seizures with preceding palpitations. Neurology evaluation was unremarkable with a normal electroencephalogram. In view of his symptoms in association with evidence of pre-excitation, he underwent a second electrophysiology study with ablation. Cryoablation in the anterior septum again achieved only transient normalization. Mapping in the non-coronary cusp identified an earliest accessory pathway potential. RF ablation was performed in the non-coronary cusp with immediate normalization of his electrocardiogram. At 6 month follow-up, he continues to have no pre-excitation on his EKG. Ablation of the anteroseptal accessory pathway in the non-coronary cusp can be safely performed in patients' refractory to conventional ablation sites and techniques.     

Misdiagnosis and exacerbation of unusual obsessive-compulsive disorder presentation with risperidone and clozapine in an adolescent girl - A case report

Obsessive-compulsive disorder (OCD) is a heterogenous disorder with different clinical presentations. The most common symptoms are those that involve contamination, possible harm, ordering/symmetry, aggressive/sexual/religious concerns and hoarding. A variety of less common symptoms have been described. Unusual OCD symptoms may lead to misdiagnosis, inappropriate treatment with possible serious side effects. In this report we present a case of an adolescent girl in which unusual OCD presentation and symptoms were misinterpreted to represent psychosis and exacerbation of OCD symptoms with risperidone and clozapine treatment. We discuss the possible pathophysiological mechanisms of OCD symptom exacerbation, clinical implications, and successful management of this case, with fluvoxamine therapy. This case may represent the first report of musical obsessions successfully managed with fluvoxamine therapy.     

Microsporum canis Infection in Three Familial Cases with Tinea Capitis and Tinea Corporis

We report a familial infection caused by Microsporum canis. The first two patients were a 30-year-old female and her son, a 5-year-old boy, who came in contact with a pet dog at a farm house. The boy then suffered from hair loss for 3 months. There were circular and patchy alopecia with diffuse scaling on his scalp. Meanwhile, his mother also developed patchy erythema and scaling on her face. Several weeks later, the boy’s sister, a 4-year-old girl, was noted to have inconspicuous scaly plaques in the center of her scalp. The development of tinea capitis in the two children and tinea corporis in their mother were diagnosed based on the positive KOH examination. Morphologic characteristics and sequencing of the internal transcribed spacers 1 and 2, amplified from primary culture isolates, confirmed that their infections were caused by the zoophilic M. canis. Repetitive sequence-based molecular typing using the DiversiLab system secreted enzymatic activity analysis, and antifungal susceptibility indicated that these isolates might share the same source. The boy and girl were cured by the treatment with oral itraconazole and topical naftifine–ketoconazole cream after washing the hair with 2 % ketoconazole shampoo, and their mother was successfully treated by terbinafine orally in combination with topical application of naftifine–ketoconazole cream.     

Primary Amoebic Meningoencephalitis in Britain

Meningoencephalitis proved to be due to an amoeba (Naegleria) has been diagnosed in Great Britain for the first time. The first patient (a boy of 2) survived longer than any previously recorded cases, but in spite of early diagnosis and treatment he died 15 days after the onset of meningeal symptoms.Two other children who were exposed to the same possible source of infection (a warm, muddy puddle) had similar symptoms and developed mild meningitis. A naegleria was isolated from the cerebrospinal fluid of one of them. Both recovered after treatment with amphotericin.     

Iminoglycinuria — A “harmless” inborn error of metabolism?

Summary A boy with iminoglycinuria was discovered during routine urinary amino acid screening of a series of children with severe visual handicap. In addition to iminoglycinuria and blindness due to Leber's tapeto-retinal degeneration, this boy has profound perceptive deafness dating from infancy. It is concluded, on the basis of a study of previous cases reported, that the iminoglycinuria is not causally related to his blindness and deafness and may represent an essentially harmless inborn error of metabolism. The view that he suffers from three distinct autosomal recessive conditions receives some support from the fact that he may be the offspring of a mating between full sibs.The results reported here are drawn in part from theses submitted by V.M.P. and D.N.W. to the University of Oxford in partial fulfilment of the requirements for the D. Phil. degree.     

Sex ratio at birth in India, its relation to birth order, sex of previous children and use of indigenous medicine

Objective

Sex-ratio at birth in families with previous girls is worse than those with a boy. Our aim was to prospectively study in a large maternal and child unit sex-ratio against previous birth sex and use of traditional medicines for sex selection.

Main Outcome Measures

Sex-ratio among mothers in families with a previous girl and in those with a previous boy, prevalence of indigenous medicine use and sex-ratio in those using medicines for sex selection.

