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Terms in this set (32)
Factors involved in testicular descent in the fetus
- Endocrine factors } androgens, estrogens, MIS, descendin;
- Genitofemoral nerve and calcitonin gene-related peptides;
- Intra-abdominal pressure;
- Growth differential;
Indications for in utero decompression of obstructive uropathy
- BOO suspected (bilateral hydronephrosis + oligohydramnios;
- Urinary indices favorable } Na <110, Cl <100, Osmolality <210, β2-microglobulin <10-20;
- Karyotype normal;
- Informed consent;
- No evidence of other congenital anomalies;
- No evidence of cystic kidneys;
- Early onset oligo (20-25wks);
RFs for renal vein thrombosis in a child
→ "Premature SPUD CAN'T Dance"
- Prematurity ;
- Sepsis ;
- Polycythemia ;
- Umbilical artery catheterization ;
- Dehydration ;
- Nephrotic syndrome;
- DM mother;
Indications for prophylactic Abx for the GU tract in kids
→ "Before 2-3 mos, VIPs Prescribe Abx In Reflux"
- UTI in child <2-3mos of age;
- Instrumentation (urethral);
- Partial obstruction (eg UPJO, ureterocele);
- Prenatal hydronephrosis, awaiting investigations;
- Awaiting radiologic tests after febrile UTI;
- Reccurent UTIs + normal GU tract
Indications for "early" GU imaging in kids
→ "UP UP Away"
- Unusual infecting organism;
- Poor response to Abx treatment after 2-3 days;
- Unknown source of infection;
- Partial obstruction (known);
- Azotemia (newly Dx'd)
Syndromes associated with unilateral renal agenesis (URA)
→ "Frazer over Turned the Bus on DVP, Killed Many"
- Frazer's syndrome;
- BOR syndrome;
- DiGeorge syndrome;
- Poland's syndrome;
- Mayer-Rokitansky-Kuster-Hauser (MRKH)
Different forms of renal fusion anomalies
→ "I See Lumpy Logs Down South" or "DILLSS"
- Inferior (most common);
- Lump or cake;
- Disc or donut;
Genetic syndromes associated with horseshoe kidney
→ "go TET the horsey"
- Edward's (trisomy 18);
Complications of a horseshoe kidney
→ "SHUT the barn"
- Tumours } RCC, TCC, Wilm's
Genetic cystic diseases of the kidney
MC JAFA Man
Multiple malformation syndrome;
Congenital nephrosis (AR);
Juvenile nephronophthisis (AR) ;
Familial glomerulocystic Dz (AD);
Medullary cystic disease (AD)
Non-heritable cystic disease of the kidney
MB MASS D
Benign multilocular cyst;
Acquired renal cystic disease;
Sporadic glomerulocystic diseae;
Diverticulum of calyx
Syndromes associated with MSK
→ "MSK is a BECH"
- Ehler's Danlos;
- Caroli's disease;
Diseases with renal cysts that have hyperplastic lining
- AD PCKD;
GU anomalies associated with VUR
→ "Visa Has Reversed DUMMIED Charges"
- Horseshoe kidney;
- Renal agenesis (URA);
- Diverticulum (bladder);
- Imperforate anus;
- Duplex system;
Indications to surgically correct VUR
→ "SHARP Blade Now"
- Scars (new);
- High grade VUR with scars;
- Anomalies at UVJ (diverticulum, etc);
- Renal impairement } growth, function, etc;
- Persisting into puberty or prior to pregnancy;
- Breakthrough UTIs;
Surgical options for VUR repair
→ "In pacific coast golf, Everyone Likes, Combined Play"
- Intravesical } Politano-Leadbetter;
} Cohen cross trigonal;
- Extravesical } Lich-Gregoire;
- Combined } Paquin
Syndromes associated with Prune Belly Syndrome
→ "Ed's Belly Turned Prune"
- Edward's syndrome (trisomy 18);
- Patau's syndrome (trisomy 13)
DDx of dilated bladder on antenatal U/S
→ "PeePee Under Abdomen MAN"
- Ureterocele (obstructing);
- Anterior obstruction (AUVs, syringocele);
- Atresia of urethra;
- Neurogenic bladder
RFs for bladder exstrophy
- Family history;
- ART used;
- Parity (multip);
- Progesterone in T1;
- Young mother
Conditions associated with PUVs
→ "P Vowels (AEIOU)"
- PBS ;
- Ejaculation abnormalities;
Contraindications to circumcision
→ "Hebrew's Bris WISH ... Do Cx"
- Bleeding disorder;
- Webbed or hidden penis;
- Illness or jaundice;
- Small penis;
- Hydrocele or hernia (large);
- Dorsal hood deformity;
- Chordee without hypospadias
RFs for UDTs
→ "Undescended Balls, Family Please ACCEPT Son"
- Underweight (low birth wt);
- Breech presentation;
- Family Hx +ve;
- Asian descent;
- Congenital anomalies;
- Estrogen exposure in utero;
- Small for gestational age
Anomalies associated with UDT (incomplete list)
→ "Will U Prune Horse's Hair Please?"
- Prune Belly syndrome;
- Horseshoe kidney;
Sex Chromosomal DSD causes:
→ Sex chromosomal DSD } "Killer XX Turned Pure but My Parts Vanished Too"
- Klinefelters (47,XXY);
- 46, XX;
- Turner's (45,XO);
- Pure gonadal dysgenesis (XX or XY);
- Partial gonadal dysgenesis;
- Vanishing testis;
- True hermaphrodite (Ovotestis DSD)
DSD classification of masculinized female
→ Masculinized female } "CAT
- Androgens (maternal);
- Tumours (ovarian or adrenal)
DSD classification of Undermasculinized male
→ Undermasculinized male } "Let's Tell All 5 Men"
- Leydig cell a genesis;
- Testosterone biosynthesis problem (StAR, 3βHSD, 17α-hydroxylase, 17,20 lyase, 17βHSD);
- AIS (complete and partial);
- 5AR deficiency;
- MIS problems (PMDS)
Features of Turner's syndrome
→ "BBLLAC SiNNNNS"
- Broad chest ;
- Bicuspid aortic valve ;
- Low-set ears ;
- Lymphedema at birth ;
- Aortic coarctation ;
- Cubitus valgus ;
- Short 4th metacarpal bone;
- Nails (hypoplastic);
- Nipples are widespread;
- Nevi (pigmented);
- Neck (webbed);
- Short stature
Investigations required in a child with ambiguous genitalia
→ "FUUKED Large, Then Gone After 17"
- Urine for 17-ketosteroids;
- Anti-Mulllerian hormone (MIS);
- serum 17-OH-progesterone & DOC
Main issues to discuss and consider when assigning gender to child with DSD
→ "Males And Females PMS"
- Malignancy risk of gonads ;
- Appearance overall ;
- Fertility potential ;
- Psychosocial outcomes;
- Minimizing medical procedures;
- Sexual function;
Causes of occult spinal dysraphism
Sacral Dysraphism DIAL CT
Aberant N. Roots,
Cauda equina tumour,
Tight Filum Terminale,
Causes of occult myelomeningocele
Viral Myelomeningocele FRIC
Viral infection in mother,
Folic Acid Deficiency,
Idiopathic Chromosomal Abnormality
Associated anomalies with MCDK
1. UPJO C/L
2. VUR C/L
3. UVJO C/L
4. Ureterocele C/L
5. Non obstructed dilated RP C/L
5. ipsilateral ureteral atresia
Ipsilateral cystic dysplasia of testes
6. Undescended testes
7. Cardiac, GI, and Pulmonary anomalies.
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