COVID-19 in Single Kidney Patient

The COVID-19 pandemic has affected all people in the world, especially those at risk of kidney disorders. Early kidney damage in patients born with unilateral renal agenesis (URA) or solitary kidney can happen. These patients are at risk of chronic kidney disease (CKD), high blood pressure, and developing proteinuria. Unilateral renal agenesis is a cause of CKD. Therefore, it is very interesting that observe a unilateral renal Patient that Suffers from COVID-19. Hence, the management of these patients with COVID‐19 is an area of interest, and a unique approach is warranted. A 43-year-old male patient with unilateral renal presented to our hospital for corona disease. The case was discussed between the nephrologists, Infectious disease specialists, and nursing head nurses for a care plan daily. The patient had unilateral renal disease, and COVID-19 could have a detrimental effect on the renal, but renal tests were normal, and the patient recovered without acute renal complications. The treatment of such patients is the need for teamwork contain nephrologists, critical care nurses, and specialists in infectious and tropical diseases. This was a new experience in Iran.

Case Report: Uterine Anomalies in Girls With a Congenital Solitary Functioning Kidney

Unilateral renal agenesis and multicystic dysplastic kidney, resulting in a contralateral solitary functioning kidney (SFK), are part of the broad spectrum of congenital anomalies of the kidney and urinary tract (CAKUT). In girls with SFK, screening for asymptomatic Müllerian anomalies of uterus and vagina is not yet routinely performed, and therefore often overlooked until clinical complications in the menstrual cycle or fertility process occur. In this case series, we report on four teenagers with congenital SFK presenting with menstrual problems due to a Müllerian anomaly. Routine peri-menarchal screening for Müllerian anomalies in girls with SFK may provide timely counseling, surgical treatment and prevention of associated complications such as endometriosis, infertility and miscarriages.

Zinner Syndrome—A Rare Cause of Recurrent Epididymitis and Infertility

Zinner syndrome (ZS) is a rare disorder that affects only men. It is characterized by a triad of abnormalities, including unilateral renal agenesis, ipsilateral seminal vesicle cysts, and atresia of the ejaculatory tract. Unfortunately, there is no consensus on the best treatment modality. We describe a case of a young male patient with recurrent epididymitis, dysuria, and frequent urination. In the diagnostic evaluation, we found an extended right seminal vesicle in the ultrasound with hyperechoic fluid inside and an absence of the right kidney. We performed magnetic resonance imaging, computed tomography, and semen analysis confirming Zinner syndrome and deteriorated semen parameters. Urethroscopic evaluation and ultrasound-guided puncture of the seminal vesicle were performed. An abscess was excluded. The cytologic evaluation showed hemosiderophages. Tamsulosin was introduced. We found no signs of relapse in a six-month observation, and the patient had no further symptoms. Therefore, minimally invasive treatment is a feasible option in young patients found with early-stage Zinner syndrome.

The case number 506 of Mostyn Embrey Syndrome: Imaging Studies and Ethics of Naming Syndromes

Background: Mostyn Embrey syndrome is a rare disorder associated with unilateral renal agenesis and malformations of the female reproductive tract. Delayed diagnosis is associated with serious diagnostic difficulties that may lead to inappropriate management including harmful surgery. The aim of this paper is to present imaging studies of case number 506 of the syndrome which was the first case of this rare syndrome in Iraq, and was originally reported in 2016. Ethics of naming syndromes relevant to this syndrome is discussed. Patients and methods: An 18-year-old female presented with acute abdominal pain and poor urine output associated with retention of urine; about three years after the onset of menses. The pain was radiating to the back and pelvis. Her menstrual cycles started at the age of fifteen, and were lasting seven days. Cycles were associated with normal flow. The first physician the patient consulted considered the diagnosis of uterine tumor and the need for hysterectomy, but the family of the patient consultedanother physician. Vaginal exam was performed and showed bulging left vaginal wall. The diagnosis of hematocolpos was made. A clotted blood was drained by trans- vaginal approach. Results: It was difficult to identify the two separate uteri and two separate cervices and to make a diagnosis of didelphys uterus on hysterosalpingography. Abdominal MRI showed left renal agenesis, and two uterine bodies, cervices, and vaginas with normal myometrium and endometrium (uterine didelphys), and normal ovaries. Conclusions: It is recommended that pediatricians, and pediatric nephrologists need to be aware of this syndrome to avoid unexpected presentations which may lead to delayed diagnosis and sometimes to inappropriate management. The imaging modalities that can be used to diagnose Mostyn Embrey syndrome include ultrasonography, hysterosal pingography and MRI. In this case, hysterosal pingography was not of much help. Ultrasound is affordable, non-invasive, widely available imaging modality that contributes to the accurate diagnosis of Mostyn Embrey syndrome. However, there can be difficulties in visualizing the vaginal septum on ultrasound which best shown on MRI. MRI can facilitate early diagnosis and thus help in the prevention of further complications. Syndromes in medicine are often named after the physician or group of physicians that discovered them or initially provided the full clinical picture or the best description of the syndrome. Unfortunately, Mostyn Embrey syndromes has been attributed unfairly and inappropriately to physicians other that those first described them in almost all previous papers.