Results

Overall there were 806 girls to 1000 boys. The sex-ratio was 720∶1000 if there was one previous girl and 178∶1000 if there were two previous girls. In second children of families with a previous boy 1017 girls were born per 1000 boys. Sex-ratio in those with one previous girl, who were taking traditional medicines for sex selection, was 928∶1000.

Conclusion

Evidence from the second children clearly shows the sex-ratio is being manipulated by human interventions. More mothers with previous girls tend to use traditional medicines for sex selection, in their subsequent pregnancies. Those taking such medication do not seem to be helped according to expectations. They seem to rely on this method and so are less likely use more definitive methods like sex selective abortions. This is the first such prospective investigation of sex ratio in second children looked at against the sex of previous children. More studies are needed to confirm the findings.     

Increased risk of death from measles in children with a sibling of opposite sex in Senegal.

OBJECTIVE--To examine whether contracting measles from a sibling of the opposite sex affects mortality. DESIGN--Prospective registration during 15-20 years of all births and deaths, including 243 measles related deaths. Measles infection was not registered; however, as in fatal cases measles was probably contracted from a maternal sibling the risk of dying during measles outbreaks was examined in families with two boys, two girls, or a boy and a girl. SETTING--31 small villages in two rural areas of eastern Senegal. SUBJECTS--766 children living in families with two children aged under 10 years during outbreaks of measles, 107 (14%) of whom died of measles. MAIN OUTCOME MEASURE--Deaths from measles, size of village, age and sex of maternal siblings. RESULTS--The interval between outbreaks in the same village was greater than 10 years. The risk of dying of measles was significantly related to age, increasing with the age difference between siblings and decreasing with the size of village. In a multiple logistic regression analysis adjusting for these background factors, children in families with a boy and a girl had a significantly higher mortality than children in families with two boys or two girls (odds ratio = 1.81, 95% confidence interval 1.17 to 2.82). The increase in risk was the same for boys and girls in families with two children one of whom was a boy and one a girl. CONCLUSION--Cross sexual transmission may be an important determinant of severity of measles infection.     

三种非典型抗精神病药对儿童青少年精神分裂症患者血脂、肝功能和认知功能的影响

摘要 目的：探讨利培酮、阿立哌唑、奥氮平分别对儿童青少年精神分裂症患者肝功能、血脂和认知功能的影响。方法：选取2015年1月至2019年12月我院收治的84例儿童青少年精神分裂症患者,采用乱数表法随机分为阿立哌唑组（n=28,阿立哌唑治疗）、利培酮组（n=28,利培酮治疗）、奥氮平组（n=28,奥氮平治疗）,均治疗8周,对比三组患者症状评分、血脂、肝功能、认知功能以及不良反应。结果：三组治疗8周后阳性与阴性症状量表（PANSS）评分整体比较无差异（P>0.05）,三组治疗8周后PANSS评分均较治疗前降低（P<0.05）。奥氮平组、利培酮组治疗8周后三酰甘油(TG)、总胆固醇(TC)、低密度脂蛋白(LDL-C)高于阿立哌唑组,且奥氮平组高于利培酮组（P<0.05）;奥氮平组、利培酮组治疗8周后高密度脂蛋白(HDL-C)低于阿立哌唑组,且奥氮平组低于利培酮组（P<0.05）。三组不良反应发生率整体比较无差异（P>0.05）。阿立哌唑组治疗8周后延迟回忆数、即刻回忆数、回忆总数、再认数评分均高于利培酮组、奥氮平组（P<0.05）。利培酮组治疗8周后ALT、AST、TBIL高于治疗前（P<0.05）,利培酮组治疗8周后ALT、AST、TBIL高于阿立哌唑组、奥氮平组（P<0.05）。结论：利培酮、阿立哌唑、奥氮平应用于儿童青少年精神分裂症中,可获得相当的治疗效果,其中利培酮对肝功能影响较大,奥氮平对人体血脂影响较大,阿立哌唑对血脂、肝功能影响轻,改善认知功能效果优于利培酮、奥氮平。     

Long term follow-up of two sibs with an autosomal recessive form of chrondrodysplasia punctata and epilepsy

