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Background: Desert hedgehog (DHH), as a member of the Hedgehog (HH) family, is mainly involved in testicular development and peripheral nerve sheath formation. A DHH variant has been identified in patients with 46, XY gonadal dysgenesis (46, XY GD) with or without neuropathy, but few reports mention the involvement of other complications.Case presentation: Here, we report a Chinese female patient who was hospitalized at 14.3 years old due to slow breast development for more than 1 year. She had a female genitalia phenotype and breast development started at 13 years old but progressed slowly. She was not yet menarche on admission, and she had intermittent muscle cramps in her hands and feet. Her karyotype analysis was 46, XY and the SRY gene was positive. Surgical exploration revealed no uterus or ovaries, and the pathology of bilateral gonads was dysplastic testis tissue, which was consistent with partial gonadal dysgenesis (PGD). Genetic analysis identified a homozygous pathogenic variant in DHH exon 3 (c.1027T>C, p. Cys343Arg). During the 6-year follow-up, she received estrogen replacement therapy, resulting in breast development progression without gender dysphoria. However, her peripheral neuropathy became more obvious, and a nerve conduction study (NCS) indicated decreased nerve conduction velocity and action potential. In addition, she also suffered complications such as obesity, insulin resistance, fatty liver, and gastric ulcers.Conclusion: In the present study, we reported a case of 46, XY GD with minifascicular neuropathy caused by a DHH homozygous variant, and we summarized the reported cases worldwide. For the first time in such patients, we showed a comparison of NCS changes with age as well as the presence of multiple complications not previously reported.

Supplementary table: Table 1–1 and Tables 1 and 2 presents related molecule for indentification in TARTs.

Objectives : To investigate the drug resistance of extended-spectrum beta-lactamase (ESBL)-producing Escherichia coli (E. coli ) and Klebsiella pneumoniae (K. pneumoniae) in children with urinary tract infection (UTI) and to provide the rationale for clinical use of antibiotics. Methods : This is a retrospective analysis of drug susceptibility in children with E. coli or K. pneumoniae-positive urine culture between August 2013 and August 2017, Shenzhen Children’s Hospital, Shenzhen, China. Drug resistance was statistically assessed using Fisher exact test and χ2 test. Results : A total of 698 cases of E. coli, 426 of which were confirmed ESBL-producing strains, and 217 cases of K. pneumoniae, including 111 ESBL-producing strains, were detected, and the difference in proportion of positive ESBL-producing strains (61.03% versus 51.15%) was statistically significant (p=0.010). The average drug resistance rates of E. coli and K. pneumoniae to piperacillin/tazobactam, meropenem, ertapenem, imipenem, and amikacin were less than 15%. The average resistance rates of ESBL-producing E. coli and K. pneumoniae to cefpodoxime, cefixime, cefazolin, and ceftriaxone was less than 98%, while average resistance rates for non-ESBL-producing bacteria to the above 4 drugs was less than 20%. Conclusion : In southern China, the proportion of ESBL-producing strains and the drug resistance rates of E. coli and K. pneumoniae in UTI in children was high, but their resistance rates to carbapenems and β-lactamase inhibitor complexes containing tazobactam were low. Carbapenems are the most effective antibacterial drugs for the treatment of ESBL-producing bacteria. Saudi Med J 2019; Vol. 40 (11): 1111-1115 doi: 10.15537/smj.2019.11.24547 How to cite this article: Keshi L, Weiwei X, Shoulin L, Xiadong L, Hao W, Junhai J, et al. Analysis of drug resistance of extended-spectrum beta-lactamases-producing Escherichia coli and Klebsiella pneumoniae in children with urinary tract infection. Saudi Med J . 2019 Nov;40(11):1111-1115. doi: 10.15537/smj.2019.11.24547.

An improved large-signal equivalent-circuit model for SiGe HBTs based on the MEXTRAM model (level 504.5) is proposed. The proposed model takes into account the soft knee effect. The model keeps the main features of the MEXTRAM model even though some simplifications have been made in the equivalent circuit topology. This model is validated in DC and AC analyses for SiGe HBTs fabricated with 0.35-μm BiCMOS technology, 1 × 8 μm2 emitter area. Good agreement is achieved between the measured and modeled results for DC and S-parameters (from 50 MHz to 20 GHz), which shows that the proposed model is accurate and reliable. The model has been implemented in Verilog-A using the ADS circuit simulator.