A Triad of Unilateral Renal Dysgenesis with Ipsilateral Seminal Vesical and Ejaculatory Duct Obstruction: An Uncommon Urogenital Congenital Anomaly, Zinner Syndrome—A Case Report

Zinner syndrome is a rare congenital anomaly of the urogenital system resulting from an in utero insult during the first trimester. This entity comprises a triad of unilateral renal agenesis/dysgenesis with ipsilateral seminal vesical and ejaculatory duct obstruction. This combination of urinary and genital abnormalities occurs because of the closely related embryological origin of these structures from the distal mesonephric (Wolffian) duct. Nearly 200 cases of seminal vesical cysts with ipsilateral renal agenesis have been reported in the literature. The affected person generally presents in early adulthood when the reproductive activity commences. In this report, we present a case of a 22-year-old male with complaints of painful ejaculation.

Hypogonadotropic-hypogonadism (Kallman Syndrome) in Young Adult

Background: Kallmann syndrome (KS) is a rare disorder first described in 1856 and later studied by Kallmann in 1944. It is now designated as olfactogenital dysplasia with an association between agenesis of the olfactory bulbs and hypogonadism. The prevalence of KS is still unknown. The reported incidence is 1 in 8000 to 1 in 10 000 in men and rare in women. More than 24 genes are underlying KS that have been identified. Mutations in these genes are thought to interfere with the expression of cell markers that guide migrating neurons, leading to failed migration of GnRH neurons and olfactory neurons to the forebrain during fetal development. The main clinical characteristics of KS include hypogonadotropic hypogonadism and anosmia or hyposmia. Less common phenotypes include cardiovascular anomalies, unilateral renal agenesis, cleft palate and cleft lip, cryptorchidism and osteoporosis. Magnetic resonance imaging (MRI) can show abnormalities of the olfactory system and other forebrain structures. Other exceptions may be discovered using MRI because of its high resolution and multiplanar capabilities, such as pituitary abnormalities.

Advances in Genetic Diagnosis of Kallmann Syndrome and Genetic Interruption

Kallmann syndrome (KS) is a rare hereditary disease with high phenotypic and genetic heterogeneity. Congenital hypogonadotropic hypogonadism and hyposmia/anosmia are the two major characterized phenotypes of KS. Besides, mirror movements, dental agenesis, digital bone abnormalities, unilateral renal agenesis, midline facial defects, hearing loss, and eye movement abnormalities can also be observed in KS patients. Because of the phenotypic heterogeneity, genetic diagnosis become increasingly valuable to distinguish KS from other disorders including normosmic congenital hypogonadotropic hypogonadism, constitutional delay of growth and puberty, CHARGE syndrome, and functional hypogonadotropic hypogonadism. Application of next-generation sequencing has promoted the discovery of novel pathogenic genes in KS pedigrees. Prenatal diagnosis is an effective method in clinical settings to decrease birth defects and block transmission of genetic disorders. However, pregnant women may suffer from physical and psychological distress when fetuses are diagnosed with congenital defects. Preimplantation genetic testing (PGT) is a prospective approach during the in vitro fertilization process that helps to interrupt transmission of hereditary diseases to offspring at an early stage. Thus, genetic testing and counseling are recommended to KS patients with family histories, prenatal diagnosis and PGT are considered to be useful options.