Long term follow-up of two sibs with an autosomal recessive form of chrondrodysplasia punctata and epilepsy: A variety of osteodysplasias are referred to with the term chondrodysplasia punctata (CDP). Here we report on two sibs, a boy and a girl, with probable autosomal recessive form of CDP and epilepsy followed-up for 30 and 19 years, respectively. Family history was unremarkable but for consanguinity. Pregnancies and deliveries were uneventful. At birth, length was 46 (-3SD) and 45 (-4SD) cm, respectively. Craniofacial dysmorphism was noted: severe nasal hypoplasia, flat face, hypertelorism, a low nasal bridge, short stature. Skeletal abnormalities included epiphyseal stippling in the thoracic spine, bilateral proximal and distal humeri, femur, tibia and bilateral carpal and tarsal bones. The boy had a hemivertebrae T12, with absence of a rib. After the age of 6 years facial dysmorphism had improved. Final height was 154 cm (-3SD) in the boy and 158 cm (-0,5SD) in the girl. The boy was operated on for scoliosis. Both sibs had club feet, the girl had also genu valgum. IQ was evaluated to be 55 in the girl and 83 in the boy. The first non febrile generalized seizure appeared in the boy when he was 11 months of age, and in the girl when she was 25 months of age. Both had many other seizures and were taking antiepileptics. EEG were abnormal. Karyotypes were normal. Extensive screening for metabolic disorders was normal. Acquired in utero CDP were excluded. We suggest the sibs described in this report have yet another provisionally unique possibly autosomal recessive syndrome, with CDP and epilepsy as phenotypic traits.     

Secondary amyloidosis due to Schistosoma mansoni infection.

Four children with Schistosoma mansoni infection and the nephrotic syndrome with varying degrees of renal dysfunction were found on histological examination to have amyloidosis. In one boy who had no evidence of renal failure complete clinical regression of his nephrotic syndrome and almost complete disappearance of renal amyloid deposits followed adequate treatment of his schistosomal infection. Conditions known to cause secondary amyloidosis were excluded in all four patients. Amyloidosis in association with mansoni infection is probably more common than is currently recognised. Early treatment of the infection, before renal function becomes impaired, may result in regression of the amyloidosis.     

Preference for a first-born boy in Western societies

Many studies in the last 45 years have shown that women prefer a boy to a girl for their first-born child, suggesting that this preference is universal in Western societies. A careful examination of these studies reveals, however, that the subjects were often women who were not pregnant and/or students. A review of sixteen studies with first-time-pregnant women showed that in most cases the opposite was true, namely, that a girl was desired more often than a boy, especially during the last two decades (from 1981 to 1996). Data concerning expectant fathers, however, indicate that they prefer a boy rather than a girl. A preference for a boy first was also observe for both non-expectant males and females. Women's preference for a male child decreased and men's preference increased slightly when the two sub-periods (before 1980vs after 1981) were compared. A difference between men and women is, however, evident whatever the sub-period: men more often prefer a boy than women. These findings suggest that something specific about being pregnant is related to the preference for a girl first.     

Homozygosity mapping to chromosome 5p15 of a gene responsible for Hartnup disorder

Hartnup disorder is an autosomal recessive phenotype involving a transporter for monoamino-monocarboxylic acids. Genetic analysis of the mouse model mapped its locus to human chromosome 11q13 (8). We report here the results of linkage analysis in two Japanese first cousin-marriage families. In the first family, the proband had Hartnup disorder and his deceased older brother was reported to have had typical Hartnup symptoms. The younger brother of the proband was shown to have decreased tryptophan absorption by oral loading test. In the second family, a 6-year-old girl, the proband, had specific hyperaminoaciduria. DNA was isolated from either blood samples or umbilical cord stumps. Genome-wide screening by homozygosity mapping was conducted. Taking into account that the older brother was affected and the younger brother was a carrier in the first family, homozygosity mapping (LOD score = 3.55) and GENEHUNTER (LOD score = 3.28) locates the locus of the Hartnup disorder on 5p15.     

Protopathic stimulant use among children with symptoms of ADHD

The purpose of the current study was to examine protopathic stimulant use among children with the symptoms of ADHD but do not have a diagnosis of ADHD. Protopathic or prodromal stimulant use refers to the use of stimulants by children with the symptoms of ADHD prior to a diagnosis of ADHD. In the current study, we examined children with the symptoms of ADHD who received stimulant treatment across time and with respect to several background variables. Our results indicate that these children who receive stimulant treatment without a diagnosis of ADHD are significantly more like to be eventually diagnosed with ADHD than not. Results also indicate that these children who receive stimulant treatment but do not yet have a diagnosis of ADHD are significantly more likely to have insurance that does not pay for diagnostic procedures. These results are discussed in view of treatment.     

Hyperviscosity in HIV infected children--a potential hazard during intravenous immunoglobulin therapy

A four year old boy with symptoms of HIV infection and serum IgG of 53.2 g/l had been treated for 16 months with regular infusions of intravenous immunoglobulin (IV IgG). During one such infusion he developed temporary neurological symptoms and signs suggestive of the hyperviscosity syndrome. Serum relative viscosity was raised at 5.0 (normal range 0.42-2.78). Subsequent IV IgG infusions given at a slower rate have been without adverse reactions. In a study of eight HIV infected children including the index case, and 20 children not infected with HIV, serum relative viscosity was significantly raised in the HIV infected children (p less than 0.01; students t-test). Viscosity correlated with total serum IgG, which was raised in all HIV infected children, and with serum IgM. In HIV infected children with very high levels of serum IgG a slow rate of IV IgG infusion should therefore be chosen due to the possibility of hyperviscosity.     

须癣毛癣菌所致脓癣病发的扫描和透射电镜观察及菌体外酶活性分析：2例报告

目的报道2例由须癣毛癣菌引起的脓癣,并对病发做扫描和透射电镜观察及菌体外分泌酶活性测定。方法例1为6岁女孩,表现为头顶部炎性肿块、脓血伴脱发;例2为1岁8个月男孩,表现为头部红斑,无脓肿及脱发。家中分别饲养宠物狗和兔。病发真菌镜检均阳性后做真菌培养、生化鉴定及分子生物学鉴定。对2例患儿病发用扫描和透射电镜进行超微结构观察。对2株分离的菌株分别进行细胞外分泌酶测定。结果2株菌均鉴定为万博节皮菌（须癣毛癣菌的有性期）。扫描电镜见例1的病发受累毛干表面大量炎性组织碎屑、红细胞、脓细胞及上皮细胞;透射电镜在毛皮质内发现菌体。扫描电镜见例2的受累毛干表面真菌孢子及炎性渗出物;透射电镜见毛发内外菌体。2株菌的外分泌酶主要为碱性磷酸酶、白氨酸芳胺酶、β-葡萄糖甙酶、N-乙酰-葡萄糖胺酶、α-甘露糖甙酶。经口服伊曲康唑胶囊等综合治疗后2例均痊愈,未见不良反应。结论确诊2例须癣毛癣菌所致脓癣,伊曲康唑胶囊等药物联合治疗有效。     

Speech in same- and different-sex twins 4 and 5 years old.

The purpose of this study was to examine the impact of the sex makeup of pairs of twins on language acquisition. Past research indicated that this variable plays a role in speech problems of twin children. The questions raised were whether being a boy or a girl and having a boy or girl co-twin affected linguistic performance. A language test was given to 30 pairs of boy-girl twins, 16 pairs of boy twins, and 16 pairs of girl twins whose average age was 4 years 8 months. Their test scores confirmed our hypotheses. The poorest performance was obtained by the boy twin pairs and the best performance, by either the girl twin pairs or the different-sex pairs. The results were interpreted in the light of findings on language learning differences between girls and boys, and also in terms of Vygotsky's zone of proximal development.     

Social and emotional complications in a clinical trial among adolescents with diabetes mellitus.

Observations are reported on the social and emotional events occurring among children with diabetes mellitus and their families while taking part in a demanding clinical trial. Participants were selected on the basis of: (1) age over 10 years, (2) "informed consent," (3) cooperation with diabetic care, and (4) family stability. Despite endeavours to apply these criteria, it subsequently emerged that one father had doubts about his daughter participating; one family was suffering from severe marital discord; a girl (11 years) and a boy (10 years) were unexpectedly distressed by the venepunctures required; and another girl (13 years) was falsifying the results of her urine tests. All the families wished to complete the trial, and only one did not because of recurrent hypoglycaemia. The psychosocial problems encountered during the trial were unpredictable and occurred despite selection. Documentation of these problems allowed appropriate emotional support to be offered to the children and their families and provided for a fuller and more reliable interpretation of the trial results than would have been possible from the numerical data alone.     

Corticosteroid-sparing effect of chromoglycate sodium and nedocromil

The most appropiate management for bronchial asthma is the control of airway inflammation. Corticosteroids are the most effective anti-inflammatory drugs available, but they have a number of side effects; most of these are dose-dependent. In children, asthma control should be accomplished with low steroid doses possibly given by inhalation. In a double-bind placebo-controlled crossover study a group of children with mild to moderate asthma received NED 16 mg/day or BDP 400 mug/day. Values for FEV(1), PEF, symptoms use ofbronchodilators overlapped, whereas bronchial hyper-responsiveness assessed by histamine bronchoprovocation challenge was better with BDP than NED. In another case, one boy with high bronchial hyper-reactivity assessed by provocation test with hypertonic solution, experienced a significant improvement only after 2 weeks of therapy with Deflazacort (2 mg/Kg/day) followed by 4 months on combined treatment with NED (16 mg/day) and BDP (300 mu/day). Authors conclude that NED could have a steroidsparing effect over long-term use.